Doff McElhinney, MD

  • Doff Bryan McElhinney



Work and Education

Professional Education

Univ of California San Francisco, San Francisco, CA, 06/09/1998


Children's Hospital of Philadelphia, Philadelphia, PA, 6/2002


Boston Children's Hospital, Boston, MA, 06/2006

Boston Children's Hospital, Boston, MA, 6/2005

Board Certifications

Adult Congenital Heart Disease, American Board of Internal Medicine

Pediatric Cardiology, American Board of Pediatrics

Conditions Treated

Cardiothoracic Surgery

All Publications

Bronchoscopy in children with tetralogy of fallot, pulmonary atresia, and major aortopulmonary collaterals. Pediatric pulmonology Sidell, D. R., Koth, A. M., Bauser-Heaton, H., McElhinney, D. B., Wise-Faberowski, L., Tracy, M. C., Hanley, F. L., Asija, R. 2017


Children with Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for developing more postoperative respiratory complications than children undergoing other types of congenital heart surgery. Bronchoscopy is used in the perioperative period for diagnostic and therapeutic purposes. In this study, we describe bronchoscopic findings and identify factors associated with selection for bronchoscopy.Retrospective case-control.All patients with TOF/PA/MAPCAs who underwent unifocalization surgery from September 2005 through March 2016 were included. Patients who underwent bronchoscopy in the perioperative period were compared to a randomly selected cohort of 172 control patients who underwent unifocalization without bronchoscopy during the study period.Forty-three children underwent perioperative bronchoscopy at a median of 9 days postoperatively. Baseline demographics were similar in bronchoscopy patients and controls. Patients who underwent bronchoscopy were more likely to have a chromosome 22q11 deletion and were more likely have undergone unifocalization surgery without intracardiac repair. These patients had a longer duration of mechanical ventilation, ICU duration, and length of hospitalization. Abnormalities were detected on bronchoscopy in 35 patients (81%), and 20 (35%) of bronchoscopy patients underwent a postoperative intervention related to abnormalities identified on bronchoscopy.Bronchoscopy is a useful therapeutic and diagnostic instrument for children undergoing unifocalization surgery, capable of identifying abnormalities leading to an additional intervention in over one third of patients. Special attention should be given to children with a 22q11 deletion to expedite diagnosis and intervention for possible airway complications.

View details for DOI 10.1002/ppul.23732

View details for PubMedID 28504356

Cerebral Oxygen Saturation in Children With Congenital Heart Disease and Chronic Hypoxemia. Anesthesia and analgesia Kussman, B. D., Laussen, P. C., Benni, P. B., McGowan, F. X., McElhinney, D. B. 2017


Increased hemoglobin (Hb) concentration accompanying hypoxemia is a compensatory response to maintain tissue oxygen delivery. Near infrared spectroscopy (NIRS) is used clinically to detect abnormalities in the balance of cerebral tissue oxygen delivery and consumption, including in children with congenital heart disease (CHD). Although NIRS-measured cerebral tissue O2 saturation (ScO2) correlates with arterial oxygen saturation (SaO2), jugular bulb O2 saturation (SjbO2), and Hb, little data exist on the interplay between these factors and cerebral O2 extraction (COE). This study investigated the associations of ScO2 and SaO2-ScO2 with SaO2 and Hb and verified the normal range of ScO2 in children with CHD.Children undergoing cardiac catheterization for CHD were enrolled in a calibration and validation study of the FORE-SIGHT NIRS monitor. Two pairs of simultaneous arterial and jugular bulb samples were drawn for co-oximetry, calculation of a reference ScO2 (REF CX), and estimation of COE. Pearson correlation and linear regression were used to determine relationships between O2 saturation parameters and Hb. Data were also analyzed according to diagnostic group defined as acyanotic (SaO2 90%) and cyanotic (SaO2 < 90%).Of 65 children studied, acceptable jugular bulb samples (SjbO2 absolute difference between samples 10%) were obtained in 57 (88%). The SaO2-SjbO2, SaO2-ScO2, and SaO2-REF CX were positively correlated with SaO2 and negatively correlated with Hb (all P < .001). Although by diagnostic group ScO2 differed statistically (P = .002), values in the cyanotic patients were within the range considered normal (69% 6%). COE estimated by the difference between arterial and jugular bulb O2 content (CaO2-CjbO2, mL O2/100 mL) was not different for cyanotic and acyanotic patients (P = .10), but estimates using SaO2-SjbO2, SaO2-ScO2, or SaO2-ScO2/SaO2 were significantly different between the cyanotic and acyanotic children (P < .001).Children with adequately compensated chronic hypoxemia appear to have ScO2 values within the normal range. The SaO2-ScO2 is inversely related to Hb, with the implication that in the presence of reduced Hb, particularly if coupled with a decreased cardiac output, the ScO2 can fall to values associated with brain injury in laboratory studies.

View details for DOI 10.1213/ANE.0000000000002073

View details for PubMedID 28514318

Transcatheter interventions in adults with congenital heart disease: Surveys from the Society for Cardiovascular Angiography and Interventions to identify current patterns of care and perception on training requirements. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Wadia, S. K., Accavitti, M. J., Morgan, G. J., Kenny, D., Hijazi, Z. M., Jones, T. K., Cabalka, A. K., McElhinney, D. B., Kavinsky, C. J. 2017


Interventional catheterization is central to the care of Adults with Congenital Heart Disease (ACHD). Current standards for care provision and training in ACHD intervention are lacking. We sought to examine trends in current practice and training among interventionalists.We analyzed the results of two separate international surveys in June 2016. One was sent to all active members from the Society of Cardiovascular Angiography and Interventions (SCAI) who self-identified Structural Heart Disease or Congenital Heart Disease as a practice area. The second survey was conducted through the Pediatric Interventional Early Career Society (PICES) aimed at pediatric and adult congenital interventionalists in their first seven years after training. The total survey sample sizes were 1,535 and 112, respectively.Response rates for the SCAI and PICES surveys were 15% (237/1,535) and 75% (84/112), respectively. Most respondents (74%) worked at institutions with pediatric and adult facilities in proximity (major medical centers). While 75% of SCAI respondents believed complex transcatheter procedures should be performed by ACHD-trained interventionalists or multidisciplinary teams, only 32% reported such care is being provided at the present time. Most pediatric and adult cardiologists surveyed (73%) do not believe current interventional fellowships provide adequate training for proficiency in ACHD interventions.ACHD management remains underdeveloped in relative terms, particularly in the United States. Significant gaps in interventional standards of practice and future training needs were recognized by this study. Our survey should serve as an impetus to establish training guidelines for physicians who seek to perform ACHD interventions.

View details for DOI 10.1002/ccd.27151

View details for PubMedID 28493591

Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary ValveImplantation. JACC. Cardiovascular interventions Tanase, D., Ewert, P., Georgiev, S., Meierhofer, C., Pabst von Ohain, J., McElhinney, D. B., Hager, A., Khn, A., Eicken, A. 2017; 10 (7): 701-708


This study sought to investigate the impact of tricuspid regurgitation (TR) on right ventricular function after percutaneous pulmonary valve implantation (PPVI).PPVI provides a less invasive alternative to surgery in patients with right ventricular-to-pulmonary artery (RV-PA) conduit dysfunction. Recovery of the right ventricle has been described after PPVI for patients with pulmonary stenosis and for those with pulmonary regurgitation. Additional TR enforces RV dysfunction by supplemental volume overload. Limited data are available on the potential of the right ventricle to recover in such a specific hemodynamic situation.In a matched cohort study, we compared patients who underwent PPVI with additional TR with those without TR.The degree of TR improved in 83% of the patients. In our patients (n= 36) exercise capacity and right ventricular volume index improved similarly 6 months after PPVI in patients with and without important TR. None of them had significant TR in the long-term follow-up of median 78 months.PPVI improves not only RV-PA-conduit dysfunction, but also concomitant TR. In patients with a dysfunctional RV-PA conduit and TR, the decision whether to fix TR should be postponed after PPVI.

View details for DOI 10.1016/j.jcin.2017.01.036

View details for PubMedID 28385408

Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation JACC-CARDIOVASCULAR INTERVENTIONS Tanase, D., Ewert, P., Georgiev, S., Meierhofer, C., von Ohain, J. P., McElhinney, D. B., Hager, A., Kuehn, A., Eicken, A. 2017; 10 (7): 701-708
Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries A 15-Year Experience With 458 Patients CIRCULATION-CARDIOVASCULAR INTERVENTIONS Bauser-Heaton, H., Borquez, A., Han, B., Ladd, M., Asija, R., Downey, L., Koth, A., Algaze, C. A., Wise-Faberowski, L., Perry, S. B., Shin, A., Peng, L. F., Hanley, F. L., McElhinney, D. B. 2017; 10 (4)


Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction.We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure.Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.

View details for DOI 10.1161/CIRCINTERVENTIONS.116.004952

View details for Web of Science ID 000397579800001

View details for PubMedID 28356265

Development and validation of a major adverse transplant event (MATE) score to predict late graft loss in pediatric heart transplantation. journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Almond, C. S., Hoen, H., Rossano, J. W., Castleberry, C., Auerbach, S. R., Yang, L., Lal, A. K., Everitt, M. D., Fenton, M., Hollander, S. A., Pahl, E., Pruitt, E., Rosenthal, D. N., McElhinney, D. B., Daly, K. P., Desai, M. 2017


There is inadequate power to perform a valid clinical trial in pediatric heart transplantation (HT) using a conventional end-point, because the disease is rare and hard end-points, such as death or graft loss, are infrequent. We sought to develop and validate a surrogate end-point involving the cumulative burden of post-transplant complications to predict death/graft loss to power a randomized clinical trial of maintenance immunosuppression in pediatric HT.Pediatric Heart Transplant Study (PHTS) data were used to identify all children who underwent an isolated orthotopic HT between 2005 and 2014 who survived to 6 months post-HT. A time-varying Cox model was used to develop and evaluate a surrogate end-point comprised of 6 major adverse transplant events (MATEs) (acute cellular rejection [ACR], antibody-mediated rejection [AMR], infection, cardiac allograft vasculopathy [CAV], post-transplant lymphoproliferative disease [PTLD] and chronic kidney disease [CKD]) occurring between 6 and 36 months, where individual events were defined according to international guidelines. Two thirds of the study cohort was used for score development, and one third of the cohort was used to test the score.Among 2,118 children, 6.4% underwent graft loss between 6 and 36 months post-HT, whereas 39% developed CKD, 34% ACR, 34% infection, 9% AMR, 4% CAV and 2% PTLD. The best predictive score involved a simple MATE score sum, yielding a concordance probability estimate (CPE) statistic of 0.74. Whereas the power to detect non-inferiority (NI), assuming the NI hazard ratio of 1.45 in graft survival was 10% (assuming 200 subjects and 6% graft loss rate), the power to detect NI assuming a 2-point non-inferiority margin was >85% using the MATE score.The MATE score reflects the cumulative burden of MATEs and has acceptable prediction characteristics for death/graft loss post-HT. The MATE score may be useful as a surrogate end-point to power a clinical trial in pediatric HT.

View details for DOI 10.1016/j.healun.2017.03.013

View details for PubMedID 28465118

A multicenter study of the impella device for mechanical support of the systemic circulation in pediatric and adolescent patients. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Dimas, V. V., Morray, B. H., Kim, D. W., Almond, C. S., Shahanavaz, S., Tume, S. C., Peng, L. F., McElhinney, D. B., Justino, H. 2017


The objective was to review the use of Impella devices (Abiomed Inc, Danvers, MA) for temporary circulatory support in pediatric and adolescent patients (age21 yrs).Options for minimally invasive circulatory support in children are limited, and published data are confined to case reports and small case series.This was a retrospective, multicenter review of Impella implants in pediatric and adolescent patients from 2009-15, using standardized data collection and INTERMACS definitions.A total of 39 implants were performed in 38 patients from 16 centers. Median age and weight were 16 yrs (4-21 yrs) and 62 kg (15-134 kg). The primary indication for implant was cardiogenic shock in 28 patients (72%). Cardiac allograft rejection, myocarditis, or cardiomyopathy were the underlying diagnosis in 23 patients (59%); 11 patients had congenital heart disease. The median duration of support was 45 hr (1-1224 hr). Indications for explant included ventricular recovery in 16 patients, transition to another device in 12, death in 5, and transplant in 1. Survival was 85% at 7 days and 68% at 30 days. Major adverse events occurred in 8 patients: hemolysis in 3, bleeding in 2, stroke in 1 (unclear if related to Impella), sepsis in 1, and critical leg ischemia in 1. An increase in aortic regurgitation was noted in three patients, with no evidence of valve injury.Temporary circulatory support with Impella devices is feasible in pediatric and adolescent patients, with acceptable risk profiles. More experience and follow up is needed to improve technical performance and patient selection. 2017 Wiley Periodicals, Inc.

View details for DOI 10.1002/ccd.26973

View details for PubMedID 28295963

Functional status of United States children supported with a left ventricular assist device at heart transplantation. journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Bulic, A., Maeda, K., Zhang, Y., Chen, S., McElhinney, D. B., Dykes, J. C., Hollander, A. M., Hollander, S. A., Murray, J., Reinhartz, O., Gowan, M. A., Rosenthal, D. N., Almond, C. S. 2017


As survival with pediatric left ventricular assist devices (LVADs) has improved, decisions regarding the optimal support strategy may depend more on quality of life and functional status (FS) rather than mortality alone. Limited data are available regarding the FS of children supported with LVADs. We sought to compare the FS of children supported with LVADs vs vasoactive infusions to inform decision making around support strategies.Organ Procurement and Transplant Network data were used to identify all United States children aged between 1 and 21 years at heart transplant (HT) between 2006 and 2015 for dilated cardiomyopathy and supported with an LVAD or vasoactive infusions alone at HT. FS was measured using the 10-point Karnofsky and Lansky scale.Of 701 children who met the inclusion criteria, 430 (61%) were supported with vasoactive infusions, and 271 (39%) were supported with an LVAD at HT. Children in the LVAD group had higher median FS scores at HT than children in the vasoactive infusion group (6 vs 5, p < 0.001) but lower FS scores at listing (4 vs 6, p < 0.001). The effect persisted regardless of patient location at HT (home, hospital, intensive care) or device type. Discharge by HT occurred in 46% of children in the LVAD group compared with 26% of children in the vasoactive infusion cohort (p = 0.001). Stroke was reported at HT in 3% of children in the LVAD cohort and in 1% in the vasoactive infusion cohort (p = 0.04).Among children with dilated cardiomyopathy undergoing HT, children supported with LVADs at HT have higher FS than children supported with vasoactive infusions at HT, regardless of device type or hospitalization status. Children supported with LVADs at HT were more likely to be discharged from the hospital but had a higher prevalence of stroke at HT.

View details for DOI 10.1016/j.healun.2017.02.024

View details for PubMedID 28363739

Transcatheter valve implantation for right atrium-to-right ventricle conduit obstruction or regurgitation after modified Bjrk-fontan procedure. Catheterization and cardiovascular interventions Shah, A. H., Horlick, E. M., Eicken, A., Asnes, J. D., Bocks, M. L., Boudjemline, Y., Cabalka, A. K., Fagan, T. E., Schubert, S., Mahadevan, V. S., Dvir, D., Osten, M., McElhinney, D. B. 2017; 89 (2): 298-305


The most common reason for reintervention after a Bjrk modification of the Fontan procedure, in which the right ventricle (RV) is incorporated into the pulmonary circulation by connecting the right atrial (RA) appendage to the RV directly or with an extra-anatomic graft, is obstruction or regurgitation of the RA-RV connection. Transcatheter implantation of a valved stent is an appealing option for the treatment of RA-RV conduit dysfunction in these patients. In the present study, we assessed early and intermediate results after transcatheter valve implantation within an obstructed or regurgitant RA-RV modified Fontan pathway.Through a retrospective multicenter registry, we collected data from 16 patients with a modified Fontan circulation who were treated with percutaneous Melody or Sapien valve implantation for dysfunction of an extra-anatomic RA-RV conduit or valve.All patients had successful and uncomplicated implantation of a Melody (n=15) or Sapien 3 (n=1) valve with hemodynamic and, in most cases, clinical improvement. During a median follow-up of 3.3 years, 3 patients died of cardiovascular causes unrelated to the procedure or the valve, and no major valve dysfunction was observed.Percutaneous transcatheter valve deployment to treat a dysfunctional RA-RV connection after a Bjrk modification of the Fontan procedure is a viable alternative to surgery, with low procedural risk, and appears to offer good early and intermediate results. 2016 Wiley Periodicals, Inc.

View details for DOI 10.1002/ccd.26648

View details for PubMedID 27465501

Reflection and Rationalization: Making Sense of the Literature on Endocarditis After Transcatheter Pulmonary Valve Replacement. Circulation. Cardiovascular interventions McElhinney, D. B. 2017; 10 (2)
Rehospitalization after pediatric heart transplantation: Incidence, indications, and outcomes. Pediatric transplantation Hollander, S. A., McElhinney, D. B., Almond, C. S., McDonald, N., Chen, S., Kaufman, B. D., Bernstein, D., Rosenthal, D. N. 2017; 21 (1)


We report the patterns of rehospitalization after pediatric heart transplant (Htx) at a single center. Retrospective review of 107 consecutive pediatric Htx recipients between January 22, 2007, and August 28, 2014, who survived their initial transplant hospitalization. The frequency, duration, and indications for all hospitalizations between transplant hospitalization discharge and September 30, 2015, were analyzed. A total of 444 hospitalization episodes occurred in 90 of 107 (84%) patients. The median time to first rehospitalization was 59.5 (range 1-1526) days, and the median length of stay was 2.5 (range 0-81) days. There were an average of two hospitalizations per patient in the first year following transplant hospitalization, declining to about 0.8 per patient per year starting at 3years post-transplant. Admissions for viral infections were most common, occurring in 93 of 386 (24%), followed by rule out sepsis in 61 of 386 (16%). Admissions for suspected or confirmed rejection were less frequent, accounting for 41 of 386 (11%) and 31 of 386 (8%) of all admissions, respectively. Survival to discharge after rehospitalization was 97%. Hospitalization is common after pediatric Htx, particularly in the first post-transplant year, with the most frequent indications for hospitalization being viral illness and rule out sepsis. After the first post-transplant year, the risk for readmission falls significantly but remains constant for several years.

View details for DOI 10.1111/petr.12857

View details for PubMedID 27891727

Circulatory support using the impella device in fontan patients with systemic ventricular dysfunction: A multicenter experience. Catheterization and cardiovascular interventions Morray, B. H., Dimas, V. V., Lim, S., Balzer, D. T., Parekh, D. R., Van Mieghem, N. M., Ewert, P., Kim, D. W., Justino, H., McElhinney, D. B., Jones, T. K. 2017


There are limited mechanical circulatory support options for patients with single ventricle (SV) anatomy. This is a multicenter, retrospective study of the Impella pump to support the systemic ventricle in a cohort of SV patients with Fontan circulation.Patients with SV anatomy supported with an Impella device from 2012 to 2015 were included. Demographic information, indication for support, adverse events and short-term outcome data were collected.Ten patients were included. The median age and weight at implant was 26 years (4-38 years) and 64 kg (15-102 kg). Indications for support were systemic ventricular failure with cardiogenic shock (n=8) or high-risk electrophysiology (EP) procedures (n=2). The median duration of support was 49 hr (2.7-264 hr). Support was discontinued for ventricular recovery in five patients, transition to another device in two patients, completion of EP procedure in two patients and death in one patient. Survival to hospital discharge was 80%. Adverse events occurred in 4 patients. There were two cases of hemolysis, one case of increasing aortic valve insufficiency with implant and one asymptomatic access site thrombus. There were no bleeding or thromboembolic events.Impella devices can provide temporary support for the systemic ventricle in SV patients as a bridge to recovery or additional device. Procedural survival and adverse event profiles are favorable. 2017 Wiley Periodicals, Inc.

View details for DOI 10.1002/ccd.26885

View details for PubMedID 28112463

Transcatheter Valve-in-Ring Implantation for the Treatment of Residual or Recurrent Tricuspid Valve Dysfunction After Prior Surgical Repair JACC-CARDIOVASCULAR INTERVENTIONS Aboulhosn, J., Cabalka, A. K., Levi, D. S., Himbert, D., Testa, L., Latib, A., Makkar, R. R., Boudjemline, Y., Kim, D. W., Kefer, J., Bleiziffer, S., Kerst, G., Dvir, D., McElhinney, D. B. 2017; 10 (1): 53-63


This study sought to describe the results of transcatheter tricuspid valve-in-ring (TVIR) implantation for treatment of tricuspid regurgitation (TR).Off-label use of transcatheter valves within surgically placed tricuspid annuloplasty prostheses has only been described in small reports. An international multicenter registry was developed to collect data on TVIR implantation.Data were collected from 13 sites on 22 patients (5 to 69 years of age) with TR who underwent catheterization with the intent to perform TVIR implantation.TVIR implantation was performed in 20 patients (91%). Most patients were severely impaired (86% in NewYork Heart Association functional class III or IV); TR was severe in 86%. A Sapien valve (Edwards Lifesciences, Irvine, California) was implanted in 17 patients and a Melody valve (Medtronic, Minneapolis, Minnesota) in 3. There were no procedural deaths. There was 1 valve embolization requiring retrieval and placement of second TVIR implant and 1 valve malposition with severe paravalvular regurgitation requiring a second TVIR implantation. Over a median follow-up of 12months, 1 patient died and 2 underwent repeat TVIR implantation, 1 of whom subsequently underwent surgical valve replacement. Significant paravalvular leak (PVL) was treated at the time of TVIR implantation in 4 patients: 3 underwent device occlusion and 1 received a second TVIR implant. On follow-up echocardiography, 15 patients had PVL (75%), the majority of which (n= 10) were trivial or mild and did not require treatment. PVL intervention was performed in 3 patients during follow-up. Functional capacity improved in most patients (70%).TVIR implantation using commercially available transcatheter prostheses is technically feasible and clinically effective in reducing TR. Paravalvular regurgitation is common and may necessitate further interventions.

View details for DOI 10.1016/j.jcin.2016.10.036

View details for Web of Science ID 000392624100010

View details for PubMedID 28057286

Risk Estimates for Atherosclerotic Cardiovascular Disease in Adults With Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Lui, G. K., Rogers, I. S., Ding, V. Y., Hedlin, H. K., MacMillen, K., Maron, D. J., Sillman, C., Romfh, A., Dade, T. C., Haeffele, C., Grady, S. R., McElhinney, D. B., Murphy, D. J., Fernandes, S. M. 2017; 119 (1): 112-118


The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using 3 validated risk assessment tools-the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the ASCVD Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1 12.6years, 51% men. At least 1 modifiable ASCVD risk factor was present in 70%; the 2 most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data were available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein were each found in around 30% of patients. The 10-year ASCVD predicted risk using all 3 tools was relatively low (i.e., at least 90% of patients <10% risk), yet the median estimated lifetime risk was 36%. In conclusion, ASCVD risk factors are prevalent in adults with CHD. The risk estimation tools suggest that this population is particularly vulnerable to ASCVD with aging and should undergo guideline-based screening and management of modifiable risk factors.

View details for DOI 10.1016/j.amjcard.2016.09.023

View details for Web of Science ID 000391246900018

View details for PubMedID 28247847

View details for PubMedCentralID PMC5334785

Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience JOURNAL OF PEDIATRICS Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Tierney, E. S. 2017; 180: 87-?
Utility and Scope of Rapid Prototyping in Patients with Complex Muscular Ventricular Septal Defects or Double-Outlet Right Ventricle: Does it Alter Management Decisions? Pediatric cardiology Bhatla, P., Tretter, J. T., Ludomirsky, A., Argilla, M., Latson, L. A., Chakravarti, S., Barker, P. C., Yoo, S., McElhinney, D. B., Wake, N., Mosca, R. S. 2017; 38 (1): 103-114


Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV). Six patients with complex anatomy and uncertainty of the optimal management strategy were included in this study. The models were subsequently used to guide management decisions, and the outcomes reviewed. 3D models clearly demonstrated the complex intra-cardiac anatomy in all six patients and were utilized to guide management decisions. In the three patients with CMVSD, one underwent successful endovascular device closure following a prior failed attempt at transcatheter closure, and the other two underwent successful primary surgical closure with the aid of 3D models. In all three cases of DORV, the models provided better anatomic delineation and additional information that altered or confirmed the surgical plan. Patient-specific 3D heart models show promise in accurately defining intra-cardiac anatomy in CHD, specifically CMVSD and DORV. We believe these models improve understanding of the complex anatomical spatial relationships in these defects and provide additional insight for pre/intra-interventional management and surgical planning.

View details for DOI 10.1007/s00246-016-1489-1

View details for PubMedID 27837304

Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia AMERICAN JOURNAL OF CARDIOLOGY Tierney, E. S., McElhinney, D. B., Freud, L. R., Tworetzky, W., Cuneo, B. F., Escobar-Diaz, M. C., Ikemba, C., Kalish, B. T., Komarlu, R., Levasseur, S. M., Puchalski, M. D., Satou, G. M., Silverman, N. H., Moon-Grady, A. J. 2017; 119 (1): 106-111
Unique Molecular Patterns Uncovered in Kawasaki Disease Patients with Elevated Serum Gamma Glutamyl Transferase Levels: Implications for Intravenous Immunoglobulin Responsiveness PLOS ONE Wang, Y., Li, Z., Hu, G., Hao, S., Deng, X., Huang, M., Ren, M., Jiang, X., Kanegaye, J. T., Ha, K., Lee, J., Li, X., Jiang, X., Yu, Y., Tremoulet, A. H., Burns, J. C., Whitin, J. C., Shin, A. Y., Sylvester, K. G., McElhinney, D. B., Cohen, H. J., Ling, X. B. 2016; 11 (12)


Resistance to intravenous immunoglobulin (IVIG) occurs in 10-20% of patients with Kawasaki disease (KD). The risk of resistance is about two-fold higher in patients with elevated gamma glutamyl transferase (GGT) levels. We sought to understand the biological mechanisms underlying IVIG resistance in patients with elevated GGT levels.We explored the association between elevated GGT levels and IVIG-resistance with a cohort of 686 KD patients (Cohort I). Gene expression data from 130 children with acute KD (Cohort II) were analyzed using the R square statistic and false discovery analysis to identify genes that were differentially represented in patients with elevated GGT levels with regard to IVIG responsiveness. Two additional KD cohorts (Cohort III and IV) were used to test the hypothesis that sialylation and GGT may be involved in IVIG resistance through neutrophil apoptosis.Thirty-six genes were identified that significantly explained the variations of both GGT levels and IVIG responsiveness in KD patients. After Bonferroni correction, significant associations with IVIG resistance persisted for 12 out of 36 genes among patients with elevated GGT levels and none among patients with normal GGT levels. With the discovery of ST6GALNAC3, a sialyltransferase, as the most differentially expressed gene, we hypothesized that sialylation and GGT are involved in IVIG resistance through neutrophil apoptosis. We then confirmed that in Cohort III and IV there was significantly less reduction in neutrophil count in IVIG non-responders.Gene expression analyses combining molecular and clinical datasets support the hypotheses that: (1) neutrophil apoptosis induced by IVIG may be a mechanism of action of IVIG in KD; (2) changes in sialylation and GGT level in KD patients may contribute synergistically to IVIG resistance through blocking IVIG-induced neutrophil apoptosis. These findings have implications for understanding the mechanism of action in IVIG resistance, and possibly for development of novel therapeutics.

View details for DOI 10.1371/journal.pone.0167434

View details for Web of Science ID 000392853100008

View details for PubMedID 28002448

View details for PubMedCentralID PMC5176264

Web-based Real-Time Case Finding for the Population Health Management of Patients With Diabetes Mellitus: A Prospective Validation of the Natural Language Processing-Based Algorithm With Statewide Electronic Medical Records. JMIR medical informatics Zheng, L., Wang, Y., Hao, S., Shin, A. Y., Jin, B., Ngo, A. D., Jackson-Browne, M. S., Feller, D. J., Fu, T., Zhang, K., Zhou, X., Zhu, C., Dai, D., Yu, Y., Zheng, G., Li, Y., McElhinney, D. B., Culver, D. S., Alfreds, S. T., Stearns, F., Sylvester, K. G., Widen, E., Ling, X. B. 2016; 4 (4)


Diabetes case finding based on structured medical records does not fully identify diabetic patients whose medical histories related to diabetes are available in the form of free text. Manual chart reviews have been used but involve high labor costs and long latency.This study developed and tested a Web-based diabetes case finding algorithm using both structured and unstructured electronic medical records (EMRs).This study was based on the health information exchange (HIE) EMR database that covers almost all health facilities in the state of Maine, United States. Using narrative clinical notes, a Web-based natural language processing (NLP) case finding algorithm was retrospectively (July 1, 2012, to June 30, 2013) developed with a random subset of HIE-associated facilities, which was then blind tested with the remaining facilities. The NLP-based algorithm was subsequently integrated into the HIE database and validated prospectively (July 1, 2013, to June 30, 2014).Of the 935,891 patients in the prospective cohort, 64,168 diabetes cases were identified using diagnosis codes alone. Our NLP-based case finding algorithm prospectively found an additional 5756 uncodified cases (5756/64,168, 8.97% increase) with a positive predictive value of .90. Of the 21,720 diabetic patients identified by both methods, 6616 patients (6616/21,720, 30.46%) were identified by the NLP-based algorithm before a diabetes diagnosis was noted in the structured EMR (mean time difference = 48 days).The online NLP algorithm was effective in identifying uncodified diabetes cases in real time, leading to a significant improvement in diabetes case finding. The successful integration of the NLP-based case finding algorithm into the Maine HIE database indicates a strong potential for application of this novel method to achieve a more complete ascertainment of diagnoses of diabetes mellitus.

View details for PubMedID 27836816

View details for PubMedCentralID PMC5124114

Subacute left ventricular outflow tract obstruction after transapical closure of a mitral paravalvular leak in the region of the aortomitral curtain ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Chikkabyrappa, S., McElhinney, D. B., Saric, M. 2016; 33 (11): 1771-1776


We report a rare case of progressive left ventricular outflow tract (LVOT) obstruction after percutaneous device closure of a mechanical prosthetic mitral valve (MV) paravalvular leak (PVL) in the region of aortomitral curtain in a patient who also had small mechanical aortic valve prosthesis with patient-prosthesis mismatch.

View details for DOI 10.1111/echo.13357

View details for Web of Science ID 000388309500021

View details for PubMedID 27576330

Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience. journal of pediatrics Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Selamet Tierney, E. S. 2016


To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had 3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.51.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P=.12). Patients with 3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P=.001), longer CVICU lengths of stay (22 vs 3 days; P<.0001), longer overall hospitalizations (28 vs 7 days; P<.0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P<.0001) than those with <3 TTEs. Eight patients with 3 TTEs did not survive, compared with 3 with <3 TTEs (P=.0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

View details for DOI 10.1016/j.jpeds.2016.09.061

View details for PubMedID 28029346

Defining and refining indications for transcatheter pulmonary valve replacement in patients with repaired tetralogy of Fallot: Contributions from anatomical and functional imaging. International journal of cardiology Tretter, J. T., Friedberg, M. K., Wald, R. M., McElhinney, D. B. 2016; 221: 916-925


Transcatheter pulmonary valve replacement (TPVR) is an important treatment option in repaired tetralogy of Fallot (TOF) and right ventricular outflow tract (RVOT) dysfunction. Indications for timing of TPVR are extrapolated from surgical pulmonary valve replacement guidelines, which are themselves controversial as published evidence is scarce and expert opinion therefore prevails. We review current indications for PVR following TOF repair, focusing on those for TPVR specifically, and discuss anatomical and functional considerations as these pertain to determination of candidacy for TPVR. Hemodynamic assessment surrounding PVR has focused on assessment of the right ventricle (RV) size and systolic function, with the goal of intervening in the asymptomatic patient prior to the development of irreversible RV deterioration and right heart failure. The impact of abnormal RV mechanics on the LV has been appreciated, with the assessment of LV function assuming higher priority in decision-making regarding possible PVR. In addition to the standard volumetric assessment, evaluation with indices of myocardial wall strain, tissue velocities, diastology, and ventricular response to exercise is emerging as tools with potential to further refine timing of PVR. We conclude that, at present, current evidence, although limited, supports a more aggressive approach in those who meet inclusion for TPVR in patients with repaired TOF and RVOT dysfunction guided by the discussed hemodynamic assessment, however, more importantly this review should lay the framework for future investigations regarding hemodynamic assessment of this population.

View details for DOI 10.1016/j.ijcard.2016.07.120

View details for PubMedID 27441469

Transcatheter Pulmonary Valve Replacement Reduces Tricuspid Regurgitation in Patients With Right Ventricular Volume/Pressure Overload. Journal of the American College of Cardiology Jones, T. K., Rome, J. J., Armstrong, A. K., Berger, F., Hellenbrand, W. E., Cabalka, A. K., Benson, L. N., Balzer, D. T., Cheatham, J. P., Eicken, A., McElhinney, D. B. 2016; 68 (14): 1525-1535


Tricuspid regurgitation (TR) is a common and important comorbidity in patients with postoperative right ventricular outflow tract (RVOT) obstruction or pulmonary regurgitation (PR). Transcatheter pulmonary valve replacement (TPVR) has become a useful tool in the management of postoperative RVOT obstruction and PR, but it is unknown whether relief of the right ventricular volume and/or pressure overload by TPVR will have a beneficial effect on TR, as is often seen with surgical pulmonary valve replacement.This study sought to assess the prevalence of and factors associated with significant TR in patients undergoing TPVR for RVOT obstruction or PR.Data were combined from 3 prospective multicenter trials of patients referred for TPVR. Follow-up data through 5 years post-implantation were analyzed.Of 300 patients studied, 77 (25.6%) had moderate or severe TR at baseline. After TPVR, TR severity was improved in 65% of those patients, and more than one-half had mild TR or less TR at discharge. Of 13 patients with severe TR pre-implantation, only 1 had severe TR at 1-year follow-up and beyond. Moderate or severe baseline TR was associated with shorter freedom from RVOT reintervention after TPVR.In this prospective multicenter study of post-operative patients with RVOT obstruction and/or PR, TR was common. In patients with significant baseline TR, TPVR resulted in clinically relevant acute reductions in TR that persisted over at least 5 years of follow-up. These observations support the application of TPVR therapy in patients with RVOT obstruction or PR who are anatomically suitable, even in the setting of significant concomitant TR.

View details for DOI 10.1016/j.jacc.2016.07.734

View details for PubMedID 27687194

Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia. American journal of cardiology Selamet Tierney, E. S., McElhinney, D. B., Freud, L. R., Tworetzky, W., Cuneo, B. F., Escobar-Diaz, M. C., Ikemba, C., Kalish, B. T., Komarlu, R., Levasseur, S. M., Puchalski, M. D., Satou, G. M., Silverman, N. H., Moon-Grady, A. J. 2016


In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24weeks of gestation with EA/TVD and serial fetal echocardiograms 4weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.

View details for DOI 10.1016/j.amjcard.2016.09.022

View details for PubMedID 27793395

Progressive intermediate-term improvement in ventricular and atrioventricular interaction after transcatheter pulmonary valve replacement in patients with right ventricular outflow tract obstruction. American heart journal Lunze, F. I., Hasan, B. S., Gauvreau, K., Brown, D. W., Colan, S. D., McElhinney, D. B. 2016; 179: 87-98


Relief of postoperative right ventricular outflow tract (RVOT) obstruction with transcatheter pulmonary valve replacement (TPVR) results in functional improvement in the short term which we investigated at baseline (BL), early follow-up (FU), and midterm FU after TPVR.Echocardiography and cardiopulmonary exercise testing were performed at BL and at early (median 6 months) and midterm FU (median 2.5years) after TPVR.Patients with RVOT obstruction (n=22, median age 17years) were studied. The max RVOT Doppler gradient fell from BL to early FU (6024 to 268mm Hg, P<.001). Left ventricular (LV) end-diastolic and stroke volume increased at early FU (both P<.001) without further change, whereas LV ejection fraction improved throughout FU (P<.001). LV end-systolic and diastolic eccentricity (leftward septal displacement) improved early (both P.003), and end-diastolic eccentricity improved further at midterm FU (P=.02). Furthermore, whereas mitral inflow A wave velocity increased (P=.003), the LV A' velocity declined early (P=.007) without further change at midterm. RV systolic and early diastolic function was impaired at BL. Whereas RV strain improved partially at early and midterm FU (P.02), RV E' velocity did not improve throughout FU. Mildly impaired LV strain at BL fully recovered by midterm FU (P.002). Peak oxygen uptake improved at early and midterm FU (all P.003).Patients with RVOT obstruction had biventricular systolic and diastolic dysfunction at BL. Relieving RVOT obstruction with TPVR reduced adverse ventricular and compensatory atrioventricular interaction, resulting in progressive biventricular functional improvement and remodeling at early and midterm FU.

View details for DOI 10.1016/j.ahj.2016.05.011

View details for PubMedID 27595683

Identifying Gaps in Technology for Congenital Interventions: Analysis of a Needs Survey from Congenital Interventional Cardiologists PEDIATRIC CARDIOLOGY Shibbani, K., Kenny, D., McElhinney, D., Hijazi, Z. M., Moran, T. 2016; 37 (5): 925-931


We carried out a device-needs survey to evaluate the gaps in device and equipment availability for congenital interventional cardiologists. As the complexity and demand for more complete solutions to congenital heart lesions increase, there is a growing need for modification and development of devices and equipment to support this endeavor. The survey was sent out via e-mail to members of the Congenital Cardiovascular Interventional Study Consortium and the Society for Cardiac Angiography and Interventions with a reach of over 350 congenital interventionalists. Responses were received from 68 cardiologists in 8 countries. In terms of the most desired device, 41% ranked bioresorbable stents as their first choice from a list of 12 possible devices. Similarly, 23% ranked large covered stents as their first choice. Twenty-seven percent of participants believed bioresorbable stents would have the greatest potential to improve morbidity of their patients, with another 27% reporting that covered stents would have the greatest impact. Fifty percent of participants reported that they would like to see large covered stents available in their country. These data point toward a perceived need for the development/approval of bioresorbable stents for the pediatric age group, as well as the need to approve the use of large covered stents in the pediatric age group in the USA.

View details for DOI 10.1007/s00246-016-1372-0

View details for Web of Science ID 000377722400016

View details for PubMedID 27064094

Postoperative Outcomes of Children With Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals Undergoing Reconstruction of Occluded Pulmonary Artery Branches ANNALS OF THORACIC SURGERY Asija, R., Koth, A. M., Velasquez, N., Chan, F. P., Perry, S. B., Hanley, F. L., McElhinney, D. 2016; 101 (6): 2329-2334


Patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for prolonged postoperative respiratory failure. We sought to understand whether patients undergoing reconstruction and incorporation of occluded pulmonary arterial branches were at risk for worse postoperative outcomes.We performed a retrospective chart review to identify patients who underwent unifocalization or unifocalization revision with incorporation of occluded pulmonary artery branches. Patients with and without occluded branches were compared, with a focus on clinical outcomes.We studied 92 patients who underwent unifocalization procedures between 2010 and 2014, 17 (18%) of whom underwent reconstruction of occluded pulmonary artery branches. Patients with occluded vessels were more likely to require staged unifocalization procedures, although more than two thirds of this cohort eventually underwent complete intracardiac repair. Durations of mechanical ventilation, intensive care, hospital stay, and the need for early reoperation were similar between the two groups.Occluded pulmonary arterial branches can be safely recruited into the pulmonary vasculature in patients with TOF/PA/MAPCAs without a significant difference in postoperative outcomes compared with patients who did not have an occluded branch. Incorporation of occluded branches may also facilitate ultimate complete intracardiac repair in this complex population of patients.

View details for DOI 10.1016/j.athoracsur.2015.12.049

View details for Web of Science ID 000376502600048

View details for PubMedID 26947013

Relative Risk Factors for Cardiac Erosion Following Transcatheter Closure of Atrial Septal Defects A Case-Control Study CIRCULATION McElhinney, D. B., Quartermain, M. D., Kenny, D., Alboliras, E., Amin, Z. 2016; 133 (18): 1738-?


Transcatheter closure of secundum atrial septal defects (ASD) using the Amplatzer septal occluder is generally safe and effective, but erosion into the pericardial space or aorta has been described. Although the absolute risk of this complication is low, there has been no assessment of relative risk factors.All erosions reported to St. Jude Medical after ASD closure with an Amplatzer septal occluder (cases) were compared with controls (matched 2:1) who underwent ASD closure but did not develop an erosion. A total of 125 erosions were reported between 2002 and 2014, including 95 with an available echocardiogram. The median duration from implant to erosion was 14 days, but was >1 year in 16 patients. Nine patients (all age 17 years) who died were more likely to have an oversized device, and to have erosion into the aorta, than survivors. Aortic or superior vena cava rim deficiencies were more common in cases than in controls. In addition, larger balloon-sized ASD diameter, Amplatzer septal occluder device size, and device size-ASD diameter difference, and smaller weight:device size ratio were associated with erosion. On multivariable analysis, deficiency of any rim, device >5 mm larger than ASD diameter, and weight:device size ratio were associated with erosion.In addition to aortic rim deficiency, which was almost universal among erosion cases, there were several relative risk factors for erosion after ASD closure with the Amplatzer septal occluder device. To understand the mechanisms of and absolute risk factors for this uncommon but serious complication, an adequately powered prospective study with thorough echocardiographic evaluation will be critical.

View details for DOI 10.1161/CIRCULATIONAHA.115.019987

View details for Web of Science ID 000375604400004

View details for PubMedID 27002094

Transcatheter Tricuspid Valve-in-Valve Implantation for the Treatment of Dysfunctional Surgical Bioprosthetic Valves An International, Multicenter Registry Study CIRCULATION McElhinney, D. B., Cabalka, A. K., AboulHosn, J. A., Eicken, A., Boudjemline, Y., Schubert, S., Himbert, D., Asnes, J. D., Salizzoni, S., Bocks, M. L., Cheatham, J. P., Momenah, T. S., Kim, D. W., Schranz, D., Meadows, J., Thomson, J. D., Goldstein, B. H., Crittendon, I., Fagan, T. E., Webb, J. G., Horlick, E., Delaney, J. W., Jones, T. K., Shahanavaz, S., Moretti, C., Hainstock, M. R., Kenny, D. P., Berger, F., Rihal, C. S., Dvir, D. 2016; 133 (16): 1582-?


Off-label use of transcatheter aortic and pulmonary valve prostheses for tricuspid valve-in-valve implantation (TVIV) within dysfunctional surgical tricuspid valve (TV) bioprostheses has been described in small reports.An international, multicenter registry was developed to collect data on TVIV cases. Patient-related factors, procedural details and outcomes, and follow-up data were analyzed. Valve-in-ring or heterotopic TV implantation procedures were not included. Data were collected on 156 patients with bioprosthetic TV dysfunction who underwent catheterization with planned TVIV. The median age was 40 years, and 71% of patients were in New York Heart Association class III or IV. Among 152 patients in whom TVIV was attempted with a Melody (n=94) or Sapien (n=58) valve, implantation was successful in 150, with few serious complications. After TVIV, both the TV inflow gradient and tricuspid regurgitation grade improved significantly. During follow-up (median, 13.3 months), 22 patients died, 5 within 30 days; all 22 patients were in New York Heart Association class III or IV, and 9 were hospitalized before TVIV. There were 10 TV reinterventions, and 3 other patients had significant recurrent TV dysfunction. At follow-up, 77% of patients were in New York Heart Association class I or II (P<0.001 versus before TVIV). Outcomes did not differ according to surgical valve size or TVIV valve type.TVIV with commercially available transcatheter prostheses is technically and clinically successful in patients of various ages across a wide range of valve size. Although preimplantation clinical status was associated with outcome, many patients in New York Heart Association class III or IV at baseline improved. TVIV should be considered a viable option for treatment of failing TV bioprostheses.

View details for DOI 10.1161/CIRCULATIONAHA.115.019353

View details for Web of Science ID 000374553400013

View details for PubMedID 26994123

Intentional Fracture of Maximally Dilated Balloon-Expandable Pulmonary Artery Stents Using Ultra-High-Pressure Balloon Angioplasty: A Preliminary Analysis. Circulation. Cardiovascular interventions Morray, B. H., McElhinney, D. B., Marshall, A. C., Porras, D. 2016; 9 (4)


Treatment with endovascular stents has become increasingly common for the management of vascular stenosis in congenital heart disease. The use of stents in smaller patients has been tempered by concerns about the potential for stent expansion to accommodate somatic growth. One solution to limited stent diameter is the intentional fracture of maximally dilated stents, which can be accomplished using ultra-high-pressure (UHP) balloons.This retrospective cohort study compared procedural characteristics and adverse events between a cohort of patients with branch pulmonary artery (PA) stents who underwent stent fracture using UHP balloons and control patients who underwent UHP redilation of previously placed PA stents without stent fracture between 2004 and 2014. Two control patients were selected for every case. Thirty-three PA stents were fractured in 31 patients with a median of 10 years after initial stent placement. The median balloon:waist ratio was 1.17 (1-1.71), and the median inflation pressure was 20 (8-30) atm. There were significant reductions in pressure gradient after angioplasty, with no difference in postangioplasty gradients between cases and controls. There were no major PA complications in the stent fracture group and no difference in the number of adverse events between the 2 groups.In this small series, PA stent fracture using UHP balloon angioplasty was feasible and did not result in major complications although predictors of successful fracture were not identified. Intentional fracture with UHP balloon angioplasty may be considered when treating stents that have become restrictive despite maximal dilation.

View details for DOI 10.1161/CIRCINTERVENTIONS.115.003281

View details for PubMedID 27059684

Aortic Root Distortion and Aortic Insufficiency During Balloon Angioplasty of the Right Ventricular Outflow Tract Prior to Transcatheter Pulmonary Valve Replacement. Journal of interventional cardiology Torres, A. J., McElhinney, D. B., Anderson, B. R., Turner, M. E., Crystal, M. A., Timchak, D. M., Vincent, J. A. 2016; 29 (2): 197-207


To describe the significance of aortic root distortion (AD) and/or aortic valve insufficiency (AI) during balloon angioplasty of the right ventricular outflow tract (RVOT) performed to rule out coronary artery compression prior to transcatheter pulmonary valve (TPV) implantation.AD/AI was assessed by retrospective review of all procedural aortographies performed to evaluate coronary anatomy prior to TPV implantation. AD/AI was also reviewed in all pre-post MPV implant echocardiograms to assess for progression.From 04/2007 to 3/2015, 118pts underwent catheterization with intent for TPV implant. Mean age and weight were 24.512 years and 64.320kg, respectively. Diagnoses were: TOF (53%), D-TGA/DORV (18%), s/p Ross (15%), and Truncus (9%). Types of RV-PA connections were: conduits (96), bioprosthetic valves (14), and other (7). Successful TPV implant occurred in 91pts (77%). RVOT balloon angioplasty was performed in 43/118pts (36%). Aortography was performed in 18/43pts with AD/AI noted in 6/18 (33%); 2 with D-TGA (1s/p Lecompte, 1s/p Rastelli), 2 with TOF, 1 Truncus and 1s/p Ross. Procedure was aborted in the 2 who developed severe AD/AI. TPV was implanted in 3/4 patients with mild AD/AI. Review of pre-post TPV implantation echocardiograms in 83/91pts (91%) revealed no new/worsened AI in any patient.AD/AI is relatively common on aortography during simultaneous RVOT balloon angioplasty. Lack of AI progression by echocardiography post-TPV implant suggests these may be benign findings in most cases. However, AD/AI should be carefully evaluated in certain anatomic subtypes with close RVOT/aortic alignments. (J Interven Cardiol 2016;29:197-207).

View details for DOI 10.1111/joic.12270

View details for PubMedID 26822282

Exploring the Role of Polycythemia in Patients With Cyanosis After Palliative Congenital Heart Surgery. Pediatric critical care medicine Siehr, S. L., Shi, S., Hao, S., Hu, Z., Jin, B., Hanley, F., Reddy, V. M., McElhinney, D. B., Ling, X. B., Shin, A. Y. 2016; 17 (3): 216-222


To understand the relationship between polycythemia and clinical outcome in patients with hypoplastic left heart syndrome following the Norwood operation.A retrospective, single-center cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Infants with hypoplastic left heart syndrome admitted to our medical center from September 2009 to December 2012 undergoing stage 1/Norwood operation.None.Baseline demographic and clinical information including first recorded postoperative hematocrit and subsequent mean, median, and nadir hematocrits during the first 72 hours postoperatively were recorded. The primary outcomes were in-hospital mortality and length of hospitalization. Thirty-two patients were included in the analysis. Patients did not differ by operative factors (cardiopulmonary bypass time and cross-clamp time) or traditional markers of severity of illness (vasoactive inotrope score, lactate, saturation, and PaO2/FIO2 ratio). Early polycythemia (hematocrit value > 49%) was associated with longer cardiovascular ICU stay (51.0 [ 38.6] vs 21.4 [ 16.2] d; p < 0.01) and total hospital length of stay (65.0 [ 46.5] vs 36.1 [ 20.0] d; p = 0.03). In a multivariable analysis, polycythemia remained independently associated with the length of hospitalization after controlling for the amount of RBC transfusion (weight, 4.36 [95% CI, 1.35-7.37]; p < 0.01). No difference in in-hospital mortality rates was detected between the two groups (17.6% vs 20%).Early polycythemia following the Norwood operation is associated with longer length of hospitalization even after controlling for blood cell transfusion practices. We hypothesize that polycythemia may be caused by hemoconcentration and used as an early marker of capillary leak syndrome.

View details for DOI 10.1097/PCC.0000000000000654

View details for PubMedID 26825044

Exploring Value in Congenital Heart Disease: An Evaluation of Inpatient Admissions. Congenital heart disease Shin, A. Y., Hu, Z., Jin, B., Lal, S., Rosenthal, D. N., Efron, B., Sharek, P. J., Sutherland, S. M., Cohen, H. J., McElhinney, D. B., Roth, S. J., Ling, X. B. 2015; 10 (6): E278-87


Understanding value provides an important context for improvement. However, most health care models fail to measure value. Our objective was to categorize inpatient encounters within an academic congenital heart program based on clinical outcome and the cost to achieve the outcome (value). We aimed to describe clinical and nonclinical features associated with value.We defined hospital encounters based on outcome per resource utilized. We performed principal component and cluster analysis to classify encounters based on mortality, length of stay, hospital cost and revenue into six classes. We used nearest shrunken centroid to identify discriminant features associated with the cluster-derived classes. These features underwent hierarchical clustering and multivariate analysis to identify features associated with each class.We analyzed all patients admitted to an academic congenital heart program between September 1, 2009, and December 31, 2012.A total of 2658 encounters occurred during the study period. Six classes were categorized by value. Low-performing value classes were associated with greater institutional reward; however, encounters with higher-performing value were associated with a loss in profitability. Encounters that included insertion of a pediatric ventricular assist device (log OR 2.5 [95% CI, 1.78 to 3.43]) and acquisition of a hospital-acquired infection (log OR 1.42 [95% CI, 0.99 to 1.87]) were risk factors for inferior health care value.Among the patients in our study, institutional reward was not associated with value. We describe a framework to target quality improvement and resource management efforts that can benefit patients, institutions, and payers alike.

View details for DOI 10.1111/chd.12290

View details for PubMedID 26219731

Exploring Value in Congenital Heart Disease: An Evaluation of Inpatient Admissions CONGENITAL HEART DISEASE Shin, A. Y., Hu, Z., Jin, B., Lal, S., Rosenthal, D. N., Efron, B., Sharek, P. J., Sutherland, S. M., Cohen, H. J., McElhinney, D. B., Roth, S. J., Ling, X. B. 2015; 10 (6): E278-E287

View details for DOI 10.1111/chd.12290

View details for Web of Science ID 000367379300004

Iatrogenic aortopulmonary communications after transcatheter interventions on the right ventricular outflow tract or pulmonary artery: Pathophysiologic, diagnostic, and management considerations CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Torres, A., Sanders, S. P., Vincent, J. A., El-Said, H. G., Leahy, R. A., Padera, R. F., McElhinney, D. B. 2015; 86 (3): 438-452


To investigate the spectrum, etiology, and management of traumatic aortopulmonary (AP) communications after transcatheter interventions on the pulmonary circulation.An iatrogenic AP communication is an unusual complication after balloon pulmonary artery (PA) angioplasty or stenting, or transcatheter pulmonary valve replacement (TPVR). However, with the increasing application of transcatheter therapies for postoperative PA stenosis and right ventricular outflow tract (RVOT) dysfunction, including percutaneous pulmonary valve replacement, consideration of the etiology, diagnosis, and management of this problem is important for interventional cardiologists performing such procedures.We present three new cases, as well as gross anatomy and histopathology data, related to AP communications after PA interventions. We also review the literature relevant to this topic. Including these new cases, there have been 18 reported cases of iatrogenic AP communication after transcatheter interventions on the PAs or RVOT, primarily patients with transposition of the great arteries who underwent PA angioplasty after an arterial switch operation, or after TPVR in patients who had undergone a Ross procedure. The likely cause of such defects is PA trauma plus distortion of the neo-aortic anastomosis resulting from angioplasty or stenting of the RVOT or central PAs, with subsequent dissection through the extravascular connective tissue and into the closely adjacent vessel through the devitalized tissue at the anastomosis.Cardiologists performing PA or RVOT interventions should be aware of the possibility of a traumatic AP communication and consider this diagnosis when confronted with suggestive signs and symptoms. 2015 Wiley Periodicals, Inc.

View details for DOI 10.1002/ccd.25897

View details for Web of Science ID 000359811600022

View details for PubMedID 25676815

Aortic Wall Injury Related to Endovascular Therapy for Aortic Coarctation CIRCULATION-CARDIOVASCULAR INTERVENTIONS Tretter, J. T., Jones, T. K., McElhinney, D. B. 2015; 8 (9)
Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction After the Ross Procedure ANNALS OF THORACIC SURGERY Gillespie, M. J., McElhinney, D. B., Kreutzer, J., Hellenbrand, W. E., El-Said, H., Ewert, P., Rhodes, J. F., Sondergaard, L., Jones, T. K. 2015; 100 (3): 996-1003
Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction After the Ross Procedure. Annals of thoracic surgery Gillespie, M. J., McElhinney, D. B., Kreutzer, J., Hellenbrand, W. E., El-Said, H., Ewert, P., Rhodes, J. F., Sndergaard, L., Jones, T. K. 2015; 100 (3): 996-1002


Right ventricular outflow tract (RVOT) conduit dysfunction is a limitation of the Ross procedure. Transcatheter pulmonary valve replacement (TPVR) could alter the impact of conduit dysfunction and the risk-benefit balance for the Ross procedure.Retrospective review of databases from 3 prospective Melody TPV (Medtronic Inc, Minneapolis, MN) trials.Among 358 patients who were catheterized with the intent to implant a Melody TPV for RVOT conduit stenosis or regurgitation (PR) as part of 3 prospective multicenter studies, 67 (19%) had a prior Ross procedure. Of these, 56 (84%) received a Melody valve; in 5 of the 11 patients who did not, the implant was aborted due to concern for coronary artery compression, and 1 implanted patient required emergent surgery for left coronary compression. The RVOT gradient decreased from a median 38 mm Hg to 13.5 mm Hg (p < 0.001). There was no or trivial PR in all but 4 patients, in whom it was mild. At a median follow-up of 4.0 years, 1 patient died from sepsis. Twelve patients underwent 14 transcatheter (n= 8) or surgical (n= 6) TPV reinterventions for obstruction with stent fracture (n= 9), endocarditis with conduit obstruction (n= 3), or reoperation (n= 2). Freedom from TPV explant was 89% 5% at 4 years. Among patients who did not undergo reintervention for obstruction, there was no change in RVOT gradient over time, and all but 1 patient had mild or less PR at last follow-up.The TPVR with the Melody valve provides acceptable early outcomes and durable valve function in the majority of Ross patients. Recurrent RVOT obstruction associated with stent fracture was the main reason for reintervention. Coronary compression is not uncommon in Ross patients and should be assessed prior to TPVR.

View details for DOI 10.1016/j.athoracsur.2015.04.108

View details for PubMedID 26190388

Aortic Wall Injury Related to Endovascular Therapy for Aortic Coarctation. Circulation. Cardiovascular interventions Tretter, J. T., Jones, T. K., McElhinney, D. B. 2015; 8 (9)


Aortic wall complications can occur in unrepaired aortic coarctation (CoA) and after surgical repair or endovascular treatment. This review summarizes the available literature and current understanding of aortic wall injury (AWI) surrounding the management of CoA, focusing specifically on acute and follow-up AWI after endovascular treatment. There have been 23 reported cases of aortic rupture after endovascular treatment for CoA, including angioplasty alone, bare metal stenting, and primary covered stent therapy. Even if these published cases represent only a minority of ruptures that have actually occurred, the incidence is substantially <1%. The incidence of acute aneurysm formation was 0% to 13% after angioplasty, 0% to 5% after bare metal stent placement, and <1% after covered stent placement. The reported incidence and natural history of both acute and new AWI during follow-up after endovascular therapy for CoA varies considerably, likely secondary to ascertainment and reporting biases and inconsistent definitions. Although important AWI after endovascular treatment of CoA seems to be declining in frequency with increasing experience and improving technology, it remains one of the most important potential adverse outcomes. Long-term surveillance for new AWI and monitoring of existing AWI is mandatory, with institution of appropriate treatment when necessary. A central research focus in this population should be determination of the appropriate treatment for both native and recurrent CoA across various ages with regard to limiting recurrent CoA and preventing associated aortic wall complications, in addition to determining the appropriate treatment of various AWI. Consistent definitions and reporting are necessary to truly understand the incidence of, risk factors for, and measures protective against AWI after angioplasty or stent implantation for CoA.

View details for DOI 10.1161/CIRCINTERVENTIONS.115.002840

View details for PubMedID 26291468

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era A Multicenter Study CIRCULATION Freud, L. R., Escobar-Diaz, M. C., Kalish, B. T., Komarlu, R., Puchalski, M. D., Jaeggi, E. T., Szwast, A. L., Freire, G., Levasseur, S. M., Kavanaugh-McHugh, A., Michelfelder, E. C., Moon-Grady, A. J., Donofrio, M. T., Howley, L. W., Tierney, E. S., Cuneo, B. F., Morris, S. A., Pruetz, J. D., van der Velde, M. E., Kovalchin, J. P., Ikemba, C. M., Vernon, M. M., Samai, C., Satou, G. M., Gotteiner, N. L., Phoon, C. K., Silverman, N. H., McElhinney, D. B., Tworetzky, W. 2015; 132 (6): 481-489


Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 276 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001).In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

View details for DOI 10.1161/CIRCULATIONAHA.115.015839

View details for Web of Science ID 000359666500003

View details for PubMedID 26059011

Clinical and hemodynamic outcomes up to 7 years after transcatheter pulmonary valve replacement in the US melody valve investigational device exemption trial. Circulation Cheatham, J. P., Hellenbrand, W. E., Zahn, E. M., Jones, T. K., Berman, D. P., Vincent, J. A., McElhinney, D. B. 2015; 131 (22): 1960-1970


Studies of transcatheter pulmonary valve (TPV) replacement with the Melody valve have demonstrated good short-term outcomes, but there are no published long-term follow-up data.The US Investigational Device Exemption trial prospectively enrolled 171 pediatric and adult patients (median age, 19 years) with right ventricular outflow tract conduit obstruction or regurgitation. The 148 patients who received and were discharged with a TPV were followed up annually according to a standardized protocol. During a median follow-up of 4.5 years (range, 0.4-7 years), 32 patients underwent right ventricular outflow tract reintervention for obstruction (n=27, with stent fracture in 22), endocarditis (n=3, 2 with stenosis and 1 with pulmonary regurgitation), or right ventricular dysfunction (n=2). Eleven patients had the TPV explanted as an initial or second reintervention. Five-year freedom from reintervention and explantation was 764% and 923%, respectively. A conduit prestent and lower discharge right ventricular outflow tract gradient were associated with longer freedom from reintervention. In the 113 patients who were alive and reintervention free, the follow-up gradient (median, 4.5 years after implantation) was unchanged from early post-TPV replacement, and all but 1 patient had mild or less pulmonary regurgitation. Almost all patients were in New York Heart Association class I or II. More severely impaired baseline spirometry was associated with a lower likelihood of improvement in exercise function after TPV replacement.TPV replacement with the Melody valve provided good hemodynamic and clinical outcomes up to 7 years after implantation. Primary valve failure was rare. The main cause of TPV dysfunction was stenosis related to stent fracture, which was uncommon once prestenting became more widely adopted.URL: Unique identifier: NCT00740870.

View details for DOI 10.1161/CIRCULATIONAHA.114.013588

View details for PubMedID 25944758

Intermediate Outcomes in the Prospective, Multicenter Coarctation of the Aorta Stent Trial (COAST) CIRCULATION Meadows, J., Minahan, M., McElhinney, D. B., McEnaney, K., Ringel, R. 2015; 131 (19): 1656-1664


The Coarctation of the Aorta Stent Trial (COAST) was designed to assess the safety and efficacy of the Cheatham Platinum stent when used in children and adults with native or recurrent coarctation. Acute outcomes have been reported. We report here follow-up to 2 years.A total of 105 patients underwent attempted implantation, with 104 successes. There were no procedural deaths, serious adverse events, or surgical intervention. All patients experienced immediate reduction in upper- to lower-extremity blood pressure difference with sustained improvement to 2 years. Rates of hypertension and medication use decreased from baseline to 12 months and remained largely unchanged at 2 years. Six aortic aneurysms have been identified: 5 were successfully treated with covered stent placement, and 1 resolved without intervention. Stent fractures were noted in 2 patients at 1 year and 11 patients at 2 years, with evidence of fracture progression. To date, only larger stent diameter was associated with stent fracture. Twelve additional fractures have occurred after 2 years. No fracture has resulted in loss of stent integrity, stent embolization, aortic wall injury, or reobstruction. Nine reinterventions occurred in the first 2 years for stent redilation and address of aneurysms, and 10 additional reinterventions occurred after 2 years.The Cheatham Platinum stent is safe and associated with persistent relief of aortic obstruction. Stent fracture and progression of fracture occur but have not resulted in clinically important sequelae. Reintervention is common and related to early and late aortic wall injury and need for re-expansion of small-diameter stents.URL: Unique identifier: NCT00552812.

View details for DOI 10.1161/CIRCULATIONAHA.114.013937

View details for Web of Science ID 000354213000011

View details for PubMedID 25869198

Percutaneous tricuspid valve implantation: two-center experience with midterm results. Circulation. Cardiovascular interventions Eicken, A., Schubert, S., Hager, A., Hrer, J., McElhinney, D. B., Hess, J., Ewert, P., Berger, F. 2015; 8 (4)
Percutaneous tricuspid valve implantation: two-center experience with midterm results. Circulation. Cardiovascular interventions Eicken, A., Schubert, S., Hager, A., Hrer, J., McElhinney, D. B., Hess, J., Ewert, P., Berger, F. 2015; 8 (4)


Severe tricuspid valve (TV) dysfunction may lead to surgical TV replacement with a biological valve prosthesis in patients with congenital heart disease. To expand the lifetime of this valve and reduce the number of surgeries, percutaneous TV implantation (PTVI) may be an effective alternative to repeated surgery. We report on our 2-center experience with PTVI.Between 2008 and 2014, 17 percutaneous valves were implanted in 16 patients with TV bioprosthesis dysfunction (9 females) from 2 centers. Median age and weight were 31.3 years (5-77.2) and 65.2 kg (17.7-107); 14 patients had congenital heart disease (univentricular heart with a right atrial to right ventricle bioprosthesis in 3, Ebstein's anomaly of the TV in 5, and other in 6), and 2 had acquired TV dysfunction. All procedures were successful (Melody n=7, Sapien 26 mm valve n=4, Sapien XT 29 mm valve n=6). One valve showed early dysfunction. It was replaced surgically and shortly after that a repeated PTVI was performed. The median duration of follow-up was 2.1 years (3 days to 6.3 years). The percutaneous valve was performing well in 15 of 16 patients.PTVI was safe and effectively improved TV function in all but 1 patient at midterm follow-up. We think that PTVI is a good alternative to repeated surgical TV replacements and that it may reduce the total number of open heart surgeries in these patients.

View details for DOI 10.1161/CIRCINTERVENTIONS.114.002155

View details for PubMedID 25873731

Tricuspid Valve Regurgitation in Congenitally Corrected Transposition of the Great Arteries and a Left Ventricle to Pulmonary Artery Conduit ANNALS OF THORACIC SURGERY Buber, J., McElhinney, D. B., Valente, A. M., Marshall, A. C., Landzberg, M. J. 2015; 99 (4): 1348-1356


The configuration of the interventricular septum can affect the function of the tricuspid valve in patients with congenitally corrected transposition of the great arteries who have a systemically functioning right ventricle. Altering septal configuration by addressing a dysfunctional conduit placed between the left ventricle (LV) and the pulmonary artery (PA) in these patients can impact septal configuration and competency of the tricuspid valve.In 38 patients with an LV to PA conduit, we evaluated relationships between conduit function, RV geometry, and tricuspid valve function, and compared these variables before and after conduit intervention.Median age at conduit implant was 4.5 years (0.5 to 36) and median total follow-up was 12 years (2 to 22). Of the 38 patients, 23 (60%) underwent conduit intervention, a median of 7.5 years after implant. In 15 of these patients (65%) the degree of tricuspid regurgitation (TR) worsened, compared with only 2 patients (15%) in the non-intervention group (p < 0.001). Worsening TR was associated with the degree of change in RV and LV ventricular diameters, change in tricuspid annulus size and tethering distance, and the degree of septal shift, as reflected by the right ventricular sphericity index (all p 0.04). In 8 of 15 patients with more severe TR at follow-up, there was also progressive RV dysfunction.Intervention for LV to PA conduit dysfunction may result in worsening TR and right ventricular function, likely due in part to altered septal shift due to changes in the interventricular pressure ratio. Management of LV to PA conduit dysfunction should take these findings into account.

View details for DOI 10.1016/j.athoracsur.2014.11.008

View details for Web of Science ID 000352162100045

View details for PubMedID 25661908

Management of Cardiovascular Risk Factors in Adults With Congenital Heart Disease JOURNAL OF THE AMERICAN HEART ASSOCIATION Lui, G. K., Fernandes, S., McElhinney, D. B. 2014; 3 (6)
Echocardiographic predictors of left ventricular dysfunction after aortic valve surgery in children with chronic aortic regurgitation. Congenital heart disease Selamet Tierney, E. S., Gal, D., Gauvreau, K., Zhou, J., Soluk, Y., McElhinney, D. B., Colan, S. D., Geva, T. 2013; 8 (4): 308-315


OBJECTIVE: Postoperative left ventricular dysfunction is associated with poor prognosis in adults with severe chronic aortic regurgitation and published practice guidelines aim to minimize this risk. However, only limited information exists in pediatrics. The goal of this study was to define preoperative risk factors for postoperative left ventricular dysfunction in children with chronic aortic regurgitation. METHODS: Patients fulfilling the following criteria were included in this study: (1) age at preoperative echocardiogram 18 years; (2) moderate aortic regurgitation; (3) mild aortic valve stenosis; (4) no additional valve disease/shunt; (5) underwent aortic valve surgery for aortic regurgitation; and (6) available preoperative and 6-month postoperative echocardiograms with adequate information. Primary outcome was postoperative left ventricular dysfunction defined as ejection fraction z-score < -2. RESULTS: Median ages at diagnosis and surgery of the 53 eligible patients were 6.9 (0.04-17.2) and 13 years (1.2-22.4), respectively. Compared with patients whose postoperative left ventricular ejection fraction was normal, those with left ventricular ejection fraction z-score < -2 (n = 10) had significantly higher preoperative left ventricular end-diastolic and systolic volumes and dimensions and lower indices of systolic function. Preoperative left ventricular ejection fraction z-score < -1 was the most sensitive (89%; confidence interval [CI] 52, 100) but least specific (58%; CI 41, 73), whereas left ventricular end-systolic diameter z-score 5 was the most specific (95%; CI 84, 99) but least sensitive (60%; CI 26, 88) outcome identifier. A combination of shortening fraction z-score < -1 or end-systolic diameter z-score 5 best identified postoperative left ventricular dysfunction with an area of 0.819 under the receiver-operator characteristic curve. CONCLUSION: Lower indices of left ventricular systolic function and severity of dilation identify children at risk for postoperative left ventricular dysfunction after aortic valve surgery. These identifiers are similar to predictors defined in adult patients albeit with different threshold values.

View details for DOI 10.1111/chd.12009

View details for PubMedID 23075071

Echocardiographic Predictors of Left Ventricular Dysfunction after Aortic Valve Surgery in Children with Chronic Aortic Regurgitation CONGENITAL HEART DISEASE Tierney, E. S., Gal, D., Gauvreau, K., Zhou, J., Soluk, Y., McElhinney, D. B., Colan, S. D., Geva, T. 2013; 8 (4): 308-315

View details for DOI 10.1111/chd.12009

View details for Web of Science ID 000326501000014

Outcomes After Stent Implantation for the Treatment of Congenital and Postoperative Pulmonary Vein Stenosis in Children CIRCULATION-CARDIOVASCULAR INTERVENTIONS Balasubramanian, S., Marshall, A. C., Gauvreau, K., Peng, L. F., Nugent, A. W., Lock, J. E., McElhinney, D. B. 2012; 5 (1): 109-117


Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid.We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 628% at 1 year and 508% at 5 years after pulmonary vein stent implantation. Stent placement acutely relieved focal obstruction in all veins. Of the 54 stents reexamined with catheterization, 32 underwent reintervention. Freedom from reintervention was 627% at 6 months and 427% at 1 year. Stent occlusion was documented in 9 cases and significant in-stent stenosis in 17 cases. Stent implantation diameter 7 mm was associated with longer freedom from reintervention (hazard ratio, 0.32; P=0.015) and from significant in-stent stenosis (hazard ratio, 0.14; P=0.002). Major acute complications occurred in 5 cases.Transcatheter stent implantation can acutely relieve PVS in children, but reintervention is common. Larger stent lumen size at implantation is associated with longer stent patency and a lower risk of reintervention.

View details for DOI 10.1161/CIRCINTERVENTIONS.111.964189

View details for Web of Science ID 000300610900021

View details for PubMedID 22253356