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Lynn Peng, MD

  • Lynn F Peng

Specialties

Cardiology

Work and Education

Professional Education

Johns Hopkins University School of Medicine, Baltimore, MD, 2001

Residency

Johns Hopkins Hospital, Baltimore, MD, 2004

Fellowship

Children's Hospital Boston, Boston, MA, 2008

Board Certifications

Pediatric Cardiology, American Board of Pediatrics

Conditions Treated

Coronary Anomalies

All Publications

Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Adamson, G. T., Peng, L. F., Lui, G. K., Perry, S. B. 2019; 93 (4): 66063

View details for DOI 10.1002/ccd.28073

View details for Web of Science ID 000461016400030

Outcomes After Initial Unifocalization to a Shunt in Complex Tetralogy of Fallot with MAPCAs. The Annals of thoracic surgery Bauser-Heaton, H., Ma, M., Wise-Faberowski, L., Asija, R., Shek, J., Zhang, Y., Peng, L. F., Sidell, D. R., Hanley, F. L., McElhinney, D. B. 2019

Abstract

Our approach to tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) emphasizes early single-stage unifocalization and intracardiac repair. However, a subset of patients with small native pulmonary arteries (PAs) and MAPCAs undergo unifocalization to a shunt rather than simultaneous intracardiac repair.Patients with TOF/MAPCAs who underwent unifocalization to a systemic-to-PA shunt by a single surgeon were reviewed. The decision to perform simultaneous intracardiac repair was based on an intraoperative flow study or empirical assessment of PA and MAPCA size.From 11/01-12/17, 57 patients with TOF/MAPCAs underwent unifocalization to a shunt at a median age of 6.9 months. Genetic abnormalities were documented in 60% of patients, including a chromosome 22q11 deletion in 25 and Alagille syndrome in 8. Twenty patients (35%) had undergone prior surgery elsewhere (n=16) and/or at our center (n=7). During a median follow-up of 5.4 years, 9 patients had additional surgery to revise the PA reconstruction before complete repair, and 38 patients underwent complete repair. Survival was 746% 5 years after unifocalization. At follow-up, the median PA:aortic systolic pressure ratio was 0.36 and was >0.50 in 2 patients.In patients with the smallest MAPCAs and PAs, single-stage unifocalization to a shunt followed by intracardiac repair yields an excellent outcome in most cases.

View details for DOI 10.1016/j.athoracsur.2019.01.030

View details for PubMedID 30772338

Pulmonary hemorrhage in children with Alagille syndrome undergoing cardiac catheterization. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Feinstein, J. A., Yarlagadda, V. V., Lin, A., Wise-Faberowski, L., McElhinney, D. B. 2019

Abstract

To evaluate the incidence, severity, and outcomes of pulmonary hemorrhage in children with Alagille syndrome (AGS) undergoing cardiac catheterization, and to find variables associated with hemorrhage in this population.Children with AGS have a high incidence of bleeding complications during invasive procedures. It has been our impression that catheterization-associated pulmonary hemorrhage is more common in children with AGS, but there are no published data on this topic.This was a retrospective single institution study of children with AGS undergoing catheterization from 2010 to 2018. Pulmonary hemorrhage was defined as angiographic or fluoroscopic evidence of extravasated blood in the lung parenchyma, or blood suctioned from the endotracheal tube with documentation of pulmonary hemorrhage by the anesthesiologist or intensivist. Univariate comparisons were made between catheterizations that did and did not have pulmonary hemorrhage.Thirty children with AGS underwent 87 catheterizations, 32 (37%) with interventions on the branch pulmonary arteries (PA). There were 26 (30%) procedures with hemorrhage, the majority (65%) of which were self-limited or required less than 24hr of mechanical ventilation. Moderate and severe hemorrhage occurred only in children with tetralogy of Fallot (TOF; 5 of 14, 36%). A higher right ventricle to aorta systolic pressure ratio (1.0 [0.85-1.1] vs. 0.88 [0.59-1.0], p = .029) and interventions on the branch PAs (14 of 26, 54% vs. 18 of 61, 30%, p = .032) were associated with hemorrhage.Pulmonary hemorrhage was common in children with AGS undergoing both intervention and diagnostic cardiac catheterization, and was associated with TOF, higher RV to aorta pressure ratio, and interventions on the branch PAs.

View details for DOI 10.1002/ccd.28508

View details for PubMedID 31584246

Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Lui, G. K., Perry, S. B. 2019

Abstract

Clinically significant unilateral pulmonary arteriovenous malformations (PAVM) can develop in patients with a Fontan circulation when there is unbalanced distribution of hepatic venous (HV) blood flow to the lungs. There are reported surgical and transcatheter techniques to treat PAVMs by rerouting HV return, with promising short-term results. We report a case of a novel, technically simple transcatheter approach to redirect HV blood flow in an adult Fontan patient with polysplenia syndrome and severe unilateral PAVMs. Our patient had a two-stage procedure, the first to redirect all HV blood flow to the affected lung with a single covered stent, and a second to confirm resolution of PAVMs and to reintroduce HV effluent to the unaffected lung. At 10-month follow-up, her oxygen saturations had increased from 75% to 93% with a marked improvement in her functional status.

View details for PubMedID 30629315

Utility of screening echocardiogram after endomyocardial biopsy for identification of cardiac perforation or tricuspid valve injury. Pediatric transplantation Peng, D. M., Kipps, A. K., Palmon, M., Tacy, T. A., Peng, L. F., Hollander, S. A., McElhinney, D. B. 2018; 22 (7): e13275

Abstract

Per protocol, our institution obtains echocardiograms immediately after each EMB to rule out procedural complication. We sought to determine the incidence of echocardiogram-detected cardiac perforation and TV injury and to evaluate the utility of routine screening echocardiogram after each EMB in the current era. At a single center, 99% (1917/1942) EMB performed in 162 patients were immediately followed by an echocardiogram per protocol. There were five newly diagnosed pericardial effusions, and only one required pericardiocentesis. In the three echocardiograms demonstrating new flail TV, only one patient underwent surgical repair 2months later. This study demonstrates the very low incidence of significant hemopericardium and TV injury after EMB in pediatric heart transplant recipients and argues against the utility of post-EMB echocardiograms to screen solely for procedural complications.

View details for PubMedID 30076684

Surgical unroofing of hemodynamically significant myocardial bridges in a pediatric population Maeda, K., Schnittger, I., Murphy, D. J., Tremmel, J. A., Boyd, J. H., Peng, L., Okada, K., Pargaonkar, V. S., Hanley, F. L., Mitchell, R., Rogers, I. S. MOSBY-ELSEVIER. 2018: 161826
Safety and Feasibility of Melody Transcatheter Pulmonary Valve Replacement in the Native Right Ventricular Outflow Tract A Multicenter Pediatric Heart Network Scholar Study JACC-CARDIOVASCULAR INTERVENTIONS Martin, M., Meadows, J., McElhinney, D. B., Goldstein, B. H., Bergersen, L., Qureshi, A. M., Shahanavaz, S., Aboulhosn, J., Berman, D., Peng, L., Gillespie, M., Armstrong, A., Weng, C., Minich, L., Gray, R. G. 2018; 11 (16): 164250

Abstract

This study sought to determine the safety and feasibility of transcatheter pulmonary valve replacement (TPVR) using the Melody valve in native (nonconduit) right ventricular outflow tracts (nRVOT), and to identify factors associated with successful TPVR.The Melody valve is Food and Drug Administration-approved for TPVR within right ventricle-to-pulmonary artery conduits and bioprosthetic pulmonary valves. However, most patients needing pulmonary valve replacement have nRVOT and TPVR has been adapted for this indication.In this multicenter retrospective study of all patients presenting for nRVOT TPVR, we collected pre-procedural magnetic resonance imaging, echocardiography, and catheterization data, and evaluated procedural and early outcomes.Of 229 patients (age 21 15 years from 11 centers), 132 (58%) had successful TPVR. In the remaining 97, TPVR was not performed, most often because of prohibitively large nRVOT (n= 67) or compression of the aortic root or coronary arteries (n= 18). There were no deaths and 5 (4%) serious complications, including pre-stent embolization requiring surgery in 4 patients, and arrhythmia in 1. Higher pre-catheterization echocardiographic RVOT gradient was associated with TPVR success (p= 0.001) and larger center volume approached significance (p= 0.08). Magnetic resonance imaging anterior-posterior and lateral RVOT diameters were smaller in implanted versus nonimplanted patients (18.0 3.6 mm vs. 20.1 3.5 mm; p= 0.005; 18.4 4.3 mm vs. 21.5 3.8 mm; p= 0.002).TPVR in the nRVOT was feasible and safe. However, nearly half the patients presenting for catheterization did not undergo TPV implantation, mainly because of prohibitively large nRVOT size. Improved understanding ofmagnetic resonance imaging data and availability of larger devices may improve the success rate for nRVOT TPVR.

View details for PubMedID 30077685

Repair of Ductus or Hemi-Truncus to One Lung and Major Aortopulmonary Collaterals to the Other Lung ANNALS OF THORACIC SURGERY Mainwaring, R. D., Rosenblatt, T. R., Patrick, W. L., Ma, M., Peng, L., Hanley, F. L. 2018; 106 (2): 56874
Surgical unroofing of hemodynamically significant myocardial bridges in a pediatric population. The Journal of thoracic and cardiovascular surgery Maeda, K., Schnittger, I., Murphy, D. J., Tremmel, J. A., Boyd, J. H., Peng, L., Okada, K., Pargaonkar, V. S., Hanley, F. L., Mitchell, R. S., Rogers, I. S. 2018

Abstract

BACKGROUND: Although myocardial bridges (MBs) are traditionally regarded as incidental findings, it has been reported that adult patients with symptomatic MBs refractory to medical therapy benefit from unroofing. However, there is limited literature in the pediatric population. The aim of our study was to evaluate the indications and outcomes for unroofing in pediatric patients.METHODS: We retrospectively reviewed all pediatric patients with MB in our institution who underwent surgical relief. Clinical characteristics, relevant diagnostic data, intraoperative findings, and postoperative outcomes were evaluated.RESULTS: Between 2012 and 2016, 14 pediatric patients underwent surgical unroofing of left anterior descending artery MBs. Thirteen patients had anginal symptoms refractory to medical therapy, and 1 patient was asymptomatic until experiencing aborted sudden cardiac arrest during exercise. Thirteen patients underwent exercise stress echocardiography, all of which showed mid-septal dys-synergy. Coronary computed tomography imaging confirmed the presence of MBs in all patients. Intravascular ultrasound imaging confirmed the length of MBs: 28.216.3mm, halo thickness: 0.590.24mm, and compression of left anterior descending artery at resting heart rate: 33.011.6%. Invasive hemodynamic assessment with dobutamine confirmed the physiologic significance of the MBs with diastolic fractional flow reserve: 0.590.13. Unroofing was performed with the patient under cardiopulmonary bypass (CPB) in the initial 9 cases and without CPB in the subsequent 5 cases. All patients were discharged without complications. The 13 symptomatic patients reported resolution of symptoms on follow-up, and improvement in symptoms and quality of life was documented using the Seattle Angina Questionnaire version 7.CONCLUSIONS: Unroofing of MBs can be safely performed in pediatric patients, with or without use of CPB. In symptomatic patients, unroofing can provide relief of symptoms refractory to medical therapy.

View details for PubMedID 30005887

An unexpected case of post-operative superior caval vein syndrome. Cardiology in the young Peng, L., Wise-Faberowski, L. 2018; 28 (6): 87981

Abstract

Superior caval vein obstruction in children after congenital heart surgery has been more associated with thrombosis formation as result of single-ventricle palliation, infection, indwelling devices/catheters, or external compression. Many of these patients will present to the cardiac catheterisation laboratory for evaluation and possible intervention. We present an unusual case of superior caval vein obstruction in a patient after Tetralogy of Fallot repair.

View details for PubMedID 29697045

An unexpected case of post-operative superior caval vein syndrome CARDIOLOGY IN THE YOUNG Peng, L., Wise-Faberowski, L. 2018; 28 (6): 87981
Repair of Ductus or Hemi-Truncus to One Lung and Major Aortopulmonary Collaterals to the Other Lung. The Annals of thoracic surgery Mainwaring, R. D., Rosenblatt, T. R., Patrick, W. L., Ma, M., Peng, L., Hanley, F. L. 2018

Abstract

BACKGROUND: There are patients born with pulmonary atresia and a ductus arteriosus or hemi-truncus to one lung and major aortopulmonary collateral arteries (MAPCAs) to the contralateral lung. The purpose of this study was to review our surgical results for this relatively rare subset of patients.METHODS: This was a retrospective review of 35 patients with ductus/hemi-truncus in association with pulmonary atresia with ventricular septal defect and MAPCAs. Our surgical algorithm is bifurcated into two strategies: 1) patients with "favorable" MAPCAs are candidates for single stage complete repair, and 2) patients with "unfavorable" MAPCAs undergo a staged approach. The median age at surgery was 3 months (range 1-7 months), and the mean number of MAPCAs was 3.1 1.3.RESULTS: Twenty patients underwent a single stage complete repair. All 20 of these patients are alive with a right ventricle to aortic pressure ratio of 0.34 0.07. The distribution of pulmonary blood flow by lung perfusion scan was 44% to the right and 56% to the left lung. Fifteen patients underwent a staged approach, including unifocalization of the MAPCAs to a central shunt. There were two interim deaths. Twelve patients have undergone complete repair with a right ventricle to aortic pressure ratio of 0.38 0.07. There was one late death after complete repair. The distribution of pulmonary blood flow was 39% to the right and 61% to the left lung.CONCLUSIONS: The data demonstrate that 32 of 35 patients with ductus/hemi-truncus and MAPCAs ultimately achieved complete repair with relatively low pulmonary artery pressures.

View details for PubMedID 29684371

Pulmonary reinterventions after complete unifocalization and repair in infants and young children with tetralogy of Fallot with major aortopulmonary collaterals JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Bauser-Heaton, H., Borquez, A., Asija, R., Wise-Faberowski, L., Zhang, Y., Downey, L., Perry, S. B., Koth, A., Peng, L. F., Algaze, C. A., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 16961707

Abstract

Our institutional approach to tetralogy of Fallot (TOF) with majoraortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit.We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis.The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 703% at 5years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 643% at 5years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size.We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.

View details for PubMedID 29352588

Interstage evaluation of homograft-valved right ventricle to pulmonary artery conduits for palliation of hypoplastic left heart syndrome JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sandeep, N., Punn, R., Balasubramanian, S., Smith, S. N., Reinhartz, O., Zhang, Y., Wright, G. E., Peng, L. F., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 1747-+

Abstract

Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling.This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015. A composite valved conduit (cryopreserved homograft valve anastomosed to a polytetrafluoroethylene tube) was placed in 100 patients (47 aortic, 32 pulmonary, 13 femoral/saphenous vein, 8 unknown), and a nonvalved conduit was used in 30 patients. Echocardiographic functional parameters were evaluated before and after stage 1 palliation and before the bidirectional Glenn procedure, and interstage interventions were assessed.On competing risk analysis, survival over time was better in the valved conduit group (P=.040), but this difference was no longer significant after adjustment for surgical era. There was no significant difference between groups in the cumulative incidence of bidirectional Glenn completion (P=.15). Patients with a valved conduit underwent more interventions for conduit obstruction in the interstage period, but this difference did not reach significance (P=.16). There were no differences between groups in echocardiographic parameters of right ventricle function at baseline or pre-Glenn.In this cohort of patients with hypoplastic left heart syndrome, inclusion of a valved right ventricle to pulmonary artery conduit was not associated with any difference in survival on adjusted analysis and did not confer an identifiable benefit on right ventricle function.

View details for PubMedID 29223842

Transcatheter Pulmonary Valve Replacement With the Melody Valve in Small Diameter Expandable Right Ventricular Outflow Tract Conduits JACC-CARDIOVASCULAR INTERVENTIONS Shahanavaz, S., Qureshi, A. M., Levi, D. S., Boudjemline, Y., Peng, L. F., Martin, M., Bauser-Heaton, H., Keeshan, B., Asnes, J. D., Jones, T. K., Justino, H., Aboulhosn, J. A., Gray, R. G., Nguyen, H., Balzer, D. T., McElhinney, D. B. 2018; 11 (6): 55464

Abstract

This study sought to evaluate the safety, feasibility, and outcomes of transcatheter pulmonary valve replacement (TPVR) in conduits16 mm in diameter.The Melody valve (Medtronic, Minneapolis, Minnesota) is approved for the treatment of dysfunctional right ventricular outflow tract (RVOT) conduits16 mm in diameter at the time of implant. Limited data are available regarding the use of this device in smaller conduits.The study retrospectively evaluated patients from 9 centers who underwent percutaneous TPVR into a conduit that was16 mm in diameter at the time of implant, and reported procedural characteristics and outcomes.A total of 140 patients were included and 117 patients (78%; median age and weight 11 years of age and 35 kg, respectively) underwent successful TPVR. The median original conduit diameter was 15 (range: 9 to 16) mm, and the median narrowest conduit diameter was 11 (range: 4 to 23) mm. Conduits were enlarged to a median diameter of 19 mm (29% larger than the implanted diameter), with no difference between conduits. There was significant hemodynamic improvement post-implant, with a residual peak RVOT pressure gradient of 7mmHg (p< 0.001) and no significant pulmonary regurgitation. During a median follow-up of 2.0 years, freedom from RVOT reintervention was 97% and 89% at 2 and 4years, respectively, and there were no deaths and 5 cases of endocarditis (incidence rate 2.0% per patient-year).In this preliminary experience, TPVR with the Melody valve into expandable small diameter conduitswas feasible and safe, with favorable early and long-term procedural and hemodynamic outcomes.

View details for PubMedID 29566801

THE DEVELOPMENT AND EFFICACY OF A PEDIATRIC CARDIOLOGY FELLOWSHIP ONLINE PREPARATORY COURSE Motonaga, K., Sacks, L., Olson, I., Balasubramanian, S., Chen, S., Peng, L., Feinstein, J., Silverman, N., Hanley, F., Axelrod, D., Krawczeski, C., Ceresnak, S. ELSEVIER SCIENCE INC. 2018: 2622
Percutaneous transcatheter pulmonary valve replacement in children weighing less than 20 kg CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Martin, M., Shahanavaz, S., Peng, L. F., Asnes, J. D., Riley, M., Hellenbrand, W. E., Balzer, D. T., Gray, R. G., McElhinney, D. B. 2018; 91 (3): 48594

Abstract

Since FDA approval of the Melody valve, transcatheter pulmonary valve replacement (TPVR) has been offered to an expanding population. Limited data exist regarding the safety and feasibility of TPVR in smaller patients.All patients weighing <20 kg who underwent catheterization for percutaneous TPVR at four centers were reviewed.Of the 51 patients reviewed, 43 (84%) had successful valve implantation and 8 (16%) were found not to be candidates due to access veins too small (n=3), coronary artery/aortic root compression (n=3), and RVOT too large (n=2). The 43 patients who underwent successful percutaneous TPVR had a median age and weight of 5.8 years (3.3-10) and 17.7 kg (13.5-19.8), and most had tetralogy of Fallot (TOF). TPVR was performed via femoral vein (FV) access in 24 patients and internal jugular vein (IJV) access in 19 patients. Median weight in patients with IJV implant was 1.1 kg lower than those who underwent transfemoral implant (17.0 vs. 18.1 kg, P=0.05). There were four adverse events: one iliac vein injury, one contained MPA tear, and two patients with post-procedure femoral vein bleeding. All patients were alive at recent follow-up, a median of 2.0 years (0.1-6.0) after TPVR. There was excellent valve function with mean Doppler gradients of 3-20 mm Hg, and trivial or mild pulmonary regurgitation in all but one patient. There were no RVOT reinterventions and no cases of endocarditis.Percutaneous TPVR can be safely performed in patients <20 kg. The procedure frequently requires IJV access, and access site/bleeding complications may be more common in this cohort.

View details for PubMedID 29193671

Endovascular stenting of obstructed right ventricle-to-pulmonary artery conduits: a fifteen-year experience Peng, Mcelhinney

Abstract

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 PubMedID 28356265

Endovascular stenting of obstructed right ventricle-to-pulmonary artery conduits: a fifteen-year experience Circulation Peng

Abstract

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

Endovascular stenting of obstructed right ventricle-to-pulmonary artery conduits: a fifteen-year experience Circulation Peng, L.

Abstract

During a pulmonary hypertensive crisis, the marked increase in pulmonary vascular resistance can result in acute right ventricular failure and death. Currently, there are no therapeutic guidelines for managing an acute crisis. This pilot study examined the hemodynamic effects of phenylephrine, arginine vasopressin, and epinephrine in pediatric patients with pulmonary hypertension.In this prospective, open-label, nonrandomized pilot study, we enrolled pediatric patients previously diagnosed with pulmonary hypertensive who were scheduled electively for cardiac catheterization. Primary outcome was a change in the ratio of pulmonary-to-systemic vascular resistance. Baseline hemodynamic data were collected before and after the study drug was administered.Eleven of 15 participants were women, median age was 9.2 years (range, 1.7-14.9 yr), and median weight was 26.8 kg (range, 8.5-55.2 kg). Baseline mean pulmonary artery pressure was 49 19 mm Hg, and mean indexed pulmonary vascular resistance was 10 5.4 Wood units. Etiology of pulmonary hypertensive varied, and all were on systemic pulmonary hypertensive medications.Patients 1-5 received phenylephrine 1 g/kg; patients 6-10 received arginine vasopressin 0.03 U/kg; and patients 11-15 received epinephrine 1 g/kg. Hemodynamics was measured continuously for up to 10 minutes following study drug administration.After study drug administration, the ratio of pulmonary-to-systemic vascular resistance decreased in three of five patients receiving phenylephrine, five of five patients receiving arginine vasopressin, and three of five patients receiving epinephrine. Although all three medications resulted in an increase in aortic pressure, only arginine vasopressin consistently resulted in a decrease in the ratio of systolic pulmonary artery-to-aortic pressure.This prospective pilot study of phenylephrine, arginine vasopressin, and epinephrine in pediatric patients with pulmonary hypertensive showed an increase in aortic pressure with all drugs although only vasopressin resulted in a consistent decrease in the ratio of pulmonary-to-systemic vascular resistance. Studies with more subjects are warranted to define optimal dosing strategies of these medications in an acute pulmonary hypertensive crisis.

View details for DOI 10.1097/PCC.0000000000000716

View details for PubMedID 27144689

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)

Abstract

One anatomic variant of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is characterized by the absence of intrapericardial pulmonary arteries. This anatomy obviates the possibility of incorporating the pulmonary arteries for reconstruction or palliative procedures. The purpose of this study was to evaluate the surgical results in patients undergoing repair of PA/VSD/MAPCAs with absent pulmonary arteries.This was a retrospective review of 35 patients who underwent surgical repair of PA/VSD/MAPCAs with absent pulmonary arteries between 2007 and 2014. The median age at the time of surgery was 3.4 months, and the median weight was 4.9 kg. All patients underwent unifocalization of MAPCAs, with an average of 3.5 1.4 MAPCAs per patient.Twenty-eight of the 35 patients (80%) underwent complete single-stage surgical repair, including unifocalization of MAPCAs, VSD closure, and right ventricle to pulmonary artery conduit. After complete repair, the average right ventricular to aortic pressure ratio was 0.33 0.07. There were no deaths in this subgroup. Seven patients (20%) were not deemed suitable candidates for VSD closure after their unifocalization procedure, and therefore underwent palliation with a central shunt. There was 1 operative death and 1 interim death. Three patients have subsequently undergone complete repair, and 2 are awaiting further evaluation and treatment.The majority of patients with PA/VSD/MAPCAs and absent pulmonary arteries can undergo complete single-stage repair with satisfactory postoperative hemodynamics. These results suggest that unifocalization of MAPCAs can provide a reasonable pulmonary vascular bed in the absence of intrapericardial pulmonary arteries.

View details for DOI 10.1016/j.athoracsur.2015.03.110

View details for PubMedID 26138766

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

Abstract

Describe outcomes following unplanned cardiac catheterization after congenital heart surgery.Utility of cardiac catheterization following congenital heart surgery is relatively understudied.Retrospective study examining demographics, indications, and outcomes of unplanned cardiac catheterization after congenital heart surgery at a single institution.Between October 2004 and April 2011, 120 patients underwent 150 unplanned postoperative cardiac catheterizations. Median day of catheterization was postoperative day 20 (range 1-269 days). Survival 30 days postcatheterization was 85%; overall survival to hospital discharge was 72%. Indications for catheterization: 63 for hemodynamic evaluation, 46 for likely intervention, and 41 for assessment of surgical repair. Of the 150 hemodynamic/interventional catheterizations, 103 (69%) were associated with a change in clinical management: 59 trans-catheter interventions, 22 re-operations, 11 changes in medication, six changes in surgical plan, and five withdrawals of support. Complications included hemorrhage in two patients, supraventricular tachycardia in two patients, and transient complete heart block requiring cardiopulmonary resuscitation in one patient.Cardiac catheterization following congenital heart surgery may enable important diagnostic and therapeutic changes in clinical and surgical management. Complications were rare.

View details for DOI 10.1002/ccd.25490

View details for Web of Science ID 000342826900018

Hemodynamic Effects of Phenylephrine, Vasopressin, and Epinephrine in Children With Pulmonary Hypertension: A Pilot Study PEDIATRIC CRITICAL CARE MEDICINE Siehr, S. L., Feinstein, J. A., Yang, W., Peng, L. F., Ogawa, M. T., Ramamoorthy, C. 2016; 17 (5): 428-437

Abstract

Describe outcomes following unplanned cardiac catheterization after congenital heart surgery.Utility of cardiac catheterization following congenital heart surgery is relatively understudied.Retrospective study examining demographics, indications, and outcomes of unplanned cardiac catheterization after congenital heart surgery at a single institution.Between October 2004 and April 2011, 120 patients underwent 150 unplanned postoperative cardiac catheterizations. Median day of catheterization was postoperative day 20 (range 1-269 days). Survival 30 days postcatheterization was 85%; overall survival to hospital discharge was 72%. Indications for catheterization: 63 for hemodynamic evaluation, 46 for likely intervention, and 41 for assessment of surgical repair. Of the 150 hemodynamic/interventional catheterizations, 103 (69%) were associated with a change in clinical management: 59 trans-catheter interventions, 22 re-operations, 11 changes in medication, six changes in surgical plan, and five withdrawals of support. Complications included hemorrhage in two patients, supraventricular tachycardia in two patients, and transient complete heart block requiring cardiopulmonary resuscitation in one patient.Cardiac catheterization following congenital heart surgery may enable important diagnostic and therapeutic changes in clinical and surgical management. Complications were rare.

View details for DOI 10.1002/ccd.25490

View details for PubMedID 24659225

Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries ANNALS OF THORACIC SURGERY Carrillo, S. A., Mainwaring, R. D., Patrick, W. L., Bauser-Heaton, H. D., Peng, L., Reddy, V. M., Hanley, F. L. 2015; 100 (2): 606-614

Abstract

Children with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) are at risk for reperfusion pulmonary edema (RPE) after unifocalization procedures to reconstruct the central pulmonary arteries. The purpose of this study was to determine the incidence of RPE, describe the clinical course of patients with RPE, and explore the mechanism of RPE in this population by measuring plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation.Levels of plasma receptor for advanced glycation end products (RAGE), intercellular adhesion molecule 1 (ICAM-1), and interleukin 6 (IL-6) were measured at baseline and postoperative day (POD) 0, 1, and 2 after unifocalization. A pediatric radiologist reviewed chest radiographs from the same time points and scored each lung segment for the degree of pulmonary edema. A pediatric interventional cardiologist reviewed the preoperative angiograms for each patient and determined the degree of stenosis for each aortopulmonary collateral vessel. RPE was defined as localized pulmonary edema with a pulmonary edema score of at least 2 occurring in the lung segment demonstrating the greatest degree of angiographic stenosis within the first 48 hours after surgery and with resolution by discharge.Thirty-five patients who underwent 37 unifocalization procedures were enrolled, and 32 patients were included in the analysis. Of these, 16 of 32 (50%) demonstrated evidence of RPE based on our defined criteria. There was no significant difference in RAGE (P=.60), ICAM-1 (P=.34), or IL-6 (P=.31) levels between those with and without RPE at any time point. The mean duration of mechanical ventilation in patients with RPE versus those without was not significantly different (5.14.2 vs 5.64.5 days, respectively; P=.57).Fifty percent of children with TOF/PA/MAPCAs undergoing unifocalization surgery developed RPE. Levels of plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation were not increased in patients with RPE compared with those without RPE. The presence of RPE did not affect the duration of respiratory failure and mechanical ventilation. The process of RPE is clinically self-limited and seems unlikely to be associated with vascular changes.

View details for DOI 10.1016/j.jtcvs.2014.01.017

View details for PubMedID 24534681

Outcomes Following Cardiac Catheterization After Congenital Heart Surgery CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Siehr, S. L., Martin, M. H., Axelrod, D., Efron, B., Peng, L., Roth, S. J., Perry, S., Shin, A. Y. 2014; 84 (4): 622-628

Abstract

Children with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) are at risk for reperfusion pulmonary edema (RPE) after unifocalization procedures to reconstruct the central pulmonary arteries. The purpose of this study was to determine the incidence of RPE, describe the clinical course of patients with RPE, and explore the mechanism of RPE in this population by measuring plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation.Levels of plasma receptor for advanced glycation end products (RAGE), intercellular adhesion molecule 1 (ICAM-1), and interleukin 6 (IL-6) were measured at baseline and postoperative day (POD) 0, 1, and 2 after unifocalization. A pediatric radiologist reviewed chest radiographs from the same time points and scored each lung segment for the degree of pulmonary edema. A pediatric interventional cardiologist reviewed the preoperative angiograms for each patient and determined the degree of stenosis for each aortopulmonary collateral vessel. RPE was defined as localized pulmonary edema with a pulmonary edema score of at least 2 occurring in the lung segment demonstrating the greatest degree of angiographic stenosis within the first 48 hours after surgery and with resolution by discharge.Thirty-five patients who underwent 37 unifocalization procedures were enrolled, and 32 patients were included in the analysis. Of these, 16 of 32 (50%) demonstrated evidence of RPE based on our defined criteria. There was no significant difference in RAGE (P=.60), ICAM-1 (P=.34), or IL-6 (P=.31) levels between those with and without RPE at any time point. The mean duration of mechanical ventilation in patients with RPE versus those without was not significantly different (5.14.2 vs 5.64.5 days, respectively; P=.57).Fifty percent of children with TOF/PA/MAPCAs undergoing unifocalization surgery developed RPE. Levels of plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation were not increased in patients with RPE compared with those without RPE. The presence of RPE did not affect the duration of respiratory failure and mechanical ventilation. The process of RPE is clinically self-limited and seems unlikely to be associated with vascular changes.

View details for DOI 10.1016/j.jtcvs.2014.01.017

View details for Web of Science ID 000342896200077

Outcomes following cardiac catheterization after congenital heart surgery. Catheterization and cardiovascular interventions Siehr, S. L., Martin, M. H., Axelrod, D., Efron, B., Peng, L., Roth, S. J., Perry, S., Shin, A. Y. 2014; 84 (4): 622-628

Abstract

The objectives of this manuscript are two-fold: (a) to describe the clinical characteristics and management of four pediatric patients with bacterial endocarditis (BE) after Melody pulmonary valve implantation (MPVI); and (b) to review the literature regarding Melody pulmonary valve endocarditis.There are several reports of BE following MPVI. The clinical course, BE management and outcome remain poorly defined.This is a multi-center report of four pediatric patients with repaired tetralogy of Fallot (TOF) and BE after MPVI. Clinical presentation, echocardiogram findings, infecting organism, BE management, and follow-up assessment are described. We review available literature on Melody pulmonary valve endocarditis and discuss the prognosis and challenges in the management of these patients.Of our four BE patients, two had documented vegetations and three showed worsening pulmonary stenosis. All patients remain asymptomatic after medical treatment (4) and surgical prosthesis replacement (3) at follow-up of 17 to 40 months. Analysis of published data shows that over half of patients undergo bioprosthesis explantation and that there is a 13% overall mortality. The most common BE pathogens are the Staphylococcus and Streptococcus species.Our case series of four pediatric patients with repaired TOF confirms a risk for BE after MPVI. A high index of suspicion for BE should be observed after MPVI. All patients should be advised to follow lifelong BE prophylaxis after MPVI. In case of BE, surgery should be considered for valve dysfunction or no clinical improvement in spite of medical treatment.

View details for DOI 10.1002/ccd.25375

View details for Web of Science ID 000340554200009

View details for PubMedID 24403185

Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance. journal of thoracic and cardiovascular surgery Asija, R., Roth, S. J., Hanley, F. L., Peng, L., Liu, K., Abbott, J., Zhuo, H., Matthay, M. 2014; 148 (4): 1560-1565

Abstract

Rare cases of aortic-left atrial tunnel exist in the literature. This case report highlights the echocardiographic characterization of this vascular anomaly and provides the first description of an aortic-left atrial tunnel closed by interventional cardiac catheterization in a pediatric patient.

View details for DOI 10.1111/chd.12059

View details for PubMedID 23601836

Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Asija, R., Roth, S. J., Hanley, F. L., Peng, L., Liu, K., Abbott, J., Zhuo, H., Matthay, M. 2014; 148 (4): 1560-1565

Abstract

Rare cases of aortic-left atrial tunnel exist in the literature. This case report highlights the echocardiographic characterization of this vascular anomaly and provides the first description of an aortic-left atrial tunnel closed by interventional cardiac catheterization in a pediatric patient.

View details for DOI 10.1111/chd.12059

View details for Web of Science ID 000329916300010

View details for PubMedID 23601836

Melody (R) Pulmonary Valve Bacterial Endocarditis: Experience in Four Pediatric Patients and a Review of the Literature CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Villafane, J., Baker, G. H., Austin, E. H., Miller, S., Peng, L., Beekman, R. 2014; 84 (2): 212-218

Abstract

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex form of congenital heart defect. There are limited data regarding late hemodynamics of patients after repair of PA/VSD/MAPCAs. This study evaluated the hemodynamics of patients who underwent complete repair of PA/VSD/MAPCSs and subsequently returned for a conduit change.This was a retrospective review of 80 children undergoing a right ventricle (RV)-to-pulmonary artery conduit replacement after complete repair of PA/VSD/MAPCAs. All patients underwent preoperative cardiac catheterization to define the cardiac physiology. Patients were an average age of 6.51.2 years, and the average interval between complete repair and conduit change was 4.51.1 years.The preoperative cardiac catheterization demonstrated an average RV right peak systolic pressure of 7022 mm Hg and pulmonary artery pressure of 3814 mm Hg. This pressure gradient of 32 mm Hg reflects the presence of conduit obstruction. After conduit change, the intraoperative RV systolic pressure was 348 mm Hg, similar to 369 mm Hg at the conclusion of the previous complete repair. The corresponding RV/aortic pressure ratios were 0.360.07 and 0.390.09, respectively.The data demonstrate that patients who underwent complete repair of PA/VSD/MAPCAs had nearly identical pulmonary artery pressures when they returned for conduit change some 4.5 years later. This finding indicates that the growth and development of the unifocalized pulmonary vascular bed is commensurate with visceral growth. We would hypothesize that complete repair, along with low RV pressures, will confer a long-term survival advantage.

View details for DOI 10.1016/j.athoracsur.2012.12.066

View details for PubMedID 23453744

Transcatheter device closure of a congenital aortic-left atrial tunnel. Congenital heart disease Sun, H. Y., Buccola, K. J., Punn, R., Silverman, N. H., Peng, L. F., Perry, S. B., Balasubramanian, S. 2014; 9 (1): E23-6

Abstract

Cerebral hypoxia-ischemia remains a complication in children with congenital heart disease. Near-infrared spectroscopy can be utilized at the bedside to detect cerebral hypoxia-ischemia. This study aimed to calibrate and validate an advanced technology near-infrared cerebral oximeter for use in children with congenital heart disease.After institutional review board approval and parental consent, 100 children less than 12 years and less than 40 kg were enrolled. Phase I (calibration) measured arterial and jugular venous saturation (SaO(2), SjO(2)) by co-oximetry simultaneously with device signals to calibrate an algorithm to determine regional cerebral saturation against a weighted average cerebral saturation (0.7 SjO(2) + 0.3 SaO(2)). Phase II (validation) evaluated regional cerebral saturation from the algorithm against the weighted average cerebral saturation by correlation, bias, precision, and A(Root Mean Square) assessed by linear regression and Bland-Altman analysis.Of 100 patients, 86 were evaluable consisting of 7 neonates, 44 infants, and 35 children of whom 55% were female, 79% Caucasian, and 41% with cyanotic disease. The SaO(2) and regional cerebral saturation ranged from 34% to 100% and 34% to 91%, respectively. There were no significant differences in subject characteristics between phases. For the entire cohort, A(RMS), bias, precision, and correlation coefficient were 5.4%, 0.5%, 5.39%, and 0.88, respectively. Age, skin color, and hematocrit did not affect these values.This cerebral oximeter accurately measures the absolute value of cerebral saturation in children over a wide range of oxygenation and subject characteristics, offering advantages in assessment of cerebral hypoxia-ischemia in congenital heart disease.

View details for DOI 10.1016/j.athoracsur.2012.05.096

View details for Web of Science ID 000310439700031

View details for PubMedID 22858270

Transcatheter Device Closure of a Congenital Aortic-Left Atrial Tunnel CONGENITAL HEART DISEASE Sun, H. Y., Buccola, K. J., Punn, R., Silverman, N. H., Peng, L. F., Perry, S. B., Balasubramanian, S. 2014; 9 (1): E23-E26

Abstract

Pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) is a complex form of congenital heart defect. One identifiable subset has small (<2.5 mm) intrapericardial branch pulmonary arteries that are (1) confluent, (2) have normal arborization, and (3) have dual-supplied collateral vessels. When this anatomy is associated with limited pulmonary blood flow, the patients are candidates for creation of an aortopulmonary window to stimulate growth of the pulmonary arteries. The purpose of this study was to review our experience with creation of an aortopulmonary window as the initial palliative procedure.This was a retrospective review of our surgical experience with 35 children undergoing aortopulmonary window creation from 2002 to 2011. Patients were identified by preoperative cardiac catheterization to define the cardiac and pulmonary artery anatomy.There was no mortality in 35 patients undergoing aortopulmonary window creation. These patients have subsequently undergone 78 cardiac procedures (with 2 operative mortalities). Eighteen of these patients have achieved complete repair, 4 patients in a second procedure, 6 patients in a third procedure, 5 patients in a fourth procedure, and 3 patients in a fifth procedure.The data demonstrate that patients can undergo creation of an aortopulmonary window with excellent early results. Few patients were amenable to complete repair at the second operation, and most required multiple reoperations to recruit sufficient arborization. We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed.

View details for DOI 10.1016/j.athoracsur.2012.03.061

View details for PubMedID 22857982

Hemodynamic Assessment After Complete Repair of Pulmonary Atresia With Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Peng, L., Kuan, C., Palmon, M., Hanley, F. L. 2013; 95 (4): 1397-1402

Abstract

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

Evaluation of Pediatric Near-Infrared Cerebral Oximeter for Cardiac Disease Late-Breaking Clinical Trial Abstract Session on Congenital Heart Surgery at the 48th Annual Meeting of the Society-of-Thoracic-Surgeons / Surgical Motion Picture Session Kreeger, R. N., Ramamoorthy, C., Nicolson, S. C., Ames, W. A., Hirsch, R., Peng, L. F., Glatz, A. C., Hill, K. D., Hoffman, J., Tomasson, J., Kurth, C. D. ELSEVIER SCIENCE INC. 2012: 152733

Abstract

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.

View details for DOI 10.1016/j.jacc.2011.09.022

View details for PubMedID 22192720

Late Outcomes in Patients Undergoing Aortopulmonary Window for Pulmonary Atresia/Stenosis and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Perry, S. B., Peng, L., Hanley, F. L. 2012; 94 (3): 842-849

Abstract

Pulmonary vein stenosis (PVS) is a rare and often lethal condition in children. The optimal treatment for congenital and postoperative PVS is unknown.We compared outcomes of conventional balloon angioplasty performed for PVS from 1999 to 2003 against cutting balloon angioplasty performed from 2004 to 2007. A total of 100 previously undilated pulmonary veins in 54 patients were studied: 48 veins dilated with conventional balloons and 52 with cutting balloons. Acute results included significantly reduced gradients and increased lumen diameters with both treatments. Acutely, cutting balloon angioplasty and conventional angioplasty yielded similar relative reduction of the PVS gradient (median 78% vs. 63%, P = 0.08) and increase in lumen diameter (median 77% vs. 59%, P = 0.07). There was one procedural death of a critically ill infant, and four cardiac arrests, but no adverse events necessitating surgical intervention. Survival free from reintervention was poor in both groups, and shorter in the cutting balloon group (73% at 1 month, 11% at 6 months, and 4% at 1 year) than in the conventional angioplasty group (77% at 1 month, 35% at 6 months, and 23% at 1 year; P = 0.01).Both conventional and cutting balloon angioplasty were effective at decreasing gradient and increasing lumen size acutely in patients with congenital and postoperative PVS, but reintervention was common with both treatments. Both methods of angioplasty provided limited benefit, and neither was curative for this complex disease.

View details for DOI 10.1002/ccd.22405

View details for Web of Science ID 000278503900022

View details for PubMedID 20146341

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

Abstract

The slope of the minute ventilation versus CO2 production relationship (VE/VCO2 slope) is an index of gas exchange efficiency during exercise. In patients with repaired tetralogy of Fallot (rTOF), it correlates negatively with exercise capacity and is one of the best predictors of peak oxygen consumption (VO2). In these patients, the magnitude of the VE/VCO2 slope is related to the severity of pulmonary blood flow maldistribution (PBFM). The purpose of this study was to determine whether, in patients with rTOF, improvements in PBFM after a successful balloon angioplasty procedure (BAP) result in improvements in peak VO2 and gas exchange during exercise.Seventeen patients with rTOF and residual pulmonary artery stenoses referred for BAP were recruited. Exercise tests were performed and PBFM determined before and after BAP.Nine patients (group 1) had a successful BAP (ie, improvement of >5 percentage points in PBFM); 8 did not (group 2). Patients in group 1 had significantly greater improvements in VE/VCO2 slope, peak VO2, and peak oxygen pulse (an index of forward stroke volume at peak exercise) than did patients in group 2. A significant correlation existed between the improvement in PBFM and the decline in the VE/VCO2 slope (r = -0.70, P = .002). Changes in peak oxygen pulse accounted for 89% of the improvement in peak VO2.In these patients, a successful BAP resulted in improved peak VO2 and more efficient gas exchange during exercise. The improvement in peak VO2 appeared to be mediated by an increase in forward stroke volume.

View details for DOI 10.1016/j.ahj.2007.08.019

View details for PubMedID 18082511

Hypoplastic Left Heart Syndrome Current Considerations and Expectations JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Feinstein, J. A., Benson, D. W., Dubin, A. M., Cohen, M. S., Maxey, D. M., Mahle, W. T., Pahl, E., Villafane, J., Bhatt, A. B., Peng, L. F., Johnson, B. A., Marsden, A. L., Daniels, C. J., Rudd, N. A., Caldarone, C. A., Mussatto, K. A., Morales, D. L., Ivy, D. D., Gaynor, J. W., Tweddell, J. S., Deal, B. J., Furck, A. K., Rosenthal, G. L., Ohye, R. G., Ghanayem, N. S., Cheatham, J. P., Tworetzky, W., Martin, G. R. 2012; 59 (1): S1-S42

Abstract

The optimal treatment for dysfunctional right ventricle-to-pulmonary artery (RV-PA) conduits is unknown. Limited follow-up data on stenting of RV-PA conduits have been reported.Between 1990 and 2004, deployment of balloon-expandable bare stents was attempted in 242 obstructed RV-PA conduits in 221 patients (median age, 6.7 years). Acute hemodynamic changes after stenting included significantly decreased RV systolic pressure (89+/-18 to 65+/-20 mm Hg, P<0.001) and peak RV-PA gradient (59+/-19 to 27+/-14 mm Hg, P<0.001). There were no deaths, and, aside from 5 malpositioned stents requiring surgical removal, there were no serious procedural complications. During follow-up of 4.0+/-3.2 years, 9 patients died and 2 underwent heart transplantation, none related to catheterization or stent malfunction. During 155 follow-up catheterizations in 126 patients, the stent was redilated in 83 patients and additional stents were placed in 41. Stent fractures were diagnosed in 56 patients (43%) and associated with stent compression and substernal location but did not cause acute hemodynamic consequences. By Kaplan-Meier analysis, median freedom from conduit surgery after stenting was 2.7 years (3.9 years in patients >5 years), with younger age, homograft conduit, conduit diameter < or =10 mm, diagnosis other than tetralogy of Fallot, Genesis stent, higher prestent RV:aortic pressure ratio, and stent malposition associated with shorter freedom from surgery. Tricuspid regurgitation and RV function did not change between stent implantation and subsequent surgery.Conduit stenting is an effective interim treatment for RV-PA conduit obstruction and prolongs conduit lifespan in most patients. Stent fractures were common but not associated with significant complications or earlier conduit reoperation.

View details for DOI 10.1161/CIRCULATIONAHA.105.607127

View details for PubMedID 16735676

Comparison of Conventional and Cutting Balloon Angioplasty for Congenital and Postoperative Pulmonary Vein Stenosis in Infants and Young Children CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Peng, L. F., Lock, J. E., Nugent, A. W., Jenkins, K. J., McElhinney, D. B. 2010; 75 (7): 1084-1090
Effect of pulmonary artery angioplasty on exercise function after repair of tetralogy of Fallot. American heart journal Sutton, N. J., Peng, L., Lock, J. E., Lang, P., Marx, G. R., Curran, T. J., O'Neill, J. A., Picard, S. T., Rhodes, J. 2008; 155 (1): 18286
Endovascular stenting of obstructed right ventricle-to-pulmonary artery conduits: a 15-year experience. Circulation Peng, L. F., McElhinney, D. B., Nugent, A. W., Powell, A. J., Marshall, A. C., Bacha, E. A., Lock, J. E. 2006; 113 (22): 25982605