Diagnosing Neonatal Aortic Coarctation in the Setting of Patent Ductus Arteriosus ANNALS OF THORACIC SURGERY 2016; 101 (3): 1005-1011
Diagnosing Neonatal Aortic Coarctation intheSetting of Patent Ductus Arteriosus. Annals of thoracic surgery 2016; 101 (3): 1005-1010
In neonates, it is challenging to diagnose aortic coarctation in the setting of a patent ductus arteriosus (PDA). Frequently, serial echocardiograms are performed, and diagnosis is delayed until the PDA closes. The purpose of this study was to identify echocardiographic predictors of neonatal coarctation in the presence of a PDA in cases in which diagnosis is uncertain.We retrospectively identified neonates diagnosed with possible but not definitive coarctation in the presence of a PDA by echocardiography (January 2004 through August 2013). The carotid-subclavian artery index (CSAi) was defined as the distal transverse arch diameter divided by the distance between the left common carotid and left subclavian arteries. Medical records were reviewed to identify patients who underwent coarctation repair within 1 year. A separate validation group was identified with the same methodology (September 2013 through April 2015).Thirty-three patients were identified (median age 1, range 0-8 days). Twelve patients (36%) underwent coarctation repair. The coarctation group had smaller aortic and mitral valves, distal transverse arch, and isthmus z scores, larger right innominate artery, and longer transverse arch compared with the remaining group (p < 0.05). The CSAi was lower in the coarctation group (p = 0.014), and a cutoff of less than 0.85 yielded a sensitivity of 0.83and specificity of 0.86 for coarctation (area under the receiver operating characteristic curve, 0.91). In the validation group (n= 12; median follow-up, 316 days), none of the 8 patients with a CSAi of greater than 0.85 have required surgery. The intraclass correlation coefficient for CSAi was 0.79 (95% confidence interval, 0.18 to 0.95).The CSAi, a simple and reproducible measure, can identify neonates at risk for aortic coarctation even in the presence of a PDA, prevent multiple echocardiographic evaluations, and hence guide appropriate resource utilization.
View details for DOI 10.1016/j.athoracsur.2015.09.050
View details for PubMedID 26652138
Assessment of Speckle-Tracking Echocardiography-Derived Global Deformation Parameters During Supine Exercise in Children PEDIATRIC CARDIOLOGY 2016; 37 (3): 519-527
Assessment of Speckle-Tracking Echocardiography-Derived Global Deformation Parameters During Supine Exercise in Children. Pediatric cardiology 2016; 37 (3): 519-527
Exercise echocardiography is an underutilized tool in pediatrics with current applications including detecting segmental wall abnormalities, assessing the utility of global ventricular function, and measuring pulmonary hemodynamics. No prior study has applied speckle-tracking echocardiography (STE) during exercise echocardiography in children. The aim of this study was to determine the feasibility of measuring speckle-tracking-derived peak systolic velocities, global longitudinal and circumferential strain, and global strain rates at various phases of exercise. Ninety-seven healthy children underwent cardiopulmonary exercise testing using supine cycle ergometry. The exercise stress test consisted of baseline pulmonary function testing, monitoring of blood pressure and heart rate responses, electrocardiographic recordings, and oxygen saturations while subjects pedaled against a ramp protocol based on body weight. Echocardiographic measurements and specifically speckle-tracking analysis were performed during exercise at baseline, at a heart rate of 160 beats per minute and at 10min after exercise. Peak systolic velocity, peak systolic strain, and peak systolic strain rate at these three phases were compared in the subjects in which all measurements were accurately obtained. We were able to complete peak velocity, strain, and strain rate measurements in all three exercise phases for 36 out of the 97 subjects tested. There was no significant difference between the feasibility of measuring circumferential versus longitudinal strain (p=0.25, B-corrected=0.75). In the 36 subjects studied, the magnitude of circumferential strain values decreased from -18.34.8 to -13.74.0% from baseline to HR 160 (p<0.0001, B-corrected<0.0001), before returning to -19.64.4% at recovery (p=0.19 when compared to baseline). Longitudinal strain did not vary significantly from baseline to HR 160 (from -17.74.4 to -16.64.4%, p=0.16); likewise the average recovery strain was no different from those values (-18.43.6%; p=0.34). Peak circumferential and longitudinal strain rates increased from baseline to HR 160, but neither decreased to baseline levels after 10min of recovery, which correlated with heart rate variations with exercise. We studied the effects of frame rate on deformation measurements and we observed no difference between measurements taken at lower (<60 frames per second, fps) and higher (60fps) frame rates. This study shows that it is technically difficult to retrospectively measure peak velocities, strain, and strain rate in exercising pediatric subjects with STE. The majority of subjects that were excluded from the study had inadequate echocardiographic images when tachycardic from increased respiratory effort and body movements near peak exercise. Improvements in technique and higher image frame rates could make application of STE to pediatric cardiopulmonary testing more successful in the future.
View details for DOI 10.1007/s00246-015-1309-z
View details for PubMedID 26671508
A Pilot Study Assessing ECG versus ECHO Ventriculoventricular Optimization in Pediatric Resynchronization Patients. Journal of cardiovascular electrophysiology 2016; 27 (2): 210-216
Cardiac resynchronization therapy indications and management are well described in adults. Echocardiography (ECHO) has been used to optimize mechanical synchrony in these patients; however, there are issues with reproducibility and time intensity. Pediatric patients add challenges, with diverse substrates and limited capacity for cooperation. Electrocardiographic (ECG) methods to assess electrical synchrony are expeditious but have not been extensively studied in children. We sought to compare ECHO and ECG CRT optimization in children.Prospective, pediatric, single-center cross-over trial comparing ECHO and ECG optimization with CRT. Patients were assigned to undergo either ECHO or ECG optimization, followed for 6 months, and crossed-over to the other assignment for another 6 months. ECHO pulsed-wave tissue Doppler and 12-lead ECG were obtained for 5 VV delays. ECG optimization was defined as the shortest QRSD and ECHO optimization as the lowest dyssynchrony index. ECHOs/ECGs were interpreted by readers blinded to optimization technique. After each 6 month period, these data were collected: ejection fraction, velocimetry-derived cardiac index, quality of life, ECHO-derived stroke distance, M-mode dyssynchrony, study cost, and time. Outcomes for each optimization method were compared.From June 2012 to December 2013, 19 patients enrolled. Mean age was 9.1 4.3 years; 14 (74%) had structural heart disease. The mean time for optimization was shorter using ECG than ECHO (9 1 min vs. 68 13 min, P < 0.01). Mean cost for charges was $4,400 700 less for ECG. No other outcome differed between groups.ECHO optimization of synchrony was not superior to ECG optimization in this pilot study. ECG optimization required less time and cost than ECHO optimization.
View details for DOI 10.1111/jce.12863
View details for PubMedID 26515428
Cardioplegia Dose Effect on Immediate Postoperative Alterations in Coronary Artery Flow Velocities After Congenital Cardiac Surgery PEDIATRIC CARDIOLOGY 2016; 37 (2): 364-371
Abnormalities in coronary artery (CA) flow detected by echocardiography are increasingly used to guide clinical decisions in patient management. Increased CA flow has been seen postoperatively in congenital cardiac surgery. This study sought to determine immediate postoperative changes in left anterior descending (LAD) CA flow velocities, and to investigate possible factors associated with these changes. CA flow in the proximal LAD was sampled with pulsed-wave Doppler during trans-esophageal echocardiography imaging in the immediate preoperative and postoperative studies in 46 subjects. The peak velocity, velocity time integral (VTI), VTI corrected for heart rate (VTIc), and VTI rate pressure product (VTIrpp) were determined. The percent change in each measure between the preoperative and postoperative study was calculated and compared to age, body surface area (BSA), cardiopulmonary bypass time, cross-clamp time, and number of cardioplegia (CP) doses. The pH, oxygen saturation, temperature, and hemoglobin concentration (Hb) were compared for those with and without increased flow characteristics. There was an overall increase in LAD flow parameters in subjects who underwent congenital cardiac surgery. There was a significant and positive correlation of percent change in VTI, VTIc, and VTIrrp with number of CP doses and lower Hb. We propose that this phenomenon is likely of multifactorial origin, involving autoregulatory mechanism disturbance. The imaging and measurement of LAD flow velocities are feasible, reliable, and is positively correlated with number of CP doses. Interpretation of postoperative LAD flow velocities should be made in the context of intraoperative events since heart rate, blood pressure, and Hb concentration also influence CA flow parameters.
View details for DOI 10.1007/s00246-015-1285-3
View details for Web of Science ID 000371415300020
View details for PubMedID 26481223
Routine intra-operative trans-oesophageal echocardiography yields better outcomes in surgical repair of CHD CARDIOLOGY IN THE YOUNG 2016; 26 (2): 263-268
Trans-oesophageal echocardiographic imaging is valuable in the pre- and post-operative evaluation of children and adults with CHD; however, the frequency by which trans-oesophageal echocardiography guides the intra-operative course of patients is unknown.We retrospectively reviewed 1748 intra-operative trans-oesophageal echocardiograms performed between 1 October, 2005 and 31 December, 2010, and found 99 cases (5.7%) that required return to bypass, based in part upon the intra-operative echocardiographic findings.The diagnoses most commonly requiring further repair and subsequent imaging were mitral valve disease (20.9%), tricuspid valve disease (16.0%), atrioventricular canal defects (12.0%), and pulmonary valve disease (14.1%). The vast majority of those requiring immediate return to bypass benefited by avoiding subsequent operations and longer lengths of hospital stay. A total of 14 patients (0.8%) who received routine imaging required further surgical repair within 1 week, usually due to disease that developed over ensuing days. Patients who had second post-operative trans-oesophageal echocardiograms in the operating room rarely required re-operations, confirming the benefit of routine intra-operative imaging.This study represents a large single institutional review of intra-operative trans-oesophageal echocardiography, and confirms its applicability in the surgical repair of patients with CHD. Routine imaging accurately identifies patients requiring further intervention, does not confer additional risk of mortality or prolonged length of hospital stay, and prevents subsequent operations and associated sequelae in a substantial subset of patients. This study demonstrates the utility of echocardiography in intra-operative monitoring of surgical repair and highlights patients who are most likely to require return to bypass, as well as the co-morbidities of such manipulations.
View details for DOI 10.1017/S1047951115000098
View details for Web of Science ID 000369084000007
View details for PubMedID 25730612
Diagnosis of Anomalous Aortic Origin of the Left Coronary Artery in a Pediatric Patient. World journal for pediatric & congenital heart surgery 2015; 6 (3): 470-473
Anomalous aortic origin of the left coronary artery is rare and confers increased risk of sudden cardiac death. Accurate diagnosis is crucial and often requires many diagnostic modalities. This case report highlights the echocardiographic characteristics and pitfalls in diagnosing the anomaly in addition to the advantages of using computed tomography and magnetic resonance imaging in a pediatric patient.
View details for DOI 10.1177/2150135114558689
View details for PubMedID 26180168
Fate of Right Ventricle to Pulmonary Artery Conduits After Complete Repair of Pulmonary Atresia and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY 2015; 99 (5): 1685-1691
Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) requires insertion of a conduit from the right ventricle to the reconstructed pulmonary arteries. Although there is extensive literature on conduit longevity for many forms of congenital heart defects, there is currently limited information for PA/VSD/MAPCAs. It is likely that conduits will have a different longevity in PA/VSD/MAPCAs because pulmonary artery pressures are higher after unifocalization than in other congenital heart defects. The purpose of this study was to evaluate the fate of right ventricle to pulmonary artery conduits after complete repair of PA/VSD/MAPCAs.This was a retrospective review of 103 infants who underwent complete repair of PA/VSD/MAPCAs with insertion of an aortic homograft conduit. The average age at complete repair was 5.1 2.8 months, average conduit size was 13 2 mm, and average conduit z score was a positive 1.6 0.9.Conduit z scores were linearly correlated with increasing homograft conduit size. The average longevity of small homografts (8 to 11 mm) was 15 4 months, medium homografts (12 to 14 mm) was 39 7 months, and large homografts (15 to 17 mm) was 50 14 months. There was a negative correlation between increasing pulmonary artery pressure and conduit longevity.Conduit z scores and conduit size worked synergistically to predict the longevity of right ventricle to pulmonary artery conduits after complete repair of PA/VSD/MAPCAs. Pulmonary artery pressure was an additional modifier of longevity. These data provide a framework for expectations of aortic homograft conduits used in the repair of PA/VSD/MAPCAs.
View details for DOI 10.1016/j.athoracsur.2014.12.071
View details for Web of Science ID 000353877900045
Diminished exercise capacity and chronotropic incompetence in pediatric patients with congenital complete heart block and chronic right ventricular pacing HEART RHYTHM 2015; 12 (3): 560-565
Chronic right ventricular (RV) pacing has been associated with decreased exercise capacity and left ventricular (LV) function in adults with congenital complete atrioventricular block (CCAVB), but not in children.The purpose of this study was to evaluate the exercise capacity and LV function in pediatric patients with CCAVB receiving chronic RV pacing.We prospectively evaluated pediatric patients with isolated CCAVB receiving atrial synchronous RV pacing for at least 5 years. Supine bicycle ergometry was performed, and LV ejection fraction (EF) was evaluated by echocardiography.Ten CCAVB subjects and 31 controls were matched for age, gender, and body surface area. CCAVB subjects had normal resting EF (63.1% 4.0%) and had been paced for 7.9 1.4 years. Exercise testing demonstrated reduced functional capacity in CCAVB patients compared to controls with a lower VO2peak (26.0 6.6 mL/kg/min vs 39.9 7.0 mL/kg/min, P <.001), anaerobic threshold (15.6 3.9 mL/kg/min vs 18.8 2.7 mL/kg/min, P = .007), and oxygen uptake efficiency slope (1210 406 vs 1841 452, P <.001). Maximum heart rate (165 8 bpm vs 185 9 bpm, P <.001) and systolic blood pressure (159 17 mm Hg vs 185 12 mm Hg, P <.019) also were reduced in CCAVB patients despite maximal effort (respiratory exchange ratio 1.2 0.1). EF was augmented with exercise in controls but not in CCAVB patients (13.2% 9.3% vs 0.2% 4.8% increase, P <.001).Clinically asymptomatic children with chronic RV pacing due to CCAVB have significant reductions in functional capacity accompanied by chronotropic incompetence and inability to augment EF with exercise.
View details for DOI 10.1016/j.hrthm.2014.11.036
View details for Web of Science ID 000349966100018
View details for PubMedID 25433143
Echocardiographic Measures Associated With Early Postsurgical Myocardial Dysfunction in Pediatric Patients With Mitral Valve Regurgitation JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2015; 28 (3): 284-293
Echocardiographic measures associated with early postsurgical myocardial dysfunction in pediatric patients with mitral valve regurgitation. Journal of the American Society of Echocardiography 2015; 28 (3): 284-293
The assessment of left ventricular (LV) systolic function using conventional echocardiographic measures is problematic in the setting of mitral regurgitation (MR) given that altered loading conditions can mask underlying ventricular dysfunction. The purpose of this study was to characterize LV function and deformation before and after effective mitral valve repair or replacement to determine echocardiographic measures associated with early postoperative myocardial dysfunction.Baseline LV function was assessed retrospectively by conventional echocardiography and speckle-tracking strain analysis pre- and postoperatively in patients diagnosed with MR between January 2000 and March 2013, excluding patients with less than mild to moderate MR preoperatively, left-sided obstructive lesions, large septal defects, or more than mild MR postoperatively.Forty-six pediatric patients were evaluated (average age, 8.26.4years). Thirteen patients had normal preoperative ejection fractions but significant postoperative dysfunction (defined as an ejection fraction < 50%). Compared with the 33 patients with normal postoperative function, age (11.57.1 vs 7.35.7 years, P=.04), global circumferential strain (-13.25.6% vs -17.14.6%, P=.02), and global circumferential strain rate (-0.940.40 vs -1.360.42 sec(-1), P=.004) were found to be statistically different. Using receiver operating characteristic curves, an older preoperative age (area under the curve, 0.67; P=.03), lower global circumferential strain magnitude (area under the curve, 0.74; P=.007), and lower global circumferential strain rate magnitude (area under the curve, 0.80; P=.0004) were determined to be factors associated with early postoperative LV dysfunction after surgical repair of MR.Strain measurements may be useful as part of the echocardiographic assessment of patients with MR and can guide timing for surgical repair in the pediatric population.
View details for DOI 10.1016/j.echo.2014.11.010
View details for PubMedID 25555521
Ventricular lead redundancy to prevent cardiovascular events and sudden death from lead fracture in pacemaker-dependent children HEART RHYTHM 2015; 12 (1): 111-116
Children requiring a permanent epicardial pacemaker(PM) traditionally have a single lead placed on the right ventricle. Lead failure in pacemaker dependent(PMD) children, however, can result in cardiovascular events(CVE) and death.To determine if redundant ventricular lead systems(RVLS) can safeguard against CVE and death in PMD children.Single-center study of PMD patients undergoing placement of RVLS from 2002-2013. Patients 21 years of age who were PMD were included. Patients with biventricular systems(BiV) systems placed for standard resynchronization indications were excluded. RVLS patients were compared to PMD patients with only a single pacing lead on the ventricle(SiV).769 patients underwent PM/ICD placement with 76 BiV implants and there were 49 PMD patients(6%). 13 patients underwent implantation of a RVLS. There was no difference between the RVLS group(n=13) and SiV PMD control group(n=24) with regard to age(RVLS 9.55.8 vs. SiV 9.46.7 years; p=0.52), weight(RVLS 38.232.6 vs. SiV 35.229.3 kg; p=0.62), indication for pacing, procedural complications or time to follow-up. There were 2 lead fractures (17%) in the RVLS group(mean follow-up 3.82.9 years) with no deaths or presentations with CVE. The SiV control group had 3 lead fractures (13%)(mean follow-up 2.82.9 years), with no deaths, but all 3 patients presented with CVE and required emergent PM placement.RVLS systems should be considered in children who are PMD and require permanent epicardial pacing. BiV pacing and RVLS may decrease the risk of CVEs in the event of lead failure in PMD patients.
View details for DOI 10.1016/j.hrthm2014.09.056
View details for Web of Science ID 000346857100022
Predictors of Mortality in Pediatric Patients on Venoarterial Extracorporeal Membrane Oxygenation PEDIATRIC CRITICAL CARE MEDICINE 2014; 15 (9): 870-877
Currently, there are no established echocardiographic or hemodynamic predictors of mortality after weaning venoarterial extracorporeal membrane oxygenation in children. We wished to determine which measurements predict mortality.Over 3 years, we prospectively assessed six echo and six hemodynamic variables at 3-5 circuit rates while weaning extracorporeal membrane oxygenation flow. Hemodynamic measurements were heart rate, inotropic score, arteriovenous oxygen difference, pulse pressure, oxygenation index, and lactate. Echo variables included shortening/ejection fraction, outflow tract Doppler-derived stroke distance (velocity-time integral), degree of atrioventricular valve regurgitation, longitudinal strain (global longitudinal strain), and circumferential strain (global circumferential strain).Cardiovascular ICU at Lucille Packard Children's Hospital Stanford, CA.Patients were stratified into those who died or required heart transplant (Gr1) and those who did not (Gr2). For each patient, we compared the change for each variable between full versus minimum extracorporeal membrane oxygenation flow for each group.None.We enrolled 21 patients ranging in age from 0.02 to 15 years. Five had dilated cardiomyopathy, and 16 had structural heart disease with severe ventricular dysfunction. Thirteen of 21 patients (62%) comprised Gr1, including two patients with heart transplants. Eight patients constituted Gr2. Gr1 patients had a significantly greater increase in oxygenation index (35% mean increase; p < 0.01) off extracorporeal membrane oxygenation compared to full flow, but no change in velocity-time integral or arteriovenous oxygen difference. In Gr2, velocity-time integral increased (31% mean increase; p < 0.01), with no change in arteriovenous oxygen difference or oxygenation index. Pulse pressure increased modestly with flow reduction only in Gr1 (p < 0.01).Failure to augment velocity-time integral or an increase in oxygenation index during the extracorporeal membrane oxygenation weaning is associated with poor outcomes in children. We propose that these measurements should be performed during extracorporeal membrane oxygenation wean, as they may discriminate who will require alternative methods of circulatory support for survival.
View details for DOI 10.1097/PCC.0000000000000236
View details for Web of Science ID 000346400100015
Arterial applanation tonometry: feasibility and reproducibility in children and adolescents. American journal of hypertension 2014; 27 (9): 1218-1224
Aortic pulse wave velocity (PWV) and augmentation index (AIx) are markers of vascular health and have recently been used in pediatric clinical trials. However, there are limited data on standardization of these measurements in pediatrics. The objective of this study was to prospectively test the feasibility and reproducibility of PWV and AIx in children and adolescents.We performed arterial tonometry on 2 different days within 2 weeks in 40 healthy subjects aged 10-19 years. PWV and AIx were measured in triplicate on each visit.The visits were separated by a mean of 3.083.7 days. We obtained PWV in 77 of 80 (96%) visits and AIx in 76 of 80 (95%) visits in triplicate. Intraclass correlation coefficients (ICCs) for PWV were 0.61 (95% confidence interval (CI) = 0-0.86) when at least 2 measurements and 0.92 (95% CI = 0-1) when 3 measurements were obtained at each visit that met the quality criteria established for adults by the manufacturer (n = 17 and 3 paired visits, respectively). For AIx, ICCs were 0.78 (95% CI = 0.58-0.88) and 0.81 (95% CI = 0.63-0.90) when measurements with an operator index 80, a measure of the quality of the waveform, were included (n = 39 and 36 paired visits, respectively).Arterial applanation tonometry is feasible and reproducible in healthy children and adolescents. AIx has excellent intervisit reproducibility, whereas the intervisit reproducibility of PWV relies on acquisition of multiple measurements that meet quality criteria established for adults. These results have implications for the methodology of future pediatric clinical trials in a population at increasingly higher risk for premature atherosclerosis.
View details for DOI 10.1093/ajh/hpu034
View details for PubMedID 24627445
Arterial Applanation Tonometry: Feasibility and Reproducibility in Children and Adolescents AMERICAN JOURNAL OF HYPERTENSION 2014; 27 (9): 1218-1224
Coronary Sinus Obstruction after Atrioventricular Canal Defect Repair CONGENITAL HEART DISEASE 2014; 9 (4): E121-E124
The coronary sinus can become obstructed with any instrumentation at or near the ostium such as in atrioventricular canal defect repairs. This complication may lead to a wide range of consequences including dyspnea, angina, myocardial infarction, and sudden death. The following report illustrates the importance of careful perioperative echocardiographic evaluation of the coronary sinus in procedures that may affect the sinus and its ostium.
View details for DOI 10.1111/chd.12096
View details for Web of Science ID 000340525400005
View details for PubMedID 23682752
Surgical repair of anomalous aortic origin of a coronary artery EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY 2014; 46 (1): 20-26
Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischaemia and sudden death. There is an ongoing controversy over the indications for surgical intervention and the efficacy of that treatment compared with the natural history. The purpose of this study was to evaluate the medium-term results of surgical repair of AAOCA.Seventy-six patients underwent surgical repair of AAOCA at our institution from 1999 to 2013. There were 55 males and 21 females, and the median age at surgery was 15 years. Forty-seven (62%) of the 76 patients had an anomalous right coronary artery, 27 had an anomalous left coronary and 2 had an eccentric single coronary ostia. Forty-one patients had preoperative symptoms of myocardial ischaemia.Surgical repair was accomplished by unroofing of an intramural coronary in 55, reimplantation in 7 and pulmonary artery translocation in 14. There has been no early or late mortality, with a median duration of follow-up of 6 years. One patient presented with severe myocardial ischaemia and subsequently underwent heart transplantation a year following AAOCA surgery. The remaining patients have all remained free of cardiac symptoms.The results of this study demonstrate two major principles. First, surgical repair of AAOCA is quite safe in centres that take care of a significant number of patients with this entity. Secondly, the surgery is highly effective in eliminating symptoms of myocardial ischaemia. The growing amount of data on postoperative patients suggests that surgical repair can prevent the adverse events seen in the untreated 'natural' history. Based on these observations, it is our current recommendation that all teenagers identified with AAOCA should undergo surgical repair.
View details for DOI 10.1093/ejcts/ezt614
View details for Web of Science ID 000339666400008
View details for PubMedID 24431169
Evaluation of Risk Factors for Prediction of Outcome in Fetal Spectrum of Atrioventricular Septal Defects CONGENITAL HEART DISEASE 2014; 9 (4): 286-293
Atrioventricular septal defects (AVSD) are very commonly diagnosed in utero. Heterotaxy/chromosomal abnormalities frequently coexist with AVSD. However, outcomes of fetal AVSD are not precisely known. We attempted to define mortality risk factors in AVSD.We retrospectively searched our database, electronic records, and echocardiograms with diagnosis of fetal AVSD from 2003 to 2012. We investigated the following risk factors: atrial situs, heart rate/rhythm, ventricular dominance/morphology, atrioventricular valve regurgitation, cardiothoracic ratio, ejection fraction, and extracardiac anomalies.Forty-five fetuses with a median gestational age of 28 weeks (17.5-37.1) were determined to have AVSD during the 10 years, of which 12 were either lost to follow-up (6) or underwent termination (6). There were 16 deaths (48%); two died in utero. Isomerism was identified in 17 of 45 (37%) fetuses (11 left atrial, 6 right atrial isomerism) and chromosomal abnormalities were identified in 12 (27%). Twenty-eight of 33 fetuses, not lost to follow-up or terminated, had extracardiac anomalies which had associated increased mortality (57% vs. 0%, P = .04). Heart block (75% vs. 43%, P = .12), left ventricular noncompaction (80% vs. 43%, P = .17), and isomerism (63% vs. 41%, P = .28) were associated with mortality but without statistical significance. Twenty-five of 45 (56%) had unbalanced AVSD. Positional abnormalities of the great arteries or semilunar valve stenosis were present in 20/45 (44%) while venous anomalies were present in 16/45 (36%). Presence of ventricular dominance, atrioventricular valve regurgitation, elevated cardiothoracic ratio, or diminished ejection fraction were not associated with mortality.Overall mortality rate for fetuses with AVSD was 48%. The presence of extracardiac anomalies is an independent risk factor for prediction of fetal or neonatal demise. Heart block, isomerism, and noncompaction in fetal AVSD appear to be associated with poor outcomes as well but did not reach statistical significance. This information is useful for counseling parents with fetus AVSD.
View details for DOI 10.1111/chd.12136
View details for Web of Science ID 000340525400012
View details for PubMedID 24102719
Supine Cycling in Pediatric Exercise Testing: Disparity in Performance Measures PEDIATRIC CARDIOLOGY 2014; 35 (4): 705-710
Supine cycling may be an important alternative modality for exercise testing. Subtle differences in supine and upright exercise physiology have been suggested but not fully explored in the pediatric population. The aim of this study was to compare peak and submaximal metabolic data in the upright and supine exercise positions. Healthy children (N=100) performed cardiopulmonary exercise tests using supine and upright cycle ergometry. Recruitment was governed by grant funding and not based on sample size calculations. Subjects exercised to exhaustion. Paired Student's t-tests were used to compare upright and supine data; simple linear regression analyses examined correlations between the two modalities. Peak heart rate was similar in both testing positions. Although peak oxygen uptake (peak VO2), oxygen uptake at anaerobic threshold (VO2@AT), VO2 when the respiratory exchange ratio is consistently at or above 1.0 (VO2@RER1.0), and the oxygen uptake efficiency slope were significantly higher in the upright position, no difference was considered of practical significance when applying Cohen's effect size criteria. Therefore, it may be reasonable for pediatric exercise laboratories to use established normative data and predictions for upright cycling position and apply them to metabolic measures obtained in supine exercise testing. Supine cycling with submaximal parameters may allow objective assessment of exercise capacity in children who undergo exercise echocardiography or cannot perform upright exercise testing.
View details for DOI 10.1007/s00246-013-0841-y
View details for Web of Science ID 000333165400021
Predictors of bronchopulmonary dysplasia or death in premature infants with a patent ductus arteriosus PEDIATRIC RESEARCH 2014; 75 (4): 570-575
Background:Preterm infants with a PDA are at risk for death or development of BPD. However, PDA treatment remains controversial. We investigated if PDA treatment and other clinical or echocardiographic (ECHO) factors were associated with the development of death or BPD.Methods:We retrospectively studied clinical and ECHO characteristics of preterm infants with birth weight <1500g and ECHO diagnosis of a PDA. Logistic regression and classification and regression tree (CART) analyses were performed to assess variables associated with the combined outcome of death or BPD.Results:Of 187 preterm infants with a PDA, 75% were treated with indomethacin or surgery and 25% were managed conservatively. Death or BPD occurred in 80 (43%). Logistic regression found lower gestational age (OR 0.5), earlier year of birth during the study period (OR 0.9), and larger ductal diameter (OR 4.3) were associated with the decision to treat the PDA, while gestational age was the only variable associated with death or BPD (OR 0.6, 95% CI 0.5-0.8).Conclusion:Only lower gestational age and not PDA treatment or ECHO score was associated with the adverse outcome of death or BPD. Further investigation of PDA management strategies and effects on adverse outcomes of prematurity is needed.Pediatric Research (2013); doi:10.1038/pr.2013.253.
View details for DOI 10.1038/pr.2013.253
View details for Web of Science ID 000333139400014
View details for PubMedID 24378897
Transcatheter device closure of a congenital aortic-left atrial tunnel. Congenital heart disease 2014; 9 (1): E23-6
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
Transcatheter Device Closure of a Congenital Aortic-Left Atrial Tunnel CONGENITAL HEART DISEASE 2014; 9 (1): E23-E26
Ebstein anomaly and Trisomy 21: A rare association. Annals of pediatric cardiology 2014; 7 (1): 67-69
This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.
View details for DOI 10.4103/0974-2069.126569
View details for PubMedID 24701093
Systemic hypertension in an infant with unrepaired tetralogy of Fallot: case report CARDIOLOGY IN THE YOUNG 2013; 23 (5): 746-748
Patients with severe right ventricular outflow tract obstruction in tetralogy of Fallot typically have right-to-left shunting, resulting in low pulmonary blood flow and cyanosis. Here we present the case of an infant with tetralogy of Fallot and severe pulmonary valve stenosis, complicated by systemic hypertension, the presence of which altered flow dynamics and possibly prevented cyanosis.
View details for DOI 10.1017/S1047951112001837
View details for Web of Science ID 000323882900017
View details for PubMedID 23149090
Echocardiographic Predictors of Early Postsurgical Myocardial Dysfunction in Pediatric Patients With Aortic Valve Insufficiency PEDIATRIC CARDIOLOGY 2013; 34 (6): 1335-1343
In chronic aortic insufficiency (AI), left-ventricular (LV) dysfunction must be detected early to allow timely surgery. Strain and strain rate have been used for this purpose in adults, but the value of this method in pediatric AI has not been established. Forty patients with moderate to severe AI were included in this retrospective study. LV function was assessed by strain analysis and conventional echocardiography both before and after surgery. Of the 32 patients with preserved preoperative ejection fraction (EF; >50%), 8 had postoperative dysfunction (<50%). Mean conventional indices of global LV systolic performance for the entire cohort of patients with AI were predominantly in the normal range before surgery. Preoperative values for LV global longitudinal strain (GLS) and strain rate (GLSr) were normal. After surgery, there was a significant decrease in shortening and EF. There was a significant decrease from preoperative to postoperative values for both GLS (-16.073.82 vs. -11.063.88; p<0.0001) and GLSr (-0.890.24 vs. -0.720.27; p=0.0021). A preoperative GLS of -15.3 (AUC=0.83, CI=0.69-0.98, p<0.0001) and a GLSr of -0.79/s (AUC=0.86, CI=0.73-0.98, p<0.0001) were determined to be predictors of early postoperative dysfunction after surgical repair of moderate to severe AI. A preoperative GLS value of -15.3 and GLSr value of -0.79/s or less are predictors of postoperative ventricular dysfunction, which is defined by EF <50%. GLS and GLSr value determination may be useful as part of the echocardiographic assessment AI and may help determine the optimal timing of surgery in pediatric patient with at least moderate AI.
View details for DOI 10.1007/s00246-013-0646-z
View details for Web of Science ID 000321919400006
Decremental Left Ventricular Deformation after Pulmonary Artery Band Training and Subsequent Repair in Ventriculoarterial Discordance JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2013; 26 (7): 765-774
BACKGROUND: Patients with ventriculoarterial discordance, such as congenitally corrected and d-transposition of the great arteries, may undergo a morphologic left ventricular (LV) training strategy consisting of surgical pulmonary artery band (PAB) placement and subsequent anatomic repair to establish ventriculoarterial concordance. The purpose of this study was to characterize morphologic LV function and deformation longitudinally using speckle-tracking strain analysis in patients with ventriculoarterial discordance who underwent LV training. METHODS: Twenty-nine patients (12 with d-transposition of the great arteries and 17 with congenitally corrected transposition of the great arteries) who underwent LV training with PAB placement were evaluated retrospectively. LV ejection fraction and global and regional longitudinal strain and strain rate were measured before and 7 5 days after PAB placement and subsequent anatomic repair. RESULTS: PAB placement caused reductions in the mean LV ejection fraction from 76.1 10.2% to 66.7 7.8% (P < .001), in mean global strain from -17.7 9% to -13.3 7.5% (P= .01), and in mean lateral wall strain from -23.3 12.8% to -17.5 10.3% (P= .01). After anatomic repair (a median of 21 months after PAB placement; range, 0.5-104 months), mean LV ejection fraction decreased further from 63.3 8.6% to 52.4 14.9% (P < .05). Mean global strain declined from -17.6% 4.4 to -12.6 4% (P= .01), and mean lateral wall strain decreased from -18.2 11.4% to -12.6 5.3% (P= .04). CONCLUSIONS: In patients with ventriculoarterial discordance undergoing PAB placement for LV training and anatomic repair, the morphologic left ventricle demonstrated decremental systolic function and global longitudinal deformation acutely. Frequent functional assessment is warranted to understand long-term myocardial mechanics in these patients.
View details for DOI 10.1016/j.echo.2013.03.017
View details for Web of Science ID 000321051000011
Aortic Valve Disease in Pulmonary Atresia and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY 2013; 95 (6): 2122-2125
Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects.This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis andinsufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in1.Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve.This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs.
View details for DOI 10.1016/j.athoracsur.2013.03.070
View details for Web of Science ID 000319335400049
View details for PubMedID 23643545
Physiologic and molecular characterization of a murine model of right ventricular volume overload. American journal of physiology. Heart and circulatory physiology 2013; 304 (10): H1314-27
Pulmonary insufficiency (PI) is a common long-term sequel after repair of tetralogy of Fallot, causing progressive right ventricular (RV) dilation and failure. We describe the physiologic and molecular characteristics of the first murine model of RV volume overload. PI was created by entrapping the pulmonary valve leaflets with sutures. Imaging, catheterization, and exercise testing were performed at 1, 3, and 6 mo and compared with sham controls. RNA from the RV free wall was hybridized to Agilent whole genome oligonucleotide microarrays. Volume overload resulted in RV enlargement, decreased RV outflow tract shortening fraction at 1 mo followed by normalization at 3 and 6 mo (39 2, 44 2, and 41 2 vs. 46 3% in sham), early reversal of early and late diastolic filling velocities (E/A ratio) followed by pseudonormalization (0.87 0.08, 0.82 0.08, and 0.96 0.08 vs. 1.04 0.03; P < 0.05), elevated end-diastolic pressure (7.6 0.7, 6.9 0.8, and 7 0.5 vs. 2.7 0.2 mmHg; P < 0.05), and decreased exercise duration (26 0.4, 26 1, and 22 1.3 vs. 30 1.1 min; P < 0.05). Subendocardial RV fibrosis was evident by 1 mo. At 1 mo, 372 genes were significantly downregulated. Mitochondrial pathways and G protein-coupled receptor signaling were the most represented categories. At 3 mo, 434 genes were upregulated and 307 downregulated. While many of the same pathways continued to be downregulated, TNF-, transforming growth factor-1 (TGF-1), p53-signaling, and extracellular matrix (ECM) remodeling transitioned from down- to upregulated. We describe a novel murine model of chronic RV volume overload recapitulating aspects of the clinical disease with gene expression changes suggesting early mitochondrial bioenergetic dysfunction, enhanced TGF- signaling, ECM remodeling, and apoptosis.
View details for DOI 10.1152/ajpheart.00776.2012
View details for PubMedID 23504182
Physiologic and molecular characterization of a murine model of right ventricular volume overload AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY 2013; 304 (10): H1314-H1327
Surgical Reconstruction of Pulmonary Stenosis With Ventricular Septal Defect and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY 2013; 95 (4): 1417-1421
Pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals (PS/VSD/MAPCAs) is an extremely rare form of congenital heart defect. Although it has been assumed that PS/VSD/MAPCAs would be similar to pulmonary atresia (PA) with VSD/MAPCA, there are currently no data to support this conjecture. This study reviewed our surgical experience with reconstruction of PS/VSD/MAPCA.This was a retrospective review of 25 patients (14 boys, 11 girls) who were born with PS/VSD/MAPCA and underwent surgical reconstruction. Preoperative pulmonary angiography was used to define the central branch pulmonary arteries and MAPCA. Patients were a median age of 4 months at the first operation.There was one operative death (4%) in this cohort of 25 patients, and complete repair was achieved in the 24 survivors (96%). There were two distinct subgroups of patients: 11 demonstrated cyanosis in the neonatal timeframe and underwent an initial procedure to augment pulmonary blood flow (+PBF). The remaining 14 patients formed the second group (-PBF). The median age at the first operation was 0.8 months in the +PBF group and 5.2 months in the -PBF group (p<0.005). Complete repair was achieved in 91% of patients in the +PBF group and in 100% in the -PBF group; however, the average number of procedures to achieve complete repair was 2.8 in the +PBF group vs 1.0 in the -PBF group (p<0.005).Outcomes for PS/VSD/MAPCAs as a whole were excellent, with a low surgical mortality and high rate of complete repair. There were two identifiable subgroups with distinctive differences required in their surgical management. These results provide a prognostic outlook for patients with PS/VSD/MAPCAs that can be compared and contrasted with PA/VSD/MAPCAs.
View details for DOI 10.1016/j.athoracsur.2013.01.007
View details for Web of Science ID 000317150600039
View details for PubMedID 23434256
Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 2013; 145 (2): 476-481
Peripheral pulmonary artery stenosis is a rare congenital heart defect frequently found in association with Williams and Alagille syndromes. Controversy exists regarding the optimal treatment of peripheral pulmonary artery stenosis, with most centers favoring catheter-based interventions. In contrast, we have preferentially used surgical reconstruction of peripheral pulmonary artery stenosis. The purpose of the present study was to review our experience with surgical reconstruction of peripheral pulmonary artery stenosis.We performed a retrospective review of patients who underwent surgical reconstruction of peripheral pulmonary artery stenosis. A total of 16 patients were identified: 7 had Williams syndrome, 6 had Alagille syndrome, and 3 had no identifiable syndrome. Detailed pulmonary angiography was performed in all patients to define stenoses at the main, branch, lobar, and segmental arterial levels. The mean preoperative right ventricular/left ventricular pressure ratio was 0.88 0.07. The surgical approach was a median sternotomy with cardiopulmonary bypass. All peripheral stenoses were augmented with pulmonary artery homograft tissue. The median age at surgery was 14 months, and concomitant procedures were performed in 9 of the 16 patients.There was 1 operative mortality (6%). The mean right ventricular/left ventricular pressure ratio decreased to 0.40 0.04 postoperatively (P < .005), representing a 55% reduction compared with the preoperative values. The patients were followed up for a median of 5 years. No late mortality occurred and reoperation was not required.The data have demonstrate that this comprehensive surgical approach to the treatment of peripheral pulmonary artery stenosis was associated with low early and no late mortality. Surgical reconstruction of the peripheral pulmonary artery stenosis resulted in a significant decrease in right ventricular pressure. We hypothesize that this reduction in right ventricular pressures will confer a long-term survival advantage for this cohort of patients.
View details for DOI 10.1016/j.jtcvs.2012.09.102
View details for Web of Science ID 000313634700030
Insights into dyssynchrony in Hypoplastic Left Heart Syndrome HEART RHYTHM 2012; 9 (12): 2010-2015
Cardiac resynchronization therapy has been proposed for treatment of hypoplastic left heart syndrome (HLHS) patients with right ventricular (RV) failure. The role of dyssynchrony, however, is poorly understood in this population.The purpose of this study was to better understand the relationship between electrical and mechanical dyssynchrony in HLHS using 3-dimensional electrical mapping, tissue Doppler indices of wall motion, and vector velocity imaging.Eleven HLHS subjects with normal RV function and ten normal subjects (age 3-18 years) were studied. Electrical and mechanical activation times and dyssynchrony indices (electrical dyssynchrony index, mechanical dyssynchrony index) were calculated using 3-dimensional electrical mapping, tissue Doppler indices, and vector velocity imaging.No differences in measures of electrical dyssynchrony were seen when comparing HLHS patients and normal patients (electrical activation time 63.3 22.8 ms vs 56.2 11.2 ms, P = .38; electrical dyssynchrony index 13.7 6.3 ms vs 11.6 3.0 ms, P = .34). However, measures of mechanical dyssynchrony were markedly abnormal in HLHS patients despite normal RV function (mechanical activation time 16 11.3 ms vs 0.91.9 ms, P = .01; mechanical dyssynchrony index 7.5 5.5 vs 0.4 0.8, P<.01).Patients with HLHS and preserved RV systolic function have normal electrical activation when compared to patients with normal right and left ventricles. In contrast, these patients demonstrate mechanical dyssynchrony compared to patients with normal right and left ventricles. This finding raises important questions about the indications for cardiac resynchronization therapy in this patient population.
View details for DOI 10.1016/j.hrthm.2012.08.031
View details for Web of Science ID 000311791900029
View details for PubMedID 23085485
Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome OXFORD UNIV PRESS INC. 2012: 235-241
Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a complex congenital heart defect that has undergone significant advances in treatment over the past 15 years. A small subset of patients with PA/MAPCAs have associated Alagille syndrome, which can have an adverse impact on many other organ systems. The purpose of this study was to review our institutional outcomes for the surgical patients with PA/MAPCAs and Alagille syndrome.This was a retrospective review of patients with PA/MAPCA's and Alagille who underwent surgical reconstruction from November 2001 to August 2011. Fifteen patients were identified in our data base. Thirteen had pulmonary atresia with ventricular septal defect (PA/VSD) and two had pulmonary atresia with intact ventricular septum (PA-IVS).There has been no early or late mortality in this cohort of 15 patients with PA/MAPCA' and Alagille syndrome. The patients have undergone a total of 38 cardiac surgical procedures. Ten of the 13 patients with PA/VSD have achieved complete repair, including unifocalization, a right ventricle to pulmonary artery conduit and closure of all intra-cardiac shunts. The three unrepaired patients with PA/VSD remain potential candidates for eventual complete repair, while the two patients with PA-IVS remain viable candidates for a single ventricle pathway. The patients in this series have also undergone 12 major non-cardiac procedures.The data demonstrate that surgical reconstruction of PA/MAPCAs can be successfully achieved in patients with Alagille syndrome. The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome.
View details for DOI 10.1093/ejcts/ezr310
View details for Web of Science ID 000306365600013
View details for PubMedID 22402453
Supine Exercise Echocardiographic Measures of Systolic and Diastolic Function in Children JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2012; 25 (7): 773-781
Echocardiography has been used to determine ventricular function, segmental wall motion abnormality, and pulmonary artery pressure before and after peak exercise. No prior study has investigated systolic and diastolic function using echocardiography at various phases of exercise in children. The aim of this study was to determine the fractional shortening (FS), systolic-to-diastolic (S/D) ratio, heart rate-corrected velocity of circumferential fiber shortening (VCFc), circumferential wall stress (WS), ratio of mitral passive inflow to active inflow (E/A), ratio of passive inflow by pulsed-wave to tissue Doppler (E/E'), and right ventricular-to-right atrial pressure gradient from tricuspid valve regurgitation jet velocity (RVP) and time duration at various phases of exercise in children.In an 8-month period (December 2007 to July 2008), 100 healthy children were evaluated, and 97 participants aged 8 to 17 years who performed complete cardiopulmonary exercise stress tests using supine cycle ergometry were prospectively enrolled. The participants consisted of 48 female and 49 male subjects with various body sizes, levels of exercise experience, and physical capacities. The cardiopulmonary exercise stress test consisted of baseline pulmonary function testing, continuous gas analysis and monitoring of blood pressure and heart rate responses, electrocardiographic recordings, and oxygen saturation measurement among participants who pedaled against a ramp protocol based on body weight. All participants exercised to exhaustion. Echocardiography was performed during exercise at baseline, at a heart rate of 130 beats/min, at a heart rate of 160 beats/min, at 5 min after exercise, and at 10 min after exercise. FS, S/D ratio, VCFc, WS, E/A, E', E/E', and RVP at these five phases were compared in all subjects.All echocardiographic parameters differed at baseline from 160 beats/min (P < .0001) except E/E', which remained at 5.4 to 5.8. Specifically, FS (from 37% to 46%), S/D ratio, VCFc (from 1.1 to 1.6), WS (from 200 to 258 g/cm(2)), E' (from 0.2 to 0.3), and RVP (from 18 to 35 mm Hg) increased from baseline to 160 beats/min and then subsequently decreased to at or near baseline, while tricuspid valve regurgitation duration decreased (from 370 to 178 msec).Normal values for systolic and diastolic echocardiographic measurements of function are now available. FS, VCFc, WS, and RVP increase with exercise and then return to near baseline levels. The E/E' ratio is unaltered with exercise in normal subjects.
View details for DOI 10.1016/j.echo.2012.03.007
View details for Web of Science ID 000305701600013
View details for PubMedID 22521368
Tissue Doppler-Derived Measurement of Isovolumic Myocardial Contraction in the Pediatric Population PEDIATRIC CARDIOLOGY 2012; 33 (5): 720-727
Multiple echocardiographic techniques have been utilized to quantify systolic function. The shortening and ejection fraction remain the most commonly used and accepted methods. However, these measures are affected by altered loading conditions, and are not applicable when ventricular geometry differs from the prolate ellipsoid typical of a left ventricle. Mitral valve annular acceleration during isovolumic contraction (IVA) has been proposed as a load independent index of left ventricular contractility. However, published values for IVA demonstrating normal function vary. In addition, the value of IVA which may discern impaired systolic function has not been established. The purpose of this study is to determine a threshold IVA value for abnormal left ventricular function in the pediatric population. Structurally/functionally normal control (n = 90) and dilated cardiomyopathy (study = 64) patients were compared for differences in left ventricular: wall stress (WS), velocity of circumferential fiber shortening (VCFc), ejection fraction (EF), ejection force, and pulsed wave-derived medial and lateral wall IVA. No difference in body surface area (p = 0.61) or gender (p = 0.53) was noted. Left ventricular ejection fraction, ejection force, VCFc, and IVA were significantly lower and WS was significantly higher in the study group (p < 0.01). The medial IVA was 1.71 0.89 m/s(2) for an EF <40%, 1.74 0.70 m/s(2) for an EF = 40-50%, 2.46 0.89 m/s(2) for an EF >50%. The lateral IVA was 1.81 1.03 m/s(2) for an EF <40%, 2.07 0.78 m/s(2) for an EF = 40-50%, 2.54 0.99 m/s(2) for an EF >50%. ROC analysis demonstrated a medial IVA of 1.97 m/s(2) as the cut-off for predicting an EF <50% with a 77% sensitivity of and specificity of 66% (AUC = 0.75, CI = 0.67-0.83, p < 0.01). ROC analysis demonstrated a lateral IVA of 2.31 m/s(2) as the cut-off for predicting an EF <50% with a 73% sensitivity of and specificity of 63% (AUC = 0.72, CI = 0.63-0.82, p < 0.01). IVA lateral of 1.93 m/s(2) or less was associated with heart transplant and death. ICC analysis demonstrated some interobserver variability in IVA measurement (0.57-0.65). The normal IVA of the medial and lateral mitral valve annulus measure over 1.97 m/s(2) and 2.31 m/s(2), respectively; values less than this cut-off are associated with EF <50%. Despite some problems with reproducibility IVA remains a promising method of screening for diminished ventricular contractility in the setting of abnormal geometry.
View details for DOI 10.1007/s00246-012-0200-4
View details for Web of Science ID 000304458900006
View details for PubMedID 22349669
Imaging of the Aorta: Embryology and Anatomy SEMINARS IN ULTRASOUND CT AND MRI 2012; 33 (3): 169-190
This review focuses on the embryology and anatomy of the aorta with some imaging examples. Dividing the aorta by segments of unique function and embryogenesis facilitates organizing the group of potential anomalies encountered. A basic understanding of the embryologic development of the aorta and its major branches helps in understanding and recognizing typical and atypical anatomic findings. Diagnostic imaging of the aorta and its major branches can be accomplished by invasive and noninvasive methods, based on the clinical scenario and the age of the patient. In this review, computed tomography and magnetic resonance imaging examples are emphasized.
View details for DOI 10.1053/j.sult.2012.01.013
View details for Web of Science ID 000305094700002
View details for PubMedID 22624964
Is There a Rote for Cardiac Resynchronization Therapy in Pediatric Pulmonary Hypertension? ELSEVIER SCIENCE INC. 2012: S29-S29
Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot ANNALS OF THORACIC SURGERY 2012; 93 (2): 677-679
Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.
View details for DOI 10.1016/j.athoracsur.2011.09.016
View details for Web of Science ID 000299540200069
View details for PubMedID 22269747
Echocardiographic Diagnosis and Prognosis of Fetal Left Ventricular Noncompaction JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2012; 25 (1): 112-120
Left ventricular noncompaction (LVNC) has rarely been described in the fetus.The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments-four segments each at the base, midpapillary, and apical regions-in the short-axis view to calculate the noncompaction/compaction ratio for each segment.Of 24 fetuses with LVNC included in the study, 22 had significant congenital heart disease, and 15 had complete heart block. Of the 16 patients with adequate follow-up and not electively terminated, 12 (81%) died or progressed to heart transplantation. The average noncompaction/compaction ratios were 2.02 in patients who died or underwent heart transplantation and 1.67 in survivors (P = .2034). Fifty-seven of 93 measured segments (61%) of the left ventricle in the patients who died or underwent heart transplantation had noncompaction/compaction ratios ? 2 compared with five of 17 measured segments (29%) in survivors (P = .0837). The average cardiovascular profile score was 6. The apical region had greater involvement of noncompaction than the midpapillary and basal regions, with ratios of 2.27, 2.14, and 1.10, respectively (P = .00035).Fetuses with LVNC have a poor prognosis that may be related to associated congenital heart disease, increased segmental involvement of noncompaction, and complete heart block and can be predicted by the cardiovascular profile score.
View details for DOI 10.1016/j.echo.2011.09.019
View details for Web of Science ID 000298276500013
View details for PubMedID 22014428
Novel Electrocardiogram Manifestations of a Tension Pneumothorax PEDIATRIC CARDIOLOGY 2011; 32 (8): 1258-1259
Surgical Results in Patients With Pulmonary Atresia-Major Aortopulmonary Collaterals in Association With Total Anomalous Pulmonary Venous Connection ANNALS OF THORACIC SURGERY 2011; 92 (5): 1756-1760
Pulmonary atresia and major aortopulmonary collaterals (PA/MAPCAs) is a complex form of congenital heart disease. One to two percent of patients with PA/MAPCAs will also have total anomalous pulmonary venous connection (TAPVC). This study summarizes our surgical experience with this rare combination of life-threatening congenital heart defects.A retrospective review was performed to identify patients who had surgery for PA/MAPCAs in association with TAPVC. From November 2001 to March 2011, 9 patients presented with this combination of defects. Eight of the 9 patients had heterotaxy with an unbalanced atrioventricular canal and functional single ventricle. The ninth patient had double outlet right ventricle (two ventricles). Timing of surgical intervention was typically predicated on the degree of pulmonary venous obstruction. The median age at surgery was 14 days. All nine patients had surgical correction of TAPVC, unifocalization of MAPCA's into a central confluence, and placement of a shunt.There was one early mortality (< 30 days) and two late mortalities. For the 6 survivors, 5 have subsequently undergone a bidirectional Glenn procedure, and 3 had completion of their Fontan. Two patients are currently at the bidirectional Glenn stage; one is a good candidate for Fontan completion while the other is not suitable. The sixth patient is awaiting further assessment.The PA/MAPCAs, in association with TAPVC, is a challenging combination of defects. The data suggest that the combination of PA/MAPCAs and TAPVC can be undertaken with a reasonable midterm prognosis.
View details for DOI 10.1016/j.athoracsur.2011.06.020
View details for Web of Science ID 000296925400045
View details for PubMedID 21944736
QTc prolongation in children following congenital cardiac disease surgery CARDIOLOGY IN THE YOUNG 2011; 21 (4): 400-410
IntroductionQTc prolongation has been reported in adults following cardiopulmonary bypass; however, this phenomenon has not been studied in children with congenital cardiac disease. This study's aim was to formally assess QTc in children undergoing cardiac surgery.Pre-operative and post-operative electrocardiograms during hospital stays were prospectively analysed on 107 consecutive patients under 18 years of age undergoing cardiac surgery. QTc was measured manually in leads II, V4, and V5. Measurements of 440 and 480 milliseconds were used to categorise patients. Peri-procedural data included bypass and cross-clamp time, medications, and electrolyte measurements. Outcome data included arrhythmias, length of mechanical ventilation, and hospital stay. Patients with post-operative new bundle branch block or ventricularly paced rhythm were excluded.In all, 59 children were included, out of which 26 had new QTc over 440 milliseconds and 6 of 59 had new QTc over 480 milliseconds post-operatively. The mean increase in post-operative QTc was 25 milliseconds, p=0.0001. QTc over 480 was associated with longer cross-clamp time, p=0.003. Other risk factors were not associated with post-operative QTc prolongation. This phenomenon was transient with normalisation occurring in 67% of patients over 60 hours on average. One patient with post-operative QTc over 440 milliseconds developed ventricular tachycardia. There was no correlation between prolonged QTc and duration of mechanical ventilation, or hospital stay.ConclusionA significant number of children undergoing cardiac surgery showed transient QTc prolongation. The precise aetiology of QT prolongation was not discerned, though new QTc over 480 milliseconds was associated with longer cross-clamp time. In this cohort, transient QTc prolongation was not associated with adverse sequela.
View details for DOI 10.1017/S1047951111000175
View details for Web of Science ID 000293593800005
View details for PubMedID 21362209
Fetal Predictors of Urgent Balloon Atrial Septostomy in Neonates with Complete Transposition JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2011; 24 (4): 425-430
In complete transposition of the great vessels, a restrictive patent foramen ovale leads to inadequate circulatory mixing and severe cyanosis. Urgent balloon atrial septostomy (BAS) improves mixing and bridges neonates to surgery. Several studies have determined risk factors in utero for poor postnatal outcomes in complete transposition of the great vessels, particularly a restrictive patent foramen ovale and ductus arteriosus. In addition to these risk factors, we studied two new features, a hypermobile septum and reverse diastolic patent ductus arteriosus shunt, to determine which patients will require an urgent BAS.We reviewed all 26 fetuses from 2001 to 2010 with complete transposition of the great vessels and closely examined the patent foramen ovale and septum primum for hypermobility, restriction, flat appearance, or redundancy. We defined hypermobility as a septum primum flap that oscillates between both atria. We also examined the ductus size and shunting pattern to evaluate whether these features contributed to urgent BAS.In total, 14 of 26 fetuses required urgent BAS with improved cyanosis. Nine fetuses had an urgent BAS and a hypermobile septum, and 12 fetuses had no urgent BAS or hypermobile septum. Eight fetuses had an urgent BAS and a reverse diastolic patent ductus arteriosus, and 11 fetuses had no urgent BAS or reverse diastolic patent ductus arteriosus. A hypermobile septum and reverse diastolic patent ductus arteriosus had a significant association with urgent BAS (P < .01, sensitivity= 0.64 and 0.57, specificity= 1.0 and 0.92, positive predictive value= 1.0 and 0.89, negative predictive value= 0.71 and 0.65). No fetus had a restrictive patent foramen ovale/ductus arteriosus.A hypermobile septum and reverse diastolic patent ductus arteriosus are new prenatal findings to help predict the need for an urgent BAS postnatally in patients with complete transposition of the great vessels.
View details for DOI 10.1016/j.echo.2010.12.020
View details for Web of Science ID 000288781700011
View details for PubMedID 21324642
The Echocardiographic Evaluation of Left Ventricular Non-compaction Pediatric Ultrasound Today 2011; 16 (6): 109-28
Annular Tilt as a Screening Test for Right Ventricular Enlargement in Patients with Tetralogy of Fallot JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2010; 23 (12): 1297-1302
Right ventricular end-diastolic volume (RVEDV) greater than 150 mL/m is a risk factor for sudden death in patients with tetralogy of Fallot (TOF) after repair. Because of its anterior placement and abnormal geometry, two-dimensional echocardiography is limited to a qualitative assessment of RVEDV. Cardiac magnetic resonance imaging (CMRI) and computed tomography angiography (CTA) are the accepted standards for quantifying RVEDV. This study evaluated the ability of a novel echocardiographic measure, the right ventricular annular tilt (RVAT), to identify patients with increased RVEDV.All patients with repaired TOF with an echocardiogram and CMRI or CTA were included in this retrospective study. The RVAT was determined by measuring the angle of the tricuspid valve plane relative to the mitral valve plane at end-diastole in the apical 4-chamber view in study (n = 38) and age-matched control (n = 74) patients. The RVEDV measurements were obtained by CMRI (n = 32) or CTA (n = 6). The study and control patients' ages were no different (11.3 and 11.8 years, P = .73).The study group RVAT was significantly higher than the control group RVAT (17.4 vs. 0.1 degrees; P < .0001). RVAT values greater than 20 degrees had a mean RVEDV of 166 60 mL/m, whereas RVAT less than 20 degrees had a mean RVEDV of 122 25 mL/m (P = .0370). Receiver operating characteristic analysis demonstrated an RVAT of 17.9 degrees as the cutoff for predicting a RVEDV of greater than 150 mL/m with a sensitivity of 75% and specificity of 73% (area under the curve = 0.76; confidence interval, 0.56-0.96; P = .0063). Intraclass correlation analysis demonstrated minimal interobserver and intraobserver variability when measuring RVAT (0.99 and 0.92).An RVAT less than 20 degrees is associated with an RVEDV less than 150 mL/m. RVAT is a useful echocardiographic technique for detecting increased RVEDV in patients with TOF and may help discern which patients should undergo RVEDV quantification by CMRI or CTA.
View details for DOI 10.1016/j.echo.2010.09.002
View details for Web of Science ID 000284624900012
View details for PubMedID 20950999
Newborn with Persistent Truncus Arteriosus and Interrupted Aortic Arch Demonstrating Reverse Left Subclavian Artery Flow PEDIATRIC CARDIOLOGY 2010; 31 (8): 1254-1256
Anomalies Associated With a Prominent Azygos Vein on Echocardiography in the Pediatric Population JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2010; 23 (3): 282-285
Prominent azygos veins (PAVs) have been described with interrupted inferior venae cavae (IVCs) with heterotaxy. At the authors' institution, cases of PAVs with uninterrupted IVCs have been noted. The aim of this study was to determine the occurrence rate of PAVs and associated lesions by echocardiography.All patients with PAVs were collected; those with interrupted and uninterrupted IVCs were assigned to groups 1 and 2. Normal controls were assigned to group 3.Among 15,849 patients from January 1, 2001, to March 31, 2008, 55 (0.3%) had PAVs, 42 (76%) in group 1 and 13 (24%) in group 2. Heterotaxy was prominent in group 1, whereas patients in group 2 had no heterotaxy. Patients in group 2 had more structurally normal hearts than those in group 1 (46% vs 14%, P<.01), partial anomalous pulmonary venous return, and one single ventricle. IVC measurements were the same in groups 2 and 3 (P=.65).This study demonstrates that a PAV without IVC interruption is not associated with heterotaxy. Patients with PAVs should be carefully examined for partial anomalous pulmonary venous return.
View details for DOI 10.1016/j.echo.2009.11.025
View details for Web of Science ID 000275221900007
View details for PubMedID 20138465
Cardiac Segmental Analysis in Left Ventricular Noncompaction: Experience in a Pediatric Population JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY 2010; 23 (1): 46-53
Echocardiography has been used to diagnose and describe left ventricular noncompaction (LVNC). No other study has investigated LVNC using the 16-segment model described by the American Heart Association and the American Society of Echocardiography in children, some of whom have congenital heart disease. Using the ratio of noncompaction to compaction, the authors analyzed the 16 segments and determined if severity was correlated with poor outcomes in a pediatric population.The 16-segment noncompaction/compaction ratio, shortening, and ejection fractions were measured retrospectively in all children with LVNC at a single institution from January 1, 2000, to June 30, 2008.Forty-four patients had LVNC, an incidence of 0.3% of laboratory admissions. Twenty-eight patients (64%) who remained alive were assigned to group 1, and 16 patients (36%) who either died or were transplanted constituted group 2. Group 2 had more patients with significant associated congenital heart disease than group 1 (50% vs 18%, P < .05). We found similar regions of involvement in the 16-segment model with sparing of basal segments and involvement of the midpapillary and apical regions (P < .001); however, patients in group 2 were noted to have more segments involved (6 vs 4, P < .05), lower shortening fractions (16% vs 29%, P < .001), and lower ejection fractions (24% vs 47%, P < .001). The ejection fraction was inversely related to the number of segments (r = -0.63, P < .01), suggesting that more noncompaction portends a worse outcome.In younger patients with noncompaction, poor outcomes such as low ejection fractions, death, and transplantation are related to the number of left ventricular segments involved. There is more associated congenital heart disease in the pediatric population, which carries a poorer prognosis than the disease reported in adult populations.
View details for DOI 10.1016/j.echo.2009.09.003
View details for Web of Science ID 000273052600011
View details for PubMedID 19857942
Echocardiography in patients with left ventricular inflow lesions. Pediatric Ultrasound Today. 2008; 13 (1): 3-23
Case report: Rhabdomyolysis induced by mibefradil in a patient treated with cyclosporine and simvastatin JOURNAL OF CLINICAL PHARMACOLOGY 1999; 39 (3): 310-312