[Show abstract][Hide abstract] ABSTRACT: Infants with shunt-dependent single-ventricle (SV) physiology are at risk for poor weight gain before superior cavopulmonary connection (SCPC). Lower weight-for-age z-score is a risk factor for prolonged length of stay (LOS) after SCPC. We sought to characterize infant growth and feeding and determine the effect of method of feeding on outcomes. Chart review of infants with shunt-dependent SV physiology born between October 2007 and September 2010 was performed. The cohort was divided into three groups based on feeding method at discharge after initial palliation; 53 in the oral feeding (PO) group, 56 in the nasogastric (NG) tube group, and 26 in the gastrostomy tube (GT) group. Birth weight z-score did not differ among groups (p = 0.39), but infants fed by NG or GT were smaller than PO-fed infants at hospital discharge (p = 0.0001), a difference that persisted through SCPC (p < 0.0001). Predictors of need for GT included Norwood procedure (p = 0.008) and longer LOS after initial palliation (p < 0.001). Interstage mortality and age at SCPC did not differ among groups. Risk factors for longer LOS at SCPC included longer LOS and need for supplemental feeds at discharge from initial palliation as well as lower weight at SCPC. Poor growth is common among infants with shunt-dependent SV physiology. Infants who require GT have lower weight-for-age z-scores at hospital discharge and remain smaller at SCPC than those fed PO. Although GT after initial palliation is associated with longer LOS after SCPC, it is not associated with an increase in interstage morbidity or mortality.
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVE: The objective of this study was to describe characteristics and early outcomes across a large multicenter cohort undergoing coarctation or hypoplastic aortic arch repair. METHODS: Patients undergoing coarctation or hypoplastic aortic arch repair (2006-2010) as their first cardiovascular operation in the Society of Thoracic Surgeons Congenital Heart Surgery Database were included. Group 1 patients consisted of those with coarctation or hypoplastic aortic arch without ventricular septal defect (coarctation or hypoplastic aortic arch, isolated); group 2, coarctation or hypoplastic aortic arch with ventricular septal defect (coarctation or hypoplastic aortic arch, ventricular septal defect); and group 3, coarctation or hypoplastic aortic arch with other major cardiac diagnoses (coarctation or hypoplastic aortic arch, other). RESULTS: The cohort included 5025 patients (95 centers): group 1, 2705 (54%); group 2, 840 (17%); and group 3, 1480 (29%). Group 1 underwent coarctation or hypoplastic aortic arch repair at an older age than groups 2 and 3 (groups 1, 2, and 3, 75%, 99%, and 88% <1 year old, respectively; P < .0001). The most common operative techniques for coarctation or hypoplastic aortic arch repair (group 1) were end-to-end (33%) or extended end-to-end (56%) anastomosis. Overall mortality was 2.4%, and was 1%, 2.5%, and 4.8% for groups 1, 2, and 3 respectively (P < .0001). Ventricular septal defect management strategies for group 2 patients included ventricular septal defect closure (n = 211, 25%), pulmonary artery band (n = 89, 11%), or no intervention (n = 540, 64%) without significant difference in mortality (4%, 1%, 2%; P = .15). Postoperative complications occurred in 36% of patients overall and were more common in groups 2 and 3. There were no occurrences of spinal cord injury (0/973). CONCLUSIONS: In the current era, primary coarctation or hypoplastic aortic arch repair is performed predominantly in neonates and infants. Overall mortality is low, although those with concomitant defects are at risk for higher morbidity and mortality. The risk of spinal cord injury is lower than previously reported.
The Journal of thoracic and cardiovascular surgery 10/2012; · 3.41 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Purpose: Infants with shunt-dependent single ventricle (SV) physiology are at risk for poor weight gain prior to Stage 2 palliation. Lower weight for age z-score is a risk factor for prolonged hospital stay after Stage 2. We evaluated the impact of mode of feeding on inter-stage weight gain.
Methods: A chart review was performed of all infants with shunt-dependent single ventricle physiology born between 8/07 and 9/10 who survived to hospital discharge after initial palliation. Z-scores were calculated based on World Health Organization data.
Table 1. Differences based on type of feeding regimen at hospital discharge following initial shunt or Stage 1 palliation
PO (n=53, 39.3%)
NG (n=57, 42.2%)
GT (n=25, 18.5%)
Length of hospital stay (days)
Birth weight (kg)
Birth weight z-score
Weight at discharge (kg)
Weight at discharge (z-score)
Change in weight z-score from birth to discharge
Weight at Stage 2 (kg)
Weight at Stage 2 (z-score)
Change in weight z-score from discharge to Stage 2
Age at Stage 2 (days)
Birth weight z-score did not differ among the three groups (p=0.47). However, infants fed by nasogastric or gastrostomy tube were smaller than orally fed infants at hospital discharge (p=0.0001), a difference that persisted through Stage 2 (p<0.0001). Infants who underwent a Norwood-type reconstruction were more likely to receive NG or G-tube feeds (p=0.004). Interstage mortality and age at Stage 2 did not differ among the three groups.
Conclusion: Infants with shunt-dependent SV physiology have significant weight loss from birth to initial hospital discharge. Growth failure persists in the interstage period before stage 2, and is not mitigated by tube feeds. Growth failure in this patient population is likely multifactorial, and close monitoring of nutritional status should continue in the interstage period.
2012 American Academy of Pediatrics National Conference and Exhibition; 10/2012
[Show abstract][Hide abstract] ABSTRACT: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect that usually presents before the age of 1 year. Several surgical options exist for the correction of ALCAPA; however, debate continues regarding the optimal repair technique in adult populations. We report the case of successful surgical repair of ALCAPA with a direct aortic implantation technique in a 44-year-old mother of 4 children.
Heart Surgery Forum 10/2012; 15(5):E284-5. · 0.56 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Twin-twin transfusion syndrome (TTTS) is a complex disorder with altered cardiovascular loading conditions that affects both donors and recipients. Myocardial tissue deformation analysis using vector velocity imaging is an angle-independent, speckle-tracking technique which can assess myocardial mechanics and may provide insight into cardiac dysfunction in TTTS.
Digital dynamic two-dimensional four-chamber views were interrogated offline. Images were acquired utilizing standard video frame rates (30 frames/s). The global longitudinal strain, systolic strain rate, and diastolic strain rate were measured in the left (LV) and right ventricles (RV) of 25 fetal pairs with TTTS and compared to 25 gestational age-matched normal controls. Pulsatility indices for the umbilical artery and middle cerebral artery were measured.
The gestational age at evaluation was 20.5 ± 1.3 weeks. The donor LV systolic strain rate was higher, while the donor RV diastolic strain rate was significantly lower, than control values. The recipient longitudinal strain, systolic strain rate, and diastolic strain rate were significantly lower for both LV and RV in comparison to controls. The donor umbilical artery pulsatility index was higher than control values (1.92 ± 0.45 vs. 1.41 ± 0.25, p < 0.001), while the donor middle cerebral artery pulsatility index was lower (1.46 ± 0.28 vs. 1.87 ± 0.21). Recipient umbilical artery and middle cerebral artery pulsatility indices were no different than control values.
In TTTS, both the donor and the recipient exhibit abnormalities of myocardial tissue deformation with ventricle-specific changes evident based on loading conditions. Donor LV systolic function is hyperdynamic due to hypovolemia and selective ejection into a low-resistance cerebrovascular circuit while the donor RV selectively ejects into a high-resistance placental circuit. Recipient RV and LV are both globally depressed with systolic and diastolic dysfunction. Further prospective validation of our findings using high frame rate analysis is indicated.
Fetal Diagnosis and Therapy 05/2012; 32(1-2):131-7. · 2.30 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In anomalous left coronary artery from the pulmonary artery (ALCAPA), infants 6 to 12 weeks will often present with symptoms consistent with reflux or bronchiolitis. In those infants diagnosed with both ALCAPA and concomitant active respiratory syncytial virus (RSV) bronchiolitis, others have reported delaying revascularization therapy until resolution of the RSV bronchiolitis. Here, we report the case of a three-month-old infant, diagnosed with ALCAPA and active RSV bronchiolitis, who underwent successful myocardial revascularization within 24 hours of presentation and diagnosis.
World journal for pediatric & congenital heart surgery. 04/2012; 3(2):267-270.
[Show abstract][Hide abstract] ABSTRACT: Outcomes in patients with ventricular dysfunction undergoing superior cavopulmonary connection (SCPC) are not well known. We reviewed records of patients undergoing SCPC at our center from December 2005 to October 2009 and studied those whose pre-SCPC echocardiograms demonstrated at least moderate systemic ventricular dysfunction. Of the 213 patients undergoing SCPC, 19 (9%) met inclusion criteria. Diagnoses were hypoplastic left heart syndrome (n = 18) and rightward unbalanced atrioventricular canal with pulmonary stenosis (n = 1). In those surviving >2 months after SCPC, ventricular function was assessed by echocardiography 4.9 (range 3.5 to 9.7) months after SCPC and was improved in ten of 17 (59%), unchanged in six of 17 (35%), and worsened in one of 17 (16%) patients. After SCPC, three patients died, and one underwent heart transplant 21.9 months after SCPC. Transplant-free survival was attained by 15 of 19 (79%) patients during follow-up of 33.0 months (range 10.8 to 51.4). Fontan completion was performed on six survivors on reaching an appropriate age. Ventricular dysfunction before SCPC is not uncommon and occurs primarily in patients with a morphologic right ventricle. Ventricular performance improves in the majority of patients after SCPC, and midterm outcomes are comparable with previous reports, suggesting that these patients remain candidates for staged palliation.
[Show abstract][Hide abstract] ABSTRACT: Previous studies have demonstrated a correlation between E:E(a) and ventricular filling pressure in adults after heart transplantation. We sought to determine if E:E(a) correlates with filling pressure after heart transplantation in children. A prospective analysis of children who have undergone heart transplantation was performed. Inflow and myocardial velocities were recorded and compared to catheter-derived filling pressures and rejection status. We performed 61 studies in 49 subjects. No correlation was found between septal E:E(a) and PCWP (r=0.14, p=0.28); or between lateral tricuspid E:E(a) and mean RAp (r=0.04, p=0.79). However, the mean PCWP was higher among subjects with elevated septal E:E(a) (>12) compared to normal E:E(a) (12.3±2.8mmHg vs. 10.1±2.9mmHg, p=0.02). Similarly, mean RAp was higher among subjects with an elevated lateral tricuspid E:E(a) (>10) compared to normal lateral tricuspid E:E(a) (7.7±2.1mmHg vs. 6.0±2.4mmHg, p=0.04). Elevated septal E:E(a) was also associated with high-grade cellular rejection (OR=17.3 [95% CI 1.4-221], p=0.028). In children following heart transplant, E:E(a) does not correlate well with the range of filling pressures seen after pediatric heart transplantation. However, a septal E:E(a)>12 is associated with elevated PCWP and high grade cellular rejection and a lateral tricuspid E:E(a)>10 is associated with elevated mean RAp.
[Show abstract][Hide abstract] ABSTRACT: To determine whether the mode of delivery of infants prenatally diagnosed with hypoplastic left heart syndrome (HLHS) affects markers of perinatal hemodynamics.
A retrospective review of patients diagnosed prenatally with HLHS and delivered within our institution was undertaken. Arterial blood gases, echocardiographic data, and markers of end organ function were compared based on route of delivery.
A total of 79 infants with HLHS were enrolled between January 2002 and December 2008. The infants delivered by elective cesarian delivery (CD) had younger gestational age compared with those delivered by vaginal delivery (VD) or by urgent CD/operative VD. Those delivered by elective CD had lower pH and higher partial pressure of CO(2) on arterial cord blood gas analysis. There were no differences in partial pressure of O(2) and base deficit among the 3 study groups. One-minute and 5-minute Apgar scores, markers of end organ function, echocardiographic parameters, length of hospitalization, and survival to discharge were similar among the groups.
Overall, newborns with a prenatal diagnosis of HLHS transitioned well to extrauterine life without significant acidosis regardless of the mode of delivery. Delivery of newborns with HLHS by elective CD did not demonstrate any hemodynamic advantage over VD in our cohort of patients.
The Journal of pediatrics 03/2011; 159(1):64-9. · 4.02 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Serum brain-type natriuretic peptide level (BNP) correlates with hemodynamic parameters measured during cardiac catheterization in adult patients with heart failure. We sought to describe the relationship of BNP with invasive hemodynamic measurements and cellular rejection in children following OHT. Children undergoing catheterization for OHT surveillance had simultaneous measurement of BNP. A total of 62 subjects were studied. The median BNP was 171 pg/mL (range 19-1130). There were significant positive correlations between BNP and mean PAP (R=0.33, p=0.009), RVSP (R=0.25, p=0.05), RVEDP (R=0.29, p=0.02), and mean RAP (R=0.39, p=0.002). Rejection grade varied from 0 to 3A (58 patients < ISHLT 3A and four patients ≥ ISHLT 3A). There was no significant difference in BNP based on cellular rejection grade. In a cohort of pediatric patients after heart transplantation, BNP correlates with direct measurements of right-sided pressures, but not with other hemodynamic measurements, time from transplant or rejection grade. This suggests that BNP may have a complimentary role in the monitoring of children following heart transplantation.
[Show abstract][Hide abstract] ABSTRACT: The majority of previous studies that described the neuropsychological effects of cardiopulmonary bypass (CPB) in children were performed after surgery in infancy for complex congenital heart disease (CHD). We sought to limit confounding variables and isolate potential independent effects of CPB by describing neuropsychological function in school-aged children after repair of acyanotic CHD.
This was a prospective study of patients who were aged 5 to 18 years and undergoing repair of acyanotic CHD. Neuropsychological testing battery included assessment of intelligence, memory, motor, attention, executive function, and behavior before and 6 months after CPB. The independent effects of anesthesia, surgery, and hospitalization on neuropsychological function were assessed by testing a surgical control group of patients who were undergoing repair of pectus deformities. In addition, an outpatient group of children with mild CHD were enrolled to assess the practice effects of serial testing.
Patients included CPB (n = 35), surgical control (n = 19), and nonsurgical (n = 12). Groups were comparable in age, gender, and race and demonstrated similar unadjusted group mean scores on baseline and 6-month follow-up neuropsychological testing. When adjusted for practice effects, the CPB group performed similar to the non-CPB groups in all assessed neuropsychological domains, with the exception of 1 of 4 tests of executive function.
When controlling for the non-CPB effects of surgery (eg, hospitalization, anesthesia, thoracotomy) and the practice effects of serial testing, there were no consistent independent effects of CPB on neuropsychological status in a cohort of children and adolescents 6 months after repair of acyanotic CHD.
[Show abstract][Hide abstract] ABSTRACT: To review our experience with the prenatal diagnosis of hypoplastic left heart syndrome (HLHS). Our goal was to establish the benchmark for perinatal and early surgical outcome in the current era, from a center with an aggressive surgical approach and a cohort with a high level of intention-to-treat.
Outcome was assessed in fetuses with HLHS following stratification into high-risk and standard-risk categories. High risk was defined as the presence of any of the following: extracardiac, genetic or chromosomal anomalies; prematurity of < 34 weeks' gestation; additional cardiac findings such as intact or highly restrictive atrial septum, severe degree of tricuspid regurgitation or severe ventricular dysfunction. Standard risk was defined as absence of these risk factors.
Of 240 fetuses evaluated over 5 years, 162 (67.5%) were in the standard-risk group and 78 (32.5%) were in the high-risk group. Of the 240 sets of parents, 38 (15.8%) chose termination or non-intervention at birth at initial prenatal counseling and 185 of the neonates (77.1%) underwent first-stage Norwood surgery with 155 surviving and 30 deaths, giving an overall Norwood operative survival of 83.8%. Breakdown by risk class reveals a significant Norwood operative survival advantage for the standard-risk group (92.8%) over the high-risk group (56.5%) (P < 0.001).
Following prenatal diagnosis of HLHS, families should be strongly encouraged to undertake comprehensive prenatal evaluation in order to obtain an accurate prognosis. One-third have additional risk factors that limit survival outcome, however two-thirds do not and have an excellent chance of early survival.
Ultrasound in Obstetrics and Gynecology 04/2010; 36(4):465-70. · 3.56 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Prenatal ventricular size discrepancy with disproportionately smaller left ventricle than right ventricle (L-R/VD) can be a marker for important left-sided structural heart disease in the newborn.
We reviewed the echocardiograms of all fetuses evaluated at our center with L-R/VD from July 1, 2004 to January 1, 2008.
Of the 35 fetuses, 20 (57%) had critical arch obstruction and underwent neonatal intervention (group 1); 15 (43%) did not require newborn intervention (group 2). Ratios comparing left with right heart structures were significantly lower in group 1 fetuses compared with group 2 fetuses. Aortic arch measurement <or= 3 mm was the most sensitive, and abnormal direction of atrial level shunting was the most specific measure to predict the need for neonatal aortic arch intervention.
Ratios expressing the magnitude of L-R/VD, direction of flow at the atrial septum, and measurements of the aortic arch help identify fetuses that will require neonatal intervention.
Journal of the American Society of Echocardiography: official publication of the American Society of Echocardiography 10/2009; 22(11):1296-301. · 2.98 Impact Factor