Short-term results in infants with multiple left heart obstructive lesions.

OBJECTIVE: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success. DESIGN: Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size, and left (LV) and right (RV) ventricle diastolic longitudinal dimensions. The valve morphology and presence of a ventricular septal defect (VSD) and coarctation were assessed. Clinical outcomes included successful 2V repair, complications, and repeat interventions or surgeries. Failed 2V repair was defined as a takedown to single ventricle (1V) physiology, cardiac transplantation, or death. RESULTS: For 2V repair, 14/19 patients were selected and for 1V, 5/19 patients were selected. Initial surgical procedures of the 2V group were simple coarctation repair (5), complex coarctation/arch reconstruction +/- septal defect closure (6), hybrid stage 1 (2), and none (1). Three of the 2V patients required reintervention in the first 90 days. The LV to RV diastolic longitudinal ratio >0.75 and mitral/tricuspid ratio of <0.8 were observed in 13/14 of the 2V patients. The LV:RV ratio and the aortic valve z score were significantly larger in the 2V group compared to the 1V group. All patients in the 1V group had a nonapex forming LV. There was no mortality with follow-up to three years of age. CONCLUSIONS: This study showed excellent short-term and midterm surgical results in the 2V population. The LV:RV diastolic longitudinal ratio may be a useful tool in the risk stratification of a successful 2V repair even in cases with a small MV.

Obesity trends in children, adolescents, and young adults with congenital heart disease.

OBJECTIVES: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD). STUDY DESIGN: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution. Patients were categorized by age and CHD diagnosis. The date of last normal weight for age and the date of first recorded weight in the range of overweight and obese were documented. RESULTS: Nine hundred sixty-eight patients with CHD were included. The prevalence of overweight and obesity was 31.5% and 16.4%, respectively. For patients who became overweight or obese, the last recorded normal weight was between 6 and 10 years of age. Electrophysiologic disease and older age were risk factors for obesity. CONCLUSIONS: Children with CHD have an increasing risk of becoming overweight and obese in early childhood. This study provides important information and identifies critical period to implement preventative measures and counsel families about the risk of obesity in CHD.

Systolic and diastolic properties of univentricular hearts in children: insights from physiologic indices that reflect calcium cycling.

Physiologic indices that reflect intracellular Ca2+ cycling were chosen to evaluate contraction and relaxation properties of the univentricular heart. We hypothesized that these indices would be impaired in univentricular hearts. With advances in surgical palliation, an increasing number of children are surviving with univentricular hearts supporting the systemic circulation. Similar to the adult failing heart, single ventricles may also manifest impaired Ca2+ cycling, which may have important therapeutic implications. In our study, we did not actually measure Ca2+ uptake or transients in the cardiac myocyte. Rather, we used previously validated physiologic indices that are known to reflect Ca2+ cycling. Sixteen children were studied, eight with single ventricles (SV) and eight as matched control subjects. Systolic properties were studied using maximal derivative of ventricular pressure (dP/dtmax), force-frequency relationship, and mechanical restitution. Diastolic properties were assessed using time constant of relaxation (tau) and the relaxation-frequency relationship. The critical HR (HRcrit) was assessed from the force-frequency relationship and relaxation-frequency relationship. DP/dtmax and tau were calculated from micromanometric tracings at increasing HRs, generated by right atrial pacing. In SV patients, dP/dtmax was lower than in the control group at each matched HR, and the force-frequency relationship was shifted downward. Restitution of contractility was slower in patients with SV. Tau was similar in both groups at lower HRs but significantly prolonged in the SV group at faster HRs. In the SV, HRcrit was significantly shifted to the left. These findings indicate impaired systolic and diastolic properties of univentricular heart, especially at increased HRs. Because these physiologic indices reflect Ca2+ cycling, it is speculated that the phenomenon of Ca2+ cycling may be impaired in the myocytes of univentricular hearts.