Faculty Bibliography

In normal pediatric echocardiograms, the distance from the junction of superior vena cava (SVC) and right atrium to inferior vena cava (IVC) and right atrium is linearly related to height. We examine this relation in children listed for heart transplant with idiopathic dilated cardiomyopathy (IDC) compared with the previously defined normal distribution of SVC-IVC to improve matching of heart sizes. Measurements of SVC-IVC and left ventricular end-diastolic diameter in 55 pediatric patients with IDC were correlated with height, weight, and body surface area. Regression analyses were performed to find the best-fit equation and correlation coefficient. Generalized linear modeling compared SVC-IVC in patients with IDC with normal SVC-IVC values from 254 patients. There was a strong linear relation in patients with IDC between SVC-IVC and height (R2=0.84) and a logarithmic relation to weight (R2=0.80). Left ventricular end-diastolic diameter did not correlate with SVC-IVC or any other parameter. In 87% of patients with IDC, SVC-IVC was over 2 SDs above predicted normal values (mean z-score=4.3±2.1). In conclusion, predicted SVC-IVC in patients with IDC was different from published norms (p<0.001). SVC-IVC in pediatric patients with IDC, although linearly related to height, is consistently above normal values.

OBJECTIVES/OBJECTIVE:This study sought to determine the incidence and predictors of recovery of normal echocardiographic function among children with idiopathic dilated cardiomyopathy (DCM). BACKGROUND:Most children with idiopathic DCM have poor outcomes; however, some improve. METHODS:We studied children <18 years of age from the Pediatric Cardiomyopathy Registry who had both depressed left ventricular (LV) function (fractional shortening or ejection fraction z-score <-2) and LV dilation (end-diastolic dimension [LVEDD] z-score >2) at diagnosis and who had at least 1 follow-up echocardiogram 30 days to 2 years from the initial echocardiogram. We estimated the cumulative incidence and predictors of normalization. RESULTS:Among 868 children who met the inclusion criteria, 741 (85%) had both echocardiograms. At 2 years, 22% had recovered normal LV function and size; 51% had died or undergone heart transplantation (median, 3.2 months), and 27% had persistently abnormal echocardiograms. Younger age (hazard ratio [HR]: 0.92; 95% confidence interval [CI]: 0.88 to 0.97) and lower LVEDD z-score (HR: 0.78; 95% CI: 0.70 to 0.87) independently predicted normalization. Nine children (9%) with normal LV function and size within 2 years of diagnosis later underwent heart transplantation or died. CONCLUSIONS:Despite marked LV dilation and depressed function initially, children with idiopathic DCM can recover normal LV size and function, particularly those younger and with less LV dilation at diagnosis. Investigations related to predictors of recovery, such as genetic associations, serum markers, and the impact of medical therapy or ventricular unloading with assist devices are important next steps. Longer follow-up after normalization is warranted as cardiac failure can recur. (Pediatric Cardiomyopathy Registry; NCT00005391).

High pulmonary vascular resistance index (PVRI) can lead to right ventricular dysfunction and failure of the donor heart early after pediatric heart transplantation. Oral pulmonary vasodilators such as sildenafil have been shown to be effective modifiers of pulmonary vascular tone. We performed a retrospective, observational study comparing patients treated with sildenafil ("sildenafil group") to those not treated with sildenafil ("nonsildenafil group") after heart transplantation from 2007 to 2012. Pre- and posttransplant data were obtained, including hemodynamic data from right heart catheterizations. Twenty-four of 97 (25%) transplant recipients were transitioned to sildenafil from other systemic vasodilators. Pretransplant PVRI was higher in the sildenafil group (6.8 ± 3.9 indexed Woods units [WU]) as compared to the nonsildenafil group (2.5 ± 1.7 WU, p=0.002). In the sildenafil group posttransplant, there were significant decreases in systolic pulmonary artery pressure, mean pulmonary artery pressure, transpulmonary gradient and PVRI (4.7 ± 2.9 WU before sildenafil initiation to 2.7 ± 1 WU on sildenafil, p=0.0007). While intubation time, length of inotrope use and time to hospital discharge were longer in the sildenafil group, survival was similar between both groups. Oral sildenafil was associated with a significant improvement in right ventricular dysfunction and invasive hemodynamic measurements in pediatric heart transplant recipients with high PVRI early after transplant.