Objective/Background Historically, the sole option for patients with a dysfunctional native right ventricular outflow tract (RVOT) requiring re-establishment of pulmonary competence has been surgical PVR. We sought to compare early outcomes of hybrid pulmonary valve replacement (PVR) combining surgical plication of the main pulmonary artery followed by transcatheter PVR, with a contemporary cohort of surgical PVR patients. Methods Retrospective chart analysis of all patients with a dilated native RVOT eligible for surgical PVR over 36 months was performed. The cohorts included patients with previous tetralogy of Fallot repair (n = 14), and previous intervention for congenital abnormality of the pulmonary valve (n = 7). Results Twenty-one patients with a dysfunctional native RVOT met criteria for PVR; 8 using the hybrid procedure (group 1: age, 31.5 +/- 17.4 years) and 13 with cardiopulmonary bypass (CPB) (group 2: age, 31 +/- 18.4 years). Valve delivery was successful in all patients with no procedural mortality. Group 1 had a lesser requirement for blood products (P =< 0.001) and a trend toward shorter hospital stay and higher post-operative hemoglobin. No patients in group 1 received inotropic support post-operatively compared to 54% of patients in group 2. Mean follow-up was 3.4 months for group 1 and 13.6 months for group 2 with the average peak gradient across the RVOT of 20.1 and 15.1 mm Hg respectively (P = 0.12), all with no more than mild PI. Conclusions Transcatheter hybrid PVR following RVOT plication provides a reasonable alternative to surgical PVR particularly in higher risk cohorts, reducing possible longer-term consequences of repeated runs of CPB. © 2016 Wiley Periodicals, Inc.

The pentalogy of Cantrell (PC) was first described in 1958. It includes five anomalies: a deficiency of the anterior diaphragm, a midline supraumbilical abdominal wall defect, a defect in the diaphragmatic pericardium, various congenital intracardiac abnormalities, and a defect of the lower sternum. Five patients showing the PC spectrum are reported. The report presents the prenatal diagnosis, the postnatal course, and the patients' outcome at a tertiary care center from June 2001 to May 2012. A literature review and the management plan for this group of patients also are discussed. All patient data were obtained via electronic medical records retrospectively after approval by the institutional review board at the home institution. The patients in the study were three males and two females. For all of the patients, a prenatal diagnosis had been determined. The mean gestational age at delivery was 36.6 weeks. One patient had associated cranial and spine malformations. All the patients had associated congenital heart disease but a normal karyotype. Four of the five patients died in the first year of life. The ages at death ranged from 0 to 259 days (mean, 46.2 ± 51.8 days). The patients who did not survive had withdrawal of care due to increased morbidity, associated complications, or parental wishes. The pentalogy of Cantrell is a wide spectrum of associations. Patients with the complete PC together with complex congenital heart disease or extracardiac malformations may have a poor prognosis. Incomplete PC cases may have a better outcome based on associated anomalies. Prenatal counseling plays a very important role in the decision-making process for the families and has a significant impact on the postnatal management. A multidisciplinary team approach is essential for successful postpartum outcomes.

Ventricular tachycardia is a rare clinical entity in pediatric patients and typically requires chemical and/or electrical intervention to convert into a sustained sinus rhythm. However, for certain forms originating from the right ventricular outflow tract, conversion with adenosine and vagal maneuvers has been demonstrated in adult patients. In this case, we suggest that pediatric patients with right ventricular outflow tract ventricular tachycardia who are hemodynamically stable may benefit from a trial of vagal maneuvers.

This report describes the use of the Gore Excluder self-expanding stent to successfully exclude a contained right ventricle to pulmonary artery conduit rupture during transcatheter pulmonary valve replacement (tPVR). This stent was designed for percutaneous abdominal aortic aneurysm exclusion; however, its use in this setting allowed progression to tPVR following conduit rupture without the need for emergent surgery.

Background: Patent Ductus Arteriosus (PDA) in premature neonates has been associated with comorbidities including chronic lung disease (CLD), and death. However, the treatment of PDA remains controversial. There have been several echocardiographic variables previously used to determine the hemodynamic significance of PDA but their utility in early prediction of clinical outcomes is not well studied. Objective: The objective of our study was to evaluate the use of a severity scoring system incorporating markers of systemic under perfusion, pulmonary over perfusion and left ventricular (LV) function in predicting clinical outcomes in premature neonates. Methods: It is a single center prospective observational study involving newborns < 32 weeks’ gestation. An echocardiogram was done within seven days of life to measure variables previously known to predict severity of shunting in PDA including pulmonary perfusion index (PPI). Predictors of CLD/death were identified using multivariate logistic regression. A severity score was derived and its ability to predict clinical outcomes was tested using a receiver operating characteristic curve. Results: We studied 98 infants with a mean (SD) gestation of 28.9 ± 1.91 weeks and birth weight of 1228.06 ± 318.94 g, respectively. We identified five echocardiographic variables along with gestational age that was independently associated with the outcome variable (PPI, LV output, Superior Mesenteric Artery [SMA] Velocity Time Integral [VTI], Peak diastolic flow velocity in Pulmonary Vein [PV Vd], and reversal of flow in diastole in descending aorta [DFR]). The range of severity score was 0 (low risk) to 12 (high risk). A higher score was associated with the primary outcome variable of CLD/death (7.5 [1.2] vs. 3.6 [1.5], p < 0.001). Our severity score had an area under the curve of 0.97 (95% CI 0.93–0.99, p < 0.001) for predicting CLD/death. Conclusion: Our new PDA severity score of 5.5 has a sensitivity and specificity of 94% and 93%, and positive and negative predictive values of 94% and 93%, respectively.