Objectives: We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS). Background: Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection. Methods: A retrospective review of patients with PAIVS at our institution from Jan 2006 to Dec 2013 was performed. Demographic, echocardiographic, procedural, and follow-up data were reviewed and analyzed. Results: Twenty-nine patients were identified. Thirteen underwent DS and 16 underwent SPS. In the DS group, 6 had radiofrequency perforation of the pulmonary valve, and 12 underwent balloon pulmonary valvuloplasty (BPV) prior to DS. SPS was performed after perforation of the pulmonary valve in 1 patient and after BPV in 4 patients. Procedural success was 92.8% in the DS group versus 93.3% in the SPS group. In the DS group, one patient required emergency shunting due to intra-procedural ductal spasm. In the SPS group there was one procedure related death, and there were six significant procedurerelated complications. Five patients required re-interventions prior to hospital discharge. The median length of hospital stay for the DS versus SPS group was 10 versus 23 days, respectively (P < 0.05). Post discharge, there were seven reinterventions in the DS group and two reinterventions in the SPS group. Conclusions: DS may provide a safe and effective alternative to SPS in selected patients with PAIVS. V C 2015 Wiley Periodicals, Inc.

Myocardial performance index (MPI), or Tei index, has become a commonly used parameter for the noninvasive, Doppler-derived assessment of global systolic and diastolic performance of the heart in both adults and children. Normal values have been established in adults and children; however, limited data exist in fetal hearts. The aim of this study was to further elucidate normal values of fetal left (LV) and right ventricle (RV) MPI values in second- and third-trimester fetuses and compare these values with other previously published data. This was a retrospective study to measure MPI in healthy fetuses. After Institutional Review Board approval, 2000 fetal echocardiography studies (FES) were acquired during a period of 4 years. Demographic parameters examined included gestational age (GA), maternal age (MA), and indication for fetal echocardiography. Fetuses with congenital heart disease, arrhythmias, or significant noncardiac fetal anomalies were excluded. The following echocardiography parameters were collected: LV ejection time (LVET), mitral valve close-to-open time (MVCO), RVET, tricuspid valve CO (TVCO), and fetal heart rate. For simplicity, LV and RV MPI values were calculated as follows: LV MPI = MVCO - LVET/LVET and RV MPI = TVCO - RVET/RVET. Four hundred twenty FES met the study criteria. LV MPI was evaluated in 230 and 190 FES in the second and third trimester, respectively. Of the 420 FES, 250 (150 in the second trimester and 100 in the third trimester) had all of the measurements required for RV MPI calculation. MA ranged between 16 and 49 years. Indications for FES included diabetes mellitus (N = 140; 33 %), suspected fetal anomalies on routine obstetrical ultrasound (N = 80; 20 %), autoimmune disorder (N = 60; 14 %), family history of CHD (N = 76; 18 %), medication exposure (N = 22; 5 %), increase nuchal thickness (N = 13; 3 %), and other indications (N = 29; 6 %). Averaged LV and RV MPI values were 0.464 ± 0.08 and 0.466 ± 0.09, respectively. Further analysis based on gestational period showed slightly greater LV and RV MPI values during the third compared with the second trimester, i.e., 0.48 and 0.49, respectively, with no statistically significant difference. There was no significant association of LV and RV MPI with heart rate. To our knowledge, this is the first study to establish normal values of fetal MPI based on a large fetal population from a single tertiary center. LV and RV MPI values were independent of GA and fetal heart rate. MPI is a useful parameter for the assessment of global cardiac function in the fetus and demonstrates good reproducibility with narrow interobserver and intraobserver variability. Its usefulness should be studied in fetal hearts with complex congenital anomalies.

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.

Stenting of systemic to pulmonary artery shunt is a safe and effective procedure and avoids surgical re-intervention. It can be performed both as a rescue procedure in patients with acute shunt occlusion and as an elective procedure to palliate patients not yet suitable for subsequent corrective or staged repair.