Cobra-Head Cuffed Vascular Graft as Right Ventricle-to-Pulmonary Artery Shunt in Norwood Procedure.

BACKGROUND: Right ventricle-to-pulmonary artery (RV-PA) shunt as a part of the Norwood procedure underwent many modifications. We present our experience with a commercially available polytetrafluoroethylene vascular graft with cobra-head cuff as an RV-PA shunt. METHODS: A consecutive series of 52 children with hypoplastic left heart syndrome (median age 8 [range, 2-68] days, median weight 3200 [range, 2060-4400] g) underwent the Norwood procedure with a cobra-head cuffed RV-PA shunt (6 mm). The cuffed end was used for the central PA reconstruction. A retrospective analysis of clinical results, PAs development, and shunt-related complications, interventions, and technique of Glenn operation was performed. The study endpoint was Glenn operation with shunt removal or interstage death. RESULTS: The hospital and late interstage mortality was 3.8% (n = 2 of 52) and 4% (n = 2 of 50), respectively, and was not shunt-related. During mean follow of 3.7 ± 2.5 years, 48 (92.3%) children underwent Glenn operation at a median age of 6 (range, 2.6-9.1) months. Angiography before the second stage revealed satisfactory branch PAs development (maximum and minimum McGoon ratio of 1.95 ± 0.36 and 1.38 ± 0.38, respectively). The mean maximal diameter of the left PA was smaller than that of the right PA (7.13 ± 2.1 mm vs 8.42 ± 2.2 mm; P = .017), without differences in mean minimal diameter. Two infants required stent implantation in proximal shunt end and 1 required urgent Glenn operation because distal shunt thrombosis. During Glenn operation, 11 (22.9%) children required patch reconstruction of central PAs. CONCLUSIONS: The cobra-head cuffed graft allowed easy and reproducible reconstruction of the central PA during the Norwood procedure. Using this technique, the development of PAs is satisfactory, the rate of shunt-related complications and interventions is low, and the second stage can be performed without patch material.

Secundum Type Atrial Septal Defect in Patients with Trisomy 21-Therapeutic Strategies, Outcome, and Survival: A Nationwide Study of the German National Registry for Congenital Heart Defects.

(1) Secundum type atrial septal defect (ASD II) is usually considered a relatively benign cardiac lesion amenable to elective closure at preschool age. Patients with trisomy 21 (T21), however, are known to have a higher susceptibility for pulmonary vascular disease (PVD). Therefore, T21 children may present with clinical symptoms earlier than those without associated anomalies. In addition, early PVD may even preclude closure in selected T21 patients. (2) We performed a retrospective analysis of the German National Register for Congenital Heart Defects including T21 patients with associated isolated ASD II. We report incidence, demographics, therapeutic strategy, outcome, and survival of this cohort. (3) Of 46,628 patients included in the registry, 1549 (3.3%) had T21. Of these, 156 (49.4% female) had an isolated ASD II. Fifty-four patients (34.6%) underwent closure at 6.4 ± 9.9 years of age. Over a cumulative follow-up (FU) of 1148 patient-years, (median 7.4 years), only one patient developed Eisenmenger syndrome and five patients died. Survival of T21 patients without PVD was not statistically different to age- and gender-matched controls from the normal population (p = 0.62), whereas children with uncorrected T21/ASD II (including patients with severe PVD, in whom ASD-closure was considered contraindicated) showed a significantly higher mortality. (4) The outcome of T21-patients with ASD II and without PVD is excellent. However, PVD, either precluding ASD-closure or development of progressive PVD after ASD-closure, is associated with significant mortality in this cohort. Thus T21 patients with ASD II who fulfill general criteria for closure and without PVD should be offered defect closure analogous to patients without T21.

Right atrial cardiac myxoma and multiple pulmonary artery aneurysms: Is there a causal relationship?

Cardiac myxomas are rare occurrences in pediatric populations, as are pulmonary artery aneurysms. We report a 17-year-old adolescent with right atrial cardiac myxoma and concomitant multiple peripheral pulmonary artery aneurysms. Histological examination indicated infiltration of the pulmonary artery wall through the embolic cardiac myxoma cells, thereby weakening it. This report highlights the probable causal relationship between these two entities, proposes the possible pathomechanism and presents the diagnostic and therapeutic dilemmas in the management of this condition.

Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

OBJECTIVES: The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS: A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3-9.8) years and median weight 12.7 (range 7.6-26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS: Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS: Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.