Author + information
- Edon Rabinowitz,
- David Meyer and
- Shilpi Epstein
Background: Pulmonary atresia or severe pulmonary stenosis (PS) with hypoplasia of the native pulmonary arteries (PAs) is seen in a subset of patients with tetralogy of Fallot (TOF). Major aortopulmonary collateral arteries (MAPCAs) may contribute to pulmonary blood flow. Outcome depends on construction of an adequate sized pulmonary arterial tree incorporating or eliminating MAPCAs as needed. We report our results with a previously described management strategy which employs an initial intervention of right ventricle to native pulmonary artery (RV-PA) conduit to promote PA growth and ultimately allow for ventricular septation with low right ventricular pressure (RVp).
Methods: We retrospectively reviewed clinical and procedural data for all patients (n=8) with TOF, pulmonary atresia or severe PS, diminutive native PAs and MAPCAs in our center from October 2008 to August 2016. Preoperative and postoperative angiography was used to determine the pulmonary arterial vessel size over time to calculate the Nakata indices.
Results: Six patients had pulmonary atresia and 2 patients had severe PS; MAPCAs were present in 2 patients. There were no deaths or serious morbidity; 1 conduit required revision prior to complete repair. At time of conduit placement median age and weight were 7.5days and 3.1kg. Complete repair with ventricular septation was achieved in all patients at a median age of 262days. Postoperative RVp was less than half systemic in all patients. Nakata index in the neonatal period was 75±43mm2/m2 and at the time of full repair 200±146 mm2/m2 (p=0.015). The rate of increase in Nakata index from birth to full repair was 17+/-16mm2/m2/mo. The RPA and LPA growth ratio was similar at 0.38±0.31mm/mo and 0.42±0.33mm/mo respectively. The 8 patients underwent 24 catheterizations with 12 interventions prior to full repair; all but 1 patient had at least 1 intervention.
Conclusions: Early palliative RV-PA conduit promotes PA growth and facilitates eventual full repair with VSD closure with low RVp and operative risk.
Poster Hall, Hall C
Sunday, March 19, 2017, 9:45 a.m.-10:30 a.m.
Session Title: Congenital Heart Disease Surgery: Updates and Innovations
Abstract Category: 11. Congenital Heart Disease: Therapy
Presentation Number: 1272-024
- 2017 American College of Cardiology Foundation