Surgical Management of Fallot's Tetralogy With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries: Multistage Versus One-Stage Repair.

A strict and rational approach to Fallot's tetralogy with pulmonary atresia and major aortopulmonary collateral arteries allows to achieve optimal results. Rehabilitative and unifocalization strategies do not constitute separate philosophies; instead the surgical strategy should be tailored to each individual patient. Based on our previous experience, the ability to achieve definitive intracardiac repair is the real determinant of both improved survival and adequate systolic right ventricular performance on mid-term follow-up.

Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries.

BACKGROUND: A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation. METHODS: The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals. RESULTS: Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3 patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7 patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%). CONCLUSIONS: A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired.

Surgical experience in the rehabilitation and reimplantation of disconnected pulmonary arteries and its effectiveness in restoring pulmonary haemodynamics and function.

OBJECTIVES: We established a policy to restore disconnected pulmonary arteries (PAs), regardless of aetiology or cardiac pathology, using a protocol-based strategy. There are conflicting reports of the need to restore disconnected PA especially if severely hypoplastic with some groups advocating surgical repair of cardiac defects to single lung with moderate to high risk. METHODS: All patients referred to our PA reconstruction programme (2007-14) with complete separation of one branch PA were included. Our surgical strategy was based on the size of the PA. In patients with mild or no PA hypoplasia, we performed single-stage implantation of the disconnected PA and repair of intracardiac defects. Two-stage approach was used in patients with severe PA hypoplasia where the disconnected PA was retrieved inside the lung then connected to the aorta with a shunt to promote growth. After 6-12 months, the PA was implanted with simultaneous repair of intracardiac defects. RESULTS: Twenty patients were included (age range = 1 week to 8 years). Aetiologies were ductal origin of distal PA in 13 patients and iatrogenic in 7 patients. Group 1 (isolated lesion) consisted of 8 patients who presented with severe pulmonary hypertension. Group 2 consisted of 12 patients with associated intracardiac defects and was subdivided into Group 2a (biventricular hearts) 8 patients and Group 2b (univentricular hearts) 4 patients. Single-stage repair was achieved in 10 patients. Direct tissue-to-tissue connection was achieved in 18 (90%) patients. The follow-up was 100% complete (median = 53.8 months). All patients are alive and asymptomatic. Implanted PAs are patent with good growth demonstrated on serial echocardiography. Only two PAs required balloon dilatation for moderate restenosis at the anastomosis. No surgical reintervention was needed. All Group 1 patients had complete resolution of the pulmonary hypertension. Lung perfusion scans showed increased uptake (24-53% of the total uptake). CONCLUSIONS: Our study demonstrated that, in paediatric age group, disconnected PAs can be restored with low risk and excellent outcomes even in patients with severely hypoplastic PA. Strategies to repair intracardiac defects to a single lung might not be the optimal approach.

Pulmonary atresia with ventricular septal defect and multifocal pulmonary blood supply: does an intensive interventional approach improve the outcome?

INTRODUCTION: Pulmonary atresia with ventricular septal defect (VSD) continues to be associated with significant morbidity and mortality, with significant institutional variation in therapeutic strategies. This study reports a single center experience utilizing an intensive transcatheter approach to promote pulmonary vascular growth. METHODS: A retrospective analysis of 20 patients undergoing surgical and transcatheter treatment for pulmonary atresia with VSD between 2002 and 2010. RESULTS: The median age at initial surgical palliation was 6.3 months (8 days to 2.5 years). Eleven patients (group 1) underwent initial surgical palliation without VSD closure and nine patients (group 2) underwent an initial complete repair with fenestrated or complete VSD closure. Group 1 had a smaller Nakata index (54 mm2/m2 vs. 134 mm2/m2 , P = .04) and a smaller absolute native pulmonary artery diameter (2.7 mm vs. 4.5 mm, P = .01) than group 2. Intraoperative angiography was performed in 10 cases to evaluate if early transcatheter intervention was warranted. The median follow-up during the study period was 2.3 years (1.6 months to 8.3 years). Of the 16 patients who survived the initial early postoperative period, 15 patients (94%) went on to receive surgical (n = 11) and/or interventional (n = 25) catheterization procedures. There was improvement in the mean Nakata index from the initial presurgical evaluation to the most recent catheterization data (38.4 mm2/m2 vs. 169.7 mm2/m2, P ≤ .05). To date, two of 11 (18%) patients in group 1 ultimately underwent surgical VSD closure. Overall mortality was six of 20 (30%) with four deaths in group 1 and two deaths in group 2. There were no procedural deaths. CONCLUSIONS: Combining surgical unifocalization procedures with subsequent early and intensive catheter-based pulmonary artery rehabilitation may improve vascular growth, ultimately rendering many patients suitable for fenestrated VSD closure. Risk stratification, including intraoperative exit angiography, is essential to determine the need for early transcatheter interventions.

Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals: neonatal pulmonary artery rehabilitation without unifocalization.

BACKGROUND: This study analyzed a protocol of neonatal rehabilitation of hypoplastic pulmonary arteries in the management of pulmonary atresia, ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs). Ideal management of patients with pulmonary atresia, VSD, and MAPCAs is the subject of controversy. METHODS: From June 2003 to December 2008, 25 consecutive patients were diagnosed with pulmonary atresia, VSD, and MAPCAs, and 20 were entered into a neonatal shunting regimen. The median age at the first operation was 3.6 weeks (range, 0.7 to 17 weeks). All patients underwent an initial central or modified Blalock-Taussig shunt, or both. Further preparatory procedures included 26 pulmonary artery patch reconstructions, 19 right ventricle-to-pulmonary artery conduits, 4 MAPCA ligations, and 4 further shunts. No patient underwent translocation of the collateral arteries. RESULTS: At the latest follow-up, 12 of 20 patients have had a complete repair at a median age of 18 months (range, 11 to 48 months), 6 are awaiting repair, and 2 are considered unlikely to be repaired. No patient was missing follow-up. Median pulmonary artery indices had grown from 14.51 to 118.7 in 17 patients. Postoperative angiograms were performed at a median of 8 months (range, 1.9 to 32.7 months) in 9 of 12 completely repaired patients. The median right/left ventricular pressure ratio was 0.64 (range, 0.54 to 0.91). CONCLUSIONS: Rehabilitation of hypoplastic native pulmonary arteries by a neonatal shunting regimen, without MAPCA translocation, for pulmonary atresia, VSD, and MAPCAs, provides encouraging results with excellent early survival.