Neonatal management of aortic coarctation with ventricular septal defect: a systematic review and meta-analysis.

INTRODUCTION: Neonatal management of aortic coarctation with ventricular septal defect is still under debate between the one-stage full repair by sternotomy versus the staged repair of the coarctation first by thoracotomy (with or without banding the pulmonary artery) followed later by subsequent closure of the ventricular septal defect. EVIDENCE ACQUISITION: The aim of this review was to synthesize the evidence in literature since 1980 for the neonatal population. A meta-analysis compared mortality between the two strategies. EVIDENCE SYNTHESIS: The analysis did not find a superiority of a strategy over the other regardless of the surgical era studied. Recoarctation rates of both strategies are presented and a management algorithm is suggested. CONCLUSIONS: Instead of comparing between the two strategies, a case-adapted management considering the anatomy of the ventricular septal defect and of the aortic arch is discussed to address this association of lesions though presenting with a wide range of settings.

The modified Fontan procedure with use of extracardiac conduit in adults: analysis of 32 consecutive patients.

BACKGROUND: Uncommonly, adults with functionally univentricular hearts are becoming candidates for a Fontan procedure. The purpose of this study was to evaluate the course of patients undergoing the modified Fontan procedure with an extracardiac conduit in recent years. METHODS: Between January 2003 and December 2013, 32 adult patients (17 female and 15 male) underwent total cavopulmonary connection (TCPC) with extracardiac conduit. The median age at procedure was 24.5 years (interquartile range [IQR] 20 to 33 years). The diagnoses included double-inlet left ventricle (DILV) in 10 patients (31.2%), tricuspid atresia in 8 patients (25%), double-outlet right ventricle in 4 patients (12.5%), heterotaxia in 4 patients (12.5%), and mitral atresia in 2 patients (6.2%). Seventy-eight percent of patients had undergone at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt (16 patients), superior cavopulmonary shunt (12 patients), and pulmonary artery banding (6 patients). All patients underwent cardiac catheterization preoperatively. Aortic cross-clamping was necessary in 15 patients for intracardiac procedures. Fenestration was required in 9 patients (28%). Four concomitant intraoperative cryoablation procedures were performed. RESULTS: There was no hospital mortality. One patient (3.1%) died 6 months after undergoing TCPC. Morbidities included prolonged pleural effusion lasting more than 7 days in 20 patients (62.5%), atrial arrhythmias in 4 patients (12.5%), and permanent pacemakers in 3 patients (9.3%). The median follow-up time was 33 months (interquartile range [IQR], 10.5 to 50 months). Actuarial survival was 91.83% (95% confidence limits, 71.07 to 97.89) at 1 year and 5 years. Ninety-two percent of patients were in New York Heart Association class I or II at follow-up. The median postoperative oxygen saturation was 95% (IQR, 93% to 95.5%). Cardiac arrhythmia occurred in 4 patients. Systolic ventricular function improved during follow-up for all patients except 1 patient, who underwent cardiac transplantation 7 months after the TCPC. CONCLUSIONS: The modified Fontan procedure with use of an extracardiac conduit can be performed in adults with encouraging early and midterm results. The majority of late survivors had improved quality of life. The incidence of late death, reoperation, arrhythmias, and thromboembolic events was low during follow-up.

To spare or not to spare ? The aortic valve in scleroderma and aortic root aneurysm.

Acute valve-sparing procedures are effective in the treatment of aortic root dilation when there is no valve pathology. In this review, we discuss the role of aortic valve-sparing procedures in the presence of connective tissue disorders such as scleroderma.