Intervention for recoarctation in the single ventricle reconstruction trial: incidence, risk, and outcomes.

BACKGROUND: Recoarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. We sought to determine the incidence of recoarctation, risk factors, and outcomes in the SVR trial. METHODS AND RESULTS: Recoarctation was defined by intervention, either catheter based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed with adjustment for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty, 39 surgical) for recoarctation at a median age of 4.9 months (range, 1.1-10.5 months). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, recoarctation was associated with the shunt type in place at the end of the Norwood procedure (hazard ratio, 2.0 for right ventricle-pulmonary artery shunt versus modified Blalock-Taussig shunt; P=0.02), and Norwood discharge peak echo-Doppler arch gradient (hazard ratio, 1.07 per 1 mm Hg; P<0.01). Subjects with recoarctation demonstrated comorbidities at pre-stage II evaluation, including higher pulmonary arterial pressures (15.4±3.0 versus 14.5±3.5 mm Hg; P=0.05), higher pulmonary vascular resistance (2.6±1.6 versus 2.0±1.0 Wood units·m(2); P=0.04), and increased echocardiographic volumes (end-diastolic volume, 126±39 versus 112±33 mL/BSA(1.3), where BSA is body surface area; P=0.02). There was no difference in 12-month postrandomization transplantation-free survival between those with and without recoarctation (P=0.14). CONCLUSIONS: Recoarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplantation/mortality, further evaluation is warranted to evaluate the effects of associated morbidities.

Complete resolution of systemic venous baffle obstruction and baffle leak using the Gore Excluder covered stent in two patients with transposition of the great arteries and prior Mustard procedure.

We present two patients with a history of Mustard repair of transposition of the great arteries. Both patients presented with exertional limitation and demonstrated superior systemic venous baffle obstruction as well as multiple baffle leaks. In both patients stent relief of obstruction and baffle leak exclusion was accomplished using a combination of bare metal stents and the aortic extension portion of the Gore Excluder covered stent (W.L. Gore and Associates, Flagstaff, Arizona).

Novel transatrial septoplasty technique for neonates with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum.

Two patients born with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum requiring emergent intervention are presented. For both patients, rapid septoplasty was performed using a hybrid approach involving direct atrial puncture and transatrial cutting balloon dilation of the atrial septum. This technique facilitates safe and rapid procedural completion and minimizes the risk of procedural complications in this very-high-risk patient population.