Intervention for Recoarctation in the Single Ventricle Reconstruction Trial

Abstract

Background Re-coarctation 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 (MBTS) or right ventricle-pulmonary artery shunt (RVPAS). We sought to determine incidence of re-coarctation, risk factors and outcomes in the SVR trial. Methods and Results Re-coarctation was defined by intervention, either catheter-based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed adjusting for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty; 39 surgical) for re-coarctation at median age 4.9 months (range: 1.1–10.5). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, re-coarctation was associated with the shunt type in place at the end of the Norwood procedure (HR 2.0 for RVPAS vs. MBTS, p=0.02), and Norwood discharge peak echo-Doppler arch gradient (HR 1.07 per 1 mmHg, p<0.01). Subjects with re-coarctation demonstrated comorbidities at pre-stage II evaluation including higher pulmonary arterial pressures (15.4±3.0 vs. 14.5±3.5 mm Hg; p=0.05), higher pulmonary vascular resistance (2.6±1.6 vs. 2.0±1.0 WU × m2; p=0.04) and increased echocardiographic volumes (end-diastolic volume: 126±39 vs. 112±33 ml/BSA1.3; p=0.02). There was no difference in 12-month post-randomization transplant-free survival between those with and without re-coarctation (p=0.14). Conclusions Re-coarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplant/mortality, further evaluation is warranted to evaluate effects of associated morbidities.

Recurrent Coarctation After Neonatal Univentricular and Biventricular Norwood-Type Arch Reconstruction

“…Despite the difference in recurrence rates, which may result from center variability, very similar risk factors for recurrent coarctation were found here. Similar to our findings, Hill and colleagues [9] also identified the use of an RV-PA shunt to be associated with increased risk of recurrent coarctation (HR, 2.0; 95% CI, 1.1-3.6; p ¼ 0.02) and found Norwood discharge PAAG to be a risk factor for recurrent coarctation. In the largest 2V NTAR study to date, we found a rate of recurrent coarctation approaching 20% for patients with 2V anatomy.…”

“…Numerous small retrospective studies have evaluated recurrent coarctation in the SV population in which arch augmentation is part of the NP. A recent large multicenter prospective study performed as a part of the Single Ventricle Reconstruction Trial identified a rate of recurrent coarctation requiring intervention of 18%, about twice our rate of 9.7% [9]. Despite the difference in recurrence rates, which may result from center variability, very similar risk factors for recurrent coarctation were found here.…”

“…(Ann Thorac Surg 2016;-:---) Ó 2016 by The Society of Thoracic Surgeons R ecurrent coarctation in patients with single-ventricle (SV) anatomy after the Norwood procedure (NP) has been evaluated in several studies, with a variable reported incidence of 9% to 38% and few risk factors for recurrent coarctation identified [1][2][3][4][5][6][7][8][9]. In our center, increasing familiarity with aortic augmentation used for the NP has translated into the use of a Norwood-type aortic arch reconstruction (NTAR) in patients with 2-ventricle (2V) anatomy with aortic coarctation and diffuse arch hypoplasia.…”

Coarctation of the aorta: Management from infancy to adulthood

“…Stent fracture is common but thus far has been clinically insignificant. Reintervention is also common, either due with catheter or surgical intervention, the presence of recoarctation did not affect survival rates in this tenuous patient population [64] .…”

“…Likely the most fragile patient population to develop recurrent coarctation is children with hypoplastic left heart syndrome or other single right ventricle lesions. These patients are at risk for significant morbidity and mortality with recoarctation due to exacerbation of atrioventricular valve regurgitation and ventricular dysfunction [64] . The Pediatric Heart Network Single Ventricle Reconstruction trial was a large, multicenter, prospective study examining the outcome of infants with single right ventricle lesions after randomization to either right ventriclepulmonary artery shunt or a modified BlalockTaussig shunt at the time of Norwood procedure [65] .…”