Transcatheter closure of a PmVSD is technically feasible and seems safe enough in children over 8 kg in weight to warrant continuation of clinical trials to assess the long-term safety and efficacy.
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.
Gianturco coils were used to embolize 77 vessels in 54 patients: 58 aortopulmonary collateral vessels, 14 Blalock-Taussig shunts, 3 arteries involved in pulmonary sequestrations and 2 venae cavae. Embolization resulted in total occlusion in 53 (69%), subtotal occlusion in 19 (25%) and partial occlusion in 3 (1 intentional). Two embolizations failed to reduce flow. Thus, 72 (95%) of 76 embolizations in which complete occlusion was the intended result resulted in total or subtotal occlusion. Analysis of the results demonstrates that completely occluded collateral vessels were longer and had a smaller diameter than did incompletely occluded vessels. Complications included six cases of inadvertent embolization to the pulmonary arteries (n = 5) or the aorta (n = 1); three were retrieved and three were left without symptoms. In addition, there was a case of severe hemolysis after intentional partial occlusion of a Blalock-Taussig shunt. The results demonstrate that coil embolization can be an effective procedure for managing a wide variety of aorto-pulmonary collateral vessels and shunts in children with congenital heart disease.
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