Percutaneous device closure of patent ductus arteriosus with pulmonary artery hypertension: long-term results.

UNLABELLED: Device closure of patent ductus arteriosus (PDA) is treatment of choice. But device closure in presence of pulmonary artery hypertension (PAH) remains a challenge. Data on patient selection, technical considerations, and complications are limited. AIM: To know the challenges and efficacy of device closure of PDA with PAH. MATERIALS AND RESULTS: Out of 1,325 cases of device closure of PDA, 246 (18.6%) with PAH formed the study material. To test the feasibility, chosen device is used to occlude PDA for ten minutes without oxygen inhalation. The device is released only if PAH reduced. PAH decreased in all except in 1 patient after closure with muscular ventricular septal occluder (MVSDO), pulmonary artery pressure (PAP) transiently increased (became supra-systemic), without significant reduction in aortic pressure. Device embolized in 8 patients (3.3%). Percutaneous retrieval was done in 4 (by snare in 2 and by fixing the cable to device in 2) and replaced with bigger devices. The surgical removal of the embolized MVSDO and ligation was done in 4 cases. All patients were on oral sildenafil and bosentan until PAP regressed to normal. Follow up was from 6 months to 9 years. No residual shunt in any patient on follow-up. The PAP regressed to normal in all except 5 cases (2.03%) of Down's syndrome with systemic PAP. CONCLUSIONS: Device closure of PDA with PAH is feasible, safe in all age groups. Temporary PDA occlusion with device is effective and time saving for evaluating pulmonary vascular reactivity. Device embolization in aorta is higher with severe PAH. Novel method of retrieval is effective.

Holt-oram syndrome associated with double outlet right ventricle: A rare association.

Holt-Oram syndrome is a rare inherited disorder that causes abnormalities of the hands, arms, and the heart. Most commonly, there are defects in the carpal bones of the wrist and in the bones of the thumb along with cardiac defects such as atrial or ventricular septal defects. We report a case of Holt-Oram syndrome with a rare association of double outlet right ventricle.