Aortopulmonary window is a rare congenital heart disease. It results from incomplete separation of the aorta and pulmonary artery at the conotruncal septum. It accounts for 0.2%-0.6% of all congenital cardiac defects. Closure should be done in all cases. We present the case of a 12-month-old female child with a large aortopulmonary window, complicated by severe pulmonary hypertension. Cardiac catheterization was performed and showed pulmonary vascular resistance 3 Woods units. Ascending aortography showed a large aortopulmonary window measuring 5 mm. Balloon sizing of the defect showed stretched diameter of 8 mm. A multifunctional occluder device 12 × 10 mm (Konar, Lifetech, Shenzhen, China) was properly positioned across the defect under transesophageal echocardiography guidance. Pulmonary angiograms showed a well seated pulmonary disc with mild encroachment on pulmonary bifurcation. Follow up transthoracic echocardiography 24 hours after the procedure showed a well seated device with no residual flow, mean pulmonary artery pressure 25 mmHg, laminar flow into main pulmonary artery and pulmonary bifurcation. To the best of our knowledge this is the first successful closure to be reported for an aortopulmonary window with Konar multifunctional occluder. Having a double disc with a low profile and small sheath size may allow closure of larger defects in smaller children. < Transcatheter closure of large aortopulmonary window in small children with severe pulmonary hypertension is possible, yet technically demanding. Having a double disc with a low profile and small sheath size allow closure of larger defects in smaller children.>.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9169000PMC
http://dx.doi.org/10.1016/j.jccase.2021.12.014DOI Listing

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