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://dx.doi.org/10.1016/j.jccase.2021.12.014 | DOI Listing |
Ultrasound Q
March 2025
Department of Echocardiography, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui, 230001, China.
Berry syndrome is a rare combination of cardiac malformations, which is characterized by the following malformations, including the aortopulmonary window, aortic right pulmonary origin, interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and an intact ventricular septum. There are few reviews on prenatal diagnosis of Berry syndrome by fetal echocardiography. We used sequential cross-sectional scanning from apex to bottom of the heart to find aortic right pulmonary origin, aortopulmonary window, and hypoplastic aortic arch.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
January 2025
Department of Pediatrics, Inova Health System, Falls Church, VA, USA.
Pulmonary atresia with ventricular septal defect (PA-VSD) is usually diagnosed by transthoracic or fetal echocardiography, with the prenatal diagnosis being feasible and accurate if fetal cardiology services are available. The limitations of transthoracic echocardiography (TTE) in the evaluation of PA-VSD include the complete evaluation of the pulmonary arteries and patent ductus arteriosus, quantitative evaluation of the right ventricle size and function, and delineation of associated cardiac anomalies such as coronary artery anomalies, anomalies of systemic or pulmonary venous return, and complex arch anomalies. Echocardiography also has limitations in evaluating hemodynamics such as flow volumes, shunts, and regurgitant fraction.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
January 2025
Division of Cardiac Surgery, University of Toronto, Toronto, ON, Canada.
The presentation of pulmonary vasculature in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) is highly variable-as is the number, size and position of the MAPCAs and their relationship with the native pulmonary artery system. The priority in the management of this disease should be attaining timely and complete unifocalization, as opposed to single-stage full repair in every case. The merit of early unifocalization is that it secures the pulmonary vascular bed by (a) avoiding loss of lung segments from progressive stenosis/atresia of MAPCA origins, (b) preventing lung injury from high pressure/flow in areas fed by large, unobstructed MAPCAs, and (c) restoring central continuity of the pulmonary vasculature.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Department of Pediatric Cardiac Intensive Care, Cardiac Center of Ethiopia and St Paul's Hospital Millennium Medical College, Addis Ababa, Ethiopia.
Adult aortopulmonary window is a rare presentation of a rare disease; only a few cases are reported to have undergone successful surgical closure without development of Eisenmenger syndrome. We describe the second oldest patient, a 25-year-old woman, who underwent successful surgical repair of aortopulmonary window after favorable indirect measures on echocardiography without the "gold standard" preoperative cardiac catheterization study. At 2 months after the operation, the patient remains in New York Heart Association class II.
View Article and Find Full Text PDFEchocardiography
January 2025
Radiology Department, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Left images: Top: (A) Echocardiography shows a dilated pulmonary artery, large aortopulmonary window (dotted line), and abnormally positioned aortic arch. (B) MIP image reveals superior RV, inferior LV, and elongated arch vessels (arrows). Bottom: MinIP shows a thin left main bronchus and non-aerated RML (asterisk).
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