Segmental Pulmonary Arterial Disease

Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle

Taylor E. Katt, Robert L. Spicer, Anji T. Yetman, Ali N. Ibrahimiye, James M. Hammel, Jeffrey A. RobinsonUniversity of Nebraska Medical Center and Children’s Hospital and Medical Center.United States Journal of the American College of Cardiology Case ReportsJACC Case Rep 2020; 2: 1716-1719DOI: 10.1016/j.jaccas.2020.05.098 AbstractWilliams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis […]

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Echocardiographic Predictors of Ductal Tissue-Related Branch Pulmonary Artery Stenosis in Pulmonary Atresia

Marisha McClean, Deliwe Ngwezi, Timothy Colen, Kandice Mah, Mohammed Al-Aklabi, Lisa HornbergerStollery Children’s Hospital and University of Alberta. Children’s Hospital LHSC and Western University. Canada Pediatric CardiologyPediatr Cardiol 2022; 43: 878-886DOI: 10.1007/s00246-021-02799-4 AbstractBranch pulmonary artery (PA) stenosis due to ductus arteriosus (DA) tissue (DA-PS) contributes to the morbidity associated with pulmonary atresia (PAtr). We sought to

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Preoperative Computed Tomographic Angiography Can Predict Need for Branch Pulmonary Artery Intervention in Patients With Ductal-Dependent Pulmonary Blood Flow

Kirsten Graff, Anthony M. Hlavacek, Nicholas Aizcorbe, Scott M. Bradley, Shahryar M. ChowdhuryMedical University of South Carolina.United States World Journal of Pediatric and Congenital Heart SurgeryWorld J Pediatr Congenit Heart Surg 2023; 14: 275-281DOI: 10.1177/21501351221151050 AbstractBackground: Neonates with ductal-dependent pulmonary blood flow (DD-PBF) are at risk for pulmonary artery (PA) stenosis. The objective of this study was

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Transcatheter management of combined patent ductus arteriosus and left pulmonary artery stenosis in congenital rubella syndrome: A series of three patients and an insight into case selection for intervention

Mahua Roy, Debasree Gangopadhyay, Noopur Goyel, Amitabha Chattopadhyay, Biswajit BandyopadhyayNH RTIICS and NSH.India Annals of Pediatric CardiologyAnn Pediatr Cardiol 2022; 15: 164-168DOI: 10.4103/apc.apc_232_20 AbstractTranscatheter closure of patent ductus arteriosus (PDA) is now the standard of care with some exceptions. Best treatment for peripheral pulmonary artery (PA) stenosis in small children is still debatable. Surgical augmentation, balloon

Transcatheter management of combined patent ductus arteriosus and left pulmonary artery stenosis in congenital rubella syndrome: A series of three patients and an insight into case selection for intervention Read More »

A germline chimeric KANK1-DMRT1 transcript derived from a complex structural variant is associated with a congenital heart defect segregating across five generations

Silvia Souza da Costa, Veniaminn Fishman, Mara Pinheiro, Andre Rodrigueiro, Maria Teresa Sanseverino, Paulo Zielinsky, Claudia M. B. Carvalho, Carla Rosenberg, Ana Cristina Victorino KrepischiUniversity of São Paulo. Siberian Branch of the Russian Academy of Sciences. Uniscience do Brasil. Hospital de Clínicas de Porto Alegre. Pontifícia Universidade Catolica do Rio Grande Do Sul. Federal University

A germline chimeric KANK1-DMRT1 transcript derived from a complex structural variant is associated with a congenital heart defect segregating across five generations Read More »

Case report: the ‘vanished’ left pulmonary artery

Jiahui Charmaine Chan, Monika Kantilal Kotecha, Jonathan Tze Liang Choo, Marielle V. Fortier, Sreekanthan SundararaghavanKK Women and Children’s Hospital. National University of Singapore. Duke-National University of Singapore Medical School. Lee Kong Chian School of Medicine and Nanyang Technological University. Singapore European Heart Journal Case ReportsEur Heart J Case Rep 2024; 8:DOI: 10.1093/ehjcr/ytae147 AbstractBackground: We report a case

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Diagnostic Strategy for Suspected Unilateral Absence of the Pulmonary Artery

Van Luong Hoang, Viet Anh Lam, Thanh Nguyen PhamHanoi National Lung Hospital. Vin University. Hai Phong University of Medicine and Pharmacy.Vietnam Current Medical ImagingCurr Med Imag 2024; DOI: 10.2174/0115734056266404231207071244 AbstractBackground: Unilateral absence of the pulmonary artery (UAPA) is a very rare congenital anomaly.Objective: To analyze the diagnostic strategy applied to seven patients with UAPA who were examined

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Elastin Insufficiency Confers Proximal and Distal Pulmonary Vasculopathy in Mice, Partially Remedied by the KATP Channel Opener Minoxidil: Considerations and Cautions for the Treatment of People With Williams-Beuren Syndrome

Russell H. Knutsen, Leah M. Gober, Elise K. Kronquist, Maninder Kaur, Danielle R. Donahue, Danielle Springer, Zu Xi Yu, Marcus Y. Chen, Yi-Ping Fu, Feri Choobdar, My-Le Nguyen, Sharon Osgood, Joy L. Freeman, Neelam Raja, Mark D. Levin, Beth A. KozelNational Heart, Lung, and Blood Institute; National Institute of Neurological Disorders and Stroke; and National

Elastin Insufficiency Confers Proximal and Distal Pulmonary Vasculopathy in Mice, Partially Remedied by the KATP Channel Opener Minoxidil: Considerations and Cautions for the Treatment of People With Williams-Beuren Syndrome Read More »

Virtual Transcatheter Interventions for Peripheral Pulmonary Artery Stenosis in Williams and Alagille Syndromes

Ingrid S. Lan, R. Thomas Collins, II, Weiguang Yang, Jeffrey A. Feinstein, Michael Ma, Jacqueline Kreutzer, Gregory T. Adamson, Alison L. MarsdenStanford University. University of Pittsburgh.United States Journal of the American Heart AssociationJ Am Heart Assoc 2022; 11:DOI: 10.1161/JAHA.121.023532 AbstractBackground: Despite favorable outcomes of surgical pulmonary artery (PA) reconstruction, isolated proximal stenting of the central PAs

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Analysis of risk factors associated with extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenoses

L. Mac Felmly, Richard D. Mainwaring, Claudia Algaze, Elisabeth Martin, Michael Ma, Frank L. HanleyStanford University School of Medicine and Lucile Packard Children’s Hospital.United States Journal of Thoracic and Cardiovascular Surgery OpenJTCVS Open 2023; 13: 344-356DOI: 10.1016/j.xjon.2023.01.011 AbstractObjective: Acute lung injury is a known complication of pulmonary artery reconstruction for peripheral pulmonary artery stenosis. Severe cases may

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