Segmental Pulmonary Arterial Disease

Isolated absence of the right pulmonary artery in a neonate: a rare but critical incidental diagnosis

Srashti Kulshrestha, Pratibha RaiManipal Hospital. Banaras Hindu University.India Cardiology in the YoungCardiol Young 2025; DOI: 10.1017/S1047951125110184 AbstractThis case underscores an uncommon presentation of pulmonary artery hypertension which, to our knowledge, has limited neonatal data. Its atypical course reveals diagnostic pitfalls that, if unrecognized, may delay effective therapy. This report highlights options for early recognition and management. It serves […]

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Clinical profile and long-term outcomes of pediatric-onset Takayasu’s arteritis: data from a mexican national referral center

Dilcia Paola Pagoaga-Vásquez, Gabriela Melendez-Ramírez, Emilia Josefina Patiño-Bahena, Alfonso Buendia-Hernández, Edgar Samuel Ramírez-Marroquín, Juan Eberto Calderon-Colmenero, Solange Gabriela Koretzky, Guering Eid-Lit, María Elena SotoInstituto Nacional de Cardiología Ignacio Chávez. Hospital Infantil de México “Federico Gómez”.  Centro Médico ABC Sur. Mexico Pediatric Rheumatology Online JournalPediatr Rheumatol Online J 2025; 23: DOI: 10.1186/s12969-025-01145-9 AbstractBackground: Takayasu arteritis (TA) is a

Clinical profile and long-term outcomes of pediatric-onset Takayasu’s arteritis: data from a mexican national referral center Read More »

[Clinical characteristics and prognosis of childhood-onset Takayasu arteritis involving pulmonary artery]

Yingjie Xu, Gaixiu Su,  Dan Zhang, Min Kang, Jia Zhu, Tong Yue, Ming Li, Min Wen, Feifei Wu, Jun Hou Jun, Shengnan Li, Jianming LaiCapital Center for Children’s Health and Capital Medical University.China Chinese Journal of PediatricsZhonghua Er Ke Za Zhi 2025; 63: 1218-1223DOI: 10.3760/cma.j.cn112140-20250511-00395 AbstractObjective: To investigate the clinical characteristics, imaging features, risk factors, and prognosis of childhood-onset

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Supravalvular aortic stenosis – Novel pathogenic ELN variant in siblings with a wide spectrum of clinical cardiovascular features and a long follow-up from infancy to adulthood

Sini Keskinen, Jussi Niemelä, Hannele Koillinen, Talvikki Boldt, Anita ArolaTurku University Hospital and University of Turku. New Children’s HospitalFinland Cardiovascular Revascularization MedicineCardiovasc Revasc Med 2025; DOI: 10.1016/j.carrev.2025.04.027 AbstractBackground: Supravalvular aortic stenosis (SVAS) is an autosomal dominantly inherited congenital cardiovascular disease caused by disruption of elastin gene (ELN), encoding elastin, an essential component of elastic arteries. It usually

Supravalvular aortic stenosis – Novel pathogenic ELN variant in siblings with a wide spectrum of clinical cardiovascular features and a long follow-up from infancy to adulthood Read More »

Arterial Tortuosity Syndrome: A Longitudinal Assessment of Cardiovascular Features and Interventions From the Collaborative for Longitudinal Aortic Research in the Young (CLARITY)

Bita Salamat, Sara B. Stephens, Jolie J. Britt, Taylor Beecroft-Dawson, Andrea Taylor, Laura Muiño Mosquera, Bert Callewaert, Aude Beyens, Alexandra Channing, Michael P. Dilorenzo, Justin Weigand, Benjamin Jacob, Eudice Fontenot, Shaine A. MorrisTexas Children’s Hospital and Baylor College of Medicine. University of Texas Health Science Center. A Twist of Fate. Ghent University Hospital and Ghent

Arterial Tortuosity Syndrome: A Longitudinal Assessment of Cardiovascular Features and Interventions From the Collaborative for Longitudinal Aortic Research in the Young (CLARITY) Read More »

Clinical Care for Cardiovascular Disease in Patients With Williams-Beuren Syndrome

R. Thomas Collins II, Verena Gravenhorst, Gilles Faury, Joanna Kwiatkowska, Christian E. H. Schmelzer, Heike Schneider, Anna Waldoch, Rainer PankauUniversity of Kentucky College of Medicine. University Medical Center and Georg-August-University Göttingen. Université Grenoble. Medical University of Gdańsk. Fraunhofer Institute for Microstructure of Materials and Systems. University Medical Center Schleswig-Holstein and Christian-Albrechts-University.United States, Germany, France and

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Decreased smooth muscle cells and fibrous thickening of the tunica media in peripheral pulmonary artery stenosis in Alagille syndrome

Yosuke Ogawa, Amane Yamamoto, Sho Yamazawa, Masako Ikemura, Yasutaka Hirata, Ryo InuzukaUniversity of Tokyo Hospital.Japan Cardiovascular PathologyCardiovasc Pathol 2025; DOI: 10.1016/j.carpath.2024.107677 AbstractAlagille syndrome is caused by mutations in genes involved in NOTCH signaling, specifically JAG1 and NOTCH2, and is associated with a high rate of peripheral pulmonary artery stenosis. In this study, we report the case

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State of the Art: Complex Peripheral Pulmonary Artery Reconstruction Techniques

Elisabeth Martin, Michael Ma, Frank HanleyStanford University School of Medicine and Lucile Packard Children’s Hospital.United States Seminars in Thoracic and Cardiovascular Surgery Pediatric Cardiac Surgery AnnualSemin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2025; 28: 101-106DOI: 10.1053/j.pcsu.2025.03.002 AbstractOver the last several years, we developed and improved our surgical approach to patients requiring pulmonary artery reconstruction in

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Pharmacologic Management of Segmental Pulmonary Hypertension in Children After Unifocalization and Pulmonary Artery Reconstruction: Initial Experience

Julian E. Cameron, Doff B. McElhinney, Esther Liu, Rachel K. Hopper, Ritu Asija, Manchula Navaratnam, Frank L. Hanley, Jeffrey A. FeinsteinLucile Salter Packard Children’s Hospital and Stanford University School of Medicine. Children’s Hospital Los Angeles and Keck School of Medicine University of Southern California.United States Pulmonary CirculationPulm Circ 2025; 15: DOI: 10.1002/pul2.70134 AbstractSegmental pulmonary hypertension (PH)

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Usefulness of exercise stress echocardiography in a patient with unilateral pulmonary branch stenosis

Ryohei Yokoyama, Yoshihiko Kodama, Kazunari Takamura, Masako Takahashi, Miyo Tanaka, Nozomi Watanabe, Hiroshi MoritakeUniversity of Miyazaki.Japan Journal of Cardiology CasesJ Cardiol Cases 2025; 31: 155-157DOI: 10.1016/j.jccase.2025.02.003 AbstractExercise stress echocardiography (ESE) is a feasible and valuable tool for evaluating subclinical pulmonary hypertension (PH). However, its utility in patients with unilateral pulmonary branch stenosis remains unclear. We present

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