Pediatric Pulmonary Vascular Disease

De novo variant in RING finger protein 213 causes systemic vasculopathy

Ayako Kashimada, Tomoko Mizuno, Eriko Tanaka, Susumu Hosokawa, Tomohiro Udagawa, Yuichi Hiraoka, Keisuke Uchida, Tomohiro Morio, Kenjiro Kosaki, Masatoshi TakagiInstitute of Science Tokyo. Kyorin University. Keio University.Japan Journal of Clinical Investigation InsightJCI Insight 2025; DOI: 10.1172/jci.insight.190094 AbstractSystemic arterial stenosis, including moyamoya disease (MMD) and middle aortic syndrome (MAS), is a rare condition of unclear etiology. MMD

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Pulmonary vascular complications in children who survive long-term after Kasai portoenterostomy for biliary atresia: a narrative review

Jie Zhang, Gong ChenChildren’s Hospital of Fudan University.China World Journal of Pediatric SurgeryWorld J Pediatr Surg 2025; 8: DOI: 10.1136/wjps-2025-001067 AbstractFollowing Kasai portoenterostomy for biliary atresia (BA), long-term survival in children is associated with chronic liver cirrhosis leading to hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH). This further causes a diverse array of respiratory and circulatory

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One-stage relief of bilateral outflow tract obstruction and left main coronary ostial stenosis in an infant with Williams syndrome: the technique

Ali H. Mashadi, Yasin Essa & Sameh M. SaidMaria Fareri Children’s Hospital and Westchester Medical Center.United States Multimedia Manual of Cardio-Thoracic SurgeryMultimed Man Cardiothorac Surg 2025; DOI: 10.1510/mmcts.2024.117 AbstractA 3-month-old, 5.2-kg infant with Williams syndrome presented with failure to thrive and a systolic murmur. He was taken to the cardiac catheterization laboratory for a planned pulmonary

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Kidney and vascular involvement in Alagille syndrome

Bruno Ranchin, Marie‑Noelle Meaux, Malo Freppel, Mathias Ruiz, Aurelie De MulHôpital Femme Mère Enfant, Hospices Civils de Lyon and Université de Lyon.France Pediatric NephrologyRediatr Nephrol 2025; 40: 891-899DOI: 10.1007/s00467-024-06562-8 AbstractAlagille syndrome (ALGS) is an autosomal dominant, multisystemic disease with a high interindividual variability. The two causative genes JAG1 and NOTCH2 are expressed during kidney development, can

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Establishment of a neonatal rat model of sequential hyperoxic hypoxia to recapitulate clinical progression of bronchopulmonary dysplasia-associated pulmonary hypertension

Dan Wang, Siqi Hu, Jingke Cao, Haoqin Fan, Ye Ma, Fan Yang, Changgen Liu, Shanghong Tang, Zhichun Feng, Yunbin Xiao, Qiuping LiAffiliated Children’s Hospital of Xiangya School of Medicine and Central South University (Hunan Children’s Hospital). Seventh Medical Center of PLA General Hospital. Second School of Clinical Medicine and Southern Medical University. Shenzhen Baoan Women’s

Establishment of a neonatal rat model of sequential hyperoxic hypoxia to recapitulate clinical progression of bronchopulmonary dysplasia-associated pulmonary hypertension Read More »

Use of milrinone in pediatric intensive care units: a multicenter survey of French-speaking countries

Florian Manoeuvrier, Morgan Recher, Marion Grimaud, Sylvain Renolleau, Olivier Brissaud, Stéphane Leteurtre, Mehdi Oualha, Charles de Marcellus, Groupe Francophone de Réanimation et d’Urgence PédiatriquesCentre-Université de Paris and Necker-Enfants Malades. CHU Amiens-Picardie. Univ.ersity of Lille and CHU Lille. CHU Bordeaux and Pellegrin-Enfants Hospital. France European Journal of PediatricsEur J Pediatr 2025; 184: DOI: 10.1007/s00431-025-06589-5 AbstractPurpose: To assess the

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Congenital Diaphragmatic Hernia – Is there a sex specific severity phenotype?

Angeo Zarfati, Luca Pio, Arimatias Raitio, Ahmed Abu-Zaid, Khadidja Khadir, Alexandra Benachi, Paul D. LostyUniversité Paris-Saclay, Assistance Publique – Hôpitaux de Paris (AP-HP), Bicêtre Hospital. University of Rome Tor Vergata. Centre de Référence Maladie Rare. University of Turku and Turku University Hospital. Alfaisal University. Antoine Béclère Hospital. University of Liverpool. Ramathibodi Hospital, Mahidol, Mahidol University.France,

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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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Marked improvement in severe pulmonary arterial hypertension following airway infection in a patient with a heterozygous BMP9 nonsense mutation: a case report

Makito Sakurai, Yohei Yamaguchi, Kei Takasawa, Susumu Hosokawa, Taku IshiiInstitute of Science Tokyo. Japanese Red Cross Musashino Hospital.Japan European Heart Journal Case ReportsEur Heart J Case Rep 2025; 9: DOI: 10.1093/ehjcr/ytaf386 AbstractBackground: Pulmonary arterial hypertension (PAH) is a severe and life-threatening disease. Genetic factors, inflammation and immune system play important roles in their pathogenesis. However, their precise

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Pectus excavatum repair during lung transplantation in a 5-year-old: A case report

Marisa E. Schwab, Elisabeth Martin, Xin Si, Stephanie D. ChaoLucile Packard Children’s Hospital, Stanford University School of Medicine.United States Interdisciplinary Cardiovascular and Thoracic SurgeryInterdiscip Cardiovasc Thorac Surg 2025; DOI: 10.1093/icvts/ivaf263 AbstractChest wall deformities are considered a risk factor for lung transplantation. A 5-year-old girl with protein surfactant C deficiency, interstitial lung disease, pulmonary hypertension, pectus excavatum

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