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A novel frameshift TBX4 variant in a family with ischio-coxo-podo-patellar syndrome and variable severity

Giada Moresco, Ornella Rondinone, Alessia Mauri, Rita Gorgoglione, Daniela Maria Grazia Graziani, Michal Dziuback, Monica Rosa Miozzo, Silvia Maria Sirchia, Luca Pietrogrande, Angela Peron, Laura FontanaUniversità degli Studi di Milano/University of Milan. ondazione IRCCS Ca Granda Ospedale Maggiore Policlinico. ASST Santi Paolo e Carlo. Meyer Children’s Hospital IRCCS. Università degli Studi di Firenze.Italy Genes and […]

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Bilateral Pulmonary Arteriovenous Malformations: Challenges in a Rare and Complex Case

Aayushi Joshi, Vasu Saini, Bhumika Bheemavarapu, Anjani Mahesh Kumar Cherukuri, Mohd. ShabanShri Guru Ram Rai Institute of Medical and Health Sciences. Jawaharlal Institute of Postgraduate Medical Education and Research. Guntur Medical College.India CureusCureus 2024; 16: DOI: 10.7759/cureus.70231 AbstractPulmonary arteriovenous malformations (PAVMs) are abnormal vascular connections between the pulmonary arteries and pulmonary veins. Despite their relatively uncommon

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Extracorporeal membrane oxygenation (ECMO) support for children with pulmonary hypertension: A single-institutional experience of outcomes

Christopher Nemeh, Nicholas Schmoke, William Patten, Eunice Clark, Yeu S. Wu, Pengchen Wang, Paul Kurlansky, William Middlesworth, Eva W. Cheung, Erika B. RosenzweigColumbia University Vagelos College of Physicians and Surgeons/NewYork-Presbyterian Morgan Stanley Children’s Hospital. United States Pulmonary CirculationPulm Circ 2024; 14: DOI: 10.1002/pul2.12442 AbstractPediatric pulmonary arterial hypertension (PAH) can present with a wide spectrum of disease

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The cardiopulmonary benefits of physiologically based cord clamping persist for at least 8 hours in lambs with a diaphragmatic hernia

Paige J. Riddington, Philip L. J. DeKoninck, Marta Thio, Calum T. Roberts, Risha Bhatia, Janneke Dekker1, Aidan J. Kashyap, Benjamin J. Amberg, Karyn A. Rodgers, Alison M. Thiel, Ilias Nitsos, Valerie A. Zahra, Ryan J. Hodges, Stuart B. Hooper, Kelly J. CrossleyHudson Institute of Medical Research. Monash University. Erasmus MC University Medical Center-Sophia Children’s Hospital.

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Current Pharmaceutical Strategies in the Management of Persistent Pulmonary Hypertension of the Newborn (PPHN): A Comprehensive Review of Therapeutic Agents

Divyanshi Kaplish, Jayant D. Vagha, Sachin Rathod, Aditya JainJawaharlal Nehru Medical College and Datta Meghe Institute of Higher Education and Research.India CureusCureus 2024; 16: DOI: 10.7759/cureus.70307 AbstractPersistent Pulmonary Hypertension of the Newborn (PPHN) is a life-threatening condition characterized by the failure of normal circulatory transition after birth, leading to sustained pulmonary hypertension and severe hypoxemia. Despite

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Could a Two-Staged Repair Be the Solution to the Dilemma of Repair Timing for Severe Congenital Diaphragmatic Hernia Requiring Extracorporeal Membrane Oxygenation?

Akiko YokoiKobe Children’s Hospital.Japan ChildrenChildren 2024; 11:DOI: 10.3390/children11101255 AbstractPurpose of review: Congenital diaphragmatic hernia (CDH) remains a significant challenge, particularly in severe cases with persistent pulmonary hypertension (PPHN) and hypoplastic lungs and heart. For patients unresponsive to conventional therapies, ECMO is required. While the surgical repair is relatively simple, determining the optimal timing for surgery in patients

Could a Two-Staged Repair Be the Solution to the Dilemma of Repair Timing for Severe Congenital Diaphragmatic Hernia Requiring Extracorporeal Membrane Oxygenation? Read More »

The Pulmonary Artery Pulsatility Index Provides No Additional Prognostic Information in Pediatric Pulmonary Arterial Hypertension

Faye E. Smits, Chantal Lokhorst, Marlies G. Haarman , Mark-Jan Ploegstra , Rolf M. F. Berger, Johannes M. DouwesBeatrix Children’s Hospital, University Medical Center Groningen and University of Groningen.Netherlands ChildrenChildren 2024; 11: DOI: 10.3390/children11101152 AbstractBackground/objectives: The pulmonary artery pulsatility index (PAPi, calculated as (SPAP – DPAP)/mRAP) has been suggested as a measure of right ventricular-vascular coupling (RVVC)

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Clinical Implications of Pretest Probability of HFpEF on Outcomes in Precapillary Pulmonary Hypertension

Yogesh N. V. Reddy, Robert P. Frantz, Paul M. Hassoun, Anna R. Hemnes, Evelyn Horn, Jane A. Leopold, Franz Rischard, Erika B. Rosenzweig, Nicholas S. Hill, Serpil C. Erzurum, Gerald J. Beck, J. Emmanuel Finet, Christine L. Jellis, StephenC. Mathai, W. H. Wilson Tang, Barry A BorlaugMayo Clinic. Johns Hopkins University. Vanderbilt University Medical Center. Weill

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Impact of Persistent Pulmonary Hypertension of the Newborn in Neonates with Dextro-transposition of the Great Arteries

Marco Modestini, Dario Massari, Susanne Huisman, Michel M.R.F. Struys, Marcus T.R. Roofthooft, Jaap Jan VosBeatrix Children’s Hospital, University Medical Center Groningen and University of Groningen.Netherlands Journal of Cardiothoracic and Vascular AnesthesiaJ Cardiothorac Vasc Anesth 2024; DOI: 10.1053/j.jvca.2024.09.141 AbstractObjectives: This study aimed to assess the impact of persistent pulmonary hypertension of the newborn (PPHN) on perioperative morbidity and

Impact of Persistent Pulmonary Hypertension of the Newborn in Neonates with Dextro-transposition of the Great Arteries Read More »

Ventricular Dysfunction in Patients With Congenital Diaphragmatic Hernia Who Die After Repair

Catherine M. Avitabile, Yang Wang, Devon Ash, Sabrina J. Flohr, Leny Mathew, Natalie Rintoul, Holly L. HedrickPerelman School of Medicine at the University of Pennsylvania and Children’s Hospital of Philadelphia. United States Journal of Pediatric SurgeryJ Pediatr Surg 2024; 60: DOI: 10.1016/j.jpedsurg.2024.162002 AbstractBackground: Quantitative echocardiographic (echo) measures of ventricular function predict mortality in pediatric pulmonary hypertension (PH),

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