Pediatric Pulmonary Vascular Disease

Pulmonary hypertension- a novel phenotypic hypothesis of Kabuki syndrome: a case report and literature review

Xiao‑xian Deng, Bo‑wen Jin, Shan‑shan Li, Hong‑mei Zhou, Qun‑shan Shen, Yun‑yan LiWuhan Asia Heart Hospital. China BioMed Central PediatricsBMC Pediatr 2023; 23: DOI: 10.1186/s12887-023-04273-x AbstractBackground: Pediatric pulmonary hypertension (PH) is a serious and rare disease that is often derived from genetic mutations. Kabuki syndrome (KS) is a chromosomal abnormality disease that has its origin in the mutation […]

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Growing up with Idiopathic Pulmonary Arterial Hypertension: An Arduous Journey

Tanguy Dutilleux, Nesrine Farhat, Ruth Heying, Marie-Christine Seghaye, Maurice BeghettiUniversity Hospital Aachen. Centre Hospitalier Universitaire de Liège. University Hospital Leuven. Children’s University Hospital Geneva.Germany, Belgium and Switzerland Pediatric ReportsPediatr Rep 2023; 15: 301-310DOI: 10.3390/pediatric15020026 AbstractIdiopathic pulmonary arterial hypertension (IPAH) is an uncommon and severe disease. We report the case of a 7-year-old boy investigated for cardiac

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Intravenous sildenafil for treatment of early pulmonary hypertension in preterm infants

Lukas Schroeder, Paulina Monno, Brigitte Strizek, Till Dresbach, Andreas Mueller, Florian KipfmuellerUniversity Children’s Hospital Bonn and University Hospital Bonn.Germany Scientific ReportsSci Rep 2023; 13DOI: 10.1038/s41598-023-35387-y AbstractData is lacking on the effect of continuous intravenous sildenafil treatment in preterm infants with early pulmonary hypertension (PH), especially in very low birth weight (VLBW) infants. Preterm infants (< 37

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Neonatal Outcomes of Term Infants Born with Meconium-Stained Amniotic Fluid

Carlo Dani, Martina Ciarcià1, Vittoria Barone, Mariarosaria Di Tommaso, Federico Mecacci, Lucia Pasquini, Simone PratesiCareggi University Hospital, Largo Brambilla. University of Florence. Italy ChildrenChildren 2023; 10 DOI: 10.3390/children10050780 AbstractBackground Meconium-stained amniotic fluid (MSAF) is considered an alarming sign of possible fetal compromise and it has recently been reported that neonatal outcome correlates with the degree of meconium

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Pediatric pulmonary arterial hypertension due to a novel homozygous GDF2 missense variant affecting BMP9 processing and activity

L. Chomette, E. Hupkens, M. Romitti, L. Dewachter, J. L.Vachiéry, S. Bailly, S. Costagliola, G. Smits, E. Tillet, Antoine BondueHôpital Erasme, Hôpital Universitaire de Bruxelles and Université Libre de Bruxelles. Laboratory BioSanté and Université Grenoble Alpes.Belgium and France American Journal of Medical GeneticsAm J Med Genet 2023; 191(8):2064-2073DOI: 10.1002/ajmg.a.63236 AbstractPulmonary arterial hypertension (PAH) is a rare

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Evaluation of preterm infants having bronchopulmonary dysplasia with echocardiography and serum biomarkers

O. Pamukcu, N. Narin, S. Sunkak, A. TuncayErciyes University School of Medicıne.Turkey Cardiology in the YoungCardiol Young 2023; DOI: 10.1017/S1047951123001361 AbstractBackground and objectives: Pulmonary hypertension is frequent in infants with bronchopulmonary dysplasia. Echocardiography is easy to perform, non-invasive, and recommended by guidelines even though solely it is not enough. Catheterisation is gold standard but invasive, expensive, and

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Severe Pulmonary Arterial Hypertension in Healthy Young Infants: Single Center Experience

Shrikiran Aroor, Koushik Handattu, Suneel C. Mundkur, Aditya Verma, Praveen C. Samuel, Akkatai S. Teli, Gujan BangaKasturba Medical College. India Indian PediatricsIndian Pediatr 2023; AbstractObjectives: We studied the clinical presentation and management of acute pulmonary arterial hypertension (PAH) in healthy young infants, and the effect thiamine therapy.Methods: Review of hospital records was conducted for 56 healthy infants

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Macitentan in the Young-Mid-term Outcomes of Patients with Pulmonary Hypertensive Vascular Disease treated in a Pediatric Tertiary Care Center

Sulaima Albinni, Julian Heno, Imre Pavo, Erwin Kitzmueller, Manfred Marx, Ina Michel‑BehnkeMedical University of Vienna.Austria Paediatric DrugsPaediatr Drugs 2023; 25: 467-481DOI: 10.1007/s40272-023-00573-y AbstractBackground: Pulmonary hypertension (PH) is a severe hemodynamic condition with high morbidity and mortality. Approved targeted therapies are limited for pediatric subjects, and treatments are widely adopted from adult algorithms. Macitentan is a safe and effective drug used for adult PH,

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Novel scoring tool of hypoxemic respiratory failure and pulmonary hypertension for defining severity of persistent pulmonary hypertension of newborn

Sage Timberline, Avni Bhatt, Sherzana Sunderji, Daniel J. Tancredi, Satyan Lakshminrusimha, Heather SiefkesUniversity of Virginia. University of California, Davis. United States Journal of PerinatologyJ Perinatol 2023; DOI: 10.1038/s41372-023-01762-w AbstractObjective: To obtain preliminary validity data for a hypoxemic respiratory failure/pulmonary hypertension (HRF/PH) score for classifying persistent pulmonary hypertension of the newborn (PPHN).Study design: Retrospective chart review of 100 consecutive

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Efficacy of Sildenafil in Infants with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension

Kacie Dillon, Vineet Lamba, Ranjit R. Philip, Mark F. Weems, Ajay J. Talati University of Tennessee Health Science Center.United States ChildrenChildren 2023; DOI: 10.3390/children10081397 AbstractBackground: Pulmonary hypertension (PH) is a common comorbidity in infants with bronchopulmonary dysplasia (BPD). Sildenafil is a widely recognized therapy for PH, but its efficacy in infants with BPD is questionable. We propose

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