Pediatric Pulmonary Vascular Disease

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,

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

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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Infantile hemangiomatosis, a rare cause of high-output heart failure and pulmonary hypertension in the newborn baby: a case report

Nurdan Erol, Abdullah Alpinar, Nihan Uygur KulcuHealth Sciences University Zeynep Kamil Women and Children Diseases Training and Research Hospital.Turkey Cardiology in the YoungCardiol Young 2023; DOI: 10.1017/S1047951123001488 AbstractInfantile hemangiomatosis is among the most common vascular tumours of childhood that is generally accepted as benign. Some cases may have multiple hemangiomas with organ involvement, especially of the

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Sedation Prior to Intubation at Birth in Infants with Congenital Diaphragmatic Hernia: An International Survey on Current Practices

Emily J. J. Horn-Oudshoorn, Alisa M. Blekherov, Gerbrich E. van den Bosch, Sinno H. P. Simons, Ronny Knol, Arjan B. te Pas, Irwin K. M. Reiss, Philip L. J. DeKoninckErasmus MC University Medical Center. Leiden University Medical Center. Netherlands NeonatologyNeonatology 2023; 120: 434-440DOI: 10.1159/000530573 AbstractIntroduction: Infants with congenital diaphragmatic hernia (CDH) are commonly intubated immediately after birth.

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Surgical treatment of anomalous right upper lobe pulmonary vein obstruction caused by compression between pulmonary artery and trachea: a case report

Benqung Zhang, Jianming Xia, Xiaoqi WangFuwai Yunnan Cardiovascular Hospital. Chinese Academy of Medical Sciences and Peking Union Medical College. China Cardiology in the YoungCardiol Young 2023; DOI: 10.1017/S1047951123002494 AbstractThe normal anatomical course of right upper lobe pulmonary vein involves drainage anteriorly to the pulmonary artery, ultimately reaching the left atrium. However, anomalies can occur with the

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Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects

Hidekazu Ishida, Jun Maeda, Keiko Uchida, Hiroyuki YamagishiOsaka University Graduate School of Medicine. Tokyo Metropolitan Children’s Medical Center. Keio University of Medicine and Health Center.Japan Journal of Cardiovascular Development and DiseaseJ Cardiovasc Dev Dis 2023; DOI: 10.3390/jcdd10080333 AbstractAlthough pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between

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