Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome

Pulmonary hypertension (PH) at high altitudes presents unique characteristics due to hypobaric hypoxia. We aimed to evaluate the association between early diagnosis and clinical outcomes among children with severe PH living at high altitudes. A retrospective analysis was conducted on 86 children, divided into three age groups at the time of diagnosis: ≤3, 3–10, and ≥10 years.

Ventricular septal defect (VSD) is one of the most common congenital heart defects. Large, uncorrected VSDs can lead to significant left-to-right shunting, resulting in pulmonary arterial hypertension (PAH) and progressive pulmonary vascular disease. Early surgical intervention is recommended but is not always possible due to various socioeconomic and health infrastructure-related issues, especially in countries like India.

The right ventricle (RV), because of its anatomic location and contractile pattern, has remained an elusive target for accurate characterization by echocardiography in both healthy children and various disease states. As such, cardiac magnetic resonance (CMR) imaging remains the gold standard for myocardial assessment and RV function.

Pulmonary arterial endothelial and smooth muscle cell homeostasis is regulated through the bone morphogenetic protein (BMP) and transforming growth factor beta (TGF-β) receptor pathways. Pathway imbalance results in pulmonary hypertension (PH). Each pathway has ligands and modulators influencing this balance. How these pathways differ in pediatric PH patients is unknown.