Left Ventricular Diastolic Dysfunction in Pediatric Pulmonary Hypertension
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Pulmonary hypertensive vascular disease (PHVD) is characterized by progressive narrowing of pulmonary arterioles, abnormally high pulmonary vascular resistance (PVR), right ventricular (RV) dysfunction, left ventricular (LV) compression, and death in ≈25% to 60% of patients with pulmonary arterial hypertension (PAH) 5 years after diagnosis.1,2 Meanwhile, consensus statements have been developed in North America and Europe specifically to guide the clinical care of children with pulmonary hypertension (PH).3,4 The challenges in the diagnosis5 and treatment6 of pediatric PH include early detection in the absence of specific symptoms or biomarkers, the complexity of the underlying etiologies, broad comorbidities, genetic syndromes, and the paucity of pediatric trial data to support and guide clinical management.7,8
See Article by Burkett et al
The estimated prevalence of PAH is 2 to 16 cases per million children.9–11 Children diagnosed with PAH associated with congenital heart disease and those with idiopathic/heritable PAH have a similar 5-year mortality in North America (29% versus 25%; REVEAL registry).1 Untreated idiopathic PAH results in death within 2 to 3 years in adults and within 1 year after diagnosis in children,12 indicating that early diagnosis and early, sufficient (probably combinatory) PAH-targeted therapy is paramount.
In children, PH is evident with a mean PA pressure ≥25 mm Hg when over 3 months of age at sea level.5,13 The term pediatric PAH (ie, group 1 PH) defines a subgroup of precapillary PH with an end-expiratory pulmonary artery wedge pressure (PAWP) <15 mm Hg and a PVR indexed to body surface area >3 WU m2. In 2011, the Pulmonary Vascular Research Institute introduced the disease entity pediatric PHVD (mean PA pressure ≥25 …