Pulmonary Hypertension in Children across Africa: The Silent Threat

Table 2

Classification of pediatric pulmonary hypertension [3, 5, 19]. denotes causes in the pediatric population.

Class 1	Subclassification
PAH	Idiopathic
	Heritable, BMPR2, ALK
	Drug-induced
PAH associated with other diseases	Untreated CHD,^-significant left to right shunt with increased pulmonary blood flow
	HIV
	Portal hypertension
	Schistosomiasis
Pulmonary venoocclusive disease	PPHN
PH due to left heart disease	LV systolic dysfunction, cardiomyopathies, unoperated ALCAPA
	LV diastolic dysfunction, HOCM
	Valvular disease, advanced RHD with severe MR
	CHD with LV/inflow/outflow obstruction, subaortic and aortic valve stenosis with/out BAV, congenital mitral stenosis, cor triatriatum, supramitral ring
PH due to lung disease	Chronic obstructive lung disease, congenital pulmonary vein stenosis
	Interstitial lung disease; pulmonary tuberculosis, bronchopulmonary dysplasia, LIP in HIV
	Disordered sleep breathing
	Altitude-related disease
Chronic thromboembolic disease
PH due to other causes	Hematological SCA—common in African children
	Metabolic disorders such as glycogen storage disorders, Hunter’s syndrome
	Systemic disorders
	Adenotonsillar hypertrophy
Multifactorial	Endomyocardial fibrosis

BMPR2: bone morphogenetic protein type II receptor; ALK: activin-like kinase type 1; BAV: bicuspid aortic valve; LIP: lymphocytic interstitial pneumonitis; ALCAPA: anomalous origin of the left coronary artery from the pulmonary artery; HIV: human immunodeficiency virus; HOCM: hypertrophic cardiomyopathy; MR: mitral regurgitation.