Etiology of pulmonary hypertension

Cardiac

Increased pulmonary blood flow associated with left-to-right shunts at the atrial, ventricular, or great vessel level

Examples: atrial septal defect, patent ductus arteriosus, transposition of the great arteries, truncus arteriosus

Left-sided obstructive lesions associated with pulmonary venous congestion.

Examples: coarctation of aorta, aortic stenosis, mitral stenosis, pulmonary vein stenosis or atresia, cor triatriatum

Cardiomyopathy

Pulmonary

Idiopathic: primary pulmonary hypertension, veno-occlusive disease.

Hypoxic vasoconstriction

Alveolar hypoventilation: sleep disorders, upper airway obstruction, neuromuscular disorder, chest wall deformity

Obstructive or restrictive lung disease: cystic fibrosis, bronchopulmonary dysplasia, interstitial fibrosis

Pneumonia

High altitude

Pulmonary hypoplasia: primary, associated with congenital diaphragmatic hernia or renal dysplasia

Peripheral pulmonary stenosis

Persistent pulmonary hypertension of the newborn

Thromboembolic

Ventriculoatrial shunt, indwelling catheters, deep vein thrombosis

Parasitic disease: filariasis, schistosomiasis

Hematologic: sickle cell disease, polycythemia

Hepatic disease

Cirrhosis, portal hypertension

Collagen vascular disease

Scleroderma, systemic lupus erythematosus, rheumatoid arthritis, mixed connective tissue disease

Granulomatous disease

Sarcoidosis

Courtesy of Robert L Geggel, MD.

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