alveolar capillary dysplasia (includes: congenital alveolar capillary dysplasia, CACD, familial persistent pulmonary hypertension of the newborn)

Epidemiology

• transitional pulmonary hypertension occurs in 1.9 of every 1000 newborn infants

Pathology

• failure of the transition to a cardiorespiratory circulation at birth results in persistent pulmonary hypertension of the newborn
• elevated pulmonary vascular resistance with extrapulmonary right-to-left shunting of blood across the patent ductus arteriosus or the foramen ovale,
• persistent pulmonary hypertension can cause life-threatening hypoxemia in newborn infants
• mortality rate of 11%

Genetics

• associated with defects in CPS1

More general terms

• congenital anomaly (birth defect)
• familial primary pulmonary hypertension

Additional terms

• alveolar
• capillary
• dysplasia

Database

• OMIM: https://mirror.omim.org/entry/265380