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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
Related
alveolar
capillary
dysplasia
General
congenital anomaly (birth defect)
familial primary pulmonary hypertension
Database Correlations
OMIM 265380
References
OMIM :accession 265380