Familial persistent pulmonary hypertension of the newborn resulting from misalignment of the pulmonary vessels (congenital alveolar capillary dysplasia).

Misalignment of the pulmonary veins with congenital alveolar capillary dysplasia, although rare, has been reported as a cause of persistent pulmonary hypertension of the newborn. Reported cases have been mainly sporadic. Familial occurrence has been reported in only three instances. We present affected sibs with this condition. In addition to pulmonary abnormalities, urogenital abnormalities, including ureteric and urethral obstruction, seem to be common. Autosomal recessive inheritance is suggested.

[1]  L. Storme,et al.  Pulmonary hypertension of the newborn and urogenital anomalies in two male siblings: a new family with misalignment of pulmonary vessels. , 1996, Genetic Counseling.

[2]  E. Zackai,et al.  Misalignment of pulmonary veins with alveolar capillary dysplasia: affected siblings and variable phenotypic expression. , 1994, The Journal of pediatrics.

[3]  H. Abdallah,et al.  Late presentation of misalignment of lung vessels with alveolar capillary dysplasia. , 1993, Critical care medicine.

[4]  P. Cox,et al.  Persistent pulmonary hypertension of the newborn due to alveolar capillary dysplasia. , 1992, Pediatric pathology.

[5]  C. Langston.  Misalignment of pulmonary veins and alveolar capillary dysplasia. , 1991, Pediatric pathology.

[6]  H. Kilbride,et al.  Misalignment of lung vessels and alveolar capillary dysplasia: a cause of persistent pulmonary hypertension. , 1989, The Journal of pediatrics.

[7]  C. Wagenvoort.  Misalignment of lung vessels: a syndrome causing persistent neonatal pulmonary hypertension. , 1986, Human pathology.

[8]  J. Khorsand,et al.  Congenital alveolar capillary dysplasia: a developmental vascular anomaly causing persistent pulmonary hypertension of the newborn. , 1985, Pediatric pathology.

[9]  I. Shohet,et al.  Familial persistent pulmonary hypertension. , 1984, Archives of disease in childhood.

[10]  W. Fox,et al.  Persistent pulmonary hypertension in the neonate: diagnosis and management. , 1983, The Journal of pediatrics.

[11]  F. Askin,et al.  Congenital alveolar capillary dysplasia--an unusual cause of respiratory distress in the newborn. , 1981, American journal of clinical pathology.

[12]  L. Reid,et al.  Persistent fetal circulation: Newly recognized structural features. , 1976, The Journal of pediatrics.