Alveolar capillary dysplasia with misalignment of the pulmonary veins associated with aortic coarctation and intestinal malrotation

• Published in: Journal of perinatology Vol. 34; no. 10; pp. 795 - 797
• Main Authors: Arreo del Val, V, Avila-Alvarez, A, Schteffer, L R, Santos, F, Deiros, L, Del Cerro, M J
• Format: Journal Article
• Language: English
• Published: New York Nature Publishing Group US 01.10.2014; Nature Publishing Group
• Subjects: 692/699/1503/1581; 692/699/1785; 692/699/75/593; 692/700/1720/3186; Abnormalities, Multiple - diagnosis; Abnormalities, Multiple - therapy; Alveoli; Aorta; Aortic Coarctation - diagnostic imaging; Aortic Coarctation - surgery; Autopsies; Autopsy; Biopsy; Bronchopulmonary dysplasia; Cardiac Surgical Procedures - methods; Care and treatment; Case studies; Development and progression; Diagnosis; Digestive System Abnormalities - diagnosis; Digestive System Abnormalities - surgery; Disease Progression; Dysplasia; Echocardiography, Doppler; Fatal Outcome; Heart; Humans; Hypertension; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Infant, Newborn; Infant, Premature; Intestinal Volvulus - diagnosis; Intestinal Volvulus - surgery; Male; Medical examination; Medicine; Medicine & Public Health; Misalignment; Mutation; Nitric oxide; Pediatric Surgery; Pediatrics; perinatal-neonatal-case-presentation; Persistent Fetal Circulation Syndrome - diagnosis; Postoperative Complications - pathology; Pulmonary arteries; Pulmonary hypertension; Pulmonary veins; Rare Diseases; Respiratory Distress Syndrome, Newborn - diagnosis; Respiratory Distress Syndrome, Newborn - etiology; Veins; Veins & arteries; Ventilators; Madrid Spain; Spain
• ISSN: 0743-8346; 1476-5543
• DOI: 10.1038/jp.2014.94

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare and lethal cause of refractory pulmonary hypertension of the newborn. We describe the clinical course of a neonate with refractory pulmonary hypertension diagnosed with ACD/MPV, aortic coarctation and other not previously reported associated malformations.