Search

Vertebral, anal, cardiac, tracheo-esophageal fistula, renal and limb (VACTERL) association is defined as a condition including at least three of the above-mentioned anomalies in the same infant. Several cardiac defects that have been reported as a part of the VACTERL association are ventricular and atrial septal defects, hypoplastic left heart syndrome, transposition of the great arteries and tetralogy of Fallot. Anomalous origin of pulmonary artery (AOPA) from the ascending aorta is an unusual and critical cardiovascular anomaly, which frequently involves the right pulmonary artery (RPA). A male neonate was delivered by normal spontaneous vaginal delivery at 39 weeks and 3 days gestation, weighting 2660 gm. He was diagnosed with VACTERL association with five abnormalities: vertebral abnormalities, anal atresia, cardiovascular anomaly (right pulmonary artery originating from ascending aorta), tracheo-esophageal fistula and renal anomalies. AOPA origination from ascending aorta as part of the VACTERL association in a neonate is a rare congenital cardiovascular malformation. Here we present a rare case of RPA originating from the ascending aorta seen with VACTERL association in a neonate.

Uhl’s anomaly is a very rare malformation of unknown cause, characterized by complete or partial absence of the right ventricular myocardium. The cardiac malformation causes progressive right heart failure, increased right-sided cardiac pressure, massive peripheral edema, and ascites. Patients usually present in infancy and rarely survive to adulthood. Previously, diagnosis was made at post-mortem evaluation, but advances in cardiac imaging now permit diagnosis during fetal life. We report a case of Uhl’s anomaly in a newborn baby imaged at 23 + 3 weeks of gestation by fetal echocardiography. There was an aneurysmally dilated thin-walled right ventricle with hypertrophy of the right ventricular apical muscles, the tricuspid valve was dysplastic, and the pulmonary valve leaflets were absent.