Key result
Staged single-ventricle palliation succeeds in a newborn with Uhl's anomaly and absent pulmonary valve.
Why the study?
Uhl's anomaly is a very rare congenital malformation characterized by absence of right ventricular myocardium, with diagnosis previously made post-mortem but now possible prenatally due to advances in cardiac imaging.
Case Report (n=1)
Staged palliation succeeded in this rare neonatal case; leaves open whether single-ventricle strategy improves outcomes in Uhl anomaly with absent pulmonary valve.
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.
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Kim et al. (2021) conducted a case report in Uhl's anomaly (n=1). Surgical palliation (right modified Blalock-Taussig shunt, tricuspid and pulmonary valve obliteration, atrial septectomy) was evaluated. A newborn male with prenatally diagnosed Uhl's anomaly, dysplastic tricuspid valve, and absent pulmonary valve leaflets successfully underwent staged single-ventricle palliation surgery.
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