Key result
Central shunt with PDA and MPA ligation successfully stabilizes infant Uhl's anomaly masquerading as pulmonary hypertension.
Why the study?
Uhl's anomaly is a rare cause of right ventricular failure in infants that can mimic pulmonary hypertension and is often under-recognized until pulmonary vascular resistance is accurately assessed.
Case Report (n=1)
May offer palliation option in neonatal Uhl's anomaly; leaves open need for larger series before practice change.
We report a case of Uhl's anomaly in a 3-week-old infant that underwent central shunt placement, patent duct us arteriosus and main pulmonary artery ligation. The infant presented with room air saturation of 43%, dilated right ventricle with decreased function and dilated right atrium. Diagnosis was established with a myocardial biopsy.
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Ganczar et al. (2015) conducted a case report in Uhl's anomaly (n=1). Central shunt placement, patent ductus arteriosus and main pulmonary artery ligation was evaluated. A 3-week-old infant with Uhl's anomaly masquerading as pulmonary hypertension was successfully managed with a central shunt along with patent ductus arteriosus and main pulmonary artery ligation.
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