Uhl’s anomaly: A one and a half ventricular repair in a patient presenting with cardiac arrest

Abstract

Uhl's anomaly, first reported in 1952, is an extremely rare congenital cardiac defect characterized by partial or complete loss of the right ventricular myocardium and unknown etiology. Fewer than 100 cases have been described. The response to medical management is poor and there is no known ideal surgical approach or timing for treatment. We report the case of a previously active adolescent male presenting with cardiac arrest, who underwent successful bidirectional cavopulmonary anastomosis ("Glenn" anastomosis) with right atrial reduction and right ventricular free wall plication.

Univentricular palliation for a child with severe Uhl's anomaly

“…There are few reports of successful surgery in the literature, which included one-and-one half ventricular correction, 4 , 5 , 7 RV volume reduction and atrial septal fenestration, univentricular palliation, 3 , 6 , 7 and heart transplantation. 12 Although the optimal surgical strategy and the timing for the operation are uncertain, in some cases, univentricular palliation has been considered as a more appropriate approach, 3 , 6 in which the RV exclusion was considered a necessary procedure.…”

“…1 , 2 , 3 Fewer than 100 cases have been described, and there are no more than 10 patients who have received successful surgical treatment. 4 At present, there is no consensus on the surgical strategy for Uhl's anomaly. 2 , 3 , 4 , 5 , 6 , 7 , 8 We present our surgical experience of a 20-month-old child with Uhl's anomaly and discuss the surgical approach for this anomaly.…”

“… 4 At present, there is no consensus on the surgical strategy for Uhl's anomaly. 2 , 3 , 4 , 5 , 6 , 7 , 8 We present our surgical experience of a 20-month-old child with Uhl's anomaly and discuss the surgical approach for this anomaly.…”

Uhl's Anomaly in Adulthood

“…Previously described surgical techniques included single ventricle, one-and-a-half ventricular repairs, Fontan procedure, and heart transplant. 5 In our case, we described bidirectional "Glenn" anastomosis, usually performed when there is a normally functioning left ventricle and a dysfunctional RV that cannot handle the whole cardiac output. 5,8 Heart-lung interactions can be challenging when managing RV failure.…”

“…[1][2][3] Clinical presentation includes manifestations of RV failure, arrhythmias, sudden cardiac arrest, and cyanosis if there is atrial-level shunting. 5,6 Uhl's anomaly has a poor prognosis, but some patients can reach adulthood. 7 Here, we will discuss the case of a 29-year-old female with Uhl's anomaly who came to our center with symptoms of right ventricular failure.…”