Surgical Options for Uhl’s Anomaly

Abstract

Uhl's anomaly is an extremely rare condition with varied clinical presentation. Surgical exclusion of the right ventricle yields gratifying results; however, surgical technique has to be adapted to the individual patient.

Univentricular palliation for a child with severe Uhl's anomaly

“…1 In patients with Uhl's anomaly, the myocardium of the left ventricle and the position of tricuspid valve are normal, even though tricuspid valve dysplasia may be presented in some cases. 1 , 7 , 9 The main manifestation of Uhl's anomaly is right heart failure, and some other manifestations may also appear, such as cyanosis, arrhythmia, thromboembolism, and conduction block. 7 , 10 , 11 Uhl's anomaly needs to be distinguished from the parchment heart, arrhythmogenic RV cardiomyopathy, and Ebstein 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 With Left Ventricular Noncompaction

“…The association of LVNC and Uhl’s anomaly has an important clinical implication for determining the appropriate management approach. Surgical RV exclusion with TCPC has been proposed for patients with Uhl’s anomaly, although the outcomes from this approach are unclear ( 6 , 7 , 8 , 9 ). Thorough evaluation of LV function and left-sided valvular hemodynamics is crucial before considering conversion to TCPC in patients with Uhl’s anomaly because this strategy transitions the hemodynamics from a 2-ventricle to single-ventricle circulation with its inherent limitations.…”

Uhl's Anomaly: 10 Years of Follow-Up of an Unoperated Patient

“…9,10,16 Although the surgical procedures for Uhl's provide reasonable short-term clinical improvement, the long-term outcome remains uncertain, and the prognosis is still adverse with high mortality. 2,16 There is no indication for primary prevention of sudden death in the presence of Uhl's anomaly. However, if secondary prevention is indicated, a subcutaneous implantable cardioverter-defibrillator is probably the most appropriate approach, given the risk for RV rupture.…”

“…Although the surgical procedures for Uhl's provide reasonable short-term clinical improvement, the long-term outcome remains uncertain, and the prognosis is still adverse with high mortality. 2 , 16 …”