A case of Uhl’s anomaly presenting with ventricular tachycardia

Univentricular palliation for a child with severe Uhl's anomaly

“…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. 1 , 9 …”

“…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. 1,9 Combined with CMR, ultrasound, and pathology results, we concluded that this case was one of the more severe forms of Uhl's anomaly, in which almost all of the right ventricular myocardium was missing, as a result, we performed a univentricular palliation rather than one and a half ventricle repair.…”

“…1,9 Combined with CMR, ultrasound, and pathology results, we concluded that this case was one of the more severe forms of Uhl's anomaly, in which almost all of the right ventricular myocardium was missing, as a result, we performed a univentricular palliation rather than one and a half ventricle repair. To prevent the huge RV from compressing the left ventricle and affecting its function, 10 we closed the remaining RV, and to eliminate the residual stagnant blood flow in the RV, which can possibly form thrombus that may fall into the systemic circulation and pulmonary circulation, 3 we closed the tricuspid valve and transected the pulmonary artery. Also, we enlarged the atrial septal defect to facilitate the flow of blood from the inferior vena cava through the atrial septum and into the left heart.…”