Single Ventricle Strategy for Uhl's Anomaly of the Right Ventricle

Clinical presentation and echocardiographic characteristics of Uhl's anomaly

“…Management of Uhl's anomaly is most often restricted to palliative care; however, reported surgical options include total cavopulmonary conversion of the right heart, additional plication of the RV, and cardiac transplantation . While the majority of patients with Uhl's anomaly are diagnosed prenatally or in infancy and rarely survive to adulthood, it is important to consider this disorder with an index of suspicion in patients presenting with RV morphologic derangements and intractable heart failure.…”

Uhl’s Anomaly: A Rare Cause of Cardiomegaly

“…However, high-risk clot formation in the RV and occurrence of embolization are thought to be much more common in surgery bypassing the right side of the heart (especially, bidirectional Glenn shunt, and one-and-a-half ventricle repair). Hoschtitzky et al (11) described an operation for surgical exclusion of the right ventricle to reduce the detrimental effects of ventricular distension and ventriculo-ventricular interaction from septal shift toward the left. Our case was also admitted with cardiac failure symptoms, such as frequent breathing and poor sucking.…”

Repairing the tricuspid valve in congenital heart diseases other than Ebstein’s

“…8,9 The right ventricle is dilated, resulting in right heart failure and tricuspid valve insufficiency, and the majority of patients present in infancy, as survival into adulthood is rare without intervention (Fig 4). 5,[9][10][11][12][13][14] Antegrade flow to the pulmonary arteries depends on right atrial contraction and paradoxical septal motion. 12 Surgical options include conversion to single-ventricular physiology and/or cardiac transplantation if the single-ventricle strategy fails.…”

“…12 Surgical options include conversion to single-ventricular physiology and/or cardiac transplantation if the single-ventricle strategy fails. [9][10][11] In select patients, this initially involves atrial septectomy, tricuspid valve exclusion, and a bidirectional Glenn anastomosis, with or without right ventricular plication. 10,[12][13][14] These variants of one-and-a-half repair may subsequently allow completion of total cavopulmonary anastomosis.…”

“…[9][10][11] In select patients, this initially involves atrial septectomy, tricuspid valve exclusion, and a bidirectional Glenn anastomosis, with or without right ventricular plication. 10,[12][13][14] These variants of one-and-a-half repair may subsequently allow completion of total cavopulmonary anastomosis. 11,12 The results of surgery have been disappointing in the past, but more recent reports based on earlier diagnosis and a more aggressive approach towards single-ventricle palliation and right Photograph showing a transatrial view of the tricuspid valve and thinned right ventricular cavity in a patient with Uhl's Anomaly in an 18-year-old female patient.…”