Cardiac arrest in an adolescent with Uhl’s anomaly: Two unusual cardiac arrhythmia mechanisms

Moak, Jeffrey P.; Pearson, Gail D.; Clark, Bradley C.; Berul, Charles I.; Cross, Russell R.; Nath, Dilip S.

doi:10.1016/j.hrcr.2016.03.004

Cited by 4 publications

(2 citation statements)

References 11 publications

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“…Six days after admission, and after significant clinical recovery, he underwent an electrophysiology study which revealed an unusual form of AV nodal reentrant tachycardia (narrow QRS tachycardia) and fascicular-ventricular reentrant circuit (wide complex tachycardia) [3]. Given the gravity of his presentation and the unusual nature and severity of his anatomic derangements, a number of options were considered, including heart transplantation, but the family was not amenable to this option.…”

Section: Case Reportmentioning

confidence: 99%

“…Histopathologically, the myocardial layer is replaced by nonfunctional fibroelastic tissue resulting in the right ventricular free wall having a parchment-like appearance. These changes lead to progressive right heart failure and/or significant arrhythmias, including ventricular arrhythmias, bilateral bundle branch block, and atrioventricular (AV) block [1], [2], [3], [4], [5], [6]. Uhl’s anomaly typically presents in infancy [2], which was the case in our patient.…”

Section: Introductionmentioning

confidence: 99%

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

Chounoune

Lowry

Ramakrishnan

et al. 2018

Journal of the Saudi Heart Association

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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.

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Section: Case Reportmentioning

confidence: 99%