Key result
Surgical excision successfully resolves massive right ventricular thrombus in severe Uhl anomaly.
Why the study?
Uhl's anomaly is an extremely rare congenital cardiac malformation characterized by partial or complete absence of right ventricular myocardium with limited clinical reports on presentations such as large right ventricle thrombus.
Case Report (n=1)
Surgical excision may be considered for large RV thrombus in Uhl's anomaly; leaves open standardized protocols for this rare cardiomyopathy.
Uhl's anomaly is an extremely rare congenital cardiac malformation and is characterized by the partial or complete absence of right ventricular myocardium. The absence of myocardium may be the result of primary non-development of myocytes or a form of selective apoptosis. It is mainly sporadic although some familial occurrences have been reported. Congestive cardiac failure is the most common mode of presentation. Associated congenital cardiac malformations are also reported. We report a case of a 17-year-old male who presented with symptoms and signs of right heart failure, during evaluation found to have large right ventricle free wall thrombus.
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Singh et al. (2019) conducted a case report in Uhl's anomaly with right heart failure and right ventricular thrombus (n=1). Surgical excision of right ventricular thrombus was evaluated. A 17-year-old male with Uhl's anomaly and right heart failure developed a large right ventricular thrombus that was successfully managed with surgical excision.
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