Uhl's anomaly. Differential diagnosis and indication for cardiac transplantation in an infant.
نویسندگان
چکیده
We report the case of an 8-month-old female infant with Uhl's anomaly, who underwent successful cardiac transplantation. The clinical findings, complementary laboratory tests, anatomic findings, and differential diagnosis of the anomaly are discussed.
منابع مشابه
Congenital absence of myocardium of right ventricle: Uhl's anomaly.
Uhl's anomaly is an extremely infrequent cardiac malformation, characterized by an almost total absence of the right ventricular myocardium. This results in the inability of the right ventricle to pump blood into the lungs, a function that is carried out by right atrial contraction. The clinical picture is characterized by right heart failure. The first case was reported by Uhl (1952), in an in...
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We report a case of Uhl's anomaly in a 2-month-old infant who presented with critical ventricular arrhythmia. The patient underwent a successfully surgical repair by semitotal resection of the right ventricle free wall, tricuspid annuloplasty, and one and one-half ventricular repair.
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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 ma...
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متن کاملUhl's anomaly detected in‐utero
Uhl's anomaly is rarely diagnosed antenatally. It is a condition characterised by partial or complete absence of the right ventricular myocardium and the heart conducting system. We present a case of Uhl's anomaly diagnosed during fetal life on prenatal ultrasound, with eight years postnatal follow-up.
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ورودعنوان ژورنال:
- Arquivos brasileiros de cardiologia
دوره 77 1 شماره
صفحات -
تاریخ انتشار 2001