Uhl’s Anomaly is a rare congenital heart disease characterized by absence of myocardial fibers of the right ventricle. A severe cardiomegaly due to right ventricle dilation is a hallmark of this syndrome. Progressive right cardiac failure with pulmonary hypertension are the main symptoms, and start at an early age. Less frequent manifestations are arrhythmias and heart blocks. Mainstay treatment is symptomatic relief although surgical approaches have been described.
We describe a case of a 29-years-old, male patient diagnosed with Uhl’s Anomaly requiring urgent perianal abscess drainage. First symptoms appeared at the age of 22 years old when he presented with fatigue for mild efforts. No other diseases are known. He had close cardiology follow-up and was on symptomatic treatment and oral anticoagulation with rivaroxaban. Preoperative evaluation showed peripheral cyanosis, digital clubbing and heart failure classified as NYHA class II. Among the preoperative blood tests done, a thrombocytopenia and a prolonged prothrombin time were highlighted. On chest X- ray a marked cardiomegaly was seen. Electrocardiography showed right bundle branch block and atrial fibrillation. Previous echocardiogram showed severe right ventricle dilation with systolic failure, pulmonary hypertension and moderate left ventricle dysfunction with an estimated ejection fraction of 35%.
After a multidisciplinary discussion, it was decided to perform surgery under general anesthesia with invasive arterial pressure monitoring, BIS along with standard monitoring care. Multifunction external electrode pads were on position before induction of anesthesia. During intraoperative period there was no critical events such as pulmonary complications, hypotension or cardiac arrest. No vasopressor drugs were used. The recovery period was at cardiology intensive care unit. Postoperative cardiac markers remained negative.
The rarity of this heart condition and the urgent context of the procedure point out the relevance of this case. Preoperative evaluation of the patient, even in the urgent context, is of outmost importance. Understanding the disease and its limitations is essential before planning the anesthetic management of this patient.
A multidisciplinary approach to this patient was extremely important on deciding the perioperative management. Coordinated and timely actions of the different specialities involved contributed to the success of this case.

Uhl’s Anomaly: description of anesthetic management on an urgent context