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Combined Heart and Liver Transplantation for Uhl's Anomaly: A Case Report
Filippo Landi1, Elena Sandoval2, Julia Martinez3
1Department of General and Digestive Surgery, Hospital Clinic, CIBEREHD, IDIBAPS, University of Barcelona, Barcelona, Spain.
Insights
This case report details the first successful combined heart-liver transplant for Uhl
Area of Science:
- Cardiology
- Transplantation Surgery
- Hepatology
Background:
- Uhl's anomaly is a rare congenital heart defect involving absent right ventricular myocardium.
- It typically presents with a poor prognosis, often fatal in the perinatal period.
- Adult survival is exceptionally rare, posing unique clinical challenges.
Observation:
- A 42-year-old male patient with Uhl's anomaly presented with heart failure and cardiac cirrhosis.
- The patient underwent a combined heart and liver transplant procedure.
- The surgery involved bicaval and piggyback techniques with specific ischemia times for each organ.
Findings:
- The patient experienced no intraoperative complications and was discharged on postoperative day 22.
- Pathology confirmed severe right ventricular cardiomyopathy and advanced liver cirrhosis.
- At 27 months post-transplant, the patient remains free of hospital admissions with normal graft function.
Implications:
- This case demonstrates the feasibility of combined heart-liver transplantation for end-stage Uhl's anomaly in adults.
- It highlights successful management principles for complex right heart disorders complicated by liver failure.
- This approach offers a potential life-saving option for rare congenital heart conditions.
Background:
Uhl's anomaly is an extremely rare congenital heart defect characterized by absence of the right ventricle myocardium and preserved left ventricular myocardium. Although the disease has a poor prognosis and is generally fatal in the perinatal period, some patients may reach adulthood.
Methods:
We describe a case of Uhl's anomaly complicated with heart failure and decompensated cardiac cirrhosis in a 42-year-old man treated by combined heart-liver transplant.
Results:
The patient underwent heart transplant using the bicaval technique followed by subsequent liver transplant with the piggyback technique without venovenous bypass. Total ischemia time was 108 minutes for the heart and 360 and 25 minutes of cold and warm ischemia, respectively, for the liver. No intraoperative complications occurred. The patient was discharged without severe complications on postoperative day 22. Pathologic examination of the organs reported advanced cirrhosis of the liver and severe dilated myocardiopathy of right ventricle with absence of myocardium and a normal left ventricle. Twenty-seven months after the transplant the patient has been free from hospital admissions, with normal function of both transplanted organs.
Conclusions:
We report the first successful combined heart-liver transplant for Uhl's anomaly indication in an adult patient. Despite of the insufficient knowledge of natural history of this exceptional disease, we successfully apply the management principles of other end-stage right heart disorders complicated with liver failure.
