Combined heart and liver transplantation in a patient supported by left ventricular assist device (LVAD) with propionic acidemia.
Nutr Metab Cardiovasc Dis
; 33(3): 667-670, 2023 03.
Article
in En
| MEDLINE
| ID: mdl-36710110
ABSTRACT
Propionic acidemia (PA) is a rare inherited metabolic disease due to inborn errors of metabolism. PA results in the accumulation of abnormal organic acid metabolites in multiple systems, mainly the central nervous system and the heart. Cardiac complications include dilated cardiomyopathy (DCM) and carry a 40-50% increased mortality risk. Liver transplantation (LT) is required in PA patients when medical treatment fails and may prevent or slow down the cardiomyopathy progression. However, severe heart disease may be a serious contraindication to LT. We present a complicated case of a PA patient, supported with a Left Ventricular Assist Device, who underwent a heart and Liver transplant. PA patients are at increased risk for metabolic acidosis during surgery, with increased anion gap and hyperammonemia. A strict multi-disciplinary approach is needed to prevent and treat metabolic decompensation. The patient had a successful heart and liver transplant after a strict treatment protocol in the pre, intra, and post-operative periods. His case highlights the complexity of PA patients and the increased risk for metabolic decompensation during surgery and provides an insight into how to manage such complicated patients.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Heart-Assist Devices
/
Liver Transplantation
/
Propionic Acidemia
/
Cardiomyopathies
Type of study:
Diagnostic_studies
/
Etiology_studies
/
Guideline
Limits:
Humans
/
Male
Language:
En
Journal:
Nutr Metab Cardiovasc Dis
Journal subject:
ANGIOLOGIA
/
CARDIOLOGIA
/
CIENCIAS DA NUTRICAO
/
METABOLISMO
Year:
2023
Type:
Article