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
en 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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Corazón Auxiliar
/
Trasplante de Hígado
/
Acidemia Propiónica
/
Cardiomiopatías
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
/
Guideline
Límite:
Humans
/
Male
Idioma:
En
Revista:
Nutr Metab Cardiovasc Dis
Asunto de la revista:
ANGIOLOGIA
/
CARDIOLOGIA
/
CIENCIAS DA NUTRICAO
/
METABOLISMO
Año:
2023
Tipo del documento:
Article