Should Advanced Friedreich’s Ataxia Be a Contraindication for Heart Transplantation? A Case Report of a Successful Procedure in a 58-Year-Old Patient

Impacto

Downloads

Downloads per month over past year

Valero Masa, María Jesús and Muñoz Blanco, Jose L. and Garrido Sánchez, Alejandro and Cuerpo, Gregorio and Castrodeza, Javier and Navarro López, Paula and Sousa Casasnovas, Iago and Villa Arranz, Adolfo and Fernández Avilés, Francisco and Martínez-Sellés d'Oliveira Soares, Manuel (2022) Should Advanced Friedreich’s Ataxia Be a Contraindication for Heart Transplantation? A Case Report of a Successful Procedure in a 58-Year-Old Patient. Journal of Cardiovascular Development and Disease, 9 (3). p. 80. ISSN 2308-3425

[thumbnail of jcdd-09-00080.pdf]
Preview
PDF
Creative Commons Attribution.

229kB

Official URL: https://doi.org/10.3390/jcdd9030080




Abstract

The information on heart transplantation (HT) in patients with Friedreich’s Ataxia (FA) is scarce, and the few published case reports are limited to young patients with mild neurological manifestations. We present the case of a 58-year-old patient with advanced FA (Scale for the Assessment and Rating of Ataxia [SARA] score 30/40), wheelchair-bound for the last 16 years and had urinary incontinence, dysarthria, and neurosensorial deafness. The patient was admitted for a refractory arrhythmic storm and had previous hypertrophic cardiomyopathy that evolved to dilated cardiomyopathy with severely reduced left ventricular ejection fraction and recurrent ventricular arrhythmias. A multidisciplinary team discussed the HT option. The patient was aware of the risks and benefits and considered worthy of the intervention, so he was listed for HT. After a successful surgical intervention, the patient had a long postoperative stay in ICU. He required a high dose of vasopressors, underwent hemofiltration for one month, suffered critical illness myopathy, had several respiratory infections and delayed tracheal extubation. Two and a half months after HT and almost five months at the hospital, the patient was successfully discharged. FA patients with severe heart conditions should be carefully evaluated by a multidisciplinary team to decide the candidacy for HT.


Item Type:Article
Uncontrolled Keywords:Friedreich’s ataxia; heart transplantation; ethics; prognosis; neuromuscular disease
Subjects:Medical sciences > Medicine > Cardiology
Medical sciences > Medicine > Surgery
ID Code:72858
Deposited On:29 Jun 2022 14:44
Last Modified:30 Jun 2022 07:28

Origin of downloads

Repository Staff Only: item control page