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Case Report

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

by
María Jesús Valero
1,
Jose L. Muñoz-Blanco
2,
Alejandro Garrido Sanchez
3,
Gregorio Cuerpo
4,
Javier Castrodeza
1,
Paula Navas
1,
Iago Sousa
1,
Adolfo Villa
1,
Francisco Fernández-Avilés
1,5 and
Manuel Martínez-Sellés
1,2,6,*
1
Department of Cardiology, Hospital Universitario Gregorio Marañón, CIBERCV, 28007 Madrid, Spain
2
ALS-Neuromuscular Unit, Department of Neurology, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain
3
Cardiac Surgery Postoperative Care Unit, Department of Anestesiology, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain
4
Department of Cardiac Surgery, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain
5
Department of Medicine, Universidad Complutense, 28040 Madrid, Spain
6
Department of Medical Sciences, Universidad Europea, 28670 Madrid, Spain
*
Author to whom correspondence should be addressed.
J. Cardiovasc. Dev. Dis. 2022, 9(3), 80; https://doi.org/10.3390/jcdd9030080
Submission received: 10 February 2022 / Revised: 3 March 2022 / Accepted: 7 March 2022 / Published: 9 March 2022
Versions Notes

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.
Keywords: Friedreich’s ataxia; heart transplantation; ethics; prognosis; neuromuscular disease Friedreich’s ataxia; heart transplantation; ethics; prognosis; neuromuscular disease

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MDPI and ACS Style

Valero, M.J.; Muñoz-Blanco, J.L.; Sanchez, A.G.; Cuerpo, G.; Castrodeza, J.; Navas, P.; Sousa, I.; Villa, A.; Fernández-Avilés, F.; Martínez-Sellés, M. Should Advanced Friedreich’s Ataxia Be a Contraindication for Heart Transplantation? A Case Report of a Successful Procedure in a 58-Year-Old Patient. J. Cardiovasc. Dev. Dis. 2022, 9, 80. https://doi.org/10.3390/jcdd9030080

AMA Style

Valero MJ, Muñoz-Blanco JL, Sanchez AG, Cuerpo G, Castrodeza J, Navas P, Sousa I, Villa A, Fernández-Avilés F, Martínez-Sellés M. 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. 2022; 9(3):80. https://doi.org/10.3390/jcdd9030080

Chicago/Turabian Style

Valero, María Jesús, Jose L. Muñoz-Blanco, Alejandro Garrido Sanchez, Gregorio Cuerpo, Javier Castrodeza, Paula Navas, Iago Sousa, Adolfo Villa, Francisco Fernández-Avilés, and Manuel Martínez-Sellés. 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, no. 3: 80. https://doi.org/10.3390/jcdd9030080

APA Style

Valero, M. J., Muñoz-Blanco, J. L., Sanchez, A. G., Cuerpo, G., Castrodeza, J., Navas, P., Sousa, I., Villa, A., Fernández-Avilés, F., & Martínez-Sellés, M. (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), 80. https://doi.org/10.3390/jcdd9030080

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