Arrhythmogenic Left Ventricular Cardiomyopathy: A Successful Case of Extracorporeal Cardiopulmonary Resuscitation

Authors

  • Mafalda Gama Unidade de Urgência Médica. Centro Hospitalar Universitário de Lisboa Central. Lisbon.
  • Isabel Cardoso Serviço de Cardiologia. Centro Hospitalar Universitário de Lisboa Central. Lisbon.
  • Mónica Palma Anselmo Hospital Professor Doutor Fernando da Fonseca. Lisbon.
  • Sílvia Aguiar Rosa Serviço de Cardiologia. Centro Hospitalar Universitário de Lisboa Central. Lisbon.
  • Pedro Gaspar da Costa Unidade de Urgência Médica. Centro Hospitalar Universitário de Lisboa Central. Lisbon.
  • Philip Fortuna Unidade de Urgência Médica. Centro Hospitalar Universitário de Lisboa Central. Lisbon.

DOI:

https://doi.org/10.20344/amp.19624

Keywords:

Arrhythmogenic Left Ventricular Cardiomyopathy, Cardiopulmonary Resuscitation, Extracorporeal Membrane Oxygenation, Heart Arrest/therapy

Abstract

A 24-year-old man suffered a witnessed cardiac arrest after a padel game. Basic life support was immediately provided. The pre-hospital emergency services team continued the resuscitation efforts, and the patient was accepted for extracorporeal cardiopulmonary resuscitation. The return of spontaneous circulation was achieved in 45 minutes. The initial assessment revealed a ST-segment elevation in leads V4-V6 and a dilated left ventricle with severe systolic dysfunction. Coronary angiography was normal. An improvement in left ventricular systolic function was observed and extracorporeal cardiac support was discontinued after 48 hours. Cardiovascular magnetic resonance imaging demonstrated hypokinesia and subepicardial fatty infiltration of the left ventricle lateral wall. Genetic testing detected a variant of uncertain significance in the ANK2 gene. The diagnosis of arrhythmogenic left ventricular myocardiopathy did not fulfill all the current diagnostic criteria, but it is a very likely diagnosis. An implantable cardioverter-defibrillator was placed. The
patient was discharged without physical or cognitive impairment.

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References

Corrado D, Zorzi A, Cipriani A, Bauce B, Bariani R, Beffagna G, et al. Evolving diagnostic criteria for arrhythmogenic cardiomyopathy. J Am Heart Assoc. 2021;10:e021987. DOI: https://doi.org/10.1161/JAHA.121.021987

Corrado D, Perazzolo Marra M, Zorzi A, Beffagna G, Cipriani A, Lazzari M, et al. Diagnosis of arrhythmogenic cardiomyopathy: the Padua criteria. Int J Cardiol. 2020;319:106-14. DOI: https://doi.org/10.1016/j.ijcard.2020.06.005

Corrado D, Basso C. Arrhythmogenic left ventricular cardiomyopathy. Heart. 2022;108:733-43. DOI: https://doi.org/10.1136/heartjnl-2020-316944

Hermans G, Clerckx B, Vanhullebusch T, Segers J, Vanpee G, Robbeets C, et al. Interobserver agreement of Medical Research Council sum-score and handgrip strength in the intensive care unit. Muscle Nerve. 2012;45:18-25. DOI: https://doi.org/10.1002/mus.22219

Richardson AS, Tonna JE, Nanjayya V, Nixon P, Abrams DC, Raman L, et al. Extracorporeal cardiopulmonary resuscitation in adults. Interim guideline consensus statement from the Extracorporeal Life Support Organization. ASAIO J. 2021;67:221-8. DOI: https://doi.org/10.1097/MAT.0000000000001344

Mohler PJ, Schott JJ, Gramolini AO, Dilly KW, Guatimosim S, duBell WH, et al. Ankyrin-B mutation causes type 4 long-QT cardiac arrhythmia and sudden cardiac death. Nature. 2003;421:634-9. DOI: https://doi.org/10.1038/nature01335

Allegue Toscano C, Coll Vidal M, Matés Ramírez J, Campuzano Larrea O, Iglesias A, Sobrino B, et al. Genetic analysis of arrhythmogenic diseases in the era of NGS: the complexity of clinical decision-making in brugada syndrome. PLoS One. 2015;7:e0133037. DOI: https://doi.org/10.1371/journal.pone.0133037

Roberts JD, Murphy NP, Hamilton RM, Lubbers ER, James CA, Kline C, et al. Ankyrin-B dysfunction predisposes to arrhythmogenic cardiomyopathy and is amenable to therapy. J Clin Investig. 2019;129:3171-84. DOI: https://doi.org/10.1172/JCI125538

Mohler PJ, Splawski I, Napolitano C, Bottelli G, Sharpe L, Timothy K, et al. A cardiac arrhythmia syndrome caused by loss of ankyrin-B function. Proc Natl Acad Sci. 2004;101:9137-42. DOI: https://doi.org/10.1073/pnas.0402546101

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Published

2023-06-16

How to Cite

1.
Gama M, Cardoso I, Palma Anselmo M, Aguiar Rosa S, Gaspar da Costa P, Fortuna P. Arrhythmogenic Left Ventricular Cardiomyopathy: A Successful Case of Extracorporeal Cardiopulmonary Resuscitation. Acta Med Port [Internet]. 2023 Jun. 16 [cited 2024 Nov. 23];36(9):598-602. Available from: https://actamedicaportuguesa.com/revista/index.php/amp/article/view/19624

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Section

Case Report