Long term biventricular support with Berlin Heart Excor in a Septuagenarian with giant-cell myocarditis

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Abstract

Giant-cell myocarditis (GCM) is known as a rare, rapidly progressive, and frequently fatal myocardial disease in young and middle-aged adults. We report about a 76 year old male patient who underwent implantation with a biventricular Berlin Heart Excor system at the age of 74 due to acute biventricular heart failure caused by giant-cell myocarditis. The implantation was without any surgical problems; however, a difficulty was the immunosuppressive therapy after implantation. Meanwhile the patient is 76 years old and lives with circulatory support for about 3 years without major adverse events. Also, in terms of mobility in old age there are no major limitations. It seems that in even selected elderly patients an implantation of a long term support with the biventricular Berlin Heart Excor is a useful therapeutic option with an acceptable outcome.

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Diagnosis, treatment, and outcome of giant-cell myocarditis in the era of combined immunosuppression.

Anne Räisänen-Sokolowski, Jukka Lehtonen, Jyri Lommi … (2012)

Giant-cell myocarditis often escapes diagnosis until autopsy or transplantation and has defied proper treatment trials for its rarity and deadly behavior. Current therapy rests on multiple-drug immunosuppression but its prognostic influence remains poorly known. We set out to analyze (1) our experience in diagnosing giant-cell myocarditis and (2) the outcome of patients on combined immunosuppression. We reviewed the histories, diagnostic procedures, details of treatment, and outcome of 32 consecutive patients with histologically verified giant-cell myocarditis treated in our hospital since 1991. Twenty-six patients (81%) were diagnosed by endomyocardial or surgical biopsies and 6 at autopsy or post-transplantation. Twenty-eight (88%) patients underwent endomyocardial biopsy. The sensitivity of transvenous endomyocardial biopsy increased from 68% (19/28 patients) to 93% (26/28) after up to 2 repeat procedures. The 26 biopsy-diagnosed patients were treated with combined immunosuppression (2-4 drugs) including cyclosporine in 20 patients. The Kaplan-Meier estimates of transplant-free survival from symptom onset were 69% at 1 year, 58% at 2 years, and 52% at 5 years. Of the transplant-free survivors, 10/17 (59%) experienced sustained ventricular tachyarrhythmias during follow-up and 3 received intracardiac defibrillator shocks for ventricular tachycardia or fibrillation. Repeat endomyocardial biopsies are frequently needed to diagnose giant-cell myocarditis. On contemporary immunosuppession, two thirds of patients reach a partial clinical remission characterized by freedom from severe heart failure and need of transplantation but continuing proneness to ventricular tachyarrhythmias.

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Idiopathic giant cell myocarditis and cardiac sarcoidosis.

Y Cooper, Lori Blauwet (2013)

Idiopathic giant cell myocarditis (GCM) and cardiac sarcoidosis (CS) are rare disorders that cause cardiomyopathy, often with ventricular arrhythmias or heart block. Infection, autoimmune processes, and genetics have all been implicated in the pathogenesis of these diseases, but the etiology for both diseases is likely a complex multifactorial process. Both GCM and CS are generally progressive despite treatment with standard heart failure and arrhythmia therapies. Making the diagnosis of GCM or CS on initial clinical presentation is possible in only a small percentage of patients, so myocardial tissue diagnosis is required. The use of multiple noninvasive imaging modalities may aid in diagnosis and assessment of response to treatment. Establishing the diagnosis of GCM or CS early is crucial, as tailored immunosuppressive treatment may significantly alter the clinical course of these patients. The prognosis of patients with GCM is poor, while the prognosis for patients with CS varies according to degree of left ventricular dysfunction.

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Idiopathic giant cell myocarditis--a distinctive clinico-pathological entity.

R Teare, A Pomerance, M. Davies (1975)

Eleven cases of idiopathic giant cell myocarditis are described, The pathological features are unmistakable with serpiginous areas of myocardial necrosis, at the margins of which giant cells can be seen on histological examination. The aetiology of the condition remains obscure but associated pathology suggests that altered immunity may be a factor. The rapid clinical course is, however, highly suggestive of an infective cause though none has been found.

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Author and article information

Contributors

Christian Bireta: Christian.bireta@med.uni-goettingen.de

Theodor Tirilomis: theodor.tirilomis@med.uni-goettingen.de

Marius Grossmann: Marius.Grossmann@med.uni-goettingen.de

Bernhard Unsöld: Bernhard.Unsoeld@med.uni-goettingen.de

Rolf Wachter: Rolf.wachter@med.uni-goettingen.de

Thorsten Perl: Torsten.Perl@med.uni-goettingen.de

Ahmad Fawad Jebran: Fawad.Jebran@med.uni-goettingen.de

Friedrich Albert Schoendube: schoendube1@med.uni-goettingen.de

Aron Frederik Popov: Popov@med.uni-goettingen.de

Journal

Journal ID (nlm-ta): J Cardiothorac Surg

Journal ID (iso-abbrev): J Cardiothorac Surg

Title: Journal of Cardiothoracic Surgery

Publisher: BioMed Central (London )

ISSN (Electronic): 1749-8090

Publication date (Electronic): 31 January 2015

Publication date PMC-release: 31 January 2015

Publication date Collection: 2015

Volume: 10

Electronic Location Identifier: 14

Affiliations

[ ]Department of Thoracic and Cardiovascular Surgery, University of Goettingen, Goettingen, Robert-Koch-Strasse 40, 37075 Goettingen, Germany

[ ]Department of Cardiology and Pneumology, University of Goettingen, Robert-Koch-Strasse, 40, 37075 Goettingen, Germany

[ ]Department of Anesthesiology, Emergency and Intensive Care Medicine, University of Goettingen, Robert-Koch-Strasse 40, 37075 Goettingen, Germany

Article

Publisher ID: 218

DOI: 10.1186/s13019-015-0218-9

PMC ID: 4320566

PubMed ID: 25637129

SO-VID: e1d61997-c037-408d-b81b-3594ab4f7998

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This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 2 November 2014

Date accepted : 18 January 2015

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ScienceOpen disciplines: Surgery

Long term biventricular support with Berlin Heart Excor in a Septuagenarian with giant-cell myocarditis

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Karger: Dermatology

Most cited references 6

Diagnosis, treatment, and outcome of giant-cell myocarditis in the era of combined immunosuppression.

Idiopathic giant cell myocarditis and cardiac sarcoidosis.

Idiopathic giant cell myocarditis--a distinctive clinico-pathological entity.

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