Cor Vasa 2024, 66(6):599-607 | DOI: 10.33678/cor.2024.078

(Isolated cardiac sarcoidosis as a cause of out-of-hospital cardiac arrest - a case report and a review)

Volodymir Dudash^a, Miloš Kubánek^a, Petr Peichl^a, Ondřej Fabián^b, Theodor Adla^c, David Zogala^d, Martina Koziar Vašáková^e, Alice Krebsová^a, Marek Šramko^a, Adrian Reichenbach^a, Jana Vrbská^a, Vojtěch Melenovský^a, Josef Kautzner^a

^a Klinika kardiologie, Institut klinické a experimentální medicíny, Praha, Česká republika

^b Pracoviště klinické a transplantační patologie, Institut klinické a experimentální medicíny a Ústav patologie a molekulární medicíny, 3. lékařská fakulta Univerzity Karlovy a Fakultní Thomayerova nemocnice, Praha, Česká republika

^c Pracoviště zobrazovacích metod, Institut klinické a experimentální medicíny, Praha, Česká republika

^d Ústav nukleární medicíny, 1. lékařská fakulta Univerzity Karlovy a Všeobecná fakultní nemocnice v Praze, Praha, Česká republika

^e Plicní klinika, 1. lékařská fakulta Univerzity Karlovy a Fakultní Thomayerova nemocnice, Praha, Česká republika

Isolated cardiac sarcoidosis is a rare disease with a challenging diagnostic process reflecting a low sensitivity of endomyocardial biopsy (EMB). Therefore, it often requires a multimodal imaging using cardiac magnetic resonance imaging (CMR) and/or positron emission tomography (PET-CT) with administration of ^18Ffluordeoxyglucose (FDG). Its clinical manifestation includes advanced atrioventricular block, ventricular tachyar- rhythmias and congestive heart failure. We aimed to illustrate pitfalls in the diagnosis of clinically isolated cardiac sarcoidosis in a young male with an aborted cardiac arrest due to ventricular fibrillation. This case inspired us to provide an updated review of the management of cardiac sarcoidosis. Case description: A 28-year-old male without any comorbidities was admitted to our department after an aborted out-of-hospital cardiac arrest due to ventricular fibrillation in February 2022. His first electrocardiogram showed sinus rhythm and a complete atrioventricular block with left bundle branch block QRS morphology. Echocardiogram and coronary angiography revealed severe non-ischemic systolic dysfunction of the non-dilated left ventricle with an ejection fraction of 25%. Findings in EMB specimens were non-specific. A biventricular cardioverter-defibrillator was implanted due to a persistent complete atrioventricular block. Cardiac magnetic resonance imaging was not done from the safety reasons due to dependency of the subject on cardiac pacing and expected poor quality due to artefacts from the implanted electrodes. In March 2022, PET-CT with administration of FDG was performed. Unfortunately, the examination was not diagnostic due to an incomplete suppression of the metabolism of carbohydrates. However, three avid lymphatic nodes were detected in mediastinum. Repeated PET-CT was performed in July 2022 with successful suppression of the metabolism of carbohydrates. The examination revealed a mismatch between focally increased accumulation of FDG and absent perfusion in anteroapical, septal, and basal segments, which is a typical finding for an inflammatory cardiac disease. In addition, there was an obvious progression of lymphadenopathy both above and under the diaphragm. Subsequently, we performed a repeated EMB, which verified cardiac sarcoidosis. This suggested a diagnosis of isolated cardiac sarcoidosis at the time of disease manifestation. These results enabled to start immunosuppression with corticosteroids and azathioprine, which was tolerated without complications. A gradual improvement in left ventricular ejection fraction up to 45% was observed and documented in September 2023. The patient remains clinically stable in the functional class II, without interventions from the implanted cardioverter-defibrillator. Since March 2023, the patient is not dependent on cardiac pacing.

Conclusion: The diagnosis of cardiac sarcoidosis should be considered in patients with non-ischemic dysfunction of the left ventricle, especially if accompanied with aneurysms of cardiac ventricles. The suspicion rises in the presence of atrioventricular blocks or occurrence of ventricular tachyarrhythmias. The difficult diagnosis of isolated cardiac sarcoidosis can be established in selected cases by multimodal imaging including CMR and PET-CT, or repeated EMB. The correct diagnosis is important both due prognostic and therapeutic implications.

Keywords: AV block, Cardiac arrest

Received: August 5, 2024; Revised: September 16, 2024; Accepted: October 2, 2024; Prepublished online: June 2, 2012; Published: December 20, 2024  Show citation