Cor Vasa 2018, 60(2):e155-e164 | DOI: 10.1016/j.crvasa.2017.05.012

Management of cardiac sarcoidosis - A practical guide

Petr Kopřiva^a,*, Martin Gřiva^a,b, Zbyněk Tüdös^c

^a Kardiologické oddělení, Kardiovaskulární centrum, Krajská nemocnice T. Bati, a.s., Zlín, Česká republika

^b I. interní klinika - kardiologická, Lékařská fakulta Univerzity Palackého a Fakultní nemocnice Olomouc, Olomouc, Česká republika

^c Radiologická klinika, Lékařská fakulta Univerzity Palackého a Fakultní nemocnice Olomouc, Olomouc, Česká republika

Sarcoidosis is a multi-system granulomatous disorder of unclear etiology which can affect any organ of the body including the heart. The heart is involved in up to 25% of sarcoidosis patients. In rare cases, the heart can be the only organ involved.
Involvement of the heart, called cardiac sarcoidosis, especially if symptomatic, significantly deteriorates the prognosis for sarcoidosis patients, which is why cardiac sarcoidosis should be not only considered, but also searched for actively. Despite recent advances in this field, diagnosis, risk-stratification, and treatment of cardiac sarcoidosis remains a challenging issue. Fortunately, several recommendations have been recently formulated which provide relatively clear guidance on the management of patients with cardiac sarcoidosis. The cornerstone of management of these patients is a multidisciplinary approach involving collaboration of cardiologists, pulmonologists, radiologists, rheumatologists, and other specialists.
Currently, diagnosis of cardiac sarcoidosis is based on an assessment of patient's symptoms, physical examination and results of standard ECG, Holter monitoring and echocardiography. This series of examinations can identify individuals with possible cardiac sarcoidosis, who should undergo, as the next step, cardiac magnetic resonance and positron emission tomography, which are the techniques of choice for the diagnosis of cardiac sarcoidosis. Histological verification, critical for establishing a definitive diagnosis, is based - in cases with a typical picture documented by imaging techniques - on an extracardiac biopsy. In some cases, when an extracardiac biopsy is not feasible, an endomyocardial biopsy is needed.
The cornerstone of treatment remains corticosteroids, in some cases in combination with other immunosuppressives, although data on their efficacy and safety from randomized trials are lacking. As the most frequent causes of death from cardiac sarcoidosis are heart rhythm disorders, be it atrioventricular blocks or ventricular arrhythmias, an irreplaceable role in the management of these patients is played by implantation of pacemakers and implantable cardioverter-defibrillators (ICD). One of the most critical issues is risk stratification of patients who, while not meeting classic criteria for ICD implantation, continue to be at high risk of sudden cardiac death and therefore should still be considered for ICD implantation. The last option for patients with advanced sarcoidosis is heart transplantation.
The present paper is an overview of presentation, diagnosis, and treatment of cardiac sarcoidosis, with special emphasis on the use of algorithms applicable in routine clinical practice.

Keywords: Cardiac magnetic resonance; Cardiac sarcoidosis; Diagnosis; Heart failure; Implantable cardioverter-defibrillator; Positron emission tomography; Sudden cardiac death; Treatment

Received: March 7, 2017; Revised: May 17, 2017; Accepted: May 20, 2017; Published: April 1, 2018  Show citation