Heart in the crossfire, from epsilon to beyond: cardiac sarcoidosis-a case report

Abstract

Background: Sarcoidosis is a great mimicker of various medical conditions, which leads to obstacles in early diagnosis and appropriate timely management.

Case summary: A 66-year-old Indian female with metabolic syndrome was initially treated for decompensated liver disease. Her baseline electrocardiogram showed right bundle branch block with a first-degree heart block. She presented 3 months later with angina and heart failure (HF) symptoms, complicated with ventricular tachycardia (VT) treated with i.v. amiodarone and anti-failure medication. Her coronary angiogram revealed mild disease, and her echocardiography showed a mildly reduced ejection fraction (EF) of 45% with regional wall motion abnormalities. Cardiac magnetic resonance imaging (CMR) revealed non-specific left ventricular (LV) patchy mid-wall to epicardial late gadolinium enhancement. Endomyocardial biopsy was complicated with cardiac tamponade and required pericardiocentesis followed by dual-chamber implantable cardioverter-defibrillator (ICD) later. Unfortunately, biopsy result was inconclusive, and serum angiotensin-converting enzyme was within the normal range. She had multiple admissions for the past 2 years for recurrent VT and decompensated HF despite the optimization of ICD setting and guideline-directed medical therapy. Repeated echocardiogram revealed similar EF with thinning of the LV basal septal segment. Her positron emission tomography (PET) scan (Tc-99 m) showed diffuse uptake at the LV myocardium and supraclavicular/mediastinal/abdominopelvic lymph nodes with a mismatch of fluorodeoxyglucose uptake at the basal-inferolateral segment (non-specific). Lymph node biopsy revealed chronic non-caseating granulomatous inflammation. Clinical diagnosis of cardiac sarcoidosis was made based on a histologic diagnosis of extracardiac sarcoidosis with cardiomyopathy/ventricular arrhythmia combined with PET/CMR findings.

Discussion: Cardiac sarcoidosis can have a myriad of symptoms, which may mimic several other disorders leading to a diagnostic challenge.

Keywords: Cardiac sarcoidosis; Case report; Epsilon wave; Immunosuppression; Sarcoidosis; Ventricular tachycardia.

Figure 1

Electrocardiogram showing a small deviation or blip following QRS complex (Epsilon wave)—arrowhead, with non-specific T wave inversions in the inferior and lateral chest leads (arrows).

Figure 2

The first electrocardiogram shows monomorphic ventricular tachycardia with possible origin from the inferoseptal region (A), with subsequent electrocardiogram post amiodarone (B) showing bradycardia with premature ventricular contractions with non-sustained ventricular tachycardia. The morphology of the premature ventricular contractions suggests the origin is from the basal anterolateral wall of the left ventricle. PVCs, premature ventricular contractions.

Figure 3

Cardiac magnetic resonance imaging showed patchy mid-wall to epicardial late gadolinium enhancement (arrow) at the apical anterior, basal anteroseptal/inferolateral, anterolateral, inferoseptal, mid-inferoseptal, mid-inferior, and apex, which was suggestive of non-ischaemic causes including infiltrative heart disease such as cardiac sarcoidosis or myocarditis. No CMR evidence to suggest arrhythmogenic right ventricular cardiomyopathy was seen. There was reduced ejection fraction of 26.3% and wall motions of all segments were normal.

Figure 4

Transthoracic echocardiogram shows thinning of the basal segment of the interventricular septum.

Figure 5

Fluorodeoxyglucose positron emission tomography–computed tomography showed diffuse but non-specific fluorodeoxyglucose uptake on the myocardium (arrow).