Introduction: Sarcoidosis is a rare, multisystem, granulomatous disease of unknown etiology. It usually presents in young adults but has been reported in children and in the elderly, especially, In Japan. Noncaseating granulomas are the pathological hallmark of sarcoidosis. Sarcoidosis usually affects the respiratory system or mediastinal lymph nodes in more than 90% of cases, but may involve almost any organ.Early autopsy studies suggest up to 27% of patients with sarcoidosis have myocardial involvement. Isolated cardiac sarcoidosis, that is, with no detectable evidence of sarcoidosis in other organs, is rare. A recent review of sarcoidosis placed isolated cardiac involvement at 2%, one of the least common manifestations. Although Sarcoidosis is not commonly fatal, cardiac involvement may be responsible for more than two-thirds of deaths. In Japan, cardiac involvement is more common in older women and responsible for as many as 58% to 85% of deaths from sarcoidosis. The most frequently involved area is the ventricular septum [one third], followed by the inferior wall, anterior left ventricle, right ventricle, and lateral left ventricle.Cardiac sarcoidosis may be an asymptomatic accompaniment to pulmonary disease or may be the presenting feature of sarcoidosis. The clinical manifestations of cardiac sarcoidosis include conduction disorders, arrhythmias, atrial and ventricular arrhythmias, congestive heart failure, valvular pathology, pericardial effusions, and sudden cardiac death. Cardiac complications are the second leading cause of sarcoidosis-related death, after respiratory complications, in the United States. Patients with CS have a worse prognosis than patients without cardiac involvement. Early Screening for cardiac involvement in patients with sarcoidosis in other organ systems is critical as sudden death may be the first manifestation of cardiac sarcoidosis. Performing a detailed clinical history to identify cardiac symptoms (including syncope, presyncope, or palpitations), a 12-lead electrocardiogram (ECG), and an echocardiogram is recommended.Further screening is advised if any of the initial screening investigations yields an abnormality, cardiovascular magnetic resonance (CMR) or fluorodeoxyglucose positron emission tomography (FDG-PET). Screening for cardiac involvement in specific clinical presentations in patients without known sarcoidosis can also identify CS patients early in their disease course. The clinical diagnosis of cardiac sarcoidosis (CS) is therefore critically important to the timely planning of therapeutic strategies. We present two patients with Cardiac Sarcoidosis.
Purpose: Our purpose is to raise awareness of cardiac involvement in Sarcoidosis and its clinical implications.
Patients and Materials: We describe two patients with cardiac Sarcoidosis which the initial presentation was heart conduction abnormality. Case 1: The first patient is a healthy 43-year-old male, works as park ranger. Presented with Syncope and transient complete atrio-ventricular block (CAVB) on ECG which required Cardio-Pulmonary Resuscitation (CPR), temporary and shortly after a permanent pacemaker implantation. Further investigation included : chest X-ray, CT and Gallium-scan indicate evidence of Hilar and Axillar lymphadenopathy. The final diagnosis of Sarcoidosis was established by Mediastinal lymph node biopsy.Case 2: The second patient is a 58-year-old female with hyperlipidemia, recently diagnosed with Pulmonic Sarcoidosis (presented two years earlier by cough and dyspnea) and was proven by a CT scan (demonstrating enlarged mediastinal lymph-nodes) and by mediastinal lymph node biopsy. The patient presented to us with fatigue, dizzines, pre-syncope and evidence of a high degree atrio-ventricular (AV) block (without signs of cardiac ischemia) required permanent pacemaker implantation. Prior to the implantation there was no evidence of inducible tachyarrhythmia on an Electro-Physiological-Study (EPS).
Summary and Conclusions: We describe two patients with Cardiac Sarcoidosis initially presented with high degree AV Block required pacemaker therapy. One patient already diagnosed with Pulmonary Sarcoidosis and in the second one the diagnosis was established during the recent event. Our recommendation, supported by expert opinion, is to preform early screening to patients with Sarcoidosis for cardiac involvement by detailed clinical history, a 12-lead ECG and echocardiogram. When needed other modalities as CMR also can be performed. Thus save lives.