Primary Cardiac Sarcoidosis with Syncope and Refractory Atrial Arrhythmia: A Case Report and Review of the Literature

Abstract

We discuss the case of a 38-year-old 3 Sarcoidosis is most often associated with the lungs, but the disease can manifest itself in any tissue. Cardiac involvement was not described until 1929. 4,5 In recent times, cardiac manifestations have been understood to play a greater role in sarcoidosis morbidity than previously thought. In this report, we present a case of primary cardiac sarcoidosis that was successfully treated with a hybrid pharmacologic, surgical, and catheterbased intervention. Case ReportA 38-year-old black man presented at our clinic for evaluation of his first syncopal episode and atrial fibrillation (AF), this last accompanied by a rapid ventricular rate that was refractory to diltiazem, metoprolol, and digoxin therapy. The patient reported shortness of breath, intermittent palpitations, and chest pain. His medical history was significant for hypertension, obstructive sleep apnea, and diabetes mellitus type 2. Further, he had an implantable cardioverter-defibrillator to prevent sudden cardiac death due to his congestive heart failure (left ventricular ejection fraction [LVEF], 0.20-0.25, at the time of implantation). No electrocardiogram before the onset of AF was available at the time of his presentation to our clinic.The patient's initial evaluation included a transesophageal echocardiogram (TEE) that suggested left atrial thrombus with a preserved LVEF of 0.50 to 0.55. Rigorous anticoagulation therapy with a target international normalized ratio (INR) of 3.0 maintained for 6 months was apparently unsuccessful in dissolving the atrial thrombus. The patient had a high risk for thrombus embolization and for further clot formation from his newly documented atrial flutter. We determined that he would benefit from a hybrid procedure incorporating surgical excision of the atrial appendage and atrial mass, with subsequent catheter-based ablation targeting the atypical flutter. The atrial flutter and AF were the only arrhythmias identified by means of rhythm monitoring.

Atrial depolarization abnormalities in pulmonary sarcoidosis

“…Various studies have reported a higher incidence of supraventricular arrhythmias in patients with biphasic P wave in the inferior leads [ 26 , 27 ]. Since cardiac sarcoidosis is associated with an increased incidence of atrial arrhythmias, including atrial fibrillation [ 7 , 28 ], the greater prevalence of biphasic P wave may be a harbinger of atrial arrhythmias in sarcoidosis. The more vertical orientation of lead III as compared to the other inferior leads could explain the early appearance of biphasic P waves in this lead.…”

“…An estimated 20–25% of patients with pulmonary/systemic sarcoidosis have asymptomatic cardiac involvement (clinically silent disease). About 40–50% of patients who die of sarcoidosis-related complications are found to have cardiac sarcoidosis at necropsy despite never having manifested any clinical evidence of myocardial involvement during their lifetime [ 6 , 7 ]. The predominant sites of myocardial involvement, in decreasing order of frequency, are the left ventricular free wall and papillary muscles, the basal aspect of the ventricular septum, the right ventricular free wall, and the atrial walls [ 8 ].…”

Palpitations as a presenting feature of multisystem sarcoidosis

“…Black men are second highest at 29.8 per 100 000. White women and men have rates of 12.1 and 9.6 cases per 100 000, revealing a clear racial predilection [2]. …”

Atrial Arrhythmias and Atrial Involvement in Cardiac Sarcoidosis

“…Few reports have proved the presence of granuloma lesions in the atria in patients with cardiac sarcoidosis in a direct way. 19) In many cases, atrial involvement of cardiac sarcoidosis has been found on the basis of diagnostic imaging of the atria, histological findings of other organs, and clinical findings. No studies have assessed images of atrial legions in CS in a systemic manner.…”