1387

Arrhythmias in a Patient With SarcoidosisJOSE L. SERRA, M.D., A.F.A.C.C., JORGE A. FIGUEROA, M.D., GUILLERMO E. ALLENDE,

M.D., and EDUARDO MOREYRA, M.D., F.A.C.C.From the Cardiovascular Unit, Sanatorio Allende, Cordoba, Argentina

Arrhythmias in a Patient With Sarcoidosis. Sarcoidosis is a multisystemic granulomatous diseaseof unknown etiology; up to 27% of cases entail cardiac involvement. Conduction abnormalities and ven-tricular tachycardia are the most common arrhythmias and can cause sudden death. We describe a patientwho developed cardiac sarcoidosis 9 years after undergoing surgery for neurosarcoidosis. He presentedwith 2:1 second-degree atrioventricular block. Ventricular tachycardia with 3 morphologies was inducedby exercise stress test. A DDD pacer/implantable cardioverter defibrillator (ICD) was implanted, whichprevented exercise-induced ventricular tachycardia in a follow-up stress test. Treatment with steroids wasinitiated. The AVB disappeared, and no further arrhythmias were documented at the 1-year follow-up. (JCardiovasc Electrophysiol, Vol. 22, pp. 1387-1390, December 2011)

atrioventricular block, cardiac MRI, implantable cardioverter defibrillator, sarcoidosis, ventricular tachycardia

Introduction

Sarcoidosis is a multisystemic granulomatous disease ofunknown etiology. Granulomas that infiltrate the heart ex-ist in up to 27% of sarcoidosis patients at autopsy, but theclinical manifestations of cardiac sarcoidosis are present inmerely 5% of cases. 1Patients present with a cardiomyopathyif the myocardial infiltration is extensive.1-3 The conductionsystem is frequently affected. These alterations can causebrady- and tachyarrhythmias that can result in palpitations,syncope, or sudden death.4 Treatment is aimed at improvingsymptoms, controlling the inflammation, and preventing thedeterioration of left ventricular function and sudden death.3

Case

A 31-year-old man experienced 6 months of malaiseand fatigue. Approximately 9 years earlier, he was diag-nosed with neurosarcoidosis and underwent resection ofa right cerebral granulomatous mass. At that time, therewas no evidence of cardiac involvement, and steroids werenot initiated. In subsequent years, he developed no signsof recurrence.

On physical examination, he was afebrile. A macularlesion was detected in the right side of the nose, measuring2 cm in diameter. A 1.5-cm mobile and painless lymphnode was palpated in the left groin. The patient’s bloodpressure was 120/60 mmHg, his pulse was 52 beats perminute (bpm), and his respiratory rate was 18 breaths perminute. His heart rhythm was irregular, with a variableS1, a midsystolic 1/6-ejection murmur in the mitral area,and a normal S2. The remainder of the physical examwas normal.

No disclosures.

Address for correspondence: Jose L. Serra, M.D., Cardiovascular Unit, Sana-torio Allende, Obispo Oro 55, PC 5000. Cordoba, Argentina. Fax: 54-351-4269271; E-mail: joseluisserra@arnet.com.ar

Manuscript received 11 March 2011; Revised manuscript received 3 May2011; Accepted for publication 9 May 2011.

doi: 10.1111/j.1540-8167.2011.02114.x

An electrocardiogram showed sinus rhythm at a rateof 90 bpm. There was a 2:1 second-degree atrioventricu-lar block (AVB) with right bundle branch block (RBBB).The chest X-ray was normal. Blood tests, including liverand renal function tests, ionic calcium, sedimentation rate(2 mm/h), troponine T (0.01 ng/mL), and angiotensin-converting enzyme levels (45 μ/L), were normal. Vi-ral serology was negative, and the echocardiogram wasnormal.

By high-definition multidetector thoracic and abdom-inal computed tomography, we noted central and periph-eral peribronchiolar and perivascular nodular lesions;superior pretracheal and perivascular mediastinal cal-cified lymph nodes, and abdominal periaortic and en-larged inguinal lymph nodes. The spleen harbored multi-ple nodular lesions. A biopsy of the inguinal lymph noderevealed noncaseating granulomas with lymphocytes, ep-ithelioid and multinucleated giant cells (Langhan’s typewith asteroid and Schaumann bodies). A cardiac mag-netic resonance image revealed delayed gadolinium up-take in the anterobasal segment of the interventricularseptum (Fig. 1). The left ventricular size and functionwere preserved. Radionuclide imaging with SESTAMIBITc 99 showed basal anteroseptal hypoperfusion at restthat improved slightly after exertion.

During an exercise stress test the baseline 2:1 secondAVB and RBBB persisted until the sinus rate reached125 bpm, when a high-grade AVB with an idioventricu-lar escape rhythm and left bundle branch block (LBBB)morphology developed at a rate of 55 bpm (Fig. 2). Thepatient went into ventricular tachycardia (VT) with anLBBB morphology and frontal left superior axis (VT # 1)and a progressively increasing heart rate, from 75 to 100bpm (cycle length 800–600 ms). Then, a second type of VTdeveloped with RBBB morphology and frontal left supe-rior axis (VT # 2) that alternated with the previous VTat similar heart rates (cycle length 520–560 ms; Fig. 3).At maximal exertion, a third type of VT emerged (VT #3) with RBBB morphology, a frontal right superior axis,and cycle lengths between 500 and 550 ms (Fig. 4). Thepatient achieved 12 Mets and a maximum blood pressureof 170/85 mmHg. He remained asymptomatic during theentire procedure.

1388 Journal of Cardiovascular Electrophysiology Vol. 22, No. 12, December 2011

Figure 1. Cardiac magnetic resonanceimage, showing delayed gadolinium up-take in the basal anteroseptal segment ofthe interventricular septum.

In the recovery phase, he experienced the same cardiacrhythms that developed during exertion but in reverseorder, returning to baseline sinus rhythm with 2:1 AVB.

A permanent DDDR pacer/ICD was inserted. A follow-up stress test showed a baseline sinus-sensed ventricularpaced rhythm. With exertion, the heart rate increased to150 bpm without evidence of ventricular arrhythmia.

Two weeks after the pacemaker/ICD was inserted,treatment with prednisone 1 mg/kg/day was initiated.During the next 14 months, the patient remainedasymptomatic, without experiencing arrhythmias or ICDevents. The conduction through the AV node recovered,and RBBB disappeared.

Figure 2. Stress test. Second stage. Id-ioventricular rhythm with atrioventricu-lar block.

Discussion

Cardiac sarcoidosis can range from a benign subclinicalcondition to a life-threatening disorder. Although autopsy-based studies have demonstrated that cardiac granulomasexist in as many as 27% of sarcoidosis patients, clinical man-ifestations are present in merely up to 5% of cases.2 Gran-ulomas have a patchy distribution, lying predominantly inthe free wall of the left ventricle and basal septum.1 Whenthe right ventricle is involved, it can mimic the clinical pre-sentation of arrhythmogenic right ventricular dysplasia.5,6

They can also affect the pericardium. Cardiomyopathy withcongestive heart failure can occur when the myocardial infil-tration is extensive.

Serra et al. Arrhythmias in a Patient With Sarcoidosis 1389

Figure 3. Stress test. Third stage. Ventric-ular tachycardia #1 (∗1) alternating withventricular tachycardia #2 (∗2).

Granulomas frequently affect the atrioventricular nodeand bundle of His, inducing AVB and various intraventricularconduction defects.7,8 These lesions can be silent or can causesyncope. Sarcoid granulomas in the myocardium can also ef-fect abnormal automaticity and alter conduction and refrac-toriness, inducing reentrant ventricular arrhythmias, whichexist in as many as 23% of patients with demonstrated car-diac involvement.4,9,10 Sudden death due to ventricular ar-rhythmias or conduction abnormalities accounts for 25–65%of mortalities in patients with cardiac sarcoidosis.11,12

There is limited evidence regarding the most effectivetreatment for patients with cardiac sarcoidosis.1 Treatmentsshould improve symptoms, control the inflammation, and

Figure 4. Stress test. Fourth stage. Ven-tricular tachycardia #3.

prevent the deterioration of left ventricular function and sud-den death.3 Steroids are believed to be capable of haltingor slowing the inflammation and fibrosis, which might ex-plain their efficacy in reverting conduction abnormalities andimproving alterations in myocardial perfusion.

Small retrospective studies have suggested that steroidsimprove long-term survival,13 but their effectiveness againstventricular arrhythmias has not been properly studied. Dueto the high rate of recurrence of VT in patients who aretreated with antiarrhythmic agents, some physicians recom-mend ICD placement in these patients.3,14-16 In cases of ad-vanced AVB, a permanent pacer is recommended to improvethe prognosis.3

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We present a case of systemic sarcoidosis with many ofthe arrhythmias that are commonly described in cardiac sar-coidosis. The baseline electrocardiogram showed 2:1 second-degree AVB and RBBB. An exercise stress test induced VTwith 3 morphologies. By cardiac MRI and radionuclide imag-ing, we noted the involvement of the anterobasal segment ofthe interventricular septum, and the biopsy of an inguinallymph node revealed noncaseating granulomas.

Based on the revised 2006 Guidelines for diagnosing car-diac sarcoidosis from the Japan Society of Sarcoidosis andOther Granulomatous Disorders, our patient met the diagnos-tic criteria for cardiac sarcoidosis and, therefore, we felt thatan endomyocardial biopsy was unnecessary.17 The extent ofthe granulomatous infiltration was insufficient to reduce leftventricular systolic function but was likely responsible forthe conduction abnormalities and the exercise-induced VT.During exertion, the altered atrioventricular conduction andincreased sympathetic drive likely facilitated reentry at theseptum, predisposing the patient to the development of VT.The modulations in VT morphology were likely related todisparate reentry loops in the same area or the same basicloop with progressive changes in its own circuit or exit site.We speculate that the absence of VT during the follow-upstress test, without changes in anatomical substrate, was at-tributed to a progressive increase in ventricular pacing duringexertion, preventing the reentry mechanism.

Treatment with steroids was effective in reverting AVB.The degree of anatomical resolution of the anteroseptal gran-uloma was unknown because a follow-up cardiac MRI andradionuclide imaging were not performed.

In conclusion, this case illustrates the spectrum of ar-rhythmias that can be observed in patients with cardiac sar-coidosis. Pacing avoided ventricular bradicardia due to AVBduring exertion, preventing exercise-induced VT and ICDevents during follow-up. Treatment with steroids improvedAV nodal conduction abnormalities.

Acknowledgment: The authors appreciate Dr. Diego Cabral’s assistance ininterpreting the cardiac magnetic resonance data.

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