A middle-aged man with progressive fatigue

Cleveland Clinic Journal of Medicine. 2009 October;76(10):564, 568-574 | 10.3949/ccjm.76a.08114

October 1, 2009|Cleveland Clinic Journal of Medicine

CAUSES OF DEATH

7. What is the most common cause of death in patients with cardiac sarcoidosis?

Respiratory failure
Conduction disturbances
Progressive heart failure
Ventricular tachyarrhythmias
None of the above

The prognosis of symptomatic cardiac sarcoidosis is not well defined, owing to the variable extent and severity of the disease. The mortality rate in sarcoidosis without cardiac involvement is about 1% to 5% per year.^63,64 Cardiac involvement portends a worse prognosis, with a 55% survival rate at 5 years and 44% at 10 years.^17,65 Most patients in the reported series ultimately died of cardiac complications of sarcoidosis, including ventricular tachyarrhythmias, conduction disturbances, and progressive cardiomyopathy.^10,17

Since corticosteroids, pacemakers, and implantable cardioverter-defibrillators have begun to be used, the cause of death has shifted from sudden death to progressive heart failure.⁶⁶

CASE CONTINUED

Figure 2. Magnetic resonance imaging of the patient's heart. The long-axis phase-sensitive image shows delayed enchancement in the basal septum and basal inferolateral walls (arrows), strongly suggesting sarcoidosis. End-systolic and end-diastolic steady-state free precession images in the same plane show a moderately hypertrophied but contractile left ventricle, which argues against ischemia.

While hospitalized, our patient had two episodes of nonsustained ventricular tachycardia (7 and 12 beats) on telemetry. Cardiac MRI showed a lesion in the basal septum most likely involving the left bundle and an area of lateral basilar involvement near the mitral annulus (Figure 2). Ventricular dyssynchrony was clearly evident on both echocardiography and MRI, with depressed left ventricular function (ejection fraction 28% on MRI, 35% on echocardiography).

Electrophysiologic testing revealed inducible monomorphic sustained ventricular tachycardia. The patient subsequently had a biventricular cardioverter-defibrillator implanted. He was started on an angiotensin-converting enzyme inhibitor and a beta-blocker for his heart failure. Further imaging of his chest and abdomen revealed lesions in his thyroid and liver. As of this writing, he is undergoing further workup. Because of active infection with Clostridium difficile, steroid therapy was deferred.

References

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