Abstract 20962: Under-diagnosis of Cardiac Sarcoidosis Using the Original Japanese Ministry of Health and Welfare Criteria
Background: Cardiac Sarcoidosis (CS) is an under-diagnosed disease with significant morbidity and mortality and is responsible for 50% of the deaths of patients (pts) with sarcoidosis. The Japanese Ministry of Health and Welfare (JMHW) has published guidelines to establish the diagnosis of CS. These original criteria did not include patient symptoms or cardiac magnetic resonance imaging (CMRI) findings.
Methods: We retrospectively identified all pts at our institution who were diagnosed with CS in the period from March 2002 – April 2010. As a later modification of the JMHW criteria took into account patient symptoms or abnormal CMRI findings to establish the diagnosis of CS, we applied both the original and modified criteria to this population.
Results: Thirty-three pts (79% African American) were identified, all of whom had extra-cardiac histological proof of sarcoidosis. There were 17 males (52%) and the mean age was 53 ± 11 years. The mean left ventricular ejection fraction was 41 ± 18 %. The mean follow up duration was 3.75 ± 4 years. Only 17 pts (52%) met the criteria for a diagnosis of CS based on the original JMHW criteria. All 17 received an implantable cardioverter-defibrillator (ICD), 8 (47%) of whom received appropriate ICD therapy (shocks or anti-tachycardia pacing) for malignant ventricular tachyarrhythmia. Sixteen pts did not fulfill the original JMHW criteria but did fulfill the modified criteria based on their presenting symptoms or abnormal CMRI findings. In this subgroup, syncope was the presenting symptom in 8 pts (50%) and 4 pts had abnormal CMRI findings. Thirteen of these 16 pts received an ICD, 5 (38%) of whom received appropriate ICD therapy. The original JMHW criteria would have missed the diagnosis in 16 of 33 patients (48%), of whom 5 (15% of the total) went on to experience potentially fatal malignant ventricular tachyarrhythmia. Importantly, appropriate ICD therapy was not predicted by JMHW criteria fulfillment.
Conclusions: Syncope is a particularly malignant presenting symptom in pts with extra-cardiac sarcoidosis. Strict reliance on the original JMHW criteria for establishing the diagnosis of CS, without regard to patient symptoms, may further perpetuate the delay in the diagnosis of CS.
- © 2010 by American Heart Association, Inc.