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Clinical Application of Diagnostic Guidelines in Cardiac Sarcoidosis
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A1109 - Clinical Application of Diagnostic Guidelines in Cardiac Sarcoidosis
Author Block: D. M. Perlman1, D. Johnson2, H. Roukoz1, R. Cogswell1, C. Shenoy1, M. Bhargava1; 1Univ of Minnesota, Minneapolis, MN, United States, 2Carleton College, Northfield, MN, United States.
Rationale: Cardiac sarcoidosis (CS) is a rare inflammatory disorder of unknown etiology with a poor prognosis. The diagnosis of CS remains a challenge with the use of multiple imaging modalities and the lack of a true gold-standard. The Japanese Ministry of Health and Welfare (JMHW) and the Heart Rhythm Society (HRS) have published separate sets of criteria to diagnose CS. There is minimal data analyzing the application of these guidelines. The aim of this study was to determine how many patients in a suspected CS cohort fit each criteria and what clinical features are associated with a definitive diagnosis of CS.
Methods: In this retrospective study, we examined patients at the University of Minnesota Sarcoidosis Clinic with confirmed or suspected CS. Data was abstracted for histologic evidence of sarcoidosis, electrocardiogram, echocardiogram, cardiac positron emission tomography (CPET), and cardiac magnetic resonance (CMR) imaging findings. The cases were evaluated according to the Japanese Ministry of Health (JMH) and the Heart Rhythm Society (HRS) diagnostic criteria. The patients were divided into 5 categories according to the amount of evidence for CS with 1 being highly likely and 5 being unlikely CS.
Results: We examined 49 cases with a possible diagnosis of cardiac sarcoidosis. Amongst the study population, 34 cases had strong evidence of extra-cardiac sarcoidosis (supportive imaging and/or tissue diagnosis of sarcoidosis), 12 patients had only CPET or CMR imaging abnormalities and 3 did not have cardiac sarcoidosis. 22 of the 34 patients with clinical evidence of extra-cardiac sarcoidosis met the JMH criteria and 30 of them met the HRS criteria. 3 patients (6%) had positive endomyocardial biopsy. The incidence of advanced heart block was low but other nonspecific ECG abnormalities were present in 86% of patients. 73% of cases had left ventricular ejection fraction below 50%. 27 patients had both PET and CMR and of these 11 (40%) had discordant results between the two advanced imaging studies.
Conclusion: In our study most patients with suspected CS met contemporary diagnostic criteria for CS. The frequency of discordant CEPT and CMR findings was high in cases who underwent both forms of advanced imaging studies. This poses a diagnostic challenge that will need further investigation. Longitudinal studies to assess prognostic features of different diagnostic criteria and imaging modalities are needed for appropriate risk-stratification of these patients.
Author Block: D. M. Perlman1, D. Johnson2, H. Roukoz1, R. Cogswell1, C. Shenoy1, M. Bhargava1; 1Univ of Minnesota, Minneapolis, MN, United States, 2Carleton College, Northfield, MN, United States.
Rationale: Cardiac sarcoidosis (CS) is a rare inflammatory disorder of unknown etiology with a poor prognosis. The diagnosis of CS remains a challenge with the use of multiple imaging modalities and the lack of a true gold-standard. The Japanese Ministry of Health and Welfare (JMHW) and the Heart Rhythm Society (HRS) have published separate sets of criteria to diagnose CS. There is minimal data analyzing the application of these guidelines. The aim of this study was to determine how many patients in a suspected CS cohort fit each criteria and what clinical features are associated with a definitive diagnosis of CS.
Methods: In this retrospective study, we examined patients at the University of Minnesota Sarcoidosis Clinic with confirmed or suspected CS. Data was abstracted for histologic evidence of sarcoidosis, electrocardiogram, echocardiogram, cardiac positron emission tomography (CPET), and cardiac magnetic resonance (CMR) imaging findings. The cases were evaluated according to the Japanese Ministry of Health (JMH) and the Heart Rhythm Society (HRS) diagnostic criteria. The patients were divided into 5 categories according to the amount of evidence for CS with 1 being highly likely and 5 being unlikely CS.
Results: We examined 49 cases with a possible diagnosis of cardiac sarcoidosis. Amongst the study population, 34 cases had strong evidence of extra-cardiac sarcoidosis (supportive imaging and/or tissue diagnosis of sarcoidosis), 12 patients had only CPET or CMR imaging abnormalities and 3 did not have cardiac sarcoidosis. 22 of the 34 patients with clinical evidence of extra-cardiac sarcoidosis met the JMH criteria and 30 of them met the HRS criteria. 3 patients (6%) had positive endomyocardial biopsy. The incidence of advanced heart block was low but other nonspecific ECG abnormalities were present in 86% of patients. 73% of cases had left ventricular ejection fraction below 50%. 27 patients had both PET and CMR and of these 11 (40%) had discordant results between the two advanced imaging studies.
Conclusion: In our study most patients with suspected CS met contemporary diagnostic criteria for CS. The frequency of discordant CEPT and CMR findings was high in cases who underwent both forms of advanced imaging studies. This poses a diagnostic challenge that will need further investigation. Longitudinal studies to assess prognostic features of different diagnostic criteria and imaging modalities are needed for appropriate risk-stratification of these patients.
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