Faculty Bibliography

Abstract Background: Early identification of coronary artery disease (CAD) among symptomatic women is critical given their worse outcomes as compared to men. We evaluated the value of the Morise score, a simple clinical risk score, for the assessment for CAD as determined by computed tomography coronary angiography (CTCA) and compared it to the Diamond-Forrester risk assessment. Methods: One hundred forty women (mean age, 64+/-11 years) with chest pain syndromes and no known CAD referred for CTCA were analyzed. Patients were risk stratified for likelihood of CAD by Morise and Diamond-Forrester scores. The presence and degree of CAD were determined by CTCA and classified as normal, nonobstructive (<50% stenosis), or obstructive (>50% stenosis). Total coronary calcium was calculated based on Agatston scores. Results: When risk was assessed by Morise vs. Diamond-Forrester, 5% vs. 7% of women were stratified as low, 41% vs. 82% as intermediate, and 54% vs. 11% as high risk for CAD, respectively. CAD was present in 95 (68%) patients; 22 (16%) had obstructive CAD, and 73 (52%) had nonobstructive CAD. Morise scores significantly correlated with calcium scores (p<0.001) as well as the presence and degree of CAD (p<0.0001). Morise scores also demonstrated significantly higher accuracy (66% vs. 48%, p<0.005) and higher sensitivity (56% vs. 16%, p<0.001) but lower specificity (82% vs. 97%, p<0.05) when compared to Diamond-Forrester risk assessment. Conclusions: The Morise score performed better than Diamond-Forrester for CAD risk assessment, which highlights the importance and power of a simple history and physical examination in determining women at risk for CAD.

Introduction: Coronary CTA is a reliable non-invasive imaging test for evaluating coronary artery disease (CAD). Obtaining diagnostic image quality (IQ) with low radiation exposure in patients with irregular heart rates such as atrial fibrillation, however, remains challenging. This study evaluated IQ and inter-reader variability using prospective ECGtriggered sequential dual-source data acquisition at end-systole (PROS-CT) for evaluation of CAD in patients with atrial fibrillation. Methods: 30 consecutive patients (22 men, mean age 64 years, mean BMI 29) with persistent atrial fibrillation who underwent PROS-CT for evaluation of pulmonary vein anatomy were identified from our cardiac CT patient registry. Images were reconstructed using standard protocols every 50ms from 250 to 400ms after the R wave trigger. Two independent, blinded readers evaluated the coronary arteries using an 18-segment model and all available reconstructions. IQ was graded on a 5-point Likert scale (worst to best) and coronary stenosis on 5-point semi-quantitative (none to severe) and binary scales (greater or less than 50%). In addition, overall IQ and diagnostic quality were graded for each reconstruction. Results: 11 patients (37%) had significant (>50% stenosis) CAD. The average heart rate was 82+/-20 bpm and the average heart rate variability (range) was 71+/-22 bpm. The mean effective radiation dose was 6.5+/-2.4 mSv and mean CTDIvol was 23.8+/-8.0 mGy. There were 304 coronary segments available for interpretation with mean IQ of 2.9+/-0.8. Diagnostic IQ was noted in 97.9% of segments, with 2 patients demonstrating at least 1 segment with non-diagnostic IQ. The 300ms reconstruction phase provided the highest IQ with 70% of patients demonstrating diagnostic IQ, but the combination of all phases (250-400ms) performed significantly better than single or other double and triple reconstruction phase combinations (p<0.0005 for all comparisons). Inter-reader variability for detection of stenosis was excellent, with 98.4% concordance using the binary scale with 50% stenosis cutoff, and 75.3% concordance using 5-point stenosis grade. Conclusions: PROS-CT with acquisition at end-systole provides good diagnostic image quality for evaluation of significant CAD in patients with atrial fibrillation. The use of multiple end-systolic phases over a 150ms window for each study improves diagnostic IQ

Primary cardiac synovial sarcoma is a rare malignancy, comprising approximately 5% of cardiac sarcomas and fewer than 0.1% of all primary cardiac tumors. Synovial sarcoma is typically characterized by a t(X;18)(p11.2;q11.2) translocation resulting in fusion of the SS18 (aka SYT) gene on chromosome 18 with the SSX1, 2, or 4 genes on the X chromosome.We report a case of primary pericardial synovial sarcoma in a 42-year-old man with dyspnea. Imaging studies showed an 8.0 x 4.8 cm enhancing pericardial mass compressing the left atrium and a large pericardial effusion with compression effect consistent with tamponade. The patient underwent partial surgical resection of the mass. Histologic examination revealed an invasive malignant neoplasm with fascicles and sheets of uniform spindle cells with pleomorphic nuclei and many mitoses (7 per 10 HPF). This monophasic pattern raises a differential diagnosis including synovial sarcoma, fibrosarcoma, epithelial sarcoma, and leiomyosarcoma. The diagnosis of synovial sarcoma was confirmed by interphase FISH on FFPE tissue with an SS18 break-apart probe that was positive for an SS18 rearrangement. The patient underwent adjuvant chemotherapy and radiotherapy. He survives at 9 months after diagnosis with residual tumor growing rapidly. A review of the literature reveals 40 case reports of primary synovial sarcoma of the heart. The mean age is 36 years with a M/F ratio of 2.25. The most common locations are pericardium (13 cases) and right atria (12). Seventeen cases are monophasic type, of which 11 have confirmed t(X;18) translocations by cytogenetics, FISH, and/or RT-PCR. The prognosis is poor; 11 patients died during the first year after diagnosis. FISH provides a valuable tool for the diagnosis of synovial sarcoma, especially for challenging cases in uncommon locations (eg, heart). Identification of patients with the SS18 translocation is important for future targeted therapies

AIM: To evaluate safety and utility of coronary computed tomography angiography (CCTA) compared to invasive coronary angiography (ICA) in new cardiomyopathy. METHODS: Eighteen patients (mean age 56.5 years, 10 males) who presented for evaluation of new onset heart failure with evidence of systolic dysfunction (ejection fraction < 40%) on echocardiography and recent ICA were prospectively enrolled. Patients with known coronary artery disease, atrial fibrillation, creatinine > 1.5 g/dL, and contraindication to intravenous contrast administration were excluded. CCTA was performed using a dual source 64-slice scanner. Mean heart rate was 75 beats per minute. Stenosis was graded for each coronary segment as: none, mild (< 50%), moderate (50%-70%), severe (> 70%), or non-evaluable. Ischemic cardiomyopathy (ICM) was diagnosed if severe stenosis was present in the left main, proximal left anterior descending artery, or two or more major arteries. RESULTS: Two patients were diagnosed with ICM by ICA. CCTA correctly identified 2 patients with ICM and 16 patients as non-ICM. CCTA successfully evaluated 240/246 coronary segments with an accuracy of 97.5%, sensitivity 70%, specificity 98.7%, positive predictive value of 70%, and negative predictive value of 98.7% for identifying severe stenosis on a per-segment level. CONCLUSION: Dual source 64-slice multi-detector CCTA is a safe, accurate, and non-invasive technique for diagnosing ICM in patients presenting during the acute phase of newly diagnosed cardiomyopathy.

ABSTRACT: OBJECTIVE: Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed recalcitrant life-threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a giant coronary aneurysm. CASE REPORT: De-identified data were collected retrospectively in case report format. Although our sixteen year old female with Behcet's vasculitis had resolution of many arterial aneurysms, she had persistent venous thrombosis of large vessels, as well as persistent, giant arterial aneurysms requiring intra-arterial coiling of a lumbar artery and coronary bypass grafting despite intensive immunosuppression including glucocorticoids, cyclophosphamide, infliximab, methotrexate, azathioprine and intravenous immunoglobulin. CONCLUSIONS: Vascular manifestations may be seen in Behcet's syndrome, including asymptomatic coronary aneurysm, which may be refractory to immunosuppression and ultimately require surgical intervention. Increased awareness is essential for prompt diagnosis and management.