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A 72 year-old mountain bicyclist suffered a biking accident. The following week, he presented to the emergency room with abdominal pain, where he was treated for superior mesenteric artery thrombosis and new-onset atrial fibrillation. Two months later, he presented with intermittent left scapular pain that radiated to the chest. Pharmacologic stress testing revealed a large reversible perfusion defect in the anterior wall with an ejection fraction of 41%. Left heart catheterization showed narrowing of the distal left main coronary artery and the proximal left anterior descending (LAD) and circumflex arteries, by what appeared to be external compression. Follow-up CT angiogram demonstrated two sinus of Valsalva aneurysms (SOVA) originating from the left and right coronary cusps. A transesophageal echocardiogram confirmed these findings and showed tremendous thrombus within the larger aneurysm. The patient was admitted to cardiothoracic surgery. Troponin was elevated to 0.16 μg/L, with EKG showing 1 mm ST-segment depressions in V4-6, prompting treatment for a non-ST segment myocardial infarction. He underwent complex surgery involving thrombus extraction and closure of the SOVA with pericardial patch repair, and coronary artery bypass grafting of the LAD and obtuse marginal arteries. After a prolonged ICU course in which the patient was managed for cardiogenic shock, he was discharged to a hospital equipped with a rehabilitation facility. Unfortunately, he developed profound lactic acidosis from ischemic bowel and passed away. SOVA are rare lesions that arise from congenital or acquired etiologies, such as this patient’s biking accident. Multi-modality imaging is important for quantifying size and anatomy. Unruptured SOVA can present as embolic phenomenon from thrombus, valvular regurgitation, or chest pain from compression of the coronary arteries. Rupture is a highly morbid condition, and so urgent referral to cardiothoracic surgery is recommended.

Introduction: Ischemic cardiomyopathy from coronary atherosclerosis is the leading cause of mortality in developed nations. However, the natural history of cardiovascular disease in contemporary pre-industrial man is not well described. The Tsimane of Bolivia are a forager-horticulturalist population in the western Amazonian basin with high levels of inflammation and a low prevalence of atherosclerosis risk factors. We sought to define the cardiovascular health of a contemporary pre-industrial population with high inflammation and low atherosclerotic risk factors. Methods: Two-Dimensional echocardiograms with Color and Doppler were obtained on 934 Tsimane over age 40. Echocardiograms were reviewed by 2 cardiologists following the American Society of Echocardiography guidelines for interpretation. Patients had baseline clinical evaluations including age, height, weight, blood pressure, and gender. Blood was obtained for serology for inflammatory markers. Results: The average age was 53 years (SD +/- 11; range 40-90 years). 455 (48.7%) were male. The prevalences of hypertension, diabetes, and smoking were 4.8%, 0.5%, and 1.3%, respectively. Mean LVEF was 66% (SD +/- 6.1). There were 4 males with LVEF less than 52% and 11 females with LVEF less than 54%. There was no measured LVEF less than 44%. Complete diastolic data was available in 910. Normal diastolic parameters were observed in 741 (81.4%). Mild diastolic dysfunction was found in 167 (18.3%) and moderate dysfunction in 2 (0.2%). No echocardiograms showed severe diastolic dysfunction. Serologic data showed elevated CRP, ESR, and IL-6 with low high density lipoprotein (HDL) and low density lipoprotein (LDL) Conclusions: In a pre-modern population with high inflammation and low atherosclerotic risk factors, cardiovascular systolic and diastolic dysfunction was a rare finding.

Background: Bicuspid aortic valve (BAV) and dilated ascending aorta frequently coexist. 10% of first degree relatives (FDRs) of probands with BAV inherit a valvular abnormality, however there is limited data concerning the occurrence of dilated aortic root in families of affected subjects. We evaluated the prevalence of aortic root dilatation among FDRs with a tricuspid aortic valve. Methods: Aortic root dimensions were measured by 2D echocardiography in BAV probands (n=48), their FDRs (n = 49) and controls without structural heart disease matched by age and gender (n=44). Aortic root was measured at: annulus, sinuses of Valsalva, sino-tubular junction and proximal ascending aorta. The dimensions were indexed by body surface area (BSA). Presence of dilatation was determined using published confidence intervals relating normal aortic diameters to BSA and age. Results: Five subjects (10%) in FDR group were found to have BAV, thus they were included in BAV group. Indexed aortic diameter was significantly different in 3 groups namely BAV>FDR>Control at annulus (2.0±0.3, 1.7±0.2, 1.6±0.1, p . BAV subjects showed significantly larger dimensions in the ascending aorta compared to FDRs and controls (p Conclusions: There is high prevalence of aortic root dilatation in the FDRs of subjects with BAV despite having normal tricuspid aortic valve. This finding suggests that aortic root dilatation is another phenotypic manifestation of BAV disease that is inherited independent of BAV.

Background: Abnormal aorta dilatation and elastic properties have been documented in subjects with bicuspid aortic valve (BAV). 10% of first degree relatives (FDRs) of probands with BAV inherit this valvular abnormality. There is no data regarding the elastic properties of aortic root in family members of BAV subjects. We evaluated the elastic properties in FDRs with normal tricuspid aortic valves. Methods: Systolic and diastolic aortic root dimensions were measured by 2D echocardiography in BAV probands (n=48) their FDRs (n = 49) and controls without structural heart disease matched by age and gender (n=44) at sinuses of Valsalva level. In addition, systolic (SBP) and diastolic blood pressure (DBP) were measured to calculate aortic distensibility, stiffness index and strain. Results: Five subjects (10%) in FDR group were found to have BAV, thus they were included in BAV group. There was no difference in age, gender, SBP and DBP in the three groups. History of hypertension was less prevalent in FDRs when compared to BAV and controls (16% vs 43% and 33%, p=0.03). All three parameters of aortic elastic properties including distensibility ×10 −3 mmHg (1.2±1.0 and 1.5±1.1 vs 2.3±1.5, p ). Conclusions: Aortic root elastic properties are abnormal in the family members of subjects with BAV despite normal tricuspid aortic valve morphology. This finding supports the hypothesis that there is a common genetic basis for BAV and abnormal elastic properties of aorta.