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CT IMAGING OF A CASE WITH BIVENTRICULAR HYPERTROPHY ABSTRACT ID- IRIA 1139 INTRODUCTION HYPERTROPHIC CARDIOMYOPATHY (HCM) Most common inherited cardiovascular disease, which manifests as diffuse or segmental left ventricular (LV) hypertrophy with a nondilated and hyperdynamic chamber. HCM is an autosomal dominant disease caused by mutations in genes encoding sarcomere proteins. Common expression of HCM is during adolescence, however patients may present at any point in life. The common phenotypes are: --asymmetric (septal) HCM --apical HCM --symmetric HCM (concentric HCM) --mass-like HCM. CASE REPORT 55 year old male Chest pain for 2 years and breathlessness for 5 months. Paroxysmal nocturnal dyspnea and orthopnea for 5 months. Not a known case of Hypertension/ diabetes mellitus . Family history- Nil relevant. EXAMINATION GENERAL EXAMINATION: BP – 146/98 mmHg, Pulse Rate - 72/ min. No pallor/icterus/cyanosis/clubbing/pedal edema/lymphadenopathy SYSTEMIC EXAMINATION: CVS- S1, S2 +. No murmur heard. OTHER SYSTEMS – normal. ELECTROCARDIOGRAM- Left ventricular hypertrophy with strain pattern. IMAGING CHEST X-RAY- Moneybag appearance due to pericardial effusion Followed by Cardiac CT was done for the suspicion of pericardial effusion and consolidation CARDIAC COMPUTED TOMOGRAPHY A (A&B) 4 chamber and short axis view of interventricular septum seen with thickness measuring 32.5 mm– suggestive of asymmetrical hypertrophy B C D C) Right ventricular free wall thickness measures 11.6mm. D) Left ventricular posterior free wall thickness measures 23.4mm. E) Left ventricular outflow tract shows no obstruction (orange arrow). E F H F) Main pulmonary artery measures- 40.4 mm. G) Right pulmonary artery- 20.3 mm. H) Left pulmonary artery – 23.1mm. I) Right upper pulmonary vein measures 18.2mm. Above findings are significant of pulmonary artery and venous hypertension. G I DIAGNOSIS Interventricular Septum thickened suggestive of asymmetrical hypertrophic cardiomyopathy. Biventricular hypertrophy. Pulmonary artery and venous hypertension. No left ventricular outflow tract obstruction. CT imaging findings correlated with Echocardiography findings as follows. ECHOCARDIOGRAM: Hypertrophic cardiomyopathy concentric variant. Asymmetrical septal hypertrophy. Septum-30mm. Left atrium dilated. Moderate pericardial effusion; posteriorly 3.8cm thickness. No Systolic anterior motion. No Left ventricular outflow tract obstruction. BACKGROUND A) Normal Heart B) asymmetric (septal) HCM with LVOT obstruction C) asymmetric (septal) HCM without LVOT obstruction D) apical HCM E) symmetric HCM (concentric HCM) F) mass-like HCM. Asymmetric involvement of the interventricular septum is the most common form (60%–70%). LV hypertrophy, with a thickened anterior mitral leaflet. Asymmetric septal wall hypertrophy at anteroseptal myocardium with and without LVOT obstruction. Apical HCM is myocardial hypertrophy predominantly involves the apex of the left ventricle (25%) of all patients . Complicated by hypertension Symmetric or concentric HCM, is characterized by concentric LV hypertrophy with a small cavity dimension and no evidence of a secondary cause. 42% occur in as many as of the cases of HCM. Mass-like HCM –manifests as a mass-like hypertrophy because of the focal segmental location of the myocardial disarray and fibrosis. CLINICAL FEATURES: --Chest pain --Dyspnea- PND and Orthopnea --Palpitations --Dizziness and Fatigue. INVESTIGATIONS ECG --Mild degrees of hypertrophy or LV hypertrophy- show strain pattern. --Septal hypertrophy-Abnormal Q waves, --Apical HCM- giant T-wave Chest X-ray --Shows LV or LA and/or RA enlargement with or without vascular redistribution in the lungs. --Aorta is typically small. --Bulge on the left heart border- reflects anteroseptal hypertrophy. ECHOCARDIOGRAPHY 30-60% show Systolic anterior motion. Peak Left Ventricular Outflow Tract gradient >30 mmHg is significanct in HCM Systolic dysfunction occurs due to wall thinning, cavity dilation, and fibrosis. Diastolic dysfunction in reduced chamber compliance, increase in chamber stiffness secondary to increased LV mass and myocardial fibrosis. Doppler echocardiography shows accuracy assessment of diastolic function demonstrating impaired relaxation CARDIAC MDCT Asymmetric hypertrophy- septal thickness is greater than or equal to 15mm The ratio of the septal thickness to the thickness of the inferior wall of the left ventricle is greater than 1.5 at the midventricular level. Apical hypertrophy- apical wall thickness of more than 15 mm Ratio of apical to basal LV wall thicknesses of 1.3–1.5. Symmetrical hypertrophy- LV wall thickening of about 14 mm. Mass like hypertrophy - It has homogeneous appearance and perfusion of adjacent normal myocardium. ECG-gated CT This test can be used to evaluate the patterns of LVH and wall motion in HCM. CARDIAC MRI MRI can be used to accurately characterize the distribution and degree of myocardial hypertrophy compared to Cardiac CT. Its advantages are to evaluate: Anatomical visualization of the entire LV wall morphology Functional analysis of wall motion volume & ejection fraction calculation --Left ventricular tract obstruction gradient --Systolic anterior motion of anterior mitral leaflet --Late Gadolinium Enhancement for identifying areas of fibrosis. TREATMENT FOR VARIOUS TYPES OF HCM Surgical myectomy Septal alcohol ablation Ventricular pacing Cardiac transplantation CONCLUSION Hypertrophic cardiomyopathy even though being a common disease, only a few cases with biventricular hypertrophy has been reported. Cardiac MDCT provides good spatial resolution through 3-D reformatted images. It can offer comprehensive information on detailed anatomic and functional information about the cardiac chambers. However Cardiac MR is the standard modality for differentiating HCM from other cardiomyopathies REFERENCES Hayashi S, Tojyo K, Uchikawa S, et al. Biventricular hypertrophic cardiomyopathy with right ventricular outflow tract obstruction associated with Noonan syndrome in an adult. Jpn Circ J 2001;65:132–5. [CrossRef][Medline] Chirillo F, Zecchel R, Stritoni P. Biventricular hypertrophic cardiomyopathy with alone obstruction to right ventricular outflow. Heart 2002;87:565. [FREE Full text] Fournier C, Bache R, Valette H, et al. Hypertrophic myocardiopathy with isolated obstruction of the right ventricle. Ann Cardiol Angeiol (Paris) 1985;34:71–4. [Medline] Maron BJ, McIntosh CL, Klues HG, et al. Morphologic basis for obstruction to right ventricular outflow in hypertrophic cardiomyopathy. Am J Cardiol 1993;71:1089–94 Henry WL, Clark CE, Epstein SE. Asymmetric septal hypertrophy. Echocardiographic identification of the pathognomonic anatomic c abnormality of IHSS. Circulation 1973; 47: 225-33.