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An elderly man with asymptomatic significant left ventricular hypertrophy

Case Presentation

A 73 year old man was admitted following an assault. He was asymptomatic other than occasional pre-syncope on rapid standing. Past medical history included surgery for bilateral carpel tunnel syndrome five years previously. There was no family history of note. He was taking Simvastatin 10mg, was a non-smoker and drank alcohol only occasionally.

Myocardial Disease

Findings on examination were: blood pressure 140/85 mmHg; regular pulse 60 beats per minute; venous pressure not elevated; and fourth heart sound on auscultation with loud P2 component. His electrocardiogram (ECG, figure 1) demonstrated sinus rhythm with first degree AV block, PR interval 228msec, ventricular rate 75bpm, small voltages with septal infarct pattern, QRS duration 82msec, QRS axis 15 degrees.

In view of the abnormal ECG the patient underwent two-dimensional echocardiography (figure 2). This demonstrated the following: normal left ventricular cavity dimensions; predominantly concentric hypertrophy (maximal septal thickness 1.7cm at the mid anteroseptum) with a hypokinetic basal anteroseptal wall; left ventricular end diastolic dimension 4.6cm; LV end systolic dimension 3.3cm; aortic root 3.3cm, left atrium 4.0cm; LV ejection fraction of 55% and significant impairment in long axis systolic function with moderate diastolic dysfunction. There was mid cavity obliteration secondary to papillary muscle contact but no LV outflow tract obstruction. Right ventricular size and function were normal with an estimated pulmonary artery systolic pressure 25mmHg.  The mitral valve leaflets were thickened but mobile with mild mitral regurgitation. The aortic valve was mildly thickened with mild aortic regurgitation.

Haematological and biochemical findings were as follows:
NT pro BNP 55pMol/L; Cardiac high sensitivity Troponin T 0.32 µg/mL; Normal renal and liver function tests; Creatinine clearance 82ml/min with no proteinuria; Serum albumin 48 g/L; IgA 4.3 g/L, IgG 8.5 g/L, IgM 0.6 g/L with no paraprotein, No Bence Jones protein and no free light chain excess; CRP 2mg/L; Hb 14.2 g/dL WBC 10.4 x 109/L, platelets 194 x109/L; Clotting profile normal

What is the differential diagnosis?
Which investigations would you request next?

Notes to editor

Authors: Dr Christopher H. Critoph, Dr Perry M. Elliott
Inherited Cardiovascular Disease Unit
The Heart Hospital - University College London - UK
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.