Double trouble: rheumatic systemic tricuspid valve disease in congenitally corrected transposition of the great arteries.

A 39-year-old male presented with one year history of progressive dyspnoea on exertion that had progressed to breathlessness on minimal exertion (New York Heart Association class III). He had also become aware of palpitations. He stated he was informed in childhood that one of the heart valves was ‘narrowed’, and he received monthly antibiotic injections but had not followed up with any physician since. He had never had any surgical or percutaneous interventions. He was not taking any regular medications at initial presentation.

On examination, he had slow irregular pulse, 45/min and was normotensive. Cardiac auscultation demonstrated 3/6 systolic murmur and 2/6 holodiastolic murmur at cardiac apex. There were no clinical features of right or left heart failure. Electrocardiogram confirmed atrial fibrillation with slow ventricular response.

Transthoracic echocardiogram demonstrated atrioventricular as well as ventriculoarterial discordance (congenitally corrected transposition of the great arteries). Systemic tricuspid valve (sTV) leaflets demonstrate typical rheumatic valvular disease pattern with thickened leaflets tips, shortened chordae, and diastolic bowing of the valve leaflets (; Supplementary online). This was associated with severe regurgitation and moderate stenosis of the sTV (; Supplementary online). Additionally, left atrium (LA) was massively dilated (LA indexed volume 95 mL/m2). No interventricular or interatrial communication was visible, and there was no left ventricular outflow tract obstruction. Subsequent cardiac magnetic resonance (CMR) imaging confirmed the echocardiographic findings (; Supplementary online) and demonstrated reduced function of the systemic right ventricle (ejection fraction 48%) in the presence of severe regurgitation of the sTV (tricuspid regurgitant fraction 49%, tricuspid regurgitant volume 68 mL) without any myocardial scarring. There was no Ebstenoid malformation of the sTV. He was commenced on anticoagulation with warfarin and has been referred for surgical replacement of the sTV.

(A) Demonstrates a four-chamber echocardiographic view with apical offset of the systemic tricuspid valve with thickened leaflets and severely dilated left atrium. (B) Demonstrates significant regurgitation through the systemic tricuspid valve. (C) Displays continuous wave Doppler through the systemic tricuspid valve with moderate stenosis and significant regurgitation. (D) Exhibits horizontal long axis view on cardiac magnetic resonance displaying thickened systemic tricuspid valve, severely dilated left atrium and trabeculated systemic right ventricle.

Why rheumatic heart disease preferentially involves mitral valve remains unknown.[1,2] However, our case suggests that perhaps it is not the intrinsic mitral valve structure that predisposes it to the immunological crossfire but rather the location of the left sided atrioventricular valve in a high pressure, high oxygen tension pathway in the circulation.

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