DR Hakim Irfan Showkat, MBBCh, MD; Rekha Mishra, MD; Vinod Sharma, MD, DM; Lokesh Chandra Gupta, MD, DM; Sadaf Anwar, PGDCC
Duplication of an atrioventricular valve is an extremely uncommon congenital anomaly that generally affects the mitral rather than the tricuspid valve.[1–7] The isolated occurrence of this condition seems extremely rare and, in most cases, it is associated with other congenital cardiac malformations that determine a patient’s outcome. Even though the Double-Orifice Tricuspid Valve (DOTV) is a rare anomaly and can be easily missed if the physician is unaware of it; it is necessary to scan for an accessory orifice in all patients in whom the atrioventricular valve appears to be small or excessively large.
An 18-year-old girl presented to Outdoor Medical Department with a history of palpitations with mild breathlessness with NYHA Class II-III for a few months. On examination, she had a lean and thin build with cardiac examination showing left parasternal heave and normal S1 with split S2. There was a a systolic murmur well heard at apex. Electrocardiography showed sinus tachycardia with right bundle branch block. Two-D Echocardiography revealed Acyanotic Congenital Heart Disease, a Partial-Atrioventricular Canal Defect, a Double-Orifice Tricuspid Valve, gooseneck deformity of LV outflow and a large Primum ASD measuring 4.1cm with left-to-right shunt. A right atrium and ventricle were markedly dilated with the patient was advised to get admitted, but was lost to follow-up.
The division of an atrioventricular valve into two similar and functioning units is described as a duplication of the valvular apparatus or a double-orifice valve. Although this anomaly is well known in the mitral position, duplication of the tricuspid valve is rare. In these situations, it is the presence of an accessory subvalvular component that distinguishes true duplication from a simple fenestration of the valvar leaflet.[1–7]
To read the full article, please go to the August 2016 Issue of CCT.