Double orifice tricuspid valve in an infant with partial atrioventricular septal defect.

Double orifice Tricuspid Valve is an extremely rare malformation with few case reports. We report such a case in an infant associated with atrioventricular septal defect.

Double orifice tricuspid valve (DOTV) is an extremely rare malformation first reported by Greenfild.[1] Each orifice is provided with a fully developed subvalvular tensor apparatus. It can either occur alone or in association with other cardiac malformations, especially with atrio ventricular septal defect (AVSD) as in our case.

One year old female child, weighing 7.7 kg was admitted to the hospital with birth weight of 2.5 kg, failure to thrive and recurrent lower respiratory tract infections. On physical examination, there was alopecia, large pointed ears, ectodermal dysplasia, and motor developmental delay. There were no features of isomerism. On auscultation, grade 3/6 early systolic murmur was audible at left upper sternal border. Transthoracic 2 D echocardiography with color Doppler showed situs solitus, levocardia, concordant atrio-ventricular-and ventriculo-arterial connections, partial AVSD with large ostium primum defect with left-to-right atrial shunt, cleft anterior mitral leaflet with trivial mitral regurgitation, tiny left ventricular to right atrium (LV-RA) shunt via a small left ventricle to right atrial septal defect, severe pulmonary valvular stenosis (Max PG = 96 mmHg), no tricuspid regurgitation, there was a double orifice of the right ventricular component of the common AV valve [Figures 1 and 2]. The annulus of the 2 openings measured 5 mm and 6 mm, with separate tensor apparatus for each opening. Color flow mapping demonstrated non-obstructed flow across both the orifices [Figure 3]. The function of both the left and right ventricle was normal. Child underwent repair of the AVSD with pulmonary valvotomy. The accessory valve was left untreated as there was no regurgitation on testing with saline. The child had a smooth postoperative course. It is difficult to diagnose DOTV by echocardiography and most of the cases are diagnosed either intra-operatively or at an autopsy. Gajjar et al. have recommended that it is necessary to scan for an accessory orifice in all patients in whom the atrioventricular valve appears to be small or excessively large.[23]

Figure 1

2d echocardiography with apical 4c view, showing double orifice tricuspid valve. RA: Right atrium, RV: Right ventricle, LA: Left atrium, LV: Left ventricle

Figure 2

2d echocardiography with colour flow apical 4c view, showing flow through both the orifices of double orifice tricuspid valve (marked by arrow). RA: Right atrium, RV: Right ventricle, LA: Left atrium, LV: Left ventricle

Figure 3

2d echocardiography with subcostal short axis view (enface view) showing double orifice of right sided component of the common AV valve. RA: Right atrium, RV: Right ventricle, LA: Left atrium, LV: Left ventricle, Arrow: DOTV