Year : 2015  |  Volume : 8  |  Issue : 1  |  Page : 78--79

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

Neeraj Awasthy, Savitri Shrivastava 
 Department of Pediatric Cardiology, Fortis Escorts Heart Institute, New Delhi, India

Correspondence Address:
Neeraj Awasthy
Department of Pediatric Cardiology, Fortis Escorts Heart Institute, Okhla Road, New Delhi - 110 075


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.

How to cite this article:
Awasthy N, Shrivastava S. Double orifice tricuspid valve in an infant with partial atrioventricular septal defect.Ann Pediatr Card 2015;8:78-79

How to cite this URL:
Awasthy N, Shrivastava S. Double orifice tricuspid valve in an infant with partial atrioventricular septal defect. Ann Pediatr Card [serial online] 2015 [cited 2020 Sep 20 ];8:78-79
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Full Text

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 [Figure 1] and [Figure 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. [2],[3]{Figure 1}{Figure 2}{Figure 3}


1Sapre A, Gopalraj SS, Kottayil BP, Kumar RK. An unusual example of isolated double-orifice tricuspid valve. Ann Pediatr Cardiol 2013;6:162-3.
2Gajjar T, Desai N. Double-orifice tricuspid valve: A rare entity. Eur J Cardiothorac Surg 2012;41:1187-9.
3Wang BX, Yin BL, Pan YZ, Yin N, Chen C, He B. The clinical experience of double orifice tricuspid valve. Heart Lung Circ 2013;22:229-30.