Double-orifice-tricuspid-valve (DOTV) is rare with only 22 cases described. Only one incidence of preoperative diagnosis has been reported whereas double orifice mitral valve is routinely identified preoperatively. Embryological origins of the lesion are not well characterised. We detail our institutional experience with this defect.

Methods

A chart review was conducted with focus on echocardiographic assessment, coexisting lesions, surgical intervention and outcomes for cases diagnosed preoperatively in 2007-2008.

Results

Six cases were identified. Associated pathologies included inlet ventricular septal defect (VSD) (2), atrioventricular septal defect (1), d TGA/VSD (1), pulmonary atresia with VSD with major aortopulmonary collaterals (1) and univentricular connection with ccTGA, mitral atresia, hypoplastic left ventricle, VSD with infundibular and valvar pulmonic stenosis (1).

Modification of the subcostal-long axis view, apical-four-chamber and parasternal views were helpful. In cases with VSD, malalignement of interventricular and interatrial septae was a feature with the accessory orifice covering the defect created by the offset.

All but one underwent surgical correction involving the closure of the minor orifice and patch closure of the VSD, often requiring temporary detachment of tricuspid supports for both the orifices. Postoperative complete heart block (CHB) was observed two with one needing permanent pacemaker. One had residual trivial TI.

Conclusions

In complex pathology the diagnosis of DOTV may be overlooked if unaware of its possibility. CHB may occur given the position of the bundle on the left side of the septum and need to attach VSD patches close to this area. Repair may be complex but the outcomes are good.