Duplication of the tricuspid valve.

Abstract

Duplication of an atrio-ventricular valve is a rare anomaly. In most instances the double, or even triple, atrio-ventricular orifice is on the left sidethe so-called "double mitral valve" (Hartmann, 1937; Wimsatt and Lewis, 1948;Schraft and Lisa, 1950;Prior, 1953; Wigle, 1957;Pachaly and Schultz, 1962;Edwards et al., 1965). Only exceptional cases of "double tricuspid valve" have been described (Sinapius, 1954;Pachaly and Schultz, 1962;Neufeld et al., 1960;Edwards et al., 1965). Another is reported here and a second is briefly reported.Case Reports Case 1. This 11-month-old boy had been deeply cyanosed from birth and was subject to frequent cyanotic attacks. On examination he was an underdeveloped child with central cyanosis and slight clubbing. A coarse systolic thrill was felt over the praecordium, associated with a grade 5/6 pansystolic murmur, with its maximum intensity in the mitral and tricuspid areas. There was also an early diastolic triple rhythm; the second sound was single.The electrocardiogram showed gross right atrial and ventricular hypertrophy patterns, with right axis deviation. The chest x-ray film showed an extremely large, globular heart with ischemic lung fields. The blood count showed no polycythemia or other abnormality.The electrocardiographic picture was thought to exclude tricuspid atresia or Ebstein's disease. Pulmonary atresia, with an intact ventricular septum and tricuspid incompetence, seemed the most likely diagnosis. The patient's condition was very poor, with daily cyanotic attacks, and the possibility of surgical relief was considered. Angiocardiography was, therefore, performed. The catheter entered the right atrium which was shown to be enormously dilated, occupying more than half the cardiac silhouette, and then passed to the left atrium and ventricle; it could not be manipulated into the right ventricle. An injection of opaque medium into the left ventricle showed an intact ventricular septum, mild 94:mitral incompetence (probably produced by the presence of the catheter), and a small atrial septal defect, possibly a foramen ovale. A Brock procedure for pulmonary valve dilatation was performed (G. Rabago) and an atretic or near atretic valve was opened to give an outlet to the right ventricular outflow tract. The immediate result was good, with relief of cyanosis, but the child died suddenly 24 hours later with cardiac arrest.Necropsy was limited to the heart. It weighed 110 g. Externally there was a very large right atrium but the ventricles and great vessels appeared normal. The right atrial wall was hypertrophied, and there was a small ostium secundum septal defect. The tricuspid valve, from its atrial aspect ( Figure A) presented two orifices, one anterior and the other lateral, with its medial half occupied by a rigid diaphragmatic septal cusp. Between the two orifices there was a fibrous band from the middle of the septal cusp to the lateral border of the fibrous annulus. The anterior orifice was roughly triangular, about 1-5 cm. across; its anterior edge had a normal...

Surgical Management of Double-Orifice Tricuspid Valve

“…Ebstein's anomaly associated with DOTV is extremely uncommon, with only four cases reported in the literature to date 19,21,27 . To our knowledge, this is the first case of a patient with Ebstein's anomaly associated with DOTV who underwent TV annuloplasty by using the accessory orifice as the functional main orifice of TV.…”

“…According to Sanchez Cascos, Rabago, and Sokolowski, 21 DOTV can be classified into three types based on the location of the defect: (1) the central type, in which a fibrous bridge divided the atrioventricular orifice completely into equal or unequal parts; (2) the hole type, in which an additional orifice with subvalvular apparatus occurred within a cusp or valve leaflet; and (3) the commissural type, in which the accessory orifice lay within a valve commissure. It is essential for the identification of DOTV that both orifices should be provided with a subvalvar tension apparatus.…”

“…In most cases, the patients’ mortality or morbidity is due to other associated congenital anomalies 20 . While the reported number of isolated DOTVs was too small to allow for meaningful conclusions, 17 in only a few cases the DOTV led to a modification of the surgery performed for the additional congenital heart defects, 28 and the existence of a DOTV was responsible for a more severe clinical course of the patient 21,27,29,30 …”

Duplication of the tension apparatus of the tricuspid valve

“…4,5 Although this anomaly is well known in the mitral position, duplication of the tricuspid valve is rare. 1,3,4 In these situations, it is the presence of accessory subvalvar components that distinguishes true duplication from simple fenestration of a valvar leaflet. 4,5 In our patient, there was a bar of muscle separating the two valvar orifices from each other (Figs 1 and 2).…”

Repairing the tricuspid valve in congenital heart diseases other than Ebstein’s

“…and Type 3, with each orifice being supported by its own tension apparatus. 15,20 Wang et al 17 state that the accessory orifice of the tricuspid valve has its own independent chordae tendinae and mastoid muscle and should be treated surgically when discovered.…”