Brit. Heart J., 1967, 29, 943. CASE REPORTS Duplication of the Tricuspid Valve A. SANCHEZ CASCOS, P. RABAGO, AND M. SOKOLOWSKI From the Cardiac Department, Fundacion Jimenez Diaz, Madrid, Spain 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. 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 Case Reports orifices, one anterior and the other lateral, with its Case 1. This 11-month-old boy had been deeply medial half occupied by a rigid diaphragmatic septal cyanosed from birth and was subject to frequent cyanotic cusp. Between the two orifices there was a fibrous band attacks. On examination he was an underdeveloped from the middle of the septal cusp to the lateral border of child with central cyanosis and slight clubbing. A the fibrous annulus. The anterior orifice was roughly coarse systolic thrill was felt over the praecordium, triangular, about 1-5 cm. across; its anterior edge had a associated with a grade 5/6 pansystolic murmur, with its normal anterior valve cusp whose chords arose from a maximum intensity in the mitral and tricuspid areas. normal Lancisi's muscle. The lateral orifice was much There was also an early diastolic triple rhythm; the smaller (less than 5 mm. across), and was guarded by two rudimentary cusps attached to chordce arising from second sound was single. The electrocardiogram showed gross right atrial and two small accessory papillary muscles. Attached by a ventricular hypertrophy patterns, with right axis devia- delicate fibrous network to the atrial aspect of the septal tion. The chest x-ray film showed an extremely large, cusp was a small (5 mm.) white nodule (Figure B). Histoglobular heart with ischemic lung fields. The blood logical examination of the nodule revealed a hyaline constitution and the network seemed to be an atypically count showed no polycythemia or other abnormality. The electrocardiographic picture was thought to ex- placed rest of the Chiari network. The right ventricle was of normal size but its wall was clude tricuspid atresia or Ebstein's disease. Pulmonary atresia, with an intact ventricular septum and tricuspid hypertrophied; the septum was intact. The pulmonary incompetence, seemed the most likely diagnosis. The valve had been opened to produce a 5 mm. orifice, but patient's condition was very poor, with daily cyanotic the cusps were fused. The left atrium and ventricle attacks, and the possibility of surgical relief was con- were normal, but the lateral cusp of the mitral valve sidered. Angiocardiography was, therefore, performed. had short chordc attached directly to the ventricular The catheter entered the right atrium which was shown wall, resulting in mild incompetence of the valve. The to be enormously dilated, occupying more than half the aorta and pulmonary artery were normal and the ductus cardiac silhouette, and then passed to the left atrium and arteriosus had closed. ventricle; it could not be manipulated into the right ventricle. An injection of opaque medium into the left Case 2. This 17-year-old boy is only briefly menventricle showed an intact ventricular septum, mild tioned here, as he was an example of Ebstein's disease, 94: 944 Cascos, Ribago, and Sokolowski FIG.-A.-Case 1: tricuspid valve, right atrial view, showing bigger anterior orifice, and smaller lateral one. B.-Case 1: rudimentary atrial network and hyaline tubercle on the tricuspid valve. C.-Case 2: tricuspid valve, atrial view. The arrow points to the hole on the anterior leaflet. D.-Case 2: tricuspid valve, ventricular view. The arrow marks the site of the hole, under which a rudimentary papillary muscle and two small chordee are seen. and will be reported as such elsewhere. He presented with slight dyspncea on exertion and precordial pain. On auscultation a triple rhythm and a pansystolic murmur were heard. The electrocardiogram showed large P waves with right bundle-branch block and a Q wave from Vl-4. The cardiac outline on the chest x-ray film was globular and the lung fields were oligemic. Cardiac catheterization was attempted, but the patient developed ventricular fibrillation which resisted correction. Necropsy of the heart revealed a large right atrium with a typical Ebstein's anomaly of the tricuspid valve which had a normal attachment of the anterior cusp, while the septal cusp was attached 2-5 cm. below the annulus fibrosus; the posterior cusp was rudimentary. The anterior cusp had a 4 mm. hole in its medial part (Figure C); two small chords. arising from a rudimentary papillary muscle were attached to the ventricular aspect of this hole (Figure D). Discussion Hartmann (1937) was the first to attempt a classification of the varieties of double atrio-ventricular valve. He distinguished three types: (1) Type L ("Loch"), in which the secondary orifice is defined by a constriction in the anterior commissure. This orifice has two cusps attached to chordae tendinese arising from the anterior papillary muscle. (2) Type B ("Brucke"), in which the two orifices are separated by a fibrous band; each is associated Duplication of the Tricuspid Valve 945 with one papillary muscle. (3) Type S (" Sonderstellung"), in which the separation of the orifices is more complete and each has an independent set of chord. and a papillary muscle. In this classification Type L is distinct from the other two, but the latter are less well separated. There is another variety which has not been included, so that a better classification would be as follows. anomaly has been described (Baker, Brinton, and Channell, 1950), but here the distinction between a simple fenestration without any subvalvar apparatus and duplication of the orifice with such apparatus, as in Case 2, has to be made. (A) Commissural type (Hartmann's type L) in which the accessory orifice is at the end of a valve commissure and its subvalvar apparatus (chordt and papillary muscle) is the normal one for that commissure, though sometimes accessory papillary muscles may be present. An example of a double tricuspid valve occurring in association with pulmonary atresia and an intact ventricular septum is reported. A second case in association with Ebstein's anomaly of the valve is briefly mentioned. A classification of the different varieties of duplication of an atrio-ventricular valve orifice is proposed. (B) Central type (Hartmann's types B and S). A fibrous band divides the atrio-ventricular orifice into either equal or unequal parts. (C) Hole type. In this variety the accessory orifice is a hole in a cusp. This form of double valve orifice is to be distinguished from a simple fenestration or cleft which has no subvalvar apparatus. It is essential for the identification of duplication of a valve that both orifices should be provided with a subvalvar apparatus, though this may be rudimentary for one of them. On the basis of this classification, Case 1 is an example of the central type and Case 2 of the hole type. The syndrome of pulmonary atresia or extreme stenosis with intact ventricular septum is well known. Greenwold et al. (1956) and more recently Davignon et al. (1961) have emphasized the occurrence of two anatomical types, depending upon the status of the tricuspid valve. In the commoner type I, both right ventricle and tricuspid valve are minute; these hearts typically present anomalous connexions between the coronary arteries and the right ventricular cavity through persistent coronary sinusoids. The rarer type II has a right ventricle which is normal or enlarged, with a tricuspid valve which is deformed and incompetent. The deformity often resembles that of the Ebstein's anomaly (Davignon et al., 1961; Elliott, Adams, and Edwards, 1963; Caddell and Whittemore, 1963). In other cases there is a less specific abnormality or an absence of tricuspid valve tissue which produces valvar incompetence (Davignon et al., 1961; Benton et al., 1962; Elliott et al., 1963; De Raibago et al., 1964; Kanjuh et al., 1964). To our knowledge, duplication of the tricuspid valve has not hitherto been reported in this condition. Fenestration of the tricuspid valve in Ebstein's Summary We are indebted to Dr. Dennis Deuchar, of the Cardiac Department, Guy's Hospital, London, who kindly revised the text and gave us valuable suggestions. References Baker, C., Brinton, W. D., and Channell, G. D. (1950). Ebstein's disease. Guy's Hosp. Rep., 99, 247. Benton, J. W., Elliott, L. P., Adams, P., Anderson, R. C., Hong, C. Y., and Lester, R. G. (1962). Pulmonary atresia and stenosis with intact ventricular septum. Amer. J. Dis. Child., 104, 161. Caddell, J. L. and Whittemore, R. (1963). Pulmonary atresia with dilated right ventricle. A case with congenital atrial flutter. Amer. J. Cardiol., 12, 254. Davignon, A. L., Greenwold, W. E., DuShane, J. W., and Edwards, J. E. (1961). Congenital pulmonary atresia with intact ventricular septum. Clinicopathologic correlation of two anatomic types. Amer. Heart_7., 62, 591. De Rabago, P., Sokolowski, M., Sanchez Cascos, A., and Varela de Seijas, J. R. (1964). El sindrome de atresia o estenosis extrema de arteria pulmonar con tabique interventricular normal. Rev. esp. Cardiol., 17, 352. Edwards, J. E., Carey, L. S., Neufeld, H. N., and Lester, R. G. (1965). Congenital Heart Disease. Correlation of Pathologic Anatomy and Angiocardiography. Saunders, Philadelphia and London. Elliott, L. P., Adams, P., Jr., and Edwards, J. E. (1963). Pulmonary atresia with intact ventricular septum. Brit. Heart J., 25, 489. Greenwold, W. E., DuShane, J. W., Burchell, H. B., Bruwer, A., and Edwards, J. E. (1956). Congenital pulmonary atresia with intact ventricular septum: two anatomic types. Proc. 29th Sci. Sess., Amer. Heart Ass., page 51. (Abstract in Circulation, 14, 945 (1956).) Hartmann, B. (1937). Zur Lehre der Verdoppelung des linken Atrioventrikularostiums. Arch. Kreisl.-Forsch., 1, 286. Kanjuh, V. I., Stevenson, J. E., Amplatz, K., and Edwards, J. E. (1964). Congenitally unguarded tricuspid orifice with coexistent pulmonary atresia. Circulation, 30, 911. Neufeld, H. N., McGoon, D. C., DuShane, J. W., and Edwards, J. E. (1960). Tetralogy of Fallot with anomalous tricuspid valve simulating pulmonary stenosis with intact septum. Circulation, 22, 1083. Cascos, Rabago, and Sokolowski 946 Pachaly, L., and Schultz, H. (1962). Zur formalen Genese der angeborenen Luckenbildungen der Atrioventrikularklappen des Herzens. Frankfurt Z. Path., 71, 531. Prior, J. T. (1953). Congenital anomalies of the mitral valve. Two cases associated with long survival. Amer. Heart J7., 46, 649. Schraft, W. C., and Lisa, J. R. (1950). Duplication of the mitral valve. Case report and review of the literature Amer. Heart J., 39, 136. Sinapius, D. (1954). Angeborene Tricuspidalinsuffizienz infolge eines Defektes im vorderen Klappensegel. Frankfurt. Z. Path., 65, 459. Wi gle, E. D. (1957). Dupicaton of the mitral valve. Bi.HatJ,1,26 Wimsatt, W. A., and Lewis, F. T. (1948). 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