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  • Title: [Congenital tricuspid insufficiency due to valvular dysplasia. Review of the literature in light of a case in a 40-year-old adult].
    Author: Lagarde O, Garabedian V, Coignard A, Duret JC, Piwnica A, Droniou J.
    Journal: Arch Mal Coeur Vaiss; 1980 Apr; 73(4):387-96. PubMed ID: 6778440.
    Abstract:
    UNLABELLED: Congenital tricuspid incompetence due to valvular dysplasia is a defect involving the leaflets (normally inserted on the ring) the cordae tendinae and papillary muscles of the tricuspid valve. It is a rare condition, usually diagnosed at open heart. A case of congenital tricuspid incompetence in a 40 year old adult is reported. Surgery indicated for resistant heart failure, confirmed the preoperative clinical, echocardiographic and haemodynamic diagnosis. The dysplasia comprised absence of cordae and papillary muscles on two thirds of the anterior leaflet. Valvular replacement with a bioprosthesis was carried out. 31 other reported cases were discovered on review of the literature since 1923. Two main groups of patients were individualised according to their clinical contexts and outcome: --The first group comprises the newborn and infants. Cyanosis, cardiomegaly and right ventricular failure in the neonatal period raised the differential diagnosis of Ebstein's anomaly and Uhl's disease. The outcome was fatal in several days to weeks. Autopsy confirmed the diagnosis. --The second group comprises patients who decompensate during adult life with right ventricular failure and degrees of tricuspid incompetence. The natural outcome with or without medical treatment may be prolonged, one such patient dying at the age of 53. Congenital tricuspid incompetence, diagnosed after elimination of other causes, was confirmed at operation. Complementary investigations: --Cardiac catheterisation showed tricuspid incompetence and its haemodynamic effects. --Echocardiography, in our case, showed dilatation of the right ventricle and pulsed Doppler echocardiography suggested tricuspid incompetence. The inability to record the anterior tricuspid leaflet, an unusual feature considering the right ventricular dilatation, contrasted with perfect definition of the septal leaflet. The absence of echocardiography in the other 31 reported cases does not allow any conclusions to be drawn as to the significances of this sign. --The tricuspid valve dysplasia was precisely defined on pathological examination: a) thickened valve, b) hypoplastic chordae and papillary muscles, c) incomplete separation between the leaflets and ventricular wall, d) focal agenesis of valvular tissues. --An associated pulmonary artery stenosis in the newborn may explain the severity of symptoms in this age group. This hypothesis, based only on pathological studies, cannot be confirmed. TREATMENT: --In the newborn, the rare surgical attempts at repair have all failed. --In the adult, surgery, indicated for heart failure resistant to all medical treatment, is as for other organic tricuspid incompetence, annuloplasty or valve replacement, by mechanical or bioprosthesis. Porcine bioprosthesis, as chosen in the case reported, has been used by some authors for 5-8 years with good results. Nevertheless, the longer-term outcome of this type of prosthesis is not yet known.
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