Ebstein's anomaly of the tricuspid valve.

Ebstein's anomaly of the tricuspid valve is a rare lesion comprising less than 1% of patients with congenital heart disease. Among congenital heart lesions, Ebstein's anomaly is one of the most diverse in presentation, severity, and management. In its most severe form, it is also one of the most lethal. In this article we present a case of a patient who developed cardiac symptoms in adulthood. We follow this with a review of the pathology, clinical presentation, evaluation, and management of Ebstein's anomaly.

[Markers of connective tissue dysplasia in patients with idiopathic atrioventricular valve prolapse and anomalously sited chordae tendineae]. / Markery displazii soedinitel'noi tkani u bol'nykh s idiopaticheskim prolabirovaniem atrioventrikuliarnykh klapanov i s anomal'no rasplozhennymi khordami.

Phenotype was investigated in 86 patients with idiopathic prolapses of atrioventricular valves and in 21 patients with displaced chordae. 10 and 4 markers of connective tissue dysplasia were distinguished for patients with the prolapses and anomalous chordae, respectively. For patients with dysplasia of connective tissue of the heart a modified version of phenotypical length is proposed: norm-anomalous chordae--prolapse of the 1st degree--prolapse of the 2nd degree. For patients with 3 and more markers echo-CG registration is advisable to reject mitral and/or tricuspid valves.

[Tricuspid insufficiency with rupture of the chordae tendineae caused by closed thoracic trauma: evaluation by transesophageal echocardiography. Description of a case]. / Insufficienza tricuspidale con rottura di corde tendinee secondaria a trauma toracico chiuso valutata con ecocardiografia transesofagea. Descrizione di un caso.

A case of severe tricuspid insufficiency with ruptured chordae tendineae due to nonpenetrating major chest trauma caused by a car accident is described. Electrocardiographic signs of complete right bundle branch block and olosystolic murmur were present and not observed before. Transthoracic echocardiography showed a significant prolapse of the septal tricuspid leaflet with severe tricuspid regurgitation and severe right heart overload, which progressively worsened. Transesophageal echocardiography confirmed the transthoracic echocardiographic findings. It also demonstrated the presence of ruptured chordae tendineae and the coexistence of a severe prolapse of the tricuspid anterior leaflet with flail movement. Although the patient remained asymptomatic, these findings prompted us to refer the case to the surgeon. The patient underwent valvuloplasty with excellent late result. In presence of traumatic tricuspid insufficiency the use of transesophageal echocardiography can be helpful to optimize the anatomic evaluation of the valvular apparatus allowing adequate therapeutic decision.

[Valvular prolapse (clinical, echocardiographic, phonocardiographic and electrocardiographic features)]. / Prolapsy klapanov (klinicheskaia, ékhokardiograficheskaia, fonokardiograficheskaia i élektrokardiograficheskaia kharakteristiki).

Clinical, echoCG, PCG and ECG examinations were performed in 42 patients with primary isolated valve prolapses: mitral, aortal and tricuspid (32, 5 and 5 patients, respectively). It is shown that degree 1 regurgitation caused no hemodynamic disturbances, that of degree 2 and 3 brought about cardial complaints in more than half the patients, hemodynamic overstrain of the heart, sound symptoms similar to those of relevant valvular disease.

[Valvular prolapse in autosomal dominant polycystic kidney]. / Il prolasso valvolare nel rene policistico autosomico.

BACKGROUND: The involvement of the cardiovascular system in hereditary connective tissue disorders has been known for a long time and produces abnormalities that usually affect valves and arterial walls. Valvular diseases in typical autosomal dominant illnesses such as polycystic kidney (PK) have recently been reported. METHODS: To test the prevalence of valvular prolapses in this disease, we studied [with clinical, chest x-ray, electrocardiographic, and echocardiographic (monobidimensional and Doppler) examination] 21 subjects with PK (Group A) and 34 unaffected relatives (Group B). 36 subjects comparable in sex, age, blood pressure, body build and renal function, with other kinds of kidney diseases, were the control group (Group C). RESULTS: In group A, 7 subjects (33.3%) were found affected by mitral valve prolapse (MVP), of whom 3 also had mitral incompetence, 4 tricuspid valve prolapse and 2 aortic cusp prolapse with mild valvular insufficiency. Another 2 patients had evidence of mitral and aortic incompetence, respectively, without valvular prolapse, annulus ectasia or morphological features of a rheumatic valvular disease. In Group B, 8 subjects (23.5%) had MVP; In Group C, MVP was seen in only 1 patient (2.7%) and aortic incompetence in one other. Statistical analysis confirmed the significance of the greater prevalence of MVP in group A and B in comparison with group C (p < 0.01). CONCLUSIONS: The high prevalence of valvular abnormalities in autosomal dominant PK suggests a common genetic disorder producing a defect in the extracellular matrix. An alternative hypothesis is that of two discrete yet adjacent genes.

Systolic tricuspid leaflet prolapse in asymptomatic young men.

The degree of systolic movement of the tricuspid valve (TV) leaflets was measured in 100 consecutive apparently healthy men 18-20 years old at the time of maximum posterosuperior motion toward or into the right atrium. Backward bowing of greater than 0.20 mm2 of the anterior leaflet, and greater than 0.15 mm2 of either the posterior or septal leaflets beyond the plane of the tricuspid annulus was found in 5% or less of the cohort irrespective of the echocardiographic view in which it was recorded. The upper 5% of these young men had greater than 0.80 mm2 of backward bowing when the leaflet motion was summed from all three views. The anterior leaflet demonstrated more bowing than either the septal or posterior leaflets. The systolic TV prolapse area correlated highly with the degree of mitral leaflet prolapse (r = 0.654, p less than 0.001). We conclude that there is a wide continuous spectrum of tricuspid valve leaflet prolapse area in healthy young men. This quantitative approach may help standardize the echocardiographic evaluation of tricuspid leaflet motion.

[Prevalence of tricuspid and aortic valve prolapse in patients with mitral valve prolapse]. / Prevalência de prolapso valvar tricúspide e aórtico em portadores de prolapso valvar mitral.

Two-dimensional echocardiograms were performed in 30 patients with mitral valve prolapse (15 females and 15 males, with an average of 33.3). The main objective was to observe the prevalence of involvement of tricuspid and aortic valves. Tricuspid valve prolapse was observed in 43.3% with anterior and septal involvement in 92.3% and posterior involvement in 15.3%. The incidence of aortic prolapse was 10% with involvement of both right coronary and non-coronary leaflets. All patients with aortic valve prolapse showed involvement of both mitral leaflets and at least two tricuspid leaflets. It is concluded that involvement of other valves such as tricuspid (43.3) and aortic (10%) is a common finding in patients with mitral valve prolapse.

Prevalência de prolapso valvar tricúspide e aórtico em portadores de prolapso valvar mitral / Prevalence of tricuspid and aortic valve prolapse in patients with mitral valve prolapse

Foram estudados, através da ecocardiografia bidimensional, 30 pacientes portadores de prolapso da valva mitral, sendo 15 do sexo feminino e 15 masculino, com idade média de 33,3 anos, com o objetivo de observar a prevalência de concomitante prolapso valvar tricúspide e aórtico. O prolapso da valva tricúspide foi obsrvado em 43,3% dos pacientes (13/30 anos, sendo que o folheto anterior e septal estava acometido em 92,3% e o posterior em 15,3% dos casos. A prevalência de prolapso da valva aórtica foi de 10% dos casos (3/30), estando ambos os folhetos avaliados - coronariano direito e näo coronariano - acometidos. Todos os pacientes com prolapso valvar aórtico apresentavam acometimento dos dois folhetos mitrais e de, no mínimo, dois folhetos tricuspídeos. Conclui-se que é comum o acometimento simultâneo das valvas tricúspide e aórtica em pacientes portadores de prolapso da valva mitral

[Tricuspid regurgitation in mitral valve prolapse studied by two-dimensional color flow mapping].

To assess the incidence of tricuspid regurgitation (TR) in mitral valve prolapse (MVP), 96 patients with MVP and 23 normal control subjects were studied. Subjects in the MVP group were further classified as a group with mitral regurgitation (MR(+) group: 61 cases), and MR(-) group (35 cases). The presence of TR in each group was studied by two-dimensional color flow mapping using a Toshiba SSH-65A apparatus. The incidence of TR was 49% in the MR(+) group and 34% in the MR(-) group, and both (35 cases). The presence of TR in each group was studied by two-dimensional color flow mapping using a Toshiba SSH-65A apparatus. The incidence of TR was 49% in the MR(+) group and 34% in the MR(-) group, and both values were statistically greater than 9% in the control group (p less than 0.001 and p less than 0.05, respectively). A female preponderance was observed only in the MR(+) group. Tricuspid valve prolapse was observed in six cases (10%) in the MR(+) group, two cases (6%) in the MR(-) group, and none in the control group. The mean tricuspid ring dimension did not differ significantly among the three groups. The female patients in MR(+) group had statistically greater measurements than the normal female subjects (p less than 0.01). In conclusion, the incidence of TR was statistically greater in female patients in the MR(+) group than in females in the other groups. It is suspected that functional or pathological changes which induce MVP are likely to progress to the tricuspid ring in female patients.

Prolapse of atrioventricular valve leaflets in the setting of double orifice.

Prolapse of the valve leaflets is reported in 2 cases in the setting of double orifice atrioventricular valve. Diagnosis in the first case, an asymptomatic 8-year-old female, was achieved by echocardiography and consisted in prolapsing leaflets of a double orifice ("bridge type") mitral valve. The second patient, a 24-year-old male, died suddenly. Postmortem examination revealed prolapsing atrioventricular valves, with double orifice ("holy type") of the septal leaflet of the tricuspid valve. There was disruption of the penetrating atrioventricular bundle into multiple fragments with fasciculo-ventricular by-pass fibers.

[Echocardiographic diagnosis of plurivalvular prolapse. Apropos of a case]. / Diagnostic échocardiographique des prolapsus plurivalvulaires. A propos d'une observation.

Sonocardiography, especially bi-dimensional, currently represents an examination of choice in the detection of valvular prolapses. The authors report one case of a triple aorto-mitral-tricuspid valvular prolapse, diagnosed with the ultrasound technique. In light of this report and the data from the literature, the frequency and the distribution of valvular affections are discussed. The technical difficulties of recording and interpreting sonographic images responsible for false positive or false negative diagnostic errors are also studied.