Neonatal Marfan Syndrome.

OBJECTIVE: The Marfan syndrome (MFS) is an autosomal dominant disorder of connective tissue resulting from pathogenic variants of the fibrillin-1 gene (FBN1) with skeletal, cardiac, and ocular involvement. STUDY DESIGN: We report on a full-term male neonate, who showed at birth characteristics and dysmorphisms suggestive of nMFS, combined with the detection of severe cardiovascular disease. A multidisciplinary team made up of neonatologists and pediatricians, cardiologists, geneticists, ophtalmologists, physiatrists and physioterapists was formed to manage this patient. RESULTS AND CONCLUSION: Early diagnosis of this rare condition is critical for adequate treatment and specific follow-up, and impacts significantly on prognosis.

Tricuspid edge-to-edge repair for tricuspid valve prolapse and flail leaflet: feasibility in comparison to patients with secondary tricuspid regurgitation.

AIMS: Transcatheter tricuspid edge-to-edge repair (T-TEER) has gained widespread use for the treatment of tricuspid regurgitation (TR) in symptomatic patients with high operative risk. Although secondary TR is the most common pathology, some patients exhibit primary or predominantly primary TR. Characterization of patients with these pathologies in the T-TEER context has not been systematically performed. METHODS AND RESULTS: Patients assigned to T-TEER by the interdisciplinary heart team were consecutively recruited in two European centres over 4 years. Echocardiographic images were evaluated to distinguish between primary and secondary causes of TR. Both groups were compared concerning procedural results. A total of 339 patients were recruited, 13% with primary TR and 87% with secondary TR. Patients with primary TR had a smaller right ventricle (basal diameter 45 vs. 49 mm, P = 0.004), a better right ventricular function (fractional area change 45 vs. 41%, P = 0.001), a smaller right (28 vs. 34 cm2, P = 0.021) and left (52 vs. 67 mL/m2, P = 0.038) atrium, and a better left ventricular ejection fraction (60 vs. 52%, P = 0.005). The severity of TR was similar in primary and secondary TR at baseline (TR vena contracta width pre-interventional 13 ± 4 vs. 14 ± 5 mm, P = 0.19), and T-TEER significantly reduced TR in both groups (TR vena contracta width post-interventional 4 ± 3 vs. 5 ± 5 mm, P = 0.10). These findings remained stable after propensity score matching. Complications were similar between both groups. CONCLUSION: T-TEER confers equally safe and effective reduction of TR in patients with primary and secondary TR.

Robot-assisted repair of tricuspid leaflet prolapse using standard valvuloplasty techniques.

While minimally invasive approaches are used routinely to correct severe mitral regurgitation due to leaflet prolapse, isolated tricuspid valve prolapse is less frequent and usually addressed via sternotomy. A 34-year-old female presented with exertional dyspnea and severe tricuspid regurgitation due to an unsupported anterior leaflet causing prolapse, a tethered septal leaflet, and dilated annulus. Herein, the technique is described of a robot-assisted tricuspid valve repair using established open valvuloplasty principles. The robotic repair was performed by the placement of Gore-Tex neochordae from the anterior papillary muscle to the anterior tricuspid leaflet, plication of the anteroseptal and anteroposterior commissures, closure of an anterior leaflet cleft, and the insertion of an annuloplasty band. The patient had an uncomplicated hospital course and was dismissed home on the third postoperative day.

Tricuspid valve repair with artificial chordae in a 72-year-old woman.

Expanded polytetrafluoroethylene sutures have been used for the replacement of chordae tendineae since 1985, especially for mitral valve prolapse. There are only a few reports of artificial chordae tendineae to treat tricuspid valve regurgitation. We report on a 72-year-old woman in NYHA class III preoperatively, who underwent successful tricuspid valve repair after preoperative echocardiography revealed tricuspid valve regurgitation grade IV, caused by prolapse of the anterior leaflet (A1-A2) and annular dilatation. Tricuspid valve repair was performed using artificial chords consisting of two polytetrafluoroethylene sutures and a ring annuloplasty. Postoperative echocardiography revealed mild tricuspid valve regurgitation of less than 1°, even after three years. Gore-Tex® sutures as used in mitral valve repair are an excellent option to replace chordae tendineae in tricuspid valve prolapse. This approach represents a safe and effective technique for tricuspid valve repair.

Ex vivo hydrodynamics after central and paracommissural edge-to-edge technique: A further step toward transcatheter tricuspid repair?

OBJECTIVES: Transcatheter approaches in heart valve disease became tremendously important and are currently established in the aortic position, but transcatheter tricuspid repair is still in its beginning and remains challenging. Replicating the surgical edge-to-edge technique, for example, with the MitraClip System (Abbott Vascular, Santa Clara, Calif), represents a promising option and has been reported successfully in small numbers of cases. However, up to now, few data considering the edge-to-edge technique as a transcatheter approach are available. This study aims to determine the ex vivo hydrodynamics after the central and paracommissural edge-to-edge technique in different pathologies. METHODS: Because of basal or apical dislocation of papillary muscles, leaflet prolapse or tethering was simulated in porcine tricuspid valves mounted on a flexible holding device. Central and paracommissural edge-to-edge techniques were evaluated successively in these pathologies. Regurgitant volume and mean transvalvular gradient were determined in a pulse duplicator. RESULTS: In this ex vivo model, the isolated edge-to-edge technique reduced tricuspid regurgitation. In the prolapse model, regurgitant volume decreased significantly after central edge-to-edge technique (from 49.4 ± 13.6 mL/stroke to 39.3 ± 14.1 mL/stroke). In the tethering model, both the central and the paracommissural edge-to-edge techniques led to a significant decrease (from 48.7 ± 13.9 to 43.6 ± 15.6 and to 41.1 ± 13.8 mL/stroke). In all cases, the reduction of regurgitant volume was achieved at the cost of significantly increased mean transvalvular gradient. CONCLUSIONS: This study provides a reduction of tricuspid regurgitation after the edge-to-edge technique in the specific experimental setup. Whether this reduction is sufficient to treat tricuspid regurgitation successfully in clinical practice remains to be established. Transcatheter approaches need to be evaluated further, probably with regard to concomitant annuloplasty for higher reduction of tricuspid regurgitation.

Repair of a dysplastic tricuspid valve using artificial chordae: case report.

Atrioventricular valve repair with artificial chordal replacement has been widely used for congenital and acquired mitral valve abnormalities, but not for tricuspid valve abnormalities. A case is presented of dysplastic tricuspid valve that was successfully repaired using artificial chordae. A 2-year-old female presented with poor weight gain. Echocardiography revealed severe tricuspid regurgitation due to dysplastic tricuspid valve, poor coaptation by prolapse of the anterior leaflet, and tethering of the septal leaflet by short chordae. The prolapsed anterior leaflet was repaired with three pairs of 6-0 expanded polytetrafluoroethylene sutures. The short chordae of the septal leaflet were detached, and the septal and posterior leaflets were sutured together. Trivial tricuspid regurgitation was noted postoperatively. There was no tricuspid regurgitation during the follow up period of three years. The present case provides further evidence that artificial chordal replacement is a useful technique even for small children with congenitally abnormal tricuspid valves.

[Surgical treatment of flail leaflet of tricuspid valve].

OBJECTIVE: To explore the approach and the result in tricuspid valve insufficiency treatment by a cusp remodeling technique. METHODS: Nine patients with severe tricuspid regurgitation, congenital lack of chordae in 6 cases and traumatic rupture of chordae in 3 cases, underwent surgical repair between April 1997 and March 2006. There were six male and three female. Their ages ranged from 8 years to 57 years. One or two segments of flail leaflets were presented in these patients. Valve repair was performed by suture of the free edge of the affected cusp segment, plication of the segment of annulus devoid of leaflet, and fixation of the neo-annulus with a flexible annuloplasty ring. RESULTS: All patients survived and recovered after the operation. Echocardiography showed good coaptation with no regurgitation of the tricuspid valve in six patients and a mild residual tricuspid regurgitation in three. A remarkable decrease in the diameter of the right ventricle (anterior to posterior) was observed: from mean (43.6 +/- 4.2) mm (range 29 mm to 64 mm) preoperatively reducing to mean (24.0 +/- 1.8) mm (range 16 mm to 32 mm) postoperatively. All patients are doing well in 1 month to 109 months follow up. CONCLUSION: The procedure provided a simple and valuable option for repair of flail leaflet of tricuspid valve caused by congenital lack of chordae or traumatic rupture of chordae.

Tricuspid valve prolapse associated with myxomatous degeneration.

Two surgical patients are presented with tricuspid valve prolapse. One had severe isolated prolapse of the posterior leaflet at its junction with the anterior leaflet accompanied by chordal elongation that was successfully repaired; the other had mild prolapse of all three leaflets with chordal elongation. Myxomatous degeneration of the tricuspid valve was the suspected underlying pathologic disorder in both patients and was histologically proven in the resected leaflet tissue of patient 1.

[Aortic and tricuspid valve involvement caused by myxoid degeneration associated with a severe mitral prolapse. Apropos of 2 patients undergoing a double valve replacement]. / Atteintes valvulaires aortique et tricuspidienne par dégénérescence myxoïde associées au prolapsus mitral grave. A propos de 2 patients ayant bénéficié d'un double remplacement valvulaire.

Idiopathic mitral valve prolapse due to severe myxoid degeneration may be associated with aortic and/or tricuspid valve disease of the same aetiology. These localisations, which usually give rise few symptoms, can be detected by echocardiography. Sometimes, however, they may give rise to serious valvular regurgitation requiring surgical correction at each diseased valve. The authors report two cases of "floppy" mitral valve prolapse, the first requiring mitral and tricuspid, and the second, mitral and aortic, valve replacement. These cases underline the value of especially by echocardiography, in view of their serious complications.

Isolated tricuspid valve prolapsed: identification using two- and three-dimensional echocardiography and transoesophageal echocardiography.

We present a case of isolated prolapse of the tricuspid anterior leaflet in an asymptomatic 34-year-old man who was referred to our hospital for a routine check up. We performed two-and three-dimensional transoesophageal echocardiography (TEE). We found three-dimensional TEE a useful, non-invasive tool that can provide additional information to two-dimensional echocardiography in the assessment of tricuspid valve prolapse.

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.

[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.