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.

Tricuspid Valve Prolapse: An Uncommon Pathology Revealed by TEE.

A 73-year-old male with a history of dilated cardiomyopathy and paroxysmal atrial fibrillation underwent transthoracic echocardiography (TTE) to evaluate for endocarditis due to fever and gram-positive cocci in chains on blood cultures. TTE revealed a 3 × 8 mm mass on the ventricular aspect of the tricuspid valve ( Figure 1A ). Subsequent transesophageal echocardiography (TEE) showed that the mass in question was actually myxomatous degeneration of the tricuspid valve (TV) and redundant chordae with significant valve prolapse. Figure 1B shows the prolapsing TV leaflets at the same level as the mitral valve. Figure 1C and 1D show the valve at the level of the annulus in early systole and then prolapsing 8 mm in mid-late systole, respectively. Tricuspid valve prolapse (TVP) is uncommon, and one study of 118,000 patients reported an incidence of 0.3%.1 Since diagnostic parameters are not clearly defined, diagnosis is often determined subjectively. One objective criteria, > 2 mm atrial displacement of the TV leaflets in the TEE parasternal short-axis view, is noted to have high diagnostic accuracy. TVP is commonly associated with mitral valve prolapse. Patients with TVP have more severe tricuspid regurgitation and right-sided chamber enlargement compared to patients with no TVP. Due to the lack of significant tricuspid regurgitation in this case, the patient was reassured, and no further intervention was recommended.

Tricuspid valve prolapse as an early predictor for severe phenotype in children with Marfan syndrome.

AIM: In Marfan syndrome, various cardiovascular pathologies, such as aortic dilatation and mitral valve pathologies, already occur in childhood and determine course of the disease. This study aimed to establish additional cardiovascular risk markers for severe Marfan phenotypes. We investigated tricuspid valve prolapse (TVP) and its predictive value for outcome of paediatric Marfan disease. METHODS: In this retrospective, observational cohort study, we identified 130 paediatric Marfan patients (10.7 ± 4.8 years) with FBN1 variants. We divided patients into two groups based on TVP presence and performed a cross-sectional analysis to investigate the association of TVP with other cardiovascular, ocular and systemic pathologies, at first and last visit. A longitudinal analysis was performed with follow-up data. RESULTS: At baseline, patients with TVP had higher incidence of aortic root dilatation (p = 0.013), mitral valve prolapse (p = 0.0001) and systemic manifestations (p = 0.025) than patients without TVP. At follow-up, previous presence of TVP predicted higher probability of aortic root dilatation (p = 0.002), mitral valve prolapse (p = 0.0001) and systemic manifestations (p = 0.002). CONCLUSION: This shows that TVP is linked to both cardiac and extracardiac Marfan manifestations and TVP is an important marker for a disease severity in these children. Therefore, TVP should be assessed routinely using echocardiography in paediatric Marfan patients.

Characteristics and Significance of Tricuspid Valve Prolapse in a Large Multidecade Echocardiographic Study.

BACKGROUND: Characteristics of tricuspid valve prolapse (TVP) on transthoracic echocardiography are not well defined. As tricuspid valve interventions are increasingly considered, information on the definition and clinical significance of TVP is needed. METHODS: At the authors' institution, between January 26, 2000, and September 20, 2018, 410 patients (0.3%) were determined to have suspected TVP. These transthoracic echocardiograms and those of 97 age- and sex-matched normal control subjects were reviewed. Interrater agreement on TVP by visual inspection was assessed in a blinded subset. Leaflet atrial displacement (AD) > 2 SDs above the mean in normal control subjects was used to identify an empiric definition of TVP Features of patients meeting this definition were evaluated. RESULTS: Three hundred twelve transthoracic echocardiograms with available and interpretable images (76.1%) were included. Interrater agreement on TVP diagnosis by visual inspection was moderate. Normal values of AD were up to 4 mm in the right ventricular inflow view and 2 mm in all other views. AD > 2 mm in the parasternal short-axis view had the best accuracy against suspected TVP to identify TVP. Those with TVP by this definition more frequently had 3 to 4+ tricuspid regurgitation (22.2% vs 3.1%; P < .001), mitral valve prolapse (MVP; 75.0% vs 3.1%; P < .001), and more clinically significant MVP (greater prevalence of 3 to 4+ mitral regurgitation). No difference in mortality was observed in those with isolated TVP versus TVP and MVP (log-rank P = .93). CONCLUSIONS: In the largest study of TVP to date, interrater agreement on TVP diagnosis by visual inspection was moderate. A cutoff of >2-mm AD in the parasternal short-axis view was optimal to define TVP. Those with TVP by this definition had more significant tricuspid regurgitation, larger right ventricles, and more clinically significant MVP. Overall, these results suggest an increased role for surveillance for TVP and the need for clear diagnostic criteria in updated guidelines.

A rare case report: tricuspid valve prolapse due to spontaneous chordae rupture in a congenitally corrected transposition of the great arteries patient.

BACKGROUND: Congenitally corrected transposition of great arteries (CCTGA) is caused by atrioventricular and ventriculoarterial discordance. Cases of CCTGA with spontaneous chordae rupture of tricuspid valve have not been reported before. CASE PRESENTATION: Here we diagnosed a 38-year-old man, who was found CCTGA 14 years ago, as spontaneous chordae rupture by real-time three dimentional transesophageal echocardiography (RT-3D-TEE). The present case is the first report to describe a CCTGA patient combine with spontaneous chordae rupture in tricuspid valve. After tricuspid valve replacement, the patient was uneventful after 6 years' follow-up. CONCLUSION: We reported a rare case with spontaneous chordae rupture of tricuspid valve in a CCTGA patient and explored its etiology here. RT-3D-TEE is an important supplement to 2-dimentional transthoracic echocardiography and can provide more accurate detections in tricuspid valve diseases in CCTGA.

Rapidly Progressive Malignant Fibrous Histiocytoma of Right Atrium: a Rare Case Report.

We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

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.

Rapidly progressive malignant fibrous histiocytoma of right atrium: a rare case report

Abstract We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

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.

Infantile Marfan syndrome in a Korean tertiary referral center / 소아과

PURPOSE: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. METHODS: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. RESULTS: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. CONCLUSION: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.

Two- and Three-dimensional Transthoracic Echocardiographic Assessment of Tricuspid Valve Prolapse with Mid-to-Late Systolic Tricuspid Regurgitation.

We present the two-dimensional echocardiographic findings of tricuspid valve prolapse with mid-to-late systolic tricuspid regurgitation and describe the incremental value provided by live/real time three-dimensional transthoracic echocardiography. We also discuss a potential pitfall when assessing the severity of regurgitation in this setting.

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.

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.