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Concept: Tricuspid valve


Ebstein’s anomaly is a rare congenital heart malformation characterised by adherence of the septal and posterior leaflets of the tricuspid valve to the underlying myocardium. Associated abnormalities of left ventricular morphology and function including left ventricular noncompaction (LVNC) have been observed. An association between Ebstein’s anomaly with LVNC and mutations in the sarcomeric protein gene MYH7, encoding β-myosin heavy chain, has been shown by recent studies. This might represent a specific subtype of Ebstein’s anomaly with a Mendelian inheritance pattern. In this review we discuss the association of MYH7 mutations with Ebstein’s anomaly and LVNC and its implications for the clinical care for patients and their family members.

Concepts: Gene, Genetics, Evolution, Heart, Right ventricle, Tricuspid valve, Congenital heart defect, Ebstein's anomaly


Wilms' tumor (WT) is the most common pediatric renal tumor that often spreads to inferior vena cava and sometimes up to right atrium (RA). We describe successful management of 3-year-old child diagnosed with WT having extension up to RA. He was operated under cardiopulmonary bypass and extubated on postoperative day 2 and discharged. Perioperative anesthesia concerns were shock from dynamic tricuspid valve obstruction, intraoperative massive blood loss, and a higher risk of pulmonary thromboembolism during tumor manipulation.

Concepts: Oncology, Blood, Heart, Vein, Right atrium, Tricuspid valve, Wilms' tumor, Inferior vena cava


Percutaneous implantation of valved stents is now routinely performed to treat pulmonary valve regurgitation and stenosis. In addition, there are isolated reports of implantation of valved stents in the tricuspid position to treat prosthetic tricuspid stenosis or regurgitation when a prosthetic valve ring exists. We present a case in which a patient with combined severe tricuspid valve and pulmonary valve disease was successfully treated with sequential implantation of percutaneous valved stents in a single procedure. The procedure was straightforward, of short duration, and dramatically improved the patient’s functional status. © 2012 Wiley Periodicals, Inc.

Concepts: Tricuspid valve


OPINION STATEMENT: Ebstein anomaly (EA) is a rare congenital heart defect that may not be detected until late in adolescence or adulthood. Since the original description in a 19-year-old laborer with severe tricuspid valve (TV) regurgitation in 1866, our understanding of this rare condition has increased to the recognition that it is an abnormality not only of the TV, but also of the right ventricle (RV). EA is the result of failure of delamination of the TV leaflets from the interventricular septum, resulting in adherence of the leaflets to the underlying myocardium. This results in a wide variety of abnormalities, including apical and posterior displacement of the dilated TV annulus; dilation of the “atrialized” portion of the RV; and fenestrations, redundancy, and tethering of the anterior leaflet of the TV. The malformed TV is usually regurgitant, but may rarely be stenotic. The clinical manifestations of EA in the adult depend on several factors, including the extent of TV leaflet distortion, degree of tricuspid regurgitation (TR), right atrial pressure, and presence of a right-to-left atrial level shunt. Over the past several decades, advances in diagnostic imaging and surgical techniques have contributed to our current management of this challenging congenital heart defect.

Concepts: Heart, Right ventricle, Ventricle, Right atrium, Tricuspid valve, Congenital heart defect, Congenital heart disease, Ebstein's anomaly


Isolated tricuspid regurgitation (TR) can be caused by primary valvular abnormalities such as flail leaflet or secondary annular dilation as is seen in atrial fibrillation, pulmonary hypertension and left heart disease. There is an increasing recognition of a subgroup of patients with isolated TR in the absence of other associated cardiac abnormalities. Left untreated isolated TR significantly worsens survival. Stand-alone surgery for isolated TR is rarely performed due to an average operative mortality of 8%-10% and a paucity of data demonstrating improved survival. When surgery is performed, valve repair may be preferred over replacement; however, there is a risk of significant recurrent regurgitation after repair. Existing society guidelines do not fully address the management of isolated TR. We propose that patients at low operative risk with symptomatic severe isolated TR and no reversible cause undergo surgery prior to the onset of right ventricular dysfunction and end-organ damage. For patients at increased surgical risk novel percutaneous interventions may offer an alternative treatment but further research is needed. Significant knowledge gaps remain and future research is needed to define operative outcomes and provide comparative data for medical and surgical therapy.

Concepts: Medicine, Cardiology, Heart, Atrial fibrillation, Pulmonary artery, Right ventricle, Tricuspid valve, Tricuspid insufficiency


OBJECTIVE: To introduce a modified Sakakibara classification system for a ruptured sinus of Valsalva aneurysm. METHODS: From February 1, 2006, to January 31, 2012, surgical repair was performed on 159 patients with a ruptured sinus of Valsalva aneurysm at Fu Wai Hospital. Of the 159 patients, 105 were men and 54 were women, with a mean age of 33.4 ± 10.7 years. The patients were divided into 5 types according to the site of the ruptured sinus of Valsalva aneurysm rupture. The 5 types were as follows: type I, rupture into the right ventricle just beneath the pulmonary valve (n = 66); type II, rupture into or just beneath the crista supraventricularis of the right ventricle (n = 17); type III, rupture into the right atrium (type IIIa, n = 21) or right ventricle (type IIIv, n = 6) near or at the tricuspid annulus; type IV, rupture into the right atrium (n = 46); and type V, other rare conditions, such as rupture into the left atrium, left ventricle, or pulmonary artery (n = 3). RESULTS: Repair of ruptured sinus of Valsalva aneurysm through aortotomy was used in 100% of those with type V and 50% of those with type IIIv. In most patients with types I, II, and IV, repair was achieved through the cardiac chamber of the fistula exit (71.2%, 64.7%, and 69.6%, respectively). Both routes of repair were used in 76.2% of patients with type IIIa. No early and late deaths occurred. The aortic valve was replaced in 33 patients. One patient (type IV) underwent reoperation for a residual shunt during the follow-up period. CONCLUSIONS: The modified classification system for ruptured sinus of Valsalva aneurysm is simple and practical for clinical use.

Concepts: Blood, Heart, Cardiovascular system, Right ventricle, Ventricle, Tricuspid valve, Left ventricle, Aortic valve


Due to the increased life expectancy and continual improvements in cardiological treatment options, diseases of the tricuspid valve, in particular tricuspid valve insufficiency will become increasingly more recognized as an interventional target. While tricuspid stenosis is rare and can be effectively treated with balloon valvuloplasty, no effective transcatheter approach to tricuspid regurgitation (TR) has yet been established. As the tricuspid annulus is a complex and highly dynamic structure that offers little resistance, orthotopic long-term fixation of transcatheter valves with the current techniques is challenging and has not yet been performed in human patients. Alternative treatment concepts include transcatheter caval valve implantation (CAVI) to address the regurgitation of blood into the caval veins, which has resulted in hemodynamic improvement and is currently undergoing further clinical investigation. Other interventional treatment concepts are aimed at tricuspid valve repair, e.g. by annular plication with the Mitralign™ device or the TriCinch™ system. In the medium-term it can be assumed that percutaneous systems and therapy options will become available for these indications whereby the functional and prognostic effects of these treatment procedures will be corroborated in the appropriate patient groups by corresponding studies.

Concepts: Heart, Right atrium, Tricuspid valve, Annulus, Abstraction, Tricuspid insufficiency, Regurgitation, Valvular heart disease


We describe a minimally invasive heart surgery application of the EinsteinVision 2.0 3D high-definition endoscopic system (Aesculap AG, Tuttlingen, Germany) in an 81-year-old man with severe tricuspid valve insufficiency. Fourteen years ago, he underwent a Ross procedure followed by a DDD pacemaker implantation 4 years later for tachy-brady-syndrome. His biventricular function was normal. We recommended minimally invasive tricuspid valve repair. The application of the aformentioned endoscopic system was simple, and the impressive 3D depth view offered an easy and precise manipulation through a minimal thoracotomy incision, avoiding the need for a rib spreading retractor.

Concepts: Heart, Minimally invasive, Right ventricle, Tricuspid valve, Cardiac surgery, Aortic valve, Tricuspid insufficiency, Cleveland Clinic


A 33-year-old woman who had received a diagnosis of tricuspid valve endocarditis caused by MRSA was evaluated for replacement of the tricuspid valve. Videos show giant systolic pulsations during jugular venous examination and severe tricuspid regurgitation during transthoracic ECG.

Concepts: Cardiology, Heart, Tricuspid valve, Jugular vein, Tricuspid insufficiency, Regurgitation, Valvular heart disease, Heart valve


Severe tricuspid regurgitation is associated with poor prognosis; however, there are limited class 1 indications for intervention, and high-surgical risk patients may go untreated. We report the first-in-human successful transcatheter tricuspid valve repair for severe tricuspid regurgitation.

Concepts: Medical terms, Patient, Tricuspid valve, Tricuspid insufficiency, Regurgitation, Classes of United States Senators