OBJECTIVE: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management.
METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement.
RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt.
CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.

Right-sided heart failure and tricuspid regurgitation are common and strongly associated with poor quality of life and an increased risk of heart failure hospitalizations and death. While medical therapy for right-sided heart failure is limited, treatment options for tricuspid regurgitation include surgery and, based on recent developments, several transcatheter interventions. However, the patients who might benefit from tricuspid valve interventions are yet unknown, as is the ideal time for these treatments given the paucity of clinical evidence. In this context, it is crucial to elucidate aetiology and pathophysiological mechanisms leading to right-sided heart failure and tricuspid regurgitation in order to recognize when tricuspid regurgitation is a mere bystander and when it can cause or contribute to heart failure progression. Notably, early identification of right heart failure and tricuspid regurgitation may be crucial and optimal management requires knowledge about the different mechanisms and causes, clinical course and presentation, as well as possible treatment options. The aim of this clinical consensus statement is to summarize current knowledge about epidemiology, pathophysiology and treatment of tricuspid regurgitation in right-sided heart failure providing practical suggestions for patient identification and management.

The diagnosis and management of mitral and tricuspid valve disease have undergone major changes in the last few years. The expansion of transcatheter interventions and widespread use of new imaging techniques have altered the recommendations for the diagnosis and treatment of these diseases. Because of the exponential growth in the number of publications and clinical trials in this field, there is a strong need for continuous updating of local protocols. The recently published 2021 European Society of Cardiology guidelines for the management of valvular heart disease did not include some of the new data on these new therapies and, moreover, the number of mitral and tricuspid interventions varies widely across Europe. Therefore, all this information must be summarized to facilitate its use in each specific country. Consequently, we present the consensus document of the Section on Valvular Disease, Cardiovascular Imaging, Clinical Cardiology, and Interventional Cardiology Associations of the Spanish Society of Cardiology for the diagnosis and management of mitral and tricuspid valve disease.

Tricuspid regurgitation (TR) is a highly prevalent condition and an independent risk factor for adverse outcomes. Multiple clinical guidelines exist for the diagnosis and management of TR, but the recommendations may sometimes vary. We systematically reviewed high-quality guidelines with a specific focus on areas of agreement, disagreement and gaps in evidence. We searched MEDLINE and EMBASE (01/01/2011 - 30/08/2021), the Guidelines International Network International, Guideline Library, National Guideline Clearinghouse, National Library for Health Guidelines Finder, Canadian Medical Association Clinical Practice Guidelines Infobase, Google Scholar and websites of relevant organizations for contemporary guidelines that were rigorously developed (as assessed by the Appraisal of Guidelines for Research and Evaluation II tool). Three guidelines were finally retained. There was consensus on a TR grading system, recognition of isolated functional TR associated with atrial fibrillation, and indications for valve surgery in symptomatic vs asymptomatic patients, primary vs secondary, and isolated TR forms. Discrepancies exist on the role of biomarkers, complementary multi-modality imaging, exercise echocardiography and cardiopulmonary exercise testing for risk stratification and clinical decision-making of progressive TR and asymptomatic severe TR, management of atrial functional TR and choice of transcatheter tricuspid valve intervention (TTVI). Risk-based thresholds for quantitative TR grading, robust risk score models for TR surgery, surveillance intervals, population-based screening programmes, TTVI indications and consensus on endpoint definitions are lacking.

The Get-With-The-Guidelines-Heart-Failure (GWTG-HF) score is a risk assessment tool to predict mortality in patients with heart-failure (HF). We aimed to evaluate the GWTG-HF score for risk stratification in HF patients with tricuspid regurgitation undergoing trans-catheter tricuspid valve repair (TTVR). In total, 181 patients who underwent TTVR via edge-to-edge repair (86%) or annuloplasty (14%) were enrolled. Patients were categorized into a low- (≤ 43 points), intermediate- (44-53 points) and high-risk score groups (≥ 54 points). TTVR led to an improvement of TR (p < 0.0001) and NYHA (p < 0.0001). Kaplan-Meier analysis and log-rank test revealed that higher GWTG-HF scores were associated with reduced rates of event-free survival regarding mortality (96% vs 89% vs 73%, respectively, p = 0.001) and hospitalization for heart failure (HHF) (89% vs 86% vs 74%, respectively, p = 0.026). After adjusting for important variables like renal function, left ventricular ejection fraction and mitral regurgitation, the GWTG-HF score remained an independent predictor of the composite endpoint of HHF or mortality (hazard ratio 1.04 per 1-point increase, p = 0.029). Other remaining predictors were renal function and mitral regurgitation. The GWTG-HF score used as a risk stratification tool of mortality and HHF maintains its prognostic value in a HF population with severe TR undergoing TTVR.

Transthoracic echocardiography is the first-line imaging modality in the assessment of right-sided valve disease. The principle objectives of the echocardiographic study are to determine the aetiology, mechanism and severity of valvular dysfunction, as well as consequences on right heart remodelling and estimations of pulmonary artery pressure. Echocardiographic data must be integrated with symptoms, to inform optimal timing and technique of interventions. The most common tricuspid valve abnormality is regurgitation secondary to annular dilatation in the context of atrial fibrillation or left-sided heart disease. Significant pulmonary valve disease is most commonly seen in congenital heart abnormalities. The aetiology and mechanism of tricuspid and pulmonary valve disease can usually be identified by 2D assessment of leaflet morphology and motion. Colour flow and spectral Doppler are required for assessment of severity, which must integrate data from multiple imaging planes and modalities. Transoesophageal echo is used when transthoracic data is incomplete, although the anterior position of the right heart means that transthoracic imaging is often superior. Three-dimensional echocardiography is a pivotal tool for accurate quantification of right ventricular volumes and regurgitant lesion severity, anatomical characterisation of valve morphology and remodelling pattern, and procedural guidance for catheter-based interventions. Exercise echocardiography may be used to elucidate symptom status and demonstrate functional reserve. Cardiac magnetic resonance and CT should be considered for complimentary data including right ventricular volume quantification, and precise cardiac and extracardiac anatomy. This British Society of Echocardiography guideline aims to give practical advice on the standardised acquisition and interpretation of echocardiographic data relating to the pulmonary and tricuspid valves.

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Despite guideline recommendations, rates of concomitant tricuspid valve repair are suboptimal, possibly due to fear of complications. We reviewed morbidity, mortality, recurrent tricuspid regurgitation, and right ventricular remodeling after guideline-directed concomitant tricuspid valve repair.
We performed guideline-directed concomitant tricuspid valve repair on 171 consecutive patients who underwent left-sided valve surgery (degenerative mitral surgery or aortic valve replacement) between May 2012 and March 2016. Exclusion criteria included functional mitral regurgitation, rheumatic disease, active endocarditis, and concomitant coronary artery bypass grafting or complex aortic surgery.
Mean age was 68 ± 12 years, and 47% (81 of 171) were women. Preoperative atrial fibrillation was present in 57% (98 of 171), and preoperative tricuspid regurgitation was moderate or higher in 64% (108 of 171). The rate of de novo pacemaker placement was 4.1% (7 of 171), and the 30-day mortality rate was 0.6% (1 of 171). Estimated survival was 95% ± 4% at 1 year and 92% ± 5% at 5 years. Freedom from moderate or worse residual/recurrent tricuspid regurgitation was 93% ± 6% at 6 months and 89% ± 8% at 3 years. Quantitative echocardiography found no significant increase in right ventricular dimensions or area at 1 year in subgroup analysis. Mean echocardiographic follow-up was 14.1 months, and mean clinical follow-up was 33.9 months.
Guideline-directed concomitant tricuspid valve repair resulted in excellent safety end points and survival. At 14 months, freedom from moderate or worse tricuspid regurgitation was high, right ventricular performance did not worsen, and the pacemaker rate was comparable to rates after isolated mitral repair. Given these findings, adherence to current guidelines regarding functional tricuspid regurgitation should be encouraged.

The aim of this study was to present a valve-in-valve (ViV) case and a step-by-step guideline on how to perform this procedure.
A 51-year-old female with a history of rheumatic heart disease and tricuspid valve replacement presented functional class deterioration, a transesophageal echocardiogram (TEE) revealed prosthetic dysfunction due to thrombosis; therefore, a valvular replacement with a 27 mm bioprosthesis (Carpentier-Edwards Perimount) was performed without complication. 3 years after the procedure, the patients presented functional class deterioration (NYHA-III) with tricuspid dysfunction by TEE and the heart team decided to perform a transcatheter tricuspid ViV replacement.
El objetivo de este artículo es presentar un caso clínico de un paciente en el cual se realizó el procedimiento valve-in-valve (ViV) en la válvula tricúspide junto con una guía de cómo llevar a cabo este procedimiento paso a paso.
Paciente femenino de 51 años de edad con antecedente de Cardiopatía Reumática presenta deterioro en clase funcional encontrándose por ecocardiografía transesofágica (ETE) disfunción de válvula protésica tricúspide secundario a trombosis, se realiza recambio valvular con prótesis biológica 27 mm (Carpentier Edwards Perimount) sin complicación. 3 años después, presenta deterioro de la clase funcional (NYHA-III) y se evidencia en ETE disfunción protésica tricúspide por lo cual se decide realizar un reemplazo ViV tricúspide transcatéter.

In this retrospective study 420 echocardiograms from a single center were reviewed showing that TAPSE was acquired in 66% while TA TDI s\' signals were recorded in 98% of all echocardiograms. Based on these results greater efforts are required to standardize acquisition and reporting of objective measurements of RV function.