Which One Predominates in Ebstein Anomaly: Tricuspid Regurgitation or Right Ventricular Dysfunction?

2020 ◽  
Vol 109 (5) ◽  
pp. 1626 ◽  
Author(s):  
Ismail Yurekli ◽  
Mert Kestelli ◽  
Habib Cakir
Author(s):  
Iskander Slama ◽  
Lilia Zakhama ◽  
Basma Herbegue ◽  
Elhem Boussabah ◽  
Saoussen Antit ◽  
...  

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Robert Zilberszac ◽  
Andreas Gleiss ◽  
Ronny Schweitzer ◽  
Piergiorgio Bruno ◽  
Martin Andreas ◽  
...  

Abstract Long and mid-term data in Low-Flow Low-Gradient Aortic Stenosis (LFLG-AS) are scarce. The present study sought to identify predictors of outcome in a sizeable cohort of patients with LFLG-AS. 76 consecutive patients with LFLG-AS (defined by a mean gradient <40 mmHg, an aortic valve area ≤1 cm2 and an ejection fraction ≤50%) were prospectively enrolled and followed at regular intervals. Events defined as aortic valve replacement (AVR) and death were assessed and overall survival was determined. 44 patients underwent AVR (10 transcatheter and 34 surgical) whilst intervention was not performed in 32 patients, including 9 patients that died during a median waiting time of 4 months. Survival was significantly better after AVR with survival rates of 91.8% (CI 71.1–97.9%), 83.0% (CI 60.7–93.3%) and 56.3% (CI 32.1–74.8%) at 1,2 and 5 years as compared to 84.3% (CI 66.2–93.1%), 52.9% (CI 33.7–69.0%) and 30.3% (CI 14.6–47.5%), respectively, for patients managed conservatively (p = 0.017). The presence of right ventricular dysfunction (HR 3.47 [1.70–7.09]) and significant tricuspid regurgitation (TR) (HR 2.23 [1.13–4.39]) independently predicted overall mortality while the presence of significant TR (HR 3.40[1.38–8.35]) and higher aortic jet velocity (HR 0.91[0.82–1.00]) were independent predictors of mortality and survival after AVR. AVR is associated with improved long-term survival in patients with LFLG-AS. Treatment delays are associated with excessive mortality, warranting urgent treatment in eligible patients. Right ventricular involvement characterized by the presence of TR and/or right ventricular dysfunction, identifies patients at high risk of mortality under both conservative management and after AVR.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Ranchordas ◽  
P Oliveira ◽  
M Madeira ◽  
M Marques ◽  
T Nolasco ◽  
...  

Abstract A 36-old-man was admitted in hospital with fever and cough, fatigue, rapidly progressive exertional dyspnoea and orthopnoea. Transthoracic echocardiogram (TTE) revealed a mildly dilated left ventricle (LV) with very low ejection fraction (EF), normally sized right chambers, severe right ventricular dysfunction, moderate tricuspid regurgitation and pulmonary artery systolic pressure (PASP) of 52mmHg. The diagnosis of myocarditis was suspected, and he was transferred to the ICU in our centre to proceed the diagnostic work up. Cardiac magnetic resonance showed a severely dilated LV with an EF of 13% and a transmural fibrotic scar on the lateral wall, without signs of myocardial oedema. There was an apical thrombus. Right ventricular EF was 25%. Coronary angiography showed thrombotic occlusion of the circumflex artery with delayed filling of marginal branches. Endomyocardial biopsy was performed but no myocardial lesion was seen. Temporary inotropic support was needed for low cardiac output. Optimized therapy for heart failure and anticoagulation were given and he received an implantable cardioverter defibrillator before discharge in NHYA class II. Two months later, he was re-admitted in his local hospital, complaining of cough with sputum, fever and shortness of breath. He was hypotensive, tachycardic and cyanotic. Blood workup revealed renal failure, coagulopathy and liver dysfunction with C-reactive protein of 10 mg/dL, but no leucocytosis. NT pro-BNP was 2459 pg/mL. Empirical antibiotics and inotropes were started. TTE revealed an EF of 10-15%, moderate functional mitral regurgitation, right ventricular dysfunction, moderate tricuspid regurgitation, and a PASP of 50 mmHg. He was transferred back to our centre. On admission, he was hemodynamically stable with perfusions of dobutamine and noradrenaline. Work up for inclusion in heart transplant waiting list was initiated. However, he deteriorated rapidly. It was decided to implant a biventricular assist device (BiVAD) for circulatory support as bridge to transplant. A short/medium term assist device was chosen as it was expected that a donor would be found in less than a month, and if this was not the case, it could be upgraded to a medium/long term device. Cannulas for LVAD were introduced on the LV apex and ascending aorta and for RVAD in the femoral vein and pulmonary artery. Good flows were achieved on both devices and there were no complications in the immediate post-operative period. He was extubated and weaned off inotropes on day 2. On day 9, a heart donor was found, and he was transplanted. No complications occurred in the post-operative period. He was discharged home on day 24, and resumed an active life. This case illustrates the usefulness of temporary devices to bridge critically ill candidates to heart transplantation. Also, it emphasizes the importance of identifying patients who definitely need a BiVAD when end-stage heart failure involves both ventricles. Abstract P1328 Figure.


2013 ◽  
Vol 145 (5) ◽  
pp. 1234-1241.e5 ◽  
Author(s):  
Lina Maria Vargas Abello ◽  
Allan L. Klein ◽  
Thomas H. Marwick ◽  
Edward R. Nowicki ◽  
Jeevanantham Rajeswaran ◽  
...  

2008 ◽  
Vol 336 (3) ◽  
pp. 224-229 ◽  
Author(s):  
Angel López-Candales ◽  
Navin Rajagopalan ◽  
Beth Gulyasy ◽  
Kathy Edelman ◽  
Raveen Bazaz

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