The impact of tricuspid valve regurgitation severity on exercise capacity and cardiac-related hospitalisations among adults with non-operated Ebstein’s anomaly

2019 ◽  
Vol 29 (06) ◽  
pp. 800-807
Author(s):  
Jonathan Buber ◽  
Ori Vatury ◽  
Robert Klempfner ◽  
Shai Tejman-Yarden

AbstractBackground:Tricuspid valve regurgitation is an inherent part of Ebstein’s anomaly, yet whether the severity of the regurgitation further impairs exercise capacity and contributes to long-term morbidity on top of the lesion severity per se is unknown.Methods:To evaluate for this potential effect, we included 30 patients with Ebstein’s anomaly who did not undergo any form of surgical interventions and had a cardiopulmonary exercise test and echocardiographic studies in this retrospective analysis. Echocardiographic studies and cardiopulmonary exercise tests were critically reviewed for lesion severity grade, tricuspid regurgitation degree, and exercise parameters. Cardiac-related hospitalisations were recorded from computerised medical records and during clinic visits.Results:Fourteen patients (47%) had moderate and 8 (27%) had severe regurgitation. Patients with ≥ moderate regurgitation exhibited significantly lower exercise capacity (median % predicted maximal oxygen consumption, 62 versus 79%, p = 0.03) and venilatory efficiency at exercise. When stratifying exercise results by regurgitation degree, a stepwise decrease in oxygen consumption and ventilatory efficiency with increasing regurgitation severity was observed, regardless of the anatomic lesion severity. During a median follow-up of 4.6 years, > moderate tricuspid regurgitation was associated with significantly lower cumulative probability of freedom from cardiac hospitalisations.Conclusions:We report that among non-operated Ebstein’s anomaly patients, greater tricuspid regurgitation severity was associated with worse exercise capacity and with overall higher probability of cardiac-related hospitalisations independent from the underlying lesion severity.

2021 ◽  

Severe tricuspid valve regurgitation secondary to Ebstein’s anomaly represents several challenges in neonates. It can result in significant respiratory and/or hemodynamic compromise that mandates urgent interventions. When conservative management fails, 2 surgical options are available: tricuspid valve repair or single ventricle palliation. The overall results of neonatal tricuspid valve repair are unsatisfactory especially in sick neonates and those with preoperative hemodynamic instability. Single ventricle palliation utilizing the Starnes procedure with right ventricular exclusion provides a quicker way to improve hemodynamics and allows rapid decompression of the right ventricle but carries the long-term disadvantages of the single ventricle pathway. We were recently faced with a challenging case of neonatal Ebstein’s anomaly resulting in severe tricuspid valve regurgitation (TR) and significant hemodynamic and respiratory instability. We performed an initial stage I palliation with a modified Starnes’ procedure, which allowed stabilization and rapid recovery of the patient to be followed 5 months later with conversion to 2-ventricle repair using the cone technique. We believe combining these 2 strategies for suitable neonatal candidates may be a useful technique that should be considered in the algorithm for neonatal Ebstein’s anomaly.


2020 ◽  
Author(s):  
Eun-Young Choi ◽  
Eun Sun Kim ◽  
Jung Yoon Kim ◽  
Seong-Ho Kim ◽  
Jae Hong Lim ◽  
...  

Abstract Background: Ebstein’s anomaly exhibits a wide variety of clinical features, and therefore, proposing a standardized treatment for it is difficult. This study was conducted to determine whether Cone repair, which has been implemented in our hospital since 2008, is more effective than conventional repair.Methods: We retrospectively analyzed the clinical information of patients with Ebstein’s anomaly who were followed-up at the hospital from 2000 to 2019. A total of 61 patients who had undergone tricuspid valve repair after 2000 were divided into the conventional and Cone repair groups and their clinical outcomes were compared.Results: Of the 170 patients, 82 (48.2%) patients received surgical treatment for the tricuspid valve, whereas 75 patients received only medical treatment. The median follow-up duration was 5.89 years. After surgery, tricuspid valve regurgitation decreased and aortic stroke volume increased in both the Cone and conventional repair groups. In the Cone repair group, no mortality and postoperative complete atrioventricular block occurred and significantly fewer cases of moderate to severe tricuspid valve regurgitation were noted after surgery compared with the conventional repair group.Conclusions: Cone repair is thought to be a method with less mortality and less occurrence of complete atrioventricular block than conventional repair.


2019 ◽  
Vol 27 (8) ◽  
pp. 688-690 ◽  
Author(s):  
Kosuke Saku ◽  
Hironori Inoue ◽  
Keisuke Yamamoto ◽  
Masahiro Ueno

A cleft in the tricuspid valve, classified as congenital dysplasia, is a rare disease. Here, we report the case of a 79-year-old man with tricuspid regurgitation due to a cleft in the anterior leaflet. The patient underwent successful tricuspid valve repair with cleft closure, chordal reconstruction, and tricuspid annuloplasty.


2018 ◽  
Vol 66 (07) ◽  
pp. 572-574 ◽  
Author(s):  
Carlo De Filippo ◽  
Antonio Totaro ◽  
Piero Pelini ◽  
Michele Mauro ◽  
Antonio Calafiore

AbstractSurgical treatment of severe functional tricuspid regurgitation associated with dilated right ventricle and increased chordal tethering (>8 mm) is challenging. We designed a technique where the anterior and posterior leaflets are detached from 50% of the annulus and a patch as large as the tricuspid orifice is sewn to augment the leaflets' tissue to force the coaptation with the septal leaflet. Annuloplasty is not performed, as it can only increase the chordal tethering, reducing the benefit of tissue augmentation. Early and midterm results in a subgroup of patients with unfavorable anatomical aspects are encouraging.


2020 ◽  
Vol 23 (6) ◽  
pp. E781-E785
Author(s):  
Saikat Das Gupta ◽  
Mauin Uddin ◽  
Siddhartha Shankar Howlader ◽  
Prodip Kumar Biswas ◽  
Mohammed Kabiruzzaman ◽  
...  

Ebstein's anomaly is a rare and complexed heart defect that affects the tricuspid valve and is accountable for around 1% of congenital cardiac abnormalities. It is one of the most common congenital causes of tricuspid valve regurgitation. Ebstein's anomaly is often diagnosed prenatally due to its severe cardiomegaly. Some individuals with this anomaly do not experience any complications until adulthood and even then its mostly minor complaints like exercise intolerance.  Atrial septal defect is most commonly (70-90%) associated with Ebstein's anomaly. However, ventricular septal defect (VSD) can be associated with 2-6% of the cases. This particular report presents a case of surgical intervention for a 20 years old female with Ebstein's anomaly that had multiple VSD's and a severe Pulmonary Stenosis (PS).


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
E Ostenfeld ◽  
F Simard ◽  
G Smith ◽  
S Ghonim ◽  
R Rydman ◽  
...  

Abstract Funding Acknowledgements Swedish Heart-Lung Foundation, Swedish Society of Medicine, Swedish Heart Association, Skåne University Hospital, Region Skåne, and Lund University Background Ebstein’s anomaly (EA) is due to failed delamination of the tricuspid valve (TV) causing TV regurgitation (TR) and right ventricular (RV) dilatation, reduced exercise capacity and survival. TV repair/replacement (TVR) aims to reduce morbidity and mortality by reducing regurgitation and RV dilatation. Exercise capacity measured by predicted peak oxygen uptake (VO2%) and ventilatory expiratory slope (VE/CO2) is often impaired in EA and both are markers for mortality in adult congenital heart disease. Cardiovascular magnetic resonance (CMR) derived risk factors for adverse events (MACE) in unrepaired EA include reduced biventricular ejection fraction (EF) and low cardiac index. Whether and how these markers are modulated by TVR requires study. Purpose We aimed to assess whether TVR modulates known markers of exercise intolerance and CMR risk factors for MACE in EA. Methods Thirty-six consecutive EA adult (age≥16 years) patients (age at operation 37.4 ± 15.4 years, 13/12 ASD/PFO closures, 64% women) who underwent TV repair/replacement (15/21) between 2004-2014 and had pre-TVR CMR were retrospectively included. Twenty-four had CMR (in median 1.7 years) after TVR. Thirty-four had cardiopulmonary exercise test with VO2% and VE/CO2 prior to TVR, 23 one year and 17 five years after TVR. For CMR biventricular assessment, delineations were performed in short-axis cine stacks. Cardiac index was computed from velocity encoded phase contrast images from aortic flow. All volumes were indexed to body surface area. TR was graded none to severe (0-3). Results Thirty patients were in NYHA class ≥2 pre-TVR, 10 at 1-year and 8 at 5-year post-TVR (Figure 1). Compared to pre-TVR, VO2% continued to increase 5 years post-TVR (60 ± 16% vs 72 ± 14%, p = 0.002), but was not significantly increased at 1-year post-TVR (69 ± 19%, p = 0.06). For VE/CO2, pre-TVR values (40 ± 15) were increased compared to 1-year post-TVR (33 ± 6, p = 0.02) but without further amelioration after 5 years (33 ± 6). Cardiac index increased (2.4 ± 0.7 vs 2.8 ± 0.5/min/m2, p = 0.02). However, RVEF decreased (52 ± 7 vs 46 ± 9%, p = 0.003) and LVEF remained unchanged (68 ± 8 vs 67 ± 8%, p = 0.3) after TVR. As expected, surgery reduced TR grade (median 3 vs 1.5), RVEDVi (174 ± 51 vs 109 ± 22ml/m2) and RV/LV ratio (2.9 ± 1.1 vs 1.7 ± 0.3, all p < 0.0001). Conclusions TV surgery for Ebstein’s anomaly clearly modulates known risk factors for adverse outcome and was associated with improved subjective and objective exercise tolerance early and mid-term after intervention. This may be due to the concurrent increase in cardiac index demonstrated by CMR and secondary to augmented LV end diastolic volume at a stable LVEF. These findings are suggestive of improved future freedom from MACE. But this needs testing including the degree to which the RVEF decline seen post-TVR mitigates potential benefits beyond symptoms. The improvement in VO2% continued for 5 years which indicates clinical improvement after TVR continues past the first year. Abstract 1162 Figure 1


2014 ◽  
Vol 24 (6) ◽  
pp. 1049-1056 ◽  
Author(s):  
Grace Freire ◽  
Thieu Nguyen ◽  
Priya Sekar ◽  
Marilyn Wilhm ◽  
Kathy Arnold ◽  
...  

AbstractPredicting outcomes of foetuses with Ebstein’s anomaly and tricuspid valve dysplasia continues to be challenging. Limited data exist on the prognostic significance of prenatal haemodynamic and functional parameters in this population. Our aim was to investigate the prognostic significance of haemodynamic and ventricular functional parameters in addition to associated morphometric parameters in patients with Ebstein’s anomaly. We reviewed medical records of foetuses with Ebstein’s anomaly and tricuspid valve dysplasia at All Children’s Hospital Johns Hopkins Medicine and Johns Hopkins University between 2005 and 2012. The main outcome was survival past 30 days from birth; participants who died in utero or <30 days after birth were considered non-survivors. There were 13 survivors and seven non-survivors. We found that participants with abnormal right ventricular function predicted by low tricuspid regurgitation velocity (<2.3 m/second) (p=0.012) and low estimated right ventricular pressure (<24 mmHg) (p=0.029), a low (<7) cardiovascular profile score (p=0.029) and high (>0.53) cardiothoracic ratio (p=0.008) at the first foetal echocardiogram were less likely to survive. In addition, participants with a fossa ovalis/atrial septal length ratio <0.36 at the last foetal echocardiogram (p=0.051) were more likely to die, albeit of borderline statistical significance. Low tricuspid regurgitation velocity and low right ventricular estimated pressure, or a low cardiovascular profile score could be potential prognostic factors for Ebstein’s anomaly and tricuspid valve dysplasia. However, future larger prospective studies are needed to confirm these initial findings.


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