scholarly journals Reconstructive surgery for Ebstein anomaly: three decades of experience

2019 ◽  
Vol 56 (2) ◽  
pp. 385-392
Author(s):  
Kevin M Veen ◽  
Mostafa M Mokhles ◽  
Jolien W Roos-Hesselink ◽  
Bas R Rebel ◽  
Johanna J M Takkenberg ◽  
...  

AbstractOBJECTIVESSince 1988, our centre employs vertical plication repair with deattachment and reattachment of the tricuspid valve for Ebstein anomaly. This study describes the characteristics and long-term outcomes of our single-centre cohort.METHODSData from all patients operated on between 1988 and 2016 were retrospectively collected. Kaplan–Meier analyses were done for survival data and mixed models were used to analyse longitudinally collected clinical and echocardiography data.RESULTSThirty-six patients (mean age: 25.4 ± 15.9 years, 36% male) were operated on using the Carpentier–Chauvaud 21 (58%) or Cone repair 15 (42%). One patient (3%) died in hospital. Two late deaths were observed, yielding a survival of 97 ± 3% at 25 years. Reoperation was performed in 6 patients after a mean follow-up of 14.1 ± 10.3 years, resulting in a freedom of reoperation of 80 ± 8% at 25 years. During follow-up, predicted probability of being in New York Heart Association III/IV did not exceed 10%. Modelling longitudinal evolution of tricuspid regurgitation showed no major changes over time. Additionally, a rigid ring repair was associated with a higher probability of tricuspid regurgitation, especially after the first years after the operation. A full Cone repair was associated with less progression of tricuspid regurgitation over time.CONCLUSIONSRepair of Ebstein abnomaly is associated with low mortality and morbidity, acceptable reoperation rate and excellent valve function over time, especially in patients with completed Cone repair. Therefore, we conclude that in our centre, repair of Ebstein abnomaly is a durable technique to treat patients.

2016 ◽  
Vol 43 (5) ◽  
pp. 392-396 ◽  
Author(s):  
Gianluca Lucchese ◽  
Lucia Rossetti ◽  
Giuseppe Faggian ◽  
Giovanni B. Luciani

Temporary tricuspid valve detachment improves the operative view of certain congenital ventricular septal defects (VSDs), but its long-term effects on tricuspid valve function are still debated.From 2002 through 2012, we performed a prospective study of 68 children (mean age, 1.28 ± 1.01 yr) who underwent transatrial closure of VSDs following temporary tricuspid valve detachment. Sixty patients had conoventricular and 8 had mid-muscular VSDs. All were in sinus rhythm. Seventeen patients had systemic pulmonary artery pressures. Preoperative echocardiograms showed trivial-to-mild tricuspid regurgitation in 62 patients and tricuspid dysplasia with severe regurgitation in 6 patients. Patients were clinically and echocardiographically monitored at 30 postoperative days, 3 months, 6 months, every 6 months thereafter for the first 2 years, and then once a year.No in-hospital or late death was observed at the median follow-up evaluation of 5.9 years. Mean intensive care unit and hospital stays were 1.6 ± 1.1 and 7.3 ± 2.7 days, respectively. Residual small VSDs occurred in 3 patients, and temporary atrioventricular block in one. After VSD repair, 62 patients (91%) had trivial or mild tricuspid regurgitation, and 6 moderate. Five of these last had severe tricuspid regurgitation preoperatively and had undergone additional tricuspid valve repair during the procedure. The grade of residual tricuspid regurgitation remained stable postoperatively, and no tricuspid stenosis was documented. All patients were in New York Heart Association class I at follow-up.Temporary tricuspid valve detachment is a simple and useful method for a complete visualization of certain VSDs without incurring substantial tricuspid dysfunction.


2019 ◽  
Vol 46 (2) ◽  
pp. 100-106
Author(s):  
Salih Salihi ◽  
H. Tarik Kiziltan ◽  
Ahmad Huraibat ◽  
Askin Ali Korkmaz ◽  
Ibrahim Kara ◽  
...  

Various techniques for treating tricuspid regurgitation have been described; however, because of scarce data about the long-term outcomes of different repairs, the optimal technique has not been established. We evaluated the effectiveness and durability of artificial neochordae implantation in the treatment of tricuspid regurgitation. From 2009 through 2014, 507 patients underwent tricuspid valve repair at our institution. Of those, 48 patients implanted with artificial neochordae were included in our study. The median age of the participants was 62 years (range, 4–77 yr) and 50% were women. Thirty patients (63%) were in New York Heart Association functional class III, and 11 (23%) were in class II. The cause of tricuspid regurgitation was functional in 33 patients (69%) and rheumatic in 15 (31%). In 46 patients, neochordae implantation was performed in addition to Kay annuloplasty (n=13) or ring annuloplasty (n=33). Forty-two patients were discharged from the hospital with absent or mild tricuspid regurgitation. The mean follow-up period was 44.3 ± 20.2 months. Follow-up echocardiograms revealed that tricuspid regurgitation was absent, minimal, or mild in 38 patients (80.8%), moderate in 7, and severe in 2. Our results indicate that the use of artificial neochordae implantation as an adjunct procedure to annuloplasty leads to effective and durable repair in comparison with conventional techniques for treating tricuspid regurgitation.


Circulation ◽  
2005 ◽  
Vol 112 (9_supplement) ◽  
Author(s):  
Jos J.M. Westenberg ◽  
Rob J. van der Geest ◽  
Hildo J. Lamb ◽  
Michel I.M. Versteegh ◽  
Jerry Braun ◽  
...  

Background— Data on reverse remodeling of the left atrium (LA) and left ventricle (LV) after restrictive annuloplasty in patients with dilated cardiomyopathy are scarce, and follow-up studies are performed with echocardiography. Methods and Results— Twenty patients with dilated cardiomyopathy and severe mitral regurgitation selected for restrictive mitral annuloplasty underwent serial MRI studies (within 1 week before surgery, and 2 months [n =18] and 1 year [n =13] after surgery). Early mortality was 10%; all patients were free from endocarditis and thromboembolism. New York Heart Association class improved from 3.2±0.4 to 1.2±0.9. Only 1 patient developed recurrent severe mitral regurgitation during follow-up and it was re-repaired. LA end-systolic volumes decreased significantly over time (from 165±48 mL to 109±23 mL to 111±28 mL; P <0.01), as did LA end-diastolic volumes (from 92±32 mL to 71±22 mL to 75±17 mL; P =0.01). LV end-diastolic volumes decreased significantly (from 244±56 mL to 184±54 mL to 195±67 mL; P <0.01), whereas end-systolic volumes did not change significantly. LV ejection fraction increased significantly (from 35±8% to 46±13% to 46±15%; P <0.01) and LV mass decreased significantly (from 150±43 grams to 132±39 grams to 136±33 grams; P =0.02). Conclusion— Restrictive annuloplasty in patients with dilated cardiomyopathy yielded excellent clinical results associated with significant LA and LV reverse remodeling over time as demonstrated by MRI.


Author(s):  
Martin Geyer ◽  
Karsten Keller ◽  
Kevin Bachmann ◽  
Sonja Born ◽  
Alexander R. Tamm ◽  
...  

Abstract Background Concomitant tricuspid regurgitation (TR) is a common finding in mitral regurgitation (MR). Transcatheter repair (TMVR) is a favorable treatment option in patients at elevated surgical risk. To date, evidence on long-term prognosis and the prognostic impact of TR after TMVR is limited. Methods Long-term survival data of patients undergoing isolated edge-to-edge repair from June 2010 to March 2018 (combinations with other forms of TMVR or tricuspid valve therapy excluded) were analyzed in a retrospective monocentric study. TR severity was categorized and the impact of TR on survival was analysed. Results Overall, 606 patients [46.5% female, 56.4% functional MR (FMR)] were enrolled in this study. TR at baseline was categorized severe/medium/mild/no or trace in 23.2/34.3/36.3/6.3% of the cases. At 30-day follow-up, improvement of at least one TR-grade was documented in 34.9%. Severe TR at baseline was identified as predictor of 1-year survival [65.2% vs. 77.0%, p = 0.030; HR for death 1.68 (95% CI 1.12–2.54), p = 0.013] and in FMR-patients also regarding long-term prognosis [adjusted HR for long-term mortality 1.57 (95% CI 1.00–2.45), p = 0.049]. Missing post-interventional reduction of TR severity was predictive for poor prognosis, especially in the FMR-subgroup [1-year survival: 92.9% vs. 78.3%, p = 0.025; HR for death at 1-year follow-up 3.31 (95% CI 1.15–9.58), p = 0.027]. While BNP levels decreased in both subgroups, TR reduction was associated with improved symptomatic benefit (NYHA-class-reduction 78.6 vs. 65.9%, p = 0.021). Conclusion In this large study, both, severe TR at baseline as well as missing secondary reduction were predictive for impaired long-term prognosis, especially in patients with FMR etiology. TR reduction was associated with increased symptomatic benefit. Graphic abstract


2020 ◽  
Author(s):  
Qiang Ji ◽  
YuLin Wang ◽  
Ye Yang ◽  
Hao Lai ◽  
WenJun Ding ◽  
...  

Abstract Background: Septal myectomy has been a standard treatment option for patients with hypertrophic obstructive cardiomyopathy (HOCM) and drug refractory symptoms. However, there are only a few experienced myectomy centers in the world so far, mainly because of high technical difficulty of myectomy. From our clinical experience, the use of the mini-invasive surgical instruments during myectomy may be beneficial to reduce the technical difficulty. This study reports the preliminary experience regarding transaortic septal myectomy using mini-invasive surgical instruments for the treatment of patients with HOCM and drug refractory symptoms, and evaluates the early results following myectomy.Methods Between March 2016 and March 2019, consecutive HOCM patients were included in this analysis who underwent isolated transaortic septal myectomy using the mini-invasive surgical instruments. Intraoperative, in-hospital and follow-up results were analyzed.Results A total of 168 eligible patients (83 males, mean 56.8 ± 12.3 years) were included. Midventricular obstruction was recorded in 7 (4.2%) patients. All included patients underwent transaortic septal myectomy with a mean aortic cross-clamping time of 36.0 ± 8.1 minutes. Nine (5.4%) patients received repeat aortic cross-clamping during surgery. Surgical mortality was 0.6%. Five (3.0%) patients developed complete atrioventricular block and required permanent pacemaker implantation. The median follow-up time was 6 months. No follow-up deaths occurred with a significant improvement in New York Heart Association functional status. The maximum gradients decreased sharply from the preoperative value (11.6 ± 7.4 mmHg vs. 94.4 ± 2 2.6 mmHg, p<0.001). The median degree of mitral regurgitation fell to 1.0 (vs. 3.0 preoperatively, p<0.001) with a significant reduction in the proportion of moderate or more regurgitation (1.2% vs. 57.7%, p<0.001).Conclusions The use of the mini-invasive surgical instruments may be beneficial to reduce the technical difficulty of transaortic septal myectomy procedure. Transaortic septal myectomy using the mini-invasive surgical instruments may be associated with favorable results.


Author(s):  
Peter Kubuš ◽  
Jana Rubáčková Popelová ◽  
Jan Kovanda ◽  
Kamil Sedláček ◽  
Jan Janoušek

Background Cardiac resynchronization therapy (CRT) is rarely used in patients with congenital heart disease, and reported follow‐up is short. We sought to evaluate long‐term impact of CRT in a single‐center cohort of patients with congenital heart disease. Methods and Results Thirty‐two consecutive patients with structural congenital heart disease (N=30) or congenital atrioventricular block (N=2), aged median of 12.9 years at CRT with pacing capability device implantation, were followed up for a median of 8.7 years. CRT response was defined as an increase in systemic ventricular ejection fraction or fractional area of change by >10 units and improved or unchanged New York Heart Association class. Freedom from cardiovascular death, heart failure hospitalization, or new transplant listing was 92.6% and 83.2% at 5 and 10 years, respectively. Freedom from CRT complications, leading to surgical system revision (elective generator replacement excluded) or therapy termination, was 82.7% and 72.2% at 5 and 10 years, respectively. The overall probability of an uneventful therapy continuation was 76.3% and 58.8% at 5 and 10 years, respectively. There was a significant increase in ejection fraction/fractional area of change ( P <0.001) mainly attributable to patients with systemic left ventricle ( P =0.002) and decrease in systemic ventricular end‐diastolic dimensions ( P <0.05) after CRT. New York Heart Association functional class improved from a median 2.0 to 1.25 ( P <0.001). Long‐term CRT response was present in 54.8% of patients at last follow‐up and was more frequent in systemic left ventricle ( P <0.001). Conclusions CRT in patients with congenital heart disease was associated with acceptable survival and long‐term response in ≈50% of patients. Probability of an uneventful CRT continuation was modest.


Circulation ◽  
2000 ◽  
Vol 102 (suppl_3) ◽  
Author(s):  
Jian Xin Qin ◽  
Takahiro Shiota ◽  
Patrick M. McCarthy ◽  
Michael S. Firstenberg ◽  
Neil L. Greenberg ◽  
...  

Background —Infarct exclusion (IE) surgery, a technique of left ventricular (LV) reconstruction for dyskinetic or akinetic LV segments in patients with ischemic cardiomyopathy, requires accurate volume quantification to determine the impact of surgery due to complicated geometric changes. Methods and Results —Thirty patients who underwent IE (mean age 61±8 years, 73% men) had epicardial real-time 3-dimensional echocardiographic (RT3DE) studies performed before and after IE. RT3DE follow-up was performed transthoracically 42±67 days after surgery in 22 patients. Repeated measures ANOVA was used to compare the values before and after IE surgery and at follow-up. Significant decreases in LV end-diastolic (EDVI) and end-systolic (ESVI) volume indices were apparent immediately after IE and in follow-up (EDVI 99±40, 67±26, and 71±31 mL/m 2 , respectively; ESVI 72±37, 40±21, and 42±22 mL/m 2 , respectively; P <0.05). LV ejection fraction increased significantly and remained higher (0.29±0.11, 0.43±0.13, and 0.42±0.09, respectively, P <0.05). Forward stroke volume in 16 patients with preoperative mitral regurgitation significantly improved after IE and in follow-up (22±12, 53±24, and 58±21 mL, respectively, P <0.005). New York Heart Association functional class at an average 285±144 days of clinical follow-up significantly improved from 3.0±0.8 to 1.8±0.8 ( P <0.0001). Smaller end-diastolic and end-systolic volumes measured with RT3DE immediately after IE were closely related to improvement in New York Heart Association functional class at clinical follow-up (Spearman’s ρ=0.58 and 0.60, respectively). Conclusions —RT3DE can be used to quantitatively assess changes in LV volume and function after complicated LV reconstruction. Decreased LV volume and increased ejection fraction imply a reduction in LV wall stress after IE surgery and are predictive of symptomatic improvement.


Circulation ◽  
2020 ◽  
Vol 141 (Suppl_1) ◽  
Author(s):  
James E Peterman ◽  
Matthew Harber ◽  
Mary Imboden ◽  
Mitchell Whaley ◽  
bradley fleenor ◽  
...  

Introduction: Mortality risk predictions are improved with routine assessment of cardiorespiratory fitness (CRF). Accordingly, an American Heart Association Scientific Statement suggests routine clinical assessment of CRF in apparently healthy adults minimally using non-exercise prediction equations, which can be calculated from common health metrics. However, no study has assessed the ability of non-exercise CRF prediction equations to accurately detect longitudinal changes. Hypothesis: Changes in estimated CRF (eCRF) would be related to directly-measured changes, yet appreciable misclassification would occur at the individual level. Methods: The sample included 987 apparently healthy adults (324 females; mean±SD age 43.1±10.4 years) who completed 2 cardiopulmonary exercise tests (CPX) at least 3 months apart (3.2±5.4 years follow-up). The change in eCRF from 27 distinct non-exercise prediction equations was compared to the change in directly-measured CRF determined from CPX. A change of ≥5% was used to classify participants as having a directional increase or decrease in CRF. Analysis included Pearson product moment correlations, standard error of estimate (SEE) values, the Benjamini-Hochberg procedure to compare eCRF with directly-measured CRF, and chi-squared tests to examine the impact of follow-up time on the percentage of participants correctly identified as having a directional increase or decrease in CRF. Results: The change in eCRF from each equation was correlated to the change in directly-measured CRF ( P <0.001) with R 2 values ranging from 0.06-0.43 and SEE values ranging from 0.9-5.9 ml·kg -1 ·min -1 . For 16 of the 27 equations, the change in eCRF was significantly different from the change in directly-measured CRF. When classifying directional changes, the prediction equations correctly categorized an average of 54% of individuals as having increased, decreased, or no change in CRF. When examining the influence of follow-up time, the average percentage of individuals correctly classified as having a directional increase in CRF was greater when the time between tests was ≤8months (54%) compared to ≥2years (28%). In contrast, the average percentage correctly classified as having a directional decrease in CRF was lower with tests ≤8months apart (8%) compared to ≥2years (73%). Conclusions: As hypothesized, discernible variability was found in the accuracy between non-exercise prediction equations and the ability of equations to accurately assess changes in directly-measured CRF over time. Considering the appreciable error that prediction equations had with detecting even directional changes in CRF, these results suggest eCRF may have limited clinical utility.


2014 ◽  
Vol 8 ◽  
pp. CMC.S14016 ◽  
Author(s):  
Carlo Lombardi ◽  
Valentina Carubelli ◽  
Valentina Lazzarini ◽  
Enrico Vizzardi ◽  
Filippo Quinzani ◽  
...  

Amino acids (AAs) availability is reduced in patients with heart failure (HF) leading to abnormalities in cardiac and skeletal muscle metabolism, and eventually to a reduction in functional capacity and quality of life. In this study, we investigate the effects of oral supplementation with essential and semi-essential AAs for three months in patients with stable chronic HF. The primary endpoints were the effects of AA's supplementation on exercise tolerance (evaluated by cardiopulmonary stress test and six minutes walking test (6MWT)), whether the secondary endpoints were change in quality of life (evaluated by Minnesota Living with Heart Failure Questionnaire—MLHFQJ and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. We enrolled 13 patients with chronic stable HF on optimal therapy, symptomatic in New York Heart Association (NYHA) class II/III, with an ejection fraction (EF) <45%. The mean age was 59 ± 14 years, and 11 (84.6%) patients were male. After three months, peak VO2 (baseline 14.8 ± 3.9 mL/minute/kg vs follow-up 16.8 ± 5.1 mL/minute/kg; P = 0.008) and VO2 at anaerobic threshold improved significantly (baseline 9.0 ± 3.8 mL/minute/kg vs follow-up 12.4 ± 3.9 mL/minute/kg; P = 0.002), as the 6MWT distance (baseline 439.1 ± 64.3 m vs follow-up 474.2 ± 89.0 m; P = 0.006). However, the quality of life did not change significantly (baseline 21 ± 14 vs follow-up 25 ± 13; P = 0.321). A non-significant trend in the reduction of NT-proBNP levels was observed (baseline 1502 ± 1900 ng/L vs follow-up 1040 ± 1345 ng/L; P = 0.052). AAs treatment resulted safe and was well tolerated by all patients. In our study, AAs supplementation in patients with chronic HF improved exercise tolerance but did not change quality of life.


2021 ◽  
Author(s):  
Linyun Xi ◽  
Chun Wu ◽  
Zhengxia Pan ◽  
Ming Xang

Abstract BackgroundBy reviewing the outcomes of four patients, we summarize our experience with the strategy of using a titanium plate to reconstruct the inferior sternal cleft in pentalogy of Cantrell (POC).MethodsThis was a retrospective analysis of 4 patients who visited our department between January 2000 and June 2020 concurrent with POC. All four patients underwent an operation, as well as cardiac ultrasound and thoracoabdominal computed tomography (CT). Cardiac malformations achieved satisfactory correction according to echocardiographs. A titanium plate was used to repair the sternal and supraumbilical abdominal defects. The hospital course, operative data, and outpatient records were reviewed.ResultsAll 4 patients had partial sternal clefts, and 4 patients underwent a single-stage operation. All 4 cases of ectopia cordis were eventually repositioned. The defect in the sternum and supraumbilical abdomen was repaired using a titanium plate. One patient with double-outlet right ventricle (DORV) developed low cardiac output syndrome and pulmonary infection, and symptomatic treatment was administered until discharge. The mean follow-up time ranged from 9 months to 10 years. No patient developed pectus excavatum, and there were no cases of retrosternal seroma or pneumothorax. The titanium plate was migratory in the second patient and was dislodged at another hospital 3.5 years postoperation, and a fibreboard was formed in the area where sternal cleft; the beating heart could not be observed outside the thoracoabdominal or thoracic wall. In the first patient, the titanium plate was torn with a small fissure at 2 years postoperation, but the fissure was not enlarged during follow-up. In the other two patients, the appearance of both the abdominal wall and lower sternum and cardiac function were good. The New York Heart Association function class was I in all four patients.ConclusionThe use of a titanium plate to construct the neosternum can yield a satisfactory exterior appearance of the thorax with a partial sternal cleft, but long-term outcomes need to be examined further.


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