CARDIOVASCULAR JOURNAL OF AFRICA • Volume 35, No 2, May – August 2024 AFRICA 75 Surgical experience in adults with Ebstein’s anomaly: long-term results Ozge Altas, Sabit Sarikaya Objective: The aim of this study was to review late results of the surgical treatment of Ebstein’s anomaly with reconstruction and replacement in adults. Methods: Medical records of 28 consecutive patients operated on between 1991 and 2014 were reviewed retrospectively. Surgical repair was performed in 19 (67.9%) patients (Hardy: two, Danielson: three, modified Danielson: six, Carpentier: three, Kay annuloplasty reinforced with ring: two), whereas tricuspid valve replacement was performed in nine patients (32.1%). Primary long-term outcomes consisted of right ventricular function, survival and freedom from re-operation. We evaluated the additional impacts of residual tricuspid insufficiency and type of surgery on survival. Results: In-hospital mortality rate was 7.1% (n = 2) due to low cardiac output status and sepsis. Patients showed a significant postoperative decrease in tricuspid regurgitation (p < 0.001), right atrial size (p < 0.001) and pulmonary hypertension (p = 0.002). The mean follow-up time was 140 ± 71.4 months, with a median of 126 months (105–192). Late mortality occurred in two patients and there was no significant difference in terms of survival based on residual tricuspid insufficiency (p = 0.57) and type of surgery (p = 0.094). Overall survival rates were 89.3, 85.4, 85.4 and 68.3% at five, 10, 15 and 20 years, respectively. Conclusion: Although complex leaflet reconstruction techniques have evolved to achieve a more physiological and durable repair, both approaches can be performed safely on specific patients and can be alternated, with acceptable rates of survival and re-operation. Keywords: tricuspid valve, Ebstein anomaly, congenital heart disease Submitted 29/1/23, accepted 16/2/23 Published online 23/3/23 Cardiovasc J Afr 2024; 35: 75–81 www.cvja.co.za DOI: 10.5830/CVJA-2023-008 Having accounted for 1% of congenital heart disease, Ebstein’s anomaly (EA) is associated with abnormalities of the tricuspid valve (TV) leaflets and right ventricle (RV), including atrialisation due to apical displacement of the tricuspid annulus. The surgical strategy for patients with EA is controversial and varies according to anatomical severity, however significant progress has been made since the early 1950s in understanding and managing the disease. Treatment was formerly directed towards valve repair rather than replacement due to unsatisfactory late results of prosthetic valves.1-3 Lately, leaflet reconstruction techniques, such as Carpentier and cone repairs, have become the firstchoice treatment when available, providing good anatomical and physiological results.4,5 Classic approaches emphasise monocuspid coaptation of the TV and cause off-centre diastolic flow, leading to late valvular regurgitation. On the other hand, the modern approach creates near-anatomical repair by re-attaching leaflets clockwise to the true annulus and induces central blood flow.5 However, these types of interventions cannot be offered to all patients. It is not feasible to perform in the presence of pulmonary hypertension (PHT), a severely enlarged RV or when there is lack of septal leaflet tissue. This report summarises our experience with the surgical management of EA using various techniques and assessing postoperative complications, long-term survival and freedom from re-operation. Methods Ethical approval was obtained from our institutional ethics committee. Informed patient consent was waived due to the retrospective nature of the study. FromMarch 1991 toDecember 2014, themedical records of 28 patients undergoing surgery for EA were examined and included in a retrospective study evaluating the long-term follow up of at least five years. Patients having significant tricuspid insufficiency (TI) with symptoms of dyspnoea or right-sided heart failure defined as New York Heart Association (NYHA) class III or IV, progressive cardiomegaly, rhythm disturbances and paradoxical embolism were indicated for surgery.6 Patients were excluded if the surgery was performed by the congenital heart surgery team or if they had congenitally corrected transposition of the great arteries or pulmonary atresia with intact ventricular septum and complex conotruncal abnormalities. Data of the demographic variables, intra-operative process and postoperative outcomes were collected retrospectively. Demographic and clinical data are presented in Table 1. Pulmonary embolism due to a right atrial thrombus was the first finding in one patient. Other patients mostly suffered from decreased exercise capacity, along with dyspnoea (82.1%) and oedema (39.2%). Cardiovascular risk factors were diabetes mellitus in one patient (3.6%), hyperlipidaemia in seven (25%), arterial hypertension in six (21.4%), and smoking was present in three patients (10.7%). Pre-operative cardiac assessment was done with transthoracic echocardiography to diagnose associated pathologies and the data are summarised in Table 2. Previous cardiac operations included partial biventricular repair for pulmonary atresia (n = 1), mitral valve replacement (MVR) and aortic valve replacement (AVR) (n = 1), and open mitral commissurotomy (n Koşuyolu High Specialisation Education and Research Hospital, Istanbul Provincial Health Directorate, Istanbul, Turkey Ozge Altas, MD, dr.ozgealtas@gmail.com Sabit Sarikaya, MD
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