CARDIOVASCULAR JOURNAL OF AFRICA • Volume 35, No 2, May – August 2024 80 AFRICA the improvement in clinical function and survival showed satisfactory results. Late outcomes regarding freedom from re-operation following cone reconstruction are limited, however Holst et al. reported 98.8% at six years in adult patients.17 Moreover, in non-cone repairs, freedom from re-operation at 10 years was reported between 77 and 82%.7,22 Some other small group studies reported the rates between 88.7 and 92.9% at 10 to 20 years of follow up.2,14 Pre-operative echocardiographic assessment is essential to determine the fastening sites of the anterior leaflet. Positioning of the papillary muscles is important for assessment of leaflet mobility. Consequently, it might result in important residual TV regurgitation following surgical repair. McLellan-Tobert et al. reported improved exercise tolerance in adults following TV surgery.12 Although late functional assessments are limited, it is certainly clear that patients showed improvement in NYHA class compared to their pre-operative status (p < 0.001). Likewise, many groups have reported excellent results with non-cone repair.2,14,22 The majority of our patients showed significant decreases in RA size and PHT (p < 0.001 and p = 0.002). We preferred to use ring annuloplasty in two patients with severe RV dysfunction and PHT, in contrast to modern approaches (Carpentier repair, cone reconstruction). Some studies reviewed the use of ring annuloplasty over De Vega’s suture annuloplasty in non-EA patients, and found no significant difference in outcome between the two techniques.24 Our results demonstrate that most patients were able to undergo a satisfactory repair with marked reduction in the degree of TI. Furthermore, a bidirectional cavopulmonary shunt or atrial septal fenestration can be pursued to off-load RV volume in the presence of RV failure and dilatation. PHT and valvular disease of the left side should be evaluated prior to shunting to avoid left ventricular end-diastolic pressure.1 It is stated with cone repair that anatomical restoration of the entire RV induces larger effective stroke volume, causing preferable RV remodelling. Holst and colleagues showed reduction in TI and RV size, as well as an increase in RV fractional area (p < 0.0001).17 However, the mean follow up was much less (3.5 ± 2.5 years) than the two decades of our patients’ follow up. Li et al. found a more synchronised RV movement pattern and decreased functional RV volume by evaluating tricuspid annular movement synchronicity index.23 Recently, MRI has been used to show increased pulmonary flow and decreased functional right ventricular end-diastolic volume, and these studies concluded that there was significant improvement in clinical status.25 The primary limitation of this study was the difficulty in collecting a large number of cases with each procedure, therefore it is hard to draw conclusions. The study consisted of a small group of patients in the period before the emergence of cone repair, therefore we are not able to generalise our study against the clinical outcomes of more recent studies. However, there have been some contra-indicated patients, complicating the results of cone repair during the last decade. Hence, we preferred to use vertical plication of the atrialised RV portion in order to avoid paradoxical motion of the RV. We believe this approach provides clinical benefit in selected patients. Another partial limitation is that MRI has been used recently, allowing for wider analysis of RV function over echocardiography. It is not possible to use MRI for all patients due to lack of availability or patients being followed up by local physicians. Conclusion Although TV function and RV remodelling has been improved with the evolution of many different techniques, the interventions should be focused on more physiological reconstruction for the durability of surgical valve repair. Recent surgical techniques are in contrast to our study, however we have shown satisfactory results obtained with simple approaches. It is questionable to standardise operative techniques against the wide spectrum of anatomical disorders. Further long-term research is required on contemporary interventions for additional assessment. The authors thank Dr Ali Karagoz for his assistance with the statistics used in this report. References 1. Fuchs M, Connolly H. Ebstein anomaly in the adult patient. Cardiol Clinics 2020; 38(3): 353–363. 2. Attenhofer Jost CH, Connolly HM, Scott CG, Burkhart HM, Warnes CA, et al. Outcome of cardiac surgery in patients 50 years of age or older with Ebstein anomaly: survival and functional improvement. J Am Coll Cardiol 2012; 59(23): 2101–2106. 3. Al-Najashi KS, Balint OH, Oechslin E, Williams WG, Silversides CK. Mid-term outcomes in adults with Ebstein anomaly and cavopulmonary shunts. Ann Thorac Surg 2009; 88(1): 131–136. 4. Carpentier A, Chauvaud S, Mac L, Relland J, Mihaileanu S, Marino JP, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988; 96(1): 92–101. 5. Da Silva JP, Baumgratz JF, Fonseca L, Afiune JY, Franchi SM, Lopes LM, et al. Ebstein’s anomaly. Results of the conical reconstruction of the tricuspid valve. Arq Bras Cardiol 2004; 82(3): 217–220. 6. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American heart association task force on clinical practice guidelines. J Am Coll Cardiol 2019; 73(12): e81–192. 0 100 200 300 Time (months) Number at risk 1.0 0.75 0.50 0.25 0.00 Survival probabilities 0 100 200 300 All strata Time (months) 28 21 5 1 Fig. 5. Freedom from re-operation.
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