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Abstract

-Background: The application of one and half-ventricular repair in Ebstein anomaly(EA) should be considered, in association with effective cone repair, to reduce the volume preload on a dysfunctional right ventricle, and to take the benefit of obtaining ante-grade pulmonary blood flow. The one and half-ventricular repair that applied in patients with hypoplastic sub pulmonic ventricle can alternate effectively the Fontan pathway. This provides pulsatility in pulmonary circulation. However, pressure elevation in superior vena cava (SVC) and right atrial hypertension can occur in patient’s undergone one and half ventricular repair. Aim The aim of this study to evaluate the short-term outcomes after cone procedure for surgical repair in Ebstein anomaly using one and half-ventricular repair versus biventricular(two ventricular) repair. Objectives: -Evaluation of early mortality after one and half-ventricular repair and, two ventricular repair with cone procedure for Ebstein anomaly surgery in pediatric patients. -Exploration of indications and benefits of one and half-ventricular repair. -Evaluation of short-term adverse events (e.g. bleeding, pleural effusion, chylothorax, reopening arrhythmia and increased superior vena cava pressure) in patients groups. -Methodology: A retrospective cohort study at National Heart Institute, enrolled 52 pediatric patients, divided into two groups: Group 1: Involved 26 patients undergone Cone with one and half ventricular repair for Ebstein anomaly, with age ranging from 9 to 137 months Group 2: Involved 26 patients undergone Cone with biventricular repair for Ebstein anomaly, with age ranging from 10 to 144 months. Manuscript Click here to access/download;Manuscript;Edited2 1.5 paper 17-10-2025.docx 2 Comparing the two groups of regarding the short-term outcomes. This entails the following variables, mortality and adverse outcomes (bleeding, pleural effusion, chylothorax, re-opening, significant tricuspid regurgitation, arrhythmia, low cardiac output syndrome and increased superior vena cava pressure). Inclusion criteriae: -Pediatric patients having Ebstein anomaly with bipartite and tripartite right ventricle. -Carpentier’s Classification type A, B and C . Exclusion criteriae: -Dysmorphic right ventricle -Unipartite right ventricle -Small pulmonary artery - Carpentier’s Classification type D. Sample size was calculated using power of 80%, with assumed incidence of superior vena cava hypertension 10% in cases of one and half ventricular repair in literature , at confidence interval of 95% using epi Info7 sample size calculator. Permission of the local Ethics Research Committee will be obtained. - Outcomes  Primary outcome: Short-term outcomes especially prolonged chest tube drainage (> 5ml/Kg/day).  Secondary outcomes : -Postoperative SVC pressure elevation (defined by mean Cavo-pulmonary shunt pressure elevation by 17 mm Hg, superior vena cava reversal of flow or pulsatility, veno-venous collaterals, or superior vena cava syndrome) -RA hypertension (which was defined as mean right atrial pressures greater than 10 mm Hg with inferior vena cava and hepatic vein flow reversal or dilation). (1) -Bleeding, chylothorax, re-opening, significant TR, arrhythmia, low cardiac output syndrome, intensive care unit stay, and post-surgical mortality. Conclusion: Our study concludes that one and a half ventricular repair shows relatively good shortterm results compared to biventricular repair for pediatric patients with EA. It offers a 3 viable surgical option, especially for patients with a small right ventricle not suitable for biventricular repair, while maintaining pulsatile forward pulmonary blood flow.

Article Type

Original Study

Subject Area

Cardiovascular and Blood Diseases

IRB Number

IHc00105

Creative Commons License

Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License
This work is licensed under a Creative Commons Attribution-NonCommercial-Share Alike 4.0 International License.

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