Rare Congenital Heart Disease Research Articles

Insight into the research history and trends of total anomalous pulmonary venous connection: a bibliometric analysis

BackgroundTotal anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease characterized by the inability of all pulmonary veins to connect to the left atrium. Our previous bibliometric article summarized the characteristics of only the 100 most cited papers in TAPVC research. The purpose of this study was to use comprehensive bibliometric analysis to examine the development history, current status, and future trends in the field of TAPVC.MethodsAll publications on TAPVC published between 2000 and 2023 were collected from the Web of Science Core Collection. The publication and citation data were quantitatively analyzed by publication year, country, institution, author, and journal. Co-authorship and co-occurrence analyses were performed using VOSviewer, and keyword and reference bursts were identified using CiteSpace. Pearson’s test was used to examine the correlations between two continuous variables.ResultsAs of July 20, 2023, we identified 368 publications with 3320 citations. These publications were published in 132 journals and authored by 1835 researchers from 457 institutions in 47 countries. For the number of publications, the top country, top institution, top author, and top journals were the United States (n = 82), Shanghai Jiao Tong University (n = 13), Huiwen Chen (n = 9), and Annals of Thoracic Surgery and Pediatric Cardiology (n = 29 each), respectively. For the number of citations, the top country, top affiliation, top author, and top journal were the United States (n = 1348), University of Toronto (n = 250), Christopher A. Caldarone (n = 315), and Annals of Thoracic Surgery (n = 746), respectively. The number of national publications significantly correlated with GDP (R = 0.887, P < 0.001), research & development (R&D) expenditure (R = 0.375, P = 0.013), population (R = 0.694, P < 0.001), and journals (R = 0.751, P < 0.001). The number of national citations significantly correlated with GDP (R = 0.881, P < 0.001), R&D expenditure (R = 0.446, P = 0.003), population (R = 0.305, P = 0.037), and journals (R = 0.917, P < 0.001). International collaboration in the field of TAPVC was not well developed. The most commonly cited publication discussed era changes in mortality and reoperation rate in TAPVC patients. The most common keywords were “total anomalous pulmonary venous connection” and “congenital heart disease”. The keyword “case report” appeared most recently, with an average occurrence year of 2021.8. The co-occurrence analysis grouped 26 keywords into six themes: surgical repair of TAPVC, postoperative pulmonary vein stenosis, surgical repair of TAPVC patients with heterotaxy, application of echocardiography in diagnosing TAPVC, application of echocardiography in the prenatal diagnosis of TAPVC, and application of the sutureless technique in the surgical repair of TAPVC patients with right atrial isomerism or a single ventricle. Citation burst detection identified 32 references with citation bursts, seven of which had ongoing citation bursts until 2023.ConclusionsThis study conducted a bibliometric analysis to provide a comprehensive overview of TAPVC research. We hope to offer new ideas for promoting development in the field of TAPVC.

Isolated left ventricular non-compaction cardiomyopathy: case report and brief review on diagnostic imaging methods

Left ventricular non-compaction cardiomyopathy (LVNC) is a rare congenital heart disease with an estimated incidence of 0.014-0.045% in a general population. It occurs due to a morphogenetic abnormality during embryogenesis that inhibits myocardial compaction and determines prominent trabeculae with deep intertrabecular spaces and thickening of the adjacent myocardium in two distinct layers. Diagnosis is generally established via transthoracic echocardiogram (TTE) with further confirmation with cardiac magnetic resonance (CMR). A 57-year-old male patient previously diagnosed with systemic arterial hypertension and chronic obstructive pulmonary disease reported chest pain and dyspnea upon exertion, headache, and dizziness. During outpatient investigation, the patient was submitted to a stress electrocardiogram that was positive for ischemia and led to a cineangiocoronariography with no signs of coronary artery obstruction. At the echocardiography lab, several trabeculations were detected in the apical region of the left ventricle. A diagnosis of LVNC was established and the results were confirmed via CMR. Management was initiated with the goal of symptom relief. LVNC is a relatively unknown pathology that is frequently underdiagnosed at earlier stages. As presented, we highlight the importance of multimodality imaging, especially TTE and CMR, in the diagnosis of this congenital heart condition.

A rare case of congenital heart disease: common pulmonary vein atresia

Common pulmonary vein atresia is a rare congenital heart disease, in which the venous return from the lungs flows through the four pulmonary veins into the cavity behind them without connection to the heart chambers. The article presents a clinical case of a newborn child with this malformation. Computed tomography showed a lack of pulmonary vein drainage in a typical location as well as the presence of a common venous collector. On the basis of the data obtained we made a correct diagnosis.

A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly.

Background: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is considered a risk factor for sudden cardiac death in this population. Still, there are very few real-life data on the impact of SVA treated invasively or conservatively on a patient's prognosis. We aimed to analyze the incidence of SVA in adults with EA, evaluate the effectiveness of catheter ablation, and analyze the impact of SVA and catheter ablation on survival in this population. Methods and results: 71 pts (median age 53 years; range 24-84 years) with EA were evaluated retrospectively from 1988 to 2020. Forty patients (56.3%) had SVA, and eighteen of them (45.0%) required at least one catheter ablation (35 procedures in total). Indications for ablation were mostly intra-atrial reentrant tachycardia (IART) and atrioventricular reentrant tachycardia (AVRT) (14 pts [77.8% and 9 pts [50.0%], respectively. IART and AVRT coexisted in nine pts. One patient suffered from persistent atrial fibrillation. Procedural effectiveness was reported in 28 (80%) cases; over a longer follow-up (mean 12.6 ± 5.4 years), only eight (44.4%) patients were completely free from SVA after the first ablation. In total, 10 patients (14%) died due to cardiovascular events. There was no difference in survival between patients with or without SVA (p = 0.9) and between ablated and non-ablated EA individuals (p = 0.89). Conclusions: Supraventricular arrhythmia is frequent in adults with Ebstein anomaly. Patients often require more than one catheter ablation but eventually become free from arrhythmias. The imaging parameters assessed by echocardiography or cardiac magnetic resonance do not seem to be associated with ablation outcomes. The impact of supraventricular arrhythmia itself or treatment with radiofrequency ablation is questionable and should be thoroughly investigated in this population.

Epidemiological Survey of Congenital Heart Disease Among Children Aged from 2 to 18 in Suo County, Nagqu, Tibet.

Lin, Tian, Huaping Jia, Yunming Li, Yongxing Xu, Bei Zhao, Dong Zheng, Hongfeng Yan, Meihui Zhao, Yanlei Li, Liping Xia, Fengxia Zhou, Cuiping Liu, Ke Ma, Ma Mi, and Jianwen Gu. Epidemiological survey of congenital heart disease among children aged from 2 to 18 in Suo County, Nagqu, Tibet. High Alt Med Biol. 00:000-000, 2024. Background: Studies have reported the prevalence of congenital heart disease (CHD) in parts of Tibet, but relative epidemiological surveys are rare. We aimed to explore the prevalence of CHD in children and its relationship with family history in Suo County, Nagqu, Tibet, an altitude of 3,980 meters. Methods: We recruited 4,002 children aged 2-18 years. Subjects underwent a family history investigation, cardiac auscultation, and clinical manifestation examination and then received echocardiographic screening. Results: The prevalence of CHD among children in Suo County was 0.97% (39 cases), much higher than the prevalence at sea level. The most common subtype was atrial septal defect, accounting for 53.9% of CHD, followed by patent ductus arteriosus (33.3%) and ventricular septal defect (12.8%). We also found that children whose mothers had previously borne children with CHD had a higher risk of CHD than those without (p = 0.002); other factors related to CHD during pregnancy, such as smoking, drinking, drug use, and viral infection, showed no statistical differences between children with and without CHD. Conclusions: The prevalence of CHD in children in Suo County is much higher than at low altitude, consisting mostly of simple forms with left-to-right shunt, with rare complex CHD. These results support implementing diagnostic and treatment plans to prevent CHD in Suo County.

Ebstein's Disease: About Two Cases Discovered Following a Rhythm Disorder

Ebstein's disease is a rare congenital heart disease. It is characterized by the low insertion of one or two tricuspid leaflets leading to atrialization of the right ventricle. The clinical forms vary, ranging from neonatal anasarca to right heart failure and arrhythmia in adolescents and adults. It can remain asymptomatic for a long time, and the discovery is fortuitous following a preoperative assessment for extracardiac surgery or other reasons. We report two clinical cases of Ebstein's disease, different in their circumstances of discovery: in the first case, the disease was discovered following an assessment of right bundle branch block with late left extrasystoles in a 20-year-old patient, and the second case, during an assessment of palpitations in a 13-year-old patient. Through these cases, we will review the circumstances of discovery of the disease by recalling the different types of cardiographic echo and therapeutic management.

A rare case of a neonate with fallot-type absent pulmonary valve and occlusion of the left main bronchus

Fallot-type absent pulmonary valve is a rare and complex congenital heart disease. Repair surgery for this condition during the neonatal period has a mortality rate of over 50%. We reported a neonate with Fallot-type absent pulmonary valve and occlusion of the left main bronchus. The patient’s pulmonary artery had unusual anatomy of a type that has not previously been reported. This case report outlines a successful treatment strategy for patients with complex congenital heart disease and airway occlusion during the neonatal period and the effect of these unusual anatomical conditions on postoperative outcomes.

Video assisted thorascopic assisted correction of left partial anomalous pulmonary venous connection: one case report

IntroductionThe left partial anomalous pulmonary vein connection is a rare congenital heart disease, especially with intact atrial septum. Now we reported a case of the left superior pulmonary vein drainage to left innominate vein through a vertical vein, and corrected with video assisted thoracoscopy.Case presentationA-59-years old man diagnosed left anomalous partial pulmonary vein connection with presentation of short breathiness and palpation, and diagnosed with computer tomography pulmonary angiography. The operation was carried out under video assisted thoracoscopy with one manipulation incision and one observational incision, the vertical vein was dissected and anastomosis with left atrial appendage. The patients recovered smoothly and postoperative CTPA showed anastomosis ostium was unobstructed.ConclusionThe left lateral thoracotomy and video assisted thoracoscopic surgery is a feasible for correction of left PAPVC with intact interatrial septum without using CPB.

Hemodynamics, anatomy, and outcomes of quadricuspid aortic valves: Multimodality imaging assessment

BackgroundQuadricuspid aortic valve (QAV) is a rare congenital heart disease with a limited body of literature. This retrospective cohort study investigates QAV morphology, function, and clinical outcomes. MethodsEchocardiography was used to assess valvular function. Morphological characteristics such as phenotypes, raphe, regurgitant orifice area (ROA), and aortic dilation (diameter >40 ​mm) were assessed by cardiac CT. Patients were followed up for the combined event of all-cause death and aortic valve replacement (AVR). ResultsNinety QAV patients (screened from 322385 CT scans) were included (mean age 55.2 ​± ​13.6 years, 61.1 ​% male). Isolated significant aortic regurgitation (AR) was present in 75.6 ​% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 ​%) and type II (3 larger and 1 smaller leaflets, 42.2 ​%) QAV. Fused raphe was present in 26.7 ​% of patients. ROACT was correlated with AR severity and aortic dilation (41.1 ​%, n ​= ​37). Among patients without AVR at baseline (n ​= ​60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3–62.8). ROACT was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 ​mm2, HR ​= ​4.25, 95%CI 1.49–12.17, p ​= ​0.007; as a continuous variable (per mm2 increment), HR ​= ​1.04, 95%CI 1.01–1.07, p ​= ​0.003). Additionally, ROACT had incremental prognostic value when added to the AR severity model (area under the receiver-operating characteristic curve increased from 86.8 to 88.4, p ​= ​0.004). ConclusionQAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROACT may be a potential ancillary prognostic marker in patients with QAV.

Clinical Case of Cor Triatriatum

Cor Triatriatum is utterly rare congenital heart disease, which has characteristic magnetic resonance and echocardiographic symptoms. Complexity of the diagnostics is an asymptomatic clinical flow, so timely diagnostics and therapy will help to defer possible complications and improve the quality of the life of these patients.

Interobserver variability in assessing treatment response of hepatocellular carcinoma to transarterial chemoembolization: A comparative study of LIRADS TR and mRECIST criteria in South America

Hepatocarcinoma (HCC) is a major cause of worldwide morbidity and mortality. Transarterial chemoebolization (TACE) is a frequent management strategy in cases of unresectability. The imaging response assessment to treatment is complex and there are different strategies that are not universal and whose performance has not been compared.

Bridge to Surgery with Pulmonary Valve Perforation and Right Ventricular Outflow Tract Stenting in an Infant with PA-VSD

Pulmonary atresia (PA) with ventricular septal defect (VSD) is a rare congenital heart disease. Blood flow to pulmonary arteries is provided by patent ductus arteriosus or major aorto-pulmonary collateral arteries (MAPCAs). Palliative interventions are required to maintain the blood supply to the pulmonary arteries in order to achieve for the complete surgical repair. In this case report, we present a patient who underwent pulmonary valve perforation and right ventricular outflow tract (RVOT) stenting as an alternative to a systemic-pulmonary artery shunt (SPS). Subsequently, surgical repair was performed ten months later.

Usefulness of three vessel‐trachea view and parasagittal plan for prenatal diagnosis of interrupted aortic arch

AbstractBackgroundInterrupted aortic arch (IAA) is a rare congenital heart disease characterized by loss of continuity between the ascending and the descending aorta. Prenatal diagnosis of IAA by echocardiography is challenging but nonetheless can be accomplished via a systematization of cardiac fetal evaluation.Case PresentationWe report a case of fetal IAA type A prenatally diagnosed through two‐dimensional echocardiography using both a three vessel‐trachea view and a sagittal view.ConclusionPrenatal counseling regarding the diagnosis and prognosis about this anomaly is still challenging nowadays due to associated anomalies/chromosomal abnormalities who may impact the prognosis. Fetal autopsy in all cases of pregnancy termination after abnormal ultrasound findings is important in order to make a full diagnosis and characterize the anomaly.

Rare loss-of-function variants in matrisome genes are enriched in Ebstein’s anomaly

Ebstein's anomaly, a rare congenital heart disease, is distinguished by the failure of embryological delamination of the tricuspid valve leaflets from the underlying primitive right ventricle myocardium. Gaining insight into the genetic basis of Ebstein's anomaly allows a more precise definition of its pathogenesis. In this study, two distinct cohorts from the Chinese Han population were included: a case-control cohort consisting of 82 unrelated cases and 125 controls without cardiac phenotypes and a trio cohort comprising 36 parent-offspring trios. Whole-exome sequencing data from all 315 participants were utilized to identify qualifying variants, encompassing rare (minor allele frequency<0.1% from East Asians in the gnomAD database) functional variants and high-confidence (HC) loss-of-function (LoF) variants. Various statistical models, including burden tests and variance-component models, were employed to identify rare variants, genes, and biological pathways associated with Ebstein's anomaly. Significant associations were noted between Ebstein's anomaly and rare HC LoF variants found in genes related to the matrisome, a collection of extracellular matrix (ECM) components. Specifically, 47 genes with HC LoF variants were exclusively or predominantly identified in cases, while nine genes showed such variants in the probands. Over half of unrelated cases (n= 42) and approximately one-third of probands (n= 12) were found to carry one or two LoF variants in these prioritized genes. These results highlight the role of the matrisome in the pathogenesis of Ebstein's anomaly, contributing to a better understanding of the genetic architecture underlying this condition. Our findings hold the potential to impact the genetic diagnosis and treatment approaches for Ebstein's anomaly.

Ebsteins anomaly management in Central and South Eastern European (CESEE) countries: current status and improvement needed

Abstract Background Ebstein anomaly (EA) of the tricuspid valve (TV) as a rare congenital heart disease (CHD) is characterized by variable morphology, hemodynamics and clinical course, associated heart defects as well. Moreover, whether and when to intervene on TV remains a big challenge. Patients with EA should therefore be managed in expert centres equipped with all diagnostic and treatment tools according to ESC guidelines (ESC-GL), including cardiac magnetic resonance (CMR) as the gold standard. Aim of the study The aim of the study was to characterize EA patient population from selected countries within Central-Southern-Eastern Europe (CESEE), evaluate the management in relation to ESC-GL and determine optimal parameters for long-term follow-up (FU) feasible in CESEE. Material and methods In retrospective cross-sectional study from 9 CESEE countries, 203 adult patients with EA were included: median age 39y, 58% women. Determined were age at diagnosis, EA complications (associated CHD, arrythmias), cardiosurgery. ECHO and CMR right ventricular (RV) characteristics, NTproBNP, 6-minute walking distance (6MWD) were analysed as follows: ECHO parameters: long-axis RV end-diastolic dimension (RVEDD,mm), long-axis end-diastolic right to left ventricle diameter ratio (RV/LV), tricuspid annular plane systolic excursion (TAPSE,mm), RV fractional area change (FAC,%), RV tissue Doppler myocardial velocity (m/s). CMR-derived analysed parameters were: ejection fraction (EF,%), indexed end-diastolic volume (EDVi,ml/m²). Results Patients were diagnosed at age &amp;lt;1y/1-18y/&amp;gt;18y in: 29,6%/31.1%/39.3%. NYHA I/II/III/IV was as follows: 41.6%/37%/22.5%/0%, median NTproBNP: 160ng/l, 6MWD: 473m. CMR-characteristics were: median RVEF: 43.2%, EDVi: 118ml/m². CMR was absent in 60% cases. Associated CHD/arrhythmias were present at 46,7%/64,4. TV surgery were indicated / underwent 30,7% patients at mean age 32y. Performed was daSilva cone-repair/other valvuloplasty/valve replacement in 36,4%/25,5%/38,2%. CMR-derived EDV correlates significantly (P&amp;lt;0.001) with ECHO-based RVEDD and RV/LV ratio. No correlations between CMR-derived EF and ECHO functional parameters were found. Conclusion Although patients with EA in CESSE region are diagnosed late, the characteristics correspond to data published so far. Despite large heterogeneity including various associated complications, patient´s care in the CESEE region does not fully meet the requirements of ESC-GL. Mainly, CMR as a golden standard for EA evaluation is not widely accessible and so not performed. Therefore, an inconsistency in management and differences in therapeutic approach is a consequence. In these conditions in view of our previously proven experience, that the RV/LV ratio correlates significantly with CMR volume parameters, ECHO could serve as a reliable and decisive imaging method in FU, however insufficient in relation to ESC-GL.

Abstract 16255: Unraveling the Complex Interplay: Atrial Flutter in a Patient With Prior Double Outlet Right Ventricular Repair

Introduction: Double Outlet Right Ventricle (DORV) is a rare complex congenital heart disease (CHD) in which both the great arteries arise predominantly or entirely from the right ventricle. It is one of the rare CHD lesions with a prevalence of about 0.9%. These patients have complex anatomy, which increases their risk of recurrent arrhythmias. We present a challenging case of Atrial Flutter (AFL) in a patient with DORV. Case: A 31-year-old male with a history of DORV repair presented with palpitations and chest pain for 2 days. His heart rate (HR) was 240 bpm. EKG showed AFL with variable block [Figure 1]. Labs were unremarkable. The patient remained in AFL with HR above 120 bpm despite receiving diltiazem and adenosine. Considering his underlying complex anatomy, ablation was deferred, and the patient was cardioverted to sinus rhythm [Figure 1]. A loop monitor was placed and was started on flecainide with outpatient follow-up at a higher centre. Discussion: Infundibulotomy, removal of obstructive muscle bundles, and a patch to widen the passageway from the right ventricle to the pulmonary arteries are all steps in DORV repair. These surgeries result in scar tissue, an akinetic right ventricular outflow tract, and pulmonary valve insufficiency. Reentrant tachycardias may form due to surgical scars. In DORV patients, Atrial tachyarrhythmias have undesirable outcomes resulting in congestive heart failure, stroke, and mortality. Antiarrhythmics and cardioversion are the cornerstones of managing AFL in patients with DORV due to increased failure rates of catheter ablation. Conclusion: This case illustrates persistent atrial arrhythmias more likely due to DORV repair, resulting in cardiac remodeling. The abnormal anatomy makes localization of irritated ectopic foci and ablations ineffective and difficult.

Clinical case of diagnosis of left ventricular non-compaction in a child

Diseases of cardiovascular system in children present an extremely urgent problem nowadays. According to the latest epidemiological studies, there has been an increase by almost 3.2 % in congenital heart disease in Ukrainian children over the past 20–30 years. Left ventricular non-compaction belongs to a group of unclassified cardiomyopathies with undetermined prevalence. At the same time, based on literature, this pathology is characterized by a late diagnosis and very high mortality. In the pathogenesis of the disease, structural changes in the myocardium occur during the embryonic stage, leading to its abnormal development. Left ventricular non-compaction is considered a genetically heterogenous disease, which is inherited in an autosomal dominant pattern. The article deals with a clinical case of diagnosis and management of a child with left ventricular non-compaction, which demonstrates to ge­neral practitioners, pediatricians, and pediatric cardiologists the algorithm for diagnosing and managing patients with a rare disease in order to make earlier diagnosis, prevent complications, and preserve patient’s life. The patient who has been suffered from pneumonia, complained of breathlessness and rapid fatigue during minor physical exertion was timely referred by the family doctor for consultation with a pediatric cardiologist. Laboratory and instrumental studies allowed to suspect a rare congenital heart disease — left ventricular non-compaction. Subsequently, the patient was regularly examined by cardiologists and cardiac surgeons, received protocol therapy, was registered in a state transplant registry, which allowed to wait for a donor with subsequent successful heart transplant surgery.

Cervical aortic arch in the pediatric population: a meta-analysis of individual patient's data.

This is the first meta-analysis to analyze all reports of published pediatric cases of cervical aortic arch (CAA) by highlighting the clinical characteristics and treatment outcomes using the reported individual data of the patients. The aim of the study is to investigate the clinical features and surgical outcomes of such a rare disease in the pediatric population. A comprehensive search was conducted in various academic databases, including PubMed, ScienceDirect, SciELO, DOAJ, and Cochrane Library, until June 2022 for case reports describing the presence of cervical aortic arch in the pediatric age. Case reports and series were included if the following criteria were met: (1) description of the cervical aortic arch; (2) patient of pediatric age; and (3) published in the English language. All other types of publications that lacked patient-specific information were excluded from the analysis. This systematic review was conducted in accordance with the PRISMA guidelines. The primary outcome measure of the analysis was early and late mortality. The literature search identified 2,272 potentially eligible articles, 72 of which met our inclusion criteria with 96 patients including the author's institutional case. At a median of 365 (90-730) days, the overall cohort registered a 7.3% (7/96) mortality rate. In the subset of patients who underwent surgery, the mortality rate was also 7.3% (4/55), and the mortality rate following surgery to treat only CAA was 2.4% (1/42). Dyspnea was identified as an independent determinant of mortality by employing the univariable Firth bias-reduced logistic regression method. Cervical aortic arch is a rare congenital heart disease that poses treatment challenges due to the high anatomical variability, diverse clinical presentations, and the presence of other concomitant diseases. The surgical treatment appears to be a safe and effective approach for resolving the symptoms, although it needs to be tailored individually for each patient. https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=346826, Identifier: CRD42022346826.

Successful Pregnancy in a Patient with Ebstein’s Anomaly: A Rare Case Report

Ebstein’s anomaly, a rare congenital heart disease, occurring in 1 in 20,000 live births. The leaflets are placed deeper into the right ventricle instead of the normal position. Pregnancy however is well tolerated and the maternal risks of pregnancy co-relates well with the degree of tricuspid regurgitation, right ventricular function and presence of cyanosis. Aim is to emphasize the meticulous follow-up with fetal &amp; maternal medicine specialist with multidisciplinary approach can give a successful outcome. Proper diagnosis and meticulous follow-up with good ANC have made this case, pregnancy with Ebstein’s anomaly successful.   Bangladesh J Obstet Gynaecol, 2022; Vol. 37(1): 68-70

Anaesthetic management of a case of Ebstein’s anomaly undergoing a non cardiac surgery

Ebstein’s anomaly is a rare congenital heart disease characterized by apical displacement of the Tricuspid valve leaflets leading to Tricuspid Regurgitation. Here we report the successful management of a case of a patient with Ebstein’s anomaly who underwent Defunctioning Tranverse Colostomy. Given the complexity of Ebstein's anomaly and the potential for associated cardiac abnormalities, it is crucial to have a multidisciplinary team involved in the patient's care.