Shone's Complex Research Articles

The landscape of congenital heart disease treated with the Ross procedure

BackgroundThe Ross procedure has excellent outcomes in the pediatric population. Some series have reported age- and anatomy-dependent outcomes, but a comprehensive analysis stratified by these variables has not been reported to date. We sought to describe the landscape of congenital heart disease (CHD) treated with the Ross procedure and identify the patients best served by this operation. MethodsOver a total of 30 years, 317 pediatric patients underwent the Ross procedure. These patients were stratified into 4 age groups—neonates (age <31 days; n = 21), infants (age 31 days to 1 year; n = 40), children (age 1-12 years; n = 165), and adolescents (age 13-18 years; n = 91)—and 3 anatomic groups—isolated aortic valve (AV) disease (n = 221), Shone complex or multilevel left ventricular outflow tract (LVOT) obstruction (n = 61), and complex CHD (n = 35). The groups were compared across outcomes. ResultsNeonates and infants had the highest rates of Shone syndrome (P < .001), complex CHD (P < .001), concomitant root enlargement (P < .001), and arch procedures (P < .001). Operative mortality and morbidity were 4.4% (n = 14/317) and 14% (n = 44/317), respectively. Both were higher in neonates and infants (P < .001 for both) but lower for patients with isolated AV disease (P < .001 for both). Ten-year survival and freedom from LVOT reintervention were 92% and 81%, respectively, and were significantly better in patients with isolated AV disease compared to those with complex CHD (P < .001 and P = .005, respectively). In neonates and infants with isolated AV disease, operative mortality was 4% (n = 1/23), morbidity was 9% (n = 2/23), 10-year survival was 85.6%, and 10-year freedom from LVOT reintervention was 88.4%. ConclusionsAmong pediatric patients, those with isolated AV disease are best served with the Ross procedure, regardless of age. Complex CHD is associated with lower survival and increased risk of LVOT reintervention.

Clinical presentation and surgical outcomes in patients with Shone's complex: a systematic review.

Shone's complex comprises ofa combination of congenital cardiac anomalies causing obstructions in the left ventricle's inflow and outflow tracts. This systematic review aims to evaluate the clinical features and surgical outcomes of Shone's complex. An electronic literature search of PubMed and Scopus was performed to identify relevant studies related to the presentation, management, and outcomes of Shone's complex. Two reviewers independently performed selection. Data on study characteristics, participant demographics, interventions, outcomes, and follow-up durations were extracted and analyzed. A total of 691 papers were identified, with 18 studies included in the final analysis. The majority of the studies (n = 12) focused on the pediatric age group. The most common clinical presentations were coarctation of the aorta (n = 17) and mitral stenosis (n = 12). Surgical interventions often involved staged approaches, prioritizing outflow before inflow obstructions. Mitral valve repair was preferred over replacement due to better long-term outcomes (n = 8). Biventricular repair was recommended due to improved postoperative outcomes, but often needed reoperations. Reoperations were common, primarily due to recurrent coarctation (n = 10), subaortic stenosis (n = 8), and mitral valve dysfunction (n = 7). Pulmonary hypertension (n = 10) and arrhythmias (n = 11) were significant complications. Most patients were in modified Ross/NYHA functional class 1 on follow-up. Mortality rates ranged from 4 to 28%, with better outcomes associated with early and strategic surgical interventions. Early diagnosis and biventricular repair were associated with better outcomes while transplantation was often an eventuality. Standardized diagnostic criteria, long-term follow-up, and consensus guidelines are needed to improve the management of this congenital heart disease.

Perioperative and Anesthetic Considerations in Shone's Complex.

Shone's complex is a congenital cardiac disease consisting of the following four lesions: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and aortic coarctation. Though not all components are required for a diagnosis, the end result is both left ventricular inflow and outflow obstruction, which typically present in patients as congestive heart failure. The complex pathology requires careful management and surgical decision-making to ensure an optimal outcome. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with Shone's complex.

Atrial and Ventricular Arrhythmia in Adults With Shone Complex: A Single-Center Cohort Study

BackgroundMultilevel obstruction in left ventricular inflow and outflow predisposes to arrhythmias in Shone’s complex (SC). ObjectivesThe purpose of this study was to study the prevalence and outcomes (heart failure [HF] hospitalization, cardiac transplant, death) of cardiac arrhythmias in adults with SC. MethodsAdults with SC (defined as ≥2 lesions out of supramitral ring, parachute mitral valve, subvalvular/valvular aortic stenosis (AS), and aortic coarctation) seen at Mayo Clinic between January 1999 and March 2020 were identified and evaluated for the presence of sustained atrial fibrillation, atrial flutter, and ventricular arrhythmias (VA). Kaplan-Meier survival analysis was used to calculate the occurrence of these arrhythmias. ResultsSeventy-three patients with SC (mean age at first visit 33 ± 13 years) were identified. Most common anomalies were valvular AS (88%), coarctation (85%), parachute mitral valve (44%), subvalvular AS (44%), and supramitral ring (25%). Atrial arrhythmias were diagnosed in 24 patients (33%) at a mean age of 34.6 ± 12.7 years. Patients with atrial fibrillation and atrial flutter had higher number of surgeries, left atrial size, right ventricular systolic pressure, and HF hospitalizations. A rhythm control approach was used in majority of patients (75% on antiarrhythmic drugs and 50% underwent catheter ablation). Sustained VA occurred in 6 of 73 patients of whom 4 had an ejection fraction <40%. Death and cardiac transplantation occurred in 11 and 3 patients, respectively, during a median follow-up of 7.3 ± 6.0 years. ConclusionsIn adults with SC, atrial arrhythmias occurred in one-third of patients, were associated with more HF hospitalizations, and frequently required rhythm control. Prevalence of sustained VA was 8% and implantable cardioverter-defibrillator implantation should be considered in those with reduced ejection fraction.

Un cas rare de complexe de Shone incomplet chez un homme de 35 ans avec un morphotype marfanoïde et revue de la littérature

Shone syndrome is a rare and complex congenital heart disease. It is characterized in its complete form, by a constellation of the four potentially obstructive lesions of the left heart side. Incomplete Shone's complex includes 2 or 3 of the characteristic lesions which, include a supravalvular mitral membrane, valvular mitral stenosis (MS) by a parachute mitral valve (PMV), subaortic stenosis and aortic coarctation (Coa). We describe here, the case of a 35-year-old man, hospitalized for chronic dyspnea and in whom, the diagnosis of incomplete Shone's complex is retained by echocardiographic analysis. Observed lesions include a parachute mitral valve responsible for mild regurgitation and moderate mitral stenosis, bicuspid aortic valve, and pseudo interruption of the aorta with dilatation of the ascending aorta and a patent ductus arteriosus. This case is the 2nd on 3942 patients in Drighil congenital heart disease registry.

(467) Shone's Complex: Not So ‘Simple’ after All

(467) Shone's Complex: Not So ‘Simple’ after All

International practice heterogeneity in pre-transplant management of pulmonary hypertension related to pediatric left heart disease.

Elevated pulmonary vascular resistance (PVR) in the setting of left heart failure may contribute to poor outcomes after pediatric heart transplant (HTx), but peri-transplant management is variable. We sought to characterize international practice by surveying physicians at pediatric HTx centers. We received 49 complete responses from 39 centers in 16 countries. Most respondents are pediatric cardiologists (90%), practice at centers offering heart (86%) and lung (55%) transplant, and perform pre-HTx acute vasoreactivity testing (AVT, 88%) in patients with elevated PVR. Half (51%) reported defining a PVR cutoff for HTx eligibility as ≤6WU m2 (56%) post-AVT (84%). The highest post-AVT PVR ever accepted for HTx ranged from 3-14.4 (median 6) WU m2 . To treat elevated pre-transplant PVR, phosphodiesterase type 5 inhibitors are most common (65%) followed by oxygen (31%), nitric oxide (14%), endothelin receptor antagonists (11%), and prostacyclins (6%). Nearly a third (31%) do not routinely use pulmonary vasodilators without implantation of a left ventricular assist device (LVAD). Case scenarios highlight treatment variability: in a restrictive cardiomyopathy scenario, HTx listing with post-transplant vasodilator therapy was favored, whereas in a Shone's complex patient with fixed PVR, LVAD ± pulmonary vasodilators followed by repeat catheterization was most common. Management of dilated cardiomyopathy with reactive PVR was variable. Most continue vasodilator therapy until HTx (16%), PVR normalizes (16%) or ≤6 months. Management of elevated PVR in children awaiting HTx is heterogenous. Evidence-based guidelines are needed to allow for longitudinal determination of optimal outcomes and standardized care.

Pacman Heart as a Congenital Cardiac Defect Associated with Flail Mitral Valve and a Partial form of Shone's Complex; A Case Report

Pacman Heart as a Congenital Cardiac Defect Associated with Flail Mitral Valve and a Partial form of Shone's Complex; A Case Report

Evaluation of midterm outcomes after Shone's complex surgery: Analysis of reoperation and mortality risk factors

The midterm results of patients who underwent biventricular repair surgery for Shone's complex were examined, and mortality and reoperation risk factors were evaluated. This retrospective study included 34 patients with Shone's complex who underwent mitral valve (MV) surgery between 2005 and 2020. A total of 19 patients (56%) had coarctation, 10 (29%) patients had subaortic stenosis, 9 (26.5%) patients had a hypoplastic aortic arch (AA), and 9 (26.5%) patients had aortic valve (AV) stenosis. Twenty-four (70.6%) patients had bileaflet AV. Associated left-sided in-flow stenotic lesions included parachute MV in 19 (56%) patients and supramitral ring in 18 (53%) patients. The estimated freedom from reoperation rate on the 6th month, 1 year and 2 years after surgery was 84.4%, 79.5%, and 71.5%, respectively. The overall mortality rate was 20.6% (seven patients) with a median follow-up of 10 months (0-41). The estimated survival rate on the 6th month, 1 year, and 3 years after surgery was 83.8%, 79.4%, and 79.4 respectively. Bicuspid aortic valve (p = .017) (HR (95% CI) = 0.130 (0.025-0.695) and hammock mitral valve (p = .038) (HR (95% CI) = 11,008 (1,146->100) were associated with mortality. The presence of a bicuspid aortic valve hammock mitral valve might have an effect on negative effect on the outcome.

Comparison of early-stage stenosis and regurgitation results among patients who underwent aortic valve repair.

This study aimed to analyse the results of paediatric aortic valve repairs in our institution. The data of 57 patients under 18 years of age who underwent aortic valve repair between 2014 and 2019 were retrospectively analysed. Early postoperative reoperation and hospital mortality rates were evaluated based on the ages of the patients, their preoperative diagnoses, and the surgical techniques used. Survival curves for groups of patients were calculated by Kaplan-Meier analysis. The rate of reoperation was 14% (n = 8), and there were no significant differences regarding valve pathologies and preoperative diagnoses (p > 0.05). Among the repair techniques, tricuspidisation was considered to be a risk factor for reoperation (p < 0.05). Augmentation, the material used (0.1 PTFE or pericardium), and the number of cusps were not found to have significant effects on reoperation or mortality. The mean follow-up period was 29.86 ± 21.30 months. The survival rates of the patients were 88%, 100%, and 88.2% for those with aortic stenosis, aortic insufficiency, and mixed disease, respectively, and no significant difference was found when these rates were evaluated with the log-rank test (p > 0.05). The mortality rate was 8.8% (n = 5), and undergoing surgery before the age of 1 year was found to be significant in terms of mortality (p = 0.032, p < 0.05). The bicuspid aortic valve group had the lowest mortality risk, while the Shone complex group had the highest. With its acceptable reoperation and mortality rates, aortic valve repair should be the first choice of treatment in the paediatric age group. Early results were satisfactory in all groups.

Strategies to mitigate inflammation in management of complex congenital heart disease complicated by "multisystem inflammatory syndrome in children".

A 6-month-old boy, a case of Shone's complex, presented in decompensated state was found to have severe mitral stenosis along with multisystem inflammatory syndrome in children (MISC) warranting urgent surgical intervention. Various modalities including cytokine-adsorbing hemofilter were used to target inflammation. Postoperatively, the child recovered from low cardiac output accompanied by decrease in the levels of inflammatory markers, inopressors, and ventilatory requirements. Open heart surgery in itself is a proinflammatory process and is best avoided during the active inflammatory phase of MISC. In the rare and unavoidable circumstance exemplified by this index case, multipronged strategy targeting inflammation as described can be successfully implemented.

Ross procedure in neonates and infants: A valuable operation with defined limits

ObjectiveThe Ross procedure is an important tool that offers autologous tissue repair for severe left ventricular outflow tract (LVOT) pathology. Previous reports show that risk of mortality is highest among neonates and infants. We analyzed our institutional experience within this patient cohort to identify factors that most affect clinical outcome. MethodsA retrospective chart review identified all Ross operations in neonates and infants at our institution over 27 years. The entire study population was analyzed to determine risk factors for mortality and define outcomes for survival and reintervention. ResultsFifty-eight patients underwent a Ross operation at a median age of 63 (range, 9-156) days. Eighteen (31%) were neonates. Eleven (19%) patients died before hospital discharge. Multiple regression analysis of the entire cohort identified young age (hazard ratio [HR], 1.037; P = .0045), Shone complex (HR, 17.637; P = .009), and interrupted aortic arch with ventricular septal defect (HR, 16.01; P = .031) as independent predictors of in-hospital mortality. Receiver operating characteristic analysis (area under the curve, 0.752) indicated age younger than 84 days to be the inflection point at which mortality risk increases. Of the 47 survivors, there were 2 late deaths with a mean follow-up of 6.7 (range, 2.1-13.1) years. Three patients (6%) required LVOT reintervention at 3, 8, and 17.5 years, respectively, and 26 (55%) underwent right ventricular outflow tract reintervention at a median of 6 (range, 2.5-10.3) years. ConclusionsRoss procedure is effective in children less than one year of age with left sided obstructive disease isolated to the aortic valve and/or aortic arch. Patients less than 3 months of age with Shone or IAA/VSD are at higher risk for morbidity and mortality. Survivors experience excellent intermediate-term freedom from LVOT reintervention.

Echocardiography in the diagnosis of Shone's complex and analysis of the causes for missed diagnosis and misdiagnosis.

BACKGROUNDShone’s complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients.AIMTo use echocardiography in the diagnosis of Shone’s complex and analyze the causes of missed diagnosis and misdiagnosis.METHODSThis was a retrospective study of patients who underwent echocardiography and repair surgery from February 14, 2008, to November 22, 2019. The patients were followed once a year at the outpatient clinic after surgery.RESULTSSixty-six patients were included. The patients were 2.7 (0.8-5.6) years of age, and 54.5% were male. Ten (15.2%) had a history of heart surgery. The most common heart defect was the Annulo-Leaflet mitral ring (ALMR) (50/66, 75.8%), followed by coarctation of the aorta (CoA) (43/66, 65.2%). The patients had a variety of combinations of defects. Only two (3.0%) patients had all four defects. None of the patients had a family history of congenital heart disease. The preoperative echocardiographic findings were examined against the intraoperative findings. Echocardiography missed an ALMR in 31 patients (47.0%), a parachute mitral valve (PMV) in one patient (1.5%), subaortic stenosis in one patient (1.5%), and CoA in two patients (3.0%).CONCLUSIONEchocardiography is an effective method to diagnose the Shone’s complex. Due to this disease’s complexity and interindividual variability, Improving the understanding of the disease can reduce misdiagnosis and missed diagnosis.

The Use of a Left Ventricular Assist Device and Pulmonary Artery Pressure Monitor in a Case of Restrictive Cardiomyopathy

<h3>Introduction</h3> Historically, restrictive cardiomyopathy has limited the use of left ventricular assist devices (LVAD) as a bridge to heart transplant (HT). We present a case that highlights the use of left ventricular assist device and remote pulmonary artery (PA) pressure monitoring as a bridge to HT to successfully reduce pulmonary vascular resistance (PVR) in a patient with restrictive cardiomyopathy. <h3>Case Report</h3> A 24 year old female with history of Shone's complex complicated by restrictive cardiomyopathy and mixed pre- and post- capillary pulmonary hypertension presented with recurrent decompensated heart failure. Patient had right heart catheterization at time of admission that demonstrated elevated filling pressures, normal cardiac output, and elevated PVR (Figure 1a). Due to elevated PVR, HT was deemed too high risk and heart-lung was considered. LVAD implantation was pursued to try and optimize pulmonary pressures and allow HT only. PA pressure monitor was placed as outpatient and help facilitate pump optimization and pulmonary hypertension therapy uptitration. Patient gradually had improvement in PA pressures and ultimately was able to be listed for HT only. <h3>Summary</h3> This report highlights the use of both LVAD and remote PA pressure monitoring to manage and ultimately decrease PVR in a patient with restrictive cardiomyopathy.

Pregnancy in Patients with Shone Complex: A Single-Center Case Series

Background: There is limited literature written on the course and outcomes for pregnant mothers with Shone complex. Methods: We describe a case series of five pregnancies in four women with Shone complex within a multidisciplinary cardio-obstetrics clinic from 2016–2018. Results: Maternal age ranged from 21–39 years. Three patients had preserved left ventricular function while one had moderately decreased function. Gestational age at presentation ranged from 6–15 weeks. There were three successful pregnancies (mean gestational age = 37 weeks, range 35–39 weeks) with one patient accounting for two unsuccessful pregnancies. All infants were delivered via Cesarean section. One infant required a NICU stay, but all other infants delivered were healthy. Conclusion: Patients with Shone complex can have successful pregnancies although complications can occur for both the mother and the baby. Comprehensive prenatal care, coordinated and consistent management during pregnancy, and tertiary care support can promote positive maternal and fetal outcomes.

Mitral valve replacement in infants and younger children

Data on mitral valve replacement (MVR) in young children is still limited. Our objective was to evaluate MVR in children below 5 years and identify factors affecting the outcomes. This retrospective study included 29 patients who had MVR from 2002 to 2020. We grouped the patients into two groups according to their age: age ≤ 24 months (n = 18) and > 24 months (n = 11). Primary cardiac diagnoses were Shone complex (n = 7; 24%), isolated congenital mitral valve abnormality (n = 11; 38%), and complete atrioventricular septal defect (n = 3; 10%). The median age was 19 month (25th–75th percentile: 11–32) and 59% were females (n = 17). The hemodynamic lesions were mitral regurgitation in 66%, mitral stenosis in 10%, and combined mitral stenosis and regurgitation in 24% of the patients. St. Jude mitral valve was the most common valve implanted (n = 19, 66%), followed by CarboMedics in 21% of the patients (n = 6). The mitral valve was implanted in the supra-annular position in 6 cases (21%). Preoperative and operative data were comparable between both groups. There was no association between valve size and position with postoperative heart block (P > 0.99, for both). The median follow-up duration was 19.4 months (8.6–102.5). Nine patients had mitral valve reoperation, six had MVR, and three had clot removal from the mitral valve. There was no effect for age group on reoperation (SHR 0.89 (95% CI 0.27–2.87), P = 0.84). Valve size significantly affected reoperation (SHR 0.39 (95% CI 0.18–0.87), P = 0.02). The supra-annular position was associated with an increased risk of reoperation (SHR 3.1 (95% CI 1.003–9.4), P = 0.049). There was no difference in survival according to the age (Log-rank P = 0.57) or valve size (Log-rank P = 0.66). Mitral valve replacement in children is associated with low morbidity and mortality. The risk of reoperation could be affected by the valve size and position rather than the age.

Bicuspid Aortic Valve in Infants, Children, and Adolescents: A Review for Primary Care Providers.

1. Talha Niaz, MBBS* 2. Jonathan N. Johnson, MD*,† 3. Frank Cetta, MD*,† 4. Timothy M. Olson, MD*,† 5. Donald J. Hagler, MD*,† 1. *Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, and 2. †Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN * Abbreviations: AHA : : American Heart Association BAV : : bicuspid aortic valve TTE : : transthoracic echocardiography Bicuspid aortic valve is the most common congenital heart defect in children, adolescents, and adults. Primary care providers play an important role in screening, referral, and follow-up of these patients and should be aware of the family screening guidelines, sports participation recommendations, and periodic follow-up requirements for adequate surveillance of the complications that arise from bicuspid aortic valve. After reading this article, readers should be able to: 1. Describe the epidemiology and anatomy of bicuspid aortic valve (BAV). 2. Understand the clinical presentation and diagnosis of BAV in infants, children, and adolescents. 3. Identify the various complications of BAV disease. 4. Discuss the management and follow-up requirements for BAV. 5. Analyze the family screening and sports participation guidelines for patients with BAV. Bicuspid aortic valve (BAV) is the most common congenital heart defect in children, adolescents, and adults. (1) It is a heterogeneous disease that affects both the aortic valve and the aorta. It can lead to many complications, including aortic valve stenosis, regurgitation, or endocarditis. (2)(3) It also can lead to dilation of the aorta, predisposing individuals to a significantly higher risk of aortic aneurysm and dissection. (4) Although most individuals with BAV present with these long-term complications during adulthood, a considerable number of patients may also present during childhood and adolescence with early-onset disease; that may require interventions in up to 12% to 15% of the patients. (5)(6) Therefore, patients with BAV require lifelong follow-up and surveillance. BAV has multiple implications in terms of sports participation and family screening, making it an important subject for primary care providers. This article reviews the anatomy, genetics, presentation, diagnosis, …

Melody valve to replace the mitral valve in small children: Lessons learned.

Objective:Infants requiring mitral valve replacement have few viable options. Recently, stented bovine jugular vein graft (Melody) has been surgically implanted in such cases. Herein, we report our experience, elaborating on evolution of implantation technique, pitfalls, as well as long-term outcome (including late dilatability).Methods:Seven Melody valves were implanted (2013–2019). The median patient age and weight were 6.7 (1.8–30.5) months and 5.8 (4.6–9.5) kg, respectively. The indications for implantation were mitral stenosis and/or regurgitation postatrioventricular septal defect (AVSD) repair (5), congenital mitral valve dysplasia (1), and Shone's complex (1). Operative technique involved shortening the valve and creating a neo-sewing ring at 2/3 (atrial)–1/3 (ventricular) junction. Implantation was followed by intraoperative balloon dilatation.Results:Five out of seven patients survived the perioperative period (one death due to technical failure and the other due to acute respiratory distress syndrome postcardiopulmonary bypass). Two out of five medium-term survivors got transplanted (1) or died due to acute myeloid leukemia (1). No valves were replaced. The mean echo gradient at discharge was a median 4 (2–6) mmHg. None of the patients showed left ventricular outflow tract or pulmonary venous obstruction. Two Melody valves were dilated late (5 months and 3 years postoperatively), resulting in decreasing mean gradients from 6 to 1 and from 17 to 4 mmHg. At last follow-up, surviving Melody had a mean gradient of 4 (1–9) mmHg.Conclusions:Mitral valve replacement with a Melody valve is feasible in infants, is reproducible, shows good immediate results, and offers the possibility of later dilatation. This technique offers a better solution compared to the existing alternatives for infants requiring a prosthetic mitral valve.

Complex congenital heart diseases among children presenting for cardiac surgery in a tertiary health facility in Enugu; South-East Nigeria. A rising trend.

Complex cardiac anomalies are a set of associated heart structural anomalies requiring some adaptive mechanism to maintain life. This is to determine pattern and prevalence of common complex cardiac anomalies among children presenting for cardiac surgery in Enugu. The children that present for cardiac surgery in our center over a six-month period were admitted and rescreened using a standardized echocardiographic method, information on biodata, oxygen saturation and 2D echo diagnosis were recorded in a proforma. A hundred and one (101) children with different structural heart diseases presented for cardiac surgery, 53 females and 48 males, 99 (98%) had congenital heart defects (49 cyanotic and 50 acyanotic). Complex cardiac anomalies were noted among 49 and 50 were non-complex, 6 (12.2%) acyanotic, and 43 (87.8%) cyanotic complex P < 0.01. This was noted among 21 females and 28 males. Children less than 3 years had 31 cases (63.3%) of the complex heart diseases while children between 5 years to 18 years had 14 (28.6%). The top three complex congenital heart disease were complex Tetralogy of Fallot which was found among 26 (53%) subjects. Tricuspid atresia and Truncus arteriosus were found in 3 children with congenial heart disease each, Shone complex was found in a child, Hypoplastic left heart syndrome was also noted in a child. The incidence of complex congenital heart diseases among prospective Paediatric cardiac surgery patients is high. Efforts should be made to improve access to preventive cardiology in order to curb these diseases.

Outcomes of biventricular repair for shone's complex.

Shone's complex is a rare lesion affecting the mitral valve (MV) and left ventricular outflow tract (LVOT). The objective of this study is to report the outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long-term survival. This retrospective study included all patients diagnosed with Shone's complex, who underwent biventricular repair. Data including patients' characteristics, type of the MV lesion and the associated lesions were collected. Patients were followed up regularly with echocardiography, and the changes in mitral and aortic valve z-score and LVOT z-score were recorded. Thirty-seven patients were included in the study, the median age was 3.4 months, and 11 patients (30.6%) had pulmonary hypertension. The main procedure performed during the first surgical intervention was coarctation repair in 26 patients (70%). Twelve patients had MV repair, and five had MV replacement. Operative mortality occurred in 1 patient (2.7%), median follow up was 52 (25-75th percentile: 22-84) months. Survival at 1, 5, and 10 years was 94.4%, 90%, and 76.9%, respectively. Reoperation was required in 13 patients, mainly for LVOT repair (n = 8). Reoperation was significantly associated with associated aortic valve lesion (p = .044). The growth of the MV z-score was 0.35 per year; p < .001, aortic valve z-score 0.086 per year; p = 0.422, and the LVOT z-score was 0.53 per year; p = .01. Biventricular repair of Shone's complex has good outcomes. Reoperation is frequently encountered, especially with low aortic valve z-score. The MV and LVOT have significant growth following Shone's complex repair.