Glenn Procedure Research Articles

Interventional Management of Acute Thrombosis of the Modified Blalock-taussig Shunt in a Case of Tricuspid Atresia

Abstract Introduction: Acute thrombosis of the modified Blalock-Taussig shunt (mBTS) in patients with univentricular physiology is a life-threatening event. Case presentation: We present a case of acute mBTS thrombosis complicating a hemodynamic evaluation before a bidirectional Glenn procedure for tricuspid atresia. After failed balloon angioplasty and catheter-directed selective thrombolysis, the patient was successfully managed by stent implantation into the thrombosed shunt. To the best of our knowledge, this was the first case of acute mBTS thrombosis treated by catheter intervention in Romania. Conclusions: Even a routine invasive hemodynamic evaluation might be complicated by a life-threatening condition. Stent implantation in the setting of acute mBTS thrombosis could be lifesaving.

The Impact of Dominant Ventricular Morphology on the Early Postoperative Course After the Glenn Procedure.

The dominant ventricular morphology affects both the early and late outcomes of the Fontan procedure, but its impact on the patients' status immediately following the Glenn procedure is unknown. This study aims to evaluate the effect of the infants' dominant ventricular morphology on the immediate course after undergoing the Glenn procedure. This single-center, retrospective study included all patients who underwent the Glenn procedure between October 2003 and May 2016. The patients were divided into two groups according to their dominant ventricular morphology. Their postoperative records were reviewed and compared. Out of the 89 patients who underwent the Glenn procedure during the study period, 40 (44.9%) had dominant right ventricular morphology and 49 (55.1%) had left ventricular morphology. There were no significant group differences in baseline characteristics or operative data. The maximal postoperative vasoactive-inotropic score was significantly higher and the extent of ventricular dysfunction was significantly more severe in the dominant right ventricle group (P < 0.05). The length of hospitalization was slightly but not significantly longer in the hypoplastic LV group. It is concluded that patients with a dominant LV morphology had a superior ventricular function and required less inotropic support compared to that of a dominant RV morphology in the immediate postoperative course following the Glenn procedure. Survival was not affected by these differences. Further study to determine the pathophysiologic basis for these differences is warranted.

Evaluation of Postoperative Follow-ups of Children Having the Glenn Procedure

Introduction: This study aimed to evaluate the treatment results, complications, and the factors affecting mortality in cases who were followed up postoperatively in the pediatric intensive care unit (PICU) after the Glenn shunt procedure. Materials and Methods: The duration of the patients on the pump during surgery, transition times to feeding, blood gas and other laboratory parameters, complications developed during follow-up in the intensive care unit, and exitus rates were evaluated retrospectively. Results: BDG was performed due to tricuspid atresia, the most common diagnosis (30.2%). In the evaluation of the relationship between postoperative patient findings and exitus, a positive correlation was found between the duration of inotropic support, early extubation, and the duration of HFNC therapy in all patients (p&lt;0.05); and a negative correlation was found in terms of the fluid the patients received on the first day after surgery (p&lt;0.05). In patients who developed complications, a negative correlation was observed only in terms of the fluid they received on the first day after surgery (p&lt;0.05) Conclusions: In terms of all patients, it was observed that mortality increased with early extubation and prolonged duration of inotropic support and HFNC therapy, and it decreased as the fluid the patients received on the first day after surgery decreased. In patients who developed complications, it was only observed that mortality decreased as the fluid the patients received on the first day after surgery decreased.

Association Between Venous Homografts and Allosensitization After Norwood Procedure.

Right ventricle (RV) to pulmonary artery (PA) shunts have become the shunt of choice at many centers for use during the Norwood procedure for single ventricle palliation. Some centers have begun to use cryopreserved femoral or saphenous venous homografts as an alternative to polytetrafluoroethylene (PTFE) for shunt construction. The immunogenicity of these homografts is unknown, and potential allosensitization could have significant implications on transplant candidacy. All patients undergoing Glenn procedure at our center between 2013 and 2020 were screened. Patients who initially underwent Norwood procedure with either PTFE or venous homograft RV-PA shunt and had available pre-Glenn serum were included in the study. The primary outcome of interest was panel reactive antibody (PRA) level at the time of Glenn surgery. Thirty-six patients met inclusion criteria (N = 28 PTFE, N = 8 homograft). Patients in the homograft group had significantly higher median PRA levels at the time of Glenn surgery (0% [IQR 0-18] PTFE vs 94% [IQR 74-100] homograft, P = .003). There were no other differences between the two groups. Despite potential improvements in PA architecture, the use of venous homografts for RV-PA shunt construction at the time of Norwood procedure is associated with significantly elevated PRA level at the time of Glenn surgery. Centers should carefully consider the use of currently available venous homografts given the high percentage of these patients who may require future transplantation.

Jacobsen Syndrome with Hypoplastic Left Heart Syndrome: Outcome after Cardiac Transplantation

Jacobsen syndrome (JS) is a rare syndrome caused by a deletion of chromosome 11q. We report a patient with JS and hypoplastic left heart syndrome (HLHS) who required cardiac transplantation. She had many of the recognized morphological features in addition to immunological (lymphopenia) and hematological (thrombocytopenia) issues. The patient underwent a Norwood procedure with a modified Blalock-Taussig shunt (MBTS) and subsequently a Glenn procedure at six months of age. She developed desaturation, with severe tricuspid regurgitation and right ventricular dysfunction, and underwent heart transplantation at 7 months of age. After the transplant, she was hospitalized several times for severe infections. The diagnosis of Jacobsen syndrome came 2 months after transplant. Now, 5 years post-transplant, she is in relatively good health-her heart is functioning normally, her hospitalization rate is getting lower, and her immunological profile is stable.

Ductus arteriosus banding to regulate excessive pulmonary blood flow in a neonate with necrotizing enterocolitis and complex congenital heart disease, including pulmonary atresia and total anomalous pulmonary venous return: a case report

BackgroundPatients with right isomerism have accompanying complex congenital heart disease, which is characterized by pulmonary atresia and total anomalous pulmonary venous return. Balanced regulation of the systemic and pulmonary circulation is essential for successful management, especially for cases complicated with necrotizing enterocolitis (NEC).Case presentationA 6-day-old male neonate with a single ventricle, pulmonary atresia, patent ductus arteriosus (DA), and total anomalous pulmonary venous return associated with right isomerism was admitted because of dyspnea, cyanosis, and melena. The patient presented circulatory incompetence due to excessive pulmonary blood flow, resulting in NEC. The patient underwent DA banding and colectomy following continuous intravenous infusion of prostaglandin E1 at six days. Subsequently, his condition improved, reaching a systemic oxygen saturation of around 80%. He underwent a bidirectional Glenn procedure and closure of colectomy at the ages of 5 and 6 months, respectively.ConclusionDA banding can be an alternative to placing an aortopulmonary shunt, which is conventional in patients with ductus-dependent pulmonary circulation, because DA banding is feasible without cardiopulmonary bypass.

Forty-year survival after Glenn procedure without Fontan procedure in patients with single ventricle.

There are no data on long-term outcomes beyond 30 years after the Glenn procedure without the subsequent Fontan procedure in patients with single-ventricle physiology. Hence, this study aimed to clarify the very long-term outcomes of these patients. This single-centre, retrospective cohort study investigated the clinical outcomes of patients with single-ventricle physiology who underwent the Glenn procedure between 1970 and 1999. Those who underwent the subsequent Fontan procedure were excluded. The primary outcome was all-cause death. The secondary outcome was a composite of all-cause death, arrhythmic events, neurological events or infective endocarditis. The prognostic factors associated with the long-term outcomes were also evaluated. In total, 36 patients were enrolled (median age at Glenn procedure: 6.2 years, 56% male). During a median follow-up of 17.6 years (interquartile range: 6.1-33.4), 21 patients died and 29 experienced the composite outcome. The 20-, 30- and 40-year overall survival after the Glenn procedure was 51.2%, 44.4% and 40.3%, respectively. The 20-, 30- and 40-year event-free survival was 36.0%, 25.5% and 14.5%, respectively. Patients with dominant left ventricular morphology had better overall survival than those with dominant right ventricular morphology (hazard ratio: 0.24, 95% confidence interval: 0.08-0.76, P = 0.014). None of the patients had liver cirrhosis but 1 had protein-losing enteropathy. The 40-year overall survival after the Glenn procedure without the subsequent Fontan procedure in patients with single-ventricle physiology was 40.3%. Dominant left ventricular morphology may be associated with better long-term overall survival than dominant right ventricular morphology.

Somatic growth in single ventricle patients: A systematic review and meta-analysis.

To map somatic growth patterns throughout Fontan palliation and summarise evidence on its key modifiers. Databases were searched for relevant articles published from January 2000 to December 2021. Height and weight z scores at each time point (birth, Glenn procedure, Fontan procedure and >5 years after Fontan completion) were pooled using a random effects meta-analysis. A random effects meta-regression model was fitted to model the trend in z scores over time. Nineteen studies fulfilled eligibility criteria, yielding a total of 2006 participants. The z scores for height and weight were markedly reduced from birth to the interstage period, but recovered by about 50% following the Glenn procedure. At >10 years after the Fontan procedure, the z scores for weight seemed to normalise despite persistent lower height, resulting in increased body mass index. The review revealed a number of modifiers of somatic growth, including aggressive nutritional management, timing of Glenn/Fontan, prompt resolution of complications and obesity prevention programmes in adolescence and adulthood. This review mapped the somatic growth of single ventricle patients and summarised key modifiers that may be amendable to improvement. These data provide guidance on strategies to further optimise somatic growth in this population and may serve as a benchmark for clinical follow-up.

Congenitally corrected transposition with left ventricular outflow obstruction and cardiac malposition: One-and-a-half ventricular repair vs. Fontan pathway?

This study was to assess the mid-term results of the one-and-a-half ventricular repair (hemi-Mustard and bidirectional Glenn procedures combined with the Rastelli procedure) and Fontan pathway for correcting congenitally corrected transposition of great artery (ccTGA) patients with left ventricular outflow tract obstruction (LVOTO) and cardiac malposition. In this retrospective study, 74 consecutive ccTGA with LVOTO and cardiac malposition underwent the one-and-a-half ventricular repair (group A; 33 cases) and Fontan operation (group B; 41 cases) between October 2011 and March 2018. The Median follow-up time was 49 (20-84) and 42 (7-85) months in groups A and B, respectively. To estimate excise tolerance the 6-min walk test (MWT) was performed. No in-hospital death. Compared with group A, group B have significantly less CPB, mechanical ventilation time, and intensive care unit stay, but prolonged pleural effusions developed more frequently in Group B. The survival probability was 90.2% (95% CI, 80.2-100%) and 97.2% (95% CI, 92-100%) at 7 years (p = 0.300) in group A and B. The probability of freedom from re-intervention were 80.6% (95% CI, 66.5-97.6%) and 97.2% (95% CI, 92-100%) at 7 years (p = 0.110). Longitudinal repeated measured echo data at every follow-up time shows that group A has more systemic ventricular EF% (p < 0.001) and less moderate systemic ventricular valve regurgitation (p < 0.001) compared with group B. Estimated by 6 MWT, group A has better outcomes for 6-min walk distance. For correction of ccTGA with LVOTO and cardiac malposition, the one-and-a-half ventricular repair had superior midterm heart function and excise tolerance.

Conservative vs surgical management in ebstein's anomaly: long-term follow-up in a real-life cohort

Abstract Background Ebstein's anomaly (EA) is a rare congenital heart disease that involves the tricuspid valve and the right ventricle, often associating atrial septal defects and atrial arrhythmias. Oligosymptomatic adults affected by this disease usually have good prognosis, while its evolution in symptomatic children is more aggressive. Clinical guidelines provide some recommendations for the former patients regarding surgical indication, but its evidence is scarce and benefit remains unclear. Purpose To compare functional capacity, ventricular function parameters and outcomes between medically and surgically treated patients with EA. Methods Retrospective cohort study of patients with EA followed-up in an adult congenital heart disease reference unit of a tertiary university hospital. Functional status and ventricular size and function parameters at baseline and follow-up, as well as clinical events, were compared between conservative and surgical groups. Results We included 72 patients; mean age was 45.2±15.2 years and 27 (37.5%) were men. Median follow-up was 6.6 (range 0.3–20.3) years. Finally, 18 (25%) patients were operated (12 tricuspid repairs, 5 tricuspid bioprostheses and 1 mechanical tricuspid prostheses). Characteristics and outcomes of conservative and surgical group are shown in Table 1. Comparison of follow-up data for each group are shown in Table 2. Of note, Glenn procedure was done in 6 (33.3%) patients of the surgical group and prosthetic thrombosis was observed in 3 (50%) patients during follow-up. Conclusions Only one fourth of patients with EA in our cohort were managed surgically. Operated patients had worse functional status and larger right ventricles at baseline. At follow-up, surgical patients had more clinical events and worse ventricular function. Exercise test parameters did not change significantly after surgery, whereas there was worsening functional capacity during follow-up in medically-managed patients. Funding Acknowledgement Type of funding sources: None.

Perioperative Management of a Child with Hypoplastic Left Heart Syndrome Undergoing Cryptorchidism Surgery

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart condition which includes abnormal development of left sided cardiac structures leading to inadequate systemic perfusion following postnatal closure of the patent ductus arteriosus (PDA).&#x0D; Surgical palliation may be accomplished through a 3 staged process&#x0D; -Norwood procedure. This surgery is usually done within the first two weeks of your child's life&#x0D; -Bidirectional Glenn procedure. This procedure is generally the second surgery. It's done when your child is between 3 and 6 months of age.&#x0D; -Fontan procedure. This surgery is usually done when your child is between 18 months and 4 years of age.&#x0D; Patients with HLHS may need to undergo other non-cardiac surgical procedures during the first years of life posing a real challenge to the anesthesiologist, surgeon and the entire medical team.&#x0D; We present the case of a 18-months old, 9 kg infant who presented for cryptorchidism surgery. Cryptorchidism or undescended testis (UDT) is one of the most common pediatric disorders of the male endocrine glands and the most common genital disorder identified at birth. The main reasons for treatment of cryptorchidism include increased risks of impairment of fertility potential, testicular malignancy, torsion and/or associated inguinal hernia.&#x0D; The intraoperative implications of the hybrid anatomy are discussed, options for anesthetic care presented, and previous reports of anesthetic care for such patients reviewed.&#x0D; Conclusion: LMA combined with local anesthesia was effective to maintaining optimal cardiac function of this child patient with HLHS&#x0D; In summary, children with palliated HLHS have anesthetic considerations that must be followed in order to reduce perioperative morbidity and mortality in this high-risk pathology.

Fibrinolytic treatment with tissue plasminogen activator in a patient with massive pulmonary thromboembolism and bidirectional Glenn procedure

Fibrinolytic treatment with tissue plasminogen activator in a patient with massive pulmonary thromboembolism and bidirectional Glenn procedure

Modified biatrial heart transplantation after bilateral bidirectional cavo-pulmonary shunts.

We describe a patient who underwent modified biatrial anastomosis heart transplantation after the bilateral Glenn procedure. We introduced a new surgical technique to use the native central pulmonary artery as systemic venous return, which was anastomosed to right atrium, and then, biatrial anastomosis was performed.

A complicated Glenn procedure: risk factors and association with adverse long-term neurodevelopmental and functional outcomes.

To determine potentially modifiable risk factors for a complicated Glenn procedure (cGP) and whether a cGP predicted adverse neurodevelopmental and functional outcomes. A cGP was defined as post-operative death, heart transplant, extracorporeal life support, Glenn takedown, or prolonged ventilation. All 169 patients having a Glenn procedure from 2012 to 2017 were included. Neurodevelopmental assessments were performed at age 2 years in consenting survivors (n = 156/159 survivors). The Bayley Scales of Infant and Toddler Development-3rd Edition (Bayley-III) and the Adaptive Behavior Assessment System-2nd Edition (ABAS-II) were administered. Adaptive functional outcomes were determined by the General Adaptive Composite (GAC) score from the ABAS-II. Predictors of outcomes were determined using univariate and multiple variable linear or Cox regressions. Of patients who had a Glenn procedure, 10/169 (6%) died by 2 years of age and 27/169 (16%) had a cGP. Variables statistically significantly associated with a cGP were the inotrope score on post-operative day 1 (HR 1.04, 95%CI 1.01, 1.06; p = 0.010) and use of inhaled nitric oxide post-operatively (HR 7.31, 95%CI 3.19, 16.76; p < 0.001). A cGP was independently statistically significantly associated with adverse Bayley-III Cognitive (ES -10.60, 95%CI -17.09, -4.11; p = 0.002) and Language (ES -11.43, 95%CI -19.25, -3.60; p = 0.004) scores and adverse GAC score (ES -14.89, 95%CI -22.86, -6.92; p < 0.001). Higher inotrope score and inhaled nitric oxide used post-operatively were associated with a cGP. A cGP was independently associated with adverse 2-year neurodevelopmental and functional outcomes. Whether early recognition and intervention for risk of a cGP can prevent adverse outcomes warrants study.

Predicting Hemodynamic Performance of Fontan Operation for Glenn Physiology using Computational Fluid Dynamics: Ten Patient-specific Cases.

Single ventricle hearts have only one ventricle that can pump blood effectively and the treatment requires three stages of operations to reconfigure the heart and circulatory system. At the second stage, Glenn procedure is performed to connect superior vena cava (SVC) to the pulmonary arteries (PA). For the third and most complex operation, called Fontan, an extracardiac conduit is used to connect inferior vena cava (IVC) to the PL and thereafter no deoxygenated blood goes to the heart. Predicting Hemodynamic Performance of Fontan Operation using computational fluid dynamics (CFD) is hypothesized to improve outcomes and optimize this treatment planning in children with single-ventricle heart disease. An important reason for this surgical planning is to reduce the development of pulmonary arteriovenous malformations (PAVM) and the need to perform Fontan revisions. The purpose of this study was to develop amodel for Fontan surgical planning and use this model to compare blood circulation in two designed graft types of Fontan operation known as T-shape and Y-graft. The functionality of grafts was compared in terms of power loss (PL) and hepatic flow distribution (HFD), a known factor in PAVM development. To perform this study, ten single-ventricle children with Glenn physiology were included and a CFD model was developed to estimate the blood flow circulation to the left and right pulmonary arteries. The estimated blood flow by CFD was compared with that measured by cardiovascular magnetic resonance. Results showed that there was an excellent agreement between the net blood flow in the right and left pulmonary arteries computed by CFD and CMR (ICC= 0.98, P-value ≥0.21). After validating the accuracy of each CFD model, Fontan operations using T-shape and Y-graft conduits were performed in silico for each patient and the developed CFD model was used to predict the post-surgical PL and HFD. We found that the PL in the Y-graft was significantly lower than in the T-shape (P-value ≤0.001) and HFD was significantly better balanced in Y-graft compared to the T-shape (P-value=0.004).

Early Outcomes of the Norwood Procedure in a Reference Center in Brazil.

ResumoFundamento Apenas dois artigos abordam os resultados precoces de pacientes com síndrome do coração esquerdo hipoplásico (SHCE) submetidos à operação de Norwood, no Brasil.Objetivos Avaliamos pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood para identificar os fatores preditivos de mortalidade precoce (nos primeiros 30 dias após a cirurgia) e intermediária (desde a sobrevida precoce até o procedimento de Glenn).Métodos Foram incluídos pacientes com SHCE submetidos em nosso serviço ao primeiro estágio da operação de Norwood de janeiro de 2016 a abril de 2019. Dados demográficos, anatômicos e cirúrgicos foram analisados. Os desfechos foram mortalidade precoce (nos primeiros 30 dias após a cirurgia), mortalidade intermediária (desde a sobrevida precoce até o procedimento de Glenn) e a necessidade de suporte pós-operatório com ECMO. Foram realizadas análises univariadas e multivariadas e calculados odds ratios, com intervalos de confiança de 95%. Um valor de p < 0,05 foi considerado estatisticamente significativo.Resultados Um total de 80 pacientes com SHCE foram submetidos ao primeiro estágio da operação de Norwood. A taxa de sobrevida em 30 dias foi de 91,3% e a taxa de sobrevida intermediária foi de 81,3%. Quatorze pacientes (17,5%) necessitaram de suporte com ECMO. Menor peso (p=0,033), estenose aórtica (vs atresia aórtica; p=0,036) e necessidade de suporte pós-operatório com ECMO (p=0,009) foram fatores preditivos independentes para mortalidade em 30 dias. A estenose da valva mitral ( vs atresia da valva mitral; p=0,041) foi um fator preditivo independente para mortalidade intermediária.Conclusão O presente estudo inclui a maior coorte brasileira de pacientes com SHCE submetidos ao primeiro estágio da operação de Norwood na era recente. Nossas taxas de sobrevida foram comparáveis às mais altas taxas de sobrevida relatadas globalmente. Baixo peso corporal, estenose valvar aórtica e necessidade de suporte pós-operatório com ECMO foram preditores independentes para mortalidade em 30 dias. A estenose da valva mitral foi o único fator preditivo independente para mortalidade intermediária.

Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.

Recently, the outcome of severe Ebstein anomaly (EA) has improved with the prevalence of the Starnes procedure. However, time-dependent changes in the size of the central pulmonary artery (PA) have not been fully understood. A retrospective chart review of patients with EA who underwent the Starnes procedure during the neonatal period and those with pulmonary atresia with intact ventricular septum (PAIVS) was performed at Fukuoka Children's Hospital. There were 14 patients in the severe EA group and 36 in the PAIVS group, with mean observational periods of 3.8 and 4.2years, respectively. No significant difference in survival was observed between the groups. However, the mean size of the central PA was smaller in the severe EA group at each surgical stage (after systemic-to-pulmonary shunt, after the bidirectional Glenn procedure, and after the Fontan procedure). A significantly larger ventricular volume was observed in the severe EA group after the Fontan procedure. The growth of the central PA was poor in patients with severe EA. Patients with severe EA should be carefully monitored in this regard both before and after undergoing the Fontan procedure. Further studies regarding long-term prognosis are expected.

The role of neutrophil-to-lymphocyte ratio in predicting mortality after bidirectional Glenn procedure.

This study aimed to address the role of various inflammation-related blood indices for the assessment of in-hospital outcomes in subjects undergoing Glenn procedure. Subjects who underwent the Glenn procedure for hypoplastic left heart syndrome were analysed retrospectively. Subjects were divided into two groups: Group 1 consisted of 78 patients who were discharged, and Group 2 included 12 patients who died after surgery. Post-operative third-day neutrophil count and neutrophil-to-lymphocyte ratio value were significantly higher in the exitus group compared to the discharged group (p = 0.006 and p = 0.003, respectively). Third-day neutrophil-to-lymphocyte ratio was positively correlated with duration of intubation (r = 0.253, p = 0.018), length of stay in ICU (r = 0.296, p = 0.006) and length of hospital stay (r = 0.297, p = 0.005). Multiple logistic regression analysis revealed that patients with high third-day neutrophil-to-lymphocyte ratio (≥6) had 14.227-fold higher risk of death compared to those with lower values. In addition, higher pulmonary arterial pressure was associated with increased risk of death. Receiver operating characteristics analysis revealed that neutrophil-to-lymphocyte ratio had 66.67% sensitivity, 84% specificity, 81.61% accuracy, 40.00% positive predictive value and 94.03% negative predictive value with a cut-off point of ≥6 to predict mortality. Third-day neutrophil-to-lymphocyte ratio and increased post-operative pulmonary arterial pressure are significant predictors for in-hospital mortality in Glenn procedure recipients. A cut-off value of ≥6 for third-day neutrophil-to-lymphocyte ratio predicts mortality with 66.67% sensitivity and 84% specificity. Given its simplicity and availability, post-operative neutrophil-to-lymphocyte ratio should be monitored on a daily basis to identify patients with high risk for mortality after Glenn procedure.

New trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques.

Ebstein's anomaly,a rare cardiac disease that accounts for <1% of all congenital heart diseases, is a right ventricular myopathy with varying degrees of delamination in the endocardium under the tricuspid valve. Cone reconstruction, first described by da Silva in 2004, is a modification of the Carpentier technique. An alternative surgical treatment for Ebstein's anomaly, it is now the preferred surgical corrective technique. This study demonstrates that our modifications of the cone repair procedure to correct Ebstein's anomaly improve valve coaptation and stabilization in the early and midterm. Of the 134 patients diagnosed with Ebstein's anomaly in our clinic between January 2012 and October 2020, 10 underwent a cone procedure and its modifications and were thus included in the study. The mean age of these patients was 28.6 ± 18.79 years and ranged from 1 to 61 years. Thirty percent of the patients were male and 70% were female. The additional procedures performed were an isolated cone procedure (two patients), anomalous pulmonary venous return repair in addition to a cone procedure (one patient), single-vessel coronary artery bypass (one patient), Glenn procedure (two patients), and tricuspid ring annuloplasty (four patients). Based on the results of this study, which assesses the outcomes of patients who underwent cone repairs with surgical modifications, we argue that right ventricular oblique plication, tricuspid valve delamination, surgical valve rotation, and tricuspid ring annuloplasty protect valve coaptation and function in the early and midterm, thereby having beneficial effects on right ventricle remodeling.

Fontan-associated liver disease.

Fontan-associated liver disease.