Bidirectional Glenn Procedure Research Articles

Collateral-Induced Hypoxemia After Bidirectional Glenn Procedure

Collateral-Induced Hypoxemia After Bidirectional Glenn Procedure

Comparison of definitive approaches for conotruncal defects following bidirectional Glenn procedure

BackgroundStaged repair is common for complex conotruncal defects, often involving bidirectional Glenn (BDG) procedure. Following the cavopulmonary shunt, both Fontan completion and biventricular conversion (BiVC) serve as definitive approaches. The...

Outcomes of single-ventricle physiology in central South Africa.

Single-ventricle physiology is a critical cardiac condition requiring early diagnosis and intervention. The objectives of this study were to report on the management and outcomes of patients diagnosed with single-ventricle physiology in central South Africa. This study was a retrospective, observational analysis of patients presenting with single-ventricle physiology at the Universitas Academic Hospital in central South Africa between November 1997 and June 2021. Patients were referred from the Free State (54%) and Northern Cape (29%) provinces and Lesotho. One hundred and fifty-four patients presented with single-ventricle physiology: 114 received interventions and 40 were not eligible for intervention. Patients presented for the first time at a median age of 34.5 days, with patients from nearby districts presenting within a few days of birth. However, patients from outlying areas presented much later. Eighty-seven patients received systemic-to-pulmonary artery shunting or pulmonary artery banding. Sixty-three patients proceeded to bidirectional Glenn procedures, and 30 patients (26%) had full palliation to Fontan. Twenty-one patients died after stage 1, six after the Glenn procedure and two after the Fontan procedure. Overall, 34 (29.8%) patients were lost to follow up. Patients in our study presented late and follow up of these patients was a challenge. The highest mortality rate occurs during the first stage of palliation. Outcomes from this study are comparable to other sub-Saharan studies.

Pulmonary artery coarctation repair in univentricular heart: indications and optimal timing.

To analyse surgical outcomes of pulmonary artery coarctation in univentricular hearts, focusing on surgical indications and optimal timing. We retrospectively reviewed 49 patients with pulmonary artery coarctation in univentricular hearts treated at our institution between 1993 and 2022. Twenty-eight patients were diagnosed before first-stage palliation. Of these, 14 underwent systemic-pulmonary shunt only as first-stage palliation (Group 1), and 14 underwent systemic-pulmonary shunt plus surgical pulmonary artery plasty as first-stage palliation (Group 2). Twenty-one patients diagnosed after first-stage palliation underwent surgical pulmonary artery plasty at the time of bidirectional Glenn procedure (Group 3). Follow-up period after initial palliation was 6±8 years. The Fontan procedure was successful in 35 patients (71%) aged 28±26 months (range 18-139). Freedom from interstage death (Group 1, 53%; Group 2, 85%; Group 3, 93%) and interstage reintervention (Group 1, 50%; Group 2, 75%; Group 3, 73%) rates were significantly lower in Group 1 (p = 0.01). Five and four patients in Group 1 and Group 3, respectively, needed additional shunts before the bidirectional Glenn procedure. In Group 1, one patient with a non-confluent pulmonary artery achieved hemi-lung Fontan circulation. In Group 2, one patient suffering with a non-confluent pulmonary artery could not achieve Fontan circulation, whereas another patient with pulmonary venous obstruction achieved hemi-lung Fontan circulation. Surgical pulmonary artery plasty performed at first-stage palliation improved outcomes of pulmonary artery coarctation in univentricular hearts, particularly when pulmonary artery coarctation had already progressed during the neonatal period or early infancy.

Factors affecting the outcomes after bidirectional Glenn shunt: two decades of experience from a tertiary referral center

BackgroundDespite the improved management of patients with a single ventricle, the long-term outcomes are not optimal. We reported the outcomes of the bidirectional Glenn procedure (BDG) and factors affecting the length of hospital stay, operative mortality, and Nakata index before Fontan completion.ResultsThis retrospective study included 259 patients who underwent BDG shunt from 2002 to 2020. The primary study outcomes were operative mortality, duration of hospital stay, and Nakata index before Fontan. Mortality occurred in 10 patients after BDG shunt (3.86%). By univariable logistic regression analysis, postoperative mortality after BDG shunt was associated with high preoperative mean pulmonary artery pressure (OR: 1.06 (95% CI 1.01–1.23); P = 0.02). The median duration of hospital stay after BDG shunt was 12 (9–19) days. Multivariable analysis indicated that Norwood palliation before BDG shunt was significantly associated with prolonged hospital stay (β: 0.53 (95% CI 0.12–0.95), P = 0.01). Fontan completion was performed in 144 patients (50.03%), and the pre-Fontan Nataka index was 173 (130.92–225.34) mm2/m2. Norwood palliation (β: − 0.61 (95% CI 62.63–20.18), P = 0.003) and preoperative saturation (β: − 2.38 (95% CI − 4.49–0.26), P = 0.03) were inversely associated with pre-Fontan Nakata index in patients who had Fontan completion.ConclusionsBDG had a low mortality rate. Pulmonary artery pressure, Norwood palliation, cardiopulmonary bypass time, and pre-BDG shunt saturation were key factors associated with post-BDG outcomes in our series.

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.

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.

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.

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

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.

Extracorporeal membrane oxygenator as a bridge to heart–liver en bloc transplant in a patient with Fontan circulation

Extracorporeal membrane oxygenator as a bridge to heart–liver en bloc transplant in a patient with Fontan circulation

Characterization of the Ejector Pump Performance for the Assisted Bidirectional Glenn Procedure

This study introduces an algebraic model informed by computational fluid dynamics (CFD) simulations to investigate the performance of the assisted bidirectional Glenn (ABG) operation on a broad range of conditions. The performance of this operation, as measured by the superior vena cava (SVC) pressure, depends on the nozzle area in its ejector pump and the patient’s pulmonary vascular resistance (PVR). Using the developed algebraic model to explore this two-dimensional parameter space shows that the ejector pump can create a pressure difference between the pulmonary artery and the SVC as high as 5 mmHg. The lowest SVC pressure is produced at a nozzle area that decreases linearly with the PVR such that, at PVR =4.2 (Wood units-m2), there is no added benefit in utilizing the ejector pump effect (optimal nozzle area is zero, corresponding to the bidirectional Glenn circulation). At PVR =2 (Wood units-m2), the SVC pressure can be lowered to less than 4 mmHg by using an optimal nozzle area of ≈2.5 mm2. Regardless of the PVR, adding a 2 mm2 nozzle to the baseline bidirectional Glenn boosts the oxygen saturation and delivery by at least 15%. The SVC pressure for that 2 mm2 nozzle remains below 14 mmHg for all PVRs less than 7 Wood units-m2. The mechanical efficiency of the optimal designs consistently remains below 30%, indicating the potential for improvement in the future. A good agreement is observed between the algebraic model and high-fidelity CFD simulations.

The Glenn Shunt Revisited, A Single Center Registry in Ain Shams University Cardiology Department

Background: Bidirectional Glenn shunts have long been available as palliative procedures for patients with single ventricle physiology that is, patients unsuitable for biventricular repair. In our country they are performed at an older age than that recommended by the literature. So, we aim to assess post bidirectional Glenn shunt patients to detect the presence of any complications and to evaluate their functional capacity. Methods: This was a descriptive study that included all patients who underwent a bidirectional Glenn shunt and were referred for follow up in Ain shams university hospitals. History taking including NYHA class and physical activity, clinical examination, six-minutes-walk test, laboratory investigations, full echocardiographic assessment were done for all patients. Some patients needed invasive cardiac catheterization. Results: Our registry included 178 patients who underwent bidirectional Glenn procedure referred for follow up in Ain Shams university hospital from January 2019 till July 2020. The mean age of the registry was 18.7 ± 8.26 years (range between 5 and 37 years), 85 males (48%) and 93 females (52%). Regarding the basic anatomy, double outlet right ventricle with hypoplastic left ventricle was the commonest. Furthermore, our descriptive study confirmed many characteristic similarities between our patients and patients in developing countries. Our patients underwent bidirectional Glenn shunt at a median age of 6 years which is considered a relatively old age but similar to other studies that have been made in developing countries like Pakistan, India and Iran. There is a significant delay in the operation in Egyptian patients due to lack of patients’ awareness, few numbers of primary health care facilities and high economic burden. Conclusion: Although the presence of slight systemic desaturation, our study demonstrated a satisfactory functional capacity among our patients; thus, bidirectional Glenn can be considered an acceptable definite univentricular repair in patients with late presentation.

Value of Follow-Up N-Terminal Probrain Natriuretic Peptide (NT-proBNP) after a Modified Fontan Procedure.

Objective To assess the value of N-terminal probrain natriuretic peptide (NT-proBNP) in short-term and long-term follow-up after a modified Fontan procedure. Methods We retrospectively enrolled children who had undergone a modified Fontan procedure in the Heart Center of Guangzhou Women and Children's Medical Center from January 2014 to September 2020 and collected data on NT-proBNP values before bidirectional Glenn procedure, before Fontan procedure, and on 1, 3, 7, 30, 90, and 180 days and 1, 2, 3, 4, 5, and 6 year after Fontan procedure. The relationship between changes in NT-proBNP levels and the outcomes in children was analyzed. Results A total of 108 children (78 boys and 30 girls, mean age: 54.62 ± 29.38 weeks) were included in the analysis. According to one-way analysis of variance, the left ventricular type and biventricular type of single ventricle physiology showed shorter duration on cardiopulmonary bypass during the operation and lower levels of NT-proBNP after the operation than the right ventricular type and univentricular type physiology. Conclusion NT-proBNP is a good indicator for mid and long-term follow-up after a modified Fontan procedure. The left ventricular type and biventricular type of single ventricle physiology show better mid and long-term benefits from the modified Fontan procedure than the right ventricular type and univentricular type physiology.

Acute neurological injury in pediatric patients with single-ventricle congenital heart disease.

Single-ventricle congenital heart disease (CHD) in pediatric patients with Glenn and Fontan physiology represents a unique physiology requiring the surgical diversion of the systemic venous return from the superior vena cava (Glenn) and then the inferior vena cava (Fontan) directly to the pulmonary arteries. Because many of these patients are on chronic anticoagulation therapy and may have right-to-left shunts, arrhythmias, or lymphatic disorders that predispose them to bleeding and/or clotting, they are at risk of experiencing neurological injury requiring intubation and positive pressure ventilation, which can significantly hamper pulmonary blood flow and cardiac output. The aim of this study was to describe the complex neurological and cardiopulmonary interactions of these pediatric patients after acute central nervous system (CNS) injury. The authors retrospectively analyzed the records of pediatric patients who had been admitted to a quaternary children's hospital with CHD palliated to bidirectional Glenn (BDG) or Fontan circulation and acute CNS injury and who had undergone intubation and mechanical ventilation. Patients who had been admitted from 2005 to 2019 were included in the study. Clinical characteristics, surgical outcomes, cardiovascular and pulmonary data, and intracranial pressure data were collected and analyzed. Nine pediatric single-ventricle patients met the study inclusion criteria. All had undergone the BDG procedure, and the majority (78%) were status post Fontan palliation. The mean age was 7.4 years (range 1.3-17.3 years). At the time of acute CNS injury, which included traumatic brain injury, intracranial hemorrhage, and cerebral infarct, the median time interval from the most recent cardiac surgical procedure was 3 years (range 2 weeks-11 years). Maintaining normocarbia to mild hypercarbia for most patients during intubation periods did not cause neurological deterioration, and hemodynamic profiles were more favorable as compared to periods of hypocarbia. Hypocarbia was associated with unfavorable hemodynamics but was necessary to decrease intracranial hypertension. Most patients were managed using low mean airway pressure (MAWP) in order to minimize the impact on preload and cardiac output. The authors highlight the complex neurological and cardiopulmonary interactions with respect to partial pressure of arterial CO2 (PaCO2) and MAWP when pediatric CHD patients with single-ventricle physiology require mechanical ventilation. The study data demonstrated that tight control of PaCO2 and minimizing MAWP with the goal of early extubation may be beneficial in this population. A multidisciplinary team of pediatric critical care intensivists, cardiac intensivists and anesthesiologists, and pediatric neurosurgeons and neurologists are recommended to ensure the best possible outcomes.

Early outcomes after the on pump bidirectional Glenn procedure: A single center experience.

Bidirectional Glenn procedure is a staged palliative procedure for patients with the univentricular hearts or complex congenital heart disease. We in our study, attempted to evaluate the preoperative characteristics, operative data and the early postoperative outcomes in the patients who underwent Bidirectional Glenn procedure at our center. In our single center retrospective experience, 115 patients underwent Bidirectional Glenn procedure from January 2015 to December 2019. The mean age of the patients was 6.55 ± 6.5 years (range from 9 months to 48 years) and a median of 5 years. The most common anatomic diagnosis was double outlet right ventricle (n = 49, 42.6%). The primary diagnosis and the additional cardiac anamolies were not associated with the adverse outcomes. The increased cardiopulmonary bypass and operative time affect the postoperative outcomes. The median oxygen saturation in the patients postoperatively was 82%. The median postoperative stay was 8 days. The early postoperative complications were seen in 29 patients (25.2%). There were 12 early deaths (10.4%) in our study. The late age of presentation and poor preoperative nutrition, increased the risk of the postoperative morbidity and mortality. Bidirectional Glenn procedure is an effective procedure to improve efficacy of the gas exchange and reduce volume overload on the single ventricle at early as well as late stages. However, the late age of presentation increases the risk of the postoperative outcomes.

Commentary: The bidirectional Glenn procedure can be useful in palliating late-presenting patients with complex cyanotic congenital heart disease.

Journal of Cardiac SurgeryVolume 36, Issue 9 p. 3215-3216 COMMENTARY Commentary: The bidirectional Glenn procedure can be useful in palliating late-presenting patients with complex cyanotic congenital heart disease John J. Lamberti MD, Corresponding Author John J. Lamberti MD jlamberti@stanford.edu orcid.org/0000-0002-7182-7414 Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Stanford University and Lucile Packard Children's Hospital, Falk Cardiovascular Research Center, Stanford, California, USA Correspondence John J. Lamberti, MD, Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Stanford University and Lucile Packard Children's Hospital, 300 Pasteur Dr., Falk Cardiovascular Research Center, Stanford, CA 94305-5407, USA. Email: jlamberti@stanford.eduSearch for more papers by this author John J. Lamberti MD, Corresponding Author John J. Lamberti MD jlamberti@stanford.edu orcid.org/0000-0002-7182-7414 Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Stanford University and Lucile Packard Children's Hospital, Falk Cardiovascular Research Center, Stanford, California, USA Correspondence John J. Lamberti, MD, Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Stanford University and Lucile Packard Children's Hospital, 300 Pasteur Dr., Falk Cardiovascular Research Center, Stanford, CA 94305-5407, USA. Email: jlamberti@stanford.eduSearch for more papers by this author First published: 06 June 2021 https://doi.org/10.1111/jocs.15723Citations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume36, Issue9September 2021Pages 3215-3216 RelatedInformation

Changes in Heart Rate and Heart Rate Variability During Surgical Stages to Completed Fontan Circulation

Arrhythmia is related to heart rate variability (HRV), which reflects the autonomic nervous regulation of the heart. We hypothesized that autonomic nervous ganglia, located at the junction of the superior vena cava’s entrance to the heart, may be affected during the bidirectional Glenn procedure (BDG), resulting in reduced HRV. We aimed to investigate changes in heart rate and HRV in a cohort of children with univentricular heart defects, undergoing stepwise surgery towards total cavopulmonary connection (TCPC), and compare these results with healthy controls. Twenty four hours Holter-ECG recordings were obtained before BDG (n = 47), after BDG (n = 47), and after total cavopulmonary connection (TCPC) (n = 45) in patients and in 38 healthy controls. HRV was analyzed by spectral and Poincaré methods. Age-related z scores were calculated and compared using linear mixed effects modeling. Total HRV was significantly lower in patients before BDG when compared to healthy controls. The mean heart rate was significantly reduced in patients after BDG compared to before BDG. Compared to healthy controls, patients operated with BDG had significantly reduced heart rate and reduced total HRV. Patients with TCPC showed reduced heart rate and HRV compared with healthy controls. In patients after TCPC, total HRV was decreased compared to before TCPC. Heart rate was reduced after BDG procedure, and further reductions of HRV were seen post-TCPC. Our results indicate that autonomic regulation of cardiac rhythm is affected both after BDG and again after TCPC. This may be reflected as, and contribute to, postoperative arrhythmic events.