Sleep-disordered breathing and lung function abnormalities in adults with congenital heart disease.

PURPOSE: Advances in treatment enables most patients with congenital heart diseases (CHD) to survive into adulthood, implying the need to address comorbid conditions in this growing cohort of patients. The aim of this study was to evaluate the prevalence of sleep-disordered breathing (SDB) and lung function abnormalities in patients with adult congenital heart disease (ACHD). METHODS: Patients with ACHD underwent level 3 sleep testing (Embletta MPR polygraphy) and pulmonary function testing. Results were stratified by the underlying haemodynamic ACHD lesion group. RESULTS: Patients with ACHD (n = 100) were middle-aged (42.3 ± 14.6 years), 54% male and slightly overweight (BMI 25.9 ± 5.5 kg/m2). Polygraphy revealed a prevalence of sleep apnoea of 39% with 15% of patients presenting with predominantly obstructive apnoeic episodes, while 23% of patients presenting primarily with central sleep apnoea. The distribution of mild, moderate, and severe sleep apnoea in the total study population was 26%, 7% and 6%, respectively. Comparison of apnoea-hypopnoea index, presence of sleep apnoea, and apnoea severity did not offer significant differences between the four ACHD lesion groups (p = 0.29, p = 0.41 and p = 0.18, respectively). Pulmonary function testing revealed obstructive lung disease in 19 of 100 patients. Concomitant chronic obstructive pulmonary disease and obstructive sleep apnoea were diagnosed in 3% of patients and were associated with profound nocturnal desaturation. CONCLUSION: The findings suggest a mild propensity amongst patients with ACHD to develop SDB that seems to be unaffected by the specific underlying congenital lesion.

How grit mediates depressive symptoms with life and job satisfaction in full-time employed adults with congenital heart disease.

BACKGROUND: Technological advancements have extended the lifespan of individuals with congenital heart disease (CHD), but physical and mental health issues can affect their life and job satisfaction. AIMS: This study examined whether grit can protect full-time employed adults with CHD from depression by exploring its mediating role between depressive symptoms and life or job satisfaction. METHODS: A cross-sectional design involved 181 adults with CHD assessed using the Patient Health Questionnaire, Grit-10, Satisfaction with Life Scale, and Basic Psychological Need Satisfaction at Work Scale. Data analysis was conducted with SPSS and Smart PLS software for partial least squares structural equation modeling, adhering to the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines. RESULTS: Indicated that depressive symptoms were negatively correlated with grit, life satisfaction, and job satisfaction, while grit was positively correlated with both satisfaction measures. Grit partially mediated the relationship between depressive symptoms and life/job satisfaction, accounting for 30.70 % and 29.11 % of the variance, respectively. CONCLUSIONS: Grit significantly mitigates the negative impact of depressive symptoms on life and job satisfaction in full-time employed adults with CHD. Nurses should identify signs of depression in adults with CHD and evaluate their grit levels. Interventions to increase grit and reduce depressive symptoms could enhance life and job satisfaction.

Socio-demographic parameters and non-cardiac comorbidity related to self-perceived quality of life in young adults after neonatal arterial switch operation for transposition of the great arteries.

Evaluating the relation of non-cardiac comorbidity and socio-demographic factors to physical and mental health-related quality of life (QOL) which has been partially found at elevated risk in young adults after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA). In a prospective reassessment study, results of 92 unselected young adults (22.8 ± 2.6 years) having undergone evaluation of QOL (SF-36) were related to non-cardiac comorbidity with special respect to neurologic and psychiatric comorbidity and to socio-demographic parameters. Neurologic (14%) contrary to psychiatric comorbidities (6.5%) were more frequent than in the general population. The educational level was higher, the rate of unemployment was double as high compared to the average German population. Significant inverse relations (p = 0.006 to 0.033) existed between physical health domains (physical functioning and general health perception) and non-cardiac, neurologic, and psychiatric comorbidity, as well as correlations between the latter domains and socio-economic status, educational level, and worse employment status (Spearman 0.22-0.41, p < 0.0001 to 0.036). Mental health domains (vitality, social functioning, psychical health) were significantly inversely related with neurologic and psychiatric comorbidity (p = 0.002 to 0.048) and correlated with higher educational level (Spearman 0.25, p = 0.019). Neurologic and psychiatric comorbidities and socio-demographic parameters are significant risk factors for a reduced QOL concerning physical and mental health in young adults with TGA after ASO. Standardized QOL measurement should be part of routine screening programs to detect subclinical physical, neurodevelopmental, and psychosocial comorbidity.

Contraception and Cardiovascular Effects: What Should the Cardiologist Know?

PURPOSE OF REVIEW: Cardiovascular disease (CVD) is the leading cause of death in women. This review highlights contraceptive options and their effects on the cardiovascular system (CVS). It provides guidance to cardiologists to make informed decisions regarding the safety of contraceptive use and cardiovascular risk stratification in the care of women of childbearing age. RECENT FINDINGS: Approximately 44% of American women live with some type of CVD. Many women use hormonal contraception during their lifetime. It is imperative that cardiologists have a robust understanding of the forms of contraception in current use and their cardiovascular effects. This contemporary review provides a comprehensive summary of available contraceptive methods to practicing cardiologists and aims to be used as a resource to guide cardiovascular specialists on contraception in the context of cardiovascular disease.

Multimodality Imaging in Ebstein Anomaly.

Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine.

Exception-Status Listing: A Critical Pathway to Heart Transplantation for Adults With Congenital Heart Diseases.

Adults with congenital heart diseases may not be candidates for conventional therapies to control ventricular systolic dysfunction, including mechanical circulatory support, which moves potential heart-transplantation recipients to a listing status of higher priority. This results in longer waitlist times and greater mortality rates. Exception-status listing allows a pathway for this complex and anatomically heterogenous group of patients to be listed for heart transplantation at appropriately high listing status. Our study queried the United Network for Organ Sharing registry to evaluate trends in the use of exception-status listing among adults with congenital heart diseases awaiting heart transplantation. Uptrend in the use of exception-status listing precedes the new allocation system, but it has been greatest since changes were made in the allocation system. It continues to remain a vital pathway for adults with congenital heart disease (whose waitlist mortality rates are often not characterized adequately by using the waitlist-status criteria) timely access to heart transplantation.

Bioelectrical Impedance Analysis as a Contemporary Biomarker of Obesity in Adults with Marfan- or Loeys-Dietz-Syndrome.

Background: It is clinically widely overlooked that many patients with Marfan- (MFS) or Loeys-Dietz-Syndrome (LDS) are obese. While anthropometric routine parameters are not very suitable, the modern Bioelectrical Impedance Analysis (BIA) seems superior for the acquisition of reliable noninvasive assessment of body composition of patients. The aim of the study was to assess the body composition of patients with MFS/LDS by BIA in order to detect occult obesity, which may be a risk marker for aortic or vascular complications. Methods: In this exploratory cross-sectional study, 50 patients (66% female; mean age: 37.7 ± 11.7 [range: 17-64] years) with a molecular genetic (n = 45; 90%) or clinical (n = 5; 10%) proven diagnosis of MFS or LDS were enrolled between June 2020 and February 2022. All BIA-measurements were performed with the Multifrequence-Impedance-Analyzer Nutriguard-MS (Data Input, Poecking, Germany). Results: The MFS/LDS collective was significantly different from an age-, sex-, and BMI-adjusted control in terms of body fat, percent cellularity, body cell mass, extra cellular mass/body cell mass index, and phase angle (all p < 0.05). The mean BIA-measured bodyfat was 31.7 ± 8.7% [range: 9.5-53.5%], while the mean calculated BMI of the included patients was 23.0 ± 4.8 kg/ m 2 [range: 15.2-41.9 kg/ m 2 ]. Therefore, using the obesity cut-off values for the body fat percentage of 25% in men and 35% in women, the BIA classifies as many as 28 patients (56.0%) as obese. In contrast only 12 patients (24.0%) were pre-obese, respectively 3 (6.0%) obese by BMI. The significant difference (p < 0.001) had an accordance of 42.7%. Overall, 15 patients (13 MFS; 2 LDS) had previous aortic surgery (n = 14) and/or interventional treatment (n = 2) for aortic complications (aneurysm, aortic dissection). 11 out of these 15 (73.3%) were currently classified as obese by BIA. Conclusions: The fact that many patients with MFS or LDS are obese is widely unknown, although obesity may be associated with impaired vascular endothelial function and an increased risk of cardiovascular complications. Also, in patients with MFS/LDS, BIA allows a reliable assessment of the body composition beyond the normal anthropometric parameters, such as BMI. In the future, BIA-data possibly may be of particular importance for the assessment of the vascular risk of MFS/LDS patients, besides the aortic diameters.

Phenotypes of adults with congenital heart disease around the globe: a cluster analysis.

OBJECTIVE: To derive cluster analysis-based groupings for adults with congenital heart disease (ACHD) when it comes to perceived health, psychological functioning, health behaviours and quality of life (QoL). METHODS: This study was part of a larger worldwide multicentre study called APPROACH-IS; a cross sectional study which recruited 4028 patients (2013-2015) from 15 participating countries. A hierarchical cluster analysis was performed using Ward's method in order to group patients with similar psychological characteristics, which were defined by taking into consideration the scores of the following tests: Sense Of Coherence, Health Behavior Scale (physical exercise score), Hospital Anxiety Depression Scale, Illness Perception Questionnaire, Satisfaction with Life Scale and the Visual Analogue Scale scores of the EQ-5D perceived health scale and a linear analogue scale (0-100) measuring QoL. RESULTS: 3768 patients with complete data were divided into 3 clusters. The first and second clusters represented 89.6% of patients in the analysis who reported a good health perception, QoL, psychological functioning and the greatest amount of exercise. Patients in the third cluster reported substantially lower scores in all PROs. This cluster was characterised by a significantly higher proportion of females, a higher average age the lowest education level, more complex forms of congenital heart disease and more medical comorbidities. CONCLUSIONS: This study suggests that certain demographic and clinical characteristics may be linked to less favourable health perception, quality of life, psychological functioning, and health behaviours in ACHD. This information may be used to improve psychosocial screening and the timely provision of psychosocial care.

Quality of life and health status of hospitalized adults with congenital heart disease in Vietnam: a cross-sectional study.

BACKGROUND: Little is known about the quality of life (QOL) and health status of adults with congenital heart disease (CHD) in developing countries. Therefore, this study aimed to describe the QOL and health status of hospitalized adults with CHD in Vietnam and investigate the association between QOL and their biological-social characteristics. METHODS: A cross-sectional study was conducted with 109 adults with CHD, hospitalized in the Vietnam National Heart Institute, between June and December 2019. Validated instruments to assess QOL and health status describing patient-reported outcomes were used, including the EuroQOL-5 Dimensions-5 Level, Satisfaction with Life Scale, and Hospital Anxiety and Depression Scale. RESULTS: The mean scores on the EuroQOL-descriptive system (EQ-DS) and EuroQOL visual analogue scale (EQ-VAS) were 0.792 (SD = 0.122, 95% confidence interval [CI] 0.769-0.815) and 66.3 (SD = 12.5, 95% CI 63.9-68.7), respectively. A total of 9.2% (n = 9) patients experienced life dissatisfaction. The prevalence of anxiety and depression were 18.7% (n = 20) and 11% (n = 12), respectively. Scores of QOL in patients aged > 30 years were lower than in those aged ≤ 30 years. Stratified multivariate logistic regression revealed that poor QOL related to being unemployed/unstable employment (OR 4.43, 95% CI 1.71-11.47, p = 0.002), life dissatisfaction associated with unmarried status (OR 4.63, 95% CI 1.2-17.86, p = 0.026), anxiety regarding unemployment/unstable employment (OR 3.88, 95% CI 1.27-11.84, p = 0.017) and complex CHD/PAH (OR 4.84, 95% CI 1.33-17.54, p = 0.016), and depression regarding unemployment/unstable employment (OR 4.63, 95% CI 1.22-17.59, p = 0.003). CONCLUSIONS: Reduced QOL and elevated psychological problems were common experiences among hospitalized adults with CHD in Vietnam. Biological-social characteristics such as unmarried status, unemployment/unstable employment, and complex CHD/PAH related to poor QOL, life dissatisfaction, anxiety, and depression.

Heart failure in adult congenital heart disease: tetralogy of Fallot.

The number of rTOF patients who survive into adulthood is steadily rising, with currently more than 90% reaching the third decade of life. However, rTOF patients are not cured, but rather have a lifelong increased risk for cardiac and non-cardiac complications. Heart failure is recognized as a significant complication. Its occurrence is strongly associated with adverse outcome. Unfortunately, conventional concepts of heart failure may not be directly applicable in this patient group. This article presents a review of the current knowledge on HF in rTOF patients, including incidence and prevalence, the most common mechanisms of heart failure, i.e., valvular pathologies, shunt lesions, left atrial hypertension, primary left heart and right heart failure, arrhythmias, and coronary artery disease. In addition, we will review information regarding extracardiac complications, risk factors for the development of heart failure, clinical impact and prognosis, and assessment possibilities, particularly of the right ventricle, as well as management strategies. We explore potential future concepts that may stimulate further research into this field.

From Other Journals: A Review of Recent Articles in Pediatric Cardiology.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) pulmonary valve repair in patients with tetralogy of Fallot and in patients with congenital pulmonary stenosis which seems to be a promising technique in selected patients, (2) the use of Losartan in patients with Marfan syndrome mostly in addition to beta blockers may result in a lower aortic root dilation rate and better clinical outcomes, (3) a summary of the characteristics of adults with congenital heart disease in the USA showed a wide variation in prevalence, associated morbidities, health care utilization and insurance type in different locations in the USA, (4) a large single center study confirmed that right ventricular morphology is associated with worse outcomes after the Fontan operation and that atrioventricular valve regurgitation is an important predictor of worse outcomes and precedes ventricular dysfunction post Fontan operation, (5) a large study describing the multi system inflammatory syndrome in children temporarily related to the COVID-19 pandemic in the USA showing that ventricular dysfunction is the most common cardiac manifestation (6) the reality of "limping to transplantation" showing that patients with one or more modifiable risk factors including mechanical ventilation, kidney or liver dysfunction are at significant increase risk post cardiac transplantation.

State-of-the-art multimodality approach to assist ablations in complex anatomies-From 3D printing to virtual reality.

Imaging of the heart anatomy plays an important role, especially in catheter ablation for the treatment of arrhythmias in adults with congenital heart disease (ACHD). We present a comprehensive overview of the current state-of-the-art modalities available to plan and guide catheter ablation in an ACHD patient. In addition to the clinical assessment of the computed tomography and the integration of 3D reconstructions into the electroanatomical mapping system, 3D printing and virtual reality assessment showed its value in preprocedural planning of the intervention.

Congenital Heart Disease in Low- and Lower-Middle-Income Countries: Current Status and New Opportunities.

PURPOSE OF REVIEW: The paper summarises the most recent data on congenital heart disease (CHD) in low- and lower-middle-income countries (LLMICs). In addition, we present an approach to diagnosis, management and interventions in these regions and present innovations, research priorities and opportunities to improve outcomes and develop new programs. RECENT FINDINGS: The reported birth prevalence of CHD in LLMICs is increasing, with clear evidence of the impact of surgical intervention on the burden of disease. New methods of teaching and training are demonstrating improved outcomes. Local capacity building remains the key. There is a significant gap in epidemiological and outcomes data in CHD in LLMICs. Although the global agenda still does not address the needs of children with CHD adequately, regional initiatives are focusing on quality improvement and context-specific interventions. Future research should focus on epidemiology and the use of innovative thinking and partnerships to provide low-cost, high-impact solutions.

Cardiopulmonary Exercise Testing for Surgical Risk Stratification in Adults with Congenital Heart Disease.

Adult congenital heart disease (ACHD) patients often require repeat cardiothoracic surgery, which may result in significant morbidity and mortality. Currently, there are few pre-operative risk assessment tools available. In the general adult population, pre-operative cardiopulmonary exercise testing (CPET) has a predictive value for post-operative morbidity and mortality following major non-cardiac surgery. The utility of CPET for risk assessment in ACHD patients requiring cardiothoracic surgery has not been evaluated. Retrospective chart review was conducted on 75 ACHD patients who underwent CPET less than 12 months prior to major cardiothoracic surgery at Children's Hospital of Wisconsin. Minimally invasive procedures, cardiomyopathy, acquired heart disease, single ventricle physiology, and heart transplant patients were excluded. Demographic information, CPET results, and peri-operative surgical data were collected. The study population was 56% male with a median age of 25 years (17-58). Prolonged post-operative length of stay correlated with increased ventilatory efficiency slope (VE/[Formula: see text] slope) (P = 0.007). Prolonged intubation time correlated with decreased peak HR (P = 0.008), decreased exercise time (P = 0.002), decreased heart rate response (P = 0.008) and decreased relative peak oxygen consumption (P = 0.034). Post-operative complications were documented in 59% of patients. While trends were noted between post-operative complications and some measurements of exercise capacity, none met statistical significance. Future studies may further define the relationship between exercise capacity and post-operative morbidity in ACHD patients.

Surgery for supraventricular tachycardia and congenital heart defects: long-term efficacy of the combined approach in adult patients.

AIMS: Supraventricular arrhythmias are a major cause of morbidity and mortality in adult patients with congenital heart disease (CHD). Intraoperative ablation offers an alternative for patients who failed ablation procedures or are requiring concomitant surgical intervention. We present our long-term results with the surgical treatment of arrhythmias in adults with CHD (ACHD) undergoing elective cardiac surgery and the clinical predictors for arrhythmia recurrence. METHODS AND RESULTS: Between 2002 and 2013, 80 consecutive patients with CHD, mean age of 39 years, underwent intraoperative ablation with monopolar irrigated radiofrequency during cardiac surgery procedures. Significant clinical predictors of arrhythmia recurrence were determined by univariate analysis. We performed 47 right-sided Maze procedures, and 33 Cox-Maze III procedures. In 75 survivors, the ablation was effective immediately. Over an average follow-up period of 72 months (12-155 months), arrhythmias recurred in nine (20%) patients after right-sided Maze, and in six (19%) patients after Cox-Maze III. Eleven patients were controlled with medical therapy, three underwent catheter ablation of the arrhythmia, and one required a permanent pacemaker. Preoperative arrhythmia length ≥3 years (P ≤ 0.001), tetralogy of Fallot (P ≤ 0.006), and preoperative atrial fibrillation (P ≤ 0.016) were associated with recurrence arrhythmia. Conversely, NYHA class <3 (P ≤ 0.047) was associated with a lower risk of recurrence. CONCLUSION: Surgical treatment of unresponsive supraventricular arrhythmia during concomitant cardiac surgery in ACHD is a safe and effective procedure. Freedom from arrhythmias recurrence is 75% after 6 years of follow-up. Long-term recurrence of arrhythmia in these patients seems to be strongly correlated to preoperative arrhythmia duration.

Evaluation of cardiac electrophysiological properties in an experimental model of right ventricular hypertrophy and failure.

BACKGROUND: Malignant arrhythmias are a major cause of sudden cardiac death in adults with congenital heart disease. We developed a model to serially investigate electrophysiological properties in an animal model of right ventricular hypertrophy and failure. METHOD: We created models of compensated (cHF; n=11) and decompensated (dHF; n=11) right ventricular failure in Wistar rats by pulmonary trunk banding. Healthy controls underwent sham operation (Control; n=13). Surface electrocardiography was recorded from extremities, and inducibility of ventricular tachycardia was evaluated in vivo by programmed stimulation. Isolated right ventricular myocardium was analysed for mRNA expression of selected genes. RESULTS: Banding caused an increased mRNA expression of both connexin 43 and the voltage-gated sodium channel 1.5, as well as a prolongation of PQ, QRS and QTc intervals. Ventricular tachycardia was induced in the majority of banded animals compared with none in the healthy control group. No differences were found between the two degrees of failure in neither the electrophysiological parameters nor inducibility. CONCLUSIONS: The electrophysiological properties of rat hearts subjected to pulmonary trunk banding were significantly changed with increased susceptibility to ventricular tachycardia, but no differences were found between compensated and decompensated right ventricular failure. Furthermore, we demonstrate that in vivo electrophysiological evaluation is a sensitive method to characterise the cardiac electric phenotype in an experimental rat model.

Lesion-specific differences for implantable cardioverter defibrillator therapies in adults with congenital heart disease.

BACKGROUND: Sudden cardiac death is a major cause of late mortality in adults with congenital heart disease (ACHD). While data exist for adults with repaired Tetralogy of Fallot (TOF), little is known about those with non-TOF lesions. We examined the relative rates in implantable cardioverter defibrillator (ICD) therapy according to congenital lesion type in a large-volume adult congenital heart center. METHODS: A cohort of 59 individuals (median follow up time, 3.2 years range 0-10) with ACHD and ICDs was stratified according to underlying congenital lesion and implant indication. Appropriate therapies were defined as any therapy for a physician-adjudicated ventricular arrhythmia. Rates of inappropriate and appropriate ICD therapies were analyzed according to several relevant clinical variables. RESULTS: Thirty-three (56%) TOF, 15 (25.4%) L- or D-transposition of great arteries, and 11 (18.6%) with other lesions were included in the analysis. Approximately half (52.5%) were implanted for primary prevention indications. During follow-up, 12 (20.3%) patients received appropriate ICD therapies and 13 (22%) patients received inappropriate therapies. The incidence of appropriate shocks among patients with TOF was 27.3% (9/33) compared to 11.5% (3/26) among non-TOF diagnoses during the follow-up time (p = 0.043). CONCLUSIONS: ACHD patients with non-TOF congenital lesions are significantly less likely to receive appropriate ICD therapy than those with TOF. Our analysis calls into question the validity of traditional ICD implantation guidelines in this growing and diverse patient population.

Experience of decortication for restrictive hemodynamics in adults with congenital heart disease.

We treated four postoperative adults with congenital heart disease with severe restrictive hemodynamics (RH), and performed decortication (DC) with the anticipation of some relief of the RH. The catheterizations before DC showed high central venous, and right and left ventricular end-diastolic pressures with "dip-and-plateau" pressure waveforms in the right and left ventricles. Upon myocardial histopathologic examination, moderate myocardial fibrotic change was demonstrated in two of three cases. DC led to decrease in type B natriuretic peptide levels in all cases, resulting in a decline in the central venous, right and left ventricular end-diastolic pressures in three cases. Successful DC-related relief of RH, dilatation of the ventricles with decline in central and end-diastolic pressures, was observed in only one case. Our limited DC-related hemodynamic improvement indicates a complexity of the severe RH, which may represent a unique intractable heart failure pathophysiology in intractable postoperative adult congenital heart disease.

Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19-58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning-Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning-Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

Risk factors and early outcomes of repeat sternotomy in 1960 adults with congenital heart disease: A 30-year, single-center study.

OBJECTIVE: Patients with congenital heart disease (CHD) increasingly live into adulthood, often requiring cardiac reoperation. We aimed to assess the outcomes of adults with CHD (ACHD) undergoing repeat sternotomy at our institution. METHODS: Review of our institution's cardiac surgery database identified 1960 ACHD patients undergoing repeat median sternotomy from 1993 to 2023. The primary outcome was early mortality, and the secondary outcome was a composite end point of mortality and significant morbidity. Univariable and multivariable logistic regression models were used to determine factors independently associated with outcomes. RESULTS: Of the 1960 ACHDs patient undergoing repeat sternotomy, 1183 (60.3%) underwent a second, third (n = 506, 25.8%), fourth (n = 168, 8.5%), fifth (n = 70, 3.5%), and sixth sternotomy or greater (n = 33, 1.6%). CHD diagnoses were minor complexity (n = 145, 7.4%), moderate complexity (n = 1380, 70.4%), and major complexity (n = 435, 22.1%). Distribution of procedures included valve (n = 549, 28%), congenital (n = 625, 32%), aortic (n = 104, 5.3%), and major procedural combinations (n = 682, 34.7%). Overall early mortality was 3.1%. Factors independently associated with early mortality were older age at surgery, CHD of major complexity, preoperative renal failure, preoperative ejection fraction, urgent operation, and postoperative blood transfusion. In addition, sternotomy number and bypass time were independently associated with the composite outcome. CONCLUSIONS: Despite the increase in early mortality with sternotomy number, sternotomy number was not independently associated with early mortality but with increased morbidity. Improvement strategies should target factors leading to urgent operations, early referral, along with operative efficiency including bypass time and blood conservation.