Adult Congenital Heart Disease Surgery Research Articles

Morbidity and Mortality in Adult Congenital Heart Surgery: Physiologic Component Augments Risk Prediction

BackgroundWe sought to evaluate whether the anatomic and physiologic stratification system (ACAP score), released as part of the American College of Cardiology/American Heart Association updated guidelines for management of adult congenital heart disease (ACHD) in 2018, better estimated mortality and morbidity after cardiac operations for ACHD. MethodsThe ACAP score was determined for 318 patients (age ≥18 years) with ACHD undergoing heart surgery at our institution between December 2001 and August 2019. The primary end point was perioperative mortality. The secondary aim was to evaluate the performance of the ACAP, The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Congenital Heart Surgery Mortality Categories, and ACHS mortality scores/categories at predicting a composite adverse outcome of perioperative mortality, prolonged ventilation, and renal failure requiring replacement therapy. Logistic regression models were built to estimate mortality and the composite outcome using anatomic and physiologic components independently and together. Receiver operating characteristic curves were created, and area under the curves were compared using the Delong test. ResultsThe median age was 37 years (interquartile range, 26.3-50.0 years). There were 9 perioperative mortalities (2.8%). With respect to perioperative mortality, the area under the curve using the anatomic component only was 0.74, which improved to 0.81 after including physiologic severity (P = .05). When physiologic severity was added to the model for the composite outcome, the discriminatory abilities of the ACHS mortality score and the STAT categories increased significantly to 0.83 (95% CI, 0.75-0.91; P = .02) and 0.82 (95% CI, 0.73-0.90; P = .04), comparable to the predictive power of ACAP. ConclusionsPhysiologic severity augments ability to predict mortality and morbidity after cardiac surgery for ACHD. There is need for more robust ACHD-specific risk models.

Assessing Risk Factors for Prolonged Intensive Care Unit Stay After Surgery for Adult Congenital Heart Disease: A Study From a Lower-Middle-Income Country.

Background Prolonged post-surgery intensive care unit (ICU) stay for congenital heart disease (CHD) has been explored in the pediatric population. However, there is limited data for adult CHD (ACHD), also called grown-up congenital heart (GUCH) disease, especially in low-resource countries where intensive care beds are scarce. This study identifies factors associated with prolonged ICU stay following surgery for ACHD in Pakistan, a lower-middle-income country (LMIC). Methods This retrospective study included all adult patients (⩾18 years) who underwent cardiac surgery with cardiopulmonary bypass for their CHD from 2011-2016 at a tertiary-care private hospital in Pakistan. Prolonged ICU stay was defined as stay >6 days (75th percentile). Regression analysis was used to explore risk factors of prolonged ICU stay. Results A total of 166 patients (53.6% males) with a mean age of 32.05 ± 12.11 years were included. Atrial septal defect repair was the most common surgery (42.2%). Most patients were categorized as Risk Adjustment for Congenital Heart Surgery 1 (RACHS-1) Category 1 (51.8%) and Category 2 (30.1%). Forty-three of 166 patients (25.9%) experienced prolonged ICU stay. Complications occurred in 38.6% of patients postoperatively, with the most common being acute kidney injury (29.5%). On multivariable logistic regression adjusted for age, gender, and RACHS-1 categories, intraoperative inotrope score, cardiopulmonary bypass time, aortic cross-clamp time duration of mechanical ventilation, and postoperative acute kidney injury (AKI) were associated with prolonged ICU stay. Conclusion Surgeons managing ACHD in LMICs must strive for shorter operative durations and the judicious use of intraoperative inotropes in addition to anticipating and promptly managing postoperative complications such as AKI, to minimize ICU stay in countries where intensive care beds are a scarce resource.

Racial Disparity: The Adult Congenital Heart Disease Surgery Perspective.

The influence of race and ethnicity on clinical outcomes in medicine are widely acknowledged. However, the effect of race on adult congenital heart disease (ACHD) surgery is not known. We sought to evaluate the possible association between race and outcomes following ACHD operations. Discharge records for patients who underwent ACHD surgery between 2005 and 2014, were isolated from an all-payer voluntary database in the United States. Hierarchical case-mix regression models and sensitivity analyses examined any complication, in-hospital mortality, and discharge disposition (home/non-home) by race (white-WP, black-BP, non-white non-black-NWNB). Of the 174,370 patients (WP: 80.8%, BP: 5.8%, NWNB: 13.4%), black patients were youngest to undergo surgery (WP: 57.9 ± 15.8years, BP: 50.2 ± 16.1years, NWNB: 51.6 ± 16.9years, P < 0.0001), the most likely to have a comorbidity (WP: 70.3%, BP: 74.3%, NWNB: 68.6%, P < 0.0001), and most likely to have had a post-operative cardiac complication (WP: 9.4%, BP: 15.3%, NWNB: 10.9%, P < 0.0001). BP had similar odds of having any complication (AOR = 0.99, 95%CI = 0.94-1.04), while NWNB had significantly decreased odds of a major complication (AOR = 0.90, 95%CI = 0.87-0.93). BP had equivalent in-hospital mortality compared to WP (AOR = 1.03, 95%CI = 0.91-1.18), while NWNB had significantly increased odds of in-hospital mortality (AOR = 1.29, 95%CI = 1.18-1.41). Among survivors, BP were less likely to discharge home (AOR = 0.88, 95%CI = 0.82-0.94), and NWNB were more likely to discharge home than WP (AOR = 1.26, 95%CI = 1.19-1.33). Race and clinical outcomes are associated among patients undergoing surgery for ACHD. Understanding why and how these factors are impactful will help improve care for this complex population.

Model for screening adult congenital heart disease surgery eligibility with echocardiography parameters.

This study aimed to screen for the eligibility of correction in cases of adult congenital heart disease (CHD). Pulmonary to systemic flow ratios (Qp/Qs) > 1.5 and pulmonary to systemic vascular resistance ratios (Rp/Rs) < 1/3, acquired by right heart catheterization (RHC), are two essential parameters. Nonetheless, performing RHC at every follow-up is impractical and even harmful. Thus, it is important to establish a model to predict Qp/Qs and Rp/Rs status before a RHC confirmation, using echocardiography parameters. A total of 1,785 patients with adult CHD were enrolled and randomly assigned to the derivation or validation groups. Echocardiogram parameters of the 974 patients in the derivation group were considered candidate predictors for surgery eligibility (Qp/Qs > 1.5 and Rp/Rs < 1/3). Binary logistic regression analyses were performed to identify the independent predictors and establish a scoring system. The scoring system was further examined in the validation group using a receiver operating characteristic (ROC) analysis. Estimated pulmonary artery systolic pressure, velocity through the pulmonary valve, and diameters of the left and right atria were identified as independent predictors. The area under the ROC curve of the predictive value in the validation group and its pre- and post-tricuspid valve malformation subgroups were 0.87 (95% confidence interval [CI]: 0.84-0.90, p < 0.01), 0.86 (95% CI: 0.82-0.91, p < 0.01), and 0.85 (95% CI: 0.79-0.90, p < 0.01), respectively. This scoring system could augment flexibility and convenience for pre-screening CHD patients' eligibility for surgery, before RHC.

Selected 2021 Highlights in Congenital Cardiac Anesthesia

This article is a review of the highlights of pertinent literature of interest to the congenital cardiac anesthesiologist and was published in 2021. After a search of the United States National Library of Medicine PubMed database, several topics emerged where significant contributions were made in 2021. The authors of this manuscript considered the following topics noteworthy to be included in this review: risk stratification in adult congenital heart disease surgery, physician burnout in pediatric cardiac anesthesia, transfusion practice in pediatric congenital heart surgery, and racial disparity and outcomes in pediatric patients with congenital heart disease.

Enhanced Assessment of Perioperative Mortality Risk in Adults With Congenital Heart Disease

BackgroundIn-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment. ObjectivesThe purpose of this study was to assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery. MethodsWe assessed perioperative survival in patients aged >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable–derived PEACH (PErioperative ACHd) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center. ResultsThe development cohort comprised 1,782 procedures performed during the study period. Re-sternotomy was undertaken in 897 (50.3%). There were 31 (1.7%) in-hospital deaths. The PEACH score showed excellent discrimination ability (area under the curve [AUC]: 0.88; 95% CI: 0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC: 0.69; 95% CI: 0.6-0.78; P = 0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), and ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC: 0.75; 95% CI: 0.72-0.77) and was well calibrated (Hosmer-Lemeshow chi-square goodness-of-fit P = 0.55). There was agreement in expected and observed perioperative mortality between cohorts. ConclusionsThe PEACH score is a simple, novel perioperative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery.

Noncardiac determinants of death and intensive care morbidity in adult congenital heart disease surgery

ObjectivesPredicting perioperative morbidity and mortality in cardiac surgery for adult congenital heart disease is challenging because it encompasses a wide spectrum of disease. There is a paucity of published outcome data, and there are no perioperative risk score calculators for this population group. We set out to identify robust determinants of morbidity and mortality in this patient population under going cardiac surgery. MethodsWe collected data on 20 socioeconomic and pathophysiologic variables in 784 consecutive adults with congenital heart disease who underwent cardiac surgery between 2004 and 2015 at a single center. Using logistic regression, we sought to identify which of these factors were associated with the primary composite adverse outcome of in-hospital mortality, prolonged ventilation exceeding 7 days, and severe acute renal failure requiring dialysis. Secondary outcome analysis identified variables that were significant predictors for 1-year mortality. ResultsComposite adverse outcome occurred in 54 of 784 patients (6.9%). Significant predictors of the composite adverse outcome by multivariate regression include Mayo End-Stage Liver Disease modified score, cognitive impairment, number of chest wall incisions from previous cardiac surgery, body mass index, and cardiac anatomic category. Two survivors of the composite adverse outcome died within a few weeks postdischarge. Only 657 of 784 patients had 1-year follow-up data; 40 of 657 patients died at 1 year. One-year mortality was predicted by anticoagulation, Mayo End-Stage Liver Disease modified score, and anatomic category. ConclusionsRecognition and quantification of noncardiac comorbidities preoperatively predict the risk of adverse events and mortality in addition to cardiac anatomic factors.

Catheter ablation of supraventricular tachycardia after tricuspid valve surgery in patients with congenital heart disease: A multicenter comparative study

Tricuspid valve (TV) surgery is often required for adult congenital heart disease (ACHD), but may hinder catheter ablation when an artificial material or imbricated tissue covers the tricuspid annulus. The purpose of this study was to determine the outcomes of catheter ablation after TV surgery in a large ACHD cohort. An international retrospective study involving 7 centers was conducted. Patients who did and did not undergo TV surgery were matched for age, lesion classification, and postsurgical duration. TV operations were classified as valve ring/replacement vs repair. One hundred thirty-six patients (42 ring/replacement, 39 repair, and 55 no TV surgery; median 32 years [IQR 20 - 46]) underwent 180 procedures targeting 239 tachycardias (cavotricuspid-isthmus dependent intraatrial reentrant tachycardia 36%, other intraatrial reentrant tachycardia 29%, focal atrial tachycardia 18%, and other supraventricular tachyarrhythmia 17%). Post-TV surgery, procedures were longer (4.3 hours vs 3.3 hours; P = .003) and required longer fluoroscopy time (31 minutes vs 18 minutes; P = .001). At least partial acute success was achieved in 81% of procedures in the TV ring/replacement group vs 94% in both TV repair and no TV surgery groups (P = .03). The difference was driven mainly by ablation of annular substrates, with acute success in 73% of TV ring/replacement, 92% of TV repair, and 94% of no TV surgery groups (P = .01). Over a median of 3.0 years, tachycardia recurred after 26% of procedures. TV ring/replacement predicted recurrence in the multivariable analysis (hazard ratio 2.4; 95% confidence interval 1.2-5.2; P = .009). After surgery for ACHD, catheter ablation success was lower and tachycardia recurrence was higher after TV valve ring/replacement surgery. The findings of this retrospective report support future larger multicenter series and prospective evaluation to determine the role of empirical annular substrate ablation.

Morbidity During Adult Congenital Heart Surgery Admissions.

The frequency of complications during Adult Congenital Heart Disease (ACHD) surgery admissions and their association to patient outcome is not well known. Our study objectives are to (1) define the frequency of complications during ACHD surgery admissions, (2) identify their risk factors, and (3) explore their association with death and resource use. We identified ACHD surgery admissions ages 18 to 49 during the years 2005-2009 from the Nationwide Inpatient Sample database. Complications were defined according to the Society of Thoracic Surgeons Short List of Complications for congenital heart surgery. We identified 16,841 ACHD surgery admissions, of which 46.9% had at least one complication. Cardiac (19.4%), respiratory (18.2%), infectious (14.1%), and acute kidney injury (6.8%) were the most common. Admissions with a complication had a longer length of stay (10days vs. 5days; p < 0.001), increased charges ($139,522 vs. $84,672; p < 0.001), and higher mortality (4.6% vs. 0.9%; p < 0.001). Adjusted risk factors for complications included non-White race (AOR 1.17, p = 0.003), government insurance AOR 1.39, high surgical complexity RACHS-1 category 3 + AOR 1.81, non-elective admission OR 2.18, chronic kidney disease AOR 2.79, chronic liver disease AOR 2.47, and CHF AOR 1.40; all p < 0.001. Complications were independently associated with death AOR 2.49, p < 0.001. Complications occur frequently during ACHD surgery admissions and are associated with increased resource use and are a risk factor for death. Identification of preventable morbidity may improve the outcomes of these complex patients.

Predictors of extracorporeal membrane oxygenation support after surgery for adult congenital heart disease in children's hospitals.

Adult congenital heart disease (ACHD) patients who undergo cardiac surgery are at risk for poor outcomes, including extracorporeal membrane oxygenation support (ECMO) and death. Prior studies have demonstrated risk factors for mortality, but have not fully examined risk factors for ECMO or death without ECMO (DWE). We sought to identify risk factors for ECMO and DWE in adults undergoing congenital heart surgery in tertiary care children's hospitals. All adults (≥18years) undergoing congenital heart surgery in the Pediatric Health Information System (PHIS) database between 2003 and 2014 were included. Patients were classified into three groups: ECMO-free survival, requiring ECMO, and DWE. Univariate analyses were performed, and multinomial logistic regression models were constructed examining ECMO and DWE as independent outcomes. Tertiary care children's hospitals. A total of 4665 adult patients underwent ACHD surgery in 39 children's hospitals with 51 (1.1%) patients requiring ECMO and 64 (1.4%) patients experiencing DWE. Of the 51 ECMO patients, 34 (67%) died. Increasing patient age, surgical complexity, diagnosis of single ventricle heart disease, preoperative hospitalization, and the presence of noncardiac complex chronic conditions (CCC) were risk factors for both outcomes. Additionally, low and medium hospital ACHD surgical volume was associated with an increased risk of DWE in comparison with ECMO. There are overlapping but separate risk factors for ECMO support and DWE among adults undergoing congenital heart surgery in pediatric hospitals.

Incidence, risk factors, and outcomes of acute kidney injury in adults undergoing surgery for congenital heart disease.

Acute kidney injury after cardiac surgery is a frequent and serious complication among children with congenital heart disease (CHD) and adults with acquired heart disease; however, the significance of kidney injury in adults after congenital heart surgery is unknown. The primary objective of this study was to determine the incidence of acute kidney injury after surgery for adult CHD. Secondary objectives included determination of risk factors and associations with clinical outcomes. This single-centre, retrospective cohort study was performed in a quaternary cardiovascular ICU in a paediatric hospital including all consecutive patients ⩾18 years between 2010 and 2013. Data from 118 patients with a median age of 29 years undergoing cardiac surgery were analysed. Using Kidney Disease: Improving Global Outcome creatinine criteria, 36% of patients developed kidney injury, with 5% being moderate to severe (stage 2/3). Among higher-complexity surgeries, incidence was 59%. Age ⩾35 years, preoperative left ventricular dysfunction, preoperative arrhythmia, longer bypass time, higher Risk Adjustment for Congenital Heart Surgery-1 category, and perioperative vancomycin use were significant risk factors for kidney injury development. In multivariable analysis, age ⩾35 years and vancomycin use were significant predictors. Those with kidney injury were more likely to have prolonged duration of mechanical ventilation and cardiovascular ICU stay in the univariable regression analysis. We demonstrated that acute kidney injury is a frequent complication in adults after surgery for CHD and is associated with poor outcomes. Risk factors for development were identified but largely not modifiable. Further investigation within this cohort is necessary to better understand the problem of kidney injury.

Twenty Years of Adult Congenital Heart Surgery: A Single-Center African Experience.

Lack of data exists on the surgical management of adult congenital heart disease on the African continent. This study was undertaken to describe the clinical profile and surgical outcome of adult patients with congenital heart disease undergoing surgery in a single-center African study population. A retrospective medical chart review of consecutive adult patients (≥18 years) undergoing congenital heart surgery between October 1995 and December 2015 at our institution was undertaken. We described cardiac diagnosis, diagnostic complexity, risk profile, and surgical morbidity and mortality. Data were collected of 233 surgical procedures performed in 219 patients (45.6% males). The most common diagnostic category was septal defects (41.2%), followed by right heart lesions (17.2%), left heart lesions (12.4%), thoracic arteries (9.0%), and conduit failure (6.9%). Twenty-four percent of patients presented in functional class III or IV, and 46% of patients met the criteria for the simple Bethesda diagnostic class. Preoperative risk factors were identified in 19.8% of patients. Corrective surgery was performed in 71.7% of cases, reoperative surgery in 27.6%, and palliative surgery in 0.8%. Right ventricle to pulmonary artery conduit placement comprised 53.1% of reoperations. The overall hospital mortality was 1.7%. Postoperative complications occurred in 26.3% of cases. This study presents a detailed description of this emerging population in a developing world context. Our outcomes data suggest that adult congenital heart disease surgery is feasible in a Southern African tertiary referral center with low operative mortality and acceptable morbidity.

Trends in Utilization of Specialty Care Centers in California for Adults With Congenital Heart Disease

The American College of Cardiology and American Heart Association guidelines recommend that management of adult congenital heart disease (ACHD) be coordinated by specialty ACHD centers and that ACHD surgery for patients with moderate or complex congenital heart disease (CHD) be performed by surgeons with expertise and training in CHD. Given this, the aim of this study was to determine the proportion of ACHD surgery performed at specialty ACHD centers and to identify factors associated with ACHD surgery being performed outside of specialty centers. This retrospective population analysis used California's Office of Statewide Health Planning and Development's discharge database to analyze ACHD cardiac surgery (in patients 21 to 65years of age) in California from 2000 to 2011. Designation as a "specialty ACHD center" was defined on the basis of a national ACHD directory. A total of 4,611 ACHD procedures were identified. The proportion of procedures in patients with moderate and complex CHD delivered at specialty centers increased from 46% to 71% from 2000 to 2011. In multivariate analysis among those discharges for ACHD surgery in patients with moderate or complex CHD, performance of surgery outside a specialty center was more likely to be associated with patients who were older, Hispanic, insured by health maintenance organizations, and living farther from a specialty center. In conclusion, although the proportion of ACHD surgery for moderate or complex CHD being performed at specialty ACHD centers has been increasing, 1 in 4 patients undergo surgery at nonspecialty centers. Increased awareness of ACHD care guidelines and of the patient characteristics associated with differential access to ACHD centers may help improve the delivery of appropriate care for all adults with CHD.

Outcome of Complex Adult Congenital Heart Surgery in the Developing World

There is scanty information on the outcome of adult congenital heart disease surgery from the developing world. This was a retrospective chart review of the surgical outcome of 153 adults with congenital heart disease over a 5-year period. Surgical atrial septal defect closure was considered "simple" while all other surgeries were considered "complex." There were 102 patients in the "simple" group and 51 in the "complex" group. Only three (2%) patients had prior operations. The "complex" group had longer bypass time and cross clamp time. Intensive care unit stay, ventilation time, and inotrope administration were longer. Major complications were more common and there were two deaths in the "complex" group. Age more than 30 years, cyanosis, and New York Heart Association class more than II were predictors of longer stay in the intensive care unit. Surgical repair of Tetralogy of Fallot in adults tended to have a longer ventilation time and intensive care unit stay with a mortality of 4%. At follow up, all patients were in New York Heart Association class I or II. Improvement of the functional class with negligible adverse events was noted in both groups. A retrospective evaluation of 153 adults with congenital heart disease who underwent open heart surgery at a single center in India showed strikingly fewer reoperations compared with large European studies. There was a similar prevalence of complex lesions. Surgical mortality was low, and long-term functional outcome was gratifying.

Nationwide Survey of Care Facilities for Adults With Congenital Heart Disease in Japan

The number of adults with congenital heart disease (CHD) is increasing rapidly, but care programs have not been fully established in Japan. Questionnaires regarding current status and resources of outpatient and in-hospital services, and management of pregnancy in patients with adult CHD (ACHD) were sent to 1,033 training hospitals for board-certified cardiologists. Useful replies were obtained from 458 hospitals (44%). In 417 hospitals (91%), at least 1 patient was followed in the outpatient clinic; however, only 14 hospitals (3%) had specialized outpatient clinics; 354 hospitals (77%) had in-hospital patients, but only 6 hospitals (2%) admitted >50 patients per year. Surgery for ACHD was performed in 232 hospitals (51%), but in 135 of these (58%), the number of operations was <5 per year. Pregnant women with CHD were managed in 157 hospitals (34%), although only 3 hospitals (2%) managed >10 cases per year. In most hospitals in Japan, a limited number of ACHD patients have been followed up and specialized multi-disciplinary facilities for ACHD need to be established.