Coronary artery disease screening in adults with congenital heart disease prior to cardiac surgery

Objective: As adults with congenital heart disease (CHD) grow older, preoperative screening for coronary artery disease (CAD) may be indicated prior to CHD surgery. Data regarding the indications for preoperative CAD screening in this population are limited. Current practice is to follow guidelines for patients with valvular heart dis‐ ease; however, the risk for CAD in certain congenital heart diagnoses may be higher than the general population. This study aimed to assess the results of preoperative CAD screening in patients prior to CHD surgery.
Design: Retrospective study.
Setting: Single tertiary center.
Patients: Patients ≥35 years that had CHD surgery from 1/1/2007 to 5/1/2017.
Outcome Measures: Data regarding CAD risk factors and preoperative CAD screen‐ ing results were obtained. Prevalence and risk factors for CAD were analyzed, along with their relationship to perioperative outcomes.
Results: A total of 73 patients underwent CAD screening with either cardiac catheteri‐ zation (56%) or computed tomography angiography (34%) prior to CHD surgery. Overall 16 (22%) patients were found to have CAD. Only two patients had severe coronary stenosis and underwent coronary bypass grafting at time of CHD surgery. Patients with CAD were more likely to be older and have history of hypertension, dyslipidemia, and tobacco smoking. CHD diagnosis was not significantly associated with presence of CAD.
Conclusion: CAD is common in asymptomatic older patients referred for screening prior to CHD surgery; however, severe CAD requiring concomitant coronary inter‐ vention is uncommon. Preoperative CAD screening should be based on age and tra‐ ditional CAD risk factors, rather than underlying CHD.     

Vasopressor magnitude predicts poor outcome in adults with congenital heart disease after cardiac surgery

Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
Results: Overall, 1040 ACHD patients had cardiac surgery during the study time frame; 243 (23.4%) met study inclusion criteria. Sixty‐two patients (25%), experi‐ enced composite poor outcome [including eight deaths within 90 days of hospital discharge (3%)]. Thirty‐eight patients (15%) endured complication related early mor‐ bidity. The maximum VIS (maxVIS) score area under the curve was 0.92 (95% CI: 0.86‐0.98) for in‐hospital mortality; and 0.82 (95% CI: 0.76‐0.89) for combined poor clinical outcome. On univariate analysis, maxVIS score ≥3 was predictive of compos‐ ite adverse outcome (OR: 14.2, 95% CI: 7.2‐28.2; P < 0.001), prolonged ICU LOS ICU LOS (OR: 19.2; 95% CI: 8.7‐42.1; P < 0.0001), prolonged mechanical ventilation (OR: 13.6; 95% CI: 4.4‐41.8; P < 0.0001) and complication related morbidity (OR: 7.3; 95% CI: 3.4‐15.5; P < 0.0001).
Conclusions: MaxVIS score strongly predicted adverse outcomes and can be used as a risk prediction tool to facilitate early intervention that may improve outcome and assist with clinical decision making for ACHD patients after cardiac surgery.     

Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring

Background: Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24‐48‐hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48‐hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations.
Objective: To address the preliminary question, we hypothesized that clinically sig‐ nificant arrhythmias would be detected on ECAM beyond 48 hours and this would lead to clinical management changes.
Methods: A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of ar‐ rhythmias detected within and beyond the first 48 hours of monitoring were com‐ pared using Kaplan‐Meier curves and Cox proportional hazard models.
Results: Three hundred fourteen patients had monitors performed [median age 31 (IQR 25‐41) years, 61% female). Significant arrhythmias were identified in 156 pa‐ tients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%).
Conclusions: ECAM detects more clinically significant arrhythmias than standard 48‐ hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48‐hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.     

Evaluation of athletes with complex congenital heart disease

As a result of improvements in congenital heart surgery, there are more adults alive today with congenital heart disease (CHD) than children. Individuals with cardiac birth defects may be able to participate in physical activities but require proper cardiovascular evaluation. The American Heart Association and American College of Cardiology released guidelines in 2015 for athletes with cardiovascular abnormalities. The guidelines express that although restriction from competitive athletics may be indicated for some, the majority of individuals with CHD can and should engage in some form of physical activity. This case study demonstrates the importance of combining all aspects of history, physical examination, ECG, and imaging modalities to evaluate cardiac anatomy and function in young athletes with complex CHD.     

Atrial fibrillation in adults with congenital heart disease following cardiac surgery in a single center: Analysis of incidence and risk factors

Objective: The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation.
Design: Retrospective analysis.
Setting: Single center, quaternary care children’s hospital.
Patients: This review included patients at least 18 years of age with known congenital heart disease who underwent cardiac surgery requiring a median sternotomy at our congenital heart center from January 1, 2012 to December 31, 2016.
Interventions: None.
Outcome Measures: The primary outcome was early postoperative atrial fibrillation. Secondary outcomes included preoperative comorbidities, preoperative echocardiographic findings, operative details, and postoperative morbidities, such length of stay, reintubation, stroke, and death.
Results: The incidence of early postoperative atrial fibrillation was 21%. Those who developed early postoperative atrial fibrillation were older (50 years vs 38 years, P =< .001), had a history of atrial fibrillation prior to surgery, had preoperative pulmonary hypertension, and had longer cardiopulmonary bypass times (103 minutes vs 84 minutes, P = .025) when compared to those who did not develop postoperative atrial fibrillation. Multivariate analysis identified age greater than 60, preoperative pulmonary hypertension, mitral valve intervention, and the need for postoperative inotropic support as being independent predictors of postoperative atrial fibrillation. Those who developed postoperative atrial fibrillation remained in the hospital longer (9 days vs 7 days, P =< .001).
Conclusions: Atrial fibrillation is a common complication following cardiac surgery in adults with congenital heart disease. Age, preoperative comorbidities, type of surgical intervention, and the need for perioperative inotropic infusions may predict the risk of atrial fibrillation in this unique patient population.     

居家心脏康复和中心心脏康复对冠心病患者心肺适能的影响

目的 探索居家心脏康复(HBCR)和中心心脏康复(CBCR)对冠心病(CHD)患者心肺适能的影响.方法 选取2018年11月至2019年10月在解放军总医院心脏康复中心门诊就诊的18～80岁的CHD患者,采用随机数表和信封法将患者分为HBCR组和CBCR组,分别以HBCR或CBCR干预3个月.对比2组患者干预前后的峰值...     

Is it safe to perform cardiac catheterizations on adults with congenital heart disease in a pediatric catheterization laboratory?

OBJECTIVE: To determine the complication rate during the catheterization in adults with congenital heart disease (CHD) in a pediatric catheterization laboratory (PCL). BACKGROUND: An increasing number of patients with CHD are surviving into adulthood, with diagnostic and interventional cardiac catheterization being essential for the management of their disease. The complication rate during the catheterization of adults with CHD has not been reported. METHODS: A retrospective chart review was performed on all adult patients (>18 years) with CHD who underwent diagnostic or interventional catheterization in our PCL within the past 8.5 years. RESULTS: A total of 576 procedures were performed on 436 adult patients (median age 26 years). Complex heart disease was present in 387/576 (67%) procedures. An isolated atrial septal defect or patent foramen ovale was present in 115/576 (20%) procedures, and 51/576 (9%) procedures were performed on patients with structurally normal hearts with arrhythmias. Interventional catheterization was performed in 378/576 (66%) procedures. There were complications during 61/576 (10.6%) procedures; 19 were considered major and 42 minor. Major complications were death (1), ventricular fibrillation (1), hypotension requiring inotropes (7), atrial flutter (3), retroperitoneal hematoma, pneumothorax, hemothorax, aortic dissection, renal failure, myocardial ischemia and stent malposition (1 each). The most common minor complications were vascular entry site hematomas and hypotension not requiring inotropes. Procedures performed on patients > or = 45 years of age had a 19% occurrence of complications overall compared with 9% occurrence rate in patients of age < 45 years (P < 0.01). CONCLUSIONS: The complication rate during the catheterization of adults with CHD in a PCL is similar to the complication rate of children with CHD undergoing cardiac catheterization. The older subset of patients are more likely to encounter complications overall. The encountered complications could be handled effectively in the PCL. With screening in place, it is safe to perform cardiac catheterization on most adults with CHD in a PCL.     

Recommendations to organize care for adults with congenital heart disease in the Czech Republic

This paper was formed by the Expert committee for congenital heart disease (CHD) in adults – a division of Czech Society of Cardiology (ČKS). It was designed as an appendix to National cardiovascular programme ČKS created in 2013 and was based on Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology created in 2014 [1].Aims of this paper are: To optimize medical care in all its aspects for adults with CHD in the Czech Republic, to facilitate easy transition between paediatric and adult medical care, to enable research in the field in order to create evidence based care, to support training of regional cardiologists and other specialists who are involved in monitoring adult patients with CHD, to help with communication with national institutions, to provide information for other medical workers and patients, to consolidate resources.     

Obesity trends in children,adolescents, and young adults with congenital heart disease

Objectives: To determine the prevalence, age of onset, and risk factors for overweight and obesity in children with congenital heart disease (CHD).
Study Design: Children with CHD who were seen at our institution from 1996 to 2017 were studied. Patients were full-time residents of the United States and were receiving all cardiac care at our institution. Patients were categorized by age and CHD diagnosis. The date of last normal weight for age and the date of first recorded weight in the range of overweight and obese were documented.
Results: Nine hundred sixty-eight patients with CHD were included. The prevalence of overweight and obesity was 31.5% and 16.4%, respectively. For patients who became overweight or obese, the last recorded normal weight was between 6 and 10 years of age. Electrophysiologic disease and older age were risk factors for obesity.
Conclusions: Children with CHD have an increasing risk of becoming overweight and obese in early childhood. This study provides important information and identifies critical period to implement preventative measures and counsel families about the risk of obesity in CHD.     

PiCCO技术对复杂先天性心脏病患儿围手术期治疗的意义

目的：探讨脉搏指数连续心排出量（pulse indicated continuous cardiac output，PiCCO）监测在复杂性先天性心脏病患儿围术期治疗的指导意义。方法：选取复杂性先天性心脏病矫治术后的患儿104例，随机分为两组，PiCCO组（51例），均放置PiCCO导管监测平均动脉压（MAP）、心输出量（CO）、连续心输出量（CC0）、外周血管阻力（SVR）、胸腔内血容量（ITBV）、全心舒张末期容量（GEDV）、血管外肺水（EVLw）等，并指导临床治疗。对照组（53例），不放置PiCCO导管，凭经验及其他常规指标指导治疗。结果：PiCC0组机械通气时间、ICU滞留时间、血管活性药物使用时间显著短于对照组，但两组患儿左室射血分数（LVEF）、脑钠尿肽（BNP）、胸片渗出情况、感染率、病死率均无统计学差异。结论：PiCCO技术在复杂性先天性心脏病患儿术后治疗中有一定的实用价值。     

Prolonged Tpeak‐Tend interval is a risk factor for sudden cardiac death in adults with congenital heart disease

Objective: Adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). However, methods for risk stratification are not yet well‐ defined. The T_peak‐T_end (TpTe) interval, a measure of dispersion of ventricular repolari‐ zation, is a risk factor for SCD in non‐ACHD patients. We aim to evaluate whether TpTe can be used in risk stratification for SCD in ACHD patients.
Design: From an international multicenter cohort of 25 790 ACHD patients, we iden‐ tified all SCD cases. Cases were matched to controls by age, gender, congenital de‐ fect, and (surgical) intervention.
Outcome Measures: TpTe was measured on a standard 12‐lead ECG. The maximum TpTe of all ECG leads (TpTe‐max), mean (TpTe‐mean), and TpTe dispersion (maximum minus minimum) were obtained. Odds ratios (OR) for SCD cases vs controls were calculated using conditional logistic regression analysis.
Results: ECGs were available for 147 cases (median age at death 33.5 years (quartiles 26.2, 48.7), 66% male) and 267 controls. The mean TpTe‐max was 97 ± 24 ms in cases vs 84 ± 17 ms in controls (P < .001); TpTe‐mean was 70 ± 16 vs 63 ± 10 ms (P < .001); and dispersion was 51 ± 22 ms vs 41 ± 16 ms (P = .02), respectively. Assessing each ECG lead separately, TpTe in lead aVR predicted SCD most accurately. TpTe in lead aVR was 71 ± 23 ms in cases vs 61 ± 13 ms in controls (P < .001). After adjusting for impaired ventricular function, heart failure symptoms, and prolonged QRS duration, the OR of SCD of TpTe in lead aVR at an optimal cutoff of 80 ms was 5.8 (95% CI 2.7‐12.4, P < .001).
Conclusions: The TpTe interval is associated with SCD in ACHD patients. Particularly, TpTe in lead aVR can be used as an independent risk factor for SCD in ACHD patients and may, therefore, add precision to current risk prediction models.     

Wide complex tachycardia and congenital heart disease.

We present a patient with congenital heart disease and haemodynamically poorly tolerated wide QRS tachycardia. Differential diagnosis and therapy are discussed. After the patient underwent heart transplantation, and the substrates for ECG abnormalities and arrhythmias were demonstrated in the explanted heart.     

A model for geographic and sociodemographic access to care disparities for adults with congenital heart disease

Background: Follow‐up at a regional adult congenital heart disease (ACHD) center is recommended for all ACHD patients at least once per the 2018 ACC/AHA guidelines. Other specialties have demonstrated poorer follow‐up and outcomes correlating with increased distance from health care providers, but driving time to regional ACHD centers has not been examined in the US population.
Objective: To identify and characterize potential disparities in access to ACHD care in the US based on drive time to ACHD centers and compounding sociodemographic factors.
Methods: Mid‐ to high‐volume ACHD centers with ≥500 outpatient ACHD visits and ≥20 ACHD surgeries annually were included based on self‐reported, public data. Geographic Information System mapping was used to delineate drive times to ACHD centers. Sociodemographic data from the 2012‐2016 American Community Survey (US Census) and the Environmental Systems Research Institute were analyzed based on drive time to nearest ACHD center. Previously established CHD prevalence estimates were used to estimate the similarly located US ACHD population.
Results: Nearly half of the continental US population (45.1%) lives >1 hour drive to an ACHD center. Overall, 39.7% live 1‐4 hours away, 3.4% live 4‐6 hours away, and 2.0% live >6 hours away. Hispanics were disproportionately likely to live a >6 hour drive to a center (p < .001). Compared to people with <1 hour drive, those living >6 hours away have higher proportions of uninsured adults (29% vs. 18%; p < .001), households below the federal poverty level (19% vs. 13%; p < .001), and adults with less than college education (18% vs. 12%; p < .001).
Conclusions: We estimate that ~45% of the continental US population lives >1 hour to an ACHD center, with 5.4% living >4 hours away. Compounding barriers exist for Hispanic, uninsured, lower socioeconomic status, and less‐educated patients. These results may help drive future policy changes to improve access to ACHD care.     

Clinic nonattendance is associated with increased emergency department visits in adults with congenital heart disease

Objective: To determine the prevalence and predictors of nonattendance in an ACHD outpatient clinic, and to examine the relationship between nonattendance and emergency department (ED) visits, hospitalizations, and death.
Methods: Patients ≥ 18 years who had scheduled appointments at an ACHD outpatient clinic between August 1, 2014 and December 31, 2014 were included. The primary outcome of interest was nonattendance of the first scheduled appointment of the study period, defined as “no-show” or “same-day cancellation.” Secondary outcomes of interest were ED visits, hospitalizations, and death until December 2017.
Results: Of 527 scheduled visits, 55 (10.4%) were nonattended. Demographic and socioeconomic characteristics such as race, income, and insurance type were associated with non-attendance (all P values < .05), whereas age, gender, and disease complexity were not. On multivariable analysis, predictors of nonattendance were black race (adjusted odds ratio [AOR] 4.95; P < .001), other race (AOR 3.54; P = .003), and history of no-show in the past (AOR 4.95; P < .001). Compared to patients who attended clinic, patients with a nonattended visit had a threefold increased odds of multiple ED visits and a significantly lower rate of ED-free survival over time. There were no significant differences in hospitalizations or death by attendance.
Conclusion: ACHD clinic nonattendance is associated with race and prior history of no-show, and may serve as a marker of higher ED utilization for patients with ACHD.     

Narrative analysis of adults with complex congenital heart disease: Childhood experiences and their lifelong reverberations

Background: With access to surgical care, >90% of today’s infants with congenital heart disease (CHD) will reach adulthood. During childhood, survivors accrue a wealth of health care experience and develop strategies for navigating life with a chronic disease.
Methods: Seeking to learn from this individualized process, we invited adults with complex CHD to participate in narrative analysis—an established qualitative research method for studying how individuals derive meaning from their personal stories. Audio recordings of 2‐4 hour free‐form interviews were transcribed and iteratively analyzed to identify common themes and detect similarities or differences in language, viewpoint and interpretation. Recruitment continued until saturation was reached (n = 10).
Results: While each narrative was unique, CHD had a pervasive effect on the autobiography of all participants. Seven themes were discussed consistently: (1) parental/sibling relationships, (2) physical limitations, (3) embarrassment/denial, (4) memories of pediatric health care, (5) transition to adult care, (6) education and career choices, and (7) relationship and reproductive choices. While some of the recalled experiences were negative, all participants also spoke positively about the effect of CHD on their lives.
Conclusions: Adults with CHD provide the voices of expert witnesses; illuminating how the pediatric journey influences their identity, choices, personal relationships and adult health care interactions. These narratives could inform and improve the contemporary care of children with heart disease.     

Opportunities for training to advance the care for adults with congenital heart disease with advanced circulatory failure

Heart failure is an emerging issue with important implications in adult patients with congenital heart disease. Practitioners with expertise in both adult congenital heart disease and heart failure are needed to manage this growing and often complex population. In the United States, the optimal training pathway to enable practitioners to best care for these patients is ill‐defined. This article explores possibilities and issues that interested trainees may encounter during their training experience.     

先天性心脏病合并艾森曼格综合征的外科治疗

目的回顾分析28例先天性心脏病合并艾森曼格综合征患者外科手术结果,评价手术治疗效果,探讨手术适应证。方法所有病例术前均予以靶向降肺动脉压为主的综合治疗,以动脉SaO2≥90％作为手术指征进行手术。结果围术期无死亡,术后随访4～24个月。1例出院后2个月猝死于肺高压危象;2例患者随访结果欠佳,心功能Ⅳ级,其中1例术后经常晕厥,反复咯血,1例术后3个月肺动脉高压右心衰伴三尖瓣大量反流。其余25例患者均较术前有不同程度的改善。结论部分以往被列为手术禁忌的艾森曼格患者仍可经充分靶向治疗后获得手术机会,并可拥有一个满意的预期结果。正确的手术适应证以及手术时机的选择,合理的围手术期处理,术后长期靶向药物治疗是降低手术风险、改善预后的关键。