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Background American Heart Association and American College of Cardiology consensus guidelines introduce an adult congenital heart disease anatomic and physiological (AP) classification system. We assessed the association between AP classification and clinical outcomes. Methods and Results Data were collected for 1000 outpatients with ACHD prospectively enrolled between 2012 and 2019. AP classification was assigned based on consensus definitions. Primary outcomes were (1) all‐cause mortality and (2) a composite of all‐cause mortality or nonelective cardiovascular hospitalization. Cox regression models were developed for AP classification, each component variable, and additional clinical models. Discrimination was assessed using the Harrell C statistic. Over a median follow‐up of 2.5 years (1.4–3.9 years), the composite outcome occurred in 185 participants, including 49 deaths. Moderately or severely complex anatomic class (class II/III) and severe physiological stage (stage D) had increased risk of the composite outcome (AP class IID and IIID hazard ratio, 4.46 and 3.73, respectively, versus IIC). AP classification discriminated moderately between patients who did and did not suffer the composite outcome (C statistic, 0.69 [95% CI, 0.67–0.71]), similar to New York Heart Association functional class and NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide); it was more strongly associated with mortality (C statistic, 0.81 [95% CI, 0.78–0.84]), as were NT‐proBNP and functional class. A model with AP class and NT‐proBNP provided the strongest discrimination for the composite outcome (C statistic, 0.73 [95% CI, 0.71–0.75]) and mortality (C statistic, 0.85 [95% CI, 0.82–0.88]). Conclusions The addition of physiological stage modestly improves the discriminative ability of a purely anatomic classification, but simpler approaches offer equivalent prognostic information. The AP system may be improved by addition of key variables, such as circulating biomarkers, and by avoiding categorization of continuous variables.

Background Long‐term survival in patients with truncus arteriosus is favorable, but there remains significant morbidity associated with ongoing reinterventions. We aimed to study the long‐term outcomes of the truncal valve and identify risk factors associated with truncal valve intervention. Methods and Results We retrospectively reviewed patients who underwent initial truncus arteriosus repair at our institution from 1985 to 2016. Analysis was performed on the 148 patients who were discharged from the hospital and survived ≥30 days postoperatively using multivariable competing risks Cox regression modeling. Median follow‐up time was 12.6 years (interquartile range, 5.0–22.1 years) after discharge from full repair. Thirty patients (20%) underwent at least one intervention on the truncal valve during follow‐up. Survival at 1, 10, and 20 years was 93.1%, 87.0%, and 80.9%, respectively. The cumulative incidence of any truncal valve intervention by 20 years was 25.6%. Independent risk factors for truncal valve intervention included moderate or greater truncal valve regurgitation (hazard ratio [HR], 4.77; P P P z ‐score before truncus arteriosus full repair and during follow‐up was associated with worsening truncal valve regurgitation. Conclusions Long‐term rates of truncal valve intervention are significant. At least moderate initial truncal valve stenosis and initial or residual regurgitation are independent risk factors associated with truncal valve intervention. Larger truncal valve root z ‐score is associated with significant truncal valve regurgitation and may identify a subset of patients at risk for truncal valve dysfunction over time.

Background— Exercise oscillatory ventilation (EOV) refers to regular oscillations in minute ventilation (V E ) during exercise. Its presence correlates with heart failure severity and worse prognosis in adults with acquired heart failure. We evaluated the prevalence and predictive value of EOV in patients with single ventricle Fontan physiology. Methods and Results— We performed a cross-sectional analysis and prospective survival analysis of patients who had undergone a Fontan procedure and subsequent cardiopulmonary exercise test. Data were reviewed for baseline characteristics and incident mortality, heart transplant, or nonelective cardiovascular hospitalization. EOV was defined as regular oscillations for >60% of exercise duration with amplitude >15% of average V E . Survival analysis was performed using Cox regression. Among 253 subjects, EOV was present in 37.5%. Patients with EOV were younger (18.8±9.0 versus 21.7±10.1 years; P =0.02). EOV was associated with higher New York Heart Association functional class ( P =0.02) and V E /V CO 2 slope (36.8±6.9 versus 33.7±5.7; P =0.0002), but not with peak V O 2 (59.7±14.3 versus 61.0±16.0% predicted; P =0.52) or noninvasive measures of cardiac function. The presence of EOV was associated with slightly lower mean cardiac index but other invasive hemodynamic variables were similar. During a median follow-up of 5.5 years, 22 patients underwent transplant or died (n=19 primary deaths, 3 transplants with 2 subsequent deaths). EOV was associated with increased risk of death or transplant (hazard ratio, 3.9; 95% confidence interval, 1.5–10.0; P =0.002) and also predicted the combined outcome of death, transplant, or nonelective cardiovascular hospitalization after adjusting for New York Heart Association functional class, peak V O 2 , and other covariates (multivariable hazard ratio, 2.0; 95% confidence interval, 1.2–3.6; P =0.01). Conclusions— EOV is common in the Fontan population and strongly predicts lower transplant-free survival.

Background— Exercise echocardiography is often applied as a noninvasive strategy to screen for abnormal pulmonary hemodynamic response, but it is technically challenging, and limited data exist regarding its accuracy to estimate pulmonary arterial pressure during exercise. Methods and Results— Among 65 patients with exertional intolerance undergoing upright invasive exercise testing, tricuspid regurgitation (TR) Doppler estimates and invasive measurement of pulmonary arterial pressure at rest and peak exercise were simultaneously obtained. TR Doppler envelopes were assessed for quality. Correlation, Bland–Altman, and receiver-operating characteristic curve analyses were performed to evaluate agreement and diagnostic accuracy. Mean age was 62±13 years, and 31% were male. High-quality (grade A) TR Doppler was present in 68% at rest and 34% at peak exercise. For grade A TR signals, echocardiographic measures of systolic pulmonary arterial pressure correlated reasonably well with invasive measurement at rest ( r =0.72, P r =0.75, P 3.0 mm Hg/L per minute increase), with 91% sensitivity and 82% specificity (area under the curve, 0.90; 95% confidence interval, 0.77–1.0; P =0.001). Conclusions— Agreement between echocardiographic and invasive measures of pulmonary pressures during upright exercise is good among the subset of patients with high-quality TR Doppler signal. While the limits of agreement are broad, our results suggest that in those patients, sensitivity is adequate to screen for abnormal pulmonary hemodynamic response during exercise.

Background— Percutaneous pulmonary valve implantation using a stent-based bioprosthetic valve provides an alternative to surgery in select patients. Systemic infections in Melody valve–implanted patients with and without identified valve involvement have been reported, yet the incidence is unknown, and risk factors remain unidentified. Methods and Results— Between 2007 and 2012, a total of 147 consecutive patients with congenital heart disease underwent Melody percutaneous pulmonary valve implantation at our institution. Demographic and clinical variables were collected at baseline and at follow-up and analyzed as predictors. The occurrence of bloodstream infection (BSI), defined as a bacterial infection treated with ≥4 weeks of antibiotics, served as our primary outcome. The mean age at implantation for the study population was 21.5±11 years, and tetralogy of Fallot was the cardiac condition in 59%. During a median follow-up of 19 months, 14 patients experienced BSI (9.5%; 95% confidence interval, 5.3%–15%). Of these, 4 (2.7%) patients had Melody valve endocarditis. Two patients died during the event, neither of whom had known valve involvement. The median procedure to infection time was 15 months (range, 1–56). In univariate analysis, male sex, previous endocarditis, in situ stents in the right ventricular outflow tract, and presence of outflow tract irregularities at the implant site were associated with BSI occurrence. Conclusions— In this cohort, 9.5% of patients who underwent Melody percutaneous pulmonary valve implantation experienced subsequent BSI, occurring 1 to 56 months after implant, and 2.7% of patients had prosthetic endocarditis. Our findings suggest that patient and nonvalve anatomic factors may be associated with BSI after percutaneous pulmonary valve implantation.

Background— Pediatric hospitals frequently perform congenital heart surgery in adults with congenital heart disease. The impact of these admissions on pediatric hospital resources is unknown. Our goals were to examine resource use by adults undergoing congenital heart surgery in pediatric hospitals, explore the association between high resource use (HRU) and inpatient death, and identify HRU risk factors. Methods and Results— We obtained inpatient data from 42 pediatric hospitals from 2000 to 2008 and selected adult congenital heart (ACH) surgery admissions. We defined HRU admissions as those exceeding the 90th percentile for total hospital charges. We performed multivariable analyses using generalized estimating equations to identify risk factors for HRU. Of 97 563 congenital heart surgery admissions to pediatric hospitals, 3061 (3.1%) were adults, accounting for 2.2% of total hospital charges. The threshold for HRU was total hospital charges ≥$213 803. Although HRU admissions comprised 10% of admissions, they accounted for 34% of charges for all ACH surgery admissions. Mortality rate was 16% for HRU admissions and 0.7% for others ( P P =0.02), risk category 3 (AOR, 13.7; P P P =0.006); depression (AOR, 3.1; P P P Conclusions— High resource use ACH surgery admissions are associated with significantly greater mortality rates. ACH admissions with greater surgical complexity, government insurance, DiGeorge syndrome, weekend admission, and depression were more likely to result in HRU.

Background Most case reports of pregnancies after surgical repair of tetralogy of Fallot have focused on cardiovascular and obstetric concerns, with relatively few authors focusing on specific intrapartum and postpartum anesthetic management strategies. Methods The Mayo Clinic Congenital Heart Disease Clinic and the Boston Adult Congenital Heart Disease Service databases were cross-referenced with the Mayo Clinic and the Brigham and Women's Hospital Department of Anesthesiology databases to identify patients with tetralogy of Fallot who delivered at their respective hospital from January 1, 1994, to January 1, 2008. We reviewed each medical record to evaluate parturient care during pregnancy, labor, and delivery with a focus on anesthetic management. Results During the 14-year study period, a total of 27 deliveries in 20 patients with repaired tetralogy of Fallot were identified. Twenty-one deliveries (78%) among 15 parturients (75%) involved a trial of labor; all parturients received neuraxial analgesia for labor and delivery, including 18 (86%) epidural, 2 (10%) combined spinal-epidural, and 1 (5%) continuous spinal anesthetic after an unintended dural puncture. Of the 21 patients undergoing labor, 3 (14%) received invasive arterial blood pressure monitoring; 5 (24%) received continuous telemetry; 3 (14%) experienced congestive heart failure that required diuresis; 4 (19%) had obstetric or neonatal complications; and 3 (14%) had anesthesia complications. Cesarean delivery was required in 4 patients (19%) because of labor complications. Concurrent cardiovascular, obstetric, and anesthetic complications in 1 patient resulted in neonatal death. Six (22%) parturients underwent elective cesarean delivery; 4 received epidural and 2 received spinal anesthesia; no anesthetic or immediate obstetric complications occurred. Among all parturients, 5 deliveries in 5 separate parturients (19% of deliveries) reported symptoms of congestive heart failure at the time of delivery. Conclusions Pregnancy outcomes for patients with repaired tetralogy of Fallot were found to be generally favorable. All patients undergoing a trial of labor or cesarean delivery had neuraxial analgesia or anesthesia. Recognition and management of congestive heart failure was necessary in 19% of deliveries.

Background— Despite the central role that pediatric hospitals play in the surgical treatment of congenital heart disease, little is known about outcomes of adult congenital cardiac surgical care in pediatric hospitals. Risk factors for inpatient death, including adult congenital heart (ACH) surgery volume, are poorly described. Methods and Results— We obtained inpatient data from 42 free-standing pediatric hospitals using the Pediatric Health Information System data base 2000 to 2008 and selected ACH surgery admissions (ages 18 to 49 years). We examined admission characteristics and hospital surgery volume. Of 97 563 total (pediatric and adult) congenital heart surgery admissions, 3061 (3.1%) were ACH surgery admissions. Median adult age was 22 years and 39% were between ages 25 to 49 years. Most frequent surgical procedures were pulmonary valve replacement, secundum atrial septal defect repair, and aortic valve replacement. Adult mortality rate was 2.2% at discharge. Multivariable analyses identified the following risk factors for death: age 25 to 34 years (adjusted odds ratio [AOR], 2.1; P =0.009), age 35 to 49 years (AOR, 3.2; P =0.001), male sex (AOR, 1.8; P =0.04), government-sponsored insurance (AOR, 1.8; P =0.03), and higher surgical risk categories 4+ (AOR, 21.5; P =0.001). After adjusting for case mix, pediatric hospitals with high ACH surgery volume had reduced odds for death (AOR, 0.4; P =0.003). There was no relationship between total congenital heart surgery volume and ACH inpatient mortality. Conclusions— Older adults, male sex, government-sponsored insurance, and greater surgical case complexity have the highest likelihood of in-hospital death when adult congenital surgery is performed in free-standing pediatric hospitals. After risk-adjustment, pediatric hospitals with high ACH surgery volume have the lowest inpatient mortality.

Background— Significant morbidities, including angina, symptomatic heart failure, and myocardial infarction, have been reported after coronary artery fistula (CAF) closure; however, predictors that may be associated with adverse outcomes have not been established. The goal of this investigation is to describe the long-term outcomes witnessed in patients with either treated or untreated CAF at our institution and to investigate whether certain features predicted adverse outcomes. Methods and Results— The records and angiograms of patients with CAF who underwent a diagnostic cardiac catheterization at Children’s Hospital Boston from 1959 through 2008 were reviewed. Of 76 patients identified, 20% were associated with additional congenital heart disease. Forty-four underwent transcatheter closure, 20 underwent surgical repair, and no intervention was performed in the remaining 12 subjects. Three patients who had initially undergone surgical closure had a second intervention, 1 underwent repeat surgery, and 2 underwent transcatheter closure. One patient who had undergone transcatheter closure underwent a second transcatheter closure for residual fistula. Major complications, including myocardial infarction, angina with coronary thrombosis, and symptomatic cardiomyopathy, occurred in 11 (15%) patients. The sole angiographic feature that was predictive of adverse outcome was drainage of the CAF into the coronary sinus ( P P P =0.006), diabetes ( P =0.05), systemic hypertension ( P P Conclusions— Long-term complications of CAF closure may include coronary thrombosis, myocardial infarction, and cardiomyopathy. CAF that drain into the coronary sinus are at particularly high-risk of long-term morbidities after closure, and strategies including long-term anticoagulation should be considered.

Background— To better define determinants of mortality in patients with univentricular physiology, a database registry was created of patients born in 1985 or earlier with Fontan surgery who were followed up at Children’s Hospital Boston. Methods and Results— A total of 261 patients, 121 of whom (46.4%) were women, had a first Fontan surgery at a median age of 7.9 years: right atrium–to–pulmonary artery connection in 135 (51.7%); right atrium to right ventricle in 25 (9.6%); and total cavopulmonary connection in 101 (38.7%). Over a median of 12.2 years, 76 (29.1%) died, 5 (1.9%) had cardiac transplantation, 5 (1.9%) had Fontan revision, and 21 (8.0%) had Fontan conversion. Perioperative mortality decreased steadily over time and accounted for 68.4% of all deaths. In early survivors, actuarial freedom from death or transplantation was 93.7%, 89.9%, 87.3%, and 82.6% at 5, 10, 15, and 20 years, respectively, with no significant difference between right atrium to pulmonary artery versus total cavopulmonary connection. Late deaths were classified as sudden in 7 patients (9.2%), thromboembolic in 6 (7.9%), heart failure–related in 5 (6.7%), sepsis in 2 (2.6%), and other in 4 (5.2%). Most sudden deaths were of presumed arrhythmic origin with no identifiable predictor. Independent risk factors for thromboembolic death were lack of antiplatelet or anticoagulant therapy (hazard ratio [HR], 91.6; P =0.0041) and clinically diagnosed intracardiac thrombus (HR, 22.7; P =0.0002). Independent predictors of heart failure death were protein-losing enteropathy (HR, 7.1; P =0.0043), single morphologically right ventricle (HR, 10.5; P =0.0429), and higher right atrial pressure (HR, 1.3 per 1 mm Hg; P =0.0016). Conclusion— In perioperative survivors of Fontan surgery, gradual attrition occurs predominantly from thromboembolic, heart failure–related, and sudden deaths.

Background— The risk of systemic thromboemboli associated with transvenous leads in the presence of an intracardiac shunt is currently unknown. Methods and Results— To define this risk, we conducted a multicenter, retrospective cohort study of 202 patients with intracardiac shunts: Sixty-four had transvenous leads (group 1), 56 had epicardial leads (group 2), and 82 had right-to-left shunts but no pacemaker or implantable cardioverter defibrillator leads (group 3). Patient-years were accrued until the occurrence of systemic thromboemboli or study termination. Censoring occurred in the event of complete shunt closure, death, or loss to follow-up. Mean ages for groups 1, 2, and 3 were 33.9±18.0, 22.2±12.6, and 22.9±15.0 years, respectively. Respective oxygen saturations were 91.2±9.1%, 88.1±8.1%, and 79.7±6.7%. During respective median follow-ups of 7.3, 9.3, and 17.0 years, 24 patients had at least 1 systemic thromboembolus: 10 (15.6%), 5 (8.9%), and 9 (11.0%) in groups 1, 2, and 3, respectively. Univariate risk factors were older age (hazard ratio [HR], 1.05; P =0.0001), ongoing phlebotomy (HR, 3.1; P =0.0415), and an transvenous lead (HR, 2.4; P =0.0421). In multivariate, stepwise regression analyses, transvenous leads remained an independent predictor of systemic thromboemboli (HR, 2.6; P =0.0265). In patients with transvenous leads, independent risk factors were older age (HR, 1.05; P =0.0080), atrial fibrillation or flutter (HR, 6.7; P =0.0214), and ongoing phlebotomy (HR, 14.4; P =0.0349). Having had aspirin or warfarin prescribed was not protective. Epicardial leads were, however, associated with higher atrial ( P =0.0407) and ventricular ( P =0.0270) thresholds and shorter generator longevity (HR, 1.9; P =0.0176). Conclusions— Transvenous leads incur a >2-fold increased risk of systemic thromboemboli in patients with intracardiac shunts.

Background— Pregnant women with congenital heart disease are at increased risk for cardiac and neonatal complications, yet risk factors for adverse outcomes are not fully defined. Methods and Results— Between January 1998 and September 2004, 90 pregnancies at age 27.7±6.1 years were followed in 53 women with congenital heart disease. Spontaneous abortions occurred in 11 pregnancies at 10.8±3.7 weeks, and 7 underwent elective pregnancy termination. There were no maternal deaths. Primary maternal cardiac events complicated 19.4% of ongoing pregnancies, with pulmonary edema in 16.7% and sustained arrhythmias in 2.8%. Univariate risk factors included prior history of heart failure (odds ratio [OR], 15.5), NYHA functional class ≥2 (OR, 5.4), and decreased subpulmonary ventricular ejection fraction (OR, 7.7). Independent predictors were decreased subpulmonary ventricular ejection fraction and/or severe pulmonary regurgitation (OR, 9.0) and smoking history (OR, 27.2). Adverse neonatal outcomes occurred in 27.8% of ongoing pregnancies and included preterm delivery (20.8%), small for gestational age (8.3%), respiratory distress syndrome (8.3%), intraventricular hemorrhage (1.4%), intrauterine fetal demise (2.8%), and neonatal death (1.4%). A subaortic ventricular outflow tract gradient >30 mm Hg independently predicted an adverse neonatal outcome (OR, 7.5). Cardiac risk assessment was improved by including decreased subpulmonary ventricular systolic function and/or severe pulmonary regurgitation (OR, 10.3) in a previously proposed risk index developed in pregnant women with acquired and congenital heart disease. Conclusions— Maternal cardiac and neonatal complication rates are considerable in pregnant women with congenital heart disease. Patients with impaired subpulmonary ventricular systolic function and/or severe pulmonary regurgitation are at increased risk for adverse cardiac outcomes.

Background— Studies have consistently shown that ventricular tachycardia (VT) and sudden cardiac death (SCD) complicate the long-term outcome after tetralogy of Fallot repair, yet the diagnostic and predictive value of electrophysiological testing in this population is uncertain. Methods and Results— A multicenter cohort of 252 patients with repaired tetralogy of Fallot undergoing programmed ventricular stimulation was followed up for 18.5±9.6 and 6.5±4.5 years after corrective surgery and electrophysiological testing, respectively. Clinical VT and/or SCD occurred in 24.6%. Sustained monomorphic VT and polymorphic VT were induced in 30.2% and 4.4%. Including polymorphic VT in the definition of inducibility improved sensitivity (66.1±6.0% versus 77.4±5.3%, P =0.0082) with a marginal reduction in specificity (81.6±2.8% versus 79.5±2.9%, P =0.0455). Positive and negative predictive values were 55.2±5.3% and 91.5±2.2%. Independent risk factors for inducibility were age at study ≥18 years (OR, 3.3), palpitations (OR, 2.8), prior palliative surgery (OR, 3.1), modified Lown criteria ≥2 (OR, 5.6), and cardiothoracic ratio ≥0.6 (OR, 3.3). Event-free survival rates in noninducible and inducible patients at 1, 5, 10, and 15 years were 97.9%, 92.8%, 89.3%, and 89.3% versus 79.4%, 62.6%, 58.7%, and 50.3%, respectively ( P P =0.0002] and polymorphic VT (RR, 12.9; P P =0.0268). Conclusions— Programmed ventricular stimulation is of diagnostic and prognostic value in risk stratifying patients with repaired tetralogy of Fallot. In this patient population, inducible sustained polymorphic VT should not be disregarded as nonspecific.

Background —Although pulmonary thromboendarterectomy is increasingly successful for the definitive treatment of chronic thromboembolic pulmonary hypertension (CTEPH), not all patients have surgically accessible disease. Others are poor surgical candidates because of comorbid illness. Therefore, for selected patients, we defined and implemented an alternative interventional strategy of balloon pulmonary angioplasty (BPA). Methods and Results —Eighteen patients (mean age, 51.8 years; range, 14 to 75 years) with CTEPH underwent BPA; they averaged 2.6 procedures (range, 1 to 5) and 6 dilations (range, 1 to 12). Selection of pulmonary artery segments for dilation required (1) complete occlusion, (2) filling defects, or (3) signs of intravascular webs. After an average of 36 months of follow-up (range, 0.5 to 66 months), the average New York Heart Association class improved from 3.3 to 1.8 ( P P P =0.007). Eleven patients developed reperfusion pulmonary edema; 3 required mechanical ventilation. Conclusions —BPA reduces pulmonary artery hypertension in patients with CTEPH and is associated with long-term improvement in New York Heart Association class and 6-minute walking distances. BPA is a promising interventional technique that warrants randomized comparison with medical therapy in CTEPH patients who are not surgical candidates.

Background Endothelium-derived nitric oxide (NO) may be an important mediator of vascular resistance in the pulmonary circulation. We tested the hypotheses that in conscious adults the endothelium, through NO production, is important in maintaining basal pulmonary vascular resistance and that it can increase NO production further in response to receptor-mediated stimulation, leading to further vasodilation. Methods and Results Pulmonary arterial resistance vessel function was studied within the distribution of a segmental lower lobe pulmonary artery in eight conscious adults 37 to 76 years old who were undergoing cardiac catheterization. Segmental blood flow was determined with use of a Doppler-tip guide wire and quantitative angiography. Drugs were administered locally within the segmental artery through an infusion catheter. N G -Monomethyl- l -arginine (L-NMMA) was used as a specific inhibitor of NO production, whereas acetylcholine (ACh) was used to test receptor-mediated vasodilation. To demonstrate that vasodilation to ACh was NO dependent, ACh response was tested alone, in the presence of L-NMMA, and in the presence of a control constrictor phenylephrine. Basal pulmonary vascular resistance was NO dependent because L-NMMA infusion resulted in a dose-dependent decrease in local flow velocity ( P P =.001). The ACh response was at least in part NO dependent because it was diminished by the presence of L-NMMA ( P P Conclusions In healthy conscious adults, (1) normal basal pulmonary resistance is maintained in part by continuous local production of NO and (2) the local NO production is responsive to receptor-mediated stimulation, leading to further vasodilation, and can be tested with ACh.

For more than half a century, growing numbers of practitioners and clinician-scientists have created systems for and practiced healthcare delivery within centers of expertise for young and older adults with congenital heart disease (CHD). To date, this has been driven largely by energy, enthusiasm, and shared mission without supportive data demonstrating the benefit of such adult CHD (ACHD) care programs in the lives of patients and families served by this developing field. Now, for the first time, Mylotte and colleagues1 present in this issue of Circulation good evidence that there is a survival advantage to ACHD patients being cared for by ACHD specialists. This confirms what those working in this field had long assumed and hoped but to date could not substantiate. Article see p 1804 The authors used their own extensively validated, province-wide, administrative longitudinal database that has been highly effective in demonstrating the demography and epidemiology of ACHD care in Quebec, Canada, as a model for North America. Their study included data from 1990 to 2005. They performed an elegant combination of time-series analysis together with case-control studies that included year-dependent survival and center-dependent survival so as to determine mortality trends within a highly monitored and geographically stable group of adults with CHD. A number of important findings emerged. Specialized ACHD care was independently associated with reduced mortality. Among patients who died, just under half died of cardiovascular diseases. Other causes of death included neoplasms (≈18%), respiratory disease (≈9%), and diseases of other systems …

We thank Witte et al for their interest in our article and encourage the critical appraisal of implantable cardioverter-defibrillator (ICD) therapy at large and in tetralogy of Fallot (TOF) in particular. We concur that decisions regarding ICD implantation should be thoughtfully considered in light of anticipated risks, known complications, and potential benefits. Indeed, one of our stated objectives was to provide caregivers with evidence-based data regarding ICD-related complications, as derived from a multicenter experience. These complications were undeniably substantial. Inappropriate shocks were observed in 5.8% of patients per year, and 80% of those shocks were triggered by sinus or supraventricular tachycardia.1 Complications other than inappropriate shocks occurred in 30% and were predominantly late lead–related. From a psychosocial perspective, feelings of anxiety and depression have been previously characterized in youths with ICDs, although most remain …

Grow old along with me! The best is yet to be, The last of life, for which the first was made: Our times are in His hand Who saith, “A Whole I planned, Youth shows but half; … —Robert Browning, “Rabbi Ben Ezra”1 In his poem “Rabbi Ben ( sic Ibn) Ezra,” Robert Browning acknowledges the multitude of physical and nonphysical determinants of life’s richness. His narrator sees these influences as interwoven and spanning the ages from youth through adulthood in timeless fashion. In this week’s issue of Circulation , Bouchardy and colleagues2 determine the prevalence of atrial (tachy)arrhythmias (AA) in adults with congenital heart disease (ACHD) and then analyze the prognostic implications of such. The authors’ findings carry far-reaching implications that, in a fashion that Browning’s narrator would have advocated, extend to principles, design, and application of medical care that begin at the earliest pediatric years and continue throughout the entire development and aging of the ACHD population. Article see p 1679 Nearly all physicians come into contact with ACHD because current estimates support a prevalence of >1 million adults and nearly 1 million children with congenital heart disease in the United States.3 Analyses of large administrative data sets have emphasized the growing burden (in excess to matched controls) of both cardiovascular and noncardiovascular (diabetes mellitus, renal dysfunction, obesity) disease currently existing in this ACHD population.4 Similarly, all physicians care for patients with AAs, the most common heart rhythm disorder that affects adults. Atrial fibrillation dominates (affecting >2.3 million Americans, with a prevalence of 0.4% to 5.5%, depending on age) and is a major source of morbidity and mortality because its onset heralds a 4- to 5-fold increase in stroke, a doubling of risk for dementia, a tripling of …

It has long been recognized that manifestations of congenital heart disease extend beyond the cardiovascular system. The extracardiac ramifications of congenital heart disease are multifaceted and bidirectional. The intricate interplay between congenital heart physiology, bi- or univentricular heart function, early and/or ongoing cyanosis, surgical and residual sequelae, and multisystemic effects are increasingly appreciated. Examples include the well-characterized neurological, hematologic, respiratory, skeletal, and hepatic consequences of cyanosis1; thromboembolic complications in surgically palliated univentricular hearts2; and dysregulation of the autonomic nervous system in various forms of congenital heart disease.3 Article p 2320 In the present issue of Circulation , Dimopoulos and associates4 provide original and important insights regarding the relationship between congenital heart disease and impaired renal function. They examine the prevalence of renal dysfunction across a wide-spectrum of adult congenital heart defects and explore implications on overall death rates. ### Heterogeneity of the Study Population A general and common challenge in congenital heart disease research that encompasses a broad spectrum of cardiac disorders is the complexity and marked heterogeneity of the study population. From a methodological perspective, there is often a trade-off between heterogeneity of the patient population and the precision of a given statistical inference. When the objective is to provide a global perspective on a novel association, such as renal dysfunction and death in adults with congenital heart disease, however, heterogeneity of the patient population may be advantageous. The nonrestrictive inclusion criteria enhance generalizability of the findings to a broad target population. Although the reported overall 9.3% prevalence of moderate to severe renal dysfunction must be interpreted within the context of a high-volume referral center, it reflects a wide spectrum of congenital heart disease subtypes. Heterogeneity of the study population also allows exploration of gradients …