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Introduction: Good outcomes in adults with repaired tetralogy of Fallot (rTOF) are not well explored. Identifying markers of a benign clinical course late after TOF repair can guide resource utilization in this growing population. Methods: Clinical and CMR data were analyzed from the International Multicenter TOF Registry (INDICATOR). The clinical outcome was a composite of death, aborted sudden death, sustained ventricular and atrial arrhythmia, non-sustained ventricular tachycardia, and New York Heart Association functional class>II. Multinomial regression explored predictors of the 3-category outcome-Good and intermediate outcomes; defined as freedom from clinical outcome at 50 and Results: The cohort had 1088 patients-good outcome, n=96; intermediate, n=747; bad, n=245 with median age at last CMR 44 (40, 47); 21 (15, 28); 31 (20, 39) years respectively. Median follow-up was 8 (5, 11) years after CMR in event-free patients. Right ventricular (RV) parameters associated with a good outcome were smaller RV end-systolic volume index, higher RV ejection fraction (EF), lower RV mass index, and lower RV mass/volume ratio. Left ventricular (LV) parameters associated with a good outcome were lower LV mass index and LV mass/volume ratio. Multivariable models showed RV EF (OR 1.56 per 10% increase, p=.009) and RV mass index (OR 1.51, per 10 g/m 2 decrease, p=.002) as independently associated with good outcome after adjusting for age at CMR. Important thresholds identified in older patients (age ≥37 years) were RVEF ≥42% and RV mass index 2 . Combined, this subgroup had a 56% likelihood of a good outcome. Conclusions: Adults with rTOF and CMR findings of adequate RV systolic function and no significant ventricular hypertrophy are likely to have a benign clinical course by age 50 years. Frequency of cardiac testing in such patients may be lower than in those not fulfilling these criteria.

Background— Pregnant women with congenital heart disease (CHD) are susceptible to cardiovascular, obstetric, and offspring complications. In women with CHD, cardiac dysfunction may compromise uteroplacental flow and contribute to the increased incidence of obstetric and offspring events. Methods and Results— We performed a prospective multicenter cohort study of pregnant women with CHD and healthy pregnant women. We compared clinical, laboratory, echocardiographic, and uteroplacental Doppler flow (UDF) parameters at 20 and 32 weeks gestation, and pregnancy outcome. We related cardiovascular parameters to UDF parameters and pregnancy outcome in women with CHD. We included 209 women with CHD and 70 healthy women. Cardiovascular parameters (N-terminal pro-B-type natriuretic peptide, left and right ventricular function) differed between both groups. UDF parameters were impaired in CHD women (umbilical artery pulsatility and resistance index at 32 weeks in CHD versus healthy women, P =0.0085 and P =0.017). The following cardiovascular parameters prepregnancy and at 20 weeks gestation were associated with UDF (umbilical artery resistance index) at 32 weeks at multivariable analysis: (1) right ventricular function (tricuspid annular plane systolic excursion) ( P =0.002), (2) high N-terminal pro-B-type natriuretic peptide ( P =0.085), (3) systemic ( P =0.001), and (4) pulmonary ( P =0.045) atrioventricular valve regurgitation. Women with CHD had more obstetric (58.9% versus 32.9%, P P =0.008) than healthy women. Impaired UDF was associated with adverse obstetric and offspring outcome. Conclusions— UDF parameters are abnormal in pregnant women with CHD. Cardiovascular function is associated with an abnormal pattern of UDF. Compromised UDF may be a key factor in the high incidence of offspring and obstetric complications in this population.

Background— Sudden cardiac death (SCD) is a major cause of mortality in adults with congenital heart disease (CHD). The aim of this study was to determine the adult CHD population at risk of SCD and the clinical parameters associated with SCD. Methods and Results— We performed a multicenter case-control study. Patients who died suddenly as a result of proven or presumed arrhythmia were included (cases). For each case, 2 controls matched on diagnosis, type of surgical intervention, age, and gender were included. From 3 databases including 25 790 adults with CHD, 1189 deaths (5%) were identified, of whom 213 patients (19%) died suddenly. Arrhythmic death occurred in 171 of 1189 patients. The underlying cardiac lesions were mild, moderate, and severe CHD in 12%, 33%, and 55% of the SCD cases, respectively. Clinical variables associated with SCD were supraventricular tachycardia (odds ratio [OR], 3.5; 95% confidence interval [CI], 1.5–7.9; P= 0.004), moderate to severe systemic ventricular dysfunction (OR, 3.4; 95% CI, 1.1–10.4; P= 0.034), moderate to severe subpulmonary ventricular dysfunction (OR, 3.4; 95% CI, 1.1–10.2; P= 0.030), increased QRS duration (OR, 1.34 [per 10-ms increase]; 95% CI, 1.10–1.34; P =0.008), and QT dispersion (OR, 1.22 [per 10-ms increase]; 95% CI, 1.22–1.48; P =0.008). Conclusions— The clinical parameters found to be associated with SCD in adults with a broad spectrum of CHD, including systemic right ventricles, are similar to those in ischemic heart disease. Moreover, even those patients with mild cardiac lesions are potentially at risk for SCD. This highlights the need for further prospective studies as well as vigilant ongoing follow-up of the adult with CHD.

Background— In patients with a right ventricle (RV) in the systemic position, tricuspid valve surgery for regurgitation beyond adolescence is a subject of debate. The aim of the present study was to evaluate the complications, survival, and benefit of tricuspid surgery in adult patients with an atrium-level correction for transposition of the great arteries or congenitally corrected transposition of the great arteries. Methods and Results— All adult patients (n=16; 7 men, 9 women; age 35±11 years) who underwent tricuspid valvuloplasty (n=8) or replacement (n=8) in the period 1999 to 2008 were included. Complications and survival were analyzed, and postoperative changes in RV function and functional class were evaluated. Tricuspid regurgitation was graded 1 to 4 according to its severity, RV dysfunction was graded as 1 to 4 (1=no dysfunction to 4=severe dysfunction), and functional status was determined according to New York Heart Association class. Although complications occurred in 11 patients, all could be managed adequately. Three patients died 109, 180, and 659 days after surgery, respectively, the first patient after tricuspid valve replacement and the latter 2 after tricuspid valvuloplasty. Overall, tricuspid valve function improved (from grade 3.1±0.8 to 0.9±1.0; P =0.001) and functional class improved (from 2.7±0.6 to 2.1±0.8; P =0.007), whereas RV function remained unchanged. After tricuspid valvuloplasty, however, recurrent moderate tricuspid valve regurgitation was observed frequently (n=3; 37%). Conclusions— Mortality is rather low after tricuspid surgery in adult patients with mild to moderate RV dysfunction. In general, tricuspid valve function and functional class improve significantly after surgery, and systemic RV function is preserved. Tricuspid valvuloplasty, however, is associated with a high rate of recurrence of regurgitation.

We thank Drs Le-Xin Wang and Zeng-Shan Ma for their comments on our publication.1 The authors state that pre- and postoperative pulmonary arterial pressure and ventricular function are important factors influencing long-term survival after corrective surgery.2,3 Therefore, they contend that without considering these factors in the analyses of long-term survival after surgery, the sex differences we reported require cautious interpretation. We recognize that pulmonary arterial pressure and ventricular function are important factors influencing long-term outcome after surgery.2,3 However, we …

To the Editor: In their recent article, Mackie et al1 demonstrated that 61% of adult patients with congenital heart disease (CHD) in Quebec, Canada, fail to receive cardiac follow-up. In the Netherlands, we found that only one third (≈8000) of adult patients with CHD fail to receive cardiac follow-up. Therefore, caution with extrapolating these data to other nations is recommended. On average, 200 000 children are born in the Netherlands each year. With a birth prevalence of around 8 per 1000 live births2 and an overall survival rate until adulthood of 85% since the early 1970s, there are an estimated 25 …

The risk of infective endocarditis (IE) remains a major concern in patients with congenital heart disease (CHD), whether unrepaired, palliated, or corrected. The overall incidence of endocarditis in adults with CHD has been reported to be 11 per 100 000 person-years, which is a considerable increase compared with the general population, in which a rate of 1.5 to 6.0 per 100 000 patient-years has been reported.1,2 In children, the incidence of IE in the general population is ≈3 times lower.3 Article see p 1412 CHD is the most prevalent underlying cardiac condition in patients with IE. Despite recommendations for antibiotic prophylaxis of IE, increased survival of children with CHD and the use of conduits and prostheses in corrective surgery may have contributed to an increasing incidence of IE.4 However, CHD-associated IE mortality has decreased substantially to 10% because of improvements in the diagnosis of IE, antimicrobial treatment, cardiac surgery, and interventional therapy.3,5 Given the prognosis, morbidity, and high cost of management of IE, IE prophylaxis has long been recommended in an attempt to minimize the incidence of IE. However, no randomized study has been conducted yet to elucidate the efficacy and usefulness of IE prophylaxis. Based on case-control studies, expert opinions, and daily practice, guidelines on IE prophylaxis from international cardiology societies in 2008/2009 were greatly simplified and resulted in a drastic reduction in and limitation of cardiac diseases and procedures in which IE prophylaxis is indicated.6,7 The guidelines now emphasize the role of primary prevention and limit antibiotic prophylaxis to the highest-risk patients undergoing the highest-risk procedures. Motivated largely by lack of robust evidence of the effectiveness of antibiotic prophylaxis and a growing recognition of the importance of bacteremias from routine …