Cardiac outcomes after pregnancy in women with congenital heart disease

Objective: Women with congenital heart disease (CHD) are at risk for adverse cardiac events during pregnancy; however, the risk of events late after pregnancy (late cardiac events; LCE) has not been well studied. A study was undertaken to examine the frequency and determinants of LCE in a large cohort of women with CHD. Design: Baseline characteristics and pregnancy were prospectively recorded. LCE (\u3e6 months after delivery) were determined by chart review. Survival analysis was used to determine the risk factors for LCE. Setting: A tertiary care referral hospital. Patients: The outcomes of 405 pregnancies were studied (318 women; median follow-up 2.6 years). Main outcome measures: LCE included cardiac death/ arrest, pulmonary oedema, arrhythmia or stroke. Results: LCE occurred after 12% (50/405) of pregnancies. The 5-year rate of LCE was higher in women with adverse cardiac events during pregnancy than in those without (27±9% vs 15±3%, HR 2.2, p=0.02). Women at highest risk for LCE were those with functional limitations/cyanosis (HR 3.9, 95% CI 1.2 to 13.0), subaortic ventricular dysfunction (HR 3.0, 95% CI 1.4 to 6.6), subpulmonary ventricular dysfunction and/or significant pulmonary regurgitation (HR 3.2, 95% CI 1.6 to 6.6), left heart obstruction (HR 2.6, 95% CI 1.2 to 5.2) and cardiac events before or during pregnancy (HR 2.6, 95% CI 1.3 to 4.9). In women with 0, 1 or \u3e1 risk predictors the 5-year rate of LCE was 762%, 2365% and 44610%, respectively (p\u3c0.001). Conclusions: In women with CHD, pre-pregnancy maternal characteristics can help to identify women at increased risk for LCE. Adverse cardiac events during pregnancy are important and are associated with an increased risk of LCE

Cardiac outcomes in adults with supravalvar aortic stenosis

Aims Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. Little data are available on outcomes in the adult population. Our aim was to define cardiac outcomes in adults with supravalvar aortic stenosis. Methods and results This is a multicentre retrospective study of cardiac outcomes in adults (≥18 years) with supravalvar aortic stenosis. We examined: (i) adverse cardiac events (cardiovascular death, myocardial infarction, stroke, heart failure, sustained arrhythmias, and infective endocarditis) and (ii) the need for cardiac surgery in adulthood. One hundred and thirteen adults (median age at first visit 19 years; 55% with Williams-Beuren syndrome; 67% with surgical repair in childhood) were identified. Adults without Williams-Beuren syndrome had more severe supravalvar aortic stenosis and more often associated left ventricular outflow tract obstructions (P < 0.001). In contrast, mitral valve regurgitation was more common in patients with Williams-Beuren syndrome. Eighty-five per cent of adults (96/113) had serial follow-up information (median follow-up 6.0 years). Of these patients, 13% (12/96) had an adverse cardiac event and 13% (12/96) had cardiac operations (7 valve repair or replacements, 4 supravalvar aortic stenosis repairs, 1 other). Cardiac surgery was more common in adults without Williams-Beuren syndrome (P = 0.007). Progression of supravalvar aortic stenosis during adulthood was rare. Conclusion Adults with supravalvar aortic stenosis remain at risk for cardiac complications and reoperations, while progression of supravalvar aortic stenosis in adulthood is rare. Valve surgery is the most common indication for cardiac surgery in adulthoo

Validation of noncompaction criteria in dilated cardiomyopathy, and valvular and hypertensive heart disease

BACKGROUND: Echocardiographic characteristics typical of isolated left ventricular noncompaction (IVNC) have been well defined. The aim of this study was to validate diagnostic criteria of IVNC in valvular or hypertensive heart disease (HHD) or dilated cardiomyopathy. METHODS: We conducted a retrospective analysis of records and blind review of videotapes of all 19 patients with IVNC seen within 7 years in comparison with randomly selected patients from the same study period with dilated cardiomyopathy (31 patients), HHD (22 patients), and chronic severe valvular heart disease: mitral regurgitation (22 patients); aortic regurgitation (20); and aortic stenosis with bicuspid (22) or tricuspid (22) valves. RESULTS: Clinical characteristics and electrocardiographic findings did not differ between IVNC and other diseases. In IVNC, all patients had noncompacted (NC) segments with a 2-layered structure and wall thickening, and in most patients perfused recesses (95%) or hypokinetic segments (89%) were present. Both hypertrabeculation or presence of a meshwork were specific for IVNC, but the sensitivity for IVNC was only 11% for hypertrabeculation, respectively, 68% for meshwork. In dilated cardiomyopathy, perfused recesses (48%) and a 2-layered structure (26%) were seen but without wall thickening of these segments; all NC criteria including wall thickening were fulfilled in one patient (3%) only. In valvular heart disease or HHD, perfused recesses and a 2-layered myocardium were rare: two patients (5%) with aortic stenosis and one patient with HHD (5%) had NC. Although in IVNC wall thickening was confined to the 2-layered myocardial segments, it was diffuse in other diseases. CONCLUSIONS: Although some NC criteria are occasionally found in other heart disease, the combination of all criteria is very specific. All criteria of NC are rarely met in other disease than IVNC (< or = 5%)

Learning strategies among adult CHD fellows

Objective:Subspecialisation is increasingly a fundamental part of the contemporary practice of medicine. However, little is known about how medical trainees learn in the modern era, and particularly in growing and relatively new subspecialties, such as adult CHD. The purpose of this study was to assess institutional-led and self-directed learning strategies of adult CHD fellows.Methods:This international, cross-sectional online survey was conducted by the International Society for Adult Congenital Heart Disease and consisted primarily of categorical questions and Likert rating scales. All current or recent (i.e., those within 2 years of training) fellows who reported training in adult CHD (within adult/paediatric cardiology training or within subspecialty fellowships) were eligible.Results:A total of 75 fellows participated in the survey: mean age: 34 ± 5; 35 (47%) female. Most adult CHD subspecialty fellows considered case-based teaching (58%) as "very helpful", while topic-based teaching was considered "helpful" (67%); p = 0.003 (favouring case-based). When facing a non-urgent clinical dilemma, fellows reported that they were more likely to search for information online (58%) than consult a faculty member (29%) or textbook (3%). Many (69%) fellows use their smartphones at least once daily to search for information during regular clinical work.Conclusions:Fellows receiving adult CHD training reported a preference for case-based learning and frequent use of online material and smartphones. These findings may be incorporated into the design and enhancement of fellowships and development of online training resources

Prolonged T-peak-T-end 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 Tpeak -Tend (TpTe) interval, a measure of dispersion of ventricular repolarization, 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 identified all SCD cases. Cases were matched to controls by age, gender, congenital defect, 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.status: publishe