The care of adults with congenital heart disease across the globe: Current assessment and future perspective: A position statement from the International Society for Adult Congenital Heart Disease (ISACHD).

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.

Optimising echocardiographic screening for rheumatic heart disease in New Zealand: not all valve disease is rheumatic.

AIMS: Echocardiography detects a greater prevalence of rheumatic heart disease than heart auscultation. Echocardiographic screening for rheumatic heart disease combined with secondary prophylaxis may potentially prevent severe rheumatic heart disease in high-risk populations. We aimed to determine the prevalence of rheumatic heart disease in children from an urban New Zealand population at high risk for acute rheumatic fever. METHODS AND RESULTS: To optimise accurate diagnosis of rheumatic heart disease, we utilised a two-step model. Portable echocardiography was conducted on 1142 predominantly Maori and Pacific children aged 10-13 years. Children with an abnormal screening echocardiogram underwent clinical assessment by a paediatric cardiologist together with hospital-based echocardiography. Rheumatic heart disease was then classified as definite, probable, or possible. Portable echocardiography identified changes suggestive of rheumatic heart disease in 95 (8.3%) of 1142 children, which reduced to 59 (5.2%) after cardiology assessment. The prevalence of definite and probable rheumatic heart disease was 26.0 of 1000, with 95% confidence intervals ranging from 12.6 to 39.4. Portable echocardiography overdiagnosed rheumatic heart disease with physiological valve regurgitation diagnosed in 28 children. A total of 30 children (2.6%) had non-rheumatic cardiac abnormalities, 11 of whom had minor congenital mitral valve anomalies. CONCLUSIONS: We found high rates of undetected rheumatic heart disease in this high-risk population. Rheumatic heart disease screening has resource implications with cardiology evaluation required for accurate diagnosis. Echocardiographic screening for rheumatic heart disease may overdiagnose rheumatic heart disease unless congenital mitral valve anomalies and physiological regurgitation are excluded.

Lung abscess in adults with tetralogy of fallot and pulmonary atresia.

We describe 5 adults with tetralogy of Fallot and pulmonary atresia who developed lung abscesses, including some infected with atypical microbial pathogens, with important morbidity. We hypothesize that patients with such anatomy are at risk for chronic pulmonary infection due to hypo-perfusion of the pulmonary parenchyma. This previously unreported clinical association should be considered in the differential diagnosis of patients with tetralogy of Fallot and pulmonary atresia who alter their respiratory state.

Long-term outcomes after the atrial switch for surgical correction of transposition: a meta-analysis comparing the Mustard and Senning procedures.

Most adults with regular transposition (the combinations of concordant atrioventricular and discordant ventriculo-arterial connections) have undergone either the Mustard or Senning procedure in childhood. It is unclear whether adverse events differ according to the surgery performed. With this in mind, we conducted a systematic review and meta-analysis to compare long-term outcomes. We searched systematically entries to MEDLINE and EMBASE databases from January 1966 through August 2003, supplementing the search by secondary sources. Comparative studies were required to include at least 10 patients in each cohort of Mustard or Senning procedure, and to report overall survival. Data were extracted by two independent reviewers. We used a component approach to assess quality. On the basis of assessment of heterogeneity, we then used a random-effects model for pooled analyses. In all, we included seven studies, incorporating 885 patients. We found a trend towards lower mortality for the 369 patients undergoing a Mustard procedure when compared to 474 submitted to the Senning operation, with a hazard ratio of 0.63 and 95% confidence intervals between 0.35 and 1.14 (p = 0.13). This trend increased with the size of the sample (p = 0.004). Obstruction in the systemic venous pathway was more common in those having the Mustard procedure, with a risk ratio of 3.5 and 95% confidence intervals from 1.8 to 7.0 (p < 0.001), with a trend towards greater obstruction of the pulmonary venous pathway in those undergoing the Senning procedure, 7.6% vs. 3.8% (p = 0.27). A trend towards fewer residual shunts was observed for those with Mustard baffles, 7.0% vs. 14.1% (p = 0.10). Sinus nodal dysfunction, however, was more common after the Mustard procedure. Data regarding atrial tachydysrhythmias was inconclusive. Systemic cardiac failure and functional capacity, was similar. We conclude that outcomes are not uniform among patients submitted to the Mustard and Senning procedures. Knowledge of such differences may facilitate stratification of risk and follow-up.

Transcatheter closure versus medical therapy of patent foramen ovale and presumed paradoxical thromboemboli: a systematic review.

BACKGROUND: The optimal strategy to prevent recurrent presumed paradoxical emboli in patients with patent foramen ovale is unknown. PURPOSE: To synthesize the current knowledge about and qualitatively assess the relative benefits of transcatheter closure versus medical therapy for patent foramen ovale. DATA SOURCES: English-language and foreign-language journals listed in the MEDLINE database from January 1985 to July 2003 were systematically searched. Secondary sources were also used. STUDY SELECTION: Secondary prevention studies of transcatheter closure or medical therapy for patent foramen ovale were required to include at least 10 patients followed for more than 1 year and to report recurrent neurologic events. DATA EXTRACTION: Data from published studies were manually extracted and summarized. DATA SYNTHESIS: Ten studies of transcatheter closure (1355 patients) and 6 studies of medical therapy (895 patients) for patent foramen ovale were included. Overall, the 1-year rate of recurrent neurologic thromboembolism with transcatheter intervention was 0% to 4.9%, and the incidence of major and minor complications was 1.5% and 7.9%, respectively. Medical management was associated with a 1-year recurrence rate of 3.8% to 12.0%. However, limitations resulting from uncontrolled data, nonstandardized definitions, vigilance of follow-up, and baseline imbalances preclude definitive conclusions about the superiority of a particular approach. General differences in study samples included older age, greater proportion of men, and higher prevalence of diabetes and smoking among medically treated patients. Patients undergoing treatment with a transcatheter device were more likely to have had multiple thromboembolic events at baseline. CONCLUSION: Transcatheter closure of patent foramen ovale may prevent a substantial proportion of cryptogenic strokes. Randomized clinical trials are needed.

Tissue velocity Doppler assessment of atrial and ventricular electromechanical coupling and atrioventricular time intervals in normal subjects.

Mechanical events and electromechanical coupling are analyzed simultaneously in the atria and ventricles using tissue velocity imaging. Normal values for these parameters are provided.