Medial abnormalities in ascending aorta, paracoarctation aorta, truncus arteriosus, and pulmonary trunk were prevalent in patients with a variety of forms of CHD encompassing a wide age range. Aortic abnormalities may predispose to dilatation, aneurysm, and rupture. Pulmonary trunk abnormalities may predispose to dilatation and aneurysm; hypertensive aneurysms may rupture. Pivotal questions are whether these abnormalities are inherent or acquired, whether CHD plays a causal or facilitating role, and whether genetic determinants are operative.

The vast majority of patients can go safely through pregnancy and delivery as long as adequate pre-pregnancy evaluation and specialized high-quality care during pregnancy and delivery are available. Pregnancy outcomes were markedly worse in patients with CMP and in developing countries.

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Objective: To provide pure cohorts of paediatric and adult patients with congenital heart disease (CHD) and infective endocarditis (IE) for making future guidelines. Design: Japanese nationwide survey. Setting: 66 Japanese institutions. Patients: 170 children, mean (SD) age 7.4 (5.7) years (range 14 days to 17 years), and 69 adults, age 32.5 (14.1) years (range 18-69) who developed IE between 1997 and 2001 (one in 240 admissions with CHD). Main outcome measures: Clinical presentation of IE. Results: 119 patients including 88 with cyanotic CHD had previous cardiac surgery. Procedures preceding IE were dental (12%) followed by cardiovascular surgery (8%). Sites of infection were left sided in 46% and right sided in 51%. Vegetation with diameter of 11 mm was documented in 151 (63%). Frequent complications were embolic events (stroke 11%, other emboli 20%) and cardiac failure (23%). The most common microorganisms were streptococci (50%) and staphylococci (37%) with methicillin resistant Staphylococcus aureus in 7.5%. Empirical treatments were penicillins (alone or with other antibiotics 57%) followed by cephems (22%) and vancomycin (11%). Surgery during active IE was common (26%), with vegetation (45%) and heart failure (29%) as the most frequent indications. Mortality was 8.8%: 8.0% among patients who received medical treatment alone and 11.1% among those with active IE who underwent surgery. The causes of death (n = 21) were surgery (7), infection (7), cardiac failure (6), and renal failure (1). Conclusions: Because of a recent increase in the incidence of IE and high mortality and complication rate, it is mandatory to establish well formulated recommendations for management of IE in paediatric and adult patients with CHD based on a large cohort. Results of this nationwide multicentre database should be helpful in establishing guidelines. P ublished guidelines used worldwide for infective endocarditis (IE) 1-6 address mainly adults with acquired cardiac disorders. Cardiologists have not had access to formulated guidelines for IE in paediatric and adult patients with congenital heart disease (CHD) based on data from a pure large cohort. Increasing numbers of patients with CHD have now reached adulthood 7 and CHD constitutes the major substrate for IE in both children and adults. [8][9][10][11][12][13][14] Because relatively few cases of CHD and IE are seen in any one institution, authors have tended to report the issue of IE by combining their clinical experience over several decades.9-12 15 16 Accordingly, there is a dearth of practical and current data of IE from a large cohort of paediatric and adult patients with CHD. 8 This study aimed at clarifying recent information on the clinical presentation of IE in a nationwide survey in Japan for the formulation of the guidelines in paediatric and adult patients with CHD.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

Whether asymptomatic hyperuricemia in the absence of comorbidities increases the risk for cardiometabolic disorders and chronic kidney disease remains controversial. This study was conducted to clarify the association between asymptomatic hyperuricemia and cardiometabolic conditions. Subjects consisted of Japanese adults between 30 and 85 years of age were enrolled in the study at Center for Preventive Medicine, St. Luke's International Hospital, Tokyo, and available at enrollment (2004) and at 5-year follow-up (2009). Subjects were excluded if they were overweight or obese, hypertensive, diabetic, dyslipidemic, had a history of gout or hyperuricemia on medications, or if they had chronic kidney disease as estimated glomerular filtration rate <60 ml/min/1.73m2. Linear and logistic regression analyses were used to examine the relationship between hyperuricemia and development of hypertension, diabetes mellitus, dyslipidemia, chronic kidney disease, and overweight/obesity (unadjusted and adjusted for age, sex, smoking, drinking habits, baseline estimated glomerular filtration rate and body mass index). 5,899 subjects without comorbidities (mean age of 47 ± 10 years, 1,864 men) were followed for 5 years. Hyperuricemia (defined as >7 mg/dL in men and ≥6 mg/dL in women) was associated with increased cumulative incidence rates of hypertension (14.9% vs 6.1%, p<0.001), dyslipidemia (23.1% vs 15.5%, p<0.001), chronic kidney disease (19.0% vs 10.7%, p<0.001), and overweight/obesity (8.9% vs 3.0%, p<0.001), while diabetes mellitus (1.7% vs 0.9%, p=0.087) showed a trend but did not reach statistical significance. In conclusion, asymptomatic hyperuricemia carries a significant risk for developing cardiometabolic conditions in Japanese individual without comorbidities.

Background-Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its dynamic nature in adults with repaired TOF. Methods and Results-Of 216 patients with TOF repair who underwent echocardiography in 1997, we identified 32 patients (mean age, 36Ϯ8.0 years) with AoRo dilatation, defined as ratio of observed to expected AoRo size by standard nomogram Ͼ1.5 (group A), and 54 TOF controls, matched for age with AoRo ratio Ͻ1.5 (group B), who underwent at least 1 previous echocardiogram in the preceding 10 years. Mean indexed AoRo size (cm/m