Objectives: To assess parents' understanding of their child's congenital heart disease in various knowledge domains and to identify significant determinants of parental knowledge. Design: Cross sectional questionnaire survey Setting: Tertiary paediatric cardiac centre. Patients: 156 parents of children with relatively simple congenital heart defects were recruited from the outpatient clinic of a tertiary cardiac centre over a three month period. The questionnaire comprised 10 items of knowledge under three domains: nature of heart disease and its treatment; impact of heart disease on exercise capacity; and infective endocarditis and its prevention. The frequency distribution of the parents' knowledge in the different domains was determined. Univariate analyses and logistic regression were performed to identify significant determinants of knowledge in selected items. Results: While 59% of parents correctly named their child's congenital heart disease, only 28.8% correctly indicated the heart lesion(s) diagrammatically. However, more than 80% of parents were aware of the indications and aims of previous surgical and transcatheter interventions. About half of the parents were aware of possible aetiologies and of the hereditary nature and symptoms attributable to underlying heart disease. Disappointingly, of the 56 parents whose children were taking cardiac medications, only 25 (44.6%) and 4 (7.1%) knew correctly the functions and important side effects of the medications, respectively. With regard to exercise capacity, 59% of parents indicated its level appropriate for the heart lesion. While 26.9% of parents had heard of the term

The deletion (D) allele of the insertion/deletion (I/D) polymorphism of the angiotensin converting enzyme (ACE) gene is strongly associated with an increased level of circulating ACE. The ACE gene polymorphism may influence the production of angiotensin II (Ang II). It has been shown that Ang II modulates fibrinolysis, that is, Ang II increases plasminogen activator inhibitor-1 (PAI-1) mRNA and plasma PAI-1 levels in vitro and in vivo. Considered together, we tested the hypothesis that the deletion allele of the ACE gene might be associated with increased levels of PAI-1. We related the ACE genotype to PAI-1 antigen levels in 603 men and 221 women attending a routine health screening. As a whole, the plasma PAI-1 level was not strongly associated with ACE genotype. Since the PAI-1 level was significantly influenced by well-known risk factors for coronary artery disease (CAD), we further analyzed the data after excluding subjects with major cardiovascular risk factors. In low-risk male subjects, the DD genotype had significantly higher levels of plasma PAI-1 (DD: 20.3 +/- 2.2; DI: 13.9 +/- 1.1; II: 13.6 +/- 1.3 ng/mL, P = .010 by ANOVA). In low-risk female subjects, the DD genotype showed a tendency to a high level of plasma PAI-1 without statistical significance. When analysis was restricted to postmenopausal women (age > or = 55 or FSH > or = 35 ng/mL), the DD genotype showed a significantly higher level of PAI-1 than subjects with the DI and II genotypes (27.7 +/- 6.2 versus 15.6 +/- 1.8 ng/mL, P = .028). The DD polymorphism of the ACE gene is associated with high PAI-1 levels in male and possibly in postmenopausal female subjects who have lower conventional cardiovascular risk factors. These results suggest that the increased ACE activity caused by DD polymorphism may play an important role in elevating the level of plasma PAI-1. Our data support the notion that the genetic variation of ACE contributes to the balance of the fibrinolytic pathway.

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Background Adult with congenital heart disease represents a new category of specialized cardiovascular interest that requires the cooperation of a number of medical and surgical disciplines, and also requires the interactions among traditional departmental jurisdiction. Uninterrupted, long-term continuity care is essential if the concerns inherent in this new and increasing patient population are to be addressed. The purpose of this study was to analyze the clinical characteristics of congenital heart disease in adolescents and adults. Methods Between October 1994 and July 1996, retrospective follow-up records and registry chart of 229 consecutive patients with congenital heart disease for over 16 years in GUCH grown-up congenital heart clinic were reviewed by a physician and a nurse specialist. Results There were 126 female and 103 male GUCH patients with the mean age of 34 14.6 years old. Among the 229 patients, there were 179 natural survivors, those without cardiac repair, and 50 postoperative survivors. Congenital heart defects were 167 shunt legions, 17 obstructive and valvular legions, 14 tetralogy of Fallot, 15 complex congenital heart anomalies and 16 others. Among the 179 natural survivors 122 68 required heart surgery or continuous medical surveillance, and among the 50 surgically repaired survivors 37 74 required reoperation for residual heart defects, constant medical treatment or consultation from other medical divisions. The reasons for the hospital vistis were cardiac operation or cardiac diagnosis in 128 56 patients, symptomatic heart conditions in 43 19 , routine heart examinations since childhood in 31 14 and others in 27 11 . Also, the patient compliances were higher in the GUCH clinic than the traditional departmental jurisdiction p 0.001 . Conclusions To achieve continuing care for the patients with congenital heart disease in adolescents and adults, it is important to develope a specialized clinic addressing the specific needs of the congenital heart disease in adolescents and adults. Korean Circulation J

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