Over the past decades, survival of patients with CHD improved significantly, making it a life-cycle disease. Hence, there is a need for a workforce that can take up the care for afflicted individuals in the different phases of the life spectrum. Each life phase is associated with specific challenges. Topics that should receive more attention in clinical care or in CHD research are parenting styles of parents of children, transfer and transition of adolescents, cumulative burden of injury in the brain in adults, and geriatric care for older persons with CHD. Nurses, along with other healthcare professionals, will play a pivotal role in building up expertise in these areas and taking up these challenges.

Klippel–Feil syndrome is a skeletal disorder characterised by low hairline and a short neck due to abnormal fusion of two or more cervical vertebrae. Although congenital heart and lung defects are infrequent, some abnormalities such as cor triatriatum, coarctation of the aorta, total anomalous pulmonary venous connection, or lung agenesis have been reported. The challenge of recognising Klippel–Feil syndrome lies in the fact that there is an association of this syndrome with other significant conditions such as skeletal, genitourinary, neurological, ear, and some cardiac defects. We report a Klippel–Feil syndrome type III 14-year-old patient with a levo-looped transposition of the great arteries. In addition, the patient had agenesis of the left upper-lung lobe.

Although ventricular septal defect is the most common form of congenital heart disease, its epidemiology is not completely understood. The Baltimore-Washington Infant Study, a case-control study of congenital cardiovascular malformations, enrolled 4,390 case infants during the study period 1981–1989. Ventricular septal defect, the most common malformation, was present in 1,465 (33%) of affected infants. The purpose of this report was to describe the epidemiological characteristics of membranous and muscular ventricular septal defects, the most common types of ventricular septal defects. Membranous ventricular septal defects (n=895) and muscular ventricular septal defects (n=429) accounted for 90% of all ventricular defects. During the study, prevalence increased from 0.9 to 2.1 /1000 live births. Associated non-cardiac defects, such as chromosomal defects, genetic syndromes, and defects of other organs were more common among infants with ventricular septal defect than among control infants, and more common among infants with membranous ventricular defect (20.8%) than among those with muscular defects (7.5%). Infants with ventricular septal defects and non-cardiac defects had increased mortality in comparison to infants with isolated ventricular septal defects or controls. Epidemiological information derived from population-based studies provide valuable data for counseling families and may allow for examination of etiologic factors.

Objective: To discuss the need for continuing cardiac surveillance in adults with hemodynamically insignificant congenital cardiac disease. Methods: In 82 patients with mild congenital cardiac malformations, aged from 17 to 32 years, we investigated the subjective health status, the health-related quality of life, any difficulties encountered in daily life, the regularity of follow-up, current diagnosis, and antibiotic prophylaxis. Results: The subjective health status, and the health-related quality of life, did not differ from those of the general population. Nevertheless, patients experienced unnecessary difficulties with choice of sport, obtaining insurance cover, and education. After clinical re-evaluation, diagnosis and antibiotic regimes had to be changed in 9 patients (11%). Conclusions: Patients with a mild congenital cardiac malformation consider themselves to be in good health. So as to fine tune the diagnosis, and update the information supplied to the patients, we suggest that at least a cardiological assessment be carried out at the age of 16 to 18 years. In this way, patients might be protected from unnecessary difficulties, such as restrictions for sport or the charging of unjustifiably high rates for insurance.

One of twins was bom with prune belly syndrome and congenital heart defect following exposure to Bendectin and Tigan. Red cell antigens and HLA typing were compatible with monozygosity. The possible associations of the prune belly syndrome to monozygotic twinning or to teratogenic agents is considered in light of this patient and review of the literature.