Congenial Heart Disease at Adult Age

__Abstract__ Congenital cardiac defects are by far the most common congenital anomalies. Of all live births around the world, approximately 1% is born with congenital heart disease.1 This number is even higher if patients with a bicuspid aortic valve are included.2 Accordingly, in the Netherlands every year around 1800 children are born with a congenital heart defect. Of these, about 50% cure spontaneously or do well without medical or surgical treatment. In the 1960’s, before the development of cardiac surgery with cardiopulmonary bypass, about 50% of the children with congenital heart disease requiring therapy died within the first year of live and less than 15% reached adulthood.3 Especially patients with transposition of the great arteries had a very poor survival: 90% of the patients died in the first month of life and less than 1% reached adult age. Of tetralogy of Fallot patients 10% survived until adult age

Congenital aortic stenosis in adults: rate of progression and predictors of clinical outcome: Reply

Dear Editor,We thank Dr. Ramaraj for his interest in our analysis on the progression of congenital aortic stenosis. Age should be regarded as an important risk factor for faster stenosis progression. The mechanism for faster progression is probably valve calcification occurring at an earlier age, promoted by the abnormal mechanical and shear stresses associated with a bicuspid aortic valve. Recent insights into the pathogenesis of calcification of the aortic valve suggest that this is an active, rather than a passive process, with features reminiscent of atherosclerosis. Inflammation, lipid infiltration, dystrophic calcification, ossification, and endothelial dysfunction have been observed in both diseases. [...

Pregnancy in women with congenital heart disease

Due to the enormous improvements in the treatment of children with congenital heart disease, an increasing number of patients reach adulthood nowadays. To achieve the aim of excellent care concerning pregnancy in this patient population, a pro-active discussion of pregnancy with all women inthis patient population is essential. Several risk stratifi cation models have been developed in order to identify high risk pregnancies and better inform patients about these risks. Counselling and dedicated care are needed to optimise both maternal and fetal outcomes. Better access to health caresystems contributes to further improvements in mortality and morbidity. Furthermore a well-developed, multidisciplinary plan for each individual patient concerning pregnancy, delivery and follow-up is essential

Pregnancy in congenital heart disease: Risk prediction and counselling

Risk prediction and counselling are the key to limit risks of complications during pregnancy in women with congenital heart disease. The WHO classification and clinical risk tools will guide the physician to the best available risk estimate, but an individualised approach and expert opinion remains paramount in counselling women with congenital heart disease with a pregnancy wish. In women with an estimated low-risk or intermediate-risk pregnancy, planned follow-up and a delivery plan made by a multidisciplinary team provides the best chance of an uncomplicated pregnancy. While the majority do well, there is a small group of women that need an explicit advice not to embark pregnancy, to prevent devastating situations