Risk of Complications During Pregnancy in Women with Congenital Aortic Valve Stenosis

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Citation
US Cardiology, 2007;4(1):91-3

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The prevalence of heart disease in pregnant women is estimated to be between 0.1 and 4%; although the prevalence has not changed for decades, the relative contribution of different types of heart disease varies according to the study population and the study period. Nowadays, in developed countries the largest group of females with an underlying heart disease consists of women with congenital heart defects (70–80%), followed by patients suffering from valvular sequelae of rheumatic fever.1 Congenital heart defects occur in approximately 0.8% of newborns. Progress in medical, interventional, and surgical treatment has largely improved the outcome for these children, leading to an excellent survival into adult life.2–4 In particular, congenital aortic valve stenosis (AS) accounts for about 5% of all congenital heart defects. Despite a male predominance (the male–female ratio is between 3:1 and 5:1), AS is considered to be the most common cause of left ventricular outflow tract (LVOT) obstruction in young women. These women reach reproductive age and want to become pregnant. Unfortunately, underlying heart disease has become the major cause of non-obstetrical maternal death and accounts for 15% of all pregnancy-related mortality in Western countries.5,6 The level of risk incurred by pregnancy depends on functional status and the specific cardiac condition, both of which determine the ability of the cardiovascular system to adapt to the physiological changes of pregnancy.

Morphological, Epidemiological, and Hereditary Aspects of Congenital Aortic Stenosis

Congenital obstruction of the LVOT may involve the subvalvular, valvular, or supravalvular part of the outflow tract complex. Stenosis due to a congenital malformation of the aortic valve is the most common form of LVOT obstruction, found in more than 50% of cases. Abnormal development of the valve commissures results in unicuspid, bicuspid, tricuspid, or even quadricuspid valves. The most common abnormality is the bicuspid valve, which accounts for more than 95% of congenital AS. A bicuspid valve seems to be an isolated defect, but in 20% of patients associated defects can be found, including coarctation of the aorta (most common), patent ductus arteriosus, and ventricular septal defect (VSD). Moreover, a bicuspid valve is found in approximately 50% of patients with coarctation of the aorta. These two lesions are the most common cardiac anomalies found in patients with Turner syndrome. In about one-third of patients, the level of the obstruction is subvalvular.

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