Bicuspid aortic valves due to accelerated degeneration predispose to aortic aortopathy
manifesting as aortic valvular stenosis (36), ascending aorta and aortic root dilatation out of proportion to hemodynamic factors, and true SOV aneurysms (37-39).
Many theories have been postulated about the cause of aortopathy
This associated aortopathy
has been thought to be caused by increased activity of intrinsic tissue structures such as matrix metalloproteinases and protein kinases within the intima and adventitia of the vessel, during embryogenesis, which predisposes these patients to aneurysms.
Intrinsic histological abnormalities of aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aortopathy
is prevalent in relatives of bicuspid aortic valve patients.
Marfan syndrome and aortopathy
, Eisenmenger's syndrome (an advanced form of pulmonary artery hypertension), peripartum cardiomyopathy with residual left ventricular dysfunction, severe aortic stenosis, and mechanical valves are all associated with high maternal and fetal death rates, and pregnancy in women with these conditions should be avoided and possibly electively terminated when it does occur, urged the experts.
With the exception of patients with Eisenmenger syndrome, severe surgical noncorrected cyanotic disease, severe pulmonary artery hypertension, pulmonary vascular obstructive disease and Marfan syndrome with aortopathy
, maternal death during pregnancy in women with heart disease is rare.