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    Cardiovascular morbidity and mortality in pregnancy


    Moderate defects 

    Moderate forms of CHD include coarctation of the aorta and tetralogy of Fallot. Coarctation of the aorta is often diagnosed and treated in childhood, but the diagnosis may also be missed. Mothers with unrepaired coarctation of the aorta are at increased risk of aortic rupture and dissection during pregnancy, pregnancy-induced hypertension, preeclampsia and eclampsia, as well as intrauterine growth retardation and prematurity.10 Even with repair, mothers are at increased risk for hypertension, pregnancy-induced hypertension, preeclampsia, eclampsia, heart failure, and arrhythmias as well as a small risk of aortic dissection.11

    Tetralogy of Fallot is the most common form of cyanotic CHD and is usually diagnosed and repaired in childhood. Mothers with repaired tetralogy of Fallot may be left with residual pulmonary regurgitation or right ventricular (RV) outflow tract obstruction, leaving either a volume or pressure overload on the RV. Either of these situations can result in maternal complications such as arrhythmias or right heart failure symptoms.12,13 Mothers with severe pulmonary regurgitation may develop worsening RV enlargement during pregnancy that does not resolve after delivery, thus increasing the risk of arrhythmias and/or heart failure. 


    Complex defects

    Some forms of CHD result in the RV serving as the “systemic ventricle,” supplying the systemic circulation. This is most commonly seen in mothers with a history of D-transposition of the great arteries with a prior “atrial switch repair” (Mustard or Senning operation) or in mothers with L-transposition of the great arteries (also known as congenitally corrected transposition). The RV is not anatomically designed for this role and, thus, may not tolerate the additional workload of pregnancy. These patients are at increased risk for arrhythmias, heart failure and a decline in RV function during pregnancy.14 Mothers with D-transposition of the great arteries who have undergone an arterial switch procedure, where the aorta is realigned to arise from the left ventricle, have significantly fewer pregnancy complications.15

    Patients with CHD may have cyanosis due to unrepaired CHD or to complications associated with their surgical repairs. Some patients with cyanotic CHD may have had a “palliative” surgical procedure to improve pulmonary blood flow or improve cyanosis, but which leaves behind significant anatomic abnormalities. Maternal cyanosis results in an increased risk of both thrombosis and bleeding and places mothers at risk for stroke, bleeding complications, and paradoxical (right to left) embolism resulting in stroke or brain abscess. Maternal cyanosis significantly increases fetal risk with a poor likelihood of fetal survival when maternal oxygen saturation < 85%.16

    Even patients with very complex forms of CHD  with only a single ventricle can now routinely survive to adulthood and reach childbearing age. The Fontan operation refers to a surgical procedure to resolve cyanosis by directing systemic venous return to the pulmonary arteries without an intervening pump, with the single ventricle supplying the power for both systemic and pulmonary blood flow. This procedure is applied to a variety of underlying defects; thus, the specific type of congenital heart defect varies from patient to patient. The Fontan operation is a palliative procedure which does not “correct” the underlying congenital heart defect, but does allow for improved quality of life and often prolonged survival into adulthood. Mothers who have undergone the Fontan operation are at a significantly increased risk for prematurity, miscarriage, low fetal birth weight as well as maternal risks of arrhythmias, heart failure, and stroke.17,18


    Valvular heart disease/native valve disease

    Valvular heart disease is another common cause of maternal cardiovascular disease complicating pregnancy and is the leading form of CVD seen in underdeveloped countries. Left-sided valvular lesions (aortic and mitral valve disease) are the most common. Both aortic and mitral regurgitation are usually well-

    tolerated in pregnancy, unless ventricular dysfunction is present. Mothers with moderate to severe obstructive valve lesions have more difficulty tolerating the volume load and increased workload during pregnancy, and these patients may develop symptoms of heart failure, chest pain, or arrhythmias (and rarely, syncope) during pregnancy.5,6 Both aortic and mitral stenosis can result in an inability to adequately increase cardiac output; thus, exertional symptoms are common. The severity of exertional symptoms is usually a good marker of how well the patient will be able to tolerate labor and delivery. Pulmonary hypertension may be seen in patients with advanced left-sided valvular disease (particularly with mitral stenosis) and these patients are at particular risk for hemodynamic compromise during labor and delivery and require careful management.19

    NEXT: Prosthetic valve disease

    Beth Brickner, MD
    Dr. Brickner is co-director of the Adult Congenital Heart Disease Program at UT Southwestern Medical Center, Dallas, Texas.


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