Although not all cyanotic lesions are repairable, with satis factory surgical correction before pregnancy, maternal and fetal outcomes are much improved. In a review of 197 pregnancies in 99 women with surgically corrected Fallot tetralogy, preg nancy was usually well tolerated, and no mothers died. Still, almost 9 percent of pregnancies were complicated by adverse and heart failure (Balci, 2011; Kamiya, 2012). For women with a pulmonary valve replacement, pregnancy does not adversely afect graft function (Oosterhof, 2006). Prior to or after con ception, women with Fallot tetralogy are ofered genetic coun seling and evaluation for 22q 11 deletion syndrome (Lindley, 2015).
Some women with Ebstein anomay, characterized by a mal positioned and malformed tricuspid valve, may reach reproduc tive age. Right ventricular failure from volume overload and cyanosis are common during pregnancy. In the absence of cya nosis, heart failure, or signiicant arrhythmias, afected women usually tolerate pregnancy well (Brickner, 2014).
Transposition of the Great Vessels
Pregnancy following surgical correction of transposition has prominent risks. Canobbio (2006) and Drenthen (2005a), each with their colleagues, described outcomes of 119 pregnancies in 68 women-90 percent had a prior Mustard procedure and 10 percent a previous Senning procedure. During pregnancy, one fourth had arrhythmias, 12 percent developed heart failure, and one subsequently required cardiac transplantation. One woman died suddenly a month after delivery, and another died 4 years later. A third of the newborns were delivered preterm. In another report of 60 pregnancies in 34 women who had undergone transposition repair, approximately a fourth ended in miscarriage or abortion, and another fourth delivered preterm (Trigas, 2014). Deterioration in functional class occurred in seven women, and documented deterioration in systolic function occurred in four women. Finally, two women required resuscitation during delivery, and one experienced supraventricular tachycardia during labor. Of other defects, in women with previously repaired truncus arteriosus and doubleoutlet right ventricle, successful-although eventful-pregnancies have also been described (Drenthen, 2008; Hoendermis, 2008).
With hypoplastic lt heart syndrome, almost 70 percent of afected women are now expected to survive into adulthood and frequently become pregnant (Feinstein, 2012). Those who have undergone a Fontan repair are at particularly high risk for complications. In brief, this procedure involves diverting blood via a surgical anastomosis from the vena cava to the pulmonary artery without passing through the right ventricle. Blood Bows passively to the pulmonary vasculature. Thus, patients with a Fontan palliation are very preload dependent (Lindley, 2015).
lead to pulmonary
FIGURE 49-4 Eisenmenger syndrome due to a ventricular septal defect (VSD). A. Substantialolet-to-right shunting through the VSD leads to morphological changes in the smaller pulmonary arteries and arterioles. Specifically, medial hypertrophy, intimal cellular proliferations, and fibrosis lead to narrowing or closure of the vessel lumen. These vascular changes create pulmonary hypertension and a resultant reversal of the intracardiac shunt (8). With sustained pulmonary hypertension, extensive atherosclerosis and calcification often develop in the large pulmonary arteries. Although a VSD is shown here, Eisenmenger syndrome may also develop in association with a large atrial septal defect or patent ductus arteriosus.