Of outcomes, one review of 14 women conceiving after a Fontan repair found that six spontaneously aborted all pregnancies, and eight others carried 14 pregnancies to viability (Cauldwell, 2016). Cardiac complications included arrhythmias and thromboembolism. Ten newborns delivered preterm, and eight neonates were small for gestational age. Similar complications attend a maternal systemic right ventricle, that is, one in which the right ventricle rather than the left pumps blood to the systemic circulation (Khan, 2015).
his describes secondary pulmonary hypertension that arises from any cardiac lesion. The syndrome develops when pulmonary vascular resistance exceeds systemic resistance and leads to concomitant right-to-Ieft shunting. The most common underlying defects are atrial or ventricular septal defects and persistent ductus arteriosus (Fig. 49-4). Patients are asymptomatic for years, but eventually pulmonary hypertension becomes severe enough to cause this shunting (Greutmann, 2015).
Pregnant women with Eisenmenger syndrome tolerate hypotension poorly, and death usually is caused by right ventricular failure with cardiogenic shock. In a review of 44 cases through 1978, maternal and perinatal mortality rates approximated 50 percent (Gleicher, 1979). In a later review of 73 pregnancies, Weiss and associates (1998) cited a 36-percent maternal death rate. Three of 26 deaths were antepartum, and the remainder of women died intrapartum or within a month of delivey. In a subsequent study of 13 gravidas, one mother died 17 days after delivery, and there were ive perinatal deaths (Wang, 2011).
Given such poor outcomes or both mother and etus, Eisenmenger syndrome is considered to be an absolute contraindication to pregnancy (Brickner, 2014; Lindley, 2015; Meng, 2017; Warnes, 2015). Management of those who do become pregnant has recently been detailed by Broberg (2016) and is discussed in the next section.
Normal resting mean pulmonary artery pressure is 12 to 16 mm Hg. Pulmonary vascular resistance in late pregnancy approximates 80 dyne/sec/cm-5, which is 34-percent less than the nonpregnant value of 120 dyne/sec/cm-5 (Clark, 1989). Pulmonay hypertension is deined in nonpregnant individuals as a resting mean pulmonary pressure >25 mm Hg.
he current clinical classification system, shown in Table 49-7, includes five groups of disorders that cause pulmonary hypertension (Galie, 2016). There are important prognostic and therapeutic distinctions between group 1 pulmonary arterial hypertension and the other groups. Group 1 indicates that a specific disease afects pulmonary arterioles. It includes idiopathic or primary pulmonary arterial hypertension as well as those cases secondary to a known cause such as connective tissue disease. For example, approximately one third of women with scleroderma and 10 percent with systemic lupus erythematosus
TABLE 49-7. Comprehensive Clinical Classification of Pulmonary Hypertension 1. Pulmonary arterial hypertension
Drug and toxin induced Associated with connective tissue disease, HIV infections, portal hypertension, congenital heart diseases, schistosomiasis
Drugs, toxins and radiation induced
Associated with connective tissue disease, HIV infection
I" Persistent pulmonary hypertension of the newborn 2. Pulmonary hypertension due to let heart disease 3. Pulmonary hypertension due to lung diseases and/or hypoxia
Other pulmonary diseases with mixed restrictive and obstructive pattern Sleep-disoriented breathing