View metadata, citation and similar papers at core.ac.uk brought to you by CORE Journal of the American College of Cardiology providedVol. by Elsevier 44, No. - 1,Publisher 2004 Connector © 2004 by the American College of Cardiology Foundation ISSN 0735-1097/04/$30.00 Published by Elsevier Inc. doi:10.1016/j.jacc.2004.04.009 EDITORIAL COMMENT of such patients, as appears in this issue of the Journal (18), should now come from an institution with a long history of Fallot’s Tetralogy and Pregnancy excellent congenital cardiac surgery and with a program Prognostication and Prophesy* devoted to the care of adult survivors with congenital heart disease. At the time of this study from the Mayo Clinic, John S. Child, MD, FACC they had in their database 147 adult women (Ն18 years old) Los Angeles, California with TOF, of which 17 had died (not related to pregnancy), 21 were lost to follow-up, and 2 were institutionalized for The odyssey of a blue baby from cyanotic infant to acyanotic developmental delay. Of the remaining 107 invited to adult is a fascinating one. The pioneering work of Dr. John participate, 72 responded to requests for follow-up infor- H. Gibbon, Jr., in developing cardiopulmonary bypass mation. Of these 72, 43 had 112 pregnancies; 82 of these technology was a springboard to the repair of complex 112 pregnancies (73%) were successful. congenital cardiovascular malformations (1). Over 50 years Of the 43 women, 8 were unrepaired at the time of their of cardiovascular surgery has resulted in one of the most 20 successful pregnancies; 5 of the 8 were cyanotic at the impressive rehabilitative events in history (2). The approx- time of 12 pregnancies. In 20 (of the 43) patients (47%), imate incidence of tetralogy of Fallot (TOF) of about 400 TOF was initially palliated by some form of aortopulmonary per million live births marks it as the most common shunt. Ultimately, all patients had repair with ventricular complex or cyanotic defect (3). Corrective surgery for TOF septal defect closure and right ventricular (RV) outflow tract now permits over 85% of such children to survive into repair. Among the 43 patients, 56% had patch reconstruc- adulthood (4,5). Approximately 50% of patients with TOF tion of the RV outflow tract, of which 40% were trans- are women, and it was one of the first disorders to be annular, a pertinent precursor for future significant pulmo- surgically palliated or repaired; as such, “repaired” tetralogy nary regurgitation. Among the 43 TOF patients with 112 is one of the most frequently encountered conditions in pregnant women (6,7). pregnancies, 16 patients had 30 miscarriages (27% rate), and there was 1 stillbirth at term (18). Low infant birth weight See page 174 was related to the maternal state of women who had not undergone reparative surgery or to morphologic pulmonary artery abnormalities. Of the seven infants who were small In the U.S., there is a preponderance of pregnant women for gestational age, five (71%) were born to women with with heart disease who are now found to have some form of untreated TOF. Of the 30 pregnancy losses (in 16 women), congenital cardiovascular malformation, whether repaired, 3 occurred in 2 women with unrepaired TOF. corrected, palliated, or untreated (7). The circulatory and The recurrence rate of congenital defects has historically respiratory physiology in the normal gravid state are well been difficult to determine with precision. A review of 6,640 known (7–9). These hemodynamic alterations are com- consecutive pregnancies evaluated by detailed fetal echocar- pounded in women with heart disease, at times with severe diography reported it is at least 2.9% in maternal and 2.2% consequences. Pregnancy in cyanotic congenital heart de- in paternal congenital heart disease, greater in the case of fects (even with Eisenmenger syndrome excluded) carries a high incidence of miscarriage, premature birth, and low outflow tract defects (such as TOF) (7,19). Other studies birth weights, the frequency of which increases with the provide estimates of incidence in live births to a parent with degree of maternal cyanosis, maternal complications, and congenital heart disease ranging from 4.9% to 14% (10,20). need for anticoagulation (7,10–13). The potential pre- These figures contrast with a spontaneous incidence rate dictors of maternal morbidity include: functional class, estimated at median of 7 per 1,000 live births (3).Inthe ventricular dysfunction, significant arrhythmias, cyanosis, report by Veldtman et al. (18), five of the live infants (6%) outflow tract obstruction, pulmonary hypertension, and had congenital abnormalities, none with TOF (one with need for anticoagulants (e.g., prosthetic valves) (8,11,12,14– muscular ventricular septal defect; one with mitral prolapse; 17). one with hypoplastic left heart syndrome; one with pyloric In the modern era, systematic data on the outcomes of stenosis; and one with cleft lip/palate, strabismus, and pregnancy in women with TOF, both those who underwent clubbed feet). One mother incidentally had a documented surgery and those who did not, are few and far between. 22q11 deletion syndrome, but this chromosomal abnormal- Therefore, it is fitting that a careful report of a large number ity was not systematically evaluated in this study. Maternal cardiovascular complications were noted in six ϳ *Editorials published in the Journal of the American College of Cardiology reflect the ( 14%) of the pregnant women: supraventricular tachycar- views of the authors and do not necessarily represent the views of JACC or the dia in two, heart failure in two, pulmonary embolism in one American College of Cardiology. (with pre-existing pulmonary hypertension), and progres- From the Ahmanson-UCLA Adult Congenital Heart Disease Center, UCLA Division of Cardiology, Department of Medicine, David Geffen School of Medicine sive RV dilation in one (with pre-existing severe pulmonary at UCLA, Los Angeles, California. regurgitation ) (18). Note that in one of the women with 182 Child JACC Vol. 44, No. 1, 2004 Editorial Comment July 7, 2004:181–3 heart failure, it appeared to be “peri-partum” cardiomyop- REFERENCES athy rather than related to her intrinsic TOF. 1. Cohn LH. Fifty years of open-heart surgery. Circulation 2003;107: The mere relief of cyanosis, although a step forward for 2168–70. the mother and fetus, does not guarantee that there may not 2. Perloff JK. Historical perspective. In: Perloff JK, Child JS, editors. be serious issues to face in the pregnant woman with TOF Congenital Heart Disease in Adults. Philadelphia, PA: W. B. Saun- ders, 1998:3–8. after long-term follow-up of the postoperative milieu. Cures 3. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am in congenital heart surgery are few and far between. The Coll Cardiol 2002;39:1890–900. challenges encountered by adults with repaired congenital 4. Murphy JG, Gersh BJ, Mair DD, et al. 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