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Fallot's Tetralogy and Pregnancy 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. Long-Term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl heart disease include major or minor residua (abnormalities J Med 1993;329:593–9. left behind) or sequelae (abnormalities caused by surgery but 5. Warnes CA, Liberthson R, Danielson GK, et al. Task force 1: the not technically considered complications), which includes changing profile of congenital heart disease in adult life. Care of the adult with congenital heart disease. Presented at the 32nd Bethesda electrophysiologic issues, anatomic or functional ventricular Conference, Bethesda, Maryland, October 2–3, 2000. J Am Coll or valvular abnormalities, and/or prosthetic materials such Cardiol 2001;37:1170–5. 6. Perloff JK, Child JS, editors. Congenital Heart Disease in Adults. as patches, conduits, and valves (4,6,21,22). Philadelphia, PA: W. B. Saunders, 1998:157. Left ventricular dysfunction may be related to chronic 7. Perloff JK, Koos B. Pregnancy and congenital heart disease: the volume overload as a result of previous long-standing large mother and fetus. In: Perloff JK, Child JS, editors. Congenital Heart Disease in Adults. Philadelphia, PA: W. B. Saunders, 1998:44–64. aortopulmonary shunts, to inadequate intraoperative myo- 8. Perloff JK. Congenital heart disease and pregnancy. Clin Cardiol cardial preservation, to significant aortic or mitral regurgi- 1994;17:579–87. tation, or even to acquired diseases (e.g., systemic arterial 9. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003;24:761–81. hypertension). Right heart dysfunction may relate to the 10. Presbitero P, Somerville J, Stone S, Aruta E, Spiegelhalter D, Rabajoli size of RV outflow incisions and/or a patch or incisional F. Pregnancy in cyanotic congenital heart disease. Outcome of mother aneurysm, residual or recurrent pulmonary stenosis or re- and fetus. Circulation 1994;89:2673–6. 11. Siu SC, Sermer M, Colman JM, et al. Prospective multicenter study of gurgitation, a large residual or recurrent ventricular septal pregnancy outcomes in women with heart disease. Circulation 2001; defect shunt, or pulmonary hypertension. 104:515–21. Prognostication suggests a knowledgeable look at the 12. Siu SC, Colman JM, Sorensen S, et al. Adverse neonatal and cardiac outcomes are more common in pregnant women with cardiac disease. status of a disease and prediction of outcome. The report by Circulation 2002;105:2179–84. Veldtman et al. (18) is reassuring in that women with 13. Foster E, Graham TP Jr., Driscoll DJ, et al. Task force 2: special health care needs of adults with congenital heart disease. Care of the well-repaired TOF with no major residua or sequelae appear adult with congenital heart disease. Presented at the 32nd Bethesda at an overall low risk of morbidity from pregnancy. It bears Conference, Bethesda, Maryland, October 2 to 3, 2000. J Am Coll adding that even if there are no significant anatomic or Cardiol 2001;37:1176–83.
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