The New England Journal of Medicine Owned, published, and (C) copyrighted, 1993, by the MASSACHUSETTS MEDICAL SOCIETY ---------------------------------------------- Volume 329(12) 16 Sep 1993 p 887 ---------------------------------------------- Eisenmenger's Syndrome In Pregnancy. [Correspondence] Kahn, Mark L. ---------------------------------------------- Outline REFERENCES ---------------------------------------------- To the Editor: Eisenmenger's syndrome consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrial, ventricular, or aortopulmonary level [1]. Pregnancy should ideally be avoided in a woman with Eisenmenger's syndrome, since it carries a high (approximately 50 percent) risk of sudden death, frequently occurring a few days postpartum [2]. Because pulmonary thromboembolism may be a cause of sudden death in this setting, [3,4] anticoagulation in the peripartum period has been suggested as a method to lower this risk, [3] but it may instead contribute to a fatal outcome. A 32-year-old woman was transferred to our hospital. At 38 weeks' gestation, she presented with a 2-month history of dyspnea on exertion, lower-extremity edema, and orthopnea. Previously, the patient had completed three successful pregnancies complicated only by postpartum uterine hemorrhage. She had had a ``benign'' heart murmur since childhood. An echocardiogram taken before her transfer revealed a dilated right atrium and right ventricle and a normal left ventricle with normal function. Color flow Doppler ultrasonography and a bubble study (to look for intracardiac shunting) showed a large secundum atrial septal defect. On arrival at our hospital the patient had a pulse of 82 and an oxygen saturation of 91 percent with oxygen delivered by nasal cannula. The pulmonary arterial pressure was 125/53 mm Hg, and the systemic blood pressure, obtained simultaneously, was 140/80 mm Hg. Labor was induced, and a healthy boy was delivered vaginally. After delivery, the patient was treated with heparin to prevent thromboembolic complications. The next day, vaginal bleeding developed. The hematocrit dropped 15 points, and there was tachycardia and a transient decrease in systemic blood pressure. Despite the discontinuation of heparin and aggressive transfusion, progressive hypoxemia developed, with pulmonary arterial pressures that exceeded systemic pressures. The patient died on the second postpartum day. This case report illustrates the potential dangers of prophylactic anticoagulation in the peripartum period in patients with Eisenmenger's syndrome. Despite pathological evidence that pulmonary thromboemboli are associated with maternal death, [3,4] the benefit of prophylactic anticoagulation has not been established. In a review of seven patients with this syndrome who received prophylactic anticoagulation, Pitts et al\implicated secondary hemorrhage as the cause of death in five [5]. In each patient, the drop in systemic blood pressure and progressive hypoxemia caused fatal right-to-left shunting. This patient was at great risk even without receiving anticoagulation, and she should have been counseled against pregnancy. Given the unfortunate presentation of a woman with Eisenmenger's syndrome late in pregnancy, however, there is no indication for anticoagulation without clinical evidence of thromboembolic phenomena. Mark L. Kahn, M.D. University of California, San Francisco San Francisco, CA 94143-0124 REFERENCES 1. Wood P. Pulmonary hypertension. Br Med Bull 1952;8:348-53. 2. Jones AM, Howitt G. Eisenmenger syndrome in pregnancy. BMJ 1965; 1:1627-31. 3. Neilson G, Galea EG, Blunt A. Eisenmenger's syndrome and pregnancy. Med J Aust 1971;1:431-4. [Medline Link] 4. Gleicher N, Midwall J, Hochberger D, Jaffin H. Eisenmenger's syndrome and pregnancy. Obstet Gynecol Surv 1979;34:721-41. [Medline Link] 5. Pitts JA, Crosby WM, Basta LL. Eisenmenger's syndrome in pregnancy: does heparin prophylaxis improve the maternal mortality rate? Am Heart J 1977;93:321-6. [Medline Link] [BIOSIS Previews Link] Accession Number: 00006024-199309160-00027 ----------------------------------------------