Correspondence

Eisenmenger's Syndrome in Pregnancy

N Engl J Med 1993; 329:887September 16, 1993

Article

To the Editor:

Eisenmenger's syndrome consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrial, ventricular, or aortopulmonary level1. 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 postpartum2. 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 five5. 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

5 References

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Citing Articles (8)

Citing Articles

1. 1

   Hong Wang, Weiyuan Zhang, Tao Liu. (2011) Experience of managing pregnant women with Eisenmenger's syndrome: Maternal and fetal outcome in 13 cases. Journal of Obstetrics and Gynaecology Research 37:1, 64-70
   CrossRef

2. 2

   Delia Maria Paternoster, Irene Pascoli, Matteo Parotto, Maddalena Cester, Kimta Nanhornguè, Elisa Merati, Silvia Arrigoni, Greta Garofalo, Maria Bertolino, Massimo Micaglio, Claudio Ripa. (2010) Pulmonary hypertension during pregnancy: management of two cases. Archives of Gynecology and Obstetrics 281:3, 431-434
   CrossRef

3. 3

   Fu-Nan Cho. (2009) Management of pregnant women with cardiac diseases at potential risk of thromboembolism — Experience and review. International Journal of Cardiology 136:2, 229-232
   CrossRef

4. 4

   Charles Fox, Philip L Kalarickal, Michael J Yarborough, John Y Jin. (2008) Perioperative management including new pharmacological vistas for patients with pulmonary hypertension for noncardiac surgery. Current Opinion in Anaesthesiology 21:4, 467-472
   CrossRef

5. 5

   Fu-Nan CHO, San-Nung CHEN. (2006) Use of extracorporeal membrane oxygenation to manage post-partum pulmonary haemorrhage associated with pulmonary hypertension and aberrant right pulmonary artery. The Australian and New Zealand Journal of Obstetrics and Gynaecology 46:6, 556-558
   CrossRef

6. 6

   Gautam Ramakrishna, Juraj Sprung, Barugur S. Ravi, Krishnaswamy Chandrasekaran, Michael D. McGoon. (2005) Impact of Pulmonary Hypertension on the Outcomes of Noncardiac Surgery. Journal of the American College of Cardiology 45:10, 1691-1699
   CrossRef

7. 7

   A GEI, G HANKINS. (2001) CARDIAC DISEASE AND PREGNANCY. Obstetrics and Gynecology Clinics of North America 28:3, 465-512
   CrossRef

8. 8

   Branko M Weiss, Lea Zemp, Burkhardt Seifert, Otto M Hess. (1998) Outcome of Pulmonary Vascular Disease in Pregnancy: A Systematic Overview From 1978 Through 1996. Journal of the American College of Cardiology 31:7, 1650-1657
   CrossRef