Correspondence

Eisenmenger's Syndrome and Pulmonary-Artery Dissection

N Engl J Med 2007; 357:718-719August 16, 2007

Article

To the Editor:

With regard to the surgical management of pulmonary-artery dissection in a patient with Eisenmenger's syndrome, described by Westaby et al. (May 17 issue),1 Eisenmenger's syndrome is defined as the reversal of a long-standing left-to-right shunt, resulting in a right-to-left shunt. This change is a consequence of progressively increasing pressure in the right side of the heart. The authors report concomitant closure of the patent ductus arteriosus. Such closure in this setting remains contentious if not dangerous without evidence of pulmonary arterial reactivity and an accurate estimation of pulmonary vascular resistance. If the patent ductus arteriosus is closed, there is a risk of suprasystemic pulmonary-artery pressure, low cardiac output, and right ventricular failure.2 Generally, if advanced pulmonary-artery hypertension is present, corrective surgery of shunts should be considered only for patients with evidence of pulmonary arterial reactivity, the presence of left-to-right shunting of at least 1.5 to 1.0, or both.3 This patient's history raises the question of whether he had advanced pulmonary-artery hypertension without reversal of shunt flow, rather than Eisenmenger's syndrome.

Jeffrey H. Shuhaiber, M.D.
Loyola University Stritch School of Medicine, Chicago, IL 60607

3 References

1. 1

   Westaby S, Evans BJ, Ormerod O. Pulmonary-artery dissection in patients with Eisenmenger's syndrome. N Engl J Med 2007;356:2110-2112
   Full Text | Web of Science | Medline

2. 2

   Schneider DJ, Moore JW. Patent ductus arteriosus. Circulation 2006;114:1873-1882
   CrossRef | Web of Science | Medline

3. 3

   Therrien J, Warnes C, Daliento L, et al. Canadian Cardiovascular Society Consensus Conference 2001 update: recommendations for the management of adults with congenital heart disease part III. Can J Cardiol 2001;17:1135-1158
   Medline

Author/Editor Response

With an unrepaired double-outlet right ventricle at 26 years of age, our patient had cyanosis and clubbing. Three United Kingdom cardiovascular centers had confirmed Eisenmenger's syndrome, although he remained active and had an acceptable quality of life. After acute dissection of the pulmonary artery, early death from rupture of the false aneurysm was a certainty.

Closure of the patent ductus arteriosus was necessary because the dissection involved the insertion of the ductus. The intracardiac shunt was the more important one; we did not close the ventricular septal defect. The patient survived the operation and was discharged from the hospital 10 days later. We therefore disagree with Shuhaiber's suggestion that closure of the patent ductus arteriosus in this patient was “contentious if not dangerous.”

Stephen Westaby, Ph.D.
John Radcliffe Hospital, Oxford OX3 9DU, United Kingdom