Correspondence

Pulmonary-Artery Dissection in Patients with Eisenmenger's Syndrome

N Engl J Med 2007; 356:2110-2112May 17, 2007

Article

To the Editor:

Adults with congenital heart disease who have pulmonary hypertension may have dissection of the pulmonary artery. Increasingly recognized as a lethal complication, pulmonary-artery dissection has usually been recorded at autopsy.1 Survivors often receive conservative care, because Eisenmenger's syndrome is deemed inoperable. Heart–lung transplantation has been performed in patients in the chronic phase of the disease.2 However, we suggest that the early, high mortality rate associated with Eisenmenger's syndrome warrants urgent conventional repair of the pulmonary artery.

A 26-year-old man with an unrepaired double-outlet right ventricle presented at our center with chest pain and breathlessness. A ventricular septal defect, patent ductus arteriosus, and Eisenmenger's syndrome had been diagnosed when he was 12 years old. With medical management, his heart condition remained in New York Heart Association class II, and he had full-time employment. Magnetic resonance imaging showed dissection of the pulmonary artery with hemopericardium (Figure 1Figure 1Magnetic Resonance Images of the Patient's Heart.). He also had severe pulmonary-valve regurgitation and profound oxygen desaturation (arterial oxygen saturation, <60%). Consistent with past experience, surgery had not been offered at another congenital heart disease center.

On reviewing the patient's case, we believed that the vigorous myocardial contractility, univentricular heart hemodynamic characteristics, and tolerance of pulmonary regurgitation indicated a potential for survival. We performed a cardiopulmonary bypass and found the origin of the dissection immediately above the valve annulus and a false aneurysm with very thin walls. We closed the patent ductus arteriosus and replaced the pulmonary root with a bioprosthetic valved conduit. A circulatory-assist device was kept on standby, but our use of inhaled nitric oxide to discontinue cardiopulmonary bypass made circulatory support unnecessary. The patient had remarkably stable hemodynamics, and he was extubated after 36 hours. Aortic and pulmonary arterial pressures were equal. When the systemic blood pressure reached 180/120 mm Hg, the arterial oxygen saturation rose to 90%. The patient was discharged after 10 days.

Although the early operative results justified our decision to operate, the longer-term prognosis must also be considered. Patients with Eisenmenger's syndrome can survive into their fourth or fifth decade with an acceptable quality of life.3 Heart–lung transplantation remains an eventual option for our patient, but it is impractical as urgent primary treatment for pulmonary dissection. Furthermore, our patient's long-term survival is likely to exceed the mean survival for patients who undergo heart–lung transplantation. The limited evidence suggests that if unrepaired, aortic dissection and pulmonary dissection result in a similarly grim prognosis. We therefore advocate urgent replacement of the pulmonary artery or pulmonary root in patients presenting with dissection. If the pathophysiological characteristics of Eisenmenger's syndrome bring the patient into the operating room, modern myocardial protection should ensure survival.

Stephen Westaby, Ph.D.
Betsy J. Evans, M.R.C.S.
Oliver Ormerod, D.M.
John Radcliffe Hospital, Oxford OX3 9DU, United Kingdom
swestaby@ahf.org.uk

3 References

1. 1

   Khattar RS, Fox DJ, Alty JE, Arora A. Pulmonary artery dissection: an emerging cardiovascular complication in surviving patients with chronic pulmonary hypertension. Heart 2005;91:142-145
   CrossRef | Web of Science | Medline

2. 2

   Tonder N, Kober L, Hassager C. Pulmonary artery dissection in a patient with Eisenmenger syndrome treated with heart and lung transplantation. Eur J Echocardiogr 2004;5:228-230
   CrossRef | Medline

3. 3

   Berman EB, Barst RJ. Eisenmenger's syndrome: current management. Prog Cardiovasc Dis 2002;45:129-138
   CrossRef | Web of Science | Medline

Citing Articles (5)

Citing Articles

1. 1

   Asumi Matsumoto, Tatsuya Kawasaki, Mayumi Takeoka, Michiyo Yamano, Chieko Sakai, Kuniyasu Harimoto, Shigeyuki Miki, Tadaaki Kamitani. (2011) Silent pulmonary artery dissection in a centenarian. Journal of Cardiology Cases
   CrossRef

2. 2

   Li-Qing Peng, Jian-Qun Yu, Zhi-Gang Chu, Hong-Mei Yuan. (2010) A Rare Case of Right and Left Pulmonary Artery Dissections on 64-Slice Multidetector Computed Tomography. Journal of Thoracic Imaging1
   CrossRef

3. 3

   Amikar Sehdev, Abhijeet Dhoble. (2010) Pulmonary artery dissection (PAD): A very unusual cause of chest pain. Journal of Hospital Medicine 5:5, 313-316
   CrossRef

4. 4

   Vera Demarchi Aiello, Maria Angélica Binotto, Lea Maria Demarchi, Antonio Augusto Lopes, Miguel Barbero Marcial. (2009) Causes of death and cardiovascular complications in adolescents and adults with congenitally malformed hearts: an autopsy study of 102 cases. Cardiology in the Young 19:05, 511
   CrossRef

5. 5

   (2007) Eisenmenger's Syndrome and Pulmonary-Artery Dissection. New England Journal of Medicine 357:7, 718-719
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