conducted during menses showed a small area of bronchial mucosa thickening, particularly evident with the

virtual bronchoscopy and not visible during the first

examination. Comparisons between the two CT scans led
to the correct diagnosis. On the basis of such findings,
flexible fiberoptic bronchoscopy, performed on the first
day of menses, disclosed a tiny submucosal red spot in the
left upper bronchus with signs of recent bleeding.
Medical therapy has been recommended as the first
choice in pulmonary endometriosis. It consists of the
suppression of endometrial tissue with progesterone (ie,
pseudopregnancy) or danazol (ie, pseudomenopause).
Danazol is a synthetic steroid with antiestrogenic and light
androgenic effects that affects ovarian hormone synthesis.11 It has proved to be effective in curing or controlling
symptoms, even in patients who are nonresponsive to
ovulation suppression,12 but a variable recurrence rate
after the cessation of therapy has been reported.1,13 Furthermore, heavy side effects of the hormonal therapy often
are observed, including climacteric symptoms, virilization,
weight gain, and sterility.4 Surgery should be the preferred
method if the patient wishes to become pregnant, if the
side effects of hormonal therapy are intolerable, or in case
of recurrence when the drug therapy is discontinued.
Pulmonary resection is indicated when a single point of
bleeding has been located definitively. For peripheral
lesions, thoracoscopic wedge resections have been successfully performed.4,14 In patients with centrally located
bronchial endometriosis, subsegmentectomy, segmentectomy, or lobectomy are required.3,1517 Treatment with
oophorectomy has been reported in the literature,18 but it
seems to be an extreme solution and should be avoided.
The precise endoscopic identification of the tracheobronchial lesions brings new therapeutic options. With the
combination of CT scanning and flexible fiberoptic bronchoscopy, the source of bleeding should be located in
every tracheobronchial endometriosis.
In our patient, we decided to proceed to laser treatment
because the lesion had been precisely located in a bronchus, which is easily reachable by the bronchoscope. We
preferred the ventilating rigid bronchoscopy mainly for
our extensive experience in the use of this procedure.
Nevertheless, a successful laser ablation of the lesion also
could have been performed through the flexible fiberoptic
bronchoscope.
No previous endoscopic treatments of this condition
have been reported in the literature. Endoscopic Nd-YAG
laser can eliminate mucosal and submucosal lesions with a
minimally invasive procedure, without significant operative risk. Such treatment could be the first line of therapy
for central airway endometriosis, provided that the source
of bleeding has been conclusively located and the lesions
can be reached with the bronchoscope. Endoscopic ablation potentially can achieve good and probably long-term
outcomes without the adverse effects of pharmacologic
therapy and surgical therapy.

References
1 Bateman ED, Morrison SC. Catamenial hemoptysis from
endobronchial endometriosis: a case report and review of
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previously reported cases. Respir Med 1990; 84:157161

2 Wang HC, Kuo PH, Kuo SH, et al. Catamenial hemoptysis
from tracheobronchial endometriosis: reappraisal of diagnostic value of bronchoscopy and bronchial brush cytology. Chest
2000; 118:12051208
3 Terada Y, Chen F, Shoji T, et al. A case of endobronchial
endometriosis treated by subsegmentectomy. Chest 1999;
115:14751478
4 Inoue T, Kurokawa Y, Kaiwa Y, et al. Video-assisted thoracoscopic surgery for catamenial hemoptysis. Chest 2001; 120:
655 658
5 Wood DJ, Krishna K, Stocks P, et al. Catamenial hemoptysis:
a rare cause. Thorax 1993; 48:1048 1049
6 Joseph J, Sahn SA. Thoracic endometriosis syndrome: new
observations from an analysis of 110 cases. Am J Med 1996;
100:164 170
7 Guidry GG, George RB. Diagnostic studies in catamenial
hemoptysis. Chest 1990; 98:260 261
8 Guidry GG, George RB, Payne DK. Catamenial hemoptysis:
a case report and review of the literature. J La State Med Soc
1990; 142:2730
9 Volkart JR. CT findings in pulmonary endometriosis. J Comput Assisted Tomogr 1995; 19:156 157
10 Karpel JP, Appel D, Merav A. Pulmonary endometriosis.
Lung 1985; 163:151159
11 Madanes AE, Farber M. Danazol. Ann Intern Med 1982;
96:625 630
12 Elliot DL, Barker AF, Dixon LM. Catamenial hemoptysis:
new methods of diagnosis and therapy. Chest 1985; 87:687
688
13 Lawrence HC. Pulmonary endometriosis in pregnancy. Am J
Obstet Gynecol 1988; 159:733734
14 Cassina PC, Hauser M, Kacl G, et al. Catamenial hemoptysis:
diagnosis with MRI. Chest 1997; 111:14471450
15 Assor D. Endometriosis of the lung: report of a case. Am J
Clin Pathol 1972; 57:311315
16 Kristianen K, Fjeld NB. Pulmonary endometriosis causing
hemoptysis: report of a case treated with lobectomy. Scand
J Thorac Cardiovasc Surg 1993; 27:113115
17 Weber F. Catamenial hemoptysis. Ann Thorac Surg 2001;
72:1750 1751
18 Joseph J, Reed CE, Sahn SA. Thoracic endometriosis: recurrence following hysterectomy with bilateral salpingo-oophorectomy and successful treatment with talc pleurodesis Chest
1994; 106:1894 1896

Successful Management of
Pregnancy in a Patient With
Eisenmenger Syndrome With
Epoprostenol*
Chris Geohas, MD; and Vallerie V. McLaughlin, MD, FCCP

Pregnancy in the setting of pulmonary hypertension

and Eisenmenger physiology is associated with a
substantial maternal and fetal risk. Such patients are
advised against pregnancy. We report a case of a
woman with an Eisenmenger atrial septal defect
diagnosed during the last trimester of pregnancy. On
presentation, she was critically ill and there was
evidence of fetal distress. She was emergently
Selected Reports

treated with IV epoprostenol, and her status improved. She underwent cesarean section and delivered a male infant with Apgar scores of 8 and 9. Her
dyspnea improved, and she was characterized as
World Health Organization functional class II on a
subsequent clinical visit. Although pregnancy should
be discouraged in women with Eisenmenger syndrome, we have demonstrated that IV epoprostenol
successfully treated a woman with Eisenmenger syndrome diagnosed in the third trimester.
(CHEST 2003; 124:1170 1173)
Key words: Eisenmenger syndrome; epoprostenol; pregnancy;
pulmonary hypertension

isenmenger syndrome consists of a congenital comE munication

between the systemic and pulmonary cir-

culation, with resultant pulmonary arterial hypertension

and reversal of flow through the defect. In a review1 of
outcome of pregnancy in patients with pulmonary vascular
disease from 1978 through 1996, the maternal mortality
rate in the Eisenmenger syndrome was 36%, which did
not represent an improvement when compared to the
previous review published in 1979. Over the past decade,
the treatment of primary pulmonary hypertension has
been revolutionized with the use of IV epoprostenol.2
Noncontrolled data suggest that epoprostenol is also useful in patients with congenital heart disease.3 We report a
case of Eisenmenger syndrome diagnosed during the third
trimester of pregnancy and successfully managed with IV
epoprostenol. To our knowledge, this is the first report of
the use of epoprostenol to manage pregnancy in a patient
with Eisenmenger physiology.

Case Report
A 21 year-old gravida 3 para 2 woman was transferred to our
3
institution in August of 2001 at 34 7 weeks gestation for the
management of dyspnea and preterm contractions. The patient
had two uncomplicated vaginal deliveries in 1997 and 1999 and
had done well during this pregnancy until several weeks prior to
hospital admission when she complained of increasing shortness
of breath and bilateral lower-extremity edema. She denied
symptoms of chest pain, light-headedness, syncope, and palpitations. Her pregnancy had been uncomplicated until her recent
hospital admission. She had gained 70 lb during her pregnancy.
Her medical history was unremarkable outside of the two
pregnancies. Her only medications included prenatal vitamins
and an albuterol inhaler. She denied the use of alcohol, tobacco,
or IV drugs.
On initial physical examination, she was in respiratory distress
with an oxygen saturation of 80% on a 100% nonrebreather mask.
She would easily desaturate to the low 70s with even minimal
*From the Division of Cardiology, Rush-Presbyterian-St. Lukes
Medical Center, Chicago, IL.
Manuscript received July 8, 2002; revision accepted March 4,
2003.
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail:
permissions@chestnet.org).
Correspondence to: Vallerie V. McLaughlin, MD, FCCP, RushPresbyterian-St. Lukes Medical Center, 1725 W. Harrison St,
Suite 020, Chicago, IL 60612; e-mail: vallerie_mclaughlin@
rush.edu
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exertion. Her heart rate was 107 beats/min, respiratory rate was
24 breaths/min, and BP was 134/73 mm Hg. Her jugular venous
pressure was elevated. Her carotid upstrokes were reduced. Her
lungs were clear to auscultation. She had a palpable right
ventricular heave. On cardiac auscultation, she was tachycardic
with a normal S1, a loud pulmonic component to the second heart
sound, 2/6 murmur of tricuspid regurgitation, and a right-sided
S3. Her abdomen was gravid. She had trace bilateral lowerextremity edema.
Her ECG demonstrated right-axis deviation and right ventricular hypertrophy. Her chest radiograph demonstrated an enlarged cardiopericardial silhouette. Her chest CT did not demonstrate any evidence of interstitial lung disease or pulmonary
embolus. Her echocardiogram demonstrated marked right atrial
and right ventricular enlargement, and severe pulmonary hypertension with an estimated pulmonary artery pressure of 120 mm Hg
(Fig 1). With color-flow Doppler echocardiography and an
injection of agitated saline solution, she had evidence of rightto-left shunting through an atrial septal defect at rest (Fig 2).
The patient was managed in conjunction with the high-risk
obstetric service who performed a biophysical profile (assessment of reactive nonstress test, fetal breathing movement,
fetal body movement, fetal tone, and amniotic fluid volume),
which suggested fetal distress. Because of her severe hypoxemia, respiratory distress, and newly diagnosed pulmonary
hypertension, the patient was started on IV epoprostenol. No
other therapies were instituted or altered. Over the ensuing
24 h, the epoprostenol was titrated up to 9 ng/kg/min with an
improvement in her oxygenation to a saturation of 90% and
tachycardia. She was then taken to the operating room where
she underwent a cesarean section and delivered a male infant
with Apgar scores of 8 and 9. Postoperatively, she received
anticoagulation with enoxaparin, 80 mg bid subcutaneously.
One week postpartum, a right-heart catheterization was performed while the patient was receiving IV epoprostenol (Table
1). The atrial septal defect was easily crossed with a multipurpose
catheter. Based on a measured oxygen uptake of 134 mL/min, her
pulmonic blood flow calculated to 3.3 L/min and systemic blood
flow to 3.5 L/min. Her pulmonary vascular resistance was 16.6
Wood units, and systemic vascular resistance was 28.2 Wood
units.
Her hospital course was complicated by an infected hematoma
at the site of her surgical incision, which required IV antibiotics.
The patient was discharged after a 2-week hospitalization receiving warfarin anticoagulation and epoprostenol. At a subsequent
clinic visit, she reported a marked improvement in her dyspnea
and was World Health Organization functional class II receiving
12 ng/kg/min of epoprostenol.

Discussion
Eisenmenger syndrome is one of the few cardiac conditions for which pregnancy is considered absolutely contraindicated. The poor outcome during pregnancy is a
result of impaired hemodynamics and exercise capacity
superimposed on the gestational cardiovascular demands
to which there is insufficient adaptation of the right heart
and poorly compliant pulmonary vasculature. Normal
physiologic changes of pregnancy can worsen right-to-left
flow across a congenital defect and result in worsening
hypoxemia.
In general, therapy of Eisenmenger syndrome is supportive.4 Patients should avoid intravascular volume depletion, heavy exercise, and high altitude. Phlebotomy
with isovolemic replacement should be performed in
patients with moderate or severe symptoms of hypervisCHEST / 124 / 3 / SEPTEMBER, 2003

1171

Figure 1. Two-dimensional, apical, four-chamber view demonstrating right atrial and ventricular
enlargement. RA right atrium; RV right ventricle; LA left atrium; LV left ventricle.

cosity, but may result in iron deficiency anemia. More

recently, IV epoprostenol has been successful in patients
with congenital heart disease.3
To our knowledge, this is the first case report of
treatment of a pregnant woman with Eisenmenger syn-

drome with IV epoprostenol. This patient received her

diagnosis late in pregnancy, beyond the time at which a
therapeutic termination could have been performed. She
was managed with a multidisciplinary approach, and her
care included cardiologists, high-risk obstetricians, and

Figure 2. Color Doppler echocardiography demonstrating flow across the atrial septal defect. See
Figure 1 legend for expansion of abbreviations.
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Selected Reports

Table 1Right-Heart Catheterization

Variables

Pressure, mm Hg

Right atrium
Pulmonary artery
Left atrium
Systemic arterial
Pulmonary vein
Inferior vena cava
Superior vena cava

98/40, 61
6

122/51, 74

O2 Saturation, %
75.0
90.4
98.0
73.4
63.0

Overline indicates mean pressure.

anesthesiologists. The relatively short-term effects of

epoprostenol likely included vasodilatation (both pulmonary and systemic) and increased cardiac output resulting
in improved oxygenation and placental blood flow. General anesthesia and cesarean section were successfully
performed in this patient, in part related to the multidisciplinary approach. She received aggressive anticoagula-

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tion, which is important as deep venous thrombosis and

pulmonary embolism are important causes of postpartum
mortality in patients with pulmonary arterial hypertension.
Although pregnancy should be discouraged in women with
Eisenmenger syndrome, we have demonstrated that IV
epoprostenol successfully treated a woman with Eisenmenger syndrome diagnosed in the third trimester.

References
1 Weiss BM, Zemp L, Seifert B, et al. Outcome of pulmonary
vascular disease in pregnancy: a systematic overview from
1978 through 1996. J Am Coll Cardiol 1998; 31:1650 1657
2 Barst R, Rubin L, Long W, et al. A comparison of continuous
intravenous epoprostenol (prostacyclin) with conventional
therapy for primary pulmonary hypertension. N Engl J Med
1996; 334:296 301
3 Rosenzweig E, Kerstein D, Barst R. Long-term prostacyclin
for pulmonary hypertension with associated congenital heart
defects. Circulation 1999; 99:1858 1865
4 Brickner ME, Hillis LD, Lange RA. Congenital heart disease
in adults. N Engl J Med 2000; 342:334 342

CHEST / 124 / 3 / SEPTEMBER, 2003

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