conducted during menses showed a small area of bron-chial mucosa thickening, particularly evident with thevirtual bronchoscopy and not visible during the firstexamination. Comparisons between the two CT scans ledto the correct diagnosis. On the basis of such findings,flexible fiberoptic bronchoscopy, performed on the firstday of menses, disclosed a tiny submucosal red spot in theleft upper bronchus with signs of recent bleeding.

Medical therapy has been recommended as the firstchoice in pulmonary endometriosis. It consists of thesuppression of endometrial tissue with progesterone (ie,pseudopregnancy) or danazol (ie, pseudomenopause).Danazol is a synthetic steroid with antiestrogenic and lightandrogenic effects that affects ovarian hormone synthe-sis.11 It has proved to be effective in curing or controllingsymptoms, even in patients who are nonresponsive toovulation suppression,12 but a variable recurrence rateafter the cessation of therapy has been reported.1,13 Fur-thermore, heavy side effects of the hormonal therapy oftenare observed, including climacteric symptoms, virilization,weight gain, and sterility.4 Surgery should be the preferredmethod if the patient wishes to become pregnant, if theside effects of hormonal therapy are intolerable, or in caseof recurrence when the drug therapy is discontinued.Pulmonary resection is indicated when a single point ofbleeding has been located definitively. For peripherallesions, thoracoscopic wedge resections have been suc-cessfully performed.4,14 In patients with centrally locatedbronchial endometriosis, subsegmentectomy, segmentec-tomy, or lobectomy are required.3,15–17 Treatment withoophorectomy has been reported in the literature,18 but itseems to be an extreme solution and should be avoided.

The precise endoscopic identification of the tracheo-bronchial lesions brings new therapeutic options. With thecombination of CT scanning and flexible fiberoptic bron-choscopy, the source of bleeding should be located inevery tracheobronchial endometriosis.

In our patient, we decided to proceed to laser treatmentbecause the lesion had been precisely located in a bron-chus, which is easily reachable by the bronchoscope. Wepreferred the ventilating rigid bronchoscopy mainly forour extensive experience in the use of this procedure.Nevertheless, a successful laser ablation of the lesion alsocould have been performed through the flexible fiberopticbronchoscope.

No previous endoscopic treatments of this conditionhave been reported in the literature. Endoscopic Nd-YAGlaser can eliminate mucosal and submucosal lesions with aminimally invasive procedure, without significant opera-tive risk. Such treatment could be the first line of therapyfor central airway endometriosis, provided that the sourceof bleeding has been conclusively located and the lesionscan be reached with the bronchoscope. Endoscopic abla-tion potentially can achieve good and probably long-termoutcomes without the adverse effects of pharmacologictherapy and surgical therapy.

References1 Bateman ED, Morrison SC. Catamenial hemoptysis from

endobronchial endometriosis: a case report and review of

previously reported cases. Respir Med 1990; 84:157–1612 Wang HC, Kuo PH, Kuo SH, et al. Catamenial hemoptysis

from tracheobronchial endometriosis: reappraisal of diagnos-tic value of bronchoscopy and bronchial brush cytology. Chest2000; 118:1205–1208

3 Terada Y, Chen F, Shoji T, et al. A case of endobronchialendometriosis treated by subsegmentectomy. Chest 1999;115:1475–1478

4 Inoue T, Kurokawa Y, Kaiwa Y, et al. Video-assisted thoraco-scopic 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: newobservations from an analysis of 110 cases. Am J Med 1996;100:164–170

7 Guidry GG, George RB. Diagnostic studies in catamenialhemoptysis. 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 Soc1990; 142:27–30

9 Volkart JR. CT findings in pulmonary endometriosis. J Com-put Assisted Tomogr 1995; 19:156–157

10 Karpel JP, Appel D, Merav A. Pulmonary endometriosis.Lung 1985; 163:151–159

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 JObstet Gynecol 1988; 159:733–734

14 Cassina PC, Hauser M, Kacl G, et al. Catamenial hemoptysis:diagnosis with MRI. Chest 1997; 111:1447–1450

15 Assor D. Endometriosis of the lung: report of a case. Am JClin Pathol 1972; 57:311–315

16 Kristianen K, Fjeld NB. Pulmonary endometriosis causinghemoptysis: report of a case treated with lobectomy. ScandJ Thorac Cardiovasc Surg 1993; 27:113–115

17 Weber F. Catamenial hemoptysis. Ann Thorac Surg 2001;72:1750–1751

18 Joseph J, Reed CE, Sahn SA. Thoracic endometriosis: recur-rence following hysterectomy with bilateral salpingo-oopho-rectomy and successful treatment with talc pleurodesis Chest1994; 106:1894–1896

Successful Management ofPregnancy in a Patient WithEisenmenger Syndrome WithEpoprostenol*

Chris Geohas, MD; and Vallerie V. McLaughlin, MD, FCCP

Pregnancy in the setting of pulmonary hypertensionand Eisenmenger physiology is associated with asubstantial maternal and fetal risk. Such patients areadvised against pregnancy. We report a case of awoman with an Eisenmenger atrial septal defectdiagnosed during the last trimester of pregnancy. Onpresentation, she was critically ill and there wasevidence of fetal distress. She was emergently

1170 Selected Reports

Downloaded From: http://journal.publications.chestnet.org/ on 10/31/2014

treated with IV epoprostenol, and her status im-proved. She underwent cesarean section and deliv-ered a male infant with Apgar scores of 8 and 9. Herdyspnea improved, and she was characterized asWorld Health Organization functional class II on asubsequent clinical visit. Although pregnancy shouldbe discouraged in women with Eisenmenger syn-drome, we have demonstrated that IV epoprostenolsuccessfully treated a woman with Eisenmenger syn-drome diagnosed in the third trimester.

(CHEST 2003; 124:1170–1173)

Key words: Eisenmenger syndrome; epoprostenol; pregnancy;pulmonary hypertension

E isenmenger syndrome consists of a congenital com-munication between the systemic and pulmonary cir-

culation, with resultant pulmonary arterial hypertensionand reversal of flow through the defect. In a review1 ofoutcome of pregnancy in patients with pulmonary vasculardisease from 1978 through 1996, the maternal mortalityrate in the Eisenmenger syndrome was 36%, which didnot represent an improvement when compared to theprevious review published in 1979. Over the past decade,the treatment of primary pulmonary hypertension hasbeen revolutionized with the use of IV epoprostenol.2Noncontrolled data suggest that epoprostenol is also use-ful in patients with congenital heart disease.3 We report acase of Eisenmenger syndrome diagnosed during the thirdtrimester of pregnancy and successfully managed with IVepoprostenol. To our knowledge, this is the first report ofthe use of epoprostenol to manage pregnancy in a patientwith Eisenmenger physiology.

Case ReportA 21 year-old gravida 3 para 2 woman was transferred to our

institution in August of 2001 at 3437

weeks’ gestation for themanagement of dyspnea and preterm contractions. The patienthad two uncomplicated vaginal deliveries in 1997 and 1999 andhad done well during this pregnancy until several weeks prior tohospital admission when she complained of increasing shortnessof breath and bilateral lower-extremity edema. She deniedsymptoms of chest pain, light-headedness, syncope, and palpita-tions. Her pregnancy had been uncomplicated until her recenthospital admission. She had gained 70 lb during her pregnancy.Her medical history was unremarkable outside of the twopregnancies. Her only medications included prenatal vitaminsand an albuterol inhaler. She denied the use of alcohol, tobacco,or IV drugs.

On initial physical examination, she was in respiratory distresswith an oxygen saturation of 80% on a 100% nonrebreather mask.She would easily desaturate to the low 70s with even minimal

exertion. Her heart rate was 107 beats/min, respiratory rate was24 breaths/min, and BP was 134/73 mm Hg. Her jugular venouspressure was elevated. Her carotid upstrokes were reduced. Herlungs were clear to auscultation. She had a palpable rightventricular heave. On cardiac auscultation, she was tachycardicwith a normal S1, a loud pulmonic component to the second heartsound, 2/6 murmur of tricuspid regurgitation, and a right-sidedS3. Her abdomen was gravid. She had trace bilateral lower-extremity edema.

Her ECG demonstrated right-axis deviation and right ventric-ular hypertrophy. Her chest radiograph demonstrated an en-larged cardiopericardial silhouette. Her chest CT did not dem-onstrate any evidence of interstitial lung disease or pulmonaryembolus. Her echocardiogram demonstrated marked right atrialand right ventricular enlargement, and severe pulmonary hyper-tension with an estimated pulmonary artery pressure of 120 mm Hg(Fig 1). With color-flow Doppler echocardiography and aninjection of agitated saline solution, she had evidence of right-to-left shunting through an atrial septal defect at rest (Fig 2).

The patient was managed in conjunction with the high-riskobstetric service who performed a biophysical profile (assess-ment of reactive nonstress test, fetal breathing movement,fetal body movement, fetal tone, and amniotic fluid volume),which suggested fetal distress. Because of her severe hypox-emia, respiratory distress, and newly diagnosed pulmonaryhypertension, the patient was started on IV epoprostenol. Noother therapies were instituted or altered. Over the ensuing24 h, the epoprostenol was titrated up to 9 ng/kg/min with animprovement in her oxygenation to a saturation of 90% andtachycardia. She was then taken to the operating room whereshe underwent a cesarean section and delivered a male infantwith Apgar scores of 8 and 9. Postoperatively, she receivedanticoagulation with enoxaparin, 80 mg bid subcutaneously.One week postpartum, a right-heart catheterization was per-formed while the patient was receiving IV epoprostenol (Table1). The atrial septal defect was easily crossed with a multipurposecatheter. Based on a measured oxygen uptake of 134 mL/min, herpulmonic blood flow calculated to 3.3 L/min and systemic bloodflow to 3.5 L/min. Her pulmonary vascular resistance was 16.6Wood units, and systemic vascular resistance was 28.2 Woodunits.

Her hospital course was complicated by an infected hematomaat the site of her surgical incision, which required IV antibiotics.The patient was discharged after a 2-week hospitalization receiv-ing warfarin anticoagulation and epoprostenol. At a subsequentclinic visit, she reported a marked improvement in her dyspneaand was World Health Organization functional class II receiving12 ng/kg/min of epoprostenol.

DiscussionEisenmenger syndrome is one of the few cardiac con-

ditions for which pregnancy is considered absolutely con-traindicated. The poor outcome during pregnancy is aresult of impaired hemodynamics and exercise capacitysuperimposed on the gestational cardiovascular demandsto which there is insufficient adaptation of the right heartand poorly compliant pulmonary vasculature. Normalphysiologic changes of pregnancy can worsen right-to-leftflow across a congenital defect and result in worseninghypoxemia.

In general, therapy of Eisenmenger syndrome is sup-portive.4 Patients should avoid intravascular volume de-pletion, heavy exercise, and high altitude. Phlebotomywith isovolemic replacement should be performed inpatients with moderate or severe symptoms of hypervis-

*From the Division of Cardiology, Rush-Presbyterian-St. Luke’sMedical Center, Chicago, IL.Manuscript received July 8, 2002; revision accepted March 4,2003.Reproduction of this article is prohibited without written permis-sion from the American College of Chest Physicians (e-mail:permissions@chestnet.org).Correspondence to: Vallerie V. McLaughlin, MD, FCCP, Rush-Presbyterian-St. Luke’s Medical Center, 1725 W. Harrison St,Suite 020, Chicago, IL 60612; e-mail: vallerie_mclaughlin@rush.edu

www.chestjournal.org CHEST / 124 / 3 / SEPTEMBER, 2003 1171

Downloaded From: http://journal.publications.chestnet.org/ on 10/31/2014

cosity, but may result in iron deficiency anemia. Morerecently, IV epoprostenol has been successful in patientswith congenital heart disease.3

To our knowledge, this is the first case report oftreatment of a pregnant woman with Eisenmenger syn-

drome with IV epoprostenol. This patient received herdiagnosis late in pregnancy, beyond the time at which atherapeutic termination could have been performed. Shewas managed with a multidisciplinary approach, and hercare included cardiologists, high-risk obstetricians, and

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

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

1172 Selected Reports

Downloaded From: http://journal.publications.chestnet.org/ on 10/31/2014

anesthesiologists. The relatively short-term effects ofepoprostenol likely included vasodilatation (both pulmo-nary and systemic) and increased cardiac output resultingin improved oxygenation and placental blood flow. Gen-eral anesthesia and cesarean section were successfullyperformed in this patient, in part related to the multidis-ciplinary approach. She received aggressive anticoagula-

tion, which is important as deep venous thrombosis andpulmonary embolism are important causes of postpartummortality in patients with pulmonary arterial hypertension.Although pregnancy should be discouraged in women withEisenmenger syndrome, we have demonstrated that IVepoprostenol successfully treated a woman with Eisen-menger syndrome diagnosed in the third trimester.

References1 Weiss BM, Zemp L, Seifert B, et al. Outcome of pulmonary

vascular disease in pregnancy: a systematic overview from1978 through 1996. J Am Coll Cardiol 1998; 31:1650–1657

2 Barst R, Rubin L, Long W, et al. A comparison of continuousintravenous epoprostenol (prostacyclin) with conventionaltherapy for primary pulmonary hypertension. N Engl J Med1996; 334:296–301

3 Rosenzweig E, Kerstein D, Barst R. Long-term prostacyclinfor pulmonary hypertension with associated congenital heartdefects. Circulation 1999; 99:1858–1865

4 Brickner ME, Hillis LD, Lange RA. Congenital heart diseasein adults. N Engl J Med 2000; 342:334–342

Table 1—Right-Heart Catheterization

Variables Pressure, mm Hg O2 Saturation, %

Right atrium 6Pulmonary artery 98/40,

—61 75.0

Left atrium 6Systemic arterial 122/51,

—74 90.4

Pulmonary vein 98.0Inferior vena cava 73.4Superior vena cava 63.0

Overline indicates mean pressure.

www.chestjournal.org CHEST / 124 / 3 / SEPTEMBER, 2003 1173

Downloaded From: http://journal.publications.chestnet.org/ on 10/31/2014