Ann Thorac Surg 2008;85:1097-1099. doi:10.1016/j.athoracsur.2007.09.007
© 2008 The Society of Thoracic Surgeons
Case Reports
Bilateral Lung Transplant With Pulmonary Thromboendarterectomy for Eisenmenger’s Syndrome
Seth D. Force, MDa,*,
Brian Kogon, MDa,
Andres Pelaez, MDb,
David C. Neujahr, MDb,
Allan M. Ramirez, MDb,
Daniel L. Miller, MDa,
E. Clinton Lawrence, MDb
a Division of Cardiothoracic Surgery, Emory University Hospital, Atlanta, Georgia
b Division of Pulmonary Medicine, Emory University Hospital, Atlanta, Georgia
Accepted for publication September 6, 2007.
* Address correspondence to Dr Force, The Emory Clinic, 1365 Clifton Rd, NE, Atlanta, GA 30324 (Email: seth_force{at}emoryhealthcare.org).
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Abstract
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Patients with secondary pulmonary hypertension frequently present for evaluation for lung transplantation. In some of these patients, Eisenmenger’s syndrome has developed from chronic left to right intracardiac shunts. A smaller group of these patients will also have associated pulmonary artery aneurysms. There is a paucity of literature discussing this topic, however, and currents reports have suggested the need to replace the abnormal pulmonary artery. This paper discusses a patient in whom Eisenmenger’s syndrome developed from an atrial septal defect, and resultant pulmonary artery aneurysms and mural thrombi, who underwent successful bilateral lung transplantation with thromboendarterectomy and atrial septal defect closure.
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Introduction
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Patients with secondary pulmonary hypertension frequently present for evaluation for lung transplantation. In some of these patients, Eisenmenger’s syndrome has developed from chronic left to right intracardiac shunts with associated right ventricular dysfunction. Although it is tempting to list these patients for a combined heart-lung transplant, most are served well with lung transplantation and correction of the cardiac defect [1]. A smaller group of these patients will also have associated pulmonary artery aneurysms. However, there is a paucity of literature discussing this topic, and current reports have suggested the need to replace the abnormal pulmonary artery. This paper discusses a patient with Eisenmenger’s syndrome from an atrial septal defect (ASD) and resultant pulmonary artery aneurysms and mural thrombi who underwent successful ASD closure, thromboendarterectomy, and bilateral lung transplantation without pulmonary artery reconstruction.
A 43-year-old woman presented to our institution for a lung transplant evaluation 13 years after being diagnosed an ASD and pulmonary hypertension. Echocardiogram revealed a severely dilated right ventricle with significantly reduced function, a mean pulmonary artery pressure of 80 mm Hg, a large secundum and inferior sinus venosus ASD, and a left ventricular ejection fraction of 65%. Right-side heart catheterization confirmed the elevated pulmonary artery pressure and estimated the pulmonary vascular resistance to be 13.5 Woods units. Chest radiograph (Fig 1A) revealed normal lung fields and enlarged pulmonary arteries bilaterally, and chest computed tomography scan (Fig 1B) showed marked dilatation of the entire pulmonary artery system and diffuse mural thickening consistent with of lower extremity or pelvic blood clots. The patient was evaluated for a pulmonary thromboendarterectomy as primary therapy; however, it was thought that her thrombi were secondary to the low flow state caused by her pulmonary hypertension and that she would be served better by transplantation. A pair of suitable donor lungs became available, and the patient was brought to the operating room, intubated, and underwent a bilateral thoracosternotomy incision. She was then placed on cardiopulmonary bypass, the heart was arrested, and the ASD was repaired using native pericardium. After this, the aortic cross clamp was removed, a pulmonary artery vent was placed, and the recipient right pneumonectomy was performed. Next, the distal right main pulmonary artery was transected and revealed mural thrombi extending to the main pulmonary artery. A pulmonary thromboendarterectomy was then performed, beginning at the transected pulmonary artery orifice. To correct the extreme size mismatch between the donor and recipient pulmonary arteries, the right main pulmonary artery was plicated by suturing together the lateral walls at the 12 o’clock position. Finally, the donor right lung was implanted in a standard fashion. The left recipient pneumonectomy and lung transplant were performed in the same fashion, including the need for pulmonary artery thromboendarterectomy and plication. The patient was then weaned from cardiopulmonary bypass and taken to the intensive care unit in stable condition. The ischemic time for both lungs was 6 hours.
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Fig 1. (A) Preoperative chest radiograph revealing enlarged pulmonary arteries. (B) Preoperative chest computed tomography scan revealing enlarged pulmonary arteries with mural thrombi.
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The final pathology revealed that 8 cm of chronic, fibrinous thrombus was removed from each pulmonary artery. The segmental pulmonary artery branches contained organized, recanalized thombus, and the arterial walls were thickened and atherosclerotic. The pulmonary parenchyma showed microscopic changes of "arterio/arteriolosclerosis and plexiform lesions," consistent with a Heath-Edwards score of five (Fig 2).
The patient did well after surgery and was extubated on the third postoperative day and discharged from the hospital, without supplemental oxygen, on the 20th postoperative day. Postoperative chest radiograph showed clear lung fields and normal pulmonary vasculature. A chest CT, performed 7 months later, showed absence of the previously identified mural thrombi and normal caliber distal branch pulmonary arteries (Fig 3). Follow-up echocardiogram revealed absence an ASD, normal left ventricular function, moderately reduced right ventricular function and an estimated pulmonary artery systolic pressure of 30 mm Hg.
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Fig 3. (A) Postoperative chest radiograph revealing normal pulmonary arteries. (B) Postoperative chest computed tomography scan revealing absence of mural thrombi and more normal distal branch pulmonary arteries.
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Comment
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The overall incidence of pulmonary artery aneurysms is unknown, and most recent articles still cite data from the 1947 paper by Deterling and Clagett [2] that reported 8 cases in 100,000 autopsies. Although there may be myriad associated pathologic conditions, most patients with pulmonary artery aneurysms have congenital heart disease and pulmonary hypertension. Some of these patients will have complications from the aneurysm, such as rupture and dissection, and therefore elective repair is recommended [3]. However, the necessity of repairing pulmonary artery aneurysms in patients without pulmonary hypertension has been debated, and several authors have reported on the lack of complications seen in these patients [4, 5]. Lung transplantation with concomitant pulmonary artery reconstruction has been reported in the setting of end-stage lung disease and a pulmonary artery aneurysm [6, 7]. However, as lung transplantation restores normal pulmonary artery pressures, there is some question about the need to repair or remove the abnormal artery.
Our patient had pulmonary artery aneurysms in the setting of an ASD and Eisenmenger’s syndrome with associated mural thrombi. We chose to repair the ASD, remove the arterial thrombi, and perform a lung transplant, which effectively removed the intracardiac shunt and restored normal pulmonary arterial pressure. This technique yielded excellent intraoperative and postoperative results. The patient is more than a year out from her surgery and doing well. This report suggests that in the lung transplant setting, just as in the nontransplant setting, pulmonary artery aneurysms with normal pulmonary artery pressures may be well tolerated, and complicated attempts to repair the arteries may not be necessary.
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Acknowledgments
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Supported, in part, by funds from the McKelvey Lung Transplant Center.
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References
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- Choong CK, Sweet SC, Guthrie TJ, et al. Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year experience J Thorac Cardiovasc Surg 2005;129:661-669.[Abstract/Free Full Text]
- Deterling RA, Clagett OT. Aneurysm of the pulmonary artery: review of the literature and report of a case Am Heart J 1947;34:71-98.
- Senbaklavaci O, Kaneko Y, Bartunek A, et al. Rupture and dissection in pulmonary artery aneurysms: incidence, cause and treatment—review and case report J Thorac Cardiovasc Surg 2001;121:1006-1008.[Free Full Text]
- van Rens MT, Westermann C, Postmus PE, Schramel F. Untreated idiopathic aneurysm of the pulmonary artery: long-term follow-up Respir Med 2000;94:404-405.[Medline]
- Veldtman GR, Dearani JA, Warnes CA. Low pressure giant pulmonary artery aneurysms in the adult: natural history and management strategies Heart 2003;89:1067-1070.[Abstract/Free Full Text]
- Force SD, Lau CL, Moazami N, Trulock EP, Patterson GA. Bilateral lung transplantation and pulmonary artery reconstruction in a patient with chronic obstructive pulmonary disease and a giant pulmonary artery aneurysm J Thorac Cardiovasc Surg 2003;126:864-866.[Free Full Text]
- Wekerle T, Klepetko W, Taghavi S, Birsan T. Lung transplantation for primary pulmonary hypertension and giant pulmonary artery aneurysm Ann Thorac Surg 1998;65:825-827.[Abstract/Free Full Text]
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Abstract
Introduction
