Ann Thorac Surg 1998;65:825-827
© 1998 The Society of Thoracic Surgeons
Case Reports
Lung Transplantation for Primary Pulmonary Hypertension and Giant Pulmonary Artery Aneurysm
Thomas Wekerle, MD,
Walter Klepetko, MD,
Shahrokh Taghavi, MD,
Tudor Birsan, MD
Department of Cardiothoracic Surgery, University of Vienna, Vienna General Hospital, Vienna, Austria
Accepted for publication October 6, 1997.
Dr Klepetko, Department of Cardiothoracic Surgery, Vienna General Hospital, Währinger Gürtel 18, A-1090 Vienna, Austria.
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Abstract
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We report the case of an 18-year-old patient with a giant pulmonary artery aneurysm and primary pulmonary hypertension who was successfully treated with bilateral lung transplantation and complete reconstruction of the pulmonary artery.
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Introduction
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Aneurysms of the pulmonary artery (PA) are an exceedingly rare finding [1]. Most cases are documented in large postmortem series, and only a few instances of successful surgical repair have been reported in the literature so far [2][3].
At the age of 14 years the male patient under discussion was diagnosed with severe primary pulmonary hypertension, but an otherwise normal configuration of the PA. During the ensuing years his clinical condition remained stable under conservative therapy with coumarin and nifedipine. However, at a routine follow-up 3 years after the initial diagnosis, a massive dilatation of the PA was noticed on a chest roentgenogram (Fig 1Fig 2). Despite the otherwise stable clinical situation, the patient therefore was considered as a candidate for combined heart-lung transplantation.
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Intraoperative view after opening of the pericardium: the large pulmonary artery aneurysm leads to distortion of the ascending aorta.
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During the next 18 months on the waiting list the condition of the patient deteriorated progressively, reaching New York Heart Association functional class IV status. In March 1996 the heart-lung block of a blood-group–identical local organ donor became available. During the harvest of the organs it turned out that the heart was unsuitable for transplantation because of extensive coronary artery calcifications. In view of the critical condition of the patient and the uncertainty whether a suitable heart-lung donor would become available in time, the decision was made to proceed with the harvest of the double-lung block and to switch the procedure to a bilateral lung transplantation with complete reconstruction of the PA.
The patient was taken to the operating room, a bilateral transsternal anterior thoracotomy was performed, and total cardiopulmonary bypass was initiated. The heart was arrested with blood cardioplegia and the aorta was cross-clamped. Both lungs were removed with stapling of the pulmonary veins and excision of both main branches and the common trunk of the PA to a level 0.5 cm above the PA valve. Excessive formations of old blood clots were found within the aneurysm sac (Fig 3). The donor heart-lung block was prepared with excision of the heart and division of the two lungs, leaving the PA trunk attached to the right lung. Both bronchial anastomoses and both venous anastomoses were performed in the standard way. The right donor PA was pulled through behind the superior vena cava and aorta and the pulmonary trunk was reanastomosed to the remaining recipient’s PA stump with 5-0 Prolene (Ethicon, Somerville, NJ) sutures. The left PA was then reanastomosed in an end-to-side fashion to the main trunk. After deairing, both lungs were reperfused and ventilated. The patient was weaned from cardiopulmonary bypass and was admitted to the intensive care unit.
Postoperatively, the patient remained hemodynamically stable with only slightly elevated pulmonary arterial pressure initially, which normalized completely within the first 3 postoperative days. The patient was discharged from the hospital 35 days after the transplantation. Eighteen months after the operation, the patient remains in New York Heart Association functional class I. Magnetic resonance imaging studies performed 1 year after transplantation showed a normal-sized heart. The end-diastolic volume of the right ventricle had decreased to 115 mL (compared with 396 mL preoperatively) and the ejection fraction of the right ventricle had improved to 0.70 (0.46 preoperatively).
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Comment
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The incidence of aneurysms of the PA is known to be very low, and few aneurysms arise as a consequence of primary pulmonary hypertension [4][5]. Whenever a large aneurysm formation of the PA is diagnosed, surgical intervention is indicated to prevent the possible danger of rupture or of intravascular thrombus formation. In the described case the rapid decline in the condition of the patient was presumably because of subtotal obstruction of the aneurysm with old blood clots, which occurred despite anticoagulation therapy. Our literature review found only 8 cases of surgical treatment of giant PA aneurysms. In none of these cases was aneurysm formation the consequence of primary pulmonary hypertension. Here we report isolated lung transplantation performed simultaneously with vascular reconstruction of the pulmonary trunk as treatment of such a condition.
Isolated bilateral lung transplantation is the standard procedure for patients suffering from primary pulmonary hypertension referred to our institution. This results in a complete normalization of the pulmonary vascular resistance and recovery of the right ventricular function thereafter [6]. In this patient we initially intended to perform combined heart-lung transplantation because of the existence of the large PA aneurysm. As the heart was found not to be suitable for transplantation, the procedure was changed to bilateral lung transplantation with reconstruction of the PA trunk. Because the complete donor PA was available for transplantation, the reconstruction procedure was markedly facilitated. However, we are convinced that even if the donor heart had been used for another transplantation, interposition of the PA main stem with any graft would have allowed a similar procedure.
This report underlines the potential of isolated lung transplantation as the optimal organ-sparing procedure for treatment of different and even more complex forms of pulmonary hypertension.
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References
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- Deterling RA, Clagett OT Aneurysm of the pulmonary artery: review of the literature and report of a case. Am Heart J 1947;34:471-498.
- Bartter T, Irwin RS, Nash G Aneurysms of the pulmonary arteries. Chest 1988;94:1065-1075.[Free Full Text]
- Chen YF, Chiu CC, Lee CS Giant aneurysm of main pulmonary artery. Ann Thorac Surg 1996;62:272-274.[Abstract/Free Full Text]
- Lüchtrath H Dissecting aneurysm of the pulmonary artery. Virchows Arch Pathol Anat 1981;391:241-247.
- Masuda S, Ishii T, Asuwa N, Ishikawa Y, Kiguchi H, Uchiyama T Concurrent pulmonary arterial dissection and saccular aneurysm associated with primary pulmonary hypertension. Arch Pathol Lab Med 1996;12:309-312.
- Globits S, Burghuber OC, Koller J, et al. Effect of lung transplantation on right and left ventricular volumes and function measured by magnetic resonance imaging. Am J Respir Crit Care Med 1994;149:1000-1004.[Abstract]
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Abstract
Introduction
