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Ann Thorac Surg 1997;64:585-586
© 1997 The Society of Thoracic Surgeons
Department of Cardiovascular Surgery and Services of Pneumology and Anaesthesia, University Hospital of Catanzaro, Catanzaro, Italy
To the Editor:
We read with interest the article by Chen and associates [1] about a case of giant aneurysm of main pulmonary artery, and we would like to report our experience in this field, discussing the clinical course and therapeutic strategy.
In 1995 a 53-year-old man was admitted to our hospital for evaluation of a mass in the area of the pulmonary notch revealed by chest roentgenography. The progressive radiologic enlargement of this mass over a 10-year period had been noted at another hospital. The history of the patient revealed cigarette abuse of 2 packs/day with a severe chronic obstructive pulmonary disease and a previous diagnosis of systemic lupus erythematosus treated with prednisolone therapy.
The laboratory tests confirmed the diagnosis of systemic lupus erythematosus, and two-dimensional transthoracic echocardiography showed a dilatation of the main pulmonary artery in the absence of any left-to-right intracardiac shunting. Nuclear magnetic resonance imaging was performed, and an aneurysm of the trunk of the pulmonary artery was demonstrated without evidence of dissection (Fig 1
). Cardiac catheterization revealed normal-appearing left and right coronary angiography, normal left ventricular angiography, and no intracardiac shunts. The mean pulmonary artery pressure was 42 mm Hg (55/32 mm Hg), and the mean capillary wedge pressure was 12 mm Hg.
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As reported by others [1], an aneurysmal pulmonary trunk without left-to-right shunting is a rare cardiac anomaly with a relatively benign prognosis in the absence of pulmonary hypertension [2]. The natural history of pulmonary artery aneurysms including contribution to the pathogenesis of pulmonary hypertension and structural arterial wall abnormalities is poorly understood. Moreover, the occurrence of these aneurysms is uncommon, and the correlation between the enlargement of the aneurysm and the level of pulmonary artery pressure is unclear.
In our case we speculate that the progressive structural defect on the arterial wall caused by systemic lupus erythematosus as previously reported [3, 4] and the corticosteroid therapy [5] were probably the primary causes of pulmonary artery aneurysm rather than the moderate pulmonary hypertension. Unfortunately, the refusal of the operation by the patient with the consequent lack of histologic findings did not allow us to confirm this hypothesis.
Angiographic findings currently represent the gold standard for careful evaluation of the aneurysmal extention and hemodynamic pressures. Moreover, according to others [6] we consider nuclear magnetic resonance a very useful noninvasive imaging modality, especially in the detection of possible intimal flap and to follow up the size of the aneurysm in the course of the years.
Although a previous report [7] indicates that surgical repair (interposition of Dacron prosthesis or homograft, reconstruction with a pericardial patch, aneurysmorraphy, or arterioplasty) is recommended when these aneurysms are discovered, it is our firm belief that operation could be performed only when the risk to the patient is acceptably low, when a progressive and constant increase in the arterial diameter is well documented, or in the presence of dissection.
We hope that this discussion is helpful to add further information regarding a cardiovascular anomaly that still presents a clinical course that is not well defined.
References
Divisions of Cardiovascular Surgery and Cardiology, Kaohsiung Medical College, 100 Shih-Chuan 1st Rd, Kaohsiung, Taiwan
Reply To the Editor:
We are grateful for the opportunity to reply to the letter of Dr Mastroroberto and associates regarding our report of giant aneurysm of main pulmonary artery [1]. Pulmonary hypertension is reputed to be an important factor in the pathogenesis of pulmonary arterial aneurysm [2]. Although pulmonary hypertension is found in some patients with aneurysm of the main pulmonary artery, the occurrence of these aneurysms among the population of patients with pulmonary hypertension is rare [3]. Thus, we would concur with their speculation that the progressive structural defect caused by systemic lupus erythematosus was probably the primary cause of pulmonary artery aneurysm rather than the moderate pulmonary hypertension. Regretfully, their speculation was not proved by histologic findings.
Reports of pulmonary arterial aneurysms with vasculitis are strikingly rare [4]. However, vasculitis syndromes (such as Behçet's disease or giant cell arteritis) can be a substrate for vascular degeneration and then pulmonary arterial aneurysm formation [5–7].
Aneurysm of the main pulmonary artery is really a rare entity. Given the paucity of available data on patients with pulmonary arterial aneurysms, the only certainty is that these aneurysms do have the potential to rupture [8]. Operative treatment is commonly recommended after the diagnosis is established [4]. This philosophy appears to be based solely on anecdotal reports of fatal aneurysm rupture [8, 9]. The aneurysmal rupture is frequently manifested clinically as sudden death and may occur in patients with associated [2] or without associated [10] pulmonary hypertension. The relationship between size and location of the aneurysm, rate of diameter enlargement, pulmonary pressures, and subsequent dissection or rupture remains undefined. Therefore, parallels drawn from the known natural history of patients with aneurysms elsewhere in the body may or may not be applicable [8]. We do think that the clinical decision regarding surgical intervention for aneurysm of the main pulmonary artery should not have a similar aggressive attitude as in patients with aneurysm of the thoracic aorta, especially in the presence of normal pulmonary artery pressure. However, surgical management would be recommended in patients with dyspnea on exertion, chest pain, hemoptysis, or huge size of the aneurysm because conservative treatment will undoubtedly result in rupture with fatal outcome [4, 8, 9].
References
This article has been cited by other articles:
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  K. K.S. Nair and A. M. Cobanoglu Idiopathic main pulmonary artery aneurysm Ann. Thorac. Surg., May 1, 2001; 71(5): 1688 - 1690. [Abstract] [Full Text] [PDF]
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