Giant (congenital ? idiopathic ?) Aneurysm of the Pulmonary Trunc in a 64 Year Old Man

This 64 year old patient  was told by his mother, that at birth a heart murmer was noted. At the age of seven, he suffered an infection with the polio virus and consecutive severe paralysis of his right leg. He never complained of cardiac symptoms. In 1963 and in 1979 a plain chest film showed a large pulmonary trunc with calcifications. Selective pulmonary arteriography was undertaken to prove pulmonary embolisms presumably having repeatedly originated from the right leg and having led to a severe pulmonary artery hypertension. However, this exam did not show pulmonary emboli. Both in 1963 and in 1979 the mean pulmonary artery pressure was 33 mm Hg.

Because of intermittent atrial fibrillation in 1979, this patient was treated with flecainid and oral anticoagulation. Few years later he was still free of cardiac symptoms and refused medications. He was referred for a cardiological work up in October 1999, still without specific cardiac symptoms.

Echocardiography showed a peak systolic pulmonary artery pressure of 64 mm Hg in a huge pulmonary artery (6.7 cm), confirmed by CMR ( Cine MR, Fig 1, T1 weighted spin echo, Fig 2). On quantitative flow, the pulmonary valve was leaking (20 ml, right ventricular stroke volume 102 ml, right ventricular EF 50%, Fig 3).

Comments:

The exact reason for this huge pulmonary trunc aneurysm remains unclear. However, given the history and the presentation, a congenital or "idiopathic" origin is very likeli; however, concommittent pulmonary embolism from the severly paretic right leg may have occured over decades.

   Figure 1                                  Figure 2                                          Figure 3

References:

Aneurysm of pulmonary trunk [letter; comment]

COMMENTS: Comment on: Ann Thorac Surg 1996 Jul; 62(1):272-4

ARTICLE SOURCE: Ann Thorac Surg (United States), Aug 1997, 64(2) p586-7

Dissecting aneurysm of the pulmonary artery associated with a large facial cavernous haemangioma.

ARTICLE SOURCE: Histopathology (England), Apr 1997, 30(4) p390-2

AUTHOR(S): Shanks JH; Coup A; Howat AJ

Pulmonary artery aneurysm: is surgery always indicated?

ARTICLE SOURCE: Acta Cardiol (Belgium), 1997, 52(5) p431-6

AUTHOR(S): Casselman F; Meyns B; Herygers P;

Pulmonary artery aneurysm.

AUTHORS: Taniguchi I; Takemoto N; Nakamura Y; Suzuki Y; Yamaga T

SOURCE: Jpn J Thorac Cardiovasc Surg 1999 May;47(5):221-5

ABSTRACT: Main pulmonary artery aneurysm is an exceedingly rare entity. We present a case of main pulmonary artery aneurysm with patent ductus arteriosus in a sixty-year-old woman. The aneurysm was successfully treated with aneurysmectomy and primary anastomosis of the defect of the main pulmonary artery, and the patent ductus arteriosus was divided. The etiology, operative indication and surgical intervention of main pulmonary artery aneurysm are discussed along with a review of the literature.

Idiopathic dilatation of the pulmonary artery: report of four cases.

AUTHORS: Ugolini P; Mousseaux E; Sadou Y; Sidi D; Mercier LA; Paquet E; Gaux JC

AUTHOR AFFILIATION: INSERM U494, Hopital Broussais, Paris, France.

SOURCE: Magn Reson Imaging 1999

ABSTRACT: Idiopathic dilatation of the pulmonary artery (IDPA) is a rare congenital disease which is usually detected fortuitously on chest x- ray, thus radiologists must be aware of this clinical entity. This report describes four cases to which Magnetic Resonance Imaging (MRI) played a major role in diagnosing IDPA and in detecting the concomitant findings observed in this disease. MRI is a non-invasive procedure with many advantages for the accurate and reproducible measurement of artery structures, which makes it the preferred option for combined use with echocardiography in the diagnosis and follow-up of patients with IDPA.