Idiopathic Dilatation of the Pulmonary Artery

E-chocardiography Journal: Alphabetical List / Chronological List / Images / Home Page

Br J Radiol. 2002 Jun;75(894):532-5.
Idiopathic dilatation of the pulmonary artery.
Ring NJ, Marshall AJ.
Departments of Diagnostic Radiology and Cardiology, Derriford Hospital, Derriford Road, Plymouth, Devon PL6 8DH, UK.

Idiopathic dilatation of the pulmonary artery is an uncommon cause of a large main pulmonary artery whose diagnosis is dependent on the exclusion of other causes of central pulmonary artery dilatation. We present four new cases who have been known to have large central pulmonary arteries for many years, and suggest that a long period of observation should be considered to be a further criterion for diagnosis as, in some patients who appear to have this condition, an underlying pathology will become apparent.

Magn Reson Imaging. 1999 Jul;17(6):933-7.
Idiopathic dilatation of the pulmonary artery: report of four cases.
Ugolini P, Mousseaux E, Sadou Y, Sidi D, Mercier LA, Paquet E, Gaux JC.
INSERM U494, Hopital Broussais, Paris, France.

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.

J Formos Med Assoc. 1996 Nov;95(11):873-6.
Idiopathic pulmonary artery aneurysm.
Fang CC, Tsai CC.
Department of Internal Medicine, Tainan Municipal Hospital, Taiwan, ROC.

The purpose of this article is to report two cases of pulmonary artery (PA) aneurysm in patients who had no evidence of left-to-right intracardiac shunting or other known relevant etiologies. One patient, a 52-year-old woman, was admitted to the hospital due to exertional palpitation, while the other patient, a 73-year-old woman, came to the hospital because of fever and cough. In both women, chest radiographic findings of a hugely dilated PA were confirmed by computed tomography (CT). In both cases, a ventriculogram showed fusiform dilatation of the main PA without evidence of pulmonary valve stenosis. The results of chest radiography, two-dimensional echocardiography, CT of the thorax, including pulmonary angiography in one patient, were compatible with the diagnosis of PA aneurysm. No specific drug regimen was administered nor was any surgical intervention performed in either patient. Both patients were asymptomatic as of the last telephone follow-up. Idiopathic PA aneurysm is a benign condition with better survival than PA aneurysms of other etiologies, because of the absence of left-to-right intracardiac shunting and significant pulmonary hypertension. PA aneurysm must be considered as part of the differential diagnosis in an enlarged pulmonary trunk seen on chest radiogram, and two-dimensional echocardiography should be performed initially.

Singapore Med J. 1996 Aug;37(4):378-9.
Two-dimensional and Doppler echocardiographic abnormalities in idiopathic dilatation of the pulmonary artery.
Chia BL, Ee BK.
Cardiac Department, National University Hospital, Singapore.

Although idiopathic dilatation of the pulmonary artery is uncommon, it requires to be distinguished from other important congenital heart diseases for which it may be confused clinically. We describe the two-dimensional (2D) and Doppler echocardiographic findings in two patients with this condition. Enlargement of the main pulmonary artery and right ventricle was demonstrated in both patients using 2D echocardiography. Pulmonary regurgitation was confirmed in Case 1 by both pulsed-Doppler echocardiographic and Doppler colour flow imaging studies and in Case 2 by microbubbles (which were generated from saline-injection into a peripheral vein) oscillating up and down across the pulmonary valve in systole and diastole respectively.

Angiology. 1996 Jan;47(1):87-92.
Idiopathic dilatation of the pulmonary artery. A case presentation.
Chang RY, Tsai CH, Chou YS, Wu TC.
Department of Internal Medicine, Mackay Memorial Hospital, Taipei, Taiwan, R.O.C.

Idiopathic dilatation of the pulmonary artery is an uncommon, but not rare, congenital anomaly that is diagnosed by exclusion and generally has a benign clinical course. A left hilar mass on the chest x-ray film is the major presentation. The authors present a case of idiopathic dilatation of the pulmonary artery with posttraumatic chest pain clinically. After serial examinations, idiopathic dilatation of the pulmonary artery was diagnosed. They discuss the phenomenon of transpulmonary valvular pressure gradient and noninvasive tools for long-term follow-up, including transesophageal echocardiography and magnetic resonance imaging.

Arch Mal Coeur Vaiss. 1994 May;86(5):663-6.
Idiopathic dilatation of the pulmonary artery. Echocardiographic aspects
Boutin C, Davignon A, Fournier A, Houyel L, Van Doesburg N.
Service de cardiologie infantile de l'hopital Sainte-Justine, Montreal, Quebec, Canada.

The authors compared 30 patients with at least one clinical sign of idiopathic dilatation of the pulmonary artery, with a group of 20 normal control subjects in order to establish diagnostic criteria for this condition. In the first group, 17 had characteristic radiological findings and 13 others had clinical signs of idiopathic dilatation of the pulmonary artery. Comparison with the control group showed significant differences (p < 0.05) in 4 parameters: 1) the diameter of the pulmonary artery at the bifurcation/m2 body surface area (2.8 +/- o.4 cm versus 2.4 +/- 0.4 cm), 2) the diameter of the aorta 2 cm beyond the aortic valve/m2 of body surface area (1.7 +/- 0.3 cm versus 2.1 +/- 0.7 cm), 3) the ratio of pulmonary artery/aortic diameters at the valve rings (1.4 +/- 0.2 versus 1.1 +/- 0.02); 4) the ratio of the aorta 2 cm beyond the valve/aortic ring (1.02 +/- 0.07 versus 1.09 +/- 0.09). The diagnostic criteria of idiopathic dilatation of the pulmonary artery in the absence of cardiac or pulmonary disease are: 1) ratio of pulmonary artery diameter at its bifurcation/aortic ring diameter or 2 cm beyond the aortic valve of > or = 1.4; 2) ratio of pulmonary/aortic ring diameters > or = 1.5.

Br Heart J. 1993 Mar;69(3):268-9.
Pulmonary artery dissection in a patient with idiopathic dilatation of the pulmonary artery: a rare cause of sudden cardiac death.
Andrews R, Colloby P, Hubner PJ.
Department of Cardiology, Groby Road Hospital, Leicester.

A 31 year old man presented with a left hilar mass. Thoracic tomography showed this mass to be the pulmonary artery, and subsequently idiopathic dilatation of the pulmonary artery was diagnosed. He remained well until 11 years later when he died suddenly. Postmortem examination confirmed idiopathic dilatation of the pulmonary artery with death due to pulmonary artery dissection and cardiac tamponade. It seems likely that idiopathic dilatation of the pulmonary artery predisposed to fatal pulmonary artery dissection.

Zhonghua Nei Ke Za Zhi. 1992 Jan;31(1):24-5, 60.
An analysis of 21 cases of idiopathic dilatation of the pulmonary artery
Zhao YJ, Cheng XS.
Cardiovascular Institute, Chinese Academy of Medical Sciences, Beijing.

Idiopathic dilatation of the pulmonary artery (IDPA) is a disease which, generally speaking, does not need treatment. But a correct differential diagnosis is of great importance. 21 cases of IDPA were reported. The clinical data showed that 66.67% of the patients were asymptomatic and all the patients had normal respiratory and cardiovascular functions. 10% of the cases had had a history of more than 20 years. The results showed that IDPA was a benign anomaly and most of its symptoms were iatrogenic. It was usually misdiagnosed as pulmonary stenosis (42.86%), atrial septal defect (38.10%), pulmonary hypertension (28.57%), and so on. Careful chest X-ray, ECG and UCG examinations are useful for the differential diagnosis, but a clinical diagnosis should be based on the normal result of right heart catheterization.

Folia Med Cracov. 1991;32(1-2):5-14.
Function and importance of the pericardium
Dubiel JP.
I Kliniki Kardiologii Akademii Medycznej im. M. Kopernika, Krakowie.

The pericardium plays an important role in the normal cardiac work. Its major functions include: maintenance of adequate cardiac position, separation from the surrounding tissues of the mediastinum, protection against ventricular dilatation, maintenance of low transmural pressure, facilitation of ventricular interdependence and atrial filling. Congenital absence of the pericardium should be differentiated first of all from atrial septal defect ostium secundum type, idiopathic dilatation of the pulmonary artery and tumours of the left mediastinum.

Am Surg. 1978 Oct;44(10):688-92.
Pulmonary artery aneurysm.
Arom KV, Richardson JD, Grover FL, Ferris G, Trinkle JK.

Less than 200 cases of main pulmonary artery aneurysms have been reported and most came from autopsy series. The aneurysms may be associated with congenital cardiovascular lesions, syphilis, degenerative changes in the arterial wall and possibly trauma. Pulmonary artery aneurysm is a true entity which can occur with or without typical clinical findings. Physicians should pay more attention to the so-called "aneurysmal dilatation" or "idiopathic dilatation" of the pulmonary artery and obtain a correct diagnosis by angiography. Since pulmonary artery aneurysm carries a risk of rupturing, the operation should be considered as soon as the lesion is recognized. Few surgical interventions have been reported.

Chest. 1978 Feb;73(2):241-2.
Idiopathic dilatation of peripheral pulmonary arteries.
Teitelbaum JE, Altman M.

The authors describe a patient with idiopathic dilatation of the peripheral pulmonary arteries radiologicaly mimicking pulmonary arteriovenous fistulae. Isolate involvement of the peripheral arteries in this patient supports a developmental defect in the arterial walls as the etiology.

Back to E-chocardiography Home Page.

The contents and links on this page were last verified on October 31, 2006.