Acquired pulmonary stenosis and pulmonary artery compression

Abstract

Three patients suffering from acquired pulmonary stenosis are described resulting from compression of the main pulmonary artery by anterior mediastinal teratoma, Hodgkin's disease, and aneurysm of the ascending aorta, respectively; also four others who had compression of the right or left primary branch of the pulmonary artery. The pulmonary artery compression was demonstrated angiographically in every case, and in the patient with right and left pulmonary artery compression the main pulmonary artery pressure tracing was characteristic of bilateral pulmonary artery stenosis. Cardiac catheterization and selective angiography are indicated in patients who have evidence of right ventricular outflow obstruction to determine the site and severity of the obstruction and to differentiate between extrinsic compression and intrinsic stenosis.There are two main types of acquired pulmonary stenosis; both are rare. The first is due to intrinsic disease of the pulmonary valve itself. The second results from extrinsic compression of the pulmonary artery and it is this with which we are concerned here. We believe it to be more common than has been realized, mainly because angiographic studies are not usually carried out on these patients.In this paper we report three cases of acquired compression of the main pulmonary artery and four of compression of its right or left primary branch. All patients have been studied by right heart catheterization and angiography at Brompton Hospital during the past four years. CASE REPORTS CASE 1 (E. T.) A completely symptom-free girl of 15 years was found on routine chest radiography to have a mass at the left hilum.On examination there was a loud pulmonary systolic ejection murmur and thrill and a single second sound. Abnormal pulsation was palpable in the second and third left intercostal spaces near the sternal edge. The electrocardiogram showed a mean frontal QRS vector of + 110' and suggested mild right ventricular hypertrophy. Chest radiography showed an oval mass in the left hilar region which measured 10 cm. in its long diameter and was continuous with the mediastinal shadow (Fig. 1). In the lateral view this mass lay anteriorly and contained a fleck of calcification. The heart appeared normal.Cardiac catheterization showed a right ventricular pressure of 90/0 mm. Hg. and a right pulmonary artery pressure of 17/3 mm. Hg. The left pulmonary artery could not be entered. The cardiac output was 4 0 1./min. and pulmonary vascular resistance 1-7units.An angiocardiogram showed that the mass was compressing and displacing the pulmonary infundibulum and main pulmonary artery downwards and backwards. The mass itself did not opacify (Figs 2 and 3).Left thoracotomy revealed a smooth, pale yellow, cystic mass, approximately 10x 7*5 cm., protruding from the mediastinum towards the left. Medially and inferiorly the mass contained a soft cystic area which rested on the pericardium over the region of the pulmonary valve, and a firmer more solid bar of tumour extended backwards from this above and lat...

Lymphoma presenting as an ejection systolic murmur

“…1,2 It is uncommon for mediastinal masses to compress the heart or pulmonary artery sufficiently to produce murmurs or haemodynamically important obstruction. 3,4 This is thought to be due to the tendency of mediastinal tumours to enlarge laterally and produce superior vena cava obstruction instead of extrinsic compression of the heart.…”

Acquired supravalvular pulmonary stenosis due to extrinsic compression by a metastatic thymic carcinoid tumor

“…In the pediatric population this is most commonly seen following corrective switch operations for transposition of the great vessels [3]. In the adult population, it is most frequently due to compression by a mediastinal structure, most commonly an aortic aneurysm arising in the ascending aorta or aortic arch, and less frequently, a mediastinal tumor mass [1,4]. Acquired pulmonary stenosis classically presents with signs and symptoms of right sided heart failure.…”

“…Extrinsic compression of the pulmonary artery has been well documented in the literature [1,2]. In the pediatric population this is most commonly seen following corrective switch operations for transposition of the great vessels [3].…”

Sarcoidosis, pulmonary hypertension, and acquired peripheral pulmonary artery stenosis

“…In addition, acquired central pulmonary artery stenosis has been reported with constrictive percarditis [3], aortic aneurysm [2,3], bronchogenic cyst [6], tuberculosis [2], and sarcoidosis [7-101. Acquired peripheral pulmonary artery stenosis is even more uncommon.…”

“…Acquired stenosis of the main or major pulmonary artery branches is uncommon and usually occurs from extensive compression from tumors (teratomas [ 1,2,3], thymoma [ 1,3], lymphoma [ 1,2,3], sarcoma [ 1,3], bronchogenic carcinoma [ 1,3], lymphoblastoma [4], and intrapericardial mesothelioma [5]). In addition, acquired central pulmonary artery stenosis has been reported with constrictive percarditis [3], aortic aneurysm [2,3], bronchogenic cyst [6], tuberculosis [2], and sarcoidosis [7-101.…”