Dissecting aneurysm of the aorta complicating hypertension

After a short period of slight limitation of physical activity, as shown by dyspnea on movement, an elderly man with persistent hypertension, dilatation of the aorta, and cardiac enlargement, and an aortic diastolic murmur, had a brief attack of substernal oppression, followed three weeks later by agonizing substernal pain, which continued for four days without remission and was accompanied by equally severe lumbar pain.The character and duration of this pain served to rule out the usual forms of angina pectoris; the continued myocardial sufficiency, and the absence of physical, including electrocardiographic, signs discouraged a diagnosis of coronary thrombosis.At necropsy, the finding of a dissecting aneurysm of the aorta, apparently of several weeks development, judging from the formation of an endothelial lining in the upper part of the new tract, provided an admirable explanation of the clinical course and symptoms. The initial rupture of the intima may be associated with the first attack of substernal distress; the splitting of the media produced the extreme pain of the second attack. Throughout the descending thoracic and abdominal aorta the advancing extravasation of blood brought about, it may be supposed, a rapid dissection with little or transient pain; but the obstruction to the progress of the aneurysm in the region of the bifurcation was productive of the pain in the lumbar region. Finally, the terminal rupture occurred through the adventitia into the left mediastinum, within which the blood was confined until the perforation into the pleural cavity.From the pathological standpoint, the comment may be added that in the aorta, as elsewhere, sclerotic changes were not marked, and there was no evidence whatever of syphilis. Both of these facts are worthy of emphasis. The later and more marked changes in the development of atherosclerosis, namely, the fatty and calcareous deposits, tend to separate the intima from the media, and an injury to the former is less likely to involve the latter; while in the earlier stage, with what Adami⁴ called the “hyaline fibroid” lesions, the connection between the two coats is intact, and an intimal tear is carried through into the media. In regard to the second point, the lamellar arrangement of the elastic fibers of the media is the basis of the formation of the dissecting aneurysm, for the blood finds it easier to separate the weak connective tissue binding these layers together and make its way along the vessel, than to rupture the tough elastic fibers which lie directly in its course. Syphilitic mesaortitis destroys this orderly arrangement of the medial lamellae, thus preventing dissection, and the syphilitic aneurysm remains localized.     

Dissecting aneurysm of the pulmonary artery in a case of unoperated patent ductus arteriosus

A 54-year-old woman with unoperated patent ductus arteriosusdeveloped fatal cardiac tamponade due to a dissecting aneurysmof the pulmonary artery causing haemopericardium. Histologyof the pulmonary artery showed the presence of cystic medialnecrosis. Previous reports of this condition are reviewed andthe role of pulmonary hypertension causing secondary cysticmedial necrosis is discussed.     

Hybrid melody pulmonary valve replacement in an adult with severe pulmonary hypertension and pulmonary artery aneurysm

A 48‐year‐old female with D‐TGA, ventricular septal defect (VSD), pulmonary stenosis, pulmonary hypertension (PAH), and total anomalous pulmonary venous connection underwent hybrid intervention for a pulmonary artery (PA) aneurysm and replacement of a dysfunctional pulmonary valve (PV). She underwent a hemi‐Mustard procedure at 9 years of age but remained cyanotic. She developed atrial fibrillation, heart failure, and functional decline at 43 years of age. A chest CT demonstrated a 6 cm PA aneurysm that upon re‐imaging at 48 years had increased to 11 cm. A catheterization procedure revealed severe PS, PR, residual VSD, severe PAH with a pulmonary vascular resistance of 30 Wood units. She was evaluated and turned down for heart‐lung transplantation at another institution. She was subsequently referred to our institution for heart‐lung transplantation but was felt to be at unacceptably high risk given the complexity of her anatomy, imaging suggesting liver cirrhosis and liver biopsy with extensive fibrosis. After extensive discussion of risk and benefits, the patient agreed to proceed with a hybrid intervention, consisting of surgical aneurysm resection/PA repair, tricuspid valve repair; PV replacement with a Melody valve, and VSD closure. There were no complications and she was discharged home within 2 weeks. Six months post procedure, she is not on oxygen, her resting room air saturation is 94%, and echocardiography shows stable Melody valve function. This case highlights the utility of a hybrid approach in the treatment of an adult with complex congenital heart disease, heart failure and severe PAH, considered at the highest risk for adverse surgical outcomes. The short‐term efficacy of the Melody valve in severe PAH is reassuring. © 2013 Wiley Periodicals, Inc.     

Huge pulmonary artery aneurysm

Pulmonary artery aneurysms (PAAs) are uncommon entities. PAAs are caused mostly by trauma (often iatrogenic), infections and Behcet’s disease (BD). Less common causes are pulmonary hypertension, congenital heart disease and neoplasm. BD is a multisystem disorder presenting with recurrent oral and genital ulcerations, as well as ocular involvement, and PAA is one of its rare complications. A case of huge PAA, in which the usual criteria for the clinical diagnosis of BD were present, is described. Transcatheter embolization resulted in clinical improvement.     

Main pulmonary artery aneurysm

Alterations of main pulmonary artery have been described in literature. Main pulmonary artery aneurysm is very rare with few available published data. We present a case of echocardiographic finding of a main pulmonary artery aneurysm in a 78-year-old Italian woman.     

Idiopathic aneurysm of the pulmonary artery

Aneurysm of pulmonary artery (PAA) is relatively rare clinical finding and appears mostly in association with significant cardiovascular or pulmonary abnormalities and is frequently caused by pulmonary hypertension. Its diagnosis is not difficult. However, guidelines for the treatment were not yet established. Patients with PAA are at risk for sudden death by a rupture of aneurysm. Therefore, surgical correction (graft replacement) is the method of choice. Nevertheless, it seems that certain group of patients with asymptomatic idiopathic aneurysm of pulmonary artery without presence of any serious cardiovascular or pulmonary abnormality can profit from conservative approach. We refer a 62-years old man with idiopathic PAA, in whom we did not indicate surgical correction. However, the long-term follow-up is necessary.     

Peripheral pulmonary artery aneurysms in patients with pulmonary artery hypertension

Pulmonary artery aneurysm (PAA), especially the peripheral type, is a rare disease. We report 2 cases of peripheral PAA with pulmonary artery hypertension. Complication associated with peripheral PAA was hemoptysis. Endovascular coil embolization was performed successfully in one patient. The other with peripheral PAA was died of massive hemoptysis. In patients with peripheral PAA, coil embolization is one therapeutic option. We summarized cases with peripheral PAA in Japan.     

Idiopathic pulmonary artery aneurysm

Pulmonary artery aneurysm is a rare clinical condition. It is known to be associated with certain congenital or acquired conditions, yet its etiology is not well understood. Its diagnosis is often not challenging. However, controversy still remains regarding its treatment. We report a case of idiopathic pulmonary artery aneurysm treated by surgical correction.