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.     

Unilateral pulmonary artery banding to promote contralateral pulmonary artery growth

A full-term baby with double-outlet right ventricle and total anomalous pulmonary venous connection (TAPVC) complicated with left main pulmonary artery (PA) stenosis, presented with heart failure caused by increased pulmonary blood flow. Based on significant discrepancies in size and development between the left and right PAs, we performed right PA banding concomitant with TAPVC repair to promote left PA growth and restrict overall PA flow. PA-graphy performed 3?months after surgery showed marked increase in the left PA size with appropriately low pressure, which enabled us to successfully complete Glenn anastomosis. Under appropriate patient selection, unilateral PA banding for patients with unbalanced peripheral PA size could serve as an effective and less invasive strategy to simultaneously promote PA growth and control PA flow.     

Combined unilateral pulmonary artery agenesis and contralateral peripheral pulmonary artery stenosis

Unilateral absence of a pulmonary artery is a rare congenital malformation. This report details the finding in two cases of this anomaly associated with peripheral stenosis of the contralateral pulmonary artery.     

Ruptured pulmonary artery aneurysm mimicking pulmonary embolism

Aneurysms of the pulmonary artery are very rare pathological vascular conditions. Peripheral pulmonary aneurysms have been reported only in a few cases. The causes of these aneurysms include extensive degenerative changes, traumas, infection and congenital malformations. Because of the imminent danger of rupture, surgical treatment should always be preferred. The following case report demonstrates one of a multitude of possible misdiagnoses for rupture of a pulmonary aneurysm.     

Reperfusion pulmonary edema after pulmonary artery thromboendarterectomy

Pulmonary artery thromboendarterectomy (PAT) is a potentially curative procedure in chronic, major vessel thromboembolic pulmonary hypertension. However, postoperative reperfusion pulmonary edema (RPE) has been a serious complication, often requiring prolonged mechanical ventilation. This entity has been described only anecdotally in the past. To characterize it more fully, we retrospectively analyzed the course and potential determinants of RPE after thromboendarterectomy in 22 patients who had PAT at our institution from 1969 through 1984. Particular attention was directed to clinical data, thrombus location, areas operated, postoperative roentgenograms, and preoperative and postoperative hemodynamic data. In all patients but 1, RPE developed within 72 h after surgery, corresponding to anatomic locations distal to vessels subjected to PAT. Regions of lung not reperfused at surgery were uniformly spared. Pulmonary capillary wedge and/or left atrial pressures preoperatively and postoperatively were not elevated. None of the preoperative data predicted which patients would develop more persistent RPE. These observations suggest that the phenomenon of RPE is a peculiar, focal form of pulmonary edema, the basis for which remains to be defined.     

Primary pulmonary artery sarcoma mimicking pulmonary thromboembolism

A 69-year-old woman presenting with dyspnea had a pericardial window created for fibrinous pericarditis. The patient subsequently developed pulmonary hypertension and a ventilation perfusion scan was compatible with pulmonary thromboembolism. A primary tumour of the pulmonary artery was suggested by angiography, computerized axial tomography and magnetic resonance imaging. Pathology confirmed a spindle cell pulmonary artery sarcoma.     

Coronary artery to pulmonary artery fistulas.

Twelve patients with a total of 14 coronary artery to pulmonary artery fistulas were discovered at the time of diagnostic coronary angiography. Six patients had severe coronary artery disease, five patients had normal coronary arteriography, one patient had insignificant coronary artery disease, and one patient had rheumatic heart disease. Only two patients had characteristic continuous murmurs; one patient had a normal coronary angiogram, and the second patient had severe coronary artery disease. Ten fistulas originated from the left anterior descending artery, three from the right coronary artery, and one from the left circumflex artery. The fistulas were either composed of one large (five fistulas) or one or more small channels (seven fistulas) or poorly defined plexiform channels (two fistulas). Hydrogen studies performed in two patients were negative and dye dilution curves performed in all patients were normal. In only four out of the six patients with severe coronary artery disease, the fistulas originated from a diseased vessel and in each case the origin was proximal to the narrowing. The pathogenesis and functional role of these fistulas is largely unknown.     

High-pressure pulmonary artery aneurysm and unilateral pulmonary artery agenesis in an adult

The presence of a pulmonary artery aneurysm, major aortopulmonary and coronary-pulmonary collateral vessels, and severe pulmonary hypertension in an adult with unilateral pulmonary artery agenesis and previous patent ductus arteriosus ligation is very rare. A 34-year-old man experienced these conditions. When he was 10 years old, catheterization and angiography revealed right pulmonary artery agenesis, dilation of the main pulmonary artery, multiple collateral vessels extending from the aorta to the right pulmonary system, and a patent ductus arteriosus (shunt ratio, 3.57) that was then ligated; the other conditions were not corrected. This adult patient was in New York Heart Association functional class II; mild central cyanosis was detected only during exercise. The right pulmonary arterial system was seen only at the right hilar area via collateral vessels from the subclavian, bronchial, internal mammary, and intercostal arteries. Angiography revealed collateral vessels from the right and circumflex coronary arteries to the right pulmonary system. The right intraparenchymal pulmonary arterial systems were patent but of small diameter (pulmonary artery pressure, 85 mmHg; ratio of peak right-to-left ventricular pressure, 0.94; peak pulmonary pressure unresponsive to 100% oxygen). Pulmonary vascular resistance was not estimated because of the risk of aneurysmal rupture. We concluded that irreversible pulmonary hypertension had developed (delayed by the patent ductus arteriosus ligation in childhood) and that the patient's only chance for survival was heart-lung transplantation. To sustain the patient until surgery, we administered sildenafil. Herein, we describe the vascular conditions that accompany unilateral absence of the pulmonary artery, and therapeutic methods.     

Primary pulmonary artery rhabdomyosarcoma

A 69-year-old woman presented with a 4-month history of dyspnoea and radiating upper-right quadrant pain and oedema in her lower extremities for more than 20 days. The ultrasonographic study of the heart revealed the adherence of a substantive hypoechoic mass (73 x 34 mm) to the antelateral wall of the pulmonary artery and resultant pulmonary stenosis. Computed tomographic imaging of the pulmonary artery revealed an irregularly shaped filling defect (approximately 41 x 39 x 59 mm) in the main pulmonary artery. The boundary of the defect was irregular, but demarcation with healthy tissue was clear. After surgical treatment, the histologic and immunohistochemical assays revealed a primary pulmonary artery rhabdomyosarcoma. Pulmonary artery rhabdomyosarcomas are usually misdiagnosed as other pulmonary artery obstructive diseases. There should be a greater focus of clinical attention and resection is the appropriate surgical treatment for such malignant tumours.     

Mycotic pulmonary artery pseudoaneurysm

We report a rare case of a mycotic pulmonary artery pseudoaneurysm that was detected on CT examination. The pulmonary artery pseudoaneurysm appeared as a rapidly growing enhancing mass without a draining vein and with evidence of surrounding acute hemorrhage on CT.     

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.