Left main coronary artery extrinsic compression syndrome: a combined intravascular ultrasound and pressure wire

The compression of the left main coronary artery (LMCA) secondary to pulmonary artery trunk dilatation is a relatively new entity that has been associated with severe pulmonary hypertension. It is associated with acyanogen congenital cardiopathies or idiopathic pulmonary arterial hypertension. The natural history is unknown and its treatment has been based more on the severity of the angiographic compression than on the objective demonstration of myocardial ischemia. We report a case of a woman with severe pulmonary arterial hypertension due to an atrial septal defect with extrinsic compression of the LMCA and a physiopathologic approach to guide its treatment.     

A case of idiopathic bronchial artery aneurysm connecting to the pulmonary artery]

Case. A 54-year old woman was found to have an abnormal shadow in a regular checkup chest X-ray film. We suspected a bronchial artery aneurysm based on a contrast-enhanced chest CT. Aortography, bronchial arteriography and pulmonary arteriography showed a bronchial artery aneurysm, 2 cm in diameter, connecting the right bronchial artery, inferior phrenic artery, and pulmonary artery. Bronchoscopy revealed dilatation and hypervascularity of moniliform submucosal vessels below the right truncus intermedius. She underwent bronchial artery embolization several times, but new feeding vessels developed each time. We considered this case required surgical resection. Bronchoscopy after aneurysmectomy revealed decrease of moniliform submucosal vessels. Histological examination revealed a three-layer structure leading to both the pulmonary and bronchial arteries. Conclusion. We diagnosed idiopathic bronchial artery aneurysm connecting to a pulmonary artery.     

Idiopathic dilatation of the pulmonary artery with pulmonic insufficiency

A case of idiopathic pulmonary artery dilatation with congenital pulmonic insufficiency is presented. The diagnosis of congenital pulmonary regurgitation is discussed, and the atypical features of the diastolic murmur are emphasized. The diagnosis can be confirmed at right heart catheterization. The long-term prognosis of this condition is still unknown.     

Pulmonary valve echo motion in pulmonary regurgitation.

Four cases are presented to illustrate the echo patterns of pulmonary valve motion in patients with pulmonary regurgitation caused by pulmonary hypertension, idiopathic dilatation of the pulmonary artery, or congenital absence of the pulmonary valve or in association with pulmonary stenosis. Absence of the pulmonary 'a' wave, fluttering of the e-f slope, and midsystolic closure or 'notching' of the valve were noted with pulmonary hypertension. In the case with idiopathic dilatation of the pulmonary artery a normal echo pattern of pulmonary valve motion along with distinct dilatation of the pulmonary artery at the valvular level were present. Pronounced dilatation and systolic expansion of the pulmonary artery along with dilatation of the right ventricle were seen with congenital absence of the pulmonary valve. No pulmonary valve could be demonstrated on multiple scans from the right ventricle to pulmonary artery. In Case 4 large 'a' waves (14 mm) were noted, indicating a reversal of the normal end-diastolic gradient across the valve and suggesting that pulmonary regurgitation in this case was associated with right ventricular outflow obstruction. Study of the echo pattern of pulmonary valve motion may therefore provide useful information in establishing the cause of pulmonary regurgitation.     

Pulmonary valve echo motion in pulmonary regurgitation.

Four cases are presented to illustrate the echo patterns of pulmonary valve motion in patients with pulmonary regurgitation caused by pulmonary hypertension, idiopathic dilatation of the pulmonary artery, or congenital absence of the pulmonary valve or in association with pulmonary stenosis. Absence of the pulmonary 'a' wave, fluttering of the e-f slope, and midsystolic closure or 'notching' of the valve were noted with pulmonary hypertension. In the case with idiopathic dilatation of the pulmonary artery a normal echo pattern of pulmonary valve motion along with distinct dilatation of the pulmonary artery at the valvular level were present. Pronounced dilatation and systolic expansion of the pulmonary artery along with dilatation of the right ventricle were seen with congenital absence of the pulmonary valve. No pulmonary valve could be demonstrated on multiple scans from the right ventricle to pulmonary artery. In Case 4 large 'a' waves (14 mm) were noted, indicating a reversal of the normal end-diastolic gradient across the valve and suggesting that pulmonary regurgitation in this case was associated with right ventricular outflow obstruction. Study of the echo pattern of pulmonary valve motion may therefore provide useful information in establishing the cause of pulmonary regurgitation.     

A case of primary leiomyosarcoma of the pulmonary artery]

A 74-year-old woman was admitted to our hospital because of right chest pain. The chest radiograph showed right hilar pulmonary artery dilatation. A mass exhibiting low intensity in T1-weighted images and high intensity in T2-weighted images was disclosed in the right pulmonary artery. Because of its clinical course and the MRI findings, the mass was thought to be tumorous tissue, and so pneumonectomy was performed. Leiomyosarcoma was diagnosed from the histological findings. Primary artery sarcoma is rare and the prognosis is considered to be extremely poor. The patient was successfully treated and had a good clinical outcome.     

Idiopathic aneurysm of the pulmonary artery trunk. Report of a case

Aneurysms of the aorta are frequent and treatment is well known, correlated with a statistical risk of rupture. Pulmonary artery aneurysms are less frequent. They may occur in connection with other conditions (infection, cardiopathy, notably pulmonary artery hypertension, endovascular trauma) or much more exceptionally regarded as idiopathic. Chest x-ray, CT-scan and digitalized pulmonary angiography and echocardiography give the diagnosis and help evaluate extension and localization. We report the case of a 72-year-old woman who developed idiopathic aneurysm of the left pulmonary artery which was discovered fortuitously. Because of the stability of the lesion and the lack of any worsening factor, we decided not to operate this high-risk patient. After 3 years, no complication has been observed and the CT-scan shows no evolution. In case of proximal idiopathic aneurysm of the pulmonary artery, the indication of surgery should be discussed.     

Mid systolic notching of the pulmonary valve in the absence of pulmonary hypertension.

In a patient with idiopathic dilatation of the pulmonary artery the pulmonary valve echogram showed a prominent mid systolic closing motion or notching indistinguishable from that seen in pulmonary hypertension. Normal right ventricular and pulmonary arterial pressures were recorded simultaneously with echocardiograms of the pulmonary valve.     

Pulmonary artery aneurysm as a cause for chest pain in a patient with Noonan's syndrome: a case report

A pulmonary artery aneurysm is defined as pulmonary artery dilatation >4 cm. Pulmonary artery aneurysms are rare findings and are usually not associated with chest pain. This is the first report of a case of pulmonary artery aneurysm causing chest pain in a patient with Noonan's syndrome. Chest X-ray showed cardiomegaly and a prominent main pulmonary artery segment and CT confirmed a pulmonary artery aneurysm with a pulmonary artery root measuring 5.6 cm. The patient underwent pulmonary valvuloplasty as well as pulmonary arterioplasty to repair the pulmonary artery aneurysm, and experienced subsequent relief of chest pain. In conclusion, pulmonary artery aneurysm is a rare complication in patients with Noonan's syndrome and can be the cause of chest pain in such patients.     

Left coronary artery arising from the pulmonary artery. Demonstration by 2-dimensional echocardiography

The authors report the case of a baby in whom an ectopic left coronary artery arising from the pulmonary artery was suspected on clinical and, in particular, electrocardiographic criteria. M mode echocardiography showed left ventricular dilatation with reduced shortening fraction of the LV internal diameter. 2D echocardiography with a mechanical sector scanner demonstrated the left coronary artery arising from the pulmonary artery. The short axis view of the basal vessels in the left parasternal incidence visualised 2 cm of the left coronary artery arising from the posterior part of the pulmonary artery.     

Cross-sectional echocardiographic findings of anomalous origin of left coronary artery from pulmonary artery.

Anomalous origin of the left coronary artery and left ventricular myocardial hypokinesis were visualised by cross-sectional echocardiography. Significant dilatation of the right coronary artery was another important finding. Cross-sectional echocardiography appears to be useful for evaluating patients with anomalous origin of the left coronary artery from the pulmonary artery.     

前列环素治疗肺动脉高压的临床应用进展

本文综述了近年来前列环素治疗肺动脉高压的临床应用进展。目前已有多种前列环素制剂可以应用于临床：静脉、皮下、口服或吸入;临床治疗范围包括特发性肺动脉高压及结缔结组织性疾病、门脉高压、HIV感染、食欲抑制剂伴肺动脉高压。临床研究结果显示前列腺素制剂可以延长患者寿命、改善心功能、改善运动耐量、改善血液动力学指标（增加心输出量、降低肺动脉压和肺血管阻力）。     

Untreated idiopathic aneurysm of the pulmonary artery; long-term follow-up

A 57-year-old man with an idiopathic aneurysm of the left pulmonary artery is presented. The aneurysm was stable over many decades. No such long-term follow-up of an idiopathic pulmonary aneurysm has been described previously. After 39 years there is no evidence of heart failure or pulmonary hypertension. Because corrective surgery has a variable outcome, and prognosis is suggested to be good in the idiopathic form of pulmonary aneurysm, no surgical correction was proposed.     

Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger's syndrome: a clinical dilemma

Although the frequency of haemoptysis in Eisenmenger's syndrome is well recognised, the high prevalence of pulmonary artery thrombus has been newly appreciated through the growing use of non-invasive imaging. Three patients with Eisenmenger's syndrome with haemoptysis are reported who underwent computed tomography pulmonary angiography and cardiovascular magnetic resonance. Each patient was found to have aneurysmal dilatation of the right pulmonary artery with large laminar thrombus. These cases illustrate a rising clinical problem in this special population-that is, how to treat and prevent large pulmonary artery thrombosis in the setting of haemoptysis. The authors discuss their approach to these cases and the known literature.     

Fibrosing mediastinitis with peripheral airway dilatation and central pulmonary artery occlusion

We present a case of fibrosing mediastinitis causing peripheral airway abnormalities on CT consisting of bronchial dilatation and wall thickening due to longstanding central pulmonary artery obstruction. The peripheral airway dilatation is similar in appearance to that which has been described in patients with chronic pulmonary embolism.     

Ultrasonic features of anomalous origin of the left coronary artery from the pulmonary artery

Two cases of anomalous origin of the left coronary artery from the pulmonary artery with and without marked dilatation of the right coronary artery were studied by M-mode echocardiography and cross-sectional echocardiography. The M-mode echocardiogram obtained from the case with the dilated right coronary artery demonstrated an abnormal structure anterior to the aortic root. The cross-sectional echocardiogram of the same case visualized the origin of the dilated right coronary artery. In addition, echocardiographic abnormalities suggesting the ischemia of the left ventricle were observed in both cases. Although many of the ultrasonic features are nonspecific, the combination of ultrasonic technique provides useful clues to the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery.     

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.     

Analysis of blood flow in pulmonary hypertension with the pulsed Doppler flowmeter combined with cross sectional echocardiography.

Blood flow patterns were analysed at nine points in the pulmonary area using the pulsed Doppler technique combined with cross-sectional echocardiography in 53 patients with heart disease and 10 healthy subjects. In subjects with a normal pulmonary artery pressure the blood flow pattern in systole showed a gradual acceleration and deceleration with a rounded summit in mid systole, designated the round type. In patients with pulmonary hypertension it showed a rapid acceleration and early deceleration with a sharp peak in early systole, designated the triangular type. The acceleration time index, defined as the ratio of the time interval from the beginning to the peak of ejection to the ejection time, showed a significant inverse correlation with mean pulmonary artery pressure. In pulmonary hypertension a prominent reverse flow occurred in the right posterior part of the pulmonary trunk during mid-systole and early diastole, indicating the presence of a vortex. Similar flow patterns were also seen in patients with idiopathic pulmonary artery dilatation. The factors responsible for the triangular type were principally the reduced capacitance and increased impedance of the pulmonary vascular tree. Those responsible for the reverse flow were the curved path of the blood flow and dilatation of the pulmonary artery.     

Analysis of blood flow in pulmonary hypertension with the pulsed Doppler flowmeter combined with cross sectional echocardiography

Blood flow patterns were analysed at nine points in the pulmonary area using the pulsed Doppler technique combined with cross-sectional echocardiography in 53 patients with heart disease and 10 healthy subjects. In subjects with a normal pulmonary artery pressure the blood flow pattern in systole showed a gradual acceleration and deceleration with a rounded summit in mid systole, designated the round type. In patients with pulmonary hypertension it showed a rapid acceleration and early deceleration with a sharp peak in early systole, designated the triangular type. The acceleration time index, defined as the ratio of the time interval from the beginning to the peak of ejection to the ejection time, showed a significant inverse correlation with mean pulmonary artery pressure. In pulmonary hypertension a prominent reverse flow occurred in the right posterior part of the pulmonary trunk during mid-systole and early diastole, indicating the presence of a vortex. Similar flow patterns were also seen in patients with idiopathic pulmonary artery dilatation. The factors responsible for the triangular type were principally the reduced capacitance and increased impedance of the pulmonary vascular tree. Those responsible for the reverse flow were the curved path of the blood flow and dilatation of the pulmonary artery.     

Transesophageal echocardiographic identification of thrombus producing obstruction of left pulmonary artery descending lobar branches and bronchial artery dilatation

We report an elderly patient in whom a thrombus in the distal left pulmonary artery was shown by transesophageal echocardiography to extend and produce obstruction of the descending lobar branches as well as dilatation of the left bronchial artery.