Relationship of pulmonary artery diastolic and pulmonary artery wedge pressures in mitral stenosis

Resting and exercise hemodynamic studies were performed in 33 patients with mitral stenosis (14 men and 19 women; average age, 25 years) in normal sinus rhythm with normal pulmonary vascular resistances. A normal pulmonary vascular resistance was assumed when the resting pressure gradient between the pulmonary artery diastolic and mean pulmonary artery wedge pressures was 5 mm. Hg or less. A satisfactory correlation existed between the pulmonary artery wedge and pulmonary artery diastolic pressures at rest (r = 0.9017) and during exercise (r = 0.8670). A method of predicting pulmonary artery wedge pressure from pulmonary artery diastolic pressure during exercise was formulated. The correlation between the predicted and measured exercise pulmonary artery wedge pressures was very close (r = 0.9561). It is suggested that during exercise the pulmonary artery diastolic pressure can be modified as above and substituted for mean pulmonary artery wedge pressure if the resting gradient between pulmonary artery wedge and pulmonary artery diastolic pressure is known.     

Evidence for local platelet activation in pulmonary vessels in patients with pulmonary hypertension secondary to chronic obstructive pulmonary disease.

To investigate the relationships between local platelet activation in pulmonary vessels and pulmonary artery pressure circulating platelet aggregates and plasma beta-thromboglobulin (beta-TG) levels were evaluated in peripheral venous blood and blood from different sites of pulmonary circulation (right ventricle, pulmonary artery and arteriolo-capillary bed) in 29 patients with COPD. Fifteen had pulmonary hypertension and 14 normal pulmonary artery pressure. In normotensive COPD no significant differences could be found in platelet aggregation and beta-TG levels among different sampling sites. On the contrary in patients with pulmonary hypertension a significant increase of platelet aggregates and beta-TG levels was found in blood withdrawn from the arteriolo-capillary bed. A significant correlation was demonstrated between platelet aggregation and both mean pulmonary artery pressure and pulmonary vascular resistance. These results indicate that in patients with pulmonary hypertension secondary to COPD a local platelet activation in pulmonary vessels does occur and may contribute to the maintainance of elevated pulmonary vascular resistance.     

Successful surgical treatment of pulmonary artery sling and partial anomalous pulmonary venous drainage in an infant

A 8 month old infant with pulmonary artery sling and partial anomalous pulmonary venous drainage was reported. The patient was seen initially with severe stridor. Diagnosis was made by barium swallow study, echocardiography, bronchoscopy, and pulmonary arteriography. The patient was operated on through a median sternotomy; the left pulmonary artery was dissected deep between the trachea and esophagus, and then reimplanted to the side of the main pulmonary artery. Systemic heparinization and microsurgical technique were used. Simultaneous intracardiac repair was carried out by means of extracorporeal circulation. The patient improved dramatically after operation. Postoperative pulmonary arteriogram revealed a wide patent left pulmonary artery, and postoperative perfusion lung scan confirmed improved perfusion of the left lung. It is concluded that pulmonary artery sling should be treated surgically as soon as diagnosed. From the surgical point of view, the left pulmonary artery should be anastomosed to the main pulmonary artery with micro-surgical technique to avoid late occlusion of the left pulmonary artery.     

Pulmonary artery sarcoma mimicking pulmonary thromboembolism

A 30 years old male patient was referred to our hospital with a diagnosis of pulmonary thromboembolism due to thorax-computerized tomography (CT) angiography, revealing a thrombus totally occluding left main pulmonary artery. The lesion was evaluated as tumoural mass. Positron emission tomography (PET)-CT revealed pathologic uptake at pulmonary artery mass. Due to localization of tumour, left pneumonectomy was performed. The pathological diagnosis revealed to be pulmonary artery sarcoma. The patient was presented because pulmonary artery sarcomas are very rare tumors and can mimick pulmonary thromboembolism. The true prevalence is underestimated as many pulmonary artery sarcomas are misdiagnosed as pulmonary thromboembolism. PET-CT may help to make a differential diagnosis.     

Primary pulmonary valve sarcoma involving pulmonary artery and right ventricular outflow tract

The group of pulmonary valve and pulmonary artery primary tumors is the most frequently represented by schwannomas, myxomas, papillary fibroelastomas, primary choriocarcinomas and sarcomas. These tumors are very rare. The most common clinical manifestation of pulmonary artery tumor is dyspnea, followed by chest pain, cough, and haemoptysis.A case of 44-year-old male with history of progressive dyspnea, fever, cough, and weight loss is presented. Imaging methods showed large saddle embolus in the right ventricle outflow tract, pulmonary valve and pulmonary artery trunk. He was admitted to a hospital for anticoagulation therapy. Since there was no clinical improvement, the patient had to undergo surgery. Nearly full artery caliber filling tumor in pulmonary artery was found with its origin in pulmonary valve. Next exploration showed several little tumors in right ventricle outflow tract and also in pulmonary artery. The final outcome of histological examination showed the presence of leiomyosarcoma high grade 3.Presented case highlights that pulmonary artery tumor should always be included in the differential diagnosis of pulmonary embolism, especially if the symptoms progress while on adequate anticoagulation, and if any risk factor for deep vein thrombosis is not present. Unfortunately, prognosis of pulmonary artery sarcomas is usually dismal, secondary to late diagnosis.     

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.     

A successful surgical repair of pulmonary stenosis caused by isolated pulmonary Takayasu’s arteritis

A 48-year-old man underwent surgery to treat pulmonary stenoses of unknown origin. The right main pulmonary artery was severely stenotic, and the orifice of the left pulmonary artery was focally constricted. The patient was nearly asymptomatic, despite marked pulmonary hypertension and right heart strain. The pressure gradients beyond the stenotic regions were 88 mmHg bilaterally. The cause of the stenoses could not be established before operation. The right main pulmonary artery and ascending aorta were successfully replaced. It was suspected that the ascending aorta was involved because it was markedly thickened and adhered tightly to the right pulmonary artery. It was therefore also replaced. However, only the transected right pulmonary artery showed histological evidence consistent with a diagnosis of Takayasu’s arteritis. The postoperative course was uneventful. The patient was completely free of symptoms six months after the operation. To our knowledge, this is the thirteenth surgically treated case of isolated pulmonary Takayasu’s arteritis to be reported.     

Right pulmonary artery obstruction and pulmonary hypertension secondary to aortitis syndrome

A 56-year-old woman with aortic arch syndrome and finally right pulmonary artery obstruction secondary to Takayasu's aortitis was presented. She had had a history of visual disturbance and dizziness when she looked upward since 1983. On admission in July, 1984, aortography showed obstruction of the right innominate artery and of the left subclavian artery. Pulmonary arterial pressure, pulmonary perfusion and ventilation images seemed to be normal at that time. After discharge from our hospital, she began in 1987, to be aware of dyspnea on effort. Because of this symptom, she was admitted again in March, 1988. The pulmonary perfusion images showed complete lack of perfusion in the right lung, and arterial blood gas showed hypoxia with 62 mmHg in PaO2, 39 mmHg in PaCO2. Cardiac catheterization confirmed pulmonary hypertension with pulmonary artery pressure of 56/18 mmHg. In conclusion, pulmonary perfusion and ventilation scintigraphy proved to be the best way to clarify the nature of a lesion of the pulmonary artery in aortitis syndrome.     

Idiopathic right pulmonary artery aneurysm with pulmonary valve insufficiency

A case of idiopathic right pulmonary artery aneurysm with pulmonary valve insufficiency simulating a mediastinal teratoma occurred in an asymptomatic 13-year-old boy. The key to correct diagnosis was pulmonary angiography. The patient was successfully treated with surgery. © 1996 Wiley-Liss, Inc.     

Two-dimensional echocardiographic diagnosis of pulmonary artery sling in infancy

The vascular anomaly in which the left pulmonary artery arises from the right pulmonary artery and passes posteriorly and leftward between the trachea and the esophagus is termed a pulmonary artery sling. Two-dimensional echocardiograms were performed in five infants with this anomaly and successfully identified it in four, including one patient with truncus arteriosus communis. The subxiphoid long-axis sweep was useful in identifying the origin and initial course of the left pulmonary artery, and short-axis subxiphoid views showed both its origin from the right pulmonary artery and its initial posterior course. Angulation toward the cardiac apex displayed the right pulmonary artery in cross section anteriorly and the left pulmonary artery in cross section posteriorly. A transducer orientation midway between the subxiphoid long- and short-axis positions was helpful in distinguishing a large right upper lobe branch of the right pulmonary artery from a pulmonary artery sling. The precordial short-axis plane displayed the origin and initial posterior and leftward course of the left pulmonary artery, while the bifurcation of the main pulmonary artery, usually easily seen in this view, could not be demonstrated. Two-dimensional echocardiography offers a rapid, noninvasive diagnosis of pulmonary artery sling in infants.     

Dissecting aneurysm of the pulmonary artery with pulmonary hypertension

Pulmonary artery dissection was observed in a 64-yr-old female patient with severe pulmonary hypertension, which was probably primary (pulmonary vascular resistance, 817 dyn.s.cm-5; normal range less than or equal to 200 dyn.s.cm-5). The patient was admitted to the hospital because of severe dyspnea on exertion. Echocardiography demonstrated a dissecting aneurysm of the pulmonary artery. Right heart catheterization revealed severe pulmonary hypertension (mean pulmonary artery pressure, 64 mm Hg; normal range, 10 to 22 mm Hg); dissection of the pulmonary artery was confirmed by pulmonary arteriography. One-year follow-up was uneventful. In the literature, 28 patients with dissecting aneurysm of the pulmonary artery are reviewed. The dissection has only been diagnosed in life in one patient (by echocardiography).     

The ductus arteriosus and stenoses of the pulmonary arteries in pulmonary atresia

Forty-one specimens with pulmonary atresia and confluent pulmonary arteries were studied to establish the frequency and the nature of anomalies of both the ductus arteriosus and the central pulmonary arteries and their possible interrelation. The specimens were divided according to the type of pulmonary atresia: valvar atresia (12 cases) versus "muscular" atresia of the pulmonary orifice (29 cases). Abnormal connexions between the duct and the adjacent great arteries, as well as stenosis of a pulmonary artery, were common in cases with atresia of the pulmonary orifice and relatively rare in cases with imperforate valve. The abnormal connexions consisted of a proximal displacement of the origin of the duct from the aorta and displacement of the entry site of the duct into the pulmonary trunk towards one of the main pulmonary arteries. A left duct thus connected to the left and a right duct to the right pulmonary artery. Pulmonary artery stenosis, exclusively occurring in those specimens with a duct connecting to a pulmonary artery, were located in the segment of pulmonary artery between the pulmonary trunk and the duct. Histological examination revealed ductal tissue in the wall of a pulmonary artery in more than half of the cases with atresia of the pulmonary orifice. Ductal tissue in a pulmonary artery and pulmonary artery stenosis frequently coexisted. It is concluded that stenoses in the central pulmonary arteries may be related to the duct. The relationship resembles that between the duct and coarctation of the aorta.     

Isolated idiopathic pulmonary artery aneurysm

Aneurysm formation of the main pulmonary artery is rare. Its natural history is not well understood and there are no clear guidelines on optimal treatment. A 20-year-old woman with a huge saccular aneurysm of the main pulmonary artery, underwent repair with a pericardial patch and concomitant reconstruction of the pulmonary valve. The patient was doing well on follow-up at 6 months; echocardiography revealed a good repair with mild to moderate pulmonary regurgitation.     

Variant of tetralogy of fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta

Absent pulmonary valve leaflets and anomalous origin of a pulmonary artery from the ascending aorta have been reported as separate lesions associated with tetralogy of Fallot but not in combination. The clinical, radiologic and pathologic findings are presented in three patients with this previously undescribed combination. A search of the literature for similar cases revealed 233 patients with absent pulmonary valve leaflets and 56 patients with anomalous origin of a pulmonary artery from the ascending aorta; these cases are reviewed. Although 17 patients with absent pulmonary valve leaflets and tetralogy of Fallot also had absence of the proximal portion of the left pulmonary artery, none had an anomalously arising pulmonary artery from the ascending aorta. In the reviewed cases, anomalous origin of the right pulmonary artery from the ascending aorta was more frequent than that of the left pulmonary artery (44 compared with 12 cases, respectively), but the latter was more often associated with tetralogy of Fallot.     

Effects of pulmonary artery remodeling on pulmonary circulation after lung volume reduction surgery

BACKGROUND: Significant pulmonary hypertension during exercise has been observed in patients with severe chronic obstructive pulmonary disease. Although favorable effects on pulmonary function and dyspnea symptoms have been demonstrated, the influence of lung volume reduction surgery (LVRS) on the pulmonary hypertension during exercise is still a controversial subject. METHODS: A pulmonary function test and 6-minute walking test were performed before and 3, 6, 12 and 24 months after LVRS (n = 12). Pulmonary hemodynamics at rest and during exercise was studied 6 months after operation. Morphology was examined in pulmonary arteries with external diameters of 100 - 200 micro m in the resected lung, and the wall thickness (defined as intima plus media) and percentage wall thickness (percentage wall thickness of the external diameter) of the pulmonary artery were calculated. RESULTS: LVRS improved early-phase pulmonary function and 6-minute walking distance. Although the increase in pulmonary capillary wedge pressure during exercise was significantly ameliorated, exercise pulmonary hypertension did not change after LVRS. The percent wall thickness was highly correlated with Delta Ppa (difference between mean pulmonary artery pressure at rest and mean pulmonary artery pressure during exercise) not only before, but also 6 months after LVRS. CONCLUSION: LVRS has no significant influence on exercise-induced pulmonary hypertension in patients with severe emphysema. From a histological analysis of the pulmonary artery in the resected lung, remodeling the pulmonary artery that may exist in the remaining lung is possibly one of the important factors preventing postoperative improvement in exercise pulmonary hypertension in patients with chronic obstructive pulmonary disease.     

Effect of coexistent coarctation of pulmonary trunk in natural history of complete absence of pulmonary valve with ventricular septal defect.

A case of complete absence of the pulmonary valve in association with a ventricular septal defect and a pulmonary artery coarctation is described. Despite these defects the patient had minimal symptoms, probably because the pulmonary artery coarctation acted as a natural banding and limited the pulmonary regurgitant flow and reduced the left-to-right shunt across the ventricular septal defect. The patient was operated on at the age of 40 and the ventricular septal defect was closed but the pulmonary artery coarctation was left alone. In view of the long-term survival in this case, it is suggested that pulmonary artery banding would reduce the symptoms and increase the life expectancy of patients with congenital absence of the pulmonary valve and a ventricular septal defect.     

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.     

Surgical management of absent right pulmonary artery with associated pulmonary hypertension

A critically ill 21-month-old girl with congenital absence of the right pulmonary artery and severe pulmonary hypertension and congestive heart failure underwent conduit restoration of flow to the right lung. This patient had marked clinical improvement and reduction in pulmonary hypertension and represents the second reported surgically-treated case for absent right pulmonary artery. Reestablishment of pulmonary artery continuity is therefore recommended when cardiac failure and pulmonary hypertension occurs.     

Effect of coexistent coarctation of pulmonary trunk in natural history of complete absence of pulmonary valve with ventricular septal defect.

A case of complete absence of the pulmonary valve in association with a ventricular septal defect and a pulmonary artery coarctation is described. Despite these defects the patient had minimal symptoms, probably because the pulmonary artery coarctation acted as a natural banding and limited the pulmonary regurgitant flow and reduced the left-to-right shunt across the ventricular septal defect. The patient was operated on at the age of 40 and the ventricular septal defect was closed but the pulmonary artery coarctation was left alone. In view of the long-term survival in this case, it is suggested that pulmonary artery banding would reduce the symptoms and increase the life expectancy of patients with congenital absence of the pulmonary valve and a ventricular septal defect.     

A case of preoperatively diagnosed primary pulmonary leiomyosarcoma]

A 29-year-old woman had been suffering from right back pain for 3 months. Chronic pulmonary thromboembolism was suspected and she was referred to our hospital. She presented with no risk factors for thromboembolism, and during the previous 6 months had lost 4 kg in body weight. Chest radiography showed nodular shadows in the lower field of the right lung. Contrast-enhanced computed tomography demonstrated a filling defect in the right pulmonary artery and nodular lesions in the lower field of the right lung, which were considered to be signs of pulmonary infarction. Absence of perfusion into the right lung was demonstrated by a perfusion scan. Right heart catheterization showed normal pressure in the pulmonary arteries, and pulmonary angiography showed an abrupt cutoff of the right pulmonary artery, which was similar to the finding of pulmonary thromboembolism. A transvenous catheter suction biopsy was performed in the right pulmonary artery and the histopathologic findings yielded a diagnosis of leiomyosarcoma. The patient underwent surgical resection under total cardiopulmonary bypass. A large tumor completely filled the right main pulmonary artery and invaded the posterior wall of the pulmonary trunk close to the left main pulmonary artery. Primary pulmonary leiomyosarcoma is a rare tumor and its prognosis is very poor. Radical surgical resection is the only effective treatment, but early diagnosis is very difficult. Transvenous catheter suction biopsy is a useful procedure for the early diagnosis of pulmonary artery sarcoma.