Comparative studies on pulmonary hypertension in lung and heart disease

Correlation between the histological changes occurring in the pulmonary blood vessels and in the alveolar wall and the cardiopulmonary hemodynamics was investigated in pulmonary hypertension due to chronic lung disease as well as to heart disease in special reference to its reversibility. The ratio of pulmonary to systemic resistance, cardiac index, the histological changes in pulmonary blood vessels and secondary changes in alveolar wall seem to be important factor of veversibility of the lesion, and should be carefully evaluated before the indication for heart and lung surgery in severe cases is made.     

门静脉和下腔静脉阻断与开放后门静脉高压犬肺动脉和肺组织超微结构改变

目的观察门静脉和下腔静脉阻断与开放后门静脉高压模型犬肺组织与肺动脉的超微结构改变。方法正常家犬18只,随机均分为阴性对照组（仅用于取肺组织和肺动脉标本）、对照组和门静脉高压模型组（采用部分结扎门静脉的方法建立患门静脉高压症犬模型并饲养12周）,进行门静脉、肝上和肝下下腔静脉阻断与开放实验。在术毕后取右下肺动脉和肺组织,应用透射电镜观察其超微结构。结果门静脉和下腔静脉阻断与开放后门静脉高压犬肺组织内皮细胞、Ⅰ型肺泡上皮细胞和Ⅱ型肺泡上皮均出现明显的病理性改变;肺泡隔纤维化明显,胶原纤维增生;肺毛细血管内血栓形成,基膜明显增厚;肺动脉血管内皮细胞损伤明显,部分脱落;肺动脉内皮细胞与中膜间隙明显增宽,内皮下水肿,内皮下胶原纤维增多。结论伴门静脉高压的肝移植犬肺组织和肺动脉的超微结构有明显改变,这可能是肝移植受者围术期出现急性肺高压和急性肺损伤的超微病理学基础之一。     

慢肺动脉栓塞的外科治疗现状

肺动脉栓塞(pulmonary thromboembolism, PTE)发病率、死亡率高,临床上漏诊、误诊严重,少数患者发展为慢性肺动脉栓塞.慢性肺动脉栓塞所致的肺动脉高压内科治疗效果不佳、预后不良,经过近2个世纪的逐步认识及临床实践,认识到体外循环低流量或间断停循环下进行肺动脉血栓内膜剥脱术是治疗该病的有效手段.术后肺动脉高压、再灌注肺水肿仍是主要并发症和手术死亡的原因,术后积极有效地防治肺动脉高压、再灌注后肺水肿和积极抗凝,预防再次栓塞是外科治疗慢性肺动脉栓塞成功的关键.     

Unilateral pulmonary venous obstruction: a case report

A 3-month-old girl with right common pulmonary venous obstruction and severe pulmonary hypertension underwent pulmonary venous reconstruction (venoplasty). However, pulmonary hypertension persisted and she died on the 69th postoperative day. Autopsy revealed a widely opened pulmonary vein at the site of the venoplasty. Histological examination showed thickened alveolar walls, thromboembolism in pulmonary arterioles and venous hypoplasia in the right lung. The venoplasty for unilateral pulmonary venous obstruction with severe pulmonary hypertension may not be the proper treatment, since pulmonary hypertension usually continues or progresses postoperatively. Pneumonectomy may be a better choice to be applied.     

Pulmonary thromboembolectomy in congenital antithrombin III deficiency associated with acute pulmonary embolism -report of a case-.

Antithrombin III (AT III) deficiency is a rare hereditary disease that predisposes to thromboembolic complications. We report a case of AT III deficiency complicated with acute pulmonary thromboembolism, successfully treated with emergency pulmonary thromboembolectomy after insertion of an inferior vena cava filter. AT III activity before treatment was found to be 44% of normal value and remained less than 50% of normal throughout the postoperative course. In his family line, both the patient's aunt and deceased father had a history of pulmonary infarction. AT III activity of the patient's aunt was 47 to 58% of normal value. The patient was discharged on the 15th day after surgery and has been doing well for four years receiving warfarin as anticoagulant therapy. Careful follow-up is essential for early detection of the recurrent pulmonary thromboembolism resulting in pulmonary hypertension and/or right heart failure.     

Pulmonary thromboendarterectomy for chronic thromboembolic obstruction of the pulmonary artery in piglets.

OBJECTIVE: The 2 main causes of death after thromboendarterectomy for chronic pulmonary thromboembolism are incomplete repermeabilization responsible for persistent pulmonary hypertension and acute high-permeability pulmonary edema. We wish to establish an experimental model of chronic pulmonary thromboembolism to replicate the conditions encountered during and after pulmonary thromboendarterectomy. METHODS: Multiple-curled coils and tissue adhesive were embolized in 6 piglets to induce complete obstruction of the left pulmonary artery, documented by angiography. After 5 weeks, the main pulmonary artery was repermeabilized by thromboendarterectomy during circulatory arrest. The left lung was reperfused ex vivo with autologous blood at constant flow, and patency of the pulmonary artery was evaluated on a barium angiogram. The endarterectomy-reperfusion procedure was also done in 6 nonembolized piglets that served as the controls. The severity of lung injury induced by 60 minutes of reperfusion was assessed on the basis of measurements of the lung filtration coefficient and of lung myeloperoxidase activity. RESULTS: Marked hypertrophy of the bronchial circulation was seen in the chronic pulmonary thromboembolism group. Thromboendarterectomy removed the organized obstructing thrombus that was incorporated into the arterial wall and restored patency of the pulmonary artery. Acute lung inflammation and high-permeability edema occurred after reperfusion, as indicated by a 1.5-fold increases in both lung filtration coefficient and lung myeloperoxidase values in the chronic pulmonary thromboembolism group; these 2 variables being correlated. CONCLUSIONS: Our model replicated the perioperative conditions of pulmonary thromboendarterectomy, suggesting that it may prove useful for improving the repermeabilization technique and for investigating the mechanisms and prevention of reperfusion injury.     

Diffuse pulmonary hemorrhage. An uncommon manifestation of Wegener's granulomatosis

We report two cases of Wegener's granulomatosis with the unusual manifestation of diffuse alveolar hemorrahge. One patient with well-documented Wegener's granulomatosis developed alveolar hemorrhage 4 weeks after leukopenia necessitated the discontinuation of cyclophosphamide. The second patient presented with pulmonary hemorrhage and died 10 days after an open-lung biopsy in which histologic features of Wegener's granulomatosis were overshadowed by alveolar hemorrhage. Lung biopsies in both cases showed marked alveolar hemorrhage and pulmonary capillaritis. The importance of recognizing capillaritis and other subtle histologic features of Wegener's granulomatosis are emphasized.     

Acute pulmonary thromboembolism after coronary artery bypass grafting]

The cases of acute pulmonary thromboembolism after coronary artery bypass surgery have been rarely reported in Japan. We, however, experienced 2 cases of them and lost them against intensive therapy. In Mitsui Memorial Hospital, 6 cases of pulmonary thromboembolism had been diagnosed in 1990 (0.074% in admission patients), four of the cases had occurred following surgical procedures. Its manifestations are deceivingly nonspecific and are difficult to distinguish perioperative myocardial infarction, pericardial tamponade, and pneumothorax, especially in postoperative states. We would like to emphasize that acute pulmonary thromboembolism is getting important problems also in Japan.     

The adult respiratory distress syndrome bronchogenic pulmonary tuberculosis

In three cases of pulmonary tuberculosis associated with the adult respiratory distress syndrome the clinical features, which were similar to those of patients with miliary tuberculosis and adult respiratory distress syndrome, included a history of cough, fever, and dyspnoea on effort, and the physical signs of fever, tachypnoea, pulmonary adventitious sounds, tachycardia, and hepatomegaly. In these cases the radiological features, though suggestive of diffuse pulmonary oedema, were more prominent on the side in which the cavitatory lesion appeared. The diagnosis of tuberculosis was made easily from direct examination of sputum. Despite early ventilatory support and antituberculous therapy, two of the three patients died. Postmortem examination of the lungs in these cases showed evidence of acute alveolar damage (loss of type 1 pneumocytes and the presence of hyaline membranes within alveolar ducts) and of chronic alveolar damage (interstitial and alveolar fibrosis).     

Two cases of chronic pulmonary thromboembolism saved by postoperative use of a percutaneous cardiopulmonary support device

We performed pulmonary thromboendarterectomy under deep hypothermic intermittent circulatory arrest in 18 patients with chronic pulmonary thromboembolism from August 2001 to January 2004. In some of these cases, reperfusion pulmonary edema prevented a satisfactory improvement in hemodynamic data soon after the surgery. Here we report two cases of chronic pulmonary thromboembolism in which we successfully prevented postoperative persistent pulmonary hypertension and hypoxia caused by severe reperfusion pulmonary edema by the use of a percutaneous cardiopulmonary support device.     

Pleural thickening and gas transfer in asbestosis

Anomalies in the ratio of transfer factor to effective alveolar volume as an indicator of pulmonary gas exchange in cases of asbestosis may be related to diffuse pleural thickening. To examine the effect of pleural disease on gas transfer the plain chest radiographs of patients with asbestosis were assessed by two observers for profusion of parenchymal opacities and extent of pleural disease and the results were related to lung function. In 30 cases of category 1 profusion of parenchymal abnormality (according to the ILO international classification of radiographs for pneumoconiosis) transfer factor was independent of the degree of pleural thickening. The ratio of transfer factor to effective alveolar volume correlated directly with the degree of pleural thickening as alveolar volume fell with increasing severity of pleural disease. The results indicate that correcting transfer factor for alveolar volume does not provide an accurate reflection of severity of diffuse parenchymal fibrosis in patients with asbestosis and even minor pleural disease.     

Negative-pressure acute tracheobronchial hemorrhage and pulmonary edema

Negative-pressure pulmonary edema is a well-known complication of an acute upper airway obstruction, which may rarely present as acute alveolar hemorrhage in cases of severe capillary stress failure. Hemorrhage from the central airways has also been reported as a rare manifestation of acute tracheobronchial injury, associated with severe disruption of the bronchial vasculature due to highly negative inspiratory pressure. In this clinical report, we describe a case of both acute tracheobronchial and alveolar hemorrhage in a young man, occurring immediately after extubation due to laryngospasm, diagnosed by bronchoscopy with bronchoalveolar lavage (BAL), measurement of the pulmonary edema fluid/plasma protein ratio, and by thoracic computed tomography (CT) scan. We propose that the patient experienced severe postobstructive negative-pressure pulmonary edema, related to increased permeability of the alveolar capillary membrane and a parallel loss of integrity of the bronchial vascular network. Our findings suggest that both changes in the bronchial circulation and mechanical stress failure of the more distal alveolar-capillary system may be induced by severe and acute upper-airway obstruction.     

Tridimensional photographic reconstruction in a study of the pathogenesis of honeycomb lung

Tridimensional photographic reconstruction of the lesions found in honeycomb lung in 10 different types of pulmonary disease was made. The pathological picture was characteristic and well defined by tridimensional microscopy and quite independent of the accompanying disease. The lesions responsible for its appearance involved the whole lobular bronchiolar system but were most marked in the terminal and respiratory bronchioli. The changes were fundamentally diffuse, saccular, and cystic bronchiolectasis. Other bronchiolar lesions were found such as changes in direction and mode of division, amputations, and anastomoses between bronchioles and cysts belonging to anatomically independent airways. There was some evidence that the bullous aspects of honeycomb lung were due to multiple valvular arrangements which let the air in but do not let it out. Honeycomb lung was, in the majority of cases, a complication of interstitial pulmonary fibrosis. Honeycombing was usually due to marked changes in the lobular bronchioles caused by the obliteration or rigidity of alveolar ducts and the corresponding alveoli and even by localization of the interstitial fibrosis in the bronchiolar wall.     

Pulmonary renal syndrome associated with Wegener’s granulomatosis: a case report and review of literature

Pulmonary renal syndromes are rare but serious complications of systemic vasculitis. The majority of these cases are related to ANCA-associated vasculitis. These syndromes represent a combination of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Here we report a case of 21-year-old female with a clinical picture of community-acquired pneumonia that rapidly evolved to pulmonary hemorrhage and acute renal failure. Combined pulse steroid treatment, immunosuppressive treatment, and plasmapheresis were instituted and resulted in the improvement of the patient. The diagnosis of Wegener’s granulomatosis was established based on the clinical and serological findings (positive cANCA). Here we review the relevant literature on pulmonary renal syndromes associated with Wegener’s granulomatosis and discuss their diverse clinicopathologic features.     

Changes of alveolar stability and phospholipids in pulmonary surfactant in acute pancreatitis

We investigated changes of alveolar stability and phospholipids in the pulmonary surfactant in case of acute pancreatitis induced in rats. Alveolar stability was examined by recording the pressure-volume relationship. The lung volumes during deflation decreased significantly at equivalent transpulmonary pressures, particularly when the pressure was lower than 6 cm H₂O. Bubble stability ratio and surface tension indicated that the surface activity of the pulmonary surfactant did decrease in the rats with acute pancreatitis. The alveolar phospholipid content decreased, and the lecithin fraction also decreased significantly, as compared to the control groups. The metabolism of alveolar lecithin was examined following intravenous administration of¹⁴C-labeled palmitate. The biological half-life of the radioactivity of alveolar lecithin was approximately 6 hours in the pancreatitis group, 12 hours in the hepatic ducts ligated group and 14 hours in the simple laparotomy group. The degradation of alveolar lecithin, as well as its synthesis, was accelerated in the rats with acute pancreatitis. However, a decrease in alveolar phospholipid, mainly in lecithin fraction, indicated that the synthesis was inadequate, to maintain normal levels and the impairment in pulmonary surfactant may result in a respiratory insufficiency. Presented at the 82nd Annual Meeting of the Japan Surgical Society, 1982, Chiba, Japan.     

Indications for and the results of pulmonary thromboendarterectomy for thromboembolic pulmonary hypertension

Most episodes of acute pulmonary embolism completely resolve, through thrombolytic mechanisms. When adequate thrombolysis fails, however, residual obstruction to pulmonary vascular flow by fibrotic remnants may result in pulmonary hypertension. The genesis of this pulmonary hypertension is complex. Apart from the simple mechanical obstruction to blood flow that may occur from occlusion of significant areas of the pulmonary vascular bed, secondary vascular changes eventually may result from increased flow and pressure in nonobstructed areas of the lung, or factors generated by localized hypoxia in occluded segments. Chronic pulmonary hypertension from thromboembolism, regardless of the mechanism, is much more prevalent than is generally appreciated. Patients develop increasing shortness of breath, and the diagnosis is often missed. Pulmonary endarterectomy offers a cure for the condition, and wider recognition of the efficacy of the operation and the entity are therefore important. Pulmonary endarterectomy is a technically demanding procedure, now performed with success at only a handful of centers. However, with proper patient selection, meticulous surgical technique, and careful postoperative management, excellent results can be obtained. A true endarterectomy (not an embolectomy) of all affected parts of the lung is performed, and cardiopulmonary bypass, systemic cooling, and circulatory arrest are essential for the visibility necessary to clear all affected areas of the pulmonary vasculature. Medical treatment is ineffective for this condition, and the alternative surgical treatment is lung transplantation. Pulmonary endarterectomy appears to be permanently curative, though an inferior vena caval filter should be placed in all patients to prevent recurrence, and the patients must have life-long anticoagulation.     

Pulmonary sarcoidosis with an alveolar radiographic pattern.

Thirty-three cases of sarcoidosis (4.4% of 746 patients) showed an alveolar radiological pattern. A study of pulmonary function was carried out in 25 patients and compared with that of 46 patients with the interstitial radiological type of sarcoidosis. Twenty-two cases have been followed up from one to six years after the initial examination. The radiographic lesions were most often bilateral and included nodules greater than 15 mm with ill-defined margins or diffuse, infiltrative, non-retractile opacities with fluffy margins. Bilateral mediastinal lymph nodes were present in 27 patients. In 20 patients an associated reticulation was found on radiography. In four patients an open lung biopsy was done. The granulomatous nodules were identical to those found in other forms of sarcoidosis, although they were more confluent in the affected areas. Clinical and functional findings did not differ from those in the more common forms of sarcoidosis. Alveolar sarcoidosis has a sudden course. The alveolar radiological patterns always disappeared, with or without steroid treatment, while reticular patterns persisted in four patients. Rapid radiological changes were observed. Some functional abnormalities persisted in cases that were followed. It is concluded that alveolar sarcoidosis is a distinct acute form of sarcoidosis.     

The tricuspid valve plasty in the surgical treatment of chronic pulmonary thromboembolism

Chronic pulmonary thromboembolism causes persistent rising of pressure in the pulmonary artery system. Durable existing of hypertension promotes the right ventricle dilatation together with tricuspid valve insufficiency occurrence, dramatizing the disease clinical course and prognosis. There were considered the surgical methods of the tricuspid cardiac valve insufficiency correction. The performance of the tricuspid valve plasty according to De Vega and using supporting ring of Carpentiere was proposed, basing on experience of surgical treatment of chronic pulmonary thromboembolism and postthrombotic pulmonary hypertension.     

Hemodynamic changes in pulmonary thromboembolism

Three variants of hemodynamic reaction to massive thromboembolism of the pulmonary artery are encountered--hyperkinetic, eukinetic, and hypokinetic. Their manifestations are determined by the patient's cardiovascular status at the onset of thromboembolism, the volume of the embolic obstruction, the degree of pulmonary hypertension, and the concomitant cardiopulmonary pathology. Effective treatment leading to marked reduction of pulmonary hypertension is conducive to increase in the proportion of the hyperkinetic type; ineffective thrombolysis, thrombosis of the inferior vena cava antiembolic filter, recurrent thromboembolism, and infarction pneumonia are the causes of diminished right-ventricular systolic function and reduced cardiac index, i. e. the formation of a hypokinetic type.     

Simultaneous occurrence of pulmonary interstitial fibrosis and alveolar cell carcinoma in one family.

The coexistence of interstitial pulmonary fibrosis and alveolar cell carcinoma is well known. The familial occurrence of a combination of these two entities, however, is very rare. We present a family of which five members had diffuse interstitial pulmonary fibrosis. Three of them had in addition alveolar cell carcinoma. In a sixth family member, evidence of alveolar cell carcinoma was present without proven interstitial fibrosis. An autosomal dominant trait is suggested as the mode of inheritance of both interstitial fibrosis and alveolar cell carcinoma in this family.