Pulmonary thromboendarterectomy.

Pulmonary thromboendarterectomy (PTE) provides a curative alternative to the otherwise fatal condition of chronic thromboembolic pulmonary hypertension (CTEPH). However, the condition is under-diagnosed due to a lack of awareness. An acceptable operative mortality of around 10% and long-term survival exceeding medical therapy or transplantation makes PTE a favourable choice for the treatment of CTEPH. Outcome is further optimised if the disease is diagnosed early and patients referred to specialised centres. An increase in the number of surgical procedures will also contribute to lower the mortality associated with this condition.     

Pulmonary hamartoma.

Eighty-nine cases of pulmonary hamartoma were studied. There were 51 men and 38 women, with a mean age of 57.5 years (range 14 to 76 years). A histologic diagnosis from examination of the resection specimens was obtained in all patients who had operations. Moreover, transthoracic needle aspiration biopsies were performed in 40 patients, with a diagnostic result in 34 (85%). The hamartomas were equally distributed in the pulmonary lobes; mean transverse diameter at the time of diagnosis was 21.7 +/- 16.2 mm. Tumor size was independent of the anatomic localization, but it correlated with the age of the patients (p less than 0.01). Tumor growth was recorded in 15 of 31 patients who had follow-up (45%); mean expansion in transverse diameter was 3.2 +/- 2.6 mm per year during an average observation time of 4.1 years (range 1 to 20 years). Pulmonary symptoms were present in 35 patients (39%). Seventy-five patients underwent operations as follows: enucleation (54), resection (11), lobectomy (5), pneumonectomy (4), and bronchoscopic removal (1). Since most pulmonary hamartomas are nonexpanding or slowly growing neoplasms, it is concluded that operation is necessary only when expansion is recorded in young or middle-aged patients and in patients with pulmonary symptoms.     

Pulmonary lymphangioleiomyomatosis.

We report a case of lymphangioleiomyomatosis (LAM) in a 33-year-old woman. Left pneumothorax was noted during a physical checkup at a local clinic. Multiple bullae in the bilateral lungs were also seen on chest computed tomography. Therefore, she underwent surgery for diagnosis and therapy. A large number of cysts of various sizes were seen in the left pleural cavity, and a cyst located at the lingular region was resected. Histopathological examination of the cyst walls showed a thin rim of abnormal muscle consisting of spindle-shaped cells resembling smooth muscle cells as well as thick, eccentric, or concentric proliferation. The examination described above confirmed the diagnosis of LAM. The patient is currently being followed up on an outpatient basis.     

Pulmonary varix.

Pulmonary varix is a rare disorder which is usually discovered by change during the third to sixth decade in an asymptomatic patient. The 37th example is reported with a review of the literature. The disorder is possibly congenital and may affect any lobe. Pulmonary angiography is the preferred procedure for diagnosis. If symptoms are present, they can usually attributed to associated cardiopulmonary disease. Two serious complications have been reported--systematic embolus from a clot in the varix (two cases suspected), and rupture leading to the death of the patient (four cases). A third hazard to the patient is an unnecessary diagnostic thoracotomy. Patients without symptoms should have periodic chest radiographs of those with haemoptysis or systemic embolism should be considered for resection of the varix.     

Pulmonary lymphangiomyomatosis.

Pulmonary lymphangiomyomatosis is a rare but distinct clinical and pathological entity. It is characterized by hamartomatous proliferation of smooth muscle around the lymphatic vessels of the lung, mediastinum, and retroperitoneum. It occurs only in menstruating women and girls and is manifested by spontaneous pneumothorax and chylous pleural or abdominal effusion. As it progresses, the lungs become increasingly involved with subsequent pulmonary insufficiency and recurrent infection. Eventually death results. This entity has not been reported previously in the surgical literature even though the thoracic surgeon is called on to both establish the diagnosis and aid in palliation. The hypothesis that the disease is estrogen dependent is reviewed and the desirability of estrogen ablation in patients with positive estrogen receptors is suggested.     

Pulmonary transplantation.

OBJECTIVE: More than 2700 lung transplants have been performed since the initial clinical success in 1983. The evolution in the techniques of lung transplantation and patient management and the effects on results are reviewed. SUMMARY BACKGROUND DATA: Improvements in donor management, lung preservation, operative techniques, immunosuppression management, infection prophylaxis and treatment, rejection surveillance, and long-term follow-up have occurred in the decade following the first clinically successful lung transplant. A wider spectrum of diseases and patients treated with lung transplant have accentuated the shortage of suitable lung donors. The organ shortage has led to the use of marginal donors and a limited experience using living, related donors. METHODS: Changes in techniques and patient selection and management are reviewed and controversial issues and problems are highlighted. RESULTS: One-year survival of greater than 90% for single-lung transplant recipients and greater than 85% for bilateral lung transplant recipients have been achieved. Complications caused by airway complications has been reduced greatly. Obliterative bronchiolitis develops in 20% to 50% of long-term survivors and is the leading cause of morbidity and mortality after the first year after transplant. CONCLUSIONS: Lung transplantation has evolved into an effective therapy for a wide variety of causes of end-stage lung disease. Wider applicability requires solutions to the problems of donor shortage and development of obliterative bronchiolitis.     

Pulmonary sequestration.

Pulmonary sequestration is a congenital anomaly in which an aberrant systemic artery arising from the thoracic or upper abdominal aorta supplies part of the lungs, usually the lower lobe. The sequestered lung may be anatomically distinct from the remainder of the lobe (extralobar), or may be included in the substance of the lobe, in which case it may or may not have bronchial communication with the rest of the bronchial tree. The patients present, often in the first two decades of life, with recurrent and severe bronchopulmonary infections. Associated anomalies are present, especially in the extralobar variety. Nine cases of sequestration are reviewed, stressing significant clinical, radiological, and arteriographic findings. Preoperative demonstration of the anomalous vessel by aortography has contributed significantly to the planning and safety of the surgical procedure, which was generally a lower lobectomy. Eight out of nine patients survived the procedure.     

Pulmonary calcinosis.

The clinical and pathological features are described of a case of pulmonary calcinosis complicating cystic disease of the renal medulla. A histopathological study of the lung revealed calcification in the alveolar walls and in the blood vessels, predominantly in the pulmonary veins and venules. The calcified deposits were also studied by electron microscopy, and appearances suggestive of active growth of the deposits were recognised. Chemical analysis of the lung revealed a calcium content some 55 times greater than that of a normal lung. There was a five-fold increase in magnesium content. Reference is made to the literature demonstrating that the chemical composition of metastic calcification differs according to whether it is visceral or non-visceral in type.     

Pulmonary blastoma.

Pulmonary blastoma is now accepted as a distinctive neoplasm. It remains rare, and only 28 cases have been reliably recorded. A further two cases are now reported, and the previous literature is reviewed. There are no specific clinical or radiological features of pulmonary blastoma. The presentation can be that of any other pulmonary tumour although a peripheral situation is usual and a large size is often attained before detection. Pulmonary blastoma is a mixed tumour with malignant epithelial and connective tissue components with a distinctive resemblance to fetal lung. The treatment of choice is surgical excision but the overall prognosis is poor. It is doubtful whether the tumour has a true blastomatous origin.     

Pulmonary artery sling.

Eighteen patients who underwent surgical treatment of pulmonary artery sling at this institution since 1969 were reviewed. Four of them had associated tracheal stenoses produced by complete cartilaginous rings (stovepipe trachea). All patients underwent operation. The median age at operation was 180 days (range, 27 days to 54 months). In the 14 patients with isolated pulmonary sling, the operation consisted of division of left pulmonary artery and reimplantation into the main pulmonary artery anterior to the trachea. In the 4 patients with stovepipe trachea, the stenotic tracheal segment was resected on cardiopulmonary bypass and the left pulmonary artery was brought anterior to the trachea before the latter was reanastomosed to the main pulmonary artery. There were no early deaths. One patient died late. The pulmonary artery anastomosis was patent in all 14 patients investigated postoperatively. Three patients have residual tracheobronchial problems. One patient is mentally retarded and is institutionalized. All other patients are symptom-free and growing normally and have normal chest radiographs.     

Pulmonary reperfusion syndrome.

"Reperfusion syndrome" of the lung may play a role in the pulmonary edema and hemorrhage that occur following pulmonary embolectomy, cardiopulmonary bypass, and shock. Bioenergetic, metabolic, and ultrastructural studies of canine lungs indicate that ventilated lung tissue could tolerate 5 hours of pulmonary arterial occlusion with minimal damage. However, a 24-hour interruption of pulmonary arterial blood flow produced a significant decrease in the ratio of adenosine triphosphate to adenosine disphosphate, and glycogen, and an increase in tissue lactate. Reperfusion of these lungs resulted in even more pronounced biochemical and ultrastructural deterioration, as well as gross pulmonary edema and hemorrhage. The lesion appears to be similar to the reperfusion damage that occurs in other organs, such as the kidney, and the skeletal and cardiac muscles.