Pulmonary hypertension secondary to neurofibromatosis: intimal fibrosis versus thromboembolism

Neurofibromatosis has been known to involve blood vessels throughout the body. Pulmonary involvement with interstitial fibrosing alveolitis has been described but no case of pulmonary vascular involvement has been reported to date. A 51 year old patient with cutaneous neurofibromatosis is described who presented with severe pulmonary hypertension and radiographic, scintigraphic, and angiographic evidence of chronic thromboembolic pulmonary hypertension. Severe intimal fibrosis consistent with vascular involvement with neurofibromatosis was found on endarterectomy with no evidence of pulmonary thromboembolism. Neurofibromatosis of pulmonary arteries should be considered as a possible cause of pulmonary hypertension.     

Partial anomalous connection of both superior pulmonary veins

Several patterns of anomalous pulmonary venous drainage have been described in the literature, and bilateral partial pulmonary anomalous vein connection (PAPVC) has been described as a rare congenital cardiac anomaly. We report an unusual type of bilateral PAPVC, involving both the superior right and left pulmonary veins draining into the left brachiocephalic vein in a young adult who was symptomatic with dyspnea and a dry cough.     

Pulmonary edema following pulmonary valvulotomy.

A previously unreported combination of critical pulmonary valve stenosis and mitral stenosis is described. The initial clinical presentation was one of right ventricular failure that obscured the evidence of pulmonary venous hypertension. Following pulmonary valvulotomy, pulmonary edema ensued because of the increased pulmonary blood flow. The importance of urgent cardiac catheterization postoperatively following an operation that increases pulmonary blood flow is discussed.     

Nodular pulmonary amyloidosis occurring in association with pulmonary lymphoma

A rare association between primary pulmonary lymphoma and pulmonary nodular amyloidosis in a 56 year old man is described.     

Improved forceps for pulmonary embolectomy

A malleable, nonocclusive forceps for surgical extraction of fresh pulmonary emboli is described. This device allows the performance of pulmonary embolectomy under direct vision with an unobscured peripheral field and reduces the potential for traumatic pulmonary artery laceration.     

Epithelioid haemangioendothelioma

Epithelioid haemangioendothelioma is a rare pulmonary neoplasm with less than 40 cases described world wide. We describe the only case to have presented with hypertrophic pulmonary osteoarthropathy who has been treated with azathioprine and has remained alive and well with no deterioration in pulmonary function since being diagnosed 16 years ago. The progression of the chest radiograph and spiral CT appearances of this rare neoplasm are described, and current views regarding the cellular origin of the neoplasm, its cytological appearance, clinical presentation and prognosis are discussed.     

Pulmonary endocrine cells in plexogenic pulmonary arteriopathy associated with cirrhosis.

A clear association has been described between numbers of pulmonary endocrine cells and the migration and/or proliferation of myofibroblasts which is thought to underlie the vascular changes seen in plexogenic pulmonary arteriopathy due to cardiac shunts and primary pulmonary hypertension. In contrast, the pulmonary endocrine system in a subject with florid pulmonary plexogenic arteriopathy associated with cirrhosis was entirely normal, suggesting possible differences in its pathogenesis.     

Simplified technique for the surgical palliation of pulmonary atresia with right ventricular hypoplasia and intact septum

A rapid, simple palliative technique for treatment of pulmonary atresia with a one-stage pulmonary valvotomy and central aortopulmonary artery shunt is described.     

多排螺旋CT对于肺栓塞的诊断

多排CT(MSCT)已替代核医学及经导管肺动脉造影成为肺栓塞的首要检查技术。急性肺栓塞的MSCT诊断是依据血管内充盈缺损及血管完全阻塞。本文介绍肺栓塞的MSCT血管成像的检查方法和对于肺栓塞的诊断价值，并讨论MSCT在肺栓塞影像诊断中的优势。     

Klippel-Trenaunay syndrome with small vessel pulmonary arterial hypertension

A patient with Klippel-Trenaunay syndrome and pulmonary arterial hypertension not associated with chronic thrombo-embolic pulmonary hypertension is described. It is hypothesised that pulmonary arterial hypertension is another complication associated with the Klippel-Trenaunay syndrome, possibly due to haemodynamic changes of small vessel abnormalities.     

Simplified technique for pericardial patch of the right ventricular outflow tract

Pericardial patching to enlarge the right ventricular outflow tract is often required in congenital cardiac surgery in lesions such as tetralogy of Fallot, pulmonary atresia, pulmonary stenosis, and pulmonary artery repair following removal of a palliative pulmonary artery band. A method of simplifying the attachment of the pericardial patch onto the right ventricular outflow tract is described.     

Emergent pulmonary embolectomy: the treatment for massive pulmonary embolus

Massive pulmonary embolus usually leads to in-hospital mortality if not treated aggressively. Four patients were seen with severe cardiorespiratory compromise resulting from massive pulmonary emboli. Emergent pulmonary embolectomy was followed by marked clinical improvement, and 3 patients were subsequently discharged from the hospital. The clinical courses of these patients are described, and massive pulmonary embolus and its management are discussed.     

Acute pulmonary oedema. A complication of upper airway obstruction

A case of postoperative laryngeal spasm is presented in which severe pulmonary oedema developed after the laryngeal spasm was relieved. The management is discussed and some suggestions for prevention of pulmonary oedema in similar circumstances made. The pathophysiology of pulmonary oedema after acute upper airway obstruction is described.     

Peripartum cardiomyopathy

A case of peripartum cardiomyopathy that occurred together with pre-eclampsia is described. This gave rise to pulmonary oedema at 36 weeks' gestation. Intensive care management and the need for invasive monitoring, especially pulmonary artery wedge pressure, of patients who develop pulmonary oedema in the peripartum period is discussed.     

Total anomalous pulmonary venous connection to the supradiaphragmatic inferior vena cava

Total anomalous pulmonary venous connection to the inferior vena cava is a rare form of total anomalous pulmonary venous connection infrequently described in the literature. We report two cases where the pulmonary venous connection was to the supradiaphragmatic portion of the inferior vena cava. In both patients, preoperative echocardiography findings were misleading, which suggested a cardiac type of total anomalous pulmonary venous connection.     

Bicuspid pulmonary valve with atrial septal defect leading to pulmonary aneurysm

Pulmonary artery aneurysms are rare. We describe 2 adult patients with pulmonary artery aneurysm with normal pulmonary pressure associated with bicuspid pulmonary valve and atrial septal defect. One patient presented with moderate pulmonary valve stenosis and was treated with open surgery; the other patient had a small atrial septal defect and mild pulmonary valve insufficiency and is periodically still being evaluated. Hemodynamic alterations associated with a pulmonary artery aneurysm are described; the influence of additional volume overload and intrinsic wall abnormalities in pulmonary valvular lesions as potential triggers for the development of these aneurysms are analyzed and therapeutic strategies are discussed.     

Cystic lung in Marfan''s syndrome.

Various pulmonary problems have been described in Marfan's syndrome. Unusual cystic lung changes in a young girl with Marfan's syndrome are described.     

Malignant chondroid syringoma presenting as multiple pulmonary nodules

Malignant chondroid syringoma, a very rare tumour, presenting with multiple pulmonary metastases in a 50 year old woman is described. Initial diagnostic confusion with pulmonary hamartoma occurred due to histopathological similarities. However, re-examination of a skin biopsy specimen taken 17 years previously from a hand lesion yielded the necessary information to identify the pulmonary lesions definitively as metastases from the original skin lesion. The features of this very rare indolent tumour are described.




     

Concomitant cor triatriatum and coronary sinus total anomalous pulmonary venous connection

Successful repair of the rare anomaly of concomitant cor triatriatum and coronary sinus total anomalous pulmonary venous connection in a neonate is described. The presence of pulmonary venous obstruction or cardiomegaly, or both, in a child with total anomalous pulmonary venous connection should alert to the possibility of a coexistent cor triatriatum.     

Pulmonary Valve Repair: An Option for an Emerging Problem

Abstract   In this report, we present our experience with successful pulmonary valve repair for pulmonary regurgitation in a 15-year-old patient who had undergone percutaneous pulmonary balloon valvuloplasty for pulmonary stenosis in the neonatal period. The pathological findings and the technique of repair are described in detail as the number of such patients is expected to increase in future. (J Card Surg 2010;25:87-89)