1/44. Post-Mustard procedure pulmonary venous obstruction: An opportunity for anatomic correction with a one-stage arterial switch.A 14-year-old boy after a Mustard procedure for transposition of the great arteries developed pulmonary hypertension secondary to baffle obstruction. This occurred over several years without apparent significant symptomatology. Systemic-level pressure prevailed in the left (pulmonary) ventricle and provided an opportunity to perform a successful one-stage arterial switch.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/44. pulmonary veno-occlusive disease in pulmonary Langerhans' cell granulomatosis.This report describes unusual clinical and pathological findings in a 29-yr-old female with pulmonary Langerhans' cell granulomatosis (LCG). During a 7-yr clinical course her condition deteriorated despite corticosteroid therapy, and she died of respiratory failure and pulmonary hypertension. At autopsy, there were widespread pulmonary veno-occlusive disease (PVOD) lesions as well as abundant advanced and healed lesions of pulmonary LCG composed of multiple cysts and stellate fibrosis. The present case demonstrates that pulmonary Langerhans' cell granulomatosis should be considered as a possible cause of pulmonary veno-occlusive disease.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
3/44. Successful arterial switch operation for post-Mustard pulmonary venous obstruction and secondary pulmonary hypertension.A 16-year-old girl presented with dyspnea 15 years after the Mustard operation for transposition of the great arteries with intact ventricular septum. An echocardiogram revealed secondary pulmonary hypertension due to pulmonary venous obstruction. cardiac catheterization showed the left (pulmonary) ventricular pressure was over the systemic level. We performed a successful one-stage switch conversion. The patient is doing well 1 year after the switch conversion.- - - - - - - - - - ranking = 5keywords = hypertension (Clic here for more details about this article) |
4/44. pulmonary veno-occlusive disease after neoadjuvant mitomycin chemotherapy and surgery for lung carcinoma.pulmonary veno-occlusive disease (PVOD), an uncommon cause of pulmonary hypertension (PH) has been reported following treatment of a variety of different malignancies with various chemotherapy. We report here the cases of two patients with non-small cell lung cancer (NSCLC) who developed fatal PH after combined treatment with surgery and a mitomycin containing perioperative chemotherapy (PCT). PVOD was documented at autopsy in one patient and was strongly suspected in the other patient who had an identical clinical presentation and in whom the work-up looking for another cause of PH was negative. mitomycin was incriminated in both cases. Without questioning the potential interest of perioperative chemotherapy in resectable NSCLC, these observations illustrate the risks related to the combination of pneumotoxic chemotherapy and thoracic surgery.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
5/44. Recurrent unilateral bacterial pneumonias and interstitial fibrosis associated with pulmonary vein atresia: successful treatment with endovascular stent implantation.A variety of pulmonary vascular disorders, such as hemangiomatosis, telangectasia, and veno-occlusive disease, may be involved in the pathogenesis of interstitial lung diseases. We describe the case of a girl with recurrent bacterial pneumonia and progressive interstitial fibrosis affecting the right lung. Morphologic evaluation of the lung biopsy showed structural changes of the vessel walls suggesting pulmonary hypertension. The echocardiogram showed the presence of centripetal blood flow in the right pulmonary artery from the periphery of the lung to the heart. A selective right angiography demonstrated the presence of pulmonary venous obstruction at the veno-atrial junction, successfully treated by endovascular stent implantation during cardiac catheterization.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
6/44. Effects of continuous IV prostacyclin in a patient with pulmonary veno-occlusive disease.pulmonary veno-occlusive disease (PVOD) is a rare but life-threatening disease. Although prostacyclin (PGI(2)) attenuates pulmonary hypertension and improves the prognosis in patients with primary pulmonary hypertension, little information is available regarding the effect of PGI(2) on patients with PVOD. This report describes a patient with severe PVOD who showed marked improvement in exercise capacity and pulmonary hemodynamics with continuous IV PGI(2) treatment. Furthermore, he experienced no clinical events for 12 months and survived for 25 months after the initiation of PGI(2) therapy. These results suggest that continuous IV PGI(2) therapy may serve as a bridge to transplantation in some cases of PVOD.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
7/44. pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II.pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension in which the vascular changes originate in the small pulmonary veins and venules. The pathogenesis is unknown and any link with primary pulmonary hypertension (PPH) has been speculative. Mutations in the bone morphogenetic protein receptor II (BMPR2) gene have been identified in at least 50% of familial cases and in 25% of sporadic cases of PPH. We report a patient with documented PVOD whose mother had severe pulmonary hypertension. Sequencing of the patient's BMPR2 coding region revealed a del44C mutation in Exon 1 that is predicted to encode for a truncated protein. Analysis of dna from family members suggests that this mutation was transmitted by the proband's mother to two of her four children. The finding of PVOD associated with a BMPR2 mutation reveals a possible pathogenetic connection with PPH.- - - - - - - - - - ranking = 3keywords = hypertension (Clic here for more details about this article) |
8/44. Acquired pulmonary vein stenosis as a cause of life-threatening pulmonary hypertension.We report the case of an infant born prematurely at 27 weeks gestational age with life-threatening pulmonary hypertension crisis as a result of left upper pulmonary vein stenosis. Surgical treatment consisted of a lobectomy, which is a safe and effective procedure. Evidence strongly suggests that the venous stenosis may have resulted from hypertonic drugs infused through an umbilical catheter facing the upper left venous-atrial junction.- - - - - - - - - - ranking = 5keywords = hypertension (Clic here for more details about this article) |
9/44. pulmonary veno-occlusive disease and the CREST variant of scleroderma.pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension. The authors describe a 48-year-old female with the calcinosis-Raynaud's phenomenon-Esophageal dysmotility-Sclerodactyly-Telangiectasia (CREST) variant of scleroderma who developed acute pulmonary hypertension with pulmonary infiltrates and a normal pulmonary capillary wedge pressure. At post mortem examination typical changes of pulmonary veno-occlusive disease were found. Similarities between this and other cases in the literature suggest a possible association between the CREST variant of scleroderma and pulmonary veno-occlusive disease.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
10/44. A case of pulmonary veno-occlusive disease associated with systemic sclerosis.The case of a patient with pulmonary veno-occlusive disease associated with systemic sclerosis is reported. The patient presented with progressive dyspnoea. echocardiography and cardiac catheterization study demonstrated right-sided heart failure. The CXR suggested pulmonary hypertension and interstitial pulmonary oedema. We suspected pulmonary veno-occlusive disease based on radiological and haemodynamic findings. Treatment with prednisolone resulted in a reduction in pulmonary arterial pressure and CXR findings improved 2 months later, but no further effect was observed. The patient died 7 months later and at autopsy the lungs showed prominent thickening of the interlobular septa and small branches of pulmonary veins showed intimal thickening.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
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