Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034063 (pulmonary edema)
 10,665 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Continuous intravenous epoprostenol (prostacyclin) produces hemodynamic and symptomatic responses and improves survival in patients with severe primary pulmonary hypertension refractory to conventional medical therapy. However, it has been recently shown that short-term infusion of epoprostenol can produce pulmonary edema in pulmonary veno-occlusive disease, presumably because of increased pulmonary perfusion in the presence of downstream vascular obstruction. We describe two additional cases of pulmonary edema complicating continuous intravenous epoprostenol in patients displaying severe pulmonary hypertension and pulmonary capillary hemangiomatosis, a rare condition characterized by the proliferation of thin-walled microvessels in the alveolar walls. This report indicates that epoprostenol therapy should not be used in patients with severe pulmonary hypertension secondary to pulmonary capillary hemangiomatosis.
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PMID:Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. 960 54

The new classification of pulmonary hypertension proposed in the joint European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines, combines pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) from separate categories into a single subcategory within pulmonary arterial hypertension (PAH) because of specific similarities in their diagnosis, prognosis, and management. These diseases are characterized histologically by their predominant involvement of small pulmonary veins (PVOD) and capillaries (PCH). Their precise prevalence is not known, but they are thought to account for 5 to 10% of the forms of PAH initially considered idiopathic. They cannot be distinguished from idiopathic PAH by their clinical or hemodynamic presentation. Only pathology examination can confirm the diagnosis, but lung biopsies are high-risk procedures and not recommended. A less invasive approach combining chest CT (centrilobular ground-glass opacities, septal lines, and mediastinal adenopathy), blood gases (resting hypoxemia), lung function tests [collapse in carbon monoxide diffusion (DLCO)] and bronchoalveolar lavage (occult intra-alveolar hemorrhage) makes it possible to screen the patients at risk of PVOD or HCP and thus avoid a lung biopsy. These patients have a poor prognosis and are at risk of developing severe pulmonary edema after the initiation of specific treatment for PAH. In view of their limited response to specific treatment and poor prognosis, pulmonary transplantation remains the treatment of choice for PVOD and HCP. In patients with the most severe disease, the prudent use of continuous intravenous epoprostenol, can serve as bridge-therapy while awaiting a lung transplant.
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PMID:[Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis]. 1991 27

This is a report of fatality immediately after administration of epoprostenol. The patient was previously diagnosed with CREST syndrome and associated interstitial lung disease. She developed worsening pulmonary hypertension and was clinically diagnosed with pulmonary veno-occlusive disease. The patient developed flash pulmonary edema and arrested after administration of low-dose epoprostenol in the intensive care unit. An autopsy revealed the patient suffered from pulmonary capillary hemangiomatosis. We review our case and what is known about this rare disease.
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PMID:Pulmonary capillary hemangiomatosis associated with CREST syndrome: a case report and review of the literature. 2045 87

Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis are rare forms of pulmonary vascular disease. We report two cases of affected children who had evidence of pulmonary hypertension 3-5 years before developing radiographic findings of pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis. Both patients experienced a moderate decrease in pulmonary arterial pressure during acute vasodilator testing. Both patients experienced an improvement in six-minute walk performance without an increase in pulmonary edema when treated with targeted therapy for pulmonary hypertension. In some patients, pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis may progress slowly over a period of months to years. A favorable acute vasodilator response may identify patients who will tolerate, and demonstrate transient clinical improvement with, medical therapy.
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PMID:Pulmonary veno-occlusive disease: Two children with gradual disease progression. 2807 Apr 82

A pulmonary vein occlusion and biopsy proven pulmonary veno-occlusive disease (PVOD) and hemangiomatosis is found in a bilateral lung transplant patient. A 61-year-old male presents with dyspnea and chest pain with minimal exertion at routine follow up on post-transplant day of 50. Chest CT demonstrates new occlusion of bilateral superior pulmonary veins and diffuse pulmonary edema. Pulmonary vein occlusion is confirmed by trans-esophageal echocardiogram, and PVOD and hemangiomatosis is corroborated with lung biopsy. Normal pulmonary capillary wedge pressure (PCWP) and reduced DLCO are also consistent with PVOD. Vigilant evaluation of large pulmonary venous thrombus is as important as of arterial thrombus in a postsurgical transplant status. A dedicated protocol of pulmonary venous phase scan would be beneficial to identify subtle pulmonary venous abnormalities. Although PVOD/PCH is normally considered in patients with nonspecific PAH symptoms, lacking of direct manifestation of PAH should not dismiss the diagnosis of PVOD/PCH, particularly in lung transplant individuals with large pulmonary vein occlusion, progressive respiratory symptoms, DLCO abnormalities, and pulmonary congestion since it may represent a wide spectrum of occlusive vascular disease.
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PMID:Pulmonary vein occlusion and veno-occlusive disease in a bilateral lung transplant patient: A case report. 3225 89

Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-ranging pathological mechanism in PAH could elucidate the clinical state of severe CHD-PAH. Using electronic medical records, we retrospectively analyzed six infants with severe CHD-PAH who had treatment-resistant PH. All patients were born with congenital malformation syndrome. After starting on a pulmonary vasodilator, five of the six patients developed complications including pulmonary edema and interstitial lung disease (ILD), and four patients had alveolar hemorrhage. After steroid therapy, the clinical condition improved in four patients, but two patients died. The autopsy findings in one of the deceased patients indicated the presence of recurrent alveolar hemorrhage, pulmonary venous hypertension, ILD, and PAH. Based on the clinical course of these CHD-PAH in patients and the literature, CHD-PAH can occur with pulmonary vascular obstructive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH), ILD, and/or alveolar hemorrhage. The severity of CHD-PAH may depend on a genetic disorder, respiratory infection, and upper airway stenosis. Additionally, pulmonary vasodilators may be involved in the development of PVOD/PCH and ILD. When patients with CHD-PAH show unexpected deterioration, clinicians should consider complications associated with PVOD/PCH and/or pulmonary disease. In addition, the choice of upfront combination therapy for pediatric patients with CHD-PAH should be selected carefully.
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PMID:Pulmonary vasodilators can lead to various complications in pulmonary "arterial" hypertension associated with congenital heart disease. 3228 88