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)

Pulmonary veno-occlusive disease (PVOD) is a rare disorder and can be misdiagnosed as idiopathic pulmonary arterial hypertension (iPAH). PVOD and iPAH often share a similar clinical presentation, genetic background, and hemodynamic profile. PVOD accounts for 5 to 10% of cases initially considered as iPAH. When compared with iPAH, PVOD is characterized by a higher male:female ratio, higher tobacco exposure, and lower PaO (2) at rest, diffusing capacity for carbon monoxide (DLCO), and oxygen saturation nadir during the 6-minute walk test. High-resolution computed tomography (HRCT) of the chest may be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines, and lymph node enlargement. Additionally, occult alveolar hemorrhage is associated with PVOD. Definitive diagnosis necessitates a surgical lung biopsy; however, this procedure is exceedingly high risk in this patient population and is generally not recommended. Therefore, a noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests, and bronchoalveolar lavage may be helpful to detect PVOD. In contrast with iPAH, PVOD is characterized by an even poorer prognosis and the possibility of developing severe pulmonary edema with specific PAH therapy. Lung transplantation remains the treatment of choice, but cautious use of specific PAH therapy can be helpful in select patients while awaiting this intervention.
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PMID:Idiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease: similarities and differences. 1963 80

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