Abstract
We report a very rare case of severe pulmonary arterial hypertension in a patient with neurofibromatosis type 1, and discuss the pathology, pathogenesis, current pulmonary hypertension classification system, and outcomes of pulmonary arterial hypertension in patients with neurofibromatosis type 1.
MeSH terms
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Adult
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Contraindications
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Fatal Outcome
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Female
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Humans
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Hypertension, Pulmonary / classification
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Hypertension, Pulmonary / etiology*
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Hypertension, Pulmonary / physiopathology
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Hypertension, Pulmonary / therapy
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Lung / diagnostic imaging
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Lung Transplantation
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Neurofibromatosis 1 / complications*
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Tomography, X-Ray Computed