Pulmonary Hypertension: A Fatal Complication of Neurofibromatosis Type 1

Authors: Gumbiene, Lina; Petrulioniene, Zaneta; Rucinskas, Kestutis; Maneikiene, Vyte; Serpytis, Pranas; Dranenkiene, Alicija; Laucevicius, Aleksandras

Source: Respiratory Care, Volume 56, Number 11, November 2011 , pp. 1844-1848(5)

Publisher: The Journal Respiratory Care Company

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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.

Keywords: pulmonary hypertension; neurofibromatosis

Document Type: Research article

DOI: http://dx.doi.org/10.4187/respcare.01030

Affiliations: 1: Department of Cardiovascular Medicine, Vilnius University Hospital Santariskiu Klinikos, Vilnius University, Vilnius, Lithuania

Publication date: 2011-11-01

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