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Respiratory failure and pulmonary hypertension associated with Klippel-Feil syndrome

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Abstract

A 28-year-old woman with a deformed thorax and kyphoscoliosis associated with Klippel-Feil syndrome developed respiratory failure with pulmonary hypertension. Pulmonary133Xe ventilation and99mTc-MAA perfusion scintigraphies showed maldistributions of lung ventilation and perfusion, and noticeably delayed133Xe washout from the lungs. Dynamic breathing MR imaging showed poor and/or asynchronous respiratory movements of the chest wall and diaphragm. These findings indicate that the perfusion-ventilation imbalance, the decreased ventilatory turnover, and expiratory flow from the alveolar space partly derived from the impaired respiratory mechanics may be responsible for the respiratory complications in this patient.

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Suga, K., Motoyama, K., Hara, A. et al. Respiratory failure and pulmonary hypertension associated with Klippel-Feil syndrome. Ann Nucl Med 13, 441–446 (1999). https://doi.org/10.1007/BF03164942

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  • DOI: https://doi.org/10.1007/BF03164942

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