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Pulmonary hypertension (PH) may be the result of many different pathological processes. PH is a rare but recognized vascular complication following major lung resection. We describe the diagnosis and management of moderate PH resulting more than 50 years in a patient who underwent a total unilateral pneumonectomy in infancy. Unfortunately, patients who undergo pneumonectomy will likely go on to develop PH and their functional status will be greatly impacted. In the case presented, we report on a patient whose PH and symptoms improved following off-label WHO group 1 treatment.
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February 2024
From the Department of Medicine, Westchester Medical Center, New York Medical College, Valhalla, NY.
Pulmonary hypertension (PH) may be the result of many different pathological processes. PH is a rare but recognized vascular complication following major lung resection. We describe the diagnosis and management of moderate PH resulting more than 50 years in a patient who underwent a total unilateral pneumonectomy in infancy.
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Article Synopsis
- A 7-week-old infant exhibited persistent noisy breathing and aspiration issues, with normal neurological exams and brain scans.
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- Genetic analysis identified a novel mutation in the MYH2 gene shared by both brothers, suggesting a link between MYH2 deficiency and recurrent aspiration in infants, highlighting the need for this mutation to be considered in similar cases.
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Patient Concerns: We presented 2 cases of neonatal CBPFM.
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