Pathology Notes

Pulmonary hypertension with unclear and/or multifactorial mechanisms Updated: 08/31/2022 © Jun Wang, MD, PhD   General features Group 5 pulmonary hypertension Associated with various disorders Hematologic disorders: MPNs, postsplenectomy, sickle cell anemia, thalassemia, etc Systemic disorders: Sarcoidosis, pulmonary Langerhans cell histiocytosis Metabolic disorders: Autoimmune thyroid disease, glycogen storage disease, Gaucher disease Other disorders: End-stage renal disease Complex congenital heart disease Pathogenesis Pathogenesis not well understood Clinical presentations Presentations of pulmonary hypertension Presentations of underlying lung diseases Treatment Management of underlying etiology   Back to pulmonary hypertension Back to contents

Pulmonary hypertension due to pulmonary artery obstruction Updated: 08/25/2022 © Jun Wang, MD, PhD   General features Group 4 pulmonary hypertension Two subgroups Chronic thromboembolic pulmonary hypertension (CTEPH) Other pulmonary artery obstructions Tumors of pulmonary artery Pulmonary artery stenosis Arteritis Mediastinal fibrosis, etc Chronic thromboembolic pulmonary hypertension Definition Mean pulmonary arterial pressure > 20 mm Hg Presence of organized, nonacute, thromboembolic material and altered vascular remodeling in the pulmonary vasculature Rare but might be life threatening Likely due to underlying hypercoagulable state Commonly with history of pulmonary embolism Worse prognosis if > 70 years, residual PH, comorbidities, etc Pathogenesis Incomplete thrombus resolution resulting organization and remodeling of pulmonary vessels Other causes resulting narrowing pulmonary vessels  Clinical presentations Features of pulmonary hypertension

Pulmonary hypertension due to lung disease and or hypoxia Updated: 10/12/2022 © Jun Wang, MD, PhD   General features Group 3 pulmonary hypertension Caused by chronic lung diseases or conditions resulting hypoxemia More common in elder population Usually associated with increased morbidity and mortality Associated disorders include Obstructive lung disease (COPD or bronchiectasis) Restrictive lung disease (eg, interstitial lung disease, kyphoscoliosis) Lung disease with mixed obstruction and restriction (eg, pulmonary fibrosis with emphysema) Hypoxia without lung disease (eg, high altitude, sleep-disordered breathing, obesity hypoventilation) Developmental lung disorders (eg, bronchopulmonary dysplasia, congenital lobar emphysema) Pathogenesis Pulmonary inflammation/hypoxia result in pulmonary vasoconstriction and remodeling Clinical presentations Presentations of underlying lung diseases History Exertional hyspnea or hypoxemia unexplainable by current lung dis