Pathology Notes

Cor pulmonale Updated:09/06/2022 © Jun Wang, MD, PhD   General features Latin word: Pulmonary heart Alteration in the structure and function of the right ventricle of the heart Caused by a primary disorder of the respiratory system Commonly associated with pulmonary hypertension Commonly chronic and progressive clinical course Acute cor pulmonale may be life threatening Etiology Acute: Pulmonary embolism (most common), acute respiratory distress syndrome Chronic: Pulmonary hypertension Risks Any conditions that may increase pulmonary artery pressure Diseases of the pulmonary parenchyma Chronic obstructive pulmonary disease Disorders of the pulmonary vessels Pulmonary hypertension Disorders affecting chest movement Kyphoscoliosis Marked obesity Neuromuscular diseases Disorders inducing pulmonary arterial constriction Metabolic acidosis Hypoxemia Pathogenesis Clinical presentations Associated with right heart failure Dyspnea on exertion: Most common Fatigue, let

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