Synonyms
PH
Definition
PH: a disease state of pulmonary vasculature with elevated blood pressure whether from increased resistance in pulmonary vessels, backup of pressure from left heart, or shunting of blood from left to right heart. Defined clinically as pulmonary arterial systolic pressure greater than 25 mmHg at rest or 30 mmHg upon exercise. Radiologically, enlarged central pulmonary arteries may be seen. Cardiac catheterization is usually necessary for first diagnosis, with subsequent work-up to search for cause. The 2013 update of WHO classification contains five main groups.
Clinical Features
Symptoms include slowly progressive dyspnea, cyanosis, fatigue, and weakness. Other symptoms may include hemoptysis in certain cases and also those of underlying conditions. Untreated, this condition results in heart failure and death.
Macroscopy
Most cases have no reliable macroscopic features within the pulmonary parenchyma. However, larger pulmonary arteries may show yellow...
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References and Further Reading
Barnett, C. F., Hsue, P. Y., & Machado, R. F. (2008). Pulmonary hypertension: An increasingly recognized complication of hereditary hemolytic anemias and HIV infection. JAMA, 299(3), 324–331.
Cool, C. D. (2008). Vascular diseases. In D. S. Zander & C. F. Farver (Eds.), Pulmonary pathology: A volume in the series: Foundations in diagnostic pathology (pp. 111–125). Philadelphia: Churchill Livingstone.
Machado, R. F., & Gladwin, M. T. (2010). Pulmonary hypertension in hemolytic disorders: Pulmonary vascular disease: The global perspective. Chest, 137(6 Suppl), 30S–38S. doi:10.1378/chest.09-3057.
Patchefsky, A. S. (2012). Nonneoplastic pulmonary disease. In S. E. Mills (Ed.), Diagnostic surgical pathology (pp. 1035–1039). Philadelphia: Lippincott Williams & Wilkins.
Simonneau, G., Gatzoulis, M. A., Adatia, I., Celermajer, D., Denton, C., Ghofrani, A., Gomez Sanchez, M. A., Krishna Kumar, R., Landzberg, M., Machado, R. F., Olschewski, H., Robbins, I. M., & Souza, R. (2013). Updated clinical classification of pulmonary hypertension. Journal of the American College of Cardiology, 62(25 Suppl), D34–D41.
Steinhorn, R. H. (2010). Neonatal Pulmonary Hypertension. Pediatric Critical Care Medicine: A Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies, 11(2 Suppl), S79–S84. doi:10.1097/PCC.0b013e3181c76cdc.
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Ross, J., Jagirdar, J. (2018). Pulmonary Hypertension. In: Cagle, P.T., Kerr, K.M. (eds) Pulmonary Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-69263-0_336
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DOI: https://doi.org/10.1007/978-3-319-69263-0_336
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