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[Primary hypertension of the lesser circulation]
Arkhiv Patologii
|January 1, 1976
Summary
Primary pulmonary hypertension (PPH) encompasses congenital and acquired forms. The article suggests reserving the term "primary pulmonary hypertension" solely for congenital cases, proposing "isolated pulmonary hypertension" as a broader alternative.
Area of Science:
- Cardiology
- Pulmonology
- Pathology
Context:
- Primary pulmonary hypertension (PPH) is a complex condition with polyetiological origins.
- Historically described as a distinct nosological form since the 1950s.
- PPH presents with two main forms: congenital and acquired.
Purpose:
- To review literature data and author's findings on primary pulmonary hypertension.
- To clarify the classification and terminology of PPH.
- To differentiate between congenital and acquired forms of pulmonary hypertension.
Summary:
- Congenital PPH involves pulmonary vessel anomalies, including retained fetal structures or intraorgan vascular anomalies.
- Morphological hallmarks of congenital PPH include endosclerosis and medial sclerosis of pulmonary arteries, alongside significant right ventricular hypertrophy.
- Acquired PPH arises from factors like drug exposure, thromboembolism, or high altitude, questioning its "primary" designation.
Impact:
- Recommends restricting "primary pulmonary hypertension" to congenital forms.
- Proposes "isolated pulmonary hypertension" as a more encompassing term for this polyetiological condition.
- Aims to improve diagnostic clarity and disease classification in pulmonary hypertension research.