Pulmonary Vascular Disease in Children with Congenital Cardiovascular Malformations
| Autor: | JR Gordon M. Folger |
|---|---|
| Rok vydání: | 1983 |
| Předmět: |
Heart Defects
Congenital Heart Septal Defects Ventricular Male medicine.medical_specialty Pediatrics Adolescent Heart disease Hypertension Pulmonary Polycythemia Disease 030204 cardiovascular system & hematology Hypoxemia 03 medical and health sciences 0302 clinical medicine medicine Humans 030212 general & internal medicine Child Cyanosis Heart Failure Clinical pathology Vascular disease business.industry Puberty medicine.disease Truncus Arteriosus Persistent Pulmonary hypertension Child Preschool Tetralogy of Fallot Female Down Syndrome medicine.symptom Abnormality Cardiology and Cardiovascular Medicine business Complication |
| Zdroj: | Angiology. 34:784-811 |
| ISSN: | 1940-1574 0003-3197 |
| DOI: | 10.1177/000331978303401205 |
| Popis: | A detailed clinical analysis of 16 patients with various forms of congenital cardiac malformations with the potential for large left-to-right shunts who developed extreme pulmonary hypertension in infancy and early childhood has been carried out. The study plan was to include only patients with sufficiently long follow up to allow analysis of their course in both the prepubescent and postpubescent periods. In early life these patients were all characterized by lack of evidence for significant development of the expected left-to-right shunt indicating inappropriate maturation of their pulmonary vascular circulation. Throughout all phases of their life, they were significantly polycythemic in comparison with a control group but this polycythemia, representative of peripheral systemic hypoxemia, increased markedly with the acquisition of puberty in all patients. These two findings indicate both an early abnormality, suggestively primary, of the pulmonary vasculature and a late progression of the disease associated with pubertal maturation. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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