Description:
It is predominantly right-to-left shunt or reversal of left-to-right shunt, which usually resuts from severe pulmonary vascular obstruction.
Complications:1. Stroke
2. Hemoptysis
3. Hyperviscosity
4. Hemostatic abnormalities.
5. Thrombocytopenia
6. Prolonged bleeding, prothrombin or partial thromboplastin times.
7. Vitamin K-dependent clotting factors deficiencies.
8. Abnormal fibrinolysis.
9. Cholelithiasis
10. Hypertrophic osteoarthropathy
11. Hyperuricemia and goutrenal dysfunction.
12. Sudden death.
Clinical Features:
Symptoms : dyspnea on exertion, chest pain, syncope, hemoptysis, angina.
Signs : cyanosis (constant or exercise), ascites, pedal edema, clubbing.
Investigations:
1. Blood tests :
a) 100% O2 does not correct arterial desaturation.
b) polycythemia (Hb > 20 g/dl common)
2. Imaging studies : echocardiography, Doppler, hemogram, catheterization
ECG shows right ventricular hypertrophy changes.
Treatment:
Surgery is indicated if medical treatment does not improve the oxygen saturation levels.
Phlebotomy may be necessary.
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