1. Case presentation- Tetralogy of Fallot- Ventricular Septal Defect Agatha Stanek

2. Case presentation 2 day -old infant presents to ER with mother with severe cyanosis Evident retarded growth, low birth weight Dyspnea upon exertion

3. Patient hx Medical Hx Low birth weight; mother gave birth to infant at age of 38 Full-term pregnancy No known allergies yet Family Hx Pseudotruncus arteriosus observed in mother’s niece Some form of cardiovascular diseases, mother reports that grandmother lost 2 children in similar manner- do not know reason why

4. Social Hx Mother is a lawyer; father is a successful real- estate agent. Live in upper-class suburban area No major stressors in family

5. Physical Exam Normal arterial and jugular venous pulses Systolic thrill along left sternal border Single S2, decreased P2 HEENT: Retinal engorgement hemoptysis

6. Differential Diagnosis

7. Diagnostic procedures Are laboratory tests appropriate? Imaging Tests? Chest X-ray: Doppler echocardiogram:

8. Diagnostic procedures cont’d Cardiac catheterization Assess severity of right ventricular outflow obstruction Locate position of VSD and its size Rule out possible coronary artery anomalies

9. Diagnosis Tetralogy of Fallot

10. Treatment Inpatient care- diagnosis and surgery Surgical measures: Blalock- Taussig shunt or modified shunt (subclavian to pulmnoary artery) Pott’s procedure: descending aorta to pulmonary artery) Waterston’s shunt: ascending aorta to pulmonary artery Total correction surgical therapy- includes patch closure of VSD and relief of right ventricular outflow obstruction

11. Treatment cont’d. Diet: -Salt restriction Activity: -As tolerated Medications: No specific drug therapy if there is no heart failure...

12. Follow-up Patient monitoring: Postoperative Doppler ultrasound 1 year from procedure Post-valvotomy SBE prophylaxis still required Regular follow up assessment for patients not undergoing surgical correction

13. Possible complications Erythrocytosis? Brain abscess, acute gouty arthritis Infective endocarditis Cerebrovascular thrombosis Postoperative? Fatal if not surgically corrected