Evaluation for Congenital Heart Diseases

1. Evaluation for Congenital Heart Diseases Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

2. Heart Diseases in Children Congenital heart diseases Rheumatic heart disease : Rheumatic fever Other acquired diseases: Kawasaki Cardiomyopathy Arrhythmia

3. Effects of CHD • No effect on a child • Decreased function when stressed • Decreased cardiopulmonary function • Other organ/system manifestation

4. Presentation of CHD • Shock like symptoms • Cyanosis • Congestive symptoms • Exercise intolerance • Asymptomatic heart murmur • Abnormality in routine chest PA • Chest pain • Syncope/ seizure/ fainting • Airway obstruction/ dysphagia

5. Shock like Symptoms • Non-specific: collapse, pallor, cold clammy skin, hypotension, oligouria, acidosis • HLHS, critical AS, IAA, COAunable to maintain systemic output during transitional circulation • Most common in neonate / infancy • Very important to recognize

6. Cyanosis • Cyanosis: more than 5.0g% of reduced Hb. • Central versus Peripheral cyanosis • Central cyanosis: CNS, heart, lung • Pathophysiology: TGA, TOF, CML, DDPC

7. Congestion • Symptoms: dyspnea on feeding, sweating, poor weight gain, frequent respiratory infection • Most common mode in infancy • Obligatory shunt lesions/ Left to right shunt lesions/ CML with no PS/ Myocarditis/ Arrhythmia

8. Asymptomatic Murmur • Heart murmur noted in routine examination • ASD, VSD, PS, AS, Bicuspid AV • Common mode in childhood • Murmur does not mean there is a CHD • No murmur does not mean there is no CHD

9. Abnormality in Chest PA Corrected TGA/ Pericardial Defect/ Cardiac Tumor

10. Chest Pain * Anomalous origin of left coronary artery from pulmonary artery * Aortic stenosis(severe) / Hypertrophic cardiomyopathy

11. Syncope/ Seizure/ Fainting • Anoxic spell of acyanotic TOF • Arrhythmia : VT, SVT, long QT syndrome • Syncope on exercise : AS, SAS, IHSS

12. Airway Obstruction/ Dysphagia

13. Airway Obstruction / Dysphagia Abnormal PA Branching

14. Mode of / Age at Presentation • Collapse : within 3-4 days • Cyanosis : usually within a month • Congestion : 1-5 months • Murmur : any time

15. Diagnosis of Cardiac Diseases • Anatomic Diagnosis • Hemodynamic Evaluation • Total assessment / Prognosis • Etiologic Diagnosis

16. Diagnostic Tools • History and physical examination • Chest X ray / EKG / Blood study • Echocardiography/ Other imaging tools • Catheterization/ Other invasive tools

17. Accuracy of Tools • History/PE : important, rarely specific • X-ray/EKG : not-confirmative • Echocardiography: confirmative, but non-invasive • Cardiac catheterization : confirmative, but invasive

18. History Taking • Cyanosis: onset, progression, anoxic spell • Congestion: feeding, wt. gain, respiratory infection, sweating, tachy-dyspnea • Exercise tolerance: easy fatigability, DOE • Possible Causes: maternal diseases, drug, infection, family history

19. Physical Examination • Inspection :general appearance, nutrition, syndrome?, facial morphology, jugular venous pulse, respiratory pattern, rate, chest retraction, alae nasi flaring, dyspnea, precordial bulging, cyanosis, clubbing • Palpation: apical pulse, precordial activity, thrill, arterial pulse, location and size of liver and spleen • Auscultation: S1, S2, abnormal sounds, murmur • Please do not pull out stethoscope before you observe patients carefully

20. Chest X-ray Heart size, shape, pulmonary vascularity

21. Chest X-ray

22. Electrocardiography

23. Purposes of Imaging • Anatomic-pathologic diagnosis • Hemodynamic assessment (velocity, flow, pressure, stress-strain) • Volume, function, wall motion, torsion • Coronary perfusion / Metabolism • Tissue characterization

24. Echocardiography • Easy, non-invasive, accurate, real-time • Anatomic and physiologic information • Changed practice of pediatric cardiology

25. Echocardiograhy - Modalities • M-mode / 2-D / 3-D • Doppler / color Doppler • Trans-thoracic, trans-esophageal, trans-abdominal, trans-vaginal, intra-cardiac, intra-vascular

26. Echocardiograhy

27. Echocardiograhy

28. M-mode Echocardiograhy

29. 3-D Echocardiograhy

30. New Development in Echo • Imaging: edge detection/auto-measurement • Doppler: 3-D flow / stress-strain • Contrast echo: coronary perfusion

31. Other Imaging Tools • Magnetic Resonance Imaging (MRI) • CT / Electron-beam CT (EBCT) • Radionuclide / SPECT • Positron Emission Tomography

32. Magnetic Resonance Imaging Spin echo Gradient echo Velocity encoded

33. Magnetic Resonance Imaging • Sectional still image/ cine image/ 3-D • Flow information / volume flow • Less window dependant / post-op study, older age / functional evaluation

34. Magnetic Resonance Imaging

35. Magnetic Resonance Imaging

40. Computerized Tomography

41. Computerized Tomography

42. Radionuclide Study

43. Radionuclide Study

44. Positron Emission Tomography

45. Purposes of Catheterization • Anatomic diagnosis • Hemodynamic assessment • Interventional procedure

46. Equipment • Biplane monitor / Cine with digital subtraction • Patient monitoring : EKG, BP, pulse oximeter • Physiologic signal amplifier and recording device • Blood gas, O2 consumption, Dye/ Thermodilution • Emergency treatment tools : • Room for Others : anesthesia, echo, exercise

47. Catheterization Room

48. Fluoroscopic Monitor

49. Physiologic Signal - Display & Recording