1. August 9, 20151 THE CARDIOVASCULAR SYSTEM IN CHILDHOOD EVALUATION AND TREATMENT SSA Conference, September 24, 2008 Joel Brenner, MD Director, Pediatric Cardiology Helen B. Taussig Children’s Congenital Heart Center

2. The Cardiovascular System in Childhood: Disability Evaluation under Social Security There may be some cynicism about governmental (and NGO) processes when it comes to health care decision making. The role of health care professionals, administrators, patient advocates, and patients is to come together to learn from each other and make the system work for the betterment of patient care.

4. Evaluation of the Cardiovascular System in Childhood Definition of cardiovascular impairment: –Any disorder that affects the proper function of the heart or circulatory system, whether congenital or acquired. Chronic CHF or ventricular dysfunction. Pain due to myocardial ischemia Syncope from any cardiac cause Central cyanosis August 9, 20154

5. The Cardiovascular System in Childhood : Disability Evaluation Under Social Security Category of Impairments Chronic heart failure Recurrent arrhythmia Congenital heart disease Heart transplant Rheumatic heart disease August 9, 20155

6. Evaluation of the Cardiovascular System in Childhood: Congenital Heart Disease Definition of CHD: any abnormality of the heart or major blood vessels present at birth –Abnormalities of septation: VSD, AV canal –Cyanotic heart disease: TOF, TGA –Obstruction to ventricular outflow: PS, AS –Major abnormalities of ventricular development: HRH, HLH August 9, 20156

7. Evaluation of the Cardiovascular System in Childhood Symptoms and signs usually observed over time—3 months Laboratory findings– appropriate, medically acceptable imaging Response to prescribed therapy Functional limitation August 9, 20157

8. Evaluation of CHF & Cyanosis Symptoms –Tachypnea –Poor feeding –Poor weight gain –Cyanotic spells –Exercise intolerance Signs –Increased respiratory rate (PVC) –Hepatomegaly, peripheral edema (SVC) –Failure to thrive –Decreased O2 sat –Elevated Hct August 9, 20158

9. Evaluation of the Cardiovascular System in Childhood Well repaired minor or moderate CHD will have no/minor medical issues and require regular but infrequent surveillance. Well palliated complex CHD will have minor to serious cardiac issues requiring regular, frequent surveillance through adult years. August 9, 20159

10. Evaluation of the Cardiovascular System in Childhood: Imaging Tests Chest X Ray Echocardiography Radionuclide angiography Cardiac catheterization Computerized tomography (CT) Magnetic resonance imaging (MRI) August 9, 201510

11. Imaging Studies: Chest X Ray Cardiomegaly is present if: –CT ratio > 60% in an infant or > 55% in a toddler or child on a 6 foot PA chest XRay –But 6 foot PA film rarely done in infants and toddlers. August 9, 201511

12. Imaging Studies: ECHO Cardiomegaly and heart dysfunction: –LVDD or LVSD > 2 standard deviations above mean for BSA –LV mass > 2 SD –SF(% of blood pumped with each beat) > 2 SD below mean for BSA August 9, 201512

13. Imaging Studies: ECHO Cardiomegaly and heart dysfunction: –LVDD or LVSD > 2 standard deviations above mean for BSA –LV mass > 2 SD –SF(% of blood pumped with each beat) > 2 SD below mean for BSA August 9, 201513

14. Imaging Studies: ECHO

15. New Imaging Modalities that need to be added to Evaluation Scheme MRI/cine –Ideal for functional evaluation, especially with complex CHD, or for patients with poor echo windows –No XRay exposure –Long acquisition time CT angiography –Better definition of vascular anatomy –Quick acquisition time –Substantial XRay exposure –Ubiquitous availability August 9, 201515

16. New Imaging Modalities that need to be added to the Evaluation Scheme MRI –Must be used in conjunction with on going clinical evaluation and care –Requires 3D reconstruction for functional and anatomic evaluation –Requires specific knowledge of cardiac anatomy CT --Must be used in conjunction with on going clinical evaluation and care --Should be used sparingly, given radiation exposure --Should be used in centers with specific expertise in complex CHD August 9, 201516

17. Evaluation and Treatment of the Cardiovascular System in Childhood: 50 years of progress 1958 HLH: uniformly fatal Critical AS: often fatal Critical PS:high risk op VSD and AV canal: palliation with pulmonary artery band TOF: palliation with Blalock-Taussig shunt TGA-uniformly fatal 2008 HLH:Norwood/Fontan palliation or transplant Critical AS: excellent palliation with balloon Critical PS: excellent relief with balloon cath VSD and AVC:most often repaired TOF: repaired TGA: repaired August 9, 201517

18. Diagnosis and Treatment of TGA: 1978 Primary care physician recognizes cyanosis, confirmed by ABG Diagnostic cardiac cath with balloon septostomy Pre-op cardiac cath Surgical repair: Mustard procedure Post op cardiac cath Frequent Holter monitoring for SVT/SSS Eventual RV failure RV AO

19. Diagnosis and Treatment of TGA:2008 Primary care physician recognizes cyanosis Echo confirms diagnosis of TGA Surgical repair:ASO Post op echo RV AO LV PA

20. MRI in Patient following Arterial Switch Procedure for TGA RV PA RV PA AO

21. Advances in Cardiac Imaging Myocardial enhancement Abnormal coronary origin

22. Evaluation of the Cardiovascular System in Childhood Patients with moderate heart disease, e.g., Tetralogy of Fallot, will often have no restrictions placed upon them during childhood, yet may develop long term problems requiring medical/surgical rx: –RV dilatation and dysfunction from chronic pulmonary valve regurgitation –Rhythm disturbance August 9, 201522

23. Evaluation of the Cardiovascular System in Childhood Patients with successfully palliated severe CHD, e.g., HLH, HRH, single ventricle, will face restrictions and limitations during childhood and likely additional difficulties during adulthood: –Progressive ventricular dysfunction –Recurrent hypoxemia –Rhythm disturbance August 9, 201523

24. Evaluation of the Cardiovascular System in Childhood: Heart Rhythm Abnormalities August 9, 201524 Complete Heart Block

25. Evaluation of the Cardiovascular System in Childhood: Heart Rhythm Abnormalities Arrhythmia: a change in the regular beat of the heart –Irregular heart beat –Tachycardia: SVT, VT –Bradycardia Syncope: loss of consciousness Near syncope: altered consciousness August 9, 201525

26. Evaluation of the Cardiovascular System in Childhood: Heart Rhythm Abnormalities Association between syncope and heart rhythm abnormality must be established and documented: –Holter monitoring –Tilt table testing –Event recorder August 9, 201526

27. Evaluation of the Cardiovascular System in Childhood: Heart Rhythm Abnormalities Syncope must occur 3 or more times within 12 months despite appropriate medical therapy –Tachycardia: chaotic, rapid rhythm –Bradycardia: profound slowing –Superimposed congestive heart failure August 9, 201527

28. Evaluation of the Cardiovascular System in Childhood: Heart Rhythm Abnormalities August 9, 201528 VTach VFib

29. Evaluation of the Cardiovascular System in Childhood: Heart Rhythm Abnormalities Syncope must occur 3 or more times within 12 months despite appropriate medical therapy –Syncope due to heart rhythm abnormality is a marker for sudden death –Symptoms occurring 3 or more times within 12 months requires more aggressive therapy August 9, 201529

30. Evaluation of the Cardiovascular System in Childhood: Inflammatory Heart Disease Rheumatic Fever Persistence of LV dilatation and dysfunction, valvular regurgitation uncommon, but readily evaluated. Kawasaki Disease Small percentage of children with residual abnormality in childhood: coronary artery aneurysms, thrombosis, obstruction. Unknown implications for ischemic heart disease in adulthood. August 9, 201530

31. Kawasaki Disease AngiogramS Anatomy

32. Heart Transplantation in Childhood The majority of transplants are done in children with cardiomyopathy / myocarditis. Palliation for complex congenital heart disease as a primary strategy or failed conventional surgical therapy is the 2 nd leading cause for transplantation.

33. August 9, 201533 Heart Transplantation In Children ISHLT J Heart Lung Trans 2007:26, 796

34. August 9, 201534 Heart Transplantation In Children January 1996 - June 2006 ISHLT data, 2007

35. August 9, 201535 Heart Transplantation In Children January 1997 – June 2006 ISHLT data, 2007

36. August 9, 201536 Heart Transplantation In Children ISHLT data, 2007

37. August 9, 201537 Heart Transplantation In Children ISHLT data, 2007

38. Evaluation and Treatment of the Cardiovascular System in Childhood: 2008 Successfully repaired, the majority of patients with minor or moderate cardiac abnormalities will have few if any medical issues/cardiac disability after the 3 month recovery period. Successfully palliated, the vast majority of patient with moderate or complex CHD will continue to require close medical surveillance and likely have cardiac symptoms and limitations. August 9, 201538

39. Evaluation of the Cardiovascular System in Childhood Many patients with complex lesions, corrected or well palliated in childhood, are now surviving into adulthood. The long-term “natural history” of operated patients with CHD, particularly those with complex lesions, is still being written. August 9, 201539

40. Evaluation of the Cardiovascular System in Childhood Evaluation will need to encompass more than imaging for evaluation of cardiac size and muscle mechanics. –Functional assessment of heart rhythm, exercise capacity, myocardial oxygen consumption will eventually need to be incorporated into guidelines. Confounding factors will influence results: –Psycho-social issues with child, adolescent and family –Exogenous obesity August 9, 201540