Pediatric Rheumatic: Issues on Moving to Adult Care

1. Pediatric Rheumatic: Issues on Moving to Adult Care Virginia C Mappala, MD Pediatric Cardiology

2. Rheumatic Heart Disease • most common acquired heart disease • 5 – 15 years • High morbidity and mortality among adolescent and young adult • Crippling: long and progressively damaging series of events going on from childhood, through adolescence, to adult life.

3. Issues? • Duration of Secondary prophylaxis (Benzathine Injection every 21 days) • Until when?...18?...21?..40?...for life? • Bacterial Endocarditis • Need for antibiotic prophylaxis • Pregnancy/Lactation and Anticoagulation • Psychosocial aspects • Exercise and sports; employment

4. Secondary Prophylaxis for RHD • An absolute must to reduce morbidity and mortality in rheumatic individuals • Effective in reducing and eliminating recurrences • Long-acting penicillin injection is more effective than oral prophylaxis • Lifetime prophylaxis important in RHD

5. AHA Duration of Secondary Prophylaxis for Rheumatic Fever, 2010

6. Secondary Prophylaxis • Consider factors: • Patient’s risk of acquiring strep infection • Anticipated recurrence rate for infection • Consequences of recurrence

7. Secondary Prophylaxis • Adolescents and Adult at INCREASED risk of recurrence: • Parents of young children • School teachers • Medical and paramedical personnel • Military cadets and service men

8. Secondary Prophylaxis • Adolescents and Adult at GREATEST risk of recurrence: • Established RHD • Recent attack of RF (within the last 3 yrs.) • Multiple attacks in the past • Children and adolescents in the crowded home • Undergone valvar surgery for RHD

9. Bacterial Endocarditis • During the past 50 years AHA guidelines recommended antimicrobial prophylaxis to prevent IE in patients with underlying cardiac conditions

10. Bacterial Endocarditis • Steckelberg and Wilson • Lifetime risk of acquisition of IE • ranged from 5 per 100 000 patient-years in the general population with no known cardiac conditions • 2160 per 100 000 patient-years in patients with prosthetic cardiac valves • 52 per 100 000 = MVP, (+) MR

11. Bacterial Endocarditis • RHD with HIGHESTrisk: • Prosthetic valves or valves repaired with prosthetic material • Previous endocarditis • Associated with congenital heart disease AHA Guidelines for Prevention of Infective Endocarditis, 2007

12. Bacterial Endocarditis • RHD with Moderate-risk: • Mitral stenosis and calcific aortic stenosis • Mitral valve prolapse with regurgitation and with or without thickened leaflets. AHA Guidelines for Prevention of Infective Endocarditis, 2007

13. Bacterial Endocarditis • All dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa AHA Guidelines for Prevention of Infective Endocarditis, 2007

14. Bacterial Endocarditis • Antiobiotic regimen administered single dose before the procedure directed against the viridans groups streptococci AHA Guidelines for Prevention of Infective Endocarditis, 2007

15. Regimen for a Dental Procedure AHA Guidelines for Prevention of Infective Endocarditis, 2007

16. Bacterial Endocarditis • Other procedures: • Respiratory tract procedures: • Incision or biopsy of the respiratory mucosa (tonsillectomy and adenoidectomy) • GI, GU Tract and other procedures • Vaginal delivery, hysterectomy, and tattooing • Generally not recommended AHA Guidelines for Prevention of Infective Endocarditis, 2007

17. Pregnancy and RHD • The high rate of teenage pregnancies combined with an endemic prevalence of rheumatic disease in developing countries results in cardiac disease being the most important comorbid state during pregnancy.

18. Pregnancy and RHD • The nature of the underlying cardiac disease needs to be considered in preconception counseling and in the prevention of pregnancy.

19. Pregnancy and RHD • Changes to the heart and blood vessels with pregnancy • Increase in blood volume: • first trimester, the volume of blood increases by 40 to 50%, remains high throughout pregnancy. • Increase in cardiac output: • Increased by 30 to 40% due to the increase in blood volume. Horstkotte D, et al, Herz. 2003 May;28(3):227-39.

20. Pregnancy and RHD • Changes to the heart and blood vessels with pregnancy • Increase in heart rate: • increase by 10 to 15 beats per minute during pregnancy. • Decrease in blood pressure: • may decrease by 10 mmHg Horstkotte D, et al, Herz. 2003 May;28(3):227-39.

21. Pregnancy and RHD • Identify those at greatest risk and institute appropriate surveillance and therapy in these patients

22. Valvular Heart Lesions Associated With High Maternal and/or Fetal Risk During Pregnancy 1. Severe AS with or without symptoms 2. AR with NYHA functional Class III–IV symptoms 3. MS with NYHA functional Class II–IV symptoms 4. MR with NYHA functional Class III–IV symptoms 5. Aortic and/or mitral valve disease resulting in severe pulmonary hypertension (pulmonary pressure .75% of systemic pressures) 6. Aortic and/or mitral valve disease with severe LV dysfunction (EF ,0.40) 7. Mechanical prosthetic valve requiring anticoagulation 8. AR in Marfan syndrome JACC Vol. 32, No. 5; ACC/AHA TASK FORCE REPORT November 1, 1998:1486–588

23. Valvular Heart Lesions Associated With Low Maternaland Fetal Risk During Pregnancy 1. Asymptomatic AS with low mean gradient (,50 mm Hg) in presence of normal LV systolic function (EF .0.50) 2. NYHA functional Class I or II AR with normal LV systolic function 3. NYHA functional Class I or II MR with normal LV systolic function 4. MVP with no MR or with mild to moderate MR and with normal LV systolic function 5. Mild to moderate MS (MVA .1.5 cm2, gradient ,5 mm Hg) without severe pulmonary hypertension 6. Mild to moderate pulmonary valve stenosis JACC Vol. 32, No. 5; ACC/AHA TASK FORCE REPORT November 1, 1998:1486–588

24. Pregnancy and RHD • Low-Risk Lesions • Chronic MR/AR • Well tolerated • New-onset AF or severe hypertension can precipitate hemodynamic deterioration • Acute MR (ruptured chordae)/AR • Pulmonary edema and life threatening cardiac decompensation * Should have operative repair before conception

25. Pregnancy and RHD • Low-Risk Lesions • If with CHF: • Digoxin, duiretics, vasodilators (hydralazine) • Ace-inhibitor contraindicated • Teratogenic • B-blockers: safe • May cause fetal bradycardia/growth retardation

26. Pregnancy and RHD • Moderate-Risk Lesions • Mitral Stenosis • Moderate to severe MS • Hemodynamic deterioration during the 3rd trim or during labor and delivery

27. Pregnancy and RHD • Moderate – severe Mitral stenosis • Physiologic increase in blood volume and HR • Elevated LA pressure • Pulmonary edema • Fetal complications (premature birth, low birth weight, respiratory distress, fetal or neonatal death) • AF • Rapid decompensation • Digoxin, duoretic, blockers • electrocardioversion

28. Pregnancy and RHD • Moderate – severe Mitral stenosis • Surgical repair or PMBV • Percutaneous valvotomy is deferred to the 2nd or 3rd trimester to avoid fetal radiation exposure during the 1st trimester

29. Pregnancy and RHD • Mild Mitral Stenosis and Pregnancy • β-Blockers are safe and well tolerated by both mother and fetus • reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis • inhibiting episodes of paroxysmal atrial fibrillation • may also prevent the formation of left atrial thrombi.

30. Pregnancy and RHD • Mostly (mild MS) can undergo vaginal delivery • If with CHF (mod-severe MS): epidural anesthesia

31. Pregnancy and RHD • High-risk lesion: • Aortic Stenosis • Mild-moderate AS with preserved LV function • Well tolerated • Severe AS (AVA < 1cm2 , mean PG >50mmHg) • 10% risk of maternal morbidity

32. Pregnancy and RHD • Aortic Stenosis • Deterioration late in the 2nd trimester or early in the 3rd trimester • Maximal medical management • Percutaneous balloon valvotomy • Cardiac surgery is needed in about 40%

33. Anticoagulation and Pregnancy • Pregnancy is a hypercoagulable state, and adequate anticoagulation for those with mechanical valves is essential. • 3 most common agents considered for use during pregnancy • Unfractionated heparin (UFH) • Low-molecular-weight-heparin (LMWH) • warfarin Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

34. Anticoagulation and Pregnancy • Maternal and fetal risks and benefits must be carefully explained before choosing the right anticoagulation • When an UFH or LMWH strategy is selected, careful dose monitoring and adjustment are recommended. Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

35. Anticoagulation and Pregnancy • Warfarin • Crosses the placenta and can harm the fetus • Safe during breastfeeding • Warfarin embryopathy(abnormalities of fetal bone and cartilage formation, fetal bleeding) • 4- 10% • Risk highest when given between 6 weeks through 12 weeks Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

36. Anticoagulation and Pregnancy • Warfarin • When given at 2nd-3rd trim • Fetal central nervous system abnormalities • Risk maybe low if given at low dose • 5mg or less per day Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

37. Anticoagulation and Pregnancy • UFH • Does not cross the placenta • Safe for fetus • Use is associated with maternal osteoporosis, hemorrhage, thrombocytopenia, or thrombosis syndrome and high incidence of thromboembolic events Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

38. Anticoagulation and Pregnancy • UFH • Maybe given parenterally or subcutaneously throughout pregnancy • Dose: 17,500 – 20,000 U BID • Titration of dose based on aPTT (2-3 times the control level) Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

39. Anticoagulation and Pregnancy • Low-molecular –weight heparin • Produces a more predictable anticoagulant response than UFH • Less likely to cause thrombosis • Minimal effect on maternal bone densitty Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

40. Anticoagulation and Pregnancy • Low-molecular –weight heparin • Given subcutaneously • Not so many studies on its efficacy on mechanical valves Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

41. Anticoagulation and Pregnancy • Anticoagulation in the pregnant patient can be difficult because of the risk profile associated with each drug regimen. • In planned pregnancies, a careful discussion about the risks and benefits of warfarin, UFH, and LMWH will help the patient and physician involved to choose an anticoagulation strategy. • Unplanned pregnancies: stop warfarin when the pregnancy is discovered and to use UFH or LMWH, at least until after the 12th week. • Elective use of bioprosthetic valves for teens needing surgery Management of Valvular Heart Disease in Pregnancy Patrick T. O'Gara, M.D., F.A.C.C.; Albert E. Raizner, M.D., F.A.C.C.

42. Psychosocial Aspect • Exercise and Sports • Improved cardiovascular fitness • Decreased : obesity, hypertension, and ischemic heart disease • Consider: • Individual • Underlying cardiac pathology • Hemodynamic status • Type of sport or exercise contemplated

43. Exercise and RHD • Type of exercise • Isotonic or isometric • Social or competitive • Contact or non-contact sport • Patients with valvular regurgitation have good tolerance to exercise • Patient with valvular stenosis poor exercise tolerance • Supervised programs of training

44. Sports Clearance • Mild Aortic Stenosis (gradient < 20 mm Hg) • Normal ECG • Normal exercise tolerance • Asymptomatic • No history of exercise related chest pain, syncope, or arrhythmia **Can participate in all competitive sports ACC/American College of Sports Medicine, Sports Clearance for Children with Heart Disease

45. Sports Clearance • Moderate Aortic Stenosis (gradient 21-40 mm Hg) • Mild LVH by echocardiography • No LV strain on ECG • Normal exercise test without ischemia or arrhythmia **Low static/ low to moderate dynamic (Class IA & IB) **Moderate static/ low dynamic (Class IIA) ACC/American College of Sports Medicine, Sports Clearance for Children with Heart Disease

46. Sports Clearance • Severe Aortic Stenosis (gradient > 50 mm Hg) **NO competitive sports ACC/American College of Sports Medicine, Sports Clearance for Children with Heart Disease

47. School/Employment and RHD • Most patients can have regular schooling. • Limit physical activities for selected patients (mod-severe MS/AR/MR) • Most patients can work • They should be given access to employment appropriate to heir physical and intellectual capabilities • Employers: consider only the capacity to perform the given job and not to anticipate future deterioration. • Restriction should exist: when the safety of other is the direct responsibility of the individual with severe RHD

48. Critical steps • Select an adult care physician to provide and coordinate comprehensive care; • Offer reproductive/genetic and career counseling; • Secure health insurance; • Educate adult care providers in managing rheumatic heart disease; • Maintain communication between patients, families and healthcare professionals.

49. Critical steps • The goals of a formal transition program should prepare young adults for the transfer of care to an adult-oriented system. • This transition in care should foster independence and a sense of control over their own care decisions, and thereby improve quality of life, life expectancy, and self-sufficiency.

50. Summary • Rheumatic Heart disease needs Secondary prophylaxis for recurrences • Infective Endocarditis remains a long-term sequalae of RHD and needs to be addressed • Preexisting cardiac valvular lesions should be evaluated with respect to the risk they impart during the stress of pregnancy. • Awareness of major cardiac drug classes that are contraindicated during pregnancy is important for the treatment of hypertension and heart failure during pregnancy. • Anticoagulation during pregnancy presents unique challenges because of its maternal and fetal side effects.