Pediatric rheumatic:issues on moving to adult care 20mins

Pediatric Rheumatic: Issues on Moving to Adult Care

Virginia C Mappala, MDPediatric CardiologyI would like to thank the organizer and council chair for giving me this task, I really have no choice, who can say no to a good friend and mentor I have no choice. Honestly, I really have difficulty formulating my lecture until the last minute. Rheumatic Heart Disease is one of the major causes of cardiovascular disease among children and young adults. It remains to be the most common acquired heart disease in developing countries. The goal of a formal transition program is to prepare young adults for transfer of care to an adult-oriented system. Care of the adolescent with RHD should include coordinated comprehensive care, with access to adequate financing if possible, education of physicians and other health care providers, and a balance communication between all the parties involved. Adolescents with RHD may suffer emotional and behavioral problems, a diminished quality of life and be subject to the consequences of misinformation.

1Rheumatic Heart Diseasemost common acquired heart disease5 15 yearsHigh morbidity and mortality among adolescent and young adultCrippling: long and progressively damaging series of events going on from childhood, through adolescence, to adult life.It is unquestionably one of the most crippling of all diseases and the greatest single cause of deathamong adults. I wish to stress this point, for you must appreciate that any attack of rheumatic infection is not an isolated incident in a person's life, but one of a long and progressively damaging series of events going on fromchildhood, through adolescence, to adult lifeThe population of guch is more of those underwent treatment and medical mgt.adolescent not the same but similar in some aspects, the burden of rhd are to those that were diagnosed later in life as compared to young child with rf2Issues?Duration of Secondary prophylaxis (Benzathine Injection every 21 days)Until when?...18?...21?..40?...for life?Pregnancy/Lactation and AnticoagulationPsychosocial aspectsExercise and sports; employmentBacterial EndocarditisNeed for antibiotic prophylaxisSecondary Prophylaxis for RHDAn absolute must to reduce morbidity and mortality in rheumatic individualsEffective in reducing and eliminating recurrencesLong-acting penicillin injection is more effective than oral prophylaxisLifetime prophylaxis important in RHDBecause oral is lack of compliance Lifetime prophylaxis impt in those with established RHD

4TypeDuration after last attackEvidence rating*Rheumatic fever with carditis and residual heart disease (persistent valvular disease)10 years or until age 40 - 45 years (whichever is longer); lifetime prophylaxis may be needed1CRheumatic fever with carditis but no residual heart disease (no valvular disease); mild MR10 years or until age 25 years (whichever is longer)1CRheumatic fever without carditis5 years or until age 21 years (whichever is longer)1C

AHA Duration of Secondary Prophylaxis for Rheumatic Fever, 2010

Secondary ProphylaxisConsider factors:Patients risk of acquiring strep infectionAnticipated recurrence rate for infection Consequences of recurrenceConsidering the difficulties for maintaining lifetime prop, exceptions can be made especially for older patients6Secondary ProphylaxisAdolescents and Adult at INCREASED risk of recurrence:Parents of young childrenSchool teachersMedical and paramedical personnelMilitary cadets and service men

These people need prophylaxis over a longer period of time7Secondary ProphylaxisAdolescents and Adult at GREATEST risk of recurrence:Established RHDRecent attack of RF (within the last 3 yrs.)Multiple attacks in the pastChildren and adolescents in the crowded homeUndergone valvar surgery for RHD

These people need prophylaxis over a longer period of time8Bacterial Endocarditis

During the past 50 years AHA guidelines recommended antimicrobial prophylaxis to prevent IE in patients with underlying cardiac conditions

However several studies have been made, questioning the efficacy of giving prophylaxis versus its risks of having resistance to the antibiotics. The AHA no longer recommends prophylaxis for infective endocarditis in most patients with rheumatic heart disease. The exceptions are patients with prosthetic valves or valves repaired with prosthetic material, patients with previous endocarditis or specific forms of congenital heart disease, and cardiac transplant recipients who develop cardiac valvulopathy. In these patients, an agent other than penicillin should be used to prevent infective endocarditis, because alpha-hemolytic streptococci have likely developed resistance to penicillin.

9Bacterial Endocarditis

Steckelberg and WilsonLifetime risk of acquisition of IE ranged from 5 per 100 000 patient-years in the general population with no known cardiac conditions2160 per 100 000 patient-years in patients with prosthetic cardiac valves52 per 100 000 = MVP, (+) MRSteckelberg and Wilson reported the lifetime risk of acquisition of IE, which ranged from 5 per 100 000patient-years in the general population with no known cardiacconditions to 2160 per 100 000 patient-years in patients who underwent replacement of an infected prosthetic cardiac valve. In that study,90 the risk of IE per 100 000 patient-years was 4.6 in patients with MVP without an audible cardiac murmur and 52 in patients with MVP with an audible murmurof mitral regurgitation. Per 100 000 patient-years, the lifetimerisk (380 to 440) for RHD was similar to that (308 to 383) forpatients with a mechanical or bioprosthetic cardiac valve. Thehighest lifetime risks per 100 000 patient-years were asfollows: cardiac valve replacement surgery for native valveIE, 630; previous IE, 740; and prosthetic valve replacementdone in patients with prosthetic valve endocarditis, 2160.10Bacterial Endocarditis

RHD with HIGHEST risk:Prosthetic valves or valves repaired with prosthetic materialPrevious endocarditis Associated with congenital heart diseaseAHA Guidelines for Prevention of Infective Endocarditis, 200711Bacterial Endocarditis

RHD with Moderate-risk:Mitral stenosis and calcific aortic stenosisMitral valve prolapse with regurgitation and with or without thickened leaflets.

AHA Guidelines for Prevention of Infective Endocarditis, 200712Bacterial Endocarditis

AHA Guidelines for Prevention of Infective Endocarditis, 2007All dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosaTransient bacteremia is common with the manipulation of the teeth and periodontal tissue 13Bacterial Endocarditis

AHA Guidelines for Prevention of Infective Endocarditis, 2007Antiobiotic regimen administered single dose before the procedure directed against the viridans groups streptococciTransient bacteremia is common with the manipulation of the teeth and periodontal tissue 14Regimen for a Dental ProcedureSituationAgent Regimen: Single Dose 30-60min before the procedureAdultsChildrenOralAmoxicillin2g50mg/kgUnable to take oral medicationAmpicillin orCefazolin or Ceftriaxone2g IM or IV50mg/kg IM or IVAllergic to penicillin or ampicillin oralCefalxin orClindamycin orAzithromycin or Clarithromycin2g600mg500mg50mg/kg20mg/kg15mg/kgAllergic to penicillin or ampicillin unable to take oral medsCefazolinor Ceftriaxone orClindamycin1g IM or IV

600mg IM or IV50mg/kg IM or IV

20mg/kg IM or IVAHA Guidelines for Prevention of Infective Endocarditis, 2007Transient bacteremia is common with the manipulation of the teeth and periodontal tissue 15Bacterial Endocarditis

Other procedures:Respiratory tract procedures:Incision or biopsy of the respiratory mucosa (tonsillectomy and adenoidectomy)GI, GU Tract and other proceduresVaginal delivery, hysterectomy, and tattooingGenerally not recommended

AHA Guidelines for Prevention of Infective Endocarditis, 2007Transient bacteremia is common with the manipulation of the teeth and periodontal tissue 16Pregnancy 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. 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. Of crucial importance in the management of the pregnant cardiac patient is to be able to identify those at greatest risk and institute appropriate surveillance and therapy in these patients. Of all of the rheumatic valvular lesions, mitral stenosis is not only the most frequent but also the one most likely to lead to a potentially serious outcome. In Africa, it is not uncommon for previously occult mitral stenosis to be discovered for the first time during pregnancy. This is easily understood when one considers the interaction between the physiological cardiovascular adjustments to pregnancy and the hemodynamics of mitral stenosis. The availability of new therapies has engendered some controversy even in the optimal management of the nonpregnant patient with mitral stenosis.23 This controversy is compounded in the pregnant patient because the risks to the fetus of drug therapy, radiation exposure during percutaneous mitral balloon valvuloplasty, or anesthesia with or without cardiopulmonary bypass during surgical commissurotomy need to be considered.

17Pregnancy and RHDThe nature of the underlying cardiac disease needs to be considered in preconception counseling and in the prevention of pregnancy. 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. Pregnancy and RHD

Horstkotte D, et al, Herz. 2003 May;28(3):227-39. During pregnancy, changes occur to the heart and blood vessels that add stress on a womans body and increase the workload of the heart. These changes include:

19Changes 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

Pregnancy and RHD

Horstkotte D, et al, Herz. 2003 May;28(3):227-39. Blood pressure may decrease during pregnancy due to hormone changes and because more of your blood is directed toward the uterus. Most of the time, there are no symptoms of blood pressure changes and no treatment is required. Your health care provider will be monitoring your blood pressure during your prenatal appointments and can tell you if you need to be concerned about changes in your blood pressure.20Pregnancy and RHD

Identify those at greatest risk and institute appropriate surveillance and therapy in these patients

Of crucial importance in the management of the pregnant cardiac patient is to be able to identify those at greatest risk and institute appropriate surveillance and therapy in these patients. Of all of the rheumatic valvular lesions, mitral stenosis is not only the most frequent but also the one most likely to lead to a potentially serious outcome. In Africa, it is not uncommon for previously occult mitral stenosis to be discovered for the first time during pregnancy. This is easily understood when one considers the interaction between the physiological cardiovascular adjustments to pregnancy and the hemodynamics of mitral stenosis. The availability of new therapies has engendered some controversy even in the optimal management of the nonpregnant patient with mitral stenosis.23 This controversy is compounded in the pregnant patient because the risks to the fetus of drug therapy, radiation exposure during percutaneous mitral balloon valvuloplasty, or anesthesia with or without cardiopulmonary bypass during surgical commissurotomy need to be considered.

21Valvular Heart Lesions Associated With High Maternal and/or Fetal Risk During Pregnancy

1. Severe AS with or without symptoms2. AR with NYHA functional Class IIIIV symptoms3. MS with NYHA functional Class IIIV symptoms4. MR with NYHA functional Class IIIIV symptoms5. Aortic and/or mitral valve disease resulting in severe pulmonaryhypertension (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 anticoagulation8. AR in Marfan syndromeJACC Vol. 32, No. 5; ACC/AHA TASK FORCE REPORT November 1, 1998:1486588Valvular Heart Lesions Associated With Low Maternaland Fetal Risk During Pregnancy

1. Asymptomatic AS with low mean gradient (,50 mm Hg) in presence ofnormal LV systolic function (EF .0.50)2. NYHA functional Class I or II AR with normal LV systolic function3. NYHA functional Class I or II MR with normal LV systolic function4. MVP with no MR or with mild to moderate MR and with normal LVsystolic function5. Mild to moderate MS (MVA .1.5 cm2, gradient ,5 mm Hg) withoutsevere pulmonary hypertension6. Mild to moderate pulmonary valve stenosisJACC Vol. 32, No. 5; ACC/AHA TASK FORCE REPORT November 1, 1998:1486588Pregnancy and RHDLow-Risk LesionsChronic MR/ARWell tolerated New-onset AF or severe hypertension can precipitate hemodynamic deteriorationAcute MR (ruptured chordae)/ARPulmonary edema and life threatening cardiac decompensation* Should have operative repair before conception

Optimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

24Pregnancy and RHDLow-Risk LesionsIf with CHF:Digoxin, duiretics, vasodilators (hydralazine)Ace-inhibitor contraindicatedTeratogenicB-blockers: safeMay cause fetal bradycardia/growth retardation

Optimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

25Pregnancy and RHDModerate-Risk LesionsMitral StenosisModerate to severe MSHemodynamic deterioration during the 3rd trim or during labor and deliveryOptimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

26Pregnancy and RHDModerate severe Mitral stenosisPhysiologic increase in blood volume and HR Elevated LA pressure Pulmonary edemaFetal complications (premature birth, low birth weight, respiratory distress, fetal or neonatal death)AF Rapid decompensationDigoxin, duoretic, blockerselectrocardioversion

Optimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

27Pregnancy and RHDModerate severe Mitral stenosisSurgical repair or PMBVPercutaneous valvotomy is deferred to the 2nd or 3rd trimester to avoid fetal radiation exposure during the 1st trimester Optimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

28Pregnancy and RHDMild Mitral Stenosis and Pregnancy-Blockers are safe and well tolerated by both mother and fetusreducing heart rate significantly ameliorate the hemodynamics of mitral stenosisinhibiting episodes of paroxysmal atrial fibrillationmay also prevent the formation of left atrial thrombi.

Optimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

29Pregnancy and RHDMostly (mild MS) can undergo vaginal deliveryIf with CHF (mod-severe MS): epidural anesthesiaOptimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

30Pregnancy and RHDHigh-risk lesion:Aortic StenosisMild-moderate AS with preserved LV functionWell tolerated Severe AS (AVA < 1cm2 , mean PG >50mmHg)10% risk of maternal morbidityOptimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

31Pregnancy and RHDAortic StenosisDeterioration late in the 2nd trimester or early in the 3rd trimesterMaximal medical managementPercutaneous balloon valvotomyCardiac surgery is needed in about 40%Optimal management of the already pregnant patient with compensated mitral stenosis requires careful assessment of the risk-benefit ratio to mother and fetus of standard pharmacological therapy (diuretics and -blockers) versus percutaneous balloon valvuloplasty. -Blockers are safe and well tolerated by both mother and fetus and by reducing heart rate significantly ameliorate the hemodynamics of mitral stenosis. -Blockers not only may have a beneficial hemodynamic effect but, by inhibiting episodes of paroxysmal atrial fibrillation, may also prevent the formation of left atrial thrombi.23 The vast majority of patients in our experience can be carried successfully through pregnancy and the puerperium by judicious use of these drugs combined with a diuretic in those with accompanying shortness of breath.

32Anticoagulation and PregnancyPregnancy is a hypercoagulable state, and adequate anticoagulation for those with mechanical valves is essential. 3 most common agents considered for use during pregnancyUnfractionated 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.Considerable differences of opinion on the management of anticoagulants during pregnancy33Anticoagulation and PregnancyMaternal and fetal risks and benefits must be carefully explained before choosing the right anticoagulationWhen 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.Considerable differences of opinion on the management of anticoagulants during pregnancy34Anticoagulation and PregnancyWarfarinCrosses the placenta and can harm the fetusSafe during breastfeedingWarfarin embryopathy (abnormalities of fetal bone and cartilage formation, fetal bleeding)4- 10%Risk highest when given between 6 weeks through 12 weeksManagement 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.Considerable differences of opinion on the management of anticoagulants during pregnancy35Anticoagulation and PregnancyWarfarinWhen given at 2nd-3rd trimFetal central nervous system abnormalitiesRisk maybe low if given at low dose 5mg or less per dayManagement 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.Considerable differences of opinion on the management of anticoagulants during pregnancy36Anticoagulation and PregnancyUFHDoes not cross the placentaSafe for fetusUse 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.Considerable differences of opinion on the management of anticoagulants during pregnancy37Anticoagulation and PregnancyUFHMaybe given parenterally or subcutaneously throughout pregnancyDose: 17,500 20,000 U BIDTitration 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.Considerable differences of opinion on the management of anticoagulants during pregnancy38Anticoagulation and PregnancyLow-molecular weight heparinProduces a more predictable anticoagulant response than UFHLess likely to cause thrombosisMinimal effect on maternal bone densittyManagement 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.Considerable differences of opinion on the management of anticoagulants during pregnancy39Anticoagulation and PregnancyLow-molecular weight heparinGiven subcutaneously Not so many studies on its efficacy on mechanical valvesManagement 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.Considerable differences of opinion on the management of anticoagulants during pregnancy40Anticoagulation and PregnancyAnticoagulation 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 surgeryManagement 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.In summary: Considerable differences of opinion on the management of anticoagulants during pregnancy41Psychosocial AspectExercise and SportsImproved cardiovascular fitness Decreased : obesity, hypertension, and ischemic heart diseaseConsider: Individual Underlying cardiac pathology Hemodynamic status Type of sport or exercise contemplatedExercise and RHDType of exerciseIsotonic or isometricSocial or competitiveContact or non-contact sportPatients with valvular regurgitation have good tolerance to exercisePatient with valvular stenosis poor exercise tolerance Supervised programs of training

Sports ClearanceMild 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 sportsACC/American College of Sports Medicine, Sports Clearance for Children with Heart DiseaseSports ClearanceModerate 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 Disease45Sports ClearanceSevere Aortic Stenosis (gradient > 50 mm Hg)**NO competitive sportsACC/American College of Sports Medicine, Sports Clearance for Children with Heart DiseaseSchool/Employment and RHDMost patients can have regular schooling.Limit physical activities for selected patients (mod-severe MS/AR/MR)Most patients can workThey 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

Critical stepsSelect 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.

Critical stepsThe 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.

SummaryRheumatic Heart disease needs Secondary prophylaxis for recurrencesInfective Endocarditis remains a long-term sequalae of RHD and needs to be addressedPreexisting 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.

THANK YOU AND GOOD DAY!