Pediatric Rheumatic: Issues on Moving to Adult Care Virginia C
Mappala, MD Pediatric Cardiology
Slide 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.
Slide 3
Issues? Duration of Secondary prophylaxis (Benzathine Injection
every 21 days) Until when?...18?...21?..40?...for life?
Pregnancy/Lactation and Anticoagulation Psychosocial aspects
Exercise and sports; employment Bacterial Endocarditis Need for
antibiotic prophylaxis
Slide 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
Slide 5
Type Duration after last attack Evidence 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 needed 1C Rheumatic fever with carditis
but no residual heart disease (no valvular disease); mild MR 10
years or until age 25 years (whichever is longer) 1C Rheumatic
fever without carditis 5 years or until age 21 years (whichever is
longer) 1C AHA Duration of Secondary Prophylaxis for Rheumatic
Fever, 2010
Slide 6
Secondary Prophylaxis Consider factors: Patients risk of
acquiring strep infection Anticipated recurrence rate for infection
Consequences of recurrence
Slide 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
Slide 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
Slide 9
Bacterial Endocarditis During the past 50 years AHA guidelines
recommended antimicrobial prophylaxis to prevent IE in patients
with underlying cardiac conditions
Slide 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
Slide 11
Bacterial Endocarditis RHD with HIGHEST risk: Prosthetic valves
or valves repaired with prosthetic material Previous endocarditis
Associated with congenital heart disease AHA Guidelines for
Prevention of Infective Endocarditis, 2007
Slide 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
Slide 13
Bacterial Endocarditis AHA Guidelines for Prevention of
Infective Endocarditis, 2007 All dental procedures that involve
manipulation of gingival tissue or the periapical region of teeth
or perforation of the oral mucosa
Slide 14
Bacterial Endocarditis AHA Guidelines for Prevention of
Infective Endocarditis, 2007 Antiobiotic regimen administered
single dose before the procedure directed against the viridans
groups streptococci
Slide 15
Regimen for a Dental Procedure SituationAgentRegimen: Single
Dose 30- 60min before the procedure AdultsChildren
OralAmoxicillin2g50mg/kg Unable to take oral medication Ampicillin
or Cefazolin or Ceftriaxone 2g IM or IV50mg/kg IM or IV Allergic to
penicillin or ampicillin oral Cefalxin or Clindamycin or
Azithromycin or Clarithromycin 2g 600mg 500mg 50mg/kg 20mg/kg
15mg/kg Allergic to penicillin or ampicillin unable to take oral
meds Cefazolinor Ceftriaxone or Clindamycin 1g IM or IV 600mg IM or
IV 50mg/kg IM or IV 20mg/kg IM or IV AHA Guidelines for Prevention
of Infective Endocarditis, 2007
Slide 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
Slide 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.
Slide 18
Pregnancy and RHD o The nature of the underlying cardiac
disease needs to be considered in preconception counseling and in
the prevention of pregnancy.
Slide 19
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.
Slide 20
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
Pregnancy and RHD Horstkotte D, et al, Herz. 2003
May;28(3):227-39.
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Pregnancy and RHD Identify those at greatest risk and institute
appropriate surveillance and therapy in these patients
Slide 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 IIIIV symptoms 3. MS with NYHA
functional Class IIIV symptoms 4. MR with NYHA functional Class
IIIIV 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, 1998:1486588
Slide 23
Valvular Heart Lesions Associated With Low Maternal and 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, 1998:1486588
Slide 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
Slide 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
Slide 26
Pregnancy and RHD Moderate-Risk Lesions Mitral Stenosis
Moderate to severe MS Hemodynamic deterioration during the 3 rd
trim or during labor and delivery
Slide 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
Slide 28
Pregnancy and RHD Moderate severe Mitral stenosis Surgical
repair or PMBV Percutaneous valvotomy is deferred to the 2 nd or 3
rd trimester to avoid fetal radiation exposure during the 1 st
trimester
Slide 29
Pregnancy and RHD Mild Mitral Stenosis and Pregnancy -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.
Slide 30
Pregnancy and RHD Mostly (mild MS) can undergo vaginal delivery
If with CHF (mod-severe MS): epidural anesthesia
Slide 31
Pregnancy and RHD High-risk lesion: Aortic Stenosis
Mild-moderate AS with preserved LV function Well tolerated Severe
AS (AVA 50mmHg) 10% risk of maternal morbidity
Slide 32
Pregnancy and RHD Aortic Stenosis Deterioration late in the 2
nd trimester or early in the 3 rd trimester Maximal medical
management Percutaneous balloon valvotomy Cardiac surgery is needed
in about 40%
Slide 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.
Slide 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.
Slide 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.
Slide 36
Anticoagulation and Pregnancy Warfarin When given at 2 nd -3 rd
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.
Slide 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.
Slide 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.
Slide 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.
Slide 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.
Slide 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.
Slide 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
Slide 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
Slide 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
Slide 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
Slide 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
Slide 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
Slide 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.
Slide 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.
Slide 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.