Transcript Pediatric rheumatic:issues on moving to adult care 20mins

Pediatric Rheumatic:
Issues on Moving to
Adult Care
Virginia C Mappala, MD
Pediatric Cardiology
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.
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
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
AHA Duration of Secondary
Prophylaxis for Rheumatic Fever, 2010
Type
Rheumatic fever with
carditis and residual
heart disease (persistent
valvular disease)
Duration after last
attack
10 years or until age 40 - 45
years (whichever is longer);
lifetime prophylaxis may be
needed
Evidence
rating*
1C
Rheumatic fever with
10 years or until age 25 years 1C
carditis but no residual (whichever is longer)
heart disease (no valvular
disease); mild MR
Rheumatic fever without 5 years or until age 21 years
carditis
(whichever is longer)
1C
Secondary Prophylaxis
Consider factors:
Patient’s risk of acquiring strep
infection
Anticipated recurrence rate for
infection
Consequences of recurrence
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
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
Bacterial Endocarditis
 During the past 50 years AHA
guidelines recommended
antimicrobial prophylaxis to
prevent IE in patients with
underlying cardiac conditions
Bacterial Endocarditis
Steckelberg and Wilson
Lifetime risk of acquisition of IE
 ranged from 5 per 100 000 patientyears 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
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
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
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
Bacterial Endocarditis
• Antiobiotic regimen
administered single dose
before the procedure directed
against the viridans groups
streptococci
AHA Guidelines for Prevention of Infective Endocarditis, 2007
Regimen for a Dental Procedure
Situation
Agent
Regimen: Single Dose 3060min before the procedure
Adults
Children
Oral
Amoxicillin
2g
50mg/kg
Unable to take
oral medication
Ampicillin or
Cefazolin or
Ceftriaxone
2g IM or IV
50mg/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
50mg/kg IM or IV
600mg IM or IV
20mg/kg IM or IV
AHA Guidelines for Prevention of Infective Endocarditis, 2007
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
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.
Pregnancy and RHD
o The nature of the underlying
cardiac disease needs to be
considered in preconception
counseling and in the
prevention of pregnancy.
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.
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.
Pregnancy and RHD
Identify those at greatest risk and
institute appropriate surveillance
and therapy in these patients
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
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, 1998:1486–588
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
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
Pregnancy and RHD
Moderate-Risk Lesions
Mitral Stenosis
Moderate to severe MS
Hemodynamic deterioration during
the 3rd trim or during labor and
delivery
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
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
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.
Pregnancy and RHD
Mostly (mild MS) can undergo
vaginal delivery
If with CHF (mod-severe MS):
epidural anesthesia
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
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%
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.
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.
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.
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.
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.
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.
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.
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.
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.
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
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
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
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
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
School/Employment and RHD
 Most patients can have regular schooling.
 Limit physical activities for selected patients (modsevere 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
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.
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.
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.
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