Pediatric Outpatient Management of ToF Post Repair
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Pediatric Outpatient Management of ToF
Post RepairAndrew S. Mackie, MD, SM
Division of Cardiology
Stollery Children’s Hospital
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Objectives
Describe the late complications that can occur in repaired ToF patients
Summarize the indications for outpatient investigations in this population
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Outline
1. Complications post ToF repair
2. Loss to follow-up
3. Existing guidelines
4. Quality metrics
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Why follow these patients?
Anticipate and monitor potential complications
Intervene early
Provide patient education
Advice on maintaining a healthy lifestylePhysical activitySmoking cessationContraception and pregnancy
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ToF: Late cardiac complications
Pulmonary regurgitation RV volume overload
Residual RVOTO
Branch pulmonary artery stenosis or hypoplasia
Residual VSD
Aortic root dilation/ aortic regurgitation
Tricuspid regurgitation
RV dysfunction
LV dysfunction
Congestive heart failure
Endocarditis
Arrhythmias
Sudden death
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ToF: Non-cardiac challenges
School and academic difficulties
22q11 deletion (15% of ToF patients)
Insurance and employability
Exercise limitations
Lack of knowledge about their heart
Need for transition and transfer to adult cardiology care
Pregnancy
Genetic implications, need for counseling
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Arrhythmias
What? Isolated PVCsNon-sustained VTSustained VT 10%Atrial flutter 30%Atrial fibrillationAV block
Why?Surgical incisions, e.g. ventriculotomyAbnormal hemodynamics, e.g. RV volume overload,
TR
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Arrhythmias: Treatment
Correct abnormal hemodynamics where possibleE.g. pulmonary valve replacement
Consider intraoperative ablation
Catheter ablation
Consider AICD for high-risk patientsQRS duration >180 msec, non-sustained VT, inducible
VT, previous palliative shunt, RV/LV dysfunction, fibrosis, history of syncope or cardiac arrest
Antiarrhythmic therapy?
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Sudden death
0.15-0.25%/ year
Mechanism presumed to be VT in most cases
Risk stratification remains imperfect
Standard clinical variables: Age at repair, chronological age, prior palliative shunt,
recurrent syncope, PR, residual RVOTO, severe RV enlargement, RV or LV dysfunction, VT, QRS > 180 msec
“Advanced” variables: Positive V stim study (EP lab), PR fraction on MRI
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Exercise
Good hemodynamics:No restrictions
Poor hemodynamics:Low intensity activities/sportsAvoid isometric exercise
Walking is OK for everyone!Eur Heart Journal 2010;31:2915
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Pregnancy
Low risk if good hemodynamics
High risk if:Significant residual RV outflow obstructionSevere TR or PR with RV volume overload
Recommendations:Preconception cardiology counseling re: pregnancy
riskGenetic counseling especially if 22q11 deletionACHD care during pregnancyCHD recurrence risk 4-6%
fetal echocardiogram
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Frigiola et al. Circulation 2013;128:1861
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Follow-up
Eur Heart Journal 2010;31:2915
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Loss to follow-up
How big a problem is this?
At what ages?
Risk factors?
How can we mitigate this problem?
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Only 47% of young adults with moderate or complex CHD were seen at a Canadian ACHD centre within 3 years of graduating from SickKids
Predictors of ACHD attendance were:cardiac surgical procedures in childhoodolder age at last pediatric visitdocumentation in chart of need for follow-up
Reid GJ et al. Pediatrics 2004
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Among a subset (n= 234) who completed questionnaires, predictors of ACHD attendance were:
Having co-morbid conditionsNot using substancesCompliance with dental prophylaxisAttending cardiac appointments without parent or
siblingsDocumentation in chart of need for follow-upReid GJ et al. Pediatrics 2004
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Mackie AS et al. Circulation 2009
Loss to follow-up during childhood
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Case- control study using mixed-methods: Medical records review Structured telephone interviews
Cases: lost to follow-up > 3 years
Controls: matched by year of birth and CHD lesion
Risk factors: No documentation in chart of need for follow-up Lower family income No cath within past 5 years Lack of awareness of the need for follow-up
Mackie et al. Cardiol Young 2011
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992 subjects at 12 U.S. ACHD centersRecruited at 1st presentation to ACHD clinic
Mean age at first gap: 19.9 years
42%: gap in cardiology care > 3 years
8%: gap in care > 10 years
Clinic location influenced gap in careGurvitz et al. JACC 2013
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Gurvitz et al. JACC 2013
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Self-reported reasons for gap in care
CHD severity Most common reasons for gap in care
Moderate CHD Felt well
Did not think needed follow-up
Not receiving any medical care
Changed or lost insurance
Moved
Gurvitz et al. JACC 2013
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U.K. Data
Wray et al. Heart 2013
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U.K. Data
Wray et al. Heart 2013
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Loss to follow-up: Consequences?
Colorado: 158 adults with moderate-complex CHD 63% had a lapse in care of > 2 years since
leaving pediatric center Most common cited reason: patient had
been told “no need for follow-up” (32%) Those with lapse of care more likely to
require surgical or catheter intervention within 6 months (OR 3.1, p= 0.003)
#1 re-intervention was PVRYeung et al. Int J Cardiol 2008
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Existing guidelines
Cong Heart Dis 2006;1:10-26 Based on “consensus meetings” held at CHOP
Review of literature
Clinical experience of group members
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All ToF patients should have (at a minimum):A thorough clinical assessmentECG
Rhythm, QRS duration
CXREchocardiogram
RVOTO, PR, RV size and function Branch PA size Residual VSD Aortic root size and AR LV function
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ToF patients may also require:MRI
PA size, PR fraction, RV size and functionCT if contraindication to MRIExercise testing
Functional capacity, exertion-related arrhythmiasHolter monitor or event recorderLung perfusion scanCardiac catheterizationEP study
Diagnostic intervention of flutter, VT Risk stratification for sudden death
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Canadian ACHD guidelines
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Guidelines vs. Quality IndicatorsClinical Guidelines Quality Indicators
Comprehensive: Cover virtually all aspects of care for a condition
Targeted: Apply to specific clinical circumstances where there is evidence that outcomes are expected to be improved
Prescriptive: Intended to influence provider behavior prospectively at the individual patient level
Observational: Measure provider behavior at an aggregate level; applied retrospectively
Flexible: Intentionally leave room for clinical judgment and interpretation
Precise: Precise language that can be applied systematically to medical records data to ensure comparability
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ESC Guidelines