Pediatric Outpatient Management of ToF Post Repair
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Transcript of Pediatric Outpatient Management of ToF Post Repair
Pediatric Outpatient Management of ToF
Post RepairAndrew S. Mackie, MD, SM
Division of Cardiology
Stollery Children’s Hospital
Objectives
Describe the late complications that can occur in repaired ToF patients
Summarize the indications for outpatient investigations in this population
Outline
1. Complications post ToF repair
2. Loss to follow-up
3. Existing guidelines
4. Quality metrics
Why follow these patients?
Anticipate and monitor potential complications
Intervene early
Provide patient education
Advice on maintaining a healthy lifestylePhysical activitySmoking cessationContraception and pregnancy
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
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
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
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?
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
Exercise
Good hemodynamics:No restrictions
Poor hemodynamics:Low intensity activities/sportsAvoid isometric exercise
Walking is OK for everyone!Eur Heart Journal 2010;31:2915
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
Frigiola et al. Circulation 2013;128:1861
Follow-up
Eur Heart Journal 2010;31:2915
Loss to follow-up
How big a problem is this?
At what ages?
Risk factors?
How can we mitigate this problem?
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
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
Mackie AS et al. Circulation 2009
Loss to follow-up during childhood
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
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
Gurvitz et al. JACC 2013
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
U.K. Data
Wray et al. Heart 2013
U.K. Data
Wray et al. Heart 2013
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
Existing guidelines
Cong Heart Dis 2006;1:10-26 Based on “consensus meetings” held at CHOP
Review of literature
Clinical experience of group members
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
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
Canadian ACHD guidelines
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
ESC Guidelines