Pediatric Outpatient
Management of ToF
Post Repair
Andrew 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 lifestyle
 Physical activity
 Smoking cessation
 Contraception and pregnancy
ToF: Late cardiac complications
 Tricuspid
regurgitation
 Pulmonary regurgitation RV volume
overload
 Residual RVOTO
 RV dysfunction
 LV
dysfunction
 Branch pulmonary artery stenosis
or hypoplasia
 Congestive heart failure
 Residual VSD
 Endocarditis
 Aortic root dilation/ aortic regurgitation
 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?
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Isolated PVCs
Non-sustained VT
Sustained VT
Atrial flutter
Atrial fibrillation
AV block
10%
30%
 Why?
 Surgical incisions, e.g. ventriculotomy
 Abnormal hemodynamics, e.g. RV volume overload, TR
Arrhythmias: Treatment
 Correct abnormal hemodynamics where possible
 E.g. pulmonary valve replacement
 Consider intraoperative ablation
 Catheter ablation
 Consider AICD for high-risk patients
 QRS 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/sports
 Avoid 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 obstruction
 Severe TR or PR with RV volume overload
 Recommendations:



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Preconception cardiology counseling re: pregnancy risk
Genetic counseling especially if 22q11 deletion
ACHD care during pregnancy
CHD 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 childhood
 older age at last pediatric visit
 documentation 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:
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Having co-morbid conditions
Not using substances
Compliance with dental prophylaxis
Attending cardiac appointments without parent or siblings
Documentation in chart of need for follow-up
Reid GJ et al. Pediatrics 2004
Loss to follow-up during childhood
Mackie AS et al. Circulation 2009
 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:
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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 centers
 Recruited 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 care
Gurvitz 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 PVR
Yeung 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 assessment
 ECG
 Rhythm, QRS duration
 CXR
 Echocardiogram
 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 function
 CT if contraindication to MRI
 Exercise testing
 Functional capacity, exertion-related arrhythmias
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Holter monitor or event recorder
Lung perfusion scan
Cardiac catheterization
EP study
 Diagnostic intervention of flutter, VT
 Risk stratification for sudden death
Canadian ACHD guidelines
Guidelines vs. Quality Indicators
Clinical 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