Pediatric outpatient management of TOF post
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Transcript Pediatric outpatient management of TOF post
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?
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:
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:
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:
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
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