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