pediatric outpatient management of tof post repair n.
Skip this Video
Download Presentation
Pediatric Outpatient Management of ToF Post Repair

Loading in 2 Seconds...

play fullscreen
1 / 36

Pediatric Outpatient Management of ToF Post Repair - PowerPoint PPT Presentation

  • Uploaded on

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.

I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
Download Presentation

PowerPoint Slideshow about 'Pediatric Outpatient Management of ToF Post Repair' - katelin-vaddock

Download Now An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.

- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
pediatric outpatient management of tof post repair

Pediatric Outpatient Management of ToF Post Repair

Andrew S. Mackie, MD, SM

Division of Cardiology

Stollery Children’s Hospital

  • Describe the late complications that can occur in repaired ToF patients
  • Summarize the indications for outpatient investigations in this population
  • Complications post ToF repair
  • Loss to follow-up
  • Existing guidelines
  • Quality metrics
why follow these patients
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

RV dysfunction

LV dysfunction

Congestive heart failure



Sudden death

ToF: Late cardiac complications
  • Pulmonary regurgitation RV volume overload
  • Residual RVOTO
  • Branch pulmonary artery stenosis or hypoplasia
  • Residual VSD
  • Aortic root dilation/ aortic regurgitation
tof non cardiac challenges
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


Isolated PVCs

Non-sustained VT

Sustained VT 10%

Atrial flutter 30%

Atrial fibrillation

AV block


Surgical incisions, e.g. ventriculotomy

Abnormal hemodynamics, e.g. RV volume overload, TR

arrhythmias treatment
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
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


Good hemodynamics:

No restrictions

Poor hemodynamics:

Low intensity activities/sports

Avoid isometric exercise

Walking is OK for everyone!

Eur Heart Journal 2010;31:2915


Low risk if good hemodynamics

High risk if:

Significant residual RV outflow obstruction

Severe TR or PR with RV volume overload


Preconception cardiology counseling re: pregnancy risk

Genetic counseling especially if 22q11 deletion

ACHD care during pregnancy

CHD recurrence risk 4-6%

fetal echocardiogram

follow up

Eur Heart Journal 2010;31:2915

loss to follow up
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


Gurvitz et al. JACC 2013

  • 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
u k data
U.K. Data

Wray et al. Heart 2013

u k data1
U.K. Data

Wray et al. Heart 2013

loss to follow up consequences
Loss to follow-up: Consequences?


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
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


Rhythm, QRS duration



RVOTO, PR, RV size and function

Branch PA size

Residual VSD

Aortic root size and AR

LV function

ToF patients mayalso require:


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