Pediatric Outpatient Management of ToF Post Repair

1. Pediatric Outpatient Management of ToF Post Repair Andrew S. Mackie, MD, SM Division of Cardiology Stollery Children’s Hospital

2. Objectives • Describe the late complications that can occur in repaired ToF patients • Summarize the indications for outpatient investigations in this population

3. Outline • Complications post ToF repair • Loss to follow-up • Existing guidelines • Quality metrics

5. 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

6. Tricuspid regurgitation RV dysfunction LV dysfunction Congestive heart failure Endocarditis Arrhythmias 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

7. 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

8. Arrhythmias What? Isolated PVCs Non-sustained VT Sustained VT 10% Atrial flutter 30% Atrial fibrillation AV block Why? Surgical incisions, e.g. ventriculotomy Abnormal hemodynamics, e.g. RV volume overload, TR

9. 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?

12. 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

13. 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

14. 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

15. Frigiola et al. Circulation 2013;128:1861

16. Follow-up Eur Heart Journal 2010;31:2915

17. Loss to follow-up • How big a problem is this? • At what ages? • Risk factors? • How can we mitigate this problem?

18. 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

19. 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

20. Loss to follow-up during childhood Mackie AS et al. Circulation 2009

21. 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

22. 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

23. Gurvitz et al. JACC 2013

24. Self-reported reasons for gap in care Gurvitz et al. JACC 2013

25. U.K. Data Wray et al. Heart 2013

26. U.K. Data Wray et al. Heart 2013

27. 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

28. Existing guidelines Cong Heart Dis 2006;1:10-26 • Based on “consensus meetings” held at CHOP  Review of literature  Clinical experience of group members

32. 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

33. ToF patients mayalso 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

34. Canadian ACHD guidelines

35. Guidelines vs. Quality Indicators

36. ESC Guidelines