1. “I Need This Form Filled Out”: Pediatric Sports Physical Assessment Aaron T Dorfman, MD Pediatric Cardiology New Jersey Section The Children’s Hospital of Philadelphia

2. Financial Disclosures None

3. Learning Objectives To review the cardiac conditions associated with sudden cardiac death in children and adolescents. To review a practical approach to screening children and teens by careful history and physical examination.

6. Incidence of Sudden Cardiac Death Incidence of cardiac arrest in children and teens is 1- 6 :100,000 per year There is a trend towards increasing incidence of these events over time. Less than 100 school-aged competitive athletes will die per year in the United States Pediatric Sudden Cardiac Arrest, Pediatrics 2012; 129, e1094

7. Cardiac Causes of Sudden Death in 1049 Athletes Maron, BJ, et al. Sudden Deaths in Young Competitive Athletes. Circulation 2009, 119:1085-1092

8. Hypertrophic Cardiomyopathy Prevalence is 1:500 in the US Natural history: 2-8% incidence per year of sudden death in the adolescent population. ECG is abnormal in 75-95% of patients Echo is usually diagnostic Recommendations: exclusion from competitive sports

9. ECG in HCM Deep S waves in V1,V2 T wave inversion in inferior and lateral leads Tall R waves in V5, V6

10. Hypertrophic Cardiomyopathy

11. Symptoms: chest pain, syncope during exertion or immediately following exercise, or palpitations. First symptom may be sudden death. Physical findings: – heart murmur of left ventricular outflow tract obstruction or mitral regurgitation. – Murmur decreases from standing to squatting. – Then increases dramatically from squatting to standing

12. Hypertrophic Cardiomyopathy Murmur Decreases Murmur Increases Decreased preload increases the murmur

13. Congenital Coronary Abnormalities There are several normal variations of coronary anatomy 2 Major potentially dangerous variants – Origin of the LCA from the right sinus of Valsalva – Origin of the RCA from the left sinus of Valsalva Incidence of anomalous RCA is much more common Anomalous LCA is ~ 6 times more lethal

14. Congenital Coronary Anomalies Normal Coronaries L P

15. Images courtesy of Matthew Harris, MD

16. Anomalous Coronaries Usually only identifiable by echo Surgical repair is possible, but controversial Exercise restriction is controversial Large registry currently enrolling – more info TBD in next 5-10 years

17. Long QT Syndrome Defined as prolonged corrected QT measurement Borderline for males 450-470 ms Borderline for females 460-480 ms Infants may be normal < 480 ms Inherited as autosomal dominant At least 12 different associated genotypes. - Sensitivity of current genotyping is ~75% ECG may be normal in 10 – 15% of known carriers of one of the associated gene mutations

18. The corrected QT interval

19. Long QT Evolving exercise restrictions Probably safe with QTc <500ms Previously strict exercise restrictions for all patients with condition Recent evidence suggests lower incidence of adverse events – No deaths and 1 appropriate ICD shock in 650 athlete- years with known LQT Johnson, J; Ackerman MJ. Br J Sports Med 2013; 47:28-33.

20. Aortic Rupture Trauma Marfan Syndrome Loeys-Dietz Syndrome Ehlers-Danlos Syndrome – vascular type IV Familial aortic root enlargement Bicuspid aortic valve Turner Syndrome

21. NJ Athlete Evaluation “Student-Athlete Cardiac Assessment Professional Development Module” – Released Summer 2015 – 43 minute video required for all healthcare providers to view in order to sign Annual sports clearance forms for NJ schools – Developed at request of NJ state legislature – This lecture does not qualify as a substitute – http://www.state.nj.us/education/students/safety/he alth/services/athlete/PDModule.shtml

22. New Jersey Mandatory Module Emphasizes a comprehensive history and physical examination Is a starting point for conversations about cardiac risk in healthy teenaged athletes Identifies some children with cardiac risk factors that may need additional screening Is sufficiently broad that it may lead to over- referral of healthy teens.

25. Chest Pain Chest pain in children and teens is NEVER CARDIAC… Except when it is.

26. Chest pain as outpatient 10 years of data from outpatients presenting to Childrens Hospital Boston 3700 patients included and followed over average of 4 years 17,800 patient years of data 37 cardiac diagnoses 0 patient deaths Saleeb, SF et al. Effectiveness of screening for Life Threatening Chest Pain in Children. Pediatrics 2011; 128; e1062

27. 3700 patients with chest pain Saleeb, SF et al. Effectiveness of screening for Life Threatening Chest Pain in Children. Pediatrics 2011; 128; e1062 Musculoskeletal (n= 1345, 36%

28. History: Chest pain Benign – Frequently at rest – “Random” times of the day – Reproducible with palpation – Changes with deep breath or position – Varying locations throughout chest

29. History: Chest Pain May be cardiac: – New, associated with fever – Only at peak exertion – Radiates to neck, shoulder or back – Severe, crushing, pain. “Like an elephant sitting on my chest” – Looks unwell with pain – Removes themselves from competition due to pain

31. Syncope Benign (Vasodepressor) syncope – Typically with change in position such as rapid standing from sitting or laying down – Prolonged standing – Hot showers – Prodrome of visual changes, tinnitus, numbness/tingling in hands and feet – Brief period of LOC with no signs of seizure – Not with sports

32. Syncope Atypical – should refer – ANY exertional or post-exertional syncope – Prolonged loss of consciousness – Sudden onset with no prodrome – Episodes increasing in frequency or severity

33. Palpitations Very common concern for children and teens Generally benign Often felt after physical activity Rarely a concern when only identified by parent Concern for arrhythmia if: – Sudden on/off, dizzy or unwell, prolonged

34. Shortness of Breath Vague symptom of many etiologies Most frequently due to pulmonary etiology or poor physical conditioning May be a sign of cardiac disease if: – Worsening over time with physical activity in the absence of pulmonary etiology – Other signs of cardiac disease

35. Family History Is there a first degree relative with: – Cardiomyopathy – Long QT (or short) – Brugada Syndrome – Marfan Syndrome – Arrhythmogenic Right Ventricular Cardiomyopathy – Sudden, unexplained cardiac arrest

36. Physical Exam Vital signs – Hypertension, tachycardia Stigmata of syndrome Abnormal cardiac murmur Diminished pulses

37. Role of Primary Care Providers in Screening Patients for Cardiac Risk Annual physical examination w/ blood pressure Annual updates of family history regarding sudden death and inherited cardiac disease Assessment for: - new cardiac symptoms chest pain, syncope, palpitations, change in exercise tolerance - change in cardiovascular exam If concerns, refer to Pediatric Cardiology for eval –Don’t send for echo only, please

38. Summary NJ sports screening module is here, and mandatory Refer to a Pediatric Cardiologist for: - suspicious cardiac symptoms - change in cardiovascular exam - high risk family history - abnormal screening ECG Call with any questions, concerns

39. Thank you! Questions?