1. Reasons to Call a Pediatric Cardiologist Premchand Anne, MD, MPH, FACC Pediatric and Adult Congenital Cardiology Pediatric Lipid Clinic St. John Providence Children’s Hospital April 1, 2015

2. Disclosures I have nothing to disclose!

3. Objectives Understand signs and symptoms of shock and heart failure due to congenital heart disease Management of absent pulses with associated hypertension Differentiate between benign and pathologic causes of syncope Recognize chest pain that is of concern Understand what to do with an elevated total cholesterol

4. Case A 2.5 month old girl is brought to you for a well-visit. Parents have noticed over the past two weeks that she has diaphoresis with feedings, along with persistent tachypnea. Her PO intake decreased over the past one to two weeks. She has decreased wet diapers. She is more tired after feeding and, in general, fussy. There is poor weight gain. Physical exam reveals a harsh 3/6 murmur at the apex with radiation to the left axilla. There are diminished pulses. Tachypnea; Liver size inc.

5. Differential Diagnoses Cardiogenic shock – Pump function Structural Hypoxemia and acidosis Arrhythmia – Obstruction LVOT obstruction Arch obstruction Tamponade

6. Differential Diagnoses Hypovolemic shock – Inadequate fluid intake – Intravascular volume loss – Fluid loss Distributive shock – Septic – Anaphylaxis – Drugs/toxins – Endocrine-Addisonian

7. Diagnosis Anomalous origin of the left coronary artery from the pulmonary artery

8. Typical Signs and Symptoms Tachypnea and/or retractions Tachycardia Decreased PO intake Poor weight gain Diaphoresis with feeds Decreased activity and fatigue Abdominal pain in older children

9. Management Treat underlying condition or provide supportive, including medications. High calorie nutrition Minimize energy expenditure Noninvasive testing – ECG – ECHO – CXR Surgical repair, if amenable

10. Case A fussy, six-month old girl is seen for a well visit in your office. Per mother, she is active, has good appetite and healthy. Examination shows a comfortable child without significant respiratory distress. There is a 3/6 harsh systolic ejection murmur noted in right upper sternal region, with radiation to the midscapular area. There are no bruits noted in the abdomen. There are diminished pulses in the lower extremities and BP in left arm 54/42.

11. Question What are the next steps? A.Calm the baby down and check the baby’s femoral pulses B.Obtain four limb blood pressures with appropriate size cuff C.Obtain an echocardiogram D.Consider getting a chest XRAY. E.All of the above

12. Differential Diagnoses Coarctation of the Aorta. Everything else! – Possible sepsis – Possible shock and heart failure

13. Case A 15-year old boy is seen in your office for a well- visit. Child’s BP has been elevated one year ago. They were lost to follow-up since last visit even though the PCP mentioned rechecking the blood pressure in 1-2 weeks. He reports intermittent headaches and exertional chest discomfort when he does sprints. No other symptoms. Physical exam: Comfortably chatting with mother. Rt arm BP: 154/92; Rt. Leg BP: 102/67. Harsh 3/6 SEM at LUSB with radiation to the back. Diminished femoral pulses. Saturation in right arm: 100%

14. Most likely diagnosis? A.Hypoplastic left heart syndrome B.Pulmonary stenosis C.Unrepaired AVCD D.L-Transposition of the great arteries with coarctation of the aorta E.D-Transposition of the great arteries.

15. A Note on Checking Blood Pressure The patient should be resting for 5 minutes while sitting upright Feet must be flat on the ground and quiet. Appropriate sized cuff (width >40% of the arm circumference halfway between the olecranon and acromion and length >80% of the arm circumference)

16. Case A 15-year old girl had a brief syncopal episode after stepping out of a hot shower. The episode lasted 3-5 seconds and was preceded by visual scintilla, auditory disturbances, and dizziness. There was no drowsiness or confusion after the episode. She reports having dizziness with acute positional changes. Work-up in the local ER with an ECG was normal; IV fluids given and discharged home.

17. Diagnosis Neurocardiogenic syncope – Neurally-mediated hypotension – Vasovagal syncope – “Fainting reflex” – Vasodepressor syncope – Autonomic dysfunction

18. Differential Diagnoses Orthostatic hypotension Prolonged QT syndrome Tachydysrhythmias or Bradycardias Obstructive cardiac disease High grade atrioventricular block Myocardial structural disease Medication toxicity

19. Predispositions to Neurocardiogenic syncope Prolonged periods of upright posture – Lines at amusement park rides Being in a warm environment Immediately after exercise Emotionally stressful events Painful hair brushing/plaiting

20. Syncope-Concerned? TO BE…NOT TO BE… Exercise-relatedHistory of fluid losses Positive ECG changesSituational Positive echo changesAbsence of cardiac disease Documented dysrhythmias

21. Strickberger, S. A. et al. Circulation 2006;113:316-327

22. Non-Invasive Testing ECG – QT interval – Hypertrophy – Blocks – Brugada changes – ARVD changes ECHO Tilt test Exercise stress test Monitor (Event versus Holter) MRI (ARVD, hypertrophy, ischemia)

23. Testing for Syncope Brignole, M. et al, Heart. 93: 130-136

24. Management Depends on the cause Consider increasing fluids and salt intake – Hypovolemia – Neurocardiogenic syncope Postural Orthostatic Tachycardia Syndrome (POTS) Sit if dizziness is present Avoid caffeinated products Mineralocorticoids, beta-blockers, midodrine… Although uncommon, consider pulmonary embolism.

25. Case A five-year-old girl reports that her “heart hurts.” She has had a few episodes, which occurred at rest and physical activity. When her mother palpated her anterior chest all, she noted rapid heart rate that was “too hard to count.” The pain usually resolves after a few minutes, and is associated with resolution of the sensed tachycardia. There is no reproducible chest pain. She denied dizziness, syncope, and difficulty breathing.

26. Case A 15-year old football player presents for a well visit. He reports having exertional discomfort only when sprinting during football practice. He denies similar discomfort when lifting weights. He describes the discomfort as a pressure below the sternum. There is no nausea, vomiting, or radiation of discomfort to the neck and arm. ECG and Echo were abnormal. He was restricted from further participation in sports to his dismay.

27. Parental Perception of Chest Pain

28. Chest Pain Etiology in Reality…

29. “6 Questions to Ask” Cincinnati Children’s Hospital Blog 1.Has the child been sick recently? 2.Was the child injured recently? 3.Is the child stressed? 4.When does it hurt? 5.How long has it been hurting? 6.How painful is it? 7.Were there any cardiac deaths in the first two decades of life? Madsen, N. http://cincinnatichildrensblog.org/healthy-living/6-questions-to-ask-when-your-child-complains-of-chest-pain/#authorbox

30. Chest Pain Characteristics Likely BenignLikely Pathologic Reproducible with palpationAssociated with syncope SharpTearing Occurs at restPressure No tachycardiaRadiation to neck and shoulder Due to hyperventilationAngina ExtracardiacRetrosternal Sudden onset and offset; worse with deep breathing and position change; several minutes into activity

31. Causes of Chest Pain Common musculoskeletal causes – Costochondritis – Trauma and over-use syndromes – Slipped rib syndrome-Tietze’s syndrome – Precordial catch syndrome-Texidor’s Twinge Psychological causes – Hyperventilation – Anxiety

32. Causes of Chest Pain Respiratory causes – Exercise-induced asthma – Pneumonia – Pleurisy – Pulmonary embolism, pneumothorax Gastrointestinal causes – Reflux – Gastritis – Esophagitis and esophageal spasms

33. Causes of Chest Pain Cardiac Causes – Tachyarrhythmias – Pericarditis – Myocarditis – Obstructions (pump and pipes) – Syndromic associations Turner’s, Marfan’s, Type IV EDS, – Ischemia Coronary anomalies; HCM or HOCM

34. Chest Pain Work-up Not everyone needs an echocardiogram or ECG; a thorough history and physical can guide the level of testing. Non-invasive and invasive testing – ECG – Echo – CXR – CT-Angiogram-myocardial bridges

35. Chest Pain Management Most often: reassurance Treat underlying etiology – Medical management – Referral to Pediatric Cardiology Medical Management Surgical management – Referral to other specialties if suspecting non- cardiac etiology

36. Cases Few fasting lipid profiles are listed below: Which profile(s) are concerning? ABCD TC217 HDL-C7738 65 LDL-C119158105120 TG105 370210 Apo B89127120105

37. Fridewald Equation Vs. Direct measurement LDL-C= TC – HDL-C- TG/5 Martin, SS et al. JACC. 2013;62(8):732-739

38. Lipid Cutoff Values Expert panel. Pediatrics. 2011; 128: S5

39. Cases Few fasting lipid profiles are listed below: Which profile(s) are concerning? ABCD TC217 HDL-C7738 65 LDL-C119158105120 TG105 370210 Apo B89127120105

40. AAP Guidelines for Lipid Screening Ages 2-8 years: selective screening if at least one risk factor Ages 9-11 years: Universal screening Ages 12-17 years: selective screening if at least one risk factor. Risk Factors: – Unhealthy body weight – Elevated blood pressure – One or both parents with hyperlipidemia – Tobacco use – DM Expert panel. Pediatrics. 2011; 128: S5

41. LDL-C Level and Duration Horton JD, et al. J Lipid Res. 2009; 50 (Suppl):S172-S177

42. Lipid Management Usually lifestyle modification Determine whether primary or secondary hyperlipidemia Hypercholesterolemia – Dietary modification with low saturated fat – BAS – Statins – Ezetimibe Hypertriglyceridemia – Decreased intake of carbohydrates, especially refined carbohydrates (candy, juice, POP, sugar); weight loss; activity – Omega-3 Fatty Acids (marine type)

43. Questions?