The Heart of the Matter

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The Heart of the Matter Is the Heart the Matter? --Chest pain in the Pediatric Patient-- 19th Interregional Symposium November 2, 2018 Session A, 9:35-10:50 am John-Charles Loo, MD Pediatric Cardiology, KP--OC

It s about holding hands. And people don t get lost if there are enough people to hold hands --Jean Vanier, L arche

A 15 year old presents with chest pain The next appropriate step is: 1. 2. 3. 4. Order an ECG Order an echocardiogram Obtain labs, including troponin Obtain more history

A 15 year old presents with chest pain A 15 year old comes in with 3-4 year history of chest pain. Pain occurs every 1-2 weeks. Pain is at rest. The pain is sharp quality, located left upper parasternal area. The pain lasts for less than 1 minute, usually just a few seconds. The pain is worse when he takes a deep breath. It hurts more to twist his body. It occurred last night while he was on his side in bed on his phone. He changed position, and it improved. The pain has not occurred with exercise, except for one time a year ago when he was in the first week of football conditioning, after a long series of sprints and drills, he was out of breath and felt a tightness in his chest, but that was a different pain. He played the rest of the season without any problem and still works out daily without any chest pain. He has had wheezing in the past and has taken Albuterol for it, but this is with URI, not really with exercise. He has no heartburn symptoms.

What are the history details that seem most helpful? A 15 year old comes in with 3-4 year history of chest pain. Pain occurs every 1-2 weeks. Pain is at rest. The pain is sharp quality, located left upper parasternal area. The pain lasts for less than 1 minute, usually just a few seconds. The pain is worse when he takes a deep breath. It hurts more to twist his body. It occurred last night while he was on his side in bed on his phone. He changed position, and it improved. The pain has not occurred with exercise, except for one time a year ago when he was in the first week of football conditioning, after a long series of sprints and drills, he was out of breath and felt a tightness in his chest, but that was a different pain. He played the rest of the season without any problem and still works out daily without any chest pain. He has had wheezing in the past and has taken Albuterol for it, but this is with URI, not really with exercise. He has no heartburn symptoms.

What are the history details that seem most helpful? A 15 year old comes in with 3-4 year history of chest pain. Pain occurs every 1-2 weeks. Pain is at rest. The pain is sharp quality, located left upper parasternal area. The pain lasts for less than 1 minute, usually just a few seconds. The pain is worse when he takes a deep breath. It hurts more to twist his body. It occurred last night while he was on his side in bed on his phone. He changed position, and it improved. The pain has not occurred with exercise, except for one time a year ago when he was in the first week of football conditioning, after a long series of sprints and drills, he was out of breath and felt a tightness in his chest, but that was a different pain. He played the rest of the season without any problem and still works out daily without any chest pain. He has had wheezing in the past and has taken Albuterol for it, but this is with URI, not really with exercise. He has no heartburn symptoms.

What I m thinking (not necessarily what you should be thinking) At first I just want to hear the story (but this takes time) I m not yet trying to fit it into a box or diagnosis (it does it by itself) Then I ask specific questions to clarify which box it fits in The first distinction is whether it seems cardiac or not (frequent with exertion) The next distinction is determining the actual diagnosis

1 big idea: Is it exertional?

Let s take a look at the causes Korean J of Peds 2015

American College of Sports Med 2017

Musculoskeletal (costochondritis, precordial catch) Days, months, or years of chest pain Seconds to several minutes Generally sharp pain Exacerbated by movement or pressure Exacerbated by deep breath shortness of breath is due to pain Palpitations follow pain

Asthma History of asthma Chest tightness, pressure, squeezing Exertional, seasonal, URI Wheezing, Cough Allergies Proper inhaler use

Gastroesophageal reflux Abdominal pain Heartburn symptoms Spicy, fried, fatty foods Sour taste in mouth Burning in chest

Concerning features 1. Helpful Clues 1. 2. 3. Sharp pain Exclusively at rest Changes with position/breathing 4. Brief 5. Wheezing/cough 6. Stomach pain, sour taste in throat Chest pain predominantly with exercise

Why does exertional chest pain matter? Let s start with (but then move away from) a classic adult with atherosclerotic heart Dz Ischemia results from relatively decreased blood flow, typically not occuring at rest Ischemia increases in proportion/frequency with exercise But why doesn t this make sense for a child? Children are too young to develop atherosclerosis. What else can kids acquire? What congenital heart disease?

Cardiac causes of pediatric chest pain Hypertrophic Cardiomyopathy Anomalous Coronary Arteries Pericarditis Aortic stenosis Kawasaki s Drugs

Pathophysiology of Hypertrophic Cardiomyopathy whrrs.org

Anomalous coronary artery (congenital) Adult vs pediatric coronary artery disease Anomalous left or right coronary artery (from aorta or from pulmonary artery) Obstruction -- decreased blood flow Deoxygenated blood -- hypoxic blood flow Surgically reparable

Anomalous left coronary from pulmonary artery Anomalous left coronary from wrong sinus

Beyond symptoms Why does the family history matter? What Dx are we worried about? Congenital Heart Disease? Coronary atherosclerotic heart disease? Arrhythmias? Cardiomyopathies? Asthma Family history Sudden, aborted, or unexplained death or cardiomyopathy Most will not know the diagnosis Deeper questions--risk factors for MI, defibrillator, age, medications, surgery? First degree relatives Autosomal dominant vs autosomal recessive transmission What about grandma? Cousin? Half-brother? Mother?

Could be Physical exam Reproducible tenderness Wheezing Abdominal tenderness Pectus excavatum Murmur Musculoskeletal Asthma GERD Musculoskeletal HCM

Who needs an EKG?

1. A 5 year old boy entering his first soccer season, asymptomatic, presents for pre-participation physical. 2. A 16 year old had 2 episodes of shortness of breath and chest tightness during conditioning week of football 1 year ago. None for the rest of the season nor the current basketball season. 3. A 12 year old has mid-sternal chest pain each time he runs the mile at school. He has asthma and has been using his inhaler prior to running. 4. 14 year old with sharp left sided chest pain, at rest, lasting a few seconds. His brother has hypertrophic cardiomyopathy diagnosed at 28 years of age.

Common athlete EKG findings (should not preclude participation) Sinus bradycardia (rate 30-60 bpm) 1st degree AV block (up to 35%) 2nd degree, mobitz I (up to 10%) Voltage LVH (24%) IRBBB (35-50%) Early repolarization (50-80%) Sinus arrhythmia is a normal finding Corrado, European Heart Journal, Jan 2010

EKG for HCM EKG is abnormal in 95% of HCM The 5% with a normal EKG has limited risk of sudden death So if the chest pain is not predominantly exertional and the EKG is normal, reassure A computer abnormal is often incorrect Call, page, fax EKG

Who gets an echocardiogram?

Why are we talking about echos when I don t order echos for kids?

Boston Children s 2006

Appropriate Use Criteria for Pediatric Echocardiography American Society of Echocardiography

Appropriate Use Criteria evaluated 1 year time period 68 pediatric cardiologists 772 echocardiograms for chest pain Median 8 echo per cardiologist 13 abnormal echocardiograms (1.7%) 3 abnormals related to chest pain (0.39%) 2 abnormals, moderate/severe (0.26%) 98% of A indications were normal All significant abnormals were A indication, no abnormals in R group

Findings consistent No abnormals in the R group 539 CP patients, 380 echos 5% abnormals, minor No significant abnormals nor related in M 1 abnormal related to CP, 0.18% Excess cost of >$47,000

Bottom line--our view If it hurts to breathe, move, or press on it, it s non-cardiac If you think it s non-cardiac, I will agree Parents (and kids) need confident reassurance EKG not required but may be helpful It may or may not go away but not to worry unless it s predominantly with exercise 60% improved by 2 years

Communicating the plan Convincingly reassuring the family Common and frequent: up to 50% of children with chest pain I ve seen this a lot, I get that too, 80 year old vs 13 year old Explain physiology: sternal joint, costochondral junction, GERD, asthma Trial of ibuprofen (even tylenol) PRN Things to watch for: regularly exertional Let them play

What about pericarditis?

And myocarditis?

Aortic stenosis?

Marfan Syndrome?

Pericarditis Myocarditis Aortic Stenosis Marfan Syndrome While the primary question of exertional chest pain does not apply to these, the history or exam for these will to point to the diagnosis.

Pericarditis Acute and constant chest pain, URI Myocarditis Pain while supine, fatigue, fever Aortic Stenosis Progressive dyspnea, murmur Marfan Syndrome Typical features, dilation vs dissection

Shaun White Tetralogy of Fallot S/p 2 surgeries 3x olympics, gold medalist in snowboarding Professional skateboarder and snowboarder