Sudden Cardiac Death in Young Athletes

Case

athlete_0.jpg
http://breakingmuscle.com/family-kids/the-very-real-dangers-of-pushing-k...

A 14-year-old girl comes to your office for a physical exam prior to entering high school. He is planning to play soccer. What are the important areas to cover in your history and physical exam? 

Introdoction

Approximately 50 young athletes die suddenly each year. Sudden cardiac death in athletes is defined as a cardiac arrest up to 6 hours after initial onset of symptoms in an individual who has not previously been recognized to have cardiovascular disease. It is estimated that 5/100,000 children have an underlying condition that makes them vulnerable to sudden death.

The American Heart Association recommends a pre-participation physical every two years by a healthcare worker who is able to obtain a complete cardiovascular history, physical exam, and recognize heart disease. An EKG is optional in the United States - unlike Europe, where it is a required part of all pre-participation physicals.

The physical exam should be done in a quiet area and the parent should be present. A thorough history and review of systems is important.

 

Pre-participation Screening

  1. Has there been any exertional chest pain or discomfort?
  2. Has there been any family history of death in members less than 50 years old including drowing?
  3. Family history of members less than 50 years old with disability from heart disease?
  4. Any history of chest pain without exercise?
  5. Any history of shortness of breath?
  6. Any history of unexplained presyncope or syncope?  Any syncope related to exercise?
  7. Any history of heart murmur?
  8. Have there been palpitations?
  9. Any history of elevated blood pressure?
  10. History of rheumatic fever, hypertension, or Kawasaki's disease?
  11. Physical exam should include blood pressure, heart rate, palpation of pulses, and chest and heart auscultation. In addition, the physician should be cognizant of physical stigmata of Marfan's and chromosomal abnormalities such as Downs and Turner's Syndrome.

 

The purpose of the athletic pre-participation evaluation is to identify athletes at risk and not to disqualify any athlete unnecessarily. If there is any suspicion, the patient should be further evaluated by a cardiologist. There is no recommendation to screen all athletes with EKGs and echocardiograms, as positive predictive value of these tests vary in the literature, and they are not cost-effective. 

 

Conditions associated with sudden unexpected cardiac death

  1. Hypertrophic Cardiomyopathy

                       hcm.png

                           http://en.wikipedia.org/wiki/Hypertrophic_cardiomyopathy
                             
    1. Most common cause of sudden death in US and most commonly in Afro-Americans
    2. Left ventricular hypertrophy with poor filling is the hallmark and often there is asymmetric septal hypertrophy creating an impedance to outflow.
    3. Symptoms: dyspnea, syncope, angina, seizures, sudden death
    4. Exam: May have harsh systolic murmur increased with valsalva or or on standing.  This is due to reduced LV volume and worsened outflow obstruction.
    5. May have EKG findings (increased voltage, prominent Q-waves, or deep T-wave inversions) and echo changes
  2. Coronary artery abnormalities
    1. Second most common cause in the US
    2. Usually an aberrant origin of left main or right coronary artery
    3. Sympoms may include angina, syncope, or sudden death
    4. Physical exam is normal
    5. Without prior infarction or arrhythmia, athletes with surgically corrected abnormalities may participate in sports.
  3. History of Kawasaki Disease
    1. If there have been no coronary artery changes or patient has resolution of coronary artery disease, patient should be cleared for competition.
  4. Myocarditis
                      
                     myocarditis.jpg

    Histopathological image of myocarditis at autopsy in a patient with
                      acute onset of congestive heart failure  
                                              http://en.wikipedia.org/wiki/Myocarditis

                                
    1. Usually have a history of being ill, including CHF
    2. Associated with atrial and ventricular tachyarrhythmias or bradyarrhythmias
    3. Sudden deaths probably related to conduction system inflammation.
    4. Should abstain from spoorts for 6 months.
  5. Marfan's Syndrome
                   
                  marfan.jpg

    www.transgenomic.com/labs/cardiology/familion/marfan-taad
                               
    1. Autosomal dominant
    2. Sudden death from aortic dissection
    3. Pectus deformities, > 95% for height, long spindly fingers, ectopic lens, high palate, kyphoscoliosis, inguinal hernias.
    4. Should refer to cardiologist.
  6. Mitral Valve Prolapse
           mvp_0.png

    In mitral valve prolapse, the leaflets of the mitral valve prolapse back into the left atrium. - http://en.wikipedia.org/wiki/Mitral_valve_prolapse
                    
    1. Midsystolic click (not holosystolic murmur) is hallmark. Echo is indicated only if mumur is present.
    2. If there are cardiac symptoms, should see cardiologist.
    3. Not all patients with MVP are excluded from competitive sports, but some risk factors include mitral regurgitation, arrythmogenic syncope, history of ventricular arrhythmia or SVT
  7. Arrhythmogenic Right Ventricular Cardiomyopathy
                 
                
    arv.jpg
    In vitro MRI and corresponding cross section of the heart in ARVD show RV dilatation with anterior and posterior aneurysms (17-year-old asymptomatic male athlete who died suddenly during a
                                  soccer game). http://en.wikipedia.org/wiki/Arrhythmogenic_right_ventricular_dysplasia

                    
    1. Fibrofatty infiltration of the RV myocardium
    2. Syncope, exercise-induced palpitations.
    3. Should not participate in competitive sports
  8. Arrhythmias
    1. Sinus node disturbance
    2. Wolff-Parkinson-White syndrome
              wpw.jpg  
       Characteristic delta wave seen in a person with Wolff–Parkinson–White syndrome. Note the
                             short PR interval. 
      http://en.wikipedia.org/wiki/Wolff%E2%80%93Parkinson%E2
                                     %80%93White_syndrome      
              
    3. Exercise-induced ventricular ectopy
    4. Prolonged QT - high risk for ventricular arrhythmia (torsades de pointes)
      1. Jervell-Lange-Nielsen syndrome associated with congenital hearing loss (autosomal dominant)
      2. Romano-Ward syndrome (autosomal recessive)
  9. Atherosclerotic coronary heart disease
                  
                   
    athero.jpg
    Micrograph of an artery that supplies the heart showing significant
           atherosclerosis and marked  luminal  narrowing.  
                               http://en.wikipedia.org/wiki/Atherosclerosis

                        
    1. Family history of hyperlipidemias should be discussed

 

References

  1. AAP Section of Cardiology and Cardiac Surgery. (2012).  Policy statement: pediatric sudden cardiac arrest.  Pediatrics, 129(4), 1094-1102. 
  2. Maron, B.J. (2003). Medical progress: sudden death in young athletesNEJM, 349(11): 1064-1075.
  3. Nishimura, R. and Holmes, D. (2004). Hypertrophic obstructive cardiomyopathyNEJM, 350(13), 1320-1327.
  4. Rodday, A.M. et. al. (2012). Electrocardiogram screening for for disorders that cause sudden cardiac death in asymptomatic children: a meta-analysisPediatrics, 129(4), 999-1010.
  5. Schoenbaun, M. et. al. (2012). Economic evaluation of strategies to reduce sudden cardiac death in young athletesPediatrics, 130(2), 380-389.

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