Pediatric screening for sudden cardiac arrest guidelines

 

The American Academy of Pediatrics just updated the 2012 guidelines on the risk assessment for sudden cardiac arrest or death in the young, suggesting this screening  should be a routine one as opposed to a targeted one for those entering competitive athletics (see sudden cardiac deaths AAPpolicy2021 in dropbox or https://pediatrics.aappublications.org/content/pediatrics/148/1/e2021052044.full.pdf 

 

Review: 

-- sudden cardiac arrest and sudden cardiac death are associated with an array of underlying cardiac conditions, including cardiomyopathies (hypertrophic,  dilated, restrictive, arrhythmogenic), 

channelopathies (long and short QT syndromes, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, idiopathic ventricular fibrillation), several congenital heart diseases, WPW, Commotio Cordis from a sudden high impact to the chest wall, anomalous coronary arteries, aortopathies (Marfan’s, bicuspid aortic valve with aortic dilatation, Ehler-Danlos)  

--lately there has been some improvement in survival in those with sudden cardiac arrest, which they attribute to increased layperson recognition of cardiac arrest, increased willingness to intervene, and ability to provide high quality CPR and use of an automated external defibrillator (though they also note that life support training in laypeople remains at a remarkably low-level) 

-- for those at higher risk, the American Heart Association suggests that cardiac screening should have a 14-point history and physical for athletic participation (based on expert opinion) 

     -- Personal history:

        -- 1. Chest pain, discomfort, tightness, or pressure related to exertion

        -- 2. Unexplained syncope or near-syncope not felt to be vasovagal or neurocardiogenic in origin

        -- 3. Excessive and unexplained dyspnea or fatigue or palpitations associated with exercise

        -- 4. Previous recognition of a heart murmur

        -- 5. Elevated systemic blood pressure

        -- 6. Previous restriction from participation in sports

        -- 7. Previous testing for the heart, ordered by a physician

        -- 8. Family history of premature death (sudden and unexpected or otherwise) before 50 y of age attributable to heart disease in $1 relative

        -- 9. Disability from heart disease in close relative

        -- 10. Hypertrophic or dilated cardiomyopathy, LQTS, or other ion channelopathies, Marfan syndrome, or clinically significant arrhythmias; specific knowledge of genetic cardiac conditions in family members

    -- Physical Examination:

        -- 11. Heart murmur, not felt to be innocent

        -- 12. Femoral pulses to exclude aortic coarctation

        -- 13. Physical stigmata of Marfan syndrome

        -- 14. Brachial artery blood pressure (sitting position), preferably taken in both arms

-- for routine screening of all children the American Pediatrics Association suggests that regardless of athletic participation, screening be performed at least every 3 years (or on entry to middle or junior high school,and into high school) from ages 6 to 21 (also based on expert opinion only), with this modified 4 question screen: 

    -- 1. Have you ever fainted, passed out, or had an unexplained seizure suddenly and without warning, especially during exercise or in response to sudden loud noises, such as doorbells, alarm clocks, and ringing telephones?

    -- 2. Have you ever had exercise-related chest pain or shortness of breath?

    -- 3. Has anyone in your immediate family (parents, grandparents, siblings) or other, more distant relatives (aunts, uncles, cousins) died of heart problems or had an unexpected sudden death before age 50? This would include unexpected drownings, unexplained auto crashes in which the relative was driving, or SIDS.

    -- 4. Are you related to anyone with HCM or hypertrophic obstructive cardiomyopathy, Marfan syndrome, ACM, LQTS, short QT syndrome, BrS, or CPVT or anyone younger than 50 years with a pacemaker or implantable defibrillator (these abbreviations reflect the conditions noted above)

    -- the EKG should be the first test ordered when there is concern about sudden cardiac arrest risk (and, do not trust the computer interpretation of the EKG)

-- see the text itself for comments on what the clinician should do for secondary prevention, communication/bereavement, evaluation remaining family members, role of licensed athletic trainers, return to activity after cardiac arrest.

 

Commentary: 

-- this change reflects a few issues: 

    -- there are cases of sudden cardiac arrest or death that are not related to intense exercise 

    -- one concern about the prior recommendation is that targeted preparticipation sports screening may well lead to inaccurate responses: the student athlete may deny risk factors in order to be able to participate in the sports that they love. So one reason for screening all children aged 6 to 21 at least every three years is to normalize the screening and extract it from just a pre-athletic participation screen 

-- they did not specifically trash the AHA 14 point screen, but they do feel that their standardized four question one that they have devised is appropriate and should be used in all kids 

-- and, another plus for the APA approach (not mentioned by them in the article) is that the 4-point screening is much more likely to be done vs the 14-point one, especially in light of the  multitude of other recommended screenings…

 

So, this all seems reasonable. But i am not sure that many of the children I have seen (or their parents) have a very accurate knowledge of the specific diseases of their relatives. And, it turns out that in a couple of studies with well-documented family history, relatives may not know or remember accurately the actual family history (a Framingham Study found that there was not a great correlation between what people said about their family history and what was actually known about their parents from the Framingham Study database). and a review of family history accuracy overall found very low sensitivity though 90-95% specificity for the conditions measured (ie, relatives did not know much; but if they did know something, it was likely accurate), though the authors do note that the accuracy probably varies lots by the underlying condition being assessed (terminal cancer more likely remembered accurately than a pacemaker in someone <50yo, for example): see family history accuracy AIM2009 in dropbox, or Berg AO. Ann Intern Med. 2009;151:872-877

 

geoff

 

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