Kids and Young Adults with SCA
on September 10, 2013
Warning Symptoms and Family History in Children and Young Adults with Sudden Cardiac Arrest
Jonathan A. Drezner, MD, Jessie Fudge, MD, Kimberly G. Harmon, MD,
Stuart Berger, MD, Robert M. Campbell, MD, and Victoria L. Vetter, MD
JABFM July–August 2012 Vol. 25 No. 4 http://www.jabfm.org
Identifying medical conditions in their dormant or early stages and preventing subsequent illness is one of the main goals of medicine. This is especially true for conditions harboring lethal outcomes if left untreated. Within the fields of EMS and EM we are often faced with the latter, so it is useful to occasionally expand our scope and review the latest on prevention. Rest assured – we will still get plenty of business.
The authors of this paper focused on 146 families of children and young adults who had unexpectedly collapsed via sudden cardiac arrest (SCA). A survey was distributed through Parent Heart Watch database asking for retrospectively recalled information, with the objective of determining “the prevalence of warning symptoms and family history” in this cohort. With a 60% survey return rate and after excluding 9 patients, 8 of which had known cardiovascular disease prior to SCA, the authors analyzed the resulting N of 79 responses. There were only 9 survivors, totaling 11%.
The final cause of death was unknown in up to one third of all patients even after autopsy, and an additional 9 (11%) had never received one. However, cardiac-related disease was found in virtually everyone else, and an additional 5 cases of it were found via molecular biopsy (post-mortem genetic testing) in 11 of the unknowns (45%). Not unexpectedly, hypertrophic cardiomyopathy and prolonged QT interval led the list of identified causes, otherwise ranging from SVT and MVP to anomalous coronary artery and myocarditis. Nearly 70% of victims were male, of ages between 5 and 29. It is especially important to note that 48% of SCAs are reported to have occurred during or within 1 hour of exercise.
According to the findings, 72% of victims had at least one cardiovascular symptom (fatigue leading ahead of chest pain or palpitations and shortness of breath) prior to their SCA, and 24% had at least one event (seizure or syncope), with a 2.6 episode average. Of note, the time scale reported for subjective symptoms ranged from 19 to 71 months prior to SCA. Nearly two-thirds of study subjects had been screened prior via either a well-child check or a sports pre-participation physical. 27% of families reported a person who has died of a heart condition before age 50.
Unfortunately, because of severe design limitations, most of which to their credit the authors acknowledge themselves, this study does not truly allow for accurate estimation of prevalence of any pre-SCA symptoms or events. It is unknown whether the PHW database provides a fair representation of all SCA cases in children and young adults. Possibility of a significant recall bias puts into question any data received, and the overall response rate of 60% is low. Finally, the symptoms put into question such as fatigue and palpitations are so common and non-specific, even when they occur in the young, they hardly provide any practical guidance for practitioners attempting screening for SCA risks.
Thus, the main value of the paper is in the important issues it raises rather than in the results it reports. As the authors point out, even as fairly recent AHA guidelines for screening of young athletes exist, they are rarely utilized consistently by the wide variety of physician (and non-physician) workers conducting such evaluations. There are no guidelines for screening of normal healthy children and young adults. In addition, no outcome-based studies have been published regarding which screening programs or other interventions used even in just athletes are actually effective. Clearly, a more focused research effort is in order and long overdue.
There are a few clues and pointers, however, that the paper does provide. The importance of a thorough cardiovascular family history is emphasized. We should pay more attention to new onset seizures in older children and young adults, many of which are believed to be cardiogenic – related to brain hypoperfusion and hypoxia occurring secondarily. Recurrent syncope, syncope on exertion and syncope is the setting of non-specific or vague chest symptoms at other times should perhaps trigger a prompt advice or referral for a cardiology evaluation. Should a heart echo be a routine standard for preprogram screening in young athletes?
Until a sufficient body of research in this area accumulates and routine practices are changed, what does all of this entail for EMS?
One thing is taking extra measures for accurate information gathering at scene and emphasizing it appropriately to the receiving MD provider. As the authors allude, there may be a vast difference between convulsions originating while the person is upright and convulsions following loss of consciousness after a brief delay. Paying attention to pulses and the rhythm strip during the seizure is important. Other clues may come from family members who will not be accompanying the patient to the ED.
The second has to do with public advocacy about actual interventions when nothing else can be done. For arrhythmias without a perfusing pulse, this means quality CPR and early defibrillation.
A great number of articles have been published in the last decade on bystander CPR and AED use by non-professionals: an adequate analysis of this literature is beyond this limited review. It is hard to debate the benefit of AEDs and their general safety for individual victims [1,2]. Still, much is yet to be learned about their appropriate and cost-effective distribution – even among professional rescuers. Earlier select studies did not show a survival benefit when AEDs were widely adapted to PD or EMT units [3,4]. Most recently, authors continue to focus on appropriate AED placement for general public access [5,6].
Until more can be said, it is the mutual duty of the EMS and EM communities to continue to steer future research efforts in this area, while continuously examining without bias new data that becomes available.
- Sana T, et al. Cardiopulmonary resuscitation alone vs. cardiopulmonary resuscitation plus automated external defibrillator use by non-healthcare professionals: a meta-analysis on 1583 cases of out-of-hospital cardiac arrest. Resuscitation. 2008 Feb;76(2):226-32. Epub 2007 Sep 17.
- Lim SH, et al. Results of the first five years of the prehospital automatic external defibrillation project in Singapore in the “Utstein style”. Resuscitation. 2005 Jan;64(1):49-57.
- Sweeney TA, et al. EMT defibrillation does not increase survival from sudden cardiac death in a two-tiered urban-suburban EMS system. Ann Emerg Med. 1998 Feb;31(2):234-40.
- Groh WJ, et al. Limited response to cardiac arrest by police equipped with automated external defibrillators: lack of survival benefit in suburban and rural Indiana–the police as responder automated defibrillation evaluation (PARADE). Acad Emerg Med. 2001 Apr;8(4):324-30.
- Winkle RA. The effectiveness and cost effectiveness of public-access defibrillation. Clin Cardiol. 2010 Jul;33(7):396-9. doi: 10.1002/clc.20790.
- Brooks, SC, et al. Determining Risk for Out-of-Hospital Cardiac Arrest by Location Type in a Canadian Urban Setting to Guide Future Public Access Defibrillator Placement. Annals of EM, May 2013.