Short Attention Span Summary
You could save a life
Is there a way to tell which child might have sudden cardiac arrest in the near future? This was a retrospective look at patients presenting, "with 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy [my abbreviation = HOCM], long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R)."
Usually family history doesn't matter much in EM. But it is highly important to ask about a history of sudden death or early pacemaker/defibrillator placement in pediatric patients presenting with syncope. HOCM, LQTS, and CPVT frequently had a known or suspicious family history. Other predictors of sudden cardiac arrest were a history of previous cardiac arrest, obviously. ALCA-R most often had exercise chest pain or history of syncope. CPVT had a high incidence of syncope and were the most likely group to have history of prior arrest.
Any child who presents with unexplained syncope (especially exercise syncope), exercise chest pain, known family history or suspicious family history needs to see a pediatric cardiologist as soon as possible and should not exert themselves at all until cleared. EM Docs has a good review of cardiac arrest in the young.
J Pediatr. 2016 Aug 5. pii: S0022-3476(16)30518-2. doi: 10.1016/j.jpeds.2016.06.088. [Epub ahead of print]
1Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Monroe Carell Jr. Children's Hospital at Vanderbilt, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN.
2The Heart Institute, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH.
3Children's Hospital of Wisconsin, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI.
4C.S. Mott Children's Hospital, Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI; Children's Hospital Colorado, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO.
5Texas Children's Hospital, Department of Pediatrics, Baylor College of Medicine, Houston, TX.
6Primary Children's Hospital, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT.
7Mayo Clinic, Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, Rochester, MN.
8Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
9Children's Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA. Electronic address: firstname.lastname@example.org.
To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R).
This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014.
A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT).
Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest.
Copyright © 2016 Elsevier Inc. All rights reserved.
PMID: 27502104 [PubMed - as supplied by publisher]