New physician guidelines to identify patients at risk of sudden cardiac death

March 31, 2022

New guidelines have recently been published for both primary care physicians and pediatric cardiologists help to identify and treat patients at risk of sudden cardiac death.

The first, a position paper, is "Sudden Death in the Young: Information for the Primary Care Provider," published in Pediatrics in July 2021. This paper, from the American Academy of Pediatrics and the Pediatric and Congenital Electrophysiology Society (PACES), provides new tools for diagnosing young patients potentially at risk of sudden cardiac death. In addition, the paper recommends screening of all children, not just those who participate in competitive athletics. The screening includes history and physical exam.

The second, a consensus statement for pediatric cardiac specialists, is the "2021 Pediatric and Congenital Electrophysiology Society (PACES) Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients," published in November 2021 in Heart Rhythm.

"These guidelines advance high-quality medical care in young patients and provide a framework to build expertise in the care and management of children both in the primary care setting and in cardiac specialty care," says Bryan C. Cannon, M.D., a pediatric cardiologist at Mayo Clinic Children's Center. He contributed to both papers.

For primary care physicians: Enhanced tools to identify risk of sudden death

"Sudden Death in the Young: Information for the Primary Care Provider" lists the cardiac conditions that may predispose to sudden death. It includes relevant clinical points that may be picked up in the history or physical exam. For example, the paper recommends asking all children, whether athletes or not, the following four questions during pre-sport physicals or during health maintenance visits a minimum of every three years or on entry into middle school and high school:

  • 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?
  • Have you ever had exercise-related chest pain or shortness of breath?
  • Has anyone in your immediate family, such as parents, grandparents or siblings, or other, more distant relatives, such as aunts, uncles, or cousins, died of heart problems or died unexpectedly before age 50? This would include unexpected drownings, unexplained auto crashes in which the relative was driving or sudden infant death syndrome.
  • Are you related to anyone with HCM or hypertrophic obstructive cardiomyopathy, Marfan syndrome, arrhythmogenic cardiomyopathy, long QT syndrome, short QT syndrome, Brugada syndrome, or catecholaminergic polymorphic ventricular tachycardia or anyone younger than 50 years with a pacemaker or implantable cardioverter-defibrillator?

Additional investigation and referral to a pediatric cardiologist or pediatric electrophysiologist is indicated when there is a positive response to any of these questions or an abnormal ECG result.

"The guidance in this paper helps primary care providers identify at-risk patients and refer them to cardiology for further evaluation," says Dr. Cannon, who was on the Pediatric and Congenital Electrophysiology Society task force on prevention of sudden death in the young.

For cardiac specialists: First-ever guidance on pacemakers specifically for children

For pediatric patients, pacemakers and implantable cardioverter-defibrillators (ICDs) represent only about 1% of device implantations in the United States. However, these devices can provide lifesaving technology even to the smallest patients.

The "2021 Pediatric and Congenital Electrophysiology Society (PACES) Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients" is the first pediatric-specific electrophysiology guideline written by care providers with specific expertise in implantation of devices in young patients with congenital heart disease.

The statement notes differences from guidelines used for adult patients. "For example, the data derived primarily from older adults on primary prevention ICD placement — based solely on a reduced left ventricular ejection fraction — do not apply as well to children," says Philip L. Wackel, M.D., a pediatric electrophysiologist at Mayo Clinic Children's Center and author of two statement sections on pacemakers.

Referring to both sets of guidelines, Dr. Wackel adds, "These papers complement each other and provide very helpful guidance to offer high-quality cardiac care, from primary care to specialty care."

Takeaways from new guidelines to identify and treat patients at risk of sudden cardiac death

  • All children should be evaluated for conditions predisposing to sudden cardiac arrest or death during routine health care with a thorough and detailed history, family history and physical examination to begin assessing risk.
  • An ECG should be the first test ordered when there is concern about risk of sudden cardiac arrest or death. It should be interpreted by a physician trained in recognizing electrical heart disease.
  • The cause of sudden cardiac arrest remains unclear in 50% of pediatric survivors. ICD implantation is recommended once reversible causes have been excluded or other beneficial treatments are considered.
  • There should be increased awareness of the possible need for a pacemaker or an ICD in those with underlying neuromuscular disorders, channelopathies, cardiomyopathies or congenital heart disease. Shared decision-making and patient- and family-centered care are recommended.

For more information

Erickson CC, et al. Sudden death in the young: Information for the primary care provider. Pediatrics. 2022;148:e2021052044.

Shah MJ, et al. 2021 Pediatric and Congenital Electrophysiology Society (PACES) expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients. Heart Rhythm. 2021;18:1888.

Mayo Clinic Children's Center.

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