Sudden Cardiac Death of the Young in Michigan: Development and Implementation of a Novel Mortality Review System

Abstract

Advances in screening, detection and treatment make Sudden Cardiac Death of the Young (SCDY) a potentially preventable condition. Since hereditary causes account for many deaths, identification of an affected individual has implications for immediate relatives; who should receive targeted screening with the aim of preventing SCDY. To develop a mortality review process for SCDY and to identify potential unmet needs for family-based, medical system and public health interventions. The Michigan Department of Community Health and Michigan State University developed a system for investigating SCDY. Review of medical records and next-of-kin (NOK) interviews were conducted. A de-identified summary of each case was presented to an expert panel. The panel identified factors that contributed to the death and possible actions to prevent future deaths. If the case was deemed to have a likely heritable cause, NOK were notified of a possible increased risk and need for evaluation of immediate family members. Twenty-three deaths aged 1–39 years between 2006 and 2008, were selected for review. Sixteen NOK were interviewed. Several primary and secondary prevention measures were identified, including enhanced pre-participation sports screening; provider education; public awareness of risk factors, symptoms, emergency response training for coaches and the general public; and creation and dissemination of emergency response and medical examiner protocols. Seventeen NOK were notified of the potential heritable cause. Investigation of these deaths has led to identification of individual, family, public and provider needs and motivated policy makers to initiate changes to prevent future SCDY.

Appendix

Synopsis

An African American male in his late teens died in the spring while playing basketball outside. His mother and grandmother were interviewed and stated the deceased was a very healthy teenager with no medical problems. He received a scholarship and was planning to attend college after he graduated from high school. His death certificate stated he died of hypertrophic cardiomyopathy. He had Medicaid health insurance with unknown co-pay.

Event History

His mother reported him coming home from school and looked fine as he usually did after school. He went to a relative’s house to play basketball. A friend told his mother that after playing basketball for a while, he looked extremely tired. The deceased walked to a chair collapsed while attempting to sit. EMS was called, which was dispatched at 20:17, enroute at 20:18 and on the scene at 20:24. The records stated the deceased was found by EMS in respiratory and cardiac arrest. No bystander CPR was recorded. An IV was started and the deceased was intubated at 20:28. Ventricular fibrillation showed on the cardiac monitor. He was defibrillated three times (200, 300, 360 joules) at 20:30. CPR was continued. Epinephrine was given at 20:31. Amiodarone 300 mg was administered at 20:34. At 20:38 Epinephrine and Atropine were repeated. At 20:42 ventricular fibrillation persisted. He was defibrillated at 360 joules again. Epinephrine, Atropine and Naloxone were re-administered at 20:43. Half ampoule of Dextrose (50%) was given at 20:44. He was transported to the hospital at 20:46. Hospital records stated the deceased arrived intubated with CPR in progress. ACLS protocol medications were administered. He was defibrillated again. The deceased developed PEA. A transvenous pacemaker was used without successful capture. With ventilation his lung sounds were clear and equal bilaterally. A central line was placed. ACLS protocol was continued and an Epinephrine drip was started. He did not respond to the resuscitation and was pronounced dead at 21:35.

Autopsy

Showed hypertrophic cardiomyopathy as evidenced by cardiomegaly, interstitial and perivascular fibrosis, myofiber disarray, and myocardial ischemia. Toxicology was negative for alcohol and illicit drugs.

NOK Interview

His mother reported that the deceased was rarely sick and only went to the doctor’s office for his annual physical prior sporting events. These records were unavailable. His mother did not report prior heart problems. His mother and half-sister had no history either. His maternal grandmother had heart problems requiring a recent open heart surgery. His mother was separated from his father for many years so the mother was unaware of any heart problems on his father’s side of the family. After her son’s death, his mother discovered the father and paternal grandparents with heart issues but did not know details. She did not know how many half-siblings were on the father’s side of the family or if these siblings had health problems. He never had formal cardiac testing done. He was a high school student and did not have a job. His mother described his eating habits as ‘fair’ and medium in fat which she stated was pretty usual for teenage boys. She said the deceased played basketball daily for at least an hour. He did not smoke nor was he around smokers at home. He was not a known user of alcohol or illicit drugs. She said she received information about the cause of death from the health care professionals at the hospital. She was not instructed to take any action as a consequence of the deceased’s death. None of her family members were tested. The deceased’s home was clean and in a good state of repair. His weight was in the 38th percentile.

Standardized form completed by mortality review panel members with summary conclusions for above death.

Case number: MSU_ _

Causal factors (number in priority order):

Patient-related factors

None

Physician-related factors

Quality of pre-participation sports physical

Questions about EMT response, particularly need for immediate defibrillation

System related factors

None

Follow-up activities that are supported by the case (number in priority order):

Educate health care providers

Criteria for screening relatives

Educate patients

None

System level changes

Feedback to EMT—particularly about immediate defibrillation

Save tissue/blood sample for genetic testing (need criteria, mechanism to pay for testing, and then dissemination of info to MEs)

Mechanism to notify relatives of need for screening

Improvement of sports physical screening—include recommended questions of American Heart Association