[17] Grown-Up Congenital Heart Disease and Sudden Death in a Medical Examiner's Population

LE Hamilton, EO Lew, EW Matshes. University of Calgary, Calgary, AB, Canada; Miami-Dade Medical Examiner's Office, Miami, FL; Southwestern Institute of Forensic Sciences, Dallas, TX

Background: Advances in the management of congenital heart disease (CHD) have allowed many children born with heart defects to survive into adulthood, and in the United States there are now more adults than children living with CHD. Even with surgical correction, however, this population is at increased risk of sudden death. There are also adults with undiagnosed CHD who first present with sudden death. A retrospective review of cases from a large medical examiner's office was conducted to examine the spectrum of CHD presenting as sudden death in adults.
Design: The case files from 1991 to 2007 of the Miami-Dade County Medical Examiner Department were reviewed in this retrospective descriptive study. Adults (individuals >16 years of age) whose cause of death was attributed to the primary or secondary effects (medical/surgical complications) of CHD were included. The following data was collected and stored in a Microsoft Access database: age, sex, race, cause and manner of death, circumstances of death, relevant clinical history, whether autopsy was performed, and autopsy findings related to cardiac and other congenital anomalies. Basic statistics were used to analyze the data.
Results: Of 51,228 adult cases investigated between 1991 and 2007, 80 cases (0.2%) were attributed to CHD. The age range was 17 to 90 years; 65% were male and 35% female. The autopsy rate was 81%. The cardiac malformations described at autopsy were:

Obstructive lesions: bicuspid AV24 (36.9%*)
Obstructive lesions: other than bicuspid AV5 (7.7%)
Flow lesions (eg. TOF, ASD, VSD, PDA)14 (21.5%)
Complex (HLHS, DORV, single ventricle)1 (1.5%)
Mixed (combination of flow and obstructive lesions)3 (4.6%)
Anomalous coronary anomalies17 (26.2%)
Unclassifiable1 (1.5%)
* % of autopsied cases with CHD

Two females died in the peripartum period. Eleven cases had a history of cardiac surgery, of which two had surgery within 2 weeks of death.
Conclusions: CHD is a rare but important cause of sudden cardiac death in adults. In a large medical examiner's population, bicuspid aortic valves and anomalous coronary arteries are the most commonly recognized entities. However a wide spectrum of simple to complex malformations may be seen, with or without prior surgery, and in a wide age group. Once a solely pediatric entity, CHD is now grown up, and in the future will likely be seen with increased frequency in forensic pathology settings.
Category: Autopsy

Monday, March 22, 2010 9:30 AM

Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 3, Monday Morning

 

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