On the 18th and 21st September, the TVNZ produced television show “Shortland Street” featured a storyline in which a cardiac inherited disease contributed to the sudden death of a patient.
The patient Lynley was undergoing a routine liposuction procedure under general anaesthetic. We see her recovering on the ward afterwards and she is recommended to stay in overnight for observation. However since she is feeling fine and missing her home comforts she self discharges. Later we find out that she has collapsed in the car park and is rushed back in to hospital. She is complaining of a fast heart rate and the nursing staff report a rapidly falling blood pressure. Her surgeon Dr Drew MacCaskill (played by Ben Barrington) decides to reoperate to investigate some internal bleeding, but during this Lynley has a cardiac arrest and dies. In the next episode we see Drew poring over Lynley’s pre operative assessment and his colleague notices some signs on her ECG that are indicative of Brugada syndrome.
Brugada Syndrome is a rare and poorly understood disorder of the heart’s electrical system. It is sometimes seen to run in families due to having a genetic cause. However it is often very hard to diagnose as the ECG signs are not detectable for much of the time, and the first (and only) symptom may be a dangerous arrhythmia of the heart causing a cardiac arrest or sudden death. When the ECG sign is detectable it is frequently misdiagnosed or considered an irrelevant observation, as in the case in Shortland Street. This is a tragedy since when someone is identified as having Brugada syndrome their risk of having a serious arrhythmia can be reduced in many ways. They should not receive medications or other substances that are known to be dangerous in Brugada Syndrome; they should undergo continuous ECG monitoring in situations such as operations. High fevers need to be brought under control and its important to avoid large amounts of alcohol, and a large meal just before going to bed.
The Cardiac Inherited Disease Group (CIDG) works to identify people in New Zealand who may have Brugada syndrome to ensure they receive the best possible treatment. They also attempt to identify the exact cause of the syndrome through the use of genetic testing, and help relatives of those affected to have their own risk assessed. The overall aim of this is to prevent people like Lynley from dying suddenly.
In the Shortland Street episodes the Doctors did not consider that identifying Brugada syndrome in Lynley after her death meant that her relatives should be informed of this, or that they would be at risk of sudden death themselves. Nor did they discuss possible genetic testing and the benefits this could have for her family.
In New Zealand a sudden death immediately after an operation would usually lead to a post mortem and Coronial review. Any time in which an inheritable cardiac condition is implicated in a death then the Coroner makes a ruling of the cause of death but also refers the case to the CIDG. CIDG then begins the process of supporting the relatives through their grief; explaining the meaning of the Coroners ruling; offer clinical assessment to all immediate relatives that wish it; and consider genetic testing if appropriate.
The Cardiac Inherited Disease Group wishes to thank TVNZ and Shortland Street for briefly highlighting this challenging condition. If you or your relatives have been affected by Brugada Syndrome and you wish to talk to somebody about it, consider contacting the CIDG Coordinator from your region. Find out who your local coordinator is by clicking here, or you can contact us.
Click here for more information on Brugada Syndrome