It might be every parent’s worst nightmare, but it nonetheless happens with alarming frequency in Ireland. Every year, an estimated 40 otherwise healthy young people in this State will die from Sudden Adult Death Syndrome (Sads) or Sudden Arrhythmic Death Syndrome. Who does it affect – and is screening an effective way to protect you and your family?
What is Sads?
Sads is typically used to refer to a sudden death when a postmortem has shown no other potential cause of death. It is sometimes confused with a sudden cardiac death, which is when someone dies suddenly and unexpectedly, but there is a cardiac issue behind the death.
When it’s a sudden cardiac death, for example, a postmortem will typically show up problems such as a blockage in a coronary artery.
With a Sads death, however, there can be little to indicate what caused the death, which is often due to electrical problems in the heart known as “channelopathies” or “ion channel disorders”.
“There’s nothing to find on a postmortem and there’s no answer. It’s horrendous for a family,” says Dr Catherine McGorrian, a consultant cardiologist and physician with the Mater Misericordiae University Hospital.
How common is it?
With about 40 deaths a year in Ireland, deaths from Sads are not as common as might be perceived.
However, as McGorrian notes, while they might be rare, “they are also absolutely devastating”.
Moreover, when it comes to deaths of those aged under 35, Ireland appears to have a higher incidence than other countries. “We do have numbers that may suggest we have an elevated rate in Ireland, but we don’t really know why,” she says.
Is there a screening programme?
The Family Heart Screening Clinic at the Mater is a charitable venture and survives only because of donations.
Given its limited resources, it focuses on screening high-risk patients – families where there has been a diagnosis of a condition, or where someone old or young died for no known cause.
While there have been many calls for a broader screening programme in Ireland, the challenge is who would do it – and, more importantly, who would pay for it.
There is little public funding for screening, and while the Government published a report by a taskforce on sudden cardiac death in 2006, little has been done since. As McGorrian notes, while the report made some “great recommendations”, it has largely languished since publication.
The one area where there has been progress is in screening for high-risk patients, which are carried out in the Mater and at the Cry screening centre in Tallaght.
It is for these reasons that Ed Donovan founded Heartaid, which provides a mobile screening service, 2½ years ago.
Typically, he provides a screening service in a group setting, and given high-profile Sads deaths among GAA athletes, has joined up with the Gaelic Players Association to screen all inter-county players.
What are the risk factors?
For McGorrian, of crucial importance is knowing your family history.
“The conditions that we look for run in families and typically Sads conditions are genetic,” she says, adding that while there is a perception that younger men are more at risk, it can affect people throughout their lives, and you can’t generalise.
It is also thought to affect sportspeople more commonly.
In Italy, for example, Donovan notes that it’s mandatory for anyone playing competitive sport to undergo cardiac screening and this has reduced incidences by as much as 89 per cent.
“If you have a heart problem and are competing at very high level, then the sport doesn’t cause the problem but it can trigger it in the person who has that tendency,” says McGorrian.
However, she adds that it isn’t actually more common in athletes. “We counted the Sads [incidents] over three years – what we found is only a minority of events happened to young people during sports,” she says.
Are there any correlations with ‘Sids’?
According to McGorrian, there is a “potential” connection between Sads and sudden infant death syndrome (Sids), or cot death.
In this respect, families who may have experienced a Sids death are considered to be at higher risk and appropriate for Sads screening.
What are the warning signs?
Many of the genetic conditions behind Sads are silent, but warning signs would include a history of unexplained collapses; a collapse or bad palpitation during sport; and severe palpitations.
Is screening effective?
“No one comes to hear they’ve a problem,” says McGorrian of patients at the Family Heart Screening clinic.
However, if there is a condition in your family, the screening process can reveal it and many such conditions will be treatable and manageable. “Diagnoses are tough for people to hear, but I say it’s a positive step for your family because we will do our very best to make sure a tragedy doesn’t happen again,” she says.
Screening typically involves a health questionnaire, an ECG and medical examination with Heartaid, while for those in the high-risk category, it can involve additional steps.
How much is screening?
The service at the Mater hospital is free, but due to restricted capacity is limited to those at high risk. If you are concerned that this might include you, you can discuss it with a family heart screening specialist on the phone to assess the risks.
Alternatively, Laya Healthcare, in conjunction with Heartaid, offers a screening free of charge to its customers. According to Donovan, clinics are held regularly in Dublin, Cork and Galway.
You can also arrange for the Heartaid mobile service to screen a group of people such as a sports team or organisation. Doing so will cost about €75 per person, according to Donovan, depending on the numbers being screened.
