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Inherited Cardiac Conditions

Checked: 23-04-2022 by Rob Adams Next Review: 23-04-2024

Overview

Inherited Cardiac Conditions are important to recognise as they can be a cause of sudden death. These conditions are known as Sudden Arrhythmic Death Syndromes (SADS). (BHF website)

Inherited heart conditions which may cause cardiac arrest if undetected and untreated include:

Hypertrophic cardiomyopathy (HCM)
Dilated cardiomyopathy (DCM)
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
Long QT syndrome (LQTS)
Brugada syndrome
Catecholaminergic polymorphic ventricular tachycardia (CPVT)
Progressive cardiac conduction defect (PCCD).

Who to refer

First degree relatives of a patient who has had a Sudden Arrhythmic Death should be referred to a cardiologist for clinical screening. These patients will usually have been informed that they need to be referred and should be provided with a letter requesting referral from the cardiologist

Referrals must come from a GP via eRS.

If you are unsure if a patient needs a referral then you can request Cardiology Advice and Guidance.

If a pathogenic mutation has been identified then 1st degree relatives should be referred to Genetics.

What to do before referral

GPs should ideally arrange an ecg and echocardiogram prior to referral as these investigations can help the cardiologists prioritise the most at risk patients.

Please include the introduction letter from the cardiologist or details (name and DOB) of the relative(s) who has died (or been identified as having an inherited condition).

Services

Cardiology Referral

Bristol Heart Institute has cardiologists with an interest in inherited cardiac conditions (ICC) and work with the local genetics service. Referrals should be made via eRS (Inherited Cardiac Conditions – University Hospitals Bristol – RA7). Please indicate on the referral that then BHI is the preferred provider if you feel this service is most appropriate.

NBT has cardiologists who can also assess patients with a possible inherited heart condition. Referrals should be made via eRS into the general cardiology clinic. Please indicate on the referral that NBT is the preferred provider if appropriate.

Genetics Referral

A referral to the Genetics RAS via eRS should be made for 1st degree relatives where a pathogenic mutation has been identified.

Resources

The British Heart Foundation website has a useful summary of SADS including a Patient information leaflet

CRY (Cardiac Risk in the Young) has a website with information about inherited cardiac conditions.

SADS.org.uk also has some information for patients and their families.

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