ICC in primary care

Inherited cardiac conditions services are provided across the South London Network.

Conditions treated in the inherited cardiac conditions service

In general we see patients with suspected inherited cardiac disease and first degree relatives (parents/siblings/children) of patients with confirmed inherited cardiac disease.

  • Inherited cardiomyopathies (inherited heart muscle disease)
    Dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic cardiomyopathy (ARVC/ALVC/ACM), restrictive cardiomyopathy, LV non-compaction cardiomyopathy, cardiomyopathy associated with Lamin and Filamin pathogenic gene variants
  • Inherited arrhythmia syndromes (inherited heart rhythm abnormalities)
    Common: Long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), Brugada syndrome (BrS)
    Rare: Short QT syndrome (SQTS), progressive cardiac system conduction disease (PCCD), early repolaristion syndrome (ERS), arrhythmogenic mitral valve prolapse
  • Unexplained cardiac arrest (also known as idiopathic ventricular fibrillation / idiopathic VF) and Sudden Arrhythmic Death Syndrome (SADS)
    Patients who have survived an unexplained cardiac arrest and first-degree relatives (parents/siblings/children) of patients who died due to sudden cardiac death
  • Aortopathies
    Marfan disease, vascular Ehlers-Danlos syndrome, Loeys-Dietz, aortic dilatation in association with a bicuspid aortic valve
  • Inherited neuromuscular disease with associated cardiac involvement
    Myotonic dystrophy, limb girdle muscular dystrophy, Becker muscular dystrophy, Duchenne muscular dystrophy female gene carriers, Emery-Dreifuss muscular dystrophy, cardiomyopathy associated with Lamin and Filamin pathogenic gene variants
  • Inherited adult metabolic disease with associated cardiac involvement
    Mitochondrial disorders, Anderson-Fabry disease

Tests frequently performed by the ICC service

  • 12 lead ECG (standard leads / high leads)
  • Signal averaged ECG (SAECG)
  • Echocardiogram
  • Holter monitoring (3 lead / 12 lead / 24 hours / 8 day event recorders)
  • Exercise testing (including cardiopulmonary exercise testing)
  • Cardiac MRI scan (CMR)
  • Ajmaline provocation test
  • Genetic testing and counselling

Referrals

Please see the individual trust guidance on how to refer a patient (links above).

Please include any relevant family history or genetic information with the referral – including cardiology clinic letters (for patient or first-degree relatives), death certificates / post-mortem reports and genetic test results.