Inherited cardiac conditions service

Providing high quality care and psychological support for the patient

Patient with family and nurseThe aim of our inherited cardiac conditions (ICC) service is to improve the diagnosis, treatment and outcome of patients with inherited heart disease. We focus on timely diagnosis, high quality care and psychological support for the patient and their family.

We offer clinics at Guy’s and St Thomas’ and King’s College Hospital. These clinics integrate clinical, familial and genetic assessment in a ‘one-stop’ service which aims to minimise the number of visits our patients need to make to hospital.

For further details about what happens when you come to clinic, please see our inherited cardiac conditions information leaflet (PDF 187Kb).

We work closely with our children's colleagues at Evelina London Children's Hospital to provide smooth transition of care from childhood, to adolescence through to adulthood.

We are also part of King’s Health Partners Academic Health Sciences Centre which brings together research, clinical excellence and world-class teaching to deliver groundbreaking advances in healthcare.

Inherited cardiac conditions

Inherited cardiac conditions are familial disorders that can affect the heart muscle and the heart rhythm. Our team of doctors and other healthcare professionals (multidisciplinary team) provide treatment and support for a range of conditions.

It is important to have specialist input for the diagnosis and treatment of these conditions and for the screening of family members because of the complex clinical and psychosocial issues involved.

  • Inherited heart muscle conditions

    Inherited heart muscle conditions are also known as cardiomyopathies.

    These include:

    • hypertrophic cardiomyopathy (HCM)
    • dilated cardiomyopathy (DCM)
    • arrhythmogenic right ventricular cardiomyopathy (ARVC) / arrhythmogenic cardiomyopathy (AC)
    • restrictive cardiomyopathy (RC)
    • left ventricular non-compaction (LVNC)
    • PRAKAG2 associated cardiomyopathy
    • AMPK related dilated cardiomyopathy (DCM)
    • genetic infiltrative conditions such as Danon disease, Fabry disease and transthyretin (TTR) amyloid.

    These conditions can lead to heart failure.

  • Inherited heart rhythm conditions

    Inherited heart rhythm conditions are also known as inherited arrhythmia syndromes.

    These include:

    • long QT syndrome (LQTS)
    • short QT syndrome (SQTS)
    • Brugada syndrome (BrS)
    • sudden arrhythmic death syndrome (SADS)
    • catecholaminergic polymorphic ventricular tachycardia (CPVT)
    • some forms of inherited Wolff-Parkinson-White (WPW) syndrome
    • idiopathic ventricular fibrillation (VF) arrest

    These conditions can cause problems with the heart rate and rhythm.

  • Other conditions

    Other conditions seen in the ICC service include:

    • inherited neuromuscular disease associated with cardiac involvement – myotonic dystrophy, limb girdle muscular dystrophy, Emery-Dreifuss muscular dystrophy, Becker muscular dystrophy and Duchenne muscular dystrophy
    • inherited  aortopathies – Marfan syndrome (MFS), Loeys Dietz syndrome (LDS), Ehlers Danlos syndrome (EDS), Arterial Tortuosity Syndrome (ATS), Turner Syndrome (TS), non-syndromic thoracic aortic aneurysms and dissection (TAAD) and bicuspid aortic valve associated aortic aneurysm and dissection
    • mitochondrial disorders with cardiac involvement.

Facilities and services

We have access to the following:

  • facilities for minimally invasive electrophysiology investigation (EP studies and implantable loop recorder (ILR) (PDF 153Kb) insertion) and coronary artery investigation (coronary angiography) (PDF 466Kb)
  • diagnostic ajmaline testing for patients with suspected Brugada syndrome
  • facilities for permanent pacemaker (PPM) and implantable cardioverter defibrillator (ICD) insertion
  • genetic testing, including specific blood tests which relate directly to ICCs.
 

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