Inherited cardiac conditions

What is the impact of inherited cardiac conditions?

If left undetected and untreated, inherited cardiac conditions can lead to sudden death at a young age from a cardiac arrest. Around 500 young people die every year in the UK as a result of a genetic heart disorder.

This is their major complication, as they are often missed or not managed appropriately, especially when the individual was otherwise healthy and/or asymptomatic and the family history was not mapped and managed.

Although it can be difficult to predict a sudden death, the current knowledge and experience on these diseases allow us to identify the majority of patients at risk and prevent further complications.

Other complications, such as stroke, non-fatal arrhythmias and heart failure can also be evaluated and managed. Now that it’s possible to prevent these complications as soon as the risk is identified, the process of risk stratification needs to be performed continuously and meticulously.

Symptoms of inherited cardiac conditions

The inherited arrhythmia syndromes, such as Long QT syndrome, typically affect the electrical channels of the heart cells, causing electrical disturbances. Meanwhile, aortopathy and the associated connective tissue diseases are the conditions that affect the large arteries within the body.

Additionally, inherited conditions include pulmonary hypertension and lipid disorders such as familial hypercholesterolaemia (lipidopathy).

Diagnosing inherited cardiac conditions

Diagnosis of inherited cardiac conditions can be difficult for a variety of reasons, including the variability of the disease expression, their complex nature and the subtlety of some of their features.

The diagnosis for many of these diseases is based on a combination of markers from different diagnostic areas such as:

• clinical and family history
• genetic
• electrocardiographic
• cardiac imaging
• histological
• arrhythmic

The most common diagnostic tests include:

• ECG
• echocardiogram
• exercise test
• angiogram
• MRI scan
• genetic testing (where appropriate, and supported by genetic counselling)

Additionally, these tests are carefully interpreted for the risk assessment of these patients.

We can provide all diagnostic tests in a one-day assessment in order to ensure the best possible service for patients.

Inherited cardiac conditions treatment

The management of patients with inherited cardiac conditions depends on their clinical expression and risk profile. It ranges from monitoring, to medical and interventional treatment.

Certain details of the patient’s history and the results of the investigation can be helpful for the specialist cardiologist to choose the appropriate patient management pathway.

Fortunately, interventions and appropriate management have substantially improved the outcome for patients with inherited cardiac conditions. Treatments can include:

• lifestyle changes
• medication
• implantable devices to correct arrhythmias
• nonsurgical procedures
• surgery

What makes us different?

Due to their nature, the clinical experience with these diseases is limited to a few centres around the world.

Our multi-disciplinary team at Royal Brompton and Harefield hospitals includes cardiologists, surgeons, genetic specialists, neurologists, imaging experts, psychologists, and specialist nurses. The team work closely together in joint clinical meetings and are supported by a cutting-edge research programme.

Together they deliver an integrated day case diagnostic service to children, adults and families and a complete programme for the management of patients.

The team works closely with each patient and family to provide complete information on their condition and the available treatment options. An important aspect of the service is the screening of family members of all ages for these inherited conditions.

Families can be seen together, easing the burden for the family in terms of travel and communication difficulties. This provides for a completely integrated care plan for both individuals and families affected by these conditions.