What are inherited cardiac conditions (ICC)?
Inherited cardiac conditions (ICC) are a group of genetic disorders that affect the heart and major vessels, in particular the heart muscle (cardiomyopathies), the heart rhythm (inherited arrhythmia syndromes) and the aorta (aortopathy).
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).
What is the impact of inherited cardiac conditions?
If left undetected and untreated, an 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 (Source: NHS inform, accessed Jan 2019).
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.
Consultants who treat inherited cardiac conditions (ICC)
At Royal Brompton & Harefield Hospitals Specialist Care, a range of cardiologists can treat inherited cardiac conditions in private patients:
- Dr John Baksi - Consultant cardiologist
- Dr Piers Daubeney - Consultant paediatric and fetal cardiologist
- Dr Alexander Lyon - Honorary consultant cardiologist, Heart failure theme lead, cardiovascular biomedical research unit
- Dr Antonis Pantazis - Consultant cardiologist, Clinical lead for cardiomyopathy
- Dr Robert Smith - Consultant interventional cardiologist
- Dr Christoph Nienaber - Consultant cardiologist
- Dr Lorna Swan - Consultant cardiologist
- Dr Nitha Naqvi - Consultant paediatric cardiologist
- Dr Mahmoud Barbir - Consultant cardiologist
- Professor Alain Fraissé - Consultant paediatric cardiologist
- Dr Laura Price - Consultant respiratory physician
Diagnosing inherited cardiac conditions (ICC)
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
- cardiac imaging
The most common diagnostic tests include:
- exercise test
- MRI scan
- genetic testing (where appropriate).
Additionally, these tests are carefully interpreted for the risk assessment of these patients.
RB&HH Specialist Care can provide all diagnostic tests in a one-day assessment in order to ensure the best possible service for patients.
Treatment for inherited cardiac conditions (ICC)
The management of patients with inherited cardiac conditions (ICC) 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 ICC. Treatments include:
- lifestyle changes
- implantable devices to correct arrhythmias
- nonsurgical procedures
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 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 with ICC.
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.