Royal Brompton & Harefield Hospitals



One of the largest Inherited Cardiac Conditions centres in the UK delivers leading diagnostic and management services

Due to the complex nature of Inherited Cardiac Conditions (ICC), the clinical experience with these diseases is limited to a few centres around the world. Royal Brompton & Harefield Hospitals (RB&HH) is one of the largest centres for the diagnosis and management of patients with ICC in the UK. Our multi-disciplinary team includes the leading specialists in this field.

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). Additional inherited conditions include Pulmonary Hypertension and lipid disorders such as Familial Hypercholesterolaemia (Lipidopathy).

The frequency of all ICC combined is approximately 1:100 worldwide and in many cases lead to sudden death at a young age. Although the prediction of sudden death can be challenging, the current knowledge and experience on these diseases allow us to identify most patients at risk and prevent further complications.

Diagnosis of these conditions can be difficult and for many of these diseases is based on a combination of markers from different diagnostic areas. The most common diagnostic tests include ECG, echocardiogram, exercise test, angiogram, MRI scan and genetic testing, where appropriate. For those patients attending our clinic, we can provide all diagnostic tests in a one-day assessment to ensure the best possible service for patients.

The management of patients with ICC depends on their clinical expression and risk profile. It ranges from monitoring, to medical and interventional treatment. Certain details of the patientsí history and the results of the investigation can be helpful for the specialist Cardiologist to choose the appropriate patient management pathway.

In particular, our recently ISO15189-accredited Clinical Genetics and Genomics laboratory offers diagnostic testing for families and individuals at risk of inherited diseases, including Cardiomyopathy, Familial Aortopathies and Arrhythmias. The molecular genetic analysis of ICCs using a comprehensive repertoire of next-generation sequencing-based tests is essential for a complete clinical diagnosis. This informs the further care of ICC patients and enables the testing of their relatives, a service international referrers can benefit from too.

Fortunately, interventions and appropriate management have substantially improved the outcome for patients with ICC.

 

 

Date of upload: 20th Jan 2017

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