ARVD/ARVC WEB PAGE

ARVD/ARVC Arrhythmogenic Right Ventricular Cardiomyopathy

Research at St.Georges Hospital Medical School, London United Kingdom.

e-mail to mnorman@sghms.ac.uk

 

DEFINITION

ARVD Arrhythmogenic Right Ventricular Dysplasia

ARVC Arrhythmogenic Right Ventricular Cardiomyopathy

The two terms are interchangeable when doctors talk about the disease.

Arrhythmogenic refers to the disease having clinical symptoms resulting from abnormal heart rhythms.

Right .The disorder is characterised by fibrofatty replacement of the right ventricular myocardium. Distinctive yellow areas of fat are seen replacing the heart muscle. It can occur on either side of the heart, but has a predilection for the right side, and for the larger chamber of the heart the right ventricle. Hence the term right ventricular.

Ventricular. There are two chambers to the heart. The ventricle is the larger chamber and receives blood from the smaller chamber the atrium. The atrium is the chamber collecting blood coming to the heart and the ventricle pumps blood away from the heart. On the right side of the heart blood comes back from the body to the heart. Blood is then pumped from the right ventricle to the lungs. From the lungs the blood returns after having become oxygenated to the left atrium and then ventricle and then pumped around the body.

Dysplasia means a developmental problem

Cardiomyopathy means a heart muscle disease.

In ARVD/ARVC doctors refer to the same condition. Essentially fat replaces the heart muscle.

The reason the term Cardiomyopathy is being used is that there is a genetic basis to the disease. Families are seen with ARVC with an autosomal dominant inheritance pattern. Several chromosomes are linked with the disorder, chr14, chr1, chr2 and chr 17. It is hoped that over the next few years the genetic basis of the disease will be determined. At St.Georges Hospital Medical School ongoing work is trying to identify families with the condition so that correct treatment can be given and that the gene may be found.

If you are someone with ARVC or are within a family with a relative with ARVC we would like to hear from you. An ongoing screening programme of families with ARVC is sponsored by the British Heart Foundation.

E-mail to mnorman@sghms.ac.uk

In terms of screening for the disease several tests are done including an electrocardiogram, an echocardiogram, signal averaged ECG, exercise testing, Holter monitoring and cardiac MRI scans. Please see below for examples of these:

 

ARVC ECHOCARDIOGRAM enlarged right ventricle is seen in the top of the picture. The diagram shows the normal relative size of the right ventricle RVOT1, as can be seen in the echo this is enlarged to 7cm, the normal size is 3cm.

The ECG in ARVC can show a characteristic feature, T wave inversion in the precordial leads, or incomplete right bundle branch block.

A cardiac MRI scan can show areas of fatty infiltration. In an MRI scan normal muscle appears grey and fatty tissue white. Below a scan of the heart using MRI has a similar orientation to the echo shown above. The right ventricle is at the top of the picture, the left ventricle at the bottom. As can be seen half of the wall of the right ventricle is replaced by fat in a young patient with ARVC.

ARVC can present with serious heart rhythm disturbance or even collapse. Patients may describe light-headedness, blackouts, especially on exercise. Below a 24hr tape recording of a patient with ARVC shows an abrupt change from the regular heart rhythm to a very fast heart rhythm called Ventricular Tachycardia. Although potentially a serious rhythm disorder it is usually controlled by beta-blockers in the majority of ARVC cases. Rarely people with ARVC have implantable defibrillators, a type of pacemaker, which senses the abnormal heart rhythm and delivers an internal electric current to correct the heart rhythm.

To go to the Hypertrophic cardiomyopathy page: http://ourworld.compuserve.com/homepages/lgdcruz/hcm -

 

Link to St. Georges Hospital Medical School WEB page http://www.sghms.ac.uk/ - link to St.Georges Hospital Medical Sch

For further info or questions on ARVC email to mnorman@sghms.ac.uk

 

Page created by Dr.Mark Norman of St.Georges Hospital Medical School. The information supplied and views given are those of Dr.Norman and not of the institution. E-mail mnorman@sghms.ac.uk