Prof Sanjay Sharma BSc(Hons), MD, FESC
Inherited Cardiac Diseases
![](https://static.wixstatic.com/media/7bec3a_c77516702620403a9c1780a4d7238648~mv2_d_3675_2176_s_2.jpg/v1/fill/w_350,h_222,al_c,q_80,usm_0.66_1.00_0.01,enc_auto/7bec3a_c77516702620403a9c1780a4d7238648~mv2_d_3675_2176_s_2.jpg)
There are over 250,000 people with inherited cardiac diseases in the UK. The most feared complication of many of these diseases is sudden death in the first three decades of life. Early detection and risk stratification has the potential to prevent catastrophies and identify other family members who might be affected. I specialise in inherited cardiac diseases and have a particular interest in cardiomyopathy, ethnic differences in cardiovascular disease, ion channel disorders and Sudden Arrhytthmic Death Syndrome (SADS). Our tertiary consultant-led inherited cardiac disease clinic (ICDC) at St George's Hospital evaluates patients with familial cardiac conditions. The clinic is nationally reputed and supports the S.E London area and receives referrals throughout UK.
Hypertrophic cardiomyopathy (HCM)
If a parent has an abnormal gene then each child has a 50% chance of inheriting HCM, Treatment is aimed at preventing complications and improving symptoms. Treatment can be obtained through lifestyle modification advice, drugs, specialised pacemakers or in some cases, surgery.
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)
The child of a parent with ARVC has a 50% chance of inheriting the condition. ARVC can be treated by modifying your lifestyle. Drug treatment may be used to control the irregular heart rhythms and a defibrillator may be fitted for those deemed to be at high risk from sudden death.
Long QT syndrome (LQTS)
Long QT syndrome (LQTS) is an ion channelopathy which affects the electrical functioning of the heart without affecting the heart’s structure. It affects about 1 in 2,000 people. Gene testing has a very important role in LQTS, and will confirm 70% of cases. Beta-blockers provide effective therapy, particularly in LQTS Type 1 cases.
Catecholaminergic polymorphic ventricular tachycardia (CPVT)
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare condition that has been associated with two genes that make proteins found inside the cell. The diagnosis is usually made after the recording of arrhythmias that are characteristic of CPVT, while the person is doing exercise.