Heart Matters: Cardiac Risk in Young, a forgotten entity in India

Surya was a just married happy young woman.  When some unusual palpitations disturbed her she assumed they were just nerves due to her new role. When they persisted, she consulted a doctor.   

An ECG and ECHO test later, the doctor told her that she suffered from Hypertrophic Cardiomyopathy (HCM) or Sudden Cardiac Death Syndrome.  Surya was thunderstruck and felt her world crumbling around her. She was referred to the dedicated Cardiac Risk in Young (C.R.Y.) clinic at Frontier Lifeline Hospital lead by Dr Rajaram Anantharaman. Following family counseling and treatment advice, she felt hopeful and went about her normal life. She was reassured that only a fraction of HCM patients developed serious life threatening arrhythmias, and that with regular follow-up and a special pacemaker (Implantable Cardioverter Defibrillator ICD), sudden death could be avoided. 

CRY - Cardiac Risk in the Young: Increasingly young people less than 40 years old are dying suddenly of Sudden Cardiac Death (SCD). Over half of SCD is due to inherited heart disease like HCM. Dedicated CRY centers are available in Western countries to check the families who are at risk or who have had an SCD due to HCM in the family.  In India, the first CRY Clinic was started by Frontier Lifeline Hospital (FLL) by Dr. Rajaram Anantharaman, Sr. Cardiologist, FLL, Dr K M Cherian Heart Foundation in March 2015. 

“Hypertrophic Cardiomyopathy HCM is due to an error in the gene (genetic mutation or misspelling) coding for heart muscle protein. This leads to disor ganized heart muscle formation with thickening and causes a serious fast heart rhythm in some patients leading to sudden death. This genetic mutation is family specific. SCD can be prevented if people are aware of this syndrome and undergo appropriate tests. If a young person dies of SCD, then the rest of the family members must undergo simple tests like ECG (heart tracing) and Echo (ultrasound scan of heart) to find out if they are also at risk. 50% of the family members can be affected by the same genetic abnormality. Once the particular genetic abnormality in that family is detected, it  is easy to offer the genetic testing to the rest of the family to identify those at risk and prevent sudden cardiac death. The key is knowing and accepting!” says Dr. Rajaram Anantharaman. With able guidance, she made the necessary changes towards a healthier lifestyle. “Fortunately, I am almost normal now. I am leading a very healthy and happy life. I know that I can even conceive. Once my baby is born, I will need to check if the baby is carrying the same gene like me,” she said. 

This is inherited due to an abnormality in the gene coding for the contractile protein of the heart muscle. It is very specific for each family (private mutation). 50% of the family members might carry this mutated gene in them, but all of them need not necessarily have the condition.  H

Once an HCM patient who is at high risk of SCD is identified, a special defibrillator pacemaker (ICD) is implanted, which will prevent SCD. How can the obstruction in HCM be corrected? It can be corrected by reducing the thickened heart muscle by surgery (Myemectomy) and making the blood flow out of heart normal, as with a 6 year old girl, whose heart Dr. Cherian operated on to remove the obstruction from left side of heart on 8th March 2017 on International Women’s Day. The girl has recovered well. FLL also conducted a CME programme on International Women’s Day, to as part of its second year celebration of its CRY Clinic to train doctors and improve their knowledge about HCM, and “Open House” seminar for the general public to educate them about this.  

Everyone greeted the visibly happy Dr. Cherian as it was his 75th birthday as well. “I’m very blessed that on this International Women’s Day I was able to operate upon a 6 year old girl with this condition. She is doing very well.” On leaving, we asked the doctor what he thinks about women, he instantly answered, “Every Man is nothing but his Mother”. At 75, he is still a perfectionist. His hands are steady and his skills impeccable. How many of us will be able to stand properly at 75 or perform simple tasks such as writing neatly? Dr Cherian continues to live his life’s work and shows that age is no barrier. Happy Birthday Dr. Cherian. God Bless.