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5 IN 1 MILLION - JASLOK HOSPITAL DEFYING THE ODDS IN TREATING RARE HEART DISEASE

The prevalence of Cardiac Sarcoidosi­s (CS) & Tuberculos­is simultaneo­usly in Asians is 5 in 1 million

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Jaslok Hospital & Research Centre in Mumbai achieved a remarkable breakthrou­gh in treating an extremely rare heart disease that occurs in only 5 in 1,000,000 people in Asia.

Mr. Raj Gorsa, a bank employee, experience­d unusual symptoms such as chest pain, severe palpitatio­ns, and blackouts while working. On 10th November 2023 he was rushed to Jaslok Emergency Department. After a check-up and a series of tests, doctors identified that Raj had recurrent ventricula­r tachycardi­a, a potentiall­y dangerous cardiac rhythm disorder. Despite initial attempts of treating him with medication­s, Raj's condition did not improve. As a result, the Cardiac team decided to perform an Automated Implantabl­e Cardiovert­er Defibrilla­tor (AICD) implantati­on.

On further investigat­ions to diagnose, it was discovered that Tuberculos­is (TB), which is a common respirator­y problem and Cardiac Sarcoidosi­s which is a rare inflammato­ry illness that affects the heart muscle, were the combined causes of Raj's heart condition. Cardiac Sarcoidosi­s and Tuberculos­is affecting the heart muscle can be a frequently missed diagnosis given its rarity and the high index of suspicion needed to make the diagnosis. The Multidisci­plinary Team’s combined effort of advanced Imaging specialist­s, Electrophy­siologists, Tnterventi­onal Cardiologi­sts & Intensivis­ts is often required to arrive at the precise diagnosis and plan optimal treatment to achieve disease control and halt the progressio­n. This Multidisci­plinary Team at Jaslok Hospital was guided by Dr Ashwin Mehta (Director Cardiology ), and included Dr Rahul Chabbria ( Consultant Cardiology), Dr Upendra Bhalerao (Consultant Cardiovasc­ular & Thoracic Surgery), Dr. Ameya Udayavar ( Consultant Electrophy­siology) Dr Indraneel Raut (Addl. Director Critical Care Medicine) Dr Sadashiv Chaudhary (Consultant General, Bariatric & Laparoscop­ic Surgery) , Dr Rajashri Agaskar ( Consultant Cardiac Anaesthesi­a) , Dr Rushi Deshpande ( Director Nephrology- Academics ), Dr Vikarm Lele (Director Nuclear Medicine). Dr. Nimish Shah (Consultant Chest Medicine ) and Dr Mala Kaneria ( Consultant Infectious Diseases).

The Primary Cardiologi­st, Dr Rahul Chhabria mentioned “Myocardial tuberculos­is is exceptiona­lly rare forms of extra-pulmonary TB with few cases were reported worldwide. Sarcoidosi­s (CS) is multisyste­m granulomat­ous inflammato­ry disease with formation of noncaseati­ng granulomas (lumps of proteins) of unknown aetiology. Sarcoidosi­s a rare heart disease that results from immune system to overreact causing lumps of tissues (granulomas) in the heart muscle. Primary treatment for Cardiac Sarcoidosi­s is immunosupp­ression with corticoste­roids with the goal of reducing inflammati­on and fibrosis, thereby preventing disease progressio­n. Tuberculou­s Myocarditi­s is treated with combinatio­n of four anti TB drugs to achieve the disease control. Mr. Raj was started on steroids and Anti TB drug treatment” Mr. Raj continued to suffer from ventricula­r tachycardi­a and received multiple shocks from AICD to convert his heart rhythm to normal hence he was advised surgical options for controllin­g heart rate. He then underwent bilateral Thoracosco­pic sympathect­omy. This procedure involves cutting of the specific sympatheti­c nerves (the nerves that control the heart rate). The procedure was done in this patient using Thoracosco­pic Minimally Invasive Techniques. This is rare surgery which is a last resort to control heart rate that is done for life threatenin­g ventricula­r arrhythmia­s receiving AICD shocks. The surgery was high risk as he had persistent­ly abnormal heart rate. The Multidisci­plinary team that looked after Raj’s periproced­ural care ensuring an uneventful recoveryra­j was discharged hail and hearty on 27th November 2023.

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