Born with achondroplasia, commonly referred to as dwarfism, Kapil Gala had multiple leg deformities and underwent eight surgeries on his feet to enable independent walking. Employed in the financial industry and managing a family independently, he faced a sudden onset of paraplegia a year ago, threatening his mobility and independence
From L-R: Dr Upendra Bhalerao, Dr Rajashree Agaskar, Khushboo Gala, Kapil Gala (patient), Dr Nihar Mehta and Dr Manish Kothari. Photo Courtesy: Jaslok Hospital and Research Centre
Mumbaikar Kapil Gala is a relieved man after the successful completion of a complex aortic valve replacement surgery earlier this month. Gala, a 39-year-old had achondroplasia, was treated Jaslok Hospital & Research Centre by a team of doctors who came together to address only the third such case in the world, according to the city hospital.
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Born with achondroplasia, commonly referred to as dwarfism, Gala had multiple leg deformities and underwent eight surgeries on his feet to enable independent walking. Employed in the financial industry and managing a family independently, he faced a sudden onset of paraplegia a year ago, threatening his mobility and independence.
The Mumbaikar was then admitted under the care of spine surgeons Dr Raghvendra Ramdasi and Dr Manish Kothari, Dr. Nihar Mehta, associate director of the Structural Heart Department at the hospital.
Gala was diagnosed with severe aortic stenosis during the pre-operative workup for spinal surgery. His bicuspid aortic valve, a congenital defect, necessitated urgent intervention before any spinal surgery could be considered.
Speaking to mid-day.com, Gala said, "I have faced many challenges due to my health conditions since childhood. However, this aortic stenosis would give me dizziness, blackouts which unbalanced my regular routines as well. At Jaslok Hospital, Dr Nihar and Dr Upendra along with Heart team gave me the right option of open heart valve surgery ensuring me a good quality life."
A multidisciplinary team, including Dr Mehta, Dr Anand Bhabhor (additional director- Critical Care), Dr Upendra Bhalerao (consultant-Cardiovascular & Thoracic Surgery), Dr. Rajashri Agaskar (consultant- Cardiac Anaesthesiology), Dr Kothari (consultant - Spine Surgery & Endoscopic Spine Surgeon), and Dr. Purnima Shah (Consultant- Neurology & Neuromuscular Specialist), came together to discuss the best course of action after Gala came in for a spine surgery.
However, in a press conference with the media, Dr Kothari said as they do a health checkup before the surgery, they found that Kapil had a weak heart. After a thorough review of echocardiograms, blood tests, CT scans, and MRI reports, the team identified several key concerns during the consultation. Kapil's bicuspid valve lacked the necessary calcium for a minimally invasive TAVI procedure. Additionally, contractures and small arteries in his feet made TAVI a less suitable option. His lung function, however, was deemed capable of handling surgery with pre-surgery exercises. Anaesthesia posed significant risks; extending his neck for intubation could lead to spinal compression and quadriplegia. Furthermore, the surgical team faced the challenge of operating without affecting his spine or legs, necessitating continuous monitoring of muscle and nerve signals throughout the procedure.
The team concluded that an open-heart surgery to implant a mechanical (metal) valve, which could last approximately 15-20 years, was the safest and most effective option. Dr Mehta performed a coronary angiography to ensure no blockages were present. With careful planning and the use of a flexible video laryngoscope and conscious sedation Patient was anaesthetised. The Aortic valve replacement surgery was successfully conducted by Dr Bhalerao.
Commenting on this case, Dr. Nihar Mehta said, "Our primary concern was the patient's safety and long-term health. We knew that TAVI was not the best option due to the anatomical challenges. It was crucial to provide a solution that ensured his survival and quality of life. We are delighted that Kapil trusted us and chose to proceed with the open-heart surgery."
Dr. Upendra Bhalerao added, "Performing open-heart surgery on a patient with such unique challenges required meticulous planning and execution. This was high risk surgery and posed considerable technical challenges due to anatomical variations and neuromuscular deformities. The successful outcome is a testament to our team's dedication and expertise."
Dr. Manish Kothari remarked, "It was a complex case, but seeing Kapil recover and regain his mobility is incredibly rewarding. His journey exemplifies the importance of interdisciplinary collaboration in medicine.” Jaslok Hospital is honoured to have played a part in enhancing Mr. Kapil’s quality of life. This case exemplifies the dedication and compassion of our medical team and reaffirms our commitment to delivering exceptional patient care."
Elated with his progres, Gala said, “Dr Nihar was like a brother to me who gave me the correct advice of not getting a TAVI done despite being a TAVI specialist to ensure my safety and long-term quality life. Dr Upendra Bhalerao, Dr Manish Kothari, nurses and entire team of Jaslok Hospital treated me as a family. I am standing so comfortably today because of their hardwork.”
The doctors have said Kapil's surgery was a success. He was off life support within 12 hours, walking with crutches by the second day, and discharged on the seventh day. The doctors said it further helped because Gala had a positive attitude and trust in the medical team were pivotal to his recovery. He continues to support his family and live a fulfilling life. The new valve will not hinder any future surgeries, including potential spinal surgery to further improve his mobility.
While Gala's surgery has been successful, does every person suffering from dwarfism and suffering from achondroplasia need such a surgery or is this a special case? Speaking to this writer, Dr Mehta explained, "Achrondroplasia is not associated with Bicuspid aortic valve stenosis. The combination is exceptionally rare Bicuspid aortic valve itself implies that our aortic valve has two leaflets from birth instead of the naturally occurring three leaflets. This exposes the valve to stressors of flow throughout the life and leads to leakages or choking of the valve between ages of 50-60 (or even older). For our patient to have achrondroplegia with cervical myelopathy with aortic stenosis was exceptionally rare which is why it needed a combination of specialists , a team based decision , out of the box planning and meticulous execution for a successful outcome."
This is a very very rare case where a person suffering from dwarfism and achondroplasia with cervical myelopathy has developed aortic stenosis. Only two cases are reported in scientific literature so far. The couple faced two major challenges. Firstly, the life threatening problem where aortic stenosis was causing him fainting attacks and chest pain so that he can't do his daily activities. But now, after heart valve replacement, he is doing everything without any symptoms. Secondly, the risk of being bedridden and losing power in all four limbs causing loss of earning and becoming dependent on someone if he develops any issues in surgery, . However, we could avoid all complications because of meticulous planning and team work," Dr Bhalerao concluded.
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