MGM Healthcare Performs Complex Mitral Valve Replacement on 14-Year-Old with Rare Heart Condition
Chennai: Surgeons at MGM Healthcare have successfully performed a complex mitral valve replacement on a 14-year-old girl diagnosed with a rare combination of heart and metabolic disorders that had left her largely bedridden with severe breathlessness.
According to the hospital, the patient was suffering from severe mitral regurgitation accompanied by left ventricular outflow tract (LVOT) obstruction. The damaged mitral valve caused blood to flow backward into the left atrium instead of circulating efficiently through the body, placing excessive strain on the heart. The narrowing of the heart’s outflow tract further reduced blood flow, worsening her condition.
Rare Combination of Medical Conditions
The surgery was considered highly challenging because the patient had mesocardia, a rare congenital condition in which the heart is positioned at the centre of the chest, with its apex pointing toward the midline rather than the left side. This unusual anatomy made surgical access to the mitral valve significantly more difficult.
The patient was also diagnosed with Morquio syndrome, a rare inherited metabolic disorder that leads to the accumulation of complex sugars in the body. The condition causes progressive skeletal abnormalities, including instability of the upper cervical spine, requiring specialised precautions during anaesthesia and surgery.
According to MGM Healthcare, several hospitals had declined to perform the procedure because of the complexity of these combined conditions.
Complex Open-Heart Surgery
The surgery was performed by Dr. R.K.R. Noveen Davidson, Senior Consultant, Paediatric Cardiac and Heart & Lung Transplant Surgeon.
During the open-heart procedure, surgeons accessed the mitral valve by making incisions through the upper heart chamber and surrounding structures, including the Superior Vena Cava Area (SVCA), Infundibular Basal Septum (ICB), and part of the Left Atrium (LA). The diseased mitral valve was replaced with a 25 mm mechanical valve, and the left ventricle was resected before the surgical incisions were closed.
The operation also relieved the left ventricular outflow tract obstruction and restored normal blood flow through the heart.
Patient Recovering After Surgery
Dr. Noveen Davidson said it is extremely rare for a patient with Morquio syndrome to also have mesocardia. He stated that the girl’s severe breathlessness had forced her to discontinue her studies and remain confined to bed.
He added that the patient is recovering well following the surgery and is expected to require three to six months for complete recovery.
Multidisciplinary Care
Commenting on the procedure, Dr. K. R. Balakrishnan, Chairman – Cardiac Sciences and Director, Institute of Heart and Lung Transplant & Mechanical Circulatory Support, said the surgery required close coordination between multidisciplinary surgical and anaesthetic teams. He noted that the current focus is on the patient’s recovery and continued support for her family.
Speaking about post-operative care, Dr. Suresh Rao K.G., Co-Director, Institute of Heart and Lung Transplant & Mechanical Circulatory Support, said managing patients with Morquio syndrome is particularly challenging due to severe upper airway abnormalities, cervical spine instability, and compromised heart and lung function. He added that the child also had a restrictive lung defect and required close monitoring after surgery.
