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Rare and High-risk surgery performed on a patient with Eisenmenger’s Syndrome

Forty-year-old Aparna, came to Fortis Malar, with excruciating pain in the lower abdomen, with loss of weight and loss of control over bowel evacuation and increased frequency of urination. Aparna had been diagnosed with multiple fibroids and as she was found to be unfit for surgery, she underwent ‘Uterine Artery Embolization’ in 2010 and 2011, which helped to reduce the size of the fibroids. Following this procedure, the patient developed ‘Deep Vein Thrombosis’.

Apart from these medical complications Ms Aparna, had heart problems from the age of 6 months. She suffered from Ventricular Septal Defect, a congenital heart defect with a large hole between two chambers of the heart. Over a period of time, when the increased pressure of the blood flow in the lung (ie pulmonary artery pressure) becomes very high, the direction of blood flow gets reversed and Eisenmenger syndrome occurs. Oxygen-poor (blue) blood from the right side of the heart flows into the left ventricle and is pumped so the body doesn't receive enough oxygen to all the organs and tissues. This makes the patient’s complexion acquire a bluish tint. Moreover, the patient was also prone to Deep Vein Thrombosis which is a dangerous condition that causes the blood to clot in her veins.

In Aparna’s case the hole which was detected at the age of 6 months was not closed. The flow of blood between left-to-right changed to right-to-left ventricles in the due course. At this stage the right side heart pressure increased and the right-to-left shunt also became rampant to cause Eisenmenger’s Syndrome. Because of the high pressure in the right side of the heart, which is transcendent to the lung, it is not treatable at this stage.

A surgical intervention involved high amount of risks because of the pre-existing Eisenmenger’s Syndrome, and the risk of Deep Vein Thrombosis, however it was the only solution for the patient’s plight. Dr Nithyaa discussed various aspects of the patient’s heart condition with the Interventional Cardiologist Dr Madan Mohan. A detailed study of the complications that may arise during a surgery was done and Dr Nithyaa made a fool-proof plan for this high-risk procedure in concurrence with Dr Suresh Rao and Dr Madan Mohan to ensure that everything from the cardiology point of view was stable. The medical team explained all the complications and expected outcome of the surgery, to the patient and her family to get them into confidence for the operation.

Dr Nithyaa led the team of surgeons that included, Dr Deepak Subramanian, General Surgeon and Dr Guru Balaji, Urologist, who performed the surgery with Dr Suresh Rao managing the Anaesthesia for this high-risk case. Under general anaesthesia, urinary catheter was inserted in view of big fibroid which was compressing both ureters causing ‘hydronephrosis’ (swelling of kidneys due to the accumulation of fluid ). In the surgery, a fibroid of 18-20 cms was identified. Bowels that were densely adhered to the uterus were gently separated and bilateral salphingo ooprestomy was performed. The abdomen closed in layers and the post-operative period was uneventful without any complications. The patient recovered well and was discharged with diet advice and medications. Ms Aparna is now thankful to her new life which was possible through surgical expertise of clinicians and state of the art facility at Fortis Malar

Senior Gynaecologist and Obstetrician Dr Nithyaa Ramanurthy said “Aparna came to us with great pain and discomfort, the treatment was a challenge medically since there is very less information available on surgical procedures performed on patients with Eisenmenger’s Syndrome. The team of experts from various specialties worked out specific plans to manage any contingency arising during the surgery so that clinical outcomes are good”

Dr Madhan Mohan, Senior Consultant – Interventional Cardiology said “We assessed the patient and found her to be suffering from very high pressure in the pulmonary artery. While the patient was diagnosed with Ventricular Septal Defect at the age of 6 months, her condition has since then deteriorated and has caused in the reversal of blood flow, which has further complicated the issue and has become untreatable. The appeal from us would be ‘do not neglect a child diagnosed with the ventricular septal defect, to ensure a better life for the child in adulthood”

Dr Suresh Rao, Senior Consultant – Cardiac Anaesthetist said “The challenge in managing Aparna, during the surgery, was to maintain her blood flow into lungs for better oxygenation, since her lung pressure was very high. At the same time, we needed to keep her blood pressure in the normal range”.

Mr Raghunath, Director, Fortis Malar Hospital said “While patients like Aparna face the burden of living a painful life due to multiple health issues, more than often it becomes difficult to manage them medically due to the absence of a multi-disciplinary approach to the treatment. Fortis Malar is well equipped to deal with medical conditions that require such an approach with the presence of experts in various fields of medicine, in this case a Senior Gynaecologist, a Senior Interventional Cardiologist, Urologist, General Surgeon and a vastly experienced Cardiac Anesthetist came together to provide a new lease of life to Aparna”.

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