Amit Soni, 30, is relieved to see his three-month-old son gaining weight slowly but consistently and his breathing pattern settling down Soni’s son, Aditya, who had a rare congenital heart and lung defect — scimitar syndrome, weighted only two kg before being treated at Fortis Hospital, Mulund, in January. Scimitar syndrome is characterised by an unusual arrangement of pulmonary veins, where the blood vessels takes the shape of a sword. Scimitar syndrome occurs in approximately one among a lakh live births. Scimitar syndrome, or pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung.This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as Scimitar syndrome after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava. This radiographic density often has the shape of a Scimitar a type of curved sword “Aditya was suffering from persistent breathing difficulty since birth. Doctors said it was pneumonia and that there were slim chances of his survival,” said Soni, a Kalyan resident. At Fortis Hospital several investigations were performed before Aditya’s defect was diagnosed. “The baby was suffering from shortness of breath, poor feeding, increased heart rate and low grade fever,” said Dr Sanah Merchant-Soomar, paediatric cardiologist, Fortis Hospital. “Scimitar syndrome needs to be treated soon, otherwise the child can develop complications such as pneumonia and lung infection. It could also lead to death,” said Dr Kaushal Pandey, cardiac surgeon, Hinduja Hospital. Aditya showed three severe complications. The right-side pulmonary veins, which carry oxygenated blood from the lung to the heart, were not attached to the left side of the heart. “This was causing the heart to work harder and could lead to heart enlargement,” said Merchant-Soomar. Due to the blockage of the aorta, the main blood vessel, the blood flow to the lower part of the body was less, which could lead to failure of kidneys and liver. His right lung had a large abnormal tissue growth, which put pressure on the lung. This lead to shortness of breath, said doctors. A large blood vessel was supplying blood to the tissue growth thus making the heart overwork. Doctors ruled out open-heart surgery as it was risky for the baby weighing only two kg. They performed a less invasive trans-catheter intervention to correct the defect. Aditya was discharged from hospital in mid January and is recovering well. “I had lost hope of seeing my baby alive. We are poor and I am trying to repay the loan that I had taken for his treatment,” said Soni, who works as a sales officer in Ulhasnagar. “Aditya will need surgery to correct the last defect and reinsert the pulmonary veins to the left side of the hear,” said Dr Merchant-Soomar. The severity of scimitar syndrome varies considerably. Some people have it and are entirely unaware, or have it diagnosed but a doctor determines that the effects are so minor that no action needs to be taken. In other cases, the defect causes health problems or puts the heart in danger, in which case surgery may be recommended to address the problem. This condition is also known as partial pulmonary venolobar syndrome.

A team of Neurosurgeons led by Dr. Sunil Kutty, Consultant Neuro & Spine Surgeon operated the dorsal spinal tumour on a 33 year old Mr Ravindra Ghatse, who was experiencing difficulty in walking, numbness & weakness in his legs.

Mr Ghatse was suffering from Neurofibromatosis type 2 (NF2), a genetic abnormality, which he inherited from his mother. Neurofibromatosis is a neurocutaneous syndrome, which gets passed down through the parents’ genes, affects the brain, spinal cord, nerves, skin, and other vital systems in the body. Of the two types of neurofibromatosis – NF1 and NF2, NF1 is more common. NF2 is characterized by the presence of bilateral acoustic neurofibroma (a type of brain tumor) and is rarer, seen in 1 in 45,000 births in India. People with NF2 usually develop benign tumors on the nerves responsible for hearing and balancing in their ears, causing hearing loss, eventual deafness, and problems with balance.

The disease was affecting Mr Ghatse’s, ability to walk and body balance. 3 months ago he had a fall while walking and was not able to get up. He visited Dr. Sunil Kutty at Fortis Hospital Kalyan and was detected to have multiple tumors i.e. a Dorsal cord neurofibroma causing spinal cord compression, a small Cervical neurofibroma, a small asymptomatic acoustic neurofibroma (Brain tumor), Peripheral neurofibroma (in right upper hand) and a tumour on the abdominal wall. This patient thus had five tumors and it seemed that there could be a few more tumors which were undetected or could generate later.

As per Dr. Sunil Kutty, Neurosurgeon at Fortis Hospital, Kalyan, “All the tumors seemed benign and most were not causing symptoms or affecting patients day to day activity hence there was no need to remove them, but the dorsal spinal cord tumor was gradually increasing and had become symptomatic causing him trouble in performing his regular physical activity. Hence we took a decision of removing the dorsal spinal tumour.”

The neurosurgeon talked at length with Mr Ghatse about the need for removal of the dorsal spinal tumor to relieve the compression on the spinal cord, the likelihood of tumor recurrence and the possible need for more surgeries to remove tumors in the future was also discussed with the patient. Patient agreed and was operated at Fortis Hospitals Kalyan with complete removal of the dorsal cord tumor. He was discharged 2 days after the surgery and his physical healing is in progress, he returned to work within a few days.

“The tumour became absolutely unbearable after a certain period of time. I was under tremendous pressure, pain and often used to worry the final outcome and its effect on my job. The surgery has been successful and has renewed my confidence,” said Mr Ravindra Ghatse.

About Fortis Hospitals Kalyan:-

In keeping with the international model of health systems, Fortis Hospitals Kalyan conceived the concept of hospitals situated at convenient locations across the city, bringing high level care within the reach of the neighbourhood.

The hospital is designed to deliver high standards of healthcare services supported by sophisticated technology and experienced medical professionals. The state-of-the-art, 80 bedded, multispecialty hospital has been established to create a centre of excellence with thrust upon Non-invasive Cardiology, Neurology, Neurosurgery, Orthopaedics, Minimal Access Surgery along with other specialties like Urology, Medical and Surgical Oncology, Endocrinology, Gynecology, Critical care, Emergency and Preventive Healthcare.

Fortis Hospitals Kalyan has an expert and experienced team of specialist consultants, technicians, nursing staff and administrators to deliver patient centric services round-the clock. Fortis Hospitals Kalyan is fully equipped with state-of-the-art 2 operations theaters, have dedicated endoscopy suite, 11 bedded patient centered ICU, 4 bedded emergency / casualty department, 24 hrs diagnostic facility and dedicated 24hrs emergency services.

Fortis Hospitals Kalyan has a corporate support cell to cater the growing demand of an organizational healthcare system for corporate. This cell, apart from helping the hospital empanelment for medical services, also provides information and assists corporate on various corporate health plans of Fortis, thereby enabling access to employees to quality medical care.

Fortis Hospitals Mulund (formerly Wockhardt Hospitals) marks a feat in the field of pediatric cardio-thoracic surgery by performing a complex beating heart coronary artery bypass surgery on an infant. A team of cardiac experts led by Dr. Suresh Joshi, Consultant Pediatric Cardiac Surgeon, Fortis Hospitals Mulund has successfully corrected a complicated and rare congenital deformity called ALCAPA (Anomalous Left Coronary Artery From the Pulmonary Artery) giving the baby a new lease of life.

Baby Sharon was born with normal delivery without any specific symptoms of heart defect during her birth. However after 4 months she suffered from severe cold and cough and was admitted in a city hospital as she was diagnosed of pneumonia. The baby seemed to suffer from increased breathlessness with a very high heart rate. On further investigation the baby was diagnosed of having an enlarged heart and the parents were advised to consult a pediatric cardiac surgeon.

“Baby Sharon was brought to us in a severe condition where she was suffering from severe breathlessness, excessive crying and was extremely feeble due to poor intake of feed.  Upon further investigation we diagnosed her with a congenital heart defect called ALCAPA (Anomalous Left Coronary Artery from the Pulmonary Artery). ALCAPA is a rare birth malformation where the positions of the arteries are switched and the left coronary artery comes out of the pulmonary artery. It occurs in approximately in 1 in 4-5 lakh live births (0.25% – 0.5% of the total birth defects) and if not treated at the appropriate time accounts for 65 % – 85 % of deaths with in 1st yr of life from congestive cardiac failure,” said Dr. Suresh Joshi, Consultant Pediatric Cardiac Surgeon, Fortis Hospitals Mulund.

At the time of birth babies born with such deformities remain active like any normal child but as the child grow the symptoms manifests into breathlessness, inactivity and weak intake of food and they grow very weak and uncontrollable.

“ALCAPA is a serious problem and requires surgery immediately after the birth or as soon as the diagnosis is confirmed. The aim of surgery is to connect the Anomalous left coronary artery with the aorta. The precise surgery depends on the exact location of the Anomalous left coronary artery. Sometimes, it can be moved, along with a button of tissue, from the pulmonary artery and sewn into the aorta. If the Anomalous left coronary artery is located too far away from the aorta to move, a “tunnel” is made from the aorta to the Anomalous left coronary artery or by pass surgery is done by using artery of left arm.”

“Usually to perform such complex surgery heart lung machine is required which is also commonly called On-pump bypass surgery.  However in the case of Baby…. we performed the surgery successfully without using heart lung machine, which means, off pump Coronary artery Bypass Graft (CABG) procedure with the self equipped instruments. Off pump bypass surgery are much common in the adult procedure group, but in neonates such procedure till date is only heard off” added Dr. Suresh Joshi, Consultant Pediatric Cardiac Surgeon, Fortis Hospitals Mulund.

The challenges that involve in this surgery were:

1.      Grafting vessels on a moving heart which needs small compact stabilizer. Stabilizer for small neonate is not devised till date.

2.      Left coronary artery was not appropriate for the graft

3.      Poor functioning heart of the baby, Ejection fraction = 25%

Beating heart surgery was performed on the baby to avoid the post operation complication like fluid retention, kidney failure, lung injuries, and acute inflammatory reaction with mild to moderate heart dysfunction, hence helping the baby for the easy & early recovery. The surgery was done without using any stabilizer as such small size stabilizer for newborn babies is not manufactured by any company in the world. It is with the skill of the surgeon who has managed out the surgery without the stabilizer. Baby Sharon’s story showcases the expertise the country has in correcting the most complex congenital defects in the rarest of condition with a combination of skillful hands and technical finesse.

“The diagnosis of Sharon suffering from congenital heart problem came to us as a surprise. When Sharon was not keeping too well, we had the remotest thought of having anything to do with her heart. In fact before this I had very less awareness on congenital heart problems. In our country many kids succumb to faith due to lack of awareness on the defects and availability of expertise to correct such deformities. We need to create more and more awareness on congenital heart disease in India and the facilities provided by hospitals like Fortis Hospitals Mulund to operate upon the most complex cases. We are immensely thankful to Dr. Suresh Joshi and the entire team at Fortis Hospitals Mulund who came to us as saviors and gave a new life to our baby” says Mr. Bonni D’Souza (father of Sharon).