Closing in on holes in the heart: finally

Chronic chest infections plagued six-year-old Mihir Sejwal's tiny frame soon after he was born. On the face of it, it would appear that Sejwal may have suffered from low immunity. However, Sejwal had tiny holes in his heart and his condition is known as ventricular septal defect (VSD) "" a heart defect present at birth that can worsen over a period of time, if left untreated. Despite repeated warnings from doctors, Sejwal's chest infections prevented him from undergoing the operation to close the holes in his heart. By the age of four the holes had expanded and his lungs were succumbing to pressure and gasping for blood. As his condition deteriorated, doctors gave up on him and eventually Sejwal was labelled inoperable. Yet, today, two years later, the six-year-old has survived his condition. The scar on his chest tells the story of a heart no longer punctured with holes, owing to what might become a welcome procedure for patients with advanced VSD.

The paediatric cardiac team at the Max Heart and Vascular Institute, Delhi, successfully tested a combination of banding and oral medication on Sejwal, to treat what they have termed Early Eisenmenger Syndrome (EES), an advanced form of VSD, where the patient's lung pressure increases and blood level decreases. Banding, though often used in cases of VSD to reduce excessive blood flow into the pulmonary artery, was now used by the doctors in combination with oral medication. In Sejwal's case, the procedure brought the lung pressure down by 50 per cent, thus allowing the doctors to operate on him. "However," warns Dr Viresh Mahajan, the paediatric cardiologist at Max who pioneered the procedure, "it is pertinent to note the stage at which this procedure was performed on Mihir. Such a procedure may not be applicable to all patients suffering from the Eisenmenger Syndrome, which is why we have termed it Early Eisenmenger."

Normally, a VSD patient passes through three stages to reach Eisenmenger Syndrome. The first is of the discovery of the hole, which, if left open, leads to the leakage of excessive blood into the lungs. Depending on the size of the hole, if the closure is delayed by a few years, the pressure and volume of the blood in the lungs continues to rise. There then comes a point at which the pulmonary artery's muscles start thickening, causing irreversible damage to the lungs. As the muscles grow, while the pressure remains high in the lungs, the volume of blood begins to decrease. And so, it is at this stage that the patient becomes asymptomatic.

In fact, this state of being asymptomatic often deceives parents into believing that their child's heart is functioning normally. However, that isn't so. Even as parents rejoice in the asymptomatic stage, the heightened pressure in the pulmonary artery causes the direction of the blood flow in the heart to reverse, thus bypassing the lungs altogether. This condition results in discolouration or blueness of the skin and nails, indicative of the stage that the Max doctors call Early Eisenmenger. Though these doctors claim that their treatment will now allow Early Eisenmenger patients to successfully undergo an operation to close the holes in their heart, they admit that this procedure cannot be applied to patients who fall under the traditional definition of the Eisenmenger Syndrome. Such patients will typically show symptoms of blue discolouration of tongue, lips and fingertips, thickening or clubbing of fingers, and thickening of the blood and decreasing ability to exercise.

Eisenmenger Syndrome, though a common outcome in India of heart defects like VSD, is a rare find in Western countries, where a newborn with a heart defect is immediately operated upon. In fact, increasingly, such operations are now being carried out on the foetus itself. However, within the medical fraternity in India opinion is sharply divided as to which stage a baby with VSD should be operated upon. According to Dr Krishna S Iyer, director, paediatric and congenital heart surgery at Escorts Heart Institute, "There are well-defined medical criteria for operating on a VSD patient. If the hole in the heart is large, it should be closed within the first six months of the baby's birth." He adds that all forms of at-birth or congenital heart defects must be treated as soon as possible. However, AIIMS' Dr Anita Saxena, professor, cardiology is of the opinion that increasing awareness of VSD in the urban areas has created mass hysteria where doctors are simply rushing every newborn into the operation theatre. "Evry case of VSD or any other heart defect does not require immediate surgery," she explains. "Sometimes the patient's body cannot cope with a surgery and the doctor actually ends up putting the baby's life in danger. Closing the hole may even endanger the patient."