- Heart tumors in newborn babies are very uncommon. Those associated with a genetic condition called Tuberous Sclerosis (TS) are called Rhabdomyoma. Multiple giant tumors (rhabdomyomas) occupying the heart cavities and causing life-threatening arrhythmias have an occurrence of 1 in million newborn babies.
- Multiple such tumors with one measuring 3cmx3cm detected in the 6-day old baby’s heart were giving rise to the serious fast heart rate of 230bpm
He was treated with a new drug to treat heart failure by decrease in size of the tumors and normalizing heart rate.
Mumbai: Bai Jerbai Wadia Hospital for Children, Parel gave a second lease of life to a 29-days-old baby boy with a rare genetic disorder Tuberous Sclerosis associated with multiple (rhabdomyomas) tumors in his heart leading to a faster heart rate of 230 bpm. The baby was successfully treated with a life-saving drug to shrink the tumors. Now, the baby is arrhythmia free, weighs 2.8 kgs and is accepting feeds like other children.
Karjat-based Kandvi family was happy after Kavita delivered a baby boy. But, the couple’s happiness was short-lived when the baby did not cry after birth. Though successfully resuscitated, the local doctors told the parents about the baby’s abnormally fast heart rate. He was referred to Badlapur where two anti-tachycardia medicines called antiarrhythmics were started. However, the baby didn’t get that much-needed relief and was rushed to Wadia hospital for further treatment.
Dr Jayashree Mishra, Senior Consultant Paediatric Cardiologist of Bai Jerbai Wadia Hospital for Children said, “The baby arrived at the hospital in a critical condition with a failing heart. On arrival, the ECG revealed a fast heart rate of 230 bpm with an abnormal rhythm. With uncontrolled heart rate, the child was going into heart failure. The scan of the baby’s heart showed multiple large and small tumors with characteristics suggestive of Rhabdomyoma. Primary Heart tumors in newborn babies are very uncommon with an incidence of 0.0017 -0.02%. Though Rhabdomyomas are the commonest primary cardiac tumors, they are overall very rare. Multiple giant and diffuse rhabdomyomas occupying the heart cavities and causing life-threatening arrhythmias have an occurrence of 1 in million newborn babies. These tumors were numerous and varied in size with one of them as large as 3cmx3cm attached to the lower pumping chambers of the heart. Rhabdomyoma especially when multiple and diffuse is known to be intimately associated with a genetic condition called Tuberous Sclerosis (TS). 50% of patients with Tuberous sclerosis develop Rhabdomyoma. TS causes benign tumors in multiple sites like the heart, skin, eyes kidneys, and brain. “On further investigation, we found that this is a familial abnormality and the child’s father and grandmother had the same genetic defect as shown by their clinical stigmata and prior clinical reports. Father and grandmother have both undergone treatment for the same condition, however, in their cases, the heart was spared. Knowledge of this genetic condition is important as 50% of children born to affected parents can inherit this condition.”
She further added, “Rhabdomyomas can cause arrhythmias, blood flow obstruction due to mass effects, and heart failure. In this baby, these tumors were giving rise to this serious fast heart rate. The tachycardia called SVT was due to an abnormal circuit formed in the heart by the tumor tissue and so it was not responding to any anti-tachycardia drugs. Management of arrhythmias includes medical treatment with antiarrhythmics, catheter ablation, placement of an implantable cardioverter defibrillator (ICD), surgical excision, and even heart transplantation. Surgery was impossible as the tumors were very widespread and is difficult to remove in a newborn baby. Catheter ablation and ICD were challenging given the small size of the baby.”
As there were not many options left to save the child’s life, it was decided to treat her with a novel drug Everolimus. “This anticancer drug worked miraculously for this critically ill newborn and over the next few days the tumor size started reducing and the heart rate came to a safe range. The heart failure came under control and the child became stable clinically. As this drug needs thorough monitoring of Blood sugar, serum lipids, and blood counts, the child was kept under observation in the Neonatal intensive care unit. This anticancer drug is known for suppressing the body’s immunity. Hence, the child was given prophylactic antibiotics and kept under strict asepsis to prevent secondary infections. At the end of three weeks, all the small tumors have regressed and the largest one decreased to 1cm size. Heart function assessed by echocardiogram is also within the normal range. “There has been no recurrence of fast heart rate in the last three weeks. This drug treatment is planned for a total duration of four weeks to allow sustained benefit. There is no side effect of this treatment and at present, the baby is fully stable with normal vitals and feeding orally. He will be discharged upon finishing four weeks of treatment,” said Dr Mishra.
In spite of many advances in the medical field, the treatment of rhabdomyoma remains challenging. Treating such a critically ill newborn requires a multidisciplinary approach with advanced infrastructure and laboratory facilities. This baby was successfully treated with the novel drug for free of cost to improve his quality of life,” underscored Dr Minnie Bodhanwala, CEO Of Wadia Hospitals.
“We were appalled and worried as our baby didn’t cry after birth. We were alarmed after noticing a rapid heartbeat and he didn’t accept feeds. Our happiness turned into a nightmare when our baby was diagnosed with multiple tumors in the heart. We have heard about tumors in the brain or lungs but not the heart. This is an unknown entity to us. We are fortunate and thankful to receive timely intervention at Wadia hospital free of cost as we were unable to afford the treatment due to financial crisis. I thank the hospital for saving my baby’s life,” concluded Kavita Kandvi.
