Bravehearts of the Foundation: Sakeena
June 8, 2018 | Contributed by R Srivatsan
After a boy, who turned out to be a handful, Sakeena was delighted to welcome into the world, and introduce her son Murshid to his baby sister. However two days after she was born, Sakeena’s baby, a six month old girl from Mannarkad, Palakkad in Kerala had a cyanosis detected, followed by an apnea on the fourth day. Following this, she was put on a ventilator at the Thrissur Medical College. Here an echo was carried out at which showed pulmonary atresia with intact ventricular septum, and so she was started on PGE1 infusion and shifted to Amrita Institute for further management.
The plan was too stent the PDA after she gained weight, and she underwent the procedure in the last week of November 2017 successfully, and discharged by mid-December, and advised continuous follow-up.
In mid-January, doctors advised that the baby be taken in for the second stage of surgery known as ICR. Nur Muhammed, the baby’s father is a daily wage labourer, originally from Assam, who fell in love with Sakeena, and thus settled in Kerala. He had no idea how he would once again raise the funds required for his little girl’s surgery. However, our contribution ensured that this little baby got the treatment that she requires. That she will go home safe in her mother’s arms, and grow up alongside her brother, and always be her father’s favourite daughter
Following are the details of her condition and surgery:
Pulmonary atresia with intact ventricular septum ( PA-IVS) is a rare congenital cardiac lesion seen in 4 /100000 live births. It accounts for less than 1% of all congenital cardiac defects seen in children. It is characterized by complete obstruction to the outflow from the right ventricle of the heart to the lungs. It is often associated with a small right ventricle chamber. Blood is thus unable to flow from the right ventricle to the pulmonary artery and lungs, and a reliable source of pulmonary blood flow is required for survival. If untreated, this condition is almost uniformly fatal. However, with timely treatment long term survival and a productive life is feasible in a substantial proportion of affected babies.
The exact reason why this condition happens is unknown. When compared with other cardiac lesions, identifiable genetic syndromes and other cardiac malformations are less common in PA/IVS and therefore the long term outlook from a developmental standpoint is generally good. PA/IVS consists of a wide variety of anatomical cardiac defects that are associated with the primary lesion of pulmonary atresia
New-borns with PA/IVS present with low oxygen saturation levels because not enough blood gets to the lungs. Much of the oxygen-poor blood returning from various organs that is destined to go to the lungs is shunted inside the heart. This circulates back into the body and results in low oxygen levels. This oxygen poor blood is blue in colour and imparts a bluish hue to the baby (cyanosis or blue baby).
If untreated, PA/IVS is an almost uniformly fatal disease; approximately 50 percent of children will die within two weeks of birth and 85 percent by six months. Neonates with PA/IVS should be cared for at a tertiary medical centre with experience in managing complex congenital heart disease (CHD).
Initial management is focused on stabilization of cardiac and pulmonary function and ensuring adequate oxygen supply to the body. This requires care in an intensive care unit and administration of a medication known as prostaglandin that keeps foetal channels (known as the arterial duct) open. However, this medication cannot be administered indefinitely. It is possible to keep these channels open for several months by procedures performed in the cardiac catheterisation laboratory (known as stenting of the PDA). In the new-born period these children often need a procedure namely PDA stenting or a temporary surgical shunt (the Blalock Taussig Shunt or BT shunt) to maintain adequate systemic oxygenation.
Eventually, however, definitive surgery needs to be performed to provide long term solution. This is accomplished through open heart surgery where efforts are made to restore normal heart circulation to the best extent possible.
The baby was admitted at Amrita Institute on 8th October, 2017 and managed in the ICU initially on medications that included prostaglandin. This was done to allow some weight gain and make the subsequent catheter procedure relatively safe. She was taken up for a catheter procedure after two weeks. In the cath lab the pulmonary valve that replaced by a thick membrane was perforated and a temporary pathway was created to allow blood to flow into the lungs by placing a stent in the outflow track of the right ventricle. She recovered well. A relatively long stay in the hospital that was required to ensure that she received feeds adequately and her mother was comfortable in taking care of her.
The baby was discharged in December 2017 and advised close follow up. She was doing well but, as expected, the blueness gradually increased as the Baby of Sakeena gained weight. This was because, as the child grew and put on some weight, the stent in the right ventricular outflow tract did not allow for an increase in the blood flow to the lungs as she grew. She was being followed at our outreach clinic in Palaghat. About three months after discharge, she was advised the next stage surgery. This required opening up of the right ventricular outflow track and removal of the stent. The goal was to restore the heart functioning to as close to normalcy as possible.
A detailed evaluation was done on admission. The little girl was taken to theatre and under cardiopulmonary B bypass and arrested heart, right ventricular outflow tract was opened and stent was removed. Aggressive right ventricular overhauling was done resecting the excessive muscle in the right ventricle and removing the fibrotic lining of the right ventricular chamber. The right ventricular outflow tract was reconstructed with transannular patch. ASD size was reduced and came off bypass. She made an excellent recovery and at discharge has a resting saturation of 90%.
This report illustrates a number of major challenges this child faced from the day she was born. The heart condition was complex requiring a multi staged approach. The baby was very small and there were serious economic and logistic challenges.