THE NINE HEADED HYDRA OF CHD MANAGEMENT IN INDIA

Managing Congenital Heart Disease (CHD) in India is fraught with challenges of unimaginable dimensions. Comparisons to the nine headed Hydra of Lernaia underlines the Herculean nature of the problem

Thefirst challenge is the lack of awareness among the medical community about early recognition, appropriate management and contemporary outcomes in CHD. With Infant Mortality Rate (IMR) at historical low levels and the traditional big 3 infant killers of malnutrition, respiratory infections and diarrhoea disorders slowly becoming a thing of the past, the time is ripe to focus on the so-called "non-traditional" causes of IMR. CHD are the commonest cause of death due to congenital malformations in the developed world. The combination of a disease with a 1% incidence throughout the world irrespective of geography and ethnicity and a population of 1.3 billion with a fertility rate among the highest in the world should suggest a major health problem even to the casual observer. However the health care system continues to remain obsessed with respiratory infections and bacterial sepsis ignoring (at their own peril) alternative explanations for a sick infant. The lack of pediatric cardiologists outside the big cities further accentuates the problem.

When a CHD is diagnosed, the next head - social pressure – crops up. Families from rural India (and even those from an educated and privileged background) share an irrational belief that babies lack the physical strength to survive through the rigours of open heart surgery. A grandmother once enthralled me with a vivid description of how medications and good food could ensure that her precious grandchild with a large VSD could grow plump following which she would bring the child back to me for surgery. This, in a 3 month old who weighed 3.5 kg at birth and 3.2 kg when I saw him, brought out hitherto unknown levels of self-control to prevent me from bursting out laughing. Every family would have their favourite story of a grand uncle who died due to surgery for CHD in 1976 when congenital heart surgery did not exist in India or of a third cousin who was very sick in infancy diagnosed with a large hole in the heart but grew up to adulthood and got married (probably with Eisenmenger syndrome).

The third challenge is the economical constraint. A corrective surgery for CHD in India costs between 1 – 4 lakhs ($1500 - $5000). To provide a perspective, similar surgeries in the West is likely to cost upwards of $ 20,000. However, even this amount is out of the reach of most families in India. In addition, critical CHD need immediate or early surgery robbing the families of precious time needed to arrange resources. This is without considering the loss of livelihood due to hospitalisation of a family already fighting to arrange money for treatment. Although the Government insurance schemes and NGOs focussed on CHD are attempting to bridge this divide, we still have a long way to go.

India has a number of centres of excellence for congenital heart diseases which provide care comparable with the Western world. However, in a country with such a vast and challenging geography, these centres remain too far away from the vast majority of the population who require their service. When a child with CHD is recognised early and appropriate emergency management is instituted and you arrange the money needed for the surgery, the next head to tackle is the transport of the sick child to a centre where definitive management can be offered. Transport by air is too expensive and also not widely available outside the metropolitan cities. Although ambulance services in our country continue to improve, most of them do not have a doctor managing the transport. The paramedics who work in ambulance services are almost exclusively trained to transport sick adults and lack the training or skill to transport sick neonates and infants. Almost all ambulances in the smaller towns and villages lack a transport ventilator to shift ventilated babies. In addition, the roads connecting these lands to nearby cities are often in a terrible shape and over-crowded. This artificially bloats the time taken to travel between points A and B.

When you conquer these 4 heads, further problems arise including availability of specialised intensive care and multi-disciplinary expertise for post-operative complications, follow up and appropriate management of residual hemodynamic problems and routine post-operative care including nutritional support, development assessment and immunization.

In such a scenario, I am happy to share a success story where a few of these heads have already been destroyed in an attempt to save a precious life. Baby A was born to non-consanguineous parents 4 years after their marriage. The perinatal period was uneventful and the baby remained well till the first immunization. A few days later, the mother noticed some feeding difficulty which she managed with native medications. When his feeding did not improve even after 3 weeks, she took him to a pediatrician who after examining him suspected a CHD. An adult cardiologist examined the child and after an echocardiogram diagnosed dilated cardiomyopathy (DCM) with very poor ventricular function. The child was started on anti-failure medications and a very bleak picture was painted to the family. They were told that the baby is likely to die in the next few weeks. The desolate family were given a ray of hope by a junior doctor in the hospital who suggested that management by a pediatric cardiologist may be of additional benefit and referred them to me. When I saw the child, the baby was breathless with tachycardia, cardiomegaly and a very large liver. A quick look at the apical 4 chamber confirmed the presence of severe LV dysfunction. Whenever I see a baby with DCM, I make a silent prayer to GOD as I move to the para-sternal short axis requesting that he bless me with an image of reverse flow in the left coronary system into the pulmonary artery. Today, my prayers were answered and I breathed a sigh of relief as I realised the baby had anomalous origin of left coronary artery from pulmonary artery (ALCAPA) – one of the few completely reversible causes of DCM. Recognising the challenges in arranging finances and transporting the child, I first decided to control heart failure. With non-invasive ventilation (NIV) and decongestive therapy, I was able to get on top of his heart failure symptoms in the next 48 hours. During this time, I organised COVID testing for the baby and the parents (a pre-requisite for surgery in these trying pandemic times). The stars were aligned well enough to bless us with negative reports. I next turned my attention to the most fearsome of the nine heads – arranging 2.5 lakhs ($ 3000) for surgery in a short span of time. Four of my close friends in school came forward as soon as I shared the requirement in my school friends Whats App group and agreed to jointly provide the money needed for the surgery. I did not have the facilities to transport a child on NIV to the nearest surgical unit 265 km away. The best strategy then was to wean off NIV and shift the child quickly to surgery. With torrential non-seasonal rain posing an additional challenge, I contemplated on the best strategy to achieve this. I was at this time blessed with the visit of some of the father’s friends enquiring about the well-being of the baby. Such visits are usually a nuisance and I do my best to keep them short. But on this occasion I was a bit patient with these friends who were local office bearers of the Tamil Nadu All Drivers Association. After hearing my dilemma, they immediately spoke with their friends working in the Police Department of all the adjoining districts. A 265 km “green corridor” was organised spanning 5 districts so the baby can be shifted without traffic snarls. Google Maps tells me that it takes 5 hours and 37 minutes to travel from my hospital to the referring hospital. This was likely to further increase considering the torrential rain. On the morning of January 11, my ambulance driver made this journey in just 3 hours and delivered the baby safely to the surgical unit. As a bonus, this received a large social media coverage with accolades pouring in for the driver who succeeded in a rather treacherous attempt.

The surgeon then performed the surgery the very afternoon. A successful repair was accomplished but the surgeon left the chest open because of the severe LV dysfunction and the relatively long bypass time. With such severe LV dysfunction, I would anticipate chest closure in 48 hours, extubation in 96-120 hours and a minimum ICU stay of a week. But when so many people had sacrificed their time, money and effort for a baby whom they did not even know and had not even cast their eyes on, it should amount to something. The surgeon was able to close the chest the very next morning (a mere 12 hours after surgery), extubate him the next evening and shift him to the ward 24 hours later where he is getting ready to make the much longer journey home tomorrow.

As I breathe in a relaxed fashion after a rather hectic week in which Baby A has dominated my thought every second I have been awake (and probably when I have been asleep as well), I wish this story would help raise awareness about CHD among both the medical community and the public and help save many more Baby A who are in dire need of life saving treatment. My heartfelt thanks to The TN All Drivers Association, the Tamil Nadu Police, the public who participated enthusiastically in the cause, my friends Arjun, Nancy, Kavin and Sakthivel who generously contributed to the surgery and to Dr Vijay Kumar, congenital heart surgeon, without whose skill and expertise this gargantuan effort would have come to nought.

The Hydra has been conquered (for now). But Hercules cannot afford to relax