Congenital heart disease in low- and middle-income countries: can India show the way?

Introduction

We live in a beautiful planet. Unfortunately, it is a planet divided into continents, regions and countries, marked by great inequalities in populations, resources and wealth. Right to health and access to quality healthcare, which is a birthright of every human being in this planet, falls victim to this man-made divide between the ‘haves’ and the ‘have-nots’. The World Bank categorizes countries into high-income, upper-middle-income, lower-middle-income and low-income countries according to the per capita GNI (gross national income) [https://datatopics.worldbank.org/world-development-indicators/the-world-by-income-and-region.html] (Fig. 1). The latter three (comprising 138 countries, per capita GNI less than $14,005) are often clubbed together as low- and middle-income countries (LMICs), indicative of the fact that they face similar social, economic and other challenges. Since every country’s government is responsible for national healthcare with an appropriate annual budget, it is not surprising that healthcare suffers in LMICs due to sheer lack of required finances and resources. Children, being the most vulnerable population group, are the biggest victims of this regrettable maldistribution of wealth.

Fig. 1.

Distribution of countries by income [https://datatopics.worldbank.org/world-development-indicators/the-world-by-income-and-region.html]

Congenital heart disease (CHD) affects about 0.8% of all live births. Consequently, of the estimated 1.35 million children born with CHD annually across the globe, the vast majority are born in the LMICs which have a twin burden of high population and high fertility rates [1] (Fig. 2). Treatment of clinically significant CHD still remains some form of intervention—surgical or transcatheter. At least a third of babies born with CHD require intervention during infancy as a life-saving measure. Since the advent of open-heart surgical techniques for the treatment of CHD in the mid 1950 s, great advances have been made and surgical correction or palliation of almost all forms of CHD, with predictable outcomes, is a reality today. However, these interventions require appropriate hospital infrastructure, manpower, equipment and a ready supply of surgical consumables and drugs. These come at a cost that is generally beyond the reach of most LMICs. Not surprising, therefore, is the fact that across the globe, a stark inequality exists in the availability and access to treatment of CHD. At one end of the spectrum, in high-income countries like the United States of America, Australia and Japan, every child born with CHD receives the best possible treatment without any monetary consideration. At the other extreme, there are the low-income countries like Afghanistan, Myanmar and many in sub-Saharan Africa where access to even simple forms of CHD is unavailable. The vast majority of the countries, however, fall into the LMIC category, where CHD treatment is available in varying levels, but remains far from ideal. Within each of these countries, there is a variable and unequal distribution of wealth within the population which has a bearing on ability to access whatever CHD care is available.

Fig. 2.

Continental distribution of burden of congenital heart disease [1]

Katewa [2] analyzed the availability and funding patterns for 193 countries across the globe and found that only 47 countries provided comprehensive CHD care with funding by government or insurance. However, only 8% of births with CHD occur within these countries. In 40 countries, which include mostly middle-income countries, high-quality pediatric cardiac services are available but are not enough in number to cater to the demand and are difficult to access due to economic or geographic limitations. These also include the most populous countries—India and China. Notably, 41% of children with CHD are born in these countries. The remaining 51% of children with CHD are born in the 106 countries which are either too poor or too small to develop any pediatric cardiac services. Not surprising that an estimated quarter of a million children with CHD die annually due to lack of timely or appropriate treatment.

Much has been written about the status of CHD and CHD surgery in LMICs over the years. Most of these publications focus on the challenges in building facilities and workforce for the delivery of care in these economically constrained regions. Many have suggested ways to improve the situation by recommending policy changes, increased advocacy as well as regional and global partnerships for training and manpower enhancement [3–5]. At the World Congress for Pediatric and Congenital Heart Surgery held in Washington in November 2023, a ‘Call to action to address the Global Burden of Congenital Heart Disease’ was made with an aim to highlight the global inequalities that exist and to find ways to reduce the same [6].

This special issue of the Indian Journal of Thoracic and Cardiovascular Surgery on ‘Congenital Heart Surgery in Low and Middle-Income Countries’ was envisaged with a view to highlight many aspects of this problem that are not discussed in published literature but remain issues that are faced by care givers in these countries on a day-to-day basis. The topics range from economics of CHD care in LMICs to management of late presentation in individual pathologies like tetralogy of Fallot and transposition of great arteries. Experts have been roped in to opine on issues like training of surgeons, the role of humanitarian fly-in and fly-out missions and the contentious area of medical tourism. In addition, the status of CHD surgery in many of the more prominent LMICs are presented by experts from those countries. The status of CHD care in Indonesia, Thailand, Vietnam, Malaysia and Pakistan are presented in detail.

It is not a surprise that such a publication should come out of India. Known for its sharp contrasts in almost every aspect of life, India showcases the best and the worst aspects of CHD surgery. In essence, India represents a unique sample of the status of CHD surgery in LMICs, with high end CHD surgery available in many metropolitan cities and little to none in the vast rural areas. Even though facilities for CHD surgery are growing every year, there still remains a huge gap between demand and supply. The situation therefore throws up many challenges, one of which is that of late presenting CHD. This is a problem unique to LMICs and one in which CHD surgeons from India have considerable experience. Many of the articles in this issue have been authored by Indian authors. Although many aspects of CHD surgery in India have been covered in the various articles in this issue, it would be appropriate to present a brief overview of the history, development, current status and future directions of CHD surgery in India here. In many ways, India can be considered to be a leader amongst the LMICs in the progress it has made in the care for children with CHD.

India demography

India, located in South Asia, is the world’s most populous country with a population in excess of 1.45 billion, which is roughly 18% of the world population. This population is spread over a land area of approximately 2.97 million square kilometers giving a high population density of 488 persons/sq. km. Urban population constitute 36.6% of the total. Population growth has slowed down to 0.89% from a high of about 2.37% in the 1980 s. The current birth rate in India is 16.70 births per 1000 population giving an estimated 24.2 million births annually. The country is divided into 28 states and 9 Union Territories, each having its unique language and cultural identity. India is listed by the World Bank under the category LMIC. Although it ranks as the fifth largest economy in the world (2024 estimated Gross Domestic Product—3.94 trillion US $), the per capita GDP is only US $2730 [https://www.imf.org/external/datamapper/profile/IND.]. Expenditure on healthcare remains low. Spending on health has dropped from 2.4% in 2018–2019 to 1.9% in 2023–2024 and from 0.30% of GDP to 0.28% [http://timesofindia.indiatimes.com/articleshow/111737183.cms?utm_source=contentofinterest&utm_medium=text&utm_campaign=cppst.].

India is known for its ethnic and religious diversity and has the dubious distinction of being known as the land of striking contrasts. Life in the bustling mega metropolises contrasts sharply with the less developed rural population that makes up the majority of the population. This contrast is also evident in the distribution and availability of healthcare, with world class facilities being available in the cities, at a cost, whilst the vast rural population often lacks basic healthcare even. This reality has a major impact on the way care is delivered for CHD across the country.

Burden of CHD in India

Estimates of the annual incidence of CHD vary widely given the paucity of accurate data. Most studies are based on either hospital data or limited population studies which have been extrapolated to the entire population. Going by the internationally accepted incidence of significant CHD to be between 0.8 and 1.0 per 100 live births, it can be estimated that about 190,000–240,000 children are born annually with CHD. About one fifth to one third of these would require some form of intervention to be able to survive infancy. This puts the burden of CHD requiring life-saving intervention in infancy at anywhere between 35,000 and 80,000 cases per year [7]. Patients who remain untreated and survive the natural history in early childhood add to a growing population of adolescent and adult CHD which is estimated at 2–3 million individuals. Given the declining fertility rate in the country, it is anticipated that the annual burden of new CHD will come down. It is also anticipated that increased availability of antenatal diagnosis of complex CHD and subsequent termination of pregnancy will also contribute to this decline.

Critical CHD is more likely to be diagnosed in urban settings where deliveries are more likely to occur in hospital settings and the neonate may be screened for CHD. Attrition of babies with critical CHD is high in the rural settings because of a higher proportion of out of hospital deliveries and sub-optimal perinatal care facilities.

History of CHD surgery in India

CHD surgery in India started as early as the mid twentieth century but witnessed significant progress only in the early twenty-first century. Manohar S.R.K. researched the early years of development of CHD surgery in India [8]. Closure of a patent ductus arteriosus was done by B.R. Billimoria at the Masina Hospital in Bombay in 1949 and this was probably the first CHD surgery to be performed in India. Reeve H. Betts in Christian Medical College, Vellore, performed a Potts shunt for Tetralogy of Fallot (TOF) in 1951 and also the first pulmonary valvotomy using venous inflow occlusion in 1953. The same year P.K. Sen performed the first repair of coarctation of the aorta in Bombay and then in 1956 successfully did the first direct vision closure of an atrial septal defect under hypothermia and inflow occlusion.

Using surface-induced hypothermia and inflow occlusion, A.K. Basu performed an open aortic valvotomy in Calcutta in 1959. The first open heart operation was performed in the form of an atrial septal defect closure using an indigenously made cardiopulmonary bypass machine by K.N. Dastoor at B.Y.L. Nair hospital in Bombay on 16 th Feb 1961. In May 1961, N. Gopinath successfully used a pump-oxygenator to close a ventricular septal defect at the Christian Medical College in Vellore. The first total correction for TOF was done by Radhakrishnan Padhi in 1963 at the Wanless Chest Hospital in Miraj. Other pioneering surgeons in those early years included Dr. Nimish A. Shah from Bombay Hospital and Dr. Stanley John from Christian Medical College, Vellore. Dr. K.M. Cherian from Railway Hospital made notable contributions in the advancement of infant cardiac surgery in the late 70 s and is generally recognized as the ‘Father of modern CHD surgery’ in India. He performed the first Senning operation in 1979 and the first arterial switch in 1984. Other surgeons who actively pursued congenital heart surgery during this period were P. Venugopal and I.M. Rao at the AIIMS, New Delhi; M.R. Girinath at Southern Railway Hospital, Perambur; and D.K. Saksena in Bombay. K.S. Iyer (the author) did the first successful rapid two-stage arterial switch in 1991 at AIIMS, New Delhi, and the first successful double switch operation in 1993 [8].

The field of CHD surgery in India has transformed in the last three decades. In the early nineties, only two or three institutions in the country had the capability to perform infant cardiac surgery. Neonatal cardiac surgery was sporadic and most generally limited to palliative procedures. The situation is much improved now with neonatal and infant surgery being offered in most metro cities and in many larger towns as well. Dedicated pediatric cardiac units have grown in number. Many factors have contributed to this change. In the early nineties, coronary artery surgery witnessed a boom and the private sector got involved in a big way. Stand-alone heart hospitals and institutions mushroomed. In some of these hospitals, CHD programs were piggy-backed on to lucrative adult cardiac programs as a goodwill service or even as a USP (unique selling product). In the late 1990 s the National Board of Examinations started the Fellowship Course in Pediatric Cardiology. With more trained pediatric cardiologists being available, surgeons were in a better position to develop CHD surgery programs. More children were diagnosed in time and referred for surgery. The larger pediatric cardiac units also became training centers and the numbers of domestically trained pediatric cardiac surgeons steadily increased.

Gradually, in a reversal of trends, pediatric cardiac surgeons who went for training overseas started returning to the homeland after training rather than settling there. As the nation’s economy gained pace starting from the early 2000 s, family incomes improved and more families could afford to pay for surgery in private hospitals. The Internet explosion and ready access to information on platforms like Google enabled faster spread of awareness, allowing parents of affected children to explore treatment options. The Pediatric Cardiac Society of India was founded in the late 1990 s and the developing specialty got a much-needed face and identity. Its annual meetings rapidly grew in scale and quality and provided much needed inspiration for prospective new entrants to the specialty. Advocacy led to many state governments setting up schemes for financial support for surgery for children with CHD from the economically weaker sections of society. As infant mortality from preventable causes such as respiratory infections and diarrheal diseases declines, CHD has become a prominent contributor in parts of the country where human developmental indices have significantly improved. ‘Indeed, the distribution of new pediatric heart programs closely mirrors this demographic transition in pediatric disease’ [9].

Funding for congenital heart surgery

Financing surgery for their child is a major hurdle faced by most parents. In the absence of a national policy for funding of CHD surgery, parents have to either fund surgery from their own reserves or seek access to one of the many alternate sources of financial support. Health insurance still has a low penetration in India and unfortunately most insurance companies do not provide cover for CHD. Private hospitals expect full payment for surgeries and generally have a package system where charges for individual procedures are listed along with the number of hospital days covered. Overstay generally entails additional charges and this becomes a bone of contention when hospital stay gets prolonged because of an unexpected complication, or post-operative sepsis, as families come prepared with a limited budget. Some private hospitals subsidize CHD surgery through corporate social responsibility (CSR) schemes. Parents working in government jobs may have access to health benefits from the central or state government which may partly or wholly pay for the surgery. Similarly, many private companies provide family health insurance for their employees which helps defray costs of surgery. In government hospitals, there are no charges for the surgery or hospital stay but most expect families to bear the costs of the consumable items and medications. Totally free surgery is provided by a few philanthropic institutions like the Sathya Sai hospitals in Puttaparthi, Bengaluru and Ahmedabad; the Sanjeevini group of hospitals in Raipur, Palwal and Navi Mumbai; and the Madhusudan Sai Medical Centre in Mudanahalli. A recent study by Faisal et al. [9] analyzed costs of pediatric surgical and interventional procedures from 24 pediatric cardiac centers and sources of funding for these procedures. Out-of-pocket financing accounted for 35% and 25% of the procedures in private and government hospitals respectively. Costs for cardiac surgical procedures ranged from Indian Rupees 65,000 to 450,000 (approx. US$ 800–5600) across all hospital groups. Although this is a fraction of what it costs in many developed countries, it remains out of the reach of most Indian families. In the past few years, the federal government as well as many state governments have stepped in with various schemes aimed at supporting poor families seeking CHD surgery. Some of these are AB-PMJAY (Ayushman Bharat-Pradhan Mantri Jan Arogya Yojana), RBSK (Rashtriya Bal Swasthya Karyakram), JSSK (Janai-Shishu Suraksha Karyakram) and RAN (Rastriya Arogya Nidhi).

The availability of quality and affordable surgery in many of the private hospitals makes it attractive for many overseas patients from the LMICs to seek treatment for CHD in India. The government of India also has an open door policy for any seeking medical help here and facilitates medical visas without delay.

Availability of surgery

In the absence of a national database, accurate numbers of surgeries performed for CHD remain speculative and is estimated to be between 40,000 and 50,000 annually. This number may seem sizeable; however, it is a far cry from the required need. There are at least 200 cardiac surgeons who perform CHD surgery almost exclusively or as a large proportion of their practice. As there is no organized national program for the treatment of congenital heart disease, most CHD surgery is performed in programs that have developed on their own, and are largely based in the non-governmental sector. In the last couple of decades, corporate hospital chains have made major inroads into the delivery of tertiary level healthcare and so it is not surprising that most of the neonatal and complex CHD is treated in these multi-specialty hospitals. Needless to say, treatment here often comes at a hefty cost, which, in the absence of health insurance, has to be borne out-of-pocket. Surgical outcomes in most of these centers compare with international standards, which is sufficient reason for many families to make major financial sacrifices for the well-being of their children. Often hospitals, and even the surgeons themselves, help out patients financially through charities and philanthropic organizations like the ‘Rotary Gift of Life’. Public hospitals on the other hand have lagged behind in the delivery of tertiary healthcare due to budgetary constraints, inappropriate staffing and hierarchical functioning. This situation has hindered the development of large pediatric cardiac programs that could address the needs of the vast majority of patients who cannot afford to go to private hospitals. Rigid recruitment policies and limited job opportunities make it difficult for trained pediatric cardiac surgeons to find a position in government hospitals. In the few hospitals that have, nevertheless, defied the odds and have managed to develop active programs, waiting lists stretch for months to years. Special mention needs to be made of the Sri Sathya Sai Sanjeevani chain of children’s heart hospitals where CHD surgery is being provided free of cost. While they still treat only a small percentage of the national burden, it is hoped that other philanthropic organizations will follow their example and set up more such facilities.

Today quality CHD surgery is available at many specialized pediatric cardiac units across the country. Multiple large units are located in the main metro cities—Northern Capital Region (New Delhi and surroundings), Chennai, Mumbai, Bengaluru and Kolkata—while many of the other major cities have one or two smaller units. The majority of the surgery, especially neonatal and infant surgery, is performed in the private or corporate hospitals and a few public hospitals. The major corporate hospital chains that provide CHD surgery are the Fortis Healthcare group, Narayana Healthcare, Apollo Hospitals, Manipal Hospitals and Aster group. Other non-governmental hospitals providing quality surgery include the Amrita Hospitals in Kochi and Faridabad, the KDA Hospital in Mumbai and U.N.Mehta Institute in Ahmedabad. Notable amongst the public hospitals are AIIMS, New Delhi; Sri Chitra Thirunal Institute of Medical Sciences, Thiruvananthapuram; and PGIMER, Chandigarh.

Challenges for the practicing pediatric cardiac surgeons in this country are many. They need to look after a diverse patient population, ranging from newborns with critical heart disease to adolescents and adults presenting with either late or neglected CHD. Patients often have co-morbidities viral infections, malnutrition and respiratory disorders are commonplace and significantly increase post-operative morbidity and even mortality. ‘Setting up a pediatric cardiac program and sustaining it is usually the surgeon’s initiative and responsibility and running a successful program with limited staff and a constant resource crunch is always a “tightrope walk”’ [10].

Training and accreditation

The basic training for cardiovascular surgeons in India is a 3-year degree course in University based medical colleges with a qualifying examination at the end leading to the award of an M.Ch. (Magistre Chirurgie) degree. Candidates need to have completed a 3-year degree course in General Surgery to be eligible for this course. A parallel course called the Dip. NB (Diplomate of the National Board) exists which is a 6-year combined course after MBBS (Bachelor of Medicine and Surgery), which is applicable to non-medical college-based hospitals. Surgeons who have completed these courses would have had exposure to basic levels of congenital heart surgery and are licenced by the National Medical Council to perform all forms of cardiac surgery independently. Most surgeons desirous of becoming full time CHD surgeons would seek further specialized training in large volume pediatric cardiac units. Most of the senior CHD surgeons in the country have done fellowships in pediatric cardiac units in the USA, the UK or Australia. Currently, there are many high-volume pediatric cardiac units within the country itself which can provide adequate training. There is no longer the need to travel overseas for specialized training in CHD surgery and most trainees would go to overseas units on short-duration observership to pick up some specific advanced skills.

As yet, there are no formal degrees awarded for Pediatric Cardiac Surgery. There is a proposal to start M.Ch. and Dip.NB in Pediatric Cardiac Surgery that is likely to be implemented soon. The Indian Association of Cardiovascular and Thoracic Surgery (IACTS) awards a 2-year fellowship in Pediatric Cardiac Surgery with a selection process and an exit examination. The 2-year fellowship training is provided in pediatric cardiac centres approved by the IACTS.

For pediatric cardiologists, there is a 3-year DM (Doctorate of Medicine) course after MD (Doctor of Medicine) in General Medicine or Pediatrics in select University Hospitals. What is more widely available is a 3-year Dip.NB course in pediatric cardiology that is provided by most of the recognised pediatric cardiac units in the country. There are no specific training avenues for pediatric cardiac anesthesiologists. There are DM and Dip.NB courses in cardiac anesthesia and those interested in pediatric cardiac anethesia get attached to a pediatric cardiac service and get trained in an apprenticeship model. Likewise, pediatric cardiac intensive care is only recently gaining importance as an independent entity. There are no recognised degrees as yet, but recently the Pediatric Cardiac Society of India has started a 2-year fellowship course in pediatric cardiac intensive care.

India has also been a training ground for pediatric cardiac specialists from neighbouring countries like Bangladesh, Sri Lanka and Nepal and from many parts of Africa.

Innovations and research

There has been a steady increase in research output and publications in the field of CHD over the years. Stanley John published the results of surgery for Tetralogy of Fallot in an older population in Circulation in 1974 [11]. Since then, publications have grown in number and have revolved around management of rheumatic valve disease, late presenting CHD and innovations in surgery and interventional cardiology. Gupta and Ameen Ahmed published an analysis of the research output in CHD from India based on publications data (1335) indexed in Scopus database during 2000–2019. Their study highlights the contributions made by Indian researchers. Their findings are as follows: ‘CHD research registered 10.0% growth, averaged 7.07 citations per paper, contributed 3.04% share to the global output and constituted 13.48% share of India’s research as international collaborative. CHD research is skewed as top 10 countries account for 83.23% of global output. India ranks as the 10 th most productive country in the world. The distribution of CHD research by type of research reveal that Tetralogy of Fallout accounted for the highest share (34.31%), followed by Aortic Stenosis (30.41%), Atrial Septal Defects (28.16%), Ventricular Septal Defects (26.22%), etc.’. ‘B. Airan, A. Saxena and S. Talwar have been the most productive authors (with 63, 63 and 59 papers) and P. Khairy (16.31 and 2.31), B. Vaidyanathan (14.3 and 2.03) and R.K. Kumar (10.53 and 1.49) have been the most impactful authors. Indian Heart Journal, Annals of Pediatric Cardiology (84 papers) and Annals of Cardiac Anaesthesia (53 papers) topped the list of most productive journals (with 110, 84 and 53 papers)’ [12]. More recently, Kadiyani et al. [13] published a review of surgical outcomes in CHD analysing all available publications on the subject. Two more publications [14, 15] have also highlighted all the innovations that Indian surgeons and cardiologists have made in the field of pediatric cardiac surgery and cardiology.

The Annals of Pediatric Cardiology (https://journals.lww.com/AOPC/pages/default.aspx) is the official publication of the Pediatric Cardiac Society of India and showcases most of the research work done in India and this part of the world.

Future directions

The mission of the present government is to make India a developed country and the world’s third largest economy by 2047—the hundredth year of Independence. Part of this mission involves a major overhaul and upgradation of the healthcare availability and delivery. Universal healthcare is a stated goal and the widespread adoption of the Prime Minister’s Ayushman Bharat scheme is a major step towards that goal. A thrust area is enhancement of the healthcare manpower needs with increase in the number of medical colleges and a substantial increase in the number of seats for specialty and super-specialty training. There is increased spread of private health insurance and all-out efforts are being made to bring CHD under its gambit. Economic prosperity also means that more families will be able to afford private healthcare. All these measures will boost the number of facilities for CHD surgery and improve their accessibility and affordability. There is also a major thrust towards local manufacture of medical equipment and consumables to reduce the dependence on expensive imports. Introduction of newer imaging technologies like 3D (3-dimensional) rendering with virtual reality and augmented reality and use of AI (Artificial Intelligence) at various levels of patient care will change the landscape of pediatric cardiac care. It is expected that India will continue to be a major source of innovations and leadership in care for CHD in the LMICs.

Author contribution

Krishna S. Iyer: conceptualization, literature review, writing.

Sivakumar Sivalingam: conceptualization, literature review, editing.

Funding

None.

Data availability

Not relevant.

Declarations

Ethics committee clearance

Not applicable

Conflict of interest

K.S.Iyer is past Editor-in-Chief and current Ombudsman of the Indian Journal of Thoracic and Cardio-vascular Surgery.

Informed consent

Not applicable.

Declaration on animal and human research

Not applicable.

Use of AI tools

Nil.