The grown-up congenital heart disease dilemma in India: A call for urgent reform in adult congenital cardiac care

Abstract

Grown-up with congenital heart disease (GUCH) represents an emerging but under-recognized public health challenge in India. With an estimated 3.5 million adults living with congenital heart disease (CHD), the country has one of the world’s largest GUCH populations. Despite improving pediatric cardiac surgery outcomes, adult CHD care remains fragmented, with critical gaps in infrastructure, training, transition protocols, and national policy. This editorial explores the epidemiological burden, systemic challenges, and recent progress, including the growth of pediatric cardiac programs, the emergence of GUCH clinics, and increased use of transcatheter interventions for the Indian GUCH patients. We need a structured, level-based care model, development of specialized centers, dedicated GUCH training, and the creation of national registries and India-specific guidelines. Bridging the divide between pediatric and adult cardiology is essential to ensure lifelong, coordinated care for this vulnerable population.

INTRODUCTION

Congenital heart disease (CHD) has long transitioned from a condition predominantly managed in childhood to a chronic, lifelong cardiovascular disorder. Over 90% of children born with CHD in developed countries are now expected to survive into adulthood, largely due to advances in pediatric cardiology, surgery, and neonatal intensive care.[1] This growing population of adults living with CHD, often termed grown-up with congenital heart disease (GUCH) or adult CHD (ACHD), requires structured, multidisciplinary, and lifelong care.

India, with its massive birth cohort and improving pediatric surgical outcomes, harbors one of the largest GUCH populations globally. Yet, this group remains largely invisible in public health planning and healthcare policy. This editorial explores the epidemiological burden, systemic challenges, current efforts, and proposes a model GUCH system adapted to India’s context, reinforced with global guidelines and recent data.

THE EPIDEMIOLOGICAL BURDEN

India sees approximately 25 million births annually. With an incidence of 8–12 per 1000 live births,[2,3] this translates to over 225,000 new CHD cases each year.[4] Approximately 50,000–60,000 of these present with moderate-to-severe CHD needing timely intervention. The prevalence of GUCH varies markedly; estimates suggest around 3000–3500 GUCH patients per million adults.[5,6] Given India’s 1.4 billion population with nearly 1 billion adult population, over 3.5 million adults are likely living with CHD, with ~1.5 million with moderate-to-complex lesions and ~300,000 with severe forms, including Eisenmenger syndrome, Fontan, or univentricular physiology. This estimate likely underrepresents the true burden due to a lack of registries or surveillance programs, high rates of missed diagnoses, and regional disparities in surgical access and outcomes. India may also host the world’s largest Eisenmenger cohort due to late diagnoses. Thus, GUCH is not a rare or orphan condition by numbers in India; it is a neglected and forgotten population that has fallen between the domains of pediatric and adult cardiology.

WHY THE GAP? A MULTIFACETED CHALLENGE

Despite a sizable and growing population of adults with CHD in India, GUCH care remains poorly organized, inconsistently delivered, and largely neglected. This gap is the result of intersecting challenges spanning infrastructure, manpower, training, financing, and sociopolitical will. Some of the challenges are summarized in Table 1.

Table 1.

Grown-up with congenital heart - care in India: An overview

Fragmented responsibility and lack of ownership

A fundamental barrier is the lack of clarity regarding who should manage GUCH patients. These patients fall in the gray zone between pediatric cardiologists (who are trained in congenital anatomy but not adult comorbidities) and adult cardiologists (who are skilled in managing hypertension, coronary disease, and arrhythmias but often unfamiliar with congenital physiology) as the primary caregivers. This has created a “no man’s land” of care, with many patients experiencing discontinuity during the transition from pediatric to adult services. In developed countries, structured transition-of-care programs have been designed to ensure continuity and optimize outcomes; in contrast, India lacks a national or institutional transition model in most centers.[7,8] Without dedicated GUCH specialists or clinics, patients are often referred from one center to another, with no designated follow-up path or documentation.

Inadequate training and lack of defined subspecialty

The current medical education system in India does not recognize ACHD as a formal subspecialty. The postgraduate cardiology curriculum focuses predominantly on ischemic heart disease, heart failure, arrhythmias, and structural interventions. Pediatric cardiology training, on the other hand, is not designed to address adult physiology, pharmacotherapy, or comorbidities such as diabetes, obesity, or atrial fibrillation. There are no structured fellowships for GUCH care in India, and international programs are inaccessible to most Indian trainees due to costs and regulatory issues. The 2020 European Society of Cardiology (ESC) guidelines for GUCH care[3] emphasize the need for specialized training pathways and recommend that moderate and complex ACHD cases be managed in dedicated centers by accredited specialists. India lacks such pathways or accreditation systems.

Sparse infrastructure and specialized centers

GUCH care is inherently multidisciplinary, requiring access to congenital heart surgeons, adult interventionalists, electrophysiologists, heart failure experts, imaging specialists (especially cardiac magnetic resonance imaging-[MRI]), and psychologists or social workers.[9] Very few Indian centers are equipped to offer this level of integrated care. Multidisciplinary GUCH teams, a standard in Europe and North America, are virtually nonexistent in India, except at a few apex institutions.

Economic and logistical barriers

CHD care is resource-intensive. A recent systematic review highlighted the barriers to accessing surgical treatment of CHD in low- and middle-income countries (LMICs) – including delayed diagnosis, lack of treatment centers, financial constraints, and gender-based discrimination.[10] Many patients from rural and low-income backgrounds cannot afford long-term follow-up, imaging, medications, or reintervention. While India may be a growing hub for affordable health care for international patients, the enormous number of unoperated CHD cases in India showcases the disparities in healthcare access.[11]

Absence of national guidelines and registry data

There are no India-specific guidelines for the management of GUCH patients. Western guidelines, while helpful, are not fully applicable in settings with a higher burden of unoperated or late-repaired disease. India lacks a national ACHD registry or surveillance program to track outcomes, complications, and healthcare utilization. Without this, planning services or lobbying for policy support is difficult. Similarly, the lack of multicenter research or clinical trials on GUCH care in India further perpetuates its invisibility in academic and policy circles.

Limited industry interest and innovation

GUCH care does not align with high-volume, high-profit procedural cardiology. As a result, the device and pharmaceutical industries have invested little in innovations or support programs for GUCH. Contrast this with structural heart disease, where percutaneous valve technologies and left atrial appendage occlusion have received rapid commercial traction.

CURRENT EFFORTS AND POSITIVE SHIFTS

Despite long-standing neglect, the landscape of GUCH care in India is beginning to change, which is encouraging. Several developments in pediatric cardiac services, surgical capabilities, and institutional models are laying the groundwork for a more structured approach to GUCH care in India. While these efforts remain localized and insufficient to meet national needs, they serve as proof of concept for what is possible with focused planning and investment.

Expansion of pediatric cardiac surgery programs

One of the most significant drivers for the change in the GUCH population in India has been the steady growth of pediatric cardiac surgical services over the past two decades.[12] Previously limited to a handful of apex institutions, pediatric cardiac surgery is now available in several government and private hospitals across the country. Several high-volume pediatric cardiac programs are successfully changing the operative outcomes of neonatal/infantile heart disease across India, thus potentially changing the landscape of the GUCH population in the country.[12] The problems encountered among GUCH patients in some of the Southern and Western parts of India may be similar to those in many Western countries. However, the most populous northern region still lags, and hence almost 50% of the GUCH population remains unoperated adults with CHD.

Emergence of grown-up with congenital heart disease clinics

Although still uncommon, dedicated GUCH clinics have been initiated in select tertiary institutions. These clinics aim to provide longitudinal care for adolescents and adults with CHD by integrating pediatric and adult cardiology services. Such dedicated GUCH clinics are being run at selected centers across the country. However, the number of such clinics is woefully inadequate for a country as big as India.

Growing role of transcatheter interventions

The rise of transcatheter interventions for structural heart disease has transformed the management of many GUCH conditions, reducing the need for repeat surgery and offering less morbid alternatives for reintervention.[13] Key interventional procedures increasingly used in Indian GUCH practice include transcatheter pulmonary valve implantation for posttetralogy of Fallot (TOF) or right ventricular outflow tract (RVOT) conduit dysfunction, and percutaneous closure of residual ventricular septal defects or paravalvular leaks.[14] The lower-cost innovation from India, MyVal (Meril Lifesciences, Vapi, India), is being increasingly used in the pulmonary position,[15] especially in dilated native RVOT.[16] However, when a vast majority of patients are paying out-of-pocket for cardiac procedures,[17] even the lower cost of MyVal is out of question for many deserving GUCH patients.[18] We need further innovations, cost reduction, and new funding models for these expensive procedures in India.[17]

Greater awareness of adult presentations of congenital heart disease

Adult cardiologists are increasingly recognizing presentations of undiagnosed or late-diagnosed CHD in adults,[19] such as atrial septal defect with paradoxical embolism, Ebstein anomaly with arrhythmias, congenitally corrected transposition of great arteries (CCTGA), coarctation of the aorta presenting as refractory hypertension, and Eisenmenger syndrome in unoperated lesions. Widespread use of imaging for structural heart disease is also increasing the pickup rates. This awareness is driving more referrals to congenital specialists and prompting collaborative care models in large hospitals.

Growing interest in research and data generation

While still in early stages, Indian centers are beginning to publish institutional experiences in GUCH management. These include case series, observational studies on TOF follow-up, and outcomes of device interventions in ACHD. A retrospective study from The Madras Medical Mission, Chennai, evaluated 153 adults undergoing congenital heart surgery and demonstrated excellent outcomes even for complex lesions, with low surgical mortality and significant improvement in functional class at follow-up. Notably, reoperations were rare, and late primary interventions were common – highlighting both the delayed presentation and the feasibility of ACHD surgery in the Indian context.[20] A recent systematic review and meta-analysis of 135 Indian studies involving over 30,000 patients undergoing CHD surgery reported a pooled in-hospital mortality rate of 5.63%, significantly higher than the 2.65% observed in Western datasets. While outcomes for GUCH surgeries and coarctation repair were comparable, most other CHD surgical categories showed elevated mortality.[21] Platforms such as the Annals of Pediatric Cardiology (APC) and the Indian Heart Journal have published Indian data, raising visibility and stimulating academic interest. However, the efforts are fairly limited considering the enormous volume of GUCH patients seen in India. For instance, a review of original articles published in the APC over the last 3 years identified only two articles (2.4%) related to the GUCH population.

CHALLENGES REMAIN!

Despite these positive efforts, GUCH care remains in the periphery of adult and pediatric cardiac services in India. The fact that only a handful of trained GUCH specialists are there does not augur well for a country as big as India. The GUCH services also took the maximum hit during the COVID-19 pandemic.[22,23]

WHAT SHOULD AN IDEAL GROWN-UP WITH CONGENITAL HEART DISEASE CARE LOOK LIKE?

An effective GUCH program in an LMIC like India should incorporate:

Level-based stratification

Following the model proposed by the ESC,[3] GUCH patients should be categorized into three levels of complexity, each with designated follow-up strategies [Figure 1]. Level 1 centers provide basic surveillance for simple, repaired lesions and can be managed by general physicians/cardiologists with access to teleconsultation. Level 2 centers offer shared care for moderate cases with diagnostic and interventional capabilities and serve regional populations. Level 3 centers are specialized referral hubs with comprehensive multidisciplinary teams, advanced imaging, surgical and electrophysiology services, and function as academic and research centers for GUCH.

Figure 1.

Suggested level-based grown-up with congenital heart disease-care pyramid for India. TOF: Tetralogy of Fallot, TAP: Transannular patch, GUCH: Grown-up with congenital heart disease, MRI: Magnetic resonance imaging, EP: Electrophysiology, TAPVC: Total anomalous pulmonary venous drainage, VSD: Ventricular septal defect, TEE: Transesophageal echocardiography, CT: Computerized tomography, PDA: Patent ductus arteriosus, ASD: Atrial septal defect, ECG: Electrocardiography, CXR: Chest X-ray

Specialized centers

Each GUCH center should serve 5–10 million people and have access to adult and pediatric cardiology, congenital imaging, electrophysiology, surgical backup, and transition care protocols.[24] Essential personnel include adult congenital cardiologists, pediatric cardiologists, congenital cardiac surgeons, cardiac anesthesiologists, electrophysiologists, imaging specialists proficient in echocardiography and cardiac MRI, heart failure and transplant experts, and an obstetric cardiology team. Support staff such as nurse coordinators, psychologists, clinical geneticists, and social workers are also vital for holistic care.[3,9]

Considering our 1 billion adult population, we need a minimum of 100 GUCH-care specialist centers. Currently, the number is <10 across India. We need to upscale some of the pediatric centers and institutes of national importance as specialized GUCH-care centers.

Transition of care

Structured transition from pediatric to adult services by 16–18 years should become the norm. Even in highly proficient pediatric cardiac centers of India, the patients are being followed by the same pediatric team due to manpower and infrastructure restrictions.

Training programs

We need to include GUCH modules in cardiology curricula and establish 1–2-year fellowships in GUCH care. Both interested and dedicated pediatric and adult cardiologists may be imparted with the necessary training to enable them to care for the varied needs of a GUCH patient comprehensively. This may happen in the pattern of Fellow of National Board format, or the Pediatric Cardiac Society of India can initiate such fellowships similar to the successfully running intensive care fellowship program. Furthermore, selected candidates may get themselves trained in the nuances of GUCH care from established Western centers to improve the processes and outcomes. Such trained manpower is urgently needed in GUCH subspecialties such as imaging, electrophysiology, and transplantation. Improving the GUCH training landscape will not only improve patient care but also improve the career opportunities for the trained pediatric cardiologist in India, which remains an area of concern for the specialty.[25]

Registry and research

There is an urgent need for a national ACHD registry and support for indigenous data-driven guidelines, rather than borrowing Western algorithms wholesale. Unoperated single ventricle palliation, operability in CHD with pulmonary hypertension, unoperated TOF with complications of cyanosis, and various CHD complicated by valve regurgitation or ventricular dysfunction are problems encountered specifically in India and other LMICs. Most of the innovations in the field of cardiology and cardiac surgery emanating from India have focused on low-cost innovations for late presenters with CHD, including the GUCH population.[14,26] We need dedicated registries to understand the issues and identify solutions.

Improving the quality of life for grown-up with congenital heart disease patients

Getting quality diagnostic and therapeutic care is only part of the problem. GUCH patients have compromised quality of life and deserve psychological and social support. We need to set up social help groups, provide cardiac rehabilitation, and offer counseling. Unfortunately, such comprehensive care pathways are lacking in even the established centers of India. Employability, marriage counseling, exercise participation, and air travel are some of the aspects that need specialized attention.

THE WAY FORWARD

Grown-up with congenital heart disease care by pediatric or adult cardiologist – An artificial divide

One of the central debates in India is: Who should care for GUCH patients? While pediatric cardiologists understand the congenital anatomy and repair history, adult cardiologists bring expertise in managing acquired comorbidities, pharmacology, and catheter-based interventions. In reality, neither specialty alone is sufficient. The solution lies in hybrid training and collaborative models, not turf wars. In India, the absence of regulatory clarity (e.g., from the National Medical Council) on adult congenital competencies exacerbates confusion.[27] What we need is training, not territoriality.

CONCLUSIONS

India stands at a crossroads in GUCH care. With over 3.5 million potential patients and with <10 specialized centers, most currently these patients “belong to no one.” GUCH patients deserve more than fragmented, reactive care. They require an integrated, compassionate, and forward-looking caregiver system. Creating dedicated GUCH training, fostering multidisciplinary teams, and institutionalizing long-term follow-up are essential. The GUCH dilemma is not just about where these patients belong – it’s about who chooses to take responsibility. It is time India rises to this challenge.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.