Looking beyond survival in congenital heart diseases: Are we there yet?

Over the past three decades, the landscape of congenital heart disease (CHD) has undergone a remarkable, transformative change in a positive direction. What was often a uniformly fatal diagnosis in many parts of the world has evolved into a survivable condition, even in low- and middle-income countries (LMICs), including India. Advances in congenital cardiac sciences, interventional and surgical techniques, perioperative care in the intensive care units, and rising institutional experiences have steadily improved early survival. Yet, as we celebrate these gains, an important question still remains: Is survival enough? The answer, clearly, is no. THE PAUCITY OF MEANINGFUL LONG-TERM DATA While mortality data are now more widely reported,[1-4] in reviews and surveys, the lack of robust national databases with wide-based participation still remains a work in progress. Although international collaboratives like the International Quality Improvement Collaborative and World Database for Pediatric and Congenital Heart Surgery have made important inroads in LMICs, the data largely remains till 30-day mortality data due to the inherent nature of such databases.[5,6] These are important metrics, but they are incomplete. Quality of CHD care can be ascertained only with meaningful data, and there is ample evidence in the literature to support this.[7] Granular, long-term outcome data can come only from well-maintained institutional databases, which remain largely absent in most LMICs, including India. The absence of structured follow-up systems, fragmented data capture, and resource constraints has resulted in a significant blind spot. We know how many children survive surgery or interventions; we know far less about how they live thereafter. FUNCTIONAL OUTCOMES: THE UNMEASURED REALITY While a child discharged after successful surgery is often labelled a “good outcome,” many of these children live with limited exercise tolerance, residual lesions, burden of multiple surgeries or re-interventions and chronic cardiac dysfunction. Functional status, one of the most meaningful patient-centered outcomes, is rarely documented in a systematic way. Simple measures such as New York Heart Association or Ross classification, exercise capacity, or school participation are seldom incorporated into routine reporting. It is time for us to move beyond survival to “meaningful survival,” and for that, functional recovery should be a central outcome metric, not an afterthought. NEURODEVELOPMENT: THE SILENT BURDEN Perhaps the most ignored consequence of CHD and its treatment is neurodevelopmental impairment. Increasing evidence suggests that children with complex CHD are at risk of cognitive, behavioral, and learning difficulties, influenced by factors such as prenatal brain development, perioperative insults, and socioeconomic environment. While a laudable multicentric study from India did recently assess the neurodevelopmental outcomes in infants with CHD after surgery, the results showed suboptimal results in this subgroup and the long road ahead for their rehabilitation.[8] More such data-centered studies with long-term assessments will show the future path. QUALITY OF LIFE: BEYOND CLINICAL METRICS Another important metric beyond survival statistics is quality of life (QoL), encompassing physical, emotional, and social well-being. This is a critical endpoint that remains insufficiently explored. The burden of suboptimal QoL often extends beyond the patient with families facing financial strain, repeated hospital visits, and long-term caregiving responsibilities. Without assessment of QoL, we risk underestimating the true cost of survival and its impact on families. LATE MORBIDITY: THE PRICE OF SUCCESS As survival improves, a significant population of children is reaching adolescence and adulthood with repaired or palliated CHD. This has brought into focus the issue of late morbidity, particularly in patients with single-ventricle physiology. The Fontan circulation, once considered the final stage of palliation, is now recognized as a chronic suboptimal physiological state with inherent limitations.[9] Complications such as protein-losing enteropathy, plastic bronchitis, liver fibrosis, and eventual Fontan failure are increasingly encountered. Similarly, systemic right ventricular dysfunction in conditions such as transposition of the great arteries poses long-term challenges. While these late sequelae are expected outcomes, without longitudinal data, the true burden remains underappreciated. THE UNIQUE CHALLENGE OF LATE PRESENTATION Adding more complexity to this landscape is the persistent problem of late presentation, particularly in LMICs. Children often have established physiological derangements such as pulmonary vascular disease, ventricular dysfunction, or malnutrition. With careful selection, the surgical correction in these late presenters is largely technically successful, but patients may carry a higher risk of suboptimal long-term outcomes.[10] Survival in this context may thus represent only a partial victory. The interplay between delayed intervention or surgery and long-term morbidity requires deeper analysis armed with real-world data. WHERE DO WE GO FROM HERE? Moving beyond survival does not require sophisticated infrastructure at the outset. It requires a shift in mindset and gradual, incremental change. A practical approach may include: Longitudinal tracking of a small set of outcomes: functional class, reintervention rates, and late complications Adding basic neurodevelopmental screening into follow-up Using simple, validated tools for QoL assessment Strengthening participation in collaborative databases and registries and multicentric studies. The journey from survival to meaningful recovery is dependent on many factors, particularly in resource-crunched environments. Yet, acknowledging this gap in our knowledge and care delivery is the first step toward bridging it. We have, as a community, made significant strides in ensuring that children with CHD survive. The next frontier is making sure that they live well.