Caring for nobody’s baby

Mani Ram Krishna Singaravelu*

Consultant – Pediatric Cardiologist, Kauvery Hospital, HeartCity, Trichy, India

In my last guest editorial, I had attempted to portray the need for a comprehensive pediatric cardiac facility in central Tamil Nadu. I had stressed the importance of timely recognition of critical congenital heart disease (CHD) in the newborn baby and the improved outcomes in the surgical and percutaneous repair of CHD. A baby with any form of critical CHD can hence expect to lead a near-normal life based on most health-related quality of life (HR-QoL) parameters. This has hence enabled most pediatric cardiologists to think beyond immediate outcomes towards supporting the survivors through the challenges beyond the acute care period. In the west, the prevalence of adults with congenital heart disease (ACHD) has superseded that of children with CHD even in the subset with complex cardiac lesions with 2/3rd of the entire CHD cohort comprised of adults [1]. India is likely to have a large ACHD population but no data is available at present on the magnitude of the problem.

As children with both repaired and uncorrected CHD grow into adolescence and adulthood, they face a unique set of medical challenges different from those of children with CHD. As they enter adulthood and turn their minds to earn a livelihood, they need guidance on the kind of roles they will be able to effectively perform without affecting their cardiac health. While most adults with corrected CHD, will be able to perform the strenuous activity, those with palliated CHD or residual defects will need restriction of activities based on their current hemodynamic condition. Adult women with repaired CHD will need guidance on conjugal life and pregnancy. Adolescents and young adults move out of the shadows of their parents and experience intense peer pressure to conform to a lifestyle that may not be permissible with their cardiac condition. They require mental support and guidance to navigate the challenges faced by them. The ACHD population in developing countries includes many intervention-naí¯ve adults who are often very sick and require specialized intensive care. The subset with repaired CHD is heterogeneous and caters to their needs a detailed understanding of their underlying hemodynamics as well as anticipation of complications specific to their anatomy. Recognizing the considerable challenges that exist in the management of the ACHD cohort, pioneers of congenital cardiology in the West such as Dr. Joseph Perloff and Dr. Jane Somerville pushed for the recognition of ACHD as an independent sub-specialty with a focused training program. Structured fellowships exist for the training of cardiologists in ACHD management and standardized guidelines have been created for the smooth transition of CHD survivors from pediatric cardiologists to ACHD specialists with a transition clinic part of most large pediatric CHD programs in the west [2-4]. Sadly, such training programs do not exist anywhere in the developing world. Pediatric cardiologists who have a better understanding of the anatomy and hemodynamics of the disease are too few and overburdened with the acute and sub-acute care of smaller children with CHD. Adult cardiology programs in our country do not offer sufficient training for the management of ACHD. In a deeply thought-out review on the challenges of ACHD management in resource-constrained settings, Dr. Anita Saxena termed the ACHD community as “nobody’s children” stressing the challenges of both pediatric and adult cardiologists in owning management of adults with CHD [5].

Dr. Saxena had elegantly listed the challenges faced in the management of ACHD in developing countries including financial challenges, lack of specialist training, lack of political will in prioritizing a small subset of patients, exclusion from insurance coverage as well as the small number of institutions with capabilities to handle ACHD patients. In particular, she had pointed out the lack of data about the disease burden in developing countries and the absence of professional organizations dedicated to caring for the ACHD community [5]. While suggesting solutions for tackling the problem, she had stressed the need to prioritize research in order to collect data on the magnitude of the problem including setting up population-based registries. This data can be used to highlight the challenges faced in ACHD management and can provide justification for establishing training programs and institutes for advanced care. In the present scenario, ACHD is managed by pediatric cardiologists and a small number of adult cardiologists with training in CHD management in India.

As part of our nascent ACHD program at Heart City over the past year, we have performed percutaneous interventions on seven adults with CHD and surgical repair on 4 adults with good outcomes in all patients. Our burgeoning ACHD clinical registry includes 23 young adults with repaired CHD who are being followed up in a structured form

References

Marelli AJ, Ionescu-Ittu R, Mackie AS, et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130(9):749-56.
Moons P, Bratt EL, De Backer J, et al. Transition to adulthood and transfer to adult care of adolescents with congenital heart disease: a global consensus statement of the ESC Association of Cardiovascular Nursing and Allied Professions (ACNAP), the ESC Working Group on Adult Congenital Heart Disease (WG ACHD), the Association for European Paediatric and Congenital Cardiology (AEPC), the Pan-African Society of Cardiology (PASCAR), the Asia-Pacific Pediatric Cardiac Society (APPCS), the Inter-American Society of Cardiology (IASC), the Cardiac Society of Australia and New Zealand (CSANZ), the International Society for Adult Congenital Heart Disease (ISACHD), the World Heart Federation (WHF), the European Congenital Heart Disease Organisation (ECHDO), and the Global Alliance for Rheumatic and Congenital Hearts (Global ARCH). Eur Heart J. 2021;42(41):4213-23.
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020. ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563-645.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019;73(12):e81-e192.
Saxena A. Adult With Congenital Heart Disease in Developing Country: Scope, Challenges and Possible Solutions. Curr Treat Options Cardiovasc Med. 2015;17(11):46.

Dr. Mani Ram Krishna Singaravelu

Consultant – Pediatric Cardiologist

Caring for nobody’s baby