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Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 137-139

Management of an adolescent boy with a congenital heart disease: The value of care coordination

Basic Health Care Services, Udaipur, Rajasthan, India

Date of Submission26-Feb-2021
Date of Decision25-Apr-2021
Date of Acceptance30-Apr-2021
Date of Web Publication31-May-2021

Correspondence Address:
Dr. Pavitra Mohan
Basic Health Care Services, 39, Krishna Colony, Bedla Road, Udaipur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ipcares.ipcares_60_21

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How to cite this article:
Goel G, Mohan P, Mohan SB. Management of an adolescent boy with a congenital heart disease: The value of care coordination. Indian Pediatr Case Rep 2021;1:137-9

How to cite this URL:
Goel G, Mohan P, Mohan SB. Management of an adolescent boy with a congenital heart disease: The value of care coordination. Indian Pediatr Case Rep [serial online] 2021 [cited 2022 Jan 17];1:137-9. Available from: http://www.ipcares.org/text.asp?2021/1/2/137/317360

In 2014, two tribal teenagers (17 and 18 years old, respectively) with congenital heart disease presented to our clinic within a span of 2 weeks. Their late presentation was not due to the mild nature of their disease, but rather a reflection of the challenges, they had faced to get a diagnosis and proper treatment since early childhood. We describe the history, management, and outcome of one of them to illustrate the kind of care coordination required for vulnerable children with medical conditions that warrant advanced health care but who lack access to health facilities that can provide it.

  Case Study Top

Ramlal (name changed) was born in Manpur village of Salumbar tehsil of Udaipur district. More than 90% of the population of the village is tribal. The nearest town is 30 km away and the nearest tertiary hospital is 100 km away. Ramlal was 16 years old at initial presentation and complained of breathlessness and easy fatigability from as far back as he could remember. When he arrived, he looked visibly breathless. His weight was 35 kg and height was 54 cm. His pulse rate was 92/min, respiratory rate was 28/min, and blood pressure was 110/72 mmHg. He had central and peripheral cyanosis, Grade III clubbing, and an oxygen saturation of 84% in room air. On auscultation, his chest was clear. The rest of the examination performed by the health worker (HW) was unremarkable. His hemoglobin level was ascertained to be 18% g by a Sahli's hemoglobinometer.

Ramlal was born at home. His father tills a small piece of land and his mother is a homemaker. He has two sisters, both of who were healthy. He had attended school till class 5 but then had to drop out due to his illness. From early childhood (about 2 years of age), Ramlal's parents had taken him to various health-care providers, including the local primary health center and even faith healers and local quacks. They had never learned that he had a heart disease. Finally, when there was no improvement, they resigned to fate and believed that his disease was due to supernatural causes. About 6 years earlier, Ramlal's condition deteriorated with acute worsening of his usual breathlessness. At that time, his parents conferred with other villagers, borrowed money, and took him to the nearest hospital 100 km away, where he was admitted. At discharge, he was informed that he has a severe heart defect since birth, which could be corrected by an operation, but the facilities for that were available only in Jaipur. His father took another loan and got him admitted in the medical college in Jaipur. An echocardiogram confirmed that he had a heart disease. He was told that the operation carried a “high risk to life” would cost about Rs. 50,000, and he should apply to the Chief Minister Relief Fund for reimbursement of the costs of surgery. Not knowing how to proceed further, and unable to get any more guidance, father and son returned disheartened to their village.

Ramlal continued to silently harbor a desire to get relieved from his misery. He kept all his papers and reports carefully, hoping against hope that his desire to get operated would be fulfilled someday. When he was 17 years old, he chanced upon the male HW from our clinic during a community outreach visit to his village and they got talking about his health issues. The HW brought him to the clinic to see if we could help him. We reviewed his studies and discovered that the echocardiography report showed a tetralogy of Fallot, adequately sized confluent pulmonary arteries, and severe infundibular and valvular pulmonary stenosis. While we were searching for options, we learned of the UN Mehta government run Institute of Cardiology in Gujarat that offered free cardiac surgery for tribal patients and reportedly had good surgical outcomes. However, documents confirming tribal status and proof of residence of the patient were required. The primary health-care team assisted the family in collecting and collating these documents. Since the family had never visited Ahmedabad, they were extremely apprehensive about going there by themselves. The PHC team was also concerned that Ramlal and his parents would not be able to navigate the process of hospitalization on their own. It was decided that the PHC physician and HW would accompany them to Ahmedabad, help with the formalities of admission, liaise with the cardiologist and cardiothoracic vascular surgeon, and arrange for blood transfusion and other prerequisites.

After getting admitted, Ramlal underwent a series of investigations to assess the preoperative state – echocardiography, cardiac catheterization, and a multiple slice pulmonary computerized tomography. These confirmed the earlier findings and revealed other intricate vascular details: the right pulmonary artery measured 8.3 mm, there was a large aortopulmonary collateral (measuring 2.8 mm) that emerged from the descending aorta at T5/T6, had a tortuous course, and ascended to T-3 level, bifurcating into two branches at the right upper lobe and right hilum. There were a few other smaller ill-defined collaterals. Based on this, surgery was planned and a week later, a trans-right atrium intracardiac repair was performed successfully. This was followed by cardiac catheterization and coiling of the major aortopulmonary collaterals a day later. The postsurgical period was uneventful and he was discharged after a week. He returned to the village a different person, pink, with abated breathlessness and no longer constantly fatigued.

At present, 6-year postsurgery, he is healthy, symptomless on accustomed work and his clubbing is reversed. He does get breathless still on heavy exertion. The community HW helped him get enrolled in a vocational training course on tailoring. After completion, he started his own shop and earns about Rs. 9000 every month. He also got married a year ago.

  Discussion Top

Most children with critical congenital heart diseases in developed countries that require surgical intervention within the 1st year of life are detected during pregnancy or in the early neonatal period. In low- and middle-income countries like India, antenatal and neonatal identification is low, many children succumb to their cardiac disease in the first few weeks of life without getting diagnosed, there are few and inequitably distributed pediatric cardiothoracic centers, and the costs of surgery are often beyond the means of a person belonging to the lower socioeconomic strata. This means that only a small proportion of all critical congenital heart diseases (CHDs) is detected in time, and fewer still are operated.[1] Those from the hinterland are even less likely to be diagnosed, operated, survive, or lead a normal life. While there are program initiatives for early detection of defects at birth like the Rashtriya Bal Suraksha Karyakram, and state-funded insurance schemes, public awareness is poor and the coverage is low. It is estimated that in India, about a million children with CHDs remain unoperated.

Many families with children from the hinterland affected with congenital disorders seek medical attention and move to and fro primary and tertiary health-care centers if they survive. They have limited resources, lack awareness, and exposure and are very fearful of visiting hospitals in the city. In the process of seeking optimal care, they may end up spending a lot of money, getting indebted, and having to sell the few assets that they have like pieces of silver jewelry, or small pieces of land. In the absence of an informed person or institution to help them navigate the complex and often unresponsive systems of health care, insurance, and social support, they often recede into oblivion, without receiving medical attention. Ramlal was indeed fortunate to have gained access to a responsive primary health-care system that coordinated his care from diagnosis till the completion of treatment and even beyond.

Care coordination is increasingly recognized as an important function or service for managing children with chronic diseases and those with special needs. Such children and their families often require assistance in negotiating advanced and expensive medical care, social support, and vocational or educational support. A care coordinator assists the family in coordinating care across systems (i.e., health care and social service) and across primary, secondary, or tertiary levels of health care.[2] To enable them to function effectively and efficiently, they should be aware of the resources (institutions, schemes, and entitlements) that are available for the care of these children and how to tap them if required. The care coordinator needs to be in essence, first of all, an advocate of the child, especially when the family has limited means and resources to do the needful themselves.

People living in remote rural areas are financially burdened and have limited education and need enormous help at all levels. In this particular case, the primary care clinic acted as the care coordinator, assisting the child to connect with the tertiary clinical care, and linking them with the entitlement that provided care free of cost. This required assisting with the documents, physical accompaniment, and negotiating care on behalf of the family. The care coordination role also extended to linking the individual with vocational training that helped the boy integrates with the social and economic life like his peers in the village. Primary care providers, especially pediatricians, should be exhorted to play the role of care coordination. The health systems and insurance mechanisms need redesigning to allow care coordination to become incentivized so that more primary care physicians and pediatricians will be motivated to assume lead roles and advocate for making systems responsive to their needs. Operational and implementation research is required to understand the pathways of care that children from disadvantaged families with reversible congenital conditions need to traverse.

It was Rudolf Virchow who stated that “A physician is a natural attorney of the poor.”[3] By extension, a pediatrician is a natural attorney of children. Coordinating care of children, especially those from poor families, and with chronic health needs, would be one step in playing that role.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

The clinic in which this child was managed has been supported through a grant by Bajaj Auto Limited, through their CSR unit.

Conflicts of interest

There are no conflicts of interest.

  References Top

Saxena A. Congenital heart disease in India: A status report. Indian Pediatr 2018;55:1075-82.  Back to cited text no. 1
Committee on Children with Disabilities. Care coordination: Integrating health and related systems of care for children with special health care needs. Pediatrics 1999;104:978-81.  Back to cited text no. 2
Brown TM, Fee E. Rudolf Carl Virchow: Medical scientist, social reformer, role model. AJPH 2006;96:2104-5.  Back to cited text no. 3


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