Importance and Feasibility of Creating Hypertrophic Cardiomyopathy Centers in Developing Countries: The Experience in India Hypertrophic cardiomyopathy (HC) is a relatively common genetic heart disease responsible for mortality and morbidity at all ages. Using contemporary treatment advances, such as implantable defibrillators, surgical myectomy, heart transplant, and modern defibrillation for out-of-hospital cardiac arrest, it is now possible to reduce HC-related mortality considerably to 0.5% per year, less than expected in the general US adult population. However, in much of the developing world, HC has not yet become a priority given the many other cardiac conditions, such as coronary artery disease and systemic hypertension, so prevalent in the most populous countries such as China and India. Management of HC is best achieved in dedicated centers within institutions, such as previously demonstrated in the United States, Canada, some European countries, and Australia. This model has recently been introduced for the first time in India at the Amrita Institute of Medical Sciences and Research in Kochi, Kerala, in which a robust program focused on HC has emerged. This novel initiative, created despite the many obstacles in the Indian health care system, is an important step forward and is reported here detail. Ó 2015 Elsevier Inc. All rights reserved. (Am J Cardiol 2015;116:332e334)

Hypertrophic cardiomyopathy (HC) is a common form of inherited heart disease with a prevalence of at least 1:500.1 This disease has been of considerable interest to cardiologists for the past 50 years but often overwhelmed in cardiovascular practice by the vast number of patients with coronary artery disease and systemic hypertension. This has been particularly true in underdeveloped countries such as India and China with vast populations (>1 billion), and compounded by limited resources without widely available health insurance to cover the costs potentially incurred by patients.2 The concept of centers or programs within divisions of cardiology dedicated to the diagnosis and management of less common diseases such as HC has been promoted as an advantageous strategy to provide patients with the best opportunity for appropriate diagnosis and treatment, extended longevity, and better quality of life.3e5 In such centers of excellence, patients with HC have the benefit of advanced imaging for diagnosis, genetic/family counseling, and all major management options, including implantable cardioverter-defibrillators for sudden death prevention and surgical myectomy (and alcohol septal ablation) for reversal of progressive heart failure because of left ventricular outflow obstruction5,6 and heart transplant for end-stage nonobstructive disease.7 In such centers, HC-related mortality in adult patients has been reduced to 0.5% per year.8 Creating such a program in a populous country such as India is a major challenge. This is particularly true when considering that only a fraction of citizens are eligible for, or have the means to obtain medical/health insurance; for example, 5% of the

See page 334 for disclosure information. Am J Cardiol 2015;116:332e334 0002-9149/15/$ - see front matter Ó 2015 Elsevier Inc. All rights reserved.

overall population of India compared with 87% in the United States.2 A novel example of one such initiative is emerging in southern India, evolving against numerous pre-existing obstacles within the Indian health care system. That program is at the Amrita Institute of Medical Sciences and Research in Kochi, Kerala, with Dr. Hisham Ahamed as Project Leader of the HC Center and Dr. K. U. Natarajan as the Chief of Cardiology (Figure 1). Justification for the HC Center Based on considerable research for the past 10 to 15 years, primarily from US investigators and European/Australian collaborators, HC is now regarded as a treatable form of heart disease with the possibility of achieving normal longevity.5,6 With the aspiration of establishing a similar level of care for Indian patients, cardiologists at Amrita have taken the initiative for the past 18 months to create an HC center model to benefit such patients in ways not previously available in India by following the strategies used by formalized programs in the United States: (1) designate 1 predominant cardiologist, well versed in the HC disease spectrum, to fulfill the requirements of Director/Project Leader, responsible for primary diagnosis and management decisions for all patients enrolled in the center, in collaboration with other cardiologists; (2) recruiting a cardiac surgeon having specific experience with the septal myectomy operation; (3) formulate an expert electrophysiology service, including paramedical staff for device follow-up, with a focus on primary prevention with implantable defibrillators, according to the US guidelines for HC9,10; (4) designate an experienced interventional cardiologist to perform alcohol ablation as a selective alternative to surgical septal myectomy; (5) train personnel for www.ajconline.org

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Figure 1. The Amrita Institute Hypertrophic Cardiomyopathy Team. Front row (seated, left to right): Dr. Rajesh Thachathodiyil (consultant cardiologist and Director of Cardiac MRI and Cardiac CT programs); Dr. C. Rajiv (consultant cardiologist and Director of Echocardiography Laboratory; Dr. K. U. Natarajan (consultant cardiologist and Chief of the Department of Cardiology and Director of Electrophysiology Services). Dr. Praveen Varma (Head of the Department of Cardiothoracic Surgery and Cardiac Transplant Unit, and myectomy surgeon); and Dr. M. Vijayakumar (consultant cardiologist and Director of Heart Failure Clinic). Back row (standing, left to right): Dr. Praveen G. Pai (consultant cardiologist and senior electrophysiologist); Dr. Ashik Sasidharan (consultant cardiologist); Dr. Hisham Ahamed (consultant cardiologist and Project Leader of the Amrita HC Clinic and Registry); Dr. Navin Mathew (consultant cardiologist and Co-director of the Cardiac Transplant Services); and Dr. Saritha Sekhar (consultant cardiologist).

advanced imaging with cardiovascular magnetic resonance and echocardiography and designated readers for interpreting HC images; (6) develop access to outside fee-for-service genetic testing; (7) in-hospital/clinic electronic retrieval of medical records; (8) systematic computer entry of data related to all HC visits in an independent spreadsheet for the purpose of clinical research; and (9) access to a heart transplant service, recognizing the growing importance of advanced heart failure in nonobstructive HC.5,8 In addition, Kochi has a well-developed and robust Pediatric Cardiology Section with 6 cardiologists and 3 surgeons, reflecting the importance attributed to the many children and adolescents identified with HC. At this time, the new center in Kochi has already enrolled 225 patients in 18 months, and the future expectation for patient volume is very high, given that the population of the state of Kerala is itself almost 35 million. This initiative in HC reflects a growing interest in such programs, as evidenced by another planned center in New Delhi at Kalra Hospital.2 Strategies for Financial Support The HC dilemma in India is related in large measure to the national medical insurance crisis in which only w5% of the population have reasonably comprehensive governmental or employer-sponsored coverage.11 Therefore, for 95% of patients, the cost of care rests almost entirely with the patient’s personal monetary resources, including either the truly wealthy upper class who do not require health insurance, average families required to assemble payment from

life savings and the assistance of relatives (including some who may reject insurance on a cultural basis), and a small minority capable of raising funds from charities. Indeed, there are numerous obstacles to universal health insurance in India, not the least of which is that 70% of the population is rural and only a minority of the country has access to allopathic (Western) medicine and modern medical treatments. In addition, some physicians are trained in homeopathy and ayurveda alternate medicine, but attempt to practice allopathic medicine with limited knowledge. The private health care sector is reportedly fraught with kickbacks, excessive commercial transactions directly between patients and doctors, malpractice, and false reporting.12 The private health insurance market is underdeveloped, and growth has been challenging and slow in expanding health care coverage. The most reliable cost estimates for evaluation and treatment in India (converted to US dollars) applicable to HC are as follows: (1) (2) (3) (4) (5) (6) (7) (8) (9) (10) (11)

12-lead electrocardiogram ¼ $2, Echocardiogram ¼ $15, Cardiovascular magnetic resonance imaging ¼ $160, Stress echocardiogram ¼ $50, Cardiac consultation ¼ $5, Implantable defibrillator ¼ $10,000, 24-Hour (Holter) electrocardiogram ¼ $20, Heart transplant ¼ $15,000, Myectomy ¼ $2,000, Genetic testing ¼ $350, and Beta-blocker ¼ $5/month.

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Although these personal out-of-pocket charges for an HC evaluation are exceedingly modest on the US scale (which would be 10- to 100-fold greater), these costs would nevertheless leave thousands of Indian patients without any of the essential diagnostic and treatment options. In contrast, it is also important to point out that with at least 1 million identifiable patients with HC in India and an estimated 10% with comprehensive insurance (or sufficient and readily available personal resources), there could still be at least 100,000 Indian patients with HC for whom contemporary treatments would be available even under the current limitations of the health care system.

Implantable Defibrillators The Indian health care environment is particularly unfavorable to primary prevention defibrillator strategies. An estimated 70% of Kochi Center patients would likely not have sufficient personal resources to cover the cost of this potentially life-saving therapy13 nor the myectomy operation.14 However, the hospital has created a model for expanding the available options to accommodate selected patients with HC: (1) special justification for defibrillators from the Federal government; (2) ex-serviceman health scheme panels; (3) an in-house charity foundation that provides substantial resources to the underprivileged based on need; and (4) the Rotary Pacemaker Bank, which donates new and refurbished devices, available at no cost. In conclusion, HC is a much more common genetic heart disease than previously regarded subjecting an important minority of patients to significant morbidity and mortality. However, in certain parts of the world, including the highly populous country of India, patients with HC have been impaired in a number of ways, including misunderstanding regarding the natural history of their disease and, most importantly, access to those contemporary diagnostic and management strategies that have reduced disease-related mortality rates so significantly in the United States.8 Formalized centers dedicated to the care of patients with HC, so successful in the United States, can be expected to have similar impact in India, even with the many logistical barriers currently encumbering the health care system.

Disclosures The author have no conflicts of interest to disclose.

Barry J. Maron, MD Hypertrophic Cardiomyopathy Center Minneapolis Heart Institute Foundation Minneapolis, Minnesota 1. Semsarian C, Ingles J, Maron MS, Maron BJ. New perspectives on the prevalence of hypertrophic cardiomyopathy. J Am Coll Cardiol 2015;65:1249e1254. 2. Maron BJ, Kalra A. Hypertrophic cardiomyopathy in the developing world: focus on India. Eur Heart J 2014;35:2492e2495. 3. Maron BJ. Hypertrophic cardiomyopathy centers. Am J Cardiol 2009;104:1158e1159. 4. Maron BJ, Rastegar H, Udelson JE, Dearani JA, Maron MS. Contemporary surgical management of hypertrophic cardiomyopathy, the need for more myectomy surgeons and disease-specific centers, and the Tufts initiative. Am J Cardiol 2013;112:1512e1515. 5. Maron BJ, Ommen SR, Semsarian C, Spirito P, Olivotto I, Maron MS. State-of the Art. Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol 2014;64:83e99. 6. Maron BJ, Braunwald E. Evolution of hypertrophic cardiomyopathy to a contemporary treatable disease. Circulation 2012;126:1640e1644. 7. Harris KM, Spirito P, Maron MS, Zenovich AG, Formisano F, Lesser JR, Mackey-Bojack S, Manning WJ, Udelson JE, Maron BJ. Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation 2006;114:216e225. 8. Maron BJ, Rowin EJ, Casey SA, Link MS, Lesser JR, Chan RHM, Garberich RS, Udelson JE, Maron MS. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies. J Am Coll Cardiol 2015;65:1915e1928. 9. Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH, Spirito P, ten Cate FJ, Wigle ED. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Task Force on clinical expert Consensus Documents and the European Society of Cardiology Committee for practice guidelines Committee to develop an expert Consensus Document on hypertrophic cardiomyopathy. J Am Coll Cardiol 2003;42:1687e1713; Eur Heart J 2003;24:1965-1991. 10. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rawkowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW. 2011 ACCF/AHA guidelines for the diagnosis and treatment of hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation 2011;124: 2761e2796; J Am Coll Cardiol 2011;58:e212ee260. 11. Rao SK. Health Insurance in India. “Financing and Delivery of Health Care Services in India”. India: Ministry of Health and Family Welfare, Government of India, 2005:pp275e277. 12. Gadre A. India’s private healthcare sector treats patients as revenue generators. BMJ 2015;350:h826. 13. Maron BJ, Spirito P, Shen W-K, Haas TS, Formisano F, Link MS, Epstein AE, Almquist AK, Daubert JP, Lawrenz T, Boriani G, Estes NA III, Favale S, Piccininno M, Winters SL, Santini M, Betocchi S, Arribas F, Sherrid MV, Buja G, Semsarian C, Bruzzi P. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. JAMA 2007;298:405e412. 14. Ommen SR, Maron BJ, Olivotto I, Maron MS, Cecchi F, Betocchi S, Gersh BJ, Ackerman MJ, McCully RB, Dearani JA, Schaff HV, Danielson GK, Tajik AJ, Nishimura RA. Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol 2005;46:470e476.

Importance and feasibility of creating hypertrophic cardiomyopathy centers in developing countries: the experience in India.

Hypertrophic cardiomyopathy (HC) is a relatively common genetic heart disease responsible for mortality and morbidity at all ages. Using contemporary ...
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