Cardiomyopathy is the serious disease of heart muscles in which heart muscles become abnormally large and weak which make it difficult to pump blood properly. These condition further results into irregular heartbeats (arrhythmias) or heart failure. Additionally, few cases involve the formation of fat or scar tissue within the heart muscles. There are various form of cardiomyopathy include:
(1) Dilated cardiomyopathy is the most common form of cardiomyopathy in children in which heart muscles get attenuated.
(2) Hypertrophic cardiomyopathy in which the heart muscles get too thick.
(3) Restrictive cardiomyopathy is a rare form of cardiomyopathy found in children’s that causes heart muscles to get stiff or rigid.
Arrhythmogenic right ventricular cardiomyopathy occurs when the muscles of heart’s right ventricle is replaced by fatty scar tissue. This is rare type of cardiomyopathy only affects one in five thousand people. Pediatric cardiomyopathies are disorders that mainly affect the muscles of the heart. At any given time point, they affect at least 100,000 children globally. In United States, at least 1 in every 100,000 children below the age of 18 years experiences a diagnosis of primary cardiomyopathy. The highest prevalence is in children under 1 year of age. Morbidity and mortality of these diseases are high and also most common cause of a heart transplant in children older than 1 year. Approximately, 40 percent of children with a symptomatic cardiomyopathy either undergo heart transplantation or die within 2 years.
Treatment & management of cardiomyopathy
Treatment of cardiomyopathy includes
1. Drug Therapy
2. Device implantation
• Pacemaker (PPM)
• Automatic Implantable Cardioverter Defibrillator (AICD)
3. Surgical options
• Heart transplantation
4. Additional treatments
• Genetic Testing
• Specialized Metabolic Treatment
Usage of stem cells on cardiomyopathy
Mesenchymal stem cells have the potential to self-replicate and differentiate into several tissue lineages, and have been implementing in regenerative therapies for cardiac diseases. MSCs may be isolated from various tissues such as adipose tissue, bone marrow and umbilical cord, but it is unclear whether these stem cells share the similar cardiopoietic and immunomodulatory properties. MSCs are unique immunologically as they have reduced expression of MHC class-I molecule, and lack of MHC class-II and co-stimulatory molecules CD80 (B7-1), CD86 (B7-2), and CD40. MSCs are immunomodulatory and immunoprivileged cells along with proregenerative effects which have been proven to be safe and to stimulate reverse remodeling in ischemic cardiomyopathy. In preclinical study, human bone marrow-derived mesenchymal stem cells (BM-MSCs) reduced myocardial infarctions and improved cardiac function and angiogenesis through intramyocardial transplantation in rat models of ischaemic cardiomyopathy. According to a case report issued in 2010 indicated that intracoronary administration of autologous MSCs in an 11 year old boy with dilated cardiomyopathy and class IV heart failure (HF) was safe and also improved the boy’s clinical condition. Afterwards, the patient’s functional class changed from IV HF to III HF and II HF, the paroxysmal nocturnal dyspnea disappeared, appetite improved and the need for hospitalization was also decrease. According to a study conducted by Butler et al, Patients were randomized to intravenously administered ischemia-tolerant MSCs (itMSCs) (1.5×106 cells/kg) or placebo; at 90 days, each group received the alternative treatment. Treatment with itMSCs resulted in statistically significant amelioration in functional capacity and health status end points. When the change from baseline to 90 days was compared after itMSC therapy with the change during the control period, six minute walk distance (6MWD) was remarkably greater by 36.47 m. Specifically, within the itMSC group, 6MWD increased by an average of 27.40 m, but it decreased by an average of 10.83 m among control patients. Additionally, Zeinaloo et al, inject autologous bone marrow mesenchymal stem cells into the left and right coronary arteries of 11-year-old boy with a diagnosis of dilated cardiomyopathy. The 1 year clinical check-up indicated an improvement of the left ventricular ejection fraction from 20 to 42 percent. In conclusion, several studies describes the role of mesenchymal stem cells for cardiomyopathy which demonstrated that stem cell therapy can be a suitable approach to treat pediatric heart failure via facilitating cardiac regeneration and increasing cardiac function.
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