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Cardiomyopathy

Cardiomyopathy. Myra Lalas Pitt. Hypertrophic Cardiomyopathy. Cardiomyopathy with hypertrophy of the LV (± RV) with histological features of myocyte hypertrophy, myofibrillar disarray, and interstitial fibrosis. The prevalence in young adults is approximately one in 500.

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Cardiomyopathy

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  1. Cardiomyopathy Myra Lalas Pitt

  2. Hypertrophic Cardiomyopathy • Cardiomyopathy with hypertrophy of the LV (± RV) with histological features of myocyte hypertrophy, myofibrillar disarray, and interstitial fibrosis. • The prevalence in young adults is approximately one in 500. • Frequent cause of sudden death, particularly in young individuals and competitive athletes. • Usually AD- the genes affected code for proteins in the sarcomere • Associated with Pompe Disease

  3. Signs and Symptoms • Systolic function is preserved; may have increased contractility; symptoms are typically caused by diastolic dysfunction (impaired ventricular relaxation) and sometimes LVOT obstruction. • May be asymptomatic • Palpitationss, dizziness, syncope, sudden death. • Age of symptom onset: from birth to middle age

  4. Treatment • Beta-blockers : mainstays of therapy. • Combination therapy (Beta-blocker and CCB) may be considered in patients with severe hypertrophy and severe outflow obstruction. • LVOTO myectomy: Children with severe LVOTO.

  5. Dilated Cardiomyopathy • Cardiomyopathy with increased ventricular size and reduced contractility in the absence of coronary, valvar, or pericardial disease. • Highest rate of disease in newborns and infants. • M> F • 1-yr mortality is about 50% without cardiac transplantation. • Genetics: 1/3 have familial form, AD (most common is cytoskeletal protein involvement), X-linked (typically occurs in boys with rapid progression; elevated CK-MM)

  6. Signs and Symptoms • Shortness of breath, fatigue, orthopnea, diaphoresis, chest pain, palpitations, exercise intolerance, syncope. • Tachypnea, tachycardia, diaphoresis, S3 or S4 gallop, holosystolic murmur (MR), hepatomegaly, ± ascites or peripheral edema.

  7. Treatment Outpatient Management ACE inhibitors and beta-blockers (metoprolol/carvedilol): Mainstays of therapy Diuretics: May be useful for symptomatic edema; no proven long-term benefit Digoxin: Now used less often, but may improve symptoms in some patients Inpatient Management Inotropic medications avoided whenever possible; reserved for compromised hemodynamic states (milrinone ± low-dose dopamine) Diuretics: Decrease pulmonary edema acutely; may improve preload in volume overload states Mechanical circulatory support: Ventricular assist devices and ECMO for those who fail medical management as a bridge to recovery or transplantation Cardiac transplantation

  8. Restrictive Cardiomyopathy • Definition: Cardiomyopathy with abnormal stiffness of the ventricular wall leading to impaired ventricular filling. • Rare type of cardiomyopathy • Has the worst outcome of all forms of cardiomyopathy (especially in children) • Sudden death occurs within 2 to 5 years of diagnosis in more than 50% of patients. • Genetics: inherited in <10% of patients, usually AD

  9. Signs and Symptoms • CHF (dyspnea, orthopnea, and abdominal pain), syncope, or sudden death. • S4; holosystolic murmur of AV valve insufficiency if the patient has pulmonary hypertension, hepatomegaly.

  10. Treatment • Heart transplantation is the only definitive therapy and is often recommended as primary therapy. • May use antiarhythmics. • In general, meds may cause acute hemodynamic changes and are usually not effective.

  11. Glycogen Storage Disease • Inheritance: AR (except some forms of type VI and IX X-linked). • Clinical presentation: Pathologic glycogen storage (eg, hepatomegaly) or organ dysfunction (eg, muscle weakness, muscular atrophy, cardiomyopathy, renal tubulopathy) or hypoglycemia.

  12. PREP Question • You are evaluating a 12-year-old boy who has been fatigued for 2 weeks. His mother reports that he had an upper respiratory tract infection 2 weeks ago, and his appetite has been decreased since then. On physical examination, he is afebrile and has a heart rate at rest of 110 beats/min. His respiratory rate is 22 breaths/min. His lungs are clear, and he has a gallop rhythm without murmurs on cardiac auscultation. You discern hepatomegaly and mild jugular venous distention.Of the following, the MOST likely diagnosis isa. anemia b. dilated cardiomyopathy c. Kawasaki disease d. primary pulmonary hypertension e. pulmonary embolism

  13. Answer: B • The gallop rhythm, hepatomegaly, and jugular venous distention described for the boy in the vignette support the diagnosis of congestive heart failure (CHF), most likely due to myocardial dysfunction associated with dilated cardiomyopathy. • Laboratory support for the myocardial failure seen in patients who have CHF can be demonstrated by an elevation in the brain natriuretic peptide value. Results of this test almost always are abnormal in patients who have significant CHF. • Among the many causes of CHF are large-volume left-to-right shunts with pulmonary overcirculation, pressure load on the myocardium, inadequate blood flow to the myocardium, infection or infiltration of the myocardium, or genetic or idiopathic diseases of the myocardium.

  14. References Lowry AW, Bhakta KY, Nag PK, "Chapter 8. Cardiology" (Chapter). Lowry AW, Bhakta KY, Nag PK: Texas Children's Hospital Handbook of Pediatrics and Neonatology:http://www.accesspediatrics.com/content/7434509. Lowry AW, Bhakta KY, Nag PK, "Chapter 17. Genetics" (Chapter). Lowry AW, Bhakta KY, Nag PK: Texas Children's Hospital Handbook of Pediatrics and Neonatology:http://www.accesspediatrics.com/content/7439230. www.uptodate.com

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