PEDS Mod 4

Question 1

This is a chronic disease of the heart muscle that affects the heart’s ability to pump blood.

It is a leading cause of sudden cardiac arrest in young people and can affect any child.

It remains the leading cause of heart transplants in children older than 1 year of age.

There is no cure for the disease.

It can be inherited or acquired.

Answer 1

Cardiomyopathy

Question 2

Enlargement of the left or both ventricles; Most common

Decreased contractility leading to inadequate cardiac output leading to heart failure (systolic).

Answer 2

Dilated cardiomyopathy

Question 3

What are the most common causes of dilated cardiomyopathy?

Answer 3

Usually idiopathic (70%)

Others are viral infection, med toxicity (eg, anthracycline), metabolic dz (eg, inborn errors of fatty acid oxidation and mitochondrial oxidative phosphorylation defects).

Question 4

What are some key s/sx of Dilated cardiomyopathy?

Answer 4

• Mimic of respiratory infection, pneumonia, or asthma.
• Decreased exercise tolerance (feeding in infants), malnutrition, diaphoresis, tachypnea, tachycardia
• Congestive signs: Hepatomegaly, rales, weak peripheral pulses, prominent gallop (S3)

Question 5

What are some key diagnostic test findings for dilated cardiomyopathy?

Answer 5

• Chest x-ray: Cardiomegaly
• EKG: Sinus tachycardia, ST-T wave changes (ST depression, T wave negativity) and left ventricular hypertrophy (left and right if bilateral)- hypertrophic pattern even if no hypertrophy present- ie QT prolonged, ST changes, Left axis deviation
• Echo: LV and LA enlargement, decreased contractility (lesions, structural abnormalities?)

Question 6

What are some outpatient treatment strategies for dilated cardiomyopathy?

Answer 6

Goal: Improve cardiac performance (preloaded, afterload contractility)

• Afterload reducing agents (Ace inhibitor-captopril, enalapril, lisinopril)-block systemic vascular resistance
• Diuretic (furosemide, thiazides, spironolactone) -control symptoms related to congestion
• +- Beta blocker (not as beneficial as in adults)
• +- aspirin, warfarin to prevent thrombus formation
• +- Anti-arrhythmia (arrhythmia common in dilated hearts)
• Treat underlying cause if found
• Close follow-up

Question 7

What are some inpatient treatment strategies for dilated cardiomyopathy?

Answer 7

• Afterload reducing agents (milrinone, nitroglycerin)
• Enhance contractility (dopamine, dobutamine)
• ECMO, ventricular assist device
• Cardiac transplantation if medical management failure

Question 8

What is the prognosis for patients with dilated cardiomyopathies?

Answer 8

Usually poor unless transplant the, then prognosis is good.

Question 9

This cardiomyopathy is diastolic, usually left ventricle not able to fill. After “burn-out” will also be systolic failure.

Answer 9

Hypertrophic cardiomyopathy

Question 10

Hypertrophic cardiomyopathy is most commonly due to what?

Answer 10

• Most common- familial
• Metabolic, mitochondrial, Freidreich’s ataxia, certain syndromes (Noonan)

(UTD: Hypertrophic cardiomyopathy in children: Clinical manifestations and diagnosis)

Question 11

This cardiomyopathy is the leading cause of sudden cardiac death in young patients. The MC presentation is in an older child, adolescent, or adult, and can occur in neonates.

Answer 11

Hypertrophic cardiomyopathy

Question 12

S/Sx:

• Obstructive vs Non-obstructive (less severe)
• Sx related to 1) HF, 2) chest pain, 3) arrhythmia (SV and Ventricular)
• Infants (<1 yr): S/Sx of heart failure
• Older children (>1 yr): often Asx unless inborn errors of metabolism
• *Sudden death
• Asymptomatic, Fatigue, Dyspnea, Chest pain, Palpitations, Presyncope or syncope
• Tachypnea, poor feeding, and poor growth
• Peripheral edema, parasternal lift, gallop
• Murmur
  
  • harsh crescendo-decrescendo systolic murmur; apex and lower left sternal border; may radiate to the axilla and base (LVOT-Left Vent outflow tract)
  • OR holosystolic murmur heard loudest at the apex that radiates to the axilla (classic MV regurg murmur)

Answer 12

Hypertrophic cardiomyopathy

Question 13

How is hypertrophic cardiomyopathy diagnosed?

Answer 13

• EKG (abnormalities seen but not dx)
• Echo (cardiac morphology, Systolic/diastolic fxn, regurgitation) - Asymmetrical septal hypertrophy, Diastolic function is almost always abnormal.
• +- Holter, Exercise test, heart MRI, cath, biopsy
• Genetic Testing and testing of 1^st degree relatives

(UTD: Hypertrophic cardiomyopathy in children: Management and prognosis)

Question 14

What is the treatment of hypertrophic cardiomyopathy?

Answer 14

TX:

• Relief of symptoms and life-saving therapy (implants); Tx does not stop progression of disease
• Decrease LVOT obstruction, O2 demand and HR (which improve LV filling)
  
  • Beta Blockers 1^st line(increases survival rates); CCB (ie verapamil) 2^nd line
  • Combo for more severe
  • Surgery: myomectomy; transplant
• Avoid dehydration
• Avoid medications that make Sx worse (vasodilators, diuretics, digoxin)
• Exercise restrictions
• Close f/u with EKG/Echo