Term
| How do the major HF etiologies differ in the adult and pediatric populations? |
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Definition
Ischemic causes dominates in adults, while non-ischemic dominates children.
1) Adult
- Ischemic (CAD)
- Non-Ischemic (HTN, Valvular disease, DIlated CM, HCM, Myocarditis)
2) Pediatric
- Non-ischemic (Congenital, Dilated CM, Myocarditis, HCM)
- Ischemic (Kawasaki and CA anomalies) |
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Term
| How do primary etiologies of pediatric HF change by age? |
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Definition
1) <1 year, high proportion of patients die from congenital defects
2) Increasing age, there is higher percentage with end-stage HF from cardiomyopathy who receive transplant. |
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Term
| What are the most common congenital defects that cause HF? |
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Definition
1) Obstructive left heart lesions
- Coarctation
- AS
- Hypoplastic left heart
2) Left to right shunts
- VSD
- PDA
- ASD (rarely cause HF)
3) Coronary anomalies
- Anomalous left CA from pulmonary artery (ALCAPA) |
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Term
| How does coarctation of the aorta develop and how do you treat it? |
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Definition
Most common congenital left heart obstruction
1) 3-10 days after birth, coincident with DA closure, "posterior shelf" of tissue narros lumen of aorta, diminishing flow distally.
- Hypo-perfusion to kidneys, gut and lower extremities
- Increased after-load causes LV function to decline, LV dilation and mitral regurgitation.
2) Treat acutely with Prostaglandin E1 to open ductus and relax aortic isthmus.
- Eventually remove affected tissue and re-anastomose aorta. |
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Term
| What form of obstructive left heart disease is associated with bicuspid aortic valve (BAV) and how is it treated? |
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Definition
| Congenital Aortic stenosis treated with balloon aortic valvuloplasty (sometimes valve replacement). |
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Term
A healthy newborn with poor feeding and difficulty breathing presents with tachycardia, hypotension, hypoglycemia, hypothermia, poor perfusion and lethargy.
What should you do? |
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Definition
This sounds like Aortic atresia or "Hypoplastic Left heart syndrome" (no aortic valve), which SHOULD have been recognized in utero with ultrasound.
Treat pre-operatively with PGE1 to create PDA so RV can feed systemic circulation.
Surgery is then done to direct RV outflow to augmented ascending aorta with blood flow to pulmonary circulation provided by systemic arterial to pulmonary arterial shunt. |
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Term
| How might HF from a VSD manifest? |
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Definition
Left to right shunt
** Not all present with HF symptoms, it depends upon RESISTANCE**
Large VSD (relative to aortic valve annulus) will have lower resistance, so more blood will enter RV and the lungs thereafter causing Pulmonary HTN.
1) Tachypnea (LV has to work harder because it is losing blood to RV)
2) Diaphoresis/dyspnea with feeds (Pulmonary edema makes it harder to breathe)
3) Cachexia (If you don't eat, you lose weight) |
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Term
| What factors determine the magnitude of a VSD shunt? |
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Definition
Size of hole and downstream resistance.
1) Big holes have lower resistance and more blood goes from LV to RV (and thus to lungs).
2) Pulmonary vascular resistance is initially greater than systemic vascular resistance, but then it drops. As PVR drops, more blood flows into lungs, causing edema.
** This is the same for a PDA ** |
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Term
| What happens to flow through a PDA as PVR drops in the first weeks of life? |
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Definition
| Less PVR means more blood will flow from aorta to pulmonary artery (left to right). |
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Term
| What factors determine flow across an ASD? |
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Definition
Unlike VSD/PDA, flow across an ASD is governed by resistance/compliance of the RA and LA, and the size of the defect.
1) LV is more muscular, so flow goes from less compliant to more compliant (LA to RA/RV)
**tend to be asymptomatic** |
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Term
| What is the physiology behind an Anomolous Left Coronary Artery from the Pulmonary Artery? How do you treat? |
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Definition
1) When pulmonary resistance drops after birth, blood PREFERS to go to the pulmonary capillary beds, rather then through the left coronary artery to the left ventricular myocardium.
2) Collateral circulation from RCA is not enough, and ischemia and cardiomyopathy occurs with decreases LV systolic function.
**look for bright papillary muscles and MR**
3) Surgical removal and replacement of LCA to aorta (improve over 6-12 months) |
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Term
| What are 2 major causes of pediatric Tachycardia and Bradycardia? |
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Definition
Tachycardia (SERIOUS because it can be long-lasting)
1) Supraventricular
2) Ventricular
Bradycardia (SERIOUS because infants have poor compliance and small ventricles, so they can't up SV)
**need a pacemaker**
1) Heart block
2) Sick sinus |
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Term
When do you use each of the following surgical techniques in infants/young children?
1) Primary "corrective"
2) Palliative
3) Interventional catheterization (Balloon valvuloplasty)
4) Mechanical support (VAD) & Transplant |
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Definition
1) VSD, PDA, ASD, Coarctation
2) Aortic atresia
3) Aortic or Pulmonic stenosis
4) Refractory HF |
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Term
True or False:
There are good treatment options for diastolic HF in children, but not for systolic HF |
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Definition
FALSE!
Opposite is true- Systolic (RAAS & SNS) > Diastolic |
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Term
| What therapeutic recommendations for treatment of pediatric HF are supported by at least a single randomized trial, with general agreement in the community? |
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Definition
Class 1, Level B
Systolic treatment is better than diastolic
1) Digoxin for symptomatic HF
2) Digoxin for mod-severe LV dysfunction
3) Diuretics for volume overload |
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