Cyanotic Heart Disease - Dolphens Flashcards Preview

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Flashcards in Cyanotic Heart Disease - Dolphens Deck (10):
1

Mild Congenital Heart Disease

 

Describe the physiologic categories of congenital heart disease and provide examples of each type.

Small Ventricular Septal Defect (VSD)

Small Patent Ductus Arteriosus (PDA)

Mild Pulmonary Stenosis (PS)

Bicuspid Aortic Valve (BAV) w/o AS or AI

Small Atrial Septal Defect (ASD)

2

Moderate Congenital Heart Disease

 

Describe the physiologic categories of congenital heart disease and provide examples of each type.

Aortic Valve Disease: mild or moderate stenosis or insufficiency

Moderate Pulmonary Stenosis

Non-Critical Coarctation

Large ASD

Complex VSD: membranous, perimembranous, non-pressure restrictive, etiology of pulmonary HTN…

3

Severe Congenital Heart Disease: CYANOTIC

 

Describe the physiologic categories of congenital heart disease and provide examples of each type.

Tetralogy of Fallot (TOF)

Transposition of Great Arteries (TGA)

Hypoplastic Right Heart: Tricuspid Atresia, Pulmonary Atresia – IVS, Ebstein’s Anomaly

Hypoplastic Left Heart: Aortic Atresia, Mitral Atresia

Single Ventricle

Double Outlet Right Ventricle (DORV)

Truncus Arteriosus

Total Anomalous Pulmonary Venous Return (TAPVR)

Critical Pulmonary Stenosis

4

Severe Congenital Heart Disease: ACYANOTIC

 

Describe the physiologic categories of congenital heart disease and provide examples of each type.

Atrio-Ventricular Septal Defect

Large VSD

Large PDA

Critical/Severe Aortic Stenosis

Severe Pulmonary Stenosis

Critical Coarctation

5

Tetraology of Fallot

 

Describe the fetal and post-natal circulation systems and the anatomic defects associated with following disorders:  Tetralogy of Fallot

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  1. Large, mal-aligned VSD
  2. Overriding aorta
  3. RVOT obstruction/pulmonary atresia
  4. RVH

most common cyanotic CHD lesion

associated with genetic syndromes: Trisomy 21, 18, 13; DiGeorge's, 22q11 deletion, Algille's syndrome

intermittent spells of extreme cyanosis: Hypercyanotic Spell

Chest X-Ray: boot shaped heart, small MPA, upturned apex

Treatment:

  • Medical: PGE1 to maintain ductus
  • Surgical: systemic-to-pulmonary shunt

Risks:

  • RV failure
  • Arrhythmia
  • prolonged chest tube drain
  • long term risk: sudden death

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6

Transposition of Great Arteries

 

   Describe the fetal and post-natal circulation systems and the anatomic defects associated with following disorders: Transposition

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D-Transposition: aorta arises from RV, pulmonary artery arises from LV
 
L-Transposition: congenitally corrected transposition, ventricular inversion, may remain asymptomatic
 
Diagnostics:
  • cyanosis without murmur
  • EKG: RAD
  • CXR: egg on string

Treatment

  • Palliative: PGE1, Ballon atrial septostomy
  • Surgical: Mustard/Senning (left - used in L-TGA) Arterial Switch operation (right - used in D-TGA)

Complications

  • prolonged bypass
  • myocardial dysfunction
  • mobilization of coronary arteries
  • risk of aortic insufficiency and ventricular arrhythmias

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7

Truncus Arteriosus

 

Describe the fetal and post-natal circulation systems and the anatomic defects associated with following disorders: Truncus Arteriosus.

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Single, arterial trunk arsing from the heart; large VSD below truncal valve

Murmur: occasionally systolic ejection click, singl loud S2

Signs and Symptoms: tachypnea, diaphoresis, cough

Treatment

  • Oxygen therapy (75-85% oxygen)
  • Complete repair
  • complications due to truncal valve

8

Coarctation of the Aorta

 

Describe the fetal and post-natal circulation systems and the anatomic defects associated with following disorders:  Coarctation

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Narrowing of the lumen of the aorta - shelf like protrusion in the juxtaductal region

often associated with Bicuspid Aortic Valve

males > females

How is it found?:

  • acute decompensation: CHF, poor distal organ perfusion
  • Hypertension: >10 mmHg gradient from upper to lower extremity
  • Murmur - continuous
  • Echo - maybe inconclusive

Complications

  • shock, acidosis
  • poor perfusion = neurologic injury, myocardial infarction
  • LV dysfunction

Treatment:

  • medical: PGE1
  • interventional: balloon aortoplasty, stent placement
  • surgical: end-to-end repair, left subclavian flap repair, subclavian translocation, patch angioplasty
    • post-op concerns:
      • systemic HTN
      • rebound HTN
      • recoarctation

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9

Hypoplastic Left Heart Syndrome

 

Describe the fetal and post-natal circulation systems and the anatomic defects associated with following disorders: Hypoplastic left heart syndrome.

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Spectrum of abnormalities - fatal within first life if no intervention

Systemic blood flow is dependent on PDA at ASD

Signs and Symptoms:

  • Cyanosis
  • Respiratory Distress - tachypnea and dyspnea
  • Hypothermic
  • poorly perfused systolic pressures < 40 mmHg
  • metabolic acidosis, hypoglycemia, hyperkalemia
  • soft, systolic ejection murmur

Treatment:

  • PGE1
  • Balloon Atrial Septostomy
  • Norwood Procedure
    • Stage 1: BT Shunt v. Sano Shunt
    • Stage 2: Bidirectional Glenn
    • Stage 3: Fontan Completion

Complications

  • AV valve dysfunction
  • Myocardial dysfunction
  • Transplant may be required

10

Goals for Treating Congenital Heart Disease

  1. Feed and Grow
  2. Protect the Lungs
  3. Perfuse the rest of the body