Cyanotic Heart Disease - Dolphens

Question 1

Mild Congenital Heart Disease

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

Answer 1

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)

Question 2

Moderate Congenital Heart Disease

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

Answer 2

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…

Question 3

Severe Congenital Heart Disease: CYANOTIC

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

Answer 3

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

Question 4

Severe Congenital Heart Disease: ACYANOTIC

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

Answer 4

Atrio-Ventricular Septal Defect

Large VSD

Large PDA

Critical/Severe Aortic Stenosis

Severe Pulmonary Stenosis

Critical Coarctation

Question 5

Tetraology of Fallot

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

Answer 5

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

Question 6

Transposition of Great Arteries

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

Answer 6

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

Question 7

Truncus Arteriosus

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

Answer 7

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

Question 8

Coarctation of the Aorta

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

Answer 8

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

Question 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.

Answer 9

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

Question 10

Goals for Treating Congenital Heart Disease

Answer 10

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