S3 EXAM 2 CONGENITAL HEART DISEASE

Question 1

Overview & Etiology

Answer 1

Congenital Heart defect
Heart or great vessel defect from abnormal fetal development or fetal structure that does not convert to extrauterine anatomy after birth
8 weeks gestation
Results from genetic & environmental factors
Fetal exposure to drugs
Maternal complications
Genetic factors and Chromosomal abnormalities
Unknown

Question 2

Risk Factors & Prevention

Answer 2

Risk Factors
Medication
Maternal DM/rubella
Alcohol & smoking during pregnancy

Prevention
Vaccinations
Management of chronic conditions
Avoid harmful substances
Daily multivitamin (400 mg folic acid)

*Echocardiogram

Question 3

Pathophysiology: Increased Pulmonary Blood Flow

Answer 3

Blood allowed to flow between left and right side of the heart
More blood pumped into lungs
Leads to pulmonary HTN
Left pressure greater than right
Blood shunted left to right
Right ventricle hypertrophies (compensation)

Question 4

Atrial Septal Defect

Answer 4

Clinical Manifestations
Infants/young have no symptoms
Heart murmur
Chest X-ray or ECG
Pulmonary arterial HTN
Atrial dysrhythmias
Exercise intolerance
CHF

Treatment
Spontaneous closure
Surgery

Question 5

Ventricular Septal Defect

Answer 5

Clinical Manifestations
Depend on size/degree of shunt
Murmur
Excessive sweating & Fatigue with feeds
Lack of growth
Frequent respiratory infections
CHF
Tachypnea with exercise

Treatment
Spontaneously close within 6 months of life
Conservative
Transcatheter closure for some defects

Question 6

Patent Ductus Arteriosus

Answer 6

Clinical Manifestations
Dyspnea
Tachycardia
Tachypnea
Full-bounding pulse
CHF
Intercostal Retractions
“Machinery” murmur

Treatment
IV indomethacin
Transcatheter occlusion

Question 7

Atrioventricular (AV) Canal (Endocardial Cushion Defect)

Answer 7

Clinical Manifestations
CHF
Tachypnea
Tachycardia
Failure to gain weight/grow
Frequent pneumonia
Pallor
Sweating
Cyanosis
Holosystolic murmur

Treatment
Surgery
Palliative pulmonary artery banding
Patches

Question 8

Clinical Manifestations: Defects that Increase Pulmonary Blood Flow

Answer 8

Increased HR
Tachypnea
Intercostal retractions
Increased metabolic rate
Periorbital edema (in CHF)
Diaphoresis when feeding
Respiratory infections
Poor weight gain
Dyspnea

Question 9

Pathophysiology: Decreased Pulmonary Blood Flow

Answer 9

Right pressure higher than left
Polycythemia
Hypercyanotic episodes (Tet spells)

Question 10

Pulmonary Stenosis

Answer 10

Noonan Syndrome

Clinical Manifestations
Dyspnea
Fatigue
Pulmonic ejection click (mild to moderate stenosis)

Treatment
Dilation by balloon valvuloplasty
Surgical resection

Question 11

Tetralogy of Fallot

Answer 11

Four Defects
Pulmonary Stenosis
Right Ventricular Hypertrophy
Ventricular Septal Defect
Overriding Aorta
Open foramen ovale or ASD

Clinical Manifestations
Hypoxia and cyanosis
Systolic murmur
Polycythemia
Metabolic acidosis
Poor growth
Clubbing of fingers
Exercise intolerance

Treatment
Manage hypercyanotic episodes
Total repair

Question 12

Clinical Manifestations: Hypercyaonotic Episodes

Answer 12

Increased RR and depth - Diaphoresis
- Increased HR - Irritable/crying
- Cyanosis - Seizures
Pallor - Loss of consciousness
Dizziness - Syncope