Week 2: Part 2 (Pediatrics) Flashcards
slides 40-60
Aortic stenosis & pulmonary stenosis are examples of
Obstructive Defects
Pulmonary stenosis
Narrowing of the pulmonary outflow tract
Abnormal thickening of the valve leaflets
Narrowing of the valve
Severe form of Pulmonary stenosis
Pulmonary atresia
Pulmonary atresia
Clinical manifestations
Often asymptomatic
Exertional dyspnea
murmur
fatigue
Pulmonary stenosis
Treatment
(mild vs severe)
Mild: Not treated, closely observed
Severe: Balloon angioplasty; pulmonary valvotomy
(Obstructive Defects)
Hypoplastic left heart syndrome
Left-sided cardiac structures develop abnormally
-Obstruction blood flow from the left ventricular outflow tract
-LV, aorta, aortic arch underdeveloped
-mitral atresia/stenosis
As the ductus closes, systemic perfusion is decreased, resulting in hypoxemia, acidosis, and shock.
T/F
In Hypoplastic left heart syndrome, perfusion actually worsens as the ductus closes.
True
“As the ductus closes, systemic perfusion is decreased, resulting in hypoxemia, acidosis, and shock.”
Hypoplastic left heart syndrome
Treatment (6)
-Prostaglandin administration
-Correction of acidosis
-Inotropes for adequate CO
-Ventilatory manipulation
-Surgical intervention three-stage approach
-Cardiac transplantation
Hypoplastic left heart syndrome
Surgical intervention 3-stage approach
Norwood procedure: Atrial septectomy, pulmonary-to-systemic artery shunt, permanent communication from RV & aorta; patch the hypoplastic aorta.
Glenn procedure: (2 - 9 months) SVC is joined to the pulmonary artery; takedown of the shunt to the lungs
Fontan procedure: (2-4 years) separates systemic from the pulmonary circulation
Mixed Defects
Transposition of the great arteries
Aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle.
Transposition of the great arteries
Results in …..
two separate, parallel circuits
Unoxygenated blood continuously circulates through the systemic circulation.
Oxygenated blood continuously circulates through the pulmonary circulation.
Transposition of the great arteries
Extrauterine survival
requires communication between the two , separate, parallel circuits
Transposition of the great arteries
Clinical manifestations & treatment
Cyanosis may be mild shortly after birth and worsen during the first day.
Treatment: Surgery to switch the arteries
(Mixed defects)
Total anomalous pulmonary venous connection (TAPVC)
Pulmonary veins connect to the right side of the heart, directly or indirectly, through one or more systemic veins that drain into the right atrium.
-Nonobstructive vs. obstructive
Total anomalous pulmonary venous connection (TAPVC)
-Clinical manifestation
-Treatment
Cyanosis
-Obstructed lesions repaired upon diagnosis
-Unobstructed lesions usually repaired in infancy
-Surgery: Anastomosis of the common pulmonary vein to the left atrium; closure of the atrial septal defect.
Truncus arteriosus
Types I through IV
I: (60%); main pulmonary artery arises from the truncus.
II: 20%; pulmonary arteries arise from the posterior aspect of the truncus.
III: 10%; pulmonary arteries arise from the lateral aspect of the truncus.
IV: Pseudotruncus; is a severe form of tetralogy of Fallot with the bronchial arteries arising from the descending aorta to supply the lungs.
Truncus arteriosus
-S/S
-Tx
mild-to-moderate cyanosis; worse with activity
Tx:
-Modified Rastelli + VSD patch closure:
divert blood flow left ventricle outflow tract→truncus
-Correct pulmonary arteries
Kawasaki disease
mucocutaneous lymph node syndrome
-acute, self-limiting systemic vasculitis
-may result in cardiac sequelae
-80% cases = children < 5Y
Kawasaki disease
Diagnosis (need 5 out of the 6 total)
-Fever 5+ days; unresponsive to antibiotics
-Bilateral conjunctivitis w/o exudate
-Erythema of oral mucosa (strawberry tongue)
-Polymorphous rash
-Cervical lymphadenopathy
-Changes in the extremities
(peripheral edema,erythema, desquamation of palms and soles)
