Congenital heart disease 2 Flashcards
During a surgical repair of TOF, the patient’s blood pressure declines by 25%, and the SpO2 decreases by 10%. What are the MOST likely explanations for these findings? (select 2)
a. pulmonary vascular resistance decreased
b. systemic vascular resistance decreased
c. myocardial contractility increased
d. preload increased
b. systemic vascular resistance decreased
c. myocardial contractility increased
Tetralogy of Fallot is characterized by the following four defects:
ventricular septal defect
aorta that overrides the RV and LV
pulmonic stenosis (obstruction to RV ejection)
RV hypertrophy
What is the most common cyanotic congenital heart anomaly?
Tetralogy of Fallot
A “tet spell” presents as
hypoxemia and cyanosis
With TOF, the best induction agent is
ketamine
With TOF< the drugs to avoid include
morphine, meperidine, and atracurium (histamine release)
A “tet spell” is precipitated by
increased sympathetic activity such as crying, agitation, pain, defecation, fright or trauma
Why might the child experiencing a tet spell assume the squatting position?
it increases intraabdominal pressure and compresses the abdominal arteries which increases RV preload, SVR, and blood flow through the RVOT
Perioperative Tet spell treatment includes
FiO2 100%
fluids to expand intravascular volume
increase SVR with phenylephrine
Reduce SNS stimulation- deepen anesthesia, short-acting beta-blocker
avoid inotropes- can worsen RVOT obstruction
avoid excessive airway pressure
place the infant in a knee-chest position to mimic squatting
What are the hemodynamic goals of tetralogy of Fallot:
increase SVR- phenylephrine
decrease PVR- nitric oxide
maintain contractility and heart rate- esmolol
increase preload- crystalloid
Why are some patients with tetralogy of Fallot polycythemic?
chronic hypoxemia stimulates increased RBC production
Failure of the fossa ovalis to close results in what type of atrial septal defect?
a. primum
b. secundum
c. sinus venosus
d. perimembranous
b. secundum
The most common type of ventricular septal defect is the
perimembranous VSD
The most common congenital cardiac anomaly in children is
a ventricular septal defect
The most common site of atrial septal defect is
fossa ovalis
Flow through an atrial septal defect is typically
left-to-right (acyanotic)
The most common site of ventricular septal defect is the
ventricular septum
Flow through the VSD is typically
left-to-right (acyanotic)
What situations should be avoided in a VSD?
situations that decrease PVR or increase SVR b/c they can increase shunt flow
The physiologic consequence of the VSD is a function of_______________
the pressure gradients between the RV and LV which depend on PVR and SVR
Early signs of an ASD include
poor exercise tolerance
Late signs of an ASD include
atrial flutter
atrial fibrillation
CHF
______________ can cause paradoxical embolism during Valsalva-like maneuvers if ____________
ASDs; RAP>LAP
Describe antibiotic prophylaxis with ASD & VSD?
not indicated for an isolated ASD but it’s indicated within six months of surgical repair
VSD is associated with the following conditions:
trisomy 13, 18, and 21
VACTERL
CHARGE
The primary concern of a VSD is the
development of a left-to-right shunt
VSD can cause ________ during valsalva-like maneuvers if RAP>LAP
paradoxical embolism
Most VSDs close by the age of:
2
In adults, the most common congenital defect is
bicuspid aortic valve
A patient is undergoing surgical repair for coarctation of the aorta. Select the BEST site to monitor the arterial blood pressure.
a. right arm
b. right leg
c. left arm
d. left leg
a. right arm
Coarctation of the aorta is the
narrowing of the thoracic aortic lumen
Where can coarctation of the aorta occur?
typically before or after the ductus arteriosus but in rare instances, proximal to the left subclavian artery
______________ usually goes unnoticed for years
Mild to moderate coarctation
Obstruction of blood flow at the level of the coarctation leads to
increased LV afterload
Describe systolic blood pressure in coarctation of the aorta.
SBP is elevated in the upper extremities
SBP is reduced in the lower exprtemities
Severe obstruction of the aorta presents
very early in life
Severe obstruction of coarctation of the aorta
requires prostaglandin E1 to keep the PDA open until surgery can be performed because it relies on a patent ductus arteriosus
_____________ is strongly associated with coarctation of the aorta
Turner syndrome
If the coarctation occurs proximal to the left subclavian artery takeoff, then the
SBP in the RUE> then the SBP in the LUE
Severe coarctation of the aorta in the neonate can present as
pink, well-perfused upper body and blue poorly-perfused lower body
Indications for surgical repair in the patient with mild to moderate coarctation of the aorta includes
exercise intolerance
chest pain
headaches
lower extremity claudication
Diagnosis of mild to moderate coarctation of the aorta may be delayed until adulthood when the patient presents with
secondary hypertension
Preductal coarctation is commonly found in
infants
Postductal coarctation is commonly found in
adults