Pathophysiology
The four primary defects present in Tetralogy of Fallot.
ventricular septal defect, overriding aorta, pulmonary stenosis and right ventricular hypertrophy
Is the reason why our patient has a sternal scar
open heart surgery (large incision and a heart-lung bypass)
Is the method of treatment with better prognosis
TPVR resulted in lower mortality and fewer major adverse events than SPVR
(SPVR is better if there are required concomitant surgical procedures)
Are 3 disadvantages of open heart surgery
1. longer stay in hospital
2. Long recovery
3. Invasive
Is the X-ray sign of ToF
Boot shaped heart
This is why Tetralogy of Fallot causes cyanosis
Stenosis of conus arteriosus (pulmonary valve) --> obstruction of RVOT --> RV hypertrophy --> De-O2 blood is shunted right to left
Is the gold standard for initial treatment of ToF
Open heart surgery (valve lasts more than 20+ years)
TPVR is usually done later on in life for repair
Is the cheaper option out of TPVR and open heart surgery in the long run
Open heart surgery
"despite longer LOS and greater in-hospital resource utilization for patients with TOF who underwent S-PVR compared with TC-PVR, mortality, and in-hospital costs are the same, and projected 5-year costs are less "
Are 3 complications associated with TPVR
1. Endocarditis
2. Valve failure
3. Need for reoperation/TPVR within 10 yrs
Is the reason why Raymond had a single S2 sound
No pulmonic valve closing (only aorta)
This causes the formation of an overriding aorta in ToF
Conus arteriosus moves towards lateral RV deviating the aorta rightward
Is the goal of any type of ToF treatment
Pulmonary valve replacement + VSD repair
Is the frequency of patients with ToF that made it to their 10 year birthday without treatment
33%
Is why patients with corrected ToF have to be monitored for life
Inevitable heart failure due to valve deterioration
Is the direction of blood movement on echo
RV --> LV --> aorta
Is the reason Raymond had high hematocrit and hemoglobin as an infant
hypoxia
Is the pathway of the TPVR performed on Raymond to reach his heart
Femoral vein --> external iliac --> common iliac --> inferior vena cava --> RA --> RV --> pulmonary valve
Is the 25-year survival rate after having had surgical intervention performed
94.5%
Free Points :)
:)
Is the location of the harsh systolic murmur heard in ToF
tricuspid area/LUSB
How does squatting affect ToF
squatting increases afterload by increasing vascular resistance --> increase pressure in LV --> less right to left shunting --> symptoms improve
Is a medication given for short-term relief of ToF symptoms in neonates
Alprostadil (PGE1 analog)
Maintains a PDA and allows shunting of blood from aorta to pulmonary arteries
Is the 5-year post-surgical survival rate if ToF is coupled with chromosomal abnormality
<85% (worse prognosis)
ToF is associated with 22q11 syndromes
Is a complication associated with either type of tx and requires re-intervention (hint: it happened to Raymond)
right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia
Is the type of VSD murmur heard in tetralogy of fallot
None
VSD is very large in ToF and murmur is inversely related to the defect's size