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What non-coronary embryonic structure is often persistent in patients with PA-IVS and venticulocoronary connections
Right venous valve
What other structure is rarely normal in PA/IVS?
Tricuspid valve
How long does it take for RV compliance to improve after decompression?
Days to weeks
How can the patency of the pulmonary valve be assessed on echo in the absence of forward flow?
Assess for regurgitation
What is the preferred diagnostic test to assess for coronary anomalies in PA/IVS?
Cardiac catheterization
What are the two types of pulmonary atresia, and which happens earlier in development?
Membranous and Muscular; Muscular.
What is functional pulmonary atresia?
When the RV is unable to generate enough pressure to open the pulmonary valve
A patient with PA/IVS undergoes pulmonary artery perforation. They remain on PGE to keep the PDA open. Echo shoes severe TR. Which complication are they at risk of?
Circular shunt
What is the appropriate repair in a patient who has a patent right coronary conection the aorta, but absent left coronary?
Transplant vs Ductal stent or shunt
A patient with PA/IVS has massive cardiomegaly on X-ray, what associated cardiac anomalies might they have?
Tricuspid Atresia or Ebstein anomaly
What is a coronary cameral fistula?
A direct connection from the ventricular chamber to a major coronary artery
Which of the following structures is almost always present in patients with PA/IVS?
a. MPA trunk
b. Ventriculocoronary connections
c. Unroofed coronary sinus
d. Multiple aortopulmonary collaterals
e. Left SVC
MPA Trunk
What is the clinical presentation of circular shunt?
Cyanosis, LV dilation, low diastolic pressure
What is a one and a half ventricle repair?
A bidirectional glenn with continued use of the RV for outflow.
Why is it difficult to characterize the RV size in PA/IVS prior to intervention?
Severe muscular hypertrophy limits the ability to determine the true cavity size
What is the hemodynamic cause of coronary stenosis and interruption in PA/IVS (think flow)
High pressure turbulent flow causing increased shear forces.
PA/IVS is estimated to occur in 0.6 in every _____ live births
10,000
What is the most common long term electrophysiologic complication of PA/IVS?
Atrial arrhythmias
What happens to ventriculo-coronary connections after successful RV decompression?
The tend to regress
Which of the following is unlikely to be present in a patient with ventriculocoronary connections?
a. Myocardial ischemia
b. Endocardial fibroelastosis
c. Pericardial effusion
d. Myocardial rupture
Endocardial fibroelastosis
What is the histopathological finding found in the coronaries in PA/IVS?
Myointimal hyperplasia
In PA/IVS, what findings are associated with an RV to LV pressure ratio less than 1
Thin RV, severe tricuspid regurgitation, globally disadvantaged right ventricle
A patient undergoes cardiac catheterization which shows the following picture. The following day, he undergoes BTT shunt takedown and placement of an RV to PA conduit. What ECG anomaly would you expect to see in this patient?
Inferior ischemia pattern. STE in II, III, aVF
What is an alternative means of RV decompression besides pulmonary valvotomy/perforation?
Tricuspid valve resection/ avulsion
What is the cause of LVOT obstruction in PA/IVS?