Pulmonary Atresia with Intact Ventricular Septum (PA/IVS) Flashcards
(35 cards)
What is pulmonary atresia with intact ventricular septum (PA/IVS)?
Characterized by atresia of the pulmonary valve, which is imperforate, and an intact ventricular septum.
The condition relies on a patent ductus arteriosus for pulmonary blood flow.
What are the key anatomical features of PA/IVS?
Significant heterogeneity based on:
* Degree and form of pulmonary atresia
* Size of the right ventricle
* Size of the tricuspid valve
* Abnormal coronary circulation
Often referred to as ‘RV dependent coronaries’.
How does PA/IVS typically present?
In the neonatal period.
Management options depend on multiple factors.
What are the management options for PA/IVS?
Options include:
* Biventricular repair
* Single ventricle palliation
* One and a half ventricle pathway
* Cardiac transplantation
Cardiac transplantation is usually reserved for the most severe variants.
What is the estimated incidence of PA/IVS?
Found in about 6 to 8.3 in 100,000 live births.
This number may be an underestimation due to fetal or in utero termination.
Is there a sex predilection for PA/IVS?
No, there is no sex predilection known at this time.
What is the current understanding of the genetic causes of PA/IVS?
There is no identifiable genetic cause known at this time.
Fill in the blank: The pulmonary valve in PA/IVS is _______.
imperforate.
True or False: The ventricular septum in PA/IVS is typically not intact.
False.
What is PA/IVS?
A complex lesion with heterogeneity in procedural pathways, prognosis, and anatomic variance.
What characterizes pulmonary atresia in PA/IVS?
Imperforate due to membranous or thicker muscular atresia.
How does the morphology of the pulmonary valve correlate with the right ventricle?
If the RV is well formed, the pulmonary valve likely has complete fusion of the cusps and commissures causing membranous atresia. If the RV is diminutive, there is likely a primitive pulmonary valve with severely narrowed or atretic infundibulum or muscular atresia.
What is the range of heterogeneity in right ventricular features?
The RV may range from a fully developed tripartite structure to a severely underdeveloped and hypoplastic inlet RV.
What has been shown to be associated with the size of the right ventricular cavity?
The Z-score of the tricuspid valve.
What causes abnormal coronary circulation in PA/IVS?
Typically due to subepicardial coronary arteries and development of sinusoidal or fistulous connections depending on RV pressure.
What conditions lead to RV-dependent coronaries?
Absent aortocoronary connections, coronary artery interruption or stenosis, or profound coronary-cameral steal or fistula.
What is typical about the tricuspid valve in PA/IVS?
Typically abnormal with a wide range of dysplasia, hypoplasia, and competency.
Why does the tricuspid valve often exhibit issues in PA/IVS?
Due to blood flow having no egress and must regurgitate, alongside restriction of the tricuspid valve leaflets and chordae from high right-sided pressures.
What is the typical state of the branch pulmonary arteries in PA/IVS?
In the majority of cases, they are confluent and fed by a left-sided patent ductus arteriosus.
What is a rare condition regarding the supply of discontinuous pulmonary arteries?
They are supplied by bilateral ducti or aortopulmonary collaterals.
What can hemodynamics differ greatly depending on?
The specific constellation of anatomic variance
What is a common symptom observed in the early neonatal period?
Progressive cyanosis
What type of murmur is typically related to tricuspid valve regurgitation?
A murmur related to the degree of tricuspid valve regurgitation and a ductal murmur
What treatment do patients often need in the neonatal period?
Prostaglandin infusion
