Download Pulmonary Atresia and Intact Ventricular Septum

Steven H. Todman, M.D.
Assistant Professor
Pediatric Cardiology
LSUHSC-Shreveport
Objectives
 Pulmonary atresia with intact ventricular septum
 1. Anatomy -Recognize the anatomic features of
pulmonary atresia with intact ventricular septum
 2. Natural history -Recognize the natural history of a
patient with pulmonary atresia with intact ventricular
septum
 3. Clinical findings -Recognize the typical clinical
findings of a patient with pulmonary atresia with
intact ventricular septum
Objectives
4. Laboratory findings
 Recognize the echocardiographic features of pulmonary
atresia with intact ventricular septum
 Recognize the ECG findings in a patient with pulmonary
atresia with intact ventricular septum
 Recognize the findings of pulmonary atresia with intact
ventricular septum by cardiac catheterization
 Recognize the cardiac MRI/CT scan findings in a patient
with pulmonary atresia with intact ventricular septum
Objectives
5. Management, including complications
 Plan appropriate management of obstructed atrial
septum in a patient with pulmonary atresia with intact
ventricular septum
 Plan the surgical or transcatheter intervention in a
patient with pulmonary atresia and intact ventricular
septum at various stages
 Recognize and manage early and long-term
complications of surgical therapy in pulmonary atresia
with intact ventricular septum, and plan appropriate
management
Which of the following is false?
 (A) Cardiac organogenesis is complete by about 5
weeks’ gestation?
 (B) The usual form of PA and IVS occurs in a left sided
heart with normal atrial relations,
 (C) The usual form of PA and IVS occurs with
concordant AV connections, and concordant VA
connections,
 (D) The usual form of PA and IVS occurs with a PDA
that mediates pulmonary blood flow.
Which of the following is false?
 (A) Cardiac organogenesis is complete by about 8
weeks’ gestation?
 (B) The usual form of PA and IVS occurs in a left sided
heart with normal atrial relations,
 (C) The usual form of PA and IVS occurs with
concordant AV connections, and concordant VA
connections,
 (D) The usual form of PA and IVS occurs with a PDA
that mediates pulmonary blood flow.
Which of the following is false?
 (A) With pulmonary atresia/intact ventricular septum
the tricuspid valve is generally normal.
 (B) The pulmonary valve is derived from endocardial
tissue within the conotruncus.
 (C) In pulmonary atresia/intact ventricular septum,
the heart may be mildly or massively enlarged.
 (D) With pulmonary atresia/intact ventricular septum
there is an obligatory right-to-left shunt at the atrial
level.
Which of the following is false?
 (A) With pulmonary atresia/intact ventricular
septum the tricuspid valve is generally abormal.
 (B) The pulmonary valve is derived from endocardial
tissue within the conotruncus.
 (C) In pulmonary atresia/intact ventricular septum,
the heart may be mildly or massively enlarged.
 (D) With pulmonary atresia/intact ventricular septum
there is an obligatory right-to-left shunt at the atrial
level.
Which of the following is false?
 (A) With PA/IVS, the z value of the tricuspid valve
correlates with the size of the RV cavity.
 (B) The caliber of the pulmonary arteries is usually
diminished.
 (C) The myocardium of patients with PA/IVS can
demonstrate ischemia, fibrosis, or infarction.
 (D) An inverse relationship exists between ventricular
endocardial fibroelastosis and extensive
ventriculocoronary communications.
Which of the following is false?
 (A) With PA/IVS, the z value of the tricuspid valve
correlates with the size of the RV cavity.
 (B) The caliber of the pulmonary arteries is
usually diminished.
 (C) The myocardium of patients with PA/IVS can
demonstrate ischemia, fibrosis, or infarction.
 (D) An inverse relationship exists between ventricular
endocardial fibroelastosis and extensive
ventriculocoronary communications.
Answer
 As opposed to PA/VSD, PA/IVS has normal caliber
pulmonary arteries.
Which of the following are false?
 (A) Ventriculocoronary connections occur in thin-
walled, low-pressure right ventricles.
 (B) Coronary artery abnormalities requiring RV blood
flow include absent aortocoronary connections.
 (C) Coronary artery abnormalities requiring RV blood
flow include coronary artery interruption or stenosis.
 (D) Coronary artery abnormalities requiring RV blood
flow include profound coronary-cameral steal or
fistula.
Which of the following are false?
 (A) Ventriculocoronary connections do not occur
in thin-walled, low-pressure right ventricles.
 (B) Coronary artery abnormalities requiring RV blood
flow include absent aortocoronary connections.
 (C) Coronary artery abnormalities requiring RV blood
flow include coronary artery interruption or stenosis.
 (D) Coronary artery abnormalities requiring RV blood
flow include profound coronary-cameral steal or
fistula.
Which of the following are false?
 (A) In the normal circulation, the aortic diastolic
pressure is the primary driving pressure for coronary
blood flow.
 (B) Factors that reduce aortic diastolic pressure or
shorten diastole will compromise coronary blood flow.
 (C) Coronary artery obstruction and stenosis may
result in aortic diastolic pressure insufficient to drive
coronary blood flow.
 (D) Prostaglandins, or systemic to pulmonary artery
shunts will reduce aortic diastolic pressure.
Which of the following are false?
 (A) The smaller the tricuspid valve, the more likely
ventriculocoronary connections are present.
 (B) There is a male sex predilection, and infants
typically are born at term.
 (C) The first and second heart sounds are single, and a
pansystolic murmur of TR is often heard.
 (D) Patients typically are hypoxemic, and refractory to
increased O2, and are hypocarbic, relecting the
tachypnea.
Which of the following are false?
 (A) The smaller the tricuspid valve, the more likely
ventriculocoronary connections are present.
 (B) There is no known sex predilection, and
infants typically are born at term.
 (C) The first and second heart sounds are single, and a
pansystolic murmur of TR is often heard.
 (D) Patients typically are hypoxemic, and refractory to
increased O2, and are hypocarbic, relecting the
tachypnea.
All of the following can present with
massive cardiomegaly, except?
 (A) Pulmonary atresia and Ebstein anomaly
 (B) Ebstein and functional pulmonary atresia
 (C) d-Transposition of the great arteries
 (D) Aortic atresia, AV and VA discordance, and severe
left AV valve regurgitation
 (E) Functional aortic atresia, AV and VA discordance,
and severe left AV valve regurgitation.
 (F) Intrapericardial teratoma
All of the following can present with
massive cardiomegaly, except?
 (A) Pulmonary atresia and Ebstein anomaly
 (B) Ebstein and functional pulmonary atresia
 (C) d-Transposition of the great arteries
 (D) Aortic atresia, AV and VA discordance, and severe
left AV valve regurgitation
 (E) Functional aortic atresia, AV and VA discordance,
and severe left AV valve regurgitation.
 (F) Intrapericardial teratoma
All of the following can present with paucity of RV
forces, and LV dominance/LVH on EKG, except?
 (A) Pulmonary atresia and intact ventricular septum
 (B) Tricuspid atresia
 (C) Double-inlet left ventricle
 (D) Hypoplastic left heart
Which of the following are false?
 (A) All patients should undergo angiocardiographic
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


imaging prior to ventricular decompression.
(B) Shunting across the atrial septum is required to
maintain cardiac output.
(C) Functional PA is seen with high pulmonary artery
pressure with poor RV function, or very severe TR.
(D) Coronary artery stenosis or interruption can be
reliably seen by echo.
(E) Prostaglandins typically increase O2 sats by
increasing pulmonary blood flow.
Which of the following are false?
 (A) All patients should undergo angiocardiographic




imaging prior to ventricular decompression.
(B) Shunting across the atrial septum is required to
maintain cardiac output.
(C) Functional PA is seen with high pulmonary artery
pressure with poor RV function, or very severe TR.
(D) Coronary artery stenosis or interruption can
not be reliably seen by echo.
(E) Prostaglandins typically increase O2 sats by
increasing pulmonary blood flow.
Which of the following are false?
 (A) LV angiography will define the presence or absence
of ventriculocoronary connections.
 (B) Selective RVOT injection can differentiate severe
stenosis of the pulmonary valve from membranous
atresia.
 (C) A balloon occlusion technique performed in the
newborn’s ascending aorta will allow imaging of the
coronary arteries, their origin, distribution, and caliber
changes indicative of stenosis or interruption.
Which of the following are false?
 (A) RV angiography will define the presence or
absence of ventriculocoronary connections.
 (B) Selective RVOT injection can differentiate severe
stenosis of the pulmonary valve from membranous
atresia.
 (C) A balloon occlusion technique performed in the
newborn’s ascending aorta will allow imaging of the
coronary arteries, their origin, distribution, and caliber
changes indicative of stenosis or interruption.
Which of the following are false?
 (A) When RV angiography does not demonstrate
ventriculocoronary connections, one can be
reasonably certain that coronary arterial stenosis or
interruption of major fistulae with coronary-cameral
flow will not be evident.
 (B) RV Angiography should be performed in frontal
and lateral projections.
 (C) Once pulmonary blood flow is established, a very
low PVR and a high SVR can result in low cardiac
output, despite high sats.
Which of the following are false?
 All are true.
Which of the following are false?
 (A) If ventriculocoronary connections and the
majority or the entirety of the coronary circulation is
RV dependent, the patient should be placed on a
univentricular palliation algorithm.
 (B) The RV can enlarge if it is satisfactorily
decompressed.
 (C) Patients with absence of bilateral proximal
coronary-aorto connections should undergo transplant
versus high-risk shunt
 (D) Absence of aorto-left coronary artery connection
should consider transplant or high-risk shunt.
Answer
 All are true
Which of the following are false?
 (A) Patients with proximal LAD interruption, proximal
RCA stenosis, significantly RV-dependent myocardial
perfusion should undergo shunt or ductal stent, or
univentricular track.
 (B) Patients with mild distal stenosis or ectasia in the
presence of ventriculocoronary connections, should
undergo RV decompression.
 (C) Patients with ventriculocoronary connections
without stenosis or interruption should undergo RV
decompression.
Answer
 All are true.
Which of the following are false?
 (A) Decompression of the RV in the setting of RV
dependent myocardial circulation often results in
suicide RV, and these patients should undergo a ductal
stent or systemic to PA shunt as the initial procedure.