Pulmonary Atresia and VSD

Pulmonary Atresia and VSD

Steven H. Todman, M.D.Assistant ProfessorPediatric CardiologyLSUHSC-Shreveport

ObjectivesPulmonary atresia with ventricular septal defect 1. Embryology

Know the embryologic basis of pulmonary atresia with ventricular septal defect

2. Etiology, epidemiology, and genetic implications Recognize the genetic syndromes associated with

pulmonary atresia with ventricular septal defect 3. Anatomy

Recognize the abnormalities of the pulmonary vascular bed in pulmonary atresia with ventricular septal defect

Recognize lesions commonly associated with pulmonary atresia with ventricular septal defect

Objectives4. Physiology Determine pulmonary and systemic blood

flow by cardiac catheterization in a patient with pulmonary atresia and ventricular septal defect

5. Natural history Recognize the natural history of a patient

with pulmonary atresia with ventricular septal defect

Objectives6. Laboratory findings Diagnose pulmonary atresia with ventricular septal defect by

echocardiography and recognize important anatomic features that could affect surgical management

Assess and interpret sources of pulmonary blood flow and adequacy of pulmonary artery size in a patient with pulmonary atresia and ventricular septal defect by angiocardiographic studies

Recognize the ECG findings in a patient with pulmonary atresia with ventricular septal defect

Recognize the cardiac MRI/CT scan findings in a patient with pulmonary atresia with ventricular septal defect

Recognize the findings of pulmonary atresia with ventricular septal defect by cardiac catheterization

Which of the following is false?(A) In PA-VSD, there are several

abnormalities in the size and distribution of the pulmonary arterial branches, and systemic collateral vessels that supply all or part of the lung parenchyma.

(B) Maternal diabetes, maternal PKU, and maternal exposure to retinoic acids and to trimethadione are associated with an increased risk of conotruncal defects in infants.

(C) 30% of patients with PA-VSD have no associated genetic anomaly.

Which of the following is false?(A) In PA-VSD, there are several

abnormalities in the size and distribution of the pulmonary arterial branches, and systemic collateral vessels that supply all or part of the lung parenchyma.

(B) Maternal diabetes, maternal PKU, and maternal exposure to retinoic acids and to trimethadione are associated with an increased risk of conotruncal defects in infants.

(C) 70% of patients with PA-VSD have no associated genetic anomaly.

Which of the following is false?(A) A patient with CHD, palatal anomalies, hypocalcemia,

immunodeficiency, speech and learning disabilities, renal anomalies, psychiatric problems, and distinct facial features may have PA/VSD.

(B) 8 to 23% of patients with TOF have a 22q11 deletion.(C) Associated vascular anomalies in PA/VSD include AP

collaterals, RAA, aberrant subclavian artery, and occur more frequently in patients with 22q11 mutation.

(D) Patients with 22q11 deletion have smaller branch PA’s than in patients without the deletion.

(E) Clinical outcomes for patients with PA-VSD and 22q11 deletion tend to be better.

Which of the following is false?(A) A patient with CHD, palatal anomalies,

hypocalcemia, immunodeficiency, speech and learning disabilities, renal anomalies, psychiatric problems, and distinct facial features may have PA/VSD.

(B) 8 to 23% of patients with TOF have a 22q11 deletion.(C) Associated vascular anomalies in PA/VSD include AP

collaterals, RAA, aberrant subclavian artery, and occur more frequently in patients with 22q11 mutation.

(D) Patients with 22q11 deletion have smaller branch PA’s than in patients without the deletion.

(E) Clinical outcomes for patients with PA-VSD and 22q11 deletion tend to be worse.

Which of the following are false?(A) About day 27, the arterial branches of the paired sixth

aortic arches form an anastamosis with the pulmonary vascular plexus, giving the lungs a dual blood supply.

(B) During normal development the branches from the sixth aortic arches enlarge, and those from the descending thoracic aorta become smaller.

(C) The larger vessels form the true pulmonary arteries and deliver blood to the alveoli or capillaries derived from the pulmonary vascular plexus.

(D) Smaller vessels from the descending thoracic aorta form the nutrient bronchial arteries.

(E) In PA/VSD there is a complete discontinuity of the RV and central PA’s, resulting in a variable source of pulmonary blood flow.

Which of the following are false?All are true.

Which of the following are false?(A) With PA-VSD, the central right and left

pulmonary arteries and/or the segmental pulmonary arteries can be confluent or non-confluent.

(B) In PA-VSD, the blood supply to the lungs is entirely from the systemic arterial circulation.

(C) When the ductus or collateral arteries connect proximally to the central pulmonary arteries or their lobar branches, the central vessels may be only mildly hypoplastic or even normal in size.

(D) With MAPCAS, the PA’s tend to be normal size.

Which of the following are false?(A) With PA-VSD, the central right and left

pulmonary arteries and/or the segmental pulmonary arteries can be confluent or non-confluent.

(B) In PA-VSD, the blood supply to the lungs is entirely from the systemic arterial circulation.

(C) When the ductus or collateral arteries connect proximally to the central pulmonary arteries or their lobar branches, the central vessels may be only mildly hypoplastic or even normal in size.

(D) With MAPCAS, the PA’s tend to be hypoplastic.

Which of the following are false?(A) A right aortic arch is present in 26-50% of

cases.(B) RVH is moderate to severe.(C) The infundibulum ends blindly and may

be fused to the RV wall.(D) The coronary arteries typically have an

abnormal distribution.(E) Pulmonary atresia/VSD may be associated

with persistent LSVC to CS, anomalous pulmonary veins, tricuspid stenosis/atresia, complete av canal, TGV, dextrocardia, and heterotaxy.

Which of the following are false?(A) A right aortic arch is present in 26-50% of

cases.(B) RVH is moderate to severe.(C) The infundibulum ends blindly and may

be fused to the RV wall.(D) The coronary arteries typically have a

normal distribution.(E) Pulmonary atresia/VSD may be associated

with persistent LSVC to CS, anomalous pulmonary veins, tricuspid stenosis/atresia, complete av canal, TGV, dextrocardia, and heterotaxy.

Which of the following are false?(A) Patients with PA-VSD often present as a

cyanotic newborn unless they have a large PDA or well-developed systemic to pulmonary collaterals.

(B) Patients with microdeletion of 22q11 tend to have more complex collateral and pulmonary arterial anatomy than patients without this genetic abnormality.

(C) There is a single second heart sound, a systolic murmur is present at the lower left sternal border, and continuous murmurs if MAPCAS are present.

Which of the following are false?(A) Patients with PA-VSD often present as a

cyanotic newborn unless they have a large PDA or well-developed systemic to pulmonary collaterals.

(B) Patients with microdeletion of 22q11 tend to have more complex collateral and pulmonary arterial anatomy than patients without this genetic abnormality.

(C) There is a single second heart sound, a systolic murmur is present at the lower left sternal border, and continuous murmurs if MAPCAS are present.

Which of the following are false?(A) RVH and RAD are common, vs. PA/IVS where RV

hypoplasia usually is present, with small QRS forces and LV preponderance.

(B) Right aortic arch is more frequently seen than with TOF.

(C) The infundibular portion of the ventricular septum is posteriorly malpositioned.

(D) The infundibular septum is fused with the RV free wall.

(E) In Truncus arteriosus the pulmonary arteries arise directly from the posterolateral aspect of the truncal root prior to the arch.

Which of the following are false?(A) RVH and RAD are common, vs. PA/IVS where RV

hypoplasia usually is present, with small QRS forces and LV preponderance.

(B) Right aortic arch is more frequently seen than with TOF.

(C) The infundibular portion of the ventricular septum is anteriorly malpositioned.

(D) The infundibular septum is fused with the RV free wall.

(E) In Truncus arteriosus the pulmonary arteries arise directly from the posterolateral aspect of the truncal root prior to the arch.

Which of the following are false?(A) Cardiac catheterization is required to

delineate size and distribution of the true pulmonary arteries, and to ascertain the extent of collaterals.

(B) RA pressure is usually normal, and LV and RV pressure is equal.

(C) True pulmonary artery pressure and resistance are normal in most instances.

(D) Entering the PA’s directly allows pulmonary arteriolar resistance to be calculated, and estimate pulmonary flow via the Fick method.

Which of the following are false?All are true.

Which of the following are false?(A) LV injection with cameras positioned to

record 70 degree right anterior oblique is important to view VSDs.

(B) Origin of the LAD from the RCA occurs in 5% of patients, and is of surgical importance.

(C) Occasionally, an evanescent negative washout pattern can be appreciated that is due to a stream of unopacified blood from a connecting PA flowing into an area of opacified pulmonary arterial tree.

Which of the following are false?(A) LV injection with cameras positioned

to record 70 degree left anterior oblique is important to view VSDs.

(B) Origin of the LAD from the RCA occurs in 5% of patients, and is of surgical importance.

(C) Occasionally, an evanescent negative washout pattern can be appreciated that is due to a stream of unopacified blood from a connecting PA flowing into an area of apacified pulmonary arterial tree.

Which of the following are false?(A) RVOT reconstruction ond unifocalization requires

all septal defects to be closed, interruption of all extracardiac sources of pulmonary arterial blood flow, and incorporation of at least 14 pulmonary arterial segments in a connection to the RV.

(B) Additionally, the central PA size should be at least 50% of normal, and RVP should be <70% that measured in the LV.

(C) VSD can be reopened if RV/LV systolic pressure is >0.85, which is the major predictor for late mortality.

(D) Patients with PA-VSD with normal or mildly elevated pulmonary pressures can tolerate pregnancy.

Which of the following are false?All are true.