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Pulmonary Atresia and VSD

Pulmonary Atresia and VSD. Steven H. Todman , M.D. Assistant Professor Pediatric Cardiology LSUHSC-Shreveport. Objectives. Pulmonary atresia with ventricular septal defect 1 . Embryology Know the embryologic basis of pulmonary atresia with ventricular septal defect

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Pulmonary Atresia and VSD

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  1. Pulmonary Atresia and VSD Steven H. Todman, M.D. Assistant Professor Pediatric Cardiology LSUHSC-Shreveport

  2. Objectives • Pulmonary 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

  3. Objectives 4. 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

  4. Objectives 6. 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

  5. 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.

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. Which of the following are false? • All are true.

  11. 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.

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. 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.

  17. 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 posteriorlymalpositioned. • (D) The infundibular septum is fused with the RV free wall. • (E) In Truncusarteriosus the pulmonary arteries arise directly from the posterolateral aspect of the truncal root prior to the arch.

  18. 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 anteriorlymalpositioned. • (D) The infundibular septum is fused with the RV free wall. • (E) In Truncusarteriosus the pulmonary arteries arise directly from the posterolateral aspect of the truncal root prior to the arch.

  19. 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.

  20. Which of the following are false? • All are true.

  21. 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.

  22. 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.

  23. Which of the following are false? • (A) RVOT reconstruction ondunifocalization 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.

  24. Which of the following are false? • All are true.

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