Pulmonary Atresia and Intact Ventricular Septum

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

2. 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

3. 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

4. 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

5. 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.

6. 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.

7. 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.

8. 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.

9. 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 endocardialfibroelastosis and extensive ventriculocoronary communications.

10. 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 endocardialfibroelastosis and extensive ventriculocoronary communications.

11. Answer • As opposed to PA/VSD, PA/IVS has normal caliber pulmonary arteries.

12. 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.

13. 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.

14. 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.

15. 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.

16. 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.

17. 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) Intrapericardialteratoma

18. 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) Intrapericardialteratoma

19. 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

20. 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 be reliably seen by echo. • (E) Prostaglandins typically increase O2 satsby increasing pulmonary blood flow.

21. 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.

22. 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.

23. 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.

24. 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.

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

26. 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.

27. Answer • All are true

28. 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.

29. Answer • All are true.

30. 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.