Adult congenital heart disease Complex plumbing made simple

Transcription

1 Adult congenital heart disease Complex plumbing made simple James Oliver Leeds

2 Bayer Disclosures

3 Q 1. With respect to atrial septal defects: 1. Severe right heart volume loading is generally a contraindication to ASD closure 2. Primum atrial septal defects should only be closed with a device if the Qp:Qs is > Severe pulmonary vascular disease occurs in less than 5% of patients with ASDs 4. Atrial arrhythmia is generally improved after ASD closure in older patients 5. Sinus venosus atrial septal defects are frequently associated with anomalous drainage of the right lower pulmonary vein

4 Q This lesion increases the risk of stroke 2. In the absence of symptoms conservative management is preferred 3. The mitral regurgitation occurs secondary to AMVL prolapse 4. 1 and 2 are true 5. 2 and 3 are true AS prim ASD 2D with 3 views of cleft.mp4

5 Q 3. Which statement is correct? 1. A pacing lead is entering the right atrium 2. An inferior sinus venous ASD is shown 3. A superior sinus venosus ASD is shown 4. 1 and 3 are true 5. 1 and 2 are true

6 Q 4. With respect to partial anomalous pulmonary venous drainage which statement is incorrect? 1. Pulmonary veins may connect to the coronary sinus 2. Pulmonary veins may connect to a branch pulmonary artery 3. Scimitar syndrome is a form of partial anomalous pulmonary venous drainage 4. Causes a left to right shunt 5. There is an association with atrial septal defect

7 Q 1. With respect to atrial septal defects: 1. Severe right heart volume loading is generally a contraindication to ASD closure 2. Primum atrial septal defects should only be closed with a device if the Qp:Qs is > Severe pulmonary vascular disease occurs in less than 5% of patients with ASDs 4. Atrial arrhythmia is generally improved after ASD closure in older patients 5. Sinus venosus atrial septal defects are frequently associated with anomalous drainage of the right lower pulmonary vein

8 Q This lesion increases the risk of stroke 2. In the absence of symptoms conservative management is preferred 3. The mitral regurgitation occurs secondary to AMVL prolapse 4. 1 and 2 are true 5. 2 and 3 are true AS prim ASD 2D with 3 views of cleft.mp4

9 Q 3. Which statement is correct? 1. A pacing lead is entering the right atrium 2. An inferior sinus venous ASD is shown 3. A superior sinus venosus ASD is shown 4. 1 and 3 are true 5. 1 and 2 are true

10 Secundum 75% ASDs Defects of fossa ovalis Primum 15% ASDs Atrioventricular septal defect spectrum Primum ASD + cleft AMVL = partial AVSD Superior sinus venosus 5% ASDs Septum anterior/inferior, RA free wall posterior Superior border absent - over-riding SVC R upper/middle PVs usually drain to SVC SVC/RA junction

11 Left to right flow High pulmonary blood flow, Qp:Qs can be 3-4:1 RA & RV dilate (volume-loaded) Flow mainly in diastole Determined by compliance of RV and LV

12 GB combined.mp4 Secundum Primum

13 Superior sinus venosus SV ASD Dick JJ combined pre-op.mp4

14 ASDs - clinical features Most commonly present in 3rd and 4th decades Progressive SOB, exercise capacity Atrial arrhythmias About 10% in those >40 yrs & 50% in those >60 yrs Recurrent chest infections Paradoxical embolism Right heart failure PA pressure generally only slightly and PVR generally normal PAP may be moderately elevated in older patients If severe, likely co-existent primary PH

15 Indications for closure Significant shunt (RV volume loading) irrespective of presence of symptoms Suspicion of paradoxical embolus at any size Exercise-induced cyanosis (unless pulmonary hypertensive) Not if established pulmonary vascular disease

16 How to close Device or surgery Device if <38 mm with 5mm margin (except to aorta) Device closable in about 80% Surgery With redirection of anomalous pulmonary veins to LA in sinus venosus ASD

17 Effect of closure Improved exercise capacity Right heart shrinks PA pressure falls Can prevent atrial arrhythmia: Best chance if closed < 40 yr If persistent AF before will stay in AF after Functional class 3-6/12 after device More historical data demonstrate a survival benefit after surgical ASD closure Humenberger et al. Eur Heart J 2011;32:553

18 Q 4. With respect to partial anomalous pulmonary venous drainage which statement is incorrect? 1. Pulmonary veins may connect to the coronary sinus 2. Pulmonary veins may connect to a branch pulmonary artery 3. Scimitar syndrome is a form of partial anomalous pulmonary venous drainage 4. Causes a left to right shunt 5. There is an association with atrial septal defect One or more pulmonary veins drain to right atrium Directly or via any systemic venous structure (e.g. SVC, coronary sinus) Physiologically like an ASD (left-to-right shunt) Presentation as for ASD with right sided volume loading Size of shunt depends on number of anomalous veins Often associated with ASDs (sinus venosus and 2% of secundum defects)

19 Brachiocephalic Ascending vein BA anom PVs CT Left upper Left middle Left atrial appendage

20 Q 5. Which statement is true? 1. Muscular VSDs are associated with aortic incompetence due to prolapse of the right coronary cusp 2. Small, restrictive VSDs are not associated with an increased risk of endocarditis 3. Moderate sized or larger VSDs cause dilatation of the right ventricle 4. Doubly committed VSDs are located in the infundibular septum 5. VSD closure should only be considered in Eisenmenger syndrome following effective pulmonary vasodilator therapy

21 Q 6. This patient has a high velocity tricuspid regurgitation jet 1. The echo shows tetralogy of Fallot 2. They have pulmonary hypertension 3. The echo shows a double chamber right ventricle 4. The echo shows a muscular ventricular septal defect 5. 2 and 3 are correct

22 Q Most cases of complete atrioventricular septal defect occur in patients with trisomy Most cases of partial atrioventricular septal defect occur in patients with trisomy In complete atrioventricular septal defect the common atrioventricular valve consists of 5 leaflets 4. 1 and 2 are true 5. 1 and 3 are true

23 Q 5. Which statement is true? Perimembranous and doubly committed 1. Muscular VSDs are associated with aortic incompetence due to prolapse of the right coronary cusp 2. Small, restrictive VSDs are not associated with an increased risk of endocarditis 3. Moderate sized or larger VSDs cause dilatation of the right ventricle 4. Doubly committed VSDs are located in the infundibular septum 5. VSD closure should only be considered in Eisenmenger syndrome following effective pulmonary vasodilator therapy left ESC say IE in up to 2 per 1000 patient years, 6 x normal population

24 Muscular Within the muscular septum Rim entirely of muscle

25 Membranous Membranous septum between inlet & outlet portions of muscular septum, just beneath RCC & NCC True membranous VSDs surrounded by fibrous tissue, perimembranous if extend to muscular septum 10 o'clock

26 Doubly committed, juxta-arterial In infundibular septum, separating the right and left ventricular outflow tracts 2 O'clock

27 Blood pumped directly in systole from LV to pulmonary arteries The LA and the LV, not the RV, see the extra flow - left heart becomes volume loaded pulmonary blood flow at systemic pressure potential for pulmonary vascular disease (Eisenmenger) Posteriorly directed AI RCC prolapse Venturi effect from VSD

28 Indications to close a VSD Symptoms attributable to VSD Volume load due to VSD AI due to aortic valve leaflet prolapse Consider if history of IE If pulmonary vascular disease, consider if Qp:Qs > 1.5 and PVR < 2/3 SVR (including after pulmonary vasodilatation), not if exercise-induced desaturation

29 Q 6. This patient has a high velocity tricuspid regurgitation jet 1. The echo shows tetralogy of Fallot 2. They have pulmonary hypertension 3. The echo shows a double chamber right ventricle 4. The echo shows a muscular ventricular septal defect 5. 2 and 3 are correct Mid-cavity RV muscle overgrowth, often associated with a VSD Results in a high pressure proximal RV chamber (high velocity TR jet) and a low pressure distal chamber

30 How to calculate a shunt Ensure IVC sampled at level of diaphragm, to include hepatic venous blood

31 Q Most cases of complete atrioventricular septal defect occur in patients with trisomy Most cases of partial atrioventricular septal defect occur in patients with trisomy In complete atrioventricular septal defect the common atrioventricular valve consists of 5 leaflets 4. 1 and 2 are true 5. 1 and 3 are true

32 Atrioventricular septal defect Partial AVSD Primum ASD Discrete MV and TV annuli Cleft mitral valve MV and TV not offset >90% in non-trisomy 21 cavsd neonate JM-H Spectrum Complete Primum ASD, VSD Common AV valve >75% in trisomy 21 Repaired in childhood If not - Eisenmenger Complete AVSD Commonest problem after cavsd repair regurg through cleft left AV valve

33 Q 8. The following statements refer to previously repaired tetralogy of Fallot A. The risk of sudden cardiac death is reported as 1 6% B. Repair infrequently results in significant pulmonary incompetence C. A QRS duration of >180 ms is an indication for a primary prevention ICD D. Pregnancy should be avoided in women with severe pulmonary incompetence 1. A and B are true 2. All are true 3. All are false 4. Only A is true 5. A and C are true

34 Q 9. The following statements refer to coarctation of the aorta A. Coarctation is one component of Shone syndrome B. Hypertension in previously treated coarctation is usually due to restenosis C. Measuring BP in the left arm is generally preferred D. Of the different surgical repair techniques, aneurysm is most strongly associated with endto-end repair E. Hypertensive patients with a pressure difference between upper and lower limbs of 10 mmhg or more should undergo intervention 1. Only E is true 2. Only A is true 3. B and C are true 4. A and E are true 5. A, B and D are true

35 Q 8. The following statements refer to previously repaired tetralogy of Fallot A. The risk of sudden cardiac death is reported as 1 6% B. Repair infrequently results in significant pulmonary incompetence C. A QRS duration of >180 ms is an indication for a primary prevention ICD D. Pregnancy should be avoid in women with severe pulmonary incompetence 1. A and B are true 2. All are true 3. All are false 4. Only A is true 5. A and C are true Generally very well tolerated in pregnancy Risk factor for VT, but not indication for ICD

36 Tetralogy of Fallot One of the commonest repaired congenital lesions, nearly 10% of CHD Right ventricular outflow obstruction Infundibular (sub valvar) stenosis Pulmonary valve stenosis Small main and branch PAs Over-riding aorta VSD Right ventricular hypertrophy

37 Tetralogy repair Closure of VSD Relief of RVOT obstruction - Resection of infundibular muscle - Transannular patch, to enlarge outflow tract - Patch enlargement of PAs

38 Tetralogy issues Trans-annular patch ToF severe PI combined SS Obligate area of incompetence Pulmonary incompetence Right ventricular dilatation and dysfunction (systolic and diastolic) Residual pulmonary stenosis - infundibular, valvar, PA/branch PAs Tricuspid regurgitation Atrial and ventricular arrhythmia

39 Pulmonary valve replacement 144 ToF repairs in Dutch centre Cuypers. Circulation 2014;130:1944

40 ToF arrhythmia 144 ToF repairs in Dutch centre 10% had PPMs and 5% ICDs Cuypers. Circulation 2014;130:1944

41 Q 9. The following statements refer to coarctation of the aorta A. Coarctation is one component of Shone syndrome B. Hypertension in previously treated coarctation is usually due to restenosis C. Measuring BP in the left arm is generally preferred D. Of the different surgical repair techniques, aneurysm is most strongly associated with end-to-end repair E. Hypertensive patients with a pressure difference between upper and lower limbs of 10 mmhg or more should undergo intervention 1. Only E is true 2. Only A is true - multilevel left heart obstruction: MS subas, AS, CoA 3. B and C are true 4. A and E are true 5. A, B and D are true

42 Q 9. The following statements refer to coarctation of the aorta A. Coarctation is one component of Shone syndrome B. Hypertension in previously treated coarctation is usually due to restenosis C. Measuring BP in the left arm is generally preferred D. Of the different surgical repair techniques, aneurysm is most strongly associated with end-to-end repair E. Hypertensive patients with a pressure difference between upper and lower limbs of 10 mmhg or more should undergo intervention 20 patch Hypertension Check BP in right arm Reported in 25 to 70% late after repair Usually no significant recoarctation

43 Q 9. The following statements refer to coarctation of the aorta A. Coarctation is one component of Shone syndrome B. Hypertension in previously treated coarctation is usually due to restenosis C. Measuring BP in the left arm is generally preferred D. Of the different surgical repair techniques, aneurysm is most strongly associated with end-to-end repair E. Hypertensive patients with a pressure difference between upper and lower limbs of 10 mmhg or more should undergo intervention Also pressure drop of 20 mmhg and: Excessive BP response to exercise Significant LVH

44 Coarctation 6-8% CHD Strong association BAV/Turner Surgical repair in children, often via left thoracotomy Generally stent in adults and recoarctation, <1% aortic rupture End-to-end Subclavian flap Patch aortoplasty

45 31% restenosis 9% significant 13% dilatation 9% discrete aneurysm If history of coarctation repair (or a left thoracotomy scar) and: Chest pain. think dissection Haemoptysis or haematenesis. think aneurysm rupture into lungs or oesophagus

46 Q 10. In patients with cyanotic heart disease: A. Measured INR may be falsely low B. Coagulation sample tubes may need less citrate than is standard C. A normal [Hb] excludes significant anaemia D. Measured plasma glucose may be falsely high E. Venesection is recommended to maintain haematocrit < 0.6 to prevent symptoms of hyperviscosity 1. A and B are true 2. B and E are true 3. C and D are true 4. A and E are false 5. E is true

47 Q 11. With respect to surgical shunts in cyanotic heart disease: A. A bidirectional Glenn shunt connects the superior vena cava to a branch pulmonary artery B. A classical Blalock-Taussig shunt will likely cause an absent radial pulse in the ipsilateral arm C. A modified Blalock-Taussig shunt consists of a prosthetic tube between a subclavian artery and the ipsilateral branch pulmonary artery D. A Glenn shunt improves systemic oxygenation but at the expense of volume loading of the heart E. An arterial shunt improves systemic oxygenation without causing volume loading of the heart 1. C and D are true 2. All are true 3. A and D are true 4. A, B and C are true 5. B and E are true

48 Q 12. With respect to the Fontan circulation: A. Desaturation may occur secondary to systemic venous to pulmonary venous collaterals B. Elevated plasma alpha-1 antitrypsin levels suggest protein losing enteropathy C. Pregnancy should be advised against D. Intravenous amiodarone is the preferred treatment for acute atrial arrhythmia E. A successful Fontan circulation should allow for participation in competitive sports 1. A, B and E are true 2. Only B is true 3. C and E are true 4. Only A is true 5. B and D are true

49 Q 10. In patients with cyanotic heart disease: A. Measured INR may be falsely low B. Coagulation sample tubes may need less citrate than is standard C. A normal [Hb] excludes significant anaemia D. Measured plasma glucose may be falsely high E. Venesection is recommended to maintain haematocrit < 0.6 to prevent symptoms of hyperviscosity INR testing sensitive to plasma:citrate ratio 1. A and B are true 2. B and E are true 3. C and D are true 4. A and E are false 5. E is true haematocrit means plasma volume - falsely INR, equivalent to under-filled tube Use tubes with less citrate if hct > 0.55 Blood glucose might measure low - in vitro glycolysis by red cells

50 Q 10. In patients with cyanotic heart disease: A. Measured INR may be falsely low B. Coagulation sample tubes may need less citrate than is standard C. A normal [Hb] excludes significant anaemia D. Measured plasma glucose may be falsely high E. Venesection is recommended to maintain haematocrit < 0.6 to prevent symptoms of hyperviscosity 1. A and B are true 2. B and E are true 3. C and D are true 4. A and E are false 5. E is true Erythrocytosis - the physiological response to O 2 Hb in normal range = relative anaemia Venesection not routinely recommended: Temporary fix Promotes iron deficiency, which is associated with stroke Can cause acute haemodynamic decompensation

51 Q 11. With respect to surgical shunts in cyanotic heart disease: A. A bidirectional Glenn shunt connects the superior vena cava to a branch pulmonary artery B. A classical Blalock-Taussig shunt will likely cause an absent radial pulse in the ipsilateral arm C. A modified Blalock-Taussig shunt consists of a prosthetic tube between a subclavian artery and the ipsilateral branch pulmonary artery without causing D. A Glenn shunt improves systemic oxygenation but at the expense of volume loading of the heart E. An arterial shunt improves systemic oxygenation without causing volume loading of the heart but at the expense of 1. C and D are true 2. All are true 3. A and D are true 4. A, B and C are true 5. B and E are true

52 Blalock-Taussig (BT) shunts Classical - subclavian artery to ipsilateral PA Modified - prosthetic tube instead Arterial shunts Shunts between aorta and PAs Risk of over-shunting Volume loading and pulmonary vascular disease Passive flow SVC to lungs Glenn shunt No volume loading IVC to heart

53 Other systemic features of cyanosis Thrombocytopaenia, bleeding and thrombosis Haemoptysis (often from bleeding collaterals) Gout Renal impairment Systemic emboli, stroke, cerebral abscess

54 Single ventricle hearts Unbalanced AVSD Can t create separate pumps for systemic & pulmonary circulations Oxygenated and deoxygenated blood mixes in ventricle, so cyanosed Tricuspid atresia Mitral atresia Double inlet left ventricle

55 Fontan circulation Surgically created circulation in which systemic venous blood flows passively to the lungs Fontan

56 Fontan circulation how is it done? Atriopulmonary Lateral tunnel Extra-cardiac conduit Systemic venous and pulmonary venous blood separated No longer cyanosed volume load exercise capacity

57 Q 12. With respect to the Fontan circulation: A. Desaturation may occur secondary to systemic venous to pulmonary venous collaterals stool B. Elevated plasma alpha-1 antitrypsin levels suggest protein losing enteropathy C. Pregnancy should be advised against D. Intravenous amiodarone is the preferred treatment for acute atrial arrhythmia E. A successful Fontan circulation should allow for participation in competitive sports 1. A, B and E are true 2. Only B is true 3. C and E are true 4. Only A is true 5. B and D are true DCCV (acute atrial arrhythmia can cause serious decompensation) VO2 max generally about 60% predicted Keep to recreational sports

58 Q 13. This MRI scan is from a patient who has undergone surgery for transposition of the great arteries A. The subpulmonary ventricle is dilated and impaired B. The patient is at risk of ventricular tachycardia C. There is an atrial septal defect D. The tricuspid regurgitation will be high velocity E. The patient has undergone arterial switch surgery 1. Only B is true 2. A and D are true 3. B, C and D are true 4. B, C and E are true 5. B and D are true

59 Q 13. This MRI scan is from a patient who has undergone surgery for transposition of the great arteries systemic RV A. The subpulmonary ventricle is dilated and impaired B. The patient is at risk of ventricular tachycardia C. There is an atrial septal defect The hole is the pulmonary venous channel D. The tricuspid regurgitation will be high velocity TV = the systemic AV valve E. The patient has undergone arterial switch surgery atrial 1. Only B is true 2. A and D are true 3. B, C and D are true 4. B, C and E are true 5. B and D are true

60 Atrial switch for TGA Senning (described 1958) or Mustard operations Lots of potential issues in the long term

61 Systemic RV dysfunction

62 Baffle leaks Baffle stenoses Plus bradycardia, atrial tachycardia and VT

63 Q 14. This patient had no right ventricular outflow obstruction. Which statement(s) is/are correct? A. The chances of survival for more than 10 years following diagnosis are very small B. Haemoptysis is a poor prognostic sign C. Anticoagulation is indicated to prevent systemic embolisation D. Modern management should include consideration of closure of the defect alongside aggressive pulmonary vasodilator treatment 1. A only 2. B and C 3. A and D 4. None 5. All

64 Q 14. This patient had no right ventricular outflow obstruction. Which statement(s) is/are correct? A. The chances of survival for more than 10 years following diagnosis are very small B. Haemoptysis is a poor prognostic sign C. Anticoagulation is indicated to prevent systemic embolisation D. Modern management should include consideration of closure of the defect alongside aggressive pulmonary vasodilator treatment 1. A only 2. B and C 3. A and D 4. None 5. All

65 Pulmonary hypertension in CHD PH = mean PAP 25 mmhg In CHD caused by high pulmonary blood flow at high pressure Irreversible injury to pulmonary arterial resistance vessels and progressive in PVR

66 Congenital defects that can cause PH ASDs not included VSD AVSD Patent ductus arteriosus Single ventricle, unrestricted pulmonary blood flow Pulmonary atresia, aortopulmonary collaterals

67 Eisenmenger physiology The extreme end of the PH spectrum in CHD PH with reversed central shunt: Starts left-to-right (VSD, AVSD, PDA) Becomes right-to-left as PAP to and above systemic pressure, cyanosis Please believe me They really are not synonymous It s the truth Cyanotic patients are not necessarily pulmonary hypertensive Although they can be Honestly I m not lying Check with another ACHD cardiologist if you like

68 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

69 Consequences of PH in CHD Impaired exercise capacity Cyanosis (if not already) RV/ventricular failure Haemoptysis: Common Only rarely causes death Not predictive of mortality Massive pulmonary arteries in Eisenmenger syndrome

70 Survival in Eisenmenger syndrome Diller. Eur Heart J 2016;37:1449

71 Treatments as for primary PH Phosphodiesterase type 5 inhibitors Sildenafil, tadalafil Endothelin-receptor antagonists Bosentan, ambrisentan, macitentan Prostanoids Improve exercise capacity, probably improve prognosis

72 Eisenmenger patients in a German registry Diller. Eur Heart J 2016;37:1449

73 Syndromes and CHD Trisomy 21 50% CHD - esp AVSD, also VSD, PDA, ToF, sec ASD Turner syndrome 25-30% CHD - BAV, coarctation Williams syndrome (7q11 deletion, elastin gene) 55-80% - supra-valvar AS, supra-valvar PS Noonan syndrome 50-80% - mainly PS & HCM 22q11 deletion (Di George) 75-80% CHD mainly ToF, truncus arteriosus, VSD, arch anomalies

74 No easy way to summarise all that Good luck! If you might be interested in ACHD as a career let me know, james.oliver5@nhs.net