acyanotic heart disease in adults reviewpptx

approach to chd in adults

1. Acyanotic congenital heart diseases in Adults Dr Manish Chokhandre Consultant Paediatric Cardiologist

2. Classification of CHD Acyanotic Increase PBF ASD VSD PDA AP window Combined Normal PBF AS/PS Cyanotic Decrease PBF TOF Pulm Atresia Increase PBF TGA TAPVC Truncus arteriosus

3. Atrial Septal Defect 30-40% of CHD in adults (2 nd only to bicuspid AV) F:M – 2-3:1 SV ASD- 1:1

4. ASD- Intro Occurs in different anatomic portion of septum-Further classify Associated defects(PS, pulmonary artery stenosis, VSD, PDA) Functional consequences depends on Size of the defect Presence of cardiac anomalies

5. Types of ASD Secundum ASD Primum ASD Sinus venosus ASDs Coronary sinus ASD PFO

6. Primum ASD (15%) - Cleft AML, ECD Secundum ASD (60-70%) -Prolapse of AML, Multiple defects SV-ASD (10%) - PAPVC/ IVC/SVC Coronary sinus ASD (<1%) - LSVC

7. PFO Not Truly an ASD (no septal tissue missing) Flap closes due to Reduced right heart pressure and PVR Elevated LA pressure Flap closure by 2 years in 70-75 %

8. Pathophysiology of ASD

9. Pathophysiology of ASD

10. RA dilatation RV dilatation Increase PBF/PAH LV underfilled

11. Clinical Features

12. RA dilatation RV dilatation Increase PBF/PAH LV underfilled CHF Fatigue Reduced exercise capacity Increase Hydrostatic pressures in lung Pulmonary oedema Dyspnoea Recurrent RTIs Increase in atrial wall stress Damage to normal conduction tissue Palpitations

13. Physical Findings

14. RA dilatation RV dilatation Increase PBF/PAH Mid-diastolic rumble Precordial Bulge Parasternal Heave Split S2 ESM Loud P2

15. CXR

16. RA dilatation RV dilatation Increase PBF/PAH LV underfilled

17. ECG

18. RA dilatation RV dilatation RAD Tall P waves Prolonged PR Wide QRS/ RBBB

19. Complications/Natural History

20. RA dilatation RV dilatation Increase PBF/PAH LV underfilled CHF Poor compliance of ventricle Increase Hydrostatic pressures in lung Pulmonary oedema Eisenmenger Increase in atrial wall stress Damage to normal conduction tissue Atrial arrhythmias

21. Natural History of ASDs In patients with an ASD less than 3 mm in size diagnosed before 3 months of age, spontaneous closure occurs in 100% of patients at 1½ years of age. Spontaneous closure occurs more than 80% in patients with defects between 3 and 8 mm before 1½ years of age. An ASD with a diameter greater than 8 mm rarely closes spontaneously.

22. Natural History of ASDs Most children with an ASD remain active and asymptomatic. Rarely, congestive heart failure (CHF) can develop in infancy. If a large defect is untreated, CHF and pulmonary hypertension develop in adults who are in their 20s and 30s . With or without surgery, atrial arrhythmias (flutter or fibrillation) may occur in adults. Infective endocarditis does not occur in patients with isolated ASDs . Cerebrovascular accident, resulting from paradoxical embolization through an ASD , is a rare complication.

23. Diagnosis ECHO(Transthoracic/ Transesophageal ) CT scan/MRI Cardiac Cath (Associated lesions/MS/PAPVC/PA pressure estimation/PVR calculations)

24. Management

25. Medical Management Exercise restriction is unnecessary. Symptomatic, anti-congestive therapy with diuretics may be indicated until closure is accomplished.

26. Small less than 3-5 mm shunt- conservative Closure of an ASD is indicated if there is a large shunt. Indicators of a large shunt include Qp:Qs ≥ 1.5 . A diastolic flow rumble in the tricuspid area, ECG evidence of right ventricular hypertrophy, Chest x-ray evidence of cardiomegaly or increased pulmonary vascular markings, or Echocardiographic evidence of right ventricular enlargement and/or paradoxical septal motion.

27. Dilemma

28. Treatment

29. Treatment of OS-ASD Device closure Surgical closure Day care No scar/Pain CPB/Cardioplegia arrest Morbidity associated with Sx MIS/median sternotomy Treatment of Primum ASD Treatment of SV ASD/ Coronary sinus ASD Stenting Transcatheter closure

30. Nonsurgical Closure

31. Surgical Closure

32. Outcome Excellent

33. VSD

34. Ventricular septal defect Ventricular level communication Most common heart disease in children Small VSD reach till adulthood Large VSD  Eisenmenger Male and female equally affected

35. Association Coarctation of the aorta ASD PDA Intracardiac obstructions such as- Subpulmonary or subaortic stenosis Mitral stenosis, and Anomalous muscle bundle of the right ventricle Incompetent atrioventricular valves.

36. Types of VSD

37. Classification: Based on anatomical location A small membranous portion and A large muscular portion: The inlet septum, The outlet septum The trabecular septum: Anterior Posterior Mid Apical

38. The “Swiss cheese” type of multiple muscular defect (involving all components of the ventricular septum) is extremely difficult to close surgically.

39. Classification: Based on size & Shunt

40. VSD Small Moderate Large Pressure Size < 1/3 Aortic root < 1/2 Aortic root =/>Aortic root >2.2:1 1.4-2.2:1 <1.4:1 Qp /Qs Classification of VSD

41. Natural history Approximately 25%-small defects close spontaneously by 18 months, 50% by 4 years, and 75% by 8-10 years. A spontaneous closure rate - 45% within the first 12 to 14 months (uncomplicated perimembranous or muscular VSD). Even large defects tend to become smaller. Defects close by two mechanisms: by muscular septum growth and by “aneurysmal tissue” from a septal leaflet of the tricuspid valve as in the case of perimembranous defects.

42. Natural history (Contd.) A large VSD during childhood is typically associated with significant left-to-right shunt and eventual development of congestive heart failure. Patients with moderate-sized VSDs can survive to adulthood before detection. Given the gradual development of symptoms in these patients, they may not present until late in the disease course. In these patients, the excess right-sided flow may lead to pulmonary vascular disease and Eisenmenger physiology if left untreated.

43. Natural history (Contd.) Endocarditis is a risk because of the presence of a high-velocity, turbulent jet into the right ventricle. Endocarditis most frequently involves the septal leaflet of the tricuspid valve apparatus at the point of jet impact.

44. Pathophysiology

45. VSD RV(Conduit) LA dilatation LV Dilatation Increase PBF/PAH

46. Symptomatology

47. VSD Increase PBF/PAH Increase Hydrostatic pressures in lung Pulmonary oedema Dyspnoea Recurrent RTIs LV dilatation Increase in SVR CHF Fatigue Reduced exercise capacity Growth Restriction

48. Clinical Findings

49. LV heave Palpable apical implse LA dilatation LV Dilatation PAH Increase Flow ESM Loud P2 VSD Small- PSM Large- Nil Increase Flow MD rumble MR/PSM

50. CXR

51. ECG

52. LAE LA Hypertrophy Increase LV forces Biv Hypertrophy Katz-Wachtel phenomenon

53. ECHO VSD-Type, Size Chamber size PAH Associated defects Complications of long standing VSD

54. Cardiac cath /MRI

55. Management

56. Treatment Moderate-Large VSD Small VSD Yearly Fu Surgery/Device closure Symptomatic QpQs ->1.5 PVR < 5 WU Surgery if Aortic valve distortion RV muscle bundle formation IE Outcome -Excellent Asymptomatic QpQs -<1.5 PSM Normal chambers No PAH

57. PDA /APW Increase PBF/PAH LA dilatation LV Dilatation Dyspnoea Recurrent RTIs Fatigue Reduced exercise capacity CHF Growth Restriction Continuous Murmur Loud P2 MDM PSM LV apex/LV heave Wide pulse pressure

58. CXR ECG

59. Diagnosis Clinical CXR ECG ECHO Cardiac Cath/CT

60. Treatment of PDA PDA Silent PDA Small PDA Mod-Large PDA Conservative No Murmur Incidental Echo finding Elective device closure Cont murmur S2 Normal Normal precordium QpQs <1.5:1 Asymptomatic Device/surgery Symptomatic Cont murmur Loud P2 Cardiomegaly Qp /Qs>1.5 Outcome- Excellent

61. VSD

62. Increase PVR RV hypertension RV hypertrophy Hypoxia Cyanosis Clubbing Polycythemia Thrombocytopenia Spontaneous bleeding Stroke,PE,DVT Eisenmenger Syndrome

63. Stenotic Lesions

64. Aortic Stenosis Valve morphology- Bicuspid, Monocuspid

65. Pathophysiology and clinical symptoms

66. Impeded flow across AV ESM Decrease perfusion to brain Syncope LVH Dyspnoea