先天性心脏病 Congenital Heart Disease (CHD)

1. 先天性心脏病Congenital Heart Disease (CHD) （二） Department of Pediatrics Soochow University Affiliated Children’s Hospital

2. PatentDuctus Arteriosus(PDA)动脉导管未闭

3. Learning objectives You should: • Know the signs , symptoms, diagnostic features and management of the common acyanotic congenital heart disease: PDA

4. PDA—concept 1 • Which is ductus Arteriosus? 2. Ductus close in response to the rise in Po2 ,blood pH and prostacyclin after birth 3. If this mechanism fails or is reserved by prostaglandin E2, the resulting connection allows blood to flow under pressure from the aorta into the pulmonaryarteries

5. PDA—concept 2 • In a term infant ，ductus ateroisus closed spontaneously in 3 months in most infant cases. • Ductus arteroisusremained patent after one year old or more– named PDA

6. PDA—concept 3 1.In a term infant ，PDA is the result of a deficiency in the structural framework of the vessel wall. 2. In the preterm infant is the result of a delay in closure. Therefore, although 100% of premature babies born at 29weeks of gestation will have a PDA, in the vast majority this closes spontaneously. 3. In contrast, 6% of all term newborn have a persistent connection between the bifurcation of the pulmonary arteries and the aortic arch.

7. PatentDuctus Arteriosus(PDA) • L--R shunt CHD • 10% of CHD • Twice as common in females as in males • In preterm infant weighing less than 1500Kg,the frequency of PDA :20%-60% • Associated lesions CoA ,or VSD(sometimes)

8. Types of PDA funnel漏斗 tubiform管状 window窗型

9. PV LA LV AO Systemic circulation PDA RA Pulmanory circulation PA RV SVC IVC Hemodynamics changes • The blood in lung field increased, Blood in systemic circulation decreased • Pulmonary hypertension(PH) ,reversible -------- irreversible Eisenmenger syndrome • Cardiac enlargement (LV,LA) • Diameter of ascending aorta is large to normal • A widened Pulse Pressure

10. Symptoms (depending on the shunt of PDA) • None (most common) • recurrent chest infections • Heart failure with large shunt

11. Signs (depending on the shunt of PDA) • None (most common) • Pink, normal or large volume, bounding /collapsing pulse • BP shows wide Pulse Pressure • Precordium is hyperdynamic with LV impulse at apex • Thrill at left infraclavicular area and second left intercostal space possible • Loud P2 with pulmonary hypertension • Third heart sound (S3) with CCF • Pulmonary crepitations and hepatomegaly with CCF • Continuous waterwheel/machinery murmur loudest at upper LSE, left infraclavicular area and back

13. Investigation • Chest X-ray (CXR) • Electrocardiography(ECG) • Echocardiography(2DE) • Cardiac catheterization and angiocardiography

14. Chest X-ray Pulmonary plethora The main pulmonary artery segment dilated Cardiomegaly (LV,LA) Diameter of ascending aorta is Large to normal 4 2 1 3 PDA case Normal

15. ECG (typical PDA) Normal or left axis deviation LA enlarged , LV hypertrophy

16. Echocardiography • The anatomic location (the size and shunt of PDA) • Color flow doppler (the direction of the shunt) • estimate the pressure pulmonary pressure or hypertension

18. Cardiac catheterization and angiocardiography

19. Course and prognosis • Closure spontaneously in infant in the vast majority • Adults with corrected defect have normal quality of life

20. Management • Medical management 1.fliud restriction 2.indomathacin and prostacyclin • Interventional therapy 1.Implantation of various umbrella or coil device 2.The first choice of treatment • Surgery ligation in premature infant

21. Device for PDA closure Amplatzer occluder device Diameter of PDA>2.5mm. Coiloccluder device (弹簧圈） Diameter of PDA<2.5mm.

24. Summary • PDA is a kind of L to R shunt CHD, • The symptom of PDA depends on the shunt. • The characteristic heart murmur and P2 • Complication: (1)Respiratory infection (2)congestive heart failure (3)endocarditis

25. Summary • PDA can close spontaneously in infant • Enlarged chambers (LV,LA) can be observed by CXR , 2DE ,and ECG • Preventing PH is the key point during the management of PDA patients

26. Question • How to detect and estimate the PH in PDA patient in clinical experience? Why? • Important Concept: • Pulmonary hypertension • differential cyanosis (Eisenmenger syndrome) • A widened Pulse Pressure

27. Tetralogy of Fallot (TOF)法洛四联症

28. Learning objectives You should; • Know the signs , symptoms, diagnostic features and management of the commonest cyanotic congenital heart disease-TOF

29. Questions for TOF 1.The mechanism and clinical findings of hypercyanotic episode (spells)? How to treat it? 2. The mechanism of squatting suddenly in TOF patient?

30. Anatomy of TOF The aorta straddles both L and R ventricle 2 Boot-shaped heart Beneath the aortic outlet 1 3 (RVOTO) 4 Resulting from RVOTO Bay(隐凹) /Oligaemia (血量减少)

31. PV LA LV AO systemic VSD Over-riding RVH RA pulmanory PA SVC RVOTO Hemodynamics changes The blood in lung field decreased (oligemia) Cardiomegaly (RV,RA) Diameter of ascending aorta is larger to normal.

32. Symptoms depending on the degree of RVOT obstruction • Cyanosis (variable, progressive) • hypercyanotic episode /blue spells / Hypoxemic spells 缺氧发作 aged 2years or less • Squat suddenly after exertion to ward off hypercyanotic spells • Exercise tolerance poor

33. Cyanosis (variable, progressive) 1.At birth the RVOT obstruction is usually not severe and cyanosis may not be obvious. 2.but this becomes evident with increasing activity, often when crawling commences around 10 months of age 3.Progressive hypoxemia results in compensatory polycythaemia, including clubbing fingers and toes (杵状指、趾）

34. Symptoms depending on the degree of RVOT obstruction • Cyanosis (variable, progressive) • hypercyanotic episode /blue spells / Hypoxemic spells 缺氧发作 aged 2years or less • Need to lie down/ Squat suddenly after exertion to ward off hypercyanotic spells • Exercise tolerance poor

35. Squat after exertion Need to lie down/ Squat suddenly after exertion to ward off hypercyanotic spells

36. Symptoms depending on the degree of RVOT obstruction • Cyanosis (variable, progressive) • hypercyanotic episode /blue spells / Hypoxemic spells 缺氧发作 aged 2years or less • Squat suddenly after exertion to ward off hypercyanotic spells • Exercise tolerance poor Need to lie down/

37. Hypoxemic spells(缺氧发作) • Paroxysmal hypercyanotic episodes arise in untreated young children aged less than 2 years, • Following defecation 排便，crying or feeding . • Blue spells are characterised by • 1.Increasing irritability • 2.Prolonged crying • 3. Rapid deep respiratory movement • 4.A dramatic exacerbation of cyanosis • During blue spells, a significant increase in RVOT obstruction, blood flow through the outflow decrease ,and the systolic murmur disappears. (mechanism)

38. signs depending on the degree of RVOT obstruction • Central cyanosis • Plethoric appearance • Hyperdynamic precordium with RV heave at left sternal edge • Palpable systolic thrill at upper LSE in50% patients • S2 aortic and single ;(due to absent pulmonary component) • Heart murmur: GradeⅡ--Ⅳ /Ⅵ rough ESM at upper LSE radiating to back

39. Practice ：typical murmur of TOF e.g. Grade Ⅱ--Ⅳ /Ⅵ ESM, P2 weaken or disappeared • Loud ESM at the upper LSE due to turbulence caused by the infundibular stenosis ESM • The large VSD little turblence and therefore does not produce a murmur.

40. Complications of TOF • Progressive cyanosis is associated with failure to thrive • Hypercyanotic spells may be associated with syncopal attacks • Cerebral ischaemia and thromboses usually occur in the first 2 years of life • Cerebral abscess develop in older children • Bacterial endocarditis and CCF are rare

41. Investigation • Blood routine Erythrocytosis , hyperglobulism and plasmahyperviscositysyndrome Avoiding dehydration such as diarrhea, vomiting and sweating • Chest X-ray (CXR) • Electrocardiography(ECG) • Echocardiography(2DE) • Cardiac catheterization and angiocardiography 红细胞增多

42. Chest X-ray 4 Normal TOF: Boot-shaped heart 2 3 1 Pulmonary oligaemia Small pulmonary conus, (concave) Cardiomegaly (RV,RA) Diameter of ascending aorta is larger

43. ECG (typical TOF) Right axis deviation RV hypertrophy

44. Echocardiography • The anatomic location • Color flow doppler the direction of the shunt • estimate the pressure gradient of RVOT

45. VSD VSD Over-riding ventricle septum Over-riding ventricle septum VSD RV outflow obstruction

46. Cardiac catheterization and angiocardiography

47. Medical management • Attempts to improve weight gain are essential • An adequate haemoglobin should be maintained ,especially in patients with severe cyanosis and those with hypercyanotic spells • Emergent treatment for.hypercyanotic spells 1.placed knee to chest position (stimulated squatting) 2.Given oxygen 3.intravenous sodium bicarbonate (acidosis 酸中毒) 4.Intravenous morphine (sedation, relief pain and RVOTO) 5. Regular oral Propranolol (心得安) until surgery

48. Management---Surgery • The palliative blalock-Taussig shunt improves pulmonary blood flow ,It is employed in severely cyanosed infants aged less than 6 months ,those who are medically unfit for a major procedure, and those with hypercyanotic spells • The definitive repair involves total reconstruction of the RV outflow tract and closure of VSD, The operative mortality is less than 5%

49. Summary • The commonest cyanotic CHD, • R to L shunt • The typical symptom : 1. Cyanosis after the neonatal period 2. Hypercyanotic spells during infancy 3. Squatting suddenly after infancy • The characteristic heart murmur and P2 decreased