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Flashcards in Congenital heart disease Deck (110):
1

Most common congenital malformation and incidence

Congenital heart disease 7-8/1000

2

Anomaly with fetal lithium exposure

Ebstein's anomaly

3

CHD that typically presents in the newborn period (4)

Coarctation of the aorta Transposition of the great arteries Tetralogy of fallot Patent ductus arteriosus

4

Defects causing left to right shunt (2)

VSD ASD

5

Obstructive lesions (2)

Aortic stenosis Pulmonary stenosis

6

CHD presenting with shock, %,, murmur

VSD AVSD PDA

7

CHD presenting with a murmur, %, murmur

Pulmonary valve stenosis Atrial septal defect

8

Presenting with cyanosis

Tetralogy of fallot Transposition of the great arteries

9

Paediatric circulation from placenta

oxygenated blood from placenta--> umbilical vein--> ductus venosus--> IVC--> R atrium--> shunted through foramen ovale--> L atrium--> L ventricle--> aorta--> brain/myocardium/ upper extremities

10

Paediatric circulation from deoxygenated blood returning via SVC

deoxygenated blood returns via SVC to R atrium--> 1/3 of blood entering R atrium does not flow through foramen ovale and flows to the R ventricle--> pulmonary arteries--> ductus arteriosus--> aorta--> systemic circulation placenta for reoxygenation

11

Most critical time for fetal heart development

Critical stage at 3-8 weeks

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What is the shunt for deoxygenated blood

Ductus arteriosus

13

What are the shunts for oxygenated blood (2)

Foramen ovale Ductus venosous

14

What does the ductus venosous connect

Umbilical vein and IVC->bypassing the liver

15

Changes in circulation at birth

1. First breath->lung open-> -ve pulmonary resistance= +pulmonic blood flow 2. Separation of low resistance placenta->systemic circulation becomes high resistance system -> ductus venosus closure 3. Increased pulmonic flow -> +left atrial pressure->foramen ovale closure 4. +oxygen concentration in blood after first breath= -ve prostaglandins-> ductus arteriosus closure 5. Closure of fetal shunts and changes in vascular resistance->infant circulation assumes normal adult adult flow

16

Epidemiology, common presentations, most common lesio

Number of live briths: 8/1000 Heart murmur Heart failure Cyanosis VSD most common

17

Investigations

Echo ECG CXR

18

At what concentration of deoxy Hb does cyanosis occur

At least 3g/dL

19

Pathogenesis of acyanotic

acyanotic heart disease: (i.e. L to R shunt, obstruction occurring beyond lungs) blood passes through pulmonic circulation g oxygenation takes place --> low levels of deoxygenated blood in systemic circulation --> no cyanosis

20

Division of acyanotic HD

1. Left to right shunt: ASD, VSD, PDA, AVSD 2. Obstructive->CA, AS, PS

21

Division of cyanotic

1. R->L shunt: TOF, ebstein's anomaly 2. Other: TGA, total anomalous pulmonary venous drainage, tricuspid atresia

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Complications/progression of L to R shunt

Pulmonary vascular disease Left ventricular dilatation and dysfunction RV hypertension and hypertrophy R to L shunts

23

Types of ASD

ostium primum (common in Down syndrome), ostium secundum (most common type, 50-70%), sinus venosus (defect located at entry of superior vena cava into right atrium

24

How common are ASD

Responsible for 5% CHD

25

Spontaneous closure rate of ASD

Spontaneous closure in 80-100% when

26

History of ASD

Asymptomatic common in childhood May present with HF / pulmonary hypertension later in adulthood

27

Physical examination in ASD: palpation, auscultation

Palpation: Normal, may have RV+ Auscultation: +flow to right, low pitch diastolic, TR, pulmonary ejection murmur Fixed splitting P2 No direct murmur

28

Investigations in ASD

Echo CXR ECG

29

Management of ASD

left-to-right shunt 1st line: observation adjunct: corrective closure adjunct: prophylactic antibiotics->after closure for 6 months (amoxicillin) right-to-left shunt reversible 1st line: corrective closure plus: prophylactic antibiotics irreversible (Eisenmenger's syndrome) 1st line: supportive medical therapy with pulmonary vasodilators->bosentan plus: monitoring and treatment of hyperviscosity plus: prophylactic antibiotics 2nd line: heart-lung transplantation

30

Why do patients with eisenmenger's develop a hyperviscocity

To cope with the hypoxemia

31

Most common CHD

Ventricular septal defect

32

Causes of VSD

Congenital Alcoholism MI

33

Most common location of VSD

Interventricular septum

34

Presentation if small VSD

Asymptomatic, normal growth and development Early/holosystolic murmur, LLSB

35

Investigations in VSD when small and management

CXR and echo are normal Most will close spontaneously

36

Moderate to large VSD: CHF presentation, clinical history, PE findings

CHF by 2 monthsd Pulmonary hypertension Clinically can have delayed growth, -ve exercise tolerance, recurrent URTI/asthma episodes PE: Holosystolic LLSB S3, S4 +on expiration, Loudness ++when small RV heave

37

Investigation findings when moderate VSD

CXR: +pulmonary vasculature, ECG: biventricular hypertrophy Cardiomegaly, CHF

38

Management of moderate VSD

Treat CHF and surgical closure by 1 year old

39

Complications of VSD

1. Endocarditis 2. Progressive aortic regurgitation 3. CHF 4. Pulmonary hypertension and refersal

40

Management of VSD

congenital: small 1st line: observation adjunct: prophylactic antibiotics congenital: medium or large asymptomatic 1st line: corrective closure adjunct: prophylactic antibiotics symptomatic with left-to-right shunt 1st line: preoperative medical therapy plus: corrective closure adjunct: prophylactic antibiotics symptomatic with right-to-left shunt (Eisenmenger's syndrome) 1st line: supportive medical therapy with pulmonary vasodilators plus: prophylactic antibiotics adjunct: monitoring and treatment of hyper-viscosity 2nd line: heart-lung transplantation

41

Functional and anatomical closure of PDA

Functional closure at 15 hours, anatomical closure within first few days

42

When is closure delayed, and is this different to PDA in term

Delayed in prematurity, different to term

43

When is spontaneous closure more common

Premature infants

44

Clinical presentations

If small may be asymptomatic TacyP Failure to thrive SOB +respiratory symptoms of URTI

45

Risk factors

Prematurity Maternal rubella Female

46

Clinical examination findings

TacyP Bounding pulse Wide pulse pressure Hyperdynamic praecordium Systolic thrill Continous machinery murmur

47

Investigations and findings

ECG: deep Q, tall R waves in 2,3,aVF, V5, V6. May have widened P wave suggesting LAE, LVH, RVH CXR: cardiomegaly, +pulmonary vascular markings Echo: Two-dimensional and/or colour Doppler evidence of a PDA. Diastolic forward flow in the pulmonary artery. Left ventricular and/or left atrial enlargement. Diastolic flow reversal in the distal aortic arch

48

When is surgery contraindicated in PDA

When pulmonary disease, right to left shunt

49

Management of PDA: premature, term/children, smptomatic and too small

Premature: Indomethacin (or ibuprofen) in premature Surgery->ligation Term and children: Percutaneous catheter closure Frusemide->if not large enough for surgery just yet and are symptomatic- can give time for them to grow Term/symptomatic and too small for percutaneous device closure: surgical ligation

50

When is surgery for PDA generally indicated

When medical therapy has failed

51

Is indomethacin ever given prophylactically

Yes, in the very low birth weight infants to avoid the hemodynamic challenges

52

What is used if the DA needs to be kept open and in what situation may this be required

Prostaglandin E Transposition of the great arteries

53

How do infants with obstructive lesions present

Pallor Cool extremities Fatigue Reduced urine output Cardiogenic shock

54

Coarctation of the aorta general features

Narrowing of aorta- most commonly at division of left subclavian/ligamentum arteriosa

55

Clinical features

1. HTN in upper extremities with hypotension in lower extremities 2. Well-developed upper body with underdeveloped lower half 3. Midsystolic murmur heard best over the back 4. Symptoms include headache, cold extremities, claudication with exercise, and leg fatigue. 5. Delayed femoral pulses when compared to radial pulses 6. Prevalence of coarctation of the aorta is increased in patients with Turner’s syndrome

56

Risk factors for COA

Strong male gender young age Turner's syndrome DiGeorge's syndrome Cardiac defects include tetralogy of Fallot, interruption of aortic arch, aortic coarctation, truncus arteriosus, and ventricular septal defects. hypoplastic left heart syndrome Shone's complex PHACE syndrome

57

Features of PHACE syndrome

A rare syndrome in which the association: 1. Posterior fossa malformations, 2. Haemangioma, 3. Arterial anomalies, 4. Cardiovascular anomalies 5. Eye abnormalities

58

CHD seen in Digeorge

Tetralogy of Fallot, interruption of aortic arch, aortic coarctation, truncus arteriosus, and ventricular septal defects.

59

Investigations and findings in COA

1. ECG shows LVH. 2. CXR a. Notching of the ribs b. “Figure 3” appearance due to indentation of the aorta at site of coarctation, with dilation before and after the stenosis 3. Echo: discrete narrowing in the thoracic aorta; pressure gradient across narrowing

60

Complications of COA

Severe treatment resistant hypertension Rupture of cerebral aneurysms Infective endocarditis Aortic dissection/aneurysm

61

Management: critical in neonates, non critical 1y, recurrent

Critical: alprostadil + surgical repair Non critical: 1y: surgical repair or percutaneous angioplasty with or without stent implantation Recurrent: percutaneous angioplasty

62

Types of aortic stenosis

1. valvular (75%) 2. subvalvular (20%), 3. supravalvular 4. idiopathic hypertrophic subaortic stenosis (IHSS) (5%)

63

Clinical presentation of AS

presence of risk factors dyspnoea chest pain syncope

64

Risk factors for AS

Strong age >60 years congenitally bicuspid aortic valve rheumatic heart disease chronic kidney disease->calcification

65

Examination findings AS

systolic murmur ≥3/6, with a diamond-shaped crescendo-decrescendo pattern that peaks in mid-systole and radiates to the carotid arteries. RUSB A2 prolonged Expiration, the pulmonic sound (P2) is heard before A2 in severe

66

Can people with AS bleed more

Yes, can develop an acquired vWD

67

Grading of murmurs

Murmurs are generally graded on a scale of 1 to 6: Grade 1: murmur is faint and heard only with effort Grade 2: murmur is faint but easily detected Grade 3: murmur is loud Grade 4: murmur is very loud and associated with a palpable thrill Grade 5: murmur is so loud that it can be heard with only the edge of the stethoscope Grade 6: murmur is extremely loud and heard even when the stethoscope is no longer in contact with the patient.

68

Severity of AS classification

Mild: Jet velocity 1.5 cm^2 Moderate: Jet velocity 3.0 to 4.0 m/second Mean pressure gradient 25 to 40 mmHg Valve area 1.0 to 1.5 cm^2 Severe: Jet velocity >4.0 m/second Mean pressure gradient >40 mmHg Valve area

69

Investigations and findings in AS

Transthoracic echo: elevated aortic pressure gradient; measurement of valve area and left ventricular ejection function ECG: may demonstrate left ventricular hypertrophy and absent Q waves, AV block, hemiblock, or bundle branch block

70

Management overview AS

1. Surgical valve replacement 2. Anticoagulation 3. Endocarditis prophylaxis

71

General characteristics of TOF

Overriding aorta Pulmonary steonsis VSD Right ventricular hypertrophy

72

Clinical features in TOF

Cyanosis Tachypnoea Tet spells Murmur is typically crescendo–decrescendo in nature and heard best at the left upper sterna border. Parasternal impulse, heave

73

What are Tet spells and what is their purpose

Patient with TOF will squat following exertion (exercise, crying)->+SVR->able to shunt blood to the lungs rather than the aorta

74

Investigations and findings in TOF

Pulse oximetry: low oxygenation CXR: boot shaped Echo: overriding aorta, RVH, pulmonary stenosis, VSD ECG: RVH with a rightwards axis, R in V1 and S in V6 above age-appropriate normals

75

Management of hypercyanotic spells

Manouevres to +venous return->knees to chest Supportive: fluids, morphine to calm, oxygen (but try not to ++activity) 2nd line: beta blockers 3rd line: phenylephrine

76

How are beta blockers thought to help during hypercyanotic spells

helps to decrease the infundibular obstruction by decreasing the heart rate, prolonging diastolic filling, and decreasing contractility

77

How does phenylephrine work to help in hypercyanotic spells of TOF

It increases SVR->shunting more blood to lungs

78

Management of infant with unremitting cyanosis of TOF

surgical shunt + extracorporeal membrane oxygenation (ECMO)

79

Management of infant with severe cyanosis and limited pulmonary flow

Alprostadil and supportive->fluid, morphine, bicarb, oxygen

80

Ongoing/long term management of TOF

Surgical repair Monitoring with possible pulmonary valve replacement Endocarditis prophylaxis

81

Endocarditis prophylaxis

Cephalexin in cardiac or amox/ampicillin preferred for dental

82

What is the most common cyanotic heart disease of the neonate

Transposition of the great arteries

83

Pathophysiology of TGA

parallel pulmonary and systemic circulations ƒƒsystemic: body  RA  RV  aorta  body ƒƒpulmonary: lungs  LA  LV  pulmonary artery  lungs ƒƒsurvival is dependent on mixing through PDA and/or atrial or ventricular septal defects

84

Physical examination in TGA

With closure of the PDA->rapid progression to severe hypoxemia, unresponsive to oxygen therapy VSD: murmur, cyanosis not prominent->progression to CHF in first few weeks No VSD: absent murmur

85

Investigations and findings in VSD

ECG: RAD/RVH, or may be normal CXR: egg on a string appearance

86

Management of TGA

ƒƒsymptomatic neonates: prostaglandin E1 infusion to keep ductus open until balloon atrial septostomy followed by switch procedure ƒsurgical repair: arterial switch performed in the first two weeks in those without a VSD while LV muscle is still strong

87

Commonest cause of death from CHD in first month of life

LV hypoplasia may include atretic or stenotic mitral and/or aortic valve, small ascending aorta, and coarctation of the aorta with resultant systemic hypoperfusion systemic circulation is dependent on ductus patency; upon closure of the ductus, infant presents with circulatory shock and metabolic acidosi

88

Management of Hypoplastic left heart syndrome

Intubate Correct metabolic acidosis IV infusion of alprostadil Surgical palliation or heart transplant

89

Characteristics of innocent murmurs

Systolic and musical No clinical significance Do not radiate Change with position. Soft <3/6. The rest of the examination is normal

90

Venous hum

Blowing continuous in systole and diastole Heard below the clavicles Disappears on lying down

91

Pulmonary flow murmur

Brief high pitched Best heard with child lying down

92

History taking with a murmur

Fatigue Breathlessness Family history Antenatal->maternal medications, syndromes etc

93

Physical examination in murmur

Murmur Signs of heart failure-> FTT, poor growth, tachyC/P, crepitations, hepatomegaly Pulse and BO Sternal heave Cyanosis->unlikely finding in children presenting with a murmur

94

Investigations in CHD

Echocardiograph CXR ECG 24 hr ECG->if palpitations/syncope Cardiac catheterisation

95

What is this murmur

Aortic stenosis

96

What is this murmur

Mitral regurgitations

97

What is this murmur

Aortic insufficiency

98

What is this murmur

Mitral stenosis

99

What is this murmur

ASD

100

What is this murmur

Benign

101

What is this murmur

VSD

102

What is this murmur

PDA

103

What is this murmur

S3

104

What is this murmur

S4

105

What is this murmur

Split S2

106

Why is polycythemia important in cyanosis

Can lead to to more apparent cyanosis

107

What CHD can cause cyanosis in first 24 hours of life

TGA

Pulmonary atresia

Tricuspid atresia

108

Which cyanotic CHD is more common in the diabetic mother

TGA is 20 times more common in diabetic mothers

109

Categories of CHD severity

1. Minor

Little ot no hemodynamic impact/no functional impact

Small VSD

Trivial PVS

Bicuspid aortic valve

Small ASD

2. Moderate

HemoD impact may produce long term complications, intervention required

ASD w/ V dilation

Coarctation >few months of age

VSD impeding flow

Anomalous pulmonary venous return

3. Severe

Major hemodynamic impact

Single ventricle

TGA

TOF

Large VSD

110

Clinical features in early weeks may suggest CHD

1. Cyanosis

2. Dusky

3. Dysmorphic

4. TachyP

5. Poor feeding, poor weight gain

6. Overactive praecordium

7. Murmur

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