Presentation is loading. Please wait.

Presentation is loading. Please wait.

Cyanotic congenital heart disease

Similar presentations


Presentation on theme: "Cyanotic congenital heart disease"— Presentation transcript:

1 Cyanotic congenital heart disease

2 Classification of congenital heart diseases
Group I : Left to right shunts Group II: Right to lefts shunts Group III: Obstructive lesions

3 Left to right shunts Atrial Septal Defect Ventricular Septal Defect
Patent Ductus Arteriosus

4 Right to Left Shunts Tetralogy of Fallot
Tricuspid atresia Ebstein’s anomaly Transposition of Great Vessels Truncus Arteriosus Total Anomalous Pulmonary Venous Return (TAPVR)

5 Obstructive Lesions Aortic stenosis Coarctation of the Aorta
Pulmonic Stenosis

6 Cyanotic heart disease
Right to Left Shunt

7 Cyanotic heart disease
bluish or purplish tinge to the skin and mucous membranes. 5 g/dL of unoxygenated hemoglobin in the capillaries  This usually correspond to oxygen saturation of 70-80%

8 Who is this guy?

9 ÉTIENNE-LOUIS ARTHUR FALLOT!
 a French physician, 1888 Fallot accurately described in detail the four anatomical characteristics of tetralogy of Fallot.

10 Tetralogy OF Fallot Most common cyanotic heart disease! 75%!

11

12 TOF 4 component! Imagine this is a HEART!

13 TOF Vetricular Septal Defect

14 TOF Vetricular Septal Defect Pulmonic Stenosis

15 TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta

16 TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta Right Ventricular hypertrophy

17 TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta Right Ventricular hypertrophy Concentric R ventricular hypertrophy without cardiac enlargement

18 TOF Vetricular Septal Defect Pulmonic Stenosis
Overriding of dextroposed aorta Right Ventricular hypertrophy Concentric R ventricular hypertrophy without cardiac enlargement Increase in right ventricular pressure*

19

20 RV and LV pressures becomes identical

21 There is little or no L to R shunt
RV and LV pressures becomes identical

22 Hence, VSD is silent There is little or no L to R shunt RV and LV pressures becomes identical

23 Right ventricle into pulmonary artery across pulmonic stenosis producing ejection systolic murmur
Hence, VSD is silent There is little or no L to R shunt RV and LV pressures becomes identical

24 Hence, the more severe the pulmonary stenosis

25 Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt

26 Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery

27 Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery Shorter the ejection systolic murmur

28 Hence, the more severe the pulmonary stenosis
The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery Shorter the ejection systolic murmur More cynosis because of less flow to the lung!

29 Hence, Severity of cyanosis is directly proportional to the severity of pulmonic stenosis Intensity of the systolic murmur is inversely related to the severity of pulmonic stenosis

30 Congestive failure never occur* because…
Right ventricle is effectively decompressed because of the ventricular septal defect. * exception

31 Congestive failure never occur* because…
Right ventricle is effectively decompressed because of the ventricular septal defect. Anemia Infective Endocarditis Systemic hypertension Unrelated myocarditis complicating TOF Aortic or pulmonary valve regurgitation * exception

32 Pulmonary obstruction results in delayed P2

33 Pulmonary artery pressure reduce
Pulmonary obstruction results in delayed P2

34 P2 become soft or inaudible
Pulmonary artery pressure reduce Pulmonary obstruction results in delayed P2

35 Since P2 is inaudible, hence S2 = A2 + P2 [S2 is single sound]
(Second Sound) S2= A2 + P2 Since P2 is inaudible, hence S2 = A2 + P2 [S2 is single sound] Aorta is displace anteriorly too, A2 become LOUD! P2 become soft or inaudible Pulmonary artery pressure reduce Pulmonary obstruction results in delayed P2

36 Ascending aorta in TOF is large, results aortic ejection click

37 Diastolic interval is clear
No S3 No S4

38 Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole

39 Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole “a” waves become prominent in JVP* *but not too tall

40 Clinical Picture Symptomatic any time after birth
Paroxysmal attacks of dyspnea Anoxic spells Predominantly after waking up Child cry Dyspnea Blue Lose conscious Convulsion Frequency varies from once a few days to many attack everyday

41 “tet spell” lethal, unpredictable episodes The mechanism
spasm of the infundibular septum, which acutely worsens the RV outlet obstruction.

42 Dyspnea on exertion Exercise intolerance

43 Sitting posture – squatting
Compensatory mechanism Squatting increases the peripheral vascular resistance, which diminishes the right-to-left shunt increases pulmonary blood flow.

44 Cyanosis during feeding
Poor feeding fussiness, tachypnea, and agitation. Birth weight is low. Growth is retarded. Development and puberty may be delayed.

45 Rarely, patient remain asymptomatic into adult life.

46 Physical examination Clubbing + Cyanosis (Variable) Squatting position
Scoliosis – Common bulging left hemithorax

47 Prominent “a” waves JVP Normal heart size Systolic trill (30%)
Mild parasternal impulse Systolic trill (30%)

48 S1 normal S2 single Murmur Ejection aortic click only A2 heard
P2 soft & delayed: INAUDIBLE Murmur Shunt murmur (VSD) absent Flow murmur: Ejection systolic, the smaller the flow the shorter the murmur Ejection aortic click

49 Retinal engorgement Hemoptysis

50 ECG ECG

51 ECG ECG

52 ECG wiLLiam moRRow ECG

53 ECG Right axis deviation (+120° to +150°)
Right or combined ventricular hypertrophy Right atrial hypertrophy Partial or complete right bundle branch block (especially true of patients after surgical repair)

54

55 Coeur en sabot (boot-shaped heart) secondary to uplifting of the cardiac apex from RVH and the absence of a normal main pulmonary artery segment

56 Normal heart size due to the lack of pulmonary blood flow and congestive heart failure

57 Decreased pulmonary vascularity

58 Right atrial enlargement
Right-sided aortic arch (20-25% of patients) with indentation of leftward-positioned tracheobronchial shadow

59 Echocardiography Reveals a large VSD overriding aorta
variable degrees of right ventricular outflow tract (RVOT) obstruction

60 Course and Complication
Each anoxic spell is potentially fatal Polycytemia Cerebrovascular thrombosis Anoxic infaction of CNS Neurological complication

61 4) LUNG is an awesome filter.
Bypassing it may not be a good idea! TOF, venous blood from gut, peripheral system by pass the lung and re-enter circulation Hence TOF can cause: Brain Abcess Infective endocarditis Paradoxical embolism

62 Management of anoxic spell
Knee chest position Humified O2 Be careful not to provoke the child Especially you are bad at gaining IV access Ask for help from someone more experience Permit the baby to remain with mother Morphine mg/Kg Subcutaneous Correct acidosis – Sodium Bicarb IV

63 Vasopressors: Methoxamine IM or IV drip Correct anemia
Propanolol 0.1mg/kg/IV during spells 0.5 to 1.0 mg/kg/ 4-6hourly orally Vasopressors: Methoxamine IM or IV drip Correct anemia GA is the last resort

64 Palliative Surgery Blalock-Taussig shunt Pott procedure
Waterston shunt

65 Blalock Taussig Shunt Subclavian artery – Pulmonary artery anastomosis

66 Modified Blalock Taussig Shunt
Goretex graft

67

68 Surgical Palliation

69 Palliative operation prolong life
Increase exercise tolerance

70 Definitive operation Closing the VSD Resecting infundibular
90% can return almost normal life after operation Complication: RBBB Residual VSD Residual Pulmonary stenosis Pulmonary regurgitation (pulmonary valve excised) Risk 5%

71 Transposition of Great Areries (TGA)
Aorta originating from the right ventricle, and pulmonary artery originating from the left ventricle Accounts for 5-7% of all congenital heart disease

72 TGA Survival is dependent on the presence of mixing between the pulmonary and systemic circulation Atrial septal defect is essential for survival 50% of patients have a VSD Usually presents in the first day of life with profound cyanosis More common in boys

73 TGA Exam : cyanosis in an otherwise healthy looking baby
Loud S2 ( aorta is anterior ) CXR : Egg on side Narrow mediastinum

74 TGA .. Acute Management PGE-1 with no supplemental O2
Maintain ductus arteriosus patency, this will increase the effective pulmonary blood flow, and thence increase the left atrial pressure, therefore inhance the left to right shunt at the atrial level Balloon atrial septostomy Life saving procedure in the presence of inadequate atrial septal defect

75 TGA .. Surgical Management
Arterial switch with re-implantation of the coronary artery to the new aortic site. Atrial switch : the old style surgery Redirecting the pulmonary and systemic venous return to result in a physiologically normal state The right ventricle remains the systemic ventricle Rarely needed

76 Truncus Arteriosus The presence of a common trunk that supply the systemic, pulmonary and coronary circulation Almost always associated with VSD % of all congenital heart disease

77 Truncus Arteriosus There are different anatomical tupes of truncus arteriosus This is relevant for surgical repair

78 Truncus Arteriosus Generally patients have increased pulmonary blood flow Degree of cyanosis is mild and may not be evident clinically until late stage with pulmonary vascular disease Presenting feature is congestive heart failure (tachypnia, hepatomegally)

79 Truncus Arteriosus Exam is significant for
Single S2 Ejection click of the abnormal truncal valve Systolic murmur of truncal valve stenosis if present Diaastolic murmur of truncal valve insufficiency Gallop CXR : Cardiomegally , increased pulmonary circulation

80 Managment Acute management
No O2 to minimize pulmonary blood flow Diuretics Afterload reduction to inhance systemic blood flow Surgical management: complete repair with VSD closure and conduit placement between the right ventricle and pulmonary arteries Long term problems : truncal valve dysfunction RV conduit obstruction

81 Trcuspid Atresia Complete absence of communication between the right atrium and right ventricle About 3 % of congenital heart disease

82 Tricuspid Atresia There is an obligate interatrial communication
Usually associated with VSD The pulmonary blood flow is dependent on the size of the VSD Pulmonary blood flow can be increased or decreased causing variable presenting symptoms If there is no VSD ( also called Hypoplastic right ventricle) the pulmonary blood flow is dependent on the PDA

83 Tricuspid Atresia- presentation
The presentation will depend on the amount of pulmonary blood flow If the PBF is decreased, the main presenting symptom is cyanosis If the PBF is increased the presentation is that of congestive heart failure CXR will also reflect the amount of pulmonary blood flow

84 Tricuspid Atresia- EKG
Very characterestic : Left axis deviation

85 Management PBF Decreased Increased No O2
Afterload reduction Diuretics PGE-1, and minimal supplemental O2 to maintain ductal patency

86 Surgical Management Single ventricle paliation
First stage : to establish a reliable source of PBF Aorta to pulmonary artery shunt ( BT shunt) Pulmonary arterial banding in cases of increased PBF Second stage: Glenn Anastomosis ( superior vena cava to pulmonary artery Third stage : Fontan anastomosis ( Inferior vena cava to pulmonary artery

87 Total Anomalous Pulmonary Venous Return (TAPVR)

88 TAPVR- Infracardiac

89 Radiography

90 Infracardiac type

91

92 Thank You


Download ppt "Cyanotic congenital heart disease"

Similar presentations


Ads by Google