sign in sign up
<<
Home , Congenital heart defect, Cyanotic heart defect

9/6/2016

FETAL CIRCULATION

CONGENITAL DISEASES 2

SYSTEMIC AND PULMONARY CIRCULATION

CONGENITAL HEART DISEASES 3

1 9/6/2016

CONGENITAL HEART DISEASE (CHD)

Incidence: 10 in 1,000 live births

Subdivisions: .Acyanotic congenital heart disease .Cyanotic congenital heart disease

CONGENITAL HEART DISEASES 4

ACYANOTIC CHD

1. Ventricular septal defect (VSD) 2. (ASD) 3. Patent (PDA) 4. AV Canal 5. 6. Aortic 7. Pulmonary stenosis

CONGENITAL HEART DISEASES 5

VENTRICULAR SEPTAL DEFECT

Bicuspid most common Excluding bicuspid valve congenital heart defect VSD accounts for 25% of all CHD in childhood.

A common concomitant with other forms of congenital heart disease; and

CONGENITAL HEART DISEASES 6

2 9/6/2016

VENTRICULAR SEPTAL DEFECT

CONGENITAL HEART DISEASES 7

VENTRICULAR SEPTAL DEFECT

CONGENITAL HEART DISEASES 8

VSD AS PRINCIPAL DEFECT 30% of cardiac malformations at birth 20% during childhood 10% during adult life

CONGENITAL HEART DISEASES 9

3 9/6/2016

VENTRICULAR SEPTAL DEFECT

VSD is the most common cardiac defect associated with chromosomal abnormalities such as trisomy D & E VSD is the most common defect with other anomalies such as

CONGENITAL HEART DISEASES 10

VENTRICULAR SEPTAL DEFECT – PROGNOSIS

75%-80% of small VSDs may close spontaneously by age 10 10%-15% of large VSDs may close spontaneously Generally large VSDs require surgery Loud, holosystolic murmur along the lower left sternal border

CONGENITAL HEART DISEASES 11

EISENMENGER

A syndrome associated with increased pulmonary vascular resistance (pulmonary ) leading to the right to left shunt in patient with large VSD and or ASD causing .

CONGENITAL HEART DISEASES 12

4 9/6/2016

ATRIAL SEPTAL DEFECT

CONGENITAL HEART DISEASES 13

ATRIAL SEPTAL DEFECT Ostium Primmum ASD: Most commonly associated with Down’s syndrome; the abnormality produces loud pan-systolic murmur due to ostium being located at low level interfering the valvular function of and or . Ostium Secundum ASD: Comprises 70% of all ASD Most common congenital heart disease fount initially in adults excluding . F>M; resulting in systolic ejection murmur at the left sternal border.

CONGENITAL HEART DISEASES 14

ATRIAL SEPTAL DEFECT

Sinus Venosus ASD: 10% of all ASD Associated with anomalous pulmonary venous return, because it occurs high on the septum

CONGENITAL HEART DISEASES 15

5 9/6/2016

ATRIAL SEPTAL DEFECT

CONGENITAL HEART DISEASES 16

PATENT DUCTUS ARTERIOSUS

CONGENITAL HEART DISEASES 17

PATENT DUCTUS ARTERIOSUS

1/2500 – 1/5000 live births 2nd and 3rd most common lesions (12-17%) F:M = 2:1 PDA causes continuous “machinery” murmur at left upper sternal border Differential cyanosis develops (clubbed toes, normal fingers)

CONGENITAL HEART DISEASES 18

6 9/6/2016

PDA

Normal Heart Heart with PDA

CONGENITAL HEART DISEASES 19

RIGHT-TO-LEFT SHUNT

VSD & PDA are volume loads on the left heart . Increased LV size . Increased LA size . Increased PA size

X-ray: LV and LA enlarged . Increased vascularity . Increased PA size EKG: LVH, LAE

CONGENITAL HEART DISEASES 20

LEFT-TO-RIGHT SHUNT ASD is a volume load to the right heart . Increased RV size . Increased RA size . Increased PA size

X-ray: RV and RA enlarged . Increased vascularity . Increased PA size

EKG: Right Axis RVH RAE

CONGENITAL HEART DISEASES 21

7 9/6/2016

COARCTATION

17% of Turner’s Syndrome have coarctation M:F = 2:1 single coarctation M:F = 1:1 complex coarctation

CONGENITAL HEART DISEASES 22

COARCTATION OF AORTA

CONGENITAL HEART DISEASES 23

COARCTATION - COMPLICATIONS

1) Hypertension 2) Congenital bicuspid valve a) Bacterial b) Aortic regurgitation c) Calcified 3) Congestive

CONGENITAL HEART DISEASES 24

8 9/6/2016

COARCTATION – PHYSICAL EXAM

1. Hypertension (primarily systolic) 2. II/VI systolic murmur 3. Absent or decreased femoral pulses with brachial femoral pulse delay 4. Ejection click 5. Notching of the ribs in x-ray

CONGENITAL HEART DISEASES 25

CYANOTIC CHD

1) Tetralogy of Fallot (TOF) 2) Total Anomalous Pulmonary Venous Connection 3) Transposition of the Great Arteries 4) Truncus Arteriosus

CONGENITAL HEART DISEASES 26

TETRALOGY OF FALLOT

CONGENITAL HEART DISEASES 27

9 9/6/2016

TETRALOGY OF FALLOT (TOF)

Incidence: • 11% of all congenital cardiac lesions • Most common cause of cyanotic heart disease in older children • 75% of children over four with cyanotic lesions have tetralogy • “boot shaped” heart

CONGENITAL HEART DISEASES 28

TETRALOGY OF FALLOT: COMPONENTS

1. Pulmonary stenosis (infundibular) 2. 3. Ventricular septa defect (VSD) 4. Right

CONGENITAL HEART DISEASES 29

TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVR)

CONGENITAL HEART DISEASES 30

10 9/6/2016

TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVR)

Pulmonary veins drain into the right , instead of left atrium X-ray: “snowstorm” appearance

CONGENITAL HEART DISEASES 31

TRUNCUS ARTERIOSUS

Single arterial trunk originating from the heart that supplies the systemic, pulmonary, and coronary circulations Present in CHF Low survival rate

CONGENITAL HEART DISEASES 32

TRUNCUS ARTERIOSUS

CONGENITAL HEART DISEASES 33

11 9/6/2016

TRANSPOSITION OF GREATER ARTERIES Pathology:

• Aorta arises from right • arises from left ventricle • Need anatomic connection between left and right heart for survival • Abnormal distribution of coronary arteries arising from aorta Incidence:

• 10% of all cardiac lesions • Male predominance of 2 to 3:1 • Most common in newborns

CONGENITAL HEART DISEASES 34

TRANSPOSITION OF GREATER ARTERIES

CONGENITAL HEART DISEASES 35

TRANSPOSITION OF GREAT VESSELS

Transposition of Great vessels (TGA) - Most common cyanotic cardiac lesion in neonates - X-ray: “egg on a string”

CONGENITAL HEART DISEASES 36

12 9/6/2016

REVIEW QUESTIONS

CONGENITAL HEART DISEASES 37

1.THE MOST COMMON ACYANOTIC CONGENITAL HEART DEFECT IN CHILDREN IS:

a) Atrial Septal Defect b) Ventricular Septal Defect c) Patent Ductus Arteriosus d) Transposition of the Great Vessels e) Tetralogy of Fallot

CONGENITAL HEART DISEASES 38

2. THE MOST COMMON FORM OF CYANOTIC CONGENITAL HEART DISEASE IN CHILDREN, ADOLESCENTS, AND ADULTS IS:

a) b) Univentricular Heart c) Aortic Stenosis d) Tetralogy of Fallot e) Truncus Arteriosus

CONGENITAL HEART DISEASES 39

13 9/6/2016

3. THE MOST COMMON ACYANOTIC FORM OF CONGENITAL HEART DISEASE ASSOCIATED WITH HYPERTENSION IN INFANTS, CHILDREN, AND ADULTS IS:

a) Coarctation of the Aorta b) Ventricular Septal Defect c) Atrial Septal Defect d) Patent Ductus Arteriosus e) Tricuspid Atresia

CONGENITAL HEART DISEASES 40

4.THE MOST COMMON CAUSE OF CYANOTIC CONGENITAL HEART DISEASE IN THE NEWBORN IS:

a) Ventricular Septal Defect b) Atrial Septal Defect c) Transposition of the Great Vessels d) Univentricular Heart e) AV Canal

CONGENITAL HEART DISEASES 41

5.THE MOST COMMON CARDIAC CAUSE OF DEATH IN THE FIRST WEEK OF LIFE:

a. Aortic and Mitral Atresia – Hypo-plastic Left Heart Syndrome b. ASD c. VSD d. Aortic Stenosis e. AV Canal

CONGENITAL HEART DISEASES 42

14 9/6/2016

6. CYANOTIC CONGENITAL HEART DEFECT WHICH DEMONSTRATES INCREASE PULMONARY VASCULARITY IN THE NEWBORN IS:

a) with intact septum b) Tricuspid Atresia c) Severe Pulmonary Stenosis d) Transposition of the Great Vessels e) VSD

CONGENITAL HEART DISEASES 43

7.THE ANATOMIC ABNORMALITY THAT DETERMINES THE PHYSIOLOGY IN TETRALOGY OF FALLOT IS:

a) The size and placement of the ventricular septal defect b) An atrial septal defect c) A right aortic arch d) An overriding aorta e) The degree of valvular and sub-valvular pulmonary stenosis

CONGENITAL HEART DISEASES 44

8. PICK ONE THING NOT ASSOCIATED WITH A PATENT DUCTUS ARTERIOSUS:

a) Prematurity b) Syndrome c) Continuous Murmur d) Volume loaded left heart with left atrial and left ventricular dilatation e) Marfan’s Syndrome

CONGENITAL HEART DISEASES 45

15 9/6/2016

9. THE PATHOGNOMONIC CLINICAL FINDING IN PATIENTS WITH ATRIAL SEPTAL DEFECT IS:

a) Left-to-Right Shunt b) Volume loaded right heart c) Early severe pulmonary vascular disease d) Fixed splitting of the second heart sound e) High altitude pulmonary edema

CONGENITAL HEART DISEASES 46

10. THE FOLLOWING IS TRUE OF AORTIC STENOSIS:

a) A bicuspid aortic valve may remain stable over time, develop aortic obstruction, or become incompetent b) May present in the newborn period with critically ill neonate c) Is commonly associated with coarctation of the aorta d) All of the above e) None of the above

CONGENITAL HEART DISEASES 47

16