Congenital Heart Disease

Question 1

Ventricular septal defect =

Answer 1

One or more openings in the inter-ventricular septum, allowing a blood shunt from the left ventricle to the right ventricle

Question 2

Locations of the VSD =

Answer 2

1. membranous
2. trabecular=muscular
3. infundibular=outlet=conal
4. inlet septum=atrioventricular canal type

Question 3

VSD symptoms:

Answer 3

The smaller the shunt , the less likely the symptoms and signs. 
Symptoms and signs : 
Exertional dyspnea 
Heart failure
Pulmonary infection 
Cyanosis

Question 4

Signs of VSD :

Answer 4

Essential :
Protosystolic/ holosystolic harsh murmur (centrifugally radiated over anterior chest)
+/- lower left parasternal thrill
Optional:
apical mid diastolic murmur( large volume shunt )
Early diastolic aortic regurgitation murmur
Central cyanosis & Clubbing appear after 10 years ( indicate Eisenmenger syndrome)

Question 5

VSD on ECG:

Answer 5

• normal in small VSD
• LVH +/- LAH in moderate
• biventricular Hypertrophy -/+ left atrial hypertrophy in large VSD
• RVH in VSD complicated by Eisenmenger syndrome

Question 6

Some facts about VSD :

Answer 6

• small muscular VSD in child probably will close spontaneously
• large VSD complicates early by heart failure

Question 7

Management of VSD :

Answer 7

• treatment of HF
• prevention of endocarditis
• closure device for membranous VSD with left to right shunt

Question 8

Surgical treatment of VSD:

Answer 8

• emergency surgery in case of HF in small child

- elective surgery when pulmonary to systemic output ratio Qp/Qs>1.6

Question 9

Contraindication for surgery in VSD :

Answer 9

Pulmonary resistance / systemic vascular resistance >0.5

Question 10

Complication of surgery in VSD :

Answer 10

Rezidual shunt
3rd degree AVblock
RBBB

Question 11

Patent ductus arteriosus=

Answer 11

Absence of the physiologic obliteration of the ductus arteriosus, soon after birth. This ductus lays between the descending thoracic aorta and the proximal left pulmonary artery branch

Question 12

Management of PDA :

Answer 12

• The arterial ductus closure in infants is tempted using INDOMETHACIN.
• Closure device
• prophylaxis of the infective endocraditis

Question 13

Surgical treatment of PDA:

Answer 13

Indication -> any PDA as long as Qp/Qs> 1.5 and the pulmonary vascular resistance lowers during vasodilator administration

Question 14

Contraindication for surgical treatment of PDA:

Answer 14

1) Eisenmenger syndrome

2) any cardiac anomaly whose survival is critically dependent on the patency of PDA

Question 15

Complications of surgical treatment of PDA :

Answer 15

Left recurrent nerve damage
Phrenic nerve damage
Residual shunt
Late repermeabilisation

Question 16

Congenital heart diseases whose survival depends critically on the permeability of the arterial ductus :

Answer 16

1) pulmonary atresia with intact interventricular septum
2) severe pulmonary stenosis
3) severe aortic coarctation
4) left heart hypoplastic syndrome
5) transposition of the great arteries with intact septae

Question 17

Tetralogy of Fallot =

Answer 17

The combination of pulmonary stenosis , large membranous VSD , overriding aorta ( misplaced) and RVH

Question 18

Symptoms of TeF:

Answer 18

-cyanosis at birth
-dyspnea upon exertion “hypoxic spell”
Triggered by feeding, crying, defecation , physical exercise.
**the child learns to alleviate the hypoxic spell by “squatting” position.

Question 19

Signs of Tef:

Answer 19

Essential: 
Central cyanosis
Clubbing 
Upper left parasternal systolic ejectional murmur -/+ systolic thrill
Single second heart sound 
Optional: PDA murmur

Question 20

ECG on TeF:

Answer 20

RVH , RAD, -/+ RAH

Question 21

ChXR and Echocard.:

Answer 21

CXR= normaly sized cardiac silhouette, “coeur en sabot”, lower right cardiac border bulging , sparse pulmonary vascular markings 
Echo= large,  nonrestrictive VSD, infundibular pulmonary stenosis , aortic root overriding the IVseptum, RVH

Question 22

Management pf TeF:

Answer 22

For infants who cannot do the sqautting position , parents have to place the baby on his side and raise his knees to the chest then call 112
Hospital: O2, Ketamine , propanolol , morphine and sodium bicarbonate. Antibiotic prophylaxis against infective endocarditis

Question 23

Surgical treatment of TeF :

Answer 23

1) palliative surgical shunt to increase pulmonary blood flow
2) definitive correction

Question 24

Complications of surgical treatment of TeF :

Answer 24

Periooperative bleeding in adults 
Pulmonary regurgitation
RBBB 
3rd degree AVB
Residual shunt at the VSD closure level

Question 25

Pulmonary stenosis=

Answer 25

Reduction of the cross section area of any of the segments of the pulmonary infundibulum or of the pulmonary artery trunk or its branches. Normal pulmonary valve opening area is > 2cm2 /m2

Question 26

Classification of pulmonary stenosis:

Answer 26

1) valvular ps ( most common) 2) subvalvular ps 3) supravalvular ps

Question 27

Sympt. And signs of PS:

Answer 27

Sympts: *the larger the PS the less likely the symptoms Exertional dyspnea , chest pain and cyanosis Signs: *newborn with severe PS may be cyanotic * other babies have the normal skin tint * lower left parasternal ejection click * harsh upper parasternal systolic murmur

Question 28

ECG and CXR of PS:

Answer 28

ECG: RVH, RAH, may also be LVH CXR: RVH, RAH , post stenotic dilation of the pulmonary artery branches , diminished pulmonary vascular markings .

Question 29

TTEcho. Of PS :

Answer 29

Valvular ps: “En dome “ opening of the dysplastic valve Post stenotic dilatation of the pA branches RVH, peak transvalvular systolic pressure

Question 30

Management of PS:

Answer 30

- in newborn with sever PS , the PDA must be kept open , until the urgent surgical correction is done - endocarditis prophylaxis - physical exercise is restricted in severe PS

Question 31

Surgical treatment of PS :

Answer 31

Indications : a) valvular PS with peak pressure gradient >80mmhg b) non valvular PS