Name:Abouhassan Abdelrahman

GP:7608M2a
Questions

1. The basic classification of congenital heart disease in adults with pulmonary blood flow
and presence/absence of cyanosis, the risk factors of congenital heart diseases .
According to diffuse cyanosis: tetralogy of Fallot (TOF) is the simultaneous occurrence of the
following four defects:
Right ventricular outflow tract obstruction (RVOTO) due to pulmonary infundibular stenosis
Right ventricular hypertrophy (RVH)
Ventricular septal defect (VSD)
Overriding aorta (the aorta is displaced above the VSD)
Transposition of the great vessels (TGV): anatomical reversal of the aorta and
the pulmonary artery
Other cardiac defects associated with TGV
VSD
Left ventricular outlet obstruction
Abnormal valves and/or coronary arteries
Tricuspid valve atresia ;
Absent or rudimentary tricuspid valve resulting in no blood flow between the
RA and the RV
Patient survival is only possible if there are interatrial and interventricular
communications (see “Other cardiac defects” below).
Ebstein anomaly: malformed tricuspid valve leaflets that are displaced into
the right ventricle with subsequent tricuspid valve regurgitation and right
heart enlargement (known as RV “atrialization”)
Other cardiac defects associated with Ebstein anomaly
Interatrial communication (e.g., PFO, ASD): seen in ∼ 90% of patients
Total anomalous pulmonary venous return (TAPVR): All four pulmonary veins
drain into the systemic venous circulation (e.g., SVC, sinus venosus, RA, IVC)
instead of the left atrium.
Other cardiac defects associated with TAPVR: allow for right-to-left shunting
and the mixing of deoxygenated and oxygenated blood
ASD
PDA
Hypoplastic left heart syndrome (HLHS): spectrum of disease consisting of
severe hypoplasia of the left ventricle with possible stenosis and/or atresia of
the mitral valve, aortic valve, or aortic arch.
2. Mechanisms of hemodynamic changes and their compensation in the atrial septal
defect, ventricular septal defect, patent ductus arteriosus, definition of pulmonary
hypertension, its type and extent, and methods of estimating pulmonary artery
pressure in the clinic.

ASD :

As the pressure in LA is higher than in RA, blood is shunted from LA ¢ RA¢ RV ( causing
RVE ) ¢ PA ( causing its dilatation ) ¢ Lung ( t blood flow
through pulmonary arterioles causing lung plethora & then
pulmonary hypertension ) ¢ The blood from lung comes back
to LA & the cycle is repeated .
- Notice that blood passing from LA to LV will be less than
normal ¢ LCOP.

: PS ¢i the resistance ( afterload) against the RV leading to :

Patent ductus arteriosus :

The aortic pressure (120/80 mmHg) is higher than pulmonary pressure (20/10 mmHg) in both _,
systole & diastole so the blood is shunted from aorta to PA in both systole & diastole
leading to:
● o i blood flow to pulmonary arteries (lung plethora)¢ i blood flow to LA¢ to LV
● causing LVE (later failure)¢ to the aorta causing high COP & high systolic BP. ●
o The escape of blood from the aorta to the PA causes low diastolic BP.
● o High SBP & low DBP ¢ hyperdynamic circulation
● o Later on, pulmonary hypertension & reversal of the shunt occur (Eisenmenger's
● syndrome)

3. Diagnostic value of ECG in these congenital heart defects and ECG changes in case of
hypertrophy of the ventricles and atria.

Right ventricle hypertrophy

Right axis deviation of +110° or more. Dominant R wave in V1 (> 7mm tall or R/S ratio > 1).
Dominant S wave in V5 or V6 (> 7mm deep or R/S ratio < 1). QRS duration < 120ms (i.e.
changes not due to RBBB).

4. Diagnostic criteria for radiography of the chest in these congenital heart defects and
configuration changes of the heart.

Boot shape heart in tetralogy of fallot

The cardiovascular imaging signs of congenital anomalies that are most often seen in radiologic practice
include the egg on a string (seen in transposition of the great arteries), snowman (total anomalous
pulmonary venous return), scimitar (partial anomalous pulmonary venous return), gooseneck
(endocardial cushion

5. Diagnostic value of Echocardiography, in particular dopplerechocardiography, in

congenital heart defects.
 Echo : enlarged chambers

SKILLS AND KNOWLEDGE Echocardiography of congenital and acquired pedi-

atric heart disease is an operator-dependent imaging technique that requires high
levels of technical and interpretive skills to maximize its diagnostic accu- racy.

6. Diagnostic possibilities of invasive methods (catheterization of heart cavities,

angiocardiography x-ray contras
Prenatal diagnosis: Most cases are now diagnosed before birth due to improvements in fetal
echocardiography.
Imaging

Echocardiography (confirmatory test)

Chest x-ray

ECG

Pulse oximetry: ↓ SpO2

Hyperoxia test: helps to distinguish cardiac from pulmonary causes of cyanosis

7. Differential diagnosis of atrial septal defect, ventricular septal defect, patent ductus
arteriosus among themselves, as well as with acquired defects, which are accompanied
by systolic murmur.

● ASD : fixed wide splitting

● VSD : pansystolic murmur
● PAD: machinerary continuous systolic – diastolic murmur

8.Complications of congenital heart disease.

Congenital heart disease complications that might develop years after you receive
treatment include:

 Irregular heartbeats (arrhythmias). Arrhythmias occur when the electrical signals

that coordinate your heartbeat don't work properly. Your heart may beat too fast, too
slowly or irregularly. In some people, severe arrhythmias can cause stroke or sudden
cardiac death if not treated. Scar tissue in your heart from previous surgeries can
contribute to this complication.

 Heart infection (endocarditis). Endocarditis is an infection of the inner lining of the

heart (endocardium). It generally occurs when bacteria or other germs enter your
bloodstream and move to your heart. Untreated, endocarditis can damage or destroy
your heart valves or trigger a stroke. If you are at high risk of endocarditis, it's
recommended that you take antibiotics one hour before dental cleanings. Regular
dental checkups are important. Healthy gums and teeth reduce the risk that bacteria
will enter the bloodstream.
  Stroke. A congenital heart defect can allow a blood clot to pass through your heart
and travel to your brain, where it reduces or blocks blood supply.

 Pulmonary hypertension. This is a type of high blood pressure that affects the

arteries in your lungs. Some congenital heart defects send more blood to the lungs,
causing pressure to build. This eventually causes your heart muscle to weaken and
sometimes to fail.

 Heart failure. Heart failure (congestive heart failure) means your heart can't pump
enough blood to meet your body's needs. Some types of congenital heart disease
can lead to heart failure.

9.The definition of Eisenmenger's syndrome, its causes, pathogenesis, changes of

cardiac hemodynamics, clinic, diagnostics, differential diagnosis with other causes
significant shortness of breath and cyanosis, medication.

Definition :

It is a condition in which a left-to-right shunt in the heart causes pulmonary hypertension,which in

turn ,causes increased pressure in the right side of the heart and reversal of the shunt into a right-
to-left shunt.

Etiology :

• VSD.
•
PDA.

• ASD.

Eisenmenger complex was applied to patients with reversal of shunt in a case ofVSD
by Dr. Victor Eisenmenger in 1897 but the definition was extended by Dr. Paul Wood
to include shunts at any level VSD, ASD, PDA , ..

Clinical picture :

1- History of congenital heart disease : VSD, PDA, ASD.

2- Pulmonary infection & hemoptysis .

3- C/P of pulmonary hypertension .

4- C/P of RSHF.

5- Decrease of the original murmur of the shunt due to low pressure gradient .

Treatment :

• Prevention is best.

• Closure of the defect is contraindicated as it increases the pressure in the right side of the heart.
• Symptomatic treatment e.g. HF. • Heart lung transplantation

10. Indications and contraindications for surgical intervention for these defects.

Contraindications for closure

Irreversible pulmonary vascular occlusive disease (class III) is a contraindication for closure.
Patients with borderline operability due to pulmonary vascular disease should be referred to a
higher center for further evaluation.

Moderate to large defects usually require surgical closure. In the absence of growth delay or severe
pulmonary hypertension, surgery can be postponed until about 2 years of age, as some defects become
smaller with time. However, surgery can be performed anytime beyond the neonatal period with minimal
morbidity or mortality