Cardiology Department

CYANOTIC

ACYANOTIC

Tetralogy of fallot (TOF) Transposition of great arteries (TGA) Tricuspid atresia Truncus arteriosus Pulmonary atresia

Atrial septal defect (ASD) Venticular septal defect (VSD) Patent ductus arteriosus (PDA) Coarctation of aorta (CoA)

Right to left shunting

cyanotic

Left to right shunting

acyanotic

Cardiology Department

10% of all congenital heart defect

Commonest cyanotic congenital heart disease in children above the age of two years constituting almost 75 % of all blue patients.

Four constituents 1. RVOT obstruction or Pulmonic stenosis 2. Ventricular septal defect (VSD) 3. overriding of aorta, and 4. Right ventricular hypertrophy

Pulmonic stenosis causes an increase in right ventricular pressure

when the right ventricular pressure is as high as the left ventricular or the aortic pressure

right to left shunting occurs

Without surgery
25~35% die in the first year of life 40~50% die by the age of 4 70% by 10 years 95% by 40 years Chronic hypoxemia lead to polycythemia

With complete repair

85% survive to adulthood

Dyspnea on exertion Cyanosis may be present from birth or make its appearance some years after birth Exercise intolerance Cyanotic spell

Patients assume a sitting posture squatting as soon as they get dyspneic. Although squatting is not specific for TOF, it is the commonest congenital lesion in which squatting is noted

 Squatting (a compensatory mechanism) is uniquely

characteristic of a right-to-left shunt that presents in the exercising child. Squatting increases the peripheral vascular resistance, which diminishes the right-to-left shunt and increases pulmonary blood flow. called cyanotic spellsor Fallots spells.

Child becomes more cyanosed while crying, these are

Due to "spasm" or contraction of a band of muscle in the right ventricle just under the pulmonary valve. When this muscle contracts, it further narrows the channel for blood flow into the lungs. As a result, oxygen delivery becomes further reduced. This causes a spell

Cyanosis clubbing systolic thrill Normal first sound Single second sound and An ejection systolic murmur.

The flow from the right ventricle into the pulmonary artery occurs across the pulmonic stenosis producing an ejection systolic murmur

more severe the pulmonic stenosis, the less the flow into the pulmonary artery and the bigger the right to left shunt more severe the pulmonic stenosis, the shorter the ejection systolic murmur and more the cyanosis

The right ventricular outflow obstruction results in the delay in the P2 since the pulmonary artery pressure is reduced, the P2 is also reduced in intensity the late and soft P2 is generally inaudible in TOF the S2 is , therefore, single and the audible sound is A2

since the aorta is somewhat anteriorly displaced, the audible single A2 is quite loud the ascending aorta in TOF is large and may result in an aortic ejection click

ECG : right axis deviation with right ventricular hypertrophy

Polycythemia ( increase Hb and packed cell volume)

Boot shaped heart (it means apex is lifted up & there is a concavity in the region of pulmonary artery) Oligaemic lung fields Hilar vessels are few, lung vessels also few

Identify the large overriding aorta, right ventricular hypertrophy and outflow obstruction.

Cardiac catherization CT Angio Cardiac MRI

Cyanotic spell : Knee chest position to increase aortic resistance. The increased aortic and left ventricular pressure reduces the rush of blood through the septal hole from the right ventricle and improves blood circulation to the lungs, decreasing the right to left shunt thus decreasing the amount of deoxygenated blood entering the systemic circulation. Oxygen through a face mask to increase the amount of oxygen in the blood.

Beta-blockers such as propranolol Morphine to reduce ventilatory drive Correction of anemia Consider urgent BT shunt

Palliative treatment Definitive treatment

Blalock-Taussig operation : connection between the right subclavian artery, and the right pulmonary artery, which increases the amount of red oxygenated blood reaching the lungs, relieving cyanosis. Pott`s shunt : descending aorta is anastomosed to the pulmonary artery Waterstont`s shunt : ascending aorta right pulmonary artery anastomosis

Total correction: The hole in the ventricular septum is closed with a patch and the obstruction to right ventricular outflow, pulmonic stenosis, is opened. These corrections allow blood flow to the lungs for oxygenation before being pumped out into the body.

Cardiology Department

An atrial septal defect is an opening in the atrial septum, or dividing wall between the two upper chambers of the heart known as the right and left atria. 10-15 % of all congenital cardiac anomalies .

1-Ostium secundum defect 2-Ostum primum defect 3-sinus venosus defect 4-coronary sinus septal defect

70% . 20% 10% < 1%

A defect in the region of the fossa ovalis The most common form of ASD Female: male 3:1 incidence.

Associated lesion : Holt- Oram-syndrome.

Holt-Oram syndrome (HOS) is a heartupper limb complex malformation with an autosomal dominant inheritance.

Musculoskeletal defects:
Upper limbs are affected.

Hypoplasia of the radius

The most common defects are radial thumb anomalies ranging from absent thumbs to displaced (distally placed), duplicated, or triphalangeal thumbs.

right hand of a 6-monthold infant with Holt-Oram syndrome, showing hypoplastic right thumb.

RA receives blood from SVC, IVC & LA Large amount of blood flow through the right side of the heart cause enlargement of RA and RV & dilatation of the PA

Recurrent chest infections Fatigue Shortness of breath Poor growth or failure to thrive

Parasternal heave Wide fixed split s2 Accentuated p2 ESM at left 2nd & 3rd interspaces

Pulmonary hypertension Recurrent chest infections Paradoxical embolism Heart failure Arrythmias

RAD RSR in lead V1 RV hypertrophy RA enlargement

Mod. Cardiomegaly RA enlargement RV enlargement Prominent MPA Plethoric lung fields

Increased right ventricular end diastolic dimensions. Location and size of ASD. Confirmation of the shunt.

Trans-esophageal ECHO (TEE) Further anatomic definition and to determine the suitability for device closure Cardiac catheterization Assess pulmonary hypertention

1-All symptomatic patients. 2- Asymptomatic patients with QP:QS ratio of at least 2:1. 3 - Paradoxical embolism

Medical therapy Diuretics, rate control and anticoagulation in Af Percutaneous device closure Secundum ASD, < 40 mm defect Surgical closure If not suitable for device closure

Thanks