Truncus arteriosus is a congenital heart defect where a single large vessel (truncus) arises from the heart instead of the usual separate aorta and pulmonary artery. This occurs when the aorta and pulmonary artery fail to separate during embryonic development. It results in oxygenated and deoxygenated blood mixing together before circulating to the body. Surgical repair is required to separate the truncus into distinct aorta and pulmonary artery.

1. By athraa ali

2. Truncus arteriosus
• Truncus arteriosus is a congenital heart defect that occurs
during fetal development. In a normal heart, the aorta and
pulmonary artery are separate vessels that carry blood away
from the heart to the rest of the body and the lungs,
respectively. However, in cases of truncus arteriosus, there is a
single large vessel (truncus) instead of the usual two separate
vessels.
• This condition occurs when the embryonic heart does not
develop normally, leading to a failure of the aorta and
pulmonary artery to separate into distinct vessels. As a result, a
single vessel arises from the heart, carrying both oxygenated
and deoxygenated blood.

3. symptoms
• Rapid breathing
• Poor feeding and
inadequate weight gain
• Cyanosis (bluish tint to the
skin, lips, and nails)
• Fatigue
• Sweating
• Congestive heart failure

4. diagnosis
• Fetal echocardiography: This test can detect truncus arteriosus
during pregnancy.
• Chest X-ray: To assess the size and shape of the heart.
• Cardiac catheterization: A procedure to examine the blood
vessels and heart chambers.

6. treatment
• The surgical procedure for
truncus arteriosus is called
"repair" or "reconstruction."
During the surgery, the
surgeon separates the
single trunk into two
vessels, the aorta and the
pulmonary artery.
Additionally, a conduit (tube)
may be used to replace the
pulmonary arteries if they
are too small. The goal of
the surgery is to redirect

7. Transposition of great
arteries
• is a congenital heart defect in which
the positions of the pulmonary artery
and the aorta are switched, leading to
two separate circulatory systems. As
a result, oxygen-poor blood circulates
continuously in the body, and
oxygen-rich blood circulates
continuously to the lungs, creating a
life-threatening condition. Usually
present with vsd

9. symptoms
• Postnatal cyanosis (not affected by exertion or supplemental oxygen)
• Tachypnea
• Cardiac examination findings
• Single, loud S2
• There is often no murmur.
• Diminished femoral pulses
• If concurrent VSD is present: systolic murmur at the left sternal
border

10. diagnosis
• Imaging
• Echocardiography (confirmatory test)
• Pulmonary artery arising from the left ventricle
• Aorta arising from the right ventricle
• Chest x-ray
• “Egg on a string” appearance of the heart
• The enlarged, globular heart resembles an egg lying on its side.
• The string represents the superior mediastinum that appears narrow due to stress-
induced thymic atrophy.
• ↑ Pulmonary vascular markings
• MRI (rarely done) showing the aorta arising from the right ventricle and
the pulmonary trunk originating from the left ventricle
• ECG: often normal
• Pulse oximetry: ↓ SpO2

13. treatment
• Surgical repair recommended within the first two
weeks of life
• Arterial switch procedure: reversal of the aorta
and pulmonary artery with insertion into the
anatomically correct ventricle as well as correction
of coronary artery supply , involves cutting off the
aorta and pulmonary arteries just above the point
where they leave the heart. Part of this surgery is
reconnecting them to the proper ventricle. The
valve stays attached to the ventricle, so what was
once the pulmonary valve is now the aortic valve

15. Tricuspid valve atresia
• congenital heart defect in
which the tricuspid valve, does
not develop properly. The
tricuspid valve normally
separates the right atrium
from the right ventricle and
helps direct blood flow through
the heart. In tricuspid valve
atresia, the valve is either
missing or not formed
correctly, leading to significant
problems with blood flow
within the heart.

17. symptoms
• Central cyanosis (occurs within days after birth)
• Tachypnea
• Cardiac examination findings
• Holosystolic murmur at the lower left sternal border
• Single S2
• Jugular venous distention with a prominent A wave
• Diminished peripheral pulses

18. diagnosis
• Imaging
• Echocardiography (confirmatory test)
• Absent tricuspid valve
• ASD
• RV hypoplasia
• Chest x-ray
• Mild cardiomegaly
• Abnormal pulmonary markings
• ECG
• LVH with left axis deviation
• Tall P waves
• Minimal R waves in precordial leads
• Pulse oximetry: ↓ SpO2

20. treatment
1.Norwood procedure: This is the initial surgery performed soon
after birth to create a new pathway for blood flow from the right
atrium to the pulmonary artery.
2.Bi-directional Glenn procedure: This is usually performed
around 4-6 months of age, redirecting blood flow from the upper
body directly to the lungs.
3.Fontan procedure: This is typically performed between 2-4
years of age, completing the redirection of blood flow so that
oxygen-poor blood bypasses the right ventricle and goes
directly to the lungs.

24. Tetralogy of fallot
• 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)

25. etiology
• Typically sporadic
• Associated with the following genetic disorders:
• DiGeorge syndrome
• Down syndrome
• Maternal exposures during pregnancy
• Alcohol consumption
• Phenylketonuria
• Diabetes

26. symptoms
• Cyanosis
• Rapid breathing and shortness of
breath, especially during feeding or
crying
• Poor weight gain
• Fatigue and irritability
• Clubbing of fingers and
• Cardiac examination findings
• Harsh systolic ejection murmur at the left
upper sternal border
• Single S2
• Possible RV heave and systolic thrill

27. diagnosis
• Imaging
• Echocardiography (confirmatory test)
• Detection of the main features of TOF
• Chest x-ray
• Boot-shaped heart (due to upturned cardiac apex and RVH)
• Normal or decreased pulmonary vascular markings
• Concave pulmonary artery segment
• ECG
• Right axis deviation
• Prominent anterior R waves (V1–V2)
• Prominent posterior S waves
• Right atrial enlargement and RVH (P pulmonale)
• Pulse oximetry: ↓ SpO2

29. treatment
• Surgical repair: performed within the first year of life
• VSD repair: patch closure of the ventricular septal defect ensuring
correct aortal positioning above the left ventricle
• Enlargement of the RVOTO: resection of the obstructive infundibular
musculature
• If early surgical management is not possible: palliative shunts
• Blalock–Thomas–Taussig shunt: a palliative surgical procedure for TOF that
connects the subclavian artery to the pulmonary artery with an interposed
graft
• Follow-up care: to prevent long-term complications, such as heart
failure, arrhythmias (e.g., ventricular tachycardia), and neurodevelopmental
impairment

31. Total anomalous pulmonary venous return
• is a congenital heart defect in
which : 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

32. Types & etiology
• The main types include
supracardiac, cardiac,
infracardiac, and mixed.
• Multifactorial (may be associated
with genetic alterations)

33. symptoms
• Cyanosis
• Respiratory failure
• Poor feeding and failure to thrive
• Hepatomegaly
• Cardiac examination findings
• Fixed split S2 or loud S2
• Systolic ejection murmur with diastolic rumble or mild to no murmur

34. diagnosis
• Prenatal diagnosis: fetal echocardiography to detect abnormal pulmonary venous return
• Imaging
• Echocardiography (confirmatory test)
• Malposition of pulmonary veins
• Enlarged right heart
• Right-to-left interatrial shunting
• Chest x-ray
• “Snowman sign” (seen in supracardiac TAPVR): the orientation of
the heart and superior mediastinum appear as the shape of a snowman
• Pulmonary hypervascularity
• Right heart enlargement
• ECG Nonspecific findings
• Right axis deviation due to RVH
• Pulse oximetry: ↓ SpO2

35. treatment
• Initial postnatal management: stabilization of cardiorespiratory status
until surgery is performed (e.g., mechanical ventilation, supplemental
O2, inotropes)
• Surgical repair: recommended in all patients regardless of the severity of disease
• Total anomalous pulmonary venous connection (TAPVC) consists of an
abnormality of blood flow in which all four pulmonary veins drain into systemic
veins or the right atrium with or without pulmonary venous obstruction