A RARE UNUSUAL HEART DEFECT – CONGENITTALY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES (CCTGA).
A rare congenital heart
defect which is known as congenitally corrected transposition of the great
arteries causes the lower half of the heart functioning and pumping to be
reversed and it has a variety of morphological presentations and is frequently
asymptomatic.
HOW DOES A HEALTHY/
NORMAL HEART FUNCTION?
The typical human heart
has two sides, blood from the body is pumped by the right side into the lungs.
Blood is pumped out of the lungs and into the body from the left side. An
atrium or the top chamber, and a ventricle or the bottom chamber are present on
either side of the body. The atrium serves as the bloods reception area. The
blood is forcefully pushed out of the ventricle entrance and exit. These valves
let the blood enter the ventricle from one side and push it out to the other
side, ensuring that blood only flows in a single direction. The lungs low
pressure is achieved by the blood being pushed through each ventricle and
valve. In order to move blood to the lungs, which are under low pressure, the
right ventricle is intended. It is larger than the left ventricle and requires
less muscle since only a slight push is needed. The left ventricles purpose is
to force blood outward toward the body, which is under greater pressure.
It has a smaller interior because of its
thick walls, which take up more room. The body’s hardest and strongest valve is
the mitral valve, which connects the left atrium and ventricle. It is made to
remain closed in the face of the bloods powerful push outward toward the body.
Your weaker, larger right
ventricle grows on the left side of your heart. It circulates your blood
throughout your body. On the right side of your heart, your stronger, smaller
left ventricle develops. It transports blood to the lung. Your body’s weakest valve,
the tricuspid valve, also serves as your mitral valve.
SIGNS & SYMPTOMS OF
CCTGA :
Depending on existing
heart issues, people with CCTGA can suffer a wide range of symptoms. There are healthy
persons who can have a normal life for many years without any therapy, and
there are sick neonates with low oxygen levels who require care right away. The
interruption in the conduction process can cause symptoms like dizziness or
exhaustion because the heart cannot pump sufficient blood throughout the body.
Blue discoloration of
skin (cyanosis)
Respiration difficulty
(SOB)
Poor pulse rate (PR)
Insufficient / lack of
appetite
Terrible weight gain.
DIAGNOSIS OF CCTGA :
CCTGA exhibit symptoms of
cardiac arrest, irregular heart beat issues like arrythmias and or heart
murmurs. They could be cyanotic and have a ventricular septal defect. In such
circumstances, a diagnosis is frequently made in childhood. During a foetal
echocardiography, echoes are used to produce precise photographs of the
developing baby’s heart. It can assist a medical professional in confirming the
transposition of the great arteries findings.
Ultrasonography or “echo”
: sound waves provide a picture of the heart in an echocardiogram.
A chest X ray can also be
used sometimes to diagnose
A recording of the
heart’s electrical activity is called an ECG.
A 3 dimensional image of
the heart’s defects can be seen using a heart MRI.
Cardiac catheterization :
A small tube is put into the heart either via the umbilicus (the belly) or a
vein or artery with in leg.
The most often utilised
methods for diagnosing CCTGA are cardiac MRI and echo as these tests can
confirm the defect properly and accurately.
TREATMENT &
MANAGEMENT :
Diuretic medication ,
digitalis, betablockers, and angiotensin converting enzyme inhibitor treatment
may be used in the heart failure management. All are useful for treating
symptoms in specific people, but none have been shown to reduce rates of death
in congenital heart disease patients.
In the case of
congenitally corrected transposition of the great arteries, it is tempting to
hypothesise that established treatment outcomes for severe LV dysfunction ( ACE
inhibitor, beta blocker, nitrate, hydralazine, aldosterone antagonist) would
have comparable results in individuals with RV failure. However, care should be
taken when administering beta blockers to patients with known conduction system
problems because total heart block may be triggered. If they are suitable
candidates, patients with deteriorating systemic ventricular function may
ultimately benefit most from heart transplantation.
In the CCTGA, surgical
corrections consist of :
Utilising patches and
stitches to seal the hole, VSD correction
Placing a tube graft to
clear a blocked left ventricular outflow tract.
Repairing a tricuspid
valve.
Repair or replace a very
leaky tricuspid valve.
Only when there are
symptomatic concomitant lesions and projected considerable haemodynamic
improvements is surgical therapy advised. Even when these anatomic components
are not immediately altered, postoperative problems like complete heart block
and increasing tricuspid regurgitation are frequent. The mirror image coronary
architecture and the shifted placement of a delicate conduction system could
make surgical restoration more difficult. The anterior descending and
circumflex branches of the right coronary artery frequently separate from one
another. This makes it more difficult to put a catheter in patients to treat
pulmonary valve narrowing or stenosis
MEDICATION :
Medicine includes
antibacterial pre-medication for operations and normal heart failure treatment
(diuretics, digitalis, beta-blockers, and ACE inhibitors). All are beneficial
for treating symptoms, but none have been shown to reduce rates of death.
Amoxicillin, ampicillin,
and clindamycin are antibacterials.
Furosemide is a diuretic.
Lisinopril, ramipril,
captopril, enalapril, and quinapril are ACE inhibitors.
Digoxin is a cardiac
glycoside.
BETA BLOCKERS: Carvedilol
and metoprolol
When
the two major blood vessels that leave the heart are in an incorrect position,
it results in a cardiac abnormality known as transposition of the great
arteries, or TGA. TGA is a potentially fatal congenital disease that exists
from birth. Infants with TGA require surgery. TGA is unusual. Even after the
main angioplasty, some patients are still living. Therefore, a patient's chance
of survival relies on the severity of their condition and the care they are
receiving.
REFERENCES
:
https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/cctga/
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