COARCTATION OF AORTA IN CHILDREN
- Main blood vessel that arises from left sided lower chamber (left ventricle) is known as This aorta supplies pure blood to all parts of the body including the brain.
- If there is blockage in distal part of this aorta just beyond the vessel that supplies blood to left arm is known as Coarctation of aorta (also known as Aortic Coarctation).
- This is a congenital heart defect (CHD) e. generally presents at birth known as, congenital coarctation of aorta.
- It may be present as a sole disease or may be associated with other heart problems like hole in the heart etc.
- Coarctation of aorta can occur in newborn, infants, children and adolescents and even in adults.
- When there is severe blockage in aorta, it gives back pressure to left ventricle and so left ventricle needs to work harder and in late stages, if not treated on time, can leads to heart failure.
- By blockage in aorta, there will be less blood supply to lower part of the body including the kidneys.
- Proximal to blockage, there will be a high pressure and it can leads to high blood pressure (Hypertension).
- Majority of the children who are having coarctation of aorta are having deformed aortic valve in the form of bicuspid aortic valve (bicuspid aortic valve).
- Additionally, it can be associated with patent ductus arteriosus, mitral regurgitation/stenosis, aortic valve stenosis/regurgitation
- Coarctation of the aorta is more commonly associated with a genetic syndrome known as ‘Turner syndrome’.
Cause of coarctation of aorta in children:
- Cause of coarctation of aorta is unknown most of the times.
Symptoms of coarctation of aorta:
- Presentation of patient with coarctation of aorta depends on severity of blockage and age of presentation.
- In neonates, it often presents as a duct dependent lesion in the form of critical coarctation of aorta (newborn critical coarctation of aorta). These will presents as shock like state with dependency on patent ductus arteriosus (PDA), symptoms can be in the form of poor milk intake, increased breathing rate, poor skin color, decrease urine output. Few neonates who are having milder variety of disease will be asymptomatic.
- In late infancy or childhood (adolescents), it is either asymptomatic or detected incidentally by a local doctor by hearing an abnormal heart sound (coarctation of aorta murmur) with poor pulses in lower limbs.
Older child may have high blood pressure (hypertension), headache, bleeding from nose, leg cramps or pains, blurring of vision, loss of consciousness etc.
Complications associated with coarctation of aorta:
- Narrowing of the aortic valve
- Brain stroke
- Aneurysm of aorta (meanning by: increase in size of the wall of the aorta)
- Kidney and/or other organ failure
- Risk of rupture/tear of aorta known as aortic dissection
- In late stages, heart failure can occur
Coarctation of aorta Diagnosis in children:
- Pre-natal testing in the form of fetal echocardiogram can reveal coarctation of aorta during pregnancy state (even before delivery of the child). It helps in planning of delivery of the child for timely initiation of treatment as soon as newborn is delivered.
- After birth, coarctation of aorta can be suspected by high blood pressure, pain in legs on walking, by hearing murmur during examination etc, and can be confirmed by doing pediatric echocardiogram from the chest of the child.
- ECG, Chest X ray and sometimes CT/MRI angiogram also help in making and confirming the diagnosis.
- When we are in doubt by above diagnostic modalities, then we need to perform cardiac catheterization in cath lab.
Treatment of coarctation of aorta in children:
- If narrowing in aorta (coarctation of aorta) is mild to moderate in intensity then we need to do follow-up of the child at regular intervals.
- However, in severe coarctation of aorta, treatment needs to be done.
- In coarctation of aorta, treatment of choice is by doing ballooning of coarctation of aorta segment (balloon dilatation of coarctation of aorta).
- Surgery is the treatment of choice in neonatal/infants coarctation of aorta. However if there is severe coarctation of aorta with severe failure of left ventricle then ballooning can be done as a palliative procedure (neonatal/infantile coarctation of aorta balloon dilatation).
- Surgical methods can be in the form of resection with end-to-end anastomosis, subclavian flap aortoplasty, bypass graft repair or patch aortoplasty.
- Coarctation of aorta surgery is done by open technique. Generally, it is done from left sided back.
Balloon Valvuloplasty (angioplasty) of coarctation of aorta:
- This is not an open-heart surgery. It is done in cardiac cath lab under fluoroscopic guidance by using vessels in thigh. We cross the valve with wire and catheters and we inflate a balloon across the stenosed aorta.
- Cost for balloon dilatation of coarctation of aorta is very reasonable now a day.
- Stenting of coarctation of aorta is a very good treatment option as compared to balloon dilatation of coarctation of aorta in adolescents and adults.
- It is a procedure of choice when balloon dilatation is ineffective or coarctation of aorta reoccurs after surgery of coarctation of aorta.
- This is not a surgery. It is done in cardiac cath lab by angiographic technique. In this, by the use of catheter and wires into the artery in groin, we place a stent across the coarctation of aorta under x ray imaging.
Coarctation of aorta balloon dilatation risks:
- Balloon valvuloplasty of aortic coarctation has very low risk of morbidity and mortality in neonates and children.
Coarctation of aorta treatment result
- Now a day, results of ballooning/stenting of coarctation of aorta are excellent.
- However, in long term, coarctation of aorta can recur in long term.
- If it recurs, then either ballooning or stenting can be planned depending on the age and weight of the patient.
- Mortality after open heart surgery is extremely rare.
- Children affected with coarctation of aorta will require long-term follow-up with a pediatric cardiologist.
Follow-up after coarctation of the aorta repair
- Child needs to be on lifelong follow-up with a pediatric cardiologist as most commonly associated problem with coarctation of the aorta is high blood pressure (hypertension).
- Sometimes, few segment of repaired aorta may become enlarge and may also rupture.
- Recurrence of coarctation of the aorta is possible in some cases even after many years of treatment/surgery.
Activity restrictions afet surgery for coarctation of aorta
- If coarctation is treated very well with good control of blood pressure, then no special contraindication is there and child can participate in normal activities without increased risk.
- But if there is residual significant obstruction, hypertension etc, we need to limit child activities.
Adults with coarctation of the aorta:
Only thing is that if, significant coarctation of aorta is present, then preferred treatment modality is by doing Stenting of Coarctation of Aorta.
Pregnancy and Coarctation of Aorta
- Even after repair of coarctation of the aorta, these patients are at a higher risk of aortic rupture, aortic dissection etc during pregnancy and delivery.
We need to control blood pressure through out the pregnancy and delivery for avoiding these complications.
Coarctation of Aorta
Coarctation of Aorta