Centers of Excellence in Interventional Cardiology and Radiology

Congenital Heart Disease

Coarctation of the aorta

The procedure performed by team of specialists from abroad!

We have opened the first section for Percutaneous Closure of Congenital Heart Diseases in Romania!

The most modern equipped angiography room , an exceptional medical team

We have the most famous abroad specialists! Team from Romania, Israel, Greece and Germany!

We have the largest experience. The youngest child was treated at the age of 5 days! Call now us!

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Generalities

What is coarctation of the aorta?

Coarctation of the aorta is a congenital anomaly representing 5-8% of all congenital cardiovascular malformations, which involves the narrowing (stenosis) of a segment of the aorta (the main vessel in the body). It can occur isolated (most frequently) or it can be associated with other cardiac malformations (ventricular septal defect, patent ductus arteriosus, valvular diseases – bicuspid aortic valve and, rarely, conditions that involve the right heart).

Classically, the coarctation is located at the level of the thoracic aorta distal to the origin of the left subclavian artery (the last branch of the initial segment of the aorta), but it can also involve the horizontal (transverse) segment of the aorta (the arch of the aorta). Rarely, the final segment of the thoracic aorta or the abdominal aorta can be involved, in these cases a longer segment of the vessel being involved.

The consequence is the obstruction of blood flow through the aorta. The narrowing is due to the ”infolding” of the internal layer of the aorta, which becomes a membrane with a central or lateral orrifice, making the flow through the vessel difficult.

Most frequently the diagnosis is made before the age of 10 years old, but sometimes (25% of cases) it is diagnosed later in life.

What are the consequences of this condition?

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The patients diagnosed with coarctation of the aorta usually have heart failure symptoms, but they can also be asymptomatic.

Typical manifestations include – depending on age, the following:

The Fetus : disproportionate ventricles and great vessels, association with other congenital cardiac abnormalities, modified echocardiographic parameters – nucal thickening, chromosomal anomalies (specific tests performed during pregnancy, i.e. Turner syndrome)

The Newborn : collapse, acidosis, heart failure, cardiac murmur which may be heard posteriorly (in the back) (abnormal sound which can be heard at a certain time in the cardiac cycle or continuously during the whole cycle), diminished or absent pulse of the lower extremities compared to that of the arms, hypertension of the upper extremities.

The Infant : heart failure (shortness of breath – during exercise or at rest, difficulty performing activities which involve a physical effort), murmur (as described previously), pulse differences between the upper and lower extremities, hypertension and, rarely, cardiomyopathy (the involvement of the heart secondary to the effort of pushing the blood through the narrowed segment, which increases the pressure inside the heart, thus leading to the thickening of the ventricular walls and the impairment of their function).

The Child, the Adolescent and the Adult : murmur, pulse differences, hypertension of the upper extremities, exercise intolerance, weakness of the legs and claudication (pain arising in the legs when walking, with various degrees depending of the distance), sensation of cold legs, cardiac arrest (due to arrythmias – rythm disturbances and/or hypertrophic cardiomyopathy – thickening of the cardiac walls secondary to high pressure, as described previously), retinal involvement because of hypertension, dissection (the occurence of a new lumen between the aortic walls) or rupture of the aorta, intracerebral hemmorhages, ìnfectious endocarditis (infections of the structures of the heart).

Indications

How can it be treated?

Because of its complications, the coarctation of the aorta can benefit of medical, surgical, or interventional (minimally invasive) treatment : either by the dilatation of the narrowed segment using a balloon (balloon angioplasty), or by the insertion of a stent (a small thin metallic device, like a tube – the procedure is called stent angioplasty), which maintains the vessel to its normal dimensions.65

Indications for treatment : include heart failure, hypertension, and the existence of a pressure gradient (pressure difference between the segment behind the stenosis and the segment after the stenosis) ≥20 mmHg (at rest) or even lower values if radiologic findings suggestive of high collateral flow (the opening of new vessels) are found, in case of progressive left ventricular hypertrophy (the thickening of the heart walls) and impairment of left ventricular function, or association of other abnormalities requiring specific treatment (valvular diseases, aortic aneurysm, septal defects..)

As far as the treatment method is concerned, it is chosen depending on the age at presentation and the anatomy of the coarctation (the aspect of the lesion), also of the neighbouring structures (the association with other defects).

How is the optimal treatment established?

As specified above, important determinants when choosing the best treatment strategy are the age of the patient and the anatomy of the stenosed segment.

Thus,

• depending on the age at presentation:

For the newborn and infant : surgical intervention and minimally invasive treatment – balloon dilation.

For the child (>25 kgs), the adolescent, and the adult : stent angioplasty is indicated.

• depending on the aspect of the lesion:

Long coarctation segments can be managed either surgically or by stent implantation.

Discrete or recurrent coarctation (after previous surgical intervention or balloon dilation) can either benefit of a new dilation, or a stent angioplasty may be performed.

The stent is preferred for: long segments of coarctation, associated istmic hypoplasia or of the aortic arch (insufficiently developed initial segments of the aorta), bending of the involved segment, coarctation recurrence (re-narrowing), or aneurysm formation after a previous intervention (either surgical or balloon dilation)

Procedure

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The procedure is performed by a technique similar to angiography, after the optimal treatment option has been established (dilatation or stent placement). It is done under general or local anesthesia.

After anesthesia, a small incision is made inguinally to visualise the femoral artery, then a sheath is introduced in the femoral artery (a plastic tube allowing the handling of all the necessary equipment). Then, heparin is being administered (a drug which prevents the formation of blood clots during the intervention), and a thin wire (guide) is being introduced through the sheath, reaching the lesion, then passing the narrowed segment, and being fixed in a branch originating in the aorta. Then a catheter (another tube) is introduced over the wire, thus reaching the narrowed segment.

Depending on the chosen technique (dilatation or stent placement), the catheter has either a balloon, or a ballon and a stent which will be subsequently deployed (usually a „balloon-in-balloon” device)

In the case of balloon angioplasty, after the catheter is positioned in the coarctation area (the position of the catheter is verified radiologically) the balloon is inflated with diluted contrast agent for 5 seconds, then the procedure is repeated two to four times at 5 minute intervals. At the end the result is verified angiographically (the diameter of the vessel and the pressure gradient).66

When a stent is implanted, the catheter is positioned in the affected segment, the stent is positioned (verified angiographically), then the first balloon (the internal one) is inflated, the position of the stent is once again verified angiographically, and the second balloon (the external one) is inflated. Once the stent is placed in the proper position the balloons are deflated and are subsequently carefully retired.

There are certain situations when for the placement of the stent a device with a single balloon is utilized (especially in the case of small children) in order to prevent the lesion of the femoral artery (for the ‚balloon-in-balloon’-type device a larger sheath is required).

After the procedure intravenous antibiotic medication is administered for 24 hours.

Is it painful?

The procedure is usually performed under general anesthesia, or local anesthesia. After the intervention antialgic therapy is administered, as there may be a minor discomfort at the incision site.

How long does it take?

The procedure takes about 1-2 hours and is performed in the cardiac catheterization laboratory.

Risks

The complications are very rare and the risks are very low, since the patient is under permanent medical surveillance. Possible complications are:

• allergic reactions to administered substances

• reactions to anesthetic compounds

• arteriovenous fistulas at the vascular puncture site

• minor bleeding at the vascular puncture site

• fever

• headache, migraine

• infection

• gaseous embolism

• cardiac arrythmia

• embolization or migration of the stent

• cerebrovascular complications (consequent to migration of the stent, aortic rupture/dissection, balloon rupture)

• hemolysis

• aortic wall/femoral artery lesion

• aortic rupture/dissection (and very rarely death)

• recoarctation (in case of balloon angioplasty)

• intrastent stenosis

• paradoxical hypertension or persistence of hypertension

• paraplegia

Before procedure

Preoperative assessment establishes if the coarctation should be treated interventionally or surgically. Echocardiography and other imaging techniques like angiography, angioCT, MRI, sometimes with 3D reconstruction – are very usefull for this assessment.

Prior to the intervention, the interventional cardiologist must be prevented about any history of allergic reactions and informed about pregnancy. Also, all the current medication needs to be clearly specified (especially the antiplatelet drugs – aspirin, clopidogrel, or anticoagulant drugs) as well as the presence of any other medical conditions (diabetes, renal disease).

Blood tests are taken including hemoglobin level, coagulation tests, renal function, and other specific tests.

The patient is admitted the day before the intervention, and he/she should not eat/drink before the procedure.

After procedure

Since the procedure is minimally invasive, the postprocedural recovery is usually very fast. After the procedure, the patient is monitored in the intensive care unit, then in the chamber where he/she will be hospitalized. Bed rest is necessary for 12-24 hours (bending of the foot will be prevented to avoid local complications at the puncture site).

The majority of patients can leave the hospital the following day, and they can recommence their usual daily activities (however, physical effort avoidance is recommended for a period of time). Indications about recovery and postprocedural treatment will be clearly specified to all patients before being discharged.

Important!

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Within our centres of excellence, both the patient and his/her doctor can choose the interventional cardiologist from our reputable team. As soon as a procedure is performed, the patient will receive a written report and a CD containing recorded images of the intervention, and the doctor is informed about his/her patient’s health status.

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