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COARCTATION OF THE AORTADiane D. SosaCaseMiss Sathi, 24 years old, admitted into the hospital with the complaints of -1) Headache, dizziness and fatigue-2 years2) Shortness of breath- 2 years.3) Pain in leg after prolong walking- 2 years.

B.P- 180/90mmHG ( in arm) Leg- not recordable.

The patient states that she developed headache, dizziness and fatigue two years back. Symptoms gradually aggravated during last two years. She also felt tiredness and shortness of breath after walking or heavy works. It was also associated with leg cramps specially after walking prolong distance. She had no H/O rheumatic fever, asthma or cyanosis of lower limbs.

2PulsesRightLeftRadial++Brachial++Axillary++Carotid++Femoral--Popliteal--Post. Tibial--Dorsalis pedis--Auscultation- An ejection systolic murmur over left sternal border, more prominent over infrascapular region.

Provisional diagnosis- Coarctation of aorta

What is Coarctation of the AortaA congenital narrowing of upper descending thoracic aorta adjacent to the site of attachment of ductus arteriosus.

Coarctation of the aorta results from marked ridge like thickening of the media of the aortic wall opposite the insertion of the patent ductus arteriosus or ligamentum arteriosum.This ridge or shelf becomes an obstruction when the patent ductus involutes and when ductal tissue in the wall of the aorta involutes.

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This narrowing restricts the amount of oxygen-rich (red) blood that can travel to the lower part of the body.Coarctation of aorta8-10% of CHDs

M:F ratio 2:1

Pathology: indentation involving anterior, lateral & posterior wall of aorta

Dilatation of the descending aorta immediately distal to the coarctation segment (poststenotic dilatation) is usually present. Varying degrees of hypoplasia of the isthmus of the aorta (the portion of the aorta between the origin of the left subclavian artery and ductus arteriosus) are present in most patients with thoracic coarctation; this hypoplasia may be significant in symptomatic coarctation of the neonate and infant; in children and adults, the isthmus may have only mild narrowing.

7Types

The transverse aortic arch (the arch between the origin of the right innominate artery and the left subclavian artery) is also hypoplastic in symptomatic neonates and infants. Collateral vessels that connect arteries from the upper part of the body to the vessels below the level of coarctation may be seen; these may be present as early as a few weeks to a few months of life.

8PREDUCTAL TYPEPDA is patent and large and provide blood flow to lower extremity.Tubular narrowing of isthmusNo shelf like narrowing in aorta.Minimal post stenotic dilatation of aorta.Minor enlargement of intercostal arteries.

POSTDUCTAL TYPEThe ductus is closed and no longer acts as a shunt.

No narrowing of isthmus.Shelf like narrowing with in the aorta in juxtaductal position.Post stenotic and prestenotic aorta is dilated.Intercostal arteries are grossly dilated.

Preductal type(infantile)

Adult type CoA

Shortness of breath, especially when exercisingIntermittent claudicationFatigueFrequent nose bleedsDizziness or faintingChest pain, especially when exercisingVery cold legs and feetStrong, throbbing headache

Subjective dataHeadache and epistaxis due to systemic hypertensionClaudication due to reduced blood flow to lower extremity

13High blood pressureAbnormal differences of blood pressure and arterial pulses in upper and lower extremitiesMurmur

Systollicmumura pressure difference of more than 20 mm Hg in favor of the arms may be considered evidence of coarctation of the aorta.The murmur associated with coarctation of the aorta may be nonspecific initially and is usually a systolic murmur in the left infraclavicular area and under the left scapula. Infants with severe coarctation may develop severe symptoms and heart failure (CHF) including:Poor weight gainPoor feedingRapid breathingExcessive sweatingPuffy eyes, face or extremitiesCool, pale or dusky skin

14Collateral Circulation

More developed in adult or postductal type as ductus is closed and collaterals are the only source of blood supply to the lower half of body.There is progressive enlargement of collateral blood vessels around the coarctation segment.Collateral flow predominantly arisese from:Subclavian artery and its branches: Internal thoracic artery , intercostal artery, scapular artery, cervical artery, vertebral artery, spinal artery.Epigastric artery.LV afterload may also gradually increase, allowing children with less severe coarctation to develop arterial collateral vessels that partially bypass the aortic obstruction. These children may be asymptomatic until hypertension is detected or another complication develops.

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VolumePressureCoarctation of the aorta imposes significant afterload on the left ventricle (LV), which results in compensatory ventricular hypertrophy.As the ductus (aortic end) constricts, the left ventricular afterload rapidly increases, with a resultant increase in left ventricular pressures (systolic and diastolic).This causes elevation of the left atrial pressure, which may open the foramen ovale, causing left-to-right shunt and dilatation of the right atrium and right ventricle.

16EchocardiogramImpression- 1) Coarctation of aorta2) Bicuspid aortic valve.3) Mild concentric LV hypertrophy4) Fair LV systolic functionescription- LA, RA, RV, PA- NormalLV- mild concentric hypertrophy. AO- dilated.IAS, IVS- intact.MV- normal in appearance.AV- Bicuspid with mild reduction in cusp separation.A constriction suggestive of Coarctation of aorta seemed to be present distal to left subclavian artery.

17CXR PA View

An inverted "3" sign of the barium-filled esophagus or a "3" sign on a highly penetrated chest radiograph (frontal view) may be visualized. Rib notching secondary to collateral vessels may also be seen.

18Chest X ray

Chest X RAY

Echocardiogram

Inverted 3 sign21CXR: heart size N with prominent ascending aorta and aortic knuckle 3 sign on over-penetrated films rib notching between 4th and 8th ribs

E sign on barium swallow

ECG: left axis deviation, LVH

Echo: coarctation visualized heart murmur- A heart murmur is simply a noise caused by the turbulence of blood flowing through the obstruction in the coarctation segment of the aorta.chest X-rayelectrocardiogram (ECG or EKG)- a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage.echocardiogram (echo)- a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. The vast majority of aortic coarctations are diagnosed by echocardiography.cardiac catheterization(cath)- a diagnostic procedure that uses threading a catheter through the arteries and veins of the groin and advancing this catheter up to the heart. Dye is squirted into the heart and aorta and pictures are taken of the anatomy. Catheterization may also be used to repair the coarctation if the child is big enough.magnetic resonance imaging (MRI)- a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.

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MRIMRI and CT:Useful in older or postoperative patients to assess residual arch obstruction, arch hypoplasia, or formation of aneurysms.

23MANAGEMENT

24Both end are incised and sewn togther

Subclavia divide distally-> sc artery flap folded down over the area of narrowingPatch a synthetic material or homograft suture25

Balloon Dilatation AngioplastyFor recurrect coarctation26ManagementTreatment in patients with congestive heart failure (CHF) includes the use of diuretics and inotropic drugs.

Prostaglandin E1 (0.05-0.15 mcg/kg/min) is infused intravenously to open the ductus arteriosus.

Ventilatory assistance is provided to patients with markedly increased work of breathing.

PG- Identical to the naturally occurring prostaglandin E1(PGE1) and possesses various pharmacologic effects, including vasodilation and inhibition of platelet aggregation. Used as palliative therapy to temporarily maintain patency of the ductus arteriosus before surgery. Inot-Used to stimulate alpha-receptor and beta-receptors in the heart and vascular bed. Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic CHF. Cardiac glycosides-These medications improve ventricular systolic function by increasing the calcium supply available for myocyte contraction.Diuretics-Promote excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention have resulted in edema or ascites.

27Thank you!Electrocardiography :in the neonate or infant with early onset of coarctation of the aorta may reveal right ventricular rather than left ventricular (LV) hypertrophy.

ECG findings in patients with late onset of coarctation of the aorta may be normal or may reveal LV hypertrophy and may show signs of LV ischemia or strain.

Sometimes, LV hypertrophy may manifest as increased S waves in leads V5and V6, the so-called posterobasal LV hypertrophy.

In the neonatal period , the ECG may reflect RVH rather than LVH. This is because the right ventricle in-utero is the dominant ventricle, and through the PDA pumps blood to the descending aorta. Therefore, coarctation of the aorta may cause an increase in the afterload of the RV as it will cause narrowing of the aortic arch-descending aorta junction.This ECG, shows rsR' pattern in the right chest leads indicating RVH. In addition there are deep S waves in the left chest leads.

PseudocoarctationIt is a rare condition presumably resulting from the congenital elongation of the aortic arch .The elongation leads to redundancy and kinking of the aorta which may appear similar to the coarctation but has no actual obstruction to the blood flow.There is no actual pressure gradient in pseudocoarctation.There is tendency of dilatation and aneurysm formation due to the turbulant flow in aorta.