Coarctation of the Aorta

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Coarctation of the aorta\: ductal-dependent congenital heart condition with narrowing of the aortic arch, usually near the
ductus arteriosus/ligamentum arteriosum; 6-8% of congenital heart defects.
Aetiology\: unclear; theories include under-development in utero due to reduced blood
the thoracic aorta causing constriction postnatally; leads to left ventricular hypertrophy and reduced pressure in distal
arteries.
Risk factors\: male gender, genetic conditions (e.g., Turner syndrome), family history; commonly associated with other
congenital heart defects (e.g., bicuspid aortic valve).
Symptoms\: poor feeding, tachypnoea, increased work of breathing, irritability, grey/
antenatally or remain undiagnosed until adulthood.
Clinical signs\: weak femoral pulses, ejection systolic murmur in left infraclavicular region, unequal blood pressure between
arms, di
femoral delay.
Investigations\: ECG (normal or left ventricular hypertrophy), chest X-ray (indentation of aortic shadow, rib notching,
congestive heart failure), echocardiogram, MRI (detailed assessment of coarctation and associated abnormalities).
Management\: prostaglandin E1 to maintain ductus arteriosus patency, dobutamine or dopamine for heart failure,
supportive care; surgery (balloon angioplasty, resection with end-to-end anastomosis, bypass graft).
Complications\: untreated severe coarctation can lead to heart failure or death; chronic hypertension, brain aneurysm
rupture, brain haemorrhage, aortic dissection, aortic aneurysm, congestive heart failure, irreversible heart damage.
Post-treatment complications\: re-coarctation, aortic aneurysm or rupture, persistent hypertension.
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A comprehensive topic overview

Introduction

Coarctation of the aorta is a ductal-dependent congenital heart condition de
usually found around the ductus arteriosus/ligamentum arteriosum. It makes up 6-8% of all congenital heart defects.
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A ductal-dependent congenital heart condition is a defect that relies upon a patent ductus arteriosus to maintain
systemic or pulmonary blood circulations.
2

Aetiology

The cause of coarctation of the aorta is unclear. There are two current theories regarding the narrowed segment of aorta\:
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The narrowed segment is under-developed in utero due to reduced blood
is thought to be important when coarctation is associated with other congenital heart defects)
Ductal tissue expands into the thoracic aorta, leading to constriction of the thoracic aorta when the ductus arteriosus
closes after birthWith coarctation of the aorta, the left ventricle works harder to pump blood through the narrowing; therefore, blood
pressure rises in the left ventricle. This can lead to left ventricular hypertrophy. It also reduces the pressure of blood
in the arteries distal to the narrowing.

Risk factors

Risk factors for coarctation of the aorta include\:
Male gender
Certain genetic conditions, particularly Turner syndrome
Family history
Coarctation of the aorta is commonly accompanied by other congenital heart defects, predominantly a bicuspid aortic
valve.
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Clinical features

The clinical presentation of coarctation of the aorta depends on the severity and the age of the infant.
History
In infancy, clinical features of coarctation include\:
Poor feeding
Tachypnoea
Increased work of breathing
Irritability
Grey and
It is important to note that these symptoms are listed as how a neonate may present. However, coarctation can also be
picked up antenatally through ultrasound in utero or remain undiagnosed until later in adulthood.
Clinical examination
In a neonate, commonly the only clinical sign of coarctation is weak femoral pulses. Other signs on clinical examination in
infancy may include\:
Ejection systolic murmur\: heard most clearly in the left infraclavicular region
Unequal blood pressure between arms
A di
A di
Cold extremities (especially feet)
Additional clinical signs that may develop over time include a left ventricular heave, radio/radial-femoral delay and
underdevelopment of the left arm and legs. If coarctation is picked up later in life, then patients will often present with
treatment-resistant hypertension and its associated complications.

Di

Considering a number of conditions are associated with coarctation of the aorta, it is important to be aware of the
di
Aortic stenosis
Cardiomyopathies
Primary hypertension
Viral myocarditisInvestigations
Bedside investigations
Relevant bedside investigations include\:
ECG\: likely normal with mild coarctation. Left ventricular hypertrophy and signs of ischaemia can occur in severe cases
Imaging
Relevant imaging investigations include\:
Chest X-ray\: there may be indentation of aortic shadow, notching on the underside of the ribs, or signs of congestive
heart failure
Echocardiogram\: determines the nature of the coarctation and identi
MRI scan\: determines the nature of the coarctation and identi

Diagnosis

Early diagnosis is important because, in severe cases of coarctation of the aorta, blood
arteriosus. Two to three days after birth, the ductus arteriosus closes; therefore, if left untreated, the condition worsens
rapidly.
All neonates have pulse oximetry measured after birth, which helps to identify critical congenital heart defects, including
severe coarctation, early.

Management

Management depends on the severity of the defect and the age at the time of diagnosis. In mild cases, patients may not
require any intervention and can live symptom-free until adulthood, sometimes undiagnosed. In severe cases, patients
undergo emergency surgery shortly after birth.
Medical management
All ductal-dependent lesions which deteriorate are treated with prostaglandin. Prostaglandin E1 is prescribed to maintain
the ductus arteriosus until surgery. This allows blood to
the coarctation.
Dobutamine or Dopamine can be used to improve contractility in those with heart failure. Supportive care might be
required to correct respiratory failure, anaemia, metabolic acidosis and hypoglycaemia that may contribute to or be a
consequence of heart failure.
Surgical management
Following medical management, surgery is performed to close the ductus arteriosus and correct the coarctation.
Surgical repair of the narrowing usually involves balloon angioplasty. Other options include resection with end-to-end
angioplasty and a bypass graft.
Balloon angioplasty involves inserting a catheter, usually into the femoral artery, and advancing it up to the area of
narrowing. The balloon is then in

Complications

Without treatment, severe forms of coarctation in babies can lead to heart failure or death.
The most common complication of coarctation of the aorta is chronic hypertension.
Other complications of coarctation include\:
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Brain aneurysm rupture
Brain haemorrhageAortic dissection
Aortic aneurysm
Congestive heart failure
In severe cases, it can lead to irreversible damage to the heart. This can result in kidney failure or other organ failure.
Complications can also occur after treatment. These include\:
Re-coarctation after repair
Aortic aneurysm or rupture
Hypertension

References

BMJ Best Practice. Aortic coarctation. August 2023. Available from\: [LINK]
Cucerea M, Simon M, Moldovan E, Ungureanu M, Marian R, Suciu L. Congenital Heart Disease Requiring Maintenance of
Ductus Arteriosus in Critically Ill Newborns Admitted at a Tertiary Neonatal Intensive Care Unit. J Crit Care Med (Targu
Mures). November 2016. Available from\: [LINK]
Law MA, Tivakaran VS. Coarctation of the Aorta. August 2023. Available from\: [LINK]
Cangussu LR, Lope MR, Barbosa RHA. The importance of the early diagnosis of aorta coarctation. February 2019. Available
from\: [LINK]

Reviewer

Dr Akmal Hussain
Consultant Paediatrician

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Contents

Introduction
Aetiology
Risk factors
Clinical features
Di
Investigations
DiagnosisSource\: geekymedics.com