Coarctation of aorta

Cardiology

Clinicals - History

Fact Explanation
Features of Heart Failure In newborns and in infants symptoms of heart failure are usually poor feeding, scalp sweating during feeds, poor weight gain and lethargy. This is due to the left ventricular output obstruction and increased end diastolic volume of the left ventricle. Acute volume overload eventually results in congestive heart failure. Features of Heart Failure
In newborns and in infants symptoms of heart failure are usually poor feeding, scalp sweating during feeds, poor weight gain and lethargy. This is due to the left ventricular output obstruction and increased end diastolic volume of the left ventricle. Acute volume overload eventually results in congestive heart failure.
History of hypertension High blood pressure is recorded in the right arm. History of hypertension
High blood pressure is recorded in the right arm.
Intracranial hemorrhage Due to rupture of intra cranial Berry aneurysm and sub arachnoid hemorrhages. Intracranial Berry aneurysm is a known association with coarctation of aorta. Patients might present with recurrent intracranial hemorrhages. Intracranial hemorrhage
Due to rupture of intra cranial Berry aneurysm and sub arachnoid hemorrhages. Intracranial Berry aneurysm is a known association with coarctation of aorta. Patients might present with recurrent intracranial hemorrhages.
Fatigue Due to heart failure and lower limb hypo perfusion. This also can cause pain or weakness in the lower limbs. Fatigue
Due to heart failure and lower limb hypo perfusion. This also can cause pain or weakness in the lower limbs.
Sudden death May be due to cardiac failure, intracranial hemorrhage or infective endocarditis. Sudden death
May be due to cardiac failure, intracranial hemorrhage or infective endocarditis.

Clinicals - Examination

Fact Explanation
Hypertension High systolic blood pressure is recorded. Hypertension
High systolic blood pressure is recorded.
Blood pressure disparity in the upper and lower limbs Reduced perfusion of the lower limbs causes low blood pressure in the lower limbs. Blood pressure difference is more than 20 mm Hg. Blood pressure disparity in the upper and lower limbs
Reduced perfusion of the lower limbs causes low blood pressure in the lower limbs. Blood pressure difference is more than 20 mm Hg.
Blood pressure disparity in the right and left arms The blood pressure is 20mm Hg or more higher in the right arm than the left arm. This occurs when the coarctation is proximal to the origin of left subclavian artery. Blood pressure disparity in the right and left arms
The blood pressure is 20mm Hg or more higher in the right arm than the left arm. This occurs when the coarctation is proximal to the origin of left subclavian artery.
Tachypnea and shortness of breath Due to heart failure. Tachypnea and shortness of breath
Due to heart failure.
Tachycardia Tachycardia and gallop rhythm are features of heart failure. Tachycardia
Tachycardia and gallop rhythm are features of heart failure.
Shock Due to congestive heart failure. Shock
Due to congestive heart failure.
Absent or weak pulse in left arm The left subclavian artery originates below the coarctation. Absent or weak pulse in left arm
The left subclavian artery originates below the coarctation.
Absent lower extremity pulses or radio-femoral delay Due to reduced perfusion of lower limbs. Absent lower extremity pulses or radio-femoral delay
Due to reduced perfusion of lower limbs.
Differential cyanosis Cyanosis in the lower extremities with pink upper limbs. This occurs when the lower limb perfusion is duct dependent. The lower extremities are perfused with deoxygenated blood coming from the pulmonary arteries. Differential cyanosis
Cyanosis in the lower extremities with pink upper limbs. This occurs when the lower limb perfusion is duct dependent. The lower extremities are perfused with deoxygenated blood coming from the pulmonary arteries.
Systolic murmur In the infraclavicular area and under the scapula on left side. Murmur may be continuous in the presence of multiple collateral vessels or in severe coarctation. Systolic murmur
In the infraclavicular area and under the scapula on left side. Murmur may be continuous in the presence of multiple collateral vessels or in severe coarctation.
Abdominal bruit This is a relatively rare presentation and it is due to the turbulent blood flow at the site of narrowing of the aorta. Abdominal bruit
This is a relatively rare presentation and it is due to the turbulent blood flow at the site of narrowing of the aorta.

Investigations - Diagnosis

Fact Explanation
Echocardiography Echocardiogram enables the diagnosis and Doppler studies can determine the pressure gradient at the site of coarctation. Echocardiography
Echocardiogram enables the diagnosis and Doppler studies can determine the pressure gradient at the site of coarctation.
Chest x ray Rib notching due to dilatation of collateral vessels. “3” sign of aorta due to the coarctation. Chest x ray
Rib notching due to dilatation of collateral vessels. “3” sign of aorta due to the coarctation.
Electrocardiogram (ECG) Shows evidence of left ventricular hypertrophy. It may show evidence of left ventricular strain pattern (Eg: ST segment depression and T wave inversion.) Electrocardiogram (ECG)
Shows evidence of left ventricular hypertrophy. It may show evidence of left ventricular strain pattern (Eg: ST segment depression and T wave inversion.)
Magnetic Resonance Imaging (MRI) MRI detects the exact location of the coarctation and the presence of collaterals. MRI and cardiac catheterization gives similar information and enable planning the treatment. Magnetic Resonance Imaging (MRI)
MRI detects the exact location of the coarctation and the presence of collaterals. MRI and cardiac catheterization gives similar information and enable planning the treatment.
Cardiac catheterization Cardiac catheterization is invasive and now rarely practiced in diagnosis. An advantage of cardiac catheterization is that it can be combined with treatment. Cardiac catheterization
Cardiac catheterization is invasive and now rarely practiced in diagnosis. An advantage of cardiac catheterization is that it can be combined with treatment.

Investigations - Management

Fact Explanation
Echocardiography Post operative echocardiogram ensure the restoration of normal anatomy. There is a considerable risk of re-stenosis, aneurysm and pseudo-aneurysm formation even after corrective interventions. Echo cardiogram enables detection of those complications. Coarctation of aorta is a life long disease and long term follow up is indicated after the definitive treatment. (There is no established evidence on the duration of follow up.) Echocardiography
Post operative echocardiogram ensure the restoration of normal anatomy. There is a considerable risk of re-stenosis, aneurysm and pseudo-aneurysm formation even after corrective interventions. Echo cardiogram enables detection of those complications. Coarctation of aorta is a life long disease and long term follow up is indicated after the definitive treatment. (There is no established evidence on the duration of follow up.)
MRI MRI is considered a better option to detect re-coarctation and aneurysm formation than the echocardiogram. Patients should be followed up with an MRI in 12 to 24 months intervals. Reduction in the compliance of the aorta is an established cause for the development of hypertension in later life. MRI can determine the vascular compliance and predict the risk of hypertension. MRI
MRI is considered a better option to detect re-coarctation and aneurysm formation than the echocardiogram. Patients should be followed up with an MRI in 12 to 24 months intervals. Reduction in the compliance of the aorta is an established cause for the development of hypertension in later life. MRI can determine the vascular compliance and predict the risk of hypertension.
Cardiac catheterization This is the gold standard investigation in detection of the re-coarctation and other vascular complications. How ever due to the increased risk of radiation exposure, it is reserved for patients with strong suspicion of the complications. Cardiac catheterization
This is the gold standard investigation in detection of the re-coarctation and other vascular complications. How ever due to the increased risk of radiation exposure, it is reserved for patients with strong suspicion of the complications.
Chest X-ray Aids in diagnosing heart failure and pre-operative treatment of heart failure. (Heart failure is an added risk. ) Chest X-ray
Aids in diagnosing heart failure and pre-operative treatment of heart failure. (Heart failure is an added risk. )
Full blood count To assess the hemoglobin level. Aids in differentiating sepsis. Full blood count
To assess the hemoglobin level. Aids in differentiating sepsis.
Exercise ECG To assess exercise tolerance prior to elective surgery. Exercise ECG
To assess exercise tolerance prior to elective surgery.
Renal function test Serum electrolytes, blood urea and serum creatinin will provide adequate information on renal function. Kidneys are one of the main organs involved in drug metabolism. Patients with renal dysfunction have increased mortality than the patients without renal disease. Renal function test
Serum electrolytes, blood urea and serum creatinin will provide adequate information on renal function. Kidneys are one of the main organs involved in drug metabolism. Patients with renal dysfunction have increased mortality than the patients without renal disease.
Fetal echocardiography This allows early detection of the coarctation and anticipation of the possible outcomes like congestive heart failure. However this is very difficult to diagnose antenatally, especially towards the late gestation. Screening is better done during early gestation, preferably in the second trimester. Fetal echocardiography
This allows early detection of the coarctation and anticipation of the possible outcomes like congestive heart failure. However this is very difficult to diagnose antenatally, especially towards the late gestation. Screening is better done during early gestation, preferably in the second trimester.

Management - Supportive

Fact Explanation
Prostaglandin infusion When the lower limb perfusion is duct dependent the patency of the ductus arteriosus is maintained with an infusion of prostaglandin. Prostaglandin infusion
When the lower limb perfusion is duct dependent the patency of the ductus arteriosus is maintained with an infusion of prostaglandin.
Treatment of cardiogenic shock Intravenous fluids and inotrops should be used. Treatment of cardiogenic shock
Intravenous fluids and inotrops should be used.
Correction of metabolic acidosis Anerobic metabolism might cause a metabolic acidosis. Correction of metabolic acidosis
Anerobic metabolism might cause a metabolic acidosis.
Treatment of heart failure Pharmacological management of heart failure should be done prior to surgery. Treatment of heart failure
Pharmacological management of heart failure should be done prior to surgery.
Treatment of hypertension Hypertension should be controlled before the surgery. Treatment of hypertension
Hypertension should be controlled before the surgery.
Antibiotic prophylaxis against infective endocarditis There is a risk of bacterial endocarditis with coarctation of aorta. Antibiotic prophylaxis against infective endocarditis
There is a risk of bacterial endocarditis with coarctation of aorta.
Screen the females for Turner syndrome Turner syndrome is associated with 10% of cases of coarctation of the aorta. Screen the females for Turner syndrome
Turner syndrome is associated with 10% of cases of coarctation of the aorta.

Management - Specific

Fact Explanation
Surgical repair The access is gained through a left lateral thoracotomy. Resection and end-to-end anastomosis is the preferred method of treatment. Subclavian flap repair and patching with a Dacron patch are less practiced treatment options. Surgical repair
The access is gained through a left lateral thoracotomy. Resection and end-to-end anastomosis is the preferred method of treatment. Subclavian flap repair and patching with a Dacron patch are less practiced treatment options.
Balloon dilation This is a first line treatment option in the management. Balloon dilation
This is a first line treatment option in the management.
Stent implantation In adolescents and adults stent placement is considered as a primary mode of treatment. Stent implantation
In adolescents and adults stent placement is considered as a primary mode of treatment.

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