Download Coarctation of the aorta is typically a disease of childhood and early

Asymptomatic coarctation in an adult female – A case report
Key wards:
Coarctation, Balloon angioplasty,Hypertension, Congenital malformation
Abstract
Coarctation of the aorta is typically a disease of childhood and early adulthood, and there is a
reduced life expectancy in patients who have not undergone correction. Survival to older age is
rare, due to severe cardiovascular complications. We describe the case of a woman first
diagnosed with coarctation of aorta at adult age.
Introduction
Coarctation of the aorta accounts for 5%–10% of congenital heart disease and occurs more
frequently in males. It is usually diagnosed during childhood by routine examination of blood
pressure and femoral pulse palpation. We describe the case of a woman first diagnosed with
coarctation of aorta at an adult age. We present this case to highlight the importance of
systematic clinical examination while evaluating patients with hypertension.
Case Report
We report a case of asymptomatic coarctation of aorta in a young female presented to our
Medical clinic. Medline search did not reveal any case reported from United Arab Emitates.
A 26 year old female was referred from Primary care centre to our medical clinic for
management of hypertension. She presented to primary care centre with abscess of the axilla
and was found to be hypertensive. She did not have any other symptoms.
Clinical examination showed moderately built young lady. Blood Pressure was 166/93 both
upper limbs. Upper limbs pulses were felt normally. However, lower limb pulses including
femorals were not felt. There was no femoral or renal bruit. There was 2/6 systolic murmur
heard below the left clavicle which radiates to left infrascapular region. In view of these clinical
findings, aortic coarctation was suspected
Investigations: Results of renal functions and urinalysis were normal. ECG showed left
ventricular hypertrophy with strain pattern. Chest x-ray showed bilateral rib notching (Figure 1).
Chest X-ray demonstrates the rib notching bilaterally due to the dilated collaterals.
CT angiography of the thorax revealed aortic coarctation of the descending segment below the
level of subclavian artery (Figure 2 and 3).
ECT Axial cuts at the level of Aortic coarctation
Axial Enhanced CT demonstrates the Aortic coarctation with the post stenotic dilatation.
Dilated collaterals were also seen (Figure 4).
Axial enhanced CT images at the level of the internal mammary vessels showing dilated internal
mammary and para vertebral collaterals.
She was started on betablocker and referred to cardiology department for echocardiogram and
further evaluation. Echocardiogram showed tight coarctation of aorta with a gradient of 50 mm
Hg. Echocardiogram also revealed bicuspid aortic valve.
Cardiac catheterization with possible stent implantation was advised.
Discussion
Coarctation of aorta is a congenital malformation that usually presents early in life. Coarctation
of the aorta occurs in 6-8% of patients with congenital heart disease. (1, 2) The male-to-female
ratio is 2:1, although this ratio is not valid in abdominal coarctation of the aorta, in which this
rare lesion predominantly affects females. No definitive racial differences have been
documented in coarctation of the aorta, although some authors have suggested that
coarctation of the aorta is less common in Asians. (3) Generally, patients with coarctation of the
aorta present early in life with CHF or later in life with hypertension. Many patients are
asymptomatic except for the incidentally noted hypertension.Studies continue to document
that coarctation of the aorta is often missed in the first year of life, (4,5)and the median age of
referral to a pediatric cardiologist in one study was 5 years. Among 2192 patients reported to
the Pediatric Cardiac Care from 1985-1993, 1337 were infants, 824 were children, and 31 were
adults. (6) Palpation of femoral pulses and measurement of blood pressure during routine
examination is necessary to avoid a delay in the diagnosis.
The mean survival for untreated patients is 35 years with a 25% survival rate beyond 50 years.
The natural history of unrepaired coarctation of the aorta includes the development of systemic
hypertension and subsequent morbidity and death from cardiovascular disease (7). The age at
correction is the most important factor for the relief of hypertension and long-term survival (8).
Coarctation of the aorta is a constricted aortic segment .The classic coarctation of the aorta is
located in the thoracic aorta distal to the origin of the left subclavian artery. Rarely, a coarcted
segment is present in the lower thoracic or abdominal aorta. The localized constriction may
form a shelf like structure with an eccentric opening or may be a membranous curtain like
structure with a central or eccentric opening. The coarctation may be discrete, or a long
segment of the aorta may be narrowed; the former is more common. Dilatation of the
descending aorta immediately distal to the coarctation segment (post-stenotic dilatation) is
usually present. 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.
The most commonly associated clinically significant defects include patent ductus arteriosus,
VSD, and aortic stenosis. Mitral valve anomalies, although less common than those of the aortic
valve, are also associated with coarctation of the aorta.
Numerous theories have been proposed for the etiology of coarctation of the aorta, including
postnatal ductal constriction,(9,10) translocation of ductal tissue on to the aorta, (11, 12) and a
theory that alterations in intrauterine blood flow cause altered flow through the aortic arch and
result in the substrate for coarctation. Coarctation of the aorta manifests when the ductus
closes starting at the pulmonary end, with gradual involution of ductal tissue toward the aorta.
Similar to most forms of congenital heart disease (CHD), the etiology of coarctation of the aorta
may be explained by multifactorial inheritance hypothesis. The prevalence of coarctation of the
aorta in genetic abnormalities such as Turner syndrome (45X), is as high as 15-20%.
Investigations
Radiography in patients with early onset of coarctation of the aorta may reveal cardiomegaly,
pulmonary edema, and other signs of congestive heart failure.
Radiography in patients with late onset of coarctation of the aorta may reveal cardiomegaly.
Rib notching secondary to collateral vessels may also be seen.
Echocardiography: Echocardiography delineates intracardiac anatomy and allows assessment of
associated significant intracardiac anomalies. The suprasternal notch 2-dimensional
echocardiographic view allows evaluation of the aortic arch to assess the transverse aortic arch,
isthmus, and severity of coarctation. Doppler echocardiography is used to measure the gradient
at the site of coarctation and to identify the pattern of diastolic runoff typically seen in patients
with severe obstruction.
MRI and CT are useful in older or postoperative patients to assess residual arch obstruction,
arch hypoplasia, or formation of aneurysms. (13, 14)
Cardiac catheterization:
Cardiac catheterization and selective cineangiography may not be required for diagnosis;
however, they allow evaluation of the severity of coarctation, anatomic nature of the aortic
obstruction (discrete vs. long segment), and arch anatomy, including hypoplasia of the
transverse arch or isthmus.
Cardiac catheterization helps confirm the diagnosis when echocardiography findings are not
completely clear.
Cardiac catheterization allows the evaluation of intracardiac anatomy and the contribution of
associated lesions to the overall hemodynamic disturbance.
Cardiac catheterization is a prerequisite for intervention in the form of either balloon
angioplasty or stent implantation in native or recurrent coarctation.
When catheterization is performed, elevations of left ventricular and ascending aortic peak
systolic pressures with a peak-to-peak systolic pressure gradient across the coarcted segment
are usually found. A peak-to-peak gradient in excess of 20 mm Hg is generally considered
indicative of significant obstruction. However, the magnitude of the gradient is not necessarily
indicative of the degree of narrowing because the gradient depends on not only the extent of
aortic narrowing but also the size and number of collateral vessels.
Treatment:
Early presentation of coarctation of the aorta:
Treatment in patients with congestive heart failure 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.
If the coarctation has a significantly adverse effect on the physiology of the associated defects,
and consequently the clinical status, the coarctation should be initially relieved with surgery or
balloon angioplasty and the patient reassessed with regard to need for intervention for the
associated defects.
Late presentation of coarctation of the aorta
Treatment of hypertension: Preoperative hypertension can be effectively treated using betablockers.
Significant hypertension or congestive heart failure is an indication for intervention. Surgical
relief of the aortic obstruction and catheter interventional techniques (balloon angioplasty and
stents) are available alternatives.
Symptomatic neonates and infants should undergo urgent intervention soon after the infant is
stabilized. Asymptomatic infants, children, adolescents, and adults should undergo the
procedure electively. If neither hypertension nor heart failure is present, elective surgical or
balloon therapy in children aged 2-5 years is suggested. Waiting beyond age 5 years for surgery
or balloon therapy to relieve aortic obstruction is not advisable because of the evidence of
residual hypertension if intervention is performed after age 5 years. (15)
Since the introduction of surgical correction by Crafoord and Nylin (1945) and by Gross and
Hufnagel (1945) in the early 1940s, surgical therapy has been the preferred treatment for aortic
coarctation. Various surgical techniques have been used to treat patients with coarctation of
the aorta, namely, resection and end-to-end anastomosis, patch aortoplasty, left subclavian
flap aortoplasty, and tubular bypass grafts. The techniques are occasionally combined or
modified to fit the individual patient's anatomy. (16) In most centers, associated defects may be
addressed at the time of surgery for coarctation by proceeding with a median sternotomy to
repair lesions.
Some groups of workers consider balloon angioplasty the initial therapy of choice and reserve
surgical intervention for the following coarctations:
Coarctations that involve the long segment of the aorta
Coarctations that are completely or almost completely occluded so that no catheter or guide
wire can be passed across the coarcted segment
Coarctations that is associated with a large patent ductus arteriosus and ventricular septal
defect that requires prompt surgical intervention for the primary cardiac problem.
Surgery compared with balloon therapy:
Based on reviews, the effectiveness of balloon angioplasty appears to be comparable with that
of surgery; the mortality rates are similar and are probably related to the associated cardiac
defects, not related to type of intervention performed), and morbidity and complication rates
are lower with balloon angioplasty than with surgical therapy. Balloon angioplasty may be an
effective alternative to surgery for the relief of aortic coarctation. (17, 18)
Summary of treatment:
At initial presentation, if cardiac failure or hypertension is the presenting problem, it should be
addressed to stabilize the patient. Subsequently, the aortic obstruction should be relieved. The
available options include surgical and catheter interventional procedures; the latter procedures
include balloon angioplasty and bare or covered stents.
In general, surgical intervention in neonates and young infants (extremely ill babies with poor
left ventricular function may benefit from balloon angioplasty), balloon angioplasty in children,
and stent deployment in adolescents and adults appear to be appropriate options. Of course,
the anatomy of the coarcted aortic segment and the aortic arch would greatly influence the
method of therapy in a given patient.
Follow up
Assess postoperative BP response to exercise prior to discontinuation of antihypertensive
therapy.
Many patients may be weaned from antihypertensive therapy over months or years following
repair of coarctation. Other patients may require some form of ongoing antihypertensive
therapy.
Search for late complications of coarctation repair, including recurrent coarctation and
aneurysm formation.
Patients should receive antibiotic prophylaxis before undergoing any bacteremia-producing
surgery or procedures, especially in view of reported incidence of bacterial endocarditis during
follow-up. (19)
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