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Coarctation of the aorta (CoA) is a relatively common defect that accounts for 5-8% of
all congenital heart defect. Coarctation of the aorta may occur as an isolated defect or in
association with various other lesions, most commonly bicuspid aortic valve and ventricular
septal defect (VSD).
DEFINITION
•
DIAGNOSTIC EXAMS/TEST
• Most often via clinical exam, echocardiogram, and chest x-ray, MRI or CT
• Diagnostic cardiac catheterization only if anatomy and hemodynamics, associated
lesions are more complex, and additional clinical questions are present (Beekman,
2008)
• Elevated blood pressures to be noted in the right arm.
• Assess patient for the presence of of pulse equality in the upper and lower
extremities.
• Obtain blood pressure in the right arm and either leg.
MEDICAL/SURGICAL MANAGEMENT
Treatment & Timing:
• o Individualized to lesion, associated conditions o Infant: If severe CoA, signs occur in first hours of life
• Immediate intervention required
• Medical – initial stabilization, inotropic support
• Prostaglandin E1 IV - maintain open ductus arteriosus o Allows for flow from RV to enter MPA, cross
the ductus, enters the aorta & perfuses the descending aorta, renal & mesentery arteries
• Surgical CoA repair
• May require individualized plan to treat any additional cardiac defects o Child, adolescent
• Repair at 2 to 3 years of age, or upon diagnosis o Adult
• In adults, endovascular stenting by cardiac cath has largely supplanted traditional surgery (Bhatt,
2015)
• o Left subclavian flap – 1966 by Waldhausen and Nahrwold (Beekman, 2008)
• Ligate left subclavian artery, open the proximal subclavian artery and beyond the CoA
• Subclavian artery flap is folded down over the CoA section and sutured into place
• Prosthetic patch aortoplasty- 1961 by Vosschulte (Beekman, 2008)
• Longitudinal incision is made across the CoA
• Area enlarged with a Dacron or Gore-Tex® patch o Bypass graft
• A tube is sewn in between the ascending &descending thoracic aorta o Outcomes
> Mortality rates vary on patient age and associated lesions
(Kaemmerer, 2011)
• Simple CoA- Low mortality: Neonate 2.1%; Infant 0.64%; Child 0% (STS.org,
2016)
• Age 2 to 5- best age to electively operate due to low surgical risk
• Death rates strongly related to complexity of any additional lesions
• Rarely diagnosed in adults > 40 year old (Bhatt, et al., 2015). Untreated CoA
has 75% mortality by age 46 years (Bhatt, et al., 2015
• After age 30 or 40- intraoperative mortality rate increases due to
degenerative changes to the aortic wall.
CONT…
> Morbidity
• Post-operative risks: o Potential paradoxical hypertension
• Spinal cord ischemia & paralysis
• Recurrent laryngeal or phrenic nerve injury
• Chylothorax
• Bleeding
• Infection
CONT…
Cardiac Catheterization: Interventional, Balloon angioplasty, potential stent
• Balloon angioplasty
• Began 1982
• Widely accepted for treating re-coarctaton
• Enlarges CoA lumen
• Produces linear intimal and medial tears at the CoA site
• Artery tear -may extend to adventitia – risk aneurysm
• Endovascular buttress, supports the arterial wall and opposes the torn media to the intima
(Krieger, 2015)
• Restenosis uncommon
• Allows for redilatation if needed as child grows, typically every 3-5 years (Beekman, 2008
• Covered stents o First covered stents (CP Covered stents) approved for use in
CoA in 2016 o Can be used to exclude an aneurysm or reduce bleeding after
intimal tear (Krieger, 2015)
NURSING MANAGEMENT AND RESPONSIBILITIES
• Monitoring for signs and symptoms of heart failure (decrease cardiac output or
fluid volume overload): fluid in lungs with crackles, difficulty breathing,
increased respiratory rate, fatigue, trouble feeding.
• Monitoring and reporting blood pressure and pulse differences in upper and
lower extremities.
• Mild cases…may go undetected until later in childhood when abnormal blood
pressures or pulses are detected in the upper vs. lower extremities…sometimes
a heart murmur may be present (systolic) that is located on the back on the left
side near the shoulder blades “interscapular”
CONT…
• Aorta can narrow again at some point and will require a procedure to re-
open
• Hypertension: some child can continue to experience hypertension long-term
and will need medications to control
• Long-term follow-up care with a cardiologist
AORTIC STENOSIS
AORTIC STENOSIS
• The heart has four valves that direct blood from the atria to the ventricles and
out into the lung vessels and the body. The aortic valve separates the left
ventricle from the aorta; in most patients, this valve consists of three leaflets
(tricuspid) that open and close smoothly and completely in response to
pressure changes during systole and diastole. When the aortic valve is
stenosed, it forces the left ventricle to work harder to overcome both diastolic
pressure and pressure caused by the stenosis. Increased contraction strength is
a compensatory mechanism used to maintain stroke volume and cardiac output
CONT…
• Over time, the increased resistance leads to muscle thickening, or dilation, and
left ventricular failure. The result of muscle thickening is decreased muscle
elasticity, ca pacity, and coronary artery blood flow and increased
myocardial workload and oxygen use. Dilation reduces the strength of muscle
contractions, which results in less blood ejected with each beat, causing
pressure to build in the atrium. The increased pressure in the pulmonary
vascular system along with left atrial dilation leads to right-sided heart failure
(HF).
CONT…
• Calcium deposits within the valve leaflets, the most common cause of AS, lead
to bulging and restriction of leaflet motion. Because calcification of the valve
is more common in the elderly, AS was originally thought to be a degenerative
disease. However, it’s now believed to be caused by an inflammatory process,
similar to the progression of atherosclerosis. In addition, history of rheumatic
fever and a congenital bicuspid valve can also lead to AS
CONT.
• Clinical presentation AS ranges from mild (Stage A) to very severe (Stage D). In
many patients, the symptoms caused by compensatory changes in the heart aren’t
apparent until the disease has progressed to a severe state. For example, initial
symptoms, such as exercise intolerance, may be overlooked or attributed to other
causes. The classic clinical AS symptoms are:
• angina, brought on by exertion and relieved with rest, caused by increased oxygen
demand and reduced coronary blood flow from the hypertrophied left ventricle.
• syncope with exertion caused by systemic vasodilation that leads to a drop in blood
pressure and a decrease in cerebral perfusion.
• dyspnea with exertion caused by pulmonary congestion, leading to HF symptoms
such as orthopnea.
DIAGNOSIS AND EVALUATION
• Transthoracic echocardiography CLINICAL BOTTOML LINE: Screening
• Noninvasive Risk factors
• Relatively inexpensive Advanced age
Atherosclerotic disease
• Provides information about History of rheumatic heart disease
• Stenosis severity
Screen only if symptoms or signs suggest valvular heart
• Cardiac function disease
• Presence and severity of valve abnormalities
Cardiac murmur
Ejection click
Use transthoracic echocardiography
WHAT SYMPTOMS OR CONDITIONS SHOULD PROMPT
CLINICIANS TO CONSIDER AORTIC STENOSIS?
3 cardinal symptoms
Angina
Dyspnea
Presyncope or syncope
Once these occur, risk of death increases
From <1% per year to 2% per month
75% of symptomatic patients die within 3 years unless they receive a
valve replacement
WHAT PHYSICAL EXAMINATION FINDINGS
INDICATE POSSIBLE AORTIC STENOSIS?
• Stage B (progressive)
• Mild or moderate aortic stenosis with normal LVEF, possible early diastolic
dysfunction
• Asymptomatic; mild-to-moderate leaflet calcification, some restriction of leaflet
motion, rheumatic valve changes with thickening of leaflets, and fusion of
commissures
• Stage C (asymptomatic severe)
• C1: Severe aortic stenosis in which the left or right ventricle remains
compensated, LVEF ≥0.50
• C2: Severe aortic stenosis with decompensation of the left or right ventricle, LVEF
<0.50
• LV hypertrophy and diastolic dysfunction
• Asymptomatic; severe calcification and severe restriction of leaflet motion
• Stage D (symptomatic severe)
• Severe aortic stenosis with or without ventricular decompensation and normal or
severely depressed LV function
• Symptomatic + stage C features
MEDICAL MANAGEMENT
Asymptomatic patients
For patients diagnosed while still asymptomatic and in Stages A-C, focus treatment on
disease monitoring, including routine follow-up appointments, echocardiographic monitoring,
and modifying cardiovascular risk factors such as smoking, diabetes, obesity, hyperlipidemia,
and hypertension. Encourage patients to remain active and exercise regularly and to take
medications as prescribed for comorbid conditions. If your asymptomatic patient has
hypertension, common in those with AS, treatment should follow evidence-based guidelines.
Angiotensin-converting enzyme (ACE) inhibitors, beta blockers, calcium channel blockers, or
thiazide-type diuretics may be prescribed. To avoid hypotensive episodes that can cause
symptoms, such as dizziness or lightheadedness, and worsen the patient’s condition,
antihypertensive medications should be started at low doses and titrated with care
Symptomatic patients
• For symptomatic AS, medical management is complex and will depend on severity of
symptoms. In addition to risk factor modification, care should focus on maximizing cardiac
function and minimizing symptoms and complications, including angina, syncope, and HF.
Treatment options to relieve your patient’s angina may include careful use of beta blockers
and nitrates to reduce myocardial oxygen consumption and dilate the coronary arteries.
Symptoms of angina may resolve with cautious use of low-dose nitroglycerine (for example,
0.3 mg sublingual tablets), as prescribed. If related to activity, physical restrictions may be
necessary, with planned periods of rest to reduce oxygen consumption and conserve
energy. Syncope may require an antiarrhythmic medication, pacemaker, or internal cardiac
defibrillator. If syncope is related to an arrhythmia, such as new-onset atrial fibrillation,
cardioversion may be required. If the event is related to exertion, advise the patient to
minimize stressful activities.
CONT…
• Symptoms of HF, such as pulmonary congestion, should be treated with diuretics or positive
inotropes such as digoxin. How ever, be careful to avoid aggressive reduction of preload,
which can lead to insufficient cardiac output and hemodynamic compromise. Because
patients with HF are at high risk for decompensation, which can lead to critical instability,
help them maintain a healthy fluid and electrolyte balance. Critically ill patients with
decompensated HF may require I.V. medications such as nitroprusside, invasive
hemodynamic monitoring, and assistive devices such as an intraaortic balloon pump to
improve cardiac output. ACE inhibitors or angiotensin receptor blockers may be used to
manage hypertension; however, be careful to avoid hypotension. Low blood pressure can
affect the heart’s pumping ability.
NURSING MANAGEMENT AND RESPONSIBILITIES
• Hemodynamic instability—Evaluate your patient for shortness of breath,
angina, hypoxia, and changes in blood pressure, pulmonary artery pressure,
and heart rate or rhythm.
• Cardiac assessment—Note the presence of a strong apical beat, weak or
delayed carotid pulse, displacement of the apex of the heart inferiorly and
laterally, presence of an ejection click, or a low-pitched harsh, systolic murmur
that radiates to the neck.
• Oxygen therapy—Monitor your patient’s oxygen saturation via pulse
oximetry, arterial blood gases, and mixed venous oxygen saturation.
CONT…
• Fluid status—Assess your patientfor the presence of pulmonary congestion
and peripheral edema, avoid extended periods of nothing by mouth
before tests or procedures so your patient doesn’t become dehydrated,
and check fluid volume status via daily weights and by monitoring intake
and output.
• Thromboembolic events—Monitor your patient with atrial fibrillation or
severely compromised left ventricular function for therapeutic
anticoagulation. Anticoagulation therapy will be lifelong for patients after
a mechanical valve replacement. Pneumatic sleeves should be used to avoid
deep vein thrombosis during hospitalization.
• Talk with your patient and his or her family about prescribed medications and the importance
of taking them as ordered, and explain the rationale behind the medication, as well as its
dosage, timing, and side effects.
• Discuss how to monitor for new or worsening symptoms, and clarify what should be reported
to the healthcare provider.
• Work with your patient to develop self-management strategies by providing education about
sodium and fluid restrictions, a hearthealthy diet, and other lifestyle changes, such as weight
loss and smoking cessation.
• To help prevent falls, instruct your patient to change positions slowly (lying to sitting or
standing) to minimize orthostatic blood pressure changes.
• Patients with advanced AS may experience anxiety and depression. Teach relaxation
techniques such as deep breathing or guided imagery that the patient can use to deal with
uncomfortable or stressful symptoms.
• Patients with symptomatic severe AS who refuse AVR or TAVR/I or
for whom these procedures would be futile may benefit from
palliative therapy. Therapies range from nonpharmacologic
options, such as Reiki, art, music, or animalassisted therapy, to
medical therapies that relieve symptoms of fatigue, dyspnea,
angina, nausea, and palliative procedures, such as a percutaneous
balloon valvuloplasty. The goal is to relieve the burden of
symptoms to improve the patient’s quality of life.
END