Coarctation of the Aorta
Jo Ann Nieves, MSN, ARNP, CPN, PNP-BC
Nur Practitioner, Adult Congenital Heart Program
Miami Children’s Hospital
Miami, Florida
Diana Arias, MSN, ARNP, FNP-C, CPN
Clinical Specialist, Cardiac Intensive Care Unit
Miami Childre n’s Hospital
书本封面
Miami, Florida
I. Embryology
A. Affects 5% to 8% of all newborns with congenital heart dia
B. Occurs during the 6th to 8th week of gestation
1. Cau of Coarctation of the Aorta (CoA) is unknown. Two theories (Beekman, 2008):
a. Ductus Tissue Theory - Postnatal constriction of aberrant ductal tissue
b. Hemodynamic Theory - Intrauterine alterations of blood flow through the
aortic arch
II. Abnormal development
A.Deformity of the aortic isthmus (where the ductus arteriosus joins the descending aorta) –
characterized by narrowing of the proximal aorta or distal to the left subclavian artery. (Moon, 2011).
1. Localized stenosis - a shelf-like infolding of the posterior aortic wall into the aortic
lumen opposite, proximal and/or distal to the ductus arteriosus (Kaemmerer, 2011)
拓怎么读2.Long hypoplastic gment- a tubular hypoplasia involving the aortic arch or the aorta
distal to the origin of the left subclavian artery and the ductal area (Kaemmerer, 2011)
Coarctation at aortic isthmus Illustrations reprinted from PedHeart Resource.
© Scientific Software Solutions, 2010. All rights rerved.
B. Simple CoA - coarctation in the abnce of other lesions
C. Complex CoA
1. Include intracardiac and/or extracardiac lesions (Kaemmerer, 2011):
计算机一级报名时间a.Bicuspid Aortic valve – occurs in nearly 85%, the valve may be stenotic or
the annulus hypoplastic
b.Ventricular ptal defect
c.Transposition of the great arteries
d.Noncardiac anomaly- aneurysm of circle of Willis in 3% to 5%
D. Genetic component
1. Turner XO syndrome- 35% of patients affected
III. Physiology
A.Left ventricular hypertension- narrowing of the aorta caus incread resistance to left
ventricular outflow therefore elevating systolic pressure
1. Upper extremity hypertension
2. Lower extremity BP lower than the upper body BP
3. “Gradient” is the difference between higher upper body & decread lower body BP
4. Fully oxygenated arterial blood – unless other lesions are prent
a.Closure of foramen ovale & ductus arteriosus after birth caus entire cardiac
output to flow through the stenotic aortic gment (Beekman, 2008)
IV. Clinical Features:
A. Cardinal features (Kaemmerer, 2011)
1.Upper body hypertension; weak, delayed femoral puls; a decrea in blood pressure
between upper and lower extremities; palpable collateral arteries over the medial
aspect of the scapulae, the lateral chest wall, and between the ribs
2. Thrill- suprasternal notch or neck vesls
3. Heave- no displaced heart sound
B. Infant
1. In vere CoA of the newborn, survival depends on patency of the ductus arteroisus
a.When ductus arteriosus clos (approximately 8 to 10 days of life) the patient
develops shock & heart failure. Metabolic disturbances, hypothermia,
hypoglycemia can occur as well.
b.Resulting in lower body & renal hypoperfusion, renal failure and/or
necrotizing enterocolitis (Beekman, 2008)
C. Child or adolescent
1. Upper extremity hypertension
a. Widened pul pressure as patient gets older
b. Variability of Right and Left Arm pressures, dependent on location of CoA in
relation to the left & right subclavian artery
2. Murmurs
a.Grade 2/6 to 3/6 systolic ejection murmur at the upper left sternal border, at
the ba & left interscapular space (Beekman, 2008)
D. Adults
1.Patients typically diagnod & treated earlier in life, but may rarely prent with
upper extremity hypertension as an adult with a native CoA (Daniels, 2008)
V. Medical/surgical interventions
A. Diagnosis: most often via clinical exam, echocardiogram, and chest x-ray, MRI or CT
1.Diagnostic cardiac catheterization (Beekman, 2008)
a.With complex anatomy and hemodynamics, of associated lesions
b. With additional clinical questions
B. Treatment & Timing: Individualized to lesion, associated conditions
1.Infant:
a.If vere: signs occur in first hours of life
(1) Immediate intervention required
(2)Medical – initial stabilization, inotropic support
(3)Prostaglandin E1 IV
Maintain open ductus arteriosus
Allows for flow from RV to enter MPA, cross the ductus,
enter the aorta, and perfu the descending aorta, renal and
menteric arteries
b. Surgical CoA repair
(1) Individualized to anatomy of CoA
(2)Plan to include treatment of any additional cardiac defects
2.Child, Adolescent
a.Repair at 2 to 3 years of age
b.Upon diagnosis
C. Surgery -4 Common types of repair- regardless of technique, usually performed via a left thoracotomy incision
1. End-to-end anastomosis– 1954 by Crawford and Nylin (See illustration below)
a. Surgical treatment of choice in most centers
春节主题征文
b. Excision of CoA area, circumferential anastomosis is completed with
interrupted sutures anteriorly (Beekman)
Ductal Segment
End-to-End Anastomosis with removal of Ductal Segment
Illustrations reprinted from PedHeart Resource.
借款条范本© Scientific Software Solutions, 2010. All rights rerved.
汽车保养市场2. Left subclavian flap – 1966 by Waldhaun and Nahrwold (Beekman, 2008)
a. Ligate left subclavian artery, open the proximal subclavian artery and
beyond the CoA
b. Fold subclavian artery flap down over the CoA ction and suture
into place
Subclavian artery
Flap
Coarctation Repair with Left Subclavian Flap
Illustrations reprinted from PedHeart Resource.
© Scientific Software Solutions, 2010. All rights rerved.
3. Prosthetic patch aortoplasty- 1961 by Vosschulte (Beekman, 2008)
a. Longitudinal incision is made across the CoA
b. Area enlarged with a Dacron or Gore-tex patch
4. Bypass graft – a tube is wn in between ascending and descending thoracic aorta
D. Outcomes
1. Mortality rates vary on patient age and associated lesions (Kaemmerer, 2011)
a. Simple CoA- Less than 1% mortality
b. Age 2 to 5- best age to electively operate due to low surgical risk
c. Death rates strongly related to complexity of lesions
眉毛里有痣d. After age 30 or 40- intraoperative mortality rate increas due to degenerative
changes to the aortic wall.
2. Morbidity
a. Post op –potential paradoxical hypertension, spinal cord ischemia & paralysis,
recurrent laryngeal or phrenic nerve injury, chylothorax, bleeding, infection
E. Significant long term issues: See Section VII B
VI. Cardiac Catheterization: Interventional (Beekman, 2008)
A. Balloon angioplasty began 1982,
1. Initially accepted for treatment of re-coarctaton
2. Not completely accepted for treating primary CoA
3. Balloon Angioplasty – enlarges CoA lumen, produces linear intimal and
medial tears at the CoA site
Balloon Angioplasty with Implantation of Stent
Illustrations reprinted from PedHeart Resource.
© Scientific Software Solutions, 2010. All rights rerved.
4. Risk: Aneurysm from extension to adventitia
B. Stent implantation following CoA angioplasty (See illustration above)
1. Endovascular buttress, supports dilated aortic gment
孔子思想主张2. Restenosis uncommon
3. Redilatation may be needed as child grows
C. Outcomes:
1. Mortality- rare beyond newborn period
a. Higher rate has been reported for angioplasty for recurrent post op CoA versus
native CoA
2. Acute complications (Beekman, 2008)
a. Femoral artery injury and thrombosis- common in infants younger than 12
months
b. Femoral artery hemorrhage
c. Cerebrovascular accident
3.Long-term problems
