Coarctation of the Aorta with Intact Ventricular Septum
These babies present in acute congestive
heart failure and appear very ill. This is due to left ventricular
failure. The left ventricle is burdened in normal heart by labor and
delivery (assuming systemic ventricle status), this will be aggravated by
coarctation leading to high left atrial pressure and high pulmonary
vascular resistance leading into right ventricular failure as well.
Prostaglandin improves condition because of
opening of patent ductus arteriosus or the ampulla at the aortic end and
will improve perfusion to the lower extremities and reduce the pressure
overload on the left ventricle.
Electrocardiography shows right ventricular
hypertrophy in the first six months and after that left ventricular
hypertrophy. Typically left ventricular hypertrophy is not seen in the
first six months of life.
Echocardiography shows the lesion and its
extent. It is important to rule out other lesions such as ventricular
septal defect, bicuspid aortic valve, aortic stenosis, mitral valve
abnormalities, patent ductus arteriosus or atrial septal defect. These
lesions could be associated with coarctation of the aorta.
Cardiac catheterization is done only when
anatomy is complex. Balloon dilatation may be performed if there is added
risk to surgery.
Surgery is the treatment of choice and the
earlier it is done the better. Rate of recurrence is 9-24%.
In surgery the left subclavian artery is
frequently sacrificed. In fact, complications from such a procedure is
rare. Recoarctation is treated with balloon valvuloplasty. 9% of simple
coarctations tend to recoarctation, 12% of coarctations with VSD develop
recoarctation and 24% of patients with interrupted aortic arch develop
Coarctation of the
Aorta with Ventricular Septal Defect
40% of babies with coarctation have
ventricular septal defects.
Almost all present in the neonatal period.
Because of ventricular septal defect an increased left-to-right shunting
results in congestive heart failure.
Chest x-ray shows signs of congestive heart
failure and increased pulmonary blood flow.
The ventricular septal defect could be
anywhere and no specific type is more common with coarctation of the
Treatment is surgical by repairing the
coarctation and either main pulmonary artery banding, both could be
performed from a lateral thoracotomy. Occasionally the ventricular septal
defect could also be repaired.
Coarctation of the
Aorta with Mitral Valve Abnormalities
Mitral valve abnormalities are seen in 25% of
patients with coarctation. 2% of patients have severe abnormalities and
10% of patients also have mitral regurgitation. Incidence of mitral
stenosis is higher with ventricular septal defect and coarctation as
compared with coarctation alone.
Mitral stenosis may be secondary to parachute
mitral valve or hypoplasia of the mitral valve ring or supravalvar ring.
Pressure gradient across the mitral valve may be exaggerated by a
ventricular septal defect due to left-to-right shunting and increased
blood flow across the mitral valve. Therefore, ventricular septal defect
closure may; decrease the pressure gradient. Mitral regurgitation even
mild may not be tolerated because of the already high pulmonary venous
Coarctation of the
Aorta with Aortic Stenosis
21% of patients with coarctation have a
normal aortic valve but only 2% are severe enough requiring surgery. 5% of
all critically ill infants with coarctation also have aortic stenosis.
Aortic stenosis could be valvar or subvalvar.
Aortic stenosis leads to decreased cardiac output with poor pulses in all
extremities masking upper and lower extremity pulse difference.
Treatment may be by repairing coarctation
first and balloon valvoplasty of the aortic valve.
Coarctation of the aorta may occur with
complex cyanotic congenital heart disease. However, it is not known to
occur with tetralogy of Fallot.
0.019 Per 1,000 live births. 1.3% of all
critically ill neonates present in 5% of all patients with aortic
- Type A is when the interruption is distal
to the left subclavian.
- Type B is when the interruption is between
the left subclavian and left carotid artery
- Type C is when the interruption is between
the left and right carotid arteries
Aortic valve deformities are present in 60%
of cases. VSD is very common. Anomalous subclavian artery is very common
especially with Type B interruption. DiGeorge syndrome is reported in 48%
Interrupted aortic arch presents like severe
coarctations but usually earlier in life.
Surgical end-to-end anastomosis or by using
Abdominal coarctation is seen with unusual
site of coarctation, Williamís syndrome, Takayasuís disease and
Fever and abdominal pain. This is more common
in males than females and usually in children less than 5 years of age. It
is very rare.
Increased circulating rennin and
catecholamines leading to mesenteric arteries spasm.
Symptoms and signs:
Fever, abdominal pain, leukocytosis, melena,
abdominal x-ray shows bowel distention.
NPO, IV fluid and hyperalimentation and
intra-lipid nutrition. Surgery if no improvement.
Hypertension in upper and lower extremities
immediately post-operatively. This may last for 1-3 weeks. Dorsalis pedis
pulses are palpated well. More common in youngster children.
Combination of poorly developed vasculature
distal to coarctation, hyperkinetic heart, hyperreninemia and fluid
Nitroprusside drip, Labetolol drip and then
boluses, Hydralazine, Esmolol and chlorthiazide may be used.