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33 Adult Congenital Heart Disease
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Fig. 33.4 Unroong
technique for repair of
anomalous coronary artery
from the right pulmonary
artery
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• Other less commonly used techniques.
– Osteoplasty (creation of new ostium at the end of the ectopic artery’s intramu-
ral segment).
– Direct reimplantation of ectopic artery at aortic root.
*Coronary bypass is NOT preferred due to high risk of graft failure due to com-
petitive ow* [8].
Vascular Rings
A vascular ring is a malformation of the aorta and its major branch vessels in which
the vessels partially or completely encircle the aerodigestive tract. In the normal
embryologic development of the aorta, the 6 major aortic (branchial) arches involute or migrate to form the aortic arch and major branch vessels. Abnormalities in
this development can lead to aberrancies in laterality and origin of branch vessels,
causing the formation of vascular rings.
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S. S. Li and J. P. Bloom
Anatomy/Pathophysiology [9]
Normal Anatomy/Development (Fig.33.5)
– 6 aortic (branchial) arches bilaterally.
– Migration and involution result in nal anatomy of aortic arch and major branch
vessels.
Fig. 33.5 Normal embryological development of the aorta
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– Left fourth aortic arch—>normal left-sided adult aorta.
– Right fourth aortic arch—>right subclavian artery.
Embryonic
vessel Outcome
Truncus
arteriosus
Aortic sac Distal ascending aorta
1st arch Maxillary artery
2nd arch Stapedial artery
3rd arch Common carotid artery
4th arch Right: proximal right subclavian artery
5th arch Involutes
6th arch Right proximal: right pulmonary artery
Right dorsal
aorta
Left dorsal aorta Aortic arch distal to left subclavian artery
Proximal ascending aorta
Pulmonary root
Brachiocephalic artery
Arch up to origin of left common carotid artery
Proximal internal carotid artery
Left: aortic arch (segment between left common carotid and left subclavian
arteries)
Right distal: involutes
Left proximal: left pulmonary artery
Left distal: ductus arteriosus
Cranial portion: right subclavian artery
Distal portion: involutes
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Vascular Ring Anatomy
Type IA: double aortic arch – Right dorsal aorta fails to involute
Type IB: vascular rings
Right aortic arch +
retroesophageal left subclavian
artery
Right aortic arch + mirror image
branching
Left aortic arch + aberrant right
subclavian artery
– Persistence of right and left fourth aortic arches
– Descending aorta on the left
– Each subclavian and common carotid arises from its
respective arch
– Left fourth arch involutes while right fourth arch
persists
– Left subclavian artery arises aberrantly behind the
esophagus from the right-sided arch
– Left-sided ligamentum arteriosum joins left
pulmonary artery to complete ring
– Left fourth arch involutes while right fourth arch
persists
– Left innominate arises anteriorly
– Ligamentum arteriosum arising from aorta (behind
esophagus) to left pulmonary artery—>forms ring
– Right subclavian artery originates distal to left
subclavian artery with retroesophageal course
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Fig. 33.6 Compression of
aerodigestive tract from
vascular ring
S. S. Li and J. P. Bloom
Presentation
• Compression of trachea and esophagus (Fig.33.6).
– Dysphagia lusoria.
– Stridor.
– Recurrent respiratory infections.
Kommerell’s Diverticulum
• Arises from a remnant fourth dorsal aortic arch.
• Can occur in both left and right aortic arch anatomy.
– Aberrant subclavian artery rises to the contralateral side.
• Consider surgery when symptomatic or diverticulum orice >3cm or descending aorta >5cm.
• Options for repair.
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– Open surgical repair.
– Hybrid endovascular repair.
– Total endovascular repair.
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Adult Coarctation oftheAorta (CoA)
While most patients with CoA are diagnosed in childhood, about 10% of patients
will present in adulthood [10]. Unlike pediatric coarctation in which surgery is the
treatment of choice, adult coarctation is often managed with transcatheter
approaches.
Presentation
• Symptoms:
– Hypertension (most common).
– Leg claudication.
– Mesenteric angina.
– Headache.
– Tinnitus.
– Epistaxis.
• Exam:
– Delayed or weak femoral pulses.
– Continuous systolic–diastolic murmur between scapulae (blood ow through
collateral vessels).
Surgical Repair [11]
• Indications for intervention:
– BP difference >20 mmHg between upper and lower limbs (regardless of
symptoms) with:
Upper limb hypertension (>140/90mmHg).
Pathologic BP response during exercise.
Signicant LV hypertrophy.
– Hypertension with ≥50% aortic narrowing relative to aortic diameter at the
level of the diaphragm.
• Surgery versus catheter-based interventions (Fig.33.7):
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a
c
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S. S. Li and J. P. Bloom
b
Fig. 33.7 Surgical interventions for aortic coarctation: (a) resection with end-to-end anastomosis,
(b) patch angioplasty enlargement, (c) resection with interposition graft, and (d) subclavian ap
enlargement
Key points Types of repair
Surgery Preferred in pediatric
patients
– In adults, increased
mortality risk due to
degenerate changes in
aortic wall
Catheterbased
Preferred in adults
– Treatment of both
native coarctation and
re-coarctation or aneurysm
after initial repair
d
Resection with end-to-end anastomosis
– High rates of re-coarctation due to narrowing
of the suture line
Patch aortoplasty
– PTFE patch is sewed at the level of the
coarctation to expand the diameter of the aorta
– Aneurysms form in 20–40% of cases opposite
to patch
– Only used in complex arch reconstruction
Subclavian ap aortoplasty
– Subclavian artery is sacriced and the orice
enlarged to create a ap using intrinsic tissue
– Avoids the use of patch or graft
– Low re-coarctation rate (3%)
Interposition graft
– Graft will not grow with the patient
– Preferred in adult-sized patients
Balloon angioplasty
– High incidence of re-stenosis when used alone
Stenting
– Sustained hemodynamic benet
– Reducing risk of dissection and aneurysm by
tacking intimal aps to aortic wall
*Balloon angioplasty+stenting is preferred
treatment in adults
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Fig. 33.8 Morphologic
classication of bicuspid
aortic valves. Type 0: no
raphe, type 1: one raphe,
type 2: two raphes
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Bicuspid Aortic Valve
Bicuspid aortic valves are one of the most common congenital abnormalities in
adults and are found in 1–2% of the population. The management of patients with
bicuspid aortic valves do not signicantly differ from the general public, although
may require more frequent monitoring for valve degeneration or aortic dilatation.
Anatomy
• Bicuspid aortic valves arise when adjacent leaets fail to separate—>only 2
cusps rather than the usual 3 (right, left, and noncoronary).
• Morphology varies according to which commissures are fused (Fig.33.8).
• Associated with coronary artery anomalies in 2% of patients.
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S. S. Li and J. P. Bloom
Presentation
• Increased risk of valvular degeneration.
– Aortic stenosis.
Harsh systolic murmur at left upper sternal border.
Fatigue, dyspnea, syncope.
– Aortic regurgitation.
Diastolic murmur.
Pulmonary congestion and right heart overload.
• Increased risk of aortic aneurysms.
– Theories for the etiology of this risk include intrinsic tissue weakness versus
abnormal ow dynamics leading to skewed stress on the aortic wall.
Surgery
• Options:
– Aortic valve replacement.
Considered in severe aortic stenosis or damaged valves.
Mechanical or bioprosthetic options depending on patient age, likelihood
of medication compliance and bleeding risk (as patients with mechanical
valves will require lifelong anticoagulation).
– Aortic valve repair.
Can be considered when leaets themselves are salvageable (i.e., not
thickened or sclerotic).
Involves resuspension of the commissures, oversewing aortic clefts, or plication of oppy leaets (Fig.33.9).
• Aortic root or ascending aorta replacement if diameter >5.5cm.
– Previously, bicuspid aortic valves were considered higher risk for aortic dis-
section in patients with aortic aneurysms, and the threshold for intervention
was lower (>4.5–5cm). Current guidelines recommend standard thresholds
for aortic root/ascending replacement.
a
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Fig. 33.9 Aortic valve
repair with resuspension of
commissures (a) and
plication of cusps (b)
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b
Cor Triatriatum Dexter [12]
Cor triatriatum is an abnormal septation within the atrium that leads to inow
obstruction to the ventricle. While cor triatriatum can occur on either side, obstruction on the left leads to a cor triatriatum sinister (CTS) which, as its name suggests,
is a more severe variant presenting in childhood. Cor triatriatum dexter (CTD) has
a milder presentation and may remain undetected until adulthood.
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Fig. 33.10 (a) Transesophageal echocardiography demonstrating cor triatriatum dexter (CTD) (b)
with Doppler demonstrating area of ow through a foramen in the membrane
S. S. Li and J. P. Bloom
Anatomy
• Abnormal septation in the atrium (can be complete, incomplete, or fenestrated)
leads to obstruction of blood ow passing from the atrium to the ventricle
(Fig.33.10).
• Membrane exists between the smooth and trabeculated parts of the atrium.
– Arises due to persistence of the right valve of the right horn of the sinus
venosus.
• Most common location of the membrane:
– To the right of the SVC, IVC, and coronary sinus.
– To the left of the coronary sinus and right of SVC and IVC.
Presentation
Cor triatriatum is a rare congenital heart disease, and CTD less common than
CTS. Presentation and symptomatology depend on location of the membrane,
degree of septation, concomitant ASD, and associated cardiac lesions.
• Common presentations:
– Systemic venous congestion (symptoms mimic tricuspid stenosis).
Elevated JVP
Hepatic congestion
Peripheral edema
Clubbing
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