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28 Diseases oftheAorta
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297
Aortic Dissection
Anatomy andPhysiology
An aortic dissection is a tear within the layers of
the aorta. The tear occurs within the intima which
causes a separation of the media, creating a new
channel which is dened as the false lumen. The
native channel is dened as the true lumen.
Throughout the aortic dissection, there are many
communications between the true and false
lumen dened as fenestrations. The presence of
an “intimal ap,” representing the intimomedial
septum between the true and false lumen, is the
most characteristic pathology in acute aortic dissection [7] (Fig. 28.7). Aortic dissections are
classied as acute versus chronic based onset
timing and on the location of the entry tear. An
acute aortic dissection is dened as onset of
symptoms within 2weeks, subacute >14days to
90days, and chronic >90days.
Historically, there were two classication systems: Debakey (1965) and Stanford (1970). The
Debakey classication denes the intimal tear
and the extent of aortic dissection. The standard
Stanford classication describes only the tear and
denes a type A as involving the proximal aorta
or type B (TBAD) which is distal to the left subclavian artery [7] (Fig.28.8).
DeBakey
Type 1. Dissection originates in the ascending
aorta, extending to descending and abdominal
aorta
Type 2. Dissection originates and is conned to
the ascending alone
Type 3. Dissection originates in the descending
aorta
3a=Supra-diaphragm
3b=Below diaphragm
The newest classication system as dened by
the Society for Vascular Surgery (SVS) in 2020
helps to overcome previous limitations and further develop more accurate communication when
describing the complex aortic dissection patient.
Within the new SVS classication scheme for
aortic dissection, the distinction between Type A
and Type B is predicated on entry tear location
alone and dened in zones [8].
When discussing aortic syndromes, intramural hematoma (IMH) as well as the penetrating
aortic ulcer (PAU) should also be included. IMH
does not have a clear tear or communication as an
aortic dissection but rather appears as hemorrhage within the aortic wall [8]. A PAU is dened
as atherosclerotic plaque that penetrates the aortic wall. PAU rupture risk is directly associated
with the ulcer depth [8].
Outer layer
Middle layer
Inner layer
Blood flow
Fig. 28.7 Development and progression of aortic dissection
AORTIC DISSECTION
False lumen
Tr ue lumen
298
Classification of Aortic Dissection
Renal ar
DeBakey Classification
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T. Totten and F. R. Arko III
Stanford Classification
Ascending
aorta
Aortic
root
Suprarenal
artery
Aortic arch
Descending
Thoracic
aorta
Diaphragm
tery
Abdominal
aorta
Healthy aorta Type I Type II Type III aType III b
Type A
Fig. 28.8 Dissection classication: Stanford and Debakey
Type B
Pathology/Description
The process of an aortic dissection is dynamic,
therefore can occur anywhere along the aorta and
present with an array of symptoms. The entry tear
occurs within the intima and media layers of the
aortic wall, creating an intimal ap in which blood
rushes into the space both proximal and distal with
multiple fenestrations between layers [7]. Rupture
of the intima and media is the initial event in most
cases of aortic dissection. The violation of the intimal surface results in formation of a cleavage
plane into the outer media and subsequent propagation for a varying distance in this plane, either
antegrade or retrograde [7] (Fig.28.7).
Aortic dissections are classied as complicated versus uncomplicated. Complicated
dissections are dened by malperfusion of end
Table 28.7 Risk factors for dissection
Uncontrolled and sudden variation in blood pressure
Cocaine
Pregnancy
Genetic predisposition (Marfan, Ehlers-Danlos
syndrome (EDS), Loeys-Dietz syndrome (LD)
Blunt force trauma
Bicuspid aortic valve
Inammatory conditions of Giant cell arteritis (GCA),
Takayasu arteritis
organs, true lumen compression, aneurysmal
degeneration, uncontrolled pain, or aortic
rupture.
Risk Factors forDissection
See Table28.7.
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Physical Exam
The patient may present with a wide array of
symptoms. The classic description is described as
a ripping, tearing, sharp, “worst ever” anterior
chest or back pain. There may be evidence of an
anxious appearing patient with tachycardia and
tachypnea. If there is a ow-limiting dissection,
the patient can present with hypoperfusion symptoms and commonly abdominal pain. The medical provider should always obtain blood pressure
readings in both upper extremities as the patient
may present with loss of pulses to extremities
which could represent ischemia. Always complete a thorough cardiac exam and auscultate for
cardiac murmur. If the dissection involves the
intracranial vessel, there could be evidence of
neurologic changes presenting with stroke,
Horner’s syndrome, voice hoarseness, and spinal
cord ischemia.
Imaging
When working up, aortic dissections consider the
following imaging modalities. The gold standard
imaging is a CT angiogram of the chest, abdomen, and pelvis. An acute dissection ap
(Fig.28.9) is thin in appearance compared with a
chronic dissection which will appear as a clearly
dened dissection ap that usually is thicker and
more dense [8]. IMH will appear as a hyperdensity within the aortic wall. PAU will appear as
an atherosclerotic lesion with ulcer-like projection within the aortic wall. A chest X-ray can
reveal widening of the cardiac or aortic silhouette
with widened mediastinum. A TTE or TEE can
be used to evaluate for any evidence of cardiac
tamponade or aortic insufciency in the setting of
a type A aortic dissection. A TTE should be
ordered with diagnosis of acute aortic dissection.
MR angiograms are not recommended. An EKG
should always be obtained in the setting of acute
type A aortic dissection. Proximal dissection into
a coronary artery (RCA) can create STEMI in
addition to aortic dissection.
Management
Prompt diagnosis and management are key, and
aortic dissections are associated with high morbidity and mortality. Type B aortic dissections are
either managed medically or occasionally surgically. The cornerstone of medical therapy is
reduction of arterial blood pressure [7]. Acute
aortic dissection patients will be admitted to ICU
for anti-impulse therapy and vasodilator therapy.
Fig. 28.9 CTA with acute thoracic aortic dissection
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T. Totten and F. R. Arko III
Beta blockade (labetalol or esmolol) is always
initiated rst as vasodilators can cause a reex
tachycardia, which can worsen the dissection.
Intravenous nicardipine is commonly used as a
vasodilator. The goal is prompt control of heart
rate and systolic blood pressure goal <120mmHg
or HR <70bpm.
Urgent surgical repair is indicated of acute
type A aortic dissections because medical treatment is associated with 60% in-hospital death
rate [7]. The anatomic goal is resection of the
aortic intimal tear to eliminate the threat of rupture and to reconstruct the aortic wall layers.
Type A dissection may require aortic valve repair/
replacement, aortic root/aortic arch/hemiarch
repair/replacement. Acute tamponade may
develop with dissection into the pericardium.
This is a surgical emergency, and pericardial tap
should be avoided as this may worsen hemodynamic collapse and delay emergent surgical
intervention.
In patients with uncomplicated type B dissection (TBAD), surgical therapy has not shown
superiority over medical therapy [7]. Therefore,
most TBAD are managed medically if uncomplicated. The goal of medical therapy is anti-impulse
control, pain control, and evaluating for malper-
fusion or disease progression. TEVAR or surgical
intervention may be warranted in TBAD with
persistent/recurring pain, uncontrolled hypertension despite medical therapies, advancing aortic
expansion, or any evidence of malperfusion. A
TEVAR may be performed to prevent late complications and promote aortic remodeling as well.
During this procedure, a stent graft is deployed in
the true lumen to cover the entry tear with the
goal of improved aortic perfusion and encouraging thrombosis of the false lumen (Fig.28.10).
Surveillance
The principal late complication of aortic dissection is aneurysmal dilatation of the outer
wall of the false lumen [7]. Regular follow-up
with serial imaging for routine surveillance is
recommended lifelong with a vascular surgery
clinic. False lumen patency or thrombosis is an
important predictor of regional luminal growth
and reintervention rate [8]. In follow-up, maximal aortic diameter is documented and followed over time. Patients with aortic dissection
undergo at least annual surveillance with CT
angiogram.
Fig. 28.10 TEVAR within true lumen excluding thrombus in false lumen
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301
Clinical Pearls
• Patients diagnosed with aortic dissection
should be referred to an aortic center when
possible.
• Anti-impulse therapy is the mainstay of treat-
ment for heart rate and blood pressure in
patients with aortic dissection.
• A bicuspid aortic valve is associated with
increased risk of aortic dissection and thoracic
aortic aneurysm.
• Genetic referral for screening should be con-
sidered in patients with acute aortic dissec-
tions, especially in the younger population.
References
1. Lawrence PF, Rigberg DA. Arterial aneurysms,
epidemiology, and natural history. Rutherford’s
vascular surgery and endovascular therapy.
2019. p. 875–83. https://www- clinicalkey- com.
ahecproxy.ncahec.net/#!/content/book/3- s2.0B9780323427913000694?scrollTo=hl0000575.
2. Jana S, Hu M, Shen M, Kassiri Z. Extracellular
matrix, regional heterogeneity of the aorta, and aortic
aneurysm. Exp Mol Med. 2019;51:1–15.
3. Dalman R, Mell M. Overview of abdominal aortic
aneurysm. 2022. https://www.uptodate.com/contents/
overview- of- abdominal- aortic- aneurysm#!.
4. Schermerhorn M, Cronenwett JL.Arterial aneurysms:
abdominal and iliac aneurysms. Rutherford vascular
surgery. 6th ed. Elsevier; 2005.
5. Abdominal aortic aneurysm. Elsevier BV; 2021.
https://www- clinicalkey- com.ahecproxy.ncahec.net/#!/content/67- s2.0- 7c5e261c- ea6f- 4cabaae7- cb8155587799.
6. White SB, Stavropoulos SW. Management of
Endoleaks following Endovascular Aneurysm
Repair. Semin Intervent Radiol. 2009 Mar;26(1):33-
8. https://doi.org/10.1055/s-0029-1208381. PMID:
21326529; PMCID: PMC3036461.
7. Black J, Cambria RP. Aortic dissection: perspectives
for the vascular/endovascular surgeon. Rutherford’s
vascular surgery. 2005. p.1512–31.
8. Lombardi JV, Hughes GC, Appoo JJ, et al. Society
for Vascular Surgery (SVS) and Society of Thoracic
Surgeons (STS) reporting standards for type B aortic
dissections. 2020. https://www.jvascsurg.org/article/
S0741- 5214(19)32649- 7/fulltext.
Part VIII
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Adult Congenital Heart Disease (ACHD)
JanaReid ArwaSaidi
1.1 Introduction
Congenital heart disease is a rapidly growing subspecialty of cardiology.
Nearly 1% of infants born in the USA have congenital heart disease [1–4].
Initially considered a disease of the pediatric population, many advances in
the eld have led to more adults now living with congenital heart disease than
children. Prior to 1940, in the absence of any meaningful treatment options,
90% of infants born with complex congenital heart disease died before adulthood [2]. Following major advancements in diagnosis and treatment, a majority of infants now live into adulthood. As such, there are currently well over
one million adults living with congenital heart disease [4, 5].
The evolution of treatment of congenital heart disease has been dramatic
over the last 60years. The development and advances in surgical, interventional, and diagnostic tools have changed the landscape of management and
have profoundly affected outcomes. The rst PDA ligation was performed in
1938. In 1944, Dr. Blalock, Dr. Taussig and Mr. Thomas devised the rst
shunt to improve pulmonary blood ow in a patient with Tetralogy of Fallot.
The invention of the heart-lung bypass machine in 1955 led to the rst repair
of an ASD, which was the start of decades of advancement in congenital heart
surgery. Catheter based procedures were developed in the 1960s and initially
treated lesions such as PDAs and ASDs and are now used in complex disease.
The rst Fontan procedure was performed in 1968, allowing children born
with a single functional ventricle to live past oneyear of age. The advent of
2D echocardiography in the 1970s permitted a major step forward in the diag-
J. Reid · A. Saidi
UF Health Congenital Heart Center, University of FL, Gainesville, FL, USA
e-mail: reidja@shands.u.edu; asaidi@pedcard.u.edu
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Adult Congenital Heart Disease (ACHD)
nosis and management of congenital heart disease. Transposition of the Great
Arteries was treated with the rst arterial switch procedure in 1975, eventually replacing the previously performed atrial switch procedure and
dramatically altering the long-term course for patients with TGA.The rst
minimally invasive heart valve replacement was performed in 2000, leading
the way for non-surgical options for a variety of congenital heart defects. 3D
imaging is now being used to help map out complex anatomy in advance of
intricate surgical interventions and stem cell therapy is an emerging eld that
is the source of many exciting research studies [6].
Hundreds of thousands of infants and children have benetted from these
advances in diagnosis, intervention, and clinical care, such that they are living
well into adulthood. While initially thought to be curative, it has been recognized over time that many of these interventions allowed children to grow up
without severe limitations, but with increased long-term morbidity and mortality, often becoming evident in the adult years. The need for uninterrupted
specialized care in an ACHD center cannot be overstated, as outcomes for
adults who have been lost to follow-up are notably worse than those who have
consistent access to care. As childhood palliative interventions reach the end
of their lifespan, adults with congenital heart disease are prone to atrial and
ventricular arrhythmias, infective endocarditis, heart failure, pulmonary
hypertension, and the need for pacemakers and/or debrillators [3, 4, 7].
They may require additional interventions for their congenital heart disease,
either via surgical or transcatheter intervention. For those with advanced disease processes that cannot be otherwise salvaged, advanced therapies including mechanical circulatory support and transplantation, sometimes
multi-organ, may be considered. Additional factors for adults with congenital
heart disease include contraception, pregnancy risk and delivery considerations, perioperative care for non-cardiac surgery and the management of
acquired cardiac and non-cardiac co-morbidities [4–7]. A multidisciplinary
model of care for adult congenital heart disease is critical to long-term health
and wellness.
Adults with congenital heart disease make up an ever growing and intriguing subset of general cardiology. Whether a simple or complex lesion, these
patients have special considerations for management. There have been many
advances in care over the last 60years and certainly the future is full of more
revolutionary innovations for those living with congenital heart defects.
References
1. Allen HD, Penny DJ, Feltes TF, Cetta F.Moss and Adams’ heart disease
in infants, children, and adolescents including the fetus and young adult.
9th ed. Wolters Kluwer; 2016.
2. Tennant PW, Pearce MS, Bythell M, Rankin J. 20-year survival of children
born with congenital anomalies: a population-based study. Lancet.
2010;375(9715):649–56. https://doi.org/10.1016/S0140- 6736(09)
61922- X.
Adult Congenital Heart Disease (ACHD)
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3. Gurvitz M, Dunn JE, Bhatt A, etal. Characteristics of adults with congenital heart defects in the United States. J Am Coll Cardiol.
2020;76(2):175–82.
4. Stout KK, Daniels CJ, Aboulhosn JA, etal. 2018 AHA/ACC Guideline for
the Management of Adults With Congenital Heart Disease: a report of the
American College of Cardiology/American Heart Association Task Force
on Clinical Practice Guidelines [published correction appears in J Am Coll
Cardiol. 2019 May 14;73(18):2361–2362]. J Am Coll Cardiol.
2019;73(12):e81–e192.
5. Baumgartner H, De Backer J, Babu-Narayan SV, etal. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J.
2021;42:563–645.
6. Kiess M.History and evolution of the treatment of adult congenital heart
disease. BCMJ. 2016;58(7):368–72.
7. Warnes CA.Adult congenital heart disease: the challenges of a lifetime.
Eur Heart J. 2017;38(26):2041–7. https://doi.org/10.1093/eurheartj/
ehw529.
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AmandaGreen andJorgeAlegria
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Simple Defects
Atrial Septal Defect (ASD) A communication
between the atria, allowing blood ow between
the systemic and pulmonary circulations [1].
These can occur in isolation or as a part of a more
complex diagnosis/constellation of defects.
Anatomy andPhysiology
Defect in the atrial septum. These can be in the
septum primum, secundum, or associated with
anomalous pulmonary veins in the case of a sinus
venosus defect (superior or inferior location).
The degree of shunting across the ASD is
determined by the size of the defect as well as the
degree of ventricular compliance. In older adults
who have decreased ventricular compliance and a
stiffer ventricle, there is a greater risk of transient
heart failure after closure of the atrial defect,
given that the stiff ventricle now must accept a
greater volume load and no longer has the “pop
off” of the atrial septum [1, 2]. The development
A. Green
Atrium Health/Sanger Heart and Vascular Institute,
Greenville, SC, USA
e-mail: amanda.green1@atriumhealth.org
J. Alegria (*)
Atrium Health/Sanger Heart and Vascular, Congenital
Heart Center, Charlotte, NC, USA
e-mail: Jorge.Alegria@atriumhealth.org
of left ventricular diastolic dysfunction with subsequent increase in left atrial pressure may result
in an increase left to right shunt in adults, especially in the presence of hypertension or coronary
artery disease.
Types ofASD
Secundum ASD: Located in septum primum, in
the region of the fossa ovalis. This is more common in females who make up 65–75% of the
patient population with secundum ASD [3].
Depending on the defect size and pulmonary vascular resistance, it can often be closed by transcatheter techniques.
Primum ASD: Also known as endocardial
cushion or AV septal defects and are associated
with abnormalities of the atrioventricular valves
[3]. Anatomically located in the septum secundum, these ASDs usually require surgical repair.
In the electrocardiogram a rst-degree atrioventricular block and left axis deviation can be
found. Left ventricular outow tract obstruction
can be present. Long-term complications in
adults may result in need of permanent pacemaker, left sided atrio-ventricular valve
replacement.
Sinus Venosus ASD: These are located along
the superior or inferior portion of the atrial
septum near the junction of the SVC or
IVC. They are often associated with partial
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
R. Musialowski, K. Allshouse (eds.), Cardiovascular Manual for the Advanced Practice Provider,
https://doi.org/10.1007/978-3-031-35819-7_29
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Sinus venosus
Secundum
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A. Green and J. Alegria
anomalous pulmonary venous return. Superior
sinus venosus is the most common form of the
two, and accounts for 5–10% of all ASDs [3].
These commonly requires surgical repair of
defect and bafing of the pulmonary veins to the
left atrium.
superior and
inferior
Coronary Sinus Septal Defect: This is a defect
in the wall of the coronary sinus also known as an
“unroofed coronary sinus,” which can result in
left to right shunting (LA CS defect RA) [3]. This
is also commonly associated with a persistent left
superior vena cava.
Primum
Physical Exam Correlations
On physical exam, there may or may not be a
murmur if the ASD is small with a low degree of
shunting. If a higher degree of left to right shunt
is present, there may be a soft systolic ejection
murmur given the increased blood ow across
the pulmonary valve annulus. S2 may have xed
splitting but this nding is not always present [3].
In patients with pulmonary hypertension, P2 may
be loud or “snappy” [3].
Adults with undiagnosed ASDs may present
with a chief complaint of fatigue, exercise intolerance, and/or palpitations [1]. Paradoxical
embolism may also occur.
EKG may demonstrate a right bundle branch
block or rSr’ pattern in secundum atrial septal
defect [1, 3].
Pathology/Description
This defect may be associated with Down
Syndrome, Holt Oram syndrome, DiGeorge syndrome, and Ellis Van Creveld syndrome [2].
There is an approximate 10% inheritance risk
from a parent with an ASD to their child [2].
Imaging: CMR, Cardiac CT, and/or TEE are
useful to evaluate ASD size, shape, rim tissue,
and pulmonary venous connections in adults with
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