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33 Aortic Disease inPregnancy
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437
Marfan Syndrome
Marfan syndrome is one of the most thoroughly studied congenital aortopathies in pregnancy. Still, there are discrepancies between major society guidelines for the management
of Marfan syndrome in pregnancy with respect to preventing
aortic dissection, and recommendations are based on observational studies and expert opinion. Women with Marfan
syndrome may experience exaggerated growth of the aorta
during pregnancy, up to 0.3mm per month [1]. Experts and
professional societies agree that women with Marfan syndrome should discuss their plans for pregnancy with their
cardiologist and obstetrician-gynecologist prior to conception. This team of physicians should carefully consider medical or surgical therapy for any underlying aortopathy prior
to conception and can develop a plan for monitoring during
pregnancy. For some patients at particularly high risk of
complication, pregnancy should be avoided altogether [13].
The European Society of Cardiology guidelines indicate
that women with Marfan syndrome with an aortic diameter
of <40mm have a low risk of dissection and should therefore
be able to proceed safely with pregnancy. Women with an
aortic diameter of >45mm should be encouraged to avoid
pregnancy, although there is limited data on the safety of
pregnancy in these women. For women with an aortic diameter between 40 and 45mm, individual decisions regarding
the management of pregnancy should be made based on the
patient’s body surface area, family history, and the rate of
growth of the aorta [16]. ESC guidelines recommend considering prepregnancy surgery for women with an aortic diameter>45 mm or an aortic diameter index >27mm/m
2
who
would like to consider pregnancy. ESC guidelines recommend the use of beta-blockers in pregnant women with
Marfan syndrome to prevent dissection, although data to
support their use is limited [16]. Alternatively, the ACC/
AHA guidelines recommend prepregnancy surgical correction of an aortic root between 40 and 44mm in diameter and
consider an aortic root diameter>45mm to be a contraindication to pregnancy [20].
Loeys-Dietz Syndrome
Management of Loeys-Dietz syndrome, an autosomal dominant connective tissue disorder involving aortic aneurysms
and tortuosity and craniofacial abnormalities, in pregnancy
is similar to the management of other congenital aortopathies in pregnancy. However, women with Loeys-Dietz syndrome are thought to have a higher rate of aortic dissection
than those with Marfan syndrome [13]. Patients should
undergo preconception counseling and should consider surgical correction prior to pregnancy. Patients with LoeysDietz syndrome who are considering pregnancy should
consider elective surgery when their aortic root is >42mm if
measured by TEE and if their aortic root is >44–46mm if
measured by CT/MRI [
7]. The European Society of
Cardiology guidelines recommend surgical repair prior to
pregnancy for Loeys-Dietz syndrome patients with an aortic
root diameter≥45mm [16]. Patients with Loeys-Dietz syndrome should follow the general ACC/AHA recommendations for monthly or bimonthly echocardiographic screening
of the aortic root during pregnancy and strict blood pressure
control.
Ehlers-Danlos Type IV
Ehlers-Danlos syndrome is a group of genetic connective tissue disorders, with at least six major disease types, each with
their own phenotypic features and patterns of inheritance.
Ehlers-Danlos type IV (also referred to as vascular type) is
associated with uterine rupture, and pregnancy is therefore
contraindicated in Ehlers-Danlos type IV patients. For
patients who are diagnosed with the condition after conception, or for patients who become pregnant despite contraindications, management can be particularly challenging. Aortic
dissection may occur in Ehlers-Danlos type IV without dilatation. European Society of Cardiology guidelines recommend beta blockade for pregnant patients with Ehlers-Danlos
type IV to reduce the risk of aortic dissection. Pregnant
women with Ehlers-Danlos type IV are also recommended to
undergo early cesarean delivery [16].
Turner Syndrome
Turner syndrome is a chromosomal abnormality associated
with short stature, webbed neck, bicuspid aortic valve, and
coarctation of the aorta. Turner syndrome patients have
lower fertility rates than the general population [21].
However, for both spontaneous and facilitated pregnancy in
patients with Turner syndrome, particular attention should
be dedicated to preventing aortic dissection. As seen with
other congenital aortopathies, there are slight differences in
recommendations for the management of patients with
Turner syndrome due to limited available evidence. The
American Society of Reproductive Medicine recommends
that pregnancy is contraindicated if there are any cardiac
defects seen on MRI prior to pregnancy, and if the preconception aortic size index is >2cm/m2 [22]. For women with
Turner syndrome who become pregnant, the American
Society of Reproductive Medicine recommends periodic
echocardiogram or MRI during pregnancy and treatment of
hypertension to prevent aortic dissection. The American
Society of Reproductive Medicine also recommends vaginal
delivery if the aortic size index (ASI, aortic diameter in

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C. A. Ball and S. Sirna
centimeters divided by body surface area in meters2) at the
time of delivery is <2cm/m2 but recommends elective cesarean section with epidural anesthesia for patients with an ASI
>2 cm/m2 before labor [22, 23]. The Turner Syndrome
Consensus Group describes a relative contraindication to
pregnancy in women with Turner syndrome who have had
prior surgical repair of cardiovascular disease, bicuspid aortic valve, aortic dilatation, or hypertension. The Turner
Syndrome Consensus Group also recommends prepregnancy management with ECG, echocardiography, and cardiac MRI and that vaginal delivery is reasonable for some
patients [22, 24].
Coarctation oftheAorta
Although many patients with coarctation of the aorta undergo
surgical repair prior to pregnancy, there are reported cases of
pregnancy in women with unrepaired coarctation. Known
potential complications of pregnancy in patients with coarctation include an increased risk of hypertensive disorders of
pregnancy. Patients with coarctation of the aorta and a peakto- peak coarctation gradient ≥20mmHg or <20mmHg with
anatomic imaging evidence of signicant coarctation and
collateral ow should consider surgical repair prior to pregnancy. Both prior to and during pregnancy patients with
coarctation of the aorta should have their blood pressure
checked in all four extremities [20].
Conclusion
Aortic disease during pregnancy is exceptionally rare but
associated with high morbidity and mortality for both mother
and fetus. Diagnosis, management, and treatment require a
team approach, including cardiologists, obstetriciangynecologists, and vascular and cardiovascular surgeons.
Although there are guidelines and recommendations available to guide management, each case needs to be addressed
on an individual basis. Particular attention should be paid
both before conception and during pregnancy to women with
genetic syndromes known to be associated with aortopathy.
References
1. Wanga S, Silversides C, Dore A, etal. Pregnancy and thoracic aortic disease: managing the risks. Can J Cardiol. 2016;32(1):78–85.
2. Kamel H, Roman MJ, Pitcher A, Devereux RB.Pregnancy and the
risk of aortic dissection or rupture. Circulation. 2016;134:527–33.
3. Bons LR, Roos-Hesselink JW.Aortic disease and pregnancy. Curr
Opin Cardiol. 2016;31(6):611–7.
4. Sanghavi M, Rutherford JD.Cardiovascular physiology of pregnancy. Circulation. 2016;130(12):1003–8.
5. Greenwood JP, Scott EM, Stoker JB, Walker JJ, Mary
DASG.Sympathetic neural mechanisms in Normal and hypertensive pregnancy in humans. Circulation. 2001;140(19):2200–4.
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7. Lansman SL, Goldberg JB, Kai M, et al. Aortic symposium
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8. Crawford JD, Hsieh CM, Schenning RC, etal. Genetics, pregnancy,
and aortic degradation. Ann Vasc Surg. 2016;30(158):e5–9.
9. Easterling TR, Benedetti TJ, Schmucker BC, Carlson K, Millard
SP. Maternal hemodynamics and aortic diameter in Normal and
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10. Manalo-Estrella P, Barker AE. Histopathologic ndings in
human aortic media associated with pregnancy. Arch Pathol.
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11. Sawlani N, Shroff A, Vidovich MI.Aortic dissection and mortality
associated with pregnancy in the United States. J Am Coll Cardiol.
2015;65:1600–1.
12. Dieter RA.Idiopathic spontaneous perforation of the aorta during
pregnancy. J Vasc Surg. 1984;18(5):311–5.
13. Roman MJ, Pugh NL, Hendershot TP, et al. Aortic complications associated with pregnancy in Marfan syndrome: the NHLBI
National Registry of genetically triggered thoracic aortic aneurysms and cardiovascular conditions (GenTAC). J Am Heart Assoc.
2016;5(8):pii: e004052.
14. Hauenstein E, Frank H, Bauer JS, et al. Takayasu’s arteritis in
pregnancy: review of literature and discussion. J Perinat Med.
2010;38(1):55–62.
15. Hiratzka LF, Bakris GL, Beckman JA, etal. Guidelines for the diagnosis and management of patients with thoracic aortic disease. J
Am Coll Cardiol. 2010;55(14):e:127–9.
16. Regitz-Zagrosek V, Ludqvist CB, Borghi C. And the task force
on the management of cardiovascular diseases during pregnancy
of the European Society of Cardiology. ESC guidelines on the
management of cardiovascular diseases during pregnancy: the task
force on the management of cardiovascular diseases during pregnancy of the European Society of Cardiology (ESC). Eur Heart J.
2011;32:3147–97.
17. Podymow T, August P.Update on the use of antihypertensive drugs
in pregnancy. Hypertension. 2008;51(4):960–9.
18. Podymow T, August P.Antihypertensive drugs in pregnancy. Semin
Nephrol. 2011;31(1):70–85.
19. US Department of Health and Human Services. FDA Pregnancy
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20. Brickner ME. Cardiovascular management in pregnancy.
Circulation. 2014;130(3):273–82.
21. Bernard V, Donadille B, Zenaty D, Courtillot C, etal. Spontaneous
fertility and pregnancy outcomes amongst 480 women with turner
syndrome. Hum Reprod. 2016;31(4):782–8.
22. Folsom LJ, Fuqua JS.Reproductive issues in women with turner
syndrome. Endocrinol Metab Clin N Am. 2015;44(4):723–37.
23. Practice Committee of American Society for Reproductive
Medicine. Increased maternal cardiovascular mortality associated with pregnancy in women with turner syndrome. Fertil Steril.
2012;97:282–4.
24. Bondy CA.Heart disease in turner syndrome. Minerva Endocrinol.
2007;32:245–61.
https://chemm.nlm.

Fetal Aortic Disorders
https://t.me/med1917
RaymondA.Dieter Jr. andMarshallGoldin
34
Introduction
Aortic disease is fairly common in the adult. Aortic processes include aneurysm, aortic occlusive disease, and dissections—especially of the thoracic aorta. Congenital
aortic diseases, aortic arch atresia, vascular ring, rightsided aorta, coarctation, A-P (aortopulmonary) window,
and PDA (patent ductus arteriosus) may all occur [1]. The
prenatal ultrasound programs facilitate the diagnosis of
structural abnormalities and the enhancement of prenatal
and postnatal treatment. Consequently, the obstetrician,
the high-risk neonatologist, and the pediatric cardiologist
are all involved in the diagnosis and treatment of the newborn with pathologic cardiovascular anomalies which
require treatment [2]. Noninvasive diagnostic advances
have resulted in optimal planning regarding denitive
treatment in this milieu.
Diagnosis
Prenatal ultrasound diagnosis of cardiovascular pathology
facilitates optimal planning of therapeutic interventions.
Directed history and treatment of the mother and family
add to a potential suspicion regarding anomalies. Neonatal
(N-echo) and fetal (F-echo) echo studies and MRI have further added to the physician’s armamentarium for perinatal
diagnosis of congenital lesions in many sites.
R. A. Dieter Jr. (*)
Cardiovascular and Thoracic Surgery, Northwestern University at
Cadence Health Emeritus, Wineld, IL, USA
M. Goldin
Cardiothoracic Surgery, Glenview, IL, USA
Diagnostic evaluation modalities for fetal
cardiovascular abnormalities
I. Routine obstetric periodic evaluation and family
history
II. Screening ultrasound
III. Consultation
A. Neonatologist
B. Perinatologist
IV. Targeted ultrasound
V. Fetal referral center
VI. Echocardiogram
A. Fetal
B. Maternal
C. Postnatal
VII. Amniotic uid evaluation
VIII. Magnetic resonance imaging
IX. Genetic testing
These diagnostic modalities have resulted in the develop-
ment of tertiary neonatal centers with subspecialized physicians, nurses, technical staff, and equipment, which enhance
treatment of at-risk pregnancies and the developing fetus [3].
Intrauterine management of these lesions may decrease gestational mortality, and postnatal therapy can further add to
survival.
Of signicance, F-echo diagnosis of a PDA is best fol-
lowed by N-echo, which often conrms the ndings.
Diagnosis of an aortic coarctation is often difcult in a fetus
with an early PDA diagnosis. Right aortic arch (RAA) is
often associated with TOF (tetralogy of Fallot), and genetic
testing in these cases has led to the diagnosis of additional
cardiac and noncardiac genetic anomalies [4]. Such genetic
testing in the fetus with RAA has led to the diagnosis of the
© Springer Nature Switzerland AG 2019
R. S. Dieter et al. (eds.), Diseases of the Aorta, https://doi.org/10.1007/978-3-030-11322-3_34
439

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autosomal dominant Loeys-Dietz syndrome in a fetus with
intrauterine diagnosis of aneurysmal dilation of the aortic
root [5, 6].
Physical examination and family history may be suggestive of potential cardiovascular pathology. Fetal heart tones
(FHT), if irregular or diminished, may be indicative of a congenital anomaly. In the 1960s, in Alaska, when the FHTs
were discovered to be 50 or so, our only recourse was close
monitoring of the mother and the FHTs. Subsequent to delivery, the infant was sent to Seattle for further evaluation and,
later, a pacemaker, with no other diagnostic abnormality
detected. No familial congenital C-V (cardiovascular) history was known. Early in our residency, we were not allowed
to discuss genetics and the possibility that future children
could be born with congenital heart defects to adult individuals who had previously undergone repair of congenital cardiac defects. We now know that congenital cardiovascular
disease may have a genetic origin, requiring comprehensive
fetal monitoring utilizing F-echo and MRI [7, 8].
Color Doppler, EKG, and fetal growth records may raise
suspicion of fetal abnormalities. Postnatal studies and neurodevelopmental assessment may suggest the need for additional investigation. Biometry consistent with age, normal
amniotic uid, and no known extra cardiac lesion are considered when evaluating for fetal C-V lesions. Echocardiographic
evaluation will aid in decision-making for diagnosis and
treatment of cardiovascular lesions. Genetic amniocentesis
studies may be considered—especially when multiple
lesions are present and fetal karyotype studies may dene a
deletion (ex. 22 q) defect.
R. A. Dieter Jr. and M. Goldin
Examples of fetal aortic abnormalities
I. Anomalous origin
A. Origin right pulmonary artery from the aorta
B. Aortopulmonary window
II. Stenotic/obstructive lesions
A. Coarctation of the aorta—association with left
ventricular noncompaction
B. Neonatal aortic thrombosis—prenatal transfer
of anticardiolipin antibodies
C. Aortic arch interruption
1. Associated left ventricular aneurysm
2. Associated with chromosomal abnormalities
III. Aortic aneurysm
A. Descending thoracic aortic aneurysm in
hydropic fetus
B. Ascending due to benign nodular myobro-
blastic lesion
C. Abdominal—associated with porencephaly
IV. Valve area
A. Transposition of great vessels
B. Giant sinus of Valsalva aneurysm
C. Aortic valve stenosis—hypoplastic left heart
syndrome
D. Aortic root dilation
V. Aortic wall inammation
VI. Right aortic arch
A. Vascular ring
B. Chromosomal abnormalities
C. Aberrant right subclavian artery
Findings
Most newborn congenital cardiac and vascular lesions are evident at or shortly after birth. These lesions were not detectable
in utero until recently as both diagnostic and therapeutic measures were either not available or of limited diagnostic scope.
With the advent of more regular obstetric visits, coupled with
more advanced diagnostic approaches, an increasing number
of fetal abnormalities are being detected. Currently, many congenital lesions are diagnosed and treated during pregnancy or
shortly after delivery [9]. Remarkably, many of these fetuses
may then continue normal gestation progress to the newborn
stage with the potential for a full lifespan to follow.
Review of the literature demonstrates multiple fetal cardiovascular defects detected by F-echo. These defects, such as the
stulous anomalous origin of the right pulmonary artery from
the aorta, may be relatively simple or more complex in nature
as seen with the presence of an aortopulmonary window [10,
11]. Stenotic or obstructive lesions, including aortic coarcta-
tion in association with left ventricular noncompaction, may
have many different characteristics [12].
Presented above are many of the congenital lesions, including aortic arch interruption associated with a left ventricular
aneurysm and chromosomal abnormalities [13]. These aneurysms may or may not be associated with pentalogy of
Cantrell. In a review of 8 fetal and 20 neonate IAA (interrupted aortic arch) cases from 1994 to 2010, 10 type A and 18
type B IAA patients were accurately diagnosed—with microdeletion of the 22q 11.2 chromosome in 6 patients [14].
Hematologic abnormalities include neonatal aortic thrombosis after prenatal transfer of anticardiolipin antibodies and
aortic wall inammation have been described [15, 16]. The
latter nding has been considered a precursor to possible
early ndings predictive of future atherosclerosis in the
adult. Aneurysm formation, a frequent nding in the adult,
has been diagnosed in a number of fetal studies. An ascending aortic aneurysm was thought to arise histologically from
a benign nodular myobroblastic lesion of hamartomatous
origin [17]. A hydropic 28-week gestation fetus was found to
have a saccular descending thoracic aortic aneurysm. The
marked brointimal hyperplasia with tunic media attenuation and thrombus were speculated to have caused after load

34 Fetal Aortic Disorders
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441
cardiac failure [18]. Pediatric abdominal aortic aneurysms
are rare but may result from a number of causes [19]. Listed
below are a number of these etiologies, including prenatal
porencephaly (a condition characterized by an intracerebral
cyst association) [19].
Pediatric thoracic and abdominal aortic aneurysm
associations
I. Intrauterine infection
II. Connective tissue diseases/gene mutations
A. Ehlers-Danlos syndrome
B. Marfan syndrome
III. Iatrogenic trauma—umbilical artery catheterization
IV. Porencephaly—intrautero vascular obstruction
Lesions located proximally near the aortic valve may
involve the root, the valve, or the sinus of Valsalva. Such
lesions include transposition of the great vessels, aneurysm
of the sinus of Valsalva, and aortic valve stenosis with hypoplastic left heart syndrome, along with aortic root dilation [6,
20–22]. Vascular ring and right aortic arch (RAA) often
occur together (reported in one series of 97 fetuses) [20].
Multiple vascular anomalies involving the RAA include vascular ring and double arch (incidence of 15.3%) and chromosomal anomalies (with 22q 11.2 microdeletion in half the
patients). As diagnostic ability has increased, the earlier recognition of fetal disease and aortic lesions has also increased.
Therapeutic Considerations
Since 1963, when intrauterine transfusion for hemolytic anemia was initiated, an ever-increasing number of fetal therapeutics has evolved—especially in the cardiovascular and
thoracic elds. Diagnosis of intrauterine congenital lesions
has led to a more intense prepartum follow-up and increased
opportunities for the treatment of these fetal lesions.
Depending on the diagnosis, treatment options include prenatal and postnatal patients. Planning and preparation must
take into consideration immediate and long-term risks
regarding viability and risk of intervention to the fetus,
infant, and mother. But subspecialty care and tertiary fetal
referral care facilities must be considered for the mother and
fetus during the remaining period of pregnancy.
Consideration for intrauterine surgery must outweigh the
risk of delaying intervention until the postpartum period.
Ideally, intervention will be performed prior to irreversible
physiological compromise. Risk and cost must be factored
into the decision-making process. The various vascular and
cardiac procedures are both complicated and risky when per-
formed in these situations with neurodevelopmental delay a
consideration in fetal aortic valvuloplasty [1]. Coarctation
screening pre- and postnatally for optimum diagnosis and
treatment timing will require close evaluation [3]. Many of
the lesions involving the proximal aorta require complicated
interventional manipulation and are not possible during fetal
development. Thus, if the lesion is low risk during delivery
and early development, intervention will be delayed until the
optimum postpartum period [23].
Vascular ring patients may have an 83% freedom from
intervention surgery at 2-year follow-up [24]. Early diagnosis of coarctation allows the family to be aware of the options,
including prostaglandin, therapy, and to delay intervention to
the ideal time [3]. Ferschl et al. have outlined therapeutic
indications for congenital cardiac lesions using percutaneous
fetal intervention [22]. But when the situation permits, or the
lesion accessibility prevents, intervention is delayed until the
postpartum period. The eld of fetal diagnosis, fetal therapy,
and awareness of genetic abnormalities have recently provided a basis for the evolution of treatment and improved
long-term success in this milieu. Molecular, genetic, and
electron therapy will progress with further enlightenment
and avoidance or elimination of many of these fetal, and possibly fatal, cardiovascular diseases.
References
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RS.Right aortic arch with a retro-esophageal Kommerall diverticulum and vascular ring in an adult. Int Surg. 2016;101:000–0001.
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2. Laraja K, Sadhwani A, Tworetzky W, Marshall AC, Gauvreau K,
Freud L, etal. Neurodevelopmental outcome in children after fetal
cardiac intervention for aortic stenosis with evolving hypo plastic left
heart syndrome. J Pediatr.
3. Evers PD, Ranade D, Lewin M, Arya B. Diagnostic approach in
fetal coarctation of the aorta: a cost-utility analysis. J Am Soc
Echocardiogr. 2017;30(6):589–94.
4. Dong D, Zhang Y, Reece EA, Wang L, Harman CR. Yanga P.
microRNA expression proling and functional annotation analysis
of their targets modulated by oxidative stress during embryonic heart
development in diabetic mice. Reprod Toxicol. 2016;65:365–74.
5. Peng R, Xie HN, Zheng J, Zhou Y, Lin MF. Fetal right aortic
arch: associated anomalies, genetic anomalies with chromosomal microarray analysis, and postnatal outcome. Prenat Diagn.
2017;37(4):329–35.
6. Viassolo V, Lituania M, Marasini M, Dietz H, Benelli F, etal. Fetal
aortic root dilation: a prenatal feature of the Loeys-Dietz syndrome.
Prenat Diagn. 2006;26(11):1081–3.
7. Dieter RA Jr, Pifarre R, Niedballa RG.Denitive surgical treatment
of the aberrant retroespophageal right subclavian artery in the adult.
Thorac Cardiovasc Surg. 1971;61:154–9.
8. Dieter RA Jr. Idiopathic spontaneous perforation of the aorta during
pregnancy. Vasc Surg. 1984;(5):311.
9. Chaney MA, Baum VC.Percutaneous fetal and cardiac interven-
tion for severe aortic stenosis and evolving left heart hypo plastic syndrome. Case conference. J Cardiothorac Vasc Anesth.
2016;30(4):1118–28.
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R. A. Dieter Jr. and M. Goldin
10. Zeng S, Zhou Q, Zhou J, Peng Q.Fetal isolated anomalous origin
of right pulmonary artery from aorta. AJP Rep. 2015;5(1):e80–1.
11. Fotaki A, Novaes J, Jicinska H, Carvalho JS.Fetal aortopulmonary
window: case series and review of the literature. Ultrasound Obstet
Gynecol. 2017;49(4):533–9.
12. Jacobs K, Giacobbe L, Aguilera M, Ramin K, Sivanandam S.A
case of fetal diagnosis of noncompaction cardiomyopathy and
coarctation of the aorta. AJP Rep. 2014;4(1):45–8.
13. Jowett V, Miller O.Prenatal diagnosis of left ventricular aneurysm
in association with interruption of the aortic arch. Cardiol Young.
2011;21(1):13–5.
14. Axt-Fliedner R, Kawecki A, Enzensberger C, Wienhard J,
Degenhardt J, et al. Fetal and neonatal diagnosis of interrupted aortic arch: associations and outcomes. Fetal Diagn Ther.
2011;30(4):299–305.
15. Tuohy J, Harrison A.Prenatal transfer of anticardiolipin antibodies
associated with fatal neonatal aortic thrombosis. Aust N Z J Obstet
Gynaecol. 2005;45(2):175–6.
16. Lo Vasco VR, Salmaso R, Zanardo V, Businaro R, Visentin S,
et al. Fetal aorta wall inammation in ultrasound-detected aortic
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2011;91(1–2):103–7.
17. Ramaswamy P, Haberman S, Kleinman C, Lytrivi ID, Thaker
HM.Ascending aortic aneurysm in a fetus due to a benign nodular
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2000;10(2):153–5.
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Ultrasound Obstet Gynecol. 2016;47(4):423–32.

Aortic Trauma inChildren
https://t.me/med1917
RaymondA.Dieter Jr., GeorgeB.Kuzycz,
RaymondA.Dieter III, andRobertS.Dieter
35
Introduction
Aortic trauma in patients under the age of 21 is an uncommon occurrence but does occur. A review of the literature
demonstrates the rarity of such concerns [1–3]. Most reports
contain less than 10 pediatric aortic trauma patients unless
the report is a review of the literature [4]. We report herein
our experience with patients who had a traumatic aortic
injury. Diagnostic and therapeutic indications require one to
be alert to the potential for and the severity of an aortic injury
requiring treatment and the associated risks. Our patients
represent varied etiologies, types of injury, and therapeutic
options in children under the age of 21years.
Patients
These patients presented to the emergency room with a varied complex of signs and symptoms and thus are best illustrated by brief descriptions of each of their situations
(Table35.1).
Patient #1
and one was due to peritoneal sepsis). The third child, a
4-year-old, was endotracheally intubated and had a gunshot
wound to the left anterior chest and a posterior left chest
paravertebral exit wound. He was unresponsive and the left
chest was opacied on x-ray. An ER left thoracotomy
revealed an entrance and exit wound of both the heart and the
aorta. We rapidly closed the cardiac lesions and clamped the
aorta. Despite intense resuscitative efforts, there was no
response and the resuscitation efforts were withdrawn.
Patient #2
A 3-year-old child racing to her father at his desk tripped and
fell upon a sharp paper spindle on the oor, which penetrated
the anterior chest, the heart, and the aorta and exited the
back. The child’s mother pulled the spindle out of the chest
and rushed her to the hospital. Following physical and radiologic diagnosis, and 1week of hospitalization, she was discharged home without surgery to close uneventful
follow-up.
Patient #3
On responding to a code blue from the ER (emergency
room), I found three patients in shock (one was due to a
Cadillac n entering the abdomen from a bicycle accident,
R. A. Dieter Jr. (*) ∙ G. B. Kuzycz
Cardiovascular and Thoracic Surgery, Northwestern University at
Cadence Health Emeritus, Wineld, IL, USA
R. A. Dieter III
University of Tennessee Medical Center, Cardiovascular and
Thoracic Surgery, Knoxville, TN, USA
R. S. Dieter
Interventional Cardiology, Vascular and Endovascular Medicine,
Loyola University Medical Center, Maywood, IL, USA
© Springer Nature Switzerland AG 2019
R. S. Dieter et al. (eds.), Diseases of the Aorta, https://doi.org/10.1007/978-3-030-11322-3_35
A 16-year-old female was playing league softball and
received a blunt injury to the abdomen and left ank.
Following emergency room resuscitation, an aortic angiography was obtained. At surgery, the aortic defect was repaired
and a salvage left aortorenal bypass graft failed to preserve
the kidney. Following left nephrectomy, the teenager did
well.
Patient #4
An 18-year-old male received multiple injuries following a
motorcycle accident including multiple long bone (all but
one) fractures, descending thoracic aorta disruption, the left
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Table 35.1 Some examples of pediatric aortic trauma
Patient Age Gender Type of injury Injury location Cause Symptoms PE
1 4 Male Anterior and posterior
chest penetration—thru
and thru
2 3 Female Thru and thru
penetration anterior and
posterior chest
3 16 Female Blunt Left renal artery torn
4 18 Male Blunt Descending thoracic
5 19 Male Blunt sudden Descending thoracic
6 20 Male Blunt Descending thoracic
Anterior and posterior
perforation of the heart
and aorta
Descending thoracic
aorta and heart
off aorta
aorta transected.
Left renal artery torn
off aorta bladder torn
off urethra
aorta trans-section
cervical fracture
aorta below left
subclavian
Gunshot Shock cardiac arrest Entrance and exit
Fall on metal
paper spindle
Blunt trauma due
to softball game
Motorcycle
accident
Dive in
pool—not full
Auto accident Pain shock Paralyzed multiple
Crying, pain Perforation anterior
Abdominal ank—pain Tender bruise
Shock, pain, all long
bones fractured,
urethra transected
Shock Paralyzed
wounds left chest—
no response
posterior chest skin
Multiple injuries,
shock
injuries
renal artery torn off the aorta, liver and spleen trauma, and
the bladder torn off the urethra. The thoracic aortic disruption was repaired through a left thoracotomy with proximal
and distal clamping. After splenectomy, a saphenous vein
graft to the left renal artery from the aorta, repair of the
abdominal aorta, and bladder to urethra repair/anastomosis,
liver repair, the patient recovered with survival of the kidney,
a functional aortorenal graft, and normal blood pressure. He
did well after hospital discharge.
Patient #5
A teenager dove into an underlled pool. He received a cervical fracture and aortic disruption below the left subclavian
artery. Following the aortic repair with a good aortic pulsation and cervical stabilization, he remained paralyzed.
Patient #6
This 20-year-old had multiple injuries with blood from the
trachea, mediastinal widening, and paraplegia following an
automobile accident. Despite surgical repair and blood transfusion, he failed to respond and died 8days later due to the
head and spinal cord injury.
Comment
In our suburban area, we have a large human population and
high vehicular speeds. Thus, a number of additional late teen
or early adult patients under the age of 25years have been
treated in our county with thoracic aortic disruption below
the origin of the left subclavian artery during the past
45years. Those individuals are not included in this discussion, but we anticipate the continued occurrence of the accidents which cause the aortic injuries.
Treatment
Correction of a traumatic aortic injury, whether in an adult or
a child, requires a correct diagnosis in a timely fashion along
with the availability of an angiographic and diagnostic lab,
of an operating room (OR) or an endovascular room (EnR),
and of the medical and support staff. Currently, communications from the scene of an accident, the emergency transport
crews, and the emergency room staff provide frequent comprehensive updates. With the relayed information, the diagnostic considerations may alert the emergency room (ER)
staff, and radiologic scanning department. Thus, the surgery
staff and the OR may be prepared for the rapid diagnosis,
treatment, and possible correction of an aortic lesion within
a few minutes of arrival at the hospital.
The ER gunshot wound did not provide an opportunity for
cardiovascular diagnostic studies and surgical planning. The
situation was grave and required immediate possible salvage
intervention—which eventually proved fruitless. Despite the
seriousness of the injuries, the remainder of the patients
herein presented had adequate time for hospital transport,
diagnostic study, and implementation of the recommended
therapy and surgery.
Table 35.2 presents the x-ray ndings in these young individuals. The condition of the patient at presentation determined the diagnostic and therapeutic decisions to best treat
the patient and the injuries at the time of examination. In the
emergency room, with no blood pressure, no pulse nor car-

35 Aortic Trauma inChildren
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Table 35.2 Some pediatric aortic trauma results
Patient X-ray Treatment OR Result Hospital location
1 Left chest opacied ER—open left chest. Plug
2 Left pleural uid Observed serial
3 Left renal artery torn off aorta
CT
4 Thoracic aortic transsection.
Multiple long bone fractures. Left
renal artery torn off aorta
CT/several angiography
5 CT Stabilize/repair Open left thoracotomy Paralyzed
6 CT/MRI
Angio
holes with ngers
x-ray
Aorto-renal bypass repair
aorta
Repair with graft aorta.
Aorta renal vein bypass.
Bladder and bone repair
Repair Open left thoracotomy Died due to head
Left thoracotomy in ER
suture heart and aorta
None, mother pulled out
spindle
Surgery laparotomy left
nephrectomy
OR
Thoracotomy Laparotomy
Splenectomy 14units of
blood.
Repair urethral separation
from the bladder
Pronounced dead
in ER
Good—home in
1week
Good Community hospital
Good 3-month
rehab all function
long-term
survival
injuries
Emergency room
pediatric teaching
hospital
Small community
hospital [1]
Community hospital
1]
[
Large community
hospital
Community hospital
445
diac activity, and an opacied thorax, the determination for
an immediate thoracotomy was readily decided.
Unfortunately, four holes (two in the heart and two in the
aorta) were the nding. After suture of the cardiac perforations and control of the aorta, we were unable to successfully
resuscitate the child despite large volume blood and electrolyte uid administration. The 3-year-old child, with penetration of the heart and aorta both anteriorly and posteriorly by
the 2–3-mm metallic straight spindle, already had the spindle
removed prior to arrival at the hospital. Diagnostic x-rays
and contrast studies were consistent with penetration of both
the heart and the aorta. A bloody pleural effusion was present, but the patient was stable hemodynamically, on serial
chest x-rays and on laboratory studies. Thus, close observation was successfully elected by the parents as the treatment
of choice.
Blunt trauma was the cause of the aortic injury in patients
3–6 causing either thoracic or abdominal aorta rupture.
Diagnostic evaluation was followed by operative treatment
in each of the deceleration thoracic injury patients with two
long-term survivals—one remained paralyzed due to the spinal cord injury. The blunt abdominal low velocity injury
occurred in a young female while playing softball. She
developed marked abdominal and left ank pain, tenderness
to touch, and bruising. CT (computerized tomography) scan
and diagnostic aortic angiography revealed the left renal
artery to be torn off the aorta, a renal contusion, and formation of a large hematoma. Surgical exploration with closure
of the aortic defect and attempted renal salvage with an aortorenal saphenous vein graft was not successful and she later
had a left nephrectomy. The child did well and was cautioned
to avoid contact activities which might injure the remaining
right renal artery or kidney. Our patients were between 3 and
21years of age, and two were female. Two patients had pen-
etrating injuries and four patients (three males) had blunt
trauma—two low velocity.
Automobiles, diving into a pool with little water, or a
motorcycle accident may all be causes of accidental thoracic
aortic trauma. The blunt trauma patient with an aortic contusion, laceration, or transaction usually will have other traumatic injuries involving the long bones, spine and spinal
cord, heart, and abdominal contents. Patient #4 had extensive
vascular (two aortic lesions), thoracic, and abdominal injuries including the left renal artery torn off the aorta and the
bladder torn off the urethra. In this patient, rapid ER diagnostic CT and x-ray studies were followed by emergency repair
of the aorta, long bones, diaphragm, splenectomy, renal
artery saphenous vein bypass, and urethral reanastomosis.
Following 3months of intensive care, he was discharged and
recovered completely. Two of the patients (#5 and 6) had
concomitant spinal cord injury with paralysis and no
improvement of their paralysis after aortic repair.
Discussion
Vascular trauma, according to Feliciano, was rst described
by Celsus in 25BC to 50 AD in his text De Medicina, in
which he described applying pressure or ligature of bleeding
vessels [5]. Vascular trauma reporting was then ignored for
over 1000years until control of bleeders and the use of a
hemostat was described by Ambroise Paré in “Bec de
Corbin” in the 1500s [5]. Wars then became instrumental in
developing lifesaving and bleeding control techniques. In the
1900s, WWI, WWII, and the Korean and the Vietnam Wars
all required surgical intervention in young traumatized soldiers for thoracic, abdominal, orthopedic, and vascular
concerns.

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R. A. Dieter Jr. et al.
Penetrating and blunt injuries created challenges that
required urgent and corrective therapeutics. Thus, control of
hemorrhage using compression, clamping, primary repair,
and vascular grafting was attempted and the results were
reported. A large number of these injuries occurred in the
young—many under 21years of age—male in the combat
situation. Rapid eld transport, control of bleeding, stabilization of the soldier, and then timely repair were required.
With the above experience, the returning war surgeons
fostered the same care in the community. They transferred
their knowledge and results from the penetrating and traumatic war scenes to the university and community hospitals.
No longer were rie shots, exploding bombs, and land mines
the only cause of a critical injury. It was now communityoriented trauma related to the deceleration automobile or
motorcycle accidents, work accident, and entertainment
accidents that required treatment. Blunt traumatic accidents
became more concerning to the physician and the family
including the bluntly injured individual who also had peripheral vascular and aortic concerns.
These concerns were not limited to the adult—especially
if one denes childhood to include individuals up to 18 or
21years of age. Reports now describe the aortic or vascular
traumatic injury in the age range of infant to 21 years. A
review of the literature provides a large source of pediatric
patients who have suffered from blunt or penetrating aortic
injury. Some reports present their ndings in the 3- to
11-year-old patients and the treatment program utilized to
repair their lesion. In a review of the literature, one notes that
the ages of those reported include the infant (18months) to
14, 16, 17, 18, and 19years of age. In our area, many pediatricians treat patients up to the age of 21, and thus we have
dened our cases as 0–21years of age. Using this denition,
many of the armed or military forces members would fall
into the children or pediatric age group.
Takach reported on three pediatric aortic disruption
patients aged 4–16 years from the Medical College of
Georgia injured from a motor vehicle accident [3]. Hormuth
reported 11 patients with blunt trauma treated at Clarian
Methodist Hospital in Indianapolis (three drivers, four passengers, three pedestrians, and one bull thrown) [6]. Of
note, most aortic injuries in this age population are due to
motor vehicles [7, 8]. Tiao also demonstrated the association of cardiac and other major vascular and nonvascular
injuries at autopsy in these patients [8]. Tashiro et al.
reviewed the Kids’ Inpatient Database (1997–2009) to
identify thoracic and abdominal aortic injury (International
Classication of Diseases—9th edition) and found 468
patients under 20years of age with motor vehicle (77%),
penetrating (10%), and rearms (8%) injuries with an overall survival of 65% [9]. Our patients fell into all three
groups-motor vehicle, penetrating, and rearm with equivalent survival.
Most traumatic aortic injuries occur in the chest—usually
just below the left subclavian artery origin. But abdominal
aortic injuries may also occur—especially at or below the
renal arteries (note our two patients). Sadaghianloo et al.
reviewed three injuries to the abdominal aorta in France and
reviewed the literature listing various authors’ experience,
the associated injuries, and the causes [10]. Choit et al.
reported on the chance of abdominal aortic injuries occurring
in association with fractures in the pediatric patient [11]. In
2009, Heck reported on a 16-month-old child with blunt
traumatic injury to the aorta [12].
Early in the treatment of aortic injuries, only a few diagnostic studies were available. These included the CBC
(complete blood count), chest roentgenograms, and exploratory surgery. With the development of CT (computerized
tomography) scans and the use of contrast vascular studies,
more reliable diagnoses were established, and simultaneously, more advanced and aggressive treatment for the aortic lesion as well as other associated injuries became
available. Open thoracotomy and clamp-clamp aortic repair
were followed by cardiopulmonary bypass support during
the repair—depending on the type, location, and number of
injuries [6, 7]. In 2005, the Texas Heart Institute reported on
the use of partial left heart bypass during aortic cross clamping in these patients [3].
With continued advances in technical and therapeutic
interventions, including MRI (magnetic resonance imaging),
the treatment of these patients has continued to evolve.
Concomitant signicant nonaortic injuries (especially cranial, spinal, and hepatic) are now more readily assessed, and
their treatment may take preference. Determinations as to the
advisability of surgery, or when to perform such, are discussed by the attending physicians (e.g., neurosurgeons,
orthopedic, cardiovascular, etc.). Both operative and nonoperative programs utilizing medication and close observation
have been followed [2, 13]. More recently, endovascular
therapy utilization for the adult with aortic lesions has
increased throughout the world. Thus, Saad reported on the
endovascular repair of a traumatic aortic transsection in a
12-year-old [14]. Goldstein then reported on the use of six
covered stents in four patients aged 11 to 14years with one
death due to intracranial trauma [15]. Two teenagers after
correction of their concomitant traumatic lesions had stents
placed for their thoracic aortic transections with ultimate
recovery, while another female teenager had an iliac limb
prosthesis placed as an interim bridge for future correction of
an aortic lesion [15–17].
In our operated patients, treated transthoracically without a stent, the long-term results were primarily related to
the underlying trauma and not the aortic therapeutic
approach [18]. Kalkwarf et al. reviewed 1292 severely
injured children to determine if predictive laboratory determinations exist to ascertain mortality odds and resuscitative
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