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33 Aortic Disease inPregnancy
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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 0.3mm 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 <40mm have a low risk of dissection and should therefore be able to proceed safely with pregnancy. Women with an aortic diameter of >45mm 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 45mm, 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 >27mm/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 44mm in diameter and consider an aortic root diameter>45mm 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 >42mm if measured by TEE and if their aortic root is >44–46mm 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45mm [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 >2cm/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 <2cm/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 oftheAorta
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 20mmHg or <20mmHg with anatomic imaging evidence of signicant 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, etal. 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.
6. Novak J, Danielson LA, Kerchner LJ, Sherwood OD, Ramirez RJ, Moalli PA, Conrad KP. Relaxin is essential for renal vaso­dilation during pregnancy in conscious rats. J Clin Invest. 2001;107(11):1469–75.
7. Lansman SL, Goldberg JB, Kai M, et al. Aortic symposium 2016: aortic surgery in pregnancy. J Thorac Cardiovasc Surg. 2017;153(2):S44–8.
8. Crawford JD, Hsieh CM, Schenning RC, etal. 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 hypertensive pregnancies. Obstet Gynecol. 1991;78(6):1073–7.
10. Manalo-Estrella P, Barker AE. Histopathologic ndings in human aortic media associated with pregnancy. Arch Pathol. 1967;83(4):336–41.
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, etal. 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 Categories. [online] Accessed 6 June 2018 via
nih.gov/pregnancycategories.htm.
20. Brickner ME. Cardiovascular management in pregnancy. Circulation. 2014;130(3):273–82.
21. Bernard V, Donadille B, Zenaty D, Courtillot C, etal. 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
RaymondA.Dieter Jr. andMarshallGoldin
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 denitive 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, Wineld, 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 signicance, F-echo diagnosis of a PDA is best fol-
lowed by N-echo, which often conrms the ndings. Diagnosis of an aortic coarctation is often difcult 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
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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 dene 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 myobro-
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 inammation 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 inammation 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 myobroblastic 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,
2022]. 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
1. Dieter RA Jr, Kuzycz GK, Kemp R, Fallah J, Budres DM, Dieter
RS.Right aortic arch with a retro-esophageal Kommerall diverticu­lum and vascular ring in an adult. Int Surg. 2016;101:000–0001.
https://doi.org/10.9738/Int’lSurg.D14.00152.1.
2. Laraja K, Sadhwani A, Tworetzky W, Marshall AC, Gauvreau K,
Freud L, etal. 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 proling 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, etal. 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.Denitive 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.
www.jpeds.com. 2017;184:130–136e4.
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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 inammation in ultrasound-detected aortic intima/media thickness and growth retardation. J Reprod Immunol. 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 myobroblastic lesion. Cardiovasc Pathol. 2006;15(5):294–6.
18. Nissen T, Silverman NH, Ursell PC. Aortic vasculopathy with aneurysm: a rare cause of fetal hydrops. Cardiol Young. 2000;10(2):153–5.
19. Tsunematsu R, Shinozaki T, Fukushima K, Yumoto Y, Hidaka N, etal. Congenital aortic aneurysm with porencephaly: a case report. Fetal Diagn Ther. 2011;29(3):248–52.
20. Verma S, Chidambaratami S, Vijaylakshmi R, Srinivasan L, Suresh I.Apparent normal arrangement pattern of three-vessel view in a fetus with transposition of great arteries and 2-malposed aorta. Ann Pediatr Cardiol. 2017;10(2):215–7.
21. Miranda JO, Callaghan N, Miller O, Simpson J, Sharland G.Right aortic arch diagnosed antenatally: associations and outcome in 98 fetuses. Heart. 2014;100(1):54–9.
22. Ferschl MB, Moon-Grady AJ, Rollins MD, Gilliss B, Schulman SR, etal. Percutaneous fetal cardiac intervention for severe aortic ste­nosis and evolving hypoplastic left-heart syndrome. J Cardiothorac Vasc Anesth. 2016;30(4):1118–28.
23. Gardiner HM, Kovacevic A, Tulzer G, Sarkola T, Herberg U, etal. Natural history of 107 cases of fetal aortic stenosis from a European multi center retrospective study. Ultrasound Obstet Gynecol. 2016;48(3):373–81.
24. D’Antonio F, Khalil A, Zidere V, Carvalho JS. Fetuses with right aortic arch: a multi center cohort study and meta-analysis. Ultrasound Obstet Gynecol. 2016;47(4):423–32.
Aortic Trauma inChildren
https://t.me/med1917
RaymondA.Dieter Jr., GeorgeB.Kuzycz, RaymondA.Dieter III, andRobertS.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 [13]. 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 21years.
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 (Table35.1).
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 opacied 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 1week 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, Wineld, 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 underlled 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 8days 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 25years have been treated in our county with thoracic aortic disruption below
the origin of the left subclavian artery during the past 45years. 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 inChildren
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Table 35.2 Some pediatric aortic trauma results
Patient X-ray Treatment OR Result Hospital location 1 Left chest opacied 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 14units of blood. Repair urethral separation from the bladder
Pronounced dead in ER
Good—home in 1week 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
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diac activity, and an opacied 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 21years 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 3months 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 25BC 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 1000years 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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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 21years 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 rie 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 denes childhood to include individuals up to 18 or 21years 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 (18months) to 14, 16, 17, 18, and 19years of age. In our area, many pedia­tricians treat patients up to the age of 21, and thus we have dened our cases as 0–21years of age. Using this denition, 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 Classication of Diseases9th edition) and found 468 patients under 20years 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 signicant 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 14years 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 [1517].
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