Journal of Vascular Surgery
Volume 8, Issue 4 , Pages 470-475, October 1988

Aortic disease associated with pregnancy☆

Presented at the Twelfth Annual Meeting of the Southern Association for Vascular Surgery, St. Thomas, Virgin Islands, Jan. 27-30, 1988.

Departments of Surgery and Anesthesia, The Johns Hopkins Hospital. Baltimore, Md.

Article Outline

Abstract 

Our experience with the management of two patients with life-threatening aortic disease during pregnancy is presented with a review of the literature. One of our patients had intimal disruption caused by trauma; the other had probable Ehlers-Danlos type IV syndrome, causing an acute dissection of the descending thoracic aorta and eventually requiring replacement of the aorta from the left subclavian artery to common iliac arteries. The challenge of treating both the pregnant woman and the fetus was managed successfully by an emergent cesarean section followed by Dacron graft replacement of the descending thoracic aorta. The literature reviewed disclosed that aneurysm expansion producing symptoms and dissection is most common during the third trimester and during labor and delivery in patients with or without Marfan's syndrome. Half of the aortic dissections in women less than 40 years of age occur in association with pregnancy. The available evidence indicates that patients with known valvular or aortic disease should have surgical repairs during the first or second trimester and thereafter have delivery by cesarean section. However, patients with acute aortic problems near term appear to be better managed by cesarean section followed promptly by treatment of the aortic disease. (J VASC SURG 1988;8:470-5.)

 

The surgeon facing the need to correct life-threatening aortic disease during pregnancy confronts the problem of saving two lives. If attention is focused primarily on the surgical repair of the aortic disease, the fetus will suffer the consequences of hypotension, hypoxia, and hypercarbia associated with occlusion of the vessels proximal to the placenta. However, if attention is focused on the fetus, delaying diagnosis and surgical therapy, both mother and child may not survive. Our experience with two cases of disruption of the descending thoracic aorta is presented and the literature reviewed to aid the surgeon in the management of these rare but challenging problems.

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Case reports 

Case 1 

A 29-year-old woman pregnant 36 weeks by date of the last menstrual period had sudden severe substernal chest and back pain. She had been completely well and normotensive throughout her pregnancy. Evaluation at the community hospital with CT scanning revealed a descending aortic dissection confined to the chest. Because of unremitting pain, she was transferred to Johns Hopkins Hospital and was subjected to aortography, which demonstrated a type IIIa aortic dissection (DcBakey classification). In addition, she was found to have small aneurysms of the subclavian arteries and the left main coronary artery was also dilated. She was transferred immediately to the operating room where a cesarean section resulted in the birth of a healthy girl. After abdominal closure, the dissected aorta was replaced with a Dacron graft from one inch distal to the left subclavian artery to the junction of the middle and lower thirds of the descending thoracic aorta. Femorofemoral bypass was used during the period of aortic occlusion. The patient had a lengthy hospital stay and was not discharged until the eighteenth postoperative day. She required large doses of antihypertensive medications for blood pressure control.

The patient was readmitted to the community hospital and subsequently referred to Johns Hopkins because of recurrent nausea, vomiting, and headache. She was found to have asymmetric blood pressures, a loud abdominal bruit, and no palpable pulses in the right lower extremity. Angiography disclosed a large abdominal aortic dissection associated with patulous coronary, vertebral, and proximal subclavian arteries. There was no visualization of the right renal, the superior mesenteric, and the distal right subclavian arteries. Evaluation for arteritis failed to disclose abnormalities in inflammatory parameters, and a biopsy of the temporal artery yielded normal results. A course of prednisone was begun but was stopped after 3 days for fear that the underlying disease was Ehlers-Danlos type IV syndrome which might be aggravated by the addition of steroids.

The patient was discharged but failed to improve and was transferred to the Methodist Hospital at Houston for possible correction of the extensive vascular problems. Replacement of the remaining thoracoabdominal aorta was done with a Dacron graft, reimplanting two pairs of intercostal arteries, three lumbar arteries, the superior mesenteric artery, the celiac axis, and both renal arteries. Although the pathologic specimen showed evidence of medial cystic necrosis, no definitive diagnosis was possible; and prednisone was started, which was sustained in modest dosages for 1 year. Nine months after aortic surgery the patient returned with ischemic symptoms in the right arm. This prompted aortosubclavian Dacron graft bypass and exclusion of the right subclavian aneurysm, which had expanded. Since then the patient has been well with stable arteries and her child is normal at 4 years 10 months.

Case 2 

A 25-year-old black woman pregnant for 32 weeks by date of the last menstrual period was brought to the Johns Hopkins emergency department after a severe automobile accident. She had not lost consciousness but was in acute distress caused by multiple injuries. The vital signs were normal and stable but the fetal heart rate varied from 55 to 70 beats/min. Extensive abrasions were evident, with swelling about the left cheek. There were second-degree burns on the buttocks. The abdomen was tender. Chest x-ray films disclosed significant widening of the mediastinum, which together with the slow fetal heart rate prompted emergent arteriography. This study disclosed a major intimal disruption of the descending thoracic aorta distal to the left subclavian artery.

The patient was taken immediately to the operating room and had a cesarean section, resulting in the birth of a healthy boy. Abdominal exploration was then performed, which revealed no major visceral injuries or bleeding sites. After abdominal closure the patient was turned onto the right side and had a left thoracotomy. The tear in the descending thoracic aorta was repaired with a Dacron graft with femorofemoral bypass during aortic occlusion. The patient and baby were discharged well at 10 days, having an uncomplicated postoperative course. The patient has been lost to follow-up.

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Literature review 

Pregnancy, aneurysm, expansion, disruption, and rupture 

The interested reader is referred to an excellent review of this subject by Barrett et al.1 Although controversy exists as to whether or not the arterial wall is truly weakened during pregnancy, there is little doubt that pregnancy creates stress for diseased arteries. Half of aortic ruptures in women less than 40 years of age are associated with pregnancy.2 Some have suggested that changes in hemodynamics that occur during pregnancy and that are associated temporally with arterial catastrophes are causative. Blood volume, cardiac output, stroke volume, and blood pressure all increase during the third trimester during which half of the ruptures occur. In a review of this subject, Konishi et al.3 found that in 51 ruptures of the aorta and its branches, 6% occurred in the first trimester, 10% in the second, and 51% in the third. Fourteen percent occurred during labor and 20% in the puerperium. Marfan's syndrome was present in only 3 of 51 cases and no diagnosis was possible in 20 cases. No intimal tears have been reported to occur below the diaphragm.

Arterial rupture is one of the leading causes of maternal death. Cerebral aneurysm rupture appears to be the most common and 12% to 80% of all maternal deaths have been related to their rupture.4 Twenty percent of all splenic artery aneurysms rupture during pregnancy,5 and in 91 cases collected by O'Grady et al.,6 only 27 mothers and five fetuses survived. Dismal survival rates also accompanied diseases of the aorta appearing during pregnancy but these cases occurred before modern surgical and anesthetic techniques. Pedowitz and Perell7 published a review in 1957 disclosing that all patients with ruptures of fusiform or saccular aortic aneurysm died. Seventeen patients with such aneurysms did not have rupture and survived pregnancy. Two subsequently died after surgical correction. Forty-five of 48 patients with dissecting aneurysms died. In another review Wolff8 reported on the outcome of 73 cases of dissecting aortic aneurysms during pregnancy, parturition, and puerperium. Nineteen patients (26%) died in 1 hour, 44 (60%) died within 48 hours, and 67 (92%) died within 10 weeks. Most dissections (89%) occurred in the ascending aorta. Therefore, although dissecting and saccular aneurysms were relatively rare, they represented catastrophic problems before current methods of diagnosis and management.

Modern therapy for dissecting aneurysms of the ascending aorta during pregnancy 

The modern era of management of ascending aortic dissections during pregnancy really began in 1983 but was ushered in by a dramatic case report of Hume and Krosnick9 published 20 years earlier (Table I).

Table I. Successful pregnancy and surgical management of dissection beginning in the ascending aorta
AuthorYearPresentationDuration of Pregnancy (wk)Age (yr)Method of diagnosisSurgical procedure(s)
Hume & Krasnick91963Chest and left leg pain40 (labor)32Celiotomy at time of cesarean section, subsequent angiographyCesarean section, left common iliac ”window“, repair ascend. aorta & valve 20 days later
Konishi et al.31980Chest and back pain3632AngiographyCesarean section followed by emposite graft and valve repair 8 wk later
Gonzalez-Lavin101983Pulmonary edema, left ventricular failure3730AngiographyCesarean section; immediate composite repair
Ferguson et al.111983Chest pain, hypotension, fetal distress39 (labor)42AngiographyCesarean section, air transport for immediate composite repair, subsequent bypass to right subclavian artery
Katz et al.121984Chest pain3238EchocardiogramCesarean section followed by immediate repair of aorta & valve
Cola & Lavin131985Chest pain in known Marfan's syndrome1 wk (sic)34Not statedComposite valve & graft, coronary graft, cesarean section 33 wk later
Pumphrey et al.141986Chronic mild chest pain3719Echocardiogram, angiographyCesarean section complicated by incomplete uterine contraction, delayed repair 2 days later
Their patient was 32 years old and presented in labor with acute chest pain and left leg ischemia. An emergency cesarean section resulted in the birth of a living baby. Thereafter a dissection was noted in the lower abdominal aorta extending into the left common iliac artery, resulting in total occlusion of the external iliac artery. The intima was removed from the common iliac artery creating a window and a bypass graft successfully restored circulation to the left leg. However, one week later left ventricular failure developed with periodic pulmonary edema that eventually proved to be intractable. Therefore, 20 days after the birth, the ascending aorta was resected with Dacron graft replacement and the aortic valve was restructured and suspended. Recovery was slow but mother and child were well at 1 year. This was truly a land-mark case and remains unique because we could find no other reports of success in dealing with a dissection of this magnitude.

Of the six case reports that followed,2, 10, 11, 12, 13, 14 one was distinctive because the patient initially had symptomatic ascending aortic dissection during the first week of pregnancy.13 The authors followed the general precepts of terminating pregnancy by cesarean section when the fetus was sufficiently developed to expect a normal infancy rather than subject mother and fetus to the risks of late pregnancy and labor. The remaining five case reports involved women in late pregnancy with acute symptoms prompting delivery by cesarean section followed by repair of the ascending aortic dissection and attendant aortic valve problems.

Although a consensus may be developing regarding the treatment of ascending aortic dissections and late term pregnancy, little data other than the case cited exist to establish management principles for women with less advanced pregnancy. The risk to the fetus necessitated by complex repairs of the ascending aorta, the aortic valve, and the coronary arteries with extensive periods of cardiopulmonary bypass remains unknown. The best information stems from a review by Becker,15 who canvassed the members of the Society of Thoracic Surgery about their experience with cardiopulmonary bypass and maternal-fetal survival. Most of the cases represented patients in the second trimester with valvular heart disease. In this group, 8 of 29 fetuses were aborted in women having mitral or aortic valve replacement. Patients requiring less time on bypass such as those having open mitral valvulotomy had far fewer problems as only 1 of 23 patients had abortion. However, the risk to the woman and hence to the fetus is certainly high in women who become symptomatic during the earlier stages of pregnancy. Therefore it seems best to proceed with definitive repairs in patients with documented dilatation of the aortic root as recommended by Pyeritz.16

Modern therapy for dissecting aneurysms of the descending aorta during pregnancy 

Some type III dissections have been treated successfully medically. Others required a combination of initial medical therapy followed by surgical repair after pregnancy was terminated (Table II).

Table II. Successful management of dissecting descending aortic aneurysms associated with pregnancy
AuthorYearPresentationDuration of Pregnancy (wk)Age (yr)DiagnosisTreatmentOutcome
Kitchen171974Chest and back pain3225Widened mediastinum, surgery (cesarean)Medical; cesarean section at 36 wk losing babyTwo successful subsequent pregnancies by cesarean section
Konishi et al.31980Chest and back pain2 days postpartum25AngiogramMedicalRepeat angiography at 6 mo normal, well at 4 yr
Konishi et al.31980Back pain, dyspnea2034Thought to be pancreatitis at 20 wk, recurrent symptoms 3 days post-partum, chest x-ray—widening.Vaginal delivery; surgical replacement of descending thoracic aorta 3 mo laterSurvival
Konishi et al.31980Back and chest pain1645AngiographyHysterotomy; surgical replacement descending thoracic aorta 10 days laterSurvival
Merin et al.181981Blunt trauma, auto accident2427Widened mediastinum, angiographyDacron graft replacement of tear with high flow normothermic femorofemoral bypass; cesarean section at 8 moSurvival of mother & child
Current reportBlunt trauma, auto accident3225Widened mediastinum, angiographyCesarean section; immediate resection of tear & replacement with Dacron graftSurvival of mother & child
Acute chest and upper back pain3629CT scan, angiographyCesearean section, immediate resection of the dissected thoracic aorta & Dacron graft; subsequent replacement of remaining descending abdominal aortaSurvival of mother & child
We were able to find two reports of patients having acute type III dissections that did not require surgical therapy. In the first,17 the patient had symptoms of acute dissection at 32 weeks' gestation. Her condition stabilized and cesarean section was successful with the delivery of a normal baby at 36 weeks. She then had two successful pregnancies, each ending with cesarean section. The second report is of a patient who had symptoms two days after the birth. Her condition also stabilized and a repeat angiogram performed at 6 months disclosed obliteration of the false lumen.3

A third patient was managed conservatively because the symptoms of dissection present at 20 weeks were mistakenly attributed to pancreatitis.3 The patient completed the pregnancy successfully, culminating in the vaginal delivery of a normal child only to have recurrent symptoms 2 days after the birth and eventual operative repair 3 months later. Another example of type III dissection occurring in the second trimester was presented by the same authors.3 In this case a 16-week pregnancy was terminated by hysterotomy. When symptoms continued, surgical replacement of the descending aorta was performed successfully 10 days later.

One other case was similar to ours and was caused by blunt trauma.18 However, as the fetus was small (6 months) the authors proceeded directly to the replacement of the aortic disruption. The pregnancy was sustained, which was attributed to high-flow normothermic femorofemoral bypass during aortic occlusion. A healthy baby was delivered by cesarean section 2 months later.

Both of our patients came for treatment closer to term and were managed better by an immediate cesarean delivery followed by repair of the descending aorta.

Abdominal aortic disruptions and pregnancy 

We were able to find only three cases of pregnancy-related rupture of the abdominal aorta since 1975. None of the patients was white. One patients was a 40-year-old, black South African.19 A typical infrarenal abdominal aortic aneurysm ruptured on the seventh postpartum day. The patient survived the operation but the surgeons were unable to construct a watertight anastomosis between the graft and the right common iliac artery. Sutures placed in this vessel simply tore out. This is the only report suggesting that arterial tissue is unsuitable for reconstruction. However, the common iliac artery was ligated and the limb survived on collateral vessels. A second patient was a 29-year-old black woman who had a sympomatic large abdominal aortic aneurysm, which was successfully resected.20 She returned with a false aneurysm thought to be septic and underwent removal of the aortic graft, aortic closure above the old anastomosis and axillobifemoral artery bypass to sustain circulation to the legs. Ten months later when the patient had a pregnancy of 24 weeks' gestation, she was admitted for disruption of the aortic turn-in associated with an aortojejunal fistula. She died during the operation. The third patient was a 25-year-old Thai woman who came for treatment 14 weeks' pregnant with an 8 cm abdominal aneurysm.21 She had a hysterotomy followed by aneurysm repair. She then became pregnant and had successful cesarean section. The aortic graft was intact by palpation.

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Discussion 

There is little doubt that pregnancy predisposes women to aortic disruption and that women with inherited conditions such as Marfan's syndrome16 or Ehlers-Danlos disorders22 deserve special consideration. Pregnancy can be successful, for in a series followed by Pyeritz,16 26 women with Marfan's syndrome had 105 pregnancies resulting in 80 live births without aortic dissection. Pyeritz recommends frequent echocardiography during pregnancy with surgical intervention should the aortic root become significantly widened. However, rupture has been reported in one patient who was observed during pregnancy and still had a type I dissection.23 Magnetic resonance imaging appears to be the optimal technique for following aortic disease in other locations because it avoids radiation exposure while providing excellent imaging of the aorta.

The evidence favors an aggressive surgical approach to significant disease of the ascending and abdominal aorta during the first and second trimesters. Localized disease of the descending thoracic aorta may also be managed best surgically. If the pregnancy is sustained, delivery should be by cesarean section. Aortic disruption in the third trimester is managed best by cesarean delivery and immediate repair of these lesions. The application of these principles has produced more reports of survivors in the last eight years than in the world's past experience.

A consensus is developing that type III dissections in nonpregnant women are best treated by normalizing blood pressure, reserving surgical repair for the patients who remain symptomatic or have documented extension of the dissection.24 If replacement of the descending thoracic aorta with reattachment of important intercostal and visceral arteries can be deferred for several months, sutures hold better and the procedure has less risk. However, the application of these principles deserves careful thought in pregnancy for two reasons. First, the mainstay of antihypertensive therapy, infusions of nitroprusside, runs the risk of inducing fetal cyanide poisoning.25 Second, the continuance of the pregnancy imposes a hemodynamic and probable metabolic burden on the injured blood vessel. To lessen these risks, pregnancy seems best terminated by cesarean delivery or hysterotomy if medical means are used to treat the acute aneurysmal disease. However, as some risk extends into the puerperium, we recommend immediate resections of localized lesions such as traumatic disruptions or type IIIa lesions producing severe symptoms. More complex dissections with abdominal reentry may be managed best by antihypertensive medication with the hope of stabilization or post-ponement of surgical repair for a more optimal time.

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References 

  1. Barrett JM, VanHooydonk JE, Boehm FH. Pregnancy related rupture of arterial aneurysms. Obstet Gynecol Surv. 1982;37:557–566
  2. Mandel W, Evans EW, Walford RL. Dissecting aortic aneurysm during pregnancy. N Engl J Med. 1954;26:1059–1061
  3. Konishi Y, Tatsuta N, Kumada K, et al.  Dissecting aneurysm during pregnancy and the puerperium. Jpn Circ J. 1980;44:726–733
  4. Miller JH, Hinkley CM. Berry aneurysms in pregnancy: a ten year report. South Med J. 1970;63:279–284
  5. Vassulotti SB, Schauller JA. Spontaneous rupture of splenic artery aneurysm during pregnancy: report of first known antepartum rupture with maternal and fetal survival. Obstet Gynecol. 1967;30:264–268
  6. O'Grady J, Day E, Toole A, Paust JC. Splenic artery aneurysm rupture in pregnancy. A review and case report. Obstet Gynecol. 1977;50:627–630
  7. Pedowitz P, Perell A. Aneurysms complicated by pregnancy. Part I. Aneurysms of the aorta and its major branches. Am J obstet Gynecol. 1957;73:720–735
  8. Wolff J. Dissecting aortic aneurysm during pregnancy, parturition, and puerperium. Ned T Verlosk (Dut). 1961;61:23–81
  9. Hume M, Krosnick G. Dissecting aneurysm in pregnancy associated with aortic insufficiency. N Engl J Med. 1963;286:174–178
  10. Gonzalez-Lavin L. Discussion following Becker RM. Intracardiac surgery in pregnant women. Ann Thorac Surg. 1983;36:457
  11. Ferguson JE, Ueland K, Stinson EB, Maley RP. Marfan's syndrome: acute aortic dissection during labor, resulting in fetal distress and cesarean section, followed by successful surgical repair. Am J Obstet Gynecol. 1983;147:759–762
  12. Katz NM, Collea JV, Moront MG, MacKenzie RD, Wallace RB. Aortic dissection during pregnancy: treatment by emergency cesarean section immediately followed by operative repair of the aortic dissection. Am J Cardiol. 1984;54:699–701
  13. Cola LM, Lavin JP. Pregnancy complicated by Marfan's syndrome with aortic arch dissection, subsequent aortic arch replacement and triple coronary artery bypass grafts. J Reprod Med. 1985;30:685–688
  14. Pumphrey CW, Fay T, Weir L. Aortic dissection during pregnancy. Br Heart J. 1986;55:106–108
  15. Becker RM. Intracardiac surgery in pregnant women. Ann Thorac Surg. 1983;36:453–458
  16. Pyeritz RE. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med. 1981;71:784–789
  17. Kitchen DH. Dissecting aneurysm of the aorta in pregnancy. J Obstet Gynaecol Br Commonw. 1974;81:410–413
  18. Merin G, Bitran D, Donchin Y, Weinstein D, Borman JB. Traumatic rupture of the thoracic aorta during pregnancy. Surgical considerations. Chest. 1981;79:99–100
  19. Parkes JR, Hendry DT, Hellberg BW, Theron LL. Postpartum ruptured abdominal aortic aneurysm: A case report. S Aft Med J. 1977;51:926–927
  20. Matseoane S, Freeman H, Williams J, Ganepola G, Brisbane S. Pseudoaneurysm of aortic stump: rupture in pregnancy. NY State J Med. 1981;81:365–367
  21. Manotaya T, Manothaya C. Pregnancy after removal of abdominal aortic aneurysm. Obstet Gynecol. 1976;47:12S–14S
  22. Snyder RR, Gilstrap LC, Hauth JC. Ehlers-Danlos syndrome and pregnancy. Obstet Gynecol. 1983;61:649–650
  23. Rosenblum NG, Grossman AR, Gabbe SG, Mennuti MT, Cohen AW. Failure of serial echocardiographic studies to predict aortic dissection in a pregnant patient with Marfan's syndrome. Am J Obstet Gynecol. 1983;146:470–471
  24. Wheat MW, Harris PD, Malm JR, Kaiser G, Bowman FO, Palmer RF. Acute dissecting aneurysms of the aorta. Treatment and results in 64 patients. J Thorac Cardiovasc Surg. 1969;58:344–351
  25. Naulty J, Cefulo RC, Lewis PE. Fetal toxicity of nitroprusside in the pregnant ewe. Am J Obstet Gynecol. 1981;139:708–712

☆ Reprint requests: G. Melville Williams, MD, 600 N. Wolfe St., Blalock 606, Baltimore, MD 21205.

PII: 0741-5214(88)90113-9

doi:10.1067/mva.1988.avs0080470

Journal of Vascular Surgery
Volume 8, Issue 4 , Pages 470-475, October 1988

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