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DIAGNOSIS AND TREATMENT

Cardiovascular Problems in Pregnant Women with the Marfan Syndrome

right arrow Uri Elkayam; Enrique Ostrzega; Avraham Shotan; and Anilkumar Mehra

15 July 1995 | Volume 123 Issue 2 | Pages 117-122

Purpose: To review the available information on the diagnostic, prognostic, and therapeutic aspects of cardiac complications in women with the Marfan syndrome during the peripartum period and to develop guidelines for the approach to these patients on the basis of this information.

Data Sources: A MEDLINE search and a manual search of bibliographies from reviewed articles.

Study Selection and Data Extraction: Articles that reported on pregnancy in patients with the Marfan syndrome or that discussed potentially relevant aspects of the syndrome.

Results: Pregnancy in the Marfan syndrome is associated with two primary problems: potential catastrophic aortic dissection and the risk for having a child with the syndrome. The risk for peripartum aortic dissection is especially high in women in whom aortic root dilatation is diagnosed before pregnancy. Gestation seems to be safer in women without preexisting cardiovascular disease; however, an event-free pregnancy cannot be guaranteed. The Marfan syndrome is inherited in an autosomal dominant manner, and the fetus has a 50% risk for inheriting the mutant gene.

Conclusions: Women with the syndrome should be counseled before conception about the risks of pregnancy to both mother and fetus. Because preconceptual dilatation of the ascending aorta seems to be an important predictor for aortic dissection, it should be excluded before pregnancy. Transesophageal echocardiography seems to be preferable for noninvasive assessment of aortic dilatation before and during pregnancy. Prophylactic use of ß-blockers may be useful in preventing aortic dilatation. Surgery should be considered during gestation in patients with progressive aortic dilatation when or before the aortic root reaches 5.5 cm. Because of the potential risk of ionizing radiation to the fetus, noninvasive methods such as transesophageal echocardiography and magnetic resonance imaging are preferred to contrast aortography for the diagnosis of aortic dissection during pregnancy. Vaginal delivery can be done in patients with the Marfan syndrome who do not have cardiovascular system abnormalities. In patients with aortic dilatation, aortic dissection, or other important cardiac abnormalities, cesarean section should be the preferred method of delivery.

The Marfan syndrome is a hereditary disorder of the connective tissue [1-5] with an estimated prevalence of 4 to 6 cases per 10 000 persons; prevalence does not differ according to sex, race, or ethnicity [4]. The syndrome is caused by abnormalities in the relation between fibrillin and fibers that are caused by an abnormal gene for fibrillin [1, 2] on chromosome 15 [3]. A family history of the disease is present in 65% to 75% of patients and is sporadic in the rest. Cardiovascular involvement, including mitral and tricuspid valve prolapse with or without valvular regurgitation, dilatation of the aorta (primarily of the ascending portion), and aortic regurgitation, is a common feature of the disease [4, 6]. Life expectancy is greatly reduced in patients with this syndrome, predominantly because of cardiac complications (aortic dilatation, dissection, and rupture) [7].


Cardiovascular Risk of Pregnancy
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Pregnancy in women with the Marfan syndrome poses two problems: a potential catastrophic and often lethal acute aortic dissection and the risk for having a child who will inherit the syndrome. In a review of the literature published up to 1980, Pyeritz [8] found reports of 32 women with the Marfan syndrome who had had at least one pregnancy. Acute aortic dissection was diagnosed in 20 of these women, of whom 16 died during or shortly after pregnancy and 4 died later in the postpartum period because of aortic rupture or regurgitation. Most of these patients had had preexisting cardiovascular abnormalities, including aortic dilatation, aortic regurgitation, coarctation of the aorta, hypertension, cardiomegaly, and ductus arteriosus.

A review of the literature since 1980 shows the description of 15 additional cases of pregnancy in women with the Marfan syndrome. Most of these reports describe cardiovascular complications during pregnancy Table 1, including 1) dilatation of the ascending aorta with the development of aortic regurgitation and congestive heart failure and 2) proximal and distal dissections of the aorta with the occasional involvement of the iliac [9, 11] and coronary arteries [13]. Most women developed cardiac complications in the second and third trimesters, although aortic dissection occurred in isolated patients a few days after conception [15], during labor [9], and 8 days after delivery [16]. Aortic dissections occurring in the 14th, 28th, and 32nd gestational weeks each resulted in a maternal death [10, 14]. Live babies were delivered before surgery by cesarean sections at the 36th week in two patients [11, 12] and at the 38th week in one patient [13]. In all three of these patients, surgical repair was done successfully 3 days to 6 weeks after delivery. In two other patients, surgery was done during pregnancy. In one of these patients [15], aortic arch replacement and triple coronary artery bypass were done a few days after conception, and a normal baby was delivered by cesarean section in the 34th gestational week. In the other patient, who had a sinus of Valsalva aneurysm and aortic regurgitation, the aortic valve was successfully replaced in the 22nd week of pregnancy. The operation, however, was complicated by maternal hypotension that resulted in a decrease in fetal heart rate from 120 to 40 beats per minute. On day 10 after surgery, the patient had cardiac arrest caused by cardiac arrhythmia and was successfully resuscitated. Pregnancy was continued for 6 more weeks, after which the patient delivered a premature baby with the Marfan syndrome and respiratory distress.


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  		Table 1. A Summary of Cases Reported since 1980 in Which Peripartum Cardiovascular Complications Occurred in Women with the Marfan Syndrome

 

The cause of the increased incidence of aortic dissection during pregnancy is not clear. An association between predisposition to dissection and the hyperdynamic and hypervolemic cardiocirculatory state of pregnancy is possible. In addition, estrogen has been reported to inhibit collagen and elastin deposition in the aorta, and progestogen has been shown to accelerate deposition of noncollagenous proteins in the aortas of rats [18]. Such structural changes in the arterial wall during pregnancy may also contribute to aortic dissection [19].

Although most reports describe severe complications related to pregnancy in women with the Marfan syndrome, these reports are probably an overpresentation of pregnancy-related complications caused by a bias toward reporting complicated rather than uncomplicated cases. Such an assumption is supported by Pyeritz [8], whose retrospective analysis of 105 pregnancies in 26 patients with the Marfan syndrome and prospective follow-up of 10 patients with the syndrome who had minimal or no preexisting cardiovascular disease showed only a low risk for maternal complications and death. In addition, two recent reports [20, 21] have described successful pregnancies in 3 patients despite mild to moderate aortic root enlargement and mitral valve prolapse in all 3 patients and a moderate degree of left ventricular systolic dysfunction in 1 patient.

In addition to the maternal risk associated with pregnancy in the Marfan syndrome, there is a risk for transmitting the disease to the fetus. The Marfan syndrome is inherited as an autosomal dominant disorder [22], and the fetus has a 50% chance of inheriting the mutant gene [5].


Preconceptual Evaluation and Consultation
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Because no large clinical trials of pregnancy in patients with the Marfan syndrome have been reported, our recommendations are made on the basis of general principles rather than trial data. Women with the Marfan syndrome should be counseled before conception about the risk for potential pregnancy-related complications and the risk for transmitting the syndrome to the offspring. Mildly affected patients should be informed about the different presentations of the disease and the possibility of more severe expression in the offspring [5]. It should be noted that because of better understanding of the gene defect of the Marfan syndrome [2, 3, 23-26], prenatal diagnosis of this disease may be possible in some patients of informative families in which the disease cosegregates with marker alleles [5, 27]. During preconceptual consultation, physicians should carefully counsel the patient and her family about the expected morbidity of the mother in years to come and the possibility of reduced life expectancy.

Many pregnancy-related complications described in patients with the Marfan syndrome emphasize the great potential for risk associated with gestation, especially in patients with cardiovascular involvement. Such cardiovascular abnormalities should be carefully evaluated before and frequently throughout pregnancy. Preconceptual dilatation of the ascending aorta seems to be an important predictor of aortic dissection during gestation and should be excluded before pregnancy. Reports of aortic dissection in the Marfan syndrome in pregnant [12] and nonpregnant patients with normal aortic root diameter [28] show that event-free pregnancy cannot be guaranteed to any patient with this syndrome. Recently, Simpson and colleagues [29] showed that transesophageal echocardiography was superior to transthoracic echocardiography in the assessment of aortic diameter and the diagnosis of aortic dissection and other cardiovascular manifestations of the Marfan syndrome. The use of transesophageal echocardiography should, therefore, be preferred for preconceptual risk stratification in women with the Marfan syndrome.


Surgery during Pregnancy
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If a pregnant patient with the Marfan syndrome has substantial dilatation of the aorta, therapeutic abortion or surgical intervention should be considered. Surgery for marked dilatation of the aorta [17] and for aortic dissection [15] has been reported during gestation. Cola and Lavin [15] recently reported successful aortic arch replacement and coronary artery bypass grafting for aortic dissection in a patient with the Marfan syndrome; the surgery was done a few days after conception with normal fetal outcome. Smith and coworkers [17] reported successful replacement of the aortic valve and the ascending aorta during the 22nd week of gestation; this was done because of symptomatic dilatation of the aorta from 5.5 cm to 7.7 cm during pregnancy.

Gott and colleagues [30] showed a 5-year survival rate of 85% in 50 patients with the Marfan syndrome after composite graft repair of the ascending aorta. They recommended preventive replacement of the ascending aorta if the aorta reaches or exceeds 60 mm. A recent study by Murgatroyd and colleagues [28] reported aortic root dimension to be 5.1 ±1.3 cm in 11 patients who developed aortic dissection. In contrast, the average aortic dimension in patients with uncomplicated courses was 3.7 ±1.3. On the basis of these data, a recent editorial [31] recommended elective replacement of the aortic root when or before the root reaches 5.5 cm in patients with the Marfan syndrome who show progressive dilatation of the aortic root by serial echocardiographic assessment, in patients with a family history of aortic dissection, and in women who are planning pregnancy.

Successful surgery for aortic dissection during pregnancy has been reported in a few cases [32-34]. It should be noted, however, that cardiac surgery in general has been shown to result in increased fetal loss [35]. For this reason, if fetal maturity can be confirmed, a cesarean section should be done before or concomitantly with thoracic surgery [8, 10, 12].


Prophylactic Use of ß-blockers
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Several preliminary studies [36-39] have suggested that ß-blocking agents may have a beneficial effect on the rate of aortic root dilatation in children and adolescents. These initial results are strongly supported by a recent report by Shores and colleagues [40], who did a randomized study of the effect of propranolol (mean dose, 212 ±68 mg/d) on the progression of aortic dilatation in adolescents and adults with the Marfan syndrome for approximately 10 years. These investigators showed a lower rate of aortic dilatation and a significantly reduced rate of aortic regurgitation, aortic dissection, cardiovascular surgery, congestive heart failure, and death in the patients treated with propranolol. The applicability of these data to pregnant patients needs to be further studied but, on the basis of available information, the prophylactic use of ß-blockers during pregnancy seems to make good clinical sense. Propranolol has been extensively used during pregnancy to treat various conditions, including hypertension [41-44], thyrotoxicosis [45, 46], hypertrophic cardiomyopathy [47, 48], and maternal [43, 49, 50] and fetal supraventricular tachyarrhythmias [41, 50-54]. Although the overall experience with this drug in pregnancy has been favorable [55], potential side effects, including fetal growth retardation [43], bradycardia, hypoglycemia, hyperbilirubinemia, and apnea at birth in the newborn, have been reported [56, 57]. Such side effects should be anticipated by the clinician. Barden and Stander [58] have shown that propranolol given to pregnant women blocks the inhibitory effects of epinephrine on myometrial activity. The nonselective ß-blocking effect of propranolol may, therefore, facilitate an increase in uterine activity. Although the clinical relevance of these findings is not clear, the use of ß1-adrenergic receptor- blocking agents, such as metoprolol and atenolol, may be preferred during pregnancy. Several studies have shown these agents to be safe when used to treat hypertension during pregnancy [59-61]. A few studies, however, have reported lower birth weight in association with exposure to atenolol during gestation [62]. ß-blocking agents are excreted in breast milk [63-65]. However, unless hepatic function in the newborn is markedly impaired, breast- feeding should not be discouraged.


Diagnosis of and Medical Therapy for Aortic Dissection during Pregnancy
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Transthoracic echocardiography has traditionally been used to assess the ascending aorta in patients with the Marfan syndrome. The recent introduction of transesophageal echocardiography has provided the clinician with a highly effective tool for the diagnosis of aortic dissection in both nonpregnant and pregnant patients [66, 67]. Because aneurysm of the aorta in the patients with the syndrome can occasionally also involve the descending aorta [68], transesophageal echocardiography seems preferable to transthoracic echocardiography for both preconceptual assessment and periodic follow-up during pregnancy. This is further supported by a recent study by Simpson and coworkers [29], who report that this technique is superior to transthoracic echocardiography in the diagnosis of aortic dissection and dilation in patients with the Marfan syndrome. Magnetic resonance imaging may be equally effective in the stable patient, but the safety of this technique during pregnancy has not been completely established. No evidence suggests that short-term exposure to an electromagnetic field can harm the fetus, but prolonged or high-level exposure to electromagnetic radiation has been linked to unfavorable effects on embryogenesis and chromosomal structure [69, 70]. Until conclusive information about the effect of magnetic resonance imaging on the fetus is established in many studies with long follow-up periods, echocardiography seems preferable [69]. If contrast aortography is done, an attempt should be made to minimize the use of radiation and to adequately shield the fetus [71].

Standard medical therapy for aortic dissection includes the use of intravenous nitroprusside and ß-blockers to control blood pressure and decrease left ventricular contractility, thereby reducing ejection velocity and minimizing shear forces [72]. The use of nitroprusside during pregnancy, however, may lead to thiocyanate toxicity in the fetus [55]. Thus, the gestational use of hydralazine to control blood pressure is preferred. Hydralazine has been used extensively to control blood pressure during pregnancy, and its safety has been well established [55].


Labor and Delivery
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The available literature and our own experience show that vaginal delivery can be done in patients with the Marfan syndrome who have normal results on cardiovascular examination and no evidence of aortic dilatation. In these patients, cesarean section should be reserved for obstetrical indications [20, 21, 73, 74]. At the same time, however, to reduce the stress of labor, epidural anesthesia to minimize pain and forceps or vacuum to shorten the second stage of labor are recommended. Both systolic and diastolic blood pressures increase markedly during uterine contractions [75]. These changes should be anticipated and prevented with ß-blocking or vasodilator agents. In patients with aortic dilatation, aortic dissection, or other important cardiac abnormalities, cesarean section should be the preferred method of delivery because it minimizes the hemodynamic changes associated with vaginal delivery. A recent report by Irons and Pollard [76] described postpartum hemorrhage of the uterine vasculature 3 days after cesarean section secondary to the Marfan syndrome. Similar postpartum hemorrhage was reported by Pyeritz [8] in 4 of 11 women with the Marfan syndrome and should be anticipated in such patients.


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From the University of Southern California School of Medicine, Los Angeles, California.
Requests for Reprints: Uri Elkayam, MD, University of Southern California School of Medicine, Division of Cardiology, 2025 Zonal Avenue, Room 7621, Los Angeles, CA 90033.


References
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1. Milewcz DM, Pyeritz RE, Crawford ES, Byers PH. Marfan syndrome: defective synthesis, secretion and extracellular matrix formation of fibrillin by cultured dermal fibroblast. J Clin Invest. 1991; 88:79-86.

2. Dietz HC, Cutting GR, Pyeritz RE, Maslen CL, Sakai LY, Corson GM, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature. 1991; 352:337-9.

3. Kainulainen K, Sakai LY, Child A, Pope FM, Puhakka L, Ryhanen L, et al. Two mutations in Marfan syndrome resulting in truncated fibrillin polypeptides. Proc Natl Acad Sci U S A. 1992; 89:5917-21.

4. Pyeritz RE, McKusick VA. The Marfan syndrome: diagnosis and management. N Engl J Med. 1979; 300:772-7.

5. Rossiter JP, Johnson TR. Management of genetic disorders during pregnancy. Obstet Gynecol Clin North Am. 1992; 19:801-13.

6. Beighton P, de Paepe A, Danks D, Finidori G, Gedde-Dahl T, Goodman R, et al. International Nosology of Heritable Disorders of Connective Tissue, Berlin, 1986. Am J Med Genet. 1988; 29:581-94.

7. Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med. 1972; 286:804-8.

8. Pyeritz RE. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med. 1981; 71:784-90.

9. Ferguson JE II, Ueland K, Stinson EB, Maly 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; 147:759-62.[Medline]

10. Baltazar RF, Mower MM, Aquino N, Udoff EJ, Friedman R. Echocardiogram of the month. Acute pulmonary edema in a pregnant patient. Arch Intern Med. 1983; 143:781-3.

11. Mor-Yosef S, Younis J, Granat M, Kedari A, Migalter A, Schenker JG. Marfan's syndrome in pregnancy. Obstet Gynecol Surv. 1988; 43:382-5.

12. 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; 146:470-1.

13. Shime J, Mocarski EJ, Hastings D, Webb GD, McLaughlin PR. Congenital heart disease in pregnancy: short- and long-term implications. Am J Obstet Gynecol. 1987; 156:313-22.

14. Metcalfe J, McAnulty JH, Ueland K, eds. Heart Disease and Pregnancy: Physiology and Management. Boston: Little, Brown; 1986: 144-9.

15. Cola LM, Lavin JP Jr. Pregnancy complicated by Marfan's syndrome with aortic arch dissection, subsequent aortic arch replacement and triple coronary artery bypass grafts. J Reprod Med. 1985; 30:685-8.

16. Barker SG, Burnand KG. Retrograde iliac artery dissection in Marfan's syndrome. A case report. J Cardiovasc Surg (Torino). 1989; 30:953-4.

17. Smith VC, Eckenbrecht PD, Hankins DV, Leach CL. Marfan's syndrome, pregnancy, and the cardiac surgeon. Mil Med. 1989; 154:404-6.

18. Wolinsky H. Effects of estrogen and progestogen treatment on the response of the aorta of male rats to hypertension. Morphological and chemical studies. Circ Res. 1972; 30:341-9.

19. Elkayam U, Rose J, Jamison M. Vascular aneurysms and dissections during pregnancy. In: Elkayam U, Gleicher N, eds. Cardiac Problems in Pregnancy. New York: Alan R. Liss; 1990:215-29.

20. Bailey MK, Hwu-Yun R, Baker JD III, Cooke JE, Conroy JM. Marfan syndrome in the parturient. J S C Med Assoc. 1989; 85:327-30.

21. Gordon CF III, Johnson MD. Anesthetic management of the pregnant patient with Marfan syndrome. J Clin Anesth. 1993; 5:248-51.[Medline]

22. Pyeritz RE. The Marfan syndrome. Am Fam Physician. 1986; 34:83-94.

23. Dietz HC, Pyeritz RE, Hall BD, Cadle RG, Hamosh A, Schwartz J, et al. The Marfan syndrome locus: confirmation of assignment to chromosome 15 and identification of tightly linked markers at 15q15-q21.3. Genomics. 1991; 9:355-61.

24. Kainulainen K, Pulkkinen L, Savolainen A, Kaitila I, Peltonen L. Location on chromosome 15 of the gene defect causing Marfan syndrome. N Engl J Med. 1990; 323:935-9.

25. Lee B, Godfrey M, Vitale E, Hori H, Mattei MG, Sarfarazi M, et al. Linkage of Marfan syndrome and a phenotypically related disorder to two different fibrillin genes. Nature. 1991; 352:330-4.

26. Maslen CL, Corson GM, Maddox BK, Glanville RW, Sakai LY. Partial sequence of a candidate gene for the Marfan syndrome. Nature. 1991; 352:334-7.

27. Godfrey M, Vandemark N, Wang M, Velinov M, Wargowski D, Tsipouras P, et al. Prenatal diagnosis and a donor splice site mutation in fibrillin in a family with Marfan syndrome. Am J Hum Genet. 1993; 53:472-80.

28. Murgatroyd F, Child A, Poloniecki J, Treasure T, Pumphrey C. Does routine echocardiographic measurement of the aortic root diameter help predict the risk of aortic dissection in the Marfan syndrome? (Abstract) Eur Heart J. 1991; 12:410.

29. Simpson IA, de Belder MA, Treasure T, Camm AJ, Pumphrey CW. Cardiac manifestations of Marfan's syndrome: improved evaluation by transesophageal echocardiography. Br Heart J. 1993; 69:104-8.

30. Gott VL, Pyeritz RE, Magovern GJ Jr, Cameron DE, McKusick VA. Surgical treatment of aneurysms of the ascending aorta in the Marfan syndrome. Results of composite-graft repair in 50 patients. N Engl J Med. 1986; 311:1070-4.

31. Treasure T. Elective replacement of the aortic root in Marfan's syndrome. Br Heart J. 1993; 69:101-3.

32. 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.

33. Pumphrey CW, Fay T, Weir I. Aortic dissection during pregnancy. Br Heart J. 1986; 55:106-8.

34. Snir E, Levinsky L, Salomon J, Findler M, Levy MJ, Vidne BA. Dissecting aortic aneurysm in pregnant women without Marfan disease. Surg Gynecol Obstet. 1988; 167:463-5.

35. Becker RM. Intracardiac surgery in pregnant women. Ann Thorac Surg. 1983; 36:453-8.

36. Ose L, McKusick VA. Prophylactic use of propranolol in the Marfan syndrome to prevent aortic dissection. Birth Defects. 1977; 13:163-9.

37. Pyeritz RE. Propranolol retards aortic root dilatation in the Marfan syndrome (Abstract). Circulation. 1983; 68:365.

38. Zahka KG, Hensley C, Glesby M, Pyeritz RE. The impact of medical and surgical therapy on the cardiovascular prognosis of the Marfan syndrome in early childhood (Abstract). J Am Coll Cardiol. 1989; 13:119A.

39. Rosen SE, Roman MJ, Pini R, Kramer-Fox R, Devereux RB. The impact of chronic ß-blockade therapy on arterial compliance in the Marfan syndrome (Abstract). Am J Med Genet. 1993; 47:157.

40. Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilatation and the benefit of long-term ß-adrenergic blockade in Marfan's syndrome. N Engl J Med. 1994; 330:1335-41.

41. Eliahou HE, Silverberg DS, Reisin E, Romem I, Mashiach S, Serr DM. Propranolol for the treatment of hypertension in pregnancy. Br J Obstet Gynaecol. 1978; 85:431-6.

42. Tcherdakoff PH, Colliard M, Berrard E, Kreft C, Dupay A, Bernaille JM, et al. Propranolol in hypertension during pregnancy. Br Med J. 1978; 2:670.

43. Pruyn SC, Phelan JP, Buchanan GC. Long-term propranolol therapy in pregnancy: maternal and fetal outcome. Am J Obstet Gynecol. 1979; 135:485-9.

44. Bott-Kanner G, Schweitzer A, Reisner SH, Joel-Cohen SJ, Rosenfeld JB. Propranolol and hydrallazine in the management of essential hypertension in pregnancy. Br J Obstet Gynaecol. 1980; 87:110-4.

45. Bullock JL, Harris RE, Young R. Treatment of thyrotoxicosis during pregnancy with propranolol. Am J Obstet Gynecol. 1975; 121:242-5.

46. Langer A, Hung CT, McAnulty JA, Harrigan JT, Washington E. Adrenergic blockade. A new approach to hyperthyroidism during pregnancy. Obstet Gynecol. 1974; 44:181-6.

47. Turner GM, Oakley CM, Dixon HG. Management of pregnancy complicated by hypertrophic obstructive cardiomyopathy. Br Med J. 1968; 4:281-4.

48. Oakley GD, McGarry K, Limb DG, Oakley CM. Management of pregnancy in patients with hypertrophic cardiomyopathy. Br Med J. 1979; 1:1749-50.

49. Cottrill CM, McAllister RG Jr, Gettes L, Noonan JA. Propranolol therapy during pregnancy, labor, and delivery: evidence for transplacental drug transfer and impaired neonatal drug disposition. J Pediatr. 1977; 91:812-4.

50. Schroeder JS, Harrison DC. Repeated cardioversion during pregnancy. Treatment of refractory paroxysmal atrial tachycardia during 3 successive pregnancies. Am J Cardiol. 1971; 27:445-6.

51. Dumesic DA, Silverman NH, Tobias S, Golbus MS. Transplacental cardioversion of fetal supraventricular tachycardia with procainamide. N Engl J Med. 1982; 307:1128-31.

52. Wladimiroff JW, Stewart PA. Treatment of fetal cardiac arrhythmias. Br J Hosp Med. 1985; 34:134-40.

53. Kleinman CS, Copel JA, Weinstein EM, Santulli TV Jr, Hobbins JC. Treatment of fetal supraventricular tachyarrhythmias. JCU J Clin Ultrasound. 1985; 13:265-73.

54. Teuscher A, Bossi E, Imhof P, Erb E, Stocker FP, Weber JW. Effect of propranolol on fetal tachycardia in diabetic pregnancy. Am J Cardiol. 1978; 42:304-7.

55. Widerhorn J, Widerhorn AL, Elkayam U. Cardiovascular pharmacotherapy in pregnancy. In: Gleicher N, Gall SA, Sibai BM, Elkayam U, Galbraith RM, Santo GE, eds. Principles and Practice of Medical Therapy in Pregnancy. Norwalk, CT: Appleton & Lange; 1992:767-83.

56. O'Connor PC, Jick H, Hunter JR, Stergachis A, Madsen S. Propranolol and pregnancy outcome (Letter). Lancet. 1981; 2:1168.

57. Tunstall ME. The effect of propranolol on the onset of breathing at birth. Br J Anaesth. 1969; 41:792.

58. Barden TP, Stander RW. Effects of adrenergic blocking agents and catecholamines in human pregnancy. Am J Obstet Gynecol. 1968; 102:226-35.

59. Hogstedt S, Lindeberg S, Axelsson O, Lindmark G, Rane A, Sandstrom B, et al. A prospective controlled trial of metoprolol-hydralazine treatment in hypertension during pregnancy. Acta Obstet Gynecol Scand. 1985; 64:505-10.

60. Sandstrom B. Adrenergic ß-receptor blockers in hypertension of pregnancy. Clin Exp Hypertens. 1982; B1:127-41.

61. Liedholm H. Atenolol in the treatment of hypertension of pregnancy. Drugs. 1983; 25:206-11.

62. Lardoux H, Gerard J, Blazquez G, Chouty F, Flouvat B. Hypertension in pregnancy: evaluation of two ß blockers atenolol and labetalol. Eur Heart J. 1983; 4(Suppl):35-40.

63. Karlberg B, Lundberg D, Aberg H. Excretion of propranolol in human breast milk. Acta Pharmacol Toxicol (Copenh). 1974; 34:222-4.

64. Bauer JH, Pape B, Zajicek J, Groshong T. Propranolol in human plasma and breast milk. Am J Cardiol. 1979; 43:860-62.

65. Liedholm H, Melander A, Bitzeu PO, Helm G, Lonnerholm G, Mattiasson I, et al. Accumulation of atenolol and metoprolol in human breast milk. Eur J Clin Pharmacol. 1981; 20:229-31.

66. Cigarroa JE, Isselbacher EM, DeSanctis RW, Eagle KA. Diagnostic imaging in the evaluation of suspected aortic dissection. Old standards and new directions. N Engl J Med. 1993; 328:35-43.

67. Stoddard MF, Longaker RA, Vuocolo LM, Dawkins PR. Transesophageal echocardiography in the pregnant patient. Am Heart J. 1992; 124:785-7.

68. Pruzinsky MS, Katz NM, Green CE, Salter LF. Isolated descending thoracic aortic aneurysm in Marfan's syndrome. Am J Cardiol. 1988; 61:1159-60.

69. Elster AD. Does MR imaging have any known effects on the developing fetus? Am J Roentgenol. 1994; 162:1493.

70. Beers GL. Biological effects of weak electromagnetic field from 0 Hz to 200 MHz. A survey of the literature with special emphasis on possible magnetic resonance effects. Magn Reson Imaging. 1989; 7:309-31.

71. Elkayam U, Gleicher N. Diagnostic approaches to maternal heart disease. In: Elkayam U, Gleicher N, eds. Cardiac Problems in Pregnancy. New York: Alan R. Liss; 1990:41-5.

72. Eagle KA, DeSanotis R. Diseases of the aorta. In: Braunwald E, ed. Heart Disease. Philadelphia: WB Saunders; 1992:1528-57.

73. Donaldson LB, DeAlverez RP. The Marfan syndrome and pregnancy. Am J Obstet Gynecol. 1965; 62:629-41.

74. Elias S, Berkowitz RL. The Marfan syndrome and pregnancy. Obstet Gynecol. 1976; 47:358-61.

75. Elkayam U, Gleicher N. Hemodynamics and cardiac function during normal pregnancy and the puerperium. In: Elkayam U, Gleicher N, eds. Cardiac Problems in Pregnancy. New York: Alan R. Liss; 1990: 5-24.

76. Irons DW, Pollard KP. Post partum haemorrhage secondary to Marfan's disease of the uterine vasculature. Br J Obstet Gynaecol. 1993; 100:279-81.


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2008 Focused Update Incorporated Into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
J. Am. Coll. Cardiol., September 23, 2008; 52(13): e1 - e142.
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 J. Thorac. Cardiovasc. Surg.Home page
Y. Hayashi, A. D. Cochrane, S. Menahem, and J. A. Smith
Neoaortic root dilatation with saccular aneurysm formation after the arterial switch operation for Taussig-Bing anomaly
J. Thorac. Cardiovasc. Surg., February 1, 2007; 133(2): 569 - 572.
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 J Am Coll CardiolHome page
R. O. Bonow, B. A. Carabello, K. Chatterjee, A. C. de Leon Jr, D. P. Faxon, M. D. Freed, W. H. Gaasch, B. W. Lytle, R. A. Nishimura, P. T. O'Gara, et al.
ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Developed in Collaboration With the Society of Cardiovascular Anesthesiologists Endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons
J. Am. Coll. Cardiol., August 1, 2006; 48(3): e1 - e148.
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 J Am Coll CardiolHome page
R. O. Bonow, B. A. Carabello, K. Chatterjee, A. C. de Leon Jr, D. P. Faxon, M. D. Freed, W. H. Gaasch, B. W. Lytle, R. A. Nishimura, P. T. O'Gara, et al.
ACC/AHA 2006 Practice Guidelines for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Developed in Collaboration With the Society of Cardiovascular Anesthesiologists Endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons
J. Am. Coll. Cardiol., August 1, 2006; 48(3): 598 - 675.
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 Eur. J. Cardiothorac. Surg.Home page
M. Sakaguchi, H. Kitahara, T. Seto, T. Furusawa, D. Fukui, N. Yanagiya, K. Nishimura, and J. Amano
Surgery for acute type A aortic dissection in pregnant patients with Marfan syndrome
Eur. J. Cardiothorac. Surg., August 1, 2005; 28(2): 280 - 283.
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 CirculationHome page
C. A. Nienaber, R. Fattori, R. H. Mehta, B. M. Richartz, A. Evangelista, M. Petzsch, J. V. Cooper, J. L. Januzzi, H. Ince, U. Sechtem, et al.
Gender-Related Differences in Acute Aortic Dissection
Circulation, June 22, 2004; 109(24): 3014 - 3021.
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 HeartHome page
S A Thorne
Pregnancy in heart disease
Heart, April 1, 2004; 90(4): 450 - 456.
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 Crit Care NurseHome page
E. J. Bridges, S. Womble, M. Wallace, and J. McCartney
Hemodynamic Monitoring in High-Risk Obstetrics Patients, I: Expected Hemodynamic Changes in Pregnancy
Crit. Care Nurse, August 1, 2003; 23(4): 53 - 62.
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 Ann. Thorac. Surg.Home page
F. F. Immer, A. G. Bansi, A. S. Immer-Bansi, J. McDougall, K. J. Zehr, H. V. Schaff, and T. P. Carrel
Aortic dissection in pregnancy: analysis of risk factors and outcome
Ann. Thorac. Surg., July 1, 2003; 76(1): 309 - 314.
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 CLIN PEDIATRHome page
C. R. Fikar and S. Koch
Etiologic Factors of Acute Aortic Dissection in Children and Younrg Adults
Clinical Pediatrics, February 1, 2000; 39(2): 71 - 80.
[Abstract] [PDF]

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