Usefulness of percutaneous balloon commissurotomy for mitral stenosis during pregnancy

Inducible multiform ventricular tachycadia in Wolff-Parkinson-White syndrome. Br Heart J 1987:58:89-95. 3. Noh CI, Gillette PC, Case CL, Zeigler VL. Clinical and electrophysiological characteristics of venhicular tachycardia in children with normal hearts. Am Hearr J 1990;120:132~1333. 4. Teo WS, Klein GJ, Guiraudon GM, Yee R, Leitch JW, McLellan D, Leather RA, Kim YH. Multiple accessory pathways in the Wolff-Parkinson-White syndrome

Usefulness of Percutaneous Stenosis During Pregnancy

as a risk factor for ventricular fibrillation. Am J Car&/ 1991:67:889-891. 5. Tomita M, Spinale FG, Crawford FA, Smith A, Zile MR. Changes in left yentricular volume, mass and function during development and regression of supraventricular tachycardia-induced cardiomyopathy: disparity between recovery of systolic vs diastolic function. Circulatron 1991;83:635~. 6. Silka MJ, Kron J, Walance CH, Cutler JE, McAnulty JH. Assessment and follow-up of pediatric survivors of wdden cardiac death. Ci,~u/arion 1990$2:341-349.

Balloon Commissurotomy

for Mitral

Bernard lung, MD, Bertrand Cormier, MD, Joseph Elias, MD, Pierre-Louis Michel, MD, Olivier Nallet, MD, Jean-Marc Porte, MD, Serge Sananes, MD, Serge Uzan, MD, Alec Vahanian, MD, and Jean Acar, MD ince its introduction in 1984, percutaneous mitral commissurotomy(PMC) has steadily gained ground S as an alternative to surgical commissurotomy in the treatment of mitral stenosis.L2 Pregnancy is a promising field for the application of PMC, given that surgery carries a high risk to the fetus, especially when an openheart procedure is required.3 Despite this, experience to date is limited and the few published seriesmainly concentrate on the repercussions of the procedure on the mother. The interest of the present series is that in addition to the efficacy of the procedure it also takes into account fetal tolerance. SinceMarch 1986 we haveper$ormedPMC in 1,142 patients, of whom 13 were pregnant women. These 13 patients form the subject of the present study. Their mean age was 30 f 8 years (range 20 to 44). One had previously undergone closed surgical commissurotomy: 8 patients were in New York Heart Association class III, and 5 in class IV All were in sinus rhythm. After echocardiographic and &oroscopic examination, the morphology of the valves was asfollows: flexible valvesand mild subvalvular disease in 4 patients, Jlexible valves but extensive subvalvular disease in 8, and calc@ed valves in 1. Six patients had mitral regurgitation grade 114and 2 grade 214 as assessedby color JEowDoppler using the transesophageal approach. All patients had single pregnancy and the mean gestational age was 26 f 4 weeks (range 20 to 33). PMC was indicated because of the persistence of incapacitating symptomsdespite medical treatment consisting of bed rest and diuretics in all cases,and p blockers in 5. In 2 patients the procedure had to be undertaken within 12 hours of admission because of refractory pulmonary edema. Oral sedation (diazepam IO mg) was administered each time before the procedure was begun. The patient’s abdomen was wrapped in a lead apron (2 mm thick) from the pubis to the diaphragm. In the last 5 patients, the apron was supported on a frame to decrease discomfort. Two thousand units of heparin were given after transseptal puncture. In the first 4 patients we used a double-balloon technique with a combination of a trefoil balloon (3 X IO mm in diameter, Schneider-Europe) and a conventional balloon (15 or 19 mm in diameter, Schneider-Europe). In the last 9 patients, an lnoue balloon was used with a stepwise dilatation technique From the Cardiology and Obstetric Departments, HBpital Tenon, 4 Rue de la Chine, 75970 Paris, Cedex 20, France. Manuscript received April 16, 1993; revised manuscript received and accepted June 23, 1993.

398

THE AMERICANJOURNALOF CARDIOLOGY VOLUME73

under echo guidance. Hemodynamics were measured wheneverpossible. No cineangiography was peformed, but the leji ventricle was opac$ed underjluoroscopy in the first 7 patients with a total of 40 ml of iopamidol (i.e., 15 g of iodine). Echocardiographic examination using both transthoracic and transesophageal approaches was pelformed on the day preceding PMC and 1 or 2 days after the procedure. Valvearea was determined by planimetry and by the pressure half-time method. The degree of mitral regurgitation and the presence of atria1 shunt were assessedby continuous color flow Doppler using transesophageal examination. In 7 patients it was possible to monitor the fetal cardiac rhythm throughout the procedure. Film-badge dosimeters were used to measure radiation in 8 patients. They were placed between the skin and the lead apron on both the anterior and posterior sides of the abdomen and calibrated to measure doses higher than 0.2 millisieverts (mSv). Results were expressedas a mean + SD. Quantitative data before and after PMC were compared using Wilcoxon’s test. The results were analyzed by a statistical software program (CSS Statistica Statso8 Inc). No technical failure occurred. The mean JEuoroscopy time was 15 + 9 minutes {range 5 to 37). PMC brought immediate hemodynamic improvement (Table I). There were no major complications. Small atria1 shunts were detected by transesophageal color Jlow Doppler in 8 patients. Transient hypotensive episodes due to a vagal reaction were observed in 3 patients at the beginning of PMC and were successfully treated by raising the lead apron, administering atropine and fluids. Uterine contractions began in 1 procedure but were rapidly countered by spasmolytics.No lasting change in fetal heart rate was ever noted. In 3 patients, minor changesdid occur but subsided spontaneously: a loss of variability without deceleration in 2, and loss of variability with transient deceleration in 1. These mild changes occurred during the episodes of maternal hypotension previously described. No modifcation of fetal heart rate was noted during balloon inflation. Abdominal radiation was always co.2 mSv, which is the threshold of the dosimeters.After the procedure, all patients improved clinically to New York Heart Association class I or II. Two patients were still pregnant and free of symptoms1 month after PMC. In 10 cases,delivery was at term (mean gestational duration 38 f 2 weeks [range 36 to 42]), and the newborn babies were normotrophic (mean body weight 2.9 + 0.7 kg [range 2.7 to 3.71) and were normal on examina-

FEBRUARY15,1994

TABLE I

Hemodynamic

and Echocardiographic

Results

Before and After Percutaneous

Mitral

Commissurotomy Valve Area lcm2)

MPAP (mm Hg)

LAP (mm Hg)

Patient

Before

After

Before

1 2 3 4 5 6 7 8 9 10 11 12 13

55 38 63 50 50 31 65 38 40 33 42

20 51 27 23 21 46 28 29 14 33

45 22 27 28 30 26 31 22 23 15 22 29

29 k 12

22 + 7

Mean

462

12

p = 0.005

After

Cl (Limlnlm2)

Mean Gradient (mm Hg)

Before

After

Before

4 5 3 20 13 10 9 10 9 22

2.8 3.3 2.6 4.0 4.0 2.7 2.8 2.6 2.2 3.3 4.8

3.2 3.3 4.5

4.0 2.7 2.3 7.3

24 31 23 29 27 18 17 18 19 20 23 27

6 8 16 9 7 5 6 4 9

10~ 6

3.2 ~0.8

3.4 + 0.8

23 k 5

8 + 4

-

p = 0.005

3.7

p = 0.20

Cl = cardiac Index; LAP = left atrlal pressure; MPAP = mean pulmonary

After -

Hemcdynamic Before

After

0.9 0.9 0.7 1.3 1.1 0.8 0.9 1.0 0.7

2.0 1.9 1.8 1.8 1.9

1.0 1.4

2.3 2.2

1.0 kO.2

2.0 + 0.2

p = 0.01

artery pressure: MR = mftral regurgltatton

p = 0.03

MR Doppler

2D-Echo Before

After

Before

After

1.0 0.8 0.9 1.1 1.6 0.8 1.0 1.1 0.7 1.1 0.8 1.0 0.7

2.1 2.1 2.1 2.0 2.6 1.8 1.6 1.9 1.7 1.9 2.0 1.8 1.7

2 0 0 1 1 0 2 0 1 1 0 1 1

2 1 0 1 2 1 2 1 1 2 1 1 1

1.0 + 0.2

1.9 + 0.3

p = 0.002

(graded O/4 to 414); 2D = 2.dimensional.

I

tion. Spontaneousvaginal delivery occurred in 5 cases and there were 5 cases of cesarean section (3 for obstetric reasons, and 2 as a precautionary measure). In I patient, cesarean section was necessary at 29 weeks of pregnancy, 3 months after PMC, because of natural membrane rupture; the fetus, which was properly formed, died 12 hours after delivery. On an averagefollow-up of 12 k 6 months (range 1 to 24) after delivery, all patients remainedsymptom-free, were off all medication, and the children were developing normally. In patients with mitral stenosis, physiologic changes lead to a great increase in pulmonary pressure, which is poorly tolerated particularly at the time of labor and delivery.4 Despite medical treatment, some patients remain symptomatic and require additional therapy. Open-heart commissurotomy during pregnancy carries a high risk of fetal mortality ranging from 20 to 30%3; it may also be a cause of fetal distress.5 Closed mitral commissurotomy is safer but fetal mortality may be as high as 12%.6 The efficacy of PMC in the treatment of mitral stenosis is now well established and it has progressively replaced closed commissurotomy in many centers. However, its use in pregnant women has remained limited (the number of published cases is now 46 in 10 reports, only 1 of which was >lO patients).7p’6 In all these reports, as well as in our own, most patients were severely symptomatic, in New York Heart Association class III or IV, with concomitant pulmonary hypertension. PMC was performed in the third trimester except in 6 cases when it took place between the 17th and the 19th weeks.7J2J3,‘5,‘6Proof of the efficacy of PMC in pregnant women has clearly been shown by remarkable improvements in functional status, decrease in pulmonary pressure, and increase in valve area.‘m’(j Such good results can reasonably be expected in young women with favorable anatomy. One could argue that in the particular circumstances of pregnancy, patients with less favorable anatomy can also be accepted for the procedure, since the primary aim is less to achieve optimal results

than to allow favorable progress of pregnancy up to normal delivery. In the series so far reported, maternal complications have been limited to a single case of cerebrovascular embolism in a patient who had not undergone transesophageal examination before PMC.14 It remains t&e, however, that the numbers so far concerned are small and that there is always an inherent risk in PMC, such as hemopericardium or mitral regurgitation, leading to open-heart surgery with its consequent risk to the mother and the fetus. To limit such risk, the procedure should only be performed by experienced interventional cardiologists. In the present series, as was also the case in the series of Ribeiro et al,‘” transient hypotension occurred in a few patients at the beginning of the procedure; this may have been due to a number of factors: change to a supine position where the uterus may compress the vena cava, the weight of the lead apron, and a vagal reaction induced by the vascular puncture. In this series only minor abnormalities of fetal heart rate were noted; they were too small to have been caused by fetal distress and occurred during the episode of maternal hypotension previously mentioned. Monitoring of fetal heart rate was performed in only 2 of the series previously cited,11*13and showed transient bradycardia. In one case where a double-balloon technique was used,” this occurred during balloon inflation. Inflation with this technique is known to induce a longer period of hypotension than with the moue technique, because inflation/deflation takes longer, and because of the presence of the guide wires. The improvement in hemodynamics resulting from PMC generally enables pregnancy to follow a normal course. Only 2 neonatal fatalities have been reported; both occurred >I month after PMC and in particular obstetric circumstances: 1 patient had a gemellar pregnancy” and 1 a history of premature birth.9 The single neonatal fatality in our own series was likewise due to an obstetric factor. The objection usually raised against PMC during pregnancy is the risk of radiation to the fetus.” Again, we have very few data to BRIEFREPORTS 399

go on becausepublished reports provide specific data in this respectin only 2 series.11J2In both, as was our own experience, the amount of radiation measuredwas very low, 0.1 mSv” and 0.5 mSv,12and far lower than that legally admitted for pregnant women subject to radiation exposure (i.e., 5 mSv).rs We have as yet no real certainty with regard to long-term effects,although good indication can be derived from the fact that the 20-year follow-up of 1,000 cases of women irradiated for radiopelvimetry at dosesof 15 to 30 mSv at the samestage of pregnancy,revealed no difference in the incidence of cancer with a control group.19Nonetheless,even if the risk of radiation is low, all the following steps are essential to keep radiation to minimum: shielding of the abdomenby a lead apron, shortening the proceduretime, using fluoroscopy sparingly, and avoiding cineangiography. The last potential complication for the fetus is subsequentthyroid dysfunction resulting from the injection of contrast medium. However, this has not been observed in the caseof dosessimilar to those we administered.20This risk can be avoided by using color Doppler flow imaging instead of cineangiography to evaluate any change in the degree of mitral regurgitation. PMC during pregnancy is thus shown to be efficacious in terms of improving the mother’s hemodynamic status; it is also well tolerated by the fetus. Nonetheless, larger series with longer follow-up are needed to conlirm these findings, particularly with regard to risks and potential long-term side effects of radiation to the fetus. The results obtained in this series suggest that PMC is an attractive procedure for pregnant women who remain symptomatic despite medical therapy.

1. Inoue K, Okawi T. Nakamura T, Kitamura F, Miyamoto N. Clinical application of tramvenous mitral commissurotomy by a new balloon catheter. J Thorax Cardiowsc Sur,q 1984;87:394-402.

2. Vahanian A, Michel PL, Cornier B, Vitoux B,.Michel X, Slama M, Entiquez Sarano L, Trabelsi S, Ben lsmail M, Acar J. Results of percutaneous mitral commissurotomy in 200 patients. Am J Card& 1989;63:847-852. 3. Bemal JM, Mimlles PJ. Cardiac surgery with cardiopulmonary bypass during pregnancy. Obst Gynecol Surv 1986;41:14. 4. Clark SL, Phelan JP, Greenspeon J, Aldahl D, Horenstein J. Labor and delivety in the presence of mitral stenosis: central hemodynamic observations. Am J Obster Gynecol 1985;152:984-988. 5. Lamb Ml’, Ross K, Johnstone AM, Manners JM. Fetal heat monitoring during open heart surgery. Two case reports. Br J Obstet Gynecol 1981;88:66%674. 6. Vosloo S, Reichart 9. The feasibility of closed mitral valvotomy in pregnancy. J Thorac Cardiovasc Surg 1987;93:6%679. 7. Safian RD, Berman AD, Sachs B, Diver DJ, Come PC, Bairn DS, McKay L, Grossman W, McKay RG. Percutaneous balloon mitral valvuloplasty in a pregnant woman with mitral stenosis. Cathet Cardiovasc Diqn 1988;15: 103-108. 8. Palacios IF, Block PC, Wilkins CT, Red&r DE, Daggett WM. Percutaneous mitral balloon valvotomy during pregnancy in a woman with severe mitral stenosis. Cather Cardiovasc Diqn 1988; 15: 109-l 11. 9. Mangione JA, Zuliani MF, Del Castillo JM, Nogueira EA. Arie S. Percutaneous double balloon mitral valvuloplasty in pregnant women. Am J Cardiol 1989; 64:9%102. 10. Smith R, Brenda D, McCredie M. Percutaneous transluminal balloon dilatation of mitral valve in pregnancy. Br Heart J 1989;61:551-553. 11. Esteves CA, Ramos AIO, Braga SLN, Harrison JK, Sousa JE. Effectiveness of percutaneous balloon mitral valvotomy during pregnancy. Am J Cardiol 1991: 68:93&934. 12. Drobinski G, Fraboulet P, Montalescot G, Moussallem N, Coutte R, Artigou JY, Grosgogeat Y. Valvuloplastie mitmle au quatri&me mois de grossesse. F’rotection foetale par un mat&au de plomb. Arch Mal Coeur 199 1;84:249-25 1. 13. Gangbar EW, Watson KR, Howard RJ, Chisholm RJ. Mittal balloon valvuloplasty in pregnancy: advantages of a unique balloon. Cathet Cardiovmc Diqn 1992:25:313-316. 14. Ruzyllo W, Dabrowski M, Woroszylska M, Rydlewska-Sadowska W. Percutaneous mitral commissurotomy with the Inoue balloon for severe mitral stenosis during pregnancy. J Heart Valve Dis 1992; 1:209-2 12. 15. Ben Farhat M, Maatouk F, Betbout F, Ayari M, Brahim H, Souissi M, Sghairi K, Gamra H. Percutaneous balloon mitral valvuloplasty in eight pregnant women with severe mitral stenosis. Eur Heart J 1992;13:1658-1664. 16. Rib&o PA, Fawzi ME, Awad M, Dunn B, Dunn CG. Balloon valvotomy for pregnant patients with severe pliable mitral stenosis using the Inoue technique with total abdominal and pelvic shielding. Am Heart J 1992; 124: 1558-1562. 17. Rib&o PA, Al Zaibag M. Editorial - mitral balloon valvotomy in pregnancy. J Heart I/a/w Dis 1992; 1:20&208. lt3. Wagner LK, Hayman LA. Pregnancy and women radiologists. Radiology 1982: 145:55%562. 19. Griem ML, Meier P, Dobben GD. Analysis of the morbidity and mortality of children irradiated in fetal life. Radiology 1967;88:347-349. 20. Giraud JR, DuguC-Ma&haud M, Fizazi T, De Tounis H, Auben J. Urographie intraveineuse pendant la grossesse et risque d’hypothyroidie foetale. J Gyn Obst Biol Repr 1983;12:4549.

Congenitally Bicuspid Aortic Valve in Multiple Family Members Brian N. Glick, MD,* and William C. Roberts, MDt he congenitally bicuspid aortic valve has been the subject of numerous publications. Leonardo da Vinci T in the 1400sdrew the valve and provided some reasons

not until the 1950sthat the bicuspid condition was recognized to be a common underlying cause of aortic valve stenosis,and it was not until 1981that it was recwhy it might function abnormally.’ In 1844, Paget ognized to be associatedwith pure aortic regurgitation called attention to the peculiar liability of congenitally unassociatedwith infective endocarditis.5The bicuspid bicuspid aortic valves to disease,but their importance as aortic valve has been the subject, directly or indirectly, something other than a pathologic curiosity was not ap- of 228 publications from this laboratory since 1962.5,c32 preciated. In 1858, Peacock3pointed out that bicuspid Despite the frequency of the congenitally bicuspid aortic valvestendedto become“thick . . . ossilled . . . induc- aortic valve (possibly as high as 1% of the population), ing first obstruction. . . and then incompetency.”Oslefl in its occurrencein >l family memberis extremely uncom1886 lirst described the abnormal liability of the bicus- mon. Only 4 reports have appeareddescribing 4 famipid aortic valve to develop infective endocarditis. It was lies (9 family members)with >l member having a congenitally bicuspid aortic valve (Table I).33-36During the past 27 years, we have encountered6 families (17 famFrom the Pathology Branch, National Heart, Lung, and Blood Instiily members)in whom >l member had aortic valve distute,National Institutes of Health, Bethesda, Maryland 20892. Manuscript received April 16, 1993; revised manuscript received and accepted ease, with bicuspid valve confirmed at operation in 11. June 24, 1993. A description of certain tindings in these 17 family *Cardiology Fellow, Georgetown University Medical Center, membersis the subject of this report. Washington, D.C. The pedigree of the 6 families is shown in Figure 1. Kurrent address: Baylor Cardiovascular Institute, Baylor University Medical Center, 3600 Gaston Avenue, Dallas, Texas 75246. In 3 families (I, II and VI), a parent and 21 child were 400

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FEBRUARY15, 1994