646

COARCTATION OF THE AORTAAND PREGNANCY

By D. W. S. GORDON, M.D., F.R.C.S., M.R.C.O.G.Chief Assistant, St. Mary's Hospital; Senior Registrar, Queen Charlotte's Maternity Hospital

and P. S. WATSON, F.R.C.S., M.R.C.O.G.Registrar, St. Mary's Hospital

Pregnancy in a woman suffering from coarctationof the aorta is of especial interest to the obstet-rician for several reasons. Its rarity alone wouldensure this since only 136 cases have so far beenrecorded. It is of further interest in that it is theonly type of heart disease for which many ob-stetricians would advocate an elective Caesareansection in preference to a vaginal delivery.Finally, the increasing scope of vascular surgery,with new techniques for excising and grafting theconstricted aorta, have brought fresh problemswhich need to be considered.

The Incidence of Coarctation of the AortaThe overall estimation for this condition is

about i in 1,500 people (Blackford, 1928), whichmakes its rarity in pregnancy at first surprising.Coarctation is, however, four or five times ascommon in the male (Abbot, 1928; Riefensteinet al., I947) and in addition, irrespective of preg-nancy, these cases may die at an early age, bothof which will obviously decrease its frequency inpregnancy. It is probable though that in thepast the condition has remained undiagnosed inpregnancy and an increasing awareness woulddisclose it more frequently. Thus the first caseto be found in the Queen Charlotte's Hospitalreports was in 1943, then another in 1950 and athird in 1953. At. St. Mary's Hospital the firstcase to be recorded was in I950, since when therehave been another two. The rarity of the con-dition in pregnancy underlines the importance ofreporting these cases when they occur, and thishas been emphasized by Novak (I947).

Finally, the further need for detail is empha-sized by the difficulty in evaluating any line ofmanagement when it is based on such smallfigures.Mortality of Coarctation Alone

This is probably in the region of 15 per cent.,although reported figures show considerablevariation. If death is going to occur, it usuallyhappens before the age of 40. Seventy-four percent. of Maud Abbott's series (1928) of 200

cases and 6I per cent. of 104 patients reported byRiefenstein et al. (I947) died before the fifthdecade. The causes of death in these series were:Aortic rupture, 25 per cent.; bacterial endo-carditis, 25 per cent.; congestive heart failure,25 per cent.; cerebral vascular accidents, io percent.; other causes, 15 per cent.

Resection of the aorta has become increasinglycommon and the operative mortality ranges from2 per cent. (Gross, 1953) to io per cent. (Clelandet al., 1956). It might be noted here that hyper-tension itself is rarely severe and only excep-tionally a cause of death itself. It may, however,be a contributory factor in causing a cerebralvascular accident, especially as congenital 'berry'aneurysms tend to be associated with coarctation.

Hazards of Coarctation in PregnancyGoodwin (1958) reported 136 cases, including

I3 of his own, of which io had been described byCleland et al. in I956. There are a further6 cases reported elsewhere not included in thispaper. Tebow et al. (I954) described 3 casesoperated on during pregnancy, and Keswick andWilson (i954) a further 3, all of which weredelivered vaginally with no mortality. To these6 are added a further 5 of our own, which makesa grand total of I47. In this group there were13 maternal deaths, giving a maternal mortalityof 9 per cent. It would appear that this mortalityhas been steadily decreasing over the years, butthis is probably due to some extent to the factthat many of the early cases were probablyreported solely because of the fatality. The mor-tality related to total number of pregnancies is13 in 367, which is 3.5 per cent. The cause ofdeath in the fatal cases is as follows: Rupture ofthe aorta or dissecting aneurysms, 6; cerebralvascular accidents, 2; congestive heart failure andpulmonary oedema, 3; other causes, 2.

It can be seen that nearly half the deaths werecaused by aortic rupture and it is worth con-sidering whether pregnancy increases this risk.Rupture of the aorta is usually due to precedingmedial necrosis. For example, the fatal case

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reported by Kinney et al. (i945) showed markedchanges in the media proximal to the level ofcoarctation with much loss of elastic tissue. Thisauthor also speculated as to the possible role ofhyperlipaemia, especially hypercholesterolaemia,which occurs to a moderate degree in normalpregnancy. Joule (I949) suggested a possiblethyroid deficiency as being important due to afailure of the gland to meet the excess require-ments demanded during pregnancy. It would beimagined that this weakness would become moreevident when associated with hypertension, butthis has not been a marked feature of those casesthat did actually rupture (Riefenstein et al., I947).

Furthermore, the evidence from Dixon andHartley's cases (i955) is that the blood pressurebehaves very much as in normal pregnancy,dropping in the middle trimester before returningto its former level. The records of 3 of ourcases showed this quite clearly. It might thenbe thought that the lability of the blood pressurecould be the precipitating cause, but althoughthis is a feature of all patients with coarctation itis not particularly evident in pregnancy. Whereasthe effects of pregnancy on a patient sufferingfrom coarctation alone are a matter for debate, ifthere is superadded heart disease then the prog-nosis is definitely worsened. The increased cardiacoutput that occurs in pregnancy will accentuateany failure present, due say to an associatedaortic incompetence. This complication is, how-ever, fortunately rare.One of the cases delivered at Queen Charlotte's

Hospital had a subsequent resection at Hammer-smith Hospital and is included in Goodwin'sseries and will not therefore be described here.

Case ReportsCase i. Mrs. J.R., aged 33, attended Queen

Charlotte's Hospital in i943 for her third preg-nancy. The first, in I929, had been deliveredby forceps and the child weighed 71 lb. In 1935the patient had had a full-term breech deliverybut this child was stillborn and weighed only 4 lb.

Past medical history was uneventful and shehad not complained of any dyspnoea or othersymptoms at her first attendance. Her bloodpressure on this occasion was I85/ioo andexamination of her cardiovascular system showeddefinite cardiac enlargement with a strong impulseand systolic murmurs in both mitral and aorticareas. The femoral pulses were absent. X-rayof her chest showed Roesler's sign on both sidesand screening confirmed the diagnosis of coarc-tation.Her ante-natal attendances were without inci-

dent, although her hypertension persisted to avarying degree throughout the pregnancy. An

external cephalic version was performed withoutan anaesthetic at the 38th week. Delivered atterm normally of a 6 lb. 8 oz. child, first stagelasting 13 hours and the second 10 minutes. Thepuerperium was quite normal.

She was referred to a cardiologist for furtherfollow-up, but failed to keep her appointment.

Case 2. Mrs. B.R., aged 26. This patient'spast medical history was also uneventful. Shehad had one child when aged 20 and this had beendelivered with difficulty by forceps at term andweighed 7 lb. I2 oz. It was noted during thispregnancy that she had a mild hypertension.Her booking blood pressure was I35/80 and roseterminally to i50/i00. It was thought then bythe cardiologist who saw her that she had someform of congenital heart disease, but no furtherdetails were forthcomin.gWhen she attended Queen Charlotte's Hospital

for this pregnancy her blood pressure was notedto be 170/80 and examination of her cardiovascularsystem showed a slight enlargement of the leftventricle with a loud systolic murmur all over thepraecordium. Anastomotic vessels were presenton her back and her femoral pulses were absent.Her ante-natal progress was normal, but

pelvimetry confirmed a degree of outlet contractand she was delivered by an elective lower seg-ment Caesarean section at the 39th week of ahealthy 7-lb. child. Her recovery was uninter-rupted and she was discharged on the i6th post-operative day. Her blood pressure at the follow-upclinic was noted to be 140/110.

Case 3. Mrs. V.W., aged 37. The diagnosisof coarctation of the aorta had been made fiveyears prior to the present pregnancy on thefindings of weak pulses in the left arm and bothfemoral arteries, absent pulsation in poplitealsand arteries of foot, rib notching, a widely con-ducted systolic murmur and evidence of leftventricular hypertrophy. At the time of theoriginal diagnosis she had an infective endarteritis,which responded to Penicillin.

During the ante-natal period of the presentpregnancy the blood pressure was raised in theright arm at nearly all visits, the maximum being170/110. The blood pressure in the left arm wason all occasions below 140/90. The patient wasdelivered at the 39th week by elective lower seg-ment Caesarean section of a living male childweighing 7 lb. The puerperium was uneventful.The only indication for abdominal delivery inthis case was the presence of coarctation of theaorta.

Case 4. Mrs. G.S., aged 25. She was knownto have a ' murmur' as a child. Coarctation ofthe aorta was diagnosed at the age of 19 on thefindings of hypertension (240/95 in the right arm,

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648 POSTGRADUATE MEDICAI, JOURNAL December 1958

FIG. i.-The terylene cloth prosthesis in position.

245/100 in the left arm), absent femoral pulses,apical systolic murmur also audible betweenscapulae, periscapular pulsation. Further investi-gation revealed an aneurysmal dilatation justdistal to the constriction. Her first pregnancy in1950 was terminated because of the coarctationand associated aneurysm. She became pregnantagain in 1955 and first attended the ante-natalclinic. Because of the aneurysmal dilatation of theaorta distal to the coarctation, and in view of thedegree of hypertension, it was decided to operatedespite the pregnancy. The coarctation andaneurysm were excised and continuity in theaorta re-established by a tubular terylene clothprosthesis.The patient had a complete miscarriage 48

hours after operation. She made a good recoveryfrom the operation. Femoral pulses becamepalpable but the hypertension persisted.

During the present pregnancy her blood pres-sure varied between 150/70 and i8o/ioo. Shewas admitted for rest and sedation at the 36th

week because albuminuria and oedema hadappeared. The blood pressure on admission wasI90/I00. The hypertension continued during thenext three weeks, but the albuminuria was presentonly occasionally. Delivery was by elective lowersegment Caesarean section at the 39th week. Itwas noted at this time that her abdominal aortawas well developed although slightly smaller thannormal and pulsating freely. The child was aliveand weighed 9 lb. i oz. The obviously large babyand the presence of an inert graft in the aortawere the chief factors determining the abdominaldelivery.

Case 5. Mrs. J.P., aged 22. At the age of i6the patient attended the medical out-patientdepartment complaining of frontal headaches,blurring of vision and coldness of her lowerextremities. A diagnosis of coarctation of theaorta was made in view of the findings of hyper-tension (80/00oo in the right arm, I60/I00 in theleft arm), absent femoral pulses, apical systolicmurmur, periscapular arterial pulsation and rib

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notching on chest x-ray. In addition, the patienthad moderately well-developed webbing of theneck. She was treated by excision of the affectedsegment of the aorta below the origin of the leftsubclavian artery together with ligation of apatent ductus arteriosis. Continuity in the aortawas obtained by end-to-end suture. She made agood recovery following the operation and theimprovement in her symptoms was maintained.She became normotensive and good arterial pulseswere present in both lower limbs.

During the present pregnancy the ante-natalperiod was uneventful until the 3oth week whenhydramnios occurred. The blood pressure at the9th week was i10/75, and it remained normaluntil the 32nd week when it was found to beI64/Io6 and was associated then with grossgeneralized oedema and albuminuria. She wastreated by bed rest, heavy sedation and saltrestriction. During the next four days her con-dition deteriorated. There was oliguria, increasingalbuminuria to 4 g. per litre, and hypertensionrising to a maximum of I70/125. In view of thisthe pregnancy was terminated at the 32nd weekby lower segment Caesarean section. The childweighed 3 lb. 7 oz., was born alive but died after30 minutes. Delivery was followed by a massivediuresis. The urine became protein-free by the6th week post-partum. The hypertension did notsettle spontaneously and she was eventually ren-dered normotensive by Serpasil 0.75 mg. andInversine 25 mg. daily in divided doses.

DiscussionIt is evident from the weight of reported evi-

dence that pregnancy in patients with coarctationof the aorta can be allowed to continue to termwithout any increase in the mortality rate whichis due to the coarctation alone. The view ex-pressed by Mendelson (1940) that pregnancyshould be terminated if diagnosed early enoughhas not found support in recent years, except inthose occasional cases where there is a seriousassociated cardiac lesion.

Interest has been aroused recently over theproblem of the safest route of delivery for thesepatients. Benham (i949) reviewed the previouslyrecorded cases, added three of his own, andconcluded that delivery should be by Caesareansection in order to avoid the rise of blood pressurein labour, which is agreed to occur by nearly allobservers. He further quotes the observation ofCook and Briggs (1903) that a similar rise mayoccur during instrumental delivery or vaginalmanipulation under anaesthesia. Pritchard (i953)considered that delivery should be by the vaginalroute as he had found a comparable mortality ratewhen comparing abdominal and vaginal routes of

delivery. It must, however, be noted that thesemortality rates were calculated on a very smallnumber of cases with only two deaths in thevaginal, and one in the abdominal series.Dixon and Hartley (I955), as a result of their

observations on two cases, found no evidence ofa rise in blood pressure during delivery, favouredvaginal delivery. Goodwin (i957), reviewing thepreviously reported cases and adding 13 of hisown, favoured vaginal delivery with a shortenedsecond stage, although the only case which hereports in detail was delivered by Caesareansection in two pregnancies.

Operative treatment of coarctation is likely toremove patients from the risk of aortic rupture ordissection, and of congestive cardiac failure.Because of the presence of a healed anastomoticscar or inert graft in the aorta, they are, however,likely to continue to be at risk from endo-aortitis,or endo-carditis where there is an associatedvalvular defect. Also, the presence of congenitalaneurysms of cerebral arteries in association withcoarctation means that surgical treatment of thelatter will not necessarily prevent death fromcerebro-vascular accidents, although reduction ofblood pressure in adolescence will reduce theliability of development, or subsequent ruptureof these aneurysms (Campbell and Baylis, 1956).

Although the evidence is not entirely clear, itmust be a logical assumption that a rise of bloodpressure would be dangerous for patients with anunresected coarctation and to a lesser extent whenresection has been performed. We consider thatit has not been clearly established that a labourwith the second stage shortened by the use offorceps can prevent this rise. One of the casesdescribed by Tebow (I954) which had beentreated by resection showed this rise quite clearly.In addition, the use of forceps to shorten thesecond stage may be a simple procedure, but inprimigravidae this is not always so and may entailmore manipulation and difficulty than first ex-pected. Similarly, the first stage itself may beprotracted and it is not possible to guarantee an' easy' delivery without being ready to perform aCaesarean section rather sooner than is normallyindicated.

If this should have to be done, it would appearthat these patients have got the worst of bothworlds. A further objection to the use of forcepsto shorten the second stage of labour in thesepatients is that strict asepsis cannot be obtainedin vaginal operations and that such a method ofdelivery would, in our view, add to the risk ofendo-aortitis or endocarditis, about which allwriters on this subject are most concerned. Weconsider that Caesarean section, carried out elec-tively, and with the full aseptic technique avail-

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able in an operating theatre, is the optimumroute of delivery for these patients, thereby avoid-ing altogether the possible rise of blood pressurein labour and delivery and lessening the risk ofsubsequent endo-aortitis. This is the view sharedby Benham (I944), Bramwell (I953), and Rosen-thal (I955). The latter writer comments that6 out of the i i cases of pregnancy and coarctationwhich terminated fatally died in labour or nearterm.

While advocating an elective Caesarean sectionfor primigravidae, where the condition is metafter a previous normal vaginal delivery, wesuggest that a further vaginal delivery could beallowed when a short first stage of labour and arapid normal second stage can confidently beexpected. The first two case reports show thiscarried out in practice. The first had had twodeliveries, there was no evidence to suggest anyexpected obstetric difficulty, and the second stagein fact lasted 10 minutes. The second patientgave a history of a difficult forceps delivery,there was some outlet contraction present, so aCaesarean section was decided on.

In addition to this proposed method of delivery,it is obvious that patients with coarctation mustreceive careful ante-natal care, periods of rest ifcardiac symptoms arise, and antibiotic prophy-laxis in the puerperium as advocated by MacLeod(1954)-Two of our cases developed pre-eclamptic

toxaemia in the last trimester of their pregnancies.It is surprising that this added hazard has notbeen noted more frequently in view of the higherincidence of toxaemia that occurs in patientssuffering from essential hypertension. If, as someauthorities suggest, the hypertension of coarcta-tion is a product of renal ischaemia, then thesepatients would be expected to be very liable todevelop pre-eclamptic toxaemia. It is also ofinterest to note that in both these cases thecoarctation had been resected.Although resection during pregnancy has been

described several times, Pritchard (1953) empha-sized the risk tb the foetus from prolonged anoxiaand this is borne out by the history of Case 4,

where abortion occurred 48 hours after operation.Webbing of the neck is a characteristic feature

of the syndrome of gonadal dysplasia describedby Turner (1938) and Albright et al. (1942), andcoarctation is sometimes found as an additionalfeature. Grumbach (1955) described 22 cases ofgonadal dysplasia with 12 showing webbing andcoarctation and found them all to be chromasomalmales, which is another typical feature of thesyndrome. Case 5 where webbing and coarctationwere present and obviously not associated withgonadal dysplasia must therefore be of exceptionalrarity.SummaryThe incidence and hazards of coarctation of

the aorta associated with pregnancy are discussed.Five case reports are presented and the obstetricmanagement described. The conclusion is drawnthat, in general, these patients are best deliveredby an elective Caesarean section.We wish to thank the obstetric surgeons at

St. Mary's Hospital and Queen Charlotte's Hos-pital, under whose care the patients were admitted,for permission to publish the case reports, andProfessor C. G. Rob for operative details ofhis case.

BIBLIOGRAPHYABBOTT, M. E. (I928), Amer. Heart J., 3, 574.ALBRIGHT, F., SMITH, P., and FRASER, R. (1942), Amer. J.

med. Sci., 204, 625.BENHAM, G. H. H. (I949), J. Obstet. Gynaec. Brit. Emp., 54, 9I.CAMPBELL, M., and BAYLIS, J. H. (1956), Brit. Heart J.,

I8, 475.CLELAND, W. P., COUNIHAN, T. B., GOODWIN, J. F., and

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Brit. Emp., 62, 83.GOODWIN, J. F. (I958), Lancet, i, i6.GROSS, R. E. (i953), Circulation, 7, 757.GRUMBACH, M. M., VAN WYCK, J. J., and WILKINS, L.

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Amer. J. med. Sci., 210, 725.MACLEOD, M. (1954), Lancet, ii, 668.NOVAK, E. R. (I947), Amer. J. Obstet. Gynaec., 53, 1054.PRITCHARD, J. A. (1953), Obstet. gynec. Surv., 8, 775.REIFENSTEIN, G. H., LEVINE, S. A., and GROSS, R. E.

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Amer. Surgeon, 20, 1277.''URNER, H. H. (1938), Endocrinology, 23, 566.

Bibliography and Acknowledgments continued from page 644, W. 9. Stanley, M.B., Ch.B.

AcknowledgmentsI wish to thank Dr. A. Thelwall Jones, Dr. G. L.

Manson and Mr. R. W. Doyle for permission topublish the case report, and Professor A. C. P.Campbell and Dr. P. J. D. Snow for their help andadvice on the histology, and Dr. W. K. McGinleyfor permission to quote from his autopsy report.

BIBLIOGRAPHYBRAIN, SIR RUSSELL (1957), Lancet, ii, 857.

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Practice of Medicine'.PAGE, J. H., and McGUBBIN, J. W. (1953), Circ. Res., I, 354SCHMIDT, C. F. (I950), ' The Cerebral Circulation in Health and

Disease', Springfield, Ill.SNOW, P. J. D., LENNARD-JONES, J. F., CURZON, G., and

STACEY, R. S. (I955), Lancet, ii, I004.TAYLOR, R. D., PAGE, I. H., and CORCORAN, A. C. (I951),

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THORSON, A., BIORK, G., BJORKMAN, G., and WALDEN-STROM, J. (1954), Amer. Heart J., 47, 795.

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