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Expected and unexpected cardiac problems

during pregnancy

M aternal heart disease complicates at least 1% ofpregnancies and is one of the most importantcauses of maternal death. In developing countriesrheumatic heart disease remains the major cause of

maternal heart disease, while in developed countriesmaternal congenital heart disease has become moreprevalent due to the improved survival of children withcongenital heart disease. In addition, the prevalence ofcoronary artery disease is rising because of unhealthylifestyle and the tendency to postpone motherhooduntil the third decade.

A report presenting data about maternal mortalityin the UK from 2000 to 2002 showed that themajority of maternal deaths occurred in women withpreviously undiagnosed heart disease.1 The haemo-dynamic burden of pregnancy can unmask previouslyasymptomatic heart disease. For example, it is notuncommon for women with rheumatic mitral stenosis

to be diagnosed for the first time during pregnancybecause the volume overload and increase in heartfrequency cause symptoms that did not occur beforepregnancy. The case reports of peripartum cardio-myopathy and coronary artery dissection in this issueillustrate that life-threatening heart disease can alsodevelop as a new disease related to pregnancy.2-4 Inthe reported cases the events occurred a few weeksbefore delivery or within days to weeks post-partum,but presentation several months after delivery is notuncommon. New cardiac disease can also coincidewith pregnancy by chance as in the case report of awoman with third-degree AV block.5 Both womenwith pre-existing yet undiagnosed heart disease andthose with new (whether or not pregnancy-related)disease are likely to present primarily to obstetricians

or primary caregivers and not to a cardiologist. A timelydiagnosis, therefore, depends on adequate awarenesson the part of their GP or obstetrician. The prevalenceof heart disease in pregnant women and its potentially

life-threatening consequences justify a careful cardiachistory, family history and physical examination in allpregnant women. Recognising heart disease duringpregnancy is challenging because the physiologicalchanges during pregnancy can cause symptoms andsigns mimicking cardiac disease. Examples are fatigue,shortness of breath, oedema and systolic ejection mur-murs. Obstetric caregivers must be able to distinguishsigns and symptoms attributable to normal pregnancyfrom cardiac pathology. Echocardiography and electro-cardiography are the first-choice investigations thatneed to be performed when pathology is suspected.The threshold for referral to a cardiologist should below.

Women with known heart disease before pregnancyneed specialist pre-conception counselling. In fact, therisk of pregnancy must be assessed in teenagers withheart disease once they become sexually active. This isespecially important when the risk for mother and/orfoetus is significantly increased or when pregnancy iscontraindicated. These teenagers need to be advisedabout safe and effective contraception. Combinedhormonal contraceptives contain both oestrogen andprogestagen and are attractive because of their reliablecontraceptive effect. However, oestrogens enhancethrombogenicity which makes them unsuitable forwomen with increased thrombotic risk, even whenthey are on oral anticoagulation therapy. This appliesto women with ischaemic heart disease, women witholder types of mechanical valves and cyanotic women.Progestagen-only methods are suitable for thesewomen. The mini-pill is, however, not recommendedbecause of poor efficacy. Other progestagen-onlymethods such as depo-provera, cerazette and implanonare both effective and safe. The Mirena intrauterinesystem is safe when implanted with endocarditisprophylaxis, although concern has been raised forwomen who would poorly tolerate the vaso-vagalcollapse that may accompany implantation (i.e. womenwith pulmonary vascular disease or Fontan circulation).6

P.G. Pieper

P.G. Pieper

Department of Cardiology, University Medical Center Groningen,

Groningen, the Netherlands

Correspondence to: P.G. Pieper

Department of Cardiology, Thorax Centre, University Medical

Center Groningen, PB 30.001, 9700 RB Groningen,

the Netherlands

E-mail: p.g.pieper@thorax.umcg.nl

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When assessing pregnancy risk, existing risk scores mustbe implemented but also disease-specific risks must betaken into account (figure 1). Data to assist in provid-ing an adequate risk assessment are available from severalpublications.6-8 Contraindications for pregnancy arepulmonary hypertension, severely depressed systemic

ventricular function (EF 40 mm and peripartum cardiomyopathy with residualimpairment of left ventricular function, because in theseconditions maternal mortality is high.6 Maternal risk issignificantly increased in women with cyanotic heartdisease, other complex congenital heart disease includ-ing Fontan circulation and systemic right ventricle, andmechanical prosthetic valves. When assessing maternalrisk, it is important to realise that if several risk factorsare present, these sometimes add up to an unacceptablyhigh risk.6,7

A recent investigation among 116 women visiting ahighly specialised congenital heart disease clinic dem-onstrated that 50% of women with a contraindicationfor pregnancy and over 30% of women with inter-mediate or high pregnancy risk did not recall beinginformed about the increased risk or contraindications.9

This illustrates the importance of clear and repeatedproactive counselling. Although pre-conception coun-selling should ideally start before transfer to adultcardiac services, the first visit to the adult cardiologistis particularly appropriate to inform the young womanabout issues concerning pregnancy. Alternatively, aspecialised nurse could provide this information. Younggirls of 16 to 18 years may not be interested in receiving

the complete information package, but there is aminimum amount of information that should beshared with them during this first visit. This shouldinclude information regarding the risk associated withpregnancy, whether pregnancy is contraindicated aswell as information about the necessity to use safe andeffective contraception. In addition, they should beadvised to visit their cardiologist for full pre-pregnancyinformation before attempting to get pregnant. In duecourse, a full discussion about disease-specific maternaland foetal risks must take place. In congenital heartdisease the inheritance risk should also be discussedand more often than not referral to a geneticist isappropriate.

In all but the heart diseases associated with thelowest risk (such as repaired ASD, VSD or patent duct,small ASD or VSD and mild pulmonary stenosis) thesafety of pregnancy for both mother and foetus willimprove when pregnancy is carefully planned. A fullclinical investigation is indicated before pregnancy.This includes physical examination, oxygen saturation,electrocardiogram, echocardiogram and exercise testing.When systemic ventricular function cannot be accurate-ly assessed echocardiographically, MRI should beperformed. Twenty-four hour electrocardiography maybe useful in women with a high risk of ventricular

arrhythmias. Pregnancy risk assessments can be op-timised using the results of these investigations. Inwomen with aortic dilatation or severe valvular diseasepre-pregnancy surgery must be considered, but thehigh risk associated with pregnancy in women with amechanical valve prosthesis must be weighed againstthe risks associated with native valve disease.10 The casereport of two women with long-QT syndrome andpregnancy-related torsades de pointes illustrates thatin such women pre-pregnancy ICD implantation is asensible policy.11 The impact on foetal well-being of anymedication the woman uses must be appreciated.Although medication doses should be kept as low aspossible and some medication should be avoided, themother-to-be needs to understand that discontinuingnecessary medication will not only negatively affect herbut also her babys health. For example, refusal to useanticoagulation for prevention of mechanical valvethrombosis may result in 200% mortality (mother andbaby). ACE inhibitors, angiotensin II antagonists andstatins are not compatible with pregnancy and needto be stopped or replaced. Beta-blocking agents (pre-

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404 Netherlands Heart Journal, Volume 16, Number 12, December 2008

Figure 1. A pregnant woman with large uncorrected atrial-septaldefect (ASD). Although pregnancy is generally well tolerated in

women with ASD, the risk of thromboembolic complications andarrhythmias is increased (courtesy J.P.M. Hamer).8

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ferably metoprolol), diuretics (except spironolactone)and digoxin are relatively safe. The risks and advantagesof different anticoagulation regimens must be discussedwith women who need anticoagulation and agreementmust be reached between the physician and the womanconcerning the preferable regimen.10 Before pregnancy

a decision should be made regarding the preferred levelof care during pregnancy: predominantly in a localhospital for the most simple lesions, in a specialist car-diology and obstetric clinic for severe, complex andrare heart disease, and shared care between these twooptions in lesions of intermediate complexity.

Substandard care was a contributing factor in 40%of cardiac maternal deaths in the UK maternal mortalityreport.1 Substandard care included poor communi-cation between responsible specialists and lack of clearlydefined management strategies. In order to avoid suchproblems a multidisciplinary team should be availableto attend to women with cardiac disease once pregnancyis established. The minimum team requirement shouldbe a cardiologist, an obstetrician and an anaesthesistwith experience in cardiac disease. Paediatric expertisewill be necessary as well, and the involvement ofspecialised nurses can be valuable. It is advisable toschedule a cardiological and obstetric consultation earlyin pregnancy, and subsequent visits at least eachtrimester but more often in complex or severe disease.Reports from each consultation must be immediatelyprovided. These reports should not only cover adescription of the current clinical situation and therapy,but also an assessment of problems that can beexpected later in pregnancy such as arrhythmias andheart failure and their management. A plan for the

management of delivery must be in place at the end ofthe second trimester.

Education of cardiologists and obstetricians as wellas anaesthesists is necessary to optimise care for

pregnant women with cardiac disease and should bepart of their standard curriculum. Two excellent andup-to-date textbooks are available for practitioners whowant to expand their knowledge about this increasinglyimportant subject.12,13

References

1 Why Mothers Die 2000-2002. Report on Confidential Enquiriesinto Maternal Deaths in the United Kingdom. Royal College ofObstetricians and Gynaecologists; London: 2004.

2 Bosch MGE, van der Voort PHJ, Bams JL, Santema JG. A seriouscomplication in the puerperium: peripartum cardiomyopathy.NethHeart J2008;16:415-8.

3 Oosterom L, de Jonge N, Kirkels JH, Klpping C, Lahpor JR. Leftventricular assist device as a bridge to recovery in a young womanadmitted with peripartum cardiomyopathy. Neth Heart J2008;16:426-8.

4 Van den Branden BJL, Bruggeling WAJ, Corbeij HMA, DunselmanPHJM. Spontaneous coronary artery dissection in the postpartumperiod. Neth Heart J2008; 16:412-4.

5 Tietge W, Danils M. A case of an acquired high-degree AV blockin a pregnant woman. Neth Heart J2008;16:419-21.

6 Thorne S, MacGregor A, Nelson-Piercy C. Risks of contraceptionand pregnancy in heart disease. Heart2006;92:1520-5.

7 Siu SC, Sermer M, Colman JM, Alvarez AN, Mercier LA, MortonBG, et al. Cardiac Disease in Pregnancy (CARPREG) Investigators.Prospective multicenter study of pregnancy outcomes in womenwith heart disease. Circulation2001;104:515-21.

8 Drenthen W, Pieper PG, Roos-Hesselink JW, van Lottum WA,Voors AA, Mulder BJ, et al. Outcome of pregnancy in women withcongenital heart disease: a literature review.J Am Coll Card2007;49:2303-11.

9 Kovacs AH, Harrison JL, Colman JL, Sermer M, Siu SC,Silversides CK. Pregnancy and contraception in congenital heartdisease: what women are not told. J Am Coll Card 2008;52:577-8.

10 Pieper PG, Balci A, van Dijk AP. Pregnancy in women withprosthetic heart valves. Neth Heart J2008;16:406-11.

11 Meregalli PG, Westendorp ICD, Tan HL, Elsman P, Kok WEM,Wilde AAM. Pregnancy and the risk of torsades de pointes in

congenital long-QT syndrome. Neth Heart J2008;16:422-5.12 Oakley C, Warnes CA, editors. Heart Disease in Pregnancy.

Second edition. Blackwell Publishing 2007.13 Steer PJ, Gatzoulis MA, Baker Ph. Heart Disease and Pregnancy.

RCOG Press, London, 2007.

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