This chapter will cover the most important cardiac conditions relevant to pregnancy. 1 Nelson-Piercy C (2007). Cardiac disease. In: Lewis G (ed). The Con \201 dential Enquiry into Maternal and Child Health (CEMACH). Saving mothers \200\231 lives: reviewing maternal deaths to make motherhood safer 2003 \200\2232005. The Seventh Report on Con \201 dential Enquiries into Maternal Deaths in the UK. London: CEMACH. This page intentionally left blank 676 CHAPTER 15 Heart disease in pregnancy Physiological changes in pregnancy Cardiac output increases early in pregnancy, reaching a maximum by the mid-second trimester. This is achieved by an increase in both stroke volume and heart rate. There is peripheral vasodilation, and a fall in systemic and pulmonary vasc ular resistance. Although there is no increase in pulmonary capillary wedge pressure (PCWP), serum colloid o smotic pressure is reduced. The colloid oncotic pressure \200\223PCWP gradient is reduced b y 28 % , making pregnant women particularly susceptible to pulmonary oedema. Pulmonary oede ma will be precipitated if there is either an increase in cardiac preload (such as infusion of \202 uids), or increased pulmonary capillary permeability (such as in pre-eclampsia), or both. In late pregnancy, in the supine position, pressure of the gravid uterus on the inferior ve na cava (IVC) causes a reduction in venous return to the heart and a consequent fall in str oke volume and cardiac output. Turning from the lateral to the supine position may result i n a 25 % reduction in cardiac output. Pregnant women should therefore be nursed in the left or right lateral position wherever possible. If the mother has to be kept on her back, the pelvis should be rotated so that the uterus drops forward, and cardiac output as well as u teroplacental blood \202 ow are optimized. Reduced cardiac output is associated with redu ction in uterine blood \202 ow and therefore in placental perfusion; this can compromise the fetus. Labor is associated with further increases in cardiac output (15 % in the \201 rst stage and 50 % in the second stage). Uterine contractions lead to auto transfusion of 300 \200 \223500 mL of blood back into the circulation, and the sympathetic response to pain and anx iety further elevates heart rate and blood pressure. Cardiac output is increased more durin g contractions but also between contractions. Following delivery, there is an immediate rise in cardiac output due to the relief of IVC o bstruction, and contraction of the uterus that empties blood into the systemic circulation. Cardiac output increases by 60 \200\22380 % , followed by a rapid decline to pre-labor val ues within about one hour of delivery. Transfer of \202 uid from the extravascular space increases venous return and stroke volume further. Those women with cardiovascular compromi se are therefore most at risk of pulmonary oedema during the second stage of labor and the immediate post-partum period. See Fig. 15.1. PHYSIOLOGICAL CHANGES IN PREGNANCY 677 Physiological changes in the cardiovascular system in pregnancy i Cardiac output 40 % i Stroke volume i Heart rate 10 \200\22320 beats per minute d Blood pressure First and second trimester l Third trimester l Central venous pressure l Pulmonary capillary wedge pressure (PCWP) d Systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR) 25 \200\22330 % d Serum colloid osmotic pressure 10 \200\22315 % ) n m i ) l ) l m m / l ( ) m p b ( ( ( t u p t u o c a t r d a r e a H C 3750 90 90 8 e m u o v e m u o v l e t a r l a m s a l P e k o r t S i Plasma volume 3500 3250 80 6 3000 80 2750 70 Heart rate Cardiac output and stroke volume 2500 70 60 4 4 Non- pregnant 2250 8 12 16 20 24 28 32 36 Post- delivery Gestation (weeks) Fig. 15.1 Physiological changes in pregnancy. Systemic and pulmonary vascular resistance fa ll during pregnancy. Blood pressure may fall in the second trimester, rising slightly in la te pregnancy. Note the cardiac output and stroke volume peak by 16 weeks \200\231 gestation . Reproduced with permission from Thorne SA (2004). Pregnancy in heart disease. Heart 90 : 450 \200\2236. 678 CHAPTER 15 Heart disease in pregnancy Normal \201 ndings in pregnancy On examination Findings may include: \200 bounding/coll apsing pulse \200 ejection systolic murmur (present in over 90 % pregnant women; may be quite loud, and audible all over the praecordium) third heart sound \200 relative sinus tachycardia \200 ectopics \200 peripheral oedem a. On electrocardiogaphy (ECG) These are partly related to changes in the position of the hear t: \200 atrial and ventricular ectopics \200 Q wave (small) and inverted T wave in lead III \200 ST-segment depression and T-wave inversion inferior and lateral leads \200 QR S axis leftward shift. Investigations \200 The amount of radiation received by the fetus during a maternal chest X-ray (CXR) is negligible and CXRs should never be withheld if clinically indicated i n pregnancy. Transthoracic and transoesophageal echocardiograms are also safe, with the usual precautions to avoid aspiration. Magnetic resonance imaging (MRI) is safe in pregnancy. \200 Routine investigation with el ectrophysiological studies and angiography is normally postponed until after pregnancy but should not be withheld in, for example, acute coronary syndromes. This page intentionally left blank 680 CHAPTER 15 Heart disease in pregnancy General considerations in pregnancy The heart has relatively less reserve than the respirat ory system. Women with heart disease may not be able to increase their cardiac output adequ ately to cope with pregnancy and delivery. The outcome and safety of pregnancy are related to the: presence and severity of pulmonary hypertension \200 presence of cyanosis \200 haemodyn amic signi \201 cance of the lesion \200 functional class as determined by the level of activity that leads to dyspnoea (New York Heart Association, NYHA). 1 Cardiac events such as stroke, arrhythmia, pulmonary oedema, and death complicating pregnan cies are predicted by: 2 \200 a prior cardiac event or arrhythmia \200 NYHA classi \201 cation > II \200 cyanosis \200 left ventricular ejection fraction (LVEF) <40 % \200 left heart obstruction (mitral valve area <2 cm 2 , aortic valve area <1.5 cm 2 , aortic valve gradient > 30 mmHg). Women with congenital heart disease are at increased risk of having a baby with congenital heart disease, and should therefore be offered detailed scanning for fetal cardiac anomalie s. Women with cyanosis (oxygen saturation <80 \200\22385 % ) have an increased risk of fetal g rowth restriction, fetal loss, and thromboembolism secondary to the reactive polycythaemia. Their chance of a live birth in one study was less than 20 % . 3 Women with the above risk factors for adverse cardiac or obstetric events should be managed and counseled by a multidisciplinary team, including cardiologists with expertise in pregn ancy, obstetricians, fetal medicine specialists, and pediatricians. Regular antenatal visit s and judicious monitoring to avoid or treat expediently any anemia or infection or cardiac decompensation are essential. There should be early involvement of obstetric anesthetists and a carefully documented plan for delivery. 1 McCaffrey FM, Sherman FS (1995). Pregnancy and congenital heart disease: The Magee Women \200\231s Hospital. J Mat Fet Med 4 : 152 \200\2239. 2 Siu SC, Sermer M, Colman JM, et al . (2001). Prospective multicenter study of pregnancy outcomes in women with heart disease. C irculation 104 : 515 \200\22321. 3 Presbitero P, Somerville J, Stone S, et al. (1994). Preg nancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation 89 : 2 673 \200\2236. This page intentionally left blank 682 CHAPTER 15 Heart disease in pregnancy Pulmonary hypertension and pregnancy Pulmonary vascular disease, whether secondary to a rev ersed large left-to- right shunt such as a ventral septal defect (VSD; Eisenmenger \200\231 s syndrome) or to lung or connective tissue disease (e.g. scleroderma), or due to primary p ulmonary hypertension, is extremely dangerous in pregnancy. Women known to have pulmonary v ascular disease should be advised from an early age to avoid pregnancy and be given appropr iate contraceptive advice. Maternal mortality is 25 \200\22340 % . 1 , 2 Most fatalities oc cur in the early puerperium. The danger relates to \201 xed pulmonary vascular resistance and an inability to increase pulmonary blood \202 ow with refractory hypox- aemia. Most deaths can be attributed to thromboembolism, hypovolaemia or pre-eclampsia. Pulmonary hypertension is de \201 ned as a non-pregnant elevation of mean (not systolic) p ulmonary artery pressure equal to or greater than 25 mmHg at rest or 30 mmHg on exercise in the absence of a left-to- right shunt. Pulmonary artery systolic (not mean) pressure is us ually estimated by using Doppler ultrasound to measure the regurgitant jet velocity across the tricuspid valve. This should be considered a screening test. There is no agreed relatio n between the mean pulmonary pressure and the estimated systolic pulmonary pressure. If the systolic pulmonary pressure estimated by Doppler is thought to indicate pulmonary hyperten sion, a specialist cardiac opinion is recommended. If there is pulmonary hypertension in th e presence of a left-to-right shunt, the diagnosis of pulmonary vascular disease is particu larly dif \201 cult and further investigation including cardiac catheterization to calcula te PVR is likely to be necessary. Pulmonary hypertension as de \201 ned by Doppler studies may also occur in mitral stenosis and with large left to right shunts that have not revers ed and, although such women may not have pulmonary vascular disease and a \201 xed PVR (o r this may not have been established prior to pregnancy), they have the potential to develo p it, and require very careful monitoring with serial echocardiograms. Management \200 In the event of unplanned pregnancy, a therapeutic termination should be offered. 3 Elective termination carries a 7 % risk of mortality, hence the importance of av oiding pregnancy if possible. If such advice is declined, multidisciplinary care, elective admission for bed rest, oxygen , and thromboprophylaxis are recommended. Therapies such as sildena \201 l and bosentan sh ould be continued if they have led to reductions in pulmonary pressures, even though the la tter is teratogenic in animals. There is no evidence that abdominal or vaginal delivery or regional versus general anesthesia improve outcome in pregnant women with pulmonary hypertension. 1 Yentis SM, Steer PJ, Plaat F (1998). Eisenmenger \200\231s syndrome in pregnancy: materna l and fetal mortality in the 1990s. Br J Obstet Gynaecol 105 : 921 \200\2232. 2 B dard E, Dimopoulos K, Gatzoulis MA (2009). Has there been any progress made on pregnancy outcomes a mong women with pulmonary arterial hypertension? Eur Heart J 30 : 256 \200\22365. 3 Thorne SA, Nelson-Piercy C, MacGregor A (2006). Risks of contraception and pregnancy in heart dise ase. Heart 92 : 1520 \200\2235. MARFAN SYNDROME AND PREGNANCY 683 Marfan syndrome and pregnancy Eighty per cent of Marfan patients have some cardiac involvem ent, most commonly mitral valve prolapse and regurgitation. Pregnancy increases the risk of aortic rupture or dissection, usually in the third trimester or early post-partum. Progres sive aortic root dilation and an aortic root dimension > 4 cm are associated with increased risk (10 % ). 1 Those with aortic roots > 4.6 cm should be advised to delay pregnancy unti l after aortic root repair. 2 Conversely, in women with minimal cardiac involve- ment and a n aortic root <4 cm, pregnancy outcome is usually good, 2 although those with a family hist ory of aortic dissection or sudden death are also at increased risk. Management \200 Monthly echocardiograms. \200 B -blockers for those with hypertension o r aortic root dilation. \200 Vaginal delivery for those with stable aortic root measureme nts but elective cesarean section with regional anesthesia if there is an enlarged or dilat ing aortic root. 1 , 2 1 Immer FF, Bansi AG, Immer-Bansi AS, et al . (2003). Aortic dissection in pregnancy: analy sis of risk factors and outcome. Ann Thorac Surg 76 : 309 \200\22314. 2 Lipscomb KJ, Clayto n Smith J, Clarke B, et al . (1997). Outcome of pregnancy in women with Marfan \200\231s sy ndrome. Br J Obstet Gynaecol 104 : 201 \200\2236. 684 CHAPTER 15 Heart disease in pregnancy Valvular heart disease in pregnancy Valvular heart disease affects 71 % of pregnancies and may be associated with an increased risk of adverse maternal, fetal, and neonatal outcomes. High-risk features include: \200 impaired left ventricular (LV function (ejection fracti on (EF)<40 % ) \200 left-sided valve stenoses 1 (aortic stenosis (AS) with valve area <1. 5 cm 2 or mitral stenosis (MS) with valve area <2.0 cm 2 ) previous maternal cardiovascular system (CVS) event (congestive cardiac failure (CCF), transient ischaemic attack (TIA), cerebrovascular accidient (CVA)), or symptoms * (NYHA class II or higher). Risk increases with each additive factor, see box opp osite. Associated with neonatal complications (preterm delivery, respiratory distress, fetal growt h retardation (FGR), intraventricular hemorrhage, death). VALVULAR HEART DISEASE IN PREGNANCY 685 Classi \201 cation of valvular heart disease risk in pregnancy Low maternal and fetal risk \200 Asymptomatic AS with mean gradient <50 mmHg and normal L V function Aortic regurgitation (AR), NYHA class I/II and normal LV function \200 Mitral regurgitati on (MR), NYHA class I/II and normal LV \200 MV prolapse with no MR or with mild \200\223m oderate MR and normal LV \200 Mild \200\223moderate mitral stenosis (MS) (mitral valve (M V) area > 1.5 cm 2 , gradient <5 mmHg), no severe pulmonary hypertension Mild \200\223moderate pulmonic stenosis (PS) High maternal and fetal risk \200 Severe AS with or without symptoms \200 AR and NYHA class III or IV symptoms \200 MS with NYHA cl ass II or higher \200 MR with NYHA class III or IV symptoms \200 Aortic valve (AV) dise ase, MV disease, or both, resulting in severe pulmonary hypertension (pulmonary artery (PA) pressure > 75 % systemic pressure) AV disease, MV disease, or both, with LV dysfunction (LVEF<40 % ) \200 Maternal cyanosis \200 Reduced functional status (NYHA class III or IV) High maternal risk \200 Impaired LV systolic function (LVEF <40 % ) \200 Previous heart failure \200 Previous CVA or TIA High neonatal risk \200 Maternal age <20 or > 35 years \200 Use of warfarin therapy th roughout pregnancy \200 Smoking during pregnancy \200 Multiple gestations Adapted from Reimold SC, Rutherford JD (2003). Valvular heart disease in pregnancy. N Engl J Med 349 : 52 \200\2239, with permission. 686 CHAPTER 15 Heart disease in pregnancy Mitral stenosis and pregnancy This is important in pregnancy because although asymptomatic at preg- nancy onset, women may deteriorate secondary to tachycardia, arrhyth- mias, or the increased cardiac output. The commonest complication is pulmonary oedema secondary to incr eased left atrial pressure, and pre- cipitated by increased heart rate or increased volume (such as occurs during the third stage of labor or following injudicious intravenous \202 uid therapy). 1 The risk is increased with severe MS (MV area <1 cm 2 ), mod- erate or sev ere symptoms prior to pregnancy, and in those diagnosed late in pregnancy. 1 Management \200 Women with severe MS should be advised to delay pregnancy until after val votomy, or, if the valve is not amenable to valvotomy, until after MV replacement. B -blockers decrease heart rate, increase diastolic \201 lling time, 2 and decrease the risk of pulmonary oedema. 1 \200 Diuretics should be continued in pregnancy. \200 If medical therapy fails, or for those with severe MS, balloon mitral valvotomy may be safely and successfully used in pregnancy if the valve is suitable. 1,2 Pe rcutaneous balloon valvotomy carries a risk of major complications of about 1 % , whereas f or surgical valvotomy the \201 gures are: closed valvotomy \200\224 fetal mortality 5 \200\22315 % , maternal 3 % , open valvotomy \200\224 fetal mortality 15 \200\22333 % , ma ternal 5 % . Women with MS should avoid the supine and lithotomy positions as much as possible for labor and delivery. Fluid overload must be avoided, and even in the presence of oliguria, withou t signi \201 cant blood loss, the temptation to give intravenous \202 uids must be resis ted. Pulmonary oedema, if it occurs, should be treated in the usual way with oxygen and diu retics, and introduction or reintroduction of a B-blocker may be useful to slow the heart r ate. 1 Desai DK, Adanlawo M, Naidoo DP, et al . (2000). Mitral stenosis in pregnancy: a four-yea r experience at King Edward VIII Hospital. Br J Obstet Gynaecol 107 : 953 \200\2238. 2 Tsia ras S, Poppas A (2009) Mitral valve disease in pregnancy: outcomes and management. Obstet M ed 2 : 6 \200\22310. OTHER VALVE LESIONS 687 Other valve lesions Mitral regurgitation Usually due to MV prolapse. Well tolerated as syst emic vascular resist- ance is low in pregnancy. LV function is important in assessing risk (normal function carries a good prognosis). Aortic stenosis Usually congenital. If severe/symptomatic, advise deferring pregnancy until surgically corrected. If already pregnant and symptomatic early on, consider termination. Surgical replacement/balloon valvuloplasty are both associated with signi \201 cant risks. Aortic regurgitation Usually well tolerated as reduced systemic vascular resistance in preg - nancy reduces the regurgitant volume. Vasodilators/diuretics are usually suf \201 cient treatment (stop angiotensin-converting enzyme inhibitor (ACE-I) and replace with nifedipine , hydralazine, etc.). 688 CHAPTER 15 Heart disease in pregnancy Mechanical heart valves in pregnancy The optimal management of women with metal heart valve replacements in pregnancy is controversial, since the interests of the mother and the fetu s are in con \202 ict. These women require lifelong anticoagulation and this must be conti nued in pregnancy because of the increased risk of thrombosis. Warfarin is associated with warfarin embryopathy if given between 6 and 12 weeks \200\231 gestation, 1 and increased ri sks of miscarriage, stillbirth, and fetal intracerebral hemorrhage. 2 There is some evidenc e that the adverse effects of warfarin are related to the dose required to maintain the int ernational normalized ratio (INR) > 2, with doses in excess of 5 mg being associated with h igher risks of teratogenesis, miscarriage and, stillbirth. 3 Heparin and low molecular weig ht heparin, even in full antico- agulant doses, are associated with increased risks of valv e thrombosis and embolic events. 1 \200\2234 Management There are three basic options: \200 continue warfarin throughout pregnancy, st opping only for delivery; this is the safest option for the mother 1,2 replace the warfarin with high-dose unfractionated or low-molecular weight heparin from 6 to 12 weeks \200\231 gestation to avoid warfarin embryopathy use high-dose unfractionated or low-molecular weight heparin throughout pregnancy. 4 Which option is chosen will depend on several factors: \200 the type of mechanical valve \200\224 the risk of thrombosis is less with the newer bilea \202 et valves (e.g. CarboMe dics) than with the \201 rst- generation ball and cage (e.g. Starr \200\223Edwards), or s econd-generation single tilting disc (e.g. Bjork \200\223Shiley) valves the position of the valve replacement \200\224 valves in the aortic rather than the mitral position, are associated with a lower risk of thrombosis 5 the number of mechanical valves \200 the dose of warfarin required to maintain a therapeu tic INR 3 \200 any previous history of embolic events. Whichever management option is cho sen, warfarin should be discon- tinued and substituted for heparin for 10 days prior to del ivery, to allow clearance of warfarin from the fetal circulation. For delivery itself, hepa rin therapy is interrupted. Warfarin is recommenced 3 \200\2235 days post-partum. In the ev ent of bleeding, or the need for urgent delivery in a fully antico- agulated patient, warfa rin may be reversed with fresh frozen plasma (FFP) 1 Chan WS, Anand S, Ginsberg JS (2000). Anticoagulation of pregnant women with mechanical h eart valves. Arch Intern Med 160 : 191 \200\2236. 2 Sadler L, McCowan L, White H, et al. (2 000). Pregnancy outcomes and cardiac complications in women with mechanical, bioprosthetic and homograft valves. Br J Obs Gynaecol 107 : 245 \200\22353. 3 Cotrufo M, De Feo M, De San to L, et al . (2002). Risk of warfarin during pregnancy with mechanical valve prostheses. O bstet Gynecol 99 : 35 \200\22340. 4 Oran B, Lee-Parritz A, Ansell J (2004) Low molecular we ight heparin for the prophylaxis of thromboembolism in women with prosthetic mechanical hea rt valves during pregnancy. Thromb Haemost 92 : 747 \200\22351. 5 Elkayam U (1999). Pregnan cy through a prosthetic heart valve. J Am Coll Cardiol 33 : 1642 \200\2235. MECHANICAL HEART VALVES IN PREGNANCY 689 and vitamin K, and heparin with protamine sulfate. Vitamin K should be avoided if possible, since it renders the woman extremely dif \201 cult to anticoagulate with warfarin after d elivery. Anticoagulation in pregnancy \200 Low molecular weight heparin (LMWH): Effective anticoag ulant. Does not cross placenta. Good bioavailability following subcutaneous administration. Easy w eight-based dosing schedule. Thrombocytopenia and osteoporosis are extremely rare. Side-eff ects : maternal hemorrhage, wound haematoma, local allergic reaction at injection site. Unfractionated heparin: Rarely used since LMWHs have become the anticoagulants of choice in pregnancy. Use in pregnancy is mainly intravenous and con \201 ned to acute management of massive pulmonary emboli and in the peri-partum period it is used for rapid control (and r eversal) of anticoagulation in case emergency delivery is required. Not effective for use w ith mechanical valves in pregnancy. Side-effects : maternal hemorrhage, thrombocytopenia (m onitor full blood count (FBC)), osteoporosis, alopecia. Warfarin: Effective oral anticoagulant. Crosses the placenta. Increases risk of miscarriage and stillbirth. Studies show it to be more effective than unfractionated heparin at preven ting valve thrombosis in pregnancy. Side-effects : fetal and maternal hemorrhage, teratogen ic in \201 rst trimester therefore avoid during this period if at all possible (use LMWH) . Aspirin: Used in low dose (75 mg) as adjunctive therapy with LMWH for pregnant women with p rosthetic valves and also in other high-risk patients (atrial \201 brillation (AF), LV dy sfunction, previous emboli). 690 CHAPTER 15 Heart disease in pregnancy Ischaemic heart disease The risk factors for? change to acute coronary syndrome (ACS) MI in pregnancy are the same as for the non-pregnant individual. The risk is increased in multig ravid women and in those who smoke, and women with diabetes, obesity, hypertension, and hyp ercholesterolaemia. 1 Infarction most commonly occurs in the third trimester and affects th e anterior wall of the heart. 1 The maternal death rate is about 10 % . In pregnancy, aetio lo- gies other than atherosclerosis are more likely (such as coronary artery thrombosis or dissection) than in the non-pregnant woman. 2 Management \200 Management of ACS is as for the non-pregnant woman. \200 Angiography sh ould not be withheld if clinically indicated. \200 Intravenous and intracoronary thrombol ysis and percutaneous transluminal coronary angioplasty and stenting have all been successf ully performed in pregnancy. Both aspirin and B-blockers are safe in pregnancy. \200 Clopidogrel also seems to be safe , although it should be discontinued prior to delivery because of the increased bleeding risk. Glycoprotein IIb/IIIa inhibitors should be avoided although there are case reports of their successful use. Statins should be discontinued prior to pregnancy as they are associated with an increased risk of malformations. 1 Ladner, HE, Danielsen B, Gilbert WM (2005). Acute myocardial infarction in pregnancy and the puerperium: a population-based study. Obstet Gynecol 105 : 480 \200\2234. 2 Roth A, Elk ayam U (1996). Acute myocardial infarction associated with pregnancy. Ann Intern Med 125 : 751 \200\2237. HYPERTROPHIC CARDIOMYOPATHY 691 Hypertrophic cardiomyopathy The danger of hypertrophic cardiomyopathy (HCM) in pregnancy re lates to LVOT obstruction that may be precipitated by hypotension or hypo- volaemia. Provid ed these are avoided, pregnancy is usually well tolerated. Management \200 B -blockers should be continued in pregnancy or initiated for symptomatic women. Epidural anesthesia/analgesia carries the risk of vasodilatation and hypotension, with cons equent increased LVOT obstruction. Any hypovolaemia will have the same effect and should be rapidly and adequately corrected. 692 CHAPTER 15 Heart disease in pregnancy Peripartum cardiomyopathy This pregnancy-speci \201 c condition is de \201 ned as the dev elopment of cardiac failure between the last month of pregnancy and 5 months post-partum, i n the absence of an identi \201 able cause or recognizable heart disease prior to the last month of pregnancy, and LV systolic dysfunction. The diagnosis should be suspected in the puerperal patient with breathlessness, tachy- cardia, or signs of heart failure. It is con \201 rmed with echocardiography. Echocardiographic criteria for peripartum LV dysfunction: 1 \200 LVEF<45 % \200 Fractio nal shortening <30 % \200 LVEDP (left ventricular end-diastolic pressure) > 2.7 cm/m 2 \200 Often, echocardiography shows the heart is enlarged with global dilatation of all fou r chambers and markedly reduced LV function. Risk factors include: \200 multiple pregnancy \200 hypertension (be it pre-existing or related to pregnancy or pre- eclampsia) \200 multiparity \200 increased age \200 African-Caribbean race. Management Treatment is as for other causes of heart failure, with: \200 oxygen \200 di uretics \200 vasodilators \200 cardioselective B -blockers \200 ACE-Is if post-partum \200 inotropes if required \200 LV assist devices \200 heart transplantation. About 50 % of women make a spontaneous and full recovery. Most case fatalities occur close to pre sentation. Recent data show a 5-year survival of 94 % . 2 Prognosis and recurrence depend o n the normalization of LV size within 6 months of delivery. 3 Those women with severe myoca rdial dysfunction, de \201 ned as LV end-diastolic dimension (cid:116)6cm and fractional s hortening (cid:100)21 % , are unlikely to regain normal cardiac function on follow- up. 4 T hose whose LV function and size do not return to normal within 6 months and prior to a subs equent pregnancy are at signi \201 cant risk of 1 Pearson GD, Veille JC, Rahimtoola S, et al . (2000). Peripartum Cardiomyopathy. National Heart, Lung and Blood Institute and Of \201 ce of Rare Diseases (NIH). Workshop Recommenda tions and Review. JAMA 283 : 1183 \200\22388. 2 Felker GM, Thompson RE, Hare JM, et al . (2 000). Underlying causes and long-term survival in patients with initially unexplained cardi omyopathy. N Engl J Med 342 : 1077 \200\22384. 3 Elkayam U, Tummala PP, Rao K, et al. (2001 ). Maternal and fetal outcomes of subsequent pregnancies in women with peripartum cardiomyo pathy. N Engl J Med 344 : 1567 \200\22371. 4 Witlin AG, Mabie WC, Sibai BM (1997). Peripart um cardiomyopathy: a longitudinal echocar- diographic study. Am J Obstet Gynecol 177 : 1129 \200\22332. PERIPARTUM CARDIOMYOPATHY 693 worsening heart failure (50 % ) and death (25 % ) or recurrent peripartum cardiomyopathy in the next pregnancy. They should therefore be advised against pregnancy. 5 Those who do rec over normal LV size and function may still be at risk of recurrent heart failure in a subse quent pregnancy, either because of a new episode of cardiomyopathy or because they have imp aired contractile reserve. To exclude the latter, a stress ECHO is advis- able prior to any future pregnancy. 6 5 Sliwa K, Fett J, Elkayam U (2006) Peripartum cardiomyopathy. Lancet 368 : 687 \200\22393. 6 Nelson-Piercy C (2006) Cardiomyopathy and pregnancy. In Steer P, Gatzoulis M, Baker P (e ds). Cardiac Disease in Pregnancy . London: RCOG Press, pp. 231 \200\22342. 694 CHAPTER 15 Heart disease in pregnancy Arrhythmias in pregnancy Atrial and ventricular premature complexes (APC, VPC) are common i n pregnancy. Many pregnant women are symptomatic from forceful heart beats that occur follo wing a compensatory pause after a VPC. Most women with symptomatic episodes of dizziness, s yncope, and palpitations do not have arrhythmias. 1 A sinus tachycardia requires investigation for possible underlying pathology such as: \200 blood loss \200 infection \200 heart failure \200 thyrotoxi cosis \200 pulmonary embolus. The commonest arrhythmia encountered in pregnancy is suprav entricular tachycardia (SVT). First onset of SVT (both accessory pathway-mediated and AV no dal re-entrant) is rare in pregnancy, but 22 % of 63 women with SVT had exacerbation of sym ptoms in pregnancy; 2 50 % of SVTs do not respond to vagal maneuvers. Management \200 Propranolol, verapamil, and adenosine have Food and Drug Administration (FDA) approval for acute termination of SVT. Adenosine has advantages over v erapamil, including probable lack of placental transfer, and may be safely used in pregnanc y for SVTs that do not respond to vagal stimulation. 3 , 4 Metoprolol is short acting and may be used to prevent or reduce symptomatic SVTs in pregnancy. Flecainide is safe and is also used in the treatment of feta l tachycardias. Propafenone and amiodarone should be avoided, 5 the latter because of interference with fetal thyroid function. Temporary and permanent pacing, cardioversion, and implantable de \201 brillators are also safe in pregnancy. 3,4 1 Lee SH, Chen SA, Wu TJ, et al . (1995). Effects of pregnancy on \201 rst onset and symp toms of paroxysmal supraventricular tachycardia. Am J Cardiol 76 : 675 \200\2238. 2 Page RL (1995). Treatment of arrhythmias during pregnancy Am Heart 130 : 871 \200\2236. 3 Mason BA , Ricci-Goodman J, Koos BJ (1992). Adenosine in the treatment of maternal paroxysmal suprav entricular tachycardia. Obstet Gynecol 80 : 478 \200\22380. 4 Adamson D, Nelson-Piercy C (2 007). Palpitations and arrhythmias in pregnancy. Heart 93 : 1630 \200\2236. 5 Magee LA, Dow nar E, Sermer M, et al. (1995). Pregnancy outcome after gestational exposure to amiodarone. Am J Obstet Gynecol 172 : 1307 \200\22311. This page intentionally left blank 696 CHAPTER 15 Heart disease in pregnancy Cardiac arrest in pregnancy This should be managed according to the same protocols as used in the non-pregnant woman, with two very important additions: \200 pregnant women (especi ally those in advanced pregnancy) should be \200\230wedged \200\231 to relieve any obstruc tion to venous return from pressure of the gravid uterus on the IVC. This can be most rapid ly achieved by turning the patient into the left lateral position. If cardiopulmonary resus citation (CPR) is required, then the pelvis can be tilted while keeping the torso \202 at to allow external chest compressions. The most senior member of the obstetric team should be summoned. This is to ensure that obs tetric causes of the collapse are considered (e.g. amniotic \202 uid embolism, massive po st-partum hemorrhage) and appropriately treated. In addition, emergency cesarean section ma y be required to aid maternal resuscitation. Endocarditis prophylaxis \200 Current UK recommendations from the National Institute for Health and Clinical Excellence (NICE) 2008 are that antibiotic prophylaxis against infectiv e endocarditis (IE) is not required for childbirth. The British Society for Antimicrobial Chemotherapy (BSAC) 2006, and the American Heart Asso ciation (AHA) have recommended cover only for patients deemed to be at high risk of develop ing IE (such as women with previous IE) and for those who have the poorest outcome if they develop IE (such as those with cyanotic congenital heart disease). \200 Fatal cases of en docarditis in pregnancy have occurred antenatally, rather than as a consequence of infectio n acquired at the time of delivery. If antibiotic prophylaxis is used for delivery it shoul d be: \200 amoxicillin 2 g IV plus gentamicin 1.5 mg/kg IV at the onset of labor or ruptu red membranes, or prior to cesarean section, followed by amoxicillin 500 mg orally (or IM/I V depending on the patient \200\231s condition) 6 hours later for women who are allergic to penicillin, vancomycin 1 g IV or teicoplanin 400 mg IV may be used instead of amoxicillin. 697 Chapter 16 Eponymous syndromes Syndromes are listed in alphabetical order 698 \200\223708 698 CHAPTER 16 Eponymous syndromes A \200\223C Aase syndrome A clinical triad of congenital anemia, triphalangeal thumbs, and VSD. The etiology is unknown. Adams \200\223Stokes syncope See Stokes \200\223Adams syndrome (b p. 706). Al \201 di \200\231s syndrome Hypertension resulting from occlusion of the coeliac axis, l eading to diversion of collateral blood \202 ow from the right renal artery. Originally d escribed as renal-splanchnic steal syndrome. 1 Andersen syndrome A triad of periodic paralysis, ventricular tachy- arrythmias, and dysmorp hic features (hypertelorism, micrognathia, low-set ears, and high arched or cleft palate, s hort stature, scoliosis, syndactyly, and clinodactyly). The periodic paralysis can be assoc iated with hyper-, hypo-, or normokalaemia. It is an autosomal dominant condition associate d with mutations in the KCNJ2 gene encoding the inward-rectifying K + channel Kir2.1. 2 Anderson \200\223Fabry disease A rare X-linked recessive lysosomal storage disorder involvi ng a de \201 ciency of the enzyme alpha galactosidase There is a resulting in accumulation of globotriasylceramide throughout the body. Cardiac manifestations include left and right ventricular hypertrophy and heart failure. Other systemic features include renal impairmen t, angiokeratomas, neuropathy, and corneal keratopathy. Treatment is now available in the f orm of enzyme-replacement therapy. Barlow \200\231s syndrome A familial form of mitral valve prolapse which is sometimes inher ited as an autosomal dominant trait. It is a genetically heterogenous syndrome, characteriz ed by \200\230billowing \200\231 of one or both of the mitral valve lea \202 ets into the left atrium during systole. On auscultation there is a midsystolic click and a late or pan systolic murmur. 20 % are asymptomatic. Females are twice as commonly affected. 3 Barth syndrome An X-linked mutation of the TAZ gene, leading to dilated cardiomyopathy, ske letal myopathy, short stature and neutropenia. 3-methylglutaconic acid excretion in the uri ne has been observed in almost all reported cases. Beemer lethal malformation syndrome A lethal syndrome of double outlet right ventricle, hyd rocephalus, dense bones, thrombocytopenia, and abnormal nasal development. 1 Al \201 di RJ, Filson EJ, Frohlich ED, Gifford RW Jr, Meaney TF (1967). Renal-splanchnic steal. Report of a case. Cleveland Clin Q 34: 43 \200\22354. 2 Donaldson MR, Jensen JL, Tr istani-Firouzi, M, et al. (2003). PIP2 binding residues of Kir2.1 are common targets of mut ations causing Andersen syndrome. Neurology 60(11): 1811 \200\22316. 3 Barlow J, Marchand B P, Pocock WA, Denny D (1963). The signi \201 cance of late systolic murmurs. Am Heart J 66 : 443. A \200\223C 699 Bland \200\223Garland \200\223White syndrome (or ALCAPA) A congenital condition featuring a nomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA) resu lting in myocardial ischaemia. Affected infants typically present with congestive heart fai lure within the \201 rst 1 \200\2232 months of life. De \201 nitive treatment involves s urgical re-implantation of the left coronary artery or bypass grafting. 1 Bouillaud \200\231s syndrome An eponym for rheumatic fever. Bouillaud was the \201 rst to emphasize the importance of cardiac involvement in the acute articular phase of rheumatic fever. 2 Bourneville \200\223Pringle disease Hamartomas of the heart and kidney associated with epil epsy, learning dif \201 culties, cerebral cortical hamartomas (tuberose sclerosis), and ad enoma sebaceum. It is inherited in an autosomal dominant manner. Renal cysts or carcinomas may occur. 3 Bradbury \200\223Egglseton syndrome An idiopathic disorder of autonomic failure characteriz ed by orthostatic hypotension, with more widespread manifestations of thermoregulatory, bow el, bladder, and sexual function disturbance. Brugada \200\231s syndrome One of the principal causes of sudden cardiac death in young adu lts in the absence of structural heart disease, secondary to mutation of the SCN5A gene on chromosome 3, inherited in an autosomal dominant fashion. This results in malfunction of a sodium channel leading to initiation and perpetuation of ventricular arrhythmias. Clinicall y there is right bundle branch block, ST elevation in V 1 to V 3 , and sudden death/syncope . The clinical phenotype may be unmasked by the administration of ajmaline (not available i n the UK) or procainamide. The only effective treatment is with an implantable cardiac de \201 brillator. 4 Carney syndrome Also known as the Carney complex. There is association of atrial myxomas wi th myxomas in other locations, e.g. breast or skin, spotty pigmentation, and endocrine over activity, e.g. pituitary or testicular tumors. The inheritance is autosomal dominant, the m utation being in the PRKAR1A gene on chromosome 17. It tends to affect individuals in their third decade. They are more likely to have bilateral myxomas and develop recurrences of th e myxoma after removal, in contrast to sporadic cases. 1 Bland EF (1933). Congenital anomalies of the coronary arteries: report of an unusual case associ- ated with cardiac hypertrophy. Am Heart J 8 : 787 \200\223801. 2 Bouillaud B (1832 ). Trait clinique du rheumatisme articulaire. Paris. 3 Bourneville DM (1880). Scl rose t ub reuse des circonvolution c r brales: Idiotie et pilepsie hemipl gique. Archives de neurologie Paris 1 : 81 \200\22391. 4 Brugada P, Brugada J (1992). Right bundle branch blo ck, persistent ST segment elevation and sudden cardiac death: a distinct clinical and elect rocardiographic syndrome. A multicenter report. J Am Coll Cardiol 20 (6): 1391 \200\2236. 700 CHAPTER 16 Eponymous syndromes D \200\223M DiGeorge syndrome A disorder resulting from deletion of the TBX1 gene on chromo some 22q11.2, leading to parathyroid hypoplasia (and hypocalcaemia), thymic hypoplasia (and low T-cell counts), and out \202 ow tract defects of the heart, including tetralogy of Fa llot, truncus ateriosus, interrupted aortic arch, right-sided aortic arch, and aberrant rig ht subclavian artery. Affected individuals typically have micrognathia, low-set ears, short philtrum, and small mouth. The Shprintzen syndrome is also caused by a disorder in the sam e gene.