Heart Disease in Pregnancy - part 6 ppt

arterial bed and decreased coronary flow reserve may contribute to these symp- toms. Autonomic dysfunction may also occur, resulting in inappropriate vasodilatation. A subset of patients have dynamic left ventricular outflow tract obstruction, which may be responsible for symptoms 3,14 (Figure 13.2). This is caused by se- vere hypertrophy of the basal septum projecting into the left ventricular out- flow tract and secondary systolic anterior motion of the mitral valve. The abnormal mitral valve motion is produced either by a ‘Venturi effect’ from an increase in velocity sucking the mitral valve into the outflow tract or by ‘drag forces’ pushing redundant mitral valve leaflets into the outflow tract. 15 Sec- ondary mitral regurgitation may be present from distortion of the mitral valve apparatus and contribute further to symptoms. The mitral valve apparatus is almost always abnormal in these patients, with anterior displacement of the Hypertrophic cardiomyopathy and pregnancy 175 Figure 13.2 Schematic diagram of the left ventricle in hypertrophic cardiomyopathy during systole. There is projection of the basal septum into the outflow tract with systolic anterior motion of the mitral valve, which results in left ventricular outflow tract obstruction. The obstruction is dynamic, dependent on the preload, afterload and contractility of the heart. By permission of the Mayo Foundation for Medical Education and Research. papillary muscles, which position the mitral valve leaflets so as to contribute further to this outflow tract obstruction. 16 It is the young patients with severe outflow tract obstruction who are at highest risk for hemodynamic deteriora- tion during pregnancy. Diagnosis of hypertrophic cardiomyopathy in patients of child-bearing age As young patients with hypertrophic cardiomyopathy are symptom free, the diagnosis of hypertrophic cardiomyopathy is primarily made through family screening or after a routine medical examination when a murmur is heard on auscultation or an abnormal ECG is found. Hypertrophic cardiomyopathy is sometimes first recognized during pregnancy when systolic murmurs lead to cardiologic referral. As the murmur of left ventricular outflow tract obstruction is dynamic, maneuvers such as squat to stand should be performed. In patients with hypertrophic cardiomyopathy, the length and intensity of a late systolic ejection murmur should significantly increase upon assuming the standing position. The ECG is almost always abnormal in patients beyond the first decade of life with hypertrophic cardiomyopathy (Figure 13.3). 17,18 It often shows features of atrial and ventricular hypertrophy that are associated with marked ST- and T-wave abnormalities. Echocardiography should always be performed in pa- 176 Chapter 13 Figure 13.3 A 12-lead ECG from a patient with hypertrophic cardiomyopathy. There is left ventricular hypertrophy present. There is high voltage as well as secondary ST–T- wave abnormalities. There is left atrial enlargement. Hypertrophic cardiomyopathy and pregnancy 177 tients with an abnormal ECG to look for the increase in left ventricular wall thickening that is considered the ‘gold standard’ of diagnosis when present (Figure 13.4). However, in some patients with an abnormal ECG, it may not identify hypertrophy, especially if it is localized to an abnormal location such as the apex. In a situation where there is significant unexplained abnormality of the ECG, repeat echocardiography with contrast enhancement or magnetic reso- nance imaging (MRI) should be performed. The presence or absence of left ventricular outflow tract obstruction should be documented both by examination and by echocardiography. Systolic anterior motion of the mitral valve with a late peaking, high-velocity Doppler signal in the left ventricular outflow tract is diagnostic of dynamic left ventricu- lar outflow tract obstruction (Figure 13.5). The magnitude of the obstruction is determined by the peak Doppler velocity (Figure 13.5 shows a velocity of more than 6 m/sec) and should be measured at rest and during provocation (after a premature ventricular contraction, during the strain phase of the Val- salva maneuver or during inhalation of amyl nitrite). Secondary mitral regurgitation, characterized by a posteriorly directed jet, should be identified and semi-quantified. Genetics and family screening Hypertrophic cardiomyopathy is usually the result of a missense mutation in the sarcomeric protein genes and transmitted in an autosomal dominant manner. There are over 200 mutations that have been identified in 10 different sarcomeric genes. 19 Thus, once the diagnosis of hypertrophic cardiomyopathy has been made, all patients should undergo genetic counseling and family screening. This should be performed even if the patient is completely asymptomatic. All first-degree relatives of the proband should be screened with physical examination, an ECG and echocardiography. In adolescence, screening should be performed every year throughout their growth spurt. In adults, screening should be performed every 5 years because hypertrophy may appear late in patients with certain gene mutations. In the future, genetic analysis should aid in helping screen the presence or absence of hypertrophic disease in first-degree relatives of patients with documented hypertrophic cardiomyopathy. Although initial data suggested that certain specific mutations may predispose to sudden death or have a benign course, 20 the accuracy of this prediction has been challenged with studies of unre- lated hypertrophic cardiomyopathy patients that demonstrated that specific malig- nant or benign mutations are rare and clinical outcomes cannot be successfully predicted. 21,22 In women who wish to become pregnant, genetic counseling is essential. There is a 50% chance that the child may have hypertrophic cardiomyopathy. If hypertrophic cardiomyopathy becomes manifest in the very early childhood years, severe disease occurs and the prognosis may be dismal. 23 The utility of prenatal ultrasound screening is controversial. In the future prenatal molecular diagnosis may be performed. 24 178 Chapter 13 (a) (b) Figure 13.4 A two-dimensional echocardiogram from a patient with hypertrophic cardiomyopathy. There is an increase in left ventricular wall thickness, with a greater increase in thickness of the ventricular septum. (a): parasternal long-axis view during diastole. (b): parasternal long-axis view during systole. Hypertrophic cardiomyopathy and pregnancy 179 (c) (d) Figure 13.4 Continued (c): short axis, diastole. (d): short axis, systole. There is systolic anterior motion of the mitral valve causing left ventricular outflow tract obstruction. Risks in pregnancy The risks in pregnancy are related either to hemodynamic deterioration or ar- rhythmias and sudden death. Most young woman with hypertrophic cardiomy- opathy will go through pregnancy without difficulty. 25–29 The increased blood volume and stroke volume of pregnancy are beneficial for the dynamic left ven- tricular outflow tract obstruction. Most women who have no or mild symptoms before the pregnancy will not develop more symptoms during the preg- nancy course. There are some who become more dyspneic as a result of the larger blood volume but this can be controlled with low-dose diuretics. In patients who have moderate to severe symptoms before the pregnancy, symptoms will worsen in 10–30% of patients, especially if there is pre-existing left ventricular outflow tract obstruction. The higher the left ventricular outflow tract gradient, the more likely that there will be progressive symptoms. 25 It is the subset of patients with very severe obstruction (gradient >100 mmHg) who are at highest risk of hemodynamic deterioration during pregnancy and delivery. Sudden death or resuscitated ventricular fibrillation is unusual but has been re- ported to occur in patients with hypertrophic cardiomyopathy during pregnan- cy. 25,30–33 In an Italian series of 100 women with hypertrophic cardiomyopathy, there were two pregnancy-related arrhythmic deaths. 25 The maternal mortality rate was 10 per 1000 live births and was higher than the expected mortality rate in the Italian population (relative risk 17.1, 95% CI 2.0–61.8). However, the ab- 180 Chapter 13 200 mmHg 0 mmHg 6 m/s Ao Lv Figure 13.5 Simultaneous left ventricular (LV) and aortic (Ao) pressure from catheterization with continuous wave Doppler of the LV outflow tract. The peak velocity of 6 m/sec by Doppler corresponds to the gradient of 150 mmHg by catheterization. solute risk of death was low and each of the two patients who died had significant risk factors for sudden cardiac death: one had severe hypertrophy and severe out- flow tract obstruction (>115mmHg) and the other a highly malignant family his- tory of hypertrophic cardiomyopathy (five young relatives died suddenly). Management in pregnancy Even though the outcome is usually favorable, some patients will develop symptoms for the first time during pregnancy, or pre-existing symptoms will get worse. When symptoms are present, beta blockade should be started Table 13.1. The dosage of beta blocker should be titrated to attain a resting heart rate less than 70 beats/min (bpm). Beta blockers have the potential to cause growth re- tardation, low Apgar scores or neonatal hypoglycemia in the infants but this is rare. 21,34 Breast-feeding is not contraindicated but atenolol, acebutolol, nadolol and sotalol are secreted in breast milk in larger amounts than the other beta blockers. Verapamil is also safe for use in pregnancy if beta blockers are not tol- erated, but can cause hemodynamic deterioration and sudden death if started in a patient with a severe resting left ventricular outflow tract obstruction. When verapamil is initiated in patients with severe left ventricular obstruction, this should be done under monitored conditions in hospital. Low-dose diuretics may be useful if patients develop pulmonary congestive symptoms during the pregnancy as a result of the volume overload. However, care must be taken not to cause too much of a reduction in preload because this may exacerbate the left ventricular outflow tract obstruction. Periodic bedrest in the left lateral decubitus position is recommended for all patients with hyper- trophic cardiomyopathy and even mild symptoms. In patients who have severe symptoms and severe outflow tract obstruction, septal reduction therapy is recommended before proceeding with pregnancy. Septal myectomy is considered the ‘gold standard’ for symptomatic patients who have persistent symptoms despite optimal medical therapy. 35 In young healthy women, the risk of the operation in experienced centers is less than 1%. 36 Septal myectomy relieves the gradient and produces an excellent reduction in symptoms. Surgical myectomy has been performed during pregnancy in the rare patient with a large outflow gradient who develops severe intractable symptoms during pregnancy. Hypertrophic cardiomyopathy and pregnancy 181 Table 13.1 General recommendations for management of patients with hypertrophic cardiomyopathy during pregnancy Document degree of left ventricular outflow tract (LVOT) obstruction and risk stratification Risk stratification for sudden death Beta blockade for symptoms Avoid decrease in preload (dehydration, over diuresis) Avoid inotropes (dopamine or dobutamine) and vasodilators (nifedipine) In the hypotensive patient balance fluids and vasoconstrictor Septal ablation has been considered an alternative to septal myectomy in pa- tients with hypertrophic cardiomyopathy and left ventricular outflow tract ob- struction. 37 However, the long-term effect of this procedure is unknown. It is not known whether or not creating a large infarct in patients already at risk of ventricular arrhythmias may enhance the propensity to develop dangerous ar- rhythmias or cause adverse ventricular remodeling with time. 38 Therefore, in women of child-bearing age, septal myectomy has been considered the proce- dure of choice in our institution (Mayo Clinic)to which such rare patients are referred. Arrhythmias are uncommon in patients with hypertrophic cardiomyopathy undergoing pregnancy. 25,26 Should atrial fibrillation or flutter occur, cardiover- sion should be considered if there is hemodynamic compromise as a result of the loss of atrial contraction and fast ventricular response. Beta blockers are usu- ally the drug of choice in preventing further episodes, although low-dose amio- darone can be used if recurrent episodes are present. Amiodarone has been used safely in pregnancy. Fetal hypothyroidism may occur so that a neonatal assess- ment after delivery is warranted but no congenital abnormalities have been noted. Aggressive risk stratification to determine those at risk for sudden death should be performed on all patients with hypertrophic cardiomyopathy. The major risk factors that predict sudden death include previous out-of-hospital arrest or documented sustained ventricular tachycardia, and a strong family history of hypertrophic cardiomyopathy with sudden death. Other ‘minor’ risk factors for the occurrence of sudden death include severe hypertrophy (thick- ness >3 cm), non-sustained ventricular tachycardia on Holter monitoring, a drop in blood pressure with exercise and perfusion defects on MRI. If multiple risk factors are present, implantation of an automatic defibrillator has been rec- ommended. 6 Successful shocks with automated defibrillators have been re- ported in pregnant patients with hypertrophic cardiomyopathy. 39 Amiodarone may be a useful suppressive antiarrhythmic agent in the rare event of recurrent intractable ventricular tachycardia during pregnancy. Labour and delivery Delivery should take place in high-risk centers experienced in the management of this condition and where continuous ECG and blood pressure monitoring are possible. Continued beta blockade and fluid replacement are necessary in the presence of dynamic outflow tract obstruction. Normal vaginal delivery is safe and cesarean section is indicated only for obstetric purposes. The use of prostaglandins for induction of labour is inadvisable because of their vasodila- tor effect, but oxytocic agents are well tolerated. Epidural anesthesia should be avoided because of the production of hypotension, and blood loss should be replaced promptly. After completion of the third stage, the patient should sit up to avoid pulmonary congestion and may require intra- venous frusemide. (Table 13.2) 182 Chapter 13 If there is evidence of left ventricular outflow tract obstruction with hemody- namic deterioration after delivery, fluids and vasoconstriction with phenyl- ephrine to increase afterload are recommended. Beta-adrenergic agents such as dopamine or dobutamine should be avoided as a result of the increase in con- tractility, outflow tract gradient and worsening hypotension that they cause. Continuous monitoring by right heart catheterization may be needed in selected cases and transesophageal echocardiography has been used to assess the hemodynamics. Antibiotic prophylaxis is needed for dental procedures during pregnancy and to cover surgical delivery. Conclusion With care in high risk patients the outcome of pregnancy in women with hy- pertophic cardioimyopathy is usually good (Table 13.3). References 1 Maron BJ, Gottdiener JS, Epstein SE. Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. A wide angle, two dimensional echocardiographic study of 125 patients. Am J Cardiol 1981;48:418– 28. 2 Davies MJ. The current status of myocardial disarray in hypertrophic cardiomyopa- thy. Br Heart J 1984;51:361–3. Hypertrophic cardiomyopathy and pregnancy 183 Table 13.2 Management of delivery in patients with hypertrophic cardiomyopathy Delivery in hospital with continuous ECG and blood pressure monitoring Normal vaginal delivery Do not use prostaglandins Prompt blood loss replacement Sit the patient up after completion of the third stage to avoid pulmonary edema Antibiotic prophylaxis Table 13.3 Outcomes in pregnant patients with hypertrophic cardiomyopathy Reference No. of No. of Cardiac Worsening of Intrauterine Observed patients pregnancies symptoms symptoms deaths deaths during during pregnancy a pregnancy Thaman et al. 26 127 271 36 (28%) 10 (<10%) 3 (2%) 0 Autore et al. 25 100 199 Not reported 6 of 40 patients Not reported 2 (15%) Probst et al. 28 41 150 27% 0% 0 0 a Numbers in parentheses are percentages. 3 Nishimura RA, Holmes DR, Jr. Hypertrophic obstructive cardiomyopathy. N Engl J Med 2004;350:1320–7. 4 Teare D. Asymmetrical hypertrophy of the heart in young adults. Br Heart J 1958;20:1–8. 5 Brock R. Functional obstruction of the left ventricle; acquired aortic subvalvar steno- sis. Guy’s Hospital Rep 1957;106:221–38. 6 Maron BJ, McKenna WJ, Danielson GK et al. American College of Cardiology/ European Society of Cardiology Clinical Expert Consensus Document on Hyper- trophic Cardiomyopathy: a report of the ACC Foundation Task Force on Clinical Ex- pert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. J Am Coll Cardiol 2003;42:1687–713. 7 Maron BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA 2002;287: 1308–20. 8 Richardson P, McKenna RW, Bristow M et al. Report of the 1995 World Health Orga- nization/International Society and Federation of Cardiology Task Force on the defi- nition and classification of cardiomyopathies. Circulation 1996;93:841–2. 9 Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA 1999;281:650–5. 10 Niimura H, Bachinski LL, Sangwatanaroj S et al. Mutations in the gene for cardiac myosin-binding protein C and late-onset familial hypertrophic cardiomyopathy. N Engl J Med 1998;338:1248–57. 11 Nagueh SF, McFalls J, Meyer D et al. Tissue Doppler imaging predicts the develop- ment of hypertrophic cardiomyopathy in subjects with subclinical disease. Circulation 2003;108:395–8. 12 Nagueh SF, Bachinski LL, Meyer D et al. Tissue Doppler imaging consistently detects myocardial abnormalities in patients with hypertrophic cardiomyopathy and pro- vides a novel means for an early diagnosis before and independently of hypertrophy. Circulation 2001;104:128–30. 13 Ho CY, Sweitzer NK, McDonough B et al. Assessment of diastolic function with Doppler tissue imaging to predict genotype in preclinical hypertrophic cardiomyopa- thy. Circulation 2002;105:2992–7. 14 Maron MS, Olivotto I, Betocchi S et al. Effect of left ventricular outflow tract ob- struction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med 2003;348:295–303. 15 Sherrid MV, Gunsburg DZ, Moldenhauer S, Pearle G. Systolic anterior motion begins at low left ventricular outflow tract velocity in obstructive hypertrophic cardiomy- opathy. J Am Coll Cardiol 2000;36:1344–54. 16 Klues HG, Maron BJ, Dollar AL, Roberts WC. Diversity of structural mitral valve al- terations in hypertrophic cardiomyopathy. Circulation 1992;85:1651–60. 17 Frank S, Braunwald E. Idiopathic hypertrophic subaortic stenosis. Clinical analysis of 126 patients with emphasis on the natural history. Circulation 1968;37:759–88. 18 Ryan MP, Cleland JGF, French JA et al. The standard electrocardiogram as a screening test for hypertrophic cardiomyopathy. Am J Cardiol 1995;76:689–94. 19 Van Driest SL, Ommen SR, Tajik AJ, Gersh BJ, Ackerman MJ. Sarcomeric genotyping in hypertrophic cardiomyopathy. Mayo Clin Proc 2005;80:463–9. 20 Watkins H, Rosenzweig A, Hwang D et al. Characteristics and prognostic implications of myosin missense mutations in familial hypertrophic cardiomyopathy. N Engl J Med 1992;326:1108–14. 184 Chapter 13 [...]... cardiomyopathy in pregnancy Acta Obstet Gynaecol Scand 2003;82:389–90 55 Expert Consensus Document on management of cardiovascular diseases during pregnancy The task force on the management of cardiovascular diseases during pregnancy Eur Heart J 2003;24: 761 –81 56 Pan P, Moore CH Doxorubicin-induced cardiomyopathy during pregnancy: three case reports of anesthetic management for cesarean and vaginal delivery in. .. Averbuch M, Bojko A, Levo Y Cardiac tamponade in the early post partum period as the presenting and predominant manifestation of systemic lupus erythematosus J Rheumatol 19 86; 13:444–5 61 Anderson, Fineron FW Aortic dissection in pregnancy- induced changes in the vessel wall and bicuspid valve in pathogenesis Br J Obstet Gynaecol 1994;101:1085–8 Heart Disease in Pregnancy, Second Edition Edited by Celia... remain undiagnosed Viruses have long been suspected of playing a part in initiating both the in ammatory process in dilated cardiomyopathy and an autoimmune process against exposed or damaged myocardial proteins, although no infective agent has been found in cases of PPCM and the causative agent is probably not an infective one Fetal cells are known to enter the maternal circulation during preg- Peripartum... and pregnancy 185 21 Van Driest SL, Ackerman MJ, Ommen SR et al Prevalence and severity of ‘benign’ mutations in the beta-myosin heavy chain, cardiac troponin T, and alphatropomyosin genes in hypertrophic cardiomyopathy Circulation 2002;1 06: 3085–90 22 Ackerman MJ, VanDriest SL, Ommen SR, et al Prevalence and age-dependence of malignant mutations in the beta-myosin heavy chain and troponin t genes in. .. routine use in every parturient woman within sizeable defined populations for insight to be gained about the true prevalence M-mode echocardiography was only just becoming available in the cardiology departments of major hospitals in the 1 960 s and so had not contributed to the diagnosis of the retrospective series of patients described in the key papers from New Orleans in 1 965 2 or from Chicago in 1971 .6. .. Publishing CHAPTER 15 Coronary artery disease Celia Oakley Symptomatic coronary artery disease is still infrequent in women of childbearing age but an increase in its prevalence in pregnancy has been attributed to modern changes in women’s lifestyles Even so most coronary events complicating pregnancy are still not the result of atheroma and are not preceded by angina Coronary atheroma More angina is being... left atrium in mitral stenosis from the left atrium or ventricle in peripartum or other cardiomyopathy paradoxical in atrial septal defect or patent foramen ovale or in cyanotic congenital heart disease from the placenta in molar pregnancy or chorion carcinoma Oxytocic drugs Ergot derivatives Bromocriptine Maternal pre-eclampsia 2 06 Chapter 15 Table 15.2 Causes of myocardial infarction during pregnancy. .. consensus opinion from the workshop in 1997 was of an incidence in between 1 in 3000 and 1 in 4000 in the USA7,19 which suggests that there may be 750–1000 cases a year in the UK Etiology Traditional predisposing causes of PPCM include multiple pregnancy, multiparity, African race, older maternal age, pre-eclampsia and a history of previous peripartum cardiomyopathy Selenium deficiency,20 infection,... clinicians’ thoughts turn to the heart Even florid failure with edema may be attributed to other causes Peripartum and other cardiomyopathic pericardial diseases 191 The higher prevalence of intracardiac thrombosis and embolism in peripartum cardiomyopathy than in dilated cardiomyopathy outside pregnancy is attributable to the hypercoagulable state existing in pregnancy, which increases the risk of intracavitary... be maintained by tachycardia and the diagnosis is often overlooked Mild cases can be managed with support stockings and frugal use of diuretics Pericardiectomy, when essential, can be performed during pregnancy without the need for cardiac bypass in patients not responding to medical therapy Most pericardial diseases can be managed as in non-pregnant patients but indometacin and high-dose aspirin can . suspected of playing a part in initiating both the in- flammatory process in dilated cardiomyopathy and an autoimmune process against exposed or damaged myocardial proteins, although no infective agent has. of heart failure, other cardiomyopathies, pre-existing dilated cardiomyopathy, acute myocardial infarction, ritodrine-induced pulmonary edema in patients given the drug by infusion in saline,. a fulminant peripartum cardiomyopathy showing low voltage and QS waves in leads V1–V3, poor R-wave progression and T-wave inversion in left ventricular leads, suggesting (old rather than evolving)