Pregnancy & Heart Disease

1. Pregnancy & Heart Disease Dr Nithin P G

2. Introduction • 0.2–4% of all pregnancies in western industrialized countries Am J ObstetGynecol 1998;179:1643–1653. • Ht Dis. & Mortality • In western countries maternal heart disease is now the major cause of maternal death during pregnancy [UK 2003-2005]. • In South India, 30% H’ge, 17% sepsis, 13% hypertensive disorders [2001-2003 Special Survey Of Deaths]. • Optimum treatment of both Mother & Fetus must be targeted Dr Nithin P G

3. Introduction • Hypertensive disorders - most frequent CV events during pregnancy [6–8% of all pregnancies] Eur. Heart J. 2011:ehr218v1-ehr218 • Among heart diseases, • Western world Congenital heart disease, most frequent CVD (75–82%), with shunt lesions predominating (20–65%). • RHD dominates in non-western countries [56–89% ]; Congenital heart disease [just 9–19%]. Eur J Heart Fail 2008;10:855-860, Circulation 2001;104:515-521. • Cardiomyopathiesare rare. Peripartum cardiomyopathy (PPCM) is the most common. Dr Nithin P G

4. Physiological changes in pregnancy

5. CO increases after 5wks, 45% by 24 wks, decreases to near normal by 10 days PP SV-increases from 8 to peak at 20wks, decreases to baseline by 2 wks PP Pl. volume- increases by 6 wks, 1.5-2 times normal by II trimester, plateaus [TBW by 6-8L, Na retension- 500-900 meq] Increased Aortic compliance, A-V shunting in uterus Dr Nithin P G

6. C.O. at labour 7L/min, increases to 9L/min to 10L/min [500 ml autotransfused/ contraction] [Epidural Anesthesia-8L/min, LSCS- 7-8L/min] • Immediately after delivery- sudden increase in blood volume by abrupt increase in venous return, autotransfusion, lack of IVC compression autotransfusion continues for 24-72 hrs [risk of Pulm. Edema] • Complex interactions of gestational hormones, RAA, PG, NO, ANP, BNP pathways produce these changes Dr Nithin P G

7. Clinical Findings in Normal Pregnancy Elevated JVP [increased plasma vol.] S1 Loud [Tachy, increased LV mass], S2 wide split accentuated [P2 delayed], occ S3 Flow murm @ aortic, pulm; ESM grade 3 @ LLSB; cervical venous hum; mammary souffle @ LLSB, Reduced B.S. at lung bases [diaphragm moves up] Apex slight down & out, prominent impulse, active precordium[volume loaded ventricles] Tachycardia, low DBP, Pulse Pressure increased [bounding pulses] Pulsatile fingertips, warm hands, occ. Quincke Pedal oedema >60% women [increased plasma vol., increased venous pressures] Dr Nithin P G

8. Investigations in normal pregnancy Dr Nithin P G

9. Pathological conditions & pregnancy

10. Hypertensive disorders • Hypertension- MC medical problem in pregnancy [15% of pregnancies & 1/4 of all antenatal admissions] • Hypertensive disorders in pregnancy have been recognized as an important risk factor for CVD in women [ Risk for CAD twice; HTN four times] Circulation 2011;123:1243-1262 • Requires high readings on two separate occasions for diagnosis Severity • Mild - >140/ 90 mm Hg • Severe- >160/110 mm Hg Dr Nithin P G

11. Hypertensive disorders Dr Nithin P G

12. Pre-eclampsia • RF- Primi, multiple fetuses,hydatidiform mole or DM • MC cause for IUGR • Features of Severe Pre-eclampsia • Right upper quadrant/epigastric pain due to liver oedema+ hepatic H’ge • Headache + visual disturbance (cerebral oedema) • Occipital lobe blindness • Hyperreflexia+clonus • Convulsions (cerebral oedema) • HELLP syndrome: hemolysis, elevated liver enzymes, low platelet count. Dr Nithin P G

13. Non-pharmacological management [normal diet without salt restriction, Calcium supplementation of at least 1 g daily, Low dose acetylsalicylic acid (75–100 mg/day) H.S. is used prophylactically in women with a h/o of early-onset (<28 weeks) pre-eclampsia] 140-149/ 90-99 • 140-149/ 90-99 + • Gestational HTN • Pre-existing HTN superimposed by gestational HTN • Subclinical organ damage or symptoms at any time during pregnancy Or, ≥150/95 Pharmacological management a-Methyldopa [SE- PP depression], Labetalol, Metoprolol Nifedipine, isradipine Pharmacological management Nitroprusside [fetal cyanide toxicity], Nitroglycerine, I.V. Labetalol, oral methyl dopa ≥170/110 Severe Pre-eclampsia Termination of pregnancy Parenteral Magnesium sulphate to prevent eclampsia Dr Nithin P G

14. Valvular Heart Disease • Risk Stenotic lesions > Regurgitant lesion [ increased C.O. increased transvalvular gradient increased upstream pressures]vs. [ reduced SVR reduces Regurgitant volume] • Left sided diseases> Right sided disease Dr Nithin P G

15. MS • Poorly tolerated [ moderate & severe MS] Tachycardia, increased plasma volume • PHT, Trans valvular gradients, PAP measurements are less reliable marker of severity • Maternal Risks- HF symptoms, Pulmonary edema in II & III trimester. AF [increases risk of T.Emb, pulmonary edema] • Fetal risks- prematurity 20-30%; IUGR 5-20% • Moderate & severe MS counseled against pregnancy without prior intervention Dr Nithin P G

16. Pharmacological management of symptoms MS with symptoms or PAH, restricted activities and β1-selective blockers are recommended. Diuretics are recommended when congestive symptoms persist despite β-blockers. BMV NYHA class III/IV or sys PAP > 50mm Hg, preferably after 20 weeks POG. [CI in asymptomatic women] • Anticoagulation • Paroxysmal or Permanent AF, LA thrombus, prior embolism • Considered in mod/sev MS with spontaneous echo contrast, LA > 40ml/m2, low CO, CCF • Delivery • Vaginal delivery in mild MS, NYHA I/II, no PAH • LSCS in Mod/Sev MS, NYHA III/IV, PAH despite medical therapy & BMV cannot be performed or failed. Dr Nithin P G

17. AS • Usually congenital bicuspid aortic valve [ always assess aortic diameters] • Even severe AS may be asymptomatic • Maternal risk HF 10%, Arrhythmias 3-25% • Fetal risk- Preterm Labour, IUGR, LBW Dr Nithin P G

18. Pharmacological management of symptoms HF- treat with diuretics AF- b-blockers, CCB to control HR, Digoxin also may be used • Pre- pregnancy intervention • Symptomatic severe AS • LVEF<50%, severe LVH (PW> 15mm) • TMT- symptoms or fallin BP • Recent progression of AS • Asc. Aorta> 50 MM (27.5mm/m2) • During Pregnancy • Severe symptomatic AS + refractory to medical therapy/ life threatening symptomsNon calcified valve may be subjected to BAV/o.w. emergency AVR • Delivery • Vaginal delivery + regional anesthesia in non-sev AS • LSCS in Sev AS Dr Nithin P G

19. Regurgitant lesions • Better tolerated • Maternal risk- HF, Arrhytmias, Progressive worsening of regurgitations • Moderate to severe Regurgitant lesions may undergo exercise testing to decide pre pregnancy intervention • Severe lesions + symptoms/ impaired LV function/ Ventricular dilatation  treated surgically, if possible repair • TV repair if moderate Secondary TR with annular dilatation >40mm, usu during left sided valve surgeries Dr Nithin P G

20. PS & PR PS is generally well tolerated • Complic of sev PS-RV failure & Arrhythmias. • Prepregnancy balloon valvuloplasty in severe stenosis (peak Doppler gradient > 64 mmHg) • LSCS is considered in patients with severe PS and in NYHA class III/IV despite medical therapy and bed rest, in whom percutaneous pulmonary valvotomy cannot be performed or has failed. Severe PR with impaired RV function • pre-pregnancy pulmonary valve replacement (preferably bioprosthesis) should be considered. Dr Nithin P G

21. Prosthetic valves Mechanical valves • Excellent H.D. Performances • Long term durability • Thrombogenic Bioprosthetic valves • Good H.D Performances • Much less thrombogenic • High risk of valve degeneration [~50% women <30yrs at 10 yr post implant] • M> A,T position • Reoperation mortality risk addl 5% Dr Nithin P G

22. Anticoagulation Strategies Maternal mort. Valve thrombosis 3.9 % 2 % OAC 4 9.2 UFH OAC 35 UFH 15 9 LMWH 3.6 LMWH OAC Arch Intern Med 2000;160:191-196 Dr Nithin P G

23. Anticoagulation Strategies • OAC throughout pregnancy best strategy [esp. if warf <5 mg, Acitrom (acenocoumarol) <2 mg] • Discontinuation of OAC b/w 6 &12 wks and replacement by UFH (a PTT ≥2× control; infusion in high risk pts) or LMWH twice daily (according to weight and target anti-Xa level 4-6 hours post-dose 0.8-1.2 U/mL in patients with a warfarin dose required of >5 mg/day • OAC discontinued and UFH (a PTT ≥2× control) or adjusted-dose LMWH (anti-Xa level 4-6 hours post-dose 0.8-1.2 U/mL) started at the 36th week • LMWH replaced by i.v. UFH at least 36 hours before planned delivery. UFH should be continued until 4-6 hours before planned delivery and restarted 4-6 hours after delivery if there are no bleeding complications • If delivery starts while on OACs, caesarean delivery is indicated to prevent fetal bleed OAC UFH/L OAC UFH/L UFH H 12 wks 36 wks 6 hrs 6 wks 6 hrs Dr Nithin P G

24. Peripartumcardiomyopathy Eur J Heart Fail 2010;12:767–778. Dr Nithin P G

25. Etiology Fas/Apo-1, C-reactiveprotein, IFN-g and IL-6 Cathepsin D in response to oxidative stress cleaves Prolactin into angiostatic & proapoptotic fragment 16 kDaProlactin Viruses Autoimmune Dr Nithin P G

26. Differential diagnosis Eur J Heart Fail 2010;12:767–778. Dr Nithin P G

27. SA- 6m & 2yr mortality rates 10% & 28%. Brazil & Haiti 6m rate 14– 16% Turkey- 4yr rate 30% LV func. returns to normal in 23–41% Dr Nithin P G Eur J Heart Fail 2010;12:767–778.

28. Natural history N Engl J Med 2001;344:1567-71. • Group 1 (28) EF after index pregnancy > 50% • Group 2 (16) EF <50 % • More HF symptoms in group 2 [44 vs. 21%] • Group 2- 3 deaths during subsequent pregnancy Am J ObstetGynecol 2008;199:415.e1-415.e5. Dr Nithin P G

29. Other cardiomyopathies DCM • Typical symptoms of HF, LV dilation, and LV systolic dysfunction of unknown origin. • Differentiation from PPCM is supported by the ‘time of manifestation’ • If not known before conception, the condition is unmasked during the I or II trimester when hemodynamic load is increasing. • Family history of DCM • Secondary cardiomyopathies, such as infiltrative , toxic CM & storage diseases manifest themselves in pregnancy. • Maternal risk- Risk of deterioration of DCM during gestation and PP. LVEF < 40% is a predictor of high risk. LVEF is <20% MTP may be considered. Dr Nithin P G

30. Other cardiomyopathies HCM • Frequently diagnosed for the first time in pregnancy by echocardiography. • Characterized by diastolic dysfunction due to hypertrophied non-compliant myocardium[ HF, Pulmonary congestion] , severe LVOTO [syncope] and arrhythmias [SVT & VT]. • Maternal risk -usually tolerate pregnancy well. Risk is increased in those symptomatic before pregnancy and in those with a high outflow gradient. Dr Nithin P G

31. Management • Managed as in non pregnant states • Hydralazine & nitrates instead of ACEI, b1- selective blockers should be used [n/b- hypoglycemia, bradycardia, resp. depression]. Diuretics used judiciously [ Aldosterone antagonists avoided]. Anticoagulation- I/C thrombus, AF HCM • b-Blockers - >mild LVOTO and/or wall thickness >15 mm to prevent sudden pulmonary congestion. Delivery under b-blockers recommended • b-Blockers- rate control in AF & to suppress ventricular arrhythmias. Verapamil second choice (AV block in the fetus). Cardioversion for persistent arrhythmia because AF poorly tolerated. Therapeutic anticoagulation as indicated • Severe LVOTO- Epidural anaesthesia must be used with caution • I.V. fluids given judiciously [in view of diastolic dysfunction] • Syntocinon slow infusion [hypotension, arrhythmias, and tachycardia] Dr Nithin P G

32. Congenital Heart diseases and PAH • Miscarriage rate higher in more complex diseases • Maternal cardiac complications in 12% of completed pregnancies & pts are at higher risk of late cardiac events after pregnancy • Offspring mortality (4%) more frequent than general population (Elective) (>20 weeks) Eur. Heart J. 2011:ehr218v1-ehr218 Dr Nithin P G

33. Shunt lesions • Hemodynamically significant shunt best repaired pre pregnancy • Insignificant lesions, good LV function no indication for closure during pregnancy • Severe PAH/ eisenmenger syndrome – high risk • Pre-pregnancy evaluation of the severity of a shunt, residual defect in case of repair, estimation of pulmonary pressures, cardiac dimensions & function • Increase in Arrhythmias, T. Emb. and worsening of NYHA class, higher incidence of preeclampsia • Hemodynamically significant shunt – SGA baby, fetal mortality Dr Nithin P G

34. Coarctation of Aorta • Unrepaired native CoA and those repaired with residual HTN, residual CoA, or aortic aneurysms have an increased risk of aortic rupture and rupture of a cerebral aneurysm during pregnancy and delivery • Risk Factors to be screened for- aortic dilatation and bicuspid aortic valve • HTN should be treated[ aggressive treatment avoided to prevent placental hypoperfusion] • Percutaneous intervention for re-CoA associated with a higher risk of aortic dissection than outside pregnancy [indic- severe HTN despite max medical Rx and there is maternal or fetal compromise] [covered stents may lower the riskof dissection]. • Vaginal delivery with epidural analgesia preferred Dr Nithin P G

35. Cyanotic congenital heart disease • Maternal complications (HF, pulmonary or systemic thrombosis, SVT, IE) occur in 30% of cyanotic pregnant patients. • If resting O2 sat. <85%- substantial maternal and fetal mortality risk expected and pregnancy is contraindicated. If 85–90%, measure it during exercise Significant and early decrease  pregnancy has poor prognosis. • With resting maternal blood saturation >90%, fetal outcome is good (<10% fetal loss). Dr Nithin P G

36. Cyanotic congenital heart disease Tetralogy of Fallot • In unrepaired patients, surgical repair is indicated before pregnancy [ Repaired TOF usually tolerate pregnancy well] • Cardiac complications during pregnancy upto 12% of patients. [Arrhythmias & HF-MC; Thr. Emb., progressive aortic root dilatation, & IE]. • Risk Factors  RV Dysfunction &/or mod to sev. PR [Pregnancy associated with persisting increase in RV size] • In repaired symptomatic TOF, RV dilatation due to severe PR, pre-pregnancy PVR (homograft) Ebstein’s anomaly • Ebstein’s anomaly without cyanosis & HF, pregnancy is often tolerated well. • Symptomatic + Cyanosis and/or HF should be treated before pregnancy or counselled against pregnancy. In severe symptomatic TR  pre-pregnancy repair . [haemodynamic status depends on TR severity & RV function] • Associated ASD & WPW syndrome. (Incidence of arrhythmias increased) • Other complications- shunt reversal and cyanosis; paradoxical emboli Dr Nithin P G

37. Cyanotic congenital heart disease TGA • Atrial switch operation (Senning or Mustard repair) • Increased risk of arrhythmias & HF • Underlying bradycardia or junctionalrhythmB-blockers with caution. • Irreversible decline in RV function in 10% cases. Pts with > moderate impairment of RV function or severe TR should be advised against pregnancy. • Arterial switch surgeries – usually normal pregnancy CCTGA • Risk depends on functional status, ventricular function, presence of arrhythmias, and associated lesions. • Complications- arrhythmias& HF • Pre-disposed to developing AV block B-blockers with caution. • Irreversible decline in RV function in 10% cases. • Patients with NYHA functional class III or IV, EF < 40% or severe TR should be counseled against pregnancy Dr Nithin P G

38. Cyanotic congenital heart disease Fontan patient • Moderate to high risk pregnancies [Esp. if the Fontan circuit is not optimal] • Atrial arrhythmias & NYHA class deterioration • Pregnancy C.I. - O2 saturation < 85% at rest, depressed ventricular function, mod to sev AV regurgitation or with protein-losing enteropathy • Premature birth, small for gestational age, and fetal death in up to 50%. • Therapeutic anticoagulation should be considered. Dr Nithin P G

39. Pulmonary Hypertension& Eisenmenger • Low pregnancy-independent exercise capacity, superimposed on the gestational CV demands, Insufficient adaptation of the right heart and Poorly compliant pulmonary vasculature. J Am CollCardiol 1998;31:1650 –7 • Even moderate PAH can worsen during pregnancy - decrease in SVR and overload of RV& “no safe cut-off value” • High maternal mortality risk is reported (30–50% in older series & 17–33% in more recent papers) in pts with severe PAH and Eisenmenger syndrome. Eur Heart J 2009;30:256–265. Eur Heart J 2009;30:256–265. Dr Nithin P G

40. Pulmonary Hypertension& Eisenmenger • Maternal death occurs in “the last trimester of pregnancy & in the first months after delivery” • pulmonary hypertensive crises • pulmonary thrombosis • refractory right heart failure. • This occurs even in patients with little or no disability before or during pregnancy. • Risk factors for maternal death are: late hospitalization, severity of PAH, and GA. • Neonatal survival rates are reported to be 87–89%. Eur Heart J 2009;30:256–265. J Am CollCardiol 1998;31:1650 –7 Dr Nithin P G

41. Management • Avoid Pregnancy & MTP • Maintain circulating Volume, and to avoid systemic Hypotension, Hypoxia, and Acidosis which may precipitate refractory HF • Supplemental O2 therapy if hypoxaemia; Haemodynamic monitoring by Swan–Ganz catheter not indicated now [PA rupture] • Diuretics must be used judiciously and at the lowest E.D. to avoid haemoconcentration and intravascular volume depletion. Microcytosis and iron deficiency should be treated with supplemental oral or i.v. iron • Anticoagulation- Continued in patients were there is indication for use outside pregnancy. Used with caution in Eisenmenger syndrome [prone to haemoptysis and thrombocytopenia]- used in PE or HF • I.V. Prostacyclin or aerosolized Iloprost [to improve haemodynamics during delivery] • Continue drugs for PAH [Bosentan-teratogenic, Sildenafil-category B] • Planned LSCS and vaginal delivery with incremental regional anaesthesia are favoured over emergency LSCS delivery. Dr Nithin P G

42. Management of cyanotic mothers • Medical • Restriction of physical activity and supplemental oxygen are recommended. • Because of the increased risk of paradoxical embolism, prevention of venous stasis (use of compression stockings & avoiding the supine position) is important. For prolonged bed rest, prophylactic heparin administration should be considered. • Haematocrit and Hb levels are not reliable indicators of hypoxaemia. • Diuretics and iron therapy are indicated in patients with Eisenmenger syndrome. • Vaginal delivery is advised in most cases [timely hospital admission, planned elective delivery, and incremental regional anaesthesia] If the maternal or fetal condition deteriorates, an early caesarean delivery should be planned. [risks of anesthesia] Dr Nithin P G

43. Aortic Diseases • Pregnancy is a high risk period for all patients with aortic pathology, and aortic pathology is reported as one of the leading causes of maternal mortality • Causes- • Heritable Aortic diseases- pre-disposing patients to both aneurysm formation and aortic dissection. [ Marfan syndrome, bicuspid aortic valve, Ehlers–Danlos syndrome, Turner syndrome, and familial forms of aortic dissection, aneurysm, or annuloaorticectasia] • Congenital heart disease (TOF, aortic coarctation) may be accompanied by aortic dilatation or aneurysm formation. • non-heritable aortic pathology Dr Nithin P G

44. Aortic Diseases • Susceptibility to dissection- hormonal changes during pregnancy [most often in the last trimester of pregnancy (50%) or the early postpartum period (33%)] • an enlarged aortic root diameter [Marfan > 45mm ;Bicuspid AoV>50mm (>27mm/m2)] • previous aortic dissection • Imaging of entire aorta performed before pregnancy • Vaginal delivery in < 40 mm, Vaginal delivery with epidural anesthesia in 40-45mm, LSCS in >45mm [ In non-marfan, >40mm] Dr Nithin P G

45. Arrhythmia • Premature extra beats and sustained tachyarrhythmias become more frequent and may even manifest for the first time during pregnancy • PSVT in 20-44% of pregnancy Am J Cardiol 2006;97(8):1206-1212 • Immediate electrical cardioversion is recommended for a/c Rx of any tachycardia with haemodynamic instability • For acute conversion of PSVT, vagalmanoeuvrefollowed by I.V. adenosine is recommended. I.V. metoprolol or propranolol can also be considered • For long-term management of SVT oral digoxin or metoprolol/propranolol is recommended. If not successful oral sotalol or ecainide may be used Dr Nithin P G

46. Arrhythmia • Immediate electrical cardioversion of VT is recommended for sustained, unstable & stable VT . • I.V. Sotalol or Procainamide may be considered for a/c conversion of sustained, haemodynamically stable, and monomorphic VT. Not responding  Amiodarone • Oral metoprolol, propranolol or verapamil is recommended in idiopathic sustained VT (Long-term management). If unsuccessful  oral sotalol, ecainide, propafenone • β-blockers recommended during pregnancy and also postpartum in congenital long QT syndrome. • ICD implantation, if clinically indicated, is recommended prior to pregnancy but if required, during pregnancy also. Implantation of PPI or ICDs (preferably one chamber) should be considered with echo guidance, especially if the fetus is beyond 8 weeks gestation. Dr Nithin P G

47. CAD • Coronary dissection [LAD] as a cause for MI • ECG & Troponin measurements in all patients with chest pain • Aortic dissecction also to be ruled out in pregnant women with chest pain • PCI treatment of choice in STEMI. STK do not cross placenta but can lead to increased H’ge • PCI in high risk NSTEMI only Dr Nithin P G

48. General Management

49. Risk stratification Circulation 2001;104:515-521 Dr Nithin P G

50. Dr Nithin P G