Peripartum cardiomyopathy PG resident.pptx

Peripartum Cardiomyopathy

1. Peripartum cardiomyopathy Dr.JENISHA MODERATOR : Dr.KAVITA BHATTI

2. CARDIOMYOPATHY The American Heart Association defines cardiomyopathy as ,“a heterogeneous group of myocardial diseases associated with mechanical and/ or electrical dysfunction”.

3. TYPES Primary cardiomyopathies - are solely or predominantly confined to heart muscle. Examples-Hypertrophic cardiomyopathy Dilated cardiomyopathy Peripartum cardiomyopathy Secondary cardiomyopathies- result from generalized systemic disorders that produce pathological myocardial involvement. (DM, SLE, chronic HTN and thyroid disorders)

4. Peripartum cardimyopathy Peripartum cardiomyopathy (PPCM) is one of the rare causes of heart failure in pregnant women. It occurs in late pregnancy or up to 5 months post-partum. It is associated with high mortality and morbidity but with chances of full recovery. The disease progresses rapidly but recovery rates are more if diagnosed early .

5. definitions

6. EPIDEMIOLOGY The incidence of PPCM is uncertain. It varies geographically . In united states, the incidence has been reported to be as low as 1 case per 4,000 live birth. In comparison to Nigeria which has higher incidence as 1 case in every 100 live births. In Japan the incidence is 1:20,000 deliveries.

7. INCIDENCE

8. CAUSES Viral myocarditis. Abnormal immune response to pregnancy. Aberrant response to the greater hemodynamic burden of pregnancy. Hormonal interactions Malnutrition Inflammation Apoptosis

9. RISK FACTORS African race Age > 30 years of age Obesity Hypertension Diabetes mellitus Multigravida Multiple pregnancy (7-15%) Pre eclampsia (22%) Chronic Hypertension.

10. Inconsistent Risk factors Anaemia Autoimmune diseases Tobacco/smoking Substance abuse Hypothyrodism Prolonged tocolysis (beta 2 agonist) If patient had 2 risk factors OR will be 11 6 or more risk factors OR 795

11. OVERALL RISK FACTORS FOR PPCM

12. AGE The incidence of PPCM is strongly associated with age. Even though the disease can strike women of any age, >50% of cases occur in women >30 years of age. Marriage at early age and early parity may contribute to early PPCM

13. HTN, PRE ECLAMPSIA & PPCM A meta-analysis of pooled data from 22 studies showed preeclampsia, HTN (Hypertension) and PPCM to be closely related. Prevalence of PPCM is higher in patients with PE (pre-eclampsia) and HTN than in general population. Pre eclampsia is associated with pulmonary edema, but systolic function is normal in this group of patients. In view of relation between the two, vascular pathologies have been proposed as an etiology for PPCM.

14. Two-hit hypothesis PPCM affects genetically susceptible women who have one of several cardiac gene mutations to include TTNC1 ,TTN & STAT3 . STAT3 enzyme was found in the myocardium of patients with end-stage heart failure secondary to PPCM. This enzyme protects the heart from reactive oxygen species that when increased generates a peptidase known as Cathepsin D This cleaves prolactin into angiostatic N terminal 16 kDA prolactin fragment that promotes apoptosis in endothelial cells and cardiomyocytes apoptosis. Pregnancy at term is further characterized by prodigious secretions of prolactin by maternal pituitary.

15. TWO –HIT HYPOTHESIS Placenta secretes high levels of antiangiogenic molecule, soluble fms -like thyrosine kinase (sFlt-1) This is further worsen by high levels of sFlt-1,which is superabundant in women with pre eclampsia,multifetal pregnancy or both. Bromocriptine therapy has been evaluated because it inhibits prolactin secretion,and studies supports its use

16. TWO-HIT HYPOTHESIS

17. PATHO- PHYSIOLOGY Hemodynamic changes Increase in preload secondary to increase in red cell mass and blood volume Increases cardiac output by 20-30% due to increase in heart rate and stroke volume by 15-25%. All these changes present during first and second trimester ,the moment when the patient with structural heart diseases will develop symptoms.

18. pathophysiology Viral myocarditis : Echo virus, Coxsackie virus and Parvo virus B19. Genetic factors Pro inflammatory state :Increased level of cytokines such as TNF alpha and interleukin-6 have been found in patients with PPCM and heart failure. Autoimmune myocarditis

19. Genetics The most notable example of this is mutations in the sarcomeric gene titin (TTN), a well-established disease gene for DCM. Several rare truncating (i.e. nonsense, frameshift, or splice site) mutations in TTN were reported in PPCM patients from European and American populations. Indeed, a number of PPCM patients have reported a positive first degree family history for heart failure and cardiomyopathy, including those with cardiomyopathy-associated mutations.

20. genetics

21. Genetic testing

22. HISTOPATHOLOGY GROSS APPEARANCE : Heart looks pale, heavier and dilated ,with mural thrombi Inside the heart, the valves look normal and the coronary vessels are patent most of the time unless known history of ischaemia exists. Pericardial effusion is occasionally found. MICROSCOPICALLY :interstitial edema , cellular swelling, fibrosis and hypertrophy is occasionally found in myocardium with often areas of abundant collection of eosinophils.

23. SYMPTOMS Paroxysmal nocturnal dyspnea Pedal edema . Orthopnea Dyspnea on exertion. Dry cough. Palpitations. Light headedness Chest pain Syncope Tachycardia

24. SIGNS Elevated Jugular venous pressure. Displaced apical impulse. Third heart sound Mitral regurgitation murmurs Cyanosis Tachycardia Arrythmia Bilateral pulmonary crackles due to pulmonary edema .

25. INVESTIGATIONS Blood investigations-CBC, electrolytes, TFT, LFT ,RFT Brain Natiuretic peptide (BNP)-elevated in patients with heart failure and PPCM. Troponin T (also used in follow up/prognosis) Chest Xray- cardiomegaly and pulmonary edema (specific for heart failure)

26. investigations ECG –Sinus tachycardia supraventricular tachycardia (AF or flutter) ventricular tachycardia ST segment and T wave abnormalities (non specific) QRS prolongation >120 milliseconds is related to increased mortality in patients with PPCM

27. investigations ECHO - it is the main study to evaluate the anatomy and functionality of the heart in patients with suspected PPCM. LVEF <45% Dilatation of left ventricles/biventricular or rarely atrial dilatation LV thrombus or atrial thrombus MR/TR (due to dilated ventricles) Cardiac MRI - to determine the volume of chambers and ventricular function.

28. INVESTIGATIONS Cardiac catheterization is only for selected patients. Left ventricular catheterization is indicated in patients with suspicion of ischaemic cardiomyopathy. Right ventricular catheterization is less frequently used for evaluation of PPCM. Endomyocardial biopsy to evaluate infiltrative diseases causing heart failure.

29. DIAGNOSIS The cut off for Acute Heart Failure : BNP>100pg/ml Raised BNP +LVEF <45 %= PPCM Raised BNP+ LVEF>45% = unlikely PPCM ( consider other cardiac and extracardiac origin of symptoms-pulmonary /amniotic fluid embolism,HTN disorders of pregnancy or eclampsia) Normal BNP +LVEF >45% =extracardiac origin of symptoms ( anaemia , pneumonia,renal disease)

30. Differential diagnosis Acute pulmonary edema due to underlying cardiac conditions. Hypertension associated heart failure. Pregnancy associated myocardial infarction (PAMI) Takotsubo cardiomyopathy Pulmonary thromboembolism Tachy arrhythmia Amniotic fluid embolism.

31. management Assessment of a patient by both NYHA criteria and WHO classification of pregnancy helps in better assessment of PPCM patients. NYHA classification takes into consideration of cardiac status alone while WHO classification includes functional status and LVEF hence may be a better indicator of prognostication. It guides in predicting maternal mortality and in planning of management of high-risk pregnancies and counselling regarding repeat pregnancies, which is an important component of management of PPCM .

32. NYHA CLASSIFICATION Class I - Disease with no symptoms Class II - Mild symptoms/effect on function or symptoms only with extreme exertion Class III - Symptoms with minimal exertion Class IV - Symptoms at rest

33. WHO CLASSIFICATION

34. management In women with a moderate or high-risk pregnancy (WHO II–III, III, and IV), pre -pregnancy counselling and delivery should be conducted in an expert center by a multidisciplinary team. The minimum team requirements are a cardiologist, obstetrician, and anesthetist expertise in the management of high-risk pregnancies in women with heart disease. Additional experts that may be involved are a geneticist, cardiothoracic surgeon, pediatric cardiologist, fetal medicine specialist, neonatologist, hematologist, nurse specialist & pulmonary specialist .

35. Aim of management in ppcm Treatment aimed at Volume overload. Afterload reduction Rhythm control Ionotropic support

36. MANAGEMENT OF PPCM Salt restriction Volume status management Patient need to be educated by a cardiologist or nurse trained in heart failure management. Neuro humoral blockade with ACE inhibitor and beta blockers are main principles of therapy as in other forms of heart failure with reduced EF. In hypertensives labetalol , carvedilol can be given. Nitrates and hydralazine are preferred instead of ACE inhibitors in preterm pregnancy with PPCM Adequate control of hypertension is achieved with methyl dopa, labetalol and calcium channel blockers . Ionotropic drugs like levosimendan and dobutamine are used in patients in shock with NYHA Class 4 and not recovering from failure.

37. MANAGEMENT Diuretics are used to decrease volume load. Drugs like Aldactone are contraindicated in pregnant women but can be started in post-partum period. These women need specialist advice during lactation. Digoxin can be given in refractory heart failure along with ACI and diuretics. Ivabradine was used for rate control in patients where betablocker was contraindicated

38. GRADES OF PPCM

39. bromocriptine LVEF >25% (no cardiogenic shock ,no ICU treatment) -Bromocriptine 2.5 mg OD for 7 days + prophylactic anticoagulation. LVEF < 25 % (and RV dysfunction +/-cardiogenic shock+/- ICU treatment) -Bromocriptine 2.5 mg BD for 14 days followed by 2.5 mg OD for another 42 days +prophylactic anticoagulation ICU treatment ,cardiogenic shock with ventilation and /or mechanical circulatory support -Bromocriptine 2.5 mg BD initially and titrate to a maximum of 10-20 mg daily depending upon serum prolactin levels until successful suppression

40. Board scheme B romocriptine. O ral Heart Failure drugs(beta- blockers,ACE inhibitors,ARB,MRA ) A nticoagulant (at least in prophylactic dose) R elaxants (iv vasodilators if SBP >100 mg) D iuretics (in case of fluid overload)

41. MANAGEMENT OF PPCM DURING LABOUR Vaginal delivery is preferred if patient is hemodynamically stable. Labour can be induced with dinoprostone gel and augment with concentrated oxytocin to avoid fluid overload

42. Indication of cs obstetric indications (Contracted pelvis,Placenta praevia) Deteriorating maternal conditions Coarctation of aorta Large aortic aneurysm Phaeochromocytoma Marfans syndrome with dilated aortic root Aortic dissection Oral anticoagulants in preterm labour Warfarin within 2 weeks of labour Severe aortic stenosis Cesarean section can be done by GA or epidural SA contraindicated in anticoagulated patients

43. First stage of labour Semi recumbent position. IV fluids to be restricted 50-75ml/hr CVP guided fluids preferably Oxygen in case of Grade3,4 NYHA(5-6L/min) Epidural analgesia for pain relief/ Pudendal block Careful monitoring of vitals hourly To look for basal crepitations,Cyanosis,Raised JVP,pedal edema

44. RECOMMENDATION OF PROHYLACTIC ANTIBIOTICS Women with prosthetic cardiac valves Past history of bacterial endocarditis Unrepaired cyanotic heart disease Cardiac transplant recipient with valve regurgitation

45. Second stage of labour Cut short second stage of labour by Forceps. Try to avoid lithotomy to decrease venous return.

46. Third stage of labour IM Oxytocin 10U during delivery of placenta Ergometrine and Prostaglandins to be avoided as much as possible(cause sudden overloading of heart by sudden increase in intravascular volume from contracted heart). Inj.Lasix IV STAT Watch for Postpartum haemorrhage Tab.Misoprostol 600 mcg PR can be given

47. Fourth stage of labour Golden hour- First one hour after delivery Increased risk for postpartum hemorrhage , anemia , thromboembolic events Monitor vitals closely Administer oxygen

48. Breast feeding It is controversial in peripartum women presenting with heart failure. 2018 ESC guidelines for the management of cardiovascular diseases during pregnancy suggest that preventing lactation may be considered in patients with severe heart failure to avoid high metabolic demands of lactation and breast feeding. To enable safe treatment with all established heart failure drugs.

49. contraception

50. PROGNOSIS GOOD PROGNOSTIC FACTORS : Small LV diastolic dimension (Less than 5.5 cm) Absence of troponin elevation. Absence of LV thrombus Non-Africa American ethnicity LVEF greater than 30-35 % POOR PROGNOSTIC FACTORS : QRS greater than 120 milliseconds Delayed diagnosis High NYHA class Multiparity African descent

51. COMPLICATIONS MATERNAL COMPLICATIONS: Thromboembolism. Arrhythmias. Progressive heart failure. Misdiagnosis as pre-eclampsia Sudden death Recurrence. FETAL COMPLICATIONS: Fetal distress from hypoxia.

52. Delayed recovery Persistence of LV dysfunction defined as EF of <50% at >6 months is a marker of irreversible PPCM. These patients tend to require advanced therapies for their heart failure like CRT (cardiac resynchronization therapy), LVAD (Left ventricular assist device) and transplantation.

53. Course & prognosis Recovery in 50-80% if EF > 50% Mortality to 30-50% reduced significantly. Women with minimal decrease in EF tend to have good prognosis But those with poor EF have high risk of death. In addition,any female who requires assist device tends to have adverse events and low survival. One third will have relapse Relapse is higher in women with persistent Left ventricular failure Normal LV function(usually within 6 months postpartum) doesn’t guarantee an uncomplicated pregnancy (20%)

54. Recurrence with subsequent pregnancy In Patients with history of PPCM, LVEF increased during index pregnancy but decreased again during the subsequent pregnancy, irrespective of earlier values. Heart failure was more frequent in those in whom LVEF has not normalized. Deaths were common in women with persistently low LVEF entering the subsequent pregnancy. There was higher incidence of premature delivery and therapeutic abortions.

55. references Williams Textbook of obstetrics 26 th edition Trongtorsak A, Kittipibul V, Mahabir S, Ibrahim M, Saint Croix GR, Hernandez GA, Chaparro S. Effects of bromocriptine in peripartum cardiomyopathy: a systematic review and meta-analysis. Heart Fail Rev. 2022 Mar;27(2):533-543 Devabhaktuni P,Chennapragada S,Manchala S,Menon R,Patil N,Bhupatiraju S.Peripartum cardiomyopathy management-multidisciplinary approach:2020 vol 9; Dec 4883-4891. Sliwa K,Bauersachs J,Arany Z,Spracklen F.Peripartum cardiomyopathy :from genetics to management European Heart Journal (2021) 42,3094-3102

56. Thank you