Peripartum cardiomyopathy Dr. Sara Al Rajy D-card Student DMCH
CASE SCENERIO…… A woman 29 yrs old admitted to CCU through emergency with complaints of severe dyspnea and fatigue on 2 nd pueperial day of her first pregnancy without h/o of any this type of symptoms before and delivery was uneventful. She had no h/o of repeated res. tract infection and previous hospital admission. O n examination pt is afebrile , BP- 140/80mmHg, pulse-90beat/min, regular, R/R-25/min, O2 saturation-80% without O2, JVP-normal, lungs- bilateral basal crepitation , heart- 1 st and 2 nd heart sound normal, S3 gallop present, oedema - moderate, NT-pro BNP- 864pg/ml, ECG shows sinus tachycardia, chest xray shows cardiomegaly with increase bilateral congestion What is the diagnosis And what are the differentials…….
Normal cardiac findings Raised JVP with prominent pulsation Brisk and diffuse apical impulse, may be shifted lateral and upward Loud S1 , S2 with widely split Occasional S3 Aorto and pulmonary flow murmur Mammary souffle oedema
incidence Type of dilated cardiomyopathy of unknown origin The European Society of Cardiology currently classifies PPCM as a nonfamilial , nongenetic form of dilated cardiomyopathy One in 4000 live birth in United State Less than 0.1 % of pregnancy with comparable good prognosis than other cardiomyopathy Present with LV systolic dysfunction
Risk factors
etiology Precise mechanism poorly defined Some proposed etiologies…… Viral myocarditis ( parvo virus, human herpes virus 6, EBV,CMV) Abnormal immune response Abnormal haemodynamic response Prolactin (produce 16kda fragment) Apoptosis and inflammation ( TNF-alpha, CRP) Selenium and malnutrition Prolonged tocolytics use ( more than 4 wks) Increase oxidative stress
Presenting symptoms… Paroxysmal nocturnal dyspnea Dyspnoea on exertion Orthopnoea Chest pain Cough Palpitation Haemoptysis Most of symptoms are similar to symptoms occur during late antepartum and post partum period
Signs………. Tachycardia, tachypnoea Regular or irregular pulse Persistant neck vein distension Blood pressure normal or decrease Apex beat shifted, thrusting in nature S3 and S4 gallop Mitral holosystolic murmur Bilateral basal crepitation Hepatomegaly (if RHF present)
What are differential diagnosis
Diagnosis confirmed after exclusion of following diseases……. Anaemia Thyroid disease septicaemia Idiopathic dilated cardiomyopathy Accelerated HTN Myocardial infarction Acute pulmonary embolism Severe preeclamsia Pulmonary vasculitis Previous valvular diseases
INVESTIGATIONS
ecg
Ecg
ecg
changes in ecg ……… Sinus tachycardia Arrythmia ( e.g atrial fibrillation) LVH with strain pattern Normal or low voltage QRS complex with inverted T wave Nonspecific ST-T wave changes Biventricular and biatrial dilatation Conduction defect
Chest x-ray
findings in x-ray Cardiac shadow is enlarged in transverse diameter, LV type apex Hyperaemic lung field ( due to pul . congestion) Oligaemic lung field ( due to severe pulmonary hypertension) Bilateral plueral effusion It may be normal x-ray ( due to acute presentation) May give false impression due to heart is pushed upward and laterally during pregnancy
ECHOCARDIOGRAPHY
echo findings…… Reduced LV wall motion is generalized or global rather than regional Dilatation of all chambers (particularly left ventricle) Left ventricular end diastolic dimension exceeds 52mm( normal 36 to 52mm) Valvular compromise including moderate to severe MR and TR Reduce ejection fraction ( <45%) LV thrombus
biomarkers….. NT- proBNP ( not specific, but good sensitive) CRP (prognosis of disease) 16-kDa Prolactin Interferon-gamma Asymmetric Dimethylarginine Cathepsin D Fas /Apo-1
Mri ( magnetic resonance imaging) Should be avoided in pregnancy If needed, then use with considering risk/ benefit ratio
management
Anticoagulants……. Pregnancy itself is hypercoaguable state with decrease body’s natural anticoagulant….. So consider anticoagulant following pt….. 1. LV EF<35% 2. paroxysmal and persistent AF 3. documented Mural thrombus 4. evidence of systemic embolism Warfarin and heparin both are safe during breast feeding
REGARDING DELIVERY AND BREAST FEEDING…. Timing and mode of delivery :- limited data ( need multidisciplinary approach) PPCM with compensated HF: normal vaginal delivery preferred Caesarean is generally reserved for obstretic indications ( e.g critically ill pt need inotropes support ) Epidural anasthesia preferred Early delivery not reqiured if maternal and fetal conditions are stable ( 2010 ESC) Breast feeding should be avoided due to potential effect of prolactin subfragmentation ( 2010 ESC)
DRUG DURING BREAST FEEDING……. Should be avoided…. Spironolactone , atenolol , diltiazem , nifedipine Can be used……. Heparin, warfarin , all ACE inhibitors ( mainly captopril , enalapril ), betablockers ( except atenolol ), digoxin
Advise during discharge Daily fluid intake 1 to 1.25L Avoid heavy work and aerobic exercise Permanent contraception Nutritional supplementation ( thiamine, other vitamins, calcitriol , iron) Contact with doctor if any features of common cold, RTI If possible light exercise ( e.g - walking) Breast feeding now discouraged for more symptomatic pt ( ESC guideline class 2b)
Follow up of patient….. Follow-up echocardiography at rest or low dose dobutamine stress test after 6 month There is actual less data about discontinuation of drug If pt improves then after 6 month gradually tapper doses of drugs upto 12 month Clinical opinion to continue ACE- inhibitor and beta- blocker upto 2 yrs
Subsequent pregnancy
Prognosis of patient Good prognosis from others form of dilated cardiomyopathy 50% patients - complete recovery 25% patients – persistant symptoms 25% patients – develops complication ( progrssive heart failue , arrythmia , thromboembolism Mortality- 3% to 9.6% Pt with persistent cardiomegaly after six month mortality rate 85% in 5yrs