DR. SNIGDHAM.S., D.N.B (OBG)SENIOR RESIDENT

PGIMERCHANDIGARH

PERIPARTUM CARDIOMYOPATHY

OUR EXPERIENCE…

32yrs G1P1L1@33+6wks with DADC twin

pregnancy refd. i/v/o Breathlessness x 14 days

Associated with palpitation, orthopnea, pedal

edema

RELEVANT HISTORY…

• VD @ term 5yrs back→ LB/girl/ 2.5kgObstetric history

• no h/o DM, Htn, Breathlessness, TBPast History

• No h/o cardiac disease, TBFamily history

• No h/o ↑BP, anemiaPresent pregnancy

CLINICAL EXAMINATION

Vitals- pulse- 110/min, BP- 150/100mmHg

Chest- mild b/l basal crepts, SpO2- 95% on O2

CVS- S1S2- normal

P/A- edema+ - ut- TS/ relaxed/ multiple fetal parts palpable/ FHS1 & 2- reg

Pedal edema+

PLAN

Evaluation of breathlessness

Antenatal steroid administration

Multidisciplinary approach- cardiologists, anesthetists

INVESTIGATIONS…

Hb- 10gm/dlTLC-11,100Platelets- 1.49SE- 135/4.8/109RFT- 22/0.7PTI- 100%

TSH- 0.08

ABG-7.51/22.9/91.7/15.6/-7.0/29%/9.8

INVESTIGATIONS…

• Pulmonary congestion• Enlarged cardiac silhouetteCXR

• Mod MR/ Mod TR• Global LV hypokinesia• LVEF-30-35%• Mod LV systolic & diastolic

dysfunction• Mild Pericardial Effusion

Echocardiography

• Mild rt. Pleural effusion; kerley B-lines

• No e/o DVTChest USG

Compression USG

TRANSABDOMINAL ULTRASONOGRAPHY

Twin live fetusesT1- Breech liquor- adequate FM+ FBM+T2- Transverse liquor- adequate FM+ FBM+

CLINICAL IMPRESSION…

G2P1L1 @ 33+6wks with twin gestation with PPCM (NYHA IV)

TREATMENT…DAY1

Inj. Lasix 20mg iv BD

Tab. Digoxin 0.125mg OD

Tab. Carvedilol 3.125 ½ BD

PLAN

Termination of pregnancy: C-delivery

Preanaesthetic evaluation and plan

TREATMENT…

Elective LSCS i/v/o maternal+ fetal indication with bl TL

Liquor- thin MSL in both sacsT1- boy/1.695/ AS- 4,6 T2- girl/1.535/AS-6,7

DOS-2hrs, IO blood loss- <300ccBP- 106/70, PR- 100-105bpmU/O- 100ml clear

Pt. shifted to TICU

COURSE AFTER LSCS 2 days stay in Trauma ICU Patient rcvd on day2- vitals- BP- 120/88, Pulse- 72/min RR-20/min, SPo2- 100% Drugs- T. carvedilol 3.125mg BD T.digoxin 0.125mg OD Inj.lasix 40mg BD T. azithromycin 500mg Inj. UFH 5000U s/c BD o Negative balance maintained

Discharged on day 8- T. Digoxin 0.125mg OD - T. Carvedilol 3.125 mg BD - T. Diucontin K 20mg @8AM - T. Lasix 20mg @4PMo

Cont.

FOLLOW UP On day 10- No complaints 2-D echo- LVEF>40% ECG- normal

Advice- Stop BF Cont. other drugs

POINTS OF DISCUSSION Criteria for diagnosis Risk factors Etiology Diagnostic dilemma Management Differential diagnosis Contraception Prognosis Future pregnancies Take home message

PERIPARTUM CARDIOMYOPATHY

4% of all cardiomyopathies 1:3000-4000 preg Dilated Cardiomyopathy developing in the last

month of pregnancy or first 5 months post-partum

Mortality rate- 5 -32% (J Am Coll Cardiol.

2000)

CRITERIA FOR DIAGNOSIS 4 Criteria

Development of Heart failure in the last month of pregnancy, or within 5 months postpartum

Absence of a determinable cause for cardiac failure Absence of heart disease before last month of

pregnancy Left Ventricle impairment demonstrated on Echo

ECHO: EF<45% M Mode fractional shortening less than 30% End diastolic dimension >2.72 cm2/m2

(Hibbard et al 1999)

FACTORS

o Advanced maternal age

o multiparity

o multiple gestations

o African American race o gestational hypertensiono preeclampsia

o diabetes

o obesity, malnutritiono poor prenatal careo breast-feedingo substance & tobaccoabuse o prolonged tocolysiso family history

ETIOLOGY Multiple studies have attempted to elucidate a

distinct etiology Theories:

Myocarditis- reported incidence 9-62% Abnormal Immune Response Maladaptive response to hemodynamic stresses of

pregnancy Genetics Metabolites of prolactin

DIAGNOSTIC DILEMMA

SOBPND

PalpitationEnlarged cardiac

silhoutteLVEF<45%

SOB↑BP

Pulmonary edema

CLINICAL PRESENTATION Symptoms:

Paroxysmal Nocturnal Dyspnea Palpitation Dyspnea on Exertion Cough Orthopnea Chest Pain Abdominal Discomfort

Signs: Cardiomegaly Edema Holosystolic murmur

DIAGNOSIS ECG- sinus tachycardia, nonspecific ST changes 2-D echocardiogram- spherical LV, MR, TR, LVEF<45% CXR- venous congestion, enlaged cardiac silhoette,

pericardial effusion Lab: CBC, BNP, TSH, Ferritin

Cardiac troponin, TNFα, IL-6, INF-γ, CRP

If persistent past initial therapy: Cardiac catheterization ?Myocardial biopsy

TREATMENT Medical management

Diureticsß-blockersDigoxinAnticoagulants

*Therapeutic decisions are influenced by drug-safety profiles during pregnancy and lactation

DRUGS Digoxin Class C

Symptomatic control Requires level monitoring Therapeutic levels 0.7-1.2

DIURETICS Lasix Class C

Reserved for cardiac conditions Not recommended in PIH May decrease placental perfusion

Thiazide Diuretics Reserved for cardiac conditions Not recommended in PIH Thrombocytopenia has been reported in breast

feeding infants

VASODILATORS Hydralazine Class C

Compatible with breastfeeding

ACE Inhibitors Class D in 2nd/3rd

trimesters Reserved for postpartum

use-compatible with BF Renal toxicity in infants

exposed in utero

BETA-BLOCKERS Class C Compatible with breast feeding Has been shown to cause IUGR in some

infants in utero.

ANTICOAGULANTS Heparin Class C

Short half life-can be discontinued prior to delivery to prevent maternal hemorrhage

Not excreted in breast milk

Warfarin Class D Contraindicated in pregnancy Safe in breast feeding. Not excreted in breast

milk.

Heart failure and pregnancy are independent risk factors for thromboembolism

OTHER THERAPY IV Immune Globulin

One retrospective study 6 PPCM treated 11 controls All 6 treated had >10 units improvement in EF,

compared only 4/11 controls (All pts had diagnosis of Myocarditis and dilated

cardiomyopathy)*McNamara et al. Circulation 2007; 95:2476

Targeted therapies such as intravenous immunoglobulin, pentoxifylline, and bromocriptine have shown promise in small trials but require further evaluation

DIFFERENTIAL DIAGNOSIS PIH

However, HF associated with PIH represents a diastolic failure, vs. systolic in PPCM

Pulmonary Embolism usually ruled out by CXR If still suspicious →spiral CT

First 12 hrs after delivery – most critical period s/o pulmonary congestion & edema must be carefully

watched for. Sedatives –given in first few days to reduce anxiety related

tachycardia. bed rest any infection to be taken seriously breast feeding c/I in class IV

POSTPARTUM PERIOD

CONTRACEPTION Condoms, diaphragms, caps and sponges include relatively

low cost and wide availability Copper IUDs carry a small risk of menstrual irregularities,

anemia, pain, and expulsion Tubal ligation Transcervical route of tubal occlusion 2 mechanical systems implanted under hysteroscopic

guidance have been approved by the FDA- Essure, Adiana Vasectomy

Combined hormonal contraceptives(Oral pills, vaginal rings, patches, injectables)-

MEC Cat2 in uncomplicated MEC Cat4 in complicated

Progesterone only contraceptives( POP, DMPA/NET-EN, LNG)- MEC Cat1 in both

CONTRACEPTION…

PROGNOSIS Mortality estimates range from 25-50% Most deaths occur within 3 months

postpartum Deaths usually caused by:

Progressive pump failure Arrhythmias Thromboembolic events

PROGNOSIS CONT… India study

20 pts. PPCMFollowed for 14 months postpartumFound several factors for deterioration:

Age >30 High Parity Later onset of treatment Worse echo findings on initial exam

*Elkayam et al. N Engl J Med 2009; 344:1567

FUTURE PREGNANCIES?? Opinions widely vary Most experts agree that patients should avoid

future pregnancy- LV dysfunction is persists > 6 months

- LVEF< 40%

Evidence supports that patients with LVEF>50% are identified as “full recovery” and if in their future pregnancy they experience relapse of heart failure, treatment is very effective

FUTURE PREGNANCIES CONT… Highly Individual

Patient education of risks MF Monitoring Cardiology involvement in decision

If future pregnancy desired: Maternal Echocardiogram per trimester Serial sonograms for growth Again, Subspecialty involvement

TAKE HOME MESSAGE Fixed criteria for diagnosis Early diagnosis and initiation of treatment is important for

prognosis Initial severity of left ventricular dysfunction is predictive

of long-term outcome Multidisciplinary approach Pharmacologic agents are the mainstay of treatment Counselling for future pregnancy High mortality rate