3. Peripartum Cardiomyopathy Paul M. Johnson Department of Internal Medicine University of North Carolina Hospitals November 11, 2009

4. Overview Defining PPCM Incidence Etiology Diagnosis Treatment Prognosis Our patient today… Key Points http://www.e-heart.org/Pages/03_Cardiomyopathy/03_Cardiomyopathy_DCM_001.htm

5. How to define PPCM NHLBI Workshop on Peripartum Cardiomyopathy (2000) – development of heart failure in the last month of pregnancy or five months after delivery – no pre-existing heart disease – no determinable cause of cardiomyopathy – LV systolic dysfunction defined by LVEF <45% or reduced shortening fraction (30% on M-mode) JAMA 2000; 283: 1183-8

6. Incidence “These Hausa-Fulani women eat large quantities of a local lake salt, kanwa, for 40 days postpartum. The syndrome is markedly more common in the hot rainy season, when evaporative water loss is less, than in the dry season. The first postpartum days are spent confined to bed in a small heated room. Once or twice daily the new mother is given hot baths with branches which have been dipped in boiling water. The combination of excessive sodium intake and diminished evaporative water excretion seems to precipitate failure in both normotensive and hypertensive patients.” - Circulation. 1977; 56: 1058-1061

7. Incidence of PPCM Reported incidence is highly variable – Nigeria among the Hausa in Zaria, as high as 1 per 100 live births up to 13% of hospital admissions for females – Haiti 1 per 300 live births – South Africa 1 per 1000 – United States between 1 per 3000 to 1 per 4000 live births about 1300 women per year, mortality of up to 5% a reported geographic predilection for Southern states Int J Cardiol 2007; 118: 295-303

8. Risk factors for PPCM advanced age >30 multiparity African descent multiple gestation obesity preeclampsia/eclampsia hypertension prolonged (>4 weeks) tocolysis with beta agonists risk factors remain poorly understood Int J Cardiol 2007; 118: 295-303

9. What causes PPCM? Lancet 2006; 368: 687–93

10. Etiology, in other words most cases are idiopathic myocarditis – predisposition to more severe forms of viral myocarditis (8.8% - 76% prevalence) autoimmune – fetal antigen response – chimerism hemodynamic – inc blood volume – inc cardiac output Lancet 2006; 368: 687–93

11. Diagnosis DOE, fatigue, pedal edema can be normal in pregnancy Be aware of warning signs – PND, crackles, JVD CXR – cardiomegaly, edema, pleural effusions EKG – normally NSR, but occasionally LVF, TWIs, Q wave Must have ECHO for diagnosis

12. Treatment of PPCM Similar to other types of CHF – optimize hemodynamics – relief of symptoms – chronic therapies that improve long-term outcomes Specifically – avoid ACEI in pregnancy (OK in breast feeding) – use of hydralazine, digoxin acceptable Anticoagulation – consider in LVEF <30% Transplant – 4-7% of patients – successful pregnancies after transplant Immunosuppression in myocarditis-mixed results, never empirically

13. Prognosis prognosis related to related to LV recovery 50% recover baseline function within 6 months www.uptodate.com from Felker et. al. N Engl J Med 2000; 342: 1077

14. Prognosis-another pregnancy? Recovered LV function – 28 women – LVEF decreased 20% in 6 women – 6 women got HF – no deaths Persistent LV dysfunction – 16 women – three deaths – HF in 7 patients – premature delivery in 6, therapeutic abortion in four Lancet 2006; 368: 687–93 NEJM 2001; 344: 1567

15. Our patient today… admitted to cardiology with sore throat and TWI on EKG five days ago diagnosed with strep throat and discharged with amoxicillin x 10 days ECHO showed EF 50-55%

16. Key Points PPCM diagnosed in 1 per 3000 to 4000 births/year Etiology is likely multifactorial, and still unclear Set diagnostic criteria – no previous disease, no other cause – onset one month before, five months after birth – LVEF < 45% 50% regain normal LVEF, 50% do not No guidelines for subsequent pregnancies

17. References Abboud, J, et. al. Peripartum cardiomyopathy: A comprehensive review. Int J of Cardiol 2007; 118: 295-303. Bales, AC, and Lang, RM. Peripartum cardiomyopathy. Accessed 11/10/09 at www.uptodate.com Elkayam, U, et. al. Maternal and fetal outcomes of subsequent pregnancies in women with peripartum cardiomyopathy. NEJM 2001; 344: 1567 Fillmore, SJ, and Perry, EH. The evolution of peripartal heart failure in Zaria, Nigeria. Some etiologic factors. Circulation. 1977;56:1058-1061 Rhamaraj, R, and Sorrell, VL. Peripartum cardiomyopathy: causes, diagnosis, and treatment. Clev Clin J Med. 2009; 76: 289-296 Sliwa, K, et. al. Peripartum cardiomyopathy. Lancet. 2006 Aug 19;368(9536):687-93.