Cardiovascular Disease and Maternal Mortality what do we know and what are the key questions?

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Cardiovascular Disease and Maternal Mortality what do we know and what are the key questions? AFSHAN HAMEED, MD, FACOG, FACC Associate Clinical Professor Maternal Fetal Medicine and Cardiology University of California, Irvine May 6 th 2012

3 million women age 18-44 in the U.S have cardiac disease ~ 1% of pregnant women

Maternal Mortality Rate, California and United States; 1991-2008 ICD-9 ICD-10 Maternal Deaths per 100,000 Live Births SOURCE: State of California, Department of Public Health, California Birth and Death Statistical Master Files, 1991-2008. Maternal mortality for California (deaths 42 days postpartum) was calculated using ICD-9 cause of death classification (codes 630-638, 640-648, 650-676) for 1991-1998 and ICD-10 cause of death classification (codes A34, O00-O95,O98-O99) for 1999-2008. United States data and HP2010 Objective were calculated using the same methods. The break in the trend line represents the change from ICD-9 to ICD-10. U.S. data is available through 2007 only. Produced by California Department of Public Health, Maternal, Child and Adolescent Health Division, February, 2011.

THE CALIFORNIA PREGNANCY-ASSOCIATED MORTALITY REVIEW (CA-PAMR) Report from 2002-2004 Maternal Death Reviews This project was supported by the federal Title V MCH block grant from the California Department of Public Health; Center for Family Health; Maternal, Child and Adolescent Health Division CDPH MCAH California Pregnancy-Associated Mortality Review Project, April, 2012

Leading Causes of Pregnancy-Associated Death based on California Death Certificates, 2002-2004; N=555 (Before CA-PAMR review) Pregnancy-Related (n=139) Not-Pregnancy-Related (n=416) Preeclampsia/eclampsia (16%) Hemorrhage (15%) Amniotic fluid embolism (8%) Cardiomyopathy (3%) Sepsis/infections (1%) Venous embolism (1%) Other complications (43%) of labor, delivery and pregnancy, excluding above Motor vehicle crash injuries (21%) Cardiovascular disease (18%) Violent injuries (homicide and suicide) (16%) Cancer or its complications (13%) Other unintentional injuries (12%) (i.e., drug overdose, non-motor vehicle accidents) All Other causes (21%) CDPH MCAH California Pregnancy-Associated Mortality Review Project, April, 2012

Misclassification for the CA-PAMR Pregnancy-Related Top Causes of Death, 2002-2004 (N=145) Pregnancy- Related Deaths (per death certificate) N (%) Not Pregnancy- Related Deaths (per death certificate) N (%) Pregnancy- Related Deaths (per CA-PAMR) N (%) Cardiovascular Disease 16 (15) 13 (36) 29 (20) Cardiomyopathy 10 (9) 9 (25) 19 (13) Other cardiovascular 6 (6) 4 (11) 10 (7) Preeclampsia/eclampsia 19 (17) 6 (17) 25 (17) Obstetric hemorrhage 16 (15) 0 16 (11) Amniotic fluid embolism 15 (14) 0 15 (10) Deep vein thrombosis/ Pulmonary embolism 12 (11) 3 (8) 15 (10) Other 31 (28) 14 (39) 45 (31) TOTAL 109 36 145 CDPH MCAH California Pregnancy-Associated Mortality Review Project, April, 2012

CA-PAMR Pregnancy-Related Deaths, Causes of Death, by Race/Ethnicity, 2002-2004 (N=145) Clinical Cause of Death White, Non- Hispanic N (%) African- American, Non-Hispanic N (%) Hispanic N (%) Asian N (%) TOTAL Cardiovascular Disease 5 (14) 12 (36) 11 (17) 1 (8) 29 (20) Cardiomyopathy* 3 (8) 9 (27) 6 (9) 1 (8) 19 (13) Other cardiovascular 2 (6) 3 (9) 5 (8) (0) 10 (7) Preeclampsia/eclampsia* 5 (14) 3 (9) 16 (25) 1 (8) 25 (17) Obstetric hemorrhage 5 (14) 2 (6) 7 (11) 2 (17) 16 (11) Amniotic fluid embolism 4 (11) 5 (15) 6 (9) (0) 15 (10) Deep vein thrombosis/ Pulmonary embolism 3 (8) 4 (12) 8 (13) (0) 15 (10) All other causes 14 (39) 7 (21) 16 (25) 8 (66) 45 (31) TOTAL 36 33 64 12 145 *p<.05 CDPH MCAH California Pregnancy-Associated Mortality Review Project, April, 2012

CA-PAMR Major Findings, Summary CA-PAMR methodology has led to identification of an increased incidence of pregnancy-related deaths in California for 2002-2004. Enhanced surveillance and expert case review revealed that cardiac disease, especially cardiomyopathy, is the leading cause of pregnancy-related deaths in California. Findings from CA-PAMR are informing: MCAH maternal health policies, programs and prevention strategies. More information is available at: http://www.cdph.ca.gov/programs/mcah/pages/default.aspx The development of Toolkits by CMQCC to improve the recognition of and response to two of the leading causes of pregnancy-related death: obstetric hemorrhage and preeclampsia. More information available at: http://www.cmqcc.org CDPH MCAH California Pregnancy-Associated Mortality Review Project, April, 2012

Case 24 yo G1P1 presented to the ER postpartum day # 10 with shortness of breath and cough for 7 days Diagnosed with upper respiratory infection Rx : Antibiotics

Case Returns 5 days later with fatigue and continued shortness of breath.

Are these symptoms cardiac?

Common Cardiac Signs & Symptoms Fatigue Shortness of breath Orthopnea Palpitations Light headedness/ dizziness Edema JVD Murmurs 96% pregnant women have functional murmur Third heart sound is common

Physiologic changes Signs and Symptoms of Pregnancy that mimic heart disease Affect diagnostic tests

CXR: Bilateral pulmonary infiltrates cannot rule out pneumonia Clinical correlation suggested DIFFERENTIAL DX Cardiac

Case Continued to be symptomatic Presents with cardiogenic shock Dx PPCMP

Peripartum Cardiomyopathy An Update Uri Elkayam, MD Professor of Medicine / Cardiology Professor of Obstetrics and Gynecology University of Southern California Los Angeles, California elkayam@usc.edu

Peripartum Cardiomyopathy Updated Definition by European Society Working Group Eur J Heart Failure 2010;12:767 PPCM is an idiopathic CMP presenting with HF secondary to LV systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of HF is found It is a diagnosis of exclusion

periartum Cardiomyopathy Clinical Presentation CHF signs and symptoms Arrhythmias (with or without CHF) Thromboembolism Asymptomatic LV dysfunction

PPCM Time of Diagnosis Elkayam et al. Circulation 2005;111:2050 75 Early Number of patients 50 25 Traditional N=123 0 < 27 28-32 33-36 37-40 1 2 3 4 5 Weeks DELIVERY Months PP

Frequency of Peripartum Cardiomyopathy 5000 4500 4000 3500 3000 2500 2000 1500 1000 500 0 1:4350 1:3261 1:3359 1: 2229 1990-1993 1994-1996 1996-1998 2000-2002 810 New cases/year between 2000-2002 The National Hospital Discharge Survey conducted by the National Center for Health Statistics. Mielniczuk LM Am J Cardiol 2006; 97:1765-1768

Number of New Cases of PPCM in the US 1995-2004 retrospective review indicates and incidence of 1:2,066* Number births in the US in 2007-4,317,119 indicating between 1000 to 2000 new case with PPCM every year in the US alone Gunderson et. al. Obstet Gynecol 2011;118:585-91

Peripartum Cardiomyopathy Incidence by maternal age Age (years) Incidence < 20 1:3731 20-24 1:7463 25-29 1:2597 30-34 1:2882 35-39 1:1043 >40 1:510 Gunderson EP et al Obstet Gynecol 2011;118:583

Peripartum Cardiomyopathy Incidence by maternal ethnicity Non Hispanic white - 1 : 2639. Non Hispanic Black - 1 : 706. Hispanic - 1 : 9901. Chinese - 1 : 4566. Filipino - 1 : 842. Other Asian/Pacific Islander - 1 : 5000. Gunderson EP et al, Obstet Gynecol 2011 ;118:583-91 Kaiser Permanente of Northern California, 119,777 women delivered between 1995-1999

Possible reasons for increased incidence of PPCM Pregnancy at older age : ~ 4 yrs increase in age of 1 st delivery since 1970 Birth in women 35-39 and 40-44 yrs increased 43% and 62% respectively Increased number of multiple gestation : Due to ART ( ~121,000 in 2001 vs ~68,000 in 1980) Improved diagnostic capabilities

Peripartum Cardiomyopathy Associated Conditions in the US Maternal age > 30 yrs African American Twin pregnancies History of HTN / Preeclampsia

Complications in 182 patients with PPCM MAJOR ADVERSE EVENTS n= 46 (25%) Death 13 (7%) Transplantation 11 (6%) Temporary circulatory support 2 (1%) Pulmonary edema 17 (9%) Thromboembolism 5 (3%) ICD or permanent pacemaker 10 (5%) Goland, Elkayam. J Cardiac Failure 2009

Delayed diagnosis is common (Goland, Elkayam, J cardiac Failure 2009) 1 week delay in diagnosis after onset of symptoms reported in 60% of 182 cases Complications preceded the diagnosis of PPCM in 50% of pts 32% of surviving patients without cardiac transplantation, had residual brain damage

Preeclampsia and PPCM An e-mail from a patient My OB was concerned with my high BP and swelling but his only thought was preeclampsia. He did tests to rule it out but didn't rule out issues with my heart or asked me if I had difficulty breathing while lying down.

Preeclampsia and PPCM Association between Preeclampsia and PPCM documented in every US study PPCM diagnosed in the majority of pts. with Preeclampsia in the PP period Preeclampsia is not associated with LV systolic dysfunction Author No of pts. Preeclam psia/htn Demakis 27 22% Witlin 28 68% Chapa 32 15% Amos 55 46% Mielmiczuk 16,296 22% Goland 182 43% Elkayam 123 41%

PPCM or Preeclampsia?

PPCM or Preeclampsia? Preeclampsia presenting with heart failure Think PPCM

What are the Key Questions?

Why are we missing cardiac diagnosis? Symptoms attributed to pregnancy Physical findings may be normal for pregnancy

Why are we missing the diagnosis? Lack of familiarity with cardiac diagnoses at various levels Confusion with other conditions that can present with symptoms of shortness of breath such as pulmonary embolism, pneumonia, asthma, preeclampsia

Why are we missing the diagnosis? Lack of attention to Triggers Persistent symptoms Abnormal vitals - tachycardia etc. Multiple presentations to seek care

Opportunities to Improve? Education and Awareness Cardiomyopathy Tachycardia Shortness of breath No response to initial treatment +/- Edema BNP +/- Echocardiogram Preeclampsia Hypertension Proteinuria +/- Edema Laboratory testing

Opportunities to Improve? Need for a structured algorithm with built-in triggers?

Cardiac symptoms should be fully evaluated regardless of pregnancy