Case reviews of rare conditions as an MCH tool to improve follow up and prevention strategies - Georgia’s experience- MCH Epidemiology Conference December, 2003, Arizona December, 2003, Arizona

iolanda Grigorescu MD, MSPH CDC/ORISE MCH epidemiologist fellow  Violanda Grigorescu MD, MSPH CDC/ORISE MCH epidemiologist fellow  Heena Joshi MS 2,3 MCH Epidemiologist MCH Epidemiologist  Emily Kahn, PhD, MPH 1,2,3 Chief MCH Epidemiology Section Chief MCH Epidemiology Section  Edma Diller, MPH 4 Epidemiologist Epidemiologist  Melissa Tobin-D’angelo, MD 2 1 Centers for Disease Control and Prevention, Division of Reproductive Health 2 Georgia Division of Public Health / 3 MCH Epidemiology Section 4 Hospital A

Peripartum cardiomiopathy (PPCM) investigation  Maternal mortality is a reportable condition in GA  Hospital A reported a maternal death due to PPCM  Cluster of PPCM suspected  GDPH/MCH Epidemiology Section was informed

Peripartum cardiomiopathy (PPCM) What is PPCM? PPCM is considered a rare life threatening cardiomyopathy of unknown cause that occurs in the peripartum period in previously healthy women

PPCM Definition/Diagnosis  Based on 4 criteria: (1) development of cardiac failure in the last month (1) development of cardiac failure in the last month of pregnancy or within 5 months after delivery, of pregnancy or within 5 months after delivery, (2) absence of a demonstrable cause for the (2) absence of a demonstrable cause for the cardiac failure, cardiac failure, (3) absence of demonstrable heart disease prior to (3) absence of demonstrable heart disease prior to the last month of pregnancy, and the last month of pregnancy, and (4) documented left systolic dysfunction (4) documented left systolic dysfunction (echocardiography). (echocardiography). Standardized definition: Pearson Gail et all, JAMA.2000; 283: Standardized definition: Pearson Gail et all, JAMA.2000; 283:

PPCM Incidence  The incidence is not known - population-based estimates are not available  Incidence rates reported in individual studies are based on the experience at a particular institution and may reflect referral bias as well as individual practice patterns.  The accepted estimate of incidence is approximately 1 per 3,000 to 1 per 4,000 live births (translated to between 1,000 and 1,300 women affected each year in the United States) Pearson Gail et all, JAMA.2000;283; Pearson Gail et all, JAMA.2000;283;

PPCM Etiology  Unknown  Hypothesized etiological factors: - myocarditis (viral, autoimmune) - myocarditis (viral, autoimmune) - familial cardiomiopathy unmasked by the increased - familial cardiomiopathy unmasked by the increased cardiac demands of pregnancy, and selenium cardiac demands of pregnancy, and selenium deficiency deficiency - abnormal immune response to pregnancy - abnormal immune response to pregnancy - maladaptive response to the hemodynamic stresses of - maladaptive response to the hemodynamic stresses of pregnancy pregnancy - stress-activated cytokines - stress-activated cytokines - prolonged tocolysis - prolonged tocolysis Demakis JG. et all, Circulation. 1971; 44: Demakis JG. et all, Circulation. 1971; 44:

PPCM Risk factors classically identified in the literature  Advanced maternal age  African American race  Multifetal pregnancy  Multiparity  Pre-eclampsia and gestational hypertension Demakis JG. et all, Circulation. 1971; 44: Demakis JG. et all, Circulation. 1971; 44:

Prognosis for women with PPCM  Depends on the normalization of left ventricular size and function within 6 months after delivery  Half of women with PPCM can remain with persistent left ventricular dysfunction  Cardiac mortality rate is higher (85% over 5 years) in this group compared with those in whom cardiac size returned to normal  The risk for subsequent pregnancies depends on the severity of the cardiomiopathy and whether it reverses itself

Peripartum cardiomiopathy (PPCM) at Hospital A

Methodology (1) Potential cases identified by Hospital A:  Data source: Hospital discharge data  Diagnosis codes: Primary cardiomyopathy Primary cardiomyopathy  Additional codes: Complications of the puerperium, postpartum Complications of the puerperium, postpartum – Cardiovascular disease in the mother – Cardiovascular disease in the mother

Methodology (2)  Charts reviews conducted  The charts were provided by Hospital A epidemiologist and data were abstracted on site  No interviews were conducted with either women who experienced a peripartum cardiomiopathy or physicians

Results (1)  19 charts reviewed and 13 cases identified ( )  12 cases were diagnosed during postpartum  One PPCM case ended in a maternal death (2002)

Hospital A Live Births 2103 PPCM 1 Live Births 2198 PPCM 1 Live Births 2203 PPCM 1 Live Births 2319 PPCM 1 Live Births 2380 PPCM Live Births ? PPCM 5 Cluster * *National estimated incidence= 1/3000 – 1/4000 live births

Results (2) Cases Total Age Race White BlackUnknown Method of delivery Vaginal C-section C-section Others (e.g. forceps) Pregnancy outcomes Term Preterm Parity Primiparity Multiparity Pregnancy- related hypertension Y N449

Discussion and Conclusions (1)  Nine PPCM cases in 2001 and 2002 (69.23%)  The age distribution mirrors the age distribution of pregnancies in general  White/Black ratio=2  Only one multiple pregnancy (twin pregnancy)  Multiparity = 4 cases (44.44%)  Four cases had pregnancy-related hypertension ( 44.44%) ( 44.44%)  Four cases had fever but without infection confirmed by laboratory tests (44.44%)

Discussion and Conclusions (2)  Hospital A had a higher incidence of PPCM cases in 2001 and 2002 compared to the national estimated incidence  No cause identified  Number was too small to make any inferences

Discussion and Conclusions (3)  Findings presented to Ob/Gyns  Hospital A developed a tracking system of PPCM cases: no more cases recorded until June 2003  Develop PPCM registry

Discussion and Conclusion (4) Why to develop a registry? To prospectively capture all women with PPCM for: (1) better incidence and prevalence estimates, (1) better incidence and prevalence estimates, (2) determination of risk factors and prognostic (2) determination of risk factors and prognostic variables, variables, (3) ascertainment of cardiovascular risks for (3) ascertainment of cardiovascular risks for subsequent pregnancies, subsequent pregnancies, (4) establishment of a centralized serum and (4) establishment of a centralized serum and tissue bank to help facilitate identification of tissue bank to help facilitate identification of the cause of PPCM, and the cause of PPCM, and (5) evaluation of therapeutic interventions (5) evaluation of therapeutic interventions

Recommendations  Education provided to all professionals involved in the care of women of childbearing age  More timely recognition and diagnosis of PPCM Better prevention strategies Better prevention strategies  Postpartum education on family planning

Lessons learned Maternal morbidity as important as maternal mortality  Maternal death is the major indicator used to measure the maternal health  For every woman who dies, many suffer serious life-threatening complications of pregnancy  Maternal morbidity is a public health problem that affect nearly 1,7 million women annually (AJPH, 2003)

Healthy People 2010  16-4 Reduce maternal deaths ( 3.3/100, 000 live births) ( 3.3/100, 000 live births)  16-5 Reduction in maternal illness and complications complications 16-5a Maternal complications during hospitalized 16-5a Maternal complications during hospitalized labor and delivery (24/100 deliveries) labor and delivery (24/100 deliveries) 16-5b Ectopic pregnancies 16-5b Ectopic pregnancies 16-5c Postpartum complications, including 16-5c Postpartum complications, including postpartum depression postpartum depression

Public Health Implications  Potentially fatal health problems among PPCM cases highlight the need for improved long-term follow up of these women  Successful intervention to reduce maternal morbidity and its effects will require collaboration between clinicians, hospitals and public health professionals  Case reviews can be used as a MCH tool to improve this collaboration

Thank you for your attention!