Peripartum Cardiomyopathy

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Am J Ther. 2006 Jul-Aug;13(4):373-5.
Reversible peripartum cardiomyopathy in a patient with prior exposure to interferon.
Razaq W, Hyde P.
Department of Hematology and Oncology, Fellow St Luke's Roosevelt Hospital, New York, NY 10019, USA.

Peripartum cardiomyopathy creates complications for 1 in 3000 to 4000 pregnant women in the US. As this rare condition is associated with a high mortality rate (50% to 85%), it has been investigated to define the possible associated causes. Several factors including hypertension, nutritional and dietary discrepancies, and, recently, myocarditis are being implicated, but the mechanism of cardiac injury is yet to be discovered. Here we present an interesting case of possible interferon-induced reversible peripartum cardiomyopathy. The patient, with a diagnosis of chronic myelogenous leukemia, had been given interferon for 6 years. The therapy was discontinued when she became pregnant, and later she presented with symptoms of heart failure 6 weeks after her c-section. Interferon is an immunomodulating agent and used as an antiviral and an anticancer agent. Interferon-related dilated cardiomyopathy has been described as a rare side effect of the drug, the mechanism of which is unknown. There is compelling data supporting the fact that both peripartum cardiomyopathy and interferon-related cardiomyopathy are autoimmune disorders; so it is suggested that interferon therapy given in the past can have an additive effect in causing dilated cardiomyopathy. It is therefore advisable to follow closely those pregnant patients; who received interferon therapy in the past, for symptoms of cardiac failure, as there can be synergistic action between interferon and pregnancy causing dilated cardiomyopathy.

Minerva Cardioangiol. 2006 Jun;54(3):331-6.
Peripartum cardiomyopathy.
Kaaja R.
Department of Obstetrics and Gynecology, Helsinki University Hospital, Helsinki, Finland.

Peripartum cardiomyopathy (PPCM) is a rare cardiac disorder of unknown cause that occurs in pregnant females, most commonly in the early postpartum period. It shares many clinical characteristics with idiopathic dilated cardiomyopathy but occurs at a younger age and is associated with a better prognosis. The etiology and pathogenic mechanisms have been difficult to study, however current hypotheses include infectious, immunologic, nutritional, and iatrogenic causes. Diagnosis is based upon the clinical presentation of congestive heart failure and objective evidence of left ventricular systolic dysfunction. Mortality rates over the past 10 years in PPCM have decreased, most likely due to advances in medical therapy for heart failure and use of implantable defibrillators. For those patients, who remain refractory to conventional pharmacologic therapy, cardiac transplantation and mechanical circulatory support are viable options. In less than 50% of PPCM patients, left ventricular function normalizes with pharmacologic therapy. However, subsequent pregnancies are often associated with recurrence of left ventricular systolic dysfunction.

J Cardiol. 2006 May;47(5):261-6.
Peripartum cardiomyopathy with antiphospholipid antibody: a case report
Seo A, Sakamoto H, Tanaka Y, Mori C, Yagi H, Mochizuki S, Mikawa H.
Division of Cardiology, Department of Internal Medicine, The Jikei University School of Medicine, Daisan Hospital, Tokyo.

A 32-year-old woman tested positive for lupus anticoagulant when she had fever of unknown origin at 18 weeks of pregnancy. Sixteen days after a normal delivery at 35 weeks, she developed dyspnea and was hospitalized with heart failure. Chest radiography showed severe pulmonary edema. Echocardiography showed dilation and diffuse hypokinesis of the left ventricle. The diagnosis was peripartum cardiomyopathy. The patient responded to diuretic and vasodilator therapy. Endomyocardial biopsy revealed mild myocardial degeneration and interstitial fibrosis. Heart failure due to coronary microthrombosis has been indicated in patients with antiphospholipid antibodies, suggesting such a relationship in this case.

Eur Heart J. 2006 Feb;27(4):441-6. Epub 2005 Sep 5.
Peripartum cardiomyopathy: inflammatory markers as predictors of outcome in 100 prospectively studied patients.
Sliwa K, Forster O, Libhaber E, Fett JD, Sundstrom JB, Hilfiker-Kleiner D, Ansari AA.
Department of Cardiology, Chris Hani Baragwanath Hospital, University of the Witwatersrand, PO Bertsham 2013, Johannesburg, South Africa.

AIMS: Peripartum cardiomyopathy (PPCM) is a disorder of unknown aetiology with a course and outcome that is largely unpredictable. We evaluated the prognostic role of multiple inflammatory markers in the plasma of a large cohort of African patients with PPCM. METHODS AND RESULTS: The study of 100 patients with newly diagnosed PPCM was single-centred, prospective, and longitudinal. Clinical assessment, echocardiography, and blood analysis were done at baseline and after 6 months of standard therapy. Inflammatory markers were measured at baseline only. Fifteen patients died. Left ventricular ejection fraction (LVEF) improved from 26.2+/-8.2 to 42.9+/-13.6% at 6 months (P<0.0001). However, normalization of LVEF (>50%) was only observed in 23%. Baseline levels of C-reactive protein correlated positively with baseline LV end-diastolic (rs=0.33, P=0.0026) and end-systolic (rs=0.35, P=0.0012) diameters and inversely with LVEF (rs=-0.27, P=0.015). Patients who died presented with significantly lower mean EF and higher Fas/Apo-1 plasma values (P<0.05). Fas/Apo-1 and New York Heart Association functional class (NYHA FC) predicted mortality at baseline. CONCLUSION: Plasma markers of inflammation were significantly elevated and correlated with increased LV dimensions and lower LVEF at presentation. Baseline Fas/Apo-1 and higher NYHA FC were the only predictors of mortality. Normalization of LVEF was only observed in 23% of this African cohort.

Curr Opin Crit Care. 2005 Oct;11(5):435-48.
Severe cardiac disease in pregnancy, part II: impact of congenital and acquired cardiac diseases during pregnancy.
van Mook WN, Peeters L.
Department of Intensive Care and Internal Medicine, University Hospital Maastricht, Maastricht, Netherlands.

PURPOSE OF REVIEW: Part II of this review gives an overview of the different maternal cardiac problems during pregnancy and their management, and developments over recent years. RECENT FINDINGS: Many studies published over the last 5 years provided new insights on different cardiac diseases in pregnancy. Publications discussed in this part of the review on cardiac disease in pregnancy, for example, provide epidemiological data on heart disease during pregnancy in general, and cardiomyopathy and ischemic heart disease in particular. In addition, we discussed the implications of a history of peripartum cardiomyopathy for a subsequent pregnancy, interventional strategies during pregnancy in women with ischemic heart disease, and the role of echocardiography in the evaluation of cardiac disease in pregnancy. SUMMARY: The prevalence of the different causes of heart disease has shifted towards congenital heart disease by the end of the millennium. In developing countries, relatively rare diseases like rheumatic fever are still common, so these diseases are increasingly 'exported' to developed countries. The group of women with congenital heart disease represents most women with heart disease during pregnancy, followed by rheumatic heart disease. With the exception of patients with Eisenmenger's syndrome, pulmonary vascular obstructive disease, and Marfan's syndrome with aortopathy, maternal death during pregnancy is rare in women with heart disease. Although the risk for mortality is low in pregnant women with preexistent cardiac disease, these women are at increased risk for serious morbidity such as heart failure, arrhythmias, and stroke.

Crit Care Med. 2005 Oct;33(10 Suppl):S340-6.
Peripartum cardiomyopathy.
Murali S, Baldisseri MR.
University of Pittsburgh School of Medicine, Clinical Services of the Heart Failure Network, and Pulmonary Hypertension Program, Pittsburgh, PA, USA.

OBJECTIVE: To provide a review of the cardiac and obstetrical literature regarding the development of peripartum cardiomyopathy and, in particular, to examine risk factors, incidence, diagnosis, prognosis, and evidence-based treatment modalities. DESIGN: An extensive review of the current literature. RESULTS: Peripartum cardiomyopathy is a cardiomyopathy of unknown cause that occurs in pregnant females, most commonly in the early postpartum period. It shares many clinical characteristics with idiopathic dilated cardiomyopathy but occurs at a younger age and is associated with a better prognosis. Diagnosis is based upon the clinical presentation of congestive heart failure and objective evidence of left ventricular systolic dysfunction. Conventional pharmacologic therapy for congestive heart failure, such as diuretics, digoxin, angiotensin-converting enzyme inhibitors, angiotensin-receptor blockers, and beta-adrenergic blockers, are routinely used and are quite effective. For those patients who remain refractory to conventional pharmacologic therapy, cardiac transplantation and mechanical circulatory support are viable options. CONCLUSION: Mortality rates in peripartum cardiomyopathy have decreased, and this is most likely related to advances over the past 5 yrs in medical therapy for heart failure. Aggressive use of implantable defibrillators has significantly reduced the risk of sudden death in these patients. For >50% of peripartum cardiomyopathy patients, left ventricular function normalizes with pharmacologic therapy. However, subsequent pregnancies almost always are associated with recurrence of left ventricular systolic dysfunction.

Europace. 2005 Sep;7(5):433-9.
Sustained ventricular tachycardia induced by dobutamine stress echocardiography: a prospective study.
Katritsis DG, Karabinos I, Papadopoulos A, Simeonidis P, Korovesis S, Giazitzoglou E, Karvouni E, Voridis E.
Department of Cardiology, Athens Euroclinic, Greece.

AIMS: To investigate the prognostic significance and electrophysiological characteristics of dobutamine stress echo (DSE)-induced sustained monomorphic ventricular tachycardia (VT). METHODS: In our department, 3022 DSE studies were carried out on 2688 patients, aged 54.7 +/- 11.8 years, over a 3.5 year period. Patients with DSE-induced VT were studied by means of coronary angiography and electrophysiological testing, and were followed-up for 17.8 +/- 9.3 months. RESULTS: During DSE, nine patients (0.3%) developed sustained monomorphic VT. Four patients had coronary artery disease, one developed spontaneous right coronary artery (RCA) dissection during DSE, one patient had peripartum cardiomyopathy and the remainder had normal coronary arteries. Logistic regression analysis did not identify clinical parameters such as left ventricular ejection fraction, documentation of an ischaemic response or the presence of non-viable myocardial segments during DSE, that could predict the occurrence of DSE-induced VT. Monomorphic VT was inducible by electrophysiological testing in two patients with CAD and reduced LVEF. During follow-up, only these two patients developed VT. CONCLUSION: Sustained monomorphic VT is a rare complication of DSE, with no predictive value for the identification of patients with coronary artery disease and no prognostic significance in patients with normal coronary arteries. No predictors of its occurrence were identified.

Int J Gynaecol Obstet. 2005 Aug;90(2):161-6.
Unrecognized peripartum cardiomyopathy in Haitian women.
Fett JD, Christie LG, Carraway RD, Ansari AA, Sundstrom JB, Murphy JG.
Department of Adult Medicine, Hopital Albert Schweitzer, Deschapelles, Haiti.

OBJECTIVE: Haitian women have a high relative incidence of clinical presentation with peripartum cardiomyopathy (PPCM): an incidence estimated at one case per three hundred live births, a ten-fold occurrence compared to American women. Our objective has been to test the hypothesis that some Haitian women may have a forme fruste of PPCM while still without clinical symptoms. METHOD: A preliminary case-control study was conducted at the Hospital Albert Schweitzer (HAS), Deschapelles, Haiti, in which 25 apparently healthy postpartum women, without cardiovascular symptoms and with a normal cardiovascular clinical examination, were selected from a consecutive list of obstetrical deliveries and screened by echocardiography for left ventricular dysfunction. RESULT: Four out of 25 patients (16%) had asymptomatic left ventricular dysfunction that subsequently evolved towards either improvement or deterioration. Supporting evidence for the existence of asymptomatic left ventricular dysfunction or forme fruste PPCM is presented. A hypothetical schema of the pathophysiology of PPCM explains how a latent phase of variable duration may exist prior to onset of detectable clinical heart failure. CONCLUSION: Screening Haitian women during the last month of pregnancy or in the early postpartum period may help to detect asymptomatic left ventricular dysfunction. Early detection and treatment of PPCM in a known high risk population could lead to improvements in maternal and fetal mortality and morbidity.

Obstet Gynecol. 2005 Jun;105(6):1303-8.
Prognostic value of echocardiography in peripartum cardiomyopathy.
Chapa JB, Heiberger HB, Weinert L, Decara J, Lang RM, Hibbard JU.
Departments of Obstetrics and Gynecology and Internal Medicine, University of Chicago, Pritzker School of Medicine, Chicago, Illinois, USA.

OBJECTIVE: To estimate whether echocardiography findings at the time of diagnosis of peripartum cardiomyopathy are predictive of persistent cardiac dysfunction. METHODS: Chart review of patients with peripartum cardiomyopathy between 1988 and 2001 was performed. Data from echocardiography, including fractional shortening and left ventricular end diastolic dimension, were recorded both at the time of diagnosis and at follow-up. Left ventricular dysfunction was defined by echocardiography as fractional shortening less than 30% and left ventricular end diastolic dimension of 4.8 cm or more. RESULTS: Of 32 patients meeting our definition for peripartum cardiomyopathy and for whom follow-up data were available, 13 (41%) had recovery of ventricular function, while 19 (59%) continued to have persistent left ventricular dysfunction. Those who did not recover cardiac function had a higher left ventricular end diastolic dimension and a lower fractional shortening at diagnosis than those who recovered. A fractional shortening value less than 20% and a left ventricular end diastolic dimension 6 cm or greater at the time of diagnosis was associated with a more than 3-fold higher risk for persistent left ventricular dysfunction. CONCLUSION: Along with being an important diagnostic tool in peripartum cardiomyopathy, echocardiography may provide significant prognostic information with regards to recovery of cardiac function.

Arq Bras Cardiol. 2005 Feb;84(2):141-6. Epub 2005 Mar 8.
Clinical characteristics associated with unfavorable outcomes in peripartum myocardiopathy
Moreira Cda C, Zanati SG, Medeiros VT, Guimaraes C, Simoes EF, Matsubara BB.
Faculdade de Medicina de Botucatu, Unesp, Botucatu. SP.

OBJECTIVE: To assess the clinical characteristics of women with a previous diagnosis of peripartum myocardiopathy and to study the characteristics associated with unfavorable outcomes. METHODS: Clinical, obstetric, and echocardiographic variables were studied in 12 patients with peripartum myocardiopathy, assessed at diagnosis and at a current appointment, when they were divided into 2 groups: FG (n = 6, without cardiac alterations) and UG (n = 6, with cardiomegaly and persistent ventricular dysfunction). The comparisons were made using the Student t test and Fisher's exact test (P < 0.05). RESULTS: At diagnosis, mean age of the patients (8 Caucasian and 4 black/non-Caucasian) was 24+/-7.4 years, all in Functional Class IV (NYHA) and 8 reporting gestational hypertension or preeclampsia. Mean follow-up time was 25 months. Ten patients developed Functional Class I/II. Comparison between the groups demonstrated that UG had lower left ventricular ejection fractions (0.30+/-0.05 vs. 0.58+/-0.09; P < 0.001) and greater LV systolic diameter (58+/-5 mm vs. 46+/-3 mm; P < 0.001) at diagnosis. An unfavorable outcome was more frequent among non-Caucasian women (P = 0.01). In the current evaluation, UG had lower relative wall thickness (0.13+/-0.02 vs. 0.17+/-0.02; P < 0.05) and greater LV mass (283+/-90 g vs. 186+/-41 g; P < 0.05). CONCLUSION: Patients with previous peripartum myocardiopathy had unfavorable outcomes associated with black race, and stronger initial cardiac alterations; a favorable outcome was associated with a reduction in myocardial mass and an increase in relative ventricular wall thickness.

J Am Soc Echocardiogr. 2005 Jan;18(1):45-8.
Risk stratification of women with peripartum cardiomyopathy at initial presentation: a dobutamine stress echocardiography study.
Dorbala S, Brozena S, Zeb S, Galatro K, Homel P, Ren JF, Chaudhry FA.
Division of Cardiology, Department of Medicine, Hahnemann University Hospital, Philadelphia, Pennsylvania.

OBJECTIVES: We sought to determine the prognostic use of inotropic contractile reserve on risk stratification and prognostication of women with peripartum cardiomyopathy. BACKGROUND: Peripartum cardiomyopathy is a rare disorder effecting women in their prime years of life. There appears to be an initial high-risk period with 25% to 50% of women dying within the first 3 months postpartum. Early risk stratification and prognostication are, thus, crucial. However, only limited data are available. METHODS: In all, 7 women (mean age 31.8 years) with peripartum cardiomyopathy and severe left ventricular (LV) dysfunction (mean LV ejection fraction [LVEF] 25.3 +/- 9.5%) were studied. Of these, 6 underwent dobutamine stress echocardiography at baseline and a follow-up resting echocardiogram at a mean of 4.7 +/- 0.9 months after initial presentation. Resting and peak inotropic contractile reserve, and follow-up LVEF, were computed. RESULTS: The mean LVEF improved significantly from baseline (25.3 +/- 9.5%) to maximal inotropic contractile reserve (53.8 +/- 12.6%) (P = .0004) and at follow-up (53.0 +/- 16.4%) (P = .006). Importantly, LVEF at maximal inotropic contractile reserve and at follow-up (5.6 months) did not differ significantly (P = .5). The mean LVEF at maximal inotropic contractile reserve correlated well with the follow-up (LVEF R = 0.79). However, the baseline LVEF did not correlate with follow-up LVEF (P = not significant). CONCLUSIONS: In patients presenting with peripartum cardiomyopathy, inotropic contractile reserve during dobutamine stress echocardiography accurately correlates with subsequent recovery of LV function and confers a benign prognosis.

Kardiol Pol. 2005 Jan;62(1):49-51.
Peripartum cardiomyopathy--a case report
Knapp M, Lachota-Korecka G, Knapp P, Korecki J, Musial WJ.
Klinika Kardiologii Akademii Medycznej, Bialystok.

Peripartum cardiomyopathy -- a case report. A case of a 32-year old woman, previously healthy, with heart failure symptoms occurring during third pregnancy, is described. In spite of standard pharmacological treatment, her condition worsened and the pregnancy had to be terminated at 28 hbd by cesarean section. The patient's condition improved and three months later normal left ventricular function as well as good exercise tolerance were observed.

Tidsskr Nor Laegeforen. 2005 Jan 6;125(1):47-8.
Comment in: Tidsskr Nor Laegeforen. 2005 Jan 6;125(1):49.
Postpartum dyspnea, edema and fever
Roland MC, Lorentzen B, Gjertsen E.
Fodeseksjonen, Kvinneklinikken, Rikshospitalet, 0027 Oslo.

BACKGROUND: Cardiomyopathy is a condition with heart failure caused by a reduction in the contractility of the heart. Peripartum cardiomyopathy is a rare, but serious condition, characterised by development of heart failure during the last month of pregnancy or the first five months after delivery. MATERIAL AND METHODS: We present a case in which a woman pregnant with twins developed heart failure a few days after giving birth. We give a short report of symptoms, clinical signs, diagnosis, treatment and prognosis for subsequent pregnancies. RESULTS AND INTERPRETATION: The condition was initially diagnosed and treated as pneumonia, but despite treatment and improvement in her laboratory tests, her condition worsened. The symptoms were dyspnoea, peripheral oedemas and pulmonary oedema. Peripartum cardiomyopathy was diagnosed after echocardiography. Treatment of heart failure with diuretics, nitroglycerine, angiotensin-converting enzyme inhibitor and beta blocker was given with good results. This case is presented in order to draw attention to a rare, but serious condition in pregnancy or the postnatal period which easily can be misjudged or mistreated.

Curr Treat Options Cardiovasc Med. 2004 Dec;6(6):481-488.
Peripartum Cardiomyopathy: Current Therapeutic Perspectives.
Phillips SD, Warnes CA.
Division of Cardiovascular Diseases.

Peripartum cardiomyopathy is a rare condition of unclear etiology that accounts for an important percentage of pregnancy-related deaths. Deaths from peripartum cardiomyopathy can be attributed to profound left ventricular failure, thromboembolic events, or arrhythmia. Prompt recognition of the condition, initiation of appropriate medical management, collaboration with perinatology for delivery management, referral to cardiac transplant centers when necessary, and counseling regarding future pregnancies is required for a successful outcome. Patients should be diagnosed by clinical evaluation and echocardiography. After establishing left ventricular dysfunction, a standard heart failure medical regimen should be instituted. Hospitalization should be considered for patients with class III or greater symptoms, or for those patients not responding to outpatient management. If the diagnosis is made in the antepartum period, delivery should be strongly considered. Endomyocardial biopsy has low yield in this situation and should not be considered standard care, especially because controversy exists over the effectiveness of immunosuppressive therapy for myocarditis. Selenium, pentoxifylline, and immune globulin have all been shown to have a beneficial effect in small series of patients. The addition of these agents to standard therapy, however, should be considered on a case-by-case basis. Anticoagulation should be considered in patients with ejection fractions less than 35%. Transplantation results in survival comparable to women with idiopathic-dilated cardiomyopathy, and should be pursued in the appropriate setting. Future pregnancies should be discouraged, even if the left ventricular function recovers. Significant improvement in ventricular function can be expected in up to 50% of patients.

An Med Interna. 2004 Oct;21(10):498-500.
Peripartum cardiomyopathy with biventricular thrombi
Sanchez-Rubio Lezcano J, Galache Osuna JG, Marquina Barcos A, Calvo Cebollero I, Diarte de Miguel JA, Placer Peralta LJ.
Servicio de Cardiologia, Hospital Universitario Miguel Servet, Zaragoza, Spain.

Peripartum cardiomyopathy is a rare cause of congestive heart failure which develops in the last month of pregnancy or during five months postpartum in women without previously known cardiac disease. Intraventricular thrombi are no unusual and can be identified by echocardiography. We report the case of a woman with peripartum cardiomyopathy whose echocardiographic study showed the presence of biventricular thrombi. This is a quite rare finding with only two cases reported in the literature. Diagnosis, treatment and special situations that could predispose to intracardiac thrombi formation are discussed.

Neth J Med. 2004 Sep;62(8):290-2.
Congestive heart failure in pregnancy: a case of peripartum cardiomyopathy.
Rosner G, Wolchock Rosner SN, Heller I, Topilsky M.
Department of Internal Medicine H, Tel-Aviv Sourasky Medical Centre, Affiliated to Sackler School of Medicine, Tel-Aviv University, 6 Weizmann Street, Tel Aviv, Israel.

A healthy 28-year-old woman developed full-blown pulmonary oedema in the 36th week of gestation. Echocardiography revealed a globally enlarged heart with reduced systolic function. A remarkable dinical response with regain of normal ventricular function was noted with early medical intervention. This case report illustrates peripartum cardiomyopathy, a unique form of dilated cardiomyopathy affecting women during/following gestation. Clinician familiarity with this entity increases the probability of prompt appropriate treatment, offering patients the best possible prognosis.

Br J Anaesth. 2004 Sep;93(3):428-39. Epub 2004 Jun 11.
Recognition and management of maternal cardiac disease in pregnancy.
Ray P, Murphy GJ, Shutt LE.
Department of Anaesthesia, St Michaels Hospital, Bristol and Department of Cardiac Surgery, Bristol Royal Infirmary, Bristol BS2 8HW, UK.

Heart disease is a leading cause of maternal death. The aim of this study is to review the most common causes of cardiac disease, highlight factors that should be recognized by the clinician, and address recent advances in the anaesthetic management of these patients. Incipient cardiac disease, including peripartum cardiomyopathy, myocardial infarction and aortic dissection, accounts for approximately one in six maternal deaths. The keys to successful diagnosis and management of incipient disease are: a high index of suspicion, particularly in women with known risk factors for cardiovascular disease; a low threshold for radiological investigations; early cardiology input; and invasive monitoring during labour and delivery. Echocardiography is a safe, non-invasive test, under-used in pregnancy. Management of pregnant women with pre-existing cardiac problems should be undertaken by multidisciplinary teams in tertiary centres. In women with pre-existing cardiac disease wishing to proceed to term, cardiac status must be optimized preoperatively and planned elective delivery is preferable. Vaginal delivery is preferable, and with careful incremental regional anaesthesia is safe in most women with cardiac disease. The presence of adequate systems for early detection, appropriate referral to specialist centres, and timely delivery with multidisciplinary support can minimize the serious consequences of poorly controlled heart disease in pregnancy.

Am J Cardiol. 2004 Jun 1;93(11):1441-3, A10.
Outcome of subsequent pregnancy in patients with documented peripartum cardiomyopathy.
Sliwa K, Forster O, Zhanje F, Candy G, Kachope J, Essop R.
Department of Cardiology, Chris Hani Baragwanath Hospital, University of the Witwatersrand, Bertsham 2013, Johannesburg, South Africa.

Subsequent pregnancy in 6 patients with previous peripartum cardiomyopathy resulted in reduction of ejection fraction by >10% in 5 patients at 1 month postpartum. Two patients with impaired ejection fraction at onset of subsequent pregnancy died 3 months postpartum due to heart failure despite optimal medical therapy. Deterioration of left ventricular function occurred uniformly postpartum and was accompanied by elevation of tumor necrosis factor-alpha plasma levels from 2.4 +/- 1.1 pg/ml at onset of subsequent pregnancy to 6.2 +/- 2.4 pg/ml at 1 month postpartum.

Int J Obstet Anesth. 2004 Jan;13(1):40-3.
Continuous spinal anesthesia for cesarean section in a parturient with severe recurrent peripartum cardiomyopathy.
Velickovic IA, Leicht CH.
Department of Anesthesiology, Western Pennsylvania Hospital, Pittsburgh, PA, USA.

The anesthetic management of labor and delivery in patients with peripartum cardiomyopathy is not well defined. Using continuous spinal anesthesia in such a rare clinical situation has not been previously reported. A patient with recurrent peripartum cardiomyopathy presented in congestive heart failure for emergent cesarean section. Continuous spinal anesthesia was successfully employed as the anesthetic technique for the procedure. In addition, it also markedly reduced the patient's symptoms. Continuous spinal anesthesia is a reliable, rapidly titratable technique, which provides excellent analgesia with minimal undesirable hemodynamic changes for patients with peripartum cardiomyopathy undergoing cesarean delivery.

Ceska Gynekol. 2003 Mar;68(2):111-4.
Obstetrical aspects of peripartum cardiomyopathy
Koucky M, Binder T, Krejci V, Zivny J.
Gynekologicko-porodnicka klinika VFN a 1. LF UK v Praze.

OBJECTIVE: The authors demonstrate a case report of the peripartum cardiomyopathy that occurred at a so far healthy woman after the delivery by the caesarean section. SUBJECT: Case report. SETTING: Department of Obstetrics and Gynecology, 1st Medical Faculty, Charles University and General Faculty Hospital, Prague. METHODS: The gravidity of a 28-year old primipara, gemini, was finished by the caesarean section because of cardiologic (benign cardiac extrasystoles) and obstetric (gemini clashing position) indication. After the delivery a heart failure started to develop. The diagnosis was closed as peripartum cardiomyopathy. The clinical state of patient stabilized after initiating adequate therapy, the follow up has been still going on. RESULTS: Peripartum cardiomyopathy is a rare disease that occurs as a left ventricular cardiac failure in pregnant women or in woman in childbed. Etiology of the disease is unknown, the disease management requires collaboration among obstetricians, cardiologists, anesthesiologists and neonatologists. Prognosis is uncertain.

Angiology. 2001 Nov;52(11):759-62.
Peripartum cardiomyopathy: clinical and therapeutic aspects.
Mehta NJ, Mehta RN, Khan IA.
Department of Internal Medicine, Long Island College Hospital, Brooklyn, NY, USA.

Peripartum cardiomyopathy is an under-recognized form of dilated cardiomyopathy of unknown cause and is associated with excess morbidity and mortality in women of childbearing age. Incidence of peripartum cardiomyopathy ranges from 1 in 1,300 to 1 in 15,000 pregnancies. The diagnostic criteria are onset of heart failure in the last month of pregnancy or in first 5 months postpartum, absence of determinable cause for cardiac failure, and absence of a demonstrable heart disease before the last month of pregnancy. Multiparity, twin births, advanced maternal age, preeclampsia, gestational hypertension, and black race are known risk factors. The exact cause of peripartum cardiomyopathy is unclear. The clinical presentation of patients with peripartum cardiomyopathy is similar to that of patients with systolic congestive heart failure. Little is known about the therapy of this disease, and the medical treatment is limited to the use of drugs for symptomatic control. Prospective, randomized, double-blind studies are needed to define the role of immunosuppressive treatment and to evaluate the role of conventional treatment of dilated cardiomyopathy in reducing the mortality rate of peripartum cardiomyopathy. About half the patients of peripartum cardiomyopathy recover without any complications. The prognosis is poor in patients with persistent cardiomyopathy. Persistence of disease after 6 months indicates irreversible cardiomyopathy and portends worse survival. The risk of developing peripartum cardiomyopathy in subsequent pregnancies remains high. The clinical and therapeutic aspects of the peripartum cardiomyopathy are discussed.

N Engl J Med. 2001 May 24;344(21):1567-71.
Erratum in: N Engl J Med 2001 Aug 16;345(7):552.
Comment in: N Engl J Med. 2001 May 24;344(21):1629-30.
Maternal and fetal outcomes of subsequent pregnancies in women with peripartum cardiomyopathy.
Elkayam U, Tummala PP, Rao K, Akhter MW, Karaalp IS, Wani OR, Hameed A, Gviazda I, Shotan A.
Department of Medicine, University of Southern California School of Medicine, Los Angeles 90033, USA.

BACKGROUND: Peripartum cardiomyopathy is a rare and sometimes fatal form of heart failure. Little is known about the outcomes of subsequent pregnancies in women who have had the disorder. METHODS: Through a survey of members of the American College of Cardiology, we identified 44 women who had had peripartum cardiomyopathy and had a total of 60 subsequent pregnancies. We then reviewed the medical records of these women and interviewed the women or their physicians. RESULTS: Among the first subsequent pregnancies in the 44 women, 28 occurred in women in whom left ventricular function had returned to normal (group 1) and 16 occurred in women with persistent left ventricular dysfunction (group 2). The pregnancies were associated with a reduction in the mean (+/-SD) left ventricular ejection fraction both in the total cohort (from 49+/-12 percent to 42+/-13 percent, P<0.001) and in each group separately (from 56+/-7 percent to 49+/-10 percent in group 1, P=0.002; and from 36+/-9 percent to 32+/-11 percent in group 2, P=0.08). During these pregnancies, a decrease of more than 20 percent in the left ventricular ejection fraction occurred in 21 percent of the women in group 1 and 25 percent of those in group 2, and symptoms of heart failure occurred in 21 percent of the women in group 1 and 44 percent of those in group 2. The mortality rate was 0 percent in group 1 and 19 percent in group 2 (P=0.06). In addition, the frequency of premature delivery was higher in group 2 (37 percent vs. 11 percent), as was that of therapeutic abortions (25 percent vs. 4 percent). CONCLUSIONS: Subsequent pregnancy in women with a history of peripartum cardiomyopathy is associated with a significant decrease in left ventricular function and can result in clinical deterioration and even death.

Am Heart J. 2000 Nov;140(5):785-91.
Myocarditis and long-term survival in peripartum cardiomyopathy.
Felker GM, Jaeger CJ, Klodas E, Thiemann DR, Hare JM, Hruban RH, Kasper EK, Baughman KL.
Department of Medicine, Division of Cardiology, and the Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD 21287, USA.

BACKGROUND: The reported mortality rate of peripartum cardiomyopathy (PPCM) is high, although the potential for spontaneous recovery of ventricular function is well established. The prevalence of myocarditis in PPCM has varied widely between studies. The purposes of this study were to define the long-term prognosis in a referral population of patients with PPCM, to determine the prevalence of myocarditis on endomyocardial biopsy in this population, and to identify clinical variables associated with poor outcome. METHODS: We analyzed clinical, echocardiographic, hemodynamic, and histologic features of 42 women with PPCM evaluated at our institution over a 15-year period. Each patient underwent an extensive evaluation, including echocardiography, endomyocardial biopsy, and right heart catheterization. Data were analyzed to identify features at initial examination associated with the combined end point of death or cardiac transplantation by the use of Kaplan-Meier survival curves and a Cox proportional hazards model. RESULTS: Three (7%) patients died and 3 (7%) patients underwent heart transplantation during a median follow-up of 8.6 years. Endomyocardial biopsy demonstrated a high prevalence of myocarditis (62%), but the presence or absence of myocarditis was not associated with survival. Of the prespecified variables assessed, only decreased left ventricular stroke work index was associated with worsened outcome. CONCLUSIONS: In patients with PPCM, (1) long-term survival is better than has been historically reported, (2) the prevalence of myocarditis is high, and (3) decreased left ventricular stroke work index is associated with worse clinical outcomes.

Dakar Med. 2000;45(2):199-201.
Biventricular thrombus complicating peripartum cardiomyopathy. A case report
Damorou FJ, Kane A, Napporn G, Thiam O, Bidani A, Diop IB, Sarr M, Ba SA, Diouf SM.
Clinique Cardiologique CHU A. Le Dantec, Dakar, Senegal.

The authors report a case of a biventricular thrombus complicating peripartum cardiomyopathy in a 38 years old female. The diagnosis was done by bidimensionnal transthoracic echocardiography which showed 2 thrombi in the apical region of the right ventricle and in the anterior and lateral wall of the left ventricle. With treatment associating salt restriction, diuretics and angiotensin-converting-enzyme- inhibitors, the evolution was good: the thrombi disappeared at the first month for the left ventricular thrombi and after 45 days for the rignt ventricular thrombi. The patient didn't experience any embolic complication.

Dakar Med. 2000;45(2):131-3.
Peripartum heart failure: the underestimated role of frequent diseases in the Sudan-Sahelian area
Kane A, Dia AA, Diop IB, Sarr M, Ba SA, Diouf SM.
Clinique Cardiologique, HALD - BP 3001.

Peripartum cardiomyopathy is rare in developped countries, but still frequent in Africa. It is defined as a heart failure occurring during peripartum, without any underlying etiology. Authors present 3 cases showing that heart failure before or after delivery may be due to causes which are frequent in the Sahelian area but generally misdiagnosed. Anemia, hypertension and rheumatic fever were the causes of heart failure in these 3 patients, but they were not apparent when the initial diagnosis was made. These observations emphasize that, despite the complex hypothesis trying to explain heart failure during the peripartum period, one should think about some frequent causes which can be misdiagnosed because of the pregnant state or the heart failure itself.

JAMA. 2000 Mar 1;283(9):1183-8.
Peripartum cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review.
Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, Ansari A, Baughman KL.
Division of Heart and Vascular Diseases, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892-7940, USA.

OBJECTIVE: Peripartum cardiomyopathy (PPCM) is a rare life-threatening cardiomyopathy of unknown cause that occurs in the peripartum period in previously healthy women. In April 1997, the National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases of the National Institutes of Health (NIH) convened a Workshop on Peripartum Cardiomyopathy to foster a systematic review of information and to develop recommendations for research and education. PARTICIPANTS: Fourteen workshop participants were selected by NHLBI staff and represented cardiovascular medicine, obstetrics, immunology, and pathology. A representative subgroup of 8 participants and NHLBI staff formed the writing group for this article and updated the literature on which the conclusions were based. The workshop was an open meeting, consistent with NIH policy. EVIDENCE: Data presented at the workshop were augmented by a MEDLINE search for English-language articles published from 1966 to July 1999, using the terms peripartum cardiomyopathy, cardiomyopathy, and pregnancy. Articles on the epidemiology, pathogenesis, pathophysiology, diagnosis, treatment, and prognosis of PPCM were included. RECOMMENDATION PROCESS: After discussion of data presented, workshop participants agreed on a standardized definition of PPCM, a general clinical approach, and the need for a registry to provide an infrastructure for future research. CONCLUSIONS: Peripartum cardiomyopathy is a rare lethal disease about which little is known. Diagnosis is confined to a narrow period and requires echocardiographic evidence of left ventricular systolic dysfunction. Symptomatic patients should receive standard therapy for heart failure, managed by a multidisciplinary team. If subsequent pregnancies occur, they should be managed in collaboration with a high-risk perinatal center. Systematic data collection is required to answer important questions about incidence, treatment, and prognosis.

Eur J Obstet Gynecol Reprod Biol. 1998 Jan;76(1):29-30.
Recurrent peripartum cardiomyopathy.
Ceci O, Berardesca C, Caradonna F, Corsano P, Guglielmi R, Nappi L.
Institute of Obstetrics and Gynaecology II, University of Bari, Italy.

Peripartum cardiomyopathy (PPCM) is an uncommon myocardial disease arising in the latter part of pregnancy or during the first five postpartum months, in the absence of any obvious cause and with no previously known heart disease. The risk of recurrence of PPCM is considered low when left ventricular size and function return to normal: We illustrate a case of peripartum cardiomyopathy recurred in subsequent pregnancy despite the rapid return to normal of heart size and function.

J Heart Lung Transplant. 1997 Aug;16(8):801-12.
The incremental risk of female sex in heart transplantation: a multiinstitutional study of peripartum cardiomyopathy and pregnancy. Cardiac Transplant Research Database Group.
Johnson MR, Naftel DC, Hobbs RE, Kobashigawa JA, Pitts DE, Levine TB, Tolman D, Bhat G, Kirklin JK, Bourge RC.
Rush Medical College, Chicago, Illinois, USA.

BACKGROUND: Controversy remains regarding the reason females seem to be at increased risk for rejection after heart transplantation. Therefore this study was performed to define the effect of a pretransplantation diagnosis of peripartum cardiomyopathy and the effect of previous pregnancy on the outcome (incidence of rejection and death) of females after heart transplantation. METHODS: In this multiinstitutional study of 3244 adult (greater than 13 years of age) heart transplant recipients, (a) the outcome of 40 females who underwent transplantation for peripartum cardiomyopathy was compared with that of 200 females of childbearing age (13 to 45 years) who underwent transplantation for other indications and (b) the posttransplantation outcome of 543 females with a history of pregnancy was compared with that of 101 nulliparous adult females and 2562 adult males. RESULTS: The posttransplantation outcome of females with a history of peripartum cardiomyopathy was similar to that of females of childbearing age who underwent transplantation for other indications. However, parous females had a significantly shorter time to first rejection (p < 0.0001) and greater cumulative rejection than nulliparous females or males. By multivariable analysis, the risk factors for cumulative rejection at 1 year were a history of pregnancy (p < 0.0001), younger recipient age (p < 0.0001), induction therapy (p < 0.0001), and the number of human leukocyte antigen-DR mismatches (p = 0.007). CONCLUSION: Our data suggest that it is previous pregnancy, and not sex per se, that is associated with an increased frequency of rejection in females after heart transplantation.

Am J Obstet Gynecol. 1997 Jan;176(1 Pt 1):189-95.
Contractile reserve in patients with peripartum cardiomyopathy and recovered left ventricular function.
Lampert MB, Weinert L, Hibbard J, Korcarz C, Lindheimer M, Lang RM.
Department of Medicine, University of Chicago Medical Center, IL 60637, USA.

OBJECTIVES: Peripartum cardiomyopathy is a rare complication of pregnancy. Thirty percent of patients with this disorder are reported to recover baseline ventricular function within 6 months of delivery, but the ability of these ventricles to respond to hemodynamic stress is unknown. The aim of this investigation was to quantitatively assess the contractile reserve of patients with a history of peripartum cardiomyopathy and recovered left ventricular function. STUDY DESIGN: Baseline left ventricular contractility was assessed by use of the load and heart rate-independent relationship between end-systolic stress and rate-corrected velocity of fiber shortening. Data were acquired from "recovered" patients (10.5 +/- 11.6 months after delivery) and compared with data from matched nonpregnant controls with use of two-dimensionally targeted M-mode echocardiography and calibrated subclavian pulse tracings that were recorded over a wide range of afterloads (end-systolic stress) generated by methoxamine (1 mg/min) infusion. Contractile reserve was assessed by a dobutamine challenge (5 micrograms/kg/min) and quantified as the vertical deviation of the dobutamine end-systolic stress minus the corrected velocity of fiber shortening data point from the baseline contractility line. RESULTS: Patients with peripartum cardiomyopathy and matched controls had normal baseline heart rates, blood pressures, ventricular dimensions, and left ventricular function. Contractile reserve, however, was reduced in patients with recovered peripartum cardiomyopathy (0.30 +/- 0.12 vs 0.17 +/- 0.04 circ/sec, p < 0.03). CONCLUSIONS: Women with a history of peripartum cardiomyopathy who have regained normal resting left ventricular size and performance have decreased contractile reserve revealed by the use of a dobutamine challenge test. Ventricles of these women may respond suboptimally to hemodynamic stress in spite of evidence of recovery by routine echocardiographic evaluation.

Am Heart J. 1995 Oct;130(4):860-70.
Comment in: Am Heart J. 1996 Dec;132(6):1320.
Peripartum cardiomyopathy.
Lampert MB, Lang RM.
Department of Medicine, University of Chicago Hospitals, IL 60637, USA.

Congestive heart failure is an uncommon complication of pregnancy with potentially life-threatening consequences. Peripartum cardiomyopathy is a disease of unknown cause in which severe left ventricular dysfunction occurs during late pregnancy or the early puerperium. In the past, the diagnosis of this entity was made on clinical grounds; however, modern echocardiographic techniques have allowed more accurate diagnoses by excluding cases of diseases that mimic the clinical symptoms and signs of heart failure. Risk factors for peripartum cardiomyopathy include advanced maternal age, multiparity, African descent, twinning, and long-term tocolysis. An extensive search for the causes of peripartum cardiomyopathy has been unrevealing. Treatment includes digitalis, diuretic agents, and vasodilators. Anticoagulation is strongly recommended, especially if ventricular function is persistent. The prognosis of peripartum cardiomyopathy is related to the recovery of ventricular function. Caution is advised in recommending subsequent pregnancy, especially if left ventricular dysfunction is persistent.

Int J Cardiol. 1992 Jul;36(1):57-9.
A low plasma selenium is a risk factor for peripartum cardiomyopathy. A comparative study in Sahelian Africa.
Cenac A, Simonoff M, Moretto P, Djibo A.
Service de Medecine Interne (P. Bene), CHRU Morvan, Brest, France.

A specific selenium deficiency is a risk factor for Keshan disease, an endemic cardiomyopathy observed in China. In a Sahelian area of Niger, plasma selenium concentration was measured by neutronic activation and particle induced X-ray emission in 35 black African women with peripartum cardiomyopathy and 36 breast-feeding women without cardiac failure as controls. The plasma selenium concentration in patients was lower (48 +/- 25 ng/ml, mean +/- standard deviation) than in controls (77 +/- 16 ng/ml) (P less than 0.0001). Moreover, 40% (14/35) patients with peripartum cardiomyopathy had very low plasma selenium concentrations, below 45 ng/ml, versus none in controls. A low plasma selenium concentration is a risk factor for the Sahelian peripartum cardiomyopathy.

Int J Cardiol. 1991 Sep;32(3):377-80.
Comment in: Int J Cardiol. 1992 May;35(2):283-4.
Peripartum cardiomyopathy: prognostic variables at initial evaluation.
Ravikishore AG, Kaul UA, Sethi KK, Khalilullah M.
Department of Cardiology, G.B. Pant Hospital, New Delhi, India.

Twenty patients with peripartum cardiomyopathy were followed up for a period ranging from 6-14 months (mean 6 +/- 2 months). At initial evaluation, 16 patients were in New York Heart Association Class IV and the remainder in Class III. During follow up, 12 patients improved to Class I, 7 patients either failed to improve or deteriorated and one patient died. Certain variables at initial evaluation were related to prognosis. The patients who deteriorated, as compared to those who improved, were significantly older (30 +/- 6.8 vs 24 +/- 3 years, P less than 0.01), of higher parity (3 +/- 1 pregnancies vs 1.5 +/- 5 pregnancies, P less than 0.001) and had later onset of symptoms after delivery (7.6 +/- 4 weeks vs 3 +/- 1.3 weeks, P less than 0.001). They also had higher echocardiographic left ventricular end diastolic dimensions (7.0 +/- 8.4 cm vs 3.0 +/- 0.8 cm, P less than 0.001) and higher mean pulmonary arterial (38 +/- 4 mmHg vs 28 +/- 6 mmHg, P less than 0.001) and pulmonary arterial wedge pressures (24 +/- 2 mmHg vs 20 +/- 2 mmHg, P less than 0.001) at cardiac catheterization. Conduction defects were present on the surface electrocardiogram in all the patients who deteriorated, as compared to 4 patients who improved. In conclusion, certain variables at initial evaluation can help in identifying high risk subsets with peripartum cardiomyopathy.

Am Heart J. 1991 Jun;121(6 Pt 1):1776-8.
Effects of subsequent pregnancy on left ventricular function in peripartum cardiomyopathy.
Sutton MS, Cole P, Plappert M, Saltzman D, Goldhaber S.
Department of Medicine, Brigham and Womens' Hospital, Harvard Medical School, Boston, Mass.

Pregnancy has been discouraged in patients with peripartum cardiomyopathy (PPCM) to avoid the risk of precipitating recurrent or progressive left ventricular dysfunction. We assessed left ventricular size and contractile function using echocardiography in four PPCM patients prior to pregnancy, during the third trimester, and a mean of 6 weeks postpartum. Left ventricular mean diameters at end diastole and at end systole prior to pregnancy (5.2 +/- 0.3 and 3.0 +/- 0.2 cm, respectively) did not change during pregnancy (5.2 +/- 0.3 and 3.1 +/- 0.2 cm). Similarly, left ventricular fractional shortening did not alter significantly during pregnancy or postpartum. Furthermore, no patient developed any symptoms or signs of left ventricular failure. All patients had normal babies, including one who had twins. We conclude that PPCM patients whose left ventricular function returns to normal may undertake further pregnancy with a normal fetal outcome and a low risk of recurrent left ventricular dysfunction.

Am Heart J. 1991 Jan;121(1 Pt 1):134-40.
High output failure in patients with peripartum cardiomyopathy: a comparative study with dilated cardiomyopathy.
Marin-Neto JA, Maciel BC, Urbanetz LL, Gallo Junior L, Almeida-Filho OC, Amorim DS.
Cardiac Catheterization Laboratory, Hospital de Clinicas, Medical School of Ribeirao Preto, Sao Paulo, Brazil.

Although few studies have reported on relatively preserved ventricular function in patient with peripartum cardiomyopathy, the condition is usually believed to have the typical low-output congestive hemodynamic pattern of the dilated congestive cardiomyopathies. Two groups of patients, 14 with peripartum cardiomyopathy and 12 with dilated congestive cardiomyopathy who were matched for gender and age, were studied. They had normal blood pressure and similar New York Heart Association functional class, nutritional status, thyroid function and routine laboratory evaluation. All patients were catheterized during stable in-hospital compensation of heart failure, which was achieved by bed rest, sodium restriction, and administration of digoxin and diuretics long (more than 3 months) after delivery. Significant differences (p less than 0.05) between patients with peripartum cardiomyopathy and those with dilated congestive cardiomyopathy were observed in regard to: (1) cardiac index: 3.34 +/- 1.36 L/min/m2 versus 2.24 +/- 0.72 L/min/m2, (2) systemic vascular resistance: 1713 +/- 567 versus 2194 +/- 603, (3) right ventricular stroke work index: 8.6 +/- 4.2 g.M/m2 versus 14.8 +/- 8.2 g.M/m2 in the peripartum cardiomyopathy and the dilated congestive cardiomyopathy groups, respectively. Three of the patients with peripartum cardiomyopathy had resting cardiac index values that were even higher than the normal upper limit for our laboratory (4.5 L/min/m2): 4.80, 5.70, and 5.63 L/min/m2. They also had nearly normal left ventricular ejection fractions: 0.68, 0.41, and 0.51, respectively. These results indicate that, unlike the common dilated cardiomyopathy, the hemodynamic pattern in patients with peripartum cardiomyopathy is not homogeneous, and some patients have high-output failure and near normal left ventricular function.

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