AIM: Subsequent pregnancies (SSPs) in patients with peripartum cardiomyopathy (PPCM) have a high risk of heart failure relapse. We report on outcome of SSPs in PPCM patients in South Africa. METHODS AND RESULTS: Of the 18 PPCM patients with a SSP, 3 patients died within 6-months follow-up. Overall relapse rate, left ventricular ejection fraction (LVEF) <50% or death after at least 6 months follow-up, was 30%, with 16% (3/18) mortality. Persistently reduced LVEF (<50%) before entering SSP was present in 44% of patients, while full recovery (LVEF≥ 50%) was present in 85%. Persistently reduced LVEF before SSP was associated with a higher mortality (27% vs 0%) and a lower rate of full recovery at follow-up. Patients obtaining standard therapy for heart failure and bromocriptine immediately after delivery displayed significantly better LVEF at follow-up and a higher rate of full recovery, with no patient dying, compared with patients obtaining standard therapy for heart failure alone. CONCLUSION: Full recovery of LVEF before SSP was associated with lower mortality and better cardiac function at follow-up. Addition of bromocriptine to standard therapy for heart failure immediately after delivery was safe and appeared to be associated with better outcome of SSP in our patients.

Background: Peripartum cardiomyopathy (PPCM) is an incompletely understood disease that causes significant morbidity and mortality in many parts of the world, including Northern Nigeria. The aims of this Thesis were: [1] to determine if selenium deficiency, serum ceruloplasmin and traditional birth practices are risk factors for PPCM, in Kano, Nigeria; [2] to describe the one year survival and left ventricular reverse remodeling (LVRR) in a group of patients with PPCM from three referral hospitals in Kano, Nigeria; [3] to identify potential electrocardiographic (ECG) predictors of PPCM; and [4] to assess right ventricular systolic dysfunction (RVSD) and remodelling in a cohort of PPCM patients in Kano, Nigeria. Materials and Methods: The studies were carried out in 3 referral hospitals in Kano, Nigeria. Study 1: This was a case-control study. Critically low serum selenium concentration was defined as &lt;70μg/L. Study 2: This was a longitudinal study. LVRR was defined as absolute increase in LV ejection fraction (LVEF) by ≥10.0% and decrease in LV end-diastolic dimension indexed to body surface area (LVEDDi) ≤33.0 mm/m2, while recovered LV systolic function as LVEF ≥55%, at 12 months follow-up. Study 3: This was a case-control study. Logistic regression models and a risk score were developed to determine ECG predictors of PPCM. Study 4: This was a longitudinal study and patients were followed up for 12 months. RVSD was defined as the presence of either tricuspid annular plane systolic excursion (TAPSE) &lt;16mm or peak systolic wave (S’) tissue Doppler velocity of RV free wall &lt;10cm/s. Recovery of RV systolic function was defined as an improvement of reduced TAPSE to ≥16mm or S’ to ≥10cm/s, without falling to reduced levels again, during follow-up. Results: Study 1: Total of 39 PPCM patients and 50 controls were consecutively recruited after satisfying the inclusion criteria. Mean serum selenium in patients (61.7±14.9μg/L) was significantly lower than in controls (118.4±45.6μg/L) (p&lt;0.001). The prevalence of serum selenium &lt;70μg/L was significantly higher among patients (76.9%) than controls (22.0%) (p&lt;0.001). The mean ceruloplasmin and prevalence of socio-economic indices, multiparity, pregnancy-induced hypertension, obesity and twin pregnancy were not different between the groups (p&gt;0.05). Logistic regression showed that rural residency significantly increased the odds for serum selenium &lt;70μg/L by 2.773 fold (p=0.037). Study 2: A total of 33 patients were followed-up. Of the 17 survivors at 12 months, 8 patients (47.1%) satisfied the criteria for LVRR, of whom 5 (29.4%) had recovered LV systolic function, but LVRR was not predicted by any variable in the regression models. The prevalence of normal LV diastolic function increased from 11.1% at baseline to 35.3% at twelve months (p=0.02). At one year follow-up, 41.4% of patients had died (two thirds of them within the first 6 months), but mortality wasn’t predicted by any variable including LVRR. Study 3: A total of 54 PPCM and 77 controls were studied. A rise in heart rate by 1 beat/minute increased the odds of PPCM by 6.4% (p=0.001), while presence of ST-T-wave changes increased the odds of PPCM by 12.06 fold (p&lt;0.001). In patients, QRS duration modestly correlated (r=0.4; p&lt;0.003) with LV dimensions and end-systolic volume index (LVESVI), and was responsible for 19.9% of the variability of the latter (R2 = 0.199; p=0.003). A risk score of ≥2 had a sensitivity of 85.2%, specificity of 64.9%, negative predictive value of 86.2% and area under the curve of 83.8% (p&lt;0.0001) for potentially predicting PPCM. Study 4: A total of 45 patients were studied. RV systolic function recovery occurred in a total of 8 patients (8/45; 17.8%), of whom 6 (75.0%) recovered in 6 months after diagnosis. The prevalence of RVSD fell from 71.1% at baseline to 36.4% at 6 months (p=0.007) and 18.8% at one year (p=0.0008 vs baseline; p=0.41 vs 6 month). Although 83.3% of the deceased had RVSD, it didn’t predict mortality in the regression models (p&gt;0.05). Conclusion: These studies have shown that selenium deficiency seems to be a risk factor for PPCM in Kano, Nigeria, related to rural residency. However, serum ceruloplasmin, customary birth practices and some other characteristics were not associated with PPCM in the study area. They have also shown that PPCM patients had modest LVRR but high mortality at one year. In addition, using the ECG risk score could help to streamline the diagnosis of PPCM with significant accuracy, prior to confirmatory investigations in postpartum women. Finally, RVSD and reverse remodelling were common in Nigerians with PPCM, in whom the first 6 months after diagnosis seem to be critical for RV recovery and survival. / Summary

Introduction: Peripartum Cardiomyopathy (PPCM) affects 1 in 3,000 pregnancies and accounts for 5% of heart transplants in US women. Preeclampsia is one of the Hypertensive Disorders of Pregnancy (HDOP) that has been epidemiologically associated with PPCM which is a form of dilated cardiomyopathy. This concurrent clinical presentation of PPCM with associated Preeclampsia appears to be increasing in rural Northeast Tennessee. The diagnosis of Peripartum Cardiomyopathy is made by echocardiographic criteria of left ventricular dysfunction with LVEF Case Presentation: A 22 year old G2P0010 @ 33 weeks and 1 day gestation was accepted as a transfer of care from an outside hospital. Patient had no prenatal care, was homeless, had a history of depression and polysubstance abuse. She presented with abdominal pain, shortness of breath, coughing, and unknown due date. Vitals on presentation were BP 175/99 mmHg, HR 113, respiratory rate 32, and temperature 99.2. Lab results showed elevated AST/ALT 234/102, LDH 903, Uric Acid 7.0, WBC 26.2, and BNP 1935. The 24 hour urine total protein resulted 4455 mg. Transthoracic echocardiogram revealed LV ejection fraction of 30 to 35% with global hypokinesis of the LV wall. The CXR was consistent with bilateral infiltrates and pulmonary edema. She was admitted to L & D and given a dose of Betamethasone for fetal lung maturity. Fetal heart tracing showed a baseline of 145 bpm with minimal variability and no accelerations or decelerations. The tocodynamometer showed contractions q 3-5 minutes and the ultrasound evaluation showed cephalic presentation. Social work and Cardiology consults were obtained. The clinical diagnoses of Peripartum Cardiomyopathy, Preeclampsia with severe features, and Bilateral Pneumonia were made and treatment included Lasix, Metoprolol, Magnesium Sulfate, Ceftriaxone, and Azithromycin. A primary low transverse cesarean delivery was performed with a live female infant weighing 1920 grams, 4 pounds 4 ounces, and Apgars: 6, 8. The patient was discharged home on Metoprolol XL 25 mg bid, Lisinopril 10 mg daily, Procardia 30 mg daily, Lovenox 40 mg daily, and Depo-Provera for contraception. Patient was informed that she would need a repeat echocardiogram in 3 months to assess left ventricular function. Discussion: In this Appalachian region, there is an increased occurrence of Peripartum Cardiomyopathy presenting concurrently with Preeclampsia. These two combined clinical entities increase maternal morbidity and mortality. From a public health perspective, this clinical case highlights the psychosocial factors such as poverty, homelessness, polysubstance abuse, and depression that may have contributed to the clinical disease. The distinct features of this preterm antepartum case of PPCM are a younger, Caucasian primigravida with singleton gestation. Healthcare providers should have heightened awareness of this clinical presentation, especially in the postpartum period.

Objetivos: Determinar a prevalência de disfunção sistólica assintomática do ventrículo esquerdo no puerpério e comparar sua evolução com os casos de miocardiopatia periparto ocorridos no mesmo período. Pacientes e Métodos: Estudo transversal entre setembro de 2002 e abril de 2005, para determinar a prevalência de disfunção ventricular assintomática no puerpério imediato e a incidência de miocardiopatia periparto no mesmo período, e entre novembro de 2007 e janeiro de 2008 para verificar a evolução clínica e ecocardiográfica destas pacientes. Os parâmetros ecocardiográficos das puérperas com disfunção ventricular assintomática também foram comparados com os de puérperas normais. Disfunção sistólica do ventrículo esquerdo foi definida como diâmetro diastólico final ≥ 5,6 cm e/ou fração de ejeção < 53,0% + encurtamento fracional sistólico < 25%. Resultados: Foram rastreadas 1182 puérperas, sendo detectados 10 casos (0,85%) de disfunção ventricular assintomática, cujas características clínicas, como raça, superfície corporal, paridade, gemelaridade e uso de tocolíticos, não foram diferentes quando comparadas com 18 controles rastreados sem disfunção. A incidência de miocardiopatia periparto no período foi de 6 casos em 10866 partos (1/1811 partos de nascidos vivos). Após uma média de 4,0 anos (2,9-5,2 anos), 7 dos 10 casos de disfunção assintomática e 5 dos 6 casos de miocardiopatia clínica realizaram nova ecocardiografia, verificando-se significativo aumento da fração de ejeção e do encurtamento fracional médios nos dois grupos, sendo que a parcela de recuperação da função foi semelhante nos grupos (p interação > 0,05). Conclusões: Ocorre disfunção ventricular no puerpério sem os achados clínicos de insuficiência cardíaca cujos parâmetros ecocardiográficos evoluem de maneira semelhante aqueles de pacientes com miocardiopatia periparto ao longo do tempo. / Objective: To determine the prevalence of asymptomatic left ventricular systolic dysfunction in puerperium and to compare its progression with that of cases of peripartum cardiomyopathy that occurred in the same study period. Patients and Methods: Cross-sectional study conducted from September 2002 to April 2005 to determine the prevalence of asymptomatic ventricular dysfunction in early puerperium and the incidence of peripartum myocardiopathy, and from November 2007 to January 2008 to obtain clinical and echocardiographic follow-up data of the study patients. Echocardiographic parameters of puerperal women with asymptomatic ventricular dysfunction were also compared with those of normal puerperal women. The parameters to define left ventricular systolic dysfunction were end-diastolic diameter ≥ 5.6 cm and/or ejection fraction < 53.0%, and systolic fractional shortening < 25%. Results: A total of 1182 puerperal women were screened, and 10 cases (0.85%) of asymptomatic ventricular dysfunction were detected. Clinical characteristics, such as ethnicity, body surface, parity, multiple gestations, and tocolytic therapy, were not different from those of the 18 normal women used as controls. The incidence of peripartum cardiomyopathy was 6 cases out of 10866 deliveries (1/1811 live births). After a mean of 4.0 years (2.9-5.2 years), 7 of the 10 patients with asymptomatic dysfunction and 5 of the 6 with clinical cardiomyopathy underwent follow-up echocardiography. A significant increase was found in mean ejection fraction and fractional shortening in the two groups, and function recovery rates were similar in the two groups (p > 0.05). Conclusions: Ventricular dysfunction may occur in the puerperium without clinical signs of heart failure, and the long-term progression of echocardiographic parameters is similar to that found in cases of peripartum cardiomyopathy.

Objetivos: Determinar a prevalência de disfunção sistólica assintomática do ventrículo esquerdo no puerpério e comparar sua evolução com os casos de miocardiopatia periparto ocorridos no mesmo período. Pacientes e Métodos: Estudo transversal entre setembro de 2002 e abril de 2005, para determinar a prevalência de disfunção ventricular assintomática no puerpério imediato e a incidência de miocardiopatia periparto no mesmo período, e entre novembro de 2007 e janeiro de 2008 para verificar a evolução clínica e ecocardiográfica destas pacientes. Os parâmetros ecocardiográficos das puérperas com disfunção ventricular assintomática também foram comparados com os de puérperas normais. Disfunção sistólica do ventrículo esquerdo foi definida como diâmetro diastólico final ≥ 5,6 cm e/ou fração de ejeção < 53,0% + encurtamento fracional sistólico < 25%. Resultados: Foram rastreadas 1182 puérperas, sendo detectados 10 casos (0,85%) de disfunção ventricular assintomática, cujas características clínicas, como raça, superfície corporal, paridade, gemelaridade e uso de tocolíticos, não foram diferentes quando comparadas com 18 controles rastreados sem disfunção. A incidência de miocardiopatia periparto no período foi de 6 casos em 10866 partos (1/1811 partos de nascidos vivos). Após uma média de 4,0 anos (2,9-5,2 anos), 7 dos 10 casos de disfunção assintomática e 5 dos 6 casos de miocardiopatia clínica realizaram nova ecocardiografia, verificando-se significativo aumento da fração de ejeção e do encurtamento fracional médios nos dois grupos, sendo que a parcela de recuperação da função foi semelhante nos grupos (p interação > 0,05). Conclusões: Ocorre disfunção ventricular no puerpério sem os achados clínicos de insuficiência cardíaca cujos parâmetros ecocardiográficos evoluem de maneira semelhante aqueles de pacientes com miocardiopatia periparto ao longo do tempo. / Objective: To determine the prevalence of asymptomatic left ventricular systolic dysfunction in puerperium and to compare its progression with that of cases of peripartum cardiomyopathy that occurred in the same study period. Patients and Methods: Cross-sectional study conducted from September 2002 to April 2005 to determine the prevalence of asymptomatic ventricular dysfunction in early puerperium and the incidence of peripartum myocardiopathy, and from November 2007 to January 2008 to obtain clinical and echocardiographic follow-up data of the study patients. Echocardiographic parameters of puerperal women with asymptomatic ventricular dysfunction were also compared with those of normal puerperal women. The parameters to define left ventricular systolic dysfunction were end-diastolic diameter ≥ 5.6 cm and/or ejection fraction < 53.0%, and systolic fractional shortening < 25%. Results: A total of 1182 puerperal women were screened, and 10 cases (0.85%) of asymptomatic ventricular dysfunction were detected. Clinical characteristics, such as ethnicity, body surface, parity, multiple gestations, and tocolytic therapy, were not different from those of the 18 normal women used as controls. The incidence of peripartum cardiomyopathy was 6 cases out of 10866 deliveries (1/1811 live births). After a mean of 4.0 years (2.9-5.2 years), 7 of the 10 patients with asymptomatic dysfunction and 5 of the 6 with clinical cardiomyopathy underwent follow-up echocardiography. A significant increase was found in mean ejection fraction and fractional shortening in the two groups, and function recovery rates were similar in the two groups (p > 0.05). Conclusions: Ventricular dysfunction may occur in the puerperium without clinical signs of heart failure, and the long-term progression of echocardiographic parameters is similar to that found in cases of peripartum cardiomyopathy.

Objetivos: Determinar a prevalência de disfunção sistólica assintomática do ventrículo esquerdo no puerpério e comparar sua evolução com os casos de miocardiopatia periparto ocorridos no mesmo período. Pacientes e Métodos: Estudo transversal entre setembro de 2002 e abril de 2005, para determinar a prevalência de disfunção ventricular assintomática no puerpério imediato e a incidência de miocardiopatia periparto no mesmo período, e entre novembro de 2007 e janeiro de 2008 para verificar a evolução clínica e ecocardiográfica destas pacientes. Os parâmetros ecocardiográficos das puérperas com disfunção ventricular assintomática também foram comparados com os de puérperas normais. Disfunção sistólica do ventrículo esquerdo foi definida como diâmetro diastólico final ≥ 5,6 cm e/ou fração de ejeção < 53,0% + encurtamento fracional sistólico < 25%. Resultados: Foram rastreadas 1182 puérperas, sendo detectados 10 casos (0,85%) de disfunção ventricular assintomática, cujas características clínicas, como raça, superfície corporal, paridade, gemelaridade e uso de tocolíticos, não foram diferentes quando comparadas com 18 controles rastreados sem disfunção. A incidência de miocardiopatia periparto no período foi de 6 casos em 10866 partos (1/1811 partos de nascidos vivos). Após uma média de 4,0 anos (2,9-5,2 anos), 7 dos 10 casos de disfunção assintomática e 5 dos 6 casos de miocardiopatia clínica realizaram nova ecocardiografia, verificando-se significativo aumento da fração de ejeção e do encurtamento fracional médios nos dois grupos, sendo que a parcela de recuperação da função foi semelhante nos grupos (p interação > 0,05). Conclusões: Ocorre disfunção ventricular no puerpério sem os achados clínicos de insuficiência cardíaca cujos parâmetros ecocardiográficos evoluem de maneira semelhante aqueles de pacientes com miocardiopatia periparto ao longo do tempo. / Objective: To determine the prevalence of asymptomatic left ventricular systolic dysfunction in puerperium and to compare its progression with that of cases of peripartum cardiomyopathy that occurred in the same study period. Patients and Methods: Cross-sectional study conducted from September 2002 to April 2005 to determine the prevalence of asymptomatic ventricular dysfunction in early puerperium and the incidence of peripartum myocardiopathy, and from November 2007 to January 2008 to obtain clinical and echocardiographic follow-up data of the study patients. Echocardiographic parameters of puerperal women with asymptomatic ventricular dysfunction were also compared with those of normal puerperal women. The parameters to define left ventricular systolic dysfunction were end-diastolic diameter ≥ 5.6 cm and/or ejection fraction < 53.0%, and systolic fractional shortening < 25%. Results: A total of 1182 puerperal women were screened, and 10 cases (0.85%) of asymptomatic ventricular dysfunction were detected. Clinical characteristics, such as ethnicity, body surface, parity, multiple gestations, and tocolytic therapy, were not different from those of the 18 normal women used as controls. The incidence of peripartum cardiomyopathy was 6 cases out of 10866 deliveries (1/1811 live births). After a mean of 4.0 years (2.9-5.2 years), 7 of the 10 patients with asymptomatic dysfunction and 5 of the 6 with clinical cardiomyopathy underwent follow-up echocardiography. A significant increase was found in mean ejection fraction and fractional shortening in the two groups, and function recovery rates were similar in the two groups (p > 0.05). Conclusions: Ventricular dysfunction may occur in the puerperium without clinical signs of heart failure, and the long-term progression of echocardiographic parameters is similar to that found in cases of peripartum cardiomyopathy.

Background: Peripartum cardiomyopathy (PPCM) is a rare, life-threatening form of heart disease, frequently associated with gene alterations and, in some cases, presenting with advanced heart failure. Little is known about ventricular assist device (VAD) implantation in severe PPCM cases. We describe long-term follow-up of PPCM patients who were resistant to medical therapy and received mechanical circulatory support or heart transplant. Methods and results: A total of 13 patients were included with mean follow-up of eight years. Mean age of PPCM onset was 33.7 ± 7.7 years. All patients were initially treated with angiotensin-converting enzyme inhibitors and beta-blockers, and four received bromocriptine. Overall, five patients received VADs (three biventricular, two isolated left ventricular) at median 27 days (range: 3 to 150) following childbirth. Two patients developed drive line infection. Due to the short support time, none of those patients had a stroke or VAD thrombosis. In total, five patients underwent heart transplantation, of which four previously had implanted VADs. Median time to transplantation from PPCM onset was 140 days (range: 43 to 776), and time to transplantation from VAD implantation were 7, 40, 132, and 735 days, respectively. All patients survived until most recent follow up, with the exception of one patient who died following unrelated abdominal surgery two years after PPCM recovery. Conclusions: In patients with severe, life-threatening PPCM refractory to medical management, mechanical circulatory support with or without heart transplantation is a safe therapeutic option.