Prevalência das Complicações Cardiovasculares nos Indivíduos com Anemia Falciforme e Outras Hemoglobinopatias: Uma Revisão Sistemática / Prevalence of Cardiovascular Complications in Individuals with Sickle Cell Anemia and Other Hemoglobinopathies: A Systematic Review

Resumo Fundamento A anemia falciforme (AF) é uma doença hereditária cujas complicações cardiovasculares são a principal causa de morte, o mesmo sendo observado em outras hemoglobinopatias. A identificação precoce dessas alterações pode modificar favoravelmente o curso da doença. Objetivo Comparar a prevalência de complicações cardiovasculares entre indivíduos com AF e indivíduos com outras hemoglobinopatias. Métodos Seguindo recomendações do protocolo PRISMA, realizou-se revisão sistemática da literatura com buscas nas bases de dados PubMed/Medline, associadas à busca manual. Incluídos estudos que analisaram a prevalência das alterações cardiovasculares nas hemoglobinopatias (AF, traço falciforme, hemoglobinopatia SC, alfatalassemia e betatalassemia). A qualidade metodológica dos artigos foi realizada pela escala de Newcastle-Ottawa. Resultados Foram selecionados para análise quatro estudos, resultando em um tamanho amostral de 582 participantes: 289 portadores de AF, 133 possuem hemoglobinopatia SC, 40 com betatalassemia, 100 indivíduos saudáveis e nenhum com alfatalassemia ou traço falcêmico. Dilatação das câmaras cardíacas, hipertrofia ventricular esquerda e direita, hipertensão pulmonar, disfunção diastólica, insuficiência mitral e insuficiência tricúspide são mais prevalentes na AF do que nas demais hemoglobinopatias consideradas. A sobrecarga miocárdica de ferro é mais frequente na talassemia maior do que na AF. A função sistólica foi similar entre as hemoglobinopatias. Conclusão Verificou-se maior comprometimento cardiovascular nos indivíduos com AF do que naqueles com as demais hemoglobinopatias, reforçando a necessidade de acompanhamento cardiovascular regular e frequente nos pacientes falcêmicos.

Abstract Background Sickle cell anemia (SCA) is a hereditary disease whose cardiovascular complications are the main cause of death, the same being observed in other hemoglobinopathies. Early identification of these changes can favorably modify the course of the disease. Objective To compare the prevalence of cardiovascular complications between individuals with SCA and individuals with other hemoglobinopathies. Method Following the recommendations of the PRISMA protocol, a systematic literature review was carried out with searches in PubMed/Medline databases, associated with a manual search. Studies that analyzed the prevalence of cardiovascular alterations in hemoglobinopathies (SCA, sickle cell trait, SC hemoglobinopathy, alpha-thalassemia and beta-thalassemia) were included. The methodological quality of the articles was assessed using the Newcastle-Ottawa scale. Results Four studies were selected for analysis, resulting in a sample size of 582 participants: 289 with SCA, 133 with SC hemoglobinopathy, 40 with beta-thalassemia, 100 healthy individuals and none with alpha-thalassemia or sickle cell trait. Dilatation of the cardiac chambers, left and right ventricular hypertrophy, pulmonary hypertension, diastolic dysfunction, mitral regurgitation and tricuspid regurgitation are more prevalent in SCA than in the other hemoglobinopathies considered. Myocardial iron overload is more frequent in thalassemia major than in sickle cell anemia. Systolic function is similar between different hemoglobinopathies. Conclusion There is greater cardiovascular impairment in individuals with SCA than in those with other hemoglobinopathies, reinforcing the necessity for regular cardiovascular follow-up in sickle cell patients.

Caracterización de gestantes con enfermedad tromboembólica venosa atendidas en el Hospital General Docente "Abel Santamaría Cuadrado" / Characterization of pregnant women with venous thromboembolic disease attended at the General Teaching Hospital "Abel SantamaríaCuadrado"

RESUMEN Introducción: la enfermedad tromboembólica venosa constituye una de lascomplicacionesdelembarazo, que puede evolucionar tórpidamente y causar la muerte. Objetivo: caracterizar a las gestantes con enfermedad tromboembólica venosa atendidas en el Hospital General Docente "Abel Santamaría Cuadrado". Métodos: se realizó un estudio observacional, descriptivo y transversal en gestantes atendidas en el Hospital General Docente "Abel Santamaría Cuadrado" entre mayo de 2018 y marzo de 2021. La muestra quedó conformada por 26 pacientes. Se empleó estadística descriptiva. Resultados: el 0,09 % de las 27 500 gestantes atendidas presentaronenfermedad tromboembólica venosa, conmayorincidenciaen el grupo etario de gestantes de 35 años o más (0,06 %). El 76,92 % desarrolló la enfermedad tromboembólica venosa durante elpuerperio, la mayor incidencia durante el puerperio se observó en pacientes sometidas a cesárea (80 %). El 57,69 % de las pacientes conenfermedad tromboembólica venosa durante la gestaciónpresentaronvárices en miembros inferiores y el 69,23 % refirieron antecedentes de trombosis venosa profunda. La enfermedad tromboembólica venosa fuecomún (57,69 %) en gestantes con un índice de masa corporal mayor de 28,6. Conclusiones: el tromboembolismo venoso presentó una baja incidencia, la cualfuemayor durante elpuerperio, se presentócomúnmente en esa etapa en aquellas que fuenecesariorealizarles cesárea. En las gestantes con antecedentes de trombosis venosa profunda fuecomún la recurrencia. La enfermedad tromboembólica venosa fue más frecuente en gestantes conun índice de masa corporal elevado.

ABSTRACT Introduction: venous thromboembolic disease constitutes one of the complications of pregnancy, which can evolve torpidly and cause death. Objective: to characterize pregnant women with venous thromboembolic disease attended at the "Abel SantamaríaCuadrado" General Teaching Hospital. Methods: an observational, descriptive and cross-sectional study was conducted in pregnant women attended at the "Abel SantamaríaCuadrado" General Teaching Hospital between May 2018 and March 2021. The sample consisted of 26 patients. Descriptive statistics were used. Results: 0,09 % of the 27,500 pregnant women attended presented venous thromboembolic disease, with a higher incidence in the age group of pregnant women aged 35 years or older (0,06 %). A total of 76,92 % developed venous thromboembolic disease during the puerperium, with the highest incidence during the puerperium being observed in patients who underwent cesarean section (80 %). Of the patients with venous thromboembolic disease during pregnancy, 57,69% presented varicose veins in the lower limbs and 69,23 % reported a history of deep vein thrombosis. Venous thromboembolic disease was common (57,69 %) in pregnant women with a body mass index greater than 28,6. Conclusions: Venous thromboembolism presented a low incidence, which was higher during the puerperium, occurring commonly at that stage in those who had to undergo cesarean section. In pregnant women with a history of deep vein thrombosis, recurrence was common. Venous thromboembolic disease was more frequent in pregnant women with a high body mass index.

Clinical characteristics and pregnancy outcomes of pregnant women with left ventricular non-compaction / 中华妇产科杂志

Objective:To investigate the clinical characteristics and pregnancy outcomes in pregnant women with left ventricular non-compaction (LVNC).Methods:The clinical data of seven pregnant women with LVNC from January 2011 to December 2021 in Beijing Anzhen Hospital,Capital Medical University were retrospectively analyzed, including age, gestational age of symptom first occured, LVNC history, clinical symptoms, New York Heart Association (NYHA) cardiac function class, echocardiography, blood brain natriuretic peptide (BNP), treatment and the maternal and fetal outcomes.Results:Five cases were diagnosed before pregnancy, of which there were three women with medication; one case diagnosed in the month of pregnancy; one case diagnosed at 36 weeks of gestation. NYHA cardiac function was grade Ⅰ in four cases and grade Ⅱ in three cases before or during the first trimester of pregnancy. Of the five pregnant women who underwent echocardiography, there were one case of left ventricular insufficiency, three cases of mild left ventricular dysfunction and one case of normal left ventricular function before or during the first trimester of pregnancy. Of the five pregnant women to the second and third trimester of pregnancy, there were one case of grade Ⅳ, one case of grade Ⅲ, two cases of grade Ⅱ-Ⅲ and one case of grade Ⅱ in NYHA class ; three cases of left ventricular insufficiency, two cases of normal left ventricular function by echocardiography four cases had cardiac symptoms at 15-24 weeks of gestation and were treated with medication. In four cases, blood BNP increased to 214-1 197 ng/L during pregnancy, and were 89-106 ng/L after termination of pregnancy. There were 4 cases with arrhythmia. Indications for termination of pregnancy: LVNC complicated with heart failure in two cases, LVNC complicated with decreased cardiac function and threatened preterm birth in one case, complicated with pregnancy at full term in two cases, LVNC complicated with severe pulmonary hypertension in one case, and left ventricular dysfunction in one case. Cesarean section in four cases in the third-trimester, in one case in the second-trimester, and forceps curettage in two cases were taken. Two full-term infants,two preterm infants were born without LVNC.Conclusions:Women diagnosed with LVNC and low left ventricular ejection fraction before pregnancy are more prone to decreased cardiac function during pregnancy. Carrying out pregnancy risk assessment and strengthening the multi-disciplinary team management of high risk factors in pregnancy are conducive to achieve good pregnancy outcomes.

Electrocardiogramas durante el tercer trimestre de embarazo: / Electrocardiography During the Third Trimester of Pregnancy:

RESUMEN Objetivo: Analizar las características electrocardiográficas en embarazadas sin patología cardiovascular. Material y métodos: Estudio de corte transversal, descriptivo, multicéntrico. Se incluyeron pacientes sin patología cardiovascular que cursaban el tercer trimestre de embarazo y que concurrieron a una evaluación cardiológica preparto entre abril y julio de 2020; todas ellas firmaron el consentimiento correspondiente. Resultados: Se analizaron 80 trazados. La mediana de la frecuencia cardíaca fue 82 lpm (RIC: 70-93 lpm). La mediana del eje QRS fue 54° (RIC: 39°-71°). Ondas q e infradesnivel del segmento ST en cara inferior y de V4 a V6 fueron hallazgos relativamente frecuentes. La mediana del QTc fue 422 mseg (RIC: 404 mseg-445 mseg). La mediana del tiempo del pico de la onda T a su fin fue 86 mseg (RIC: 74-95 mseg). Conclusión: Las alteraciones más frecuentes ocurrieron en DIII, DII, aVf y de V4 a V6. Las ondas q y el infraST fueron los cambios principales. Fueron infrecuentes las desviaciones del eje, la taquicardia sinusal o el QTc prolongado.

ABSTRACT Objective: The aim of this study was to analyze the electrocardiographic characteristics in pregnant women without cardiovascular disease. Methods: This was a descriptive, cross-sectional, multicenter study, including patients without cardiovascular disease in their third trimester of pregnancy, who underwent cardiac evaluation before delivery between April and July 2020. All patients signed the corresponding informed consent. Results: A total of 80 tracings were analyzed. Median heart rate was 82 bpm (IQR 70-93 bpm) and median QRS axis was 54° (IQR 39°-71°). Q waves and ST segment depression were relatively frequent in inferior leads and from V4 to V6. Median QTc was 422 msec (IQR 404-445 msec) and median time from T wave peak to T wave end was 86 msec (IQR 74-95 msec). Conclusion: The most common changes occurred in T wave peak to T wave end and from V4 to V6. Main changes included q waves and ST-segment depression. Axis deviations, sinus tachycardia or prolonged QTc were rare.

Clinical analysis of pregnancy with cardiovascular diseases for a decade of Beijing Anzhen Hospital / 中华妇产科杂志

Objective:To analyze the clinical data of pregnant women complicated with cardiovascular disease in our center in the past 10 years, and to explore the trend of incidence, clinical diagnosis, and treatment of the disease.Methods:Clinical data of pregnant women with cardiovascular disease who delivered in Beijing Anzhen Hospital, Capital Medical University from 2010 to 2019 were collected and analyzed retrospectively. According to the time of the establishment of multidisciplinary team (MDT) in the center, the pregnant women were divided into the first 5-year group (2010-2014) and the second 5-year group (2015-2019). The general data, the composition of pregnancy complicated with cardiovascular disease and the changes of maternal and infant outcomes of the two groups were analyzed.Results:(1) During 2010-2019, there were 2 267 cases of pregnancy complicated with cardiovascular disease (836 cases in the first 5-year group and 1 431 cases in the second 5-year group), with a total incidence of 10.2% (2 267/22 334). Among all kinds of cardiovascular diseases, arrhythmia (41.0%, 930/2 267) and congenital heart disease (38.2%, 865/2 267) were more common. (2) There were 212 cases (25.4%, 212/836) and 426 cases (29.8%, 426/1 431) classified as Ⅲ or Ⅳ by modified WHO cardiovascular risk classification in the first 5-year group and the second 5-year group, respectively, and the difference was statistically significant ( χ2 =5.076, P=0.024). Among all kinds of cardiovascular diseases, there were 111 cases (13.3%, 111/836) and 159 cases (11.1%, 159/1 431) with valvular disease in the first 5-year group and the second 5-year group, respectively. The change of the component ratio was -16.5% (the difference was significant when the absolute value of change>10%), showing a significant decreasing trend. Aortic disease was found in 16 cases (1.9%, 16/836) and 56 cases (3.9%, 56/1 431), respectively, with a significant upward trend of 105.3%. (3) The mortality rate of pregnant women with cardiovascular disease was 1.0% (22/2 267), and 1.2% (10/836) and 0.8% (12/1 431) in the first 5-year grouop and the second 5-year group, respectively. There was no significant difference between the two groups ( χ2=0.702, P=0.402). ICU occupancy rates in the first 5-year group and the second 5-year group were 25.6% (214/836) and 20.7% (296/1 431), respectively, and the difference between the two groups was statistically significant ( χ2=7.306, P=0.007). There were no significant differences in cesarean section rate, mortality rate and incidence of adverse events between the two groups of pregnant women, and there were no significant differences in birth weight, preterm birth rate, mortality rate and asphyxia rate between the two groups of newborns (all P>0.05). Conclusions:Pregnancy complicated with cardiovascular disease is a common cause of adverse obstetric outcomes. There are various types of specific cardiovascular diseases, and the prognosis varies greatly. In recent years, the disease composition ratio has changed, and the severity and complexity of diseases have increased. Hierarchical management, MDT and individual management could improve the treatment level and reduce adverse outcomes.

Perioperative factors affecting the length of hospital stay after cesarean section of pregnant women with heart disease / 中华妇产科杂志

Objective:To explore the related factors influencing the length of hospital stay(LOS) of pregnant women with heart disease (PWHD) after cesarean section.Methods:A total of 306 patients with PWHD who underwent cesarean section from January 2012 to March 2019 were collected. Among them, 203 patients had not undergone heart surgery (uncorrected group) and 103 patients who had undergone heart surgery (corrected group) during the same period. Demographic, perioperative and postoperative data were recorded. Predictors associated with postoperative LOS were determined using univariate and multivariate linear regression analysis models.Results:(1) The median LOS after cesarean section in the uncorrected group was 6 days (5-8 days). The results of univariate linear regression analysis showed that 38 parameters had significant impact on LOS ( P<0.05). The results of multivariate linear regression analysis showed that 5 parameters were independent risk factors for prolonged LOS in the uncorrected group; among them, the median LOS in uncorrected group with hypertensive disorders of pregnancy was 3 days longer than that in patients with PWHD alone [7 days (5-8 days) vs 4 days (4-5 days), β=0.195, P=0.001]; the median LOS in uncorrected group with high serum creatinine was 3 days longer than normal patients [7 days (5-13 days) vs 4 days (4-5 days), β=0.145, P=0.015]; the LOS of patients who chose general anesthesia was 2 days longer than that of patients who chose spinal anesthesia [6 days (4-8 days) vs 4 days (4-5 days), β=0.154, P=0.007]; the LOS of patients with postoperative pulmonary infection was 4 days longer than that of patients without pulmonary infection [8 days (5-15 days) vs 4 days (4-5 days), β=0.269, P<0.01]; the LOS of patients who admitted to ICU after surgery was 2 days longer than that not admitted patients [6 days (5-8 days) vs 4 days (4-5 days), β=0.268, P<0.01]. (2) The median LOS after cesarean section in corrected group was 4 days (4-5 days). The results of univariate linear regression analysis showed that 8 parameters had significant impact on the LOS (all P<0.05). The results of multivariate linear regression analysis showed that 2 parameters, which were American Society of Anesthesiologists (ASA) grade ( β=0.198, P=0.028) and intraoperative blood loss ( β=0.285, P=0.003), were the independent risk factors for prolonged LOS in corrected group. Conclusion:Preoperative with hypertensive disorders of pregnancy, preoperative creatinine increase, intraoperative general anesthesia, postoperative pulmonary infection, and postoperative admission to ICU are independent predictors of prolonged LOS in uncorrected patients with PWHD; ASA classification and intraoperative bleeding are independent predictor of prolonged postoperative LOS in patients with corrected PWHD.

Clinical features and maternal and infant outcomes of right-sided infective endocarditis during pregnancy: a case report and literature review / 中华围产医学杂志

Objective@#To investigate the clinical features, treatment strategies, and maternal and infant outcomes of pregnancy complicated by right-sided infective endocarditis (RSIE) to provide evidence for clinical management.@*Methods@#By searching literature on RSIE during pregnancy from the databases of CNKI, Wanfang Database, VIP, CBM, PubMed, OVID, EMbase and ScienceDirect, relevant information were collected to analyze the clinical manifestations, risk factors, positions of intracardiac vegetations, results of blood culture, treatment strategies and maternal and infant outcomes of RSIE.@*Results@#A total of 15 articles were retrieved, involving 18 infected gravidas with the average age of (27.7±4.8) years and average gestational age at onset of (27.8±6.9) weeks. Fever (n=14), cough (n=12), anemia (n=8) and shortness of breath or dyspnea (n=8) were the common symptoms. Cardiac murmurs were detected on auscultation in seven cases, of which six were systolic murmurs and one was unspecified. Heart sounds of five cases were clear on auscultation without any murmurs. Nine cases were complicated by pulmonary embolism and five by heart failure. The major risk factors were congenital heart diseases (10/18) and intravenous drug abuse (6/18). Vegetations were commonly seen on the tricuspid valves (10/18), followed by the pulmonary valves (4/18). The rate of positive blood culture was high (15/16) with Staphylococcus (9/15) and Streptococcus (3/15) being the primary pathogens. Most pregnancies were timely ended by cesarean section. Apart from receiving fundamental antibiotic therapy for infective endocarditis, 11 patients underwent cardiac surgery, including vegetation removal, valve repair or replacement and surgery for congenital heart diseases, before or after pregnancy or during cesarean section based on their gestational age, condition, and cardiopulmonary function. There was no maternal death, but one neonatal death was reported due to severe asphyxia following cesarean section at 28 weeks. Maternal and neonatal outcomes were good during follow-up.@*Conclusions@#Pregnancy complicated by RSIE is rare and complex, requiring early diagnosis and individualized treatment. Adequate and full-course antibiotic therapy, appropriate surgical procedures and timely termination are of great importance for improving maternal and infant outcomes.

Clinical features and maternal and infant outcomes of right-sided infective endocarditis during pregnancy:a case report and literature review / 中华围产医学杂志

Objective To investigate the clinical features,treatment strategies,and maternal and infant outcomes of pregnancy complicated by right-sided infective endocarditis (RSIE) to provide evidence for clinical management.Methods By searching literature on RSIE during pregnancy from the databases of CNKI,Wanfang Database,VIP,CBM,PubMed,OVID,EMbase and ScienceDirect,relevant information were collected to analyze the clinical manifestations,risk factors,positions of intracardiac vegetations,results of blood culture,treatment strategies and maternal and infant outcomes of RSIE.Results A total of 15 articles were retrieved,involving 18 infected gravidas with the average age of (27.7 ± 4.8) years and average gestational age at onset of (27.8 ± 6.9) weeks.Fever (n=1 4),cough (n=1 2),anemia (n=8) and shortness of breath or dyspnea (n=8) were the common symptoms.Cardiac murmurs were detected on auscultation in seven cases,of which six were systolic murmurs and one was unspecified.Heart sounds of five cases were clear on auscultation without any murmurs.Nine cases were complicated by pulmonary embolism and five by heart failure.The major risk factors were congenital heart diseases (10/18) and intravenous drug abuse (6/18).Vegetations were commonly seen on the tricuspid valves (10/18),followed by the pulmonary valves (4/18).The rate of positive blood culture was high (15/16) with Staphylococcus (9/15) and Streptococcus (3/15) being the primary pathogens.Most pregnancies were timely ended by cesarean section.Apart from receiving fundamental antibiotic therapy for infective endocarditis,1 1 patients underwent cardiac surgery,including vegetation removal,valve repair or replacement and surgery for congenital heart diseases,before or after pregnancy or during cesarean section based on their gestational age,condition,and cardiopulmonary function.There was no maternal death,but one neonatal death was reported due to severe asphyxia following cesarean section at 28 weeks.Maternal and neonatal outcomes were good during follow-up.Conclusions Pregnancy complicated by RSIE is rare and complex,requiring early diagnosis and individualized treatment.Adequate and full-course antibiotic therapy,appropriate surgical procedures and timely termination are of great importance for improving maternal and infant outcomes.

Analysis of maternal deaths with cardiovascular diseases in Shanghai obstetric heart disease intensive care unit within twenty-six years / 中华妇产科杂志

Objective@#To analyze risk factors, cardiovascular complications, time of death, gestational age of delivery and offspring outcomes in the maternal deaths with cardiovascular diseases (CVD).@*Methods@#Totally 4 112 cases of pregnant women with CVD in Shanghai obstetric heart disease intensive care unit within 26 years (from January 1993 to December 2018) were collected, and 20 maternal deaths within these cases were analyzed retrospectively.@*Results@#(1) Among the 20 deaths, structural heart diseases accounted for 90% (18/20), pregnancy induced heart diseases was 10% (2/20) while there was no dysfunctional heart disease. The mortality of pregnant women with CVD was 0.486% (20/4 112). (2) The following risk factors were common in these women, getting pregnant without counselling (95%, 19/20) , New York Heart Association classⅢ or Ⅳcardiac function (70%, 14/20), complicated with pulmonary hypertension (75%, 15/20) and prior heart events (60%, 12/20). And 85% (17/20) deaths occurred in puerperium, 15% (3/20) occurred before labor,while no death occurred during labor. And 65% (13/20) deaths died due to heart failure, 20% (4/20) deaths were due to pulmonary hypertension crisis, 5% (1/20) died on sudden cardiac arrest, rupture of aortic dissection and sudden death, respectively.@*Conclusions@#Women with CVD should get pregnant after strict evaluation. Pulmonary hypertension is one of the most severe contraindications to pregnancy, especially in patients with moderate to severe pulmonary hypertension. The puerperium period is a critical period that threatens the safety of these patients. Since heart failure is the most common cause of death, it is necessary to prevent and treat heart failure and to monitor heart function dynamically, especially in those with structural abnormal heart diseases. Moreover, it is also of importance to standardize antenatal care and to identify the severity of heart diseases in time.

Miocardiopatia não compactada em gestante com linfoma não Hodgkin. Relato de caso / Non-compaction cardiomyopathy in pregnant women with non-Hodgkin lymphoma: a case report

A miocardiopatia não compactada é uma doença congênita rara, que pode ocorrer isoladamente ou associada a outros defeitos, por falha no processo de compactação das fibras miocárdicas, resultando na persistência de trabeculações e recessos profundos. A associação entre a miocardiopatia não compactada e gestação é incomum na literatura, assim como a relação com macroglobulinemia de Waldenstrom, um tipo de linfoma não Hodgkin. Descrevemos aqui a rara associação destas três patologias. Trata-se de paciente do sexo feminino, sem antecedentes hematológicos, neoplasias ou cardiopatias, que procurou o serviço com queixa de astenia progressiva, dores no corpo, perda ponderal importante e anemia. Na investigação diagnóstica, a imunoeletroforese de proteína constatou pico monoclonal em IgM Kappa, com inventário medular por imunofenotipagem e biópsia de medula óssea com Kappa+, CD19+, CD20+, CD38 e CD79b, confirmando diagnóstico de neoplasia de linfócitos B maduros. Na terapêutica, optou-se pelo esquema de primeira linha com dexametasona, rituximabe e ciclofosfamida (DRC) − este último considerado agente alquilante cardiotóxico. Em triagem pré-quimioterápica, o eletrocardiograma mostrou alteração da repolarização ventricular anterosseptal. O ecocardiograma transtorácico evidenciou trabeculações excessivas no ápice do ventrículo esquerdo, sugerindo não compactação do miocárdio. A ressonância magnética confirmou o diagnóstico. Foi iniciada terapia com metoprolol e ácido acetilsalisílico. Todavia, após o último ciclo de terapia quimioterápica, paciente descobriu gravidez (G1P1A0). O período gestacional e o puerpério evoluíram sem manifestações clínicas de insuficiência cardíaca, em classe funcional I (New York Heart Association), mesmo com redução da fração de ejeção do ventrículo esquerdo ao ecocardiograma transtorácico. (AU)

Non-compaction cardiomyopathy is a rare congenital disease that can occur in isolation or associated with other defects, due to failure in compaction of myocardial fiber, resulting in persistence of myocardial trabeculations and deep recesses. The association between non-compaction cardiomyopathy and gestation, as well as the relationship with Waldenstrom's macrobulinemia, a type of Non-Hodgkin's Lymphoma (NHL), are not common in the literature. This study describes the rare association of these three pathologies. This is the case of a female patient with no history of hematological, neoplastic, or heart diseases, who sought the service with complaints of progressive weakness, body aches, important weight loss, and anemia. During the diagnostic investigation, protein immunoelectrophoresis showed a monoclonal peak in IgM Kappa monoclonal gammopathy, with a medullary inventory by immunophenotyping and bone marrow biopsy with Kappa+, CD19+, CD20+, CD38 and CD79b, confirming the diagnosis of mature B-cell lymphocyte neoplasm. The first line therapy chosen was dexamethasone, rituximab, and cyclophosphamide (CKD), with the latter being considered a cardiotoxic alkylating agent. At pre-chemotherapy screening, the electrocardiogram showed an alteration of the anteroseptal ventricular repolarization. Transthoracic echocardiography (ETT) showed excessive trabeculations at the apex of the left ventricle (LV), suggesting no compaction of the myocardium. The magnetic resonance imaging confirmed the diagnosis.Therapy with metoprolol and acetylsalicylic acid was started. However, after the last cycle of chemotherapy, the patient found she was pregnant (G1P1A0). The gestational and puerperium period progressed with no clinical manifestations of heart failure, in functional class I (New York Heart Association), albeit the reduction of the ejection fraction of the left ventricular shown in the transthoracic echocardiography. (AU)

Clinical analysis of 24 cases of aortic dissection during pregnancy / 中华妇产科杂志

plus stented elephant trunk implantation (Bentall+Sun′s surgery), aortic root replacement (Bentall surgery), stent implantation, thoracic and abdominal aorta replacement. The aortic operation time of the 19 patients were 5 gestational weeks to 1 month after delivery. The relation between aortic operation and the termination of pregnancy: 4 patients underwent aorta surgery after termination of pregnancy, 9 patients had cesarean section and aorta surgery at the same time, 6 patients underwent aorta surgery before cesarean section. ②5 patients did not receive arota surgery, 2 patients of type A dissection and 1 patient of type B dissection died before the surgery;2 cases of type B dissection underwent conservative treatment. The termination time of pregnancy was 6-37 gestational weeks, with the average of (26 ± 10) weeks. (3)Maternal and fetal outcomes:20 patients survived after treatment (83%,20/24) and 4 patients died (17%,4/24). 10 cases were live births, including 4 full-term infants and 6 preterm premature infants. The birth weight of the neonates was 1 080-3 800 g, with the average of (2 302±764) g. Three of them were very low birth weight infants and 1 was low birth weight infant;3 neonates had mild asphyxia. The neonates were followed up for 0.5 to 10 years, with the average time of (1.4 ± 1.7) years. So far the infants′ development was good.Conclusions Pregnancy with aortic dissection is pernicious. Early identification, prompt diagnosis and prompt interventing of the vascular surgery are necessary to the safety of mother and fetus.

Clinical treatment of infective endocarditis with vegetations in pregnant women and the outcomes of gestation / 中华妇产科杂志

Objective To investigate the clinical treatment of infective endocarditis with vegetations in pregnant women and the outcomes of the gestation. Methods Nine cases of pregnant women diagnosed as infective endocarditis with vegetations in Beijing Anzhen Hospital, Capital Medical University from January 2001 to October 2015 were enrolled in retrospective analysis. Consultations were held by doctors from department of obstetrics, anesthesiology, cardiology, cardial surgery and extracorporeal circulation to decide the individualized treatment plan for the 9 cases of pregnant women after admissions. Clinical treatments including general treatment, anti-infection treatment, cardiac surgery, and termination of pregnancy surgery were completed through collaboration among related departments. The clinical characters, therapeutic regimens, maternal and neonatal outcomes of the 9 cases were analyzed. Results (1)Clinical characters: the ages of the 9 cases of pregnant women were from 25 to 36 years old. The onset gestational ages were from 19 to 36 weeks. Clinical symptoms: fever, cough, sputum and progressive anemia were the main symptoms. Patients had cyanosis of lips, could not lie on the back or even be orthopnea, when heart failure happened. Heart murmur was audible and splenomegaly was touched in physical examination. Blood cultures were positive. Basic heart disease types: 7 cases of congenital heart diseases included 2 cases of aortic insufficiency, 1 case of mitral insufficiency, 1 case of patent ductus arteriosus, 1 case of right ventricular outflow tract stenosis and 2 cases of ventricular septal defect.Two cases of rheumatic heart diseases included 1 case of mitral stenosis, 1 case of mitral stenosis after artificial disc changed and jammed. According to endocardial vegetations attached position there were 3 cases of mitral valve vegetations, 2 cases of pulmonary valve vegetations, 3 cases of aortic vegetations and 1 case of right ventricular outflow tract neoplasm. Preoperative heart function classification:1 case of levelⅡ, 3 cases of levelⅢ, 5 cases of levelⅣ.(2)Treatments:general treatment included oxygen uptake, rest in bed, cardiac strengthen and diuretic therapy, etc. Combined and adequate antibiotics were applied in anti-infection treatment according to drug sensitive test. Nine cases of pregnant women were all performed surgical treatment of heart diseases and removal of the endocardial vegetations. Caesarean sections were performed for 2 cases in second trimester and for 7 cases in last trimester. Cardiac surgery and caesarean section were operated in 6 cases at the same time among 22-34 weeks of pregnancy. Cardiac surgery were respectively operated in 2 cases 11 days and 32 days after the caesarean section at 33, 37 weeks of pregnancy. While Cardiac surgery was operated (at 26 weeks of pregnancy) before the caesarean section (at 37 weeks of pregnancy) in another 1 case. (3) Maternal and neonatal outcomes:7 cases of pregnant women were rescued successfully, while 2 cases of pregnant women were death. Postoperative heart function classification: 1 case of level Ⅰ, 2 cases of levelⅡ, 4 cases of levelⅢand 2 cases of levelⅥ. Neonatal survivals were 6 cases including 2case of full-term infants, 4 cases of preterm infants. Stillbirth or neonatal death were 3 cases, which included 2 cases performed caesarean section in second trimester and 1 case of very low weight infant who was given up treatment by family because of severe asphyxia. Followed up periods were from 1 to 7 years with an average time of (2.0 ± 1.6) years. Infants and young children grew and developed well during the period of follow up. Conclusions The risk is extremely high of pregnancy with infective endocarditis with vegetations. But there is still a way to save the maternal and neonatal life by using a multidisciplinary collaboration formulation and implementation of individualized treatment plan and selecting the appropriate time for heart surgery and the termination of pregnancy.

Diagnostic value of random spot urine albumin/creatinine ratio in women with hypertensive disorders complicating pregnancy / 天津医药

Objective To explore the correlation of random spot albuminuria to creatinine ratio (ACR) and 24 h uri?nary protein, and the diagnostic value of ACR in women with hypertensive disorders complicating pregnancy (HDCP). Meth?ods A total of 584 women with HDCP (169 with gestational hypertension, 205 with mild pre-eclampsia, 173 with severe pre-eclampsia and 37 with chronic hypertension) and 2 038 normal pregnant women were included in this study. Changes of ACR, 24-h urinary protein and other biochemical indicators were compared between groups. Bivariate correlation analysis was used to analyze the relationship between ACR, 24-h urinary protein and other biochemical indicators. Receiver operat?ing characteristic curve (ROC) was used to analyze the sensitivity, specificity and optimal cut off value of ACR for predicting HDCP. Results (1)There were significantly higher levels of ACR, 24-h urinary protein, blood urea nitrogen (BUN), serum creatinine (Cr), uric acid (UA), total cholesterol (TC) and low density lipoprotein cholesterol (LDL) and significantly lower val?ues of platelet (PLT), serum albumin and total protein in women with severe preeclampsia than those of other groups ( P <0.05). (2) There were positive correlation between ACR and 24-h urinary protein, BUN, Cr, UA, D-dimer, TC, LDL (P <0.05). (3) The optimal cut off value of ACR for predicting gestational hypertension, mild preeclampsia, severe preeclampsia (24-h urinary protein≥2 g,≥5 g) were 1.44, 10.48, 39.84, 94.91 g/mol separately. Conclusion There is positive correla?tion between ACR and 24 hour urinary protein. ACR can be used for diagnosing and estimating the severity degree of hyper?tensive disorders complicating pregnancy.

Predictive Value of Echocardiography in Peripartum Cardiomyopathy / 中国医学影像学杂志

Purpose To analyze the echocardiographic features of peripartum cardiomyopathy (PPCM) using Logistic regression, and to screen the indexes which can be used for the prognosis of PPCM. Materials and Methods Fifty patients who were diagnosed as PPCM by echocardiography were divided into recovered group (30 cases) and non-recovered group (20 cases), all the patients underwent echocardiography, left ventricular end-diastolic diameter (LVEDd) was measured in the parasternal long axis view, left ventricular ejection fraction (LVEF) was measured using Simpson biplane method in the apical four-chamber and two-chamber view to look for the evidence of left ventricular thrombosis, if complicated with pulmonary hypertension, continuous wave Doppler was used for measuring the peak velocity of tricuspid valve regurgitation, and estimating of pulmonary artery systolic pressure, Logistic regression model was established and receiver operating characteristic (ROC) curve was generated to evaluate the prediction value of Logistic regression model. Results Compared with non-recovered group, there was statistically significant difference of LVEDd, LVEF, left ventricular thrombosis, pulmonary hypertension and re-checked LVEF (t= -4.33, 7.64 and 11.54, P0.05). Three sonographic features LVEDd, LVEF and left ventricular mural thrombus) could be used for the establishment of Logistic model (χ2=5.14, 11.59 and 14.58, P<0.05). The prediction accuracy of the model was 90.0% (45/50, P<0.001) and the area under ROC curve was 0.945±0.030 (P<0.001). Conclusion Logistic regression analysis can be applied for the screening of ultrasound index for PPCM, LVEDD, LVEF and left ventricular wall thrombus can predict the prognosis of PPCM accurately.

Maternal and fetal outcomes with aortic dissection in pregnant patients with Marfan syndrome / 中华妇产科杂志

Objective To evaluate the clinical characteristics of aortic dissection in pregnant patients with Marfan syndrome and the maternal and fetal outcomes in cardiovascular surgery. Methods Seven pregnant women with Marfan syndrome with aortic dissection were identified, who were treated in Beijing Anzhen Hospital Affiliated to Capital Medical University between January 2012 and September 2014. Patient charts were reviewed for cardiovascular surgery, occurrence of complications, clinical features and the maternal and fetal outcomes. Results (1)Among 7 patients, 4 cases were diagnosed as type A aortic dissection and 3 were cases diagnosed as type B aortic dissection. The diagnosis mainly depends on CT angiography. New York Heart Association(NYHA)classify into 5 of levelⅡ, 1 of levelⅢ, 1 of leveIⅣ. Except for 1 patient with cardiac tamponade lead to heart failure, the remaining 6 cases had no complications.(2)Three patients underwent heart surgery with cardiopulmonary bypass in second trimester and two patients underwent heart surgery in third trimester. Two patients terminated pregnancy before heart surgery(one of whom underwent artificial abortion,one of whom underwent cesarean section in second trimester).(3)The methods of cardiovascular surgeries were as follow:3 of Bentall+Sun′, 1 of Bentall+Sun′+right coronary artery bypass grafting, 1 of Bentall, 1 of the whole chest aorta replacement surgery, and 1 of femoral artery catheter chest aorta with membrane mesh stent implantation. The diameter of aortic roots measured during operation were 5 cm in 2 cases, 7 cm in 2 cases and 10 cm in 2 cases respectively. Among the 7 cases, 3 were conducted cesarean sections during cardiovascular surgery,1 was terminated pregnancy due to intrauterine fetal death after cardiovascular surgery, and 1 was conducted cesarean section due to severe early-onset preeclampsia at 30 weeks of pregnancy after cardiovascular surgery. (4)Among the 7 cases, 3 were conducted cesarean sections during cardiovascular surgery, the order of which was implemented cesarean section under general anesthesia firstly and then operated cardiovascular surgery with cardiopulmonary bypass and heparinization. Two were conducted cardiovascular surgery after termination of pregnancy by early artificial abortion operation or hysterotomy. 1 was conducted Bentall surgery at 18 weeks of pregnancy, after that the patient was receiving warfarin until the fetal brain hemorrhage was examined by ultrasound at 31 weeks of pregnancy;the patient was conducted cesarean section due to intrauterine fetal death. 1 was conducted heart surgery at 24 weeks of pregnancy and continue the pregnancy to 30 weeks, was conducted cesarean section due to severe early-onset preeclampsia at 30 weeks of pregnancy.(5)Among 7 patients, three patients underwent heart surgery with cardiopulmonary bypass combined with cesarean section. Two patients terminated pregnancy after heart surgery. Two patients terminated pregnancy before heart surgery. Six patients were alive and one patient died of multiple organ failure.(6)3 cases of newborn with birth body mass between 1 080 to 1 490 g.1 case of birth died after 14 d. 2 cases for newborns were alive without exception. Conclusion Aortic dissection poses serious risk for pregnant women with Marfan syndrome and the fetus, and the mortality rates for both the mother and the fetus are high. Early diagnosis and appropriate treatment should be based on maternal and fetal conditions (such as aortic dissection, gestational age). When fetus is mature, cardiovascular surgery should be carried out with cesarean section. A multi-disciplinary team between obstetric and cardiovascular surgery is crucial to the outcome of these critical patients.

Analysis of high risk factors for patient death and its clinical characteristics on pregnancy associated with pulmonary arterial hypertension / 中华妇产科杂志

Objective Study of pulmonary hypertension (PAH) during pregnancy has characteristics of the high risk factors for patient death and its clinical characteristics.Methods Death in patients with clinical data was collected from January 2006 to October 2013 in Beijing Anzhen Hospital Affiliated to Capital Medical University treated 8 cases of pregnancy complicated with PAH in hospital. According to the mechanism of PAH patients will be divided into two categories, Idiopathic pulmonary arterial hypertension (IPAH) in 4 cases, 4 cases of secondary PAH [are secondary to congenital heart disease, also known as congenital heart disease associated PAH (CHD-PAH)]. Analyze the clinical features of 8 cases of patients and pregnancy outcome.Results (1) In 8 patients,4 cases were IPAH, none of them with primary diseases, and they were complicated with severe tricuspid regurgitation. 4 cases were CHD-PAH, all with Eisenmenger′s syndrome. 8 patients were not preconception counseling and regular prenatal examination. (2) The pregestational cardiac function of 8 cases was gradeⅠ-Ⅱ, and it was gradeⅢ-Ⅳon admission. The estimation pressure (sPAP) of pulmonary artery systolic by echocardiography was 101 mmHg (1 mmHg=0.133 kPa). In 8 patients, 7 cases were in pregnancy 27 weeks and beyond for treatment since the clinical symptoms increased, 1 case of pregnant 18 weeks for treatment caused by the increased clinical symptoms. (3) In 8 patients, 1 patient with CHD-PAH secondary to patent ductus arteriosus, its sPAP was 170 mmHg, dead at 12 hours after admission; the remaining 7 cases termination with cesarean section. 4 patients with IPAH were continuous epidural anesthesia, including 1 case for the intraoperative PAH crisis and respiratory and cardiac arrest with general anesthesia, 3 cases of CHD-PAH patients in 1 case with continuous epidural anesthesia, 2 cases of general anesthesia.(4) In 8 patients, 7 cases of median death time were 3 days after delivery, including 4 cases of IPAH patients death for 2.5 days after delivery;the causes of death were PAH crisis and heart failure. Time of death in 4 cases of CHD-PAH, 1 case was dead at 12 hours after admissions, the remaining 3 cases median death time were 13 days after delivery;the death causes for 4 cases of CHD-PAH were PAH crisis and multiple organ failure. (5) In 8 patients, 1 patient with CHD-PAH secondary to patent ductus arteriosus in gestational week 31 stillbirths occur. 1 case of pregnant 19 weeks had treatment of caesarean operation, the remaining 6 cases respectively at 28-30 weeks of gestation live birth, neonatal survival. (6) Before delivery, 4 cases of IPAH and 3 cases of CHD-PAH patients treated with alprostadil, iloprost, sildenafil, reduction of pulmonary artery pressure treatment, 1 case of CHD-PAH patient was dead after 12 hours in hospital, no drug treatment.Conclusions (1) PAH in patients need for consultation prior to conception, pregnancy must conduct regular prenatal examination, symptoms occur during pregnancy, the cardiac function was significantly decreased, and no improvement of drug treatment should be early terminated the pregnancy. (2) Compared with the pregnant women with CHD-PAH, faster progress and poor prognosis in patients with IPAH disease. (3)The patients during cesarean operation or intrapartumare easy to cause PAH crisis and heart failure or multiple organ failure. Taking active measures to maintain stability of hemodynamics is the key to prevent the occurrence of death of pregnant women with PAH.

Clinical study on sildenafil in treatment of pregnant women with pulmonary arterial hypertension / 中华妇产科杂志

Objective To explored the effect of sildenafil in treatment of pregnant women with pulmonary arterial hypertension.Methods From January 2012 to November 2013,64 pregnant women with pulmonary arterial hypertension (PAH) were randomly divided into group and control group.Control group:16 cases with mild and 16 cases with moderate PAH.To treatment with low-flow oxygen,low-salt diet therapy,cardiac,etc.sildenafil group:15 cases were mild pulmonary hypertension,and 17 cases moderate PAH.Treatment sildenafil 25 mg,rid in this study.Then the variation of the blood oxygen saturation,pulmonary artery systolic pressure,hemodynamic parameters and pregnancy outcome,including delivery modes,neonatal weight,morbidity of mother and fetus were compared.Results (1) Cardiac function and pulmonary hypertension:control group:the proportion of cardiac functional class Ⅰ-Ⅱ reduced from 81％ (26/32) to 56％ (18/32) significantly after treatment (P ＜ 0.05).Sildenafil group:the proportion of cardiac functional class Ⅰ-Ⅱ increased from 75％ (24/32) to 84％ (27/32) significantly after treatment (P ＜ 0.05).Between two groups,the proportion of mild and moderate turning to server PAH patients were significant differentce (P ＜ 0.05).(2) The pregnancy outcome of two group:the premature birth rate,low birth weight rate and cesarean section rate of 9％ (3/32),9％ (3/32)and 69％ (22/32)in sildenafil group were significantly lower than 16％ (5/32),19％ (6/32) and 81％ (26/32) in control group (P ＜0.05).The rate of vaginal delivery,term pregnancy and neonatal weight of 31％ (10/32),91％ (29/32) and (3 214 ±306) g in sildenafil group were different with 19％ (6/32),84％ (27/32) and (3 004 ±458) g in control group (P ＜ 0.05).(3) Hemodynamic parameters:in control group,arterial partial pressure of oxygen,oxygen saturation and left ventricular ejection fraction,pulmonary systolic pression were (80 ± 5) ％ to (72 ±8)％,(87 ±8) to(83±9) mmHg(1 mmHg=0.133 kPa),0.77 ±0.24 to 0.70 ±0.38 and (63 ±9) to (69 ± 12) mmHg before and after treatment,which showed remarkable decreased trends(P ＜ 0.05).The other parameter were not significantly different (P ＞ 0.05).In sildenafil group,arterial partial pressure of oxygen,oxygen saturation and left ventricular ejection fraction,pulmonary systolic pression showed increased trend before and after treatment,which were (80 ± 9) ％ to (88 ± 9) ％,(84 ± 3) to (89 ± 7) mmHg,0.70 ± 0.32 to 0.79 ± 0.27 (P ＜ 0.05),in the mean time,pulmonary systolic pression showed decreased trend from (65 ± 18) to (60 ± 13) mmHg (P ＜0.05).The other parameter did not show significant different (P ＞ 0.05).Conclusions Sildenafil treatment can significantly improve the clinical symptoms,cardiac function and hemodynamic parameters.It also could significantly improve pregnancy outcomes,reduce premature delivery,the incidence of low birth weight children,and cesarean section rate.

Severe idiopathic pulmonary arterial hypertension in pregnancy: a review of 10 cases / 中华妇产科杂志

Objective To study the impact of severe idiopathic pulmonary arterial hypertension (IPAH) on pregnancy outcome and to investigate the effect of multidisciplinary approach during pregnancy on the pregnancy outcome in pregnant woman with severe IPAH.Methods Between March 2007 and November 2013,10 pregnant women with severe IPAH undergoing treatment in Beijing Anzhen Hospital were studied retrospectively.Hemodynamic measurements,medical therapy,manner of delivery,anesthetic administration,multidisciplinary management and outcomes were assessed.Results All 10 cases were first diagnosed at the mean of (24 ± 3) weeks during the pregnancy.The systolic pulmonary artery pressure (sPAP) estimated by transthoracic echocardiography was (95.6 ± 1.3) mmHg (1 mmHg =0.133 kPa).Seven cases of class Ⅲ and 3 cases of class Ⅳ were recorded by World Health Organization functional class.One patient underwent pregnancy termination at gestational age of 21 weeks with no maternal death or complications,nine patients continued pregnancy and all the patients underwent cesarean section.The mean pregnancy length was (31 ± 5) weeks.Nine had cesarean deliveries during continuous epidural anesthesia,and one during general anesthesia.There were three maternal deaths in hospital (5,2,3 days postpartum),and seven patients were alive,and the average hospitalization days was (8 ± 4) days.One fetus lost with cesarean section.Two were term delivery,and seven cases were premature delivery.The average weight is (1 948 ± 731) g and nine were alive and no malformation.Conclusions Because of maternal mortality in patients with severe IPAH remains prohibitively high,patients should continue to be counseled to avoid pregnancy.Women with severe IPAH who become pregnancy should be followed by multidisciplinary approach,and cesarean deliveries during continuous epidural anesthesia are a relatively safe way for pregnancy termination in patients with severe IPAH.