Severe thrombotic microangiopathy accompanied by liver rupture and multiorgan failure at week 26 of pregnancy.

OBJECTIVE: Case of a primigravid woman who suffered from severe PTMS (postpartum thrombotic microangiopathy syndrome) in the 26th week of pregnancy. DESIGN: Case report. SETTING: Department of Gynecology and Obstetrics, Hospital Nový Jičín; Department of Gynecology and Obstetrics, University Hospital Ostrava; Department of Hematooncology, University Hospital Ostrava; Department of Anaesthesiology and Resuscitation, University Hospital Ostrava. RESULTS: A thirty-one-year old primigravid woman was admitted to a secondary level institution due to epigastric pain and spontaneous rupture of membranes at 26th week of pregnancy. On admission her blood pressure was 140/90 mm Hg and an intrauterine fetal death was confirmed. The patients condition deteriorated quickly, resulting in a hypertensive crisis (220/120 mm Hg), which did not respond to medication over a two hour period. Emergency caesarean section was performed, but the patients condition progressed to HELLP syndrome class I, DIC and MOF. She was transferred to the intensive care unit (ICU) of the district referral hospital 38 hours postpartum. On admission to ICU, liver rupture was diagnosed which was managed conservatively. Therapeutic plasma exchange (TPEX) was initiated on day 2 postpartum in response to falling platelets and continued for 6 days. Due to acute kidney injury (AKI), the patient required dialysis for 21 days. The patients condition improved gradually and at 28 days after admission to ICU she was transferred back to the referring hospital. The consensus reached by the treating teams was that PTMS was the most likely diagnosis. CONCLUSION: This case demonstrates that PTMS improves (usually rapidly) after TPEX is initiated. It also emphasises the importance of maintaining a high index of suspicion for PTMS so that life-saving TPEX can be initiated, because it does not respond to classical treat-ment used in the management of HELLP syndrome. Other research suggests patients may also require a terminal complement blockade with the anti-C5 monoclonal antibody (eculizumab). Further research could focus on diagnostic tests to distinguish PTMS from HELLP to identify which patients would most benefit from these treatments.

Echogenic foci in fetal heart from a pediatric cardiologists point of view.

OBJECTIVES: To evaluate the occurrence and the significance of echogenic foci in the fetal heart and to assess the prognosis of the fetus and child. SETTING: Department of Pediatrics and Prenatal Cardiology, Department of Pediatrics, University Hospital, Faculty of Medicine, Ostrava. DESIGN: Original article. METHODS: A retrospective study was conducted between 2008-2017. Fetal echocardiography was performed in the second trimester of pregnancy in the study population. The identification of echogenic heart foci, and their follow up during and after the pregnancy were performed by a pediatric cardiologist. RESULTS: In the monitored period, a total of 27,633 fetuses were examined. Isolated cardiac hyperechogenic foci were detected in 3% (829/27,633) of the fetuses. The foci was found in 93%, 5%, and 2% in the left ventricle, mainly in valvular apparatus of the mitral valve, in the both ventricles, and in the right ventricle, respectively. In 1% (11/829) of the fetuses with cardiac echogenic foci, the others concomitant pathologies (tricuspid regurgitation, extrasystoles, renal pathology) were found. No genetic abnormalities were detected in the fetuses with cardiac hyperechogenic foci. CONCLUSION: The echogenic focus in fetal heart is a relatively common, mostly insignificant finding, with any serious consequences for the fetus and the child.

[Development of prenatal diagnostics of congenital heart defects, profit of standardized scanning planes]. / Vývoj prenatální diagnostiky vrozených srdecních vad, zisk z jednotlivých ultrazvukových projekcí.

OBJECTIVE: To audit the development and success rate of prenatal detection of congenital heart defects (CHDs), and to evaluate the effectiveness of diagnostics performed in standardized scanning planes. SETTING: Department of Pediatrics, University Hospital Ostrava. DESIGN: Retrospective study. METHODS: Ultrasound examination of fetal heart (fetal echocardiography) was performed in the second trimester pregnancy. The observed region was the Moravian-Silesian region; the assessment was performed in the retrospective study performed between 2000- 2016. The knowledge of all significant heart defects in the region, processing of data from genetic reporting, further examination of all prenatal pathologies by a pediatric cardiologist, presence of a pediatric cardiologist at all autopsies, with a precise description of the defect, birth of a pathological new-born at a specialized centre. Analysis of detected CHDs was performed in relation to the ultrasound scans used. RESULTS: During the monitored 17-year period, a total of 748 (3.8 cases per 1,000 foetuses) of prenatally identified and postnatally significant CHDs were observed in the total population of 198,300 foetuses. There were 53% (393/748) CHDs detected prenatally and 47% (355/748) of cases were not prenatally recognized. The effectiveness of CHD screening has improved progressively, from the initial 10% up to the current 74%. The best results were obtained using the basic four-chamber (4CH) scan; the results in practice gradually decreased, from the basic 4CH projection to the aortic arch. CONCLUSION: The effectiveness of prenatal detection of congenital heart defects gradually improves, namely in cases of hypoplasia and significant ventricular anomalies, with up to 100% prenatally detected cases in the past three years. The level of detection statistically decreases, from the four-chamber projection to out-flow tracts, great arteries and the aortic arch. Congenital heart defect is generally well detectable prenatally, and is usually observed as an isolated anomaly. The most important factors include a precise diagnosis, overall examination of the pregnancy and correct counselling provided for the affected family.

[HELLP syndrome requiring therapeutic plasma exchange due to progression to multiple organ dysfunction syndrome with predominant encephalopathy, respiratory and renal insufficiency]. / HELLP syndrom vyzadující plazmaferézu pro rozvoj multiorgánové dysfunkce s dominující encefalopatií, respiracní a renální insuficiencí.

OBJECTIVE: Case report of woman with twin pregnancy complicated by HELLP syndrome which progressed to multiple organ dysfunction syndrome with predominant encephalopathy, renal and respiratory insufficiency with the need to perform repeated therapeutic plasma exchange. DESIGN: Case report. SETTING: Department of gynecology and obstetrics, University Hospital in Ostrava; Departmet of hematooncology, University Hospital in Ostrava; Department of gynecology and obstetrics, Vsetín hospital; Department of hematology and transfusion, Vsetín Hospital. RESULTS: Case of 35-year-old III gravida/II para with previously normal ongoing twin bichorionic biamniotic pregnancy in week 35+0, which was admitted to secondary care delivery room for three days lasting "flu like" symptoms and the right upper quadrant pain. She was icteric, exhausted, but normotensive (120/75 mm Hg). Acute caesarean section was performed for suspected fetal hypoxia and HELLP syndrome. The laboratory exams confirmed coagulopathy and HELLP syndrome second class during the operation. Blood loss was 800 ml. Despite standard treatment of HELLP syndrome, the condition had developed to renal insufficiency, bilateral fluidothorax, alveolar pulmonary edema, encephalopathy and hypertension. In laboratory results dominates markers of coagulopathy, thrombocytopenia and microangiopathic hemolytic anemia with presence of schistocytes. Due to multiple organ dysfunction syndrome with hemolysis of unclear origin, patient was transferred to referral hospital on sixth postoperative day. Therapeutic plasma exchange (TPEX) was promptly begun. Improvement of laboratory parameters occurred already after the first TPEX and after two days there was a significant improvement of neurological status. Nine TPEX procedures were performed. The treatment was terminated after platelet count reached 100×109/l. She was discharged from hospital 21 days after delivery. After exclusion of other clinical entities, the case was closed as postpartum thrombotic microangiopathic syndrome. CONCLUSION: Postpartum thrombotic microangiopathic syndrome includes states, which resemble HELLP syndrome with their laboratory result and clinical expression, but their behavior is different - progressively worsening with signs of disseminated intravascular coagulation and complex microangiopathy with multiple organ dysfunction. It is not responding to classic treatment of HELLP syndrome. In this cases usually improvement of patients condition follow initiation of therapeutic plasma exchange.

Early identification of women with HELLP syndrome who need plasma exchange after delivery.

OBJECTIVES: To compare the laboratory course of HELLP syndrome between patients who recover and those who progress to postpartum thrombotic microangiopathic syndrome (PTMS) and require postpartum plasma exchange (PPEX) and to describe maternal characteristics and morbidity in women with PTMS. METHODS: In this retrospective analysis, 81 patients recovered and 5 progressed. Values for aspartate aminotransferase (AST), lactate dehydrogenase (LDH), bilirubin, platelets (Plt), urea, and creatinine at 0, 8, 16, 24, 48, and 72 hours postpartum in both groups were analyzed and compared. We also described maternal characteristics and morbidity of patients who progressed to PTMS. RESULTS: Patient groups differed significantly at 72 hours postpartum for Plt and LDH values and at 24 and 48 hours for bilirubin. Trends for AST and Plt differed significantly between the recovery and progression groups in the first 48 hours. Patients who progressed had acute kidney injury and other severe maternal morbidity, including one case of maternal death. CONCLUSIONS: Women with HELLP syndrome without clear Plt and AST improvement in the first 48 hours and with acute kidney injury, neurological impairment, or respiratory distress syndrome are at risk of progressing to PTMS. They should be administered PPEX between 24 and 72 hours postpartum.

[Folic acid and prevention of the neural tube defects]. / Kyselina listová a prevence rozstepových vad centrálního nervového systému.

OBJECTIVE: Overview of neural tube defect in relation to use of folic acid among fertile-age women. DESIGN: Review article. SETTING: Obstetrics and gynecology department OU and FN Ostrava. METHODS AND RESULTS: Neural tube defects (NTDs) are one of the most common birth defects in the Czech Republic. The relation between using of folic acid and decrease of the incidence NTDs was first described in 1965. Fertile-age women are not able to get enough folate from their diet, therefore right timing and proper dosing of folic acid is the object of numerous studies. Many countries started theirs food fortification programs because of the high percentage of unplanned pregnancies and often low compliance. CONCLUSION: The role of folic acid in prevention of NTDs is undeniable. Due to the large number of unplanned pregnancies long-term supplementation is recommended for all fertile-age women. If pregnancy is planned, the suplementation should begin at least one month before pregnancy. In both cases it is necessary to continue until the end of the week of 12. dosage should be individualized according to risk.