[Reimplantation of heart valve prosthesis at the 25th week of gestation]. / Reimplantace umelé srdecní chlopne ve 25. týdnu tehotenství.

OBJECTIVE: Pregnancy in a woman with thrombosis of heart valve prosthesis at the 25th week of gestation and fetal death during reimplantation of prosthesis with the use of extracorporeal circulation. SUBJECT: Case report. SETTING: Department of Gynecology and Obstetrics, 2nd Medical Faculty of Charles University, Motol Hospital, Prague. SUBJECT AND METHOD: Patient L. S., 24 years old, first pregnancy, admitted to coronary heart unit at the 25th week of gestation with a blocked heart valve prosthesis, NYHA IV, left heart failure, and pulmonary edema. There was an insufficient anticoagulation therapy during pregnancy and a thrombosis of the prosthetic heart valve was suspected from that reason. Reimplantation of a prosthetic heart valve with the use of extracorporeal circulation was indicated in spite of a possible risk for the fetus. The thrombosis was confirmed during cardio surgical operation and a change of the prosthesis was successfully performed. After the patient was converted to extracorporeal circulation, bradycardia and intrauterine fetal death occurred. With regard to the patient's coagulation and circulatory instability, further management was necessary because of fetal death--termination of pregnancy by minor caesarean section was the only alternative. Six hours later an 850 g weight dead fetus was delivered. There were no serious complications during the postoperative period. CONCLUSION: Reimplantation of a prosthetic heart valve from vital indication was performed at the 25th week of gestation. After conversion of mother to extracorporeal circulation, fetal death occurred. The patients was released with satisfactory cardiopulmonal compensation.

[Turner syndrome: overview of problems, present status, proposals for care and a protocol for monitoring in childhood, adolescence and adulthood]. / Turneruv syndrom: prehled problematiky, soucasný stav, návrh koncepce péce a protokol sledování v detství, adolescenci a v dospelosti.

BACKGROUND: Girls and adolescents with Turner syndrome (TS) usually receive intensive medical care in a multidisciplinary team, coordinated by paediatric endocrinologist. Majority of them are discharged from specialist clinics following the induction of puberty and attainment of final height. Patients with Turner syndrome have a reduced life expectancy, they are known to have multi-system impairments in addition to the short stature and to the absence of sexual development. Aim of this study is to propos a continuous follow-up by multidisciplinary team of physicians starting in childhood and following the discharge from the paediatric care. METHODS AND RESULTS: This paper highlights the medical and psychosocial problems associated with Turner syndrome in childhood, adolescence and in adulthood. Analysis of these problems served as a background to management strategy. CONCLUSIONS: Women with Turner syndrome are at risk of number of medical problems. Quality of their life and the life expectancy can be improved with increasing awareness to comorbities associated with Turner syndrome. Assisted reproduction technologies has recently offered a chance for pregnancy and delivery of a healthy child also to women with Turner syndrome. Therefore, long-term follow-up by multidisciplinary team of physicians knowledgeable about these medical problems is necessary. Introduction of a centralised system of systematic multidisciplinary approach to patients with Turner syndrome from childhood and adolescence to adulthood seems to be very important.

Are cyanosed adults with congenital cardiac malformations depressed?

OBJECTIVE: To assess the incidence of depression, and the ability to interact socially, in adult patients with chronic cyanosis and congenital cardiac malformations. DESIGN: Prospective study of consecutive patients. SETTING: Single institution, tertiary referral centre. PATIENTS: Between 1993 and 2000, we assessed 76 patients with congenital cardiac malformations and persistent cyanosis, having a median age of 36.5 years, with a range from 19 to 64 years, at the time of referral. Female patients accounted for just under half (48.6%) of the sample. Just under two-fifths of the cohort (39.5%) had functionally univentricular cardiac anatomy, while 14.8% had tetralogy of Fallot with pulmonary atresia and aorto-pulmonary collateral arteries, and 17% had the Eisenmenger syndrome. During the period of follow-up, 17 (22.4%) of the patients died. ASSESSMENT: We used clinical interviews and non-invasive assessment, employing Zung's questionnaire which provides a scale for the self-rating of depression. On this scale, a score above 50 points is indicative of depression. RESULTS: Of the survivors, 32 (54%) completed the self-rating questionnaires. Of these, 20 responders (63%) considered that they lead full lives, including sexual activities, while 26 (81%) had never harboured suicidal thoughts. Depression was diagnosed in 11 responders (34%), with a mean score of 66.9, standard deviation of 8.7, and a range from 53 to 89. The remaining 21 patients (66%) were without signs of depression, scoring a mean of 41.5, with standard deviation of 5.5, and a range from 35 to 46. Depression was associated with older age (40.5 years versus 33.5 years, p = 0.01), worse functional state in the classification of the New York Heart Association (2.95 versus 2.48, p = 0.03), and unemployment (p < 0.0001), but independent from the severity of cyanosis, the level of the haematocrit, the saturation of oxygen, or previous surgical treatment. CONCLUSIONS: To our knowledge, this is the first evidence suggesting a relatively high incidence of depression in adults with congenital cardiac malformations and persistent cyanosis. Larger, multi-centric studies will be needed to confirm or refute these findings.

[Transcatheter closure of ostium secundum atrial septal defects--initial experience with treatment of adult patients]. / Katetrizacní uzáver defektu septa síní typu ostium secundum--nase první zkusenosti s lécbou dospelých nemocných.

The authors present their initial experience with the use of Amplatzer septal occluder for transcatheter closure of secundum atrial septal defect. For patients where the secundum atrial septal defect type is haemodynamically significant or when paradoxical embolization via the defect is suspected, the catheterization technique is an alternative for cardiosurgical correction of the defect. The morphology of the defect which is the basic criterium for indication of a transcatheter closure is evaluated on transoesophageal echocardiographic examination. The closure takes place under skiascopic and transoesophageal echocardiographic control. The procedure in all three patients (2 women--age 58 and 62 years, 1 man--24 years) treated at our department was without complications. Consistent with formerly published data it may be concluded from our initial experience that when all indication criteria are respected the closure of secundum atrial defect by means of Amplatzer septal occluder is a safe technique and a relatively easy operation with a high rate of primary success.

[The most frequent congenital heart defects in the adult population and their echocardiographic diagnosis]. / Nejcastejsí vrozené srdecní vady v dospelé populaci a jejich echokardiografická diagnostika.

The congential heart disease (CHD) occur inabout 6% of all live newborn infants. About 77% of children with CHD live to adulthood. The number of adult patients with CHD increases owing to thedevelopment of cadiology, improved possibilities of non-invasive diagnostic methods with use of echocardiography and esophageal echocardiography. The clinic for adult patients with congenital heart defects at the Faculty Hospital in Motol registers 1,100 patients. The most frequent anomalites include atrial septal defects (26%), ventircular septal defects (21%), aortic coarctation (15%) and Fallot's tetralogy (8%). Complex cyanotic CHD represent the total of 7% of patients, as well as the congenital aortic stenosis or incompetence (7%) and pulmonary stenosis (7%).

Prognostic value of the amount of dysfunctional but viable myocardium in revascularized patients with coronary artery disease and left ventricular dysfunction. Investigators of this Multicenter Study.

OBJECTIVES: The purpose of our study was to assess the prognostic importance of the amount of dysfunctional but viable myocardium in revascularized patients with coronary artery disease (CAD) and left ventricular (LV) dysfunction. BACKGROUND: The amount of dysfunctional but viable myocardium predicts the functional improvement after revascularization and may offer more precise risk stratification of patients referred for bypass surgery or coronary angioplasty. METHODS: Two hundred and seventy-four consecutive patients with CAD and LV ejection fraction < or =40% underwent low-dose dobutamine echocardiography for viability assessment. One hundred and thirty-three of them were revascularized using either coronary artery bypass surgery (118 patients) or coronary angioplasty (15 patients) and entered this study. To quantify the amount of dysfunctional but viable myocardium, wall motion was scored using 16-segment model. The dysfunctional segments were defined as viable if they exhibited improvement in their thickening by at least 1 grade with dobutamine infusion. The patients were followed up for a mean period of 20+/-12 months (range, 2 to 48) for cardiac mortality and nonfatal cardiac events including myocardial infarction, unstable angina pectoris requiring hospitalization and hospitalization for heart failure. Standard follow-up echocardiography was performed 3 to 6 months after revascularization. RESULTS: Twenty-nine patients exhibited a large amount of dysfunctional but viable myocardium (> or =6 segments, group A), 60 patients had a small amount of dysfunctional but viable myocardium (2 to 5 segments, group B) and 44 patients were found to have dysfunctional myocardium irreversibly damaged (group C). Similar prerevascularization LV ejection fractions of 35%+/-5%, 34%+/-4%, 36%+/-4% in groups A, B and C increased to 47%+/-6% (p < 0.01 vs. baseline, p < 0.01 vs. groups B and C), to 40%+/-5% (p < 0.01 vs. baseline) and to 37%+/-6% (p = NS vs baseline), respectively, after revascularization. The greatest functional improvement after revascularization in group A patients was accompanied by a lower rate of cardiac events during follow-up (2 vs. 18 in group B, p < 0.05, and vs. 17 in group C, p < 0.01) and better cardiac event-free survival according to Kaplan-Meier survival analysis (p < 0.05 vs. groups B and C, respectively). CONCLUSION: In revascularized patients with CAD and moderate or severe LV dysfunction, the presence of a large amount of dysfunctional but viable myocardium identifies patients with the best prognosis.

Atrial septal defect in adults.

BACKGROUND: The value of operation of atrial septal defect (ASD) in adults, especially after 40 years, is still discussed. METHODS AND RESULTS: In 1994-95 57 adults with unoperated ASD were examined clinically, echocardiographically and in 75% by catheterization. Type primum was present in 11%, type secundum in 77% and sinus venosus in 11%. Group A comprised 28 patients aged 20 to 40 years (average 29), group B comprised 29 patients aged 40 to 62 years (average 51). The groups (B:A) did not differ in pulmonary to systemic flow (Qp/Qs) (2.4:2.2) or pulmonary arteriolar resistance (PAR) (group B 2 U.m2, group A 1.7 U.m2), the older patients had worse New York Heart Association (NYHA) classification, more frequent tricuspid regurgitation (group B 96%, group A 45%), significantly larger right ventricles and pulmonary arteries, higher mean pulmonary artery pressure (group B 26 mmHg, group A 17 mmHg) and right ventricle end-diastolic pressure (RVEDP group B 10, group A 8.8 mmHg). All defects larger than 10 mm by transesophageal echocardiography (TEE) had Qp/Qs 1.5 or more. Forty patients were operated with zero mortality, in three cases by minithoracothomy. Postoperatively, 50% of group A and 63% of group B felt better, NYHA classification was significantly better in both groups. Tricuspid regurgitation decreased in both groups as well as the size of right ventricle. The size of the left ventricle enlarged after operation in group A. CONCLUSION: This study suggests to operate adults with ASD larger than 10 mm by TEE with signs of right ventricle overload and/or Qp/Qs 1.5 or more, who have normal PAR. Operative mortality was zero in both age groups, the functional repair was better in younger patients (under 40 years).

[Congenital heart defects in adults worldwide and in the Czech Republic]. / Vrozené srdecní vady dospelých ve svete a u nás.

Congenital heart disease of adults is a new area of cardiology with specific diagnostic, therapeutic, social and economic problems of these patients. The First Medical Clinic of the Second Medical Faculty, Charles University assembled so far data on more than 1200 adult patients with congenital heart disease from different districts in Bohemia and Moravia. Atrial septal defects are recorded most frequently (25.7%), followed by ventricular septal defects (25.0%) and coarctation of the aorta (12.4%). 50.3% of the patients were treated by surgery, most frequently patients with a patent ductus arteriosus--in 83.8%, with coarctation of the aorta (82.8%) and an atrial septal defect (60.5%). The authors review reasons why for the care of these patients in other countries specialized centres developed and the foundation of such a centre in the Czech Republic is discussed.

[Transesophageal echocardiography in adults with congenital heart defects]. / Transezofageální echokardiografie u dospelých s vrozenými srdecními vadami.

The authors give an account of their experience with transesophageal echocardiography (TEE) in 61 adult patients with congenital heart disease. The main indications for examination in this group of patients were: confirmation of an atrial septal defect, unsatisfactory visualization, in particular in complex defects, evaluation of the results of surgical correction, detailed valvular morphology, in particular of the mitral and aortic valve, search for the source of embolization, suspected aneurysm of the aorta. A high diagnostic yield of TEE was obtained in: defects of the atrial septum, or other pathological conditions affecting the atrium, defects of the atrioventricular septum (AV), pathology of the AV valves, in particular the mitral one, pathology of the aortic valve and the efflux tract of the left ventricle, aortic arch and its descendent part, transposition of the large arteries or other complex defects, incl. conditions following surgical correction. A small diagnostic contribution of TEE was obtained when visualizing defects of the ventricular septum, pulmonary valves and the efflux tract of the right ventricle and the distal portions of the ascendant aorta.