Evaluation of contraceptive methods in women with congenital heart disease in Germany, Hungary and Japan.

AIMS: For women with congenital heart defects (CHD), pregnancy may pose a health risk. Sexually active women with CHD without the desire for own children or for whom pregnancy would imply considerable health risks require adequate counselling regarding appropriate contraception. This study gathers data on the contraceptive behaviour of women with CHD from three different cultural regions. METHODS AND RESULTS: 634 women with CHD from Germany, Hungary and Japan were surveyed regarding contraception and contraceptive methods (CM) used. The patients were divided into groups according to different criteria such as pregnancy associated cardiovascular risk or "safety" of the contraceptive methods used. 59% of the study participants had already gained experience with CM. The average age at the first time of use was 18.4 years; the German patients were significantly younger at the first time of using a CM than those from Hungary and Japan. Overall the condom was the method used the most (38%), followed by oral contraceptives (30%) and coitus interruptus (11%). The range of CM used in Japan was much smaller than that in Germany or Hungary. Unsafe contraceptives were currently, or had previously been used, by 29% of the surveyed patients (Germany: 25%, Hungary: 37%, Japan: 32%). CONCLUSION: Most women with CHD use CM. There are differences between the participating countries. Adequate contraceptive counselling of women with CHD requires considering the individual characteristics of each patient, including potential contraindications. For choosing an appropriate CM, both the methods' "safety", as well as the maternal cardiovascular risk, are important.

Comparison of transpulmonary thermodilution, transthoracic echocardiography and conventional hemodynamic monitoring in neonates and infants after open heart surgery: a preliminary study.

BACKGROUND: Transpulmonary thermodilution (TPTD) is an increasingly popular method used to monitor the complex hemodynamic changes in critically ill children. The purpose of our study was to examine the relationship between transthoracic echocardiographic (TTE) parameters and global hemodynamic variables derived from TPTD and those derived from conventional measurements in infants and neonates undergoing corrective cardiac surgery. METHODS: After approval from the Ethics Committee of Gottsegen György Hungarian Institute of Cardiology and individual parental consent were obtained, patients were prospectively enrolled. In parallel with continuous postoperative conventional monitoring, TPTD was measured four times daily, and TTE was performed once per day. Conventional hemodynamic, TPTD and TTE parameters were compared with weighted linear regression statistics and a Pearson correlation. RESULTS: One hundred forty-five TPTD measurements and 35 TTE examinations of thirteen enrolled patients were analyzed. Global end-diastolic volume index (GEDVI) was correlated with the fractional shortening (SF, r=0.67, P=0.001) measured by TTE. Among the preload parameters, the percentage change of GEDVI between two consecutive time points showed a pertinent correlation with changes of cardiac index (r=0.67, P=0.001) and changes of stroke volume index (r=0.57, P=0.008). Percentage changes in SF demonstrated a strong negative correlation with changes of left ventricular end-systolic diameter (r=-0.86, P<0.001). There was no significant relationship between alterations in arterial or central venous pressure values with TTE or TPTD parameters. CONCLUSION: Both TPTD and TTE may be used in the estimating volumetric preload parameters. The time course of TPTD-derived parameters may have clinical relevance in pediatric critical care practice.

Dwarfism and cytochrome P450-mediated C-6 oxidation of plant steroid hormones.

BRs (brassinosteroids) are plant steroid hormones that are essential for normal plant development. The dramatic dwarfism exhibited by mutants in the CYP (cytochrome P450) enzymes involved in BR biosynthesis indicates a role for these hormones in plant growth and development. Since the mid-1990s, collaborative research has been geared towards developing a better understanding of the CYP85 class of CYPs involved in BR biosynthesis in both Arabidopsis and tomato. Some of the most recent observations include the fact that certain CYP85 CYPs catalyse the synthesis of the most bioactive BR, BL (brassinolide). Current evidence suggests that evolution of this function may have occurred independently in different dicotyledonous species. Interestingly, BL accumulates in tomato fruits, highlighting a key role for this hormone in fruit development. At the same time as developing a better understanding of the enzymatic function of these CYPs, we have also carried out experiments towards characterizing where and when these genes are expressed and mechanisms of their regulation. As expected for a hormone involved in growth and development, biosynthetic gene promoter activity is associated with young rapidly growing cells and with fruit development.

The future of cardiovascular imaging and non-invasive diagnosis: a joint statement from the European Association of Echocardiography, the Working Groups on Cardiovascular Magnetic Resonance, Computers in Cardiology, and Nuclear Cardiology, of the European Society of Cardiology, the European Association of Nuclear Medicine, and the Association for European Paediatric Cardiology.

Advances in medical imaging now make it possible to investigate any patient with cardiovascular disease using multiple methods which vary widely in their technical requirements, benefits, limitations, and costs. The appropriate use of alternative tests requires their integration into joint clinical diagnostic services where experts in all methods collaborate. This statement summarises the principles that should guide developments in cardiovascular diagnostic services.

Identification of virulence-associated genes of Pseudomonas viridiflava activated during infection by use of a novel IVET promoter probing plasmid.

Analysis of virulence mechanisms of plant pathogens is often limited by the lack of genetic tools that can be used to identify genes that are preferentially expressed during their interactions with plants. In the present study, we used the newly constructed IVET (in vivo expression technique) plasmid pIviGK and the corresponding antibiotic resistance-based selection method to identify genes that encode pathogenicity factors of the soft rot-causing bacterium Pseudomonas viridiflava. These included pel, the gene encoding pectate lyase, which is responsible for the development of soft rot symptoms. We have also isolated and characterized the gene mviNpv encoding a putative novel membrane associated virulence factor of P. viridiflava. A mutation in mviNpv was shown to influence motility as well as virulence of P. viridiflava. The mviNpv gene is expressed to a moderate level in LB media and its expression increases under inducing conditions as was shown by measuring in planta expression dynamics of the fused gfp reporter gene.

PEDMEANS: a computer program for the interpretation of pediatric electrocardiograms.

The interpretation pediatric electrocardiograms (ECGs) is complicated because of the strong age-dependency of the diagnostic criteria. We wanted to develop and evaluate a computer program for the interpretation of pediatric 12-lead ECGs. Continuous age-dependent normal limits were established based on ECGs from 1,912 healthy Dutch children. Additionally, a reference interpretation was obtained for 1,718 ECGs recorded at the Sophia Children's Hospital. The total set of ECGs was divided in a training set of 1076 ECGs and a test set of 642 ECGs. All ECGs were recorded at a sampling rate of 1,200 Hz. Based on the normal limits and the training set, diagnostic rules were formalized in an iterative process by using expert interviews and automatic rule induction. The resultant rules were evaluated on the test set. The performance of the program, on our study population, appears to justify its use in a clinical setting. Preferably, the program should also be evaluated in other clinical centers.

[Closure of the patent ductus arteriosus by means of cardiac catheterization]. / Ductus Botalli persistens zárása szívkatéteres módszerrel.

Authors report their results with transcatheter closure of patent arterial duct during a period of 1 year. Thirty patients underwent the procedure at the mean age of 5.22 yrs (range 5 mos-22.3 yrs) and mean bodyweight of 19.1 kg (range 5.8-73 kg). There were 9 males and 21 females. The diagnosis of the patent arterial duct was established by physical examination and noninvasive techniques. The procedures were attempted in all patients above 5 kg bodyweight with patent arterial duct and normal pulmonary artery pressure, irrespective of the shunt-size. All procedures were performed under general anesthesia in one session with the diagnostic cardiac catheterisation. Transcatheter closure was successful in 29 patients. In one patient the device embolized into the left pulmonary artery, snaring was unsuccessful, surgical closure of the patent duct and removal of the device took place uneventfully. Control aortography 15 minutes after the coil placement showed insignificant residual shunt through the patent duct in 8 patients (27%), while echocardiography at 1 month proved complete closure in all. The patients were followed by noninvasive methods. No mortality was observed. Authors emphasize the low risk and cost-effectiveness of the procedure. They stress the importance of the patients selection. Authors applied the technique for the first time and introduced it to the regular patient-care practice in Hungary.

[Management of aortic recoarctation by transluminal balloon angioplasty in childhood]. / A recoarctatio aortae kezelése transluminalis ballon-angioplasticával gyermekkorban.

Recoarctation balloon angioplasty is an interventional technique for the treatment of restenosis of the aortic isthmus after surgery. At present it is the treatment of choice prior to surgery. Authors report their experience with the first 30 patients in this country. Age less than 1 year at surgery is considered as a risk factor. The diagnosis of recoarctation was made by noninvasive techniques. Balloon angioplasty was performed in one session with the haemodynamic study. Twenty-nine successful procedures were performed, 1 patient only improved after the dilatation. Systemic hypertension decreased in all, but one patient, the diameter of the narrow segment increased in all patients. No mortality was observed. In one patient femoral artery injury developed without definite impairment of the circulation of the lower extremity. It is concluded, that balloon angioplasty is an effective and safe treatment, carries a lower risk for the patient, than surgery, and hospital stay is shorter. The procedure is cost-effective. Therefore, it is proposed for widespread use in the country.

Long-term follow-up (10 to 17 years) after Mustard repair for transposition of the great arteries.

BACKGROUND: The management strategies of patients who underwent Mustard repair for transposition (of the great arteries were changed in the 1970s: infants became eligible for direct surgical repair, so Blalock-Hanlon atrioseptostomy could be avoided, and cold cardioplegia was introduced for myocardial preservation. Data are lacking, however, regarding whether these changes have had positive effects on the long-term outcome. We therefore conducted a follow-up study on all 91 patients who underwent a Mustard repair for transposition of the great arteries in our institution between 1973 and 1980 to assess the incidence and clinical importance of sequelae as well as health-related quality of life for these patients. METHODS: Patients who were alive and could be traced through local registrar's offices received an invitation to participate in the follow-up study, which consisted of an interview, physical examination, echocardiography, exercise testing, and standard 12-lead and 24-hour electrocardiography. RESULTS: Patients operated on in the first 4 years had a significantly higher mortality rate and higher incidence of sinus node dysfunction than did patients operated on in the subsequent 4 years (25% vs 2% and 41% vs 3%, respectively). In contrast, the incidence of baffle obstruction necessitating reoperation was significantly higher in the second group. There were no significant differences in echocardiographic findings and exercise capacity between patients operated on in the first 4 years and in the subsequent 4 years. None of the patients had right ventricular failure; a mild degree of baffle leakage or obstruction was seen in 22% of the patients, and the mean exercise capacity was decreased to 84% +/- 16% of normal. CONCLUSION: The changes introduced between 1973 and 1980 have resulted in a considerable reduction of mortality and incidence of sinus node dysfunction but have also resulted in a more frequent need for reoperation.

[Percutaneous transluminal valvuloplasty in severe neonatal pulmonary stenosis]. / Percutan transluminaris ballon valvuloplastica újszülöttkori súlyos pulmonális stenosisban.

The authors made diagnosis of valvar pulmonary stenosis in a 1-day old neonate. The severe stenosis was treated by means of pulmonary balloon valvuloplasty at the age of 4 days. The initial suprasystemic pressure in the right ventricle decreased to normal after the procedure. Transluminal balloon valvuloplasty is an effective therapeutic method for severe and critical valvar pulmonary stenosis and casually may serve as an alternative of surgical treatment. This case was the first successful procedure of pulmonary balloon valvuloplasty in Hungary at neonatal age.

The use of an internal mammary artery for creating a systemic-to-pulmonary artery shunt.

We report on two patients who had systemic-to-pulmonary artery shunts created by the use of the internal mammary artery (IMA). The first patients was operated on more then 30 years ago (the case has never been published) and the second one move recently. We give a brief summary of the ten cases of the same operation published so far, and emphasize the usefulness of the IMA that stays open despite the initially poor run-off and is capable of supplying increasing amounts of blood to the growing pulmonary arteries.

Cardiac status and health-related quality of life in the long term after surgical repair of tetralogy of Fallot in infancy and childhood.

The long-term results of surgical repair of tetralogy of Fallot were assessed by means of extensive cardiologic examination of 77 nonselected patients 14.7 +/- 2.9 years after surgical repair of tetralogy of Fallot in infancy and childhood. Because of the frequent use of a transannular patch (56%) for the relief of right ventricular outflow tract obstruction, the prevalence of elevated right ventricular systolic pressure was low (8%), but the prevalence of substantial right ventricular dilation with severe pulmonary regurgitation was high (58%). The exercise capacity of patients with a substantially dilated right ventricle proved to be significantly decreased (83% +/- 19% of predicted) when compared with that of patients with a near normal sized right ventricle (96% +/- 13%). Eight out of 10 patients who had needed treatment for symptomatic arrhythmia had supraventricular arrhythmia, which makes supraventricular arrhythmia--in numbers--a more important sequela in the long-term survivors than ventricular arrhythmia. Older age at the time of the operation and longer duration of follow-up were not associated with an increase in prevalence or clinical significance of sequelae.

Long-term follow-up after surgical closure of ventricular septal defect in infancy and childhood.

OBJECTIVES: The purpose of this study was to assess the health-related quality of life of patients who underwent surgical closure of a ventricular septal defect at a young age between 1968 and 1980. BACKGROUND: Since the beginning of open heart surgery for congenital cardiac malformations, the surgical techniques have continually improved. As a result, even infants have become eligible for surgical repair. Long-term follow-up data are not available on the health-related quality of life of nonselected patients after surgical repair at a young age. We therefore conducted a follow-up study of 176 infants and children consecutively operated on in one institution between 1968 and 1980. METHODS: Patients who were alive and could be traced through the offices of local registrars received an invitation to participate in the follow-up study, consisting of an interview, physical examination, echocardiography, exercise testing and standard 12-lead and 24-h electrocardiography. RESULTS: One hundred nine patients (78% of those eligible for follow-up) participated. The mean interval after operation (+/- SD) was 14.5 +/- 2.6 years. Eighty-four percent of the patients assessed their health as good or very good, and 89% had been free of any medical or surgical intervention since the operation. At physical examination all patients were in good health. Their mean exercise capacity was 100 +/- 17% (range 56% to 141%) of predicted values; 84% of the patients had a normal exercise capacity. Echocardiography demonstrated a small residual ventricular septal defect in seven patients (6%). There were no signs of pulmonary hypertension. No patient had symptomatic arrhythmias. CONCLUSIONS: Long-term results of surgical closure of ventricular septal defect in infancy and childhood are good. Pulmonary hypertension is absent. Personal health assessment is comparable to that of the normal population, as is exercise capacity, even though many patients have anatomic, hemodynamic or electrophysiologic sequelae.

Clinical significance of maternal anti-Ro/SS-A antibodies in children with isolated heart block.

OBJECTIVES: We studied 30 consecutive children with isolated heart block to assess the clinical impact of the presence of maternal anti-Ro/SS-A antibodies for isolated heart block. BACKGROUND: Isolated heart block in children, often associated with maternal autoimmune disease leading to anti-Ro/SS-A auto-antibody production, is an infrequent but potentially lethal disorder. METHODS: Thirty children with isolated heart block were studied with respect to medical history and electrocardiographic (ECG) analysis. The presence of anti-Ro/SS-A antibodies was determined in the maternal serum. We also examined the ECGs of all brothers and sisters of the patients for conduction abnormalities. RESULTS: Twenty-one of the 30 children had an anti-Ro/SS-A-positive mother (group A); the other 9 children had an anti-Ro/SS-A-negative mother (group B). Comparison of the clinical data from both mothers and children revealed that these two groups differed significantly with respect to the following: Prenatal diagnosis and obstetric complications occurred more often in group A, whereas progression to complete block, QRS width > 0.08 s, premature ventricular contractions and ventricular standstills > 4.5 s occurred more often in group B. In addition, mothers of children in group A reported more spontaneous abortions. All siblings of children in groups A and B had normal ECGs, excluding a subclinical form of heart block. CONCLUSIONS: Two types of heart block can be recognized: Congenital heart block is associated with maternal anti-Ro/SS-A antibodies and numerous obstetric and neonatal complications. It is diagnosed prenatally or at birth and is usually complete at onset and probably has a substantial recurrence risk. Heart block that is acquired later in life is not associated with maternal autoimmunity and has no risk for recurrence. It often presents as a partial block but progresses to complete block in time.

Long-term follow-up (9 to 20 years) after surgical closure of atrial septal defect at a young age.

To assess the long-term cardiac status after surgical closure of an atrial septal defect (ASD) at a young age, 104 of 135 children who consecutively underwent surgery (aged 0 to 14 years) at 1 institution between 1968 and 1980 participated in a follow-up study and underwent a complete cardiologic examination. Mean follow-up was 14.5 +/- 2.8 years. Most patients (87%) believed their health to be good or very good. At physical examination, all patients were in good health. Ninety-three patients (89%) were in sinus rhythm. Echocardiography showed that right ventricular dilatation was present in 27 patients (26%), 2 of whom had a residual ASD. Bicycle ergometry revealed that 88 patients (88%) had a normal exercise capacity. Both supraventricular and ventricular arrhythmias were observed in 67% of patients by 24-hour ambulatory electrocardiography, but only 3 (3%) had received antiarrhythmic medication, and 4 (4%) had needed a pacemaker. In the group of patients with right ventricular dilatation, the exercise capacity and prevalence of arrhythmias did not differ significantly from those in the group with a normal sized right ventricule. The outcome in patients with a secundum-type ASD was not different from that of those with a sinus venosus-type ASD. The finding of anatomic, functional or electrophysiologic abnormalities was not associated with a longer duration of follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

An acardiac infant: the extreme form of the twin-twin transfusion syndrome.

A description of the pathologic and clinical findings in a twin with twin-to-twin transfusion syndrome is given. One of them died immediately after birth, with the autopsy revealing the histologic absence of the myocardium. The surviving infant presented with short term heart failure and with persistent left ventricular hypertrophy over 6 months. The circulatory changes in the twin-to-twin transfusion syndrome can result in complete myocardial involution in one and an unexpectedly long compensatory left ventricular hypertrophy in the other infant. Caution is advised in order to avoid misdiagnosing hypertrophic cardiomyopathy. In such a case a longer follow-up period is recommended.

Maternal autoantibodies and congenital heart block: no evidence for the existence of a unique heart block-associated anti-Ro/SS-A autoantibody profile.

One of the rare examples of the transfer of autoimmune disease from mother to (unborn) child is the neonatal lupus syndrome. This syndrome comprises the development of fetal heart disease (congenital heart block) or neonatal skin rash and is specifically associated with maternal anti-Ro/SS-A autoantibodies. Previous studies have suggested that especially maternal autoantibody reactivity against the 52 kDa protein of the Ro/SS-A antigen and/or against the La/SS-B antigen is responsible for the development of congenital heart block (CHB). To determine the CHB-associated antibody response in more detail, we analysed the presence of autoantibodies in sera from mothers of children with isolated heart block. All 14 mothers of children with congenital heart block were positive for anti-Ro/SS-A antibodies. Remarkably, their antibody profile, including recognition of different Ro/SS-A proteins and autoantibody levels against these proteins, did not differ from anti-Ro/SS-A positive mothers of healthy children. In contrast, all 8 anti-Ro/SS-A negative mothers had children with acquired heart block. We conclude from our data that maternal anti-Ro/SS-A antibodies are essential for CHB but that fine analysis of this autoantibody response does not predict the occurrence of CHB.

Cardiac tamponade in a preterm neonate secondary to a chest tube.

In a preterm infant, chest tubes were inserted for treatment of bilateral pneumothoraces. Hemorrhagic pericardial effusion with cardiac tamponade developed, probably resulting from traumatic injury by the left chest tube. The infant survived due to timely diagnostic and therapeutic intervention. No recurrence of pericardial effusion was seen and follow-up showed normal psychomotor development.

[Application of non-glycoside inotropic agents in newborn infants]. / Het gebruik van niet glycoside inotropica bij de pasgeborene.

Non glycoside inotropic agents are regularly used in the treatment of critically ill neonates. There are reasons to believe that the neonate reacts differently to these inotropic agents because of differences in physiology and maturation. In this paper the oxygen consumption and oxygen transport, heart failure in the newborn, effects of non glycoside inotropic agents in general and the doses of the commonly used inotropic agents (dopamine, dobutamine and isoproterenol) will be discussed. Finally a review of the literature and a therapeutic advise on the use of inotropic agents in the neonate is presented.