Right ventricular expression of extracellular matrix proteins, matrix-metalloproteinases, and their inhibitors over a period of 3 years after heart transplantation.

Fibrillar collagens I and III, nonfibrillar collagen IV, and the glycoproteins fibronectin and laminin, are elements of the myocardial extracellular matrix (ECM). Alterations in the normal concentrations and ratios of these elements may reflect remodeling in response to physiologic stress. In the case of patients' post-heart transplantation (HTx), specific patterns of alteration may herald myocardial dysfunction. Right ventricular biopsies were taken from the same 28 HTx patients before implantation and 1 week, 2 weeks, and 1, 2, and 3 years after HTx. The above-noted five ECM proteins, six matrix metalloproteinases (MMPs) and two of their tissue inhibitors (TIMPs) were detected by immunohistochemistry and scored as cells per square millimeter or semiquantitatively. The total connective tissue fibers were detected by connective tissue stain and morphometry. Variations in these ECM components were followed in the same patient cohort over 3 years. In summary, during the first 2 weeks after HTx, a predominant increase in connective tissue occurred. Increases in MMP-8 and MMP-9 were found. By 3 years after transplantation, there was a decrease of connective tissue fibers and a significant reduction of all ECM components and an increase in MMPs and TIMPs. These findings may reflect a pattern of remodeling specific to the transplanted heart.

Identification and characterisation of a novel KCNQ1 mutation in a family with Romano-Ward syndrome.

Romano-Ward syndrome (RWS), the autosomal dominant form of the congenital long QT syndrome, is characterised by prolongation of the cardiac repolarisation process associated with ventricular tachyarrhythmias of the torsades de pointes type. Genetic studies have identified mutations in six ion channel genes, KCNQ1, KCNH2, SCN5A, KCNE1 and KCNE2 and the accessory protein Ankyrin-B gene, to be responsible for this disorder. Single-strand conformation polymorphism (SSCP) analysis and subsequent DNA sequence analysis have identified a KCNQ1 mutation in a family that were clinically conspicuous due to several syncopes and prolonged QTc intervals in the ECG. The mutant subunit was expressed and functionally characterised in the Xenopus oocyte expression system. A novel heterozygous missense mutation with a C to T transition at the first position of codon 343 (CCA) of the KCNQ1 gene was identified in three concerned family members (QTc intervals: 500, 510 and 530 ms, respectively). As a result, proline 343 localised within the highly conserved transmembrane segment S6 of the KCNQ1 channel is replaced by a serine. Co-expression of mutant (KCNQ1-P343S) and wild-type (KCNQ1) cRNA in Xenopus oocytes produced potassium currents reduced by approximately 92%, while IKs reconstitution experiments with a combination of KCNQ1 mutant, wild-type and KCNE1 subunits yielded currents reduced by approximately 60%. A novel mutation (P343S) identified in the KCNQ1 subunit gene of three members of a RWS family showed a dominant-negative effect on native IKs currents leading to prolongation of the heart repolarisation and possibly increases the risk of malign arrhythmias with sudden cardiac death.

Perinatal outcome of fetal complete atrioventricular block: a multicenter experience.

The clinical course and outcome of 55 fetuses with complete atrioventricular (AV) block detected prenatally were studied to identify factors that affect the natural history of this lesion. In 29 fetuses (53%) complete AV block was associated with complex structural heart defects, usually left atrial isomerism (n = 17) or discordant AV connection (n = 7). The other 26 fetuses had normal cardiac anatomy; in 19 cases the mother had connective tissue disease or tested positive for antinuclear antibodies. Six fetuses showed progression from sinus rhythm or second degree block to complete AV block. Of the 55 pregnancies, 5 were terminated and 24 fetuses or neonates died; at the end of the neonatal period 26 fetuses were still alive. Fetal or neonatal death correlated significantly with the presence of structural heart defects (4 of 29 surviving, p less than 0.001), hydrops (0 of 22 surviving, p less than 0.001), an atrial rate less than or equal to 120 beats/min (1 of 12 surviving, p less than 0.005) or a ventricular rate less than or equal to 55 beats/min (3 of 21 surviving, p less than 0.001). Mean atrial and ventricular rates were higher in surviving than in nonsurviving fetuses (142 +/- 8 vs. 127 +/- 21 beats/min, p less than 0.002; 64 +/- 8 vs. 52 +/- 8 beats/min, p less than 0.001, respectively). A slow atrial rate, however, was frequently associated with left atrial isomerism.(ABSTRACT TRUNCATED AT 250 WORDS)

Electrophysiologic effects and efficacy of intravenous propafenone in terminating atrioventricular reentrant tachycardia in children.

In pediatric patients with atrioventricular reentrant tachycardia, intravenous propafenone exhibits its electrophysiologic effects in a dose-dependent manner by slowing or blocking retrograde conduction at the accessory connection. The high drug efficacy (81%) in terminating tachycardia is not dependent on patient age or retrograde conduction properties of the accessory connection.

The index of pulmonary vascular disease in children with congenital heart disease: relationship to clinical and haemodynamic findings.

OBJECTIVE: We asked whether a scoring system [index of pulmonary vascular disease (IPVD)] that quantifies the individual pulmonary vascular pathology would relate to postoperative survival in patients with congenital heart disease and pulmonary hypertension (PH). METHODS: Lung biopsy specimens from 28 patients at a median age of 6 months (1 month to 21 years) were analysed qualitatively and morphometrically. The IPVD and other morphometric parameters were related to haemodynamic findings and survival. RESULTS: Mean pulmonary artery pressure (PAP) was 44 mmHg (15-72 mmHg), and the resistance to pulmonary perfusion was 5 U x m(2) (0.9-14 U x m(2)). There were three early (in-hospital) and three late deaths during the follow-up period of 2.5 years (6 months to 7 years). Incipient plexiform lesions were observed in one infant with trisomy 21 and complete atrioventricular septal defect (cAVSD). An IPVD score above the upper critical limit (>2.2) was not observed during the first year of life. On discriminant analysis, morphometric parameters could not predict mortality ( P=0.08). CONCLUSIONS: The IPVD is not helpful to predict surgical mortality during the first year of life. Patients with trisomy 21 and cAVSD may show advanced pulmonary vascular disease in infancy.

Metabolites of the L-arginine-NO pathway in patients with left-to-right shunt.

OBJECTIVES: The endogenous production of metabolites of the L-arginine-NO pathway has been found to be altered in patients with left-to-right shunt and pulmonary hypertension. The objective of this study was to analyze the influence of age and of the magnitude of the left-to-right shunt on plasma levels of L-arginine, cyclic guanosine monophosphate (cGMP), nitrite and nitrate in children and young adults presenting with left-to-right shunt. METHODS: Twenty-nine patients with ventricular septal defect (n=18), atrial septal defect (n=6) and atrioventricular canal (n=5) were assigned to group I when the ratio of pulmonary to systemic blood flow (Qp/Qs) was less than 1.5 (n=10) and to group II when Qp/Qs > or = 1.5 (n=19). At cardiac catheterization blood samples were taken from the pulmonary vein or left ventricle. In 33 controls peripheral venous blood was obtained. cGMP levels were determined by radioimmunoassay, L-arginine, nitrite and nitrate by high performance liquid chromatography (HPLC). RESULTS: L-arginine plasma levels were lower in group II than in controls (51.7 [23.3-82.2] versus 60.5 [32.4-85.9] pmol/l; p < 0.05 by KRUSKAL-WALLIS). Age did not influence the L-arginine plasma levels (p = 0.30). cGMP levels depended on age (p<0.01) and mean pulmonary artery pressure (p <0.01) but not on high pulmonary blood flow (p=0.85; ANOVA). Plasma nitrite and nitrate were not different in both groups and when compared with controls (nitrite: 26.0 [23.5-31.0] micromol/l; nitrate: 26.8 [24.0-32.0] micromol/l). CONCLUSIONS: Age and pulmonary artery pressure exert important effects on plasma cGMP. Measurement of nitrite and nitrate in plasma alone may not reflect the endogenous NO production. Future studies should evaluate the role of plasma levels of L-arginine in patients with high pulmonary blood flow undergoing repair of their defect.

Intracardiac yolk sac tumor in an infant girl.

We report about a two and a half year old girl with a primary yolk sac tumor (YST) of the heart, located in the interventricular septum. The girl was admitted to the hospital with a systolic cardiac murmur. Echocardiography revealed a smoothly surfaced tumor which based on the interventricular septum below the aortic valve and filled out more than half of the left ventricular cavity. Diagnosis of a yolk sac tumor was established on a frozen section of a biopsy taken at surgery and confirmed by the examination of the resected material. Excessively elevated serum alpha-fetoprotein (AFP) levels contributed to this diagnosis. Follow up examinations of the patient including computed tomography (CT) of head, thorax and abdomen, did not indicate an extracardiac tumor manifestation. We found no previous report of a primary intracardial yolk sac tumor in the literature.

[Inborn long QT-syndrome in childhood: clinical presentation and treatment]. / Angeborene Long-QT-Syndrome im Kindesalter: Klinische Präsentation und Behandlung.

During the last decade, the understanding of the long QT-syndrome (LQTS) as an inherited arrhythmogenic disease has dramatically increased. The LQTS has been recognized to be a heterogeneous family of ion-channel disorders caused by numerous mutations in at least six distinct gene loci, thus, explaining the prolongation of the myocardial repolarization. Consecutive studies of the LQTS advanced our knowledge of pathophysiology, clinical course and possible therapeutic impact. As a genetically determined disorder the clinical manifestation of the LQTS naturally starts in childhood. In 34% of the children, syncope or cardiac arrest was found to occur before the age of 15 years. In addition, 54% of all LQTS patients who died from sudden cardiac death were less than 20years old. Most cases in children are identified by the detection of a prolonged QT interval while evaluating unexplained cases of syncope or by family investigations of an index patient. In children more than in adults, however, normal values of QT interval duration are dependent from age, gender, heart rate and circadian variations. Therefore, the moreover applied correction formulas for the QT interval to heart rate ratio have to be used with caution in the pediatric setting. A useful and reliable tool for the analysis of QT duration, QT patterns, and its circadian variation is multilead digital Holter recordings. The determination of the diagnosis is based on clinical findings according to the criteria of the "International LQTS Registry". Genetic investigations, however, are actually diagnostic in about 50% of the patients, but are not effective as a clinical screening tool. The genetically determined LQTS types (LQT1-LQT6) differ significantly in terms of reaction to triggering stimuli that may induce life-threatening arrhythmias and their response to treatment. In LQT1, physical stress will more likely induce Torsades de Pointes than in the LQT3, which is more sensitive to emotional stress. The typical LQTS treatment is life-long medication with beta blocking agents; however, in LQT3 patients with mutations in the cardiac sodium channel gene, treatment with mexiletine may have advantages. In order to prevent bradycardia or by short-long sequences inducible torsades, the use of implantable pacemakers is recommended. Stellectomy to minimize cardiac adrenergic susceptibility has proven to be less effective in children. Recently, the rapid technical improvement of implantable defibrillators led to a more frequent use of these devices in children. In order to sufficiently manage pediatric LQTS patients in the future and to reduce the risk of sudden cardiac death due to inherited arrhythmias, a national and international multicenter approach is necessary.

[Pulmonary microembolism: pulmonary hemodynamics following trauma and hemorrhage (author's transl]. / Pulmonale Mikroembolie: Pulmonale Hämodynamik nach Trauma und Blutentzug

Standardized bone trauma by means of open osteotomy is performed on both hind legs of 16 out of 24 anaesthetised mongrels. During the following 6 hours the most important parameters of blood coagulation and the serum lipids are estimated at regular intervals. Subsequently in a number of the animals 50% of the total blood volume is withdrawn continuously over a period of one hour. All animals without the preceding trauma survive the hemorrhage. The animals in the trauma group die on the average 42 minutes after the beginning of the hemorrhage. The trauma causes an acute decrease in total platelet count (GTZ) to 40% of the pretraumatic value. During an initial phase of hypercoagulability there is a formation of reversible microaggregations containing platelets and fibrin, caused by an increased turnover of coagulation factors. Secondary fibrinolysis develops in the traumatised animals. A further increase in coagulation is caused by the following hemorrhage. In combination with decreased and inhibited fibrinolysis, a disseminated intravascular coagulation state is found and results in irreversible pulmonary microthrombosis. Massive pulmonary fat deposits cannot be found histologically in spite of an increase in serum triglycerid levels to 35% above the pretraumatic values. In dogs great amounts of fat are filtered by the glomeruli and are demonstrable in the tubular epithelium. Histological examinations show a marked disseminated pulmonary microthrombosis of platelets, fibrin and fat in those animals with trauma and hemorrhage. Only the accompanying hypovolemia produces the characteristic histologic changes of the so-called Pulmonary Microembolism Syndrome.

Oxygen-mediated pulmonary vasodilation and plasma levels of endothelin-1, atrial natriuretic peptide and cyclic GMP in patients with left-to-right shunt and pulmonary hypertension.

This study was performed to evaluate the role of endogenous endothelin-1 (ET-1), atrial natriuretic peptide (ANP) and cyclic guanosine monophosphate (cGMP) in patients with left-to-right shunt and pulmonary hypertension. Further objectives were to study a possible feedback mechanism between ANP and ET-1 and to examine the influence of ANP on cGMP plasma levels. Finally, the role of these hormones in oxygen-mediated pulmonary vasodilation was examined. Plasma concentrations of ET-1, ANP and cGMP were studied in 39 patients with congenital heart disease and left-to-right shunt. Blood samples were taken from the pulmonary artery and pulmonary vein at cardiac catheterization at baseline and after breathing oxygen for 20 min. Patients were grouped according to the presence or absence of pulmonary hypertension (defined as mean Pp/Ps > or = 0.5). Patients with pulmonary hypertension (n = 18) were found to have significantly higher plasma ANP (665 [59-1358] versus 267 [47-832] pg/ml) and cGMP (21.5 [3.6-82.2] versus 7.8 [0-14.6] nM/L) levels than patients without pulmonary hypertension (n = 21). Pulmonary venous ET-1 plasma concentrations were above normal limits in one patient only. ANP plasma levels were not related to ET-1 and cGMP concentrations. There was no transpulmonary gradient for any of the factors. Pulmonary vasodilation in response to oxygen was found in 7 of 18 patients with PH, but was not associated with significant changes in ET-1, ANP or cGMP plasma concentrations. Patients with congenital heart disease and PH show an increase both in vasoconstrictive and vasodilating factors. The mechanism of oxygen-mediated vasodilation in these patients remains to be elucidated.

[Emergency from anesthesia in small children. From laryngospasm to prolonged apnea]. / Narkoseausleitung beim Kleinkind. Vom Laryngospasmus zur prolongierten Apnoe.

Postoperative laryngospasm during emergence from anaesthesia represents a potentially life-threatening complication. Even if this is successfully overcome using drug therapy, new, serious problems may develop. We report the case of a 3 1/2 -year-old boy of African descent weighing 15 kg who developed a laryngospasm during emergence from anaesthesia. Because the airway obstruction could not be controlled by deepening the anaesthesia again and administering anti-obstructive drugs, the boy was given 15 mg succinylcholine. Thereafter prolonged apnea developed such that the patient had to be admitted to the pediatric intensive care unit. The child was extubated 6 h later and the further course was normal so that he could be released from the hospital the following day. Further diagnostic study revealed a dibucaine-sensitive, fluoride-resistant pseudocholinesterase in the plasma, which is a rare form of atypical pseudocholinesterase, explaining the prolonged arousal phase after the administration of succinylcholine. Three significant aspects of this case are discussed: 1. risk factors and treatment of perioperative airway obstruction 2. factors and treatment of prolonged apnea, and 3. delayed arousal reactions and their management in an outpatient setting.

Prediction of normal QT intervals in children.

Measuring QT intervals in individual children is of great importance, particularly in view of increasing evidence linking long QT syndrome to subsequent risk for sudden death. Three hundred seventy-three healthy subjects, 185 women and 188 men, aged 5.2 to 16.5 years, were investigated with a 12-lead-standard electrocardiogram (ECG). Values for predicted QTp50 and QTp95 (percentiles) were calculated by using the cycle length (RR interval [RRI]) and the measured QT interval. We used multiple regression analysis to test the influence of possible important variables and the resulting data were used to generate tables. Additionally, predicted QTp values were compared to QTc values after Bazett's correction. RRI, body height, age, and sex turned out to influence the QTp values most. For clinical use, data are presented in tabular form by RRI and age for both genders. The tables are of great clinical value in predicting the upper limits of normal QTp95 for individual children. Bazett's correction tends to underestimate the values found in our data when heart rate increases.

Cardiac dysrhythmias in pediatric patients before and 1 year after transcatheter closure of atrial septal defects using the amplatzer septal occluder.

To prospectively assess the incidence of cardiac dysrhythmias before and after closure of atrial septal defects (ASDs) using the Amplatzer septal occluder (ASO), 24-hour Holter electrocardiograms (ECGs) were performed before and 1 year after the procedure in 23 pediatric patients (9 male and 14 female). Patients' ages ranged from 2 to 15 years (mean, 7.1 years). All had an ASD of the secundum type that was completely closed (n = 22) or had a small residual shunt (n = 1). No preexisting dysrhythmia was present in 22 patients; atrioventricular nodal reentrant tachycardia had been diagnosed in 1 patient. During the observation period, no clinical dysrhythmia occurred. Analysis of the Holter ECGs before the intervention showed regular sinus rhythm in 20 patients and sinus rhythm with intermittent atrial rhythm in 3 patients. Atrial premature complexes (APCs) were detected in 1 patient, and a ventricular couplet was present in 1 patient. The Holter ECG 1 year after the intervention showed sinus rhythm in 18 patients and sinus rhythm with intermittent atrial rhythm in 5 patients. APCs were still observed in 1 patient and seen for the first time in 1 patient; 1 patient and rare ventricular premature complexes. In conclusion, cardiac dysrhythmias on Holter ECG in pediatric patients before and 1 year after transcatheter ASD closure with the ASO device are rare and benign. Regular Holter monitoring seems to be useful in detecting late dysrhythmias.

Pulmonary vasoreactivity and vasoactive mediators in children with pulmonary hypertension.

We assessed the effect of oxygen, nitric oxide (NO) and prostanoids (prostacyclin and iloprost) on pulmonary hemodynamics and plasma levels of vasoactive mediators in children with pulmonary hypertension (PH). It is not known whether the hemodynamic response during acute vasodilator testing correlates with changes in plasma levels of endothelin-1 (ET-1), cyclic guanosine monophosphate (cGMP) and cyclic adenosine monophosphate (cAMP). In this retrospective analysis 14 children at a median age of 4 years and 3 months [1.8 months-13 years] with a median pulmonary resistance to perfusion of 10.1 [2.1-37.7]. Wood-Units x m2 were studied. Diagnoses included PH due to congenital heart disease (AVSD n = 5; VSD n = 2; PDA n = 1) or unknown causes (n = 6). The ratios of pulmonary/systemic pressure (Pp/Ps) and of pulmonary/systemic resistance (Rp/Rs) were recorded a) at baseline, b) during oxygen (FiO2 = 1.0) and c) while on NO (80 ppm max., at FiO2 = 0.23). In 13 out of 14 children prostanoids were given additionally: 7 received prostacyclin (i.v.) and 6 were given iloprost which was nebulized. ET-1, cGMP and cAMP were measured in blood samples taken from the pulmonary vein or left ventricle at baseline, during increased FiO2, during NO inhalation and while on prostanoids. Pulmonary vasodilation in response to oxygen was found in 2/14 patients. 4/14 patients responded to NO and 2/7 to prostacyclin i.v. Increased FiO2 was not associated with changes in plasma concentrations of ET-1, cGMP or cAMP. NO inhalation was followed by an increase in cGMP levels from 10.9 [5.5-55.4] nM/L to 21.3 [6.4-76.3] nM/L independent from the individual hemodynamic response. Oxygen and NO identify most children with reactive pulmonary vasculature. cGMP plasma levels do not correlate with individual hemodynamic responses to NO.

Changes of coagulation and fat-metabolism following pulmonary microembolism after trauma and hemorrhage.

In 16 of 24 anesthetized mongrel dogs a standardized bone trauma was performed to both hind legs by open osteootmy. During the following 6 hours the most important hemodynamic parameters, total platelet count (GTZ) and its separate fractions (NKF, KF) were continously monitored. Five hours after the trauma 50% of the total blood volume (taken to be 80 ml/kg body weight) was withdrawn from two of the four groups of animals. The trauma caused no direct effect on the pulmonary hemodynamics. In the following hours there was a slight decrease of cardiac output (HZV) in the traumatised animals. Besides a moderate increase in mean pulmonary pressure (MPP), pulmonary vascular resistance (PVR) increased by 72% of the pretraumatic values. The application of intravascular catheters diminished the total platelet count markedly in the injured, as in the uninjured animals. The increase in PVR was caused less by a mechanical obstruction of the pulmonary capillary bed than by vasoactive substances released by the platelets. The following hemorrhage led to a more marked hemodynamic reaction in the injured animals. All these animals died before the end of the calculated hemorrhage. All animals without trauma survived. Irreversible aggregation of platelets developed in the pulmonary capillary bed caused by the marked pulmonary hypocirculation. Histological examination showed a marked disseminated pulmonary microthrombosis in all animals with trauma and hemorrhage. The initial phase of experimental pulmonary microembolism caused by trauma was characterized by pulmonary hypocirculation and an activated coagulation with simultaneously diminished total platelet count.

Assessment of adriamycin cardiotoxicity in children by systolic time intervals.

In a prospective study, 44 children receiving Adriamycin (ADR) for various neoplastic diseases underwent serial estimations of the systolic time intervals (STI) for the noninvasive assessment of left ventricular myocardial performance. Five of the 44 children developed clinical signs of ADR-related congestive heart failure at a cumulative dose of less than 550 mg/m2 body surface area. Clinical symptoms, changes in the electrocardiogram and in the chest X-ray were preceded in every case by changes of the STI, mainly a prolongation of the left ventricular pre-ejection period (PEP), or a decrease of the ejection time (ET)/pre-ejection period (PEP) ratio (ET/PEP). A continous increase of the PEP and a decrease of the ET/PEP-ratio also gave an indication of myocardial dysfunction during ADR treatment in the other children without clinical signs of congestive heart failure. This subclinical cardiotoxic effect of ADR below the critical cumulative dose of 550 mg/m2 was observed in children with pre-existent myocardial damage, with preceding thoracic irradiation, or during concurrent chemotherapy, of which cyclophosphamide seemed to be most important. Thus, the estimation of the STI proved helpful and reliable in the early detection of incipient heart failure and in the selection of high risk patients in children receiving ADR treatment.

Long-term evaluation of cardiac function utilizing systolic time intervals in children with chronic renal failure.

Cardiac function was assessed in 11 children with end-stage chronic renal failure (CRF) by a prospective longitudinal study. Left ventricular performance was determined noninvasively by mechanocardiographic determination of systolic time intervals during three consecutive stages of the disease: on conservative treatment (CT), on regular hemodialysis (HD) and after successful renal transplantation (TP). The mean ratio of the pre-ejection period to left ventricular ejection time (PEP/LVET) was slightly increased on CT (0.33) compared to normal (0.29), and markedly increased 6 and 12 months after start of HD (0.38 and 0.40, respectively), indicating impairment of left ventricular performance which was clinically undetectable. 12 months after TP mean PEP/LVET was normal (0.31). The upper normal limit of PEP/LVET was exceeded by two children on CT, seven at 12 months after start of HD and by two children 12 months after TP, respectively. It is concluded that subclinical forms of uremic heart disease are common in children on HD and tend to resolve after TP.

Mechanocardiographic assessment of systolic time intervals in normal children.

Systolic time intervals (STI) were measured in 469 normal children, aged 5.0 to 16.9 years, using simultaneous recordings of the electrocardiogram, phonocardiogram and an external carotid pulse tracing. The data were analyzed to define the influence of sex, age, cardiac cycle length, body weight (BW), body length (BL), and body surface area (BSA) on the left ventricular STI. Regression, equations were evaluated for the calculation of normal standards for the following STI: electromechanical systole (QS2), left ventricular ejection time (LVET), pre-ejection period (PEP) systolic quotient (PEP/LVET), isovolumic contraction time (IVCT), electroacustical delay (QS1), and additionally for the so-called tension time (TT), upstroke time (UT), and the ascending index (UT/LVET) of the carotid pulse wave. Heart rate was found to influence all of the STI but the ratio UT/LVET, which revealed to be independent of any variable. Besides heart rate, BSA proved to be a second relevant independent variable for QS2 and LVET, respectively BL for PEP, PEP/LVET and QS1. These findings are in accordance to the results of comparable echocardiographic studies but disagree previous mechanocardiographic studies in smaller groups of children in whom age and not the above-mentioned parameters of the individual development seemed to influence the STI. A second set of equations was evaluated for the calculation of 95%-prediction intervals for the particular STI permitting to define abnormalities of the STI in individual children.

[Ectopia cordis. Report of a prenatally diagnosed cases and a short review of the literature]. / Ectopia cordis. Bericht über einen pränatal diagnostizierten Fall und eine kurze Ubersicht über die Literatur.

Ectopia cordis is defined as a congenital malposition of the heart partially or completely outside the thorax. Severe structural malformations of the heart are very frequently associated and are responsible for the extremely poor prognosis. Only 16 out of 219 cases reported in the literature up to now have survived. We report on a case of Ectopia cordis thoraco-abdominalis associated with an univentricular heart and a hypoplastic pulmonary vascular bed detected prenatally in the 32nd week of gestation. The male newborn died 23 h after a spontaneous and uneventful delivery at term. Considering the poor prognosis no surgical attempts had been undertaken to correct the malformation.