[Heterotaxia syndrome without congenital cardiac defects in dilated cardiomyopathy]. / Heterotaxie-Syndrom ohne kongenitalen Herzfehler bei dilatativer Kardiomyopathie.

UNLABELLED: HISTORY AND OUTPATIENT INVESTIGATION: A 61-year-old woman presented with a six-month history of chest pain and shortness of breath on normal activity. The past medical history included hypertension treated with ACE inhibitors. While still an outpatient a diagnosis was made of cardiomyopathy with left atrial and ventricular dilatation, systolic and diastolic heart failure, moderate mitral valve regurgitation and newly documented atrial fibrillation. INVESTIGATION: Right heart catheterization was carried out via the right femoral vein. The inferior vena cava was found to be on the left, none on the right. Venous inflow was via a dilated hemiazygos vein, a persistent left superior vena cava and a markedly dilated coronary sinus into the right atrium. The hepatic veins were also directly connected to the right atrium, as was shown by retrograde perfusion during a venogram. Coronary heart disease was excluded by angiography, but a right heart catheterization was not possible because of the atypical venous connections. Ultrasound examination revealed abdominal situs inversus and polysplenia. Magnetic resonance imaging of the thorax demonstrated bilateral bilobar lungs and bilateral hyparterial bronchi. DIAGNOSIS, TREATMENT AND COURSE: Heterotaxia with anomalous systemic veins and visceral defects was revealed during a diagnostic work-up, which was indicated by the finding of a dilated cardiomyopathy with chronic atrial fibrillation, moderate mitral valve regurgitation and arterial hypertension. Arterial hypertension and heart failure were successfully treated by medication. Attempts at rhythm control were unsuccessful. Safety measures were established to prevent thromboembolic complications and endocarditis. CONCLUSION: Congenital anomalies of the systemic veins in adults are often discovered incidentally, because they are usually asymptomatic. They may cause diagnostic and therapeutic difficulties in cardiology, phlebology and surgery. These anomalies may increase the risk of thrombotic and thrombembolic events. Together with polysplenia and situs inversus they are phenotypical components of heterotaxia. They may have various clinical consequences and may occur spontaneously or may be familial.

[Modified ECG-guidance for optimal central venous catheter tip positioning. A transesophageal echocardiography controlled study]. / Optimierte Positionierung zentraler Venenkatheter durch eine modifizierte Anwendung der intravasalen Elektrokardiographie. Validierung mithilfe der transösophagealen Echokardiographie.

BACKGROUND: Intraatrial electrocardiography (ECG) is a well-established method for central-venous catheter (CVC) placement and an intraatrial position is assumed, when a significantly increased P-wave is registered. However, an increase in P-wave amplitude also occurs in other positions. Therefore we evaluated CVC tip positioning by means of transesophageal echocardiography (TEE) at a maximum P-wave amplitude. PATIENTS AND METHODS: In this prospective randomized study the right or left internal jugular vein was cannulated with 100 patients in each group and catheter tip positioning was guided by means of ECG. The catheter was fixed at the position of maximum P-wave amplitude and the insertion depth was registered. The relationship of the CVC tip position to the superior edge of the crista terminalis was demonstrated with the help of TEE. RESULTS: In all patients the catheter tip was found +/- 0.5 cm from the superior edge of the crista terminalis at the transition from the superior vena cava to the right atrium. On x-ray control, all catheters ran along the length of the vessel wall of the superior vena cava. CONCLUSIONS: A maximum P-wave is derived even at the entrance to the right atrium. This explains why ECG-guided CVC placement -- based on the largest P-wave amplitude -- consistently resulted in correct positioning of the CVC tip at the transition from the superior vena cava to the right atrium.

The influence of the Univent endotracheal tube on internal jugular vein cannulation.

This prospective clinical investigation assessed the effect of placement of a Univent tube on the anatomy of the internal jugular veins and the success of cannulation of the left internal jugular vein. After obtaining informed consent, 48 adult patients were enrolled. Of these, 42 patients were eligible and were divided into two groups: Univent tube (group U, n=21) and wire enforced endotracheal tube (group C, n=21). The Univent tube group were having a left thoracotomy. Using horizontal ultrasound scans just above the thyroid gland, the internal jugular vein was visualized and measured before and after Univent placement. The number of needle passes necessary to cannulate the left internal jugular vein in the two groups was also compared. Univent tubes were associated with lateral displacement of the right carotid artery and internal jugular vein on the convex side of the Univent tube, with compression of the right internal jugular vein by the artery, resulting in a kidney-shaped cross-section of the vein. On the left (concave side of the tube), the neck was indented, the sheath of the left carotid artery was displaced medially, and the left internal jugular vein distorted to an ellipse. There was a significant increase in the lateral diameter and a decrease in the cross-sectional area of the left internal jugular vein (t-test, P < 0.05). The first attempt at cannulation of the left internal jugular vein failed significantly more often in the Univent group (13/21 vs 5/21 in group C, Chi-square 6.22, P=0.025). Cannulation of the internal jugular vein before placement of the Univent tube, or placement with ultrasound guidance is suggested.

ECG recording of central venous catheter misplaced in inferior thyroid artery.

A 71-year-old male patient with liver metastases secondary to rectal carcinoma was scheduled for hemi-hepatectomy. Two months earlier he had undergone subtotal resection of the thyroid gland. Prior to surgery, a triple-lumen catheter and an introducer sheath were introduced into the right internal jugular vein using a landmark technique. No problems occurred during insertion of the triple-lumen catheter, but resistance was noticed during insertion of the 8.5 FG introducer sheath. After placement of the introducer sheath, intra-arterial misplacement was confirmed using a pressure transducer. The opportunity was taken to record and compare intravascular ECG by the arterial and venous catheters before removal. No difference was noticed in the P-wave patterns; both showed a marked increase. Surgical exploration of the neck, recommended by the vascular surgeon consulted, showed that the carotid artery was not injured. The introducer sheath had completely punctured the right internal jugular vein and entered the inferior thyroid artery. A thrill was felt. The management of this case is discussed, with suggestions for best practice. Intravascular ECG was unhelpful in differentiating between venous and arterial placement of the catheter.

[Uncorrected transposition of the great arteries and large ventricular septum defect perioperative management of a caesarean section]. / Unkorrigierte Transposition der grossen Arterien und grosser Ventrikelseptumdefekt. Perioperatives Management einer Sectio caesarea.

Patients with congenital cyanotic heart disease are a challenge to the anaesthetist due to the individual haemodynamic characteristics. Maintaining a balance between systemic and pulmonary-vascular resistance is crucial. Here we outline the successful perioperative management of a 24-year-old female with uncorrected transposition of the great arteries (D-TGA) and large septal defect of the ventricle (functionally single ventricle). She was transferred to our perinatologic centre in the 32nd week of pregnancy with symptoms of increasing cardial insufficiency. The peripartum management was agreed upon at an interdisciplinary conference and caesarean section was performed in the 35th week of pregnancy with epidural anaesthesia and no significant problems. Due to hypercoagulability and the risk of "paradoxical" embolism, low molecular weight heparin was given for 6 weeks post partum. The infant was underweight and was admitted to the neonatal intensive care unit, where she made a satisfactory progress.

The postoperative stability of the first metatarsal

The hallux valgus deformity is characterised by an increased first intermetatarsal angle, which can be reduced by osteotomies on the first metatarsal bone, such as the Proximal osteotomy and the Scarf shaft osteotomy. Osteotomy and subsequent screw fixations were performed on 18 matched pairs of cadaveric specimens to compare the postoperative stability of these two common types of operative correction. Biomechanical testing with plantar force was carried out and failure load was measured for each specimen. The results indicate that the threaded bones provide a high postoperative loading capacity. When maximal strain was exceeded, the specimens failed in the proximal third, irrespective of the type of osteotomy. Moreover, we found that in contrast to the living age the mineral density and the individual geometry of the bone have a marked influence on the postoperative loading capacity. Finally, static biomechanical studies demonstrated that the Scarf osteotomy is significantly more stable than the commonly used Proximal osteotomy. Regarding the early postoperative mobilization of the patients concerned, the Scarf osteotomy proved to be superior, but the Proximal osteotomy requires a more cautious rehabilitation program (AU)

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Persistent left superior vena cava and central venous catheter position: clinical impact illustrated by four cases.

Variations in the course of the blood vessels are often incidental findings during clinical examination. A persistent left superior vena cava (LSVC) is really not rare (healthy individuals, 0.3-0.5%; patients with congenital heart disease, 4%) and serious complications have been described during catheterization in adults with LSVC (shock, cardiac arrest, angina). Therefore variations of the superior vena cava should be considered, especially when central venous catheterization via the subclavian or internal jugular vein is difficult. We describe the embryogenesis and the anatomic variations of persistent LSVC. Subsequently we suggest a classification of superior vena cava according to the positioning of a central venous catheter on the chest radiograph: type I, normal anatomy; type II, only persistent left superior vena cava; type IIIa, right and left superior vena cava with connection; type IIIb, right and left superior vena cava without connection. This classification is illustrated by four clinical cases.

Unusual blood supply to the pancreas by a dorsal pancreatic artery.

A variation of the blood supply to the pancreas was observed in an 89-year-old female, in which the celiac trunk gave off four arteries: the hepatic, splenic, left gastric arteries and an additional dorsal pancreatic artery. One of the branches the dorsal pancreatic artery joined with the superior mesenteric artery to form a longitudinal anastomosis. The anterior and posterior pancreaticoduodenal arcades arose from branches of the superior pancreaticoduodenal and the dorsal pancreatic arteries. The inferior pancreaticoduodenal artery, a branch of the superior mesenteric artery, was missing. The majority of the pancreas was, therefore supplied by the dorsal pancreatic artery. The clinical implications of this finding are that the size, location and course of a dorsal pancreatic artery should be established given its central role in the blood supply to the pancreas observed in the present study.

Pulmonary atresia with hypoplastic right ventricle. A clinical embryological study.

An unusual case of pulmonary atresia with an aberrant karyotype of 46,XX,t(6;8)(p21.2;q11.2) is reported. Fetal ultrasonic examination at the 20th week of gestation revealed a hypoplastic right ventricle and an intact interventricular septum. Authors summarize their postnatal findings in fetal heart and the large adjacent vessels with special reference to the pathogenesis of this rare congenital heart defect. The observation delineates right-ventricular outflow tract obstruction associated with an abnormal pulmonary blood supply. The anatomy of the systemic pulmonary collaterals was studied and correlated with multifocal disorders in the system of the pharyngeal arch arteries in the early embryonic development.

Three-dimensional reconstruction of the anal striated musculature in a human fetus.

Conflicting opinions in the recent literature indicate that the morphological organization and function of the anorectal continence organ has, up to now, not been clearly understood. But a clear imagination of the spatial arrangements of this compound muscle system is of clinical relevance for the pediatric surgeon performing reconstructive surgery. We analyzed 18-microm sections of the pelvic region of 4 human fetuses in order to describe the individual components of this muscle complex. A series of 630 Azan-stained sections was the base for the computer-assisted 3D reconstruction of the levator ani and the external sphincter complex in a male human fetus (14th week p.c.). In this context, special attention was paid to the intermediate muscle layer of the puborectalis which develops ventrally from the funnel-shaped levator ani and joins the tripartite ring system of the sphincter muscle dorsally. Our findings lead to a clear imagination of the spatial arrangement of this intermediate layer and characterize the anorectal muscle complex as an integrated ensemble in which the puborectalis holds a key position.

[The extralobar sequestration of the fetal lung]. / Die extralobare Sequestration einer fetalen Lunge.

The extralobar sequestration is a rare pulmonary malformation. An accurate antenatal evaluation is required for a timely therapy and subsequently a good outcome. Here an unusual case of extralobar pulmonary sequestration in a male human fetus is reported. Antenatal ultrasound at 28th week of gestation has revealed a fetal hydrothorax in coexistence with pulmonary hypoplasia and an isolated pulmonary structure. Authors summarise their postnatal findings with special reference to the pathogenesis of an accessory lung. The aim of this report is to define the association of clinical, gross, and histological features of this rare congential malformation in order to improve the antenatal diagnosis. This case indicates that an extralobar pulmonary sequester is not connected to the tracheobronchial tree, and that the arterial as well as the venous blood supply is realised by aberrant systemic vessels. Moreover, histologically revealed dilatations of the normally differentiated terminal airways within the sequester suggest that hyperechogenity can not be a reliable diagnostic criterion. For the accurate assessment of a pulmonary sequestration a detailed antenatal evaluation of both, the arterial and the venous blood supply is essential.

Anatomic bases of the forearm compartment syndrome.

One of the most common sites for the compartment syndrome (CS) is the forearm. Its compartments have been studied by injection of colored gelatin into the particular anatomical spaces. The three "pressure-measuring-points" recommended in the clinical literature to measure intracompartmental tissue pressure in equivocal diagnostic cases were used for the dye injections on the forearms of five preserved cadavers of adults. However, instead of the compartments especially affected in CS two adjacent spaces were revealed. In order to elucidate the clinical relevant spaces two additional approaches for the injection had been used. Cross-sections at 15 mm intervals of the injected forearms had been performed. Some of them are presented and schematically summarized in this article. Recent studies have suggested that there are different guidelines for description of the anatomically isolated spaces. However, especially one of these spaces seems to be responsible for the CS on the forearm. The remarkable features of this so called "deep flexor compartment" are its very restrictive envelopes, its rare fascial contacts, its impermeable seal in proximal-radial direction as well as the extremely endangered structures within the compartment. The flexor carpi ulnaris muscle is recommended to be the "primary structure" for measuring the tissue pressure as well as for surgical decompression. The article reviews the anatomical base of the CS.

[New aspects of the ontogenetic development of the human colon]. / Neue Aspekte zur ontogenetischen Entwicklung des menschilichen Colons.

The parts of the fetal colon--later retroperitoneally located (ascending and descending colon)--were examined in 31 human fetuses in order to determine their ontogenetic formation, position, and dorsal attachment. The present data cast doubt upon so-called "descending of the caecum" described in current textbooks of embryology. Considering the literature available a new concept of the formation of the right colon flexure and the ascending colon is presented.

[The altered membrane of the erythrocyte. I. Ultrahistochemical and biocellular investigations for the detection of activated acetylcholinesterase (ACHE) and demasking of IgG receptor sites (author's transl)]. / Die alterierte Erythrocytenmembran. I. Ultrahistochemische und zellbiologische Untersuchungen zur Aktivierung der Acetylcholinesterase (ACHE) und Demaskierung von IgG-Receptoren.

Different states of the erythrocyte membrane with regard to its disintegration are characterized. The binding power of autologous and allogenic IgG, the degree of the activation of the membrane associated acetylcholinesterase (inhibited in the intact plasmalemma of red blood cells), and the membrane vesiculation served as criteria. The findings demonstrate that, obviously, the IgG binding increases in dependence on the extent of the disturbance of the membrane structure. The acetylcholinesterase is increasingly activated. The enzyme can be demonstrated by spectrophotometrical and ultrahistochemical methods. Microvesiculation is understood as expression of fundamental disturbances of the membrane structure. These disturbances express local remodelling processes in the membrane of banked red blood cells. Highly extended damage of red blood cells after mechanical stress, heat or urea incubation lead to comparatively high rates of vesiculation, partially even to cell fragmentation. Extremely spectrindeficient ghosts tend to microvesiculation, which leads to complete microvesicular decay of the ghost membrane. The membrane associated autologous IgG is demonstrated by means of immuneological and ultrahistochemical methods. Its importance as homeostatically effective immun-signal for the elimination of red blood cells aged in vivo or in vitro, ghosts and microvesicles by the reticulohistiocytic-system is evidenced by means of model experiments. Molecular mechanisms for unmasking of IgG-receptor sites and activation of acetylcholineesterase in the altered erythrocyte membrane are discussed.

[Genesis of microcytes in hemodialysis patients with chronic uremia]. / Untersuchungen zur Genese von Mikrocyten bei Hämodialysepatienten mit chronischer Urämie.

The study is concerned with factors that may reduce the life time of erythrocytes of patients under intermittent haemodialysis. The red cell population of these patients consisted of micro-, normo-, macro- and megalocytes. The classification of the cell size was shiftet to greater diameters in comparison with healthy donors. The evaluation of the biological half time of erythrocytes labelled with 51Cr resulted in a decrease of about 16 days. The autoradiographic evaluation of the erythrocyte size-distribution patterns 1 and 24 hours after the injection of labelled red cells into the donors circulation clearly demonstrated a decrease of macro- and megalocytes. Simultaneously, the number of microcytes rose considerably. The measurement of erythrocyte deformability could demonstrate a clearcut relationship of flexibility for a) disturbed erythropoiesis resulting in the production of macro- and megalocytes, and b) fragmentation and premature elimination from the circulation due to unfavourable rheologic properties of erythrocytes of abnormal big and small sizes.