[Adults with congenital heart disease presenting to the emergency department : Potential pitfalls]. / Erwachsene mit angeborenen Herzfehlern im Notaufnahmedienst : Was ist zu beachten?

The number of adults with congenital heart disease (ACHD) already exceeds the number of children with congenital heart disease in the industrialized world. ACHD patients often show complex pathophysiology and anatomy even after reparative cardiac surgery. In case of complications patients may rapidly deteriorate and become unstable, even when they were asymptomatic or had only mild symptoms before the onset of the complication. Compared to all patients seen by emergency physicians, emergencies in ACHD patients are still rare. This review is aimed to guide management in ACHD emergency situations. Approximately two-thirds of all emergency admissions are caused by arrhythmias or acute heart failure. Sustained arrhythmias may rapidly lead to acute cardiac decompensation in ACHD patients. If medical treatment fails or patients present in hemodynamically unstable conditions, prompt electrical cardioversion is mandatory. Symptomatic bradycardia may require urgent pacemaker implantation. Depending on the underlying heart defect, placement of temporary transvenous pacemaker leads may be impossible. Acute heart failure in ACHD patients is often caused by acute right heart failure. Other more frequent emergencies are infections, syncope, thromboembolic events, and aortic dissection. It is highly recommended to contact the tertiary care center that follows the patient regularly early in case of patient presentation to the emergency room.

[Pulmonary hypertension associated with left heart disease: recommendations of the Cologne Consensus Conference 2016]. / Pulmonale Hypertonie bei Linksherzerkrankungen: Empfehlungen der Kölner Konsensus-Konferenz 2016.

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. While the guidelines contain detailed recommendations regarding pulmonary arterial hypertension (PAH), they contain only a relatively short paragraph on other, much more common forms of PH such as PH due to left heart disease. Despite the lack of data, targeted PAH treatments are increasingly being used for PH associated with left heart disease. This development is of concern because of limited ressources and the need to base treatments on scientific evidence. On the other hand, PH is a frequent problem that is highly relevant for morbidity and mortality in patients with left heart disease, representing an unmet need of targeted PH therapies. It that sense, the practical implementation of the European Guidelines in Germany requires the consideration of several specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, several working groups were initiated, one of which was specifically dedicated to PH associated with left heart disease. This article summarizes the results and recommendations of this working group.

[Pulmonary hypertension in grown-ups with congenital heart disease: Recommendations of the Cologne Consensus Conference 2016]. / Pulmonale Hypertonie bei Erwachsenen mit angeborenen Herzfehlern: Empfehlungen der Kölner Konsensus-Konferenz 2016.

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for different forms of PH, and specifically address PH associated with congenital heart disease (CHD). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in grown-ups with congenital heart disease (GUCH). This article summarizes the results and recommendations of this working group.

Endothelial function in contemporary patients with repaired coarctation of aorta.

OBJECTIVE: Previous studies have suggested endothelial dysfunction in adult patients after repair of aortic coarctation (CoA). It has been proposed to play a key role in the pathogenesis of arterial hypertension in the absence of re-coarctation. We aimed to assess the presence of endothelial dysfunction, the number of endothelial progenitor cells (EPC), and the levels of proinflammatory cytokines associated with endothelial injury in contemporary patients after CoA repair. METHODS: For this prospective observational study, 20 CoA patients and 22 healthy controls were recruited. Digital reactive hyperaemia was measured by peripheral arterial tonometry. Flow cytometry was used to quantify EPCs, and a comprehensive panel of laboratory markers of endothelial dysfunction was measured. RESULTS: Half the patients had known arterial hypertension requiring medical treatment. Indices of reactive hyperaemia showed no significant difference between CoA patients (1.96±0.32) and controlss (1.765±0.48) (p=0.82). Circulating EPCs, defined by the number of CD34(+), CD34(+)/KDR(+), CD34(+)/AC133(+), CD34(+)/AC133(+)/KDR(+) or CD34(+)/CD45(-) labelled cells were equally not significantly different between the groups. Furthermore, plasma levels of inflammatory mediators and markers of endothelial function (IL-6, IL-8, ICAM1 and VCAM1) were not significantly different between the groups, as were vascular endothelial growth factor levels (p>0.05, for all). CONCLUSIONS: By contrast with earlier reports, no clinically significant difference in endothelial function between adult patients with coarctation repair and healthy controls could be demonstrated. Therefore, endothelial dysfunction may not necessarily be present in this population. Further studies are required to identify mechanisms and to develop strategies to avoid arterial hypertension in these patients.

Prevalence and prognostic implication of restenosis or dilatation at the aortic coarctation repair site assessed by cardiovascular MRI in adult patients late after coarctation repair.

BACKGROUND: Cardiovascular magnetic resonance (CMR) is ideal for assessing patients with repaired aortic coarctation (CoA). Little is known on the relation between long-term complications of CoA repair as assessed by CMR and clinical outcome. We examined the prevalence of restenosis and dilatation at the repair site and the long-term outcome in patients with repaired CoA. METHODS AND RESULTS: CMR imaging and clinical data for adult CoA patients (247 patients aged 33.0 ± 12.8 years, 60% male), were analyzed. The diameter of the aorta at the repair site was measured on CMR and its ratio to the aortic diameter at the diaphragm (repair site-diaphragm ratio, RDR) was calculated. Restenosis (RDR≤70%) was present in 31% of patients (and significant in 9% [RDR<50%]), and dilatation (RDR>150%) in 13.0%. A discrete aneurysm at the repair site was observed in 9%. Restenosis was more likely after resection and end-end anastomosis, whereas dilatation after patch repair. Systemic hypertension was present in 69% of patients. Of the hypertensive patients, blood pressure (133 ± 20/73 ± 10 mm Hg) was well controlled in 93% with antihypertensive therapy. Mortality rate over a median length of 5.9 years was low (0.69% per year, 95% CI: 0.33-1.26), but significantly higher than age-matched healthy controls (standardised mortality ratio 2.86, CI 1.43-5.72, p<0.001). CONCLUSION: Restenosis or dilatation at the CoA repair site as assessed by CMR is not uncommon. Medium term survival remains good, however, albeit lower than in the general population. Life-long follow-up and optimal blood pressure control are likely to secure a good longer term outlook in these patients.

Pulmonary arterial hypertension in adults with congenital heart disease.

Pulmonary arterial hypertension (PAH) is a common problem in adult patients with congenital heart disease. We review available data on aetiology, clinical presentation, prognosis and management of PAH in this setting. In addition, we discuss general management strategies and emerging disease-targeting therapies.

[(Re)habilitation after cochlear implantation]. / (Re)habilitation nach Versorgung mit einem Kochleaimplantat.

Over the last years, indications for cochlear implants (CIs) have changed dramatically. The benefits depend on the preconditions of the individual patient as well as on the subsequent (re)habilitation. Therefore, many variables influencing the hearing and speech perception of a CI user must be kept in mind. As an example, the special situation of children having Turkish as their mother tongue is described. The most convincing argument concerning (re)habilitation is its benefit. Indeed, this benefit represents the final standard of quality and serves as the yardstick for standard assessments of (re)habilitation quality. CI (re)habilitation includes medical, pedagogical, audiological, hearing and speech, and psychological therapeutic aspects.

Early natural auditory-verbal education of children with profound hearing impairments in the Federal Republic of Germany: results of a 4 year study.

Early education of children with hearing impairments has been carried out in the FRG for the past ca. 40 years using a variety of different educational concepts. One of these concepts is the natural auditory-verbal approach. By supporting the development of hearing, even amongst children with profound hearing impairments, it stakes the claim of being capable of initiating the children's development of natural auditory-verbal skills, which are then comparable to those of children with normal hearing. Nevertheless, to this date, no empirical study of the approach and its measure of success had ever been undertaken. During the course of this study, the measures involved in a hearing-oriented system of early education were comprehensively examined. The analysis was performed on the advancements made in the natural auditory-verbal skills of infants suffering a loss of hearing of 90 dB or more and who were educated in such a way. The study comprised 103 children with profound hearing impairments, who were younger than 24 months old at the time the study began. Their respective developments were followed in three separate surveys between 1996 and 1998. The results were analysed using bivariate as well as statistical correlation methods. The children's development is impeded by such factors as late initial diagnosis; delayed supply of hearing aids and late commencement of early education; poorly-adjusted hearing aids and short periods of wearing them. A system of early education that is only to a very limited degree hearing-oriented, and a family environment where little is spoken and where the child's auditory disability receives only scant attention, may also play a part. On the other hand, under favourable circumstances, even children with profound hearing-impairments may attain a development level of natural auditory-verbal skills which corresponds to that of children who can hear well-perhaps with a certain time-delay, and certainly involving a greater effort on the part of the children, but basically, in the same natural way and with a very similar quality of results. The early education system must urgently be improved by the introduction of screening for new-born infants. As far as devices are concerned, the cochlear-implant (CI) will very soon be standard equipment for infants with profound hearing-impairments. Within institutional facilities, there is a broad consensus that the goal of these measures consists of the development of speech communication skills in children. In practice, however, the strategies for realising this goal are not only very different, but also, partly, have downright counterproductive effects.

Do deaf people see better? Texture segmentation and visual search compensate in adult but not in juvenile subjects.

The research concerning the visual perception in deaf subjects has led to contradictory results: Deaf subjects have been reported to show enhanced visual perceptual skills compared to hearing subjects (Neville & Lawson, 1987). On the other hand, there are indications that acoustic deprivation may produce an inferiority in all sensory modalities (Myklebust, 1964). These contradictions may be due to methodological differences: The investigators selected different conditions (e.g. attentive/nonattentive) and various samples of deaf subjects (e.g., different age, language, and aetiology groups). In our study, we tested a large sample of deaf subjects with texture segmentation and visual search conditions, which allowed us to differentiate between visual processing with and without attentional load. All deaf subjects had profound hearing loss within the first year of life. Our results suggest that the visual processing capacity of deaf children and adolescents does not exceed that of age- and gender-matched hearing subjects. Rather, deaf school children show deficits in visual processing in conditions with and without attentional load. Age (6 to 20 years), language used (oral, sign, oral + sign), and aetiology for deafness (genetic, maternal rubella, perinatal, infection in the first year of life, unknown) did not consistently influence the results. The deficits in visual processing were partially compensated for in adult deaf subjects. The performances of deaf and hearing adults in trials that could be solved preattentively did not differ statistically significantly, but in attention-dependent trials the deaf subjects were more efficient than the hearing controls. We conclude that visual compensation for deafness is limited to attention-dependent tasks and does not develop until adulthood.

Results of cochlear implantation in patients with severe to profound hearing loss--implications for patient selection.

In patients with some residual hearing and minor benefit from conventional hearing aids, the benefits of cochlear implantation have to be weighed carefully against eventual adverse effects. In this study, pre- and post-operative thresholds as well as functional results after cochlear implantation are reported; 17 of 44 implanted adults had residual hearing pre-operatively (mean threshold(250 to 4000 Hz): 106 dB HL) in the implanted ear. Residual hearing in the implanted ear could not, in general, be preserved post-operatively. Seventeen of 44 implanted children had some amount of residual hearing in the implanted ear pre-operatively (implanted ear: 114 dB HL; contralateral ear: 109.9 dB HL; mean thresholds(250 to 4000 Hz))). Contrary to the results in adults, residual hearing in the implanted ear remained statistically unchanged. Hearing in the contralateral ear increased significantly from 109.9 to 101.9 dB HL post-operatively. This increase was mainly attributed to maturation of the central auditory pathway. In adults with residual hearing, the monosyllable word recognition scores increased significantly from 9 per cent pre-operatively to 42 per cent post-operatively. Children with residual hearing tended to perform better on speech-related test material compared to children without prior auditory experience. Cochlear implantation is indicated in adults and children with residual hearing and minor benefit from conventional amplification. The contralateral ear in children should be considered for additional acoustical stimulation.

[Management of idiopathic urticaria with H1 + H2 antagonists. A crossover double blind long-term study]. / Behandlung der idiopathischen Urticaria mit H1 + H2-Antagonisten. Ergebnisse einer Crossover-Doppelblind-Langzeitstudie.

A randomized crossover double-blind study in a selected group of patients with idiopathic urticaria (15 patients; 10 females, 5 males; 20-80 years old) has been performed in order to examine the clinical efficacy of H1 + H2-antagonists in this disease, as compared with the H1-antagonist alone and with placebo. Chlorpheniramine (4 X 4 mg/d) and cimetidine (4 X 400 mg/d) were administered, each of them over 4 weeks, after one week wash-out period. The number of wheals, the time of their persistance as well as the presence of itching were daily registered and then evaluated. The entire group showed no difference between antihistamines and placebo. Nevertheless, 4 patients have registered a definitely better response to the H1-antagonist, 4 other patients responded distinctly better to the combined H1 + H2-treatment and 5 patients showed no preference at all. In a long-term follow up period of 3 months the effect of chlorpheniramine became even better, whereas the response to the combined treatment remained unchanged. It seems that the clinical efficacy of H1 + H2-antagonists is rather moderate in idiopathic urticaria. Nevertheless, in individual cases non-responding to H1-antagonist the combined administration of H1 + H2-antagonists may be more beneficial for the patient.

[H2-antagonists and possibilities for their therapeutic use in dermatology]. / H2-Antagonisten und Möglichkeiten ihrer therapeutischen Anwendung in der Dermatologie.

H2-antagonists differ from the commonly applied antihistamines (H1-antagonists) by blocking a different spectrum of histamine-mediated pharmacologic reactions. Their effects on the skin as the target organ may be stronger, weaker, or even reverse. The main representative of this group of drugs is cimetidine. Other compounds are still in experimental stages. Some controversial effects were reported in urticaria, pruritus, atopic dermatitis, mastocytosis of the skin, and also in acne and psoriasis. With polyetiologic symptoms, as are manifested in cases of urticaria and pruritus, the efficacy of the drug may depend on the underlying disease. In acne and psoriasis, the clinical type and stage of the disease may also play a major role in the outcome of such studies. Experimental and clinical findings suggest that cimetidine has some immunomodulating effect in terms of influencing the delayed type skin hypersensitivity. The intake of cimetidine should be registered in patch testing. Application of H2-antagonists may be beneficial in diseases with reduced immune resistance (generalized mycotic infections). Serious group-specific side-effects of H2-antagonists are not yet known. Several side-effects have been reported following oral intake of cimetidine; however, their frequency seems rather low.

Antagonistas H2 y su posible aplicacion en terapeutica dermatologica. / H2 antagonists and their possible use in dermatological therapeutics

Los antagonistas H2 constituyen un grupo relativamente nuevo de medicamentos que se diferencian de los antihistaminicos habituales (antagonistas H1) porque bloquean diferentes acciones farmacologicas de la histamina. El principal representante de este grupo de medicamentos es la cimetidina. Los resultados hasta ahora comunicados en urticaria,prurito, dermatitis atopica, mastocitosis, acne y psoriasis son en parte contradictorios. En el caso de sintomas polietiologicos como la urticaria y el prurito, los efectos terapeuticos probablemente dependan de la enfermedad subyacente. Estudios experimentales y clinicos sugieren que la cimetidina actua como inmunomodulador, influenciando las reacciones cutaneas de tipo tardio. Su aplicacion puede ser de beneficio en enfermedades con respuestas immunologicas deficientes. Acciones colaterales serias, grupo-especificas de antagonistas H2, practicamente no se conocen. Existen multiples comunicaciones sobre efectos colaterales de la cimetidina,pero su frecuencia es en general baja

Antagonistas H2 y su posible aplicacion en terapeutica dermatologica. / H2 antagonists and their possible use in dermatological therapeutics

Los antagonistas H2 constituyen un grupo relativamente nuevo de medicamentos que se diferencian de los antihistaminicos habituales (antagonistas H1) porque bloquean diferentes acciones farmacologicas de la histamina. El principal representante de este grupo de medicamentos es la cimetidina. Los resultados hasta ahora comunicados en urticaria,prurito, dermatitis atopica, mastocitosis, acne y psoriasis son en parte contradictorios. En el caso de sintomas polietiologicos como la urticaria y el prurito, los efectos terapeuticos probablemente dependan de la enfermedad subyacente. Estudios experimentales y clinicos sugieren que la cimetidina actua como inmunomodulador, influenciando las reacciones cutaneas de tipo tardio. Su aplicacion puede ser de beneficio en enfermedades con respuestas immunologicas deficientes. Acciones colaterales serias, grupo-especificas de antagonistas H2, practicamente no se conocen. Existen multiples comunicaciones sobre efectos colaterales de la cimetidina,pero su frecuencia es en general baja

[alpha 1-antitrypsin determination in children with obstructive lung disease (author's transl)]. / Uber die Alpha1-Antitrypsin-Bestimmung (AAT) bei Kindern mit obstruktiven Ventilationsstörungen.

In 184 children and juvenile with type I respiratory allergies the AAT-content in serum was measured by the rocket technique. The results do show neither disease--nor agedependant changes. The mean of the total was 283 mg%. This value does agree very well with the normal values reported in literature. In 5 patients however (2,71%) of the total collective an intermediary AAT-deficiency was found. This frequency though lies within the percentage of occurrence in non-selected patients. The individual analysis of these cases did not give any valid connection to history, age, lung function, immunoglobulins and course of disease. On the basis of the demonstrated results it can be said, that the determination of AAT in serum patients with type-I-allergy is of no practical diagnostic value.