Health-related quality of life in paediatric patients with congenital heart defects: association with the type of heart defect and the surgical technique.

The aim of the study was to investigate the impact of a number of surgical interventions for a various congenital cardiac defects (CHDs) on self-reported HRQoL. METHODS: Patients who had received corrective surgery of several congenital heart defects (surgical VSD closure, Fallot, TGA after atrial or arterial switch or Fontan-type circulation for univentricular AV-connection) were interviewed in the office of their home peadiatric cardiologist. HRQoL in children along 7 dimensions was assessed using a standardised questionnaire (PEDQoL); information on the medical case history of each respondent was also collected. STATISTICS: HRQoL was assessed in the questionnaire by asking about the frequency (never, rarely, often, always) of specific negative experiences; more frequent experiences indicate a lower quality of life. Frequency expressions were transformed into numerical values (25, 50, 75, 100%), and mean values for HRQoL were calculated for each patient and for each domain. Differences in HRQoL among patients with different types of interventions were analysed using the Mann-Whitney Test or the Kruskal-Wallis Test as appropriate; p values <0.05 were considered to indicate significant differences, while p values <0.1 were considered to indicate notable trends. RESULTS: Patients: 169 patients (60% male, 40% female) were part of the study. The mean age was 11.6 years; 50 patients had surgical VSD closure, 52 surgeries for Tetralogy of Fallot (22 transannular patch, 18 no transannular patch, 12 inaccurate description), 40 had complete transposition of the great arteries (28 atrial switch, 12 arterial switch), 22 had a Fontan-type procedure for univentricular AV-connection. HRQoL differed little among patients with different CHDs for the items "relation to friends," "interactions in the affected families", and "own body image". For other items, notable differences emerged: patients with univentricular hearts rated their physical capacity worse and showed a tendency towards negative ratings in other domains. On the other hand, patients after Fallot or TGA correction tended to rate their HRQoL in several domains as relatively high. Focusing on the mode of surgery for Fallot repair, respectively, TGA correction the only significant difference was found for "physical capacity" in TGA (atrial vs. arterial repair). Mustard patients tended to rate most items worse. Physical capacity was rated worst by patients with a Fontan circulation. Repeated surgery led to lower ratings for all domains except "physical capacity" and "body image". CONCLUSIONS: Different surgical techniques for CHD do not affect children's and adolescents' self-reported HRQoL to the extent that one would expect. This observation is in line with observations in groups of children with different chronic diseases. Specialised psychosocial support is necessary in order to maintain this positive self-evaluation and to ensure patients are able to lead autonomous personal and professional lives.

Positive or negative fructose breath test results do not predict response to fructose restricted diet in children with recurrent abdominal pain: results from a prospective randomized trial.

OBJECTIVES: To perform a prospective, blinded, randomized interventional trial in patients with recurrent abdominal pain. The primary endpoint was to determine the abdominal pain intensity after 2 weeks of fructose restricted diet. Secondary endpoints were changes of pain frequency and a secondary symptom score (SSS). METHODS: 103 individuals with recurrent abdominal pain for more than 3 months were randomized. 51 patients were allocated to group A (diet) and 52 to group B (no diet). 2 weeks later the patients underwent hydrogen breath test and were assigned to the test positive or negative group to identify patients with fructose malabsorption. RESULTS: 2 weeks after intervention the pain score decreased significantly from a median 5.5 in group A to 4 and did not change significantly in group B (5.3 to 5). In group A both patients with positive and negative breath tests had a significant lower pain score (-2 and -1.75, respectively). Frequency of abdominal pain decreased in both groups but without significant difference, SSS improved only in group A from median 6 to 3.5. Positive breath test was no predicting factor, neither was abdominal pain during the test. CONCLUSIONS: Fructose restricted diet in children and adolescents with recurrent abdominal pain may be of benefit to improve both abdominal pain symptoms and other secondary symptoms. Since a negative breath test result does not exclude a positive response to fructose restriction, the hydrogen breath test does not seem to be the appropriate diagnostic mean to predict the response to the diet.

Health related quality of life after corrective surgery for congenital heart disease.

UNLABELLED: After corrective surgery for congenital heart defects (CHD) many patients suffer from residual defects, some with reduced cardiorespiratory capacity and possible impairment of their health related quality of life (HRQoL). The aim of our study is to evaluate, how children after surgery for CHD rate their HRQoL. METHOD: A standardised questionnaire deve-loped for self-rating in children between 8 and 18 years and dealing with 7 different domains concerning the HRQoL (friends, family, physical functioning, cognition, body image, emotional function and autonomy) was independently answered from patients and their parents during an outpatient visit at their pediatric cardiologist throughout Germany. RESULTS: 173 patients (40% female, 60% male, mean age 11.6 years) were interviewed, 167 questionnaires could be evaluated. The mean time interval after surgery was 9.8±3.4 years. Patient had had surgery for complete different types of CHD (ventricular septal defects n=50, Tetralogy of Fallot n=51, univentricular heart n=26, transposition of great arteries n=40). The results were compared with those of an age-matched control group (n=169). Patients with CHD reported a better HRQoL than the controls for all items (p<0.01). There was no significant gender specific difference. After puberty, the rating for most items, except of "body image", had become very similar compared to controls. Pa-rents assessed their children significantly worse in 3 domains (friends, body image and emotion; p<0.01). CONCLUSION: Children with congenital heart defects are able to develop coping structures, that enable them to live a normal life from their individual point of view. Integration in psychosocial structures seems to be rather normal when compared to healthy controls. Many patients considered their HRQoL as even better.

Assessment of humidity and temperature sensors and their application to seating.

Humidity and temperature are considered to be important factors in designing comfortable seat surfaces. A small number of studies have attempted to address this;however the methods used were limited regarding the placement of their sensors. This study aimed to design a sensor array system to investigate changes in humidity and temperature for eventual use in the study of factors affecting sitting comfort and incontinence detection. The system was subjected to three types of experiments: sensor response verification, thermal radiation testing and in situ trials. The variance in output within each type of sensor was small (+3.5% and +0.38 degrees C) and there was no apparent change to the variance in output of the sensors, when used in air or on a foam cushion loaded with a 50 kg sand bag (p > 0.1). In the human sitting experiments, although the profile from sensors under the thighs and ischial tuberosities were similar, the magnitude of change could be affected by position and body mass of the subject. This was especially noticeable with the sensors under the coccyx. These results support the use of multiple sites for sensor placement over the use of a single site when studying these parameters at the interface between subject and seating material at the seat base.

Present risk of anthracycline or radiation-induced cardiac sequelae following therapy of malignancies in children and adolescents.

Anthracyclines are very potent drugs in the therapy of malignancies in childhood. The major dose limiting adverse effect of these drugs is the risk of dilated cardiomyopathy. We performed a retrospective study on 168 patients who were treated with anthracyclines for a malignant disease with or without chest radiation at the department of Pediatric Hematology and Oncology at the University of Duesseldorf between 2000 and 2004. During and after chemotherapy the patients were screened by echocardiography and ECG examinations prior to each administration of anthracyclines. Only four patients presented with adverse cardiac events, one of whom developed acute cardiac failure. This patient was additionally treated with chest radiation. Three of the four patients showed intermittent arrhythmias, mainly supraventricular tachycardia. One of them presented with atrial ectopic tachycardia and left ventricular dysfunction. We conclude that the frequency of cardiac sequelae after chemotherapy with anthracyclines is low under present guidelines. Detection of early cardiac sequelae may be more difficult than in the past. Only one patient with cardiac sequelae in our study group was diagnosed by regular performed examinations for cardiac sequelae of chemotherapy. We therefore need to modify our screening methods to increase the effectiveness of detection of cardiac dysfunction prior to clinical manifestation.

Thermal screening of facial skin arterial hot spots using non-contact infrared radiometry.

Non-contact infrared thermometry of facial skin offers advantages over less accessible internal body sites, especially when considering mass screening for febrile infectious disease. The forehead offers an obvious site, but does not present an isothermic surface, as various small arteries passing close to the surface create 'hot-spots'. The aim of this study is to use non-contact infrared (IR) thermometry to determine the link between the temperature at specific facial skin sites and clinical body temperature. A sample of 169 asymptomatic adults (age range 18-54 years) was screened with IR thermometers (Braun Thermoscan proLT for auditory meatus (AM) temperature representing clinical body temperature, and a Raytek, Raynger MX for skin surface temperature). Peak IR skin temperature was measured over the course of each posterior auricular artery (PAA) and each superficial temporal artery (STA). In a sub-group (n = 54) the peak skin temperature of the forehead's metopic region (MR) was also recorded. There were no differences (P > 0.05) between the PAA and STA at 34.2 +/- 0.9 degrees C and 34.2 +/- 0.7 degrees C, respectively, which were 2.5 degrees C cooler than the AM temperature (36.7 +/- 0.5 degrees C, p < 0.001). Although there was no correlation between AM and PAA or STA there was a correlation (r2 = 0.63, p < 0.001) between PAA and STA. There were no asymmetric temperature differences between the left and right sides and males had warmer skin over the MR (F, 33.6 +/- 0.7 degrees C versus M, 34.4 +/- 0.6 degrees C, p < 0.001). Although a lack of correlation between either PAA or STA and AM was apparent in asymptomatics, further research in symptomatics is required to determine the usefulness of these measurements in mass screening of conditions such as fever.

The vagaries of ear temperature assessment.

There have been a growing number of reports suggesting that ear temperature measurement is unreliable and by implication so is the device. Examination of the measurement site, the tympanic membrane (eardrum) and the walls of the external auditory meatus (ear canal) reveals that at least some of the unreliability might derive from poor aiming of the infrared thermometer: the ear canal walls have a lower temperature than the eardrum. Additionally, anatomical properties of the ear canal may increase the difficulty of aiming the thermometer at the eardrum. Furthermore, the rich vascularization, innervation and variations in skin properties (thickness, oil secretion and hair) along the length of the ear canal affect the black body-like nature of the structure. It is concluded that such factors are more likely underlying reasons for the difficulties in reliably reproducing temperatures from this site. We conclude that concerns should extend beyond the reliability of the device and there should be greater study of the measurement site. The argument extends to all sites chosen for clinical assessment of the patient, as previous studies of the alternative temperature measurement sites are also unfortunately few and tend to be lacking in scientific rigour.

The effect of factors such as handedness, sex and age on body temperature measured by an infrared 'tympanic' thermometer.

BACKGROUND: The effectiveness of IRET has been called into question. However, there are a number of factors that might affect ear temperature measurement. Some of these are the focus of this study. METHOD: 366 asymptomatic subjects (0.75 to 53 years old) volunteered to take part in this ethically approved study. The data were analysed in respect to handedness, sex and age. RESULTS: Two-way ANOVAs showed significant interactions between age group and sex (first ear measured, p = 0.017; second p = 0.298), and between handedness and sex (first ear measured, p = 0.011; second p = 0.040) in relation to their ear temperature. Ear temperatures of males tended to be lower than females, however this only found significance in the 'Early Puberty' age group (p < 0.001). DISCUSSION: Significant differences in ear temperature were seen for handedness, sex and age. CONCLUSION: We have shown that handedness, sex and age might be contributory factors to ear temperature differences in the population.

[Extracranial aneurysm of the carotid artery in a 12-year-old girl]. / Aneurysma verum der Arteria carotis im Kindesalter.

Extracranial aneurysms of the carotid artery in childhood are rare, and we found just nine cases described in the literature within the last 20 years. We report on the clinical appearance, diagnosis, and therapy of a carotid artery aneurysm in a 12-year-old girl and review the literature.

Acute hemorrhagic pericarditis in a child with pneumonia due to Chlamydophila pneumoniae.

Chlamydophila pneumoniae is mainly responsible for respiratory tract infections but has also been associated with endocarditis and myocarditis. We report a case of pneumonia in a child with hemorrhagic pericardial effusion with a positive result by a new C. pneumoniae TaqMan PCR, suggesting a pericardial inflammation directly induced by C. pneumoniae. C. pneumoniae should be suspected in patients with community-acquired pneumonia and concurrent pericarditis. Empirical treatment with azithromycin seems feasible.

Management of the persistent ductus arteriosus in infants of very low birth weight: early and long-term results.

The hemodynamically relevant persistent ductus arteriosus (PDA) impairs pulmonary and cardiac function. Frequently, PDA can be closed only via surgery. In this retrospective study, early and long-term results in very low birth weight newborns are evaluated. Eighty-seven of 634 very low weight newborns presented with PDA All patients (pts; age: +/-14 days; weight: +/-1064 g) were ventilator-dependent. Surgical closure (after 29+/-5 days) was indicated if echocardiography and prolonged ventilation (>20+/-2 days) evidenced a hemodynamically relevant PDA. Sixteen pts, in which indomethacin therapy failed preoperatively are included in the 36 surgically treated pts; no pt died intra- or early postoperatively (<3 day). Overall mortality 30 days after delivery was n=9. Early plus late mortality was n=19. Long-term follow-up (3-12 years) in 46 (68%) pts: 15 were solely physically, 11 were mentally and neurologically, and 4 were physically, mentally and neurologically retarded. From these 30 pts, 15 were severely (e.g. tetraspasm; severe cerebral paresis) and 15 were slightly (e.g. psychosomatic and language development prolongation) retarded. Sixteen pts exhibited no disability; no long-term complications owing to surgery. The relatively large number of neurological injuries was not owing to chromosomal syndromes or pre-existing abnormalities but can be explained by severe and frequent prematurity, hypoxia, and intracerebral bleeding. Indomethacin was successful only in a few patients. Early surgery (after frustran early indomethacin therapy) of a hemodynamically relevant PDA is recommended. In the long-term, severe disabilities develop.

Cardiomyopathy and pericardial effusion in infancy point to a fatty acid b-oxidation defect after exclusion of an underlying infection.

Pericardial effusion and cardiomyopathy in the first year of life point to a fatty acid b-oxidation defect as underlying disease after exclusion of infectious causes. We report two patients with the early-onset, cardiac phenotype of very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency; in one patient, severe pericardial effusion was the predominating symptom. Because specific noninvasive treatment is available in fatty acid b-oxidation defects that reverses cardiomyopathy and pericardial effusion, early diagnosis is essential to adjust therapy accordingly.

[Aortico-left ventricular tunnel: a rare differential diagnosis of congenital aortic insufficiency]. / Aortico-linksventrikulärer Tunnel: Seltene Differentialdiagnose einer kongenitalen Aorteninsuffizienz.

Aortico-left ventricular tunnel (ALVT) is a rare congenital cardiac malformation that presents with cardiac failure and clinical findings of severe aortic regurgitation in early infancy. The diagnosis should be made with 2-D echocardiography by demonstrating an abnormal tunnel-like structure that connects the ascending aorta with the left ventricle. Up to 45% of the patients may have associated cardiac defects, and the development of this bizarre lesion is still unclear. In contrast to congenital aortic regurgitation, ALVT has a better prognosis. Early surgical intervention is curative, but non-operated children die because of heart failure. Therefore, knowledge of this rare cardiac malformation is mandatory if an infant presents with findings of aortic regurgitation.

Volumetric analysis of the right ventricle in children with congenital heart defects: comparison of biplane angiography and transthoracic 3-dimensional echocardiography.

BACKGROUND: Three-dimensional echocardiography is a non-invasive imaging technique. The fact that it permits volumetric analyses independently of geometrical assumptions makes it a putatively useful method for the precise measurement of the volumes of the irregularly shaped right ventricles in children. The aim of this study was to assess the feasibility of this method and its agreement with angiocardiography based estimates of right ventricular volume in children with congenital heart disease. METHODS: We studied 102 children with congenital heart disease. The angiocardiographic right ventricular volumetry was performed using a biplanar technique using Simpson's rule and corrected with Lange's correction factors. The echo data sets were registered trans-thoracically with a rotating transmitter. Volumes were calculated after manual planimetry by adding the volumes of the individual slices. RESULTS: Calculation of right ventricular volume echocardiographically was possible only in 34% of patients, mostly infants and toddlers. In comparison to angiocardiography, the measured volumes were 1.1 +/- 6.9 ml (19.5 +/- 34.1%) or 6.3 +/- 9.4 ml (42.5 +/- 33.6%) smaller during systole or diastole, respectively. The limits of agreement were -12.5 and 13.6 ml, or 12.45 and 25.15 ml during systole or diastole, respectively. When plotted to a logarithmical scale, the correlation coefficients r2 were 0.70 for systolic and 0.79 for diastolic measurements. CONCLUSION: Transthoracic 3-dimensional echocardiography with a rotating transmitter is feasible for volumetry only in small children. The volumes measured were significantly smaller than the ones calculated from the angiocardiographic images. The correlation between the two methods is moderate.

Balloon valvoplasty in infants with tetralogy of Fallot: effects on oxygen saturation and growth of the pulmonary arteries.

Balloon valvoplasty was undertaken in 27 patients with tetralogy of Fallot for first-stage palliation. Indications were arterial saturation of oxygen < 80%, hypoxic spells and duct-dependant pulmonary perfusion. The dilation was performed following diagnostic heart catheterization. Saturations improved from 75% +/- 8.5 before valvoplasty to 85% +/- 8.4 after the procedure, and worsened little to 83% +/- 9.6 at follow-up after 3.4 months. The pulmonary valvar orifice was hypoplastic in most patients (Z = -3.3 +/- 1.2), and did not change after the procedure. The cross-sectional area of the pulmonary arteries was diminished initially, reflected by a Nakata index of 186 +/- 95 mm2/m2. After valvoplasty, we found widening of the vessels (Nakata index 225 +/- 100 mm2/m2). At follow-up no further growth of the arteries was observed (Nakata index 209 +/- 109 mm2/m2). The procedure was complicated by hypoxic spells in three patients which were controlled by intravenous propranolol, and deep venous thrombosis in four patients. Our data demonstrate that balloon valvoplasty is feasible for initial palliation in patients with tetralogy of Fallot. It does not, however, produce growth of the pulmonary arteries or of the pulmonary valve.

Volumetric analysis of the right and left ventricle in a porcine heart model: comparison of three-dimensional echocardiography, magnetic resonance imaging and angiocardiography.

UNLABELLED: Three-dimensional echocardiography and magnetic resonance imaging allow the volumetric analysis of ventricular volumes independent of geometric assumptions. The aim of the study was to compare these methods and the common angiocardiography in a cardiac model of known volume. METHODS/MATERIALS: Right and left ventricular (RV, LV-) volumes were measured in a specific animal model directly ('true volume') and with different imaging techniques. Three-dimensional echocardiography (3D-Echo) and magnetic resonance imaging (MRI), both of which permit a volume estimation without necessitating geometric assumptions, and angiocardiographic volumetry which is based on the Simpson rule were used in this study. RESULTS: The best results were achieved with MRI (RV: r(2)=0.99, mean difference: -1. 9+/-3.3%; LV: difference r(2)=0.99,: 2.9+/-5.0%). Likewise, 3D-Echo showed a very good correlation with the true volumes (RV: r(2)=0.93, difference: 9.3+/-6.3%; LV r(2)=0.96, difference: 4.8+/-9.9%). The greatest deviations were observed during angiocardiographic volumetry (LV: r(2)=0.98; difference: 14.4+/-9.2%), particularly when measuring the right ventricle (RV: r(2)=0.82, difference: 57. 9+/-40.1%). Consequently, the direct comparison between 3D-Echo and the other methods yielded the best correspondence with MRI (RV: Bias: 3.7 ml, limits of agreement: 7.7 ml; LV: Bias: 3.7 ml, limits of agreement: 4.9 ml). In contrast, the differences between 3D-Echo and angiocardiography were marked (RV: Bias: 25.5 ml, limits of agreement: 11.1 ml; LV: Bias: 8.7 ml, limits of agreement: 13.2 ml). CONCLUSION: In a porcine cardiac model, 3D-Echo permits a relatively precise measurement of ventricular volumes with a slight under-estimation. MRI yielded the most precise volumetry, and the correlation between 3D-Echo and MRI was quite good. Particularly for the right ventricle, the angiocardiographic measurement was attached with the greatest error and thus appears ill-suited for the volumetry of geometrically more complex ventricles.

Volume measurement of the left ventricle in children with congenital heart defects: 3-dimensional echocardiography versus angiocardiography.

BACKGROUND: Volume measurement of the left ventricle is currently done by 2-dimensional echocardiography or angiocardiography. However, for the calculation of volumes by these methods, geometrical assumptions must be made. For a precise ventricular volumetry, independent of mathematical assumptions, imaging techniques as the 3-dimensional echocardiography (3D-echo) are required, which permit the imaging of the real ventricular shape. The aim of the study was therefore to detect, whether 3D-echocardiography is suitable for left ventricular volumetry in children with congenital heart disease and whether the 3D-echocadiographically measured volumes correlate sufficiently well with angiocardiographically measured left ventricular volumes. METHODS: 102 children with congenital heart disease were investigated. For angiocardiographic left ventricular volume measurement Simpson's rule was used. Results were corrected with Lange's correction factors. The 3D-echo data sets were registered with a rotating transthoracic transducer. Ventricular volumes were calculated after manual planimetry by summation of the volumes of the single slices. RESULTS: The left ventricular volume could be calculated by 3D-echo in 83% of patients of all ages. In comparison to angiocardiography, the measured volumes were 0.6 +/- 3. 3 ml (0.9 +/- 25.8%) or 7.1 +/- 28.4 ml (7.4 +/- 12.1%) smaller during systole or diastole, respectively. The correlation coefficients r(2) were 0.89 for systolic and 0.93 for diastolic measurements after logarithmic transformation. Pressure or volume overload did not influence significantly the difference between the two methods. CONCLUSION: Transthoracic 3D-echocardiography with a rotating transmitter is feasible for volumetry of the left ventricle in most children. The volumes measured by 3D-echo were significantly smaller than those calculated from the angiocardiography by Simpson's rule. The measurements were not influenced by the kind of load of the ventricle. The correlation between the two methods is good.

Anomalous origin of all coronary arteries from the pulmonary trunk.

The origin of both coronary arteries from the pulmonary artery is a rare cardiac malformation. We report a baby who presented with an echocardiographically diagnosed perimembranous ventricular septal defect and normal left ventricular (LV) function. Later on the boy developed failure to thrive and increasing tachypnea. At the age of 5 weeks the ECG showed that LV strain and echocardiographic LV function had worsened (FS 18%). Echocardiography and heart catheterization showed that all coronary arteries originated from the pulmonary trunk. Intraoperative inspection revealed a single ostium for the right and left coronary artery in the nonfacing sinus of the pulmonary trunk. A tube was constructed connecting the coronary artery to the ascending aorta. Coronary perfusion was sufficient and the sinus rhythm was restored. However, in the early postoperative period there was a sudden deterioration of cardiac output followed by cardiac arrest. Reanimation was not successful.

Complete AV-block in two children with immunohistochemical proven myocarditis.

UNLABELLED: We report two children with acquired third degree AV-block caused by acute myocarditis. The diagnosis was proven by endomyocardial biopsy. Severe lymphocytic myocardial infiltration was shown using immunohistological methods. One of the children was treated with prednisone During therapy conduction disturbance nearly disappeared and infiltration was markedly reduced in a subsequent biopsy. In the other patient the parents refused immunosuppressive treatment and a permanent pacemaker was necessary for persistent bradycardia. CONCLUSION: Immunohistological staining of an endomyocardial biopsy can be used to establish the diagnosis of myocarditis in patients with atypical clinical manifestation, such as complete AV-block, and can support the decision for therapy. In one patient improvement was documented by the disappearance of inflammatory activity in a repeated biopsy.

Left aortic arch and right descending aorta--interruption or severe coarctation of the aortic arch in the newborn.

Three consecutive neonates (aged 7, 14 and 30 days, body-weight 2980 g, 3000 g and 3400 g respectively) with interruption of the aorta (n = 2) and severe coarctation (n = 1) in the presence of left aortic arch and right descending aorta are reported. Associated lesions were an aortopulmonary window in the first case and an unrestrictive ventricular septal defect in the two others. Intractable heart failure and the complexity of the malformation led to the decision of a staged operation. A prosthetic graft was interposed between the ascending and descending aorta via a right thoracotomy in order to bridge the atretic or hypoplastic segment without using extracorporeal circulation. There was no intraoperative complication. One patient developed a thrombocytopenia within the frame of a sepsis syndrome and died on day 5 after operation, death being caused by a massive bleeding into the left thoracic cavity, although the operation was carried out via a right thoracotomy. Angiography 1 year after operation revealed a good flow through the grafts and no stenosis at the site of the anastomoses. The two surviving infants are clinically well without any medication. The reported operative technique provides an alternative palliative possibility to manage critically ill neonates without any obstacle to later definitive repair.