Respiratory syncytial virus-associated hospitalizations over three consecutive seasons in children with congenital heart disease.

The purpose of this investigation was to analyze the burden of respiratory syncytial virus (RSV)-related hospitalizations in infants and children with congenital heart disease (CHD) over three consecutive RSV seasons. Retrospectively, all children with hemodynamically significant (HS-CHD) and not significant (HNS-CHD) CHD born between 2004 and 2008 at a tertiary care university hospital and identified by ICD-10 diagnoses were included. Data on RSV-related hospitalizations over the first three years of life covering at least three RSV seasons (November-April) were analyzed. The overall incidence of RSV-related hospitalization was 9.6 % (58/602), without a statistically significant difference between HS-CHD and HNS-CHD (7.3 % vs. 10.4 %; p = 0.258). Recommendation of palivizumab prophylaxis did not influence the RSV hospitalization rates between groups. Patients with HS-CHD and early surgery were significantly less often hospitalized due to RSV compared to those with delayed surgery (1.3 % vs. 14.3 %; p = 0.003). The median duration of hospitalization was 8.5 days (HS-CHD: 14 vs. HNS-CHD: 7 days; p = 0.003). Thirteen patients (22.4 %) were admitted to the intensive care unit (ICU), for a median of 10 days. The median age at admission was 2 months, with a significant difference between HS-CHD and HNS-CHD (6 vs. 2 months; p = 0.001). The majority (97 %) of RSV-related hospitalizations occurred before 12 months of age. Patients with HS-CHD had a significantly more severe course of RSV disease and were older at the time of hospitalization. Early surgery seemed to significantly reduce the risk of RSV hospitalization during the first RSV season.

Acylated ghrelin increases after controlled short-time exercise in school-aged children.

Ghrelin is a peptide hormone, which plays an important role in appetite regulation. The effects of exercise on ghrelin plasma concentrations are still not clear, especially in children and adolescents. The aim of this study was to investigate the response of acylated and total ghrelin concentrations to controlled exercise in school-aged children. Thirty-six healthy school-aged children (mean age 12.61 years, SD ± 0.39) underwent a controlled bicycle exercise test. Before and immediately after exercise, blood samples were taken in order to measure plasma ghrelin concentrations. The control group consisted of 24 healthy school-aged children. After controlled short-time exercise, total ghrelin concentrations showed no significant difference, whereas acylated ghrelin concentrations increased significantly (P<0.001) in the study population compared with the control group. Moreover, we found a correlation between the proportional increase of acylated ghrelin and the duration of exercise (P<0.01), and between the proportional increase of acylated ghrelin and maximal performance (P<0.01). Increased levels of acylated ghrelin after exercise could be a physiological response to ensure a sufficient caloric intake after energy consumption in children and adolescents.

Early-life pulmonary arterial hypertension with subsequent development of diffuse pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia type 1.

The case history is presented of a male infant who was thought to have idiopathic pulmonary arterial hypertension (PAH) at 3 months of age. Subsequently the PAH decreased unexpectedly and diffuse pulmonary arteriovenous malformations (PAVMs) were seen at 6.9 years of age for the first time. Hereditary haemorrhagic telangiectasia type 1 (HHT1) related to an endoglin mutation was diagnosed. At 10.3 years of age a lung biopsy showed diffuse PAVMs as well as pulmonary arteriopathy with medial hypertrophy. This is the first case of HHT1 presenting with PAH at such a young age. The subsequent decrease in pulmonary arterial pressure (PAP) was probably caused by the development of PAVMs. In the presence of PAVMs, measurement of the PAP may underestimate the extent of PAH-related vasculopathy.

Right ventricular function in patients treated with inhaled nitric oxide after cardiac surgery for congenital heart disease in newborns and children.

Measurement of right ventricular (RV) function is essential for complete assessment of the effects of inhaled nitric oxide in the postoperative cardiac patient; nitric oxide therapy can result in a decrease in pulmonary vascular resistance and improved echocardiographic RV ejection fraction without necessarily inducing a significant change in pulmonary artery pressure.

Inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis.

OBJECTIVE: The aim of this study was to evaluate the effects of inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis. METHODS: Inhaled nitric oxide (mean 4.1 +/- 0.7 ppm, 1.5 to 10 ppm) was administered in 13 patients (mean age 5.6 +/- 1.6 years, 1.5 to 17 years) with critical pulmonary perfusion (central venous pressure > 20 mm Hg or transpulmonary pressure gradient > 10 mm Hg) in the early postoperative period after total cavopulmonary connection (n = 9) or after bidirectional Glenn anastomosis (n = 4). RESULTS: In patients after total cavopulmonary connection inhaled nitric oxide therapy decreased central venous pressure by 15.3% +/- 1.4% (p = 0.0001) and transpulmonary pressure gradient by 42% +/- 8% (p = 0.0008) and increased mean systemic arterial and left atrial pressures by 12% +/- 3.6% (p = 0.011) and 28% +/- 8% (p = 0.007), respectively. Arterial and venous oxygen saturations improved by 8.2% +/- 1% (p = 0.005) and 14% +/- 4.3% (p = 0.03), respectively. In patients after bidirectional Glenn anastomosis inhaled nitric oxide therapy resulted in a decrease of central venous pressure by 22% +/- 1% and of the transpulmonary pressure gradient by 55% +/- 6% and improved arterial and venous oxygen saturations by 37% +/- 29% and 11% +/- 3%, respectively. Mean systemic arterial and left atrial pressures remained nearly unchanged. No toxic side effect was observed in any patient. CONCLUSION: Inhaled nitric oxide may play an important role in the management of transient critical pulmonary perfusion caused by reactive elevated pulmonary vascular resistance in the early postoperative period after Fontan-type operations and bidirectional Glenn anastomosis.

Successful treatment of CMV retinitis with ganciclovir after allogeneic marrow transplantation.

Cytomegalovirus (CMV) infection of the retina is a well recognized complication in patients with the acquired immune deficiency syndrome but is rarely seen after bone marrow transplantation (BMT). Among a variety of drugs ganciclovir so far appears to be the most effective therapy for CMV retinitis, but in previous studies relapses occurred in all patients in whom ganciclovir was interrupted. We report the clinical findings in a 22-year-old BMT recipient who developed bilateral exudative CMV retinitis 64 days after BMT despite prophylactic treatment with high-titer CMV-immunoglobulins and transfusions of CMV-negative blood products and donor bone marrow. During a 12 day course of treatment with 7.5 mg/kg/day of ganciclovir the CMV retinitis improved and viruria ceased on day 4 of therapy. In contrast to the previous reports, CMV retinitis in this patient continued to improve even after ganciclovir was stopped and eventually complete healing of all intraretinal lesions as well as total reconstitution of the visual acuity was achieved. He is now free of disease and without relapse of CMV retinitis more than 1 year after transplantation.

Endocrine function after bone marrow transplantation without the use of preparative total body irradiation.

Ten children who underwent allogeneic (n = 5) or autologous (n = 5) bone marrow transplantation (BMT) for chronic myelogenous leukaemia (n = 2), acute lymphoblastic leukaemia (n = 1), acute myelogenous leukaemia (n = 2), severe aplastic anaemia (n = 2), malignant histiocytosis (n = 1), neuroblastoma (n = 1) and teratoma (n = 1) were assessed for endocrinological function. Transplant preparative regimens consisted of high-dose cyclophosphamide, high-dose cyclophosphamide in combination with high-dose busulphan, high-dose melphalan as well as BACT (BCNU, cytarabine, cyclophosphamide and 6-thioguanine) chemotherapy. None of the patients received total body irradiation (TBI). Median survival following BMT was 37 months (range 7-115). Growth hormone deficiency was present in only one patient; none of the patients had abnormal thyroid or adrenocortical function. This is in contrast to previous reports in which growth hormone deficiency and abnormal thyroid and adrenocortical function occurred in a much higher percentage of patients after BMT conditioned with TBI.

Busulfan/cyclophosphamide plus bone marrow transplantation is not sufficient to eradicate the malignant clone in juvenile chronic myelogenous leukemia.

Bone marrow transplantation plays an essential role in the successful treatment of both juvenile and adult chronic myelogenous leukemia. Recently, it has been reported that conditioning with high doses of busulfan can successfully replace total body irradiation (TBI), in patients with acute myelogenous leukemia as well as adult chronic myelogenous leukemia. We report here the case of a 29-month-old boy with juvenile chronic myelogenous leukemia (JCML) transplanted with HLA-identical bone marrow after conditioning with busulfan, etoposide and cyclophosphamide. Successful engraftment was followed by early relapse on day 67. A second HLA-identical transplant was performed following myeloablative treatment with TBI. Engraftment was once again successful and the patient remains free of disease more than 24 months after transplantation. We conclude that busulfan is insufficient in eradicating JCML and that TBI is required prior to transplantation.

Transcatheter coil occlusion of residual interatrial communications after Fontan procedure.

OBJECTIVE: To assess the use of detachable coils as an alternative method to occlude interatrial communications after Fontan operations. DESIGN: Descriptive clinical study of selected patients after Fontan operation with interatrial communications inappropriate for transcatheter umbrella occlusion. SETTING: Tertiary paediatric cardiac referral centre. PATIENTS: Seven patients after Fontan operation with residual interatrial communications of various types producing a right to left shunt. INTERVENTIONS: Transcatheter placement of detachable coils with a diameter of 3 or 5 mm within the interatrial communication. RESULTS: A total of 14 coils were successfully placed within persistent patent fenestrations of the interatrial baffle, residual leaks at the suture line between the patch material and the right atrial wall, and unusual venous interatrial communications. The mean (SD) aortic oxygen saturation increased from 88 (1.1)% (range 86-89%) to 92 (1.3)% (range, 89-93%; p < 0.001) and the mean (SD) right atrial pressure rose from 9.7 (2) mm Hg (range, 6-11) to 10.6 (2.4) mm Hg (range, 6-13; p < 0.05) after coil implantation. In five patients, complete obliteration of the interatrial shunt was shown by angiography after coil implantation. At a mean (SD) follow up of 10 (4) months (range, 3-15) a residual interatrial shunt was detected by Doppler colour echocardiography in only one patient, and oxygen saturations ranged from 90% to 95% (mean, 92%). There were no late coil embolisations, thromboembolic events, or haemolysis in any patient. CONCLUSIONS: Detachable coils can be used successfully to occlude residual interatrial communications after the Fontan procedure. In selected cases, in whom intended transcatheter umbrella occlusion of residual interatrial leaks is not possible, the use of detachable coils might offer a safe alternative method to eliminate interatrial right to left shunting after the Fontan procedure.

Effect of neonatal treatment with capsaicin on carrageenan-induced paw oedema in the rat.

The time course of the paw oedema induced by the subplantar injection of carrageenan was studied in rats treated neonatally with capsaicin and in their vehicle-treated controls. In the capsaicin-treated rats, which show a permanent deficit of unmyelinated primary sensory neurones, carrageenan produced an oedema which was larger and lasted longer than in the vehicle-treated rats. Pretreatment with the histamine liberator compound 48/80 reduced the carrageenan-induced paw oedema only in the capsaicin-treated rats whereas pretreatment with indomethacin reduced it in both groups of rats. The increased and prolonged inflammatory response to carrageenan in capsaicin-treated rats may be explained by an enhanced release of histamine from mast cells and may also reflect a 'trophic disorder of the denervated skin'.

Surgical emergency embolectomy for the treatment of fulminant pulmonary embolism in a preterm infant.

A massive pulmonary embolism, demonstrated by echocardiography developed in a 3-week-old preterm infant. An etiologic explanation could not be obtained from either history or clinical and laboratory findings. Pulmonary embolectomy was performed as an emergency procedure because of severe hemodynamic impairment despite intensive medical therapy. In children who have massive pulmonary embolism who remain in a compromised hemodynamic state despite intensive medical therapy, pulmonary embolectomy may be considered the alternative emergency treatment.

Inhaled nitric oxide in infants and children after open heart surgery.

OBJECTIVE: To assess the effects of inhaled nitric oxide (NO) on oxygenation and pulmonary circulation in infants and children with critical pulmonary perfusion and/or hypoxemia after open heart surgery. STUDY: A prospective case series report. SETTING: A multidisciplinary pediatric intensive care unit in a University hospital PATIENTS: From June 1993 to March 1996 37 pediatric patients after open heart surgery were treated with inhaled NO. Their mean age was 2.9+/-0.6 years, their mean body weight 12.6+/-1.8 kg. METHODS: Inhaled NO was applied using a microprocessor controlled delivery system which continuously measured NO and NO2 by the chemilumniscence method. Monitoring included ECG, continuous pulse oximetry (SaO2), arterial (AP), central venous (CVP) and left atrial (LAP) pressures and in 8 patients a pulmonary artery (PAP) pressure. Inhaled NO was started at an SaO2 <90% with a fraction of inspired oxygen concentration (FiO2) >0.7, at a mean pulmonary artery pressure (MPAP) >50% of the mean arterial pressure (MAP), and in patients after Fontan-procedure at a CVP-LAP pressure gradient >10 mmHg. RESULTS: The mean dose of inhaled NO was 3.7+/-0.3 ppm and the mean duration was 112+/-14.7 hours. For the whole group SaO2 increased from 79.6+/-2.3 to 90.1+/-1.5% (p<0.01) within 20 minutes of NO-inhalation. Inhaled NO significantly decreased the MPAP from 47.8+/-4 to 27.5+/-2.3 mmHg (p<0.01) in 8 patients with postoperative pulmonary hypertension and significantly decreased the transpulmonary pressure (CVP-LAP) from 14.3+/-0.8 to 7.3+/-0.9 mmHg (p<0.01) in 16 patients after Glenn- or Fontan-procedure. CONCLUSIONS: Inhaled NO is very effective to decrease pulmonary artery pressure, to improve oxygenation, and to improve Fontan-circulation in infants and children after open heart surgery.

[Infections in the neutropenic phase following bone marrow transplantation: comparison of laminar airflow isolation with conventional isolation]. / Infektionen während der neutropenischen Phase nach Knochenmarktransplantation: Vergleich einer laminaren Air-flow-Isolation mit einer konventionellen Isolation.

From May 1978 to September 1989 45 patients underwent 25 allogeneic and 21 autologous bone marrow transplantations (BMT) and 1 peripheral stem cell transplantation for the following indications: severe aplastic anemia (n = 4), hematological malignancies (n = 28), malignant solid tumours (n = 12) and sideroblastic anemia (n = 1). The first group of 20 patients was isolated in a conventional hospital room, while management of the aplastic phase in the second group of 25 patients was performed in a laminar air flow (LAF) unit. All patients received total decontamination. In a retrospective analysis the number of positive blood cultures during the neutropenic period was 85% in the first group, as compared with 40% in the second group, and the number of febrile episodes was 85% versus 64%, respectively. Despite the fact that the septic morbidity was lower in the LAF group, mortality during the neutropenic period (15% in group I versus 16% in group II) was unaffected and survival rate (45% in group I versus 36% in group II) did not improve. We conclude that LAF protection will only have a positive impact on survival rate if the incidence of non-infectious complications of BMT, such as organ toxicity or graft-versus-host disease, is likewise reduced.

[Total cavo-pulmonary connection as a therapeutic principle in anatomical and functional univentricular heart]. / Die totale cavo-pulmonale Konnektion als therapeutisches Prinzip bei anatomisch und funktionell univentrikulären Herzen.

The original Fontan operation was employed for patients with tricuspid atresia, but its application has been extended to a broad spectrum of congenital cardiac defects with a functional or anatomical single ventricle. From 1989 to 1995, 35 patients (23 males, 12 females) underwent a modified Fontan procedure; their age ranged from 14 months to 15 years (mean 5.1 a). Indications for operation were the following: D(S)ILV = 16, TA = 9, MA = 3, TGA with straddling AV-valve = 2, DORV = 4 and criss-cross heart = 1. The same surgical technique was utilized in all patients with redirection of the systemic venous return by means of a bidirectional cavopulmonary anastomosis and an intraatrial baffle, adaptable to all the various forms of underlying anatomy. Associated anomalies such as dextrocardia, coarctation, anomalous systemic or pulmonary venous return, subaortic stenosis and situs anomalies were present in 26%, 9%, 26%, 9%, and 20%, respectively. 12 patients were presumed to be at high risk and underwent a two-stage procedure (n = 7 bidirectional Glenn preceding the definitive repair and n = 5 a fenestration of the intraatrial baffle). Risk factors in our group of patients were: age under 2 years, abnormal systemic venous drainage, stenotic pulmonary arteries, PVR over 2 Wood units, mPAP over 15 mmHg, AV-valve incompetence, subaortic obstruction and ventricular dysfunction. Two patients had failure of the total cavo-pulmonary connection necessitating the take-down to a bidirectional Glenn anastomosis in the early postoperative period, with one death. Hospital mortality was 24% (n = 8). One child did not survive complications following the fenestration closure 4 months postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

[Clinical relevance and indications for cardiac magnetic resonance imaging 2013: an interdisciplinary expert statement]. / Klinischer Stellenwert und Indikationen zur Magnetresonanztomografie des Herzens 2013: Ein interdisziplinäres Expertenstatement.

During the last years the indications of Cardiac Magnetic Resonance Imaging (CMRI) have been continuously expanded. However, the acceptance of the method by cardiologists and radiologists does not correlate with respect to the diagnostic potential. Several factors, such as expensive equipment, relatively long examination times, high technical know how and lack of remuneration, limit the application of CMRI in everyday clinical practice. Furthermore, doctors tend to apply more conventional, well established diagnostic procedures, the access to the method is still limited and there exist difficulties in the interdisciplinary collaboration. The interdisciplinary Austrian approach to Cardiac Imaging is aimed to improve the aforementioned problems and to support the implementation of CMRI in the diagnostic tree of cardiac diseases thus enabling a cost efficient management of patients in cardiology.

[How does the time of diagnosis affect the course of disease in children with kawasaki syndrome? A retrospective analysis at one center]. / Welchen Einfluss haben unterschiedliche Diagnosezeiten auf den Krankheitsverlauf von Kindern mit Kawasaki-Syndrom? Eine retrospektive monoinstitutionelle Analyse.

BACKGROUND: Kawasaki syndrome was described for the first time by Tomisaku Kawasaki in 1967. This disease is characterized by panvasculitis of the small blood vessels of the skin, the mucous membranes, the internal organs and the coronary vessels and has an unclear etiology. Inflammatory changes in the coronary vessels or late diagnosis are prognostically unfavorable for the early and late mortality. AIM OF THE STUDY: Since two of our patients with Kawasaki syndrome with a short, severe course died despite receiving state-of-the-art treatment, we retrospectively evaluated the medical records of all the children we have treated since October 1978 with regard to the symptoms at the time of diagnosis, intervals between the onset of the disease, diagnosis, beginning of treatment and the result of treatment. PATIENTS: Kawasaki syndrome was diagnosed in 80 patients in the period from October 1978 to October 2007. The patients were grouped according to the phase of the disease and the number of organs affected at the time of diagnosis (Asai-Score) as well as the treatment carried out. The time of the first presentation for diagnosis by the pediatrician was also considered. METHOD: This is a single-institution retrospective analysis of the medical records, echocardiography and angiography findings of all patients. In view of the change of therapy in that year, patients who had been diagnosed before 1987 were compared with those diagnosed after 1987. RESULTS: Before 1987, the patients were treated solely with high doses of acetylsalicylic acid (50-100 mg/kg/day p.o. over two to four weeks). Out of a total of 36 patients, 13 showed involvement of the coronary arteries that persisted in seven patients despite treatment. After 1987, all patients received intravenous immunoglobulins (4 x 0.5 g/kg/day resp. 1 x 2 g/kg i.v. over 12 hours). In 18 out of 44 patients, the coronary arteries were affected at the time of diagnosis, but this did not persist in any of the patients. One child died in each group. Comparing the two treatment groups also revealed that a physician was consulted for the first time after a very much shorter duration of the disease in the second treatment period (3+/-1.8 vs. 6+/-2.4 days after onset of the illness) and that a pediatrician was consulted much more frequently as the first port of call. This was reflected in a significantly earlier beginning of treatment and a simultaneous significantly lower Asai score. CONCLUSION: The retrospective evaluation of all medical records did not reveal any plausible explanation for the fatal course of the disease in one child in each of the two treatment periods. Besides the combination therapy with intravenous immunoglobulin and oral administration of acetylsalicylic acid, the greater age and the earlier commencement of treatment appeared to be salient factors resulting in complete cure of the disease in the surviving patients in the second period of treatment.

Disastrous course of recurrent infective endocarditis after tooth extraction in a young patient with bicuspid aortic valve.

In a patient with moderate malformations of the left-ventricular valves disregard of antibiotic prophylaxis after tooth extraction resulted in massive mitral- and aortic regurgitation. After surgical reconstruction a relapse produced an aorto left-ventricular fistula with septic embolizations and cardiac decompensation. Valve replacement with a homograft reconstituted the hemodynamic situation. During both episodes the Duke criteria for infective endocarditis supported the diagnosis.

Atypical Bland-White-Garland syndrome with stenosis of the origin of the left coronary artery: catheter intervention after mammary artery bypass stenosis and residual fistula to the pulmonary trunk.

A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland-White-Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula and stenosis of the shunt at the implantation site developed that resulted in a dominant perfusion of the LCA from the right coronary artery. Interventional catheterization was performed with stenting of the LCA mammary artery anastomosis and subsequent coil occlusion of the residual fistula. After this intervention the LCA was exclusively perfused by the mammarian bypass with no residual leak to the pulmonary artery.

[Closure of the persistent ductus with detachable coils]. / Verschluss des persistierenden Duktus mit ablösbaren Coils.

34 patients aged 3 months to 20 years underwent transcatheter-occlusion of their patent ductus arteriosus with detachable coils (Cook). Eight patients had a residual ductus after previous implantation of a Rashkind-occluder; 7 patients had various other cardiac malformations in addition. Only 6 patients had a large ductus with a diameter between 3 and 4 mm; all had systolic-diastolic murmurs. All other patients had ductus-diameters below 3 mm; three of them had systolic-diastolic murmurs, 17 had systolic murmurs, and 8 patients had no murmur at all. The ductus was closed in 24 patients using arterial access only, in 6 patients via a venous, and in 4 patients both via venous and arterial catheterization. One coil was used in 23 patients, 2 coils in 9, and 3 coils in 2 patients. There were no complications of the intervention. Within 24 hours 31 patients (93%) had complete closure of the ductus and 32 patients (94%) after 6 months. Coil-embolisation of the persistent ductus is a quick, safe and cheap method to close a ductus and has clear advantages compared to an operation.