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R Kozlik-Feldmann

Publications and source records attributed to R Kozlik-Feldmann.

At least 19 recordsLinked to original sources
PubMed2006

Recurrent pericarditis in children: elevated cardiac autoantibodies.

UNLABELLED: Recurrent pericarditis is a rare disease in childhood. Nevertheless, it may represent a challenge to the clinician due to its resistance to anti-inflammatory treatment. The initial etiology often remains unclear; specific laboratory parameters predicting the frequency or severity of the recurrences are lacking. We report on four patients with recurrent pericarditis in whom antimyolemmal antibodies (AMLAs) were detected. A prolonged persistence of IgM-type AMLAs was found in three patients: two of them presented with acute inflammation as the initial event and one with 48 recurrences during 5.5 years. The fourth patient showed a fast conversion from IgM to IgG-type AMLAs after a less acute initial presentation and showed 4 mild recurrences during the 48-month follow-up. CONCLUSION: We were able to detect AMLAs in four children with recurrent pericarditis. This finding may be attributed to an auto-immunological disease following a first, acute event. We propose the detection of AMLAs in all children with unexplained recurrent pericarditis. Pediatric patients with a persistence of IgM-type AMLAs may face frequent recurrences and should be monitored therefore more closely. In addition, medical treatment may be changed in these patients with a slower tapering of the dosage of steroidal and non-steroidal antiinflammatory drugs.

Acute Disease↗
PubMed2006

First experience with the 2005 modified Gore Helex ASD occluder system.

We report about our initial experience for the in 2005 modified Helex (Gore) device for closure of atrial septal defects (ASD) and persistent foramen ovale (PFO). Major changes were made at the delivery system for simplifying the Helex implantation procedure. We treated 11 patients, 8 children and 3 adults, with ages between 3 and 62 years. In 10 patients the diagnosis was a relevant ASD with volume overload of the right heart (Left to right shunts between 30 and 50%). One adult (age 58 years) have had a small left to right shunt with a PFO-like defect and the history of 2 neurologic embolic events. In 3 patients we found 2 defects. In all patients a Helex occluder was implanted successfully. The mean fluoroscopy time was 8,4 minutes. The immediate occlusion rate after 24 hours was 91%. In all cases there was a very good adaptation of the device to the anatomical structures. In this small series, the Helex occluder appears to offer a reliable system of occlusion for small and moderate ASDs and for PFO with minimal risk of major complications.

Adolescent↗
PubMed2006

Interventional embolization of a giant pulmonary arteriovenous malformation with right-left-shunt associated with hereditary hemorrhagic telangiectasia.

A 46-year old woman experienced an episode of arterial desaturation despite administration of 100% oxygen during anesthetization for an elective laparoscopy. Further evaluation revealed a giant pulmonary arteriovenous malformation (PAVM) with right-left shunt associated with previously undiagnosed hereditary hemorrhagic telangiectasia (HHT, Morbus Osler- Weber-Rendu). The PAVM was treated interventionally with an Amplatzer duct occluder. Transcatheter embolization of the PAVM was well tolerated with symptomatic and hemodynamic improvement. CT scan after six months demonstrated correct position of the duct occluder in the left pulmonary artery with nearly complete occlusion of the feeding vessel.PAVMs are rare direct communications between pulmonary arteries and veins, associated with HHT in the majority of cases and often presenting with dyspnea or major neurological complications due to paradoxic embolism. In this case report, we present a rational and stepwise diagnostic workup for this rare medical condition and show that transcatheter embolization is an appropriate treatment for larger malformations.

Arteriovenous Malformations↗
PubMed2006

Calculating sympathovagal balance from heart rate variability: are there alternatives in adolescents?

OBJECTIVE: Assessing sympathovagal balance by calculating LF/HF-ratio from power spectral analysis (PSA) of heart rate variability (HRV) may be difficult in adolescents as chaotic breathing leads to methodical bias and metronomic breathing is not easy to perform. Diastolic blood pressure variability (dBPV) is less influenced and may therefore offer more stable values for calculations. The present study was performed on 72 paediatric subjects to investigate possible alternative LF/HF-calculations from PSA of HRV and dBPV. METHODS AND RESULTS: Seventy-two paediatric individuals in three groups: 12 controls, 17 heart- and heart-lung-transplanted children (TX) and 43 adolescents born small for gestational age (SGA). Short-term beat-to-beat HRV and BP-recordings were made supine and during active standing. Ratios calculated: LF/HF from HRV, LF/HF from dBPV, LF-dBPV/HF-HRV and LF-HRV/HF-dBPV. LF/HF from dBPV as well as LF-HRV/HF-dBPV did not correlate with LF/HF-HRV. Correlation of LF/HF from HRV and LF-dBPV/HF-HRV was high especially in TX and in patients with resting heart rate of above 90 beats per minute. CONCLUSIONS: In adolescents, the ratio of LF-dBPV/HF-HRV may be an alternative method for calculating sympathicovagal balance being less influenced by breathing patterns. In younger patients with elevated resting heart rate, but also in patients with very low HRV such as TX-patients this method could be a supplemental diagnostic tool whenever autonomic nervous control on the cardiocirculatory system has to be assessed.

Adolescent↗
PubMed2005

Results of pediatric cardiac transplantation -- long-term results of a 15-year experience.

INTRODUCTION: Heart transplantation (HTx) has increasingly become a therapeutic option for end-stage heart failure of any origin in children. Short- and mid-term results are promising. However, long-term outcome has been a matter of concern because of acute or chronic rejection and side effects of immunosuppression. We performed a retrospective study of up to 15-years of follow-up on this patient entity. METHODS: Between 1988 and 2004, 58 HTx were performed in 55 children (cardiomyopathy (DCM) 32, congenital heart disease (CHD) 23, Re-HTx 3). Mean age was 9.1 +/- 7.2 years (4 days - 17.9 years). Twenty-nine patients had a total of 51 previous operations. RESULTS: Operative mortality was 4/58 (6.8 %) due to primary graft failure. Late mortality was 7/54 (12.1 %) due to acute rejection (2), pneumonia (2), intracranial hemorrhage (1), suicide (1) and lymphoma (1). Mean follow-up was 5.2 +/- 4.2 years. One-, 5-, and 10-year survival was 86 %, 80 % and 80 %, respectively, and improved significantly after 1995 (92 % and 92 %; p = 0.04). Survival was comparable for DCM and CHD patients (1-year: 88 % vs. 82 %; p = 0.19; 5-years: 84 % vs. 77 %; p = 0.12). Three patients with therapy resistant rejection and assisted circulation required retransplantation and are alive. Freedom from acute rejection was 46 % with primary cyclosporine immunosuppression and 63 % with tacrolimus. Ninety-eight percent of the survivors are at home and in excellent cardiac condition. CONCLUSION: Pediatric heart transplantation is a curative treatment for DCM and CHD with excellent clinical mid-term results. However, further follow-up is necessary to evaluate long-term side effects of immunosuppressants. Donor shortage remains a problem.

Cardiomyopathy, Dilated↗
PubMed2003

[Extracorporeal membrane oxygenation (ECMO) as cardiac assist device in pediatrics].

Extracorporeal membrane oxygenation (ECMO) is a common treatment for severe respiratory failure. However, ECMO can also be used as cardiac support. ECMO is the most common mechanical circulatory assist device in pediatrics, followed by pulsatile ventricular assist devices. Advantages are the simultaneous support of respiratory and hemodynamic functions and the fast bedside availability. The major disadvantage is the limited duration of therapy. The transthoracic connection of ECMO after cardiac failure in cardiothoracic surgery in childhood allows a controlled weaning on the intensive care unit. Weaning from ECMO is successful in about 45 to 80 % in cases of circulatory support; survival rates depend on patient selection, treatment protocol and indication. Bleeding problems, thromboembolic complications, hemolysis, infections, renal and neurologic complications are major problems.

Cardiac Surgical Procedures↗
PubMed2002

Borderline hypoplastic left heart malformations: Norwood palliation or two-ventricle repair?

BACKGROUND: Patients with hypoplastic left heart syndrome (HLHS) and associated malformations undergo Norwood palliation or potentially a two-ventricle repair. METHODS: Since 8/99, 8 patients with typical HLHS and two with DILV underwent Norwood/Fontan palliation (group I). Three other patients (group II) had two-ventricle repair. Anatomy was: aortic atresia, coarctation, unrestrictive VSD (patient 1), hypoplastic mitral and aortic valve, arch and LV, coarctation (patients 2 and 3). Surgical procedures were Norwood arch reconstruction with either Rastelli operation (patient 1) or ASD-closure (patients 2 and 3). RESULTS: Operative mortality in group I was 1/8 (day 22; RV-failure). Two patients died before Glenn (sepsis, RV-failure). Six patients underwent Glenn procedure successfully. No patient died in group II. Echocardiography after 13 +/- 7.4 months showed mild homograft dysfunction (patient 1) and an LVOT-gradient of 20 mmHg (patient 3). Clinical condition of all survivors in both groups is good. CONCLUSION: Some anatomical subsets of HLHS with borderline mitral valves and small left ventricles may undergo two-ventricle repair despite severe LVOTO. Mortality and morbidity seem to be lower, but selection criteria are so far not defined.

Adolescent↗
PubMed2002

Minimally invasive surgery for congenital heart defects in paediatric patients.

BACKGROUND: In selected cases, minimally-invasive approaches are favoured for the correction of congenital heart defects with regard to better cosmetic results. METHODS: Between July 1999 and April 2001, 25 children (9 male; mean age 5.8 +/- 4.1 years; mean weight 19.6 +/- 12.6 kg) were operated on using minimally invasive approaches. Diagnoses were: ASD (n = 19), VSD (n = 2), ostium primum defect (n = 3) and Tetralogy of Fallot (n = 1). Female patients with ASD underwent a limited right anterolateral thoracotomy. A ministernotomy was chosen in male patients, in patients under 6 months of age, and in patients with malformations other than ASD. Cannulation was always performed via the chest incision. RESULTS: There were no perioperative complications. Mean operation time was 3.23 +/- 0.89 h. Twelve patients were extubated immediately after surgery, mean ventilation time in the others was 12.1 +/- 11.7 h. Mean ICU stay and hospital stay were 1.5 +/- 0.75 days and 8.3 +/- 2.2 days, respectively. Follow-up (mean 4.8 +/- 4.6 months) was uneventful. CONCLUSIONS: Intracardiac repair of some congenital heart defects can be performed safely and effectively via minimally-invasive approaches. Indications are expanding towards more complex defects. Exposure for precise repair is good, additional incisions can be avoided, and cosmetic results have been excellent.

Adolescent↗
PubMed2001

Effect of cardiopulmonary bypass on myocardial function, damage and inflammation after cardiac surgery in newborns and children.

UNLABELLED: Postoperative measurement of cardiac troponin I, creatine kinase and procalcitonin reflects myocardial damage and systemic inflammatory response after cardiac surgery with cardiopulmonary bypass in children. Pulse-contour cardiac output technique is a less invasive tool for determining postoperative cardiac function. OBJECTIVE: The aim of our study was to investigate myocardial lesions and systemic inflammatory response after cardiac surgery with cardiopulmonary bypass in children suffering from congenital heart defects. METHODS: The elevation of cardiac troponin I (cTnI), creatine kinase (CK) and procalcitonin (PCT) was evaluated in relationship to duration of aortic cross-clamping, incisional trauma and cardiac bypass temperature in 37 paediatric patients. To assess postoperative cardiac function, the cardiac index was measured in 7 children using the PiCCO (pulse contour cardiac output) technique. RESULTS: CTnI and PCT both peaked on the day of surgery and slowly decreased postoperatively in case of an uncomplicated course. The median values of both parameters differed significantly from the day of surgery until the fourth postoperative day in children with an aortic cross-clamping time (CCT) longer than 80 minutes or after ventriculotomy in comparison to patients with shorter clamping times or atriotomy only. CK values showed similar results, but were less significant than cTnI. A relationship between cTnI, CK or PCT and the body temperature during cardiopulmonary bypass was not found. The cardiac indices (CI) measured by the PiCCO technique in the first 48 hours after surgery showed normal values. CONCLUSION: In summary, perioperative measurement of cTnI, CK and PCT reflects myocardial damage and systemic inflammatory response and allows an improved peri- and postoperative management. PiCCO technique is an excellent, less invasive tool to determine postoperative cardiac function.

Biomarkers↗
PubMed2000

Switch from a BIVAD to a LVAD in a boy with Kawasaki disease.

A 9-year-old boy with Kawasaki disease survived after two severe myocardial infarctions. Thereafter pharmacologically untreatable ventricular arrhythmia and rapidly deteriorating heart failure developed in the patient. After 19 days of biventricular and a further 27 days of left univentricular mechanical circulatory support with the Berlin Heart (Cardiotechnica, Berlin, Germany) assist device the boy successfully underwent heart transplantation. At a follow-up of 54 months, the boy is leading an active and unrestricted life.

Arrhythmias, Cardiac↗
PubMed1997

A post-receptor defect of adenylyl cyclase in severely failing myocardium from children with congenital heart disease.

The aim of this study was to determine whether a defect at the post-receptor level of adenylyl cyclase may also contribute to the decreased effectiveness of cAMP-increasing agents in severely failing patients with congenital heart disease. The severity of congestive heart failure in 31 patients with congenital heart disease was graded by a scoring system which included a description of historical and clinical variables. Patients were divided into a group with no or mild heart failure (score < or = 6) and a group with severe heart failure (score > 6). beta-Adrenoceptor-stimulated adenylyl cyclase activity was significantly decreased by 65% in patients with severe heart failure in comparison to the group of patients with no or mild heart failure. In addition, receptor-independent adenylyl cyclase stimulation by forskolin was reduced by 52% in patients with score > 6 compared to patients with score < or = 6. This post-receptor defect of adenylyl cyclase was apparently due to a decrease in the activity of catalytic subunit of adenylyl cyclase as adenylyl cyclase stimulation by forskolin in the presence of Mn2+ which uncouples catalytic subunit from the G proteins, G(s) and G(i), was also significantly diminished in the patients with severe heart failure. In contrast, the level of inhibitory G protein alpha-subunits was apparently not different in the two groups. In summary, the data indicate that a defect at the catalytic subunit of adenylyl cyclase apparently contributes to the decreased effectiveness of cAMP-increasing agents in severely failing patients with congenital heart disease.

Adenosine Diphosphate↗
PubMed1997

Surgical treatment of the Bland-White-Garland syndrome in early infancy: subclavian to coronary artery anastomosis via left anterolateral thoracotomy extended by a transverse sternotomy.

The case of a 6-week-old girl with left coronary artery originating from the pulmonary artery (Bland-White-Garland syndrome) is reported. Therapy consisted of a subclavian-coronary artery anastomosis via left anterolateral thoracotomy extended by transverse sternotomy. This approach allowed both the easy dissection of the left subclavian artery and the use of extracorporeal circulation while doing the anastomosis. Operation and postoperative course were uneventful. Angiography after one year revealed a patent anastomosis and a totally recovered left-ventricular function.

Coronary Artery Bypass↗
PubMed1996

Long-term effects of formoterol and salbutamol on bronchial hyperreactivity and beta-adrenoceptor density on lymphocytes in children with bronchial asthma.

Long-term treatment with short-acting beta 2-sympathomimetic drugs has recently been suggested to be due to a rise in asthma mortality. This effect has been attributed to an increase in bronchial hyperreactivity, a desensitization of beta 2-adrenoceptors and/or a rebound effect after cessation of the therapy. Formoterol, a new long-acting beta 2-symathomimetic drug, has been reported to possess not only bronchodilatation but also antiinflammatory effects. The aim of the present study was to investigate whether long-term effects of salbutamol and/or formoterol could deteriorate asthma management. Therefore, in a trial lasting 90 days, we evaluated the effects of the two drugs on lung function, on the protection they provided against inhalative provocation with histamine, and on tachyphylaxis as monitored by the beta-adrenergic density on mononuclear leukocytes (MNL). Two groups of 11 children each with stable asthma were treated with daily doses of either 4 x 200 micrograms salbutamol or 2 x 24 micrograms formoterol in monotherapy. The lung function was measured six times during the trial period, on day 1, and on day 90, before and after inhaling the drug and at all investigations both with and without histamine provocation. In both groups, the bronchodilatory effect of either formoterol or salbutamol remained constant. The lung function values before and after drug inhalation (specific airway resistance sRAW, forced expiration volume FEV1, vital capacity VC) did not alter significantly. After histamine provocation, the protection was more pronounced in the formoterol group. No changes in the beta-adrenoceptor density on MNL and no significant side effects were seen throughout the trial period. We therefore conclude that formoterol protects significantly better against the bronchial challenge with histamine than does salbutamol. Both drugs did not deteriorate the lung function in asthmatic children. In addition, there was no evidence that long-term treatment leads to a desensitization of beta 2-sympathomimetic effects.

Adrenergic beta-Agonists↗
PubMed1993

Distribution of myocardial beta-adrenoceptor subtypes and coupling to the adenylate cyclase in children with congenital heart disease and implications for treatment.

In congestive heart failure, down-regulation of myocardial beta-adrenoceptors (beta-AR) due to an elevated sympathetic tone is well known. In infancy and childhood, heart failure is usually related to congenital heart disease (CHD). Therefore, 71 samples of right atrial tissue of infants and children with CHD undergoing cardiac surgery were studied for beta-adrenoceptor density and distribution of the beta 1-/beta 2-AR subtypes. In 49 cases, the coupling of the beta-AR to the adenylate cyclase (AC) was examined. In a further study of 19 myocardial samples, AC was selectively stimulated with beta 1- or beta 2-AR whereas the other subtype was blocked by an antagonist. The following results were obtained: (1) Infants and children with severe acyanotic or cyanotic CHD had severely reduced beta-AR densities. (2) In most of the cases, the beta-AR down regulation is beta 1-subtype selective, but in critically ill newborns with congenital aortic valve stenosis or transposition of the great arteries, there is additional significant beta 2-AR down-regulation. In Fallot patients treated with the beta-antagonist propranolol, a significant increased beta-AR number compared with untreated Fallot patients was found. (3) beta-Adrenoceptor reduction in CHD is correlated with elevated noradrenaline plasma levels, thus proving a sympathetic dysregulation. (4) In CHD with moderate hemodynamic load, beta 2-AR coupling to AC was markedly more efficient than beta 1-AR coupling. The small number of myocardial beta 2-AR produced most of the cyclic adenosine monophosphate. (5) In severe acyanotic and cyanotic CHD, a partial decoupling of the beta 2-AR to the AC occurred.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenylyl Cyclases↗
PubMedDate not supplied

Echocardiographic assessment of left ventricular size and function in normal children from infancy to adolescence: acoustic quantification in comparison with traditional echocardiographic techniques.

The purpose of this study was to establish normal left ventricular acoustic quantification (AQ) reference values for children from infancy to adolescence and to compare AQ-derived parameters with traditional M-mode and Doppler indices. We studied 150 normal, healthy children aged 2 days to 14 1/2 years. Left ventricular parasternal end diastolic area and left ventricular end diastolic volume calculated by AQ were linearly related to the body surface area to the 1.1 and 1.4 powers. AQ parasternal peak filling rate and atrial fractional area change in the neonatal period were 140% and 142% of the adolescent's values, respectively, and decreased to 110% and 112% by 36 months of age. The duration of the rapid filling phase and the rapid filling contribution, as identified by AQ, was shorter or lower than that measured by the Doppler method. Interobserver variability of AQ parameters ranged from 8.1% for the ejection fraction to 18.2% for the peak filling rate. Manual biplane determinations of volumes were slightly higher than AQ calculations with highly significant correlations (p <0.001). Our data permit the determination of normal ranges of AQ parameters in relation to body surface area or age.

Acoustic Impedance Tests↗
PubMedDate not supplied

["Real time"-3D-echocardiography: a valuable new tool in preoperative assessment of complex cardiac defects].

Evaluating complex cardiac defects in small children preoperatively requires multiple diagnostic procedures including echocardiography, but also the invasive methods such as cardiac catheterisation, computer-tomography and magnetic resonance imaging. We tried to assess the complex anatomy of the atrioventricular valves in atrioventricular septal defect using bedside real-time three-dimensional echocardiography and comparing these results to the anatomic findings at the time of operation.

Age Factors↗