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Biomedical subjects

H Netz

Publications and source records attributed to H Netz.

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

[Tilt-table-testing in childhood: non-invasive, continuous hemodynamic monitoring].

BACKGROUND: Approximately 15% of children experience a syncope before the age of 18 years. Tilt table testing represents the diagnostic gold standard whenever a neurocardiogenic spell is suspected. Two methods of continuous, non-invasive hemodynamic monitoring during the tilt table test are presented and their usefulness for clinical routine is discussed. PATIENTS: 4 patients with suspected neurocardiogenic syncope. METHODS: Tilt table testing according to standard protocol; non-invasive, continuous blood-pressure measurement by finger cuffs; impedance cardiography for the assessment of cardiac output. RESULTS: In 3 patients a syncope occured during the tilt table test. In pt. 4 characteristic symptoms led to diagnosis prior to syncope. Every patient represents a definite type of neurocardiogenic syncope (cardioinhibitory with asystolia, vasodepressory, mixed as well as postural tachycardia syndrome). CONCLUSIONS: Both monitoringmethods allow a differentiated analysis of the cardiovascular interactions during the tilt table test. Thus, specific regulatory patterns may be diagnosed and specific treatment strategies may be offered.

Adolescent↗
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

Young adults born small for gestational age: is reduced baroreceptor sensitivity a risk factor for hypertension?

UNLABELLED: BACKGROUND. Adults born small for gestational age (SGA) are at increased risk for the metabolic syndrome and cardiovascular disease. HYPOTHESIS: Impaired short-term blood pressure regulation may contribute to the development of hypertension in patients born SGA. METHODS: In all, 43 patients born SGA (18 female, age 19.4 +/- 0.3 years) were evaluated by beat-to-beat blood pressure and heart rate registration during rest and mental and orthostatic stress. The study group was divided into Group 1 with normal resting blood pressure (n=32) and Group 2 with slightly elevated blood pressure (n=11). Baroreceptor sensitivity (BRS) was calculated. Fasting insulin as well as lipid levels were correlated with hemodynamic parameters. RESULTS: Eleven of the 43 study patients (25%) had a slightly elevated resting systolic blood pressure (SBP) rising during mental and orthostatic stress. Body mass index (BMI) and fasting insulin levels correlated strongly with SBP in Group 2. Baroreceptor sensitivity was lower in Group 2 at rest (p < 0.05). CONCLUSIONS: Three components of metabolic syndrome (elevated BP, high BMI, elevated insulin levels) correlate strongly in young adolescents born SGA; BRS is reduced in prehypertensive patients. Close follow-up is warranted during adult life as they are predisposed for developing a metabolic syndrome with elevated cardiovascular risk.

Female↗
PubMed2006

[Quantification of right ventricular function in congenital heart disease: correlation of 3D echocardiography and MRI as complementary methods].

PURPOSE: In congenital heart disease, the exact determination of the right ventricular function is of high importance for therapeutic and especially surgical planning. The aim of this study was to correlate the parameters of the right ventricular function in MRI and 3D echocardiography to determine the agreement of both modalities. MATERIALS AND METHODS: In 18 patients suffering from congenital heart disease, 3D echocardiography was performed using a Philips Sonos 7500 system. In MRI short axis slices with a 4-mm distance were acquired using an SSFP sequence on a Siemens Sonata or Symphony System. Volumetry for both modalities was performed on an external workstation (Tomtec) using the EchoView software. RESULTS: Enddiastolic and endsystolic volumes showed a highly significant correlation with coefficients of 0.996 and 0.990, respectively. In echocardiography there was a systematic slight underestimation of enddiastolic volumes and overestimation of endsystolic volumes. The Wilcoxon test did not show significant differences between the volumes and ejection fractions assessed by both modalities. CONCLUSION: There is an excellent correlation in the quantification of right ventricular volumes in MRI and 3D echocardiography, which allows a comparison of acquired volumes in clinical follow-up.

Adolescent↗
PubMed2005

Impedance cardiography and beat-to-beat blood pressure monitoring in diagnosis of syncope in long-QT syndrome.

Syncope represents a diagnostic challenge in patients affected by long-QT syndrome (LQTS). It is crucial for the therapeutic decision to distinguish between potentially life-threatening ventricular tachycardias (Torsadede-pointes) and-especially during adolescence-neurocardiogenic syncopes. This case report presents a patient with LQTS2 (mutation in the HERG gene) on medication with beta-blocker, in whom a head-up-tilt table test was performed after syncope of unknown origin. The test was chosen in order to reproduce the circumstances under which the syncope had happened. The monitoring device consisted of impedance cardiography as well as non-invasive beat-to-beat blood-pressure measurement. By these means it was possible to register a reduced peripheral vascular resistance after tilting the patient, a reduced cardiac output and bradycardia leading to syncope after four minutes of upright posture. This was suggestive for neurocardiogenic syncope as a cause for the spell experienced. Further non-invasive diagnostic methods were normal. As the patient's family history was negative for syncopes, Torsade-de-pointes seemed unlikely.In this special case the non-invasive monitoring system of cardiac output, peripheral vascular resistance and beat-to-beat blood pressure measurements was useful as a supplemental tool during evaluation of syncope and helpful in decision-making against implantation of an ICD and for a more intense treatment with betablockers. Such monitoring devices offer new insights into the orthostatic regulation in young patients.

Blood Pressure Determination↗
PubMed2005

Heart failure in pediatric patients.

Paradigms in understanding heart failure have changed as the knowledge of pathophysiology and its molecular basis has deepened. In the chronic compensated state of heart failure recent research has focussed on the body's regulatory mechanisms. Today heart failure is understood as a complex interplay of hemodynamic and neurohormonal factors. In pediatrics a large variety of heterogeneous conditions cause heart failure. Some require special therapeutic approaches such as the infusion of prostaglandin for ductal patency, the careful maintenance of balance between systemic and pulmonary circulations or operative treatment. In newborns with critical congenital lesions and in patients in the postoperative period management of acute heart failure becomes important. Chronic heart failure as it is understood today is present in patients with cardiomyopathies and in an increasing number of pediatric patients after palliative surgery. In heart failure due to left-to-right shunts a similar activation of compensatory neurohormonal mechanisms as in adults with chronic heart failure was found. In small clinical trials treatment of these activated compensatory mechanisms with angiotensin-converting enzyme inhibitors and beta-blockers showed beneficial effects in pediatric patients. However large clinical multicenter trials as performed in the adult population should be conducted.

Adrenergic beta-Antagonists↗
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

[15-year-old patient with decompensated heart failure and tachycardia with negative P-waves in inferior ECG recordings. An indication for heart transplantation?].

A 15-year-old girl was admitted with signs of severe cardiac failure. There were no symptoms of cardiac insufficiency 4 weeks before hospital admission. She presented with permanent supraventricular tachycardia with negative P-waves in leads II, III and aVF, the heart rate was 150 beats per minute. The electrophysiological examination showed a permanent junctional reentry tachycardia. A postero-septal accessory pathway could be eliminated successfully by radiofrequency catheter ablation. Immediately after the procedure cardiac function deteriorated with slight decrease of the strongly reduced cardiac output. Intensive care and application of dobutamine led to clinical stability. During a follow-up of two years the young patient showed permanent sinus rhythm and an age related physical strain. This case report documents the rapid and severe manifestation of cardiac failure owing to permanent junctional reentry tachycardia in a 15-year-old girl. She was referred for consideration of heart transplantation. Invasive electrophysiological treatment led to permanent sinus rhythm with improvement of left ventricular function.

Adolescent↗
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

Prenatal diagnosis of congenital heart disease and neonatal outcome--a six years experience.

BACKGROUND: Prenatal diagnosis of congenital heart disease (CHD) is important for proper perinatal and neonatal management, as CHD occurs in approximately eight of 1000 live births. Antenatal detection of cardiac malformations is crucial as altered obstetric and neonatal management may be required and the neonate will benefit from delivery at a tertiary care center. METHODS: A retrospective comparison between pre- and postnatal diagnoses was accomplished. All fetal echocardiographic scans performed and CHD's detected postnatally between January 1994 and December 1999 by the department of obstetrics and gynecology and pediatric cardiology - Grosshadern, Munich University Hospital were reviewed. RESULTS: The incidence of CHD was 1.5% (116/ 7913). CHD was suspected by prenatal ultrasound in 57 fetuses. Postnatally, 116 congenital cardiac anomalies were diagnosed. Overall, the intrauterine detection rate was 38% (44/116). 72% (84) of the diagnosed anomalies were Septal Defects, 16% (19) disorders of the Atrioventricular Valves and 11% (13) Anomalies of the Great Vessels. In 10% (11) of fetuses chromosomal abnormalities were found. 8% (9) newborns suffered from congenital syndromes. In 55% (64) children were delivered preterm (<37 weeks of gestation). 5% (6) of the neonates required surgery within their first year of life. 28% (33) of children with CHD did not survive the first year of life. CONCLUSION: As the prenatal diagnosis is important for pre-, peri- and postnatal management, diagnostic accuracy is essential. Nevertheless, the accuracy of postnatal pediatric echo still exceeds that of the prenatal scan.

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

Long-term results of total repair of tetralogy of Fallot in adulthood: 35 years follow-up in 104 patients corrected at the age of 18 or older.

Long-term survival after surgical repair of tetralogy of Fallot (TOF) is reported to be excellent if the patients are corrected in childhood. However, age at operation has been demonstrated as an important risk-factor. The aim of our study was to investigate whether adult patients also benefit from surgery. From December 1958 to May 1977, 739 patients underwent a correction of their TOF with pulmonary stenosis at our institution. Foreigners (n = 52) and those who moved to a foreign country (n = 13) were excluded from further analysis. Sixteen patients were lost during follow-up (98% complete). Of the remaining patient population (n = 658; mean age: 12.2 +/- 8.6 years; range 2-67 years), 104 patients were 18 years or older at the time of correction. Operative (n = 25) and one-year (n = 8) deaths were excluded for long-term calculations, resulting in a study group of 71 patients. Actuarial 10, 20, 30, and 35-year survival rates were 94%, 93%, 83%, and 72% respectively, and not different from normal life expectancy. The most common cause of death was congestive heart failure (n = 3), followed by myocardial infarction (n = 2) and sudden death (n = 2). Parameters influencing longterm survival could not be detected. At follow-up (mean 27.7 years), more than 80% (n = 48) of the 58 survivors reported themselves to be in NYHA functional class I or II and 95% (n = 55) were in a better condition than before the operation. Repair of tetralogy of Fallot in adulthood shows excellent results with normal life expectancy for the patients.

Adult↗
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↗