Stabilisation of cardiopulmonary function in newborns with congenital diaphragmatic hernia using lung function parameters and hemodynamic management.

OBJECTIVE: Evaluation of lung function parameters and additional use of prostaglandin E1 (PGE1) for the stabilisation of cardiopulmonary function in patients with congenital diaphragmatic hernia (CDH) and pulmonary hypertension (PHT). DESIGN: Observational study. PATIENTS: Between 2007 and 2009 8 patients with CDH have been treated in our pediatric intensive care unit (gestational age 34 + 0 - 40 + 4 weeks, birth weight 2 160-3 840 g). All patients required respiratory support. Gentle mechanical ventilation adapted to the degree of pulmonary hypoplasia based on serially measurements of lung function parameters to find appropriate ventilator settings has been performed. MAIN RESULTS: Functional residual capacity (FRC) and compliance of the respiratory system in all patients were markedly reduced. A FRC between 9.3-10.6 ml/kg and compliance between 1.1-1.8 ml/kPa/kg indicated pronounced hypoplasia of the lungs. Doppler flow patterns through the arterial duct were classified into left-to-right, right-to-left and bidirectional shunting and correlated to the degree of PHT. The additional use of PGE1 to reopen the arterial duct and to stabilize right ventricular function led to an amelioration of severe PHT and preoperative stabilisation in 2 newborns with pronounced pulmonary hypoplasia. All patients underwent successful surgery, and did not show any complications after 2 years follow-up. CONCLUSION: Measurements of lung function parameters and adaptation of mechanical ventilation to the degree of pulmonary hypoplasia and additional therapy with PGE1 may help to improve the outcome in CDH patients.

Cardiac troponin I release after transcatheter atrial septal defect closure depends on occluder size but not on patient's age.

OBJECTIVE: To examine whether transcatheter closure of secundum atrial septal defect (ASD) with the Amplatzer septal occluder leads to more myocardial injury in children than in adults. DESIGN: In a prospective study with children and adults cardiac troponin I (cTnI) serum concentrations were determined by immunoassay (AxSYM, Abbott Laboratories) before, during, and up to 20 months after surgical or transcatheter ASD closure. PATIENTS: Four groups of patients were studied: transcatheter ASD closure (group 1: 22 children, age range 3.26-14.7 years; group 2: 22 adults, 18.0-67.3 years), surgical ASD closure (group 3: 18 children, 3.12-13.5 years), and diagnostic catheterisation (group 4: 12 children, 2.68-15.0 years). RESULTS: cTnI concentrations were significantly increased after occluder implantation with higher serum concentrations in children than in adults (immediately after implantation: group 1, 3.2 (4.4) microg/l; group 2, 1.1 (4.2) microg/l; four hours after implantation: group 1, 4.8 (5.0) microg/l; group 2, 1.7 (2.3) microg/l; both p < 0.01, group 1 v group 2; one day after implantation: group 1, 3.0 (5.7) microg/l; group 2, 2.2 (5.2) microg/l) but were less than 20% of those after surgical ASD closure (group 3; p < 0.001) where the highest cTnI concentration was found (37.1 (26.3) microg/l). Diagnostic catheterisation (group 4) was not associated with detectable cTnI increase. From the cTnI concentrations the total amount of cTnI released after ASD closure was estimated for each patient. This was dependent on the size of the occluder (p < 0.05) but not on the patient's age or procedural duration. CONCLUSION: In regard to interventional ASD closure our data do not provide evidence that the child's myocardium is more vulnerable. Transcatheter ASD closure induces minor myocardial lesion, the extent of which depends on the size of the Amplatzer septal occluder but is irrespective of the patient's age.

Partial trisomy 22 resulting from rearrangements between chromosomes 11/22 and 16/22: a report of two cases.

UNLABELLED: Two male infants with partial trisomy 22 resulting from a rearrangement between chromosomes 11/22 and 16/22 were admitted to the Children's Hospital of the University of Leipzig within the space of two months. The characteristic phenotype of the infants is described and compared with the data on liveborn infants with trisomy 22, as reported in the literature. One of the infants reported here showed a prenatally detected hygroma colli. To the best of our knowledge this is the first description of a hygroma colli in this chromosomal disorder. CONCLUSION: Infants with trisomy 22 can present with variable phenotypes. It is important to bear the phenotype of chromosome 22 infants in mind.

[Idiopathic left ventricular tachycardia in childhood: typical clinical findings and different therapeutic options]. / Idiopathische linksventrikuläre Tachykardie im Kindesalter: typische Befunde und verschiedene Therapieoptionen.

We report 3 adolescents with structurally normal heart who were referred to hospital due to long-lasting palpitations. Initial 12-lead-ECG showed sustained, monomorphic ventricular tachycardia, right bundle branch block QRS morphology and axis deviation. After failure of different anti-arrhythmic drugs finally the intravenous medication with verapamil led to termination of ventricular tachycardia in all three patients. All clinical findings and the responsiveness to verapamil are consistent with the diagnosis of idiopathic left ventricular tachycardia. In one patient an electrophysiological study was done and increased left ventricular vulnerability was shown. After inducing a tachycardia originating from the left ventricle radiofrequency catheter ablation of the left-posterior fascicle was successfully performed. The tachycardia was not inducible after this ablation. Since that investigation the patient has been asymptomatic without anti-arrhythmic treatment. Two of three patients have been on oral verapamil prophylactically and have been free of symptoms.

[Echocardiographic online quantification of left ventricular systolic function in children: comparison with conventional off-line determination]. / Echokardiographische On-line-Quantifizierung der linksventrikulären systolischen Funktion bei Kindern: Vergleich mit der konventionellen off-line-Bestimmung.

Accurate and efficient echocardiographic on-line determination of left ventricular volume would be advantageous in the care of children with congenital heart disease and children with hemodynamic instability. The prospective study was performed to evaluate the clinical usefulness of the on-line automatic border detection system (acoustic quantification: AQ) for determination of left ventricular volumes and ejection fraction in comparison to the conventional off-line method (manual tracing). 107 patients were enrolled in the study. The ages ranged from 0.1 to 18.8 years (mean 8.3 +/- 5.6). All patients were studied in the apical four-chamber plane for acoustic quantification (AQ) and manual tracing as well. Left ventricular volumes were determined using the mono-plane Simpson's rule. Left ventricular end-diastolic volumes obtained by AQ correlated well but were slightly underestimated compared to those determined by manual tracing (r = 0.99). Left ventricular endsystolic volumes by AQ correlated well but were also slightly underestimated compared to those obtained by manual tracing (r = 0.98). Mean ejection fraction was 61.1 +/- 6.8% by AQ compared with 61.5 +/- 5.9% by manual tracing. Linear regression analysis demonstrated good correlation: y = 0.77x + 14.1, r = 0.89; p < 0.001. Measurement of left ventricular volumes and ejection fraction by AQ using automatic border detection compares well with measurements done by manual tracing. However, AQ tends to underestimate to some degree. The time necessary for acquisition of data was similar in both methods. AQ seems to be a promising method for real-time estimation of left ventricular volume, even in children.

A report on pathological changes of the dipole moment in children suffering from anthracycline cardiomyopathy.

Cardiomyopathy is a severe complication of the tomour therapy with anthracyclines. Since even minor disturbances of myocardial cell membranes could influence the dipole moment of the heart the noninvasive measurements of this parameter might be useful, particularly in the paediatric population. 17 children aged 2-15 years treated clinically for various malign blood diseases were examined repeatedly with a modified Nelson lead-system up to 3 years. The dipole moment was evaluated by visual comparison of the calculated horizontal and frontal VCGs, evaluation of 3 distinctive vectors, as well as of the magnitude curves and the velocity curves, all of them compared with the normal age dependent percentiles of each respective child. Conventional 12-lead ECGs were used to confirm rhythm disturbances and alterations of P-, PQ-, QRS-, and QT-durations. Our results show that damages of the heart are different during the time of the drug administration, then consisting of acute toxic reactions such as sudden dilatation and/or rhythm disturbances, and of long-term disturbances leading to growth retardation of the heart with the danger of chronic congestive heart failure months or years after the end of the anthracycline treatment. Morphological and biochemical damages of myofibrils caused by the toxicity of anthracyclines precede functional restraints of the heart. A noninvasive method for an early and reliable diagnosis of these damages is urgently needed, particularly for children. Measurements of the dipole moment with the Nelson-lead system seem to offer this diagnostic tool which aims possible changes of the drug administration protocol. (Fig. 3, Ref. 15.)

[Echocardiography determination of shunt volume in children with atrial septal defect]. / Echokardiographische Bestimmung der Shuntgrösse bei Kindern mit Vorhofseptumdefekt.

The accuracy of echocardiographic estimation of left-to-right shunts was studied prospectively in children with secundum ASD. Fifty-one consecutive patients were examined from March 1987 to August 1991. Mean age was 64/12 years with a minimum age of 12/12 and a maximum of 131/12 years. Twenty-five children were included in the first part of the study. The ratios of right and left atrial and ventricular areas and pulmonary and aortic diameters were correlated with the left-to-right shunts determined by oximetry (mean shunt 48.7%; min. 10.1%, max. 73.2%). The pulmonary/aortic diameter ratio provided the closest correlation (r = 0.95; p < 0.01). During the second part of the study the left-to-right shunt was estimated using the regression equations developed from part one. Thus, in 26 subsequent patients these values were compared with oximetric shunt values. Again, the pulmonary/aortic diameter ratio provided the closest correlation (r = 0.97). The pair comparison yielded a difference of 0.7 +/- 3.8%. Based on these results since 1991, surgery for ASD II has been performed without preoperative cardiac catheterization in 33 patients, provided echocardiographic shunt estimation was > or = 48%.

[Quantitative Doppler echocardiography diagnosis of congenital aortic and pulmonary artery stenoses]. / Quantitative Doppler-echokardiographische Diagnostik von angeborenen Aorten- und Pulmonalstenosen.

The aim of the present study was to check the value of cw-Doppler echocardiography for the assessment of severity of congenital aortic and pulmonary stenoses in children and adolescents. Over a period of 3 years 217 children and adolescents with aortic and 98 with pulmonary stenosis, resp., underwent echocardiography. In 21 patients with aortic and in 23 with pulmonary stenosis pressure gradients were determined by cw-Doppler as well as intracardiac pressure measurement. The comparison showed excellent accordance between both sorts of data for aortic stenoses (r = 0.939) and pulmonary stenosis (r = 0.969). These results show that Doppler echocardiography is a reliable diagnostic method for the determination of pressure gradients. Thus, it allows correct selection of children and adolescents with aortic stenosis for surgery. So far, 12 patients have been operated on without preoperative cardiac catheterization. Indication for balloon valvuloplasty in patients with valvular pulmonary stenosis can be based safely on 2 D- and Doppler echocardiography.

[Echocardiography diagnosis of persistent ductus arteriosus]. / Echokardiographische Diagnostik des persistierenden Ductus arteriosus (PDA).

Over a period of 2 years PDA was diagnosed in 151 children by means of 2-D-echo combined with Doppler technique. In 47 patients the lesion was also visualized by colour coded Doppler echocardiography. Sixty-seven children had isolated ductus arteriosus, whereas the others had additional cardiac malformations. In 90 patients PDA was confirmed by invasive diagnosis and/or operation or by autopsy. The diagnosis was made on the first echocardiographic examination in all but 2 infants with subaortic ventricular septal defect and pulmonary hypertension. The practical procedure is described in detail. The authors conclude that combined two-dimensional and Doppler echo allow a safe diagnosis of PDA.

Long-term results of anastomotic operations in 120 children with cyanotic congenital heart disease operated on the the first two years of life.

The author analyse results of anastomosis in 120 children with cyanotic heart disease operated on in the first two years of life. At the age up to 3 months of life 21 infants were operated on, 11 (52.4%) of whom died in connection with the procedure. Between the 3rd and 24th month of life 99 children were operated on with a death rate of 11.1%. In 94 children hypoxic spells disappeared, cyanosis and hypoxia were relieved. In the majority of patients, however, the duration of an improved general condition was limited to five years. Both immediate and long-term results depended first of all on the age of the child and on the type of heart lesion. Patients with tetralogy of Fallot had the lowest operative mortality (7.1%) and the best long-term results. In pulmonary atresia, which has poor prognosis without operation, the results of anastomosis were the least gratifying. The authors consider an anastomotic operation, especially the Blalock and Waterston shunt, to be an important life-saving procedure in infants with cyanotic congenital heart disease with hypoxic spells and critical hypoxia.

[Subclavian steal syndrome in childhood (author's transl)]. / Subclavian-Steal-Syndrom im Kindesalter.

The authors report on four children with subclavian steal associated with coarctation or interrupted aortic arch. All children showed a stenotic origin of the left subclavian; in addition three of them had an extreme hypoplasia of the proximal segment of this artery. Two children had typical symptoms of insufficiency of the vertebral and basilar circulation (headache, dizziness, syncopal attacks, vision and hearing disorders) since the age of 7 and 8 years, respectively. The clinical findings, such as reduced pulses and blood pressure of the respective arm, may suggest the provisional diagnosis. The principles of congenital subclavian steal are discussed.