Microdeletion 22q11 in complex cardiovascular malformations.

Besides DiGeorge, velocardiofacial and conotruncal anomaly face syndromes, some of the isolated congenital heart diseases have also been associated with a chromosomal deletion in 22q11. These disease entities, which had originally been considered to have a different genetic background, are now included in the CATCH-22 microdeletion complex. CATCH 22 is an acronym for cardiac defect, abnormal facies, thymic hypoplasia or aplasia and T-cell deficiency, cleft palate, hypoparathyroidism, and hypocalcemia. In the present study, we focused on the complex cardiovascular defects (CCVD) and screened 40 patients for a microdeletion of 22q11 by fluorescence in situ hybridization using the D22S75 DNA p…

Metabolische Alkalose nach herzchirurgischen Eingriffen im Kindesalter

Effects of Tolazoline and Prostacyclin on Pulmonary Hypertension in Infants After Cardiac Surgery

Objective To evaluate the hemodynamic effects of tolazoline and prostacyclin in infants with pulmonary vasospasm after cardiac surgery. Design Prospective cohort study. Setting Pediatric ICU. Patients The cohort consisted of 42 infants and children with congenital heart disease and pulmonary hypertension who underwent corrective surgery and were monitored postoperatively using pulmonary artery catheters. Fourteen infants (2 to 12 months old) in this group required postoperative treatment with tolazoline or prostacyclin. Interventions Tolazoline was administered as a bolus of 0.5 mg/kg for treatment of persistent pulmonary hypertension or acute pulmonary hypertensive crisis. If its effective…

Mitral and aortic regurgitation in 84 patients with mucopolysaccharidoses

In echocardiographic and necropsy studies nodular thickening of the mitral valve and, less frequently, of the aortic valve has been found in 60%-90% of patients with mucopolysaccharidoses (MPS). Little is known about the haemodynamic consequences of these morphological changes. In this study 84 unselected patients with different enzymatically proven MPS and 84 age and sex matched, healthy persons were studied prospectively by colour Doppler flow mapping. The patients' age ranged from 1 to 47 years (median 8.1 years). Mitral and aortic regurgitation were defined as a holosystolic or holodiastolic jet originating from the valve into the left atrium or the left ventricular outflow tract, respe…

Nitric oxide and prostacyclin lower suprasystemic pulmonary hypertension after cardiopulmonary bypass

In a 3-week-old male newborn persistent suprasystemic pulmonary hypertension developed after surgical valvulotomy for a critical aortic valve stenosis. Because of a residual transvalvular pressure gradient of 35 mmHg and postoperative left as well as right ventricular dysfunction, treatment with inhaled nitric oxide (NO) and intravenously infused prostacyclin (PGI2) was attempted. Low-dose inhaled NO and low dose PGI2 corrected severe pulmonary hypertension and led to an increase in cardiac output. Treatment with NO but not PGI2 was accompanied by a rise in PaO2 and systemic blood pressure. Interruption of NO administration led to a rapid increase in pulmonary arterial pressure to suprasyst…

Independent right lung high frequency and left lung conventional ventilation in the management of severe air leak during ARDS

Summary Pulmonary air leaks are one of the most common problems in patients with the adult respiratory distress syndrome, ARDS. We report what we believe to be the first case in which unilateral high-frequency ventilation combined with contralateral conventional positive pressure ventilation has been used successfully to manage severe air leak in an infant with ARDS.

Transcatheter recanalisation and stenting of a closed ductus arteriosus in duct dependent lung perfusion

In patients with the congenital cardiac malformation of tetralogy of Fallot, occasionally one pulmonary artery, usually the left, seems angiographically to be absent.1 This pulmonary artery is usually present, but discontinuous with the pulmonary trunk, having originally been supplied by a patent arterial duct. With closure of the duct, the receiving flow to that pulmonary artery is by small collateral vessels, which leads to reduced growth of the involved pulmonary vessels and impedes definite surgical repair. We report a case of a 2 day old, 1890 g, premature, cyanotic boy (oxygen saturation 82%) with tetralogy of Fallot, right sided aortic arch, and discontinuity between the pulmonary tr…

Continuous measurement of cardiac output by the Fick principle in infants and children: comparison with the thermodilution method.

To compare a system that continuously monitors cardiac output by the Fick principle with measurements by the thermodilution technique in pediatric patients.Prospective direct comparison of the above two techniques.Pediatric intensive care unit of a university hospital.25 infants and children, aged 1 week to 17 years (median 10 months), who had undergone open heart surgery were studied. Only patients without an endotracheal tube leak and without a residual shunt were included.The system based on the Fick principle uses measurements of oxygen consumption taken by a metabolic monitor and of arterial and mixed venous oxygen saturation taken by pulse- and fiberoptic oximetry to calculate cardiac…

Analgosedierung mit Fentanyl/Midazolam nach Korrektur angeborener Herzfehler

Abstract There is no standard therapy in the management of postoperative pain control following corrective cardiac surgery of congenital heart disease. Assessment in the preverbal age is difficult. In a randomized study we compared a combined treatment of fentanyl and midazolam, given as continuous infusion versus single dose application. A pain assessment score was used to measure the effectiveness of analgosedation in addition to recording nurseries observations. Fentanyl and midazolam are an appropriate combination for postoperative pain treatment. Continuous application is considered to be more effective concerning basic anxiety, cumulative dosage and to avoid volume overload in infants…

Normal values of M mode echocardiographic measurements of more than 2000 healthy infants and children in central Europe

OBJECTIVE—To obtain normal M mode (one dimensional) echocardiographic values in a substantial sample of normal infants and children.
DESIGN—Data were obtained over three years from a single centre in central Europe.
PATIENTS—2036 healthy infants and children aged one day to 18 years.
METHODS—In line with recommendations for standardising measurements from M mode echocardiograms, and using digital echocardiographic equipment, measurements were obtained of the following: right ventricular anterior wall thickness at end diastole, right ventricular end diastolic dimension, thickness of interventricular septum at end diastole and end systole, thickness of posterior wall of the left ventricle at …