Aortic root abscess without involvement of the aortic valve in a child caused by Panton-Valentine leukocidin-positive staphylococci.

Aortic root abscess without any involvement of the aortic valve is extremely rare. This report describes an 11-year-old girl with subperiosteal abscess due to a virulent organism called Panton-Valentine leukocidin-secreting staphylococci. Although her initial transthoracic echocardiogram did not show any cardiac abnormality, she subsequently experienced an aortic root abscess communicating with the left ventricle.

Congenital atresia of the ostium of left main coronary artery: a rare coronary anomaly, diagnostic difficulty and successful surgical revascularization.

We report the case of an 8-month-old infant who was referred for mechanical circulatory support (extracorporeal membrane oxygenation). Aortogram was compatible with the diagnosis of anomalous origin of left coronary artery to pulmonary trunk. A definitive diagnosis of atresia of the left coronary ostium was only established intraoperatively.

Absent pulmonary valve syndrome with intact ventricular septum: role of ductus arteriosus revisited.

We report two patients (four and twelve days old respectively) with heart failure due to absent pulmonary valve and patent ductus arteriosus. The ductus arteriosus in both patients had unusual course arising early from the aortic arch and maintaining an acute angle with the aortic arch. The etiology of absent pulmonary valve syndrome is still not clear.

Holt-Oram syndrome: a clinical genetic study.

A clinical and genetic study of the Holt-Oram syndrome (HOS) has been carried out in the United Kingdom involving 55 cases designated Holt-Oram syndrome, together with their parents and sibs. Data from the clinical assessment of both familial and isolated cases were used to define the HOS phenotype and to outline the spectrum of abnormalities, especially factors affecting severity. Skeletal defects affected the upper limbs exclusively and were bilateral and asymmetrical.

Total one-stage repair of aortopulmonary window and interrupted aortic arch in a neonate.

The congenital anomaly of aortopulmonary window is rare. Its combination with interruption of the aortic arch has been previously recognized and reported in infants and children. Early and complete repair of such a congenital anomaly may prevent progression to cardiac failure and premature death.

Extracorporeal membrane oxygenation and the treatment of critical pulmonary hypertension in congenital heart disease.

Certain forms of congenital heart disease (CHD) confer a high risk for the development of severe pulmonary hypertension before and after corrective cardiac surgery. Extracorporeal membrane oxygenation (ECMO) has theoretical benefits in the treatment of this complication in that it assures oxygenation, corrects acid-base balance and provides haemodynamic support at the same time as allowing lung rest from ventilation. We examined our experience of the 117 children and neonates supported with ECMO between November 1989 and July 1993.

Cardiovascular effects of carbon dioxide in ventilated preterm infants.

Sick preterm infants may, under certain conditions, demonstrate blood pressure passive cerebral blood flow in response to changes in arterial carbon dioxide tension. Blood pressure in turn depends on cardiac output and peripheral resistance. A Doppler technique for assessing cardiac output compared favourably in terms of reproducibility to a thermodilution technique in a group of infants undergoing cardiac catheterization for congenital heart disease.

Airway function in infants with vascular rings: preoperative and postoperative assessment.

Aortic arch anomalies in infancy often cause intrathoracic airway obstruction. Airway function was assessed as part of the diagnostic evaluation in six symptomatic infants both by plethysmography and using a chest compression technique to obtain partial flow-volume loops. Two infants had normal intrathoracic airway function and their symptoms were unrelated to aortic arch abnormalities.

A rare anomaly of the aortic arch complex.

A six-month-old girl presented with heart failure. Cardiac catheterisation showed a ventricular septal defect and an anomaly of the aortic arch complex, which is a challenge to embryologic theory.

Acute infective pericarditis in infancy.

Infective pericarditis is rare; the characteristic presenting illness in 3 children, each aged under one year, is recorded. Early diagnosis is important since surgical drainage of the pericardium is often necessary in addition to antibiotic therapy. The clinical features may suggest pericarditis, but diagnosis is readily and reliably confirmed by echocardiography.

Balloon atrial septostomy under two dimensional echocardiographic control.

Two dimensional echocardiography has been used to guide balloon atrial septostomy in five cases of transposition of the great arteries. A single lumen balloon catheter was used. The catheter can be accurately located within the left atrium before inflation of the balloon, thus avoiding some of the traumatic accidents possible during septostomy.

Surgical management of coarctation of aorta with ventricular septal defect. Multivariate analysis.

Optimal management of coarctation with ventricular septal defect is difficult. Should one treat the coarctation, the ventricular septal defect, or both? This dilemma was investigated by reviewing 39 successive patients, aged less than 4 months, undergoing coarctation repair without pulmonary artery banding. Twelve hospital deaths occurred.

Factors influencing survival after balloon atrial septostomy for complete transposition of great arteries.

Despite balloon atrial septostomy within the first days of life, some patients with complete transposition of the great arteries die before reaching elective definitive surgery in the second six months of life. To discover why, we analysed the fate of 144 patients who had balloon atrial septostomy after 1966, using a modified logrank survival test with multivariate capability. Patients were withdrawn "alive" on reaching definitive surgery.

Use of high kilovoltage filtered beam radiographs for detection of bronchial situs in infants and young children.

Determination of atrial situs is of cardinal importance in the analysis of complex congenital heart lesions, and is best predicted from bronchial situs. Previous methods for assessing bronchial morphology, however, are unsuited to the very young patient. To assess bronchial morphology, 100 consecutive patients under 18 months of age (medium 57.

Long-term comparison of metoprolol and methyldopa in the treatment of hypertension.

The effect of the cardioselective beta-adrenoreceptor blocking compound, metoprolol, was compared with methyldopa in the long-term management of hypertension. Thirty patients given metoprolol and twenty-six given methyldopa were treated for 2 years. The maximum dose of metoprolol was 200 mg twice daily (average 308 mg) and of methyldopa 1,000 mg twice daily (average 1,120 mg).

Late results of surgical treatment of transposition of the great arteries.

In summary, many of these patients have achieved a normal working capacity, but as a group there is a statistically significant reduction when compared to healthy children (p < 0.01). There was no significant difference between the group of patients who had their Mustard operation in infancy and later.

Aortic arch interruption presenting with absence of all limb pulses.

Three patients with aortic arch anomalies presented with severe heart failure, acidaemia, and poor or absent upper and lower limb pulses. Prominent carotid arterial pulsations were detected which distinguished the condition clinically from aortic atresia. In 2 patients with a left aortic arch, interruption was distal to the left common carotid artery and there was an aberrant right subclavian.