Anomalous pulmonary venous connections were found in seven (0-6%) of 1183 patients with tetralogy of Fallot. Three patients had totally anomalous connections (one supracardiac, one direct to coronary sinus, and one mixed supracardiac and infracardiac) and four patients had partially anomalous pulmonary venous connections. All patients presented with the clinical features of tetralogy of Fallot. Anomalous pulmonary venous drainage was suspected clinically in only one patient in whom there was a scimitar sign on the chest radiograph. The exact diagnosis was established by cross sectional echocardiography (one), preoperative or postoperative angiography (five), or at necropsy (one). Surgery was performed in six patients. Total correction without re-routing of the anomalously draining veins was successful in all those with partially anomalous connections, with no significant long term sequelae (follow up median 17 years). Of those with totally anomalous connections, the full diagnosis was made only at necropsy in one patient, successful one-stage correction was performed in one, and the other patient, who had partially obstructed mixed drainage, died shortly after one-stage correction. Histological examination of the lung biopsy specimen in this patient showed grade 2 pulmonary vascular disease.Tetralogy of Fallot with anomalous pulmonary venous connections is a rare association. Careful preoperative assessment is required in those with totally anomalous connections. Patients and methodsWe reviewed the records of 1183 patients with a diagnosis of tetralogy of Fallot investigated at the Brompton and National Heart Hospitals in the past 20 years. Only patients with atrial situs solitus were reviewed; we excluded those with isomerism of the atrial appendages.Seven patients (0.6%) had abnormalities of pulmonary venous connection. Their ages at presentation ranged from three days to four months. In only one was the complete diagnosis made by cross sectional echocardiography. In the remainder the diagnosis was made either at the time of cardiac catheterisation (four patients), corrective surgery and later cardiac catheterisation (one), or at necropsy (one). The table gives Case reports

We have used flexible fibreoptic bronchoscopy using sedation and local anaesthesia in 50 children aged 2-19 years (median 10) using an Olympus BFP20 instrument. Indications were opportunistic pneumonias (n=11), persistent atelectasis (n=11), recurrent pneumonia (n=7), misceilaneous lower airway disease (n=7), recurrent wheezing (n=3), haemoptysis (3), to diagnose infection or rejection of heart-lung transplants (n=3), stridor (n=2), suspected airway compression (n=l), evaluation of tracheostomy (n=l), and suspected foreign body (n=l). In 43 cases (86%) the diagnosis was related to the primary indication. In five (10%) unrelated abnormalities were found, and five (10%) were normal. In 13 (26%) treatment was altered as a result of flexible fibreoptic bronchoscopy. Complications were transient respiratory arrest (n=2), hypoxia (n=2), pneumonia (n=2), and laryngospasm (n= 1). AU complications were followed by complete recovery.Our results suggest that flexible fibreoptic bronchoscopy is safe. Advantages over rigid bronchoscopy include greater visual range, fewer complications, and the avoidance of a general anaesthetic. Though invasive it can yield important diagnostic and therapeutic information.

No abstract

Objective-To investigate the feasibility of negative extrathoracic pressure ventilation as a respiratory support following phrenic nerve palsy after cardiac surgery.

Two cases of fatal idiopathic persistent pulmonary hypertension presented late in the neonatal period. Lungs were examined histologically by light and electron microscopy, and immunocytochemical studies were used to identify nerves. There was extension of medial smooth muscle distally along the arterial pathway so that most precapillary arteries had completely muscular walls, which in some cases completely obliterated the vessel lumen. Enlarged endothelial cells also contributed to the reduction in the size of the lumen. Nerve fibres accompanying muscular arteries were found in the alveolar region, more distal than is normal. The predominant neuropeptide was the vasoconstrictor tyrosine. Possible infection. At 2 months of age it was noted that he became cyanosed when crying and feeding. He also had tachypnoea and sweatiness at rest. Arterial blood gas analysis while breathing air showed: pH 7-42, carbon dioxide tension 4-9 kPa, oxygen tension 4-7 kPa, and base excess 0-7. He was referred to the Brompton Hospital for investigation of suspected cyanotic heart disease.On examination his weight had fallen to below the third centile for age. He was cyanosed and there was a prominent right ventricular impulse. The pulmonary component of the second sound was loud but there were no murmurs. His respiratory rate was 46 breaths/ minute. The chest radiograph showed mild cardiomegaly and normal pulmonary vascular markings. The electrocardiogram showed a mean frontal QRS axis of + 150°and right ventricular hypertrophy. The cross sectional echocardiogram showed normal anatomy of the heart with normal atrioventricular and ventriculoarterial connections. The right atrium was enlarged and the interatrial septum bowed from right to left. The right ventricle was grossly dilated with poor systolic function. The parasternal short axis view showed that the left ventricle was 'squashed', suggesting a high right ventricular pressure.Cardiac catheterisation was carried out under general anaesthesia. The oxygen saturation in the aorta was 53%. The right ventricular pressure was 90/0-4 mm Hg with a simultaneous left ventricular pressure of 68/0-5 mm Hg. Using an estimated oxygen consumption of 150 ml/min/ m2 pulmonary vascular resistance was 24 units/ m m air and 10 5 units/i2 in 100% oxygen.Angiography showed poor right ventricular function but no intracardiac abnormalities. A diagnosis of persistent pulmonary hypertension of the newborn was made, and a pulmonary arterial line was left in.The patient was extubated into headbox oxygen. The pulmonary artery pressure at this stage was 156/80 mm Hg with a systemic pressure of 80/30 mm Hg. Prostacyclin and adenosine were infused through the pulmonary arterial line. The patient was placed in a continuous subatmospheric (negative) extrathoracic pressure chamber and given 100% oxygen. None of these manoeuvres succeeded in lowering the pulmonary artery pressure appreciably, and it remained higher than the systemic pressure. On the day after cardiac catheterisation the patient had a card...