Recurrent noisy pneumothorax mimicking pericarditis.

Sounds related to the cardiac cycle may have an extra-cardiac origin. We report a case of recurrent noisy pneumothorax producing a loud rub, audible at a distance from the patient and initially diagnosed as pericarditis. The sounds and their mechanisms of production in this condition are also discussed.

Pulmonary vein stenosis mimicking chronic lung disease.

UNLABELLED: The presence of recurrent respiratory symptoms and right heart enlargement in an ex-premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography. CONCLUSION: This report describes a patient with pulmonary vein stenosis in whom the diagnosis was delayed as she had symptoms and signs more commonly associated with chronic lung disease.

Transoesophageal echocardiographic assessment of fixed subaortic obstruction in children.

OBJECTIVE: To evaluate the clinical usefulness of transoesophageal echocardiography in the assessment of children with fixed left ventricular outflow tract stenosis. PATIENTS AND METHODS: Eight consecutive children, aged over 5 years, with fixed subaortic stenosis and one child with fixed subpulmonary left ventricular outflow tract stenosis were prospectively assessed by precordial and transoesophageal echocardiography. RESULTS: Transoesophageal images of the left ventricular outflow tract were much clearer than precordial images in all patients except one with a prosthetic mitral valve. Improved visualisation provided further information on the nature of the lesion (additional chordal attachment of the mitral valve in one, accessory atrioventricular valve tissue with aneurysm formation in one), on the extent of the lesion (circumferential in three), and on the very close relation of a ridge to the aortic valve leaflets in one. Transoesophageal Doppler did not provide any additional information on aortic regurgitation and was unreliable for gradient estimation across the left ventricular outflow tract. CONCLUSIONS: Transoesophageal imaging provides an excellent means of visualising lesions in the left ventricular outflow tract and can be useful in a few children and adolescents in whom precordial echocardiography does not provide adequate information. The technique can also be used intraoperatively to define the full extent of the obstructive lesion and to assess residual lesions after surgery.

Influence of colour Doppler echocardiography on the ultrasonic assessment of congenital heart disease: a prospective study.

OBJECTIVE: To evaluate the additional information provided by colour Doppler in the ultrasonic assessment of congenital heart disease. PATIENTS AND METHODS: A prospective study of 215 children (age range 1 day-16 years) presenting with clinical signs of congenital heart disease. RESULTS: Colour Doppler was essential for the diagnosis of an anomalous left coronary artery and altered the management of a patient initially diagnosed as having cardiomyopathy. Colour Doppler provided extra information, but without major impact on management, in the following: the diagnosis of ventricular septal defects associated with other defects, of multiple ventricular septal defects, of anomalous pulmonary venous drainage, and of mild mitral regurgitation; the demonstration of site of coarctation, of stenotic or hypoplastic pulmonary artery branches, of unobstructed flow through a right atrial membrane, and of left ventricle to right atrium regurgitation; the assessment of the width of the duct and of flow through the patent foramen ovale in transposition and tricuspid atresia; the differentiation of pulmonary atresia from critical pulmonary stenosis and the measurement of maximum velocity of tricuspid regurgitation. CONCLUSIONS: Ideally all patients should undergo colour Doppler studies before cardiac surgery to ensure a more accurate diagnosis. However, since the additional information provided does not affect the management in most patients, machines without colour Doppler can provide a satisfactory service in paediatric cardiology centres in countries where resources are limited.

Double aortic arch, tetralogy of Fallot with pulmonary atresia and atrioventricular septal defect.

A double aortic arch is usually an isolated abnormality. We describe a case with a previously undescribed combination of tetralogy of Fallot with pulmonary atresia, complete atrioventricular septal defect, and left patent arterial duct in association with a double aortic arch. A complete diagnosis was made by echocardiography. Meticulous suprasternal echocardiography must be employed to avoid overlooking an unsuspected aortic arch abnormality.

Transoesophageal echocardiographic assessment of primum, secundum and sinus venosus atrial septal defects.

We compared the ability of transthoracic and transoesophageal echocardiography to determine the presence and site of an atrial septal defect and associated anomalous pulmonary venous connexions in 13 school age children (aged 5 to 15 years) and 12 adults (aged 25 to 68 years). Transthoracic echocardiography detected atrial septal defects in 12 children and 6 adults. Transoesophageal echocardiography confirmed the position of 16 (13 secundum, 3 primum) of these 18 defects but altered the diagnosis from a secundum defect to a sinus venosus defect in one and from a sinus venosus defect to a high secundum defect in another. In addition to these 18, transoesophageal echocardiography diagnosed a defect in 5 adults (3 secundum and 2 sinus venosus defects) and 1 child (secundum defect). In an adult with inconclusive transthoracic findings, transoesophageal echocardiography enabled clear visualisation of the atrial septum and excluded an atrial septal defect. Transoesophageal echocardiography showed anomalous attachment of a pulmonary vein into the region of a sinus venosus defect (n = 3) but did not show anomalous connexions to the superior caval vein (n = 3) or the inferior caval vein (n = 1). Transoesophageal echocardiography provides a reliable method of diagnosing or excluding an atrial septal defect in patients with inconclusive transthoracic findings and is of particular diagnostic value in sinus venosus defects.

Diagnosis of mitral valve aneurysm by transoesophageal echocardiography.

In a patient with mitral valve aneurysm precordial echocardiography suggested a mistaken diagnosis of infective endocarditis. Transoesophageal echocardiographic examination established the correct diagnosis, which was subsequently confirmed at operation. Transoesophageal echocardiography gives better resolution of lesions associated with the mitral valve than precordial examination and may improve the diagnostic accuracy.

Balloon atrial septostomy under echocardiographic control: six years' experience and evaluation of the practicability of cannulation via the umbilical vein.

Balloon atrial septostomy was undertaken under cross sectional echocardiographic control in 63 consecutive infants: in no case was fluoroscopic imaging required. The procedure was performed in the cardiac catheterisation laboratory, ward side room, or at the bedside in the neonatal intensive care unit. Catheterisation via the umbilical vein was attempted in 37 infants aged less than 48 hours old and was successful in 27. No complication was clearly attributable to the procedure though two infants died. A nine day old child died from disseminated intravascular coagulation the day after septostomy by the iliofemoral route and another, aged nine days, died of necrotising enterocolitis which had developed when he was eight days old, after umbilical catheterisation at eight hours. Balloon atrial septostomy is a safe and easy procedure under cross sectional echocardiographic imaging control. Catheterisation via the umbilical vein was safe, easy to perform, and is appropriate in infants aged less than 48 hours.

Doppler ultrasound and the silent ductus arteriosus.

A clinically undetectable, small ductus arteriosus was identified by Doppler ultrasonography in 21 individuals. Infants were excluded from the study and no patient had pulmonary hypertension. Persistence of the ductus arteriosus is likely to be more common than shown by less sensitive diagnostic methods. Some patients considered to have infective endocarditis with a normal heart may have a silent ductus arteriosus. Evidence of such an association would justify ligation or antibiotic cover as prophylactic measures.

Pulmonary artery sling: definitive diagnosis by colour Doppler flow mapping avoiding cardiac catheterisation.

Pulmonary artery sling is a rare abnormality that usually presents in infancy with wheeze or stridor. Diagnoses by cross sectional echocardiography, computed tomography, and magnetic resonance imaging have been described but pulmonary arteriography was regarded as essential for a definite diagnosis. The use of colour Doppler adds to the diagnostic certainty of cross sectional echocardiography and colour Doppler provided a definite diagnosis in an infant that allowed surgical repair without the need for cardiac catheterisation. Postoperative flow through the left pulmonary artery was also assessed by Doppler, and this avoided the need for repeat catheterisation or perfusion lung scanning.

Congenital fistula between the left ventricle and coronary sinus: elucidation by colour Doppler flow mapping.

A fistula between the left ventricle and the coronary sinus was diagnosed by Doppler echocardiography and confirmed by cardiac catheterisation in a symptom free child who presented with clinical signs of mitral regurgitation. A similar abnormality has been reported after repeated mitral valve replacement and after myocardial infarction but a congenital fistula of this type has not been described before.