E. Riss

The value of cardiac catheterization and cineangiography in infantile lobar emphysema

Lobar emphysema is an uncommon cause of respiratory distress in infancy. Congenital heart disease is seen in about 20% of the patients with infantile (congenital) lobar emphysema. We described six infants with lobar emphysema. In three of them a congenital heart disease was demonstrated by cardiac catheterization and cineangiography; two had a tetralogy of Fallot with right aortic arch and the third infant a ventricular septal defect. The pulmonary angiography showed stretching of the arteries with very poor filling of the peripheral arteries and a characteristic smaller pulmonary vein in the affected lobe. In all the six patients the pulmonary artery pressure was normal. All the patients underwent lobectomy with good results. We feel that a preoperative cardiac catheterization and cineangiography is of value in this very sick group of infants.

Thyrotoxicosis Induced by Amiodarone, a New Efficient Antiarrhythmic Drug With High Iodine Content

Amiodarone is an antiarrhythmic agent with high iodine content. Ten patients treated with amiodarone developed thyrotoxicosis. I131 uptakes were negligible, and TT3 levels low in relation to TT4 levels, and sometimes even normal.Cessation of amiodarone caused thyroid functions to return to normal in one to five months, unrelated to propylthiouracil treatment. Eight of the patients had normal thyroid glands on radioscan or palpation. All patients tested had normal TRH tests.Thyrotoxicosis is a relatively common complication of amiodarone treatment, probably caused by its high iodine content. It is possible in apparently normal thyroid glands, suggesting failure of the homeostatic mechanisms controlling thyroid synthesis and release in these patients.Amiodarone is very efficient in controlling tachyarrhythmias and angina pectoris, situations in which thyrotoxicosis is dangerous. Thyroid function tests should therefore be drawn periodically, and the complication considered whenever tachyarrhythmias worsen on treatment with amiodarone.

Atrioventricular Block in Situs Ambiguus and Left Isomerism (Polysplenia Syndrome)

Three patients are described who had situs ambiguus and left isomerism (polysplenia syndrome) and advanced atrioventricuiar block. One presented with a complex bradyarrhythmia with Wenckebach block. The other two had congenital atrioventricular block with a narrow QRS at a ventricular rate of 80 per minute, an atrial rate of 150′ per minute, and both had a P wave axis directed superiorly and to the right in one, and superiorly to the left in the other. This ECG pattern was not observed in more than 400 adult patients with complete A‐V block treated in our service. It is our opinion that in infants and children with heart disease the presence of complete A‐V block with narrow QRS and an unusual P waves axis directed superiorly is strongly suggestive of left isomerism. The incidence rate of complete A‐V block in left isomerism is nearly twenty percent of the cases described.

Angiography of azygos continuation of inferior vena cava in situs ambiguus with left isomerism (polysplenia syndrome)

SummaryWe have in our records 11 patients with situs ambiguus and left isomerism (polysplenia). Ten had an interruption of the inferior vena cava (IVC) with azygos continuation; in eight cases the continuation was to the left superior vena cava (LSVC), in one case to the right superior vena cava (RSVC) and in one case to both the LSVC and RSVC. Two patients underwent surgical correction by a baffle procedure of the functioning single atrium. The angiographic confirmation of the azygos system anatomy is important in planning the surgical correction of patients with left isomerism. Clinically, given the same heart defects with normal pressures, the right-sided drainage is probably more favorable, producing less peripheral desaturation.

Single arterial trunk arising from the aortic arch associated with coarctation of the aorta

A 6-day-old baby with a single arterial trunk arising from the aortic arch is reported. The baby had coarctation of aorta with patent ductus arteriosus and ostium primum atrial septal defect. A review of the literature in English reveals no other case reported of an infant with this association of anomlies.

Right aortic arch with isolation of the left subclavian artery

A 5-year-old boy with cyanotic heart disease and weak pulses in the left arm is described. Cardiac catheterization and cineangiography confirmed the diagnosis of tetralogy of Fallot and right aortic arch with isolation of the left subclavian artery. In addition to the aortogram and right ventricular cineangiography, pulmonary angiography was performed, demonstrating that blood did not reach the left subclavian artery through a left ductus arteriosus.