Atsutoshi Tsuji

An infant with aneurysm of the thoracoabdominal aorta and blood elastase activity

Aortic aneurysms are rarely encountered in infancy. We recently had in our care a Japanese male infant with a gigantic aortic aneurysm in the thoracoabdominal region that was detected by a chest xray, taken before an operation for ptosis of the eyelids, at 11 months of age. At birth he weighed 3120 g and measured 47 cm. It was noted that he had an odd-looking face with ptosis of the eyelids. His psychomotor milestones were normal. There was no hereditary disease, anomaly, or early unexpected death in family history. Physical examination on admission revealed the following: body temperature, 37.2~ pulse, 130/ min, and respiratory rate, 36/min. The infant s blood pressure was 108/60 mmHg, weight 7420 g, and length 69 cm. The apex beat was in the fifth intercostal space at the left midclavicular line. The first and second heart sounds were normal. There was a murmur audible at the left upper sternal border. The radial and femoral pulses were equal. The laboratory data were not contr ibutory except for elastase activity, which was decreased in granulocytes and increased in whole blood (Table 1). The thoracic aortogram showed a markedly dilated thoracoabdominal aneurysm extending from the left distal subclavian ar tery to the coeliac axis. At 1.5 years of age a bypass grafting of the thoracoabdominal aneurysm was successfully performed. The postoperat ive course was uneventful and he is enjoying regular physical activity at present. The resected specimen showed advanced medial necrosis with conspicuous smooth muscle cell involvement, but lacking the cystic medial necrosis seen in Marfan s syndrome. The pathophysiology of this case is unknown at present. Nonetheless , the finding of an elevated elastase activity in whole blood may be pertinent to the basic abnormality. The granulocyte elastase is released in response to inflammation, to play a role in the dissolution of various kinds of proteins, such as elastin, collagen, fibrinogen, and others. A causal relationship may exist between the increased whole blood elastase activity and the unique form of medial necrosis in this patient.

Recovery from congenital complete atrioventricular block

SummaryCongenital complete atrioventricular block without any other intracardiac anomaly reverted to sinus rhythm at the age of 7 years. The girl had had heart failure and Adams-Stokes attacks when aged 1 year, requiring pacemaker implantation. Electrocardiography confirmed sinus rhythm for the first time at the age of 7 years. At the same time, a treadmill test yielded a normal tracing without any arrhythmias.

Electrocardiographic standard for the diagnosis of right ventricular hypertrophy in complete right bundle branch block.

It is generally considered that in complete right bundle branch block, the electrocardiographic standard for the diagnosis of right ventricular hypertrophy is not applicable.

Venous Pressure Measurements in Infants With and Without Heart Failure

Venous pressures of 50 infants were measured at the sagital sinus. The range of normal venous pressure was between 8 and 21cm. H2O. (Mean; 14.48cm. H2O). With the exception of an infant who had predominant left‐sided heart failure, venous pressures of infants in failure were markedly elevated, up to 33cm. H2O. Their venous pressures returned to normal as they recovered from heart failure following anti‐congestive therapy. In pneumonia or other respiratory diseases, however, venous pressure remained within normal range although clinical manifestations suggested the possibility of heart failure. The sagital sinus appears to be a reliable site for venous pressure measurements.

Gigantic aneurysm in the thoraco-abdominal aorta of an infant.

We report the case of a boy with a gigantic aneurysm in the thoraco‐abdominal region which was detected by a chest X‐ray taken prior to surgical correction of ptosis of the eyelids at 11 months of age. At 18 months, he successfully underwent aneurysm exclusion and bypass grafting. A biopsy from the thoracic aorta revealed medial degeneration with conspicuous smooth muscle cell involvement. Laboratory examination showed altered elastase activity in the granulocytes and whole blood. The present case may represent a unique form of aneurysm in infancy.

Diagnosis and Morphological Classification of Pulmonary Arteriovenous Fistula by 99mTc MAA

Pulmonary A‐V fistula was diagnosed and classified morphologically based on the pattern of time‐activity curves by 99m Tc MAA. If the diameter of the fistula is large, macro‐albumin easily reveals a shunt. If the iistula has the form of an “aneurysm,” the degree of shunt is generally slight.

Four Cases of Left Aortic Arch and Right Descending Aorta

We studied 4 cases of left aortic arch and right descending aorta. Swallowing disturbance and vomiting were observed in one case. In the other cases, there were no symptoms related to esophageal or tracheal compression.

Development of Adipose Tissue with Special Reference to Recovery from Adipose Hypocellularity in Congential Heart Disease.

Thirty two children with normal growth and development for age, and 57 children with serious congenital heart disease were investigated for height, weight, total body fat, fat cell size, total fat cell number, and lean body mass as a function of age. Examination of fat samples was carried out by a modification of the method of Hirsch and Gallian. In children with normal growth (ages 1–14 years) fat cell number continues to increase, but fat cell size does not change with increasing age. Reduced fat in congenital heart disease is primarily due to decreased fat cell number. If cardiac defects are repaired between 1 and 5 years of age recovery of flat cell number can be achieved.