Biomedical subjects
Publications and source records attributed to J A Culham.
Computed tomographic scans of the lower thorax were obtained of ten infants whose plain chest radiographs showed poorly defined right heart borders. Satisfactory studies in eight of these children showed the explanation of the silhouette sign. Pulmonary or pleural disease was seen in seven of the eight. In one, the thymus obscured the heart border. A poorly defined right heart border in children in the first year of life is often abnormal.
Six of eight patients survived anatomic correction of transposition of the great arteries and repair of associated cardiovascular anomalies at the Hospital for Sick Children, Toronto, Canada. Two of the six survivors also had tricuspid atresia, and continuity between the right atrium and the subaortic outlet chamber in these patients was provided by a valved external conduit, in addition to the arterial switch and coronary artery reimplantation. Five of the six patients have undergone complete postoperative clinical, hemodynamic and angiocardiographic investigation and form the basis of this communication. Our present indications for anatomic repair are discussed.
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Severe stress can produce ulceration and uncontrollable hemorrhage in the pediatric age group. Modern angiographic techniques, using embolization of a Silastic balloon to occlude a visualized bleeding vessel, can successfully control hemorrhage and avoid surgical intervention and its resultant disordered gastric physiology. Recently this technique was used to control hemorrhage from the gastroduodenal artery in a 1-year-old child with severe respiratory sepsis. This is the youngest reported child in whom the technique has been used to control bleeding from this vessel.
From 1965 to 1979, 44 patients with Potts and 50 with Waterston aortopulmonary anastomoses were studied angiographically. Fifty-two of the 95 shunts had been banded to limit growth of the anastomosis. Later the internal diameter of the anastomosis was measured in 34 children when the cardiac defect was repaired. On those with late measurements, growth was limited effectively in 17 of the 18 (94%) shunts that had been banded, whereas five of the 16 (31%) unbanded anastomoses grew to more than 6.5 mm internal diameter (p = 0.05). The difference in incidence of moderate or severe stenosis of the pulmonary artery near or at the anastomosis nearly reached a significant level (p = 0.07), occurring in 50% of children with banded shunts in comparison with 31% of children with unbanded shunts. Mean pulmonary artery pressures were obtained in 77 children, 36 with potts and 41 with Waterston shunts. Six of 43 with a banded anastomosis had a mean pulmonary artery pressure above 30 mm Hg, the highest being 43 mm Hg. Seven of 34 children with an unbanded anastomosis had a mean pulmonary artery pressure of 30 mm Hg or more, and in three the pressures were over 50mm Hg. In unbanded Potts or Waterston shunts the incidence of pulmonary artery stenosis was 60%. This high incidence discourages the use of these aortopulmonary anastomoses if other shunts can be constructed safely and effectively.
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Three cases for longstanding untreated cyanotic congenital heart disease are presented. In addition to collateral circulation from bronchial, intercostal, and inferior phrenic arteries, thoracic aortic vasa vasorum were visualized. We believe this may represent an additional collateral pathway to the lungs. The angiography of these cases is presented.
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A pulmonary vein angiogram was performed using a pressure injection with the tip of an end and side hole catheter in a near-wedge position; extravasation of contrast material into a bronchus occurred, causing severe bronchoconstriction. It may be possible to avoid this complication by the use of an end hole balloon catheter in the wedge position, slow flow rates of contrast material, and low pressure injections.
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A prospective clinical and echocardiographic diagnosis of single ventricle was made in 42 patients. Each was evaluated for the number of atrioventricular (A/V) valves, presence of an outflow chamber (OC), A/V valve-semilunar continuity, and orientation of the great arteries. Angiographic correlations were subsequently obtained in 40 and autopsies in 12. The overall diagnosis of single ventricle was substantiated in 39. Two other patients diagnosed as single ventricle by angiography were thought to have large ventricular septal defects on echocardiography. Tricuspid valve was interpreted as septum in one. The angiographic diagnosis of single ventricle was incorrect in another, correctly diagnosed by echocardiography and confirmed at pathology. The differential diagnosis also included A-V canal, L-transposition of the great arteries, double outlet right ventricle, and tricuspid atresia. This last condition has to be differentiated on clinical evidence. The echocardiograms were of particular value in determining the number of A/V valves. Two great arteries were demonstrated in 74% of patients and their relationship was correctly determined in 79% of these. Both imaging techniques agreed closely as to A/V valve-semilunar continuity and presence of an OC, but both showed some inaccuracies compared to pathological specimens. The echocardiogram helps both in planning catheterization and in evaluating the overall diagnosis.
To assess the effect of hydrocarbon pneumonitis on the developing lung, we studied the pulmonary function of 17 asymptomatic children, 8 to 14 years after the initial insult. Fourteen of the 17 subjects (82%) had one or more pulmonary function abnormalities, the most frequent being a high volume of isoflow. Volume of isoflow, ratio residual volume to total lung capacity, slope of phase III, flow rates at 50% and 25% of vital capacity and 60% of the total lung capacity, one-second forced expiratory volume, and maximum midexpiratory flow rate differed significantly (P less than .05) from values in control groups. Closing volume and closing capacity were not significantly different. Residual abnormalities present in children after hydrocarbon pneumonitis can be explained on the basis of small airway obstruction and/or loss of elastic recoil. These children may be at risk for the development of chronic lung disease as adults when they are exposed to exogenous factors such as air pollution or smoking.
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There is considerable heterogeneity among pathologic mechanisms responsible for subaortic obstruction in infancy. Although the relatively discrete forms of subaortic stenosis are uncommon in this age group, conoventricular malalignment, maladherent mitral valve, accessory endocardial cushion tissue, restrictive bulboventricular foramen, and a poorly expanded suboartic conus are responsible for most types of subaortic stenosis in this age group. The angiocardiographic recognition of these types of subaortic obstruction is aided by awareness of the anatomic framework in which they are found. For example: (1) conoventricular malalignment is commonly noted in patients with complete interruption of the aortic arch; (2) restriction at bulboventricular foramen level results in functional subaortic stenosis in those patients whose transposed aorta originates above an infundibular chamber; and (3) a poorly expanded subaortic conus may result in subaortic stenosis in patients whose ventricular loop and conotruncus have twisted in opposite directions (the anatomically corrected malposition). Subaortic obstruction in infancy is not uncommon, occurring in 99 of 1,362 necropsied infants with congenital heart disease. Nearly 70% of the patients with subaortic stenosis had obstructive anomalies of the aortic arch. Subaortic obstruction can be demonstrated angiographically, but high quality angiocardiographic technique is a prerequisite.
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Extreme slowing of hepatic arterial flow occurred in 2 children who had angiographic examination under general anethesia with halothane. The incidence of this striking angiographic "halothane effect" was 32%. It was thought to be caused by a specific increase in hepatic vasomotor tone at the arterial, sinusoidal or venous level. The functional significance of the effect is unclear and its role in the production of hepatic dysfunction or massive hepatic necrosis is conjectural. No untoward effects were noted in our patients.
Selective aortic root angiography was performed in 18 patients with the clinical diagnosis of hypoplastic left heart syndrome in order to allow more precise correlation with echocardiography. Aortic valve atresia and severe hypoplasia of the left heart chambers subsequently were substaniated at necropsy in each instance. Wide variation in the size and shape of the aortic root and ascending aorta was demonstrated angiographically in these patients. In some patients, despite aortic valve atresia, the dimensions of the aortic root and sinuses of Valsalva at angiography were almost normal. In addition, this technique allowed the demonstration of primitive vascular communications between the coronary artery and left ventricular cavity in 1 patient (coronary-cameral fistula). The realization that the aortic root may approach nearly normal size in some patients with the hypoplastic left heart syndrome is essential to the echocardiographic diagnosis of this disease and to the differential diagnosis from other cardiac and noncardiac causes of severe neonatal congestive heart failure.