Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study.

OBJECTIVES: Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved. METHODS: The UK and Ireland Collaborative study of Pulmonary Atresia with Intact Ventricular Septum is an ongoing population-based study of all patients born with this disease from 1991 through 1995. All available clinical, morphologic, and investigative variables were directly reviewed, and risk factor analysis was performed for poor outcome. RESULTS: One hundred eighty-three patients presented with pulmonary atresia with intact ventricular septum. Fifteen underwent no procedure, and all died. Of the remainder, 67 underwent a right ventricular outflow tract procedure (catheter or surgical), 18 underwent an outflow tract procedure with shunt, and 81 underwent a systemic-to-pulmonary shunt alone. One- and 5-year survival was 70.8% and 63.8%, respectively. Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P < .001) were independent risk factors for death. The presence of coronary artery fistulae, right ventricular dependence, or the tricuspid valvar z score did not prove to be risk factors for death. After up to 9 years of follow-up, 29% have achieved a biventricular repair, 3% a so-called one-and-a-half ventricular repair, and 10.5% a univentricular repair, with 16.5% still having a mixed circulation (41% died). CONCLUSIONS: This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.

Relationship between peripheral airway dysfunction, airway obstruction, and neutrophilic inflammation in COPD.

BACKGROUND: Considerable research has been conducted into the nature of airway inflammation in chronic obstructive pulmonary disease (COPD) but the relationship between proximal airways inflammation and both dynamic collapse of the peripheral airways and HRCT determined emphysema severity remains unknown. A number of research tools have been combined to study smokers with a range of COPD severities classified according to the GOLD criteria. METHODS: Sixty five subjects (11 healthy smokers, 44 smokers with stage 0-IV COPD, and 10 healthy non-smokers) were assessed using lung function testing and HRCT scanning to quantify emphysema and peripheral airway dysfunction and sputum induction to measure airway inflammation. RESULTS: Expiratory HRCT measurements and the expiratory/inspiratory mean lung density ratio (both indicators of peripheral airway dysfunction) correlated more closely in smokers with the severity of airflow obstruction (r = -0.64, p<0.001) than did inspiratory HRCT measurements (which reflect emphysema severity; r = -0.45, p<0.01). Raised sputum neutrophil counts also correlated strongly in smokers with HRCT indicators of peripheral airway dysfunction (r = 0.55, p<0.001) but did not correlate with HRCT indicators of the severity of emphysema. CONCLUSIONS: This study suggests that peripheral airway dysfunction, assessed by expiratory HRCT measurements, is a determinant of COPD severity. Airway neutrophilia, a central feature of COPD, is closely associated with the severity of peripheral airway dysfunction in COPD but is not related to the overall severity of emphysema as measured by HRCT.

Growth potential in the new aortic arch after non-end-to-end repair of aortic arch interruption in infancy.

BACKGROUND: Complete repair of infants with interrupted arch and ventricular septal defect through a midline incision has been the preferred method for more than 20 years. End-to-end anastomosis can result in restenosis if there is excess tension. Two methods of reducing this tension have been described, and the subsequent growth of the new aortic arch is demonstrated. METHODS: In 2 infants (5 and 9 months old) the duct was used to create a new aortic arch. In 3 other younger infants the left carotid artery was divided, turned down, and anastomosed to the descending aorta to form the new arch. These operations were performed through the midline at the same time as the ventricular septal defect was closed. RESULTS: All 5 patients are well now 8 to 19 years postoperatively. One patient required reoperation for stenosis at the anastomotic site, but all have subsequently shown good growth on follow-up angiographic and magnetic resonance imaging studies. CONCLUSIONS: Although end-to-end repair is best, these alternative methods have shown very satisfactory aortic growth into adult life.

Magnetic resonance angiography in subclavian steal syndrome.

A case is reported of a patient with the subclavian steal syndrome in whom the reversed blood flow of the vertebral artery was shown by phase encoded magnetic resonance angiography.

Evaluation of T1N0M0 lung cancer with CT.

Medical records and radiologic studies of 238 patients with non-small cell lung cancer who had preoperative evaluation by chest radiography and CT were reviewed. Thirty-six patients were staged as T1N0M0 by chest radiograph. Of this group, 18 (50%) had abnormalities on CT requiring additional evaluation. Confirmation of abnormalities was by tissue sampling or clinical follow-up. Evidence for unresectable spread of disease was obtained in 12 (33%). We conclude that routine preoperative staging of T1N0M0 lung cancer with CT has a positive impact on patient management.

MRI of Askin's tumor. Case report at 1.5 T.

This represents the first case of an Askin's tumor demonstrated on MRI. It showed a large pleural-based mass which trapped pleural fluid in large pseudotumors. The disease was unilateral and involved the mediastinum as well. Magnetic resonance imaging was helpful in demonstrating extrathoracic disease in the area of the right brachial plexus.

Small bore catheter drainage and sclerotherapy for malignant pleural effusions.

The accumulation of large amounts of fluid in the pleural space is a common sequela of disseminated carcinomatosis. Traditional management has included therapeutic thoracentesis or the placement of a large bore chest tube for drainage with the subsequent installation of a sclerosing agent in an attempt to achieve pleural symphysis. An evaluation of all patients treated in this manner during a 4-year period was undertaken to assess the degree of success obtained with a large bore standard chest tube versus a small pigtail catheter. A study group consisting of 20 patients with a total of 24 pleural effusions was treated with drainage and sclerotherapy. In this group, eight of 13 effusions were adequately treated with pigtail catheter drainage and sclerotherapy, compared with four of 11 effusions adequately treated with standard chest tube drainage and sclerotherapy. Although the numbers are small, it appears that pigtail catheter drainage and sclerosis is at least as successful as the more traditional drainage with the standard chest tube.

A clinical review of patients undergoing resection for pulmonary hamartoma.

Both the radiology and surgery literature mention "popcorn calcification" as a diagnostic aid in identifying pulmonary hamartomas. Nineteen patients underwent resection of pulmonary hamartomas at North Carolina Memorial Hospital between January 1969 and February 1983, representing 1.9 per cent of all thoracotomies performed for pulmonary disease. Of the 19 patients, there were 12 men (63%) and 7 women (37%), and most were asymptomatic. Chest x-ray demonstrated 12 right-sided and 7 left-sided lesions. All masses were located peripherally in the lung parenchyma and in no instance was calcification present. Fourteen wedge resections and 5 lobectomies were performed. Pathologic examination confirmed the absence of calcification; however, in two cases (11%) an associated malignancy was present. There were no operative deaths and only minor postoperative complications. All problems resolved without difficulty. These data show that pulmonary hamartomas are not commonly associated with calcification, and there may be an increased association with carcinoma of the lung. Excision can be performed with minimal morbidity and is necessary to distinguish hamartomas from malignant lesions.

Massive hemoptysis associated with isolated peripheral pulmonary artery stenosis.

A fatal case of massive hemoptysis in a patient with isolated peripheral pulmonary artery stenosis is described. Such patients are predisposed to developing pulmonary artery aneurysms. When hemoptysis develops, erosion of an aneurysm into an adjacent bronchus should be considered and aggressive diagnostic evaluation undertaken.

Percutaneous small bore catheter drainage in the management of lung abscesses.

For patients with pyogenic lung abscesses who do not respond to medical therapy, thoracotomy with pulmonary resection is the widely-accepted treatment of choice. Six patients with lung abscess who failed to respond to conservative medical management were treated by percutaneous catheter drainage using small catheters (10 Fr or smaller). Five patients showed prompt clinical improvement and the sixth improved after a modification in antibiotic therapy. All patients recovered with radiographic resolution of the abscess and were well at followup periods from two months to two years. In such patients, percutaneous drainage with small catheters provides an excellent clinical result with minimal risk and trauma.

CT demonstration of central pulmonary venous and arterial occlusive diseases.

Computed tomography was used to evaluate three patients with complicated pulmonary vascular patterns. The physiologic and anatomic changes seen on chest radiography were well defined by CT and in each case CT suggested the correct diagnosis.

"Pigtail" catheter drainage in thoracic surgery.

Small-bore percutaneous drainage catheters have been used extensively in the management of abdominal disorders and are gaining acceptance in thoracic problems. The records have been reviewed in 44 consecutive patients in whom 53 small-bore catheters were placed for thoracic disease, including empyema, 20; effusion, 9; pneumothorax, 10; lung abscess, 4; and mediastinal cyst, 1. Insertion was performed under fluoroscopy in 88%, computed tomography in 6%, and ultrasonography in 6%. Catheters ranged in size from 6.5F to 12F, but catheter size did not seem to influence outcome (p = 0.6). There was complete resolution of the problem in 75% (33 of 44) of the patients, although 20% of them required more than one catheter. Treatment was successful in all cases of lung abscess. These patients had been previously treated with standard medical therapy for 12 to 53 days without significant improvement in their clinical condition. Patients with empyema composed the largest group treated. Eighty percent (16 of 20) of them had a loculated (LOC) process. The LOC group appeared to have a slightly better success rate than did the non-LOC group (75 versus 50%, p = 0.33). When the catheters failed to resolve the problem (empyema, 6; chylous effusion, 1; malignant effusion, 3; and spontaneous pneumothorax, 1, either operation (9.1%) or a standard thoracostomy tube (13.6%) was required. The complications rate was 20%, but most of these were minor problems including pneumothorax, 5; catheter occlusion 3; fractured catheter, 1; infection, 1; and significant subcutaneous emphysema, 1.(ABSTRACT TRUNCATED AT 250 WORDS)

A multimodality projection console for the radiology conference room.

A multimodality projection console for the radiology conference room is described. The configuration of the TV camera, view boxes, switches, and adjacent lectern allows better transmission and easier manipulation of images during consultation and lecture sessions with the faculty, and house officers.