No abstract

Absent pulmonary valve syndrome (APVS); the combination of tetralogy of Fallot (TOF) with agenesis of the pulmonary valve, is a relatively rare cardiac malformation. Despite the anatomic similarity with classic TOF, the pathophysiology is strikingly different. Data on 10 patients (3 male, 7 female) with APVS, treated between January 1978 and December 1995, were retrospectively reviewed. During this period a total of 2920 children underwent correction of a variety of congenital cardiac anomalies, of which 246 patients (8%) had a correction for TOF. Two patients with APVS presented within the first four months of life with severe cardiorespiratory distress and required several operative procedures. The remaining eight patients had only mild to moderate respiratory and/or cardiac symptoms and elective intracardiac repair was performed on those between the ages of 10 months and 9.5 years. Associated cardiac anomalies seen in five patients included aberrant coronary artery, absent or interrupted left pulmonary artery, partial AVSD and aberrant azygos continuation. In those electively corrected, the strategies used were ventriculotomy (7), pulmonary homograft (3) and aneurysmorrhaphy (2). There were two deaths, one in each group of patients, as a result of progressive respiratory insufficiency and cardiac tamponade, respectively. The follow-up of the eight survivors ranged from 2 to 11 years (median 6.75). All have a normal effort tolerance; only one child is on digoxin therapy, and one child continues to suffer bronchospastis episodes. Our experience with infants with this lesion is limited but underlines the different approaches required, depending on the age of presentation.

Fifty-seven patients who had 72 thoracic sympathectomies have been reviewed. Twenty-five patients were preoperatively diagnosed as having primary Raynaud's phenomenon, 14 as having Raynaud's phenomenon secondary to arterial occlusion, 17 as having ischemia secondary to arterial occlusion and one as having hyperhidrosis. Twenty percent of the patients initially diagnosed as having primary Raynaud's phenomenon at the time of operation developed a collagenosis during the follow-up period. Improvement after 8 years' follow-up did not depend on the primary indication. Up to the fifth year after surgery a relapse was seen in cases of primary Raynaud's phenomenon. In secondary Raynaud's phenomenon a gradual decrease in improvement of 2% a year was seen during follow-up. In ischemia secondary to arterial occlusion, after 2 years the percentage of improved patients remained constant at a level of 70%. The mean improvement after 8 years follow-up according to the survival test of Gehan was 70 +/- 10%.

From 1954 to 1985, 150 metastases were removed in 80 patients (55 males, 25 females) with an age range from 8 to 82 years. The role of pulmonary resection for metastatic lesions of the period 1954 to 1975 (group I) was compared to the period 1976 to 1985 (group II). In group I, 48 metastases were resected in 35 patients and in group II, 102 metastases in 45 patients. The surgical mortality in the total population was 1%. The average interval from diagnosis of the primary neoplasm to diagnosis of thoracic metastases was 4 years in both groups. Primary neoplasm localization did not differ in the 2 groups. In both groups approximately 50% of the patients were without symptoms. Wedge resection and lobectomy were the most frequent procedures followed by segmentectomy and pneumonectomy. The median post thoracotomy survival was 21 months in group I and 36 months in group II. Although the tumor-free interval, presenting symptoms and surgery did not differ in the 2 groups, the actuarial 5-year survival in group I was 31%, and 53% for group II. Neither sex, age nor the lung resection type significantly affected the therapeutic results. Good prognostic factors were a non-seminomatous testicular tumor as the primary tumor, a tumor-free interval longer than 60 months and a tumor-doubling time longer than 136 days. Poorer results were obtained in the presence of N2 metastases, and of a large tumor volume. It seems that with the increased effectiveness of chemotherapy, especially in non-seminomatous testicular tumor, the role of surgery is changing. Surgery is now also indicated to resect metastases unresponsive to chemotherapy and to obtain histology of stabilized lesions after chemotherapy.