Death occurring within one week of cardiac transplantation: findings in eight patients.

Between October 1982 and July 1988, 85 patients underwent orthotopic heart transplantation at Methodist Hospital of Indiana. Excluding perioperative deaths, survival rates at six months, one year, two years and three years were 94%, 80%, 74% and 61%, respectively. However, eight patients (9.4%) died within one week of the transplantation. Causes of death included acute failure of the right side of the heart in four patients; compression of the proximal portions of the coronary arteries in one patient; hyperacute rejection in one patient; acute pneumonia and the adult respiratory distress syndrome in one patient; and sudden death of unknown etiology in one patient. The varied causes of death in this group of patients made it inaccurate to assume a particular cause of death for an individual patient, based on the length of the postoperative period alone. We reviewed these eight deaths in detail to better understand and, therefore, reduce the risk of early postoperative death in future patients.

Holoprosencephaly, ear abnormalities, congenital heart defect, and microphallus in a patient with 11q- mosaicism.

We report on a newborn male with deletion of part of 11q, the 27th reported case. Our patient had some of the clinical characteristics of the 11q- syndrome, but his male gender, liveborn status, q21 breakpoint, and mosaicism were unusual. In addition, he demonstrated holoprosencephaly, with cyclopia and arhinencephaly, manifestations previously unreported in the 11q- syndrome. We discuss the above points and review the literature on 11q-.

Squamous carcinoma of the lung in a nonirradiated, nonsmoking patient with juvenile laryngotracheal papillomatosis.

Juvenile laryngotracheal papillomatosis (JLTP)--a disease characterized by the growth of multiple, recurrent, koilocytolic papillomas of the upper respiratory tree--occurs in 1,500 to 2,000 infants and children in the United States every year. In rare instances, the papillomas, which are thought to be caused by a human papillomavirus (HPV), may extend into the distal bronchi and lungs. They are often excised surgically or by laser resection, but may regress spontaneously. Patients who receive radiation or cytotoxic drugs for this condition, or subsequently become smokers, may be predisposed to the development of bronchopulmonary carcinoma. Only six cases of bronchopulmonary carcinoma arising in persons with a history of JLTP but no history of these predisposing factors have been reported. Herein, we report the occurrence of squamous carcinoma of the left lung in a 28-year-old woman with JLTP since the age of 2 1/2 years. The carcinoma as well as randomly chosen papillomas excised over a period of years demonstrated positive nuclear staining for papillomavirus structural antigen. This is consistent with the current belief that human papillomavirus may be an important factor in the development of squamous carcinomas in various anatomic locations.

Status of the myocardium and infarct-related coronary artery in 19 necropsy patients with acute recanalization using pharmacologic (streptokinase, r-tissue plasminogen activator), mechanical (percutaneous transluminal coronary angioplasty) or combined types of reperfusion therapy.

In acute myocardial infarction, myocardial salvage is dependent on rapid restoration of blood flow. Pharmacologic (streptokinase, recombinant tissue-type plasminogen activator), mechanical (percutaneous transluminal coronary angioplasty, guide wire perforation) or combined forms of reperfusion therapy can accomplish this goal, but their effects on infarcted myocardium and vessel occlusion site have not been compared at necropsy. The heart of 19 necropsy patients who had received various forms of acute reperfusion therapy was studied: 14 had pharmacologic or combined forms of reperfusion therapy (13 streptokinase and 1 tissue-type plasminogen activator, including 4 with combined balloon angioplasty) and 5 had had purely mechanical (balloon angioplasty) reperfusion therapy. Reperfusion was initially clinically successful in all 19 patients with the average time from onset of symptoms to reperfusion being 3.7 hours. Necropsy observations separated the 19 patients into distinct subgroups based on changes in the myocardium and infarct-related coronary arteries. Of the 19 patients, 14 (74%) had hemorrhagic myocardial infarction and they all received pharmacologic or combined forms of reperfusion therapy. The remaining five patients (26%) had nonhemorrhagic (anemic) infarction and were treated with balloon angioplasty therapy alone. Increased luminal cross-sectional area was present in 8 of 9 patients with acute balloon angioplasty but severe coronary atherosclerotic plaque remained in 9 of 10 patients without acute balloon angioplasty. Severe hemorrhage surrounded angioplasty sites in all four patients who also received streptokinase or tissue-type plasminogen activator. Severe bleeding at the angioplasty site compromised the dilated coronary lumen in one patient. No patient with angioplasty alone had intraplaque bleeding. Thus, acute coronary balloon angioplasty reperfusion therapy alone appears to avoid the potentially adverse effects of myocardial and intraplaque hemorrhage while simultaneously increasing luminal cross-sectional area at the site of acute occlusion.