1/6. Right heart failure as the dominant clinical picture in a case of primary amyloidosis affecting the pulmonary vasculature.A 91-year-old female patient died of right heart failure and pulmonary hypertension. The autopsy revealed multi-organ vascular amyloidosis and pulmonary alveolar septal amyloidosis with no evidence of parenchymal myocardial amyloid deposition. This is a rare example of cor pulmonale secondary to pulmonary amyloidosis.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
2/6. Pulmonary tumor embolism to alveolar septal capillaries. An unusual cause of sudden cor pulmonale.Although metastatic spread of tumor to the lungs is common, subsequent production of cor pulmonale is not. The involvement of pulmonary alveolar capillaries causing sudden cor pulmonale is very rare. We describe a patient who presented with chest pain and sudden shortness of breath. autopsy disclosed diffuse pulmonary microembolism to septal capillaries caused by tumor cells from a squamous cell carcinoma of the cervix. To our knowledge, this is the second report of this kind of pulmonary tumor embolism.- - - - - - - - - - ranking = 5keywords = alveolar (Clic here for more details about this article) |
3/6. recurrence of sleep apnea syndrome following tracheostomy. A shift from obstructive to central apnea.This report describes an unusual case of severe obstructive sleep apnea and alveolar hypoventilation leading to hypersomnolence and cor pulmonale, which were corrected by tracheostomy. Four years later, after a 22.5-kg weight gain, nocturnal apneas of similar frequency, duration, and depth of desaturation reappeared but were totally central in origin. The central apneas were eliminated with home nocturnal positive-pressure ventilation via cuffed tracheostomy tube. Each time the patient's apneas were corrected (obstructive: tracheostomy; central: mechanical ventilation), daytime alveolar hypoventilation disappeared rapidly. Yearly right heart catheterizations and radionuclide ejection fractions documented pulmonary hypertension and right heart failure, with resolution following tracheostomy and recurrence after appearance of central apneas. The changes in hemodynamic status corresponded to the patient's weight, presence of apnea, daytime alveolar hypoventilation, and treatment of nocturnal oxyhemoglobin desaturation. This case illustrates the theory of a common etiology of both central and obstructive apnea through abnormal respiratory controller gain and points to several roles obesity may play in apnea.- - - - - - - - - - ranking = 3keywords = alveolar (Clic here for more details about this article) |
4/6. Tumor cell embolism to pulmonary alveolar capillaries. Cause of sudden cor pulmonale.A 51-year-old woman, with a 13-month history of widely metastatic breast carcinoma treated with radical mastectomy and chemotherapy, developed sudden shortness of breath and chest pain. Rapidly progressive pulmonary hypertension was documented that failed to respond to supportive measures, and the patient died. The lungs at autopsy demonstrated tumor microemboli in the form of noncohesive, individual cells within the capillaries of approximately 40% of the pulmonary alveolar septae. This case is remarkable for widespread involvement of the alveolar septal capillaries as the cause of acute cor pulmonale.- - - - - - - - - - ranking = 6keywords = alveolar (Clic here for more details about this article) |
5/6. Alveolar hypoventilation and cor pulmonale associated with chronic airway obstruction in infants with down syndrome.Four infants with down syndrome developed cor pulmonale and heart failure in association with chronic upper airway obstruction. Features of the sleep apnea syndrome were conspicuous; namely, noisy breathing with retraction, cyanosis and frequent apnea during sleep, and daytime lethargy and somnolence. The clinical picture masqueraded as cyanotic congenital heart disease. Arterial blood gas analyses revealed alveolar hypoventilation, especially during sleep. The nature of the obstructive element was variable. adenoidectomy provided partial relief in one patient, and tonsillectomy and adenoidectomy resulted in temporary improvement in two others. Three patients were markedly benefitted by tracheostomy. Functional inspiratory pharyngeal closure was demonstrated fluorographically in one patient. Infants with down syndrome may be predisposed to upper airway obstruction by virtue of hypoplasia of facial and oropharyngeal structures and generalized hypotonia. Additional obstructive elements may be contributed by hypertrophied lymphoid tissue, excessive secretions, and glossoptosis. Removal of the obstructive element is helpful, but functional obstruction may only be relieved by tracheostomy.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
6/6. Bilateral sequential lung transplantation for pulmonary alveolar microlithiasis.Pulmonary alveolar microlithiasis (PAM) is characterized by deposition of calcium phosphate within the alveolar airspaces. There is currently no effective medical therapy and affected individuals may progress to end-stage lung disease requiring transplantation. Two patients with PAM underwent bilateral sequential lung transplantation. This study reviews the clinical manifestations of PAM and discusses the particular difficulties that may be encountered in the use of lung transplantation as treatment for this uncommon disease. Also addressed is the question of recurrence in the allograft.- - - - - - - - - - ranking = 6keywords = alveolar (Clic here for more details about this article) |