1/8. Medical management of porto-pulmonary hypertension and right heart failure prior to living-related liver transplantation.Porto-pulmonary hypertension and right heart failure are relatively prevalent complications of end-stage liver disease and may increase mortality of patients undergoing cadaveric orthotopic liver transplantation. Even with extensive pre-transplant evaluation, these complications are frequently diagnosed unexpectedly in the operating room and transplant procedure may need to be aborted due to high perioperative mortality from both right and left ventricular failure. Living-related liver transplantation is a new surgical alternative to cadaveric liver transplantation, but presence of porto-pulmonary hypertension increases its postoperative mortality as well. Due to inherent elective nature, however, living-related liver transplantation may allow for preoperative hemodynamic optimization and treatment of right ventricular failure. To the authors' knowledge, this is the first reported case of an adult patient with porto-pulmonary hypertension who underwent successful living-related liver transplantation. Favorable transplantation outcome was obtained in this case through good hemodynamic control with long-term IV epoprostenol therapy, preoperative right heart calcification, and perioperative administration of pulmonary vasodilators and inotropic agents.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/8. Prolonged survival after single lung transplantation for pulmonary hypertension secondary to systemic lupus erythematosus.Pulmonary hypertension is one of the life-threatening complications of systemic lupus erythematosus, but these patients are often excluded from lung transplantation candidacy due to the nature of underlying multiple system involvement. We report a long-term survival after single lung transplantation in a case of systemic lupus erythematosus with severe pulmonary hypertension. It suggests that single lung transplantation may be considered in such patients, especially in condition of limited donor organ supply.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/8. Late "adult form" scimitar syndrome presenting with "infant form" complications.This case report describes an 85-year-old woman with complications resulting from scimitar syndrome, anomalous drainage of the right pulmonary veins into the inferior vena cava. In addition to being the oldest reported case of this congenital anomaly, this is the first report of pulmonary vascular complications from scimitar manifested in the adult. The magnitude of the vascular shunt and advanced nature of the disease precluded surgical or percutaneous intervention in this patient.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/8. Pulmonary sarcoidosis: A clinico-pathological study.The light and electron microscopic changes in biopsy tissue from the lung of a 30-year-old housewife severeley incapacitated by diffuse pulmonary sarcoidosis with pulmonary hypertension are presented. The lung tissue was distorted by numerous granulomas in the interstitial tissues and within alveoli. Many pulmonary blood vessels including arteries were damaged by the granulomas. The ultrastructural features of the epithelioid cells were found to be distinctive and probably specific. The giant cells which accompanied the epithelioid cells contained two types of inclusion body: one appeared to be related to the Schaumann body but the nature and origin of the second type was not clear. Many of the granulomas were surrounded by avascular fibrous tissue which contained, in addition to mature fibroblasts, myofibroblasts and a primitive form of cell that appeared to be a fibroblast precursor. It was conjectured that the myofibroblasts, through their contractile powers, might increase the distortion of the lung architecture and thereby the patient's disability. The alveolar walls were thickened by a diffuse infiltrate of macrophages and epithelioid cells but there was no excess of collagen and elastic fibres. The evidence suggested that the epithelioid cells developed from macrophages. From the cellular nature of the diffuse infiltration of the alveolar walls and the absence of fibrosis it seemed that the disease was still at an early and active stage, a conclusion strengthened by the fact that treatment with corticosteroids led to marked and sustained clinical improvement.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
5/8. 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 = nature (Clic here for more details about this article) |
6/8. Systemic lupus erythematosus associated with multiple nodular hyperplasia of the liver.Three autopsy cases of systemic lupus erythematosus with unique association of multiple nodular hyperplasia (MNH) of the liver, portal hypertension, and hypertensive pulmonary vascular disease are reported. None of the patients had received oral contraceptive or androgenic steroid, but they were treated with glucocorticoids for 2 to 11 years. Raynaud's phenomenon, sclerodactyly, and mild impairment of the kidney were the common clinical features. Macroscopically, MNH is characterized by many nodules scattered throughout the non-cirrhotic liver, and histologically, each nodule is made up of normal-appearing hepatocytes and not encapsulated. Portal tracts are scanty in the nodules. MNH seems to be a regenerative-hyperplastic process, but its true nature still remains unclear. Relationships between MNH and portal hypertension, MNH and pulmonary hypertension, and collagen disease and pulmonary hypertension are discussed. A brief review of the literature concerning multiple benign hepatocellular tumors similar to MNH is also presented.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/8. syncope in a 2-year-old: ED presentation of primary pulmonary hypertension.We describe the case of a previously well 2-year-old girl who was found to have primary pulmonary hypertension (PPH) after presenting to the ED with syncope. Although physical examination findings were normal, an ECG and chest radiograph showed signs of PPH. The patient's hospital course was significant for syncopal episodes and sudden death while she awaited a heart transplant. This case illustrates that suspected cardiac syncope in the young warrants a focused ED evaluation with attention to causes of a life-threatening nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/8. Monoclonal endothelial cells in appetite suppressant-associated pulmonary hypertension.Anorexigens such as aminorex fumarate and dexfenfluramine are associated with the development of severe pulmonary hypertension (PH), which clinically and histopathologically is considered indistinguishable from idiopathic or primary pulmonary hypertension (PPH). For the current study, we asked whether anorexigen-associated PH is characterized by monoclonal pulmonary endothelial cell proliferation (such as in PPH) or, alternatively, is associated with a polyclonal endothelial cell proliferation as found in secondary PH. Analysis of clonality by the human androgen receptor assay was performed in microdissected endothelial cells of plexiform lesions of two patients with anorexigen-associated PH. The four plexiform lesions of Patient 1 and the six of Patient 2 with anorexigen-associated PH exhibited a monoclonal expansion of pulmonary endothelial cells, with a mean clonality ratio of 0.03 /- 0.01 SE. Our results indicate that appetite suppressant-associated PH is identical to PPH not only in clinical and histopathologic features but also, at a molecular level, in terms of the monoclonal nature of the endothelial cell proliferation. The anorexigens may accelerate the growth of pulmonary endothelial cells in patients with predisposition to develop PPH.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |