1/47. Persistent pulmonary hypertension in a neonate with cystic adenomatoid malformation of the lung following lobectomy: survival with prolonged extracorporeal membrane oxygenation therapy.A full-term neonate is reported with congenital cystic adenomatoid malformation of the lung treated by lobectomy with development of pulmonary hypertension. The infant was successfully treated with extracorporeal membrane oxygenation (ECMO) for persistent pulmonary hypertension, which developed postoperatively. An 18-day course of venovenous ECMO was necessary to effectively reverse the severe pulmonary hypertension. This was probably a result of significant pulmonary hypoplasia of the compressed lung. Although not all congenital cystic adenomatoid malformations of the lung are associated with pulmonary hypoplasia and persistent pulmonary hypertension, this is one case where severe pulmonary hypertension developed secondary to a mass effect by a large lesion in the chest.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/47. Congenital misalignment of pulmonary vessels and alveolar capillary dysplasia: how to manage a neonatal irreversible lung disease?Congenital misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia is a rare condition consisting of anomalous veins in bronchovascular bundles, a decreased number of alveolar capillaries, and increased muscularization of pulmonary arterioles. In the literature, infants reported as having such a malformation developed respiratory distress with persistent pulmonary hypertension and ultimately died. We report the case of an infant with MPV and alveolar capillary dysplasia who was unresponsive to maximal cardiorespiratory support, including high-frequency oscillatory ventilation and inhaled nitric oxide; the infant died of pulmonary hemorrhage after 19 days, during venoarterial extracorporeal membrane oxygenation bypass. We conclude that the diagnosis of MPV and alveolar capillary dysplasia should be considered during autopsy of infants who have died of irreversible persistent pulmonary hypertension. If a lung biopsy in infants with prolonged refractory hypoxemia confirms such diagnosis before death, expensive and invasive treatments such as extracorporeal membrane oxygenation could be avoided.- - - - - - - - - - ranking = 0.4keywords = membrane (Clic here for more details about this article) |
3/47. cholesterol granulomas of the lungs associated with microangiopathic hemolytic anemia and thrombocytopenia in pulmonary hypertension.cholesterol granulomas unrelated to endogenous lipoid pneumonia, pulmonary alveolar proteinosis, or cholesterol pneumonia are a rare finding during pneumectomy or autopsy. They have been occasionally reported in association with pulmonary hypertension. We report a case where these lesions were associated with long-standing pulmonary hypertension and microangiopathic hemolytic anemia and thrombocytopenia. Plexiform lesions were present in the pulmonary vasculature secondary to pulmonary hypertension, causing hemolysis and thrombocytopenia. We suggest that destruction of red blood cells and platelets could provide membrane lipids that are taken up by phagocytic cells, which promotes the formation of these cholesterol deposits.- - - - - - - - - - ranking = 0.2keywords = membrane (Clic here for more details about this article) |
4/47. Neonatal pertussis requiring extracorporeal membrane oxygenation.Despite widespread immunization against bordetella pertussis, whooping cough remains potentially fatal in susceptible populations such as neonates. A case of neonatal pertussis with severe pulmonary hypertension (PH) requiring extracorporal membrane oxygenation (ECMO) is described. PH associated with pertussis severe enough to require ECMO is frequently irreversible and associated with a poor prognosis.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
5/47. Inhaled nitric oxide for oligohydramnios-induced pulmonary hypoplasia: a report of two cases and review of the literature.We describe the clinical courses of two premature infants, a male born at 29(4/7) weeks' gestational age after an 8-week period of rupture of membranes (ROM) and severe oligohydramnios, and a female infant born at 31 weeks' gestational age after an 18-week period of ROM and severe oligohydramnios. Within hours after birth, despite intubation and aggressive ventilation, both infants developed fulminant hypoxic respiratory failure. Their clinical courses were consistent with pulmonary hypertension and both infants were transferred for trials of inhaled nitric oxide (iNO). Both infants had dramatic responses to iNO, suggesting that the pulmonary disease seen after prolonged oligohydramnios may have a component of nitric oxide-sensitive pulmonary hypertension. The goals of this article are to (1) review oligohydramnios-induced pulmonary hypoplasia, (2) discuss patients at highest mortality risk, and (3) describe the effects of iNO on pulmonary hypertension in infants with hypoxemia following prolonged ROM and severe oligohydramnios.- - - - - - - - - - ranking = 0.2keywords = membrane (Clic here for more details about this article) |
6/47. Uveal effusion and angle-closure glaucoma in primary pulmonary hypertension.PURPOSE: To report a patient with uveal effusion and intermittent angle-closure glaucoma associated with primary pulmonary hypertension. methods: Observational case report. RESULTS: A 78-year-old woman with primary pulmonary hypertension noticed pain and decreased vision in her right eye. Ocular examination disclosed excessive dilation of conjunctival and episcleral veins and uveal effusion with annular ciliochoroidal detachment in both eyes. The right eye revealed corneal edema with folds in the descemet membrane, congested iris vessels, and a markedly shallow anterior chamber with a partially closed angle, corresponding to a recent attack of angle-closure glaucoma. Systemic treatment of her congestive heart failure with an angiotensin-II receptor antagonist resulted in a partial resolution of the uveal effusion and complete normalization of the anterior chamber depth. CONCLUSION: Primary pulmonary hypertension may cause uveal effusion, leading to a forward displacement of the lens-iris diaphragm and intermittent angle-closure glaucoma.- - - - - - - - - - ranking = 0.2keywords = membrane (Clic here for more details about this article) |
7/47. Misalignment of lung vessels: diagnostic role of conventional histology and immunohistochemistry.Misalignment of lung vessels represents a rare congenital anomaly that may cause respiratory failure in the newborn. It is characterized by abnormal position of pulmonary veins and venules that lie adjacent to arteries and bronchi, and it is usually associated with a decreased number of alveolar capillaries (i.e., alveolar capillary dysplasia), although these two conditions have been separately described. awareness of this anomaly is required by pathologists because it can be easily overlooked on lung biopsy or autopsy, and because definite diagnosis relies on histology. We report the case of a newborn male baby who developed respiratory distress 18 h after an uncomplicated delivery. The patient died on the 7th day, after high frequency oscillatory ventilation, nitric oxide inhalation and extracorporeal membrane oxygenation were unsuccessful. On autopsy, histology and immunohistochemistry demonstrated diffuse changes, fulfilling diagnostic criteria of misalignment of lung vessels and of alveolar capillary dysplasia in both lungs, with muscularization of very peripheral pulmonary arteries and a prominent interstitial and periadventitial fibrosis. Diffuse distribution of vessel misalignment could explain the rapid onset of respiratory failure, and the presence of diffuse fibrosis might have contributed to irreversible respiratory dysfunction by impairment of lung parenchyma extensibility.- - - - - - - - - - ranking = 0.2keywords = membrane (Clic here for more details about this article) |
8/47. An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation.Dehydrated hereditary stomatocytosis (DHS) belongs to the heterogeneous class of hemolytic anemias with leaky red cell membranes. splenectomy is a highly deleterious treatment, because it favors, with virtually no exception, the occurrence of thromboembolic disease. We describe here the extreme case of a patient with DHS and an associated sickle cell trait. splenectomy was carried out due to a splenic infarction that occurred during an airplane journey. About 12 years later, the patient noticed an exertional dyspnea, which gradually worsened to such a degree that she became severely incapacitated within 5 years. Eventually, the patient developed a cor pulmonale associated with chronic thromboembolic pulmonary hypertension (CTEPH) and successfully underwent a heart-lung transplant operation. This case ranks as one of the most severe examples ever recorded of the effect that splenectomy may have in DHS patients. Nonetheless, it represents the first case to receive a heart-lung transplant.- - - - - - - - - - ranking = 0.2keywords = membrane (Clic here for more details about this article) |
9/47. Fatal pulmonary hypertension associated with pertussis in infants: does extracorporeal membrane oxygenation have a role?OBJECTIVE: The deaths of 4 infants who had confirmed pertussis infection at our hospital during the past year motivated us to review their cases as well as the use of extracorporeal membrane oxygenation (ECMO) in respiratory failure caused by pertussis. methods: Retrospective chart reviews of the 4 infants who had pertussis and died at Vanderbilt University Medical Center (VUMC) from May 2001 to May 2002 were conducted. The Extracorporeal life Support Organization (ELSO) database is an international voluntary registry established in 1986 to compile passive reports of adult and pediatric patients placed on ECMO. We searched this database for pertussis cases and analyzed the clinical outcomes by age, ventilatory management, and measurements of cardiopulmonary status. RESULTS: All 4 infants who died from pertussis infection at VUMC were younger than 3 months and had severe pulmonary hypertension, and ECMO therapy was considered for respiratory failure. review of the international ELSO database, focusing only on children from 1986 to July 2002, revealed a total of 23,970 patients placed on ECMO. Since the first pertussis case treated with ECMO in 1990, a total of 61 children with pertussis have been treated with ECMO, representing an increase from 0.09% to 1% of the total cases. Mean age of the pertussis patients placed on ECMO was 88 days (1 day-2.7 years). The overall mortality was 70.5% (43 of 61) but was significantly higher for infants who were younger than 6 weeks (84%) compared with infants who were older than 6 weeks (61%). When evaluating pre-ECMO management, survivors had received significantly higher mean positive end expiratory pressures than nonsurvivors (11.1 /- 4.5 vs 7.3 /- 3.1 cmH(2)O) and had significantly higher serum pH than nonsurvivors (7.31 /- 0.14 vs 7.14 /- 0.19). There was no effect of duration of ECMO, positive inspiratory pressure, mean arterial pressure, ECMO mode (venoarterial vs venovenous mode), or sex on mortality. CONCLUSIONS: Although the ECMO registry is not an active surveillance system, it suggests that the numbers of children who require ECMO for pertussis have significantly increased. The high fatality rates of pertussis patients who have placed on ECMO are alarming and should stimulate reevaluation of whether this high-risk intervention is beneficial to patients with pertussis. This review, coupled with the 4 infants who died of pertussis at VUMC, suggests that improved measures are needed to prevent pertussis in infants.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
10/47. extracorporeal membrane oxygenation combined with endovascular embolization for management of neonatal high-output cardiac failure secondary to intracranial arteriovenous fistula. Case report.The authors describe a novel approach to the management of high-output heart failure secondary to an intracranial high-flow dural arteriovenous fistula (DAVF) by using extracorporeal membrane oxygenation (ECMO). To the best of the authors' knowledge, this represents the first report of an embolization performed in conjunction with the use of an ECMO circuit and the first report in which an ECMO cannula was used for intraarterial access for cerebral angiography. A 2-day-old girl presented with severe, high-output heart failure secondary to a high-flow intracranial DAVF. The patient was neurologically intact and no brain parenchymal abnormalities were revealed on computerized tomography scanning of the head, but she suffered severe heart failure, pulmonary hypertension, and liver and renal dysfunction. The patient underwent three endovascular embolization procedures involving coils and liquid embolic agents. Despite a decrease in the DAVF flow, the patient had only transient improvement in her pulmonary hypertension, and venoarterial ECMO therapy was instituted. Another embolization was performed while the patient was receiving ECMO therapy. Her cardiovascular status improved, she was weaned from ECMO, and she was eventually discharged home to her family. extracorporeal membrane oxygenation can be used to sustain severely ill neonates with high-output heart failure secondary to intracranial AVFs. Embolization can be performed while the patient is receiving ECMO therapy.- - - - - - - - - - ranking = 1.2keywords = membrane (Clic here for more details about this article) |
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