1/13. Congenital misalignment of pulmonary veins with alveolar capillary dysplasia causing persistent neonatal pulmonary hypertension: report of two affected siblings.Misalignment of pulmonary vessels with alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Most of the reported cases have been sporadic. We present two consecutive affected siblings with this disorder. This is the fifth reported family occurrence of this condition. In addition to the pulmonary abnormality, one of our cases had duodenal atresia.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
2/13. Alveolar capillary dysplasia: a cause of persistent pulmonary hypertension of the newborn.The term alveolar capillary dysplasia refers to complex vascular abnormalities which have recently been identified in some infants with persistent pulmonary hypertension. We report four cases admitted to our institution for severe pulmonary hypertension unresponsive to maximal cardiorespiratory support, including high-frequency ventilation, inhaled nitric oxide and extracorporeal membrane oxygenation. The four infants died of refractory hypoxaemia. The diagnosis of alveolar capillary dysplasia was established by necropsy. We have used these cases as an opportunity for a thorough review of the literature containing comments regarding aetiology, pathophysiology, clinical presentation, associated malformations and treatment trials. CONCLUSION: alveolar capillary dysplasia should be ruled out in all newborn infants presenting severe idiopathic pulmonary hypertension associated with malformations. Open lung biopsy may prevent from using costly, invasive and probably ineffective procedures such as extracorporeal membrane oxygenation.- - - - - - - - - - ranking = 0.6keywords = alveolar (Clic here for more details about this article) |
3/13. Persistent pulmonary hypertension of the newborn due to alveolar capillary dysplasia.Three unrelated female term infants died when less than 1 month old from intractable pulmonary hypertension associated with deficient capillaries in airspace walls, anomalous small pulmonary veins in bronchiolar-arterial rays, and medial thickening in small pulmonary arteries together with peripheral muscularization. This complex vascular abnormality in the lungs has been termed alveolar capillary dysplasia and/or misalignment of lung vessels in seven previously reported cases. Each infant also showed abnormally immature parenchymal development in the lungs, as was noted in four of the seven prior cases. One had phocomelia; four of the seven prior cases had a variety of congenital anomalies. The primary pulmonary vascular anomaly is likely to be a failure of fetal lung vascularization dating from the second trimester and to be due to action of an unknown teratogen. Centroacinar veins may represent bronchial veins that do not normally develop beyond the ends of cartilaginous bronchi. Pulmonary arterial occlusive changes are interpreted as reactive to obstruction at the level of pulmonary arterioles.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
4/13. Persistent pulmonary hypertension in a neonate with vein of Galen arteriovenous malformation.Vein of Galen aneurysmal malformation (VGAM) often leads to death in the neonatal period, mainly due to congestive heart failure. Chronic and excessive pulmonary flow in utero and postnatally is attributed to large VGAM and right ventricular overload. We report a male neonate with VGAM complicated by severe heart failure and persistent pulmonary hypertension. Endovascular coil embolization of VGAM was performed, resulting in improvement of congestive heart failure; however, severe persistent pulmonary hypertension led to death 2 days after the embolization. Postmortem examination showed marked right and left ventricular hypertrophy and impressive muscular thickening of intra-alveolar arterioles. Neonatal embolization seems to be beneficial only in babies without suprasystemic pulmonary hypertension. Therefore, early delivery and repair of VGAM should be considered before the onset of persistent pulmonary hypertension.- - - - - - - - - - ranking = 0.2keywords = alveolar (Clic here for more details about this article) |
5/13. Fatal persistent pulmonary hypertension presenting late in the neonatal period.Two cases of fatal idiopathic persistent pulmonary hypertension presented late in the neonatal period. Lungs were examined histologically by light and electron microscopy, and immunocytochemical studies were used to identify nerves. There was extension of medial smooth muscle distally along the arterial pathway so that most precapillary arteries had completely muscular walls, which in some cases completely obliterated the vessel lumen. Enlarged endothelial cells also contributed to the reduction in the size of the lumen. Nerve fibres accompanying muscular arteries were found in the alveolar region, more distal than is normal. The predominant neuropeptide was the vasoconstrictor tyrosine. Possible aetiological factors in persistent pulmonary hypertension of the newborn are increased muscularity of the peripheral pulmonary arteries antenatally, an increase in the number of vasoconstrictor nerves, or an imbalance in the production of leukotrienes and prostacyclins in the perinatal period.- - - - - - - - - - ranking = 0.2keywords = alveolar (Clic here for more details about this article) |
6/13. Historical controls for extracorporeal membrane oxygenation in neonates.A retrospective review of all patients cared for who met Loe's criteria for extracorporeal membrane oxygenation (ECMO) was conducted covering the 3-yr period 1983 through 1985. There were five out of 3,726 admissions who met criteria for ECMO (three of 127 outborn admissions). All infants were greater than 2 kg birth weight and met criteria based on alveolar-arterial oxygen pressure difference (P[A-a]O2), barotrauma criteria, or both P(A-a)O2 and barotrauma criteria. All infants had persistent pulmonary hypertension. Two patients also had hyaline membrane disease and one also had asphyxia and meconium aspiration. All patients were treated with conventional therapy and all survived. There were no patients who met criteria for ECMO and died and there were no patients referred for ECMO during this period. Published criteria for ECMO estimate a control group mortality rate of 80% to 94%. mortality for this series was 0%. (Ninety-five percent confidence interval for mortality in a group of five survivors is 0% to 45%.) Controlled trials of ECMO were not done initially because it was considered unethical. This series shows that historical mortality rates are no longer valid and that controlled trials must be done.- - - - - - - - - - ranking = 0.2keywords = alveolar (Clic here for more details about this article) |
7/13. Misalignment of lung vessels and alveolar capillary dysplasia: a cause of persistent pulmonary hypertension.Two infants with fatal persistent pulmonary hypertension are described. Morphologically there was misalignment of the lung vessels, with the veins and the arterioles anomalously related, often sharing the same adventitial sheet. The capillaries did not make contact with the alveolar epithelium. The arterioles had increased medial muscle, and there was extension of the arteriolar muscularization to the precapillary level. The fraction of the parenchyma that was septal and connective tissue was increased. The acini had a decreased complexity, with immature alveoli and with a decreased radial alveolar count. The cause appeared to be related to abnormal capillary and venous plexus formation and migration. This syndrome seems to be identical with that described in three previous reports and probably represents a specific cause of persistent pulmonary hypertension.- - - - - - - - - - ranking = 1.2keywords = alveolar (Clic here for more details about this article) |
8/13. Congenital alveolar capillary dysplasia: a developmental vascular anomaly causing persistent pulmonary hypertension of the newborn.The clinical course and histologic findings are presented of an infant with an unusual form of pulmonary dysplasia. Characteristic sonographic findings and progressive hypoxemia led to the diagnosis of persistence of the fetal circulation. The patient expired despite ventilatory and pharmacologic intervention. Postmortem findings of severe pulmonary capillary hypoplasia, despite normal anatomical and biochemical parenchymal maturation, were observed. It is suggested that factors controlling pulmonary capillary maturation may be significantly different from those involved in airway and pulmonary parenchymal development.- - - - - - - - - - ranking = 0.8keywords = alveolar (Clic here for more details about this article) |
9/13. Misalignment of pulmonary veins with alveolar capillary dysplasia: affected siblings and variable phenotypic expression.Misalignment of pulmonary veins with alveolar capillary dysplasia is recognized as a rare cause of persistent pulmonary hypertension of the neonate. Until now, misalignment of pulmonary veins was thought to be a random occurrence, but its appearance in siblings at our institution suggests that there may be a familial predisposition. There have been reports of variable expression and variable severity in this disease; our report describes this variability in family members.- - - - - - - - - - ranking = 1keywords = alveolar (Clic here for more details about this article) |
10/13. Congenital syphilis associated with persistent pulmonary hypertension of the neonate--a clinico-pathological case study.Congenital syphilis remains a significant clinical problem, especially in developing countries. We report a fatal case of congenital syphilis complicated by persistent pulmonary hypertension and hypoxic ischaemic encephalopathy. OBJECTIVE: To describe the association of congenital syphilis with persistent pulmonary hypertension of the newborn (PPHN). METHOD: Case report of a single patient. RESULTS: fatal outcome of one baby with congenital syphilis and associated PPHN despite maximal conventional treatment. Histological examination of the lungs revealed pulmonary oedema, intra-alveolar haemorrhages, localised bronchopneumonia and marked interstitial infiltrates of lymphocytes, plasma cells, macrophages and fibroblasts with interstitial fibrosis. Examination of peripheral pulmonary arteries revealed focal excessive muscularisation with increased adventitial connective tissue. DISCUSSION: Reviewing our own experience and available literature, this case study supports the infrequent association of congenital syphilis with PPHN. However, when it occurs, this combination appears to be fatal. CONCLUSION: More research is warranted to clarify the role of inflammatory mediators in congenital syphilis of the lung.- - - - - - - - - - ranking = 0.2keywords = alveolar (Clic here for more details about this article) |
| | Next -> |