Topic
Alveolar capillary dysplasia
About: Alveolar capillary dysplasia is a research topic. Over the lifetime, 219 publications have been published within this topic receiving 5569 citations. The topic is also known as: Alveolar capillary dysplasia with misalignment of pulmonary veins.
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TL;DR: Congenital lung anomalies are increasingly discovered on routine prenatal ultrasound or incidentally during postnatal imaging for respiratory insufficiency of unknown origin and their management is considered.
Abstract: The developing lung is a highly intricate organ with endless possibilities for maldevelopment. Individually, congenital abnormalities of the lung are rare but collectively they form an important group of conditions that are not infrequently encountered by physicians. The range of malformations is broad and the clinical impact is very variable, depending on the degree of malformation. Congenital lung anomalies are increasingly discovered on routine prenatal ultrasound or incidentally during postnatal imaging for respiratory insufficiency of unknown origin. This article groups these conditions under their traditional headings and considers their management.
11 citations
TL;DR: A newborn male baby who developed respiratory distress and pneumothorax 11 h after an uncomplicated delivery and deteriorated despite full ventilatory support and extracorporeal membrane oxygenation (ECMO) is reported.
Abstract: Alveolar capillary dysplasia, although rare, is a universally fatal form of persistent pulmonary hypertension of the newborn. We report a case of a newborn male baby who developed respiratory distress and pneumothorax 11 h after an uncomplicated delivery. He deteriorated despite full ventilatory support and extracorporeal membrane oxygenation (ECMO). Open lung biopsy provided a diagnosis of alveolar capillary dysplasia and decision was made to withdraw treatment.
10 citations
TL;DR: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, developmental lung disorder, which usually presents as persistent pulmonary hypertension of the newborn (PPHN) unresponsive to treatment.
Abstract: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, developmental lung disorder, which usually presents as persistent pulmonary hypertension of the newborn (PPHN) unresponsive to treatment. The authors present their own experience with three cases admitted during the last 15 years.
10 citations
TL;DR: Open lung biopsy provides clinically useful information in infants receiving ECMO therapy and may be complex and represent both the effects of ECMO and progression of the underlying disease.
10 citations
TL;DR: For neonates with a diagnosis of persistent pulmonary hypertension of the newborn, irreversible pulmonary dysplasia should be considered when extracorporeal membrane oxygenation is initiated on day of life ≥5 and/or the duration of extracORM oxygenation ≥10 days.
Abstract: OBJECTIVE Neonates with an irreversible pulmonary dysplasia such as alveolar capillary dysplasia, surfactant protein deficiency, and pulmonary lymphangiectasis may have a deteriorating clinical course requiring cardiopulmonary support with extracorporeal membrane oxygenation. These neonates are often difficult to distinguish from those with persistent pulmonary hypertension of the newborn. The objective of this study was to identify clinical variables that distinguish infants with irreversible pulmonary dysplasia from those with persistent pulmonary hypertension of the newborn before, and while receiving, extracorporeal membrane oxygenation support. DESIGN A retrospective analysis of the Extracorporeal Life Support Registry from 2000 to 2010 was performed. SETTING A total of 114 extracorporeal membrane oxygenation centers providing data to the Extracorporeal Life Support Registry. PATIENTS All neonates day of life 0-31 reported to the Extracorporeal Life Support Registry with irreversible pulmonary dysplasia and persistent pulmonary hypertension of the newborn were identified. INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS Patient demographics, pre-extracorporeal membrane oxygenation variables, and survival were analyzed. Univariate analysis was performed using Student's t test or Fisher's exact test, and variables found to be significant underwent multivariate analysis by logistic regression. Neonates with irreversible pulmonary dysplasia were placed on extracorporeal membrane oxygenation later (day of life 5.3 vs. 3.0, p = .045) and for a longer duration (11.1 vs. 6.8 days, p < .001) than those with persistent pulmonary hypertension of the newborn. Initiation of extracorporeal membrane oxygenation at day of life ≥5 (p = .026) and a duration of extracorporeal membrane oxygenation ≥10 days (p = .003) were independent predictors of irreversible pulmonary dysplasia by multivariate analysis. No differences in demographics, blood gas values, or vascular access were observed. Survival to discharge was significantly lower for neonates with irreversible pulmonary dysplasia (3%) vs. persistent pulmonary hypertension of the newborn (81%, p < .001). CONCLUSION Although neonates with irreversible pulmonary dysplasia and persistent pulmonary hypertension of the newborn have similar presentations, those with irreversible pulmonary dysplasia require extracorporeal membrane oxygenation support later in the perinatal period and for a longer duration. For neonates with a diagnosis of persistent pulmonary hypertension of the newborn, irreversible pulmonary dysplasia should be considered when extracorporeal membrane oxygenation is initiated on day of life ≥5 and/or the duration of extracorporeal membrane oxygenation ≥10 days.
10 citations