Showing papers on "Alveolar capillary dysplasia published in 2002"

Alveolar capillary dysplasia: a cause of persistent pulmonary hypertension of the newborn.

Abstract: 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.

Incidence of alveolar capillary dysplasia in severe idiopathic persistent pulmonary hypertension of the newborn

Abstract: Objective: To determine the incidence and outcome and to review the management of alveolar capillary dysplasia (ACD) among newborns with severe idiopathic persistent pulmonary hypertension (PPHN). Methods: A retrospective review of medical records of infants admitted to a paediatric intensive care unit from 1982 to 2000 with a diagnosis of severe PPHN, and re-examination of lung histological sections was carried out. Results: Thirteen new-born infants with pulmonary hypertension not associated with any known cause were identified. All were treated with conventional mechanical ventilation or high-frequency oscillatory ventilation with high inspired-oxygen and non-specific pulmonary vasodilators. Nine infants were also treated with inhaled nitric oxide therapy and eight with extracorporeal membrane oxygenation (ECMO). Seven infants died and six survived. At autopsies, the histological features of ACD were seen in the six who had died in the newborn period. All these had been treated with ECMO. In two of these six infants, lung biopsies had been performed showing similar features, suggesting the possibility of diagnosis during life. In the remaining infant, who died at 3 months of age, there was only marked hypertrophy of the muscle coat in the small pulmonary arteries. Conclusions: Alveolar capillary dysplasia is probably not as rare a condition as previously suggested in sporadic case reports from literature on the subject. It should be entertained as a cause of otherwise severe idiopathic PPHN of the newborn, particularly if ECMO is required. Diagnosis during life is possible by lung biopsy. It is uncertain if survival occurs with milder forms of the condition.

Intravenous prostacyclin combined with inhaled nitric oxide therapy for an infant with alveolar capillary dysplasia

Abstract: Alveolar capillary dysplasia (ACD) was first reported by Janney et al. in 1981.1 The clinical features of the disease resemble those of persistent pulmonary hypertension of the neonate (PPHN). However, ACD can be differentiated from idiopathic PPHN by the following characteristics: (i) there are usually no predisposing factors in ACD, including asphyxia, prematurity and infection; and (ii) there is a latent period from birth to the onset of the disease, a so-called ‘honeymoon period’, with a mean duration of 48 h.1,2 The clinical course of the disease is serious, with a mortality rate of 100%. We present a newborn infant with ACD, treated with a combination therapy of i.v. prostacyclin (PGI2) and inhalation of nitric oxide (NO). The patient survived for 90 days, the longest survival ever reported.