Late presentation of misalignment of lung vessels with alveolar capillary dysplasia

Abdallah, Hasan I.; Karmazin, Nelly; Marks, Lloyd A.

doi:10.1097/00003246-199304000-00026

Cited by 72 publications

(51 citation statements)

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“…Age of death varied from 2 to 61 days. 7,9 Sex distribution of MPV and alveolar capillary dysplasia is similar. Most occurrences are sporadic; however, a familial predisposition has been described in two cases.…”

Section: Discussionmentioning

confidence: 93%

“…The severity of illness was different between infants, and a transitory response to maximal supportive treatments, including high-frequency oscillatory ventilation, inhaled nitric oxide, or ECMO, was described. 5,7,8 However, all reported infants died; this is not surprising considering that the underlying pathophysiology is an irreversible structural defect. Age of death varied from 2 to 61 days.…”

Section: Discussionmentioning

confidence: 96%

“…4,7 Features may also include a reduced number of alveoli and patchy distribution of histopathologic changes. Failure of formation and ingrowth of alveolar capillaries leads to the absence of a normal air-blood barrier.…”

Section: Discussionmentioning

confidence: 99%

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Congenital Misalignment of Pulmonary Vessels and Alveolar Capillary Dysplasia: How to Manage a Neonatal Irreversible Lung Disease?

et al. 2000

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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. Journal of Perinatology 2000; 3:189-192.Congenital misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia is a rare disorder of pulmonary vascular development associated with persistent pulmonary hypertension (PPHN) and unremitting hypoxemia. It was first described in 1981 in a full-term infant girl who developed respiratory distress and PPHN at birth and died of hypoxemia after 40 hours. At autopsy, a decreased number of alveolar capillaries, increased muscularization of pulmonary arterioles, and anomalous veins in bronchovascular bundles were detected. In the literature, 22 cases have been described with this condition. All infants developed respiratory distress with PPHN within the first 2 days of life. Despite a transient response to maximal supportive therapies, all patients died. [1][2][3][4][5][6] CASE REPORTThis newborn infant girl was born at 41 weeks' gestation by cesarean section; the pregnancy was uncomplicated, birth weight was 2790 gm, and Apgar scores were 7 and 9 at 1 and 5 minutes, respectively. At 3 hours of age, a cyanotic spell occurred and ambient oxygen concentration increased. Next, respiratory status deteriorated and respiratory distress occurred; the infant was intubated at 20 hours, and mechanical ventilation was started. Fractional inspired oxygen (FIO 2 ) and ventilatory settings were progressively increased; at FIO 2 of 100%, arterial blood gas showed a pH of 7.24, PCO 2 of 41 mm Hg, PO 2 of 26 mm Hg, HCO 3 of 21, and base excess of Ϫ4. The chest radiograph showed well-aerated lungs and nonspecific diffuse haziness. Echocardiography showed an anatomically normal heart with a right to left shunt through a patent ductus arteriosus and a small foramen ovale. An infection screen was performed, and ampicillin and gentamicin were administered. Inotropic support with dopamine and dobutamine was started to maintain normal blood pressure values. The baby continued to ...

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Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 96%

See 1 more Smart Citation

Congenital Misalignment of Pulmonary Vessels and Alveolar Capillary Dysplasia: How to Manage a Neonatal Irreversible Lung Disease?

et al. 2000

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“…11 Rare reports also indicate that the clinical presentation of ACD may occur after the immediate perinatal period. 6,12 However, infants with this condition usually present with early severe respiratory failure requiring maximal medical intervention and have a rapid recurrence of symptoms of pulmonary hypertension after ECMO therapy has been discontinued, leading to death within hours to days. Tolazaline and prostacyclin treatment have also been reported, 1-3,5,9 -11 but without clinical success.…”

Section: Discussionmentioning

confidence: 99%

“…Alveolar capillary dysplasia (ACD) with misaligned pulmonary veins is an exceedingly rare and apparently lethal cause of persistent pulmonary hypertension of the newborn (PPHN), previously reported in the literature in only 21 cases. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] The final diagnosis in the majority of these cases could be made only after microscopic pathologic examination of the lung on autopsy. However, recent reports have described open lung biopsy as a diagnostic alternative.…”

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confidence: 99%