Long survival of congenital alveolar capillary dysplasia patient with NO inhalation and epoprostenol: Effect of sildenafil, beraprost and bosentan

Abstract

The case is described herein of a patient with alveolar capillary dysplasia with double-outlet right ventricle and duodenal atresia who survived for a remarkably long time. The newborn girl was born at a gestational age of 36 weeks and weighed 1926 g. One min after delivery the Apgar score was 4. The patient had persistent pulmonary hypertension (PH) and needed nitric oxide inhalation and i.v. epoprostenol all through her life. Although other oral medications for PH were tried, they could not be used in practice because of gastrointestinal complications. The patient died on the 237 th day of life as a result of worsening PH associated with infection.

Antenatal gastrointestinal anomalies in neonates subsequently found to have alveolar capillary dysplasia

“…Most infants with ACD die from hypoxemic respiratory failure within the first 2 weeks of life. There are eight cases in which long‐term survival beyond the neonatal period have been described and one infant survived for 36 months . In this case series, two infants with generalized lung involvement presented within the first 2 days of life and died the day after.…”

A late presenter and long-term survivor of alveolar capillary dysplasia with misalignment of the pulmonary veins

“…Thus far, six infants with ACD/MPV beyond the neonatal period have been reported, and the longest survivor has been an 8-month-old child [1][2][3][4][5][7][8][9]. One report describing two sibling cases shows that a patient with less severe symptoms has a patchy pattern of capillary deficiency and abnormal distal air space, which may be correlated with the late onset [4]; however, this finding remains controversial because some reports describing histological appearance of lung tissues taken from early and late presenters have been strikingly similar [1-3, 5, 8].…”

Dysplasie alvéolo-capillaire avec mésalignement des veines pulmonaires : une cause de cyanose néonatale réfractaire létale

“…Malgré les traitements disponibles, y compris l'ECMO, l'évolu-tion se fait constamment vers le décès par complication de l'hypoxie. La survie maximale rapportée avait été de huit mois [9,10], mais généralement le décès survient dans le premier mois de vie. L'analyse histologique du tissu pulmonaire prélevé avant ou après le décès est actuellement la référence pour le diagnostic ( fig.…”

“…La littérature rapporte de nombreux cas de pneumothorax uni-ou bilatéraux associés à la DAC/MVP, probablement du fait de stratégies ventilatoires agressives. Afin de diminuer l'HTAP, les vasodilatateurs artériels pulmonaires tels que le monoxyde d'azote (NO) inhalé, le sildénafil, le bosentan, le trepostinil ou l'époprosténol peuvent être utilisés avec un effet transitoire mais n'empêchent en aucun cas le décès[10]. L'ECMO donne le temps de réaliser une biopsie pulmonaire, mais ne permet pas de survie à long terme.…”