Pierre Robin syndrome and pulmonary hypertension

Five infants with Pierre Robin syndrome developed evidence of carbon dioxide retention and congestive cardiac failure despite measures to alleviate upper airway obstruction. Investigations included chest radiography, electrocardiography, echocardiography, and cardiac catheterization; pulmonary hyper...

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Veröffentlicht in:Journal of pediatric surgery 1985-02, Vol.20 (1), p.49-52
Hauptverfasser: Dykes, E.H., Raine, P.A.M., Arthur, D.S., Drainer, I.K., Young, D.G.
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container_end_page 52
container_issue 1
container_start_page 49
container_title Journal of pediatric surgery
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creator Dykes, E.H.
Raine, P.A.M.
Arthur, D.S.
Drainer, I.K.
Young, D.G.
description Five infants with Pierre Robin syndrome developed evidence of carbon dioxide retention and congestive cardiac failure despite measures to alleviate upper airway obstruction. Investigations included chest radiography, electrocardiography, echocardiography, and cardiac catheterization; pulmonary hypertension was diagnosed. In two cases raised main pulmonary artery pressures of 40 mm Hg and 120 mm Hg were recorded. Relief of upper airway obstruction was achieved by tracheostomy in three cases and nasopharyngeal intubation in two cases, with reversal of signs of cor pulmonale in each. Four patients progressed well with no recurrence of cardiac problems but one died suddenly one month after apparently successful management by tracheostomy.
doi_str_mv 10.1016/S0022-3468(85)80391-2
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subjects Female
Humans
Hypertension, Pulmonary - diagnosis
Hypertension, Pulmonary - etiology
Hypertension, Pulmonary - therapy
Infant
Infant, Newborn
Intubation, Intratracheal
Male
Pierre Robin syndrome
Pierre Robin Syndrome - complications
Pierre Robin Syndrome - therapy
Positive-Pressure Respiration
Pulmonary Heart Disease - prevention & control
Pulmonary Heart Disease - therapy
pulmonary hypertension
Tracheotomy
title Pierre Robin syndrome and pulmonary hypertension
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