Pulmonary Vascular Disease: Pulmonary Vascular Disease: PAH |

Long-Term Mechanical Ventilation as Adjunctive Therapy for Children With Severe Pulmonary Hypertension FREE TO VIEW

Paul Sammut, MBBCh
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University of Nebraska Medical Center, Omaha, NE

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A515. doi:10.1016/j.chest.2016.02.537
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SESSION TITLE: Pulmonary Vascular Disease: PAH

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Saturday, April 16, 2016 at 11:45 AM - 12:45 PM

PURPOSE: The mortality rate of infants with severe chronic lung disease and pulmonary hypertension (PH) remains very high at around 70%, despite the availability of multiple vasodilator agents which have greatly improved the survival of children with pulmonary hypertension in general. The effects of hypercarbia on pulmonary artery pressure (PAP) have been shown to be variable, but most clinicians believe it has a significant pulmonary vasoconstricting effect and a negative effect on the vasodilating capability of other agents. Mechanical ventilation has the capability of improving the level of hypercarbia in these patients and, therefore, the potential to improve the severity of pulmonary hypertension. Very few clinical studies have been done on this topic and almost no reports can be found in the pediatric literature.

METHODS: We describe the outcomes of 6 children who underwent tracheostomy tube placement and long-term (7 months to 8 years) mechanical ventilation, in addition to multiple agent vasodilator therapy, and who fall into the category described above and almost certainly would have died without this therapy. Three children had pulmonary hypoplasia due to congenital diaphragmatic hernias, two had bilateral hypoplasia due to other etiologies and one had severe multi-cystic lung disease with anomalous pulmonary arterial supply. All received mechanical ventilation soon after birth and a tracheostomy tube later on. Two underwent ECMO for a period early in their course.

RESULTS: None of the patients has died. Three children, aged 8 years, 17 months and 7 months, have persistent severe PH (>2/3 sytemic level) and remain ventilated but are active and one attends school. The child with multi-cystic lungs remains ventilated but has near-normal PAP. One child has normal PAP at baseline and enjoys many daytime hours off the ventilator. The sixth was decannulated at 33 months and is an active 4 year old with normal PAP.

CONCLUSIONS: We conclude that, in children with severe lung disease and severe PH, long-term mechanical ventilation offers an effective clinical benefit as adjunctive therapy to vasodilator medications in those who, without such therapy, death is the likely outcome.

CLINICAL IMPLICATIONS: Long-term mechanical ventialation can offer the chance of possible resolution of PH in some children with severe lung disease and, in those who’s PH will not improve, it helps maintain survival such that other interventions (such as lung transplantation) may be more successful than if tried in the neonatal period.

DISCLOSURE: The following authors have nothing to disclose: Paul Sammut

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