Stabilisation of Cardiopulmonary Function in Newborns with Congenital Diaphragmatic Hernia Using Lung Function Parameters and Hemodynamic Management

 

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Abstract

Objective:

Evaluation of lung function parameters and additional use of prostaglandin E1 (PGE1) for the stabilisation of cardiopulmonary function in patients with congenital diaphragmatic hernia (CDH) and pulmonary hypertension (PHT).

Design:

Observational study.

Patients:

Between 2007 and 2009 8 patients with CDH have been treated in our pediatric intensive care unit (gestational age 34 + 0 – 40 + 4 weeks, birth weight 2 160–3 840 g). All patients required respiratory support. Gentle mechanical ventilation adapted to the degree of pulmonary hypoplasia based on serially measurements of lung function parameters to find appropriate ventilator settings has been performed.

Main Results:

Functional residual capacity (FRC) and compliance of the respiratory system in all patients were markedly reduced. A FRC between 9.3–10.6 ml/kg and compliance between 1.1–1.8 ml/kPa/kg indicated pronounced hypoplasia of the lungs. Doppler flow patterns through the arterial duct were classified into left-to-right, right-to-left and bidirectional shunting and correlated to the degree of PHT. The additional use of PGE1 to reopen the arterial duct and to stabilize right ventricular function led to an amelioration of severe PHT and preoperative stabilisation in 2 newborns with pronounced pulmonary hypoplasia. All patients underwent successful surgery, and did not show any complications after 2 years follow-up.

Conclusion:

Measurements of lung function parameters and adaptation of mechanical ventilation to the degree of pulmonary hypoplasia and additional therapy with PGE1 may help to improve the outcome in CDH patients.

Zusammenfassung

Hintergrund:

Evaluierung von Lungenfunktionsparametern und des zusätzlichen Einsatzes von Prostaglandin E1 (PGE1) zur kardiopulmonalen Stabilisierung bei Patienten mit angeborener frühmanifester Zwerchfellhernie (CDH) und pulmonaler Hypertension (PHT).

Design:

Beobachtungsstudie.

Patienten:

Von 2007 bis 2009 wurden 8 Patienten mit CDH (Gestationsalter 34 + 0 bis 40 + 4 Wochen, Geburtsgewicht 2 160–3 840 g) auf unserer pädiatrischen Intensivstation behandelt. Alle Patienten benötigten respiratorische Unterstützung. Eine milde maschinelle Beatmung, angepasst an den Grad der Lungenhypoplasie (bestimmt durch serielle Lungenfunktionsmessungen), wurde durchgeführt.

Ergebnisse:

Die funktionelle Residualkapazität (FRC) und Compliance des respiratorischen Systems war bei allen Patienten mit CDH deutlich reduziert. Eine FRC von 9,3–10,6 ml/kg und eine Compliance zwischen 1,1–1,8 ml/kPa/kg wiesen auf eine ausgeprägte Lungenhypoplasie hin. Das Flussmuster im Ductus arteriosus wurde mittels Echokardiografie untersucht und in Links-Rechts-, Rechts-Links- und bidirektionalen Shunt eingeteilt und zum Schweregrad der PHT korreliert. Der Einsatz von PGE1 zur Wiedereröffnung des Ductus arteriosus und Stabilisierung der rechtsventrikulären Funktion führte bei 2 Neugeborenen mit ausgeprägter Lungenhypoplasie zur Verbesserung der schweren PHT und präoperativen Stabilisierung. Alle Patienten wurden erfolgreich operiert und zeigten keine Komplikationen nach 2 Jahren Follow-up.

Zusammenfassung:

Messung von Lungenfunktionsparametern, Anpassung der maschinellen Beatmung an den Grad der Lungenhypoplasie sowie zusätzlicher Einsatz von PGE1 können zu einer Optimierung der Therapie von Patienten mit CDH beitragen.

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