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

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.

Key words

arterial duct - prostaglandin E1 - congenital diaphragmatic hernia - pulmonary hypertension - functional residual capacity - lung compliance

Schlüsselwörter

Ductus arteriosus - Prostaglandin E1 - angeborene Zwerchfellhernie - pulmonale Hypertension - funktionelle Residualkapazität - Lungencompliance

• References

• 1 Aspelund G, Fisher JC, Simpson LL et al. Prenatal Lung-Head Ratio: Threshold to Predict Outcome for Congenital Diaphragmatic Hernia. J Matern Fetal Neonatal Med 2011; Oct 4 [Epub ahead of print]
• 2 Bagolan P, Casaccia G, Crescenzi F et al. Impact of a current treatment protocol on outcome of high-risk congenital diaphragmatic hernia. J Pediatr Surg 2004; 3: 313-318
• 3 Balassy C, Kasprian G, Brugger PC et al. Assessment of lung development in isolated congenital diaphragmatic hernia using signal intensity ratios on fetal MR imaging. Eur Radiol 2010; 4: 829-837
• 4 Bohn D, Tamura M, Perrin D et al. Ventilatory predictors of pulmonary hypoplasia in congenital diaphragmatic hernia, confirmed by morphologic assessment. J Pediatr 1987; 3: 423-431
• 5 Butt W, Taylor B, Shann F. Mortality prediction in infants with congenital diaphragmatic hernia: potential criteria for ECMO. Anaesth Intensive Care 1992; 4: 439-442
• 6 Chao PH, Huang CB, Liu CA et al. Congenital diaphragmatic hernia in the neonatal period: review of 21 years’ experience. Pediatr Neonatol 2010; 2: 97-102
• 7 Chiu P, Hedrick HL. Postnatal management and long-term outcome for survivors with congenital diaphragmatic hernia. Prenat Diagn 2008; 7: 592-603
• 8 Christ T, Loersch F, Kohl T et al. Effektivität der fetoskopischen trachealen Ballonokklusion bei Neugeborenen mit Zwerchfellhernie. Klin Padiatr 2010; GNPI_PO_81 Abstract-.
• 9 Deprest JA, Gratacos E, Nicolaides K et al. Changing perspectives on the perinatal management of isolated congenital diaphragmatic hernia in Europe. Clin Perinatol 2009; 2: 329-47 ix
• 10 Dinger J, Peter-Kern M, Goebel P et al. Effect of PEEP and suction via chest drain on functional residual capacity and lung compliance after surgical repair of congenital diaphragmatic hernia: preliminary observations in 5 patients. J Pediatr Surg 2000; 10: 1482-1488
• 11 Dinger J, Topfer A, Schaller P et al. Functional residual capacity and compliance of the respiratory system after surfactant treatment in premature infants with severe respiratory distress syndrome. Eur J Pediatr 2002; 9: 485-490
• 12 Finer NN, Barrington KJ. Nitric oxide for respiratory failure in infants born at or near term. Cochrane Database Syst Rev 2001; 4CD000399-.
• 13 Heling KS. Stellenwert der sonographischen Lungenbiometrie in der pränatalen Vorhersage einer Lungenhypoplasie 2003;
• 14 Hosgor M, Tibboel D. Congenital diaphragmatic hernia; many questions, few answers. Paediatr Respir Rev 2004; S277-S282
• 15 Hulskamp G, Pillow JJ, Dinger J et al. Lung function tests in neonates and infants with chronic lung disease of infancy: functional residual capacity. Pediatr Pulmonol 2006; 1: 1-22
• 16 Jani JC, Cannie M, Peralta CF et al. Lung volumes in fetuses with congenital diaphragmatic hernia: comparison of 3D US and MR imaging assessments. Radiology 2007; 2: 575-582
• 17 Jani JC, Nicolaides KH, Gratacos E et al. Severe diaphragmatic hernia treated by fetal endoscopic tracheal occlusion. Ultrasound Obstet Gynecol 2009; 3: 304-310
• 18 Kattan J, Godoy L, Zavala A et al. Improvement of survival in infants with congenital diaphragmatic hernia in recent years: effect of ECMO availability and associated factors. Pediatr Surg Int 2010; 7: 671-676
• 19 Keller RL, Tacy TA, Hendricks-Munoz K et al. Congenital diaphragmatic hernia: endothelin-1, pulmonary hypertension, and disease severity. Am J Respir Crit Care Med 2010; 4: 555-561
• 20 Mohseni-Bod H, Bohn D. Pulmonary hypertension in congenital diaphragmatic hernia. Semin Pediatr Surg 2007; 2: 126-133
• 21 Reiss I, Schaible T, van den HL et al. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium consensus. Neonatology 2010; 4: 354-364
• 22 Ruano R, Duarte SA, Pimenta EJ et al. Comparison between fetal endoscopic tracheal occlusion using a 1.0-mm fetoscope and prenatal expectant management in severe congenital diaphragmatic hernia. Fetal Diagn Ther 2011; 1: 64-70
• 23 Ruano R, Takashi E, da Silva MM et al. Prediction and probability of neonatal outcome in isolated congenital diaphragmatic hernia using multiple ultrasound parameters. Ultrasound Obstet Gynecol 2012 Jan 39(1): 42-49
• 24 Schaible T, Veit M, Tautz J et al. Serum cytokine levels in neonates with congenital diaphragmatic hernia. Klin Padiatr 2011; 414-418
• 25 Shiyanagi S, Okazaki T, Shoji H et al. Management of pulmonary hypertension in congenital diaphragmatic hernia: nitric oxide with prostaglandin-E1 versus nitric oxide alone. Pediatr Surg Int 2008; 10: 1101-1104
• 26 Skari H, Bjornland K, Frenckner B et al. Congenital diaphragmatic hernia in Scandinavia from 1995 to 1998: Predictors of mortality. J Pediatr Surg 2002; 9: 1269-1275
• 27 Stressig R, Fimmers R, Eising K et al. Preferential streaming of the ductus venosus and inferior caval vein towards the right heart is associated with left heart underdevelopment in human fetuses with left-sided diaphragmatic hernia. Heart 2010; 19: 1564-1568
• 28 Tanabe M, Yoshida H, Iwai J et al. Doppler flow patterns through the ductus arteriosus in patients with congenital diaphragmatic hernia. Eur J Pediatr Surg 2000; 2: 92-95
• 29 Tei C, Ling LH, Hodge DO et al. New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function – a study in normals and dilated cardiomyopathy. J Cardiol 1995; 6: 357-366
• 30 The Congenital Diaphragmatic Hernia Study Group . Defect size determines survival in infants with congenital diaphragmatic hernia. Pediatrics 2007; 651-657
• 31 Tohyama M, Baba A, Tsuno T et al. Aneurysmal dilatation of ductus arteriosus during lipo-prostaglandin E1 therapy for diaphragmatic hernia. Eur J Pediatr 1993; 11: 877-879
• 32 Tracy ET, Mears SE, Smith PB et al. Protocolized approach to the management of congenital diaphragmatic hernia: benefits of reducing variability in care. J Pediatr Surg 2010; 6: 1343-1348