Pediatric Surgery International

, Volume 24, Issue 10, pp 1101–1104 | Cite as

Management of pulmonary hypertension in congenital diaphragmatic hernia: nitric oxide with prostaglandin-E1 versus nitric oxide alone

  • Satoko Shiyanagi
  • Tadaharu OkazakiEmail author
  • Hiromichi Shoji
  • Toshiaki Shimizu
  • Toshitaka Tanaka
  • Satoru Takeda
  • Kazunari Kawashima
  • Geoffrey J. Lane
  • Atsuyuki Yamataka
Original Article



Prostaglandin-E1 (PGE1) is used at most centers for treating pulmonary hypertension (PH) in congenital diaphragmatic hernia (CDH) because it has been regarded as effective. The aim of this study was to investigate the role of PGE1 for treating PH in CDH.


We reviewed 49 CDH cases with echocardiography-proven PH. PH was treated with PGE1 and nitric oxide (NO) and high frequency oscillatory ventilation (HFOV) from 1997 to 2001 (PG + NO; n = 19) and with NO and HFOV from 2002 to 2007 (NO; n = 30).


Subject demographics, severity of PH, and presence of other anomalies were not significantly different between the two groups. In the PG + NO group, 12/19 (63.2%) survived (PG + NO-s) and 7/19 (36.8%) died (PG + NO-d). In the NO group, 21/30 (70.0%) survived (NO-s) and 9/30 (30.0%) died (NO-d). Survival rates were not significantly different. In the NO-s group, spontaneous closure of the ductus arteriosus (DA) was significantly earlier compared with the PG + NO-s group (P < 0.01; 4.0 ± 0.9 vs. 9.5 ± 2.2 days after birth). DA diameters were significantly larger in groups that died compared with groups that survived (< 0.01), and PH persisted in groups that died. In the NO-s group, surgery was possible significantly earlier compared with the PG + NO-s group (P < 0.01; 3.75 ± 0.67 vs. 6.12 ± 0.78 days after birth). No NO-s case developed a PH crisis even though PGE1 was not used. Hospital stay was significantly shorter in the NO-s group compared with the PG + NO-s group (P < 0.05; 39.9 ± 19 vs. 53.2 ± 23 days).


Nitric oxide alone would appear to simplify the management of CDH with PH and provide better outcome.


Congenital diaphragmatic hernia Pulmonary hypertension Prostaglandin-E1 Nitric oxide 


  1. 1.
    The Congenital Diaphragmatic Hernia Study Group (1999) Does extracorporeal membrane oxygenation improve survival in neonates with congenital diaphragmatic hernia? J Pediatr Surg 34:720–725CrossRefGoogle Scholar
  2. 2.
    Bagolan BP, Casaccia G, Creacenzi F, Nahom A, Trucchi A, Giorlandino C (2002) Impact of a current treatment protocol of high-risk congenital diaphragmatic hernia. J Pediatr Surg 39:313–318CrossRefGoogle Scholar
  3. 3.
    Bedoyan JK, Blackwell SC, Treadwell MC, Johnson A, Klein MD (2005) Congenital diaphragmatic hernia: associated anomalies and antenatally diagnosis. Pediatr Surg Int 20:170–176Google Scholar
  4. 4.
    Okuyama H, Kubota A, Oue T, Kuroda S, Ikegami R, Kamiyama M, Kitayama Y, Yagi M (2002) Inhaled nitric oxide with early surgery improves the outcome of antenatally diagnosed congenital diaphragmatic hernia. J Pediatr Surg 37:1188–1190PubMedCrossRefGoogle Scholar
  5. 5.
    Inamura N, Kubota A, Nakajima T, Kayatani F, Okuyama H, Oue T, Kawahara H (2005) A proposal of new therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia. J Pediatr Surg 37:357–366Google Scholar
  6. 6.
    Okazaki T, Kohno S, Hasegawa S, Urushihara N, Yoshida A, Kawano S, Saito A, Tanaka Y (2003) Congenital diaphragmatic hernia: efficacy of ultrasound examination in its management. Pediatr Surg Int 19:176–179PubMedGoogle Scholar
  7. 7.
    Okawada M, Okazaki T, Yamataka A, Yanai T, Kato Y, Kobayashi H, Lane GJ, Miyano T (2006) Efficacy of protocolized management for congenital diaphragmatic hernia. A review of 100 cases. Pediatr Surg Int 22:925–930PubMedCrossRefGoogle Scholar
  8. 8.
    O’Tool SJ, Karamanoukian HL, Glick PL (1996) Cardiopulmonary consequences of congenital diaphragmatic hernia. J Pedinatol 16:34–39Google Scholar
  9. 9.
    Buss M, Williams G, Dilley A, Jones O (2006) Prevention of heart failure in the management of congenital diaphragmatic hernia by maintaining ductal patency. A case report. J Pediatr Surg 41:E9–E11PubMedCrossRefGoogle Scholar
  10. 10.
    Kitagawa M, Hislop A, Boyden EA, Reid L (1971) Lung hypoplasia in congenital diaphragmatic hernia: a quantitative study of airway, artery, and alveolar development. Br J Surg 58:342–346PubMedCrossRefGoogle Scholar
  11. 11.
    Levin DL (1978) Morphological analysis of the pulmonary vascular bed in congenital left sided diaphragmatic hernia. J Pediatr 92:805–809PubMedCrossRefGoogle Scholar
  12. 12.
    Geggel RL, Muraphy JD, Langleben D, Crone RK, Vacanti JP, Reid LM (1985) Congenital diaphragmatic hernia: arterial structural changes and persistent pulmonary hypertension after surgical repair. J Pediatr 107:357–364Google Scholar
  13. 13.
    Shah N, Jacob T, Exler R, Morrow S, Ford H, Albanese C, Wiener E, Rowe M, Billiar T, Simmons R (1994) Inhaled nitric oxide in congenital diaphragmatic hernia. J Pediatr Surg 29:1010–1015PubMedCrossRefGoogle Scholar
  14. 14.
    The national inhaled nitric oxide study group (1997) Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. Pediatrics 99:838–845CrossRefGoogle Scholar
  15. 15.
    Leveque C, Hamza J, Berg AE, Larrieu FB, Laguenie G, Flaud FG, Couturier C, Egu JF, Mekouar R, Maurice CS (1994) Successful repair of a severe left congenital diaphragmatic hernia during continuous inhalation of nitric oxide. Anesthesiology 80:1171–1175PubMedCrossRefGoogle Scholar
  16. 16.
    Christou H, Marter LJ, Wessel DL, Allred EN, Kane JW, Thompson JE, Stark AR, Kourembanas S (2000) Inhaled nitric oxide reduces the need for ECMO in infants with persistent pulmonary hypertension of the newborn. Crit Care Med 28:3722–3727PubMedCrossRefGoogle Scholar
  17. 17.
    Siebert JR, Haas JE, Beckwith JB (1984) Left ventricular hypoplasia in congenital diaphragmatic hernia. J Pediatr Surg 19:567–571PubMedCrossRefGoogle Scholar
  18. 18.
    The Congenital Diaphragmatic Hernia Study Group (2006) Treatment evolution in high-risk congenital diaphragmatic hernia. Ann Surg 244:505–513Google Scholar

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Satoko Shiyanagi
    • 1
  • Tadaharu Okazaki
    • 1
    Email author
  • Hiromichi Shoji
    • 2
  • Toshiaki Shimizu
    • 2
  • Toshitaka Tanaka
    • 3
  • Satoru Takeda
    • 3
  • Kazunari Kawashima
    • 4
  • Geoffrey J. Lane
    • 1
  • Atsuyuki Yamataka
    • 1
  1. 1.Department of Pediatric General and Urogenital SurgeryJuntendo University School of MedicineBunkyo-kuJapan
  2. 2.Department of PediatricsJuntendo University School of MedicineBunkyo-kuJapan
  3. 3.Department of Obstetrics and GynecologyJuntendo University School of MedicineBunkyo-kuJapan
  4. 4.Department of ObstetricsGonohashi Ladies’ ClinicKoto-kuJapan

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