Inhaled nitric oxide in congenital diaphragmatic hernia

Nishit Shah, Timothy Jacob, Renee Exler, Stephen Morrow, Henri Ford, Craig Albanese, Eugene Wiener, Marc Rowe, Timothy Billiar, Richard Simmons, Etsuro Motoyama, Don Nakayama

Research output: Contribution to journalArticlepeer-review

59 Scopus citations


Pulmonary hypertension is a major complication of congenital diaphragmatic hernia (CDH). Inhaled nitric oxide (NO) is a selective pulmonary vasodilator because it produces vasodilatation of the pulmonary vasculature without systemic hypotension. In experimental and clinical studies, inhaled NO ameliorates pulmonary hypertension and improves gas exchange. The goal of the present study was to determine the extent to which infants with CDH respond to inhaled NO. Four newborn infants with CDH complicated by severe respiratory insufficiency and right-to-left shunting received inhaled NO. In three patients, postductal oxygenation improved in response to small concentrations of NO (5 to 10 ppm); two received NO after operative repair, and the third both before and after repair. However, tachyphylaxis developed in all cases within 1 to 6 days. A fourth patient received inhaled NO in an attempt at weaning from ECMO. He did not respond, remaining hypoxic despite 80 ppm NO, and continued to require ECMO. In the three patients who responded to inhaled NO, plasma nitrites and nitrates (stable oxidative and products of NO) accumulated over time, but not in the patient who did not respond. The accumulation of nitrite and nitrate in plasma may reflect alveolar-capillary NO absorption, and may identify patients who will respond to continued inhaled NO. Methemoglobin remained below 1.9% in all four babies. Selected infants with CDH may respond to NO, but the benefit may be temporary.

Original languageEnglish (US)
Pages (from-to)1010-1015
Number of pages6
JournalJournal of Pediatric Surgery
Issue number8
StatePublished - Aug 1994
Externally publishedYes


  • Diaphragmatic hernia, congenital, pulmonary hypertension
  • inhaled nitric oxide

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery


Dive into the research topics of 'Inhaled nitric oxide in congenital diaphragmatic hernia'. Together they form a unique fingerprint.

Cite this