Pediatric Congenital Diaphragmatic Hernia Medication

Updated: Dec 22, 2020
  • Author: Robin H Steinhorn, MD; Chief Editor: Dharmendra J Nimavat, MD, FAAP  more...
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Medication Summary

Medical therapy in congenital diaphragmatic hernia (CDH) is directed toward stabilizing blood pressure and circulating volume, pulmonary distress, and hypoxemia.


Vasoactive agents

Class Summary

Judicious use of vasoactive agents may increase cardiac output without affecting systemic or pulmonary vascular resistance.

Dopamine (Intropin)

Dopamine increases blood pressure primarily via stimulation of alpha-adrenergic receptors; however, its mechanism of action in newborn infants remains controversial because of developmental differences in endogenous norepinephrine stores and expression and function of alpha-adrenergic receptors. Dosage must be individualized.

Dobutamine (Dobutrex)

Increases blood pressure primarily via stimulation of beta1-adrenergic receptors. It appears to have a more prominent effect on cardiac output than on blood pressure.

Milrinone (Primacor)

Bipyridine-positive inotrope and vasodilator with little chronotropic activity. Mode of action differs from that of digitalis glycosides and catecholamines. Selectively inhibits PDE III in cardiac and smooth vascular muscle, resulting in reduced afterload, reduced preload, and increased inotropy.


Opioid analgesics

Class Summary

These agents are used for deep sedation to allow adequate mechanical ventilation. They may be particularly useful in decreasing sympathetic pulmonary vasoconstriction in response to noxious stimuli, such as suctioning.

Fentanyl (Duragesic, Sublimaze)

Synthetic opioid that is 75-200 times more potent than morphine. It is highly lipophilic and protein-bound. Prolonged exposure leads to accumulation in fat and delays the weaning process. Used alone, fentanyl causes minor cardiovascular compromise, although the addition of benzodiazepines or other sedatives may result in decreased cardiac output and blood pressure.


Neuromuscular relaxing agents

Class Summary

Paralysis is sometimes necessary in an infant who is unstable despite adequate sedation; however, the use of paralysis is controversial and should be reserved for unusual cases in which the infant cannot be treated with appropriate sedation.

Pancuronium (Pavulon)

Relatively long-acting nondepolarizing muscle relaxant. Onset of action is 1-2 min, and duration of action is 45-90 min. Excretion is renal (80%) and hepatic (20%), and duration of action may be longer if renal or hepatic failure is present.

Vecuronium (Norcuron)

Has few to no adverse hemodynamic adverse effects and may be preferred over pancuronium as a muscle relaxant in the infant with PPHN; however, it is more expensive than pancuronium.

Intermediate-acting nondepolarizing muscle relaxant. Onset of action is 1-2 min, and duration of action is 45-90 min. Primary route of excretion is hepatic.


Pulmonary vasodilating agents

Class Summary

Nitric oxide is an important mediator of vascular tone that was approved as a therapeutic modality for infants with PPHN. It is delivered as an inhaled gas. At least two multicenter studies did not show that inhaled nitric oxide decreases mortality or the need for extracorporeal support in infants with CDH; however, it may be useful in stabilizing an infant while evaluating or transferring for ECMO.

Nitric oxide (INOmax)

The FDA approved nitric oxide for the treatment of PPHN in December 1999. Produced endogenously from action of enzyme NO synthetase on arginine. Relaxes vascular smooth muscle by binding to heme moiety of cytosolic guanylate cyclase, activating guanylate cyclase and increasing intracellular levels of cGMP, which then leads to vasodilation. When inhaled, NO decreases pulmonary vascular resistance and improves lung blood flow.

Optimal dose is unknown, although most investigators agree that doses >20 ppm are not beneficial and may be harmful. Administration should occur under controlled conditions, with access to ECMO if needed. NO2 and methemoglobin levels should be frequently monitored, and weaning should gradually occur. Abrupt discontinuation may be associated with severe rebound pulmonary hypertension.