Treatment of rebound and chronic pulmonary hypertension with oral sildenafil in an infant with congenital diaphragmatic hernia.
OBJECTIVE
We describe a case of chronic pulmonary hypertension in a 7-wk-old infant with congenital diaphragmatic hernia and an oral teratoma. Our patient was dependent on low-dose inhaled nitric oxide and was still very unstable with systemic right ventricular pressures leading to frequent oxygen desaturations. We administered sildenafil therapy to stabilize the infant with discontinuation of inhaled nitric oxide. We describe successful discontinuation of the inhaled therapy as well as a period of stabilization and improvement with continued sildenafil administration.


METHODS
Case report.


METHODS
Intensive care nursery in tertiary academic center.


METHODS
A 7-wk-old infant with congenital diaphragmatic hernia who was mechanically ventilated from birth.


METHODS
Oral sildenafil 0.3 mg/kg/dose every 12 hrs.


RESULTS
Right ventricular pressure (from tricuspid valve regurgitant flow) to systemic systolic arterial pressure was measured by echocardiogram. Right ventricular to systemic pressure ratio was marginally improved with the initiation of sildenafil therapy. Inhaled nitric oxide was successfully discontinued, and the patient clinically stabilized temporarily, but he ultimately succumbed to his pulmonary hypertension.


CONCLUSIONS
Sildenafil may be a useful therapy for chronic pulmonary hypertension in congenital diaphragmatic hernia, but further studies of safety and efficacy need to be performed.
Cardiovascular Research Institute and the Department of Pediatrics, The University of California San Francisco, San Francisco, CA, USA.
Roberta L
RL
Keller
Shannon E G
SE
Hamrick
Joseph A
JA
Kitterman
Jeffrey R
JR
Fineman
Samuel
S
Hawgood
D015106:3',5'-Cyclic-GMP Phosphodiesterases; D002908:Chronic Disease; D054706:Cyclic Nucleotide Phosphodiesterases, Type 5; D004452:Echocardiography; D006548:Hernia, Diaphragmatic / Q000150:complications*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D007223:Infant; D009569:Nitric Oxide; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use*; D010727:Phosphoric Diester Hydrolases / Q000378:metabolism; D010879:Piperazines / Q000627:therapeutic use*; D011687:Purines; D000068677:Sildenafil Citrate; D013450:Sulfones
D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D009569:Nitric Oxide; D000068677:Sildenafil Citrate; D010727:Phosphoric Diester Hydrolases; D015106:3',5'-Cyclic-GMP Phosphodiesterases; D054706:Cyclic Nucleotide Phosphodiesterases, Type 5; C517381:PDE5A protein, human