Abnormal vascular tone in infants and children with lung hypoplasia: Findings from cardiac catheterization and the response to chronic therapy.
OBJECTIVE
We describe four cases of chronic pulmonary hypertension in infants and children with chronic lung disease and pulmonary hypoplasia due to severe congenital diaphragmatic hernia (CDH) or congenital cystic adenomatoid malformation (CCAM). We report data from cardiac catheterization under various conditions: baseline respiratory support and room air, hyperoxic and inhaled nitric oxide challenge. We further report cardiac catheterization measures after chronic pulmonary vasodilator therapy with sildenafil alone or a combination of sildenafil and inhaled nitric oxide (three patients).


METHODS
Case series.


METHODS
Tertiary academic center.


METHODS
Infants and children ages 0-11 yrs with CDH (n = 3) or CCAM (n = 1) with evidence of chronic pulmonary hypertension by echocardiogram and cor pulmonale (n = 3).


METHODS
Catheterization and pulmonary vasodilator therapy.


RESULTS
Pulmonary vascular resistance, pulmonary arterial pressure, and changes in these measures were assessed. A 20% change in pulmonary vascular resistance was considered a clinically significant response. Ten catheterizations were performed in four patients. All patients had elevated pulmonary vascular resistance and pulmonary arterial pressures at initial catheterizations and significant vasodilation during inhaled nitric oxide.


CONCLUSIONS
Chronic lung disease following pulmonary hypoplasia from CDH and CCAM is associated with abnormal pulmonary vascular tone in infants and children with evidence of chronic pulmonary hypertension. Chronic pulmonary vasodilator therapy may improve pulmonary vascular function and enhance lung growth in infants and children who are treated during their period of potential for rapid lung growth.
Keller
Roberta L
RL
Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA.
Moore
Phillip
P
Teitel
David
D
Hawgood
Samuel
S
McQuitty
John
J
Fineman
Jeffrey R
JR
D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000150:complications; D005260:Female; D016166:Free Radical Scavengers / Q000627:therapeutic use; D006548:Hernia, Diaphragmatic / Q000150:complications; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology / Q000601:surgery*; D007223:Infant; D008168:Lung / Q000002:abnormalities* / Q000098:blood supply*; D008171:Lung Diseases / Q000150:complications; D008297:Male; D009569:Nitric Oxide / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D011687:Purines; D000068677:Sildenafil Citrate; D013450:Sulfones; D014665:Vasodilator Agents / Q000627:therapeutic use
D016166:Free Radical Scavengers; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D009569:Nitric Oxide; D000068677:Sildenafil Citrate