The Relationship of Pulmonary Artery Pressure and Survival in Congenital Diaphragmatic Hernia

Peter W. Dillon, Robert E. Cilley, David Mauger, Christopher Zachary, Andreas Meier, R. P. Altman, B. Gaines, P. Glick, D. Caniano, P. Donahoe

Research output: Contribution to journalArticle

131 Citations (Scopus)

Abstract

Background/Purpose: Pulmonary hypertension is an integral part of the pathophysiology of the respiratory failure associated with congenital diaphragmatic hernia. Hypothesizing that the evolution of the pulmonary hypertension would determine clinical outcome, the authors examined pulmonary artery pressures in relation to survival in their CDH patients. Methods: The authors performed a retrospective chart review of all neonates with the CDH from 1991 to 2002 at their institution eliminating infants with complex congenital heart disease, prematurity, or limited treatment. Cardiac ECHO data were used to estimate pulmonary artery pressures as a ratio to systemic pressure. Statistical analyses of estimated pulmonary pressure ratios stratified by survival status and time were performed using X2 and Fisher's Exact Test methods. Results: Forty-seven full-term CDH infants with 428 cardiac ECHO evaluations were studied. Long-term survival rate was 74%. Forty-nine percent of patients had normal pulmonary artery pressure estimates within the first 3 weeks of life. All patients survived. Seventeen percent had persistent systemic or suprasystemic pressure estimates unrelieved by treatment interventions resulting in 100% mortality rate. Thirty-four percent had intermediate reductions in pressure estimates over time with 75% survival rate. Systemic pulmonary artery pressures were associated with decreased survival at all time-points when compared with normal-pressure survivors: week 1, 60% (P < .003); week 3, 38% (P < .007); week 6, 0% (P < .02). Conclusions: The evolution of pulmonary hypertension is a critical determinant of survival in CDH patients with current treatment strategies. Three groups can be modeled with markedly different clinical performance patterns. Using serial cardiac ECHO examinations, pulmonary artery pressure estimations can be used to predict clinical outcome.

Original languageEnglish (US)
Pages (from-to)307-312
Number of pages6
JournalJournal of pediatric surgery
Volume39
Issue number3
DOIs
StatePublished - Jan 1 2004

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Pulmonary Artery
Pressure
Survival
Pulmonary Hypertension
Survival Rate
Congenital Diaphragmatic Hernias
Respiratory Insufficiency
Survivors
Heart Diseases
Therapeutics
Newborn Infant
Lung
Mortality

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Dillon, Peter W. ; Cilley, Robert E. ; Mauger, David ; Zachary, Christopher ; Meier, Andreas ; Altman, R. P. ; Gaines, B. ; Glick, P. ; Caniano, D. ; Donahoe, P. / The Relationship of Pulmonary Artery Pressure and Survival in Congenital Diaphragmatic Hernia. In: Journal of pediatric surgery. 2004 ; Vol. 39, No. 3. pp. 307-312.
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The Relationship of Pulmonary Artery Pressure and Survival in Congenital Diaphragmatic Hernia. / Dillon, Peter W.; Cilley, Robert E.; Mauger, David; Zachary, Christopher; Meier, Andreas; Altman, R. P.; Gaines, B.; Glick, P.; Caniano, D.; Donahoe, P.

In: Journal of pediatric surgery, Vol. 39, No. 3, 01.01.2004, p. 307-312.

Research output: Contribution to journalArticle

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T1 - The Relationship of Pulmonary Artery Pressure and Survival in Congenital Diaphragmatic Hernia

AU - Dillon, Peter W.

AU - Cilley, Robert E.

AU - Mauger, David

AU - Zachary, Christopher

AU - Meier, Andreas

AU - Altman, R. P.

AU - Gaines, B.

AU - Glick, P.

AU - Caniano, D.

AU - Donahoe, P.

PY - 2004/1/1

Y1 - 2004/1/1

N2 - Background/Purpose: Pulmonary hypertension is an integral part of the pathophysiology of the respiratory failure associated with congenital diaphragmatic hernia. Hypothesizing that the evolution of the pulmonary hypertension would determine clinical outcome, the authors examined pulmonary artery pressures in relation to survival in their CDH patients. Methods: The authors performed a retrospective chart review of all neonates with the CDH from 1991 to 2002 at their institution eliminating infants with complex congenital heart disease, prematurity, or limited treatment. Cardiac ECHO data were used to estimate pulmonary artery pressures as a ratio to systemic pressure. Statistical analyses of estimated pulmonary pressure ratios stratified by survival status and time were performed using X2 and Fisher's Exact Test methods. Results: Forty-seven full-term CDH infants with 428 cardiac ECHO evaluations were studied. Long-term survival rate was 74%. Forty-nine percent of patients had normal pulmonary artery pressure estimates within the first 3 weeks of life. All patients survived. Seventeen percent had persistent systemic or suprasystemic pressure estimates unrelieved by treatment interventions resulting in 100% mortality rate. Thirty-four percent had intermediate reductions in pressure estimates over time with 75% survival rate. Systemic pulmonary artery pressures were associated with decreased survival at all time-points when compared with normal-pressure survivors: week 1, 60% (P < .003); week 3, 38% (P < .007); week 6, 0% (P < .02). Conclusions: The evolution of pulmonary hypertension is a critical determinant of survival in CDH patients with current treatment strategies. Three groups can be modeled with markedly different clinical performance patterns. Using serial cardiac ECHO examinations, pulmonary artery pressure estimations can be used to predict clinical outcome.

AB - Background/Purpose: Pulmonary hypertension is an integral part of the pathophysiology of the respiratory failure associated with congenital diaphragmatic hernia. Hypothesizing that the evolution of the pulmonary hypertension would determine clinical outcome, the authors examined pulmonary artery pressures in relation to survival in their CDH patients. Methods: The authors performed a retrospective chart review of all neonates with the CDH from 1991 to 2002 at their institution eliminating infants with complex congenital heart disease, prematurity, or limited treatment. Cardiac ECHO data were used to estimate pulmonary artery pressures as a ratio to systemic pressure. Statistical analyses of estimated pulmonary pressure ratios stratified by survival status and time were performed using X2 and Fisher's Exact Test methods. Results: Forty-seven full-term CDH infants with 428 cardiac ECHO evaluations were studied. Long-term survival rate was 74%. Forty-nine percent of patients had normal pulmonary artery pressure estimates within the first 3 weeks of life. All patients survived. Seventeen percent had persistent systemic or suprasystemic pressure estimates unrelieved by treatment interventions resulting in 100% mortality rate. Thirty-four percent had intermediate reductions in pressure estimates over time with 75% survival rate. Systemic pulmonary artery pressures were associated with decreased survival at all time-points when compared with normal-pressure survivors: week 1, 60% (P < .003); week 3, 38% (P < .007); week 6, 0% (P < .02). Conclusions: The evolution of pulmonary hypertension is a critical determinant of survival in CDH patients with current treatment strategies. Three groups can be modeled with markedly different clinical performance patterns. Using serial cardiac ECHO examinations, pulmonary artery pressure estimations can be used to predict clinical outcome.

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