Alveolar capillary dysplasia: Diagnostic potential for cardiac catheterization

Susan R. Hintz, Julie A. Vincent, Paul T. Pitlick, Jeffrey R. Fineman, Robin H Steinhorn, Grace E. Kim, William E. Benitz

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

OBJECTIVE: Alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Infants with this condition die despite maximal medical intervention including inhaled nitric oxide therapy and extracorporeal membrane oxygenation. To date, diagnosis of this lethal condition was made by open lung biopsy or during postmortem examination. We examined the possibility that distinct cardiac catheterization findings could be used in the diagnosis of this lethal disorder. STUDY DESIGN: We present three infants with fatal persistent pulmonary hypertension of the newborn refractory to extracorporeal membrane oxygenation and inhaled nitric oxide therapy, two with postmortem autopsy confirmation of alveolar capillary dysplasia. Each infant underwent cardiac catheterization to complete the diagnostic evaluations. RESULTS: Significant right ventricular hypertension and normal pulmonary venous return were demonstrated, but a markedly diminished or absent capillary blush phase was noted in each infant. This finding is distinct from the normal capillary blush seen in infants with persistent pulmonary hypertension of the newborn of other etiologies. CONCLUSION: Cardiac catheterization may provide a useful alternative to tissue examination in the diagnosis of alveolar capillary dysplasia.

Original languageEnglish (US)
Pages (from-to)441-446
Number of pages6
JournalJournal of Perinatology
Volume19
Issue number6 PART. 1
StatePublished - Sep 1999
Externally publishedYes

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Cardiac Catheterization
Persistent Fetal Circulation Syndrome
Extracorporeal Membrane Oxygenation
Autopsy
Nitric Oxide
Pulmonary Hypertension
Alveolar capillary dysplasia
Biopsy
Lung
Therapeutics

ASJC Scopus subject areas

  • Obstetrics and Gynecology
  • Pediatrics, Perinatology, and Child Health

Cite this

Hintz, S. R., Vincent, J. A., Pitlick, P. T., Fineman, J. R., Steinhorn, R. H., Kim, G. E., & Benitz, W. E. (1999). Alveolar capillary dysplasia: Diagnostic potential for cardiac catheterization. Journal of Perinatology, 19(6 PART. 1), 441-446.

Alveolar capillary dysplasia : Diagnostic potential for cardiac catheterization. / Hintz, Susan R.; Vincent, Julie A.; Pitlick, Paul T.; Fineman, Jeffrey R.; Steinhorn, Robin H; Kim, Grace E.; Benitz, William E.

In: Journal of Perinatology, Vol. 19, No. 6 PART. 1, 09.1999, p. 441-446.

Research output: Contribution to journalArticle

Hintz, SR, Vincent, JA, Pitlick, PT, Fineman, JR, Steinhorn, RH, Kim, GE & Benitz, WE 1999, 'Alveolar capillary dysplasia: Diagnostic potential for cardiac catheterization', Journal of Perinatology, vol. 19, no. 6 PART. 1, pp. 441-446.
Hintz SR, Vincent JA, Pitlick PT, Fineman JR, Steinhorn RH, Kim GE et al. Alveolar capillary dysplasia: Diagnostic potential for cardiac catheterization. Journal of Perinatology. 1999 Sep;19(6 PART. 1):441-446.
Hintz, Susan R. ; Vincent, Julie A. ; Pitlick, Paul T. ; Fineman, Jeffrey R. ; Steinhorn, Robin H ; Kim, Grace E. ; Benitz, William E. / Alveolar capillary dysplasia : Diagnostic potential for cardiac catheterization. In: Journal of Perinatology. 1999 ; Vol. 19, No. 6 PART. 1. pp. 441-446.
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