Alveolar capillary dysplasia: Diagnostic potential for cardiac catheterization

被引：12

作者：

Hintz S.R. ^{[1
,7
]}

Vincent J.A. ^{[3
]}

Pitlick P.T. ^{[2
]}

Fineman J.R. ^{[4
]}

Steinhorn R.H. ^{[5
]}

Kim G.E. ^{[6
]}

Benitz W.E. ^{[1
]}

机构：

[1] Div. of Neonatal and Devmtl. Med., Department of Pediatrics, Stanford Univ. School of Medicine, Palo Alto, CA

[2] Division of Cardiology, Department of Pediatrics, Stanford Univ. School of Medicine, Palo Alto, CA

[3] Department of Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI

[4] Division of Critical Care Medicine, Department of Pediatrics, University of California, San Francisco, CA

[5] Division of Neonatology, Children's Memorial Hospital, Northwestern University, Chicago, IL

[6] Department of Pathology, Stanford Universtty, School of Medicine, Palo Alto, CA

[7] Div. of Neonatal and Devmtl. Med., Stanford University, Palo Alto, CA 94304, 750 Welch Koad

来源：

Journal of Perinatology | 1999年 / 19卷 / 6期

关键词：

D O I：

10.1038/sj.jp.7200243

中图分类号：

学科分类号：

摘要：

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.

引用

页码：441 / 446

页数：5

共 15 条

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