In Utero Development of Hypertensive Necrotizing Pulmonary Arterial Lesions: Report of a Case Associated with Premature Closure of the Ductus Arteriosus and Pulmonary Hypoplasia

Oyer, Calvin E; Feit, Lloyd R; Rogers, Beverly B; Kuhn, Charles

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Volume 9, Issue 1 , Pages 39-47, January 2000

In Utero Development of Hypertensive Necrotizing Pulmonary Arterial Lesions:

Report of a Case Associated with Premature Closure of the Ductus Arteriosus and Pulmonary Hypoplasia

Calvin E Oyer, MD
- Departments of Pathology, Women and Infants' Hospital and Brown University School of Medicine, Providence, RI, USA
- Address for correspondence: Calvin E. Oyer, MD, Department of Pathology, Women and Infants' Hospital, Providence, RI 02906, USA. Tel: (401) 831-2465; Fax: (401) 274-5519
Lloyd R Feit, MD
- Departments of Pediatrics, Women and Infants' Hospital and Brown University School of Medicine, Providence, RI, USA
Beverly B Rogers, MD
- Department of Pathology, Children's Medical Center and Southwestern Medical School, University of Texas, Dallas, TX, USA
Charles Kuhn, MD
- Department of Pathology, Memorial Hospital of Rhode Island, Pawtucket, RI, USA
- Department of Pathology, Brown University School of Medicine, Providence, RI, USA

Received 8 July 1999; accepted 23 September 1999.

Abstract

Premature closure of the ductus arteriosus (PCDA) is an uncommon defect in which pulmonary hypertension (PH) has been documented by echocardiography in patients and by direct measurement after experimental PCDA in animals. The pulmonary vascular histology in human cases has received little attention but in the few recorded observations the vessels were either normal or showed increased muscularity. We report the case of a 31 week hydropic female stillborn monozygotic twin in whom postmortem examination disclosed PCDA and hypoplasia of the lungs. Atypical plexiform lesions with necrotizing pulmonary arteritis were present. These lesions represent vascular consequences of severe pulmonary hypertension produced by greatly enhanced blood flow through a restricted vascular bed resulting from the combined effects of these two abnormalities. The findings in this case of PCDA with presumed severe PH indicate that severe pulmonary vascular changes can develop in utero and that the interval of time needed for development of such changes in secondary PH is relatively short.