The Familial Occurrence of Primary Pulmonary Hypertension

doi:10.1059/0003-4819-65-4-672

The Familial Occurrence of Primary Pulmonary Hypertension

JAMES D. ROGGE;
MARVIN E. MISHKIN; and
PASQUALE D. GENOVESE, M.D., F.A.C.P.
Indianapolis, Indiana

CAPT., MC, USAF
CAPT., MC, USAF

Requests for reprints should be addressed to Pasquale D. Genovese, M.D., Veterans Administration Hospital,
1481 W. 10th St., Indianapolis, Ind. 46207
.

Excerpt

Primary pulmonary hypertension is a disease characterized by increased arterial pressure in the pulmonary circulation caused by changes which appear to be intrinsic to the pulmonary vasculature. The pathology of these changes has been described for some time and has been discussed by several authors (1-5). Although many theories about the cause of these changes have been proposed, none have been proved. Because of this lack of a proved pathogenic mechanism, some authors (6-8) have used the term "idiopathic," instead of primary pulmonary hypertension.

The clinical features of the disease have been so well described that some people feel the

Acknowledgment

The authors wish to acknowledge the assistance of David E. Rosenbaum, M.D. and Lall G. Montgomery, M.D. for their assistance with the autopsy material.

Summario in Interlingua

Primari hypertension pulmonar es un morbo characterisate per elevate nivellos de tension arterial in le circulation pulmonar causate per alterationes apparentemente intrinsec in le vasculatura pulmonar. Le pathogenese del morbo non es clar. Un characteristica de primari hypertension pulmonar, le qual ha non essite commentate extensemente, es su occurrentia occasional in forma familial.

Es describite un familia in que duo subjectos ha habite le morbo demonstratemente durante que un tertie es suspicite de haber lo. In un del afficite subjectos, un scrutinio pulmonar esseva executate con macroaggregatos de albumina marcate con radio-iodo, resultante in constatationes normal. Le secunde afficite subjecto esseva un femina qui moriva durante le administration de anesthesia in le parturition de su secunde infante. Su prime infante ha monstrate anormalitates roentgenographic, sed su tension pulmono-arterial es currentemente normal. Il es possibile que iste patiente ha precoce primari hypertension pulmonar con alternante periodos de tono normal e augmentate in le vasculatura pulmonar. Sex altere membros del familia esseva studiate, sed omne istes esseva sin evidentia del morbo.

Es revistate le litteratura concernite con le occurrentia familial de primari hypertension pulmonar. Plures del aspectos interessante e inusual del morbo es commentate.

Article and Author Information

From the Medical Service, Veterans Administration Hospital, and the Department of Medicine, Indiana University School of Medicine, Indianapolis, Ind.
This work was supported in part with facilities provided by grant HE 06308, National Heart Institute, U. S. Public Health Service Postgraduate Research Training grant HTS-5363, National Heart Institute, and the Indiana Heart Association.
Dr. Mishkin performed this work during his tenure as a U. S. Public Health Service Fellow.
- Received March 23, 1966.
- Accepted April 25, 1966.