PMID- 8641032
OWN - NLM
STAT- MEDLINE
DCOM- 19960717
LR  - 20190623
IS  - 0009-7322 (Print)
IS  - 0009-7322 (Linking)
VI  - 93
IP  - 7
DP  - 1996 Apr 1
TI  - Isolated peripheral pulmonary artery stenoses in the adult.
PG  - 1417-23
AB  - BACKGROUND: Isolated peripheral pulmonary artery stenosis (PPS) in the adult is 
      rare and frequently unsuspected. We review in this article our experience with 12 
      adult patients with isolated PPS, half of whom had been previously diagnosed with 
      chronic pulmonary thromboembolic disease. METHODS AND RESULTS: The presentation, 
      evolution, and management of 12 adults with isolated PPS, 17 to 51 years of age 
      (mean, 36.2 +/- 9.7 years), were evaluated. Presenting symptoms were dyspnea and 
      fatigue. Three patients had New York Heart Association (NYHA) functional class 
      III or greater. Lung perfusion scans revealed multiple segmental abnormalities in 
      flow distribution in all patients. Oxygen desaturation at rest was present in 4 
      patients. At catheterization, right ventricular (RV) pressure was suprasystemic 
      in 2 patients, systemic in 1, and more than half-systemic in 7. All had multiple 
      bilateral non-uniform stenoses in segmental and subsegmental arteries. Balloon 
      pulmonary angioplasty (BPA) to decrease RV hypertension and improve pulmonary 
      flow distribution was performed in 11 patients. After BPA, vessel diameter 
      increased > 50% in 10 patients, distal pulmonary artery pressure increased > or = 
      30% in 6, and RV pressure decreased > 30% in 5. One patient died shortly after 
      BPA as a result of pulmonary hemorrhage. Immediate procedural success was 
      achieved in 9 of 11 patients. At a mean follow-up period of 52 +/- 32 months, 7 
      patients had sustained symptomatic improvement (NYHA class I-II). CONCLUSIONS: We 
      describe a severe syndrome of isolated PPS in the adult that mimics chronic 
      pulmonary thromboembolic disease. Pulmonary hemodynamics and angiography are 
      required for definitive diagnosis. BPA may offer these patients successful 
      short-term reduction in RV hypertension and alleviation of symptomatology.
FAU - Kreutzer, J
AU  - Kreutzer J
AD  - Department of Cardiology, Children's Hospital, Boston MA 02115, USA.
FAU - Landzberg, M J
AU  - Landzberg MJ
FAU - Preminger, T J
AU  - Preminger TJ
FAU - Mandell, V S
AU  - Mandell VS
FAU - Treves, S T
AU  - Treves ST
FAU - Reid, L M
AU  - Reid LM
FAU - Lock, J E
AU  - Lock JE
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Circulation
JT  - Circulation
JID - 0147763
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age of Onset
MH  - Angioplasty, Balloon
MH  - Cardiac Catheterization
MH  - Dyspnea/etiology
MH  - Fatigue/etiology
MH  - Female
MH  - Follow-Up Studies
MH  - Hemodynamics
MH  - Humans
MH  - Hypertrophy, Right Ventricular/etiology/pathology
MH  - Male
MH  - Middle Aged
MH  - Pulmonary Artery/diagnostic imaging/embryology/pathology
MH  - Pulmonary Valve Stenosis/complications/diagnosis/*epidemiology/therapy
MH  - Radiography
MH  - Ultrasonography
RF  - 49
EDAT- 1996/04/01 00:00
MHDA- 1996/04/01 00:01
CRDT- 1996/04/01 00:00
PHST- 1996/04/01 00:00 [pubmed]
PHST- 1996/04/01 00:01 [medline]
PHST- 1996/04/01 00:00 [entrez]
AID - 10.1161/01.cir.93.7.1417 [doi]
PST - ppublish
SO  - Circulation. 1996 Apr 1;93(7):1417-23. doi: 10.1161/01.cir.93.7.1417.