PMID- 23339456
OWN - NLM
STAT- MEDLINE
DCOM- 20130711
LR  - 20211021
IS  - 1465-993X (Electronic)
IS  - 1465-9921 (Print)
IS  - 1465-9921 (Linking)
VI  - 14
IP  - 1
DP  - 2013 Jan 22
TI  - Loss of alveolar membrane diffusing capacity and pulmonary capillary blood volume 
      in pulmonary arterial hypertension.
PG  - 6
LID - 10.1186/1465-9921-14-6 [doi]
AB  - BACKGROUND: Reduced gas transfer in patients with pulmonary arterial hypertension 
      (PAH) is traditionally attributed to remodeling and progressive loss of pulmonary 
      arterial vasculature that results in decreased capillary blood volume available 
      for gas exchange. METHODS: We tested this hypothesis by determination of lung 
      diffusing capacity (DL) and its components, the alveolar capillary membrane 
      diffusing capacity (D(m)) and lung capillary blood volume (V(c)) in 28 
      individuals with PAH in comparison to 41 healthy individuals, and in 19 PAH 
      patients over time. Using single breath simultaneous measure of diffusion of 
      carbon monoxide (DL(CO)) and nitric oxide (DL(NO)), DL and D(m) were respectively 
      determined, and V(c) calculated. D(m) and V(c) were evaluated over time in 
      relation to standard clinical indicators of disease severity, including brain 
      natriuretic peptide (BNP), 6-minute walk distance (6MWD) and right ventricular 
      systolic pressure (RVSP) by echocardiography. RESULTS: Both DL(CO) and DL(NO) 
      were reduced in PAH as compared to controls and the lower DL in PAH was due to 
      loss of both D(m) and V(c) (all p < 0.01). While DL(CO) of PAH patients did not 
      change over time, DL(NO) decreased by 24 ml/min/mmHg/year (p = 0.01). 
      Consequently, D(m) decreased and V(c) tended to increase over time, which led to 
      deterioration of the D(m)/V(c) ratio, a measure of alveolar-capillary membrane 
      functional efficiency without changes in clinical markers. CONCLUSIONS: The 
      findings indicate that lower than normal gas transfer in PAH is due to loss of 
      both D(m) and V(c), but that deterioration of D(m)/V(c) over time is related to 
      worsening membrane diffusion.
FAU - Farha, Samar
AU  - Farha S
AD  - Respiratory Institute, Cleveland Clinic, Cleveland, OH 44195, USA. farhas@ccf.org
FAU - Laskowski, Daniel
AU  - Laskowski D
FAU - George, Deepa
AU  - George D
FAU - Park, Margaret M
AU  - Park MM
FAU - Tang, W H Wilson
AU  - Tang WH
FAU - Dweik, Raed A
AU  - Dweik RA
FAU - Erzurum, Serpil C
AU  - Erzurum SC
LA  - eng
GR  - R37 HL060917/HL/NHLBI NIH HHS/United States
GR  - U10 HL109250/HL/NHLBI NIH HHS/United States
GR  - UL1 TR000439/TR/NCATS NIH HHS/United States
GR  - HL60917/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20130122
PL  - England
TA  - Respir Res
JT  - Respiratory research
JID - 101090633
SB  - IM
MH  - Adult
MH  - *Blood Volume
MH  - Capillaries/*physiopathology
MH  - Diffusion
MH  - Familial Primary Pulmonary Hypertension
MH  - Female
MH  - Humans
MH  - Hypertension, Pulmonary/*physiopathology
MH  - Male
MH  - Pulmonary Alveoli/chemistry/*physiopathology
MH  - *Pulmonary Diffusing Capacity
PMC - PMC3560152
EDAT- 2013/01/24 06:00
MHDA- 2013/07/13 06:00
PMCR- 2013/01/22
CRDT- 2013/01/24 06:00
PHST- 2012/10/23 00:00 [received]
PHST- 2013/01/17 00:00 [accepted]
PHST- 2013/01/24 06:00 [entrez]
PHST- 2013/01/24 06:00 [pubmed]
PHST- 2013/07/13 06:00 [medline]
PHST- 2013/01/22 00:00 [pmc-release]
AID - 1465-9921-14-6 [pii]
AID - 10.1186/1465-9921-14-6 [doi]
PST - epublish
SO  - Respir Res. 2013 Jan 22;14(1):6. doi: 10.1186/1465-9921-14-6.