Pulmonary function studies as an aid in the differential diagnosis of pulmonary hypertension

Williams, M. Henry; Adler, Jack J.; Colp, Charlotte

doi:10.1016/0002-9343(69)90221-6

Cited by 26 publications

(6 citation statements)

References 14 publications

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“…The very abnormal gas transfer coefficient is much lower than is normally seen in patients with primary pulmonary hypertension, and is more compatible with the proven vasculitis [9,10]. The fact that the patient subsequently developed evidence of a cutaneous vasculitis supports the hypothesis that the pulmonary hypertension was secondary to rheumatoid vasculitis.…”

Section: Discussionmentioning

confidence: 52%

Fatal pulmonary hypertension and rheumatoid vasculitis

Balagopal

Costa²,

Greenstone³

1995

Eur Respir J

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Section: Discussionmentioning

confidence: 52%

Fatal pulmonary hypertension and rheumatoid vasculitis

Balagopal

Costa²,

Greenstone³

1995

Eur Respir J

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“…Williams et al [5] suggested that pulmonary function tests may be of value in differentiating pulmo nary hypertension due to fibrotic pulmo nary disease from primary disease of the pulmonary vasculature. These authors found that patients with pulmonary fibro sis had marked reductions of vital capac ity, total lung capacity, and DLco.…”

Section: Discussionmentioning

confidence: 99%

“…The presence of Kerley B lines, pleural effusion, and patchy irregularities on a standard chest roentgenogram and in homogeneity of the pulmonary perfusion scan represent clues to the diagnosis of pulmonary veno-occlusive disease [2][3][4][5],…”

Section: Introductionmentioning

confidence: 99%

“…Williams et al [5] suggested that severe impairment of carbon monoxide diffusion (D|.to ) in dicates the presence of interstitial pulmo nary fibrosis causing pulmonary hyper tension. A recent study of 187 patients with primary pulmonary hypertension re ported that the mean DL{0 for these pa tients was 69% of predicted [6], The DLco also remains normal or mildly reduced for patients with chronic thrombotic occlu sion of the pulmonary arteries [7,8], These studies suggest that DLco can differentiate pulmonary hypertension caused by inter stitial lung diseases from pulmonary hy pertension caused by pulmonary vascular disease.…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary Veno-Occlusive Disease Associated with Severe Reduction of Single-Breath Carbon Monoxide Diffusing Capacity

Tv²,

Hill

1988

Respiration

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A 49-year-old woman presented with pulmonary hypertension, profound arterial hypoxemia, and a single-breath carbon monoxide diffusing capacity (D_Lco) which was 17% of predicted. History, physical examination, and chest roentgenograms did not suggest the presence of parenchymal pulmonary disease. Spirometry and lung volume measurements were within normal limits. Pulmonary veno-occlusive disease was diagnosed by lung biopsy. This case illustrates the severe reduction of D_Lco which can be associated with pulmonary veno-occlusive disease.

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“…This finding is in keeping with previous reports of patients with pulmonary hypertension secondary to pulmonary vascular disease, and contrasts with the severe impairment of diffusion capacity seen in patients with pulmonary hypertension resulting from parenchymal lung disease.' 5 Arterial hypoxaemia was almost invariably present (mean Pao2 58 mm Hg (7-7 kPa)) and Pao2 was greater than 80 mm Hg (107 kPa) in only 13% of cases. Right to left intracardiac shunting is the obvious explanation for hypoxaemia in the group with Eisenmenger physiology.…”

Section: Methodsmentioning

confidence: 95%

Pulmonary function in advanced pulmonary hypertension

1987

Journal of Cardiothoracic Anesthesia

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Pulmonary mechanical function and gas exchange were studied in 33 The availability of human heart-lung transplantation at our institution has led to the referral of a large number of patients with advanced pulmonary hypertension for consideration of surgery. So far 33 patients suffering from primary pulmonary hypertension or pulmonary hypertension secondary to congenital heart disease with Eisenmenger physiology have been studied in our pulmonary function laboratory (data from nine of them have been reported' but are included here and expanded to provide a more complete evaluation). Previous reports of similar patients have been limited to single case studies or small groups and have shown disparate results'-6; isolated reports of a restrictive ventilatory defect3 and patients with airflow obstruction2 have appeared.Given this background, a larger series such as this one, in which patients were comprehensively studied in the same laboratory with a uniform protocol, should provide a more accurate assessment of lung function in patients with pulmonary hypertension. MethodsThirty three patients suffering from advanced (stage IV, New York Heart Association) pulmonary vascular disease resulting from primary pulmonary hypertension (18 cases) and from congenital heart disease with Eisenmenger physiology (15 cases) were considered suitable for heart-lung transplantation after examination by at least two independent physicians who agreed that life expectancy in each case was probably less than 12 months. Cardiac catheterisation and pulmonary angiography were performed before referral in all cases. These were not repeated here in view of the associated morbidity and mortality. All clinical data, however, were reviewed in detail and patients with known causes of pulmonary hypertension other than congenital heart disease were excluded.Ages ranged from 17 to 45 with a mean of 32 years, and there were 17 men and 16 women. Almost half of the patients were smokers, with a mean smoking history of 18 pack years. No patient had a history of asthma, and blood eosinophil counts were consistently within normal limits.Pulmonary function was assessed by standard methods.' In brief, lung volumes, flow rates, 131

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Pulmonary function studies as an aid in the differential diagnosis of pulmonary hypertension

Cited by 26 publications

References 14 publications

Fatal pulmonary hypertension and rheumatoid vasculitis

Fatal pulmonary hypertension and rheumatoid vasculitis

Pulmonary Veno-Occlusive Disease Associated with Severe Reduction of Single-Breath Carbon Monoxide Diffusing Capacity

Pulmonary function in advanced pulmonary hypertension

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