Survival in Patients with Primary Pulmonary Hypertension

D’Alonzo, Gilbert E.; Barst, Robyn J.; Ayres, Stephen M.; Bergofsky, Edward H.; Brundage, Bruce H.; Detre, Katherine M.; Fishman, Alfred P.; Goldring, Roberta M.; Groves, Berton M.; Kernis, Janet T.; Levy, Paul S.; Pietra, Giuseppe G.; Reid, Lynne; Reeves, John Τ.; Rich, Stuart; Vreim, Carol E.; Williams, George W.; Wu, Margaret

doi:10.7326/0003-4819-115-5-343

Cited by 3,193 publications

(2,329 citation statements)

References 21 publications

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“…arrhythmia and left ventricular dysfunction caused by LMCA compression may contribute to a higher incidence of sudden death in patients with pulmonary hypertension. 8,9 In one report, a patient with extrinsic LMCA compression suffered from a non-Q wave myocardial infarction, 1 despite management with nifedipine and nitrates, and died from cardiogenic shock 2 days after presentation. Other reports have not demonstrated significant ischemia in patients similar to our patient with pulmonary hypertension and LMCA compression.…”

Section: Discussionmentioning

confidence: 99%

Extrinsic Compression of Left Main Coronary Artery by the Pulmonary Trunk Secondary to Pulmonary Hypertension Documented Using 64‐Slice Multidetector Computed Tomography Coronary Angiography

Safi

Eslami

Shabestari

et al. 2009

Clinical Cardiology

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Compression of left main coronary artery (LMCA) secondary to pulmonary trunk dilatation is a newly recognized entity that has been associated with severe pulmonary hypertension. In this paper we present a case of extrinsic compression of LMCA caused by dilated pulmonary trunk secondary to pulmonary hypertension documented using 64-slice multidetector computed tomography (MDCT) coronary angiography. This case is followed by a review of the literature. IntroductionThe extrinsic compression of left main coronary artery (LMCA) secondary to pulmonary artery trunk dilatation is a relatively newly described syndrome that has been associated with severe pulmonary hypertension. It is primarily related to congenital heart disease or idiopathic pulmonary hypertension 1 -6 but other causes of pulmonary hypertension can be the triggering factor for this syndrome. The following case is an example of extrinsic LMCA compression caused by secondary pulmonary hypertension related to chronic sarcoidosis and pulmonary embolism.

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Section: Discussionmentioning

confidence: 99%

Extrinsic Compression of Left Main Coronary Artery by the Pulmonary Trunk Secondary to Pulmonary Hypertension Documented Using 64‐Slice Multidetector Computed Tomography Coronary Angiography

Safi

Eslami

Shabestari

et al. 2009

Clinical Cardiology

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“…The plexogenic form occurs in 28±80% of cases of PPH [5,6], and is also found in patients with chronic thromboembolic pulmonary hypertension (CT-EPH) [7]. Generally, PPH has a poor prognosis with a median survival of 3±4 yrs from the time of diagnosis [8,9].…”

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confidence: 99%

“…Generally, a gas exchange impairment exists in PPH patients with hypoxaemia and hypocapnia. The transfer factor of the lung for carbon monoxide (TL,CO) is generally reduced to~70% of the predicted value [8,11]. No association between TL,CO and the severity of PPH has been found [10].…”

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confidence: 99%

Diffusion capacity and haemodynamics in primary and chronic thromboembolic pulmonary hypertension

Steenhuis¹,

Groen²,

Koeter³

et al. 2000

Eur Respir J

100

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The transfer factor of the lung for carbon monoxide (TL,CO) is decreased in patients with pulmonary hypertension. The pulmonary membrane diffusion capacity (Dm) and pulmonary capillary blood volume (Vc), were studied to establish: 1) the relative contribution of the components of the transfer factor to the decrease in TL,CO; 2) whether differences exist between primary pulmonary hypertension (PPH) and chronic thromboembolic pulmonary hypertension (CTEPH); and 3) the relationship between these parameters and haemodynamic parameters.Dm and Vc were determined in 19 patients with PPH and in eight patients with CTEPH. The patients had been referred for consideration for lung transplantation. Haemodynamic parameters were assessed by heart catheterization.In the PPH group, Vc was reduced in 12 of 19 patients (mean SD Vc 72 14% of the predicted value) and Dm in 17 of 19 patients (60 22% pred). In the CTEPH group, Vc was reduced in six of eight patients and Dm in seven of eight patients. The mean TL,CO Dm and Vc values were similar to those in the PPH group.The reduction in pulmonary membrane diffusion capacity was significantly greater than that in pulmonary capillary blood volume. No differences in pulmonary and cardiovascular functional values were found between the groups. Right atrial pressure showed a significant negative correlation with pulmonary capillary blood volume and an increased pulmonary vascular resistance was associated with a decrease in pulmonary membrane diffusion capacity. These results suggest pronounced functional impairment of the alveolocapillary membrane in these patients. Eur Respir J 2000; 16: 276±281.

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“…The median survival in 1980s was reported as 2.8 years in 194 patients with iPAH after the diagnosis was made. 16 The actuarial survival rates were 68-77% at 1 year, 40-56% at 3 years and 22-38% at 5 years in two different studies. 16,17 At that time most patient received conventional therapy including oral anticoagulants, diuretics, oxygen, cardiac glycosides, and calcium channel blockers.…”

Section: Natural Historymentioning

confidence: 92%

“…16 The actuarial survival rates were 68-77% at 1 year, 40-56% at 3 years and 22-38% at 5 years in two different studies. 16,17 At that time most patient received conventional therapy including oral anticoagulants, diuretics, oxygen, cardiac glycosides, and calcium channel blockers. Poor prognosis was associated with New York Heart Association (NYHA) functional class III or IV, right heat failure, elevated right atrial pressure, decreased cardiac output or low mixed venous saturation.…”

Section: Natural Historymentioning

confidence: 92%

Pulmonary hypertension—“state of the art” management in 2012

Saxena

2012

Indian Heart Journal

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A B S T R A C TPulmonary artery hypertension (PAH) is a pathological condition of small pulmonary arteries, characterised by vascular proliferation and remodelling. The pulmonary artery pressure and pulmonary vascular resistance progressively rise, leading to right heart failure and death. Pulmonary artery hypertension may be secondary to various conditions, or it may be idiopathic where no underlying cause is identifiable. Earlier, only symptomatic treatment was available for such patients which did not change the natural history of the disease. However, over the years, improvement in understanding the pathogenesis has resulted in the development of targeted approaches to the treatment of PAH. Survival advantage has also been shown with some of the pharmacologic agents. This review article discusses the current management strategy for PAH with special emphasis on an idiopathic variety, in an Indian context.

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Survival in Patients with Primary Pulmonary Hypertension

Cited by 3,193 publications

References 21 publications

Extrinsic Compression of Left Main Coronary Artery by the Pulmonary Trunk Secondary to Pulmonary Hypertension Documented Using 64‐Slice Multidetector Computed Tomography Coronary Angiography

Extrinsic Compression of Left Main Coronary Artery by the Pulmonary Trunk Secondary to Pulmonary Hypertension Documented Using 64‐Slice Multidetector Computed Tomography Coronary Angiography

Diffusion capacity and haemodynamics in primary and chronic thromboembolic pulmonary hypertension

Pulmonary hypertension—“state of the art” management in 2012

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