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Sample records for resistance pulmonary arteries

  1. Vascular narrowing in pulmonary arterial hypertension is heterogeneous: rethinking resistance

    NARCIS (Netherlands)

    Rol, N.; Timmer, E.M.; Faes, T.J.; Noordegraaf, A.V.; Grunberg, K.; Bogaard, H.J.; Westerhof, N.

    2017-01-01

    In idiopathic pulmonary arterial hypertension (PAH), increased pulmonary vascular resistance is associated with structural narrowing of small (resistance) vessels and increased vascular tone. Current information on pulmonary vascular remodeling is mostly limited to averaged increases in wall

  2. Pulmonary arterial dysfunction in insulin resistant obese Zucker rats

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    Cogolludo Angel

    2011-04-01

    Full Text Available Abstract Background Insulin resistance and obesity are strongly associated with systemic cardiovascular diseases. Recent reports have also suggested a link between insulin resistance with pulmonary arterial hypertension. The aim of this study was to analyze pulmonary vascular function in the insulin resistant obese Zucker rat. Methods Large and small pulmonary arteries from obese Zucker rat and their lean counterparts were mounted for isometric tension recording. mRNA and protein expression was measured by RT-PCR or Western blot, respectively. KV currents were recorded in isolated pulmonary artery smooth muscle cells using the patch clamp technique. Results Right ventricular wall thickness was similar in obese and lean Zucker rats. Lung BMPR2, KV1.5 and 5-HT2A receptor mRNA and protein expression and KV current density were also similar in the two rat strains. In conductance and resistance pulmonary arteries, the similar relaxant responses to acetylcholine and nitroprusside and unchanged lung eNOS expression revealed a preserved endothelial function. However, in resistance (but not in conductance pulmonary arteries from obese rats a reduced response to several vasoconstrictor agents (hypoxia, phenylephrine and 5-HT was observed. The hyporesponsiveness to vasoconstrictors was reversed by L-NAME and prevented by the iNOS inhibitor 1400W. Conclusions In contrast to rat models of type 1 diabetes or other mice models of insulin resistance, the obese Zucker rats did not show any of the characteristic features of pulmonary hypertension but rather a reduced vasoconstrictor response which could be prevented by inhibition of iNOS.

  3. Pulmonary arterial hypertension : an update

    NARCIS (Netherlands)

    Hoendermis, E. S.

    2011-01-01

    Pulmonary arterial hypertension (PAH), defined as group 1 of the World Heart Organisation (WHO) classification of pulmonary hypertension, is an uncommon disorder of the pulmonary vascular system. It is characterised by an increased pulmonary artery pressure, increased pulmonary vascular resistance

  4. Measurement of pulmonary vascular resistance of Fontan candidates with pulmonary arterial distortion by means of pulmonary perfusion imaging

    International Nuclear Information System (INIS)

    Park, In-Sam; Mizukami, Ayumi; Tomimatsu, Hirofumi; Kondou, Chisato; Nakanishi, Toshio; Nakazawa, Makoto; Momma, Kazuo

    1998-01-01

    We measured the distribution of blood flow to the right (R) and left lung (L) by means of pulmonary perfusion imaging and calculated pulmonary vascular resistance (Rp) in 13 patients, whose right and left pulmonary artery pressures were different by 2 to 9 mmHg due to pulmonary arterial distortion (5 interruption, 8 stenosis). The right lung/left lung blood flow ratio was determined and from the ratio and the total pulmonary blood flow, which was determined using the Fick's principle, the absolute values of right and left pulmonary blood flow were calculated. Using the right and left pulmonary blood flow and the right and left pulmonary arterial pressures, right and left pulmonary vascular resistance were calculated, separately. Vascular resistance of the whole lung (Rp) was then calculated using the following equation. 1/(Rp of total lung)=1/(Rp of right lung)+1/(Rp of left lung). Rp calculated from this equation was 1.8+/-0.8 U·m 2 and all values were less than 3 U·m 2 (range 0.3-2.8). Rp estimated from the conventional method using the total pulmonary blood flow and pulmonary arterial pressures, without using the right/left blood flow ratio, ranging from 0.4 to 3.8 U·m 2 and 5 of 13 patients showed Rp>3 U·m 2 . All patients underwent Fontan operation successfully. These data indicated that this method is useful to estimate Rp and to determine the indication of Fontan operation in patients with pulmonary arterial distortions. (author)

  5. The relationship of pulmonary vascular resistance and compliance to pulmonary artery wedge pressure during submaximal exercise in healthy older adults.

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    Wright, Stephen P; Granton, John T; Esfandiari, Sam; Goodman, Jack M; Mak, Susanna

    2016-06-15

    A consistent inverse hyperbolic relationship has been observed between pulmonary vascular resistance and compliance, although changes in pulmonary artery wedge pressure (PAWP) may modify this relationship. This relationship predicts that pulmonary artery systolic, diastolic and mean pressure maintain a consistent relationship relative to the PAWP. We show that, in healthy exercising human adults, both pulmonary vascular resistance and compliance decrease in relation to exercise-associated increases in PAWP. Pulmonary artery systolic, diastolic and mean pressures maintain a consistent relationship with one another, increasing linearly with increasing PAWP. Increases in PAWP in the setting of exercise are directly related to a decrease in pulmonary vascular compliance, despite small decreases in pulmonary vascular resistance, thereby increasing the pulsatile afterload to the right ventricle. The resistive and pulsatile components of right ventricular afterload (pulmonary vascular resistance, Rp; compliance, Cp) are related by an inverse hyperbolic function, expressed as their product known as RpCp-time. The RpCp-time exhibits a narrow range, although it may be altered by the pulmonary artery wedge pressure (PAWP). Identifying the determinants of RpCp-time should improve our understanding of the physiological behaviour of pulmonary arterial systolic (PASP), diastolic (PADP) and mean (mPAP) pressures in response to perturbations. We examined the effect of exercise in 28 healthy non-athletic adults (55 ± 6 years) who underwent right heart catheterization to assess haemodynamics and calculate Rp and Cp. Measurements were made at rest and during two consecutive 8-10 min stages of cycle ergometry, at targeted heart-rates of 100 beats min(-1) (Light) and 120 beats min(-1) (Moderate). Cardiac output increased progressively during exercise. PASP, PADP, mPAP and PAWP increased for Light exercise, without any further rise for Moderate exercise. RpCp-time decreased for

  6. The Critical Role of Pulmonary Arterial Compliance in Pulmonary Hypertension

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    Prins, Kurt W.; Pritzker, Marc R.; Scandurra, John; Volmers, Karl; Weir, E. Kenneth

    2016-01-01

    The normal pulmonary circulation is a low-pressure, high-compliance system. Pulmonary arterial compliance decreases in the presence of pulmonary hypertension because of increased extracellular matrix/collagen deposition in the pulmonary arteries. Loss of pulmonary arterial compliance has been consistently shown to be a predictor of increased mortality in patients with pulmonary hypertension, even more so than pulmonary vascular resistance in some studies. Decreased pulmonary arterial compliance causes premature reflection of waves from the distal pulmonary vasculature, leading to increased pulsatile right ventricular afterload and eventually right ventricular failure. Evidence suggests that decreased pulmonary arterial compliance is a cause rather than a consequence of distal small vessel proliferative vasculopathy. Pulmonary arterial compliance decreases early in the disease process even when pulmonary artery pressure and pulmonary vascular resistance are normal, potentially enabling early diagnosis of pulmonary vascular disease, especially in high-risk populations. With the recognition of the prognostic importance of pulmonary arterial compliance, its impact on right ventricular function, and its contributory role in the development and progression of distal small-vessel proliferative vasculopathy, pulmonary arterial compliance is an attractive target for the treatment of pulmonary hypertension. PMID:26848601

  7. Krüppel-like Factor 5 contributes to pulmonary artery smooth muscle proliferation and resistance to apoptosis in human pulmonary arterial hypertension

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    Paulin Roxane

    2011-09-01

    Full Text Available Background Pulmonary arterial hypertension (PAH is a vascular remodeling disease characterized by enhanced proliferation of pulmonary artery smooth muscle cell (PASMC and suppressed apoptosis. This phenotype has been associated with the upregulation of the oncoprotein survivin promoting mitochondrial membrane potential hyperpolarization (decreasing apoptosis and the upregulation of growth factor and cytokines like PDGF, IL-6 and vasoactive agent like endothelin-1 (ET-1 promoting PASMC proliferation. Krüppel-like factor 5 (KLF5, is a zinc-finger-type transcription factor implicated in the regulation of cell differentiation, proliferation, migration and apoptosis. Recent studies have demonstrated the implication of KLF5 in tissue remodeling in cardiovascular diseases, such as atherosclerosis, restenosis, and cardiac hypertrophy. Nonetheless, the implication of KLF5 in pulmonary arterial hypertension (PAH remains unknown. We hypothesized that KLF5 up-regulation in PAH triggers PASMC proliferation and resistance to apoptosis. Methods and results We showed that KFL5 is upregulated in both human lung biopsies and cultured human PASMC isolated from distal pulmonary arteries from PAH patients compared to controls. Using stimulation experiments, we demonstrated that PDGF, ET-1 and IL-6 trigger KLF-5 activation in control PASMC to a level similar to the one seen in PAH-PASMC. Inhibition of the STAT3 pathway abrogates KLF5 activation in PAH-PASMC. Once activated, KLF5 promotes cyclin B1 upregulation and promotes PASMC proliferation and triggers survivin expression hyperpolarizing mitochondria membrane potential decreasing PASMC ability to undergo apoptosis. Conclusion We demonstrated for the first time that KLF5 is activated in human PAH and implicated in the pro-proliferative and anti-apoptotic phenotype that characterize PAH-PASMC. We believe that our findings will open new avenues of investigation on the role of KLF5 in PAH and might lead to the

  8. Pulmonary artery aneurysm

    African Journals Online (AJOL)

    Enrique

    Hughes-Stovin's disease,. Behcet's disease), collagen vascular diseases, connective tissue disorders,. (Marfan's syndrome, Ehler's-Danlos. 30. SA JOURNAL OF RADIOLOGY • October 2004. CASE REPORT. Pulmonary artery aneurysm.

  9. Lung irradiation induces pulmonary vascular remodelling resembling pulmonary arterial hypertension

    NARCIS (Netherlands)

    Ghobadi, G.; Bartelds, B.; van der Veen, S. J.; Dickinson, M. G.; Brandenburg, S.; Berger, R. M. F.; Langendijk, J. A.; Coppes, R. P.; van Luijk, P.

    Background Pulmonary arterial hypertension (PAH) is a commonly fatal pulmonary vascular disease that is often diagnosed late and is characterised by a progressive rise in pulmonary vascular resistance resulting from typical vascular remodelling. Recent data suggest that vascular damage plays an

  10. Perinatal development influences mechanisms of bradykinin-induced relaxations in pulmonary resistance and conduit arteries differently.

    Science.gov (United States)

    Boels, P J; Deutsch, J; Gao, B; Haworth, S G

    2001-07-01

    As bradykinin (BYK) relaxes conduit (EPA) and resistance (RPA) pulmonary arteries from both perinatal and adult lungs, we investigated whether this vasodilator's relaxation-mechanisms were altered during perinatal development, differed between EPA and RPA and differed with other endothelium-dependent vasodilators, acetyicholine (ACH) and substance P (SP). Arteries from mature foetal (5 days), neonatal (approximately 5 min), newborn (60-84 h) and adult pigs (> or =6 months) were isolated, mounted for in vitro isometric force recording, activated with PGF(2alpha) (30 micromol/l) and relaxed with BYK (10 pmol/l-1 micromol/l), SP (10 pmol/l-0.1 micromol/l) or ACH (1 nmol/l-1 mmol/l). (i) BYK: L-NAME (100 micromol/l) attenuated relaxations in foetal EPA ( approximately 55%) but nearly abolished them in the adult ( approximately 80%). In RPA, L-NAME nearly abolished ( approximately 90%) relaxations in the foetus and this effect diminished progressively with age to approximately 20% in the adult. Indomethacin (IND, micromol/l) attenuated relaxations in neonatal (approximately 25%), new-born and adult EPA (both approximately 45%). Together, L-NAME and IND abolished relaxations in all EPA and in neonatal RPA but not in older RPA. SKF525a (100 micromol/l) attenuated relaxations in foetal RPA ( approximately 4%), diminishing in the adult RPA to approximately 10%. Together, SKF52Sa and L-NAME largely abolished relaxations in postnatal RPA (approximately 80%). Activation with K(+)=125 mmol/l attenuated relaxations in adult EPA (approximately 80%), foetal RPA ( approximately 45%) and neonatal RPA (approximately 75%) and abolished relaxations in RPA from older ages. (ii) ACH: L-NAME abolished relaxations in new-born EPA and RPA. In adult EPA, combined L-NAME and IND moderately attenuated relaxations. (iii) SP: Combined application of L-NAME and IND attenuated relaxations to a similar degree in new-born and adult EPA and RPA. In postnatal EPA, BYK-relaxations depend completely on

  11. Ultrasound diagnosis of pulmonary sling with proximal stenosis of left pulmonary artery and patent arterial duct

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    Wojciech Mądry

    2013-03-01

    Full Text Available Authors discuss methods of echocardiographic diagnosis of the pulmonary sling with stenosis and hypoplasia of the left pulmonary artery and patent arterial duct with massive left‑to‑right shunt, based on a case of the newborn with resistant to treatment heart failure, with initial diagnosis of patent ductus arteriosus, referred to surgical treatment. The optimal echocardiographic views permitting establish diagnosis of the pulmonary sling were suggested. The special attention was paid to high parasternal and suprasternal views visualizing vessels of the upper mediastinum as well as characteristic differences between the normal and pathologic picture. The typical features of the echocardiogram suggesting pulmonary sling, like the lack of the left pulmonary artery in its expected position, and the abnormal branching pattern of the right pulmonary artery were indicated. The greatest diagnostic difficulties in visualization of the abnormal route of the left pulmonary artery were related to the presence of air‑containing tissues, like lungs and central airways between the ultrasound probe and area of interest. The other was the masking influence of the large patent arterial duct, that may mimic the left pulmonary artery arising from the pulmonary trunk. The other entities requiring differentiation with sling, like aplasia of the left lung, the direct or indirect aortic origin of the left pulmonary artery, were discussed. The role of other visualization technics, like computed 3D tomography, and magnetic nuclear resonance, as well as direct visualization of central airways with bronchoscopy in establishing precise diagnosis were stressed.

  12. Drugs induced pulmonary arterial hypertension.

    Science.gov (United States)

    Seferian, Andrei; Chaumais, Marie-Camille; Savale, Laurent; Günther, Sven; Tubert-Bitter, Pascale; Humbert, Marc; Montani, David

    2013-09-01

    Pulmonary arterial hypertension (PAH) is a rare disorder characterized by progressive obliteration of the pulmonary microvasculature, resulting in elevated pulmonary vascular resistance and premature death. According to the current classification, PAH can be associated with exposure to certain drugs or toxins, particularly appetite suppressant drugs, such as aminorex, fenfluramine derivatives and benfluorex. These drugs have been confirmed to be risk factors for PAH and were withdrawn from the market. The supposed mechanism is an increase in serotonin levels, which was demonstrated to act as a growth factor for the pulmonary arterial smooth muscle cells. Amphetamines, phentermine and mazindol were less frequently used but are also considered as possible risk factors for PAH. Dasatinib, a dual Src/Abl kinase inhibitor, used in the treatment of chronic myelogenous leukaemia was associated with cases of severe PAH, in part reversible after its withdrawal. Recently several studies raised the potential endothelial dysfunction that could be induced by interferon, and few cases of PAH have been reported with interferon therapy. Other possible risk factors for PAH include: nasal decongestants, like phenylpropanolamine, dietary supplement - L-Tryptophan, selective serotonin reuptake inhibitors, pergolide and other drugs that could act on 5HT2B receptors. Interestingly, PAH remains a rare complication of these drugs, suggesting possible individual susceptibility and further studies are needed to identify patients at risk of drugs induced PAH. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  13. Genetics Home Reference: pulmonary arterial hypertension

    Science.gov (United States)

    ... Home Health Conditions Pulmonary arterial hypertension Pulmonary arterial hypertension Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Pulmonary arterial hypertension is a progressive disorder characterized by abnormally high ...

  14. Pulmonary arterial hypertension

    Science.gov (United States)

    2013-01-01

    Pulmonary arterial hypertension (PAH) is a chronic and progressive disease leading to right heart failure and ultimately death if untreated. The first classification of PH was proposed in 1973. In 2008, the fourth World Symposium on PH held in Dana Point (California, USA) revised previous classifications. Currently, PH is devided into five subgroups. Group 1 includes patients suffering from idiopathic or familial PAH with or without germline mutations. Patients with a diagnosis of PAH should systematically been screened regarding to underlying mutations of BMPR2 gene (bone morphogenetic protein receptor type 2) or more rarely of ACVRL1 (activine receptor-like kinase type 1), ENG (endogline) or Smad8 genes. Pulmonary veno occusive disease and pulmonary capillary hemagiomatosis are individualized and designated as clinical group 1'. Group 2 'Pulmonary hypertension due to left heart diseases' is divided into three sub-groups: systolic dysfonction, diastolic dysfonction and valvular dysfonction. Group 3 'Pulmonary hypertension due to respiratory diseases' includes a heterogenous subgroup of respiratory diseases like PH due to pulmonary fibrosis, COPD, lung emphysema or interstitial lung disease for exemple. Group 4 includes chronic thromboembolic pulmonary hypertension without any distinction of proximal or distal forms. Group 5 regroup PH patients with unclear multifactorial mechanisms. Invasive hemodynamic assessment with right heart catheterization is requested to confirm the definite diagnosis of PH showing a resting mean pulmonary artery pressure (mPAP) of ≥ 25 mmHg and a normal pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg. The assessment of PCWP may allow the distinction between pre-capillary and post-capillary PH (PCWP > 15 mmHg). Echocardiography is an important tool in the management of patients with underlying suspicion of PH. The European Society of Cardiology and the European Respiratory Society (ESC-ERS) guidelines specify its role

  15. Lung irradiation induces pulmonary vascular remodelling resembling pulmonary arterial hypertension.

    Science.gov (United States)

    Ghobadi, G; Bartelds, B; van der Veen, S J; Dickinson, M G; Brandenburg, S; Berger, R M F; Langendijk, J A; Coppes, R P; van Luijk, P

    2012-04-01

    Pulmonary arterial hypertension (PAH) is a commonly fatal pulmonary vascular disease that is often diagnosed late and is characterised by a progressive rise in pulmonary vascular resistance resulting from typical vascular remodelling. Recent data suggest that vascular damage plays an important role in the development of radiation-induced pulmonary toxicity. Therefore, the authors investigated whether irradiation of the lung also induces pulmonary hypertension. Different sub-volumes of the rat lung were irradiated with protons known to induce different levels of pulmonary vascular damage. Early loss of endothelial cells and vascular oedema were observed in the irradiation field and in shielded parts of the lung, even before the onset of clinical symptoms. 8 weeks after irradiation, irradiated volume-dependent vascular remodelling was observed, correlating perfectly with pulmonary artery pressure, right ventricle hypertrophy and pulmonary dysfunction. The findings indicate that partial lung irradiation induces pulmonary vascular remodelling resulting from acute pulmonary endothelial cell loss and consequential pulmonary hypertension. Moreover, the close resemblance of the observed vascular remodelling with vascular lesions in PAH makes partial lung irradiation a promising new model for studying PAH.

  16. Suction/Inspiration against resistance or standardized Mueller maneuver : a new breathing technique to improve contrast density within the pulmonary artery: a pilot CT study.

    Science.gov (United States)

    Gutzeit, Andreas; Froehlich, Johannes M; Wälti, Stephan; Roos, Justus E; Meissnitzer, Matthias; Hergan, Klaus; von Weymarn, Constantin; Czell, David; Goyen, Matthias; Reischauer, Carolin

    2015-11-01

    Our aim was to prospectively investigate whether the recently introduced suction/inspiration against resistance breathing method leads to higher computed tomography (CT) contrast density in the pulmonary artery compared to standard breathing. The present study was approved by the Medical Ethics committee and all subjects gave written informed consent. Fifteen patients, each without suspicious lung emboli, were randomly assigned to four different groups with different breathing maneuvers (suction against resistance, Valsalva, inspiration, expiration) during routine CT. Contrast enhancement in the central and peripheral sections of the pulmonary artery were measured and compared with one another. Peripheral enhancement during suction yielded increased mean densities of 138.14 Hounsfield units (HU) (p = 0.001), compared to Valsalva and a mean density of 67.97 HU superior to inspiration (p = 0.075). Finally, suction in comparison to expiration resulted in a mean increase of 30.51 HU (p = 0.42). Central parts of pulmonary arteries presented significantly increased enhancement values (95.74 HU) for suction versus the Valsalva technique (p = 0.020), while all other mean densities were in favour of suction (versus inspiration: p = 0.201; versus expiration: p = 0.790) without reaching significance. Suction/Inspiration against resistance is a promising technique to improve contrast density within pulmonary vessels, especially in the peripheral parts, in comparison to other breathing maneuvers. • Suction/Inspiration against resistance is promising to improve contrast density within the pulmonary artery. • Patients potentially suffering pulmonary embolism are able to follow suction/inspiration against resistance. • Contrast density after suction is superior in comparison to other breathing maneuvers.

  17. A feasible method for non-invasive measurement of pulmonary vascular resistance in pulmonary arterial hypertension: Combined use of transthoracic Doppler-echocardiography and cardiac magnetic resonance. Non-invasive estimation of pulmonary vascular resistance.

    Science.gov (United States)

    Yan, Chaowu; Xu, Zhongying; Jin, Jinglin; Lv, Jianhua; Liu, Qiong; Zhu, Zhenhui; Pang, Kunjing; Shi, Yisheng; Fang, Wei; Wang, Yang

    2015-12-07

    Transthoracic Doppler-echocardiography (TTE) can estimate mean pulmonary arterial pressure (MPAP) and pulmonary capillary wedge pressure (PCWP) reliably, and cardiac magnetic resonance (CMR) is the best modality for non-invasive measurement of cardiac output (CO). We speculated that the combined use of TTE and CMR could provide a feasible method for non-invasive measurement of pulmonary vascular resistance (PVR) in pulmonary arterial hypertension (PAH). Right heart catheterization (RHC) was undertaken in 77 patients (17M/60F) with PAH, and simultaneous TTE was carried out to evaluate MPAP, PCWP and CO. Within 2 days, CO was measured again with CMR in similar physiological status. Then, PVR was calculated with the integrated non-invasive method: TTE-derived (MPAP-PCWP)/CMR-derived CO and the isolated TTE method: TTE-derived (MPAP-PCWP)/TTE-derived CO, respectively. The PVR calculated with integrated non-invasive method correlated well with RHC-calculated PVR (r = 0.931, 95% confidence interval 0.893 to 0.956). Between the integrated non-invasive PVR and RHC-calculated PVR, the Bland-Altman analysis showed the satisfactory limits of agreement (mean value: - 0.89 ± 2.59). In comparison, the limits of agreement were less satisfactory between TTE-calculated PVR and RHC-calculated PVR (mean value: - 1.80 ± 3.33). Furthermore, there were excellent intra- and inter-observer correlations for the measurements of TTE and CMR ( P  TTE and CMR provides a clinically reliable method to determine PVR non-invasively. In comparison with RHC, the integrated method shows good accuracy and repeatability, which suggests the potential for the evaluation and serial follow-up in patients with PAH. In PAH, the non-invasive measurement of PVR is very important in clinical practice. Up to now, however, the widely accepted non-invasive method is still unavailable. Since TTE can estimate (MPAP-PCWP) reliably and CMR is the best image modality for the measurement of CO, the combined

  18. Suction/inspiration against resistance or standardized Mueller maneuver: a new breathing technique to improve contrast density within the pulmonary artery: a pilot CT study

    International Nuclear Information System (INIS)

    Gutzeit, Andreas; Froehlich, Johannes M.; Weymarn, Constantin von; Goyen, Matthias; Waelti, Stephan; Roos, Justus E.; Meissnitzer, Matthias; Hergan, Klaus; Czell, David; Reischauer, Carolin

    2015-01-01

    Our aim was to prospectively investigate whether the recently introduced suction/inspiration against resistance breathing method leads to higher computed tomography (CT) contrast density in the pulmonary artery compared to standard breathing. The present study was approved by the Medical Ethics committee and all subjects gave written informed consent. Fifteen patients, each without suspicious lung emboli, were randomly assigned to four different groups with different breathing maneuvers (suction against resistance, Valsalva, inspiration, expiration) during routine CT. Contrast enhancement in the central and peripheral sections of the pulmonary artery were measured and compared with one another. Peripheral enhancement during suction yielded increased mean densities of 138.14 Hounsfield units (HU) (p = 0.001), compared to Valsalva and a mean density of 67.97 HU superior to inspiration (p = 0.075). Finally, suction in comparison to expiration resulted in a mean increase of 30.51 HU (p = 0.42). Central parts of pulmonary arteries presented significantly increased enhancement values (95.74 HU) for suction versus the Valsalva technique (p = 0.020), while all other mean densities were in favour of suction (versus inspiration: p = 0.201; versus expiration: p = 0.790) without reaching significance. Suction/Inspiration against resistance is a promising technique to improve contrast density within pulmonary vessels, especially in the peripheral parts, in comparison to other breathing maneuvers. (orig.)

  19. Suction/inspiration against resistance or standardized Mueller maneuver: a new breathing technique to improve contrast density within the pulmonary artery: a pilot CT study

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    Gutzeit, Andreas [Paracelsus Medical University Salzburg, Department of Radiology, Salzburg (Austria); Hirslanden Hospital St. Anna, Institute of Radiology and Nuclear Medicine, Clinical Research Unit, Lucerne (Switzerland); Kantonsspital Winterthur, Department of Radiology, Winterthur (Switzerland); Froehlich, Johannes M.; Weymarn, Constantin von; Goyen, Matthias [Hirslanden Hospital St. Anna, Institute of Radiology and Nuclear Medicine, Clinical Research Unit, Lucerne (Switzerland); Waelti, Stephan [Cantonal Hospital St. Gallen, Department of Radiology and Nuclear Medicine, St. Gallen (Switzerland); Roos, Justus E. [Duke University Medical Center, Department of Radiology, Durham, NC (United States); Meissnitzer, Matthias; Hergan, Klaus [Paracelsus Medical University Salzburg, Department of Radiology, Salzburg (Austria); Czell, David [Cantonal Hospital Winterthur, Department of Neurology, Winterthur (Switzerland); Reischauer, Carolin [Paracelsus Medical University Salzburg, Department of Radiology, Salzburg (Austria); Hirslanden Hospital St. Anna, Institute of Radiology and Nuclear Medicine, Clinical Research Unit, Lucerne (Switzerland)

    2015-11-15

    Our aim was to prospectively investigate whether the recently introduced suction/inspiration against resistance breathing method leads to higher computed tomography (CT) contrast density in the pulmonary artery compared to standard breathing. The present study was approved by the Medical Ethics committee and all subjects gave written informed consent. Fifteen patients, each without suspicious lung emboli, were randomly assigned to four different groups with different breathing maneuvers (suction against resistance, Valsalva, inspiration, expiration) during routine CT. Contrast enhancement in the central and peripheral sections of the pulmonary artery were measured and compared with one another. Peripheral enhancement during suction yielded increased mean densities of 138.14 Hounsfield units (HU) (p = 0.001), compared to Valsalva and a mean density of 67.97 HU superior to inspiration (p = 0.075). Finally, suction in comparison to expiration resulted in a mean increase of 30.51 HU (p = 0.42). Central parts of pulmonary arteries presented significantly increased enhancement values (95.74 HU) for suction versus the Valsalva technique (p = 0.020), while all other mean densities were in favour of suction (versus inspiration: p = 0.201; versus expiration: p = 0.790) without reaching significance. Suction/Inspiration against resistance is a promising technique to improve contrast density within pulmonary vessels, especially in the peripheral parts, in comparison to other breathing maneuvers. (orig.)

  20. Rhabdomyosarcoma of the pulmonary artery

    International Nuclear Information System (INIS)

    Barth, J.; Lehmann, H.; Thermann, M.; Horny, H.P.; Stein, H.; Kiel Univ.; Kiel Univ.; Kiel Univ.

    1982-01-01

    A case of a 55-year-old man with the histological diagnosis rhabdomyosarcoma of the left pulmonary artery has been seen. Lung scanning and pulmonary arteriography are the clues for the diagnostical procedure. 55 cases from the literature are reviewed and clinical findings of the early and late stages of the diseases are discussed. Surgical treatment is the therapy of choice if ever possible; aggressive chemotherapy might be an acceptable alternative. (orig.) [de

  1. Noninvasively assessed pulmonary artery stiffness predicts mortality in pulmonary arterial hypertension .

    NARCIS (Netherlands)

    Gan, C.T.; Lankhaar, J.W.; Westerhof, N.; Marcus, J.T.; Becker, A.; Twisk, J.W.R.; Boonstra, A.; Postmus, P.E.; Vonk Noordegraaf, A.

    2007-01-01

    Aims: Decreased total compliance of the pulmonary vascular bed is associated with increased mortality in patients with pulmonary arterial hypertension (PAH). We investigated whether proximal pulmonary artery stiffness, in terms of area distensibility and noninvasively assessed relative area change

  2. Pulmonary artery-to-pulmonary artery anastomoses: angiographic demonstration in patients with chronic thromboembolic pulmonary hypertension

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    Hodson, J. [Department of Imaging, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Graham, A. [Department of Imaging, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Hughes, J.M.B. [Department of Respiratory Medicine, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Gibbs, J.S.R. [Department of Cardiology, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Jackson, J.E. [Department of Imaging, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom)]. E-mail: jejackson@hhnt.org

    2006-03-15

    AIM: To describe direct pulmonary artery-to-pulmonary artery anastomoses seen at pulmonary angiography in patients with chronic thromboembolic pulmonary hypertension and discuss their possible significance. MATERIALS AND METHODS: Between 1 August 2000 and 31 July 2004 43 patients (male-to-female ratio 25:18) with a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) underwent selective pulmonary angiography to assess the extent of disease and suitability for surgical pulmonary endarterectomy. The mean pulmonary artery pressure ranged from 27-84 mmHg (average of 51 mmHg). Selective bilateral digital subtraction pulmonary angiograms performed in all individuals were reviewed for the presence of intrapulmonary collaterals. RESULTS: In 15 of the 43 patients (male-to-female ratio =7:8) definite (n=12) or probable (n=3) pulmonary artery-to-pulmonary artery anastomoses were demonstrated. Of the remaining 28 patients in whom intrapulmonary collaterals were not seen it was felt that in 16 the angiograms were of insufficient diagnostic quality (grades 4-5) to exclude their presence. Twelve patients, eight of whom had angiograms of sufficient diagnostic quality (grades 1-3), demonstrated one or more areas of luxury perfusion but intrapulmonary collaterals were not seen. CONCLUSION: Direct pulmonary artery-to-pulmonary artery anastomoses were demonstrated in patients with chronic thromboembolic pulmonary hypertension, which to our knowledge have not been previously described. The importance of these collateral vessels is unclear but they may play a role in the maintenance of pulmonary parenchymal viability in patients with chronic pulmonary embolic disease. The rate of development of these collaterals and their prognostic significance in patients with chronic thromboembolic pulmonary hypertension are areas worthy of further study.

  3. Pulmonary artery-to-pulmonary artery anastomoses: angiographic demonstration in patients with chronic thromboembolic pulmonary hypertension

    International Nuclear Information System (INIS)

    Hodson, J.; Graham, A.; Hughes, J.M.B.; Gibbs, J.S.R.; Jackson, J.E.

    2006-01-01

    AIM: To describe direct pulmonary artery-to-pulmonary artery anastomoses seen at pulmonary angiography in patients with chronic thromboembolic pulmonary hypertension and discuss their possible significance. MATERIALS AND METHODS: Between 1 August 2000 and 31 July 2004 43 patients (male-to-female ratio 25:18) with a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) underwent selective pulmonary angiography to assess the extent of disease and suitability for surgical pulmonary endarterectomy. The mean pulmonary artery pressure ranged from 27-84 mmHg (average of 51 mmHg). Selective bilateral digital subtraction pulmonary angiograms performed in all individuals were reviewed for the presence of intrapulmonary collaterals. RESULTS: In 15 of the 43 patients (male-to-female ratio =7:8) definite (n=12) or probable (n=3) pulmonary artery-to-pulmonary artery anastomoses were demonstrated. Of the remaining 28 patients in whom intrapulmonary collaterals were not seen it was felt that in 16 the angiograms were of insufficient diagnostic quality (grades 4-5) to exclude their presence. Twelve patients, eight of whom had angiograms of sufficient diagnostic quality (grades 1-3), demonstrated one or more areas of luxury perfusion but intrapulmonary collaterals were not seen. CONCLUSION: Direct pulmonary artery-to-pulmonary artery anastomoses were demonstrated in patients with chronic thromboembolic pulmonary hypertension, which to our knowledge have not been previously described. The importance of these collateral vessels is unclear but they may play a role in the maintenance of pulmonary parenchymal viability in patients with chronic pulmonary embolic disease. The rate of development of these collaterals and their prognostic significance in patients with chronic thromboembolic pulmonary hypertension are areas worthy of further study

  4. Management of pulmonary arterial hypertension.

    LENUS (Irish Health Repository)

    Judge, Eoin P

    2013-02-01

    Pulmonary arterial hypertension (PAH) is a complex disease with a high mortality. Management of this disease is underpinned by supportive and general therapies delivered by multidisciplinary teams in specialist centres. In recent years, a number of PAH-specific therapies have improved patient outcomes. This article will discuss the management of PAH in the context of relevant recently published studies in this area.

  5. Pulmonary Arterial Hypertension

    Science.gov (United States)

    ... and may possibly fail. Because the blood has dif culty getting through the lungs to pick up ... Arterial Hypertension? The cause of PAH is often dif cult to determine. Your health care provider will ...

  6. Prediction of neonatal respiratory distress syndrome in term pregnancies by assessment of fetal lung volume and pulmonary artery resistance index.

    Science.gov (United States)

    Laban, Mohamed; Mansour, Ghada M; Elsafty, Mohammed S E; Hassanin, Alaa S; EzzElarab, Sahar S

    2015-03-01

    To develop reference cutoff values for mean fetal lung volume (FLV) and pulmonary artery resistance index (PA-RI) for prediction of neonatal respiratory distress syndrome (RDS) in low-risk term pregnancies. As part of a cross-sectional study, women aged 20-35 years were enrolled and admitted to a tertiary hospital in Cairo, Egypt, for elective repeat cesarean at 37-40 weeks of pregnancy between January 1, 2012, and July 31, 2013. FLV was calculated by virtual organ computer-aided analysis, and PA-RI was measured by Doppler ultrasonography before delivery. A total of 80 women were enrolled. Neonatal RDS developed in 11 (13.8%) of the 80 newborns. Compared with neonates with RDS, healthy neonates had significantly higher FLVs (P<0.001) and lower PA-RIs (P<0.001). Neonatal RDS is less likely with FLV of at least 32 cm(3) or PA-RI less than or equal to 0.74. Combining these two measures improved the accuracy of prediction. The use of either FLV or PA-RI predicted neonatal RDS. The predictive value increased when these two measures were combined. Copyright © 2014 International Federation of Gynecology and Obstetrics. Published by Elsevier Ireland Ltd. All rights reserved.

  7. Pulmonary perfusion patterns and pulmonary arterial pressure.

    Science.gov (United States)

    Scott, James A

    2002-08-01

    To use artificial intelligence methods to determine whether quantitative parameters describing the perfusion image can be synthesized to make a reasonable estimate of the pulmonary arterial (PA) pressure measured at angiography. Radionuclide perfusion images were obtained in 120 patients with normal chest radiographs who also underwent angiographic PA pressure measurement within 3 days of the radionuclide study. An artificial neural network (ANN) was constructed from several image parameters describing statistical and boundary characteristics of the perfusion images. With use of a leave-one-out cross-validation technique, this method was used to predict the PA systolic pressure in cases on which the ANN had not been trained. A Pearson correlation coefficient was determined between the predicted and measured PA systolic pressures. ANN predictions correlated with measured pulmonary systolic pressures (r = 0.846, P artificial intelligence methods helps to reveal physiologic information not readily apparent at visual image inspection. Copyright RSNA, 2002

  8. Pulmonary Artery Dissection: A Fatal Complication of Pulmonary Hypertension

    Directory of Open Access Journals (Sweden)

    Chuanchen Zhang

    2016-01-01

    Full Text Available Pulmonary artery dissection is extremely rare but it is a really life-threatening condition when it happens. Most patients die suddenly from major bleeding or tamponade caused by direct rupture into mediastinum or retrograde into the pericardial sac. What we are reporting is a rare case of a 46-year-old female patient whose pulmonary artery dissection involves both the pulmonary valve and right pulmonary artery. The patient had acute chest pain and severe dyspnea, and the diagnosis of pulmonary artery dissection was confirmed by ultrasonography and CT angiography. Moreover, its etiology, clinical manifestations, and management are also discussed in this article.

  9. Thymoquinone attenuates monocrotaline-induced pulmonary artery hypertension via inhibiting pulmonary arterial remodeling in rats.

    Science.gov (United States)

    Zhu, Ning; Zhao, Xuyong; Xiang, Yijia; Ye, Shiyong; Huang, Jie; Hu, Wuming; Lv, Linchun; Zeng, Chunlai

    2016-10-15

    Pulmonary artery remodeling induced by excess proliferation, migration and apoptosis resistance of pulmonary arterial smooth muscle cells (PASMCs) is a key component in pulmonary artery hypertension (PAH). Thymoquinone (TQ) triggers cancer cells apoptosis through multiple mechanisms. In addition, TQ inhibits migration of human nonsmall-cell lung cancer cells and human glioblastoma cells. In the current study, we investigated effects of TQ on MCT-induced PAH in rats and its underlying mechanisms. After 2weeks of monocrotaline injection (MCT, 60mg/kg), Male Sprague-Dawley rats received TQ (8mg/kg, 12mg/kg, 16mg/kg) or olive oil per day for 2weeks. Hemodynamic changes, right ventricular hypertrophy, and lung morphological features were examined 4weeks later. In addition, TUNEL, PCNA, α-SMA, Bax and Bcl-2 were detected by immunohistochemistry staining. Bax, Bcl-2, cleaved caspase-3, cleaved poly (ADP-ribose) polymerase (PARP) MMP2, MMP9 and activation of p38MAPK and NF-κB were assessed by Western blot. MCT-induced an increase in pulmonary blood pressure and right ventricular hypertrophy, which were attenuated by TQ treatment. TQ also blocked MCT-induced pulmonary arterial remodeling, proliferation of PASMCs, elevation of MMP2 and downregulation of ratio of Bax/Bcl-2, cleaved caspase-3 and cleaved PARP. Furthermore, TQ inhibited MCT-induced activation of p38MAPK and NF-κB. TQ ameliorates MCT-induced pulmonary artery hypertension by inhibiting pulmonary arterial remodeling partially via p38MAPK/NF-κB signaling pathway in rats. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  10. Systemic Arterial-to-Pulmonary Artery Shunt Utilization

    African Journals Online (AJOL)

    multiruka1

    In certain circumstances, such as cyanotic neonates with tetralogy of Fallot (4) or cyanotic patients with. Tetralogy of Fallot and hypoplastic pulmonary arteries. (5), better outcomes are obtained if definitive surgery. (total correction or palliation) is preceded by creation of a systemic arterial-to-pulmonary artery shunt (SAPAS).

  11. Current insights on the pathogenesis of pulmonary arterial hypertension.

    Science.gov (United States)

    Perros, Frédéric; Dorfmüller, Peter; Humbert, Marc

    2005-08-01

    Regardless of the initial trigger, the elevated pulmonary arterial pressure and vascular resistance in patients with pulmonary arterial hypertension are primarily caused by remodeling and thrombosis of small- and medium-sized pulmonary arteries and arterioles, as well as sustained vasoconstriction. The process of pulmonary vascular remodeling involves all layers of the vessel wall and is complicated by cellular heterogeneity within each compartment. Indeed, each cell type (endothelial cells, smooth muscle cells, and fibroblasts), as well as inflammatory cells and platelets, may play significant roles in this condition. Recent studies have emphasized the relevance of several mediators in this condition, including prostaglandin-I (2) (prostacyclin), nitric oxide, endothelin-1, angiopoietin-1, 5-hydroxytryptamine (serotonin), cytokines, chemokines, and members of the transforming growth factor beta (TGF-beta) superfamily. Targeting some of these dysfunctional pathways (prostacyclin, nitric oxide, and endothelin-1) has been beneficial in subjects displaying pulmonary arterial hypertension.

  12. ECG monitoring of treatment response in pulmonary arterial hypertension patients

    NARCIS (Netherlands)

    Henkens, I.R.; Gan, C.T.; van Wolferen, S.A.; Hew, M.; Boonstra, A.; Twisk, J.W.R.; Kamp, O.; van der Wall, E.E.; Schalij, M.J.; Vonk Noordegraaf, A.; Vliegen, H.W.

    2008-01-01

    Background: The potential use of the ECG for monitoring treatment effects in patients with pulmonary arterial hypertension (PAH) has not been investigated. We evaluated whether the ECG is useful for monitoring treatment response based on changes in pulmonary vascular resistance (PVR). Methods: An

  13. Arterial pulmonary hypertension in noncardiac intensive care unit

    Directory of Open Access Journals (Sweden)

    Mykola V Tsapenko

    2008-10-01

    Full Text Available Mykola V Tsapenko1,5, Arseniy V Tsapenko2, Thomas BO Comfere3,5, Girish K Mour1,5, Sunil V Mankad4, Ognjen Gajic1,51Division of Pulmonary and Critical Care Medicine; 3Division of Critical Care Medicine; 4Division of Cardiovascular Diseases, Mayo Epidemiology and Translational Research in Intensive Care (M.E.T.R.I.C, Mayo Clinic, Rochester, MN, USA; 2Division of Pulmonary and Critical Care Medicine, Brown University, Miriam Hospital, Providence, RI, USAAbstract: Pulmonary artery pressure elevation complicates the course of many complex disorders treated in a noncardiac intensive care unit. Acute pulmonary hypertension, however, remains underdiagnosed and its treatment frequently begins only after serious complications have developed. Significant pathophysiologic differences between acute and chronic pulmonary hypertension make current classification and treatment recommendations for chronic pulmonary hypertension barely applicable to acute pulmonary hypertension. In order to clarify the terminology of acute pulmonary hypertension and distinguish it from chronic pulmonary hypertension, we provide a classification of acute pulmonary hypertension according to underlying pathophysiologic mechanisms, clinical features, natural history, and response to treatment. Based on available data, therapy of acute arterial pulmonary hypertension should generally be aimed at acutely relieving right ventricular (RV pressure overload and preventing RV dysfunction. Cases of severe acute pulmonary hypertension complicated by RV failure and systemic arterial hypotension are real clinical challenges requiring tight hemodynamic monitoring and aggressive treatment including combinations of pulmonary vasodilators, inotropic agents and systemic arterial vasoconstrictors. The choice of vasopressor and inotropes in patients with acute pulmonary hypertension should take into consideration their effects on vascular resistance and cardiac output when used alone or in

  14. Phosphodiesterase 10A upregulation contributes to pulmonary arterial hypertension

    OpenAIRE

    Tian, Xia

    2009-01-01

    Pulmonary arterial hypertension (PAH) is a progressive disease defined by an elevation of pulmonary vascular resistance due to sustained vessel contraction and enhanced vascular remodeling. The abnormal tone and remodeling in the pulmonary vasculature are believed to be related, at least in part, to the decrease of cyclic nucleotide levels that are controlled by cyclic nucleotide phosphodiesterases (PDEs). PDEs, of which 11 families have been identified, maintain homeostasis...

  15. Computed tomographic diagnosis of pulmonary artery aneurysm

    International Nuclear Information System (INIS)

    Maeno, Kouji; Kontani, Kazuhiro; Ito, Makoto; Sakurai, Noboru; Sawada, Taisei; Fukeda, Yasuhiko; Takata, Shigeo; Ikeda, Takayuki; Hattori, Nobu.

    1986-01-01

    Pulmonary artery aneurysms are rare lesions. Clagett et al reported that one aneurysm of the pulmonary artery may be found in approximately 14,000 necropsies. We have experienced a case of giant pulmonary artery aneurysm confirmed by computed tomography. A 38 year-old man with atrial septal defect admitted to Kanazawa City Hospital. He had been pointed out of a right hilar mass when he was 26 years old. His complaint was bloody sputum and cough. Pulmonary angiography was not useful for the definite diagnosis because of its mural thrombi. Enhanced computed tomography showed a giant pulmonary artery aneurysm with a mural thrombi in its cavity. This shows that enhanced computed tomography is very useful for the diagnosis of a pulmonary artery aneurysm with a mural thrombi in its cavity. (author)

  16. Pulmonary Artery Agenesis: A Case Series

    Directory of Open Access Journals (Sweden)

    Meltem Ağca

    2015-04-01

    Full Text Available Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis.The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated retrospectively 5 pulmonary artery agenesis cases diagnosed in our clinics between 1998-2010 with respect to the literature.

  17. Role of Lipoxygenase Metabolites of Arachidonic Acid in Enhanced Pulmonary Artery Contractions of Female Rabbits

    OpenAIRE

    Pfister, Sandra L.

    2011-01-01

    Pulmonary arterial hypertension is characterized by elevated pulmonary artery pressure and vascular resistance. In women the incidence is 4 fold greater than that in men. Studies suggest sustained vasoconstriction is a factor in increased vascular resistance. Possible vasoconstrictor mediators include arachidonic acid-derived lipoxygenase metabolites. Our studies in rabbits showed enhanced endothelium-dependent contractions to arachidonic acid in pulmonary arteries from females compared to ma...

  18. [Physiopathology of pulmonary arterial hypertension. Cellular and molecular aspects].

    Science.gov (United States)

    Perros, Frédéric; Humbert, Marc

    2005-02-12

    The combined effects of vasoconstriction, remodelling of the pulmonary vessel walls and in situ thrombosis contribute to the increase in pulmonary vascular resistance during pulmonary arterial hypertension. Vascular remodelling involves all the sheaths of the vessel wall and all the cell types of which it is composed (endothelial cells, smooth muscle cells, fibroblasts, inflammatory cells and platelets). Excessive vasoconstriction has been related to a defect in the function of expression of the potassium channels and endothelial dysfunction. This leads to chronic insufficiency in the production of vasodilators, notably nitrogen monoxide and prostacyclin and the excessive production of vasoconstrictors such as endotheline-1. These defects contribute to the increase in vascular tonus and pulmonary vascular remodelling and represent pertinent pharmacological targets. Certain growth factors, including those of the super-family of transforming growth factor beta, angiopoietine-1 and serotonin, may play a part in the pathogenesis of pulmonary arterial hypertension.

  19. Long-Term Survival in Idiopathic Pulmonary Arterial Hypertension Associated with Massive Pulmonary Artery Dilation

    Directory of Open Access Journals (Sweden)

    Vanja Petrovic

    2011-01-01

    Full Text Available The present report describes two patients with long-term survival after being diagnosed with idiopathic pulmonary arterial hypertension more than 20 years earlier. Both patients were treated with calcium channel blockers for several years and are currently maintained on bosentan, an oral endothelin receptor antagonist. Severe dilation of the main pulmonary arteries is present in both patients and may be related to long-term survival with idiopathic pulmonary arterial hypertension.

  20. Echocardiographic Assessment of Pulmonary Arterial Hypertension for Pediatricians and Neonatologists

    Directory of Open Access Journals (Sweden)

    Gregory James Skinner

    2017-09-01

    Full Text Available There is a growing awareness of the role that increased pulmonary vascular resistance (PVR plays in many pathologies; therefore, assessment of pulmonary artery pressure (PAP is an increasingly requested investigation in the critical care environment. This article will go through the basic concepts regarding PAP and PVR, then will go on to outline the various echocardiographic parameters which are used to assess them. Finally, an outline of how to undertake this assessment will be presented.

  1. The arterial load in pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    A. Vonk-Noordegraaf

    2010-09-01

    Full Text Available The anatomical differences between the pulmonary and systemic arterial system are the main cause of the difference in distribution of compliance. In the pulmonary arterial system compliance is distributed over the entire arterial system, and stands at the basis of the constancy of the RC-time. This distribution depends on the number of peripheral vessels, which is ∼8–10 times more in the pulmonary system than the systemic tree. In the systemic arterial tree the compliance is mainly located in the aorta (80% of total compliance in thoracic-abdominal aorta. The constant RC-time in the pulmonary bed results in proportionality of systolic and diastolic pressure with mean pressure and, in turn, in the constant ratio of oscillatory and mean power.

  2. Treatment options for paediatric pulmonary arterial hypertension

    NARCIS (Netherlands)

    Berger, R M F; Bonnet, D

    2010-01-01

    Pulmonary arterial hypertension (PAH) is a serious, progressive condition, which can present idiopathically or secondary to conditions such as systemic sclerosis or congenital heart disease. The condition exists in both adult and paediatric forms, which possess several similar characteristics. Adult

  3. Isolated Left Pulmonary Artery Agenesis: A Case Report

    Directory of Open Access Journals (Sweden)

    Tansel Ansal Balcı

    2012-08-01

    Full Text Available Unilateral pulmonary artery agenesis without any cardiovascular malformation is a rare anomaly. We present the imaging findings of a patient who was diagnosed as isolated left pulmonary artery agenesis. A 27-year-old female patient was admitted to our hospital due to dyspnea during exercise for five years. Chest X-ray revealed minimally small left pulmonary hilum and left lung. She was admitted to our clinic with the suspicion of pulmonary artery pathology. Absent perfusion of the left lung with normal ventilation was visualized on scintigraphy. MDCT angiography of pulmonary arteries showed absent left main pulmonary artery with systemic collaterals around left hemithorax. Pulmonary artery agenesis can be asymptomatic and isolated until adulthood. Both scintigraphy and CT angiography images of pulmonary artery agenesis of a patient are rare in the literature. Pulmonary ventilation- perfusion scintigraphy can be used not only for pulmonary embolism but also pathologies involving pulmonary artery and its branches. (MIRT 2012;21:80-83

  4. Idiopathic aneurysm of pulmonary artery

    Energy Technology Data Exchange (ETDEWEB)

    Pacheco, Julio B. Cota; Pimentel, Patricia N.; Knust, Beatriz S., E-mail: jcota@uol.com.br [Clinica de Cardiologia Cota Pacheco, Mogi das Cruzes, SP (Brazil)

    2015-07-15

    Because it is a very rare isolated lesion, we decided to present this case of idiopathic pulmonary artery aneurysm (IPAA) and review the cases published in the literature in order to correlate our clinical and imaging findings, as well as management based on patient data. IPAA was first described in a case of autopsy by Bristowe in 1860 and later in 1947 by Deterling and Claggett, whose prevalence was lower than eight to one hundred thousand. Although the use of diagnostic imaging methods has been applied in a very large population in recent decades, this lesion has been most often described in postmortem examinations. Therefore, it is important to be aware of possible clinical symptoms, at times non-specific, as well as the signs on imaging studies. In this study, therefore, the report of a case of an asymptomatic patient whose diagnosis was done through color Doppler echocardiography in a routine test in 2012, later confirmed by simple chest computed tomography (chest CT) and cardiac catheterization as IPAA and its branches. We discussed the literature available and the possibilities for treatment and the use of color Doppler echocardiography as an initial diagnostic tool for such a rare and intriguing disease. (author)

  5. Recent trends in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Rajagopalan Natarajan

    2011-01-01

    Full Text Available Pulmonary hypertension is a serious and unrelenting pulmonary vascular disorder that affects the functional quality of patients and significantly decreases their life span. If diagnosed early, with the number of new therapeutic options that are available, a better quality of life can be provided for a protracted length of time. It is likely that the available treatment will change the natural course of the disease and perhaps prolong survival. As symptoms are often subtle in the early stages of the disease it is imperative that physicians are aware of the manifestations of this condition. A thorough investigation of patients suspected of this condition is essential so that appropriate treatment can be initiated promptly. The routine workup of a patient suspected to have pulmonary hypertension could easily be carried out in any well-equipped peripheral hospital in many affluent and advanced countries. However, it must be mentioned that in some less advanced countries the necessary work up can only be done in major teaching hospitals. Both pulmonologists and cardiologists should be aware of the pathophysiology of pulmonary arterial hypertension, the workup and the treatment options that are available. Patients with refractory pulmonary hypertension should be referred to these research centers for enrolment into any ongoing drug trials as well as for evaluation for heart−lung, single lung, or double lung transplantation. This paper is primarily aimed at pulmonologists and cardiologists taking care of these patients. Unless indicated otherwise this paper mainly deals with WHO group 1 pulmonary hypertension which is designated pulmonary arterial hypertension. Extensive review of the literature spanning the last 30 years was made through Medline using titles such as primary pulmonary hypertension, pulmonary arterial hypertension, secondary pulmonary hypertension, and pulmonary vascular diseases.

  6. Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries.

    Science.gov (United States)

    Rabinowitz, Edon J; Epstein, Shilpi; Kohn, Nina; Meyer, David B

    2017-09-01

    Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm 2 /m 2 (range 15-144 mm 2 /m 2 ) and at time of septation 184 mm 2 /m 2 (range 56-510 mm 2 /m 2 ; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

  7. Diabetes Mellitus Associates with Increased Right Ventricular Afterload and Remodeling in Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Whitaker, Morgan E; Nair, Vineet; Sinari, Shripad; Dherange, Parinita; Natarajan, Balaji; Trutter, Lindsey; Brittain, Evan L; Hemnes, Anna R; Austin, Eric; Patel, Kumar; Black, Stephen M; Garcia, Joe G N; Yuan, Jason X; Vanderpool, Rebecca; Rischard, Franz; Makino, Ayako; Bedrick, Edward J; Desai, Ankit A

    2018-02-05

    Diabetes mellitus is associated with left ventricular hypertrophy and dysfunction. Parallel studies have also reported associations between diabetes mellitus and right ventricle dysfunction and reduced survival in patients with pulmonary arterial hypertension. However, the impact of diabetes mellitus on the pulmonary vasculature has not been well-characterized. We hypothesized that diabetes mellitus and hyperglycemia could specifically influence right ventricular afterload and remodeling in patients with Group I pulmonary arterial hypertension, providing a link to their known susceptibility to right ventricular dysfunction. Using an adjusted model for age, gender, pulmonary vascular resistance, and medication use, associations of fasting blood glucose, glycated hemoglobin, and the presence of diabetes mellitus were evaluated with markers of disease severity in 162 patients with pulmonary arterial hypertension. A surrogate measure of increased pulmonary artery stiffness, elevated pulmonary arterial elastance (P=0.012), along with reduced log(pulmonary artery capacitance) (P=0.006) were significantly associated with the presence of diabetes mellitus in patients with pulmonary arterial hypertension in a fully adjusted model. Similar associations between pulmonary arterial elastance and capacitance were noted with both fasting blood glucose and glycated hemoglobin. Furthermore, right ventricular wall thickness on echocardiography was greater in pulmonary arterial hypertension patients with diabetes, supporting the link between right ventricular remodeling and diabetes. Cumulatively, these data demonstrate that an increase in right ventricular afterload, beyond pulmonary vascular resistance alone, may influence right ventricular remodeling and provide a mechanistic link between the susceptibility to right ventricular dysfunction in patients with both diabetes mellitus and pulmonary arterial hypertension. Copyright © 2018. Published by Elsevier Inc.

  8. Pulmonary Artery Agenesis with Bronchial Asthma

    Directory of Open Access Journals (Sweden)

    Hassan Ghobadi

    2014-05-01

    Full Text Available Unilateral Pulmonary Artery Agenesis (UPAA is a rare congenital anomaly during the 4 th  week of gestational age. It is defined as an absence of pulmonary parenchyma and its supporting artery. A 9-year-old girl was admitted to our hospital because of chronic cough. Chest examination showed a decrement in lung sound of right hemi-thorax with expiratory wheeze. Chest radiography (CXR revealed a semi-opaque right hemi-thorax. Chest CT with intra-venous contrast demonstrated absence of the right pulmonary artery and lung parenchyma with hyper-inflated left lung and dextro-position of mediastinum. This case emphasizes that in patients with respiratory compliant and chronic cough CXR must be done to rule out similar diagnosis other than asthma.    

  9. Targeted therapies in pulmonary arterial hypertension.

    Science.gov (United States)

    Montani, David; Chaumais, Marie-Camille; Guignabert, Christophe; Günther, Sven; Girerd, Barbara; Jaïs, Xavier; Algalarrondo, Vincent; Price, Laura C; Savale, Laurent; Sitbon, Olivier; Simonneau, Gérald; Humbert, Marc

    2014-02-01

    Pulmonary arterial hypertension (PAH) is a rare disorder characterized by progressive obliteration of small pulmonary arteries that leads to elevated pulmonary arterial pressure and right heart failure. During the last decades, an improved understanding of the pathophysiology of the disease has resulted in the development of effective therapies targeting endothelial dysfunction (epoprostenol and derivatives, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors). These drugs allow clinical, functional and hemodynamic improvement. Even though, no cure exists for PAH and prognosis remains poor. Recently, several additional pathways have been suggested to be involved in the pathogenesis of PAH, and may represent innovative therapies. In this summary, we review conventional therapy, pharmacological agents currently available for the treatment of PAH and the benefit/risk ratio of potential future therapies. © 2013 Elsevier Inc. All rights reserved.

  10. Pulmonary arterial hypertension in congenital heart disease: Correlation of radiologic index with hemodynamic data

    International Nuclear Information System (INIS)

    Choi, Young Hi

    1984-01-01

    It is well known that pulmonary arterial hypertension in congenital heart disease is an important prognostic factor, as is pulmonary vascular resistance. So it is tempting to get certain radiologic index that could predict the presence and the degree of pulmonary arterial hypertension. A total of 152 cases of left to right shunt with pulmonary arterial hypertension and 50 cases of left to right shunt without pulmonary arterial hypertension is presented, in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between March 1981 and February 1983. Statistical analysis of plain radiography findings with the emphasis on the correction of radiologic index with the hemodynamic data. The results are as follows: 1. The incidence of pulmonary arterial hypertension is much less in arterial septal defect than other two disease groups of left to right shunt. 2. PA/T ratio correlates well with pulmonary arterial pressure (r=0.674), especially in mild pulmonary hypertension group. No correlation in moderate pulmonary hypertension group in significant level. 3. PA/T ratio is below 38 in total cases of normal control group and in 32 cases (21.0%) among 152 cases of pulmonary arterial hypertension group. 4. The average PA/T ratio in normal pressure group of left to right shunt is 35.3, which has no significant difference from that of normal control group. 5. The average CT ratio of pulmonary arterial hypertension group is 59.0, which is larger than 49.1 of normal control group. The CT ratio shows no correlation with the pulmonary arterial pressure in statistically significant level. 6. The higher the pulmonary arterial pressure, the larger the Rp/Rs value. The Rp/Rs in atrial septal defect is 0.193 in average, the lowest value in comparison with other two disease groups.

  11. Pulmonary venous remodeling in COPD-pulmonary hypertension and idiopathic pulmonary arterial hypertension

    DEFF Research Database (Denmark)

    Andersen, Kasper Hasseriis; Andersen, Claus Bøgelund; Gustafsson, Finn

    2017-01-01

    Pulmonary vascular arterial remodeling is an integral and well-understood component of pulmonary hypertension (PH). In contrast, morphological alterations of pulmonary veins in PH are scarcely described. Explanted lungs (n = 101) from transplant recipients with advanced chronic obstructive...

  12. Adenosine Receptors As Drug Targets for Treatment of Pulmonary Arterial Hypertension

    OpenAIRE

    Alencar, Allan K. N.; Montes, Guilherme C.; Barreiro, Eliezer J.; Sudo, Roberto T.; Zapata-Sudo, Gisele

    2017-01-01

    Pulmonary arterial hypertension (PAH) is a clinical condition characterized by pulmonary arterial remodeling and vasoconstriction, which promote chronic vessel obstruction and elevation of pulmonary vascular resistance. Long-term right ventricular (RV) overload leads to RV dysfunction and failure, which are the main determinants of life expectancy in PAH subjects. Therapeutic options for PAH remain limited, despite the introduction of prostacyclin analogs, endothelin receptor antagonists, pho...

  13. Bronchial arterial infusion versus bronchial combined pulmonary arterial infusion for pulmonary metastatic tumors

    International Nuclear Information System (INIS)

    Dong Sheng; Dong Weihua; Jia Ningyang; Zhang Dianbo; Xiao Xiangsheng

    2008-01-01

    Objective: To evaluate the pulmonary metastatic tumor response to different ways of transcatheter arterial infusion. Methods: Thirty-five patients with pulmonary metastatic tumors were randomized divided into two groups: 15 patients with 49 lesions treated with bronchial arterial infusion (BAI) and 20 patients with 65 lesions treated with bronchial arterial infusion (BM)combined with pulmonary arterial infusion (PAI). The therapeutic response was assessed by the WHO evaluation criteria. Results: The total effective rate(CR + PR) of BAI was 65.3% (32/49), PAI + BAI was 61.5%(40/65) showing no statistical difference. The median survival time of BAI was 9 mo, BAI + PAI was 11.5 mo, demonstrating no statistical significance. Conclusions: BAI should be the primary treatment for pulmonary metastatic tumor. (authors)

  14. Identity crisis in pulmonary arterial hypertension.

    Science.gov (United States)

    Ruffenach, G; Bonnet, S; Rousseaux, S; Khochbin, S; Provencher, S; Perros, F

    2018-01-01

    Pulmonary arterial hypertension (PAH) shares many hallmarks with cancer. Cancer cells acquire their hallmarks by a pathological Darwinian evolution process built on the so-called cancer cell "identity crisis." Here we demonstrate that PAH shares the most striking features of the cancer identity crisis: the ectopic expression of normally silent tissue-specific genes.

  15. A review of wave mechanics in the pulmonary artery with an emphasis on wave intensity analysis

    DEFF Research Database (Denmark)

    Su, Junjing; Hilberg, Ole; Howard, Luke

    2016-01-01

    Mean pulmonary arterial pressure and pulmonary vascular resistance (PVR) remain the most common haemodynamic measures to evaluate the severity and prognosis of pulmonary hypertension. However, PVR only captures the non-oscillatory component of the right ventricular hydraulic load and neglects...... reflection are observed in patients with pulmonary hypertension and in animal models exposed to hypoxia. Studying wave propagation makes a valuable contribution to the assessment of the arterial system in pulmonary hypertension, and here, we briefly review the current state of knowledge of the methods used...

  16. Right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus

    International Nuclear Information System (INIS)

    Lee, Peter; McCauley, Roy; Westra, Sjirk; Baba, Timothy

    2006-01-01

    Pulmonary aplasia and bronchopulmonary foregut malformations in which a patent communication between the foregut and the pulmonary system is present are rare congenital abnormalities. Pulmonary aplasia associated with a pulmonary sling is an even rarer abnormality. We report a unique case of right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus diagnosed by multidetector helical CT. (orig.)

  17. Skeletal muscle abnormalities in pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    Ana Paula Breda

    Full Text Available Pulmonary arterial hypertension is a progressive disease that is characterized by dyspnea and exercise intolerance. Impairment in skeletal muscle has recently been described in PAH, although the degree to which this impairment is solely determined by the hemodynamic profile remains uncertain. The aim of this study was to verify the association of structural and functional skeletal muscle characteristics with maximum exercise in PAH.The exercise capacity, body composition, CT area of limb muscle, quality of life, quadriceps biopsy and hemodynamics of 16 PAH patients were compared with those of 10 controls.PAH patients had a significantly poorer quality of life, reduced percentage of lean body mass, reduced respiratory muscle strength, reduced resistance and strength of quadriceps and increased functional limitation at 6MWT and CPET. VO2 max was correlated with muscular variables and cardiac output. Bivariate linear regression models showed that the association between muscular structural and functional variables remained significant even after correcting for cardiac output.Our study showed the coexistence of ventilatory and quadriceps weakness in face of exercise intolerance in the same group of PAH patients. More interestingly, it is the first time that the independent association between muscular pattern and maximum exercise capacity is evidenced in PAH, independently of cardiac index highlighting the importance of considering rehabilitation in the treatment strategy for PAH.

  18. Metabolomic heterogeneity of pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    Yidan Zhao

    Full Text Available Although multiple gene and protein expression have been extensively profiled in human pulmonary arterial hypertension (PAH, the mechanism for the development and progression of pulmonary hypertension remains elusive. Analysis of the global metabolomic heterogeneity within the pulmonary vascular system leads to a better understanding of disease progression. Using a combination of high-throughput liquid-and-gas-chromatography-based mass spectrometry, we showed unbiased metabolomic profiles of disrupted glycolysis, increased TCA cycle, and fatty acid metabolites with altered oxidation pathways in the human PAH lung. The results suggest that PAH has specific metabolic pathways contributing to increased ATP synthesis for the vascular remodeling process in severe pulmonary hypertension. These identified metabolites may serve as potential biomarkers for the diagnosis of PAH. By profiling metabolomic alterations of the PAH lung, we reveal new pathogenic mechanisms of PAH, opening an avenue of exploration for therapeutics that target metabolic pathway alterations in the progression of PAH.

  19. [Pulmonary arterial hypertension: a flavor of autoimmunity].

    Science.gov (United States)

    Perros, Frédéric; Humbert, Marc; Cohen-Kaminsky, Sylvia

    2013-01-01

    It is admitted that autoimmunity results from a combination of risks such as genetic background, environmental triggers, and stochastic events. Pulmonary arterial hypertension (PAH) shares with the so-called prototypic autoimmune diseases, genetic risk factors, female predominance and sex hormone influence, association with other chronic inflammatory and autoimmune diseases, defects in regulatory T cells function, and presence of autoantibodies. Case reports have been published indicating the beneficial effect of some immunosuppressive and anti-inflammatory therapies in PAH, supporting the potential role of immune mechanisms in the pathophysiology of the disease. In this review, we discuss the current knowledge on autoimmune mechanisms operating in PAH, especially mounting a local autoimmune response inside the pulmonary tissue, namely pulmonary lymphoid neogenesis. A better understanding of the role of autoimmunity in pulmonary vascular remodelling may help develop targeted immunomodulatory strategies in PAH. © 2013 médecine/sciences – Inserm.

  20. Pulmonary arterial hypertension: an update on diagnosis and treatment.

    Science.gov (United States)

    Stringham, Richard; Shah, Nipa R

    2010-08-15

    Pulmonary arterial hypertension is defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest or 30 mm Hg during physical activity. Pulmonary arterial hypertension is classified into subgroups, including idiopathic, heritable, and pulmonary arterial hypertension associated with other conditions. A detailed history, thorough physical examination, and most importantly, a high index of suspicion are essential to diagnosis. Evaluation includes echocardiography and exclusion of other causes of symptoms. Targeted laboratory testing can help identify the subgroup of pulmonary arterial hypertension. Right heart catheterization is required to confirm the diagnosis. Standard treatment options include oral anticoagulation, diuretics, oxygen supplementation, and for a small percentage of patients, calcium channel blockers. Newer treatments include prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors. Combination therapy has been shown to improve pulmonary arterial pressure, but more research is needed. Interventional procedures for patients with pulmonary arterial hypertension include balloon atrial septostomy and lung transplantation.

  1. Recent Strategies in Treatment of Pulmonary Arterial Hypertension, A Review

    OpenAIRE

    Fallah, Flora

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a disease characterized by an elevation in pulmonary artery pressure that can lead to right ventricular failure and death. The pulmonary circulation has to accommodate the entire cardiac output in each cardiac cycle and evolution has adapted to this by making it a low-pressure high-flow system. However, pathology can affect both the arterial and venous components of this system. Pulmonary venous hypertension mainly refers to diseases that result in ele...

  2. Pulmonary Effective Arterial Elastance as a Measure of Right Ventricular Afterload and Its Prognostic Value in Pulmonary Hypertension Due to Left Heart Disease.

    Science.gov (United States)

    Tampakakis, Emmanouil; Shah, Sanjiv J; Borlaug, Barry A; Leary, Peter J; Patel, Harnish H; Miller, Wayne L; Kelemen, Benjamin W; Houston, Brian A; Kolb, Todd M; Damico, Rachel; Mathai, Stephen C; Kasper, Edward K; Hassoun, Paul M; Kass, David A; Tedford, Ryan J

    2018-04-01

    Patients with combined post- and precapillary pulmonary hypertension due to left heart disease have a worse prognosis compared with isolated postcapillary. However, it remains unclear whether increased mortality in combined post- and precapillary pulmonary hypertension is simply a result of higher total right ventricular load. Pulmonary effective arterial elastance (Ea) is a measure of total right ventricular afterload, reflecting both resistive and pulsatile components. We aimed to test whether pulmonary Ea discriminates survivors from nonsurvivors in patients with pulmonary hypertension due to left heart disease and if it does so better than other hemodynamic parameters associated with combined post- and precapillary pulmonary hypertension. We combined 3 large heart failure patient cohorts (n=1036) from academic hospitals, including patients with pulmonary hypertension due to heart failure with preserved ejection fraction (n=232), reduced ejection fraction (n=335), and a mixed population (n=469). In unadjusted and 2 adjusted models, pulmonary Ea more robustly predicted mortality than pulmonary vascular resistance and the transpulmonary gradient. Along with pulmonary arterial compliance, pulmonary Ea remained predictive of survival in patients with normal pulmonary vascular resistance. The diastolic pulmonary gradient did not predict mortality. In addition, in a subset of patients with echocardiographic data, Ea and pulmonary arterial compliance were better discriminators of right ventricular dysfunction than the other parameters. Pulmonary Ea and pulmonary arterial compliance more consistently predicted mortality than pulmonary vascular resistance or transpulmonary gradient across a spectrum of left heart disease with pulmonary hypertension, including patients with heart failure with preserved ejection fraction, heart failure with reduced ejection fraction, and pulmonary hypertension with a normal pulmonary vascular resistance. © 2018 American Heart Association

  3. Method for imaging pulmonary arterial hypoplasia

    International Nuclear Information System (INIS)

    Triantafillou, M.

    2000-01-01

    Full text: Pulmonary hypoplasia represents an incomplete development of the lung, resulting in the reduction of distended lung volume. This is associated with small or absent number of airway divisions, alveoli, arteries and veins. Unilateral pulmonary Hypoplasia is often asymptomatic and may be demonstrated as a hypodense lung on a chest X-ray. Computer Tomography (CT) scanning would show anatomical detail and proximal vessels. Magnetic Resonance Imaging (MRI) will show no more detail than which the CT scan has already demonstrated. It is, also, difficult to visualise collateral vessels from systemic and/or bronchial vessels on both these modalities. Pulmonary Angiography would give the definitive answer, but it is time consuming and has significant risks associated with the procedure. There are high costs associated with these modalities. Nuclear Medicine Ventilation/Perfusion (V/Q) scan performed on these patients would demonstrate diminished ventilation due to reduced lung volume and absence of perfusion to the hypoplastic lung. To date, we have performed V/Q lung scan on two children in our department. Both cases demonstrate diminished ventilation with no perfusion to the hypoplastic lung. Though the gold standard is Pulmonary Angiography, V/Q scanning is cost effective, less time consuming and a non invasive procedure that can be performed as an outpatient. It is accurate as it demonstrates absent lung perfusion, confirming the patient has pulmonary arterial hypoplasia. Copyright (2000) The Australian and New Zealand Society of Nuclear Medicine Inc

  4. Isolated Unilateral Absent Branch Pulmonary Artery with Peripheral Pulmonary Stenosis and Coronary Artery Disease

    Directory of Open Access Journals (Sweden)

    Sunil Abhishek B

    2017-09-01

    Full Text Available Isolated Unilateral Absent Pulmonary Artery (UAPA is a rare congenital anomaly. It is usually associated with congenital heart defects. A 45 year old male patient presented with complaints of fever with cough and expectoration for 15 days and retrosternal chest discomfort for the previous 2 days. ECG showed diffuse ST segment depression with T wave inversion in the inferior and lateral leads. Coronary Angiogram done through the right femoral approach revealed diffusely diseased Left Anterior Descending (LAD artery that was totally cut off at the mid segment. The Left Circumflex (LCx artery was providing blood supply to the right middle and lower lung areas. There was another collateral arising from the Left Subclavian Artery supplying the right middle and lower lung areas. The left pulmonary artery was normal, but branches supplying the middle and lower lobes of the right lung were absent and the upper lobe branch had pulmonary stenosis. UAPA is a rare clinical entity; collaterals from coronaries are extremely rare in this condition and till now there has not been any case report of unilateral absent branch pulmonary artery with peripheral stenosis of other branches, on the affected side and associated coronary artery disease.

  5. Adrenomedullin alleviates pulmonary artery collagen accumulation in rats with pulmonary hypertension induced by high blood flow.

    Science.gov (United States)

    Pang, Lulu; Qi, Jianguang; Gao, Yang; Jin, Hongfang; Du, Junbao

    2014-04-01

    Collagen accumulation is one of the important pathologic changes in the development of pulmonary hypertension. Previous research showed that adrenomedullin (ADM) mitigates the development of pulmonary hypertension. The present study explored the role of ADM in the development of pulmonary artery collagen accumulation induced by high pulmonary blood flow, by investigating the effect of ADM [1.5 μg/(kg h)] subcutaneously administered by mini-osmotic pump on pulmonary hemodynamics, pulmonary vascular structure and pulmonary artery collagen accumulation and synthesis in rats with high pulmonary blood flow induced by aortocaval shunting. The results showed that ADM significantly decreased mean pulmonary artery pressure (mPAP) and the ratio of right ventricular mass to left ventricular plus septal mass [RV/(LV+SP)], attenuated the muscularization of small pulmonary vessels and relative medial thickness (RMT) of pulmonary arteries in rats with high pulmonary blood flow. Meanwhile, ADM ameliorated pulmonary artery collagen deposition represented by a decrease in lung tissue hydroxyproline, collagens I and III content and pulmonary artery collagens I and III expression, reduced collagen synthesis represented by a decrease in lung tissue procollagens I and III mRNA expression. The results suggest that ADM plays a protective role in the development of pulmonary hypertension induced by high blood flow, by inhibiting pulmonary procollagen synthesis and alleviating pulmonary artery collagen accumulation. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. Autonomic nervous system involvement in pulmonary arterial hypertension.

    Science.gov (United States)

    Vaillancourt, Mylène; Chia, Pamela; Sarji, Shervin; Nguyen, Jason; Hoftman, Nir; Ruffenach, Gregoire; Eghbali, Mansoureh; Mahajan, Aman; Umar, Soban

    2017-12-04

    Pulmonary arterial hypertension (PAH) is a chronic pulmonary vascular disease characterized by increased pulmonary vascular resistance (PVR) leading to right ventricular (RV) failure. Autonomic nervous system involvement in the pathogenesis of PAH has been demonstrated several years ago, however the extent of this involvement is not fully understood. PAH is associated with increased sympathetic nervous system (SNS) activation, decreased heart rate variability, and presence of cardiac arrhythmias. There is also evidence for increased renin-angiotensin-aldosterone system (RAAS) activation in PAH patients associated with clinical worsening. Reduction of neurohormonal activation could be an effective therapeutic strategy for PAH. Although therapies targeting adrenergic receptors or RAAS signaling pathways have been shown to reverse cardiac remodeling and improve outcomes in experimental pulmonary hypertension (PH)-models, the effectiveness and safety of such treatments in clinical settings have been uncertain. Recently, novel direct methods such as cervical ganglion block, pulmonary artery denervation (PADN), and renal denervation have been employed to attenuate SNS activation in PAH. In this review, we intend to summarize the multiple aspects of autonomic nervous system involvement in PAH and overview the different pharmacological and invasive strategies used to target autonomic nervous system for the treatment of PAH.

  7. Ambulatory pulmonary arterial pressure in primary pulmonary hypertension: variability, relation to systemic arterial pressure, and plasma catecholamines.

    OpenAIRE

    Richards, A M; Ikram, H; Crozier, I G; Nicholls, M G; Jans, S

    1990-01-01

    The variability of pulmonary arterial pressure, the relation of pulmonary pressure to systemic pressure, pulmonary pressure responses to stimuli (exercise, hypoxia, smoking, free ambulation), and plasma catecholamine responses were assessed in five patients with primary pulmonary hypertension. Ambulatory monitoring techniques provided data for the computerised analysis of continuous, beat-to-beat, direct recordings of both pulmonary and systemic arterial pressures for 8 to 10 hours. The absol...

  8. [Novel immunopathological approaches to pulmonary arterial hypertension].

    Science.gov (United States)

    Perros, Frédéric; Montani, David; Dorfmüller, Peter; Huertas, Alice; Chaumais, Marie-Camille; Cohen-Kaminsky, Sylvia; Humbert, Marc

    2011-04-01

    Inflammation is important for the initiation and the maintenance of vascular remodeling in the most commun animal models of pulmonary hypertension (PH), and its therapeutical targeting blocks PH development in these models. In human, pulmonary vascular lesions of PH are also the source of an intense chemokine production, linked to inflammatory cell recruitment. However, arteritis is uncommon in PH patients. Of note, current PH treatments have immunomodulatory properties. In addition, some studies have shown a correlation between levels of circulating inflammatory mediators and patients' survival. The study of autoimmunity in the pathophysiology of pulmonary arterial hypertension is becoming an area of intense investigation. New immunopathological approaches to PH should allow the development of innovative treatments for this very severe condition.

  9. Evaluation of the grading and disorder assessment of congenital heart disease with pulmonary arterial hypertension

    International Nuclear Information System (INIS)

    Ding Zhongru; Qin Yongwen

    2008-01-01

    Pulmonary arterial hypertension is one of the most common and serious complications in congenital heart disease. Identification of whether the pulmonary, arterial hypertension is dynamic or resistance remains as the great importance for deciding to transfer for surgery, intervention or conservative therapy and directly concerning with the prognosis and choice of treatment. This review mainly deals with the problems such as grading, staging, pathophysiology and the correlative mechanism with clinical assessment of pulmonary. arterial hypertension in congenital heart disease and furthermore providing comprehensive informations for clinical diagnosis and treatment. (authors)

  10. Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

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    Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong [Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang (Korea, Republic of); Kim, Seon Jeong [Dept. of Radiology, Myongji Hospital, Goyang (Korea, Republic of)

    2017-05-15

    Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia.

  11. Changes in large pulmonary arterial viscoelasticity in chronic pulmonary hypertension.

    Directory of Open Access Journals (Sweden)

    Zhijie Wang

    Full Text Available Conduit pulmonary artery (PA stiffening is characteristic of pulmonary arterial hypertension (PAH and is an excellent predictor of mortality due to right ventricular (RV overload. To better understand the impact of conduit PA stiffening on RV afterload, it is critical to examine the arterial viscoelastic properties, which require measurements of elasticity (energy storage behavior and viscosity (energy dissipation behavior. Here we hypothesize that PAH leads to frequency-dependent changes in arterial stiffness (related to elasticity and damping ratio (related to viscosity in large PAs. To test our hypothesis, PAH was induced by the combination of chronic hypoxia and an antiangiogenic compound (SU5416 treatment in mice. Static and sinusoidal pressure-inflation tests were performed on isolated conduit PAs at various frequencies (0.01-20 Hz to obtain the mechanical properties in the absence of smooth muscle contraction. Static mechanical tests showed significant stiffening of large PAs with PAH, as expected. In dynamic mechanical tests, structural stiffness (κ increased and damping ratio (D decreased at a physiologically relevant frequency (10 Hz in hypertensive PAs. The dynamic elastic modulus (E, a material stiffness, did not increase significantly with PAH. All dynamic mechanical properties were strong functions of frequency. In particular, κ, E and D increased with increasing frequency in control PAs. While this behavior remained for D in hypertensive PAs, it reversed for κ and E. Since these novel dynamic mechanical property changes were found in the absence of changes in smooth muscle cell content or contraction, changes in collagen and proteoglycans and their interactions are likely critical to arterial viscoelasticity in a way that has not been previously described. The impact of these changes in PA viscoelasticity on RV afterload in PAH awaits further investigation.

  12. Changes in Large Pulmonary Arterial Viscoelasticity in Chronic Pulmonary Hypertension

    Science.gov (United States)

    Wang, Zhijie; Lakes, Roderic S.; Golob, Mark; Eickhoff, Jens C.; Chesler, Naomi C.

    2013-01-01

    Conduit pulmonary artery (PA) stiffening is characteristic of pulmonary arterial hypertension (PAH) and is an excellent predictor of mortality due to right ventricular (RV) overload. To better understand the impact of conduit PA stiffening on RV afterload, it is critical to examine the arterial viscoelastic properties, which require measurements of elasticity (energy storage behavior) and viscosity (energy dissipation behavior). Here we hypothesize that PAH leads to frequency-dependent changes in arterial stiffness (related to elasticity) and damping ratio (related to viscosity) in large PAs. To test our hypothesis, PAH was induced by the combination of chronic hypoxia and an antiangiogenic compound (SU5416) treatment in mice. Static and sinusoidal pressure-inflation tests were performed on isolated conduit PAs at various frequencies (0.01–20 Hz) to obtain the mechanical properties in the absence of smooth muscle contraction. Static mechanical tests showed significant stiffening of large PAs with PAH, as expected. In dynamic mechanical tests, structural stiffness (κ) increased and damping ratio (D) decreased at a physiologically relevant frequency (10 Hz) in hypertensive PAs. The dynamic elastic modulus (E), a material stiffness, did not increase significantly with PAH. All dynamic mechanical properties were strong functions of frequency. In particular, κ, E and D increased with increasing frequency in control PAs. While this behavior remained for D in hypertensive PAs, it reversed for κ and E. Since these novel dynamic mechanical property changes were found in the absence of changes in smooth muscle cell content or contraction, changes in collagen and proteoglycans and their interactions are likely critical to arterial viscoelasticity in a way that has not been previously described. The impact of these changes in PA viscoelasticity on RV afterload in PAH awaits further investigation. PMID:24223157

  13. Pulmonary endothelial cell DNA methylation signature in pulmonary arterial hypertension.

    Science.gov (United States)

    Hautefort, Aurélie; Chesné, Julie; Preussner, Jens; Pullamsetti, Soni S; Tost, Jorg; Looso, Mario; Antigny, Fabrice; Girerd, Barbara; Riou, Marianne; Eddahibi, Saadia; Deleuze, Jean-François; Seeger, Werner; Fadel, Elie; Simonneau, Gerald; Montani, David; Humbert, Marc; Perros, Frédéric

    2017-08-08

    Pulmonary arterial hypertension (PAH) is a severe and incurable pulmonary vascular disease. One of the primary origins of PAH is pulmonary endothelial dysfunction leading to vasoconstriction, aberrant angiogenesis and smooth muscle cell proliferation, endothelial-to-mesenchymal transition, thrombosis and inflammation. Our objective was to study the epigenetic variations in pulmonary endothelial cells (PEC) through a specific pattern of DNA methylation. DNA was extracted from cultured PEC from idiopathic PAH ( n = 11), heritable PAH ( n = 10) and controls ( n = 18). DNA methylation was assessed using the Illumina HumanMethylation450 Assay. After normalization, samples and probes were clustered according to their methylation profile. Differential clusters were functionally analyzed using bioinformatics tools. Unsupervised hierarchical clustering allowed the identification of two clusters of probes that discriminates controls and PAH patients. Among 147 differential methylated promoters, 46 promoters coding for proteins or miRNAs were related to lipid metabolism. Top 10 up and down-regulated genes were involved in lipid transport including ABCA1, ABCB4, ADIPOQ, miR-26A, BCL2L11. NextBio meta-analysis suggested a contribution of ABCA1 in PAH. We confirmed ABCA1 mRNA and protein downregulation specifically in PAH PEC by qPCR and immunohistochemistry and made the proof-of-concept in an experimental model of the disease that its targeting may offer novel therapeutic options. In conclusion, DNA methylation analysis identifies a set of genes mainly involved in lipid transport pathway which could be relevant to PAH pathophysiology.

  14. Pulmonary artery aneurysm in Bechcet's disease: helical computed tomography study

    International Nuclear Information System (INIS)

    Munoz, J.; Caballero, P.; Olivera, M. J.; Cajal, M. L.; Caniego, J. L.

    2000-01-01

    Behcet's disease is a vasculitis of unknown etiology that affects arteries and veins of different sizes and can be associated with pulmonary artery aneurysms. We report the case of a patient with Behcet's disease and a pulmonary artery aneurysm who was studied by means of plain chest X ray, helical computed tomography and pulmonary arteriography. Helical computed tomography is a reliable technique for the diagnosis and follow-up of these patients. (Author) 9 refs

  15. Drug-induced pulmonary arterial hypertension: a recent outbreak

    Directory of Open Access Journals (Sweden)

    Gérald Simonneau

    2013-09-01

    Full Text Available Pulmonary arterial hypertension (PAH is a rare disorder characterised by progressive obliteration of the pulmonary microvasculature resulting in elevated pulmonary vascular resistance and premature death. According to the current classification PAH can be associated with exposure to certain drugs or toxins, particularly to appetite suppressant intake drugs, such as aminorex, fenfluramine derivatives and benfluorex. These drugs have been confirmed to be risk factors for PAH and were withdrawn from the market. The supposed mechanism is an increase in serotonin levels, which was demonstrated to act as a growth factor for the pulmonary artery smooth muscle cells. Amphetamines, phentermine and mazindol were less frequently used, but are considered possible risk factors, for PAH. Dasatinib, dual Src/Abl kinase inhibitor, used in the treatment of chronic myelogenous leukaemia was associated with cases of severe PAH, potentially in part reversible after dasatinib withdrawal. Recently, several studies have raised the issue of potential endothelial dysfunction that could be induced by interferon, and a few cases of PAH have been reported with interferon therapy. PAH remains a rare complication of these drugs, suggesting possible individual susceptibility, and further studies are needed to identify patients at risk of drug-induced PAH.

  16. Abnormal pulmonary artery stiffness in pulmonary arterial hypertension: in vivo study with intravascular ultrasound.

    Directory of Open Access Journals (Sweden)

    Edmund M T Lau

    Full Text Available BACKGROUND: There is increasing recognition that pulmonary artery stiffness is an important determinant of right ventricular (RV afterload in pulmonary arterial hypertension (PAH. We used intravascular ultrasound (IVUS to evaluate the mechanical properties of the elastic pulmonary arteries (PA in subjects with PAH, and assessed the effects of PAH-specific therapy on indices of arterial stiffness. METHOD: Using IVUS and simultaneous right heart catheterisation, 20 pulmonary segments in 8 PAH subjects and 12 pulmonary segments in 8 controls were studied to determine their compliance, distensibility, elastic modulus and stiffness index β. PAH subjects underwent repeat IVUS examinations after 6-months of bosentan therapy. RESULTS: AT BASELINE, PAH SUBJECTS DEMONSTRATED GREATER STIFFNESS IN ALL MEASURED INDICES COMPARED TO CONTROLS: compliance (1.50±0.11×10(-2 mm(2/mmHg vs 4.49±0.43×10(-2 mm(2/mmHg, p<0.0001, distensibility (0.32±0.03%/mmHg vs 1.18±0.13%/mmHg, p<0.0001, elastic modulus (720±64 mmHg vs 198±19 mmHg, p<0.0001, and stiffness index β (15.0±1.4 vs 11.0±0.7, p = 0.046. Strong inverse exponential associations existed between mean pulmonary artery pressure and compliance (r(2 = 0.82, p<0.0001, and also between mean PAP and distensibility (r(2 = 0.79, p = 0.002. Bosentan therapy, for 6-months, was not associated with any significant changes in all indices of PA stiffness. CONCLUSION: Increased stiffness occurs in the proximal elastic PA in patients with PAH and contributes to the pathogenesis RV failure. Bosentan therapy may not be effective at improving PA stiffness.

  17. Secretory clusterin is upregulated in rats with pulmonary arterial hypertension induced by systemic-to-pulmonary shunts and exerts important roles in pulmonary artery smooth muscle cells.

    Science.gov (United States)

    Liu, X; Meng, L; Li, J; Meng, J; Teng, X; Gu, H; Hu, S; Wei, Y

    2015-02-01

    Phenotype modification of pulmonary artery smooth muscle cells (PASMCs) (excessive proliferation, migration and impaired apoptosis) plays central roles in pulmonary vascular remodelling of pulmonary arterial hypertension (PAH); however, the potential mechanism and contributing factors involved in the phenotype alteration in PASMCs are still not completely elucidated. This study attempted to investigate the expression pattern of secretory clusterin (sCLU), a prosurvival protein, in systemic-to-pulmonary shunt-induced PAH rats and the potential roles of sCLU in pulmonary vascular remodelling. An original rat model of systemic-to-pulmonary shunt-induced PAH was established by combined surgery as we previously reported. Lung tissues were harvested at specific time points for real-time polymerase chain reaction, Western blot and immunohistochemisty analysis; meanwhile, plasma was collected for enzyme-linked immunosorbent assay. Cell culture experiments were performed using cultured human PASMCs (HPASMCs). Expression of sCLU was significantly increased in lungs exposed to systemic-to-pulmonary shunt. Moreover, plasma sCLU levels were markedly elevated with the progression of PAH in rats and also presented a positive correlation with pulmonary hemodynamic indices. In vitro cell culture assay indicated that sCLU expression and secretion increased with the phenotype modification of HPASMCs; furthermore, sCLU promoted HPASMCs proliferation, migration and apoptosis resistance, at least in part, via Erk1/2 and Akt signalling pathways. These results demonstrate that sCLU is functionally an important phenotype modulator of PASMCs, and its upregulation in lung tissues may exert a deteriorative role in pulmonary vascular remodelling. © 2014 Scandinavian Physiological Society. Published by John Wiley & Sons Ltd.

  18. Therapeutic effect of prostaglandin E1 in monocrotaline-induced pulmonary arterial hypertension rats.

    Science.gov (United States)

    Lee, Jae Chul

    2017-03-01

    Pulmonary arterial hypertension (PAH) is a severe pulmonary vascular disease characterized by sustained increase in pulmonary arterial pressure and excessive thickening and remodeling of distal small pulmonary arteries. During disease progression, PAH include increase in mean pulmonary arterial pressure, right ventricular (RV) enlargement, increased pulmonary vascular resistance, and smooth muscle hypertrophy in pulmonary arterioles. Several anti-PAH therapies targeting various pathways involved in PAH progression have been approved by the Food and Drug Adminstration. However, many of the currently available anti-PAH drugs suffer from a number of limitations, including short biological half-life, and poor pulmonary selectivity. Prostaglandin E1 (PGE1) is a potent vasodilator with selectivity toward pulmonary circulation when it is administered via the pulmonary route. However, PGE1 has a very short half-life of 5-10 minutes. Therefore, we hypothesized that long-term effect of PGE1 could reduce mal-adaptive structural remodeling of the lung and heart and prevent ventricular arrhythmias in monocrotaline-induced rat model of PAH. Our results revealed that PGE1 reduced ventricular hypertrophy, protein expressions of endothelin-1 and endothelin receptor A, and the expression of fibrosis. These results support the notion that PGE1 can improve the functional properties of RV, highlighting its potential benefits for heart and lung impairment.

  19. Serotonin inhibits apoptosis of pulmonary artery smooth muscle cells through 5-HT2A receptors involved in the pulmonary artery remodeling of pulmonary artery hypertension.

    Science.gov (United States)

    Liu, Ya; Tian, Hongyan; Yan, Xiaoli; Fan, Fenling; Wang, Wenping; Han, Junli

    2013-03-01

    Decreased pulmonary artery smooth muscle cell (PASMC) apoptosis play a key role in pulmonary artery remodeling during pulmonary artery hypertension (PAH), but the mechanisms involved are unclear. Serotonin (5-HT) inhibits apoptosis in many pathologic processes by activating the 5-HT2A receptor. Therefore, we hypothesized that 5-HT may be the promoter of decreased apoptosis in PAH through the 5-HT2A receptor. We found that inhibition of the 5-HT2A receptor prevented the increase in pulmonary artery pressure and pulmonary artery remodeling in rats stimulated by monocrotaline. This effect was accompanied by increased apoptosis in the pulmonary artery. Cultured PASMCs stimulated with 5-HT showed a decrease in apoptosis with increased phosphorylation of extracellular signal regulated kinase 1/2 (ERK1/2), pyruvate dehydrogenase kinase (PDK), and mitochondrial transmembrane potential. These effects were markedly prevented by a 5-HT2A receptor inhibitor, an ERK1/2 activation inhibitor peptide I, or a PDK inhibitor. In conclusion, 5-HT inhibited PASMC apoptosis by activating the 5-HT2A receptor through the pERK1/2 and PDK pathways.5-HT decreasing apoptosis through 5-HT2A receptor is involved, at least in part, in pulmonary artery remolding.

  20. PULMONARY ARTERIAL HYPERTENSION IN CONNECTIVE TISSUE DISEASES

    Directory of Open Access Journals (Sweden)

    A. V. Volkov

    2015-01-01

    Full Text Available The lecture considers the problem of one of the rare manifestations of systemic diseases (pulmonary arterial hypertension and shows the need for early diagnosis, careful differential diagnosis, and verification of diagnosis, by applying invasive procedures to evaluate central hemodynamics. It gives a model for screening patients with systemic sclerosis, which simplifies the determination of indications for the use of diagnostic verification methods. Current approaches to drug therapy are described and the issues of better survival of patients with poor prognosis of this disorder are discussed. 

  1. Giant coronary artery aneurysm after Takeuchi repair for anomalous left coronary artery from the pulmonary artery.

    Science.gov (United States)

    Dunlay, Shannon M; Bonnichsen, Crystal R; Dearani, Joseph A; Warnes, Carole A

    2014-01-01

    A 33-year-old woman with an anomalous left coronary artery arising from the pulmonary artery who had undergone Takeuchi repair at age 7 years presented for evaluation. The Takeuchi procedure creates an aortopulmonary window and an intrapulmonary tunnel that baffles the left coronary artery to the aorta. A mediastinal mass was identified as a giant aneurysm of the left coronary artery resulting in compression of the pulmonary artery and left upper pulmonary vein. The patient underwent open repair with patch closure at the aortic entrance of the left coronary Takeuchi repair and resection and evacuation of the aneurysm. A saphenous vein graft to the left anterior descending artery was performed. Postoperative echocardiography demonstrated normal left ventricular function. This is the first reported case of giant aneurysm formation after Takeuchi repair. The reported complications have included the development of pulmonary artery stenosis at the intrapulmonary baffle, baffle leak, decreased left ventricular function, and mitral regurgitation. In conclusion, late complications of the Takeuchi procedure are common, underscoring the importance of lifelong follow-up at a center with experience in treating coronary anomalies. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Pulmonary Arterial Capacitance Predicts Cardiac Events in Pulmonary Hypertension Due to Left Heart Disease.

    Directory of Open Access Journals (Sweden)

    Koichi Sugimoto

    Full Text Available Although pulmonary hypertension due to left heart disease (LHD-PH accounts for the largest proportion of pulmonary hypertension, few reports on the epidemiological analysis of LHD-PH exist. Recently, pulmonary arterial capacitance (PAC has attracted attention as a possible factor of right ventricular afterload along with pulmonary vascular resistance. We therefore investigated the clinical significance of PAC in LHD-PH.The subject consisted of 252 LHD-PH patients (145 men, mean age 63.4 ± 14.7 years diagnosed by right heart catheterization. PAC was estimated by the ratio between stroke volume and pulmonary arterial pulse pressure. Patients were classified into four groups according to the PAC (1st quartile was 0.74 to 1.76 ml/mmHg, the 2nd quartile 1.77 to 2.53 ml/mmHg, the 3rd quartile 2.54 to 3.59 ml/mmHg, and the 4th quartile 3.61 to 12.14 ml/mmHg. The end-points were defined as rehospitalization due to worsening heart failure and/or cardiac death. The Cox proportional hazard regression model was used to determine what variables were associated with cardiac events.The patients in the 1st quartile had the lowest cardiac index and stroke volume index, and the highest mean pulmonary arterial pressure, mean pulmonary capillary wedge pressure, and pulmonary vascular resistance compared with the 2nd, 3rd, and 4th quartiles. Fifty-four patients experienced cardiac events during the follow-up period (median 943 days. The event-free rate of the 1st quartile was significantly lower than that of the 3rd and 4th quartiles (66.7% vs 82.5% [3rd quartile], P = 0.008; and 92.1% [4th quartile], P < 0.001. The Cox hazard analysis revealed that PAC was significantly associated with cardiac events (HR 0.556, 95% CI 0.424-0.730, P < 0.001.PAC is useful in the prediction of cardiac event risk in LHD-PH patients.

  3. Pulmonary Arterial Capacitance Predicts Cardiac Events in Pulmonary Hypertension Due to Left Heart Disease

    Science.gov (United States)

    Sugimoto, Koichi; Yoshihisa, Akiomi; Nakazato, Kazuhiko; Jin, Yuichiro; Suzuki, Satoshi; Yokokawa, Tetsuro; Misaka, Tomofumi; Yamaki, Takayoshi; Kunii, Hiroyuki; Suzuki, Hitoshi; Saitoh, Shu-ichi; Takeishi, Yasuchika

    2016-01-01

    Background Although pulmonary hypertension due to left heart disease (LHD-PH) accounts for the largest proportion of pulmonary hypertension, few reports on the epidemiological analysis of LHD-PH exist. Recently, pulmonary arterial capacitance (PAC) has attracted attention as a possible factor of right ventricular afterload along with pulmonary vascular resistance. We therefore investigated the clinical significance of PAC in LHD-PH. Methods The subject consisted of 252 LHD-PH patients (145 men, mean age 63.4 ± 14.7 years) diagnosed by right heart catheterization. PAC was estimated by the ratio between stroke volume and pulmonary arterial pulse pressure. Patients were classified into four groups according to the PAC (1st quartile was 0.74 to 1.76 ml/mmHg, the 2nd quartile 1.77 to 2.53 ml/mmHg, the 3rd quartile 2.54 to 3.59 ml/mmHg, and the 4th quartile 3.61 to 12.14 ml/mmHg). The end-points were defined as rehospitalization due to worsening heart failure and/or cardiac death. The Cox proportional hazard regression model was used to determine what variables were associated with cardiac events. Results The patients in the 1st quartile had the lowest cardiac index and stroke volume index, and the highest mean pulmonary arterial pressure, mean pulmonary capillary wedge pressure, and pulmonary vascular resistance compared with the 2nd, 3rd, and 4th quartiles. Fifty-four patients experienced cardiac events during the follow-up period (median 943 days). The event-free rate of the 1st quartile was significantly lower than that of the 3rd and 4th quartiles (66.7% vs 82.5% [3rd quartile], P = 0.008; and 92.1% [4th quartile], P < 0.001). The Cox hazard analysis revealed that PAC was significantly associated with cardiac events (HR 0.556, 95% CI 0.424–0.730, P < 0.001). Conclusion PAC is useful in the prediction of cardiac event risk in LHD-PH patients. PMID:27875533

  4. Pedunculated Pulmonary Artery Sarcoma Suggested by Transthoracic Echocardiography.

    Science.gov (United States)

    Wang, Xiaobing; Ren, Weidong; Yang, Jun

    2016-04-01

    Pulmonary artery sarcoma (PAS) is an extremely rare malignancy. It is usually found after it grows large enough to occupy almost the entire lumen of the pulmonary artery and causes serious clinical symptoms. Thus, it is usually difficult to distinguish PAS from pulmonary thromboembolism based on imaging examinations. Few case reports had shown the attachment of PAS to pulmonary artery, a key characteristic for diagnosis, and differential diagnosis of PAS. In this case, we found a PAS, which did not cause local obstruction and some tumor emboli, which obstructed the branches of the pulmonary arteries and caused pulmonary hypertension and clinical symptoms. Transthoracic echocardiography (TTE) revealed a part of the tumor attached to the intima of the main pulmonary artery with a peduncle and had obvious mobility, which was suggestive of PAS and differentiated it from the pulmonary thromboembolism. To our knowledge, this is the first case report of a pedunculated PAS suggested by TTE. Combined with pulmonary artery computed tomography angiography, the diagnosis of PAS is strongly suggested before the operation. This case indicates that TTE could reveal the attachment and mobility of PAS in the main pulmonary and may provide useful information for the diagnosis and differential diagnosis of PAS, especially a pedunculated PAS. © 2015, Wiley Periodicals, Inc.

  5. Isolated Unilateral Pulmonary Artery Agenesis complicated by Symptomatic Aspergilloma

    LENUS (Irish Health Repository)

    Daly, A

    2017-11-01

    Isolated unilateral pulmonary artery agenesis is a rare diagnosis. Poor blood flow to the lung parenchyma renders the tissue susceptible to opportunistic infections. We present the unusual case of isolated unilateral pulmonary artery agenesis complicated by aspergilloma. Management options and considerations are discussed.

  6. Pulmonary artery pulse pressure and wave reflection in chronic pulmonary thromboembolism and primary pulmonary hypertension.

    Science.gov (United States)

    Castelain, V; Hervé, P; Lecarpentier, Y; Duroux, P; Simonneau, G; Chemla, D

    2001-03-15

    The purpose of this time-domain study was to compare pulmonary artery (PA) pulse pressure and wave reflection in chronic pulmonary thromboembolism (CPTE) and primary pulmonary hypertension (PPH). Pulmonary artery pressure waveform analysis provides a simple and accurate estimation of right ventricular afterload in the time-domain. Chronic pulmonary thromboembolism and PPH are both responsible for severe pulmonary hypertension. Chronic pulmonary thromboembolism and PPH predominantly involve proximal and distal arteries, respectively, and may lead to differences in PA pressure waveform. High-fidelity PA pressure was recorded in 14 patients (7 men/7 women, 46 +/- 14 years) with CPTE (n = 7) and PPH (n = 7). We measured thermodilution cardiac output, mean PA pressure (MPAP), PA pulse pressure (PAPP = systolic - diastolic PAP) and normalized PAPP (nPAPP = PPAP/MPAP). Wave reflection was quantified by measuring Ti, that is, the time between pressure upstroke and the systolic inflection point (Pi), deltaP, that is, the systolic PAP minus Pi difference, and the augmentation index (deltaP/PPAP). At baseline, CPTE and PPH had similar cardiac index (2.4 +/- 0.4 vs. 2.5 +/- 0.5 l/min/m2), mean PAP (59 +/- 9 vs. 59 +/- 10 mm Hg), PPAP (57 +/- 13 vs. 53 +/- 13 mm Hg) and nPPAP (0.97 +/- 0.16 vs. 0.89 +/- 0.13). Chronic pulmonary thromboembolism had shorter Ti (90 +/- 17 vs. 126 +/- 16 ms, p PPAP (0.26 +/- 0.01 vs. 0.09 +/- 0.07, p < 0.01). Our study indicated that: 1) CPTE and PPH with severe pulmonary hypertension had similar PA pulse pressure, and 2) wave reflection is elevated in both groups, and CPTE had increased and anticipated wave reflection as compared with PPH, thus suggesting differences in the pulsatile component of right ventricular afterload.

  7. Endothelin receptor antagonist and airway dysfunction in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Borst Mathias M

    2009-12-01

    Full Text Available Abstract Background In idiopathic pulmonary arterial hypertension (IPAH, peripheral airway obstruction is frequent. This is partially attributed to the mediator dysbalance, particularly an excess of endothelin-1 (ET-1, to increased pulmonary vascular and airway tonus and to local inflammation. Bosentan (ET-1 receptor antagonist improves pulmonary hemodynamics, exercise limitation, and disease severity in IPAH. We hypothesized that bosentan might affect airway obstruction. Methods In 32 IPAH-patients (19 female, WHO functional class II (n = 10, III (n = 22; (data presented as mean ± standard deviation pulmonary vascular resistance (11 ± 5 Wood units, lung function, 6 minute walk test (6-MWT; 364 ± 363.7 (range 179.0-627.0 m, systolic pulmonary artery pressure, sPAP, 79 ± 19 mmHg, and NT-proBNP serum levels (1427 ± 2162.7 (range 59.3-10342.0 ng/L were measured at baseline, after 3 and 12 months of oral bosentan (125 mg twice per day. Results and Discussion At baseline, maximal expiratory flow at 50 and 25% vital capacity were reduced to 65 ± 25 and 45 ± 24% predicted. Total lung capacity was 95.6 ± 12.5% predicted and residual volume was 109 ± 21.4% predicted. During 3 and 12 months of treatment, 6-MWT increased by 32 ± 19 and 53 ± 69 m, respectively; p Conclusion This study gives first evidence in IPAH, that during long-term bosentan, improvement of hemodynamics, functional parameters or serum biomarker occur independently from persisting peripheral airway obstruction.

  8. Inspiratory muscle training in pulmonary arterial hypertension.

    Science.gov (United States)

    Saglam, Melda; Arikan, Hulya; Vardar-Yagli, Naciye; Calik-Kutukcu, Ebru; Inal-Ince, Deniz; Savci, Sema; Akdogan, Ali; Yokusoglu, Mehmet; Kaya, Ergun Baris; Tokgozoglu, Lale

    2015-01-01

    The purpose of this study was to investigate the effects of inspiratory muscle training (IMT) on functional capacity, respiratory muscle strength, pulmonary function, quality of life, and fatigue and dyspnea perception in patients with pulmonary arterial hypertension (PAH). Twenty-nine clinically stable PAH patients were included in this study. These patients were randomly assigned to a 6-week IMT program (14 patients) or to a sham IMT protocol (15 patients). Before and after the treatment, pulmonary function, respiratory muscle strength, functional capacity, dyspnea and fatigue perception, and quality of life were evaluated. There were significant increases in maximal inspiratory and expiratory pressure, forced expiratory volume in 1 second (% predicted) and 6-minute walk distance in the IMT group compared with the control group (P Inspiratory muscle training promotes significant improvements in respiratory muscle strength and functional capacity, thus resulting in a reduction of dyspnea during activities of daily living and less fatigue in PAH patients. Inspiratory muscle training is a clinically practical treatment for PAH without any complications.

  9. Apical Hypertrophic Cardiomyopathy in Association with PulmonaryArtery Hypertension

    Directory of Open Access Journals (Sweden)

    Mehdi Peighambari

    2012-09-01

    Full Text Available Apical Hypertrophic Cardiomyopathy is an uncommon condition constituting 1% -2% of the cases with Hypertrophic Cardiomyopathy (HCM diagnosis. We interestingly report two patients with apical hypertrophic cardiomyopathy in association with significant pulmonary artery hypertension without any other underlying reason for pulmonary hypertension. The patients were assessed by echocardiography, cardiac catheterization and pulmonary function parameters study.

  10. Congenital unilateral absence of the pulmonary artery in adults

    International Nuclear Information System (INIS)

    Gonzalez Garcia, Mauricio; Escalante Mora, Hector A; Lozano Castillo, Alfonso J

    2000-01-01

    Unilateral absence of a pulmonary artery is a rare anomaly. It occurs with pulmonary ipsilateral hypoplasia and it's frequently associated with other cardiovascular malformations. The majority of the cases are diagnosed in childhood. This is a case report of two adult patients of the Hospital Central de la Policia Nacional in Bogota, Colombia, with unilateral absence of the pulmonary artery one isolated and the other with patent ductus arteriosus. We describe the clinical and roentgenographic findings of this congenital anomaly

  11. Pulmonary artery dilatation: an overlooked mechanism for angina pectoris.

    Science.gov (United States)

    Ginghina, Carmen; Popescu, Bogdan A; Enache, Roxana; Ungureanu, Catalina; Deleanu, Dan; Platon, Pavel

    2008-07-01

    Dilatation of the pulmonary artery may lead to the compression of adjacent structures. Of those, the extrinsic compression of the left main coronary artery is the most worrisome. We present the case of a 48-year-old woman who was diagnosed with pulmonary artery dilatation due to severe, thromboembolic pulmonary hypertension. She also had angina and coronary angiography revealed a 70% ostial stenosis of the left main coronary artery. The presence of this isolated lesion in a young woman without risk factors for atherosclerosis suggests extrinsic compression of the left main coronary artery by the dilated pulmonary artery as the likely mechanism. The patient underwent direct stenting of the left main coronary stenosis with a good result.

  12. [Cardiac catheterization and pulmonary vasoreactivity testing in children with idiopathic pulmonary arterial hypertension].

    Science.gov (United States)

    Zhang, Chen; Li, Qiangqiang; Liu, Tianyang; Gu, Hong

    2014-06-01

    As an important method of hemodynamic assessment in idiopathic pulmonary arterial hypertension (IPAH), cardiac catheterization combined with pulmonary vasoreactivity testing remains with limited experience in children, and the acute pulmonary vasodilator agents as well as response criteria for vasoreactivity testing remain controversial. The aim of this study was to investigate the clinical importance, agent selection, and responder definition of cardiac catheterization combined with pulmonary vasoreactivity testing in pediatric IPAH. The patients admitted to Department of Pediatric Cardiology of Beijing Anzhen Hospital between April 2009 and September 2013 with suspected IPAH, under 18 years of age, with WHO functional class II or III, were enrolled. All the patients were arranged to receive left and right heart catheterization and pulmonary vasoreactivity testing with inhalation of pure oxygen and iloprost (PGI2) respectively. Hemodynamic changes were analyzed, and two criteria, the European Society of Cardiology recommendation criteria (Sitbon criteria) and traditional application criteria (Barst criteria), were used to evaluate the test results. Thirty-nine cases of children with suspected IPAH underwent cardiac catheterization. In 4 patients IPAH was excluded; 4 patients developed pulmonary hypertension crisis. The other 31 patients received standard cardiac catheterization and pulmonary vasoreactivity testing. Baseline mean pulmonary artery pressure (mPAP) was (66 ± 16) mmHg (1 mmHg = 0.133 kPa), and pulmonary vascular resistance index (PVRI) (17 ± 8) Wood U · m². After inhalation of pure oxygen, mPAP fell to (59 ± 16) mmHg, and PVRI to (14 ± 8) Wood U · m² (t = 4.88 and 4.56, both P hypertension crisis is an important complication of cardiac catheterization in pediatric IPAH. Younger age, general anesthesia, crisis history, and poor heart function are important risk factors for pulmonary hypertension crisis. PGI2 is a relatively ideal agent for

  13. [Drug therapy of pulmonary arterial hypertension in 2014].

    Science.gov (United States)

    Aschermann, Michael; Jansa, Pavel

    2014-04-01

    Pulmonary arterial hypertension (PAH) is a primary pulmonary arteriolar disease, characterized by a progressive increase in pulmonary vascular resistance and pressure in the pulmonary circulation. It progressively leads to hypertrophy of the right ventricle and with no treatment to its failure and patient´s death. Etiology of pulmonary hypertension (PH) has been reclassified repeatedly, most recently during the 4th World Symposium on Pulmonary Hypertension held in 2008 [1]. Currently, the first group contains PAH with either unknown or known cause (systemic connective tissue disease, liver disease, congenital heart disease, HIV infection, abuse of anorexic agents). Current drug therapy of PAH is divided into conventional (anticoagulant therapy, calcium channel blockers, therapy of chronic heart failure) and specific (prostanoids, endothelium receptor antagonists, phosphodiesterase 5 inhibitors). Patients with positive vasodilator test are indicated for the high doses treatment of calcium channel blockers. Patients with negative vasodilator test are indicated for chronic anticoagulant therapy and specific drug therapy either as mono-therapy, or as combined therapy. Recent years have brought a wide range of new treatments modalities, especially in the field of pharmacotherapy. In addition, other treatment modalities have been tested, for example application of stem cells. Drugs in research include several groups: 1. vasodilators: fasudil, adrenonedullin, activators and stimulators of guanylate cyclase, vasoactive intestinal peptide (VIP); 2. Anti-inflammatory agents: inhibitor of elastase, antagonist of B cells, immunosuppressive agents, inhibitor of HDAC1; 3. agents affecting metabolism: nitrites, PPAR antagonists, antioxidants, serotonin receptor antagonist and serotonin transporter blockers, statins, inhibitors of Rho-kinase; 4. apoptosis inductors of smooth muscle cells: tyrosine-kinase inhibitors, elastase inhibitors; 5. agents influencing vascular regeneration

  14. Transcatheter Treatment of “Pulmonary Artery Hypertension” due to Patent Ductus Arteriosus and Pulmonary Artery Stenosis

    OpenAIRE

    Gaio, Gianpiero; Santoro, Giuseppe; D'Alto, Michele; Palladino, Maria Teresa; Russo, Maria Giovanna; Caianiello, Giuseppe; Calabrò, Raffaele

    2006-01-01

    The association between large, left-sided patent ductus arteriosus and severe, peripheral, right pulmonary artery stenosis with no other cardiac malformation is an unreported condition that might be misdiagnosed as pulmonary hypertension due to long-standing ductal shunt. A 57-year-old man with supposed hypertensive patent ductus arteriosus underwent confirmatory cardiac catheterization. At angiography, a severe pre-hilar right pulmonary artery stenosis (peak pressure gradient, 65 mmHg) was f...

  15. Evaluation of pulmonary artery flow in acute massive pulmonary thromboembolism with MRI

    International Nuclear Information System (INIS)

    Li Yongzhong; Li Kuncheng; Zhao Xigang; Zhao Hong

    2004-01-01

    Objective: To probe into the value of MR imaging in evaluating the pulmonary artery hemodynamics and pulmonary artery pressure in acute massive pulmonary embolism. Methods: MR studies were performed in 21 patients with acute massive pulmonary embolism (diagnosed by contrast enhanced MR pulmonary angiography) and 20 healthy volunteers. The pulmonary artery hemodynamic parameters, such as the diameters of main and right pulmonary artery, peak velocity, average velocity, flow volume, flow patterns, and ejection acceleration time in main pulmonary artery were measured. The findings in patients and volunteers were compared. The hemodynamic parameters in patients were correlated with mean pulmonary artery pressure acquired with right heart catheterization. Results: The diameters of main pulmonary artery (2.93 vs 2.52 cm) and right pulmonary artery (2.49 vs 1.92 cm) in patients and volunteers showed significant differences (t=3.55, P<0.01 and t=4.19, P<0.01, respectively); Peak velocity (85.29 vs 100.63 cm/s), average velocity (11.00 vs 17.12 cm/s), flow volume (89.15 vs 98.96 ml/s), and ejection acceleration time (105.09 vs 163.85 ms) in main pulmonary artery were significantly different between patients and volunteers (t values were 2.89, 6.37, 2.21, and 9.46, respectively; P values were 0.01, <0.01, 0.03, and <0.01, respectively). The peak velocity-time curve of main pulmonary artery acquired with velocity encoded cine of MR in patients demonstrated earlier and lower peak velocity as well as abnormal retrograde flow. In addition, linear correlations were seen between the mean pulmonary pressure and the diameter of main pulmonary artery (r=0.62, P=0.001), diameter of right pulmonary artery (r=0.63, P=0.001), and ejection acceleration time (r=-0.55, P=0.005). Conclusion: MR imaging is a promising technique not only for the detection of pulmonary thromboemboli but also for the evaluation of hemodynamic parameters in pulmonary hypertension. (author)

  16. [Pulmonary arterial hypertension: changing approaches to management].

    Science.gov (United States)

    Sidorenko, B A; Preobrazhenskiĭ, D V; Batyraliev, T A; Belenkov, Iu N

    2011-01-01

    The review is devoted to different aspects of pulmonary arterial hypertension (PAH); new classification of PAH is published in 2010. There are idiopathic PAH and PAH associated with other diseases. Current guidelines recommend to treat PAH only after the verification of diagnosis with right heart catheterization and acute tests with vasodilators. Patients-reactors should be treated with calcium antagonists. The following drugs related to one of three categories should be used in PAH: (1) prostanoids (epoprostenol, iloprost et al.); (2) blockers of endothelin receptors (bosentan, ambrisentan, sitaxsentan); (3) phosphodiesterase 5 type inhibitors (sildenafil, tadalafil et al.) In majority of cases the combined treatment is used, usually the combination of bosentan and sildenafil is used.

  17. Targeting Pulmonary Endothelial Hemoglobin α Improves Nitric Oxide Signaling and Reverses Pulmonary Artery Endothelial Dysfunction.

    Science.gov (United States)

    Alvarez, Roger A; Miller, Megan P; Hahn, Scott A; Galley, Joseph C; Bauer, Eileen; Bachman, Timothy; Hu, Jian; Sembrat, John; Goncharov, Dmitry; Mora, Ana L; Rojas, Mauricio; Goncharova, Elena; Straub, Adam C

    2017-12-01

    Pulmonary hypertension is characterized by pulmonary endothelial dysfunction. Previous work showed that systemic artery endothelial cells (ECs) express hemoglobin (Hb) α to control nitric oxide (NO) diffusion, but the role of this system in pulmonary circulation has not been evaluated. We hypothesized that up-regulation of Hb α in pulmonary ECs contributes to NO depletion and pulmonary vascular dysfunction in pulmonary hypertension. Primary distal pulmonary arterial vascular smooth muscle cells, lung tissue sections from unused donor (control) and idiopathic pulmonary artery (PA) hypertension lungs, and rat and mouse models of SU5416/hypoxia-induced pulmonary hypertension (PH) were used. Immunohistochemical, immunocytochemical, and immunoblot analyses and transfection, infection, DNA synthesis, apoptosis, migration, cell count, and protein activity assays were performed in this study. Cocultures of human pulmonary microvascular ECs and distal pulmonary arterial vascular smooth muscle cells, lung tissue from control and pulmonary hypertensive lungs, and a mouse model of chronic hypoxia-induced PH were used. Immunohistochemical, immunoblot analyses, spectrophotometry, and blood vessel myography experiments were performed in this study. We find increased expression of Hb α in pulmonary endothelium from humans and mice with PH compared with controls. In addition, we show up-regulation of Hb α in human pulmonary ECs cocultured with PA smooth muscle cells in hypoxia. We treated pulmonary ECs with a Hb α mimetic peptide that disrupts the association of Hb α with endothelial NO synthase, and found that cells treated with the peptide exhibited increased NO signaling compared with a scrambled peptide. Myography experiments using pulmonary arteries from hypoxic mice show that the Hb α mimetic peptide enhanced vasodilation in response to acetylcholine. Our findings reveal that endothelial Hb α functions as an endogenous scavenger of NO in the pulmonary endothelium

  18. A Review of Targeted Pulmonary Arterial Hypertension-Specific Pharmacotherapy

    Directory of Open Access Journals (Sweden)

    Ali Ataya

    2016-12-01

    Full Text Available Significant advances in the understanding of the pathophysiology of pulmonary arterial hypertension over the past two decades have led to the development of targeted therapies and improved patient outcomes. Currently, a broad armamentarium of pulmonary arterial hypertension-specific drugs exists to assist in the treatment of this complex disease state. In this manuscript, we provide a comprehensive review of the current Food and Drug Administration (FDA-approved pulmonary arterial hypertension-specific therapies, and their supporting evidence for adults, targeting the nitric oxide, soluble guanylate cyclase, endothelin, and prostacyclin pathways.

  19. [Aerosolized iloprost therapy for pulmonary hypertensive crisis in 4 patients with idiopathic pulmonary arterial hypertension].

    Science.gov (United States)

    Deng, Ke-wu; Zhou, Yu-jie; Xu, Xi-qi; Wu, Ming-ying; Wang, Guo-hong; Bian, Hong; Chen, Bo; Wang, Chun-bo

    2012-10-01

    To summary the efficacy and safety of aerosolized iloprost in patients with pulmonary hypertensive crisis. On the basis of conventional therapy, aerosolized iloprost (10 µg per time for 10 - 15 min in 2 hours interval, 8 times per day) was administered to four patients with idiopathic pulmonary arterial hypertension and pulmonary hypertensive crisis. Blood pressure, heart rate, systemic artery oxygen saturation, systolic pulmonary arterial pressure (sPAP) measured by echocardiography and the adverse events were analyzed. After aerosolized iloprost therapy, sPAP was significantly decreased and systemic artery oxygen saturation was improved. Adverse events (nausea, vomiting, diarrhea, dry cough) were observed in two patients, and the iloprost use was stopped in one patient due to severe vomiting and diarrhea. Aerosolized iloprost could significantly reduce the sPAP and improve the systemic artery oxygen saturation in patients with pulmonary hypertension crisis.

  20. Longitudinal distribution of pulmonary vascular resistance after endotoxin administration in sheep.

    Science.gov (United States)

    Pearl, R G; Baer, E R; Siegel, L C; Benson, G V; Rice, S A

    1992-01-01

    Pulmonary hypertension may increase pulmonary capillary pressure and exacerbate pulmonary edema in acute respiratory failure. The effects of pulmonary hypertension on pulmonary capillary pressure depend on the longitudinal distribution of pulmonary vascular resistance. Since pulmonary hypertension occurs during acute respiratory failure, we hypothesized that acute respiratory failure may produce time-dependent changes in the longitudinal distribution of pulmonary vascular resistance. Therefore, we measured pulmonary capillary pressure and the longitudinal distribution of pulmonary vascular resistance in an animal model of acute respiratory failure. Escherichia coli endotoxin (2.5 to 5.0 micrograms/kg) was administered over a 1-hr period in eight anesthetized sheep. Pulmonary and systemic hemodynamics, including pulmonary artery occlusion pressure (PAOP), pulmonary capillary pressure, and the longitudinal distribution of pulmonary vascular resistance, were measured over the next 5 hrs. Pulmonary capillary pressure was estimated by analysis of the pressure decay following pulmonary artery balloon inflation. Endotoxin administration resulted in sustained pulmonary hypertension for the subsequent 5 hrs of the study. Pulmonary capillary pressure was increased 7 mm Hg above baseline at 0.5 and 0.75 hrs during the infusion of endotoxin but returned to baseline values at 1.5 hrs. Despite sustained pulmonary hypertension, pulmonary capillary pressure remained at baseline values for the duration of the study. Similar to pulmonary capillary pressure, pulmonary venous (or postcapillary) resistance was increased approximately four-fold over baseline at 0.5 and 0.75 hrs after initiating endotoxin administration, but returned to baseline values by the end of endotoxin administration and remained at baseline values throughout the remainder of the study. In contrast, pulmonary arterial (or precapillary) resistance remained at values approximately three times baseline during the

  1. The role of increased pulmonary blood flow in pulmonary arterial hypertension

    NARCIS (Netherlands)

    van Albada, ME; Schoemaker, RG; Kemna, MS; Cromme - Dijkhuis, A; van Veghel, R; Berger, RMF

    Chronic increased pulmonary blood flow is considered a pre-requisite for the induction of advanced vascular lesions in pulmonary arterial hypertension in congenital heart defects. The aim of the present study was to characterise the effects of increased pulmonary flow induced by an aortocaval shunt

  2. Pulmonary lymphoid neogenesis in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Perros, Frédéric; Dorfmüller, Peter; Montani, David; Hammad, Hamida; Waelput, Wim; Girerd, Barbara; Raymond, Nicolas; Mercier, Olaf; Mussot, Sacha; Cohen-Kaminsky, Sylvia; Humbert, Marc; Lambrecht, Bart N

    2012-02-01

    Patients with idiopathic pulmonary arterial hypertension (IPAH) present circulating autoantibodies against vascular wall components. Pathogenic antibodies may be generated in tertiary (ectopic) lymphoid tissues (tLTs). To assess the frequency of tLTs in IPAH lungs, as compared with control subjects and flow-induced PAH in patients with Eisenmenger syndrome, and to identify local mechanisms responsible for their formation, perpetuation, and function. tLT composition and structure were studied by multiple immunostainings. Cytokine/chemokine and growth factor expression was quantified by real-time polymerase chain reaction and localized by immunofluorescence. The systemic mark of pulmonary lymphoid neogenesis was investigated by flow cytometry analyses of circulating lymphocytes. As opposed to lungs from control subjects and patients with Eisenmenger syndrome, IPAH lungs contained perivascular tLTs, comprising B- and T-cell areas with high endothelial venules and dendritic cells. Lymphocyte survival factors, such as IL-7 and platelet-derived growth factor-A, were expressed in tLTs as well as the lymphorganogenic cytokines/chemokines, lymphotoxin-α/-β, CCL19, CCL20, CCL21, and CXCL13, which might explain the depletion of circulating CCR6(+) and CXCR5(+) lymphocytes. tLTs were connected with remodeled vessels via an ER-TR7(+) stromal network and supplied by lymphatic channels. The presence of germinal center centroblasts, follicular dendritic cells, activation-induced cytidine deaminase, and IL-21(+)PD1(+) follicular helper T cells in tLTs together with CD138(+) plasma cell accumulation around remodeled vessels in areas of immunoglobulin deposition argued for local immunoglobulin class switching and ongoing production. We highlight the main features of lymphoid neogenesis specifically in the lungs of patients with IPAH, providing new evidence of immunological mechanisms in this severe condition.

  3. Pulmonary arterial lesions in explanted lungs after transplantation correlate with severity of pulmonary hypertension in chronic obstructive pulmonary disease

    DEFF Research Database (Denmark)

    Carlsen, Jørn; Hasseriis Andersen, Kasper; Boesgaard, Søren

    2013-01-01

    by the presence and severity of pulmonary hypertension (PH) assessed by right-heart catheterization in 3 hemodynamically distinct groups: (1) non-PH (mean pulmonary arterial pressure [mPAP]50 mm Hg; median HE Grade 4 (range 3-6), with generalized arterial dilatation and plexiform lesions. CONCLUSIONS: The extent...

  4. Transcatheter Embolization of Pulmonary Artery False Aneurysm Associated with Primary Pulmonary Hypertension

    International Nuclear Information System (INIS)

    Hiraki, T.; Kanazawa, S.; Mimura, H.; Yasui, K.; Okumura, Y.; Dendo, S.; Yoshimura, K.; Takahara, M.; Hiraki, Y.

    2004-01-01

    A 29-year-old woman with primary pulmonary hypertension presented with recurrent hemoptysis. Contrast-enhanced CT of the chest demonstrated the enhanced mass surrounded by consolidation related to parenchymal hemorrhage. Pulmonary angiography suggested that the mass was a pulmonary artery false aneurysm. After a microcatheter was superselectively inserted into the parent artery of the falseaneurysm, the false aneurysm was successfully treated by transcatheterembolization with coils. Her hemoptysis has never recurred

  5. Role of chymase in cigarette smoke-induced pulmonary artery remodeling and pulmonary hypertension in hamsters

    OpenAIRE

    Yang Ting; Xu Dan; An Jin; He Guang-Ming; Chen Ya-Juan; Chen Lei; Ning Yun-Ye; Zhang Shang-Fu; Han Su-Xia; Wang Tao; Zhang Xiao-Hong; Wen Fu-Qiang

    2010-01-01

    Abstract Background Cigarette smoking is an important risk factor for pulmonary arterial hypertension (PAH) in chronic obstructive pulmonary disease (COPD). Chymase has been shown to function in the enzymatic production of angiotensin II (AngII) and the activation of transforming growth factor (TGF)-β1 in the cardiovascular system. The aim of this study was to determine the potential role of chymase in cigarette smoke-induced pulmonary artery remodeling and PAH. Methods Hamsters were exposed ...

  6. Pulsatile haemodynamic parameters are predictors of survival in paediatric pulmonary arterial hypertension

    NARCIS (Netherlands)

    Douwes, Johannes M.; Roofthooft, Marcus T. R.; Bartelds, Beatrijs; Talsma, Melle D.; Hillege, Hans L.; Berger, Rolf M. F.

    2013-01-01

    Background: There is a need for reliable prognostic parameters in pulmonary arterial hypertension (PAH), especially in children. Pulsatile components of the right ventricular afterload, represented by pulmonary arterial compliance (PACi) and pulmonary stroke volume (PSVi), may provide important

  7. Pulmonary arterial hypertension associated with congenital heart disease

    Directory of Open Access Journals (Sweden)

    Michele D'Alto

    2012-12-01

    Full Text Available Pulmonary arterial hypertension (PAH is a common complication of congenital heart disease (CHD, with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD. The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges. This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.

  8. Bronchial arteries: an arteriosclerosis-resistant circulation.

    Science.gov (United States)

    Kotoulas, Christophoros; Melachrinou, Maria; Konstantinou, George N; Alexopoulos, Dimitrios; Dougenis, Dimitrios

    2010-01-01

    Until now, it is unknown whether and to what extent arteriosclerotic disease affects the bronchial arteries. We conducted this pilot study to estimate the prevalence of arteriosclerosis of the bronchial arteries, to correlate it with certain clinicolaboratory arteriosclerotic parameters or any coexistent coronary artery disease (CAD) and to validate the clinical significance. Bronchial arteries 10-15 mm long were obtained from 40 patients with a mean age of 62.3 years who underwent major thoracic procedures. Their medical history and detailed clinical and laboratory arteriosclerotic risk factors were documented. The mean diameter of bronchial artery specimens was 0.97 mm. Histology revealed medial calcific sclerosis only in 1 patient (2.5%) without simultaneous, established atherosclerotic lesions or narrowing of the lumen. Furthermore, the vessel diameter was significantly correlated not only with the advanced stage of the disease (p = 0.031), but also with the proximal occlusion of the bronchial tree (p = 0.042). We noted a marginally not significant correlation between arteriosclerosis and metabolic syndrome (p = 0.075), independent from a history of CAD (p = 0.84). Bronchial arteries exhibit only medial calcific sclerosis. CAD and chronic obstructive pulmonary disease do not seem to affect them in terms of atherosclerotic alteration findings or vessel diameter changes. The bronchial resistance to arteriosclerosis might support the mediastinal status quo through their anastomoses, contributing to all its structures, and might be indirect evidence of a different physiological function of the bronchial endothelium, which needs to be further investigated. Copyright 2009 S. Karger AG, Basel.

  9. Idiopathic pulmonary artery aneurysm treated with surgical correction and concomitant coronary artery bypass grafting.

    Science.gov (United States)

    Arnaoutakis, George; Nwakanma, Lois; Conte, John

    2009-07-01

    Idiopathic pulmonary artery aneurysm is a rare clinical entity, and therefore the natural course and clinical management are not well established. We present the case of an elderly woman with a symptomatic idiopathic pulmonary artery aneurysm who underwent surgical repair along with simultaneous coronary artery bypass grafting. With long-term follow-up presented in this report, we describe the safety and durability of surgical repair.

  10. Pediatric Perioperative Pulmonary Arterial Hypertension: A Case-Based Primer

    Directory of Open Access Journals (Sweden)

    Shilpa Shah

    2017-10-01

    Full Text Available The perioperative period is an extremely tenuous time for the pediatric patient with pulmonary arterial hypertension. This article will discuss a multidisciplinary approach to preoperative planning, the importance of early identification of pulmonary hypertensive crises, and practical strategies for postoperative management for this unique group of children.

  11. MAO-A promoter polymorphism and idiopathic pulmonary arterial ...

    Indian Academy of Sciences (India)

    Eddahibi S., Humbert M., Fadel E., Raffestin B., Darmon M.,. Capron F. et al. 2001 Serotonin transporter overexpression is re- sponsible for pulmonary artery smooth muscle hyperplasia in pri- mary pulmonary hypertension. J. Clin. Invest. 108, 1141–1150. Edgar A. J., Chacón M. R., Bishop A. E., Yacoub M. H. and Polak.

  12. Persistent pulmonary hypertension of the newborn with transposition of the great arteries

    NARCIS (Netherlands)

    Roofthooft, Marcus T. R.; Bergman, Klasina A.; Ebels, Tjark; Bartelds, Beatrijs; Berger, Rolf M. F.; Waterbolk, T

    Background. Persistent pulmonary hypertension of the newborn (PPHN) in patients with transposition of the great arteries (TGA) is reported to be a high-risk and often therapy-resistant condition, associated with a high mortality. However, data on its incidence and prognosis are scarce and originate

  13. Definition, classification, and epidemiology of pulmonary arterial hypertension.

    Science.gov (United States)

    Hoeper, Marius M

    2009-08-01

    Pulmonary arterial hypertension (PAH) is a distinct subgroup of pulmonary hypertension that comprises idiopathic PAH, familial/heritable forms, and PAH associated with connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus (HIV) infection, and some other conditions. The hemodynamic definition of PAH was recently revised: PAH is now defined by a mean pulmonary artery pressure at rest > or =25 mm Hg in the presence of a pulmonary capillary wedge pressure or =30 mm Hg during exercise) that was used in the old definition of PAH has been removed because there are no robust data that would allow defining an upper limit of normal for the pulmonary pressure during exercise. The revised classification of pulmonary hypertension still consists of five major groups: (1) PAH, (2) pulmonary hypertension due to left heart disease, (3) pulmonary hypertension due to chronic lung disease and/or hypoxia, (4) chronic thromboembolic pulmonary hypertension, and (5) miscellaneous forms. Modifications have been made in some of these groups, such as the addition of schistosomiasis-related pulmonary hypertension and pulmonary hypertension in patients with chronic hemolytic anemia to group 1.

  14. Gene expression profile in flow-associated pulmonary arterial hypertension with neointimal lesions

    NARCIS (Netherlands)

    van Albada, Mirjam E.; Bartelds, Beatrijs; Wijnberg, Hans; Mohaupt, Saffloer; Dickinson, Michael G.; Schoemaker, Regien G.; Kooi, Krista; Gerbens, Frans; Berger, Rolf M. F.

    Pulmonary arterial hypertension (PAH) is a pulmonary angioproliferative disease with high morbidity and mortality, characterized by a typical pattern of pulmonary vascular remodeling including neointimal lesions. In congenital heart disease, increased pulmonary blood flow has appeared to be a key

  15. NMDA-Type Glutamate Receptor Activation Promotes Vascular Remodeling and Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Dumas, Sébastien J; Bru-Mercier, Gilles; Courboulin, Audrey; Quatredeniers, Marceau; Rücker-Martin, Catherine; Antigny, Fabrice; Nakhleh, Morad K; Ranchoux, Benoit; Gouadon, Elodie; Vinhas, Maria-Candida; Vocelle, Matthieu; Raymond, Nicolas; Dorfmüller, Peter; Fadel, Elie; Perros, Frédéric; Humbert, Marc; Cohen-Kaminsky, Sylvia

    2018-02-14

    Background -Excessive proliferation and apoptosis resistance in pulmonary vascular cells underlie vascular remodeling in pulmonary arterial hypertension (PAH). Specific treatments for PAH exist, mostly targeting endothelial dysfunction, but high pulmonary arterial pressure still causes heart failure and death. Pulmonary vascular remodeling may be driven by metabolic reprogramming of vascular cells to increase glutaminolysis and glutamate production. The N-methyl-D-aspartate receptor (NMDAR), a major neuronal glutamate receptor, is also expressed on vascular cells, but its role in PAH is unknown. Methods -We assessed the status of the glutamate-NMDAR axis in the pulmonary arteries of PAH patients and controls, through mass spectrometry imaging, western blotting and immunohistochemistry. We measured the glutamate release from cultured pulmonary vascular cells using enzymatic assays, and analyzed NMDAR regulation/phosphorylation through western blot experiments. The effect of NMDAR blockade on human pulmonary arterial smooth muscle cell (hPASMC) proliferation was determined using a BrdU incorporation assay. We assessed the role of NMDARs in vascular remodeling associated to pulmonary hypertension (PH), both in smooth muscle-specific NMDAR knockout mice exposed to chronic hypoxia and in the monocrotaline rat model of PH using NMDAR blockers. Results -We report glutamate accumulation, upregulation of the NMDAR, and NMDAR engagement reflected by increases in GluN1-subunit phosphorylation, in the pulmonary arteries of human PAH patients. K v channel inhibition and ETAR activation amplified calcium-dependent glutamate release from hPASMCs, and ETAR and PDGFR activation led to NMDAR engagement, highlighting crosstalk between the glutamate-NMDAR axis and major PAH-associated pathways. The PDGF-BB-induced proliferation of hPASMCs involved NMDAR activation and phosphorylated GluN1 subunit localization to cell-cell contacts, consistent with glutamatergic communication between

  16. Erythropoietin Attenuates Pulmonary Vascular Remodeling in Experimental Pulmonary Arterial Hypertension through Interplay between Endothelial Progenitor Cells and Heme Oxygenase

    NARCIS (Netherlands)

    van Loon, Rosa Laura E; Bartelds, Beatrijs; Wagener, Frank A D T G; Affara, Nada; Mohaupt, Saffloer; Wijnberg, Hans; Pennings, Sebastiaan W C; Takens, Janny; Berger, Rolf M F

    2015-01-01

    BACKGROUND: Pulmonary arterial hypertension (PAH) is a pulmonary vascular disease with a high mortality, characterized by typical angio-proliferative lesions. Erythropoietin (EPO) attenuates pulmonary vascular remodeling in PAH. We postulated that EPO acts through mobilization of endothelial

  17. Updated treatment algorithm of pulmonary arterial hypertension.

    Science.gov (United States)

    Galiè, Nazzareno; Corris, Paul A; Frost, Adaani; Girgis, Reda E; Granton, John; Jing, Zhi Cheng; Klepetko, Walter; McGoon, Michael D; McLaughlin, Vallerie V; Preston, Ioana R; Rubin, Lewis J; Sandoval, Julio; Seeger, Werner; Keogh, Anne

    2013-12-24

    The demands on a pulmonary arterial hypertension (PAH) treatment algorithm are multiple and in some ways conflicting. The treatment algorithm usually includes different types of recommendations with varying degrees of scientific evidence. In addition, the algorithm is required to be comprehensive but not too complex, informative yet simple and straightforward. The type of information in the treatment algorithm are heterogeneous including clinical, hemodynamic, medical, interventional, pharmacological and regulatory recommendations. Stakeholders (or users) including physicians from various specialties and with variable expertise in PAH, nurses, patients and patients' associations, healthcare providers, regulatory agencies and industry are often interested in the PAH treatment algorithm for different reasons. These are the considerable challenges faced when proposing appropriate updates to the current evidence-based treatment algorithm.The current treatment algorithm may be divided into 3 main areas: 1) general measures, supportive therapy, referral strategy, acute vasoreactivity testing and chronic treatment with calcium channel blockers; 2) initial therapy with approved PAH drugs; and 3) clinical response to the initial therapy, combination therapy, balloon atrial septostomy, and lung transplantation. All three sections will be revisited highlighting information newly available in the past 5 years and proposing updates where appropriate. The European Society of Cardiology grades of recommendation and levels of evidence will be adopted to rank the proposed treatments. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  18. Identifying "super responders" in pulmonary arterial hypertension.

    Science.gov (United States)

    Halliday, Stephen J; Hemnes, Anna R

    2017-01-01

    Pharmacotherapeutic options for pulmonary arterial hypertension (PAH) have increased dramatically in the last two decades and along with this have been substantial improvements in survival. Despite these advances, however, PAH remains a progressive and ultimately fatal disease for most patients and only epoprostenol has been shown to improve survival in a randomized control trial. Clinical observations of the heterogeneity of treatment response to different classes of medications across the phenotypically diverse PAH population has led to the identification of patients who derive significantly more benefit from certain medications than the population mean, the so-called "super responders." This was first recognized among PAH patients with acute vasodilator response during invasive hemodynamic testing, a subset of whom have dramatically improved survival when treated with calcium channel blocker (CCB) therapy. Retrospective studies have now suggested a sex discrepancy in response to endothelin receptor antagonists (ERA) and phosphodiesterase inhibitors, and more recently a few studies have found genomic associations with response to CCBs and ERAs. With increasing availability of "omics" technologies, recognition of these "super responders," combined with careful clinical and molecular phenotyping, will lead to advances in pharmacogenomics, precision medicine, and continued improvements in survival among PAH patients.

  19. Rare associations of tetralogy of Fallot with anomalous left coronary artery from pulmonary artery and totally anomalous pulmonary venous connection.

    Science.gov (United States)

    Sen, Supratim; Rao, Suresh G; Kulkarni, Snehal

    2016-06-01

    We describe the cases of two patients with tetralogy of Fallot, aged 4 years and 8 months, who were incidentally detected to have concomitant anomalous left coronary artery from pulmonary artery and total anomalous pulmonary venous connection, respectively, on preoperative imaging. They underwent surgical correction with good mid-term outcomes. In this study, we discuss the embryological basis, physiological effects, and review the literature of these two unusual associations. Awareness of these rare associations will avoid missed diagnoses and consequent surgical surprises.

  20. Aortic dissection presenting with secondary pulmonary hypertension caused by compression of the pulmonary artery by dissecting hematoma: a case report

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Ryu, Sang Wan; Choi, Yong Sun; Ahn, Byoung Hee

    2004-01-01

    The rupture of an acute dissection of the ascending aorta into the space surrounding the pulmonary artery is an uncommon occurrence. No previous cases of transient pulmonary hypertension caused by a hematoma surrounding the pulmonary artery have been documented in the literature. Herein, we report a case of acute aortic dissection presenting as secondary pulmonary hypertension

  1. Scintigraphic study of blood perfusion of the pulmonary artery in pulmonary tuberculosis

    International Nuclear Information System (INIS)

    Certain, D.A.; Brolio, R.; Salomon, G.C.; Carvalho, N.; Barbosa, Z.L.M.

    1972-01-01

    Blood perfusion in the pulmonary artery is studied by pulmonary scintigraphy with macroaggregated albumin 131 I, in 74 cases of pulmonary tuberculosis. Results shown by scintigraphy are compared to those observed in roentgenography and also correlated with the extension and degree of the lesions, and with the presence of associated tuberculosis, considered capable of changing the scintigraphic picture. The reduction of blood flow in the pulmonary artery are observed in cases of infiltrative, fibrotic of caseous lesions, as well as in cases of minor lesions [pt

  2. Lineage Analysis in Pulmonary Arterial Hypertension

    Science.gov (United States)

    2012-06-01

    cells. We induced experimental pulmonary hypertension in SM22 Cre x mT/mG mice, by injecting monocrotaline pyrrole into the pulmonary circulation of...were observed in the pulmonary vascular lesions in monocrotaline -injected rats (Sahara 2007).   Endothelial to mesenchymal transition refers to...relative to the induction of experimental pulmonary hypertension : a) Acquisition and breeding of VE Cadherin CreER-T2 tamoxifen- inducible conditional Cre

  3. Preliminary Experience with GORE-TEX® Grafting for Right Ventricle-Pulmonary Artery Conduits

    Science.gov (United States)

    Molina, J. Ernesto

    1986-01-01

    A consecutive series of 12 patients between the ages of 6½ and 37 years underwent implantation of venous ventricle-pulmonary artery conduits. GORE-TEX material without prosthetic valves was used. Four patients had L-transposition of the great vessels with ventricular septal defect (VSD) and pulmonary stenosis; four had tetralogy of Fallot with pulmonary atresia; and one had double outlet right ventricle, pulmonary stenosis, and a complete form of A-V canal. Two patients had D-transposition of the great arteries, VSD, and pulmonary stenosis; and one patient had L-transposition of the great arteries and isolated pulmonary stenosis. All patients had low pulmonary resistance and pressures. The technique for implanting this noncrimped type of prosthesis is described in detail. Follow-up ranged from 2 months to 5½ years. GORE-TEX offers a good choice of material for the construction of this type of conduit to prevent pseudointima formation and obstruction, which is often observed in woven Dacron grafts. PMID:15226844

  4. Recent strategies in treatment of pulmonary arterial hypertension, a review.

    Science.gov (United States)

    Fallah, Flora

    2015-01-26

    Pulmonary arterial hypertension (PAH) is a disease characterized by an elevation in pulmonary artery pressure that can lead to right ventricular failure and death. The pulmonary circulation has to accommodate the entire cardiac output in each cardiac cycle and evolution has adapted to this by making it a low-pressure high-flow system. However, pathology can affect both the arterial and venous components of this system. Pulmonary venous hypertension mainly refers to diseases that result in elevated venous pressure and occurs mainly from mitral valve and left-sided heart disease. Standard treatment options include oral anticoagulation, diuretics, oxygen supplementation, and for a small percentage of patients, calcium channel blockers. Newer treatments include prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors. This article reviews the current treatments strategies for PAH and provides guidelines for its management.

  5. Cytotoxic cells and granulysin in pulmonary arterial hypertension and pulmonary veno-occlusive disease.

    Science.gov (United States)

    Perros, Frédéric; Cohen-Kaminsky, Sylvia; Gambaryan, Natalia; Girerd, Barbara; Raymond, Nicolas; Klingelschmitt, Isabelle; Huertas, Alice; Mercier, Olaf; Fadel, Elie; Simonneau, Gerald; Humbert, Marc; Dorfmüller, Peter; Montani, David

    2013-01-15

    Pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD) both display occlusive remodeling of the pulmonary vasculature responsible for increased pulmonary vascular resistances. Cytotoxic T (CTL), natural killer (NK), and natural killer T (NKT) cells play a critical role in vascular remodeling in different physiological and pathological conditions. Granulysin (GNLY) represents a powerful effector protein for all these subpopulations. To analyze the cytolytic compartment of inflammatory cells in patients with PAH and PVOD. The overall functional status of the cytolytic compartment was studied through epigenetic analysis of the GNLY gene in explanted lungs and in peripheral blood mononuclear cells. Flow cytometry technology allowed analysis of specific circulating cytolytic cells and GNLY contents. A GNLY-specific ELISA allowed measurement of GNLY serum concentrations. A decrease in GNLY demethylation in the gDNA extracted from peripheral blood mononuclear cells and explanted lungs was found specifically in PVOD but not in PAH. This was associated with a decrease in populations and subpopulations of CTL and NKT and an increase of NK populations. Despite the reduced granulysin-containing cells in patients with PVOD, GNLY serum levels were higher, suggesting these cells were wasting their content. Furthermore, the increase of GNLY concentration in the serum of PVOD was significantly higher than in patients with PAH. PVOD is characterized by alterations of circulating cytotoxic cell subpopulations and by epigenetic dysregulation within the GNLY gene. Our findings may be helpful in the quest to develop needed diagnostic tools, including flow cytometry analyses, to screen for suspected PVOD in patients with pulmonary hypertension.

  6. Role of lipoxygenase metabolites of arachidonic acid in enhanced pulmonary artery contractions of female rabbits.

    Science.gov (United States)

    Pfister, Sandra L

    2011-04-01

    Pulmonary arterial hypertension is characterized by elevated pulmonary artery pressure and vascular resistance. In women the incidence is 4-fold greater than that in men. Studies suggest that sustained vasoconstriction is a factor in increased vascular resistance. Possible vasoconstrictor mediators include arachidonic acid-derived lipoxygenase (LO) metabolites. Our studies in rabbits showed enhanced endothelium-dependent contractions to arachidonic acid in pulmonary arteries from females compared with males. Because treatment with a nonspecific LO inhibitor reduced contractions in females but not males, the present study identified which LO isoform contributes to sex-specific pulmonary artery vasoconstriction. The 15- and 5- but not 12-LO protein expressions were greater in females. Basal and A23187-stimulated release of 15-, 5-, and 12-hydroxyeicosatetraenoic acids (HETEs) from females and males were measured by liquid chromatography/mass spectrometry. Only 15-HETE synthesis was greater in females compared with males under both basal and stimulated conditions. Vascular contractions to 15-HETE were enhanced in females compared with males (maximal contraction: 44±6%versus 25±3%). The specific 15-LO inhibitor PD146176 (12 μmol/L) decreased arachidonic acid-induced contractions in females (maximal contraction: 93±4% versus 57±10%). If male pulmonary arteries were incubated with estrogen (1 μmol/L, 18 hours), protein expression of 15-LO and 15-HETE production increased. Mechanisms to explain the increased incidence of pulmonary hypertension in women are not known. Results suggest that the 15-LO pathway is different between females and males and is regulated by estrogen. Understanding this novel sex-specific mechanism may provide insight into the increased incidence of pulmonary hypertension in females.

  7. Rehabilitation in patients with pulmonary arterial hypertension.

    Science.gov (United States)

    Keusch, Stephan; Turk, Alexander; Saxer, Stéphanie; Ehlken, Nicola; Grunig, Ekkehard; Ulrich, Silvia; On Behalf Of The Swiss Society Of Pulmonary Hypertension

    2017-07-11

    Exertional dyspnoea is a leading symptom in patients with pulmonary arterial hypertension (PAH). Patients suffering from PAH report poor quality of life, have skeletal muscle dysfunction and in the absence of advanced medical therapy deteriorate progressively due to right heart failure which can lead to death. For decades, patients with PAH were advised to avoid exercise in fear of exacerbated right heart failure. Recently, it has been shown that a highly supervised rehabilitation programme in expert centres leads to significant improvements in symptoms, quality of life, exercise capacity and may even enhance haemodynamics in selected stable patients treated with advanced regimens of PAH-targeted drugs. As a consequence of these promising results, pulmonary rehabilitation performed in an expert centre has been included in recent guidelines. The underlying mechanisms are not completely understood, but positive effects can be measured in different organ systems such as skeletal muscles, the cardiopulmonary system and immune system (inflammation), and also on the psychological level. Thus, improvements in 6-minute walking distance (6MWD), peak oxygen uptake (VO2 peak), muscle strength and muscle endurance, as well as physical and mental quality of life scores (SF-36 questionnaire) have been shown. Different training protocols have been used. Essential are qualified patient selection in expert centres, a low workload endurance and dumbbell (weight lifting) training avoiding strenuous exercise and exhaustion, thorough patient education and close supervision by experts especially during the first weeks. Adverse events may occur (e.g., pre-/syncope, arrhythmia, respiratory infections). PAH patients tend to overestimate their physical capacity, not perceiving their own limits properly, which makes education and expert advice even more important as exercise training can also worsen the right heart failure. Therefore, a core issue of the multidisciplinary rehabilitation is

  8. The effect of ACE inhibition on the pulmonary vasculature in combined model of chronic hypoxia and pulmonary arterial banding in Sprague Dawley rats

    Science.gov (United States)

    Clarke, Shanelle; Baumgardt, Shelley; Molthen, Robert

    2010-03-01

    Microfocal CT was used to image the pulmonary arterial (PA) tree in rodent models of pulmonary hypertension (PH). CT images were used to measure the arterial tree diameter along the main arterial trunk at several hydrostatic intravascular pressures and calculate distensibility. High-resolution planar angiographic imaging was also used to examine distal PA microstructure. Data on pulmonary artery tree morphology improves our understanding of vascular remodeling and response to treatments. Angiotensin II (ATII) has been identified as a mediator of vasoconstriction and proliferative mitotic function. ATII has been shown to promote vascular smooth muscle cell hypertrophy and hyperplasia as well as stimulate synthesis of extracellular matrix proteins. Available ATII is targeted through angiotensin converting enzyme inhibitors (ACEIs), a method that has been used in animal models of PH to attenuate vascular remodeling and decrease pulmonary vascular resistance. In this study, we used rat models of chronic hypoxia to induce PH combined with partial left pulmonary artery occlusion (arterial banding, PLPAO) to evaluate effects of the ACEI, captopril, on pulmonary vascular hemodynamic and morphology. Male Sprague Dawley rats were placed in hypoxia (FiO2 0.1), with one group having underwent PLPAO three days prior to the chronic hypoxia. After the twenty-first day of hypoxia exposure, treatment was started with captopril (20 mg/kg/day) for an additional twenty-one days. At the endpoint, lungs were excised and isolated to examine: pulmonary vascular resistance, ACE activity, pulmonary vessel morphology and biomechanics. Hematocrit and RV/LV+septum ratio was also measured. CT planar images showed less vessel dropout in rats treated with captopril versus the non-treatment lungs. Distensibility data shows no change in rats treated with captopril in both chronic hypoxia (CH) and CH with PLPAO (CH+PLPAO) models. Hemodynamic measurements also show no change in the pulmonary vascular

  9. The hemodynamic response to dopamine and nitroprusside following right atrium-pulmonary artery bypass (Fontan procedure).

    Science.gov (United States)

    Williams, D B; Kiernan, P D; Schaff, H V; Marsh, H M; Danielson, G K

    1982-07-01

    Cardiac output is critically dependent upon pulmonary vascular resistance after right atrium-pulmonary artery bypass (Fontan procedure), since there is no pulmonary ventricle in the circulation. Inotropic agents, including dopamine, may increase pulmonary vascular resistance and, therefore, might have an adverse effect on cardiac output. The present study determined the hemodynamic responses to dopamine and nitroprusside of 9 patients following right atrium-pulmonary artery bypass. Particular attention was given to effects on cardiac output (CI), pulmonary vascular resistance, and right atrial pressure (RAP). Baseline hemodynamic data were measured without drugs, with dopamine at 7.5 micrograms/kg/min, with sodium nitroprusside up to 5.0 micrograms/kg/min, and with a combination of dopamine, 7.5 micrograms/kg/min, and sodium nitroprusside, 1.0 micrograms/kg/min. Right and left atrial pressures (LAP), mean arterial blood pressure (BP), heart rate (HR), and CI were measured. Stroke volume index and pulmonary arteriolar resistance index were calculated. The increase in CI from baseline (1.98 +/- 0.86 liters per minute) was significant for infusions of dopamine (2.75 +/- 1.05, p less than 0.001), sodium nitroprusside (2.57 +/- 0.78, p less than 0.001), and both drugs (2.74 +/- 0.84, p less than 0.001). The increased CI was achieved primarily by a significant increase in HR with dopamine and by an increase in stroke volume index with sodium nitroprusside. With a similar increment in CI, the RAP was significantly decreased from baseline (21 +/- 4 torr) with sodium nitroprusside (15 +/- 3, p less than 0.001) but was unchanged with dopamine. Pulmonary arteriolar resistance index decreased significantly from baseline (375 +/- 230 dynes sec cm-5/m2) with sodium nitroprusside (169 +/- 132, p less than 0.001), and, interestingly, with dopamine as well (273 +/- 165, p less than 0.05). Both dopamine and sodium nitroprusside in these dosages have favorable effects on CI and

  10. Unilateral pulmonary artery agenesis: An unusual cause of unilateral ARDS

    Directory of Open Access Journals (Sweden)

    Lakshmi Saladi, MD

    Full Text Available Unilateral pulmonary artery agenesis (UPAA is a rare malformation that can present as an isolated anomaly or may be associated with certain congenital cardiac anomalies, such as tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, truncus arteriosus and pulmonary atresia. Clinical presentation is non-specific which makes the diagnosis elusive; chronic dyspnea, hemoptysis or recurrent infections are the most common manifestations. Patients may remain asymptomatic until adulthood. There is no definitive treatment for patients with UPAA. Acute respiratory distress syndrome (ARDS is usually a bilateral disease, unilateral ARDS has been described after lung resection or trauma. We present a case of a 39 year-old woman who developed unilateral ARDS and was later diagnosed with isolated UPAA. Keywords: Unilateral pulmonary artery agenesis, Acute respiratory distress syndrome, Pulmonary hypertension, Pulmonary edema, Hemoptysis

  11. Coenzyme Q supplementation in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Jacqueline Sharp

    2014-01-01

    Full Text Available Mitochondrial dysfunction is a fundamental abnormality in the vascular endothelium and smooth muscle of patients with pulmonary arterial hypertension (PAH. Because coenzyme Q (CoQ is essential for mitochondrial function and efficient oxygen utilization as the electron carrier in the inner mitochondrial membrane, we hypothesized that CoQ would improve mitochondrial function and benefit PAH patients. To test this, oxidized and reduced levels of CoQ, cardiac function by echocardiogram, mitochondrial functions of heme synthesis and cellular metabolism were evaluated in PAH patients (N=8 in comparison to healthy controls (N=7, at baseline and after 12 weeks oral CoQ supplementation. CoQ levels were similar among PAH and control individuals, and increased in all subjects with CoQ supplementation. PAH patients had higher CoQ levels than controls with supplementation, and a tendency to a higher reduced-to-oxidized CoQ ratio. Cardiac parameters improved with CoQ supplementation, although 6-minute walk distances and BNP levels did not significantly change. Consistent with improved mitochondrial synthetic function, hemoglobin increased and red cell distribution width (RDW decreased in PAH patients with CoQ, while hemoglobin declined slightly and RDW did not change in healthy controls. In contrast, metabolic and redox parameters, including lactate, pyruvate and reduced or oxidized gluthathione, did not change in PAH patients with CoQ. In summary, CoQ improved hemoglobin and red cell maturation in PAH, but longer studies and/or higher doses with a randomized placebo-controlled controlled design are necessary to evaluate the clinical benefit of this simple nutritional supplement.

  12. Relationship between pulmonary artery volumes at computed tomography and pulmonary artery pressures in patients with- and without pulmonary hypertension

    International Nuclear Information System (INIS)

    Froelich, Jens J.; Koenig, Helmut; Knaak, Lennard; Krass, Stefan; Klose, Klaus J.

    2008-01-01

    Objectives: This study was designed to determine the relationship between pulmonary artery (PA) volumes at computed tomography (CT) and PA pressures at right-sided heart catheterization in patients with and without pulmonary hypertension (PAH) to develop a noninvasive CT method of PA pressure quantification. Materials and methods: Sixteen patients with chronic sleep apnea syndrome underwent contrast enhanced helical CT (slice thickness 3 mm; pitch 2; increment 2 mm) at inspiration. Eight patients had PAH while cardiopulmonary disease has been excluded in eight other patients. Vascular volumes were determined using a 3D technique (threshold seeded vascular tracing algorithm; thresholds -600 H [lower] and 3000 H [upper]). Right-sided heart catheterization measurements were available for linear regression analysis of PA volumes and pressures. Results: Correlation between PA pressures and volumes (normalized for BMI), was high in both groups (without PAH: r = .85; with PAH .90, Pearson). Compared to elevated PA pressures in patients with pulmonary hypertension (p < .005), PA volumes also were significantly increased (p < .05) among the groups. Conclusions: High correlation was found between PA volumes and mean PA pressures in patients with- and without PAH. Significant differences in PA volumes at CT-volumetry may admit non-invasive determination of pulmonary hypertension

  13. Relationship between pulmonary artery volumes at computed tomography and pulmonary artery pressures in patients with- and without pulmonary hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Froelich, Jens J. [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: jens.froelich@klinikum-hef.de; Koenig, Helmut [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: helmut.koenig@siemens.com; Knaak, Lennard [Department of Medicine, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: froehlic@staff.uni-marburg.de; Krass, Stefan [MeVis Research, Universitaetsallee 29, 28359 Bremen (Germany)], E-mail: krass@mevis.de; Klose, Klaus J. [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: klose@med.uni-marburg.de

    2008-09-15

    Objectives: This study was designed to determine the relationship between pulmonary artery (PA) volumes at computed tomography (CT) and PA pressures at right-sided heart catheterization in patients with and without pulmonary hypertension (PAH) to develop a noninvasive CT method of PA pressure quantification. Materials and methods: Sixteen patients with chronic sleep apnea syndrome underwent contrast enhanced helical CT (slice thickness 3 mm; pitch 2; increment 2 mm) at inspiration. Eight patients had PAH while cardiopulmonary disease has been excluded in eight other patients. Vascular volumes were determined using a 3D technique (threshold seeded vascular tracing algorithm; thresholds -600 H [lower] and 3000 H [upper]). Right-sided heart catheterization measurements were available for linear regression analysis of PA volumes and pressures. Results: Correlation between PA pressures and volumes (normalized for BMI), was high in both groups (without PAH: r = .85; with PAH .90, Pearson). Compared to elevated PA pressures in patients with pulmonary hypertension (p < .005), PA volumes also were significantly increased (p < .05) among the groups. Conclusions: High correlation was found between PA volumes and mean PA pressures in patients with- and without PAH. Significant differences in PA volumes at CT-volumetry may admit non-invasive determination of pulmonary hypertension.

  14. Cardiac and pulmonary artery mensuration in feline heartworm disease

    International Nuclear Information System (INIS)

    Schafer, M.; Berry, C.R.

    1995-01-01

    A retrospective study was undertaken to quantify thoracic radiographic changes in cats with heartworm diseases, (Dirofilaria immitis). Using a blinded study format, the cardiac silhouette, thoracic cavity and pulmonary arteries were measured from thoracic radiographs of 21 cats with feline heartworm disease and 30 cats without known cardiac or pulmonary vessel pathology. Measured data were normalized to the thoracic cavity or bony structures within the radiographic field of view. The measurements were compared between the two groups of cats using an unpaired, two-tailed Student's t-test, with a p value of < 0.05 being considered significant. Cats with feline heartworm disease had enlargement of the craniocaudal aspect of the cardiac silhouette and normalized cardiac:thoracic ratio (p < 0.05) on the lateral view. Also, there was significant enlargement of the central and peripheral caudal lobar pulmonary arteries and their normalized ratios (p < 0.05) in the heartworm infected cats as visualized on the ventrodorsal projection. Tortuosity of the pulmonary arteries was seen in three of the 21 infected cats. Eleven of the 21 cats with feline heartworm disease had pulmonary parenchymal changes. Based on the present study, central and peripheral pulmonary artery enlargement as viewed on the ventrodorsal radiograph was the single best radiographic indicator of feline heartworm disease

  15. Brain natriuretic peptide in pulmonary arterial hypertension: biomarker and potential therapeutic agent

    Directory of Open Access Journals (Sweden)

    Brian Casserly

    2009-11-01

    Full Text Available Brian Casserly, James R KlingerDivision of Pulmonary and Critical Care Medicine, The Memorial Hospital of Rhode Island, Pawtucket, RI, Rhode Island Hospital, Providence, RI, Alpert Medical School of Brown University, Providence, RI, USAAbstract: B-type natriuretic peptide (BNP is a member of the natriuretic peptide family, a group of widely distributed, but evolutionarily conserved, polypeptide mediators that exert myriad cardiovascular effects. BNP is a potent vasodilator with mitogenic, hypertrophic and pro-inflammatory properties that is upregulated in pulmonary hypertensive diseases. Circulating levels of BNP correlate with mean pulmonary arterial pressure (mPAP and pulmonary vascular resistance (PVR in patients with pulmonary arterial hypertension (PAH. Elevated plasma BNP levels are associated with increased mortality in patients with PAH and a fall in BNP levels after therapy is associated with improved survival. These findings have important clinical implications in that a noninvasive blood test may be used to identify PAH patients at high-risk of decompensation and to guide pulmonary vasodilator therapy. BNP also has several biologic effects that could be beneficial to patients with PAH. However, lack of a convenient method for achieving sustained increases in circulating BNP levels has impeded the development of BNP as a therapy for treating pulmonary hypertension. New technologies that allow transdermal or oral administration of the natriuretic peptides have the potential to greatly accelerate research into therapeutic use of BNP for cor pulmonale and pulmonary vascular diseases. This review will examine the basic science and clinical research that has led to our understanding of the role of BNP in cardiovascular physiology, its use as a biomarker of right ventricular function and its therapeutic potential for managing patients with pulmonary vascular disease.Keywords: brain natriuretic peptide, pulmonary artery hypertension

  16. Practical considerations for the pharmacotherapy of pulmonary arterial hypertension.

    Science.gov (United States)

    Bishop, Bryan M; Mauro, Vincent F; Khouri, Samer J

    2012-09-01

    Pulmonary arterial hypertension is a devastating disease. Before the 1990s, when pharmacologic treatment was finally approved, only supportive therapy was available, consisting of anticoagulation, digoxin, diuretics, and supplemental oxygen. Calcium channel blocker therapy was also an option, but only a small percentage of patients respond to it. However, starting with epoprostenol in 1996, the number of drugs approved to treat pulmonary arterial hypertension increased. Three distinct classes of drugs were developed based on the pathophysiology of the disease: the prostanoids, endothelin-1 receptor antagonists, and phosphodiesterase type 5 inhibitors. The prostanoids are administered either parenterally or by inhalation to replace the lack of prostacyclin within the pulmonary arterial vasculature. The endothelin-1 receptor antagonists were the first class of oral drugs to be developed, but drug interactions and adverse effects are prominent with this class. The phosphodiesterase type 5 inhibitors increase the second messenger cyclic guanosine monophosphate (GMP) that is induced by nitric oxide stimulation. All of the drugs within these three classes are distinct in and of themselves, and their clinical use requires in-depth knowledge of pulmonary arterial hypertension and its pathophysiology. Because these drugs have different mechanisms of action, combination therapy has shown promise in patients with severe disease, although data are still lacking. This article should serve as a practical guide for clinicians who encounter patients with pulmonary arterial hypertension and the drugs used for the treatment of this devastating disease. © 2012 Pharmacotherapy Publications, Inc. All rights reserved.

  17. Pathways and Drugs in Pulmonary Arterial Hypertension - Focus on the Role of Endothelin Receptor Antagonists.

    Science.gov (United States)

    Madonna, Rosalinda; Cocco, Nino; De Caterina, Raffaele

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a group of diseases characterized by a progressive increase of pulmonary vascular resistance (PVR), initially due to abnormal pulmonary vasoconstriction in response to endothelial injury. Recent studies have here confirmed the prominent role of endothelin (ET)-1 in vasoconstriction and remodelling of the pulmonary microcirculation. In patients who are acute-vasoreactive, classical treatments for PAH are calcium channels blockers (CCBs), while drugs targeting the prostacyclin, nitric oxide and endothelin pathways, i.e., prostanoids, phosphodiesterase (PDE)-5 inhibitors and endothelin receptor antagonists (ERAs), respectively, are indicated in non-vasoreactive patients or in vasoreactive patients not responding to initial CCB therapy. Randomised, placebo-controlled trials have shown that ERAs improve pulmonary haemodynamics, exercise capacity, functional status and clinical outcome in patients with PAH. Here we provide an overview of the currently recommended diagnostic and therapeutic work-up in PAH, with special emphasis on ERAs.

  18. Isolated agenesis of the right pulmonary artery with late manifestation of pulmonary hypertension

    International Nuclear Information System (INIS)

    Huebsch, P.; Pichler, W.; Lang, I.; Mlczoch, J.

    1987-01-01

    The case of a woman patient of 25 years of age with acute cardiac decompensation is presented. The chest X-ray as well as the lung scan showed the typical features of absence of the right pulmonary artery. The diagnosis was confirmed angiographically. The sudden and late onset of symptoms of pulmonary hypertension is a remarkable feature. (orig.) [de

  19. Pulmonary artery remodeling differs in hypoxia- and monocrotaline-induced pulmonary hypertension

    NARCIS (Netherlands)

    van Suylen, R. J.; Smits, J. F.; Daemen, M. J.

    1998-01-01

    In the present study we analyzed structural characteristics of muscular pulmonary arteries and arterioles in two classic models of pulmonary hypertension, the rat hypoxia and monocrotaline models. We hypothesized that an increase in medial cross-sectional area would result in reduction of the lumen

  20. Changes in pulmonary artery pressure affect survival differently in lung transplant recipients who have pulmonary hypertension or chronic obstructive pulmonary disease.

    Science.gov (United States)

    O'Keefe, Kathryn L; Kilic, Ahmet; Pope-Harman, Amy; Hayes, Don; Kirkby, Stephen; Higgins, Robert S D; Whitson, Bryan A

    2014-08-01

    Pulmonary hypertension and right ventricular dysfunction can complicate lung transplant. Pulmonary artery pressures affect outcome are uncertain during wait list. We evaluated changes in wait list pulmonary artery pressures on survival after lung transplant. We queried the United Network for Organ Sharing/Standard Transplant Analysis and Research registry from 1987 to 2012 for all lung transplants. Recipients with unique pulmonary artery pressure measurements upon listing and transplant were included. Mean pulmonary artery pressure was rated as increased (increase > 5 mm Hg), decreased (decrease > 5 mm Hg), or unchanged (variation pulmonary artery pressure during the listing period (P ≤ .0001). In recipients with chronic obstructive pulmonary disease, survival was poorer when mean pulmonary artery pressure was increased than decreased (P ≤ .03). In recipients with primary pulmonary hypertension, survival was poorer when mean pulmonary artery pressure was decreased than increased (P ≤ .02). Proportional hazards analysis showed that increases in mean pulmonary artery pressure independently affected survival (hazard ratio, 0.78; 95% confidence interval, 0.62-0.96). Although the mechanism is unknown, an increase in mean pulmonary artery pressure in patients with chronic obstructive pulmonary disease is associated with poorer survival after lung transplant. In contrast, patients with primary pulmonary hypertension with decreased mean pulmonary artery pressure have poorer survival after lung transplant. In patients with primary pulmonary hypertension, changes in pulmonary artery pressure may be a surrogate for a failing right ventricular function. In chronic obstructive pulmonary disease, the change in pressure suggests an undetermined progressive process. Further study of right ventricular function is warranted to determine the effects of changes in pulmonary artery pressure on lung transplant recipients.

  1. [Arterial rigidity in patients with chronic obstructive pulmonary disease].

    Science.gov (United States)

    Karoli, N A; Dolishniaia, G R; Rebrov, A P

    2012-01-01

    The aim of this open study was to estimate arterial rigidity in patients with chronic obstructive pulmonary disease (COPD). It included 105 patients above 40 years of age. Exclusion criteria were clinical signs of CHD, peripheral atherosclerosis, and other severe chronic diseases in the exacerbation phase. The control group was comprised of 27 practically healthy volunteers. The arterial fluid was detected using a Tensioclinic arteriograph (Tensiomed, Hungary). Arterial rigidity was estimated in patients of two age groups (below and above 60 years) with COPD of different severity The results suggest the development of arterial wall lesions in proportion to the patients' age and COPD severity. It was shown that excessive arterial rigidity and accelerated pulse wave reflection (increased speed of pulse wave propagation and augmentation index) exert significant influence on the elevation of central arterial pressure. Enhanced rigidity of the arterial wall being a cardiovascular risk factor further prospective studies are needed.

  2. Pulmonary arterial hypertension in children: diagnosis using ratio of main pulmonary artery to ascending aorta diameter as determined by multi-detector computed tomography

    International Nuclear Information System (INIS)

    Caro-Dominguez, Pablo; Manson, David E.; Compton, Gregory; Humpl, Tilman

    2016-01-01

    The ratio of the transverse diameter of the main pulmonary artery (MPA) to ascending aorta as determined at multi-detector CT is a tool that can be used to assess the pulmonary arterial size in cases of pulmonary arterial hypertension in children. To establish a ratio of MPA to ascending aorta diameter using multi-detector CT imaging suggestive of pulmonary arterial hypertension in children. We hypothesize that a defined ratio of MPA to ascending aorta is identifiable on multi-detector CT and that higher ratios can be used to reliably diagnose the presence of pulmonary arterial hypertension in children. We calculated the multi-detector CT ratio of MPA to ascending aorta diameter in 44 children with documented pulmonary arterial hypertension by right heart catheterization and in 44 age- and gender-matched control children with no predisposing factors for pulmonary arterial hypertension. We compared this multi-detector-CT-determined ratio with the MPA pressure in the study group, as well as with the ratio of MPA to ascending aorta in the control group. A threshold ratio value was calculated to accurately identify children with pulmonary arterial hypertension. Children with documented primary pulmonary arterial hypertension have a significantly higher ratio of MPA to ascending aorta (1.46) than children without pulmonary arterial hypertension (1.11). A ratio of 1.3 carries a positive likelihood of 34 and a positive predictive value of 97% for the diagnosis of pulmonary arterial hypertension. The pulmonary arteries were larger in children with pulmonary arterial hypertension than in a control group of normal children. A CT-measured ratio of MPA to ascending aorta of 1.3 should raise the suspicion of pulmonary arterial hypertension in children. (orig.)

  3. Pulmonary arterial hypertension in children: diagnosis using ratio of main pulmonary artery to ascending aorta diameter as determined by multi-detector computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Caro-Dominguez, Pablo; Manson, David E. [University of Toronto, Department of Diagnostic Imaging, The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada); Compton, Gregory [University of Toronto, Department of Diagnostic Imaging, The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada); Epworth Hospital, Epworth Medical Imaging, Richmond, VIC (Australia); Humpl, Tilman [University of Toronto, Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, ON (Canada)

    2016-09-15

    The ratio of the transverse diameter of the main pulmonary artery (MPA) to ascending aorta as determined at multi-detector CT is a tool that can be used to assess the pulmonary arterial size in cases of pulmonary arterial hypertension in children. To establish a ratio of MPA to ascending aorta diameter using multi-detector CT imaging suggestive of pulmonary arterial hypertension in children. We hypothesize that a defined ratio of MPA to ascending aorta is identifiable on multi-detector CT and that higher ratios can be used to reliably diagnose the presence of pulmonary arterial hypertension in children. We calculated the multi-detector CT ratio of MPA to ascending aorta diameter in 44 children with documented pulmonary arterial hypertension by right heart catheterization and in 44 age- and gender-matched control children with no predisposing factors for pulmonary arterial hypertension. We compared this multi-detector-CT-determined ratio with the MPA pressure in the study group, as well as with the ratio of MPA to ascending aorta in the control group. A threshold ratio value was calculated to accurately identify children with pulmonary arterial hypertension. Children with documented primary pulmonary arterial hypertension have a significantly higher ratio of MPA to ascending aorta (1.46) than children without pulmonary arterial hypertension (1.11). A ratio of 1.3 carries a positive likelihood of 34 and a positive predictive value of 97% for the diagnosis of pulmonary arterial hypertension. The pulmonary arteries were larger in children with pulmonary arterial hypertension than in a control group of normal children. A CT-measured ratio of MPA to ascending aorta of 1.3 should raise the suspicion of pulmonary arterial hypertension in children. (orig.)

  4. Pulmonary arterial hypertension in congenital cardiac disease - the need for refinement of the Evian-Venice classification

    NARCIS (Netherlands)

    van Albada, Mirjam E.; Berger, Rolf M. F.

    Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified, in the Evian-Venice classification, as Pulmonary Arterial Hypertension, which includes a heterogeneous group of conditions. Emerging options for treatment of patients with pulmonary arterial

  5. Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions

    NARCIS (Netherlands)

    Blok, Ilja M.; van Riel, Annelieke C. M. J.; Mulder, Barbara J. M.; Bouma, Berto J.

    2015-01-01

    Pulmonary arterial hypertension is a serious complication of adult congenital heart disease associated with systemic-to-pulmonary shunts. Although early shunt closure restricts development of pulmonary arterial hypertension, patients remain at risk even after repair. The development of pulmonary

  6. Is cardiac resynchronization therapy for right ventricular failure in pulmonary arterial hypertension of benefit?

    Science.gov (United States)

    Rasmussen, Jason T; Thenappan, Thenappan; Benditt, David G; Weir, E Kenneth; Pritzker, Marc R

    2014-12-01

    Pulmonary arterial hypertension is a manifestation of a group of disorders leading to pulmonary vascular remodeling and increased pulmonary pressures. The right ventricular (RV) response to chronic pressure overload consists of myocardial remodeling, which is in many ways similar to that seen in left ventricular (LV) failure. Maladaptive myocardial remodeling often leads to intraventricular and interventricular dyssychrony, an observation that has led to cardiac resynchronization therapy (CRT) for LV failure. CRT has proven to be an effective treatment strategy in subsets of patients with LV failure resulting in improvement in LV function, heart failure symptoms, and survival. Current therapy for pulmonary arterial hypertension is based on decreasing pulmonary vascular resistance, and there is currently no effective therapy targeting the right ventricle or maladaptive ventricular remodeling in these patients. This review focuses on the RV response to chronic pressure overload, its effect on electromechanical coupling and synchrony, and how lessons learned from left ventricular cardiac resynchronization might be applied as therapy for RV dysfunction in the context of pulmonary arterial hypertension.

  7. Sirtuin 1 regulates pulmonary artery smooth muscle cell proliferation: role in pulmonary arterial hypertension.

    Science.gov (United States)

    Zurlo, Giada; Piquereau, Jérôme; Moulin, Maryline; Pires Da Silva, Julie; Gressette, Mélanie; Ranchoux, Benoît; Garnier, Anne; Ventura-Clapier, Renée; Fadel, Elie; Humbert, Marc; Lemaire, Christophe; Perros, Frédéric; Veksler, Vladimir

    2018-05-01

    Energy metabolism shift from oxidative phosphorylation toward glycolysis in pulmonary artery smooth muscle cells (PASMCs) is suggested to be involved in their hyperproliferation in pulmonary arterial hypertension (PAH). Here, we studied the role of the deacetylase sirtuin1 (SIRT1) in energy metabolism regulation in PASMCs via various pathways including activation of peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α), master regulator of mitochondrial biogenesis. Contents of PGC-1α and its downstream targets as well as markers of mitochondrial mass (voltage-dependent anion channel and citrate synthase) were diminished in human PAH PASMCs. These cells and platelet-derived growth factor-stimulated rat PASMCs demonstrated a shift in cellular acetylated/deacetylated state, as evidenced by the increase of the acetylated forms of SIRT1 targets: histone H1 and Forkhead box protein O1. Rat and human PASMC proliferation was potentiated by SIRT1 pharmacological inhibition or specific downregulation via short-interfering RNA. Moreover, after chronic hypoxia exposure, SIRT1 inducible knock out mice displayed a more intense vascular remodeling compared with their control littermates, which was associated with an increase in right ventricle pressure and hypertrophy. SIRT1 activator Stac-3 decreased the acetylation of histone H1 and Forkhead box protein O1 and strongly inhibited rat and human PASMC proliferation without affecting cell mortality. This effect was associated with the activation of mitochondrial biogenesis evidenced by higher expression of mitochondrial markers and downstream targets of PGC-1α. Altered acetylation/deacetylation balance as the result of SIRT1 inactivation is involved in the pathogenesis of PAH, and this enzyme could be a promising therapeutic target for PAH treatment.

  8. Efficacy of pulmonary artery banding in patients with uneventricular defects

    Directory of Open Access Journals (Sweden)

    Ю. С. Синельников

    2015-10-01

    Full Text Available Long-term results where evaluated for different methods of pulmonary artery banding (PAB in 25 patients with univentricular congenital heart defects with used three methods. Hospital mortality was 8%. Second stage palliation was performed in 61% of patients. More tight PA banding in patients with univentriclar gave opportunity to perform 2nd stage of operation in 100% of patients, decrease complications and mortality rate, modify pulmonary circulation effectively.

  9. Measurement of regional pulmonary blood volume in patients with increased pulmonary blood flow or pulmonary arterial hypertension

    International Nuclear Information System (INIS)

    Wollmer, P.; Rozcovek, A.; Rhodes, C.G.; Allan, R.M.; Maseri, A.

    1984-01-01

    The effects of chronic increase in pulmonary blood flow and chronic pulmonary hypertension on regional pulmonary blood volume was measured in two groups of patients. One group of patients had intracardiac, left-to-right shunts without appreciable pulmonary hypertension, and the other consisted of patients with Eisenmenger's syndrome or primary pulmonary hypertension, i.e. patients with normal or reduced blood flow and severe pulmonary hypertension. A technique based on positron tomography was used to measure lung density (by transmission scanning) and regional pulmonary blood volume (after inhalation of /sup 11/CO). The distribution of pulmonary blood volume was more uniform in patients with chronic increase in pulmonary blood flow than in normal subjects. There were also indications of an absolute increase in intrapulmonary blood volume by about 15%. In patients with chronic pulmonary arterial hypertension, the distribution of pulmonary blood volume was also abnormally uniform. There was, however, no indication that overall intrapulmonary blood volume was substantially different from normal subjects. The abnormally uniform distribution of pulmonary blood volume can be explained by recruitment and/or dilatation of vascular beds. Intrapulmonary blood volume appears to be increased in patients with intracardiac, left-to-right shunts. With the development of pulmonary hypertension, intrapulmonary blood volume falls, which may be explained by reactive changes in the vasculature and/or obliteration of capillaries

  10. Continuous inhaled iloprost in a neonate with d-transposition of the great arteries and severe pulmonary arterial hypertension.

    Science.gov (United States)

    Dykes, John C; Torres, Marilyn; Alexander, Plato J

    2016-03-01

    This report describes the case of a neonate with d-transposition of the great arteries and severe pulmonary arterial hypertension stabilised in the post-operative period with continuous iloprost nebulisation. To our knowledge, this is the first documented method of treating post-operative severe pulmonary arterial hypertension with continuous inhaled iloprost in a patient with complex CHD. We found this method of delivering the drug very effective in stabilising haemodynamic swings in the setting of severe pulmonary arterial hypertension.

  11. [A case of pulmonary artery injury which might have been caused by a pulmonary artery catheter during the mitral valve replacement].

    Science.gov (United States)

    Uematsu, Yuki; Murata, Tetsuya; Taruishi, Chieko; Sudani, Tomoko; Takada, Motoshi; Suzuki, Akira

    2013-02-01

    We experienced a pulmonary artery injury which might have been caused by a pulmonary artery catheter (PAC). A 66-year-old woman after mitral valve replacement, developped massive tracheal bleeding during weaning from the cardiopulmonary bypass. Transesophageal echocardiography revealed the air bell in the right pulmonary artery synchronized with ventilation. We speculated pulmonary artery injury and performed pulmonary artery angiography; however, it could not clarify the bleeding point. Surgical approach found the air leak from the erasure of the bronchus intermedius and the lobectomy led to lifesaving. A pulmonary artery injury caused by PAC is very rare, but life-threatening. In this case, she had some risk factors (i. e. 60 years or older, female, short statue, and mitral valve disease); however, it was hard to predict the pulmonary injury from these factors.

  12. Macitentan for the treatment of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Kholdani CA

    2014-11-01

    Full Text Available Cyrus A Kholdani,1 Wassim H Fares,2 Terence K Trow2 1Section of Pulmonary, Critical Care and Sleep Medicine, 2Yale Pulmonary Vascular Disease Program, Section of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA Abstract: Macitentan is the most recently approved dual endothelin-receptor antagonist (ERA for the treatment of symptomatic pulmonary arterial hypertension. Compared to other available ERAs, it demonstrates superior receptor-binding properties, with consequently improved tissue penetration, and a longer duration of action allowing for once-daily dosing. It has a favorable adverse-effect profile, with notably no demonstrable increase in the risk of hepatotoxicity or peripheral edema, but like other ERAs, it is potentially limited by significant anemia. Phase I data have demonstrated a favorable drug–drug interaction profile and no need for dose adjustment with hepatic and renal impairment. In the pivotal SERAPHIN study, treatment of symptomatic pulmonary arterial hypertension patients with macitentan led to statistically significant improvements in functional class, exercise tolerance, and hemodynamic parameters, in addition to a reduction in morbidity in an event-driven long-term trial. Keywords: endothelin, endothelin receptor antagonists, macitentan, pulmonary arterial hypertension

  13. Congenital arteriovenous fistula between the internal mammary artery and the pulmonary artery

    NARCIS (Netherlands)

    P.W.J.C. Serruys (Patrick); H. van Meurs-van Woezik

    1984-01-01

    textabstractThis is the fourth reported case of congenital arteriovenous fistula between the internal mammary artery and pulmonary artery. Precise and complete diagnostic evaluation is required to localize, delineate and appreciate the haemodynamic significance of this type of arteriovenous shunt. A

  14. Contrast Media Delivery in the Assessment of Anomalous Left Coronary Artery From the Pulmonary Artery.

    Science.gov (United States)

    Saade, Charbel; Al-Hamra, Salam; Al-Mohiy, Hussain; El-Merhi, Fadi

    2016-05-01

    A patient with a history of mitral valve prolapse and regurgitation that was corrected with a mitral ring repair 15 years earlier received a diagnosis of anomalous left coronary artery arising from the pulmonary artery and underwent repair. Coronary computed tomography angiography (CTA) was employed to image the patient before surgical intervention. Synchronizing contrast media administration to opacify the right coronary artery in the arterial phase and the left coronary artery in the venous phase required a test-bolus approach. Matching compromised cardiovascular dynamics with patient-specific contrast media administration protocols was improved considerably with the use of a test-bolus technique during electrocardiography-gated coronary CTA.

  15. Pulmonary Arterial Stent Implantation in an Adult with Williams Syndrome

    International Nuclear Information System (INIS)

    Reesink, Herre J.; Henneman, Onno D. F.; Delden, Otto M. van; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

    2007-01-01

    We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent implantation. Short-term follow-up showed a good clinical result with excellent patency of the stents but early restenosis of the segments in which only balloon angioplasty was performed. These stenoses were subsequently also treated successfully by stent implantation. Stent patency was observed 3 years after the first procedure

  16. [Definition and classification of pulmonary arterial hypertension].

    Science.gov (United States)

    Nakanishi, Norifumi

    2008-11-01

    Pulmonary hypertension(PH) is a disorder that may occur either in the setting of a variety of underlying medical conditions or as a disease that uniquely affects the pulmonary vasculature. Because an accurate diagnosis of PH in a patient is essential to establish an effective treatment, a classification of PH has been helpful. The first classification, established at WHO Symposium in 1973, classified PH into groups based on the known cause and defined primary pulmonary hypertension (PPH) as a separate entity of unknown cause. In 1998, the second World Symposium on PPH was held in Evian. Evian classification introduced the concept of conditions that directly affected the pulmonary vasculature (i.e., PAH), which included PPH. In 2003, the third World Symposium on PAH convened in Venice. In Venice classification, the term 'PPH' was abandoned in favor of 'idiopathic' within the group of disease known as 'PAH'.

  17. Late Posthemorrhagic Structural and Functional Changes in Pulmonary Circulation Arteries

    Directory of Open Access Journals (Sweden)

    S. A. Andreyeva

    2008-01-01

    Full Text Available Objective: to reveal the major regularities and mechanisms of morphological changes in the rat pulmonary circulation arteries in the late posthemorrhagic period and to compare them with age-related features of the vessels. Materials and methods: experiments to generate graduated hemorrhagic hypotension with the blood pressure being maintained at 40 mm Hg were carried out on young (5—6-month albino male Wistar rats. Throughout hypotension and 60 days after blood loss, the blood was tested to determine low and average molecular-weight substances by spectrophotometry and the pro- and antioxidative systems by chemiluminescence. Pulmonary circulation arteries were morphologically studied in young animals, rats in the late posthemorrhagic period and old (24—25-month rats. Results. Sixty-minute hemorrhagic hypotension leads to the development of endotoxemia and imbalance of the pro- and antioxidative systems, the signs of which are observed in the late periods (2 months after hypotension. At the same time, the posthemorrhagic period is marked by the significant pulmonary circulation arterial morphological changes comparable with their age-related alterations in old rat. This shows up mainly in the reorganization of a connective tissue component in the vascular wall: the elevated levels of individual collagen fibers, their structural changes, elastic medial membrane destruction and deformity. At the same time, there is a change in the morphometric parameters of vessels at all study stages while their lowered flow capacity is only characteristic for intraorgan arteries. Conclusion: The increased activity of free radical oxidation and endotoxemia may be believed to be one of the causes of morphological changes in pulmonary circulation arteries in the late posthemorrhagic period, which is similar to age-related vascular alterations. Key words: hemorrhagic hypotension, pulmonary circulation arteries, free radical oxidation, endotoxemia, remodeling, late

  18. Computerized axial tomography of the chest for visualization of ''absent'' pulmonary arteries

    International Nuclear Information System (INIS)

    Sondheimer, H.M.; Oliphant, M.; Schneider, B.; Kavey, R.E.W.; Blackman, M.S.; Parker, F.B. Jr.

    1982-01-01

    To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with ''absent'' left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease

  19. Update on the clinical utility of sildenafil in the treatment of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Gautam V Ramani

    2010-05-01

    Full Text Available Gautam V Ramani, Myung H ParkUniversity of Maryland, Baltimore, MD, USAAbstract: Sildenafil is an orally administered phosphodiesterase type 5 inhibitor that is approved for the treatment of pulmonary arterial hypertension (PAH. The hemodynamic effects of sildenafil are mitigated primarily via potentiating the effects of endogenous nitric oxide, leading to smooth muscle cell relaxation and reductions in pulmonary arterial pressures and pulmonary vascular resistance. When added to standard background therapy in patients with idiopathic or associated PAH from congenital heart disease, anorexigen use, or connective tissue disease, sildenafil treatment results in improved exercise capacity as measured by 6 minute walk distance, improved hemodynamics, and favorable changes in quality of life. Sildenafil use is contraindicated with concomitant nitrate administration, and caution should be exercised when used in combination with antihypertensive agents due to risks of precipitating hypotension. Side effects are generally mild, and include flushing, headaches, and epistaxis. The combination of sildenafil with intravenous epoprostenol is safe and well tolerated, and further improves exercise capacity. Sildenafil is approved only for treatment of PAH, and although emerging data suggest a potential role in treating other types of pulmonary hypertension, larger trials are required to confirm these findings. Keywords: sildenafil, pulmonary arterial hypertension, phosphodiesterase type 5 inhibitor

  20. Anomalous left coronary artery from the pulmonary artery

    Science.gov (United States)

    ... anomalies of mitral valve. In: Sellke FW, del Nido PJ, Swanson SJ, eds. Sabiston and Spencer Surgery ... of the coronary arteries. In: Sellke FW, del Nido PJ, Swanson SJ, eds. Sabiston and Spencer Surgery ...

  1. Changing demographics of pulmonary arterial hypertension in congenital heart disease

    NARCIS (Netherlands)

    Mulder, B. J. M.

    2010-01-01

    Pulmonary arterial hypertension (PAH) is a serious complication of congenital heart disease (CHD). Without early surgical repair, around one-third of paediatric CHD patients develop significant PAH. Recent data from the Netherlands suggest that >4% of adult CHD patients have PAH, with higher rates

  2. SECONDARY PULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC DISEASES OF CONNECTIVE TISSUE

    Directory of Open Access Journals (Sweden)

    N. A. Shostak

    2016-01-01

    Full Text Available Modern definition of pulmonary arterial hypertension (PAH as well as data on prevalence and incidence of secondary PAH in systemic disease of connective tissue is presented,  including data of USA, France and Scotland registers. The main chains of pathogenesis, classification approaches, clinical features and diagnostics are described. 

  3. SECONDARY PULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC DISEASES OF CONNECTIVE TISSUE

    Directory of Open Access Journals (Sweden)

    N. A. Shostak

    2009-01-01

    Full Text Available Modern definition of pulmonary arterial hypertension (PAH as well as data on prevalence and incidence of secondary PAH in systemic disease of connective tissue is presented,  including data of USA, France and Scotland registers. The main chains of pathogenesis, classification approaches, clinical features and diagnostics are described. 

  4. Selexipag for the Treatment of Pulmonary Arterial Hypertension

    DEFF Research Database (Denmark)

    Sitbon, Olivier; Channick, Richard; Chin, Kelly M

    2015-01-01

    BACKGROUND: In a phase 2 trial, selexipag, an oral selective IP prostacyclin-receptor agonist, was shown to be beneficial in the treatment of pulmonary arterial hypertension. METHODS: In this event-driven, phase 3, randomized, double-blind, placebo-controlled trial, we randomly assigned 1156 pati...

  5. Clopidogrel in infants with systemic-to-pulmonary-artery shunts

    DEFF Research Database (Denmark)

    Wessel, David L; Berger, Felix; Li, Jennifer S

    2013-01-01

    BACKGROUND: Infants with cyanotic congenital heart disease palliated with placement of a systemic-to-pulmonary-artery shunt are at risk for shunt thrombosis and death. We investigated whether the addition of clopidogrel to conventional therapy reduces mortality from any cause and morbidity relate......-related morbidity. (Funded by Sanofi-Aventis and Bristol-Myers Squibb; ClinicalTrials.gov number, NCT00396877.)....

  6. Aneurysmal dilatation of the pulmonary artery trunk and its major ...

    African Journals Online (AJOL)

    She was admitted for cough and dyspnea of acute onset without fever or chest pain. The patient was in respiratory distress, afebrile and ... The causes are dominated by infectious diseases, inflammatory arteritis, and congenital heart disease and acquired valvular heart disease. Pulmonary arterial hypertension can cause ...

  7. Calcium-channel blockers in pulmonary arterial hypertension.

    Science.gov (United States)

    Chaumais, Marie-Camille; Macari, Elise Artaud; Sitbon, Olivier

    2013-01-01

    Voltage-activated calcium channels are a family of membrane proteins that provide the major influx pathway for calcium in many different types of cells. Calcium-channel blockers inhibit the calcium influx into vascular cells leading to relaxation of smooth muscle cells and vasodilatation. Vasoconstriction of small pulmonary arteries is recognized as a component of the pathogenesis of pulmonary arterial hypertension and treatment with calcium-channel blockers appears to be rational in this setting. No randomized controlled trial has been performed to demonstrate the beneficial effects of calcium-channel blockers in the treatment of patients with pulmonary arterial hypertension. However, uncontrolled studies have suggested that long-term administration of high-dose calcium antagonists dramatically improves survival in a small subset of patients who respond acutely to those drugs, compared with unresponsive patients. The initial response to an acute vasodilator test with inhaled nitric oxide or intravenous prostacyclin or adenosine accurately identifies patients with pulmonary arterial hypertension who are likely to respond to long-term treatment with calcium-channel blockers.

  8. Pulmonary arterial hypertension as a manifestation of lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Stark, P.; Sargent, E.N.; Boylen, T.; Jaramillo, D.

    1987-08-01

    We present five patients with systemic lupus erythematosus (SLE) who developed pulmonary arterial hypertension and cor pulmonale in the course of their disease. The clinical features, as well as, the radiological manifestations of this rare manifestation of SLE are discussed. A vasculitic process is the most likely cause of this complication. Therapy is ineffective and the prognosis is poor.

  9. Role of iloprost and bosentan in pulmonary arterial hypertension ...

    African Journals Online (AJOL)

    Results: Several studies were able to outline the haemodynamic advantages of iloprost and bosentan in pulmonary arterial hypertension, as evident by improvement in six-minute walk test of patients treated with these agents. Conclusion: This review was able to outline the pharmacotherapeutic benefits and role played by ...

  10. Cardiac causes of pulmonary arterial hypertension: assessment with multidetector CT

    Energy Technology Data Exchange (ETDEWEB)

    Hoey, Edward T.D.; Gopalan, Deepa; Agrawal, S.K.B. [Papworth Hospital, Cambridge (United Kingdom); Screaton, Nicholas J. [Papworth Hospital, Cambridge (United Kingdom); Papworth Hospital NHS Trust, Diagnostic Centre, Department of Radiology, Papworth Everard, Cambridgeshire (United Kingdom)

    2009-11-15

    The causes of pulmonary arterial hypertension (PAH) are diverse and include multiple congenital and acquired cardiac diseases as well as diseases primarily affecting the pulmonary vasculature, lung, pleura and chest wall. The traditional role of CT in evaluating PAH includes assessment of pulmonary vasculature and lung parenchyma with limited assessment of the heart. Advances in multidetector CT technology with improved spatial and temporal resolution now permit accurate delineation of cardiac morphology. CT pulmonary angiography (CTPA) is widely utilised in the workup of patients with suspected pulmonary vascular disease and can identify both pulmonary and cardiac causes. As the initial presentation for CTPA is often precipitated by nonspecific, unexplained symptoms and therefore undertaken by a general radiologist, it is important that a systematic approach to the interpretation of these studies, including cardiac evaluation, is routinely adopted. This paper reviews the CT evaluation in pulmonary hypertension with a particular focus on the cardiac causes, their subclassification into congenital systemic to pulmonary shunts and secondary to left heart disease, and their imaging features. It emphasises the use of a systematic approach to interpretation of CTPA examinations both in patients with known PAH and those with previously unsuspected disease. (orig.)

  11. [Arterial stiffness in patients with chronic obstructive pulmonary disease].

    Science.gov (United States)

    2012-01-01

    To estimate arterial stiffness (AS) in patients with chronic obstructive pulmonary disease (COPD). A total of 112 COPD patients over 40 years of age entered a population-based trial. The patients with coronary heart diseases, peripheral vascular atherosclerosis, other severe chronic diseases in exacerbation were withdrawn. The control group consisted of 26 healthy volunteers matched by gender and age. AS was measured at arteriograph "Tensioclinic" ("Tensiomed", Hungary). COPD patients, first of all elderly ones, had abnormal properties of arterial wall. Increased arterial rigidity and pulse wave reflection (accelerated pulse wave velocity and high index of augmentation) are strongly associated with elevation of central arterial pressure. High arterial wall stiffness in COPD patients suggests an increased risk of cardiovascular diseases that necessitates examination in prospective studies.

  12. Central extracorporeal membrane oxygenation requiring pulmonary arterial venting after near-drowning.

    Science.gov (United States)

    Kimura, Mitsutoshi; Kinoshita, Osamu; Fujimoto, Yoshifumi; Murakami, Arata; Shindo, Takahiro; Kashiwa, Koichi; Ono, Minoru

    2014-02-01

    Extracorporeal membrane oxygenation (ECMO) is an effective respiratory and circulatory support in patients in refractory cardiogenic shock or cardiac arrest. Peripheral ECMO sometimes requires left heart drainage; however, few reports state that pulmonary arterial (PA) venting is required during ECMO support. We present a case of a 14-year-old boy who required PA venting during ECMO support after resuscitation from near-drowning in freshwater. A biventricular assist device with an oxygenator implantation was intended on day 1; however, we were unable to proceed because of increasing of pulmonary vascular resistance from the acute lung injury. Central ECMO with PA venting was then performed. On day 13, central ECMO was converted to biventricular assist device with an oxygenator, which was removed on day 16. This case suggests that PA venting during ECMO support may be necessary in some cases of respiratory and circulatory failure with high pulmonary vascular resistance after near-drowning.

  13. Anomalous origin of the left coronary artery from the pulmonary artery in children: diagnostic use of multidetector computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Shen, Quanli; Yao, Qiong; Hu, Xihong [Children' s Hospital of Fudan University, Department of Radiology, Shanghai (China)

    2016-09-15

    Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly. It is important to demonstrate the anomalous origin of the left coronary artery and its course before surgery. To explore the clinical diagnostic use of multidetector CT coronary angiography in detecting anomalous origin of the left coronary artery from the pulmonary artery in children. Nine children (2 boys, 7 girls) ages 2 months to 9 years with surgically confirmed anomalous origin of the left coronary artery from the pulmonary artery were studied. Clinical data, transthoracic echocardiography and CT coronary angiography images were retrospectively analyzed. Transthoracic echocardiography correctly diagnosed anomalous origin of the left coronary artery from the pulmonary artery in 7 of 9 patients (95% CI: 40-97%). CT coronary angiography revealed the anomalous origin of the left coronary artery in all children (95% CI: 66-100%). In a 4-year-old girl and a 9-year-old girl, CT coronary angiography showed dilation of the right coronary artery and collateral circulation between the right and the left coronary arteries. CT coronary angiography is a useful method to show the anomalous origin of the coronary artery in children with anomalous origin of the left coronary artery from the pulmonary artery, especially for patients in whom origin of the left coronary artery cannot be detected by transthoracic echocardiography. (orig.)

  14. Segmentation and quantification of pulmonary artery for noninvasive CT assessment of sickle cell secondary pulmonary hypertension.

    Science.gov (United States)

    Linguraru, Marius George; Pura, John A; Van Uitert, Robert L; Mukherjee, Nisha; Summers, Ronald M; Minniti, Caterina; Gladwin, Mark T; Kato, Gregory; Machado, Roberto F; Wood, Bradford J

    2010-04-01

    Pulmonary arterial hypertension (PAH) is a progressive vascular disease that results in high mortality and morbidity in sickle cell disease (SCD) patients. PAH diagnosis is invasive via right heart catheterization, but manual measurements of the main pulmonary artery (PA) diameters from computed tomography (CT) have shown promise as noninvasive surrogate marker of PAH. The authors propose a semiautomated computer-assisted diagnostic (CAD) tool to quantify the main PA size from pulmonary CT angiography (CTA). A follow-up retrospective study investigated the potential of CT and image analysis to quantify the presence of PAH secondary to SCD based on PA size. The authors segmented the main pulmonary arteries using a combination of fast marching level sets and geodesic active contours from smoothed pulmonary CTA images of 20 SCD patients with proven PAH by right heart catheterization and 20 matched negative controls. From the PA segmentation, a Euclidean distance map was calculated and an algorithm based on fast marching methods was used to compute subvoxel precise centerlines of the PA trunk (PT) and main left/right PA (PM). Maximum distentions of PT and PM were automatically quantified using the centerline and validated with manual measurements from two observers. The pulmonary trunk and main were significantly larger (p image processing and extraction of PA biomarkers show great potential as a surrogate indicator for diagnosis or quantification of PAH, and could be an important tool for drug discovery and noninvasive clinical surveillance.

  15. Congenital anomalies of the pulmonary arteries: spectrum of findings on computed tomography.

    Science.gov (United States)

    Bueno, J; Flors, L; Mejía, M

    Congenital anomalies of the pulmonary arteries are uncommon. They can occur in isolation or in association with congenital heart defects. Isolated congenital anomalies remain undiscovered until they are reported as incidental findings on imaging tests, usually not until adolescence. We review the embryological development and normal anatomy of the pulmonary arteries as well as the spectrum of computed tomography findings for various congenital anomalies: unilateral interruption of the pulmonary artery, anomalous origin of the left pulmonary artery (pulmonary artery sling), idiopathic aneurysm of the pulmonary artery, and other anomalies associated with congenital heart defects. Congenital anomalies of the pulmonary arteries represent a diagnostic challenge for clinicians and radiologists. Computed tomography is useful for their diagnosis, and general radiologists need to be familiar with their imaging appearance because they are often discovered incidentally. Copyright © 2016 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Anomalous left the pulmonary dilemma coronary artery artery from a ...

    African Journals Online (AJOL)

    Two patients had a nonspecific history of respiratory distress, failure to thrive or difficulry with feeding. Course and management (Fig. 3). Three patients underwent a reimplantation of the anomalous left coronary artery into the ascending aorta. One died at opera- tion with extending myocardial infarction related to technical.

  17. Existing drugs and agents under investigation for pulmonary arterial hypertension.

    Science.gov (United States)

    Sharma, Mala; Pinnamaneni, Sowmya; Aronow, Wilbert S; Jozwik, Bartosz; Frishman, William H

    2014-01-01

    Pulmonary arterial hypertension is a progressive and debilitating disorder with an associated high morbidity and mortality rate. Significant advances in our understanding of the epidemiology, pathogenesis, and pathophysiology of pulmonary hypertension have occurred over the past several decades. This has allowed the development of new therapeutic options in this disease. Today, our selection of therapeutic modalities is broader, including calcium channel blockers, prostanoids, endothelin receptor antagonists, phosphodiesterase inhibitors, and soluble guanylate cyclase stimulators, but the disease remains fatal. This underscores the need for a continued search for novel therapies. Several potential pharmacologic agents for the treatment of pulmonary arterial hypertension are under clinical development and some promising results with these treatments have been reported. These agents include rho-kinase inhibitors, long-acting nonprostanoid prostacyclin receptor agonists, tyrosine protein kinase inhibitors, endothelial nitric oxide synthase couplers, synthetically produced vasoactive intestinal peptide, antagonists of the 5-HT2 receptors, and others. This article will review several of these promising new therapies and will discuss the current evidence regarding their potential benefit in pulmonary arterial hypertension.

  18. Review of bosentan in the management of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Eli Gabbay

    2008-01-01

    Full Text Available Eli Gabbay1, John Fraser2, Keith McNeil31Western Australian Lung Transplant Unit and Pulmonary Hypertension Service, Royal Perth Hospital, Western Australia, Australia; 2Critical Care Research Group, The Prince Charles Hospital, Rode Road, Chermside, Queensland, Australia; 3Transplant and Pulmonary Vascular Disease Unit, The Prince Charles Hospital, Rode Road, Chermside, Queensland, AustraliaAbstract: The dual endothelin receptor antagonist, bosentan, is an orally active therapy, which is effective in the treatment of pulmonary arterial hypertension (PAH. This review critically appraises the evidence for the efficacy of bosentan in idiopathic and familial PAH, in PAH associated with connective tissue disease and in PAH which may develop in association with other conditions. Data from the pivotal placebo controlled studies and their open labeled extensions as well as long term survival and quality of life data is presented. Data is also presented on the potential benefit of bosentan in patients with inoperable chronic thromboembolic pulmonary hypertension. The safety and tolerability of bosentan as well as drug interactions are discussed. Dosage recommendations in adults and pediatrics are presented. An algorithm is provided to guide the reader in monitoring potential increases in alanine and aspartate transaminase levels that may occur with bosentan use and the dose adjustments that are recommended as a result of any increase in the levels of these enzymes are shown. Finally, the role of bosentan as part of combination therapy in PAH is examined.Keywords: bosentan, pulmonary arterial hypertension, review

  19. Giant high-pressure pulmonary artery aneurysm in an elderly patient with chronic obstructive pulmonary disease.

    Science.gov (United States)

    Morais, Sandra A; Oliveira, Hugo M; de Almeida, José R; Eiras, Eduardo; Silva, Ana Catarina; Gavina, Cristina

    2016-03-01

    The authors report the case of a 74-year-old man, with a history of chronic obstructive pulmonary disease (COPD), GOLD grade 3, stable for the past two decades, who was admitted to our center with severe right heart failure. The chest radiograph showed moderate heart enlargement mainly of the right atrium and pulmonary artery, similar to previous chest radiographs in the previous 20 years. The transthoracic echocardiogram showed a pulmonary artery aneurysm (PAA), dilatation of the right chambers with pulmonary artery systolic pressure of 52 mmHg, and preserved right ventricular systolic function. A thoracic computed tomography scan confirmed the presence of a giant PAA 72 mm in diameter. The patient was started on high-dose diuretics, with significant clinical improvement. After optimization of medical therapy right heart catheterization was carried out with the patient in optimal clinical condition, which revealed mild precapillary pulmonary hypertension with a mean pulmonary artery pressure of 26 mmHg. On the basis of the clinical and imaging findings a stable, giant, high-pressure, PAA was diagnosed secondary to pulmonary hypertension induced by COPD, with a 20-year follow-up without need for surgical repair, which helped in our decision to maintain medical surveillance. The recent onset of heart failure is explained by the unfavorable evolution of COPD. This case may change the attitude expressed in previous studies favoring the choice of an invasive approach to treat giant high-pressure PAAs, instead supporting the maintenance of medical treatment. Copyright © 2016 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  20. Characterization of proximal pulmonary arterial cells from chronic thromboembolic pulmonary hypertension patients

    Directory of Open Access Journals (Sweden)

    Quarck Rozenn

    2012-03-01

    Full Text Available Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH is associated with proximal pulmonary artery obstruction and vascular remodeling. We hypothesized that pulmonary arterial smooth muscle (PASMC and endothelial cells (PAEC may actively contribute to remodeling of the proximal pulmonary vascular wall in CTEPH. Our present objective was to characterize PASMC and PAEC from large arteries of CTEPH patients and investigate their potential involvement in vascular remodeling. Methods Primary cultures of proximal PAEC and PASMC from patients with CTEPH, with non-thromboembolic pulmonary hypertension (PH and lung donors have been established. PAEC and PASMC have been characterized by immunofluorescence using specific markers. Expression of smooth muscle specific markers within the pulmonary vascular wall has been studied by immunofluorescence and Western blotting. Mitogenic activity and migratory capacity of PASMC and PAEC have been investigated in vitro. Results PAEC express CD31 on their surface, von Willebrand factor in Weibel-Palade bodies and take up acetylated LDL. PASMC express various differentiation markers including α-smooth muscle actin (α-SMA, desmin and smooth muscle myosin heavy chain (SMMHC. In vascular tissue from CTEPH and non-thromboembolic PH patients, expression of α-SMA and desmin is down-regulated compared to lung donors; desmin expression is also down-regulated in vascular tissue from CTEPH compared to non-thromboembolic PH patients. A low proportion of α-SMA positive cells express desmin and SMMHC in the neointima of proximal pulmonary arteries from CTEPH patients. Serum-induced mitogenic activity of PAEC and PASMC, as well as migratory capacity of PASMC, were increased in CTEPH only. Conclusions Modified proliferative and/or migratory responses of PASMC and PAEC in vitro, associated to a proliferative phenotype of PASMC suggest that PASMC and PAEC could contribute to proximal vascular remodeling in CTEPH.

  1. Adenosine Receptors As Drug Targets for Treatment of Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Allan K. N. Alencar

    2017-12-01

    Full Text Available Pulmonary arterial hypertension (PAH is a clinical condition characterized by pulmonary arterial remodeling and vasoconstriction, which promote chronic vessel obstruction and elevation of pulmonary vascular resistance. Long-term right ventricular (RV overload leads to RV dysfunction and failure, which are the main determinants of life expectancy in PAH subjects. Therapeutic options for PAH remain limited, despite the introduction of prostacyclin analogs, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and soluble guanylyl cyclase stimulators within the last 15 years. Through addressing the pulmonary endothelial and smooth muscle cell dysfunctions associated with PAH, these interventions delay disease progression but do not offer a cure. Emerging approaches to improve treatment efficacy have focused on beneficial actions to both the pulmonary vasculature and myocardium, and several new targets have been investigated and validated in experimental PAH models. Herein, we review the effects of adenosine and adenosine receptors (A1, A2A, A2B, and A3 on the cardiovascular system, focusing on the A2A receptor as a pharmacological target. This receptor induces pulmonary vascular and heart protection in experimental models, specifically models of PAH. Targeting the A2A receptor could potentially serve as a novel and efficient approach for treating PAH and concomitant RV failure. A2A receptor activation induces pulmonary endothelial nitric oxide synthesis, smooth muscle cell hyperpolarization, and vasodilation, with important antiproliferative activities through the inhibition of collagen deposition and vessel wall remodeling in the pulmonary arterioles. The pleiotropic potential of A2A receptor activation is highlighted by its additional expression in the heart tissue, where it participates in the regulation of intracellular calcium handling and maintenance of heart chamber structure and function. In this way, the activation of A2A

  2. Adenosine Receptors As Drug Targets for Treatment of Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Alencar, Allan K N; Montes, Guilherme C; Barreiro, Eliezer J; Sudo, Roberto T; Zapata-Sudo, Gisele

    2017-01-01

    Pulmonary arterial hypertension (PAH) is a clinical condition characterized by pulmonary arterial remodeling and vasoconstriction, which promote chronic vessel obstruction and elevation of pulmonary vascular resistance. Long-term right ventricular (RV) overload leads to RV dysfunction and failure, which are the main determinants of life expectancy in PAH subjects. Therapeutic options for PAH remain limited, despite the introduction of prostacyclin analogs, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and soluble guanylyl cyclase stimulators within the last 15 years. Through addressing the pulmonary endothelial and smooth muscle cell dysfunctions associated with PAH, these interventions delay disease progression but do not offer a cure. Emerging approaches to improve treatment efficacy have focused on beneficial actions to both the pulmonary vasculature and myocardium, and several new targets have been investigated and validated in experimental PAH models. Herein, we review the effects of adenosine and adenosine receptors (A 1 , A 2A , A 2B , and A 3 ) on the cardiovascular system, focusing on the A 2A receptor as a pharmacological target. This receptor induces pulmonary vascular and heart protection in experimental models, specifically models of PAH. Targeting the A 2A receptor could potentially serve as a novel and efficient approach for treating PAH and concomitant RV failure. A 2A receptor activation induces pulmonary endothelial nitric oxide synthesis, smooth muscle cell hyperpolarization, and vasodilation, with important antiproliferative activities through the inhibition of collagen deposition and vessel wall remodeling in the pulmonary arterioles. The pleiotropic potential of A 2A receptor activation is highlighted by its additional expression in the heart tissue, where it participates in the regulation of intracellular calcium handling and maintenance of heart chamber structure and function. In this way, the activation of A 2A

  3. Quantitative analysis of pulmonary artery and pulmonary collaterals in preoperative patients with pulmonary artery atresia using dual-source computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Yin Lei [Department of Radiology, Provincial Hospital of Fu Jian province (China); Lu, Bin, E-mail: blu@vip.sina.com [Department of Radiology, Cardiovascular Institute and Fu Wai Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, 167 Bei-li-shi Street, Beijing (China); Han Lei [Department of Radiology, Cardiovascular Institute and Fu Wai Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, 167 Bei-li-shi Street, Beijing (China); Wu Runze; Johnson, Laura [Siemens Healthcare (China); Xu Zhongying; Jiang Shiliang; Dai Ruping [Department of Radiology, Cardiovascular Institute and Fu Wai Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, 167 Bei-li-shi Street, Beijing (China)

    2011-09-15

    Objective: To evaluate the value of dual-source computed tomography (DSCT) in quantitatively measuring pulmonary arteries and major aortopulmonary collateral vessels in comparison with conventional angiographic (CA) on preoperative patients with pulmonary artery atresia and ventricular septal defect (PAA-VSD). Materials and methods: Twenty PAA-VSD patients who had complete imaging data of DSCT, CA and echocardiography (ECHO) studies were retrospectively analyzed. Using final clinical diagnosis as the standard, results of DSCT, CA and ECHO on the detection of cardiac malformations, measurement of diameters of pulmonary artery and collateral vessel, as well as the values of McGoon ratio, pulmonary arterial index (PAI) and total neopulmonary arterial index (TNPAI) were derived and compared. Results: In 20 patients, 51 of 54 (94.4%) cardiac malformations were visualized by DSCT, whereas 42 (77.8%) by ECHO (p = 0.027). Fourteen cases with aortopulmonary collateral vessels were all (100%) detected by DSCT, whereas 5 cases (35.7%) by ECHO (p = 0.001), and 13 cases (92.9%) by CA (p = 0.995). Sixteen cases with confluence of native pulmonary arteries were diagnosed by DSCT, whereas 10 cases by CA (p = 0.024). Measurement of the diameters of pulmonary arteries, collateral vessels, and descending aorta at the level of diaphragm were correlated well between DSCT and CA (r = 0.95-0.99). McGoon ratio (DSCT = 1.18 {+-} 0.60, CA = 1.23 {+-} 0.64), PAI (DSCT = 130.96 {+-} 99.38 mm{sup 2}/m{sup 2}, CA = 140.91 {+-} 107.87 mm{sup 2}/m{sup 2}) and TNPAI (DSCT = 160.31 {+-} 125.62 mm{sup 2}/m{sup 2}, CA = 169.14 {+-} 122.81 mm{sup 2}/m{sup 2}) were calculated respectively, without significant differences between DSCT and CA by paired t-tests (all p > 0.05). Conclusion: DSCT was efficient for evaluating and measuring native pulmonary artery and aortopulmonary collateral vessels prior to surgical procedures in PAA-VSD patients. Combined with echocardiography, DSCT showed potential to

  4. Quantitative analysis of pulmonary artery and pulmonary collaterals in preoperative patients with pulmonary artery atresia using dual-source computed tomography

    International Nuclear Information System (INIS)

    Yin Lei; Lu, Bin; Han Lei; Wu Runze; Johnson, Laura; Xu Zhongying; Jiang Shiliang; Dai Ruping

    2011-01-01

    Objective: To evaluate the value of dual-source computed tomography (DSCT) in quantitatively measuring pulmonary arteries and major aortopulmonary collateral vessels in comparison with conventional angiographic (CA) on preoperative patients with pulmonary artery atresia and ventricular septal defect (PAA-VSD). Materials and methods: Twenty PAA-VSD patients who had complete imaging data of DSCT, CA and echocardiography (ECHO) studies were retrospectively analyzed. Using final clinical diagnosis as the standard, results of DSCT, CA and ECHO on the detection of cardiac malformations, measurement of diameters of pulmonary artery and collateral vessel, as well as the values of McGoon ratio, pulmonary arterial index (PAI) and total neopulmonary arterial index (TNPAI) were derived and compared. Results: In 20 patients, 51 of 54 (94.4%) cardiac malformations were visualized by DSCT, whereas 42 (77.8%) by ECHO (p = 0.027). Fourteen cases with aortopulmonary collateral vessels were all (100%) detected by DSCT, whereas 5 cases (35.7%) by ECHO (p = 0.001), and 13 cases (92.9%) by CA (p = 0.995). Sixteen cases with confluence of native pulmonary arteries were diagnosed by DSCT, whereas 10 cases by CA (p = 0.024). Measurement of the diameters of pulmonary arteries, collateral vessels, and descending aorta at the level of diaphragm were correlated well between DSCT and CA (r = 0.95-0.99). McGoon ratio (DSCT = 1.18 ± 0.60, CA = 1.23 ± 0.64), PAI (DSCT = 130.96 ± 99.38 mm 2 /m 2 , CA = 140.91 ± 107.87 mm 2 /m 2 ) and TNPAI (DSCT = 160.31 ± 125.62 mm 2 /m 2 , CA = 169.14 ± 122.81 mm 2 /m 2 ) were calculated respectively, without significant differences between DSCT and CA by paired t-tests (all p > 0.05). Conclusion: DSCT was efficient for evaluating and measuring native pulmonary artery and aortopulmonary collateral vessels prior to surgical procedures in PAA-VSD patients. Combined with echocardiography, DSCT showed potential to replace CA for evaluating pulmonary artery

  5. Labeling the pulmonary arterial tree in CT images for automatic quantification of pulmonary embolism

    NARCIS (Netherlands)

    Peters, R.J.M.; Marquering, H.A.; Dogan, H.; Hendriks, E.A.; De Roos, A.; Reiber, J.H.C.; Stoel, B.C.

    2007-01-01

    Contrast-enhanced CT Angiography has become an accepted diagnostic tool for detecting Pulmonary Embolism (PE). The CT obstruction index proposed by Qanadli, which is based on the number of obstructed arterial segments, enables the quantification of PE severity. Because the required manual

  6. Cardiac MRI in pulmonary artery hypertension: correlations between morphological and functional parameters and invasive measurements

    Energy Technology Data Exchange (ETDEWEB)

    Alunni, Jean-Philippe; Otal, Philippe; Rousseau, Herve; Chabbert, Valerie [CHU Rangueil, Department of Radiology, Toulouse (France); Degano, Bruno; Tetu, Laurent; Didier, Alain [CHU Larrey, Department of Pneumology, Toulouse (France); Arnaud, Catherine [CHU Rangueil, Department of Methods in Clinical Research, Toulouse (France); Blot-Souletie, Nathalie [CHU Rangueil, Department of Cardiology, Toulouse (France)

    2010-05-15

    To compare cardiac MRI with right heart catheterisation in patients with pulmonary hypertension (PH) and to evaluate its ability to assess PH severity. Forty patients were included. MRI included cine and phase-contrast sequences, study of ventricular function, cardiac cavity areas and ratios, position of the interventricular septum (IVS) in systole and diastole, and flow measurements. We defined four groups according to the severity of PH and three groups according to IVS position: A, normal position; B, abnormal in diastole; C, abnormal in diastole and systole. IVS position was correlated with pulmonary artery pressures and PVR (pulmonary vascular resistance). Median pulmonary artery pressures and resistance were significantly higher in patients with an abnormal septal position compared with those with a normal position. Correlations were good between the right ventricular ejection fraction and PVR, right ventricular end-systolic volume and PAP, percentage of right ventricular area change and PVR, and diastolic and systolic ventricular area ratio and PVR. These parameters were significantly associated with PH severity. Cardiac MRI can help to assess the severity of PH. (orig.)

  7. [Taurine induces apoptosis in pulmonary artery smooth muscle cells].

    Science.gov (United States)

    Zhang, Xiaodan; Sheng, Jiejing; Zhang, Caixiaz; Zhao, Fenghua

    2012-03-01

    To study the effect of taurine on apoptosis in PASMCs, and whether the death-receptor pathway act in the mechanism. Culture the PASMCs, and divided the cells into control, SD. Acridine orange(AO) assay and western-blot analysis on the expression of Bax, Bcl-2, Procaspase-3 and Fas were used to study the mechanism. A major finding of this study is that the Tau effects many apoptosis index, such as increasing the expression of Bax and Fas, decreasing the expression of Procaspase-3, and Bcl-2, accrescencing the mitochondrial depolarization, causing the nuclear shrinkage, all these datas demonstrated that Tau induced the apoptosis in pulmonary artery smooth muscle cells through mitochondrial-dependent pathway. Tau induces the apoptosis in pulmonary artery smooth muscle cells through death-receptor.

  8. Pulmonary artery wave propagation and reservoir function in conscious man: impact of pulmonary vascular disease, respiration and dynamic stress tests

    DEFF Research Database (Denmark)

    Su, Junjing; Manisty, Charlotte; Simonsen, Ulf

    2017-01-01

    and dynamic stress tests. Right heart catheterization was performed using a pressure and Doppler flow sensor tipped guidewire to obtain simultaneous pressure and flow velocity measurements in the pulmonary artery in control subjects and patients with pulmonary arterial hypertension (PAH) at rest. In controls...

  9. Surgical repair for a coronary-pulmonary artery fistula with a saccular aneurysm of the coronary artery.

    Science.gov (United States)

    Izumi, Kenta; Hisata, Yoichi; Hazam, Shiro

    2009-06-01

    The patient, a 69-year-old woman, had been diagnosed with a heart murmur. A chest X-ray at a local clinic had shown an abnormal shadow. Since CT revealed a 3-cm-diameter mass close to the pulmonary artery, we performed a coronary angiography and diagnosed her as having a coronary artery aneurysm associated with a coronary-pulmonary artery fistula. We incised the aneurysm under cardiac arrest, the wall of which had three openings that were suture closed from the inside and outside. The coronary-pulmonary artery fistula was suture closed. A postoperative angiography confirmed the disappearance of the coronary artery aneurysm and the abnormal blood vessels. The patient had an uneventful postoperative course and was discharged on postoperative day 15. We report a rare case of coronary-pulmonary artery fistula with a coronary artery aneurysm for which surgery was followed by an uneventful postoperative course.

  10. Racial and ethnic differences in pulmonary arterial hypertension

    OpenAIRE

    Al-Naamani, Nadine; Paulus, Jessica K.; Roberts, Kari E.; Pauciulo, Michael W.; Lutz, Katie; Nichols, William C.; Kawut, Steven M.

    2017-01-01

    This study explores the racial and ethnic differences in presentation, severity, and treatment of patients with pulmonary arterial hypertension (PAH) in a large multicenter registry. African American and Hispanic patients are more likely to present with associated PAH compared to non-Hispanic whites. Hispanic patients with PAH were less likely to be treated with PAH-specific medications compared to non-Hispanic whites.

  11. Role of Dimethylarginine Dimethylaminohydrolases (DDAH) in pulmonary arterial hypertension

    OpenAIRE

    Pullamsetti, Soni

    2005-01-01

    Idiopathic pulmonary arterial hypertension (IPAH) is a progressive and life- limiting disorder which is associated with impaired bioactivity and/or synthesis of endogenous nitric oxide (NO). The mechanisms resulting in this impairment are multifactorial. Recently, the impact of endogenous NO-synthase inhibitors such as dimethylarginines (ADMA and SDMA) has come into the focus of attention for various endothelial dysfunction associated cardiovascular disorders. As current evidence strongly sug...

  12. The measurement of blood speed in the pulmonary artery trunk

    International Nuclear Information System (INIS)

    Saro, J.P.; Bula-Cruz, J.; Rafael, J.A.; Botelho, M.F.; Lima, J.P.

    1998-01-01

    The paper describes a non invasive methodology for the measurement of blood speed in the pulmonary artery trunk. The methodology has been tested with a moving radioactive tracer (nuclear medicine). An image processing technique is proposed, for detection and analysis of a moving object with variable shape and intensity over time (radioactive bolus). Experiments on the application of the technique in nuclear medicine are critically analysed. (authors)

  13. Delayed post-lobectomy pulmonary artery stump thrombosis

    Directory of Open Access Journals (Sweden)

    Leith Sawalha

    2015-01-01

    Full Text Available We present a 67 year old male patient who underwent VATS right upper lobectomy with en bloc chest wall resection and right lower lobe superior segmentectomy for atypical Ewing Sarcoma. Serial chest CT scan done more than two years after the initial resection showed a new filling defect in the right upper pulmonary artery stump. A repeat chest CT scan after three months of oral anticoagulation showed complete resolution of the filling defect.

  14. Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

    Directory of Open Access Journals (Sweden)

    Tselios K

    2016-12-01

    Full Text Available Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz, University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada Abstract: Systemic lupus erythematosus (SLE is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH, after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg with normal pulmonary capillary wedge pressure (≤15 mmHg and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan, phosphodiesterase type 5 inhibitors (sildenafil, and vasodilators (epoprostenol. Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively. Keywords: systemic lupus erythematosus, pulmonary arterial hypertension, immunosuppressive, transthoracic echocardiogram, endothelin receptor antagonists

  15. Efficacy of 'breath holding at ease' during CT pulmonary angiography in the improvement of contrast enhancement in pulmonary arteries

    International Nuclear Information System (INIS)

    Li, YeYuan Jourena; Ardley, Nicholas; Lau, Kenneth K.; Lau, Theodore

    2013-01-01

    The aim of this study is to assess whether breath-hold at ease by patients during the CT pulmonary angiography (CTPA) will improve contrast enhancement in pulmonary arteries. Retrospective review was conducted on 51 consecutive CTPA studies undertaken in the traditional method of patients taking deep inspiration and breath-hold immediately prior to scanning, and subsequently 75 consecutive CTPA studies with patients breathing-holding at ease immediately prior to and during the scanning. Attenuation values in pulmonary arteries of different anatomical levels in both groups were measured in Hounsfield unit (HU). Results were compared between the two groups. The mean attenuation value in pulmonary arteries including pulmonary trunk, main and proximal pulmonary arteries calculated in HU in the 'deep inspiration and breath-hold' group was 327 HU (95% confidence interval: 315 to 339 HU). The mean attenuation value in pulmonary arteries in the 'breath hold at ease' group was calculated to be 390 HU (95% confidence interval: 381 to 399 HU); that is, an overall 17.95% increase (P-value<0.0001) in attenuation value in this group. By asking patients to breath-hold at ease instead of taking deep inspiration and breath hold prior to CTPA scan can effectively improve contrast enhancement in pulmonary arteries, therefore producing a higher-quality CT pulmonary angiography scan.

  16. Efficacy of 'breath holding at ease' during CT pulmonary angiography in the improvement of contrast enhancement in pulmonary arteries.

    Science.gov (United States)

    Li, YeYuan Jourena; Lau, Kenneth K; Ardley, Nicholas; Lau, Theodore

    2013-08-01

    The aim of this study is to assess whether breath-hold at ease by patients during the CT pulmonary angiography (CTPA) will improve contrast enhancement in pulmonary arteries. Retrospective review was conducted on 51 consecutive CTPA studies undertaken in the traditional method of patients taking deep inspiration and breath-hold immediately prior to scanning, and subsequently 75 consecutive CTPA studies with patients breathing-holding at ease immediately prior to and during the scanning. Attenuation values in pulmonary arteries of different anatomical levels in both groups were measured in Hounsfield unit (HU). Results were compared between the two groups. The mean attenuation value in pulmonary arteries including pulmonary trunk, main and proximal pulmonary arteries calculated in HU in the 'deep inspiration and breath-hold' group was 327 HU (95% confidence interval: 315 to 339 HU). The mean attenuation value in pulmonary arteries in the 'breath hold at ease' group was calculated to be 390 HU (95% confidence interval: 381 to 399 HU); that is, an overall 17.95% increase (P-value breath-hold at ease instead of taking deep inspiration and breath hold prior to CTPA scan can effectively improve contrast enhancement in pulmonary arteries, therefore producing a higher-quality CT pulmonary angiography scan. © 2013 The Authors. Journal of Medical Imaging and Radiation Oncology © 2013 The Royal Australian and New Zealand College of Radiologists.

  17. Pulmonary artery endothelial cell phenotypic alterations in a large animal model of pulmonary arteriovenous malformations after the Glenn shunt.

    Science.gov (United States)

    Kavarana, Minoo N; Mukherjee, Rupak; Eckhouse, Shaina R; Rawls, William F; Logdon, Christina; Stroud, Robert E; Patel, Risha K; Nadeau, Elizabeth K; Spinale, Francis G; Graham, Eric M; Forbus, Geoffrey A; Bradley, Scott M; Ikonomidis, John S; Jones, Jeffrey A

    2013-10-01

    Longevity of the superior cavopulmonary connection (SCPC) is limited by the development of pulmonary arteriovenous malformations (PAVM). The goal of this study was to determine whether phenotypic changes in pulmonary artery endothelial cells (PAEC) that favor angiogenesis occur with PAVM formation. A superior vena cava to right pulmonary artery connection was constructed in 5 pigs. Pulmonary arteries were harvested at 6 to 8 weeks after surgery to establish cultures of PAEC and smooth muscle cells, to determine cell proliferation, gene expression, and tubule formation. Abundance of proteins related to angiogenesis was measured in lung tissue. Contrast echocardiography revealed right-to-left shunting, consistent with PAVM formation. While the proliferation of smooth muscle cells from the right pulmonary artery (shunted side) and left pulmonary artery (nonshunted side) were similar, right PAEC proliferation was significantly higher. Expression profiles of genes encoding cellular signaling proteins were higher in PAECs from the right pulmonary artery versus left pulmonary artery. Protein abundance of angiopoietin-1, and Tie-2 (angiopoietin receptor) were increased in the right lung (both p SCPC concomitantly with differential changes in PAEC proliferative ability and phenotype. Moreover, there was a significant increase in the angiopoietin/Tie-2 complex in the right lung, which may provide novel therapeutic targets to attenuate PAVM formation after a SCPC. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  18. Neonatal pulmonary artery reconstruction during shunting to treat and prevent juxtaductal coarctation.

    Science.gov (United States)

    Brink, Johann; MacIver, Robroy; Lee, Melissa G Y; Konstantinov, Igor E; Cheung, Michael; Brizard, Christian P; d'Udekem, Yves

    2015-02-01

    Coarctation of the pulmonary artery may lead to its early obstruction. We investigated the outcomes of a strategy of aggressive reconstruction of the pulmonary arteries in the neonatal period. From 2000 to 2013 a total of 162 consecutive neonates (Gore-Tex (W. L. Gore & Associates, Newark, DE) patch (18), or autologous pericardium (6). Ten patients were directed to univentricular palliation and 18 to biventricular repair. There was 1 in-hospital death (4%) and 2 deaths after hospital discharge (inter-stage mortality, 7%). The follow-up of the 25 survivors was complete. After a mean of 3 ± 3 years, patients with single ventricle palliation reached the following stages: shunts (2); one and a half ventricle repair (1); bidirectional cavopulmonary shunt (4); and Fontan (2). Fourteen of the patients destined for biventricular physiology reached complete repair while 2 patients were still with shunts. There was no pulmonary artery occlusion. Focal narrowing or pulmonary artery hypoplasia was the main indication for 10 of the subsequent 36 reinterventions. Neonatal pulmonary artery reconstruction effectively prevents pulmonary artery occlusion and warrants pulmonary artery growth in the majority of cases of juxtaductal pulmonary artery coarctation. A number of these patients needed enlargement of their central pulmonary arteries in subsequent procedures. Indications of this reconstruction at the time of systemic-to-pulmonary shunting remains to be specified. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  19. Astragalus Polysaccharides Attenuate Monocrotaline-Induced Pulmonary Arterial Hypertension in Rats.

    Science.gov (United States)

    Yuan, Lin-Bo; Hua, Chun-Yan; Gao, Sheng; Yin, Ya-Ling; Dai, Mao; Meng, Han-Yan; Li, Piao-Piao; Yang, Zhong-Xin; Hu, Qing-Hua

    2017-01-01

    Astragalus polysaccharides (APS) have been shown to possess a variety of biological activities including anti-oxidant and anti-inflammation functions in a number of diseases. However, their function in pulmonary arterial hypertension (PAH) is still unknown. Rats received APS (200[Formula: see text]mg/kg once two days) for 2 weeks after being injected with monocrotaline (MCT; 60[Formula: see text]mg/kg). The pulmonary hemodynamic index, right ventricular hypertrophy, and lung morphological features of the rat models were examined, as well as the NO/eNOS ratio of wet lung and dry lung weight and MPO. A qRT-PCR and p-I[Formula: see text]B was used to assess IL-1[Formula: see text], IL-6 and TNF-[Formula: see text] and WB was used to detect the total I[Formula: see text]B. Based on these measurements, it was found that APS reversed the MCT-induced increase in mean pulmonary arterial pressure (mPAP) (from 32.731[Formula: see text]mmHg to 26.707[Formula: see text]mmHg), decreased pulmonary vascular resistance (PVR) (from 289.021[Formula: see text]mmHg[Formula: see text][Formula: see text] min/L to 246.351[Formula: see text]mmHg[Formula: see text][Formula: see text][Formula: see text]min/L), and reduced right ventricular hypertrophy (from 289.021[Formula: see text]mmHg[Formula: see text][Formula: see text][Formula: see text]min/L to 246.351 mmHg[Formula: see text][Formula: see text][Formula: see text]min/L) ([Formula: see text]0.05). In terms of pulmonary artery remodeling, the WT% and WA% decreased with the addition of APS. In addition, it was found that APS promoted the synthesis of eNOS and the secretion of NO, promoting vasodilation and APS decreased the MCT-induced elevation of MPO, IL-1[Formula: see text], IL-6 and TNF-[Formula: see text], reducing inflammation. Furthermore, APS was able to inhibit the activation of pho-I[Formula: see text]B[Formula: see text]. In couclusion, APS ameliorates MCT-induced pulmonary artery hypertension by inhibiting pulmonary arterial

  20. Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

    Directory of Open Access Journals (Sweden)

    Blesneac Cristina

    2016-06-01

    Full Text Available Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH, that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.

  1. Effects of streptozotocin-induced diabetes on the pharmacology of rat conduit and resistance intrapulmonary arteries

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    Howarth Frank C

    2009-01-01

    Full Text Available Abstract Background Poor control of blood glucose in diabetes is known to promote vascular dysfunction and hypertension. Diabetes was recently shown to be linked to an increased prevalence of pulmonary hypertension. The aim of this study was to determine how the pharmacological reactivity of intrapulmonary arteries is altered in a rat model of diabetes. Methods Diabetes was induced in rats by the β-cell toxin, streptozotocin (STZ, 60 mg/kg, and isolated conduit and resistance intrapulmonary arteries studied 3–4 months later. Isometric tension responses to the vasoconstrictors phenylephrine, serotonin and PGF2α, and the vasodilators carbachol and glyceryl trinitrate, were compared in STZ-treated rats and age-matched controls. Results STZ-induced diabetes significantly blunted the maximum response of conduit, but not resistance pulmonary arteries to phenylephrine and serotonin, without a change in pEC50. Agonist responses were differentially reduced, with serotonin (46% smaller affected more than phenylephrine (32% smaller and responses to PGF2α unaltered. Vasoconstriction caused by K+-induced depolarisation remained normal in diabetic rats. Endothelium-dependent dilation to carbachol and endothelium-independent dilation to glyceryl trinitrate were also unaffected. Conclusion The small resistance pulmonary arteries are relatively resistant to STZ-induced diabetes. The impaired constrictor responsiveness of conduit vessels was agonist dependent, suggesting possible loss of receptor expression or function. The observed effects cannot account for pulmonary hypertension in diabetes, rather the impaired reactivity to vasoconstrictors would counteract the development of pulmonary hypertensive disease.

  2. [Pulmonary arterial hypertension. Part II: Medical and surgical treatment].

    Science.gov (United States)

    Manes, Alessandra; Marinelli, Alessandro; Palazzini, Massimiliano; Negro, Luca; Leci, Enri; Gambetti, Simona; Bachetti, Cristina; Beciani, Elena; Conficoni, Elisa; Branzi, Angelo; Galiè, Nazzareno

    2009-06-01

    Treatment of pulmonary arterial hypertension (group 1 of clinical classification) has been recently characterized by important progresses, particularly in pharmacological therapy. Only until few years ago, patients with pulmonary arterial hypertension were treated with non-specific drugs, such as diuretics and digoxin for right heart failure and calcium-channel blockers in the minority of cases, responders to the acute vasoreactivity test. In addition, use of oral anticoagulant treatment was supported by uncontrolled studies. In the last 15 years (in particular in the last 8 years) different randomized controlled trials assessing the functional, clinical and hemodynamic efficacy of three classes of targeted drugs (prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors) with pulmonary vascular dilating and antiproliferative effects have been performed. This information has allowed the proposal of an evidence-based treatment algorithm. Treatment starts with general measures (physical activity, fertility control, respiratory tract infection, etc.) and supportive therapy (anticoagulant therapy, diuretics, oxygen, digoxin). Patients who respond to the acute vasoreactivity test (10% of idiopathic form) are treated with high doses of calcium-channel blockers, non-responders with targeted therapies either on monotherapy or combination. Usually an oral active drug is initiated and a second compound of a different class is combined in case of non-satisfactory response to the first treatment. Combination therapy should be performed only in specialized centers with large experience on use of targeted therapies and their relevant side effects. In case of failure of medical therapy, possible options are balloon atrial septostomy and/or listing for lung or heart-lung transplantation. As available treatments do not constitute a cure for pulmonary arterial hypertension, further progresses are expected in the near future.

  3. [Analysis of factors related to pulmonary hypertensive crisis in patients with idiopathic pulmonary arterial hypertension].

    Science.gov (United States)

    Zhang, Chen; Li, Qiangqiang; Zhu, Yan; Gu, Hong

    2014-06-10

    To explore the risk and protective factors for pulmonary hypertensive crisis (PHC) in patients with idiopathic pulmonary arterial hypertension (IPAH). A retrospective study was performed for 65 patients with a diagnosis of IPAH between January 2010 and December 2013. According to clinical manifestations, they were divided into two groups of susceptibility and non-susceptibility to PHC. Clinical and hemodynamic parameters were analyzed in univariate and multivariate manners. Among them, there were 32 males and 33 females with a mean age of (14.4 ± 12.3) (10/12-47.3) years. Twenty-three patients had typical manifestations of PHC and 18 of them were induced by exercises.Univariate analysis revealed that the proportion of patients with World Health Organization (WHO) functional class III-IV in PHC-susceptible group was significantly higher than PHC-nonsusceptible group (60.9% vs 23.8%, P = 0.003) while the percentage of patent foramen ovale in PHC-susceptible group was significantly lower than PHC-nonsusceptible group (8.7% vs 45.2%, P = 0.003).In patients with WHO functional classI-II, hemodynamic variables including the decline of pulmonary arterial pressure and positive rate in vasoreactivity testing in PHC-susceptible group were significantly higher than PHC-nonsusceptible group.In patients with WHO functional class III-IV, baseline pulmonary arterial pressure and mean right atrial pressure in PHC-susceptible group were significantly higher than those in PHC-nonsusceptible group. Multivariate Logistic regression analysis revealed that, for those with WHO functional class III-IV (OR = 23.45, 95%CI: 2.85-193.09) and the decline of systolic pulmonary arterial pressure in vasoreactivity testing (OR = 1.12, 95%CI: 1.04-1.22) were independent risk factors for PHC in IPAH patients while patent foramen ovale (OR = 0.01, 95%CI: 0.00-0.52) was a protective factor. PHC in IPAH patients is correlated with WHO functional class, pulmonary vascular reactivity, baseline pulmonary

  4. Visualization of peripheral pulmonary artery red thrombi utilizing optical coherence tomography

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Cheng; Wang, Wei; Zhong, Nan Shan; Zeng, Guang Qiao; Zhang, Nuo Fu [The First Affiliated Hospital of Guangzhou Medical College, Guangzhou (China)

    2013-10-15

    Optical coherence tomography (OCT) is a new imaging technique capable of obtaining high-resolution intravascular images and has been used in interventional cardiology. However, an application of OCT in pulmonary arteries had seldom been documented. In this case, OCT imaging is performed in peripheral pulmonary arteries and shows mural red thrombi. Subsequently, the red thrombi are aspirated and confirmed by a histological examination. These findings suggest that OCT may be a useful tool to depict peripheral pulmonary artery thrombi.

  5. Arterial tortuosity syndrome: An extremely rare disease presenting as a mimic of pulmonary sling

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    Amy Farkas, MD

    2018-02-01

    Full Text Available Pulmonary sling is the anatomic variant defined by the aberrant origin of the left pulmonary artery from the right pulmonary artery. This patient presented with a mimic of pulmonary sling as a result of an extremely rare condition, arterial tortuosity syndrome (ATS. The patient was first diagnosed with pulmonary sling on prenatal echocardiogram performed by cardiology. Computed tomography angiography of the chest obtained at birth to evaluate respiratory depression demonstrated ATS. The early detection of ATS has been demonstrated to improve patient outcome. This case provides an overview of the typical imaging features of ATS to aid radiologists in making this uncommon diagnosis.

  6. [Anatomic study and CT angiography with 3D reconstructions of the pulmonary arterial tree].

    Science.gov (United States)

    Seizeur, R; Gérard, R; Marion, J; Lefèvre, C; Nonent, M; Forlodou, P; Sénécail, B

    2010-08-01

    To prove in vivo and on cadaveric lungs, the constancy of the collateral type of distribution for the right and left pulmonary arteries as described in classical books and modern studies; to estimate the frequency of the main branches observed and define an arterial tree of reference set up with the most common branches for reading the CT pulmonary angiographies. Sixty three-dimensional reconstructions of pulmonary arterial trees (right: 30; left: 30) using the Volume Rendering Technique (VRT) performed from CT angiographic studies of 30 patients without bronchial, arterial or pulmonary pathology. Dissection of 16 pulmonary arteries from eight fresh cadavers injected with latex. Finally, 76 pulmonary arteries (right: 38; left: 38) were examined. The most common division of the pulmonary artery is a collateral distribution but in seven cases from 38 (18.4%) at right and one case from 38 (2.6%) at left the artery ended in two or three terminal lobar trunks which provided the segmental arteries. The mediastinal artery for the upper right lobe was always found, with a complementary scissural artery in 89.5% of cases; a middle lobar trunk was observed in 22 of the 38 right lungs (57.9%). On the left tree, four different but usually not coexisting mediastinal arteries were identified for the upper lobe, three for the culmen and one for the lingula; six various scissural arteries were noted, three for the culmen and three for the lingula; the segment 6 received one or two segmental arteries, exceptionally three. An arterial tree of reference could be defined for both sides. The division in terminal lobar trunks of the pulmonary arteries is a variation demonstrated for the first time. The data obtained from 3D reconstruction imaging in vivo are in conformity with the results of studies performed on injected cadaveric lungs for this point and to define the arterial tree of reference. Copyright 2010 Elsevier Masson SAS. All rights reserved.

  7. Evaluation of arterial blood flow changes by orbital Doppler in chronic obstructive pulmonary disease and asthma.

    Science.gov (United States)

    Yakut, Zeynep Ilerisoy; Karadağ, Remzi; Ozol, Duygu; Senturk, Aysegul

    2015-01-01

    Both chronic obstructive pulmonary disease (COPD) and asthma are important causes of morbidity and mortality worldwide. They primarily affect the lungs, but they have various extrapulmonary manifestations. The aim of our study was to evaluate the hemodynamic changes in orbital vessels of the patients with COPD and asthma using color Doppler ultrasonography and compare the results with healthy control subjects. Thirty-seven patients with COPD, 37 patients with asthma, and 41 healthy control subjects were included in this study. All patients with COPD were in moderate to severe group according to GOLD (Global Initiative for Chronic Obstructive Lung Disease), and similarly, all patients with asthma were in moderate to severe persistent group according to GINA (Global Initiative for Asthma) 2006 guidelines. End-tidal carbon dioxide, peripheral oxygen saturation, pulse rate, and respiratory rate were measured by using pulse oximeter in all patients. Measurements were performed in only 1 randomly selected eye of each participant. The peak systolic velocity, end diastolic velocity, and resistance index were measured in the central retinal artery, temporal posterior ciliary artery, and nasal posterior ciliary artery using the color Doppler ultrasonography technique. The peak systolic velocity, end diastolic velocity, and resistance index values of temporal posterior ciliary artery and nasal posterior ciliary artery were significantly higher in COPD and asthma than in the control subjects. There was no difference between asthma and COPD. We concluded that retrobulbar hemodynamics change in COPD and asthma is showing 1 of the systemic effects in these diseases.

  8. [Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery].

    Science.gov (United States)

    Furuya, Tomonori; Iida, Ryoji; Konishi, Jyumpei; Kato, Jitsu; Suzuki, Takahiro

    Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although there are several reports regarding pregnancy in patients with unilateral absence of a pulmonary artery, there are no case reports describing anesthesia for Cesarean section in a patient with unilateral absence of a pulmonary artery. We present a patient with unilateral absence of a pulmonary artery who underwent Cesarean sections twice at the ages of 24 and 26 years under spinal anesthesia for surgery and epidural analgesia for postoperative pain relief. Both times, spinal anesthesia and epidural analgesia enabled successful anesthesia management without the development of either pulmonary hypertension or right heart failure. Spinal anesthesia combined with epidural analgesia is a useful anesthetic method for a Cesarean section in patients with unilateral absence of a pulmonary artery. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  9. Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery.

    Science.gov (United States)

    Furuya, Tomonori; Iida, Ryoji; Konishi, Jyumpei; Kato, Jitsu; Suzuki, Takahiro

    Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. Although there are several reports regarding pregnancy in patients with unilateral absence of a pulmonary artery, there are no case reports describing anesthesia for Cesarean section in a patient with unilateral absence of a pulmonary artery. We present a patient with unilateral absence of a pulmonary artery who underwent Cesarean sections twice at the ages of 24 and 26 years under spinal anesthesia for surgery and epidural analgesia for postoperative pain relief. Both times, spinal anesthesia and epidural analgesia enabled successful anesthesia management without the development of either pulmonary hypertension or right heart failure. Spinal anesthesia combined with epidural analgesia is a useful anesthetic method for a Cesarean section in patients with unilateral absence of a pulmonary artery. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

  10. Advanced pulmonary arterial hypertension: mechanical support and lung transplantation

    Directory of Open Access Journals (Sweden)

    Sonja Bartolome

    2017-12-01

    Full Text Available The development of targeted therapies has transformed the outlook for patients with pulmonary arterial hypertension (PAH; however, some patients fail to achieve an adequate clinical response despite receiving maximal treatment. For these patients, lung transplantation remains an important therapeutic option, and recommendations for transplantation are included in the current European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension. Although lung transplantation is not without risk, overall long-term survival rates are good and substantial improvements in quality of life have been reported for lung transplant recipients. In this review, we describe the important considerations prior to, during and after transplantation, including the role of mechanical support, in patients with advanced PAH.

  11. Down-Regulation of TRPM8 in Pulmonary Arteries of Pulmonary Hypertensive Rats

    Directory of Open Access Journals (Sweden)

    Xiao-Ru Liu

    2013-06-01

    Full Text Available Background: Pulmonary hypertension (PH is characterized by profound vascular remodeling and alterations in Ca2+ homeostasis in pulmonary arterial smooth muscle cells (PASMCs. Multiple transient receptor potential melastatin-related (TRPM subtypes have been identified in vascular tissue. However, the changes in the expression and function of TRPM channels in pulmonary hypertension have not been characterized in detail. Methods: We examined the expression of TRPM channels and characterized the functions of the altered TRPM channels in two widely used rat models of chronic hypoxia (CH- and monocrotaline (MCT-induced PH. Results: CH-exposed and MCT-treated rats developed severe PH and right ventricular hypertrophy, with a significant decrease in TRPM8 mRNA and protein expression in pulmonary arteries (PAs. The downregulation of TRPM8 was associated with significant reduction in menthol-induced cation-influx. Time-profiles showed that TRPM8 down-regulation occurred prior to the increase of right ventricular systolic pressure (RVSP and right ventricular mass index (RVMI in CH-exposed rats, but these changes were delayed in MCT-treated rats. The TRPM8 agonist menthol induced vasorelaxation in phenylephrine-precontracted PAs, and the vasorelaxing effects were significantly attenuated in PAs of both PH rat models, consistent with decreased TRPM8 expression. Conclusion: Downregulation of TRPM8 may contribute to the enhanced vasoreactivity in PH.

  12. Role of chymase in cigarette smoke-induced pulmonary artery remodeling and pulmonary hypertension in hamsters

    Directory of Open Access Journals (Sweden)

    Yang Ting

    2010-03-01

    Full Text Available Abstract Background Cigarette smoking is an important risk factor for pulmonary arterial hypertension (PAH in chronic obstructive pulmonary disease (COPD. Chymase has been shown to function in the enzymatic production of angiotensin II (AngII and the activation of transforming growth factor (TGF-β1 in the cardiovascular system. The aim of this study was to determine the potential role of chymase in cigarette smoke-induced pulmonary artery remodeling and PAH. Methods Hamsters were exposed to cigarette smoke; after 4 months, lung morphology and tissue biochemical changes were examined using immunohistochemistry, Western blotting, radioimmunoassay and reverse-transcription polymerase chain reaction. Results Our results show that chronic cigarette smoke exposure significantly induced elevation of right ventricular systolic pressures (RVSP and medial hypertrophy of pulmonary arterioles in hamsters, concurrent with an increase of chymase activity and synthesis in the lung. Elevated Ang II levels and enhanced TGF-β1/Smad signaling activation were also observed in smoke-exposed lungs. Chymase inhibition with chymostatin reduced the cigarette smoke-induced increase in chymase activity and Ang II concentration in the lung, and attenuated the RVSP elevation and the remodeling of pulmonary arterioles. Chymostatin did not affect angiotensin converting enzyme (ACE activity in hamster lungs. Conclusions These results suggest that chronic cigarette smoke exposure can increase chymase activity and expression in hamster lungs. The capability of activated chymase to induce Ang II formation and TGF-β1 signaling may be part of the mechanism for smoking-induced pulmonary vascular remodeling. Thus, our study implies that blockade of chymase might provide benefits to PAH smokers.

  13. Canine pulmonary vein-to-pulmonary artery ratio: echocardiographic technique and reference intervals.

    Science.gov (United States)

    Birettoni, F; Caivano, D; Patata, V; Moïse, N S; Guglielmini, C; Rishniw, M; Porciello, F

    2016-12-01

    The size of the pulmonary veins (PVs) and pulmonary arteries (PAs) changes in response to hemodynamic alterations caused by physiological events and disease. We sought to create standardized echocardiographic methods for imaging the right ostium of the pulmonary veins (RPVs) and the right pulmonary artery (RPA) using specific landmarks and timing to quantify vessel diameters and phasic changes during the cardiac cycle. Fifty client-owned healthy dogs prospectively recruited. M-mode and 2-dimensional images were obtained from modified right parasternal long and short axis views. Right ostium of the pulmonary veins and RPA measurements were timed with electrical [peak of the QRS complex (RPV QRS and RPA QRS ) and end of T wave (RPV T and RPA T )] or mechanical events [RPV and RPA vessels at their respective maximal (RPV MAX ; RPA MAX ) and minimal (RPV MIN ; RPA MIN ) diameters]. Right ostium of the pulmonary veins and RPA measurements were also indexed to the aorta. In normal dogs regardless of the echocardiographic view or time in the cardiac cycle, the RPV/RPA ratio approximated 1.0. Mechanically timed fractional changes (distensibility indices) in RPV and RPA diameters did not differ (p=0.99; 36.9% and 36.8%, respectively). ECG-timed fractional changes (distensibility indices) in RPV and RPA diameter were at least 50% smaller than mechanically timed changes (p<0.05). RPV:Ao and RPA:Ao ranged between 0.3 and 0.6, with lower values obtained in diastole and larger values in systole (p<0.0001). Multiple positive and negative deflections were identified on the RPV and RPA M-mode tracings. This study provides detailed methodology and 2D and M-mode reference intervals for the RPV and RPA dimensions and the phasic changes during the cardiac cycle of the dog using echocardiography. Copyright © 2016 Elsevier B.V. All rights reserved.

  14. Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

    International Nuclear Information System (INIS)

    Griffin, N.; Allen, D.; Wort, J.; Rubens, M.; Padley, S.

    2007-01-01

    Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity

  15. Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Allen, D. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Wort, J. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Rubens, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-06-15

    Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity.

  16. Impact of age on pulmonary artery systolic pressures at rest and with exercise

    Directory of Open Access Journals (Sweden)

    Garvan C Kane

    2016-06-01

    Full Text Available Aim: It is not well known if advancing age influences normal rest or exercise pulmonary artery pressures. The purpose of the study was to evaluate the association of increasing age with measurements of pulmonary artery systolic pressure at rest and with exercise. Subjects and methods: A total of 467 adults without cardiopulmonary disease and normal exercise capacity (age range: 18–85 years underwent symptom-limited treadmill exercise testing with Doppler measurement of rest and exercise pulmonary artery systolic pressure. Results: There was a progressive increase in rest and exercise pulmonary artery pressures with increasing age. Pulmonary artery systolic pressures at rest and with exercise were 25 ± 5 mmHg and 33 ± 9 mmHg, respectively, in those <40 years, and 30 ± 5 mmHg and 41 ± 12 mmHg, respectively, in those ≥70 years. While elevated left-sided cardiac filling pressures were excluded by protocol design, markers of arterial stiffness associated with the age-dependent effects on pulmonary pressures. Conclusion: These data demonstrate that in echocardiographically normal adults, pulmonary artery systolic pressure increases with advancing age. This increase is seen at rest and with exercise. These increases in pulmonary pressure occur in association with decreasing transpulmonary flow and increases in systemic pulse pressure, suggesting that age-associated blood vessel stiffening may contribute to these differences in pulmonary artery systolic pressure.

  17. [Aneurysmal dilatation of pulmonary artery and its branches on mitral stenosis: a case report].

    Science.gov (United States)

    Yaméogo, N V; Ndiaye, M B; Diao, M; Cabral-Ciss, E C; Sarr, M; Ba, S A

    2013-02-01

    Pulmonary arteries aneurysms are rare and the rarely described bilateral aneurysms. A 45-year-old patient carrier of mitral stenosis was admitted for dyspnoea class III of the NYHA, chest pain and a not infectious cough. The clinical examination found semiology of mitral stenosis, tricuspid incapacity and pulmonary arterial hypertension. The electrocardiogram showed atrial fibrillation and right ventricle hypertrophy. Chest X-ray found a cardiomegaly, an aspect of double outline of the inferior right bow, a prominent aspect of the left average bow reminding an aneurysm of the left pulmonary artery. The echocardiography Doppler found a pure tight mitral stenosis (mitral surface=0.6 cm(2)), a dilation of the trunk of the pulmonary artery (diameter=74 mm) and of its branches (diameter of the right pulmonary artery=28 mm, diameter of the left pulmonary artery=36 mm) seat of a spontaneous contrast. The left atrium and right cardiac cavities were also dilated. There was an important tricuspid incapacity with a major pulmonary hypertension (systolic pulmonary arterial=109 mmHg). The thoracic angioscan showed a pseudoaneurysm of the trunk of pulmonary artery and its branches to their distal parts. Under diuretic, anticoagulating and analgesic treatment the clinical signs improved however the spontaneous contrast persisted. The patient was rejected by the surgery for exceeded clinical board. She is at present followed in our service for 5 months. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  18. Patient engagement and self-management in pulmonary arterial hypertension

    DEFF Research Database (Denmark)

    Graarup, Jytte; Ferrari, Pisana; Howard, Luke S

    2016-01-01

    of the patient may improve their ability to cope with pulmonary arterial hypertension, as well as help them to become effective in the self-management of their disease. Successful patient engagement can be achieved through effective education and the delivery and communication of timely, high-quality information....... A multidisciplinary approach involving healthcare professionals, carers, patient associations and expert patient programmes can also encourage patients to engage. Strategies that promote patient engagement can help to achieve the best possible care and support for the patient and also benefit healthcare providers....

  19. Protein losing enteropathy secondary to a pulmonary artery stent

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    Narayanswami Sreeram

    2012-01-01

    Full Text Available A 2-year-old patient with hypoplastic left heart syndrome presented 6 months following Fontan completion with protein-losing enteropathy (PLE. He had undergone stent implantation in the left pulmonary artery after the Norwood procedure, followed by redilation of the stent prior to Fontan completion. Combined bronchoscopic and catheterization studies during spontaneous breathing confirmed left bronchial stenosis behind the stent, and diastolic systemic ventricular pressure during expiration of 25 mm Hg. We postulate that the stent acts as a valve, against which the patient generates high expiratory pressures, which are reflected in the ventricular diastolic pressure. This may be the cause of PLE.

  20. Potassium Channel Subfamily K Member 3 (KCNK3) Contributes to the Development of Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Antigny, Fabrice; Hautefort, Aurélie; Meloche, Jolyane; Belacel-Ouari, Milia; Manoury, Boris; Rucker-Martin, Catherine; Péchoux, Christine; Potus, François; Nadeau, Valérie; Tremblay, Eve; Ruffenach, Grégoire; Bourgeois, Alice; Dorfmüller, Peter; Breuils-Bonnet, Sandra; Fadel, Elie; Ranchoux, Benoît; Jourdon, Philippe; Girerd, Barbara; Montani, David; Provencher, Steeve; Bonnet, Sébastien; Simonneau, Gérald; Humbert, Marc; Perros, Frédéric

    2016-04-05

    Mutations in the KCNK3 gene have been identified in some patients suffering from heritable pulmonary arterial hypertension (PAH). KCNK3 encodes an outward rectifier K(+) channel, and each identified mutation leads to a loss of function. However, the pathophysiological role of potassium channel subfamily K member 3 (KCNK3) in PAH is unclear. We hypothesized that loss of function of KCNK3 is a hallmark of idiopathic and heritable PAH and contributes to dysfunction of pulmonary artery smooth muscle cells and pulmonary artery endothelial cells, leading to pulmonary artery remodeling: consequently, restoring KCNK3 function could alleviate experimental pulmonary hypertension (PH). We demonstrated that KCNK3 expression and function were reduced in human PAH and in monocrotaline-induced PH in rats. Using a patch-clamp technique in freshly isolated (not cultured) pulmonary artery smooth muscle cells and pulmonary artery endothelial cells, we found that KCNK3 current decreased progressively during the development of monocrotaline-induced PH and correlated with plasma-membrane depolarization. We demonstrated that KCNK3 modulated pulmonary arterial tone. Long-term inhibition of KCNK3 in rats induced distal neomuscularization and early hemodynamic signs of PH, which were related to exaggerated proliferation of pulmonary artery endothelial cells, pulmonary artery smooth muscle cell, adventitial fibroblasts, and pulmonary and systemic inflammation. Lastly, in vivo pharmacological activation of KCNK3 significantly reversed monocrotaline-induced PH in rats. In PAH and experimental PH, KCNK3 expression and activity are strongly reduced in pulmonary artery smooth muscle cells and endothelial cells. KCNK3 inhibition promoted increased proliferation, vasoconstriction, and inflammation. In vivo pharmacological activation of KCNK3 alleviated monocrotaline-induced PH, thus demonstrating that loss of KCNK3 is a key event in PAH pathogenesis and thus could be therapeutically targeted.

  1. Transcriptome Analysis and Gene Identification in the Pulmonary Artery of Broilers with Ascites Syndrome.

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    Fei Yang

    Full Text Available Pulmonary arterial hypertension, also known as Ascites syndrome (AS, remains a clinically challenging disease with a large impact on both humans and broiler chickens. Pulmonary arterial remodeling presents a key step in the development of AS. The precise molecular mechanism of pulmonary artery remodeling regulating AS progression remains unclear.We obtained pulmonary arteries from two positive AS and two normal broilers for RNA sequencing (RNA-seq analysis and pathological observation. RNA-seq analysis revealed a total of 895 significantly differentially expressed genes (DEGs with 437 up-regulated and 458 down-regulated genes, which were significantly enriched to 12 GO (Gene Ontology terms and 4 KEGG (Kyoto Encyclopedia of Genes and Genomes pathways (Padj<0.05 regulating pulmonary artery remodeling and consequently occurrence of AS. These GO terms and pathways include ribosome, Jak-STAT and NOD-like receptor signaling pathways which regulate pulmonary artery remodeling through vascular smooth cell proliferation, inflammation and vascular smooth cell proliferation together. Some notable DEGs within these pathways included downregulation of genes like RPL 5, 7, 8, 9, 14; upregulation of genes such as IL-6, K60, STAT3, STAT5 Pim1 and SOCS3; IKKα, IkB, P38, five cytokines IL-6, IL8, IL-1β, IL-18, and MIP-1β. Six important regulators of pulmonary artery vascular remodeling and construction like CYP1B1, ALDH7A1, MYLK, CAMK4, BMP7 and INOS were upregulated in the pulmonary artery of AS broilers. The pathology results showed that the pulmonary artery had remodeled and become thicker in the disease group.Our present data suggested some specific components of the complex molecular circuitry regulating pulmonary arterial remodeling underlying AS progression in broilers. We revealed some valuable candidate genes and pathways that involved in pulmonary artery remodeling further contributing to the AS progression.

  2. Acute pulmonary edema following inflation of arterial tourniquet.

    Science.gov (United States)

    Santhosh, M C B; Pai, R B; Rao, R P

    2014-10-01

    Arterial tourniquets are used as one of the methods for reducing blood loss and for allowing blood free surgical field. A 20-year-old, 45 kg healthy female with a sphere shaped pendunculated hemangioma in the popliteal fossa of her left lower limb was applied with arterial tourniquet after exsanguination. The procedure was performed under general anesthesia. Soon after exsanguination and tourniquet inflation, the patient developed pulmonary edema which subsided after deflating the tourniquet. The clinical evolution, treatment and pathophysiology of this complication are described. Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.

  3. A Case of Pulmonary Hypertension Due to Fistulas Between Multiple Systemic Arteries and the Right Pulmonary Artery in an Adult Discovered for Occulted Dyspnoea.

    Science.gov (United States)

    Li, Ji-Feng; Zhai, Zhen-Guo; Kuang, Tu-Guang; Liu, Min; Ma, Zhan-Hong; Li, Yi-Dan; Yang, Yuan-Hua

    2017-08-01

    Pulmonary hypertension (PH) can be caused by a fistula between the systemic and pulmonary arteries. Here, we report a case of PH due to multiple fistulas between systemic arteries and the right pulmonary artery where the ventilation/perfusion scan showed no perfusion in the right lung. A 32-year-old male patient was hospitalised for community-acquired pneumonia. After treatment with antibiotics, the pneumonia was alleviated but dyspnoea persisted. Pulmonary hypertension was diagnosed using right heart catheterisation, which detected the mean pulmonary artery pressure as 37mmHg. The anomalies were confirmed by contrast-enhanced CT scan (CT pulmonary angiography), systemic arterial angiography and pulmonary angiography. Following embolisation of the largest fistula, the haemodynamics and oxygen dynamics did not improve, and even worsened to some extent. After supportive therapy including diuretics and oxygen, the patient's dyspnoea, WHO function class and right heart function by transthoracic echocardiography all improved during follow-up. Pulmonary hypertension can be present even when the right lung perfusion is lost. Closure of fistulas by embolisation, when those fistulas act as the proliferating vessels, may be harmful. Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  4. Arterial morphology responds differently to Captopril then N-acetylcysteine in a monocrotaline rat model of pulmonary hypertension

    Science.gov (United States)

    Molthen, Robert; Wu, Qingping; Baumgardt, Shelley; Kohlhepp, Laura; Shingrani, Rahul; Krenz, Gary

    2010-03-01

    Pulmonary hypertension (PH) is an incurable condition inevitably resulting in death because of increased right heart workload and eventual failure. PH causes pulmonary vascular remodeling, including muscularization of the arteries, and a reduction in the typically large vascular compliance of the pulmonary circulation. We used a rat model of monocrotaline (MCT) induced PH to evaluated and compared Captopril (an angiotensin converting enzyme inhibitor with antioxidant capacity) and N-acetylcysteine (NAC, a mucolytic with a large antioxidant capacity) as possible treatments. Twenty-eight days after MCT injection, the rats were sacrificed and heart, blood, and lungs were studied to measure indices such as right ventricular hypertrophy (RVH), hematocrit, pulmonary vascular resistance (PVR), vessel morphology and biomechanics. We implemented microfocal X-ray computed tomography to image the pulmonary arterial tree at intravascular pressures of 30, 21, 12, and 6 mmHg and then used automated vessel detection and measurement algorithms to perform morphological analysis and estimate the distensibility of the arterial tree. The vessel detection and measurement algorithms quickly and effectively mapped and measured the vascular trees at each intravascular pressure. Monocrotaline treatment, and the ensuing PH, resulted in a significantly decreased arterial distensibility, increased PVR, and tended to decrease the length of the main pulmonary trunk. In rats with PH induced by monocrotaline, Captopril treatment significantly increased arterial distensibility and decrease PVR. NAC treatment did not result in an improvement, it did not significantly increase distensibility and resulted in further increase in PVR. Interestingly, NAC tended to increase peripheral vascular density. The results suggest that arterial distensibility may be more important than distal collateral pathways in maintaining PVR at normally low values.

  5. Anaesthetic management of infants posted for repair of anomalous origin of left coronary artery from pulmonary artery

    Directory of Open Access Journals (Sweden)

    Chitralekha Patra

    2017-01-01

    Full Text Available First described in 1908, anomalous origin of left coronary artery from pulmonary artery is a very rare congenital anomaly. Here, the right coronary artery is usually enlarged and has a normal origin from aorta. Numerous collaterals connect the two coronary arteries over right ventricular outflow tract or interventricular septum. It is one of the most common causes of myocardial ischaemia and infarction in children.

  6. Measurement of pulmonary arterial elastance in patients with systolic heart failure using Doppler echocardiography

    Science.gov (United States)

    Taghavi, Sepideh; Esmaeilzadeh, Maryam; Amin, Ahmad; Naderi, Nasim; Abkenar, Hooman Bakhshandeh; Maleki, Majid; Mitra, Chitsazan

    2016-01-01

    Objective: A reliable and easy-to-perform method for measuring right ventricular (RV) afterload is desirable when scheduling patients with systolic heart failure to undergo heart transplantation. The present study aimed to investigate the accuracy of echocardiographically-derived pulmonary arterial elastance as a measurement of pulmonary vascular resistance by comparing it with invasive measures. Methods: Thirty-one patients with moderate to severe systolic heart failure, including 22 (71%) male patients, with a mean age of 41.16±15.9 years were enrolled in the study. Right heart catheterization and comprehensive echocardiography during the first hour after completion of cardiac catheterization were performed in all the patients. The pulmonary artery elastance was estimated using the ratio of end-systolic pressure (Pes) over the stroke volume (SV) by both cardiac catheterization [Ea (PV)-C] and echocardiography [Ea (PV)-E]. Results: The mean Ea (PV)-C and Ea (PV)-E were estimated to be 0.73±0.49 mm Hg/mL and 0.67±0.44 mm Hg/mL, respectively. There was a significant relation between Ea (PV)-E and Ea (PV)-C (r=0.897, pechocardiography and catheterization methods for estimating Ea (PV), investigated by the Bland-Altman method, showed a mean bias of -0.06, with 95% limits of agreement from -0.36 mm Hg/mL to 0.48 mm Hg/mL. Conclusion: Doppler echocardiography is an easy, non-invasive, and inexpensive method for measuring pulmonary arterial elastance, which provides accurate and reliable estimation of RV afterload in patients with systolic heart failure. PMID:26467379

  7. How to prevent echocardiographic misinterpretation of Gerbode type defect as pulmonary arterial hypertension.

    Science.gov (United States)

    Tehrani, Faramarz; Movahed, Mohammad-Reza

    2007-12-01

    We present a rare case of left ventricular to right atrial communication, a Gerbode type defect discovered in an adult female, originally misinterpreted as pulmonary arterial hypertension. The case report will be followed by the review of the literature and a discussion about how to prevent echocardiographic misinterpretation of this defect as pulmonary arterial hypertension using careful echocardiographic examination.

  8. Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+12 trial.

    Science.gov (United States)

    González-Saiz, Laura; Fiuza-Luces, Carmen; Sanchis-Gomar, Fabian; Santos-Lozano, Alejandro; Quezada-Loaiza, Carlos A; Flox-Camacho, Angela; Munguía-Izquierdo, Diego; Ara, Ignacio; Santalla, Alfredo; Morán, María; Sanz-Ayan, Paz; Escribano-Subías, Pilar; Lucia, Alejandro

    2017-03-15

    Pulmonary arterial hypertension is often associated with skeletal-muscle weakness. The purpose of this randomized controlled trial was to determine the effects of an 8-week intervention combining muscle resistance, aerobic and inspiratory pressure-load exercises on upper/lower-body muscle power and other functional variables in patients with this disease. Participants were allocated to a control (standard care) or intervention (exercise) group (n=20 each, 45±12 and 46±11years, 60% women and 10% patients with chronic thromboembolic pulmonary hypertension per group). The intervention included five, three and six supervised (inhospital) sessions/week of aerobic, resistance and inspiratory muscle training, respectively. The primary endpoint was peak muscle power during bench/leg press; secondary outcomes included N-terminal pro-brain natriuretic peptide levels, 6-min walking distance, five-repetition sit-to-stand test, maximal inspiratory pressure, cardiopulmonary exercise testing variables (e.g., peak oxygen uptake), health-related quality of life, physical activity levels, and safety. Adherence to training sessions averaged 94±0.5% (aerobic), 98±0.3% (resistance) and 91±1% (inspiratory training). Analysis of variance showed a significant interaction (group×time) effect for leg/bench press (Pexercise group (P0.1). We found a significant interaction effect (Pexercise. An 8-week exercise intervention including aerobic, resistance and specific inspiratory muscle training is safe for patients with pulmonary arterial hypertension and yields significant improvements in muscle power and other functional variables. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  9. Hypoxia-induced glucose-6-phosphate dehydrogenase overexpression and -activation in pulmonary artery smooth muscle cells: implication in pulmonary hypertension

    Science.gov (United States)

    Chettimada, Sukrutha; Gupte, Rakhee; Rawat, Dhwajbahadur; Gebb, Sarah A.; McMurtry, Ivan F.

    2014-01-01

    Severe pulmonary hypertension is a debilitating disease with an alarmingly low 5-yr life expectancy. Hypoxia, one of the causes of pulmonary hypertension, elicits constriction and remodeling of the pulmonary arteries. We now know that pulmonary arterial remodeling is a consequence of hyperplasia and hypertrophy of pulmonary artery smooth muscle (PASM), endothelial, myofibroblast, and stem cells. However, our knowledge about the mechanisms that cause these cells to proliferate and hypertrophy in response to hypoxic stimuli is still incomplete, and, hence, the treatment for severe pulmonary arterial hypertension is inadequate. Here we demonstrate that the activity and expression of glucose-6-phosphate dehydrogenase (G6PD), the rate-limiting enzyme of the pentose phosphate pathway, are increased in hypoxic PASM cells and in lungs of chronic hypoxic rats. G6PD overexpression and -activation is stimulated by H2O2. Increased G6PD activity contributes to PASM cell proliferation by increasing Sp1 and hypoxia-inducible factor 1α (HIF-1α), which directs the cells to synthesize less contractile (myocardin and SM22α) and more proliferative (cyclin A and phospho-histone H3) proteins. G6PD inhibition with dehydroepiandrosterone increased myocardin expression in remodeled pulmonary arteries of moderate and severe pulmonary hypertensive rats. These observations suggest that altered glucose metabolism and G6PD overactivation play a key role in switching the PASM cells from the contractile to synthetic phenotype by increasing Sp1 and HIF-1α, which suppresses myocardin, a key cofactor that maintains smooth muscle cell in contractile state, and increasing hypoxia-induced PASM cell growth, and hence contribute to pulmonary arterial remodeling and pathogenesis of pulmonary hypertension. PMID:25480333

  10. [A rare cause of exertional dry cough: agenesis of the left pulmonary artery associated with pulmonary hypoplasia].

    Science.gov (United States)

    Laaraje, Azzeddine; El Hafidi, Naima; Mahraoui, Chafik

    2017-01-01

    Agenesis of the left pulmonary artery associated with hypoplasia of the ipsilateral lung is a rare congenital malformation in children; it can be discovered fortuitously or because of the presence of recurrent respiratory infections. Diagnosis is based on thoracic angioscanner. Treatment is essentially conservative. We report the case of a 6-year old child with agenesis of the left pulmonary artery associated with hypoplasia of the ipsilateral lung detected because of exertional dry cough.

  11. H2S inhibits pulmonary arterial endothelial cell inflammation in rats with monocrotaline-induced pulmonary hypertension.

    Science.gov (United States)

    Feng, Shasha; Chen, Siyao; Yu, Wen; Zhang, Da; Zhang, Chunyu; Tang, Chaoshu; Du, Junbao; Jin, Hongfang

    2017-03-01

    This study aimed to determine whether hydrogen sulfide (H 2 S) inhibits pulmonary arterial endothelial inflammation in rats with monocrotaline (MCT)-induced pulmonary hypertension and its possible mechanisms. Twenty-four male Wistar rats were divided randomly into control, MCT, and MCT+H 2 S treatment groups. Human pulmonary arterial endothelial cells (HPAEC) were cultured and divided into four groups: control, MCT, MCT+H 2 S, and H 2 S. Pulmonary artery pressure was determined using a right cardiac catheterization procedure 3 weeks after MCT administration. Pulmonary vascular morphological changes and inflammatory infiltration were measured. Endogenous H 2 S levels, cystathionine-γ-lyase (CSE) expression, and inflammatory cytokines were determined both in vivo and in vitro. In addition, phosphorylation of NF-κB p65 and IκBα was detected by western blotting, and NF-κB p65 nuclear translocation, as well as its DNA-binding activity, was determined. Pulmonary hypertension and vascular remolding developed 3 wks after MCT administration, with elevated lung tissue inflammatory infiltration and cytokine level associated with activation of the NF-κB pathway, both in vivo and in vitro. However, the endogenous H 2 S/CSE pathway was downregulated in MCT rats. By contrast, an H 2 S donor markedly reduced pulmonary artery pressure, pulmonary vascular structural remolding, and increased lung inflammatory infiltration and cytokine levels of MCT-treated rats. Meanwhile, H 2 S reversed the activation of the NF-κB pathway successfully. The downregulated pulmonary arterial endothelial H 2 S/CSE pathway is involved in the pulmonary inflammatory response in MCT-treated pulmonary hypertensive rats. H 2 S attenuated endothelial inflammation by inhibiting the NF-κB pathway.

  12. Screening for pulmonary arterial hypertension in systemic sclerosis

    Directory of Open Access Journals (Sweden)

    J-L. Vachiéry

    2009-09-01

    Full Text Available The onset and progression of pulmonary arterial hypertension (PAH in patients with systemic sclerosis (SSc can be particularly aggressive; however, effective treatments are available. Therefore, early identification of patients with suspected PAH, confirmation of diagnosis, and intervention is essential. PAH may be challenging to diagnose in its earliest stages, particularly in populations that have multiple causes of breathlessness, and, therefore, screening is required. The optimal screening tools and methodology are, as yet, unknown, and this is confounded by a lack of consensus over which patients to screen. Current practice favours annual screening of all SSc patients using Doppler echocardiography to detect elevated right heart pressures. This will typically identify most patients with the various forms of pulmonary hypertension found in SSc. The optimum thresholds for Doppler echocardiography are still subject to investigation, especially for patients with mild pulmonary hypertension, and this technique may, therefore, yield a significant number of false-positives and a currently unknown number of false-negatives. Confirmatory right heart catheterisation remains necessary in all suspected cases. Further research is needed to identify the optimal tools and the screening approach with greatest specificity and selectivity.

  13. Therapies for pulmonary arterial hypertension: where are we today, where do we go tomorrow?

    Directory of Open Access Journals (Sweden)

    Andrei Seferian

    2013-09-01

    Full Text Available Pulmonary arterial hypertension (PAH is a progressive disease characterised by remodelling of small pulmonary arteries leading to an increased pulmonary vascular resistance, right ventricular failure and death. Available treatments try to re-establish the equilibrium on three signalling pathways: the prostacyclin, the endothelin (ET-1 and the nitric oxide. Prostanoids, such as epoprostenol or treprostinil have a vasodilator, antiproliferative and immunomodulatory effect and, despite the administration inconveniences, represent established therapies for severe cases of PAH. Recently oral prostacyclin receptor agonists have shown encouraging results. Many clinical studies targeting the vasoconstrictor ET-1 pathway with receptor antagonists like bosentan and ambrisentan have shown strong results, even more optimism coming from macitentan, the newest drug. Sildenafil and tadalafil, two phosphodiesterase type-5 inhibitors, have shown improved exercise capacity by increasing the nitric oxide level. Riociguat, acting on the same nitric oxide pathway, as a guanylatecyclase activator, has shown promising results in clinical trials and will be available soon. Long-awaited results for tyrosin-kinase inhibitor, imatinib, as an antiproliferative therapy in PAH have been disappointing, due to severe adverse events. In conclusion, although it remains a disease with severe prognosis, the past 20 years have represented a huge progress in terms of treatments for PAH with interesting opportunities for the future.

  14. Bosentan in the treatment of pulmonary arterial hypertension with the focus on the mildly symptomatic patient

    Directory of Open Access Journals (Sweden)

    Christopher J Valerio

    2009-08-01

    Full Text Available Christopher J Valerio, John G CoghlanDepartment of Cardiology, Royal Free Hospital, London, UKAbstract: Pulmonary arterial hypertension (PAH is a progressive disease with poor survival outcomes. Bosentan is an oral endothelin-1 receptor antagonist (ERA that has been shown in a large randomized placebo-controlled trial (BREATHE-1 to be effective at improving exercise tolerance in patients with PAH in functional class III and IV. Further studies have been conducted showing: benefit in smaller subgroups of PAH, eg, congenital heart disease, efficacy in combination with other PAH therapies, eg, sildenafil, improved long-term survival compared with historical controls. More recently, controlled trials of new ERAs have included patients with milder symptoms; those in functional class II. Analysis of the functional class II data is often limited by small numbers. These trials have generally shown a similar treatment effect to bosentan, but there are no controlled trials directly comparing these new ERAs. The EARLY trial exclusively enrolled functional class II patients and assessed hemodynamics at 6 months. Though significant, the reduction in pulmonary vascular resistance is merely a surrogate marker for the intended aim of delaying disease progression. Significant adverse effects associated with bosentan include edema, anemia and transaminase elevation. These may preclude a long duration of treatment. Further studies are required to determine optimum treatment strategy in mild disease.Keywords: pulmonary arterial hypertension, bosentan, endothelin-1 receptor antagonist

  15. Drug resistance patterns in pulmonary tuberculosis

    International Nuclear Information System (INIS)

    Khoharo, H.K.; Shaikh, I.A.

    2011-01-01

    Objective: To determine the resistance patterns of mycobacterium tuberculosis (MTB) isolates among category I and II patients of pulmonary tuberculosis. Methods: This cross sectional study was conducted at the Department of Medicine, Liaquat University of Medical and Health Sciences Jamshoro, from November 2008 to September 2009. Patients were divided into category I and II. The sputa were collected, stained with Ziehl-Nielsen (Z-N) staining and ultimately inoculated on Lowenstein-Jensen (L-J) media for six weeks. Out of 890 pulmonary tuberculosis (PTB) patients, the growth was obtained in 285 cases. The Drug sensitivity testing (DST) for Isoniazid (INH), Rifampicin (RIF), Ethambutol (EMB) Pyrazinamide (PZA) and Streptomycin (SM) were performed. The data was analyzed on SPSS 10.0. A p-value of <0.05 was taken as significant. Result: Out of 285 cases, 176 (61.75%) were male and 109 (38.24%) female. The mean age was 37 +- 19.90 years. The DST showed drug sensitive and drug resistant isolates in 80 (28.05%) and 205 (71.92%) cases respectively (p=0.001). The drug resistant tuberculosis (DR-TB) rates for individual drugs; INH, RIF, EMB, PZA and SM were 51,22%, 15.4%, 13.33%, 9%12, and 3.85% respectively (p=0.03). The MDR-TB isolates were detected in 120 (42.10%) cases, including 5 (5.88%) in category I and 115 (57.50%) in category II patients (p=0.0001). Conclusion: Drug resistant and multidrug resistant tuberculosis was observed mainly in category II patients. However, primary MDR was also observed in category I patients and reflects dissemination of MDR cases within the community. (author)

  16. Change of extracellular signal-regulated kinase expression in pulmonary arteries from smokers with and without chronic obstructive pulmonary disease.

    Science.gov (United States)

    Liu, Kui; Liu, Xian-Sheng; Yu, Mu-Qing; Xu, Yong-Jian

    2013-01-01

    Cigarette smoking may contribute to pulmonary hypertension in chronic obstructive pulmonary disease (COPD) by resulting in pulmonary vascular remodeling that involves pulmonary artery smooth muscle cell (PASMC) proliferation. However, the molecular mechanism underlying this process remains poorly understood. The purpose of this study was to investigate the role of extracellular signal-regulated kinase (ERK) in pulmonary arteries from smokers with normal lung function and smokers with mild to moderate COPD. The peripheral lung tissues were obtained from 14 nonsmokers with normal lung function, 18 smokers with normal lung function, and 16 smokers with mild to moderate COPD. The morphological changes of pulmonary arteries were observed by hematoxylin-eosin (HE) staining. Primary cultured human pulmonary artery smooth muscle cells (HPASMCs) were exposed to cigarette smoke extract (CSE). Cell proliferation was determined by cell counting and Methyl thiazolyl tetrazolium assay. Protein expression was analyzed by western blotting. Morphometrical analysis showed that the pulmonary vessel wall thickness in smoker group and COPD group was significantly greater than that in nonsmoker group (P < .01). The protein level of ERK was significantly increased in smoker group and COPD group as compared with nonsmoker group (P < .01). The expression of ERK was significantly increased in HPASMCs at protein levels when HPASMCs were treated with 5% CSE (P < .01), which significantly promoted the proliferation of HPASMCs (P < .01). Increased expression of ERK might be involved in the pathogenesis of abnormal proliferation of PASMCs in smokers with and without COPD.

  17. Segmental and subsegmental pulmonary arteries: evaluation with electron-beam versus spiral CT.

    Science.gov (United States)

    Schoepf, U J; Helmberger, T; Holzknecht, N; Kang, D S; Bruening, R D; Aydemir, S; Becker, C R; Muehling, O; Knez, A; Haberl, R; Reiser, M F

    2000-02-01

    To compare contrast agent-enhanced spiral and electron-beam computed tomography (CT) for the analysis of segmental and subsegmental pulmonary arteries. CT angiography of the pulmonary arteries was performed in 56 patients to rule out pulmonary embolism. Electron-beam CT was performed in 28 patients. The other 28 patients underwent spiral CT with comparable scanning protocols. The depiction of segmental and subsegmental arteries was analyzed by three independent readers. The contrast enhancement in the main pulmonary artery was measured in each patient. Analysis was performed in 1,120 segmental and 2, 240 subsegmental arteries. One segmental (RA7, P =.010) and two subsegmental (LA7b, P =.029; RA6a+b, P =.038) arteries in paracardiac and basal segments of the lung were depicted significantly better with electron-beam CT. There was no statistically significant difference between electron-beam and spiral CT in the total number of analyzable peripheral arteries depicted. The mean contrast enhancement in the main pulmonary artery was 362 HU in electron-beam CT studies versus 248 HU in spiral CT studies. Detailed visualization of peripheral pulmonary arteries is well within the scope of advanced CT techniques. Electron-beam CT has minor advantages in analyzing paracardiac arteries, probably because of reduction of motion artifacts and higher contrast enhancement. Further studies are needed to establish whether electron-beam CT allows a more confident diagnosis of emboli in these vessels.

  18. Mycotic pulmonary artery aneurysm as an unusual complication of thoracic actinomycosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyung-Soo; Lee, Sang-Yeub [Wonkwang University College, Iksan (Korea, Republic of); Oh, Yu-Whan; Noh, Hyung-Jun; Lee, Ki-Yeol; Kang, Eun-Young [Korea University Hospital and Korea University College, Seoul (Korea, Republic of)

    2004-03-15

    Although pulmonary artery aneurysms are a rare vascular anomaly, they are seen in a wide variety of conditions, such as congenital heart disease, infection, trauma, pulmonary hypertension, cystic medial necrosis and generalized vasculitis. To our knowledge, mycotic aneurysms caused by pulmonary actinomycosis have not been reported in the radiologic literature. Herein, a case of pulmonary actinomycosis complicated by mycotic aneurysm is presented. On CT scans, this case showed focal aneurysmal dilatation of a peripheral pulmonary artery within necrotizing pneumonia of the right lower lobe, which was successfully treated with transcatheter embolization using wire coils.

  19. Erythropoietin Attenuates Pulmonary Vascular Remodeling in Experimental Pulmonary Arterial Hypertension through Interplay between Endothelial Progenitor Cells and Heme Oxygenase

    OpenAIRE

    van Loon, Rosa Laura E; Bartelds, Beatrijs; Wagener, Frank A D T G; Affara, Nada; Mohaupt, Saffloer; Wijnberg, Hans; Pennings, Sebastiaan W C; Takens, Janny; Berger, Rolf M F

    2015-01-01

    BACKGROUND: Pulmonary arterial hypertension (PAH) is a pulmonary vascular disease with a high mortality, characterized by typical angio-proliferative lesions. Erythropoietin (EPO) attenuates pulmonary vascular remodeling in PAH. We postulated that EPO acts through mobilization of endothelial progenitor cells (EPCs) and activation of the cytoprotective enzyme heme oxygenase-1 (HO-1). METHODS: Rats with flow-associated PAH, resembling pediatric PAH, were treated with HO-1 inducer EPO in the pre...

  20. Erythropoietin Attenuates Pulmonary Vascular Remodeling in Experimental Pulmonary Arterial Hypertension through Interplay between Endothelial Progenitor Cells and Heme Oxygenase

    OpenAIRE

    van Loon, Rosa Laura E.; Bartelds, Beatrijs; Wagener, Frank A. D. T. G.; Affara, Nada; Mohaupt, Saffloer; Wijnberg, Hans; Pennings, Sebastiaan W. C.; Takens, Janny; Berger, Rolf M. F.

    2015-01-01

    Background Pulmonary arterial hypertension (PAH) is a pulmonary vascular disease with a high mortality, characterized by typical angio-proliferative lesions. Erythropoietin (EPO) attenuates pulmonary vascular remodeling in PAH. We postulated that EPO acts through mobilization of endothelial progenitor cells (EPCs) and activation of the cytoprotective enzyme heme oxygenase-1 (HO-1). Methods Rats with flow-associated PAH, resembling pediatric PAH, were treated with HO-1 inducer EPO i...

  1. Assessment of pulmonary artery pressure by echocardiography—A comprehensive review

    Directory of Open Access Journals (Sweden)

    Sathish Parasuraman

    2016-09-01

    Full Text Available Pulmonary hypertension is a pathological haemodynamic condition defined as an increase in mean pulmonary arterial pressure ≥ 25 mmHg at rest, assessed using gold standard investigation by right heart catheterisation. Pulmonary hypertension could be a complication of cardiac or pulmonary disease, or a primary disorder of small pulmonary arteries. Elevated pulmonary pressure (PAP is associated with increased mortality, irrespective of the aetiology. The gold standard for diagnosis is invasive right heart catheterisation, but this has its own inherent risks. In the past 30 years, immense technological improvements in echocardiography have increased its sensitivity for quantifying pulmonary artery pressure (PAP and it is now recognised as a safe and readily available alternative to right heart catheterisation. In the future, scores combining various echo techniques can approach the gold standard in terms of sensitivity and accuracy, thereby reducing the need for repeated invasive assessments in these patients.

  2. A Case of Pulmonary Artery Aneurysm Associated with Patent Ductus Arteriosus : Detection by Radionuclide Cardiac Angiography

    International Nuclear Information System (INIS)

    Sohn, I.; Lee, M. C.; Cho, B. Y.; Koh, C. S.; Yoon, Y. S.; Hong, C. Y.; Rho, J. R.; Youn, K. M.; Han, M. C.

    1981-01-01

    A Case of main pulmonary artery aneurysm in a 9-year-old boy with patent ductus arteriosus is presented. In this case presented with a huge mass density on the chest X-ray, radionuclide cardiac angiography showed a vascular lesion, which was confirmed as an aneurysm of the main pulmonary artery at roentgenologic angiogram. The aneurysm appeared following an episode of bacterial endocarditis and pulmonary hypertension. A successful aneurysmectomy with multiple ligation of ductus arteriosus was performed.

  3. A Case of Pulmonary Artery Aneurysm Associated with Patent Ductus Arteriosus : Detection by Radionuclide Cardiac Angiography

    Energy Technology Data Exchange (ETDEWEB)

    Sohn, I.; Lee, M. C.; Cho, B. Y.; Koh, C. S.; Yoon, Y. S.; Hong, C. Y.; Rho, J. R.; Youn, K. M.; Han, M. C. [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1981-09-15

    A Case of main pulmonary artery aneurysm in a 9-year-old boy with patent ductus arteriosus is presented. In this case presented with a huge mass density on the chest X-ray, radionuclide cardiac angiography showed a vascular lesion, which was confirmed as an aneurysm of the main pulmonary artery at roentgenologic angiogram. The aneurysm appeared following an episode of bacterial endocarditis and pulmonary hypertension. A successful aneurysmectomy with multiple ligation of ductus arteriosus was performed.

  4. A mycotic pulmonary artery aneurysm associated with candida endocarditis: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Jin Il; Lee, Ji Won; Jeong, Yeon Joo; Song, Seung Hwan [Pusan National University School of Medicine, Medical Research Institute, Pusan National University Hospital, Busan (Korea, Republic of)

    2014-03-15

    We report a case of a mycotic pulmonary aneurysm associated with Candida endocarditis in a 53-year-old male with lymphoma. The initial diagnosis was a pulmonary artery aneurysm attributable to vasculitis, such as that associated with Behcet's disease, but a mycotic pulmonary artery aneurysm was later considered as a differential diagnosis. Identification of valve vegetation on the chest CT was helpful in this regard. We review the literature on the disease etiology, radiological findings, and management options.

  5. Pulmonary atresia and ventricular septal defect with collaterals to right lung associated with anomalous left pulmonary artery from the ascending aorta

    Energy Technology Data Exchange (ETDEWEB)

    Khositseth, Anant [Mahidol University, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Bangkok (Thailand); Siripornpitak, Suvipaporn; Pornkul, Ratanaporn [Mahidol University, Department of Radiology, Ramathibodi Hospital, Bangkok (Thailand)

    2010-12-15

    We present a 10-month-old boy with cyanosis. This is a rare case of pulmonary atresia, ventricular septal defect (VSD), major aorto-pulmonary collateral arteries (MAPCAs) to the right lung with absent native right pulmonary artery (RPA) in association with anomalous left pulmonary artery (LPA) from the ascending aorta (AAo). Echocardiography was unable to identify all of the cardiovascular abnormalities. Multidetector CT demonstrated all of these abnormalities and is the investigation of choice instead of cardiac catheterization. (orig.)

  6. Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review

    International Nuclear Information System (INIS)

    Cowles, Robert A.; Berdon, Walter E.

    2007-01-01

    The landmark 1933 case report from Massachusetts General Hospital by Bland, White and Garland (Am Heart J 8:787-801) described a 3-month-old child with progressive feeding problems, cardiomegaly on chest radiography, and EKG evidence of left ventricular damage. Of interest was the fact that the vigilant father of the infant was Aubrey Hampton, a radiologist and future chairman of radiology at Massachusetts General Hospital. At autopsy, the left coronary artery originated from the pulmonary artery rather than from the aorta. Effective treatment for this condition was not available until 1960 when Sabiston, Neill and Taussig showed that the blood flowed from the left coronary artery toward the pulmonary artery. The anomalous left coronary artery was ligated at its junction with the pulmonary artery and the child survived. This historical review of Bland-White-Garland syndrome, now known as anomalous left coronary artery arising from the pulmonary artery (ALCAPA), stresses the continued diagnostic significance of cardiomegaly on chest radiography and EKG changes suggesting left ventricular damage in 2- to 3-month-old infants with feeding intolerance or irritability. With a high index of suspicion, an echocardiogram can be obtained to confirm the diagnosis. Modern surgical methods involve left coronary artery translocation and afford excellent outcomes. (orig.)

  7. Pulmonary hyperinflation due to gas trapping and pulmonary artery size: The MESA COPD Study.

    Directory of Open Access Journals (Sweden)

    Hooman D Poor

    Full Text Available Pulmonary hypertension is associated with increased morbidity and mortality in chronic obstructive pulmonary disease (COPD. Since pulmonary artery (PA size increases in pulmonary hypertension, we measured PA cross-sectional area using magnetic resonance imaging (MRI to test the hypothesis that pulmonary hyperinflation due to gas trapping is associated with PA cross-sectional area in COPD.The MESA COPD Study recruited participants with COPD and controls from two population-based cohort studies ages 50-79 years with 10 or more pack-years and free of clinical cardiovascular disease. Body plethysmography was performed according to standard criteria. Cardiac MRI was performed at functional residual capacity to measure the cross-sectional area of the main PA. Percent emphysema was defined as the percentage of lung voxels less than -950 Hounsfield units as assessed via x-ray computed tomography. Analyses were adjusted for age, gender, height, weight, race-ethnicity, the forced expiratory volume in one second, smoking status, pack-years, lung function, oxygen saturation, blood pressure, left ventricular ejection fraction and percent emphysema.Among 106 participants, mean residual volume was 1.98±0.71 L and the mean PA cross-sectional area was 7.23±1.72 cm2. A one standard deviation increase in residual volume was independently associated with an increase in main PA cross-sectional area of 0.55 cm2 (95% CI 0.18 to 0.92; p = 0.003. In contrast, there was no evidence for an association with percent emphysema or total lung capacity.Increased residual volume was associated with a larger PA in COPD, suggesting that gas trapping may contribute to pulmonary hypertension in COPD.

  8. Left ventricular dysfunction in patients with suspected pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Francisca Gavilanes

    2014-12-01

    Full Text Available OBJECTIVE: To evaluate the role of right heart catheterization in the diagnosis of pulmonary arterial hypertension (PAH. METHODS: We evaluated clinical, functional, and hemodynamic data from all patients who underwent right heart catheterization because of diagnostic suspicion of PAH-in the absence of severe left ventricular dysfunction (LVD, significant changes in pulmonary function tests, and ventilation/perfusion lung scintigraphy findings consistent with chronic pulmonary thromboembolism-between 2008 and 2013 at our facility. RESULTS: During the study period, 384 patients underwent diagnostic cardiac catheterization at our facility. Pulmonary hypertension (PH was confirmed in 302 patients (78.6%. The mean age of those patients was 48.7 years. The patients without PH showed better hemodynamic profiles and lower levels of B-type natriuretic peptide. Nevertheless, 13.8% of the patients without PH were categorized as New York Heart Association functional class III or IV. Of the 218 patients who met the inclusion criteria, 40 (18.3% and 178 (81.7% were diagnosed with PH associated with LVD (PH-LVD and with PAH, respectively. The patients in the HP-LVD group were significantly older than were those in the PAH group (p < 0.0001. CONCLUSIONS: The proportional difference between the PAH and PH-LVD groups was quite significant, considering the absence of echocardiographic signs suggestive of severe LVD during the pre-catheterization investigation. Our results highlight the fundamental role of cardiac catheterization in the diagnosis of PAH, especially in older patients, in whom the prevalence of LVD that has gone undiagnosed by non-invasive tests is particularly relevant.

  9. Genistein attenuates monocrotaline-induced pulmonary arterial hypertension in rats by activating PI3K/Akt/eNOS signaling.

    Science.gov (United States)

    Zheng, Zeqi; Yu, Songping; Zhang, Wan; Peng, Yongchao; Pu, Mingyu; Kang, Ting; Zeng, Junyi; Yu, Yuefei; Li, Guorong

    2017-01-01

    Phytoestrogen genistein may be useful to treat pulmonary arterial hypertension (PAH). However, its mechanism is still not clear. The aim of the present study was to confirm the therapeutic effects of phytoestrogen genistein on PAH in monocrotaline-induced rat model and to explore its mechanism. Sprague-Dawley male rats were randomly divided into 4 groups: control group (n=8), PAH group (n=8), genistein treament group with three different doses (n=8 in each dose group) and group of PI3K inhibitor LY294002. The rat model of PAH was induced by monocrotaline (MCT). The situation of survival of rats was observed. Pathological studies of lung and heart tissues were performed. Western-blot detection of P-Akt and P-eNOS expression levels in lung tissue was carried out. Nitrate reductase analysis was used to measure nitric oxide (NO) in lung tissue. Genistein treatment resulted in significant improvement in the speed of tricuspid regurgitation, diameter of pulmonary artery, mean pulmonary artery pressure and right ventricular hypertrophy index. Genistein treatment also resulted in significant improvement in the stenosis of pulmonary artery, proliferation of smooth muscle, right ventricular hypertrophy and myocardial hypertrophy. These therapeutic effects were more obvious with increasing dose of genistein. After genistein treatment, amelioration in survival rates of PAH rats was observed. PI3K inhibitor LY294002 could block these therapeutic effects. In rat lung tissue, P-Akt, P-eNOS and NO expressions were increased significantly in genistein treatment group when compared with PAH group (p0.05). We confirmed that genistein could relax pulmonary vascular resistance, reduce pulmonary artery pressure, improve right heart function and ameliorate survival rate in the rat model of PAH. Our study suggested that its mechanism was related with PI3K/Akt/eNOS signal pathway. Phytoestrogen genistein may become a new and effective drug for patients with PAH.

  10. [Central arterial pressure in patients with chronic obstructive pulmonary disease].

    Science.gov (United States)

    Gel'tser, B I; Brodskaia, T A

    2008-01-01

    To study central (aortic) arterial pressure (CAP) and aortic stiffness in patients with chronic obstructive pulmonary disease (COPD) of different severity. Non-invasive arteriography with Tensio Climo TL1 arteriograph (TensioMed, Hungary) was made to measure aortic stiffness and systolic pressure (SAP) in 54 COPD patients and 25 healthy controls. The difference between the central and peripheral SAP (delta SAP) and central/ peripheral pressure correspondence index (CI) were estimated. Indirect arteriography has found that patients with moderate and severe COPD have stable elevated central SAP which is close to brachial SAP while in healthy controls the difference between central and peripheral SAP is 10.2 +/- 2.1 mmHg. With progression of COPD severity, deltaSAP diminishes while CI rises showing growing disproportion between central and peripheral blood pressure. In severe COPD physiological difference between them disappears. In COPD increased CAP is associated with impaired mechanical properties of the arterial bed and myocardial contractility proved by significant links between CAP and left ventricular ejection fraction index and key parameters of arterial stiffness. Aortic CAP, delta SAP and CI are additional informative criteria of COPD severity and high cardiovascular risk as shown by their close correlation with hypoxemia, severity and duration of the disease.

  11. Analysis of the degree of pulmonary thallium washout after exercise in patients with coronary artery disease

    International Nuclear Information System (INIS)

    Levy, R.; Rozanski, A.; Berman, D.S.; Garcia, E.; Van Train, K.; Maddahi, J.; Swan, H.J.

    1983-01-01

    An abnormal increase in pulmonary thallium activity may be visualized on post-stress thallium images in patients with coronary artery disease. Because this increased pulmonary thallium activity usually disappears by the time of redistribution imaging, this study was designed to assess whether measurement of the degree of pulmonary thallium washout between stress and redistribution might improve the detection of increased pulmonary thallium activity in patients with coronary artery disease. Quantitative analysis revealed abnormal (that is, greater than 2 standard deviations of normal values) pulmonary thallium washouts in 59 (64%) of 92 patients with coronary artery disease, but in only 2 (25%) of 8 subjects with angiographically normal arteries (p less than 0.06). By comparison, the visual analysis of pulmonary thallium washout and use of initial pulmonary to myocardial thallium ratio were significantly (p less than 0.05) less sensitive in detecting abnormality in patients with coronary artery disease. Abnormal pulmonary thallium washout was related to both the anatomic extent and functional severity of disease: it occurred with greatest frequency in patients with multivessel disease and in those with exercise-induced left ventricular dysfunction (p less than 0.005). When added to the quantitative analysis of myocardial scintigraphy, the analysis of pulmonary thallium washout increased the detection of coronary artery disease from 84 to 93% (p less than 0.05), but the sample size was too small to assess specificity

  12. Copper Dependence of Angioproliferation in Pulmonary Arterial Hypertension in Rats and Humans

    Science.gov (United States)

    Mizuno, Shiro; Guignabert, Christophe; Al Hussaini, Aysar A.; Farkas, Daniela; Ruiter, Gerrina; Kraskauskas, Donatas; Fadel, Elie; Allegood, Jeremy C.; Humbert, Marc; Noordegraaf, Anton Vonk; Spiegel, Sarah; Farkas, Laszlo; Voelkel, Norbert F.

    2012-01-01

    Obliteration of the vascular lumen by endothelial cell growth is a hallmark of many forms of severe pulmonary arterial hypertension. Copper plays a significant role in the control of endothelial cell proliferation in cancer and wound-healing. We sought to determine whether angioproliferation in rats with experimental pulmonary arterial hypertension and pulmonary microvascular endothelial cell proliferation in humans depend on the proangiogenic action of copper. A copper-depleted diet prevented, and copper chelation with tetrathiomolybdate reversed, the development of severe experimental pulmonary arterial hypertension. The copper chelation–induced reopening of obliterated vessels was caused by caspase-independent apoptosis, reduced vessel wall cell proliferation, and a normalization of vessel wall structure. No evidence was found for a role of super oxide–1 inhibition or lysyl–oxidase–1 inhibition in the reversal of angioproliferation. Tetrathiomolybdate inhibited the proliferation of human pulmonary microvascular endothelial cells, isolated from explanted lungs from control subjects and patients with pulmonary arterial hypertension. These data suggest that the inhibition of endothelial cell proliferation by a copper-restricting strategy could be explored as a new therapeutic approach in pulmonary arterial hypertension. It remains to be determined, however, whether potential toxicity to the right ventricle is offset by the beneficial pulmonary vascular effects of antiangiogenic treatment in patients with pulmonary arterial hypertension. PMID:22162909

  13. Up-regulation of the mammalian target of rapamycin complex 1 subunit Raptor by aldosterone induces abnormal pulmonary artery smooth muscle cell survival patterns to promote pulmonary arterial hypertension

    Science.gov (United States)

    Aghamohammadzadeh, Reza; Zhang, Ying-Yi; Stephens, Thomas E.; Arons, Elena; Zaman, Paula; Polach, Kevin J.; Matar, Majed; Yung, Lai-Ming; Yu, Paul B.; Bowman, Frederick P.; Opotowsky, Alexander R.; Waxman, Aaron B.; Loscalzo, Joseph; Leopold, Jane A.; Maron, Bradley A.

    2016-01-01

    Activation of the mammalian target of rapamycin complex 1 (mTORC1) subunit Raptor induces cell growth and is a downstream target of Akt. Elevated levels of aldosterone activate Akt, and, in pulmonary arterial hypertension (PAH), correlate with pulmonary arteriole thickening, which suggests that mTORC1 regulation by aldosterone may mediate adverse pulmonary vascular remodeling. We hypothesized that aldosterone-Raptor signaling induces abnormal pulmonary artery smooth muscle cell (PASMC) survival patterns to promote PAH. Remodeled pulmonary arterioles from SU-5416/hypoxia-PAH rats and monocrotaline-PAH rats with hyperaldosteronism expressed increased levels of the Raptor target, p70S6K, which provided a basis for investigating aldosterone-Raptor signaling in human PASMCs. Aldosterone (10−9 to 10−7 M) increased Akt/mTOR/Raptor to activate p70S6K and increase proliferation, viability, and apoptosis resistance in PASMCs. In PASMCs transfected with Raptor–small interfering RNA or treated with spironolactone/eplerenone, aldosterone or pulmonary arterial plasma from patients with PAH failed to increase p70S6K activation or to induce cell survival in vitro. Optimal inhibition of pulmonary arteriole Raptor was achieved by treatment with Staramine-monomethoxy polyethylene glycol that was formulated with Raptor-small interfering RNA plus spironolactone in vivo, which decreased arteriole muscularization and pulmonary hypertension in 2 experimental animal models of PAH in vivo. Up-regulation of mTORC1 by aldosterone is a critical pathobiologic mechanism that controls PASMC survival to promote hypertrophic vascular remodeling and PAH.—Aghamohammadzadeh, R., Zhang, Y.-Y., Stephens, T. E., Arons, E., Zaman, P., Polach, K. J., Matar, M., Yung, L.-M., Yu, P. B., Bowman, F. P., Opotowsky, A. R., Waxman, A. B., Loscalzo, J., Leopold, J. A., Maron, B. A. Up-regulation of the mammalian target of rapamycin complex 1 subunit Raptor by aldosterone induces abnormal pulmonary artery

  14. Main pulmonary artery cross-section ratio is low in fetuses with tetralogy of Fallot and ductus arteriosus-dependent pulmonary circulation.

    Science.gov (United States)

    Ebishima, Hironori; Kurosaki, Kenichi; Yoshimatsu, Jun; Shiraishi, Isao

    2017-08-01

    This study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes. The Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio - the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation. We included two infants with pulmonary atresia and six infants with ductus-dependent pulmonary circulation, who underwent systemic-to-pulmonary shunt surgeries at ⩽1 month of age. The Z-scores of the main pulmonary artery and the pulmonary artery-to-aorta ratio in fetuses with ductus-dependent pulmonary circulation were lesser than those in fetuses with ductus independence, but not significantly. The main pulmonary artery cross-section ratio in fetuses with ductus dependence was significantly lesser (0.65±0.44 versus 1.56±0.48, ptetralogy of Fallot.

  15. Endothelial dysfunction in pulmonary arterial hypertension: an evolving landscape (2017 Grover Conference Series).

    Science.gov (United States)

    Ranchoux, Benoît; Harvey, Lloyd D; Ayon, Ramon J; Babicheva, Aleksandra; Bonnet, Sebastien; Chan, Stephen Y; Yuan, Jason X-J; Perez, Vinicio de Jesus

    2018-01-01

    Endothelial dysfunction is a major player in the development and progression of vascular pathology in pulmonary arterial hypertension (PAH), a disease associated with small vessel loss and obstructive vasculopathy that leads to increased pulmonary vascular resistance, subsequent right heart failure, and premature death. Over the past ten years, there has been tremendous progress in our understanding of pulmonary endothelial biology as it pertains to the genetic and molecular mechanisms that orchestrate the endothelial response to direct or indirect injury, and how their dysregulation can contribute to the pathogenesis of PAH. As one of the major topics included in the 2017 Grover Conference Series, discussion centered on recent developments in four areas of pulmonary endothelial biology: (1) angiogenesis; (2) endothelial-mesenchymal transition (EndMT); (3) epigenetics; and (4) biology of voltage-gated ion channels. The present review will summarize the content of these discussions and provide a perspective on the most promising aspects of endothelial dysfunction that may be amenable for therapeutic development.

  16. Computerized axial tomography of the chest for visualization of ''absent'' pulmonary arteries

    Energy Technology Data Exchange (ETDEWEB)

    Sondheimer, H.M. (Upstate Medical Center, Syracuse, NY); Oliphant, M.; Schneider, B.; Kavey, R.E.W.; Blackman, M.S.; Parker, F.B. Jr.

    1982-05-01

    To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with ''absent'' left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease.

  17. Automated 3D Volumetry of the Pulmonary Arteries based on Magnetic Resonance Angiography Has Potential for Predicting Pulmonary Hypertension.

    Directory of Open Access Journals (Sweden)

    Fabian Rengier

    Full Text Available To demonstrate feasibility of automated 3D volumetry of central pulmonary arteries based on magnetic resonance angiography (MRA, to assess pulmonary artery volumes in patients with pulmonary hypertension compared to healthy controls, and to investigate the potential of the technique for predicting pulmonary hypertension.MRA of pulmonary arteries was acquired at 1.5T in 20 patients with pulmonary arterial hypertension and 21 healthy normotensive controls. 3D model-based image analysis software was used for automated segmentation of main, right and left pulmonary arteries (MPA, RPA and LPA. Volumes indexed to vessel length and mean, minimum and maximum diameters along the entire vessel course were assessed and corrected for body surface area (BSA. For comparison, diameters were also manually measured on axial reconstructions and double oblique multiplanar reformations. Analyses were performed by two cardiovascular radiologists, and by one radiologist again after 6 months.Mean volumes of MPA, RPA and LPA for patients/controls were 5508 ± 1236/3438 ± 749, 3522 ± 934/1664 ± 468 and 3093 ± 692/1812 ± 474 μl/(cm length x m2 BSA (all p<0.001. Mean, minimum and maximum diameters along the entire vessel course were also significantly increased in patients compared to controls (all p<0.001. Intra- and interobserver agreement were excellent for both volume and diameter measurements using 3D segmentation (intraclass correlation coefficients 0.971-0.999, p<0.001. Area under the curve for predicting pulmonary hypertension using volume was 0.998 (95% confidence interval 0.990-1.0, p<0.001, compared to 0.967 using manually measured MPA diameter (95% confidence interval 0.910-1.0, p<0.001.Automated MRA-based 3D volumetry of central pulmonary arteries is feasible and demonstrated significantly increased volumes and diameters in patients with pulmonary arterial hypertension compared to healthy controls. Pulmonary artery volume may serve as a superior

  18. Automated 3D Volumetry of the Pulmonary Arteries based on Magnetic Resonance Angiography Has Potential for Predicting Pulmonary Hypertension.

    Science.gov (United States)

    Rengier, Fabian; Wörz, Stefan; Melzig, Claudius; Ley, Sebastian; Fink, Christian; Benjamin, Nicola; Partovi, Sasan; von Tengg-Kobligk, Hendrik; Rohr, Karl; Kauczor, Hans-Ulrich; Grünig, Ekkehard

    2016-01-01

    To demonstrate feasibility of automated 3D volumetry of central pulmonary arteries based on magnetic resonance angiography (MRA), to assess pulmonary artery volumes in patients with pulmonary hypertension compared to healthy controls, and to investigate the potential of the technique for predicting pulmonary hypertension. MRA of pulmonary arteries was acquired at 1.5T in 20 patients with pulmonary arterial hypertension and 21 healthy normotensive controls. 3D model-based image analysis software was used for automated segmentation of main, right and left pulmonary arteries (MPA, RPA and LPA). Volumes indexed to vessel length and mean, minimum and maximum diameters along the entire vessel course were assessed and corrected for body surface area (BSA). For comparison, diameters were also manually measured on axial reconstructions and double oblique multiplanar reformations. Analyses were performed by two cardiovascular radiologists, and by one radiologist again after 6 months. Mean volumes of MPA, RPA and LPA for patients/controls were 5508 ± 1236/3438 ± 749, 3522 ± 934/1664 ± 468 and 3093 ± 692/1812 ± 474 μl/(cm length x m2 BSA) (all p<0.001). Mean, minimum and maximum diameters along the entire vessel course were also significantly increased in patients compared to controls (all p<0.001). Intra- and interobserver agreement were excellent for both volume and diameter measurements using 3D segmentation (intraclass correlation coefficients 0.971-0.999, p<0.001). Area under the curve for predicting pulmonary hypertension using volume was 0.998 (95% confidence interval 0.990-1.0, p<0.001), compared to 0.967 using manually measured MPA diameter (95% confidence interval 0.910-1.0, p<0.001). Automated MRA-based 3D volumetry of central pulmonary arteries is feasible and demonstrated significantly increased volumes and diameters in patients with pulmonary arterial hypertension compared to healthy controls. Pulmonary artery volume may serve as a superior predictor for

  19. Isorhynchophylline protects against pulmonary arterial hypertension and suppresses PASMCs proliferation

    International Nuclear Information System (INIS)

    Guo, Haipeng; Zhang, Xin; Cui, Yuqian; Deng, Wei; Xu, Dachun; Han, Hui; Wang, Hao; Chen, Yuguo; Li, Yu; Wu, Dawei

    2014-01-01

    Highlights: • We focus on PASMCs proliferation in the pathogenesis of PAH. • Isorhynchophylline inhibited PASMCs proliferation and alleviated PAH. • IRN blocked PDGF-Rβ phosphorylation and its downstream signal transduction. • IRN regulated cyclins and CDKs to arrest cell cycle in the G0/G1 phase. • We reported IRN has the potential to be a candidate for PAH treatment. - Abstract: Increased pulmonary arterial smooth muscle cells (PASMCs) proliferation is a key pathophysiological component of pulmonary vascular remodeling in pulmonary arterial hypertension (PAH). Isorhynchophylline (IRN) is a tetracyclic oxindole alkaloid isolated from the Chinese herbal medicine Uncaria rhynchophylla. It has long been used clinically for treatment of cardiovascular and cerebrovascular diseases. However, very little is known about whether IRN can influence the development of PAH. Here we examined the effect of IRN on monocrotaline (MCT) induced PAH in rats. Our data demonstrated that IRN prevented MCT induced PAH in rats, as assessed by right ventricular (RV) pressure, the weight ratio of RV to (left ventricular + septum) and RV hypertrophy. IRN significantly attenuated the percentage of fully muscularized small arterioles, the medial wall thickness, and the expression of smooth muscle α-actin (α-SMA) and proliferating cell nuclear antigen (PCNA). In vitro studies, IRN concentration-dependently inhibited the platelet-derived growth factor (PDGF)-BB-induced proliferation of PASMCs. Fluorescence-activated cell-sorting analysis showed that IRN caused G0/G1 phase cell cycle arrest. IRN-induced growth inhibition was associated with downregulation of Cyclin D1 and CDK6 as well as an increase in p27Kip1 levels in PDGF-BB-stimulated PASMCs. Moreover, IRN negatively modulated PDGF-BB-induced phosphorylation of PDGF-Rβ, ERK1/2, Akt/GSK3β, and signal transducers and activators of transcription 3 (STAT3). These results demonstrate that IRN could inhibit PASMCs proliferation and

  20. Isorhynchophylline protects against pulmonary arterial hypertension and suppresses PASMCs proliferation

    Energy Technology Data Exchange (ETDEWEB)

    Guo, Haipeng; Zhang, Xin [Department of Critical Care Medicine, Qilu Hospital of Shandong University, Jinan 250012 (China); Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China); Cui, Yuqian [Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China); Deng, Wei [Department of Cardiology, Renmin Hospital of Wuhan University, Wuhan 430060 (China); Xu, Dachun [Department of Cardiology, Shanghai Tenth People’s Hospital of Tongji University, Shanghai 200072 (China); Han, Hui; Wang, Hao [Department of Critical Care Medicine, Qilu Hospital of Shandong University, Jinan 250012 (China); Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China); Chen, Yuguo [Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China); Li, Yu, E-mail: qlliyu@126.com [Department of Respiratory, Qilu Hospital of Shandong University, Jinan 250012 (China); Wu, Dawei, E-mail: wdwu55@163.com [Department of Critical Care Medicine, Qilu Hospital of Shandong University, Jinan 250012 (China); Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China)

    2014-07-18

    Highlights: • We focus on PASMCs proliferation in the pathogenesis of PAH. • Isorhynchophylline inhibited PASMCs proliferation and alleviated PAH. • IRN blocked PDGF-Rβ phosphorylation and its downstream signal transduction. • IRN regulated cyclins and CDKs to arrest cell cycle in the G0/G1 phase. • We reported IRN has the potential to be a candidate for PAH treatment. - Abstract: Increased pulmonary arterial smooth muscle cells (PASMCs) proliferation is a key pathophysiological component of pulmonary vascular remodeling in pulmonary arterial hypertension (PAH). Isorhynchophylline (IRN) is a tetracyclic oxindole alkaloid isolated from the Chinese herbal medicine Uncaria rhynchophylla. It has long been used clinically for treatment of cardiovascular and cerebrovascular diseases. However, very little is known about whether IRN can influence the development of PAH. Here we examined the effect of IRN on monocrotaline (MCT) induced PAH in rats. Our data demonstrated that IRN prevented MCT induced PAH in rats, as assessed by right ventricular (RV) pressure, the weight ratio of RV to (left ventricular + septum) and RV hypertrophy. IRN significantly attenuated the percentage of fully muscularized small arterioles, the medial wall thickness, and the expression of smooth muscle α-actin (α-SMA) and proliferating cell nuclear antigen (PCNA). In vitro studies, IRN concentration-dependently inhibited the platelet-derived growth factor (PDGF)-BB-induced proliferation of PASMCs. Fluorescence-activated cell-sorting analysis showed that IRN caused G0/G1 phase cell cycle arrest. IRN-induced growth inhibition was associated with downregulation of Cyclin D1 and CDK6 as well as an increase in p27Kip1 levels in PDGF-BB-stimulated PASMCs. Moreover, IRN negatively modulated PDGF-BB-induced phosphorylation of PDGF-Rβ, ERK1/2, Akt/GSK3β, and signal transducers and activators of transcription 3 (STAT3). These results demonstrate that IRN could inhibit PASMCs proliferation and

  1. Pulmonary Artery Endothelial Cell Phenotypic Alterations in a Large Animal Model of Pulmonary Arteriovenous Malformations Following the Glenn Shunt

    Science.gov (United States)

    Kavarana, Minoo N.; Mukherjee, Rupak; Eckhouse, Shaina R.; Rawls, William F.; Logdon, Christina; Stroud, Robert E.; Patel, Risha K.; Nadeau, Elizabeth K.; Spinale, Francis G.; Graham, Eric M.; Forbus, Geoffrey A.; Bradley, Scott M.; Ikonomidis, John S.; Jones, Jeffrey A.

    2014-01-01

    Background: Longevity of the superior cavopulmonary connection (SCPC) is limited by the development of pulmonary arteriovenous malformations (PAVM). The goal of this study was to determine whether phenotypic changes in pulmonary artery endothelial cells (PAEC) that favor angiogenesis occur with PAVM formation. Methods: A superior vena cava to right pulmonary artery connection was constructed in 5 pigs. Pulmonary arteries were harvested at 6-8 weeks following surgery to establish cultures of PAEC and smooth muscle cells, to determine cell proliferation, gene expression, and tubule formation. Abundance of proteins related to angiogenesis was measured in lung tissue. Results: Contrast echocardiography revealed right-to-left shunting, consistent with PAVM formation. While the proliferation of smooth muscle cells from the right pulmonary artery (RPA) (shunted side) and left pulmonary artery (LPA) (non- shunted side) were similar, right PAEC proliferation was significantly higher. Expression profiles of genes encoding cellular signaling proteins were higher in PAECs from the RPA vs. LPA. Protein abundance of angiopoietin-1, and Tie-2 (angiopoietin receptor) were increased in the right lung (both pSCPC. This study found that PAVM development occurred concomitantly with differential changes in PAEC proliferative ability and phenotype. Moreover, there was a significant increase in the angiopoietin/Tie-2 complex in the right lung, which may provide novel therapeutic targets to attenuate PAVM formation following a SCPC. PMID:23968766

  2. Pulmonary Arterial Hypertension in Glycogen Storage Disease Type I

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    Rachel D. Torok MD

    2017-05-01

    Full Text Available Pulmonary arterial hypertension (PAH is a rare and highly fatal disease that has been reported in 8 patients with glycogen storage disease type I (GSDI. We describe an additional case of an acute presentation of PAH in a 14-year-old patient with GSDI, which was successfully treated with inhaled nitric oxide and sildenafil. We investigated the incidence of PAH in 28 patients with GSDI on routine echocardiography and found no evidence of PAH and no significant cardiac abnormalities. This study highlights that PAH is a rare disease overall, but our case report and those previously described suggest an increased incidence in patients with GSDI. Should cardiopulmonary symptoms develop, clinicians caring for patients with GSDI should have a high degree of suspicion for acute PAH and recognize that prompt intervention can lead to survival in this otherwise highly fatal disease.

  3. Mazindol: a risk factor for pulmonary arterial hypertension?

    Science.gov (United States)

    Konofal, Eric; Benzouid, Cherine; Delclaux, Christophe; Lecendreux, Michel; Hussey, Elizabeth

    2017-06-01

    Mazindol is an imidazo-isoindole derivative, a tricyclic compound and a non-amphetamine central nervous system stimulant that blocks dopamine and norepinephrine reuptake. Mazindol was withdrawn from the US and European markets in 1999 for reasons unrelated to its efficacy or safety around a time when other anorexic drugs were found to be associated with the development of pulmonary arterial hypertension (PAH). Despite the use of mazindol for decades, reports of PAH due to mazindol intake have been extremely rare. Recent interest on mazindol has emerged for the treatment of narcolepsy and attention-deficit/hyperactivity disorder. Therefore, an updated understanding of the potential benefits and risks of mazindol in these patient populations is warranted. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. Selexipag in the treatment of pulmonary arterial hypertension: design, development, and therapy

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    Hardin EA

    2016-11-01

    Full Text Available Elizabeth Ashley Hardin,1 Kelly M Chin2 1Department of Internal Medicine, Division of Cardiology, 2Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA Abstract: Pulmonary arterial hypertension is characterized by abnormalities in the small pulmonary arteries including increased vasoconstriction, vascular remodeling, proliferation of smooth muscle cells, and in situ thrombosis. Selexipag, a novel, oral prostacyclin receptor agonist, has been shown to improve hemodynamics in a phase II clinical trial and reduce clinical worsening in a large phase III clinical trial involving patients with pulmonary arterial hypertension. In this paper, we describe the prostacyclin signaling pathway, currently available oral prostanoid medications, and the development and clinical use of selexipag. Keywords: selexipag, pulmonary arterial hypertension, prostacyclin

  5. CCR5 as a treatment target in pulmonary arterial hypertension.

    Science.gov (United States)

    Amsellem, Valérie; Lipskaia, Larissa; Abid, Shariq; Poupel, Lucie; Houssaini, Amal; Quarck, Rozenn; Marcos, Elisabeth; Mouraret, Nathalie; Parpaleix, Aurélien; Bobe, Régis; Gary-Bobo, Guillaume; Saker, Mirna; Dubois-Randé, Jean-Luc; Gladwin, Mark T; Norris, Karen A; Delcroix, Marion; Combadière, Christophe; Adnot, Serge

    2014-09-09

    Pulmonary arterial hypertension (PH), whether idiopathic or related to underlying diseases such as HIV infection, results from complex vessel remodeling involving both pulmonary artery smooth muscle cell (PA-SMC) proliferation and inflammation. CCR5, a coreceptor for cellular HIV-1 entry expressed on macrophages and vascular cells, may be involved in the pathogenesis of PH. Maraviroc is a new CCR5 antagonist designed to block HIV entry. Marked CCR5 expression was found in lungs from patients with idiopathic PH, in mice with hypoxia-induced PH, and in Simian immunodeficiency virus-infected macaques, in which it was localized chiefly in the PA-SMCs. To assess the role for CCR5 in experimental PH, we used both gene disruption and pharmacological CCR5 inactivation in mice. Because maraviroc does not bind to murine CCR5, we used human-CCR5ki mice for pharmacological and immunohistochemical studies. Compared with wild-type mice, CCR5-/- mice or human-CCR5ki mice treated with maraviroc exhibited decreased PA-SMC proliferation and recruitment of perivascular and alveolar macrophages during hypoxia exposure. CCR5-/- mice reconstituted with wild-type bone marrow cells and wild-type mice reconstituted with CCR5-/- bone marrow cells were protected against PH, suggesting CCR5-mediated effects on PA-SMCs and macrophage involvement. The CCR5 ligands CCL5 and the HIV-1 gp120 protein increased intracellular calcium and induced growth of human and human-CCR5ki mouse PA-SMCs; maraviroc inhibited both effects. Maraviroc also reduced the growth-promoting effects of conditioned media from CCL5-activated macrophages derived from human-CCR5ki mice on PA-SMCs from wild-type mice. The CCL5-CCR5 pathway represents a new therapeutic target in PH associated with HIV or with other conditions. © 2014 American Heart Association, Inc.

  6. Screening and management of pulmonary arterial hypertension in systemic sclerosis

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    Vivek Nagaraja

    2017-01-01

    Full Text Available Systemic sclerosis-associated pulmonary hypertension (SSc-PH and pulmonary arterial (PA hypertension (SSc-PAH are well-recognized manifestations. SSc-PH is a hemodynamic observation, and it is important to identify underlying etiology. SSc patients commonly have mixed etiology for SSc-PH due to interstitial lung disease, PAH, and left heart disease. SSc-PAH is associated with high morbidity and mortality. Early detection of PAH through routine screening improves survival in patients with SSc. Right heart catheterization is mandatory to diagnose PAH. SSc-PAH patients should be managed by a multidisciplinary team comprising of rheumatologist, pulmonologist, cardiologist, and physiotherapist. Various pharmacotherapy options to treat SSc-PAH are derived from the idiopathic PAH management. Upfront or sequential combination therapy of PAH-specific drugs seems to confer a clinical benefit compared to monotherapy. Cardiopulmonary rehabilitation should be considered as a part of the management plan. Lung transplantation is a consideration in patients who are not responding to pharmacotherapy. Although the long-term prognosis of SSc-PAH has been historically poor, the landscape is gradually changing with early detection and institution of treatment.

  7. Macitentan for the treatment of pulmonary arterial hypertension.

    Science.gov (United States)

    Hong, Irene S; Coe, Holly V; Catanzaro, Linda M

    2014-04-01

    To review the pharmacology, safety, and efficacy of macitentan. PubMed, EMBASE, and ClinicalTrials.gov were searched using the terms macitentan and ACT-064992. Phase II and III trials were reviewed in our primary analysis; data from phase I trials and other studies were reported as applicable. Macitentan is a dual endothelin receptor antagonist (ERA) approved for the treatment of pulmonary arterial hypertension (PAH). Current treatment options for PAH include 2 other ERAs, phosphodiesterase type 5 inhibitors, prostanoids, and calcium channel blockers. Recently published guidelines do not assert a preference for individual agents. Two trials evaluated the safety and efficacy of macitentan. The phase II study was a 12-month placebo-controlled trial involving patients with idiopathic pulmonary fibrosis; the primary end point was change in forced vital capacity. No significant treatment effect was observed. The phase III study was a placebo-controlled trial involving patients with PAH. The primary end point was time to first occurrence of a composite of outcomes, including all-cause death and PAH worsening. Over a median period of 115 weeks, macitentan 10 mg and 3 mg daily significantly reduced morbidity and mortality. Commonly reported adverse effects included worsening of PAH, peripheral edema, upper-respiratory-tract infection, and anemia. Macitentan represents the latest addition to the PAH armamentarium. Compared with other ERAs, clinical advantages may include fewer contraindications, use in hepatic impairment, and once-daily administration. However, further comparative studies are necessary to ascertain its place in therapy.

  8. 53. Bilateral ductal stenting for nonconfluent pulmonary arteries in a newborn

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    K. Al Dhahri

    2016-07-01

    Full Text Available Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Limited data available for bilateral PDA stenting. Bilateral PDA stenting in nonconfluent pulmonary arteries is challenging procedure but can be considered as an option in the management of complex conditions like this. 12 days old Preterm (36 weeks gestation male baby with birth weight of 2.6 kg developed respiratory distress with severe cyanosis and desaturation upto 50%. Baby was intubated and started on Prostaglandin 0.05 mic/kg/mt. His saturation improved to 80%. Echocardiogram showed complex cyanotic heart disease, Situs ambiguous, dextrocardia, complete unbalanced AV septal defect, pulmonary atresia , nonconfluent small branch pulmonary arteries supplied by the bilateral patent ductus arteriosus (PDA from right aortic arch and all four pulmonary veins form a confluence and drain into superior vena cava(SVC through vertical vein with no obstruction. Baby was taken up for PDA stenting. descending aortogram showed right aortic arch with vertical tortuous duct to right pulmonary artery (RPA and another short duct with acute angle from left subclavian artery to left pulmonary artery (LPA . Both ducti stented with coronary stents. Vertical vein angiogram showed both lungs drain to a confluence and then to SVC via ascending vertical vein with no obstruction. After stenting lung perfusion improved and the baby was stable and maintained 80% saturation on room air. Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Our case is unique with Heterotaxy, TAPVC, Dextrocardia and double ducti. Eventhough bilateral ductal stenting is technically challenging it is successful through femoral artery approach.

  9. Gadomer-17-enhanced 3D navigator-echo MR angiography of the pulmonary arteries in pigs

    Energy Technology Data Exchange (ETDEWEB)

    Abolmaali, Nasreddin D.; Vogl, Thomas J. [Institute for Diagnostic and Interventional Radiology, Johann Wolfgang Goethe University, Frankfurt am Main (Germany); Hietschold, Volker [Institute and City Hospital for Radiological Diagnostics, Carl Gustav Carus University, Dresden (Germany); Appold, Steffen [Clinic and City Hopsital for Radiotherapy and Radiological Oncology, Carl Gustav Carus University, Dresden (Germany); Ebert, Wolfgang [Research Laboratories of Schering AG, Berlin (Germany)

    2002-03-01

    The goal of this study was visualisation and quality assessment of the pulmonary arteries in pigs with modified navigator-echo magnetic resonance angiography using an intravascular contrast agent. Five sedated pigs were examined in a clinical 1.5-T system with modified three-dimensional navigator-echo magnetic resonance angiography (slice thickness 3 mm, pixel size 2.4 x 1.8 mm{sup 2}) to evaluate the pulmonary arteries. Using a phased-array four-element thorax coil the entire thorax was scanned before and after intravenous infusion of a gadolinium-based intravascular contrast agent. Assessment of image quality, enhancement-related contrast-to-noise ratio (CNR) measurements and improvement of visibility of peripheral pulmonary vessels was performed. Improvement of quality using Gadomer-17 was found for smaller vessels; pulmonary trunks and the main pulmonary arteries were sufficiently imaged without enhancement. Mean rise of CNR measured in the pulmonary trunks was 28.64% (P=0.0002), mean rise of CNR in the main pulmonary arteries and the segmental arteries were 79.6% and 148.2%, respectively. Mean distance between the visible peripheral end of 60 sub-segmental arteries and the inner thoracic wall was 12.2{+-}0.4 mm, and was significantly (P=0.00002) reduced after contrast infusion to 8.0{+-}0.4 mm. The combination of inherent flow sensitivity of navigator-echo angiography and Gadomer-17 proved effective for imaging of the pulmonary arteries. In contrast to standard contrast-enhanced pulmonary MRA studies, breath holding is not required. Further studies and the evaluation of findings of patients suffering from pulmonary embolism are needed to evaluate the possible benefits of a higher spatial resolution which is achievable using navigator-echo techniques in contrast to the higher temporal resolution of ultra-fast pulmonary MRA. (orig.)

  10. Danshensu prevents hypoxic pulmonary hypertension in rats by inhibiting the proliferation of pulmonary artery smooth muscle cells via TGF-β-smad3-associated pathway.

    Science.gov (United States)

    Zhang, Ning; Dong, Mingqing; Luo, Ying; Zhao, Feng; Li, Yongjun

    2018-02-05

    Hypoxic pulmonary hypertension is characterized by the remodeling of pulmonary artery. Previously we showed that tanshinone IIA, one lipid-soluble component from the Chinese herb Danshen, ameliorated hypoxic pulmonary hypertension by inhibiting pulmonary artery remodeling. Here we explored the effects of danshensu, one water-soluble component of Danshen, on hypoxic pulmonary hypertension and its mechanism. Rats were exposed to hypobaric hypoxia for 4 weeks to develop hypoxic pulmonary hypertension along with administration of danshensu. Hemodynamics and pulmonary arterial remodeling index were measured. The effects of danshensu on the proliferation of primary pulmonary artery smooth muscle cells and transforming growth factor-β-smad3 pathway were assessed in vitro. Danshensu significantly decreased the right ventricle systolic pressure, the right ventricle hypertrophy and pulmonary vascular remodeling index in hypoxic pulmonary hypertension rats. Danshensu also reduced the increased expression of transforming growth factor-β and phosphorylation of smad3 in pulmonary arteries in hypoxic pulmonary hypertension rats. In vitro, danshensu inhibited the hypoxia- or transforming growth factor-β-induced proliferation of primary pulmonary artery smooth muscle cells. Moreover, danshensu decreased the hypoxia-induced expression and secretion of transforming growth factor in primary pulmonary adventitial fibroblasts and NR8383 cell line, inhibited the hypoxia or transforming growth factor-β-induced phosphorylation of smad3 in rat primary pulmonary artery smooth muscle cells. These results demonstrate that danshensu ameliorates hypoxic pulmonary hypertension in rats by inhibiting the hypoxia-induced proliferation of pulmonary artery smooth muscle cells, and the inhibition effects is associated with transforming growth factor-β-smad3 pathway. Therefore danshensu may be a potential treatment for hypoxic pulmonary hypertension. Copyright © 2017 Elsevier B.V. All rights

  11. Effects of cigarette smoke on endothelial function of pulmonary arteries in the guinea pig

    Directory of Open Access Journals (Sweden)

    Martínez Anna

    2009-08-01

    Full Text Available Abstract Background Cigarette smoking may contribute to pulmonary hypertension in chronic obstructive pulmonary disease by altering the structure and function of pulmonary vessels at early disease stages. The objectives of this study were to evaluate the effects of long-term exposure to cigarette smoke on endothelial function and smooth muscle-cell proliferation in pulmonary arteries of guinea pigs. Methods 19 male Hartley guinea pigs were exposed to the smoke of 7 cigarettes/day, 5 days/week, for 3 and 6 months. 17 control guinea pigs were sham-exposed for the same periods. Endothelial function was evaluated in rings of pulmonary artery and aorta as the relaxation induced by ADP. The proliferation of smooth muscle cells and their phenotype in small pulmonary vessels were evaluated by immunohistochemical expression of α-actin and desmin. Vessel wall thickness, arteriolar muscularization and emphysema were assessed morphometrically. The expression of endothelial nitric oxide synthase (eNOS was evaluated by Real Time-PCR. Results Exposure to cigarette smoke reduced endothelium-dependent vasodilatation in pulmonary arteries (ANOVA p Conclusion In the guinea pig, exposure to cigarette smoke induces selective endothelial dysfunction in pulmonary arteries, smooth muscle cell proliferation in small pulmonary vessels and reduced lung expression of eNOS. These changes appear after 3 months of exposure and precede the development of pulmonary emphysema.

  12. Peripheral pulmonary arteries: identification at multi-slice spiral CT with 3D reconstruction

    International Nuclear Information System (INIS)

    Coche, Emmanuel; Pawlak, Sebastien; Dechambre, Stephane; Maldague, Baudouin

    2003-01-01

    Our objective was to analyze the peripheral pulmonary arteries using thin-collimation multi-slice spiral CT. Twenty consecutive patients underwent enhanced-spiral multi-slice CT using 1-mm collimation. Two observers analyzed the pulmonary arteries by consensus on a workstation. Each artery was identified on axial and 3D shaded-surface display reconstruction images. Each subsegmental artery was measured at a mediastinal window setting and compared with anatomical classifications. The location and branching of every subsegmental artery was recorded. The number of well-visualized sub-subsegmental arteries at a mediastinal window setting was compared with those visualized at a lung window setting. Of 800 subsegmental arteries, 769 (96%) were correctly visualized and 123 accessory subsegmental arteries were identified using the mediastinal window setting. One thousand ninety-two of 2019 sub-subsegmental arteries (54%) identified using the lung window setting were correctly visualized using the mediastinal window setting. Enhanced multi-slice spiral CT with thin collimation can be used to analyze precisely the subsegmental pulmonary arteries and may identify even more distal pulmonary arteries. (orig.)

  13. Reactive Oxygen Species-Reducing Strategies Improve Pulmonary Arterial Responses to Nitric Oxide in Piglets with Chronic Hypoxia-Induced Pulmonary Hypertension

    Science.gov (United States)

    Dikalova, Anna; Slaughter, James C.; Kaplowitz, M.R.; Zhang, Y.; Aschner, Judy L.

    2013-01-01

    Abstract Aims: There are no effective treatments for chronic pulmonary hypertension in infants with cardiopulmonary disorders associated with hypoxia, such as those with chronic lung disease. These patients often have poor or inconsistent pulmonary dilator responses to inhaled nitric oxide (iNO) therapy for unknown reasons. One possible explanation for poor responsiveness to iNO is reduced NO bioavailability caused by interactions between reactive oxygen species (ROS) and NO. Our major aim was to determine if strategies to reduce ROS improve dilator responses to the NO donor, S-nitroso-N-acetyl-penicillamine (SNAP), in resistance pulmonary arteries (PRAs) from a newborn piglet model of chronic pulmonary hypertension. Results: The dilation to SNAP was significantly impaired in PRAs from piglets with chronic hypoxia-induced pulmonary hypertension. ROS scavengers, including cell-permeable and impermeable agents to degrade hydrogen peroxide (H2O2), improved dilation to SNAP in PRAs from chronically hypoxic piglets. Treatment with agents to inhibit nitric oxide synthase and NADPH oxidase, potential enzymatic sources of ROS, also improved dilation to SNAP in PRAs from hypoxic piglets. Innovation: Our studies are the first to utilize a newborn model of chronic pulmonary hypertension to evaluate the impact of a number of potential therapeutic strategies for ROS removal on responses to exogenous NO in the vessels most relevant to the regulation of pulmonary vascular resistance (PRA). Conclusions: Strategies aimed at reducing ROS merit further evaluation and consideration as therapeutic approaches to improve responses to iNO in infants with chronic pulmonary hypertension. Antioxid. Redox Signal. 18, 1727–1738. PMID:23244497

  14. Pulmonary artery enlargement and cystic fibrosis pulmonary exacerbations: a cohort study

    Science.gov (United States)

    Wells, J. Michael; Farris, Roopan F.; Gosdin, Taylor A.; Dransfield, Mark T.; Wood, Michelle E.; Bell, Scott C.; Rowe, Steven M.

    2017-01-01

    Background Acute pulmonary exacerbations are associated with progressive lung function decline and increased mortality in cystic fibrosis (CF). The role of pulmonary vascular disease in pulmonary exacerbations is unknown. We investigated the association between pulmonary artery enlargement (PA:A>1), a marker of pulmonary vascular disease, and exacerbations. Methods We analyzed clinical, computed tomography (CT), and prospective exacerbation data in a derivation cohort of 74 adult CF patients, measuring the PA:A at the level of the PA bifurcation. We then replicated our findings in a validation cohort of 190 adult CF patients. Patients were separated into groups based on the presence or absence of a PA:A>1 and were followed for 1-year in the derivation cohort and 2-years in the validation cohort. The primary endpoint was developing ≥1 acute pulmonary exacerbation during follow-up. Linear and logistic regression models were used to determine associations between clinical factors, the PA:A ratio, and pulmonary exacerbations. We used Cox regression to determine time to first exacerbation in the validation cohort. Findings We found that PA:A>1 was present in n=37/74 (50%) of the derivation and n=89/190 (47%) of the validation cohort. In the derivation cohort, n=50/74 (68%) had ≥1 exacerbation at 1 year and n=133/190 (70%) in the validation cohort had ≥1 exacerbation after 2 years. PA:A>1 was associated with younger age in both cohorts and with elevated sweat chloride (100.5±10.9 versus 90.4±19.9mmol/L, difference between groups 10.1mmol/L [95%CI 2.5–17.7], P=0.017) in the derivation group. PA:A>1 was associated with exacerbations in the derivation (OR 3.49, 95%CI 1.18–10.3, P=0.023) and validation (OR 2.41, 95%CI 1.06–5.52, P=0.037) cohorts when adjusted for confounders. Time to first exacerbation was shorter in PA:A>1 versus PA:Apulmonary exacerbation risk in two well-characterized cohorts. PA:A may be a predictive marker in CF. PMID:27298019

  15. Isolated pulmonary veno-occlusive disease and pulmonary arterial thrombosis in systemic sclerosis – a lethal combination

    Directory of Open Access Journals (Sweden)

    Arun Jeevagan

    2010-05-01

    Full Text Available Arun JeevaganGeneral Medicine, Ipswich NHS Hospital, UKBackground: Isolated pulmonary hypertension secondary to systemic sclerosis is not uncommon. Our patient with systemic sclerosis presented with a very aggressive form of pulmonary hypertension due to a lethal combination of pulmonary veno-occlusive disease (PVOD and pulmonary arterial thrombosis. This combined presentation has never before been reported in medical literature.Case report: A 75-year-old woman with a 4-month history of atypical chest pains was admitted with a 3-week history of worsening symptoms of shortness of breath, reduced exercise tolerance, and bilateral pitting edema. On examination she had thickened skin in her hands, telangiectasia on her face, maculopapular rash in her legs, raised jugular venous pressure, and bilateral pitting edema. Her autoimmune profile revealed positive anticentromere antibodies, and her echocardiogram showed right ventricular systolic pressure of 91 mmHg. She also had renal impairment secondary to hypoperfusion. A diagnosis of isolated pulmonary hypertension secondary to limited systemic sclerosis was made. As she was clinically improving on slow diuretic infusion and awaiting transfer to a specialist center for management of pulmonary hypertension, our patient died due to cardiopulmonary arrest. Her postmortem revealed that she died of a combination of PVOD and pulmonary arteriopathy due to thrombosis.Conclusion: This is clearly a unique case both in presentation and difficulty of management. Pulmonary vasodilators used in therapy of pulmonary arteriopathy can be detrimental in patients with PVOD. There is no definitive investigation, curative treatment, or management, that exists for a combination of PVOD and pulmonary arteriopathy due to thrombosis secondary to systemic sclerosis.Keywords: pulmonary veno-occlusive disease, pulmonary arterial hypertension, systemic sclerosis, pulmonary arteriopathy with thrombosis

  16. Right Heart End-Systolic Remodeling Index Strongly Predicts Outcomes in Pulmonary Arterial Hypertension: Comparison With Validated Models.

    Science.gov (United States)

    Amsallem, Myriam; Sweatt, Andrew J; Aymami, Marie C; Kuznetsova, Tatiana; Selej, Mona; Lu, HongQuan; Mercier, Olaf; Fadel, Elie; Schnittger, Ingela; McConnell, Michael V; Rabinovitch, Marlene; Zamanian, Roham T; Haddad, Francois

    2017-06-01

    Right ventricular (RV) end-systolic dimensions provide information on both size and function. We investigated whether an internally scaled index of end-systolic dimension is incremental to well-validated prognostic scores in pulmonary arterial hypertension. From 2005 to 2014, 228 patients with pulmonary arterial hypertension were prospectively enrolled. RV end-systolic remodeling index (RVESRI) was defined by lateral length divided by septal height. The incremental values of RV free wall longitudinal strain and RVESRI to risk scores were determined. Mean age was 49±14 years, 78% were female, 33% had connective tissue disease, 52% were in New York Heart Association class ≥III, and mean pulmonary vascular resistance was 11.2±6.4 WU. RVESRI and right atrial area were strongly connected to the other right heart metrics. Three zones of adaptation (adapted, maladapted, and severely maladapted) were identified based on the RVESRI to RV systolic pressure relationship. During a mean follow-up of 3.9±2.4 years, the primary end point of death, transplant, or admission for heart failure was reached in 88 patients. RVESRI was incremental to risk prediction scores in pulmonary arterial hypertension, including the Registry to Evaluate Early and Long-Term PAH Disease Management score, the Pulmonary Hypertension Connection equation, and the Mayo Clinic model. Using multivariable analysis, New York Heart Association class III/IV, RVESRI, and log NT-proBNP (N-Terminal Pro-B-Type Natriuretic Peptide) were retained (χ 2 , 62.2; P right heart metrics, RVESRI demonstrated the best test-retest characteristics. RVESRI is a simple reproducible prognostic marker in patients with pulmonary arterial hypertension. © 2017 American Heart Association, Inc.

  17. Effects of endogenous carbon monoxide on collagen synthesis in pulmonary artery in rats under hypoxia.

    Science.gov (United States)

    Gong, Li-min; Du, Jun-bao; Shi, Lin; Shi, Yun; Tang, Chao-shu

    2004-01-23

    To study the role of endogenous carbon monoxide (CO) in collagen metabolism during hypoxic pulmonary vascular remodeling, a total of 18 Wistar rats were used in the study and they were randomly divided into three groups: hypoxia group (n = 6), hypoxia with zinc protoporphyrin-IX (ZnPP-IX) group (n = 6) and control group (n = 6). The measurement of mean pulmonary artery pressure (mPAP) and carboxyhemoglobin (HbCO) formation in lung tissue homogenates was measured. A morphometric analysis of pulmonary vessels was performed, in which the percentage of muscularized arteries (MA); partially muscularized arteries (PMA) and nonmuscularized arteries (NMV) in small and median pulmonary vessels, relative medial thickness (RMT) and relative medial area (RMA) of pulmonary arteries were analyzed. Collagen type I and III and transforming growth factor-beta3 (TGF-beta3) expressions were detected by immunohistochemical assay. The expressions of procollagen type I and III and TGF-beta3 mRNA were detected by in situ hybridization. The results showed that ZnPP-IX significantly increased mPAP and markedly decreased HbCO formation in lung tissue homogenates in rats under hypoxia (P ZnPP-IX, the percentage of muscularized arteries of small and median pulmonary vessels was obviously increased, and RMT and RMA of intra-acinar muscularized pulmonary arteries were markedly increased compared with hypoxic rats. Ultrastructural changes, such as hyperplasia and hypertrophy of endothelial cells (ECs) and smooth muscle cells (SMCs) and the increased number of SMCs in synthetic phenotype were found in intra-acinar pulmonary muscularized arteries of hypoxic rats treated with ZnPP-IX. Meanwhile, ZnPP-IX promoted the expression of collagen type I and III and TGF-beta3 protein in pulmonary arteries of rats under hypoxia (P ZnPP-IX elevated obviously the expressions of procollagen type I and III mRNA, and TGF-beta3 mRNA in pulmonary arteries of rats under hypoxia (P ZnPP-IX played an important role in

  18. Quantitative determination of pulmonary artery flow by scintiscanning in patients with mitral stenosis

    International Nuclear Information System (INIS)

    Carvalho, N.; Fujioka, T.; Dias Neto, A.; Papaleo Netto, M.

    1974-01-01

    In twenty-two patients with pure or predominant mitral stenosis, with pulmonary hypertension, the quantitative blood flow of the pulmonary artery systems through the distribution of macroaggregated radio-iodinated ( 131 I) albumin is studied, by pulmonary digital scanning. Through the relations ship between the radioactive concentration in each organ, it is possible to classify three types of images: normal, balanced and inverted. This technique is useful for quantitative analysis of pulmonary artery flow, as a test that should preced cardiac catheterization and in the follow-up of the surgical results [pt

  19. Pulmonary Artery Occlusion and Mediastinal Fibrosis in a Patient on Dopamine Agonist Treatment for Hyperprolactinemia

    DEFF Research Database (Denmark)

    Su, Junjing; Simonsen, Ulf; Carlsen, Jørn

    2017-01-01

    was treated with cabergoline and bromocriptine for hyperprolactinemia, presented with progressive dyspnea over several months. Based on the clinical investigation results, in particular, elevated pulmonary arterial pressures and significant perfusion defects on computed tomography (CT) pulmonary angiography...... and ventilation/perfusion (V/Q) scintigraphy, chronic thromboembolic pulmonary hypertension (CTEPH) was initially considered the most plausible diagnosis. However, during an attempted pulmonary endarterectomy, loose fibrous tissues were observed in the mediastinum and cryosection of the right pulmonary artery...... in the pathogenesis of pulmonary hypertension. In conclusion, as CT and V/Q scintigraphy findings can occasionally be deceptive, physicians should be particularly aware of differential diagnoses in patients without obvious history of venous thromboembolism that are suspected of having chronic thromboembolic pulmonary...

  20. STARS knockout attenuates hypoxia-induced pulmonary arterial hypertension by suppressing pulmonary arterial smooth muscle cell proliferation.

    Science.gov (United States)

    Shi, Zhaoling; Wu, Huajie; Luo, Jianfeng; Sun, Xin

    2017-03-01

    STARS (STriated muscle Activator of Rho Signaling) is a sarcomeric protein, which expressed early in cardiac development and involved in pathological remodeling. Abundant evidence indicated that STARS could regulate cell proliferation, but it's exact function remains unclear. In this study, we aimed to investigate the role of STARS in the proliferation of pulmonary arterial smooth muscle cells (PASMC) and the potential effect on the progression of pulmonary arterial hypertension (PAH). In this study, we established a PAH mouse model through chronic hypoxia exposure as reflected by the increased RVSP and RVHI. Western blot and RT-qPCR detected the increased STARS protein and mRNA levels in PAH mice. Next, we cultured the primary PASMC from PAH mice. After STARS overexpression in PASMC, STARS, SRF and Egr-1 were up-regulated significantly. The MTT assay revealed an increase in cell proliferation. Flow cytometry showed a marked inhibition of cell apoptosis. However, STARS silence in PASMC exerted opposite effects with STARS overexpression. SRF siRNA transfection blocked the effects of STARS overexpression in PASMC. In order to further confirm the role of STARS in PAH mice in vivo, we exposed STARS knockout mice to hypoxia and found lower RVSP and RVHI in knockout mice as compared with controls. Our results not only suggest that STARS plays a crucial role in the development of PAH by increasing the proliferation of PASMC through activation of the SRF/Egr-1 pathway, but also provides a new mechanism for hypoxia-induced PAH. In addition, STARS may represent a potential treatment target. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  1. Effect of propofol and etomidate on normoxic and chronically hypoxic pulmonary artery

    Directory of Open Access Journals (Sweden)

    Marthan Roger

    2006-03-01

    Full Text Available Abstract Background Chronic alveolar hypoxia results in sustained arterial constriction, and increase in pulmonary vascular resistance leading to pulmonary artery hypertension (PAHT. The aim of this study was to investigate the effect of propofol and etomidate on pulmonary artery (PA reactivity in chronically hypoxic (CH rats, a model of pulmonary arterial hypertension (PAHT, in normoxic animals, and human PA. Methods CH rats were maintained 14 days at 380 mmHg pressure in a hypobaric chamber. Human tissue was retrieved from histological lung pieces from patients undergoing resection for carcinoma. Cumulative concentrations of anaesthetics were tested on isolated vascular rings precontracted with phenylephrine (PHE or 100 mM KCl. Statistical comparisons were done by ANOVA, followed, when needed, by Student t tests with Bonferroni correction as post-hoc tests. Results In normoxic rat PA, maximal relaxation (Rmax induced by etomidate and propofol was 101.3 ± 0.8% and 94.0 ± 2.3%, respectively, in KCl-precontracted rings, and 63.3 ± 9.7% and 46.1 ± 9.1%, respectively, in PHE-precontracted rings (n = 7. In KCl-precontracted human PA, Rmax was 84.7 ± 8.6 % and 66.5 ± 11.8%, for etomidate and propofol, respectively, and 154.2 ± 22.4 % and 51.6 ± 15.1 %, respectively, in PHE-precontracted human PA (n = 7. In CH rat PA, the relaxant effect of both anaesthetics was increased in PHE-precontracted and, for etomidate only, in KCl-precontracted PA. In aorta, CH induced no change in the relaxant effect of anaesthetics. Conclusion Propofol and etomidate have relaxant properties in PA from human and normoxic rat. The relaxant effect is specifically accentuated in PA from CH rat, mainly via an effect on the pharmacomechanical coupling. Etomidate appears to be more efficient than propofol at identical concentration, but, taking into account clinical concentrations, etomidate is less potent than propofol, which effect was in the range of clinical doses

  2. Noninvasive Demonstration of Dual Coronary Artery Fistulas to Main Pulmonary Artery with 64-Slice Multidetector-Computed Tomography: A Case Report

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    Yoshiki Noda

    2010-01-01

    Full Text Available Coronary artery fistulas, including coronary pulmonary fistulas, are usually discovered accidently among the adult population when undergoing invasive coronary angiographies. We report here a 58-year-old woman with dual fistulas originating from the left anterior descending coronary artery and right coronary sinus to the main pulmonary artery, demonstrating noninvasively with multidetector-computed tomography (MDCT and transthoracic echocardiography (TTE.

  3. Isolated unilateral absence of the right pulmonary artery in two cats visualized by computed tomography angiography

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    Tyler JM Jordan

    2016-10-01

    Full Text Available Case series summary Two cats were evaluated for progressive exercise intolerance, dyspnea and unilateral infiltrate of the left lung. Computed tomography angiography (CTA revealed absence of the right pulmonary artery in both cats with systemic arterial collateral vessels perfusing the right segmental pulmonary arteries. In one case, the collateral vessels arose from the esophageal artery, while in the other case they derived off the right costocervical trunk. One cat was diagnosed with pulmonary hypertension and was euthanized owing to progressive respiratory distress despite medical management with sildenafil, pimobendan, clopidogrel and furosemide. The other cat, without echocardiographic evidence of pulmonary hypertension, was successfully managed with furosemide and enalapril for more than 4 years. Relevance and novel information CTA allowed visualization of a rare congenital heart malformation, unilateral absence of the right pulmonary artery, in two cats and accurately characterized the source of collateral blood supply to the affected lung. Severe pulmonary hypertension may be a negative prognostic factor in cats with this condition as medical therapy in the cat without evidence of pulmonary hypertension resolved clinical signs, while the cat with severe pulmonary hypertension died from the disease.

  4. Bovine aortic arch and idiopathic pulmonary artery aneurysm associated with bronchial compression

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    Süleyman Sezai Yıldız

    2015-09-01

    Full Text Available The left common carotid artery originating from the brachiocephalic trunk is termed the bovine aortic arch. Although it is the third most-common normal variant found in 9% humans, the origin of this term remains unclear. Until now, It has not been reported in the literature bovine aortic arch togetherness with pulmonary aneurysm and bronchial compression. Herein, we present a case with bovine aorta arch and pulmonary artery aneurysm associated with bronchial compression, which is incidentally detected by X-ray film. A 56-year-old Caucasian female admitted to the cardiology clinic with complaint of chest pain. Physical examination was unremarkable. Blood biochemistry values and cardiac markers were in normal range. Chest radiography revealed a widened mediastinum and prominent pulmonary conus with no active pulmonary disease. A subsequent transthoracic echocardiography revealed left ventricular hypertrophy, left atrial enlargement (diameter: 41 mm, mild mitral and tricuspid valve insufficiency, dilatation of main pulmonary artery (parasternal short-axis view diameter: 33 mm, normal pulmonary artery pressure and normal left ventricular systolic function. Computed tomography revealed bovine aortic arch associated with pulmonary artery aneurysm (diameter: 53 mm. And left main bronch of trachea was critically squeezed by aortic arch. Aortic and pulmonary vascular anomalies should be considered in patients with chest pain. And, identification with imaging modalities is important for prevention of chronic and irreversible complications.

  5. Membrane properties of smooth muscle cells in pulmonary arteries of the rat.

    Science.gov (United States)

    Suzuki, H; Twarog, B M

    1982-05-01

    Electrical properties of the membrane of smooth muscle cells in the rat main pulmonary artery (MPA) and a small pulmonary artery (SPA) were compared. MPA and SPA differed in several important respects, suggesting characteristic quantitative and qualitative differences in membrane properties. 1) Resting membrane potentials were similar in both (MPA 52.2 +/- 1.3 mV; SPA 51.5 +/- 1.7 mV). The cells displayed no spontaneous electrical activity. The muscle layers in both MPA and SPA showed cablelike properties; a graded local response to outward current pulses was observed, but no action potentials were evoked. 2) Tetraethylammonium chloride (TEA, 1-5 mM) depolarized, increased membrane resistance, and suppressed rectification in MPA. TEA strongly depolarized SPA and contraction ensued. 3) The maximum membrane depolarization produced by a 10-fold increase in extracellular [K+] was 48 mV in MPA and 47 mV in SPA. In K+-free solution gradual depolarization was observed in SPA, but the membrane potential in MPA was not modified. Restoration of K+-containing solution produced equivalent hyperpolarization in both tissues, indicating a similar degree of stimulation of electrogenic Na+-K+ pumping. 4) A Na+-deficient solution did not affect the membrane potential in MPA but depolarized SPA.

  6. Contribution of Impaired Parasympathetic Activity to Right Ventricular Dysfunction and Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension.

    Science.gov (United States)

    da Silva Gonçalves Bós, Denielli; Van Der Bruggen, Cathelijne E E; Kurakula, Kondababu; Sun, Xiao-Qing; Casali, Karina R; Casali, Adenauer G; Rol, Nina; Szulcek, Robert; Dos Remedios, Cris; Guignabert, Christophe; Tu, Ly; Dorfmüller, Peter; Humbert, Marc; Wijnker, Paul J M; Kuster, Diederik W D; van der Velden, Jolanda; Goumans, Marie-José; Bogaard, Harm-Jan; Vonk-Noordegraaf, Anton; de Man, Frances S; Handoko, M Louis

    2018-02-27

    The beneficial effects of parasympathetic stimulation have been reported in left heart failure, but whether it would be beneficial for pulmonary arterial hypertension (PAH) remains to be explored. Here, we investigated the relationship between parasympathetic activity and right ventricular (RV) function in patients with PAH, and the potential therapeutic effects of pyridostigmine (PYR), an oral drug stimulating the parasympathetic activity through acetylcholinesterase inhibition, in experimental pulmonary hypertension (PH). Heart rate recovery after a maximal cardiopulmonary exercise test was used as a surrogate for parasympathetic activity. RV ejection fraction was assessed in 112 patients with PAH. Expression of nicotinic (α-7 nicotinic acetylcholine receptor) and muscarinic (muscarinic acetylcholine type 2 receptor) receptors, and acetylcholinesterase activity were evaluated in RV (n=11) and lungs (n=7) from patients with PAH undergoing heart/lung transplantation and compared with tissue obtained from controls. In addition, we investigated the effects of PYR (40 mg/kg per day) in experimental PH. PH was induced in male rats by SU5416 (25 mg/kg subcutaneously) injection followed by 4 weeks of hypoxia. In a subgroup, sympathetic/parasympathetic modulation was assessed by power spectral analysis. At week 6, PH status was confirmed by echocardiography, and rats were randomly assigned to vehicle or treatment (both n=12). At the end of the study, echocardiography was repeated, with additional RV pressure-volume measurements, along with lung, RV histological, and protein analyses. Patients with PAH with lower RV ejection fraction (<41%) had a significantly reduced heart rate recovery in comparison with patients with higher RV ejection fraction. In PAH RV samples, α-7 nicotinic acetylcholine receptor was increased and acetylcholinesterase activity was reduced versus controls. No difference in muscarinic acetylcholine type 2 receptor expression was observed. Chronic

  7. The evolution of prostacyclins in pulmonary arterial hypertension: from classical treatment to modern management.

    Science.gov (United States)

    Burger, Charles D; D'Albini, Lesley; Raspa, Susan; Pruett, Janis A

    2016-01-01

    Prostacyclins for the treatment of pulmonary arterial hypertension (PAH) have historically been covered under the insurance medical benefit because they require durable medical equipment and are administered by an intravenous, subcutaneous, or inhalation route. However, more treatment options that target the prostacyclin pathway have become available. As the number and type of options expand, an improved understanding of these drugs will aid managed care decision makers in evaluating new treatment options and making clinically sound and cost-effective treatment decisions. PAH is a progressive disease of pulmonary vascular remodeling that increases pulmonary vascular resistance and often results in right-side heart failure and death if left untreated. Adverse event profiles, the complexity of administration modalities, and potential complications must be considered when administering prostacyclin therapy. Traditional modes of administration, with their potential challenges and complications, may have contributed to the unmet need for an oral agent. Another consideration for managed care decision makers is that oral agents are generally covered under the insurance pharmacy benefit. Access to oral medications with long-term outcomes data, as well as the improved convenience of oral therapy, may help patients with PAH maximize function by maintaining a more convenient and consistent therapeutic regimen.

  8. Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases

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    V Govindaraj

    2017-01-01

    Full Text Available Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.

  9. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature

    NARCIS (Netherlands)

    A.D.J. ten Harkel (Arend); N.A. Blom (Nico); J. Ottenkamp (Jaap)

    2002-01-01

    textabstractOBJECTIVE: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). BACKGROUND: Isolated UAPA is a rare anomaly. Some case

  10. [The relationship between pulmonary arterial and small airway inflammation in smokers with and without chronic obstructive pulmonary disease].

    Science.gov (United States)

    Lao, Qifang; Zeng, Xiaoliang; Zhong, Xiaoning; Zhang, Jianquan; He, Zhiyi

    2014-12-01

    To investigate the relationship between pulmonary arterial and small airway inflammation in smokers with normal lung function and smokers with chronic obstructive pulmonary disease (COPD). Patients requiring lung resection for peripheral lung cancer were divided into group A (nonsmokers with normal lung function, n = 10), group B (smokers with normal lung function, n = 13) and group C (smokers with stable COPD, n = 10). Normal pulmonary tissue was obtained more than 5 cm away from cancer lesion. The pathomorphological changes of the pulmonary muscularized arteries (MA) and small airways were observed by HE and Victoria blue-Van Gieson's stains.Lymphocytes infiltrated in the MA and small airways were observed by immunohistochemical methods. The characteristics and the correlations between pulmonary arterial inflammation and small airway inflammation were analyzed. The thickness of MA wall in the three groups was (119 ± 11), (139 ± 25) and (172 ± 28) µm respectively. The total small airway pathology score was (49 ± 10), (101 ± 34) and (163 ± 36) respectively. The score in group B and C was significantly higher than that in group A (P 0.05). The infiltration of CD(+)(3)T-lymphocytes and CD(+)(8)T-lymphocytes in the whole layer of MA was positively correlated with the total small airway pathology score respectively (r = 0.431,0.633, P arteries and small airways is the same kind of inflammation, mainly in the adventitia of pulmonary arteries and small airways. They are a part of pulmonary inflammation in COPD and promote the development of COPD.

  11. Anomalous Origin of One Pulmonary Artery From the Ascending Aorta: From Diagnosis to Treatment in Angola.

    Science.gov (United States)

    Manuel, Valdano; Sousa-Uva, Miguel; Morais, Humberto; Magalhães, Manuel P; Pedro, Albino; Miguel, Gade; Nunes, Maria A S; Gamboa, Sebastiana; Júnior, António P F

    2015-10-01

    Anomalous origin of one pulmonary artery is a rare congenital heart disease in which one pulmonary artery branch originates from the ascending aorta. To describe the experience of a cardiothoracic center in an African country to repair anomalous origin of one pulmonary artery in the context of Portugal-Angola collaboration. Between March 2011 and March 2015, four consecutive patients with anomalous origin of pulmonary artery branch underwent surgical correction. The mean age was 1.6 months. The mean weight was 4 kg. All had right pulmonary artery branch originating from the ascending aorta. All patients underwent direct implantation of right pulmonary branch to main pulmonary artery. Two patents had patent ductus arteriosus and one had atrial septal defect. Two patients had pulmonary hypertension. There was no registration of death. The mean cardiopulmonary bypass time was 75.5 ± 4.5 minutes, mean aortic cross-clamping time was 40 ± 5.6 minutes, and mean duration of the postoperative intensive care unit stay was 6.8 ± 5.7 days. At discharge, one patient had residual gradient of 25 mm Hg, the remainder had no significant gradient. The mean follow-up time was 11 months (5-28 months). One week after discharge, one patient presented operative wound dehiscence. At the last follow-up, all patients were alive, and no significant residual gradient or stenosis at site of anastomosis was observed. No reintervention was required. Anomalous origin of one pulmonary artery is a rare but potentially treatable lesion if operated early in life. Direct implantation was a good technique with good short-term results. © The Author(s) 2015.

  12. Percutaneous transhepatic venous embolization of pulmonary artery aneurysm in Hughes-Stovin syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Kyung Ah; Kim, Man Deuk; Oh, Do Yun; Park, Pil Won [Bundang CHA General Hospital, Pochon CHA University, Seongnam (Korea, Republic of)

    2007-08-15

    Hughes-Stovin syndrome is an extremely rare entity. We present a case of a 42-year-old man, who developed deep vein and inferior vena cava (IVC) thrombosis, repeated internal bleeding and pulmonary artery aneurysms (PAAs). The patient presented with massive hemoptysis and with PAAs of a 2.5 cm maximum diameter. We describe the successful percutaneous transhepatic venous embolization of the PAAs due to occluded common vascular pathways to the pulmonary artery.

  13. Assessment of airways compression by MR imaging in children with aneurysmal pulmonary arteries

    International Nuclear Information System (INIS)

    Ditchfield, M.R.; Culham, J.A.G.

    1995-01-01

    Magnetic resonance imaging (MRI) has an established role in the accurate and non invasive assessment of airways compression by congenital vascular rings and pulmonary artery slings, making angiography of these lesions unnecessary. This role can be broadened to encompass other vascular compressive lesions, as in the two pediatric patients described here with aneurysmal pulmonary arteries of different etiology, one congenital and the other acquired. (orig.)

  14. A unique case of pulmonary artery catheter bleeding from the oximetry connection port

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    Suman Rajagopalan

    2014-12-01

    Full Text Available Pulmonary artery catheter is an invasive monitor usually placed in high-risk cardiac surgical patients to optimize the cardiac functions. We present this case of blood oozing from the oximetry connection port of the pulmonary artery catheter that resulted in the inability to monitor continuous cardiac output requiring replacement of the catheter. The cause of this abnormal bleeding was later confirmed to be due to a manufacturing defect.

  15. Flash pulmonary edema in patients with renal artery stenosis--the Pickering Syndrome

    DEFF Research Database (Denmark)

    Pelta, Anna; Andersen, Ulrik B; Just, Sven

    2010-01-01

    We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases.......We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases....

  16. Quality of life as a prognostic marker in pulmonary arterial hypertension

    OpenAIRE

    Fernandes, Caio JCS; Martins, Barbara CS; Jardim, Carlos VP; Ciconelli, Rozana M; Morinaga, Luciana K; Breda, Ana Paula; Hoette, Susana; Souza, Rogério

    2014-01-01

    Background: Improvement in quality of life together with better survival are the ultimate goals in the treatment of pulmonary arterial hypertension (PAH) patients. the objective of this study was to evaluate the health-related quality of life (HRQL) of pulmonary arterial hypertension (PAH) patients with the SF-36 generic questionnaire and to identify the prognostic implication of this assessment.Methods: Fifty-four consecutive newly diagnosed PAH patients (WHO classification group I) in a sin...

  17. Helicity and Vorticity of Pulmonary Arterial Flow in Patients With Pulmonary Hypertension: Quantitative Analysis of Flow Formations.

    Science.gov (United States)

    Schäfer, Michal; Barker, Alex J; Kheyfets, Vitaly; Stenmark, Kurt R; Crapo, James; Yeager, Michael E; Truong, Uyen; Buckner, J Kern; Fenster, Brett E; Hunter, Kendall S

    2017-12-20

    Qualitative and quantitative flow hemodynamic indexes have been shown to reflect right ventricular (RV) afterload and function in pulmonary hypertension (PH). We aimed to quantify flow hemodynamic formations in pulmonary arteries using 4-dimensional flow cardiac magnetic resonance imaging and the spatial velocity derivatives helicity and vorticity in a heterogeneous PH population. Patients with PH (n=35) and controls (n=10) underwent 4-dimensional flow magnetic resonance imaging study for computation of helicity and vorticity in the main pulmonary artery (MPA), the right pulmonary artery, and the RV outflow tract. Helicity and vorticity were correlated with standard RV volumetric and functional indexes along with MPA stiffness assessed by measuring relative area change. Patients with PH had a significantly decreased helicity in the MPA (8 versus 32 m/s 2 ; P flow hemodynamic character in patients with PH assessed via quantitative analysis is considerably different when compared with healthy and normotensive controls. A strong association between helicity in pulmonary arteries and ventricular-vascular coupling suggests a relationship between the mechanical and flow hemodynamic domains. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  18. Rosiglitazone Attenuated Endothelin-1-Induced Vasoconstriction of Pulmonary Arteries in the Rat Model of Pulmonary Arterial Hypertension via Differential Regulation of ET-1 Receptors

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    Yahan Liu

    2014-01-01

    Full Text Available Pulmonary arterial hypertension (PAH is a fatal disease characterized by a progressive increase in pulmonary arterial pressure leading to right ventricular failure and death. Activation of the endothelin (ET-1 system has been demonstrated in plasma and lung tissue of PAH patients as well as in animal models of PAH. Recently, peroxisome proliferator-activated receptor γ (PPARγ agonists have been shown to ameliorate PAH. The present study aimed to investigate the mechanism for the antivasoconstrictive effects of rosiglitazone in response to ET-1 in PAH. Sprague-Dawley rats were exposed to chronic hypoxia (10% oxygen for 3 weeks. Pulmonary arteries from PAH rats showed an enhanced vasoconstriction in response to ET-1. Treatment with PPARγ agonist rosiglitazone (20 mg/kg per day with oral gavage for 3 days attenuated the vasocontractive effect of ET-1. The effect of rosiglitazone was lost in the presence of L-NAME, indicating a nitric oxide-dependent mechanism. Western blotting revealed that rosiglitazone increased ETBR but decreased ETAR level in pulmonary arteries from PAH rats. ETBR antagonist A192621 diminished the effect of rosiglitazone on ET-1-induced contraction. These results demonstrated that rosiglitazone attenuated ET-1-induced pulmonary vasoconstriction in PAH through differential regulation of the subtypes of ET-1 receptors and, thus, provided a new mechanism for the therapeutic use of PPARγ agonists in PAH.

  19. Relationship of daily arterial blood pressure monitoring readings and arterial stiffness profile in male patients with chronic obstructive pulmonary disease combined with arterial hypertension

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    Karoli N.A.

    2013-06-01

    Full Text Available The aim of the study was to determine correlation between arterial blood pressure daily rhythm and daily profile of arterial stiffness in male patients with chronic obstructive pulmonary disease (COPD and arterial hypertension. Materials et methods: Prospective investigation comprised 45 male patients with COPD and arterial hypertension. Individuals of 40 years younger and 80 years elder, patients with diabetes, stroke, angina pectoris, or heart infarction, vascular diseases, and exacerbation of chronic disease, bronchial and pulmonary diseases of other etiology were excluded from the analyses. Comparison group included 47 patients with essential arterial hypertension and without chronic respiratory diseases closely similar on general parameters with patients from main clinical series. Twenty-four-hour arterial blood pressure monitoring (ABPM and daily arterial stiffness monitoring were performed using BPLab® MnSDP-2 apparatus (Petr Telegin, Russian Federation. Results: Patients with COPD combined with arterial hypertension with raised arterial stiffness measures prevail over individuals in essential hypertension group. There is pathological alteration of the ABPM circadian rhythm and raised «Pressure load» values in raised arterial stiffness group. Conclusion: We found ABPM raised parameters in patients with COPD and arterial hypertension. It confirms necessity of ABPM in daily arterial stiffness assessment in patients with COPD.

  20. A case of William's syndrome associated peripheral pulmonary arterial stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan [College of Medicine, Yeungam University, Daegu (Korea, Republic of)

    1988-06-15

    William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures.

  1. Inspiratory and expiratory pulmonary CT: change of bronchial and accompanying pulmonary arterial diameter during respiration in healthy adult men

    International Nuclear Information System (INIS)

    Jeon, Eui Yong; Kim, Chang Soo; Choi, Yo Won; Jeon, Seok Chol; Yun, Ho Ju; Kim, Sun I; Seo, Heung Suk; Hahm, Chang Kok; Park Chung Ki

    1996-01-01

    To evaluate changes in the diameter of bronchi and pulmonary arteries during respiration. The ratio of the diameter of matched inner bronchi and accompanying pulmonary arteries was calculated from full inspiration and expiration HRCT of healthy men. In 106 pairs of matched bronchi and pulmonary arteries, the bronhchial-arterial ratios were0.61 ±0.18(upper lung), 0.72±0.21(lower lung), and 0.65±0.20(total) at full inspiration and 0.51±0.32(upper lung), 0.52±0.15(lower lung), and 0.51±0.26(total) at full expiration HRCT, denotiong a statistically significant increase in bronchial diameter of the entire lung at full inspiration. The inner diameter of the bronchus was larger than the diameter of the accompanying pulmonary artery by 0.1% at full expiration and 8.5% at full inspiration. The result of this study would be helpful in understanding the physiology of the lung during respiration

  2. Peripheral arterial disease in patients with chronic obstructive pulmonary disease.

    Science.gov (United States)

    Pecci, R; De La Fuente Aguado, J; Sanjurjo Rivo, A B; Sanchez Conde, P; Corbacho Abelaira, M

    2012-10-01

    Cardiovascular disease (CV) is the second leading cause of morbidity and mortality in chronic obstructive pulmonary disease (COPD). Peripheral arterial disease (PAD) is associated with cardiovascular disease, and its risk factors are common to other atherosclerotic diseases. The objective is to determine the prevalence of PAD in a population of patients with COPD using the ankle / brachial index (ABI) and to investigate the relationship between PAD and lung disease severity. In a prospective cross-sectional study, 246 patients with COPD were recruited. Patients were enrolled consecutively according to their admission to Povisa hospital from September 1, 2008, until March 1, 2010, and were assessed by clinical history, spirometry and ABI. The COPD severity was graded by GOLD criteria in spirometry. Overall, 84 patients (36.8%) had abnormal ABI results and 59 (70.2%) were asymptomatic for PAD. COPD patients with PAD had a higher prevalence of moderate to severe COPD (61.9% vs. 41.7%, P=0.004), lower mean forced expiratory volume in 1 second (FEV1) values (46.7% ± 15 vs. 52.3±14%, P=0.001) and a higher prevalence of hypertension (69% vs. 54.3%, P=0.03) and previous cardiovascular disease (34.5% vs. 21.3%, P=0.03). There was a high prevalence of asymptomatic PAD in the COPD patients we examined. Abnormal ABI results were associated with a higher prevalence of cardiovascular risk factors and more severe lung disease. The diagnosis of peripheral arterial disease in COPD is important because this is an entity that limits the patient's physical activity and impairs their quality of life in addition to turn it into a high cardiovascular risk patient that requiring additional therapeutic measures.

  3. Upregulated Genes In Sporadic, Idiopathic Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Yacoub Magdi H

    2006-01-01

    Full Text Available Abstract Background To elucidate further the pathogenesis of sporadic, idiopathic pulmonary arterial hypertension (IPAH and identify potential therapeutic avenues, differential gene expression in IPAH was examined by suppression subtractive hybridisation (SSH. Methods Peripheral lung samples were obtained immediately after removal from patients undergoing lung transplant for IPAH without familial disease, and control tissues consisted of similarly sampled pieces of donor lungs not utilised during transplantation. Pools of lung mRNA from IPAH cases containing plexiform lesions and normal donor lungs were used to generate the tester and driver cDNA libraries, respectively. A subtracted IPAH cDNA library was made by SSH. Clones isolated from this subtracted library were examined for up regulated expression in IPAH using dot blot arrays of positive colony PCR products using both pooled cDNA libraries as probes. Clones verified as being upregulated were sequenced. For two genes the increase in expression was verified by northern blotting and data analysed using Student's unpaired two-tailed t-test. Results We present preliminary findings concerning candidate genes upregulated in IPAH. Twenty-seven upregulated genes were identified out of 192 clones examined. Upregulation in individual cases of IPAH was shown by northern blot for tissue inhibitor of metalloproteinase-3 and decorin (P Conclusion Four of the up regulated genes, magic roundabout, hevin, thrombomodulin and sucrose non-fermenting protein-related kinase-1 are expressed specifically by endothelial cells and one, muscleblind-1, by muscle cells, suggesting that they may be associated with plexiform lesions and hypertrophic arterial wall remodelling, respectively.

  4. HMGB1 promotes the development of pulmonary arterial hypertension in rats.

    Directory of Open Access Journals (Sweden)

    Yukari Sadamura-Takenaka

    Full Text Available Pulmonary arterial hypertension (PAH is characterized by increased pulmonary vascular resistance leading to right ventricular failure and death. Recent studies have suggested that chronic inflammatory processes are involved in the pathogenesis of PAH. However, the molecular and cellular mechanisms driving inflammation have not been fully elucidated.To elucidate the roles of high mobility group box 1 protein (HMGB1, a ubiquitous DNA-binding protein with extracellular pro-inflammatory activity, in a rat model of PAH.Male Sprague-Dawley rats were administered monocrotaline (MCT. Concentrations of HMGB1 in bronchoalveolar lavage fluid (BALF and serum, and localization of HMGB1 in the lung were examined over time. The protective effects of anti-HMGB1 neutralizing antibody against MCT-induced PAH were tested.HMGB1 levels in BALF were elevated 1 week after MCT injection, and this elevation preceded increases of other pro-inflammatory cytokines, such as TNF-α, and the development of PAH. In contrast, serum HMGB1 levels were elevated 4 weeks after MCT injection, at which time the rats began to die. Immunohistochemical analyses indicated that HMGB1 was translocated to the extranuclear space in periarterial infiltrating cells, alveolar macrophages, and bronchial epithelial cells of MCT-injected rats. Anti-HMGB1 neutralizing antibody protected rats against MCT-induced lung inflammation, thickening of the pulmonary artery wall, and elevation of right ventricular systolic pressure, and significantly improved the survival of the MCT-induced PAH rats.Our results identify extracellular HMGB1 as a promoting factor for MCT-induced PAH. The blockade of HMGB1 activity improved survival of MCT-induced PAH rats, and thus might be a promising therapy for the treatment of PAH.

  5. Recanalization of an occluded left pulmonary artery: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Ali Ibrahim Elarabi

    2017-01-01

    Full Text Available We report an 8-year-old male child with tetralogy of Fallot (TOF, who developed left pulmonary artery (LPA atresia, following surgical repair of TOF and left pulmonary arterioplasty at the age of 6 years. He underwent successful radiofrequency recanalization and stenting of the LPA. The LPA exhibited satisfactory growth for 3 months, following recanalization and stenting.

  6. The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle

    NARCIS (Netherlands)

    Manders, E.; Rain, S.; Bogaard, H.J.; Handoko, M.L.; Stienen, G.J.M.; Vonk Noordegraaf, A.; Ottenheijm, C.A.C.; de Man, F.S.

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a fatal lung disease characterised by progressive remodelling of the small pulmonary vessels. The daily-life activities of patients with PAH are severely limited by exertional fatigue and dyspnoea. Typically, these symptoms have been explained by right heart

  7. Color Doppler evaluation of the ocular arterial flow changes in chronic obstructive pulmonary disease

    Energy Technology Data Exchange (ETDEWEB)

    Ozer, Tulay [Department of Radiology, Karaelmas University, School of Medicine, Tip Fakueltesi, Radyoloji AD, 67600-Kozlu, Zonguldak (Turkey)]. E-mail: ozertulay@yahoo.com; Altin, Remzi [Department of Pulmonary Medicine, Karaelmas University, School of Medicine, Kozlu, Zonguldak (Turkey); Ugurbas, Suat Hayri [Department of Ophthalmology, Karaelmas University, School of Medicine, Kozlu, Zonguldak (Turkey); Ozer, Yetkin [Department of Anesthesiology and Reanimation, Karaelmas University, School of Medicine, Kozlu, Zonguldak (Turkey); Mahmutyazicioglu, Kamran [Department of Radiology, Karaelmas University, School of Medicine, Tip Fakueltesi, Radyoloji AD, 67600-Kozlu, Zonguldak (Turkey); Kart, Levent [Department of Pulmonary Medicine, Karaelmas University, School of Medicine, Kozlu, Zonguldak (Turkey)

    2006-01-15

    Purpose: To evaluate the hemodynamic changes in the extraocular orbital vessels of the patients with chronic obstructive pulmonary disease (COPD), using color Doppler ultrasonography (CDU) technique, and to compare the results with those of healthy control subjects. Methods: Forty-five patients with COPD and 17 healthy control subjects were included in this study. Patients with COPD were classified according to the Global Initiative for Chronic Obstructive Lung Disease (GOLD). Fifteen patients of stage I COPD (mild airflow limitation), stage II COPD (worsening airflow limitation) or stage III COPD (severe airflow limitation) were enrolled into Group I, II and III, respectively. End tidal carbon dioxide (EtCO{sub 2}), peripheral oxygen saturation (SpO{sub 2}), pulse rate (PR) and respiratory rate (RR) were measured by using capnograph/pulse oximeter in all patients. Measurements were performed in only one randomly chosen eye of each participant. The peak systolic velocity (PSV), end-diastolic velocity (EDV), and resistance index (RI) were measured in the ophthalmic artery (OA), central retinal artery (CRA), lateral short posterior ciliary artery (LPCA) and medial short posterior ciliary artery (MPCA), using CDU technique. Results: The PSV measurements of the OA were significantly higher in Groups II and III compared to control group (p < 0.01, p < 0.001, respectively). The RI values from OA and CRA were significantly higher in Group II and III than the control group (p < 0.05). The RI values of LPCA and MPCA were also significantly higher in Group II than the control subjects (p < 0.05). When RI values were compared, mean values of LPCA and MPCA were significantly lower in Group III than in Group II (p < 0.05). There were no significant differences between Group I and control patients about PSVs, EDVs and RI values of all arteries Statistically significant correlations were found for the EtCO{sub 2} with PSV (r = 0.53, p < 0.01) and EDV (r = 0.51, p < 0.01) of the

  8. Tetralogy of Fallot with origin of left pulmonary artery from the ascending aorta

    International Nuclear Information System (INIS)

    Robida, A.; Fettich, D.

    1985-01-01

    Anomalous origin of the left pulmonary artery in tetralogy of Fallot was diagnosed in a 4-year-old boy by cardiac catheterization. Corrective surgery was performed. The child died immediately following the surgical procedure. Postmortem examination revealed obstructive pulmonary vascular disease of the left lung and normal histology of right lung vessels. Early recognition and surgical correction of the anomaly is important with the view to preventing obstructive pulmonary vascular disease. (orig.)

  9. Tetralogy of Fallot with origin of left pulmonary artery from the ascending aorta

    Energy Technology Data Exchange (ETDEWEB)

    Robida, A.; Fettich, D.

    1985-09-01

    Anomalous origin of the left pulmonary artery in tetralogy of Fallot was diagnosed in a 4-year-old boy by cardiac catheterization. Corrective surgery was performed. The child died immediately following the surgical procedure. Postmortem examination revealed obstructive pulmonary vascular disease of the left lung and normal histology of right lung vessels. Early recognition and surgical correction of the anomaly is important with the view to preventing obstructive pulmonary vascular disease.

  10. Bronchial Artery Embolization in the Management of Pulmonary Parenchymal Endometriosis with Hemoptysis

    International Nuclear Information System (INIS)

    Kervancioglu, Selim; Andic, Cagatay; Bayram, Nazan; Telli, Cumali; Sarica, Akif; Sirikci, Akif

    2008-01-01

    Pulmonary parenchymal endometriosis is extremely rare and usually manifests itself with a recurrent hemoptysis associated with the menstrual cycle. The therapies proposed for women with endometriosis consist of medical treatments and surgery. Bronchial artery embolization has become a well-established and minimally invasive treatment modality for hemoptysis, and to the best of our knowledge, it has not been reported in pulmonary endometriosis. We report a case of pulmonary parenchymal endometriosis treated with embolotheraphy for hemoptysis.

  11. Upfront triple combination therapy-induced pulmonary edema in a case of pulmonary arterial hypertension associated with Sjogren's syndrome

    Directory of Open Access Journals (Sweden)

    Kimikazu Takeuchi

    Full Text Available Clinical efficacy of combination therapy using vasodilators for pulmonary arterial hypertension (PAH is well established. However, information on its safety are limited. We experienced a case of primary Sjogren's syndrome associated with PAH where the patient developed pulmonary edema immediately after the introduction of upfront triple combination therapy. Although the combination therapy successfully stabilized her pre-shock state, multiple ground glass opacities (GGO emerged. We aborted the dose escalation of epoprostenol and initiated continuous furosemide infusion and noninvasive positive pressure ventilation (NPPV, but this did not prevent an exacerbation of pulmonary edema. Chest computed tomography showing diffuse alveolar infiltrates without inter-lobular septal thickening suggests the pulmonary edema was unlikely due to cardiogenic pulmonary edema and pulmonary venous occlusive disease. Acute respiratory distress syndrome was also denied from no remarkable inflammatory sign and negative results of drug-induced lymphocyte stimulation tests (DLST. We diagnosed the etiological mechanism as pulmonary vasodilator-induced trans-capillary fluid leakage. Following steroid pulse therapy dramatically improved GGO. We realized that overmuch dose escalation of epoprostenol on the top of dual upfront combination poses the risk of pulmonary edema. Steroid pulse therapy might be effective in cases of vasodilator-induced pulmonary edema in Sjogren's syndrome associated with PAH. Keywords: Steroid therapy, Ground glass opacity, Inter-lobular septal thickening, Epoprostenol, Acute respiratory distress syndrome, Trans-capillary fluid leakage

  12. Circulating biomarkers in pulmonary arterial hypertension: update and future direction.

    Science.gov (United States)

    Pezzuto, Beatrice; Badagliacca, Roberto; Poscia, Roberto; Ghio, Stefano; D'Alto, Michele; Vitulo, Patrizio; Mulè, Massimilano; Albera, Carlo; Volterrani, Maurizio; Fedele, Francesco; Vizza, Carmine Dario

    2015-03-01

    Pulmonary arterial hypertension (PAH) is a complex disease with a poor prognosis. In recent years, great advances have occurred in our understanding of the pathophysiologic mechanisms underlying the characteristic vascular proliferative lesions, thus allowing the development of several specific drugs. Nevertheless, PAH still presents a high mortality; therefore, early diagnosis and prognostic stratification seem to be of paramount importance in order to choose the best therapeutic strategies. Circulating biomarkers have been proposed as potentially noninvasive and objective parameters for diagnosis, prognosis, and response to therapy. The molecules evaluated to date, including markers of dysfunction and neurohormonal activation, myocardial injury, inflammation and oxidative stress, vascular damage and remodelling, end-organ failure, and gene expression, reflect the complex pathophysiology of PAH. However, not one of these shows all the characteristics of the ideal biomarker; thus, a multiparameter approach is probably desirable. Moreover, future direction could be research of structural proteins specifically expressed in the pathologic tissue that act as disease-specific markers. This report presents an extensive review of circulating biomarkers in PAH and some consideration about potential future direction in this area. Copyright © 2015 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

  13. Beyond a single pathway: combination therapy in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Olivier Sitbon

    2016-12-01

    Full Text Available There is a strong rationale for combining therapies to simultaneously target three of the key pathways implicated in the pathogenesis of pulmonary arterial hypertension (PAH. Evidence to support this strategy is growing, and a number of studies have demonstrated that combination therapy, administered as either a sequential or an initial regimen, can improve long-term outcomes in PAH. Dual combination therapy with a phosphodiesterase-5 inhibitor and an endothelin receptor antagonist is the most widely utilised combination regimen. However, some patients fail to achieve their treatment goals on dual therapy and may benefit from the addition of a third drug. The use of triple therapy in clinical practice was previously reserved for patients with severe disease due to the need for parenteral administration of prostanoids. Although triple therapy with parenteral prostanoids plays a key role in the management of severe PAH, the approval of oral therapies that target the prostacyclin pathway means that all three pathways can now be targeted with oral drugs at an earlier disease stage. Furthermore, there is evidence demonstrating that this approach can delay disease progression. Based on the evidence available, it is becoming increasingly clear that all PAH patients should be offered the benefits of combination therapy.

  14. Challenges in the diagnosis and treatment of pulmonary arterial hypertension.

    LENUS (Irish Health Repository)

    2012-12-01

    Advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have resulted in significant improvements in outcomes for patients with this devastating and progressive disease. However, because of the non-specific nature of its symptoms, and the low level of suspicion among clinicians, prompt and accurate diagnosis of PAH as a rare disease remains a challenge. This article explains some of the issues that need to be addressed when faced with a patient with suspected PAH and describes how noninvasive and invasive techniques can be used effectively to ensure an accurate diagnosis. The availability of PAH-specific therapy means that once diagnosed, patients have a much greater chance of survival than they would have had in the past. However, despite improved survival, mortality is still high and, therefore, there is still room for improvement. It is currently recommended that patients with an inadequate clinical response to treatment receive sequential combination therapy; however, supportive data are still scarce. Although there is no clear explanation, these findings may be explained by the design and end-points chosen in clinical trials, the changing population of PAH and a need to improve the management strategy in this disease. Indeed, there is a clear need for randomised controlled studies that investigate whether adopting individualised treatment strategies, including upfront combination therapy, could help to optimise long-term management of patients with PAH.

  15. How to detect disease progression in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    J-L. Vachiéry

    2012-03-01

    Full Text Available Pulmonary arterial hypertension (PAH is a rapidly progressive disease, ultimately leading to right heart failure and death. Accumulating evidence indicates that intervention early in disease progression results in better outcomes than delaying treatment. In this review we will discuss the assessments and strategies that can be used to monitor disease progression and guide clinical management. Many tools, such as symptoms, functional classification, exercise capacity, haemodynamic measures, findings on cardiac imaging and levels of biomarkers, have shown to be prognostic for survival both at diagnosis and during treatment. However, attempts to define goal thresholds have produced a variety of results. Several groups have developed risk calculators to estimate individual patients' mortality risk, but the accuracy of these tools across different patient populations remains unknown. What is clear is the importance of regularly assessing a range of parameters and then tailoring treatment goals to each patient. In addition, the use of a multidisciplinary team approach is crucial in order to support patients through all aspects of managing their condition. There is still an urgent need for prospective collaborative initiatives to assess novel goals and improve treatment strategies that would allow physicians to personalise and optimise clinical management for their patients with PAH.

  16. Identifying “super responders” in pulmonary arterial hypertension

    Science.gov (United States)

    Halliday, Stephen J.; Hemnes, Anna R.

    2017-01-01

    Pharmacotherapeutic options for pulmonary arterial hypertension (PAH) have increased dramatically in the last two decades and along with this have been substantial improvements in survival. Despite these advances, however, PAH remains a progressive and ultimately fatal disease for most patients and only epoprostenol has been shown to improve survival in a randomized control trial. Clinical observations of the heterogeneity of treatment response to different classes of medications across the phenotypically diverse PAH population has led to the identification of patients who derive significantly more benefit from certain medications than the population mean, the so-called “super responders.” This was first recognized among PAH patients with acute vasodilator response during invasive hemodynamic testing, a subset of whom have dramatically improved survival when treated with calcium channel blocker (CCB) therapy. Retrospective studies have now suggested a sex discrepancy in response to endothelin receptor antagonists (ERA) and phosphodiesterase inhibitors, and more recently a few studies have found genomic associations with response to CCBs and ERAs. With increasing availability of “omics” technologies, recognition of these “super responders,” combined with careful clinical and molecular phenotyping, will lead to advances in pharmacogenomics, precision medicine, and continued improvements in survival among PAH patients. PMID:28597766

  17. Pulmonary arterial hypertension : a comprehensive review of pharmacological treatment.

    Science.gov (United States)

    Berman Rosenzweig, Erika; Barst, Robyn J

    2006-01-01

    The treatment of pediatric pulmonary arterial hypertension (PAH) is challenging due to the serious nature of the disease, its rapid progression, and the limited treatment options available. While oral calcium channel antagonists and continuous intravenous epoprostenol have been used successfully for over a decade, novel treatment options - including prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors - may change the course of this disease for many children in the future.Prostacyclin analogs offer the benefit over continuous intravenous epoprostenol of an alternative delivery system. However, the efficacy of these medications compared with intravenous epoprostenol and the risk/benefits of each analog need to be weighed in future trials, which need to include larger numbers of pediatric patients to optimize therapy and outcome for individual children with PAH.For patients who do not have an acute response to vasodilator testing or have failed treatment with oral calcium channel antagonists, endothelin receptor antagonists may offer a viable treatment option. Furthermore, in the future, the addition of endothelin receptor antagonists to long-term therapy with calcium channel antagonists or to epoprostenol or a prostacyclin analog may increase the overall efficacy of treatment of PAH. Large multi-institutional randomized trials to determine whether sildenafil is effective and safe for the long-term treatment of PAH in children are in progress.A comprehensive review of these newer agents with an emphasis on the pathobiology/pathophysiology of PAH provides insight into the future management of pediatric PAH patients.

  18. Iptakalim attenuates hypoxia-induced pulmonary arterial hypertension in rats by endothelial function protection.

    Science.gov (United States)

    Zhu, Rong; Bi, Li-Qing; Wu, Su-Ling; Li, Lan; Kong, Hui; Xie, Wei-Ping; Wang, Hong; Meng, Zi-Li

    2015-08-01

    The present study aimed to investigate the protective effects of iptakalim, an adenosine triphosphate (ATP)-sensitive potassium channel opener, on the inflammation of the pulmonary artery and endothelial cell injury in a hypoxia-induced pulmonary arterial hypertension (PAH) rat model. Ninety-six Sprague-Dawley rats were placed into normobaric hypoxia chambers for four weeks and were treated with iptakalim (1.5 mg/kg/day) or saline for 28 days. The right ventricle systolic pressures (RVSP) were measured and small pulmonary arterial morphological alterations were analyzed with hematoxylin and eosin staining. Enzyme-linked immunosorbent assay (ELISA) was performed to analyze the content of interleukin (IL)-1β and IL-10. Immunohistochemical analysis for ED1(+) monocytes was performed to detect the inflammatory cells surrounding the pulmonary arterioles. Western blot analysis was performed to analyze the expression levels of platelet endothelial cell adhesion molecule-1 (PECAM-1) and endothelial nitric oxide synthase (eNOS) in the lung tissue. Alterations in small pulmonary arteriole morphology and the ultrastructure of pulmonary arterial endothelial cells were observed via light and transmission electron microscopy, respectively. Iptakalim significantly attenuated the increase in mean pulmonary artery pressure, RVSP, right ventricle to left ventricle plus septum ratio and small pulmonary artery wall remodeling in hypoxia-induced PAH rats. Iptakalim also prevented an increase in IL-1β and a decrease in IL-10 in the peripheral blood and lung tissue, and alleviated inflammatory cell infiltration in hypoxia-induced PAH rats. Furthermore, iptakalim enhanced PECAM-1 and eNOS expression and prevented the endothelial cell injury induced by hypoxic stimuli. Iptakalim suppressed the pulmonary arteriole and systemic inflammatory responses and protected against the endothelial damage associated with the upregulation of PECAM-1 and eNOS, suggesting that iptakalim may represent a

  19. Contribution of reactive oxygen species to the pathogenesis of pulmonary arterial hypertension

    Science.gov (United States)

    Naik, Jay S.; Weise-Cross, Laura; Detweiler, Neil D.; Herbert, Lindsay M.; Yellowhair, Tracylyn R.; Resta, Thomas C.

    2017-01-01

    Pulmonary arterial hypertension is associated with a decreased antioxidant capacity. However, neither the contribution of reactive oxygen species to pulmonary vasoconstrictor sensitivity, nor the therapeutic efficacy of antioxidant strategies in this setting are known. We hypothesized that reactive oxygen species play a central role in mediating both vasoconstrictor and arterial remodeling components of severe pulmonary arterial hypertension. We examined the effect of the chemical antioxidant, TEMPOL, on right ventricular systolic pressure, vascular remodeling, and enhanced vasoconstrictor reactivity in both chronic hypoxia and hypoxia/SU5416 rat models of pulmonary hypertension. SU5416 is a vascular endothelial growth factor receptor antagonist and the combination of chronic hypoxia/SU5416 produces a model of severe pulmonary arterial hypertension with vascular plexiform lesions/fibrosis that is not present with chronic hypoxia alone. The major findings from this study are: 1) compared to hypoxia alone, hypoxia/SU5416 exposure caused more severe pulmonary hypertension, right ventricular hypertrophy, adventitial lesion formation, and greater vasoconstrictor sensitivity through a superoxide and Rho kinase-dependent Ca2+ sensitization mechanism. 2) Chronic hypoxia increased medial muscularization and superoxide levels, however there was no effect of SU5416 to augment these responses. 3) Treatment with TEMPOL decreased right ventricular systolic pressure in both hypoxia and hypoxia/SU5416 groups. 4) This effect of TEMPOL was associated with normalization of vasoconstrictor responses, but not arterial remodeling. Rather, medial hypertrophy and adventitial fibrotic lesion formation were more pronounced following chronic TEMPOL treatment in hypoxia/SU5416 rats. Our findings support a major role for reactive oxygen species in mediating enhanced vasoconstrictor reactivity and pulmonary hypertension in both chronic hypoxia and hypoxia/SU5416 rat models, despite a

  20. Contribution of reactive oxygen species to the pathogenesis of pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    Nikki L Jernigan

    Full Text Available Pulmonary arterial hypertension is associated with a decreased antioxidant capacity. However, neither the contribution of reactive oxygen species to pulmonary vasoconstrictor sensitivity, nor the therapeutic efficacy of antioxidant strategies in this setting are known. We hypothesized that reactive oxygen species play a central role in mediating both vasoconstrictor and arterial remodeling components of severe pulmonary arterial hypertension. We examined the effect of the chemical antioxidant, TEMPOL, on right ventricular systolic pressure, vascular remodeling, and enhanced vasoconstrictor reactivity in both chronic hypoxia and hypoxia/SU5416 rat models of pulmonary hypertension. SU5416 is a vascular endothelial growth factor receptor antagonist and the combination of chronic hypoxia/SU5416 produces a model of severe pulmonary arterial hypertension with vascular plexiform lesions/fibrosis that is not present with chronic hypoxia alone. The major findings from this study are: 1 compared to hypoxia alone, hypoxia/SU5416 exposure caused more severe pulmonary hypertension, right ventricular hypertrophy, adventitial lesion formation, and greater vasoconstrictor sensitivity through a superoxide and Rho kinase-dependent Ca2+ sensitization mechanism. 2 Chronic hypoxia increased medial muscularization and superoxide levels, however there was no effect of SU5416 to augment these responses. 3 Treatment with TEMPOL decreased right ventricular systolic pressure in both hypoxia and hypoxia/SU5416 groups. 4 This effect of TEMPOL was associated with normalization of vasoconstrictor responses, but not arterial remodeling. Rather, medial hypertrophy and adventitial fibrotic lesion formation were more pronounced following chronic TEMPOL treatment in hypoxia/SU5416 rats. Our findings support a major role for reactive oxygen species in mediating enhanced vasoconstrictor reactivity and pulmonary hypertension in both chronic hypoxia and hypoxia/SU5416 rat models

  1. Modeling of pharmacokinetics, efficacy, and hemodynamic effects of macitentan in patients with pulmonary arterial hypertension.

    Science.gov (United States)

    Krause, Andreas; Zisowsky, Jochen; Dingemanse, Jasper

    2018-04-01

    Macitentan is the first endothelin receptor antagonist with demonstrated efficacy on morbidity and mortality in pulmonary arterial hypertension (PAH) in the pivotal study SERAPHIN. The pharmacokinetics (PK) of macitentan and its active metabolite, ACT-132577, were characterized in a population model. Efficacy and hemodynamics (pharmacodynamics, PD) were related to PK based on PK/PD modeling. Sex, age, and body weight influenced the PK to a statistically significant extent. Model-based simulations showed that these variables are clinically not relevant. Concomitant use of PAH medication (PDE-5 inhibitors) did not influence macitentan trough concentration to a relevant extent. Efficacy and hemodynamics showed clear differences from placebo for macitentan concentrations on 3 and 10 mg with consistent superior effects for 10 mg. After 6 months, PAH patients showed model-predicted 6-min walk distance (6-MWD) improvements of 1.0 m on placebo compared to 29.8 and 34.1 m on 3 and 10 mg of macitentan, respectively. Higher macitentan concentrations were associated with reductions in pulmonary vascular resistance (PVR), mean right atrial and pulmonary arterial pressure, and total pulmonary resistance (TPR) and increases in cardiac index (CI) and mixed venous oxygen saturation. Statistical significance was determined for PVR, TPR, and CI but not for 6-MWD. In addition, PVR showed more pronounced differences between active treatment and placebo than 6-MWD. Modeling identified statistically significant inter-patient differences; simulations to assess the magnitude of the effects permitted clinical judgment. The same approach will allow for extrapolation to children. Hemodynamic markers might be better markers of treatment effects than 6-MWD. The SERAPHIN study and its open-label extension are registered with ClinicalTrials.gov with identifiers NCT00660179 (https://www.clinicaltrials.gov/ct2/show/NCT00660179) and NCT00667823 (https://clinicaltrials.gov/ct2/show

  2. Impact of Chronic Hypoxia on Proximal Pulmonary Artery Wave Propagation and Mechanical Properties in Rats.

    Science.gov (United States)

    Su, Junjing; Logan, Charmilie C; Hughes, Alun D; Parker, Kim H; Dhutia, Niti M; Danielsen, Carl Christian; Simonsen, Ulf

    2018-03-16

    Arterial stiffness and wave reflection are important components of the ventricular afterload. Therefore, we aimed to assess the arterial wave characteristics and mechanical properties of the proximal pulmonary arteries (PAs) in the hypoxic pulmonary hypertensive rat model. After 21 days in normoxic or hypoxic chambers (24 animals in each group), the animals underwent transthoracic echocardiography and pulmonary artery catheterization with a dual-tipped pressure and Doppler flow sensor wire. Wave intensity analysis (WIA) was performed. Artery rings obtained from the pulmonary trunk, right and left PAs and the aorta were subjected to a tensile test to rupture. Collagen and elastin content was determined. In hypoxic rats, proximal PA wall thickness, collagen content, tensile strength per unit collagen, maximal elastic modulus and wall viscosity increased; while the elastin:collagen ratio and arterial distensibility decreased. Arterial pulse wave velocity was also increased and the increase was more prominent in vivo than ex vivo. Wave intensity was similar in the hypoxic and normoxic animals with negligible wave reflection. In contrast, aortic maximal elastic modulus remained unchanged, while the wall viscosity decreased. There was no evidence of altered arterial wave propagation in the proximal PAs of hypoxic rats, while the extracellular matrix protein composition altered and the collagen tensile strength increased. This was accompanied by altered mechanical properties in vivo and ex vivo.

  3. [Regulatory role of calcium activated chloride channel in pulmonary vascular structural remodeling in rats with pulmonary arterial hypertension induced by high pulmonary blood flow].

    Science.gov (United States)

    Wang, K; Pang, Y S; Su, D Y; Ye, B B; Qin, S Y; Liu, D L; Han, Y L

    2016-09-01

    To explore the regulatory role of calcium activated chloride channel (CaCC) in vascular structural remodeling in pathogenesis of pulmonary arterial hypertension (PAH) induced by high pulmonary blood flow. An abdominal aorta and inferior vena cava shunting operation was used to induce high pulmonary blood flow and establish a PAH rat model.Seventy-five SD rats were randomly divided into normal, sham, shunt, niflumic acid (NFA) 1(0.2 mg/(kg·d))and NFA 2 (0.4 mg/(kg·d)) groups. There were 15 rats in each group. Pulmonary artery pressure and vascular structural remodeling were measured, arteriole contraction ratio among these groups were compared using vascular tone analysis system, and the electrophysiology of pulmonary artery smooth muscle cell (PASMC) was recorded using patch clamp technology. Differences between multiple groups were compared through variance analysis and that between groups with q test. Compared with normal ((14.4±1.3 ) mmHg, 1 mmHg=0.133 kPa)and sham groups ((13.5±2.3 ) mmHg), mean pulmonary artery pressure in shunt group ((27.4±2.4 ) mmHg) increased significantly (Ppulmonary artery pressure in NFA 1 group ((21.2±2.0) mmHg) and NFA 2 group ((22.3±2.0) mmHg) decreased significantly (PPulmonary vascular structural remodeling including pulmonary artery stenosis presented in shunt group. Compared with normal ((114.3±1.2)%) and sham ((115.5±1.1)%) groups, arteriole contraction ratio to 10(-5) mol/L phenylephrine in shunt group ((132.6±1.4)%) increased significantly (Ppulmonary vascular structural remodeling alleviated in NFA 1 and NFA 2 groups. Arteriole contraction ratio in NFA 1 group ((126.4±1.3)%) and NFA 2 group ((124.6±1.0)%) decreased significantly compared with shunt group (Ppulmonary arterial hypertension induced by high pulmonary blood flow through regulating membrane potential. NFA attenuate pulmonary vascular structural remodeling and pulmonary pressure through decreasing CaCC current density of PASMC membrane.

  4. Preoperative preparation of patients with arterial or pulmonary hypertension in noncardiac surgery.

    Science.gov (United States)

    Ivanović, Branislava; Tadić, Marijana; Marković, Dejan; Bradi, Zeljko; Janković, Radmilo; Kalezić, Nevena

    2011-01-01

    Arterial hypertension is not an independent risk factor in cardiovascular complications in noncardiac surgery. Nevertheless, preoperative evaluation is necessary and includes estimation of arterial hypertension grade and possible damage of target organs. In patients with first and second grade of arterial hypertension postponement of elective intervention is not necessary, only optimization of therapy. On the other hand, patients with third level arterial hypertension have benefit if intervention is postponed till the reduction of arterial pressure. There is no indication that any of the antihypertensive drug groups has advantage in the preoperative treatment of hypertension. Unlike arterial hypertension pulmonary hypertension increases the risk of cardiac morbidity and mortality in the perioperative period. In patients with pulmonary hypertension, anesthesia and surgery may be complicated with heart failure, hypoxia and arrhythmias. Preoperative and postoperative treatments include calcium channel blockers, prostanoids, endothelin receptor antagonists and inhibitors of phosphodiesterase type 5.

  5. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    International Nuclear Information System (INIS)

    Griffin, N.; Mansfield, L.; Redmond, K.C.; Dusmet, M.; Goldstraw, P.; Mittal, T.K.; Padley, S.

    2007-01-01

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution

  6. Primary pulmonary arterial hypertension with preserved right ventricular function leads to lower extremity venous insufficiency.

    Science.gov (United States)

    Aldemir, Mustafa; Emren, Sadık Volkan; Balçık, Çınar; Onrat, Ersel; Gürsoy, Merve

    2018-04-01

    Objectives Pulmonary hypertension with heart failure is related to venous insufficiency. However, there is no clear data whether pulmonary arterial hypertension with preserved right ventricular function cause venous insufficiency. In this study, we aim to investigate the relation between pulmonary arterial pressure with venous insufficiency in pulmonary arterial hypertension patients with preserved right ventricular function. Methods Between January 2012 and October 2014, 38 patients with a diagnosis of pulmonary arterial hypertension and 47 control group patients were included. Venous disability score and venous segmental disease score of both groups were calculated in order to measure venous insufficiency. The relationship between venous disability score and venous segmental disease scores and mean pulmonary arterial pressure and World Heart Organization functional capacity was examined. Results Total venous segmental disease score (5 ± 3.9 vs. 2 ± 1.8 p right venous segmental disease score (2.6 ± 2.2 vs. 1 ± 0.9 p disability scores (2.2 ±1 vs. 1.6 ± 0.7 p disability score was only weakly related (r = 0.343, p = 0.037). Total venous segmental disease score (r = 0.606, p disability scores (r = 0.601, p right ventricular function.

  7. Electron beam CT diagnosis of congenital unilateral absence of pulmonary artery

    International Nuclear Information System (INIS)

    Zhou Yuan; Dai Ruping; Cao Cheng; Zhang Gejun; Jing Baolian

    2003-01-01

    Objective: To evaluate the clinical value of electron beam CT (EBCT) in diagnosing congenital unilateral absence of pulmonary artery (UAPA). Methods: Patients with clinically suspected pulmonary artery disease or primary pulmonary hypertension underwent EBCT scanning. EBCT confirmed the diagnosis of UAPA in 11 patients, who were also evaluated with echocardiography and chest roentgenography. Cardioangiography and nuclear ventilation-perfusion scan were performed in some patients for a comparative study. Results: 4 female adults had UAPA with out associated congenital anomaly. 3 male children with coexisting complex congenital abnormality had unilateral absence of the left pulmonary artery and 4 patients coexisted other simple cardiovascular anomaly. EBCT scanning simultaneously displayed topographic pattern of both unilateral absence of pulmonary artery and coexisting congenital cardiovascular anomaly, as well as lung diseases. Conclusion: UAPA diagnosed in childhood usually has unilateral absence of the left pulmonary artery and associated congenital cardiovascular anomaly, while UAPA diagnosed in adult usually has UAPA on the right side without associated congenital anomaly. EBCT is one of the optimal imaging techniques in diagnosing UAPA and it greatly increases the diagnostic efficacy than echocardiography dose. Both EBCT and cardioangiography have their own advantages, however, EBCT, as a noninvasive method, should be complementary and not exclusive

  8. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Mansfield, L. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Redmond, K.C. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Dusmet, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Goldstraw, P. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Mittal, T.K. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-03-15

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution.

  9. Pulmonary Artery Occlusion and Mediastinal Fibrosis in a Patient on Dopamine Agonist Treatment for Hyperprolactinemia

    DEFF Research Database (Denmark)

    Su, Junjing; Simonsen, Ulf; Carlsen, Jørn

    2017-01-01

    Unusual forms of pulmonary hypertension include pulmonary hypertension related to mediastinal fibrosis and the use of serotonergic drugs. Here, we describe a patient with diffuse mediastinal fibrosis and pulmonary hypertension while she was on dopamine agonist therapy. A young woman, who...... and ventilation/perfusion (V/Q) scintigraphy, chronic thromboembolic pulmonary hypertension (CTEPH) was initially considered the most plausible diagnosis. However, during an attempted pulmonary endarterectomy, loose fibrous tissues were observed in the mediastinum and cryosection of the right pulmonary artery...... showed fibrosis and chronic inflammation. Subsequent investigations revealed that diffuse mediastinal fibrosis with concurrent pulmonary hypertension, and not CTEPH, was the most likely diagnosis and cabergoline and bromocriptine may have triggered the fibrotic changes. Both drugs are ergot...

  10. Adult Presentation of Right Lung Agenesis and Left Pulmonary Artery Sling

    International Nuclear Information System (INIS)

    Espinosa, L.; Agarwal, P.

    2008-01-01

    The combination of right lung agenesis and left pulmonary artery (LPA) sling is a rare entity that has been described only in the pediatric population. Cross-sectional imaging modalities such as magnetic resonance imaging (MRI) and computed tomography (CT) have an advantage over echocardiography and pulmonary angiography in demonstrating the anomalous left pulmonary artery, particularly in the presence of coexisting lung agenesis, as exemplified in this case. We report the first case of this rare entity in an adult. It is important to be aware that this abnormality, though rare, can present even in adulthood, and therefore close attention should be paid to the course of the pulmonary artery to ensure detection of a sling in association with lung agenesis

  11. Adult Presentation of Right Lung Agenesis and Left Pulmonary Artery Sling

    Energy Technology Data Exchange (ETDEWEB)

    Espinosa, L.; Agarwal, P. (Div. of Cardiothoracic Radiology, Dept. of Radiology, Univ. of Michigan, Ann Arbor, MI (US))

    2008-02-15

    The combination of right lung agenesis and left pulmonary artery (LPA) sling is a rare entity that has been described only in the pediatric population. Cross-sectional imaging modalities such as magnetic resonance imaging (MRI) and computed tomography (CT) have an advantage over echocardiography and pulmonary angiography in demonstrating the anomalous left pulmonary artery, particularly in the presence of coexisting lung agenesis, as exemplified in this case. We report the first case of this rare entity in an adult. It is important to be aware that this abnormality, though rare, can present even in adulthood, and therefore close attention should be paid to the course of the pulmonary artery to ensure detection of a sling in association with lung agenesis

  12. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA in an Old Adult

    Directory of Open Access Journals (Sweden)

    Maryam Esmaeilzadeh

    2011-09-01

    Full Text Available The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA is a rare congenital cardiac malformation. It presents predominantly in infancy and its main presenting feature is myocardial ischemia or heart failure. Survival to adulthood is quite uncommon. If untreated, mortality from ALCAPA approaches 90% in infancy; early recognition and surgical correction are, therefore, essential. With early surgical correction, the prognosis is good. There are two types of ALCAPA syndrome: the infant type and the adult type, each of which has different manifestations and outcomes. Infants experience myocardial infarction and congestive heart failure, and approximately 90% die within the first year of life. A literature review regarding this anomaly in teenagers and adults show that only 25 cases have been diagnosed during life and 18 additional cases of ALCAPA in these age groups have been diagnosed post mortem. We present a rare case of a 60-year-old man, who referred to our center due to dyspnea on exertion from the previous year without any history of chest pain and diagnosed as ALCAPA. Given the absence of ischemia and the patient’s age, only medical therapy was recommended.

  13. Pulmonary Artery Imaging in Patients with Chronic Thromboembolic Pulmonary Hypertension: Comparison of Cone-Beam CT and 64-Row Multidetector CT.

    Science.gov (United States)

    Hinrichs, Jan B; von Falck, Christian; Hoeper, Marius M; Olsson, Karen M; Wacker, Frank K; Meyer, Bernhard C; Renne, Julius

    2016-03-01

    To compare the depiction of pulmonary arteries in pulmonary arterial catheter-based contrast-enhanced cone-beam CT with peripheral intravenous contrast-enhanced multidetector CT in patients with suspected chronic thromboembolic pulmonary hypertension. In 20 patients (15 men and 5 women, 63.4 y ± 16.3), cone-beam CT using a catheter placed in the main pulmonary artery and 64-row multidetector CT using an appropriate venous access were performed. Contrast enhancement was measured in the main pulmonary artery, the right and left pulmonary arteries, and the left atrium. The amount of peripheral vessel conspicuity adjacent to the pleural surface (distance from vessel-to pleura) was measured. Two readers (R1, R2) independently evaluated the pulmonary arteries for image quality and pathologic findings in both modalities. Contrast density was higher in the main pulmonary artery and right and left pulmonary arteries (P cone-beam CT. The smallest distance between clearly delineated vessels and the pleura was significantly lower on cone-beam CT images (P cone-beam CT (κ = 0.79) and multidetector CT (κ = 0.78), whereas intermodality agreement was moderate (R1, κ = 0.60; R2, κ = 0.59). Both readers detected more weblike stenoses with cone-beam CT (76; 22%) compared with multidetector CT (25; 7%). Cone-beam CT shows improved contrast between pulmonary arteries and the left atrium and allows a more detailed depiction of the pulmonary arteries. Copyright © 2016 SIR. Published by Elsevier Inc. All rights reserved.

  14. Diagnostic Value of Transthoracic Echocardiography in Patients With Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery

    Science.gov (United States)

    Li, Rong-Juan; Sun, Zhonghua; Yang, Jiao; Yang, Ya; Li, Yi-Jia; Leng, Zhao-Ting; Liu, Guo-Wen; Pu, Li-Hong

    2016-01-01

    Abstract Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality associated with early infant mortality and sudden death in adults. Transthoracic echocardiography (TTE) plays an important role in early detection and diagnosis of ALCAPA as a noninvasive modality. However, its diagnostic value is not well studied. The purpose of this study is to determine the performance of TTE in the diagnostic assessment of ALCAPA as compared with coronary CT and invasive coronary angiography. A total of 22 patients (13 women and 9 men, mean age, 12.9 ± 19.5 years) with ALCAPA who underwent echocardiographic examination for clinical diagnosis were retrospectively reviewed and analyzed. Transthoracic echocardiographic features of ALCAPA were analyzed and its diagnostic value was compared with invasive coronary angiography and coronary CT angiography (CTA) with surgical findings serving as the gold standard. Surgery was performed in all of the patients to establish the dual coronary artery system. Five underwent the Takeuchi procedure and 17 had re-implantation of the anomalous left coronary artery. Of 20 patients, echocardiographic diagnoses were in good agreement with findings at surgery, resulting in the diagnostic accuracy of 90.9%. Two cases were misdiagnosed—one as the right coronary artery to pulmonary artery fistula and the other as rheumatic heart disease. The echocardiographic features of these patients with ALCAPA included: abnormal left coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow in 20 patients; enlargement of the right coronary artery in 17 patients; abundant intercoronary septal collaterals in 17 patients; and moderate and significant mitral regurgitation in 14 patients. The diagnostic accuracy of invasive coronary angiography (in 17 patients) and coronary CTA (in 9 patients) was 100%. This study shows that TTE is an accurate, noninvasive imaging modality

  15. Pulmonary Artery Dimensions as a Prognosticator of Transplant-Free Survival in Scleroderma Interstitial Lung Disease.

    Science.gov (United States)

    Gleason, James Benjamin; Patel, Krunal B; Hernandez, Felix; Hadeh, Anas; Highland, Kristin B; Rahaghi, Franck; Mehta, Jinesh P

    2017-08-01

    Systemic sclerosis is a chronic debilitating autoimmune disease characterized by endothelial dysfunction and multi-organ fibrosis. Interstitial lung disease, a common manifestation of SSc, is termed scleroderma-related interstitial lung disease (SSc-ILD) and along with pulmonary hypertension contributes to a majority of deaths in SSc. SSc-ILD patients frequently develop pulmonary hypertension, which prognosticates a poorer outcome. We investigated pulmonary artery dimensions as an outcome predictor in patients with SSc-ILD. A retrospective chart review abstracting data from SSc-ILD patients evaluated at a large tertiary care center was performed. HRCT imaging was reviewed and pulmonary artery (PA) and ascending aorta (Ao) diameters were measured for calculation of the PA:Ao ratio. Additionally, demographics, vital signs, spirometric parameters, comorbidities, and mean pulmonary artery pressures were collected when available. Outcome analysis with lung transplant or death events within 4 years based on pulmonary artery size as well as PA:Ao ratio was performed. 70 SSc-ILD patients were identified. Mean pulmonary artery diameter and PA:Ao ratio was 31.17 and 1.07 mm, respectively. Patients with a pulmonary artery diameter ≥32 mm had higher risk of lung transplantation or death (p < 0.001) within 4 years. Patients with a PA:Ao ratio ≥1.1 also had higher risk of lung transplantation or death (p < 0.001) within 4 years. Unadjusted outcomes analyses also identified PA:Ao ratio ≥1.1 as an independent outcome predictor (hazard ratio 3.30, p < 0.001). In SSc-ILD patients, a PA:Ao ratio ≥1.1 is associated with higher risk of lung transplant or death. These data suggest that PA:Ao dimension may be used for prognostication in SSc-ILD.

  16. THE RESTRICTED SURGICAL RELEVANCE OF MORPHOLOGIC CRITERIA TO CLASSIFY SYSTEMIC-PULMONARY COLLATERAL ARTERIES IN PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT

    NARCIS (Netherlands)

    DERUITER, MC; GITTENBERGERDEGROOT, AC; BOGERS, AJJC; ELZENGA, NJ

    1994-01-01

    Now that systemic-pulmonary collateral arteries are used for unifocalization in patients with pulmonary atresia and ventricular septal defect, the question arises whether morphologic criteria of these collateral arteries could help to provide better results. In an attempt to classify the morphologic

  17. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL).

    Science.gov (United States)

    Benza, Raymond L; Miller, Dave P; Gomberg-Maitland, Mardi; Frantz, Robert P; Foreman, Aimee J; Coffey, Christopher S; Frost, Adaani; Barst, Robyn J; Badesch, David B; Elliott, C Gregory; Liou, Theodore G; McGoon, Michael D

    2010-07-13

    Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use. Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance >32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men >60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique. We identified key predictors of survival based on the patient's most recent evaluation and formulated a contemporary prognostic equation. Use of this tool may allow the individualization and optimization of therapeutic strategies. Serial follow-up and reassessment are warranted. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00370214.

  18. Radionuclide scanning in a study of the pulmonary blood flow with the open arterial canal

    International Nuclear Information System (INIS)

    Rizaev, M.N.; Gulyamov, D.S.; Khodzhibekov, M.Kh.; Anvarov, M.A.

    1980-01-01

    Characteristic features of the distribution of the arterial pulmonary blood flow have been studied in 32 patients with the open arterial flow by lung scanning with albumin macroaggregate and 131 I. Research was conducted using the gamma-Ochamber ''Fo Gamma LFV'' and the scanner ''Magnaskaner 500I''. Disordered distribution of the pulmonary blood flow expressed in its unilateral decrease was detected in 17 patients (52.9%). A higher frequency of pulmonary hypertension was noted in these patients versus those with a relatively normal distribution of the pulmonary blood flow. A severe course of the disease was observed in high pulmonary hypertension combined with sharp suppression of the blood flow in one of the lungs or with signs of the shift from the right to the left side

  19. Architecture of fibrin network inside thrombotic material obtained from the right atrium and pulmonary arteries: flow and location matter.

    Science.gov (United States)

    Mazur, Piotr; Sobczyński, Robert; Ząbczyk, Michał; Babiarczyk, Paulina; Sadowski, Jerzy; Undas, Anetta

    2013-01-01

    Pulmonary embolectomy is a treatment option in selected patients with high-risk pulmonary embolism (PE). Efficiency of thrombus degradation in PE largely depends on the architecture of its fibrin network, however little is known about its determinants. We present the case of a 56-year-old woman with high-risk PE and proximal deep-vein thrombosis, whose thrombotic material removed during embolectomy from the right atrium and pulmonary (lobar and segmental) arteries has been studied using scanning electron microscopy (SEM). SEM images showed that distally located thrombi are richer in densely-packed fibrin fibers and contain more white cells and less erythrocytes than the proximal ones and the atrial thrombus. Fibrin fibers alignment along the flow vector was observed in the thrombi removed from high-velocity flow pulmonary arteries, and not in the atrial thrombus. The content of denser fibrin network and platelet aggregates was increased in segmental thromboemboli. Our findings describe the relation between thrombus architecture and location, and might help to elucidate thrombus resistance to anticoagulant therapy in some PE patients.

  20. Definition and classification of pulmonary hypertension.

    Science.gov (United States)

    Humbert, Marc; Montani, David; Evgenov, Oleg V; Simonneau, Gérald

    2013-01-01

    Pulmonary hypertension is defined as an increase of mean pulmonary arterial pressure ≥25 mmHg at rest as assessed by right heart catheterization. According to different combinations of values of pulmonary wedge pressure, pulmonary vascular resistance and cardiac output, a hemodynamic classification of pulmonary hypertension has been proposed. Of major importance is the pulmonary wedge pressure which allows to distinguish pre-capillary (pulmonary wedge pressure ≤15 mmHg) and post-capillary (pulmonary wedge pressure >15 mmHg) pulmonary hypertension. Pre-capillary pulmonary hypertension includes the clinical groups 1 (pulmonary arterial hypertension), 3 (pulmonary hypertension due to lung diseases and/or hypoxia), 4 (chronic thrombo-embolic pulmonary hypertension) and 5 (pulmonary hypertension with unclear and/or multifactorial mechanisms). Post-capillary pulmonary hypertension corresponds to the clinical group 2 (pulmonary hypertension due to left heart diseases).

  1. Emergency Endovascular Management of Pulmonary Artery Aneurysms In Behcet's Disease: Report of Two Cases and a Review of the Literature

    International Nuclear Information System (INIS)

    Cantasdemir, Murat; Kantarci, Fatih; Mihmanli, Ismail; Akman, Canan; Numan, Furuzan; Islak, Civan; Bozkurt, A. Kursat

    2002-01-01

    his report describes two patients with a known history of Behcet's disease in whom massive hemoptysis developed from rupture of pulmonary artery aneurysms. The high recurrence rate of complications related to pulmonary artery aneurysms and even the aneurysms themselves due to inadequacy of medical therapy and the disadvantages of surgical treatment make these aneurysms candidates for endovascular management.The pulmonary artery aneurysms reported here were successfully treated with endovascular embolization using n-butylcyanoacrylate. Pulmonary artery aneurysm embolization in Behcet's disease has been reviewed in the light of relevant literature

  2. Remodeling of the pulmonary artery induced by metastatic gastric carcinoma: a histopathological analysis of 51 autopsy cases

    International Nuclear Information System (INIS)

    Ishiwatari, Takao; Yamamoto, Yoshiro; Nakayama, Haruo; Shibuya, Kazutoshi; Okubo, Yoichiro; Tochigi, Naobumi; Wakayama, Megumi; Nemoto, Tetsuo; Kobayashi, Junko; Shinozaki, Minoru; Aki, Kyoko; Sasai, Daisuke

    2014-01-01

    Gastric carcinoma remains the second commonest cause of cancer deaths worldwide. Presence of the carcinoma cell in the pulmonary artery is serious condition that might cause remodeling of the pulmonary artery. The present study conducted detailed histopathological analyses to elucidate how gastric carcinoma cells may affect the structure and hemodynamics of pulmonary arteries. Remodeling of the pulmonary artery was assessed based on measurements of arterial diameters and stenosis rates from the autopsies, and their correlation were also validated. We additionally calculated 95 percent confidential intervals (CIs) for the rate of stenosis in groups of pulmonary arteries of different caliber zones (under 100, 100 to 300, and over 300 micrometer). The right ventricular thickness was measured and examined whether it correlated with the rate of pulmonary arterial stenosis. A total of 4612 autopsy cases were recorded at our institute, among which 168 had gastric carcinoma. Finally, 51 cases of the gastric carcinoma were employed for the study which had carcinoma cells in the lumen of the pulmonary artery. The mean right ventricular wall thickness of these cases was 3.14 mm. There were significant positive associations between the rates of pulmonary arterial stenosis and right ventricular thickness from pulmonary arteries of diameter under 100, 100 to 300, and over 300 micrometer. In these zones, 31, 31, and 33 cases had rates of pulmonary arterial stenosis that were below the lower limit of the 95 percent CI values, respectively. On the other hand, among cases with significant pulmonary stenosis, 17 of 18 cases with stenosis in the over 300 micrometer zone involved pulmonary arteries of both in the under 100 and 100 to 300 micrometer zones. One-third of autopsy with advanced gastric carcinoma had carcinoma cells in lumen of pulmonary artery, but implantation and proliferation may be essential to induce intimal thickening that causes an increasing of pulmonary arterial

  3. Comparing pulmonary resistance measured with an esophageal balloon to resistance measurements with an airflow perturbation device

    International Nuclear Information System (INIS)

    Coursey, D C; Johnson, A T; Scharf, S M

    2010-01-01

    The airflow perturbation device (APD) perturbs flow and mouth pressure during regular breathing. Ratios of mouth pressure perturbation magnitudes to flow perturbation magnitudes were used to calculate inspiratory, expiratory and average respiratory resistances. Resistance measurements with the APD were compared to pulmonary resistances directly measured with an esophageal balloon. Six healthy subjects were tested during tidal breathing when known external resistances were added during inspiration, during expiration and during both inspiration and expiration. When the baseline averaged balloon measured pulmonary resistance was subtracted from the baseline averaged APD measured resistance, the difference between them was 0.92 ± 1.25 (mean ± SD) cmH 2 O L –1 s –1 . Compared to the magnitude of the known increase in the added resistance, the APD measured resistance increased by 79%, whereas directly measured pulmonary resistance increased only by 56%. During addition of external resistances to both inspiration and expiration, the changes in inspiratory and expiratory pulmonary resistance were only 36% and 62% of the added resistance, respectively. On the other hand, the APD inhalation and exhalation resistance measured between 82% and 76% of the added resistance. We conclude that the APD detects changes in external resistance at least as well as, and probably better than, classical measurements of pulmonary resistance

  4. Systematic Review of the Economic Burden of Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Gu, Shuyan; Hu, Huimei; Dong, Hengjin

    2016-06-01

    Pulmonary arterial hypertension (PAH), as a life-threatening disease with no efficient cure, may impose a tremendous economic burden on patients and healthcare systems. However, most existing studies have mainly emphasised epidemiology and medications, while large observational studies reporting on the economic burden are currently lacking. To review and evaluate evidence on the costs of PAH and the cost effectiveness of PAH treatments, and to summarise the corresponding cost drivers. Systematic literature searches were conducted in English-language databases (PubMed, Web of Science, ScienceDirect) and Chinese-language databases (China National Knowledge Infrastructure, Wanfang Data, Chongqing VIP) to identify studies (published from 2000 to 2014) assessing the costs of PAH or the cost effectiveness of PAH treatments. The search results were independently reviewed and extracted by two reviewers. Costs were converted into 2014 US dollars. Of 1959 citations identified in the initial search, 19 papers were finally included in this analysis: eight on the economic burden of PAH and 11 on economic evaluation of PAH treatments. The economic burden on patients with PAH was rather large, with direct healthcare costs per patient per month varying from $2476 to $11,875, but none of the studies reported indirect costs. Sildenafil was universally reported to be a cost-effective treatment, with lower costs and better efficacy than other medications. Medical costs were reported to be the key cost drivers. The economic burden of patients with PAH is substantial, while the paucity of comprehensive country-specific evidence in this area and the lack of reports on indirect costs of PAH warrant researchers' concern, especially in China.

  5. T-helper 17 cell polarization in pulmonary arterial hypertension.

    Science.gov (United States)

    Hautefort, Aurélie; Girerd, Barbara; Montani, David; Cohen-Kaminsky, Sylvia; Price, Laura; Lambrecht, Bart N; Humbert, Marc; Perros, Frédéric

    2015-06-01

    Inflammation may contribute to the pathobiology of pulmonary arterial hypertension (PAH). Deciphering the PAH fingerprint on the inflammation orchestrated by dendritic cells (DCs) and T cells, key driver and effector cells, respectively, of the immune system, may allow the identification of immunopathologic approaches to PAH management. Using flow cytometry, we performed immunophenotyping of monocyte-derived DCs (MoDCs) and circulating lymphocytes from patients with idiopathic PAH and control subjects. With the same technique, we performed cytokine profiling of both populations following stimulation, coculture, or both. We tested the immunomodulatory effects of a glucocorticoid (dexamethasone [Dex]) on this immunophenotype and cytokine profile. Using an epigenetic approach, we confirmed the immune polarization in blood DNA of patients with PAH. The profile of membrane costimulatory molecules of PAH MoDCs was similar to that of control subjects. However, PAH MoDCs retained higher levels of the T-cell activating molecules CD86 and CD40 after Dex pretreatment than did control MoDCs. This was associated with an increased expression of IL-12p40 and a reduced migration toward chemokine (C-C motif) ligand 21. Moreover, both with and without Dex, PAH MoDCs induced a higher activation and proliferation of CD4+ T cells, associated with a reduced expression of IL-4 (T helper 2 response) and a higher expression of IL-17 (T helper 17 response). Purified PAH CD4+ T cells expressed a higher level of IL-17 after activation than did those of control subjects. Lastly, there was significant hypomethylation of the IL-17 promoter in the PAH blood DNA as compared with the control blood. We have highlighted T helper 17 cell immune polarization in patients with PAH, as has been previously demonstrated in other chronic inflammatory and autoimmune conditions.

  6. Effect of azithromycin in combination with simvastatin in the treatment of chronic obstructive pulmonary disease complicated by pulmonary arterial hypertension.

    Science.gov (United States)

    Wang, Peidong; Yang, Jie; Yang, Yanwei; Ding, Zhixin

    2017-01-01

    To evaluate the effect of azithromycin in combination with simvastatin in the treatment of chronic obstructive pulmonary disease (COPD) complicated by pulmonary arterial hypertension. Eighty-six patients who developed COPD and pulmonary arterial hypertension and received treatment from August 2013 to October 2014 were selected and randomly divided into an observation group and a control group using random number table, 43 in each group. Patients in the control group were orally administrated 20 mg of simvastatin, once a day. Patients in the observation group took 0.25g of azithromycin enteric-coated tablets, once a day, besides simvastatin. The treatment course of both groups was six months. Blood gas analysis indexes, forced expiratory volume in first second (FEV 1 ), six minutes walking distance, dyspnea grade and blood lipid parameter were recorded and compared between the two groups. Arterial partial pressure of oxygen (PaO 2 ) and arterial partial pressure of carbon dioxide (PaCO 2 ) of the observation group were (68.13±3.03) mmHg and (45.08±2.27) mmHg after treatment, respectively. In the control group, the values were (60.01±4.72) mmHg and (38.93±1.61) mmHg, respectively. The improvement amplitude of the observation group was superior to that of the control group ( P peripheral systolic blood pressure (PSBP) and peripheral diastolic blood pressure (PDBP) of patients in the observation group were both lower than those of the control group, and the difference had statistical significance ( P arterial hypertension as it can significantly relieve ventilation disturbance, improve lung function, and decrease pulmonary arterial pressure. Hence it is worth clinical promotion.

  7. Targeted drugs for pulmonary arterial hypertension: a network meta-analysis of 32 randomized clinical trials

    Directory of Open Access Journals (Sweden)

    Gao XF

    2017-05-01

    Full Text Available Xiao-Fei Gao,1 Jun-Jie Zhang,1,2 Xiao-Min Jiang,1 Zhen Ge,1,2 Zhi-Mei Wang,1 Bing Li,1 Wen-Xing Mao,1 Shao-Liang Chen1,2 1Department of Cardiology, Nanjing First Hospital, Nanjing Medical University, Nanjing, 2Department of Cardiology, Nanjing Heart Center, Nanjing, People’s Republic of China Background: Pulmonary arterial hypertension (PAH is a devastating disease and ultimately leads to right heart failure and premature death. A total of four classical targeted drugs, prostanoids, endothelin receptor antagonists (ERAs, phosphodiesterase 5 inhibitors (PDE-5Is, and soluble guanylate cyclase stimulator (sGCS, have been proved to improve exercise capacity and hemodynamics compared to placebo; however, direct head-to-head comparisons of these drugs are lacking. This network meta-analysis was conducted to comprehensively compare the efficacy of these targeted drugs for PAH.Methods: Medline, the Cochrane Library, and other Internet sources were searched for randomized clinical trials exploring the efficacy of targeted drugs for patients with PAH. The primary effective end point of this network meta-analysis was a 6-minute walk distance (6MWD.Results: Thirty-two eligible trials including 6,758 patients were identified. There was a statistically significant improvement in 6MWD, mean pulmonary arterial pressure, pulmonary vascular resistance, and clinical worsening events associated with each of the four targeted drugs compared with placebo. Combination therapy improved 6MWD by 20.94 m (95% confidence interval [CI]: 6.94, 34.94; P=0.003 vs prostanoids, and 16.94 m (95% CI: 4.41, 29.47; P=0.008 vs ERAs. PDE-5Is improved 6MWD by 17.28 m (95% CI: 1.91, 32.65; P=0.028 vs prostanoids, with a similar result with combination therapy. In addition, combination therapy reduced mean pulmonary artery pressure by 3.97 mmHg (95% CI: -6.06, -1.88; P<0.001 vs prostanoids, 8.24 mmHg (95% CI: -10.71, -5.76; P<0.001 vs ERAs, 3.38 mmHg (95% CI: -6.30, -0.47; P=0.023 vs

  8. Right ventricular septomarginal trabeculation hypertrophy is associated with disease severity in patients with pulmonary arterial hypertension.

    Science.gov (United States)

    Dong, Yang; Sun, Jiayu; Yang, Dan; He, Juan; Cheng, Wei; Wan, Ke; Liu, Hong; Greiser, Andreas; Zhou, Xiaoyue; Han, Yuchi; Chen, Yucheng

    2018-03-31

    To characterize the morphological change in the right ventricle (RV) of patients with pulmonary artery hypertension (PAH) and further explore the correlation between septomarginal trabeculation (SMT) and right ventricular (RV) function, myocardial fibrosis, and exercise capacity in patients with PAH. Sixty untreated PAH patients were prospectively included from May 2016 to April 2017. All patients underwent comprehensive clinical evaluation and cardiac magnetic resonance (CMR). The area and diameter of the basal segment of SMT, and the mass of SMT were measured on cine SSFP images. Relationship between parameters of SMT and RV ejection fraction (RVEF), 6 min walking distance (6MWD), myocardial fibrosis and pulmonary vascular resistance (PVR) were evaluated by Pearson's correlation and logistic regression. Predictive performance of SMT parameters for reduced RVEF or impaired 6MWD was evaluated by receiver operating characteristics (ROC) analysis. Compared with SMT diameter index and mass index, SMT area index (SMT Ai) in basal segment was the best parameter to show correlation with RVEF (r = - 0.496, P Ai had the strongest predictive value for reduced RVEF (AUC = 0.756), and impaired 6MWD (AUC = 0.813). SMT parameters were closely correlated with RV systolic function and 6MWD in patients with PAH. SMT Ai is a simple imaging indicator for the severity of PAH.

  9. Autologous Transfusion of Stored Red Blood Cells Increases Pulmonary Artery Pressure

    Science.gov (United States)

    Pinciroli, Riccardo; Stowell, Christopher P.; Wang, Lin; Yu, Binglan; Fernandez, Bernadette O.; Feelisch, Martin; Mietto, Cristina; Hod, Eldad A.; Chipman, Daniel; Scherrer-Crosbie, Marielle; Bloch, Kenneth D.; Zapol, Warren M.

    2014-01-01

    Rationale: Transfusion of erythrocytes stored for prolonged periods is associated with increased mortality. Erythrocytes undergo hemolysis during storage and after transfusion. Plasma hemoglobin scavenges endogenous nitric oxide leading to systemic and pulmonary vasoconstriction. Objectives: We hypothesized that transfusion of autologous blood stored for 40 days would increase the pulmonary artery pressure in volunteers with endothelial dysfunction (impaired endothelial production of nitric oxide). We also tested whether breathing nitric oxide before and during transfusion could prevent the increase of pulmonary artery pressure. Methods: Fourteen obese adults with endothelial dysfunction were enrolled in a randomized crossover study of transfusing autologous, leukoreduced blood stored for either 3 or 40 days. Volunteers were transfused with 3-day blood, 40-day blood, and 40-day blood while breathing 80 ppm nitric oxide. Measurements and Main Results: The age of volunteers was 41 ± 4 years (mean ± SEM), and their body mass index was 33.4 ± 1.3 kg/m2. Plasma hemoglobin concentrations increased after transfusion with 40-day and 40-day plus nitric oxide blood but not after transfusing 3-day blood. Mean pulmonary artery pressure, estimated by transthoracic echocardiography, increased after transfusing 40-day blood (18 ± 2 to 23 ± 2 mm Hg; P transfusing 3-day blood (17 ± 2 to 18 ± 2 mm Hg; P = 0.5). Breathing nitric oxide decreased pulmonary artery pressure in volunteers transfused with 40-day blood (17 ± 2 to 12 ± 1 mm Hg; P Transfusion of autologous leukoreduced blood stored for 40 days was associated with increased plasma hemoglobin levels and increased pulmonary artery pressure. Breathing nitric oxide prevents the increase of pulmonary artery pressure produced by transfusing stored blood. Clinical trial registered with www.clinicaltrials.gov (NCT 01529502). PMID:25162920

  10. Platelet-derived growth factor expression and function in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Perros, Frédéric; Montani, David; Dorfmüller, Peter; Durand-Gasselin, Ingrid; Tcherakian, Colas; Le Pavec, Jérôme; Mazmanian, Michel; Fadel, Elie; Mussot, Sacha; Mercier, Olaf; Hervé, Philippe; Emilie, Dominique; Eddahibi, Saadia; Simonneau, Gérald; Souza, Rogério; Humbert, Marc

    2008-07-01

    Platelet-derived growth factor (PDGF) promotes the proliferation and migration of pulmonary artery smooth muscle cells (PASMCs), and may play a role in the progression of pulmonary arterial hypertension (PAH), a condition characterized by proliferation of PASMCs resulting in the obstruction of small pulmonary arteries. To analyze the expression and pathogenic role of PDGF in idiopathic PAH. PDGF and PDGF receptor mRNA expression was studied by real-time reverse transcription-polymerase chain reaction performed on laser capture microdissected pulmonary arteries from patients undergoing lung transplantation for idiopathic PAH. Immunohistochemistry was used to localize PDGF, PDGF receptors, and phosphorylated PDGFR-beta. The effects of imatinib on PDGF-B-induced proliferation and chemotaxis were tested on human PASMCs. PDGF-A, PDGF-B, PDGFR-alpha, and PDGFR-beta mRNA expression was increased in small pulmonary arteries from patients displaying idiopathic PAH, as compared with control subjects. Western blot analysis revealed a significant increase in protein expression of PDGFR-beta in PAH lungs, as compared with control lungs. In small remodeled pulmonary arteries, PDGF-A and PDGF-B mainly localized to PASMCs and endothelial cells (perivascular inflammatory infiltrates, when present, showed intensive staining), PDGFR-alpha and PDGFR-beta mainly stained PASMCs and to a lesser extent endothelial cells. Proliferating pulmonary vascular lesions stained phosphorylated PDGFR-beta. PDGF-BB-induced proliferation and migration of PASMCs were inhibited by imatinib. This effect was not due to PASMC apoptosis. PDGF may play an important role in human PAH. Novel therapeutic strategies targeting the PDGF pathway should be tested in clinical trials.

  11. Vascular histopathologic reaction to pulmonary artery banding in an in vivo growing porcine model.

    Science.gov (United States)

    Nedorost, Lukáš; Uemura, Hideki; Furck, Anke; Saeed, Imran; Slavik, Zdenek; Kobr, Jiří; Tonar, Zbyněk

    2013-10-01

    Pulmonary artery banding (PAB) is used as a surgical palliation to reduce excessive pulmonary blood flow caused by congenital heart defects. Due to the lack of microscopic studies dealing with the tissue remodeling caused by contemporary PAB materials, this study aimed to assess histologic changes associated with PAB surgery by analyzing local tissue reaction to the presence of Gore-Tex strips fixed around the pulmonary artery. Gore-Tex strips were used for PAB in a growing porcine model. After 5 weeks, histologic samples with PAB (n = 5) were compared with healthy pulmonary arterial segments distal to the PAB or from a sham-treated animal (n = 1). Stereology was used to quantify the density of the vasa vasorum and the area fraction of elastin, smooth muscle actin, macrophages, and nervi vasorum within the pulmonary arterial wall. The null hypothesis stated that samples did not differ histopathologically from adjacent vascular segments or sham-treated samples. The PAB samples had a greater area fraction of macrophages, a lower amount of nervi vasorum, and a tendency toward decreased smooth muscle content compared with samples that had no PAB strips. There was no destruction of elastic membranes, no medionecrosis, no pronounced inflammatory infiltration or foreign body reaction, and no vasa vasorum deficiency after the PAB. All the histopathologic changes were limited to the banded vascular segment and did not affect distal parts of the pulmonary artery. The study results show the tissue reaction of palliative PAB and suggest that Gore-Tex strips used contemporarily for PAB do not cause severe local histologic damage to the banded segment of the pulmonary arterial wall after 5 weeks in a porcine PAB model.

  12. [Measurement of Rho-kinase in peripheral blood monocytes in patients with pulmonary arterial hypertension related to chronic obstructive pulmonary diseases].

    Science.gov (United States)

    Cai, Qian; Wu, Shangjie; Zhao, Xuefeng

    2012-05-01

    To determine effects of the RhoA/Rho kinase signaling pathway on patients with pulmonary arterial hypertension related to chronic obstructive pulmonary diseases by testing levels of Rho-associated coiled-coil containing protein kinase 1(ROCK1) in peripheral blood monocytes in healthy subjects, patients with chronic obstructive pulmonary diseases (COPD), and patients with pulmonary arterial hypertension related to COPD. Ten healthy subjects (Group A), 10 patients with COPD (Group B), and 10 patients with pulmonary arterial hypertension related to COPD (Group C) were enrolled, all of whom were hospitalized in the Third Hospital of Changsha between Dec. 2010 and Apr. 2011. Twenty milliliters of blood was collected from each subject. The peripheral blood mononuclear cells (PBMC) were separated by Percoll and, monocytes were incubated. Levels of ROCK1 in the three groups were measured by ELISA. The pulmonary function was measured by spirometric tests, and the pulmonary arterial systolic pressure (PASP) was detected by color Doppler echocardiogram. 1)The PASP in Group C was significantly higher than that of Groups A and B(P0.05). Rho kinase plays a key role in the pathogenesis of pulmonary arterial hypertension. The ROCK1 may be a marker of the severity of pulmonary arterial hypertension related to COPD.

  13. Is chronic obstructive pulmonary disease associated with increased arterial stiffness?

    DEFF Research Database (Denmark)

    Janner, Julie H; McAllister, David A; Godtfredsen, Nina S

    2012-01-01

    We hypothesize that airflow limitation is associated with increasing arterial stiffness and that having COPD increases a non-invasive measure of arterial stiffness - the aortic augmentation index (AIx) - independently of other CVD risk factors....

  14. Therapeutic Benefits of Induced Pluripotent Stem Cells in Monocrotaline-Induced Pulmonary Arterial Hypertension.

    Directory of Open Access Journals (Sweden)

    Wei-Chun Huang

    Full Text Available Pulmonary arterial hypertension (PAH is characterized by progressive increases in vascular resistance and the remodeling of pulmonary arteries. The accumulation of inflammatory cells in the lung and elevated levels of inflammatory cytokines in the bloodstream suggest that inflammation may play a role in PAH. In this study, the benefits of induced pluripotent stem cells (iPSCs and iPSC-conditioned medium (iPSC CM were explored in monocrotaline (MCT-induced PAH rats. We demonstrated that both iPSCs and iPSC CM significantly reduced the right ventricular systolic pressure and ameliorated the hypertrophy of the right ventricle in MCT-induced PAH rats in models of both disease prevention and disease reversal. In the prevention of MCT-induced PAH, iPSC-based therapy led to the decreased accumulation of inflammatory cells and down-regulated the expression of the IL-1β, IL-6, IL-12α, IL-12β, IL-23 and IFNγ genes in lung specimens, which implied that iPSC-based therapy may be involved in the regulation of inflammation. NF-κB signaling is essential to the inflammatory cascade, which is activated via the phosphorylation of the NF-κB molecule. Using the chemical inhibitor specifically blocked the phosphorylation of NF-κB, and in vitro assays of cultured human M1 macrophages implied that the anti-inflammation effect of iPSC-based therapy may contribute to the disturbance of NF-κB activation. Here, we showed that iPSC-based therapy could restore the hemodynamic function of right ventricle with benefits for preventing the ongoing inflammation in the lungs of MCT-induced PAH rats by regulating NF-κB phosphorylation.

  15. The dilatation of main pulmonary artery and right ventricle observed by enhanced chest computed tomography predict poor outcome in inoperable chronic thromboembolic pulmonary hypertension.

    Science.gov (United States)

    Ema, Ryogo; Sugiura, Toshihiko; Kawata, Naoko; Tanabe, Nobuhiro; Kasai, Hajime; Nishimura, Rintaro; Jujo, Takayuki; Shigeta, Ayako; Sakao, Seiichiro; Tatsumi, Koichiro

    2017-09-01

    Dilatation of the pulmonary artery and right ventricle on chest computed tomography images is often observed in patients with pulmonary hypertension. The clinical significance of these image findings has not been defined in chronic thromboembolic pulmonary hypertension. We investigated whether the pulmonary arterial and right ventricle dilatation was associated with poor outcome in chronic thromboembolic pulmonary hypertension. This was a retrospective cohort investigation in 60 subjects with inoperable chronic thromboembolic pulmonary hypertension diagnosed consecutively between 1997 and 2010 at Chiba University Hospital. Digital scout multi-detector chest computed tomography images were obtained. The main pulmonary arterial to ascending aortic diameter ratio and the right ventricular to left ventricular diameter ratio were calculated. Main pulmonary arterial to ascending aortic diameter ratio ranged from 0.85 to 1.84, and right ventricular to left ventricular diameter ratio ranged from 0.71 to 2.88. During the observation period of 1284.5days (range, 21-4550days), 13 patients required hospitalization due to worsening; 6 of them died. Kaplan-Meier analysis showed significant differences in hospitalization between the patients with main pulmonary arterial to ascending aortic diameter ratio of ≥1.1 and pulmonary hypertension. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  16. Dysregulated renin-angiotensin-aldosterone system contributes to pulmonary arterial hypertension

    Science.gov (United States)

    De Man, Frances; Tu, Ly; Handoko, Louis; Rain, Silvia; Ruiter, Gerrina; François, Charlène; Schalij, Ingrid; Dorfmüller, Peter; Simonneau, Gérald; Fadel, Elie; Perros, Frederic; Boonstra, Anco; Postmus, Piet; Van Der Velden, Jolanda; Vonk-Noordegraaf, Anton; Humbert, Marc; Eddahibi, Saadia; Guignabert, Christophe

    2012-01-01

    Rationale Patients with idiopathic pulmonary arterial hypertension (iPAH) often have a low cardiac output. To compensate, neurohormonal systems like renin-angiotensin-aldosterone system (RAAS) and sympathetic nervous system are upregulated but this may have long-term negative effects on the progression of iPAH. Objectives Assess systemic and pulmonary RAAS-activity in iPAH-patients and determine the efficacy of chronic RAAS-inhibition in experimental PAH. Measurements and Main Results We collected 79 blood samples from 58 iPAH-patients in the VU University Medical Center Amsterdam (between 2004–2010), to determine systemic RAAS-activity. We observed increased levels of renin, angiotensin (Ang) I and AngII, which was associated with disease progression (p<0.05) and mortality (p<0.05). To determine pulmonary RAAS-activity, lung specimens were obtained from iPAH-patients (during lung transplantation, n=13) and controls (during lobectomy or pneumonectomy for cancer, n=14). Local RAAS-activity in pulmonary arteries of iPAH-patients was increased, demonstrated by elevated ACE-activity in pulmonary endothelial cells and increased AngII type 1 (AT1) receptor expression and signaling. In addition, local RAAS- upregulation was associated with increased pulmonary artery smooth muscle cell proliferation via enhanced AT1-receptor signaling in iPAH-patients compared to controls. Finally, to determine the therapeutic potential of RAAS-activity, we assessed the chronic effects of an AT1-receptor antagonist (losartan) in the monocrotaline PAH-rat model (60 mg/kg). Losartan delayed disease progression, decreased RV afterload and pulmonary vascular remodeling and restored right ventricular-arterial coupling in PAH-rats. Conclusions Systemic and pulmonary RAAS-activities are increased in iPAH-patients and associated with increased pulmonary vascular remodeling. Chronic inhibition of RAAS by losartan is beneficial in experimental PAH. PMID:22859525

  17. Surgical Treatment of Complications of Pulmonary Tuberculosis, including Drug-Resistant Tuberculosis

    Directory of Open Access Journals (Sweden)

    Rajhmun Madansein

    2015-03-01

    Full Text Available Surgery for drug-resistant tuberculosis has been shown to be safe and effective, with similar level of mortalities associated with surgical intervention observed with that for lung cancer. While surgery has been an option to treat TB in the pre-antibiotic era, it is now increasingly used to treat complications of pulmonary TB, particularly in patients with drug-resistant TB who do not respond to medical treatment. The two most frequent indications for lung resection in drug- resistant TB, are i failed medical treatment with persistent sputum positivity or ii patients who have had medical treatment and are sputum negative, but with persistent localized cavitary disease or bronchiectasis. Massive hemoptysis is a potentially life-threatening complication of TB. Lung resection is potentially curative in patients with massive hemoptysis and cavitary or bronchiectatic disease. Bronchial artery embolization in these patients has a high success rate but bears also the risk of recurrence. Lung resection can be safely undertaken in selected patients with HIV co-infection and pulmonary complications of TB. Ambulatory drainage is a novel, safe, affordable and effective method of draining a chronic TB associated empyema thoracis. We review here the current surgical treatment of the complications of pulmonary TB and discuss the experience from the Durban Cardiothoracic Surgery Unit for the surgical treatment of patients with complicated pulmonary TB.

  18. Inhaled nitric oxide and arterial oxygen tension in patients with chronic obstructive pulmonary disease and severe pulmonary hypertension

    Science.gov (United States)

    Katayama, Y.; Higenbottam, T. W.; d Diaz; Cremona, G.; Akamine, S.; Barbera, J. A.; Rodriguez-Roisin, R.

    1997-01-01

    BACKGROUND: Inhaled nitric oxide (NO) is a selective pulmonary vasodilator which can improve gas exchange in acute lung injury. However, it is uncertain that this effect on arterial oxygenation can be generalised to all lung diseases. METHODS: The effects of inhaled NO on gas exchange were studied in nine patients with chronic obstructive pulmonary disease (COPD), 11 patients with severe pulmonary hypertension, and 14 healthy volunteers. A randomized sequence of 40 ppm of NO or air was inhaled for 20 minutes through an orofacial mask. RESULTS: Inhaled NO reduced mean (SE) transcutaneous arterial oxygen tension (TcPO2) from 9.6 (0.3) to 8.9 (0.4) kPa in healthy volunteers and from 7.4 (0.6) to 7.0 (0.5) kPa in patients with COPD. There was no change in TcPO2 in patients with severe pulmonary hypertension. During inhalation of NO and air no change occurred in transcutaneous arterial carbon dioxide tension (TcPCO2), arterial oxygen saturation (SaO2) measured by pulse oximeter, or cardiac output determined by the transthoracic impedance method. CONCLUSIONS: Inhaled NO does not improve TcPO2 nor increase cardiac output in normal subjects and patients with COPD, suggesting that inhaled NO worsens gas exchange. This could represent inhaled NO overriding hypoxic pulmonary vasoconstriction in COPD. The finding that TcPO2 also fell when normal subjects inhaled NO suggests that a similar mechanism normally contributes to optimal gas exchange. Whilst inhaled NO can improve oxygenation, this effect should not be considered to be a general response but is dependent on the type of lung disease. 


 PMID:9059470

  19. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

    Energy Technology Data Exchange (ETDEWEB)

    Greenberg, S.B.; Crisci, K.L.; Koenig, P.; Robinson, B.; Anisman, P.; Russo, P. [St. Christopher`s Hospital for Children, Front Street at Erie Avenue, Philadelphia, PA 19134 (United States)

    1997-12-01

    Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. (orig.) With 2 figs., 3 tabs., 11 refs.

  20. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

    International Nuclear Information System (INIS)

    Greenberg, S.B.; Crisci, K.L.; Koenig, P.; Robinson, B.; Anisman, P.; Russo, P.

    1997-01-01

    Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. (orig.)

  1. Pleural opening impairs respiratory system compliance and resistance in off-pump coronary artery bypass grafting.

    Science.gov (United States)

    Tavolaro, K C; Guizilini, S; Bolzan, D W; Dauar, R B; Buffolo, E; Succi, J E; Gomes, W J

    2010-12-01

    This study evaluated the effect of pleurotomy on respiratory system compliance and resistance in off-pump coronary artery bypass (OPCAB) using the left internal thoracic artery (LITA). Thirty-two patients were prospectively allocated into two groups: OP group (n = 16 patients with open left pleural cavity); IP group (N.=16 patients with intact pleural cavity). Static and dynamic lung compliance and total respiratory system resistance calculation were recorded at anesthesia induction (before chest opening) and immediately after chest closure. Static lung compliance values significantly decreased after chest closure in both groups (P tube insertion induced significant reduction in static lung compliance and increase in total respiratory system resistance, furthermore contributing to impair pulmonary dysfunction in the early postoperative period after OPCAB.

  2. Aberrant Chloride Intracellular Channel 4 Expression Contributes to Endothelial Dysfunction in Pulmonary Arterial Hypertension

    Science.gov (United States)

    Wojciak-Stothard, Beata; Abdul-Salam, Vahitha B.; Lao, Ka Hou; Tsang, Hilda; Irwin, David C.; Lisk, Christina; Loomis, Zoe; Stenmark, Kurt R.; Edwards, John C; Yuspa, Stuart H.; Howard, Luke S.; Edwards, Robert J.; Rhodes, Christopher J.; Gibbs, J Simon R.; Wharton, John; Zhao, Lan; Wilkins, Martin R.

    2014-01-01

    Background Chloride intracellular channel 4 (CLIC4) is highly expressed in the endothelium of remodelled pulmonary vessels and plexiform lesions of patients with pulmonary arterial hypertension (PAH). CLIC4 regulates vasculogenesis through endothelial tube formation. Aberrant CLIC4 expression may contribute to the vascular pathology of PAH. Methods and Results CLIC4 protein expression was increased in plasma and blood-derived endothelial cells from patients with idiopathic PAH (IPAH) and in the pulmonary vascular endothelium of 3 rat models of pulmonary hypertension. CLIC4 gene deletion markedly attenuated the development of chronic hypoxia-induced pulmonary hypertension in mice. Adenoviral overexpression of CLIC4 in cultured human pulmonary artery endothelial cells compromised pulmonary endothelial barrier function and enhanced their survival and angiogenic capacity, while CLIC4 shRNA had an inhibitory effect. Similarly, inhibition of CLIC4 expression in blood-derived endothelial cells from patients with IPAH attenuated the abnormal angiogenic behaviour that characterises these cells. The mechanism of CLIC4 effects involves p65-mediated activation of nuclear factor-κB, followed by stabilisation of hypoxia-inducible factor-1α and increased downstream production of vascular endothelial growth factor and endothelin-1. Conclusions Increased CLIC4 expression is an early manifestation and mediator of endothelial dysfunction in pulmonary hypertension. PMID:24503951

  3. Congenital anomalous/aberrant systemic artery to pulmonary venous fistula: Closure with vascular plugs & coil embolization

    Directory of Open Access Journals (Sweden)

    Pankaj Jariwala

    2014-01-01

    Full Text Available A 7-month-old girl with failure to thrive, who, on clinical and diagnostic evaluation [echocardiography & CT angiography] to rule out congenital heart disease, revealed a rare vascular anomaly called systemic artery to pulmonary venous fistula. In our case, there was dual abnormal supply to the entire left lung as1 anomalous supply by normal systemic artery [internal mammary artery]2 and an aberrant feeder vessel from the abdominal aorta. Left Lung had normal bronchial connections and normal pulmonary vasculature. The fistula drained through the pulmonary veins to the left atrium leading to ‘left–left shunt’. Percutaneous intervention in two stages was performed using Amplatzer vascular plugs and coil embolization to close them successfully. The patient gained significant weight in follow up with other normal developmental and mental milestones.

  4. The mechanical properties of the systemic and pulmonary arteries of Python regius correlate with blood pressures.

    Science.gov (United States)

    van Soldt, Benjamin J; Danielsen, Carl Christian; Wang, Tobias

    2015-12-01

    Pythons are unique amongst snakes in having different pressures in the aortas and pulmonary arteries because of intraventricular pressure separation. In this study, we investigate whether this correlates with different blood vessel strength in the ball python Python regius. We excised segments from the left, right, and dorsal aortas, and from the two pulmonary arteries. These were subjected to tensile testing. We show that the aortic vessel wall is significantly stronger than the pulmonary artery wall in P. regius. Gross morphological characteristics (vessel wall thickness and correlated absolute amount of collagen content) are likely the most influential factors. Collagen fiber thickness and orientation are likely to have an effect, though the effect of collagen fiber type and cross-links between fibers will need further study. © 2015 Wiley Periodicals, Inc.

  5. Erythropoietin attenuates pulmonary vascular remodeling in experimental pulmonary arterial hypertension through interplay between endothelial progenitor cells and heme-oxygenase

    Directory of Open Access Journals (Sweden)

    Rosa L.E. Loon

    2015-08-01

    Full Text Available BackgroundPulmonary arterial hypertension (PAH is a pulmonary vascular disease with a high mortality, characterized by typical angio-proliferative lesions. Erythropoietin (EPO attenuates pulmonary vascular remodeling in PAH. We postulated that EPO acts through mobilization of endothelial progenitor cells (EPCs and activation of the cytoprotective enzyme heme oxygenase-1 (HO1.MethodsRats with flow-associated PAH, resembling pediatric PAH, were treated with HO-1 inducer EPO in the presence or absence of the selective HO-activity-inhibitor tin-mesoporphyrin (SnMP. HO-activity, circulating EPCs and pulmonary vascular lesions were assessed after 3 weeks.ResultsIn PAH-rats, circulating EPCs were decreased and HO-activity was increased compared to control. EPO-treatment restored circulating EPCs and improved pulmonary vascular remodeling, as shown by a reduced wall thickness and occlusion rate of the intra-acinar vessels. Inhibition of HO-activity with SnMP aggravated PAH. Moreover, SnMP treatment abrogated EPO-induced amelioration of pulmonary vascular remodeling, while surprisingly further increasing circulating EPCs as compared with EPO alone.ConclusionsIn experimental PAH, EPO treatment restored the number of circulating EPC’s to control level, improved pulmonary vascular remodeling, and showed important interplay with HO-activity. Inhibition of increased HO-activity in PAH-rats exacerbated progression of pulmonary vascular remodeling, despite the presence of restored numbers of circulating EPC’s. We suggest that both EPO-induced HO1 and EPCs are promising targets to ameliorate the pulmonary vasculature in PAH.

  6. Erythrocyte deformability and segmental pulmonary vascular resistance: osmolarity and heat treatment.

    Science.gov (United States)

    Hakim, T S

    1988-10-01

    The site and nature of change in resistance to blood flow in canine left lung lobe preparation after changes in blood viscosity were assessed by using the arterial and venous occlusion (AVO) technique and the vascular pressure-flow relationship. Blood viscosity was changed by erythrocyte (RBC) shrinkage and swelling with hypertonic and hypotonic NaCl solutions and by RBC membrane rigidification with heat treatment (49 degrees C for 1 h). The results show that although all three methods of changing blood viscosity increased the pulmonary vascular resistance (PVR) by 15-50%, the site and nature of the change in PVR were different in each case. The AVO data showed that the increase in PVR with heat treatment of RBC's was due entirely (100%) to increased resistance of the middle microvascular segment, whereas deviation from normal osmolarity potentiated the resistance in arterial, middle, and venous segments. By examining the effect of osmolarity in plasma-perfused lobes, it was possible to separate the increase in PVR due to changes in RBC deformability from those due to other factors. The increase in arterial and venous resistances with hypertonic solution was attributed in part (approximately 50%) to factors other than RBC's; however, the increase in middle resistance was entirely due to RBC crenation. The increase in arterial and venous resistances with hypotonic solutions was small and was apparently caused by factors other than RBC swelling, whereas the increase in middle resistance was partially (approximately 50%) due to RBC swelling and partially to other factors (e.g., endothelial cell hydration).(ABSTRACT TRUNCATED AT 250 WORDS)

  7. Health-related quality of life in patients with pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Christie Jason

    2005-08-01

    Full Text Available Abstract Background Improved outcomes with expanding treatment options for patients with pulmonary arterial hypertension present the opportunity to consider additional end-points in approaching therapy, including factors that influence health-related quality of life. However, comparatively little is known about health-related quality of life and its determinants in patients with pulmonary arterial hypertension. Methods Health-related quality of life was evaluated in a cross sectional study of 155 outpatients with pulmonary arterial hypertension using generic and respiratory-disease specific measurement tools. Most patients had either World Health Organization functional Class II or III symptoms. Demographic, hemodynamic and treatment variables were assessed for association with health-related quality of life scores. Results Patients with pulmonary arterial hypertension suffered severe impairments in both physical and emotional domains of health-related quality of life. Patients with idiopathic ("primary" pulmonary arterial hypertension had the best, and those with systemic sclerosis the worst health-related quality of life. Greater six-minute walk distance correlated with better health-related quality of life scores, as did functional Class II versus Class III symptoms. Hemodynamic measurements, however, did not correlate with health-related quality of life scores. No differences in health-related quality of life were found between patients who were being treated with calcium channel antagonists, bosentan or continuously infused epoprostenol at the time of quality of life assessment. Conclusion Health-related quality of life is severely impaired in patients with pulmonary arterial hypertension and is associated with measures of functional status. Specific associations with impaired health-related quality of life suggest potential areas for targeted intervention.

  8. Mechanisms of endothelium-dependent relaxation evoked by anandamide in isolated human pulmonary arteries

    OpenAIRE

    Baranowska-Kuczko, Marta; Kozłowska, Hanna; Kozłowski, Mirosław; Schlicker, Eberhard; Kloza, Monika; Surażyński, Arkadiusz; Grzęda, Emilia; Malinowska, Barbara

    2014-01-01

    Endocannabinoids contract, relax or do not affect vessels with different calibre and tone in the pulmonary circulation in four species. The aim of the present study was to determine the mechanisms involved in the anandamide-induced relaxation of human pulmonary arteries (hPAs). Studies were performed in the isolated hPAs pre-constricted with the prostanoid TP receptor agonist, U-46619. To detect fatty acid amide hydrolase (FAAH) expression, Western blots were used. Anandamide concentration de...

  9. In Pulmonary Arterial Hypertension, Reduced BMPR2 Promotes Endothelial-to-Mesenchymal Transition via HMGA1 and Its Target Slug

    NARCIS (Netherlands)

    Hopper, Rachel K.; Moonen, Jan-Renier A. J.; Diebold, Isabel; Cao, Aiqin; Rhodes, Christopher J.; Tojais, Nancy F.; Hennigs, Jan K.; Gu, Mingxia; Wang, Lingli; Rabinovitch, Marlene

    2016-01-01

    Background-We previously reported high-throughput RNA sequencing analyses that identified heightened expression of the chromatin architectural factor High Mobility Group AT-hook 1 (HMGA1) in pulmonary arterial endothelial cells (PAECs) from patients who had idiopathic pulmonary arterial hypertension

  10. Decreased creatine kinase is linked to diastolic dysfunction in rats with right heart failure induced by pulmonary artery hypertension

    NARCIS (Netherlands)

    Fowler, Ewan D.; Benoist, David; Drinkhill, Mark J.; Stones, Rachel; Helmes, Michiel; Wüst, Rob C. I.; Stienen, Ger J. M.; Steele, Derek S.; White, Ed

    2015-01-01

    Our objective was to investigate the role of creatine kinase in the contractile dysfunction of right ventricular failure caused by pulmonary artery hypertension. Pulmonary artery hypertension and right ventricular failure were induced in rats by monocrotaline and compared to saline-injected control

  11. Development of Occlusive Neointimal Lesions in Distal Pulmonary Arteries of Endothelin B Receptor–Deficient Rats: A New Model of Severe Pulmonary Arterial Hypertension

    Science.gov (United States)

    Ivy, D. Dunbar; McMurtry, Ivan F.; Colvin, Kelley; Imamura, Masatoshi; Oka, Masahiko; Lee, Dong-Seok; Gebb, Sarah; Jones, Peter Lloyd

    2007-01-01

    Background Human pulmonary arterial hypertension (PAH) is characterized by proliferation of vascular smooth muscle and, in its more severe form, by the development of occlusive neointimal lesions. However, few animal models of pulmonary neointimal proliferation exist, thereby limiting a complete understanding of the pathobiology of PAH. Recent studies of the endothelin (ET) system demonstrate that deficiency of the ETB receptor predisposes adult rats to acute and chronic hypoxic PAH, yet these animals fail to develop neointimal lesions. Herein, we determined and thereafter showed that exposure of ETB receptor–deficient rats to the endothelial toxin monocrotaline (MCT) leads to the development of neointimal lesions that share hallmarks of human PAH. Methods and Results The pulmonary hemodynamic and morphometric effects of 60 mg/kg MCT in control (MCT+/+) and ETB receptor–deficient (MCTsl/sl) rats at 6 weeks of age were assessed. MCTsl/sl rats developed more severe PAH, characterized by elevated pulmonary artery pressure, diminished cardiac output, and right ventricular hypertrophy. In MCTsl/sl rats, morphometric evaluation revealed the presence of neointimal lesions within small distal pulmonary arteries, increased medial wall thickness, and decreased arterial-to-alveolar ratio. In keeping with this, barium angiography revealed diminished distal pulmonary vasculature of MCTsl/sl rat lungs. Cells within neointimal lesions expressed smooth muscle and endothelial cell markers. Moreover, cells within neointimal lesions exhibited increased levels of proliferation and were located in a tissue microenvironment enriched with vascular endothelial growth factor, tenascin-C, and activated matrix metalloproteinase-9, factors already implicated in human PAH. Finally, assessment of steady state mRNA showed that whereas expression of ETB receptors was decreased in MCTsl/sl rat lungs, ETA receptor expression increased. Conclusions Deficiency of the ETB receptor markedly

  12. The Effect of Umbilical Cord Blood Derived Mesenchymal Stem Cells in Monocrotaline-induced Pulmonary Artery Hypertension Rats.

    Science.gov (United States)

    Lee, Hyeryon; Lee, Jae Chul; Kwon, Jung Hyun; Kim, Kwan Chang; Cho, Min-Sun; Yang, Yoon Sun; Oh, Wonil; Choi, Soo Jin; Seo, Eun-Seok; Lee, Sang-Joon; Wang, Tae Jun; Hong, Young Mi

    2015-05-01

    Pulmonary arterial hypertension (PAH) causes right ventricular failure due to a gradual increase in pulmonary vascular resistance. The purposes of this study were to confirm the engraftment of human umbilical cord blood-mesenchymal stem cells (hUCB-MSCs) placed in the correct place in the lung and research on changes of hemodynamics, pulmonary pathology, immunomodulation and several gene expressions in monocrotaline (MCT)-induced PAH rat models after hUCB-MSCs transfusion. The rats were grouped as follows: the control (C) group; the M group (MCT 60 mg/kg); the U group (hUCB-MSCs transfusion). They received transfusions via the external jugular vein a week after MCT injection. The mean right ventricular pressure (RVP) was significantly reduced in the U group after the 2 week. The indicators of RV hypertrophy were significantly reduced in the U group at week 4. Reduced medial wall thickness in the pulmonary arteriole was noted in the U group at week 4. Reduced number of intra-acinar muscular pulmonary arteries was observed in the U group after 2 week. Protein expressions such as endothelin (ET)-1, endothelin receptor A (ERA), endothelial nitric oxide synthase (eNOS) and matrix metalloproteinase (MMP)-2 significantly decreased at week 4. The decreased levels of ERA, eNOS and MMP-2 immunoreactivity were noted by immnohistochemical staining. After hUCB-MSCs were administered, there were the improvement of RVH and mean RVP. Reductions in several protein expressions and immunomodulation were also detected. It is suggested that hUCB-MSCs may be a promising therapeutic option for PAH.

  13. Anomalous renal artery is potential cause of resistant hypertension ...

    African Journals Online (AJOL)

    Background: Drug-resistant hypertension can be attributable to secondary hypertension and other causes. Anomalous renal artery is uncommon but can be a potential cause of resistant hypertension. Case Report: We highlight the challenges in management of resistant hypertension and describe its unusual association ...

  14. Detection of experimentally produced acute pulmonary arterial occlusion by methyl iodide-131 inhalation imaging

    International Nuclear Information System (INIS)

    Grossman, Z.D.; McAfee, J.G.; Subramanian, G.

    1981-01-01

    Methyl iodide-131 (CH 3 I-131) is described as an agent for detection of acute experimentally produced pulmonary arterial occlusion in dogs. When gaseous CH 3 I-131 is inhaled, radioactivity passes instantaneously from the alveoli to the lung capillary bed. Where pulmonary blood flow exists, activity is washed out into the systemic circulation, but in areas of blood stasis, a transient pulmonary hot spot remains. CH 3 I-131 is easily produced and inexpensive, but administration is awkward and strict radiation safety precautions are mandatory

  15. Ruptured penetrating ulcer of the ascending aorta with pulmonary artery compression.

    Science.gov (United States)

    Okiwelu, Ngozichukwuka; Finn, Chris; Vanden Driesen, Rohan; Sanders, Lucas; Joshi, Pragnesh

    2016-03-01

    Pulmonary artery involvement has been reported in various degrees of complicated dissection of the ascending aorta. The prognosis remains poor without high-risk surgical intervention, but conservative management can be considered in high-risk cases. We report a case of nonoperative management of an octogenarian who presented with a contained rupture of his proximal ascending aorta, likely from a penetrating atherosclerotic ulcer. It was complicated by extrinsic compression of the pulmonary trunk and transient pulmonary hypertension without features of acute right heart failure. He remained alive at the one-year follow-up. © The Author(s) 2014.

  16. The Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA: a Case Series and Brief Review

    Directory of Open Access Journals (Sweden)

    Aliasghar Moeinipour

    2016-02-01

    Full Text Available Background Anomalous left coronary artery from the pulmonary artery (ALCAPA is a rare congenital cardiovascular defect that occurs in approximately 1/300 000 live births or 0.5% of children with congenital heart disease. There are two types of ALCAPA syndrome: the infant type and the adult type. The most infants experience myocardial infarction and congestive heart failure, and approximately 90% die within the first year of life; also, without early surgical intervention they have a dismal prognosis. Materials and Methods We report 3- year experiences from January 2013 to January 2016 of Imam Reza Hospital center (a tertiary referral hospital North East of Iran that consist of all patients with ALCAPA syndrome. Results The Takeuchi procedure, were successfully performed in five children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA. There was no death and significant mitral regurgitation postoperative (n=0 in this short study. All of patients (n=5 had evidence of improving ischemic myocardium status by increasing of ejection fraction and regional wall motion of left ventricular in follow up echocardiography. Conclusion The only cure treatment for ALCAPA syndrome is surgical intervention that needs to be performed immediately after diagnosis to prevent myocardial infarction and chronic heart failure. Today, establishing a system with two coronary arteries is the goal in definitive surgical repair. The Takeuchi procedure is a prefer method to establish a two-coronary repair for ALCAPA.

  17. Obesity-related pulmonary arterial hypertension in rats correlates with increased circulating inflammatory cytokines and lipids and with oxidant damage in the arterial wall but not with hypoxia.

    Science.gov (United States)

    Irwin, David C; Garat, Chrystelle V; Crossno, Joseph T; MacLean, Paul S; Sullivan, Timothy M; Erickson, Paul F; Jackman, Matthew R; Harral, Julie W; Reusch, Jane E B; Klemm, Dwight J

    2014-12-01

    Obesity is causally linked to a number of comorbidities, including cardiovascular disease, diabetes, renal dysfunction, and cancer. Obesity has also been linked to pulmonary disorders, including pulmonary arterial hypertension (PAH). It was long believed that obesity-related PAH was the result of hypoventilation and hypoxia due to the increased mechanical load of excess body fat. However, in recent years it has been proposed that the metabolic and inflammatory disturbances of obesity may also play a role in the development of PAH. To determine whether PAH develops in obese rats in the absence of hypoxia, we assessed pulmonary hemodynamics and pulmonary artery (PA) structure in the diet-resistant/diet-induced obesity (DR/DIO) and Zucker lean/fatty rat models. We found that high-fat feeding (DR/DIO) or overfeeding (Zucker) elicited PA remodeling, neomuscularization of distal arterioles, and elevated PA pressure, accompanied by right ventricular (RV) hypertrophy. PA thickening and distal neomuscularization were also observed in DIO rats on a low-fat diet. No evidence of hypoventilation or chronic hypoxia was detected in either model, nor was there a correlation between blood glucose or insulin levels and PAH. However, circulating inflammatory cytokine levels were increased with high-fat feeding or calorie overload, and hyperlipidemia and oxidant damage in the PA wall correlated with PAH in the DR/DIO model. We conclude that hyperlipidemia and peripheral inflammation correlate with the development of PAH in obese subjects. Obesity-related inflammation may predispose to PAH even in the absence of hypoxia.

  18. “Denervation” of autonomous nervous system in idiopathic pulmonary arterial hypertension by low-dose radiation: a case report with an unexpected outcome

    Directory of Open Access Journals (Sweden)

    Hohenforst-Schmidt W

    2014-03-01

    Full Text Available Wolfgang Hohenforst-Schmidt,1 Paul Zarogoulidis,2 Filiz Oezkan,3 Christian Mahnkopf,4 Gerhard Grabenbauer,5 Alfons Kreczy,6 Rolf Bartunek,7 Kaid Darwiche,3 Lutz Freitag,3 Qiang Li,8 Haidong Huang,8 Thomas Vogl,9 Patrick LePilvert,10 Theodora Tsiouda,11 Kosmas Tsakiridis,12 Konstantinos Zarogoulidis,2 Johannes Brachmann11II Medical Clinic, Coburg Clinic, University of Würzburg, Coburg, Germany; 2Pulmonary Department-Oncology Unit, G Papanikolaou General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 3Department of Interventional Pneumology, Ruhrlandklinik, West German Lung Center, University Hospital, University of Duisburg-Essen, Essen, Germany; 4II Medizinische Klinik, Klinik für Kardiologie, Angiologie, Pneumologie, Klinikum Coburg, 5Department of Radiotherapy, 6Department of Pathology, Cytology and Molecular Diagnostics, 7Institute of Diagnostic and Interventional Radiology, Coburg Clinic, University of Wüerzburg, Coburg, Germany; 8Department of Respiratory Diseases, Changhai Hospital/First Affiliated Hospital of the Second Military Medical University, Shanghai, People’s Republic of China; 9Department of Diagnostic and Interventional Radiology, Goethe University of Frankfurt, Frankfurt, Germany; 10Interventional Drug Delivery Systems and Strategies (ID2S2, Medical Cryogenics, Lakeland Court Jupiter, FL, USA; 11Internal Medicine Unit, Theagenio Cancer Hospital, Thessaloniki, 12Cardiothoracic Surgery Department, Saint Luke Private Hospital, Thessaloniki, GreeceAbstract: Vasointestinal peptide metabolism plays a key physiological role in multimodular levels of vasodilatory, smooth muscle cell proliferative, parenchymal, and inflammatory lung reactions. In animal studies, vasointestinal peptide relaxes isolated pulmonary arterial segments from several mammalian species in vitro and neutralizes the pulmonary vasoconstrictor effect of endothelin. In some animal models, it reduces pulmonary vascular resistance in vivo and

  19. Efficacy and safety of regenerative cell therapy for pulmonary arterial hypertension in animal models: a preclinical systematic review protocol.

    Science.gov (United States)

    Suen, Colin M; Zhai, Alex; Lalu, Manoj M; Welsh, Christopher; Levac, Brendan M; Fergusson, Dean; McIntyre, Lauralyn; Stewart, Duncan J

    2016-05-25

    Pulmonary arterial hypertension (PAH) is a rare disease (15 cases per million) that is characterized by widespread loss of the pulmonary microcirculation and elevated pulmonary vascular resistance leading to pathological right ventricular remodeling and ultimately right heart failure. Regenerative cell therapies (i.e., therapies involving cells with stem or progenitor-like properties) could potentially restore the effective lung microcirculation and provide a curative therapy for PAH. Preclinical evidence suggests that regenerative cell therapy using endothelial progenitor cells or mesenchymal stem cells may be beneficial in the treatment of PAH. These findings have led to the completion of a small number of human clinical trials, albeit with modest effect compared to animal studies. The objective of this systematic review is to compare the efficacy and safety of regenerative cell therapies in preclinical models of PAH as well as assess study quality to inform future clinical studies. We will include preclinical studies of PAH in which a regenerative cell type was administered and outcomes compared to a disease control. The primary outcome will be pulmonary hemodynamics as assessed by measurement of right ventricular systolic pressure and/or mean pulmonary arterial pressure. Secondary outcomes will include mortality, survival, right ventricular remodeling, pulmonary vascular resistance, cardiac output, cardiac index, pulmonary acceleration time, tricuspid annular systolic excursion, and right ventricular wall thickness. Electronic searches of MEDLINE and EMBASE databases will be constructed and reviewed by the Peer Review of Electronic Search Strategies (PRESS) process. Search results will be screened independently in duplicate. Data from eligible studies will be extracted, pooled, and analyzed using random effects models. Risk of bias will be assessed using the SYstematic Review Centre for Laboratory animal Experimentation (SYRCLE) risk of bias tool, and

  20. Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan

    NARCIS (Netherlands)

    Schuuring, Mark J.; Vis, Jeroen C.; Duffels, Marielle G.; Bouma, Berto J.; Mulder, Barbara J. M.

    2010-01-01

    Pulmonary arterial hypertension (PAH) is a progressive disease with poor survival outcome. PAH is classified by the 2009 updated clinical classification of pulmonary hypertension and a major subgroup is PAH due to congenital heart disease (CHD) with systemic-to-pulmonary shunt. CHD-PAH is a result

  1. Iron Deficiency in COPD Associates with Increased Pulmonary Artery Pressure Estimated by Echocardiography

    DEFF Research Database (Denmark)

    Plesner, Louis L; Schoos, Mikkel M; Dalsgaard, Morten

    2017-01-01

    OBJECTIVES: Iron deficiency (ID) might augment chronic pulmonary hypertension in chronic obstructive pulmonary disease (COPD). This observational study investigates the association between ID and systolic pulmonary artery pressure estimated by echocardiography in non-anaemic COPD outpatients...... Vmax indicative of pulmonary hypertension was considered present for values ≥ 2.9 m/s. RESULTS: In a total of 75 included patients, 31 (41%) had ID. These patients had a significantly higher TR Vmax (3.02 vs. 2.77 m/s, p=0.01) and lower diffusion capacity of carbon monoxide (40% vs. 50% of predicted, p......0.05). Ferritin inversely correlated with TR Vmax in ID patients (-0.37 (p=0.04)). The prevalence of TR Vmax ≥ 2.9 m/s was twice as high in patients with ID (58% vs. 29%) and odds ratio of pulmonary hypertension in ID...

  2. Use of pulmonary artery catheter in coronary artery bypass graft. Costs and long-term outcomes.

    Directory of Open Access Journals (Sweden)

    Fei Xu

    Full Text Available Pulmonary artery catheters (PAC are used widely to monitor hemodynamics in patients undergoing coronary bypass graft (CABG surgery. However, recent studies have raised concerns regarding both the effectiveness and safety of PAC. Therefore, our aim was to determine the effects of the use of PAC on the short- and long-term health and economic outcomes of patients undergoing CABG.1361 Chinese patients who consecutively underwent isolated, primary CABG at the Cardiovascular Institute of Fuwai Hospital from June 1, 2012 to December 31, 2012 were included in this study. Of all the patients, 453 received PAC during operation (PAC group and 908 received no PAC therapy (control group. Short-term and long-term mortality and major complications were analyzed with multivariate regression analysis and propensity score matched-pair analysis was used to yield two well-matched groups for further comparison.The patients who were managed with PAC more often received intraoperative vasoactive drugs dopamine (70.9% vs. 45.5%; P<0.001 and epinephrine (7.7% vs. 2.6%; P<0.001. In addition, costs for initial hospitalization were higher for PAC patients ($14,535 vs. $13,873, respectively, p = 0.004. PAC use was neither associated with the perioperative mortality or major complications, nor was it associated with long-term mortality and major adverse cardiac and cerebrovascular events. In addition, comparison between two well-matched groups showed no significant differences either in baseline characteristics or in short-term and long-term outcomes.There is no clear indication of any benefit or harm in managing CABG patients with PAC. However, use of PAC in CABG is more expensive. That is, PAC use increased costs without benefit and thus appears unjustified for routine use in CABG surgery.

  3. The importance of echocardiography in diagnostics of idiopathic pulmonary arterial hypertension: A case report

    Directory of Open Access Journals (Sweden)

    Stojković Gabrijela

    2011-01-01

    Full Text Available Introduction. Idiopathic pulmonary arterial hypertension (IPAH is rare and difficult progressive disease with prevalence of approximately 15 cases per million residents, with predominant female cases. Case Outline. A 47-year-old female presented with symptoms and signs of the right heart chambers failure. Over prior seven years the patient had the feeling of suffocation and fatigue when walking, and received treatment for bronchial asthma. Physical examination revealed a marked loud second heart sound over the pulmonary artery. Electrocardiogram: right ventricular hypertrophy. Spirometric (pulmonary capacity test, cardiac perfusion scan and spiral CT scanning excluded secondary pulmonary arterial hypertension. Blood testing for connective tissue diseases and HIV were within normal reference limits. Transthoracic colour Doppler echocardiography demonstrated a mild tricuspid regurgitation with high values of estimated maximal and middle systolic pressure of the right ventricle (135/110 mm Hg, and excluded previous heart disease. Cardiac catheterization confirmed IPAH diagnosis, with systolic right ventricular pressure of 101/47/66 mm Hg and pulmonary capillary pressure of 30/13/10 mm Hg. Basic therapy with sildenafil, nevertheless, considerable limitations of strain tolerance was still present. Conclusion. IPAH is a severe heart disease with non-specific signs and symptoms. Screening for IPAH is transthoracic colour Doppler echocardiography shows high correlation with cardiac catheterization.

  4. New targets for pulmonary arterial hypertension: going beyond the currently targeted three pathways.

    Science.gov (United States)

    Huertas, Alice; Tu, Ly; Guignabert, Christophe

    2017-09-01

    Pulmonary arterial hypertension (PAH) is a hemodynamic state defined by a resting mean pulmonary arterial pressure at or above 25 mmHg with a normal pulmonary capillary wedge pressure, ultimately leading to right heart failure and premature death. Although considerable progress has been made in the development of drug therapies for PAH targeting abnormalities found in the three main pathobiologic pathways (nitric oxide, prostacyclin, and endothelin-1), there is no drug available to specifically stop the progressive cellular accumulation into the pulmonary artery vessel wall. Indeed, this pulmonary vascular remodeling is a key pathological feature in PAH, contributing to the progressive narrowing of the lumen responsible to the functional decline and to the right ventricle hypertrophy and dysfunction. Because numerous important discoveries in the PAH pathogenesis have been recently made, our improved understanding of additional pathways in this condition will presumably lead to the development of novel and more powerful therapeutic strategies in the near future. In this review, we highlight some recent biological findings and discuss the opportunities that could lead to the identification of new promising targets in PAH paving the way for future therapeutic strategies.

  5. Antioxidant Mechanism of Rutin on Hypoxia-Induced Pulmonary Arterial Cell Proliferation

    Directory of Open Access Journals (Sweden)

    Qian Li

    2014-11-01

    Full Text Available Reactive oxygen species (ROS are involved in the pathologic process of pulmonary arterial hypertension as either mediators or inducers. Rutin is a type of flavonoid which exhibits significant scavenging properties on oxygen radicals both in vitro and in vivo. In this study, we proposed that rutin attenuated hypoxia-induced pulmonary artery smooth muscle cell (PASMC proliferation by scavenging ROS. Immunofluorescence data showed that rutin decreased the production of ROS, which was mainly generated through mitochondria and NADPH oxidase 4 (Nox4 in pulmonary artery endothelial cells (PAECs. Western blot results provided further evidence on rutin increasing expression of Nox4 and hypoxia-inducible factor-1α (HIF-1α. Moreover, cell cycle analysis by flow cytometry indicated that proliferation of PASMCs triggered by hypoxia was also repressed by rutin. However, N-acetyl-L-cysteine (NAC, a scavenger of ROS, abolished or diminished the capability of rutin in repressing hypoxia-induced cell proliferation. These data suggest that rutin shows a potential benefit against the development of hypoxic pulmonary arterial hypertension by inhibiting ROS, subsequently preventing hypoxia-induced PASMC proliferation.

  6. Antioxidant mechanism of Rutin on hypoxia-induced pulmonary arterial cell proliferation.

    Science.gov (United States)

    Li, Qian; Qiu, Yanli; Mao, Min; Lv, Jinying; Zhang, Lixin; Li, Shuzhen; Li, Xia; Zheng, Xiaodong

    2014-11-18

    Reactive oxygen species (ROS) are involved in the pathologic process of pulmonary arterial hypertension as either mediators or inducers. Rutin is a type of flavonoid which exhibits significant scavenging properties on oxygen radicals both in vitro and in vivo. In this study, we proposed that rutin attenuated hypoxia-induced pulmonary artery smooth muscle cell (PASMC) proliferation by scavenging ROS. Immunofluorescence data showed that rutin decreased the production of ROS, which was mainly generated through mitochondria and NADPH oxidase 4 (Nox4) in pulmonary artery endothelial cells (PAECs). Western blot results provided further evidence on rutin increasing expression of Nox4 and hypoxia-inducible factor-1α (HIF-1α). Moreover, cell cycle analysis by flow cytometry indicated that proliferation of PASMCs triggered by hypoxia was also repressed by rutin. However, N-acetyl-L-cysteine (NAC), a scavenger of ROS, abolished or diminished the capability of rutin in repressing hypoxia-induced cell proliferation. These data suggest that rutin shows a potential benefit against the development of hypoxic pulmonary arterial hypertension by inhibiting ROS, subsequently preventing hypoxia-induced PASMC proliferation.

  7. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature

    NARCIS (Netherlands)

    ten Harkel, A. Derk Jan; Blom, Nico A.; Ottenkamp, Jaap

    2002-01-01

    OBJECTIVE: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). BACKGROUND: Isolated UAPA is a rare anomaly. Some case reports exist, but the best

  8. Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification

    NARCIS (Netherlands)

    van Riel, Annelieke C. M. J.; Schuuring, Mark J.; van Hessen, Irene D.; Zwinderman, Aielko H.; Cozijnsen, Luc; Reichert, Constant L. A.; Hoorntje, Jan C. A.; Wagenaar, Lodewijk J.; Post, Marco C.; van Dijk, Arie P. J.; Hoendermis, Elke S.; Mulder, Barbara J. M.; Bouma, Berto J.

    2014-01-01

    The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the

  9. Outcome of Pediatric Patients With Pulmonary Arterial Hypertension in the Era of New Medical Therapies

    NARCIS (Netherlands)

    van Loon, Rosa Laura E.; Roofthooft, Marcus T. R.; Delhaas, Tammo; van Osch-Gevers, Magdalena; ten Harkel, Arend D. J.; Strengers, Jan L. M.; Backx, Ad; Hillege, Hans L.; Berger, Rolf M. F.

    2010-01-01

    Little is known about the effects of "second-generation drugs" (prostanoids, endothelin receptor antagonists, 5-phosphodiesterase inhibitors) in children with pulmonary arterial hypertension (PAH). This study describes the outcome of a national cohort of children with PAH in an era when these drugs

  10. Comparison of an Endotracheal Cardiac Output Monitor to a Pulmonary Artery Catheter

    Science.gov (United States)

    2017-12-04

    ile methods of fluid resuscitation remain controversial , maintenance of a patent airway and hemodynamic stabi lity as indicated by invasive...Edwards LifeSciences, Irvine, CA) pulmonary artery catheter (PAC) under hypothermic and hemorrhagic conditions. Methods Power analysis (G*Power...3.1) suggested 8 an imals would be sufficient for comparisons. After induction of anesthesia, instrumentation , and stabilization in experiment 1

  11. Pulmonary artery location during microgravity activity: Potential impact for chest-mounted Doppler during space travel

    Science.gov (United States)

    Hadley, A. T., III; Conkin, J.; Waligora, J. M.; Horrigan, D. J., Jr.

    1984-01-01

    Doppler, or ultrasonic, monitoring for pain manifestations of decompression sickness (the bends) is accomplished by placing a sensor on the chest over the pulmonary artery and listening for bubbles. Difficulties have arisen because the technician notes that the pulmonary artery seems to move with subject movement in a one-g field and because the sensor output is influenced by only slight degrees of sensor movement. This study used two subjects and mapped the position of the pulmonary artery in one-g, microgravity, and two-g environments using ultrasound. The results showed that the pulmonary artery is fixed in location in microgravity and not affected by subject position change. The optimal position corresponded to where the Doppler signal is best heard with the subject in a supine position in a one-g environment. The impact of this result is that a proposed multiple sensor array on the chest proposed for microgravity use may not be necessary to monitor an astronaut during extravehicular activities. Instead, a single sensor of approximately 1 inch diameter and mounted in the position described above may suffice.

  12. Percutaneous Retrieval of a Pulmonary Artery Catheter Knot in Pacing Electrodes

    International Nuclear Information System (INIS)

    Valenzuela-Garcia, Luis Felipe; Almendro-Delia, Manuel; Gonzalez-Valdayo, Miguel; Munoz-Campos, Juan; Dorado-Garcia, Jose C.; Gomez-Rosa, Francisco; Vazquez-Garcia, Rafael; Calderon-Leal, Jose M.

    2007-01-01

    To illustrate a successful approach to resolving a pulmonary artery catheter knot in the pacing leads of a cardiac resynchronization device. When planning invasive monitoring for patients having right chamber electrodes, fluoroscopic-guided catheter insertion and extraction is advisable. In the event of coiling or knotting, an interventional radiologist should be contacted as soon as possible to avoid serious complications

  13. Prognostic factors in pediatric pulmonary arterial hypertension : A systematic review and meta-analysis

    NARCIS (Netherlands)

    Ploegstra, Mark-Jan; Zijlstra, Willemijn M. H.; Douwes, Johannes M.; Hillege, Hans L.; Berger, Rolf M. F.

    2015-01-01

    BACKGROUND: Despite the introduction of targeted therapies in pediatric pulmonary arterial hypertension (PAH), prognosis remains poor. For the definition of treatment strategies and guidelines, there is a high need for an evidence-based recapitulation of prognostic factors. The aim of this study was

  14. Sildenafil add-on therapy in paediatric pulmonary arterial hypertension, experiences of a national referral centre

    NARCIS (Netherlands)

    Douwes, Johannes M.; Roofthooft, Marcus T. R.; Van Loon, Rosa L. E.; Ploegstra, Mark-Jan; Bartelds, Beatrijs; Hillege, Hans L.; Berger, Rudolphus

    2014-01-01

    Objective In paediatric pulmonary arterial hypertension (PAH), the effectiveness of add-on combination PAH-therapy has not yet been systematically studied. The purpose of this study was to determine the effect of sildenafil add-on therapy in paediatric PAH patients treated with bosentan. Methods In

  15. Pulmonary artery thrombosis in a patient with right‑sided heart failure

    African Journals Online (AJOL)

    2013-09-19

    Sep 19, 2013 ... features of right-sided heart failure accompanied with dizziness. He was diagnosed to have pulmonary artery thrombosis ... was no history of vertigo. He denied history of orthopnea or paroxysmal nocturnal ... hypertension, diabetes mellitus, or asthma. He was a reformed smoker and heavy alcohol drinker ...

  16. Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease

    NARCIS (Netherlands)

    Zijlstra, Willemijn M. H.; Douwes, Johannes M.; Ploegstra, Mark-Jan; Krishnan, Usha; Roofthooft, Marcus T. R.; Hillege, Hans L.; Ivy, D. Dunbar; Rosenzweig, Erika B.; Berger, Rolf M. F.

    Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore

  17. Thickening of the pulmonary artery wall in acute intramural hematoma of the ascending aorta

    Directory of Open Access Journals (Sweden)

    Lardani Héctor

    2007-01-01

    Full Text Available Abstract Background The occurrence of pulmonary artery obstruction in the course of acute aortic dissection is an unusual complication. The mechanism implicated is the rupture of the outer layer of the aorta and the subsequent hemorrhage into the adventitia of the pulmonary artery that causes its wall thickening and, at times, produces extrinsic obstruction of the vessel. There are no reports of this complication in acute intramural hematoma. Case presentation An 87-year-old woman was admitted to the hospital in shock after having had severe chest pain followed by syncope. An urgent transesophageal echocardiogram revealed the presence of acute intramural hematoma, no evidence of aortic dissection, severe pericardial effusion with cardiac tamponade, and periaortic hematoma that involved the pulmonary artery generating circumferential wall thickening of its trunk and right branch with no evidence of flow obstruction. Urgent surgery was performed but the patient died in the operating room. The post mortem examination, in the operating room, confirmed that there was an extensive hematoma around the aorta and beneath the adventitial layer of the pulmonary artery, with no evidence of flow obstruction. Conclusion This is the first time that this rare complication is reported in the scenario of acute intramural hematoma and with the transesophageal echocardiogram as the diagnostic tool.

  18. Unilateral absence of lung perfusion resulting from endarteritis of the pulmonary artery

    International Nuclear Information System (INIS)

    Calegaro, J.U.M.; Turini, T.L.

    This case shows endarteritis of left pulmonary artery in the angiographic study with absent perfusion of the left lung in the MAA 131 I scintigraphy. Considerations are made conderning the presumable diagnosis of infection by 'toxocara canis' larval form. The literature review don't point out a similar case. (author) [pt

  19. Pulmonary arterial hypertension in rats due to age-related arginase activation in intermittent hypoxia.

    Science.gov (United States)

    Nara, Akina; Nagai, Hisashi; Shintani-Ishida, Kaori; Ogura, Sayoko; Shimosawa, Tatsuo; Kuwahira, Ichiro; Shirai, Mikiyasu; Yoshida, Ken-ichi

    2015-08-01

    Pulmonary arterial hypertension (PAH) is prevalent in patients with obstructive sleep apnea syndrome (OSAS). Aging induces arginase activation and reduces nitric oxide (NO) production in the arteries. Intermittent hypoxia (IH), conferred by cycles of brief hypoxia and normoxia, contributes to OSAS pathogenesis. Here, we studied the role of arginase and aging in the pathogenesis of PAH in adult (9-mo-old) and young (2-mo-old) male Sprague-Dawley rats subjected to IH or normoxia for 4 weeks and analyzed them with a pressure-volume catheter inserted into the right ventricle (RV) and by pulsed Doppler echocardiography. Western blot analysis was conducted on arginase, NO synthase isoforms, and nitrotyrosine. IH induced PAH, as shown by increased RV systolic pressure and RV hypertrophy, in adult rats but not in young rats. IH increased expression levels of arginase I and II proteins in the adult rats. IH also increased arginase I expression in the pulmonary artery endothelium and arginase II in the pulmonary artery adventitia. Furthermore, IH reduced pulmonary levels of nitrate and nitrite but increased nitrotyrosine levels in adult rats. An arginase inhibitor (N(ω)-hydroxy-nor-1-arginine) prevented IH-induced PAH and normalized nitrite and nitrate levels in adult rats. IH induced arginase up-regulation and PAH in adult rats, but not in young rats, through reduced NO production. Our findings suggest that arginase inhibition prevents or reverses PAH.

  20. “Anomalous right pulmonary artery left atrial fistula”: Growth in vain

    Directory of Open Access Journals (Sweden)

    Vishal Kumar Jain

    2015-12-01

    Full Text Available We report a case of direct communication between the right pulmonary artery and the left atrium; a rare cause of central cyanosis in 10 year old boy, emphasizing the role of multislice computed tomography scanner in delineating the complex vascular abnormality over more invasive conventional angiography.

  1. Acute Vasodilator Response in Pediatric Pulmonary Arterial Hypertension : Current Clinical Practice From the TOPP Registry

    NARCIS (Netherlands)

    Douwes, Johannes M.; Humpl, Tilman; Bonnet, Damien; Beghetti, Maurice; Ivy, D. Dunbar; Berger, Rolf M. F.

    2016-01-01

    BACKGROUND In pulmonary arterial hypertension (PAH), acute vasodilator response testing (AVT) is considered important to identify adult patients with favorable prognosis using calcium-channel blocker (CCB) therapy. However, in pediatric PAH, criteria used to identify acute responders and CCB use are

  2. Leptin, visfatin, insulin resistance, and body composition change in chronic obstructive pulmonary disease.

    Science.gov (United States)

    Eker, Suzan; Ayaz, Lokman; Tamer, Lulufer; Ulubas, Bahar

    2010-02-01

    The aim of the study was to compare endocrine parameters such as leptin, visfatin, insulin resistance, exercise capacity and body composition change, the pulmonary functions test (PFT) and arterial blood gases (ABG) parameters of chronic obstructive pulmonary disease (COPD) patients and in healthy controls. Fifty-five patients with COPD and without malnutrition and 25 healthy controls were included in our study. The serum leptin, visfatin, tumor necrosis factor alpha (TNF-alpha) and insulin resistance, body fat-free mass (FFM) and fat mass (FM) were measured in the groups. Additionally, body mass index (BMI) was calculated and the 6-minute walk test (6MWT), PFT and ABG analyses were performed in all of the cases. No difference in BMI between the COPD group and controls was determined. Serum leptin and visfatin levels, FFM and 6MWT distance were significantly lower in the patients with COPD (p leptin levels and BMI (r = 0.333, p = 0.027), and with FM (r = 0.365, p = 0.029). Serum visfatin level was correlated with the percentage of forced expiratory volume in the first second in the patients with COPD (r = 0.371, p = 0.013). HOMA-IR (Homeostasis model assessment of insulin resistance) and serum TNF-alpha levels in the patients with COPD were found to be significantly higher than controls (p = 0.001, p COPD. Evaluating the patients not only with the pulmonary function and also systemically, contributes to minimizing the mortality and morbidity.

  3. A brief overview of mouse models of pulmonary arterial hypertension: problems and prospects

    Science.gov (United States)

    Gomez-Arroyo, Jose; Saleem, Sheinei J.; Mizuno, Shiro; Syed, Aamer A.; Bogaard, Harm J.; Abbate, Antonio; Taraseviciene-Stewart, Laimute; Sung, Yon; Kraskauskas, Donatas; Farkas, Daniela; Conrad, Daniel H.; Nicolls, Mark R.

    2012-01-01

    Many chronic pulmonary diseases are associated with pulmonary hypertension (PH) and pulmonary vascular remodeling, which is a term that continues to be used to describe a wide spectrum of vascular abnormalities. Pulmonary vascular structural changes frequently increase pulmonary vascular resistance, causing PH and right heart failure. Although rat models had been standard models of PH research, in more recent years the availability of genetically engineered mice has made this species attractive for many investigators. Here we review a large amount of data derived from experimental PH reports published since 1996. These studies using wild-type and genetically designed mice illustrate the challenges and opportunities provided by these models. Hemodynamic measurements are difficult to obtain in mice, and right heart failure has not been investigated in mice. Anatomical, cellular, and genetic differences distinguish mice and rats, and pharmacogenomics may explain the degree of PH and the particular mode of pulmonary vascular adaptation and also the response of the right ventricle. PMID:22307907

  4. [Supervised exercise training in patients with pulmonary arterial hypertension - analyses of the effectiveness and safety].

    Science.gov (United States)

    Saxer, S; Rhyner, M; Treder, U; Speich, R; van Gestel, A J R

    2012-02-01

    Both in today's scientific research and in clinical practice, there exists a need to address the uncertainty concerning the effectiveness and safety of cardiopulmonary exercise training (CPET) in patients with pulmonary arterial hypertension (PAH). It is commonly believed that CPET may be dangerous for patients with PAH, because increasing pressure on the pulmonary arteries may worsen right-sided heart failure. Recently, the first clinical trials on exercise training in patients with pulmonary hypertension reported promising results. Extension of the walking distance at the 6-minute walk test improved quality of life, endurance capacity and a reduction in symptoms were observed after CPET. Furthermore, CPET was well tolerated by the patients in five clinical trials. In conclusion, it may be postulated that CPET is an effective therapy in patients with PAH and was tendentially well tolerated by the patients.

  5. Insulin resistance and associated dysfunction of resistance vessels and arterial hypertension

    DEFF Research Database (Denmark)

    Henriksen, Jens Henrik; Møller, Søren

    2005-01-01

    vascular resistance, high arterial compliance, increased cardiac output, secondary activation of counterregulatory systems (renin-angiotensin-aldosterone system, sympathetic nervous system, release of vasopressin), and resistance to vasopressors. The vasodilatory state is mediated through adrenomedullin...

  6. Prognostic factors in pediatric pulmonary arterial hypertension: A systematic review and meta-analysis.

    Science.gov (United States)

    Ploegstra, Mark-Jan; Zijlstra, Willemijn M H; Douwes, Johannes M; Hillege, Hans L; Berger, Rolf M F

    2015-04-01

    Despite the introduction of targeted therapies in pediatric pulmonary arterial hypertension (PAH), prognosis remains poor. For the definition of treatment strategies and guidelines, there is a high need for an evidence-based recapitulation of prognostic factors. The aim of this study was to identify and evaluate prognostic factors in pediatric PAH by a systematic review of the literature and to summarize the prognostic value of currently reported prognostic factors using meta-analysis. Medline, EMBASE and Cochrane Library were searched on April 1st 2014 to identify original studies that described predictors of mortality or lung-transplantation exclusively in children with PAH. 1053 citations were identified, of which 25 were included for further analysis. Hazard ratios (HR) and 95% confidence intervals were extracted from the papers. For variables studied in at least three non-overlapping cohorts, a combined HR was calculated using random-effects meta-analysis. WHO functional class (WHO-FC, HR 2.7), (N-terminal pro-) brain natriuretic peptide ([NT-pro]BNP, HR 3.2), mean right atrial pressure (mRAP, HR 1.1), cardiac index (HR 0.7), indexed pulmonary vascular resistance (PVRi, HR 1.3) and acute vasodilator response (HR 0.3) were identified as significant prognostic factors (p ≤ 0.001). This systematic review combined with separate meta-analyses shows that WHO-FC, (NT-pro)BNP, mRAP, PVRi, cardiac index and acute vasodilator response are consistently reported prognostic factors for outcome in pediatric PAH. These variables are useful clinical tools to assess prognosis and should be incorporated in treatment strategies and guidelines for children with PAH. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  7. Pharmacokinetic interactions among imatinib, bosentan and sildenafil, and their clinical implications in severe pulmonary arterial hypertension

    Science.gov (United States)

    Renard, Didier; Bouillon, Thomas; Zhou, Ping; Flesch, Gerard; Quinn, Debbie

    2015-01-01

    Aims This study characterized the population pharmacokinetics (PK) of imatinib in patients with severe pulmonary arterial hypertension (PAH), investigated drug–drug interactions (DDI) among imatinib, sildenafil and bosentan, and evaluated their clinical implications. Methods Plasma concentrations of imatinib, bosentan and sildenafil were collected in a phase III study and were used to characterize the PK of imatinib in this population. DDIs among the three drugs were quantified using a linear mixed model and log-transformed drug concentrations. Results The population mean estimates of apparent clearance (CL/F) and volume (V/F) were 10.8 l h–1 (95% CI 9.2, 12.4 l h–1) and 267 l (95% CI 208, 326 l), respectively. It was estimated that sildenafil concentrations increased, on average, by 64% (95% CI 32%, 103%) and bosentan concentrations by 51% (95% CI 12%, 104%), in the presence of imatinib. Despite increased concentrations of co-medications, treatment differences between imatinib and placebo for change in 6 min walk distance and pulmonary vascular resistance were relatively constant across the entire concentration range for sildenafil and bosentan. Overall, higher concentrations of imatinib and bosentan were not associated with increasing liver enzymes (serum glutamic oxaloacetic transaminases [SGOT]/serum glutamic-pyruvic transaminase [SGPT]). Conclusions Population PKs of imatinib in patients with severe PAH were found comparable with those of patients with chronic myeloid leukemia. Imatinib was found effective regardless of the co-medications and showed intrinsic efficacy beyond merely elevating the concentrations of the co-medications due to DDIs. There was no evidence of increased risk of liver toxicity upon co-administration with bosentan. PMID:25581063

  8. Rapamycin nanoparticles localize in diseased lung vasculature and prevent pulmonary arterial hypertension.

    Science.gov (United States)

    Segura-Ibarra, Victor; Amione-Guerra, Javier; Cruz-Solbes, Ana S; Cara, Francisca E; Iruegas-Nunez, David A; Wu, Suhong; Youker, Keith A; Bhimaraj, Arvind; Torre-Amione, Guillermo; Ferrari, Mauro; Karmouty-Quintana, Harry; Guha, Ashrith; Blanco, Elvin

    2017-05-30

    Vascular remodeling resulting from pulmonary arterial hypertension (PAH) leads to endothelial fenestrations. This feature can be exploited by nanoparticles (NP), allowing them to extravasate from circulation and accumulate in remodeled pulmonary vessels. Hyperactivation of the mTOR pathway in PAH drives pulmonary arterial smooth muscle cell proliferation. We hypothesized that rapamycin (RAP)-loaded NPs, an mTOR inhibitor, would accumulate in diseased lungs, selectively targeting vascular mTOR and preventing PAH progression. RAP poly(ethylene glycol)-block-poly(ε-caprolactone) (PEG-PCL) NPs were fabricated. NP accumulation and efficacy were examined in a rat monocrotaline model of PAH. Following intravenous (IV) administration, NP accumulation in diseased lungs was verified via LC/MS analysis and confocal imaging. Pulmonary arteriole thickness, right ventricular systolic pressures, and ventricular remodeling were determined to assess the therapeutic potential of RAP NPs. Monocrotaline-exposed rats showed increased NP accumulation within lungs compared to healthy controls, with NPs present to a high extent within pulmonary perivascular regions. RAP, in both free and NP form, attenuated PAH development, with histological analysis revealing minimal changes in pulmonary arteriole thickness and no ventricular remodeling. Importantly, NP-treated rats showed reduced systemic side effects compared to free RAP. This study demonstrates the potential for nanoparticles to significantly impact PAH through site-specific delivery of therapeutics. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. Thallium-201 myocardial imaging in young adults with anomalous left coronary artery arising from the pulmonary artery

    International Nuclear Information System (INIS)

    Moodie, D.S.; Cook, S.A.; Gill, C.C.; Napoli, C.A.

    1980-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery (Bland-Garland-White syndrome) may produce myocardial ischemia, infarction, and frequently death in infancy. Some patients, however, develop satisfactory coronary artery collaterals and are relatively asymptomatic into adulthood. Very little is known about their myocardial perfusion patterns. We studied three young adults with this condition using stress thallium-201 myocardial imaging. Electrocardiograms in two patients demonstrated old arterolateral myocardial infarctions. Preoperative stress exercise tests were positive in all three patients. Marked perfusion abnormalities were found in the proximal anterolateral wall in all patients, and one patient also had a posterolateral defect. Postoperatively, all stress tests returned to normal. Thallium imaging demonstrated improvement in ischemic areas, but old scars persisted

  10. Salvianolic acid A attenuates vascular remodeling in a pulmonary arterial hypertension rat model.

    Science.gov (United States)

    Chen, Yu-Cai; Yuan, Tian-Yi; Zhang, Hui-Fang; Wang, Dan-Shu; Yan, Yu; Niu, Zi-Ran; Lin, Yi-Huang; Fang, Lian-Hua; Du, Guan-Hua

    2016-06-01

    The current therapeutic approaches have a limited effect on the dysregulated pulmonary vascular remodeling, which is characteristic of pulmonary arterial hypertension (PAH). In this study we examined whether salvianolic acid A (SAA) extracted from the traditional Chinese medicine 'Dan Shen' attenuated vascular remodeling in a PAH rat model, and elucidated the underlying mechanisms. PAH was induced in rats by injecting a single dose of monocrotaline (MCT 60 mg/kg, sc). The rats were orally treated with either SAA (0.3, 1, 3 mg·kg(-1)·d(-1)) or a positive control bosentan (30 mg·kg(-1)·d(-1)) for 4 weeks. Echocardiography and hemodynamic measurements were performed on d 28. Then the hearts and lungs were harvested, the organ indices and pulmonary artery wall thickness were calculated, and biochemical and histochemical analysis were conducted. The levels of apoptotic and signaling proteins in the lungs were measured using immunoblotting. Treatment with SAA or bosentan effectively ameliorated MCT-induced pulmonary artery remodeling, pulmonary hemodynamic abnormalities and the subsequent increases of right ventricular systolic pressure (RVSP). Furthermore, the treatments significantly attenuated MCT-induced hypertrophic damage of myocardium, parenchymal injury and collagen deposition in the lungs. Moreover, the treatments attenuated MCT-induced apoptosis and fibrosis in the lungs. The treatments partially restored MCT-induced reductions of bone morphogenetic protein type II receptor (BMPRII) and phosphorylated Smad1/5 in the lungs. SAA ameliorates the pulmonary arterial remodeling in MCT-induced PAH rats most likely via activating the BMPRII-Smad pathway and inhibiting apoptosis. Thus, SAA may have therapeutic potential for the patients at high risk of PAH.

  11. Elevated Plasma Endothelin-1 and Pulmonary Arterial Pressure in Children Exposed to Air Pollution

    Science.gov (United States)

    Calderón-Garcidueñas, Lilian; Vincent, Renaud; Mora-Tiscareño, Antonieta; Franco-Lira, Maricela; Henríquez-Roldán, Carlos; Barragán-Mejía, Gerardo; Garrido-García, Luis; Camacho-Reyes, Laura; Valencia-Salazar, Gildardo; Paredes, Rogelio; Romero, Lina; Osnaya, Hector; Villarreal-Calderón, Rafael; Torres-Jardón, Ricardo; Hazucha, Milan J.; Reed, William

    2007-01-01

    Background Controlled exposures of animals and humans to particulate matter (PM) or ozone air pollution cause an increase in plasma levels of endothelin-1, a potent vasoconstrictor that regulates pulmonary arterial pressure. Objectives The primary objective of this field study was to determine whether Mexico City children, who are chronically exposed to levels of PM and O3 that exceed the United States air quality standards, have elevated plasma endothelin-1 levels and pulmonary arterial pressures. Methods We conducted a study of 81 children, 7.9 ± 1.3 years of age, lifelong residents of either northeast (n = 19) or southwest (n = 40) Mexico City or Polotitlán (n = 22), a control city with PM and O3 levels below the U.S. air quality standards. Clinical histories, physical examinations, and complete blood counts were done. Plasma endothelin-1 concentrations were determined by immunoassay, and pulmonary arterial pressures were measured by Doppler echocardiography. Results Mexico City children had higher plasma endothelin-1 concentrations compared with controls (p < 0.001). Mean pulmonary arterial pressure was elevated in children from both northeast (p < 0.001) and southwest (p < 0.05) Mexico City compared with controls. Endothelin-1 levels in Mexico City children were positively correlated with daily outdoor hours (p = 0.012), and 7-day cumulative levels of PM air pollution < 2.5 μm in aerodynamic diameter (PM2.5) before endothelin-1 measurement (p = 0.03). Conclusions Chronic exposure of children to PM2.5 is associated with increased levels of circulating endothelin-1 and elevated mean pulmonary arterial pressure. PMID:17687455

  12. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: Does the beneficial effect persist?

    NARCIS (Netherlands)

    van Loon, Rosa Laura E.; Hoendermis, Elke S.; Duffels, Marielle G. J.; Vonk-Noordegraaf, Anton; Mulder, Barbara J. M.; Hillege, Hans L.; Berger, Rolf M. F.

    2007-01-01

    Background Data on long-term response to bosentan in adults and especially children with pulmonary arterial hypertension (PAH) associated with systemic-to-pulmonary shunt are scarce. Methods We studied bosentan efficacy in 30 patients (20 adults, 10 children) with the disease at short- (4 months),

  13. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt : Does the beneficial effect persist?

    NARCIS (Netherlands)

    van Loon, Rosa Laura E.; Hoendermis, Elke S.; Duffels, Marielle G. J.; Vonk-Noordegraaf, Anton; Mulder, Barbara J. M.; Hillege, Hans L.; Berger, Rolf M. F.

    2007-01-01

    BACKGROUND: Data on long-term response to bosentan in adults and especially children with pulmonary arterial hypertension (PAH) associated with systemic-to-pulmonary shunt are scarce. METHODS: We studied bosentan efficacy in 30 patients (20 adults, 10 children) with the disease at short- (4 months),

  14. Large and medium-sized pulmonary artery obstruction does not play a role of primary importance in the etiology of sickle-cell disease-associated pulmonary hypertension

    NARCIS (Netherlands)

    van Beers, Eduard J.; van Eck-Smit, Berthe L. F.; Mac Gillavry, Melvin R.; van Tuijn, Charlotte F. J.; van Esser, Joost W. J.; Brandjes, Dees P. M.; Kappers-Klunne, Mies C.; Duits, Ashley J.; Biemond, Bart J.; Schnog, John-John B.

    2008-01-01

    Background: Pulmonary hypertension (PHT) occurs in approximately 30% of adult patients with sickle-cell disease (SCD) and is a risk factor for early death. The potential role of pulmonary artery obstruction, whether due to emboli or in situ thrombosis, in the etiology of SCD-related PHT is unknown.

  15. Balloon pulmonary angioplasty: a treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    Aiko eOgawa

    2015-02-01

    Full Text Available In chronic thromboembolic pulmonary hypertension, stenoses or obstructions of the pulmonary arteries due to organized thrombi can cause an elevation in pulmonary artery resistance, which in turn can result in pulmonary hypertension. Chronic thromboembolic pulmonary hypertension can be cured surgically by pulmonary endarterectomy; however, patients deemed unsuitable for pulmonary endarterectomy due to lesion, advanced age, or comorbidities have a poor prognosis and limited treatment options. Recently, advances have been made in balloon pulmonary angioplasty for these patients, and this review highlights this recent progress.

  16. HEART TRANSPLANTATION IN PATIENT WITH POST-RUPTURE PSEUDOANEURYSM OF SEGMENTAL BRANCH OF PULMONARY ARTERY

    Directory of Open Access Journals (Sweden)

    M. L. Gordeev

    2014-01-01

    Full Text Available Right heart catheterization (RHC should be performed on all candidates in preparation for listing for cardiac transplantation. Patient, 64 y. o., with chronic heart failure NYHA III class, had developed a rare complication while performing that procedure – a rupture of segmental branch of pulmonary artery (PA with pulmonary haemorrhage. The episode of pulmonary bleeding was stopped conservatively without surgical management. There was a pseudoaneurysm formation of segmental branch of PA 2,7 × 2,8 cm with signs of thrombosis. Afterfi ve months the patient underwent heart transplantation without severe complications in perioperative period. No more recurrent episodes of pulmonary haemorrhage were identifi ed.

  17. Novel assessment of haemodynamic kinetics with acute exercise in a rat model of pulmonary arterial hypertension.

    Science.gov (United States)

    Brown, Mary Beth; Chingombe, Tsungai J; Zinn, Abigail B; Reddy, Jagadeshwar G; Novack, Rachel A; Cooney, Sean A; Fisher, Amanda J; Presson, Robert G; Lahm, Tim; Petrache, Irina

    2015-06-01

    What is the central question of this study? The acute effect of exercise at moderately high intensity on already-elevated pulmonary arterial pressures and right ventricular wall stress in a rat model of pulmonary arterial hypertension (PAH) is unknown. What is the main finding and its importance? We show, for the first time, that in a rat model of PAH, exercise induces an acute reduction in pulmonary artery pressure associated with lung endothelial nitric oxide synthase activation, without evidence of acute right ventricular inflammation or myocyte apoptosis. Haemodynamic measures obtained with traditional invasive methodology as well as novel implantable telemetry reveal an exercise-induced 'window' of pulmonary hypertension alleviation, supporting future investigations of individualized exercise as therapy in PAH. Exercise improves outcomes of multiple chronic conditions, but controversial results, including increased pulmonary artery (PA) pressure, have prevented its routine implementation in pulmonary arterial hypertension (PAH), an incurable disease that drastically reduces exercise tolerance. Individualized, optimized exercise prescription for PAH requires a better understanding of disease-specific exercise responses. We investigated the acute impact of exercise on already-elevated PA pressure and right ventricular (RV) wall stress and inflammation in a rat model of PAH (PAH group, n = 12) induced once by monocrotaline (50 mg kg(-1) , i.p.; 2 weeks), compared with healthy control animals (n = 8). Single bouts of exercise consisted of a 45 min treadmill run at 75% of individually determined aerobic capacity (V̇O2max). Immediately after exercise, measurements of RV systolic pressure and systemic pressure were made via jugular and carotid cannulation, and were followed by tissue collection. Monocrotaline induced moderate PAH, evidenced by RV hypertrophy, decreased V̇O2max, PA muscularization, and RV and skeletal muscle cytoplasmic glycolysis detected by

  18. A 50-year-old woman with haemoptysis, cough and tachypnea: cholesterol pneumonia accompanying with pulmonary artery hypertension.

    Science.gov (United States)

    Li, Mengxi; Zhang, Nuofu; Zhou, Ying; Li, Jinhui; Gu, Yingying; Wang, Jian; Liu, Chunli

    2017-03-01

    Lipoid pneumonia is an uncommon disease caused by the presence of lipid in the alveoli. Here we described a case of a 50-year-old woman with haemoptysis, cough and tachypnea, who was diagnosed with cholesterol pneumonia accompanying with pulmonary artery hypertension. The extremely high pulmonary artery pressure achieved, in this case, is alarming and should alert the physicians that the cholesterol pneumonia may be one of the underlying causes of pulmonary artery hypertension. After a treatment of methylprednisolone, her clinical symptoms were significantly improved, which suggested that steroid might be a promising therapeutic for patients with cholesterol pneumonia. © 2015 John Wiley & Sons Ltd.

  19. Anomalous origin of the left pulmonary artery from the ascending aorta: successful surgical correction in an infant with fallot's tetralogy

    Directory of Open Access Journals (Sweden)

    Amaral Fernando

    2002-01-01

    Full Text Available We describe the case of a 40-day-old female patient with a history of breathlessness since birth who was referred to our hospital for surgical correction of common arterial trunk. The invasive investigation disclosed a Fallot¢s tetralogy anatomy associated with an anomalous origin of the left pulmonary artery from the ascending aorta. Immediately after diagnosis, the patient underwent a successful total surgical correction of the defect, including simultaneous anastomosis of the left pulmonary artery to the pulmonary trunk.

  20. Anomalous left-to-right shunting communication between the ascending aorta and right pulmonary artery in a dog.

    Science.gov (United States)

    Scollan, Katherine; Salinardi, Brenda; Bulmer, Barret J; Sisson, D David

    2011-06-01

    Anomalies of conotruncal septation are rare in dogs and uncommon in humans. Congenital conotruncal defects most commonly reported in veterinary medicine include aorto-pulmonary window and persistent truncus arteriosus. We report a case of an anomalous vessel connecting the ascending aorta to the right pulmonary artery causing left-to-right shunting, left-sided volume overload, and pulmonary overcirculation. Transesophageal echocardiography, cardiac catheterization, and contrast-enhanced computed tomography assisted in the diagnosis and facilitated the surgical correction of the anomalous vessel. The authors hypothesize this defect represents an unusual anomalous vessel connecting the ascending aorta to the right pulmonary artery. Copyright © 2011 Elsevier B.V. All rights reserved.

  1. Constitutively active form of natriuretic peptide receptor 2 ameliorates experimental pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Nobutoshi Nawa

    2016-01-01

    Full Text Available We recently found a constitutively active mutant of natriuretic peptide receptor 2 (caNPR2; V883M, which synthesizes larger amounts of cyclic guanosine monophosphate (cGMP intracellularly without any ligand stimulation than existing drugs. The aim of this study was to investigate the therapeutic effects of gene transduction using caNPR2 for pulmonary arterial hypertension (PAH. In vitro gene transduction into human pulmonary arterial smooth muscle cells using Sendai virus (SeV vectors carrying caNPR2 induced 10,000-fold increases in the synthesis of cGMP without ligand stimulation, and the proliferation of caNPR2-expressing cells was significantly attenuated. The PAH model rats generated by hypoxia and the administration of SU5416 were then treated with SeV vectors through a direct injection into the left pulmonary artery. Right ventricular systolic pressure was significantly decreased 2 weeks after the treatment, while systemic blood pressure remained unchanged. Histological analyses revealed that the medial wall thickness and occlusion rate of pulmonary arterioles were significantly improved in caNPR2-treated lungs. Neither the systemic integration of virus vectors nor side effects were observed. The massive stimulation of cGMP synthesis by gene therapy with caNPR2 was safe and effective in a PAH rat model and, thus, has potential as a novel therapy for patients with severe progressive PAH.

  2. Pulmonary Artery Pulsatility Index Is Associated With Right Ventricular Failure After Left Ventricular Assist Device Surgery.

    Science.gov (United States)

    Morine, Kevin J; Kiernan, Michael S; Pham, Duc Thinh; Paruchuri, Vikram; Denofrio, David; Kapur, Navin K

    2016-02-01

    Right ventricular failure (RVF) is a major cause of morbidity and mortality after CF-LVAD implantation. We explored the association of pulmonary artery compliance (PAC), pulmonary artery elastance (PAE), and pulmonary artery pulsatility index (PAPi) in addition to established parameters as preoperative determinants of postoperative RVF after CF-LVAD surgery. We retrospectively reviewed 132 consecutive CF-LVAD implantations at Tufts Medical Center from 2008 to 2013. Clinical, hemodynamic, and echocardiographic data were studied. RVF was defined as the unplanned need for a right ventricular assist device or inotrope dependence for ≥14 days. Univariate analysis was performed. RVF occurred in 32 of 132 patients (24%). PAC and PAE were not changed, whereas the PAPi was lower among patients with versus without postoperative RVF (1.32 ± 0.46 vs 2.77 ± 1.16; P < .001). RA pressure, RA to pulmonary capillary wedge pressure ratio (RA:PCWP), and RV stroke work index (RVSWI) were also associated with RVF. Using receiver operating characteristic curve-derived cut-points, PAPi < 1.85 provided 94% sensitivity and 81% specificity (C-statistic = 0.942) for identifying RVF and exceeded the predictive value of RA:PCWP, RVSWI, or RA pressure alone. PAPi is a simple hemodynamic variable that may help to identify patients at high risk of developing RVF after LVAD implantation. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Calcilytics enhance sildenafil-induced antiproliferation in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Yamamura, Aya; Yagi, Satomi; Ohara, Naoki; Tsukamoto, Kikuo

    2016-08-05

    Idiopathic pulmonary arterial hypertension (IPAH) is a progressive and fatal disease of the pulmonary artery resulting from currently unidentified etiology. IPAH is pathologically characterized as sustained vasoconstriction and vascular remodeling of the pulmonary artery. Phosphodiesterase type 5 (PDE5) inhibitors have been clinically used in the treatment of IPAH. Recently, we have shown that Ca(2+)-sensing receptor (CaSR) antagonists, or calcilytics, inhibit excessive cell proliferation of pulmonary arterial smooth muscle cells (PASMCs) from IPAH patients. In this study, the additive or synergistic effect of calcilytics on antiproliferation following PDE5 inhibition was examined in IPAH-PASMCs by MTT assay. Treatment with sildenafil blocked the excessive cell proliferation of IPAH-PASMCs in a concentration-dependent manner with an IC50 value of 16.9μM. However, sildenafil (0.03-100μM) did not affect the cell growth of PASMCs from normal subjects and patients with chronic thromboembolic pulmonary hypertension (CTEPH). Co-treatment with 0.3μM NPS2143, a calcilytic, additively enhanced the antiproliferative effect induced by sildenafil (3 or 30μM) in IPAH-PASMCs. Additionally, the inhibitory effect of calcilytics, NPS2143 or Calhex 231 (1 or 10μM), on excessive cell proliferation of IPAH-PASMCs was synergistic increased in the presence of 1μM sildenafil. Similar results were obtained by BrdU incorporation assay. These findings reveal that calcilytics additively/synergistically enhance the antiproliferative activity mediated by PDE5 inhibition, suggesting that a combination therapy of a PDE5 inhibitor with a calcilytic may be useful as a novel therapeutic approach for IPAH. Copyright © 2016 Elsevier B.V. All rights reserved.

  4. Hypoxia and nitric oxide exposure promote apoptotic signaling in contractile pulmonary arterial smooth muscle but not in pulmonary epithelium.

    Science.gov (United States)

    Postolow, F; Fediuk, J; Nolette, N; Hinton, M; Dakshinamurti, S

    2011-12-01

    Neonatal pulmonary hypertension is characterized by hypoxia, abnormal vascular remodeling, and impaired alveolarization. Nitric oxide (NO) regulates cell replication and activation of apoptosis. Our objective was to examine cell phenotype-specific effects of hypoxia and NO exposure on cumulative apoptotic signal in neonatal pulmonary epithelial cells and arterial smooth muscle. Primary cultured newborn porcine pulmonary arterial myocytes and epithelial cells were grown in normoxic (21% O2) or hypoxic conditions (10% O2). Myocyte phenotype was predetermined by serum-supplementation or -deprivation. Cells were exposed to sodium nitroprusside (10(-7) -10(-4)  M) or diluent for 3 days. Cell survival was estimated by MTT assay; BAX, Bcl-2, and cleaved caspase-3 by Western blot; cell cycle entry by laser scanning cytometry. Hypoxic epithelial cells exhibited a small increase in anti-apoptotic Bcl2, and decrease in BAX. Cell survival and active caspase-3 were unchanged. Exposure to NO had no impact on epithelial apoptosis, but initiated necrosis. In contractile myocytes, pro-apoptotic BAX abundance and caspase-3 activation were increased by hypoxia, augmented by NO exposure promoting apoptosis. Hypoxia decreased BAX/Bcl-2 ratio and promoted survival of synthetic myocytes; NO increased apoptosis of normoxic synthetic myocytes, but decreased apoptosis of hypoxic synthetic myocytes. The effect of NO on pulmonary apoptosis is phenotype-dependent. A cumulative apoptotic effect of hypoxia and NO in vitro exerted on contractile myocytes may lead to contraction of this subpopulation, while synthetic myocyte survival and proliferation is enhanced by hypoxia and NO. Epithelial survival is unaffected. We speculate that alveolar rarefaction reported after neonatal hypoxia may arise from growth arrest in the vascular rather than the epithelial compartment. Copyright © 2011 Wiley Periodicals, Inc.

  5. Clinical use of extended-release oral treprostinil in the treatment of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Pugliese SC

    2016-01-01

    Full Text Available Steven C Pugliese,1 Todd M Bull1,2 1Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, 2UCD Pulmonary Vascular Disease Center, Division of Pulmonary Sciences and Critical Care Medicine and Cardiology, Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, USA Abstract: The development of parenteral prostacyclin therapy marked a dramatic breakthrough in the treatment of pulmonary arterial hypertension (PAH. Intravenous (IV epoprostenol was the first PAH specific therapy and to date, remains the only treatment to demonstrate a mortality benefit. Because of the inherent complexities and risks of treating patients with continuous infusion IV therapy, there is great interest in the development of an oral prostacyclin analog that could mimic the benefits of IV therapy. Herein, we highlight the development of oral prostacyclin therapy, focusing on oral treprostinil, the only US Food and Drug Administration approved oral prostacyclin. Recent Phase III clinical trials have shown the drug to improve exercise tolerance in treatment-naïve PAH patients, but not patients on background oral therapy. Oral treprostinil appears to be most efficacious at higher doses, but its side effect profile and complexities with dosing complicate its use. While oral treprostinil’s current therapeutic role in PAH remains unclear, ongoing studies of this class of medication should help clarify their role in the treatment of PAH. Keywords: oral treprostinil, pulmonary arterial hypertension, selexipag

  6. Primary intimal sarcoma of the pulmonary artery 1 case report and retrospective analysis of 20 cases published in China

    International Nuclear Information System (INIS)

    Guo Lingchuan; Chen Yanbin

    2012-01-01

    Objective: To explore the clinical and histopathological features of primary, intimal sarcoma of the pulmonary artery. Methods: One case of primary intimal sarcoma of the pulmonary artery diagnosed in our hospital and 20 cases published openly in China were analysed retrospectively. Results: The clinical presentation of primary intimal sarcoma of the pulmonary artery was progressive dyspnea, cough, chest pain, syncope, bloody phlegm, fever and fatigue. From the radiological finding, its signs were of pulmonary, artery dilation, reduced pulmonary vasculature and cardiomegaly, soft tissue mass near the right ventricle outflow tract. Histological examination of intimal sarcoma of the pulmonary artery revealed a poorly differentiated mesenchymal tumor of fibroblastic or myofibroblastic differentiation, consisting of mildly atypical spindle cells with atypia, mitosis, and nuclear polymorphism. Immunohistochemical analysis showed positive staining with antibodies against vimentin, alpha-smooth muscle actin, while negative for desmin. Conclusion: Primary, intimal sarcoma of the pulmonary artery has atypical clinical and radiological manifestation with poor prognosis, its definite diagnosis depends on histopathology. (authors)

  7. Low WSS and High OSI Measured by 3D Cine PC MRI Reflect High Pulmonary Artery Pressures in Suspected Secondary Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Terada, Masaki; Takehara, Yasuo; Isoda, Haruo; Uto, Tomohiro; Matsunaga, Masaki; Alley, Marcus

    2016-01-01

    Institutional Review Board (IRB)-approved prospective study was conducted to test whether objective and quantitative hemodynamic markers wall shear stress (WSS) and oscillatory shear index (OSI) measured by three-dimensional (3D) cine phase-contrast (PC) can reflect pulmonary arterial hypertension (PAH). Seventeen consecutive patients of suspected secondary PAH were examined for pulmonary artery pressures (PAPs) with right heart catheterization (RHC) and three-dimensional (3D) cine PC MR. Based on the RHC data, patients were subdivided into two groups of 12 non-PAH (median age of 74.5 years) and 5 PAH (median age of 77 years) patients. Based on 3D cine PC magnetic resonance (MR), hemodynamic parameters including spatially averaged systolic WSS (sWSS), diastolic WSS (dWSS), mean WSS (mWSS), OSI and blood vessel section area (BVSA) at the pulmonary arterial trunk were calculated. Streamline images in the pulmonary arteries were also assessed. All the parameters were compared between non-PAH and PAH groups. sWSS (N/m(2)) and mWSS (N/m(2)) of PAH group was lower than that of non-PAH group (0.594 ± 0.067 vs. 0.961 ± 0.590, P = 0.001), (0.365 ± 0.035 vs. 0.489 ± 0.132, P = 0.027). OSI of PAH group was higher than that of non-PAH (0.214 ± 0.026 vs. 0.130 ± 0.046, P = 0.001). sWSS, mWSS, and dWSS were inversely correlated and OSI was positively correlated to mean PAP or systolic PAP with r values of -0.638 (P = 0.005), -0.643 (P = 0.005), -0.485 (P = 0.049), and 0.625 (P = 0.007); or -0.622 (P = 0.008), -0.629 (P = 0.007), -0.484 (P = 0.049), and 0.594 (P = 0.012), respectively. sWSS was also inversely correlated to BVSA with r value of -0.488 (P = 0.049), and OSI was correlated to BVSA with r value of 0.574 (P = 0.016). Vortex or helical flows were observed more frequently in PAH patients. The low sWSS and mWSS as well as high OSI measured with 3D cine PC MR could be potential hemodynamic markers for the increased PAP in suspected secondary PAH patients.

  8. Fractal Dimension Analysis of MDCT Images for Quantifying the Morphological Changes of the Pulmonary Artery Tree in Patients with Pulmonary Hypertension

    International Nuclear Information System (INIS)

    Sun, Haitao; Li, Ning; Guo, Lijun; Gao, Fei; Liu, Cheng

    2011-01-01

    The aim of this study was to use fractal dimension (FD) analysis on multidetector CT (MDCT) images for quantifying the morphological changes of the pulmonary artery tree in patients with pulmonary hypertension (PH). Fourteen patients with PH and 17 patients without PH as controls were studied. All of the patients underwent contrast-enhanced helical CT and transthoracic echocardiography. The pulmonary artery trees were generated using post-processing software, and the FD and projected image area of the pulmonary artery trees were determined with Image J software in a personal computer. The FD, the projected image area and the pulmonary artery pressure (PAP) were statistically evaluated in the two groups. The FD, the projected image area and the PAP of the patients with PH were higher than those values of the patients without PH (p < 0.05, t-test). There was a high correlation of FD with the PAP (r = 0.82, p < 0.05, partial correlation analysis). There was a moderate correlation of FD with the projected image area (r = 0.49, p < 0.05, partial correlation analysis). There was a correlation of the PAP with the projected image area (r = 0.65, p < 0.05, Pearson correlation analysis). The FD of the pulmonary arteries in the PH patients was significantly higher than that of the controls. There is a high correlation of FD with the PAP.

  9. Fractal Dimension Analysis of MDCT Images for Quantifying the Morphological Changes of the Pulmonary Artery Tree in Patients with Pulmonary Hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Sun, Haitao; Li, Ning; Guo, Lijun; Gao, Fei; Liu, Cheng [Shandong University, Shandong Medical Imaging Research Institute, Shandong (Korea, Republic of)

    2011-06-15

    The aim of this study was to use fractal dimension (FD) analysis on multidetector CT (MDCT) images for quantifying the morphological changes of the pulmonary artery tree in patients with pulmonary hypertension (PH). Fourteen patients with PH and 17 patients without PH as controls were studied. All of the patients underwent contrast-enhanced helical CT and transthoracic echocardiography. The pulmonary artery trees were generated using post-processing software, and the FD and projected image area of the pulmonary artery trees were determined with Image J software in a personal computer. The FD, the projected image area and the pulmonary artery pressure (PAP) were statistically evaluated in the two groups. The FD, the projected image area and the PAP of the patients with PH were higher than those values of the patients without PH (p < 0.05, t-test). There was a high correlation of FD with the PAP (r = 0.82, p < 0.05, partial correlation analysis). There was a moderate correlation of FD with the projected image area (r = 0.49, p < 0.05, partial correlation analysis). There was a correlation of the PAP with the projected image area (r = 0.65, p < 0.05, Pearson correlation analysis). The FD of the pulmonary arteries in the PH patients was significantly higher than that of the controls. There is a high correlation of FD with the PAP.

  10. Cognitive, emotional, and quality of life outcomes in patients with pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Glissmeyer Eric W

    2006-03-01

    Full Text Available Abstract Background The effects of pulmonary arterial hypertension on cardiovascular and physical function are well documented. Limited information exists regarding the effects of pulmonary arterial hypertension on cognitive function despite patient reports of problems with memory and attention. Our primary purpose was to determine if a prospectively identified cohort of pulmonary arterial hypertension patients had cognitive sequelae. Our secondary purpose was to determine the relationships between cognitive sequelae and neuropsychological test scores with depression, anxiety, and quality of life. Methods Forty-six adults with pulmonary arterial hypertension underwent assessment of cognitive function, depression, anxiety, and quality of life using standardized neuropsychological tests and questionnaires. The patients' scores were compared to normal population data. Medical, affective, neuropsychological, and quality of life data for patients with and without cognitive sequelae were compared using analysis of variance, Chi-square, or Fisher exact tests for categorical data. Correlations assessed relationships between neuropsychological test scores, depression, anxiety, quality of life, and medical data. Results Cognitive sequelae occurred in 58% (27/46 of the pulmonary arterial hypertension patients. Patients with cognitive sequelae had worse verbal learning, delayed verbal memory, executive function, and fine motor scores compared to patients without cognitive sequelae. Twenty-six percent of patients had moderate to severe depression and 19% had moderate to severe anxiety. Depression, anxiety and quality of life were not different for patients with or without cognitive sequelae. Our patients had decreased quality of life, which was associated with worse working memory. Conclusion Patients with pulmonary arterial hypertension have cognitive impairments, depression, anxiety, and decreased quality of life. Depression, anxiety, and quality of life

  11. Rapid Fatal Outcome from Pulmonary Arteries Compression in Transitional Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Ioannis A. Voutsadakis

    2009-01-01

    Full Text Available Transitional cell carcinoma of the urinary bladder is a malignancy that metastasizes frequently to lymph nodes including the mediastinal lymph nodes. This occurrence may produce symptoms due to compression of adjacent structures such as the superior vena cava syndrome or dysphagia from esophageal compression. We report the case of a 59-year-old man with metastatic transitional cell carcinoma for whom mediastinal lymphadenopathy led to pulmonary artery compression and a rapidly fatal outcome. This rare occurrence has to be distinguished from pulmonary embolism, a much more frequent event in cancer patients, in order that proper and prompt treatment be initiated.

  12. Missed diagnosis of atresia of the right pulmonary artery in woman with left-sided pneumothorax

    DEFF Research Database (Denmark)

    Dagnegård, Hanna; Ryom, Philip

    2016-01-01

    Isolated pulmonary atresia is an uncommon condition, which can go undiagnosed for a long time in asymptomatic patients. Sometimes, diagnosis can be made at pregnancy due to respiratory symptoms. There is no known increased risk of pneumothorax. We here present a case where a second-time pregnant ...... woman with an unknown atresia of the right pulmonary artery received a left-sided pneumothorax. The diagnosis was initially missed in spite of adequate imaging and the condition progressed to respiratory stop. We describe the course of diagnostics and the chosen strategy of treatment....

  13. Congenital pulmonary steal associated with Tetralogy of Fallot, right aortic arch and an isolated left carotid artery

    Energy Technology Data Exchange (ETDEWEB)

    Tozzi, R.; Kiley, R.; Doyle, E.; Hernanz-Schulman, M.; Genieser, N.; Ambrosino, M.; Pinto, R.

    1989-07-01

    In patients with Tetralogy of Fallot, collateral supply to the pulmonary artery from systemic arterial sources is frequently encountered. However, collateral blood flow arising from the cerebral circulation has, to our knowledge, not been reported in this condition. We describe a patient with Tetralogy of Fallot in whom the left pulmonary artery filled directly via retrograde flow from the left carotid artery. Review of the literature in search of a similar case was unrevealing. A theoretical embryologic basis for this anomaly is discussed. (orig.).

  14. Congenital pulmonary steal associated with Tetralogy of Fallot, right aortic arch and an isolated left carotid artery

    International Nuclear Information System (INIS)

    Tozzi, R.; Kiley, R.; Doyle, E.; Hernanz-Schulman, M.; Genieser, N.; Ambrosino, M.; Pinto, R.

    1989-01-01

    In patients with Tetralogy of Fallot, collateral supply to the pulmonary artery from systemic arterial sources is frequently encountered. However, collateral blood flow arising from the cerebral circulation has, to our knowledge, not been reported in this condition. We describe a patient with Tetralogy of Fallot in whom the left pulmonary artery filled directly via retrograde flow from the left carotid artery. Review of the literature in search of a similar case was unrevealing. A theoretical embryologic basis for this anomaly is discussed. (orig.)

  15. Change of pulmonary arterial and bronchial diameter during respiration : HRCT findings

    International Nuclear Information System (INIS)

    Yang, Sang Kyu; Kwak, Byung Kook; Kim, Young Min; Jung, Gul Ho; Lee, Shin Hyung; Lee, Chang Joon; Song, Chi Ho

    1997-01-01

    To evaluate the changes and normal ranges of the artery-bronchus ratio(ABR) during respiration We analyzed HRCT of 10 healthy adults. The HRCT findings of ten healthy adults were analysed. CT scanning was performed with 1mm collimation at 3mm intervals during full inspiration and full expiration, with a range during inspiration from 2cm to 4cm above the carina and from 4cm above to 2cm below the right hemidiaphragm. The range during expiration was from 1cm to 3cm above the carina and from 4cm above to 2cm below the right hemidiaphragm. ABiR (defined as the diameter of pulmonary artery divided by the inner diameter of the bronchus), ABoR (defined as the diameter of pulmonary artery divided by the outer diameter of the bronchus) and BLR (defined as the inner diameter of the bronchus divided by the outer diameter of the bronchus) were measured on the display console. The mean inner diameter of the bronchi was 2.04±0.73mm during inspiration and 1.68±0.51mm during expiration, while the mean diameter of the arteries was 3.95±1.03mm during inspiration and 4.37±1.09mm during expiration. The diameters of the bronchi were thus seen to increase during inspiration, and the diameters of the pulmonary arteries, to decrease. The mean thickness of the bronchial wall was 1.07±0.19mm during inspiration and 1.06±0.24mm during expiration;thus, no change in thickness was seen during respiration (p<0.05). Mean ABiR was 2.01±0.60 (range 1.15-4.58) during inspiration and 2.59±0.74(range 1.16-4.9) during expiration, and in all cases the inner diameter of the bronchus was less than that of the accompanying pulmonary artery. Mean ABoR was 0.91±0.19 during inspiration and 1.09±0.22 during expiration. While for BLR, the corresponding fingures were 0.46±0.06, and 0.44±0.09. HRCT is a useful tool for evaluating changes in the pulmonary arteries and bronchi during respiration

  16. [Resistant arterial hypertension and coarctation of the aorta].

    Science.gov (United States)

    Martínez-Quintana, Efrén; Rossique-Delmas, Pilar; Rodríguez-González, Fayna

    2014-01-01

    Coarctation of the aorta accounts for around 5 percent of all congenital heart defects. Many of these patients develop arterial hypertension, and occasionally resistant arterial hypertension, despite adequate correction. This may lead to potentially fatal complications such as heart failure, aortic dissection, cerebrovascular events, or myocardial infarction. Therefore, a correct diagnosis must be made and an appropriate treatment started to reduce arterial hypertension, arteriosclerotic vascular disease, as well as the increased risk of cardiovascular morbidity and mortality. Copyright © 2014 Sociedad Española de Arteriosclerosis. Published by Elsevier España. All rights reserved.

  17. Colchicine Depolymerizes Microtubules, Increases Junctophilin-2, and Improves Right Ventricular Function in Experimental Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Prins, Kurt W; Tian, Lian; Wu, Danchen; Thenappan, Thenappan; Metzger, Joseph M; Archer, Stephen L

    2017-05-31

    Pulmonary arterial hypertension (PAH) is a lethal disease characterized by obstructive pulmonary vascular remodeling and right ventricular (RV) dysfunction. Although RV function predicts outcomes in PAH, mechanisms of RV dysfunction are poorly understood, and RV-targeted therapies are lacking. We hypothesized that in PAH, abnormal microtubular structure in RV cardiomyocytes impairs RV function by reducing junctophilin-2 (JPH2) expression, resulting in t-tubule derangements. Conversely, we assessed whether colchicine, a microtubule-depolymerizing agent, could increase JPH2 expression and enhance RV function in monocrotaline-induced PAH. Immunoblots, confocal microscopy, echocardiography, cardiac catheterization, and treadmill testing were used to examine colchicine's (0.5 mg/kg 3 times/week) effects on pulmonary hemodynamics, RV function, and functional capacity. Rats were treated with saline (n=28) or colchicine (n=24) for 3 weeks, beginning 1 week after monocrotaline (60 mg/kg, subcutaneous). In the monocrotaline RV, but not the left ventricle, microtubule density is increased, and JPH2 expression is reduced, with loss of t-tubule localization and t-tubule disarray. Colchicine reduces microtubule density, increases JPH2 expression, and improves t-tubule morphology in RV cardiomyocytes. Colchicine therapy diminishes RV hypertrophy, improves RV function, and enhances RV-pulmonary artery coupling. Colchicine reduces small pulmonary arteriolar thickness and improves pulmonary hemodynamics. Finally, colchicine increases exercise capacity. Monocrotaline-induced PAH causes RV-specific derangement of microtubules marked by reduction in JPH2 and t-tubule disarray. Colchicine reduces microtubule density, increases JPH2 expression, and improves both t-tubule architecture and RV function. Colchicine also reduces adverse pulmonary vascular remodeling. These results provide biological plausibility for a clinical trial to repurpose colchicine as a RV-directed therapy for PAH

  18. Novel ROCK inhibitors for the treatment of pulmonary arterial hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Shaw, Duncan; Hollingworth, Greg; Soldermann, Nicolas; Sprague, Elizabeth; Schuler, Walter; Vangrevelinghe, Eric; Duggan, Nicholas; Thomas, Matthew; Kosaka, Takatoshi; Waters, Nigel; van Eis, Maurice J. (Novartis)

    2014-10-01

    A novel class of selective inhibitors of ROCK1 and ROCK2 has been identified by structural based drug design. PK/PD experiments using a set of highly selective Rho kinase inhibitors suggest that systemic Rho kinase inhibition is linked to a reversible reduction in lymphocyte counts. These results led to the consideration of topical delivery of these molecules, and to the identification of a lead molecule 7 which shows promising PK and PD in a murine model of pulmonary hypertension after intra-tracheal dosing.

  19. Altered gene expression in pulmonary tissue of tryptophan hydroxylase-1 knockout mice: implications for pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    Richard B Rothman

    Full Text Available The use of fenfluramines can increase the risk of developing pulmonary arterial hypertension (PAH in humans, but the mechanisms responsible are unresolved. A recent study reported that female mice lacking the gene for tryptophan hydroxylase-1 (Tph1(-/- mice were protected from PAH caused by chronic dexfenfluramine, suggesting a pivotal role for peripheral serotonin (5-HT in the disease process. Here we tested two alternative hypotheses which might explain the lack of dexfenfluramine-induced PAH in Tph1(-/- mice. We postulated that: 1 Tph1(-/- mice express lower levels of pulmonary 5-HT transporter (SERT when compared to wild-type controls, and 2 Tph1(-/- mice display adaptive changes in the expression of non-serotonergic pulmonary genes which are implicated in PAH. SERT was measured using radioligand binding methods, whereas gene expression was measured using microarrays followed by quantitative real time PCR (qRT-PCR. Contrary to our first hypothesis, the number of pulmonary SERT sites was modestly up-regulated in female Tph1(-/- mice. The expression of 51 distinct genes was significantly altered in the lungs of female Tph1(-/- mice. Consistent with our second hypothesis, qRT-PCR confirmed that at least three genes implicated in the pathogenesis of PAH were markedly up-regulated: Has2, Hapln3 and Retlna. The finding that female Tph1(-/- mice are protected from dexfenfluramine-induced PAH could be related to compensatory changes in pulmonary gene expression, in addition to reductions in peripheral 5-HT. These observations emphasize the intrinsic limitation of interpreting data from studies conducted in transgenic mice that are not fully characterized.

  20. Immunohistochemical examination of plexiform-like complex vascular lesions in the lungs of broiler chickens selected for susceptibility to idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Hamal, Krishna R; Erf, Gisela F; Anthony, Nicholas B; Wideman, Robert F

    2012-01-01

    Idiopathic pulmonary arterial hypertension (IPAH) is a disease of unknown cause that is characterized by elevated pulmonary arterial pressure and pulmonary vascular resistance, and by extensive vascular remodelling. In human IPAH patients, remodelling of the pulmonary vasculature results in the formation of plexiform lesions in the terminal pulmonary arterioles. Various molecules are expressed in the human plexiform lesions, including alpha smooth muscle actin, von Willebrand factor, vascular endothelial growth factor, vascular endothelial growth factor receptor type 2, hypoxia inducible factor-1α, survivin, tenascin, collagen, fibronectin, and various immune/inflammatory cells such as, cytotoxic lymphocytes, B lymphocytes, MHC class II cells, and monocytes/macrophages are also present. Plexiform lesions rarely develop in the lungs of laboratory animals, but plexiform-like complex vascular lesions (CVL) do develop spontaneously in the lungs of broiler chickens from an IPAH-susceptible line. To examine angioproliferative and immune-system-related activities associated with CVL in broiler lungs, paraformaldehyde-fixed, paraffin-embedded lung sections from 8-week-old to 24-week-old broiler chickens were stained immunohistochemically using monoclonal or polyclonal antibodies specific for angioproliferative molecules and immune/inflammatory cells. The CVL in the lungs of broiler chickens exhibited positive staining for both angioproliferative molecules and immune/inflammatory cells. These observations combined with the close histological resemblance of broiler CVL to the plexiform lesions of human IPAH patients further validates chickens from our IPAH-susceptible line as an excellent animal model of spontaneous plexogenic arteriopathy.

  1. Pulmonary Arterial Hypertension in Adults: Novel Drugs and Catheter Ablation Techniques Show Promise? Systematic Review on Pharmacotherapy and Interventional Strategies

    Directory of Open Access Journals (Sweden)

    Salvatore Rosanio

    2014-01-01

    Full Text Available This systematic review aims to provide an update on pharmacological and interventional strategies for the treatment of pulmonary arterial hypertension in adults. Currently US Food and Drug Administration approved drugs including prostanoids, endothelin-receptor antagonists, phosphodiesterase type-5 inhibitors, and soluble guanylate-cyclase stimulators. These agents have transformed the prognosis for pulmonary arterial hypertension patients from symptomatic improvements in exercise tolerance ten years ago to delayed disease progression today. On the other hand, percutaneous balloon atrioseptostomy by using radiofrequency perforation, cutting balloon dilatation, or insertion of butterfly stents and pulmonary artery catheter-based denervation, both associated with very low rate of major complications and death, should be considered in combination with specific drugs at an earlier stage rather than late in the progression of pulmonary arterial hypertension and before the occurrence of overt right-sided heart failure.

  2. [Genistein attenuates monocrotaline-induced pulmonary arterial hypertension in rats by up-regulating heme oxygenase-1 expression].

    Science.gov (United States)

    Zhang, Yukun; Wang, Daoxin; Zhu, Tao; Li, Changyi

    2012-02-01

    To study the effect of genistein on the expression of heme oxygenase-1 (HO-1) in rats with pulmonary arterial hypertension (PAH) induced by monocrotaline (MCT). Sixty male Sprague-Dawley rats were randomly divided into 4 groups (n=15), namely the control group, model group, low-dose (20 µg/kg) genistein group and high-dose (80 µg/kg) genistein group. The hemodynamic parameters were measured and the remodeling of pulmonary small arteries was observed by electron microscope (EM). The expression of HO-1 in the lung tissues were detected by Western blotting. Compared with the model group, genistein treatment significantly reduced the elevated mean pulmonary arterial pressure, improved the right ventricular hypertrophy index, and increased the expression of HO-1 in a dose-dependent manner. Genistein attentuates pulmonary arterial hypertension in MCT-treated rats possibly by up-regulation of HO-1 in the lung tissues.

  3. Early Manifestation of Supravalvular Aortic and Pulmonary Artery Stenosis in a Patient with Williams Syndrome

    Directory of Open Access Journals (Sweden)

    Jong Uk Lee

    2016-04-01

    Full Text Available Williams syndrome (WS is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS and peripheral pulmonary artery stenosis (PPAS are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.

  4. Covered Stent and Coils Embolization of a Pulmonary Artery Pseudoaneurysm After Gunshot Wound

    Energy Technology Data Exchange (ETDEWEB)

    Huet, Nicolas, E-mail: nhuet@chu-grenoble.fr; Rodiere, Mathieu, E-mail: mrodiere@chu-grenoble.fr [Hôpital Universitaire de Grenoble and Université Grenoble Alpes, Department of Radiology and Medical Imaging (France); Badet, Michel, E-mail: michel.badet@ch-chambery.fr [Centre Hospitalier Métropôle Savoie, site de Chambéry, Intensive Care Unit (France); Michoud, Marie, E-mail: marie.michoud@ch-chambery.fr [Centre Hospitalier Métropôle Savoie, Site de Chambéry, Department of Radiology (France); Brichon, Pierre-Yves, E-mail: pybrichon@chu-grenoble.fr [Hôpital Universitaire de Grenoble and Université Grenoble Alpes, Department of Thoracic and Vascular Surgery (France); Ferretti, Gilbert, E-mail: gferretti@chu-grenoble.fr; Thony, Frédéric, E-mail: fthony@chu-grenoble.fr [Hôpital Universitaire de Grenoble and Université Grenoble Alpes, Department of Radiology and Medical Imaging (France)

    2016-05-15

    We report the first case of endovascular covered stent implantation for the treatment of a large pulmonary artery pseudoaneurysm (PAPA) following a right thoracic gunshot wound. After resuscitation and hemodynamic stabilization, a CT angiography was performed to analyze the neck size of the PAPA and its position relative to the branches of the parent artery. Covered stent implantation with additional coil embolization was successfully performed. At the 4-year follow-up, the stents remained patent and there was neither pseudoaneurysm recurrence nor treatment-related complication.

  5. Covered Stent and Coils Embolization of a Pulmonary Artery Pseudoaneurysm After Gunshot Wound

    International Nuclear Information System (INIS)

    Huet, Nicolas; Rodiere, Mathieu; Badet, Michel; Michoud, Marie; Brichon, Pierre-Yves; Ferretti, Gilbert; Thony, Frédéric

    2016-01-01

    We report the first case of endovascular covered stent implantation for the treatment of a large pulmonary artery pseudoaneurysm (PAPA) following a right thoracic gunshot wound. After resuscitation and hemodynamic stabilization, a CT angiography was performed to analyze the neck size of the PAPA and its position relative to the branches of the parent artery. Covered stent implantation with additional coil embolization was successfully performed. At the 4-year follow-up, the stents remained patent and there was neither pseudoaneurysm recurrence nor treatment-related complication.

  6. Severe infundibular pulmonary stenosis and coronary artery stenosis with ventricular tachycardia 24 years after mediastinal irradiation

    International Nuclear Information System (INIS)

    Ichinose, Tetsuo; Miyano, Hiroshi; Kimura, Toru; Yamashita, Haruyo; Takizawa, Kei; Nakazato, Yuji; Kawai, Sachio; Daida, Hiroyuki; Kawasaki, Shiori; Amano, Atushi

    2005-01-01

    A 28-year-old man developed severe infundibular pulmonary stenosis (PS), coronary artery stenosis with sustained ventricular tachycardia (VT) 24 years after mediastinal irradiation (total amount of 40 Gray) for non-Hodgkin's lymphoma. Repair of right ventricular outflow tract and coronary artery bypass graft procedure were performed. Infundibular PS was successfully relieved after operation and VT was also controlled by medication. Mediastinal irradiation often causes various cardiac complications after a latent period. Therefore, continuous careful observation is mandatory in patients with the history of mediastinal irradiation. (author)

  7. Relationship between chronic obstructive pulmonary disease and subclinical coronary artery disease in long-term smokers

    DEFF Research Database (Denmark)

    Rasmussen, Thomas; Køber, Lars; Pedersen, Jesper Holst

    2013-01-01

    Cardiovascular conditions are reported to be the most frequent cause of death in patients with chronic obstructive pulmonary disease (COPD). However, it remains unsettled whether severity of COPD per se is associated with coronary artery disease (CAD) independent of traditional cardiovascular ris...... factors. The aim of this study was to examine the relationship between the presence and severity of COPD and the amount of coronary artery calcium deposit, an indicator of CAD and cardiac risk, in a large population of current and former long-term smokers....

  8. Successful Interventional Management for Pulmonary Arterial Injury Secondary to Pacemaker Implantation

    Directory of Open Access Journals (Sweden)

    Hiroyuki Tokue

    2016-01-01

    Full Text Available Subclavian vein puncture is a relatively fast and safe technique to access the right heart for placement of pacemaker leads. Hemothorax related to injury of the pulmonary artery (PA is a rare complication of subclavian vein access but can be life-threatening. We report a case of hemothorax occurring after subclavian vein puncture for pacemaker implantation. No cases of transcatheter arterial embolization for PA injury secondary to pacemaker implantation have been reported. Understanding of this rare complication after pacemaker implantation along with its specific clinical presentation may lead to early diagnosis and intervention.

  9. Computational fluid dynamics of the right ventricular outflow tract and of the pulmonary artery: a bench model of flow dynamics.

    Science.gov (United States)

    Mosbahi, Selim; Mickaily-Huber, Elizabeth; Charbonnier, Dominique; Hullin, Roger; Burki, Marco; Ferrari, Enrico; von Segesser, Ludwig K; Berdajs, Denis A

    2014-10-01

    The reconstruction of the right ventricular outflow tract (RVOT) with valved conduits remains a challenge. The reoperation rate at 5 years can be as high as 25% and depends on age, type of conduit, conduit diameter and principal heart malformation. The aim of this study is to provide a bench model with computer fluid dynamics to analyse the haemodynamics of the RVOT, pulmonary artery, its bifurcation, and left and right pulmonary arteries that in the future may serve as a tool for analysis and prediction of outcome following RVOT reconstruction. Pressure, flow and diameter at the RVOT, pulmonary artery, bifurcation of the pulmonary artery, and left and right pulmonary arteries were measured in five normal pigs with a mean weight of 24.6 ± 0.89 kg. Data obtained were used for a 3D computer fluid-dynamics simulation of flow conditions, focusing on the pressure, flow and shear stress profile of the pulmonary trunk to the level of the left and right pulmonary arteries. Three inlet steady flow profiles were obtained at 0.2, 0.29 and 0.36 m/s that correspond to the flow rates of 1.5, 2.0 and 2.5 l/min flow at the RVOT. The flow velocity profile was constant at the RVOT down to the bifurcation and decreased at the left and right pulmonary arteries. In all three inlet velocity profiles, low sheer stress and low-velocity areas were detected along the left wall of the pulmonary artery, at the pulmonary artery bifurcation and at the ostia of both pulmonary arteries. This computed fluid real-time model provides us with a realistic picture of fluid dynamics in the pulmonary tract area. Deep shear stress areas correspond to a turbulent flow profile that is a predictive factor for the development of vessel wall arteriosclerosis. We believe that this bench model may be a useful tool for further evaluation of RVOT pathology following surgical reconstructions. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery

  10. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: The Role of Multislice Computed Tomography (MSCT)

    Science.gov (United States)

    Al Umairi, Rashid Saif; Al Kindi, Faiza; Al Busaidi, Fadhila

    2016-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality also known as Bland-White-Garland syndrome. The incidence of ALCAPA is about 1 in every 300,000 live births, and constitutes 0.24% and 0.46% of all congenital cardiac disease. It has a high infant mortality rate reaching up to 90% if left untreated. For many years, the diagnosis of ALCAPA was by angiography or autopsy. However, multislice computed tomography (MSCT) is a non-invasive imaging tool that allows accurate, non-invasive diagnosis of ALCAPA. Here we report a case of ALCAPA in a six-month-old girl who presented with a two-week history of cough, fever, tachypnea, and sweating during feeding. During admission, an echocardiogram was performed that revealed ALCAPA, which was confirmed using CT. We discuss the role of MSCT in its diagnosis. PMID:27602196

  11. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: The Role of Multislice Computed Tomography (MSCT

    Directory of Open Access Journals (Sweden)

    Rashid Saif Al Umairi

    2016-09-01

    Full Text Available Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA is a rare congenital coronary abnormality also known as Bland-White-Garland syndrome. The incidence of ALCAPA is about 1 in every 300,000 live births, and constitutes 0.24% and 0.46% of all congenital cardiac disease. It has a high infant mortality rate reaching up to 90% if left untreated. For many years, the diagnosis of ALCAPA was by angiography or autopsy. However, multislice computed tomography (MSCT is a non-invasive imaging tool that allows accurate, non-invasive diagnosis of ALCAPA. Here we report a case of ALCAPA in a six-month-old girl who presented with a two-week history of cough, fever, tachypnea, and sweating during feeding. During admission, an echocardiogram was performed that revealed ALCAPA, which was confirmed using CT. We discuss the role of MSCT in its diagnosis.

  12. Value of MR phase-contrast flow measurements for functional assessment of pulmonary arterial hypertension

    International Nuclear Information System (INIS)

    Ley, Sebastian; Mereles, Derliz; Gruenig, Ekkehard; Puderbach, Michael; Schoeck, Helena; Eichinger, Monika; Ley-Zaporozhan, Julia; Fink, Christian; Kauczor, Hans-Ulrich

    2007-01-01

    Goals of our study were to compare the pulmonary hemodynamics between healthy volunteers and patients with pulmonary arterial hypertension (PAH) and correlate MR flow measurements with echocardiography. Twenty-five patients with PAH and 25 volunteers were examined at 1.5 T. Phase-contrast flow measurements were performed in the ascending aorta and pulmonary trunk, resulting in the following parameters: peak velocity (cm/s), average blood flow (l/min), time to peak velocity (ms), velocity rise gradient and pulmonary distensibility (cm 2 ). The bronchosystemic shunt was calculated. In PAH patients transthoracic echocardiography and right-heart catheterization (RHC) served as the gold standard. In comparison to volunteers, the PAH patients showed significantly reduced pulmonary velocities (P = 0.002), blood flow (P = 0.002) and pulmonary distensibility (P = 0.008). In patients, the time to peak velocity was shorter (P<0.001), and the velocity rise gradient was steeper (P = 0.002) than in volunteers. While in volunteers the peak velocity in the aorta was reached earlier, it was the reverse in patients. Patients showed a significant bronchosystemic shunt (P = 0.01). No meaningful correlation was found between MRI measurements and echocardiography or RHC. MRI is a feasible technique for the differentiation between PAH and volunteers. Further studies have to be conducted for the absolute calculation of pressure estimates. (orig.)

  13. Bronchial artery ligation modifies pulmonary edema after exposure to smoke with acrolein.

    Science.gov (United States)

    Hales, C A; Barkin, P; Jung, W; Quinn, D; Lamborghini, D; Burke, J

    1989-09-01

    Pulmonary edema can follow smoke inhalation and is believed to be due to the multiple chemical toxins in smoke, not the heat. We have developed a synthetic smoke composed of aerosolized charcoal particles to which one toxin at a time can be added to determine whether it produces pulmonary edema. Acrolein, a common component of smoke, when added to the synthetic smoke, produced a delayed-onset pulmonary edema in dogs in which the extravascular lung water (EVLW) as detected by a double-indicator technique began to rise after 42 +/- 2 (SE) min from 148 +/- 16 to 376 +/- 60 ml at 165 min after smoke exposure. The resulting pulmonary edema was widespread macroscopically but appeared focal microscopically with fibrin deposits in alveoli adjacent to small bronchi and bronchioles. Bronchial vessels were markedly dilated and congested. Monastral blue B when injected intravenously leaked into the walls of the bronchial vessels down to the region of the small bronchioles (less than or equal to 0.5 mm ID) of acrolein-smoke-exposed dogs but not into the pulmonary vessels. Furthermore, ligation of the bronchial arteries delayed the onset of pulmonary edema (87 +/- 3 min, P less than 0.05) and lessened the magnitude (232 +/- 30 ml, P less than 0.05) at 166 +/- 3 min after acrolein-smoke exposure.(ABSTRACT TRUNCATED AT 250 WORDS)

  14. Pulmonary complications in patients undergoing coronary artery bypass grafting at a hospital in Maceio, Brazil

    Directory of Open Access Journals (Sweden)

    Ana Carolina do Nascimento Calles

    Full Text Available Abstract Introduction: Despite technological advances aimed at reducing complications and hospital stay, pulmonary complications in the postoperative period of CABG surgery remain one of the most important causes of morbidity. These may be related to the patient's quality of life, the use of cardiopulmonary bypass, as well as a decline in postoperative respiratory muscle strength, contributing to the emergence of restrictive respiratory disorders. Objective: Assess pulmonary complications in patients undergoing coronary artery bypass graft surgery and identify their association with risk factors. Methods: A retrospective study with non-probability convenience sampling of 168 patients at the Hospital do Coração of Alagoas, undergoing CABG between January 2009 and October 2013. Results: The 168 patients undergoing CABG had a mean age of 60.08 ± 10.03 years, 24.4% women and 75.6% men. Among the cardiovascular risk factors were: Hypertension (89.9%; Heredity for cardiovascular disease (54.8%; Diabetes Mellitus (45.2%; Dyslipidemia (41.1%; Smoking (32.1%; and Stroke (1.2%. Pulmonary complications after surgery include pleural effusion (17.9%; atelectasis (5.4%; pneumothorax, pulmonary embolism, and acute respiratory failure (1.2%; and bronchopneumonia (0.6%. Female patients and those aged 70 years or older experienced more pulmonary complications after surgery. Conclusion: Women and patients older than 70 years had the most severe postoperative pulmonary complications, the most common being pleural effusion and atelectasis.

  15. Anomalous origin of the right coronary artery from the pulmonary artery: an autopsied sudden death case with severe atherosclerotic disease of the left coronary artery.

    Science.gov (United States)

    Nagai, T; Mukai, T; Takahashi, S; Takada, A; Saito, K; Harada, K; Mori, S; Abe, N

    2014-03-01

    Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare anomaly. It may contribute to myocardial ischemia or sudden death, although the lesion is usually asymptomatic. We report a sudden death case of a 58-year-old man with ARCAPA coexisting with severe atherosclerotic coronary artery disease. He had been healthy until he complained of chest pain, several days before death, despite the discovery of heart murmur in childhood and suspicion of valvular heart disease. The autopsy revealed not only typical findings of the right coronary anomaly with well-developed collateral circulations but also severe atherosclerotic lesions of the left coronary artery, and ischemic change of the myocardium in the left and right coronary arterial perfusion territory. In addition to the "coronary steal" phenomenon primarily caused by ARCAPA, the reduced flow of both coronary arteries and further increase of "coronary steal" due to atherosclerotic obstructive coronary disease might have contributed to the patient's death. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  16. Influence of changes in the pulmonary artery pressure on ventilation requirements in patients undergoing mitral valve replacement.

    Directory of Open Access Journals (Sweden)

    Muralidhar K

    2004-01-01

    Full Text Available The study was designed to evaluate the influence of changes in pulmonary artery pressure on the ventilation requirements in patients undergoing mitral valve surgery. Thirty patients with mitral valve disease with significant pulmonary arterial hypertension undergoing mitral valve replacement under cardiopulmonary bypass were included in this prospective study. All patients had a pulmonary artery catheter placed after the anaesthetic induction. The minute ventilation was adjusted to achieve an arterial carbon dioxide tension (PaCO2 of 35-40 mm Hg. After a stabilisation period of 15 minutes, the pulmonary artery pressure and the minute volume needed for maintaining a PaCO2 of 35-40 mm Hg in the precardiopulmonary bypass, post-cardiopulmonary bypass and six hours postoperatively were measured after ensuring stable haemodynamics and normothermia. There was a significant decrease in the mean pulmonary artery pressure from pre-cardiopulmonary bypass value of 41.3+/-15 mm Hg to 29.3+/-8 mm Hg in the postcardiopulmonary bypass period and subsequently to 25.5+/-7 mm Hg in the intensive care unit. There was a corresponding increase in the minute volume requirements from a pre-cardiopulmonary bypass value of 6.8+/-1 L/min to 8.0+/-1 L/min in the post cardiopulmonary bypass period and then to 9.4+/-1.2 L/min in the postoperative period. We conclude that there is a significant decrease in the pulmonary blood volume and a subsequent decrease in the pulmonary artery pressure after a successful mitral valve replacement in patients with pulmonary arterial hypertension. This is associated with a significant increase in the requirement of minute ventilation to maintain normocarbia.

  17. Prostaglandin E1 Attenuates Pulmonary Artery Remodeling by Activating Phosphorylation of CREB and the PTEN Signaling Pathway

    OpenAIRE

    Lai, Ying-Ju; Hsu, Hsao-Hsun; Chang, Gwo-Jyh; Lin, Shu-Hui; Chen, Wei-Jan; Huang, Chung-Chi; Pang, Jong-Hwei S.

    2017-01-01

    The depletion of cyclic adenosine monophosphate (cAMP) response element binding protein (CREB) and phosphatase and tensin homolog (PTEN) is the critical mediator of pulmonary arterial hypertension (PAH). We hypothesized that the activation of phosphorylated CREB (pCREB) and PTEN could inhibit the AKT signaling pathway to attenuate pulmonary arterial remodeling in rats with monocrotaline-induced PAH. We observed decreased PTEN and pCREB in idiopathic PAH versus control tissue. We reduced PTEN ...

  18. Hilum-to-hilum Gore-Tex tube replacement of central pulmonary arteries.

    Science.gov (United States)

    Sughimoto, Koichi; Konstantinov, Igor E; Brizard, Christian P; d'Udekem, Yves

    2015-01-01

    Patients born with hypoplastic pulmonary arteries require recurrent procedures of shunting, patch reconstructions, balloon dilatations, and occasionally stenting to achieve adult-size vessels. We have applied a hilum-to-hilum Gore-Tex conduit replacement for the stenosed central pulmonary arteries to 12 consecutive patients with a Gore-Tex tube of 14 mm (9 patients) or 12 mm (3 patients) at a median age of 6.7 years (range, 1.6 to 16.9). There were 8 patients with biventricular repair (2 patients with heart transplantation) and 4 patients with Fontan completions. After a follow-up time of 25 ± 22 months, there was no mortality, reintervention, or restenosis. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  19. Quercetin Inhibits Pulmonary Arterial Endothelial Cell Transdifferentiation Possibly by Akt and Erk1/2 Pathways

    Directory of Open Access Journals (Sweden)

    Shian Huang