scholarly journals Pulmonary Hypertension and Its Response to Treatment in a Patient with Kyphosis-related Alveolar Hypoventilation

2018 ◽  
Vol 57 (7) ◽  
pp. 1003-1006
Author(s):  
Shinji Okada ◽  
Ayumi Sugawara ◽  
Shunsuke Yamagata ◽  
Satoshi Takeuchi ◽  
Zenta Watanuki
Keyword(s):  
Pulmonary Hypertension ◽  
Response To Treatment ◽  
Alveolar Hypoventilation

scholarly journals Pulmonary Hypertension Associated With Scleroderma and Connective Tissue Disease: Potential Molecular and Cellular Targets

2017 ◽  
Vol 16 (2) ◽  
pp. 61-67
Author(s):  
Maria Trojanowska
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Molecular Mechanisms ◽  
Critical Role ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Response To Treatment ◽  
Organ Systems

Systemic sclerosis (SSc) is characterized by autoimmunity, small-vessel vasculopathy, and fibrosis causing damage in multiple organ systems. Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of SSc, occurring in patients with the limited (lcSSc) and diffuse (dcSSc) forms of the disease and affecting 8% to 15% of patients.12 While pulmonary hypertension associated with connective tissue disease (CTD-PAH) has similar clinical features as idiopathic PAH, 1-year survival and freedom from hospitalization are lower in CTD-PAH.3 SSc-PAH has the worst 1-year survival rate at 82% compared with other connective tissue diseases, including systemic lupus erythematosus, mixed connective tissue disease, and rheumatoid arthritis.34 Despite the recent progress in the development of disease-targeted therapies, patients with SSc-PAH have a poorer response to treatment and a worse prognosis than other subgroups of PAH.1 Autoimmunity and prolonged vasculopathy preceding the development of clinical manifestations of SSc-PAH may play a critical role in the poorer outcome of SSc-PAH patients.1 This article will provide an overview of the recent findings related to cellular and molecular mechanisms associated with the development of PAH, with an emphasis on SSc-PAH.

scholarly journals Sex Differences in Pulmonary Hypertension

2021 ◽  
Vol 2 ◽  
Author(s):  
Juan José Rodriguez-Arias ◽  
Ana García-Álvarez
Keyword(s):  
Pulmonary Hypertension ◽  
Sex Differences ◽  
Pulmonary Arterial Hypertension ◽  
Animal Models ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Gender Bias ◽  
Response To Treatment ◽  
Experimental Animal Models ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) includes multiple diseases that share as common characteristic an elevated pulmonary artery pressure and right ventricular involvement. Sex differences are observed in practically all causes of PH. The most studied type is pulmonary arterial hypertension (PAH) which presents a gender bias regarding its prevalence, prognosis, and response to treatment. Although this disease is more frequent in women, once affected they present a better prognosis compared to men. Even if estrogens seem to be the key to understand these differences, animal models have shown contradictory results leading to the birth of the estrogen paradox. In this review we will summarize the evidence regarding sex differences in experimental animal models and, very specially, in patients suffering from PAH or PH from other etiologies.

Chronic Alveolar Hypoventilation Secondary to Macroglossia in the Beckwith-Wiedemann Syndrome

PEDIATRICS ◽  
1982 ◽  
Vol 70 (5) ◽  
pp. 695-697 ◽  
Author(s):  
Dean F. Smith ◽  
Frederick G. Mihm ◽  
Michael Flynn
Keyword(s):  
Pulmonary Hypertension ◽  
Airway Obstruction ◽  
Early Recognition ◽  
Upper Airway ◽  
Upper Airway Obstruction ◽  
Morbidity And Mortality ◽  
Airway Compromise ◽  
Alveolar Hypoventilation

Chronic upper airway obstruction has been shown to cause secondary reversible pulmonary hypertension. Many pathophysiologic processes can produce such obstruction. A 3-month-old child with Beckwith-Wiedemann syndrome who manifested chronic upper airway obstruction secondary to macroglossia is reported. Early recognition and therapy of airway compromise may decrease morbidity and mortality in this syndrome and others in which macroglossia is present.

Clinical Profile, Prognostic Implication, and Response to Treatment of Pulmonary Hypertension in Patients With Severe Aortic Stenosis

2011 ◽  
Vol 107 (7) ◽  
pp. 1046-1051 ◽  
Author(s):  
Itsik Ben-Dor ◽  
Steven A. Goldstein ◽  
Augusto D. Pichard ◽  
Lowell F. Satler ◽  
Gabriel Maluenda ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Aortic Stenosis ◽  
Severe Aortic Stenosis ◽  
Clinical Profile ◽  
Response To Treatment ◽  
Prognostic Implication

scholarly journals Non-invasive imaging of global and regional cardiac function in pulmonary hypertension

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321774200 ◽  
Author(s):  
Tim Crowe ◽  
Geeshath Jayasekera ◽  
Andrew J. Peacock
Keyword(s):  
Pulmonary Hypertension ◽  
Cardiac Function ◽  
Left Ventricular ◽  
Cardiac Response ◽  
Response To Treatment ◽  
Advantages And Disadvantages ◽  
Non Invasive ◽  
Pulmonary Hemodynamic ◽  
Regional Cardiac Function ◽  
Rv Function

Pulmonary hypertension (PH) is a progressive illness characterized by elevated pulmonary artery pressure; however, the main cause of mortality in PH patients is right ventricular (RV) failure. Historically, improving the hemodynamics of pulmonary circulation was the focus of treatment; however, it is now evident that cardiac response to a given level of pulmonary hemodynamic overload is variable but plays an important role in the subsequent prognosis. Non-invasive tests of RV function to determine prognosis and response to treatment in patients with PH is essential. Although the right ventricle is the focus of attention, it is clear that cardiac interaction can cause left ventricular dysfunction, thus biventricular assessment is paramount. There is also focus on the atrial chambers in their contribution to cardiac function in PH. Furthermore, there is evidence of regional dysfunction of the two ventricles in PH, so it would be useful to understand both global and regional components of dysfunction. In order to understand global and regional cardiac function in PH, the most obvious non-invasive imaging techniques are echocardiography and cardiac magnetic resonance imaging (CMRI). Both techniques have their advantages and disadvantages. Echocardiography is widely available, relatively inexpensive, provides information regarding RV function, and can be used to estimate RV pressures. CMRI, although expensive and less accessible, is the gold standard of biventricular functional measurements. The advent of 3D echocardiography and techniques including strain analysis and stress echocardiography have improved the usefulness of echocardiography while new CMRI technology allows the measurement of strain and measuring cardiac function during stress including exercise. In this review, we have analyzed the advantages and disadvantages of the two techniques and discuss pre-existing and novel forms of analysis where echocardiography and CMRI can be used to examine atrial, ventricular, and interventricular function in patients with PH at rest and under stress.

scholarly journals Non-Group 1 Pulmonary Hypertension Associated With Systemic Sclerosis: An Under-studied Patient Population

2017 ◽  
Vol 16 (2) ◽  
pp. 68-75
Author(s):  
Zafia Anklesaria ◽  
Rajeev Saggar ◽  
Ariss Derhovanessian ◽  
Rajan Saggar
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
World Health ◽  
Response To Treatment ◽  
Specific Therapy ◽  
Pulmonary Manifestations ◽  
Group 3 ◽  
Group 2 ◽  
The Impact ◽  
Group 1

Background: Systemic sclerosis (SSc) is a heterogeneous disorder that results in multiorgan dysfunction. The most common pulmonary manifestations are pulmonary hypertension (PH) and interstitial lung disease (ILD). Systemic sclerosis may be complicated by World Health Organization (WHO) Group 1 PH (SSc-PAH), which is the most well-studied subtype. The PH associated with SSc may also be secondary to underlying left heart disease (SSc-PH-LHD) or ILD (SSc-PH-ILD), and these subgroups are classified as WHO Group 2 and Group 3 PH, respectively. These non-WHO Group 1 PH subsets are notoriously under-studied. Available data suggest that the impact of PH-specific therapy in SSc-PH-LHD and SSc-PH-ILD is limited and survival is poor despite attempted treatment. Implication for clinicians: Most research and clinical trials surrounding PH in SSc have thus far focused on WHO Group 1 SSc-PAH. There are limited data surrounding therapeutic options for WHO Group 2 (SSc-PH-LHD) and Group 3 PH (SSc-PH-ILD) phenotypes. This review aims to summarize and consolidate the data surrounding these 2 distinct clinical phenotypes and to emphasize the available prognostic and treatment considerations. Conclusions: Given the unique pathophysiology, prognostic implications, and poor response to treatment of WHO Group 2 and 3 SSc-PH phenotypes, there is an overwhelming need for more data to best understand optimal management strategies. The focus should be individual patient-level prognostication, how and when to initiate and manage PH-specific therapy, and appropriate triage with regard to the timing of lung (or heart-lung) transplantation.

A Rare Case of Primary Amyloidosis, Presenting with Severe Pulmonary Hypertension and Bilateral Pleural Effusion.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5100-5100
Author(s):  
Aref Agheli ◽  
Alka Arora ◽  
Maged Khalil ◽  
Theresa Dumlao ◽  
Seema Naik ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pleural Effusion ◽  
Basement Membranes ◽  
Bilateral Pleural Effusion ◽  
Response To Treatment ◽  
Primary Amyloidosis ◽  
X Rays ◽  
Severe Pulmonary Hypertension ◽  
Rare Presentation ◽  
History Of

Abstract Pulmonary arterial hypertension secondary to vascular deposition is a rare presentation of Primary Amyloidosis. The natural history of pulmonary hypertension and concomitant right-sided cardiac failure is not very well understood and is associated with a very short median survival. This syndrome is a marker of advanced Amyloidosis Persistent pleural effusion is another much rarer presentation of pulmonary involvement in Primary Amyloidosis and is secondary to pleural infiltration, which sometimes is difficult to distinguish from the cardiomyopathic causes. The incidence of pleural effusion in primary Amyloidosis is 1–2%. This syndrome also has a very poor prognosis, and has limited response to treatment, however, pleurodesis has been temporarily useful in some cases. Case report: A 74 year old African American female, non-smoker, with history of hypertension, who presented with shortness of breath and persistent productive cough and 18 pounds weight loss for the last six months. The initial chest x-rays showed bilateral pleural effusion and a transudate fluid on analysis. Cardiac workup for an atypical chest pain revealed moderate to severe pulmonary hypertension without myocardial infiltration on 2-D echocardiogram, and normal coronary arteries on cardiac catheterization. Extensive workup did not show any secondary cause of pulmonary hypertension, however, the patient gradually developed nephrotic range proteinuria and kidney biopsy showed infiltration of 10 nm fibrils, involving 3+ segmental mesangium, and 2+ segmental glomerular basement membranes. This was consistent with renal Primary Amyloidosis, AL Lambda type. A random urine and serum sample for immunofixation also revealed an M- spike of 23 mg/dl in gamma region. A concomitant bone marrow biopsy revealed presence of monoclonal IgG lambda plasma cell (CD38 bright) population, without any B-cell or T-cell markers. Conclusion: Pulmonary hypertension and pleural effusion are two rare and lethal presentations of pulmonary involvements in Primary Amyloidosis, and it should be considered in the differential diagnosis of any patient presenting with pulmonary symptoms. There is no definite treatment of these syndromes, although combinations of steroids and melphalan, along with colchicines, vincristine, adriamycin, and thalidomide have been used with minimum satisfactory outcomes. Autologous stem cell transplantation (SCT) provides the opportunity to give higher doses of chemotherapy and may be of benefit in some cases.

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