scholarly journals Severe Pulmonary Hypertension as Initial Presentation of SLE: A Case Report and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Rabia Kiani ◽  
Muhammad Danial Siddiqui ◽  
Hamza Tantoush
Keyword(s):  
Pulmonary Hypertension ◽  
Lupus Erythematosus ◽  
Early Recognition ◽  
Treatment Options ◽  
Right Heart Failure ◽  
Young Female ◽  
Pulmonary Artery Hypertension ◽  
Initial Presentation ◽  
Severe Pulmonary Hypertension ◽  
Appropriate Treatment

Severe pulmonary artery hypertension (PAH) is a rare initial presentation of systemic lupus erythematosus (SLE). SLE associated with PAH carries worse prognosis that isolated SLE. However, there has been improvement in mortality of the patients in the recent years owing to newer treatment options available. Early recognition remains of prime importance. We present here a case of young female who presented with severe pulmonary hypertension with right heart failure leading to cardiogenic shock and was found to have SLE. She was started on appropriate treatment; however, given the severity of her illness, the patient did not survive. This case highlights the importance of early recognition and prompt treatment of SLE-associated PAH, which might improve the survival rate in the patients.

scholarly journals Pulmonary Hypertension in Acute and Chronic High Altitude Maladaptation Disorders

2021 ◽  
Vol 18 (4) ◽  
pp. 1692
Author(s):  
Akylbek Sydykov ◽  
Argen Mamazhakypov ◽  
Abdirashit Maripov ◽  
Djuro Kosanovic ◽  
Norbert Weissmann ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
High Altitude ◽  
Clinical Experience ◽  
Current Knowledge ◽  
Hypoxic Pulmonary Vasoconstriction ◽  
Treatment Options ◽  
Right Heart Failure ◽  
Channel Blockers ◽  
The Right ◽  
Extensive Clinical Experience

Alveolar hypoxia is the most prominent feature of high altitude environment with well-known consequences for the cardio-pulmonary system, including development of pulmonary hypertension. Pulmonary hypertension due to an exaggerated hypoxic pulmonary vasoconstriction contributes to high altitude pulmonary edema (HAPE), a life-threatening disorder, occurring at high altitudes in non-acclimatized healthy individuals. Despite a strong physiologic rationale for using vasodilators for prevention and treatment of HAPE, no systematic studies of their efficacy have been conducted to date. Calcium-channel blockers are currently recommended for drug prophylaxis in high-risk individuals with a clear history of recurrent HAPE based on the extensive clinical experience with nifedipine in HAPE prevention in susceptible individuals. Chronic exposure to hypoxia induces pulmonary vascular remodeling and development of pulmonary hypertension, which places an increased pressure load on the right ventricle leading to right heart failure. Further, pulmonary hypertension along with excessive erythrocytosis may complicate chronic mountain sickness, another high altitude maladaptation disorder. Importantly, other causes than hypoxia may potentially underlie and/or contribute to pulmonary hypertension at high altitude, such as chronic heart and lung diseases, thrombotic or embolic diseases. Extensive clinical experience with drugs in patients with pulmonary arterial hypertension suggests their potential for treatment of high altitude pulmonary hypertension. Small studies have demonstrated their efficacy in reducing pulmonary artery pressure in high altitude residents. However, no drugs have been approved to date for the therapy of chronic high altitude pulmonary hypertension. This work provides a literature review on the role of pulmonary hypertension in the pathogenesis of acute and chronic high altitude maladaptation disorders and summarizes current knowledge regarding potential treatment options.

scholarly journals Genistein, a Soy Phytoestrogen, Reverses Severe Pulmonary Hypertension and Prevents Right Heart Failure in Rats

Hypertension ◽  
2012 ◽  
Vol 60 (2) ◽  
pp. 425-430 ◽  
Author(s):  
Humann Matori ◽  
Soban Umar ◽  
Rangarajan D. Nadadur ◽  
Salil Sharma ◽  
Rod Partow-Navid ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Severe Pulmonary Hypertension ◽  
Right Heart

Pulmonary Hypertension Associated With Myocardial Amyloid Degeneration: a Case Report

2021 ◽  
Author(s):  
Lu Yan ◽  
Qixian Zeng ◽  
Changming Xiong ◽  
Zhihui Zhao ◽  
Qing Zhao ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Blood Vessels ◽  
Amyloid Fibrils ◽  
Right Heart Failure ◽  
Pulmonary Artery Hypertension ◽  
Pulmonary Vasculature ◽  
Vascular Involvement ◽  
Right Heart ◽  
Protein Deposits

Abstract Background: There is very little literature on Pulmonary hypertension associated with myocardial amyloid degeneration. At present, only 10 cases pulmonary hypertension cased by amyloid protein deposits in the pulmonary blood vessels have been reported by Eder et al. We reported a case that the patient was pulmonary artery hypertension combined with myocardial amyloid change. It’s aim to claims that pulmonary hypertension is most likely caused by amyloid fibrin deposition in pulmonary blood vessels.Case presentation: We report a case of a 65-year-old male patient with with AL and ATTR combined type amyloidosis who developed right heart failure because of severe pulmonary hypertension. Pulmonary hypertension due to deposition of amyloid in the pulmonary vasculature is an uncommon finding; however, it should be considered in cases of unexplained pulmonary hypertension in patients with amyloidosis.Conclusion: we present a men with amyloidosis who developed dyspnea and right heart failure and was diagnosed with pulmonary hypertension, most probably secondary to pulmonary vascular involvement by amyloid fibrils.

Unilateral interruption of pulmonary artery with pulmonary hypertension: a case for heart lung transplantation?

2019 ◽  
Vol 29 (5) ◽  
pp. 704-707 ◽  
Author(s):  
Roopesh Singhal
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Lung Transplantation ◽  
Congenital Heart ◽  
Right Heart Failure ◽  
Adverse Outcomes ◽  
Severe Pulmonary Hypertension ◽  
Right Heart ◽  
Right Pulmonary Artery

AbstractUnilateral interruption of pulmonary artery is a rare congenital anomaly which is usually associated with other congenital heart disease. Even more rarely it may occur in isolation. Most of the cases are incidentally detected in adulthood. Some cases develop pulmonary hypertension for yet unknown reasons; such cases usually present in infancy with right heart failure. Surgical correction in such cases is associated with adverse outcomes. Heart lung transplantation should be considered in such patients. We report a 3-year-old boy with interruption of right pulmonary artery with severe pulmonary hypertension and right heart failure who was considered for heart lung transplantation.

scholarly journals Hyperthyroidism with Biventricular Heart Failure and Cirrhotic Transformation of the Liver

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Rashmi Dhital ◽  
Shivani Vyas ◽  
Priyadarshani Sharma ◽  
Theresa Lynn ◽  
Oreoluwa Oladiran ◽  
...  
Keyword(s):  
Early Recognition ◽  
Treatment Options ◽  
Young Female ◽  
Good Prognosis ◽  
Thyroid Peroxidase ◽  
Free T4 ◽  
Gradual Improvement ◽  
Fluid Analysis ◽  
Cardiac Cirrhosis ◽  
Congestive Liver

Cardiovascular symptoms remain the most common presenting features and leading causes of death in hyperthyroidism. We report a young female with reported thyroid disease and medication noncompliance presenting with atrial fibrillation, severe atrioventricular regurgitation, severely dilated right heart with reduced function, and moderate pulmonary hypertension (PH), which was further complicated by congestive liver injury with ascites and pancytopenia. Thyroid work-up revealed suppressed TSH, elevated free T4 and T3 along with elevated anti-thyroglobulin antibodies, thyroid peroxidase antibodies, and thyroid-stimulating immunoglobulin, suggesting Graves’ thyrotoxicosis. Ultrasound of the abdomen was suggestive of liver cirrhosis and ascites, which was thought to be cardiac cirrhosis, after multiple negative work-ups for alternate causes of cirrhosis. Ascitic fluid analysis revealed portal hypertension as the cause. The patient was restarted on antithyroid medication with gradual improvement of thyroid function and in clinical and echocardiogram findings. In contrast to primary PH that carries a poor prognosis and has limited treatment options, PH due to Graves’ disease carries a good prognosis with prior reports of resolution after appropriate treatment, emphasizing the importance of early recognition. Also, unlike cirrhosis caused by alcohol or viral hepatitis, the effect of cardiac cirrhosis on overall prognosis has not been clearly established.

Systemic lupus and catastrophic antiphospholipid syndrome manifesting as cardiogenic shock

Lupus ◽  
2019 ◽  
Vol 28 (11) ◽  
pp. 1350-1353 ◽  
Author(s):  
M Schultz ◽  
K Wimberly ◽  
M Guglin
Keyword(s):  
Cardiogenic Shock ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Early Recognition ◽  
Case Series ◽  
Young Female ◽  
Systemic Lupus ◽  
Rare Manifestation ◽  
Autoimmune Condition ◽  
Peripartum Period

In this case series we present two young female patients presenting in the peripartum period with cardiogenic shock. Both patients had underlying autoimmune diseases, one with systemic lupus erythematosus (SLE) and the other with antiphospholipid syndrome (APS). In both cases cardiogenic shock was a direct manifestation of their autoimmune condition, and with prompt diagnosis and management both patients were able to recover. This case series illustrates the importance of early recognition of cardiogenic shock as a rare manifestation both of SLE and APS.

scholarly journals Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective

2016 ◽  
Vol 140 (3) ◽  
pp. 230-240 ◽  
Author(s):  
Anja C. Roden ◽  
Eunhee S. Yi
Keyword(s):  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Additional Treatment ◽  
Langerhans Cell ◽  
Braf V600e ◽  
First Line ◽  
Clonal Proliferation ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Appropriate Treatment

Context Pulmonary Langerhans cell histiocytosis (PLCH) is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. PLCH is characterized by bronchiolocentric nodules and/or cysts in an upper and mid lung distribution with sparing of the costophrenic angles. The diagnosis can be challenging and often requires transbronchial biopsy or surgical lung biopsy. Pulmonary hypertension is a relatively common and sometimes severe complication of PLCH. The pathogenesis of PLCH is still debated. Recently, BRAF V600E mutation and BRAF expression have been identified in some patients with PLCH, suggesting that at least a subset of PLCH has a clonal proliferation. While smoking cessation is the first-line treatment of PLCH, some patients might require additional treatment and eventually transplant. Given that the lesional cells of PLCH express BRAF in some patients, MAPKinase pathway–targeted treatment might be useful for therapy-resistant patients. Objective —To present the more recently recognized clinical and pathologic aspects of PLCH, including pulmonary hypertension in PLCH, pathogenesis, and treatment, as well as the basic diagnostic approach to PLCH. Data Sources Authors' own research, and search of literature database (PubMed) and UpToDate. Conclusions —Despite the recent progress, more studies are needed to elucidate the biology of PLCH for identification of prognostic factors and appropriate treatment options, especially for therapy-refractory PLCH cases.

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