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1.
BMJ Case Rep ; 14(9)2021 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-34493555

RESUMO

We report a case of a previously fit woman who presented at 26 weeks into her fourth pregnancy with a dry cough. Following a nasopharyngeal swab, she was diagnosed with a pertussis infection, and treated with antibiotics. A chest X-ray showed right atrial dilatation and an echocardiogram was scheduled outpatient. However, after re-presenting with worsening cough and dyspnoea, an inpatient echocardiogram was performed which suggested elevated pulmonary pressures with significant tricuspid regurgitation, as confirmed by subsequent cardiac catheterisation. She had an elective caesarean section at 34 weeks and underwent repeat right heart catheterisation which revealed persistent, and likely pre-existing, pulmonary arterial hypertension. This case highlights the importance of thorough assessment of non-obstetric symptoms in pregnancy in formulating alternative differentials, even after a diagnosis has been made, to prevent potentially life-threatening conditions from being missed. It also shows that although often associated, respiratory and cardiac causes may coexist separately.


Assuntos
Hipertensão Pulmonar , Insuficiência da Valva Tricúspide , Coqueluche , Cesárea , Ecocardiografia , Feminino , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Gravidez , Coqueluche/complicações , Coqueluche/diagnóstico , Coqueluche/tratamento farmacológico
3.
Hematology ; 26(1): 684-690, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34493173

RESUMO

BACKGROUND: Sickle cell anaemia affects about 4 million people across the globe, making it an inherited disorder of public health importance. Red cell lysis consequent upon haemoglobin crystallization and repeated sickling leads to anaemia and a baseline strain on haemopoiesis. Vaso-occlusion and haemolysis underlies majority of the chronic complications of sickle cell. We evaluated the clinical and laboratory features observed across the various clinical phenotypes in adult sickle cell disease patients. METHODS: Steady state data collected prospectively in a cohort of adult sickle cell disease patients as out-patients between July 2010 and July 2020. The information included epidemiological, clinical and laboratory data. RESULTS: About 270 patients were captured in this study (165 males and 105 females). Their ages ranged from 16 to 55 years, with a median age of 25 years. Sixty-eight had leg ulcers, 43 of the males had priapism (erectile dysfunction in 8), 42 had AVN, 31 had nephropathy, 23 had osteomyelitis, 15 had osteoarthritis, 12 had cholelithiasis, 10 had stroke or other neurological impairment, 5 had pulmonary hypertension, while 23 had other complications. Frequency of crisis ranged from 0 to >10/year median of 2. Of the 219 recorded, 148 of the patients had been transfused in the past, while 71 had not. CONCLUSION: The prevalence of SLU, AVN, priapism, nephropathy and the other complications of SCD show some variations from other studies. This variation in the clinical parameters across different clinical phenotypes indicates an interplay between age, genetic and environmental factors.


Assuntos
Anemia Falciforme , Adolescente , Adulto , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Anemia Falciforme/metabolismo , Anemia Falciforme/patologia , Colelitíase/etiologia , Colelitíase/metabolismo , Colelitíase/patologia , Feminino , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/metabolismo , Hipertensão Pulmonar/patologia , Nefropatias/epidemiologia , Nefropatias/etiologia , Nefropatias/metabolismo , Nefropatias/patologia , Úlcera da Perna/epidemiologia , Úlcera da Perna/etiologia , Úlcera da Perna/metabolismo , Úlcera da Perna/patologia , Masculino , Pessoa de Meia-Idade , Nigéria/epidemiologia , Osteoartrite/epidemiologia , Osteoartrite/etiologia , Osteoartrite/metabolismo , Osteomielite/epidemiologia , Osteomielite/etiologia , Osteomielite/metabolismo , Osteomielite/patologia , Priapismo/epidemiologia , Priapismo/etiologia , Priapismo/metabolismo , Priapismo/patologia , Estudos Prospectivos , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/metabolismo , Acidente Vascular Cerebral/patologia
4.
Angiol Sosud Khir ; 27(3): 16-21, 2021.
Artigo em Russo | MEDLINE | ID: mdl-34528584

RESUMO

This article is a review of the findings of experimental and clinical studies of a new method of treatment of pulmonary hypertension - pulmonary artery denervation with the help of radiofrequency ablation, cryodenervation and ultrasonic impact. Pulmonary artery denervation results in decreased neurogenic tonic sympathetic and, probably, increased parasympathetic effects on pulmonary vessels. On models of experimental monocrotaline-induced pulmonary hypertension in various-species animals, it was determined that pulmonary artery denervation is followed by decreased activity of local pulmonary renin-angiotensin system, slowed processes of remodeling of pulmonary vessels, hypertrophy and fibrosis of the right ventricle, with inhibition of progression of pulmonary hypertension by means of suppression of extracellular signal-regulated kinase 1/2 (ERK 1/2) which regulates differentiation, proliferation and migration of smooth muscle cells. However, the problem of the pattern of pulmonary microcirculation changes (pre- and postcapillary resistance, capillary filtration coefficient) after pulmonary artery denervation warrants further study. The findings of clinical studies in patients with pulmonary hypertension suggest that pulmonary artery denervation inducing a decrease of pressure therein, as well as pulmonary vessel resistance did not lead to normalization of pulmonary haemodynamics.The mentioned impact partially removes the neurogenic component of multicircuit and multifactorial regulation of pulmonary circulation. Therefore, along with pulmonary artery denervation, further search for pharmacological agents selectively influencing pulmonary vessels remains a problem of current importance.


Assuntos
Hipertensão Pulmonar , Animais , Denervação , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/cirurgia , Monocrotalina , Artéria Pulmonar/cirurgia , Resistência Vascular
6.
Curr Cardiol Rep ; 23(10): 147, 2021 08 19.
Artigo em Inglês | MEDLINE | ID: mdl-34410530

RESUMO

PURPOSE OF REVIEW: Chronic thromboembolic pulmonary hypertension (CTEPH), included in group 4 PH, is an uncommon complication of acute pulmonary embolism (PE), in which emboli in the pulmonary vasculature do not resolve but rather form into an organized scar-like obstruction which can result in right ventricular (RV) failure. Here we provide an overview of current diagnosis and management of CTEPH. RECENT FINDINGS: CTEPH management is complex with treatments that range from surgery, percutaneous interventions, to medical therapies. Current CTEPH medical therapies have largely been repurposed from pulmonary arterial hypertension (PAH). The diagnosis of CTEPH can be challenging, requiring a multimodality approach to differentiate from disease mimics. While these treatments improve symptoms, they may not reverse the underlying pathology of CTEPH.


Assuntos
Angioplastia com Balão , Hipertensão Pulmonar , Embolia Pulmonar , Doença Crônica , Endarterectomia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/terapia
7.
Am J Case Rep ; 22: e932956, 2021 Aug 26.
Artigo em Inglês | MEDLINE | ID: mdl-34433800

RESUMO

BACKGROUND Myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), are associated with pulmonary hypertension (PH) and malignant lymphomas. Although the underlying mechanisms have not been completely clarified, it has been suggested that the Janus kinase 2 (JAK2) mutation, which is frequently identified in PV, can be involved in the development and/or progression of these distinct diseases in patients with MPNs. However, no reports have described the coexistence of PH and malignant lymphoma in patients with MPNs. CASE REPORT A 79-year-old man being treated for PV for 27 years and PH for 5 years was hospitalized due to severe dyspnea at rest. His soluble interleukin-2 receptor levels gradually increased and the chest computed tomography showed remarkable progression of the lung lesions and an enlargement of the mediastinal and axillary lymph nodes. A lymph node biopsy was performed and the patient was diagnosed with diffuse large B-cell lymphoma (DLBCL). Owing to his poor condition, chemotherapy was not initiated, and he died on the 89th day of hospitalization. The pathological autopsy revealed the destruction of alveolar structures with neoplastic space-occupying lesions of DLBCL. Multifactorial features of PH associated with MPNs, including the intimal thickening of pulmonary arteries accompanied by megakaryocytes and obstructed pulmonary arteries with organized thrombi in the lung tissue specimens, were observed. We found a JAK2 mutation based on a genetic analysis of the patient's bone marrow. CONCLUSIONS We present the rare case of a patient who had PV with a JAK2 mutation, which coexisted with PH and DLBCL, and he developed severe refractory respiratory failure.


Assuntos
Hipertensão Pulmonar , Linfoma Difuso de Grandes Células B , Transtornos Mieloproliferativos , Policitemia Vera , Idoso , Medula Óssea , Humanos , Hipertensão Pulmonar/etiologia , Linfoma Difuso de Grandes Células B/complicações , Masculino , Policitemia Vera/complicações , Policitemia Vera/genética
8.
Int J Mol Sci ; 22(16)2021 Aug 11.
Artigo em Inglês | MEDLINE | ID: mdl-34445357

RESUMO

The biological activities of interleukins, a group of circulating cytokines, are linked to the immuno-pathways involved in many diseases. Mounting evidence suggests that interleukin-1ß (IL-1ß) plays a significant role in the pathogenesis of various types of hypertension. In this review, we summarized recent findings linking IL-1ß to systemic arterial hypertension, pulmonary hypertension, and gestational hypertension. We also outlined the new progress in elucidating the potential mechanisms of IL-1ß in hypertension, focusing on it's regulation in inflammation, vascular smooth muscle cell function, and extracellular remodeling. In addition, we reviewed recent studies that highlight novel findings examining the function of non-coding RNAs in regulating the activity of IL-1ß and its associated proteins in the setting of hypertension. The information collected in this review provides new insights into understanding the pathogenesis of hypertension and could lead to the discovery of new anti-hypertensive therapies to combat this highly prevalent disease.


Assuntos
Hipertensão/etiologia , Interleucina-1beta/fisiologia , Animais , Matriz Extracelular/patologia , Matriz Extracelular/fisiologia , Feminino , Regulação da Expressão Gênica , Humanos , Hipertensão/patologia , Hipertensão/fisiopatologia , Hipertensão Induzida pela Gravidez/etiologia , Hipertensão Induzida pela Gravidez/patologia , Hipertensão Induzida pela Gravidez/fisiopatologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/patologia , Hipertensão Pulmonar/fisiopatologia , Inflamação/complicações , Inflamação/fisiopatologia , Interleucina-1beta/genética , Músculo Liso Vascular/fisiopatologia , Gravidez , RNA não Traduzido/fisiologia , Remodelação Vascular/fisiologia
9.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 46(7): 774-779, 2021 Jul 28.
Artigo em Inglês, Chinês | MEDLINE | ID: mdl-34382596

RESUMO

The clinical data for a patient with pulmonary hypertension complicating mediastinal fibrosis secondary to tuberculosis admitted to the Second Xiangya Hospital, Central South University has been retrospectively analyzed, and the relevant literature has been reviewed. A 55-year-old Han Chinese woman initially presented with increasing shortness of breath on exertion for 7 months was admitted to our hospital in August 2013. Admission examination revealed an increased erythrocyte sedimentation rate, positive in T-SPOT TB test, multiform lesions in both lungs, the enlarged lymph nodes with calcification in mediastinum and bilateral hilar, the narrowed bilateral main bronchial lumen, and the thickened bilateral pleural, as well as adhesion band in the left pleural cavity under the thoracoscope. These results indicated that mycobacterium tuberculosis infection may be present. After 4 months of anti-tuberculosis treatment, there is no improvement in symptom of the patient. In 2016, the patient was admitted to our hospital again because of shortness of breath after exercise. The computed tomography pulmonary angiography revealed ill-defined soft tissue density lesions with calcification on bilateral hilar and mediastinum, encasing the major mediastinal vascular structures. The lesions compressed and wrapped the pulmonary artery and vein branches, resulting in their lumens narrowed severely. Moreover, the main pulmonary artery trunk was widened and the right ventricular was enlarged significantly. The patient was finally diagnosed as mediastinal fibrosis with the potential pathogenic mechanisms being tuberculosis, secondary pulmonary hypertension. Mediastinal fibrosis is an uncommon, benign and progressive condition characterized by an invasive proliferation of the fibrous tissue within the mediastinum, which can lead to pulmonary hypertension. Due to lack of specific clinical manifestations, diagnosis is difficult in the early stage. Physicians should pay attention to mediastinal fibrosis when accepting patients with unexplained pulmonary hypertension. Contrast-enhanced CT should be performed as early as possible to avoid mis-diagnosis or missed diagnosis.


Assuntos
Hipertensão Pulmonar , Mediastinite , Tuberculose , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Mediastinite/complicações , Mediastinite/diagnóstico por imagem , Mediastino/diagnóstico por imagem , Pessoa de Meia-Idade , Estudos Retrospectivos , Esclerose
10.
Curr Cardiol Rep ; 23(10): 141, 2021 08 19.
Artigo em Inglês | MEDLINE | ID: mdl-34410515

RESUMO

PURPOSE OF REVIEW: Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon complication of acute pulmonary embolism (PE), in which the red, platelet-rich thrombus does not resolve but forms into an organized yellow, fibrotic scar-like obstruction in the pulmonary vasculature. Here we review the pathobiology of CTEPH. RECENT FINDINGS: Our current knowledge has predominantly been informed by studies of human samples and animal models that are inherently limited in their ability to recapitulate all aspects of the disease. These studies have identified alterations in platelet biology and inflammation in the formation of a scar-like thrombus that comprised endothelial cells, myofibroblasts, and immune cells, along with a small vessel pulmonary arterial hypertension-like vasculopathy. The development of CTEPH-specific therapies is currently hindered by a limited knowledge of its pathobiology. The development of new CTEPH medical therapies will require new insights into its pathobiology that bridge the gap from bench to bedside.


Assuntos
Hipertensão Pulmonar , Embolia Pulmonar , Tromboembolia , Animais , Doença Crônica , Células Endoteliais , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Embolia Pulmonar/complicações , Embolia Pulmonar/terapia , Tromboembolia/etiologia
11.
Harefuah ; 160(7): 415-418, 2021 Jul.
Artigo em Hebraico | MEDLINE | ID: mdl-34263566

RESUMO

INTRODUCTION: In patients diagnosed with pulmonary hypertension (PH), V/Q lung scintigraphy performed in Nuclear Medicine departments can demonstrate pulmonary embolisms and diagnose chronic thromboembolic pulmonary hypertension (CTEPH). Lung scintigraphy has higher sensitivity and specificity for CTEPH in comparison to other imaging methods, and is considered to be the method of choice in the workup of patients with unexplained PH. In the case presented, lung scintigraphy performed at two time points in a patient with PH demonstrated chronic bilateral pulmonary embolisms and aided in the diagnosis of CTEPH.


Assuntos
Hipertensão Pulmonar , Medicina Nuclear , Embolia Pulmonar , Doença Crônica , Dispneia/diagnóstico , Dispneia/etiologia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico , Cintilografia , Síncope
12.
J Card Surg ; 36(10): 3936-3938, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34291490

RESUMO

Pulmonary angiosarcoma (PAS) is a malignant tumor of the vascular wall of mesenchymal origin. PAS is rare and has unknown etiology and poor prognosis. Depending on the location, angiosarcoma can lead to serious obstructive and embolic complications, as well as severe pulmonary hypertension and right ventricular heart failure. Patients with PAS are often mistakenly diagnosed with pulmonary embolism, chronic thromboembolic pulmonary hypertension, or lung tumors. Here, we present a clinical case of combined treatment of PAS.


Assuntos
Hemangiossarcoma , Hipertensão Pulmonar , Embolia Pulmonar , Doença Crônica , Endarterectomia , Hemangiossarcoma/complicações , Hemangiossarcoma/diagnóstico , Hemangiossarcoma/cirurgia , Humanos , Hipertensão Pulmonar/etiologia
14.
Kardiologiia ; 61(6): 28-34, 2021 Jul 01.
Artigo em Russo, Inglês | MEDLINE | ID: mdl-34311685

RESUMO

Aim    To present clinical observations of the novel coronavirus infection (COVID-19) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) after a surgical intervention in the form of thromobendarterectomy from pulmonary artery branches.Material and methods    The Acad. E.N. Meshalkin National Medical Research Center performed 127 open surgical interventions for CTEPH in the form of thromobendarterectomy from 2016 through 2020. The present study enrolled 113 patients included into the follow-up care group and into the Center Registry who were followed up for more than 6 months after the surgery. Clinical and functional features of COVID-19 were evaluated in the studied group.Results    In the follow-up care group, 5 (4.4%) postoperative CTEPH patients had COVID-19. One patient had asymptomatic disease, and others had typical clinical symptoms and bilateral polysegmental pneumonia. There were no cases requiring artificial ventilation and no lethal outcomes. All patients with COVID-19 received anticoagulants as a basis therapy for CTEPH, and two patients who had residual pulmonary arterial hypertension (PAH) additionally received a PAH-specific therapy. During the treatment of COVID-19, no adjustment of the anticoagulant or PAH-specific therapy was required.Conclusion    The group of patients with CTEPH is a unique pathophysiological model for studying the effect of COVID-19 under the conditions of compromised pulmonary circulation. In the studied follow-up care group, the COVID-19 morbidity was 4.4 % without fatal outcomes. Evaluation of the role of chronic anticoagulant and PAH-specific therapy in COVID-19 postoperative patients as well as evaluation of the role of COVID-19 in CTEPH progression merit further investigation.


Assuntos
COVID-19 , Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Embolia Pulmonar , Doença Crônica , Humanos , Hipertensão Pulmonar/etiologia , Embolia Pulmonar/complicações , SARS-CoV-2
15.
Medicine (Baltimore) ; 100(27): e25915, 2021 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-34232164

RESUMO

ABSTRACT: Early right ventricular dysfunction in patients with non-advanced idiopathic pulmonary fibrosis (IPF) has not been fully elucidated. Thus, we aimed to assess right ventricular functions in IPF patients and controls by speckle-tracking strain echocardiography at rest and peak exercise.We screened 116 IPF patients from February to August 2019 to include 20 patients with no history of oxygen therapy, peripheral saturation levels ≥92% at rest, Gender-Age-Physiology Index score ≤5, and modified Medical Research Council score ≤3. Additionally, we enrolled 10 matched controls. Transthoracic echocardiography images were acquired at rest and during a cardiopulmonary exercise test. We analyzed 2-dimensional echocardiographic parameters and right ventricular function using the global longitudinal strain assessed by the 2-dimensional speckle-tracking technique.In the control group, we found normal values of right ventricle longitudinal strain (RVLS) at rest and at peak exercise, the latter being much more negative (-23.6 ±â€Š2.2% and -26.8 ±â€Š3.1%, respectively; P < .001). By contrast, RVLS values in the IPF group increased from -21.1 ±â€Š3.8% at rest to -17.0 ±â€Š4.5% at peak exercise (P < .001). The exercise revealed a difference between the 2 groups as the mean RVLS values moved during peak exercise in opposite directions. Patients with IPF got worse, whereas control patients presented improved right ventricular contractility.Right ventricular dysfunction was unveiled by speckle-tracking echocardiography during exercise in non-advanced IPF patients. We suggest that this reflects an inadequate right ventricular-arterial coupling decreasing the right ventricular longitudinal contraction during exercise in these patients. This parameter may be useful as an early index of suspected pulmonary hypertension.


Assuntos
Terapia por Exercício/métodos , Exercício Físico/fisiologia , Ventrículos do Coração/fisiopatologia , Hipertensão Pulmonar/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Contração Miocárdica/fisiologia , Função Ventricular Direita/fisiologia , Idoso , Estudos Transversais , Ecocardiografia , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Fibrose Pulmonar Idiopática/complicações , Masculino
16.
Artigo em Inglês | MEDLINE | ID: mdl-34300070

RESUMO

Acute high-altitude illnesses are of great concern for physicians and people traveling to high altitude. Our recent article "Acute Mountain Sickness, High-Altitude Pulmonary Edema and High-Altitude Cerebral Edema, a View from the High Andes" was questioned by some sea-level high-altitude experts. As a result of this, we answer some observations and further explain our opinion on these diseases. High-Altitude Pulmonary Edema (HAPE) can be better understood through the Oxygen Transport Triad, which involves the pneumo-dynamic pump (ventilation), the hemo-dynamic pump (heart and circulation), and hemoglobin. The two pumps are the first physiologic response upon initial exposure to hypobaric hypoxia. Hemoglobin is the balancing energy-saving time-evolving equilibrating factor. The acid-base balance must be adequately interpreted using the high-altitude Van Slyke correction factors. Pulse-oximetry measurements during breath-holding at high altitude allow for the evaluation of high altitude diseases. The Tolerance to Hypoxia Formula shows that, paradoxically, the higher the altitude, the more tolerance to hypoxia. In order to survive, all organisms adapt physiologically and optimally to the high-altitude environment, and there cannot be any "loss of adaptation". A favorable evolution in HAPE and pulmonary hypertension can result from the oxygen treatment along with other measures.


Assuntos
Doença da Altitude , Hipertensão Pulmonar , Edema Pulmonar , Altitude , Humanos , Hipertensão Pulmonar/etiologia , Hipóxia , Oxigênio , Edema Pulmonar/etiologia
17.
Medicine (Baltimore) ; 100(25): e26483, 2021 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-34160461

RESUMO

ABSTRACT: To investigate the importance of pulmonary vascular measurements on computed tomography (CT) in predicting pulmonary hypertension (PH) and worse outcomes in diffuse cystic lung diseases (DCLDs).We conducted a cross-sectional study of patients with DCLDs. Patients underwent pulmonary function tests, a six-minute walk test (6MWT), chest CT, transthoracic echocardiography, and right heart catheterization. Pulmonary artery (PA) diameter and PA-ascending aorta ratio (PA-Ao ratio) were obtained from CT. Mean pulmonary artery pressure (mPAP) from right heart catheterization was correlated with tomographic, functional, and echocardiographic variables. The association between the PA-Ao ratio with outcomes was determined by Kaplan-Meier curves.Thirty-four patients were included (18 with pulmonary Langerhans cell histiocytosis and 16 with lymphangioleiomyomatosis, mean age 46 ±â€Š9 years). Forced expiratory volume in the first second and lung diffusing capacity for carbon monoxide were 47 ±â€Š20% and 38 ±â€Š21% predicted, respectively. PA diameter and PA-Ao ratio were 29 ±â€Š6 mm and 0.95 ±â€Š0.24, respectively. PA-Ao ratio > 1 occurred in 38.2% of patients. PA-Ao ratio was a good predictor of PH. mPAP correlated best with PA-Ao ratio, PA diameter, oxygen desaturation during six-minute walk test, and echocardiographic variables. Patients with PA-Ao ratio > 1 had greater mPAP, and a higher risk of death or lung transplantation (log-rank, P < .001) than those with PA-Ao ratio ≤ 1.The PA-Ao ratio measured on CT scan has a potential role as a non-invasive tool to predict the presence of PH and as a prognostic parameter in patients with DCLDs.


Assuntos
Aorta/diagnóstico por imagem , Hipertensão Pulmonar/diagnóstico , Pneumopatias/complicações , Transplante de Pulmão/estatística & dados numéricos , Artéria Pulmonar/diagnóstico por imagem , Adulto , Aorta/patologia , Estudos Transversais , Ecocardiografia , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Pulmão/irrigação sanguínea , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pneumopatias/mortalidade , Pneumopatias/patologia , Pneumopatias/cirurgia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Artéria Pulmonar/patologia , Curva ROC , Medição de Risco/estatística & dados numéricos , Tomografia Computadorizada por Raios X , Teste de Caminhada
18.
Am J Physiol Heart Circ Physiol ; 321(2): H318-H338, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-34142886

RESUMO

Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by recurrent or unresolved pulmonary thromboemboli, leading to perfusion defects and increased arterial wave reflections. CTEPH treatment aims to reduce pulmonary arterial pressure and reestablish adequate lung perfusion, yet patients with distal lesions are inoperable by standard surgical intervention. Instead, these patients undergo balloon pulmonary angioplasty (BPA), a multisession, minimally invasive surgery that disrupts the thromboembolic material within the vessel lumen using a catheter balloon. However, there still lacks an integrative, holistic tool for identifying optimal target lesions for treatment. To address this insufficiency, we simulate CTEPH hemodynamics and BPA therapy using a multiscale fluid dynamics model. The large pulmonary arterial geometry is derived from a computed tomography (CT) image, whereas a fractal tree represents the small vessels. We model ring- and web-like lesions, common in CTEPH, and simulate normotensive conditions and four CTEPH disease scenarios; the latter includes both large artery lesions and vascular remodeling. BPA therapy is simulated by simultaneously reducing lesion severity in three locations. Our predictions mimic severe CTEPH, manifested by an increase in mean proximal pulmonary arterial pressure above 20 mmHg and prominent wave reflections. Both flow and pressure decrease in vessels distal to the lesions and increase in unobstructed vascular regions. We use the main pulmonary artery (MPA) pressure, a wave reflection index, and a measure of flow heterogeneity to select optimal target lesions for BPA. In summary, this study provides a multiscale, image-to-hemodynamics pipeline for BPA therapy planning for patients with inoperable CTEPH. NEW & NOTEWORTHY This article presents novel computational framework for predicting pulmonary hemodynamics in chronic thromboembolic pulmonary hypertension. The mathematical model is used to identify the optimal target lesions for balloon pulmonary angioplasty, combining simulated pulmonary artery pressure, wave intensity analysis, and a new quantitative metric of flow heterogeneity.


Assuntos
Hemodinâmica , Hipertensão Pulmonar/fisiopatologia , Artéria Pulmonar/fisiopatologia , Embolia Pulmonar/fisiopatologia , Angioplastia com Balão , Doença Crônica , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Modelos Cardiovasculares , Modelos Teóricos , Artéria Pulmonar/diagnóstico por imagem , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/terapia
19.
Am J Physiol Heart Circ Physiol ; 321(2): H309-H317, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-34170196

RESUMO

Pulmonary hypertension (PH) observed during respiratory syncytial virus (RSV) bronchiolitis is associated with morbidity and mortality, especially in children with congenital heart disease. Yet, the pathophysiological mechanisms of RSV-associated PH remain unclear. Therefore, this study aimed to investigate the pathophysiological mechanism of RSV-associated PH. We used a translational mouse model of RSV-associated PH, in which wild-type (WT) and suppression of tumorigenicity 2 (ST2) knockout neonatal mice were infected with RSV at 5 days old and reinfected 4 wk later. The development of PH in WT mice following RSV reinfection was evidenced by elevated right ventricle systolic pressure, shortened pulmonary artery acceleration time (PAT), and decreased PAT/ejection time (ET) ratio. It coincided with the augmentation of periostin and IL-13 expression and increased arginase bioactivity by both arginase 1 and 2 as well as induction of nitric oxide synthase (NOS) uncoupling. Absence of ST2 signaling prevented RSV-reinfected mice from developing PH by suppressing NOS uncoupling. In summary, ST2 signaling was involved in the development of RSV-associated PH. ST2 signaling inhibition may be a novel therapeutic target for RSV-associated PH.NEW & NOTEWORTHY We report that the pathogenic role of ST2-mediated type 2 immunity and mechanisms contribute to RSV-associated pulmonary hypertension. Inhibiting ST2 signaling may be a novel therapeutic target for this condition.


Assuntos
Bronquiolite Viral/genética , Hipertensão Pulmonar/genética , Proteína 1 Semelhante a Receptor de Interleucina-1/genética , Pulmão/metabolismo , Infecções por Vírus Respiratório Sincicial/genética , Animais , Animais Recém-Nascidos , Arginase/genética , Arginase/metabolismo , Bronquiolite Viral/complicações , Bronquiolite Viral/metabolismo , Moléculas de Adesão Celular/genética , Moléculas de Adesão Celular/metabolismo , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/metabolismo , Interleucina-13/genética , Interleucina-13/metabolismo , Camundongos , Camundongos Knockout , Óxido Nítrico Sintase Tipo I/genética , Óxido Nítrico Sintase Tipo I/metabolismo , Óxido Nítrico Sintase Tipo II/genética , Óxido Nítrico Sintase Tipo II/metabolismo , Óxido Nítrico Sintase Tipo III/genética , Óxido Nítrico Sintase Tipo III/metabolismo , Reinfecção , Infecções por Vírus Respiratório Sincicial/complicações , Infecções por Vírus Respiratório Sincicial/metabolismo , Vírus Sinciciais Respiratórios
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