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2.
Int J Cardiovasc Imaging ; 35(8): 1535-1548, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31175525

RESUMO

Congenital pulmonary artery (PA) anomalies comprise a rare and heterogeneous spectrum of disease, ranging from abnormal origins to complete atresia. They may present in early infancy or more insidiously in adulthood, often in association with congenital heart disease such as tetralogy of Fallot or other syndromes. In recent years, cross-sectional imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), has become widely utilized for the noninvasive assessment of congenital PA diseases, supplementing echocardiography and at times supplanting invasive angiography. In this article, modern CT and MRI techniques for imaging congenital PA disorders are summarized. The key clinical features, cross-sectional imaging findings, and treatment options for the most commonly encountered entities are then reviewed. Emphasis is placed on the ever-growing role of cross-sectional imaging options in facilitating early and accurate diagnosis and tailored treatment.


Assuntos
Angiografia por Tomografia Computadorizada/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Artéria Pulmonar/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Hemodinâmica , Humanos , Angiografia por Ressonância Magnética , Imagem Cinética por Ressonância Magnética , Tomografia Computadorizada Multidetectores , Valor Preditivo dos Testes , Prognóstico , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar , Reprodutibilidade dos Testes
3.
Zhonghua Wei Zhong Bing Ji Jiu Yi Xue ; 31(5): 647-649, 2019 May.
Artigo em Chinês | MEDLINE | ID: mdl-31198157

RESUMO

OBJECTIVE: In acute hypoxia, pulmonary vascular will contract and divert blood to better ventilated area to optimize ventilation/perfusion matching, which is known as hypoxic pulmonary vasoconstriction (HPV). In chronic hypoxia, irreversible pulmonary vascular remodeling can be induced, characterized by pulmonary artery middle smooth muscle cells and the outer fiber cell hyperplasia in luminal stenosis and pulmonary artery hypertension (PAH) eventually. Furthermore, PAH can cause increased ventricular afterload, and right heart failure in severe cases. Pulmonary artery smooth muscle cell (PASMC) elevated Ca2+ concentration is one of the most important factors of its contractions, proliferation and migration. Recent studies on Ca2+ promoting in HPV were summarized in order to provide evidence for clinical prevention of hypoxia and therapeutic PAH.


Assuntos
Cálcio/metabolismo , Hipóxia/metabolismo , Miócitos de Músculo Liso/metabolismo , Artéria Pulmonar/fisiopatologia , Vasoconstrição/fisiologia , Humanos
4.
Life Sci ; 232: 116546, 2019 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-31176777

RESUMO

Pulmonary arterial hypertension (PAH) is reported to contribute to right ventricular failure and death. PAH of variable degrees is often related to congenital heart disease (CHD). Galectin-3 (Gal-3) has been proven to be of great importance in PAH and CHD. Therefore, we investigated the specific mechanism of Gal-3 in CHD-PAH. Patients with CHD-PAH were enrolled to detect the changes of T-cell subsets, cytokine levels, and other related inflammatory cells in the plasma and to assess the Gal-3 levels in the serum. Next, CHD-PAH mouse models were established and treated with restored or depleted Gal-3 to evaluate the systolic pulmonary artery pressure (sPAP) and right ventricular hypertrophy index (RVHI), to determine levels of IL-4, IL-5, IL-13, AKT and p-AKT along with proliferation of pulmonary artery smooth muscle cells (PASMCs). Finally, we explored the effects of adoptive transfer of CD4+T cells on CHD-PAH in mice with Gal-3 knockdown to further investigate the role of Gal-3 in vivo. Initially, Gal-3 was up-regulated in patients with CHD-PAH. Subsequently, it was demonstrated that restored Gal-3 increased sPAP and RVHI, and promoted proliferation of PASMCs by activating the immune response with elevated levels of IL-4, IL-5, IL-13 and p-AKT. Finally, adoptive transfer of CD4+T cells promoted CD4+T cell perivascular infiltration and the progression of CHD-PAH in mice with Gal-3 knockdown. Collectively, the current study suggests a facilitating role of Gal-3 in pulmonary artery remodeling and progression of CHD-PAH via activation of Th2.


Assuntos
Galectina 3/metabolismo , Cardiopatias Congênitas/metabolismo , Hipertensão Pulmonar/metabolismo , Adulto , Idoso , Animais , Modelos Animais de Doenças , Feminino , Galectina 3/sangue , Galectina 3/imunologia , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/imunologia , Cardiopatias Congênitas/fisiopatologia , Humanos , Hipertensão Pulmonar/imunologia , Hipertensão Pulmonar/fisiopatologia , Imunomodulação/fisiologia , Interleucina-13/metabolismo , Interleucina-4/metabolismo , Interleucina-5/metabolismo , Masculino , Camundongos , Camundongos Endogâmicos BALB C , Pessoa de Meia-Idade , Miócitos de Músculo Liso/metabolismo , Proteínas Proto-Oncogênicas c-akt/metabolismo , Artéria Pulmonar/fisiopatologia , Remodelação Vascular/fisiologia
5.
Int J Cardiovasc Imaging ; 35(8): 1465-1472, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31123846

RESUMO

An accurate distinction between isolated post-capillary pulmonary hypertension (Ipc-PH) and combined post- and pre-capillary pulmonary hypertension (Cpc-PH) is integral to therapy and prognosis in heart failure (HF). This study aimed to compare the ability of four previously validated Doppler estimates of pulmonary vascular resistance (PVRDoppler) to distinguish Ipc-PH from Cpc-PH in a well-defined HF population. Consecutive subjects referred for HF assessment underwent standard echocardiography immediately followed by right heart catheterization (RHC). Subjects with atrial fibrillation, acute coronary syndrome, significant valvular disease or poor image quality were excluded. PVRDoppler estimates were correlated with invasive PVR and agreement was studied using Bland-Altman analysis. Receiver operating characteristics analyses were performed to determine the ability of PVRDoppler methods to identify PVR > 3WU. 55 HF subjects (58 ± 16 years, 55% Ipc-PH) were analyzed. PVRDoppler estimates demonstrated weak to modest associations with invasive PVR. The Doppler method proposed by Abbas et al. demonstrated relatively strong discriminatory ability to distinguish Ipc-PH from Cpc-PH (AUC = 0.79; 95% CI 0.63-0.96; p = 0.001). However, Bland-Altman analysis revealed wide limits of agreement (bias = 0; SD = 1.83WU) and greater variability at higher mean PVR. Conclusions: PVRDoppler estimates demonstrate reasonable ability to distinguish Ipc-PH from Cpc-PH but may not be reliable independent PH distinguishers in HF.


Assuntos
Ecocardiografia Doppler de Pulso , Insuficiência Cardíaca/complicações , Hipertensão Pulmonar/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Circulação Pulmonar , Resistência Vascular , Adulto , Idoso , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Valor Preditivo dos Testes , Artéria Pulmonar/fisiopatologia , Reprodutibilidade dos Testes
6.
Int J Cardiovasc Imaging ; 35(8): 1509-1524, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31049753

RESUMO

Multi-energy computed tomography (MECT) refers to acquisition of CT data at multiple energy levels (typically two levels) using different technologies such as dual-source, dual-layer and rapid tube voltage switching. In addition to conventional/routine diagnostic images, MECT provides additional image sets including iodine maps, virtual non-contrast images, and virtual monoenergetic images. These image sets provide tissue/material characterization beyond what is possible with conventional CT. MECT provides invaluable additional information in the evaluation of pulmonary vasculature, primarily by the assessment of pulmonary perfusion. This functional information provided by the MECT is complementary to the morphological information from a conventional CT angiography. In this article, we review the technique and applications of MECT in the evaluation of pulmonary vasculature.


Assuntos
Angiografia por Tomografia Computadorizada/métodos , Hemodinâmica , Hipertensão Pulmonar/diagnóstico por imagem , Tomografia Computadorizada Multidetectores/métodos , Imagem de Perfusão/métodos , Artéria Pulmonar/diagnóstico por imagem , Circulação Pulmonar , Embolia Pulmonar/diagnóstico por imagem , Humanos , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/terapia , Valor Preditivo dos Testes , Prognóstico , Artéria Pulmonar/fisiopatologia , Embolia Pulmonar/fisiopatologia , Embolia Pulmonar/terapia , Interpretação de Imagem Radiográfica Assistida por Computador
7.
Ter Arkh ; 91(1): 24-31, 2019 Mar 10.
Artigo em Inglês | MEDLINE | ID: mdl-31090367

RESUMO

AIM: Assess the prevalence, clinical course, current therapy, and mortality in patients with pulmonary arterial hypertension (PAH) in the National Registry. MATERIALS AND METHODS: In the prospective study we included patients over 18 years of age with diagnosed PAH [idiopathic PAH (IPAH); Drug- and Toxin-Induced Pulmonary Hypertension; inherited PAH; PAH associated with congenital heart disease (PAH-CHD); PAH associated with systemic connective tissue disease (PAH-CTD); PAH associated with HIV infection (PAH-HIV); with portal pulmonary hypertension (portoPAH)]. The observation was carried out in 15 expert centers of Russia from 01.01.2012 to 31.12.2017. RESULTS: Our registry included 470 patients with PAH: IPAH - 41.5%, PAH-CHD - 36%, PAH-CTD - 19.5%, inherited PAH - 0.4%, portoPAH - 1.9%, PAH-HIV - 0.4%, Drug- and Toxin-Induced PAH - 0.4%. The prevalence among women was 84%. The mean age at the time of patient enrollment in the registry for the overall group of PAH was 42.7±15.3 years. The distance in the 6-minute walking test was 361.3±129.3 m. Among all patients with PAH, 65% had functional class (FC) III/IV at the time of diagnosis, among IPAH - 62%. 69.9% received PAH-specific therapy, of which 62.1% - monotherapy, 32.7% - dual combination therapy, and 5.2% triple therapy. Sildenafil is the most commonly prescribed drug in the regimen of monotherapy. 31.6% of patients were treated with bosentan, 6.4% - riociguat, 3.4% - ambrisentan, 2.1% - macitentan and 2.0% iloprost. Survival of patients with PAH was 98.9% at 1 year of follow-up, 94.1% at 3 years and 86.0% at 5 years. CONCLUSION: The registry data indirectly indicates the need to increase efforts aimed at improving the diagnosis of systemic connective tissue diseases in adults, as well as congenital heart defects in children for timely surgical treatment. In recent years, PAH-specific drugs of the new generation have been introduced into clinical practice, but currently in Russia there are no parenteral prostanoids, which are recommended for the most severe patients.


Assuntos
Anti-Hipertensivos/uso terapêutico , Hipertensão Pulmonar/tratamento farmacológico , Artéria Pulmonar/fisiopatologia , Adulto , Idoso , Feminino , Cardiopatias Congênitas/complicações , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/mortalidade , Pessoa de Meia-Idade , Estudos Prospectivos , Sistema de Registros , Federação Russa/epidemiologia , Taxa de Sobrevida
8.
Int J Cardiovasc Imaging ; 35(9): 1637-1649, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31037473

RESUMO

Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of connective tissue disease (CTD). Right atrial (RA) function is essential to maintaining adequate total right heart function in PAH. However, little is known about prognostic utility of RA function in CTD-PAH. RA longitudinal strain (LS) and strain rate (LSR) were evaluated in 53 consecutive patients (51 female, mean age 42 ± 15 years) with CTD-PAH, including systemic lupus erythematosus (SLE) (33.7%), mixed connective tissue disease (MCTD) (32.1%), primary Sjögren's syndrome (pSS) (26.4%), and systemic sclerosis (SSc) (3.8%). At a mean follow-up of 19.3 ± 10.9 months, 20 patients (37.7%) were clinically worse. The group with clinical events had worse clinical conditions and poorer RA function at baseline compared with the group that had no clinical events. RA LS independently reflected World Health Organization functional class (WHO FC) after adjusting for RA area (RAA), tricuspid regurgitation (TR) grade, right ventricular (RV) global longitudinal strain (GLS), and pulmonary vascular resistance (PVR) (P = 0.006). Receiver operator characteristic (ROC) curve analysis indicated that RA LS < 22.9% was predictive of clinical worsening during follow-up (sensitivity = 80%; specificity = 87.9%; area under the curve (AUC) = 0.858), and the Kaplan-Meier curve confirmed that RA LS ≥ 22.9% was associated with more favorable long-term outcomes compared to RA LS < 22.9% (log-rank P < 0.01). On univariate Cox proportional hazards analysis, PVR, RVGLS, RAA, and RA LS were associated with long-term outcome, while RA LS was the only independent predictor in the multivariate analysis. Our findings suggest that RA LS measurements by speckle-tracking echocardiography (STE) can independently reflect the extent of right heart failure and predict clinical outcomes in patients with CTD-PAH. RA LS < 22.9% is associated with a higher risk of clinical worsening.


Assuntos
Pressão Arterial , Função do Átrio Direito , Doenças do Tecido Conjuntivo/complicações , Ecocardiografia Doppler/métodos , Átrios do Coração/diagnóstico por imagem , Cardiopatias/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Interpretação de Imagem Assistida por Computador/métodos , Artéria Pulmonar/fisiopatologia , Adulto , Idoso , Doenças do Tecido Conjuntivo/diagnóstico , Progressão da Doença , Feminino , Átrios do Coração/fisiopatologia , Cardiopatias/etiologia , Cardiopatias/fisiopatologia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Fatores de Tempo , Função Ventricular Direita , Remodelação Ventricular
11.
Eur J Radiol ; 114: 137-145, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31005164

RESUMO

PURPOSE: Cardiac magnetic resonance (CMR) is a substitute technique for noninvasively assessing pulmonary hemodynamics. Some preliminary studies have shown that CMR has the potential to quantify pulmonary vascular resistance (PVR). However, the evaluative value has not been well established. The purpose of the systematic review is to assess the feasibility of CMR in the measurement of PVR in patients with pulmonary hypertension (PH). METHODS: Studies were retrieved from multiple databases. Methodological evaluation of CMR and right heart catheterization (RHC) in estimating PVR were obtained from included studies. The Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) tool was used to assess the quality of studies. The results of comparisons of continuous variables are reported as weighted mean difference (WMD), together with the corresponding 95% confidence intervals (CIs). Summary correlation coefficient (r) values were extracted from each study, and 95% CIs were calculated after Fisher's z transformation. Sensitivity analysis was conducted to investigate potential heterogeneity. RESULTS: A total of 15 studies were included in the systematic review, and 6 of these studies were included in the meta-analysis. The pooled WMD with fixed-effects analysis revealed no statistical significance between PVR-CMR and PVR-RHC in patients with PH (WMD = 0.278 WU; 95% CI: -0.415 to 0.972; p = 0.431). The pooled r value for all studies was 0.85 (95% CI: 0.81, 0.89), and notable heterogeneity was evident. The pooled r value after the exclusion of one heterogeneous article was 0.81 (95% CI: 0.74, 0.87) and was not significantly heterogeneous. CONCLUSIONS: CMR and RHC have good consistency in the testing of PVR in the meta-analysis. The systematic review shows that completely noninvasive evaluation of PVR with CMR in patients with pH is feasible.


Assuntos
Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Espectroscopia de Ressonância Magnética , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Resistência Vascular/fisiologia , Cateterismo Cardíaco , Débito Cardíaco/fisiologia , Estudos de Viabilidade , Humanos , Monitorização Fisiológica
12.
BMJ Case Rep ; 12(4)2019 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-30967450

RESUMO

A 29-year-old female patient presented with chest pain, breathlessness and syncope on the background of constitutional symptoms, oral ulceration and a rash. Multiple investigations were performed, including a CT pulmonary angiogram (CTPA) that was initially felt to show imaging features consistent with a diagnosis of chronic thromboembolic disease (CTED). The patient was referred to a tertiary pulmonary hypertension centre and the possibility of pulmonary vasculitis was raised. Subsequent positron emission tomography (PET)-CT revealed imaging features supporting this diagnosis. The patient was treated with intravenous cyclophosphamide infusions, following which her symptoms improved. A repeat PET-CT 6 months after treatment showed resolution in pulmonary artery and mediastinal uptake, but persistence of pulmonary artery occlusions on a repeat CTPA. A final diagnosis of pulmonary vasculitis secondary to Behçet's disease was made. This case report aims to raise awareness of the imaging features of CTED and its mimics.


Assuntos
Hipertensão Pulmonar/diagnóstico , Artéria Pulmonar/diagnóstico por imagem , Vasculite/diagnóstico , Administração Intravenosa , Adulto , Antirreumáticos/administração & dosagem , Síndrome de Behçet/complicações , Ciclofosfamida/administração & dosagem , Diagnóstico Diferencial , Ecocardiografia , Feminino , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/tratamento farmacológico , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Artéria Pulmonar/fisiopatologia , Tromboembolia/complicações , Tromboembolia/diagnóstico , Vasculite/complicações , Vasculite/tratamento farmacológico
13.
Int J Cardiovasc Imaging ; 35(8): 1473-1481, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31016501

RESUMO

The purpose of this study was to assess in pediatric pulmonary artery (PA) reconstruction candidates the feasibility and added utility of preoperative chest computed tomography angiography (CTA) using dual-energy technique, from which perfused blood volume (PBV)/iodine maps can be generated as a surrogate of pulmonary perfusion. Pediatric PA reconstruction patients were prospectively recruited for a new dose-neutral dual-energy CTA protocol. For each case, the severity of anatomic PA obstruction was graded by two pediatric cardiovascular radiologists in consensus using a modified Qanadli index. PBV maps were qualitatively reviewed and auto-segmented using Siemens syngo.via software. Associations between Qanadli scores and PBV were assessed with Spearman correlation (r) and ROC analysis. Effective radiation doses were estimated from dose-length product and ICRP 103 k-factors, using cubic Hermite spline interpolation. 19 patients were recruited with mean (SD) age of 6.0 (5.1), 11 (57.9%) female, 11 (73.7%) anesthetized. Higher QS correlated with lower PBV, both on a whole lung (r = - 0.54, p < 0.001) and lobar (r = - 0.50, p < 0.001) basis. The lung with lowest absolute PBV was predictive of the lung with highest Qanadli score, with AUC of 0.70 (95% CI 0.47-0.93). Qualitatively, PBV maps were heterogeneous, corresponding to multifocal PA stenoses, with decreased iodine content in areas of most severe obstruction. In conclusion, dual-energy chest CTA is feasible for pediatric PA reconstruction candidates. PBV maps show deficits in regions of more severe anatomic obstruction and may serve as a novel biomarker in this population.


Assuntos
Angiografia por Tomografia Computadorizada/métodos , Tomografia Computadorizada Multidetectores/métodos , Artéria Pulmonar/diagnóstico por imagem , Procedimentos Cirúrgicos Reconstrutivos , Estenose de Artéria Pulmonar/diagnóstico por imagem , Procedimentos Cirúrgicos Vasculares , Adolescente , Fatores Etários , Criança , Pré-Escolar , Tomada de Decisão Clínica , Estudos de Viabilidade , Feminino , Humanos , Lactente , Masculino , Imagem de Perfusão/métodos , Projetos Piloto , Valor Preditivo dos Testes , Estudos Prospectivos , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/cirurgia , Circulação Pulmonar , Interpretação de Imagem Radiográfica Assistida por Computador , Índice de Gravidade de Doença , Estenose de Artéria Pulmonar/fisiopatologia , Estenose de Artéria Pulmonar/cirurgia
14.
Int J Cardiovasc Imaging ; 35(8): 1483-1497, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31030315

RESUMO

Magnetic resonance imaging (MRI) plays an increasingly important role in the non-invasive evaluation of the pulmonary vasculature. MR angiographic (MRA) techniques provide morphological information, while MR perfusion techniques provide functional information of the pulmonary vasculature. Contrast-enhanced MRA can be performed at high spatial resolution using 3D T1-weighted spoiled gradient echo sequence or at high temporal resolution using time-resolved techniques. Non-contrast MRA can be performed using 3D steady state free precession, double inversion fast spin echo, time of flight or phase contrast sequences. MR perfusion can be done using dynamic contrast-enhanced technique or using non-contrast techniques such as arterial spin labelling and time-resolved imaging of lungs during free breathing with Fourier decomposition analysis. MRI is used in the evaluation of acute and chronic pulmonary embolism, pulmonary hypertension and other vascular abnormalities, congenital anomalies and neoplasms. In this article, we review the different MR techniques used in the evaluation of pulmonary vasculature and its clinical applications.


Assuntos
Hemodinâmica , Pneumopatias/diagnóstico por imagem , Angiografia por Ressonância Magnética , Imagem por Ressonância Magnética , Imagem de Perfusão/métodos , Artéria Pulmonar/diagnóstico por imagem , Circulação Pulmonar , Humanos , Interpretação de Imagem Assistida por Computador , Pneumopatias/fisiopatologia , Pneumopatias/terapia , Valor Preditivo dos Testes , Prognóstico , Artéria Pulmonar/fisiopatologia
15.
Nagoya J Med Sci ; 81(1): 19-30, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30962652

RESUMO

Pulmonary hypertension (PH) is a hemodynamic state that is characterized by a resting mean pulmonary artery pressure ≧ 25 mmHg. The common forms of PH are pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension (CTEPH), PH caused by left-heart disease, and PH due to lung disease. Previously regarded as untreatable, the treatment of PAH has dramatically advanced since the introduction of the drug epoprostenol in 1999, with three-year survival rates improving from 30%-40% to over 85%. Drugs available for the specific treatment of PAH include endothelin-receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogs, and prostacyclin-receptor agonists. In the past decade, management and treatment of CTEPH have also improved. While pulmonary endarterectomy used to be the only option for the treatment of CTEPH, newer treatments include a soluble guanylate cyclase stimulator, which has proven to be an efficacious targeted therapy. Other cases benefit from balloon pulmonary angioplasty.


Assuntos
Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/fisiopatologia , Animais , Endarterectomia , Antagonistas dos Receptores de Endotelina/uso terapêutico , Humanos , Hipertensão Pulmonar/cirurgia , Artéria Pulmonar/efeitos dos fármacos , Artéria Pulmonar/patologia , Artéria Pulmonar/fisiopatologia , Embolia Pulmonar
16.
Ann Thorac Surg ; 108(1): 154-159, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30928554

RESUMO

BACKGROUND: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) is a rare form of congenital heart disease characterized by the entirety of pulmonary blood flow originating from systemic vessels. This study measured the residual collateral flow after harvesting of the MAPCAs for surgical repair. METHODS: The study enrolled 32 patients with pulmonary atresia with ventricular septal defect and MAPCAs who were undergoing their first surgical procedure. The median age was 6.8 months, and median weight was 5.7 kg. The patients had a mean of 4.2 ± 0.7 MAPCAs. The cardiopulmonary bypass circuit was modified to contain a diversion loop in the left ventricular vent system to accurately measure residual collateral flow. During the period of aortic cross-clamp (for ventricular septal defect repair), the diversion loop was opened for 1-minute intervals, and the residual collateral flow collected. The systemic perfusion temperature was 25° and flow rate was 100 mL · kg-1 · min-1. RESULTS: The mean residual collateral flow was 5.5 mL · kg-1 · min-1 (range, 0.8 to 15.2 mL · kg-1 · min-1). The corresponding calculated pulmonary blood flow-to-systemic blood flow ratio values ranged from 1.01 to 1.36. There was a significant correlation between residual collateral flow and preoperative saturation (p < 0.05). CONCLUSIONS: The data demonstrate a wide range of residual collateral flow values after harvesting of the MAPCAs. The amount of residual collateral flow was correlated with preoperative saturation. These results suggest that some patients at the higher end of this spectrum may require adjustments in pump flow to assure adequate systemic perfusion.


Assuntos
Circulação Colateral/fisiologia , Defeitos dos Septos Cardíacos/fisiopatologia , Atresia Pulmonar/fisiopatologia , Aorta/anormalidades , Aorta/diagnóstico por imagem , Aortografia , Defeitos dos Septos Cardíacos/cirurgia , Humanos , Lactente , Oxigênio/sangue , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/fisiopatologia
17.
Int J Cardiovasc Imaging ; 35(9): 1581-1586, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30968263

RESUMO

A growing body of evidence has demonstrated that pulmonary arterial capacitance (PAC) is the strongest hemodynamic predictor of clinical outcomes across a wide spectrum of cardiovascular disease, including pulmonary hypertension and heart failure. We hypothesized that a ratio of right ventricular stroke volume (RVOT VTI) to the associated peak arterial systolic pressure (PASP) could function as a reliable non-invasive surrogate for PAC. We performed a prospective study of patients undergoing simultaneous transthoracic echocardiography and right heart catheterization (RHC) for various clinical indications. Measurements of the RVOT VTI/PASP ratio from echocardiographic measurements were compared against PAC calculated from RHC measurements. Correlation coefficients and Bland-Altman analysis compared the RVOT VTI/PASP ratio with PAC. Forty-five subjects were enrolled, 38% were female and mean age was 54 years (SD 13 years). The reason for referral to RHC was most commonly post-heart transplant surveillance (40%), followed by heart failure (22%), and pulmonary hypertension (18%). Pre-capillary pulmonary hypertension was present in 18%, isolated post-capillary pulmonary hypertension was present in 13%, and combined pre-and post-capillary pulmonary hypertension was present in 29%. The RVOT VTI/PASP ratio was obtainable in the majority of patients (78%), and Pearson's correlation demonstrated moderately-strong association between PAC and the RVOT VTI/PASP ratio, r = 0.75 (P < 0.001). Bland-Altman analysis demonstrated good agreement between measurements without suggestion of systematic bias and a mean difference in standardized units of - 0.133. In a diverse population of patients and hemodynamic profiles, we validated that the ratio of RVOT VTI/PASP to be a reliably-obtained non-invasive marker associated with PAC.


Assuntos
Pressão Arterial , Ecocardiografia Doppler , Hipertensão Pulmonar/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Circulação Pulmonar , Volume Sistólico , Capacitância Vascular , Função Ventricular Direita , Adulto , Idoso , Cateterismo Cardíaco , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Artéria Pulmonar/fisiopatologia
18.
Int J Pediatr Otorhinolaryngol ; 120: 210-214, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30897517

RESUMO

BACKGROUND: Obstructive sleep apnea syndrome in childhood has aroused great interest due to its cardiovascular repercussions and its adverse effects on the quality of life of the affected individuals. However, fundamental aspects of the syndrome remain unknown. OBJECTIVE: Herein we prospectively assessed pulmonary artery systolic pressure (PASP) and nasal flow in children with obstructive oral breathing with an indication for adenoidectomy and/or tonsillectomy and their relationship to the obstructive apnea and hypopnea index (OAHI). METHODS: Twenty-one children were evaluated at the time of the surgical indication (T0) and 18 months later (T1). Polysomnography, and rhinomanometry data were collected when we evaluated PASP. RESULTS: Among the 21 children, 13 (61.9%) presented an altered OAHI at T0. Fourteen children (66.7%) underwent surgery. Of these, nine (64.3%) had an altered OAHI at T0 and seven (50.0%) at T1. Of the seven non-operated children, four (57.1%) had an altered OAHI at T0 and two (33.3%) at T1. Mean nasal flow increased in both groups independently of surgery (p- ≤ 0.001). PASP exhibited a significant reduction between T0 and T1 in the operated group (p ≤ 0.001). OAHI of the operated group did not show a significant decrease over time (p = 0.074). In the non-operated children, the average nasal flow increased (p < 0.001), the PASP values did not reduce (p = 0.99), and the OAHI increased and then decreased over time (p = 0.025). CONCLUSION: PASP decreased significantly and OAHI did not normalize in the operated group. Mean nasal airflow increased in the operated and non-operated groups.


Assuntos
Pressão Sanguínea , Artéria Pulmonar/fisiopatologia , Respiração , Apneia Obstrutiva do Sono/fisiopatologia , Apneia Obstrutiva do Sono/cirurgia , Adenoidectomia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Nariz/fisiopatologia , Polissonografia , Estudos Prospectivos , Rinomanometria , Índice de Gravidade de Doença , Sístole , Fatores de Tempo , Tonsilectomia
19.
Pediatr Cardiol ; 40(4): 841-847, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30830280

RESUMO

This study aimed to clarify the characteristics of pulmonary arterial resistance (Rp)-compliance (Cp) coupling in individuals with Down syndrome (DS), who have increased risks of pulmonary arterial hypertension (PAH). We performed cardiac catheterization before and after corrective surgery in 85 DS infants and 85 controls with congenital heart disease and PAH. We retrospectively collected hemodynamic data and compared Rp and Cp between the groups. Age at surgery was 3.5 (2.6-4.6) months. The first and second catheterizations were performed 1 month before and after corrective surgery in both groups. Preoperative Cp in DS patients was significantly lower than that in controls [2.27 (1.62-3.0) vs. 2.50 (1.86-3.31) mL/mmHg/m2, p = 0.039], although there was no significant difference in mean pulmonary arterial pressure and Rp between the groups. Analysis of covariance revealed that the slopes of the preoperative regression lines for the logarithmic transformations of Rp and Cp were identical in DS patients and controls (p = 0.299). However, the postoperative regression line was shifted downward in DS patients after corrective surgery. Postoperative home oxygen therapy (HOT) was performed in 39 patients (36 DS patients) and multivariate logistic regression analysis revealed that postoperative HOT was significantly related to low preoperative Cp (p = 0.039) and DS (p = 0.0001). Individuals with DS have the unique pulmonary vasculature characterized with low Cp that is related to postoperative HOT.


Assuntos
Síndrome de Down/complicações , Cardiopatias Congênitas/complicações , Hipertensão Pulmonar/fisiopatologia , Artéria Pulmonar/fisiopatologia , Cateterismo Cardíaco/métodos , Estudos de Casos e Controles , Feminino , Cardiopatias Congênitas/cirurgia , Hemodinâmica/fisiologia , Humanos , Lactente , Masculino , Estudos Retrospectivos
20.
Int J Mol Sci ; 20(6)2019 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-30909527

RESUMO

Pulmonary arterial hypertension (PAH) is characterized by pulmonary arterial proliferation and remodeling, resulting in a specific increase in right ventricle systolic pressure (RVSP) and, ultimately right ventricular failure. Recent studies have demonstrated that caffeic acid phenethyl ester (CAPE) exerts a protective role in NF-κB-mediated inflammatory diseases. However, the effect of CAPE on PAH remains to be elucidated. In this study, monocrotaline (MCT) was used to establish PAH in rats. Two weeks after the induction of PAH by MCT, CAPE was administrated by intraperitoneal injection once a day for two weeks. Pulmonary hemodynamic measurements and pulmonary artery morphological assessments were examined. Our results showed that administration of CAPE significantly suppressed MCT-induced vascular remodeling by decreasing the HIF-1α expression and PDGF-BB production, and improved in vivo RV systolic performance in rats. Furthermore, CAPE inhibits hypoxia- and PDGF-BB-induced HIF-1α expression by decreasing the activation of the AKT/ERK pathway, which results in the inhibition of human pulmonary artery smooth muscle cells (hPASMCs) proliferation and prevention of cells resistant to apoptosis. Overall, our data suggest that HIF-1α is regarded as an alternative target for CAPE in addition to NF-κB, and may represent a promising therapeutic agent for the treatment of PAH diseases.


Assuntos
Ácidos Cafeicos/farmacologia , MAP Quinases Reguladas por Sinal Extracelular/metabolismo , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/metabolismo , Subunidade alfa do Fator 1 Induzível por Hipóxia/metabolismo , Álcool Feniletílico/análogos & derivados , Fator de Crescimento Derivado de Plaquetas/metabolismo , Proteínas Proto-Oncogênicas c-akt/metabolismo , Animais , Apoptose/efeitos dos fármacos , Linhagem Celular , Proliferação de Células/efeitos dos fármacos , Modelos Animais de Doenças , Expressão Gênica , Hemodinâmica/efeitos dos fármacos , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/tratamento farmacológico , Hipertrofia Ventricular Direita/tratamento farmacológico , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/metabolismo , Hipertrofia Ventricular Direita/fisiopatologia , Imuno-Histoquímica , Álcool Feniletílico/farmacologia , Fator de Crescimento Derivado de Plaquetas/genética , Artéria Pulmonar/efeitos dos fármacos , Artéria Pulmonar/metabolismo , Artéria Pulmonar/fisiopatologia , Ratos , Transdução de Sinais/efeitos dos fármacos , Remodelação Vascular/efeitos dos fármacos
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