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1.
Artigo em Inglês | MEDLINE | ID: mdl-38759116

RESUMO

OBJECTIVES: Measures of right heart size and function are prognostic in systemic sclerosis-associated pulmonary hypertension (SSc-PH), but the importance of myocardial tissue characterisation remains unclear. We aimed to investigate the predictive potential and interaction of cardiovascular magnetic resonance (CMR) myocardial tissue characterisation and right heart size and function in SSc-PH. METHODS: A retrospective, single-centre, observational study of 148 SSc-PH patients confirmed by right heart catheterization who underwent clinically-indicated CMR including native myocardial T1 and T2 mapping from 2016 to 2023 was performed. RESULTS: Sixty-six (45%) patients died during follow-up (median 3.5 years, range 0.1-7.3). Patients who died were older (65 vs 60 years, p= 0.035) with more dilated (RVEDVi and RVESVi, p< 0.001), hypertrophied (RVMi, p= 0.013) and impaired (RVEF, p< 0.001) right ventricles, more dilated right atria (RAi, p= 0.043) and higher native myocardial T1 (p< 0.001).After adjustment for age, RVESVi (p = 0.0023) and native T1 (p = 0.0024) were independent predictors of all-cause mortality. Both RVESVi and native T1 remained independently predictive after adjusting for age and PH subtype (RVESVi p < 0.001, T1 p = 0.0056). Optimal prognostic thresholds for RVESVi and native T1 were ≤38 mL/m2 and ≤1119 ms, respectively (p < 0.001). Patients with RVESVi ≤ 38 mL/m2 and native T1 ≤ 1119 ms had significantly better outcomes than all other combinations (p < 0.001). Furthermore, patients with RVESVi > 38mL/m2 and native T1 ≤ 1119 ms had significantly better survival than patients with RVESVi > 38mL/m2 and native T1 > 1119ms (p = 0.017). CONCLUSION: We identified prognostically relevant CMR metrics and thresholds for patients with SSc-PH. Assessing myocardial tissue characterisation alongside RV function confers added value in SSc-PH and may represent an additional treatment target.

2.
Eur Respir J ; 57(2)2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-32631835

RESUMO

Health-related quality of life (HRQoL) scores assess symptom burden in pulmonary arterial hypertension (PAH) but data regarding their role in prognostication and risk stratification are limited. We assessed these relationships using the emPHasis-10 HRQoL measure.1745 patients with idiopathic PAH (IPAH), drug-induced PAH (DPAH), heritable PAH (HPAH) (collectively "(I/D/H)PAH"), or connective tissue disease-associated PAH (CTD-PAH), who had completed emPHasis-10 questionnaires at one of six UK referral centres between 2014 and 2017, were identified. Correlations with exercise capacity and World Health Organization (WHO) functional class were assessed, and exploratory risk stratification thresholds were tested.Moderate correlations were seen between emPHasis-10 scores and 6-min walk distance (r=-0.546), incremental shuttle walk distance (r=-0.504) and WHO functional class (r=0.497) (all p<0.0001). Distribution of emPHasis-10 score differed significantly between each WHO functional class (all p<0.0001). On multivariate analysis, emPHasis-10 score, but not WHO functional class, was an independent predictor of mortality. In a risk stratification approach, scores of 0-16, 17-33 and 34-50 identified incident patients with 1-year mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO functional class III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis-10 scores had improved exercise capacity (p<0.0001) and patients who transitioned between risk groups demonstrated similar survival to patients originally in those risk groups.The emPHasis-10 score is an independent prognostic marker in patients with (I/D/H)PAH or CTD-PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity.


Assuntos
Doenças do Tecido Conjuntivo , Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Doenças do Tecido Conjuntivo/complicações , Humanos , Qualidade de Vida , Reino Unido
3.
Br J Anaesth ; 126(4): 774-790, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33612249

RESUMO

BACKGROUND: The risk of complications, including death, is substantially increased in patients with pulmonary hypertension (PH) undergoing anaesthesia for surgical procedures, especially in those with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). Sedation also poses a risk to patients with PH. Physiological changes including tachycardia, hypotension, fluid shifts, and an increase in pulmonary vascular resistance (PH crisis) can precipitate acute right ventricular decompensation and death. METHODS: A systematic literature review was performed of studies in patients with PH undergoing non-cardiac and non-obstetric surgery. The management of patients with PH requiring sedation for endoscopy was also reviewed. Using a framework of relevant clinical questions, we review the available evidence guiding operative risk, risk assessment, preoperative optimisation, and perioperative management, and identifying areas for future research. RESULTS: Reported 30 day mortality after non-cardiac and non-obstetric surgery ranges between 2% and 18% in patients with PH undergoing elective procedures, and increases to 15-50% for emergency surgery, with complications and death usually relating to acute right ventricular failure. Risk factors for mortality include procedure-specific and patient-related factors, especially markers of PH severity (e.g. pulmonary haemodynamics, poor exercise performance, and right ventricular dysfunction). Most studies highlight the importance of individualised preoperative risk assessment and optimisation and advanced perioperative planning. CONCLUSIONS: With an increasing number of patients requiring surgery in specialist and non-specialist PH centres, a systematic, evidence-based, multidisciplinary approach is required to minimise complications. Adequate risk stratification and a tailored-individualised perioperative plan is paramount.


Assuntos
Consenso , Prova Pericial/normas , Hipertensão Pulmonar/cirurgia , Assistência Perioperatória/normas , Complicações Pós-Operatórias/prevenção & controle , Prova Pericial/métodos , Humanos , Hipertensão Pulmonar/diagnóstico , Assistência Perioperatória/métodos , Complicações Pós-Operatórias/diagnóstico
4.
Heart Lung Circ ; 27(11): e101-e104, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29678310

RESUMO

Pulmonary hypertension is a serious condition with multiple underlying aetiologies which require different treatment strategies. We present a case of severe idiopathic pulmonary arterial hypertension in a 20-year-old patient with ongoing breathlessness. She was initially diagnosed with asthma and panic attacks in community care. As the symptoms became progressively worse, she was referred for pulmonary hypertension clinic assessment. Ventilation/perfusion single-photon emission computed tomography (V/Q SPECT) showed grossly abnormal perfusion defects which were mismatched to the ventilation scan, suggestive of chronic thromboembolic disease. However, corroborating computed tomographic (CT) pulmonary angiogram and invasive pulmonary angiography showed no thromboembolic disease. Histological examination of the pulmonary arteries post-mortem showed changes consistent with idiopathic pulmonary arterial hypertension. This case highlighted the clinical challenges in interpreting the investigation results and phenotyping pulmonary hypertension. V/Q SPECT might have a role in visualising the extent of vasculopathies in pulmonary arterial hypertension.


Assuntos
Hipertensão Pulmonar/diagnóstico , Artéria Pulmonar/diagnóstico por imagem , Pressão Propulsora Pulmonar/fisiologia , Tomografia Computadorizada de Emissão de Fóton Único/métodos , Cateterismo Cardíaco , Angiografia por Tomografia Computadorizada , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Pulmão/diagnóstico por imagem , Embolia Pulmonar , Relação Ventilação-Perfusão , Adulto Jovem
5.
Thorax ; 72(11): 1035-1045, 2017 11.
Artigo em Inglês | MEDLINE | ID: mdl-28904006

RESUMO

Treatment of acute emergencies in patients with pulmonary arterial hypertension (PAH) can be challenging. In the UK and Ireland, management of adult patients with PAH is centred in eight nationally designated pulmonary hypertension (PH) centres. However, many patients live far from these centres and physicians in local hospitals are often required to manage PAH emergencies. A committee of physicians from nationally designated PH centres identified the 'most common' emergency clinical scenarios encountered in patients with PAH. Thereafter, a review of the literature was performed centred on these specified topics and a management approach was developed based on best available evidence and expert consensus. Management protocols were developed on the following PAH emergencies: chest pain (including myocardial ischaemia), right ventricular failure, arrhythmias, sepsis, haemoptysis ('CRASH'), as well as considerations relevant to surgery, anaesthesia and pregnancy. Emergencies are not uncommon in PAH. While expertise in PAH management is essential, all physicians involved in acute care should be aware of the principles of acute management of PAH emergencies. A multidisciplinary approach is necessary, with physicians from tertiary PH centres supporting care locally and planning safe transfer of patients to PH centres when appropriate.


Assuntos
Cuidados Críticos , Hipertensão Pulmonar/terapia , Papel do Médico , Arritmias Cardíacas/etiologia , Bacteriemia/microbiologia , Dor no Peito/etiologia , Ensaios Clínicos como Assunto , Medicina Baseada em Evidências , Hemoptise/etiologia , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/mortalidade , Irlanda , Guias de Prática Clínica como Assunto , Prognóstico , Fatores de Risco , Resultado do Tratamento , Reino Unido , Disfunção Ventricular Direita/etiologia
6.
Rheumatology (Oxford) ; 56(12): 2197-2203, 2017 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-29029203

RESUMO

Objectives: SSc-pulmonary arterial hypertension (SSc-PAH) is associated with worse response to therapy and survival when compared with idiopathic PAH. It is suggested that the vasculopathy in SSc may involve postcapillary pulmonary venules resulting in pulmonary veno-occlusive disease (PVOD). This may underlie the lower gas transfer and worse outcome on therapy. We sought to test whether CT signs of PVOD (CTS-PVOD) were frequent in SSc-PAH and whether they were associated with pulmonary oedema on therapy and worse survival. Methods: CT thorax of 66 SSc patients with precapillary pulmonary hypertension (PH) were blindly scored by two radiologists for CTS-PVOD (⩽1 or ⩾ 2). Case note and radiograph review determined the presence of pulmonary oedema on therapy. Results: Fifty-nine patients (89%) had ⩽1 CTS-PVOD and only 7 (11%) had ⩾2 CTS-PVOD. Pulmonary oedema on therapy was relatively common in those with ⩾2 CTS-PVOD. On univariate analysis ⩾2 CTS-PVOD were associated with a trend towards worse survival. Conclusion: CTS-PVOD were less frequent in this SSc-PAH cohort than in previous reports but the presence of at least two of these signs is associated with pulmonary oedema on therapy and a trend towards worse survival on univariate analysis.


Assuntos
Hipertensão Pulmonar/diagnóstico por imagem , Pneumopatia Veno-Oclusiva/diagnóstico por imagem , Escleroderma Sistêmico/complicações , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Hipertensão Pulmonar/complicações , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Prognóstico , Edema Pulmonar/diagnóstico por imagem , Edema Pulmonar/etiologia , Pneumopatia Veno-Oclusiva/etiologia , Tomografia Computadorizada por Raios X/métodos
7.
Clin Exp Rheumatol ; 35(1): 80-84, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-27494156

RESUMO

OBJECTIVES: To review our clinical experience of this rare condition and describe the clinical features and response to therapy in a cohort of patients with erythromelalgia (EM), a rare condition, characterised by paroxysmal hyperthermia of the extremities with erythema, pain and intense burning. METHODS: A review was made of the electronic and paper medical records of patients with the diagnosis of EM, with a telephone interview to verify and complete clinical information relating treatment and outcome. RESULTS: 46 patients (41 females) were included in this study. Mean age was 57 years and mean duration of symptoms was 16 years. Raynaud's phenomenon was present in 36 patients (80%) and 4 patients (9%) had systemic sclerosis. Smoking (current or previous) was identified as a possible risk factor in 26 cases and exposure to chronic vibration in 3 cases. Overall, the effect on quality of life was mild in 15% of cases, moderate in 30% and severe in 48%. The most common symptoms were burning (96%), heat (93%), pain (87%), and redness (83%). Symptoms affected the lower limbs in 98% of cases, upper limbs in 76%, face in 20% and trunk in 11%. Triggers included heat (85%), exercise (78%) and time of day (76%). Various medications were tried, showing poor effect in most cases. Intravenous iloprost was given to 27 patients, with benefit in 17 patients (63%). CONCLUSIONS: Erythromelalgia is a rare chronic debilitating condition. Exercise, heat and night time are common triggers. Current medical therapies are seldom effective and further research is sorely needed.


Assuntos
Eritromelalgia/diagnóstico , Eritromelalgia/tratamento farmacológico , Iloprosta/uso terapêutico , Inibidores da Agregação Plaquetária/uso terapêutico , Adulto , Eritromelalgia/complicações , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , Doença de Raynaud/complicações , Fatores de Risco , Fumar , Avaliação de Sintomas , Resultado do Tratamento
8.
Echocardiography ; 34(6): 817-824, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28612517

RESUMO

BACKGROUND: Data are scarce about short-term right ventricular changes in pulmonary hypertension. Two-dimensional knowledge-based reconstruction of the right ventricle with 2D echocardiography (2DKBR) has been shown to be a valid alternative to Cardiac MRI. PATIENTS AND METHODS: In this longitudinal study 25 pulmonary hypertension patients underwent 2DKBR of the right ventricle, assessment of NT-proBNP levels and functional class at baseline and after a mean follow-up of 6.1 months. Patients were followed up clinically for a further mean of 8.2 months. The majority of patients had connective tissue disease (CTD) associated pulmonary arterial hypertension (n=15) or chronic thromboembolic pulmonary hypertension (CTEPH; n=6). A total of 15 patients underwent an intervention, either new targeted therapy, escalation of targeted therapy or pulmonary endarterectomy. A total of 10 clinically stable patients were routinely followed up without any change in therapy. RESULTS: There were significant improvements in the right ventricular end-diastolic volume index (111±29 mL/m² vs 100±36 mL/m²; P=.038), end-systolic volume index (72±23 mL/m² vs 61±25 mL/m²; P=.001), and ejection fraction (35±10% vs 40±9%; P=.030). Changes in NT-proBNP levels correlated strongest with changes in end-systolic volume index (r=-.77; P=<.0001). Four patients experienced clinical worsening during extended follow-up, dilatation of the right ventricle was associated with clinical worsening. CONCLUSION: In a CTD and CTEPH dominated patient population significant reverse remodeling and improvement of ejection fraction occurred despite a short follow-up and was paralleled by significant changes in NT-proBNP levels. Further right ventricular dilatation was associated with worse clinical outcome. 2DKBR is a feasible substitute for Cardiac MRI to follow-up right ventricular indices in pulmonary hypertension.


Assuntos
Ecocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/terapia , Processamento de Imagem Assistida por Computador/métodos , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
9.
Circ Res ; 114(4): 677-88, 2014 Feb 14.
Artigo em Inglês | MEDLINE | ID: mdl-24334027

RESUMO

RATIONALE: Evidence is increasing of a link between interferon (IFN) and pulmonary arterial hypertension (PAH). Conditions with chronically elevated endogenous IFNs such as systemic sclerosis are strongly associated with PAH. Furthermore, therapeutic use of type I IFN is associated with PAH. This was recognized at the 2013 World Symposium on Pulmonary Hypertension where the urgent need for research into this was highlighted. OBJECTIVE: To explore the role of type I IFN in PAH. METHODS AND RESULTS: Cells were cultured using standard approaches. Cytokines were measured by ELISA. Gene and protein expression were measured using reverse transcriptase polymerase chain reaction, Western blotting, and immunohistochemistry. The role of type I IFN in PAH in vivo was determined using type I IFN receptor knockout (IFNAR1(-/-)) mice. Human lung cells responded to types I and II but not III IFN correlating with relevant receptor expression. Type I, II, and III IFN levels were elevated in serum of patients with systemic sclerosis associated PAH. Serum interferon γ inducible protein 10 (IP10; CXCL10) and endothelin 1 were raised and strongly correlated together. IP10 correlated positively with pulmonary hemodynamics and serum brain natriuretic peptide and negatively with 6-minute walk test and cardiac index. Endothelial cells grown out of the blood of PAH patients were more sensitive to the effects of type I IFN than cells from healthy donors. PAH lung demonstrated increased IFNAR1 protein levels. IFNAR1(-/-) mice were protected from the effects of hypoxia on the right heart, vascular remodeling, and raised serum endothelin 1 levels. CONCLUSIONS: These data indicate that type I IFN, via an action of IFNAR1, mediates PAH.


Assuntos
Hipertensão Pulmonar/imunologia , Interferon-alfa/imunologia , Interferon beta/imunologia , Receptor de Interferon alfa e beta/imunologia , Escleroderma Sistêmico/imunologia , Animais , Células Cultivadas , Quimiocina CXCL10/imunologia , Quimiocina CXCL10/metabolismo , Modelos Animais de Doenças , Células Endoteliais/citologia , Células Endoteliais/imunologia , Endotelina-1/imunologia , Endotelina-1/metabolismo , Hipertensão Pulmonar Primária Familiar , Humanos , Hipertensão Pulmonar/metabolismo , Interferon-alfa/metabolismo , Interferon-alfa/farmacologia , Interferon beta/metabolismo , Interferon beta/farmacologia , Interferon gama/imunologia , Interferon gama/farmacologia , Pulmão/citologia , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Técnicas de Cultura de Órgãos , Receptor de Interferon alfa e beta/genética , Receptor de Interferon alfa e beta/metabolismo , Escleroderma Sistêmico/metabolismo , Transdução de Sinais/imunologia
10.
Arthritis Rheum ; 65(4): 1074-84, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23280155

RESUMO

OBJECTIVE: To determine whether patients with systemic sclerosis (SSc) and borderline mean pulmonary artery pressure (PAP) at cardiac catheterization are more likely to develop pulmonary hypertension (PH) than those in whom pulmonary pressure is normal. METHODS: Patients with SSc in whom PH and significant interstitial lung disease had been excluded at baseline were enrolled in our prospective cohort. Analysis of followup data identified patients who met prespecified criteria prompting repeat catheterization to reassess for possible PH. Using Kaplan-Meier, receiver operating characteristic, and Cox regression methods, we studied the development of PH and death. RESULTS: Of 228 patients in this study, 86 had borderline mean PAP (21-24 mm Hg) at baseline. Following prespecified criteria, 76 patients underwent repeat catheterization, and 29 of these developed PH. Two cases were related to disease of the left side of the heart. The average mean PAP increased from baseline (20.2 mm Hg) to followup (24.3 mm Hg) (P<0.05 by Student's t-test). Patients with borderline mean PAP were more likely to develop PH than patients with mean PAP≤20 mm Hg (P<0.001 by log rank test, hazard ratio [HR] 3.7). A transpulmonary gradient (TPG)≥11 mm Hg at baseline also predicted PH (P<0.001 by log rank test, HR 7.9). Incident development of pulmonary arterial hypertension (PAH) was not benign, with a mortality of 18% within 3 years. CONCLUSION: Our findings indicate that borderline mean PAP and an elevated TPG in patients with SSc predict progression to PH. These patients should be monitored closely for the development of PH. Our findings indicate that catheterization data are useful in patients considered at risk of PAH.


Assuntos
Hipertensão Pulmonar/fisiopatologia , Pulmão/irrigação sanguínea , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar , Pressão Propulsora Pulmonar , Escleroderma Sistêmico/fisiopatologia , Idoso , Cateterismo Cardíaco , Estudos de Coortes , Progressão da Doença , Hipertensão Pulmonar Primária Familiar , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/mortalidade , Estimativa de Kaplan-Meier , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Prospectivos , Curva ROC , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/mortalidade
11.
Clin Med (Lond) ; 23(5): 449-454, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37775164

RESUMO

Pulmonary hypertension (PH) is common, with an estimated prevalence of approximately 1% that increases with age. Prompt and accurate diagnosis is key to institute timely and appropriate therapy to improve symptoms and prognosis. The international guidelines for the diagnosis and management of PH have recently been updated, with a lowering of the haemodynamic threshold for diagnosis to a mean pulmonary artery pressure >20 mmHg. New diagnostic algorithms and revised indications for screening in at-risk groups have been developed to facilitate early referral to specialist PH centres. This includes fast-track referral pathways for patients who are either clinically high-risk or are at-risk for pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). This review summarises key changes in the PH guidelines for general physicians who are, most often, the first healthcare professionals to encounter these patients and consequently have a key role as referrers into specialist PH services.


Assuntos
Hipertensão Pulmonar , Médicos , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Prognóstico , Doença Crônica
12.
Arthritis Rheumatol ; 75(3): 449-458, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36122180

RESUMO

OBJECTIVE: Pulmonary hypertension (PH) is a serious complication of systemic sclerosis (SSc). In this study, we explored the prediction of short-term risk for PH using serial pulmonary function tests (PFTs) and other disease features. METHODS: SSc patients in whom disease onset occurred ≥10 years prior to data retrieval and for whom autoantibody specificity and PFT data were available were included in this study. Mixed-effects modeling was used to describe changes in PFTs over time. Landmarking was utilized to include serial assessments and stratified Cox proportional hazards regression analysis with landmarks as strata was used to develop the PH prediction models. RESULTS: We analyzed data from 1,247 SSc patients, 16.3% of whom were male and 35.8% of whom had diffuse cutaneous SSc. Anticentromere, antitopoisomerase, and anti-RNA polymerase antibodies were observed in 29.8%, 22.0%, and 11.4% of patients, respectively, and PH developed in 13.6% of patients. Over time, diffusing capacity for carbon monoxide (DLco) and carbon monoxide transfer coefficient (Kco) declined in all SSc patients (up to 1.5% per year) but demonstrated much greater annual decline (up to 4.5% and 4.8%, respectively) in the 5-7 years preceding PH diagnosis. Comparisons between multivariable models including either DLco, Kco, or forced vital capacity (FVC)/DLco ratio, demonstrated that both absolute values and change over the preceding year in those measurements were strongly associated with the risk of PH (hazard ratio [HR] 0.93 and 0.76 for Kco and its change; HR 0.90 and 0.96 for DLco and its change; and HR 1.08 and 2.01 for FVC/DLco ratio and its change; P < 0.001 for all). The Kco-based model had the greatest discriminating ability (Harrell's C-statistic 0.903). CONCLUSION: Our findings strongly support the importance of PFT trends over time in identifying patients at risk of developing PH.


Assuntos
Hipertensão Pulmonar , Escleroderma Sistêmico , Humanos , Masculino , Feminino , Hipertensão Pulmonar/complicações , Pulmão , Monóxido de Carbono , Capacidade Vital
13.
Eur Heart J Cardiovasc Imaging ; 24(4): 463-471, 2023 03 21.
Artigo em Inglês | MEDLINE | ID: mdl-35775814

RESUMO

AIMS: Cardiovascular involvement in systemic sclerosis (SSc) is heterogeneous and ill-defined. This study aimed to: (i) discover cardiac phenotypes in SSc by cardiovascular magnetic resonance (CMR); (ii) provide a CMR-based algorithm for phenotypic classification; and (iii) examine for associations between phenotypes and mortality. METHODS AND RESULTS: A retrospective, single-centre, observational study of 260 SSc patients who underwent clinically indicated CMR including native myocardial T1 and T2 mapping from 2016 to 2019 was performed. Agglomerative hierarchical clustering using only CMR variables revealed five clusters of SSc patients with shared CMR characteristics: dilated right hearts with right ventricular failure (RVF); biventricular failure dilatation and dysfunction (BVF); and normal function with average cavity (NF-AC), normal function with small cavity (NF-SC), and normal function with large cavity (NF-LC) sizes. Phenotypes did not co-segregate with clinical or antibody classifications. A CMR-based decision tree for phenotype classification was created. Sixty-three (24%) patients died during a median follow-up period of 3.4 years. After adjustment for age and presence of pulmonary hypertension (PH), independent CMR predictors of all-cause mortality were native T1 (P < 0.001) and right ventricular ejection fraction (RVEF) (P = 0.0032). NF-SC and NF-AC groups had more favourable prognoses (P≤0.036) than the other three groups which had no differences in prognoses between them (P > 0.14). Hazard ratios (HR) were statistically significant for RVF (HR = 8.9, P < 0.001), BVF (HR = 5.2, P = 0.006), and NF-LC (HR = 4.9, P = 0.002) groups. The NF-LC group remained significantly predictive of mortality after adjusting for RVEF, native T1, and PH diagnosis (P = 0.0046). CONCLUSION: We identified five CMR-defined cardiac SSc phenotypes that did not co-segregate with clinical data and had distinct outcomes, offering opportunities for a more precision-medicine based management approach.


Assuntos
Insuficiência Cardíaca , Hipertensão Pulmonar , Escleroderma Sistêmico , Humanos , Volume Sistólico , Estudos Retrospectivos , Imagem Cinética por Ressonância Magnética/métodos , Função Ventricular Direita , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico por imagem , Prognóstico , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Espectroscopia de Ressonância Magnética , Valor Preditivo dos Testes
14.
Clin Exp Rheumatol ; 30(2 Suppl 71): S81-5, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22691215

RESUMO

OBJECTIVES: Cardiac involvement is recognised as a poor prognostic factor among systemic sclerosis (SSc) patients, contributing significantly to mortality. We assessed the role of N-TproBNP in SSc-related cardiac involvement in a retrospective cohort of patients. METHODS: Serum N-TproBNP levels were measured in 21 SSc patients with clinically significant cardiac involvement and in a control group of 42 SSc patients without any evidence of heart involvement. All patients had normal pulmonary artery systolic pressure and none had serum creatinine above 140 micromol/L. RESULTS: Compared with those without cardiac involvement, N-TproBNP was significantly increased in SSc patients with heart involvement (median 11 and 219 pmol/L respectively, p<0.0001; CI 136-445). Receiver operating characteristic curves of N-TproBNP to predict the presence of cardiac involvement in SSc gave a sensitivity of 90.5% at a cut-off level of 50 pmol/L, with a specificity of 97.6%. By logistic regression analysis, N-TproBNP levels of 50 pmol/L were shown to be a strong predictor of heart involvement (OR 78, p<0.001, 95%CI 14-424). Moreover, a significant progressive reduction in N-TproBNP after initial presentation of cardiac involvement was observed in a subset of patients during 6 months of follow-up (p=0.023). Levels of N-TproBNP above the median value of 219 pmol/L did not predict survival (p=0.895, by log-rank). N-TproBNP levels strongly correlated negatively with LVEF (r=-0.7384, p=0.0002). CONCLUSIONS: These data suggest N-TproBNP peptide may be a surrogate marker for cardiac involvement in SSc, selectively identifying patients with severe impairment of cardiac function.


Assuntos
Cardiopatias/etiologia , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue , Escleroderma Sistêmico/complicações , Adulto , Idoso , Biomarcadores/sangue , Feminino , Cardiopatias/sangue , Cardiopatias/diagnóstico , Cardiopatias/mortalidade , Cardiopatias/fisiopatologia , Humanos , Estimativa de Kaplan-Meier , Modelos Logísticos , Londres , Masculino , Pessoa de Meia-Idade , Razão de Chances , Valor Preditivo dos Testes , Curva ROC , Estudos Retrospectivos , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/mortalidade , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Volume Sistólico , Regulação para Cima , Função Ventricular Esquerda
15.
Arthritis Rheum ; 63(11): 3531-9, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21769839

RESUMO

OBJECTIVE: To construct a readily applicable formula for selecting patients with systemic sclerosis (SSc) for right-sided heart catheterization (RHC) based on the results of their pulmonary function tests (PFTs). METHODS: The diagnostic value of PFT variables was quantified in 386 patients with SSc against data obtained from RHC. RESULTS: We derived the following formula using data from 257 patients: predicted mPAP = 136 - SpO2 - 0.25 × DLCO % predicted, where mPAP is the mean pulmonary artery pressure, SpO2 is the oxygen saturation as measured by pulse oximetry, and DLCO is the diffusing capacity for carbon monoxide. We validated the formula in the remaining 129 SSc patients. The area under the curve was 0.75 (95% confidence interval [95% CI] 0.67, 0.84). Using a predicted threshold of 25 mm Hg, the sensitivity was 90.1% (95% CI 82, 96) and the specificity was 29.2% (95% CI 17, 44). When used as a screening procedure in a typical scleroderma patient population, it is projected that those with an mPAP below 25 mm Hg are unlikely to have pulmonary hypertension (PH; prevalence 4.4%), those with a predicted mPAP of 25-35 mm Hg are at average risk of having PH (prevalence of 11.3%), and those with a formula-predicted mPAP above 35 mm Hg are likely to have PH (prevalence of 62.9%), thus justifying RHC. In patients with equivocal findings on echocardiography, a high formula-predicted mPAP is strongly associated with the presence of PH. CONCLUSION: We derived and validated an easily applied formula for determining pulmonary function in patients with SSc that identifies subgroups with a low, average, or high prevalence of PH. It provides information that is complementary to echocardiography and that should improve the selection of patients for RHC.


Assuntos
Hipertensão Pulmonar/diagnóstico , Pulmão/fisiopatologia , Escleroderma Sistêmico/complicações , Determinação da Pressão Arterial/métodos , Cateterismo Cardíaco , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Masculino , Valor Preditivo dos Testes , Testes de Função Respiratória , Escleroderma Sistêmico/fisiopatologia , Sensibilidade e Especificidade
16.
Lancet ; 375(9721): 1203-11, 2010 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-20362815

RESUMO

Sudden sensorineural hearing loss is usually unilateral and can be associated with tinnitus and vertigo. In most cases the cause is not identified, although various infective, vascular, and immune causes have been proposed. A careful examination is needed to exclude life threatening or treatable causes such as vascular events and malignant diseases, and patients should be referred urgently for further assessment. About half of patients completely recover, usually in about 2 weeks. Many treatments are used, including corticosteroids, antiviral drugs, and vasoactive and oxygen-based treatments. Although no treatment is proven, we recommend a short course of oral high-dose corticosteroids. There is much to learn about pathogenesis of sudden sensorineural hearing loss, and more clinical trials are needed to establish evidence-based management.


Assuntos
Perda Auditiva Neurossensorial , Administração Oral , Glucocorticoides/administração & dosagem , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/terapia , Humanos , Prognóstico
18.
BMC Med Educ ; 10: 68, 2010 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-20932302

RESUMO

BACKGROUND: Information technology is finding an increasing role in the training of medical students. We compared information recall and student experience and preference after live lectures and video podcasts in undergraduate medical education. METHODS: We performed a crossover randomised controlled trial. 100 students were randomised to live lecture or video podcast for one clinical topic. Live lectures were given by the same instructor as the narrator of the video podcasts. The video podcasts comprised Powerpoint™ slides narrated using the same script as the lecture. They were then switched to the other group for a second clinical topic. Knowledge was assessed using multiple choice questions and qualitative information was collected using a questionnaire. RESULTS: No significant difference was found on multiple choice questioning immediately after the session. The subjects enjoyed the convenience of the video podcast and the ability to stop, review and repeat it, but found it less engaging as a teaching method. They expressed a clear preference for the live lecture format. CONCLUSIONS: We suggest that video podcasts are not ready to replace traditional teaching methods, but may have an important role in reinforcing learning and aiding revision.


Assuntos
Educação de Graduação em Medicina/métodos , Tecnologia Educacional/métodos , Rememoração Mental , Estudantes de Medicina , Ensino/métodos , Webcasts como Assunto , Estudos Cross-Over , Tecnologia Educacional/instrumentação , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Disseminação de Informação , Pesquisa Qualitativa , Inquéritos e Questionários
20.
BMJ Case Rep ; 12(12)2019 Dec 02.
Artigo em Inglês | MEDLINE | ID: mdl-31796457

RESUMO

Calcium pyrophosphate deposition (CPPD) disease is a crystal arthropathy primarily affecting peripheral joints, most commonly the wrist and the knees. However, CPPD in the cervical spine is a rare entity. This report describes a case of CPPD of the cervical spine which presents with symptoms of neck pain and brachalgia. A 62-year-old woman presented with left-sided upper limb and neck pain. MRI scanning revealed a low signal abnormality within the C6 and C7 vertebrae, and the possibility of lymphoma was raised. The patient was recalled for gadolinium-enhanced scans which showed perivertebral and marrow enhancement. Fine-needle aspirate histology initially suggested a spindle cell tumour or lymphoma. However, CT-guided biopsy showed positively birefringent crystals, confirming CPPD. CPPD of the spine is a rare differential of nerve impingement in the cervical spine when MRI scanning perivertebral signal enhancement. Furthermore, CPPD of the spine can mimic malignancy.


Assuntos
Vértebras Cervicais/patologia , Condrocalcinose/patologia , Doenças da Coluna Vertebral/patologia , Biópsia por Agulha Fina , Pirofosfato de Cálcio/análise , Vértebras Cervicais/diagnóstico por imagem , Condrocalcinose/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Biópsia Guiada por Imagem , Linfoma/diagnóstico , Pessoa de Meia-Idade , Cervicalgia/etiologia , Doenças da Coluna Vertebral/diagnóstico , Tomografia Computadorizada por Raios X
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