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1.
Chest ; 157(1): 89-98, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31351047

RESUMO

BACKGROUND: Patients with interstitial lung disease (ILD) may develop pulmonary hypertension (PH), often disproportionate to the severity of the ILD. The right ventricular to left ventricular diameter (RV:LV) ratio measured at CT pulmonary angiogram (CTPA) has been shown to provide valuable information in patients with pulmonary arterial hypertension and to predict death or deterioration in acute pulmonary embolism. METHODS: Demographic characteristics, ILD subtype, echocardiography, and detailed CTPA measurements were collected in consecutive patients undergoing both CTPA and right heart catheterization at the Royal Brompton Hospital between 2005 and 2015. Fibrosis severity was formally scored according to CT criteria. The RV:LV ratio at CTPA was evaluated by using three different methods. Cox proportional hazards analysis was used to assess the relation of CTPA-derived parameters to predict death or lung transplantation. RESULTS: A total of 92 patients were included (64% male; mean age 65 ± 11 years) with an FVC 57 ± 20% predicted, corrected transfer factor of the lung for carbon monoxide 22 ± 8% predicted, and corrected transfer coefficient of the lung for carbon monoxide 51 ± 17% predicted. PH was confirmed at right heart catheterization in 78%. Of all the CTPA-derived measures, an RV:LV ratio ≥ 1.0 strongly predicted mortality or transplantation at univariate analysis (hazard ratio, 3.26; 95% CI, 1.49-7.13; P = .003), whereas invasive hemodynamic data did not. The RV:LV ratio remained an independent predictor at multivariate analysis (hazard ratio, 3.19; 95% CI, 1.44-7.10; P = .004), adjusting for an ILD diagnosis of idiopathic pulmonary fibrosis and CT imaging-derived ILD severity. CONCLUSIONS: An increased RV:LV ratio measured at CTPA provides a simple, noninvasive method of risk stratification in patients with suspected ILD-PH. This should prompt closer follow-up, more aggressive treatment, and consideration of lung transplantation.

2.
Pulm Circ ; 9(2): 2045894019859474, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31246163

RESUMO

A 36-year-old woman presented with recurrent pulmonary emboli (PE) despite oral anticoagulation. She was a type I diabetic with severe gastroparesis requiring insertion of multiple long-term peripherally inserted central catheters (PICC) over a 10-year period. Imaging at presentation demonstrated a PICC-associated mobile mass in the right atrium and signs of pulmonary hypertension (PH). She was thrombolyzed and fully anticoagulated, and diabetic management without PICC strongly recommended. PH persisted, however, and she developed chronic thromboembolic pulmonary hypertension (CTEPH), for which successful pulmonary endarterectomy (PEA) surgery led to symptomatic and hemodynamic improvement. This was the first case of CTEPH reported related to long-term PICC use outside the setting of malignant disease, and a novel observation that the PEA specimen contained multiple plastic fragments. Long-term PICC placement increases the risk of CTEPH, a life-threatening, albeit treatable, complication.

4.
Pulm Circ ; 9(2): 2045894019845615, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30945593

RESUMO

Early diagnosis of pulmonary artery hypertension (PAH) is diagnostically challenging given the extent of pulmonary vascular remodeling required to bring about clinical signs and symptoms. Exercise testing can be invaluable in this setting, as stressing the cardiopulmonary system may unmask early disease. This report describes a young patient with a positive family history of PAH in whom contemporaneous invasive cardiopulmonary exercise testing and surgical lung biopsy reveal the novel association between exercise pulmonary hypertension (ePH) and early histological changes of PAH. Exercise PH currently carries no pathological correlates which means the hemodynamic effects of early pulmonary vascular remodeling remain unknown. Following the recent proceedings from the World Symposium in Pulmonary Hypertension 2018, which broaden the hemodynamic definition of PAH, this report suggests an important association between ePH and early pulmonary vascular remodeling supporting a role for exercise hemodynamic evaluation in patients at increased familial risk of PAH.

6.
Int J Cardiol ; 277: 229-234, 2019 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-30448018

RESUMO

BACKGROUND: Improvements in availability and accuracy of diagnostic testing in pulmonary embolism (PE) in the last 20 years have more recently been paralleled by the introduction of additional anticoagulation agents and treatment strategies. These developments are likely to shape potentially important changes in PE incidence, associated mortality and treatment complications. METHODS: We investigated trends in PE incidence, PE-related mortality and bleeding risk by analysing Hospital Episodes Statistics for England. RESULTS: Between 1997 and 2015, 464,046 patients (53.9% female) were hospitalized with PE in England. The annual number of hospitalizations with an associated diagnosis of PE more than doubled over this period (24,366 in 1998 vs. 53,108 in 2014), with a corresponding increase in PE hospitalization rate (50.2 to 97.8 per 100,000 population/year), evident in all age categories. Mortality at 1 and 3 months decreased over the study period and was significantly associated with age, treatment era and comorbidities. The risk of bleeding resulting in hospitalization or death within 3 and 12 months after the index PE admission increased over the study period (4.3%/5.1% for 1998-2004 versus 6.1%/7.2% for 2010-2014, p < 0.001 for both comparisons). CONCLUSIONS: The incidence of PE doubled in England between 1997 and 2015, likely attributable to raised awareness and ability to diagnose less severe cases. While PE-associated mortality decreased, there was an increase in bleeding risk. Renewed efforts directed at reducing the incidence of bleeding, including consideration of anticoagulation regimens and investigation of anticoagulation requirement in patients with low-risk features, are needed.


Assuntos
Hemorragia/diagnóstico , Hemorragia/mortalidade , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Estudos de Coortes , Inglaterra/epidemiologia , Feminino , Hospitalização , Humanos , Incidência , Lactente , Masculino , Pessoa de Meia-Idade , Mortalidade/tendências , Fatores de Risco , Adulto Jovem
8.
J Am Coll Cardiol ; 72(22): 2778-2788, 2018 12 04.
Artigo em Inglês | MEDLINE | ID: mdl-30497564

RESUMO

Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis. Identification of patients who have developed PH and who may benefit from further investigations (including cardiac catheterization) and treatment is thus extremely important. A systematic review and survey of experts from the United Kingdom and Ireland were performed to assess current knowledge and practice on echocardiographic screening for PH in ACHD. This paper presents the findings of the review and expert statements on the optimal approaches when using echocardiography to assess ACHD patients for PH, with particular focus on major subgroups: patients with right ventricular outflow tract obstruction, patients with systemic right ventricles, patients with unrepaired univentricular circulation, and patients with tetralogy of Fallot with pulmonary atresia.


Assuntos
Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Hipertensão Pulmonar/diagnóstico por imagem , Programas de Rastreamento/métodos , Ecocardiografia/tendências , Cardiopatias Congênitas/epidemiologia , Humanos , Hipertensão Pulmonar/epidemiologia , Imagem Cinética por Ressonância Magnética/métodos , Imagem Cinética por Ressonância Magnética/tendências , Programas de Rastreamento/tendências
9.
Eur J Appl Physiol ; 118(7): 1415-1426, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29713818

RESUMO

BACKGROUND: Right ventricular (RV) dysfunction and heart failure with preserved ejection fraction may contribute to exercise intolerance in obesity. To further define RV exercise responses, we investigated RV-arterial coupling in obesity with and without development of exercise pulmonary venous hypertension (ePVH). METHODS: RV-arterial coupling defined as RV end-systolic elastance/pulmonary artery elastance (Ees/Ea) was calculated from invasive cardiopulmonary exercise test data in 6 controls, 8 obese patients without ePVH (Obese-ePVH) and 8 obese patients with ePVH (Obese+ePVH) within a larger series. ePVH was defined as a resting pulmonary arterial wedge pressure < 15 mmHg but ≥ 20 mmHg on exercise. Exercise haemodynamics were further evaluated in 18 controls, 20 Obese-ePVH and 17 Obese+ePVH patients. RESULTS: Both Obese-ePVH and Obese+ePVH groups developed exercise RV-arterial uncoupling (peak Ees/Ea = 1.45 ± 0.26 vs 0.67 ± 0.18 vs 0.56 ± 0.11, p < 0.001, controls vs Obese-ePVH vs Obese+ePVH respectively) with higher peak afterload (peak Ea = 0.31 ± 0.07 vs 0.75 ± 0.32 vs 0.88 ± 0.62 mL/mmHg, p = 0.043) and similar peak contractility (peak Ees = 0.50 ± 0.16 vs 0.45 ± 0.22 vs 0.48 ± 0.17 mL/mmHg, p = 0.89). RV contractile reserve was highest in controls (ΔEes = 224 ± 80 vs 154 ± 39 vs 141 ± 34% of baseline respectively, p < 0.001). Peak Ees/Ea correlated with peak pulmonary vascular compliance (PVC, r = 0.53, p = 0.02) but not peak pulmonary vascular resistance (PVR, r = - 0.20, p = 0.46). In the larger cohort, Obese+ePVH patients on exercise demonstrated higher right atrial pressure, lower cardiac output and steeper pressure-flow responses. BMI correlated with peak PVC (r = - 0.35, p = 0.04) but not with peak PVR (r = 0.24, p = 0.25). CONCLUSIONS: Exercise RV-arterial uncoupling and reduced RV contractile reserve further characterise obesity-related exercise intolerance. RV dysfunction in obesity may develop independent of exercise LV filling pressures.


Assuntos
Função Atrial , Tolerância ao Exercício , Hipertensão Pulmonar/fisiopatologia , Contração Miocárdica , Obesidade/fisiopatologia , Função Ventricular , Idoso , Circulação Coronária , Exercício , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Masculino , Pessoa de Meia-Idade , Obesidade/complicações , Obesidade/diagnóstico por imagem , Circulação Pulmonar
10.
Heart ; 104(23): 1963-1969, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-29776964

RESUMO

OBJECTIVES: Approximately 5%-10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain. METHODS: All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis. RESULTS: A total of 310 patients (mean age 34.9±12.3 years, 36.8% male) were enrolled. The majority had Eisenmenger syndrome (58.4%), 15.2% had a prior defect repair and a third had Down syndrome. At baseline, 14.2% had a prior history of arrhythmia, mostly supraventricular arrhythmia (86.4%). During a median follow-up of 6.1 years, 64 patients developed at least one new arrhythmic episode (incidence 3.47% per year), mostly supraventricular tachycardia or atrial fibrillation (78.1% of patients). Arrhythmia was associated with symptoms in 75.0% of cases. The type of PAH-CHD, markers of disease severity and prior arrhythmia were associated with arrhythmia during follow-up. Arrhythmia was a strong predictor of death, even after adjusting for demographic and clinical variables (HR 3.41, 95% CI 2.10 to 5.53, p<0.0001). CONCLUSIONS: Arrhythmia is common in PAH-CHD and is associated with an adverse long-term outcome, even when managed in a specialist centre.


Assuntos
Arritmias Cardíacas , Cardiopatias Congênitas , Hipertensão Pulmonar , Efeitos Adversos de Longa Duração/mortalidade , Qualidade de Vida , Adulto , Arritmias Cardíacas/classificação , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/terapia , Feminino , Grécia/epidemiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico , Incidência , Expectativa de Vida , Masculino , Pessoa de Meia-Idade , Administração dos Cuidados ao Paciente/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença
11.
ERJ Open Res ; 4(2)2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29750141

RESUMO

European Respiratory Society (ERS) guidelines recommend the assessment of patients with interstitial lung disease (ILD) and severe pulmonary hypertension (PH), as defined by a mean pulmonary artery pressure (mPAP) ≥35 mmHg at right heart catheterisation (RHC). We developed and validated a stepwise echocardiographic score to detect severe PH using the tricuspid regurgitant velocity and right atrial pressure (right ventricular systolic pressure (RVSP)) and additional echocardiographic signs. Consecutive ILD patients with suspected PH underwent RHC between 2005 and 2015. Receiver operating curve analysis tested the ability of components of the score to predict mPAP ≥35 mmHg, and a score devised using a stepwise approach. The score was tested in a contemporaneous validation cohort. The score used "additional PH signs" where RVSP was unavailable, using a bootstrapping technique. Within the derivation cohort (n=210), a score ≥7 predicted severe PH with 89% sensitivity, 71% specificity, positive predictive value 68% and negative predictive value 90%, with similar performance in the validation cohort (n=61) (area under the curve (AUC) 84.8% versus 83.1%, p=0.8). Although RVSP could be estimated in 92% of studies, reducing this to 60% maintained a fair accuracy (AUC 74.4%). This simple stepwise echocardiographic PH score can predict severe PH in patients with ILD.

12.
Heart ; 104(1): 45-50, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-28663364

RESUMO

OBJECTIVES: Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome. METHODS: Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years. RESULTS: Age at baseline was 34.6±11.4 years and 34.1% were men. Mean platelet count and MPV were 152.6±73.3×109/L and 9.6±1.2 fL, respectively. A strong inverse correlation was found between platelet count and haemoglobin concentration and MPV. During the study, there were 39 deaths, and 21 thrombotic and 43 bleeding events. On univariate Cox regression analysis, patients with a platelet count <100×109/L had a twofold increased mortality (HR 2.10, 95% CI 1.10 to 4.01, p=0.024). Platelet count was not associated with an increased risk of thrombosis. However, there was a threefold increased thrombotic risk with MPV >9.5 fL (HR 3.50, 95% CI 1.28 to 9.54, p=0.015). Patients with either severe secondary erythrocytosis (>220g/L) or anaemia (<130g/L) were at higher risk of thrombotic events (HR 3.93, 95% CI 1.60 to 9.67, p=0.003; and HR 4.75, 95% CI 1.03 to 21.84, p=0.045, respectively). CONCLUSIONS: Thrombocytopaenia significantly increased the risk of mortality in ES. Furthermore, raised MPV, severe secondary erythrocytosis and anaemia, but not platelet count, were associated with an increased risk of thrombotic events in our adult cohort.


Assuntos
Plaquetas/fisiologia , Complexo de Eisenmenger/sangue , Previsões , Trombocitopenia/sangue , Adulto , Complexo de Eisenmenger/complicações , Complexo de Eisenmenger/mortalidade , Feminino , Seguimentos , Humanos , Masculino , Volume Plaquetário Médio , Contagem de Plaquetas , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Trombocitopenia/complicações , Reino Unido/epidemiologia
14.
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
15.
Physiol Rep ; 5(7)2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28373412

RESUMO

Chronic thromboembolic disease (CTED) is suboptimally defined by a mean pulmonary artery pressure (mPAP) <25 mmHg at rest in patients that remain symptomatic from chronic pulmonary artery thrombi. To improve identification of right ventricular (RV) pathology in patients with thromboembolic obstruction, we hypothesized that the RV ventriculo-arterial (Ees/Ea) coupling ratio at maximal stroke work (Ees/Eamax sw) derived from an animal model of pulmonary obstruction may be used to identify occult RV dysfunction (low Ees/Ea) or residual RV energetic reserve (high Ees/Ea). Eighteen open chested pigs had conductance catheter RV pressure-volume (PV)-loops recorded during PA snare to determine Ees/Eamax sw This was then applied to 10 patients with chronic thromboembolic pulmonary hypertension (CTEPH) and ten patients with CTED, also assessed by RV conductance catheter and cardiopulmonary exercise testing. All patients were then restratified by Ees/Ea. The animal model determined an Ees/Eamax sw = 0.68 ± 0.23 threshold, either side of which cardiac output and RV stroke work fell. Two patients with CTED were identified with an Ees/Ea well below 0.68 suggesting occult RV dysfunction whilst three patients with CTEPH demonstrated Ees/Ea ≥ 0.68 suggesting residual RV energetic reserve. Ees/Ea > 0.68 and Ees/Ea < 0.68 subgroups demonstrated constant RV stroke work but lower stroke volume (87.7 ± 22.1 vs. 60.1 ± 16.3 mL respectively, P = 0.006) and higher end-systolic pressure (36.7 ± 11.6 vs. 68.1 ± 16.7 mmHg respectively, P < 0.001). Lower Ees/Ea in CTED also correlated with reduced exercise ventilatory efficiency. Low Ees/Ea aligns with features of RV maladaptation in CTED both at rest and on exercise. Characterization of Ees/Ea in CTED may allow for better identification of occult RV dysfunction.


Assuntos
Circulação Pulmonar/fisiologia , Embolia Pulmonar/fisiopatologia , Disfunção Ventricular Direita/fisiopatologia , Adulto , Idoso , Animais , Doença Crônica , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Suínos
16.
Pulm Circ ; 4(1): 137-41, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25006429

RESUMO

Pulmonary endarterectomy offers a symptomatic and survival benefit in patients with chronic thromboembolic pulmonary hypertension through sustained improvement in right ventricular function. However, its role in patients with symptom limitation, chronic thrombotic obstruction, and a normal pulmonary hemodynamic profile is less clear. Cardiopulmonary exercise testing (CPET) stresses the cardiopulmonary system and has a characteristic response in pulmonary hypertension. CPET may therefore reveal abnormalities in patients with chronic thrombotic obstruction where hemodynamic investigations conducted at rest are reassuring. Using incremental CPET, we demonstrated improvements in right ventricular performance and ventilatory efficiency following pulmonary endarterecomy in a patient with preoperative exercise limitation and normal pulmonary hemodynamics. Careful evaluation of exercise responses may extend the potential benefit offered by pulmonary endarterectomy in patients with chronic thromboembolic obstruction irrespective of their resting hemodynamic profile.

17.
Cardiol Rev ; 22(5): 233-40, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24621859

RESUMO

This review explores the historical development of conductance catheterization of the human right ventricle (RV) and its current diagnostic role in assessing RV function in an era of contemporary noninvasive imaging. Significant advances in echocardiography, cardiothoracic computed tomography, and cardiac magnetic resonance in the last decade have led a paradigm shift away from invasive hemodynamic assessment toward noninvasive measurement of RV function. However, imaging modalities are not without some disadvantages. For example, they do not provide the beat-to-beat pressure-volume assessment that is obtained by conductance catheterization, which is still arguably the gold standard for measuring cardiac performance. Right heart catheterization remains an integral part of the assessment of patients with RV dysfunction, and a burgeoning interest in early RV insufficiency in a range of cardiopulmonary diseases has brought the detailed interrogation of RV function back into focus. Although conductance volume assessment of the RV is more limited than the left ventricle, the scope for improving the understanding of RV adaptation to pulmonary and valvular pathologies and RV myocardial disease by using a conductance catheter still exists. We review the up-to-date functional applications of echocardiography and computed tomography/cardiac magnetic resonance to assess the RV and illustrate the potential of contemporary conductance catheter techniques to complement noninvasive imaging in the assessment of RV function.


Assuntos
Cateterismo Cardíaco/tendências , Técnicas de Imagem Cardíaca/tendências , Cateteres , Função Ventricular Direita , Ecocardiografia , Humanos , Imagem por Ressonância Magnética , Tomografia Computadorizada Multidetectores , Contração Miocárdica
18.
J Appl Physiol (1985) ; 116(4): 355-63, 2014 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-24356516

RESUMO

Pressure-volume loops describe dynamic ventricular performance, relevant to patients with and at risk of pulmonary hypertension. We used conductance catheter-derived pressure-volume loops to measure right ventricular (RV) mechanics in patients with chronic thromboembolic pulmonary arterial obstruction at different stages of pathological adaptation. Resting conductance catheterization was performed in 24 patients: 10 with chronic thromboembolic pulmonary hypertension (CTEPH), 7 with chronic thromboembolic disease without pulmonary hypertension (CTED), and 7 controls. To assess the validity of conductance measurements, RV volumes were compared in a subset of 8 patients with contemporaneous cardiac magnetic resonance (CMR). Control, CTED, and CTEPH groups showed different pressure-volume loop morphology, most notable during systolic ejection. Prolonged diastolic relaxation was seen in patients with CTED and CTEPH [tau = 56.2 ± 6.7 (controls) vs. 69.7 ± 10.0 (CTED) vs. 67.9 ± 6.2 ms (CTEPH), P = 0.02]. Control and CTED groups had lower afterload (Ea) and contractility (Ees) compared with the CTEPH group (Ea = 0.30 ± 0.10 vs. 0.52 ± 0.24 vs. 1.92 ± 0.70 mmHg/ml, respectively, P < 0.001) (Ees = 0.44 ± 0.20 vs. 0.59 ± 0.15 vs. 1.13 ± 0.43 mmHg/ml, P < 0.01) with more efficient ventriculoarterial coupling (Ees/Ea = 1.46 ± 0.30 vs. 1.27 ± 0.36 vs. 0.60 ± 0.18, respectively, P < 0.001). Stroke volume assessed by CMR and conductance showed closest agreement (mean bias +9 ml, 95% CI -1 to +19 ml) compared with end-diastolic volume (+48 ml, -16 to 111 ml) and end-systolic volume (+37 ml, -21 to 94 ml). RV conductance catheterization detects novel alteration in pressure-volume loop morphology and delayed RV relaxation in CTED, which distinguish this group from controls. The observed agreement in stroke volume assessed by CMR and conductance suggests RV mechanics are usefully measured by conductance catheter in chronic thromboembolic obstruction.


Assuntos
Cateterismo Cardíaco , Hipertensão Pulmonar/etiologia , Artéria Pulmonar/fisiopatologia , Embolia Pulmonar/complicações , Volume Sistólico , Disfunção Ventricular Direita/etiologia , Função Ventricular Direita , Pressão Ventricular , Adulto , Idoso , Pressão Arterial , Estudos de Casos e Controles , Cateterismo de Swan-Ganz , Hipertensão Pulmonar Primária Familiar , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/fisiopatologia , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/fisiopatologia , Reprodutibilidade dos Testes , Disfunção Ventricular Direita/diagnóstico , Disfunção Ventricular Direita/fisiopatologia
19.
Thromb Res ; 132(6): 659-65, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24157082

RESUMO

INTRODUCTION: Persistent obstruction in the pulmonary artery following acute pulmonary embolism (PE) can give rise to both chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic disease without PH (CTED). We hypothesised that cardiopulmonary exercise testing (CPET) may be able to differentiate patients with CTEPH and CTED following unresolved PE which may help guide patient assessment. MATERIALS AND METHODS: Fifteen patients with CTEPH and 15 with CTED all diagnosed after PE underwent CT pulmonary angiography, CPET and resting right heart catheterisation. Exercise variables were compared between patients with CTEPH, CTED and 10 sedentary controls and analysed as predictors of a CTEPH diagnosis. Proximal thrombotic burden in CTEPH and CTED was quantified using CT criteria. RESULTS: Physiological dead space (Vd/Vt) (34.5±11.4 vs 50.8±6.6 %, p<0.001) and alveolar-arterial oxygen gradient (29±16 vs 46±12mmHg, p <0.001) at peak exercise strongly differentiated CTED and CTEPH groups respectively. Resting ventilatory efficiency also differed from control subjects. In both univariate and multivariate analyses, peak exercise Vd/Vt predicted a diagnosis of CTEPH (ROC AUC>0.88, 0.67 - 0.97) despite a similar degree of proximal thrombotic obstruction to the CTED group (67.5, 55 - 70% and 72.5, 60 - 80% respectively, p=0.08). CONCLUSIONS: Gas exchange at peak exercise differentiates CTED and CTEPH after PE that can present with no apparent relation to the degree of proximal thrombotic burden. A potential role for CPET exists in guiding further clinical investigations in this setting.


Assuntos
Exercício/fisiologia , Hipertensão Pulmonar/metabolismo , Embolia Pulmonar/metabolismo , Troca Gasosa Pulmonar/fisiologia , Doença Crônica , Teste de Esforço , Tolerância ao Exercício/fisiologia , Feminino , Humanos , Hipertensão Pulmonar/sangue , Masculino , Pessoa de Meia-Idade , Embolia Pulmonar/sangue
20.
Chest ; 144(2): 522-530, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23430021

RESUMO

BACKGROUND: The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is a disease-specific assessment tool used for the evaluation and follow-up of patients with pulmonary hypertension (PH). We describe a novel use for this questionnaire in its potential to predict clinical deterioration (CD) in two patient cohorts with subtypes of PH, idiopathic pulmonary arterial hypertension (IPAH), and chronic thromboembolic pulmonary hypertension (CTEPH) during an 8-year period. METHODS: We retrospectively analyzed CAMPHOR scores obtained at baseline and at follow-up visits in patients under the care of our unit over an 8-year period to assess CD and survival, as well as 6-min walk distance (6MWD) and New York Heart Association (NYHA) class. RESULTS: Using Cox regression, we demonstrated a significant predictive effect of CD from total CAMPHOR scores at study enrollment in IPAH and CTEPH (hazard ratios, 1.03 [95% CI, 1.01-1.05] and 1.04 [95% CI, 1.02-1.06] per unit score increase, respectively), as well as from CAMPHOR subscales as independent predictors. This predictive effect is diluted after adjusting for the prognostic effect of 6MWD and NYHA class. Repeated CAMPHOR assessment over time appears not to add predictive value of CD to that obtained at diagnosis, although it still informs physicians of important changes in self-reported symptoms. CONCLUSIONS: When emphasis is placed on the evaluation of patient perceptions, CAMPHOR may represent an alternative method of estimating the likelihood of CD.


Assuntos
Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Inquéritos e Questionários , Tromboembolia/complicações , Doença Crônica , Progressão da Doença , Hipertensão Pulmonar Primária Familiar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Retrospectivos , Caminhada/fisiologia
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