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AIMS: The use of loop diuretics in pulmonary arterial hypertension (PAH) is less frequent compared with heart failure. The clinical and prognostic characteristics of PAH patients according to loop diuretic use remain unexplored. In this study, we retrospectively analysed the characteristics and survival of PAH patients requiring different doses of loop diuretics. METHODS AND RESULTS: Patients diagnosed with PAH between 2001 and 2022 at seven European centres for the management of PAH. According to the median equivalent dose of furosemide in the overall cohort, patients were divided into two subgroups: no/low-dose loop diuretic and high-dose loop diuretic. Primary outcome was 5 year all-cause mortality. Among the 397 patients included, 227 (57%) were treated with loop diuretics. Median daily furosemide equivalent dose was 25 mg, and accordingly patients were divided in no/low dose (i.e. ≤25 mg, n = 257, 65%) vs. high dose (i.e. >25 mg, n = 140, 35%). Patients in the high-dose group were older, more likely to have comorbidities, and had a more severe disease according to the ESC/ERS risk category. Crude 5 year survival was significantly shorter in patients in the high-dose group, but after adjustment for age, sex, and risk category, high loop diuretic dose was not significantly associated with the primary outcome. CONCLUSIONS: Use of high dose of loop diuretics in PAH is associated with a higher burden of comorbidities, more severe disease, and worse survival. However, in PAH, the need of high loop diuretic dose is a marker of disease severity and not an independent prognostic factor.
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A comprehensive evaluation of risk, using multiple indices, is necessary to provide reliable prognostic information and guide therapy in pulmonary arterial hypertension (PAH). The current ESC/ERS guidelines suggest using a three-strata model for incident (newly diagnosed) patients and a four-strata model for prevalent patients with PAH. The four-strata model serves as a fundamental risk-stratification tool and relies on a minimal dataset of indicators that must be considered during follow-up. Nevertheless, there are still areas of vagueness and ambiguity when classifying and managing patients in the intermediate-risk category. For these patients, considerations should include right heart imaging, hemodynamics, as well as individual factors such as age, sex, genetic profile, disease type, comorbidities, and kidney function. The aim of this report is to present case studies, with a specific focus on patients ultimately classified as intermediate risk. We aim to emphasize the challenges and complexities encountered in the realms of diagnosis, classification, and treatment for these particular patients.
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Anti-Hipertensivos , Guias de Prática Clínica como Assunto , Hipertensão Arterial Pulmonar , Humanos , Fatores de Risco , Medição de Risco , Anti-Hipertensivos/uso terapêutico , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/fisiopatologia , Hipertensão Arterial Pulmonar/terapia , Hipertensão Arterial Pulmonar/epidemiologia , Feminino , Masculino , Valor Preditivo dos Testes , Medicina Baseada em Evidências/normas , Resultado do Tratamento , Pessoa de Meia-Idade , Tomada de Decisão Clínica , Artéria Pulmonar/fisiopatologia , Pressão Arterial/efeitos dos fármacos , Técnicas de Apoio para a DecisãoRESUMO
AIM: To obtain real-world evidence about the features and risk stratification of pulmonary arterial hypertension (PAH) with a left heart disease (LHD) phenotype (PAH-LHD). METHODS AND RESULTS: By reviewing the records of consecutive incident PAH patients at 7 tertiary centers from 2001 to 2021, we selected 286 subjects with all parameters needed to determine risk of death at baseline and at first follow-up with COMPERA and COMPERA 2.0 scores. Fifty seven (20%) had PAH-LHD according to the AMBITION definition. Compared with no-LHD ones, they were older, had higher BMI, more cardiovascular comorbidities, higher E/e' ratio and left atrial area, but lower BNP concentrations and better right ventricular function and pulmonary hemodynamics. Survival was comparable between PAH-LHD and no-LHD patients, although the former were less commonly treated with dual PAH therapy. Both COMPERA and COMPERA 2.0 discriminated all-cause mortality risk of PAH-LHD at follow-up, but not at baseline. Risk profile significantly improved during follow-up only when assessed by COMPERA 2.0. At multivariable analysis with low-risk status as reference, intermediate-high and high-risk, but not LHD phenotype, were associated with higher hazard of all-cause mortality. Results were comparable in secondary analyses including patients in the last 10 years and atrial fibrillation and echocardiographic abnormalities as additional criteria for PAH-LHD. CONCLUSIONS: In real life, PAH-LHD patients are frequent, have less severe disease and are less likely treated with PAH drug combinations than no-LHD. The COMPERA 2.0 model may be more appropriate to evaluate their mortality risk during follow-up and how it is modulated by therapy.
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BACKGROUND: An app providing material for education and entertaining is a possible way to support patients and healthcare providers in achieving person-centered care. METHODS: An app tailored on the Fondazione Toscana Gabriele Monasterio (FTGM), a research hospital treating cardiac and lung disorders, was created. A pilot evaluation project was conducted on consecutive patients hospitalized for heart or lung disorders. Patients were asked to complete an assessment questionnaire. RESULTS: The FTGM app provides information on diagnostic and therapeutic investigations, hospital and healthcare personnel, and includes content for entertainment and learning. It was tested on 215 consecutive patients (75% men, 66% aged >60âyears, and 40% with a primary or middle school degree). Sixty-nine percentage of patients used the FTGM app, including 67% of patients aged >80âyears and 65% of those with an elementary education (65%). Patients gave positive feedback on the app layout. Many (76%) looked for information on doctors and nurses in the 'People' section. Sixty-five percent of responders had used at least one of the sections called 'Music' and 'Museum visits'. The app helped many patients perceive the hospital as a more liveable place (68%), and to feel less anxious (76%), and more engaged in the diagnostic and therapeutic workup (65%). Overall, the majority of responders (87%) rated the app as 'excellent' or 'good', and almost all (95%) would have recommended other patients to use the app. CONCLUSIONS: The FTGM app is a possible tool to improve patient wellbeing during hospitalization.
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Pneumopatias , Aplicativos Móveis , Feminino , Humanos , Masculino , Saúde Digital , Pacientes Internados , Pneumopatias/diagnóstico , Pneumopatias/terapia , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou maisRESUMO
Obstructive sleep apneas (OSA) are a breathing disorder characterized by recurrent apneas and hypopneas associated with complete or partial obstruction of the upper airways during sleep, resulting in disturbed sleep architecture, repeated hypoxemia and awakenings, and daytime sleepiness. OSA syndrome affects up to 34% of men and 17% of women in Western countries. Abnormalities in upper airway anatomy (frequently due to obesity), muscle tone, or neural control of breathing are the main causes. OSA is associated with impaired cognitive function and favors the onset of hypertension, being a major determinant of resistant hypertension, and may favor cardiovascular diseases (e.g., coronary artery disease and heart failure), thereby increasing mortality. Polysomnography and (cardio)-respiratory portable systems are used to diagnose and determine the severity of OSA. Management of OSA includes lifestyle modifications, such as weight loss and avoidance of supine sleep position, and continuous positive airway pressure. Mandibular advancement devices and upper airway surgery may also be appropriate for some patients. Hypoglossal nerve stimulation and pharmacological interventions are currently investigated to improve symptoms and outcomes.
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Doenças Cardiovasculares , Doença da Artéria Coronariana , Hipertensão , Apneia Obstrutiva do Sono , Masculino , Humanos , Feminino , Doenças Cardiovasculares/complicações , Fatores de Risco , Apneia Obstrutiva do Sono/complicações , Apneia Obstrutiva do Sono/terapia , Doença da Artéria Coronariana/complicações , Hipertensão/complicações , Pressão Positiva Contínua nas Vias Aéreas/efeitos adversosRESUMO
BACKGROUND: Risk scores are important tools for the prognostic stratification of pulmonary arterial hypertension (PAH). Their performance and the additional impact of comorbidities across age groups is unknown. METHODS: Patients with PAH enrolled from 2001 to 2021 were divided in ≥65 years old vs <65 years old patients. Study outcome was 5-year all-cause mortality. French Pulmonary Hypertension Network (FPHN), FPHN noninvasive, Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) and Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL 2.0) risk scores were calculated and patients categorized at low, intermediate and high risk. Number of comorbidities was calculated. RESULTS: Among 383 patients, 152 (40%) were ≥65 years old. They had more comorbidities (number of comorbidities 2, IQR 1-3, vs 1, IQR 0-2 in <65 years patients). Five-year survival was 63% in ≥65 vs 90% in <65 years. Risk scores correctly discriminated the different classes of risk in the overall cohort and in the older and younger groups. REVEAL 2.0 showed the best accuracy in the total cohort (C-index 0.74, standard error-SE- 0.03) and older (C-index 0.69, SE 0.03) patients, whereas COMPERA 2.0 performed better in younger patients (C-index 0.75, SE 0.08). Number of comorbidities was associated with higher 5-year mortality, and consistently increased the accuracy of risk scores, in younger but not in older patients. CONCLUSIONS: Risk scores have similar accuracy in the prognostic stratification of older vs younger PAH patients. REVEAL 2.0 had the best performance in older patients and COMPERA 2.0 had it in younger patients. Comorbidities increased the accuracy of risk scores only in younger patients.
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Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Humanos , Idoso , Hipertensão Arterial Pulmonar/epidemiologia , Hipertensão Pulmonar Primária Familiar , Fatores de Risco , Sistema de Registros , Medição de RiscoRESUMO
Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure >20 mmHg at rest during right heart catheterization. PH prevalence is about 1% of the global population. The PH clinical classification includes five groups: pulmonary arterial hypertension, PH associated with left heart disease, PH associated with lung disease, PH associated with pulmonary artery obstructions, PH with unclear and/or multifactorial mechanisms. In case of clinical suspicion, echocardiography is the first-line tool to start the diagnostic process. Right heart catheterization is the gold standard for diagnosis of PH, requires great experience and should be performed in expert centers. The classification of the PH patient in a specific subgroup requires multidisciplinary clinical and instrumental skills that only a reference center can provide. This document proposes a clinical pathway for the management of PH patients in the Tuscany region in order to standardize access to specialized care.
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Cardiopatias , Hipertensão Pulmonar , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Procedimentos Clínicos , Cateterismo Cardíaco , EcocardiografiaRESUMO
BACKGROUND: Pulmonary hypertension (PH) is a frequent and detrimental condition. Right heart catheterization (RHC) is the gold standard to identify PH subtype (precapillary from postcapillary PH) and is key for treatment allocation. In this study, the novel echocardiographic biventricular coupling index (BCI), based on the ratio between right ventricular stroke work index and left ventricular E/E' ratio, was tested for the discrimination of PH subtype using RHC as the comparator. METHODS: BCI was derived in 334 consecutive patients who underwent transthoracic echocardiography and RHC for all indications. BCI was then tested in a validation cohort of 1,349 patients. RESULTS: The accuracy of BCI to identify precapillary PH was high in the derivation cohort (area under the curve, 0.82; 95% CI, 0.78-0.88; P < .001; optimal cut point, 1.9). BCI identified patients with precapillary PH with high accuracy also in the validation cohort (area under the curve, 0.87 [95% CI, 0.85-0.89; P < .001]; subgroup with PH: area under the curve, 0.91 [95% CI, 0.89-0.93; P < .001]; cut point, 1.9; sensitivity, 82%; specificity, 89%; positive predictive value, 77%; negative predictive value, 92%). BCI outperformed both the D'Alto score (Z = 3.56; difference between areas = 0.05; 95% CI, 0.02-0.07; P < .001) and the echocardiographic pulmonary-to-left atrial ratio index (Z = 2.88; difference between areas = 0.02; 95% CI, 0.01-0.04; P = .004). CONCLUSIONS: BCI is a novel, noninvasive index based on routinely available echocardiographic parameters that identifies with high accuracy patients with precapillary PH. BCI may be of value in the screening workup of patients with PH.
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Hipertensão Pulmonar , Cateterismo Cardíaco , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Função Ventricular DireitaRESUMO
AIMS: This study aimed to evaluate a novel echocardiographic algorithm for quantitative estimation of pulmonary artery wedge pressure (PAWP) and pulmonary vascular resistance (PVR) in patients with heart failure and pulmonary hypertension (PH) scheduled to right heart catheterization (RHC). METHODS AND RESULTS: In this monocentric study, 795 consecutive patients (427 men; age 68.4 ± 12.1 years) undergoing echocardiography and RHC were evaluated. Multiple regression analysis was performed to identify echocardiographic predictors of PAWP and PVR measured by RHC in the derivation group (the first 200 patients). The diagnostic accuracy of the model was then tested in the validation group (the remaining 595 patients). PH was confirmed by RHC in 507 (63.8%) patients, with 192 (24.2%) cases of precapillary PH, 248 (31.2%) of postcapillary PH, and 67 (8.4%) of combined PH. At regression analysis, tricuspid regurgitation maximal velocity, mitral E/e' ratio, left ventricular ejection fraction, right ventricular fractional area change, inferior vena cava diameter, and left atrial volume index were included in the model (R = 0.8, P < 0.001). The model showed a high diagnostic accuracy in estimating elevated PAWP (area under the receiver operating characteristic curve = 0.97, 92% sensitivity, and 93% specificity, P < 0.001) and PVR (area under the receiver operating characteristic curve = 0.96, 89% sensitivity, and 92% specificity, P < 0.001), outperforming 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging recommendations (P < 0.001) and Abbas' equation (P < 0.001). Bland-Altman analysis showed satisfactory limits of agreement between echocardiography and RHC for PAWP (bias 0.7, 95% confidence interval -7.3 to 8.7) and PVR (bias -0.1, 95% confidence interval -2.2 to 1.9 Wood units), without indeterminate cases. CONCLUSIONS: A novel quantitative echocardiographic approach for the estimation of PAWP and PVR has high diagnostic accuracy in patients with heart failure and PH.
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Ecocardiografia , Função Ventricular Esquerda , Idoso , Idoso de 80 Anos ou mais , Humanos , Masculino , Pessoa de Meia-Idade , Pressão Propulsora Pulmonar , Volume Sistólico , Resistência VascularRESUMO
BACKGROUND: Pulmonary arterial hypertension (PAH) is characterized by the right ventricle (RV) remodeling and pulmonary endothelial dysfunction. We studied cardiac perfusion and innervation in PAH with a cadmium-zinc-telluride (CZT) scanner and lung uptake impairment of 123I-metaiodobenzylguanidine (123I-MIBG). METHODS: In 13 patients with newly diagnosed PAH and 11 dilated cardiomyopathies (DCM, for comparative purposes), we assessed early and delayed 123I-MIBG uptake ratios of lung-to-mediastinum (L/M) and heart-to-mediastinum (H/M) on anterior planar images. A quantitative myocardial innervation with 123I-MIBG and perfusion with 99mTc-tetrofosmin using CZT-SPECT was performed. All patients underwent right heart catheterization. RESULTS: Early and delayed L/M ratios in PAH were lower than DCM (1.47 ± 0.14 vs 1.98 ± 0.11 and 1.40 ± 0.13 vs 1.83 ± 0.09; P < .001), while early and delayed H/M were impaired but not different (1.73 ± 0.20 vs 1.65 ± 0.18 and 1.73 ± 0.27 vs 1.58 ± 0.19). RV perfusion and early innervation were significantly higher in PAH compared to DCM (68.4 ± 13.4 vs 28.6 ± 4.1 and 58.8 ± 9.3 vs 27 ± 2.2; P < .001); delayed RV innervation was not evaluable. RV/LV perfusion and innervation ratios were significantly related (R = 0.74; P < .0001) and had a significant correlation with clinical, hemodynamic, and morpho-functional parameters, including L/M ratios. CONCLUSION: Cardio-pulmonary scintigraphy through a perfusion and innervation study is feasible and may identify pulmonary vascular and RV remodeling, as in PAH.
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Circulação Coronária , Coração/inervação , Hipertensão Arterial Pulmonar/diagnóstico por imagem , Remodelação Ventricular/fisiologia , 3-Iodobenzilguanidina , Adulto , Idoso , Idoso de 80 Anos ou mais , Cádmio , Cardiomiopatia Dilatada/diagnóstico por imagem , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Hipertensão Arterial Pulmonar/fisiopatologia , Telúrio , Tomografia Computadorizada de Emissão de Fóton Único , ZincoRESUMO
BACKGROUND: Right ventricular (RV) function and right atrial (RA) remodeling are major determinants of outcome in pulmonary arterial hypertension (PAH). Strain echocardiography is emerging as a valuable approach for the study of RV and RA function. We sought to assess the incremental prognostic value of serial combined speckle-tracking examination of right chambers in newly diagnosed therapy-naïve PAH patients. METHODS: The study endpoint was a composite of all-cause mortality, hospitalizations due to worsening PAH, and initiation of parenteral prostanoids. Patients were assessed at baseline and at first revaluation after initiation of treatment. Right ventricular free-wall longitudinal strain (FWLS) and RA peak atrial longitudinal strain (PALS) were used as measures of RV and RA function. RESULTS: Eighty-three patients were included. Mean RV-FWLS and RA-PALS were -13.9% ± 6.1% and 23.1% ± 11.4%. The best performing prognostic score among the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension, French Pulmonary Hypertension Registry, and Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) scores was the REVEAL (area under the curve = 0.79, P < .001). With the identified cutoffs, both RV-FWLS (hazard ratio for RV-FWLS < -13.2% = 0.366; 95% CI, 0.159-0.842; P = .018) and RA-PALS (hazard ratio for RA-PALS > 20% = 0.399; 95% CI, 0.176-0.905; P = .028) were independently associated with the primary outcome after correction for the REVEAL score. The combined assessment of RV-FWLS and RA-PALS in addition to the REVEAL score determined a net improvement in prediction of 0.439 (95% CI, 0.070-0.888, P = .04). At 5 months (interquartile range, 4-8) of follow-up, RV-FWLS and RA-PALS improved significantly only in patients free from the primary outcome (P < .001 and P = .001, respectively). CONCLUSIONS: The combined assessment of RV-FWLS and RA-PALS determined an improvement in outcome prediction of validated prognostic risk scores and should be considered within the multiparametric evaluation of patients with PAH.
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Hipertensão Pulmonar , Hipertensão Arterial Pulmonar , Disfunção Ventricular Direita , Função do Átrio Direito , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Disfunção Ventricular Direita/diagnóstico por imagem , Função Ventricular DireitaAssuntos
Tamponamento Cardíaco/etiologia , Hemangioma/tratamento farmacológico , Pericárdio/efeitos dos fármacos , Pirazóis/efeitos adversos , Piridonas/efeitos adversos , Idoso , Dispneia/etiologia , Eletrocardiografia/métodos , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Derrame Pericárdico/etiologia , Pericárdio/anormalidades , Pirazóis/farmacocinética , Pirazóis/uso terapêutico , Piridonas/farmacocinética , Piridonas/uso terapêutico , Radiografia/métodosRESUMO
In chronic heart failure (CHF) due to systemic cardiovascular disease, obese patients have better survival. Bodyweight versus survival was analyzed post hoc in subjects with limited scleroderma (SSc) and isolated pulmonary artery hypertension (PAH), i.e. with CHF due to pulmonary vascular disease. Rheumatologists referred scleroderma subjects for evaluation, and PAH was ascertained by right heart catheterization (RHC). Forty-nine SSc-PAH subjects were stratified by body mass index (BMI): obese 7 (14.3 %), overweight 11 (22.4 %), normal weight 21 (42.9 %), and underweight 10 (20.4 %) for 24-month follow-up and pooled together for long-term 72-month follow-up. Survival was analyzed by Kaplan-Meier method. Multivariate Cox proportional hazards modeling helped to assess variables associated to survival. At 24 months (17 events), survival increases with BMI across four groups (logrank for trend P = 0.031). By Cox multivariate mortality, best model included: BMI (P = 0.043), low lung diffusion (DLco, P = 0.007), and reduced stroke volume index (SVI, P = 0.017). At 72 month (37 events), higher BMI values were associated with better survival but not significantly (P = 0.076). By multivariate modeling BMI did not enter any model, whereas low DLco entered all (P < 0.001). Also low SVI (P = 0.02) and low mixed venous saturation (SvO2, P = 0.009) were associated with the prognosis. From PAH diagnosis to final event, BMI had small (5.4 %), but significant decline (P < 0.001). This is ascribed to CHF progression, and may explain BMI predictive power weakening. The results suggest BMI decline should be contrasted, DLco is useful for screening and with SVI and SvO2 for assessing prognosis and treatment.
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Peso Corporal/fisiologia , Hipertensão Pulmonar/mortalidade , Artéria Pulmonar/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Índice de Massa Corporal , Feminino , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Modelos Logísticos , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Testes de Função Respiratória/estatística & dados numéricos , Fatores de Risco , Esclerodermia Limitada/epidemiologia , Esclerodermia Limitada/mortalidade , Análise de SobrevidaRESUMO
OBJECTIVE: To assess the accuracy of chest radiography (CXR) in predicting pulmonary hypertension (PH). METHODS: We studied 108 consecutive patients with suspected PH who underwent right heart catheterization (RHC). All were PH treatment naives. Hemodynamic criteria included a mean pulmonary artery pressure >25 mmHg at rest, and a mean pulmonary wedge pressure <15 mmHg. Postero-anterior and lateral CXR were obtained shortly before RHC. To avoid a selection bias which could be introduced by examining only patients with suspected PH, we included in the analysis the CXR of 454 additional patients with different diagnosis: 57 with left heart failure (LHF) and pulmonary venous hypertension at RHC, 197 with chronic obstructive pulmonary disease, and 200 non-obstructed controls. CXR were examined independently by 4 raters, who were blinded to clinical, hemodynamic, and spirometric data. The diagnosis of PH was made if a prominent main pulmonary artery was associated with anyone of: isolated enlargement of right ventricle, right descending pulmonary artery >16 mm in diameter, pruning of peripheral pulmonary vessels. RESULTS: Eighty-two patients had PH confirmed at RHC. Weighted sensitivity of CXR was 96.9% (95% confidence interval, 94.9 to 98.2%), and weighted specificity 99.8% (95% confidence interval, 99.6 to 99.9%). By considering the 165 patients who underwent RHC, weighted sensitivity of CXR was unchanged, and weighted specificity decreased to 99.1%. None of the patients with PH were misclassified as having LHF, and vice versa. CONCLUSIONS: CXR is accurate in predicting PH. It may aid clinicians in selecting patients with suspected PH for hemodynamic ascertainment.