Impacto del cateterismo derecho de ejercicio en el diagnóstico diferencial de la enfermedad tromboembólica pulmonar crónica / Value of exercise right heart catheterization in the differential diagnosis of chronic thromboembolic pulmonary disease

Introduction and objectives Chronic thromboembolic disease refers to the presence of chronic thrombotic pulmonary vascular thrombosis without pulmonary hypertension (PH) at rest but with exercise limitation after pulmonary embolism (PE). Our aim was to evaluate the hemodynamic response to exercise in these patients and its correlation with the values reached in cardiopulmonary exercise testing. Methods We included symptomatic patients with persistent pulmonary thrombosis after PE. We excluded patients with left heart disease or significant PH (mean pulmonary arterial pressure [mPAP] >25mmHg, pulmonary vascular resistance >3 WU, and pulmonary capillary wedge pressure [PCWP] >15mmHg). Cardiopulmonary exercise testing and exercise right heart catheterization were performed. Exercise-induced precapillary PH was defined as mPAP/CO slope >3 and PCWP/CO slope <2mmHg/l/min. The hemodynamic response and the values obtained in cardiopulmonary exercise testing were compared between patients with and without exercise-induced precapillary PH. Results We studied 36 patients; 4 were excluded due to incomplete hemodynamic data. Out of the 32 patients analyzed; 3 developed a pathological increase in PCWP. Among the remaining 29 patients (mean age, 49.4±13.7 years, 34.5% women), 13 showed exercise-induced PH. Resting mPAP was higher in those who developed exercise-induced PH (23.3±5.4 vs 19.0±3.8mmHg; P=.012), although CO was similar in the 2 groups. Patients with exercise-induced PH exhibited data of ventilatory inefficiency with reduced values of end-tidal CO2 pressure at the anaerobic threshold (32.8±3.0 vs 36.2±3.3mmHg; P=.021) and a higher Ve/VCO2 slope (34.2±4.8 vs 30.7±5.0; P=.049). Conclusions Exercise limitation and ventilatory inefficiency could be attributable to exercise-induced precapillary PH in a subgroup of patients with persistent pulmonary thrombosis and dyspnea (AU)

Introdución y objetivos La enfermedad tromboembólica crónica se define como la trombosis crónica de la vasculatura pulmonar y disnea, sin hipertensión pulmonar (HP) en reposo tras una embolia de pulmón. El estudio evaluó la hemodinámica al esfuerzo en estos pacientes y su relación con la ergoespirometría. Métodos Se incluyó a pacientes sintomáticos con trombosis pulmonar crónica tras una embolia de pulmón. Se excluyó a los pacientes con cardiopatía izquierda o HP significativa en reposo (presión arterial pulmonar media [PAPm] >25mmHg, resistencia vascular pulmonar >3 UW y presión capilar pulmonar [PCP] >15mmHg). Se realizó una ergoespirometría y un cateterismo derecho de ejercicio. La HP precapilar al ejercicio se definió como las pendientes PAPm/gasto cardiaco >3 y PCP/gasto cardiaco <2mmHg/l/min. Se comparó la respuesta hemodinámica y ergoespirométrica entre pacientes con y sin HP precapilar al ejercicio. Resultados Se estudió a 36 pacientes, excluyéndose 4 por calidad subóptima en el registro hemodinámico. Tres pacientes presentaron una elevación patológica de la PCP. De los 29 restantes (edad, 49,4±13,7 años; el 34,5% mujeres), en 13 se halló HP precapilar al ejercicio. La PAPm basal fue mayor en aquellos con HP al ejercicio (23,3±5,4 frente a 19,0±3,8mmHg; p=0,012), con similar gasto cardiaco. Aquellos con HP al ejercicio exhibieron valores reducidos de la presión de CO2 exahalada en el umbral anaeróbico (32,8±3,0 frente a 36,2±3,3mmHg; p=0,021) y mayor pendiente Ve/VCO2 (34,2±4,8 frente a 30,7±5,0; p=0,049). Conclusiones La HP precapilar al ejercicio se asocia con limitación funcional e ineficiencia ventilatoria en un subgrupo de pacientes con trombosis arterial pulmonar crónica y disnea persistente (AU)

Value of exercise right heart catheterization in the differential diagnosis of chronic thromboembolic pulmonary disease.

INTRODUCTION AND OBJECTIVES: Chronic thromboembolic disease refers to the presence of chronic thrombotic pulmonary vascular thrombosis without pulmonary hypertension (PH) at rest but with exercise limitation after pulmonary embolism (PE). Our aim was to evaluate the hemodynamic response to exercise in these patients and its correlation with the values reached in cardiopulmonary exercise testing. METHODS: We included symptomatic patients with persistent pulmonary thrombosis after PE. We excluded patients with left heart disease or significant PH (mean pulmonary arterial pressure [mPAP] >25mmHg, pulmonary vascular resistance >3 WU, and pulmonary capillary wedge pressure [PCWP] >15mmHg). Cardiopulmonary exercise testing and exercise right heart catheterization were performed. Exercise-induced precapillary PH was defined as mPAP/CO slope >3 and PCWP/CO slope <2mmHg/l/min. The hemodynamic response and the values obtained in cardiopulmonary exercise testing were compared between patients with and without exercise-induced precapillary PH. RESULTS: We studied 36 patients; 4 were excluded due to incomplete hemodynamic data. Out of the 32 patients analyzed; 3 developed a pathological increase in PCWP. Among the remaining 29 patients (mean age, 49.4±13.7 years, 34.5% women), 13 showed exercise-induced PH. Resting mPAP was higher in those who developed exercise-induced PH (23.3±5.4 vs 19.0±3.8mmHg; P=.012), although CO was similar in the 2 groups. Patients with exercise-induced PH exhibited data of ventilatory inefficiency with reduced values of end-tidal CO2 pressure at the anaerobic threshold (32.8±3.0 vs 36.2±3.3mmHg; P=.021) and a higher Ve/VCO2 slope (34.2±4.8 vs 30.7±5.0; P=.049). CONCLUSIONS: Exercise limitation and ventilatory inefficiency could be attributable to exercise-induced precapillary PH in a subgroup of patients with persistent pulmonary thrombosis and dyspnea.

Robustness of [18F]FDG PET/CT radiomic analysis in the setting of drug-induced cardiotoxicity.

BACKGROUND AND OBJECTIVES: Standardization of radiomic data acquisition protocols is still at a very early stage, revealing a strong need to work towards the definition of uniform image processing methodologies The aim of this study is to identify sources of variability in radiomic data derived from image discretization and resampling methodologies prior to image feature extraction. Furthermore, to identify robust potential image-based biomarkers for the early detection of cardiotoxicity. METHODS: Image post-acquisition processing, interpolation, and volume of interest (VOI) segmentation were performed. Four experiments were conducted to assess the reliability in terms of the intraclass correlation coefficient (ICC) of the radiomic features and the effects of the variation of voxel size and gray level discretization. Statistical analysis was performed separating the patients according to cardiotoxicity diagnosis. Differences of texture features were studied with Mann-Whitney U test. P-values <0.05 after multiple testing correction were considered statistically significant. Additionally, a non-supervised k-Means clustering algorithm was evaluated. RESULTS: The effect of the variation in the voxel size demonstrated a non-dependency relationship with the values of the radiomic features, regardless of the chosen discretization method. The median ICC values were 0.306 and 0.872 for absolute agreement and consistency, respectively, when varying the discretization bin number. The median ICC values were 0.678 and 0.878 for absolute agreement and consistency, respectively, when varying the discretization bin size. A total of 16 first order, 6 Gray Level Co-occurrence Matrix (GLCM), 4 Gray Level Dependence Matrix (GLDM) and 4 Gray Level Run Length Matrix (GLRLM) features demonstrated statistically significant differences between the diagnosis groups for interim scans (P<0.05) for the fixed bin size (FBS) discretization methodology. However, no statistically significant differences between diagnostic groups were found for the fixed bin number (FBN) discretization methodology. Two clusters based on the radiomic features were identified. CONCLUSIONS: Gray level discretization has a major impact on the repeatability of the radiomic features. The selection of the optimal processing methodology has led to the identification of texture-based patterns for the differentiation of early cardiac damage profiles.

Evolución y pronóstico de la insuficiencia tricuspídea moderada-grave tras tromboendarterectomía pulmonar o angioplastia de arterias pulmonares / Outcome of moderate-severe tricuspid regurgitation after pulmonary endarterectomy or balloon pulmonary angioplasty

Introducción y objetivos: No está definido el abordaje de la insuficiencia tricuspídea (IT) funcional moderada-grave en los pacientes con hipertensión pulmonar tromboembólica crónica tras la tromboendarterectomía pulmonar (TEA) o angioplastia con balón de las arterias pulmonares (ABAP). El objetivo de este estudio es analizar la evolución y los predictores de IT residual tras el procedimiento, así como su impacto pronóstico. Métodos: Estudio observacional unicéntrico. Se incluyó a 72 pacientes sometidos a TEA y 20 que completaron el programa de ABAP con diagnóstico de hipertensión pulmonar tromboembólica crónica y presentaban IT moderada-grave antes del procedimiento intervencionista. Resultados: La prevalencia de IT moderada-grave tras el procedimiento fue del 29%, sin diferencias entre los tratados con TEA o ABAP (el 30,6 frente al 25%; p=0,78). En el grupo con IT persistente se hallaron mayores presión arterial pulmonar media (40,2± 1,9 frente a 28,5±1,3mmHg; p<0,001), resistencia vascular pulmonar (472 [347-710] frente a 282 [196-408] dyn·s/cm5; p <0,001) y área de la aurícula derecha (23,0 [21-31] frente a 16,0 [14,0-20,0]; p <0,001) tras el procedimiento comparado con el de pacientes con IT ausente-ligera. La resistencia vascular pulmonar> 400dyn.s/cm5 y el área de la aurícula derecha> 22 cm2 tras el procedimiento se asociaron de manera independiente con la persistencia de la IT, pero no se identificaron predictores antes de la intervención. La IT moderada-grave residual y la presión pulmonar media> 30mmHg se asociaron con mayor mortalidad en 3 años de seguimiento. Conclusiones: La IT moderada-grave residual posterior a TEA o ABAP se asoció con la persistencia de una mayor poscarga y un persistente remodelado desfavorable de las cámaras cardiacas derechas tras el procedimiento. La IT moderada-grave y la hipertensión pulmonar residual se asociaron con un peor pronóstico a 3 años. (AU)

Introduction and objectives: The management of persistent moderate-severe tricuspid regurgitation (TR) in patients with chronic thromboembolic pulmonary hypertension after treatment with pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA) is not well defined. This study aimed to analyze the progression and predictors of significant persistent postintervention TR and its prognostic impact. Methods: This single-center observational study included 72 patients undergoing PEA and 20 who completed a BPA program with a previous diagnosis of chronic thromboembolic pulmonary hypertension and moderate-to-severe TR. Results: The postintervention prevalence of moderate-to-severe TR was 29%, with no difference between the PEA- or BPA-treated groups (30.6% vs 25% P=.78). Compared with patients with absent-mild postprocedure TR, those with persistent TR had higher mean pulmonary arterial pressure (40.2±1.9 vs 28.5±1.3mmHg P <.001), pulmonary vascular resistance (472 [347-710] vs 282 [196-408] dyn.s/cm5; P <.001), and right atrial area (23.0 [21-31] vs 16.0 [14.0-20.0] P <.001). The variables independently associated with persistent TR were pulmonary vascular resistance> 400 dyn.s/cm5 and postprocedure right atrial area> 22cm2. No preintervention predictors were identified. The variables associated with increased 3-year mortality were residual TR and mean pulmonary arterial pressure> 30mmHg. Conclusions: Residual moderate-to-severe TR following PEA-PBA was associated with persistently high afterload and unfavorable postintervention right chamber remodeling. Moderate-to-severe TR and residual pulmonary hypertension were associated with a worse 3-year prognosis. (AU)

Outcome of moderate-severe tricuspid regurgitation after pulmonary endarterectomy or balloon pulmonary angioplasty.

INTRODUCTION AND OBJECTIVES: The management of persistent moderate-severe tricuspid regurgitation (TR) in patients with chronic thromboembolic pulmonary hypertension after treatment with pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA) is not well defined. This study aimed to analyze the progression and predictors of significant persistent postintervention TR and its prognostic impact. METHODS: This single-center observational study included 72 patients undergoing PEA and 20 who completed a BPA program with a previous diagnosis of chronic thromboembolic pulmonary hypertension and moderate-to-severe TR. RESULTS: The postintervention prevalence of moderate-to-severe TR was 29%, with no difference between the PEA- or BPA-treated groups (30.6% vs 25% P=.78). Compared with patients with absent-mild postprocedure TR, those with persistent TR had higher mean pulmonary arterial pressure (40.2±1.9 vs 28.5±1.3mmHg P <.001), pulmonary vascular resistance (472 [347-710] vs 282 [196-408] dyn.s/cm5; P <.001), and right atrial area (23.0 [21-31] vs 16.0 [14.0-20.0] P <.001). The variables independently associated with persistent TR were pulmonary vascular resistance> 400 dyn.s/cm5 and postprocedure right atrial area> 22cm2. No preintervention predictors were identified. The variables associated with increased 3-year mortality were residual TR and mean pulmonary arterial pressure> 30mmHg. CONCLUSIONS: Residual moderate-to-severe TR following PEA-PBA was associated with persistently high afterload and unfavorable postintervention right chamber remodeling. Moderate-to-severe TR and residual pulmonary hypertension were associated with a worse 3-year prognosis.

Electrocardiogram Changes in the Spectrum of TTNtv Dilated Cardiomyopathy: Accuracy and Predictive Value of a New Index for LV-Changes Identification.

BACKGROUND: Truncating TTN variants (TTNtv) are the main cause of dilated cardiomyopathy (DCM). The dynamic nature of this entity has previously been described. Based on own empirical observations and previous evidences, this study assessed repolarisation patterns and the possible association with morphological and functional status of TTNtv-DCM patients. METHODS: Electrocardiograms (ECGs) of index patients with TTNtv-DCM and their relatives were included and matched in time with an echocardiogram. All individuals were classified into five phenotype groups: 1) Reduced left ventricular ejection fraction (LVEF <50%); 2) Recovered LVEF: at least 10% increase and LVEF >30% after optimal medical treatment; 3) Borderline phenotype (mildly enlarged ventricle and/or hyper-trabeculation); 4) Genotype positive, phenotype negative; and 5) Non-carriers. All electrocardiograms were evaluated by two blinded observers in qualitative and quantitative terms [T index (mm)=Σ T-wave amplitude (V5, V6, II, aVF)] and these data were compared with demographic and clinical information. The Δ T-index was calculated in those individuals with more than one electrocardiogram. RESULTS: Seventy-eight (78) electrocardiograms were included (46% female, mean age 50 years). T-index and prevalence of an abnormal T-wave had significantly different results among the groups (p<0.0001). Age and haemodynamic factors were shown to be ECG-modifiers, especially in phenotype-negative patients. T-index enabled individuals with reduced LVEF (<2.5) to be identified and to differentiate patients with favourable and unfavourable responses to treatment (Δ T index >3.5 and ≤2, respectively). CONCLUSION: Repolarisation changes enabled characterisation of the spectrum of TTNtv-DCM. The T-index identified potential carriers and patients with the worst profiles of the spectrum of the disease.

Un «corazón» en el corazón: agenesia parcial del pericardio / A "heart" within a heart: partial absence of the pericardium

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Unexpected Favourable Course of Coronavirus Disease 2019 in Chronic Thromboembolic Pulmonary Hypertension Patients / Curso favorable inesperado de la enfermedad por coronavirus 2019 en pacientes con hipertensión pulmonar tromboembólica crónica

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Effect of Coronavirus Disease 2019 in Pulmonary Circulation. The Particular Scenario of Precapillary Pulmonary Hypertension.

The Coronavirus Disease of 2019 (COVID-19) has supposed a global health emergency affecting millions of people, with particular severity in the elderly and patients with previous comorbidities, especially those with cardiovascular disease. Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) could represent an especially vulnerable population because of the high mortality rates reported for respiratory infections. However, the number of COVID-19 cases reported among PAH and CTEPH patients is surprisingly low. Furthermore, the clinical picture that has been described in these patients is far from the severity that experts would expect. Endothelial dysfunction is a common feature between patients with PAH/CTEPH and COVID-19, leading to ventilation/perfusion mismatch, vasoconstriction, thrombosis and inflammation. In this picture, the angiotensin-converting enzyme 2 plays an essential role, being directly involved in the pathophysiology of both clinical entities. Some of these common characteristics could explain the good adaptation of PAH and CTEPH patients to COVID-19, who could also have obtained a benefit from the disease's specific treatments (anticoagulant and pulmonary vasodilators), probably due to its protective effect on the endothelium. Additionally, these common features could also lead to PAH/CTEPH as a potential sequelae of COVID-19. Throughout this comprehensive review, we describe the similarities and differences between both conditions and the possible pathophysiological and therapeutic-based mechanisms leading to the low incidence and severity of COVID-19 reported in PAH/CTEPH patients to date. Nevertheless, international registries should look carefully into this population for better understanding and management.

Influence of long-standing pulmonary arterial hypertension and its severity on pulmonary artery aneurysm development.

Pulmonary artery aneurysm (PAA) is a common finding in patients with long-term pulmonary arterial hypertension (PAH). The influence of PAH severity in the development of PAA remains unclear. We sought to determine whether PAA development is related to PAH severity and whether treatment optimization based on risk profile estimation is effective to stop pulmonary artery (PA) enlargement. This is a retrospective study of 125 PAH patients who underwent an imaging test (computed tomography or magnetic resonance) combined with a right heart catheterization within a six-month period. A multivariate analysis was performed to identify independent risk factors for PAA. Patients who underwent an additional imaging-test and RHC during follow-up were analyzed to evaluate changes on PA dimensions. PAA was diagnosed in 42 (34%) patients. PAA was more frequent in patients with congenital heart disease and toxic oil syndrome. PAH time-course showed to be an independent risk factor for PAA (HR 1.051, 95% CI 1.013-1.091, p = 0.008) whereas PAH severity did not. Twenty-six patients underwent a follow-up imaging-test and catheterization. After treatment optimization, a non-significant reduction of mean PA pressure was observed (58.5 mmHg [43.5-70.8] vs. 55.5 mmHg [47.5-66.3], p = 0.115) and a higher proportion of patients achieved a low-risk profile (19% vs. 35%, p = 0.157). However, the PA diameter significantly increased (40.4 ± 10.1 mm vs. 42.1 ± 9.6 mm; p = 0.003). PAA is a common condition in long-standing PAH but its development is not necessarily related to PAH severity. Despite stabilization after treatment optimization, a progressive PA dilatation was observed.