Acute, subacute and chronic mountain sickness. / Mal de montaña de tipo agudo, subagudo y crónico.

More than 100 million people ascend to high mountainous areas worldwide every year. At nonextreme altitudes (<5500 m), 10-85% of these individuals are affected by acute mountain sickness, the most common disease induced by mild-moderate hypobaric hypoxia. Approximately 140 million individuals live permanently at heights of 2500-5500 m, and up to 10% of them are affected by the subacute form of mountain sickness (high-altitude pulmonary hypertension) or the chronic form (Monge's disease), the latter of which is especially common in Andean ethnicities. This review presents the most relevant general concepts of these 3 clinical variants, which can be incapacitating and can result in complications and become life-threatening. Proper prevention, diagnosis, treatment and management of these conditions in a hostile environment such as high mountains are therefore essential.

Pulmonary embolism treatment. Detection of chronic thromboembolic pulmonary hypertension. / Tratamiento de la embolia de pulmón. Detección de la hipertensión pulmonar tromboembólica crónica.

The correct stratification of pulmonary embolism risk (PE) is essential for decision-making, regarding treatment and defining the patient's place of admission. In high-risk PE, urgent re-establishment of pulmonary circulation and admission to a critical unit is required. The reperfusion treatment of choice is systemic thrombolysis, although in certain situations, especially when there is a contraindication for it, we will evaluate a surgical embolectomy or one of the catheter-guided therapies. In the rest of PE, the treatment of choice will be anticoagulation. Currently, direct oral anticoagulants have become the treatment of choice for the treatment of PE, due to their better safety profile. However, low molecular weight heparins and subsequently antivitamins K, remain the most used treatment, because they are funded by the public system. In cases of PE with cardiorespiratory arrest and / or cardiogenic shock, whenever available at our center, we must consider the indication of extracorporeal membrane oxygenation. The recent creation of PE response teams (PERT team), have meant an improvement in the care of patients with intermediate-high and high risk PE. During the follow-up of patients with PE, it is essential to perform a correct screening of chronic thromboembolic pulmonary hypertension, in order to perform a correct diagnostic and therapeutic approach.

[Cost effectiveness and budget impact analysis of inhaled nitric oxide in a neonatal unit from the perspective of the public health system]. / Costo efectividad y análisis de impacto presupuestario del óxido nítrico inhalatorio neonatal en un hospital, desde la perspectiva del sistema público de salud.

Inhaled nitric oxide (iNO) is currently the first-line therapy in severe hypoxaemic respiratory failure of the newborn. Most of regional neonatal centres in Chile do not have this therapeutic alternative. OBJECTIVE: To determine the cost effectiveness of inhaled nitric oxide in the treatment of respiratory failure associated with pulmonary hypertension of the newborn compared to the usual care, including the transfer to a more complex unit. PATIENTS AND METHOD: A clinical decision tree was designed from the perspective of Chilean Public Health Service. Incremental cost effectiveness rates (ICER) were calculated, deterministic sensitivity analysis was performed, and probabilistic budget impact was estimated using: TreeAge Pro Healthcare 2014 software. RESULTS: The iNO option leads to an increase in mean cost of $ 11.7 million Chilean pesos (€15,000) per patient treated, with an ICER compared with the usual care of $23 million pesos (€30,000) in case of death or ECMO avoided. By sensitising the results by incidence, it was found that from 7 cases and upwards treated annually, inhaled nitric oxide is less costly than the transfer to a more complex unit. CONCLUSIONS: From the perspective of a Chilean regional hospital, incorporating inhaled nitric oxide into the management of neonatal respiratory failure is the optimal alternative in most scenarios.

Multidetector computed tomography shows reverse cardiac remodeling after double lung transplantation for pulmonary hypertension. / Remodelado cardíaco inverso en pacientes con hipertensión pulmonar tras trasplante bipulmonar. Estudio con tomografía computarizada multidetector.

OBJECTIVE: To use multidetector computed tomography (MDCT) to evaluate the structural changes in the right heart and pulmonary arteries that occur in patients with severe pulmonary hypertension treated by double lung transplantation. MATERIAL AND METHODS: This was a retrospective study of 21 consecutive patients diagnosed with severe pulmonary hypertension who underwent double lung transplantation at our center between 2010 and 2014. We analyzed the last MDCT study done before lung transplantation and the first MDCT study done after lung transplantation. We recorded the following variables: diameter of the pulmonary artery trunk, ratio of the diameter of the pulmonary artery trunk to the diameter of the ascending aorta, diameter of the right ventricle, ratio of the diameter of the left ventricle to the diameter of the right ventricle, and eccentricity index. Statistical analysis consisted of the comparison of the means of the variables recorded. RESULTS: In all cases analyzed, the MDCT study done a mean of 24±14 days after double lung transplantation showed a significant reduction in the size of the right heart chambers, with improved indices of ventricular interdependency index, and reduction in the size of the pulmonary artery trunk (p<0.001 for all the variables analyzed). CONCLUSION: Patients with pulmonary hypertension treated by double lung transplantation present early reverse remodeling of the changes in the structures of the right heart and pulmonary arterial tree. MDCT is useful for detecting these changes.

[The role of nursing in right heart catheterization. Advantages from peripheral venous access]. / Papel de enfermería en el cateterismo cardiaco derecho. Ventajas del acceso venoso periférico.

INTRODUCTION: Pulmonary hypertension (PH) is a serious disease that requires early diagnosis to achieve a better patient prognosis. Right heart catheterization (RHC) has become the main diagnostic test for this disease, measuring the pressures from the right heart chambers invasively, using a catheter placed through venous access. Nursing performance has an important role in RHC through peripheral venous access due to its well-done skills for canalization and knowledge about the management and care of these accesses. RHC performed through peripheral venous access provide advantages over those performed through central venous access. OBJECTIVE: To analyze the benefits from RHC through peripheral venous access compared to those performed through central venous access, as well as highlighting the role of nursing during this type of procedures. METHOD: A retrospective, descriptive, and observational study was performed for patients who underwent RHC in our center between January 2019 to January 2023. We analyzed clinical characteristics, access, fluoroscopy parameters, periprocedural complications, and hospital admissions. RESULT: A total of 115 patients were included. The average age was 65±12 years, with 58.1% of females. Risk stratification of PH was the reason for conducting RHC in 82.9%. The anterocubital veins became the main approach (72.2%), performed by hemodynamics nurses, while the central venous ones composed the rest (27.8%), which were done by cardiology specialized doctors. We observed a significant reduction in radiation dose in RHC via anterocubital route compared to central venous access (4.4Gycm2 vs 12.5Gycm2 [IQR: 4.5]; P<.001), and it does also in fluoroscopy times (2.3minutes vs 4.6minutes [IQR: 2.6]; P<.001). No complications were recorded, independently of the approach. Patients who underwent a scheduled catheterization were discharged more frequently on the same day of the procedure whether a peripheral approach was performed (77.2%, 44 of 57 patients), in comparison with the central one (28.6%) (P=.001). CONCLUSIONS: The RHC is an essential tool for the diagnosis of PH, achieving nursing such an important role for those performed by peripheral venous access. Peripheral venous access provides benefits and advantages like the reduction of radiation exposure and scan times, reduced hospital stay. All this could bring greater comfort, safety and better quality of care to the patient.

Cardiopulmonary phenotype in systemic sclerosis associated pulmonary hypertension.

INTRODUCTION: Pulmonary hypertension (PH) associated with systemic sclerosis (SSc) increases morbidity and mortality. Cardiopulmonary comorbidities, as per the 2021 PH consensus, play a role in the choice of therapy between monotherapy and combination therapy. METHODS: A cross-sectional study was conducted in patients with SSc based on the 2013 ACR/EULAR criteria or very early disease (VEDOSS 2011). PH was considered if they met the following criteria: pulmonary artery systolic pressure (PASP)>39mmHg or peak tricuspid regurgitation velocity (PTRV)>3.4m/s, PASP between 33 and 39mmHg or PTRV between 2.9 and 3.4m/s plus two additional findings suggestive of PH. PH was classified as type 2 if LVEF<50% or moderate to severe diastolic dysfunction was present; type 3 if extensive interstitial disease on tomography>20% or forced vital capacity (FVC)<75%; type 4 if abnormalities related to embolism were detected on scintigraphy or tomography. If patients did not meet these criteria, they were classified as type 1 PH. Complete data on cardiopulmonary risk factors and other factors were required. The frequency of these factors in the population and differences between groups based on risk factors were estimated. RESULTS: A total of 228 patients were selected. Three had type 2 PH, 24 had type 3, and 40 had type 1 PH, with the majority (75%) having at least one cardiopulmonary risk factor, and 47.5% having more than one. Mild diastolic dysfunction (25%) and hypertension (35%) were the most prevalent. In the type 1 PH group, those with risk factors experienced an increase in the number of years with Raynaud's phenomenon, anticentromere antibodies, and gastrointestinal symptoms (p<0.05). CONCLUSION: In patients with PH, 75% have one, and 45% have two or more risk factors.

[Update in the management of chronic thrombo-embolic pulmonary hypertension]. / Actualización del abordaje de la hipertensión pulmonar tromboembólica crónica.

Chronic thrombo-embolic pulmonary hypertension (CTEPH) is a potentially curable form of pulmonary hypertension (PH) that develops in up to 3% of patients after pulmonary embolism (PE). In these patients, PE does not resolve, leading to organized fibrotic clots, with the development of precapillary PH as a result of the proximal obstruction of the pulmonary arteries. In addition, a distal microvasculopathy may also develop, contributing to the increase of pulmonary vascular resistance. Transthoracic echocardiography is the diagnostic tool that allows to establish the suspicion of PH. Ventilation-perfusion lung scintigraphy is the fundamental tool in the study of patients with suspected CTEPH; if it is normal, virtually rules out the diagnosis. Right heart catheterization is mandatory for the diagnosis of these patients. CTEPH is defined as the existence of symptoms, residual perfusion defects and precapillary PH after a minimum period of three months of anticoagulation. Pulmonary angiography helps determine the extent and surgical accessibility of thromboembolic lesions. CTEPH patients are candidates for long-term anticoagulation. Pulmonary endarterectomy is the treatment of choice, resulting in significant clinical and hemodynamic improvement. About 25% of patients have residual PH post-endarterectomy. Balloon pulmonary angioplasty is an endovascular technique that targets more distal lesions, being potentially useful for patients with inoperable CTEPH or persistent/recurrent PH post-endarterectomy. Both types of patients may also benefit from pharmacological treatment for PH. These three therapies are the cornerstone of CTEPH treatment, which has evolved towards a multimodal approach.

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.

Chronic thromboembolic pulmonary hypertension treatment and sex: Systematic review and meta-analysis.

BACKGROUND AND OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic pulmonary hypertension leading to right heart failure and death. While pulmonary endarterectomy is the treatment of choice, some patients might benefit from medical therapy or balloon pulmonary angioplasty. Sex differences in outcomes of these therapies are not well characterized. MATERIAL AND METHODS: We conducted a systematic review and meta-analysis to investigate sex differences in outcomes of various therapies for CTEPH. We searched MEDLINE, PubMed, Embase, CINAHL and the Cochrane Library databases between January 1, 2010 and April 30, 2021, published in English. We pooled incidence estimates using random-effects meta-analyses. We evaluated heterogeneity using the I2 statistic. We assessed publication bias using Begg's and Egger's tests. This study is registered in PROSPERO, CRD42021268504. RESULTS: A total of 19 studies met the eligibility criteria, but only 3 trials provided separate outcomes for women and men. Two studies evaluated the efficacy of BPA, and one study evaluated the efficacy of riociguat (129 patients). Overall, 57.3% of patients were women and 62.6% were in functional class III. Mean time of follow-up was 55.5 (SD 26.1) weeks. Women showed a significantly better response in cardiac index (mean difference [MD], 0.10L/min/m2; 95% confidence interval [CI], 0.04-0.16; I2=0%; P=0.001). Alternatively, the reduction of pulmonary vascular resistances was significantly higher for men than for women (MD, 161.17dynscm-5; 95% CI, 67.99-254.35; I2=0%; P=0.0007). CONCLUSIONS: Women and men might show different hemodynamic responses to riociguat or BPA for CTEPH.

[The importance of data in Pulmonary Arterial Hypertension: from international registries to Machine Learning]. / La importancia de los datos en la hipertensión arterial pulmonar: de los registros internacionales al machine learning.

Real-world registries have been critical to building the scientific knowledge of rare diseases, including Pulmonary Arterial Hypertension (PAH). In the past 4 decades, a considerable number of registries on this condition have allowed to improve the pathology and its subgroups definition, to advance in the understanding of its pathophysiology, to elaborate prognostic scales and to check the transferability of the results from clinical trials to clinical practice. However, in a moment where a huge amount of data from multiple sources is available, they are not always taken into account by the registries. For that reason, Machine Learning (ML) offer a unique opportunity to manage all these data and, finally, to obtain tools that may help to get an earlier diagnose, to help to deduce the prognosis and, in the end, to advance in Personalized Medicine. Thus, we present a narrative revision with the aims of, in one hand, summing up the aspects in which data extraction is important in rare diseases -focusing on the knowledge gained from PAH real-world registries- and, on the other hand, describing some of the achievements and the potential use of the ML techniques on PAH.

Alveolar capillary dysplasia with misalignment of the pulmonary veins: A surgical lung biopsy and autopsy in a full-term newborn.

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare and lethal interstitial lung disorder, caused by a congenital abnormality affecting the development of the parenchyma and pulmonary vessels. We report the case of a newborn at the end of 40 weeks of pregnancy, who showed no cardiopulmonary anomalies in prenatal control ultrasounds. However, after delivery, pulmonary hypertension and hypoxemic respiratory failure became apparent. She died after 12 days from refractory hemodynamic and respiratory failure despite intensive therapy. A surgical lung biopsy and clinical autopsy were performed, both revealing the same histopathological signs consistent with this disorder. In our case, the findings of digestive and genital malformations, together with the genetic result of the alteration in the FOXF1 gene, led us to conclude the definitive diagnosis of alveolar capillary dysplasia.

Treatment patterns and factors associated with adherence in pulmonary arterial hypertension.

OBJECTIVE: Improving understanding of actual pulmonary hypertension (PH) treatment adherence patterns is crucial to properly treating these patients. We aimed to primarily assess adherence to treatments used for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) specific therapies, identify potential factors related to it and secondly describe its treatment patterns. METHODS: A 6-month observational cross-sectional study in a tertiary care hospital was conducted. Patients with PH-targeted therapy who picked it up in the ambulatory hospital pharmacy and who had been on treatment with the same drug for at least 1 year were included. Adherence was assessed as: 1) Proportion of days covered (PDC); and 2) Simplified Medication Adherence Questionnaire (SMAQ). PDC ≥80% was considered adherent. Statistical analyses were performed to evaluate the study outcomes. Logistic regressions were estimated to identify the association between baseline characteristics and factors associated with adherence. P < 0.05 indicated statistical significance. RESULTS: A total of 63 patients with 127 different treatments were included, 71.4% were females with a mean age (SD) of 59 (15) years. PAH was the most common diagnosis (74.6%). Double therapy was used in 39.7% of patients, being the combination of Macitentan + Tadalafil and Ambrisentan + Tadalafil the most prescribed. Endothelin receptor antagonists were the most used treatment (40.2%). Adherence according to PDC was 93.7%, showing no great differences depending on the targeted drug used, and according to SMAQ 61.9%. The agreement degree of both methods was slight (65.1%; Kappa 0.12). Only female sex (OR: 0.23, 95% CI: 0.06-0.90; p = 0.035) was associated with worse adherence in the SMAQ method but not in the PDC. Adverse events were reported by a 55.6% of participants and the perception of effective treatment was high (95.2%). CONCLUSIONS: Adherence to PH therapy differs depending on the assessment method; PDC showed greater adherence rate than SMAQ. According to SMAQ, female sex may have a negative impact on adherence in this cohort, but PDC revealed no factors influencing it. No notable differences in adherence between treatment types were found and generally patients felt the treatments were effective in controlling their disease.

[Pulmonary hypertension in patients infected with human immunodeficiency virus: current situation]. / Hipertensión arterial pulmonar en pacientes con infección por el virus de la inmunodeficiencia humana: situación actual.

The increase in survival that has been achieved with the new treatments in the era of highly active antiretroviral therapy, has enabled clinicians and researchers to analyze issues that emerge in the long term in patients with HIV infection. Although the majority of cardiovascular complications have been widely described, the pathogenesis of pulmonary arterial hypertension is still poorly understood, and is one of the more complex and feared complications as it worsens the prognosis and quality of life of these patients This article reviews newer aspects related to the aetiology, symptoms, diagnosis and treatment of this disease.

Study of left ventricular systolic dysfunction, left ventricular diastolic dysfunction and pulmonary hypertension in CKD 3b-5ND patients-A single centre cross-sectional study.

INTRODUCTION: Cardiovascular diseases are associated with increased morbidity and mortality among CKD (chronic kidney disease) population. Recent studies have found increasing prevalence of PH (pulmonary hypertension) in CKD population. Present study was done to determine prevalence and predictors of LV (left ventricular) systolic dysfunction, LVDD (left ventricular diastolic dysfunction) and PH in CKD 3b-5ND (non-dialysis) patients. METHODS: A cross sectional observational study was done from Jan/2020 to April/2021. CKD 3b-5ND patients aged ≥15 yrs were included. Transthoracic 2D (2 dimensional) echocardiography was done in all patients. PH was defined as if PASP (pulmonary artery systolic pressure) value above 35mm Hg, LV systolic dysfunction was defined as LVEF (left ventricular ejection fraction)≤50% and LVDD as E/e' ratio >14 respectively. Multivariate logistic regression model was done to determine the predictors. RESULTS: A total of 378 patients were included in the study with 103 in stage 3b, 175 in stage 4 and 100 patients in stage 5ND. Prevalence of PH was 12.2%, LV systolic dysfunction was 15.6% and LVDD was 43.65%. Predictors of PH were duration of CKD, haemoglobin, serum 25-OH vitamin D, serum iPTH (intact parathyroid hormone) and serum albumin. Predictors of LVDD were duration of CKD and presence of arterial hypertension. Predictors of LV systolic dysfunction were eGFR (estimated glomerular filtration rate), duration of CKD, serum albumin and urine protein. CONCLUSION: In our study of 378 CKD 3b-5ND patients prevalence of PH was 12.2%, LV systolic dysfunction was 15.6% and LVDD was 43.65%.

External validation of the InShape II study algorithm for exclusion of chronic thromboembolic pulmonary hypertension in patients with pulmonary thromboembolism.

BACKGROUND AND AIM: The most severe long-term complication of pulmonary embolism (PE) is chronic thromboembolic pulmonary hypertension (CTEPH), and its early diagnosis often requires numerous diagnostic tests. The InShape II study proposes an early screening algorithm that aims to reduce the number of echocardiographic studies. The objective of our study is to validate this algorithm in our patient cohort. MATERIALS AND METHODS: We retrospectively analyzed patients admitted to Hospital Rey Juan Carlos between November 2017 and February 2020, who were diagnosed with PE based on computed tomography angiography (CTA). Patients were followed for at least one year, and clinical, laboratory, and complementary test data were collected at three months and one year. The InShape II algorithm was applied to these patients to validate its results. RESULTS: During the study period, 236 patients were diagnosed with PE, of which 137 were excluded. The algorithm was validated in 99 patients. Applying the InShape II score, 19 echocardiograms would have been performed (three of them with intermediate-high probability of CTEPH), while 80 echocardiograms would have been avoided (two of them with intermediate-high probability). This yielded a sensitivity of 60% and a specificity of 83% for the score, with an area under the curve (AUC) of 0.715 (95% CI: 0.472-0.958). CONCLUSIONS: Our results support the notion that the InShape II algorithm could be a useful tool for initial screening of CTEPH in low-incidence settings, as it would avoid unnecessary echocardiograms that do not provide additional value.

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.

Echocardiographic analysis of pulmonary arterial systolic pressure in pulmonary hypertension.

INTRODUCTION: The reliability of pulmonary arterial systolic pressure by transthoracic echocardiography is limited by its variability to define pulmonary hypertension. OBJECTIVE: To know the variability of pulmonary arterial systolic pressure estimated by echocardiography in pulmonary hypertension. Their demographic variables were obtained. METHODS: From 2016-2020 subjects with pulmonary hypertension were recruited, with pulmonary artery systolic pressure estimated by transthoracic echocardiography and by right heart catheterization. Data were analyzed using the Bland-Altman descriptive statistic and the intraclass correlation coefficient (95% confidence interval). RESULTS: 152 subjects, age 60 ± 12 years, were studied. Body mass index 27.64 ± 4.69 kg/m2. The pulmonary artery systolic pressure estimated by transthoracic echocardiography 58.99 ± 18.62 vs. cardiac catheterization 55.43 ± 16.79 mmHg. Mean difference (bias) -3.6 (29.1, -36.2) and intraclass correlation coefficient 0.717 (0.610, 0.794). CONCLUSIONS: Variability is wide, and agreement is substantial for pulmonary artery systolic pressure. It is recommended to estimate only as screening for pulmonary hypertension.

INTRODUCCIÓN: La confiabilidad de la presión sistólica arterial pulmonar por ecocardiografía transtorácica se encuentra limitada por su variabilidad para definir la hipertensión pulmonar. OBJETIVO: Conocer la variabilidad en la presión sistólica arterial pulmonar estimada por ecocardiografía en la hipertensión pulmonar. MÉTODOS: En el periodo 2016-2020 se captaron sujetos con hipertensión pulmonar que tuvieron estimada la presión sistólica de la arteria pulmonar por ecocardiografía transtorácica y por cateterismo cardiaco derecho. Se obtuvieron sus variables demográficas. Los datos se analizaron con el estadístico descriptivo de Bland-Altman y el coeficiente de correlación intraclase (intervalo de confianza al 95%). RESULTADOS: Se estudiaron 152 sujetos, edad 60 ± 12 años. Índice de masa corporal 27.64 ± 4.69 kg/m2. La presión sistólica de la arteria pulmonar por ecocardiografía transtorácica 58.99 ± 18.62 vs. cateterismo cardiaco 55.43 ± 16.79. Diferencia media (sesgo) ­3.6 (29.1, ­36.2) y coeficiente de correlación intraclase 0.717 (0.610, 0.794). CONCLUSIONES: La variabilidad es amplia y el acuerdo es sustancial con la presión sistólica de la arteria pulmonar. Se aconseja estimarla solo como tamizaje de la hipertensión pulmonar.

Pulmonary artery obstruction index, pulmonary artery diameter and right ventricle strain as prognostic CT findings in patient with acute pulmonary embolism.

OBJECTIVE: This study was designed to determine predictors of pulmonary hypertension and signs of right heart dysfunction caused by pulmonary embolism (PE) that may lead to early detection of high-risk patients. So the predictive value of pulmonary artery obstruction index (PAOI), measured by pulmonary CT angiography (PCTA) in the acute setting, in predicting the patients susceptible to PE cardiac complications was evaluated. Also two other PCTA indices, pulmonary artery diameter (PAD), and right ventricle (RV) strain, in these patients were investigated and their predictive value for cardiac complications on follow up echocardiography were demonstrated. MATERIALS AND METHODS: In the study 120 patients with a definite diagnosis of PE were included. The PAOI, PAD and RV strain were measured using PCTA at the time of the initial diagnosis. Transthoracic echocardiography was done 6 months after the diagnosis of PE and RV echocardiographic indices were measured. Pearson correlation was used to investigate correlation between PAOI, PAD, RV strain and signs of right heart dysfunction. RESULTS: PAOI was strongly correlated with systolic pulmonary artery pressure (SPAP) (r=0.83), RV systolic pressure (r=0.78) and RV wall thickness (r=0.61) in long-term follow up echocardiography. A higher rate of RV dysfunction and RV dilation was detected among the patients with higher PAOI (P<0.001). PAOI≥18 was strongly predictive for development of RV dysfunction. Also developments of pulmonary hypertension, RV systolic hypertension, RV dilation, RV dysfunction, and RV hypertrophy were significantly more common among patients with higher PAD and RV strain (P<0.001). CONCLUSIONS: PAOI, PAD and RV strain are sensitive and specific PCTA indices that can predict the development of long-term complications such as pulmonary hypertension and right heart dysfunction, at the time of initial PE diagnosis.

Fatal Pulmonary Veno-Occlusive Disease and Systemic Juvenile Idiopathic Arthritis: Case Report and Literature Review.

Systemic juvenile idiopathic arthritis (sJIA) is a chronic childhood inflammatory disease. SJIA accounts for approximately 5-15 per cent of all cases of JIA and has a high morbidity and mortality rate. In this disease, pulmonary complications (PC) other than pleuritis are much less frequent and not easily recognised by clinicians. Pulmonary hypertension, the most severe PC, is associated with uncontrolled disease and use of biologic therapies. We present a case of a school-age female with sJIA who died of acute cardiopulmonary instability secondary to pulmonary venous-occlusive disease demonstrated by necropsy. We describe her clinical evolution. We also undertook a narrative review of the literature about PC in sJIA to discuss the current state of the art regarding this complication. High disease activity and the use of multiple therapies include disease-modifying anti-rheumatic drugs should be a red flag for clinicians when discounting PC and pulmonary hypertension. The combination of chest X-ray, electrocardiogram and echocardiogram appear to be the best tests to achieve an early diagnosis.