Simplified risk stratification based on cardiopulmonary exercise test: A Spanish two-center experience.

A simplified 4-strata risk stratification approach based on three variables is widespread in pulmonary arterial hypertension (PAH) at follow-up. This study aimed to assess the impact of replacing the 6-min walk test (6MWT) with the peak 02 uptake evaluated by the cardiopulmonary exercise test (CPET) on risk stratification by this scale. We included 180 prevalent patients with PAH from two reference hospitals in Spain, followed up between 2006 and 2022. Patients were included if all the variables of interest were available within a 3-month period on the Spanish Registry of Pulmonary Arterial Hypertension (REHAP): functional class (FC); NT-proBNP; 6MWT; and CPET. The original 4-strata model (NT-proBNP, 6MWT, FC) identified most patients at low or intermediate-low risk (36.7% and 51.1%, respectively). Notably, the modified scale (NT-proBNP, CPET, FC) improved the identification of patients at intermediate-high risk up to 18.9%, and at high risk up to 1.1% in comparison with the previous 12.2% and 0.0% in the original scale. This new model increased the number of patients correctly classified into higher-risk strata (positive NRI of 0.06), as well as classified more patients without events in lower-risk strata (negative NRI of 0.04). The proposed score showed a slightly superior prognostic capacity compared with the original model (Harrel's C-index 0.717 vs. 0.709). Using O2 uptake instead of distance walked in the 6MWT improves the identification of high-risk patients using the 4-strata scale. This change could have relevant prognostic implications and lead to changes in the specific treatment of PAH.

Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease.

OBJECTIVE: This study assessed the long-term effects of triple therapy with prostanoids on patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), as there is limited information on the safety and efficacy of this treatment approach. METHODS: A retrospective cohort study was conducted on patients with PAH-CHD who were actively followed up at our centre. All patients were already receiving dual combination therapy at maximum doses. Clinical characteristics, including functional class (FC), 6-minute walking test distance (6MWTD) and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, were documented before initiating triple therapy and annually for a 2-year follow-up period. RESULTS: A total of 60 patients were included in the study, with a median age of 41 years and 68% being women. Of these, 32 had Eisenmenger syndrome, 9 had coincidental shunts, 18 had postoperative PAH and 1 had a significant left-to-right shunt. After 1 year of triple combination initiation, a significant improvement in 6MWTD was observed (406 vs 450; p=0.0027), which was maintained at the 2-year follow-up. FC improved in 79% of patients at 1 year and remained stable in 76% at 2 years. NT-proBNP levels decreased significantly by 2 years, with an average reduction of 199 ng/L. Side effects were experienced by 33.3% of patients but were mostly mild and manageable. Subgroup analysis showed greater benefits in patients without Eisenmenger syndrome and those with pre-tricuspid defects. CONCLUSIONS: Triple therapy with prostanoids is safe and effective for patients with PAH-CHD, improving FC, 6MWTD and NT-proBNP levels over 2 years. The treatment is particularly beneficial for patients with pre-tricuspid defects and non-Eisenmenger PAH-CHD.

Frailty Is Common in Heart Transplant Candidates But Is Not Associated With Clinical Events and Is Reversible After Heart Transplantation.

Assessment of frailty before heart transplant (HT) is recommended but is not standard in most HT protocols. Our objective was to evaluate frailty at inclusion in HT list and during follow-up and to assess the influence of baseline frailty on prognosis. A prospective multicenter study in all adults included in the nonurgent HT waiting list. Frailty was defined as Fried's frailty phenotype score ≥3. Mean follow-up was 25.9 ± 1.2 months. Of 99 patients (mean age 54.8 [43.1 to 62.5] years, 70 men [70.7%]), 28 were frail (28.3%). A total of 85 patients received HT after 0.5 ± 0.01 years. Waiting time was shorter in frail patients (0.6 years [0.3 to 0.8] vs 0.2 years [0.1 to 0.4], p = 0.001) because of an increase in priority. Baseline frailty was not associated with overall mortality, (hazard ratio 0.99 [95% confidence interval 0.41 to 2.37, p = 0.98]). A total of 16 transplant recipients died (18.8%). Of the remaining 69 HT recipients, 65 underwent frailty evaluation during follow-up. Patients without baseline frailty (n = 49) did not develop it after HT. Of 16 patients with baseline frailty, only 2 were still frail at the end of follow-up. Frailty is common in HT candidates but is reversible in most cases after HT and is not associated with post-transplant mortality. Our results suggest that frailty should not be considered an exclusion criterion for HT.

From Double-Chambered Right Ventricle to Double-Chambered Left Ventricle: Unusual Evolution of a Ventricular Septal Defect.

We present the first imaging registry of the progressive isolation of an apical chamber of the right ventricle caused by the hypertrophy of the moderator band generated from the hemodynamic effect of a ventricular septal defect, leaving the apex of the right ventricle as an accessory chamber of the left ventricle. (Level of Difficulty: Advanced.).

Hipertensión arterial pulmonar / Pulmonary arterial hypertension

La hipertensión arterial pulmonar (HAP) es una enfermedad rara caracterizada por un remodelado adverso del árbol arterial que conlleva un aumento de las resistencias vasculares con el subsecuente incremento de la poscarga del ventrículo derecho y el desarrollo, finalmente, de insuficiencia cardiaca. El curso de la enfermedad conlleva un mal pronóstico, al que se asocia el retraso en el diagnóstico e inicio de tratamiento motivado por la clínica inespecífica y el desconocimiento de la entidad. Las recomendaciones más recientes se centran en optimizar el diagnóstico precoz diferencial frente a otras causas de hipertensión pulmonar, con el fin de iniciar un tratamiento adecuado utilizando como referencia la estimación de riesgo de mortalidad del paciente. En los últimos años, con la mejora en el proceso diagnóstico, la aparición de nuevos tratamientos específicos y la creación de unidades de referencia especializadas en esta enfermedad, se ha conseguido mejorar el pronóstico y la calidad de vida de los pacientes con HAP. (AU)

Pulmonary arterial hypertension (PAH) is a rare disease characterized by adverse remodeling of the arterial tree leading to increased vascular resistance with subsequent increase in right ventricular afterload and eventual development of heart failure. The nonspecific clinical manifestations and lack of knowledge of pathology lead to a poor prognosis associated with delay in diagnosis and initiation of treatment. The most recent recommendations focus on optimizing the early differential diagnosis with other causes of pulmonary hypertension to initiate appropriate treatment based on the mortality risk estimation. In the last years, with the improvement in the diagnostic process, the emergence of new specific treatments, and the creation of specialized referral units for this pathology, the prognosis, and quality of life of patients with PAH have improved significantly. (AU)

The role of cardiopulmonary exercise testing in identifying and monitoring pulmonary veno-oclusive disease: a case report with ING.

Background: Pulmonary veno-occlusive disease (PVOD) is a subgroup of pulmonary arterial hypertension (PAH) where vascular remodelling affects mainly the post-capillary vessels. It is characterized by a particularly worse prognosis and by the risk of developing life-threatening pulmonary oedema, especially after PAH-targeted therapy. Therefore, suspicion of PVOD is crucial to guide the patient's management. In the absence of specific genetic or histological findings, diagnosis has traditionally relied on the recognition of non-invasive indicators associated with a high likelihood of PVOD. The cardiopulmonary exercise testing (CPET) arises as a promising additional tool both to identify these patients and to guide their management. Case summary: We report the case of a young female patient with dyspnoea and clinical suspicion of PVOD. The diagnostic workup is thoroughly described stressing the valuable and readily accessible information that CPET can provide, in addition to the data of radiological and lung function tests. Once diagnosed, she was started on PAH-targeted therapy with subsequent improvement. The patient underwent a complete reassessment with satisfactory findings, including those of the CPET. Discussion: Pulmonary veno-occlusive disease diagnosis is still one of the most difficult tasks that pulmonary hypertension physicians have to deal with. An accurate and timely PVOD diagnosis can be challenging, as it is to decide the most appropriate timing of referal to the lung transplant team, and CPET may serve these purposes. Through this case, we would like to review one of the typical clinical courses that PVOD may present and how to analyse the information provided by the diagnostic tests.

Pulmonary arterial hypertension. / Hipertensión arterial pulmonar.

Pulmonary arterial hypertension (PAH) is a rare disease characterized by adverse remodeling of the arterial tree leading to increased vascular resistance with subsequent increase in right ventricular afterload and eventual development of heart failure. The nonspecific clinical manifestations and lack of knowledge of pathology lead to a poor prognosis associated with delay in diagnosis and initiation of treatment. The most recent recommendations focus on optimizing the early differential diagnosis with other causes of pulmonary hypertension to initiate appropriate treatment based on the mortality risk estimation. In the last years, with the improvement in the diagnostic process, the emergence of new specific treatments, and the creation of specialized referral units for this pathology, the prognosis, and quality of life of patients with PAH have improved significantly.

Prevalence and characterization of frailty, depression, and cognitive impairment in patients listed for heart transplantation: Results of the FELICITAR prospective registry.

INTRODUCTION: It is recommended to assess frailty prior to heart transplantation (HT). Our objective was to assess the prevalence of frailty in patients listed for HT. METHODS: The FELICITAR registry (Frailty Evaluation after List Inclusion, Characteristics and Influence on TrAnsplantation And Results) is a prospective registry that includes patients listed for HT in three centers, from January 2017 to April 2019. We assessed the presence of frailty, depression, cognitive impairment, and quality of life when included. RESULTS: Ninety-nine patients were included. Of this group, 30.6% were frail, 55 (56.1%) had depression (treated only in nine patients), and 51 (54.8%) had cognitive impairment. Compared with non-frail patients, frail patients were more frequently hospitalized when included in HT waiting list (P = .048), had a lower upper-arm circumference (P = .026), had a lower Barthel index (P = .001), more anemia (P = .010), higher rates of depression (P = .001), poorer quality of life (P = .001), and lower hand-grip strength (P < .001). In multivariate analysis hand-grip strength (odds ratio .91; 95% confidence interval .87-.96, P < .001) and Barthel index (odds ratio .90; 95% confidence interval .82-.99, P = .024) were associated with frailty. CONCLUSIONS: Frailty, depression, and cognitive impairment are common in patients included in HT waiting list. Frailty is strongly associated with hand-grip strength.

Respuesta al ECG de enero de 2020 / Response to ECG, January 2020

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Bloqueo auriculoventricular permanente tras test de flecainida / Permanent atrioventricular block after flecainide testing

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ECG de enero de 2020 / ECG, January 2020

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Uso del oxigenador extracorpóreo de membrana venoarterial en pacientes con tromboembolia pulmonar de alto riesgo / Use of extracorporeal membrane oxygenator in massive pulmonary embolism

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