Aims In patients with chronic advanced heart failure (HF) signs of congestion are not always evident on clinical examination. We aim to validate the ultrasound (US)-assessed jugular vein distensibility (JVD), as a non-invasive tool to identify patients with normal right atrial pressure (RAP≤7 mmHg). Methods and Results In a single-center prospective study, we assessed chronic HF patients with a left ventricular ejection fraction (LVEF)<50% who underwent a pulmonary artery catheterization in the setting of advanced HF therapies workup. We first identify the JVD threshold (Valsalva/rest ratio of the vein diameter) of 1.6 that allowed the most accurate discrimination between patients with RAP≤7 vs. >7 mmHg (area under the curve [AUC] of 0.74; p<0.0001) in a calibration cohort of 100 patients (mean age 53 years, median LVEF 25%). Based on this JVD threshold, we defined patients with low JVD (≤1.6; n=58; median RAP 8 mmHg) and high JVD (>1.6, n=42; median RAP 4 mmHg). Then, we tested the threshold in 101 patients (validation cohort), where we found comparable results (AUC of 0.82; p<0.0001). The JVD threshold had 0.86 and 0.94 predictive positive values to identify patients with RAP≤7 mmHg in the calibration and validation cohorts. Finally, the low JVD vs. high JVD group had a 42.7% vs. 16.1% incidence of major cardiac events at 2 years (log-rank p=0.006), showing its prognostic value. Conclusion US-assessed JVD is an accurate diagnostic tool to identify advanced HF patients with normal RAP. This tool could be tested in the ambulatory setting to modulate diuretic/vasodilator therapies.
Aims Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis, which could potentially affect any organ system. However, there have only been a few reports on cardiac involvement. In fact, it most commonly involves the sinuses, lungs, and kidneys with necrotizing granulomatous vasculitis. In 12% of a large series of patients with GPA there was cardiac involvement, largely manifested by pericarditis and coronary arteritis. Methods and results We describe a rare case of a 23-year-old girl, with no pathological history, at exception of a recent flu-like syndrome for which she carried out the search for SARS-CoV-2 RNA through nasopharyngeal swab, results negative. After a month, she went to the emergency department for a syncopal episode and subsequent head trauma. On this occasion, echocardiogram performed showed the presence of left ventricular systolic dysfunction due to hypokinesia of the middle distal segments; CT angiography of the chest revealed the presence of pulmonary embolism. For this reason, the patient was admitted to the cardiac intensive care unit, where EKG shown anterolateral myocardial infarction with ST elevation and immediately was performed coronary angiography, that evidenced two-vessel disease, with subsequent ineffective attempt to angioplasty. Due to the intercurrent appearance of hyposthenia and paraesthesia in the left upper limb, CT angiography of the brain was performed with detection of lower right pre central frontal hypodensity, suspected for recent ischaemic lesion and hypodensity of the right carotid artery as recent thrombosis. In light of the multi-organ involvement of ischaemic nature and the young age of the patient, rheumatological evaluation was carried out, with execution of a laboratory tests that showed the presence of positivity for ANCA anti-PR3 antibodies, on the basis of which was diagnosed GPA, and rituximab therapy was immediately initiated, with clinical benefit. Conclusions Cardiac involvement of GPA was first reported by Wegener in 1936. Classical or generalized GPA is characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis. Widespread disseminated vasculitis involving both small arteries and veins occurs to a greater or lesser degree as the disease progresses. A localized form of GPA limited primarily to the upper and lower respiratory tracts has been described. Despite histopathological diagnosis of GPA, with autoantibodies against to circulatory neutrophilic cytoplasmic antigens, we can diagnose GPA easily and early. GPA must be kept in mind as the differential diagnosis of new onset cardiomyopathy, especially in the existence of pulmonary and renal pathologies. The clinical presentation of GPA can be so diverse that the list of differential diagnoses is vast, ranging from infections (fungal, bacterial, and mycobacterial) to other vasculitides, including Henoch–Schönlein purpura, sarcoidosis, Behcet syndrome, and malignancies. Despite that involving the heart is well described, significant cardiac complications occurring during the course of the disease are rare.
Aims An increasing number of patients with heart failure (HF) progresses to an advanced stage, characterized by persistent and sever symptoms and worse prognosis. A detailed characterization of patients with advanced HF is needed to optimize clinical management and timely refer for heart transplant or left ventricular assist device implantation. Methods and results A retrospective analysis was performed on patients with HF who were admitted to hospital or performed an outpatient visit at our centre (Spedali Civili di Brescia, Brescia, Italy) from 1 January 2020 to 31 December 2020, and who had at least one of the following high-risk characteristics: (1) previous or ongoing requirement for inotropes; (2) persisting New York Heart Association (NYHA) class III or IV and/or persistently high natriuretic peptides (BNP or NT-proBNP); (3) end-organ dysfunction, defined as worsening renal or liver dysfunction in the setting of HF; (4) ejection fraction (EF) <20%; (5) recurrent appropriate defibrillator shocks; (6) more than 1 hospitalization for HF in the last year; (7) persisting fluid overload and/or increasing diuretic requirement; (8) consistently low blood pressure (systolic blood pressure <90–100 mmHg); and (9) inability to up-titrate or need to decrease/cease HF therapies, such as beta-blockers, angiotensin-converting enzyme inhibitors, angiotensin receptor-neprilysin inhibitors, or mineralocorticoid receptor antagonists. The updated 2018 Heart Failure Association (HFA)—European Society of Cardiology (ESC) criteria for defining advanced HF were evaluated. The primary endpoint was all-cause mortality; secondary endpoints were a composite of all-cause mortality or hospitalization for HF and a composite of all-cause mortality or hospitalization for any reason. Among 493 patients with HF who were hospitalized or performed an outpatient visit in 2020, 230 (46.7%) had at least one high risk criterion and were included in the study. Mean age was 75.5 ± 11.9 years, 156 patients (67.8%) were men, and 160 patients (69.6%) were hospitalized and included as inpatients. Median EF was 38% [interquartile range (IQR): 25–50%] and 117 patients (50.9%) had HF with reduced EF (<40%); median NT-proBNP was 4044 (IQR: 2262–7664) pg/mL. Among the included 230 patients, 38 (16.5%) had all four updated HFA-ESC criteria defining advanced HF, 53 (23.0%) had American College of Cardiology (ACC)/American Heart Association (AHA) stage D, 21 (9.1%) had INTERMACS profile 1–3. In-hospital mortality was 10.6% (among inpatients). After a median follow-up of 301 (214–442) days, a total of 62 patients died (27.0%), and the secondary endpoints of all-cause death or HF hospitalization and all-cause death or any hospitalization were observed in 107 (46.5%) and 139 (60.4%) patients, respectively. Patients fulfilling all four updated HFA-ESC criteria for advanced HF had a higher risk of all-cause mortality (unadjusted HR: 2.06; 95% CI: 1.18–3.60; P = 0.011), also after adjustment for covariates of interest (adjusted HR: 2.20; 95% CI: 1.03, 4.70; P = 0.041). Conclusions In our contemporary, real-world cohort of HF patients with high-risk characteristics, mid-term prognosis was poor, and the use of updated HFA-ESC criteria defining advanced HF identified a subset at increased risk of mortality.
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