This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance ...(6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.
Pulmonary arterial hypertension (PAH) is a rare but well-known cardiovascular condition potentially associated with human immunodeficiency virus (HIV) infection and is currently recognized to be one ...of the most ominous noninfectious HIV complications. Although there is no clear evidence supporting the use of any medication for the treatment of HIV-related PAH, many of the currently available agents have been shown to exert some clinical benefits HIV-PAH patients. To date, no data are available regarding the potential effects of sitaxsentan, a selective endothelin type-A receptor antagonist, in this peculiar patient population. We report the case of a successful switch to sitaxsentan in a HIV-infected patient with PAH initially receiving bosentan who developed a late treatment-related side-effect.
Background:
Renal function is a powerful prognostic variable in patients with heart failure (HF). Hospitalisations for acute HF (AHF) may be associated with further worsening of renal function (WRF).
...Methods and results:
We analysed the clinical significance of WRF in 318 consecutive patients admitted at our institute for AHF. WRF was defined as the occurrence, at any time during the hospitalisation, of both a ≥25% and a ≥0.3 mg/dL increase in serum creatinine (s-Cr) from admission (WRF-Abs-%).
Results:
Patients were followed for 480±363 days. Fifty-three patients (17%) died and 132 (41%) were rehospitalised for HF. WRF-Abs-% occurred in 107 (34%) patients. At multivariable survival analysis, WRF-Abs-% was an independent predictor of death or HF rehospitalisation (adjusted HR, 1.47; 95%CI, 1.13-1.81; p=0.024). The independent predictors of WRF-Abs-%, evaluated using multivariable logistic regression, were history of chronic kidney disease (p=0.002), LV ejection fraction (p=0.012), furosemide daily dose (p=0.03) and NYHA class (p=0.05) on admission.
Conclusion:
WRF is a frequent finding in patients hospitalised for AHF and is associated with a poor prognosis. Severity of HF and daily furosemide dose are the most important predictors of the occurrence of WRF.
Abstract
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.
Abstract
Aims
Primary cardiac tumors are generally benign. In one series of over 12 000 autopsies, only seven cases of malignant primary cardiac tumors were identified, for an incidence of less than ...0.1%. By comparison, metastatic involvement of the heart is over 20 times more common and has been reported in autopsy series in up to one in five patients dying of cancer. Myxoid liposarcoma (MLS) is the second most common subtype of liposarcoma and it usually occurs in deep tissues of the extremity, especially in the calf or thigh. Some tumors have round cell areas that represent histologic progression to high-grade tumours. Round cells, defined as > 5% of the total cells, are associated with higher malignancy and metastatic potential, resulting in an unfavourable outcome in patients affected. Patients with MLS tend to have metastases to extra pulmonary sites, such as abdominal wall, abdominal cavity, retroperitoneum, and bone, even in the absence of pulmonary metastases. Although several authors have reported a high proportion of extra pulmonary metastases of MLS, cardiac metastasis is extremely rare.
Methods and results
We present a case of a young woman who underwent resection of calf liposarcoma with the onset of cardiac metastases after 18 years, with cardiac tamponade as a clinical onset. MRI confirmed the cardiac solid mass already evident on CT scan, located along the free anterior wall of the right ventricle in the mid-basal area; the lesion seemed to have pericardial implantation with the free wall of the right ventricle was not well cleaved by the lesion in the Cine-MRI sequences. However it was always visible in the post-contrast sequences without evident protrusion of the lesion into the cavity or signs of thrombosis. The parietal pericardium was located on the periphery of the lesion and was not recognizable in some points. The lesion consisted of two portions, a lower one in which some components with characteristics of adipose signal were recognizable, while the upper one was more solid and vascularized. There was persistence of inhomogeneous and diffuse late enhancement at the lesion level. PET revealed pathological hyper accumulations of radiopharmaceuticals in the heart lesion along the anterior wall of the right ventricle, with central hypocaptation area, possible expression of necrotic-colliquative phenomena. Other hyper accumulations were found in the mediastinal lymph node in the pre-carenal area. These findings were referable to the presence of glucose hyper metabolic tissue of suspected neoplastic significance. The patient temporarily declined surgical excision, but after one month, due to the worsening of her symptoms, she returned to emergency room: The CT scan showed a volumetric increase in cardiac mass with a vertical diameter of 8 cm and adhering to the right ventricle for 7 cm. Thanks to the possibility of surgery, patient underwent exeresis of the capsulated epicardial mass adhering to the anterior wall of the right ventricle and to the pericardium, with removal of the ventricular wall and reconstruction with a bovine pericardium patch and without postoperative complications.
Conclusions
In MLS the time intervals between the onset of primary disease and cardiac metastasis were reported to be relatively long, ranging from 1 to 25 years. The initial site of metastatic disease in our patient was the heart: she had a solitary cardiac tumour that presented 18 years after the primary surgery in the absence of metastases at other sites. When a cardiac metastasis is found incidentally, it is usually incurable.
Aims
Brain natriuretic peptide (BNP), NT-proBNP and troponins are useful for the assessment of patients with heart failure. Few data exist about their serial changes and their prognostic value in ...patients with acute heart failure (AHF).
Methods and results
NT-proBNP and troponin-T plasma levels were measured at baseline, after 6, 12, 24, 48 h and at discharge in 116 consecutive patients with AHF and no evidence of acute coronary syndrome. NT-proBNP levels were 4421 pg/mL at baseline, declined after 24 h and reached their nadir at 48 h (2703 pg/mL). Troponin-T was detectable in 48% of patients. During a median follow-up of 184 days, 52 patients died or had a non-fatal cardiovascular hospitalisation. At a multivariable analysis including clinical and echo-Doppler variables, NT-proBNP plasma levels at discharge, detectable troponin-T plasma levels, and NYHA class at discharge were the only independent prognostic factors.
Conclusion
In patients with AHF, NT-proBNP levels decline 24 h after the initiation of intravenous therapy and troponin-T is detectable in 48% of cases. NT-proBNP levels at discharge, detectable troponin-T levels, NYHA class and serum sodium have independent prognostic value.
Introduction:
Dyspnoea and peripheral oedema, caused by fluid redistribution to the lungs and/or by fluid overload, are the main causes of hospitalization in patients with heart failure and are ...associated with poor outcomes. Treatment of fluid overload should relieve symptoms and have a neutral or favorable effect on outcomes.
Areas covered:
We first consider the results obtained with furosemide administration, which is still the mainstay of treatment of congestion in patients with heart failure. We then discuss important shortcomings of furosemide treatment, including the development of resistance and side effects (electrolyte abnormalities, neurohormonal activation, worsening renal function), as well as the relationship of furosemide - and its doses - with patient prognosis. Finally, the results obtained with potential alternatives to furosemide treatment, including different modalities of loop diuretic administration, combined diuretic therapy, dopamine, inotropic agents, ultrafiltration, natriuretic peptides, vasopressin and adenosine antagonists, are discussed.
Expert opinion:
Relief of congestion is a major objective of heart failure treatment but therapy remains based on the administration of furosemide, an agent that is often not effective and is associated with poor outcomes. The results of the few controlled studies aimed at the assessment of new treatments to overcome resistance to furosemide and/or to protect the kidney from its untoward effects have been mostly neutral. Better treatment of congestion in heart failure remains a major unmet need.
Anemia is one of the most frequent co-morbidities in the patients with heart failure. Its prevalence increases from 4-7% in the subjects with asymptomatic left ventricular dysfunction to >30% in the ...patients with severe heart failure. Renal insufficiency, activation of inflammatory mediators, and treatment with renin-angiotensin antagonists seem to be its main determinants. The results of many studies agree in showing that anemia is a powerful independent determinant of survival in patients with heart failure. However, the mechanisms of this relation are still incompletely understood. Moreover a favourable effect on prognosis of the correction of anemia has not been shown, yet, and also controlled studies assessing its effects on exercise tolerance have yielded controversial results.
Clinical trials have consistently shown the benefits of beta-blocker treatment in patients with chronic heart failure (HF). As a result, bisoprolol, carvedilol, and metoprolol succinate are now ...indicated for the treatment of all patients with chronic HF who do not have major contraindications. Bisoprolol is the first beta-blocker shown to improve survival in an outcome trial. In the Cardiac Insufficiency Bisoprolol Study II (CIBIS-II), all-cause mortality and sudden death were reduced in patients treated with bisoprolol compared with those on placebo (11.8% vs 17.3%; p < 0.0001 and 3.6% vs 6.3%, p < 0.002; respectively) regardless of age, NYHA functional class, and co-morbidities. Further studies have shown both the efficacy of bisoprolol on secondary endpoints and patients subgroups as well its high cost effectiveness. More recently, CIBIS-III has shown similar efficacy and safety of the initiation of HF treatment with either bisoprolol or enalapril, with a tendency to a survival advantage with bisoprolol. Nowadays, the role of bisoprolol, as well as that of carvedilol and metoprolol succinate, in HF treatment is firmly established and research is mainly focused on implementation of treatment and better dosing. This article will summarize evidence for the efficacy of bisoprolol in the treatment of HF.
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