Distant embolisation in infective endocarditis: characteristics and outcomes.

BACKGROUND: Infective endocarditis is a severe and potentially lethal cardiac disease. Recognition of the clinical features of endocarditis, such as distant embolisation, and adequate treatment should be initiated promptly given the grim perspective of upcoming virulent pathogens. METHODS: We report on our registry-based experience with outcomes of consecutive patients with infective endocarditis with distant embolisation. We aimed to describe the patient characteristics of infective endocarditis complicated by distant organ embolisation and the safety aspects of continuing endocarditis treatment at home in these patients. RESULTS: From November 2018 through April 2022, 157 consecutive patients were diagnosed with infective endocarditis. Of them, 38 patients (24%) experienced distant embolisation, either in the cerebrum (n = 18), a visceral organ (n = 5), the lungs (n = 7) or the myocardium (n = 8). Pathogens identified in blood cultures were predominantly streptococcal variants (43%), with only one culture-negative endocarditis case. Of the 18 patients with cerebral embolisation, 12 had neurological complaints and most often discrete abnormal findings on neurological examination. Six of the 8 cardiac embolism patients experienced chest pain before admission. Visceral organ and pulmonary embolism occurred silently. Of the 38 patients with distant embolisation, 17 could be discharged earlier by providing antibiotic treatment at home without complications. CONCLUSION: This registry-based single-centre experience showed an incidence of distant embolisation in daily care of 24%. Cerebral and coronary embolisation provoked symptoms, while visceral emboli remained silent. Pulmonary emboli may present with inflammatory signs. Distant embolisation was not in itself a contra-indication for outpatient endocarditis@home treatment.

[Outpatient parenteral antimicrobial therapy for infective endocarditis]. / Thuisbehandeling van infectieuze endocarditis.

Bacterial endocarditis is associated with high morbidity and mortality and requires a long hospitalization due to long-term intravenous antimicrobial therapy. It is possible to partially treat selected and stable patients at home. We present 3 patients partially treated at home with intravenous antibiotics for proven complicated endocarditis. Patient A presented with a septic shock and mitral valve endocarditis. Patient B presented with an ICD lead endocarditis and patient C presented with an mitral valve endocarditis. All 3 patients had a complicated endocarditis and presented with extensive embolic dissemination. Following the initial complicated clinical course, the patients were discharged for antibiotic home treatment after clinical improvement. Subsequent treatment was successful and reduced their hospital stay with more than 14 days. Thanks to transmural cooperation with the home-care colleagues, we can safely provide antibiotic care at home so that stabilized endocarditis patients can be treated in their own habitat.

Too much of a good thing: a case report of traumatic drop attacks and syncope due to orthostatic hypertension.

BACKGROUND: Orthostatic hypertension (OHT) is the clinical opposite to orthostatic hypotension and is an under-recognized and poorly understood clinical phenomenon. Patients may experience disabling symptoms such as dizziness, chest pain, and shortness of breath. In addition, OHT is associated with important clinical outcomes such as silent cerebral infarcts and cognitive decline. CASE SUMMARY: We present the case of a 67-year-old female who experienced frequent drop attacks with and without transient loss of consciousness causing various injuries. A range of standard diagnostic procedures did not yield an explanation for her symptoms but head-up tilt (HUT) testing showed OHT and induced most of her symptoms. Upon initiation of doxazosin, an alpha-blocking drug, she was free of symptoms and blood pressure response was normal on the repeat HUT test. DISCUSSION: To our knowledge, this is the first report of syncope due to OHT. Orthostatic hypertension is a heterogeneous condition and may occur in young, otherwise healthy individuals but also in older patients with cardiovascular comorbidities. It is thought that symptoms occur because of excessive venous pooling (causing a drop in cardiac output) or adrenergic hypersensitivity (resulting in cerebral vasoconstriction or acute rise in cardiac afterload). Since our patient had a marked response to an alpha-blocking agent, we think baroreflex hypersensitivity is the most likely cause of her complaints. Though syncope is probably rare, OHT should be regarded as a possible explanation of orthostatic symptoms.

Atherosclerosis in patients with cyanotic congenital heart disease.

BACKGROUND: Cyanotic patients with congenital heart disease (CHD) might be protected against atherosclerosis. METHODS AND RESULTS: Atherosclerotic risk factors and carotid intima - media thickness (IMT) were investigated in adults with cyanotic CHD and in unaffected age- and sex-matched controls. Fifty-four cyanotic patients (30 men, mean age 38, range 19-60 years) and 54 controls were included. Mean transcutaneous saturation of the cyanotic patients was 81+/-6%. Mean carotid IMT adjusted for age was significantly decreased in cyanotic patients compared to controls (0.55+/-0.1 mm vs 0.58+/-0.08 mm: DeltaIMT =0.04 mm [SE 0.015], P=0.01). In cyanotic patients lower total cholesterol levels were observed (4.4+/-1 mmol/L vs 4.9+/-1 mmol/L; P=0.02), as well as lower thrombocyte levels (173+/-81 x 10(9) /L vs 255+/-54 x 10(9) /L; P<0.01), higher bilirubin levels (18.6+/-11 micromol/L vs 12.7+/-6 micromol/L; P<0.01), and lower diastolic and systolic blood pressure (71+/-9 mmHg vs 76+/-9 mmHg, P<0.01; 113+/-14 mmHg vs 124+/-12 mmHg, P<0.01, respectively). CONCLUSIONS: In patients with cyanotic CHD carotid IMT, and hence atherosclerosis disease risk, was decreased. This might be due to a combination of reduced atherosclerotic risk factors such as lower blood pressure, lower total cholesterol levels, higher bilirubin levels and lower thrombocyte levels.

Duration of right ventricular contraction predicts the efficacy of bosentan treatment in patients with pulmonary hypertension.

AIMS: In patients with pulmonary hypertension (PH), elevated endothelin-1 levels are associated with prolonged duration of right ventricular (RV) contraction, which induces leftward ventricular septal bowing with impaired left diastolic filling. We hypothesized that baseline RV contraction duration predicts efficacy of endothelin receptor antagonist, bosentan. METHODS AND RESULTS: Eighteen PH patients (age 57, range 35-79 years, 33% male) received bosentan. Six minute walk distance (6-MWD) and echocardiography were performed at baseline and after 1 year follow-up. After 1 year of treatment, 6-MWD increased (mean 60 +/- 41 m) in 67% of patients (responders). Baseline RV contraction duration was longer in responders, compared with non-responders (612 +/- 66 vs. 514 +/- 23 ms; P < 0.01). A baseline RV contraction duration >550 ms was associated with improved 6-MWD (sensitivity 83%, specificity 83%; P < 0.01). CONCLUSION: An improvement of 6-MWD during bosentan treatment was associated with a decrease in RV contraction duration and could be predicted by a baseline RV contraction duration >550 ms.

Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist?

BACKGROUND: Data on long-term response to bosentan in adults and especially children with pulmonary arterial hypertension (PAH) associated with systemic-to-pulmonary shunt are scarce. METHODS: We studied bosentan efficacy in 30 patients (20 adults, 10 children) with the disease at short- (4 months), and long-term follow-up (through 2.7 years). World Health Organization functional class (WHO class), transcutaneous oxygen saturation, and 6-minute walk distance were assessed at baseline, 4 months, 1 year, 1.5 years, and at latest follow-up (median 2.7 years). RESULTS: At baseline, children tended to have more severe disease compared with adults with regard to WHO class and congenital heart defects. At 4 months' follow-up, WHO class and 6-minute walk distance significantly improved in both adults and children. During long-term follow-up, this improvement persisted through 1 year but declined thereafter in the total group. In the children, a progressive decline in exercise capacity was observed from 1-year follow-up, whereas in the adults, improvement lasted longer. No change from baseline was seen in transcutaneous oxygen saturation. Three (10%) patients died, 2 (7%) discontinued bosentan, and 5 (17%) required additional PAH therapy (of whom 1 eventually died). One- and 2-year persistence of beneficial bosentan effect was 68% and 43% (total group), 78% and 57% (adults), and 50% and 20% (children), respectively. CONCLUSIONS: Our experience with bosentan suggests short-term improvement in both adults and children with PAH associated with systemic-to-pulmonary shunt. At long-term follow-up, a progressive decline in beneficial bosentan effect was observed. The decline appeared most pronounced in the pediatric patients, who, in this study, tended to have more severe disease at baseline.

Down patients with Eisenmenger syndrome: is bosentan treatment an option?

BACKGROUND: Favorable results of treatment with bosentan in patients with Eisenmenger syndrome are available. However, data in Down patients are lacking. In this study, we evaluate the therapeutic role of bosentan treatment in Down patients with Eisenmenger syndrome. METHODS: In this open-label study, 24 Down patients (>18 years) with Eisenmenger syndrome (17 males) were treated with bosentan. Their mean age was 38 years (range 19-55 years). All Down patients were evaluated at baseline and during follow-up with laboratory tests, six-minute walk test (6-MWT), Doppler echocardiography, and quality of life questionnaires. RESULTS: The median follow-up of Down patients treated with bosentan was 11.5 months (range 3-23 months). Induction of oral bosentan therapy was well tolerated among all 24 Down patients. Bosentan treatment was generally well tolerated. No serious adverse drug reactions were noted. Median 6-MWT increased from 296 m (range 40-424 m) to 325 m (range 84-459 m, p<0.05) after 12 weeks. After 26 and 52 weeks of treatment with bosentan, median 6-MWT distance was 276 m (range 140-462 m, n=15, p=0.6) and 287 m (range 131-409 m, n=7, p=0.3), respectively. Quality of life questionnaire scores remained stable during treatment. CONCLUSION: Also patients with Down syndrome may benefit from bosentan treatment when they have Eisenmenger syndrome. Medical treatment appears to be safe and the treatment effects do not deviate from those observed in Eisenmenger patients without Down syndrome.

Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down's syndrome.

Pulmonary arterial hypertension associated with congenital heart disease caused by systemic-to-pulmonary shunting was associated with a high risk of morbidity and mortality. In this retrospective study, the longer term treatment effect of bosentan on exercise capacity and quality of life (QoL) were evaluated in 58 adult patients (>18 years) with pulmonary arterial hypertension associated with congenital heart disease, including patients with Down's syndrome. All patients were evaluated at baseline and during follow-up using laboratory tests, 6-minute walk test, QoL questionnaires, and Doppler echocardiography. Treatment efficacy was analyzed separately for patients without (n = 30) and with Down's syndrome (n = 28). Median follow-up of all patients treated with bosentan was 22 months (range 3 to 36). In patients without Down's syndrome, mean 6-minute walk distance increased from 427 +/- 97 to 461 +/- 104 m (p <0.01) after 6 months of treatment, followed by a gradual return to baseline and disease stabilization. QoL improved significantly during treatment and was maintained during 18 months of follow-up (p <0.05). In patients with Down's syndrome, 6-minute walk distance and QoL were stable during treatment. In conclusion, findings suggested that in patients without Down's syndrome, longer term bosentan treatment resulted in a persistent improvement in QoL and stabilization of exercise capacity.

Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease.

AIM: To investigate the role of pulmonary arterial hypertension (PAH) in adult patients born with a cardiac septal defect, by assessing its prevalence and its relation with patient characteristics and outcome. METHODS AND RESULTS: From the database of the Euro Heart Survey on adult congenital heart disease (a retrospective cohort study with a 5-year follow-up), the relevant data on all 1877 patients with an atrial septal defect (ASD), a ventricular septal defect (VSD), or a cyanotic defect were analysed. Most patients (83%) attended a specialised centre. There were 896 patients with an ASD (377 closed, 504 open without and 15 with Eisenmenger's syndrome), 710 with a VSD (275, 352 and 83, respectively), 133 with Eisenmenger's syndrome owing to another defect and 138 remaining patients with cyanosis. PAH was present in 531 (28%) patients, or in 34% of patients with an open ASD and 28% of patients with an open VSD, and 12% and 13% of patients with a closed defect, respectively. Mortality was highest in patients with Eisenmenger's syndrome (20.6%). In case of an open defect, PAH entailed an eightfold increased probability of functional limitations (New York Heart Association class >1), with a further sixfold increase when Eisenmenger's syndrome was present. Also, in patients with persisting PAH despite defect closure, functional limitations were more common. In patients with ASD, the prevalence of right ventricular dysfunction increased with systolic pulmonary artery pressure (OR = 1.073 per mm Hg; p<0.001). Major bleeding events were more prevalent in patients with cyanosis with than without Eisenmenger's syndrome (17% vs 3%; p<0.001). CONCLUSION: In this selected population of adults with congenital heart disease, PAH was common and predisposed to more symptoms and further clinical deterioration, even among patients with previous defect closure and patients who had not developed Eisenmenger's physiology.