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INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is known to be associated with varying degrees of autonomic and cardiovascular dysfunction. Recent case reports showed that ALS may be linked to Takotsubo syndrome (TTS). We assessed the frequency of TTS in an incident ALS cohort from Sardinia, Italy, and investigated the relationship of TTS with ALS course. METHODS: We retrospectively examined a 10-year (2010-2019) incident cohort of ALS patients of Sardinian ancestry, reported TTS frequency and patients' clinical characteristics. Following, we checked for TTS among patients with ALS onset after 2019 and focused on the same features as for the incident cohort. RESULTS: Our incident cohort included 344 ALS patients and 5 of them (1.45%) developed TTS. All were female and their median onset age was 71.5 years (IQR 62.75-77). Two patients had spinal and three bulbar onset, though all patients had bulbar involvement and were at an advanced stage of disease (ALSFRS ≤ 25, King's ≥ 3) at TTS diagnosis. We identified a potential TTS trigger in three patients (hospitalization for PEG placement, pneumonia). Among patients who had ALS onset after 2019, we identified a further TTS case and described it. CONCLUSION: TTS is not a rare condition in ALS. Female sex, bulbar involvement, and later age of disease onset may be important risk factors for developing this cardiac condition and a physical or psychological trigger is often observed. Despite autonomic dysfunction in ALS has been already demonstrated, the precise physiopathological mechanism underlying TTS needs to be further clarified.

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Prevention of the first variceal haemorrhage should start when the patients have developed medium sized to large varices. Non-selective beta-blockers are the first-line treatment; band ligation is roughly equivalent to beta-blockers and is the first choice for patients with contraindications or intolerance to beta-blockers. Treatment of acute bleeding should aim at controlling bleeding and preventing early rebleeding and complications, especially infections. Combined endoscopic and pharmacological treatment with vasoactive drugs can control bleeding in up to 90% of patients. All patients who survive a variceal bleed should be treated with beta-blockers or band ligation to prevent rebleeding. All patients in whom bleeding cannot be controlled or who continue to rebleed can be treated with salvage TIPS or, in selected cases, with surgical shunts. Liver transplantation should be considered for patients with severe liver insufficiency in which first-line treatments fail.

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Portal hypertensive gastropathy (PHG) is characterized by changes in the endoscopic appearance of the gastric mucosa, specific for portal hypertension. The identification of the elementary lesions of PHG allowed the development of a reproducible classification, defining mild and severe pictures, and the execution of a natural history study. This study showed a 80% overall prevalence of PHG in patient with cirrhosis of the liver and a correlation between duration of the disease and development of PHG. PHG has often been shown to be a fluctuating condition, thus suggesting that its pathophysiology is not only related to portal hypertension, but also to other, yet unknown, factors. Bleeding from PHG did not occur in patients with a recent diagnosis of liver cirrhosis. Acute and chronic bleeding occurred in 2.5% and 12% of patients, respectively. The death rate from acute PHG bleeding was lower (12.5%) than the death rate of variceal bleeding (39.1%). Vasoactive drugs can be used in the treatment of acute PHG bleeding. For chronic bleeding, non selective 13-blockers and, if needed, iron, are the treatment of choice. TIPS or surgical portosystemic shunt may be considered for acute or chronic PHG bleeding, if medical treatment fails. Clinical controlled trials are needed to evaluate the efficacy of these or other treatments.

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Concerns about the increasing medical care costs are causing the medical community to focus its attention on the appropriate of diagnostic tests such as echocardiography. Prerequisite to a better utilization of the limited economic resources assigned to our health care system is an analysis of how, why, and with which results diagnostic tests with a widespread use and relevant cost, like echocardiography, are requested. During the last 2 weeks of September 1994, a transversal, observational study was carried out at 13 hospital echocardiographic laboratories. Ordering physician characteristics, reasons for ordering the test, cardiological diagnostic tests previously performed and their relationship with the test results, were evaluated with a questionnaire completed by the physician who performed the test, in all the out-patients undergoing echocardiogram in that fortnight. Five hundred and sixteen consecutive questionnaires were successfully completed. Fourty-five percent of the echocardiograms were ordered by cardiologists, 35% by general practitioners, 10% by internists, and 10% by other specialists. Hypertension (16.4%) and ischemic heart disease (14.8%) were the most common indications for the test, followed by palpitations or arrhythmias (7.5%), mitral valve prolapse or mitral valve disease (7.3%), chest pain or angina pectoris (6.3%), cardiac murmur (5.5%), dyspnea or heart failure (5.2%), aortic valve disease (5%), prosthetic heart valve evaluation (4.6%), others (27%). Before undergoing the echocardiogram, 433 (84%) patients underwent an electrocardiogram, 242 (47%) a cardiological clinical evaluation, 196 (38%) a chest X-ray, and 191 (37%) had had a previous echocardiogram. The most common echocardiographic diagnosis was normal (29.2%) followed by hypertensive heart disease (16.2%), mitral valve disease (12.3%), aortic valve disease (10.5%), ischemic heart disease (9.3%), cardiomyopathy (4.9%) normal prosthetic heart valve function (4.5%), pericardial effusion (3.8%), others (11.3%). Among the echocardiograms ordered by cardiologists, 21.8% were normal in comparison with 35.4% of those ordered by general practitioners (p < 0.004), 35.3% of those ordered by internists (p = 0.04), 35.3% of those ordered by other specialists (p = 0.04). Among the 284 patients whose echocardiograms were not requested by a cardiologist, only 215 (76%) had undergone an electrocardiogram and only 68 (24%) a clinical evaluation by a cardiologist. In these patients, the frequency of normal echocardiograms was not influenced by having undergone a previous electrocardiogram or a chest X-ray. Conversely, patients in whom the echocardiogram was ordered after a cardiology consult showed a significant lower frequency of normal results compared to patients not evaluated by a cardiologist (23% vs 39%; p < 0.05). More than 50% of the echocardiograms performed in out-patients are ordered by physicians who are not cardiologists. Among these echocardiograms, about 1 out of 3 results normal. This finding suggests an improper use of echocardiogram as a screening tool by non-cardiologists in out-patients. A preceding clinical evaluation by a cardiologist, but not an electrocardiogram or a chest X-ray alone, may determine a more appropriate use of the test being associated with a reduced frequency of normal results.

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Doppler echocardiographic characteristics of normally functioning Allcarbon prostheses were studied in 149 consecutive patients with 157 valves in the mitral (n = 73) and aortic (n = 84) positions whose function was considered normal by clinical and echocardiographic evaluation. In the mitral position, the mean gradient and the effective mitral orifice area were not significantly different in either the 25-mm or the 31-mm size valves (from 5 +/- 1 to 4 +/- 1 mmHg and from 2.2 +/- 0.6 to 2.8 +/- 0.9 cm2, respectively; P = ns for both). Conversely, peak gradient was significantly and inversely correlated to actual orifice area (r = -0.70; P < 0.0006), decreasing from 15 +/- 3 mmHg in the 25-mm size valve to 9 +/- 1 mmHg in the 31-mm size. In the aortic position, the mean gradient was 29 +/- 8 mmHg in the 19-mm size valve; it decreased to 8 +/- 2 mmHg in the 29-mm size. Effective prosthetic aortic valve area, calculated using the continuity equation, ranged between 0.9 +/- 0.1 cm2 for the 19-mm size valve to 4.1 +/- 0.7 cm2 for the 29-mm size. By analysis of variance, effective prosthetic aortic valve area differentiated various valve sizes (F = 25.3; P < 0.0001) better than peak (F = 5.34; P = 0.012) or mean (F = 4.34; P = 0.0052) gradients alone, and it correlated better with actual orifice area (r = 0.89, r = -0.70 and r = -0.65, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)

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A 62-year-old woman with a Bjork-Shiley prosthetic mitral valve, presented with pulmonary edema and cardiogenic shock. Doppler echocardiography showed a significant increase of trans-prosthetic gradient due to a thrombotic obstruction. Successful treatment with rt-PA is described. From a subsequent analysis of previous echocardiograms a longstanding asymptomatic thrombus is hypothesised.

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The detection of a coronary artery fistula (CAF) is usually by chance and, because of the to-and-fro characters of the murmur, a misdiagnosis of a patent ductus arteriosus is frequently made. Correct diagnosis can only be reached after hemodynamic investigation completed by angiography. This lesion is often accompanied by few or no symptoms in infancy, but may become symptomatic with aging even if the shunt is small: complications such as endocarditis, rhythm disturbances, aneurysmatic dilation and rupture have been reported. Five cases of CAF have been studied in our institution: 3 of them have been operated upon in extracorporeal circulation with good results. Principles of management are controversial: many authors advocate delay of treatment until symptoms appear: in our opinion, based on the natural history of the disease and on our experience, closure of the fistula in pediatric age, even in asymptomatic patients, is the treatment of choice.

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