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Pulmonary arteriovenous malformations (PAVMs) associated with hereditary hemorrhagic telangiectasia may cause severe cerebral complications that may be prevented by embolization therapy. We retrospectively compared the diagnostic value of noninvasive tests for the screening of treatable (amenable to embolization) PAVMs in a series of 105 patients, using chest computerized tomography (CT) and/or pulmonary angiography as a 'gold standard.' Patients had assessment of dyspnea, chest radiograph, alveolar-arterial PO2 gradient under 100% oxygen (AaPO2), contrast echocardiography, and radionuclide perfusion lung scanning. Contrast echocardiography in the supine position was the most sensitive test (93%). The sensitivity of self-reported dyspnea (59%), chest radiograph alone (70%), measurement of AaPO2 by the 100% oxygen method (62%), or radionuclide lung scanning (71%), was not suitable for efficient screening. A 100% sensitivity and negative predictive value could be obtained when combining anteroposterior chest radiograph and contrast echocardiography. Our data support a screening algorithm based on the combined use of contrast echocardiography and anteroposterior chest radiograph, followed by chest CT if either test is positive. An alternative is to screen directly by chest CT. However, this algorithm may obviate the need for chest CT in patients without PAVM, who represent a majority of patients with hereditary hemorrhagic telangiectasia. [ABSTRACT FROM AUTHOR]

We report the case of a 70-year-old woman with rheumatic mitral stenosis and a transient ischaemic attack. Transoesophageal echocardiography revealed a cystic mass in the right atrium, hanging to the interatrial septum by a pedicle, not circulating. The mass was heterogeneous and suggested a tumour (myxoma) or a thrombus. Surgical resection showed it was an interatrial septal aneurysm, closed on itself, filled with blood. The usual causes of cardiac tumours and pathogeny of large interatrial aneurysms are discussed. [ABSTRACT FROM AUTHOR]

Background: Trials of patent foramen ovale (PFO) closure to prevent recurrent stroke have been inconclusive. We investigated whether patients with cryptogenic stroke and echocardiographic features representing risk of stroke would benefit from PFO closure or anticoagulation, as compared with antiplatelet therapy., Methods: In a multicenter, randomized, open-label trial, we assigned, in a 1:1:1 ratio, patients 16 to 60 years of age who had had a recent stroke attributed to PFO, with an associated atrial septal aneurysm or large interatrial shunt, to transcatheter PFO closure plus long-term antiplatelet therapy (PFO closure group), antiplatelet therapy alone (antiplatelet-only group), or oral anticoagulation (anticoagulation group) (randomization group 1). Patients with contraindications to anticoagulants or to PFO closure were randomly assigned to the alternative noncontraindicated treatment or to antiplatelet therapy (randomization groups 2 and 3). The primary outcome was occurrence of stroke. The comparison of PFO closure plus antiplatelet therapy with antiplatelet therapy alone was performed with combined data from randomization groups 1 and 2, and the comparison of oral anticoagulation with antiplatelet therapy alone was performed with combined data from randomization groups 1 and 3., Results: A total of 663 patients underwent randomization and were followed for a mean (±SD) of 5.3±2.0 years. In the analysis of randomization groups 1 and 2, no stroke occurred among the 238 patients in the PFO closure group, whereas stroke occurred in 14 of the 235 patients in the antiplatelet-only group (hazard ratio, 0.03; 95% confidence interval, 0 to 0.26; P<0.001). Procedural complications from PFO closure occurred in 14 patients (5.9%). The rate of atrial fibrillation was higher in the PFO closure group than in the antiplatelet-only group (4.6% vs. 0.9%, P=0.02). The number of serious adverse events did not differ significantly between the treatment groups (P=0.56). In the analysis of randomization groups 1 and 3, stroke occurred in 3 of 187 patients assigned to oral anticoagulants and in 7 of 174 patients assigned to antiplatelet therapy alone., Conclusions: Among patients who had had a recent cryptogenic stroke attributed to PFO with an associated atrial septal aneurysm or large interatrial shunt, the rate of stroke recurrence was lower among those assigned to PFO closure combined with antiplatelet therapy than among those assigned to antiplatelet therapy alone. PFO closure was associated with an increased risk of atrial fibrillation. (Funded by the French Ministry of Health; CLOSE ClinicalTrials.gov number, NCT00562289 .).

Objectives: Although septal myectomy is the technique of choice for hypertrophic cardiomyopathy, the surgical management of concomitant mitral valve lesions is controversial. Various complex surgeries have been proposed to address mitral valve lesions. We propose a simple option using an edge-to-edge mitral valve repair through the aortic valve in addition to the septal myectomy., Methods: We performed an observational analysis of our prospectively collected database. The clinical follow-up was done by telephone contact with each patient. The echocardiographic follow-up was performed in our Department of Cardiology or by the referring cardiologist., Results: Between January 2009 and March 2016, we operated 22 symptomatic patients (mean age 48.5 years, males 59%). The mean interventricular septum diameter and resting intraventricular gradient were 25.8 mm and 75.4 mmHg, respectively. The systolic anterior motion was present in every patient. The mean mitral regurgitation grade was 2.4. There were no in-hospital deaths. Two (9%) patients required a pacemaker. After a mean follow-up of 26.3 months, the mean New York Heart Association functional class decreased from 2.5 to 1.2 ( P  < 0.001). The echocardiographic follow-up showed a sustained significant reduction of the septal thickness ( P  < 0.001), resting intraventricular gradient ( P  < 0.001), presence of systolic anterior motion ( P  < 0.001) and grade of mitral regurgitation ( P  = 0.002)., Conclusions: Septal myectomy remains the gold standard of any surgery for hypertrophic cardiomyopathy owing to its good clinical and echocardiographic results. The edge-to-edge mitral valve repair is an additional simple option to avoid the systolic anterior motion and effectively reduce the grade of mitral regurgitation., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Rationale: Currently available data do not provide definitive evidence on the comparative benefits of closure of patent foramen ovale, oral anticoagulants and antiplatelet therapy in patients with patent foramen ovale-associated cryptogenic stroke, Aim: To assess whether transcatheter patent foramen ovale closure plus antiplatelet therapy is superior to antiplatelet therapy alone and whether oral anticoagulant therapy is superior to antiplatelet therapy, for secondary stroke prevention in patients aged 16 to 60 years with a large patent foramen ovale or a patent foramen ovale associated with an atrial septal aneurysm, and an otherwise unexplained ischaemic stroke or retinal ischaemia., Sample Size: Six hundred and sixty-four patients were included in the study., Methods and Design: CLOSE is an academic-driven, multicentre, randomized, open-label, three-group, superiority trial with blinded adjudication of outcome events. The trial has been registered with Clinical Trials Register (Clinicaltrials.gov, NCT00562289). Patient recruitment started in December 2007. Patient follow-up will continue until December 2016. Expected mean follow-up = 5.6 years., Study Outcomes: The primary efficacy outcome is the occurrence of fatal or nonfatal stroke. Safety outcomes include fatal, life-threatening or major procedure- or device-related complications and fatal, life-threatening or major haemorrhagic complications., Discussion: CLOSE is the first specifically designed trial to assess the superiority of patent foramen ovale closure over antiplatelet therapy alone and the superiority of oral anticoagulants over antiplatelet therapy to prevent stroke recurrence in patients with patent foramen ovale-associated cryptogenic stroke., (© 2016 World Stroke Organization.)

Aims: To evaluate the benefit of adding Losartan to baseline therapy in patients with Marfan syndrome (MFS)., Methods and Results: A double-blind, randomized, multi-centre, placebo-controlled, add on trial comparing Losartan (50 mg when <50 kg, 100 mg otherwise) vs. placebo in patients with MFS according to Ghent criteria, age >10 years old, and receiving standard therapy. 303 patients, mean age 29.9 years old, were randomized. The two groups were similar at baseline, 86% receiving β-blocker therapy. The median follow-up was 3.5 years. The evolution of aortic diameter at the level of the sinuses of Valsalva was not modified by the adjunction of Losartan, with a mean increase in aortic diameter at the level of the sinuses of Valsalva of 0.44 mm/year (s.e. = 0.07) (-0.043 z/year, s.e. = 0.04) in patients receiving Losartan and 0.51 mm/year (s.e. = 0.06) (-0.01 z/year, s.e. = 0.03) in those receiving placebo (P = 0.36 for the comparison on slopes in millimeter per year and P = 0.69 for the comparison on slopes on z-scores). Patients receiving Losartan had a slight but significant decrease in systolic and diastolic blood pressure throughout the study (5 mmHg). During the study period, aortic surgery was performed in 28 patients (15 Losartan, 13 placebo), death occurred in 3 patients [0 Losartan, 3 placebo, sudden death (1) suicide (1) oesophagus cancer (1)]., Conclusion: Losartan was able to decrease blood pressure in patients with MFS but not to limit aortic dilatation during a 3-year period in patients >10 years old. β-Blocker therapy alone should therefore remain the standard first line therapy in these patients., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Unlabelled: Hereditary hemorrhagic telangiectasia (HHT) is a genetic disease characterized by cutaneous, mucosal, and sometimes visceral arteriovenous malformations. Severe hepatic manifestations have been characterized in a subgroup of patients, but few data are available in previously nonscreened patients. We prospectively evaluated liver involvement and its cardiac consequences in such patients. Between 2000 and 2005, we prospectively evaluated the clinical, biological, and hepatic Doppler sonography (DS) characteristics of 102 consecutive HHT patients (mean age, 52.5 years; range, 19-88; 80.4%) with an identified genetic mutation. Patients were segregated into three different severity groups according to DS values. Factors predictive of an abnormal DS, according to predetermined criteria, and of a high cardiac index were identified by logistic and linear regression analysis, respectively. Abnormal liver biology and clinical signs of hepatic involvement were present in 35.3% and 27.5% of cases, respectively. Abnormal DS (defined as at least enlargement of the main hepatic artery) was observed in 56 (54.9%) cases, and direct or indirect signs of significant fistulas were present in 26 (25.5%) cases. Abnormal liver biology and a mutation involving the ACVRL1 gene were predictive of hepatic ultrasound (US) abnormalities. The diameter of the main hepatic artery and the presence of focal nodular hyperplasia (FNH) were predictive of a higher cardiac index., Conclusion: This large prospective series of previously nonscreened HHT patients identified a subgroup at risk of liver involvement (patients with abnormal liver biology and ACVRL1 mutations) and a subgroup with a higher cardiac index: future studies will show whether such patients would benefit from systematic DS screening and long-term cardiac surveillance.

Background: Cardiac resynchronization therapy (CRT) produces an early improvement in left ventricular (LV) function in patients with congestive heart failure (CHF), but little is known about its effects on right ventricular (RV) function., Aim: To assess the early effects of CRT on RV function using myocardial strain analysis., Methods: Fifty CHF patients (New York Heart Association class III/IV, left ventricular ejection fraction [LVEF] less than 35%, QRS greater than 120 ms) were studied before and three months after CRT. RV chamber dimension was quantified using tricuspid annulus diameter and RV short- and long-axis dimensions. RV function was assessed by tricuspid annulus plane systolic excursion and velocity (V(s)) and lateral wall strain. RV mechanical dyssynchrony was calculated using the difference in time-to-peak strain between septal and lateral wall., Results: After three months, LVEF had increased significantly (from 22+/-6 to 27+/-9%; P<0.01) and LV end-diastolic volumes had decreased significantly (from 232+/-73 to 219+/-78 ml; P<0.05) in patients with LV mechanical dyssynchrony at baseline (n=35). RV dimensions did not change significantly, but there was an early improvement in RV function as demonstrated by an increase in V(s) (from 5.3+/-2.4 to 6.4+/-1.8 cm s(-1), P=0.001) and RV lateral wall basal and mid strain (from 23+/-9 to 28+/-9%, P=0.009 and from 20+/-7 to 25+/-8%, P=0.01, respectively). The improvement in RV strain occurred in patients with septal RV lead position and correlated with the magnitude of RV dyssynchrony at baseline (r=0.74; P<0.05)., Conclusion: After three months, CRT improved RV function significantly in CHF patients before any significant change in RV dimensions.

Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by the presence of telangiectases and arteriovenous malformations. In some families in whom a form of idiopathic pulmonary arterial hypertension cosegregated with HHT, mutations in the ACVRL1 gene were present., Purpose: We noninvasively measured the pulmonary artery systolic pressure (PASP) in a group of patients with HHT., Methods: Doppler transthoracic echocardiography and mutation analysis by direct sequencing were used., Results: We studied 68 patients (age 19-84 years, mean 50.75 + 15.11; 32 females) and PASP measurement was possible in 44 (64. 7%); in addition, 9 of them (20.5%) showed elevated values. Molecular analysis identified mutations in the ACVRL1 gene in 7 of these 9 subjects. Even on exclusion of relatives of the single case with known pulmonary hypertension, 5 of 37 patients (13.5%) still showed values higher than those of controls., Conclusion: The data indicate that elevated PASP values are a frequent and previously unrecognized complication of HHT. Because clinically significant pulmonary artery hypertension (a relevant cause of morbidity and mortality) may subsequently develop in these patients, we propose that the measurement of PASP should be included among the parameters recorded for all patients undergoing Doppler transthoracic echocardiography during routine clinical screening.

Background: Several testing options are available to detect asymptomatic coronary artery disease (CAD). Dobutamine stress echocardiography (DSE) has been reported to increase the sensitivity and specificity of stress testing to detect CAD. Most studies concerned patients with known or suspected CAD who have a high pretest probability of disease. We aimed to perform a preliminary evaluation of DSE in atherothrombotic stroke., Methods: Patients with transient ischemic attack or nondisabling ischemic stroke attributable to an atherothrombotic source were prospectively recruited. Patients with a history of angina pectoris or electrocardiographic signs of previous myocardial infarction were excluded. DSE was considered positive when regional reduction or deterioration of myocardial thickening developed in 1 segment. Coronary angiography was performed in patients with positive DSE., Results: Sixty-four patients were recruited. Analysis of DSE was possible in 60 patients. Overall the test provided clinically useful information in 60/64 patients studied (>90%). DSE was positive in 9 patients (15%). Coronary angiography was performed in 8 patients, high-grade focal lesions were found in 3 patients, and 5 patients showed diffuse atheroma. Univariate logistic regression analysis showed that the main factor predictive of a positive DSE was the presence of an aortic arch atheroma (p = 0.003). Multivariate logistic regression analysis showed that two factors had an independent predictive value of positive DSE: aortic arch atheroma (p = 0.007) and dyslipidemia (p = 0.09)., Conclusion: DSE may improve prevention of further vascular events in patients with an atherothrombotic source of ischemic stroke. This screening may be of particular benefit to patients with an aortic arch atheroma., (Copyright (c) 2006 S. Karger AG, Basel.)

Aortic valve sparing operations are now widely accepted for ascending aortic aneurysm surgery. We herein report our experience of the Tirone David procedure in larger indications. From January 1997 to August 2003, 50 Tirone David procedure have been performed on 36 male and 14 female (mean age: 60 +/- 15). Five patients presented a Marfan disease and 4 acute dissections. Grade III or IV aortic insufficiency was frequent (40%). Aortic diameter was not particularly dilated, ranging from 44 to 78 mm (mean: 57 +/- 10 mm). Mean ejection fraction: 57 +/- 10%. Mean left ventricular end diastolic diameter =63 +/- 7 mm. An associated mitral valve repair and 1 coronary bypass were necessary. Mean cross clamp and bypass times =94 min and 122 +/- 28 min respectively. There was one in-hospital mortality. Secondary mortality affected 2 patients (non-cardiac deaths), for a cumulative follow-up of 946 months. During follow-up continence control was always excellent, only 1 bicuspid valve had an aortic insufficiency >grade II. Tirone David procedure gave satisfactory results as regards both aortic ectasia and aortic regurgitation control. We consider it feasible even in case of aortic dissection but caution is required when facing bicuspid aortic valves.

The case of a right-to-left shunt-induced hypoxemia with an abnormal return of the inferior vena cava (AIVCR) into the left atrium (LA) is reported in a 30-year-old male with cyanosis and polycythemia. The chest X ray and the lung CT scan was normal. Spirometry was normal but the transfert-CO coefficient (KCO) was lowered. Hypoxemia was observed at rest and worsening during exercise. The alveolo-arterial oxygen tension difference under hyperoxia was increased (56 kPa). Contrast echocardiography (CEch) suggested the presence of an AIVCR with a right-to-left shunt only observed by the inferior route. The inferior vena cava (IVC) angiography and the magnetic resonance imaging demonstrated an AIVCR characterized by a direct drainage of IVC in the left atrium. The good tolerance can be explained by the association of AIVCR with an inter-auricular septal defect resulting in a left-to-right shunt which partially corrected the right-to-left shunt. After surgical treatment, arterial blood gases normalized, KCO remained low and CEch became negative.

A 31 year old right handed woman presented with acute onset of aphasia which cleared over two days. CT-scan showed a left middle cerebral artery infarct within Wernicke area. Initial transesophageal two-dimensional echocardiography disclosed a mitral valve lesion suggesting a thrombus. She was discharged on oral anticoagulant treatment. A second stroke occurred ten months later involving left lenticulo-striate arteries area. Echocardiography remain unchanged. Subsequently, giant Lambl's excrescences of mitral valve was confirmed by operation and pathologic examination. The majority of patients with Lambl's excrescences are asymptomatic. However surface thrombus is common with this tumors which reposant a potential us for cerebral embolization. These tumors should be operated since complete excision is the only definitive means of eliminating the source of recurrent embolization.

Minor potential cardioembolic sources of stroke such as atrial septal aneurysms (ASA) or patent foramen ovale (PFO) are important risk factors for cryptogenic stroke. We aim to determine the prevalence of these abnormalities through an exhaustive etiological workup including transesophageal echocardiography and cervical arteries assessment in stroke patients younger than 60 years of age who had no evidence of a significant source of embolism. We classified 118 stroke patients into four groups according to transesophageal echocardiography (TEE) and cervical arteries assessment findings. Group A, consisted of 30 (25.4%) patients who had an arteriopathy likely related to stroke without any cardiac abnormality; Group B, 49 (41%) patients who had only a potential cardiac source; Group C, 9 (7.6%) patients who had an obvious arterial source of stroke and incidental cardiac abnormalities, and Group D, 30 (25.4%) patients who had neither cardiac nor arterial source. Data were analysed with X2 test for the comparison of risk factors between groups. Variance analysis was used to compare age between groups. Significance was assessed as p < 0.05. ASA represented 56.8% of the cardiac abnormalities and was diagnosed in 35.4% of the 79 patients who had an unexplained stroke (B and D). A PFO was found in 34.1% of the patients who had a cryptogenic stroke (B and D). According to Fisher's exact test, ASA was significantly associated to PFO (p << 0.001). According to this selection one fourth of the patients might have a truly cryptogenic stroke as the etiological workup failed to demonstrate any source of stroke. Comparison between groups showed that the patients in whom an arterial source was detected also had a potential cardioembolic source in 23% of the cases (C), versus 62% in patients who had no arterial source (B and D) (p = 0.0007). Our study confirmed the strong association between ASA, PFO and stroke. Although there was a lower incidence of minor potential cardioembolic sources in patients who had a cervical artery disease, we suggest a systematic TEE screening in all patients with stroke without major cardiac source, in order to ensure a better prevention.

Between 1974 and 1990, 58 patients underwent tricuspid valve replacement with a porcine bioprosthesis (Hancock 42, Carpentier-Edwards 16) in the course of polyvalvular replacement (double 21, triple 37). Early postoperative mortality was 12%: 16 patients died secondarily, usually of cardiac causes. The actuarial survival (1 patient lost to follow-up) was 81 +/- 11% at 5 years and 60 +/- 17% at 10 years. Two patients were reoperated for dysfunction of a Hancock bioprosthesis, 11 and 15 years after implantation. At long-term, with an average follow-up of 108 +/- 48 months, 82% of survivors (28/34) were clinically improved. Doppler echocardiography was performed in 29 patients in February 1991. In 21 cases, with a follow-up of 88 +/- 40 months, the bioprosthesis was normal with an average diastolic transprosthetic pressure gradient of 3.8 +/- 1.7 mmHg. In 7 patients followed up for 129 +/- 40 months (p < 0.05) moderate dysfunction of the Hancock prosthesis was observed with a mean diastolic pressure. Severe dysfunction of a Hancock prosthesis was observed in 1 case. Fixed pulmonary hypertension was noted in 11 cases and was associated with a poor clinical result and a raised mean diastolic transprosthetic pressure gradient. The durability and haemodynamic performance of tricuspid porcine bioprostheses are satisfactory in the long term. Prosthetic dysfunction is correlated to the duration of implantation of the bioprosthesis and to persistent pulmonary hypertension.

The ALPHA program aimed at testing the feasibility and the efficiency of a multifactorial, primary preventive intervention for cardiovascular diseases. It dealt with two occupational populations: intervention and control (without any randomization), each amounting to about 12,000 people. Largely because of the economic crisis, only 9,598 and 9,558 people were seen at the 1976 initial examination, and only 43 per cent of these were re-examined at the 1981-82 final examination. In 1976, there were some significative differences between the two populations (more smokers, and less anti-hypertensive treatments in the intervention group). The same differences were found in 1981-82. So the intervention was not proved efficient, and the two populations could be pooled for the present analysis restricted to the french people (to diminish the risk of misunderstanding), aged 20-59 at the initial 1976 examination. We used the following (arbitrary) criteria of hypertension: being treated for hypertension, and/or two BP readings at 160 and/or 94 mmHg at a few minutes interval. 1) In 1981-82, 659 men and 492 women answered that they were still treated for hypertension, and/or had been previously said that they had hypertension or too much blood pressure. At this 1981-82 examination, 298 of these men (45 per cent) and 294 of these women (58 per cent) did not meet the criteria of hypertension. 2) In 1976, 3,114 men and 2,429 women did not meet the criteria of hypertension. At the 1981-82 examination, they were asked whether they had had any antihypertensive treatment previously given. 139 were still under antihypertensive treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Unlabelled: The aim of the study was to assess the prognostic value of serum creatinine (SC), in treated hypertensive subjects (HT). In a clinical population of 2738 HT consecutively examined between october 1969 and december 1982, as in-patients (n = 1599) or out-patients (n = 1139): 152 had a moderate elevation (ME) of SC: below 178 USI but above the "normal limit" (124 USI in men, 106 USI in women); 95 had a severe elevation (SE) of SC: above 17 USI. Whenever possible, each of these cases was matched to a control, belonging to the same population: same sex, same age (+/- 5 years), same group (in-patient or out-patient), same date of initial examination (+/- 6 months), but normal SC. 119 ME and 63 SE could be matched. The response rate of the mortality survey (november 1985) was 100 per cent. The mean follow-up period was 10 years (range 3 to 16 years)., Results: At initial examination, ME and SE cases differed significantly from their controls in several variables (mainly: more frequent T or J-ST ECG abnormalities and stage III-IV retinal changes); The life-table survival rate was moderately and non significantly reduced in ME cases (57 per cent at 10 years vs 66 per cent in controls); this decrease was only observed and significant (p less than 0.001) in subjects aged 60 to 79 years. In SE cases, survival rate was markedly reduced (43 per cent at 10 years vs 73 per cent in controls, p less than 0.001) and this reduction is observed at least since the age 40.(ABSTRACT TRUNCATED AT 250 WORDS)