Echocardiographic clues to diagnosis of dystrophin related dilated cardiomyopathy.
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Adult↗
Biomedical subjects
C Rapezzi
Publications and source records attributed to C Rapezzi.
Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction.
OBJECTIVES: To investigate diagnostic routes, echocardiographic substrates, outcomes and prognostic factors in patients with isolated ventricular non-compaction (IVNC) identified by echocardiographic laboratories with referral from specialists and primary care physicians. PATIENTS AND DESIGN: Since 1991, all patients with suspected IVNC were flagged and followed up on dedicated databases. Patients were divided into symptom-based and non-symptom-based diagnostic subgroups. RESULTS: 65 eligible patients were followed up for 6-193 months (mean 46 (SD 44). In 53 (82%) patients, IVNC was associated with variable degrees of left ventricular (LV) dilatation and hypokinesia, and in the remaining 12 (18%) LV volumes were normal. Diagnosis was symptom based in 48 (74%) and non-symptom based in 17 (26%) (familial referral in 10). The non-symptom-based subgroup was characterised by younger age, lower prevalence of ECG abnormalities, better systolic function and lower left atrial size, whereas the extent of non-compaction was not different. No major cardiovascular events occurred in the non-symptom-based group, whereas 15 of 48 (31%) symptomatically diagnosed patients experienced cardiovascular death or heart transplantation (p = 0.01, Kaplan-Meier analysis). Independent predictors of cardiovascular death or heart transplantation were New York Heart Association class III-IV, sustained ventricular arrhythmias and left atrial size. CONCLUSIONS: IVNC is associated with a broad spectrum of clinical and pathophysiological findings, and the overall natural history and prognosis may be better than previously thought. Adult patients with incidental or familial discovery of IVNC have an encouraging outlook, whereas those who have symptoms of heart failure, a history of sustained ventricular tachycardia or an enlarged left atrium have an unstable course and more severe prognosis.
Adult↗
Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance.
OBJECTIVE: To investigate the prevalence and distribution of gadolinium (Gd) enhancement at cardiac magnetic resonance (CMR) imaging in patients with cardiac amyloidosis (CA) and to look for associations with clinical, morphological, and functional features. PATIENTS AND DESIGN: 21 patients with definitely diagnosed CA (nine with immunoglobulin light chain amyloidosis and 12 transthyretin related) underwent Gd-CMR. RESULTS: Gd enhancement was detected in 16 of 21 (76%) patients. Sixty six of 357 (18%) segments were enhanced, more often at the mid ventricular level. Transmural extension of enhancement within each patient significantly correlated with left ventricular (LV) end systolic volume (r = 0.58). The number of enhanced segments correlated with LV end diastolic volume (r = 0.76), end systolic volume (r = 0.6), and left atrial size (r = 0.56). Segments with > 50% extensive transmural enhancement more often were severely hypokinetic or akinetic (p = 0.001). Patients with > 2 enhanced segments had significantly lower 12 lead QRS voltage and Sokolow-Lyon index. No relation was apparent with any other clinical, morphological, functional, or histological characteristics. CONCLUSION: Gd enhancement is common but not universally present in CA, probably due to expansion of infiltrated interstitium. The segmental and transmural distribution of the enhancement is highly variable, and mid-ventricular regions are more often involved. Enhancement appears to be associated with impaired segmental and global contractility and a larger atrium.
Amyloidosis↗
Long term outcome in patients with silent versus symptomatic ischaemia during dobutamine stress echocardiography.
OBJECTIVES: To compare the long term prognosis of patients having silent versus symptomatic ischaemia during dobutamine stress echocardiography (DSE). DESIGN: Observational study. SETTING: Tertiary referral centre. PATIENTS: 931 patients who experienced stress induced myocardial ischaemia during DSE. RESULTS: Silent ischaemia was present in 643 of 931 patients (69%). The number of dysfunctional segments at rest (mean (SD) 9.6 (5.1) v 8.8 (5.0), p = 0.1) and of ischaemic segments (3.5 (2.2) v 3.8 (2.1), p = 0.2) was comparable in both groups. During a mean (SD) follow up of 5.5 (3.3) years, there were 169 (18%) cardiac deaths and 86 (9%) non-fatal infarctions. Multivariable Cox regression analysis showed age (hazard ratio (HR) 1.1, 95% confidence interval (CI) 1.02 to 1.05), previous myocardial infarction (HR 1.4, 95% CI 1.1 to 2.0), and number of ischaemic segments during the test (HR 2.0, 95% CI 1.0 to 3.7) as independent predictors of cardiac death and myocardial infarction. For every additional ischaemic segment there was a twofold increment in risk of late cardiac events. The annual cardiac death or myocardial infarction rate was 3.0% in patients with symptomatic ischaemia and 4.6% in patients with silent ischaemia (p < 0.01). Silent induced ischaemia was an independent predictor of cardiac death and myocardial infarction (HR 1.7, 95% CI 1.1 to 2.0). During follow up symptomatic patients were treated more often with cardioprotective therapy (p < 0.01) and coronary revascularisation (145 of 288 (50%) v 174 of 643 (27%), p < 0.001). CONCLUSIONS: Patients with silent ischaemia had a similar extent of myocardial ischaemia during DSE compared to patients with symptomatic ischaemia but received less cardioprotective treatment and coronary revascularisation and experienced a higher cardiac event rate.
Angina Pectoris↗
Amyloid deposition as a cause of atrial remodelling in persistent valvular atrial fibrillation.
AIM: The spectrum of histological alterations, namely atrial amyloidosis, in the right and left atria of patients with chronic persistent atrial fibrillation (AF) and rheumatic heart disease is not completely known. METHODS AND RESULTS: One hundred and twenty-eight atrial appendages (66 left and 62 right), obtained from 72 patients with rheumatic valve disease and chronic AF undergoing cardiac surgery for valve replacement or repair and AF treatment were histologically evaluated for the presence of amyloid deposits. One hundred and four specimens of left and right auricles from 52 patients in sinus rhythm with severe chronic heart failure undergoing heart transplant were also analyzed (controls). Amyloid was found in 33 (46%) valvular patients with chronic persistent AF and in 6 (12%) controls. Amyloid was related to the presence and duration of AF, was more frequently found in left atrial samples and was independent of age. On stepwise logistic regression analysis, AF duration and female gender were independently related to amyloid deposition. CONCLUSIONS: Patients with long-standing AF and rheumatic heart disease have a very high prevalence of atrial amyloidosis. Amyloid deposition is more frequent in left than in right atrial appendage and correlates with AF duration and female gender. Amyloid deposition could constitute an additional histological feature in the structural remodeling of atria during long-standing AF, at least in rheumatic valve disease. Persistence of AF might play a pivotal role in promoting amyloid deposition.
Aged↗
[Angiotensin receptor antagonists and ACE inhibitors in heart failure: alternative or combined therapies?].
The theoretical advantages of the angiotensin II receptors blockers (ARB) are slowly but progressively becoming a reality for patients with congestive heart failure. ARB are recommended in patients who cannot take ACE inhibitors as a plausible alternative for modulating the renin angiotensin system. After the recently published Val-HeFT trial, the addition of an ARB (valsartan) can be considered in patients who remain severely symptomatic despite optimal therapy in order to reduce hospitalization for heart failure and improve symptoms particularly if the patients cannot tolerates beta-blockers. Among patients with type 2 diabetes and no clinical signs of congestive heart failure ARB (losartan) are able to reduce the probability of developing congestive heart failure.
Angiotensin Receptor Antagonists↗
Precipitating factors and decision-making processes of short-term worsening heart failure despite "optimal" treatment (from the IN-CHF Registry).
This study sought to prospectively assess which factors were related to short-term worsening heart failure (HF) leading to or not to hospital admission, in long-term outpatients followed by cardiologists. The subsequent decision-making process was also analyzed. The study population consisted of 2,701 outpatients enrolled in the registry of the Italian Network on Congestive Heart Failure (IN-CHF) and followed by 133 cardiology centers (19% of all existing Italian cardiology centers). Clinical and follow-up data were collected by local trained clinicians; 215 patients (8%) had short-term decompensation (on average 2 months after the index outpatient visit). Multivariate analysis showed that previous hospitalization, long duration of symptoms, ischemic etiology, atrial fibrillation, higher functional class (New York Heart Association classification III to IV), higher heart rate, and low systolic blood pressure were independently associated with HF destabilization. Poor compliance (21%) and infection (12%) were the most frequent precipitating factors, but a precipitating factor was not identified in 40% of the patients. Poor compliance was more common in women, but no other clinical characteristics emerged as being related with a specific precipitating factor. Fifty-seven percent of the patients with a short-term recurrence of worsening HF required hospital admission; infusion treatment with inotropes and/or vasodilators was necessary in 19% of them. Long-term therapy was changed in 48% of the patients. Thus, in ambulatory HF patients, short-term worsening HF can be predicted according to the clinical characteristics on an outpatient basis. Nearly 1/3 of precipitating factors can be prevented. Patient education and avoidance of inappropriate treatment may reduce the number of relapses.
Aged↗
Usefulness of transesophageal echocardiographic monitoring to improve the outcome of stent-graft treatment of thoracic aortic aneurysms.
The stent-graft procedure is becoming an alternative to surgery for treatment of many diseases of the descending thoracic aorta. This study evaluated the role of transesophageal echocardiography (TEE), used in combination with fluoroscopy and angiography, in monitoring the outcome of stent-graft placement. Twenty-two consecutive patients were submitted to stent-graft positioning in the descending aorta for various pathologies (7 patients had type B aortic dissections, 6 had thoracic aneurysms, 2 had thoraco-abdominal aneurysms, and 7 had post-traumatic aortic aneurysms). Before stent-graft deployment, TEE changed the proximal site of stent positioning initially identified by angiography in 33% of patients (5 of 15) with aortic aneurysms because of calcifications or atheromas that could interfere with stent adhesion to the aortic wall and that were not seen on angiography. In 28% of patients (2 of 7) with aortic dissection, TEE showed the guidewire in the false lumen, allowing an immediate repositioning. After stent-graft deployment, color Doppler TEE showed a perigraft leak in 7 patients, whereas angiography detected a perigraft leak in only 2 patients (p = 0.02). In 4 of these patients, further balloon expansions resulted in resolution of the leak. In the remaining 3 patients, additional stent-graft positioning was necessary. Considering the total patient cohort, TEE yielded relevant information, resulting in procedure changes in 59% (13 of 22). In conclusion, TEE provided additional information with respect to angiography in all phases of stent-graft treatment, improving immediate outcome and reducing complications.
Adult↗
Primary endoleakage in endovascular treatment of the thoracic aorta: importance of intraoperative transesophageal echocardiography.
OBJECTIVES: Endovascular treatment of the thoracic aorta has developed as an efficacious alternative to open surgical repair. However, despite the high primary success rate, perigraft leakage constitutes the major concern in long-term follow-up. Endoleaks are widely reported both in abdominal and thoracic endovascular series and are usually identified by intraoperative angiography. Transesophageal echocardiography is a sensitive imaging technique in the evaluation of aortic diseases, widely used to monitor cardiac surgery. The aim of this study was to evaluate the efficacy of transesophageal echocardiography in leakage detection during endovascular stent procedures of the thoracic aorta. METHODS: Intraoperative transesophageal echocardiography was used in conjunction with angiography in 25 patients subjected to endovascular stent treatment of the descending thoracic aorta. Spiral computed tomographic scanning was performed before discharge and 3, 6, and 12 months after treatment. RESULTS: Information from transesophageal echocardiography was relevant in the selection of the landing zone in 62% of cases. In 8 patients, transesophageal echocardiography with color Doppler sonography showed a perigraft leak, 6 of which were not visible on angiography, suggesting the need for further balloon expansion or graft extension. Postoperative computed tomographic scanning in the 25 patients showed 1 endoleak, which sealed spontaneously. At 3 months, computed tomographic examination confirmed the absence of perigraft leakage in all patients. CONCLUSIONS: During implantation of a stent-graft in the descending thoracic aorta, transesophageal echocardiography provides information in addition to that provided by angiography, improving immediate and late procedural results.
Adult↗
[Comparison of candesartan, enalapril and their combination in congestive heart failure].
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Angiotensin Receptor Antagonists↗
[Interventional or conservative approaches in acute coronary syndromes].
The past decade has witnessed a dramatic expansion in the scope of both mechanical and pharmacologic methods for treating patients with acute coronary syndromes. New device technologies, such as intracoronary stenting, more potent and more fibrin-specific thrombolytic agents, and new antithrombotic and antiplatelet drugs are now available. What is the best strategy to treat patients with acute coronary syndromes still remains a matter of debate. This article briefly reviews this topic.
Acute Disease↗
The case against outpatient parenteral inotropic therapy for advanced heart failure
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Journal Article↗
[Critical analysis of beta blockers in heart failure: certainty and incompleteness].
Heart failure is one of the commonest debilitating conditions of industrialized society, with mortality and morbidity comparable with that of the common neoplastic diseases. The role of beta-blockers in heart failure has been the subject of debate for many years. The results of recent prospective, placebo-controlled studies of the addition of beta-blockers to standard therapy in patients with chronic heart failure have confirmed a significant beneficial effect on ventricular function, clinical status, morbidity and mortality. The importance of these trials suggests that beta-adrenergic blocker therapy can save one life out of every 35 patients treated with mild-to-moderate heart failure. These major trials have used one of four beta-blockers (metoprolol, bisoprolol, carvedilol, or bucindolol) in varying study designs with different patient populations. Beta-blockers improve function of the failing left ventricle, prevent or reverse progressive left ventricular dilation, chamber sphericity, and hypertrophy, and consequently have a positive impact on cardiac remodeling. Beta-blockers also reduce heart rate and left ventricular wall stress, leading to reduced myocardial oxygen consumption, a clear benefit to the failing heart. Moreover, beta-blockers can improve the intrinsic contractile function of cardiomyocytes and have been shown to improve myocardial energetics in heart failure, possibly through desirable changes in substrate utilization. Many important clinical questions still remain unanswered. These questions include whether beta-blockers are of benefit in patients with severe NYHA functional class (IIIB-IV), in patients with asymptomatic left ventricular dysfunction, in the extreme elderly, in patients with diabetes mellitus and renal impairment. Furthermore, it is not clear whether beta-blockade by itself is the real mechanism of clinical benefit. Although certain effects of beta-blockers may be considered class effects, it is not yet clear whether there are differences between beta 1-selective antagonists and nonselective agents. Major studies are currently being undertaken to address the above questions.
Adrenergic beta-Antagonists↗
Fifteen years of ACE-inhibition in congestive heart failure.
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Angiotensin-Converting Enzyme Inhibitors↗
The case against outpatient parenteral inotropic therapy for advanced heart failure.
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Ambulatory Care↗
Infective endocarditis in hypertrophic cardiomyopathy: prevalence, incidence, and indications for antibiotic prophylaxis.
BACKGROUND: The literature on infective endocarditis in hypertrophic cardiomyopathy (HCM) is virtually confined to case reports. Consequently, the risk of endocarditis in HCM remains undefined. METHODS AND RESULTS: We assessed the occurrence of endocarditis in 810 HCM patients evaluated between 1970 and 1997. Endocarditis was diagnosed in 10 patients, 2 of whom were excluded from analysis of prevalence and incidence because they were referred for acute endocarditis. At first evaluation, echocardiographic features consistent with prior endocarditis were identified in 3 of 808 patients, a prevalence of 3.7 per 1000 patients (95% CI, 0.8 to 11). Of 681 patients who were followed, 5 developed endocarditis, an incidence of 1.4 per 1000 person-years (95% CI, 0.5 to 3.2); outflow obstruction was present in each of these 5 patients and was associated with the risk of endocarditis (P=0.006). In the 224 obstructive patients, incidence of endocarditis was 3.8 per 1000 person-years (95% CI, 1.6 to 8.9) and probability of endocarditis 4. 3% at 10 years. Left atrial size was also associated with the risk of endocarditis (P=0.007). In patients with both obstruction and atrial dilatation (>/=50 mm), incidence of endocarditis increased to 9.2 per 1000 person-years (95% CI, 2.5 to 23.5). Analysis of all 10 patients with endocarditis identified outflow obstruction in each and atrial dilatation in 7. CONCLUSIONS: Endocarditis in HCM is virtually confined to patients with outflow obstruction and is more common in those with both obstruction and atrial dilatation. These results indicate that antibiotic prophylaxis is required only in patients with obstructive HCM.
Acute Disease↗
[Endovascular treatment in diseases of the thoracic aorta].
PURPOSE: To determine whether endovascular treatment of thoracic aorta conditions can be an effective alternative approach to surgical repair. MATERIAL AND METHODS: July 1997 to February 1999, eighteen patients (16 men and 2 women; 58.6 +/- 14.8 years) presenting with different kinds of descending aorta conditions were selected for the endovascular treatment. All patients exhibited severe comorbid pulmonary and/or cardiovascular medical conditions which increased surgical risk. All implants were performed in the operating room under fluoroscopic and TEE guidance. Clinical and imaging follow-up was performed 1, 3, 6 and 12 months later. RESULTS: The endovascular treatment was successful in 17 cases. No deaths or major complications occurred. No leakage was evident at post-procedure angiography. The patients were discharged after 6 +/- 4 days. MRI or CT study performed before hospital discharge showed aneurysms exclusion in 16 patients. In the four cases of dissection, thrombosis of the false lumen was evident since the first follow-up study. In the group of patients (11 cases) with 6 months follow-up, the diameters of stented aortic segments decreased. No late leakage was observed and thrombosis was complete in all cases. DISCUSSION: The natural history of aortic aneurysms and dissection is progressive toward dilation and aortic rupture. Surgery of descending thoracic aorta is burdened with a mortality of 8-12% in elective cases and over 50% in emergency cases or aortic dissection. The endovascular treatment of aortic conditions was introduced in clinical practice in 1991 and literature data show that it is effective, with lower mortality and morbidity rates than surgical treatment. CONCLUSION: Our results stress the feasibility and effectiveness of endovascular procedure in the treatment of complex thoracic aorta conditions even in high risk patients. Thus, endovascular treatment of thoracic aorta can be considered an effective alternative approach to conventional surgery.
Adult↗