Outcomes in Mitral Regurgitation Due to Flail Leaflets. A Multicenter European Study

Objectives: The purpose of this study was to assess incidence and predictors of events associated with nonsurgical and surgical management of severe mitral regurgitation (MR) in European institutions. Background: The management of patients with MR remains disputed, warranting multicenter studies to define clinical outcome in routine clinical practice. Methods: The MIDA (Mitral Regurgitation International DAtabase) is a registry created for multicenter study of MR with echocardiographically diagnosed flail leaflet as a model of pure, organic MR. Our cases were collected from 4 European centers. We enrolled 394 patients (age 64 ± 11 years; 67% men; 64% in New York Heart Association functional class I to II; left ventricular ejection fraction 67 ± 10%). Results: During a median follow-up of 3.9 years, linearized event rates/year under nonsurgical management were 5.4% for atrial fibrillation (AF), 8.0% for heart failure (HF), and 2.6% for death. Mitral valve (MV) surgery was performed in 315 (80%) patients (repair in 250 of 315, 80%). Perioperative mortality, defined as death within 30 days from the operation, was 0.7% (n = 2). Surgery during follow-up was independently associated with reduced risk of death (adjusted hazard ratio [HR] 0.42, 95% confidence interval [CI] 0.21 to 0.84; p = 0.014). Benefit was largely driven by MV repair (adjusted HR vs. replacement 0.37, 95% CI 0.18 to 0.76; p = 0.007). In 102 patients strictly asymptomatic and with normal ventricular function, 5-year combined incidence of AF, HF, or cardiovascular death (CVD) was 42 ± 8%. In these patients, surgery also reduced rates of CVD/HF (HR 0.26, 95% CI 0.08 to 0.89; p = 0.032). Conclusions: In this multicenter study, nonsurgical management of severe MR was associated with notable rates of adverse events. Surgery especially MV repair performed during follow-up was beneficial in reducing rates of cardiac events. These findings support surgical consideration in patients with MR due to flail leaflets for whom MV repair is feasible. © 2008 American College of Cardiology Foundation

Dilated-Hypokinetic Evolution of Hypertrophic Cardiomyopathy Prevalence, Incidence, Risk Factors, and Prognostic Implications in Pediatric and Adult Patients

ObjectivesThis study sought to investigate the incidence, risk factors, and prognosis of dilated-hypokinetic evolution in a large cohort of patients with hypertrophic cardiomyopathy (HCM) followed up at a cardiology center serving both the pediatric and the adult population.BackgroundThe available data on this evolution of HCM mainly regards prevalence (rather than incidence) in adults, with very little being known about the pediatric population.MethodsA total of 222 consecutive HCM patients (65% men, 19% ≤18 years old) were prospectively evaluated for a mean follow-up of 11 ± 9 years.ResultsA diagnosis of dilated-hypokinetic HCM was made in 12 patients at first evaluation (11 without previous septal myectomy surgery; prevalence, 4.9%). Twelve of the 210 patients with classic HCM at first evaluation underwent dilated-hypokinetic evolution (incidence, 5.3/1,000 patient-years). Patients with prevalent/incident dilated-hypokinetic evolution were younger at first evaluation (32 ± 14 years vs. 41 ± 21 years, p = 0.04) and more often had a family history of HCM (61% vs. 26%, p = 0.002) or sudden death (43% vs. 19%, p = 0.01) with respect to patients who maintained classic HCM. Moreover, they showed greater interventricular septum (23 ± 3 mm vs. 19 ± 6 mm, p = 0.004) and posterior wall (15 ± 3 mm vs. 13 ± 4 mm, p = 0.006) thickness. Cardiovascular death-free survival was lower among patients with dilated-hypokinetic HCM (p < 0.04). Cox proportional hazards regression analysis identified left ventricular wall thickness (hazard ratio [HR] = 1.07; 95% confidence interval [CI], 1.01 to 1.14; p = 0.03) and end-diastolic diameter (HR = 1.08; 95% CI 1.04 to 1.11; p = 0.0001) as independent predictors of cardiovascular death.ConclusionsDilated-hypokinetic evolution is rare but not exceptional in HCM. Young age at diagnosis, family history of HCM, and greater wall thickness are incremental risk factors for dilated-hypokinetic HCM, which carries an ominous prognosis

Left atrial size is a potent predictor of mortality in mitral regurgitation due to flail leaflets results from a large international multicenter study

Background-Left atrium (LA) enlargement is common in organic mitral regurgitation (MR) and is an emerging prognostic indicator. However, outcome implications of LA enlargement have not been analyzed in the context of routine clinical practice and in a multicenter study. Methods and Results-The Mitral Regurgitation International DAtabase (MIDA) registry enrolls patients with organic MR due to flail leaflets, diagnosed in routine clinical practice, in 5 US and European centers. We investigated the relation between LA diameter and mortality under medical treatment and after mitral surgery in 788 patients in sinus rhythm (64±12 years; median LA, 48 [43 to 52] mm). LA diameter was independently associated with survival after diagnosis (hazard ratio, 1.08 [1.04 to 1.12] per 1 mm increment). Compared with patients with LA<55 mm, those with LA â¥55 mm had lower 8-year overall survival (P<0.001). LA â¥55 mm independently predicted overall mortality (hazard ratio, 3.67 [1.95 to 6.88]) and cardiac mortality (hazard ratio, 3.74 [1.72 to 8.13]) under medical treatment. The association of LA â¥55 mm and mortality was consistent in subgroups. Similar excess mortality associated with LA â¥55 mm was observed in asymptomatic and symptomatic patients (P for interaction, 0.77). In patients who underwent mitral surgery, LA â¥55 mm had no impact on postoperative outcome (P<0.20). Mitral surgery was associated with greater survival benefit in patients with LA â¥55 mm compared with LA <55 mm (P for interaction, 0.008). Conclusions-In MR caused by flail leaflets, LA diameter â¥55 mm is associated with increased mortality under medical treatment, independent of the presence of symptoms or left ventricular dysfunction. © 2011 American Heart Association, Inc

Acute heart failure in patients with acute aortic syndrome: Pathophysiology and clinical-prognostic implications

Aims Although acute heart failure (AHF) is a potential complication of acute aortic syndromes (AAS), its clinical details and management implications have been scarcely evaluated. This study aimed to assess prevalence, pathophysiological mechanisms, impact on treatment, and in-hospital mortality of AHF in AAS. Methods and results Data were collected from a prospective AAS registry (398 patients diagnosed between 2000 and 2013). Patients with AHF were identified by the presence of dyspnoea as the presentation symptom or radiological signs of pulmonary congestion or cardiogenic shock, including patients with cardiac tamponade (CT). AHF frequency was 28% (Stanford type A 32% vs. type B 20%, P = 0.01). Four mechanisms leading to AHF were identified, alone or in combination: CT (26%), aortic regurgitation (25%), myocardial ischaemia (17%), and hypertensive crisis (10%). In type A patients, aortic regurgitation and CT were the most frequent mechanisms, whereas myocardial ischaemia and hypertensive crisis were the most frequent in type B patients. Although no difference was noted for diagnostic times, AHF at presentation led to a longer surgical delay in type A AAS. In-hospital mortality was higher in patients with AHF compared with those without (34% vs. 17%, P &lt; 0.001). After multivariable analysis, AHF was associated with increased risk of in-hospital death (adjusted odds ratio 1.97, 95% confidence interval 1.14-3.36, P = 0.014). Conclusion AHF occurs in more than a quarter of patients with AAS of both type A and type B, is due to a variety of pathophysiological mechanisms, and is associated with increased surgical delay and in-hospital mortality. © 2015 The Authors European Journal of Heart Failure © 2015 European Society of Cardiology

Clinical use of doppler echocardiography in organic mitral regurgitation: From diagnosis to patients\u2019 management

Knowledge of mitral regurgitation (MR) is essential for any care provider, and not only for those directly involved in the management of cardiovascular diseases. This happens because MR is the most frequent valvular lesion in North America and the second most common form of valve disease requiring surgery in Europe. Furthermore, due to the ageing of the general population and the reduced mortality from acute cardiovascular events, the prevalence of MR is expected to increase further. Doppler echocardiography is essential both for the diagnosis and the clinical management of MR. In the present article, we sought to provide a practical step-by-step approach to help either performing a Doppler echocardiography or interpreting its findings in light of contemporary knowledge on organic (but not only) MR

The elusive link between aortic wall histology and echocardiographic anatomy in bicuspid aortic valve: implications for prophylactic surgery

OBJECTIVE: Prediction of aortic dissection or rupture is extremely difficult in patients with bicuspid aortic valve. We aimed to identify clinical and echocardiography predictors of histological abnormalities of the aortic wall in patients with bicuspid aortic valve undergoing aortic surgery. METHODS: We assessed the histology of the aortic wall and clinical and echocardiography variables in a cohort of patients with bicuspid aortic valve (n = 127) and a wide spectrum of valvar disease who underwent replacement of the ascending aorta (with or without aortic valve surgery). Histology was classified using a 5-grade system developed by Larson and Edward. RESULTS: Histological alterations of the aortic wall were absent/mild (grade 0-1) in 77 patients (61%) and moderate/severe (grade 2-3) in 50 (39%). Patients with moderate/severe histological alterations were younger (47 ± 17 vs 53 ± 16; p = 0.042). Eighteen patients out of 48 (38%) with an ascending aorta diameter ≤ 4.5 cm had grade 2-3 aortic wall disease as did 8 out of 18 (44%) with a diameter ≤ 4 cm. Nineteen out of 46 (41%) patients with a maximal ascending aortic area/height ratio &lt; 10 cm(2) m(-1) had moderate/severe histological alterations. Multivariate logistic regression analysis showed that the indexed diameter of the aortic annulus was significantly associated with grade 2-3 aortic wall disease (odds ratio (OR): 12.22, 95% confidence interval (CI): 1.65-90.38, p = 0.014). CONCLUSIONS: A high proportion of patients with bicuspid aortic valve and mild to moderate aortic dilatation have severe histological abnormalities of the aortic wall that are not predictable by clinical and echocardiographic findings. These observations suggest that risk stratification for aortic dissection or rupture in patients with bicuspid aortic valve is so far quite suboptimal and future investigations are warranted

In the era of the valve-in-valve: is transcatheter aortic valve implantation (TAVI) in sutureless valves feasible?

Sutureless aortic valve implantation has emerged as an innovative alternative for treatment of aortic stenosis. By avoiding the placement of sutures, this approach aims to improve surgical outcomes by facilitating less traumatic minimally invasive approaches and reducing cross-clamp and cardiopulmonary bypass duration. However, the absence of sutures may have detrimental effects after sutureless interventions, including paravalvular leakages, valve dislocation, and stent-infolding. Transcatheter aortic valve-in-valve implantation (A-ViV) is emerging as a valuable procedure in patients with dysfunctioning biological aortic valves who are deemed inoperable with conventional surgery. Here we present the first-in-man case of trans-femoral implant of a balloon expandable aortic valve in a leaking sutureless self-expandable valve

Different types of cardiomyopathy associated with isolated ventricular noncompaction

Although mainly described in the context of dilated and hypokinetic left ventricles, it is unclear whether isolated ventricular noncompaction (IVNC) is a distinct cardiomyopathy, a subtype of dilated cardiomyopathy, or a morphogenetic disorder. To investigate the spectrum of cardiomyopathies associated with IVNC, children and adults with stringent echocardiographic diagnoses of IVNC were reviewed. Seventy-three patients (12 children aged <15 years) seen since 1994 satisfied stringent echocardiographic criteria for IVNC. Sixty-five patients (89%; 11 children) had dilated cardiomyopathy, 2 adults had clear-cut hypertrophic cardiomyopathy, 1 adult had restrictive cardiomyopathy (to the investigators' knowledge, the first reported example of this particular association), and 5 patients (1 child) had normal left ventricular morphology and function. In conclusion, knowledge that IVNC can co-exist with restrictive and hypertrophic cardiomyopathy (in addition to the dilated form) supports the concept that IVNC is a morphologic trait rather than a distinct cardiomyopathy. This knowledge should be taken into account during echocardiographic examination and encourage the use of contrast echocardiography (and magnetic resonance) and could also orient molecular biology studies

Inferior Q waves in apparently healthy subjects: Should we take a deep breath? An electrocardiographic, echocardiographic and cardiac magnetic resonance study

Aim To evaluate the diagnostic accuracy of electrocardiographic inferior Q waves persistence during inspiration and echocardiographic segmental wall motion abnormalities for the detection of previously unsuspected silent myocardial infarction, by using cardiac magnetic resonance as the gold standard. Methods We prospectively enrolled 50 apparently healthy subjects with inferior Q waves on routine electrocardiogram and high atherosclerotic risk profile. Patients underwent electrocardiogram during deep inspiration, standard transthoracic echocardiography, and cardiac magnetic resonance. Results Inferior Q waves during deep inspiration persisted in 10 subjects (20%) and cardiac magnetic resonance was positive in 10 (20%). Between the 10 positive cardiac magnetic resonance subjects 8 showed persistence of inferior Q waves, giving a sensitivity of 80% (95%;CI 44.4-97.5%) and a specificity of 95% (95%;CI 83.1-99.4%). Segmental wall motion abnormalities were present overall in 10 subjects (20%), but only in 5 of the 10 positive cardiac magnetic resonance subjects, giving a sensitivity of 87.5% (95% CI 73.2-95.8) and specificity of 50% (95% CI 18.7-81.3). Conclusions Electrocardiographic inferior Q waves persistence during deep inspiration is a simple test with a high accuracy for diagnosis of silent myocardial infarction. Standard echocardiography resulted less accurate