Patients with hypertrophic cardiomyopathy (HCM) exhibit a variable phenotype with ventricular hypertrophy as the cardinal manifestation and left ventricular (LV) outflow tract obstruction (LVOTO) as ...a key pathophysiologic determinant. Patients with severe LVOTO usually present with exertional dyspnea, exertional syncope, and heart failure symptoms, while successful relief of LVOTO by pharmacological or invasive interventions leads to a dramatic improvement in clinical status. Proper management of obstructive HCM remains challenging and poses numerous clinical dilemmas. Since the development of surgical myectomy over half a century ago, progress in the management of LVOTO in HCM has paralleled technological advances in genetic testing, cardiac imaging, arrhythmic prophylaxis, cardiac surgery and interventional cardiology. These changes have been incorporated in dedicated scientific guidelines on both sides of the Atlantic. However, either the 2011 American guidelines or the 2014 European guidelines remain largely based on expert consensus for lack of recommendations with level of evidence A regarding any of the treatment options commonly employed in HCM. Consequently, management of obstructive HCM patients remains largely subjective and dependent on clinical judgment, local expertise, and patient preference. Following the trend that has emerged for other cardiac diseases amenable to invasive interventions, adequate evaluation and management of obstruction in HCM today requires a multidisciplinary team capable of optimizing referral, choosing the best available options, minimizing complications and ensuring state-of-the-art results. The concept of an HCM Heart Team is coming of age. This review aims to provide an update of available pharmacologic and invasive options for the management of LVOTO in HCM, either in adulthood or in childhood, highlighting areas for multidisciplinary integration and future development.
•LV outflow tract gradient is a key pathophysiologic determinant of HCM.•Treatment of obstructive HCM is challenging and poses numerous clinical dilemmas.•HCM is transitioning into an exciting phase of a pharmacologic discovery.•Extensive knowledge of HCM is needed to perform septal reduction treatments.•Management of obstruction in HCM today requires a multidisciplinary heart team.
Contemporary series of postpericardiotomy syndrome (PPS) are lacking. The aim of this study was to evaluate the incidence, time course, features at presentation, risk factors, and prognosis of PPS. ...The study population consisted of 360 consecutive candidates to cardiac surgery enrolled in a prospective cohort study. PPS was diagnosed in 54 patients (15.0%; mean age 66 ± 12 years, 48.1% women): 79.6% in the first month, 13.0% in the second month, and 7.4% in the third month. Specific symptoms, signs, or features were pleuritic chest pain (55.6%), fever (53.7%), elevated markers of inflammation (74.1%), pericardial effusion (88.9%), and pleural effusion (92.6%). Cardiac tamponade was rare at presentation (1.9%). Female gender (hazard ratio 2.32, 95% confidence interval 1.22 to 4.39, p = 0.010), and pleura incision (hazard ratio 4.31, 95% confidence interval 2.22 to 8.33, p <0.001) were identified as risk factors in multivariate analysis. Patients with PPS had longer cardiac surgery stays (11.5 ± 4.6 vs 9.9 ± 4.7 days, p = 0.021) and rehabilitation stays (16.4 ± 6.7 vs 12.4 ± 6.2 days, p <0.001) and more readmissions (13.0% vs 0%, p <0.001). Adverse events after a mean follow-up period of 19.8 months were recurrences (3.7%), cardiac tamponade (<2%), but no cases of constriction. In conclusion, despite advances in cardiac surgery techniques, PPS is a common postoperative complication, generally occurring in the first 3 months after surgery. Severe complications are rare, but the syndrome is responsible for hospital stay prolongation and readmissions. Female gender and pleura incision are risk factors for PPS.
Abstract Objective To identify the exercise echocardiographic determinants of long-term functional capacity, in patients with chronic ischemic mitral regurgitation, after restrictive mitral valve ...annuloplasty (RMA) or mitral valve replacement (MVR). Methods We retrospectively analyzed 121 patients with significant chronic ischemic mitral regurgitation, who underwent RMA (n = 62) or MVR (n = 59), between 2005 and 2011. Preoperatively, all patients underwent a resting echocardiographic examination, and a 6-minute walking test (6-MWT) to measure distance. Resting and exercise stress echocardiography, and the 6-MWT were repeated at 41 ± 16.5 months. Results After surgery, the 6-MWT distance significantly improved in the MVR group, and decreased in the RMA group (+37 ± 39 m vs −24 ± 49 m, respectively; P < .0001). Exercise indexed effective orifice area was significantly higher in the MVR, versus the RMA, group (MVR: change from 1.3 ± 0.2 cm2 /m2 to 1.5 ± 0.3 cm2 /m2 ; RMA: change from 1.1 ± 0.3 cm2 /m2 to 1.2 ± 0.3 cm2 /m2 ; P = .001). The mean mitral gradients significantly increased from rest to exercise, in both groups, but to a greater extent in the RMA group (change from 4.4 ± 1.4 to 11 ± 3.6 mm Hg; MVR: change from 4.3 ± 1.8 to 9 ± 3.5 mm Hg; P = .006). On multivariate analysis, MVR and exercise indexed effective orifice area were the main independent determinants of postoperative 6-MWT. In the RMA group, 25 patients experienced late mitral regurgitation recurrence, severe in 9 (14%) of them. The rate of postoperative cardiovascular events was significantly higher in the RMA group (21% vs MVR: 8%; P = .03). Follow-up survival was 83% in the RMA group and 88% in the MVR group ( P = .54). Conclusions For chronic ischemic mitral regurgitation, MVR versus RMA was associated with better postoperative exercise hemodynamic performance and long-term functional capacity.
End-stage hypertrophic cardiomyopathy (ES-HC) has an ominous prognosis. Whether genotype can influence ES-HC occurrence is unresolved. We assessed the spectrum and clinical correlates of ...HC-associated mutations in a large multicenter cohort with end-stage ES-HC. Sequencing analysis of 8 sarcomere genes (MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, and ACTC1) and 2 metabolic genes (PRKAG2 and LAMP2) was performed in 156 ES-HC patients with left ventricular (LV) ejection fraction (EF) <50%. A comparison among mutated and negative ES-HC patients and a reference cohort of 181 HC patients with preserved LVEF was performed. Overall, 131 mutations (36 novel) were identified in 104 ES-HC patients (67%) predominantly affecting MYH7 and MYBPC3 (80%). Complex genotypes with double or triple mutations were present in 13% compared with 5% of the reference cohort (p = 0.013). The distribution of mutations was otherwise indistinguishable in the 2 groups. Among ES-HC patients, those presenting at first evaluation before the age of 20 had a 30% prevalence of complex genotypes compared with 19% and 21% in the subgroups aged 20 to 59 and ≥60 years (p = 0.003). MYBPC3 mutation carriers with ES-HC were older than patients with MYH7, other single mutations, or multiple mutations (median 41 vs 16, 26, and 28 years, p ≤0.001). Outcome of ES-HC patients was severe irrespective of genotype. In conclusion, the ES phase of HC is associated with a variable genetic substrate, not distinguishable from that of patients with HC and preserved EF, except for a higher frequency of complex genotypes with double or triple mutations of sarcomere genes.
Abstract Background Prognostic stratification in heart failure (HF) is crucial to guide clinical management and treatment decision-making. Currently available models to predict HF outcome have ...multiple limitations. We developed a simple risk stratification model, based on routinely available clinical information including comorbidities, the Cardiac and Comorbid Conditions HF (3C-HF) Score, to predict all-cause 1-year mortality in HF patients. Methods We recruited in a cohort study 6274 consecutive HF patients at 24 Cardiology and Internal Medicine Units in Europe. 2016 subjects formed the derivation cohort and 4258 the validation cohort. We entered information on cardiac and comorbid candidate prognostic predictors in a multivariable model to predict 1-year outcome. Results Median age was 69 years, 35.8% were female, 20.6% had a normal ejection fraction, and 65% had at least one comorbidity. During 5861 person-years follow-up, 12.1% of the patients met the study end-point of all-cause death (n = 750) or urgent transplantation (n = 9). The variables that contributed to outcome prediction, listed in decreasing discriminating ability, were: New York Heart Association class III–IV, left ventricular ejection fraction < 20%, no beta-blocker, no renin–angiotensin system inhibitor, severe valve heart disease, atrial fibrillation, diabetes with micro or macroangiopathy, renal dysfunction, anemia, hypertension and older age. The C statistic for 1-year all-cause mortality was 0.87 for the derivation and 0.82 for the validation cohort. Conclusions The 3C-HF score, based on easy-to-obtain cardiac and comorbid conditions and applicable to the 1-year time span, represents a simple and valuable tool to improve the prognostic stratification of HF patients in daily practice.
Objective Whether right ventricular dysfunction affects clinical outcome after coronary artery bypass grafting with or without surgical ventricular reconstruction is still unknown. The aim of the ...study was to assess the impact of right ventricular dysfunction on clinical outcome in patients with ischemic cardiomyopathy undergoing coronary artery bypass grafting with or without surgical ventricular reconstruction. Methods Of 1000 patients in the Surgical Treatment for Ischemic Heart Failure with coronary artery disease, left ventricular ejection fraction 35% or less, and anterior dysfunction, who were randomized to undergo coronary artery bypass grafting or coronary artery bypass grafting + surgical ventricular reconstruction, baseline right ventricular function could be assessed by echocardiography in 866 patients. Patients were followed for a median of 48 months. All-cause mortality or cardiovascular hospitalization was the primary end point, and all-cause mortality alone was a secondary end point. Results Right ventricular dysfunction was mild in 102 patients (12%) and moderate or severe in 78 patients (9%). Moderate to severe right ventricular dysfunction was associated with a larger left ventricle, lower ejection fraction, more severe mitral regurgitation, higher filling pressure, and higher pulmonary artery systolic pressure (all P < .0001) compared with normal or mildly reduced right ventricular function. A significant interaction between right ventricular dysfunction and treatment allocation was observed. Patients with moderate or severe right ventricular dysfunction who received coronary artery bypass grafting + surgical ventricular reconstruction had significantly worse outcomes compared with patients who received coronary artery bypass grafting alone on both the primary (hazard ratio, 1.86; confidence interval, 1.06-3.26; P = .028) and the secondary (hazard ratio, 3.37; confidence interval, 1.36-8.37; P = .005) end points. After adjusting for all other prognostic clinical factors, the interaction remained significant with respect to all-cause mortality ( P = .022). Conclusions Adding surgical ventricular reconstruction to coronary artery bypass grafting may worsen long-term survival in patients with ischemic cardiomyopathy with moderate to severe right ventricular dysfunction, which reflects advanced left ventricular remodeling.
Objective Mitral valve annuloplasty and mitral valve replacement are common strategies for the management of functional ischemic mitral regurgitation with ischemic cardiomyopathy. However, mitral ...valve annuloplasty may create some degree of functional mitral stenosis. The purpose of this study was to compare the mitral valve hemodynamics in patients with functional ischemic mitral regurgitation undergoing mitral valve annuloplasty or mitral valve replacement, using exercise echocardiography. Methods We performed resting and exercise echocardiography in 70 patients matched for indexed effective orifice area, systolic pulmonary arterial pressure, and left ventricular ejection fraction after mitral valve annuloplasty or mitral valve replacement with coronary artery bypass grafting. Results There was no significant difference between the 2 groups regarding baseline demographic and clinical data. Exercise systolic pulmonary arterial pressure was higher in the mitral valve annuloplasty group compared with the mitral valve replacement group (from 36.3 ± 8.1 mm Hg to 55 ± 12 mm Hg, vs mitral valve replacement: 33 ± 6 mm Hg to 42 ± 6.2 mm Hg, P = .0001). Exercise-induced improvement in effective orifice area and indexed effective orifice area was better in the mitral valve replacement group (mitral valve replacement: +0.23 ± 0.04 vs mitral valve annuloplasty: −0.1 ± 0.09 cm², P = .001, for effective orifice area; mitral valve replacement: +0.14 ± 0.03 vs mitral valve annuloplasty: −0.04 ± 0.07 cm²/m², P = .03, for indexed effective orifice area). Exercise indexed effective orifice area was correlated with exercise systolic pulmonary arterial pressure ( r = −0.45; P = .01). In a multivariable analysis mitral valve annuloplasty, postoperative indexed effective orifice area and resting mitral peak gradients were independent predictors of elevated systolic pulmonary arterial pressure during exercise. Conclusions In patients with functional ischemic mitral regurgitation, mitral valve annuloplasty may cause functional mitral stenosis, especially during exercise. Mitral valve annuloplasty was associated with poor exercise mitral hemodynamic performance, lack of mitral valve opening reserve, and markedly elevated postoperative exercise systolic pulmonary arterial pressure compared with mitral valve replacement.
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