Surgical septal myectomy for hypertrophic cardiomyopathy in Greece: a single-center initial experience.

INTRODUCTION: Surgical septal myectomy is thought to be the gold standard of treatment for obstructive hypertrophic cardiomyopathy (HCM) with obstruction symptoms refractory to optimal medical therapy. In Europe, during the last 2 decades, myectomy has been set aside, while alcohol septal ablation has been widely promoted. In this paper, we analyze our first experience of surgical septal myectomy in a small cohort of patients with HCM coming from a single tertiary center. METHODS: Thirty-two patients (16 male, 50%) with a mean age of 58.1 ± 14.4 (range 12-79 years) underwent myectomy for HCM symptoms refractory to negative inotropic agents. The technique used for the myectomy was the one introduced by Andrew Morrow. Mean follow-up time after procedure was 16.8 ± 13.3 months with a median of 13 months (range 4-58 months). RESULTS: Post-myectomy, there was a significant improvement in patients' NYHA class (from 3.3 ± 0.46 to 1.38 ± 0.49, p<0.0005), while interventricular septum thickness was reduced from 2.3 ± 0.4 cm to 1.6 ± 0.4 cm (p<0.0005), and peak gradient at the site of obstruction from 94.9 ± 29 to 16.7 ± 7.9 mmHg (p<0.0005). During the follow-up period, only 1 out of 32 patients died, from non-cardiovascular causes, with the overall survival post-myectomy being 97.2% (95%CI: 94.5-99.9%) at 1-year follow up. CONCLUSIONS: Surgical septal myectomy in patients with HCM and drug-refractory symptoms is a safe procedure that greatly improves symptoms and quality of life. Further follow-up of our patients is mandatory in order to determine whether the good survival rate achieved at 1 year will persist.

QRS analysis using wavelet transformation for the prediction of response to cardiac resynchronization therapy: a prospective pilot study.

BACKGROUND: Wider QRS and left bundle branch block morphology are related to response to cardiac resynchronization therapy (CRT). A novel time-frequency analysis of the QRS complex may provide additional information in predicting response to CRT. METHODS: Signal-averaged electrocardiograms were prospectively recorded, before CRT, in orthogonal leads and QRS decomposition in three frequency bands was performed using the Morlet wavelet transformation. RESULTS: Thirty eight patients (age 65±10years, 31 males) were studied. CRT responders (n=28) had wider baseline QRS compared to non-responders and lower QRS energies in all frequency bands. The combination of QRS duration and mean energy in the high frequency band had the best predicting ability (AUC 0.833, 95%CI 0.705-0.962, p=0.002) followed by the maximum energy in the high frequency band (AUC 0.811, 95%CI 0.663-0.960, p=0.004). CONCLUSIONS: Wavelet transformation of the QRS complex is useful in predicting response to CRT.

A de novo novel cardiac ryanodine mutation (Ser4155Tyr) associated with catecholaminergic polymorphic ventricular tachycardia.

We describe the case of a 14-year-old girl with a history of syncopal episodes triggered by stress or exercise. Catecholaminergic polymorphic ventricular tachycardia was diagnosed with the aid of an implantable loop recorder. The genetic testing of the patient and her family revealed a de novo novel missense mutation (Ser4155Tyr) in the exon 90 of the ryanodine receptor gene. This mutation affects a highly conserved residue (S4155) and results to replacement of serine (S) with tyrosine (Y) leading to change in physical and chemical properties. The girl was treated with an implantable defibrillator, metoprolol and flecainide. Over 1 year of follow-up she had no recurrence of ventricular tachycardia.

Quinidine: an "endangered species" drug appropriate for management of electrical storm in brugada syndrome.

Brugada syndrome is an inherited channelopathy associated with an increased risk of syncope and sudden cardiac death. In rare cases it can be manifested with electrical storm. We report two cases of Brugada syndrome that presented with electrical storm and were treated successfully with oral quinidine, an "endangered species" drug.

Clinical characteristics and natural history of hypertrophic cardiomyopathy with midventricular obstruction.

BACKGROUND: The prevalence, clinical characteristics and natural history of patients with hypertrophic cardiomyopathy (HCM) and midventricular obstruction (MVO) have not been adequately studied. METHODS AND RESULTS: A single-center cohort consisting of 423 patients (mean age, 49.3±17.2 years; 66.2% male) was thoroughly followed up for a median of 84 months (7 years; range, 6-480 months). MVO, characterized by the echocardiographic appearance of midventricular muscular apposition with a simultaneous mid-cavitary gradient ≥30mmHg, was identified in 34 patients (8%). Patients with MVO tended to be more symptomatic during their initial evaluation (>90% presented with NYHA class ≥II) compared to the rest of the HCM cohort. Apical aneurysm formation was identified in more than one-fourth of patients with MVO (26.5%), being a characteristic of the group. On multivariate Cox regression hazard analysis, presence of MVO strongly predicted progression to end-stage (burnt out) HCM and related heart failure (HF) deaths (hazard ratio, [HR], 2.62; 95% confidence interval [CI]: 1.2-8.8; P=0.047), as well as sudden death and associated lethal arrhythmic events (HR, 3.3; 95% CI: 1.26-8.85; P=0.016). CONCLUSIONS: MVO is a distinct phenotype of HCM associated with unfavorable prognosis in terms of end-stage HCM, sudden death and lethal arrhythmic events. The high adverse outcome rate necessitates early recognition of MVO and appropriate therapeutic interventions.

Increased right atrial volume index predicts low Duke activity status index in patients with chronic heart failure.

INTRODUCTION: The aim of the present study was to examine the value of the right atrial volume index (RAVI) as predictor of functional capacity in patients with heart failure. METHODS: A total of 51 patients with stable chronic heart failure of ischaemic or non-ischaemic aetiology were prospectively enrolled. The systolic function of the right ventricle was quantified using the tricuspid annular plane systolic excursion (TAPSE). Right atrial volume was measured in the apical 4-chamber view and was indexed to body surface area. The functional capacity was assessed by the Duke Activity Status Index (DASI). RESULTS: Patients with a low functional capacity (DASI<10) had lower TAPSE (1.4 ± 0.3 cm versus 2.0 ± 0.4 cm, p<0.001), higher RAVI (42 ± 15 ml/m(2) versus 22 ± 9 ml/m(2), p<0.001), higher estimated right ventricular systolic pressure (61 ± 13 mmHg versus 40 ± 16 mmHg, p<0.001), larger right ventricular end-diastolic diameter (4.7 ± 0.8 cm versus 3.6 ± 0.7 cm, p<0.001) and lower left ventricular ejection fraction (26 ± 6% versus 30 ± 7%, p=0.022). Multivariate analysis revealed that TAPSE was the single independent predictor of DASI. In the subgroup of patients with reduced right ventricular systolic function (TAPSE<2 cm), RAVI was the single independent predictor of low DASI. In the overall population RAVI=30.6 ml/m(2) had 75% sensitivity and 83% specificity in predicting DASI<10. Within the subgroup of patients with TAPSE<2 cm, RAVI=30.6 ml/m(2) had better sensitivity and specificity (79% and 90% respectively) in predicting DASI<10. CONCLUSIONS: Increased right atrial volume index predicts low functional capacity quantified by the Duke Activity Status Index in patients with stable chronic heart failure.

Patients with hypertrophic cardiomyopathy at risk for paroxysmal atrial fibrillation: advanced echocardiographic evaluation of the left atrium combined with non-invasive P-wave analysis.

AIMS: The maintenance of sinus rhythm is crucial for the functional capacity of patients with hypertrophic cardiomyopathy (HCM). Using a multimodality approach, we attempted to identify potential predictors of paroxysmal atrial fibrillation (PAF) in HCM patients. METHODS AND RESULTS: Thirty HCM patients (17 males, mean age 57.9 ± 13.6) with at least one documented PAF episode and 32 age- and sex-matched HCM control patients as well as 25 healthy volunteers were studied in sinus rhythm. Study subjects underwent 2D echocardiography including a colour Doppler myocardial imaging evaluation of the left atrium (LA). Additionally, an orthogonal electrocardiogram was acquired; P-wave duration, maximum, and mean energies were calculated for each subject at each orthogonal lead and the composite vector axis using the Morlet wavelet analysis. Compared with HCM controls, in HCM-PAF patients, LA antero-posterior diameter was significantly enlarged (LADAP: 46.1 ± 5.9 vs. 40.0 ± 4.7 mm, P < 0.001), peak strain rate of the LA lateral wall in the reservoir phase was significantly decreased (LAT peak SR-S: 1.93 ± 0.51 vs. 2.55 ± 0.83 s(-1), P < 0.01), and P-wave duration in the Z-lead was significantly prolonged (P-durZ: 106.9 ± 24.6 vs. 86.2 ± 14.3 ms, P < 0.001). Cut-off values and areas under the curve (AUCs) for individual parameters were 42.0 mm, 2.32 s(-1), and 98.8 ms and 0.81, 0.74, and 0.78, respectively. A multivariable model combining LADAP, LAT peak SR-S and P-durZ had an AUC of 0.90, a sensitivity of 0.87, and a specificity of 0.91 for identifying PAF patients. CONCLUSION: P-wave duration combined with LA antero-posterior diameter and myocardial deformation indices resulted in a higher power for discriminating HCM-PAF patients, when compared with individual parameters derived from either wavelet analysis or 2D echocardiography.

Differences in Clinical Presentation and Findings between Idiopathic Dilated and Ischaemic Cardiomyopathy in an Unselected Population of Heart Failure Patients.

We explored the differences in epidemiologic, clinical, laboratory and echocardiographic characteristics between idiopathic dilated (IDCM) and ischaemic cardiomyopathy (ICM).Consecutive patients with stable chronic heart failure evaluated at a tertiary cardiac centre were enrolled. Clinical examination, blood tests and echocardiographic study were performed. A total of 76 patients (43 IDCM, 33 ICM) were studied. IDCM patients were younger (p<0.001) and female gender was more prevalent (p=0.022). NYHA class and left ventricular ejection fraction were similar. IDCM patients had lower rates of dyslipidaemia (p<0.001) but smoked more than ICM patients (p=0.023) and had higher rates of family history of sudden cardiac death (p=0.048). Blood pressure was similar but resting heart rate was higher in IDCM patients (p=0.022). IDCM patients presented less frequently with peripheral oedema or ascites (p=0.046 and 0.020, respectively) and showed better right ventricular function on echocardiogram. QRS duration was similar between groups but only in IDCM patients there was a positive correlation between QRS duration and age (r=0.619, p<0.001). Cardiac output was similar but functional capacity assessed by the Duke Activity Status Index was better in IDCM (p=0.036). Despite these differences, IDCM and ICM patients received similar treatments. Patients with IDCM were younger, presented lower rates of right ventricular dysfunction and clinical right ventricular failure and had better functional capacity. Additional differences in clinical and laboratory findings exist pointing to a different patient population with diverse prognosis and potential need for individualized management.

Somatostatin analogue scintigraphy in a patient with viral myocarditis.

Myocarditis may present clinically with a wide range of manifestations and often remains unrecognized. The diagnosis of myocarditis traditionally has been based on histological findings, but endomyocardial biopsy has a low sensitivity and clinicians are reluctant to proceed with an invasive diagnostic technique. Among newer diagnostic approaches, cardiac magnetic resonance imaging has gained acceptance as an efficient noninvasive tool to determine myocardial inflammation. In this context, imaging with radiolabeled somatostatin analogues could also be relevant because of their ability to delineate inflammatory sites. In conclusion, a case is presented in which somatostatin receptor imaging of the myocardium with (99m)Tc-depreotide tomography was used in the assessment of viral myocarditis.

Prognostic value of right ventricular diastolic function indices in hypertrophic cardiomyopathy.

AIMS: Despite the fact that the role of left ventricular diastolic dysfunction in hypertrophic cardiomyopathy (HCM) patients' prognosis has been established, the effect of increased right ventricular (RV) diastolic filling pressures still remains unclear. The purpose of this study was to determine the prognostic significance of transthoracic echocardiographic indices of RV diastolic function (tricuspid inflow and tricuspid annulus tissue Doppler imaging) in HCM patients. METHODS AND RESULTS: We followed up 386 patients diagnosed with HCM (aged 49.3 ± 17.2 years; 65% male) for a median period of 67 months (interquartile range 26-189 months). Primary endpoints were considered mortality due to heart failure (HF) (13 patients) and total cardiovascular (TC) mortality [HF, sudden cardiac death and its equivalents (35 patients)]. Patients presenting with an increased RV E/E(r) ratio (ratio of tricuspid in flow E wave to E(r) wave obtained by tissue Doppler imaging at the lateral tricuspid annulus) had a 1.6 times greater risk for HF mortality [hazard ratio (HR): 1.6, 95% confidence interval (CI): 1.1-2.4, P = 0.03] while patients with shortened tricuspid E wave deceleration time (DTE) had a 1.1 greater risk for SCD (HR: 1.1, 95% CI: 1.01-1.2, P = 0.03). Following ROC analysis, the optimal RV indices' cut-off values for the recognition of our study endpoints were assessed [E/E(r) = 6.88, sensitivity 75%, specificity 77.4%, area under curve (AUC) 0.847, P = 0.017 for HF mortality and DTE < 239 ms, sensitivity 62.5%, specificity 56.7%, AUC 0.642, P = 0.05 for TC mortality]. CONCLUSION: The establishment of RV restrictive physiology appears to have significant predictive value in HCM, regardless of the presence of other detrimental risk factors.

Ibutilide for the Cardioversion of Paroxysmal Atrial Fibrillation during Radiofrequency Ablation of Supraventricular Tachycardias.

Direct current electrical cardioversion (DC-ECV) is the preferred treatment for the termination of paroxysmal atrial fibrillation (AF) that occurs during radiofrequency ablation (RFA) of supraventricular tachycardias (SVT). Intravenous Ibutilide may be an alternative option in this setting. Thirty-four out of 386 patients who underwent SVT-RFA presented paroxysmal AF during the procedure and were randomized into receiving ibutilide or DC-ECV. Ibutilide infusion successfully cardioverted 16 out of 17 patients (94%) within 17.37 ± 7.87 min. DC-ECV was successful in all patients (100%) within 17.29 ± 3.04 min. Efficacy and total time to cardioversion did not differ between the study groups. No adverse events were observed. RFA was successfully performed in 16 patients (94%) in the ibutilide arm and in all patients (100%) in the DC-ECV arm, p = NS. In conclusion, ibutilide is a safe and effective alternative treatment for restoring sinus rhythm in cases of paroxysmal AF complicating SVT-RFA.

Cardiac arrest caused by torsades de pointes tachycardia after successful atrial flutter radiofrequency catheter ablation.

A 66-year-old woman underwent successful radiofrequency catheter ablation for long-lasting, drug refractory fast atrial flutter. Two days later she had a cardiac arrest due to torsades de pointes (TdP) tachycardia attributed to relative sinus bradycardia and QT interval prolongation. After successful resuscitation further episodes of TdP occurred, which were treated with temporary pacing. Because of concomitant systolic dysfunction due to ischemic and valvular heart disease she was finally treated with an implantable defibrillator. In conclusion we strongly advise prolonged monitoring for 2 or more days for patients with structural heart disease following successful catheter ablation for long lasting tachyarrhythmias.

Effects of renin-angiotensin system inhibition on right ventricular function in patients with mild essential hypertension.

INTRODUCTION: Systemic hypertension is known to affect both left and right ventricular (RV) function. Little is known about the effect of the renin-angiotensin system (RAS) inhibition on global RV function in patients with essential hypertension. MATERIALS AND METHODS: Forty patients (17 male, mean age 47 ± 10 years) with mild hypertension free of cardiovascular disease were assessed by echocardiography at baseline and after nine months of antihypertensive treatment with RAS inhibitors. Tissue Doppler imaging derived myocardial performance index (MPI) of the left and right ventricle was used as an index of global ventricular function. RESULTS: Both left ventricular (LV) and RV MPI were increased at baseline and were reduced after treatment (LV MPI reduced from 0.42 ± 0.06 to 0.39 ± 0.05, p < 0.001 and RV MPI was reduced from 0.34 ± 0.06 to 0.32 ± 0.05, p < 0.005). There was a positive correlation between mitral and tricuspid E/A ratio both at baseline and at month nine after treatment (r = 0.661, p < 0.001 and r = 0.503, p < 0.005 respectively). LV mass index and interventricular septum thickness were decreased after treatment. No correlation was found between MPI improvement and blood pressure reduction. CONCLUSIONS: RAS inhibition in patients with mild hypertension results in an improvement of RV global function which is unrelated to the reduction in blood pressure.

Practice patterns of cardiologists, general practitioners, and internists for managing supraventricular tachycardias in Greece.

OBJECTIVES: Supraventricular tachycardias (SVT) often lead to emergency room and primary care visits. Not only cardiologists, but also general practitioners (GPs) and internists are involved to an increasing extent in the acute and long-term management of SVT. We aimed to explore the differences between practice patterns of cardiologists and noncardiologists with regard to SVT management in Greece. METHODS: A cross-sectional questionnaire survey was conducted among 250 cardiologists and 250 GPs/internists from various areas across Greece. RESULTS: A response rate of 61.8% was obtained. Vagal maneuvers were the initial therapeutic approach for SVT termination; however, 22% of noncardiologists would rather start with an antiarrhythmic drug. Adenosine was the most popular drug for SVT termination, but the GPs/internists would use it less often than the cardiologists (67 vs. 86%, P<0.001). The GPs/internists would keep the patient for at least 24 h or more after SVT termination, while 48% of the cardiologists would discharge the patient within the first 3 h. Noncardiologists would more often suggest a 24-h Holter recording than the cardiologists (73 vs. 55%, P<0.005). With regard to the long-term management of SVT, the GPs/internists would prescribe antiarrhythmic drugs earlier than the cardiologists, and seem to be less familiar with the indications for the electrophysiological testing and ablation. CONCLUSION: Significant differences in practice patterns exist in Greece with regard to SVT management between cardiologists and noncardiologists. The GPs/internists seem to rely more on antiarrhythmic drugs and tend to underestimate the role of ablation therapy for the long-term management of SVT.

Spontaneous dissection of right coronary artery manifested with acute myocardial infarction.

Spontaneous coronary artery dissection is a rare cause of acute ischemic coronary events and sudden cardiac death. It usually occurs in young women without traditional risk factors for coronary artery disease during pregnancy or postpartum period. However, it has also been reported in patients with atherosclerotic coronary disease. We present a case of spontaneous right coronary artery dissection in a 48-year male with recent myocardial infarction and previous percutaneous coronary intervention.

An interesting case of cardiac amyloidosis initially diagnosed as hypertrophic cardiomyopathy.

Cardiac involvement occurs frequently in primary amyloidosis and is associated with heart failure hospitalizations and poor survival. The initial presentation of the disease may be misleading, resulting in under-diagnosis of cardiac amyloidosis and late initiation of treatment. We present a case of cardiac amyloidosis initially misdiagnosed as hypertrophic cardiomyopathy and we discuss the key findings of the disease along with the latest evidence regarding the management and prognosis of cardiac amyloidosis.

The use of transtelephonic loop recorders for the assessment of symptoms and arrhythmia recurrence after radiofrequency catheter ablation.

Radiofrequency catheter ablation (RFA) is an effective treatment of arrhythmias. However, patients often remain symptomatic after the procedure. We aimed to assess the arrhythmia recurrence after successful RFA in relation to patients' symptoms using transtelephonic loop recorders. Thirty-six consecutive patients (age 50 +/- 14 years, 17 males/19 females) were enrolled after successful RFA for atrioventricular (AV) nodal reentrant tachycardia (n = 21), AV reentrant tachycardia (n = 8), atrial tachycardia (n = 2), atrial fibrillation/flutter (n = 4), and ventricular tachycardia (n = 1). During 23 +/- 6 days of follow-up, 679 events were recorded, 246 of which were true arrhythmic events, mostly (56%) asymptomatic. The vast majority of these true arrhythmic events were due to trivial arrhythmias (extrasystoles or sinus tachycardia), equally distributed among symptomatic and asymptomatic episodes. Arrhythmia relapse was shown in four patients, who had a total of nine episodes, eight of which were symptomatic. No high degree AV block was detected. Overall, symptom recurrence had low sensitivity (44%) and high specificity (95%) for the detection of any arrhythmia, and high sensitivity (89%) but low specificity (58%) for the detection of relapse. In conclusion, transtelephonic monitoring was a useful tool for the assessment of symptoms after RFA and its use may be reserved for the most symptomatic patients to detect a relapse or to reassure them for the benign nature of their symptoms.

Prevalence and clinical outcomes of incidentally diagnosed hypertrophic cardiomyopathy.

The purpose of the present study was to evaluate the prevalence and prognosis of patients incidentally diagnosed with hypertrophic cardiomyopathy (HC). We studied 380 consecutive patients with HC (49.3 +/- 17.2 years; 65% men) for a median of 58 months (range 6 to 454). The patients were divided into 2 groups: those incidentally diagnosed from routine examination findings (precordial murmur and/or abnormal electrocardiographic findings) and those diagnosed either because of symptomatic status or by screening because of a family history of HC. Those patients who had been incidentally diagnosed constituted 29.2% of our study cohort. Although overall mortality did not differ between the 2 groups (p = 0.12), the patients diagnosed either because of symptoms or a family history tended to have at least a 4.5-fold greater risk of cardiovascular death (relative risk 4.5, 95% confidence interval 1.04 to 19.6, p = 0.04) and a 4.22 greater risk of sudden death (relative risk 4.22, 95% confidence interval 1.0 to 18.22, p = 0.04). Despite the greater sudden death mortality among the nonincidentally diagnosed patients, no statistically significant difference was found concerning the sudden death risk factor frequency (p = 0.96) between the 2 groups. In conclusion, the discrepancy between the low numbers of patients reported by published registries and the relatively high prevalence of the disease in the general population can be attributed to the large number of patients who remain asymptomatic, even throughout their life, awaiting an accidental diagnosis. Those patients with an incidental diagnosis have a more benign course, as shown by the total cardiovascular and composite sudden death mortality. A high level of awareness and suspicion for HC among physicians is essential for clinical recognition of such patients.

Differences in management of atrial fibrillation between cardiologists and non-cardiologists in Greece.

INTRODUCTION: We aimed to assess trends in the management of atrial fibrillation (AF) at various levels of medical care in Greece and to compare the treatment practices of cardiologists to those of non-cardiologists. METHODS: From January to May 2007, 500 questionnaires were mailed to cardiologists, internists and general practitioners, randomly selected from regional medical associations. Questions assessed management practices for paroxysmal, persistent and permanent AF. RESULTS: A total of 309 physicians (194 cardiologists and 115 non-cardiologists) responded. Cardiologists showed no preference regarding the site of cardioversion of paroxysmal AF, whereas non-cardiologists tend to cardiovert paroxysmal AF in the emergency department. Intravenous amiodarone is the most frequently used antiarrhythmic agent for cardioversion by both groups (63% vs. 71%, p=NS). Cardiologists utilise propafenone or ibutilide more frequently than non-cardiologists (24% vs. 11%, p<0.05 and 10% vs. 2%, p<0.01 respectively), while 12% of non-cardiologists would use digitalis for cardioversion (vs. 0.5% of cardiologists, p<0.001). Cardiologists prescribe commonly, but less frequently than non-cardiologists (42% vs. 59%, p<0.01) an antiarrhythmic drug after the first episode of paroxysmal AF, propafenone being the most popular among cardiologists (66%) and amiodarone (33%) or digitalis (23%) among general practitioners/internists. Beta-blockers are considered as first choice agents for rate control among cardiologists, while non-cardiologists would prescribe mainly digitalis. Antiplatelet agents were suggested by most physicians after cardioversion of the first episode of AF in low-risk patients. Cardiologists prefer aspirin, while non-cardiologists would prescribe clopidogrel as first choice antiplatelet agent. Both groups would recommend anticoagulants in high risk patients; nevertheless, in elderly patients without other risk factors, anticoagulants are more often prescribed by cardiologists (79% vs. 50%, p<0.001). CONCLUSIONS: Important differences exist in the management of AF between cardiologists and general practitioners/internists in Greece. Non-cardiologists overuse digitalis, underuse beta-blockers, prefer clopidogrel to aspirin and are reluctant to prescribe anticoagulants in the elderly.