Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm formation in a single family: case report.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous disease. An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular (LV) apical aneurysms in the absence of coronary artery disease. CASE PRESENTATION: We describe a case of HCM with midventricular obstruction and apical aneurysm formation in 3 patients coming from a single family. This HCM pattern was detected by 2D-echocardiography and confirmed by cardiac magnetic resonance imaging. A cardioverter defibrillator was implanted in one of the patients because of non-sustained ventricular tachycardia detected in 24-h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test. The defibrillator activated 8 months after implantation by suppression of a ventricular tachycardia providing anti-tachycardia pacing. The patient died due to refractory heart failure 2 years after initial evaluation. The rest of the patients are stable after a 2.5-y follow-up period. CONCLUSION: The detection of apical aneurysm by echocardiography in HCM patients may be complicated. Ventricular tachycardia arising from the scarred aneurysm wall may often occur predisposing to sudden death.

Identification of high risk patients with hypertrophic cardiomyopathy in a northern Greek population.

BACKGROUND: The percentage of hypertrophic cardiomyopathy (HCM) patients who are in high risk for Sudden Death (SD) constitutes only a minority of all HCM population but the incidence of SD in this subset is high (at least 5% annually). The identification of this small but important proportion of high risk HCM patients has been the clue in the clinical evaluation of these patients. METHODS: Our study cohort consisted from 123 patients with HCM who are currently followed up in our Institution. Five clinical risk factors were assessed: a family history of premature SD, unexplained syncope, Non Sustained Ventricular Tachycardia (NSVT) on 24-h ECG monitoring, Abnormal Blood Pressure Response (ABPR) during upright exercise testing and Maximum left ventricular Wall Thickness (MWT) > or =30 mm. The purpose of our study was the identification of high risk HCM patients coming from Northern Greece. RESULTS: Fifteen patients (12.2%) of the whole cohort had MWT > or = 30 mm, 30 patients (24.4%) had an ABPR to exercise, 17 patients (13.8%) had episodes of NSVT in 24-h Holter monitoring, 17 patients (13.8%) suffered from syncope, and 8 patients (6.5%) had a positive family history of premature SD. Data analysis revealed that 74 patients (60.1%) had none risk factor. Twenty four patients (19.5%) had 1 risk factor, 17 patients (13.8%) had 2 risk factors, 4 patients (3.25%) had 3 risk factors, and 4 patients (3.25%) had 4 risk factors, while none patient had 5 risk factors. Twenty five patients (20.3%) had 2 or more risk factors. CONCLUSION: This study for the first time confirms that, although a 60% of patients with HCM coming from a regional Greek population are in low risk for SD, a substantial proportion (almost 20%) carries a high risk for SD justifying prophylactic therapy with amiodaron or ICD implantation.

Prognostic significance of right ventricular diastolic function in thalassaemia major.

While left ventricular (LV) restrictive filling pattern is an ominous echocardiographic finding in thalassaemia major (TM), the prognostic significance of right ventricular (RV) diastolic function in patients with TM has not been thoroughly investigated. We studied 45 TM asymptomatic transfusion-dependent patients with normal LV systolic function by Doppler echocardiography. The 15-year cumulative survival rate was 34% in patients with RV restrictive filling pattern (RFP) and 82% in patients with RV non-RFP (log-rank = 10.41, p = 0.0013). Doppler estimation of RV filling pattern is very important in evaluating the prognosis of TM patients and should be performed routinely and using a standardised followup protocol.

Diagnostic value of stored electrograms in pacemaker patients.

OBJECTIVE: Stored electrograms (EGMs) are diagnostic tools in modern pacemakers, providing data concerning arrhythmia occurrence and device function. We sought to validate the impact of stored EGMs on diagnosis and verification of arrhythmias presented after pacemaker implantation. METHODS AND RESULTS: We enrolled 71 consecutive patients (mean age 64 +/- 8 years, 51 men) with a standard indication for pacemaker implantation. The following pacemaker devices were implanted: DDDR = 57, VDDR = 5, VVIR = 9. EGM triggers were atrial tachycardia (AT), non-sustained ventricular tachycardia (NSVT) and ventricular tachycardia (VT). We retrieved and analysed 362 EGMs at 3 and 6 months after implantation. The EGMs were triggered by AT in 260 EGMs (72%), by NSVT in 80 (22%) and by VT in 22 (6%). In total, 243 episodes (67%) confirmed the arrhythmic events, while 119 episodes (33%) were classified as false-positive. They were divided into false-positive AT in 85 EGMs (33%), false-positive NSVT in 28 (35%) and false-positive VT in 6 EGMs (27%). The false-positive AT cases were caused by ventricular far-field sensing from the atrial channel in 34 EGMs (40%) and noise or myopotentials in 51 EGMs (60%). Most of the false-positive VT and NSVT episodes were due to atrial fibrillation or atrial tachycardias in 21 EGMs (62%), while the rest were caused by noise in 11 EGMs (32%) or ventricular T wave oversensing in 2 EGMs (6%). CONCLUSIONS: Stored EGMs in pacemaker patients were diagnostic in two thirds of arrhythmic episodes. They are useful tools to diagnose arrhythmias and identify sensing problems, and they contribute to optimal device programming and patient management.

The utility of the standard 12-lead electrocardiogram in the prediction of proximal right coronary artery occlusion in acute inferior myocardial infarction.

Prior studies have proposed several electrocardiogram criteria for identifying patients with acute inferior ST-segment elevation myocardial infarction (iSTEMI) caused by obstruction of the proximal part of the right coronary artery (RCA). We applied 11 of these criteria and three new ones to the admission electrocardiograms of 80 patients admitted with an acute iSTEMI in order to evaluate their utility. All patients received thrombolytic treatment and underwent coronary angiography during the hospitalization. Four previously described criteria (ST-segment depression in lead V1, ST-segment depression in leads V1-V3, maximum ST-segment depression in the precordial leads, and ST-segment depression in lead V3 of 1) were useful in identifying patients with obstruction of the proximal part of the RCA. Among the six criteria, ST depression in V1-V3 had the highest specificity (77.2%) and positive predictive value (56.5%), and a new criterion-the arithmetic sum of the ST-elevation in V3/ST-elevation in III < 0.5--had the highest sensitivity (80.9%) and negative predictive value (86.7%). Six criteria were helpful in identifying patients with acute iSTEMI caused by obstruction of the proximal part of the RCA. One of these has not been previously reported and has the higher specificity and negative predictive value.

Subaortic and midventricular obstructive hypertrophic cardiomyopathy with extreme segmental hypertrophy.

BACKGROUND: Subaortic and midventricular hypertrophic cardiomyopathy in a patient with extreme segmental hypertrophy exceeding the usual maximum wall thickness reported in the literature is a rare phenomenon. CASE PRESENTATION: A 19-year-old man with recently diagnosed hypertrophic cardiomyopathy (HCM) was referred for sudden death risk assessment. The patient had mild exertional dyspnea (New York Heart Association functional class II), but without syncope or chest pain. There was no family history of HCM or sudden death. A two dimensional echocardiogram revealed an asymmetric type of LV hypertrophy; anterior ventricular septum = 49 mm; posterior ventricular septum = 20 mm; anterolateral free wall = 12 mm; and posterior free wall = 6 mm. The patient had 2 types of obstruction; a LV outflow obstruction due to systolic anterior motion of both mitral leaflets (Doppler-estimated 38 mm Hg gradient at rest); and a midventricular obstruction (Doppler-estimated 43 mm Hg gradient), but without apical aneurysm or dyskinesia. The patient had a normal blood pressure response on exercise test and no episodes of non-sustained ventricular tachycardia in 24-h ECG recording. Cardiac MRI showed a gross late enhancement at the hypertrophied septum. Based on the extreme degree of LV hypertrophy and the myocardial hyperenhancement, an implantation of a cardioverter-defibrillator was recommended prophylactically for primary prevention of sudden death. CONCLUSION: Midventricular HCM is an infrequent phenotype, but may be associated with an apical aneurysm and progression to systolic dysfunction (end-stage HCM).

Balloon valvuloplasty as destination therapy in elderly with severe aortic stenosis: a cardiac catheterization study.

BACKGROUND: In the current era of transcatheter aortic valve replacement, there is renewed interest in balloon aortic valvuloplasty (BAV) and invasive hemodynamic evaluation of aortic stenosis (AS). The current report aimed to study the invasive hemodynamics of severe AS patients treated with BAV as destination therapy and to identify factors associated with better hemodynamic outcome and prognosis. METHODS: From 2009 to 2012, 63 high risk elderly patients were treated with BAV as destination therapy for symptomatic severe AS and were all prospectively included in the study. Their hemodynamics were invasively evaluated during catheterization, pre- and post-BAV at the same session. All Post-BAV patients were regularly followed-up. RESULTS: The patients (82 ± 6 years, 52% male) had post-BAV aortic valve area index (AVAi) significantly increased and mean pressure gradient (MPG) significantly reduced. During the follow-up of 0.9 (maximum 3.3) years, those with post-BAV AVAi < 0.6 cm(2)/m(2) compared with the AVAi ≥ 0.6 cm(2)/m(2) group had significantly higher mortality (60% vs. 28%, log-rank P = 0.02), even after adjusting for age, gender, atrial fibrillation, chronic kidney disease, diabetes mellitus, coronary artery disease and EuroSCORE [HR: 5.58, 95% confidence interval (CI): 1.62-19.20, P = 0.006]. The only independent predictor of moderate AS post-BAV was the pre-BAV AVAi increase by 0.1cm(2)/m(2) (OR: 3.81, 95% CI: 1.33-10.89, P = 0.01). Pre-BAV AVAi ≥ 0.39 cm(2)/m(2) could predict with sensitivity 84% and specificity 70% the post-BAV hemodynamic outcome. CONCLUSIONS: BAV as destination therapy for severe AS offered immediate and significant hemodynamic improvement. The survival was significantly better when a moderate degree of AS was present.

Clinical profile and therapeutic management of patients with atrial fibrillation in Greece: results from the Registry of Atrial Fibrillation to Investigate New Guidelines (RAFTING).

INTRODUCTION: Atrial fibrillation (AF) is the most common arrhythmia with significant morbidity, including a 5-fold increase in stroke risk. The management of AF, including antithrombotic therapy (AT), varies considerably among countries. Representative data concerning AF features and management in Greece are generally lacking. METHODS: The Registry of Atrial Fibrillation To Investigate New Guidelines (RAFTING) is a country-wide prospective observational study of AF in Greece that enrolled consecutive patients with a diagnosis of AF in emergency departments of 31 hospitals of different types according to the population's geographical distribution. RESULTS: RAFTING enrolled 1127 patients, 51% females, aged 71 ± 12 years. Paroxysmal AF was present in 54% of patients and newly diagnosed AF in 28%; 68% of patients with a previous AF history had undergone a median of 4 cardioversions. A high rate of comorbidities was present, including arterial hypertension in 75% and heart failure in 40%. The median CHADS2 and CHA2DS2VASc scores were 2 and 3, respectively; AT had been prescribed in 87% of non-newly diagnosed patients, with warfarin being prescribed in 56% of them. Among all patients on warfarin, INR values were within therapeutic range in 34% of cases during inhospital measurement. Hospital admission occurred in 82% of cases, with in-hospital mortality 0.8%. CONCLUSIONS: RAFTING provides updated insights into the current features and management of AF in Greece. The majority of patients have a sufficiently high risk to warrant oral anticoagulation and further attempts to comply with the existing guidelines are warranted.

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.

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.

Chronotropic incompetence and its relation to exercise intolerance in hypertrophic cardiomyopathy.

BACKGROUND: Diminished functional capacity is common in hypertrophic cardiomyopathy (HCM), although the underlying mechanisms are complicated. We studied the prevalence of chronotropic incompetence and its relation to exercise intolerance in patients with HCM. METHODS: Cardiopulmonary exercise testing was performed in 68 patients with HCM (age 44.8 ± 14.6 years, 45 males). Chronotropic incompetence was defined by chronotropic index (heart rate reserve)/(220-age-resting heart rate) and exercise capacity was assessed by peak oxygen consumption (peak Vo(2)). RESULTS: Chronotropic incompetence was present in 50% of the patients and was associated with higher NYHA class, history of atrial fibrillation, higher fibrosis burden on cardiac MRI, and treatment with ß-blockers, amiodarone and warfarin. On univariate analysis, male gender, age, NYHA class, maximal wall thickness, left atrial diameter, peak early diastolic myocardial velocity of the lateral mitral annulus, history of atrial fibrillation, presence of left ventricular outflow tract obstruction (LVOTO) at rest, and treatment with beta-blockers were related to peak Vo(2). Peak heart rate during exercise, heart rate reserve, chronotropic index, and peak systolic blood pressure were also related to peak Vo(2). On multivariate analysis male gender, atrial fibrillation, presence of LVOTO and heart rate reserve were independent predictors of exercise capacity (R(2) = 76.7%). A cutoff of 62 bpm for the heart rate reserve showed a negative predictive value of 100% in predicting patients with a peak Vo(2) <80%. CONCLUSIONS: Blunted heart rate response to exercise is common in HCM and represents an important determinant of exercise capacity.

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 coronary artery dissection and acute myocardial infarction during pregnancy.

Spontaneous coronary artery dissection is a rare cause of infarction in the general population, but a common cause of myocardial infarction during pregnancy, and even nowadays carries a high risk of mortality. We describe the case of a 31-year-old primigravida who presented during the 34th week of pregnancy with the chief complaint of chest pain and was found to suffer from myocardial infarction. She was otherwise healthy and had no known risk factors for coronary artery disease. The patient was treated conservatively with nitrates, aspirin, heparin and beta-blockers, eventually sustained a large myocardial infarction and, after an initial period of instability, remained asymptomatic. A week later she delivered 3 healthy babies. A coronary angiogram performed 3 months after the initial event revealed an extensive obstructive dissection of the circumflex coronary artery. The decision whether to pursue percutaneous coronary intervention, surgical revascularisation or appropriate medical therapy, is based on clinical presentation, the extent of the dissection, and the amount of ischaemic myocardium at risk. Cardiologists must be aware of this rare entity during pregnancy or postpartum, since early diagnosis and treatment are crucial for the survival of the mother and the foetus.

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.

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.

Effects of renin-angiotensin system inhibition on left atrial function of hypertensive patients: an echocardiographic tissue deformation imaging study.

BACKGROUND: Although renin-angiotensin system (RAS) inhibitors have beneficial effects on left ventricular myocardium, their effect on left atrial (LA) function remains unknown. The aim of this study was to evaluate the effect of treatment with RAS inhibitors on LA function of patients with essential hypertension. METHODS: Forty hypertensive patients (17 males, mean age 47.1 +/- 1.5, mean blood pressure 158.3 +/- 1.8/97.1 +/- 0.7 mm Hg) were studied using LA strain and strain rate (SR) imaging before and after 9 months of treatment with angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs). RESULTS: Standard echocardiographic parameters of LA function (LA volumes, ejection fraction, active and passive emptying fraction, and ejection force), as well as left ventricular diastolic indexes did not change with RAS-blocking treatment. However, peak systolic LA strain and SR were significantly higher at study end compared to baseline (77.8 +/- 5.2% vs. 63.3 +/- 4.1%, P < 0.001 and 3.9 +/- 0.2 s(-1) vs. 3.1 +/- 0.2 s(-1), P < 0.0001, respectively). No correlation was found between changes in systolic or diastolic blood pressure, and changes in strain or SR change during treatment. CONCLUSIONS: LA strain and SR imaging improved after reduction of blood pressure with RAS inhibitors in hypertensive patients, whereas standard LA echocardiographic parameters remained unchanged. LA strain/SR values may have a role in detecting subclinical myocardial involvement in essential hypertension at an early stage; the association between change in these indexes after antihypertensive treatment and clinical outcome merits further evaluation.