A challenging pacemaker implantation in the presence of a giant right coronary aneurysm compressing the right atrium.

Giant coronary artery aneurysm is an uncommon disease, treated with surgical intervention or percutaneous coil embolization. A thrombosed aneurysm can cause extrinsic compression on the cardiac chambers, with potential hemodynamic effects and may cause problems when we need to implant a cardiac device. We present a case of difficult pacemaker implantation in a patient with 3 syncopes, first-degree AV block and complete left bundle branch block on electrocardiogram. The patient presented a giant aneurysm of the right coronary artery (85 x 90 mm), thrombosed, with right atrial compression. The pacemaker implantation was hampered by the difficulty of passing the lead through the compressed right atrium; indeed, only with simultaneous echocardiographic and fluoroscopic guidance, was it possible to complete the procedure. This case demonstrates the utility of echocardiogram, in particular settings, in cardiac stimulation procedures.

Takotsubo syndrome and pheochromocytoma: an insidious combination.

Pheochromocytoma is a rare adrenal tumor characterized by the secretion of catecholamines and vasoactive peptides. It can cause a catecholaminergic storm and lead to acute coronary syndromes. We present the case of a 53-year-old man, without any medical history, who arrived to the hospital following a spinal trauma due a fall. He presents back and retrosternal pain, with a clinical status of acute pulmonary edema, sinus tachycardia with left bundle branch block, left ventricular apical ballooning with depressed systolic function. Blood tests show a very important increase of Troponin and transaminases. A contrast chest-abdomen CT highlighted a right adrenal solid mass, with a diameter of 78mm, partial capsular laceration, compression of the inferior vena cava and the hepatic parenchyma. The clinical condition of the patient rapidly worsens from a respiratory and hemodynamic point of view, with cardiogenic shock, anuria and sepsis, refractory to all the medical treatments, until the patient died. The autopsy confirmed that the abdominal mass was a pheochromocytoma, broken after the trauma suffered. The resulting catecholaminergic storm caused a myocardial ischemia with Takotsubo syndrome, with cardiogenic shock. This unfortunate case confirms the pheochromocytoma as important risk factor for the onset of Takotsubo syndrome, and the how dramatic and severe a catecholaminergic storm can be.

Therapeutic management of ventricular arrhythmias in Andersen-Tawil syndrome.

Andersen-Tawil Syndrome (ATS) is a rare periodic paralysis with typical skeletal and neuromuscular features. Cardiac involvement may range from asymptomatic ventricular arrhythmias to sudden death. Its management remains challenging and the choice between antiarrhythmic drug therapy and implantable cardioverter defibrillator (ICD) is not simple. We present a case of ATS patient with episodes of bidirectional ventricular tachycardia, well controlled by flecainide therapy initially, which in particular conditions of fever and hypokaliemia had a cardiac arrest with ventricular fibrillation, with neurological sequelae and need of an ICD implant. A review of the therapeutic management of this disease is presented.

Clinical Spectrum and Outcome of Patients With Non-ST-Segment Elevation Acute Coronary Syndrome and No Obstructive Coronary Atherosclerosis.

BACKGROUND: Because approximately 10% of patients with no-ST-segment elevation acute coronary syndrome (NSTE-ACS) show no obstructive coronary artery disease (NOCAD) on angiography, we assessed the spectrum of diagnoses and the predictors of outcome of these patients. METHODS AND RESULTS: We studied 178 patients admitted to a coronary care unit with an initial diagnosis of NSTE-ACS, based on clinical, ECG and laboratory data, but found to have NOCAD. The final diagnosis in these patients was heterogeneous; true NSTE-ACS (ie, coronary thrombosis on an unstable plaque) was ascertained in 1 patient (0.6%), whereas diagnosis at discharge was microvascular NSTE-ACS in 56.2% of patients, variant angina in 10.1%, myocarditis in 8.9%, takotsubo disease in 7.9%, tachyarrhythmia-related chest pain in 6.7%, and non-cardiac pain in 9.6%. At 24.5-month follow-up, 21 deaths (11.8%) had occurred, 9 (5.1%) from cardiovascular causes, including 2 (1.12%) coronary deaths. By multivariable Cox analysis, age only predicted global (hazard ratio [HR] 1.07 [1.02-1.12]; P=0.006) and cardiovascular (HR 1.08 [1.01-1.16]; P=0.04) mortality; non-coronary vascular disease was the main predictor of cardiovascular death or readmission for cardiovascular disease (HR 3.28 [1.75-6.14]; P<0.001) and coronary death or readmission for angina (HR 3.20 [1.26-8.14]; P=0.014). CONCLUSIONS: Patients with an initial diagnosis of NSTE-ACS constitute a heterogeneous population with different final diagnoses. Patients have a rather high rate of fatal events, most of which, however, are not related to coronary causes. (Circ J 2016; 80: 1600-1606).

Determinants of heart rate turbulence in individuals without apparent heart disease and in patients with stable coronary artery disease.

AIMS: To assess the characteristics and determinants of heart rate turbulence (HRT) in individuals without any apparent heart disease and in patients with coronary artery disease (CAD). METHODS AND RESULTS: Heart rate turbulence parameters, turbulence onset (TO), and turbulence slope (TS) were calculated on 24 h electrocardiogram recordings in 209 individuals without any heart disease (group 1) and in 157 CAD patients (group 2). In group 1, only age independently predicted abnormal TO (≥0%) [odds ratio (OR), 1.05; P<0.001], while predictors of abnormal TS (≤2.5 ms/RR) were age (OR, 0.85; P < 0.001) and hypertension (OR, 0.19; P = 0.028). In group 2 patients, only age independently predicted TO (OR, 1.03; P = 0.038), while age (OR, 0.90; P = 0.001) and left ventricular ejection fraction (LVEF; OR, 1.07; P = 0.008) predicted TS. Heart rate turbulence values were different in groups 1 and 2. Turbulence onset was (mean, standard deviation) -1.80 ± 2.24 vs. -0.73 ± 1.61%, respectively (P < 0.001), whereas TS was (median, interquartile interval) 5.83 (3.25-10.55) vs. 2.93 (1.73-5.81) ms/RR, respectively (P < 0.001). Coronary artery disease group, however, did not predict abnormal HRT parameters in multivariable analyses, both in the whole population and when comparing two subgroups matched for age and gender. Age and (for TS) LVEF, indeed, were the only independent predictors of abnormal HRT. CONCLUSIONS: Age is a major HRT determinant both in subjects without any apparent heart disease and in stable CAD patients. Hypertension and LVEF contribute independently to HRT in these two groups, respectively. Coronary artery disease group was not by itself associated with abnormal HRT parameters in multivariable analyses.

Ultrasound-guided Axillary Vein Puncture for Cardiac Device Implantation: A Safe and Effective Approach.

Ultrasound-guided axillary vein access is an effective alternative to conventional subclavian and cephalic access for cardiac implantable electronic device implantation. The aim of this study was to compare the safety, efficacy, and radiation exposure data of the ultrasound-guided axillary approach with other conventional access techniques. The study population included 130 consecutive patients, stratified as 65 (64% male; median age, 79 years) in the study group and 65 (66% male; median age, 81 years) in the control group. We performed a retrospective not-randomized analysis by comparing ultrasound-guided axillary vein puncture with subclavian and cephalic approaches in order to test the effect on X-ray exposure, total procedure time, and complications. Significant differences were observed in terms of radiation exposure, including fluoroscopy time (median, 95 s [study group] vs. 193 s [control group]; P < .001), air kerma (median, 29 mGy [study group] vs. 55.7 mGy [control group]; P < .001), and dose-area product (median, 8219 mGy·cm2 [study group] vs. 16736 mGy·cm2 [control group]; P < .001). The median procedure time was 45 min in the study group but 50 min in the control group (P < .05). Complications occurred in 6 control group patients (1 urticaria contrast medium-related, 3 pneumothorax, 2 subclavian artery puncture) and 2 study group patients (2 axillary artery puncture). We conclude that the ultrasound-guided axillary venous approach is a fast, feasible, and safe technique for cardiac lead implantation. It allows a significant reduction in fluoroscopy time without prolonging the procedural time. This approach offers direct visualization of the vessel during the puncture, so it can be useful in patients who cannot receive contrast medium, those who require "difficult" thoracic approaches (emphysema, too much or too little fat tissue), or those on anticoagulant therapy.

T-wave alternans in apparently healthy subjects and in different subsets of patients with ischaemic heart disease.

AIMS: Microvolt T-wave alternans (TWA) predicts arrhythmic risk in patients with ischaemic heart disease (IHD). While TWA has widely been assessed by the spectral method, it has been poorly characterized in healthy people as well as in IHD patients by the modified moving average (MMA) method. METHODS AND RESULTS: We enrolled 729 consecutive subjects, referred for exercise stress test (EST). T-wave alternans was assessed by the MMA method, considering all 12 electrocardiogram (ECG) leads (TWA_tot) or the 6 ECG pre-cordial leads only (TWA_prec). Patients were divided into five groups: (i) no history of IHD and normal EST (Group 1); (ii) no history of IHD but positive EST (Group 2); (iii) ischaemic heart disease without any acute myocardial infarction [AMI (Group 3)]; (iv) old AMI (Group 4); (v) recent AMI (Group 5). T-wave alternans values >95th percentile of those measured in Group 1 were considered 'abnormal'. The 95th percentile of TWA values in Group 1 was 75 µV for TWA_tot and 65 µV for TWA_prec. T-wave alternans values and prevalence of abnormal TWA increased from Groups 1-2 to Group 5 (P< 0.00001 for both). Group 4 and Group 5, compared with Group 1, showed a significant higher prevalence of abnormal values of TWA_tot [odds ratio (OR) 1.70 (P= 0.002), and 2.07 (P= 0.01), respectively] and TWA_prec [OR 1.51 (P= 0.02) and 2.37 (P= 0.003), respectively] at multivariable analysis. In IHD patients EST-induced ischaemia did not influence TWA; in AMI patients, impaired left ventricular function was associated with higher TWA values. CONCLUSIONS: In healthy people, TWA_tot and TWA_prec were ≤75 and ≤65 µV, respectively, in 95% of subjects. In IHD patients TWA values were higher compared with healthy individuals; a history of AMI was independently associated with abnormal TWA values.

Prevalence and clinical correlates of early repolarization and J wave in a large cohort of subjects without overt heart disease.

BACKGROUND: Recent studies have suggested that early repolarization (ER) is associated with increased risk of ventricular tachyarrhythmias. Early repolarization in these studies, however, was defined as J-wave (terminal QRS slurring or notching) or J-point elevation rather than typical ST-segment elevation (STE). Prevalence and characteristics of these different findings in the general population are poorly known. In this study, we assessed prevalence and correlates of STE typical of ER and of J wave in a large population of noncardiac subjects. METHODS: We prospectively collected electrocardiograms of 4176 consecutive subjects without heart disease at our hospital. RESULTS: Early repolarization was found in 84 subjects (2.0%) and J wave in 663 (15.9%). Among ER subjects, a J wave was present in 60 (71.4%). Variables independently associated with both ER and J wave included young age, male sex, and lower heart rate. There was no increased history of symptoms (palpitations and syncope) possibly related to arrhythmias in STE or J-wave subjects. CONCLUSIONS: Typical ER pattern and J wave are common in noncardiac subjects, particularly in young people, and are not associated with symptoms potentially related to arrhythmias.

Relationship between cardiac autonomic function and sustained ventricular tachyarrhythmias in patients with an implantable cardioverter defibrillators.

AIMS: Low left ventricular ejection fraction (LVEF) is the main indication of implantable cardioverter defibrillators (ICD) in patients with dilated cardiomyopathy (DCM) for the primary prevention of sudden cardiac death, but ICD therapy at follow-up occurs in a minority of patients. We investigated whether heart rate variability (HRV) may improve risk stratification in DCM patients. METHODS AND RESULTS: We studied 42 patients (age 67.3 ± 3.5; 37 males) who had undergone ICD implant for either idiopathic or ischaemic DCM (LVEF <40%) 34.6 ± 19.7 months prior to the study (range 6-84). Patients underwent 24 h electrocardiographic Holter monitoring, and HRV was assessed over 2 hours in the afternoon showing stable sinus rhythm. Left ventricular ejection fraction was measured by two-dimensional echocardiography. The serum levels of C-reactive protein and N-terminal pro-B-type natriuretic peptide (NT-proBNP) were also obtained. The primary endpoint was the occurrence of appropriate ICD shocks in the 6 months preceding the study. The occurrence of appropriate ICD discharge from ICD implant was considered as a secondary endpoint. In the last 6 months, appropriate ICD shocks had occurred in seven patients (17%). There were no differences between patients with and without ICD shocks in clinical variables, as well as in LVEF and in C-reactive protein and NT-proBNP serum levels. In contrast, most HRV parameters were significantly depressed in patients with, compared with those without, ICD shocks; the most significant difference was shown for the average of the standard deviations of RR intervals in all consecutive 5 min segments (n » 12) within the 2 h (26.7 ± 9 vs. 39.7 ± 14 ms; P = 0.02) in the time domain and for LF amplitude (8.4 ± 3 vs. 14.8 ± 7 ms; P = 0.02) in the frequency domain. Implantable cardioverter defibrillator discharge had occurred in 11 patients (26%) since ICD implant (average 35 months). No clinical or laboratory variable showed significant differences between patients with or without ICD discharge, except very low-frequency (VLF) amplitude (23.8 ± 7 vs. 30.8 ± 10.6 ms, respectively; P = 0.049). CONCLUSION: In ICD patients with reduced LVEF, several depressed HRV indices were significantly associated with appropriate ICD shocks in the previous 6 months, and VLF amplitude was the only variable significantly associated with ICD shocks recorded since ICD implant. These data suggest that full HRV analysis might be helpful for improving risk stratification for life-threatening ventricular arrhythmias and ICD indication in patients with DCM.

Exercise stress test results in patients with bare metal stents or drug-eluting stents: pathophysiological and clinical implications.

BACKGROUND: Drug-eluting stents (DES) have reduced restenosis following percutaneous coronary intervention (PCI), but they seem to be associated with increased coronary endothelial dysfunction compared to bare metal stents (BMS). No data are available about the prognostic value of exercise stress test (EST) in PCI patients in the DES era. METHODS AND RESULTS: The 160 patients with coronary artery disease (CAD) who underwent PCI with either BMS (n=86) or DES (n=74) were studied. EST was performed 1 month after PCI. DES patients had a higher rate of positive EST compared to BMS patients (49% vs 32%; P=0.03). At a median follow-up of 18 months DES showed a lower rate of target vessel revascularization (TVR) (hazard ratio (HR) 0.37, P=0.07), but a higher rate of acute myocardial infarction (AMI) (HR 3.33, P=0.08). At multivariate Cox-regression time to 1 mm ST and low-workload ischemia were independent predictors of AMI (HR 0.96, P=0.03; and HR 6.24, P=0.009, respectively), as well as of TVR (HR 0.96, P=0.007; and HR 6.43, P=0.001, respectively). CONCLUSIONS: DES implantation is associated with a higher rate of positive EST, compared to BMS, 1 month after PCI, likely due to a higher prevalence of endothelial dysfunction. EST seems to be helpful in predicting clinical outcome in patients with coronary stent implantation.

Myocardial scar location as detected by cardiac magnetic resonance is associated with the outcome in heart failure patients undergoing surgical ventricular reconstruction.

OBJECTIVES: Post-infarction myocardial scar causes adverse left ventricular remodelling and negatively affects the prognosis. We sought to investigate whether scar extent and location obtained by cardiac magnetic resonance may affect the reverse remodelling and survival of heart failure patients undergoing surgical ventricular reconstruction. METHODS: From January 2011 to December 2015, 151 consecutive patients with previous myocardial infarction and left ventricular remodelling underwent surgical ventricular reconstruction at our Institution, of which 88 (58%) patients had a preoperative protocol-standardized late gadolinium enhancement (LGE)-cardiac magnetic resonance examination during the week before surgery. We excluded 40 patients with devices (26%), 15 patients with irregular heart rhythm (permanent atrial fibrillation, 10% not included in the device group) or mixed contraindications (severe claustrophobia or presence of material magnetic resonance not compatible). Among the 145 survivors, 11 patients received an implantable cardioverter defibrillator after surgery (mostly for persistent low ejection fraction) and were excluded as well, yielding a total of 59 patients (48 men, aged 65 ± 9 years) who repeated a protocol-standardized LGE-cardiac magnetic resonance examination even 6 months postoperatively and therefore represent the study population. Patients were grouped according to the presence of LGE in the antero-basal left ventricular segments (Group A) or the absence of LGE in the same segments (Group B). The postoperative left ventricular end-systolic volume index was considered the primary end-point. RESULTS: After surgery, left ventricular end-systolic volume index and end-diastolic volume index significantly decreased (P < 0.001, for both), while diastolic sphericity index and ejection fraction significantly increased (P = 0.015 and P < 0.001, respectively). The presence of LGE in the antero-basal left ventricular segments (10 patients, Group A) was the only independent predictor of outcome (P = 0.02) at multivariate analysis, being the postoperative left ventricular end-systolic volume index significantly higher compared to that of patients of Group B (49 patients) (78 ± 26 ml/m2 vs 55 ± 20 ml/m2, P = 0.003). Furthermore, patients with a postoperative left ventricular end-systolic volume index >60 ml/m2 showed a higher risk of cardiac events (hazard ratio = 3.67, P = 0.02). CONCLUSIONS: In patients undergoing surgical ventricular reconstruction, LGE scar location affects the left ventricular reverse remodelling, which in turn might limit the survival benefit.

Clinical correlates and prognostic value of flow mediated dilation in patients with non-ST segment elevation acute coronary syndromes.

Endothelial dysfunction can predict cardiovascular outcomes in several populations of patients. The aim of this study was to assess the severity, time course, and clinical implications of endothelial dysfunction in patients with non-ST-segment elevation (NSTE) acute coronary syndromes (ACS). Sixty patients with NSTE ACS (mean age 62 ± 8 years, 44 men) and 40 controls with stable coronary artery disease (CAD) (mean age 63 ± 10 years, 27 men) were studied. In patients with NSTE ACS and in those with stable CAD, endothelial function was assessed <12 hours after admission and at 3-month follow-up by measuring right brachial artery dilation after 5 minutes of forearm ischemia (flow-mediated dilation [FMD]). Clinical outcomes were assessed after a median follow-up period of 32 months (range 14 to 36). The primary end point was a combination of cardiac death or readmission for new ACS or recurrence of angina pectoris. FMD on admission was significantly lower in patients with NSTE ACS compared to those with stable CAD (2.1 ± 1.2% vs 4.8 ± 1.9%, p <0.001). FMD improved significantly at 3-month follow-up in patients with NSTE ACS, becoming comparable to that in patients with stable CAD (5.7 ± 2.6% vs 5.5 ± 1.7%, p = 0.93). During follow-up, 14 cardiac events (23%) occurred in patients with NSTE ACS. On multivariate analysis, only diabetes (hazard ratio 18.1, 95% confidence interval 3.9 to 83.9, p <0.001) and FMD at 3 months (hazard ratio 0.78, 95% confidence interval 0.61 to 0.99, p = 0.04) were independent predictors of the primary end point in patients with NSTE ACS. In conclusion, endothelial function is markedly impaired in the acute phase of NSTE ACS but improves significantly at 3-month follow-up. In patients with NSTE ACS, FMD at 3 months after the acute event is a significant independent predictor of cardiac outcomes.

Evidence of increased platelet reactivity in the first six months after acute ST segment elevation myocardial infarction.

INTRODUCTION: Platelets play a crucial role in the pathogenesis of acute coronary syndromes. Accordingly, previous studies showed increased platelet reactivity on admission in these patients. In this study we assessed platelet reactivity at short-medium term follow-up in patients with ST-segment elevation acute myocardial infarction (STEMI). MATERIALS AND METHODS: Fifty-nine patients (58 ± 11 years, 45 men), treated with primary angioplasty, were studied 1 month after STEMI. Thirty-five patients were retested at 6 months. Twenty matched patients with stable coronary artery disease served as controls. Platelet reactivity was assessed by flow cyometry at rest and at peak exercise, with and without adenosine diphosphate (ADP) stimulation, by measuring monocyte-platelet aggregates (MPAs) and glycoprotein IIb/IIIa (CD41) expression in the MPA gate, and CD41 and fibrinogen receptor (PAC-1) expression in the platelet gate. RESULTS: Compared to controls, basal MPAs and CD41 in the MPA gate were higher in STEMI patients both at 1 month (p = 0.001 and p = 0.002, respectively) and at 6 months (p = 0.03 and p = 0.01, respectively). Basal CD41 and PAC-1 expression was also higher in STEMI patients at the two assessments compared to controls (P<0.001 for both). Exercise induced a similar increase in platelet reactivity in patients and controls. ADP induced a higher increase in CD41 platelet expression in STEMI patients compared to controls both at 1 and 6 months (P < 0.001). CONCLUSION: Platelet reactivity is increased in the first 6 months after STEMI. The persistence of increased platelet reactivity in this time period may play a role in the early recurrence of coronary events after STEMI.

Relation between cardiovascular risk factors and coronary microvascular dysfunction in cardiac syndrome X.

BACKGROUND: The causes of coronary microvascular dysfunction (CMVD) in patients with cardiac syndrome X (CSX) are largely unknown. Common cardiovascular risk factors (CVRFs) and increased markers of inflammation have been associated with CMVD in some studies, but their role in determining CMVD in CSX patients remains poorly known. METHODS AND RESULTS: We studied 71 CSX patients (56 ± 9 years, 23 men) and 20 healthy volunteers (52 ± 7 years, nine men). Using transthoracic Doppler echocardiography, coronary microvascular vasodilator function was assessed in the left anterior descending coronary artery as the ratio of diastolic coronary blood flow (CBF) velocity at peak intravenous adenosine administration and during cold pressor test (CPT) to the respective basal CBF velocity values. Common CVRFs tended to be more frequent and C-reactive protein (CRP) levels were higher (P < 0.001) in CSX patients than in controls. Both CBF responses to adenosine (2.05 ± 0.6 vs. 2.92 ± 0.9, P < 0.001) and to CPT (1.71 ± 0.6 vs. 2.42 ± 0.7, P < 0.001) were lower in CSX patients than in controls. The differences between the two groups in CBF response to adenosine and in CBF response to CPT remained highly significant (P < 0.01 for both) after adjustment for all CVRFs, including serum CRP levels. CONCLUSION: In CSX patients, both endothelium-dependent and endothelium-independent CMVD cannot be reliably predicted by CVRFs (including serum CRP levels), alone or in combination.

Usefulness of impairment of cardiac adrenergic nerve function to predict outcome in patients with cardiac syndrome X.

Patients with cardiac syndrome X (CSX) have an excellent long-term prognosis, but a significant number show worsening angina over time. Previous studies have found a significant impairment of cardiac uptake of iodine-123-meta-iodobenzylguanidine (MIBG) on myocardial scintigraphy, indicating abnormal function of cardiac adrenergic nerve fibers. The aim of this study was to assess whether cardiac MIBG results can predict symptomatic outcome in patients with CSX. Cardiac MIBG scintigraphy was performed in 40 patients with CSX (mean age 58 ± 5 years, 14 men). Cardiac MIBG uptake was measured by the heart/mediastinum uptake ratio and a single photon-emission computed tomographic regional uptake score (higher values reflected lower uptake). Clinical findings, exercise stress test parameters, sestamibi stress myocardial scintigraphy, and C-reactive protein serum levels were also assessed. At an average follow-up of 79 months (range 36 to 144), no patient had died or developed acute myocardial infarction. Cardiac MIBG defect score was significantly lower in patients with worsening versus those without worsening of angina status (13 ± 7 vs 38 ± 28, p = 0.001), in those with versus those without hospital readmission because of recurrent chest pain (15 ± 9 vs 35 ± 29, p = 0.01), and in those who underwent versus those who did not undergo repeat coronary angiography (11 ± 7 vs 36 ± 27, p = 0.001). Significant correlations were found between quality of life (as assessed by the EuroQoL scale) and heart/mediastinum ratio (r = 0.48, p = 0.002) and cardiac MIBG uptake score (r = -0.69, p <0.001). No other clinical or laboratory variable showed a significant association with clinical end points. In conclusion, in patients with CSX, abnormal function of cardiac adrenergic nerve fibers, as assessed by an impairment of cardiac MIBG uptake, identifies those with worse symptomatic clinical outcomes.