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Patients undergoing percutaneous coronary intervention (PCI) with a clinical indication for oral anticoagulation (OAC) in addition to antiplatelet therapy (APT) necessitate rigorous evaluation of bleeding and ischemic risk to guide therapy. The optimal OAC/APT drug combination and duration of treatment is not known. This study aimed to evaluate the incidence of patients undergoing PCI with an OAC indication and the rationale for post-PCI combined OAC/APT selection in clinical practice. Consecutive patients undergoing PCI with an indication for combined OAC/APT were included in a 12-month retrospective case series. Patient demographics, clinical characteristics, prescribed OAC/APT regimens, and rationale for drug selection were reviewed. PCI was performed in 1650 patients during the study period, with an indication for OAC/APT in 133 (8.1%). A combination of aspirin, P2Y12 inhibitor, and OAC was the most frequently prescribed regime on discharge (n = 103, 81%). Dual antiplatelet therapy (DAPT) in combination with OAC was continued for a mean duration of 6.4 ± 4.4 weeks (range 3-52 weeks) before one antiplatelet was discontinued. There was no significant difference between the mean CHA2DS2-VASc or HAS-BLED score of patients with atrial fibrillation discharged on OAC/DAPT compared with alternate combinations (DAPT alone or OAC/single APT), 3.6 ± 1.3 versus 3.8 ± 1, P = 0.37 and 2.04 ± 0.7 versus 2.05 ± 1.0, P = 0.98, respectively. This case series identifies high variability in OAC/APT treatment duration and limited application of risk scoring systems and high-risk PCI characteristics in the selection of OAC/APT regimens. A more systematic patient assessment is needed to help standardize OAC/APT prescribing for this important patient cohort.

Background Transcatheter aortic valve implantation (TAVI) has proven efficacy in the treatment of aortic stenosis (AS). Understandably, there is increasing enthusiasm for its use to treat aortic regurgitation (AR). However, there are significant anatomical differences between AS and AR which make TAVI for AR more complex. Case summary We present the case of technically challenging TAVI for severe AR, which was complicated by a traumatic ventricular septal defect (VSD) that required percutaneous closure. To our knowledge, this is the first published case of VSD post-TAVI for AR. Discussion This unanticipated complication highlights anatomical differences between TAVI use in AS and AR. Lack of aortic valve calcification and excessive annular compliance made stable deployment of a self-expanding valve extremely challenging. Despite device oversizing, repeated embolization of the prosthesis into the left ventricular outflow tract traumatized the interventricular septum.

Background Infective endocarditis (IE) is a potentially life-threatening infection of the heart’s endocardial surface. Despite advances in the diagnosis and management of IE, morbidity and mortality remain high. Aim To characterize the demographics, bacteriology and outcomes of IE cases presenting to an Irish tertiary referral centre. Design Retrospective cohort study. Methods Patients were identified using Hospital Inpatient Enquiry and Clinical Microbiology inpatient consult data, from January 2005 to January 2014. Patients were diagnosed with IE using Modified Duke Criteria. Standard Bayesian statistics were employed for analysis and cases were compared to contemporary international registries. Results Two hundred and two patients were diagnosed with IE during this period. Mean age 54 years. Of these, 136 (67%) were native valve endocarditis (NVE), 50 (25%) were prosthetic valve endocarditis (PVE) and 22 (11%) were cardiovascular implantable electronic device-associated endocarditis. Culprit organism was identified in 176 (87.1%) cases and Staphylococcal species were the most common (57.5%). Fifty-nine per cent of NVE required surgery compared to 66% of PVE. Mean mortality rate was 17.3%, with NVE being the lowest (12.5%) and PVE the highest (32%). Increasing age was also associated with increased mortality. Fifty-three (26.2%) patients had embolic complications. Conclusions This Irish cohort exhibited first-world demographic patterns comparable to those published in contemporary international literature. PVE required surgery more often and was associated with higher rates of mortality than NVE. Embolic complications were relatively common and represent important sequelae, especially in the intravenous drug user population. It is also pertinent to aggressively treat older cohorts as they were associated with increased mortality.

Hereditary haemochromatosis is often not diagnosed until adulthood. Iron overload cardiomyopathy initially results in diastolic dysfunction and can result in arrhythmias and irreversible cardiac failure if untreated. The aim of this study was to investigate whether patients with newly diagnosed hereditary haemochromatosis without signs of heart failure exhibit subclinical alterations of cardiac function and to determine if cardiac function improved after 1 year of venesection. Baseline echocardiography was performed on 25 patients with newly diagnosed hereditary haemochromatosis with elevated serum ferritin levels. The test was repeated after 1 year of treatment with venesection. Tissue Doppler imaging (TDI) and deformation (strain) imaging using speckle tracking were performed. Left atrial force was measured according to the Newtonian principle, in which force (dynes) = mass × acceleration. Left atrial force was calculated by the Manning method expressed as ρ × 0.53 × mitral annular orifice area × (peak A velocity)2. Radial strain showed a significant improvement after 1 year of venesection (increase from 38.8 to 52.6). The LAF showed a significant decrease after 1 year of venesection (median decrease = 0.6 (IQR 0, 1.60), p = 0.0004). Iso-volumetric relaxation time (IVRT) decreased significantly in patients after 1 year of venesection (decrease from 107.4 ± 16.2 to 97.68 ± 15.4 ms, p (0.0187)). Among all measurements, radial strain, IVRT and left atrial force were shown to significantly improve following a 1-year course of venesection, suggesting that these parameters could be used to identify subclinical cardiac dysfunction in patients with iron overload secondary to hereditary haemochromatosis and to guide intensification of venesection therapy.

Background Spontaneous coronary artery dissection (SCAD) is a recognized cause of acute coronary syndrome (ACS). Pregnancy, the postpartum period, and illicit drug use have all been reported as potential triggers. Case summary We describe the case of a 41-year-old patient who presented to the emergency department with chest pain in the setting of recent cocaine and amphetamine use. The patient was 4 months postpartum following an uncomplicated pregnancy. Past medical history was non-contributory, with no known risk factors for ischaemic heart disease. Electrocardiogram was normal but high-sensitivity troponin T was significantly elevated. Coronary angiography revealed multi-vessel SCAD. This was managed conservatively as the patient remained clinically stable and pain free without high-risk anatomy (left main stem or proximal two-vessel coronary artery dissection). Discussion Spontaneous coronary artery dissection must be considered in a postpartum patient presenting with ACS, particularly in the context of environmental stressors such as illicit drug use. Coronary angiography is key to determine diagnosis and guide management. Conservative therapy is favoured, except for patients with ongoing ischaemia, haemodynamic instability, and left main stem involvement. In this case, we suspect SCAD occurred due to the haemodynamic effects of cocaine and amphetamines in the context of structural arterial changes of the postpartum state.

ObjectivesCT coronary angiography (CTCA) is a well-validated clinical tool in the evaluation of chest pain. In our institution, CTCA availability was increased in January 2020, and subsequently, expanded further to replace all exercise testing during the COVID-19 pandemic. Our objective was to assess the impact of increased utilisation of CTCA on length of stay in patients presenting with chest pain in the prepandemic era and during the COVID-19 pandemic.MethodsStudy design was retrospective. Patients referred for cardiology review between October 2019 and May 2020 with chest pain and/or dyspnoea were broken into three cohorts: a baseline cohort, a cohort with increased CTCA availability and a cohort with increased CTCA availability, but after the national lockdown due to COVID-19. Coronary angiography and revascularisation, length of stay and 30-day adverse outcomes were assessed.Results513 patients (35.3% female) presented over cohorts 1 (n=179), 2 (n=182), and 3 (n=153). CTCA use increased from 7.8% overall in cohort 1% to 20.4% in cohort 3. Overall length of stay for the patients undergoing CTCA decreased from a median of 4.2 days in cohort 1 to 2.5 days in cohort 3, with no increase in 30 days adverse outcomes. Invasive coronary angiogram rates were 45.8%, 39% and 34.2% across the cohorts. 29.6% underwent revascularisation in cohort 1, 15.9% in cohort 2 and to 16.4% in cohort 3.ConclusionsIncreased CTCA availability was associated with a significantly reduced length of stay both pre-COVID-19 and post-COVID-19 lockdown, without any increase in 30-day adverse outcomes.

Introduction: There is a paucity of studies on right ventricular (RV) function in patients with obstructive sleep apnoea (OSA). Depressed RV function is related to sleep apnoea severity based on the apnoea hypopnea index (AHI). Therefore we used basic echocardiographic measurements of RV function to evaluate early systolic changes (improvements) pre and post the application of Continuous Positive Airway Pressure (CPAP). Aim: To investigate RV function and the effect of CPAP on RV function in patients with newly diagnosed OSA. Methods: Newly diagnosed patients with OSA were selected. Eleven patients with an Apnoea–hypopnea index (AHI) greater than 10 were enrolled to receive CPAP therapy and were assessed after 12 weeks. CPAP compliance was set at >4hrs usage per night on 70% of all nights. Echocardiography (ECHO) including RV assessment was performed at baseline and after 12 weeks of CPAP therapy. Right Ventricular Diameter (RVD1), Total Annular Plane Systolic Excursion (TAPSE) and Fractional Area Changes (FAC) were measured. Results: Our measurements showed that Right Ventricular Size (RVD1) and FAC was reduced and RV function had improved as assessed by TAPSE over the 12 weeks and these improvements were statistically significant: (p Conclusion: RV function can improve as early as 12 weeks into CPAP therapy as assessed by simple echocardiography measurements of RV size and function.

Background In 2013, PPCI was implemented as the reperfusion strategy for STEMI in Ireland. A PPCI strategy may result in increased delays to treatment, which may negate the anticipated benefit of PPCI over fibrinolysis provided in a local hospital. We wished to explore the real world effect of a PPCI strategy on clinical outcomes for a cohort of rural based patients distant from their designated PPCI centre (at a projected 60–90 min transfer time). Methods We conducted this retrospective cohort study at 2 rural hospitals and at a PPCI centre. We identified 2 cohorts of patients with STEMI. The first comprised all patients presenting to the rural hospitals between 01/01/2011 to 31/12/2012 with STEMI (Pre-PPCI cohort). The second comprised all patients from the catchment area of these hospitals presenting with STEMI to the PPCI centre between 01/01/2015 to 31/12/2016 (PPCI cohort). For each cohort we compared baseline characteristics, timeliness of initiation of reperfusion therapy, in hospital mortality, and for patients who survived to discharge the prevalence of LV dysfunction at 180 days. We tested for significance using chi square test. Results We identified 127 STEMI patients (97 M/30F), median age 60.6 (Range 31–94). A door to needle time of less than 30 minutes was achieved in the Pre-PPCI cohort in 68% of eligible patients (n=73) (median 21 mins (IQR 13–31 mins)) and a reperfusion time Conclusions Projected patient transfer times utilised by healthcare planners may underestimate the challenges faced in delivering optimal reperfusion times for rural patients in clinical practice. We found delays to PPCI were commonplace, and overall we did not demonstrate a superiority for PPCI over fibrinolysis in this setting. In light of the trend towards better outcomes for patients directly transferred from the community to the PPCI hospital, a comprehensive strategy of direct transfer needs to be rigorously pursued.

Introduction The appropriate anti-thrombotic regime after a percutaneous intervention (PCI) for patients who have an indication for an oral anticoagulant (OAC) is not standardised and represents a challenge to clinicians who are guided by a patient’s perceived bleeding and ischaemic risks. Hence, we sought to describe our current clinical practice for patients undergoing PCI with an OAC indication. Methods A retrospective review of patient’s clinical notes was performed to identify those discharged with an indication for an OAC after PCI between October 2018 and March 2019. These patients were further scrutinised for the antithrombotic-OAC regime employed, bleeding risk and ischaemic risk. Results Over a 6-month period, 9.7% (n=69) of patients (14.5% female, mean age 71.2±9.7 years, 9.7% ACS presentation) undergoing PCI had an indication for an OAC on discharge. Atrial fibrillation (AF) was the OAC indication in 84% of cases (n=58) and these patients had a mean CHA2DS2-VASc score of 3.6±1.2 and mean HAS-BLED score of 2±0.6. Standard drug-eluting stents were deployed in the majority of cases while Polymer-free BioFreedom stents were used for 31.9% (n=22) of patients. A variety of antithrombotic-OAC regimes were prescribed on discharge, table 1. Dual antiplatelet therapy (DAPT) without an OAC was prescribed for 2 patients on discharge who experienced inpatient bleeding complications. Apixaban and rivaroxaban were prescribed for 43.5% (n=30) and 36.2% (n=25) of patients respectively on discharge, warfarin was prescribed for a minority of patients (6.9%, n=6). DAPT with an OAC was prescribed for a mean of 7.4±5.3 weeks (range 4 –> 26 weeks) in 81% (n=56) of patients. Once the planned duration of combination DAPT-OAC was complete, clopidogrel was continued as the single antiplatelet of choice in addition to an OAC in 50% (n=28) of cases. For patients with AF, a discharge regime of an OAC with clopidogrel in the absence of aspirin did not appear to be related to patient’s CHA2DS2-VASc or HAS-BLED score. This pattern was operator dependent. Bleeding and ischaemic rates at 6-month follow up are pending. Conclusions A clear preference for novel OACs after PCI was observed. However, the choice of antithrombotic and duration of OAC was highly variable. This likely reflects the challenge in assessing individuals bleeding and ischaemic risks.

BackgroundStudying variability in the care provided to secondary prevention coronary heart disease (CHD) outpatients can identify interventions to improve their outcomes.MethodsWe studied outpatients who had an index CHD event in the preceding 6–24 months. Eligible CHD events included acute coronary syndrome (ACS) and coronary revascularisation for stable chronic coronary syndrome (CCS). Site training was provided by a core team and data were collected using standardised methods.ResultsBetween 2017 and 2019, we enrolled 721 outpatients at nine Irish study sites; 81% were men and mean age was 63.9 (SD ±8.9) years. The study examination occurred a median of 1.16 years after the index CHD event, which was ACS in 399 participants (55%) and stable-CCS in 322. On examination, 42.5% had blood pressure (BP) >140/90 mm Hg, 63.7% had low-density lipoprotein cholesterol (LDL-C) >1.8 mmol/L and 44.1% of known diabetics had an HbA1c >7%. There was marked variability in risk factor control, both by study site and, in particular, by index presentation type. For example, 82% of outpatients with prior-ACS had attended cardiac rehabilitation versus 59% outpatients with prior-CCS (pConclusionsDespite international secondary prevention guidelines broadly recommending the same risk factor targets for all adults with CHD, we found marked differences in outpatient risk factor control and management on the basis of hospital location and index CHD presentation type (acute vs chronic). These findings highlight the need to reduce hospital-level and patient-level variability in preventive care to improve outcomes; a lesson that should inform CHD prevention programmes in Ireland and around the world.

Background: TAVI is a percutaneous approach to aortic valve replacement in high surgical risk patients deemed inoperable. Aim: To evaluate the early and mid-term outcomes for an Irish TAVI cohort over a six-year period at St James's Hospital and Blackrock Clinic, Dublin, Ireland. Results: In total 147 patients, 56% male with an average age of 82 underwent TAVI between December 2008 and December 2014. Thirty day, one year and two year survival was 90.5%, 83% and 71% respectively. Major vascular complications and renal failure were the biggest predictors of mortality at 30 days (p = 0.02). We observed a pacing rate of 13.5%, the majority in patients who had Medtronic Corevalve implants (p

Optimal reperfusion in STEMI is the key goal of the National ACS programme. The aims of the programme are a diagnosis to door time (DDT) of We used the Code STEMI database collected prospectively and HIPE data to identify our patient cohort. From January to December 2015 487 patients in total were identified as diagnosed with a STEMI or transferred to St James as part of the National ACS programme. Looking at all-comers; 222 (45%) were transferred from another hospital, 206 (42%) from the field, 29 (6%) from our ED. The average DDT of the patients from outside hospitals was 141 minutes (median 110, range 18–798), 63% were outside the 90 minutes DDT. The average RT was 148 minutes (median 128, range 25–599), 57% of the patients were outside the recommended 120 minutes for RT. Only 7 patients (1.4%) were thrombolysed prior to transfer. There are inevitable delays when arranging transfer of Code STEMI patients from an outside hospital to the primary PCI centre. It is expected that a proportion of patients will have to undergo thrombolysis as the initial reperfusion strategy. The data we collected in the largest PPCI centre in the country highlights that thrombolysis is being under-utilised and needs to be considered in all inter-hospital STEMI transfers.

Introduction The rates of Transcatheter aortic valve implantation (TAVI) in comparison to surgical aortic valve replacement have steadily increased in the last ten years for treatment of severe symptomatic aortic stenosis in high-risk surgical candidates who would have otherwise been managed conservatively. Methods of vascular access management have also changed. Aim The aim of our study was to assess the vascular access routes, closure device methods, individual failure rates of each device and the need for vascular surgical intervention for TAVI cases at our single centre in the Republic of Ireland. Methods A retrospective data collection was conducted of elective TAVI procedures performed from December 2008 to February 2018 at a single centre in Dublin. All patients had severe symptomatic aortic stenosis as per international guidelines and were deemed unsuitable for surgical aortic valve replacement by the local heart team. The angiographic, echocardiographic, laboratory and radiographic data as well as access route, closure device type and mode of failure were recorded in a database. Results A total of 129 patients (mean age 81.4±5.96 years of age, 51% female) had a TAVI implantation at our centre between December 2008 and February 2018.In 120 (93.02%) cases, the right femoral artery was the access site for the valve delivery sheath. In 117 (90.69%) of the total cases, the access was percutaneous. Four patients (3.10%) required vascular cut down, 6 cases (4.65%) required mini thoracotomy and 3 patients (2.32%) required mini sternotomy. Twenty -two patients (18.80%) experienced closure device failure, of which 18 patients (81.81%) had a leak, one patient (4.45%) had a dissection, one patient (4.45%) had an occlusion and two patients (9.09%) had a perforation. Of those twenty-two patients who had a closure device failure, 10 patients (45.45%) were treated successfully with pressure haemostasis and 4 cases (18.81%) were treated successful with balloon inflation. Seven patients (31.81%) required vascular surgery and one patient (4.45%) was treated by an interventional radiologist. In 111 of the cases, the closure device was recorded. 26 Proglide devices, 9 Vivasure devices and 76 Prostar devices were used. The Vivasure devices had a 55.55% failure rate. Five of the Proglide devices failed (19.23%) and 11(of the Prostar devices failed (14.47%). Of the 7 people requiring urgent vascular surgery, 5 has a failed Prostar device, one had a Vivasure device and in the last case the closure device was not documented. Conclusion The majority of our cases were performed via the right Femoral artery with percutaneous access. While vascular closure device failures remain a clinically important complication of TAVI procedures, the need for surgical repair is low.

Background In patients with multivessel coronary artery disease presenting with STEMI, the CVLPRIT and PRAMI trials recommend undertaking only culprit artery PCI at the time of Primary PCI (PPCI); staged revascularization may then be performed at a later stage, in the days to weeks after PPCI. Purpose The aim of this study was to review patients presenting with STEMI and multivessel disease and compare one-year outcomes of those undergoing multivessel PCI (MVPCI) at the time of PPCI to those undergoing staged PCI after initial acute culprit artery intervention. Methods St. James’s Hospital is Dublin’s largest PCI centre. We identified all patients undergoing PPCI for STEMI between January and December 2015 using the Code STEMI database. Electronic health records, chart review and HIPE data were used to further characterize patients. The number of patients undergoing MVPCI during index procedure was recorded, as was the number undergoing staged intervention. Those referred for CABG were excluded from further analysis Average patient length of stay and need for re-intervention and mortality at one year were noted. Results There were 417 patients with a confirmed diagnosis of STEMI, either clinically or angiographically; out of this 364 (87.2%) underwent PPCI. 153 patients (36.6%) presenting with STEMI had multi-vessel coronary artery disease, 136 (88.8%) had PCI and 17 (11.2%) had CABG. Of the 136 undergoing PCI for multi-vessel disease, 59 (43.4%) underwent MVPCI during PPCI; the average age of this group was 61 years and 12 (21%) were female. 77 (56.6%) patients underwent staged PCI, the average age was 62 years and 13 (17%) were female. Of those undergoing staged PCI 21 (27%) occurred during the index patient admission and 56 (73%) occurred as an outpatient. Within one-year of follow-up 4 (6.7%) in the MVPCI group and 6 (7.8%) in the staged PCI group had represented with recurrent MI requiring further revascularization, thus the relative risk of MVPCI patients presenting with recurrent MI was 0.8701, though the p value was non-significant at 0.82. 4 patients (6.7%) in MVPCI and 10 patients (12.9%) in staged PCI represented with angina, this represents a relative risk of 0.522, again with a non-significant p-value of 0.25. 1 patient died in MVPCI group, though this was due to non-cardiac causes; there were no deaths in the staged PCI group. Median hospital stay was 8.4 days (range 4.4–17.7) in the MVPCI group and 6.3 days (range 3.3–26.2) in the staged intervention group. Conclusion In the largest Irish Primary PCI center, we found there no significant difference in patients representing with recurrent MI requiring repeat revascularization in multivessel PCI as compared to staged PCI. There was a trend for those undergoing staged PCI to represent with more angina than those undergoing MVPCI, though this did not reach statistical significance. There was no difference in length of stay between cohorts.

Introduction Heart failure activates the renin-angiotensin aldosterone system, which leads to hypokalaemia. This is aggravated by upregulated sympathetic drive and the use of increased amounts non-potassium sparing diuretics during acute exacerbations. Mild hypokalaemia has been shown to increase cardiovascular events in a treated heart failure population. In patients with NYHA class I to III patients, elevations of serum potassium have been shown to reduce the incidence of fatal arrhythmias and sudden cardiac death. Serum potassium levels should be maintained above 4 mmol/l in heart failure patients. The aim of this study was to assess the prevalence of hypokalaemia in a cohort of patients admitted with decompensated heart failure. Methods We performed a retrospective review of all patients admitted to St. James’s Hospital during a one year period from 1st January 2015 to 31st December 2015, with a principal diagnosis of congestive cardiac failure. A total of 330 patients were included. Blood results on admission, 24 hours and the final result prior to discharge were included for analysis. Results On admission, 67 (22%) of the 309 patients with admission bloods had a serum potassium value of less than 4.0 mmol/l. Of the sub-group of patients that had bloods taken at both admission and 24 hours, (n=287) the percentage of patients with a serum potassium less than 4.0 mmol/l at 24 hours significantly increased from 21% to 36% (p The average serum potassium of all patients fell significantly during the first 24 hours from 4.44 mmol/L to 4.17 mmol/l (p Patients who had a serum potassium of less 3.5 mmol/l after 24 hours had a significantly lower serum magnesium on admission than the patients whose 24 hour potassium result was within the normal range. (0.71 mmol/L vs 0.81 mmol/L, p Of the patients surviving to discharge, over 33% were discharged with serum potassium values less than 4.0 mmol/l. Conclusion Our results demonstrate that a significant number of patients with heart failure have lower than desirable serum potassium levels on admission, through their inpatient stay and on discharge (figure 2). Within a cohort of patients hospitalised for exacerbations of heart failure, serum potassium levels fall during the first 24 hours (figure 1). There is a significant correlation between hypomagnesaemia on admission and the prediction of hypokalaemia 24 hours later. Hypokalaemia has been shown to be an independent predictor of mortality in heart failure. This study highlights the importance of close monitoring of serum potassium levels early in the admission, and suggests that identification of hypomagnesaemia can predict hypokalaemia at 24 hours. The authors advise early consideration of aldosterone antagonists or potassium replacement in these patients.

Background: Latest research shows that the lower resting values of right ventricular (RV) myocardial % strain may represent a physiologic change rather than subclinical myocardial damage. Therefore, we assessed load-independent changes to the RV as a consequence of high intensity training by measuring the Isovolumic acceleration (IVA) of the free wall of the RV in conjunction with NT pro-BNP measured by an electrochemiluminescence assay. Methods: Seventeen controls (mean age 27 4), 24 soccer footballers (mean age 24 4), and 18 elite rowers (mean age 22 4) were studied. Left ventricular (LV) and RV % strain were measured using two-dimensional (2D) speckle based automated functional imaging (AFI) software. RV free wall IVA was measured using pulsed-wave tissue Doppler at the lateral tricuspid annulus. Standard 2D echo were used to measured RV parameters including the Tei index (systolic and diastolic function) and the total annular plane systolic excursion (TAPSE) of the RV annulus. NT pro-BNP was measured by an electrochemiluminescence assay. Results: The RV diameter was increased in the footballers and elite rowers compared with controls (P < 0.001). RV wall size was greater in the elite rowers compared with controls and footballers (P = 0.002). The peak IVA of the RV was higher in the rowers, compared with the footballers and to controls (P < 0.001). The mean LV and RV % myocardial strain were lower in the elite athletes and the footballers compared with controls (P < 0.001). There was no difference in RV Tei index, levels of BNP, and TAPSE across all subjects. Conclusions: This study showed a significant increase in IVA of the RV of athletes despite reduced myocardial % strain and normal levels in NT-proBNP. This suggests that the decrease in % strain is not a consequence of myocardial damage, but may represents a part of the physiological response to endurance exercise. Therefore, a reduced IVA in a remodeled RV could herald a pathological response. (Echocardiography 2013;30:929-935)

Objectives Four variants (K60N, Q128R, G202R, and A592E) in the nebulette gene were identified in patients with dilated cardiomyopathy (DCM) and endocardial fibroelastosis. We sought to determine if these mutations are cardiomyopathy causing. Background Nebulette aligns thin filaments and connects them with the myocardial Z-disk, playing a role in mechanosensation. Methods We generated transgenic mice with cardiac-restricted overexpression of human wild-type or mutant nebulette. Chimera and transgenic mice were examined at 4, 6, and 12 months of age by echocardiography and cardiac magnetic resonance imaging. The hearts from embryos and adult mice were assessed by histopathologic, immunohistochemical, ultrastructural, and protein analyses. Rat H9C2 cardiomyoblasts with transient expression of nebulette underwent cyclic mechanical strain. Results We identified lethal cardiac structural abnormalities in mutant embryonic hearts (K60N and Q128R). Founders of the mutant mouse lines developed DCM with severe heart failure. An irregular localization pattern for nebulette and impaired desmin expression were noted in the proband and chimeric Q128R mice. Mutant G202R and A592E mice exhibited left ventricular dilation and impaired function with specific changes in I-band and Z-disk proteins by 6 months of age. The mutations modulated distribution of nebulette in the sarcomere and Z-disk during stretch of H9C2 cells. Conclusions Nebulette is a new susceptibility gene for endocardial fibroelastosis and DCM. Different mutations in nebulette trigger specific mechanisms, converging to a common pathological cascade leading to endocardial fibroelastosis and DCM.

BACKGROUND A standard 12-lead electrocardiogram (ECG) is performed in all hypertensive patients, primarily to detect left ventricular hypertrophy. Echocardiographic assessment of hypertensive subjects reveals that abnormalities in diastolic function occur more commonly and earlier than increased left ventricular mass. However, ECG changes associated with diastolic dysfunction (DD) remain poorly defined; we assessed the ventricular activation time (VAT) (i.e., the time for the ventricle to depolarize) as a potential marker for DD in early hypertension. METHODS Ninety subjects (aged 46 +/- 1.3 years; 43 men) with newly diagnosed, untreated hypertension underwent ECG and comprehensive two-dimensional echocardiography. Left ventricular DD was echocardiographically assessed using Canadian Consensus Guidelines. We compared VAT, which corresponds to the QR interval in the 12-lead ECG, with echocardiographic parameters of DD. RESULTS VAT was prolonged in subjects with DD (46.3 +/- 0.4 vs. 39.6 +/- 0.3 ms, P < 0.01). There was a significant correlation between VAT and tissue Doppler imaging (TDI) (early diastolic velocity) e' (r = -0.53, P < 0.0001), (ratio of early and late diastolic velocities) e'/a' (r = -0.53, P < 0.0001), transmitral Doppler (TMD) (early peak filling rate, and early deceleration peak) E/A (r = -0.32, P = 0.001), and (ratio of early diastolic mitral inflow and early diastolic velocities) E/e' (r = 0.44, P < 0.0001). CONCLUSION Prolongation of the VAT is associated with DD in patients with newly diagnosed untreated hypertension.

Syncope is a common clinical problem accounting for up to 6% of hospital admissions. Little is known about resource utilisation for patients admitted for syncope management in Ireland.To determine the utilisation of resources for patients admitted for syncope management.Single centre observational case series of consecutive adult patients presenting to an acute hospital Emergency Department with syncope over a 5-month period.Two-hundred and fourteen of 18,898 patients (1.1%) had a syncopal episode, 110 (51.4%) of whom were admitted. Mean length of stay was 6.9 days. Sixty-four of these admissions were deemed unnecessary by retrospective review when compared to ESC guidelines. Eighty-five (77.3%) admitted patients had cardiac investigations and 56 (51%) had brain imaging performed.Syncope places a large demand on overstretched hospital resources. Most cases can be managed safely as an outpatient and to facilitate this, hospitals should develop outpatient Syncope Management Units.

Objective To examine the long-term effects of standard chemotherapy on myocardial function in asymptomatic breast cancer survivors using two-dimensional speckle tracking echocardiography. Methods Seventy women (chemotherapy group) aged 5468 years who had received anthracycline treatment with (n¼19) or without (n¼51) adjuvant trastuzumab up to 6 years previously, and 50 female controls were studied. Left ventricular systolic (ejection fraction (EF%), peak systolic myocardial excursion, (Sm)) and diastolic (peak mitral E and A velocities, six-point average of mitral annular E 9 velocities) function, 2D global and regional longitudinal and radial strain were determined using standard 2D Doppler and tissue Doppler echocardiographic methods and speckle tracking software. Results Despite normal EF% (6264% vs 6063%, p¼0.051) the chemotherapy group had reduced E/A ratios (0.960.3 vs 1.160.3, p¼0.003), global E 9 (10.262 vs 11.262.3, p¼0.036), global Sm (9.061.3 vs 9.661.3, p¼0.029) and global longitudinal 2D strain (� 18.162.2 vs � 19.661.8, p¼0.0001) in comparison with controls. In 18 (26%) of the chemotherapy group, global longitudinal strain was below the lower limit of the control group. Cigarette smoking was a negative predictor of longitudinal strain, but only in the chemotherapy group. Radial strain did not differ significantly between the two groups. There were no significant differences in EF%, global Sm and longitudinal strain between trastuzumab-treated individuals and controls. Conclusions Subclinical systolic and diastolic myocardial abnormalities were present in asymptomatic breast cancer survivors up to 6 years after standard chemotherapy. Cigarette smoking had a negative effect on longitudinal strain in these individuals. Adjuvant trastuzumab treatment did not appear to have an additive adverse impact on myocardial function in the mediumelong term.

Left ventricular (LV) diastolic dysfunction identifies patients at risk of developing heart failure and may be common in patients with hypertension. The prevalence of LV diastolic dysfunction in patients with newly diagnosed hypertension was compared using criteria provided by the Canadian Consensus, European Study Group, and American Medical Association guidelines. One hundred twenty patients with newly diagnosed untreated hypertension (mean age 46.9 +/- 2.1 years; 62 men, 58 women) with increased blood pressure (clinic >140/90 mm Hg, daytime ambulatory >135/85 mm Hg) underwent comprehensive 2-dimensional echocardiography. Transmitral inflow velocities were measured using pulse-wave Doppler with and without Valsalva's maneuver, and a comprehensive assessment of tissue Doppler velocities was performed. The prevalence of LV diastolic dysfunction varied according to criteria used. There was a high prevalence of LV diastolic dysfunction (59%; n = 71) using Canadian Consensus guidelines; 27% of patients (n = 32) had a pseudonormal pattern unmasked using Valsalva's maneuver and 32% (n = 39) had impaired relaxation at rest. Significantly fewer patients (10%; n = 12) had this diagnosis using European or American Medical Association guidelines (23%; n = 27). Using tissue Doppler imaging (early-late diastolic velocity ratio

Background— In autoimmune disorders, circulating autoantibodies identify healthy relatives at risk years before clinical presentation. Healthy relatives of patients with dilated cardiomyopathy (DCM) who have echocardiographic changes, including left ventricular enlargement or depressed fractional shortening at baseline, have increased medium-term risk for DCM development. Approximately one third of relatives have serum anti-heart autoantibodies (AHAs) at baseline; we intended to assess their potential role in predicting DCM development. Methods and Results— Baseline evaluation, including electrocardiography, echocardiography, and AHA, was performed in 592 asymptomatic relatives of 169 consecutive DCM patients (291 males and 301 females; mean age 36±16 years). Relatives were classified in accordance with published echocardiographic criteria; those who did not have DCM were followed up (median of 58 months). DCM among relatives was diagnosed by echocardiography at follow-up. Of the 592 individuals evaluated, 77% were assessed as normal, 4.4% as having DCM, and 19% as possibly affected on the basis of depressed fractional shortening without ventricular dilatation in 17 and left ventricular enlargement without systolic dysfunction in 94. Five-year follow-up of 311 relatives revealed that 26 had progressed (13 to DCM, 11 to left ventricular enlargement, and 2 to depressed fractional shortening). Relatives who developed DCM were more frequently AHA-positive than those who did not (69% versus 37%, P =0.02). Five-year probability of progression to DCM, among normal or possibly affected relatives, was higher in AHA-positive cases ( P =0.03). By Cox regression, positive AHAs at baseline were independent predictors of progression (RR 2.26, CI 1 to 5.1, P =0.03). Conclusions— Among healthy relatives of DCM patients, AHAs are independent predictors of disease development within 5 years.

Most patients with hypertrophic cardiomyopathy (HC) have reduced maximal oxygen consumption (VO2max) during exercise. The degree of impairment is poorly predicted by the magnitude of hypertrophy, left ventricular (LV) outflow tract obstruction, and other conventional markers of disease severity. The aim of this study was to determine the usefulness of N-terminal-pro-B-type natriuretic peptide (NT-pro-BNP) as a marker of exercise performance in HC. Plasma NT-pro-BNP was measured in 171 consecutive patients (mean age 46 +/- 18 years) who underwent echocardiography and cardiopulmonary exercise testing. The mean log NT-pro-BNP was 2.79 +/- 0.5; log NT-pro-BNP levels were higher in women patients (p = 0.001) and patients with chest pain (p = 0.010), in New York Heart Association class > or = II (p = 0.009), with atrial fibrillation (p < 0.001), with systolic impairment (p = 0.025), and with LV outflow tract obstructions (p < 0.0001). NT-pro-BNP levels were also correlated with maximal wall thickness (r = 0.335, p < 0.0001), left atrial size (r = 0.206, p = 0.007), and the mitral Doppler E/A ratio (r = 0.197, p = 0.012). The mean percent VO2max achieved was 73.8 +/- 22.6%; percent VO2max was smaller in patients with systolic impairment (p = 0.044) and LV outflow tract obstructions (p = 0.025). There were inverse correlations between percent VO2max and NT-pro-BNP (r = -0.352, p = 0.001), LV end-systolic cavity size (r = -0.182, p = 0.031), and left atrial size (r = -0.251, p = 0.003). On multivariate analysis, only NT-pro-BNP was correlated with percent VO2max. A NT-pro-BNP level of 316 ng/L had 78% sensitivity and 44% specificity (area under the curve 0.616) for predicting percent VO2max < 80%. In conclusion, NT-pro-BNP levels correlate with peak oxygen consumption in HC and are more predictive of functional impairment than other conventional markers of disease severity.

The optimal pacing site in cardiac resynchronization therapy (CRT) remains controversial. Tissue synchronization imaging is a novel echocardiographic technique that color-codes for areas of maximal delay in myocardial velocities. This study aimed to identify whether the left ventricular (LV) pacing lead position in CRT should be guided by a patient's area of maximal mechanical delay. Fifty-four patients with advanced heart failure were assessed echocardiographically before and 6 months after CRT. Response was analyzed according to the relation between the LV lead position and the area of maximal delay to peak velocity by tissue synchronization imaging in the first half of the ejection phase: group 1 (n = 22) had lead placement corresponding to the segment of maximal delay; group 2 (n = 13) had lead placement 1 segment adjacent; and group 3 (n = 19) had lead placement remote from this site. Evidence of LV reverse remodeling and improved systolic function was documented in group 1 (mean percentage decrease in end-systolic volume 23%) more than in group 2 (mean decrease 15%), and more than in group 3 (mean increase 8.9%, p0.0001 compared with groups 1 and 2). In group 1, 16 of 22 patients had reverse remodeling (15% decrease in end-systolic volume); reverse remodeling was seen in 7 of 13 patients in group 2 and 1 of 19 in group 3. The placing of the lead position proximal to the site of maximal delay by tissue synchronization imaging was correlated with reverse remodeling (r = 0.449, p = 001). Of 7 patients with delay confined to the septum and anterior wall only, none had evidence of reverse remodeling after CRT. In conclusion, pacing at the site of maximal mechanical delay was associated with reverse remodeling. Individually tailored LV lead positioning should be considered before CRT.

OBJECTIVES The aim of this study was to investigate the clinical expression of adenosine monophosphate-activated protein kinase (AMPK) gene mutations (PRKAG2) in adenosine monophosphate (AMP) kinase disease based on 12 years follow-up of known mutation carriers and to define the prevalence of PRKAG2 mutations in hypertrophic cardiomyopathy (HCM). BACKGROUND Adenosine monophosphate-activated protein kinase gene mutations cause HCM with Wolff-Parkinson-White syndrome and conduction disease. METHODS Clinical evaluation of 44 patients with known AMP kinase disease was analyzed. Mutation analysis of PRKAG2 was performed by fluorescent single-strand confirmation polymorphism analysis and direct sequencing of abnormal conformers in 200 patients with HCM. RESULTS Only one additional mutation was identified. The mean age at clinical diagnosis in the 45 gene carriers was 24 years (median 20 years, range 9 to 55 years). Symptoms of palpitation, dypspnea, chest pain, or syncope were present in 31 (69%) gene carriers; 7 (15%) complained of myalgia and had clinical evidence of proximal myopathy. Skeletal muscle biopsy showed excess mitochondria and ragged red fibers with minimal glycogen accumulation. Disease penetrance defined by typical electrocardiogram abnormalities was 100% by age 18 years. Thirty-two of 41 adults (78%) had left ventricular hypertrophy (LVH) on echocardiography, and progressive LVH was documented during follow-up. Survival was 91% at a mean follow-up of 12.2 years. Progressive conduction disease required pacemaker implantation in 17 of 45 (38%) at a mean age of 38 years. CONCLUSIONS The AMP kinase disease is uncommon in HCM and is characterized by progressive conduction disease and cardiac hypertrophy and includes extracardiac manifestations such as a skeletal myopathy, consistent with a systemic metabolic storage disease. Defects in adenosine triphosphate utilization or in specific cellular substrates, rather than mere passive deposition of amylopectin, may account for these clinical features.

Aims Non-compaction of the left ventricle (LVNC) is a disorder of endomyocardial morphogenesis that results in multiple trabeculations in the left ventricular myocardium. The current literature suggests that LVNC in adults is rare and associated with a poor prognosis. Given that the disorder is present at birth and that several studies have reported asymptomatic familial disease in some patients, we hypothesized that there is a long pre-clinical phase of the disease. The aim of this study was to define the prognosis and familial incidence of LVNC. Methods and results This study cohort comprised 45 patients (mean age at diagnosis 37 years) consecutively identified at a referral centre for cardiomyopathy over a 10-year period. Twenty-eight patients (62%) had dyspnoea at presentation; 41 (91%) an abnormal ECG; and 30 (66%) left ventricular dilatation and impaired systolic function. Nine patients (20%) had non-sustained ventricular tachycardia on 24 h Holter monitoring. Mean survival from death or transplantation was 97% at 46 months. There were three thromboembolic events in two patients (4%). On systematic family screening, 8 of 32 (25%) asymptomatic relatives had a range of echocardiographic abnormalities, including LVNC, LVNC with impaired systolic function, and left ventricular enlargement without LVNC. Conclusion This study demonstrates that LVNC is associated with a better prognosis than previously reported. In patients with familial disease, relatives may have features consistent with dilated cardiomyopathy rather than LVNC.

Background In elite athletes left ventricular morphologic changes are predicted to alter passive pressure/volume characteristics by reducing myocardial stiffness and increasing compliance. Aim We investigated the utility of a new Doppler tissue index based on the pressure volume relation ((E/Ea)/LVEDD), which provides a measure of myocardial stiffness, and assessed its usefulness in detecting cardiac adaptation in elite rowers. Methods Thirty-six international rowers (mean age 27±7 years,) who had trained intensively for 15-20 hours per week for more than 5 years and a control group of 30 sedentary but otherwise normal subjects (mean age 26±8 years,) were consented and enrolled into the study. The groups were similar in age and gender. Left ventricular (LV) septal and posterior wall thickness, mass, chamber size, transmitral Doppler peak early (E) and late (A) diastolic filling velocities and isovolumic relaxation times were measured. Early diastolic myocardial velocities (Ea) were averaged from 4 sites at the mitral annulus; Diastolic stiffness was assessed with the use of three indices E, Ea, and the left ventricular end diastolic diameter in diastole (LVEDD). The ratio, [(E/Ea)/LVEDD], represents a pressure/volume relationship and provides a novel index of diastolic stiffness. Rowers were further divided into 2 groups based on the presence or absence of left ventricular hypertrophy (LVH), ≤ 12mm and > 12mm. Results There was no significant difference in Ea (4 site average) between the two groups, but there was a difference in the stiffness index, with rowers having significantly more compliant ventricles (p=0.0003). When compared with controls and adjusted for body surface area (BSA) and heart rate this difference remained statistically significant (p= 0.016). When the rowers were divided into 2 groups based on the presence or absence of LVH there was no difference in the stiffness index (p = 0.68) Conclusions The key distinguishing feature of intense training is a reduction of myocardial stiffness despite the development of left ventricular hypertrophy.

Periprosthetic valve leak can develop as a complication of valve replacement surgery and may manifest as symptomatic valvular regurgitation, heart failure, or haemolysis. We report a case of severe mitral periprosthetic valve leak requiring a two-stage percutaneous closure technique with multiple Amplatzer® III vascular plugs.© 2011 Wiley-Liss, Inc.

BACKGROUND Diastolic dysfunction is common in early hypertension. We hypothesized that improvement in diastolic dysfunction is blood pressure (BP) dependent and may occur early with treatment in newly diagnosed untreated hypertensive patients. METHODS Forty untreated hypertensive subjects (age 52 +/- 1.4 years, mean +/- s.e.m.) with diastolic dysfunction based on Canadian Consensus Guidelines, received either bendroflumethiazide 2.5 mg (1.25 mg for the first month), or candesartan 16 mg (8 mg for the first month). Left ventricular (LV) structure and function, early diastolic velocity (E') and systolic velocity, and systolic myocardial velocity (Sm) were assessed echocardiographically using M-mode, 2-dimensional, and tissue Doppler imaging (TDI) before and at 1 and 3 months following treatment. RESULTS Antihypertensive treatment reduced BP significantly at 3 months (168 +/- 2/97 +/- 1-143 +/- 2/86 +/- 1 mm Hg, P < 0.0001). Both drugs had similar and significant effects on TDI E' which increased from 7.8 +/- 0.2 to 10 +/- 0.3 cm/s (P < 0.001). The improvement in TDI E' was independent of LV mass index (LVMI) regression but was significantly related to the improvement in Sm (r = 0.73, P < 0.0001) and the fall in systolic BP (R = 0.51, P < 0.001). Normalization of diastolic function was associated with better control of BP (130 +/- 4/81 +/- 2 mm Hg vs. 149 +/- 2/88 +/- 1 mm Hg, P < 0.05). In a stepwise regression model, reduction in systolic BP (P < 0.001) and TDI Sm (P < 0.0001) emerged as independent determinants of improvement in TDI E' with no contribution from age, gender or change in relative wall thickness (RWT) (R(2) = 0.68, P < 0.0001). CONCLUSIONS Achieving good BP control and enhancement in systolic function determines the improvement in diastolic function in early hypertension.

Critical coronary stenoses have been shown to contribute to only a minority of acute coronary syndromes (ACS) and sudden cardiac death. Autopsy studies have identified a subgroup of high-risk patients with disrupted vulnerable plaque and modest stenosis. Consequently, a clinical need exists to develop methods to identify these plaques prospectively before disruption and clinical expression of disease. Recent advances in invasive and noninvasive imaging techniques have shown the potential to identify these high-risk plaques. The anatomical characteristics of the vulnerable plaque such as thin cap fibroatheroma and lipid pool can be identified with angioscopy, high frequency intravascular ultrasound, intravascular MRI, and optical coherence tomography. Efforts have also been made to recognize active inflammation in high-risk plaques using intravascular thermography. Plaque chemical composition by measuring electromagnetic radiation using spectroscopy is also an emerging technology to detect vulnerable plaques. Noninvasive imaging with MRI, CT, and PET also holds the potential to differentiate between low and high-risk plaques. However, at present none of these imaging modalities are able to detect vulnerable plaque neither has been shown to definitively predict outcome. Nevertheless in contrast, there has been a parallel development in the physiological assessment of advanced atherosclerotic coronary artery disease. Thus recent trials using fractional flow reserve in patients with modest non flow-limiting stenoses have shown that deferral of PCI with optimal medical therapy in these patients is superior to coronary intervention. Further trials are needed to provide more information regarding the natural history of high-risk but non flow-limiting plaque to establish patient-specific targeted therapy and to refine plaque stabilizing strategies in the future.

Inflammation is increasingly considered to be involved in the pathogenesis of acute coronary syndromes. We documented persistent elevation in the levels of soluble ICAM-1 and soluble VCAM-1 and a decrease in the levels of soluble E-selectin in the first 72 hours of acute presentation in patients with unstable angina and subendocardial myocardial infarction.

Background Left atrial force (LAF) has been proven useful in assessing changes in diastolic performance in inherited cardiomyopathy. However, it is unclear whether LAF has the same potential in assessing diastolic performance in infiltrative disorders. Therefore the aim of the study is to examine whether changes in diastolic performance following treatment for Hereditary Haemochromatosis (HH) over a one year period can be assessed using LAF. Methods We followed 49 patients with HH with elevated serum ferritin levels (852 mcg/L ± 189) at initial diagnosis and after undergoing treatment with venesection for 1 year. Diastolic myocardial function was measured by the ratio of mitral peak velocity of early filling (E) to early diastolic mitral annular velocity (e’) (E/e’ ratio). Left atrial volume (LAV) was measured by the biplane area/length method. Left atrial force was assessed according to the Newtonian principle, in which force (dynes) = mass—acceleration. Mass is defined by the product of the density of blood (i.e.1.06 g/cm 3 ) and the volume of blood passing through the mitral annulus during atrial contraction. Left atrial force was calculated by the Manning method expressed as, 0.53—mitral annular orifice area—(peak A velocity). Results Follow up data are available of 25 of 49 subjects. There was no difference in age, gender, body surface area (P = 0.089) compared to 20 matched controls. Over the time course of treatment there was no significant change in the E/e as a marker of diastolic myocardial function (median change pre-post = 0, inter-quartile range (IQR: -0.8, 0.4), p = 0.55). There was a significant difference pre and post venesection in LAV (median pretreatment 24 ml/m 2 , post 18 ml/m 2 , p = 0.017) and serum ferritin (pre 851, mcg/L, post 239 mcg/L ± 101). We applied a non-parametric signed rank test and the median left atrial force was 5.6 kdynes pre- treatment and 4.4 kdynes post. LAF showed a significant decrease (median decrease = 0.6, (IQR 0, 1.60), p = 0.0004). Conclusion LAF is a precise haemodynamic monitor for early detection of LA dysfunction and may help monitor response to treatment in patients with hereditary hemochromatosis.

Aim Transcatheter aortic valve implantation (TAVI) has become a therapeutic option for high-risk or nonoperable patients with severe symptomatic aortic valve stenosis. We reviewed our patient cohort analysing early and mid term outcomes. Methods Records of 147 patients who underwent TAVI between 12/08 and 12/14 at St James Hospital and Blackrock Clinic. A database of baseline demographics, pre procedural and procedural characteristics and complications was created. Contacting patients, next of kin or the primary care doctor confirmed clinical follow up and pacing rates after discharge. Results 147 patients underwent TAVI with a mean age of 82 (SD-5), with 56% males (83/147). 87 received a Corevalve and the remaining 61 an Edwards prosthesis. Mean EURO II score was 9.2 ± 6.46 for the total cohort. Table 1 further illustrates baseline demographics. Access was via transfemoral route 135/147 (91%), trans apical or transaortic in (7%) and subclavian in (3%). Mean length of stay was 9.97 days. Major vascular complications occurred in 18 cases (12.2%) and pacing rates post implant was 13.5% of which 90% were Corevalve implants (P At 30 days 9.52% of patients had died, 3 intraoperatively. One-year and two year mortality was 17% and 32.5% respectively. Of overall deaths cardiac causes were implicated in 71% (10/147) at 30 days (P The strongest predictor of death at one year was a GFR Conclusion This analysis represents one of the first in Ireland evaluating mortality and morbidity post TAVI implantation. When compared to international data our rates of survival and complications continue to be favourable.

Cardiac amyloidosis typically presents with diastolic heart failure, but asymmetrical septal hypertrophy with outflow tract obstruction has been described. We illustrate the case of a 71-year-old woman with biopsy-proven cardiac amyloidosis and severe medical comorbidities with refractory severe heart failure who had asymmetric septal hypertrophy, systolic anterior motion (SAM) of the mitral valve, and a resting left ventricular outflow tract gradient of 86 mm Hg, increasing to 102 mm Hg on Valsalva maneuver. She underwent percutaneous transluminal septal myocardial ablation (PTSMA) with a dramatic resolution of her SAM and outflow tract obstruction, confirmed by intracavitary pressure wire measurements. PTSMA is technically feasible in this context, and correction of outflow tract obstruction may represent a new therapeutic target in cardiac amyloidosis.

A 58-year-old female with a medical history of ≈2 years of chronic atrial fibrillation (on warfarin treatment), previous right mastectomy for invasive ductal carcinoma, and hypertension presented with a hypertensive emergency and mental status changes. Her symptoms resolved with appropriate antihypertensive treatment. The international normalized ratio on presentation was 2.9. During an additional work-up for transient neurological symptoms, transesophageal echocardiography was performed, revealing a 2×2-cm, well-circumscribed, spherical, mobile echodensity within the ostium of the left atrial appendage (LAA), …

Idiopathic dilated cardiomyopathy is often familial, and apparently healthy relatives may have latent, early, or undiagnosed established disease.To determine the prevalence and natural history of asymptomatic cardiac abnormalities among sampled relatives of unselected patients referred for management of dilated cardio-myopathy.Prospective cohort study.767 asymptomatic relatives of 189 consecutive unselected patients with dilated cardiomyopathy.Clinical evaluation, including history, physical examination, electrocardiography, and echocardiography, was performed. Participants were classified in accordance with published echocardiographic criteria. Sampled relatives who did not have evidence of dilated cardiomyopathy at the initial evaluation were followed for a median of 57 months (range, 1 to 133 months).Of the 767 patients evaluated, 592 (77.2%) were assessed as healthy, 35 (4.6% [95% CI, 3.7% to 7.6%]) had dilated cardiomyopathy, 119 (15.5% [CI, 12.5% to 18.8%]) had left ventricular enlargement without systolic dysfunction, and 21 (2.7% [CI, 1.9% to 4.9%]) had depressed fractional shortening without ventricular dilatation. At follow-up, progression to dilated cardiomyopathy occurred in 13 (10%) relatives with left ventricular enlargement or depressed fractional shortening versus 3 (1.3%) healthy relatives. In a multivariate model, only left ventricular enlargement or depressed fractional shortening independently predicted progression to dilated cardiomyopathy (hazard ratio, 10.0 [CI, 2.8 to 35.5]; P0.001).Because relatives had to be willing to participate and be available geographically, selection bias may have occurred.Treatable asymptomatic dilated cardiomyopathy was identified in 4.6% of asymptomatic relatives. In addition, left ventricular enlargement and depressed fractional shortening were common in asymptomatic relatives of patients with dilated cardiomyopathy and were associated with a statistically significant medium-term risk for disease progression. Evaluation of relatives of patients with cardiomyopathy is recommended.

Echocardiography plays an increasingly important role in diagnosis and management of patients with infective and noninfective endocarditis. Significant changes to diagnostic criteria for endocarditis have occurred, and there are evolving trends associated with an older population presenting with endocarditis, greater numbers of patients with prosthetic heart valves and complex congenital heart disease, and an exponential increase in the numbers of patients eligible for pacing and defibrillator implants. The diagnostic accuracy of echocardiography has been enhanced by developments such as multiplanar transesophageal probes, real-time three-dimensional echocardiography, and intracardiac echocardiography. Standard echocardiography techniques now define the need for and timing of surgical intervention in endocarditis and may help determine the duration of treatment and follow-up after diagnosis.

To determine the frequency of systolic impairment (SI) and its impact on the natural history of hypertrophic cardiomyopathy (HCM).1080 patients (mean (SD) age 43 (15) years, 660 men) with HCM were evaluated. Initial assessment included history, examination, 48 hour Holter monitoring, cardiopulmonary exercise testing, and echocardiography; SI was defined as a fractional shortening (FS)or = 25%. Survival data were collected at clinic visits or by direct communication with patients and their general practitioners. The results of serial echocardiography in 462 patients with normal FS at presentation are also reported.26 (2.4%) patients (49 (14) years, 18 men) had SI at the initial visit. During follow up (58 (49) months), nine (34.6%) died or underwent cardiac transplantation compared with 108 (10.2%) patients with normal FS (p = 0.01). Five year survival from death (any cause) or transplantation was 90.1% (95% confidence interval (CI) 87.8 to 92.4) in patients with normal systolic function versus 52.4% (95% CI 25.2 to 79.6, p0.0001) in patients with SI. In patients who underwent serial echocardiography, 22 (4.8%, aged 41 (15) years) developed SI over 66 (40) months; the annual incidence of SI was 0.87% (95% CI 0.54 to 1.31). On initial evaluation patients who developed SI had a higher frequency of syncope (67 (15.2%) v 10 (45.5%) of those who did not develop SI, p = 0.001), non-sustained ventricular tachycardia (91 (20.6%) v 11 (50%), p = 0.002), and an abnormal blood pressure response on exercise (131 (29.7%) v 15 (68.2%), p = 0.001). Patients with SI had greater wall thinning (p = 0.001), left ventricular cavity enlargement (p0.0005), and deterioration in New York Heart Association functional class (p = 0.001) during follow up. Thirteen (59.1%) patients who progressed to SI died or underwent transplantation compared with 38 (8.6%) patients who maintained normal systolic function.SI is an infrequent complication of HCM but, when present, is associated with a poor prognosis.

Idiopathic dilated cardiomyopathy is a common cause of heart failure. Half of cases are believed to be hereditary, and mutations in cardiac sarcomeric contractile protein genes have been reported with autosomal dominant inheritance. We used mutation analysis suitable for identification of both dominant and recessive mutations to investigate the sarcomeric gene for cardiac troponin I (TNNI3) in 235 patients with dilated cardiomyopathy. We identified a novel TNNI3 mutation in a family with recessive disease. Functional studies showed impairment of troponin interactions that could lead to diminished myocardial contractility. TNNI3 is the first recessive gene identified for this condition, and we suggest that other such genes could be pinpointed by mutation analyses designed to identify homozygous mutations.

Isolated left ventricular noncompaction (LVNC) is a form of cardiomyopathy that most commonly presents in infancy with a hypertrophic and dilated left ventricle characterized by deep trabeculations and intertrabecular recesses. Our goal was to determine the frequency of mutations in G4.5, alpha-dystrobrevin, and FK Binding protein-12 in isolated LVNC patients. No mutations were identified in 47 of the 48 patients studied, while a splice site acceptor site mutation of intron 10 of G4.5 was identified in one patient, resulting in the deletion of exon 10 from the mRNA.

Numerous studies have shown that biventricular pacing (BVP) improves symptoms and exercise capacity in patients with congestive cardiac failure (CCF) and left bundle branch block (LBBB). The mechanism of symptomatic improvement has been attributed to changes in ventricular synchrony. However, the degree of asynchrony is not a good predictor of response.1,2 The aim of this study was to assess whether the presence of a restrictive mitral filling pattern, previously shown to be a marker for diastolic ventricular interaction (DVI) in patients with CCF,3 might identify a cohort of patients more likely to respond to BVP.

OBJECTIVES The aim of this study was to determine the natural history of patients with hypertrophic cardiomyopathy (HCM) and severe left ventricular hypertrophy (LVH) (i.e., maximal left ventricular wall thickness [MLVWT] greater than or equal to30 mm) and whether changes in cardiac morphology influence the course of the disease.BACKGROUND Severe LVH is common in young and rare among elderly patients with HCM. This has been explained by a high incidence of sudden death. We hypothesized that this age-related difference might be explained by left ventricular wall thinning.METHODS A total of 106 (age 33 +/- 15 years; 71 males) consecutive patients with severe LVH underwent history taking, examination, electrocardiography, echocardiography, cardiopulmonary exercise testing, and Holter analysis. Survival data were collected at subsequent clinic visits or by communication with patients and their general practioners. In order to assess morphologic and functional changes, 71 (67.0%) patients (mean age 31 +/- 15 years; 47 males) followed at our institution underwent serial (greater than or equal to1 year) assessment.RESULTS Of the 106 patients, the majority (78 [71.6%]) were

Objectives: To determine whether pregnancy is well tolerated in hypertrophic cardiomyopathy. Setting: Referral clinic. Design: The study cohort comprised 127 consecutively referred women with hypertrophic cardiomyopathy. Forty (31.5%) underwent clinical evaluation before pregnancy. The remaining 87 (68.5%) were referred after their first pregnancy. All underwent history, examination, electrocardiography, and echocardiography. Pregnancy related symptoms and complications were determined by questionnaire and review of medical and obstetric records where available. Results: There were 271 pregnancies in total. Thirty six (28.3%) women reported cardiac symptoms in pregnancy. Over 90% of these women had been symptomatic before pregnancy. Symptoms deteriorated during pregnancy in fewer than 10%. Of the 36 women with symptoms during pregnancy, 30 had further pregnancies. Symptoms reoccurred in 18 (60%); symptomatic deterioration was not reported. Heart failure occurred postnatally in two women (1.6%). No complications were reported in 19 (15%) women who underwent general anaesthesia and in 22 (17.4%) women who received epidural anaesthesia, three of whom had a significant left ventricular outflow tract gradient at diagnosis after pregnancy. Three unexplained intrauterine deaths occurred in women taking cardiac medication throughout pregnancy. No echocardiographic or clinical feature was a useful indicator of pregnancy related complications. Conclusions: Most women with hypertrophic cardiomyopathy tolerate pregnancy well. However, rare complications can occur and therefore planned delivery and fetal monitoring are still required for some patients.

Acute coronary syndromes are characterized by the expression of proinflammatory cytokines such as C-reactive protein (CRP). Sustained upregulation of inflammatory markers is associated with an adverse prognosis. Vitamin E is known to have significant anti-inflammatory properties and has been associated with a reduction in cardiovascular events in some studies of high-risk patients. The mechanism of benefit remains controversial. We conducted a randomized, double-blind placebo controlled trial of vitamin E 400 IU daily for 6 months in 110 patients with acute coronary syndromes. Serum samples were collected at enrollment and at 2, 4, and 6 months. CRP, interleukin-6 and the soluble cell adhesion molecules were measured. Vitamin E levels increased significantly in the treatment group (from 31 micromol/l at baseline to 51 micromol/l, p