Multiparametric assessment of right ventricular function in heart transplant recipients by echocardiography and relations with pulmonary hemodynamics.

OBJECTIVE: Right ventricular (RV) dilatation and dysfunction are usually present in heart transplant (HTx) patients and worsened with residual pulmonary hypertension (PH). We aimed to determine the ability of different echocardiographic modalities to evaluate RV function in comparison with cardiac magnetic resonance (CMR) and their relations with pulmonary hemodynamics in HTx patients. METHODS: A total of 62 data sets [echocardiographic, hemodynamic, and CMR] were acquired from 35 HTx patients. Comprehensive echocardiography, including two-dimensional (2D) transthoracic echocardiography, speckle tracking echocardiography, and three-dimensional (3D) echocardiography, was performed. Mean pulmonary artery pressure (mPAP) was obtained invasively from right heart catheterization. The correlations between all echocardiographic parameters and CMR imaging data and the differences between patients with and without residual PH were evaluated. RESULTS: Diastolic and systolic RV volumes and RV ejection fraction (RVEF) by 3D echocardiography correlated strongly with CMR-derived volumes and RVEF (r = .91, r = .79, r = .64; p < .0001 for each, respectively). Among other parameters, RV fractional area change (r = .439; p < .001) and RV free wall longitudinal strain (RVFW-LS) (r = -.34; p < .05) correlated moderately with CMR-RVEF, whereas tricuspid annulus S' velocity (r = .29; p < .05) and tricuspid annular systolic plane excursion (r = .27; p < .05) correlated weakly with CMR-RVEF. Additionally, 3D-RVEF and RVFW-LS were significantly decreased in studies with mPAP ≥ 20 mm Hg in comparison to those with mPAP < 20 mm Hg (47.7 ± 3.7 vs. 50.9 ± 5.3, p = .04 and -15.5 ± 3.1 vs. -17.5 ± 3, p = .03, respectively). CONCLUSION: The best method for the evaluation of RV function in HTx recipients is 3D echocardiography. Besides, the subclinical impact of residual PH on RV function can be best determined by RVFW-LS and 3D-RVEF in these patients.

Pulmonary Artery Distensibility is Worsened in Obstructive Sleep Apnea Syndrome.

BACKGROUND: Obstructive sleep apnea syndrome (OSAS) leads to right ventricular (RV) dysfunction and pulmonary hypertension (PH) in the later stages. Early determination of these conditions is very important. OBJECTIVES: We aimed to evaluate the correlations of pulmonary artery distensibility, right pulmonary artery fractional shortening (RPA-FS), and pulmonary artery stiffness (PAS) with PH among newly diagnosed OSAS patients. METHODS: We prospectively evaluated 34 newly diagnosed OSAS patients and 28 controls. The study subgroups were determined according to the apnea-hypopnea index (AHI). All patients underwent a transthoracic echocardiographic examination. Conventional RV parameters, PAS, and RPA-FS parameters were measured. RESULTS: RPA-FS was significantly lower in the OSAS group (p < 0.001) and positively correlated with tricuspid annular systolic excursion (TAPSE) (p = 0.047) and pulmonary acceleration time (PAT) (p = 0.006), and inversely correlated with systolic pulmonary artery pressure (sPAP) (p = 0.013), and PAS (p < 0.001). Consistent with this result, PAS was significantly worse in the patients with OSAS compared to the controls (27.1 ± 3.5 to 15.8 ± 2.7, p < 0.001), and inversely correlated with RPA-FS (p < 0.001), PAT (p = 0.001), and TAPSE (p = 0.035). PAS was positively correlated with sPAP (p = 0.001). There were statistically significant differences for both PAS and RPA-FS among the OSAS subgroups with regards to the severity of disease (p < 0.001). The correlation analyses showed a significantly positive correlation between RPA-FS and mean O2 saturation. RPA-FS was also inversely correlated with AHI. Similarly, PAS was positively correlated with AHI and arousal index. CONCLUSIONS: PAS and RPA-FS are worsened in patients with OSAS, and are correlated with PH and severity of OSAS.

Mitral Patient-Prosthesis Mismatch Predicts Suboptimal Hemodynamic Recovery after Mitral Valve Replacement.

BACKGROUND: A possible relationship between an obstructive prosthesis and suboptimal hemodynamic recovery, as reflected by unsatisfactory regression in systolic pulmonary artery pressure (sPAP) and functional tricuspid regurgitation (FTR) following mitral valve replacement (MVR), was investigated. A delineating effective orifice area index (EOAI) value was sought in order to define a patient-prosthesis mismatch. METHODS: A total of 128 patients undergoing isolated mechanical MVR were followed up for a mean of 46 ± 9 months. Patients were allocated to two groups. Group I comprised 83 patients (65%) exhibiting a satisfactory (≥30%) regression in sPAP and FTR, while group II comprised 45 patients with a <30% (suboptimal) decrease in these parameters. A cutoff value for the prosthetic mitral valve in-vivo EAOI was explored as a predictor of postoperative hemodynamic recovery. RESULTS: The mean in-vivo EOAI differed significantly between the groups (1.23 cm2/m2 in group I versus 1.11 cm2/m2 in group II; p <0.0001). The consequent receiver operating characteristic curve analysis revealed an EOAI of 1.19 cm2/m2 as the cut-off value, below which a suboptimal postoperative regression in pulmonary hypertension and FTR is predicted. CONCLUSIONS: An in-vivo EOAI <1.19 cm2/m2 strongly suggests a suboptimal hemodynamic recovery following MVR with the bileaflet mechanical prosthesis used in the present study. Although no direct relationship between prosthesis size and in vivo EOAI was demonstrated, the logical approach is to implant a prosthesis of the largest possible size.

Intracoronary epinephrine in the treatment of refractory no-reflow after primary percutaneous coronary intervention: a retrospective study.

BACKGROUND: Despite the advances in medical and interventional treatment modalities, some patients develop epicardial coronary artery reperfusion but not myocardial reperfusion after primary percutaneous coronary intervention (PCI), known as no-reflow. The goal of this study was to evaluate the safety and efficacy of intracoronary epinephrine in reversing refractory no-reflow during primary PCI. METHODS: A total of 248 consecutive STEMI patients who had undergone primary PCI were retrospectively evaluated. Among those, 12 patients which received intracoronary epinephrine to treat a refractory no-reflow phenomenon were evaluated. Refractory no-reflow was defined as persistent TIMI flow grade (TFG) ≤ 2 despite intracoronary administration of at least one other pharmacologic intervention. TFG, TIMI frame count (TFC), and TIMI myocardial perfusion grade (TMPG) were recorded before and after intracoronary epinephrine administration. RESULTS: A mean of 333 ± 123 mcg of intracoronary epinephrine was administered. No-reflow was successfully reversed with complete restoration of TIMI 3 flow in 9 of 12 patients (75%). TFG improved from 1.33 ± 0.49 prior to epinephrine to 2.66 ± 0.65 after the treatment (p < 0.001). There was an improvement in coronary flow of at least one TFG in 11 (93%) patients, two TFG in 5 (42%) cases. TFC decreased from 56 ± 10 at the time of no-reflow to 19 ± 11 (p < 0.001). A reduction of TMPG from 0.83 ± 0.71 to 2.58 ± 0.66 was detected after epinephrine bolus (p < 0.001). Epinephrine administration was well tolerated without serious adverse hemodynamic or chronotropic effects. Intracoronary epinephrine resulted in significant but tolerable increase in heart rate (68 ± 13 to 95 ± 16 beats/min; p < 0.001) and systolic blood pressure (94 ± 18 to 140 ± 20; p < 0.001). Hypotension associated with no-reflow developed in 5 (42%) patients. During the procedure, intra-aortic balloon pump counterpulsation was required in two (17%) patients, transvenous pacing in 2 (17%) cases, and both intra-aortic balloon counterpulsation and transvenous pacing in one (8%) patients. One patient (8%) died despite all therapeutic measures. CONCLUSION: Intracoronary epinephrine may become an effective alternative in patients suffering refractory no-reflow following primary PCI.

Cognitive Decline in Patients with Leukoaraiosis Within 5 Years after Initial Stroke.

BACKGROUND: Leukoaraiosis (LA) is closely associated with cognitive deficits. The association between LA and cognitive disorders, such as mild cognitive impairment (MCI) and dementia, after initial stroke has not been systematically studied. In this study, we sought to identify whether LA contributes to the occurrence of certain type of cognitive disorders after initial stroke. METHODS: Data from our Stroke Registry were examined, and 5-year follow-up data for LA and cognitive disorders were analyzed. We performed Kaplan-Meier analysis and log-rank test to assess the predictive value of LA for risk of cognitive decline and the Cox proportional hazards model to test the risk factors studied as independent determinants of cognitive impairment. RESULTS: The frequency of patients with normal cognitive function decreased significantly at 5 years compared with initial stroke (78% vs 70%; odds ratio, 1.51; 95% confidence interval, 1.41-1.62). Of 8784 patients, 1659 (19%) had dementia and 964 (11%) had MCI at the final analysis. After 5 years of follow-up, survival analysis showed that all patients with LA had an increased probability of MCI compared with those without LA (P < .0001). Patients with LA had an increased chance of dementia compared with those without LA (P < .0001) at the end of follow-up. Cognitive decline probability was significantly higher in patients with severe LA compared with those with mild/moderate LA (P < .0001). Cox regression analyses showed that recurrence of stroke (hazard ratio [HR], 3.92 [95% CI, 3.26-4.72]), hypertension (HR, 1.11 [95% CI, 1.0-1.22]), LA (HR, 1.15 [95% CI, 1.05-1.25]), age (HR, 1.05 [95% CI, 1.04-1.06]), hypercholesterolemia (HR, .86 [95% CI, .77-.95]), higher LDL cholesterol (HR, 1.21 [95% CI, 1.11-1.32]), lower HDL cholesterol (HR, .90 [95% CI, .83-.98]), coronary heart disease (HR, .85 [95% CI, .77-.94]), and National Institutes of Health Stroke Scale score at admission (HR, .77 [95% CI, .72-.82]) were also significantly associated with cognitive impairments. CONCLUSIONS: Our findings suggest that patients with LA may be at risk of developing new cognitive impairments at long-term period after initial stroke. The evaluation of the concomitant risk factors, besides providing insights about the possible mechanisms behind the cognitive dysfunction present in LA, may be of help for the prevention of cognitive impairments.

Myocardial Infarction in Young Adults: Diagnosis Begins Through Inspection.

Spontaneous coronary artery dissection (SCAD) is an atypical cause of myocardial infarction, predominantly seen in women. Among various predisposing factors, genetic vasculopathies such as connective tissue diseases significantly contribute to SCAD. This report discusses a 36-year-old male diagnosed with vascular type Ehlers-Danlos syndrome following an anterior myocardial infarction and explores relevant literature.

The relationship between changes in peritoneal membrane solute transfer characteristics and cardiac remodeling in patients with peritoneal dialysis.

INTRODUCTION: We aimed to determine the relationship between the dilatation of the heart chambers and the change in peritoneal membrane solute transfer characteristics (PMTC) in long-term peritoneal dialysis (PD) patients. METHODS: This is a retrospective, single-center study including the follow-up of maintenance PD patients. According to the changes in PMTC from baseline to the last visit, patients were divided into three groups; stable (n = 11), increased (n = 41), and decreased transporters (n = 35). RESULTS: Left atrium (LA) and Right ventricle (RV) dilatation were more prominent in the PMTC-decreased group compared to PMTC-increased and stable groups (p < 0.001 and p = 0.07, respectively). The Cox regression analysis showed that only decreased PMTC was associated with LA dilatation (HR 2.89 [CI 95%1.54, 5.45] p < 0.01) and RV dilatation (HR 3.01 [CI 95%1.40, 6.21] p < 0.01). CONCLUSION: PD can be associated with unfavorable dynamic changes in cardiac structure and functions even at the subclinical level.

The prognostic value of right ventricular outflow tract velocity time integral in patients with pulmonary hypertension.

AIMS: Right ventricular (RV) failure is one of the leading causes of death in patients with pulmonary hypertension (PH). Conventional echocardiographic parameters are not included in risk stratification and follow-up for prognostic assessment due to PH's diverse nature and the RV's complex geometry. RV outflow tract velocity time integral (RVOT VTI) is a simple, non-invasive estimate of pulmonary flow and an echocardiographic surrogate of RV stroke volume. In this study, we aimed to define the prognostic value of RVOT VTI in PH patients. METHODS: Sixty-three subjects with idiopathic PAH (IPAH) (n = 23), connective tissue disease-associated PAH (CTD-associated PAH) (n = 19) and chronic thromboembolic pulmonary hypertension (CTEPH) (n = 21) were retrospectively included. A comprehensive two-dimensional echocardiographic evaluation, including RVOT-VTI measurement, was performed during the follow-up and the New York Heart Association functional class (NYHA FC), 6 min walk distance (6MWD) and brain natriuretic peptide (BNP) levels were recorded. RESULTS: The median age of the whole cohort was 63 years (52-68), and 47 (74.6%) of the patients were women. The median follow-up period was 20 months (11-33), and 20 (31.7%) patients died in this period. BNP values were higher [317 (210-641) vs 161 (47-466), P = 0.02], and 6MWD values were lower [197.5 ± 89.5 vs 339 ± 146.3, P < 0.0001] in the non-survivor group, and the non-survivor group had a worse NYHA-FC (P = 0.02). Among echocardiographic data, tricuspid annular plane systolic excursion (TAPSE) (15.4 ± 4.8 vs 18.6 ± 4.2, P = 0.01) and RVOT VTI (11.9 ± 4.1 vs 17.2 ± 4.3, P < 0.0001) values were lower whereas right atrial area (RAA) (26.9 ± 10.1 vs 22.2 ± 7.1, P = 0.04) values were higher in the non-survivor group. The area under curve of the RVOT VTI for predicting mortality was 0.82 [95% confidence interval (CI) 0.715-0.940, P < 0.0001], and the best cut-off value was 14.7 cm with a sensitivity of 80% and specificity of 77%. Survival was significantly lower in subjects with RVOT VTI ≤ 14.7 cm (log-rank P < 0.0001). Survival rates for patients with RVOT VTI ≤ 14.7 cm were 70% at 1 year, 50% at 2 years, %29 at 3 years and 21% at 5 years. The univariate determinants of all-cause mortality were BNP [hazard ratio (HR) 1.001 (1.001-1.002), P = 0.001], 6MWD [HR 0.994 (0.990-0.999), P = 0.012] and NYHA-FC III-IV [HR 3.335 (1.103-10.083), P = 0.03], TAPSE [HR 0.838 (0.775-0.929), P = 0.001], RAA [HR 1.072 (1.013-1.135), P = 0.016] and RVOT VTI [HR 0.819 (0.740-0.906), P < 0.0001]. RVOT VTI was found to be the only independent determinant of mortality [HR 0.857 (0.766-0.960), P = 0.008]. CONCLUSIONS: The decreased RVOT VTI predicts mortality in patients with PH and each 1 mm decrease in RVOT VTI increases the risk of mortality by 14.3%. This parameter might serve as an additional parameter in the follow-up of these patients especially when 6MWD and NYHA-FC could not be determined.

[Mean platelet volume and the extent of coronary atherosclerosis in patients with stable coronary artery disease]. / Kararli anjinasi olan koroner arter hastalarinda ortalama trombosit hacmi ve koroner aterosklerozun yayginligi

OBJECTIVES: The aim of this study was to assess the relationship between mean platelet volume (MPV) and the presence and extent of coronary artery disease (CAD) in patients who underwent coronary angiography for stable chest pain. STUDY DESIGN: A total of 540 patients (350 male, 190 female; mean age: 59.6±11.4 years) were consecutively included in the study. The patients were divided into four groups according to the presence and extent of their CAD as follows: Group 1 - patients with no significant CAD, group 2 - one vessel disease, group 3 - two vessel diseases, and group 4 - three vessel disease. Also, the Gensini score of each coronary angiogram was calculated. RESULTS: There were 159 patients in group 1, 169 in group 2, 110 in group 3, and 102 in group 4. As expected, we found significant differences among the groups regarding mean age and other coronary risk factors including gender, hypertension, diabetes mellitus, hyperlipidemia, smoking, and family history of ischemic heart disease. However, there were no significant differences among the groups regarding platelet counts and MPV values (8.5±0.1 fl, 8.5±1.2 fl, 8.6±0.9 fl and 8.6±0.9 fl, MPV values of groups 1-4, respectively). Although the Gensini score was found to be significantly correlated with age, plasma uric acid level, white blood cell count, hemoglobin level, fasting blood glucose, and high density lipoprotein level, no significant association was detected between MPV and Gensini score values. CONCLUSION: This study is one of the largest studies assessing the relationship between MPV and the extent of coronary atherosclerosis in patients with stable CAD to date. However, we found no association between MPV and the presence and extent of coronary atherosclerosis.

Heritable Pulmonary Arterial Hypertension Diagnosed during the Postpartum Period: A Case Report and Literature Review.

Approximately one-third of bone morphogenic protein receptor-2 (BMPR2) mutation carriers develop pulmonary arterial hypertension (PAH), which indicates that additional risk factors are needed for the manifestation of the disease. It is questionable whether pregnancy is a risk factor for PAH development in these patients. We represent a 30-year-old woman with a heterozygous BMPR2 mutation who was diagnosed with PAH during the postpartum period and reviewed the literature in this report. We also discussed the possible underlying mechanisms that might have resulted in PAH development during pregnancy in BMPR2 mutation carriers.

The Usefulness of the TAPSE/sPAP Ratio for Predicting Survival in Medically Treated Chronic Thromboembolic Pulmonary Hypertension.

OBJECTIVE: The ventriculoarterial uncoupling has been linked with unfavorable results as measured noninvasively by tricuspid annular plane systolic excursion divided by systolic pulmonary artery pressure (TAPSE/sPAP). However, its prognostic importance in chronic thromboembolic pulmonary hypertension (CTEPH) is limited. Thus, we determine the effect of the TAPSE/sPAP ratio on outcomes and predictors of all-cause mortality in these patients. METHODS: We analyzed 56 subjects with medically treated CTEPH. Two-dimensional echocardiographic examination and right heart catheterization findings were recorded from the hospital database. Baseline New York Heart Association functional class (NYHA-FC), 6-min walk distance (6MWD), and brain natriuretic peptide (BNP) test results were recorded. RESULTS: The median age was 65.5 years. Over a median follow-up time of 27 months, 29 (51.8%) patients died. BNP values were higher (P = 0.008), 6MWD values were lower (P = 0.004), and NHYA-FC (P = 0.0001) was worse in the non-survivor group. TAPSE (P = 0.0001) and TAPSE/sPAP ratio (P = 0.001) were significantly lower and pulmonary vascular resistance (PVR) was higher in the non-survivor group (P = 0.03). The best cut-off value for the TAPSE/sPAP ratio for predicting mortality was 0.20 mm/mmHg and the survival rates were significantly lower in the TAPSE/sPAP ratio ≤0.20 group (log-rank P = 0.012). 6MWD (P = 0.005), NHYA-FC III-IV (P = 0.0001), TAPSE/sPAP ratio ≤0.20 (P = 0.017), PVR (P = 0.008), and TAPSE/sPAP ratio ≤0.20 combined with NYHA-FC III-IV (P = 0.0001) were significant determinants and TAPSE/sPAP ratio ≤0.20 combined with NYHA-FC III-IV was the only independent predictor of mortality (P = 0.003). CONCLUSION: Medically treated CTEPH patients with a TAPSE/sPAP ratio ≤0.20 had lower survival rates. TAPSE/sPAP ratio≤0.20 combined with NYHA-FC III-IV was the independent predictor of poor prognosis.

Approach to optimal assessment of right ventricular remodelling in heart transplant recipients: insights from myocardial work index, T1 mapping, and endomyocardial biopsy.

AIMS: Right ventricular (RV) dysfunction is an important cause of graft failure after heart transplantation (HTx). We sought to investigate relative merits of echocardiographic tools and cardiac magnetic resonance (CMR) with T1 mapping for the assessment of functional adaptation and remodelling of the RV in HTx recipients. METHODS AND RESULTS: Sixty-one complete data set of echocardiography, CMR, right heart catheterization, and biopsy were obtained. Myocardial work index (MWI) was quantified by integrating longitudinal strain (LS) with invasively measured pulmonary artery pressure. CMR derived RV volumes, T1 time, and extracellular volume (ECV) were quantified. Endomyocardial biopsy findings were used as the reference standard for myocardial microstructural changes. In HTx recipients who never had a previous allograft rejection, longitudinal function parameters were lower than healthy organ donors, while ejection fraction (EF) (52.0 ± 8.7%) and MWI (403.2 ± 77.2 mmHg%) were preserved. Rejection was characterized by significantly reduced LS, MWI, longer T1 time, and increased ECV that improved after recovery, whereas RV volumes and EF did not change MWI was the strongest determinant of rejection related myocardial damage (area under curve: 0.812, P < 0.0001, 95% CI: 0.69-0.94) with good specificity (77%), albeit modest sensitivity. In contrast, T1 time and ECV were sensitive (84%, both) but not specific to detect subclinical RV damage. CONCLUSION: Subclinical adaptive RV remodelling is characterized by preserved RV EF despite longitudinal function abnormalities, except for MWI. While ultrastructural damage is reflected by MWI, ECV, and T1 time, only MWI has the capability to discriminate functional adaptation from transition to subclinical structural damage.

Left atrial mechanics for secondary prevention from embolic stroke of undetermined source.

AIMS: Anticoagulation is not justified unless atrial fibrillation (AF) is detected in cryptogenic stroke (CS) patients. We sought to explore whether left atrial (LA) remodelling is associated with embolic stroke of undetermined source (ESUS). METHODS AND RESULTS: In this prospective study, we evaluated consecutively 186 patients in sinus rhythm who presented with an acute ischaemic stroke (embolic and non-embolic) and sex- and age-matched controls. We performed continuous electrocardiogram (ECG) monitoring to capture paroxysmal AF episodes as recommended by the guidelines. After 12 months of follow-up, continuous ECG monitoring was repeated in patients with undetected AF episodes. We quantified LA reservoir and contraction strain (LASr and LASct) by speckle-tracking, LA volumes by 3D echocardiography. Out of 186 patients, 149 were enrolled after comprehensive investigation for the source of ischaemic stroke and divided into other cause (OC) (n = 52) and CS (n = 97) groups. CS patients were also subdivided into AF (n = 39) and ESUS (n = 58) groups. Among CS patients, LA strain predicted AF independently from CHARGE-AF score and LA volume indices. ESUS group, despite no captured AF, had significantly worse LA metrics than OC and control groups. AF group had the worst LA metrics. Moreover, LASr predicted both CS (embolic stroke with and without AF) and ESUS (embolic stroke with no detected AF) independently from LAVImax and CHA2DS2-VASc score. LASr >26% yielded 86% sensitivity, 92% specificity, 92% positive, and 86% negative predictive values for the identification of ESUS (areas under curve: 0.915, P < 0.0001, 95% confidence interval: 0.86-0.97). CONCLUSION: Echocardiographic quantification of LA remodelling has great potential for secondary prevention from ESUS.

[Extraction of a large vegetation and ICD lead using the Evolution Mechanical Dilator Sheath]. / ICD elektrodu üzerinde gelisen büyük bir vejetasyonun, elektrot ile birlikte Evolution mekanik dilatör kilif kullanilarak çikarilmasi

A 74-year-old male patient presented with complaints of fatigue, fever, and worsening health status. He had a 34-month history of cardioverter-defibrillator (ICD) implantation due to ischemic cardiomyopathy. Transthoracic and transesophageal echocardiography showed a mobile mass, 24 x 11 mm in size, consistent with a vegetation attached to the right ventricular ICD lead. Surgical removal of the electrode was considered to be highly risky for the patient, thus percutaneous removal was decided. Due to adhesions, manual traction of the lead with a standard stylet was ineffective. Complete lead extraction was accomplished using the Evolution Mechanical Dilator Sheath, which is a new mechanical sheath with a stainless steel bladed tip. No complications occurred during or after the procedure. Following a histopathologic diagnosis of vegetation, the patient received a six-week antibiotic therapy, after which a new ICD was implanted on the contralateral side. To our knowledge, this case represents the largest lead vegetation extracted by this new device. Although its safety should be validated by increasing number of cases, it seems that its simple use would make this device a new interesting tool among the instruments available for lead extraction.

[Acute results of percutaneous mitral balloon valvuloplasty]. / Perkütan mitral balon valvüloplastinin kisa dönem sonuçlari

OBJECTIVES: We aimed to analyze acute clinical, echocardiographic, and hemodynamic results and long-term event-free survival of percutaneous mitral balloon valvuloplasty (PMBV) in the treatment of hemodynamically significant mitral stenosis (MS). STUDY DESIGN: We retrospectively reviewed 577 patients (454 females, 123 males; mean age 38±11 years) who underwent PMBV for moderate or severe MS. Acute procedural success was defined as the achievement of mitral valve area (MVA) >1.5 cm2 and absence of grade 3-4 mitral regurgitation. In addition, 489 patients were interrogated by phone calls for event-free survival (death, redo PMBV, mitral valve replacement) after at least one year (mean 54 months) of the procedure. RESULTS: The procedure was successful in 547 patients (94.8%) and unsuccessful in 30 patients (5.2%). Among baseline echocardiographic and hemodynamic parameters, MVA was significantly lower (p=0.0001) and moderate/severe tricuspid regurgitation was significantly more common (p=0.031) in patients with failure. The mean Wilkins scores were similar in the two groups (p>0.05). Failure was related to suboptimal valve opening (MVA <1.5 cm2) in 20 patients (66.7%), and grade 3-4 mitral regurgitation in 10 patients (33.3%). There were no in-hospital death, cardiac tamponade, or cerebrovascular accident. Acute complications included iatrogenic atrial septal defect (n=51, 8.8%) and groin hematoma (n=4, 0.7%). Inquiry for long-term outcomes showed that four patients had died, while 21 patients and six patients had undergone mitral valve replacement and redo PMBV, respectively. CONCLUSION: Our data suggest that PMBV is a safe and effective treatment option in MS and preprocedural MVA and tricuspid regurgitation are associated with acute failure of the procedure.

Longitudinal Strain and Strain Rate for Estimating Left Ventricular Filling Pressure in Heart Transplant Recipients.

Traditional parameters have limited value to estimate left ventricular filling pressure (LVFP) in orthotropic heart transplant (OHT) recipients. We hypothesized that global longitudinal strain (GLS), diastolic, and systolic strain rate (SR) would be depressed in OHT recipients with elevated LVFP and could overcome the limitations of traditional parameters. We studied consecutively OHT patients at the time of endomyocardial biopsies and retrospectively pretransplantation studies conforming to the same protocol. Comprehensive echocardiography with strain measurements was performed. Results were compared with pulmonary capillary wedge pressure (PCWP) obtained from right heart catheterization that was performed just after the echocardiography study. In all, 74 studies were performed in 50 OHT recipients. Mean PWCP was 11.8 ± 4.3 mm Hg (range: 4 to 25 mm Hg). Several parameters, but not left atrial volume index, mitral inflow velocities, annular velocities, and their ratio (E/e'), were different between studies with normal (n = 47) and elevated PCWP (n = 27). Area Under Curve for GLS (0.932*), E/e'SR (0.849*), and systolic SR (0.848*) (*p <0.0001) were more accurate than traditional parameters for predicting PCWP>12 mm Hg. GLS, systolic SR and E/e'SR remained accurate regardless of LV ejection fraction and allograft vasculopathy. Meanwhile, E/e' was accurate to predict PWCP in native failing hearts before transplantation. Changes in GLS and E/e'SR tracked accurately changes in PCWP. In conclusion, traditional indices of diastolic function perform poorly in OHT recipients, whereas GLS and E/e'SR provide reliable means of LVFP, irrespective of ejection fraction and allograft vasculopathy. These parameters also track reasonably well the changes in LVFP.

Cardiac Involvement in Glycogen Storage Disease Type IV: Two Cases and the Two Ends of a Spectrum.

Glycogen storage disease type IV (GSD IV) is an autosomal recessive disorder due to the deficiency of α 1,4-glucan branching enzyme, resulting in an accumulation of amylopectin-like polysaccharide in various systems. We describe two cases, a 23-year-old girl with dilated cardiomyopathy who presented with progressive dyspnea and fatigue and a 28-year-old girl with hypertrophic cardiomyopathy who was asymptomatic, secondary to the accumulation of amylopectin-like fibrillar glycogen, in heart. In both patients, the diagnosis was confirmed by enzyme assessment. Our patients showed that GSD IV is not only liver or skeletal muscle disease, but also it can be presented in different form of the spectrum of cardiomyopathy from dilated to hypertrophic and from asymptomatic to decompensated heart failure. Also, to our knowledge, this is the first hypertrophic cardiomyopathy case due to GSD IV in the literature.

[Coronary artery ectasia: its frequency and relationship with atherosclerotic risk factors in patients undergoing cardiac catheterization]. / Koroner arter ektazisi: Koroner anjiyografi uygulanan hastalardaki sikligi ve aterosklerotik risk faktörleri ile iliskisi.

OBJECTIVE: Coronary artery ectasia (CAE) is defined as local or generalized aneurysmal dilatation of the coronary arteries. We aimed to determine the frequency of CAE at our center and to compare clinical and angiographic characteristics between patients with isolated CAE and CAE with coronary artery disease (CAD). METHODS: From February 2004 to December 2005, 12.514 patients were retrospectively analyzed by two independent operators who underwent coronary angiography. Coronary artery diameters were measured using qualitative computed angiography. CAD risk factors were recorded for all patients. Unpaired Student's t-test and Chi-square test were used for statistical analysis. RESULTS: CAE was diagnosed in 201 patients (1.59%). The majority (78%) were male. The mean age was 61±10.8 years (range, 25 to 82 years). The cases were divided into 2 groups as isolated CAE (Group 1) (14.9%) and CAE with CAD (Group 2) (85.1%). The risk factors of CAD were similar between two groups. The frequency of arterial involvement was: the right coronary artery (RCA) 54.3%; circumflex artery (Cx), 48.3%; the left anterior descending artery (LAD), 40.4%. CAE affected only one major vessel in 64.2% of cases and all 3 vessels in 9%. Isolated CAE was most commonly detected in Cx (47%). The type of CAE was determined according to Markis and Harikrishnan classification. The most prevalent involvement was Markis type 4 and Harikrishnan type 4a. Although atypical angina was the most common clinical presentation in both groups, acute coronary syndrome was more frequent in Group 2 (p=0.018). CONCLUSION: The risk factors of CAD and the manner of clinical presentation were considerably similar in both groups and this situation was consistent with similar etiopathogenesis of two diseases.