Cardiac involvement of primary hyperoxaluria accompanied by non-compaction cardiomyopathy and patent ductus arteriosus.

Primary hyperoxaluria is a rare hereditary metabolic disorder resulting in accumulation of calcium oxalate in visceral organs, including the heart. We report a 19-year-old male with non- compaction cardiomyopathy combined with patent ductus arteriosus awaiting combined liver-kidney transplantation for primary hyperoxaluria. After surgical closure of the patent ductus arteriosus, the patient underwent a successful renal and subsequent liver transplantation. The presence of hypertrophic cardiomyopathy in hyperoxaluria patients has been reported before, but this is the first report of non-compaction myocardium with patent ductus arteriosus in a patient with primary hyperoxaluria. At the third month after combined liver and renal transplantation, improvement in cardiac functions were observed. Primary hyperoxaluria is a clinical entity to be taken into consideration in differential diagnosis of hypertrophied myocardium with high myocardial echocardiographic intensity. In cases of hyperoxaluria, additional congenital abnormalities may complicate the clinical picture.

Remote monitoring of left ventricular assist device parameters after HeartAssist-5 implantation.

Although several left ventricular assist devices (LVADs) have been used widely, remote monitoring of LVAD parameters has been available only recently. We present our remote monitoring experience with an axial-flow LVAD (HeartAssist-5, MicroMed Cardiovascular, Inc., Houston, TX, USA). Five consecutive patients who were implanted a HeartAssist-5 LVAD because of end-stage heart failure due to ischemic (n=4) or idiopathic (n=1) cardiomyopathy, and discharged from hospital between December 2011 and January 2013 were analyzed. The data (pump speed, pump flow, power consumption) obtained from clinical visits and remote monitoring were studied. During a median follow-up of 253 (range: 80-394) days, fine tuning of LVADs was performed at clinical visits. All patients are doing well and are in New York Heart Association Class-I/II. A total of 39 alarms were received from three patients. One patient was hospitalized for suspected thrombosis and was subjected to physical examinations as well as laboratory and echocardiographic evaluations; however, no evidence of thrombus washout or pump thrombus was found. The patient was treated conservatively. Remaining alarms were due to insufficient water intake and were resolved by increased water consumption at night and summer times, and fine tuning of pump speed. No alarms were received from the remaining two patients. We believe that remote monitoring is a useful technology for early detection and treatment of serious problems occurring out of hospital thereby improving patient care. Future developments may ease troubleshooting, provide more data from the patient and the pump, and eventually increase physician and patient satisfaction. Despite all potential clinical benefits, remote monitoring should be taken as a supplement to rather than a substitute for routine clinical visits for patient follow-up.

Right ventricular and septal function in patients with pulmonary hypertension.

OBJECTIVE: Pulmonary hypertension (PHT) exacerbates the functions of both ventricles. This prospective, randomised study was planned to investigate the effects of PHT on kinetics of both ventricles and the septum. METHODS: Twenty-five patients were randomly selected among the patients who had been planned to undergo mitral valve replacement (MVR) because of isolated mitral stenosis and divided into two groups according to their preoperative pulmonary artery pressure (PAP) values. Blood pool gated single photon emission tomography (BPGS) and transthoracic echocardiography were performed. Ventricles' regional, global and functional parameters were also assessed by using pulsed wave Doppler tissue imaging (DTI). RESULTS: Preoperative and postoperative PAP of the group 1 (PAP < 50 mmHg) were 40.0 ± 2.8 and 30.0 ± 2.6 mmHg (p = 0.03), group 2 (PAP ≥ 50 mmHg) were 71.9 ± 4.7 and 50.6 ± 3.5 mmHg (p < 0.05). The global right and left ventricle scores were decreased after the operation. The decrement was only significant in group 2. Considering the septal kinetics, right ventricle septal score was decreased from 7.6 to 3.3 (p < 0.05) in group 1, from 3.8 to 1.6 (p < 0.05) in group 2 postoperatively. CONCLUSION: Following MVR, a decrement in PAP values, and an improvement in ventricular function, especially in the right ventricular and septal kinetics were achieved. Furthermore, it was found that both DTI and BPGS techniques are beneficial to investigate the functional changes postoperatively and in the follow-up period of the patients who undergo mitral valve surgery.

The use of three-dimensional echocardiography in the visualization of pseudoaneurysm of the mitral-aortic intervalvular fibrosa.

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (MAIF) is an uncommon but serious complication of aortic valve endocarditis. A 23-year-old woman was referred to our institution with the diagnosis of aortic valve endocarditis thought to be complicated by an aortic root abscess. Two-dimensional transthoracic echocardiography revealed a vegetation attached to the left coronary cuspis of the aortic valve and a false aneurysm-like structure in the MAIF at the left ventricular outflow tract. The diagnosis of MAIF pseudoaneurysm was confirmed by three-dimensional echocardiography. The patient died a few hours after admission because of worsening of her neurological status. An abscess-like structure detected in a patient with aortic valve endocarditis should be differentiated from a pseudoaneurysm of the MAIF.

A Short-Term In Vivo Evaluation of the Istanbul Heart Left Ventricular Assist Device in a Pig Model.

OBJECTIVES: A continuous-flow centrifugal blood pump system has been recently developed as an implantable left ventricular assist device for patients with endstage heart failure. The objective of this study was to evaluate the initial in vivo performance of a newly developed left ventricular assist device (iHeart or Istanbul heart; Manufacturing and Automation Research Center, Koc University, Istanbul, Turkey) in an acute setting using a pig model. MATERIALS AND METHODS: Three pigs (77, 83, 92 kg) received implants via a median sternotomy, with animals supported for up to 6 hours. An outflow cannula was anastomosed to the ascending aorta. Anticoagulation was applied by intravenous heparin administration. During the support period, pump performance was evaluated under several flow and operating conditions. All pigs were humanely sacrificied after the experiments, and organs were examined macroscopically and histopathologically. RESULTS: Flow rate ranged between 1.5 and 3.6 L/min with pump speeds of 1500 to 2800 revolutions/min and motor current of 0.6 to 1.3 A. Initial findings confirmed thatthe iHeart ventricular assist device had sufficient hydraulic performance to support the circulation. During the experimental period, plasma free hemoglobin levels were found to be within normalranges.Thrombus formation was not observed inside the pump in all experiments. CONCLUSIONS: The iHeart ventricular assist device demonstrated encouraging hemodynamic performance and good biocompatibility in the pig model for use as an implantable left ventricular assist device. Further acute in vivo studies will evaluate the short-term pump performance prior to chronic studies for long-term evaluation.

Coronary artery to left ventricular fistula demonstrated by transthoracic echocardiography.

Coronary artery fistulas are rare coronary anomalies which generally require coronary angiography for definitive diagnosis. Improvements in ultrasound technology has enabled direct, transthoracic visualization of long portions of coronary arteries. We report a patient with a symptomatic coronary to left ventricular fistula, which was diagnosed with transthoracic echocardiography.

Complete regression of massive cardiac involvement associated with acute T cell leukemia following chemotheraphy.

Adult T cell leukemia/lymphomas are aggressive disorders, which infiltrate not only the bone marrow but extensively the visceral organs as well. A case with left ventricular systolic dysfunction with myocardial infiltration and massive pericardial effusion which was demonstrated with echocardiography is discussed. The patient responded well to pericardial drainage and subsequent chemotherapy. The dramatic improvement in echocardiographic findings after chemotherapy gave a clue to investigate suspected patients with aggressive leukemia and lymphomas for exclusion of leukemic infiltration of myocardium.

P wave dispersion is prolonged in patients with Wilson's disease.

AIM: To investigate the P wave dispersion as a non-invasive marker of intra-atrial conduction disturbances in patients with Wilson's disease. METHODS: We compared Wilsonos disease patients (n = 18) with age matched healthy subjects (n = 15) as controls. The diagnosis was based on clinical symptoms, laboratory tests (ceruloplasmin, urinary and hepatic copper concentrations). P wave dispersion, a measurement of the heterogeneity of atrial depolarization, was measured as the difference between the duration of the longest and the shortest P-waves in 12 lead electrocardiography. RESULTS: All the patients were asymptomatic on cardiological examination and have sinusal rhythm in electrocardiography. Left ventricular and left atrial diameters, left ventricular ejection fraction and left ventricular mass index were similar in both groups. The Wilson's disease patients had a significantly higher P wave dispersion compared with the controls (44.7 +/- 5.8 vs 25.7 +/- 2.5, P < 0.01). CONCLUSION: There was an increase in P wave dispersion in cardiologically asymptomatic Wilson's disease patients which probably represents an early stage of cardiac involvement.

Elastic properties of aorta are impaired in patients with slow coronary flow phenomenon.

BACKGROUND: Increased stiffening and decreased distensibility of the large arteries are associated with the presence of coronary artery disease and has been related to increased cardiovascular mortality in different populations. AIM: Aim of this present study was to investigate the elastic properties of the aortic wall in patients with slow coronary flow phenomenon. MATERIAL AND METHOD: We studied 20 patients with slow coronary flow phenomenon (age: 40+/-12 years)and 15 normal control subjects by echocardiography. Aortic strain (%) and distensibility (10(-3) mmHg(-1)) were calculated from the echocardiographically-derived thoracic Ao diameters (mm). The measurement of pulse pressure was obtained by cuff sphygmomanometry. RESULTS: There was no difference in the left ventricular ejection fraction, left ventricular end-diastolic and end-systolic diameters, left atrial diameters, left ventricular mass index between patients with slow coronary flow phenomenon and control groups. Maximal aortic diastolic diameter was increased in patients with slow coronary flow phenomenon compared with control group (p<0.05). Ao distensibility and Ao strain were lower in the patients with slow coronary flow phenomenon compared with control group (p<0.05). CONCLUSION: Reduced thoracic aortic elastic properties in patients with slow coronary flow phenomenon,assessed by echocardiography, apart from demonstrating subclinical atherosclerosis may also contribute to the etiopathogenesis of the slow coronary flow phenomenon necessitating more aggressive primary preventive measure.

A case of simultaneous anterior, inferior, and right ventricular ST-segment elevation myocardial infarction due to occlusion of the wrapped left anterior descending coronary artery.

Simultaneous anterior, inferior, and right ventricular ST-segment elevation myocardial infarction is an unusual condition. A 50-year-old male patient presented with severe, squeezing chest pain of an hour onset. ST-segment elevations were detected in all precordial derivations, DII, DIII, aVF and V3R, V4R. Coronary angiography showed a significant lesion in the left anterior descending (LAD) coronary artery, just below the second diagonal branch. Balloon predilatation was performed after 40 minutes of admission, followed by bare metal stent implantation, which resulted in ST-segment resolution in all leads and relief of chest pain. Echocardiography showed dyskinesia of the left ventricular apical wall, and hypokinesia of the interventricular septum and inferior wall. Left ventricular ejection fraction was 40%. Coronary angiography and cardiac CT angiography demonstrated a wrapped LAD. The patient was discharged five days after percutaneous coronary intervention (PCI) with stabilization of his clinical status. Ten days after PCI, he presented with chest pain associated with left ventricular anterior and inferior reinfarction. Successful balloon dilatation was performed for thrombotic in-stent restenosis. His clinical condition improved and he was asymptomatic for a month, during which no signs of deterioration were observed in electrocardiographic and echocardiographic findings.

The effect of baseline pulmonary artery pressure on right ventricular functions after mitral balloon valvuloplasty for rheumatic mitral stenosis: a tissue Doppler imaging study.

OBJECTIVES: We evaluated the effect of baseline pulmonary artery pressure (PAP) on right ventricular functions after percutaneous mitral balloon valvuloplasty (PMBV) for rheumatic mitral stenosis (MS). STUDY DESIGN: The study included 56 patients (15 males, 41 females; mean age 35 years) who underwent PMBV for isolated rheumatic MS. The patients were divided into two groups according to the baseline median systolic pulmonary artery pressure (PAP > or =40 mmHg, n=33; PAP <40 mmHg, n=23) measured before PMBV by echocardiography. Right ventricular function was assessed by pulse wave Doppler tissue imaging and the Tei index. Assessments were repeated 48 hours and three months after PMBV. RESULTS: The peak systolic (S) velocity of the lateral tricuspid annulus did not differ between the two groups at baseline. In patients with pulmonary artery hypertension (PAH), it showed a slight increase at 48 hours, but fell behind the baseline at three months. In patients without PAH, it showed a significant increase at 48 hours and remained unchanged at three months. Peak late diastolic (A) velocities were significantly higher at all times in patients without PAH. Patients with PAH had a significantly higher E/A ratio both at baseline and at 48 hours; however, at three months, this difference disappeared. Patients with PAH had higher isovolumic relaxation time (IVRT) at baseline and 48 hours; however, final IVRT was lower than the baseline only in patients with PAH. Isovolumic contraction time showed a steady but insignificant increase in both groups over three months. E-wave deceleration time showed a significant increase and contraction time showed a slight increase over three months only in patients with PAH. The baseline Tei index was higher in patients with PAH (p=0.004). Changes in the Tei index over time were not significant. CONCLUSION: Our findings suggest that, If PMBV is performed before the development of PAH, it may be more effective in the improvement of right ventricular longitudinal functions in patients with rheumatic MS.

[Exercise capacity in patients with metabolic syndrome in the presence of normal coronary arteries]. / Koroner arterleri normal bulunan metabolik sendromlu hastalarda egzersiz kapasitesi.

OBJECTIVES: We evaluated exercise capacity and other exercise parameters in patients with metabolic syndrome and angiographically normal coronary arteries. STUDY DESIGN: Sixty-one patients with angiographically normal coronary arteries were evaluated in two groups according to the presence (n=32; 24 females, 8 males; mean age 59+/-10 years) or absence (n=29; 18 females, 11 males; mean age 59+/-9 years) of metabolic syndrome as proposed by the International Diabetes Federation. All the patients underwent exercise treadmill testing with the modified Bruce protocol, whereby the following variables were determined: workload achieved in metabolic equivalents, total exercise time, percentage of target heart rate achieved, double product, heart rate recovery, chronotropic response and incompetence. The relationships between exercise parameters and echocardiographic and clinical variables were evaluated. RESULTS: The two groups were similar with respect to age, left ventricular dimensions, left ventricular mass index, ejection fraction, and left atrial diameters. The incidence of diastolic dysfunction was significantly higher in patients with metabolic syndrome (71.9% vs 41.4%; p=0.016). The maximum workload achieved was significantly lower (10+/-2 ml/kg/min vs 12+/-2 ml/kg/min; p=0.024) and the initial double product was significantly higher (11.6x10(3) mmHg.pulse/min vs 10.1x10(3) mmHg.pulse/min, p=0.04) in patients with metabolic syndrome. Hypertensive patients exhibited significantly lower maximum workload and total exercise time (p<0.05). Hyperglycemic subjects had significantly lower maximum workload (p<0.05). CONCLUSION: Metabolic syndrome is associated with decreased exercise capacity and each component of this syndrome affects exercise test parameters individually.

Isolated right ventricular rupture: a rare complication of myocardial infarction.

Right ventricular rupture is a rare complication after myocardial infarction and a few cases were reported. We present the case of a 65-year-old Caucasian man with inferior and right ventricular myocardial infarction which was complicated by right ventricular rupture following successful percutaneous revascularization. The transthoracic echocardiography revealed right ventricular free wall rupture with pericardial effusion. The patient underwent a pericardial drainage by pericardiotomy without myocardial repair because of the self-limiting pattern of the right ventricular free wall rupture. The formation of a mural thrombus at the small rupture site limited the leakage and contributed to the outstanding favourable prognosis of the patient with early revascularization even without surgical repair.

Comparison of traditional risk factors, natural history and angiographic findings between coronary heart disease patients with age <40 and >or=40 years old.

OBJECTIVE: In this study we aimed to examine the angiographic findings, traditional risk factors and natural history of Turkish patients <40 and >or=40 years old with coronary heart disease (CHD). METHODS: The records of 491 patients with stable angina pectoris or acute coronary syndrome (ACS), who had undergone coronary angiography (CAG) were reviewed. The patients <40 years (group 1) and >or=40 years (group 2) were compared. RESULTS: The study population was classified as group 1 with 240 patients (mean age 35.7+/-3.4 years) and group 2 with 251 patients (mean age 61.0+/-9.7 years). Smoking, family history, hypercholesterolemia, hypertriglyceridemia and low levels of high-density lipoprotein cholesterol were more prevalent in group 1 while diabetes mellitus, hypertension was higher in group 2. The common presentation among <40 years patients was ACS whereas stable angina was the most common presentation in patients >or=40 years old. Patients in group 1 showed a preponderance of single-vessel disease whereas patients of group 2 showed dominance of multivessel disease. Early clinical course of patients with ACS in group 1 was better than in group 2. CONCLUSION: Our study shows a significantly different clinical, angiographic and biochemical profile in <40 years patients with CHD compared with >or=40 years patients. Dominance of smoking and dyslipidemias that are the preventable risk factors in premature CHD patients is an important threat for our community health. Healthy life styles should be encouraged beginning from young ages and new precautions about smoking must be taken.

Fine-tuning management of the Heart Assist 5 left ventricular assist device with two- and three-dimensional echocardiography.

INTRODUCTION: Left ventricular assist device (LVAD) implantation is a viable therapy for patients with severe end-stage heart failure, providing effective haemodynamic support and improved quality of life. The Heart Assist 5 (Micromed Cardiovascular Inc, Houston, TX) continuous-flow LVAD has been on the market in Europe since May 2009. METHODS: We evaluated nine Heart Assist 5 LVAD patients with two- and three-dimensional transthoracic echocardiographic (TTE) and transoesophageal echocardiographic (TEE) parameters between December 2011 and December 2013. The pre-operative TTE LVAD evaluations included left ventricular (LV) function and structure, quantification of right ventricular (RV) function and tricuspid regurgitation (TR), assessment of aortic and mitral regurgitation, and presence of patent foramen ovale and intra-cardiac clots. Peri-operative TEE determined the inflow cannula and septum position, and assessed the de-airing process while weaning from cardiopulmonary bypass. Post-operative serial follow-up TTE showed the surgical results of LVAD implantation, determined the overall structure and function of the LV, RV and TR, and observed the inflow and outflow cannula position. RESULTS: Nine patients who had undergone Heart Assist 5 LVAD implantation and had been followed up for more than 30 days were included in this study. Eight patients had ischaemic cardiomyopathy and one had adriamycin-induced cardiomyopathy. Pre-implantation data: the mean age of the patients was 52 ± 13 (34-64) years, mean body surface area (BSA) was 1.8 ± 0.2 (1.6-2.0) m2, mean cardiac index (CI) was 2.04 ± 0.4 (1.5-2.6) l/min/m2, mean cardiac output (CO) was 3.7 ± 0.7 (2.6-4.2) l/min, mean ejection fraction (EF) was 23 ± 5 (18-28)%, and right ventricular fractional area contraction (RVFAC) was 43 ± 9 (35-55)%. One patient had aortic valve replacement (AVR) during the LVAD implantation, and excess current alarms and increased power were suspected to be caused by a possible thrombus. Close follow up with TTE studies were carried out to clear the LV of thrombus formation, and the inflow cannula position was checked to maintain the septum in the midline, so preventing the suction cascade. Four patients were followed up for more than two years, and two were followed up for more than a year. Three patients died due to multi-organ failure. Follow-up speed-change TTE studies of six patients showed that the mean speed was 9 800 ± 600 (9 500-10 400) rpm, and mean CO was 4.7 ± 0.3 (4.3-5.0) l/min during the three-month post-implant period. CONCLUSION: We believe that TTE can play a major role in managing LVAD patients to achieve optimal settings for each patient. A large series is mandatory for assessment of echocardiographic studies on Heart Assist 5 LVAD.

[Has the mortality rate from acute myocardial infarction fallen substantially in recent years? Single center data on elderly patient population]. / Akut miyokard infarktüsünden ölüm oranlarinda son yillarda azalma oldu mu? Yasli hasta populasyonunda tek merkez sonuçlari.

OBJECTIVE: We aimed to compare the trend in clinical approach and interventions, in-hospital mortality rate in elderly patients with acute myocardial infarction (AMI) in a single reference center within subsequent years, 2000-2002. METHODS: In our retrospective analysis within years 2000 and 2003 we could reach 160 eligible patients' data files, who were hospitalized for of AMI and aged above 70 years. RESULTS: Within three years we evaluated data of 105 male and 55 female eligible patients (mean age: 74.0+/-3.3 years). In-hospital mortality was observed in 39 (24%) patients with a median admission-to-mortality time of 24 hours. In 33 (20%) of the cases AMI involved more than one myocardial wall. Sixty-one percent of the whole population and 80% of the patients with early in-hospital mortality had reduced left ventricular ejection fraction. The comparison of treatment approaches within three years revealed a growing tendency for application of percutaneous transluminal coronary angioplasty (PTCA), surgical interventions and for the use of beta-blockers, angiotenzin converting enzyme inhibitors and lipid lowering agents (p<0.05). We did not observe any difference in mortality rates at subsequent years. CONCLUSION: In our single center analysis we observed changes in treatment policy in elderly AMI population, which was in concordance with the trends in international arena. But we were not able to show any reduction in mortality rate. Beyond the diverse ethnicity of our patient population, the relative delayed time to hospital admission, more extensive infarct area, lower administration of interventional procedures and primary PTCA, and most importantly the relative short time interval we analyzed may be contributing factors for still high in-hospital mortality in elderly population.

Extrinsic left atrial compression in a patient with achalasia.

Left atrial compression by the esophagus, the stomach, or both is an uncommon but important cause of hemodynamic compromise. Achalasia is a disease of the esophagus with dilatation of the distal part and constriction of the lower oesophageal sphincter. Dilated esophagus is a rare cause of left atrial compression. Timely and precise diagnosis is of paramount importance in cases of left atrial compression and echocardiography enables clinicians to make a differential diagnosis successfully in most cases. Correction of primary pathology will usually lead to the return of normal hemodynamic function. We describe a case of extrinsic left atrial compression caused by the dilated esophagus due to achalasia that caused paroxysms of atrial tachycardia and hemodynamic compromise.

Myocardial integrated ultrasonic backscatter for early detection of cardiac involvement in patients with Wilson disease.

BACKGROUND/AIMS: Videodensitometry is a feasible noninvasive ultrasound tissue characterization method allowing early detection of myocardial changes. This study aimed to investigate ultrasonic backscatter properties of the myocardium in Wilson disease patients. MATERIALS AND METHODS: We compared cardiologically asymptomatic Wilson disease patients (W group) (n=18) with age-matched (26.7±9.6 years) healthy controls (C group) (n=15). Diagnosis of Wilson disease was made on the basis of clinical manifestations, family history, and laboratory findings and confirmed by liver biopsy. Transthoracic echocardiographic quantitative texture analysis was performed on data from the septum and left ventricular posterior wall, and mean gray level (MGL) histograms at end-diastole (d) and end-systole (s) were obtained after background correction (c). Cyclic variation index (CVI) was calculated using the formula [(cMGLd - cMGLs) / cMGLd] ×100. RESULTS: There were no significant differences in sex, age, body mass index, heart rate or blood pressure, and conventional echocardiographic parameters between the 2 groups. The cMGLs value of the posterior wall was higher in the W group than in the C group (30.9±2.6 vs. 22.2±2.7, p=0.033). The W group had a significantly lower CVI of the septum than did the C group (-22±4.4% vs. 43.4 ±12.9%, p<0.001), and there was no significant difference in the CVI of the posterior wall (-67.0±15.9% vs. 41.7±18.6%, p=0.32). CONCLUSION: Abnormalities in two-dimensional echocardiographic grey-level distributions were present in Wilson disease patients. These videodensitometric myocardial alterations were significantly lower in Wilson disease patients than in the controls, and this probably represents an early stage of cardiac involvement.