Serum levels of trace minerals and heavy metals in severe COPD patients with and without pulmonary hypertension.

Aim: The aim of the current study was to assess the serum levels of trace minerals/heavy metals in COPD patients with and without pulmonary hypertension (PH) and to investigate their correlations to demographic, clinical, and biochemical variables. Materials and methods: This cross-sectional study was performed in Van Yuzuncu Yil University Medical Faculty between April 2013 and July 2013. Cases were allocated into three groups: Group 1 consisted of severe COPD patients; Group 2 was made up of COPD patients with PH; and healthy controls constituted Group 3. Demographic, radiological, and biochemical variables, as well as the serum levels of trace minerals and heavy metals, were noted and compared in these three groups. Results: COPD patients were older and had higher rates of smoking habit, diabetes mellitus, and hypertension compared to the control group. Carotid intima-media thickness was increased bilaterally, and serum levels of Co, Cu, and Fe were higher in COPD patients. Left carotid intima-media thickness was increased, and serum levels of Cd, Co, and Fe were found to be higher in COPD cases with PH compared to COPD patients without PH. Conclusion: Our results show that serum levels of trace minerals and heavy metals may be altered in COPD and PH.

The relation of platelet-lymphocyte ratio and coronary collateral circulation in patients with non-ST segment elevation myocardial infarction.

INTRODUCTION: Recently, platelet-to-lymphocyte ratio (PLR) has emerged as a significant inflammatory marker and a novel predictor of major adverse consequences in cardiovascular disease. AIM: In this study, we aimed to explore the relationship between PLR and coronary collateral circulation (CCC) in patients with non-ST elevation myocardial infarction (NSTEMI). MATERIAL AND METHODS: Clinical and laboratory data of 386 patients who underwent coronary angiography were evaluated retrospectively. The patients were classified into 2 groups as follows: poor CCC (group 1: Rentrop grades 0-1) and good CCC (group 2: Rentrop grades 2-3). The PLR was calculated from the complete blood count. RESULTS: The PLR values of the patients with poor CCC were significantly higher than those of patients with good CCC (153.9 ±26.6 vs. 129.8 ±23.5, p < 0.001). In the multiple logistic regression tests, PLR (odds ratio: 1.51, 95% confidence interval: 1.27-1.74; p < 0.001) and hs-CRP (odds ratio: 1.56, 95% CI: 1.03-2.11; p < 0.001) were found to be independent predictors of poor CCC. The receiver operating characteristic (ROC) curve analysis yielded a cutoff value of 140.5 for PLR to predict poor CCC with 79% sensitivity and 71% specificity, with the area under the ROC curve being 0.792 (95% CI: 0.721-0.864). CONCLUSIONS: Our study revealed that high PLR is independently associated with poor coronary collateral circulation in patients with NSTEMI.

The effect of low-sodium dialysate on ambulatory blood pressure measurement parameters in patients undergoing hemodialysis.

BACKGROUND: End stage renal disease is related to increased cardiovascular mortality and morbidity. Hypertension is an important risk factor for cardiovascular disorder among hemodialysis (HD) patients. The aim of this study was to investigate the effect of low-sodium dialysate on the systolic blood pressure (SBP) and diastolic blood pressure (DBP) levels detected by ambulatory BP monitoring (ABPM) and interdialytic weight gain (IDWG) in patients undergoing sustained HD treatment. PATIENTS AND METHODS: The study included 46 patients who had creatinine clearance levels less than 10 mL/min/1.73 m(2) and had been on chronic HD treatment for at least 1 year. After the enrollment stage, the patients were allocated low-sodium dialysate or standard sodium dialysate for 6 months via computer-generated randomization. RESULTS: Twenty-four hour SBP, daytime SBP, nighttime SBP, and nighttime DBP were significantly decreased in the low-sodium dialysate group (P<0.05). No significant reduction was observed in both groups in terms of 24-hour DBP and daytime DBP (P=NS). No difference was found in the standard sodium dialysate group in terms of ABPM. Furthermore, IDWG was found to be significantly decreased in the low-sodium dialysate group after 6 months (P<0.001). CONCLUSION: The study revealed that low-sodium dialysate leads to a decrease in ABPM parameters including 24-hour SBP, daytime SBP, nighttime SBP, and nighttime DBP and it also reduces the number of antihypertensive drugs used and IDWG.

A Novel Echocardiographic Method for Assessing Arterial Stiffness in Obstructive Sleep Apnea Syndrome.

BACKGROUND AND OBJECTIVES: Obstructive sleep apnea syndrome (OSAS) is associated with increased arterial stiffness and cardiovascular complications. The objective of this study was to assess whether the color M-mode-derived propagation velocity of the descending thoracic aorta (aortic velocity propagation, AVP) was an echocardiographic marker for arterial stiffness in OSAS. SUBJECTS AND METHODS: The study population included 116 patients with OSAS and 90 age and gender-matched control subjects. The patients with OSAS were categorized according to their apnea hypopnea index (AHI) as follows: mild to moderate degree (AHI 5-30) and severe degree (AHI≥30). Aortofemoral pulse wave velocity (PWV), carotid intima-media thickness (CIMT), brachial artery flow-mediated dilatation (FMD), and AVP were measured to assess arterial stiffness. RESULTS: AVP and FMD were significantly decreased in patients with OSAS compared to controls (p<0.001). PWV and CIMT were increased in the OSAS group compared to controls (p<0.001). Moreover, AVP and FMD were significantly decreased in the severe OSAS group compared to the mild to moderate OSAS group (p<0.001). PWV and CIMT were significantly increased in the severe group compared to the mild to moderate group (p<0.001). AVP was significantly positively correlated with FMD (r=0.564, p<0.001). However, it was found to be significantly inversely related to PWV (r=-0.580, p<0.001) and CIMT (r=-0.251, p<0.001). CONCLUSION: The measurement of AVP is a novel and practical echocardiographic method, which may be used to identify arterial stiffness in OSAS.

Platelet-to-Lymphocyte Ratio May Predict the Severity of Calcific Aortic Stenosis.

BACKGROUND: Platelet-to-lymphocyte ratio (PLR) is an emerging inflammatory indicator which is closely associated with adverse cardiovascular events. Therefore, we aimed to investigate the relationship between PLR and the severity of calcific aortic stenosis (AS). MATERIAL AND METHODS: The study was designed as a retrospective study. A total of 86 consecutive patients with calcific AS were divided into two groups as mild-to-moderate AS and severe AS according to the transaortic mean pressure gradient. PLR levels were calculated from the complete blood count (CBC). RESULTS: Platelet to lymphocyte ratio was significantly higher in severe and mild-to-moderate AS groups when compared to the control subjects (151±31.2, p<0.001, 138±28.8 vs. 126±26.5, p=0.008, respectively). In the subgroup analysis of AS patients, PLR was found to be higher in the severe AS group compared to mild-to-moderate group (p<0.001). A significant correlation was found between PLR and transaortic mean pressure gradient in patients with AS (r=0.421, p<0.001). CONCLUSIONS: Our study results demonstrated that increased PLR correlates with the severity of calcific AS.

Oxidative stress parameters and their correlation with clinical, metabolic and polysomnographic parameters in severe obstructive sleep apnea syndrome.

The aim of the present study was to assess the levels of oxidative stress markers, catalase (CAT), glutathione peroxidase (GPX) and malondialdehyde (MDA) in severe OSAS and to investigate any correlation between oxidative stress markers and clinical, metabolic and polysomnographic parameters. A total of 30 patients with severe OSAS and 30 healthy controls were included in this cross-sectional, clinical study. Demographic data, polysomnographic, biochemical and clinical indices as well as serum levels of CAT, MDA and GPX were measured and compared in OSAS and control groups. Furthermore, OSAS patients with and without pulmonary hypertension (PHT) were evaluated in terms of levels of CAT, MDA and GPX. Patients with severe OSAS exhibited significantly lower serum levels of CAT (P<0.001) and GPX (P<0.001). Serum MDA levels were remarkably higher in OSAS group (P<0.001). Correlation analysis revealed that levels of CAT and GPX were correlated with apnea-hypopnea index and there was a correlation between serum levels of MDA and CRP. Severe OSAS patients with and without PHT did not reveal any differences for CAT (P=0.789), MDA (P=0.805) and GPX levels (P=0.281). Our results have shown that oxidative stress markers significantly changed in patients with severe OSAS. This information is noteworthy because documentation of the role of oxidative stress in OSAS may have important implications regarding diagnosis, monitoring, treatment and prognosis.

Acute vasoreactivity test results in severe pulmonary hypertension patients with chronic obstructive pulmonary disease: our experience with 29 cases.

AIM: The aim of the current study is to evaluate acute vasoreactivity test (AVT) results in severe pulmonary hypertension patients with chronic obstructive pulmonary disease and to compare the demographical, clinical, and laboratory variables in positive and negative cases. METHODS: This retrospective, clinical study was performed on 29 cases in the departments of cardiology and chest diseases of our tertiary care center. AVT was positive in 12 (41.4%) cases and negative in 17 (58.6%) cases. Demographical variables, cardiopulmonary indicators, and laboratory findings were compared in these two subgroups. RESULTS: The mean age was 62.3±7.8 years for AVT negative group, while it was 64.8±7.3 years in AVT positive group (P=0.38). Except for the changes in systolic, diastolic, and mean pulmonary arterial pressures before and after iloprost administration, there were no statistically significant differences regarding any of the parameters under investigation in both groups. CONCLUSION: Despite the high rate of positivity for AVT in severe pulmonary hypertension patients with chronic obstructive pulmonary disease, none of the variables under investigation displayed a noteworthy difference between AVT negative and positive groups. Identification of factors likely to influence AVT results is important for establishment of appropriate treatment protocols especially for AVT negative cases.

Sleep Apnea in Patients with Rheumatic Mitral Stenosis.

BACKGROUND AND AIM OF THE STUDY: It was hypothesized that sleep disorders might be present due to functional heart failure in patients with mitral stenosis (MS), and might be accompanied by obstructive-type sleep disorders. To examine this hypothesis, non-smoker and non-obese patients with rheumatic MS and without concomitant diseases were investigated for sleep disorders. METHODS: Patients admitted to the cardiology outpatient clinic at the authors' institution for rheumatic MS during a one-year period were included in the study. Patients were evaluated using the Epworth sleepiness scale, apnea-hypopnea index (AHI), and echocardiographic examination. The relationship between echocardiographic findings and AHI was assessed. RESULTS: Eighteen patients were included in the study. All patients had sinus rhythm and normal electrocardiography recordings, with no coronary artery disease or concomitant cardiac disease. Obstructive sleep apnea (OSA) was determined in 16 patients. AHI was positively correlated with the mean apnea duration, mean gradient, rapid eye movement (REM)-AHI and non-REM-AHI. AHI, pulmonary artery pressure, mitral valve area, mean gradient, left ventricular end-systolic diameter, left ventricular end-diastolic diameter and mean REM-AHI differed significantly according to the degree of MS. AHI was shown to be increased as the degree of MS increased. CONCLUSION: The frequency of OSA was found to be significantly higher in patients with MS. In addition to cardiac problems, these patients need to be evaluated also for sleep problems and treated appropriately.

Serum levels of trace minerals and heavy metals in severe obstructive sleep apnea patients: correlates and clinical implications.

PURPOSE: The aim of the present study was to evaluate the serum levels of trace minerals and heavy metals in obstructive sleep apnea (OSA) patients and to investigate whether there is a correlation between levels of trace minerals and demographic and biochemical variables. METHODS: This clinical, case-control study was performed on 61 OSA patients and 36 healthy controls. Demographic, radiological, biochemical variables, as well as serum levels of trace minerals (magnesium, copper, iron, zinc, manganese, cobalt) and heavy metals (lead, cadmium) were compared in OSA and control groups. In addition, correlation of serum levels of these substances to demographic, biochemical, and radiological parameters was tested. RESULTS: Not only serum levels of cholesterol, triglycerides, cadmium, cobalt, copper, iron, magnesium, manganese, lead, and zinc were found to be higher, but also carotid intima-media thickness (CIMT) was increased in OSA patients. Increase in CIMT was found to be correlated with levels of cobalt, copper, iron, magnesium, manganese, and zinc. CONCLUSION: Our results have shown that serum levels of trace minerals and heavy metals were higher in OSA. This difference may ensource from deterioration of the balance of these substances due to oxidative stress and inflammation. Significance of these findings with respect to the etiopathogenesis, diagnosis, and treatment of OSA warrants further trials.

An overlooked cause of resistant hypertension: upper airway resistance syndrome - preliminary results.

OBJECTIVE: Upper airway resistance syndrome is a sleep-disordered breathing syndrome that is characterized by repetitive arousals resulting in sympathetic overactivity. We aimed to determine whether upper airway resistance syndrome was associated with poorly controlled hypertension. METHODS: A total of 40 patients with resistant hypertension were enrolled in the study. All of the patients underwent polysomnographic examinations and 24-hour ambulatory blood pressure monitoring to exclude white coat syndrome and to monitor treatment efficiency. Among 14 upper airway resistance syndrome patients, 2 patients had surgically correctable upper airway pathologies, while 12 patients were given positive airway pressure therapy. RESULTS: All patients underwent polysomnographic examinations; 22 patients (55%) were diagnosed with obstructive sleep apnea and 14 patients (35%) were diagnosed with upper airway resistance syndrome, according to American Sleep Disorders Association criteria. The patients with upper airway resistance syndrome were younger and had a lower body mass index compared with other patients, while there were no difference between the blood pressure levels and the number of antihypertensive drugs. The arousal index was positively correlated with systolic blood pressure level (p=0.034; rs=0.746), while the Epworth score and AHI were independent of disease severity (p=0.435, rs=0.323 and p=0.819, rs=-0.097, respectively). Eight patients were treated with positive airway pressure treatment and blood pressure control was achieved in all of them, whereas no pressure reduction was observed in four untreated patients. CONCLUSIONS: We conclude that upper airway resistance syndrome is a possible secondary cause of resistant hypertension and that its proper treatment could result in dramatic blood pressure control.

An overlooked cause of resistant hypertension: upper airway resistance syndrome - preliminary results

OBJECTIVE: Upper airway resistance syndrome is a sleep-disordered breathing syndrome that is characterized by repetitive arousals resulting in sympathetic overactivity. We aimed to determine whether upper airway resistance syndrome was associated with poorly controlled hypertension. METHODS: A total of 40 patients with resistant hypertension were enrolled in the study. All of the patients underwent polysomnographic examinations and 24-hour ambulatory blood pressure monitoring to exclude white coat syndrome and to monitor treatment efficiency. Among 14 upper airway resistance syndrome patients, 2 patients had surgically correctable upper airway pathologies, while 12 patients were given positive airway pressure therapy. RESULTS: All patients underwent polysomnographic examinations; 22 patients (55%) were diagnosed with obstructive sleep apnea and 14 patients (35%) were diagnosed with upper airway resistance syndrome, according to American Sleep Disorders Association criteria. The patients with upper airway resistance syndrome were younger and had a lower body mass index compared with other patients, while there were no difference between the blood pressure levels and the number of antihypertensive drugs. The arousal index was positively correlated with systolic blood pressure level (p = 0.034; rs = 0.746), while the Epworth score and AHI were independent of disease severity (p = 0.435, rs = 0.323 and p = 0.819, rs = -0.097, respectively). Eight patients were treated with positive airway pressure treatment and blood pressure control was achieved in all of them, whereas no pressure reduction was observed in four untreated patients. CONCLUSIONS: We conclude that upper airway resistance syndrome is a possible secondary cause of resistant hypertension and that its proper treatment could result in dramatic blood pressure control. .

Giant dumbbell-shaped schwannoma but not transforaminal: transdiaphragmatic.

A 31-year-old man reported a 3-month history of right upper abdominal pain and effort dyspnoea. The laboratory data demonstrated no abnormality. On abdominal ultrasound, a gross solid lesion with smooth borders was seen behind the liver. CT scan revealed a huge tumour, 23×17×15 cm in size, it was in the right posterior mediastinum and extended to the right retroperitoneum. In sagittal plane CT images, the lesion was like a dumbbell shape. CT-guided biopsy revealed a spindle cell tumour.

Isovolumic contraction acceleration before and after percutaneous closure of atrial septal defects.

AIM: To compare systemic right ventricular function by isovolumic myocardial acceleration before and 6 months after the percutaneous closure of atrial septal defects (ASD). MATERIAL AND METHODS: Patients admitted to our tertiary center for the percutaneous closure of atrial septal defects between January 2010 and August 2012 constituted the study group. Right ventricular function of patients was assessed by tissue Doppler echocardiography before and after surgery. Echocardiographic data in patients were compared to age-matched controls without any cardiac pathology and studied in identical fashion mentioned below. RESULTS: A total of 44 patients (24 males, 20 females) and 44 age-matched controls (25 males, 19 females) met the eligibility criteria for the study. Right ventricular end-diastolic and end-systolic volume, right ventricular end-diastolic diameter measurements on echocardiogram, and pulmonary artery pressures in both pre- and post-ASD groups were significantly higher than in controls. Tricuspid annular plane systolic excursion and isovolumic myocardial acceleration measurements significantly increased after the percutaneous closure of the defect; however, post-ASD measurements were still significantly lower than the controls. CONCLUSIONS: Atrial septal defect device closure resulted in a significant increase of isovolumic myocardial acceleration measurements. Tissue Doppler analysis of regional myocardial function offers new insight into myocardial compensatory mechanisms for acute and chronic volume overload of both ventricles.

Evaluation of airway wall thickness via high-resolution computed tomography in mild intermittent asthma.

INTRODUCTION: This study aims to evaluate bronchial thickness via thorax high-resolution computed tomography (HRCT) in subjects with mild intermittent asthma in comparison with healthy control subjects. METHODS: A total of 37 out-patients (mean ± SD age = 36.7 years (9.7 years); 54.8% males) with mild intermittent asthma and 13 healthy control subjects (mean ± SD age = 25.0 years (2.9 years); 61.5% males) were included in this case control study. Data on demographics, pulmonary function test results, and segmental and subsegmental thorax HRCT results were recorded. The ratio of bronchial wall thickness to bronchial lumen diameter (T/D) and bronchial wall area percentage (WA%) were calculated for all cases. RESULTS: Subject and control groups were similar in terms of pulmonary function test results, and total and subsegmental T/D values. Mean ± SD subsegmental WA% values at the level of inferior pulmonary vein (55.6% [16.8%] vs. 41.7% [7.4%], P = .047) and 2 cm above the diaphragm (49.8% [15.8%] vs. 38.6% [10.4%], P = .046) were significantly higher in subjects than control subjects. No significant correlation of overall and subsegmental T/D and WA% values to age in both groups, and to asthma duration in subjects. CONCLUSION: Our findings revealed an increase in bronchial wall thickness in peripheral airways in subjects with mild intermittent asthma regardless of the duration of asthma. This may indicate a need to also administer anti-inflammatory or bronchodilator therapy, which is effective in peripheral airways during the early period of the disease.

Relationship between coronary artery disease and pulmonary arterial pressure in patients with chronic obstructive pulmonary disease.

The aim of the present study was to determine whether there is a relationship between coronary artery disease and pulmonary hypertension and whether pulmonary hypertension is an additional risk factor for the presence and extent of coronary artery disease in patients with chronic obstructive pulmonary disease. Patients diagnosed with chronic obstructive pulmonary disease and pulmonary hypertension, and undergone diagnostic coronary angiography for evaluation of suspected coronary artery disease constituted the study group. Patients were divided into two groups according to the presence or absence of coronary artery disease and compared for age, gender, accompanying chronic disease, and pulmonary function tests. A total of 95 patients were recruited in the study. Comparison of the groups revealed that two groups were significantly different on gender (p=0.029), presence of hypertension (p=0.027), and biomass (p=0.040). Correlation analysis of variables revealed that male gender (rs=0.224, p=0.029), hypertension (rs=0.227, p=0.07) were positively correlated with the presence of coronary artery disease. FEV1/FVC ratio (rs=-0.253, p=0.013) and sPAP (rs=-0.215, p=0.037) were negatively correlated with the presence of coronary artery disease. High prevalence of coronary artery disease in patients with pulmonary hypertension secondary to chronic obstructive pulmonary disease was found. However, no correlation between the presence and severity of coronary artery disease and pulmonary hypertension was detected.

Increased QT dispersion and P wave dispersion in major depressive disorder.

BACKGROUND: QT and P wave dispersion parameters can indicate abnormalities in autonomic nervous system and cardiac functioning. OBJECTIVES: To determine QT and P wave dispersion in patients with major depressive disorder compared with healthy volunteers. METHODS: Fifty newly diagnosed patients with major depressive disorder and 50 age- and sex-matched healthy volunteers underwent 12-lead electrocardiography. QT interval, QT dispersion, heart rate-corrected QT dispersion and P wave dispersions were calculated manually by a blinded specialist. RESULTS: Groups were comparable in terms of age, sex, body mass index, smoking status, metabolic diseases and left ventricular ejection fraction. The major depressive disorder group had significantly higher QT dispersion (58.5±9.9 versus 41.7±3.8; P<0.001), heart rate-corrected QT dispersion (62.5±10.0 versus 45.2±4.3; P<0.001) and P wave dispersion (46.9±4.8 versus 41.5±5.1; P<0.001). CONCLUSION: Increased QT dispersion, heart-rate corrected QT dispersion and P wave dispersion in major depressive disorder patients may be indicative of autonomic imbalance and increased risk of cardiac morbidity and mortality.

A Behcet's Disease Patient with Right Ventricular Thrombus, Pulmonary Artery Aneurysms, and Deep Vein Thrombosis Complicating Recurrent Pulmonary Thromboembolism.

Intracardiac thrombus, pulmonary artery aneurysms, deep vein thrombosis, and pulmonary thromboembolism are rarely seen symptoms of Behcet's disease. A 20-year-old female patient was admitted for complaints of cough, fever, palpitations, and chest pain. On the dynamic thorax computed tomograms (CT) obtained because of significantly enlarged hilar structures seen on chest radiograms, aneurysmal dilatation of the pulmonary artery segments bilaterally, chronic thrombus with collapse, and consolidation substances compatible with pulmonary embolism involving both lower lobes have been observed. It is learned that, four years ago, the patient had been diagnosed with Behcet's disease and received colchicine treatment but not regularly. The patient was hospitalized. On the transthoracic echocardiogram, a thrombosis with a dimension of 4.2 × 1.6 cm was recognized in the right ventricle. On abdomen CT, aneurysmal iliac veins and deep vein thrombus on Doppler ultrasonograms were diagnosed. At the controls after three months of immunosuppressive and anticoagulant therapies, some clinical and radiological improvements were recognized. The patient suspended the treatment for a month and the thrombus recurred. We present our case in order to show the effectiveness of immunosuppressive and anticoagulant therapies and rarely seen pulmonary thromboembolism in recurrent Behcet's disease.

Assessment of corneal endothelial cell density in patients with keratoconus not using contact lenses.

PURPOSE: To assess the corneal endothelial cell density (ECD) in keratoconus patients with no history of contact lens use. SETTING: Yuzuncu Yil University, School of Medicine and Van Training and Research Hospital, Department of Ophthalmology, Van, Turkey. DESIGN: Cross-sectional controlled study. METHODS: The eyes of 65 patients with the diagnosis of keratoconus with no history of contact lens wear and the eyes of 40 healthy controls were prospectively examined using the Heidelberg Retinal Tomography Rostock Cornea Module (HRT3/RCM). The average ECD from the two groups were then compared. RESULTS: Of the cases with keratoconus, 44 (67.7%) were men and 21 (32.3%) were women. The mean age was 20.9±6.8 (range=10-41) years. Of the controls, 28 (70%) were men and 12 (30%) were women. The mean age was 23.9±5.8 (range=14-35) years. Of the 65 eyes with keratoconus, 19 (29.2%) had mild keratoconus, 21 (32.3%) had moderate keratoconus, and 25 (38.5%) had severe keratoconus. The mean ECD was 2731.6±303.2 cells/mm2 in cases with keratoconus and 2664.9±319.5 cells/mm2 in controls. There was no difference between the densities (unpaired t-test, P=0.4). No significant relationships were found between the ECD data and central corneal thickness or steepest keratometric. CONCLUSIONS: Endothelial cell density was unaltered in keratoconic patients without a history of contact lens use when compared with healthy controls. Change in ECD is independent from the central corneal thickness and the stage of keratoconus.

Swyer-James-MacLeod syndrome with an anomalous origin of coronary artery: Case report.

Swyer-James (Macleod) syndrome was first defined in the 1950s by Swyer, James and Macleod in patients with unilateral hyperlucent lungs. Coronary artery anomalies are congenital anomalies that affect a small part of the population. They constitute about 1-2% of congenital heart diseases. The incidence of a left coronary artery arising from the right coronary sinus Valsalva has been reported as 0.017%, and 1.3% among coronary artery anomalies. We hereby present this case since the case was diagnosed in adult age and was accompanied by a rare congenital heart disease.