Effects of phosphate binders on bone biomarkers and bone density in haemodialysis patients.

AIM: The incidences of osteoporosis, fracture and vascular calcification increase concordantly with the progression of chronic kidney disease (CKD). CKD-mineral bone disease (CKD-MBD) induced by hyperphosphatemia is a major pathophysiologic mechanism. The effects of phosphate binders on bone turnover biomarkers and bone mineral density (BMD) in haemodialysis patients are still inconclusive. Our aim is to demonstrate the effects of these phosphate binders on different aspects of CKD-MBD. METHODS: We conducted a prospective cohort of 65 haemodialysis patients to investigate the effect of 12-month monotherapy of phosphate binders composing calcium-based phosphate binders (CPB) or non-calcium-based phosphate binders (NCPB), including sevelamer and lanthanum, on bone turnover biomarkers and BMD changes. The performance of bone turnover biomarkers to predict low BMD was attentively determined. RESULTS: When compared with CPB, NCPB use was associated with higher levels of bone turnover biomarkers. NCPB was also associated with lower BMD at lumbar and distal radius. Total procollagen type 1N-terminal propeptide (P1NP), bone-specific alkaline phosphatase (BALP), and tartrate-resistant acid phosphatase 5b (TRAP5b) provided the best performance for diagnosing low BMD in haemodialysis patients. CONCLUSION: Switching from CPB to NCPB might increase bone biomarkers and prevent the development of adynamic bone disease. On the contrary, NCPB should be cautiously used in haemodialysis patients who already had low BMD. P1NP, BALP and TRAP5b could be used to guide the appropriate management, including anti-resorptive and anabolic medications, and predict low BMD in haemodialysis patients treated with phosphate binders.

Incidence and associated factors of deep vein thrombosis in Thai surgical ICU patients without chemoprophylaxis: one year study.

BACKGROUND: Deep vein thrombosis (DVT) is a major problem in the intensive care unit (ICU) patients, especially in Western countries. However; because the incidence of DVT in Asia ICU is lower, chemoprophylaxis (i.e., anticoagulant) is not routinely utilized. The aim of the present study was to identify the incidence and associated factors of DVT in Thai surgical ICU (SICU) patients without chemoprophylaxis. MATERIAL AND METHOD: SICU patients admitted between June 2011 and July 2012 were screenedfor lower extremity DVT using doppler ultrasonography. Stepwise logistic regression was performed to identify associated factors for the development of DVT. RESULTS: Three hundred andfive patients were included in the study, 174 were male (57%) and 131 were female (43%), with ages ranged from 15 to 99 years (mean 62.8 years). Eleven patients had DVT identified (DVT rate 3.6%), two of these had symptomatic pulmonary embolisms. The associated factors for the development of D VT were prior history of venous thromboembolism (p < 0.001, OR 34.3, 95% CI 14.6-80.5), orthopedics group (p < 0.001, OR 27.2, 95% CI 5.2-142.1), and female (p = 0.034, OR 14.3, 95% CI 1.7-102.5). CONCLUSION: The incidence of D VT in Thai SICU patients was 3.6%. Further study is required to identify method and effectiveness of DVT prophylaxis in Asian ICU patients.

Unusual cause of severe transvalvular eccentric jet AR: early structure valve deterioration of Trifecta valves by flail leaflet undergoing valve-in-valve transcatheter aortic valve replacement.

The Trifecta tissue valve (Abbott, Illinois, USA) is an externally mounted bovine pericardial aortic valve (AV) prosthesis with adequate haemodynamic performance and better early results than another option. However, concerns have been raised about its durability. Recently, reports have emerged about an increased incidence of early structural valve failure after Trifecta implantation, where leaflet tear(s) with dehiscence along the stent post was the primary mode of early failure. In this article, we present the case of a patient in her 70s, 7 years after AV replacement with a Trifecta valve, who developed progressive dyspnoea. Physical examination revealed signs of chronic severe aortic regurgitation (AR). The initial transthoracic echocardiogram showed severe transvalvular AR, but the aetiology could not be determined. Cardiac computed tomography (CT) revealed a flail non-coronary cusp of the Trifecta bioprosthetic valve without vegetation. After discussion, we concluded that our patient was suitable for valve-in-valve transcatheter aortic valve replacement (ViV TAVR).

The presence of epsilon waves in all precordial leads (V1 -V6 ) in a 13-year-old boy with arrhythmogenic right ventricular dysplasia (ARVD).

Electrocardiographic feature is included in the diagnostic criteria for arrhythmogenic right ventricular dysplasia (ARVD) based on the Revised Task Force criteria 2010. The epsilon wave, which reflects delayed conduction of the right ventricle, is considered to be one of the major diagnostic criteria. We reported a 13-year-old Thai boy with ARVD who presented with ventricular tachycardia. The presence of epsilon wave in all precordial leads (V1 -V6 ) was observed in standard 12-lead EKG. Extensive scarring of the right and left ventricle was seen on cardiac MRI. The extensive Epsilon wave found in this patient may reflect the extensive ventricular wall involvement.

Double orifice mitral valve: a report of two cases with different severity and treatment.

Background: Double orifice mitral valve (DOMV), a rare congenital heart disease, is characterized by a 2-orifice mitral valve (MV) separated by a tissue bridge, causing a spectacles-like morphology. DOMV can present with various severity ranging from asymptomatic to severe valvular dysfunction including mitral regurgitation (MR) and mitral stenosis (MS), as well as symptoms from coexisting congenital anomalies. Echocardiography is the mainstay of the investigation for a DOMV. We described two cases with DOMV who presented with different disease severity resulting in different treatment decisions. Case Description: In the first case, a 52-year-old woman presented with overt left-sided heart failure. The echocardiogram revealed DOMV with ruptured chordae tendineae of the anterior mitral valve leaflet (AMVL) causing severe MR which led the patient to undergo surgical MV replacement. Intraoperative findings confirmed a diagnosis of DOMV. After surgery, the patient could perform daily activities and light exercises without recurrent heart failure. In the second case, on the other hand, a 36-year-old woman was incidentally diagnosed with DOMV from an echocardiographic workup for symptomatic premature ventricular contraction (PVC). After controlled PVC with radiofrequency ablation, her symptom completely resolved and DOMV was classified as asymptomatic which led to the decision of a watchful waiting strategy. Conclusions: These cases highlight the diversity of DOMV manifestations and the importance of appropriate investigations, particularly echocardiography, to evaluate valvular pathology and contemplating the treatment strategy.

Reference values of myocardial native T1 and extracellular volume in patients without structural heart disease and had negative 3T cardiac magnetic resonance adenosine stress test.

Background: To establish the reference values of native T1 and extracellular volume (ECV) in patients without structural heart disease and had a negative adenosine stress 3T cardiac magnetic resonance. Methods: Short-axis T1 mapping images were acquired using a modified Look-Locker inversion recovery technique before and after administration of 0.15 mmol/kg gadobutrol to calculate both native T1 and ECV. To compare the agreement between measurement strategies, regions of interest (ROI) were drawn in all 16 segments then averaged to represent mean global native T1. Additionally, an ROI was drawn in the mid-ventricular septum on the same image to represent the mid-ventricular septal native T1. Results: Fifty-one patients (mean 65 years, 65 % women) were included. Mean global native T1 averaged from all 16 segments and a mid-ventricular septal native T1 were not significantly different (1221.2 ± 35.2 vs 1228.4 ± 43.7 ms, p = 0.21). Men had lower mean global native T1 (1195 ± 29.8 vs 1235.5 ± 29.4 ms, p < 0.001) than women. Both mean global and mid-ventricular septal native T1 were not correlated with age (r = 0.21, p = 0.13 and r = 0.18, p = 0.19, respectively). The calculated ECV was 26.6 ± 2.7 %, which was not influenced by either gender or age. Conclusions: We report the first study to validate the native T1 and ECV reference ranges, factors influencing T1, and the validation across measurement methods in older Asian patients without structural heart disease and had a negative adenosine stress test. These references allow for better detection of abnormal myocardial tissue characteristics in clinical practice.

An unusual cause of high-output heart failure from the iliac arteriovenous fistula after lumbar discectomy: A case report.

A minority of patients with heart failure present in a high-output state. We described an uncommon case of high-output heart failure caused by an iliac arteriovenous fistula (IAVF), a rare but serious complication after lumbar discectomy surgery (LDS). A 44-year-old man with no notable medical condition except a history of herniated nucleus pulposus necessitating the L4-L5 LDS 5 years ago presented with clinical signs of progressive high-output heart failure. Physical examination revealed wide pulse pressure with bruit and systolic thrill at the right inguinal region. Computed tomographic angiography confirmed the IAVF from the right common iliac artery to the left common iliac vein. There was a significant shunting to the venous system, causing severe dilatation of the inferior vena cava. Notably, the preoperative lumbar magnetic resonance imaging performed 5 years ago demonstrated that the herniated disc was located at the L4-L5 level, which corresponded to the location of IAVF. The patient successfully underwent endovascular closure by covered stent leading to the gradual resolution of symptoms and hemodynamic parameters. Although vascular complications from the LDS are very uncommon, most patients develop severe symptoms from worsening high-output heart failure. This case highlights the essence of careful history taking, physical examinations, and appropriate investigations in guiding the diagnosis and contemplating the treatment strategy.

High-Sensitivity Troponins and Subclinical Coronary Atherosclerosis Evaluated by Coronary Calcium Score Among Older Asians Living With Well-Controlled Human Immunodeficiency Virus.

Background: Elevated levels of high-sensitivity cardiac troponin (hs-cTn) are suggestive of myocardial cell injury and coronary artery disease. We explored the association between hs-cTn and subclinical arteriosclerosis using coronary artery calcification (CAC) scoring among 337 virally suppressed patients with human immunodeficiency virus (HIV) who were ≥50 years old and without evidence of known coronary artery disease. Methods: Noncontrast cardiac computed tomography and blood sampling for hs-cTn, both subunit I (hs-cTnI) and subunit T (hs-cTnT), were performed. The relationship between CAC (Agatston score) and serum hs-cTn levels was analyzed using Spearman correlation and logistic regression models. Results: The patients, of whom 62% were male, had a median age of 54 years and had been on antiretroviral therapy for a median of 16 years; the CAC score was >0 in 50% of patients and ≥100 in 16%. Both hs-cTn concentrations were positively correlated with the Agatston score, with correlation coefficients of 0.28 and 0.27 (P < .001) for hs-cTnI and hs-cTnT, respectively. hs-cTnI and hs-cTnT concentrations of ≥4 and ≥5.3 pg/mL, respectively, provided the best performance for discriminating patients with Agatston scores ≥100, with a sensitivity and specificity of 76% and 60%, respectively, for hs-cTnI and 70% and 50% for hs-cTnT. In multivariable logistic regression analysis, each log unit increase in hs-cTnI level was independently associated with increased odds of having an Agatston score ≥100 (odds ratio, 2.83 [95% confidence interval, 1.69-4.75]; P <.001). Although not an independent predictor, hs-cTnT was also associated with an increased odds of having an Agatston score ≥100 (odds ratio, 1.58 [95% confidence interval, .92-2.73]; P = .10). Conclusions: Among Asians aged ≥50 years with well-controlled HIV infection and without established cardiovascular disease, 50% had subclinical arteriosclerosis. Increasing hs-cTnI and hs-cTnT concentrations were associated with an increased risk of severe subclinical arteriosclerosis, and hs-cTn may be a potential biomarker to detect severe subclinical arteriosclerosis.

The discrepancies between clinical and histopathological diagnoses of cardiomyopathies in patients with stage D heart failure undergoing heart transplantation.

BACKGROUND: This study aimed to determine the etiology of stage-D heart failure (HF) and the prevalence and prognosis of misdiagnosed cardiomyopathy in patients undergoing heart transplantation. METHODS AND RESULTS: We retrospectively reviewed 127 consecutive patients (mean age, 42 years; 90 [71%], male) from February 1994 to September 2021 admitted for heart transplant in our tertiary center. Pre-transplant clinical diagnosis was compared with post-transplant pathological diagnosis. The most common misdiagnosed cardiomyopathy was nonischemic cardiomyopathy accounting for 6% (n = 8) of all patients. Histopathological examination of explanted hearts in misdiagnosed patients revealed 2 arrhythmogenic cardiomyopathy, 2 sarcoidosis, 1 hypertrophic cardiomyopathy, 1 hypersensitivity myocarditis, 1 noncompacted cardiomyopathy, and 1 ischemic cardiomyopathy. Pre-transplant cardiac MRI and endomyocardial biopsy (EMB) were performed in 33 (26%) and 6 (5%) patients, respectively, with both performed in 3 (3% of patients). None of the patients undergoing both cardiac tests were misdiagnosed. During the 5-years follow-up period, 2 (25%) and 44 (37%) patients with and without pretransplant misdiagnosed cardiomyopathy died. There was no difference in survival rate between the groups (hazard ratio: 0.52; 95% CI:0.11-2.93; P = 0.314). CONCLUSIONS: The prevalence of misdiagnosed cardiomyopathy was 6% of patients with stage-D HF undergoing heart transplantation, the misdiagnosis mostly occurred in nonischemic/dilated cardiomyopathy. An accurate diagnosis of newly detected cardiomyopathy gives an opportunity for potentially reversing cardiomyopathy, including sarcoidosis or myocarditis. This strategy may minimize the need for advanced HF therapy or heart transplantation. With advances in cardiac imaging, improvements in diagnostic accuracy of the etiology of HF can improve targeting of treatment.

Concomitant myocarditis and painless thyroiditis after AstraZeneca coronavirus disease 2019 vaccination: a case report.

BACKGROUND: Incidence of myocarditis following messenger RNA coronavirus disease 2019 vaccination has been widely described, but this clinical scenario after adenoviral vector coronavirus disease 2019 vaccination has only been rarely reported. In addition, a few case reports of thyroiditis after adenoviral vector coronavirus disease 2019 vaccination have been published. CASE PRESENTATION: A 55-year-old Thai woman presented with palpitation without neck pain 14 days after receiving AstraZeneca coronavirus disease 2019 vaccination. Electrocardiography revealed sinus tachycardia. Her blood tests showed elevation of cardiac troponin and free triiodothyronine with suppressed serum thyroid stimulating hormone, reflecting a hyperthyroid status. Evidence of myocardial inflammation and necrosis from cardiac magnetic resonance imaging supported the diagnosis of recent myocarditis. Laboratory results and imaging findings were consistent with thyroiditis. After 3 weeks of symptomatic treatment, her symptom and blood tests had returned to normal. CONCLUSIONS: This case demonstrates that the adenoviral vector coronavirus disease 2019 vaccine could possibly cause myocarditis and painless thyroiditis. Clinicians should have a high index of suspicion and promptly evaluate these conditions, despite minimal symptoms.

Higher epicardial fat in older adults living with HIV with viral suppression and relationship with liver steatosis, coronary calcium and cardiometabolic risks.

OBJECTIVES: HIV infection is associated with ectopic fat deposition, which leads to chronic inflammation and cardiometabolic dysregulation. We assessed the epicardial adipose tissue (EAT) volume and its associated factors among people with HIV (PWH). DESIGN: A cross-sectional study. METHODS: We conducted a cross-sectional study among PWH aged at least 50 years and age-matched and sex-matched HIV-negative older individuals in Bangkok, Thailand. Participants underwent a noncontrast, cardiac computed tomography (CT) scan to assess coronary artery calcium (CAC) score and EAT between March 2016 and June 2017. Multivariate linear regression analyses were used to investigate HIV-related factors, cardiac and metabolic markers associated with EAT volume. RESULTS: Median age was 55 years [interquartile range (IQR) 52-60] and 63% were men. Median duration of antiretroviral therapy (ART) was 16 years with 97% had HIV-1 RNA less than 50 copies/ml and median CD4 + cell count of 617 cells/µl. Median EAT volume was significantly higher in PWH [99 (IQR 75-122) cm 3 ] than HIV-negative individuals [93 (IQR 69-117) cm 3 ], P  = 0.022. In adjusted model, factors associated with EAT volume included male sex ( P  = 0.045), older age ( P  < 0.001), abnormal waist circumference ( P  < 0.001) and HOMA-IR ( P  = 0.01). In addition, higher CAC score was independently associated with EAT volume. Higher mean EAT volume was seen in PWH with severe liver steatosis than those without steatosis ( P  = 0.018). In adjusted PWH-only model, duration of HIV was significantly associated with higher EAT volume ( P  = 0.028). CONCLUSION: In an aging cohort, PWH had higher EAT volume than HIV-negative controls. EAT was also independently associated with central fat accumulation, insulin resistance, liver steatosis and CAC score.

Prediction of nonviable myocardium by ECG Q-Wave parameters: A 3.0 T cardiovascular magnetic resonance study.

INTRODUCTION: The presence of a Q-wave on a 12-lead electrocardiogram (ECG) has been considered a marker of a large myocardial infarction (MI). However, the correlation between the presence of Q-waves and nonviable myocardium is still controversial. The aims of this study were to 1) test QWA, a novel ECG approach, to predict transmural extent and scar volume using a 3.0 Tesla scanner, and 2) assess the accuracy of QWA and transmural extent. METHODS: Consecutive patients with a history of coronary artery disease who came for myocardial viability assessment by CMR were retrospectively enrolled. Q-wave measurements parameters including duration and maximal amplitude were performed from each surface lead. A 3.0 Tesla CMR was performed to assess LGE and viability. RESULTS: Total of 248 patients were enrolled in the study (with presence (n = 76) and absence of pathologic Q-wave (n = 172)). Overall prevalence of pathologic Q-waves was 27.2% (for LAD infarction patients), 20.0 % (for LCX infarction patients), and 16.8% (for RCA infarction patients). Q-wave area demonstrated high performance for predicting the presence of a nonviable segment in LAD territory (AUC 0.85, 0.77-0.92) and a lower, but still significant performance in LCX (0.63, 0.51-0.74) and RCA territory (0.66, 0.55-0.77). Q-wave area greater than 6 ms mV demonstrated high performance in predicting the presence of myocardium scar larger than 10% (AUC 0.82, 0.76-0.89). CONCLUSION: Q-wave area, a novel Q-wave parameter, can predict non-viable myocardial territories and the presence of a significant myocardial scar extension.

Intra-coronary bone marrow mononuclear cell transplantation in patients with ST-elevation myocardial infarction: a randomized controlled study.

BACKGROUND: Stem cell transplantation is a potential treatment to improve left ventricular ejection fraction (LVEF) after ST elevation myocardial infarction (STEMI). However, the outcomes still are controversial. OBJECTIVE: To determine the 6-month LVEF of the patients who underwent intra-coronary bone marrow mononuclear cell (BMC) transplantation in patients with STEMI compared with controlled subjects. MATERIAL AND METHOD: After successful percutaneous coronary intervention (PCI) in STEMI patients who had LVEF was less than 50% were randomized to intra-coronary BMC transplantation or control. Bone marrow aspiration of 100 cc was performed in the morning. After cellprocessing for three hours, the suspension of BMC about 10 cc were infused to infracted area using standard PCI technique. Balloon occlusion for three minutes was performed during cell infusion. Cardiac magnetic resonance imaging was used to determine LVEF scar volume and LV volume before and six-month follow-up. RESULTS: Between September 2006 and July 2008, 23patients (11 in BMC group and 12 in control group) were enrolled. Mean BMC count before transplant was 420 x 10(6) cell with 96% viability. At six-month follow-up, New York Heart Association function class significantly improved in both groups (2.3 +/- 0.6 to 1.2 +/- 0. 4 for BMC and 2.3 +/- 0.7 to 1.3 +/- 0.5 for control group) but no difference was seen between groups. However, scar volume, wall motion score index, and LVEF did not show improvement after six months in both groups (33.7 +/- 7.7 to 33.5 +/- 7.6 for BMC and 31.1 +/- 7.1 to 32.6 +/- 8.3 for control group). No complication was observed during the procedure. CONCLUSION: BMC transplantation intra-coronary in patients with STEMI in KCMH was feasible and safe but LVEF improvement could not be demonstrated.

Left ventricular infarct size assessed with 0.1 mmol/kg of gadobenate dimeglumine correlates with that assessed with 0.2 mmol/kg of gadopentetate dimeglumine.

OBJECTIVE: To determine myocardial infarct (MI) size during cardiovascular magnetic resonance at 1.5 Tesla using 0.1 mmol/kg body weight of gadobenate dimeglumine (Gd-BOPTA) and 0.2 mmol/kg body weight of gadopentetate dimeglumine (Gd-DTPA). METHODS: Twenty participants (16 men, 4 women), aged 58 +/- 12 years, with a prior chronic MI were imaged in a crossover design. Participants received 0.2 mmol/kg body weight of Gd-DTPA and 0.1 mmol/kg body weight of Gd-BOPTA on 2 occasions separated by 3 to 7 days. RESULTS: The correlations were high between Gd-DTPA and Gd-BOPTA measures of infarct volume (r = 0.93) and the percentage of infarct relative to left ventricular myocardial volume (r = 0.85). The size and location of the infarcts were similar (P = 0.9) for the 2 contrast agents. Interobserver correlation of infarct volume (r = 0.91) was high. CONCLUSIONS: In chronic MI, late gadolinium enhancement identified with a single 0.1 mmol/kg body weight dose of Gd-BOPTA is associated in volume and location to a double (0.2 mmol/kg body weight) dose of Gd-DTPA. Lower doses of higher relaxivity contrast agents should be considered for determining left ventricular myocardial infarct size.

Feasibility and safety of intra-coronary bone marrow mononuclear cell transplantation in ST elevation myocardial infarction patients.

BACKGROUND: Stem cell transplantation is a potential treatment to improve left ventricular ejection fraction (LVEF) after ST elevation myocardial infarction (STEMI). However technique and mode of transplantation, type of cells, number of cells, and when to transplant are still unknown. OBJECTIVE: To determine the feasibility and safety of bone marrow mononuclear cell (BMC) intra-coronary transplantation and 6-months results in patients with STEMI. MATERIAL AND METHOD: After successful percutaneous coronary intervention (PCI) in STEMI patients who did not have flow re-established within 12 hours and poor LVEF (less than 50%) by echocardiography were enrolled Bone marrow aspiration of 100 cc was performed in the morning. After cell processing for 3 hours, the suspension of BMC about 10 cc were infused to infarcted area using standard PCI technique. Balloon occlusion for 3 minutes was performed during cell infusion. Cardiac magnetic resonance imaging was used to determine LVEF scar volume and LV volume before and 6 months after transplantation. RESULTS: Five patients were enrolled between May and August 2006. Duration of STEMI before transplantation ranged from 18 days to 14 years. Total amount of BMC ranged from 67 x 10(6) to 335 x 10(6). Number of CD 34 and CD 133+ cells were approximation to be 0.7 x 10(6) to 7.7 x 10(6) and 0.01 x 10(6) to 3.04 x 10(6). LVEF was increased from 36.4 at baseline to 43.3 at 6-month. NT pro-BNP level was decreased from 1105 ng/ml at baseline to 288 pg/ml at 6-month. No complications such as chest pain, no re-flow phenomenon, ventricular arrhythmia, or hypotension was detected during the procedure. CONCLUSION: Intra-coronary BMC transplantation in patients with STEMI in our center is feasible and safe. LVEF was slightly improved; however, a randomized controlled study is needed.

Effect of Sodium Thiosulfate on Arterial Stiffness in End-Stage Renal Disease Patients Undergoing Chronic Hemodialysis (Sodium Thiosulfate-Hemodialysis Study): A Randomized Controlled Trial.

BACKGROUND: Arterial stiffness (AS) and vascular calcification are significantly related to a high cardiovascular mortality risk in hemodialysis (HD) patients. Intravenous sodium thiosulfate (IV STS) can prevent and delay the vascular calcification progression in uremic states; however, the STS effect on AS has not been assessed. This study aimed to evaluate the STS efficacy on vascular calcification and AS in HD patients. METHODS: Fifty HD patients with abnormal AS, as measured via the cardio-ankle vascular index (CAVI ≥8), were prospectively randomized to open-label 12.5 g IV STS during the last HD hour twice weekly for 6 months (n = 24) or the usual care (control group; n = 26). Patients and treating physicians were not blinded. The CAVI, coronary artery calcification (CAC) score, hemodynamics, and biochemical parameters were measured at the baseline and at 3 and 6 months. RESULTS: All the baseline parameters were comparable. The IV STS significantly reduced the CAVI when compared to the control group (mean CAVI difference = -0.53; 95% CI -1.00 to -0.06; p = 0.03). A significant CAVI improvement was seen in those patients without diabetes mellitus. The natural logarithm of the CAC volume score was significantly increased in the control group. The high sensitivity C-reactive protein level was slightly lowered in the IV STS group (not significant). CONCLUSION: The intradialytic STS treatment significantly reduced the AS, as measured by the CAVI, and stabilized the vascular calcification in the HD patients. STS may be a novel therapeutic strategy for delaying and treating the structural and functional vascular wall abnormalities in HD patients.

Very late presentation of anomalous origin of the left coronary artery from the pulmonary artery: case report.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. The enlarged right coronary artery provides retrograde collaterals to supply the left ventricle then preferentially directs into the lower pressure pulmonary artery system causing coronary steal phenomenon. Few patients who survive through adulthood without surgery must have abundant, well-formed functioning collaterals with adequate perfusion of the left ventricle. We present the oldest reported patient with ALCAPA to undergo corrective surgery. CASE PRESENTATION: A 79-year-old woman presented with a 3-months history of worsening shortness of breath and orthopnea. Physical examination discovered a soft continuous murmur at the left upper chest. Transthoracic echocardiography demonstrated an unusual, tubular-like structure inside the interventricular septum with a turbulent flow from color Doppler. Moreover, there was a severe mitral regurgitation from posterior mitral leaflet restriction associated with ventricular remodeling in combination with mitral annular dilatation. Coronary angiography and coronary computed tomography angiography established the diagnostic hallmark of ALCAPA syndrome. Stress cardiovascular magnetic resonance perfusion imaging demonstrated no myocardial ischemia suggesting adequate collateral circulation. Remarkably, there was a left coronary ostial stenosis, which served as a protective mechanism against myocardia ischemia by limiting the steal effect. The patient successfully underwent the ligation of anomalous artery at its origin in combination with bioprosthetic mitral valve replacement. Her postoperative course was uneventful. CONCLUSIONS: This case utilized multimodality imaging for delineating the course of abnormal vessels and helping to formulate therapeutic decision.