Repair of a stenotic bicuspid aortic valve by extensive leaflet shaving and commissuroplasty.

The surgical repair of the bicuspid aortic valve in children has been shown to have good outcomes. The repair can be done by the delicate use of native valve tissue without using patch material. The patient is an 8-year-old boy diagnosed with a stenotic bicuspid aortic valve and supravalvular aortic stenosis. He had a balloon valvuloplasty when he was 6 years old. This video tutorial gives detailed information on how to repair the aortic valve by reshaping the aortic cusps and reconstructing the aortic commissures.

Early outcomes of usage of cryoFORM® probe for cryomaze procedure in congenital heart surgery.

OBJECTIVES: This study aimed to evaluate the early outcomes of patients who underwent a concomitant therapeutic maze procedure for congenital heart surgery. MATERIALS AND METHODS: Between 2019 and 2020, eight patients underwent surgical cryoablation by using the same type of cryoablation probe. RESULTS: Three patients had atrial flutter, two had Wolf-Parkinson-White syndrome, two intra-atrial reentrant tachycardia, and one had atrial fibrillation. Four patients underwent electrophysiological study. Preoperatively, one patient was on 3, two were on 2, five were on 1 antiarrhythmic drug. Six patients underwent right atrial maze and two underwent bilateral atrial maze. Five out of six right atrial maze patients underwent right atrial reduction. Nine different lesion sets were used. Some of the lesions were combined and applied as one lesion. In Ebstein's anomaly patients, the lesion from coronary sinus to displaced tricuspid annulus was delicately performed. The single ventricle patient with heterotaxy had junctional rhythm at the time of discharge and was the only patient who experienced atrial extrasystoles 2 months after discharge. Seven of the eight patients were on sinus rhythm. No patient needed permanent pacemaker placement. CONCLUSION: Cryomaze procedure can be applied in congenital heart diseases with acceptable arrhythmia-free rates by selecting the appropriate materials and suitable lesion sets. The application of cryomaze in heterotaxy patients can be challenging due to differences in the conduction system and complex anatomy. Consensus with the electrophysiology team about the choice of the right-left or biatrial maze procedure is mandatory for operational success.

Early Infant Symptomatic Patients With Tetralogy of Fallot With Absent Pulmonary Valve: Pulmonary Artery Management and Airway Stabilization.

BACKGROUND: Tracheobronchomalacia and airway obstruction from severely dilated pulmonary arteries in tetralogy of Fallot with absent pulmonary valve (TOF-APV) has been associated with high rates of respiratory failure and mortality (15% to 25%). It is not known whether aggressive pulmonary artery (PA) or direct airway intervention during early definitive cardiac repair improves outcomes. METHODS: A retrospective observational study was made of all patients undergoing surgical repair for TOF-APV at our center between 2006 and 2018. RESULTS: Twenty patients underwent repair at a median age of 51 days and PA Z-scores of 8.1. Twelve patients had a valve implanted, 6 of whom required reoperation for valve replacement at a median of 9 months (range, 3 to 28) compared with 8 who had initial transannular patch, and only 1 patient required subsequent valve replacement (P < .05). Seven patients had central PAs replaced with thin-walled Gore-Tex (WL Gore, Flagstaff, AZ) grafts; none of these required PA reoperation during a median follow-up of 26.5 months, whereas 3 of 13 patients who did not have PA replacement with Gore-Tex required subsequent PA reoperation (P < .05). Concomitant airway interventions (eg, tracheobronchopexy/plasty) were performed in 4 patients and none required subsequent airway interventions, whereas 2 patients not having initial airway intervention required subsequent tracheopexy (P < .05). Three patients in the cohort eventually required tracheostomy (15%), and 2 patients died (10%; on postoperative days 30 and 326); none had received initial airway intervention. CONCLUSIONS: Pulmonary artery replacement and aggressive direct airway management at initial definitive repair of cardiac TOF-APV can be performed safely with acceptable survival outcomes and low rates of airway and PA reintervention.

Pentraxin 3 in primary percutaneous coronary intervention for ST elevation myocardial infarction is associated with early irreversible myocardial damage : Kinetic profile, relationship to interleukin 6 and infarct size.

BACKGROUND: The inflammatory marker long pentraxin 3 (PTX3) has been shown to be a strong predictor of 30-day and one-year mortality after acute myocardial infarction. The aim of this study was to evaluate the kinetic profile of PTX3 and its relationship with interleukin 6 (IL-6), high-sensitive C-reactive protein (hs-CRP) and infarct size. METHODS: PTX3, IL-6 and hs-CRP were measured at predefined time points, at baseline (before percutaneous coronary intervention (PCI)), at 12 and 72 hours after PCI in 161 patients with first-time ST elevation myocardial infarction (STEMI). RESULTS: PTX3 and IL-6 levels increased in the early phase, followed by a gradual decrease between 12 and 72 hours. There were statistically significant correlations between PTX3 and IL-6 in general, for all time points and for changes over time (0-72 hours). In a linear mixed model, PTX3 predicted IL-6 (p < 0.001). PTX3 is also correlated with hs-CRP in general, and at each time point post PCI, except at baseline. PTX3, IL-6 and hs-CRP were all significantly correlated with infarct size in general, and at the peak time point for maximum troponin I. In addition, there was a modest correlation between IL-6 levels at baseline and infarct size at 72 hours after PCI (ρ = 0.23, p = 0.006). CONCLUSIONS: PTX3 had a similar kinetic profile to IL-6, with an early increase and decline, and was statistically significantly correlated with markers of infarct size in STEMI patients post primary PCI. Baseline levels of IL-6 only predicted infarct size at 72 hours post PCI.

Comparison of trace element levels after cardiopulmonary bypass between cyanotic and acyanotic patients.

Trace elements are essential micronutrients for the human body. In this study, we evaluated the alterations in copper, chromium, manganese, selenium, magnesium, zinc, iron, arsenic, boron, and silicon levels in children with cyanotic and acyanotic CHD who underwent cardiac surgery with cardiopulmonary bypass. Participants were divided into the following three groups: patients acyanotic CHDs (n=34), patients with cyanotic CHDs (n=30), and healthy controls (n=30). Blood samples were collected before the surgery and 1 hour after the sternum was closed. Serum trace elements were determined by Inductively Coupled Plasma Optical Emission Spectrometer-ICAP 6000. The baseline serum arsenic, manganese, and zinc levels of both patient groups were lower compared with controls, but there was no significant difference between baseline serum trace element levels of cyanotic and acyanotic patients. In both the patient groups, there was a significant decrease in postoperative serum arsenic, boron, copper, and zinc levels, and a significant increase in postoperative serum iron and magnesium levels. Silicon levels increased in cyanotic patients. Alterations in trace element levels were in the same direction in cyanotic and acyanotic patients. Copper, zinc, and manganase replacement may be needed after on-pump cardiac surgery.

Alterations in antioxidant and oxidant status of children after on-pump surgery for cyanotic and acyanotic congenital heart diseases.

OBJECTIVE: Oxidative stress refers to an imbalance between reactive oxidative species and antioxidants. In this case-controlled, prospective, observational study, we investigated the total oxidant status, total antioxidant status, oxidative stress index, and albumin and C-reactive protein levels of children with cyanotic and acyanotic congenital heart diseases who had undergone on-pump cardiac surgery. METHOD: The study groups consisted of 60 patients with congenital heart disease, who were operated under cardiopulmonary bypass, and a control group of 30 healthy individuals. The patients were classified into two groups. Among them, one was a patient group that consisted of 30 patients with acyanotic congenital heart disease and the other group consisted of 30 patients with cyanotic congenital heart disease. In the patient groups, blood samples were collected before surgery and at one and 24 hours following surgery. In control groups, blood samples were collected once during hospital admission. RESULTS: No statistically significant differences were found between the groups in terms of baseline total oxidant status, total antioxidant status, and oxidative stress index values. Regarding the postoperative first-hour and 24-hour total oxidant status and total antioxidant status levels as well as oxidative stress index values, there were no significant differences between the groups, except for an increase in total antioxidant status levels (p=0.002) 24 hours after surgery in cyanotic patients. CONCLUSION: There was no difference between oxidative stress status of cyanotic and acyanotic congenital heart disease patients and healthy individuals. Oxidative stress status of cyanotic and acyanotic patients does not change after cardiac surgery under cardiopulmonary bypass.

Impact of Coronary Artery Anatomy in Arterial Switch Procedure on Early Mortality and Morbidity.

OBJECTIVE: Arterial switch operation has become the treatment of choice for neonates with transposition of the great arteries. The most important step of the procedure is transferring the coronary arteries to the neoaorta successfully. This study shows the impact of coronary anatomy on early mortality and morbidity after arterial switch operation. METHODS: Ninety-two patients with transposition of the great arteries who underwent arterial switch operation between October 2010 and September 2014 were included in this retrospective study. The patients were classified into two groups: group I (n = 68, patients with usual coronary artery anatomy) and group II (n = 24, patients with unusual coronary artery anatomy). Median age was 10 days (6-25 days) in group I and 14 days (7-29 days) in group II. In group I, 25 patients had ventricular septal defect, nine patients had coarctation of the aorta or distal aortic arch hypoplasia, seven patients had Taussig Bing anomaly. In group II, nine patients had ventricular septal defect, one patient had coarctation of aorta, and one patient had Taussig Bing anomaly. RESULTS: Regarding the postoperative variables, no significant statistical difference was found between two groups. But cardiopulmonary bypass time is significantly longer in group II (P = .004). There was no difference in complications including the mortality (P = .265). It is statistically found that associated anomalies did not affect the mortality and complication rates. All mortality cases (n = 4) were related to ventricular dysfunction in group II, whereas only four of the eight patients died because of ventricular dysfunction in group I. CONCLUSION: Coronary artery pattern was not a predictor of mortality in early postoperative period. There may be an impact of unusual coronary artery pattern on the development of ventricular dysfunction as a cause of mortality.

Neutrophil-to-lymphocyte ratio in obstructive sleep apnea; a multi center, retrospective study.

OBJECTIVE: Systemic inflammation is important in pathophysiology of obstructive sleep apnea (OSA) and its comorbidity. Neutrophil to lymphocyte ratio (N/L ratio) is a novel inflammation index that has been shown to independently predict poor clinical outcomes. In this study, we aimed to evaluate the role of N/L ratio in OSA patients and comparing with other well-known inflammatory marker, C-reactive protein (CRP). PATIENTS AND METHODS: We conducted a retrospective analysis of 481 patients with mild, moderate and severe OSA (163,158 and 160 patients, respectively) and leukocyte profiles of 80 sex-, age- and body mass index- matched healthy controls. Patients were excluded if they had underlying cancer, chronic inflammatory disease, any systemic infection, uncontrolled hypertension and diabetes mellitus, a known acute coronary syndrome, valvular heart disease, a known thyroid, renal or hepatic dysfunction. RESULTS: We found that N/L Ratio in severe OSA patients was significantly higher compared with mild, moderate, OSA patients and healthy controls (p < 0.001). However, there was no difference between mild and moderate OSA patients (p = 0.636). There was also no significant difference between mild-moderate OSA patients and healthy groups (p = 0.150). CRP levels were not different in all OSA stages (p = 0.595). By Spearman correlation, there was no correlation between CRP and N/L ratio. CONCLUSIONS: N/L ratio, which is quick, cheap, easily measurable novel inflammatory marker with routine complete blood count analysis, is a surrogate marker of obstructive sleep apnea severity.

Left coronary artery stenosis causing left ventricular dysfunction in two children with supravalvular aortic stenosis.

Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome (WBS) and other isolated elastin gene deletions. Cardiovascular manifestations associated with WBS are characterized by obstructive arterial lesions such as SVAS and pulmonary artery stenosis in addition to bicuspid aortic valve and mitral valve prolapse. However, coronary artery ostial stenosis may be associated with SVAS, and it increases the risk of sudden death and may complicate surgical management. In this report, we present our experience with two patients having SVAS and left coronary artery ostial stenosis with associated left ventricular dysfunction.

Complete atrioventricular septal defect and pulmonary stenosis diagnosed in a 49-year-old woman after 10 uneventful births.

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature.

Endovenous laser ablation for saphenous vein insufficiency: short- and mid-term results of 230 procedures.

OBJECTIVE: The present study aimed to evaluate the efficacy of endovenous laser ablation with a 1470-nm laser and to analyze the short- to mid-term results of endovenous laser ablation procedures to treat great saphenous vein insufficiency. METHOD: In this retrospective study, 200 patients (230 limbs) with symptomatic varicose veins secondary to great saphenous vein insufficiency treated with 1470-nm endovenous laser ablation were studied. Patients were evaluated clinically on the first day, first week, first month, and sixth month after the operation. Treated limbs were evaluated as separate treatment events. RESULTS: The short-term occlusion rate was 99% and mid-term occlusion rate was 100%. Induration or swelling was the most common minor complication (13%). No major complication such as deep venous thrombosis and pulmonary embolus occurred. Preoperatively documented mean venous clinical severity score significantly reduced from 4.9±2.3 to 2.5±1.1 (p<0.05). CONCLUSION: Endovenous laser ablation procedure of great saphenous vein with a 1470-nm diode laser is a minimally invasive, safe, and efficient treatment option in all-suitable patients with high short- and mid-term success rate.

Myocardial bridging in a child with hypertrophic obstructive cardiomyopathy.

Myocardial bridging (MB) is a rare coronary anomaly in children which is typically associated with hypertrophic obstructive cardiomyopathy (HCM) or left ventricular hypertrophy. Hypertrophic obstructive cardiomyopathy is the leading cause of sudden cardiac death in young patients, and the association of myocardial ischemia with sudden death has been recognized in patients with HCM. In this report, we describe an 11-year-old boy presented with a history of exertional, nonspecific chest pain and palpitations. Cardiac catheterization revealed MB of the left anterior descending coronary artery with evidence of intramyocardial obstruction during systole. Surgical unroofing of the affected coronary artery segment resulted in complete recovery. Myocardial bridging should be included in the differential diagnosis of children presenting with signs of ischemia.

A comparison of three tumescent delivery systems in endovenous laser ablation of the great saphenous vein.

Different systems for delivering tumescent solution exist in endovenous laser ablation (EVLA). This study evaluated three different tumescent delivery systems in patients with primary varicose veins due to great saphenous vein reflux who were treated with EVLA. In this prospective non-randomized study, 60 patients with isolated GSV varicose veins were divided into three groups. All patients received EVLA treatment. Three different tumescent solution delivery systems were used. Systems consisted of a needle and a syringe in Group 1, a needle connected to an infusion bag system in Group 2 and a peristaltic infiltration pump in Group 3. Tumescent delivery durations were in Group 1: 6.56 SD 1.18 minutes, Group 2: 6.05 SD 2.19 minutes and Group 3: 5.19 SD 1.15 minutes (P = 0.014). In the outcomes of the study there were no significant difference between groups. Although peristaltic pump systems might provide shorter tumescent delivery durations without hand fatigue, shorter duration does not have any practical importance (about 1 minute and also it is not cost-effective. For delivering tumescent solutions in EVLA procedures, there was no major superiority between systems.

The relation of serum 25-hydroxyvitamin-D levels with severity of obstructive sleep apnea and glucose metabolism abnormalities.

Obstructive sleep apnea (OSA) and 25-hydroxyvitamin-D3 (25-OH-D) deficiency are two separate disorders associating with obesity, inflammation, and impaired glucose metabolism. We aimed to investigate the vitamin D status of OSA patients regarding to potential links between lower vitamin D levels and abnormal glucose metabolism, which is one of the main adverse outcomes of OSA. Study group is composed of 190 non-diabetic subjects who were suspected of having OSA. Subjects undergone polysomnography and were grouped due to apnea-hypopnea indices (AHI) as controls (AHI < 5, n = 47), mild OSA (5 ≤ AHI < 15, n = 46), moderate OSA (15 ≤ AHI < 30, n = 47), and severe OSA (AHI ≥ 30, n = 50). Serum 25-OH-D, HbA1c, insulin levels were measured and 75-g oral glucose tolerance test was performed. Serum 25-OH-D level (ng/ml) of OSA patients were lower than control subjects (17.4 ± 6.9 vs. 19.9 ± 7.8), and decrement was parallel to severity of OSA; as 18.2 ± 6.4 (5 ≤ AHI < 15), 17.5 ± 7.4 (15 ≤ AHI < 30), and 16.3 ± 6.9 (AHI > 30), respectively (P = 0.097, r = -0.13). However, severe female OSA patients had significantly lower 25-OH-D levels (11.55 ng/ml), while control males had the highest mean value (21.7 ng/ml) (P < 0.001). Frequency of insulin resistance (IR) was 48%, prediabetes 41%, diabetes 16% in OSA patients. Mean 25-OH-D level of insulin resistant subjects (HOMA-IR ≥ 2.7, n = 77, AHI = 35.5) was lower than non-insulin resistant subjects (HOMA-IR < 2.7, n = 113, AHI = 19.8) as 16.18 ± 7.81 versus 19.2 ± 6.6, respectively (P = 0.004). 25-OH-D level of 91 non-diabetic subjects (n = 91, AHI = 19.7) was 19.5 ± 7.4, prediabetics (n = 75, AHI = 28.7) was 17.45 ± 6.9, and diabetics (n = 24, AHI = 46.3) was 13.8 ± 5.3 (P = 0.02). We showed that subjects with more severe OSA indices (AHI ≥ 15) tended to present lower vitamin D levels correlated to increased prevalence of IR, prediabetes, and diabetes. Vitamin D deficiency may play a role and/or worsen OSA's adverse outcomes on glucose metabolism. OSA patients may be considered for supplementation treatment which was shown to ameliorate abnormal glucose metabolism and inflammation.

A novel provisional aortopulmonary shunt may help avoid neonatal cardiopulmonary bypass: report of two cases.

A novel temporary aortopulmonary shunt, constructed between the aorta and main pulmonary artery with flexible cannulas, was used to facilitate right ventricular outflow tract reconstruction in one neonate and creation of a central aortopulmonary shunt in a second neonate. Although cardiopulmonary bypass is readily available in Turkey, the strategy described in the case report may prove especially useful in developing nations with limited access to cardiopulmonary bypass (CPB).

CPAP treatment in the coexistence of obstructive sleep apnea syndrome and metabolic syndrome, results of one year follow up.

OBJECTIVE: To investigate the effect of one year continuous positive airway pressure (CPAP) treatment on metabolic syndrome (MS) prevalence and components in patients diagnosed with both obstructive sleep apnea syndrome (OSAS) and metabolic syndrome. METHODS: This was a single center, observational prospective cohort study. 38 patients who were diagnosed with OSAS after polysomnographic analysis in sleep laboratory and diagnosed with MS according to National Cholesterol Education Program Adult Treatment Panel III (NCEP ATP III) guideline and underwent CPAP treatment were followed for one year. After the 1 year of follow up period on CPAP treatment, the prevalence of MS was evaluated again. RESULTS: 20 (13 male, 7 female) of 38 patients completed the entire study. Mean age was 50+/-7.7.4 patients were under treatment for diabetes mellitus (DM), 9 for hypertension (HT). After one year of follow up on CPAP treatment, the prevalence of MS decreased by 45%. When each components of MS were evaluated, no significant difference was found in fasting blood glucose, triglyceride levels and systolic and diastolic blood pressure after treatment (p>0.05). However, significant difference was observed in waist circumference (p=0.002), HDL cholesterol (p=0.001) and BMI (p=0.01) after treatment. DISCUSSION: If MS accompanies OSAS, which is a cardiovascular risk factor by itself, treatment indications of CPAP should be reevaluated. Thus, if OSA patients meet the criteria of MS even though they do not have obvious DM, HT and hyperlipidemia, initiating CPAP treatment at lower AHI levels may contribute to the prevention and development of cardiovascular disease.

Haemostatic actions of the folkloric medicinal plant extract Ankaferd Blood Stopper.

Ankaferd Blood Stopper (ABS), a standardized mixture of five plants, has been used historically as a haemostatic agent but its mechanism of action remains unknown. This study investigated the in vitro effects of ABS on haemostatic parameters. When added to plasma or serum, ABS induced the very rapid formation of a protein network and erythrocyte aggregation. The levels of coagulation factors II, V, VII, VIII, IX, X, XI, and XIII were not affected by ABS. Plasma fibrinogen activity and antigen levels were decreased following the addition of ABS, in parallel with the prolonged thrombin time. Total protein, albumin, and globulin levels decreased after the addition of ABS. Our findings suggest that ABS stimulates the formation of an encapsulated protein network that provides focal points for erythrocyte aggregation. ABS has the therapeutic potential to be used for the management of haemorrhage and this agent should be investigated further in clinical trials.

Gene expression profiling of skin and draining lymph nodes of rats affected with cutaneous contact hypersensitivity.

OBJECTIVES AND DESIGN: The present study aimed at a broad genome expression analysis of rat skin and draining lymph nodes affected with allergic contact dermatitis (ACD). METHODS: ACD was elicited in sensitized SD rats with 0.5% 2,4-dinitrofluorobenzene on skin areas distant from the sensitization sites. Involved skin and lymph nodes were dissected 8 and 24 h after challenge. RNA was hybridized on Affymetrix GeneChips. Expression data were analyzed with the GeneSpring software. RESULTS: Expression of 1,882 out of 8,799 examined genes in skin or lymph nodes was significantly (p < or = 0.01) changed compared to levels in normal tissue. After an additional 2- fold filtering, four expression patterns were selected and interpreted. Prominently up-regulated genes were IL-6, CCR-5, CCL-2, CCL-3, CXCL-1, CXCL-10, TIMP-1, OX-40, calgranulin b, ST2, beta-defensin, iNOS, STAT-1, MMP-3, MMP-9, MMP-12, and MMP-13. CONCLUSIONS: In addition to previously reported transcript changes, the present findings suggest/support that plasma cells, mast cells, and a specific IFN-gamma pathway play a substantial role in the pathogenesis of ACD.

HLA-A*0201-restricted cytolytic responses to the rtTA transactivator dominant and cryptic epitopes compromise transgene expression induced by the tetracycline on system.

The tetracycline-controlled transcription system (Tet-on) is widely used to regulate gene expression in mammalian cells. In gene therapy applications, immune responses to Tet-on proteins such as the rtTA transcription factor have been reported, raising concerns about their occurrence in humans. To monitor the HLA class I cytolytic responses against Tet-on regulators, we characterized the immunogenic CD8+ epitopes within rtTA and tTS regulators using HLA-A*0201 class I transgenic mice. Epitope prediction programs, HLA-A*0201 binding assays, and peptide immunization were used to select a set of immunogenic peptides within rtTA and tTS sequences. To identify further the rejection epitopes, we expressed Tet-on protein components in vivo and found a single dominant rtTA186 CTL epitope in the rtTA tetracycline repressor domain. Target cells expressing rtTA were susceptible to CTL lysis, and rtTA expression compromised muscle transgene engraftment. To reduce the occurrence of immune responses to rtTA protein, we mutated the dominant rtTA186 epitope and found that this leads to the appearance of subdominant epitopes. As a result, we think that an epitope modification strategy is not applicable to blunt the immune response in this model. Moreover, the identification of HLA-A*0201 rtTA epitopes allowed us to demonstrate here that the delivery of the Tet-on system with weakly immunogenic rAAV vectors does not trigger primary CTL responses in mice, in contrast to DNA transfer. Altogether, the existence of HLA-A*0201 rtTA epitopes may lead to the occurrence of immune responses depending on vectors and local inflammation in gene therapy applications involving rtTA-based regulatory systems.

Identification of an HLA-A*0201-restricted epitopic peptide from human dystrophin: application in duchenne muscular dystrophy gene therapy.

Dystrophin-based gene therapy treatments aimed at correcting the Duchenne muscular dystrophy phenotype require stable expression of normal dystrophin (DYST) protein in myocytes without immune responses, which would compromise long-term expression. To predict cytotoxic T-cell-mediated responses elicited by transgenes, we used here H-2-negative HLA-A*0201 transgenic mice and identified human DYST epitopes, which elicit HLA-A*0201-restricted cytotoxic T cell activities. Among a series of eight peptides predicted from the human DYST sequence, not shared with the endogenous mouse DYST sequence, four of them were able to bind to HLA-A*0201 molecules and to induce cytotoxic T lymphocyte (CTL) responses. After human DYST DNA transfer in muscle of HLA-A*0201 mice, only the human DYST1281 epitope, located in the spectrin-like repeat 9 domain, induced strong CD8(+) CTL responses. Using the corresponding human DYST1281 peptide/HLA-A*0201 tetramer, we detected human DYST1281-specific CD8(+) T cells in peripheral lymphoid organs and blood of HLA-A*0201 mice injected with human DYST DNA. Our results demonstrate that muscle injection with human DYST DNA systematically triggers CTL responses against this HLA-A*0201-restricted human DYST1281 peptide, which is present in long human DYST isoforms. Identification of such immunodominant human DYST epitopes and use of peptide/HLA tetramers will allow the immunomonitoring of CTL responses in HLA-phenotyped Duchenne muscular dystrophy patients undergoing gene therapy. Finally, the knowledge of HLA-A*0201-restricted human DYST peptides will be of importance to test, in mouse models, new immunomodulatory interventions allowing long-term engraftment of human dystrophin.