Clinical and molecular evaluation of MEFV gene variants in the Turkish population: a study by the National Genetics Consortium.

Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disorder with recurrent fever, abdominal pain, serositis, articular manifestations, erysipelas-like erythema, and renal complications as its main features. Caused by the mutations in the MEditerranean FeVer (MEFV) gene, it mainly affects people of Mediterranean descent with a higher incidence in the Turkish, Jewish, Arabic, and Armenian populations. As our understanding of FMF improves, it becomes clearer that we are facing with a more complex picture of FMF with respect to its pathogenesis, penetrance, variant type (gain-of-function vs. loss-of-function), and inheritance. In this study, MEFV gene analysis results and clinical findings of 27,504 patients from 35 universities and institutions in Turkey and Northern Cyprus are combined in an effort to provide a better insight into the genotype-phenotype correlation and how a specific variant contributes to certain clinical findings in FMF patients. Our results may help better understand this complex disease and how the genotype may sometimes contribute to phenotype. Unlike many studies in the literature, our study investigated a broader symptomatic spectrum and the relationship between the genotype and phenotype data. In this sense, we aimed to guide all clinicians and academicians who work in this field to better establish a comprehensive data set for the patients. One of the biggest messages of our study is that lack of uniformity in some clinical and demographic data of participants may become an obstacle in approaching FMF patients and understanding this complex disease.

Pharmacokinetics of intravenous meloxicam, ketoprofen and tolfenamic acid in chukar partridge (Alectoris chukar).

1. The aim of this study was to determine the pharmacokinetics of meloxicam (MLX, 1 mg/kg body weight (BW)), ketoprofen (KETO, 2 mg/kg BW), and tolfenamic acid (TA, 2 mg/kg BW) in chukar partridge (Alectoris chukar) following intravenous (IV) administration.2. Twenty-four healthy chukar partridges were randomly divided into three equal groups (n = 8) as MLX, KETO and TA. Plasma concentrations of MLX, KETO and TA were measured using high-performance liquid chromatography-ultraviolet detection and analysed using non-compartmental analysis.3. No adverse effects were determined in chukar partridges after IV administration of MLX, KETO and TA. MLX, KETO and TA were detected in plasma up to 10, 12 and 12 h, respectively. The terminal elimination half-life of MLX, KETO and TA was 1.22, 1.77 and 1.95 h, respectively. MLX, KETO and TA exhibited volumes of distribution at a steady-state of 0.03, 0.23 and 0.41 l/kg BW, respectively. The total plasma clearance of MLX, KETO and TA was 0.02, 0.11 and 0.15 l/h/kg, respectively. The extraction ratios for MLX, KETO and TA were calculated as 0.002, 0.011 and 0.016, respectively.4. MLX, KETO and TA offer treatment in chukar partridges for various conditions with an absence of adverse reactions and properties such as short elimination half-life and low volume of distribution. However, there is a need to establish the safety and adverse effects of repeated administration, pharmacokinetics of other administration routes and pharmacological efficacy of MLX, KETO and TA in chukar partridges.

Expression and DNA methylation profiles of EZH2-target genes in plasma exosomes and matched primary tumor tissues of the patients with diffuse large B-cell lymphoma

Aims Diffuse large B-cell lymphoma (DLBCL) is the most common type of aggressive lymphoma. This study was designed to compare epigenetic alterations observed in Enhancer of Zeste Homolog 2 (EZH2)-target genes between plasma-derived exosomes and primary tumors in DLBCL patients. Main methods Exosomes were isolated from plasma of 21 DLBCL patients and 21 controls. We analyzed the methylation status of the target genes using methylation-specific PCR. We also examined whether the exosomes and the tumor samples contained transcripts of the target genes. Key findings We found that CDKN2A and CDKN2B were methylated in both plasma exosomes and primary tumor tissue samples. None of the transcripts were found in the exosomes except CDKN1B which was expressed in 8 (38%) of the exosome samples. Significance This study showed that plasma exosomes might preferably package certain target molecules from primary tumors and the exosomes containing dual methylated DNAs of CDKN2A and CDKN2B, or CDKN1B transcript may contribute to DLBCL pathogenesis (AU)

Expression and DNA methylation profiles of EZH2-target genes in plasma exosomes and matched primary tumor tissues of the patients with diffuse large B-cell lymphoma.

AIMS: Diffuse large B-cell lymphoma (DLBCL) is the most common type of aggressive lymphoma. This study was designed to compare epigenetic alterations observed in Enhancer of Zeste Homolog 2 (EZH2)-target genes between plasma-derived exosomes and primary tumors in DLBCL patients. MAIN METHODS: Exosomes were isolated from plasma of 21 DLBCL patients and 21 controls. We analyzed the methylation status of the target genes using methylation-specific PCR. We also examined whether the exosomes and the tumor samples contained transcripts of the target genes. KEY FINDINGS: We found that CDKN2A and CDKN2B were methylated in both plasma exosomes and primary tumor tissue samples. None of the transcripts were found in the exosomes except CDKN1B which was expressed in 8 (38%) of the exosome samples. SIGNIFICANCE: This study showed that plasma exosomes might preferably package certain target molecules from primary tumors and the exosomes containing dual methylated DNAs of CDKN2A and CDKN2B, or CDKN1B transcript may contribute to DLBCL pathogenesis.

Increased Expression of Pentraxin 3 in Placental Tissues from Patients with Unexplained Recurrent Pregnancy Loss.

Pentraxin 3 (PTX3), a prototypical member of the long pentraxin subfamily, is a evolutionarily conserved multimeric pattern recognition receptor involved in the humoral component of the innate immune system. Pentraxin 3 is released when tissue is stressed or damaged, and interacts with many different ligands. Pentraxin 3 exerts a pivotal role both as a regulator and as an indicator of inflammatory response in the pathogenesis of many diseases such as sepsis, vasculitis and preeclampsia. Uncontrolled inflammatory response is considered a major cause of unexplained recurrent pregnancy loss (URPL). We determined the PTX3 messenger ribonucleic acid (mRNA) and protein expression levels in placentai tissues from 50 women with URPL, and made comparison with those in 50 age-matched control subjects. In quantitative real-time polymerase chain reaction (qRT-PCR) and immunohistochemistry analyses, PTX3 mRNA and protein levels, respectively, were significantly increased in URPL patients compared with their respective controls (p = 0.0001). Although no significant correlations were identified between PTX3 expression levels and clinical parameters such as maternal age, numbers of previous pregnancy losses, and gestational age at miscarriage, PTX3 mRNA expression was significantly higher in patients with no live births than in women with previous live births (p = 0.0001). Our study suggests that tissue-specific expression of PTX3 is associated with URPL. Further larger studies are required to determine whether PTX3 expression can be used as a biomarker to manage URPL in routine clinical practice.

Pharmacokinetics of cefquinome in red-eared slider turtles (Trachemys scripta elegans) after single intravenous and intramuscular injections.

The purpose of this study was to evaluate the pharmacokinetics of cefquinome (CFQ) following single intravenous (IV) or intramuscular (IM) injections of 2 mg/kg body weight in red-eared slider turtles. Plasma concentrations of CFQ were determined by high-performance liquid chromatography and analyzed using noncompartmental methods. The pharmacokinetic parameters following IV injection were as follows: elimination half-life (t1/2λz ) 21.73 ± 4.95 hr, volume of distribution at steady-state (Vdss ) 0.37 ± 0.11 L/kg, area under the plasma concentration-time curve (AUC0-∞ ) 163 ± 32 µg hr-1  ml-1 , and total body clearance (ClT ) 12.66 ± 2.51 ml hr-1  kg-1 . The pharmacokinetic parameters after IM injection were as follows: peak plasma concentration (Cmax ) 3.94 ± 0.84 µg/ml, time to peak concentration (Tmax ) 3 hr, t1/2λz 26.90 ± 4.33 hr, and AUC0-∞ 145 ± 48 µg hr-1  ml-1 . The bioavailability after IM injection was 88%. Data suggest that CFQ has a favorable pharmacokinetic profile with a long half-life and a high bioavailability in red-eared slider turtles. Further studies are needed to establish a multiple dosage regimen and evaluate clinical efficacy.

Mutational status of EZH2 and CD79B hot spots in mature B-cell non-Hodgkin's lymphomas: novel CD79B variations have been revealed.

OBJECTIVE: We aimed to determine the hot spot mutational frequencies of Enhancer of Zeste homolog 2 (EZH2) and cluster of differentiation 79B (CD79B) genes in a cohort of mature B-cell non-Hodgkin's lymphomas. PATIENTS AND METHODS: DNA samples from formalin-fixed and paraffin embedded (FFPE) tissues from a total of 37 patients with mature B-cell non-Hodgkin lymphomas were included in the study. Molecular genetic analysis was performed by direct sequencing of the DNA samples. RESULTS: We analyzed formaldehyde fixed-paraffin embedded (FFPE) tumor tissue samples from 17 female and 20 male patients with a median age of 63.7 years at the time of diagnosis. None of the patients had previously reported hot spot mutations in EZH2 and CD79B, but previously unreported single nucleotide variations of CD79B were present in nine patients. rs779833118 was the most frequent variation (7/37 patients, 18.9%). A non-synonymous variation rs757407417, which could have a potentially damaging outcome, was detected in two patients. CONCLUSIONS: None of the patients had well-known hot spot mutations in EZH2 and CD79B. However, we detected novel CD79B variations in mature B-cell non-Hodgkin's lymphoma patients.

Doppler ultrasonographic evaluation of the carotid and vertebral arteries in children with Crimean-Congo hemorrhagic fever.

AIM: In children with Crimean-Congo hemorrhagic fever (CCHF), the aim of present study was to determine the cerebral hemodynamic changes assessed with Doppler ultrasonography with measurements of lumen diameter (LD), peak systolic velocity (PSV), end-diastolic velocity (EDV), resistive index (RI), pulsatility index (PI), and total cerebral blood flow volume (tCBFV) of common carotid artery (CCA), internal carotid artery (ICA, and vertebral artery (VA). METHODS: This case-control study examined children with clinically defined CCHF as cases (N.=24) and healthy children as controls (N.=17). The CCHF and healthy children underwent ultrasonography of the CCA, ICA and VA for the measurements of flow velocity and cerebral blood flow volume. Doppler ultrasonography were performed in the CCHF children if vital signs were stable and fever lower than 37.5 °C. RESULTS: There was no significant difference between the CCHF and healthy children in the lumen diameter of study arteries (P>0.05). PSV and EDV values were significantly higher in the CCHF children compared to the controls (P<0.05). Overall, the CCHF did not cause considerable change in RI and PI values (P>0.05). tCBFV values of CCHF children was found higher compared to healthy controls (P<0.05). CONCLUSION: Overall, in children, CCHF causes an increase in the cerebral blood flow as determined by Doppler ultrasonography with flow velocity and cerebral blood flow volume measurements; however, this increase need to be accepted as mild to moderate degree. Several pathophysiological derangements may be contributory to the increased cerebral blood flow in children with CCHF.

Association of calcium channel blocker use with lower hemoglobin levels in chronic kidney disease.

AIM: To search whether calcium channel blockers (CCBs) are associated with lower hemoglobin levels in chronic kidney disease (CKD) patients who are not on renal replacement therapy (RRT), vitamin D and anti-anemic treatment. PATIENTS AND METHODS: CKD patients were classified into two groups. Patients on CCBs treatment (103 patients) and patients not using CCBs (104 patients) were compared cross-sectionally regarding clinical findings, complete blood count (CBC), biochemistry and regular medication use. Patients with polycystic kidney disease, comorbidities that could influence CBC other than iron deficiency of obscure origin, patients receiving RRT, erythropoietin (EPO), vitamin D, phosphate binders and drugs that could influence CBC were excluded. Under dependent variable of CCB use, all significantly different independent variables were subjected to multivariate binary logistic regression analysis (MBLRA). RESULTS: Lower hemoglobin, lower bilirubinemia, higher serum EPO, higher systolic blood pressure were observed in CCB users. Two groups were similar concerning age, gender, BMI, CKD etiology, CKD stage, pretibial edema prevalence, cardiothoracic index, diastolic blood pressure, corrected reticulocyte count, BUN, creatinine, eGFR, proBNP, parathormone, alkaline phosphatase, phosphorous, corrected calcemia, sCRP, relative EPO deficiency and prevalence of relative EPO deficient patients. Groups were comparable regarding comorbidities, types and usage frequencies of all antihypertensive medications other than CCBs. Higher systolic blood pressure and lower hemoglobin were significantly associated with CCB use after MBLRA. CONCLUSIONS: Hemoglobin was significantly lower in CCB users compared to non-users, among CKD patients who did not receive RRT, EPO, phosphate binders, vitamin D, iron, vitamin B12 and folic acid.

A case of toxic epidermal necrolysis-like skin lesions with systemic lupus erythematosus and review of the literature.

OBJECTIVE: Subepidermal bullous lesions and toxic epidermal necrolysis-like (TEN-like) lesions can occur in patients with systemic lupus erythematosus (SLE). In this report, we describe a case of a patient previously diagnosed with SLE who experienced TEN-like skin lesions with unusual subacute progression in the context of the current literature. METHODS: We present a recent case of TEN-like lupus erythematosus and review of studies published in English identifying SLE cases mimicking TEN, accessed via PubMed and Google Scholar databases. The keywords used in the search were: TEN, SLE, TEN-like SLE, and TEN-like lesions. The search covered all articles from January 1980 to November 2011. RESULTS: A 52-year-old male presented with fatigue, weakness, and weight loss (23 kg in two months). Skin redness started across nose and cheeks six months before admission. Bilateral pleural effusions were observed in a thorax tomography taken in the referral hospital two months prior to admission. Because of articular involvement, antinuclear antibody (ANA), and anti-dsDNA positivity, the patient was diagnosed with SLE. We initiated a punch skin biopsy, and the findings were consistent with Stevens-Johnson syndrome. There was marked basal layer necrosis in the epidermis, and there was predominantly lymphohistiocytic infiltrate in the dermis. A total of 22 cases, including our case, with TEN-like lupus erythematosus were reported in the literature. In addition, cutaneous lupus had positive ANAs in 18 of 22 patients (81.8%). The patients were aged 12 to 76 years; 21 cases were women and only one patient was male. DISCUSSION: Skin involvement, including the rare variant of TEN-like acute cutaneous SLE, is very common among SLE patients. The acute syndrome of pan-epidermolysis or apoptotic pan-epidermolysis may become a useful designation when considering a clinical diagnosis of drug-induced TEN or SLE. Further studies are required to verify our findings.

Assessment of cardiac ultrasonography in predicting outcome in adult cardiac arrest.

OBJECTIVE: A prospective follow-up study to evaluate the ability of cardiac ultrasonography performed by emergency physicians to predict resuscitation outcome in adult cardiac arrest patients. METHODS: Ultrasonographic examination of the subxiphoid cardiac area was made immediately on presentation to the emergency department with pulseless cardiac arrest. Sonographic cardiac activity was defined as any detected motion within the heart including the atria, ventricles or valves. Successful resuscitation was defined as any of: return of spontaneous circulation for ≥ 20 min; return of breathing; palpable pulse; measurable blood pressure. RESULTS: The study enrolled 149 patients over an 18-month period. The presence of sonographic cardiac activity at the beginning of resuscitation was significantly associated with a successful outcome (19/27 [70.4%] versus 55/122 [45.1%] patients without cardiac activity at the beginning of resuscitation). CONCLUSIONS: Ultrasonographic detection of cardiac activity may be useful in determining prognosis during cardiac arrest. Further studies are needed to elucidate the predictive value of ultrasonography in cardiac arrest patients.

The determination of relationship between "excision repair cross-complementing group 1" (ERCC1) gene T19007C and C8092A single nucleotide polymorphisms and clinicopathological parameters in non-small cell lung cancer.

DNA repair plays a key role in prevention of carcinogenesis and one of the most important DNA repair mechanisms is nucleotide excision repair (NER) pathway. This pathway includes a number of genes such as excision repair cross-complementing group 1 (ERCC1) gene which are responsible for the 5' incision of damaged DNA. A reduced DNA repair capacity associated with ERCC1 mRNA level has been observed in lung carcinogenesis. Two single nucleotide polymorphisms (SNPs) in ERCC1 gene, T19007C (rs11615) and C8092A (rs3212986), reportedly predict to affect the mRNA of ERCC1 in non-small cell lung cancer (NSCLC). To examine the role of two common SNPs in ERCC1 gene further, we conducted this study where 80 cases histopatologically diagnosed as NSCLC were genotyped. Genomic DNA was extracted from formalin-fixed, paraffin embedded tissues and two SNPs were analyzed using real-time PCR. The distributions of TT, TC, and CC genotypes of the T19007C SNP were 40, 44 and 16%, respectively. Significantly increased frequency of the patients carrying at least one 19007C allele was observed in early stage compared to advanced stage (P=0.002). And also, the frequency of TC and CC genotypes significantly increased in younger patients compared to older patients (P=0.035). Regarding C8092A SNP, the distribution of CC, CA, and AA genotypes was 38, 51 and 11%, respectively. There was no significant difference in the genotype distribution between C8092A SNP and clinicopathological parameters. This study indicated that harboring at least one 19007C allele may have protective effect in NSCLC.

Expression of ERCC1 and its clinicopathological correlations in non-small cell lung cancer.

Excision Repair Cross-Complementing Group 1 (ERCC1) is an important DNA repair gene, playing critical role in nucleotide excision repair pathway and having a significant influence on genomic instability. Some studies support that ERCC1 might be a potential predictive and prognostic marker in non-small cell lung cancer (NSCLC). ERCC1 has also been shown to be a promising biomarker in NSCLC treated with a cisplatin-based regimen. Therefore, the determination of ERCC1 expression at DNA, mRNA and protein level in different stages of NSCLC is still an important topic in the cancer. Ninety-one formalin-fixed paraffin-embedded tumor samples histopathologically diagnosed as NSCLC were examined in this study. ERCC1 expression at protein level were scored by immunohistochemistry. The gene amplification and mRNA expression levels for ERCC1 were determined by real-time quantitative PCR. There was complete concordance among the three methods in 39 tumor samples (42.9%). A strong correlation was found between DNA amplification and mRNA expression (r=0.662) while there was no correlation between mRNA and protein assessment for ERCC1 expression (r=-0.013). ERCC1 expression at mRNA and DNA level (63.1 and 84.2%, respectively) in tumors at stage III was higher than at the other stages. In contrast, the protein expression at stage II and III (56.6 and 52.6%, respectively) of NSCLC was lower than that of tumors with stage I NSCLC. These results show that the mechanism by which ERCC1 expression might play a role in tumor behavior. This study was also confirmed that the appropriate validation and qualification in methods used for ERCC1 status were needed before its clinical application and implementation.

The roles of extracellular matrix proteins, apoptosis and c-kit positive cells in the pathogenesis of ureteropelvic junction obstruction.

AIM: To investigate histopathological changes in ureteropelvic junction obstruction (UPJO) from an etiological perspective. PATIENTS AND METHODS: Medical records of patients with UPJO were reviewed and pathological specimens collected. Nephrectomy materials from forensic autopsies were taken as controls. Specimens were assessed with light microscopy. Fibronectin, type 4 collagen, laminin, Bax and Bcl-2 expression for apoptosis, together with interstitial cells of Cajal determination with c-kit were determined immunohistochemically. Staining scores were evaluated semiquantitatively. Results were evaluated using Mann-Whitney U-test. RESULTS: Control group comprised 14 children (median age, 3.5 years; 6 months-17 years). Study group comprised 22 children with UPJO (median age, 9 months; 1 month-10 years). Light microscopy revealed non-specific inflammation, epithelial proliferation and atrophy with fibrosis in the smooth muscle of the UPJ in all patients. Fibronectin, type 4 collagen and laminin were found to be significantly increased in UPJO at the intrafascicular space of smooth muscle and the matrix of stroma. Bcl-2 expression was increased in UPJO. c-Kit was unable to stain interstitial cells of Cajal, but staining for mast cells was significant. CONCLUSIONS: High expression of fibronectin, laminin and type 4 collagen may indicate a relation to the pathogenesis of UPJO. Defective kidney morphogenesis, during branching and tubulogenesis of ureteric bud, may be responsible for this congenital pathology.

Extended endoscopic endonasal approach to the pterygopalatine fossa: anatomic study.

AIM: Our aim in this study was to recognize the endoscopic anatomy of the pterygopalatine fossa (PPF) and the anatomic variations of the related neurovascular structures, to define the endoscopic endonasal approach to this region. METHODS: Six fresh adult cadavers were studied (N=6) by endoscopic endonasal approach. To reach the pterygopalatine fossa endonasally, we performed the middle meatus transpalatine approach in 2 cadavers, the middle meatus transnasal approach in 2 cadavers and the inferior turbinatectomy transnasal approach in 2 cadavers. The superior and posterior walls of the maxillary sinus were defined and studied. The sphenopalatine foramen is widened by drilling the orbital process of the foramen and the sphenopalatine artery was exposed. The posterior wall of maxillary sinus was opened to expose the pterygopalatine fossa and its neurovascular contents, which were studied and documented. RESULTS: The PPF was easily approached by endoscopic transnasal transmaxillary approach. The PPF region was best exposed by the middle meatus tranasal approach. In the PPF; infraorbital nerve, vidian nerve, major palatine nerve, the infraorbital artery, internal maxillary artery, sphenopalatine artery, descending palatine artery, posterior superior alveolar artery and buccal artery were exposed. According Morton and Khan morphologic classification of the third portion of the internal maxillary artery we found intermediate type in 2 cadavers (33.3%), M' type in 2 cadavers (33.3%), T' type in 1 cadaver (16.7%) and Y' type in 1 cadaver (16.7%). Also, according to Chio and Park classification of the branching type of the infraorbital artery, posterior superior alveolar artery and deep palatine artery, we found type I in 4 cadavers (66%) and type II in 2 cadavers (33%). CONCLUSIONS: During the endoscopic transnasal transmaxillary approach to the PPF, it is possible to face wide range of variations in every phase of the approach. Understanding the anatomy of this region and the neurovascular relations from the endoscopic view by cadaver dissections will help us to performed more controlled and safe surgery.

Folate and vitamin B12 levels in abnormal pap smears: a case control study.

OBJECTIVE: This study aimed to explore the risk of abnormal cervical cytology in relation to serum folate and vitamin B12 levels, and demographic characteristics. PATIENTS AND METHODS: A case-control study was carried out among women attending the gynecology clinic for cervical cytology screening. At the follow-up clinic visit, fasting blood samples were collected from 103 cases with abnormal cervical cytology of the cervix and 175 controls with cytological normal smears and serum folate and vitamin B12 levels were measured. Data from cases and controls were compared. Logistic regression analysis was used to estimate the relative odds of abnormal cytology with increasing vitamin B12 levels. RESULTS: There were no significant differences between cases and controls in demographic characteristics and mean folate levels (p > 0.05). The mean vitamin B12 level of cases was significantly lower than that of controls (p < 0.001). Dichotomized logistic regression analyses of vitamin B12 levels were significantly different (p < 0.001). The predicted percentages at a cut-off value of 0.5 were as follows: sensitivity 70.0%, specificity 74.6%, positive predictive value 71.8% and negative predictive value 72.6%. The logistic regression analysis of the 1st and the 4th quartiles of vitamin B12 levels showed a significant difference p < 0.001, OR: 1.525 (CI, 1.175-1.875). CONCLUSION: The results of this study suggest that lower vitamin B12 levels are associated with abnormal cervical cytology. It is recommended that women should consume not only folate-rich foods such as fruits and vegetables but also vitamin B12-rich foods such as meat, fish, milk products and eggs in a balanced way.

Internal thoracic artery as main blood supply to the lower limbs. Case report.

A 70-year-old male patient was admitted with symptoms of unstable angina pectoris and claudication. He presented critical left main and right coronary artery disease and juxtarenal aortic occlusion at the same time. Internal thoracic artery increased in diameter with many collateral branch arteries distally. Coronary artery bypass graft operation was performed immediately. Saphenous veins were used for conduit. Internal thoracic artery was let intact to avoid any ischemic problem of the lower extremities. DSA performed postoperatively showed that the internal thoracic artery was the unique blood supply to the leg. In the absence of emergency of peripheral revascularization two staged surgical approach (CABG first and aortobifemoral bypass some months later) was considered. However, in patients with severe peripheral vascular disease or even in patients whom intraaortic balloon-pump indicated postoperatively, combined surgical approach is necessary. In all coronary artery disease patients with Leriche syndrome, ITA dependent lower extremity circulation should be thought preoperatively. Extra-anatomic peripheral bypass is a reasonable alternative choice in combined procedures with low morbidity, especially in elderly patients having coexisting disease.

Management of a large pseudo-aneurysm secondary to balloon angioplasty for aortic coarctation.

Balloon dilation of native aortic coarctation has been employed safely and effectively in selected patients and with good results during follow-up. However, pseudo-aneurysm formation at the site of dilatation occurs in about 2%-8% of patients. Although the majority of these aneurysms remain stable, they occasionally may increase in size during follow-up, raising concerns in regard to potential rupture, massive bleeding, and death. We report a case of aneurysm formation after balloon dilation of native coarctation of the aorta, treated successfully with the interposition of a tube graft.

Right ventricular outflow tract reconstruction for tetralogy of fallot with abnormal coronary artery: experience with 35 patients.

BACKGROUND: An abnormal coronary artery crossing the right ventricle outflow tract (RVOT) complicates complete repair of tetralogy of Fallot (TOF). We have evaluated surgical options for RVOT reconstruction for this group of patients. METHODS: Between 1984 and 2002, 35 TOF patients with abnormality of coronary arteries underwent total correction. Age of these patients ranged from 1 to 14 years (mean 5.8 +/- 2.8 years). All patients were diagnosed by echocardiography and 25 patients had cardiac catheterization. All the abnormal coronary arteries were spared at the operation. In 24 patients a transannular patch was applied for RVOT reconstruction. In three patients with an adequate pulmonary annulus, oblique ventriculotomy incisions, and in two patients, transatrial approaches were performed. "Two-patch" technique was chosen for two patients. In four patients placement of an extracardiac conduit was necessary. RESULTS: Mortality was observed in four patients, in two of them due to suspected myocardial ischemia. None of the surviving patients needed reoperation. All of them were in NYHA class I. The follow-up period for patients without extracardiac conduit was between 14 and 96 months (mean 50.2 months) and for the patients with extracardiac conduit ranged 36 to 98 months (63.5 months). The mean gradients measured by echocardiography were, respectively, 5 mmHg (range 0 to 35 mmHg) and 23.75 mmHg (range 20 to 25 mmHg). CONCLUSIONS: Definitive repair of TOF patients with abnormal coronary arteries can be performed in early childhood, but care should be taken to leave at least 1 cm of myocardium between the sutureline and the abnormal coronary artery. Detailed evaluation of the patients preoperatively is mandatory to identify the strategy and timing of the operation.