We report a mildly symptomatic 12-year-old boy with a very rare congenital anomaly-isolated unilateral pulmonary vein atresia. Diagnosis was made using CT angiography and its role in diagnosis is discussed.

BackgroundInflammatory dilated cardiomyopathy (iDCM) is a common debilitating disease with poor prognosis that often leads to heart failure and may require heart transplantation. The aim of this study was to evaluate sera and biopsy samples from chronic iDCM patients, and to investigate molecular mechanism associated with left ventricular remodeling and disease progression in order to improve therapeutic intervention.MethodsPatients were divided into inflammatory and non-inflammatory DCM groups according to the immunohistochemical expression of inflammatory infiltrates markers: T-lymphocytes (CD3), active-memory T lymphocyte (CD45Ro) and macrophages (CD68). The inflammation, apoptosis, necrosis and fibrosis were investigated by ELISA, chemiluminescent, immunohistochemical and histological assays.ResultsThe pro-inflammatory cytokine IL-6 was significantly elevated in iDCM sera (3.3 vs. 10.98 μg/ml; P < 0.05). Sera levels of caspase-9, −8 and −3 had increased 6.24-, 3.1- and 3.62-fold, (P < 0.05) and only slightly (1.3-, 1.22- and 1.03-fold) in biopsies. Significant release of Hsp60 in sera (0.0419 vs. 0.36 ng/mg protein; P < 0.05) suggested a mechanistic involvement of mitochondria in cardiomyocyte apoptosis. The significant MMP9/TIMP1 upregulation in biopsies (0.1931 - 0.476, P < 0.05) and correlation with apoptosis markers show its involvement in initiation of cell death and ECM degradation. A slight activation of the extrinsic apoptotic pathway and the release of hsTnT might support the progression of chronic iDCM.ConclusionsData of this study show that significant increase of IL-6, MMP9/TIMP1 and caspases-9, −8, −3 in sera corresponds to molecular mechanisms dominating in chronic iDCM myocardium. The initial apoptotic pathway was more activated by the intramyocardial inflammation and might be associated with extrinsic apoptotic pathway through the pro-apoptotic Bax. The activated intrinsic form of myocardial apoptosis, absence of necrosis and decreased fibrosis are most typical characteristics of chronic iDCM. Clinical use of anti-inflammatory drugs together with specific anti-apoptotic treatment might improve the efficiency of therapies against chronic iDCM before heart failure occurs.

Background Risk stratification in patients with non-ischemic dilated cardiomyopathy (NI-DCM) is essential to treatment planning. Global longitudinal strain (GLS) predicts poor prognosis in various cardiac diseases, but it has not been evaluated in a cohort of exclusively NI-DCM. Although deformation parameters have been shown to reflect diastolic function, their association with other hemodynamic parameters needs further elucidation. We aimed to evaluate the association between GLS and E/GLS and invasive hemodynamic parameters and assess the prognostic value of GLS and E/GLS in a prospective well-defined pure NI-DCM cohort. Methods and results Forty-one patients with NI-DCM were enrolled in the study. They underwent a standard diagnostic workup, including transthoracic echocardiography and right heart catheterization. During a five-year follow-up, 20 (49%) patients reached the composite outcome measure: LV assist device implantation, heart transplantation, or cardiovascular death. Pulmonary capillary wedge pressure (PCWP), mean pulmonary artery pressure, pulmonary vascular resistance (PVR) correlated with GLS and E/GLS (p < 0.05). ROC analysis revealed that GLS and E/GLS could identify elevated PCWP (≥ 15 mmHg) and PVR (> 3 Wood units). Survival analysis showed GLS and E/GLS to be associated with short- and long-term adverse cardiac events (p < 0.05). GLS values above thresholds of –5.34% and -5.96% indicated 18- and 12-fold higher risk of poor clinical outcomes at one and five years, respectively. Multivariate Cox regression analysis revealed that GLS is an independent long-term outcome predictor. Conclusion GLS and E/GLS correlate with invasive hemodynamics parameters and identify patients with elevated PCWP and high PVR. GLS and E/GLS predict short- and long-term adverse cardiac events in patients with NI-DCM. Worsening GLS is associated with incremental risk of long-term adverse cardiac events and might be used to identify high-risk patients.

Kęstutis Lankutis1, Virgilijus Lebetkevičius2, Virgilijus Tarutis2, Vidmantas Žilinskas2, Sigitas Čibiras2, Rita Sudikienė1, Daina Liekienė2, Vytautas Sirvydis21 Vilniaus universiteto ligoninės Santariškių klinikų Širdies chirurgijos centras,Santariškių g. 2, LT-08661 Vilnius2 Vilniaus universiteto Širdies chirurgijos centras,Santariškių g. 2, LT-08661 VilniusEl paštas: chirurgai@santa.lt Įvadas / tikslas Straipsnyje apžvelgiama mūsų techniškai sunkios arterijų sukeitimo operacijos (Jatene) ir stambiųjų kraujagyslių transpozicijos vaikams patirtis. Ligoniai ir metodai Retrospektyviai nagrinėjami 48 ligoniai, kuriems mūsų centre buvo atlikta arterijų sukeitimo operacija. Daugeliu atvejų – tai d-transpozicija ir keturiais atvejais buvo dvigubas ištekėjimas iš dešiniojo skilvelio ir transpozicija (Taussig-Bing tipas). Operacijos atliktos naudojant dirbtinę kraujotaką, kartu atlikta anatomiškai radikali ydos korekcija. Rezultatai Nuo 2002 m. sausio mėn. iki 2007 m. kovo mėn. operuoti 27 kūdikiai. Šiuo laikotarpiu, išmokus operacijos technikos, taikyta standartinė operacijos metodika. Operacinis šios grupės mirtingumas 11%. Dažniausia mirties priežastis – miokardo išemija dėl nesėkmingos vainikinių arterijų perkėlimo procedūros, esant nepalankiai anatominei jų padėčiai. Dėl pooperacinių vėlyvųjų komplikacijų stebėti keturi ligoniai, kuriems buvo įvairaus laipsnio plaučių arterijos stenozė. Du ligoniai dėl šios komplikacijos operuoti. Išvados Arterijų sukeitimo operacijos kaip fiziologiškiausios yra pirmo pasirinkimo vaikams, kuriems yra stambiųjų kraujagyslių transpozicija. Pastaraisiais metais pasiekta gana gerų ir priimtinų mirtingumo ir kokybės rodiklių. Pagrindiniai žodžiai: arterijų sukeitimo operacija, Jatene operacija, pagrindinių arterijų transpozicija Our experience in arterial switch operation of the great arteries Kęstutis Lankutis1, Virgilijus Lebetkevičius2, Virgilijus Tarutis2, Vidmantas Žilinskas2, Sigitas Čibiras2, Rita Sudikienė1, Daina Liekienė2, Vytautas Sirvydis21 Cardiac Surgery Centre of Vilnius University Hospital „Santariškių klinikos“,Santariškių str. 2, LT-08661 Vilnius2 Cardiac Surgery Centre, Vilnius University, Santariškių str. 2, LT-08661 VilniusE-mail: chirurgai@santa.lt Background / objective A review of our experience in the technically demanding arterial switch operation (Jatene) in transposition of the great arteries in children. Patients and methods 48 children who underwent an arterial switch operation at our Centre were retrospectively reviewed. The underlying pathology was d-transposition of the great arteries and a double outlet right ventricle of Taussig-Bing transposition type in four cases. The operation was performed in cardiopulmonary bypass for anatomically repair of the transposition. Results From January 2002 – March 2007, 27 infants underwent an arterial switch operation. Operative mortality in this period was 11%, in most cases from myocardial ischemia following unsuccessful transfer of a dangerous pattern of coronary arteries. Postoperative morbidity occurred in four patients who presented various degrees of pulmonary stenosis artery. Two patients required reoperation. Conclusions The arterial switch operation is considered to be a procedure of choice for correction of transposition of the great arteries. The operation involves acceptable mortality and morbidity. Key words: arterial switch operation, Jatene operation, transposition of the great arteries

A p þ v a l g o sVilniaus universiteto Ðirdies chirurgijos centre nuo 1987 metø atliekamos ðirdies transplantacijos operacijos. Per ðá laikotarpá recipientø sàraðuose buvo áraðyti 268 pacientai, atliktos 47 ðirdies transplantacijos. Ðiuolaikiniai medikamentinio, elektrofiziologinio ir chirurginio gydymo metodai gerokai padidino pacientø, laukianèiø ðirdies transplantacijos, iðgyvenimà, taèiau ðirdies transplantacija tebëra veiksmingiausias gydymo metodas terminalinëmis ðirdies nepakankamumo stadijomis. Pastaraisiais metais Vilniaus universiteto Ðirdies chirurgijos klinikoje ádiegti nauji diagnostikos metodai (spiroergometrija, NT-pro-BNP nustatymas) leidþia praktiðkai pritaikyti ðiuolaikinius atrankos kriterijus. Ðiame straipsnyje remiantis literatûros apþvalga ávardyti pagrindiniai recipientø atrankos ðirdies transplantacijai kriterijai, taikomi ir Vilniaus universiteto Ðirdies chirurgijos centre. Pagrindiniai þodþiai: ðirdies nepakankamumas, recipientø atranka, transplantacijaHeart transplantations at Vilnius University Heart Surgery Centre have been performed since 1987. During all this period, 268 patients were included into the recipients' list and 47 heart transplantations were performed. The current medical, electrophysiological and surgical methods have obviously increased the survival of patients who are waiting for heart transplantation.