Abstract:

Introduction: the importance of intravascular diagnostic methods and the frequency of their use in clinical practice is steadily increasing. However, in the Russian Federation, studies on the analysis of possibilities of intravascular imaging or physiology are sporadic, and statistical data are presented only in very generalized form. This makes it relevant to create a specialized register dedicated to these diagnostic methods.

Aim: was to present the structure, tasks and possibilities of the Russian registry for the use of intravascular imaging and physiology based on results of the first year of its operation.

Material and methods: In total, in 2021, forms were filled out for 2632 studies in 1356 patients.

Studies included all types of intravascular imaging and physiology - intravascular ultrasound, optical coherence tomography, measurement of fractional flow reserve and non-hyperemic indices.

The registry's web-based data platform includes 14 sections and 184 parameters to describe all possible scenarios for applying these methodologies. Data entry is possible both from a stationary computer and from mobile devices, and takes no more than one minute per study. Received material is converted into Excel format for further statistical processing.

Results: 13 departments participated in the register, while the share of the eight most active ones accounted for 97,5% of all entered forms. On average, 1.9 studies per patient were performed, with fluctuations between clinics from 1,6 to 2,9. Studies of the fractional flow reserve accounted for 40% of total data array, intravascular ultrasound - 37%, optical coherence tomography - 23%. Of all studies, 80% were performed on coronary arteries for chronic coronary artery disease, 18% - for acute coronary syndrome, 2% were studies for non-coronary pathology. In 41% of cases, studies were performed at the diagnostic stage, without subsequent surgery. In 89,6% of cases, this was due to the detection of hemodynamically insignificant lesions, mainly by means of physiological assessment. In 72% of cases, the use of intravascular imaging or physiology methods directly influenced the tactics or treatment strategy - from deciding whether to perform surgery or not to choose the optimal size of instruments or additional manipulations to optimize the outcome of the intervention. In the clinics participating in the register, the equipment of all major manufacturers represented on the Russian market was used.

Conclusions: the design of the online registry database is convenient for data entry. Participation in the registry of most departments that actively and systematically use methods of intravascular imaging and physiology ensured the representativeness of obtained data for analysis in interests of both practical medicine and industry, as well as for scientific research in the field of intravascular imaging and physiology. The register has great potential for both quantitative and qualitative improvement.

Abstract:

Results of successful surgical treatment of a patient with an extremely rare disease - Parkes-Weber- Rubashov syndrome, manifestating by arteriovenous malformations of the lower limb and spinal cord are presented. Endovascular embolization of arteriovenous malformation of the lower limb was treated with use of three Flipper coils due to the severity of the clinical symptoms. A conclusion about the effectiveness of this method of treatment is presented. 

References

1.     Ferrero E., Ferri M., Viazzo A. Parkes-Weber syndrome and giant superficial femoral artery aneurysm. Treatment by endovascular therapy and follow-up of 8 years. Ann Vasc Surg. 2011; 25(3): 384.e9-384.e15.

2.     Boon L.M., Mulliken J.B. Assignment of a locus for dominantly inherited venous malformations to chromosome 9p. Hum. Mol. Genet. 1994; 3: 1583-1587.

3.     Brouillard P, Vikkula M. Genetic causes of vascular malformations. Hum. Mol. Genet. 2007; 16: R140-R149.

4.     Volz K.R., Kanner C.D., Evans J. Klippel-Tmnaunay Syndrome: Need for Careful Clinical Classification. J. Ultrasound Med. 2016; 10: 7863/ultra.15.08007.

5.     Namba K. and Nemoto S. Parkes Weber Syndrome and Spinal Arteriovenous Malformations. AJNR Am. J. Neuroradiol. 2013; 34: E110-E112.

6.     Djindjian M., Djindjian R. Spinal cord arteriovenous malformations and the Klippel-Trenaunay-Weber syndrome. Surg Neurol. 1977; 8:229-37.

7.     Greene A.K., Kieran M., Burrows PE. Wilms Tumor Screening Is Unnecessary in Klippel-Trenaunay Syndrome. Pediatrics. 2004; 113: e326 - e329.

8.     Fernandez-Pineda I., Lopez-Gutierrez J.C. Parkes-Weber syndrome associated with a congenital short femur of the affected limb. Ann Vasc Surg. 2009; 23(2): 257.e1-2.

9.     Revencu N., Boon L.M., Mulliken J.B. Parkes Weber syndrome, vein of Galen aneurysmal malformation, and other fast-flow vascular anomalies are caused by RASA1 mutations. Hum Mutat. 2008; 29(7): 959-65.

10.   Revencu N., Boon L.M. Parkes Weber syndrome, vein of Galen aneurysmal malformation, and other fast-flow vascular anomalies are caused by RASA1 mutations. Hum Mutat. 2008; 29:959-65.

11.   Thiex R., Mulliken J.B. A novel association between RASA1 mutations and spinal arteriovenous anomalies. AJNR Am J Neuroradiol. 2010; 31:775-79.

12.   Sato T.N., Tozawa Y Distinct roles of the receptor tyrosine kinases Tie-1 and Tie-2 in blood vessel formation. Nature. 1995; 376: 70-74.

13.   Lelievre E., Bourbon PM. Deficiency in the p110alpha subunit of PI3K results in diminished Tie2 expression and Tie2(-/-)-like vascular defects in mice. Blood. 2005; 105: 3935-3938.

14.   Boon L.M., Mulliken J.B. RASA1: Variable phenotype with capillary and arteriovenous malformations. Curr Opin Genet Dev. 2005; 15(3): 265-269.

15.   Revencu N. Parkes Weber syndrome, vein of Galen aneurysmal malformation, and other fast-flow vascular anomalies are caused by RASA1 mutations. Hum Mutat. 2008; 29(7):959-965.

16.   Burrows PE., Gonzalez-Garay M.L., Rasmussen J.C. Lymphatic abnormalities are associated with RASA1 gene mutations in mouse and man. PNAS. 2013; 110: 8621-8626.

17.   Konyushevskaya A.A., Yaroshenko S.Ya. Klinicheskiy sluchay redkoy nasledstvennoy patologii - sindrom Klippelya-Trenone-Vebera-Rubashova v praktike vracha-pediatra [Rare hereditary pathology in practice of pediatrics - Klippel-Trenone-Weber-Rubashov syndrome (case report)]. Zdorov'ye rebenka. 2014; 2(53): 117 - 122 [In Russ].

18.   Behr G.G. CM-AVM syndrome in a neonate: Case report and treatment with a novel flow reduction strategy. Vasc Cell. 2012; 4(1): 19.

19.   Wijn R.S. Phenotypic variability in a family with capillary malformations caused by a mutation in the RASA1 gene. Eur J Med Genet. 2012; 55(3):191-195.  

Abstract:

Background: There are no randomized trials describing outcomes of multivessel percutaneous coronary interventions (PCI) (in primary anc staged revascularization) with second generation drug eluting stents (DES) in patients with ST-elevation myocardial infarction (STEMI). We are presenting preliminary results of randomized trial (NCT01781715)

Materials and methods: Six-month outcomes of 89 consecutive patients with STEMI and multivessel coronary artery disease (CAD) (SYNTAX 18.6±7.9 points) undergoing primary PCI with zotarolimus-eluting stents (Resolute Integrity; Medtronic) were studied. We used two strategies of multivessel stenting: in primary PCI (MS primary) and multivessel stenting in staged revascularisation (MS staged) (8.5±4.2 days).

Results: We evaluated results in the overall cohort of patients, including two study groups (MS primary and MS staged). During follow-up of 6 months there was no cardiac death in overall group. We observed 3 (3.4%) non-fatal myocardial infarction (MI) due to definite stent thromboses (ST) (1.3% on the number of stents). Target vessel revascularization (TVR) was performed in 2 cases (2.2%). Major adverse cardiac event (MACE) (cardiac death, MI, TVR) was diagnosed in 4.5%.

Conclusions: Resolute Integrity stents in STEMI patients with multivessel CAD are satisfactory safely and effectively as part of the strategy of multivessel stenting in primary PCI and multivessel staged PCI (8.5±4.2 days).

References

1.     Sorjja P., Gersh B.J., Cox D.A. Impact of multivessel disease on reperfusion success and clinical outcomes in patients undergoing primary percutaneous coronary intervention for acute myocardial infarction. Eur. Heart J. 2007; 28:1709-16.

2.     Jang H.L., Hun S.P., Shung Ch.Ch. Wee Hyun Park and Korea Acute Myocardial Infarction Registry Investigators. Predictors of six-month major adverse cardiac events in 30-day survivors after acute myocardial infarction (from the Korea Acute Myocardial Infarction Registry). Am. J. Cardiol. 2009;104:182-89.

3.     Rasoul S., Ottervanger J.P., de Boer M.J. Predictors of 30- day and 1-year mortality after primary percutaneous coronary intervention for ST-elevation myocardial infarction. Coron. Artery Dis. 2009; 20: 415-21.

4.     Webb J.G. Lowe A.M., Sanborn T.A. et al. Percutaneous coronary intervention for cardiogenic shock in the SHOCK trial. J.Am. Coll. Cardiol. 2003;42:138-86.

5.     Smith S.C., Jr., Feldman T.E., Hirshfeld J.W. Jr. et al. ACC/AHA/SCAI 2005 Guideline Update for Percutaneous Coronary Interventiondsummary article: a report of the AmericanCollege of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/SCAI Writing Committee to Update the 2001 Guidelines for Percutaneous Coronary Intervention). Circulation. 2006;113:156-75.

6.     Ijsselmuiden A.J., Ezechiels J., Westendorp I.C., et al. Complete versus culprit vessel percutaneous coronary intervention in multivessel disease: a randomized comparison. Am.Heart.J. 2004;148:467-74.

7.     Politi L., Sgura F., Rossi R., et al. A randomised trial of target-vessel versus multi-vessel revascularisation in ST-elevation myocardial infarction: major adverse cardiac events during long-term follow-up. Heart. 2010; 96:662-67.

8.     Fox K., Garcia M.A., Ardissino D. Guidelines on the management of stable angina pectoris: executive summary. The Task Force on the Management of Stable Angina Pectoris of the European Society of Cardiology. Eur. Heart J. 2006;27:1341-81.

9.     Gabriel S., Stefan K., James D.A. The Task Force on the management of ST-segment elevation acute myocardial infarction of the European Society of Cardiology (ESC). European Heart Journal. 2012. doi:10.1093/eurheartj/ehs215.

10.   Roe M.T., Cura F.A., Joski PS. Initial experience with multivessel percutaneous coronary intervention during mechanical reperfusion for acute myocardial infarction. Am. J. Cardiol. 2001; 88:170-173.

11.   Corpus R.A., House J.A., Marso S.P et al. Multivessel percutaneous coronary intervention in patients with multivessel disease and acute myocardial infarction. Am. Heart. J. 2004; 148:493-500.

12.   Widimsky P., Holmes Jr. David R. How to treat patients with ST-elevation acute myocardial infarction and multivessel disease? European Heart Journal Advance Access published November 30, 2010. European Heart Journal doi:10.1093/eurheartj/ehq410.

13.   Politi L., Sgura F., Rossi R. et al. A randomised trial of target-vessel versus multi-vessel revascularization in ST-elevation myocardial infarction: major adverse cardiac events during long-term follow-up. Heart.2010;96:662-667.

14.   Varani E., Balducelli M., Aquilina M. et al. Single or multivessel percutaneous coronary intervention in ST-elevation myocardial infarction patients. Catheter Cardiovasc. Interv. 2008;72:927-933.

15.   Roe M.T., Cura F.A., Joski PS. Initial experience with multivessel percutaneous coronary intervention during mechanical reperfusion for acute myocardial infarction. Am. J.Cardiol. 2001;88:170-173.

16.   Hannan E.L., Samadashvili Z., Walford G. Culprit vessel percutaneous coronary intervention versus multivessel and staged percutaneous coronary intervention for ST- segment elevation myocardial infarction patients with multivessel disease. JACC Cardiovasc. Interv. 2010; 3:22-31.

17.   Goldstein J.A., Demetriou D., Grines C.L. Multiple complex coronary plaques in patients with acute myocardial infarction. N. Engl. J. Med. 2000;343:915-22.

18.   Тарасов Р. С., Ганюков В. И., Шушпанников П. А. Исходы различных стратегий реваскуляризации у больных инфарктом миокарда с элевацией сегмента ST при многососудистом поражении в зависимости от тяжести поражения коронарного русла по шкале «- SYNTAX». Российский кардиологический журнал. 2013; 100(2):31-32. 

Abstract:

Despite the fact that so far in the literature, many cases of endovascular closure of paravalvular leak (PVL), this type of intervention is unusual and is associated with a complex technical issues. In addition, the majority of publications devoted to the correction of mitral and aortic PVL, while the description of the closing of the tricuspid valve (TV) PVL are rare.

Below is a description of our first experience of endovascular correction of TV PVL in 54 years ole patient, who underwent TV repair with «Neokor-32» - supporting ring as a correction of atrial septal defect, TV insufficiency One year after surgery the patient reported a decrease in physical activity tolerance. Echocardiography diagnosed hemodynamically significant PVL of TV, 6mm size with leakage between the left ventricle and the right atrium and formation of pulmonary hypertension. PVL was successfully treated by endovascular correction with using of device for closure of ventricular septal defect.  

References

1.     Galea A. et al. Practical applications of digital tomosynthesis of the chest. Clinical radiology. 2014; 69(4): 424-430.

2.     de Koste J. R. S. et al. Digital tomosynthesis (DTS) for verification of target position in early stage lung cancer patients. Medical physics. 2013; 40(9): 091904.

3.     Dobbins III J. T. et al. Digital tomosynthesis of the chest for lung nodule detection: interim sensitivity results from an ongoing NIH-sponsored trial. Medical physics. 2008; 35(6): 2554-2557.

4.     Vikgren J. et al. Comparison of Chest Tomosynthesis and Chest Radiography for Detection of Pulmonary Nodules: Human Observer Study of Clinical Cases 1. Radiology. 2008; 249(3): 1034-1041.

5.     Quaia E. et al. Digital tomosynthesis as a problemsolving imaging technique to confirm or exclude potential thoracic lesions based on chest X-ray radiography. Academic radiology. 2013; 20(5): 546-553.

6.     Jung H. N. et al. Digital tomosynthesis of the chest: utility for detection of lung metastasis in patients with colorectal cancer. Clinical radiology. 2012; 67(3): 232-238.

7.     Nikitin M. M. Possibilities of digital tomosynthesis in the diagnosis of various forms of pulmonary tuberculosis. REJR. 2016; 6 (1): 35-47. [In Russ].

8.     F.Kovach F., Zhebek Z. X-ray anatomical basics of lungs’ examinations. Budapest, 1958; 364 p. [In Russ].

9.     Trofimova T. N. ed. Human X-ray anatomy. SPb.: Publishing house SPbMAPO. 2005; 496 p. [In Russ].

10.   Sapin M. R. ed. Human Anatomy. Moscow, M.: Medicine. 2001; 640 p. [In Russ].

11.   Sinelnikov R. D., Sinelnikov Ya. R. Atlas of human anatomy. M.: Medicine. 1996; 344 p. [In Russ].

12.   Kokov L. S., ed. X-ray Atlas of comparative anatomy. M.: Radiology-Press., 2012; 388 p. [In Russ].

Abstract:

Endovascular correction of atrial septal defect (ASD) has become the «gold standard» of treatment, both in children and adults. In case of complicated anatomy of the defect (multiple defects, its large size, lack of edges, aneurysm of atrial septum), experts often chose surgical correction of such pathology Accumulated experience of interventional cardiology and appearance of specialized tools allow to perform a successful intervention in a non-standart situation.

Article describes cases of a successful endovascular correction of ASD in a two year child and adult patient with complicated anatomy factors. In both cases, during echocardiography, we diagnosed multiple ASD with aneurysm of atrial septum, accompanied by clinical symptoms. During multidisciplinary discussions, we identified indication for endovascular correction of the defect.

We performed successfull correction of ASD with occluder for closure of patent foramen ovale, and complete termination of left-to-right shunt on the operating table.

References

1.    Sharykin A.S. Vrozhdennye poroki serdca. [Congenital heart diseases] Moskva. Izdatel'stvo «Binom»,2009; S. 384 [In Russ].

2.     Medvedeva S.V. Metodicheskie rekomendacii. Dispansernoe nabljudenie detej s vrozhdennymi porokami serdca i sosudov [Guidelines. Clinical observation of children with congenital heart disease and blood vessels]. 2005; 5-20 [In Russ].

3.    Burakovskij V.I., Buharin V.A., Podzolkov V.I. i dr. Serdechno-sosudistaja hirurgija. Vrozhdennye poroki serdca [Cardiovascular surgery. Congenital heart diseases]. M. Medicina. 1989; 45-382 [In Russ].

4.    Dergachev A.V., Trojan V.V., Adzeriho I.Je., Kozlov O.A., Sprindzhuk M.V. Vrozhdennye poroki serdca s obednennym legochnym krovotokom. Uchebno-metodicheskoe posobie. Chast' 1 [Congenital heart diseases with depleted pulmonary circulation. Guidelines. Part 1.]. Mn.: BelMAPO. 2007; 29 il. 27 [In Russ].

5.    Amikulov B.D. Vrozhdennye poroki serdca blednogo tipa u vzroslyh [«Pale» congenital heart diseases in adults.]. Serdechno-sosudistaja hirurgija . 2004; 2: 3-9 [In Russ].

6.     Bokerija L.A., Gorbachevskij S.V. i dr. Nedostatochnost’ trikuspidal'nogo klapana i ee vlijanie na rezul'taty hirurgicheskogo lechenija defekta mezhpredserdnoj peregorodki u bol'nyh starshe 40 let. Serdechno-sosudistye zabolevanija [Tricuspid failure and its influence on results of surgical treatment of atrial septal defect in patients elder than 40]. 2009; 10 (2): 5-10 [In Russ].

7.     Nechkina I.V., Sokolov A.A. Kovalev I. A., Varvarenko V. I., Krivoshhekov E. V. Remodelirovanie serdca u detej posle jendovaskuljarnoj i hirurgicheskoj korrekcii defekta mezhpredserdnoj peregorodki [Cardiac remodeling in children after endovascular and surgical correction of atrial septal defect]. Sibirskij medicinskij zhurnal. 2012; 27(3): 77-81 [In Russ].

8.     Kurek V.V., Kulagin A.E. Anesteziologija i intensivnaja terapija detskogo vozrasta. Prakticheskoe rukovodstvo [Anesthesiology and intensive care of children]. M.: Medicinskoe informacionnoe agentstvo. 2011; S 992 [In Russ].

9.     Fredriksen P.M., Chen A., Veldtman G., Hechter S., Therrien J., Webb G. Exercise capacity in adult patients with congenitally corrected transposition of the great arteries. Heart. 85 (2). 191-195.

10.   Carole A., Warnes and ather. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). J Am CollCardiol. 2008; 52(23):1890-1947. doi:10.1016/j.jacc.2008.10.002

11.   ProkseljK., Kozelj M., ZadnikV., Podnar T. Echocardiographic characteristics of secundum-type atrial septal defects in adult patients: implications for percutaneous closure using Amplatzer septal occluders. J Am Soc Echocardiography. 2004.Nov. 17(11):1167-1172.

12.   Bokerija L.A., Kagramanov I.I., Alekjan B.G., i dr. Sravnitel'naja ocenka otdalennyh rezul'tatov korrekcii defekta mezhpredserdnoj peregorodki s pomoshhju otkrytogo i jendovaskuljarnogo metodov [Comparative estimation of long-term results of surgical and endovascular correction of atrial septal defect]. Bolezni serdca i sosudov. 2009; 3: 33-40 [In Russ].

13.   Baumgartner H., Bonhoeffer P, De Groot N.M. et al. ESC Guidelines for the management of grown-up congenital heart disease. (new version 2010).Eur Heart J. 2010; 31(23): 2915-57. doi:10.1093/eurheartj/ehq 249. Epub 2010 Aug 27.

14.   King T.D., Mills N.L. Nonoperative closure of atrial septal defects. Surgery. 1974; 75: 383-388.

15.   Kazmi T., Sadiq M., Asif-ur-Rehman, Hyder N., Latif F. Intermediate and long-term outcome of patients after device closure of ASD with special reference to complications. J Ayub Med CollAbbottabad. 2009; 21(3): 117-121.

16.   Rigatelli G., Dell'Avvocata F., Cardaioli P Five-year follow-up of transcatheterintracardiac echocardiography-assisted closure of interatrial shunts. Med. 2011; 12(6): 355-361. doi: 10.1016/j.carrev.2011.04.003. Epub2011 Jun 28.

17.   Jonas R.A. Comprehensive surgical management of congenital heart disease. London. 2004; р. 151-160.

18.   Tarasov R.S., Kartashjan Je.S., Ganjukov V.I.i dr. Transkateternaja korrekcija defekta mezhpredserdnoj peregorodki u detej razlichnyh vozrastnyh grupp[Transcatheter correction of atrial septal defect in different age children]. Rossijskij kardiologicheskij zhurnal. 2013; 3: 40-44 [In Russ].