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CLINICAL CASE OF SIMULTANEOUS RADICAL TREATMENT OF TETRALOGY OF FALLOT WITH MAJOR AORTOPULMONARY COLLATERAL ARTERIES
Kuatbekov K.N., Egizekov A.L., Musagaliev D.T., Maslov T.V., Sydykov E.T., Mishin A.V., Baizhigitov N.B., Suieubekov B.Y.
Center of Modern Medicine «Mediterra», Institute of Surgery LLP, Almaty, Kazakhstan Abstract
Tetralogy of Fallot (TOF) combined with major aortopulmonary collateral arteries (MAPCA) is a severe congenital heart defect due to the combination of a triad of cardiac malformation withan additional vascular anomaly of the small circulatory system. To date, there is no single accepted standard in which sequence and according to which criteria radical surgical correction of combined anomalies is indicated. This paper describes a clinical case of simultaneous opentreatment of TOF and endovascular occlusion of the MAPCA in an 8-month old child. Based on the evidence base of a large study, our patient belonged to the group where after TOF correction the therapeutic way of MAPCA treatment was used at first, which was ineffective in 38% and led to surgical methods of MAPCA occlusion. Choosing the way of simultaneous simultaneous surgical treatment of two pathologies allowed us to minimize postoperative risks and achieve good clinical results.
https://doi.org/10.35805/BSK2022IV013 Kuatbekov K.N. orcid.org/0000-0002-2679-5097 Egizekov A.L.
orcid.org/0000-0001-7979-2239 Musagaliev D.T. orcid.org/0000-0002-1914-8449 Maslov T.V.
orcid.org/0000-0002-8720-6508 Sydykov E.T.
orcid.org/0000-0003-2146-1634 Mishin A.V.
orcid.org/0000-0003-2858-6181 Baizhigitov N.B. orcid.org/0000-0001-5041-4277 Suieubekov B.Y.
orcid.org/0000-0001-8675-1435
Corresponding author.
Mishin A.V. - Cardiac surgeon,
Department of Pediatric Cardiac
Surgery, Center for Modern Medicine
«Mediterra» LLP «Institute of Surgery»,
Almaty Kazakhstan
E-mail: avm_mishin@mail.ru
Conflict of interest
The authors declare that they have no
conflicts of interest
Keywords:
Tetralogy of Fallot, major aortopul-monary collateral arteries, clinical case, congenital heart disease, heart surgery
Улкен колка-екпе коллатералдары бар Фалло тетрадасын симуляцияланган радикалды емдеудi жYрriзудщ клиникалык жагдайы
Куатбеков К.Н., Егизеков А.Л., Мусагалиев Д.Т., Маслов Т.В., Сыдыков Е.Т., Мишин А.В., Байжигитов Н.Б., Суйеубеков Б.Е.
«Медитерра» Заманауи медицина орталыгы, ЖШС «Хирургия институты», Алматы Казахстан
Ацдатпа
Фалло тетрадасы (ФТ) улкен цолца-екпе коллатералдарымен 6ipiKmipmaeH журек ацауыныц триа-дасыныц кшi цанайналымы шецбер^ц цосымша тамырлы ауытцуымен уйлесуне байланысты ауыр туа бткен журек ацауы болып табылады. БYгiнгi куш бiрiктiрiлген ауытцуларды радикалды хирургиялыц тузету цандай реттлкпен жэне цандай критерийлер бойынша орындалатыны туралы керсетлген б'рыцгай цабылданган стандарт жоц. Бул жумыста 8 айлыц балага ФТ жэне эндоваскулярлыц сэулелк окклюзияны ашыц эдспен бiр мезглде емдеудц клиникалыц жагдайы сипатталган. Yлкен зерттеудц дэлелд'1 базасына суйене отырып, б'зд'щ науцас ФТ тузетлгеннен кейн алдымен сэулелерд'1 емдеудц терапиялыц жолы цолданылган топца кiрдi, оныц 38%-ы тиiмсiз болып шыцты, бул сэулелерд'1 окклюзиялаудыц хирургиялыц эдстерне жугшуге экелд'1. Б'рден ек патологияны симуляциялыц хирургиялыц емдеу жолын тацдау б'!зге операциядан кей^ цауiп-цатерлердi азайтуга жэне оцтайлы клиникалыц нэтижеге цолжетюзуге мумкШк берд'1.
Хат алысатын автор. Мишин А.В. - «Хирургия институты» ЖШС «Медитерра» заманауи медицина орталыгы, балалар кардиохирургиясы белiмiнiц кардиохирургы, Алматы ц., Крзацстан
E-mail: avm_mishin@mail.ru
Мудделер цацтыгысы
Авторлар мудделер цацтыгысыныц жоцтыгын мэлiмдейдi
ТYЙiн сездер:
Фалло тетрадасы, улкен аорто-екпе коллатералдары, клиникалыц жагдай, туа вткен журек ацауы, журекоперациясы.
Клинический случай проведения симультанного радикального лечения тетрады Фалло с большими аорто-лёгочными коллатералями
Куатбеков К.Н., Егизеков А.Л., Мусагалиев Д.Т., Маслов Т.В., Сыдыков Е.Т., Мишин А.В., Байжигитов Н.Б., Суйеубеков Б.Е.
Центр современной медицины «Mediterra», ТОО «Институт хирургии», г. Алматы, Казахстан
Автор для корреспонденции. Мишин А.В. - кардиохирург, отделения детской кардиохирургии, Центр современной медицины «Mediterra» ТОО «Институт хирургии», г. Алматы, Казахстан E-mail: avm_mishin@mail.ru
Конфликт интересов
Авторы заявляют об отсутствииконфликта интересов
Ключевые слова:
тетрада Фалло, большие аорто-лёгочные коллатерали, клинический случай, врожденный порок сердца, операция на сердце
Аннотация
Тетрада Фалло ^Ф) в сочетании с большими аорто-легочными коллатералями (MAPCA) представляет собой тяжелый врожденным порок сердца, обусловленный сочетанием триады кардиального порока с дополнительной сосудистой аномалией малого круга кровообращения. На сегодняшний день не существует единого принятого стандарта в какой последовательности и по каким критериям показана радикальная хирургическая коррекция сочетанных аномалий. В настоящей работе представлено описание клинического случая одновременного лечения открытым способом ТФ и эндоваскулярной окклюзии БАЛК ребёнку 8 месяцев. Основываясь на доказательной базе крупного исследования, наш пациент входил в группу где после коррекции ТФ сначала применялся терапевтический путь лечения БАЛК, в 38% который оказывался неэффективным что приводило прибегать к хирургическим методам окклюзии БАЛК. Выбрав путь симультанного хирургического лечения сразу двух патологий, позволило нам минимизировать послеоперационные риски и добиться хорошего клинического результата.
Introduction
Tetralogy of Fallot (TOF) combined with major aortopulmonary collateral arteries (MAPCA) is a severe congenital heart disease aggravated by additional volume of cross over blood flow from the large to small circulatory system. There is no special standard for transcatheter occlusion of collaterals, there are no clear algorithms of surgical tactics, and therefore the surgeon faces the task of determining the indications and times tages of correction of combined TOF and MAPCA pathology. To determine the hemodynamic significance of MAPCA, and thus indicate the need for their one-stage closure, an attempt was made in The Second Xiangya Hospital of China on 380 patients operated on over a 10-year period [1]. These patients accounted for 28.1% of all operated TOF (1351 cases), of which MAPCA occlusion was performed in 4.8% of cases. A mathematical formula for the MAPCA occlusion index was derived, where K=((£R2)/Wt), R is MAPCA diameter and Wt is patient weight. With K>2, collateral occlusion and surgical correction are recommended to be performed simultaneously, with 1< K< 2, the need forcollateral occlusion depends on the postoperative condition of patients with more than the standard increase in artificial pulmonary ventilation (APV) time for this pathology. In this group, the need for MAPCA occlusion was 38%.
During radical TOF correction, if hemodynamically significant MAPCA are not closed, severe congestion in the small circle of the circulation and bypass of the great circle of thecirculation are expected in the postoperative period [2]. In such situations, a longer period of ventilatory ventilation is required and many patients have complications such as low cardiac output syndrome, pulmonary edema, lung infection and pleural effusion. The tandem approach, in which endovascular MAPCA occlusion immediately after radical open surgery is the second stage, effectively solves this problem [3]. Case study
A child 8 months old, 7 kg was admitted to the
clinic on 09/28/2022 with diagnosis: TOF, PFO, PDA, MAPCA with complaints of shortness of breath, lividity of the skin, rapid fatigue during feeding. The basic diagnostic tests: ECG, echocardiography (ECHO) were performed. Instead of contrast computed tomography (CT), cardiac catheterization (CC) was performed. The defect was confirmed by ECHO data: Aorta: 1.4 cm, bulbus shifted to the right by 50%. MAPCA of 0.3 cm was not excluded. Pulmonary artery (PA): valve 0.55 cm; flaps compacted, trunk 0.6 cm. The branches of the pulmonary artery are 0.6 cm each. The gradient on the PA valve was 76/45 mmHg. Degree 0-1 regurgitation. Interventricular septum: subaortic defect 1.1 cm. Interatrial septum: PFO 0.15cm. Cardiac cavities: right sections moderately dilated, Myocardial contractility: satisfactory. To clarify cardiac anatomy and extracardiac vascular structures, the following was performed: CC from the right ventricle (RV)-the aorticarch with the PA trunk and branches was contrasted simultaneously, the RV cavity increased in volume, moderate hypoplasia of the PA trunk and valve ring, moderate kinking of the left PA branch mouth, the middle and distal segments of PA branches were developed satisfactorily (Fig. 1). There was marked tubular stenosis in the exiting RV. In thoracic aortography, there is contrasting hypervascularization zones of both lungs across all fields, due to the presence of a developed MAPCA - bronchial and intercostal arteries branching off in the projection Th 2-3 of the thoracic spine, with adiameter of MAPCA afferent arteries of 1.5-3.0 mm (Fig. 2). Tonometry in mm Hg: PA trunk 30/5, middle 13, PJ75/3, distal segment of right and left branches of PA 24/7 (middle 12). The diameter of the distal segments of the PA right 6.4 mm (Z score 0.03), left 7.6 mm (Z score1.69) PA trunk 7.3mm (Z score-2.12) (Fig.3), PA valvering 5.5 mm, aorta valvering 13.8 mm, at diaphragm level 6.1 mm.
Diagnosis
TOF, subclavian stenosis with moderate hypo-plasia of the valvering and PA trunk. Developed MAPCA network on both sides.
ВЕСТНИК ХИРУРГИИ КАЗАХСТАНА №73 • 2022
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Figure 1.
Angiogram. Enlarged and hypertrophied right ventricle, exit section with marked tubular stenosis, hypoplasia of the pulmonary artery trunk and valvering, satisfactorily developed pulmonary artery branches
Figure 2.
Angiogram. MAPC - bronchial branch originating from the arch B of the aortic segment with a diameter of 3.0 mm
Figure 3.
Z score of the pulmonary artery branches and trunk by cardiac cavity probing
Height (cm): Weight (kg): BSA formuia:
Site
RVD: EVSd: IVSs: LVIDd: LVIDs: LVPWd: LVPWs: Aortic Annutus: Sinuses: ST Junction: Transverse Arch: Isthmus: Distai Arch: Ao at Diaphragm: Pulmonary Annutus: MPA: RPA: UA:
Mitral Anntrfus: Tricuspid Annuius: Left Atrium:
Measured (cm) Mean
68
DuBois
J 0.35 M
0.73
0.64
0.76
Update
1.21 0.41 0.57 2.39 1,48 0.34 0.66 o.ss 1.22 0.97 0.99 0.69 0.74 0.71 1.01 1.05 0.64 0.57 1.43 1.61 1,45 reset
Range
[0.31 - 1. (0,29 - 0. (0.42 - 0 (2.02 - 2. (1.20 - 1. (0.25 - 0. (0.51 - 0 (0.74 - 1. (1,02 - 1. (0,77 - I. (0.77 - 1. (0.53 - 0. (0,56 - 0. (0.57 - 0. (0.78 - 1. (0,81 - 1. (0.49 - 0. (0.43 - 0 (1.16 - 1. (1.18 - 2. (1.15 - 1.
80) 59) 77)
31)
32) 47) 34) 03) 46) 20) 28) 91) 96)
33)
29)|-2.12 36)
34) 75) 90) 20) 33)
0.03 1.69
On 11.10.2022 we performed elective surgical correction under the conditions of cardiopulmonary bypass: VSD repair with anautopericardial patch, in fundibulotomy, PA valve commissurotomy with valve and PA fibrous ring preservation, RV outlet plasty with anautopericardial patch, PFO suturing. After the operation the child was immediately taken from the cardiac surgery room to the Cath-Lab operating room. Course of intervention: retrograde puncture and catheterization of the right common femoral artery by Seldinger. A JR 3.5 4Fr type catheter was and placed on the aortic arch, and thoracic aortography was performed, in which contrasting
zones of hypervascularization of both lungs were noted throughout the fields, due to the presence of a developed 3 mm diameter MAPCA system (Figure 4). The source of MAPCA was a bronchial branch originating from the aorticarch. Given the angiographic findings, MAPCA embolization was recommended. A BS Renegade microcatheter was installed at the kostium of the pathological branch, and embolization with BS Contour 45-150|jM pVa particles - 1 vial was performed. At the control angiography the contrast intensity was reduced by 100% (Fig. 5). There were no complications during the operation.
Figure 4.
Angiogram. Catheter inserted into MAPCA, thoracic aortography with contrasting areas of hypervascularization of both lungs across all fields
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BECTHMK XMPyPrMM KA3AXCTAHA №73 • 2022
Control postoperative ECHO: tricuspid valve 1st degree regurgitation. Mitral valve: 0-1 degree regurgitation. Aorta: 0-1 degree regurgitation. Abdominal aorta: main blood flow. Pressure gradient on the PA valve: 10/5 mmHg, regurgitation 0-1 degree. Left ventricular ejection fraction 64%. Interventricular septum: patch, shunt 1 mm. Atrial septum: tightly sutured. The child was extubated 44 hours after the simultaneous surgeries, on the 6th postoperative day he was transferred to the specialized department and discharged on the10th day. Discussion
MAPCA is one of the significant factors influencing the final outcome of radical TOF correction [4]. At the early stage, cardiac surgeons did not know much about collaterals and did not treat them when performing radical TOF correction. With the development of medical and computed tomography technologies, cardiac surgeons gradually deepened their knowledge of aortopulmonary collaterals and their treatment became an important part of radical TOF correction [5].
It is logical to try to close all available additional collaterals in open surgery in one step after cardiac TOF correction, but technical difficulties in the form of collateral search, expansion of the operating field and surgery time, in most cases the inability to close all sources of additional blood supply, have led to the development of a staged approach of radical treatment involving interventional technologies. Endovascular techniques have a number of advantages over hard-to-reach surgical areas and are used both before, during, and after open surgery [6].
At the current stage, when the task is to perform simultaneous cardiovascular surgical interventions, a universal hybrid operating room [7], which allows performing open and closed surgery simultaneously, is deployed on the basis of highly specialized clinics. A tour center, we were able to minimize the time factor
between the first and second stages of TOF radical correction with MAPCA, thanks to the functional location of two closely situated multi-targetoperating theatres on one floor of the surgical unit. Most studies on MAPCA treatment have focused on MAPCA occlusion before TOF correction, but MAPCA occlusion before surgical correction could lead to a further decrease in saturation, and therefore the patient required surgical correction immediately after transcatheter closure of MAPCA.
To reduce morbidity and mortality, endovascular MAPCA occlusion or surgical ligation should be performed in patients with a collateral diameter to body weight ratio of at least 0.5 mm/kg. In patients with values of approximately 0.2 to 0.5mm/kg, prolongation of APV should have priority over transcatheter occlusion, and for patients with values below 0.2 mm/ kg no additional treatment is required [8]. In our child's case, this value left 3.5 in the presence of 3.0 mm collaterals. We prioritized the closure of collaterals immediately after TOF correction with the desire not to prolong APV, which would have increased the risks of APV-associated complications.
Conclusion
In our clinical case based on the experience of the world leading clinics, the principle of simultaneous treatment of severe combined pathology of Tetralogy of Fallot with major aortopulmonary collateral arteries was successfully applied. Taking into account the evidence base, our patient belonged to the intermediate group, where single-stage correction of TOF with MAPCA occlusion was performed in 38% of cases. During radical open correction of TOF, the valve complex of the PA trunk was preserved and MAPCA was embolized immediately within several minutes after the first operation, which allowed to minimize postoperative risks and achieve a good clinical result.
Figure 5.
Angiogram. End result after MAPCA embolization: at control angiography, the contrast intensity of pulmonary vascularization was reduced by 100%
References
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2. Miyahara K, Maeda M, Sakurai H, et al. Repair of tetralogy of fallot in an adult; theimportance of preoperative examination for major aortopulmonary collateral arteries. Kyobu Geka. The Japanese Journal of Thoracic Surgery. 2002;55(9):779-783. 7.
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4. McElhinney DB, Reddy VM, Hanley FL. 8. Tetralogy of Fallot with major aortopulmonaryco llaterals:earlytotalrepair.PediatrCardiol.1998Jul-Aug;19(4):289-96.doi: 10.1007/s002469900312. PMID: 9636252.
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M.J., Déry J. Tetralogy of Fallot: Preoperative assessment with MR and CT imaging. Diagn Interv Imaging. 2016 May; 97(5):531-41. doi:10.1016/j.diii.2016.01.009. Sato Y., Ogino H., Hara M., Satake M., Oshima H., Banno T., Mizuno K., Mishima A., Shibamoto Y. Embolization of collateral vessels using mechanically detachable coils inyoung children with congenital heart disease. Cardiovasc Intervent Radiol. 2003 Nov-Dec;26(6):528-33.doi: 10.1007/s00270-003-2723-9. PMID:15061176 Moore J.W., Ing F.F., Drummond D., Berdjis F., Clapp S.K., Grifka R.G., Nihill M.R., Mullins C.E. Transcatheter closure of surgical shunts in patients with congenital heart disease. Am J Cardiol. 2000 Mar 1;85(5):636-40.doi:10.1016/ s0002-9149(99)00824-3.PMID:11078280 Cheng J, Fan C, Tang M, Shu Y, Yang J. Initial Research on Postoperative Management of Tetralogy of Fallot with Major Aortopulmonary Collaterals.Cardiology.2016;134(4):406-10.doi: 10.1159/000445046.
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