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19
II. ХИРУРГИЯ
УДК 616.12-007-0.53.1:6160.89
TETRALOGY OF FALLOT:
DIAGNOSTICS AND SURGICAL TREATMENT.
LITERATURE REVIEW
ABOUT THE ÄUTHORS
Sagatov l.Y. -
Ph.D., Head of Scientific management Department of A.N. Syzganov National Scientific center of Surgery
Sagatov I.Y., Shmonin V.M.
National Scientific Center of Surgery named after A.N. Syzganov, Almaty, Kazakhstan
Keywords
tetralogy of Fallot, radical correction, the results
Abstract
The article presents the current views on diagnostics and surgical treatment of tetralogy of Fallot. The described methods of palliative and radical correction of tetralogy of Fallot, presents an immediate and remote results of surgical treatment. Particular attention is paid to new innovative methods for the restoration of anatomical and hemodynamic disorders in tetralogy of Fallot.
Фалло тетрадасы: диагностикасы мен оталау емк Эдебиет шолуы
АВТОРЛАР ТУРАЛЫ
Сагатов I.E. -
м.^д., А.Н.Cызfaнав ^ты^д^ш YfXO-н ■ГЗЖ менеджментi бeлiмiнiц б^сш^^сы
CaFaTOB I.E., Шмонин В.М.
А.Н. Сыз?анов атында?ы Улттык, ?ылыми хирургия орталы^ы, Алматы, Казахстан
Туйш свздер
Фaлла тeтpaдacы, радикалды ота, нэтижелерi
Ацдатпа
Мaкaлaдa Фaлла тeтpaдacынын диaгнаcтикacы мен атaлay eмiнiц зaмaнayи кeзкapacтapы кeлтipiлгeн. Соны-мен aap, Фaлла тeтpaдacынын пaллиaтивтi жене paдикaлды eмдey еща, xиpypгиялык емнщ жaкын жене aлшaк каpытындылapы кeлтipiлгeн. Фaлла тeтpaдacындaFЫ aнaтамиялык жене гeмадинaмикaлык бYзылыcтapды ^лпыт кeлтipyдiн жaнß иннавaциялык едicтepiнe eperne кецш белнген.
Тетрада Фалло: диагностика и хирургическое лечение. Обзор литературы
ОБ АВТОРАХ
Сагатов И.Е. -
д.м.н., руководитель отдела менеджмента НИР ННЦХим. А.Н. Сызганова
Сагатов И.Е., Шмонин В.М.
Национальный научный центр хирургии им. А.Н. Сызганова, Алматы, Казахстан
Ключевые слова
тeтpaдa Фaлла, paдикaльнaя каppeкция, peзyльтaты
Аннотация
В статье представлены современные взгляды на диагностику и хирургическое лечение тетрады Фалло. Описаны методы паллиативной и радикальной коррекции тетрады Фалло, представлены непосредственные и отдаленные результаты хирургического лечения. Отдельное внимание уделено новым инновационным методам восстановления анатомических и гемодинаимческих нарушений при тетраде Фалло.
Background
Frequency of tetralogy of Fallot (TOF) riches 6-7% among congenital heart disease (CHD) or 0,21 - 0,26 per 1000 newborns. 30% of children with TOF die in their first year of life [10, 17, 20]. Among cyanotic forms of CHD in children older than a year TOF's frequency reaches 50-75%. Amidst the vices, requiring surgical treatment in the chil-drens in early age, TOF takes third place after patent ductus arteriosus (PDA) and ventricular septal defect (VSD) making up 14,9%. TOF includes four anomalies: VSD, obstruction of the outflow of blood from the right ventricle, the location of the aorta above the defect of the interventricular septum (dextraposition), hypertrophy of the right ventricle.
The name itself is historically closely connected with a name of a pathologist from Marseille Fallot E.-L. A., who sistematized observation and described clinical picture and pathological anatomy by highlighting the defect as an independent naso-logical unit in 1888.
The basis of formation of a heart of a child with TOF is a rotation of an arterial cone counterclockwise. As are sults of this phenomenon aortic valve keeps its embryological position and remains to the right of the pulmonary. Such a "right position" of the aortic root leads to the fact that with TOF it seems to be sitting astride the septum. Should be understood, however, that essence of dextraposi-tion of aorta in such cases determined not only by its location above the interventricular septum, but also by rotating the arterial cone counter clockwise. Moreover, rotation of arterial cone counterclockwise at the same time causes anterior and left displacement of the right arterial (pulmonary) cone. These factors explain a certain lengthening of the pulmonary cone and anterior displacement of the aortic valve relative to the atrioventricular.
Rotation of the arterial cone counterclockwise simultaneously leads to the rotation of the septum of the arterial cone, which, along with parallel anterior displacement, does not allow it to connect to the interventricular septum (which explains the presence of VSD in TOF) and the bulbous-ventricular fold. In addition, the anterior displacement of the septum -arterial cone causes a narrowing of the pulmonary cone. Inthatway, rotation of the arterial cone counter clockwise is the main factor affectingto the formation of TOF. This is a separate independent process, not related to the underdevelopment of the arterial trunk, which determines the "uneven division of the bulb" (according to the characteristics of domestic authors) and allows to understand a number of issues that cannot be explained by the concept of "correct bulb of the heart". Upon the main features of cardiac embryogenesis in this defect, it is also
worth noting the "resorption" of the middle portion of the bulbo-ventricular fold, which determines the presence of mitral-aortic fibrous contact [17].
A review of the literature shows variant anomalies are associated as a single anomaly with TOF; coarctation of the aorta, anomalous left coronary artery originates from the pulmonary artery or a coronary fistula to the pulmonary trunk, left pulmonary artery (LPA) sling, or unilateral absence of pulmonary artery. But the association of TOF with LPA sling, ARCAPA, and tracheal bronchus was not described to date in the literature [1].
Diagnostics
Chest X-ray. A typical radiological picture of a patient with TOF is characterized by the following symptoms:
1) depletion of pulmonary blood flow;
2) the "retraction" of the arc of the pulmonary artery along the left contour of the heart in a direct projection;
3) the characteristic configuration of the heart in the form of a "wooden shoe" due to the top rounded and raised above the diaphragm;
4) an increase and hypertrophy of the right heart.
In patients with TOF after previously performed
surgical interventions, in particular systemic pulmonary anastomoses, there might be seen asymmetry of the pulmonary pattern due to its moderate amplification in the basal zone from the side of the operation may be observed. In rare cases, after the operation of the "central" systemic pulmonary anastomoses and the development of such a rare complication as pulmonary hypertension in a child, the pulmonary pattern may be "chopped off" on the periphery.The pale form of TOF is characterized by radiological signs of normovolemia of the pulmonary circulation or even by an increase in the pulmonary pattern in arterial type. The presence of major aorto-pulmonary collateral arteries (MAPCA) in a patient with TOF leads to the formation of a pulmonary pattern represented by narrow convoluted vessels.
At the present stage of the development of diagnostics, using two-dimensional echocardiographic and Doppler echocardiographic studies, TOF can be fully verified with a detailed assessment of each of the anatomical components of the defect.
The use of echocardiography in the diagnostic of TOF algorithm allows:
- to detail the components and level of stenosis of the excretory tract of the right ventricle (subvalvular, supravalvular);
- to determine the size and evaluate the degree of hypoplasia of the valve ring of the pulmonary artery, the trunk of the pulmonary
artery itself and the initial departments of the branches of the pulmonary artery;
- visualize and describe the localization and size of the cerebrospinal fluid;
- assess the degree of aortic dextraposition;
- to identify the presence of mitral-aortic contact, which is a decisive moment in the differential diagnosis between TOF and double passage of vessels from the right ventricle.
An important task in conducting echocardiography in a patient with TOF is to determine the degree of balance of the ventricles of the heart by assessing their linear dimensions (final systolic and diastolic volume).For surgeons, such an echocardiography indicator as indexed (correlated with the patient's body surface area) final diastolic volume of the left ventricle has a great importance, allowing to judge the degree of development of the ventricle and its functional capabilities.So, with the value of this indicator less than 35 ml/m2, the condition of the ventricle is regarded as anatomical hypoplasia, which determines the further choice of the method of surgical treatment - the implementation as the first stage of various options for systemic-pulmonary anastomoses.
The use of two-dimensional Doppler echocardiography when examining a patient with TOF makes it possible:
- to assess the degree of stenosis of prostate cancer by determining the gradient of systolic pressure between the right ventricle and pulmonary artery, which, as a rule, reaches sufficiently large values (of the order of 100 mm Hg);
- identify the presence of antegrade blood flow through the valve of the pulmonary artery;
- to detect additional systolodiastolic flows in the lumen of the pulmonary artery in the presence of PDA or MAPCA.
In addition to determining the main anatomical components of Echocardiography defect, the study can help in visualizing concomitant intracardiac abnormalities, such as atrial communication, additional defects of the interventricular septum, insufficiency of the atrioventricular valves, abnormal location of the initial parts of the coronary arteries crossing the right ventricular outflow tract (RVOT), and others.
In case of unsatisfactory visualization of the structures of the heart and blood vessels during echocardiography, as well as to identify additional abnormalities (atresia of one of the pulmonary arteries, deformity of branches due to previous palliative operations, suspected peripheral pulmonary stenosis), the cardiac cavity catheterization and angiocardiography are used in the diagnostic algorithm of TOF.
Cardiac catheterization and angiocardiography. These research methods are fundamental to formulate the final clinical diagnosis and determine the type and extent of surgical intervention.
The use of angiocardiography with angiomor-phometry in patients with TOF allows you to:
- study in detail the levels of narrowing (subval-vular, at the level of the pulmonary artery ring, supravalvular) and the degree of narrowing of the excretory tract of the right ventricle;
- identify concomitant anomalies, such as coarctation of the aorta, abnormal drainage of the pulmonary veins, defect in the aortopul-monary septum, double aortic arch, etc .;
- diagnose the presence of messages between the aorta and the trunk of the pulmonary artery;
- indirectly assess the size of the left ventricle;
- determine the degree of development of the pulmonary artery system with angiomorpho-metric calculations (calculation of the Nakata and McGoon indices, as well as the ratio of the diameters of different sections of the pulmonary arterial tree to the diameter of the descending aorta at the diaphragm).
Angiocardiography studies in patients with TOF are performed:
- right ventriculography in direct and lateral projection;
- pulmonary arteriography in direct and axial projection;
- left ventriculography;
- aortography.
Right ventriculography in direct and lateral projection with TOF allows to:
- to visualize multicomponental (most cases) stenosis of RVOT on account of subvalve, valve and supravalve components;
- in directly estimate the level of development of pulmonary arterial bed;
- identify pronounced regurgitation on the venous atrioventricular valve;
- measure the pressure in the right ventricle and pulmonary artery and determine the systolic pressure gradient between the right ventricle and pulmonary artery, it is calculated by pressure curves, obtained while conducting a catheter from the right ventricle to the pulmonary artery.
When performing aortography in patients with TOF, the following are possible:
- diagnosis of the presence of extracardiac systemic pulmonary shunts (open ductus arterio-sus, aorto-pulmonary septal defect, previously performed systemic pulmonary anastomoses);
- assessment of the number and degree of development of the large aorto-pulmonary collaterals;
- identification of pathology of the aortic arch (coarctation of the aorta, vascular rings);
- determination of the presence and course of coronary arteries crossing the RVOT.
The left ventriculography in the diagnosis of TOF helps to assess the size and development of the left ventricle, the degree of aortic dextraposi-tion, confirm the presence of mitral-aortic contact, pulmonary trunk and pulmonary arteries. For a an-giocardiometry, an analysis of angiocardiograms is carried out under the condition of a clear visibility of all segments of the pulmonary artery in frames in dynamics in phases from the final diastole to the final systole. Since the valve ring, pulmonary trunk and pulmonary arteries in the systole phase have maximum dimensions, the calculations are carried out in this phase of the cardiac cycle in direct projection, adjusted for magnification.
When conducting angiomorphometric calculations in patients with TOF, the following indicators are evaluated: pulmonary arterial index (Nakata index), McGoon index.The Nakata Index was developed and proposed with the aim of indirectly evaluating blood flow in each lung in patients with CHD with reduced pulmonary blood flow [15]. This technique involves the calculation of the pulmonary arterial index based on the ratio of the sum of the cross-sectional areas of each branch of the pulmonary artery at the level of the discharge of the first upper lobar branch to the surface area of the patient's body. Based on the obtained data, it is possible to determine the passage of a sufficient amount of blood to the peripheral sections of the pulmonary arterial bed, which indirectly confirms the absence of an obstacle at this level after performing a radical correction of TOF. According to the authors who proposed the calculation of this indicator, the value of the pulmonary arterial index above 150 mm2/m2 is a criterion for the possibility of performing radical surgery in patients with TOF, with lower values it is recommended to perform palliative interventions. Normally, the value of this indicator is on average 300 + 30 mm2/m2.
The McGoon index is the ratio of the sum of the diameters of the mouths of the branches of the pulmonary artery (B1+C1) to the diameter of the descending aorta (dAo) and reflects the degree of development of the central departments of the main branches of the pulmonary artery. Normally, the value (B1+C1)/dAo is at least 1,5. A lower value of this indicator indicates the presence of local wellhead stenosis of the main branches of the pulmonary artery, requiring reconstruction with a radical correction of TOF.
The ratio of the diameters of different sections of the pulmonary arterial tree with the diameter of the descending aorta at the diaphragm level in patients with CHD is also an important angiomorpho-
metric parameter characterizing the presence of local narrowing of various sections of the pulmonary arterial bed, requiring elimination during surgical treatment of the defect.
Angiocardiographic examination with angiomor-phometry has a great importance for determining the variant anatomy of the defect, the volume of surgical intervention and predicting the immediate and long-term results of the operation.
Surgical treatment
There is no doubt for the need of surgical care for patients with tetralogy of Fallot.Over the course of more than 60 years of surgical history of the treatment of the disease, various options for palliative interventions have been developed and proposed, including endovascular procedures, as well as a technique for radical correction of TOF [1, 2, 18, 21, 22, 26, 27]. The choice of one or another method of surgical correction is determined by:
1) symptomatic course of vice;
2) age of the patient;
3) anatomical form of the defect (the degree of development of the pulmonary arterial bed and left ventricle, the presence of MAPCA);
4) degree of an arterial hypoxemia.
Palliative interventions
Palliative interventions performed according to a closed method should primarily include various options for systemic-pulmonary anastomoses:
- classic Blalok-Taussig anastomosis (using the subclavian artery sewn into the branch of the pulmonary artery);
- Waterstone-Cooley anastomosis - between the right pulmonary artery and the ascending aorta;
- Potts anastomosis - between the descending aorta and the left pulmonary artery;
- anastomosis between the pulmonary and subclavian arteries using a synthetic prosthesis (modified Blalok-Taussig anastomosis);
- central anastomosis between the ascending aorta and the trunk of the pulmonary artery using a prosthesis.
Indications for performing such operations are:
- symptomatic flow of the defect with the heavy dyspnea-cyanotic sezarius of anamnesis;
- the heavy clinical station of a patient, due to severe arterial hypoxemia (arterial blood oxygen saturation of 75%);
- the presence of echocardiographically confirmed anatomical hypoplasia of the left ventricle (the value of the indexed EDV of the left ventricular (LV)) is less than 35 ml/m2 in combination with the small size of the mitral valve fibrous ring);
- long-term administration of b-blockers by the patient.
The creation of systemic-pulmonary anastomoses, as a rule, leads to an improvement in the clinical condition of the child due to a decrease in the degree of arterial hypoxemia, makes it possible to prepare the LV for subsequent radical correction (RC) of TOF due to the increased functional load on it due to the increased return of blood to the left heart, and may also lead to an increase in the pulmonary arterial bed on the side of the operation performed.
Another type of palliative intervention performed according to a closed procedure is various endovascular procedures. It includes:
1) transluminal balloon valvuloplasty of pulmonary valvular stenosis in TOF (with dominance of the valve component of stenosis of the excretory part of the right ventricle) [16];
2) transluminal balloon angioplasty and stenting of the central and peripheral departments of the branches of the pulmonary artery (in the presence of hemodynamically significant local narrowing of the pulmonary arterial bed);
3) Embolization of the large aorto-pulmonary collaterals, which are an additional source of blood flow to the pulmonary circulation. As a palliative, some authors use stenting of RVOT. A significant increase in blood saturation was noted. Repeated echocardiography before surgery showed a statistically significant increase in the size of the right and left pulmonary arteries, as well as an altered ratio of McGoon (p<0,05). RVOT stenting is a safe and beneficial option for patients with TF with surgical risk factors or adverse anatomy. By increasing pulmonary blood flow, improving blood saturation and providing pulmonary artery growth, this method is a bridge for the surgical repair of the defect [3, 23].
4) Some authors suggest permanent electrical stimulation of the right ventricle in patients after radical correction of TOF. They note that chronic apical stimulation of the RV after TOF correction leads to an improvement in the clinical status with preservation of the systolic function of the pancreas and prevention of progressive prolongation of the QRS segment. Therefore, pacing may be useful in patients in the postoperative period after TOF correction, in whom there are signs of RV insufficiency [4, 8, 9, 14].
Radical correction of tetralogy of Fallot
With a favorable anatomical variant of TOF, primary radical correction of the defect is possible. The main conditions for implementing this are:
- symptomatic course of the defect;
- patient's age more than 3 months;
- absence of LV hypoplasia (index of indexed EDV LV of more than 35 ml/m2);
- satisfactory development of the pulmonary arterial bed (Nakata index more than 200 mm2/
m2);
- moderate manifestations of arterial hypoxemia (arterial blood oxygen saturation of more than 75%);
- lack of pronounced, hemodynamically significant MAPCA;
- lack of a long history of b-blockers in anamnesis.
The operation of the RC TOF is performed in the conditions of cardiopulmonary bypass, hypothermia and combined pharmacological cold cardiople-gia and includes three main stages:
1) resection of infundibular stenosis;
2) plastic VSD;
3) reconstruction of RVOT.
The first stage is the resection of infundibular stenosis. In 90-95% of cases with TF, there is a need to expand the output section of the right ventricle and the trunk of the pulmonary artery, in connection with which the longitudinal right venntricu-lotomy is indicated. The pancreas is opened in the excretory section in the avascular zone by a longitudinal section.
After ventriculotomy, the infundibular stenosis is revised, the degree of displacement and hypertrophy of the conical septum is assessed. Then proceed to the dissection and resection of the muscle bundles of the hypertrophic conical septum. First, the parietal pedicle of the conical septum is widely dissected, avoiding damage to the valves of the aortic valve. Further, the very structure of the conical septum is partially resected. Where in, care should be taken, since damage to the septal branches of the left coronary artery, as well as perforation of the interventricular septum itself, is possible.
Valvular stenosis of the pulmonary artery is eliminated by dissection of fused cusps along the commissures or by partial edge resection of the cusps during their severe deformation, after which the pulmonary valve ring should pass a metal bouge of a calculated diameter corresponding to the surface of the patient's body. However, quite often with TF, there is hypoplasia of the ring and trunk of the pulmonary artery, in connection with which the ven-triculotomic incision is extended through the ring of the pulmonary artery to its trunk until bifurcation and the sizes of the branches of the pulmonary artery are estimated using metal bougie of the calculated diameter.
The second stage is the closure of the VSD. Access to the VSD during plastic surgery is based
on the anatomical location of the defect. With TOF, the most common non-restrictive subaortic VSD cancer, in connection with which the plastic defect is performed through atrial access. The subarte-rial location of the cerebrospinal fluid is much less common, and its closure is done through extraven-triculotomy. Xenopericardium or synthetic material is used as a patch for plastic surgery of VSD. When determining the size of a patch for VSD, focus should be set on the size of the aortic root and the defect itself, since the size of the patch should correspond to the diameter of the aortic root and slightly exceed the size of the defect. Ficcation of the patch on sides of the defect carried out by continuous wrapping or separate U-shaped seams on gaskets in compliance with those anatomical landmarks, which were mentioned earlier. One of the features of stitching on to the patch is the presence one additional ficsating U-shaped seam behind the base of the septal valve of the tricuspid valve.
The third stage, RC of TOF, includes reconstruction of RVOT, which is carried out under control of metal meter for a given body surface area.In most cases, transannular plastic surgery of the prostate gland and the trunk of the pulmonary artery is performed using a xenopericardial patch with a monostable. As studies showed, monostable locking function is important enough in soon postoperative period after RC of TOF, since, acting as a valve of the pulmonary artery, it prevents the volume over-
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