Nghiên cứu đặc điểm lâm sàng, cận lâm sàng, siêu âm doppler tim ở bệnh nhân KNT bán phần trước và sau phẫu thuật tt tiếng ang

MINISTRY OF EDUCATION MINISTRY OF DEFENSE AND TRAINING 108 INSTITUTE OF CLINICAL MEDICAL AND PHARMACEUTICAL SCIENCES TRAN THI AN STUDY ON CLINICAL, SUBCLINICAL, ECHOCARDIOGRAPHIC CHARACTERISTICS OF PATIENTS WITH PARTIAL ATRIOVENTRICULAR SEPTAL DEFECT BEFORE AND AFTER SURGERY Specialized: Internal Cardiology Code: 62.72.01.41 SUMMARY OF DOCTORAL DISSERTATION Ha Noi – 2019 THIS DISSERTATION WAS DONE AT 108 INSTITUTE OF CLINICAL MEDICAL AND PHARMACEUTICAL SCIENCES Science supervisor: Associate Professor, PhD: Pham Nguyen Son Reviewer 1: Associate Professor, PhD Pham Thi Hong Thi Reviewer 2: Associate Professor, PhD Hoang Dinh Anh Reviewer 3: Associate Professor, PhD Luong Cong Thuc The dissertation will be defended in front of the Council Evaluation at: The dissertation can be found at library of: Vietnam National Library 108 Institute of clinica l medical and pharmaceutical sciences INTRODUCTION OF THE DISSERTATIONPREAMBLE AVSD (atrioventricular septal defect) is an anomal characterized by a lack of atrioventricular septal wall with a variety of abnormalities of the atrioventricular valves The cause of this abnormality is the incomplete connection of the endothelium during pregnancy.AVSD accounts for to 3–5% of CHD (congenital heart defects), and 60% of these cases are partial AVSD The AVSD repair surgery was first performed in 1951 by Clarence Dennis at the University of Minnesota and cardiopulmonary technology was also first applied in the world.There are many categories of AVSD, but currently AVSD is classified into two groups: complete and partial form The appropriate time for surgical treatment as well as long-term results are issues that have been interested and studied by many authors around the world The rate of reoperation is still high of 10-25%, depending on each the center, mainly due to the progression of MR (mitral valve regurgitation) or LVOTO (left ventricular ouflow tract obstruction) Therefore, long-term follow-up after surgerywith echocardiography is a mandatory indication for patients with AVSD There are many major cardiac surgery centers performed partial AVSD surgery in Vietnam however there have not been yet many general studies on the diagnosis, the diagnostic means, the role of echocardiography in diagnosis, prognosis and indications for surgery, treatment methods as well as preoperative characteristics affecting treatment results, changes in cardiac morphology and function after surgery of Vietnamese patients Therefore, we performed the study "Study on clinical, subclinica l and echocardiographic characteristis of patients with partial AVSD before and after the surgery" Objectives of the study a Investigate the clinical, subclinical characteristics and Doppler echocardiography of patients with partial AVSD b Evaluate clinical, subclinical and morphologica l, fuctional cardiac changes after surgery in patients with partial AVSD Scientific and practical significance and new contributions of the study This study is a significant scientific and practical research, provides new contributions to the cardiovascular profession in general and to echocardiography in particular: – This study gives a relatively comprehensive view of partial AVSD in Vietnamese in the following aspects: + Clinical: the main symptoms are dyspnea (NYHA II 56,7%), systolic murmur of MR and TR (88.1% and 53.7%, respectively) + Chest X-ray: increased cardiothoracic ratio and increased pulmonary circulation suggestive of left to right shunt flow + ECG: there are some typical signs such as left axis (62.7%), incomplete right bundle branch block (67.2%) + Echocardiography: characterized by the presence of the primum ASD (100%) in combination with cleft of anterior mitral valve (97%) The increase in pulmonary pressure was proportional to the diameter of the ASD and the degree of pulmonary pressure was closely related to the time of mechanical ventilation after surgery The percentage of moderate to severe mitral valve rergurgitation was 86.6% and that ofmoderate to severe tricuspid valve regurgitation was 79,1% The degree of valve regurgitation is proportional to number of valve repair techniques – The study also showed that the efficacy and safety of pAVSD repair surgery vary due to patient’s age, weight as well as the generalcondition.The efficacy and safety of the surgery revealed through the improvement of clinical indexes, the assessment of morphology and cardiac function by echocardiography (decreased pulmonary pressure, decreased MR grade, decreased TR grade, preserved systolic function after surgery, reduce the diameter of RV ) Transthoracic echocardiography is a simple, inexpensive, easy-to-use diagnostic tool to evaluate treatment results and longterm follow-up The layout of the disse rtation – The dissertation has 136 pages including sections: Introduction (3 pages), chapter I: Overview (33 pages), chapter II: Objects and research methods (26 pages), chapter III: Results (39 pages), Chapter IV: Discussion (32 pages), Conclusion (2 pages), Recommendations (1 page) – The dissertation has 52 tables, charts, 31 pictures, diagrams Use 123 references (20 Vietnamese documents, 97 English documents, French documents) CHAPTER I OVERVIEW 1.1 Basic knowledge about partial AVSD 1.1.1 History of research and embryology, anatomical abnormalities of partial AVSD In 1846, AVSD was first described by Peacock, the lesion identification was incomplete atrial and ventricular septal wall In 1875, Rokitansky was the one who used the term "complete" and "partial" to describe this pathology The anatomical standard of partial AVSD is primum ASD and cleft of anterior leaf mitral valve (few cases not have) Partial AVSD has separated mitral valve and tricuspide valve with separated and complete valve rings 1.1.3 Pathophysiology of partial AVSD Because of anatomical abnormalities, many patients with AVSD have one or more of the following disorders: shunt via ASD, left and right atrioventricular valve regurgitation Without surgery, about 15% of untreated patients will develop pulmonary vascular disease and atrial fibrillation in adolescence 1.1.4 Diagnosis of partial AVSD 1.1.4.1 Diagnosis of partial AVSD The clinical manifestations of the partial AVSD change and are related to hemodynamic changes Clinical symptoms often appear late with the symptoms such as shortness of breath, palpitations, and fatigue Physical signs: a systolic murmur due to increased flow through the pulmonary valve, the seconde sound of pulmonary valve is loud and splited (prolonging the pulmonary component of the T2) In addition, the systolic murmur of MR or TR can be heard 1.1.4.2 Paraclinical partial AVSD Chest X ray Right ventricular and pulmonary arterylobes are usuallydilated and there is signs of increased pulmonary perfusion ECG Classically, the ECG has a left axis with angles from to – 90 Signs of right ventricular hypertrophy with rsR'in the precordial leads Left precordial leads or qRs or qRS reflect the degree of right ventricular hypertrophy Right bundle branch block is also common Doppler echocardiography Echocardiography allows to identify and classify the AVSD morphology In addition to assess morphological changes, echocardiography also evaluates changes in hemodynamic adn functional parameters Atrioventricular valve morphology: mitral valve and tricuspide valve are on the same plane, mitral valve leaves and tricuspide leaves cling to the tip of the ventricular septum, with separate atrioventricular valve holes Cleft of atrioventricular valve: the subcostal view, the parasternal short axis view and apical four-chamber view provide a clear view of the atrioventricular valves Cleft of anterior mitral valve directly toward to the inlet ventricular septum Variation in the left ventricular outlet:the anteriorly aortic shift, not “wedged” between the MV and TV loop, causes the aorta anterior to the atrioventricular junction which may cause LVOTO Characteristics of the primum ASD: Focal are seen extending to the atrioventricular valve, no atrioventricular segment, size varies but often is wide Several other combined characteristics: The extension of the LVOT with the ratio of outlet/inlet > Counter-clockwise displacement of the MV chordare The balance/imbalance of the two ventricles and the two atriums There might have inlet VSD without shunt or trivial flow And some other abnormalities can be seen (ventricular dysplasia, stenosis of the RVOT) Hemodynamic and functional parameters Echocardiographic parameters include: left ventricular size and function, right ventricular size, degree of MR, TR, ASD shunt, PAP and pulmonary flow (Qp), aortic flow (Qs) The above parameters can be assessed simply and accurately by Doppler echocardiography and can be repeated many times, safely and inexpensively In the world, the basic knowledge about the disease as well as the treatment of surgery have been studied for a long time In 1954, Lillehei and co-workers successfully carried out the first partial AVSD repair surgery with the good results The study of Hani K Najm collected data of 180 childrens who had surgery to repair of partial AVSD from 7/1982 to 12/1996 in Canada, the average age was 3.6 years (1 month - 16.4 years) The short term death rate is 1.6% Other complications: atrial arrhythmia, transient atrioventricular block soon after surgery The average postoperative follow up time with echocardiography was 4.6 ± 3.6 years (2 months - 13.7 years) showed that ASD residual shunts accounted for 1%, mild (or no), moderate and severe MR were 85%, 14% and 1% respectively Research of Krupickova et al (2000 – 2015) on 51 symptomatic patients with partial and transitional AVSD with mean age of 179 days (0 - 357 days), of which 31% of patients had severe valve anomalies The in hospital death rate was 5.9%, 22% of patients had to undergo re-surgery (4 days - 5.1 years), patient had to replace mechanical valve Multivariate analysis showed that unfavorable anatomical status of MV is an independent risk factor for reoperation MV Besides, the study of Barnett and colleagues on adult patients (from 13 - 65 years old, the average age is 48 years old), with a Qp/Qs ratio of 3.9 (from 2.4 to 4.4) showed no deaths during hospital stay, improved heart failure through NYHA postoperative evaluation of patients This suggests the safety and the effect of partial AVSD surgeryand should be recommended for all patients to prevent changes in morphology and cardiac function 1.2.2 Studies in Vietnam In Vietnam, there is a lot of difficulty in early diagnosistherefore many patients come for treatment at high age compared to the recommended age of operation Le Thi Thanh Xuan and Nguyen Tan Vien published research results on ehocardiography of morphology and hemodynamics in children with AVSD The results showed that the complete AVSD accounted for 71.6%, the rest was partial AVSD; 44% had atrioventricular valve regurgitation, of which none had severe atrioventricular valve regurgitation, 48% had pulmonary hypertesion, 11% had other combined heart defects Research of Bui Duc Phu and Le Ba Minh Du at Hue Central Hospital on surgical results of 17 cases of AVSD from 1/2000 to 6/2005 There are no death related surgery, the atrioventricular valve regurgitation improved Most recently (in 2015), Dao Quang Vinh conducted a study to evaluate the results of partial AVSD surgery The study included 89 patients, the early and first 6-month mortality rate accounted for 1.1%, 1.1% severe MR need to be reoperated The severity of MR decreased and heart failure improved CHAPTER SUBJECTS AND METHODS OF THE STUDY 2.1 Object of research Including 67 patients, diagnosed with partial AVSD and had indication for operation at Hanoi Heart Hospital The period was from January 2011 to December 2014  Inclusion criteria: Patients were recruited when the following criteria were met: a The patient was diagnosed of partial AVSD based on echocardiography results in Ha Noi Heart Hospital: + Primum atrial septal atrial (or unique atrial form) + MV and TV are separate and located on the same plane + There are cleft(s) of anterior MV leaflet (few not have) b The patient was indicated surgery and had surgery to repair partial AVSD at Hanoi Heart Hospital c Patients agreed to participate in the study  Exclusion criteria: a The patient was accompanied by another complex CHD b Partial AVSD with manifestations of Eisenmenger syndrome (patients with frequent cyanosis, echocardiogrphy showing bidirectional or right to left shunt mainly, cardiac catheterization with pulmonary resistance > 10 Wood) c The patient was operated d Patients with severe medical illness accompanied e Patient and family members did not agree to participate in the study f Patients did not come for follow-up visits or later than weeks  Sample size se lection method: Due to the low proportion of patients with partial AVSD, we selected a convenient method 2.2 Research methodology 2.2.2 Research design: prospective 2.2.3 Steps to conduct research: We conducted data on patient's medical history, clinica l examination, subclinical tests, etc according to the pre-designed study sample The patient evaluation follow up times included: before surgery (time M-1), after surgery and before 11 Calculate the value of echocardiography in diagnosis: Diagnosis of surgery Total (+) (–) Diagnosis of echocardiography Total (+) a c a+c (–) b d b+d a+b c+d a +b+c+d Sensitivity = a/(a+b); Specificity = d/(c+d) Positive predictive value = a / (a + c); Negative predictive value = d / (b + d) The results were presented in tables and charts 2.3 Research ethics The study did not violate ethical regulations when studying biomedical research Before recruited in this study, patients were fully explained about the purpose, requirements and content of the study After that, those patients who voluntarily participated would be included in the research, had full corrective surgery when indicated and consulted with the whole hospital, the report of the consultation and the patients agree to surgery The patient's condition and other personal information is kept confidential The study was approved by the hospital-level ethics committee Do not take patients to test unrecognized treatments The purpose of the study is to protect and improve public health 12 RESEARCH CHART 13 CHAPTER RESEARCH RESULTS 3.1 General characteristics of the study patient group The median age was 192 months (16 years), the youngest of months, the oldest of 64 years We divided patients into age groups, from years old and younger (22.4%), from to years old (14.9%), from to 16 years old (13.4%) and over 16 years old (49,3%) The distribution of patients by gender male/ female is 46.3% and 53.7% 3.2 Clinical and subclinical characteristics of the subjects 3.2.1 Clinical characteristics of research subjects – Reasons for detecting the disease: various, dyspnea accounted for 22.4% and other reasons 29.9% – Functional characteristics: the most common symptom is shortness of breath with 56.7% of patients at NYHA II, 1.5% at NYHA III, no patients at NYHA IV – Physical characteristics:the splitted S2 at pulmonary valve location were 46.3% and 23.9%, respectively, systolic murmur of MR and TR were 88.1% and 53.7% respectively – Patients with Down syndrome were 7.5% – Children get often recurrent bronchitis and delayed weight (40% and 26.7% in children under years, respectively) 3.2.2 Subclinical characteristics of research subjects 3.2.2.1 Some subclinical characteristics of the research subjects Chest X-ray: 94.0% with cardiothoracic ratio > 50%, 49.3% with signs of increased pulmonary circulation ECG: – Some basic parameters: sinus rhythm was 91%, patients with atrial fibrillation (7.5%) and patient with BAV III (1.5%) ECG 14 axis was mainly left axis (62.7%) 01 case of WPW (1.5%), no other arrhythmias – Some characteristics of conduction system: incomplete right bundle branch block was primary (67.2%) BAV I was also common (34.3%) 3.2.2.2 Some characteristics of the Doppler echocardiography of the research subjects Some basic parameters – The majority of patients had good systolic left ventricular (EF) function before surgery and there was no difference between age groups There were 16.4% of cases with left ventricular dilatation, but up to 92.5% with right ventricular dilatation – Heart valve anatomy characteristics: 97% with "cleft" on anterior leaf of MV 89.6% of patients had balanced papillary muscle (10.4% had muscle columns but unbalanced) 22.4% had "cleft" on septal leaf of TV 22.4% had dysplasia TV – Valve regurgitation characteristics: 65,7% were severe regurgitation of mitral valve, and 47,8% were severe regurgitation of tricuspid valve – Heart septal perforation: large primium ASD (100%) with median diameter of 22 mm, 94% left-right shunt, 6% had bidirectional shunt but not often – Some hemodynamic characteristics: 13.6% of patients did not have pre-operated HTAP, the severity of HTAP was: 18.2% mild, 39.4% moderate and 28.8% severe The highest PAP group (≥ 60 mmHg) was the oldest (median is 20 years old) 3.3 Clinical, subclinical and morphological changes, cardiac function after surgery in the study patient group 3.3.1 Clinical changes after surgery 15 The change of functional signs: 58,2% patients was dyspnea before surgery with NYHA II, III however, 100% patients had NYH I at the follow-up time of month, months, months Changes of physical signs – The rate of systolic murmur of MR and TR postop were much lower than preop (before surgery and after months, MR murmur reduced from88.1% to 15.4%, TR murmur from 53.7% to 0%) – A strong and splited T2 sound is almost non-existent in patients after surgery 3.3.2 Subclinical changes after surgery 3.3.2.1 Changes of some subclinical characteristics Chest x-ray: 94% patients hadcardiothoracicratio ≥ 50% before surgery which reduced to 32.7% one month after surgery, signs of increased pulmonary circulation decreased from 49.4% to 1.9% ECG: There was no significant change with parameters such as heart rate pattern, ECG axis, bundle branch block, atrioventricular block 3.3.2.2 Changes in echocardiography characteristics aftersurgery Some basic parameters: increased LV end – diastolic diameter, in contrast, decreased RV end – diastolic diameter compared to before surgery and no significant change in the EF index Changes in regurgitation of atrioventricular valve: there was a significant improvement in the degree of MR and TR over time 16 Table 3.31 The degree of ventricular valve regurgitation over time M-1 (1) Mo (2) M1 (3) M3 (4) M6 (5) n (%) n (%) n (%) n (%) n (%) Characteristics p Mitral regurgitation No - Mild (13,4) 39 (58,2) 36 (69,2) 29 (70,7) 29 (74,4) Moderate 14 (20,9) 25 (37,3) 15 (28,9) (22,0) (17,9) Severe 44 (65,7) (4,5) (1,9) (7,3) (7,7) p5-1: