scholarly journals Assessment of pulmonary arteries in patients with tetralogy of Fallot

2018 ◽  
Vol 22 (4) ◽  
pp. 48
Author(s):  
I. A. Kozyrev ◽  
A. A. Morozov ◽  
A. K. Latypov ◽  
A. V. Vasilets ◽  
N. A. Kotin ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Tetralogy Of Fallot ◽  
Surgical Management ◽  
Conflict Of Interest ◽  
Pulmonary Arteries ◽  
Z Score ◽  
Complete Repair ◽  
Z Scores ◽  
Left And Right

<p><strong>Aim.</strong> The study aimed to evaluate the size of pulmonary arteries in patients with tetralogy of Fallot during preoperative and intraoperative stages and to find out the influence of preoperative sizes on the surgical management.<strong></strong></p><p><strong>Methods. </strong>Between January 2015 and May 2018, 50 patients with tetralogy of Fallot younger seven months underwent primary complete or palliative repair. The data obtained from patients' files and include echocardiography, computed tomography and surgical reports. Dimensions of right and left pulmonary arteries were analyzed by calculating the Z-score and Nakata indices.<strong></strong></p><p><strong>Results.</strong> Z-scores of the left and right pulmonary arteries were significantly smaller by echocardiography comparing to computed tomography (p&lt;0.01). Z-scores of left and right pulmonary arteries measuring by computed tomography were also smaller than the size measured during surgery (p=0.05). Nakata index according to computed tomography was significantly smaller (p&lt;0.05) than this index calculated from surgical reports.<strong></strong></p><p><strong>Conclusion.</strong> In patients with tetralogy of Fallot, the size of pulmonary arteries according to the visualization methods is smaller than size measured during surgery. Intraoperative sizing of pulmonary arteries could influence surgical management, expanding indications for complete repair in some patients.</p><p>Received 6 July 2018. Revised 24 August 2018. Accepted 28 August 2018.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Author declare no conflict of interest.</p><p><strong>Acknowledgment</strong><br />We would like to express our gratitude to Ya.A. Yermolenko for Figure 1 used in the article.</p>

scholarly journals The immediate results of valve-sparing complete repair of tetralogy of Fallot

2019 ◽  
Vol 23 (1) ◽  
pp. 9
Author(s):  
I. A. Kozyrev ◽  
A. A. Morozov ◽  
E. V. Grekhov ◽  
I. A. Averkin ◽  
M. L. Gordeev ◽  
...  
Keyword(s):  
Cardiopulmonary Bypass ◽  
Tetralogy Of Fallot ◽  
Conflict Of Interest ◽  
Pulmonary Valve ◽  
Early Postoperative Period ◽  
Cardiopulmonary Bypass Time ◽  
Complete Repair ◽  
Z Scores ◽  
Valve Sparing ◽  
Repair Group

<p><strong>Aim.</strong> The present study aimed to evaluate the immediate results after the primary valve-sparing complete repair of tetralogy of Fallot.<br /><strong>Methods.</strong> The study included patients with tetralogy of Fallot who were aged &lt;6 months and who underwent primary complete repair between January 2017 and December 2018. According to the surgical approach, the patients were divided into two groups: valve-sparing repair and transannular plasty. Data were obtained from medical records and included echocardiography findings and surgical information. The dimensions of the pulmonary valve were assessed using Z-scores.<br /><strong>Results.</strong> In total, 38 patients were included in this study. Among these, 25 (66%) patients were included in the valve-sparing repair group. The valve-sparing repair group had lower cardiopulmonary bypass time, epinephrine support duration, and pulmonary valve Z-scores than the transannular plasty group. Additionally, the valve-sparing repair group had a significantly higher postoperative pulmonary valve Z-score than the preoperative score. <br /><strong>Conclusion.</strong> Valve-sparing complete repair of tetralogy of Fallot is possible in the majority of patients. Pulmonary valve preservation reduces cardiopulmonary bypass time and provides a more stable early postoperative period by reducing the epinephrine support duration.</p><p>Received 13 March 2019. Revised 24 March 2019. Accepted 8 April 2019.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p>

Abstract 16503: Comparison of Management Strategies for the Neonate With Symptomatic Tetralogy of Fallot and Hypoplastic Branch Pulmonary Arteries: Results From the Congenital Catheterization Research Collaborative

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Christopher J Petit ◽  
Mark Law ◽  
Andrew C Glatz ◽  
Paul Chai ◽  
Jennifer Romano ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Reference Group ◽  
Primary Repair ◽  
Management Strategies ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Treatment Strategies ◽  
Z Score ◽  
Risk Management Strategies ◽  
Secondary Outcomes

Introduction: Symptomatic neonates with tetralogy of Fallot (sTOF) and hypoplastic branch pulmonary arteries (hPA) are at high risk. Management strategies include staged repair (SR) - initial palliation (IP) followed by later complete repair (CR) - or primary repair (PR). A balanced comparison of these approaches is needed in the sTOF neonate with hPA. Methods: Consecutive neonates with sTOF and hPA - defined as one hPA z-score <-2.0 with contralateral z-score <0 - who underwent SR or PR from 2005-17 were retrospectively reviewed by the Congenital Catheterization Research Collaborative. Primary outcome was mortality and secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital, ICU and surgical morbidities, and reintervention. Outcomes were compared between treatment strategies using propensity score adjustment (PSA) to account for baseline differences between groups (PR=reference group). Results: The cohort comprised 188 neonates including 121 SR (IP: 29 transcatheter; 54 surgery) and 67 PR patients. Presence of pulmonary atresia, lower gestational age and birthweight were more common in the SR cohort. Median right and left hPA z-scores were -2.19 (IQR -2.65, -1.92) and -2.33 (-2.84, -1.99), respectively. There were significant differences in treatment strategy across centers (p<0.01). Overall survival was similar between groups, before (p=0.33) and after PSA (HR 1.91, 0.48-7.7, p=0.36). Reintervention was more common in the SR group overall (HR 1.7, 1.03-2.7, p=0.04), but there was no difference after definitive repair (HR 1.21, 0.71-2.0, p=0.49). Secondary outcomes are shown in Table 1. Conclusions: In this multicenter comparison of SR or PR for management of neonates with sTOF and hPA, after PSA, no difference in mortality was found. Neonatal morbidities largely favored the SR group, but cumulative morbidities and reintervention favored the PR group. Further study is needed to determine late impact of these early advantages to SR.

Surgical management of complete atrioventricular septal defect with tetralogy of Fallot: results from a national audit database

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
D Dorobantu ◽  
R Tulloh ◽  
S Stoica
Keyword(s):  
Tetralogy Of Fallot ◽  
Surgical Management ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
National Audit ◽  
Treatment Pathways ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Repair ◽  
Complete Atrioventricular

Abstract Background Complete atrioventricular septal defect (CAVSD) with tetralogy of Fallot (CAVSD-ToF) is an uncommon association, and the management is more difficult than for each lesion in isolation. Depending on the anatomy, clinical status and ventricular morphology, definitive repair is offered either as staged or single operation with few undergoing single or one and a half ventricle procedures. Currently available data are limited to single centre studies. Purpose We aim to use data from a national audit dataset to describe outcomes in the surgical management of CAVSD-ToF with data from the national audit. Methods All children with CAVSD and tetralogy of Fallot (including double outlet right ventricle and pulmonary atresia variants) undergoing the first cardiac procedure between 2000–2013 were included and all subsequent procedures extracted. Three treatment pathways were defined: definitive primary procedure (“primary complete repair”), staged repair or “unbalanced ventricles repair” (Glenn shunt or modified Fontan). Continuous data is expressed as median (range). Survival data obtained using the Kaplan Meier method and subgroup comparisons done using the log-rank test. Results A total of 125 patients were included, 50.4% male, first procedure at 150 (1–2770) days. Down syndrome was present in 79.4% (63/79 of those with reported non-cardiac comorbidities), while 8% (10/125) had heterotaxy syndrome. “Primary complete repair” was performed in 40% (n=50), at 319 (33–2770) days. Staged repair was undertaken in 28% (n=35), first palliation at 80 (1–733) days. An “unbalanced ventricles” approach was used in 16.8% (n=21), first procedure at 135 (1–1742) days). The other 15.2% (n=19), first procedure at 53 (range 1–436) days, had at least one palliation procedure, but no identifiable committed pathway. Survival at 5 years from the first procedure in “primary complete repair” compared to the remainder of the group is shown in the Figure. No significant differences in survival were found. Specifically in the “unbalanced ventricle repair” group, just 1 patient died during follow-up. A total of 85 patients (68%) achieved either primary or staged “complete repair”. Early mortality (30 day) after the “complete repair” was 6.6% (similar for both approaches, p=0.9). Survival at 5 years after “complete repair” was 82.7% (similar for both approaches, p=0.8). Freedom from any cardiac re-intervention at 5 years was 69.5% (77.6% after primary, 58.1% after staged repair, p=0.03). Conclusion Early-life mortality in this heterogeneous group of CAVSD-ToF remains high, even in patients receiving definitive “complete repair”. We found no survival differences in the medium term between primary repair and a palliation approach, but more cardiac re-interventions were undertaken after staged complete repair. Surprisingly good results were seen in the unbalanced ventricles group, but longer follow up is needed to examine their attrition and longer term outcome. Survival at 5 years by treatment option Funding Acknowledgement Type of funding source: None

Main pulmonary artery cross-section ratio is low in fetuses with tetralogy of Fallot and ductus arteriosus-dependent pulmonary circulation

2017 ◽  
Vol 27 (6) ◽  
pp. 1162-1166 ◽  
Author(s):  
Hironori Ebishima ◽  
Kenichi Kurosaki ◽  
Jun Yoshimatsu ◽  
Isao Shiraishi
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Cross Section ◽  
Pulmonary Circulation ◽  
Ductus Arteriosus ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Cross Section Ratio ◽  
Section Ratio ◽  
Z Scores

AbstractObjectivesThis study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes.MethodsThe Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio – the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation.ResultsWe included two infants with pulmonary atresia and six infants with ductus-dependent pulmonary circulation, who underwent systemic-to-pulmonary shunt surgeries at ⩽1 month of age. The Z-scores of the main pulmonary artery and the pulmonary artery-to-aorta ratio in fetuses with ductus-dependent pulmonary circulation were lesser than those in fetuses with ductus independence, but not significantly. The main pulmonary artery cross-section ratio in fetuses with ductus dependence was significantly lesser (0.65±0.44 versus 1.56±0.48, p<0.005). Besides, the flow of the ductus arteriosus was directed from the aorta to the pulmonary artery in the ductus arteriosus-dependent group during the fetal period.ConclusionsThe main pulmonary artery cross-section ratio was the most significant variable for predicting postnatal outcomes in fetuses with tetralogy of Fallot.

scholarly journals Surgical management of biatrial myxoma: case report

2021 ◽  
Vol 25 (4) ◽  
pp. 118
Author(s):  
T. A. Simonyan ◽  
I. I. Scopin ◽  
I. M. Tsiskaridze ◽  
I. Yu. Farulova ◽  
E. A. Babajanyan
Keyword(s):  
Mitral Valve ◽  
Surgical Management ◽  
Tricuspid Valve ◽  
Conflict Of Interest ◽  
Right Atrium ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Support Ring ◽  
Ventricular Ejection Fraction ◽  
Left And Right

<p>Heart tumours account for approximately 0.2 % of all tumours: of these, approximately 75 % of all primary heart tumours are benign and 50 % of them are myxomas. Further, myxomas make up 0.0017 % of the general population of patients with cardiovascular disease. Biatrial myxomas, i.e. tumours in both the left and right atria, can be in the form of a ‘butterfly’ or a ‘dumbbell’, and account for &lt; 1 % of all cardiac myxomas. Here we describe the successful surgical management of a rare case of a large biatrial myxoma and concomitant atrioventricular valve insufficiency. Briefly, 2D transthoracic echocardiography findings included an end-diastolic volume of 90 ml, an end-systolic volume of 40 ml and a left ventricular ejection fraction of 55 % (according to Simpson). The fibrous ring of the mitral valve measured 36 mm with a regurgitation degree of 2 while the fibrous ring of the tricuspid valve was 42 mm in size and the regurgitation grade was 3. Lesion size in the cavity of the left and right atrium were 73 × 38 mm and 80 × 42 mm, respectively. These neoplasia were surgically removed under peripheral cardiopulmonary bypass, hypothermia and cold cardioplegia (Custodiol). The myxomas were accessed through the right atrium, according to Giradon and were resected without defragmentation. Next, mitral valve plasty using a soft support ring and annuloplasty of the tricuspid valve were performed according to de Vega. The duration of artificial circulation was 150 minutes and that of myocardial ischaemia was 100 minutes. The patient was extubated 11 hours after surgery, spent 22 hours in the intensive care unit and was discharged on the 14th day after surgery. Surgical resection of biatrial myxomas before the development of serious irreversible or life-threatening complications can provide rapid symptomatic relief in congestive heart failure.</p><p>Received 30 May 2021. Revised 22 August 2021. Accepted 23 August 2021.</p><p><strong>Funding: </strong>The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p><p><strong>Contribution of the authors<br /> </strong>Literature review: T.A. Simonyan<br /> Drafting the article: T.A. Simonyan, I.M. Tsiskaridze<br /> Critical revision of the article: T.A. Simonyan, I.Yu. Farulova, E.A. Babajanyan<br /> Surgical treatment: I.I. Scopin, I.M. Tsiskaridze<br /> Final approval of the version to be published: T.A. Simonyan, I.I. Scopin, I.M. Tsiskaridze, I.Yu. Farulova, E.A. Babajanyan</p>

A Methodology to Assess Subregional Geometric Complexity for Tetralogy of Fallot Patients

2019 ◽  
Vol 2 (4) ◽  
Author(s):  
Leslie M. Louvelle ◽  
Matthew G. Doyle ◽  
Glen S. Van Arsdell ◽  
Cristina H. Amon
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Pulmonary Arteries ◽  
Surgical Techniques ◽  
Right Ventricular Outflow Tract ◽  
Average Diameter ◽  
Cross Sectional ◽  
Specific Patient ◽  
Left And Right

Abstract During surgical repair of tetralogy of fallot (TOF), pulmonary valve preservation (preservative repair) has demonstrated improved long-term outcomes compared to repairs that incise into the valve annulus (nonpreservative repair). Given the influence of geometry on hemodynamics, the success of preservative repair may be linked to the suitability of the preoperative patient geometry. However, the specific patient anatomies that may be predisposed to successful preservative repair are unknown due to significant interpatient variability in right ventricular outflow tract (RVOT) and pulmonary artery geometries, as well as the limitations in current methods of subregional geometric analysis. As a first step toward understanding the link between geometry and hemodynamics in TOF patients at a subregion level, we characterize the TOF geometry from the right ventricular infundibulum (INF) to the left and right pulmonary arteries. Our process consists of segmentation of magnetic resonance (MR) images and analysis of cross-sectional slices of the geometries along the centerlines. For the INF, main, left, and right pulmonary arteries individually, we quantify geometric parameters important in determining hemodynamic characteristics such as flow separation and recirculation, which can influence the degree of regurgitation. Specifically, we calculate the diameter along the subregion length, the average diameter, length, and tortuosity for each segment, as well as the bifurcation, left pulmonary artery (LPA) and right pulmonary artery (RPA) branch angles. This approach enables direct geometric comparisons within and among patients and allows for observation of the range in anatomic presentation. We have applied this approach to a dataset of 11 postoperative TOF patients, repaired with both preservative and nonpreservative surgical techniques.

Survey of multinational surgical management practices in tetralogy of Fallot

2018 ◽  
Vol 29 (1) ◽  
pp. 67-70 ◽  
Author(s):  
Sara Hussain ◽  
Osman Al-Radi ◽  
Tae-Jin Yun ◽  
Zhongdong Hua ◽  
Budi Rahmat ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Surgical Management ◽  
Management Practices ◽  
Primary Repair ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Management Technique ◽  
Z Score ◽  
Case Volume ◽  
Elective Repair

AbstractBackgroundA wide variety of surgical strategies are used in tetralogy of Fallot repair. We sought to describe the international contemporary practice patterns for surgical management of tetralogy of Fallot.MethodsSurgeons from 18 international paediatric cardiac surgery centres (representing over 1800 tetralogy of Fallot cases/year) completed a Research Electronic Data Capture-based survey. Participating countries include: China (4), India (2), Nepal (1), Korea (1), Indonesia (1), Saudi Arabia (3), Japan (1), Turkey (1), Australia (1), United States of America (2), and Canada (1). Summary measures were reported as means and counts (percentages). Responses were weighted based on case volume/centre.ResultsPrimary repair is the prevalent strategy (83%) with variation in age at elective repair (range). Approximately 47% of sites use patient age as a factor in determining the strategy, with age <3 months being the common cut-off for staged repair. In addition, patient weight of <3 kg is an indication for staged repair in 80% of participating institutions. Trans-atrial ventricular septal defect closure is the preferred approach in 62% of sites. Approximately 70% of responders reported using pulmonary valve z-score to guide right ventricular outflow tract management technique with the most prevalent cut-off for annulus preservation being a z-score of −3. Estimated incidence of annulus preservation is 53%. Minimal trans-annular incision is performed in >90% of all trans-annular repairs.ConclusionsIn this cohort representing 11 countries, there is variation in tetralogy of Fallot surgical management with no consensus on standard of practice. A large international prospective cohort study would allow analysis of impact of underlying anatomy and repair strategy on early and late outcomes.

scholarly journals Validity of Pulmonary Valve Z-Scores in Predicting Valve-Sparing Tetralogy Repairs—Systematic Review †

Children ◽  
2019 ◽  
Vol 6 (5) ◽  
pp. 67
Author(s):  
Raina Sinha ◽  
Vasu Gooty ◽  
Subin Jang ◽  
Ali Dodge-Khatami ◽  
Jorge Salazar
Keyword(s):  
Pulmonary Valve ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
P Value ◽  
Complete Relief ◽  
Z Score ◽  
Z Scores ◽  
Valve Sparing

There is a lack of consensus regarding the preoperative pulmonary valve (PV) Z-score “cut-off” in tetralogy of Fallot (ToF) patients to attempt a successful valve sparing surgery (VSS). Therefore, the aim of this study was to review the available evidence regarding the association between preoperative PV Z-score and rate of re-intervention for residual right ventricular outflow tract (RVOT) obstruction, i.e. successful valve sparing surgery. A systematic search of studies reporting outcomes of VSS for ToF was performed utilizing PubMed, EMBASE, and Scopus databases. Patients with ToF variants such as pulmonary atresia, major aortopulmonary collaterals, absent pulmonary valve, associated atrioventricular septal defect, and discontinuous pulmonary arteries were excluded. Out of 712 screened publications, 15 studies met inclusion criteria. A total of 1091 patients had surgery at a median age and weight of 6.9 months and 7.2 kg, respectively. VSS was performed on the basis of intraoperative PV assessment in 14 out of 15 studies. The median preoperative PV Z-score was −1.7 (0 to −4.9) with a median re-intervention rate of 4.7% (0–36.8%) during a median follow-up of 2.83 years (1.4–15.8 years). Quantitatively, there was no correlation between decreasing preoperative PV Z-scores and increasing RVOT re-intervention rates with a correlation coefficient of −0.03 and an associated p-value of 0.91. In observational studies, VSS for ToF repair was based on intraoperative evaluation and sizing of the PV following complete relief of all levels of obstruction of the RVOT, rather than pre-operative echocardiography derived PV Z-scores.

P268 Anomalies of pulmonary arteries in Tetralogy of Fallot in developing countries: study of 100 cases in indian population

2020 ◽  
Vol 41 (Supplement_1) ◽  
Author(s):  
A Tiwari ◽  
PARAG Barwad ◽  
U M A Dabi
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Surgical Management ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Great Vessels ◽  
Pulmonary Artery Abnormalities

Abstract Introduction Right ventricular outflow tract (RVOT) obstruction in some or other form is one of the major components of Tetralogy of Fallot. It can occur at any level, isolated or in combination, from Infundibulum (most common), Pulmonary valve, Main pulmonary artery, Right and Left pulmonary arteries and up to their segmental branches. Incidence of pulmonary artery anomalies are reported between 15-20%, though some reported as high as 40%.  Purpose  The objective of this retrospective  observational study is to determine the presence and degree of pulmonary artery  abnormalities and associated cardiac defects in patients with Tetralogy of Fallot and to  define the pulmonary arterial anatomy to guide the further surgical management. Method A total of 100 newly diagnosed or follow up cases of TOF irrespective of age (range 5 days- 32 years) and gender (32 female, 68 male) who were planned for surgical management and referred for evaluation of pulmonary artery anatomy. All patient underwent non-ECG gated CT pulmonary Angiography in Toshiba 64 slice CT scanner.  CT data was analyzed retrospectively to look for cardiac and pulmonary artery anatomy by one independent observer having experience in cardiac radiology. Results 92 patient had infundibular and valvular stenosis and 8 patients has atretic pulmonary valve with additional supravalvar pulmonary stenosis in 17% patient. Out of total 100 patients, 35% had pulmonary artery abnormalities. This included 19 patient (19%) with isolated main pulmonary artery (MPA) abnormality, 6 % had isolated left pulmonary artery (LPA) abnormality, 2 had isolated right pulmonary artery abnormality, 4% had  combined MPA and LPA  abnormality, 2% had combined MPA and RPA abnormalities and 1% patient had all 3 (MPA,LPA,RPA) involvement in form of stenosis or hypoplasia. As TOF is commonly associated with other cardiac structural anomalies, we encountered following associations in our study – aortopulmonary collaterals (37%), Patent ductus arteriosus (29%), right sided aortic arch (19%), bilateral superior vena cava (10%), bovine arch (3%), double aortic arch (2%), d malposition of great vessels (2%), l malposition of great vessels (1%), situs inversus (1%), dextrocardia (1%), annuloaortic ectasia (1%) aortopulmonary window (1%) and cardiac totally anomalous pulmonary venous connection (1%). Conclusions Anomalies of pulmonary arteries are important determinant of surgical outcome of TOF patients. CT angiography is now established modality to delineate pulmonary artery and other anatomical details in TOF patient. Pulmonary artery anomaly can range from isolated branch pulmonary stenosis to diffuse hypoplasia of pulmonary vasculature, based on which management can differ from complete correction to palliative or medical management only. Our study emphasize the importance of pulmonary artery evaluation and its variation among patients of TOF. Abstract P268 Figure.

scholarly journals Unbalanced atrioventricular septal defect with dominant right ventricle: diagnostic criteria, indications for biventricular correction, and results. A clinical observation series

2021 ◽  
Vol 49 (5) ◽  
pp. 347-354
Author(s):  
V. P. Didyk ◽  
V. A. Belov ◽  
O. N. Bukhareva ◽  
O. A. Laryutina ◽  
T. L.  Akobyan ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Right Ventricle ◽  
Diagnostic Criteria ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Z Score ◽  
Biventricular Repair ◽  
Cavopulmonary Anastomosis ◽  
Left And Right ◽  
The Mean

Relevance: Surgical treatment for congenital heart disease (CHD) with borderline left ventricle (LV) dimensions is one of the most challenging issues in current pediatric cardiac surgery. Despite the prevailing general trend to the expansion of indications for biventricular repair of an unbalanced atrioventricular septal defect (AVSD) with a dominant right ventricle (RV), the diagnostic criteria for imbalance, feasibility of radical repair and its functional results are intensively discussed in the literature.Aim: To present quantitative criteria for the assessment of the LV size which could serve as an indication for biventricular repair of the right dominant unbalanced AVSD.Materials and methods: We present a  series of 4  patients with AVSD and dominant RV, who underwent radical repair of the defect. The mean age of the patients was 2.4 years, and 3 of 4 had concomitant CHD: tetralogy of Fallot, double outlet right ventricle with pulmonary artery stenosis, and moderate hypoplasia of the aortic arch with subaortic stenosis. Three of 4 patients had previously undergone palliative interventions: two of them – pulmonary bending, one, the first stage of univentricular repair (atrioseptostomy, cavopulmonary anastomosis). Two children had been diagnosed with Down syndrome. The mean left ventricular end diastolic diameter (LVEDD) at baseline was 17.9 mm, corresponding to Z score of -5.4.Results: All four patients underwent biventricular repair of the defect with a  two-patch technique. In one case, it was supplemented with placement of the pulmonary trunk homograft prosthesis, in the other with pulmonary valve commissurotomy and cavopulmonary anastomosis due to moderate tricuspid valve stenosis and in the third case the mitral valve was replaced. In 3 patients pacemaker implantation was performed. The criterion for feasibility of complete septation was the ratio of the longitudinal dimensions of the left and right ventricles (LAR > 0.7), confirmed by computed tomography performed before the surgery. The mean LVEDD after surgery was 28.3 mm, corresponding to Z score of -0.8. At the time of the hospital discharge, the left and right atrioventricular valves insufficiency did not exceed 2 and 1 degrees, respectively.Conclusion: Computed tomography allows for accurate measurement and comparison of the longitudinal dimensions of the heart ventricles with LAR index as a tool for assessment of the LV size before the biventricular repair. An additional prospective study is required to objectify the data obtained and identify predictors of an unfavorable outcome of radical repair in patients with right dominant unbalanced AVSD.

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