Half Ventricle Repair Research Articles

Experience of Surgical Treatment of Complex Forms of Ebstein’s Anomaly through the Method of Hemodynamic Correction

Ebstein’s anomaly (EA) is a rare and complex congenital heart disease, with a prevalence of 4 to 7 cases per 100,000 live births. At this stage of the development of EA surgery, significant progress has been made in our understanding and treatment of EA, but the question of the tactics of treating complex forms, especially in the period of infancy and early childhood, remains open. Due to significant phenotypic heterogeneity, the treatment of EA varies greatly from patient to patient and depends on many factors that require a comprehensive approach. The choice of the method of surgical correction directly depends on the anatomical features: the degree of dysplasia, rotation and displacement of the tricuspid valve, the degree of atrialization of the right ventricle, the combination with other congenital heart diseases, and the severity of hemodynamic disorders.
 The aim. To evaluate perioperative characteristics in patients with complex forms of EA, immediate and long-term results after hemodynamic correction.
 Materials and methods. In the period from 1996 to 2022, at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine, one and a half ventricle repair was performed in 18 patients with complex forms of EA. There were 12 (67%) male patients, 6 (33%) female patients. The median age of the patients at the time of surgery was 30 months [3.5; 312]. The main diagnostic method in determining the defect and assessing the immediate and remote results was echocardiography and probing of the heart cavities. In order to qualitatively evaluate the perioperative, immediate and long-term results to determine the optimal tactics and terms of surgical intervention, prevention of possible complications, all the patients were divided into two age groups: I group up to 2.5 years including 9 patients (50%), II group older than 2.5 years including 9 patients (50%).
 Results. In the postoperative period, 2 (11%) deaths were noted in both groups. In all the deceased patients, the cause of death was acute heart failure and acute respiratory failure. The analysis of the postoperative period showed normal results in such a cohort of highly complex patients of both groups, but a more stable course was noted among patients of the I group, as indicated by statistically significant values for the duration of mechanical ventilation, stay in the intensive care unit, stay in the hospital, the average dose of sympathomimetic support and its duration, the total duration of exudation in the comparison groups.
 An uncomplicated course of the early postoperative period was observed in 8 (44%) patients. The remaining 10 (56%) patients of both groups had: I group, 3 (33%) patients: 7 complications; II group, 5 (55%) patients: 13 complications. In 5 patients (2 patients in I group and 3 patients in II group), attention-grabbing acute heart failure was observed, which required high doses and long-term sympathomimetic support, and in 2 patients caused death. The average observation period was 45 months [6; 190]. No deaths were observed during the observation period among 13 (72%) patients of both groups, contact was lost with 3 (17%) patients. There were no violations in the functioning of the right ventricle, but the recovery of the systolic function of the left ventricle occurred only in 8 (44%) patients.
 Tricuspid insufficiency was minimal in 5 (28%) patients, moderate in 6 (33%), severe in 2 (11%) patients.
 Conclusions. Determining the tactics of treatment of complex forms of EA is a serious problem in patients of various ages, especially in newborns and infants, due to pronounced dilatation and systolic dysfunction of the left ventricle, a sharp decrease in systemic output due to changes in the geometry of the left ventricle. The technique of hemiventricular correction shows very promising results and may become the technique of choice for patients with severe forms of EA and pronounced right ventricular insufficiency.

Comparison of the Methods of Surgical Treatment of Complex Congenital Heart Defects Combined with Right Ventricular Hypoplasia

Surgical treatment of complex congenital heart defects (CHD) in patients who cannot undergo radical correction due to pronounced hypoplasia or dysfunction of the right ventricle (RV) remains one of the most urgent problems in pediatric cardiac surgery. In 1989, Billingsley et al. first reported the addition of bidirectional cavopulmonary anastomosis to definitive biventricular repair in patients with RV hypoplasia, pulmonary artery atresia, and intact interventricular septum in four patients, and introduced the term one and half ventricle repair (1.5VR). The method of 1.5VR consists of bidirectional cavopulmonary anastomosis, which reduces the volume load on the RV, in addition to the complete repair of other CHDs.
 The aim. To assess perioperative characteristics of patients, immediate and long-term results after surgical correction of complex CHD through the method of 1.5VR.
 Materials and methods. In the period from 1996 to 2022, surgical correction was performed in 33 patients with complex CHD combined with hypoplasia and/or dysfunction of the RV at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. There were 21 male patients (64%) and 12 female patients (36%). The mean age of the patients at the time of surgery was 57.9 months. Me = 34 [3.5; 312] months. The main method of diagnosis in determining the defect and assessing the immediate and long-term results was echocardiographic examination and probing of the heart cavities, which made it possible to assess the morphology, kinetics of the heart structures, and hemodynamic state. Depending on the selected tactics of surgical treatment, all the patients were divided into two groups: group І (n=21) with patients operated through the method of 1.5VR, and group ІІ (n = 12) including those operated through the single ventricle pathway.
 Results. In the early postoperative period, there were 3 (9%) deaths in both groups: one (4.7%) death in group I, and 2 (16%) deaths among patients of group II. In all the deceased patients, the cause of death was acute heart failure, which subsequently led to multiple organ failure. In one patient of the group II, in addition to heart failure, acute cerebrovascular accident occurred. In the group I, the average indicator of systemic saturation (92±6.2%) at discharge from the hospital was higher, than that in group II (87±4.7%). Average duration of mechanical ventilation (10±7.3 vs. 15±7.8 hours), total exudation (65±34 vs. 88.8±39.9 hours), total duration of sympathomimetic support (71±27.5 vs. 108±75.5 hours) and its doses (4.8±2 vs. 6.5±3.8 μkg/kg/hour) were significantly lower in the group I, therefore, the patients of the group II stayed almost twice as long in the intensive care department (146±56 vs. 96±49.8 hours), although the total length of hospital stay did not differ significantly (25±3.8 vs. 26±12.3 days). An uncomplicated course of the early postoperative period was observed in 15 (46%) patients. Other 18 (54%) patients had complications: 8 patients (24%) of group I had 9 complications, and 10 patients (30%) of group II had 14 complications. The mean observation period was 48±29.8 months, (6 to 190 months). No deaths were observed during the observation period in 26 patients (79%) of both groups, 4 (12%) patients were lost to follow-up. Total cavоpulmonary anastomosis (Fontan procedure) was performed in 7 patients (58%) of the group II. Hemodynamic indicators in the examined 17 patients (81%) of the group I showed good long-term results that did not require other interventions. In 15% (3 of 20) of the patients of the group I, a high mean pressure in the right atrium of 14±1.8 mmHg attracts attention with dilatation of the inferior vena cava and hepatic veins, and 5 (20%) patients had high mean pressure of 16±2.7 mmHg in the superior vena cava. Transplantation-free survival during the observation period in patients of both groups was 100%.
 Conclusions. 1.5VR is a reasonable alternative to the palliative Fontan strategy in children in whom the anatomic or functional status of the RV is between biventricular reconstruction and the single ventricle pathway and shows good immediate and long-term results.

Bidirectional Glenn shunt with tricuspid valve resection in patients with infective endocarditis.

Bidirectional Glenn shunt with tricuspid valve resection in patients with infective endocarditis.

УСПЕШНАЯ КОРРЕКЦИЯ ОБЩЕГО АТРИОВЕНТРИКУЛЯРНОГО КАНАЛА С ТЕТРАДОЙ ФАЛЛО И ГИПОПЛАЗИЕЙ ПРАВОГО ЖЕЛУДОЧКА У РЕБЕНКА 2 ЛЕТ 4 МЕСЯЦЕВ

The paper presents a description of a case of successful one and a half ventricular correction of a complex congenital cardiac anomalies, including the common atrioventricular canal, tetralogy of Fallot, and hypoplasia of the right ventricle in a child (2 year and 4 months). Ararecongenitalanomalycharacterized by acombinationof intracardiac defects, required an innovative surgical approach, the exclusivity of this clinical case has determined. The surgical strategy of complete atrioventricular canal defect is determined by a number of factors. One of the most important is balance between right and left ventricular outputs. The balanced type with adequate development of the ventricles involves the biventricular repair performance. Unbalancedatrioventricular canal defects includea hypoplastic ventricle. Reconstructive surgeryfor onehypoplasticventricle is oriented towards the degree of hypoplasia. In particular, a mild right ventricular hypoplasia allows perfoming biventricular radical operation while a severe degree suggests univentricular repair. In case of borderline, moderate degree of hypoplastic right ventricle the one and half ventricle repair can be carried out. Another important point is the presence of concomitant pulmonic stenosis required the choice of optimal method and material for reconstruction which is not always obvious and often it is the subject of debates. This report presents a description of the diagnostic stages with an emphasis on determining the type of balance of the common atrioventricular canal, the degree of hypoplasia of the right ventricle, the approach to choosing the optimal method for correcting the defect in general and the material for reconstructing the outflow pathway from the right ventricle in particular, as well as the subsequent successful correction of congenital multicomponent cardiac abnormalities in a young patient.

Cone Reconstruction with Successful One and A Half Ventricle Repair for Ebstein Anomaly

Ebstein anomaly is a rare pathology among congenital cardiac diseases with a prevalance of 0.5%. Ebstein anomaly patients suffer from arrhytmia, severe right ventricular dysfunction and left ventricular dysfunction due to cyanosis and right ventricular involvement. Most of the patients are presented at infancy, childhood and rarely after adolescence ages. Biventricular repair has a high mortality and morbidity and unacceptable func- tional results in Ebstein anomaly patients with severe right ventricular dysfunction, one and a half ventricle repair yields better results. In our case, we presented a one and a half ventricle repair with a successful Cone type repair in a 34-year-old male patient who referred to our clinic with cardiac arrest presentation and was detected to have functional type C Ebstein anomaly.

Restriction of Atrial Septal Defect Leads to Growth of Hypoplastic Ventricle in Patients with Borderline Right or Left Heart

Patients with borderline hypoplastic right or left ventricle and VSD may be candidates for either single ventricle palliation or staged ventricular recruitment (SVR) followed by eventual biventricular conversion. Components of SVR include restriction of atrial septal defects (ASD) without ventricular septal defects (VSD) closure and addition of accessory pulmonary blood flow. This study evaluated the impact of ASD restriction on ventricular growth and function. We retrospectively reviewed patients with borderline ventricular hypoplasia and VSD who underwent a staged ventricular recruitment (SVR) procedure from 2012 to June 2019. Pre- and post-recruitment MRI and echocardiogram data were compared and analyzed. We excluded cases in which we intentionally restricted VSD with simultaneous ASD restriction. Forty-six patients (41 with right-dominant ventricle, 25 with risk factors for Fontan procedure) underwent SVR at a median age of 15.1 months' (interquartile range (IQR), 7.2-37.2 months'). The median indexed ventricular end-diastolic volume, end-systolic volume, and stroke volume according to cardiac MRI significantly increased at median 11.0 months' (IQR:7.8~14.1 months') after recruitment. Among them, except 2 operative mortalities after SVR, 26 patients underwent bi-ventricular repair (56.5% including one and a half ventricle repair) at a median of 8.0 months' (IQR: 6.2-12.2 months') after recruitment. Fifteen patients await biventricular completion, and 3 patients underwent single ventricle palliation. Pulmonary blood flow (Qp) tended to increase after recruitment regardless of type of pulmonary blood flow modification without statistical significance. Six patients died at a median duration of 6.5 months' (IQR: 2.9-11.7) after SVR; 3 patients died after biventricular completion, 2 after recruitment, and 1 after returning to single ventricle palliation. All of them were considered poor Fontan candidates due to severe atrioventricular valve regurgitation, pulmonary hypertension, pulmonary vein stenosis, or airway stenosis. Restriction of the atrial septum leads to the growth of hypoplastic ventricle in patients with ventricular septal defects who undergo SVR regardless of the preoperative characteristics, and eventual biventricular repair can be performed in a subgroup of these patients. Future work is necessary to optimize timing of SVR and method of accessory pulmonary blood flow.

Arrhythmic Risk in Paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum

Introduction: While previous studies only focused on the arrhythmic risk associated with specific correction strategies, this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair. Methods: In this single centre observational cohort study, we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment, patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes. Surgical history and clinical outcomes were reviewed. Results: 86 patients were included in the study (54 male [62.8%], mean age 16.4 ± 6.1 years), with median follow-up from definitive repair of 12.8 years (6.4–18.9 years). They underwent three different final repairs: 23 patients (26.7%) univentricular palliation, 43 (50%) biventricular correction, and 20 (23.3%) one and a half ventricle correction. Thirteen patients (15%) developed arrhythmia: 6 patients (all the subgroups) sinus node disfunction (SND); 2 (biventricular repair) premature ventricular complexes; 2 (one and a half ventricle repair) non-sustained ventricular tachycardia; 1 (biventricular repair) intra-atrial re-entrant tachycardia; 1 (one and a half ventricle repair) supraventricular tachyarrhythmia; 1 (biventricular repair) atrial fibrillation. Three patients with SND needed a pacemaker implantation. Only Fontan circulation showed an association with SND, while the other two groups heterogeneous types of arrhythmias. Conclusions: The low arrhythmic risk is related to surgical repair, it does not appear to be associated with native cardiomyopathy, and it appears to increase with length of follow up. Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.

Conversion of prior univentricular repairs to septated circulation: Case selection, challenges, and outcomes.

Complex congenital heart defects that present earlier in life are sometimes channelled in the single ventricle pathway, because of anatomical or logistic challenges involved in biventricular correction. Given the long-term functional and survival advantage, and with the surgeons' improved understanding of the cardiac anatomy, we have consciously explored the feasibility of a biventricular repair in these patients when they presented later for Fontan completion. We present a single institution's 10-year experience in achieving biventricular septation of prior univentricular repairs, the technical and physiological challenges and the surgical outcomes. Between June 2010 and December 2019, 246 patients were channelized in the single ventricle pathway, of which 32 patients were identified as potential biventricular candidates at the time of evaluation for Fontan palliation, considering their anatomic feasibility. The surgical technique involves routing of the left ventricle to the aorta across the ventricular septal defect, ensuring an adequate sized right ventricular cavity, establishing right ventricle-pulmonary artery continuity and taking down the Glenn shunt with rerouting of the superior vena cava to the right atrium. This is a retrospective study where we reviewed the unique physiological and surgical characteristics of this subset of patients and analysed their surgical outcomes and complications. Biventricular conversion was achieved in all cases except in 3 patients, who had the Glenn shunt retained leading to a one and a half ventricle repair. The average age of the patients was 4.9years of whom 18 were male. The average cardiopulmonary bypass time was 371min with an average cross clamp time of 162min. There was one mortality in a patient with corrected transposition of great arteries (c-TGA) with extensive arterio-venous malformations (AVMs). At a median follow-up of 60months, all patients remained symptom free except two with NYHA II symptoms, one being treated for branch pulmonary artery stenosis with balloon dilatation and the other with multiple AVMs who needed coil closure. One patient with branch pulmonary artery (PA) stenosis required balloon dilatation and stent placement. The possibility of achieving the surgical goal in this unique subset of patients evolves with the progressive experience of the congenital heart surgeon. Case selection is a crucial aspect in achieving the desired outcome, and this 'borderline' substrate is often recognized at the time of evaluation for the Fontan completion. A comprehensive preoperative imaging and planning helps in achieving the surgical septation and reconnection to achieve the desired physiological circulation. Though technically challenging, the surgery has excellent short- and mid-term outcomes as evidenced by our 10-year experience.

Does addition of bi-directional cavo-pulmonary shunt to tricuspid repair in advanced cases of Ebstein anomaly result in better outcomes?

Ebstein anomaly can be managed by single ventricular, bi-ventricular and one and a half ventricular repairs. We present midterm results of Comprehensive Tricuspid Valve repair (CTVR) with bi-directional cavo-pulmonary shunt (BCPS). In this prospective observational study (Jan2012-July2018), 69 patients underwent surgery for Ebstein anomaly. In Group I (n = 48; 69.6%), all patients got CTVR and a BCPS (one and a half ventricle repair). Group II (n = 15; 21.8%) consisted of a similar repair without BCPS (bi-ventricle repair). All patients were echocardiographed at six monthly intervals. Median age of the cohort was 17years (range 1-68). 12 (17.4%) patients were Carpentier type B, 51 (73.9%) were type C and 6 (8.7%) were type D. There were two early mortalities (2.89%). At a mean follow up of 3.2 ± 1.2years, there were no late deaths and one delayed repair-failure in each group. Group I had significantly lower mean TR grade (1.2 ± 0.4vs1.6 ± 0.5, p = 0.03) as compared to Group II without a significant difference in the mean gradients (1.5 ± 0.5vs1.6 ± 0.6, p = 0.4). Mean indexed TAPSE (15.0 ± 6.7vs.16.6 ± 5.6mm/m2, p = 0.21), NYHA class (1.2 ± 0.4vs1.3 ± 0.4) and six-minute walk distance (506 vs 507m, p = 0.7) was similar in both groups. One and a half ventricle repair of Ebstein anomaly gives a more functionally competent, non-stenotic and durable tricuspid valve as compared to a two-ventricle repair. BCPS doesn't result in facial swelling or AV malformations. Preload reduction by BCPS may allow the myopathic ventricle to remodel.

Numerical Simulation of the Effect of Pulmonary Vascular Resistance on the Hemodynamics of Reoperation After Failure of One and a Half Ventricle Repair.

ObjectiveThe one and a half ventricle repair (1.5VR) is a common clinical choice for patients with right heart dysfunction. Considering the influence of blood circulation failure and reoperation in urgent need, this essay aims to explore the hemodynamic effects of different pulmonary vascular resistance (PVR) values on reoperation after 1.5VR failure.MethodsThe lumped parameter model (LPM) was used to simulate the reoperation, including the return biventricular repair (2VR), ligation of azygos vein (1.5VR′) and return single ventricular repair (1.0VR). Firstly, the debugging parameters were used to simulate the hemodynamics of 2VR. Secondly, the value of PVR was changed from one to four times while the other parameters remained unchanged. Finally, 15 cardiac cycles were simulated and the 15th result was obtained. In this work, the left and right ventricular stroke work and their sum (Plv, Prv, Ptotal), the left and right ventricular ejection fraction (LVEF, RVEF), the mean Cardiac Output (mCO) and the mean pressure and flow-rate ratio of superior and inferior vena cava (mPsvc\\mPivc and mQsvc\\mQivc), respectively, were used to describe the hemodynamics of reoperation.ResultsWith the change of PVR from one to four times, the values of Plv, Prv, Ptotal, LVEF, and RVEF gradually decreased. The change rate of Plv, Ptotal and LVEF of 1.0VR were the largest in the three kinds of reoperation. The change rate of Prv of 1.5VR′ was larger than that of 2VR, but it was the opposite for their EF change rate. The mCO of 2VR, 1.5VR′, and 1.0VR decreased by 18.53%, 37.58%, and 48.07%, respectively. The mPsvc\\mPivc of 1.5VR′ increased from 3.76 to 6.77 and the mQsvc\\mQivc decreased from 0.55 to 0.36, while the mPsvc\\mPivc and mQsvc\\mQivc of 2VR and 1.0VR remained 1 and 0.67, respectively. The peak value of the tricuspid flow-rate (Qti) waveform of 2VR and 1.5VR′ changed from “E peak” to “A peak.”ConclusionThe numerical results demonstrate the highly reoperation-dependent hemodynamic consequences and their responses to variations in PVR. Comprehensive analysis of EF, mCO and ventricular stroke work indicates that PVR has a greater impact on 1.5VR′ and 1.0VR. Therefore, we suggest that the selection strategy of reoperation should focus on PVR.

Surgical treatment of Ebstein anomaly: A unicenter experience

Aim: Ebstein anomaly is a rare congenital cardiac disorder that causing by failure of delamination of the tricuspid valve. Various surgical methods have been developed so far as treatment options. Our initial experience and short term follow up reported herein.Materials and Methods: Ten patients underwent surgical treatment with a diagnosis of Ebstein anomaly in our center between 2007 and 2017. Two patients underwent one and a half ventricle repair and Danielson method applied to eight patients. The X-clamping and cardiopulmonary by-pass (CPB) timing, duration in intensive care unit (ICU), extubation time and need for blood product were compared between Danielson and one and a half ventricle repair applied patients.Results: There was no mortality among patients. In the one and a half repair applied group both X-clamping (p=0.044) and CPB time (p=0.044) was longer compared to Danielson group. On the other hand there is no significantly difference between groups in ICU duration (p=0.400) or extubation timing (p=0.889). Furthermore, need for blood product and amount of drainage were not different between groups when two infant age cases were excepted while calculating. Conclusion: Danielson method is safe and effective in the repair of Ebstein anomaly and can be applied at infant age with similar clinical outcomes. Nevertheless, one and a half repair option is also beneficial in patients with a large size atrialized right ventricular volume. The last decision should be confirmed by heart team considering patient characteristics and surgeons’ past experience.

Early and Long-Term Outcomes of Surgical Treatment of Ebstein's Anomaly.

ObjectiveThis study aimed to evaluate Ebstein’s anomaly surgical correction and its early and long-term outcomes.MethodsA retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein’s anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction.ResultsValve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients’ mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure.ConclusionIn our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.

Abstract 16788: Anatomical Repair Can Be Facilitated by Three-Dimensional Cardiac Printing in the Patients With Double Outlet Right Ventricle

Introduction: Determination of optimal strategy between single ventricle palliation (SVP) versus left ventricular recruitment (LVR) as a systemic ventricle is challenging in the patients with complicated double outlet right ventricle (DORV). We sought that three-dimensionally printed cardiac models (3DM) may enables the surgeons to make an intuitive preoperative decision potentially in favor of LVR. Hypothesis: Use of 3DM can extend the candidates for LVR and improve surgical outcomes. Methods: We retrospectively reviewed the patients with DORV from Jan 2008 to Jun 2018 in single center. The patients with discordant atrioventricular (AV) connection, complete AV septal defect and atresia of AV valves were excluded. The patients without at least one of LVR precluding factors such as left or right ventricular hypoplasia, mitral valve (MV) or tricuspid valve (TV) straddling, TV interposition in potential baffle course, huge ventricular septal defect (VSD), multiple VSDs and remote VSD were excluded. All patients were divided in SVP or LVR group except two patients without clear determination of strategy. Finally, 100 patients were enrolled and since 2016, 3DM was made in the patients with LVR precluding anatomy for better consensus formation whether LVR could be feasible in 26 patients. Risk factor analysis was done for SVP and adverse outcome. Results: Thirty-five patients enrolled as SVP strategy (Fontan operation in 14, Kawashima operation in 1, interstage mortality in 12, follow-up loss in 3 and waiting scheduled operation in 5) and 65 patients enrolled as LVR strategy (complete anatomical correction in 50, one and half ventricle repair in 6 and waiting scheduled operation in 9). In multivariate analysis, LV hypoplasia ( P =0.003) and MV straddling ( P =0.015) were significant LVR precluding factors and the use of 3DM was a favorable factor for LVR ( P =0.039, OR=0.212). Risk factor for mortality were LV outflow tract obstruction ( P =0.006) and LV hypoplasia ( P =0.043). The use of 3DM did not reach statistical significance on mortality ( P =0.132). Conclusions: In DORV patients, LVR can be precluded especially with LV hypoplasia and MV straddling however, LVR can be facilitated by direct-vision driven preoperative decision about complicated features with 3DM.

The outcomes of operation for 237 patients with Ebstein anomaly

Objective: To evaluate the early and long-term outcomes cardiac surgery of patients with Ebstein anomaly. Methods: The clinic data of 237 patients with Ebstein anomaly received surgical procedures from March 2004 to December 2017 at Department of Cardiac Surgery, First Hospital of Tsinghua University was analyzed retrospectively. There were 105 male and 132 female patients with age of (19.4±16.7) years (ranging from 3 months to 64 years). The surgical procedures include anatomical repair in 188 patients, one and a half ventricle repair in 37 patients, tricuspid valve repair in 4 patients, tricuspid valve replacement in 10 patients, and Fontan procedure in 3 patients (total cavopulmonary connection in 2 patients; Glenn procedure in 1 patient). Results: The early mortality was 2.1% (n=5). One case of atrioventricular (0.4%) newly occurred. There were 228 patients available to follow-up. The range of follow-up duration was 3 to 168 months. Late survival was 99.1% (2 cases of late death) at 10 years. Three patients received reoperation (1.3%), including tricuspid valve repair of 1 patient and one and a half ventricle repair of 2 patients). Indication of tricuspid valve regurgitation improved from 3.6±0.3 to 1.5±0.4. Survival rate at 5 and 10 years was 98.6% and 98.2%, respectively. Conclusions: The principle of the techniques is to reconstruct the tricuspid valve and right ventricle anatomically. For most cases, the anatomical repair was demonstrated with low mortality, less complications and excellent durability at long-term follow-up. If the tricuspid valve is severely hypoplastic, one and a half ventricle repair and valve replacement may be alternatie.

Transcatheter perforation of atretic pulmonary valve by the stiff end of a coronary wire in neonates with pulmonary atresia with intact ventricular septum: A solution in developing countries

ObjectivesTo evaluate the safety of using the stiff end of a coronary wire to perforate an atretic pulmonary valve (PV) in patients with pulmonary atresia with intact ventricular septum (PAIVS). BackgroundRadiofrequency perforation is an accepted modality to perforate the PV in patients PAIVS. However, the high cost precludes its widespread use. Patients and methodsThis is a single-center experience that spanned from March 2013 to January 2016 and involved 13 neonates who were severely cyanotic with PAIVS and with ductal-dependent pulmonary circulation. The stiff end of a coronary wire was used to perforate the atretic PV anterogradely, followed by balloon pulmonary valvuloplasty. ResultsThe mean age of patients was 3.9 ± 2.7 days and their mean weight was 2.8 ± 0.19 kg. The mean oxygen saturation was 77.1 ± 3.2%. All had membranous pulmonary atresia, with patent infundibulum and tripartite right ventricle. The valve was successfully perforated in 11 out of 13 patients. Death occurred in two patients (15.4%) owing to heart failure and sepsis. Patent ductus arteriosus stenting was performed 2 days after the procedure in one patient because of cyanosis followed by one and half ventricle repair at of age 5 months. Two patients (15.4%) had one and a half ventricle repair at age of 5 months and 6 months owing to insufficient anterograde pulmonary flow. Two patients (15.4%) underwent second intervention with balloon dilatation of the valve. The remaining seven patients (53.8%) had no further intervention. Two cases (15.4%) had femoral artery thrombosis treated with streptokinase. The mean duration of follow-up was 13.17 ± 7 months. There was significant improvement in the degree of tricuspid incompetence. There was a significant growth in the tricuspid valve annulus during the follow-up (the mean Z score increased from −0.8 ± 0.9 to 0.1 ± 0.9) (p = 0.003). There was also a significant increase in the tricuspid valve annulus/mitral valve annulus ratio as its mean increased from 0.73 ± 0.10 to 0.86 ± 0.11 during follow-up (p < 0.001). ConclusionPerforation of the atretic PV in selected cases with membranous atresia and patent infundibulum using the stiff end of a coronary wire is an effective alternative to using radiofrequency perforation.

One and half ventricle repair: rationale, indications, and results.

Surgical strategies in patients with functionally or anatomically borderline right ventricles include a high-risk biventricular repair, a Fontan procedure, or a one and half ventricle repair (also referred to as the partial biventricular repair). One and half ventricle repair (1.5VR) circumvents the high early mortality of a biventricular repair and also the late morbidity of the Fontan. The two most common indications for a 1.5VR are a small pulmonary ventricle and a dilated poorly functioning pulmonary ventricle. Extension of 1.5VR to patients undergoing anatomical repair for congenitally corrected transposition of great arteries, straddling tricuspid valves, and severe Ebstein's anomaly has facilitated biventricular repair with decreased mortality. We reviewed the relevant literature on this subject in detail and describe its rationale, indications and its early and late results.

Right atrium and superior vena cava pressure measurements in a novel animal model to study one and a half ventricle repair as compared to Fontan type procedure

Background &amp; Objectives: To evaluate the advantages of the one and a half ventricle repair on maintaining a low pressure in the inferior vena cava district. Also evaluate the competition of flows at the superior vena cava – right pulmonary artery anastomosis site, in order to understand the hemodynamic interaction of a pulsatile flow in combination to a laminar one. Materials &amp; Methods: Adult rabbits (n=30) in terminal anaesthesia with a follow up of 8 h were used, randomly distributed in three experimental groups: Group 1: animals with an anastomosis between superior vena cava and right pulmonary artery, as a model of one and one half ventricle repair; Group 2: animals with the cavopulmonary anastomosis followed by clamping of the right pulmonary artery proximal to the anastomosis; and Group 3: sham animals. Pressures of superior vena cava and pulmonary arteries were afterwards measured, in a resting condition as well as after induced pharmacological stress test.Results: In Group 1, superior vena cava pressure was significantly higher, while venous pressure in the inferior vena cava – right atrium district was constant or lower in comparison with the other groups. After stress test, the pressure in the superior vena cava and the heart rate both increased further, but the right ventricular, right atrial and pulmonary artery pressures remained similar to the values in a resting condition. This proved that the inferior vena cava return was well-preserved, and no venous hypertension was present in the inferior vena cava district even after stress test (good exercise tolerance).Conclusion: One and one half ventricle repair can be considered a good surgical strategy for maintaining a low pressure in the inferior vena cava district with potential for right ventricle growth, restoring the more physiological circulation in borderline or failing right ventricle conditions. The experiment presented a positive finding in favour of one and one half ventricle repair, as compared to Fontan type procedure.

Transcatheter pulmonary valve perforation using chronic total occlusion wire in pulmonary atresia with intact ventricular septum

Background:Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies.Objective:To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation.Methods:This is a single-center, nonrandomized, retrospective study conducted during June 2008 to September 2015. Twenty-four patients with PA IVS were selected for the procedure during the study period. The median age and weight of the study population were 8. days and 2.65 kg, respectively. Four patients were excluded after right ventricular angiogram as they showed right ventricular-dependent coronary circulation. The pulmonary valve perforation was attempted using various types of CTO wires based on the tip load with variable penetrating characteristics.Results:The procedure was successful in 16 of twenty patients using CTO wires: Shinobi in nine, Miracle in four, CROSS-IT in two, and Conquest Pro in one. Two patients had perforation of right ventricular outflow tract (RVOT). Pericardiocentesis was required in one patient to relieve cardiac tamponade. Later, the same patient underwent successful hybrid pulmonary valvotomy. The other patient underwent ductus arteriosus (DA) stenting. Balloon atrial septostomy was needed in three cases with systemic venous congestion. Desaturation was persistent in five cases necessitating DA or RVOT stenting to augment pulmonary blood flow. There were two early and two late deaths. The mean follow-up was 22.66 ± 16 months. Three patients underwent one and half ventricle repair and one Blalock–Taussig shunt during follow-up.Conclusion:Perforation of the pulmonary valve can be done successfully using CTO wires in selected cases of pulmonary atresia with intact ventricular septum.

Biventricular repair versus uni-ventricular repair for pulmonary atresia with intact ventrical septum: A systematic review.

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.

Neonatal Pulmonary Artery Reconstruction During Shunting to Treat and Prevent Juxtaductal Coarctation

Coarctation of the pulmonary artery may lead to its early obstruction. We investigated the outcomes of a strategy of aggressive reconstruction of the pulmonary arteries in the neonatal period. From 2000 to 2013 a total of 162 consecutive neonates (< 90 days) underwent systemic-to-pulmonary shunting. Twenty-eight of them underwent the following concomitant reconstruction of the central pulmonary arteries: end-to-end anastomosis (4), patch reconstruction of the pulmonary arteries (24) with 0.4 mm Gore-Tex (W. L. Gore & Associates, Newark, DE) patch (18), or autologous pericardium (6). Ten patients were directed to univentricular palliation and 18 to biventricular repair. There was 1 in-hospital death (4%) and 2 deaths after hospital discharge (inter-stage mortality, 7%). The follow-up of the 25 survivors was complete. After a mean of 3 ± 3 years, patients with single ventricle palliation reached the following stages: shunts (2); one and a half ventricle repair (1); bidirectional cavopulmonary shunt (4); and Fontan (2). Fourteen of the patients destined for biventricular physiology reached complete repair while 2 patients were still with shunts. There was no pulmonary artery occlusion. Focal narrowing or pulmonary artery hypoplasia was the main indication for 10 of the subsequent 36 reinterventions. Neonatal pulmonary artery reconstruction effectively prevents pulmonary artery occlusion and warrants pulmonary artery growth in the majority of cases of juxtaductal pulmonary artery coarctation. A number of these patients needed enlargement of their central pulmonary arteries in subsequent procedures. Indications of this reconstruction at the time of systemic-to-pulmonary shunting remains to be specified.