The Great Arteries With Intact Ventricular Septum Research Articles

Two-Stage Arterial Switch for Transposition of the Great Vessels in Older Children

BackgroundThis study investigated a 2-stage arterial switch operation (ASO) to treat transposition of the great arteries (TGA) with intact ventricular septum (TGA-IVS) in late referral patients. MethodsWe retrospectively analyzed patients with TGA-IVS or TGA with restricted ventricular septal defects who had undergone 2-stage ASO at our institution from February 2007 to August 2018. Included were 41 patients: 21 (51.2%) who had undergone long-term 2-stage ASO and 20 (48.8%) who had undergone rapid 2-stage ASO. ResultsThe long-term 2-stage group was older at ASO (3.5 vs 25 months; P < .001). Results were more satisfactory in the long-term group than in the rapid group for intensive care unit time (P = .004), mechanical ventilation time (P = .004), and length of stay (P = .007). No in-hospital death occurred in the long-term group, and the postoperative course was more manageable in the long-term group than in the rapid group. However, the risk of significant neoaortic regurgitation was lower in the rapid group, which also had a better left ventricular ejection fraction. ConclusionsThe long-term group achieved better early-term outcomes than the rapid group. However, a high risk of neoaortic regurgitation and myocardial dysfunction was also noted.

Comprehensive Functional Echocardiographic Assessment of Transposition of the Great Arteries: From Fetus to Newborn.

To improve the understanding of the pathophysiology of transposition of the great arteries with intact ventricular septum (TGA/IVS) and the cardiac remodeling occurring from fetal to neonatal life, we performed a morphometric and functional echocardiographic assessment in fetuses and newborns. This was a prospective case-control study performed in a tertiary referral center, which included fetuses and newborns with a diagnosis of TGA/IVS between 2011 and 2018. Morphometry and systolic and diastolic function parameters were compared with age and body surface-matched healthy controls. Twenty-one TGA/IVS patients were included during the study period and morphometric and functional echocardiographic data were recorded. TGA/IVS patients showed morphometric and functional changes of increased overall volume and output, predominantly in the aortic component from fetus to newborn, probably due to compensatory mechanisms secondary to brain hypoxia.

Anatomical Classifications of the Coronary Arteries in Complete Transposition of the Great Arteries and Double Outlet Right Ventricle with Subpulmonary Ventricular Septal Defect.

Objective To discuss the anatomical morphologies of the coronary arteries and frequencies of unusual coronary arteries in complete transposition of the great arteries and double outlet right ventricle (DORV) associated with a subpulmonic ventricular septal defect (VSD). Methods Between March 1999 and August 2012, 1,078 patients with complete transposition of the great arteries or DORV with subpulmonary VSD underwent arterial switch operations (ASOs) and were visually evaluated to classify their coronary artery morphology during open heart surgery. Results The coronary arteries could be classified into five patterns with several subtypes. Unusual coronary arteries were observed in 248 of the 1,078 cases, providing a frequency of 23.01%. The frequencies of the patients with transposition of the great arteries with intact ventricular septum (TGA/IVS), TGA/VSD, and DORV with subpulmonary VSD were 17.65, 23.28, and 31.84%, respectively. The most common morphologies were the right coronary artery (RCA) originating from sinus 1 and circumflex (CX) originating from sinus 2 (1R, AD; 2CX; 26.50%); the CX originating from sinus 2 (1AD; 2R, CX; 21.36%); the RCA, left anterior descending artery, and CX originating from single sinus 2 (2R, AD, CX; 13.24%). The in-hospital mortalities of the patients with or without unusual coronary arteries after ASO were 14.1 and 6.02%, respectively. Conclusion Patients with complete transposition of the great arteries or DORV with subpulmonary VSD have a high frequency of unusual coronary arteries, which might greatly impact on the mortality for ASO. Improving the preoperative diagnostic criteria for coronary artery morphology may significantly increase the success rate for ASOs.

Abstract 216: Leveraging the First Statewide Congenital Cardiac Consortium to Reduce Variation in Modifiable Preoperative Management Among Neonates Undergoing Arterial Switch Operation

Objective: Seven public and private California cardiac centers have joined to form the first statewide consortium created to improve the quality and value of congenital cardiac care. The overall goals of the consortium are: 1) to understand variation in modifiable perioperative processes of care among participating hospitals, and 2) to determine whether standardization of those process measures will reduce adverse events and cost. In this pilot study, preoperative management of neonates undergoing arterial switch operation (ASO) within one center was analyzed for variation and these process measures were then associated with increased inpatient costs and length of stay. Methods: Through retrospective analysis of neonates undergoing ASO for Transposition of the Great Arteries with Intact Ventricular Septum (TGA/IVS) (n=34) at one center between 2004-2014, variation within the following preoperative process measures was described: feeding protocols, ventilation management, use of balloon atrial septostomy (BAS) and prostaglandin, and timing of surgery. Independent review by echocardiologists (SS, SP, AMG) confirmed preoperative and discharge diagnoses. Risk factors for increased total hospital length of stay (TLOS), postoperative length of stay (PLOS) and inpatient hospital costs were analyzed by univariable linear regression. Results: The population (N=34) was predominantly male (80%, 27/34) with low overall prenatal diagnosis rate (27%, 9/34). Birth weight was 3295±521 grams and gestational age (GA) was 39.1±1.4 weeks. Variation was found in duration of preoperative hospital stay (7.1±3.7 days), proportion of preoperative stay spent in the Cardiac ICU (0.58±0.35), use of BAS (53%, 18/34), preoperative enteral feeding (50%, 17/34) and preoperative extubation (24%, 8/34). Increased birth weight was negatively associated with costs (p=0.01), TLOS (p=0.14) and PLOS (p=0.02). Delayed timing of surgery was positively associated with TLOS (p=0.0004) and total costs (p=0.01) but not PLOS (p=0.93). Preoperative Cardiac ICU management trended towards negative association with total costs (p=0.20) but not TLOS (p=0.35) or PLOS (p=0.99) when compared with preoperative Neonatal ICU management. Lower GA, preoperative BAS, preoperative enteral feeding and preoperative extubation were not significantly associated with increased costs, TLOS, and PLOS. Conclusions: Variations in preoperative management of neonates undergoing ASO for TGA/IVS are significantly associated with total healthcare costs and hospital LOS. Concatenated data from the remaining consortium members will be critical to validate these findings and to power morbidity and mortality analysis. Integrated findings will allow the consortium to develop and implement consensus based recommendations regarding standardization of those process measures that will reduce major complications and cost.

Prenatal diagnosis of transposition of the great arteries over a 20-year period: improved but imperfect.

To evaluate temporal trends in the prenatal diagnosis of transposition of the great arteries with intact ventricular septum (TGA/IVS) and its impact on neonatal morbidity and mortality. We included in this study cohort newborns with TGA/IVS who were referred for surgical management to our center over a 20-year period (1992-2011). The study period was divided into five 4-year periods and the primary outcome was rate of prenatal diagnosis. Secondary outcomes included neonatal preoperative status and perioperative survival. Of the 340 patients with TGA/IVS, 81 (23.8%) had a prenatal diagnosis. The rate of prenatal diagnosis increased over the study period, from 6% in 1992-1995 to 41% in 2008-2011 (P < 0.001). Compared to patients with a postnatal diagnosis, balloon atrial septostomy (BAS) was performed earlier in patients with a prenatal diagnosis (0 days after delivery vs 1 day after delivery, respectively; P < 0.001) and fewer prenatally diagnosed neonates required mechanical ventilation (55.6% vs 68.0%; P = 0.03). Between patients with a prenatal or postnatal diagnosis of TGA/IVS, there were no statistically significant differences in the incidence of preoperative acidosis (16.0% vs 25.5%; P = 0.1), need for preoperative extracorporeal membrane oxygenation (2.5% vs 2.7%; P = 1.0) or mortality (one preoperative and no postoperative deaths among prenatally diagnosed patients compared with four preoperative and six postoperative deaths among postnatally diagnosed patients). The prenatal detection rate of TGA/IVS has improved but still remains below 50%, suggesting the need for strategies to increase detection rates. The mortality rate was not statistically significantly different between prenatally and postnatally diagnosed patients, however, there were significant preoperative differences with regard to earlier BAS and fewer neonates that required mechanical ventilation. Ongoing work is required to ascertain whether prenatal diagnosis confers long-term benefits.

Simple peritoneal dialysis in the treatment of severe congenital heart disease after operation complicated with low cardiac output syndrome in children

Objective To study the effect of simple peritoneal dialysis on the treatment of severe congenital heart disease(CHD) after operation complicated with low cardiac output syndrome(LCOS) in children with perioperative therapy. Methods After operation, fifty-three cases of severe CHD complicated with LCOS and the application of easy peritoneal dialysis were collected from Jan.to Jun.2013 in Department of Children's Surgical Intensive Care Specialist, Bayi Children's Hospital were collected.Functional parameters of vital organs, e.g.lung, kidney and heart were observed.Ages varied from 2 days to 2 years old, body weight from 2 to 8 kg.It contained 8 cases of complete transposition of great arteries with intact ventricular septum(TGA/IVS), 10 cases of Tetralogy of Fallot(TOF), 10 cases of pulmonary atresia(PA), 6 cases of interruption of aortic arch(IAA), totally anomalous pulmonary venous drainage(TAPVC) in 6 cases, 5 cases of coarctation of the aorta(COA), 4 cases with right ventricular double outlet(DORV), 3 cases with endocardial cushion defect(CAVC), 1 case with ventricular septal defect(VSD). Results Fifty cases were completely cured, postoperative simple peritoneal dialysis treatment for 2 to 10 days, living ICU treatment time 2-15 days, urine output returns to normal 1-3 days, serum creatinine, blood urea nitrogen recovery time after 3-10 days, lactic acid to normal time 1-3 days, and 3 cases complicated with severe LCOS received extracorporeal membrane oxygenation therapy for difficult recovery, and postoperative recovery was good.Three cases died. Conclusions Easy peritoneal dialysis is convenient, safe, minimally invasive, inexpensive and effective.It can improve survival rate and reduce complications, shorten the time of mechanical ventilation and ICU residence time, hospitalization time and cost savings. Key words: Congenital heart disease; Simple peritoneal dialysis; Low cardiac output syndrome; Child

Left ventricular torsion abnormalities in patients after the arterial switch operation for transposition of the great arteries with intact ventricular septum

BackgroundThe arterial switch operation (ASO) is currently the treatment of choice for infants with transposition of the great arteries (TGA). Little is known, however, about the alteration of anatomic left ventricular (LV) torsional mechanics after the operation. This study sought to evaluate LV torsion in patients of transposition of the great arteries with intact ventricular septum (TGA/IVS) using speckle tracking echocardiography. MethodsEchocardiographic images were prospectively acquired in 32 infants (age range, 0.5–60months) who successfully underwent ASO repair at about 1month of age and in 48 normal controls. They were divided into early and late categories according to the age at the time of the study. The LV peak systolic torsion and systolic twisting and diastolic untwisting velocities were determined by speckle tracking. Mitral inflow velocity obtained by Pulsed-wave Doppler and mitral annular velocities drawn by septal tissue Doppler were also analyzed. ResultsCompared with controls, the early postoperative group (TGA1) had significantly higher septal E/e′ (P=0.000). In contrast, septal e′ velocity (P=0.000), LV peak apical rotation (P=0.01), twist (P=0.02) and peak untwisting velocity (PUV) (P=0.001) were lower in patients than in controls. For the normal younger group (Control1), PUV correlated positively with e′ (r=0.68, P<0.001). No significant difference in LV twisting and untwisting was noted between the TGA2 and Control2. ConclusionsTwo dimensional speckle tracking echocardiography may sensitively detect impaired LV torsional mechanics in patients with TGA/IVS early after ASO, and the impairment of LV relaxation leads to increased LV filling pressure which is consistent with higher E/e′. However, all patients recovered well thereafter and the overall midterm outcome of ASO is satisfactory.

Evaluation of left ventricular geometry and function by echocardiography in patients after arterial switch operation

Objective To study the early and mid-term outcome in terms of left ventricular(LV)geometry and function in patients with transposition of great arteries with intact ventricular septum (TGA/IVS) undergoing arterial switch operation.Methods Twenty patients were followed up and divided into 2 groups according to age:the earlier group and the later group.Age and sexual matched controls were also designed as control group 1 and control group 2.LV posterior wall thickness(PWTd),dimension(LVIDd)and length(L) at end diastolic phase,LV end-diastolic volume (EDV),end-systolic volume (ESV),ejection fraction (EF),mitral valve early (E) and late (A) inflow velocities and E/A,isovolumic relaxation time (IVRT) were obtained by two-dimensional echocardiography,normalized isovolumic relaxation time (IVRTn),wall thickness index (PWTd/LVIDd) and LV geometry index(LVIDd/L) were calculated,and these indexes between groups were compared.Results ①Compared with control group 1,PWTd in earlier group was higher [(0.41 + 0.06)cm vs (0.36 + 0.05)cm,P =0.022],but LVIDd had no significant statistical difference [(2.08 + 0.21)cm vs (2.21 + 0.23)cm,P =0.117],indicating PWTd/ LVIDd differed between groups(0.20 + 0.04 vs 0.16 + 0.01 P =0.001).②No abnormalities were observed in later group.③Compared with different age groups in TGA,EDV,ESV,L,LVIDd,PWTd were significantly higher in later group(P =0.000).There were obvious correlations between EDV,ESV,L,LVIDd and examined age (r =0.90,0.83,0.73,0.90,P =0.000,R2 =0.809,0.694,0.528,0.808) while there were no significant correlation between PWTd and examined age(r =0.56,P =0.000,R2 =0.309).Conclusions Early after operation,LV is undergoing hypertrophy or hyperplasia in TGA/IVS,but will recover in the mid-term period,and the function is normal all the time indicating that the LV myocardium may avoided irreversible pathological changes if operated in time.All in all,LV develops well in mid-term period. Key words: Echocardiography; Ventricular function, left; Heart defects, congenital; Transposition of great arteries ; Arterial switch operation

ASSESSMENT OF LEFT VENTRICLE GEOMETRY AND FUNCTION PATTERN AFTER ARTERIAL SWITCH OPERATION FOR D-TRANSPOSITION OF THE GREAT ARTERIES WITH INTACT VENTRICULAR SEPTUM USING TWO-DIMENSIONAL ECHOCARDIOGRAPHY

ObjectivesThe aims of this report were to study the early and mid-term outcome in terms of LV geometry and function in patients with transposition of the great arteries with intact...

EVALUATION OF LEFT VENTRICULAR TORSION AFTER ARTERIAL SWITCH OPERATION FOR D-TRANSPOSITION OF THE GREAT ARTERIES WITH INTACT VENTRICULAR SEPTUM BY TWO-DIMENSIONAL SPECKLE TRACKING IMAGING

ObjectivesTo evaluate anatomic left ventricular twist and untwist mechanics in patients of transposition of the great arteries with intact ventricular septum (TGA/IVS) up to 4 years after arterial switch operation...

Echocardiographic study for the children with transposition of the great arteries with intact ventricular septum after neonatal period

Objective To identify the suitable echocardiographic predictor,the standard 2-demensional echocardiography and speckle tracking imaging (STI) techniques were applied for the late referred children with transposition of great arteries with intact ventricular septum (TGA-IVS) after neonatal period.Methods 22 children with TGA-IVS after neonatal period were enrolled.According to the intraoperative left ventricular (LV)/right ventricular systolic pressure ratio,they received pulmonary artery banding (PAB group,n =10) or arterial switch operation (ASO group,n =12).15 body surface area matched healthy children were served as control group,Echocardiography was performed before the operation.The dynamic images in basal and apical short axis view were collected respectively.Meanwhile,using the STI technique,the rotation curves at apex and base was analyzed.Results All the patients discharged eventually.A right to left bulging of the ventricular septum was observed in all the patients by the 2D short-axis view.There were no differences in wall thickness and ejection fraction between control,PAB and ASO group.Compared with the control group,the end diastolic diameter,end diastolic volume and indexed LV mass in PAB and ASO group were significantly reduced,however,no difference in the end diastolic diameter and volume and LV mass was observed between the PAB and ASO group.The hypoplasia of LV in PAB and ASO group was associated with an extended time to peak basal rotation and reduced basal rotation and global torsion.Furthermore,the shorter time to peak apical rotation and reduced apical rotation and global torsion were only observed in PAB group.Conclusions The standard echocardiography could be useful for the initial evaluation for regressed LV of the children with TGA-IVS.However,STI allows a much more precious approach to the assessment of LV regressing after neonatal period.The apical rotation and global torsion might be the better predictor to identify the borderline or regressed LV for the surgical management for the children with TGA-IVS. Key words: Echocardiography; Ventricular function,left; Heart defects,congenital; Transposition of the great arteries; Left ventricular retraining; Speckle tracking imaging

Late Primary Arterial Switch for Transposition of the Great Arteries With Intact Ventricular Septum in an African Population

The arterial switch operation (ASO) is the optimal management of transposition of the great arteries with intact ventricular septum (TGA-IVS) within the first 3 weeks of life; beyond this age optimal treatment is debatable. The authors adopted a strategy of primary ASO for TGA-IVS in the first 10 weeks of life regardless of left ventricular (LV) status. This report reviews the early outcomes with this management approach. Between August 2006 and December 2009, 22 patients with TGA-IVS underwent the primary ASO. Sixteen of them were less than 21 days old (early switch group) and 6 were between 31 and 66 days old (late switch group). A review of their hospital records was performed to determine outcomes in the 2 groups. Operative variables and postoperative outcomes were recorded. There was 1 hospital death in the early switch group (6.3%) but none in the late group (0%). Temporary mechanical circulatory support was required in 1 patient (6.3%) in the early switch group and in 2 of the 6 (33.3%) in the late switch group. One late death of undetermined cause occurred in the late switch group 8 weeks after discharge. No significant difference could be demonstrated between the 2 groups in terms of operative variables and the measured postoperative outcomes. It is concluded that the age limit for the primary ASO can be extended to at least 10 weeks; temporary mechanical circulatory support may be required as a rescue.

Primary arterial switch operation for transposition of the great arteries with intact ventricular septum - is it safe after three weeks of age?

In patients with transposition of the great arteries with intact ventricular septum (TGA-IVS), late presentation for surgical treatment is not uncommon. Earlier experience suggested an excess mortality for the primary arterial switch operation (ASO) when performed after the third week of life in such patients. The true age limit for the primary ASO, however, remains speculative. A best evidence topic was written to determine the safety of extending the age limit of the primary ASO for TGA-IVS beyond three weeks of age. Eight of 74 relevant papers constituted the best evidence to address the issue. Sufficient level II evidence was found to suggest that in experienced institutions in the current era, the ASO for TGA-IVS may be performed primarily in infants three to eight weeks of age with comparable early outcomes to younger patients. Kang et al. investigated 275 neonates (younger than 21days) and 105 infants (age range 21-185days) undergoing the primary ASO for TGA-IVS. They found no significant difference in terms of in-hospital mortality (5.5% vs. 3.8%) or need for mechanical left ventricular (LV) support (3.6% vs. 5.7%) between the younger and older groups, respectively. Of the younger group, 3.8% developed postoperative LV failure leading to death or mechanical LV support. All nine patients older than two months (age range 61-185days) survived to discharge although two (aged 69 and 86days) required mechanical LV support for postoperative LV failure. The postoperative course of the late ASO group, however, was significantly prolonged as reflected in the duration of postoperative ventilation (4.9 vs. 7.1days, P=0.012) and length of postoperative stay (12.5 vs. 18.9days, P=0.001). In the report of Sarris et al., 52 patients with TGA-IVS who were older than four weeks of age (36 were older than eight weeks) underwent a primary ASO with early mortality comparable with younger patients (2% vs. 3%, respectively). For infants between the ages of two and six months, the strength of the evidence favoring this management approach is limited by the small sample size of reported series and the increased requirement for mechanical circulatory support for postoperative LV failure.

Persistent pulmonary hypertension of the newborn with transposition of the great arteries: successful treatment with bosentan

Persistent pulmonary hypertension of the newborn (PPHN) occurs in 1-4% of neonates with transposition of the great arteries with intact ventricular septum (TGA/IVS). This association is often lethal. To our knowledge, only eight survivors have been described in the literature, two of whom benefited from extracorporeal membrane oxygenation (ECMO). We report two cases of PPHN complicating a TGA/IVS that were refractory to multiple therapies and resolved 48 hours after initiation of bosentan therapy. Bosentan, an oral dual endothelin-1 receptor antagonist, is a new treatment for pulmonary arterial hypertension that was both effective and safe in these two cases of TGA/IVS with PPHN. To our knowledge, it is the first use of bosentan in newborns.

The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study☆

To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2). LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three). LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.