Unbalanced Ventricles Research Articles

Repair of the complete atrioventricular septal defect-impact of postoperative moderate or more regurgitation.

To study the risk factors for mortality, moderate or more left atrioventricular valve regurgitation (LAVVR) and reoperation after the surgical repair of complete atrioventricular septal defect (cAVSD) in a single centre. The current study is a retrospective review of patients who underwent surgical repair of cAVSD between 2000 and 2021. Patients with unbalanced ventricles not amenable to biventricular repair, double outlet right ventricle and malpositioned great arteries were excluded. The clinical predictors of outcome for end points were analysed with univariate and multivariable Cox regression analysis or Fine-Gray modelling for competing risks. Time-dependent end points were estimated using the Kaplan-Meier curve analysis and cumulative incidence curves. The median follow-up time was 2.3 years. Among 220 consecutive patients were 10 (4.6%) operative and 21 late mortalities (9.6%). A total of 26 patients were identified to have immediate postoperative moderate or more regurgitation and 10 of them ultimately died. By multivariable analysis prematurity and having more than moderate regurgitation immediately after the operation were identified as predictors of overall mortality (P = 0.003, P = 0.012). Five- and ten-year survival rates were lower for patients with immediate postoperative moderate or more LAVVR {51.9% [confidence interval (CI): 27.5-71.7%]} when compared to patients without moderate or more regurgitation [93.2% (CI: 87.1-96.4%) and 91.3% (CI: 83.6-95.5%)]. The patients who undergo cAVSD repair remain subjected to a heavy burden of disease related to postoperative residual LAVVR. Immediate postoperative moderate or more LAVVR contributes significantly to overall mortality. Whether a second run of bypass can decrease this observed mortality should be investigated.

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

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

Single-ventricle palliation in children with atrioventricular septal defect and transposition of the great arteries: 45 years of experience.

The association of atrioventricular septal defect and transposition of the great arteries is very rare. As a rule, these patients have unbalanced ventricles. However, there have been no studies describing the results of single-ventricle palliation in these children. All children who underwent surgery with a diagnosis of atrioventricular septal defect and transposition of the great arteries were included in the study. Data were obtained from medical records. A total of 38 patients with atrioventricular septal defect and transposition of the great arteries underwent single-ventricle palliation at the study institution between 1971 and 2016. The mean follow-up was 12.4 years (median: 14.6 years, range 2-43.3 years). Most children had unbalanced atrioventricular septal defect (94.7%, 36/38). Survival was 67.6% (95% confidence interval [CI]: 50.0-80.2%) at 10 years and 57.8% (95% CI: 38.0-73.4%) at 20 years. By 10 years, 58.6% (95% CI: 40.8-72.7%) had progressed to Fontan completion, while 32.5% (95% CI: 18.2-47.6%) had died. In patients achieving Fontan completion, 20-year event-free survival was 73.3% (95% CI: 34.8-91.3%), while 5.0% (95% CI: 0.4-20.5%) had undergone cardiac transplantation and 21.7% (95% CI: 3.2-50.8%) had undergone takedown of the Fontan circulation. Freedom from atrioventricular valve surgery was 57.0% (95% CI: 37.2-72.7%) at 10 and 20 years. The association of atrioventricular septal defect and transposition of the great arteries is very rare, and most of these children have unbalanced ventricles. Single-ventricle palliation results in 25-year overall survival of 50%. However, in patients, who had Fontan completion, survival was 75% at 25 years after Fontan operation.

Double orifice and atrioventricular septal defect: dealing with the zone of apposition†.

A double orifice of the left atrioventricular valve (LAVV) associated with atrioventricular septal defects (AVSD) can significantly complicate surgical repair. This study reports our experience of AVSD repair over 3 decades, with special attention to the zone of apposition (ZoA) of the main orifice, and presents a technique of hemivalve pericardial extension in specific situations. We performed a retrospective study from 1987 to 2016 on 1067 patients with AVSD of whom 43 (4%) had a double orifice, plus 2 additional patients who required LAVV pericardial enlargement. Median age at repair was 1.3 years. Mean follow-up was 8.2 years (1 month-32 years). Associated abnormalities of the LAVV subvalvular apparatus were found in 7 patients (5 parachute LAVV and 2 absence of LAVV subvalvular apparatus). ZoA was noted in 4 patients (9%): partially closed in 15 (35%) and completely closed in 24 (56%). Four patients required, either at first repair or secondarily, a hemivalve enlargement using a pericardial patch without closure of the ZoA. The early mortality rate was 7% (n = 3), all before 2000. Two patients had unbalanced ventricles and the third had a single papillary muscle. There were no late deaths. Six patients (14%) required 7 reoperations (3 early and 4 late reoperations) for LAVV regurgitation and/or dysfunction, of whom 4 (9%) required mechanical LAVV replacement (all before 2000). Freedom from late LAVV reoperation was 97% at 1 year, 94% at 5 years and 87% at 10, 20 and 30 years. Unbalanced ventricles (P = 0.045), subvalvular abnormalities (P = 0.0037) and grade >2 LAVV postoperative regurgitation (P = 0.017) were identified as risk factors for LAVV reoperations. Freedom from LAVV mechanical valve replacement was 95% at 1 year, 90% at 5 years and 85% at 10, 20 and 30 years. An anomalous LAVV subvalvular apparatus was identified as a risk factor for mechanical valve replacement (P = 0.010). None of the patients who underwent LAVV pericardial extension had significant LAVV regurgitation at the last follow-up examination. Repair of AVSD and double orifice can be tricky. Preoperative LAVV regurgitation was not identified as an independent predictor of surgical outcome. LAVV hemivalve extension appears to be a useful and effective alternate surgical strategy when the ZoA cannot be closed.

Reoperations for left atrioventricular valve dysfunction after repair of atrioventricular septal defect

Postoperative left atrioventricular valve (LAVV) dysfunction is known to be the principal risk factor influencing outcome after repair of all types of atrioventricular septal defect (AVSD). The purpose of the present study was to identify the risk factors for reoperation and to assess the outcomes after reoperation for LAVV dysfunction. Records of 412 patients who underwent anatomical repair for different types of AVSD from January 2000 to July 2012 were reviewed. The study group (n = 60) included 13 additional patients for whom repair ± LAVV reoperation was performed in a primary institution. Outcomes, independent risk factors, reoperation and death were analysed. There were 7 early, (1.7%) and 1 late death. Forty-seven (11.4%) required 64 reoperations for LAVV dysfunction. The median delay for the first LAVV reoperation was 3.5 months (range: 5 days to 10.0 years). Unbalanced ventricles with small left ventricle [odds ratio (OR) = 4.06, 95% confidence interval (CI): 1.58-10.44, P = 0.004], double-orifice LAVV (OR = 5.04, 95% CI: 1.39-18.27, P = 0.014), prior palliative surgery (OR = 3.5, 95% CI: 1.14-10.8, P = 0.029) and discharge echocardiography documenting LAVV regurgitation grade >2 (OR = 21.96, 95% CI: 8.91-54.09, P < 0.001) were found to be independent risk factors for LAVV reoperation. Twelve-year survival and freedom from LAVV reoperation rates were, respectively, 96.1% (95% CI: 94.1-98.1) and 85.8% (95% CI: 81.3-90.3). Survival was significantly worse in patients who underwent LAVV reoperation (P < 0.001) and in those who underwent valve replacement vs valve repair (P = 0.020). After AVSD repair, LAVV dysfunction appears to be the principal factor that influences outcome. It can usually be managed by repair. Need for multiple reoperations is not uncommon. Long-term outcome in patients with repaired LAVV is favourable.

Complete atrioventricular septal defect: Outcome of pulmonary artery banding improved by adjustable device

We sought to evaluate pulmonary artery banding in infants with complete atrioventricular septal defects. From 2000 to 2009, 20 infants with complete atrioventricular septal defects underwent pulmonary artery banding because of unsuitable anatomy (unbalanced ventricles, associated lesions, or both) or clinical condition (infection, chronic lung disease, or noncardiac malformation). Patients were divided into 2 groups: the conventional PAB group (n = 13 [65%]; mean age, 74 ± 56 days [range, 6-187 days]; mean weight, 3.3 ± 1.1 kg [range, 2.1-5.8 kg]) and the FloWatch-PAB group (n = 7 [35%]; mean age, 111 ± 40 days [range, 81-187 days]; mean weight, 4.3 ± 1.2 kg [range, 3.2-6.1 kg]). There was no statistical difference in age or weight. Preoperative mechanical ventilation was required in 3 (23%) of 13 infants in the conventional PAB group and 5 (71%) of 7 infants in the FloWatch-PAB group (P < .05). Ten (77%) of 13 infants in the conventional PAB group died versus 0 (0%) of 7 infants in the FloWatch-PAB group (P < .001). Sternal closure was delayed in 6 (46%) of 13 infants in the conventional PAB group and 0 (0%) of 7 infants in the FloWatch-PAB group (P < .05). The mean duration of mechanical ventilation, intensive care unit stay, and hospital stay was significantly longer (P < .05) in the conventional PAB group than in the FloWatch-PAB group (21 ± 17 days [range, 4-61 days] vs 3 ± 2 days [range, 1-8 days], 22 ± 18 days [range, 5-61 days] vs 7 ± 6 days [range, 2-21 days], and 54 ± 12 days [range, 40-71 days] vs 29 ± 25 days [range, 9-81 days], respectively). Left atrioventricular valve regurgitation increased (mild to moderate) in 2 infants in the conventional PAB group and decreased (severe to moderate) in 2 infants in the FloWatch-PAB group. Six of 10 survivors (1 in the conventional PAB group and 5 in the FloWatch-PAB group) underwent pulmonary artery debanding and repair after a median interval of 125 days (range, 34-871 days); 4 of 10 are awaiting repair. In selected patients with complete atrioventricular septal defects, pulmonary artery banding followed by late repair is a viable alternative strategy. In our study the FloWatch-PAB device resulted in improved survival and made later repair possible in a better clinical state.

Double outlet right ventricle: Opinions regarding management

In the current era of superb surgical results for congenital heart disease, several management options are available for patients born with double outlet right ventricle (DORV). The surgical repair of DORV is tailored to address the variety of abnormalities associated with this lesion. The treatment strategies are dependent upon the anatomy and relationship of the ventricular septal defect (VSD) and the great arteries. For patients with subaortic or doubly committed VSDs, without right ventricular outflow tract obstruction, the usual repair is an intraventricular tunnel from the VSD to the aorta. If right ventricular outflow tract obstruction exists, then augmentation of the right ventricular outflow tract or conduit placement from the right ventricle to the pulmonary artery is necessary. For the "Taussig-Bing anomaly" (subpulmonary VSD) an arterial switch operation is indicated with tunneling of the VSD to the neoaorta. For patients with a remote VSD, complex atrioventricular valve abnormalities, or unbalanced ventricles, a staged palliative approach is warranted. This approach will result in the patient having "single ventricle" physiology, and "Fontan anatomy" after the staging surgeries. Outcomes are determined by the surgery performed. Patients who require conduit placement from right ventricle to pulmonary artery will need subsequent surgery for replacement of the conduit. Complex intraventricular tunnels are at risk for developing subaortic obstruction. Patients who had arterial switch operations have thus far had excellent results. However, these patients may be at risk for development of neoaortic valve regurgitation requiring subsequent reoperation. Complex biventricular intracardiac repairs have been associated with a higher risk of reoperation. In the past 10 to 15 years, patients undergoing Fontan operations have had improved early and late survival. However, long-term problems after the Fontan operation include arrhythmia and development of protein-losing enteropathy.

Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome

Background Several studies have shown that Down's syndrome is not a risk factor for biventricular repair of complete atrioventricular septal defects. However, few data are available about the comprehensive outcome of all the cardiac surgical procedures in patients with trisomy 21, including palliative surgery. Methods This is a retrospective study of 206 consecutive patients who underwent cardiac surgery from January 1992 to January 2002. Data about mortality and morbidity were analyzed and the impact of Down's syndrome was evaluated. Results Overall mortality was 7.7%. Actuarial survival was 94% among patients with Down's syndrome versus 86% of the group with normal karyotype ( p = 0.12). The presence of unbalanced ventricles was the only independent risk factor affecting survival at multivariate analysis ( p < 0.0001). The need for a Norwood type surgery was more frequent among non-Down patients (12.0% vs 1.5%, p = 0.02) as was the prevalence of pulmonary artery banding operations (22.9% vs 9.3%, p = 0.04). Cumulative mortality after palliation was higher in non-Down patients (44% vs 2.9%, p = 0.0001). Freedom from reoperation was lower in the group with normal chromosomes in respect to patients with Down's syndrome (81.4% vs 94.6%, p = 0.04), due to the higher prevalence of anomalies of the mitral valve (4.9% vs 1.8%, p = 0.03) or left ventricular outflow tract (7.3% vs 0%, p = 0.01). Conclusions Down patients showed a decreased risk for biventricular repair and lower mortality and morbidity in cases of complex cardiac malformations requiring complex palliative operations.

Remote control of pulmonary blood flow: a dream comes true.

The indication for pulmonary artery banding is currently limited by several factors. Previous attempts have failed to produce adjustable pulmonary artery banding with reliable external regulation. An implantable, telemetrically controlled, battery-free device (FloWatch) developed by EndoArt SA, a medical company established in Lausanne, Switzerland, for externally adjustable pulmonary artery banding was evaluated on minipigs and proved to be effective for up to 6 months. The first human implant was performed on a girl with complete atrioventricular septal defect with unbalanced ventricles, large patent ductus arteriosus and pulmonary hypertension. At one month of age she underwent closure of the patent ductus arteriosus and FloWatch implantation around the pulmonary artery through conventional left thoracotomy. The surgical procedure was rapid and uneventful. During the entire postoperative period bedside adjustments (narrowing or release of pulmonary artery banding with echocardiographic assessment) were repeatedly required to maintain an adequate pressure gradient. The early clinical results demonstrated the clinical benefits of unlimited external telemetric adjustments. The next step will be a multi-centre clinical trial to confirm the early results and adapt therapeutic strategies to this promising technology.

Remote control of pulmonary blood flow: a dream comes true.

The indication for pulmonary artery banding is currently limited by several factors. Previous attempts have failed to produce adjustable pulmonary artery banding with reliable external regulation. An implantable, telemetrically controlled, battery-free device (FloWatch) developed by EndoArt SA, a medical company established in Lausanne, Switzerland, for externally adjustable pulmonary artery banding was evaluated on minipigs and proved to be effective for up to 6 months. The first human implant was performed on a girl with complete atrioventricular septal defect with unbalanced ventricles, large patent ductus arteriosus and pulmonary hypertension. At one month of age she underwent closure of the patent ductus arteriosus and FloWatch implantation around the pulmonary artery through conventional left thoracotomy. The surgical procedure was rapid and uneventful. During the entire postoperative period bedside adjustments (narrowing or release of pulmonary artery banding with echocardiographic assessment) were repeatedly required to maintain an adequate pressure gradient. The early clinical results demonstrated the clinical benefits of unlimited external telemetric adjustments. The next step will be a multi-centre clinical trial to confirm the early results and adapt therapeutic strategies to this promising technology.

Biventricular repair of transposition of the great arteries and unbalanced ventricles

Background: It is well established that the arterial switch operation is the surgical procedure of choice in patients with transposition of the great arteries and balanced ventricular anatomy. The surgical approach of choice in patients with transposition but unbalanced ventricular size is unknown. Objectives: Since the beginning of the arterial switch operation program, patients with transposition of the great arteries and unbalanced ventricles underwent biventricular repair by means of the arterial switch operation and repair of any associated lesions, either through a single or staged surgical procedure. The aim of this retrospective study is to analyze whether this approach can be proposed to such patients. Methods: Forty-four patients with transposition of the great arteries and unbalanced ventricles underwent this surgical approach since 1984. Two groups were defined: group I had transposition with a dominant right ventricle (n = 28), and group II had transposition with a dominant left ventricle (n = 16). In group I the median age and weight at the arterial switch operation were 8.5 days (range, 5-70 days) and 3.1 kg (range, 1.5-3.7 kg), respectively. The median end-diastolic left ventricular volume, mass, and long-axis ratio were 15 mL/m2 (range, 11-16 mL/m2), 31.5 g/m2 (range, 20-66 g/m2), and 0.85 (range, 0.9-0.7), respectively. The mitral valve diameter was slightly hypoplastic, with a median z value of –1.22 (range, –0.3 to 3.7). In group 2 the median age and weight at the arterial switch operation were 42 days (range, 8 days–15 years) and 3.5 kg (range, 2.8-35 kg), respectively. Associated lesions in this group were coarctation in 9 and single (n = 12) or multiple (n = 4) ventricular septal defects. The median long-axis ratio and tricuspid z value were 0.6 (range, 0.3-0.8) and –0.9 (range, –0.5 to 3.3), respectively. In this group 9 patients had a single-stage procedure with fenestrated ventricular defect patches, atrial septal defect patches, or both; 7 patients underwent the staged approach. Results: In group I there was 1 early death from sepsis after weaning from postoperative extracorporeal membrane oxygenation. Three patients had severe pulmonary hypertension, one of whom died 1 year later. All survivors demonstrated, at discharge from the hospital, equilibrated ventricular size, with a median left ventricular end-diastolic volume of 25 mL/m2 (range, 21-30 mL/m2). In group II there were 2 early and 1 late deaths. All early deaths occurred in patients without voluntary residual intracardiac shunts. Median early postoperative long-axis ratio and tricuspid z value were 0.8 (range, 0.7-1) and –0.2 (range, 0.74 to 1.2), respectively. Conclusion: This study demonstrates that the arterial switch operation in patients with transposition of the great arteries and unbalanced ventricles remains a good surgical option.

985-55 Surgical Repair of Double Outlet Right Ventricle with Non-committed Ventricular Septal Defect

Between January 1978 and June 1993, 32 patients presented for management of double outlet RV with non-committed VSD. 29 patients had definitive palliation or repair with 3 early and 1 late deaths (hospital mortality: 10.4%). The VSD type was muscular in 4, inlet in 12 and perimembranous but distant from both great arteries in 16 patients. Complicating anomalies with a major influence on management were present in 23 children (72%). These included aortic arch interruption (3), coarctation of the aorta (4), pulmonary stenosis (11), pulmonary atresia (2). multiple VSOs (3), AV discordance (2), left atrial isomerism (3), unbalanced ventricles (8) and criss-cross AV connection (2) with 11 children having more than one of these lesions. Initial palliation was performed in 20 patients and consisted of pulmonary artery banding in 8 (with repair of an interrupted aortic arch in 2), systemic-pulmonary artery shunt in 9 and repair of coarctation of the aorta in 3. Of these, 17 subsequently had definitive surgery: Fontan procedure in 9 and biventricular repair in 8 (arterial switch in one, intraventricular baffle in 7). 3 patients died following attempted biventricular repair. 12 patients underwent repair as the initial procedure: Fontan procedure in 2 and biventricular repair in 10 (arterial switch in 3 and intraventricular baffle in 7) with no deaths. Thus, of the 29 patients who had definitive surgery, 11 had Fontan procedures and 18 biventricular repair with no death in the Fontan group and 3 early deaths in the biventricular repair group (not statistically significant by Fischer's exact test, p = 0.27). Only 2 patients have required reoperations, both for residual VSDs. 9 of the Fontan procedures were undertaken in patients who had complex anatomy (e.g. AV discordance, unbalanced ventricles, multiple VSDs, atrial isomerism or pulmonary atresia), whereas 14 children of the biventricular repair group had non-complex anatomy (p &lt; 0.005). Surgery for double outlet RV with non-committed VSD needs to be tailored to individual patients, but biventricular repair was achieved in a substantial majority of patients with non-complex anatomy with relatively low risk and a low incidence of reoperations.

Atrioventricular Canal Defects: Results of Repair in the Current Era

Between December 1986 and December 1990, 37 consecutive patients underwent repair of complete atrioventricular (AV) canal with the two-patch technique. Mean age at repair was 22 months and 51% were less than 1 year of age. Eighteen (48.6%) had undergone previous palliative operations. Two operative deaths (5.4%) occurred and another patient died in-hospital for an early mortality of 8.1%. One late death (2.9%) has occurred from a respiratory infection. Actuarial survival is 87.7% at 3 years. Small size (p less than 0.05), unbalanced ventricular size (p less than 0.05), New York Heart Association (NYHA) Class IV (p less than 0.05), and severe preoperative AV valve insufficiency (p less than 0.05) were significant preoperative risk factors for death. Five survivors (14.7%) required reoperation for severe AV valve insufficiency (two) or patch leaks (three). The risk for reoperation was increased in non-Down's patients (p less than 0.02). All survivors are in NYHA Class I (93%) or II (7%). The risk for early AV valve insufficiency was increased in patients who did not have the cleft sutured (p less than 0.05), and in those with unbalanced ventricles (p less than 0.01). Risk of late AV valve insufficiency was increased only by small size (p less than 0.02). Previous pulmonary artery banding did not increase the risk of repair or of postoperative AV valve insufficiency. Complete AV canal can be repaired with low mortality. Pulmonary artery banding may still have a role to play in the very small (4-5 kg) infant in refractory heart failure.