Ross-Konno Operation Research Articles

The Fate of the Left Ventricular Outflow Tract Following Interrupted Aortic Arch Repair.

Objectives: To examine the probability of left ventricular outflow tract (LVOT) reintervention following interrupted aortic arch (IAA) repair in neonates with LVOT obstruction (LVOTO) risk. Methods: This retrospective multicenter study included 150 neonates who underwent IAA repair (2003-2017); 100 of 150 (67%) had isolated IAA repair (with ventricular septal defect closure) and 50 of 150 (33%) had concomitant LVOT intervention: conal muscle resection (n = 16), Ross-Konno (n = 7), and Yasui operation (n = 27: single-stage n = 8, staged n = 19). Demographic and morphologic characteristics were reviewed. Factors associated with LVOT reoperation were explored using multivariable analysis. Results: Concomitant LVOT intervention was more likely in neonates with type B IAA, bicuspid aortic valve, aberrant right subclavian artery, smaller aortic valve annulus, and ascending aorta dimensions. On follow-up, five-year freedom from LVOT reoperation was highest following Ross-Konno (100%), 77% following Yasui (mainly for neo-aortic regurgitation), 77% following isolated IAA repair (mainly for LVOTO), and 47% following IAA repair with concomitant conal resection, P = .033. While all patients had low peak LVOT gradient at time of discharge, those who had conal resection developed higher gradients on follow-up (P = .007). Ross-Konno and Yasui procedures were associated with higher right ventricular outflow tract (RVOT) reoperation. In the cohort following isolated IAA repair, aortic sinus Z score was associated with LVOT reoperation. Conclusions: Both Yasui and Ross-Konno operations effectively mitigate late LVOTO risk. The highest risk of reintervention for LVOTO was associated with conal muscle resection while the lowest risk is associated with Ross-Konno. The RVOT reoperation risk in patients who had Ross-Konno or Yasui does not seem to affect survival.

Innovation in surgical treatment of hypertrophic obstructive cardiomyopathy in children.

Hypertrophic cardiomyopathy is the second most common cardiomyopathy affecting children and adolescents and is the main cause of sudden death of young athletes. The natural prognosis of children with severe hypertrophic obstructive cardiomyopathy is not optimistic, and it is not uncommon for children with hypertrophic obstructive cardiomyopathy who do not respond to medication. Surgical treatment is often the only solution. Conventional surgical methods in the past include classic or modified extended Morrow operation, classic or modified Konno operation, and Ross-Konno operation. In recent years, with the development of minimally invasive surgery, various minimally invasive surgical methods have emerged endlessly. Because the incision of minimally invasive cardiac surgery is significantly smaller than that of traditional surgery, it causes less trauma, recovers quickly after surgery, and has the advantage of no difference in surgical effect compared with traditional median sternotomy. Tally endoscopic transmitral myectomy, RTM, minimally right thoracotomy, and other surgical methods have achieved encouraging results in adults and some older children with hypertrophic obstructive cardiomyopathy. The appearance of transapical beating-heart septectomy has brought the treatment of hypertrophic obstructive cardiomyopathy from the era of cardiopulmonary bypass and cardiac arrest to a new era of minimally invasive beating-heart surgery. In the past, there were few articles about the treatment of children with hypertrophic obstructive cardiomyopathy. This article reviewed the new progress and prognosis of surgical treatment of children with hypertrophic obstructive cardiomyopathy at home and abroad.

Left Ventricular Dysfunction Following the Ross-Konno Reconstruction is Independent of the Operation.

The Ross-Konno (RK) operation is a well-established surgical treatment for combined left ventricular outflow tract obstruction and aortic valve pathology in children. Prior study has demonstrated that mechanical and electrical dyssynchrony exist post-RK compared to normal controls. The purpose of this study was to evaluate myocardial function pre- and post-RK as defined by echocardiography.Patients undergoing the RK operation (n = 13; median age: 1.3years; range: 0.5-13.3years) were evaluated by echocardiography at defined timepoints: pre-RK, post-RK, 1-year post-RK, and 2years post-RK. Defined parameters of left ventricular performance were analyzed: systolic mechanical dyssynchrony (M-Dys), global left ventricular circumferential strain (GCS), and diastolic relaxation fraction (DRF). Patients with post-operative atrioventricular block (n = 6) were analyzed separately.No pre- versus post-RK differences existed in M-Dys, GCS, or DRF in patients both with and without post-RK atrioventricular block. Further, 1- and 2-year follow-up post-RK demonstrated significant heterogeneity in evaluated parameters of function with no pre- and post-RK differences in M-Dys, GCS, or DRF.Mechanical dyssynchrony exists post-RK reconstruction in both short- and long-term follow-up yet these echocardiographic parameters of ventricular performance are independent of the RK operation. Further study is, therefore, warranted to define causal relationships for observed short- and long-term ventricular dysfunction post-RK as the findings of the present study suggest a deleterious mechanism apart from the technical RK reconstruction.

Delayed biventricular repair of interrupted aortic arch with left ventricular outflow tract obstruction in 22q11.2 deletion syndrome: a case report.

In patients with critical left ventricular outflow tract obstruction but adequately sized ventricles, the treatment of choice is biventricular repair. Several options have been proposed, including neonatal Yasui or Ross-Konno operation. However, each of these procedures carries a high mortality risk, especially in syndromic neonates. Here, we report the case of a patient with 22q11.2 deletion syndrome and a diagnosis of interrupted aortic arch type B2, ventricular septal defect and left ventricular outflow tract obstruction. As a means to avoid high-risk neonatal surgery in this patient, we pursued a strategy of delayed biventricular repair involving initial hybrid Norwood palliation followed by a Yasui-type operation at 3months. Although this strategy turned out to be successful, proactive monitoring for the development of ductal stent stenosis during follow-up after the hybrid procedure remains crucial to prevent hemodynamic complications such as cardiac failure and systemic hypoperfusion.

The Ross and Ross-Konno Operation in Neonates and Infants

When replacement of the aortic valve in neonates and infants is required, the Ross-Konno procedure provides a high-quality left ventricular outflow with good mid-term durability. The procedural outcome is highly dependent on the pre-procedural condition of the neonate or infant. Most series contain substantial numbers of post-balloon valvotomy patients as emergent or urgent management of severe, acute regurgitation. In patients with compromised ventricular function, post-operative mechanical support may be required but is generally associated with a good outcome. The neonatal and infant autograft may perform better in the long term than un-supported autografts performed later in life. Early definitive or delayed treatment of left ventricular outflow tract pathology associated with aortic interruption increasingly involves an infant Ross-Konno. The procedure also has a role in the recruitment of small left ventricles. Concomitant mitral pathology or aortic obstruction magnifies complexity, but the operative approach to the Ross-Konno remains the same.

The Ross-Konno operation for neonates and infants with severe aortic incompetence following treatment for critical aortic stenosis.

Aortic valve stenosis in neonates and infants is associated with congestive cardiac failure, and balloon or surgical valvuloplasty provides relief of stenosis. Occasionally severe aortic insufficiency necessitates urgent aortic valve replacement. We reviewed our experience with the Ross-Konno procedure in patients <1 year. Between October 2013 and May 2020, 36 patients underwent balloon (34) or surgical (2) aortic valvuloplasty for aortic stenosis. Six patients subsequently underwent a Ross-Konno procedure. The median age at operation was 55 (27-116) days and weight was 4.25 (2.5-12) kg. All patients were in severe cardiac failure and had a small aortic annulus with Z-score -3.1 (-1 to -4.4). There were no early or late deaths. At the latest follow-up at 39 (13-60) months, ventricular function had improved in all patients and no patient was on anti-failure medication. On echocardiography, there wasno more than trivial aortic regurgitation and no left ventricular outflow tract obstruction. One patient required right ventricle to pulmonary artery conduit replacement and one patient had homograft stenting. Despite the severe preoperative haemodynamic compromise, the urgent Ross-Konno procedure was associated with excellent operative survival and recovery of ventricular function. The need for reintervention to the pulmonary conduit remains a cause for concern.

An Opportunity Lost: Meta-Analyses or Thoughtful Synthesis?

In this issue of The Annals of Thoracic Surgery, Rajab and colleagues1 performed a meta-analysis of 18 studies that describe outcomes after the Ross or Ross-Konno operation among neonates. The authors found a pooled “early” mortality rate of 24%, with a surprising higher mortality in more recently published studies. Late mortality was reported as 43%, which we infer (as it is not explicitly defined) is defined as any death beyond 30 days of the procedure. Not unexpectedly, there was wide variation in the reported early mortality, which is likely due to the experience of the center and the existence of concomitant mitral valve pathology or hypoplastic left-sided structures.

Good News or Bad News? Considering Physician and Patient Perspectives on Outcomes

In this issue of The Annals of Thoracic Surgery, Bové and colleagues1 from Belgium report long-term follow-up for a series of 137 children with congenital aortic valve disease who underwent a Ross-only or Ross-Konno operation using a root replacement technique.

The Pulmonary Autograft After the Ross Operation: Results of 25-Year Follow-Up in a Pediatric Cohort

Progressive autograft dilation and need for later reoperation remain major concerns of the Ross procedure. The study investigates the clinical outcome after the Ross operation, including a longitudinal analysis of autograft dimensions over 25 years. From November 1991 to April 2019, 137 patients underwent a Ross procedure at the University Hospitals of UCL (Université catholique de Louvain)-Brussels and Ghent. Inclusion criteria were less than or equal to 18 years of age and pulmonary autograft implantation by root replacement. Outcome focused on survival, reoperation rate, and autograft size evolution through linear mixed-model analysis. A Ross or Ross-Konno operation was performed in 110 (80%) and 27 (20%) patients at a median age of 10.4 (interquartile range [IQR], 4.7-14.3) years and 0.5 (IQR, 0.04-5.2) years, respectively. Overall 10-year and 20-year survival was 87% ± 3% and 85% ± 3%, respectively, but was 93% ± 3% for isolated Ross patients. Right ventricular outflow tract-conduit exchange was required in 20.3%, whereas autograft-related reoperation was performed in 14 (10.7%) patients at a median interval of 14 (IQR, 9-16) years, for aortic regurgitation (n= 2) and autograft dilation (n= 12). Autograft z-values increased significantly at the sinus and sinotubular junction (STJ) compared with the annulus (annulus= 0.05 ± 0.38/y, sinus= 0.14 ± 0.25/y, STJ= 0.17 ± 0.34/y; P= .015). The z-value slope for autograft dimensions was significantly steeper for Ross-Konno vs Ross patients (annulus: P= .029; sinus: P < .001; STJ: P= .012), and for children having aortic arch repair (annulus: P= .113, sinus: P= .038; STJ: P= .029). The Ross operation offers children requiring aortic valve replacement an excellent survival perspective, with an acceptable risk of autograft reoperation within the first 25 years. Contrary to the autograft annulus, dilation of the sinus and STJ size is of concern. Closer surveillance of autograft dimensions might be required in patients who underwent a Ross-Konno procedure or aortic arch reconstruction.

Ventricular interactions and electromechanical dyssynchrony after Ross and Ross-Konno operations

ObjectivesRoss and Ross-Konno operations are associated with the inherent risk of residual ventricular septal dysfunction and injury to the conduction system. However, comprehensive biventricular functional outcomes on magnetic resonance imaging after Ross and Ross-Konno procedures are unknown. The purpose of this study was to evaluate and compare the degree of electrical and mechanical dyssynchrony using cardiac magnetic resonance imaging in patients late after Ross and Ross-Konno operations. MethodsPatients following Ross operation (n = 16), Ross-Konno operation (n = 13), and healthy controls (n = 12) underwent cardiac magnetic resonance imaging strain deformation analysis to quantify left ventricular (LV) intraventricular dyssynchrony and right ventricular (RV)–LV interventricular dyssynchrony. Mechanical dyssynchrony indices were correlated with the degree of electrical dyssynchrony as assessed by QRS duration, as well as with magnetic resonance imaging-derived biventricular and autograft regurgitation parameters. ResultsPatients in the Ross and Ross-Konno groups had reduced LV global longitudinal strain when compared with controls (both P values < .01). Both Ross (P < .05) and Ross-Konno (P < .01) groups demonstrated increased RV–LV interventricular dyssynchrony compared with controls. Patients in the Ross-Konno group also had increased septal LV intraventricular dyssynchrony when compared with control and Ross groups (both P values < .01). The global LV intramechanical dyssynchrony strongly correlated with QRS duration (R = 0.716; P < .001). There was no association between mechanical dyssynchrony and aortic regurgitation fraction. ConclusionsLong-term follow-up of patients who underwent Ross and Ross-Konno operations demonstrates reduced LV global longitudinal strain suggestive of chronically increased afterload independent of autograft function. Additionally, Ross-Konno operation is associated with impaired LV intraventricular mechanics and electrical dyssynchrony.

Long-term outcomes of Ross and Ross-Konno operations in patients under 15years of age.

The Ross operation is an excellent surgical option for young children, regardless of aortic pathology type. However, failure rates are concerning during the second postoperative decade. We sought to determine the predictors of long-term outcomes after Ross and Ross-Konno operation performed in childhood. We performed 34 Ross and 9 Ross-Konno operations in pediatric patients (age < 15years) from 1996 to 2016, and retrospectively evaluated the long-term results. The postoperative follow-up period was 13.5 ± 3.9years. In the Ross group, there were one inpatient death and one death after discharge. There were no inpatient deaths in the Ross-Konno group. There were 6 reoperations in the Ross group and 1 in the Ross-Konno group for left ventricular outflow tract (LVOT). Cumulative survival rates were 96.8% and 100% in the Ross and Ross-Konno groups, respectively. The reoperation free rate for LVOT /RVOT (right ventricular outflow tract) were 98.6/85.5% and 91.9/63.4% in the 5th and 10th years of follow-up, respectively. Patients who underwent the operations at age > 8.6years had higher risks of reoperation for LVOT. Aortic annulus measurements > 24mm or aorta/pulmonary artery diameter (Ao/PA) ratios > 1.2 conferred higher risks of reoperation for LVOT. Long-term outcomes after Ross and Ross-Konno operations in children were satisfactory. However, new-onset aortic regurgitation was progressive and reoperation was needed in some children. Age, aortic annulus diameter, and Ao/PA ratio may be able to predict of long-term outcomes after Ross and Ross-Konno operations.

The Ross-Konno procedure in neonates and infants less than 3 months of age.

Patients with severe left ventricular outflow tract obstruction often suffer from impaired left ventricular function, endocardial fibroelastosis and borderline-sized structures. The early Ross-Konno operation can offer complete repair due to outflow tract stenosis and enable the functional recovery and growth of small structures. Between 2008 and March 2017, 44 early Ross-Konno procedures were performed at our centre. Thirty-five patients were neonates, and 9 were infants less than 3 months of age. A retrospective single-centre investigation was carried out analysing early and late deaths, postoperative complications and reoperations during the follow-up period. Potential prognostic influence factors as previous fetal intervention, associated lesions as presence of a VSD or hypoplastic aortic arch or severe endocardial fibroelastosis were examined. The in-hospital mortality rate was 7% (3 of 44), and the late mortality rate was 2%. There were no deaths in the group with ventricular septal defects (9 cases), and there was 1 death in the group with critical aortic stenosis without arch repair (1 of 24; 4%). The most deaths occurred in patients with critical aortic stenosis and aortic arch repair (3 of 11; 27%) (P = 0.012). Fifteen patients with foetal intervention had a mid-term survival rate of 87%. Reoperations were necessary in 19 of 40 surviving patients during a mean follow-up period of 5.9 years. The early Ross-Konno procedure can enable biventricular repair even in borderline left ventricles with good long-term outcome. Thus far, there were no reoperations at the level of the autograft or the left ventricular outflow tract in our cohort. The highest operative risk was observed in patients with critical aortic stenosis and aortic arch hypoplasia.

The Ross Procedure in Pediatric Patients: A 20-Year Experience of Ross Procedure in a Single Institution.

BackgroundThe Ross/Ross-Konno procedure is considered a good option for irreparable aortic valve disease in pediatric patients because of its hemodynamic performance and potential for growth of the pulmonary autograft. This study is a review of the long-term results of our 20-year experience with the Ross and Ross-Konno operations in a single institution.MethodsBetween June 1995 and January 2016, 16 consecutive patients (mean age, 6.0±5.9 years; range, 16 days to 17.4 years) underwent either a Ross operation (n=9) or a Ross-Konno operation (n=7). The study included 12 males and 4 females, with a median follow-up period of 47 months (range, 6 to 256 months).ResultsThere were no cases of in-hospital or late mortality. Six reoperations were performed in 5 patients. Four patients underwent right ventricular-pulmonary artery (RV-PA) conduit replacement. Two patients underwent concomitant replacement of the pulmonary autograft and RV-PA conduit 10 years and 8 years after the Ross operation, respectively. The rate of freedom from adverse outcomes of the pulmonary autograft was 88% and 70% at 5 and 10 years, respectively. The rate of freedom from valve-related reoperations was 79% and 63% at 5 and 10 years, respectively.ConclusionPulmonary autografts demonstrated good durability with low mortality. The Ross/Ross-Konno procedure is a good option that can be performed safely in pediatric patients with aortic valve disease, even in a small-volume center.

The Ross and Ross-Konno operations for congenital aortic valve diseases in infants and children

The Ross and Ross-Konno operations for congenital aortic valve diseases in infants and children

Left Ventricular Outflow Tract Obstruction after Ross-Konno Operation

INTRODUCTION: SAM (systolic anterior motion) is defined as the dynamic motion of the anterior mitral valve leaflet to the left ventricular outflow tract (LVOT) during systole. In this study, we present a case which we encountered with significant left ve...

PULMONARY AUTOGRAFT OPERATION FOR LEFT VENTRICLE OUTFLOW TRACT OBSTRUCTION

Pulmonary autograft operation, combined with anterior aortoventriculoplasty (Ross-Konno operation) is an effective procedure for relief of multilevel left ventricle outflow tract (LVOT) obstruction. We aim to analyzed first experience with Ross-Konno operation in Ukraine.

Konno Operation: The 2015 Kyoto Symposium Konno Memorial Lecture.

The Konno operation consists of a prosthetic aortic valve replacement by using an anterior enlargement of the small aortic annulus. The original procedure includes a longitudinal incision in the aortic septum placed near the midpoint between the two coronary ostia, a vertical incision in the outflow tract of the right ventricle to join the septal incision, prosthetic aortic valve replacement, and patch reconstruction of the outflow tracts of both ventricles by means of a fusiform Dacron patch. The concept of this operation has been applied in other complex operations, such as modified Konno operation, Ross-Konno operation, and aortic valve replacement after arterial switch operation.

Experience With Biventricular Intervention for Neonates With Mitral Valve Abnormalities in the Setting of Critical Left-Side Heart Obstruction

Mitral valve (MV) abnormalities may adversely impact outcomes after biventricular repair of critical left-side heart obstruction. Neonates with left-side heart obstruction and MV abnormality (anatomically abnormal or hypoplastic but otherwise normal [Z-score-1.5 to-4]) who underwent biventricular repair from December 2005 to December 2012 were included. Institutional selection criteria determined suitability for biventricular repair. Primary outcomes (death, univentricular conversion, pulmonary hypertension) and secondary outcomes (reinterventions) were compared between hypoplastic and anatomically abnormal MV groups. Freedom from reintervention was determined by Kaplan-Meier analysis. There were 55 patients with hypoplastic MV (n= 35) or anatomically abnormal MV (n= 20). Initial interventions included arch repair (n= 45, with ventricular septal defect closure in 7), balloon aortic valvuloplasty (n= 8), and Ross-Konno operation (n= 2). None had initial MV intervention. Follow-up was available on 46 patients (84%) at a median of 25 months. Primary outcome occurred in 6 patients (13%): 2 deaths, 1 univentricular conversion, and 3 pulmonary hypertension. Twenty-six patients (57%) underwent 36 follow-up interventions, often for arch obstruction (n= 26). All follow-up MV interventions (n= 4) occurred in patients with anatomically abnormal MV. Median reintervention-free time was 7.3 months. Freedom from reintervention at 6, 9, and 12 months was 65%, 38%, and 27%, respectively. Neither MV size nor anatomy was associated with primary outcome. Using our selection criteria, most neonates with left-side heart obstruction and MV abnormalities who underwent biventricular repair had reasonable survival with complex reintervention needs in early follow-up. The MV reintervention occurred in patients with abnormal anatomy, not hypoplasia.

The importance of hybrid stage I palliation for neonates with critical aortic stenosis and reduced left ventricular function.

The optimal management strategy for neonates with congenital aortic stenosis, two balanced ventricles, and duct-dependent systemic circulation (critical aortic stenosis) is still controversial. Thirteen patients with critical aortic stenosis underwent balloon aortic valvotomy (BAV) between 1996 and 2013, at the median age of 1 day old (range 0-28). Since 2010, bilateral pulmonary artery banding with ductal stenting following BAV was conducted for patients with reduced left ventricular (LV) function as a hybrid stage I palliation for the bridge to decision for further treatment. A follow-up was completed on all patients and the median follow-up period was 3.3 years (max 16.0). The overall survival rate at 15 years was 67.1 %. Six of the seven patients with maintained LV function could go on to the definitive Ross or Konno-aortic valve replacement at the median duration of 311 days after initial BAV, without any mortality. Three of four patients with reduced LV function died before 2010 with conventional treatment. With use of a hybrid stage I palliation, one of two patients ultimately underwent Fontan completion at 38 months of age and the other successfully underwent the definitive Ross-Konno operation at 9 months of age after recovery of the LV function. Although a statistically significant improvement has not been observed yet, the application of hybrid stage I palliation following BAV would be a favorable alternative for patients with reduced LV function to avoid a high-risk neonatal Ross or Norwood-type operation, and also to determine further treatment carefully.

The Ross, Konno, and Ross-Konno operations for congenital left ventricular outflow tract abnormalities.

Operations for left ventricular outflow tract abnormalities are centred on hemodynamic conditions that relate to subvalvar stenosis, valvar stenosis/regurgitation, aortic annular hypoplasia, and supravalvar aortic stenosis. Operative interventions over the years have evolved because the intervening outcomes proved to be unsatisfactory. The resection for subvalvar aortic stenosis has progressed from a fibrous "membrane" resection to a more extensive fibromuscular resection. Operative solutions for valvar aortic stenosis and regurgitation have resulted in operative interventions that depend on simple commissurotomy, leaflet extensions, prosthetic mechanical valve replacement, biologic valve replacement, including the pulmonary autograft, and operations to treat aortic annular stenosis. Although there are enthusiastic proponents for all of these strategies, the fact remains that none have proven to be curative; patients can expect to undergo further procedures during their lifetimes. The short- and mid-term solutions to these left ventricular outflow tract abnormalities have improved based on operations that have been attended by increasing operative complexity. The purpose of this review is to chronicle the operative steps of the Ross operation, the Konno-Rastan operation, the modified Konno operation, the Ross-Konno operation, and the modified Ross-Konno operation.