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: 1.3 years; range: 0.5-13.3 years) were evaluated by echocardiography at defined timepoints: pre-RK, post-RK, 1-year post-RK, and 2 years 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.

Surgical Relief of Left Ventricular Outflow Tract Obstruction in Adults With Congenital Aortic Stenosis and Associated Aortic Annulus Hypoplasia and/or Subaortic Obstruction.

BACKGROUND: In children and adolescents, a Ross/Konno operation is commonly done to both enlarge the aortic root and provide a competent aortic valve with relief of left ventricular outflow tract obstruction (LVOTO). Optimum management is not so straightforward in adults. METHODS: Between 1995 and 2014, 16 patients of mean age 39.4 years (18-57 years) with hypoplastic aortic annulus (AA) measuring 20mm and less, and mean aortic valve/LVOT gradient of 61mmHg (30-70mmHg) presented for surgery. RESULTS: Eight patients with mean LVOT/AA diameter 19.6mm (18-20mm) underwent an "inclusion-cylinder" type Ross procedure (RP). Eight patients with more severe LVOT/AA obstruction, with mean diameter of 17.4mm (16-19mm) underwent mechanical aortic valve replacement (AVR) with standard Konno-type aortoventriculoplasty. There was zero early and late mortality; with mean follow-up of 11.6 years (3-21 years) in the Ross group and 6 years (2-10 years) in the Konno-AVR group. One patient in the Konno-AVR group had reoperation after 2 years for RVOT obstruction. The postoperative echocardiograms of these patients at last follow-up show residual mean gradient across LVOT/AA of 4.4mmHg (2-6mmHg) after RP, and 11.9mmHg (8-17mmHg) after Konno-AVR. CONCLUSIONS: In adults, the "inclusion-cylinder" Ross-procedure is a good alternative for mild to moderate aortic root hypoplasia. However, for cases with severe LVOT obstruction, a Ross-Konno is not possible with the same method of autologous support used in a non-Konno RP, and this could be expected to have an impact on late durability and the need for further intervention, in a group that has already undergone multiple procedures in childhood. Both methods of RP and Konno-AVR lead to excellent early and late results.

The Ross-Konno Procedure With or Without Mitral Valve Surgery: A Systematic Review With Individual Data Pooling.

Background: The Ross-Konno procedure is a technically demanding surgical option to treat multilevel left ventricular outflow tract obstruction. Methods: A systematic review with pooled analyses was conducted according to PRISMA criteria on studies published between January 2000 and May 2022 that assessed outcomes following the Ross-Konno intervention in children. Individual patient data were extracted from published Kaplan-Meier curves using digitalization software. Overall survival and freedom from reintervention were assessed by time-to-event approaches. Determinants of one-year survival were investigated by meta-regression analyses. Results: Ten studies with a total population of 274 patients were included. The overall pooled early (≤30 days) survival rate was 86.9% (95% CI [87.6%-78.4%]). Five-year survival rates in patients without and with (N = 50 [18.2%] of 274 total patients) concomitant mitral valve surgery were 82.5% (95% CI [87.6%-77.4%]) versus 56.1% (95% CI [74.1%-38.1%]), hazard ratio 2.67, 95% CI (1.44-4.93), P < .0001. Five- and ten-year freedom from pulmonary autograft reoperation rates were 93.5% and 90.9%, respectively. Five- and ten-year freedom from right ventricular outflow tract reoperation rates were 74.3% and 57.3%, respectively. By meta-regression analysis, resection of endocardial fibroelastosis (N = 32 [11.7%] of 274 total patients) was associated with superior one-year survival (P = .027). Conclusion: The Ross-Konno procedure is associated with substantial early mortality and gradual attrition thereafter. Mortality is higher in patients with concomitant mitral valve surgery. Resection of endocardial fibroelastosis is associated with superior survival. Right ventricular outflow tract reinterventions are common.

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.

Huge, invasive, and destructive Abiotrophia defectiva endocarditis of the aortic valve and the aortic wall: a case report of an emergency but successful Ross-Konno operation in a child.

Background: Abiotrophia defectiva forms Gram-positive cocci, is part of normal oropharyngeal and gastrointestinal flora, and is rarely involved in endocarditis in children population. Its special nutritional requirements and subacute clinical course may delay diagnosis and proper treatment, leading to life-threatening consequences. Case summary: We report a rare case of huge and destructive A. defectiva infective endocarditis (IE) of the aortic valve and the aortic wall in a 3-year-old child, in follow-up after surgical valvuloplasty for congenital aortic stenosis. The child presented at our department with signs of left side hemiplegia. Transthoracic echocardiography showed severe aortic regurgitation due to large vegetation extending to the aortic wall up to the aortic arch. Blood cultures resulted positive for A. defectiva. He was initially treated conservatively with antibiotic therapy. Ten days after admission, because of clinical deterioration, he required intubation and an emergency Ross-Konno operation. Despite the critical conditions and highly risky surgery, the child recovered well and was discharged home 5 weeks after the operation. Discussion: Abiotrophia defectiva IE is rare in children. Since 1995, only 16 cases of A. defectiva IE have been reported in children, including our case. This pathogen has a higher rate of complications when affecting children rather than adult population. Our case demonstrates that conservative strategy with antibiotics is rarely resolutive in the case of IE caused by A. defectiva. Whenever one or more indications for surgery are present, surgical intervention should always be taken into consideration, even if clinical conditions are prohibitive and surgery is at very high risk.

Aortic Atresia or Complex Left Outflow Tract Obstruction in the Presence of a Ventricular Septal Defect.

Severe left outflow tract obstruction (LVOTO) is not always associated with hypoplastic left heart syndrome (HLHS). Aortic valvar atresia or complex LVOTO in the presence of a large ventricular septal defect (VSD) are a rare group of lesions that offer the possibility of biventricular repair. The Yasui procedure is the commonest surgical approach which can be performed as a primary neonatal correction or as a staged procedure with a Norwood followed by a subsequent Rastelli. This article reviews the surgical outcomes and decision-making process. Both strategies are reasonable with the trend toward staged procedure in the setting of the additional interrupted arch, with neonatal survival of > 90% in the modern era and excellent long-term survival. Re-intervention is mostly related to conduit revision and the need for re-operation for LVOTO is rare. Deciding between conventional repair and the Yasui in cases of LVOTO/VSD can be difficult and there are no uniform accepted criteria. In a typical neonate, an aortic annulus < 4.5 mm is generally the limit of acceptability for a conventional repair. In selected cases of LVOTO/VSD, an alternative to the Yasui is the Ross-Konno. Retrospective comparisons between the 2 techniques are difficult due to differing patient characteristics (especially associated with mitral valve disease) but the neonatal Ross has been associated with higher early mortality.

The Ross-Konno procedure for congenital aortic stenosis.

Left ventricular outflow tract (LVOT) obstruction can result from supravalvular, valvular and/or subvalvular etiologies. Congenital aortic valvular stenosis is frequently associated with aortic annular hypoplasia. Aortoventriculoplasty with pulmonary autograft, "The Ross-Konno" operation, provides more or less a radical solution to multilevel LVOT obstruction by enlarging the aortic annulus and the subvalvular area, thus relieving both valvular and subvalvular obstructions. In addition to this, the procedure carries the major advantage of having a competent autograft in the LVOT. An autograft that has the potential for growth and provides excellent quality of life without the need for anticoagulation. The procedure is most commonly performed as a complete root implantation, harvesting the coronary arteries as buttons, and harvesting the autograft with a muscle skirt to allow a single unit reconstruction of the LVOT. The procedure has been modified over time to minimize the risk of conduction tissue injury and the development of complete heart block by modifying the interventricular septal incision. The Ross-Konno procedure has changed the approach to patients with complex multilevel LVOT obstruction. However, while it can be performed early in life, one should be aware that this procedure is technically demanding and can be associated with a higher surgical risk. Nevertheless, it is considered "the ultimate solution" for those with complex multilevel LVOT obstruction.

Isolated Sub-Pulmonic Valve Endocarditis in a Patient With a History of Konno Procedure and Mechanical Aortic Valve.

Pulmonic and sub-pulmonic valve endocarditis are rarely encountered in clinical practice. We present the first case of isolated sub-pulmonic endocarditis. A 30-year-old man with a history of mechanical aortic valve presented to the emergency department with multiple complaints including nausea, vomiting, body aches, and fevers. The patient underwent surgical resection for sub-aortic stenosis followed by a modified Konno procedure later in life. A modified basal short-axis view on the trans-thoracic echocardiogram revealed a sub-pulmonic mobile structure highly suggestive of infective endocarditis. Blood cultures grew methicillin-sensitive Staphylococcus aureus within 24 hours. Higher oxygen demand prompted chest imaging, chest CT showed the development of bilateral airspace consolidation, suggestive of pneumonia. After treatment with extended intravenous antibiotics, follow-up echocardiogram four months later showed no identifiable sub-pulmonic vegetation. This case describes a situation where clinicians may suspect infective endocarditis in a typical location such as a mechanical aortic valve. However, in patients who develop pneumonia, infective endocarditis of the right heart should be suspected. The pulmonic valve and sub-pulmonic ridge are often difficult to image given their anatomical location, a modified basal short-axis view on trans-thoracic echocardiogram can better image these structures.

Long-term outcomes after the paediatric Ross and Ross-Konno procedures.

OBJECTIVES: The Ross procedure is an attractive option for the management of aortic valve disease in paediatric patients. We reviewed our experience with the paediatric Ross procedure to determine survival and freedom from reoperation in the third decade after surgery. METHODS: We reviewed the data of 124 paediatric patients [71% male, median age at time of surgery 11.1 years (interquartile range 6-14.8 years); 63.7% bicuspid aortic valve], who underwent the Ross procedure at 2 tertiary centres from April 1991 to April 2020. The Ross-Konno procedures were performed on 14 (11.3%) patients. Deaths were cross-checked with the national health insurance database, and survival status was available for 96.8% of the patients. The median follow-up time was 12.1 years (interquartile range 3-18 years). RESULTS: There were 3 early and 6 late deaths. All early deaths occurred in patients aged <1 year at the time of surgery. The 25-year survival was 90.3%. Actuarial freedom from reoperation (linearized rates in parentheses) was as follows: Autograft reoperation was 90.8% (0.48%/patient-year) and right ventricular outflow tract (RVOT) reoperation was 67% (2.07%/patient year) at 25 years. The univariable Cox-proportional hazard analysis revealed younger age at time of surgery (P < 0.001), smaller implanted valve size (P < 0.001) and the use of a xenograft rather than a homograft (P < 0.001) as predictors of RVOT reoperation. At multivariable Cox-proportional hazard analysis, only age was an independent risk factor for RVOT reoperation (P = 0.041). CONCLUSIONS: The Ross and the Ross-Konno procedures are associated with good outcomes in paediatric patients. Reoperation of the RVOT is frequent and associated with younger age.

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

OBJECTIVES: 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. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.

Subcoronary Ross/Ross-Konno operation in children and young adults: initial single-centre experience.

OBJECTIVES: We sought to evaluate the outcome after modified subcoronary Ross/Ross-Konno operation in children and young adults. METHODS: Between January 2013 and January 2019, a total of 50 patients with median age of 6.3 years (range 0.02-36.5 years, 58% males), including 10 infants (20%), received modified subcoronary Ross/Ross-Konno operation at our institution. Survival, morbidity, reinterventions, aortic valve function and aortic root dimensions were analysed. RESULTS: At a median follow-up of 31.2 months (range 14.4-51 months), there were 1 early death and 1 late death, both in the infant group. The overall survival at 5 years after the operation was 95%. Two patients needed aortic valve replacement, 11 and 15 months after their Ross operation. At 5 years, freedoms from reoperation on the autograft and on the right ventricle to pulmonary artery conduit were 94% and 97%, respectively. Freedom from aortic valve regurgitation greater than mild was 97% at 5 years. Median dimensions of the aortic root at all levels remained in normal range at last visit. Forty-four patients (95%) were in New York Heart Association class I with normal left ventricular function. CONCLUSIONS: The initial experience with the subcoronary Ross/Ross-Konno operation in children and young adults showed excellent outcome. The mortality and morbidity among infants remain significant. The described technique is reproducible and might be advantageous in situations when prosthetic supporting techniques interfere with somatic growth.

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

OBJECTIVES: Ross 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. METHODS: Patients 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. RESULTS: Patients 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. CONCLUSIONS: Long-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 15 years of age.

OBJECTIVE: 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. METHODS: We performed 34 Ross and 9 Ross-Konno operations in pediatric patients (age < 15 years) from 1996 to 2016, and retrospectively evaluated the long-term results. RESULTS: The postoperative follow-up period was 13.5 ± 3.9 years. 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 > 8.6 years had higher risks of reoperation for LVOT. Aortic annulus measurements > 24 mm or aorta/pulmonary artery diameter (Ao/PA) ratios > 1.2 conferred higher risks of reoperation for LVOT. CONCLUSIONS: 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.

Ross-Konno Procedure With Cylinder Mitral Valve Replacement in 49 Days Old Infant.

A neonate with antenatally diagnosed severe aortic valve stenosis, left ventricular outflow tract obstruction, left ventricular endocardial fibroelastosis, and severe mixed mitral valve disease was admitted to our center for balloon aortic valvuloplasty. On day 49, we performed a Ross-Konno procedure and mitral valve replacement on the baby, whose weight was 3.4 kg. Right ventricle-to-pulmonary artery continuity was restored with a handmade trileaflet Gore-Tex conduit. We used a handmade cylindrical prosthesis made from decellularized equine pericardium in the mitral position. We observed improvement in left ventricular function and good performance of aortic, pulmonary, and mitral prostheses at four months of follow-up.

Twenty-year experience with the Ross-Konno procedure.

OBJECTIVES: The Ross-Konno procedure is a last resort for patients with complex multilevel left ventricular outflow tract obstruction (LVOTO) often having other cardiovascular anomalies. It is typically preceded by multiple surgeries. Literature is scarce on long-term follow-up series. Therefore, we have reviewed our 20-year experience with this procedure in order to provide insights in patients' outcomes and to optimize patient selection. METHODS: Between January 1995 and December 2014, 48 patients underwent the Ross-Konno procedure. The median age at operation was 12.8 months (range, 11 days to 31 years). Twenty-two (46%) patients were under 1 year of age. Forty-four (92%) patients had undergone a total of 82 previous procedures. Eleven (23%) patients had concomitant surgery, predominantly mitral valve (n = 5) and aortic arch surgery (n = 5). RESULTS: The median follow-up time was 4.3 years (range, 0-20 years). There were 6 (12.5%) early deaths and 4 (8.3%) late deaths. Estimated overall survival at 5, 10 and 15 years was 83, 79 and 70%, respectively. Poor LV function was a risk factor for early mortality (odds ratio = 9.5; 95% confidence interval = 1.4-63.7; P = 0.020). Twelve patients required a total of 29 procedures in 17 reoperations. Five patients required reoperation for autograft failure at a median of 14 years (range, 5-15 years) postoperatively. Estimated freedom from all causes of reoperation at 5, 10 and 15 years was 82, 55 and 30%, respectively. All patients had complete and durable relief of LVOTO. At latest follow-up, 5 patients had a sinus of Valsalva Z-score of 5 or greater. One patient had Grade II autograft insufficiency. CONCLUSIONS: The Ross-Konno procedure is a durable solution for multilevel LVOTO in a highly complex patient population with high incidence of previous procedures. High early mortality rates in patients with impaired left ventricular function emphasize the importance of patient selection. Freedom from reoperation shows a continuous attrition rate. Reoperation for autograft failure may occur late after the Ross-Konno procedure.

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.

Neonatal Ross-Konno operation and endocardial fibroelastosis resection after foetal percutaneous aortic valve balloon valvuloplasty: a complex approach to rescue the left heart.

We report a case of a patient who presented with aortic stenosis and a borderline left ventricle during foetal life. A balloon aortic valve valvuloplasty was performed in uterus, and in the postnatal period for relief of the left ventricular outflow tract obstruction followed by a Ross-Konno procedure with fibroelastosis resection. These successful interventions allowed left ventricular growth and the conversion to a biventricular circulation after a single-stage surgery.

Comparison of the Ross/Ross-Konno aortic root in children before and after the age of 18 months.

OBJECTIVES: Evaluation of long-term results after the Ross/Ross-Konno operation in patients <18 months of age, focusing on pulmonary autograft performance. METHODS: Retrospective analysis of patients who underwent the Ross/Ross-Konno operation (1991-2011). Data were obtained from hospital records and follow-up was 100% complete. RESULTS: Between January 1991 and December 2011, 140 patients underwent the Ross/Ross-Konno operation and 22 patients were <18 months of age (male/female: 15/7). The median age was 166 days. 14 patients had a Ross operation and 8 patients a Ross-Konno operation. Presentation at surgery was aortic valve stenosis in 13, regurgitation in 7 and mixed disease in 2. Only 4 patients (18%) had no surgery prior to Ross/Ross-Konno, and among the others the previous most frequent operation was aortic valvotomy (55%). There were 3 early deaths, all in high-risk cases with poor preoperative left ventricular function. At discharge there was no neoaortic regurgitation in 10, trivial in 4 and mild in 5. The median follow-up is 10.8 years (range 0.96-21). There was 1 late death due to progressive ventricular dysfunction 2.4 years after Ross-Konno and mitral valve replacement. Survival for patients <18 months was 81% at 5-10 years (18 patients) and for older patients was 98.2% at 5-10 years. There was no neoaortic regurgitation in 8 patients, mild in another 8 and moderate in 2 with a freedom from moderate regurgitation significantly lower in comparison with older patients (100-80% vs 83.5-73.4% at 5-10 years). Freedom from aortic reoperation for patients <18 months was significantly higher compared with older (100 vs 95.4-84.4% at 5-10 years, P < 0.04). Reoperation in the RVOT was higher in younger patients compared with the other (85-64.6% vs 97.2 and 84.7% at 5-10 years P = 0.02). Z-score aortic root diameter remained constant in the <18-months group compared with significant dilatation in the >18-months patients (P < 0.01). CONCLUSIONS: Ross/Ross-Konno can be invaluable in the younger age group but not without risk in the setting of ventricular dysfunction. Long-term performance of the neoaortic valve is significantly better than in older children, which is related to maintenance of normal root dimensions compared with progressive dilatation in patients undergoing Ross/Ross-Konno at older ages.

Use of ß-blockade and levosimendan for separation from extracorporeal life support in an infant with postoperative diastolic dysfunction.

Diastolic dysfunction is common in infants and neonates with left ventricular (LV) outflow tract obstruction and may lead to low-cardiac output in the postoperative period. We present a management strategy for severe postrepair diastolic dysfunction in an infant with critical congenital aortic stenosis and LV hypertrophy, employing ß-blockade and levosimendan.