Aortic valve repair in a low-birth-weight neonate with poor ventricular function: staged tricuspidization of the unicuspid valve.

Congenital severe isolated aortic stenosis in neonates requires prompt intervention because left ventricular function may deteriorate rapidly. Surgical repair of a stenotic aortic valve in neonates allows debridement of fibrous material and precision commissurotomy. As such, the child would end up with a much better-quality aortic valve compared to the uncontrolled tear that results following balloon dilatation. The meticulous debridement and precise commissurotomy are important in achieving long-term durability of the aortic valve repair, with the expectation that the patient may keep the native aortic valve at least into adolescence or adulthood. If feasible, it is important to avoid placement of patches while repairing the valve, so that the growth potential of the cusps is preserved. The valve could be revisited and repaired further, so that, ideally, the native valve could be retained for life, thus avoiding aortic valve replacement entirely. We present a low-birth-weight neonate with a dysplastic unicuspid aortic valve, severe aortic valve stenosis and decreased left ventricular function who underwent repair of the aortic valve.

Long-Term Results of Congenital Aortic Stenosis Treatment in the Era of Percutaneous Balloon Valvuloplasty: Up to 33 Years Follow-Up.

Background The goal of this study was to evaluate long-term results of percutaneous balloon valvuloplasty (BVPL) used exclusively for initial management of congenital aortic stenosis in children. Methods and Results A total of 409 consecutive pediatric patients (134 newborns, 275 older patients) who underwent BVPL as initial treatment of aortic stenosis in a single nationwide pediatric center were subjected to a retrospective follow-up study. The resulting follow-up time reached a median of 18.5 (interquartile range, 12.2-25.1) years. Successful BVPL was defined by residual Doppler gradient <70/40 (systolic/mean) mm Hg. The primary end point was death; secondary end points included any valve reintervention, balloon revalvuloplasty, any aortic valve surgery, and aortic valve replacement, respectively. BVPL effectively reduced the peak and mean gradient both immediately and at the latest follow-up (P<0.001). There was significant procedure-related progression of aortic insufficiency (P<0.001). Higher aortic annulus z score was predictive for severe aortic regurgitation (P<0.05) and lower z score for insufficient gradient reduction (P<0.05). The actuarial probability of survival/survival free from any valve reintervention was 89.9%/59.9%, 85.9%/35.2%, and 82.0%/26.7% at 10, 20, and 30 years after first BVPL, respectively. Left ventricular dysfunction or arterial duct dependency as the indication for BVPL was predictive of both worse survival and survival free from any reintervention (P<0.001). Lower aortic annulus z score and lower balloon-to-annulus ratio were predictive of a need for revalvuloplasty (P<0.001). Conclusions Percutaneous BVPL provides good initial palliation. In patients with hypoplastic annuli and left ventricular or mitral valve comorbidity, the results are less favorable.

Case report: Aortic valve endocarditis and recurrent pulmonary valve stenosis.

BACKGROUND: We discuss a rare case of an adult patient with different pathologies involving the aortic and pulmonary valves in need of surgery. CASE PRESENTATION: The patient had a history of congenital PV stenosis and surgical valvuloplasty. Almost 50 years later the patient underwent a complex second heart surgery due to infective endocarditis of the aortic valve and high-grade restenosis of the pulmonary valve. Replacement of the aortic and pulmonary valve, as well as reconstruction of the RVOT and closure of a persistent foramen ovale, followed. Postoperative course was uneventful and the patient was discharged home a week after surgery. CONCLUSION: Simultaneous surgery of pulmonary and aortic valves due to different pathologies is rare but can be performed successfully even in advanced age and can improve quality of life.

Long-term outcomes of primary aortic valve repair for isolated congenital aortic stenosis in children.

OBJECTIVE: We aimed to assess the long-term outcomes of children with isolated congenital aortic stenosis who underwent primary aortic valve repair. METHODS: Records of all children (n = 111) with isolated congenital aortic stenosis who underwent primary aortic valve repair between 1980 and 2016 were reviewed. An optimal operative outcome consisted of a residual left ventricular outflow tract peak systolic gradient <35 mm Hg and trivial or less aortic insufficiency. RESULTS: Median age at surgery was 0.4 years (interquartile range, 1 month-7.9 years) and median weight at surgery was 7.0 kg (interquartile range, 3.7-25.0 kg). Fifty-two patients (46.8%; 52/111) underwent aortic valve repair with the use of patch material. Early mortality was 0.9% (1/111). Late mortality was 0.9% (1/110). Freedom from aortic valve reoperation was 52.1% (95% CI, 38.7-63.8) at 10 years. Freedom from aortic valve replacement was 67.9% (95% CI, 55.4-77.5) at 10 years. An optimal outcome was achieved in 48 patients (43.2%; 48/111). At 10 years, freedom from aortic valve reoperation was 78.2% (95% CI, 63.1-87.8) in patients with an optimal outcome, compared with 39.4% (95% CI, 22.8-55.6) in those with a suboptimal outcome (P = .03). Tricuspid aortic valve was associated with a suboptimal outcome (P = .01). CONCLUSIONS: Aortic valve repair achieves relief of congenital aortic stenosis with very low early mortality and excellent long-term survival, even in neonates. Although nearly half of the patients required aortic valve reoperation by 10 years, two-thirds of the patients remain free from aortic valve replacement. An optimal outcome was more commonly achieved with bicuspid aortic valves compared with tricuspid aortic valves.

Surgical Valvuloplasty Versus Balloon Dilation for Congenital Aortic Stenosis in Pediatric Patients.

BACKGROUND: For children with congenital aortic stenosis (AS) who are candidates for biventricular repair, valvuloplasty can be achieved by surgical aortic valvuloplasty (SAV) or by transcatheter balloon aortic dilation (BAD). We aimed to evaluate the longer term outcomes of SAV versus BAD at our institution. METHODS: We retrospectively reviewed the outcomes of 2 months to 18 years old patients who underwent SAV or BAD at our institution between January 1990 and July 2018. Baseline and follow-up characteristics were assessed by echocardiography. Long-term survival, freedom from reintervention, freedom from aortic valve replacement (AVR), and aortic regurgitation were evaluated. RESULTS: A total of 212 patients met inclusion criteria (SAV = 123; BAD = 89). Age, sex, aortic insufficiency (AI), and aortic valve gradient were similar between the groups. At 10 years, 27.9% (19/68) of SAV patients and 58.3% (28/48) of BAD patients had moderate or worse AI (P = .001), and reintervention occurred in 39.2% (29/74) of SAV patients and 78.6% (44/56) of BAD patients (P < .001). Kaplan-Meier analysis revealed overall survival was 96.8% (119/123) for SAV and 95.5% (85/89) for SAV (P = .87). At 10 years, 35% (23/66) of SAV patients and 54% (23/43) of BAD patients underwent AVR (P = .213). CONCLUSIONS: Surgical aortic valvuloplasty demonstrated greater gradient reduction, less postoperative and long-term AI, and a lower reintervention rate at 10 years than BAD. There was no difference in survival or AVR reintervention rate. Surgical aortic valvuloplasty is a durable and efficacious intervention and should continue to be considered a favorable choice for palliation of valvular AS.

Implantation of a decellularized aortic homograft in a child.

The implantation of a decellularized aortic homograft in children and young adults has been shown to be a good alternative to existing surgical approaches. Lower risk of calcification and the potential of growth render a homograft a promising valve substitute. The child presented in this video tutorial is a 10-year-old boy diagnosed with congenital aortic stenosis which was treated by balloon valvuloplasty early in life. Current echocardiographic findings show severe aortic regurgitation and stenosis. The tutorial provides detailed insight into how to implant a decellularized aortic homograft as a total root replacement.

Strategies to Minimise Need for Prosthetic Aortic Valve Replacement in Congenital Aortic Stenosis-Value of the Ross Procedure.

To examine the role and outcomes of all interventions for aortic stenosis in children, with focus on freedom from reintervention and the aim to minimise prosthetic aortic valve replacement (pAVR) during childhood. Retrospective analysis of 194 consecutive children who underwent any aortic valve intervention for a biventricular repair strategy at a single institution between 1995 and 2017. Data were obtained from hospital records and follow-up was 100% complete. Over a 22-year period, 194 children underwent total 313 aortic valve procedures: Primary interventions were surgical valvotomy (SV)/surgical repair (SR) in 94 (48.5%), balloon valvuloplasty (BV) in 60 (30.9%), pAVR in 8 (4.1%) and Ross/Ross-Konno procedure in 32 (16.5%). Median age at first intervention was 1.1 years (interquartile range [IQR] 0.1-9.4) and varied with type of intervention: SV/SR were most common in neonates (33, 75%) and infants (35, 68%), whilst BV was most frequent in older children (42, 42%). Operative survival was 99% (2 early deaths, both in neonates with critical aortic stenosis and poor left ventricular function) and 15-year survival was 95%. A Ross procedure was performed in 79 (40.7%) patients over the 15-year study period, 1 of whom required late pAVR for autograft failure. Freedom from any reintervention after SV/SR and BV was 41% and 40% at 10 years, compared to 90% at 10 years with the Ross procedure (P < 0.001). Among neonatal SV/SR and BV, 98% required reintervention during childhood with no difference between groups. Valve morphology did not influence freedom from ultimate valve replacement. In patients who went on to have a Ross procedure, median time from initial intervention to Ross was 2.8 years (IQR 0.1-11.9) in neonates and 6.0 years (IQR 3.1-7.5) in all other age groups. Overall freedom from pAVR was 97% at 10 years and was similar in the SV/SR and BV groups. A strategy of simple valve repair and primary Ross procedure provides excellent survival and good freedom from pAVR. However, reintervention rates after simple interventions for congenital AS are high, especially in younger age groups. The Ross procedure offers the best freedom from reintervention of any technique and wider use of primary Ross in younger age groups should be considered.

Valvular aortic stenosis in three cats.

Aortic stenosis affects 0.028% of cats in a shelter population, with valvular aortic stenosis compromising almost half of these cases. Of congenital heart diseases reported in cats, aortic stenosis is the second most common one, affecting 17% of these cases. Existing literature on valvular aortic stenosis is scant, and thus, presentation and prognosis of affected animals is poorly understood. In this case series, we describe three cats with confirmed valvular aortic stenosis. All cases were diagnosed echocardiographically, and all three had visible aortic valve leaflet fusion and a poststenotic dilation of the ascending aorta. Congestive heart failure developed in all three cases, and prognosis was poor. This case report highlights the existence of aortic valve dysplasia in cats and may allow clinicians a better understanding of the clinical presentation of this congenital abnormality.

Outcomes Following Balloon Aortic Valvuloplasty Versus Surgical Valvotomy in Congenital Aortic Valve Stenosis: A Meta-Analysis.

BACKGROUND: The optimal treatment for congenital aortic stenosis (AS) has been debated over the past three decades of experience with both balloon aortic valvuloplasty (BAV) and surgical aortic valvotomy (SAV). While BAV has been the mainstay of therapy for children with AS in most centers, recent single-center reports suggest superior results following SAV. METHODS: We queried Medline, EMBASE and Web of Science for eligible studies. RESULTS: A total of 18 studies were included in our meta-analysis: SAV alone (n = 3), BAV alone (n = 10), and both (n = 5). The mean follow-up duration of BAV patients was 6.5 years, while the mean follow-up duration for SAV patients was 7.2 years. Mortality rates following BAV and SAV were 11% (95% CI, 8-14) and 10% (95% CI, 7-15), respectively. Reintervention following initial procedure for treatment of AS was higher following BAV (37% [95% CI, 30-44]) compared with SAV (25% [95% CI, 20-31]). The predominant reintervention for both the BAV and SAV groups was surgery (SAV or aortic valve replacement [AVR]); the surgical reintervention rate was 59% for BAV (95% CI, 51-66) and 75% for SAV (95% CI, 48-91). Mean time to reintervention was shorter for BAV (2.7 years [95% CI, 1.4-4.1]) compared with SAV (6.9 years [95% CI, 4.4-9.4]). AVR following BAV was 20% (95% CI, 17-23) and following SAV was 17% (95% CI, 12-25). Long-term and mid-term follow-up in these studies showed moderate to severe aortic insufficiency (AI) was present in 28% (95% CI, 20-37) and 19% (95% CI, 12-27) in BAV and SAV patients, respectively. CONCLUSIONS: The rate of reintervention following BAV is higher than following SAV. However, survival rates, AVR, and development of late AI following BAV and SAV are equivalent. The costs associated with the two therapies in terms of hospital days and other morbidities should be considered in future comparative studies.

Long-Term Fate of the Neoaortic Root After Neonatal Ross Operation: A Case Series.

The aim of this study is to analyze the adaptation properties of the pulmonary autograft in four infants who underwent the Ross operation before one year of life. The patients underwent serial echocardiographic assessments of the autograft diameters at short- and long-term follow-up and values were reported as the Z scores for normal aortic and pulmonary diameters. At a median follow-up time of 18.5 years (range: 18.2-19.4 years), all the patients are alive, none requiring autograft reinterventions. This series shows excellent adaptation potential of the "infant pulmonary autograph" in the long-term, during somatic growth of the patient.

[Infective Endocarditis in Right Ventricle( RV)-Pulmonary Artery( PA) Conduit Late after the Ross Procedure;Report of a Case].

Ross procedure has been found to have a lower incidence of infective endocarditis compared to other aortic replacement procedure using prosthetic valves. We report a case of 25-year-old man who underwent Ross procedure for congenital aortic stenosis and regurgitation when he was 7 years old. He presented with fever and was highly suspected of infective endocarditis. All sets of blood cultures were positive for Heamophilus parainfluenzae. Autologous pericardial pulmonary valve was severely stenotic and computed tomography (CT) scan and radio isotope (RI) scan revealed infection at the stenotic valve. We performed right ventricle (RV)-pulmonary artery (PA) conduit replacement and he was discharged after completion of intravenous antibiotic treatment. We experienced a rare case of infective endocarditis in a patient late after Ross procedure. Prophylaxis against infective endocarditis is mandatory even in patients with infection resistant Ross procedure.

Aortic Valve Interventions in Pediatric Patients.

Aortic valve (AV) disease in pediatric patients requires a complex decision process that has an impact on decades of life. The aim of this review is to summarize the current evidence surrounding AV interventions in this patient population. In neonates with critical aortic stenosis, the relative merit of surgical vs balloon valvuloplasty is debated and practices vary depending on centers' experience with little comparative literature. In children and adolescents, AV repair has regained interest in the last decades with encouraging early and mid-term results. The Ross procedure represents the best AV replacement option as it offers growth potential, excellent hemodynamics, low rates of endocarditis, and thromboembolism without the risks of anticoagulation. Based on contemporary literature, we propose a management algorithm for children AV disease.

Pre-intervention morphologic and functional echocardiographic characteristics of neonates with critical left heart obstruction: a Congenital Heart Surgeons Society (CHSS) inception cohort study.

AIMS: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions. METHODS AND RESULTS: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%). Only 17% of patients with AS/MS had an apex-forming left ventricle, compared with 0% of those with AA/MA and AA/MS (P < 0.0001). Aortic arch hypoplasia and coarctation were common across all four groups, while those with AA/MA and AA/MS had the smallest ascending aorta diameters. Flow in the ascending aorta was retrograde in 43% and 10% of the patients with AS/MS and iAS, respectively. The right ventricle was apex forming in 100% of patients with AA/MA and AA/MS, 96% with AS/MS and 70% with iAS (P < 0.0001). Moderate to severe tricuspid regurgitation was present in 13% of all patients. CONCLUSION: This large multi-institutional study generates insight into the distribution of the functional and morphologic spectrum in patients with critical left-sided heart disease and identifies differences in these functional and morphologic characteristics between the main anatomic subtypes of critical left heart obstruction.

Aortic stenosis of the neonate: A single-center experience.

OBJECTIVES: Because data for neonates are limited, optimal management of critical aortic stenosis remains controversial (balloon valvotomy [BV] or open valvoplasty [OV]). In a center with balanced experience in both methods, we hypothesized that OV can provide a better individualized approach than blunt BV and better serve long-term outcomes. METHODS: A retrospective review of data and follow-up (survival, freedom from operation/replacement) of all neonates, suitable for biventricular repair, undergoing aortic valve procedure (1989-2015), was performed. RESULTS: One hundred three patients were concomitantly treated (BV [n = 51], OV [n = 52). Median age was 8 days, median aortic annulus Z-score was -1.3 for BV (range, -3.9 to 2.0) and OV (-3.9 to 3.2) groups. Operative mortality after BV or OV was 8% (n = 4) and 4% (n = 2), respectively. With a 13-year median follow-up, 10-year freedom from operation was 36% and 66% after BV or OV, respectively. Valve replacement was ultimately required in 32 patients (n = 20 [39%] in the BV group; n = 12 [23%] in the OV group) within a 5.9-year median time. After OV, tricuspid arrangement of the repaired aortic valve provided a 10-year freedom from operation and replacement of 87% and 95%, respectively. In multivariate analysis, associated left heart malformations, BV, nontricuspid geometry, and inadequate post procedural result were predictive of operation and replacement. CONCLUSIONS: In neonates with critical aortic stenosis, both methods (BV and OV) offer excellent survival benefit. OV significantly minimizes the need for operation, whereas BV did not postpone age of replacement. Clearly superior results are achieved with OV when a post repair tricuspid arrangement is obtained.

Outcomes after mechanical aortic valve replacement in children and young adults with congenital heart disease.

OBJECTIVES: There are little recent data on the outcomes of mechanical aortic valve replacement (AVR) in children and young adults with congenital aortic valve disease. We sought to review the survival and associated thromboembolic or bleeding complications after mechanical AVR at a single center. METHODS: Data were retrospectively collected for 121 patients undergoing prosthetic AVR from 2000 to 2014. Kaplan-Meier estimates and Cox proportional hazards were employed. RESULTS: Median age at AVR was 16 years (interquartile range, 12-22.8 years). The valves implanted were the St Jude valve (St Jude Medical Inc, St Paul, Minn) in 79 patients (62%), the On-X valve (On-X Life Technologies Inc, Austin, Tex) in 45 patients (35%), and CarboMedics (Sorin SpA, Milan, Italy) in 3 patients (2.4%). Median valve size was 23 mm (range, 21-25 mm). There were 5 early deaths (3.9%). Median follow-up was 5 years (range, 1.6-9.2 years; 600 patient-years). There were 14 deaths during follow-up. Survival was 90.6% ± 2.8% at 1 year, 85.4% ± 3.7% at 5 years, and 81.5% ± 4.5% at 10 years. Freedom from aortic valve reoperation was 98% ± 1.4% at 1 and 5 years, 91.5% ± 3.9% at 7 years, and 78.4% ± 6.9% at 10 years and at latest follow-up. Univariable analysis identified younger age, lower weight, and use of a 16-mm CarboMedics valve as predictors of reoperation. Valve sizes of 16 or 17 mm have a significantly higher risk of reoperation compared with larger valves (log-rank test, P < .001). At multivariable analysis, only younger age was a significant independent predictor of reoperation (hazard ratio, 0.84; 95% confidence interval, 0.71-0.99; P = .038). All patients were treated with warfarin to a goal international normalized ratio of 2.0 to 3.0. Four patients (3.1%; 0.66% per patient-year) had thromboembolic complications, and 5 patients (3.9%; 0.83% per patient-year) had bleeding events during follow-up. CONCLUSIONS: Mechanical AVR in patients with congenital heart disease has excellent short- and midterm outcomes. Younger age was an independent predictor of reoperation.

Immediate And Midterm Results Of Balloon Aortic Valvuloplasty In Children With Aortic Valve Stenosis With Special Reference To Dysplastic Aortic Valve.

BACKGROUND: This study was conducted to determine the immediate and midterm outcome of balloon aortic valvuloplasty in children from age 1 month to 16 years, with special reference to mean balloon to aortic valve ratio along with dooming versus dysplastic valve. METHODS: This was a retrospective cohort study. Patients who underwent balloon aortic valvuloplasty in a single tertiary care hospital were reviewed regarding immediate outcome, morphology of aortic valve, mean balloon to aortic valve ratio and complications during procedure from January 2006 till December 2016. RESULTS: Of 171 patients, 80.11% had fall to good results while 73.1% had adequate outcome. Mean gradient fall of more than 50% or Peak systolic gradient decreased significantly post ballooning, which indicates good results. The age ranges from 1-92 months, 89.4% patients from age group less than1 year had adequate outcome, (p=0.017). In terms of morphology, 55.6% cases had doming while 44.4% cases had dysplastic aortic valve. There was no significant difference between dysplastic and doming valves in terms of outcome (p=0.224). Only 6 patients (3.5%) developed significant aortic regurgitation. Regarding short-term and intermediate outcome 92% of the patients were free from a second intervention and there was no significant difference between complications regarding balloon to aortic valve ratio also. CONCLUSIONS: Our 10-year experience showed that balloon aortic valvuloplasty is a safe and effective therapy, irrespective of age groups. The complications rate is low and good outcome is achieved by keeping balloon to aortic valve ratio of ±0.9 irrespective of morphology of valve..

Immediate and Long-Term Follow Up Results of Balloon Aortic Valvuloplasty in Congenital Bicuspid Aortic Valve Stenosis Among Young Patients.

BACKGROUND: The study aim was to investigate the immediate and long-term results of balloon aortic valvuloplasty (BAV) in young patients (aged ≤20 years) with congenital bicuspid aortic valve presenting with severe aortic stenosis. METHODS: The study involved a single tertiary care center-based retrospective data analysis of immediate and long-term outcomes in patients following balloon valvuloplasty between 2000 and 2011. A total of 92 young patients (mean age 12.7 years; 95% CI 8.3-16.7 years) with aortic stenosis due to congenital bicuspid aortic valve and who underwent BAV were studied. RESULTS: The mean follow up period was 5.7 ± 1.3 years. Intervention resulted in successful BAV (≥50% reduction in baseline gradient) in 79 patients (85.9%; group A) and partially successful BAV (<50% reduction in baseline gradient) in eight patients (8.7%; group B). BAV failed in five patients (5.4%; group C). The mean left ventricular systolic pressure was decreased from 155.6 mmHg (95% CI 132.7-186.3 mmHg) to 100.9 mmHg (95% CI 82.1-119.6 mmHg; p <0.001), and the mean aortic valve gradient from 40.7 mmHg (95% CI 25.12-56.22 mmHg) to 17.2 mmHg (95% CI 15.83- 21.23 mmHg; p <0.001). Mean changes in aortic valve area and mean gradient were significantly different between successful BAV groups (A and B) and the failed BAV group (p = 0.001). Different grades of aortic regurgitation were noted in 32 patients (34.78%) after BAV (severe regurgitation in 2.18%). A post hoc analysis showed sustained gradient reductions at one- and five-year follow up investigations (p <0.05). The need for surgery was much lower in the successful BAV groups. CONCLUSIONS: The success of BAV in bicuspid aortic valve stenosis in younger patients was approximately 85%. Although 10% of patients developed re-stenosis and required surgical intervention, the majority did well during the follow up period. It was concluded that BAV is a viable option in adolescents and young children with bicuspid aortic valve without severe calcification.

Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report.

BACKGROUND: Unicuspid unicommissural aortic valve is an extremely rare congenital anomaly that usually presents in adulthood but can rarely present in infancy. We report a 17-year-old patient with congenital aortic stenosis secondary to unicuspid unicommissural aortic valve that was successfully treated with aortic valve replacement. CASE PRESENTATION: The patient was diagnosed with aortic stenosis after a murmur was heard in the newborn nursery and subsequently underwent aortic balloon valvuloplasty 6 weeks after birth. He had been regularly followed up since and underwent numerous cardiac catheterizations, including another aortic balloon valvuloplasty at age 13. During follow-up at age 17, the patient presented with symptomatic severe aortic stenosis and mild left ventricular hypertrophy. Aortic valve replacement was planned since the patient was nearly adult-sized and to reduce the risk of cardiac decompensation. During the operation an unicuspid unicommissural aortic valve was revealed. The patient recovered well post-operatively. He was discharged 5 days after the surgery in good condition and was completely symptom-free at follow-up 6 weeks later. CONCLUSIONS: Unicuspid aortic valve is a rare congenital anomaly that can cause congenital aortic stenosis. It is seldom diagnosed pre-operatively but should be suspected in infants presenting with aortic stenosis.

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

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