Expanded polytetrafluoroethylene conduits with curved and handsewn bileaflet designs for right ventricular outflow tract reconstruction.

OBJECTIVE: This study reviewed the application of curved and bileaflet designs to pulmonary expanded polytetrafluoroethylene conduits with diameters of 10 to 16 mm and characterized this conduit on in vitro experiment, including particle image velocimetry. METHODS: All patients who received this conduit between 2010 and 2022 were evaluated. Three 16-mm conduits were tested in a circulatory simulator at different cardiac outputs (1.5-3.6 L/minute) and bending angles (130°-150°). RESULTS: Fifty consecutive patients were included. The median operative body weight was 8.4 kg (range, 2.6-12 kg); 10-, 12-, 14-, and 16-mm conduits were used in 1, 4, 6, and 39 patients, respectively. In 34 patients, the conduit was implanted in a heterotopic position. The overall survival rate was 89% at 8 years with 3 nonvalve-related deaths. There were 10 conduit replacements; 5 16-mm conduits (after 8 years) and 1 12-mm conduit (after 6 years) due to conduit stenosis, and the remaining 4 for reasons other than conduit failure. Freedom from conduit replacement was 89% and 82% at 5 and 8 years, respectively. Linear mixed-effects models with echocardiographic data implied that 16-mm conduits were durable with a peak velocity <3.5 m/second and without moderate/severe regurgitation until the patient's weight reached 25 kg. In experiments, peak transvalvular pressure gradients were 11.5 to 25.5 mm Hg, regurgitant fractions were 8.0% to 14.4%, and peak Reynolds shear stress in midsystolic phase was 29 to 318 Pa. CONCLUSIONS: Our conduits with curved and bileaflet designs have acceptable clinical durability and proven hydrodynamic profiles, which eliminate valve regurgitation and serve as a reliable bridge to subsequent conduit replacement.

The Ross procedure versus repair for treatment of a unicuspid aortic valve in adults†.

OBJECTIVES: Aortic stenosis or regurgitation in patients with a unicuspid valve morphology requires interventions early in life. We have performed either primary valve repair or the Ross procedure. The goal of this study was to compare the midterm results of repair and pulmonary autograft replacement. METHODS: Between December 1998 and April 2022, a total of 345 patients (77% male; mean age 34 ± 9.7 years) underwent treatment of a unicuspid aortic valve. Patients were excluded if they were <18 years (n = 84) or >54 years (n = 3) at the time of the operation. The remaining cohort was divided into 2 groups: 167 (64%) patients underwent valve repair; 91 (36%) patients underwent pulmonary autograft replacement.The indications for surgery were aortic regurgitation (n = 104), aortic stenosis (n = 45), combined disease (n = 103) and endocarditis (n = 6). Fifty-one patients had root dilatation (>43 mm) with aortic regurgitation (repair n = 23; Ross n = 28). Mean follow-up was 5.9 years (SD: 5 years) [range 0.1-22.3 years]. RESULTS: There were 1 early and 3 late deaths; 47 patients required reintervention. Survival at 10 years was 95% in the Ross group and 97% after valve repair (P = 0.769). Freedom from reintervention at 10 years was 98% in the Ross group and 80% after valve repair (P = 0.012). A receiver operating characteristics curve analysis showed a trend towards better durability in patients < 26 years. CONCLUSIONS: The ideal treatment of the unicuspid aortic valve remains debatable. Repair of a unicuspid valve can be considered a bridge to pulmonary autograft replacement, at least in younger patients. The appropriate times to replace and to repair require further investigation.

Surgical outcomes of pulmonary artery sling and congenital tracheal stenosis with right lung anomaly.

OBJECTIVES: This study aimed to investigate whether computed tomographic indices support surgical decision-making in patients with pulmonary artery sling, congenital tracheal stenosis and right lung underdevelopment. METHODS: A total of 38 patients with pulmonary artery sling and congenital tracheal stenosis underwent tracheoplasty. Patients were divided into 3 groups based on anatomical categorization: group normal lung (NL; n = 21), group H (right lung hypoplasia, n = 12) and group A (right lung agenesis or aplasia, n = 5). Using preoperative computed tomographic images, the severity of the tracheal bending due to the right posterior deviation of the aortic arch and the relative length of the left pulmonary artery for reimplantation was evaluated. RESULTS: Slide tracheoplasty posterior to the aortic arch with pulmonary artery reimplantation was performed in 32 patients (21, 10 and 1 in groups NL, H and A, respectively). Slide tracheoplasty anterior to the aortic arch was performed in 4 patients (2 patients each in groups H and A). Among the 6 patients with severe tracheal bending who underwent slide tracheoplasty posterior to the aortic arch, 5 required aortopexy for tracheomalacia. The overall mortality rate was 3% (group NL, n = 1). The relative length of the left pulmonary artery in group H (0.85) was significantly shorter than that in group NL (1.36, P < 0.0001). CONCLUSIONS: In patients with right lung underdevelopment, preoperative computed tomography elucidated the tracheal bending due to right posterior deviation of the aortic arch, which compromised tracheoplasty and shortness of the left pulmonary artery for pulmonary artery sling repair.

A New Technique of Aortoventricular Patch Enlargement and Root Replacement for Annular Hypoplasia.

The Ross-Konno operation is effective for enlargement of a hypoplastic aortic annulus and left ventricular outflow tract. It is, however, an invasive operation with the potential for early and late cardiac morbidity. We propose a new technique of annular and outflow tract enlargement that avoids the septal incision while effectively opening up the outflow tract and aortic annulus.

Ross operation after failure of aortic valve repair.

BACKGROUND: Repair failure remains one of the most important complications of aortic valve reconstruction. Young patients might benefit from a Ross procedure in such a scenario, provided it can be performed safely and with adequate durability. The aim of this study was to assess the safety and clinical outcomes of a Ross operation following a failed repair. METHODS: Between 1996 and 2019, 80 patients (male, 76%; mean age, 31±13 years) underwent a Ross procedure after a median of 6.6 (1.7-15.9) years following an initial aortic valve repair. The previous valve repair was performed for unicuspid (53%), bicuspid (39%), tricuspid (7%), and quadricuspid morphology (1%). Median follow-up after the Ross operation was 2.8 (0.964-13.25) years, mean 5±5 years (92% complete). RESULTS: Median cardiopulmonary bypass and cross-clamp times were 144 [106-154] minutes and 98 [79-113] minutes, respectively. Thirty-two patients (40%) required a concomitant procedure, most commonly, an ascending aortic replacement (n=23). There were no peri-operative deaths, myocardial infarctions, or neurological complications. There was one late death from a non-cardiac cause. At 10 years, overall survival was 99%±1%, similar to that of an age- and gender-matched population. Nine patients required re-intervention after their Ross procedure (five on the autograft and four on the pulmonary conduit). The autograft re-interventions were valve-sparing procedures in all patients. The cumulative incidence of re-intervention on the autograft at 8 years was 5.1%±3.1%. CONCLUSIONS: The stepwise strategy of an initial valve repair followed by Ross operation represents a safe and valid option for failed aortic valve repair. It is associated with low peri-operative morbidity. Mid-term survival is excellent, similar to that of a matched general population. The probability of re-intervention after the Ross procedure appears similar to that of a primary Ross operation, deeming it a warranted consideration in cases of failed aortic valve repair.

Unicuspid aortic valve repair with bicuspidization in the paediatric population.

OBJECTIVES: Aortic stenosis or regurgitation that requires operations in children often results from unicuspid valve morphology. In all paediatric patients with this anomaly, we have performed unicuspid valve repair by bicuspidization, creating a new commissure via adding patch material. This study reviewed our experience with this procedure. METHODS: All patients with a unicuspid aortic valve who underwent bicuspidization at ≤18 years of age between 2003 and 2018 were evaluated. Autologous pericardium had initially been used for cusp augmentation. Since 2014, decellularized xenogeneic tissue or expanded polytetrafluoroethylene membrane has been applied. RESULTS: There were 60 consecutive patients. The median operative age was 13 (1-18) years. Thirty patients had prior surgical or catheter valvuloplasties. Aortic regurgitation, aortic stenosis and their combination were present in 22, 11 and 27 patients, respectively. Autologous pericardium decellularized tissue and expanded polytetrafluoroethylene were used in 45, 11 and 4 patients, respectively, without intraoperative conversion to valve replacement. The overall survival was 96% at 10 years with 2 late deaths. Twenty patients underwent aortic valve reoperation due to patch degeneration (n = 11), suture dehiscence of the patch (n = 3), subaortic stenosis (n = 3) or other reasons (n = 3). Freedom from aortic valve reoperation was 73% and 50% at 5 and 10 years, respectively. Fifteen patients (25%) required valve replacement with pulmonary autograft (n = 14) or prosthesis (n = 1) 5.2 (0.2-13) years after bicuspidization at the age of 19 (10-32) years. CONCLUSIONS: Bicuspidization is a safe and reproducible alternative to valve replacement with acceptable freedom from reoperation. It can serve as a bridge to valve replacement providing superior outcomes in adults.

Selective Lesser Curvature Augmentation With Geometric Study for Repair of Aortic Arch Obstruction.

BACKGROUND: We repaired aortic coarctation and interrupted aortic arch with extended end-to-end anastomosis (EAA) through median sternotomy and performed lesser curvature augmentation with a pulmonary autograft patch (PAP) in selected patients with a long gap between anastomotic sites. We reviewed these outcomes and geometric implications. METHODS: All neonates and infants with biventricular morphology who underwent aortic arch reconstruction through median sternotomy between 2005 and 2019 were evaluated. Aortic arch geometry was analyzed with computed tomography routinely performed before and after surgery from 2009 on. RESULTS: There were 91 consecutive patients (median age, 1.2 months). Ten patients received PAP. One early death and no late deaths were noted. Overall survival was 98.9% at 10 years. Two left bronchomalacia and 1 recoarctation occurred in patients with EAA. Freedom from recoarctation was 97.4% at 10 years. We examined 68 patients with computed tomography. We used PAP in patients with a significantly longer gap between anastomotic sites indexed by the square root of the body surface area; its cutoff value was 29.0 mm/m (area under the curve, 0.86 mm/m). The PAP created a significantly greater arch angle (median, 91° versus 83°) and arch/descending diameter ratio (median, 1.2 versus 1.0) and preserved the arch width indexed by the square root of the body surface area (median, before surgery: 35.7 versus 34.4 mm/m; after surgery: 36.5 versus 29.9mm/m), compared with EAA. CONCLUSIONS: Aortic arch reconstruction with the current combined strategy provides satisfactory outcomes. Guided by geometric analysis, lesser curvature augmentation can be applied to patients who might experience recoarctation or airway compression with a directly anastomosed aortic arch.

Cusp-based geometric height-oriented repair of a pediatric aortic valve.

Progressive aortic regurgitation can occur in pediatric patients due to root dilation with conotruncal anomalies or cusp prolapse associated with a ventricular septal defect. It is treated using various approaches influenced by personal preferences and institutional experience. We applied geometrical concepts developed for adult aortic valve repair to pediatric valves. The basal ring and sinotubular junction are downsized in relation to the geometric height of the cusp by external suture annuloplasty. The length of the cusp free margin is then adjusted with central plication, guided by measuring the effective height of the cusp. This approach facilitates the reproducibility and predictability of pediatric aortic valve repair.

Geometry of cusp and root determines aortic valve function.

The aortic valve is the functional unit of cusp and root. Various geometrical and functional analyses for the aortic valve unit have been executed to understand normal valve configuration and improve aortic valve repair. Different concepts and procedures have then been proposed for reparative approach, and aortic valve repair is still not standardized like mitral valve repair. It has become apparent, however, that interpretation of the geometry of the aortic cusp and root and its appropriate application to operative strategy lead to creating a functioning aortic valve. Herein, the aortic valve geometry and its clinical implications are reviewed to provide information for the selection of appropriate operative strategies.

Aortic root remodeling in bicuspid and tricuspid aortic valves-long-term results.

BACKGROUND: Root remodeling is one form of valve-preserving root replacement to treat patients with aortic regurgitation (AR) and root aneurysm. We have consistently used it for patients with and without connective tissue disease and different aortic valve morphologies. The objective of this retrospective study was to review 23 years of experience with root remodeling. METHODS AND PATIENTS: Between 10/95 and 9/2018, 1004 of 1038 root remodeling procedures were performed in patients with tricuspid (n = 589) or bicuspid (n = 414) anatomy of the aortic valve in our institution. Aortic aneurysm was present in 932 cases, 73 procedures were performed for acute aortic dissection type A. The severity of aortic regurgitation ranged from grade I to grade IV (mean 2.5 ± 0.8). RESULTS: All patients underwent root remodeling, concomitant operations were performed in 433, and cusp repair in 883 instances. Hospital mortality was 2%. Overall freedom from reoperation was 92% at 10 years and 89% at 15 years. It was 94% for tricuspid valves at 10 and 15 years, and 88% for bicuspid aortic valves at 10 years and 80% at 15 years (p = 0.003). CONCLUSION: In conclusion, root remodeling is a viable option in valve-preserving root replacement. If combined with careful assessment and, if necessary, correction of aortic valve form reproducible restoration of aortic valve function can be achieved.

Mid-term durability of polytetrafluoroethylene patches in unicuspid aortic valve repair.

OBJECTIVES: Unicuspid aortic valve (UAV) morphology is a cause for aortic valve dysfunction in childhood or adolescence. Repair requires the use of patch material, and polytetrafluoroethylene (PTFE) has been proposed for this purpose because of lack of calcification. We reviewed our mid-term experience with PTFE for the repair of UAV to analyse the durability of this technique. METHODS: Out of 21 patients with an UAV undergoing aortic valve repair for severe aortic regurgitation between 2014 and 2016, 11 patients (52%) were treated using PTFE patch material. Aortic regurgitation was present in all patients, the primary indication for surgery was regurgitation in 8, stenosis in 2 and aneurysm in 1. Symmetric bicuspidization of the UAV was performed in all. One patient required additional root remodelling for root dilatation, and another 3 tubular ascending aortic replacement. RESULTS: No patient died in hospital or during follow-up. Seven patients (63.6%) required reoperation for progressive AR. Freedom from reoperation was 58% at 1 and 35% at 5 years postoperatively. At reoperation the PTFE patches were found dehisced from aortic wall and/or native cusp tissue. In 3 patients re-repair was performed; a stable result was achieved in 1. Two patients underwent valve replacement 3 months and 1 year postoperatively. The other 4 patients underwent valve replacement. CONCLUSIONS: The repair of UAVs using PTFE patch is associated with poor durability, a more durable patch with better healing characteristics material is needed.

Switch to Extracorporeal Membrane Oxygenation During Cardiac and Tracheal Repair.

Simultaneous repair of congenital tracheal and cardiovascular lesions remains challenging in small patients. We describe two infants weighing less than 3 kg who underwent successful tracheoplasty with concomitant correction of complex heart anomalies. In both operations, cardiopulmonary bypass was switched to extracorporeal membrane oxygenation after cardiac repair to optimize hemostatic function with transfusion and maintain activated clotting time at 200 to 240 seconds. Slide tracheoplasty was performed in a bloodless field, which prevented intraoperative hemorrhage from running down the divided lower trachea into the lung and causing airway obstruction. Both patients were weaned from extracorporeal support during surgery and extubated within 9 days.

Computed tomography-based surgical strategy for total anomalous pulmonary venous connection.

OBJECTIVES: We used computed tomographic angiography (CTA)-based surgical planning to clarify the anatomical indications of sutureless repair technique for total anomalous pulmonary venous connection. The mid-term impact of the current surgical strategies was evaluated. METHODS: One hundred twelve patients underwent repair for total anomalous pulmonary venous connection. The study period was divided into era 1 (1996-2010, n = 56) and era 2 (2011-2018, n = 56). Patients with single ventricular heart (SVH) were included. In era 2, the indications for primary sutureless repair and branch pulmonary vein incision were based on CTA findings. RESULTS: For patients with biventricular heart, the 5-year survival was 69% and 97% in eras 1 and 2, respectively (P = 0.0024). For patients with SVH, the 5-year survival was 21% and 70% in eras 1 and 2, respectively (P = 0.0007). During the follow-up period, the evidence of post-repair pulmonary vein stenosis (PVS) was observed in 12 patients with biventricular heart [era 1, 8 patients (23%); era 2, 4 patients (13%)], and 14 patients with SVH [era 1, 6 patients (60%); era 2, 8 patients (36%)]. Using multivariable analysis, preoperative CTA was associated with improved survival in both biventricular heart and SVH and associated with post-repair PVS-free survival in SVH. Since 2011, 12 patients with post-repair PVS underwent multiple reintervention with 1 recorded death (5-year survival: 88%). CONCLUSIONS: CTA-based surgical strategy for total anomalous pulmonary venous connection provided significant survival benefit. Although post-repair PVS could occur in era 2, aggressive reintervention appeared to be associated with improved survival and vein patency.

Reexamining remodelling in children.

OBJECTIVES: Some studies have suggested that root remodelling is unsuitable as valve-sparing aortic root replacement in children because of the increased risk of valve failure. This study reviewed our experience with root remodelling in children. METHODS: All patients who underwent root remodelling at the age of ≤18 years between 1999 and 2016 were evaluated. In 2004, cusp effective height was measured intraoperatively and prolapse was corrected with central plication on the cusp. Suture annuloplasty was introduced after 2009 for annular dilatation. RESULTS: There were 17 consecutive patients. The median age at operation was 14 (2.8-18) years. Aortic valve morphology was tricuspid in 10 patients, bicuspid in 5 patients, unicuspid in 1 patient and a pulmonary autograft in 1 patient. Marfan syndrome, Loeys-Dietz syndrome and other connective tissue diseases were present in 11, 1 and 2 patients, respectively. Five patients had more than moderate aortic regurgitation. The median graft size used for root remodelling was 24 (18-26) mm. Cusp repair and annuloplasty were performed in 15 and 14 patients, respectively. The mean follow-up time was 6.5 ± 4.3 years. One patient with preoperatively severely depressed ventricular function died in the hospital from persistent heart failure. One patient (operated on before 2004) required aortic valve reoperation due to cusp prolapse. One patient with a unicuspid valve had developed moderate aortic regurgitation, and the other 14 patients had mild or less regurgitation. The median diameter of the sinus of Valsalva at the last follow-up was 36 (30-43) mm, Z-score of 1.5 (-3.5 to 3.9). CONCLUSIONS: Root remodelling can be performed in children with favourable results by appropriate cusp repair and annuloplasty.

Bicuspidization and Annuloplasty Provide a Functioning Configuration to the Unicuspid Aortic Valve.

BACKGROUND: This study reviewed a 12-year experience with a bicuspidization procedure that created a new commissure and added a patch for unicuspid aortic valve repair. METHODS: All patients with a unicuspid aortic valve who underwent bicuspidization with autologous pericardium between 2003 and 2014 were evaluated. The new commissure had initially been created on the rudimentary anterior commissure, and since 2007, symmetric orientation was designed. Suture external annuloplasty was introduced after 2009 for basal ring dilatation. RESULTS: There were 137 consecutive patients. The median age at operation was 26 years (range, 3 to 64 years). Aortic regurgitation, aortic stenosis, and combined aortic regurgitation and stenosis were present in 68 (50%), 15 (11%), and 54 (39%) patients, respectively. A total of 71 patients were operated on after 2009. Annuloplasty was added in 47 patients. There were 2 early and 3 late deaths. Overall survival was 96% at 10 years. A total of 47 patients required aortic valve reoperation because of patch degeneration (n = 20), suture dehiscence of patch (n = 17), and other causes (n = 10). Freedom from aortic valve reoperation was 77% and 59% at 5 and 10 years, respectively, and 82% and 75% at 5 and 8 years in patients operated on after 2009, respectively. Suture dehiscence of the patch occurred significantly more often in patients with a basal ring diameter of 28 mm or larger before 2009 (38% in 5 years), and it decreased significantly with annuloplasty (9% in 5 years). CONCLUSIONS: Bicuspidization and annuloplasty create a functioning valve configuration in unicuspid aortic valve repair. Patch degeneration remains the main cause of reintervention and is the limiting factor for durability.

The Konno Operation Is a Durable Option for Relief of Aortic Stenosis in Patients With Complex Left Ventricular Outflow Tract Obstruction-A Single-Center 20-Year Experience.

BACKGROUND: A Konno operation with a mechanical prosthesis may be applied in patients with complex left ventricular outflow tract obstruction to avoid further operations. We reviewed our 20-year experience with the Konno operation. METHODS: All patients who underwent the Konno operation between 1996 and 2015 were evaluated. Study end points were survival and reoperations. RESULTS: Twenty-one consecutive patients were included. The median age at operation was 12 years (5 months to 34 years). Twenty (95%) patients had prior interventions for left-sided heart lesions. Additional mitral valve disease was present in 17 (81%) patients. The preoperative mean pressure gradient over the left ventricular outflow tract was 50 ± 25 mm Hg. The median size of implanted valve prostheses was 21 mm (16-25 mm). Concomitant procedures for left-sided heart lesions were performed in six patients, including two mitral valve replacements. There were two hospital mortalities (9.5%) and four late mortalities (19%). Overall survival was 85% ± 7.8% and 72% ± 11% at 5 and 10 years, respectively. In two patients, mitral valve replacement was performed during the same hospital admission. During a mean follow-up time of 7.6 ± 4.8 years, two patients required late reoperations, one for mitral valve replacement and one for heart transplantation. Freedom from late reoperation at 10 years was 89% ± 7.4%. CONCLUSIONS: The Konno operation can be considered as a definitive option with a low probability of reoperation on the left ventricular outflow tract in patients with complex left ventricular heart disease. Subsequent operations focus on the treatment of additional mitral valve disease, which remains the cause of mortality and morbidity.

Cusp Nadir Relocation by Root Remodeling in Unicuspid Aortic Valve Repair.

We describe a root remodeling technique for the unicuspid and unicommissural aortic valve with a moderately hypoplastic anterior commissure. This technique achieves symmetric bicuspidization without patch insertion to cusps. Partial cusp fusion of the anterior commissure is divided, and two symmetric tongues of a Dacron graft are sutured to each center of cusp insertion lines. The cusp nadirs are relocated posteriorly, and the bicuspidized valve has two sufficiently high commissures from a new angulated virtual basal ring. To optimize valve configuration, annuloplasty and prolapse correction according to effective height concept are added. This technique is a promising option in select patients.

Myosin binding protein H-like (MYBPHL): a promising biomarker to predict atrial damage.

Myosin binding protein H-like (MYBPHL) is a protein associated with myofilament structures in atrial tissue. The protein exists in two isoforms that share an identical amino acid sequence except for a deletion of 23 amino acids in isoform 2. In this study, MYBPHL was found to be expressed preferentially in atrial tissue. The expression of isoform 2 was almost exclusively restricted to the atria and barely detectable in the ventricle, arteria mammaria interna, and skeletal muscle. After atrial damage induced by cryo- or radiofrequency ablation, MYBPHL was rapidly and specifically released into the peripheral circulation in a time-dependent manner. The plasma MYBPHL concentration remained substantially elevated up to 24 hours after the arrival of patients at the intensive care unit. In addition, the recorded MYBPHL values were strongly correlated with those of the established biomarker CK-MB. In contrast, an increase in MYBPHL levels was not evident in patients undergoing aortic valve replacement or transcatheter aortic valve implantation. In these patients, the values remained virtually constant and never exceeded the concentration in the plasma of healthy controls. Our findings suggest that MYBPHL can be used as a precise and reliable biomarker to specifically predict atrial myocardial damage.