Replacement In Children Research Articles

Ross Versus Non-Ross Aortic Valve Replacement in Children: A 22-Year Single Institution Comparison of Outcomes

The Ross aortic valve replacement (AVR) has been the AVR of choice for children at our center since 1993. Absence or inadequate quality of the pulmonary valve or, less commonly, family or surgeon preference caused us to select an alternative AVR prosthesis for some children. This review compares the outcomes of 42 children who received a non-Ross AVR with 115 children undergoing Ross root replacement at our institution during the most recent 22 years. A retrospective chart review of the 42 pediatric non-Ross AVRs was compared with 115 Ross AVRs. The mean age at AVR was 11.0 ± 6.5 years (range, 1 month to 18 years) for the non-Ross and 11.4 ± 5.5 years (range, 6weeks to 18 years) for the Ross groups. Follow-up was 7.8 ± 6.2 years for the Ross group and 8.7 ± 6.5 years for the non-Ross group. The Ross AVR technique was modified in 2000; these modifications were applied to 72 patients (63%) of the total Ross AVR group. All-cause early and late mortality of the non-Ross AVR group was 17% compared with 4% for the Ross cohort (p= 0.017). The actuarial survival rate at 20 years for the non-Ross AVR group was 81% compared with 94% for the Ross group (p= 0.018). Reintervention rate was 29% (12/38) in the non-Ross group and 28% (32/115) in the entire Ross group (60% [26/43] before 2000, and 8% [6/72] after 2000). Thromboemboli, bleeding, and endocarditis occurred in 2.4%, 1.4%, and 10% in the non-Ross group compared with 0.9%, 2.6%, and 1.7% in the Ross AVR group (p= 0.46, 0.01, and 0.02), respectively. Patients in the non-Ross group had a significantly higher recurrent or persistent aortic valve gradient (>20 mm Hg) than did patients in the Ross group at most recent follow-up (p < 0.05). Lower mortality, valve-related complications, and better hemodynamics were seen after the Ross AVR than with other types of AVR prostheses. The Ross AVR remains the treatment of choice for children who have an adequate pulmonary valve. Reoperation for Ross root dilatation, regurgitation, or both (only 6% since our modifications in 2000) has markedly reduced the main drawbacks of this AVR technique. The Ross AVR affords the growing child the advantages of a growing, autologous, viable valve substitute and eliminates the lifelong disadvantages of mechanical and xenograft valves.

The Midterm Outcomes of Bioprosthetic Pulmonary Valve Replacement in Children

The purpose of this study was to assess the outcomes of bioprosthetic pulmonary valve replacement (PVR) in children. This is a retrospective review of all bioprosthetic PVR in children (≤ 20-year old) between 1992 and 2013 at a single institution. Most outcomes studied included pulmonary valve reintervention and bioprosthetic valve function. A total of 136 bioprosthetic PVRs were identified for 123 patients. The median age and body weight at the time of operation were 13.2 years and 48.4 kg. There were 1 early death and 3 late deaths during the median follow-up of 7.2 years (0-22.0 years). The actuarial transplant-free survival was 97.6% at 10 years. There were 43 bioprosthesis reinterventions with 29 reoperations and 14 catheter-based interventions. The freedom from bioprosthesis reintervention was 89.6% and 55.0% at 5 and 10 years, respectively. Echocardiographic bioprosthesis dysfunction (≥ moderate bioprosthesis insufficiency, ≥ 50 mmHg peak gradient through bioprosthesis, or bioprosthesis endocarditis with vegetation) was found in 57 bioprostheses. The freedom from bioprosthesis dysfunction was 74.0% and 32.8% at 5 and 10 years, respectively. Results from the Cox proportional hazards models showed that age had significant association with freedom from bioprosthesis reintervention and freedom from bioprosthesis dysfunction (P < 0.001 and P = 0.03), whereas bioprosthesis type had nonsignificant association with freedom from bioprosthesis dysfunction (P = 0.068). Bioprosthetic PVR in children had excellent early outcomes but rapidly deteriorating midterm outcomes. Careful and close follow-up are necessary for children with bioprosthesis in the pulmonary position.

Right ventricular Hemodynamic Alteration after Pulmonary Valve Replacement in Children with Congenital Heart Disease

Introduction:  In patients who underwent surgery to repair Tetralogy of Fallot, right ventricular dilation from pulmonary regurgitation may be result in right ventricular failure, arrhythmias and cardiac arrest. Hence, pulmonary valve replacement may be necessary to reduce right ventricular volume overload. The aim of present study was to assess the effects of pulmonary valve replacement on right ventricular function after repair of Tetralogy of Fallot.  Materials and  Method:  This retrospective study was carried out between July 2011 and October 2013 on 21 consecutive patients in Chamran Heart Center (Esfahan). The study included 13 male (61.9) and 8 female (38.1). Cardiac magnetic resonance was performed before, 6 and 12 months after pulmonary valve replacement in all patients (Babak Imaging Center, Tehran) with the 1.5 Tesla system. The main reason for surgery at Tetralogy of Fallot repaired time was Tetralogy of Fallot + Pulmonary insufficiency (17 cases) and Tetralogy of Fallot + Pulmonary atresia (4 cases). Right ventricular function was assessed before and after pulmonary valve replacement with Two-dimensional echocardiography and ttest was used to evaluate follow-up data.  Results:  Right ventricular end-diastolic volume, right ventricular end- systolic volume significantly decreased (P value E� 0.05).Right ventricular ejection fraction had a significant increase (P value E� 0.05). Right ventricular mass substantially shrank after pulmonary valve replacement. Moreover, pulmonary regurgitation noticeably decreased in patients. The other hemodynamic parameter such as left ventricular ejection fraction improved but was not significant (P value= 0.79). Conclusion:  Pulmonary valve replacement can successfully restores the impaired hemodynamic function of right ventricle which is caused by direct consequence of volume unloading in patient. Pulmonary valve surgery in children with Tetralogy of Fallot who have moderate to severe pulmonary regurgitation leads to an improvement of right ventricular function. Â

Trends in Pulmonary Valve Replacement in Children and Adults With Tetralogy of Fallot

Operative correction of tetralogy of Fallot frequently results in pulmonary insufficiency and chronic volume overload, which have been linked to increased risk for adverse outcomes. No consensus recommendations for the timing of pulmonary valve replacement (PVR) exist. The aim of this study was to examine the pattern of PVR in the United States from 2004 to 2012. The Pediatric Health Information Systems database was used to perform an observational study of children and adults ≥10years of age with diagnoses of tetralogy of Fallot who underwent PVR at 35 centers in the United States from 2004 and 2012, to assess the rate of PVR and the age at which is performed. Mixed-effects multivariate regression was used to account for patient-level covariates and center-level covariance. Additional analyses assessed for trends in cost, hospital length of stay (LOS), intensive care unit LOS, and in-hospital mortality over the study period. In total, 799 subjects at 35 centers underwent PVR over the study period. The number of PVRs performed per year increased significantly over the study period. There was significant between-center heterogeneity in age at PVR (p <0.001). Age at PVR, intensive care unit LOS, hospital LOS, and cost did not change over the study period. In conclusion, PVR in patients with tetralogy of Fallot is being performed more frequently, without an accompanying change in the age at PVR or other measurable outcomes. There issignificant variability in the age at which PVR is performed among centers across the United States. This highlights the need for additional research guiding the optimal timing of PVR.

THU0374 Vitamin D Levels and Effects of Vitamin D Replacement in Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome

BackgroundThe periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome, is an autoinflammatory disease characterised by regularly recurrent fever episodes, due to seemingly unprovoked inflammationObjectivesTo assess serum 25-hydroxy cholecalciferol...

Aortic Valve Repair and Replacement in Children

Several aortic valve (AV) pathologies might necessitate intervention. Percutaneous or surgical AV repair is generally recommended as the initial management strategy in children with AV disease, offering the advantage of stabilization of the heart dimensions and improvement of patients' symptoms. When AV repair is not possible or fails, AV replacement is necessary and is associated with several challenges in children. This review will focus on treatment strategy, AV repair techniques, AV replacement choices and outcomes of AV disease management in children.

Is the Ross Procedure a Suitable Choice for Aortic Valve Replacement in Children With Rheumatic Aortic Valve Disease?

Ross procedure is the aortic valve replacement of choice in children. Nonetheless, late autograft reoperation for dilatation and/or regurgitation is concerning. We examine whether Ross procedure is suitable in children with rheumatic fever. Medical records of 104 children with rheumatic fever who underwent Ross procedure were reviewed (1991-2004). Competing risks methodology determined time-related prevalence and associated factors for two mutually exclusive end states: autograft reoperation and death prior to subsequent reoperation. Mean age was 13.8 ± 2.7, 83 (80%) were males. Hemodynamic dysfunction was primarily regurgitation (n = 92, 88%) and stenosis/mixed (n = 12, 12%). Competing risks analysis showed that in ten years after the Ross procedure, 1% of patients died, 32% underwent autograft reoperation, and 67% were alive and free from reoperation. Ten-year freedom from aortic regurgitation greater than or equal to moderate was 63%. Ten-year freedom from autograft reoperation was 65% for regurgitation versus 90% for stenosis/mixed disease. Risk factors for autograft reoperation were earlier surgery year (PE: 0.26 ± 0.06 per year; P < .001), additional surgery (PE: 0.82 ± 0.39, P = .04), no annular stabilization (PE: 1.21 ± 0.61, P = .05). Ten-year freedom from homograft replacement was 83%. Risk factors were fresh homografts (PE: 1.36 ± 0.71; P = .06) and aortic homografts (PE: 1.15 ± 0.59; P = .05). Ten-year freedom from any cardiac reoperation was 53%. Concomitant cardiac surgery was risk factor (PE: 1.37 ± 0.47; P = .004). Ross procedure in children with rheumatic fever is associated with excellent survival but results are plagued by aortic regurgitation and frequent autograft reoperation. Risk factors include preoperative regurgitation, concomitant surgery, dilated annulus, and earlier surgery era. Better patient selection in later era has mitigated autograft reoperation risk. Continued, improved candidate selection, along with modifications in autograft implantation and root/sinotubular stabilization techniques, may further decrease late autograft failure.

小児(15歳未満)における生体弁人工弁置換術の遠隔期成績

小児(15歳未満)における生体弁人工弁置換術の遠隔期成績

Age Less Than Two Years Is Not a Risk Factor for Mortality After Mitral Valve Replacement in Children

Outcomes for mitral valve replacement (MVR) in the pediatric population are generally reported as poor, particularly in patients less than 2 years old. We compared long-term morbidity and mortality associated with MVR between patients less than 2 years old and patients 2 to 18 years old. We evaluated pediatric patients undergoing MVR from March 1990 to November 2007 at our institution. Morbidity and mortality was compared between patients less than 2 years and patients 2 to 18 years old. Primary endpoints measured were postoperative survival, long-term survival, reoperation, cerebrovascular accident or transient ischemic attack, and significant bleeding events. Forty-five patients underwent 54 MVRs. Median age was 3.1 years; 18 patients were under 2 years. Median follow-up time was 5.4 years. There was no statistically significant difference between long-term or short-term survival between the two age groups, with 30-day survival of 89% (younger patients) versus 100% (older patients), and 10-year survival of 82% (younger patients) versus 85% (older patients). Freedom from reoperation for the younger age group was 40% at 10 years versus 96% for the older patients, p = 0.003. In our population, there was no statistically significant difference in survival between patients less than 2 years and patients 2 to 18 years. In children undergoing MVR, age less than 2 years remains a risk factor for reoperation but not for mortality.

Long-Term Outcome After Annular Mechanical Mitral Valve Replacement in Children Aged Less Than Five Years

When mitral valve repair in children is not possible, mitral valve replacement (MVR) is an alternative, which could result in better outcomes. We report the long-term outcome after replacement of systemic atrioventricular valve with bileaflet mechanical valves in annular position in patients less than 5 years of age. Between 1983 and 2008, 29 MVRs were performed in children aged 1.82 ± 1.34 years (range, 95 days to 4.6 years); the average weight was 8.06 ± 2.92 kg (range, 4.7 to 15 kg). The main indication (86%) was severe regurgitation. Prosthesis size ranged between 17 mm and 25 mm. The follow-up was completed and totaled 373 patient-years (12.4 ± 8.6). Four children presented with permanent atrioventricular block. Freedom from thromboembolic and hemorrhage events at 1, 5, 15 years was 100%, 96.1%, and 91.3%, respectively. All deaths occurred within 2 months after MVR and were 5 children (17%). No late mortality was observed. Freedom of redo MVR at 5, 10, and 15 years was 95%, 86%, and 70%, respectively. At MVR, weight 7 kg or less and age 1 year or less (p = 0.03) were associated with redo MVR. During redo MVR, a larger mechanical valve was implanted (+2 sizes), with no postoperative death. Currently, 23 patients are in New York Heart Association class I, and 1 patient is in class II. The results of mechanical annular MVR are acceptable in children. Anticoagulants are well tolerated, with little thromboembolic complication. Long-term results for patients who survive the hospital period are excellent, and reinterventions show safe results.

Frequency and Methods of Gastrojejunal Tube Replacement in Children

Gastrojejunal (GJ) tubes are increasingly used for enteral feeding in children in whom gastric feeding either is not tolerated or is contraindicated. The most common complications associated with GJ tube use are mechanical failure (clogging, cracking, deterioration) and dislodgement. Less common, but more significant, complications are bowel perforation, aspiration, and feeding intolerance. Some of these complications may be prevented by replacing GJ tubes at regular intervals. Methods to direct a GJ tube include 1) guidewire, 2) gastroduodenoscopy-guidewire, 3) esophagogastroduodenoscopy-forceps, and 4) fluoroscopy-guidewire. Clinical experience to determine the ideal method and optimal timing of GJ tube replacement is evolving. GJ tube replacement using a guidewire through the prior GJ tube, without endoscopy, fluoroscopy, or sedation, is the least risky method, but is also the least likely to be successful.

Mechanical Aortic Valve Replacement in Children and Adolescents After Previous Repair of Congenital Heart Disease

Due to improved outcome after surgery for congenital heart defects, children, adolescents, and grown-ups with congenital heart defects become an increasing population. In order to evaluate operative risk and early outcome after mechanical aortic valve replacement (AVR) in this population, we reviewed patients who underwent previous repair of congenital heart defects. Between July 2002 and November 2008, 15 (10 male and 5 female) consecutive patients (mean age 14.5 +/- 10.5 years) underwent mechanical AVR. Hemodynamic indications for AVR were aortic stenosis in four (27%), aortic insufficiency in eight (53%), and mixed disease in three (20%) after previous repair of congenital heart defects. All patients had undergone one or more previous cardiovascular operations due to any congenital heart disease. Concomitant cardiac procedures were performed in all of them. In addition to AVR, in two patients, a mitral valve exchange was performed. One patient received a right ventricle-pulmonary artery conduit replacement as concomitant procedure. The mean size of implanted valves was 23 mm (range 17-29 mm). There were neither early deaths nor late mortality until December 2008. Reoperations were necessary in five (33%) and included implantation of a permanent pacemaker due to complete atrioventricular block in two (15%), mitral valve replacement with a mechanical prosthesis due to moderate to severe mitral regurgitation in one (7%), aortocoronary bypass grafting due to stenosis of a coronary artery in one (7%), and in one (7%), a redo subaortic stenosis resection was performed because of a secondary subaortic stenosis. At the latest clinical evaluation, all patients were in good clinical condition without a pathological increased gradient across the aortic valve prosthesis or paravalvular leakage in echocardiography. Mechanical AVR has excellent results in patients after previous repair of congenital heart defects in childhood, even in combination with complex concomitant procedures. Previous operations do not significantly affect postoperative outcome.

Frequency and Methods of Gastrojejunal Tube (GJT) Replacement in Children - A Single Center Experience

Frequency and Methods of Gastrojejunal Tube (GJT) Replacement in Children - A Single Center Experience

Outcome After Mechanical Aortic Valve Replacement in Children and Young Adults

We asked whether aortic valve replacement using a mechanical prosthesis would allow normalization of left ventricular function and structure in children and young adults. We performed a clinical follow-up examination in 30 patients with aortic valve replacement at 25 years of age or younger, including conventional and tissue Doppler echocardiography and magnetic resonance imaging. Aortic valve replacement was performed at the median age of 14.3 years (range, 7.6 to 24.3 years) using a mechanical prosthesis (St. Jude Medical; median diameter, 23 mm; range, 17 to 27 mm). Indications were severe aortic stenosis in 6 of 30 patients, aortic regurgitation in 20 of 30 patients, or a combination of aortic stenosis and regurgitation (4 of 30 patients). Aortic valve replacement was a reoperation in 12 of 30 patients who primarily underwent aortic valvotomy at a median of 7.1 years (range, 1.0 to 11.3 years). In-hospital mortality was 0%. Follow-up was a median of 6 years (range, 1.2 to 14.5 years). Twenty-nine of 30 patients were in New York Heart Association functional class I without thromboembolic complications, cerebrovascular accidents, or major bleeding on oral anticoagulation. Left ventricular dilatation before aortic valve replacement was present in 20 of 30 patients but normalized in all but 4 patients on follow-up. Most patients showed a normal end-diastolic volume on magnetic resonance imaging, and 23 of 26 patients showed a normal left ventricular ejection fraction (median, 0.53; range, 0.33 to 0.75). Peak systolic strain of the left ventricular myocardium was a median of -13.3% (range, -0.5% to -31%), and was normal in 28 of 30 patients. Aortic valve replacement in children and young adults offers a good treatment option and may lead to normalization of left ventricular size and function in most patients.

Oesophageal Replacement in Children

The usual indications for oesophageal replacement in childhood are intractable corrosive strictures and long-gap oesophageal atresia. Generally, paediatric surgeons attempt to preserve the native oesophagus with repeat dilatations. However, when this is not successful, an appropriate conduit must be fashioned to replace the oesophagus. The neo-oesophagus should allow normal oral feeding, not have gastro-oesophageal reflux, and be able to function well for the life-time of the patient. A Medline search for oesophageal replacement, oesophageal atresia, gastric transposition, colon transposition, gastric tube, caustic stricture was conducted. The commonest conduits including whole stomach, gastric tube, colon and jejunum are all discussed. No randomised controlled studies exist comparing the different types of conduits available for children. The techniques used tend to be based on personal preference and local experience rather than on any discernible objective data. The biggest series with long-term outcome are reported for gastric transposition and colon replacement. Comparison of a number of studies shows no significant difference in early or late complications. Early operative complications include graft necrosis, anastomotic leaks and sepsis. Late problems include strictures, poor feeding, gastro-oesophageal reflux, tortuosity of the graft and the development of Barrett's oesophagus. The biggest series, however, seem to have lower complications than small series probably reflecting the experience, built up over years, in their respective centres. Long-term follow-up is recommended because of the risks of late strictures, excessive tortuosity of the neo-oesophagus and the development of Barrett's oesophagus.

Complex Aortic Valve Repair as a Durable and Effective Alternative to Valve Replacement in Children With Aortic Valve Disease

Complex Aortic Valve Repair as a Durable and Effective Alternative to Valve Replacement in Children With Aortic Valve Disease

Oesophageal Replacement in Children with Indolent Stricture of the Oesophagus

To evaluate the indications for and results of oesophageal replacement for acquired oesophageal stricture in children. This was a descriptive interventional study. Between 1987 and 2003, patients who had peptic or corrosive stricture were included in the study. Age and gender were analysed and any history of ingestion of caustic agents or untreated reflux was noted. Types of symptoms such as progressive dysphagia to solids and liquids and extent of respiratory infections were evaluated. Nutritional deprivation was also measured. Success or failure of dilatation at the time of endoscopy was taken into account. The criterion for oesophageal replacement was recurrence of stricture within 3 weeks of the previous dilatation. Patients were prepared with general supportive care. Feeding gastrostomy was performed in 12 patients who were severely malnourished. No patients underwent oesophagectomy of the strictured oesophagus. Short- and long-term complications were recorded. Of the 54 patients, 34 were male and 20 were female. Colonic conduit and reverse gastric tube were used in 27 patients each. Three patients had leak and were treated conservatively. Six patients developed anastomotic stricture but benefited remarkably from periodic dilatation and, in the long-term, remained symptom-free. Results were generally very gratifying. Most patients who require oesophageal conduit do well and catch up with their growth and development.

Early Experience With Valve-Sparing Aortic Root Replacement in Children

Aortic valve-sparing root replacement has become an established treatment for adults with aneurysms of the ascending aorta, but there is limited experience in children, for whom the advantages of avoiding valve prostheses are particularly attractive. A retrospective clinical study was undertaken to examine results of aortic valve-sparing operations in pediatric patients at a single institution. Clinical and echocardiographic data were obtained from chart review and outpatient follow-up examination. Between 1997 and 2004, 19 children underwent aortic valve-sparing root replacement. Mean age was 12.2 +/- 4.4 years, and mean weight was 51.5 +/- 22.9 kg. Median length of clinical follow-up was 58 months (range, 5 to 91). Fifteen of 19 patients (78.9%) had Marfan syndrome and 1 had aortic root dilation late after the arterial switch operation. Mean preoperative root diameter was 4.7 +/- 0.6 cm, with an average Z score of 7.7 +/- 1.9. Fourteen patients (73.7%) underwent root remodeling, whereas 5 (26.3%) had a reimplantation procedure. One patient required concomitant mitral valve repair. There was no operative mortality and only 1 reoperation for bleeding (5.3%). Median length of hospital stay was 5 days (range, 3 to 12). At latest follow-up, 3 of 19 patients (15.8%) have required late aortic valve replacement. Fifteen patients have no or mild aortic valve insufficiency, and 1 has moderate but stable valve regurgitation. No patient with a reimplantation procedure has had a reoperation or more than mild insufficiency, and no patient has suffered endocarditis or thromboembolism. Valve-sparing aortic root replacement is a viable alternative to root replacement with mechanical or biological prostheses in children, and can be accomplished with minimal morbidity and mortality. Reimplantation procedures appear to have more durable results than root remodeling techniques, and should be strongly considered for pediatric patients with aortic root enlargement secondary to connective tissue disorders and other forms of congenital heart disease.

Predictors of Left Ventricular Performance After Valve Replacement in Children and Adolescents with Chronic Aortic Regurgitation

Aortic valve replacement has been recommended in patients who have severe symptoms, in patients with extreme left ventricle (LV) dilatation (end diastolic dimension >4 SD above normal) or LV ejection fraction <50%. However, the occurrence of advanced symptoms or severe LV dilatation raises concern about irreversible LV dysfunction. This study sought to determine the influence of preoperative symptoms, LV size and function on mortality, and postoperative LV performance in children and adolescence after valve replacement for aortic regurgitation (AR). A total of 49 patients 18 years old or younger (mean, 13.9 +/- 3) who underwent valve replacement for chronic AR between 1991 and 2001 were followed up for 1-10 years (mean, 3.3 +/- 2.1). Baseline and postoperative characteristics were compared between 13 patients (group 1) with extreme LV dilatation and 34 patients (group 2) with a lesser degree of LV enlargement. Preoperative low ejection fraction (p < 0.008), extreme LV dilatation (p < 0.05), and LV end systolic dimension >4 SD above normal (p < 0.05) were predictors of persistent LV dysfunction. Extreme LV dilatation (p < 0.0003), LV end systolic dimension (p < 0.0007), and reduced LV ejection fraction (p < 0.01) predicted persistent LV dilatation. In the setting of chronic AR, preoperative symptoms, LV systolic function, and LV internal dimensions are the main predictors for persistent LV dysfunction and dilatation. Surgical correction should be performed before LV systolic dysfunction and/or extreme LV enlargement occurs.

Predictors of Left Ventricular Performance After Valve Replacement in Children and Adolescents with Chronic Aortic Regurgitation

Predictors of Left Ventricular Performance After Valve Replacement in Children and Adolescents with Chronic Aortic Regurgitation