Editorial: Partial left ventriculectomy for dilated cardiomyopathy in children

AbstractJ Thorac Cardiovasc Surg 1999;117:918–91

3D Ultrasound-Guided Motion Compensation System for Beating Heart Mitral Valve Repair

Beating heart intracardiac procedures promise significant benefits for patients, however, the fast motion of the heart poses serious challenges to surgeons. We present a new 3D ultrasound-guided motion (3DUS) compensation system that synchronizes instrument motion with the heart. The system utilizes the fact that the motion of some intracardiac structures, including the mitral valve annulus, is largely constrained to translation along one axis. This allows the development of a real-time 3DUS tissue tracker which we integrate with a 1 degree-of-freedom actuated surgical instrument, real-time 3DUS instrument tracker, and predictive filter to devise a system with synchronization accuracy of 1.8 mm RMSE. User studies involving the deployment of surgical anchors in a simulated mitral annuloplasty procedure demonstrate that the system increases success rates by over 100%. Furthermore, it enables more careful anchor deployment by reducing forces to the tissue by 50% while allowing instruments to remain in contact with the tissue for longer periods.Engineering and Applied Science

Late left ventricular dysfunction after anatomic repair of congenitally corrected transposition of the great arteries

ObjectiveEarly results for anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) are excellent. However, the development of left ventricular dysfunction late after repair remains a concern. In this study we sought to determine factors leading to late left ventricular dysfunction and the impact of cardiac resynchronization as a primary and secondary (upgrade) mode of pacing.MethodsFrom 1992 to 2012, 106 patients (median age at surgery, 1.2 years; range, 2 months to 43 years) with ccTGA had anatomic repair. A retrospective review of preoperative variables, surgical procedures, and postoperative outcomes was performed.ResultsIn-hospital deaths occurred in 5.7% (n = 6), and there were 3 postdischarge deaths during a mean follow-up period of 5.2 years (range, 7 days to 18.2 years). Twelve patients (12%) developed moderate or severe left ventricular dysfunction. Thirty-eight patients (38%) were being paced at latest follow-up evaluation. Seventeen patients had resynchronization therapy, 9 as an upgrade from a prior dual-chamber system (8.5%) and 8 as a primary pacemaker (7.5%). Factors associated with left ventricular dysfunction were age at repair older than 10 years, weight greater than 20 kg, pacemaker implantation, and severe neo-aortic regurgitation. Eight of 9 patients undergoing secondary cardiac resynchronization therapy (upgrade) improved left ventricular function. None of the 8 patients undergoing primary resynchronization developed left ventricular dysfunction.ConclusionsLate left ventricular dysfunction after anatomic repair of ccTGA is not uncommon, occurring most often in older patients and in those requiring pacing. Early anatomic repair and cardiac resynchronization therapy in patients requiring a pacemaker could preclude the development of left ventricular dysfunction

Valve-sparing aortic root replacement and remodeling with complex aortic valve reconstruction in children and young adults with moderate or severe aortic regurgitation

ObjectivesThe durability of valve-sparing aortic root procedures with aortic regurgitation due to leaflet disease is questioned. Here, we review our experience in combined aortic root and valve reconstruction in children and young adults.MethodsAll valve-sparing aortic root procedures from 2000 to 2012 were reviewed, and patients with aortic valve repair beyond resuspension were included. Root procedures were classified as replacement with reimplantation, root remodeling, or aortic annular and sinotubular junction stabilization. The primary end point was structural valve deterioration, a composite of aortic valve reoperation and/or moderate or greater regurgitation at follow-up.ResultsThirty-four patients were included during the study period. The surgery consisted of reimplantation in 13 patients, remodeling in 16 patients, and annular and sinotubular junction stabilization in 5 patients. Valve repair consisted of leaflet procedures in 26 patients and subannular reduction in 15 patients. During a median follow-up of 4.2 months (range, 2 weeks-8 years), there were 5 reoperations for aortic valve replacement due to aortic regurgitation, and 2 patients presented with moderate or greater regurgitation. Freedom from structural valve deterioration was 70.1% ± 10.3% at 1 year and remained stable thereafter, although it was significantly worse in the reimplantation group (P = .039). A more severe degree of preoperative aortic regurgitation (P = .001) and smaller graft to aortic annulus ratio (P = .003) were predictors of structural valve deterioration.ConclusionsValve-sparing root and valve reconstruction can be done with low operative risk and allows valve preservation in most patients. These data should question the assumption that reimplantation is superior when associated with complex valve reconstruction

Mild postoperative acute kidney injury and outcomes after surgery for congenital heart disease

ObjectiveThe effect of mild acute kidney injury (AKI) on outcomes after heart surgery in children is unclear. We sought to characterize the epidemiology of mild AKI associated with surgery for congenital heart disease (CHS-AKI) in children.MethodsWe conducted a single-center, retrospective cohort study of 693 patients (aged 6 days-18 years) who underwent heart surgery in 2009. The prevalence of AKI within 72 hours of surgery was determined using the 3-stage Acute Kidney Injury Network criteria. Factors associated with both hospital length of stay and AKI were used in a proportional hazards model to test the association of stage 1 AKI with hospital length of stay.ResultsThe median age of the patients was 11.5 months (interquartile range, 3-54 months). Eighteen percent of the cohort had single ventricle heart disease and 54% underwent RACHS-1 category 3 or higher surgery. The prevalence of stages 1, 2, and 3 AKI in this cohort was 11% (n = 77), 3% (n = 19), and 1% (n = 8), respectively. Factors independently associated with AKI were prematurity, single ventricle physiology, peak postoperative lactic acid concentration, cardiopulmonary bypass time, and a history of heart surgery. Stage 2 or greater CHS-AKI was associated with hospital length of stay (adjusted hazard ratio [AHR], 0.53; 95% confidence interval [CI], 0.33-0.87; P = .01), but stage 1 was not (AHR, 0.85; 95% CI, 0.66-1.10; P = .22).ConclusionsAKI occurs after surgery for congenital heart disease but may be less common than previously reported. Although moderate to severe CHS-AKI is independently associated with prolonged recovery after heart surgery, mild disease does not appear to be

Mitral valve replacement in infants and children 5 years of age or younger: Evolution in practice and outcome over three decades with a focus on supra-annular prosthesis implantation

ObjectiveSuccessful mitral valve replacement in young children is limited by the lack of small prosthetic valves. Supra-annular prosthesis implantation can facilitate mitral valve replacement with a larger prosthesis in children with a small annulus, but little is known about its effect on the outcomes of mitral valve replacement in young children.MethodsOne hundred eighteen children underwent mitral valve replacement at 5 years of age or younger from 1976–2006. Mitral valve replacement was supra-annular in 37 (32%) patients.ResultsSurvival was 74% ± 4% at 1 year and 56% ± 5% at 10 years but improved over time (10-year survival of 83% ± 7% from 1994–2006). Factors associated with worse survival included earlier mitral valve replacement date, age less than 1 year, complete atrioventricular canal, and additional procedures at mitral valve replacement, but not supra-annular mitral valve replacement. As survival improved during our more recent experience, the risks of supra-annular mitral valve replacement became apparent; survival was worse among patients with a supra-annular prosthesis after 1991. A pacemaker was placed in 18 (15%) patients within 1 month of mitral valve replacement and was less likely in patients who had undergone supra-annular mitral valve replacement. Among early survivors, freedom from redo mitral valve replacement was 72% ± 5% at 5 years and 45% ± 7% at 10 years. Twenty-one patients with a supra-annular prosthesis underwent redo mitral valve replacement. The second prosthesis was annular in 15 of these patients and upsized in all but 1, but 5 required pacemaker placement for heart block.ConclusionsSupra-annular mitral valve replacement was associated with worse survival than annular mitral valve replacement in our recent experience. Patients with supra-annular mitral valve replacement were less likely to have operative complete heart block but remained at risk when the prosthesis was subsequently replaced

Mechanisms of tricuspid regurgitation in patients with hypoplastic left heart syndrome undergoing tricuspid valvuloplasty

ObjectivesTricuspid regurgitation (TR) remains a risk factor for morbidity and mortality through staged palliation in patients with hypoplastic left heart syndrome (HLHS). Reports on the mechanisms associated with TR in patients with HLHS are limited. Thus, we sought to describe our experience with tricuspid valve (TV) repair in these patients, focusing on the mechanisms of TR and corresponding surgical techniques.MethodsWe performed a retrospective single-center review (January 2000 to December 2012) of patients with HLHS undergoing TV repair and completing Fontan circulation. We evaluated the pre- and postoperative echocardiograms, intraoperative findings, and surgical techniques used.ResultsA total of 53 TV repairs were performed in 35 patients with HLHS completing staged palliation. TV repairs were performed at stage II in 15, between stage II and III in 4, at stage III in 27, and after stage III in 7. The surgical techniques for valvuloplasty included annuloplasty (38%), anteroseptal (AS) commissuroplasty (66%), anterior papillary muscle repositioning (11%), multiple commissuroplasties (9%), septal-posterior commissuroplasty (9%), and fenestration closure (4%). The predominant jet of TR emanated along the AS commissure in 68% of the cases. All patients survived the procedure and were discharged. Preoperative echocardiography showed a dilated TV annulus on the lateral dimension, anteroposterior dimension, and area that was significantly reduced after TV repair (P < .0001). The preoperative mean TR, as assessed by lateral (P = .002) and anteroposterior (P = .005) vena contracta, was also significantly reduced after TV repair. TV repair did not significantly affect right ventricular systolic function immediately after surgery (P = .17) or at the most recent follow-up visit (P = .52). Patients with anterior leaflet prolapse were at increased risk of worse outcomes, including moderate or greater right ventricular dysfunction (P = .02). Patients requiring reoperation for TV repair were younger (6.3 vs 28.1 months, P < .0001) at the initial operation. One patient died of heart failure. Freedom from TV replacement and transplant-free survival were both 97% at the most recent follow-up point.ConclusionsTR in patients with HLHS commonly emanates from the AS commissure. The associated mechanisms are often annular dilatation and anterior leaflet prolapse. Preoperative anterior leaflet prolapse was associated with worse outcomes. Annuloplasty, closure of the AS commissure, and repositioning of the anterior papillary muscle are effective in addressing TR in the short- and mid-term in this challenging population

Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart

ObjectiveBorderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation—endocardial fibroelastosis resection with mitral or aortic valvuloplasty—on left ventricular function and clinical outcomes.MethodsAll patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test.ResultsBetween 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1–38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively.ConclusionIn patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group

A novel cardioport for beating-heart, image-guided intracardiac surgery

Objective Intracardiac beating-heart procedures require the introduction and exchange of complex instruments and devices. To prevent potential complications such as air embolism and bleeding, a universal cardioport was designed and tested. Methods The design consists of a port body and a series of interchangeable sleeves. The port uses a fluid purging system to remove air from the instrument before insertion into the heart, and a valve system minimizes blood loss during instrument changes. Results The cardioport was tested ex vivo and in vivo in pigs (n = 5). Beating-heart procedures, such as septal defect closure and mitral valve repair, were modeled. Ex vivo trials (n = 150) were performed, and no air emboli were introduced using the port. In comparison, air emboli were detected in 40% to 85% of the cases without the use of the port-based purging system. Port operation revealed excellent ergonomics and minimal blood loss. Conclusions A novel cardioport system designed to prevent air entry and blood loss from transcardiac instrument introduction was shown to be an enabling platform for intracardiac beating-heart surgery. The port system improves safety and facilitates further development of complex instruments and devices for transcardiac beating-heart surgery.Center for Integration of Medicine and Innovative Technology (Award 07-026)National Institutes of Health (U.S.) (National Heart, Lung, and Blood Institute Award 5R01HL073647)Massachusetts Technology Transfer Cente