Morphological analysis of ventricular septal defect by echocardiography for prediction of aortic regurgitation in pediatric population.

Ventricular septal defects (VSD) are the most common congenital heart diseases in children. Among them, perimembranous VSD (pm-VSD) have a higher risk of complications, including aortic valve prolapse and aortic regurgitation (AR). The aim of our study was to assess echocardiographic criteria associated with AR during follow-up of pm-VSD. Forty children with restrictive pm-VSD, followed-up in our unit and who underwent a workable echocardiographic evaluation between 2015 and 2019 were included and retrospectively analyzed. The propensity score was used to match 15 patients with AR to 15 patients without AR. Median age was 2.2 year [1.4-5.7]. Median weight was 14 kg [9.9-20.3]. Aortic annulus z-score, Valsalva sinus z-score, sinotubular junction z-score, valve prolapse and commissure commitment were significantly different between the two groups (p = 0.047, p = 0.001, p = 0.010, p = 0.007, p < 0.001 respectively). Aortic root dilatation, aortic valve prolapse and commissure commitment to a perimembranous VSD are associated to aortic regurgitation.

Biomechanical evaluation of aortic regurgitation from cusp prolapse using an ex vivo 3D-printed commissure geometric alignment device.

BACKGROUND: Aortic regurgitation (AR) is one of the most common cardiac valvular diseases, and it is frequently caused by cusp prolapse. However, the precise relationship of commissure position and aortic cusp prolapse with AR is not fully understood. In this study, we developed a 3D-printed commissure geometric alignment device to investigate the effect of commissure height and inter-commissure angle on AR and aortic cusp prolapse. METHODS: Three porcine aortic valves were explanted from hearts obtained from a meat abattoir and were mounted in the commissure geometric alignment device. Nine commissure configurations were tested for each specimen, exploring independent and concurrent effects of commissure height and inter-commissure angle change on AR and aortic cusp prolapse. Each commissure configuration was tested in our 3D printed ex vivo left heart simulator. Hemodynamics data, echocardiography, and high-speed videography were obtained. RESULTS: AR due to aortic cusp prolapse was successfully generated using our commissure geometric alignment device. Mean aortic regurgitation fraction measured for the baseline, high commissure, low commissure, high commissure and wide inter-commissure angle, high commissure and narrow inter-commissure angle, low commissure and wide inter-commissure angle, low commissure and narrow inter-commissure angle, wide commissure, and narrow commissure configurations from all samples were 4.6 ± 1.4%, 9.7 ± 3.7%, 4.2 ± 0.5%, 11.7 ± 5.8%, 13.0 ± 8.5%, 4.8 ± 0.9%, 7.3 ± 1.7%, 5.1 ± 1.2%, and 7.1 ± 3.1%, respectively. CONCLUSIONS: AR was most prominent when commissure heights were changed from their native levels with concomitant reduced inter-commissure angle. Findings from this study provide important evidence demonstrating the relationship between commissure position and aortic cusp prolapse and may have a significant impact on patient outcomes after surgical repair of aortic valves.

Syndrome of ventricular septal defect and aortic regurgitation - A 22-year review of its management

Abstract Introduction: The presence of aortic regurgitation (AR) in the setting of ventricular septal defect (VSD) has always been a management challenge. Methods: This is a retrospective study looking at patients who underwent VSD closure with or without aortic valve intervention between January 1st, 1992 and December 31st, 2014 at the Institute Jantung Negara. This study looked at all cases of VSD and AR, where AR was classified as mild, moderate, and severe, the intervention done in each of this grade, and the durability of that intervention. The interventions were classified as no intervention (NI), aortic valve repair (AVr), and aortic valve replacement (AVR). Results: A total of 261 patients were recruited into this study. Based on the various grades of AR, 105 patients had intervention to their aortic valve during VSD closure. The rest 156 had NI. All patients were followed up for a mean time of 13.9±3.5 years. Overall freedom from reoperation at 15 years was 82.6% for AVr. Various factors were investigated to decide on intervening on the aortic valve during VSD closure. Among those that were statistically significant were the grade of AR, size of VSD, age at intervention, and number of cusp prolapse. Conclusion: We can conclude from our study that all moderate and severe AR with small VSD in older patients with more than one cusp prolapse will need intervention to their aortic valve during the closure of VSD.

Early Ventricular Septal Defect Closure Prevents the Progression of Aortic Regurgitation: A Long-Term Follow-Up Study.

According to current short-term evidence, ventricular septal defect (VSD) closure should be performed as early as possible after aortic regurgitation (AR) diagnosis in pediatric patients to prevent AR progression. However, long-term follow-up data are lacking. Therefore, our aim was to evaluate the long-term follow-up (≥ 10 years) of patients who underwent VSD closure as early as possible after AR diagnosis and to evaluate whether early VSD closure prevents progression of AR. This was a retrospective cohort study of 42 patients with VSD and AR who had undergone VSD closure at a median age of 2.9 years, with a median waiting period from AR diagnosis to VSD closure of 3.4 months and follow-up of a median 13.1 years (interquartile range 10.0-15.8 years). The preoperative degree of AR was trivial in 25 patients, mild in 15, and moderate in 2. Of the 33 patients followed up for ≥ 10 years, none required aortic valve replacement and there was no incidence of mortality. The degree of AR improved or did not change, except in 1 patient. The size of the VSD (odds ratio [OR] 0.85; 95% confidence interval [CI] 0.62-1.18; p = 0.33), time from diagnosis to surgery (OR 1.00; 95% CI 1.000-1.001; p = 0.657), and age at the time of surgery (OR 1.00; 95% CI 0.998-1.004; p = 0.452) were not predictive of persistent postoperative AR. Therefore, VSD closure performed as early as possible after AR diagnosis could successfully prevent AR progression in patients with less than moderate preoperative AR, eliminating the need for aortic valve replacement and valvuloplasty.

Safety and Efficacy of Transcatheter Occlusion of Perimembranous Ventricular Septal Defect with Aortic Valve Prolapse: A Six-Year Follow-Up Study.

BACKGROUND: With the rapid development of transcatheter techniques and instruments, transcatheter occlusion for patients with perimembranous ventricular septal defect (pVSD) and aortic valve prolapse (AVP) was constantly being tried, while the efficacy and safety of pVSD with AVP remain controversial. OBJECTIVE: The aim of this study was to evaluate long-term efficacy and safety of transcatheter occlusion of pVSD with AVP. METHODS: We retrospectively analyzed 164 children with pVSD and AVP who underwent transcatheter occlusion between January 2013 and November 2014. AVP was divided into 3 degrees according to right coronary leaflet morphology at end-diastole during aortic root angiography. Patient demographic and clinical data were collected. RESULTS: There were 97 males and 67 females (median age, 40.0 (30.0-62.7) months; average weight, 16.94 ± 9.02 kg). Mild (n = 63), moderate (n = 89), and severe (n = 12) AVP success rates were 93.7%, 89.9%, and 58.3%, respectively. Immediately after procedure, there was no new-onset aortic regurgitation (AR) above trivial degree, residual shunt above mild degree, or complications requiring medication or operation, except for 1 patient who developed transient complete atrioventricular block. During follow-up, 1 mild AVP patient aggravated from mild to moderate AR and 1 moderate AVP patient aggravated from trivial to moderate AR. The new-onset AR in mild, moderate, and severe AVP was 2%, 1.8%, and 20%, respectively. AR disappeared in 17 patients. Residual shunt occurred in 9 patients after procedure, 4 of which disappeared during the follow-up period. No serious complications occurred in any patient during follow-up. Five-year cardiovascular event-free survival rates for mild, moderate, and severe AVP were 89.6%, 94.5%, and 80.0%, respectively. CONCLUSION: Transcatheter occlusion of pVSD with mild and moderate AVP has a high success rate and few complications, which is safe and effective in long-term follow-up. Transcatheter occlusion of pVSD with severe AVP has low success rates and high AR incidence. Therefore, transcatheter occlusion of pVSD with AVP is recommended for mild to moderate, but not severe, AVP.

Syndrome of Ventricular Septal Defect and Aortic Regurgitation - A 22-Year Review of its Management.

INTRODUCTION: The presence of aortic regurgitation (AR) in the setting of ventricular septal defect (VSD) has always been a management challenge. METHODS: This is a retrospective study looking at patients who underwent VSD closure with or without aortic valve intervention between January 1st, 1992 and December 31st, 2014 at the Institute Jantung Negara. This study looked at all cases of VSD and AR, where AR was classified as mild, moderate, and severe, the intervention done in each of this grade, and the durability of that intervention. The interventions were classified as no intervention (NI), aortic valve repair (AVr), and aortic valve replacement (AVR). RESULTS: A total of 261 patients were recruited into this study. Based on the various grades of AR, 105 patients had intervention to their aortic valve during VSD closure. The rest 156 had NI. All patients were followed up for a mean time of 13.9±3.5 years. Overall freedom from reoperation at 15 years was 82.6% for AVr. Various factors were investigated to decide on intervening on the aortic valve during VSD closure. Among those that were statistically significant were the grade of AR, size of VSD, age at intervention, and number of cusp prolapse. CONCLUSION: We can conclude from our study that all moderate and severe AR with small VSD in older patients with more than one cusp prolapse will need intervention to their aortic valve during the closure of VSD.

Inter-operative determination of the aortic root and cusp geometry associated with the aortic regurgitation grade.

PURPOSES: To perform successful aortic valve plasty (AVP) and valve-sparing root replacement (VSRR), a sufficient understanding of the aortic root and cusp geometry is required. Several key parameters of the aortic root and cusp geometry were, therefore, measured intraoperatively. METHODS: Forty-nine patients (63.1 ± 16.6 y.o., 40 males) were measured during the surgeries. The patients were divided into 3 groups; including patients with no or mild aortic valve regurgitation (AR) (n = 13), moderate AR (n = 18), and severe AR (n = 18). RESULTS: There were no significant differences in the body surface area (1.72 ± 0.19 m2) among the 3 groups (p = 0.858). The effective height (EH) and geometric height (GH) of right coronary cusp were smaller than those of the others (EH: p = 0.068, GH: p < 0.01). The insertion line length (ILL) and free margin length (FML) of each leaflet tended to be significantly longer according to the AR grade(ILL: p < 0.01, FML: p < 0.01). The FML/AVJ ratios were significantly different (p < 0.01). The FML/ILL ratios were constant with the ratio of 0.88 ± 0.1(p = 0.624). CONCLUSIONS: Although the ILL and FML tended to increase with the AR grade, the FML/ILL ratio remained constant. For successful AVP and VSRR, adequately maintaining the FML/ILL ratio is necessary to prevent remarkable cusp prolapse while also preserving its favorable cusp motion.

A Novel Aortic Regurgitation Model from Cusp Prolapse with Hemodynamic Validation Using an Ex Vivo Left Heart Simulator.

Although ex vivo simulation is a valuable tool for surgical optimization, a disease model that mimics human aortic regurgitation (AR) from cusp prolapse is needed to accurately examine valve biomechanics. To simulate AR, four porcine aortic valves were explanted, and the commissure between the two largest leaflets was detached and re-implanted 5 mm lower to induce cusp prolapse. Four additional valves were tested in their native state as controls. All valves were tested in a heart simulator while hemodynamics, high-speed videography, and echocardiography data were collected. Our AR model successfully reproduced cusp prolapse with significant increase in regurgitant volume compared with that of the controls (23.2 ± 8.9 versus 2.8 ± 1.6 ml, p = 0.017). Hemodynamics data confirmed the simulation of physiologic disease conditions. Echocardiography and color flow mapping demonstrated the presence of mild to moderate eccentric regurgitation in our AR model. This novel AR model has enormous potential in the evaluation of valve biomechanics and surgical repair techniques. Graphical Abstract.

[Aortic Regurgitation and Stenosis Associated with Ventricular Septal Defect in the Elderly;Report of a Case].

A 63-year-old man had ventricular septal defect (VSD) and had been followed up without heart failure. Recently, he had palpitation caused by atrial fibrillation and the echocardiography revealed moderate aortic valve regurgitation and stenosis with right coronary cusp prolapse due to subpulmonary ventricular septal defect. He underwent patch closure of VSD, aortic valve replacement with mechanical valve, and maze procedure. In recent years, advanced case like this patient is rare because most of patients with subpulmonary VSD and right coronary cusp prolapse are operated in childhood.

Prognostic factors associated with postoperative adverse outcomes in patients with aortic valve prolapse.

BACKGROUND AND AIM: Aortic valve (AV) cusp prolapse and subsequent aortic insufficiency (AI) are 2 of factors leading to left ventricular (LV) enlargement and decreased LV function. Aortic valve replacement (AVR) has been the standard surgical procedure for AI. However, few data is available on the prognosis of these patients undergoing AVR procedure, especially in Chinese population. The study aims to evaluate the potential risk factors affecting the mid-term adverse outcomes after AVR. METHODS: One hundred thirty-four patients (mean age: 46.7 years old) with AV cusp prolapse and severe AI who all received surgical aortic valve replacement were recruited in our hospital between January 1, 2009 and December 30, 2017. The clinical characteristics, echocardiography parameters, as well as operative parameters were obtained. The primary endpoint included death, heart failure development, and reoperation. RESULTS: There were 14 adverse events altogether with the primary endpoint during a median follow-up of 8.6 (6-10) months. The multivariable Cox regression analysis revealed that baseline LVEDD (hazard rate, HR = 1.08, 95% CI: 1.01-1.15, P = .021), moderate pulmonary hypertension (HR = 9.36, 95% CI: 1.81-48.28, P = .008), and the time of assisted mechanical ventilation (HR = 1.01, 95% CI: 1.00-1.01, P = .022) were independently associated with the primary endpoint. Kaplan-Meier survival curve showed a significant worse survival free of the endpoint for patients with LVEDD≥70 mm, indexed LVEDD≥37.3 mm/m (the mean in this study), indexed LVESD≥25 mm/m or baseline LVEF <50% (all P<.05). CONCLUSION: Baseline enlarged LV dimensions, low LV function, moderate pulmonary hypertension, and prolonged assisted mechanical ventilation may predict the poor mid-term postoperative outcomes for AV cusp prolapse patients undergoing AVR procedure.

Redo Valve-Sparing Root Replacement for Delayed Cusp Derangement From Ventricular Septal Defect.

A 28-year-old man with ventricular septal defect, double-chambered right ventricle with associated right ventricular outflow tract obstruction, and anomalous right coronary artery underwent resection of the double-chambered right ventricle, transaortic ventricular septal defect repair, and unroofing of anomalous right coronary artery. Two years later, he returned with delayed presentation of ventricular septal defect flow funnel-related aortic cusp prolapse and symptomatic severe aortic regurgitation. He underwent reoperative valve-sparing aortic root replacement and aortic cusp repair with an excellent outcome.