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.

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.

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.

Transcatheter closure of ventricular septal defect in aortic valve prolapse and aortic regurgitation.

OBJECTIVE: To report intermediate follow-up result of transcatheter closure of ventricular septal defect (VSD) in presence of aortic valve prolapse (AVP) with or without aortic regurgitation (AR). METHOD: This is a retrospective review of 19 patients with VSD with AVP with AR who underwent transcatheter closure in between September 2011-July 2014. Mean age was 8 years (1-16 years, standard deviation [SD] 4.08 years) and mean weight was 26.03kg (9-81.5kg, SD 16.57kg). Among them 2 had subarterial VSD, 6 had subaortic VSD and 11 had perimembranous VSD. All of them had mild AVP and 13 of them had AR (trivial or mild). Median VSD size was 4.3mm (4-6mm). Transcatheter closure was done either by retrograde technique using the Amplatzer Duct Occluder-II in 17 patients or antegrade technique using the Duct Occluder-I in 2 cases. Mean follow-up period was 18 months (12-36 months). RESULT: Immediate major complications were encountered in 2 (10.5%) cases. Significant aggravation of device related AR was seen in one case & device embolised to right pulmonary artery in another case and both of them were managed surgically. During follow up, 1 child had significant additional VSD requiring device closure. One child developed moderate AR, requiring surgery. None of the other had shown any increase in severity of AR. CONCLUSION: Device closure of VSD in presence of mild AVP and mild AR appears to be safe. Longer follow-up is necessary to draw final conclusion.

Evaluation of Ventricular Septal Defect with Special Reference to the Spontaneous Closure Rate, Subaortic Ridge, and Aortic Valve Prolapse II.

The medical records of 2283 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. One thousand eight hundred and twenty-three patients had been followed 1 month to 26 years (median 4 years) by echocardiography. Most of 460 patients could not be followed due to transportation of the institution. VSD was perimembranous in 68.8% (1255), trabecular muscular in 21.7% (395), muscular outlet in 6% (109), muscular inlet in 2.6% (48), and doubly committed subarterial in 0.9% (16). Defect size was classified in 66.8% (1218) as small, in 15.7% (286) as moderate, and in 17.5% (319) as large. VSD closed spontaneously in 18.8% (343 of 1823 patients) by ages 40 days to 24.9 years (median, 1.8 years). One hundred fifty-seven of 1255 perimembranous defects (12.5%) and 167 of 395 trabecular muscular defects (42%) closed spontaneously (p < 0.001). Defect size became small in 306 (16.8%) of patients with VSD at a median of 2.5 years. Aneurysmal transformation was detected in 32.9% (600), left ventricular-to-right atrial shunt in 9.7% (176), subaortic ridge in 2.6% (48) of 1823 patients who were followed. In 381 (20.9%) of the 1823 patients, the VSD had been closed by a surgical or transcatheter technique. Surgery is required in one-fifth of patients with subaortic ridge or aortic valve prolapse. In conclusion, isolated VSDs are usually benign abnormalities that tend to shrink and close spontaneously.

Transcatheter Closure of Intracristal Ventricular Septal Defect With Mild Aortic Cusp Prolapse Using Zero Eccentricity Ventricular Septal Defect Occluder.

BACKGROUND: Transcatheter closure is a well-established therapy for patients with perimembranous ventricular septal defects (VSDs), but with limited experience in intracristal VSDs (IVSDs) with aortic cusp prolapse (ACP). METHODS AND RESULTS: From 2012 to 2014, we reviewed 38 patients with IVSDs complicated with mild ACP who underwent device closure, and, in light of the findings, assessed the effect of transcatheter intervention on preoperative mild ACP. The zero eccentric VSD occluder was chosen for closure (Shanghai Shape Memory Alloy Ltd, Shanghai, China). The mean defect was 4.8±1.6 mm (range, 2-8) as measured by transthoracic echocardiography and the mean device size was 10.1±2.1 mm (range, 4-14). Placement of the device was successful in 35 patients (92.1%). In the remaining 3 patients (7.9%), major complications occurred and they were converted to surgical intervention: severe aortic regurgitation (AR) in 2 patients and occluder dislodgement in 1 patient. During the follow-up (median 14.2 months; range, 3-24), no deaths, residual shunt, late-onset AR, heart block, or device failure occurred. CONCLUSIONS: The mid-term prognostic results of high success rate and low complications rate in this study are inspiring. Transcatheter closure of IVSD with mild ACP can be performed safely and effectively as an alternative to surgery in selected patients.

Demonstration of doubly committed juxta-arterial ventricular septal defect with aortic valve prolapse by cardiac computed tomography.

A 24-year-old man presented to our hospital with symptoms of dyspnea and palpitation for 2 weeks. Cardiac CT showed not only a leaflet coaptation defect in the aortic valve but also a small ventricular septal defect (VSD) immediately beneath the prolapsed right coronary cusp. A shunt flow in the direction of the right ventricular outflow tract though the defect indicated the doubly committed juxta-arterial type of VSD. A doubly committed juxta-arterial VSD of 3 mm was confirmed and repaired via pulmonary arteriotomy.

Laubry-Pezzi syndrome with aortic root dilatation treated with a bentall and De Bono procedure.

Laubry-Pezzi syndrome is a clinical entity in which prolapse of an aortic valve cusp into a subjacent ventricular septal defect (VSD) due to Venturi effect results in progressive aortic valve insufficiency. Aortic valve prolapse is found in over 5% of children with VSDs, most commonly in association with supracristal VSDs, and the risk of development of aortic regurgitation increases during childhood, peaking at 5 to 10 years of age. The VSD closure eliminates the low-pressure zone that is the cause of ongoing aortic valve cusp deformity and, if performed early, prevents the development of aortic regurgitation. However, the management of this rare pathology is still a matter of some debate with respect to indications, operative techniques, and timing. We report the case of a patient with Laubry-Pezzi syndrome, originally operated on at six years of age for VSD closure and plasty between the left and the right aortic cusps. However, in the follow-up the patient developed severe aortic regurgitation and aortic root dilatation. Eventually, a Bentall and De Bono procedure was performed.

Obesity is associated with higher long-term mortality after aortic valve replacement with small prosthesis.

OBJECTIVE: Although many studies have evaluated the impacts of obesity on various medical treatments, it is not known whether obesity is related to late mortality with implantation of small aortic prosthesis. This study evaluated the effect of obesity on late survival of patients after aortic valve replacement (AVR) with implantation of small aortic prosthesis (size ≤ 21 mm). METHODS: From January 1998 to December 2008, 536 patients in our institution who underwent primary AVR (307 patients with smaller prostheses) survived the 30 days after surgery. Patients were categorised as normal weight if body mass index (BMI) was ≤ 25 kg/m(2), as overweight if BMI 25-30 kg/m(2), and as obese if BMI ≥ 30 kg/m(2). Data were collected at the third-month (M), sixth-M, first-year (Y), third-Y, fifth-Y, and eighth-Y after operation. RESULTS: By multivariable analysis, obesity was a significant independent factor of late mortality (hazard ratio [HR]: 1.59; p=0.006). The obese and overweight groups of patients exhibited lower survival (p<0.001) and a higher proportion in NYHA class III/IV (p<0.01) compared with the normal group. Lower EOAI and higher left ventricular mass index were found in the obese and overweight groups, but we saw no significant variance in LVEF among the three groups. CONCLUSIONS: Obesity was associated with increased late mortality of patients after AVR with implantation of small aortic prosthesis. Being obese or and overweight may also affect the NYHA classification, even in the longer term.

Assessment and repair of aortic valve cusp prolapse: implications for valve-sparing procedures.

OBJECTIVES: Cusp prolapse causing aortic insufficiency is associated with unique echocardiographic, clinical, and surgical features. Recognition and appropriate surgical repair of this pathologic condition can not only treat affected patients but also improve results of aortic valve-sparing procedures, for which pre-existing or induced cusp prolapse is an important cause of failure. METHODS: Of 428 patients undergoing aortic valve repair, 195 (46%) were treated for cusp prolapse, and 111 (57%) of those had trileaflet aortic valve and make up this cohort. Cusp disease was the sole mechanism for aortic insufficiency (isolated group) in 50 patients whereas aortic dilatation was contributory in 61 (associated group). In total, 144 cusps were repaired in 111 patients. Preoperative echocardiograms, intraoperative findings, and clinical and echocardiographic outcomes were reviewed. RESULTS: On preoperative echocardiography, presence of an eccentric aortic insufficiency jet, regardless of severity, had 92% sensitivity and 96% specificity for the detection of single cusp prolapse. A transverse fibrous band was characteristically identified on the prolapsing cusp (sensitivity 57%; specificity 92%), correctly localizing a prolapsing cusp in all cases. Freedom from aortic valve reoperation at 8 years was 100% in the isolated group and 93% ± 5% in the associated group (p = 0.33). Freedom from recurrent aortic insufficiency (>2+) at 5 years was 90% ± 5% in the isolated and 85% ± 8% in the associated group (P = .54). The choice of surgical technique did not affect aortic insufficiency recurrence at follow-up (P = .6). CONCLUSIONS: Recognition and repair of isolated aortic cusp prolapse provides durable midterm outcome. An eccentric aortic insufficiency jet and a fibrous band can aid in the diagnosis and localization of cusp prolapse associated with ascending aortic disease and may help to improve results of aortic valve-sparing procedures.

Anatomic and functional aortic valvuloplasty for correction of aortic valve prolapse in ventricular septal defect with aortic insufficiency.

We describe a new procedure of aortic valvuloplasty for aortic valve prolapse in ventricular septal defect with aortic insufficiency syndrome. This technique allows an anatomic and functional aortic valve reconstruction that prevents late failure of aortic valve repair and reoperation. Midterm results demonstrate the feasibility and durability of this new procedure.