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.

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.

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.

[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.

Echocardiographic findings of cardiovascular involvement in Behçet's disease and post-operative complications after cardiac surgery.

OBJECTIVES: The aim of this study was to analyse the spectrum of echocardiographic findings in patients with cardiovascular involvement in Behçet's disease (BD) and followed up the post-operative complications. METHODS: We enrolled 26 BD patients who underwent first cardiac surgery in Anzhen Hospital, Beijing, China. Medical records and echocardiographic findings were retrospectively analysed. RESULTS: The 26 patients consisted of 4 women and 22 men. 22 (84.6%) of the patients were diagnosed with moderate/severe aortic regurgitation (AR). Some distinctive echocardiographic features with AR were observed, including prolapse of aortic cusps, vegetation-like mobile lesions, an echo-free space mimicking aortic root abscess and aortic aneurysm formation. 3 (11.5%) of the patients were diagnosed with isolated descending aortic aneurysm. 1(3.8%) of the patients was diagnosed with pulmonary artery aneurysm. BD was preoperatively diagnosed by clinicians in 20 patients. And 6 patients were diagnosed post-operatively by clinicians. In a total of 26 patients, post-operative complications occurred in 8 (30.7%) patients. The complications occurred in the 6 patients diagnosed post-operatively and 2 patients diagnosed pre-operatively. The post-operative complications of these patientsincluded aortic paravalvular leakage, coronary-graft anastmotic leakage and mitral paravalvular leakage. CONCLUSIONS: The most common echocardiographic feature of cardiovascular involvement in BD is severe aortic regurgitation with prolapse of aortic cusps, vegetation-like mobile lesions, an echo-free space mimicking aortic root abscess or aortic aneurysm formation. Accurate preoperative diagnosis of BD is beneficial to the choice of immunosuppressive therapy before and after surgery, which is likely to reduce postoperative complications especially for patients with severe lesions.

Aortic Valve Prolapse and Aortic Regurgitation During Long-Term Follow Up in Children with Ventricular Septal Defect.

BACKGROUND: Aortic valve prolapse (AVP) and aortic regurgitation (AR) can develop in a subset of patients with ventricular septal defect (VSD). The incidence and progression of AVP and AR with VSD at long-term follow up was evaluated. METHODS: The records of 2,275 patients with isolated VSD who had been diagnosed using echocardiography at the present authors' institution between 1988 and 2014 were reviewed. RESULTS: AVP was detected in 178 patients (7.8%), using echocardiography. Of 178 patients with AVP, AR was detected in 124 (AR incidence 5.4%). A total of 142 patients was followed medically during a median of 10 years after AVP had appeared. Initially, no AR was noted in 66 of these patients, trivial AR in 41, mild AR in 30, moderate in three, and severe in two. Trivial AR developed in 20 and mild in eight of 66 patients who had no AR. In 18 of 61 patients (29.5%), trivial AR progressed to mild during a median of 3.6 years, and in five of 18 patients (27.7%) mild AR progressed to moderate during a median of 2.3 years. Postoperatively, AR improved in 17 patients, remained unchanged in 12, and worsened in four at between two months and 16.5 years of follow up (median 6.6 years). CONCLUSIONS: Frequent (six-month) echocardiographic evaluation in patients with perimembraneous or muscular outlet VSD after AVP and AR development may be useful. In addition, surgical intervention in patients with perimembraneous or muscular outlet VSD, AVP and mild AR may prevent the worsening of AR.

Measurement of Aortic Valve Coaptation and Effective Height Using Echocardiography in Patients with Ventricular Septal Defects and Aortic Valve Prolapse.

Decreased coaptation height in adults has been identified as a marker of early valve failure. We evaluated aortic valve coaptation and effective height in healthy children and in children with a ventricular septal defect (VSD) with aortic cusp prolapse (ACP), using echocardiography. We included 45 subjects with VSD with ACP, 27 did not develop aortic regurgitation (AR) by ACP and 18 developed AR by ACP, and 83 healthy children as controls. Aortic root anatomy was estimated using the parasternal long-axis view. We measured the diameter of aortic valve (AV), coaptation height (CH), and effective height (EH) of the aortic valve. We defined the ACH (CH/AV ratio) and AEH (EH/AV ratio) indices as follows: [Formula: see text]. There were significant differences in ACH and AEH between the groups (control vs VSD with ACP vs VSD with ACP and AR, median ACH [%], 35.1 vs 32.0 vs 22.1; median AEH [%], 52.0 vs 48.0 vs 34.4, respectively; P < 0.01]). Intra-cardiac repair (ICR) was performed in 15 cases. Significant increases were observed in ACH and AEH before and after ICR (median ACH [%], before: 27.0, after: 32.7, P < 0.05; median AEH (%), before 38.5, after 45.8, P < 0.05). Measurement of ACH and AEH may allow direct and non-invasive assessment of the severity of VSD with ACP, which could aid clinicians in determining the need and timing for surgical intervention.

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.

[Quadricuspid Aortic Valve: Report of a Case].

A 69-year-old woman with general fatigue was admitted to our hospital for further examination of mediastinal lymph node swelling. Mediastinal lymph node sampling was planned. Preoperative transthoracic echocardiography revealed severe aortic regurgitation, and aortic valve replacement combined with mediastinal lymph node sampling was performed. Following lymph node sampling, the ascending aorta was opened, and the aortic valve was found to be quadricuspid. An accessory cusp was existed between the left coronary cusp and the non coronary cusp. Aortic valve replacement was successfully performed. A quadricuspid aortic valve is a very rare anomaly.

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.

Hemodynamic Performance and Durability of Mosaic Bioprostheses for Aortic Valve Replacement, up to 13 Years.

BACKGROUND: This study evaluated the mid to long-term durability and hemodynamics of the small-size Mosaic bioprosthesis, a third-generation stented porcine bioprosthesis, for aortic valve replacement (AVR). METHODS AND RESULTS: From 2000 to 2012, 207 patients (117 women; age, 74±8 years; body surface area, 1.48±0.25 m(2)) underwent AVR with a Mosaic bioprosthesis. The mean follow-up period was 3.5±2.7 years (maximum, 12.4 years) and the follow-up rate was 93.7%. A 19-, 21-, 23-, 25-, and 27-mm prosthesis was used in 103, 53, 35, 13, and 3 patients, respectively. The measured effective orifice area was 1.17±0.25, 1.29±0.19, 1.39±0.24, and 1.69 cm(2)for the 19-25 mm prostheses, and the mean transvalvular pressure gradient was 19.4±6.0, 18.5±5.8, 16.5±7.3, and 13.2±2.9 mmHg, respectively. The left ventricular mass regression was significant (P<0.05) with rates of 74.6±18.8%, 75.5±30.2%, 68.1±30.5%, 55.9±12.9%, and 49.2%, respectively. The 30-day mortality rate was 1.9% and the 5- and 10-year actuarial survival rates were 86.0% and 73.7%, respectively. Valve-related comorbidities occurred in 3 patients (structural valve deterioration [SVD] in 1 after 7.2 years, and prosthetic valve endocarditis in 2). Freedom from SVD at 10-year was 96.7%. CONCLUSIONS: The mid to long-term performance of the small Mosaic bioprosthesis was satisfactory, with excellent hemodynamics and few valve-related adverse events.

Long-Term Results after Aortic Valve-Sparing-Reimplantation Operation (David) in Bicuspid Aortic Valve.

BACKGROUND AND AIM OF THE STUDY: The aortic valve-sparing reimplantation operation (David) is increasingly used in patients with aortic root aneurysm and intact cusps. David's procedure is also feasible in patients with bicuspid aortic valve (BA), though few long-term data are available. METHODS: An analysis was conducted of the long-term echocardiographic data from patients with BAV who had undergone David's procedure at early and long-term follow up (FU) examinations. RESULTS: Between 1994 and 2010, a total of 30 patients with BAV underwent David's procedure at the authors'institution. There were no in-hospital or late deaths, and the mean long-term FU was 6.64 ± 3.54 years (range: 3.2-20.1 years). The mean aortic regurgitation (AR) grade was increased from 0.26 ± 0.37 at early FU to 0.70 ± 0.80 at long-term FU (p = 0.013). Four patients (13%) had to be reoperated after 10.00 ± 4.74 years (range: 5.49-17.06 years) due to aortic stenosis (n = 1) and aortic insufficiency (n = 3); the latter three patients had a significant prolapse of both the fused and the non-coronary cusp of reconstruction. CONCLUSION: The mean AR grade was increased significantly but was ≤ I-II in 59% of patients and ≤ II in 93% of patients after a mean FU of 6.6 years. Patients with prolapsing non-coronary leaflet at reconstruction seemed vulnerable to recurrent AR. Among patients, survival was excellent, and the reoperation rate and hemodynamics acceptable. Long-term follow up data are necessary to further refine the surgical techniques employed and to improve the results achieved.

Reliability and identification of aortic valve prolapse in the horse.

BACKGROUND: The objectives were to determine and assess the reliability of criteria for identification of aortic valve prolapse (AVP) using echocardiography in the horse. RESULTS: Opinion of equine cardiologists indicated that a long-axis view of the aortic valve (AoV) was most commonly used for identification of AVP (46%; n=13). There was consensus that AVP could be mimicked by ultrasound probe malignment. This was confirmed in 7 healthy horses, where the appearance of AVP could be induced by malalignment. In a study of a further 8 healthy horses (5 with AVP) examined daily for 5 days, by two echocardiographers standardized imaging guidelines gave good to excellent agreement for the assessment of AVP (kappa>0.80) and good agreement between days and observers (kappa >0.6). The technique allowed for assessment of the degree of prolapse and measurement of the prolapse distance that provided excellent agreement between echocardiographers, days and observers (kappa/ICC>0.8). Assessments made using real-time zoomed images provided similar measurements to the standard views (ICC=0.9), with agreement for the identification of AVP (kappa>0.8). Short axis views of the AoV were used for identification of AVP by fewer respondents (23%), however provided less agreement for the identification of AVP (kappa>0.6) and only adequate agreement with observations made in long axis (kappa>0.5), with AVP being identified more often in short axis (92%) compared to long axis (76%). Orthogonal views were used by 31% of respondents to identify the presence of AVP, and 85% to identify cusp. Its identification on both views on 4 days was used to categorise horses as having AVP, providing a positive predictive value of 79% and negative predictive value of 18%. Only the non-coronary cusp (NCC) of the AoV was observed to prolapse in these studies. Prolapse of the NCC was confirmed during the optimisation study using four-dimensional echocardiography, which concurred with the findings of two-dimensional echocardiography. CONCLUSIONS: This study has demonstrated reliable diagnostic criteria for the identification and assessment of AVP that can be used for longitudinal research studies to better define the prevalence and natural history of this condition.

Valvular heart disease in patients with osteogenesis imperfecta.

Osteogenesis imperfecta (OI) or "brittle bone disease" is a rare connective tissue hereditary disorder. The most common clinical presentation of OI is bone fractures. OI also involves extraskeletal structures; however, cardiovascular manifestations are rare. In this report, we describe the cardiovascular anomalies of patients with OI who underwent valve surgery and review the literature on this subject.

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.

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.