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.

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.

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.

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 misdiagnosed as aortic sinus aneurysm in patients with ventricular septal defect. Analysis of the echocardiographic findings.

OBJECTIVES: To retrospectively analyzed the preoperative echocardiographic findings of 9 patients with doubly committed ventricular septal defect (VSD) associated with aortic valve prolapse and to summarize the reasons for misdiagnosis. Methods: This retrospective study was conducted in Yijishan Hospital, Wannan, Anhui, Chinabetween June 2005 and May 2015. Using transthoracic echocardiography (TTE), 92 patients were diagnosed with doubly committed VSD associated with rupture of an aortic sinus aneurysm. The operative findings proved to be in accordance with the echocardiographic findings in 83 patients. Nine patients were confirmed as showing doubly committed VSD associated with perforation of a prolapsed aortic valve. We primarily discussed the 9 patients who were misdiagnosed by TTE. Results: Aneurysm-like protrusions could be detected by 2-dimensional TTE in the right ventricular outflow tract (RVOT) in 9 patients. All of the aneurysm-like protrusions were confirmed by surgery as over-enlarged right coronary leaflets bulging into the RVOT. Conclusion:Occasionally, in patients with doubly committed VSD, aortic valve prolapse can be misdiagnosed as an aortic sinus aneurysm. To reduce the misdiagnosis rate and to provide a more precise preoperative diagnosis, multi-section, and multi-angle observation of these diseases should be performed.

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.

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.

[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.

Successful percutaneous management of partial avulsion of the native aortic valve complex complicating transcatheter aortic valve replacement.

This is the first reported case of partial aortic valve and annulus rupture during transcatheter aortic valve replacement, and its successful management through percutaneous means. It stresses the fact that even severe procedural complications can often be treated by a heart team endovascularly, without requiring sternotomy.

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.

Innocent left ventricular outflow tract membrane masquerading as vegetation.

Innocent left ventricular outflow tract (LVOT) membranes are a rare entity and found incidentally on echocardiography. The authors report a case of innocent LVOT membrane in a patient who was admitted with ischaemic stroke. Initial transthoracic echocardiography showed a possible valvular vegetation which was thought to be the cause of embolic stroke. Anticoagulation with coumadin and antibiotics were started. Subsequent Transesophageal echocardiography showed that it was more consistent with innocent LVOT membrane and not vegetation. Anticoagulation and antibiotics were discontinued, and on a follow-up over 5 years later, the membrane was stable in size and location without any complications.