Assessment of Asymptomatic Severe Aortic Regurgitation by Doppler-Derived Echo Indices: Comparison with Magnetic Resonance Quantification

Reliable quantification of aortic regurgitation (AR) severity is essential for clinical management. We aimed to compare quantitative and indirect echo-Doppler indices to quantitative cardiac magnetic resonance (CMR) parameters in asymptomatic chronic severe AR. Methods and Results: We evaluated 104 consecutive patients using echocardiography and CMR. A comprehensive 2D, 3D, and Doppler echocardiography was performed. The CMR was used to quantify regurgitation fraction (RF) and volume (RV) using the phase-contrast velocity mapping technique. Concordant grading of AR severity with both techniques was observed in 77 (74%) patients. Correlation between RV and RF as assessed by echocardiography and CMR was relatively good (rs = 0.50 for RV, rs = 0.40 for RF, p < 0.0001). The best correlation between indirect echo-Doppler and CMR parameters was found for diastolic flow reversal (DFR) velocity in descending aorta (rs = 0.62 for RV, rs = 0.50 for RF, p < 0.0001) and 3D vena contracta area (VCA) (rs = 0.48 for RV, rs = 0.38 for RF, p < 0.0001). Using receiver operating characteristic analysis, the largest area under curve (AUC) to predict severe AR by CMR RV was observed for DFR velocity (AUC = 0.79). DFR velocity of 19.5 cm/s provided 78% sensitivity and 80% specificity. The AUC for 3D VCA to predict severe AR by CMR RV was 0.73, with optimal cut-off of 26 mm2 (sensitivity 80% and specificity 66%). Conclusions: Out of the indirect echo-Doppler indices of AR severity, DFR velocity in descending aorta and 3D vena contracta area showed the best correlation with CMR-derived RV and RF in patients with chronic severe AR.

A heart in a heart: a case report of spontaneous flail of bicuspid aortic valve

Background Aortic regurgitation is attributed to congenital and acquired abnormalities of the aortic valve or aortic valve supporting structures. The most common cause of aortic regurgitation is atherosclerotic degeneration of the valve, especially in the presence of a bicuspid aortic valve. Case summary A 25-year-old Persian man with no past medical history, developed dyspnea since 1 week before his first visit to the physician. He was an active person up to this time, and had no history of trauma or chest pain. Transthoracic echocardiography showed severe aortic regurgitation in the context of flail bicuspid aortic valve, with no evidence of endocarditis. Laboratory tests including blood cultures were negative for infection. The patient underwent aortic valve replacement and the diagnosis was confirmed at time of surgery. Conclusion This case represents noninfective and nontraumatic spontaneous flail of bicuspid aortic valve.

Can left ventricular function recover following Bentall procedure in a patient with concomitant noncompacted left ventricular myocardium?

We report a rare case of successful left ventricular restoration of left ventricular hypertrabeculation/noncompaction (LVHT) after Bentall procedure in a patient with severe aortic regurgitation (AR) and aortic root aneurysm. At 1-year follow-up, he remained well with echocardiography showing the improved contractility of the noncompacted left ventricle. This case report emphasize that timely surgical correction of severe AR may also lead to improvement of systolic dysfunction along with concomitant LVHT.

Abstract 17189: Radiation-induced Accelerated Multi-valve Disease In A Patient With Subaortic Membrane

A 69-year-old woman with history of right breast cancer status post lumpectomy and radiation (4.2 cGy) 18 months prior, presented with progressive NYHA class III dyspnea. TTE obtained prior to her cancer diagnosis was notable for normal LV size and wall thickness, a subaortic membrane with systolic mean Doppler gradient of 21 mmHg and a mildly thickened mitral valve without significant stenosis or regurgitation. On presentation, TTE was notable for severely increased concentric LV wall thickness (LV mass index 140 g/m 2 ), preserved ejection fraction, increased RV wall thickness, subaortic stenosis with systolic mean Doppler gradient 35 mmHg, moderate-severe aortic regurgitation, thickened mitral valve with posterior leaflet tethering and anterior leaflet override causing severe mitral regurgitation and moderate mitral stenosis (valve area 1.7 cm 2 by pressure half-time). She underwent a surgical intervention with septal myectomy, subaortic membrane resection, aortic root reconstruction, aortic and mitral valve replacement with bioprostheses. She did well post-operatively and had complete resolution of symptoms following cardiac rehabilitation. Radiation-induced valvular heart disease is linked to the total dose of radiation, use of sequential chemotherapy and time since irradiation, with a typical latency of 10-20 years. However, there is paucity of data regarding the influence of these variables in patients with pre-existing valve disease. This case illustrates dramatic acceleration of underlying aortic and mitral valve pathology in a patient with a subaortic membrane, 18 months after radiation. We also noted development of significant concentric LV hypertrophy that was disproportionate to the degree of obstruction across the sub-aortic membrane. Potential candidates for radiation therapy must be carefully screened for pre-existing valve disease, which should warrant increased vigilance and early screening for progressive valve disease.

Aortic Valve Replacement for Quadricuspid Aortic Valve Associated With Right Coronary Ostium Anomaly: a Case Report

Background: Quadricuspid aortic valve is a rare congenital heart disease that may be associated with coronary ostium anomalies. Care should be taken to avoid occluding or compressing the coronary ostium while performing aortic valve replacement.Case presentation: Herein, we report a case of a 59-year-old woman who underwent aortic valve replacement for a quadricuspid aortic valve with severe aortic regurgitation. Intraoperatively, the aortic valve had four cusps of almost equal size and the right coronary artery arose adjacent to one of the commissures. The annular stitches were placed in a non-everting mattress fashion with pledgets on the ventricular side, and stitches near the right coronary ostium were transitioned to the subannular ventricular myocardium to maintain the distance from the ostium. Further, we selected a small prosthesis because oversized prosthetic valve could potentially compress the right coronary ostium.Conclusions: While performing aortic valve replacement for a quadricuspid aortic valve associated with a right coronary ostium anomaly, careful selection of the size of the prosthesis and modification of the annular stitches are essential to prevent obstruction of the coronary ostium.

Transcatheter Aortic Valve Dislocation into the Left Ventricle Caused by the Watermelon Effect

Transcatheter aortic valve implantation (TAVI) is a well-established method for treating severe aortic stenosis. Prosthetic valve dislocation immediately after deployment is a rare and feared complication. We present a case of a patient with severe, symptomatic aortic stenosis who was admitted for TAVI. During balloon inflation the valve (Edwards SAPIEN XT) dislocated into the left ventricle as a result of the watermelon effect caused by a narrow, severely calcified sino-tubular junction. A second valve of the same type and size was immediately implanted in a suboptimal position in order to reduce severe aortic regurgitation. This facilitated the use of veno-arterial extracorporeal membrane oxygenation support (V-A ECMO). The patient was thereafter stabilized and transferred for urgent surgery, where both prosthetic valves were removed. The aortic valve was replaced with an additional reconstruction of the ascending aorta.

Azygos Vein ICD Lead Implantation Lowers Defibrillation Threshold in a Patient with Hypertrophic Cardiomyopathy

A 14-year-old boy with hypertrophic cardiomyopathy (HCM) diagnosed at the age of 1 year and with massive left ventricular hypertrophy suffered an episode of ventricular fibrillation during mild effort. He underwent a dual-chamber implantable cardioverter defibrillator (ICD) implantation. The defibrillation threshold testing (DFT) was ineffective. Subcutaneous multi-coli arrays tunneled into the left postero-lateral position and connected to the superior vena cava (SVC) port of the dual-chamber ICD were added to increase the myocardial mass involved in the defibrillation shock pathway. A new DFT was unsuccessful. The patient was transferred to our hospital for myectomy. An epicardial defibrillation patch was placed on the left ventricular lateral wall, but again, DFT testing was ineffective using the right ventricular (RV) coil to lateral patch as shock pathway. Another epicardial defibrillation patch was then placed on the inferior wall. In this case, DFT testing was effective with a defibrillation pathway between the two patches and the can. In November 2015, a high shock impedance alarm was recorded through remote monitoring, thus compromising the safety of the ICD shock pathway. The patient underwent the implant of a new trans-venous defibrillation coil lead in the azygos vein. After few months, the patient developed symptomatic severe aortic regurgitation and underwent an aortic valve replacement. During the operation, DFT testing was performed and was successful. Our case illustrates that azygous vein ICD lead implantation is efficacious in HCM with massive hypertrophy and high DFT, and prompts further studies to systematically investigate its efficacy in this particular subgroup of the HCM population.