Left Coronary Cusp Research Articles

Implications of Asymmetry and Valvular Morphotype on Echocardiographic Measurements of the Aortic Root in Bicuspid Aortic Valve

Transthoracic echocardiography (TTE) and magnetic resonance imaging (MRI) have yielded excellent results in aortic root diameter measurement in patients with tricuspid aortic valve. However, accuracy in bicuspid aortic valve (BAV), often associated with aortic root asymmetry, is not fully defined. The aim of this study was to determine the agreement between TTE and MRI in proximal ascending aortic diameters in patients with BAVs. Seventy-six consecutive patients with BAVs (mean age, 53±15years; 65% men) who underwent both TTE and MRI for ascending aortic assessment in a follow-up protocol were included in the study. Maximum aortic root and ascending aortic diameters were compared. For the whole population, TTE slightly underestimated aortic root diameter (difference, -0.8±2.9mm; P=.02). However, agreement was significantly better in BAV with fusion of the left and right coronary cusps than with fusion of the right coronary and noncoronary cusps, both with (type 1) and without (type 0) raphe (mean difference, 0.1±2.5 vs -2.8±2.8mm, P<.001, respectively). In raphe BAV, mean absolute differences of maximum diameters between both techniques were significantly greater in asymmetric versus symmetric aortic roots (3.3±2.2 vs 1.6±1.9mm, P=.002). BAV type and root asymmetry were independent related to measurement disagreement between both modalities. Although TTE is the technique of choice in the follow-up of patients with BAVs, aortic root diameter measurements may be inaccurate in the presence of root asymmetry and in BAV with fusion of the right coronary and noncoronary cusps. In these cases, cross-sectional imaging, with MRI or computed tomography, to confirm aortic diameters may be advisable.

Optimal positioning of self-expanding valves before deployment decreases paravalvular regurgitation following transcatheter aortic valve replacement.

To evaluate the association between measurements performed during Medtronic CoreValve (MCV) deployment and paravalvular leak (PVL). The MCV can be recaptured and repositioned, allowing the TAVR operator to implant at a more favorable position. The association between angiographic measurements of MCV position while the valve is recapturable and PVL post deployment has not been investigated. 493 patients undergoing TAVR with MCV (January 2011-July 2017) were included. PVL was defined as intraprocedural aortic regurgitation that was judged clinically to require balloon postdilation. Depth of the valve at the left coronary cusp (LCC) and noncoronary cusp (NCC) were measured when the valve was 80% deployed. An optimal cutoff value for the ratio LCC/NCC for PVL was identified in 40 patients. Using this cutoff value, the association between LCC/NCC and PVL was then validated in 453 patients. The median LCC/NCC was 1.51 (interquartile range 1.06-1.89).The optimal cutoff value for LCC/NCC was 1.48 (93% sensitivity, 77% specificity, AUC0.85). In the validation group 112 (24.7%) patients had PVL. For LCC/NCC ≥ 1.48, the incidence of PVL was lower compared to LCC/NCC < 1.48 (9.58% vs. 41.78%, P < 0.0001). LCC/NCC of 1.48 had a sensitivity of 79.5% and specificity of 63.6% for PVL (AUC0.72). In a multivariate model, LCC/NCC < 1.48 independently predicted PVL (OR = 6.67, 95% CI 3.96-11.23, P < 0.0001). Positioning the MCV such that the LCC/NCC is ≥1.48 may result in less PVL.

Low power ablation for left coronary cusp ventricular tachycardia—Efficacy and long-term outcome

BackgroundThe left coronary cusp is an uncommon but well-known site for the ablation of idiopathic ventricular tachycardia (VT). Proximity to the left coronary ostium makes ablation of this arrhythmia challenging. Different power settings have been described by various operators. Our objective was to describe the outcomes with low power ablation. MethodsOnce mapping confirmed origin from the left coronary cusp, ablation was performed if the best site was situated at least 5 mm from the left coronary ostium. Ablation was started at 15 W and, if successful, was stopped after 30 s. When required, higher powers were used up to 30 W. ResultsTen patients with VT or premature ventricular beats mapped to the left coronary cusp were included in the study. No ablation was performed in one patient because of proximity to the left coronary ostium. Successful ablation was performed in eight of the other nine patients with a mean power of 18.1 ± 5.3 W and duration of 42.2 ± 13.5 s. There were no complications. All the eight patients remained free of recurrence at 16.8 ± 16.5 months of follow-up. ConclusionsVT can be ablated from the left coronary cusp close to the left coronary ostium. Ablation with low power is effective in achieving immediate success which is also durable with time while avoiding complications.

Anomalous Left Coronary Artery From the Right Sinus of Valsalva in a Patient Presenting with ST-Segment Elevation in Lead aVR: A Case Report

BackgroundAnomalous coronary artery origins appear in roughly 1% of coronary angiograms, and up to 15% of syncope and sudden cardiac death events can be attributed to anomalous coronaries. Patients with an anomalous coronary artery arising from the opposite sinus may initially present with syncope and electrocardiographic findings of ischemia. Case ReportWe describe a case in which an adolescent male presented with exercise-induced angina and syncope, and his initial electrocardiogram (ECG) showed diffuse ST-segment depression with ST-segment elevation in lead aVR. Cardiac catheterization revealed there was no coronary ostium in the left coronary cusp, and the left coronary artery had an anomalous origin from the right cusp. The patient received urgent left internal mammary artery-to-left anterior descending artery coronary bypass and a saphenous vein graft to the ramus intermedius. After he underwent 6 months of medical therapy with β-blockade and angiotensin-receptor blockade, his left ventricular systolic function improved to low-normal level (left ventricular ejection fraction, approximately 50%). Why Should an Emergency Physician Be Aware of This?ST-segment elevation in lead aVR is strongly prognostic for left main or triple-vessel coronary artery disease. However, in patients who present with syncope and few other coronary artery disease risk factors, this ECG finding should be suggestive of an ischemic event caused by an anomalous left coronary artery. Early recognition of this pattern of clinical signs and ECG findings by an emergency physician could be critical for making the correct diagnosis and risk stratifying the patient for early coronary angiography and urgent surgical revascularization.

Familial Anomalous Origin of Right Coronary Artery from the Left Coronary Sinus

Anomalous origin of a coronary artery from the opposite sinus of Valsalva is a rare congenital anomaly. The prevalence of familial clustering of coronary artery anomalies is unknown. Here we describe the case of a father and son, both of whom presented with major adverse cardiac events due to Anomalous origin of a coronary artery from the opposite sinus of Valsalva and both had right coronary artery arising from the left coronary cusp with an interarterial course.

Aortic elasticity and the influence of valve morphology in children with bicuspid aortic valve.

The study group included 44 patients and 42 controls. Patients were divided into groups according to the type of valve opening as horizontal - fusion between right and left coronary cusps - and vertical - fusion between right-non-coronary cusps; according to age they were divided into younger (5-10 years) and older patients (11-16 years). Our study did not include valve phenotype with fusion between left and non-coronary cusps. Systolic-diastolic diameters of aortic annulus, sinus valsalva, sinutubular junction, arcus, and ascending-descending and abdominal aorta were measured and z-scores were obtained. Aortic strain, distensibility, and stiffness index were calculated. Flow-mediated dilatation of brachial artery was studied. z-Scores at annulus, sinus valsalva, sinutubular junction, and ascending aorta were higher in study patients (p=0.001, p=0.0001, p=0.0001, p=0.0001, respectively). z-Scores of sinus valsalva and sinotubular junction were higher in the horizontal group than in the vertical group (p=0.006, p=0.023, respectively). z-Score was over +2 in 51% of patients with horizontal morphology and 33% of patients with vertical morphology (p=0.0001). Ascending aorta was more distensible and less stiff in the study group (11.3±5.63 versus 7.91±4.5, p=0.002; 4.76±3.60 versus 6.19±3.44 cm2.dyn-1.10-6, p=0.033, respectively). Stiffness index of ascending, arcus, and abdominal aorta were higher in the horizontal group (p=0.004, p=0.038, p=0.006, respectively). Ascending aorta was more distensible and less stiff in the younger group (p=0.007, p=0.027, respectively) but did not differ in the older group compared with the control group. Aortic dimensions are enlarged in patients with bicuspid aortic valve starting from childhood, suggesting the presence of generalised aortopathy. Aortic elasticity is increased at young age and decreased with age.

Papillary fibroelastoma of the aortic valve’s left coronary leaflet: case reports and review

Papillary fibroelastoma is a relatively rare cardiac tumor, which etiology is undisclosed and not entirely clear. A relatively small number of case descriptions and investigations makes it difficult to accumulate the material, which is absolutely necessary in view of the diversity and ambivalence of the tactics of the management of the different groups of patients. The article represents the case reports of the papillary fibroelastoma of the left coronary leaflet of the aortic valve in a 79 y.o. man and 15 y.o. woman. In both cases patients hadn't any complains; both fibroelastomas were accidentally detected. Due to echocardiography and CMR, it was succeed to obtain data on the hemodynamic significance of these tumors, to reveal their precize localization and to assess their mobility, also to differentiate them with other masses.

Impact of Aortic Root Anatomy and Geometry on Paravalvular Leak in Transcatheter Aortic Valve Replacement With Extremely Large Annuli Using the Edwards SAPIEN 3 Valve

ObjectivesThe aim of this study was to determine factors affecting paravalvular leak (PVL) in transcatheter aortic valve replacement (TAVR) with the Edwards SAPIEN 3 (S3) valve in extremely large annuli. BackgroundThe largest recommended annular area for the 29-mm S3 is 683 mm2. However, experience with S3 TAVR in annuli >683 mm2 has not been widely reported. MethodsFrom December 2013 to July 2017, 74 patients across 16 centers with mean area 721 ± 38 mm2 (range: 684 to 852 mm2) underwent S3 TAVR. The transfemoral approach was used in 95%, and 39% were under conscious sedation. Patient, anatomic, and procedural characteristics were retrospectively analyzed. Valve Academic Research Consortium–2 outcomes were reported. ResultsProcedural success was 100%, with 2 deaths, 1 stroke, and 2 major vascular complications at 30 days. Post-dilatation occurred in 32%, with final balloon overfilling (1 to 5 ml extra) in 70% of patients. Implantation depth averaged 22.3 ± 12.4% at the noncoronary cusp and 20.7 ± 9.9% at the left coronary cusp. New left bundle branch block occurred in 17%, and 6.3% required new permanent pacemakers. Thirty-day echocardiography showed mild PVL in 22.3%, 6.9% moderate, and none severe. There was no annular rupture or coronary obstruction. Mild or greater PVL was associated with larger maximum annular and left ventricular outflow tract (LVOT) diameters, larger LVOT area and perimeter, LVOT area greater than annular area, and higher annular eccentricity. ConclusionsTAVR with the 29-mm S3 valve beyond the recommended range by overexpansion is safe, with acceptable PVL and pacemaker rates. Larger LVOTs and more eccentric annuli were associated with more PVL. Longer term follow-up will be needed to determine durability of S3 TAVR in this population.

Evaluation of the pulmonary artery potential using a 20-polar circumferential catheter and three-dimensional integrated intracardiac echocardiography.

Prolongation of the pulmonary artery potentials (PAPs) in response to short coupling intervals was related to polymorphic QRS configurations during the ventricular tachycardia originating above the pulmonary valve (PA-VT). This prospective study was aimed to investigate the mechanisms of polymorphic changes during the PA-VT. We performed the mapping above the pulmonary valve using a 20-polar circumferential catheter and three-dimensional integrated intracardiac echocardiography in 9 consecutive patients with outflow tract arrhythmias undergoing catheter ablation (UMIN ID: UMIN000021682). The location of successful ablation was right ventricular outflow tract (RVOT) in 6 patients, above the pulmonary valve in 1 patient, left coronary cusp in 1 patient, and unknown in 1 patient. The PAP was detected in six (67%) patients with bipolar voltage of 0.56 ± 0.27mV. Pacing from bipolar electrodes of the circumferential catheter located above the pulmonary valve captured the PA myocardium only in 1 patient who had the PA-VT (100% in PA-VT vs 0% in non-PA-VT, P = 0.0046), and slight changes of the QRS morphology was observed in accordance with the conduction delay from the stimulus to activation of the RVOT myocardium. The selective PAP capture with conduction delays evoked by bipolar stimulations through a 20-polar circumferential catheter may be a characteristic property of patients with the PA-VT. Conduction delays within the PA and PA-RVOT junction appears to contribute polymorphic QRS changes during the PA-VT.

Outcomes with a latest generation self-expandable, intra-annular, re-sheathable transcatheter heart valve system: analysis of patients with impaired left ventricular function and determinants for pacemaker implantation.

We herein report a single-center experience with the Portico TAVI system. Main focus was set on outcomes in patients with impaired left ventricular (LV) function and predictors for permanent pacemaker (PPM) implantation. Between 05/2014 and 10/2017, 106 consecutive patients received TAVI using the Portico device. For comparison of patients with/without impaired LV function, and determination of multi-slice computed tomography and electrocardiogram correlates for PPM implantation subgroups were built (Subgroup I: LVEF ≥ 50%, n = 69, Subgroup II: LVEF < 50%, n = 37; Subgroup A: no PPM implantation, n = 89; Subgroup B: PPM implantation, n = 17). Data were retrospectively analyzed. Device success was 95.3% (101/106) with resulting transvalvular peak/mean pressure gradients of 10.6 ± 5.6/5.4 ± 3.1 and PVL ≥ moderate in 5/106 patients (4.7%). 30-day mortality was 4.7% (5/106; subgroup I 3/69, 4.3%; subgroup II 2/37, 5.4%; p = 1.0). Regarding VARC-2 adjudicated clinical endpoints stroke (3/106, 2.8%; subgroups I/II: 2/69 vs. 1/37, p = 1.0) and acute kidney injury (9/106, 8.5%; subgroups I/II: 6/69 vs. 3/37, p = 1.0), no significant differences were found. Postprocedural PPM implantation occurred in 17.9% (19/106) of the patients. Logistic regression for PPM implantation demonstrated predictive character of left coronary cusp (LCC) calcium load in zone 1 (annular plane to coronary ostia) (p = 0.05, threshold 193 mm3) and total calcium load of zone 1 (p = 0.05, threshold 751.2 mm3). The Portico valve demonstrates a satisfying safety outcome, especially for patients with impaired LV function. Results of the CE-mark trial were confirmed, except for higher PPM rates. PPM rates are in accordance with other reports on this TAVI platform.

Impact of "high" implantation on functionality of self-expandable bioprosthesis during the short- and long-term outcome of patients who undergo transcatheter aortic valve implantation: Is high implantation beneficial?

High position of the self-expandable bioprosthesis CoreValve/Evolut R has been proved to affect immediate hemodynamics of the valve. Whether this may have any impact on long-term procedural outcome has not been defined yet. The purpose of this study was to assess whether the final position of aortic bioprosthesis affects its long-term functionality. Consecutive patients (pts) who underwent successful TAVI procedure were evaluated and separated into 2 groups according to the implantation depth (ID): Group I: pts with 4mm <ID ≤13mm; Group II: pts with ID ≤4mm. ID was measured utilizing the final aortography after device delivery and was defined as the distance both from the native non- and left coronary cusp to the deepest edge of the deployed bioprosthesis in the left ventricle. Clinical outcome and echocardiographic parameters were recorded before the procedure, at discharge, at 1-month and 1-year-follow-up. One hundred and ninety-eight pts (80±5.5years, 107 males [54%]) treated with the CoreValve/Evolut R bioprostheses were recorded. Group I appeared to have higher peak gradient (17±6.5 vs 14±5.5mmHg, P=.02) as well as Vmax (2±0.4 vs 1.84±0.38m/s, P=.02) at follow-up after 1year when compared with Group II (ID <4mm). Grouping for ID did not affect all-cause 1-year mortality. Paravalvular aortic regurgitation, as well as LVEF at discharge, proved to be independent predictors of all-cause 1-year mortality when adjusted for cofactors. Implantation depth under 4mm seems to have a favorable effect on long-term hemodynamic valve functionality.

Predictors of paravalvular regurgitation and permanent pacemaker implantation after TAVR with a next-generation self-expanding device.

To identify predictors of paravalvular regurgitation (PVR) and permanent pacemaker implantation (PPI) following TAVR with a next-generation self-expanding device. Device landing zone (DLZ) calcification, angiographic implantation depth, and baseline and procedural characteristics were analyzed in 212 patients being treated with the ACURATE neo aortic bioprosthesis. PVR was none/trace in 57.1% and ≥ mild in 42.9% (37% mild, 6% moderate). DLZ calcification (705 (IQR 240-624) vs. 382 (IQR 240-624)mm3; P < 0.001) as well as absolute calcium asymmetry (233 ± 159 vs. 151 ± 151mm3; P < 0.001) was significantly higher in patients with PVR ≥ mild. On multivariate analysis, calcification of the aortic valve cusps (AVC) > 410.6mm3 was independently associated with PVR ≥ mild. PPI rate was 10.3% (n = 20). Patients with and without need for PPI had similar total DLZ calcium volume (740 (IQR 378-920) vs. 536 (IQR 315-822)mm3; P = 0.263), but exhibited different calcium distribution patterns: LVOT calcium > 41.4mm3 in the sector below the left coronary cusp (LVOTLC) was associated with increased PPI risk (26.9 vs. 7.7%; P = 0.008). The quantity of AVC calcium predicts residual PVR. Multivariable analysis identified LVOTLC calcium, pre-existing RBBB, and age > 82.7years as independent predictors of PPI. Based on these risk factors, a patient's individual PPI risk can be stratified ranging from 3.8 to 100%.

Transcatheter Laceration of Aortic Leaflets to Prevent Coronary Obstruction During Transcatheter Aortic Valve Replacement: Concept to First-in-Human

ObjectivesThis study sought to develop a novel technique called bioprosthetic or native aortic scallop intentional laceration to prevent coronary artery obstruction (BASILICA). BackgroundCoronary artery obstruction is a rare but fatal complication of transcatheter aortic valve replacement (TAVR). MethodsWe lacerated pericardial leaflets in vitro using catheter electrosurgery, and tested leaflet splaying after benchtop TAVR. The procedure was tested in swine. BASILICA was then offered to patients at high risk of coronary obstruction from TAVR and ineligible for surgical aortic valve replacement. BASILICA used marketed devices. Catheters directed an electrified guidewire to traverse and lacerate the aortic leaflet down the center line. TAVR was performed as usual. ResultsTAVR splayed lacerated bovine pericardial leaflets. BASILICA was successful in pigs, both to left and right cusps. Necropsy revealed full length lacerations with no collateral thermal injury. Seven patients underwent BASILICA on a compassionate basis. Six had failed bioprosthetic valves, both stented and stent-less. Two had severe aortic stenosis, including 1 patient with native disease, 3 had severe aortic regurgitation, and 2 had mixed aortic valve disease. One patient required laceration of both left and right coronary cusps. There was no hemodynamic compromise in any patient following BASILICA. All patients had successful TAVR, with no coronary obstruction, stroke, or any major complications. All patients survived to 30 days. ConclusionsBASILICA may durably prevent coronary obstruction from TAVR. The procedure was successful across a range of presentations, and requires further evaluation in a prospective trial. Its role in treatment of degenerated TAVR devices remains untested.

Catheter ablation of ventricular arrhythmias with QRS morphology resembling that of aortic sinus cusp arrhythmias: Significance of mapping the left pulmonary sinuscusp.

There is a close anatomical relationship between aortic sinus cusp (ASC) and the left pulmonary sinuscusp (LPSC). The study was to investigate the significance of mapping and ablation of LPSC for ventricular arrhythmias (VAs) with QRS morphology resembling that of ASC arrhythmias. In 33 consecutive patients undergoing successful catheter ablation of idiopathic VAs with ASC arrhythmia-like QRS morphology, LPSC was mapped carefully. Among the 33 patients studied, QRS morphology resembled that of arrhythmias of either right or left coronary cusp (LCC or RCC) origin in 17 and 16 patients, respectively. Out of 12 arrhythmias with the earliest potential in LCC during left-sided mapping, an earlier potential in LPSC than in LCC was recorded in 2 arrhythmias, the same earliest potential in LPSC as in LCC was recorded in 6 arrhythmias, and 5 (42%) were finally ablated successfully in LPSC using the reversed U curve. Out of 16 arrhythmias with the earliest potential in the RCC during left-sided mapping, an earlier potential in LPSC than in RCC was recorded in 4 arrhythmias, the same earliest potential in LPSC as in RCC was recorded in 3 arrhythmias, and 4 (25%) were finally ablated successfully in LPSC using the reversed U curve. For VAs with ASC arrhythmia-like QRS morphology, mapping in LPSC could have unique electrophysiologic characteristics, and some of them could be eliminated in LPSC using reversed U curve.

Fetal severe aortic regurgitation due to absence of left coronary leaflet of aortic valve

AbstractCongenital absence of aortic valve cusps is a rare congenital heart malformation, which is commonly associated with other cardiac defects. Isolated absence of aortic valve or one leaflet is extremely rare and has not been described antenatally. We present the first case of a fetus with isolated congenital severe aortic regurgitation from absent aortic cusp and pathological specimen of this rare entity. On fetal echocardiography at 19 weeks of gestation, there were dilated aortic root and severe aortic regurgitation causing ‘to and fro’ colour flow signals across left ventricular outflow. This caused severe LV systolic dysfunction, significant ventricular dyssynchrony and cardiomegaly. On fetal autopsy, aortic root was dilated. Left coronary cusp was absent while other cusps showed mild thickening. Left ventricle was dilated and hypertrophied. Pulmonary valve leaflets were normal.

Aortic valve replacement using autologous pericardium: single centre experience with the Ozaki technique.

To assess the clinical implementation and report preliminary results of a novel technique called the Ozaki procedure for stentless aortic valve replacement through reconstruction of the valve leaflets from autologous pericardium. Between September 2015 and May 2017 30 patients (20 males, mean ± standard deviation age 66.83 ± 10.55 years) suffering from aortic stenosis (AS, n = 7), aortic regurgitation (AR, n = 12), or a combination of both (AS/AR, n = 11) were assigned for an Ozaki procedure. The glutaraldehyde-treated autologous pericardium was intraoperatively customised and tailored according to individual sinus measurements and appropriate Ozaki templates (CE marked). Mean and peak preoperative transvalvular pressure gradients in patients with AS were 46.34 ± 14.71 and 78.00 ± 22.54 mm Hg, respectively and effective orifice area was 0.93 ± 0.26 cm2. Ejection fraction was preserved at 57.37 ± 10.33%. Twenty-four valves were tricuspid and 6 bicuspid; 13 patients had concomitant cardiac surgery (coronary artery bypass graft, mitral valve repair, replacement of ascending aorta). Mean ± SD cross-clamp time for replacement only was 85.18 ± 18.10 minutes and perfusion time 104.76 ± 38.52 minutes. Cusp sizes were 27.76 ± 3.52 mm for the left coronary cusp (CC), 28.20 ± 3.51 mm for the right CC and 29.20 ± 3.34 mm for non-CC. Mean and peak postoperative gradients decreased to 8 ± 3.55 and 14.8 ± 6.21 mm Hg, respectively. Mean length of stay on the intensive care unit was 2.19 ± 2.34 days and in-hospital stay was 8.81 ± 2.04 days after isolated Ozaki procedures. No pacemaker had to be implanted after an isolated Ozaki procedure. Thirty-day mortality was 3.33% (n = 1). After 3 months, no patient presented with aortic stenosis, and regurgitation of the substituted valves was graded nil/trace in 85.71%, mild in 10.71%, and moderate in 3.57% of the patients. Ejection fraction remained unchanged at 58.89 ± 11.29%. No reoperation was required within the first 3 months. This aortic valve replacement technique has become available only recently. In our experience, it can be mastered after a relatively short training period, and has become part of our routine clinical toolbox. The use of autologous pericardium in combination with excellent haemodynamics may have the potential to overcome the structural disadvantages of biological aortic valves, to be beneficial in infective endocarditis, and to represent an alternative for patients with small annuli.

Single Coronary Artery Anomaly: A Case Report and Review of Literature.

IntroductionSingle coronary artery is a rare anomaly, which is usually associated with other cardiac congenital abnormalities.Case ReportA 56-year-old female presented with unstable angina. The patient reported complaints of typical chest pain on exertion few months prior to presentation, which progressed to become at rest. The pain was associated palpitations and dizziness. Past medical history was significant for hypertension and hyperlipidemia. Vital signs were stable. Physical examination was non-remarkable. Electrocardiogram showed normal sinus rhythm, with intermittent episodes of sinus bradycardia, and non-specific T-wave changes. Trans-thoracic echocardiogram showed normal left ventricular function and no segmental wall-motion abnormalities. Selective coronary angiography showed a normal left main coronary artery arising from left coronary cusp. The left main branched to a normal left anterior descending artery and to the left circumflex artery; a large vessel which supplied also the territory of the right coronary artery (RCA) through its terminal extension. Aortography showed absence of RCA with no other vessels arising from the right or non-coronary cusps. The patient was managed conservatively and discharged home with resolution of symptoms.ConclusionsWe report a rare case of isolated single coronary artery with absent RCA. The patient presented with unstable angina, and was managed conservatively. Cardiologists should be aware of this rare condition, which carries a potential risk of sudden cardiac death.

Challenges Faced During Surgical Management of the Anomalous Right Coronary Artery Arising From the Left Coronary Cusp in Adults

Purpose: The anomalous right coronary artery (ARCA) arising from the left coronary cusp is an uncommon congenital anomaly with varied clinical outcome, including sudden cardiac death. Surgical intervention in such patients is performed to prevent this fatal complication. A thorough knowledge of the proximal structures, including the take-off portion, is important in formulating the best surgical treatment option. Recent experiences of a single surgeon (MM) dealing with the ARCA highlight the challenges surgeons can face when dealing with this anomaly intraoperatively.

A Case of Severe Aortic Regurgitation Caused by Fissured Aortic Cusps Reconstructed with Autologous Pericardium

An 82-year-old woman was hospitalised in our institution because of worsening dyspnoea on effort. She had a history of aortic valve reconstruction with autologous pericardium (AVrC) for severe aortic stenosis 1 year and 9 months ago. Her transthoracic echo examination revealed that she had severe aortic regurgitation with preserved left ventricular systolic function. Compared with her previous echo findings shortly after AVrC, her left ventricle was clearly dilated with newly detected moderate mitral regurgitation. Transoesophageal echo was performed for further investigation, which showed that, in particular, her right coronary cusp (RCC) was obviously prolapsed toward the left ventricular outflow tract with wide aortic regurgitant jet. After conservative medical therapy, aortic valve replacement and mitral annular plasty were performed. Her removed autologous aortic cusps clarified the details: not only did the RCC, but also left coronary cusp have two clefts on both ends of each cusp, and the non–coronary cusp had one fissure on one end. Her symptoms improved dramatically after aortic valve replacement. Aortic valve reconstruction with autologous pericardium is characterised by excellent valvular durability with a low mortality. However, the patient in this case suffered from rapid deterioration of the reconstructed autologous aortic valve. According to a previous report, one AVrC case was required reoperation due to aortic regurgitation caused by prolapse of RCC, which was the same cusp as in this case. Autologous RCC for AVrC is usually the smallest and made from thinner side of pericardium. This may be the reason why RCC is the culprit cusp when aortic regurgitation occurs shortly after AVrC.

Ankylosing Spondylitis and Anti–Phospholipid Syndrome Resulting in Non–Bacterial Thrombotic Endocarditis: Challenges in Valvular Management

We present a difficult management case of a 39-year-old men with controlled ankylosing spondylitis who developed non–bacterial thrombotic endocarditis. He was initially admitted with pneumonia, when thoracic auscultation unveiled a pan–systolic murmur. Transthoracic echocardiogram showed moderate aortic stenosis (AS) and aortic regurgitation (AR). Subsequent transoesophageal echocardiogram (TOE) showed thickened right and left coronary cusps with moderate eccentric AR. This was thought to be secondary to valvulitis from ankylosing spondylitis and was planned for observant management. Repeat TOE at 6 months showed that his aortic valve pathology progressed to severe AS and AR, with a large mass on the anterior aspect of the valve measuring 1.3 × 1.2 cm (Figure 1). Soon after, he was admitted with an ischaemic right arm, from a thromboembolism, and underwent an uncomplicated thrombectomy. During this admission, TOE showed persistence of the mass structure and severe AS and AR. Serology was positive for primary antiphospholipid syndrome. A literature review showed that patients with antiphospholipid syndrome undergoing cardiac surgery had a perioperative morbidity and mortality of up to 50%. Owing to his extreme surgical risk, a consensus decision, from multiple specialities, was obtained to postpone surgery until he was anticoagulated for 3 months. With meticulous coordination with anaesthetics and haematology, he underwent an uncomplicated surgical mechanical aortic valve replacement. Histology showed myxoid degenerative changes of the valve without thrombus. At the 3-month review, he remained well and has planned life-long anticoagulation with international normalised ratio between 2.5 and 3.5. This case exemplifies the importance of multidisciplinary management of complex, systemic disorders where evidenced-based treatment and guidelines are deficit.