Mitral Annular Disjunction of Degenerative Mitral Regurgitation: Three-Dimensional Evaluation and Implications for Mitral Repair.

BACKGROUND: The dynamic consequences of mitral annular disjunction (MAD) on the mitral apparatus and the left ventricle remain unclear and are crucial in the context of mitral surgery. Thus, the aim of this study was to assess mitral valvular, annular, and ventricular dynamics in mitral valve prolapse (MVP) stratified by presence of MAD. METHODS: In 61 patients (mean age, 62 ± 11 years; 25% women) with MVP and severe mitral regurgitation undergoing mitral surgery between 2009 and 2016, valvular and annular dimensions and dynamics by two-dimensional transthoracic and three-dimensional transesophageal echocardiography and left ventricular dimensions and dynamics were analyzed stratified by presence of MAD before and after surgery. RESULTS: MAD (mean, 8 ± 3 mm) was diagnosed in 27 patients (44%; with a mean effective regurgitant orifice area of 0.55 ± 0.20 cm2 and similar to patients without MAD), more frequently in bileaflet prolapse (52% vs 18% in patients without MAD, P = .004), consistently involving P2 (P = .005). Patients with MAD displayed larger diastolic annular areas (mean, 1,646 ± 410 vs 1,380 ± 348 mm2), circumferences (mean, 150 ± 19 vs 137 ± 16 mm), and intercommissural diameters (mean, 48 ± 7 vs 43 ± 6 mm) compared with those without MAD (P ≤ .008 for all). Dynamically, mid- and late systolic excess intercommissural diameter, annular area, and circumference enlargement were associated with MAD (P ≤ .01 for all). MAD was also associated with dynamically annular slippage, larger prolapse volume and height (P ≤ .007), and larger leaflet area (mean, 2,053 ± 620 vs 1,692 ± 488 mm2, P = .01). Although patients with MAD compared with those without MAD showed similar ejection fractions (mean, 65 ± 5% vs 62 ± 8%, respectively, P = .10), systolic basal posterior thickness was increased in patients with MAD (mean, 19 ± 2 vs 15 ± 2 mm, P < .001), with higher systolic thickening of the basal posterior wall (mean, 74 ± 27% vs 50 ± 28%) and higher ratio of basal wall thickness to diameter (P ≤ .01 for both). However, after mitral repair, MAD disappeared, and LV diameter, wall thickness, and wall thickening showed no difference between patients with MAD and those without MAD (P ≥ .10 for all). CONCLUSIONS: MAD in patients with MVP involves a predominant phenotype of bileaflet MVP and causes profound annular dynamic alterations with considerable expansion and excess annular enlargement in systole, potentially affecting leaflet coaptation. MAD myocardial and annular slippage simulates vigorous left ventricular function without true benefit after surgical annular suture. Thus, although MAD does not hinder the feasibility and quality of valve repair, it requires careful suture of ring to ventricular myocardium, lest it persist postoperatively.

Concomitant Mitral Regurgitation in Patients With Chronic Aortic Regurgitation.

BACKGROUND: Etiology, mechanisms, and survival of mitral regurgitation (MR) plus hemodynamically-significant chronic aortic regurgitation (AR) are mostly unknown. OBJECTIVES: The purpose of this study was to investigate the prevalence, mechanisms, etiologies, and survival impact of coexistent ≥ moderate MR in AR patients. METHODS: Consecutive patients with ≥ moderate-severe AR were retrospectively identified between 2004 and 2019. RESULTS: Of 1,239 eligible patients (61 ± 18 years, 80% men), 1,072 (86%) had pure AR, and 167 (14%) had AR + MR (9% functional mitral regurgitation [FMR] [84% nonischemic] and 5% organic mitral regurgitation [OMR] [62% degenerative]). At baseline transthoracic echocardiogram, pure AR versus AR + OMR versus AR + FMR exhibited differences in age (59 ± 18, 62 ± 16, and 73 ± 14 years, respectively), female sex (18%, 27%, and 39%, respectively), symptoms (36%, 41%, and 64%, respectively), atrial fibrillation (5%, 17%, and 36%, respectively), left ventricular (LV) ejection fraction (59%, 58%, and 46%, respectively), LV end-systolic dimension and volume index, ≥ moderate tricuspid regurgitation (TR) (7%, 35%, and 53%, respectively), and right ventricular systolic pressure (32 ± 11, 45 ± 15, and 50 ± 14 mm Hg, respectively), all p < 0.0001. After a median follow-up of 5.2 years (interquartile range: 2.2 to 10.0 years) and adjusting for demographics, New York Heart Association functional class, aortic valve surgery, LV ejection fraction, LV end-systolic dimension and volume index, presence of FMR was independently associated with all-cause mortality (p ≤ 0.004). Compared with pure AR, AR + MR + TR exhibited the highest adjusted risk of death (2.4-fold; p < 0.0001). When compared with expected population survival, excess mortality risks of pure AR, AR + OMR, and AR + FMR were 1.25-fold, 1.76-fold, and 2.34-fold, respectively (all p ≤ 0.02). CONCLUSIONS: In hemodynamically significant AR, coexistent MR is not uncommon (approximately 14%) and mostly comprises FMR and less commonly OMR. As compared with pure AR, AR + MR + TR exhibit the largest mortality risk. Both AR + OMR and AR + FMR carry a survival penalty compared with the general population, but AR + FMR is associated with the largest excess mortality and represents an advanced stage within the AR clinical spectrum.

Shear Stress and Aortic Strain Associations With Biomarkers of Ascending Thoracic Aortic Aneurysm.

BACKGROUND: This study aims to investigate the association of wall shear stress (WSS) and aortic strain with circulating biomarkers including matrix metalloproteinases (MMP), tissue inhibitors of metalloproteinase (TIMP), and exosomal level of microRNA (miRNA) in ascending aortic aneurysms of patients with bicuspid or tricuspid aortic valve. METHODS: A total of 76 variables from 125 patients with ascending aortic aneurysms were collected from (1) blood plasma to measure plasma levels of miRNAs and protein activity; (2) computational flow analysis to estimate peak systolic WSS and time-average WSS (TAWSS); and (3) imaging analysis of computed tomography angiography to determine aortic wall strain. Principal component analysis followed by logistic regression allowed the development of a predictive model of aortic surgery by combining biomechanical descriptors and biomarkers. RESULTS: The protein activity of MMP-1, TIMP-1, and MMP-2 was positively correlated to the systolic WSS and TAWSS observed in the proximal ascending aorta (eg, R = 0.52, P < .001, for MMP-1 with TAWSS) where local maxima of WSS were found. For bicuspid patients, aortic wall strain was associated with miR-26a (R = 0.55, P = .041) and miR-320a (R = 0.69, P < .001), which shows a significant difference between bicuspid and tricuspid patients. Receiver-operating characteristics curves revealed that the combination of WSS, MMP-1, TIMP-1, and MMP-12 is predictive of aortic surgery (area under the curve 0.898). CONCLUSIONS: Increased flow-based and structural descriptors of ascending aortic aneurysms are associated with high levels of circulating biomarkers, implicating adverse vascular remodeling in the dilated aorta by mechanotransduction. A combination of shear stress and circulating biomarkers has the potential to improve the decision-making process for ascending aortic aneurysms to a highly individualized level.

Institutional learning experience for combined edge-to-edge tricuspid and mitral valve repair.

BACKGROUND: Transcatheter edge-to-edge repair with MitraClip is only approved for treatment of mitral regurgitation but is increasingly used to treat concomitant tricuspid regurgitation (TR) due to its common coexistence and association with poor outcomes. This study aimed to describe the learning curve associated with the challenge of off-label treatment of concomitant TR. METHODS: This is a retrospective review of initial and consecutive patients who underwent combined edge-to-edge repair of mitral and tricuspid valves (TVs) at our institution from August 2017 to October 2019. RESULTS: Repair of both valves with MitraClip was performed in 22 patients (median age 81.5 years, 32% female). Mean procedure time was 176 ± 47 min; mean fluoroscopy time was 65 ± 24 min. Procedure duration in the first tertile was significantly longer (223 ± 13 min) than in the third tertile (143 ± 23 min, p = .0003). Median number of total clips placed per case was 3; in 15 patients (68%), the anterior and septal leaflets of the TV were clipped. The average changes in mean right atrial (RA) and left atrial (LA) pressures were -1.7 ± 2.5 mmHg (p = .0080) and -3.2 ± 4.6 mmHg (p = .0045), respectively. The average changes in RA and LA V-wave heights were -3.3 ± 4.0 mmHg (p = .0009) and -8.1 ± 9.9 mmHg (p = .038), respectively. There was a significant trend toward decreasing residual TR over the course of the series (p = .046). At 30 days, survival was 100% and mean NYHA class decreased from 2.8 to 1.8 (p < .0001). CONCLUSIONS: Combined edge-to-edge tricuspid and mitral valve repair is safe and feasible. With experience, procedure duration and residual TR decreased.

Patterns of ascending aortic dilatation and predictors of surgical replacement of the aorta: A comparison of bicuspid and tricuspid aortic valve patients over eight years of follow-up.

BACKGROUND: Predictors of thoracic aorta growth and early cardiac surgery in patients with bicuspid aortic valve are undefined. Our aim was to identify predictors of ascending aorta dilatation and cardiac surgery in patients with bicuspid aortic valve (BAV). METHODS: Forty-one patients with BAV were compared with 165 patients with tricuspid aortic valve (TAV). All patients had LV EF > 50%, normal LV dimensions, and similar degree of aortic root or ascending aorta dilatation at enrollment. Patients with more than mild aortic stenosis or regurgitation were excluded. A CT-scan was available on 76% of the population, and an echocardiogram was repeated every year for a median time of 4 years (range: 2 to 8 years). Patterns of aortic expansion in BAV and TAV groups were analyzed by a mixed-effects longitudinal linear model. In the time-to-event analysis, the primary end point was elective or emergent surgery for aorta replacement. RESULTS: BAV patients were younger, while the TAV group had greater LV wall thickness, arterial hypertension, and dyslipidemia than BAV patients. Growth rate was 0.46 ±â€¯0.04 mm/year, similar in BAV and TAV groups (p = 0.70). Predictors of cardiac surgery were aorta dimensions at baseline (HR 1.23, p = 0.01), severe aortic regurgitation developed during follow-up (HR 3.49, p 0.04), family history of aortic aneurysm (HR 4.16, p 1.73), and history of STEMI (HR 3.64, p < 0.001). CONCLUSIONS: Classic baseline risk factors were more commonly observed in TAV aortopathy compared with BAV aortopathy. However, it is reassuring that, though diagnosed with aneurysm on average 10 years earlier and in the absence of arterial hypertension, BAV patients had a relatively low growth rate, similar to patients with a tricuspid valve. Irrespective of aortic valve morphology, patients with a family history of aortic aneurysm, history of coronary artery disease, and those who developed severe aortic regurgitation at follow-up, had the highest chances of being referred for surgery.

Coexistent bicuspid aortic valve and mitral valve prolapse: epidemiology, phenotypic spectrum, and clinical implications.

AIMS: Bicuspid aortic valve (BAV) and mitral valve prolapse (MVP) are common but the prevalence and significance of coexistent conditions are unknown. This study investigated the prevalence, phenotypic expression, and clinical significance of coexistent MVP-BAV. METHODS AND RESULTS: Retrospective comparison of MVP-BAV and MVP-tricuspid aortic valve (TAV) prevalence including de novo echocardiographic analysis of all MVP-BAV patients between 2005 and 2015 was performed. The community prevalence of MVP-BAV was 2.7% vs. 3.4% for MVP-TAV (P = 0.45). Posterior mitral leaflet (PML)-MVP was the most common phenotype in both BAV and TAV (P = 0.38), but anterior mitral leaflet (AML)-MVP was twice more prevalent in BAV (31% vs. 15%, P < 0.0001). Among 130 subjects with coexistent MVP-BAV (81% men, 51 ± 16 years old), 31 (24%) exhibited AML:PML length ratio ≥3:1, termed large-AML prolapse (LAP-BAV), who had predominant BAV regurgitation when compared with those with non-LAP-BAV (P ≤ 0.001). An extreme phenotype of LAP-BAV with giant-AML prolapse and diminutive PML (GAP-BAV) was identified in 18/130 (14%) subjects. Compared with posterior-MVP-BAV, GAP-BAV patients were younger (42 ± 15 vs. 64 ± 12 years, P < 0.0001), had larger aortic annulus (28 ± 3 vs. 26 ± 2 mm, P = 0.01), and 61% had ≥ moderate BAV regurgitation (vs. 16%, P = 0.0007). Mitral repair occurred in 37/130 (28%) subjects. After median follow-up 5.5 months (4-83), 4/5 (80%) GAP-BAV patients required redo surgery for recurrent mitral regurgitation vs. 2/31 (6%) for non-LAP-BAV (P = 0.001). CONCLUSION: The community prevalence of coexistent MVP-BAV is comparable to MVP-TAV and their most common phenotype is posterior-MVP. However, anterior-MVP is twice as prevalent in MVP-BAV. A large-AML phenotype (LAP-BAV) with predominant BAV regurgitation affects 24% of MVP-BAV patients. An extreme phenotype of anterior-MVP (GAP-BAV) affects 14% of BAV patients; characterized by exceptionally large AML, diminutive PML, high mitral and aortic regurgitation prevalence, and high mitral repair failure rate.

Long-term outcomes of melody valve-in-valve implantation for bioprosthetic mitral valve dysfunction.

OBJECTIVES: This study reports long-term outcomes percutaneous Melody valve (Medtronic, Minneapolis, Minnesota) valve-in-valve therapy in patients with prosthetic valve dysfunction. BACKGROUND: Repeat valve surgery is associated with high morbidity and mortality. Transcatheter mitral valve-in-valve is a promising alternative in patients at high-risk for cardiac surgery. METHODS: We conducted a retrospective cohort analysis of 13 patients who underwent Melody valve-in-valve for bioprosthetic dysfunction from July 2011 to October 2013. RESULTS: Mean age was 74.4 years, 46% male, and STS score 13.5 ± 6.8%. All patients had NYHA class 3 or 4 symptoms with improvement to class 1 or 2 in 82% post procedure. Median follow-up was 4.5 years with longest follow-up of 5.5 years. Thirty-day mortality was 15.4% with 1-year mortality of 25% and no other reported deaths until 4.5 years. 76.9% of patients had mitral gradient of 5 mmHg or less immediately postprocedure. One patient required repeat valve procedure for structural deterioration at 4.4 years. At 1, 3, and 5 year follow-ups 75% of patients were NYHA class 1 or 2, mean gradients were 4.5 ± 0.6, 6.8 ± 0.5, and 7.5 ± 0.7, respectively. Using 4-point scale, mitral regurgitation postprocedure was 0.8 ± 0.6. At 1, 3, and 5 years this increased to 1.0 ± 1.1, 1.3 ± 1.3, and 2.5 ± 2.1, respectively. CONCLUSIONS: Melody valve-in-valve therapy was effective with 1-year symptomatic improvement. Prosthesis deterioration requiring repeat intervention was observed in one patient. Larger cohorts with multicenter registries are needed to determine the role of this percutaneous valve-in-valve therapy as valve technology and procedural techniques improve.

Incidence and Management of Hemopericardium: Impact of Changing Trends in Invasive Cardiology.

OBJECTIVE: As invasive cardiovascular care has become increasingly complex, cardiac perforation leading to hemopericardium is a progressively prevalent complication. We sought to assess the frequency, etiology, and outcomes of hemorrhagic pericardial effusions managed through a nonsurgical echo-guided percutaneous strategy. PATIENTS AND METHODS: Over a 10-year period (January 1, 2007, to December 31, 2016), 1097 unique patients required pericardiocentesis for clinically important pericardial effusions. Of these 411 had drainage of hemorrhagic effusions (defined as a pericardial hemoglobin level >50% of serum hemoglobin or frank blood in the setting of cardiac perforation). Clinical characteristics, echocardiographic data, details of the procedure, and outcomes were determined. RESULTS: Median patient age was 67 years (interquartile range, 56-76 years), and 60% were men. The procedure was emergent in 83% and elective in 17%. The site of pericardiocentesis was determined by echo-guidance in all: 68% from the left para-apical region, 18% from the left or right parasternal areas, and 14% were subxyphoid. Half (n=215 [52%]) occurred after cardiac perforation with percutaneous interventional procedure (ablation, n=94; device lead implantation, n=65; percutaneous coronary intervention, n=22; other, n=34), whereas 30% followed cardiac or thoracic surgery. Pericardial fluid volume drained was 546±440 mL. In 94% of cases, echo-guided pericardiocentesis was the only treatment of the effusion needed, whereas definitive surgery was required in 25 (6%) cases for persistent bleeding or acute management of the underlying etiology. There was no procedural mortality. Late mortality was better for hemorrhagic effusions compared with a contemporary cohort with nonhemorrhagic effusions. CONCLUSION: Echocardiographic guidance allows rapid successful pericardiocentesis in the setting of hemopericardium related to microperforation with interventional procedures, malignancy, or pericarditis, with most not requiring surgical intervention. Surgery should remain the first-line approach for aortic dissection or myocardial rupture.

Clinical Outcome of Degenerative Mitral Regurgitation: Critical Importance of Echocardiographic Quantitative Assessment in Routine Practice.

BACKGROUND: Echocardiographic quantitation of degenerative mitral regurgitation (DMR) is recommended whenever possible in clinical guidelines but is criticized and its scalability to routine clinical practice doubted. We hypothesized that echocardiographic DMR quantitation, performed in routine clinical practice by multiple practitioners, predicts independently long-term survival and thus is essential to DMR management. METHODS: We included patients diagnosed with isolated mitral valve prolapse from 2003 to 2011 and any degree of mitral regurgitation quantified by any physician/sonographer in routine clinical practice. Clinical/echocardiographic data acquired at diagnosis were retrieved electronically. The end point was mortality under medical treatment analyzed by Kaplan-Meier method and proportional hazard models. RESULTS: The cohort included 3914 patients (55% male) mean age (±standard deviation) 62±17 years with left ventricular ejection fraction 63±8% and median after routinely-measured effective regurgitant orifice area (EROA) [interquartile range], 19 [0-40] mm2. During follow-up (6.7±3.1 years), 696 patients died under medical management, and 1263 underwent mitral surgery. In multivariate analysis, routinely-measured EROA was associated with mortality (adjusted hazard ratio, 1.19; 95% confidence interval, 1.13-1.24; P<0.0001 per 10 mm2) independently of left ventricular ejection fraction and end-systolic diameter, symptoms, and age/comorbidities. The association between routinely-measured EROA and mortality persisted with competitive risk modeling (adjusted hazard ratio, 1.15; 95% confidence interval, 1.10-1.20; P<0.0001 per 10 mm2), or in patients without guideline-based class I/II surgical triggers (adjusted hazard ratio, 1.19; 95% confidence interval, 1.10-1.28; P<0.0001 per 10 mm2) and in all subgroups examined (all P<0.01). Spline curve analysis showed that, compared with general population mortality, excess mortality appears for moderate DMR (EROA ≥20 mm2), becomes notable at EROA ≥30 mm2, and steadily increases with higher EROA levels (eg, higher EROA levels beyond the 40 mm2 threshold). CONCLUSIONS: Echocardiographic DMR quantitation is scalable to routine practice and is independently associated with clinical outcome. Routinely-measured EROA is strongly associated with long-term survival under medical treatment. Excess mortality versus the general population appears in the moderate DMR range and steadily increases with higher EROA. Hence, individual EROA values should be integrated into therapeutic considerations, in addition to categorical DMR grading.

Transcatheter and Surgical Management of Mitral Paravalvular Leak: Long-Term Outcomes.

OBJECTIVES: The aim of this study was to report the use trends and immediate and long-term outcomes of a large cohort of patients who underwent redo surgery or transcatheter repair of paravalvular leaks (PVLs) at a tertiary referral center. BACKGROUND: Percutaneous treatment of mitral PVL has emerged as an alternative to surgical treatment in high-risk surgical candidates. There are limited data on the utilization trends, safety, and efficacy of both procedures in the management of mitral PVL. METHODS: Patients who underwent treatment of mitral PVL at the Mayo Clinic between January 1995 and December 2015 were enrolled. Utilization trends, procedural details, technical success, and in-hospital and long-term outcomes were assessed. RESULTS: Three hundred eighty-one patients underwent percutaneous (n = 195) or surgical (n = 186) treatment of mitral PVLs. The mean age was 66 ± 12 years, and 37% of patients had bioprosthetic valves. Technical success was higher in the surgical group (95.5% vs. 70.1%; p < 0.001). In-hospital major adverse events were more common after surgery (22.5% vs. 7.7%; p < 0.001). In-hospital death occurred in 3.1% and 8.6% of patients undergoing percutaneous and surgical treatment, respectively (p = 0.027). However, in a multivariate logistic regression analysis, only active endocarditis, chronic renal failure, and severe mitral annular calcifications were significant predictors of in-hospital mortality. Reintervention rates were similar (11.3% vs. 17.2% in the percutaneous and surgical groups, respectively; p = 0.10), with the majority of reinterventions in the percutaneous group occurring early because of residual leak or persistent hemolysis. After risk adjustment, there was no significant difference in long-term survival between patients who underwent surgical versus transcatheter treatment of PVLs. CONCLUSIONS: In contemporary practice, patients with symptomatic mitral PVLs are best treated with an integrated team approach incorporating both surgical and percutaneous techniques. Patient selection and timing of intervention are critical to achieve optimal results.

Prognosis of Light Chain Amyloidosis With Preserved LVEF: Added Value of 2D Speckle-Tracking Echocardiography to the Current Prognostic Staging System.

OBJECTIVES: This study evaluated whether 2-dimensional speckle-tracking echocardiography (2D-STE) has incremental value for prognosis over traditional clinical, echocardiographic, and serological markers-with main focus on the current prognostic staging system-in light-chain (AL) amyloidosis patients with preserved left ventricular ejection fraction. BACKGROUND: Cardiac amyloidosis (CA) is the major determinant of outcome in AL amyloidosis. The current prognostic staging system is based primarily on serum levels of cardiac troponin T (cTnT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and free light chain differential (FLC-diff). METHODS: Consecutive patients with biopsy-proven AL amyloidosis and left ventricular ejection fraction ≥55% were divided into group 1 with CA (n = 63) and group 2 without CA (n = 87). Global longitudinal strain (GLS) by 2D-STE was performed with Vivid E9 (GE Healthcare Co., Milwaukee, Wisconsin) and syngo Velocity Vector Imaging (VVI) software (Siemens Medical Solutions USA, Inc., Malvern, Pennsylvania) (GLSGE and GLSVVI, respectively). RESULTS: Thirty-two deaths (51%) occurred in group 1 and 13 (15%) in group 2 (p ≤ 0.001). Group 1 had thicker walls, lower early diastolic tissue Doppler velocity at septal mitral annulus, and greater left ventricular mass, left atrial volume, glomerular filtration rate, FLC-diff, cTnT, and NT-proBNP (p < 0.001). For the entire cohort, GLSGE ≥ -14.81, GLSVVI ≥-15.02, cTnT, NT-proBNP, FLC-diff, age, left ventricular wall thickness, early diastolic tissue Doppler velocity at septal mitral annulus, diastolic dysfunction grade, glomerular filtration rate, deceleration time, and left atrial volume were univariate predictors of death. In a multivariate Cox model, GLSGE ≥-14.81 (hazard ratio [HR]: 2.68; 95% confidence interval [CI]: 1.07 to 7.13; p = 0.03), FLC-diff, NT-proBNP, and age were independent predictors of survival. There was also a strong trend for GLSVVI ≥-15.02 (HR: 2.44; 95% CI: 0.98 to 6.33; p = 0.055). Using a nested logistic regression model, GLSGE (p = 0.03) and GLSVVI (p = 0.05) provided incremental prognostic value over cTnT, NT-proBNP, and FLC-diff. For survival analysis limited to group 2 (non-CA), GLSGE and GLSVVI both predicted all-cause mortality (GLSGE HR: 1.23; 95% CI: 1.03 to 1.47 [p = 0.02]; GLSVVI HR: 1.22; 95% CI: 1.01 to 1.49 [p = 0.04], respectively). CONCLUSIONS: 2D-STE predicted outcome and provided incremental prognostic information over the current prognostic staging system, especially in the group without CA.

Aborted TAVR following aortic balloon valvuloplasty.

An 80-year-old man with symptomatic severe aortic valve stenosis was referred for transcatheter aortic valve replacement (TAVR) after balloon aortic valvuloplasty (BAV). The TAVR procedure was aborted because of identification of a mobile mass attached to the leading edge of the right cusp of the aortic valve on a transesophageal echocardiography. Surgical aortic valve replacement (SAVR) was performed and this mass was found to be an aortic cusp fenestration rupture that was caused by the BAV.

Mitral hemi-arcade: an unusual modification of a rare anomaly.

Congenital anomalies of the mitral valve are rare. A mitral arcade is defined as a fibrous continuity between the papillary muscles and the anterior mitral leaflet creating a hammock like suspension. We present images of a mitral anomaly that consists of a direct attachment of the anterolateral papillary muscle to the anterior mitral leaflet, which we have labeled as a "hemi-arcade." We discuss the surgical findings and review the available literature regarding a mitral "arcade".

Coronary artery fistula: single-center experience spanning 17 years.

BACKGROUND: Coronary artery fistula (CAF) is an uncommon form of congenital heart disease. It is often diagnosed incidentally during angiograms. We have reported on clinical characteristics, diagnosis, and management of CAF. METHODS: Retrospective review of a tertiary referral institution's database identified 30 patients with CAF between 1987 and 2004. Mean follow-up was 31.61 +/- 48.03 months. RESULTS: Mean age was 60 +/- 12.7 years. Most common site of CAF origin was the left anterior descending artery (LAD) (14, 46.7%). The most common site of drainage was the main pulmonary artery (15, 50%). Therapeutic strategies were based on symptoms and shunt size. Conservative management was the option in 17 patients (56.7%) with small shunts and mild or no symptoms. Patients with moderate/severe symptoms and/or large shunts were treated with either percutaneous embolization (6, 20%) or surgical ligation (7, 23.3%). Four patients (13.3%) died during follow-up. No deaths were reported in the embolization group, two patients died of cancer in the conservative management group, and two patients died in the surgical group due to cardiac tamponade and cancer, respectively. CONCLUSIONS: Origin of CAF was predominantly from the left system. Clinical presentations were variable depending on type, size of fistula, and the presence of other cardiac conditions. Management of CAF is still controversial and treatment of adult asymptomatic patients with nonsignificant shunting is still a matter of debate. Newer imaging modalities may enhance noninvasive diagnosis. A national registry is necessary for further insights into optimal treatment for large fistulae and the natural history of smaller fistulae.