Progressive worsening of aortic regurgitation due to detachment of the aortic valve commissure with multimodality imaging to elucidate pathogenesis: a case report.

Background: Aortic regurgitation (AR) associated with detachment of the aortic valve commissure is extremely rare. We present a case of progressively worsening severe chronic AR due to detachment of the aortic valve commissure during hospitalization that was confirmed with multimodality imaging. Case summary: A 50-year-old male with Marfan syndrome visited our hospital to receive treatment for cholelithiasis. Pre-operative examination revealed severe AR and aortic root aneurysm. Because the patient was asymptomatic, it was decided that cholecystectomy should be performed first. However, the patient's heart failure worsened acutely when his blood pressure increased just before induction of anaesthesia. The patient required intubation and management of heart failure. Five days later, the patient underwent cholecystectomy. He was treated for heart failure and underwent open heart surgery on the 35th hospital day. Intraoperative transoesophageal echocardiography revealed that his AR was caused by both enlargement of the aortic root and localized dissection of the aortic valve commissure, which was supported by intraoperative findings and histopathological evaluation. Aortic regurgitation was exacerbated by a new localized dissection, resulting in acute worsening of heart failure. Discussion: Aortic valve commissure detachment can easily lead to sudden onset of severe AR, deteriorating haemodynamics, and acute pulmonary oedema. Since delayed medical treatment leads to poor clinical outcomes, prompt and accurate diagnosis and appropriately timed surgical intervention are essential. This very rare case of severe AR worsening due to spontaneous aortic valve commissure dissection was evaluated with multiple modalities during hospitalization. Understanding this clinical condition will help cardiologists provide better medical care.

Non-rheumatic giant left atrium: An illustrative case successfully treated by surgical intervention.

A 45-year-old male presented to us with decompensated heart failure. He had been diagnosed as having atrial fibrillation when he was 31 years old. Transthoracic and transesophageal echocardiography revealed an excessive left atrial (LA) enlargement with left ventricular dysfunction and severe functional mitral regurgitation. There were no specific findings of rheumatic valve disease. He underwent surgical mitral valve replacement and LA volume reduction surgery after optimal medical therapy. Surgically-removed specimens of the LA and the anterior mitral leaflet were examined and there were no specific histopathological findings suggesting the specific etiology of the giant LA in this patient. The patient's condition significantly improved after the surgery without any cardiac events ever since. .

Longitudinal Evaluation of Mitral Valve Leaflet Remodeling After Acute Myocardial Infarction: Serial Quantitation of Valve Geometry Using Real-Time 3-Dimensional Echocardiography.

BACKGROUND: Recent animal studies have suggested that mitral valve (MV) leaflet remodeling can occur even without significant tethering force and that the postinfarct biological reaction would contribute to the histopathologic changes of the leaflet. We serially evaluated the MV remodeling in patients with anterior and inferior acute myocardial infarction (MI), by using 2- and 3-dimensional transthoracic echocardiography. Additional histopathologic examinations were performed to assess the leaflet pathology. METHODS: Sixty consecutive first-onset acute MI (anterior MI, n=30; inferior MI, n=30) patients who underwent successful primary percutaneous coronary intervention were examined (1) before primary percutaneous coronary intervention, (2) at 6-month follow-up, and (3) at follow-up 1 year or later after onset. MV complex geometry including MV leaflet area and thickness was analyzed using dedicated software. Additional histopathologic study compared 18 valves harvested during surgery for ischemic mitral regurgitation (MR). RESULTS: MV area and thickness incrementally increased during the follow-up period. MV leaflet area significantly increased (anterior MI: 5.59 [5.28-5.98] to 6.54 [6.20-7.26] cm2/m2, P<0.001; inferior MI: 5.60 [4.76-6.08] to 6.32 [5.90-6.90] cm2/m2, P<0.001), and leaflet thickness also increased (anterior MI: 1.09 [0.92-1.24] to 1.45 [1.28-1.60] mm/m2, P<0.001; inferior MI: 1.15 [1.03-1.25] to 1.44 [1.27-1.59] mm/m2, P<0.001); data represent onset versus ≥1 year. Larger annuls, larger tenting, and a reduced leaflet area/annular ratio with smaller coaptation index were observed in patients with persistent ischemic MR compared with those without significant ischemic MR. Histopathologic examinations revealed that MV thickness was significantly greater in chronic ischemic MR compared with acute ischemic MR (1432.6±490.5 versus 628.7±278.7 µm; P=0.001), with increased smooth muscle cells and fibrotic materials. CONCLUSIONS: MV leaflet remodeling progressed both in area and thickness after MI. This is the first clinical study to record the longitudinal course of MV leaflet remodeling by serial echocardiography.

Anatomical and physiological assessment of a symptomatic anomalous origin of the right coronary artery from the pulmonary artery by noninvasive imaging examinations.

A 58-year-old female visited our hospital complaining of fatigue and repetitive pre-syncope during exercise. She had suffered with those symptoms for decades and had visited some hospitals where she did not get conclusive diagnosis. She was ultimately diagnosed as having subclinical ventricular septal defect unrelated to her symptoms. Echocardiography revealed normal ventricular function, but color Doppler showed multiple abnormal intramyocardial blood flow signals in ventricular septum and moderator band, which suggested abnormally developed coronary perfusion. Dilated left main trunk and abnormal color Doppler signal running into the main pulmonary trunk were also observed. Coronary computed tomography angiography revealed that right coronary artery originated from pulmonary artery and made connection to distal left anterior descending artery, which led to the final diagnosis of anomalous origin of the right coronary artery from the pulmonary artery. Exercise stress echocardiography showed wall motion abnormalities in inferior region which suggested ischemia in right coronary artery (RCA) territory. Her symptoms could be derived from the myocardial ischemia. She was referred to surgical intervention considering the possible risk of cardiac events including sudden death. Re-implantation of the abnormal RCA to the aorta was performed successfully. Her long-year persistent symptoms were completely alleviated, and repeat exercise stress echocardiography showed no evidence of ischemia. .

Unique mechanism of mitral valve prolapse in atrial septal defect: Three-dimensional insights into mitral complex geometry using real-time transesophageal echocardiography.

BACKGROUND: Mitral valve prolapse (MVP) is often identified in patients with atrial septal defect (ASD), which occasionally require surgical intervention at the time of ASD closure or even long after the surgery. Ventricular and valvular geometric characteristics in preoperative ASD patients were evaluated by three-dimensional (3D) transesophageal echocardiography. METHODS AND RESULTS: Mitral valve (MV) complex geometry was quantitatively measured by 3D transesophageal echocardiography in 11 ASD patients (Qp/Qs > 1.5) and 11 controls. The ASD group had a significantly larger indexed prolapse volume and height, with a larger anterior mitral leaflet than controls (0.53 [0.33-0.75] vs 0.057 [0.027-0.11] mL/m2 , P = .0001; 2.89 [2.13-3.50] vs 0.92 [0.48-1.32] mm/m2 , P < .0001; 391.3 [346.4-445.1] vs 295.3 (281.9-330.0) mm2 /m2 , P = .011, respectively). The right ventricular (RV)-to-left ventricular (LV) end-systolic diameter ratio was larger in the ASD group than in the control group (1.34 [0.96-1.45] vs 0.85 [0.75-0.88], P = .004). The indexed inter-papillary muscle distance (IPMD) was significantly shorter in the ASD group than in the control group (7.77 [6.55-8.24] vs 9.71 [8.64-10.8] mm/m2 , P = .011). IPMD was significantly correlated with the RV-LV end-systolic diameter ratio (r = -.70, P = .017). CONCLUSIONS: Inward shift of the LV papillary muscle tips due to RV dilation may be a major mechanism of MV prolapse in ASD. At the same time, positive remodeling of the anterior leaflet was observed in the ASD group, which may compensate for the billowing leaflet geometry to maintain effective coaptation. Three-dimensional assessment of the MV apparatus geometry will help to further understand perioperative mitral regurgitation in patients with ASD.

Significance of preoperative right ventricular function on mid-term outcomes after surgical ventricular restoration for ischemic cardiomyopathy.

OBJECTIVES: To analyze our surgical experiences with surgical ventricular restoration (SVR) for dilated ischemic cardiomyopathy (ICM) and to determine the significance of preoperative right ventricular (RV) function on outcomes. METHODS AND RESULTS: This study retrospectively analyzed 19 patients who underwent SVR between April 2010 and May 2016. Their mean age and New York Heart Association functional class were 62 ± 11 years and 2.9 ± 0.8, respectively. The preoperative mean left ventricular (LV) end-systolic volume index and LV ejection fraction (LVEF) were 134 ± 56 mL/m2 and 24 ± 7%, respectively. The preoperative mean RV fractional area change (RVFAC) to quantify RV systolic function was 33 ± 13%, as assessed by transthoracic echocardiography. The mean follow-up period was 47 ± 20 months. Three patients died of cardiac causes during the follow-up, with the 3-year and 5-year freedoms from cardiac-related death of 89% and 79%, respectively. Major adverse cardiac events (MACEs) occurred in ten patients, with the 3-year and 5-year MACE-free survival rates of 58% and 41%, respectively. RVFAC (risk ratio [RR] = 0.92, 95% confidence interval [CI] 0.86-0.98, p = 0.01) and LVEF (RR = 0.83, 95% CI 0.68-0.97, p = 0.02) were significant predictors of MACEs in the multivariate analysis. Patients with RVFAC of < 35% had significantly poorer MACE-free survival rates (33% at 3 years) than those with RVFAC of ≥ 35% (80% at 3 years). CONCLUSION: SVR for ICM provided acceptable freedom from cardiac-related death; however, MACEs commonly occurred and was associated with RV dysfunction.

Functional Mitral Regurgitation: Imaging Insights, Clinical Outcomes and Surgical Principles.

Functional mitral regurgitation (MR; FMR) is the most common type of MR and its development is associated with increased morbidity and mortality. Leaflet tethering with apical shift of the papillary muscle due to adverse left ventricular remodeling and loss of normal leaflet coaptation is the principal mechanism of FMR. Echocardiography plays a central role in the assessment of the FMR. The development of 3D echocardiography has allowed for assessment of the geometric changes of mitral valve morphology and spatial relationship with the left ventricle that accompanies FMR. 2D/3D echocardiographic findings, clinical outcomes of FMR are reviewed and role of surgical intervention is discussed.