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Portopulmonary hypertension (PPHTN) is pulmonary arterial hypertension (PAH) associated with portal hypertension. It is a common condition among liver transplantation candidates; however, its association with congenital absence of the portal vein (CAPV) has not yet been established. CAPV is a very rare developmental anomaly, which is usually accompanied by abnormal mesenteric drainage that bypasses the liver. Here, we report a rare case of severe PPHTN secondary to CAPV.

Improvement in left ventricular (LV) systolic function after aortic valve replacement (AVR) has been observed in patients with aortic valve stenosis (AS). However, the factors that predict such recovery remain unclear. We sought to identify the predictive value of the LV spherical shape for LV functional recovery after "isolated" AVR in patients with severe AS and LV dysfunction. We examined 90 patients with severe AS and LV systolic dysfunction by echocardiography before and after AVR. Patients with known coronary artery disease, significant aortic or mitral regurgitation, and other cardiac surgery were excluded. LV end-diastolic and end-systolic volumes indexes and ejection fraction (EF) were measured by the Simpson method. LV mass index was calculated by the area-length method. LV end-diastolic and end-systolic sphericity were calculated as the ratio of the minor axis to the major axis of the left ventricle in apical 4-chamber view. The postoperative EF was significantly associated with preoperative EF, end-diastolic and end-systolic volumes indexes, LV mass index, and end-diastolic and end-systolic sphericity (all p0.001). Multivariate analysis revealed that preoperative EF, end-systolic volume index, and end-diastolic sphericity were independent parameters predicting postoperative EF. The sensitivity and specificity in predicting normalization of EF (or =50%) after AVR were 65% and 83% for end-diastolic sphericity0.57 and 68% and 91% for end-systolic sphericity0.47, respectively. In conclusion, LV spherical shape and dilatation predicted poor LV functional recovery after isolated AVR in severe AS.

Geometry of the proximal isovelocity surface area (PISA) in functional mitral regurgitation (MR) is elongated, leading to underestimation of the effective regurgitant orifice (ERO) area. This underestimation could be corrected by a new hemiellipsoidal method. Thirty patients with functional MR were examined by real-time 3-dimensional (D) echocardiography. Two ERO areas were calculated from 3D measurements: ERO area by the hemispheric method and that by the new hemiellipsoidal method with our customized program. Each ERO area was compared with that by the 2D quantitative Doppler method. Color 3D images showed an elongated PISA geometry including 2 geometric types ("mountain" or "valley") in all patients with functional MR. Our hemiellipsoidal method could be adapted for all geometric types of PISA and underestimated ERO area by only 26%, whereas the underestimation by the hemispheric PISA method was 49%. The underestimation by the hemispheric PISA method can be significantly corrected by our hemiellipsoidal method.

The geometry of the proximal isovelocity surface area (PISA) of functional mitral regurgitation (MR), which is conventionally assumed to be a hemisphere, remains to be clarified. We investigated the 3-dimensional (3D) geometry of PISA of functional MR as opposed to that of MR due to mitral valve prolapse (MVP) by real-time 3D echocardiography with color Doppler capability.Twenty-seven patients with functional MR and 27 patients with MVP were examined. The horizontal PISA length in the commissure-commissure plane and each PISA radius in 3 anteroposterior planes (medial, central, and lateral) were measured by real-time 3D echocardiography with 3D software. The effective regurgitant orifice (ERO) area was calculated with the maximum PISA radius and compared to that by 2D quantitative Doppler method.En-face 3D color Doppler images showed an elongated and slightly curved PISA geometry along the leaflet coaptation in functional MR, whereas the geometry was rounder in MVP. The PISA horizontal length in functional MR was longer than that in MVP (2.3 +/- 0.4 vs 1.2 +/- 0.2 cm, P.001). The PISA method with the maximum radius underestimated the ERO area by 2D quantitative Doppler method (by 24%) in functional MR, but not in MVP.The geometry of PISA in functional MR was elongated, distinctly different from the more focal pathology of MVP, leading to underestimation of the ERO area by PISA method.

Background In patients undergoing dialysis, aortic valve calcification and aortic stenosis are frequently found. However, the rate of progression of aortic stenosis is still unclear. Methods In all, 55 consecutive patients undergoing dialysis were followed up by echocardiography for a period of 12 months. Patients were divided into two groups: noncalcification (no or mildly calcified aortic valve) and calcification (moderate or heavily calcified aortic valve). Results The rate of progression of maximum aortic jet velocity and degression of aortic valve area were more rapid in calcification group than in noncalcification group (0.37 ± 0.36 m/s and 0.17 ± 0.29 m/s, P = .027; 0.17 ± 0.15 cm 2 and 0.04 ± 0.07 cm 2 , P Conclusions The degree of aortic stenosis progressed more rapidly in patients undergoing dialysis with aortic valve calcification than without aortic valve calcification.

The purpose of this study was to evaluate the feasibility of high-frequency transthoracic echocardiography for measuring the wall thickness and luminal area of the left anterior descending coronary artery (LAD). Fifteen patients underwent simultaneous high-frequency transthoracic echocardiography and intravascular ultrasound (IVUS) examinations. There were good agreements for wall thickness (0.38 +/- 0.05 vs 0.38 +/- 0.06 mm, p = 0.0004) and luminal area (3.24 +/- 1.05 vs 3.32 +/- 1.34 mm2, p0.0001) between high-frequency transthoracic echocardiography and IVUS measurements. High-frequency transthoracic echocardiography was reliable in the measurement of the wall thickness and luminal area of the LAD.

Background The precise mechanism of takotsubo-like left ventricular (LV) dysfunction remains unclear. Methods and Results Eight consecutive patients with takotsubo-like LV dysfunction were studied. In the acute phase and 3 weeks later, the coronary flow velocity spectrum and coronary flow velocity reserve (CFVR) were recorded by Doppler guidewire. The deceleration time of diastolic velocity (DDT; ms) was measured from the peak diastolic velocity to the point where the extrapolated line intersected the baseline. In all cases, CFVR of the 3 coronary arteries increased during follow-up (1.7±0.5 to 2.4±0.5 for the left anterior descending artery (p

Although coronary flow reserve (CFR) has been reported to be restricted in various conditions, there has been no report of CFR for patients with congestive heart failure (CHF). The purpose of this study was to assess coronary flow characteristics for patients with CHF.We studied 15 patients with CHF: 8 with dilated myocardiopathy and 7 with hypertensive heart disease. Phasic coronary flow velocities were obtained in the left anterior descending coronary artery at rest and during hyperemia (0.15 mg/kg/min adenosine triphosphate infusion intravenously) by transthoracic echocardiography before and after treatment of CHF. CFR was obtained from the ratio of hyperemic/baseline diastolic mean velocity.CFR was significantly restricted in the condition of CHF compared with that after improvement of CHF (1.5 +/- 0.2 vs 2.0 +/- 0.3, P.01). Baseline diastolic mean velocity in the condition with CHF was significantly greater than that after improvement of CHF (41 +/- 13 cm/s vs 33 +/- 13 cm/s, P = .04), although maximal hyperemic diastolic mean velocity was not significantly different before and after improvement of CHF (63 +/- 20 cm/s vs 61 +/- 19 cm/s, P = .68). After improvement of CHF, heart rate, along with left ventricular end-diastolic volume and dimension, were significantly decreased, and deceleration time of transmitral early filling flow was increased compared with before treatment of CHF. Blood pressure and ejection fraction were not significantly different before and after treatment of CHF.Restriction of CFR is demonstrated during CHF because of the elevation of baseline resting flow velocity, which might be related to increase in left ventricular preload and heart rate.

We assessed right ventricular (RV) geometric remodeling quantitatively in patients with chronic pulmonary hypertension (PH) secondary to left-sided heart disease using real-time 3-dimensional echocardiography by comparing segmental and total volumes to that in normal subjects. The comparison result revealed that RV geometric remodeling in the PH group mainly occurred at the basal, mid-basal, and mid-segments. Total RV end-diastolic and end-systolic volumes in the PH group were significantly larger than that in normal subjects.

Background The inner-half layer of the left ventricular (LV) wall is primarily affected by ischemia and increased LV afterload. We hypothesized that LV wall thickening of inner-half layer and coronary microvascular function are impaired in hypertensive patients, especially in those with electrocardiographic (ECG) strain, which is a marker of LV hypertrophy and adverse prognosis. Therefore, the aim of this study is to investigate the association of the ratio of inner- to outer-half layer of the LV myocardial deformation and coronary microvascular function with ECG strain in hypertensive patients. Methods We studied 98 hypertensive patients and 13 controls. Hypertensive patients were divided into 2 groups, with (S+) and without (S−) ECG strain. Coronary flow velocity reserve (CFVR) of the left anterior descending artery was evaluated using transthoracic echocardiography. Circumferential and radial strains of the LV wall were analyzed by two-dimensional echocardiographic speckle tracking method, and the inner- to outer-half layer ratio (in/out ratio) was assessed. Results Coronary flow velocity reserve of S+ (2.06 ± 0.65) was significantly smaller than those in S− and controls (3.03 ± 0.65 and 3.38 ± 0.51, respectively). In/out ratio in both circumferential and radial strains were decreased as well as in S+ patients. Furthermore, in/out ratio was directly proportional to CFVR. Conclusions Hypertensive patients with ECG strain possessed severely impaired CFVR and inner-half myocardial deformation. Moreover, the more severe the LV hypertrophy progresses, the greater is the impairment of coronary microvascular and LV inner-half myocardial deformation. Thus, CFVR and in/out ratio are useful quantitative markers that can render sensitive assessment of physiological changes in hypertensive heart disease.

Unexpected postoperative left ventricular (LV) dysfunction after valve repair for mitral regurgitation (MR) occurs in some patients with normal preoperative LV function. Identification of factors that predispose to such LV dysfunction would enhance our understanding of the indications and outcomes of surgery.We retrospectively analyzed pre- and postoperative (median fourth day) echocardiograms of 174 patients undergoing valve repair for pure and isolated MR. Preoperative MR volume was quantified by the quantitative Doppler and/or proximal isovelocity surface area method.There was an incremental predictive value of MR quantification over the current recommendations (global chi(2) from 48.14 to 81.57, P.001; Hosmer-Lemeshow test, P = .98), for postoperative LV dysfunction, defined as ejection fraction50%. The independent predictors were MR volume and LV end-systolic dimension (P.001 and P = .01, respectively). Sixty-nine patients underwent surgery before development of the current surgical criteria, namely, symptoms, atrial fibrillation, preoperative LV dysfunction, or pulmonary hypertension. Of these, MR volume was the only independent significant predictor (P.001) of unexpected postoperative LV dysfunction that developed in 14 patients (20%). Unexpected LV dysfunction could be predicted with sensitivity of 86% (95% CI 67%-100%) and specificity of 89% (95% CI 81%-97%), using the optimal cutoff of 80 mL for MR volume.Doppler-derived preoperative MR volume is a powerful predictor of unexpected postoperative LV dysfunction. Prompt mitral valve repair may be beneficial for patients with high likelihood of successful repair and MR volume/=80 mL.

Background: Little attention is given to development of mitral regurgitation (MR) in adults with atrial septal defect (ASD). The aim of the study was to determine the associated factors of MR in ASD adults before surgical repair and the fate of moderate to severe MR after surgery. Methods: We examined 71 consecutive patients with secundum ASD (47 ± 16 years) who underwent surgical repair. Clinical and echocardiographic variables including size of left and right heart systems and severity of MR and tricuspid regurgitation (TR) were investigated before and early after surgery. Results: Before ASD closure, 14 patients (20%) had moderate to severe MR and 25 patients (35%) showed mitral valve (MV) prolapse. The ASD patients with moderate to severe MR showed worse cardiovascular symptoms, increased occurrence of atrial fibrillation and MV prolapse, and greater left ventricular (LV) end-diastolic volume, left atrial area, and TR severity than those with none to mild MR (all P < 0.05). Among preoperative variables, TR severity, left atrial area, LV end-diastolic volume, and MV prolapse were associated with preoperative MR severity in all the patients (all P < 0.03). Isolated ASD closure (n=46) decreased MV prolapse (P=0.008). Preoperative moderate to severe MR decreased after ASD closure with and without MV surgery (n=9 and 5, respectively; both P < 0.05). Conclusions: Preoperative MR severity was associated with TR severity, dilated left heart chambers, and MV prolapse. MR decreased after ASD closure with and even without MV surgery.

Earlier studies demonstrated the ability of real-time 3-dimensional (3D) echocardiography (3DE) to measure left ventricular outflow tract (LVOT) area (A(LVOT)) in patients with hypertrophic cardiomyopathy (HCM). However, its clinical value is unknown.We sought to investigate the feasibility and accuracy of real-time 3DE-derived A(LVOT) to diagnose significant LVOT obstruction in a large number of patients with HCM.A total of 162 patients with HCM had 3DE by using a volumetric system. The smallest A(LVOT) during systole was determined by moving a 2-dimensional plane in 3D space. The pressure gradient across LVOT was assessed by continuous wave Doppler method. Provocation was performed in patients without significant LVOT obstruction (pressure gradient across LVOT50 mm Hg) at rest.Twenty (12%) patients with poor image quality of 3DE were excluded; 16 (28%) patients with a volumetric system, but only 4 (4%) patients with commercial equipment (P.001). In the remaining 142 patients, A(LVOT) inversely correlated with pressure gradient across LVOT both at rest (r = 0.82, P.001) and after provocation (r = 0.60, P.001). The value of A(LVOT) less than 0.85 cm(2) and less than 2.0 cm(2) predicted resting and provokable LVOT obstruction with sensitivity of 87% and 81%, and specificity of 77% and 90%, respectively.Real-time 3DE measurement of A(LVOT) was successful in diagnosing and quantifying LVOT obstruction at rest and after provocation in a large number of patients with HCM.

Geometric changes of tricuspid valve, particularly leaflets configuration, in functional tricuspid regurgitation (TR) remain to be defined. We sought to investigate geometric changes of tricuspid valve tenting in functional TR secondary to pulmonary hypertension by transthoracic real-time 3-dimensional echocardiography (3DE). Real-time 3DE was performed in 30 individuals (17 patients with TR and 13 control subjects). We used a novel software system with 3DE to reconstruct tricuspid geometry at midsystole. In patients with TR, tricuspid leaflets were tethered into right ventricle with apparent tenting showing a mountain-like bulging. Maximum tenting site was mostly located at the center of the tenting. Tenting volume was larger (4.2 +/- 2.4 vs 1.1 +/- 0.6 cm(3), P.001), and the maximum and mean tenting lengths were longer, than in control subjects. Annular area was also larger (11.3 +/- 2.3 vs 8.7 +/- 1.8 cm(2), P = .003) than in control subjects. Geometric changes in functional TR secondary to pulmonary hypertension were characterized by enlargement of tricuspid tenting volume and dilatation of annulus. This study suggested usefulness of the novel system with 3DE in evaluation of tricuspid valve geometry.

Background— New surgical strategies to restore the saddle shape of the mitral annulus are expected to increase annuloplasty effectiveness. Preoperative and postoperative configuration of the curved annulus, however, is difficult to quantify with 2-dimensional echocardiography. We sought to investigate the geometric deformity in the mitral annulus in ischemic mitral regurgitation (MR), comparing inferior and anterior myocardial infarction (MI) with the use of a custom quantitation software system with transthoracic 3-dimensional echocardiography. Methods and Results— We performed real-time 3-dimensional echocardiography in 23 patients with ischemic MR attributable to inferior MI or anterior MI and in 10 controls. Three-dimensional data were cropped into 18 radial planes, and we manually marked the annulus in mid systole. Three-dimensional annular images were reconstructed, and annular circumferences, areas, and heights were quantified. Annulus was significantly more dilated and flattened in ischemic MR than in controls and was further deformed in anterior MI as compared with inferior MI (control: circumference 9.9±0.7 cm, area 9.6±0.5 cm 2 , height 5.0±0.7 mm; inferior MI: circumference 11.5±1.2 cm [ P 2 [ P P P P 2 ] P P P P Conclusions— Mitral annulus flattens in ischemic MR. Deformity of the mitral annulus was greater in anterior MI group than in the inferior MI group.

An 80-year-old woman underwent aortic valve replacement with Freestyle stentless prosthetic valve for the stenosis. Four months later, she was admitted with myocardial ischemia. Coronary angiography revealed severe stenosis in the ostium of both right and left coronary arteries. Coronary artery bypass grafting was performed. One year later, percutaneous coronary intervention was carried out for the bilateral coronary arteries because of unstable angina. Intravascular ultrasonography demonstrated localized, membranous, homogeneous, and severe stenoses in the ostium of the right and left coronary arteries. Histological examination of a specimen taken by directional coronary atherectomy showed intimal hypertrophy, mucinous degeneration, and hyaline degeneration without reactive change. There were no findings of atherosclerosis. These clinical, angiographical histological and intravascular ultrasonography findings suggest that the immunological reaction to the heterograft was the mechanism of the bilateral ostial coronary arteries stenoses in the present case. The possibility of immunological reaction after aortic valve replacement with heterograft should be considered. There have been no report on intravascular echocardiographic and histological findings.

Development of left ventricular hypertrophy in severe aortic stenosis is associated with coronary microcirculatory dysfunction, as demonstrated by impaired coronary flow reserve. Recently, coronary flow reserve can be assessed noninvasively by transthoracic Doppler echocardiography (TTDE). This study assessed the relationship between coronary flow reserve obtained by TTDE and the hemodynamic parameters and left ventricular mass index in patients with aortic stenosis.Consecutive 29 patients (15 men, 14 women, mean age 72 +/- 11 years) with isolated mild to severe aortic stenosis were studied using TTDE to assess coronary flow reserve. Peak transvalvular pressure gradient across the aortic valve (peak AVG) and aortic valve area were measured by TTDE. Left ventricular mass index was measured by echocardiography.There were significant correlations between coronary flow reserve and peak AVG (r = -0.570, p = 0.001), left ventricular mass index (r = -0.620, p0.001), aortic valve area (r = 0.740, p0.001), and left ventricular rate pressure product (r = -0.660, p0.001). Multiple regression analysis showed that aortic valve area and peak AVG were independent factors for coronary flow reserve (p0.001, p = 0.048).Impairment of coronary flow reserve in patients with aortic stenosis is related to aortic valve area and peak AVG, rather than the degree of left ventricular hypertrophy.

Purpose: In cardiac 2-[F-18]fluoro-2-deoxy-D-glucose (FDG)-positron emission tomography (PET) examination, interpretation of myocardial viability in the low uptake region (LUR) has been difficult without additional perfusion imaging. We evaluated distribution patterns of FDG at the border zone of the LUR in the cardiac FDG-PET and established a novel parameter for diagnosing myocardial viability and for discriminating the LUR of normal variants. Materials and Methods: Cardiac FDG-PET was performed in patients with a myocardial ischemic event (n = 22) and in healthy volunteers (n = 22). Whether the myocardium was not a viable myocardium (not-VM) or an ischemic but viable myocardium (isch-VM) was defined by an echocardiogram under a low dose of dobutamine infusion as the gold standard. FDG images were displayed as gray scaled-bull’s eye mappings. FDG-plot profiles for LUR (= true ischemic region in the patients or normal variant region in healthy subjects) were calculated. Maximal values of FDG change at the LUR border zone (a steepness index; Smax scale/pixel) were compared among not-VM, isch-VM, and normal myocardium. Results: Smax was significantly higher for n-VM compared to those with isch-VM or normal myocardium (ANOVA). A cut-off value of 0.30 in Smax demonstrated 100% sensitivity and 83% specificity for diagnosing n-VM and isch-VM. Smax less than 0.23 discriminated LUR in normal myocardium from the LUR in patients with both n-VM and isch-VM with a 94% sensitivity and a 93% specificity. Conclusion: Smax of the LUR in cardiac FDG-PET is a simple and useful parameter to diagnose n-VM and ischVM, as well as to discriminate thr LUR of normal variants.

Ischemic mitral regurgitation (IMR) is a functional regurgitation characterized by structurally normal leaflets and subvalvular apparatus and is an important complication after myocardial infarction that is associated with excess mortality. 1 Reconstructive surgery, which restores a more normal alignment between the mitral annulus and displaced papillary muscles, might be beneficial in patients with IMR. However, little is known about the 3-dimensional (3-D) geometric changes of mitral leaflets and annulus after such reconstructive surgery for patients with IMR. We developed novel software, named Anatomical Image Creation System (AICS), which allows 3-D visualization and quantitative analysis of the mitral leaflets and annulus by using transthoracic real-time 3-D echocardiography (RT3DE). 2 We already demonstrated the apparent tenting of the mitral leaflets with flattened annulus in patients with IMR using this system. 3 In the present study we evaluated the 3-D geometric changes of the mitral leaflets and annulus in patients after reconstructive surgery for IMR using AICS. Methods We studied 3 patients who underwent mitral ring annuloplasty and concomitant left ventricular (LV) restorative surgery for severe IMR and severe LV systolic dysfunction caused by coronary artery disease. All the RT3DE examination was performed 1 week before and 3 weeks after the operation. LV systolic function and degree of mitral regurgitation (MR) were quantified by using 2-dimensional echocardiography. Using the transthoracic volumetric image by the RT3DE system with AICS, we created 3-D images of the mitral leaflets and annulus in midsystole for the 3-D quantitative measurements. The mitral leaflets’ tenting volume was calculated as a volume enclosed between the 3-D annular plane and the mitral leaflets. Mitral annular size was measured by using those 3-D data sets as well (surface area, circumference, commissure-commissure diameter, and anterior-posterior diameter). Details of the 3-D image creation and measurements are described in our previous reports. 2,3 All 3 patients provided written informed consent to the study protocol, which was approved by the Committee for the Protection of Human Subjects in Research at Kawasaki Medical School.