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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.

Objective. Although preservation of mitral annulus and papillary muscle alignment is important in mitral valve surgery, it has been difficult to measure the precise distance between mitral annulus and papillary muscles in clinical setting. We sought to quantify the distances between mitral annulus and papillary muscles by using the 3D quantitation software system, REAL VIEW®.Methods. We examined 10 patients with functional mitral regurgitation due to dilated cardiomyopathy and 10 normal controls by real-time 3D echocardiography. Using REAL VIEW®, the 3D images of mitral annulus and the tips of papillary muscles were reconstructed in end-systole. Mitral annular area and circumference, mitral leaflet tenting length and volume, and the 6 distances between the tips of papillary muscles and corresponding points of the mitral annulus were measured three-dimensionally.Results. Patients with functional mitral regurgitation due to dilated cardiomyopathy had larger annular size, apparent leaflet tenting and longer papillary muscle to mitral annular distances compared with the controls.Conclusions. We demonstrated 3D echocardiographic measurements of papillary muscle to mitral annular distances with REAL VIEW®, in clinical setting. This technique should be applicable to understandable demonstration and quantitative evaluation of subvalvular geometry in various mitral valve diseases.

We investigated the degree of mitral valve coaptation with a custom quantitation software system using transthoracic three-dimensional (3D) echocardiography. With real-time 3D echocardiography, we obtained transthoracic volumetric images in 20 healthy subjects and 20 patients with dilated cardiomyopathy. With our novel software system, the surface area of mitral valve tenting in the onset of mitral leaflet closure [O] and the timing of maximum closure of mitral leaflet [M] were reconstructed for quantitative measurement. The coaptation index was calculated by the following formula: [(3D tenting surface area in O-3D tenting surface area in M)/3D tenting surface area in O]. The coaptation index in patients with dilated cardiomyopathy was significantly smaller than that in healthy subjects (11% +/- 4.1% vs. 18% +/- 8.0%, P = .004). The custom quantitation software system with 3D echocardiography allowed us to assess the degree of mitral valve coaptation.

Background. Mitral valve prolapse is defined by two-dimensional (2D) echocardiography as an existence when the leaflet exceeds the annular level. However, the annular level set by 2D echocardiography differs in each view because of the "saddle-shape" of the annulus. In this study, we sought to investigate the degree of mitral valve prolapse from the three-dimensional (3D) dataset, which was created by the newly developed quantitative software system with transthoracic real-time 3D echocardiography, and then compare the data with normal subjects.Methods. Thirteen patients with mitral valve prolapse who were planned to have mitral valve repair (anterior leaflet, 6 patients; posterior leaflet, 7) and 21 healthy subjects were studied. Echocardiographic exams were performed by SONOS 7500 with X4 probe before surgery. Transthoracic volumetric images (full-volume mode) were obtained from the apical view. A newly developed software was used to crop the 3D data, and the mitral annulus and leaflets were traced in each cropped image. 3D images of the mitral leaflets and annulus were then automatically reconstructed for 3D quantitation. Prolapsed leaflet area was defined as the region that exceeded estimated 3D mitral annular level into the left atrium. We analyzed the % prolapsed area (ratio of the prolapsed leaflet area to the mitral annular area), and the maximum prolapse height from the 3D annular level to the maximum prolapsed site.Results. In patients with mitral valve prolapse, % prolapsed area was 20.9±5.8% (anterior 22.5±7.3%, posterior 19.4±4.0%). Maximum prolapse height was 8.4±5.8mm (anterior5.7±2.7mm, posterior10.7±6.9mm). In 16 of 21 healthy subjects, a small degree of prolapse was seen in the middle portion of the anterior scallop (% prolapsed area was 7.96±6.94%, pConclusions. Mitral valve prolapse could be quantitatively assessed by newly developed software system with real-time 3D echocardiography. Investigation of new standard of mitral valve prolapse in 3D insights would be required.

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.

Background.Although mitral complex geometry alteration has been speculated as a major mechanism for the reduction in mitral regurgitation (MR) after heart failure treatment, it has been difficult to recognize the mechanistic changes of the mitral apparatus because of the complex morphology and anatomical position. We investigated the contributions of the dynamic changes in mitral valve geometry with papillary muscle (PM) alignment to the reduction of functional MR after intensive heart failure treatment.Methods.Two dimensional/three-dimensional (2D/3D) echocardiography was performed in 10 patients with decompensated heart failure and functional MR before and after intensive therapy. We used novel software to analyze the volumetric images recorded by real-time 3D echocardiography. Reconstructed 3D images showed both leaflets and annulus configuration in relation to PM position. We measured maximum tenting length (max-Tent-L); mean tenting length (mean-Tent-L) and tenting volume (Tent-V); distances from anterior annulus to anterolateral PM (A-tethering length) and posteromedial PM (P-tethering length); the distance between two PM (interpapillary distance); and the angle made by the anterolateral PM, anterior annulus, and posteromedial PM (interpapillary angle).Results.MR decreased after intensive treatment in all patients (pConclusions.Dynamic changes in PM position during heart failure treatment resulted in the reduction of mitral valve tenting, which improved functional MR.

A 71-year-old woman was admitted with sudden-onset shoulder and back pain. The electrocardiograph showed sinus rhythm with ST-segment elevation in leads V4-6 and abnormal Q waves in leads V4-5. Echocardiography at the time of admission revealed akinesis in the left ventricular apical wall. However, coronary angiography did not reveal a stenotic lesion in any of the coronary arteries. Left ventriculography revealed apical ballooning with basal hyperkinesis. Ten hours after onset, the patient suddenly collapsed and went into cardiopulmonary arrest. This is a rare case of left ventricular rupture with takotsubo-like left ventricular dysfunction.

The incidence of aortic stenosis (AS) is increasing, and the progression of AS is more rapid in chronic renal failure patients treated by dialysis than those in the general population. We present 2 cases of rapid progression of AS in chronic renal failure patients treated by dialysis. Both cases had no findings of AS at the first echocardiographic examination. After 3.5 years (case 1) and 2 years (case 2) later, severe AS was noted in both patients. The progression of AS was more rapid in our 2 cases compared with previous reports. Frequent echocardiographic examination should be performed to evaluate valvular calcification and stenosis in patients with chronic renal failure treated by chronic dialysis.

Background The precise mechanism of tako-tsubo-like left ventricular (LV) dysfunction remains unclear, although recent studies have shown that activation of sympathetic tone might be involved. However, local release of catecholamines from cardiac sympathetic efferent neurons in patients with tako-tsubo-like LV dysfunction remains poorly understood. The purpose of this study was to investigate evidence of local release of catecholamines from the hearts of patients with tako-tsubo-like LV dysfunction. Methods and Results Five consecutive patients with tako-tsubo-like LV dysfunction were studied. After confirming LV apical ballooning and a normal coronary angiogram, sampling of blood for the measurement of plasma catecholamine levels was performed at the aortic root (Ao) and coronary sinus (CS). In all 5 cases, increased local release of norepinephrine from the heart was documented (597, 4,238, 2,121, 486, 371 pg/ml at the Ao; 836, 5,719, 3,386, 658, 472 pg/ml at the CS, respectively). Conclusions Increased cardiac catecholamines might cause the transient LV apical ballooning in patients with tako-tsubo-like LV dysfunction. (Circ J 2008; 72: 106 - 108)

Objective The purpose of this study was to evaluate the rate of progression of mild and moderate aortic stenosis in patients aged 80 years and older. Methods In all, 41 patients with mild and moderate aortic stenosis were included and divided into two groups by age: 19 patients aged 80 years and older (mean 84 ± 4 years), and 21 patients younger than 80 years (mean 66 ± 6 years). Results The rate of degression of aortic valve area was more rapid in the 80 years and older age group than that in the younger than 80 years age group (−0.05 ± 0.06 and −0.10 ± 0.08 cm2/y, P = .014). Univariate and multivariate analysis of the rate of degression of aortic valve area were performed, and age was the only independent predictor of the rate of degression of aortic valve area. Conclusion Progression of mild and moderate aortic stenosis in patients aged 80 years and older was more rapid than that in those aged younger than 80 years.

We report a case with perforated mitral valve aneurysm in the anterior leaflet associated with lateral scallop prolapse. Transesophageal echocardiography (TEE) revealed an apparent prolapse of the lateral segment of the mitral leaflet with mitral regurgitation and a perforated aneurysm in the anterior leaflet of the mitral valve. In this patient, an eccentric regurgitant jet toward the anterior leaflet due to lateral scallop prolapse caused structural weakening and then the aneurysm in the anterior leaflet.

A giant ball-thrombus was found in the left atrium in 78 year-old female with non-valvular lone atrial fibrillation. Intraoperative echocardiography revealed the thrombus was floating freely inside the left atrium. The thrombus was sometimes scarcely entrapped into the mitral annulus and bounded back toward the left atrium. The removed thrombus was 45×35×20mm in size.