Sinus venosus atrial septal defect and partial anomalous pulmonary venous connection in a patient with dextrocardia.

An 85-year-old woman presenting with symptomatic pulmonary hypertension (PH) with a history of dextrocardia was referred to our facility for treatment of heart failure. Significant shunting was detected by measuring oxygen saturation during right heart catheterisation (RHC). CT with contrast revealed sinus venosus atrial septal defect (SVASD) and partial anomalous pulmonary venous connection (PAPVC), in which the left upper and middle lobar pulmonary veins returned to the superior vena cava and right atrium. Despite medical treatment, the patient died, and an autopsy was performed. SVASD and PAPVC are rare congenital anomalies. RHC with measurement of oxygen saturation and CT with contrast should be considered in patients with unexplained right atrial and ventricular enlargement or suspected PH.

Successful catheter ablation of persistent atrial fibrillation is associated with improvement in functional tricuspid regurgitation and right heart reverse remodeling.

Atrial fibrillation (AF) is a common disease that changes cardiac morphology, especially in the left atrium (LA). It is now known that certain categories of functional mitral regurgitation (MR) are associated with AF; however, the influence of AF on right cardiac morphology is not fully understood. Our aim in this study was to investigate the association between AF and right cardiac morphology. This was a retrospective cohort study of 86 patients with persistent AF without other cardiac disease who underwent catheter ablation (CA). Seventy-one patients had sustained sinus rhythm (SR) (SR Group) and 15 patients had sustained AF (AF Group) during the study period. We compared the changes in the right cardiac dimensions and tricuspid regurgitation (TR) between the groups 12 months after CA. Patients' baseline echocardiographic assessments revealed that the LA volume index was significantly smaller in the SR group than in the AF group (46.8 ± 11.9 ml/m2 vs 59.3 ± 12.8 ml/m2, respectively; p < 0.01). Comparing baseline data with the 12-month follow-up data, in the SR group, right atrial area (RAA, cm2), tricuspid annular diameter (mm), and tricuspid regurgitant jet area (cm2) were significantly decreased compared with the AF group (19.5 ± 4.5-15.5 ± 3.6 vs 20.7 ± 3.6-19.7 ± 2.3; 30.5 ± 4.9-26.4 ± 3.9 vs 28.7 ± 4.0-28.8 ± 3.1; and 1.4 [interquartile range (IQR) 0.7-2.6]-0.6 [IQR 0.2-1.2] vs 1.2 [IQR 1.1-1.5]-0.9 [IQR 0.4-1.3], respectively). On multivariate analysis, change in RAA correlated with the reduction in tricuspid regurgitant jet area (R = 0.51, p < 0.001). In conclusion, successful CA for persistent AF led to right heart reverse remodeling, and our findings suggested that persistent AF was associated with RAA dilatation and TR.

H2FPEF Score for the Prediction of Exercise Intolerance and Abnormal Hemodynamics in Japanese　- Evaluation by Exercise Stress Echocardiography Combined With Cardiopulmonary Exercise Testing.

BACKGROUND: Both the H2FPEF-score and nomogram-score, which consist of simple clinical parameters, can assist in diagnosing "early" heart failure with preserved ejection fraction (HFpEF) and only exertional dyspnea, but their these usefulness in Japanese remains unclear. We sought to investigate the correlation between these scores and exercise response, including the peak oxygen uptake (V̇O2), the pulmonary artery systolic pressure (PASP), the ratio of early diastolic transmitral flow velocity to early diastolic mitral annular velocity (E/e') and stroke volume (SV) using exercise stress echocardiography (ESE) combined with cardiopulmonary exercise testing (CPET).Methods and Results:In this single-center, retrospective cross-sectional study the H2FPEF-score and nomogram-score were calculated in a total of 139 patients who underwent ESE combined with CPET. The scores correlated with peak V̇O2(r=-0.48, r=-0.44), PASP (r=0.23, r=0.29) and SV (r=-0.32, r=-0.19) at peak exercise. The nomogram-score correlated with E/e' (r=0.24). The prevalence of exercise intolerance (percent predicted peak V̇O2<75% and <50%) increased as the H2FPEF-score increased and reached 88.9% and 22.2% among the patients with high H2FPEF-score (6-9 points). CONCLUSIONS: The H2FPEF-score may be useful as the initial step to diagnosing 'early' HFpEF. The nomogram-score may be more useful in Japanese because of its more universal association with exercise response than the H2FPEF-score.

Early mitral inflow velocity to left ventricular global strain ratio predicts limited exercise capacity.

BACKGROUND: Estimation of left ventricular filling pressure (LVFP) is the essential part of the echocardiographic workup for exercise intolerance. The ratio of the mitral early filling velocity to the left ventricular global longitudinal strain (E/LVGLS) has emerged as a novel index of LVFP. This study aimed to investigate the relationship between E/LVGLS and exercise capacity. METHODS AND RESULTS: We retrospectively reviewed 90 patients with exertional dyspnea who underwent echocardiography and cardiopulmonary exercise test. Patients were classified into three groups according to their percent-predicted maximal oxygen consumption (ppVo2 ) (G â‰§ 75, ppVo2  > 75%, n = 20; G 50-75, ppVo2 75-50%, n = 57; G < 50, ppVo2  < 50%, n = 13). Measurements were (a) the relationship between ppVo2 and E/LVGLS and (b) the efficiency of integrated diastolic assessment using E/LVGLS, left ventricular volume, mitral annular early diastolic velocity (E'), and tricuspid regurgitation to identify the patient with impaired exercise capacity. Univariate linear regression analysis demonstrated that E/LVGLS had significant correlation with ppVo2 (ρ = 0.52, P < 0.001). The area under the ROC of E/LVGLS for ppVo2  < 50% was 0.86 (95%CI 0.75-0.97). The DeLong test showed that E/LVGLS was efficient to detect ppVo2  < 50% than E/E' (P = 0.007). When used in the integrated assessment of LVFP as the alternative for E/E', E/LVGLS improved risk classification for impaired exercise capacity. CONCLUSION: E/LVGLS may be a more efficient index than E/E' to identify exercise impairment as a single index and as a part of the integrated diastolic assessment.

Exercise-Stress Echocardiography and Effort Intolerance in Asymptomatic/Minimally Symptomatic Patients With Degenerative Mitral Regurgitation Combined Invasive-Noninvasive Hemodynamic Monitoring.

Background Effort intolerance, measured objectively by reduced peak oxygen consumption (Vo2), has been considered as an important prognosticator in degenerative mitral regurgitation (MR). However, its mechanism is unknown. Methods and Results In 25 asymptomatic/minimally symptomatic patients with grade III+ or greater degenerative MR undergoing semisupine invasive exercise testing, Doppler estimates and invasive measurement of systolic (SPAP) and mean pulmonary artery pressure (MPAP) and cardiac output (CO) were simultaneously obtained. Echocardiographic estimates of SPAP, MPAP, and CO correlated well with invasive measurement at peak exercise (bias, SPAP, -0.7±7.4 mm Hg; MPAP, 1.2±6.3 mm Hg; CO, 0.2±2.5 L/min). Heart rate reserve (ß, 3.997; 95% CI, 2.704-5.290 per 41.5% increase; P<0.001), MPAP/CO slope (ß, -3.846; 95% CI, -5.926 to -1.766 per 4.85 mm Hg/L per minute increase; P=0.001), and tricuspid annular plane systolic excursion/SPAP slope (ß, 4.094; 95% CI, 2.252-5.936 per 0.22 mm/mm Hg increase; P=0.003) were associated with peak Vo2 even after adjustment for increase in MR vena contracta during exercise and peak SPAP. The MPAP/CO slope of 4.13 had a sensitivity and a specificity for predicting effort intolerance (%predicted peak Vo2 <70%) of 57% and 91%, respectively, whereas the tricuspid annular plane systolic excursion/SPAP slope of 0.25 had a respective sensitivity and specificity of 86% and 82%. Conclusions The agreement between echocardiographic and invasive measures of pulmonary artery pressures and CO during exercise is acceptable. In patients with degenerative MR, effort intolerance is predominantly because of chronotropic incompetence, abnormal pulmonary vascular reserve, and limited right ventricular contractile reserve and not simply because of exercise-induced MR or pulmonary hypertension.

Mitral systolic velocity at peak exercise predicts impaired exercise capacity in patients with heart failure with preserved ejection fraction.

BACKGROUND: Nearly half of patients with heart failure have normal left ventricular ejection fraction (LVEF), but their prognosis is no better than those with reduced LVEF. Although peak oxygen consumption (VO2 ) is an independent predictor of mortality in heart failure, it is unclear how cardiac function during exercise contributes to peak VO2 . Therefore, we explored the useful parameters measured by exercise stress echocardiography to predict peak VO2 in patients with heart failure with preserved LVEF (HFpEF). METHODS AND RESULTS: We assessed 80 patients being investigated for effort intolerance or dyspnea and finally analyzed 50 patients who satisfied the HFpEF criteria. Mean peak VO2 was 16.4±2.8 mL/kg/min. Twenty-three patients (46.0%) achieved a peak VO2 <16.0 mL/kg/min (Weber class C or D). There was a significant relationship between mitral systolic velocity (S') and cardiac output (CO) at rest (R=.55, P<.0001) and peak exercise (R=.64, P<.0001). The absolute increase in S' from rest to peak exercise also correlated with the absolute increase in CO (R=.32, P=.02). Multivariate logistic regression analysis showed that S' at peak exercise independently predicted peak VO2 . Receiver-operator characteristic curve analysis identified that an S' at peak exercise of ≤8.13 cm/s predicted a peak VO2 <16.0 mL/kg/min (sensitivity 95.7%, specificity 44.4%, area under curve 0.70, 95% confidence interval 0.55-0.84, P=.004). CONCLUSIONS: Mitral systolic velocity at peak exercise accurately reflects peak VO2 and may facilitate stratification of risk in patients with HFpEF.

Efficacy of pulmonary vein isolation on left atrial function in paroxysmal and persistent atrial fibrillation and the dependency on its baseline function.

BACKGROUND: The effects of pulmonary vein (PV) isolation in atrial fibrillation (AF) on left atrial (LA) function or PV flow have not been well documented. METHODS: We examined the LA function and PV flow before and 3-6 months after PV isolation in 67 AF patients (34 paroxysmal [PAF] and 33 persistent [CAF]) using transesophageal echocardiography. RESULTS: AF recurred in 6/34 patients with PAF and in 6/33 patients with CAF 6 months after PV isolation. A larger LA dimension, a lower systolic PV flow velocity, and a lower ratio of systolic to diastolic PV flow velocity were related to a higher incidence of AF recurrence. The increment of left atrial appendage (LAA) flow velocity (55% vs. 22%) and systolic PV flow velocity (57% vs. 20%) after PV isolation tended to be greater in CAF than in PAF. The changes in LAA flow velocity had reverse correlations with the baseline values before PV isolation (PAF: r = -0.73, CAF: r = -0.58). The changes in mitral flow velocity during atrial contraction in PAF had reverse correlations with the baseline values before PV isolation (r = -0.84). The changes in systolic and diastolic PV flow velocity of PAF had reverse correlations with the baseline values before PV isolation (r = -0.56, r = -0.66). CONCLUSION: The baseline LA function may affect AF recurrence as well as the improvement of LA function, and the benefit of successful PV isolation might be greater in CAF than in PAF.

An extremely large coronary aneurysm associated with a quadricuspid aortic valve in an adult patient.

A 68-year-old woman exhibited an increasingly protruding mass on the left heart border on chest X-ray. Transthoracic echocardiography revealed an echo-free mass in the anterior pericardial space. Transesophageal echocardiography revealed blood flow from the proximal left anterior descending coronary into a large coronary artery aneurysm measuring 61 mm × 51 mm in diameter and a quadricuspid aortic valve with a small cusp between the left and right coronary cusps. Coronary angiography demonstrated the presence of a coronary aneurysm connected to the proximal left coronary anterior descending artery. A giant coronary artery aneurysm and pulmonary artery fistulas extending from the left and right coronary arteries were confirmed by surgeons and successfully treated with surgery.