Echocardiography for Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a genetic cardiomyopathy. The prevalence of phenotypic expression, in the absence of another systemic or cardiac disease causing increased left ventricular (LV) wall thickness, is estimated to be 1:500. The frequency of clinical presentation is far less, highlighting the need for a non-invasive diagnostic imaging tool. Echocardiography is readily available and allows for structural characterization and hemodynamic assessment of the hypertrophic heart and to screen patients at-risk for HCM, such as first degree relatives of affected individuals, and differentiate HCM from the athletic heart. Echocardiography can also be used to assess for anatomic abnormalities of the mitral valve apparatus that may exacerbate LV outflow track obstruction and to further risk stratify patients during exercise. Finally, echocardiography plays an integral role in guiding alcohol septal ablation procedures.     

评价致心律失常性右室心肌病右室功能超声指标与磁共振的相关性

目的用目前公认的5个超声指标评价致心律失常性右室心肌病（ARVC）患者的右室功能,研究其与磁共振（MRI）结果的相关性。方法对11例ARVC患者行超声及MRI检查,测量右室功能指标并行相关分析。本研究除了运用传统的心尖四腔心法测量右室面积改变分数（RVFAC 4C）外,增加了胸骨旁右室三腔心切面法测量右室面积改变分数（RVFAC RV 3C）。结果 5个指标中胸骨旁短轴RVFAC RV 3C、三尖瓣环收缩峰值速度、三尖瓣环收缩位移与MRI结果相关,r值分别为0.72、0.65、0.67。结论胸骨旁短轴RVFAC RV3C是评价ARVC患者右室功能的重要指标并且其与MRI测量的结果具有高度的相关性。     

Usefulness of right atrial volume index in predicting outcome in chronic systolic heart failure

BackgroundRight ventricular (RV) dysfunction is associated with poor prognosis in patients with heart failure (HF). Echocardiographic assessment of RV systolic function is challenging. The ability to visualize the right atrium (RA) allows a quantitative, highly reproducible assessment of RA volume.ObjectiveThe aim is to study the relationship between the right atrial volume index (RAVI) and prognosis in patients with chronic systolic HF.Methods120 patients with chronic systolic HF and left ventricular ejection fraction (LVEF) <40% were enrolled. The RA volume was calculated by Simpson’s method using single-plane RA area and indexed to body surface area (RAVI). RV systolic assessment was done using the RV fractional area change (RVFAC), and peak systolic velocity (Sa_tri) using tissue Doppler imaging at the tricuspid annulus. The primary endpoint was death, urgent transplantation, or acute HF episode requiring hospital admission during a follow-up of 1 year.ResultsFollow up was complete for 117 of 120 patients. Fifty-two patients reached the primary endpoint. The mean RAVI was higher in patients with adverse events (45.5 ± 15 ml/m² versus 25.2 ± 11 ml/m², p < 0.001), and increased with worsening LVEF, RVFAC, Sa_tri (Spearman’s r = −0.46, r = −0.45, r = −0.59, p < 0.001 for all). RAVI was not correlated with estimates of RV diastolic dysfunction. The cut-off threshold for RAVI to predict the primary endpoint using receiver-operating characteristic curve was 29 ml/m² (area under the curve was 0.89%, 95% confidence interval: 0.82–0.95) with a sensitivity of 92%, and a specificity of 75%. NYHA > 2 (OR = 2.1, p < 0.01), and RAVI (OR = 1.6, p < 0.05) were found to be independent predictors of adverse outcome.ConclusionIn patients with chronic systolic HF, RAVI is an independent predictor of adverse outcome with a threshold value of 29 ml/m².     

Echocardiographic evolution of pulmonary artery pressure after acute pulmonary embolism. Results from IPER registry

Aims

The aim of the study is to describe the course of the echocardiographically measured pulmonary artery systolic pressure (PAsP) in a series of patients included in the Italian Pulmonary Embolism Registry (IPER).

Methods

Patients with confirmed PE received an echo-Doppler evaluation within 24 hours from hospital admission and after one year. Pulmonary hypertension (PH) was considered “likely” , “possible” or “unlikely” with a right ventricular-right atrial (RV-RA) pressure gradient > 45 mm Hg, between 32 and 45 mm Hg and ≤ 31 mm Hg and no additional echocardiographic variables suggestive of PH, respectively.

Results

We studied 286 patients (169 females and 117 males, mean age 67 ± 15; mean follow-up 387 ± 45 days): 240 had a baseline tricuspid regurgitation (TR) and a RV-RA gradient of variable degree. PH was considered likely, unlikely and possible in 97, 93 and 50 patients respectively. At FU echocardiography, 6 patients (2.1%) had a likely PH and all of them were part of the group of 97 patients with a baseline likely PH; 24 patients (8.4%) had a possible PH, and 67% of them had an initial likely PH. No patients with a baseline unlikely PH or without TR developed a follow-up PH (both likely or possible). The probability to show a likely PH at FU echocardiography for patients with a baseline RV-RA gradient > 45 mm Hg was 6.2%, while the probability not to have a likely PH for patients with a baseline RV-RA gradient ≤ 45 mm Hg was 100%.

Conclusion

In our study population of patients with acute PE, we observed that those presenting with a baseline echocardiographic RV-RA pressure gradient ≤ 45 mm Hg were completely free from a likely PH after 1-year.     

Tetralogy of Fallot with restrictive ventricular septal defect by accessory tricuspid leaflet tissue

In tetralogy of Fallot septal defect is usually large because of malalignment of outlet septum, restrictive defect has been reported rarely. We present a case of tetralogy of Fallot with accessory tricuspid leaflet tissue restricting ventricular septal defect. The report includes echocardiographic and catheter images of this rare presentation of tetralogy of Fallot.     

Left atrial spindle cell sarcoma - Case report

Primary spindle cell sarcoma of the left atrium is an extremely rare tumour. Surgical excision is the mainstay of treatment since it responds poorly to chemotherapy or radiotherapy. In spite of all the treatment, the prognosis remains poor due to inadvertent delay in diagnosis, few therapeutic options and propensity to metastasize. We present a 47-year-old male who underwent a surgical excision of a left atrial mass in February 2010. It was proved to be a high-grade spindle cell sarcoma on histopathology. He presented again in October 2010 with recurrence of the tumour for which he was re-operated. However, the tumour recurred again within one month, to which the patient succumbed.     

A complete right bundle-branch block masking Brugada syndrome

A 69-year-old man without structural heart disease was admitted for syncope. His electrocardiogram displayed complete right bundle branch (CRBBB). A coved type ST elevation was observed with transient normalization of CRBBB giving rise to a normal QRS. These findings suggest that Brugada syndrome can be masked by CRBBB.     

Percutaneous mechanical support for the failing right heart

Right ventricular (RV) failure is an increasingly common clinical problem that may require mechanical support. In contrast to severe left ventricular failure, RV failure is typically more reversible. Therefore, application of shorter-term percutaneous support devices is potentially attractive. Current innovations promise greater availability of such percutaneous RV support devices. This article considers the available mechanical approaches to provide hemodynamic support to treat profound RV failure in the common clinical scenarios in which percutaneous mechanical RV support may be most beneficial.