Frenemies within: An Endocarditis Case in Behçet’s Disease

A 57-year female patient diagnosed with Behçet’s disease, on azathioprine, was noticed to have at a routine examination antinuclear and antiphospholipid antibodies. An overlapping lupus-like syndrome was diagnosed; hydroxychloroquine and aspirin were added. Three years later, the patient presented with dyspnea and sweating, with no fever. A cardiac bruit was noted; a giant vegetation was detected by echocardiography. Laboratory revealed severe thrombocytopenia, antiphospholipid antibodies and low complement. Blood cultures were positive for Abiotrophia defectiva serology and also revealed a chronic Coxiella burnetii infection. Antibiotic therapy, low-dose anticoagulation and control of the underlying disease mildly improved the platelet count, which fully recovered only after cardiac valve replacement. However, the Behçet’s disease, initially quiescent, flared after the therapy of infections. We discuss potential links between Behçet’s disease and the occurrence of antinuclear and antiphospholipid antibodies and Coxiella endocarditis in this setting. We also highlight the differences between the endocarditis in Behçet’s disease, antiphospholipid syndrome, Coxiella burnetii and Abiotrophia defectiva infection, respectively. Intracellular infections may modify the presentation of autoimmune diseases. Confounding clinical features of Coxiella persistent infection and non-bacterial thrombotic endocarditis in Behçet’s disease warrant further insight.

Left atrial functional assessment and mortality in patients with severe aortic stenosis with sinus rhythm

Background Aortic valve stenosis (AS) is the most common primary valvular heart disease leading to surgical or percutaneous aortic valve replacement (AVR) in Europe and its prevalence keeps growing. While other risk factors in severe AS are well documented, little is known about the prognostic value of left atrial (LA) function in AS. Our aim is to clarify the relationship between LA function measured at severe AS diagnosis (evaluated by means of volumetric assessment) and all-cause mortality during follow-up. Methods We retrospectively evaluated patients diagnosed with severe AS for the first time at our echocardiography laboratory. We evaluated LA reservoir, conduit and pump function by measuring LA volumes at different timings of cardiac cycle. Treatment strategy was decided according to heart team consensus and patient decision. We divided patients into groups according to terciles of LA reservoir, conduit and pump function. Primary outcome was defined by the occurrence of all-cause mortality during follow-up. Results A total of 408 patients were included in the analysis, with a median follow-up time of 45 months (interquartile range 54 months). 57.9% of patients underwent AVR and 44.9% of patients registered the primary outcome during follow-up. Left atrial emptying fraction (LAEF) was the best LA functional parameter and the best overall parameter in discriminating primary outcome (AUC 0.845, 95%CI 0.81–0.88, P < 0.001). After adjustment for clinical, demographic and echocardiographic variables, cumulative survival of patients with LAEF < 37% and LAEF 37 to 53% relative to patients with LAEF ≥54% remained significantly lower (HR 13.91, 95%CI 6.20–31.19, P < 0.001 and HR 3.40, 95%CI 1.57–7.37, P = 0.002, respectively). After adjustment for AVR, excess risk of LAEF < 37% and LAEF 37 to 53% relative to LAEF ≥54% remained significant (HR 11.71, 95%CI 5.20–26.40, P < 0.001 and HR 3.59, 95%CI 1.65–7.78, P = 0.001, respectively). Conclusions In patients with a first diagnosis of severe AS, LA function, evaluated by means of volumetric assessment, is an independent predictor of all-cause mortality and a more potent predictor of death compared to classical severity parameters. These data can be useful to identify high-risk patients who might benefit of AVR.

Tissue Doppler velocities for ruling out rejection in heart transplant recipients in the daily routine of the echocardiography laboratory: a feasibility study

Funding Acknowledgements Type of funding sources: None. Background The sum of systolic and early diastolic myocardial velocities at lateral mitral annulus, evaluated by tissue Doppler (in absolute values, s’+e’) in the apical four chambers view is a parameter whose value for ruling out acute cellular rejection (ACR) in heart transplant (HTx) recipients has been acknowledged by the European and Brazilian recommendations for the use of imaging in HTx. A recent study has shown its independent association with ACR in the context of classical and myocardial deformation variables, with a negative predictive value (NPV) of 98% for treatment requiring ACR (TR-ACR, grade ≥ 2R) for a cut-off point of ≥23 cm/s. This work was performed under experimental conditions (only one expert echocardiographer, only one high-end equipment). Purpose Our objective was to study the diagnostic utility of this parameter for ruling out rejection in HTx recipients in the daily routine of the echocardiography laboratory. Methods From October 2017 to May 2020, serial echocardiograms were performed to 33 consecutive HTx recipients, in the 3 hours after the routine surveillance endomyocardial biopsies (EMB), in the first year after HTx. Three sonographers, seven cardiology residents and six expert echocardiographers participated in the acquisition and interpretation of the images, with seven different echocardiographic machines in only one centre. We analysed the association of s’+e’ with the presence of ACR, and the NPV of s’+e’ ≥23 cm/s for ruling out TR-ACR was investigated. Results A total of 176 pairs of EMB and echocardiogram were obtained. The value of s’+e’ was significantly lower with higher severity of rejection: 25.6 ± 5.5 cm/s, 23.8 ± 5.1 cm/s and 21.6 ± 3.5 cm/s for ACR grade 0R (n = 91, 52%), 1R (n = 67, 38%) and ≥2R (n = 18, 10%), respectively, p = 0.005. It was also lower when comparing studies with (≥1R) and without rejection (23.4 ± 4.9 cm/s versus 25.6 ± 5.5 cm/s, p = 0.005), or TR-ACR versus the rest of series (21.6 ± 3.5 cm/s versus 24.8 ± 5.3 cm/s, p = 0.01). The area under curve for the detection of TR-ACR was 0.67 (CI95% 0.56-0.78), p = 0.02. A cut-off point of ≥23 cm/s, present in 57% of the studies, showed a NPV of 95% for TR-ACR. Conclusion Lateral mitral annulus velocities showed an excellent NPV of 95% for TR-ACR detection in HTx recipients when evaluated in the daily routine of an echocardiography laboratory with a wide variety of operators and echocardiographic equipment. This finding could be useful for reducing the number of EMB in selected cases.

Left atrial functional assessment and mortality in patients with severe aortic stenosis with sinus rhythm

Background: Aortic valve stenosis (AS) is the most common primary valvular heart disease leading to surgical or percutaneous aortic valve replacement (AVR) in Europe and its prevalence keeps growing. While other risk factors in severe AS are well documented, little is known about the prognostic value of left atrial (LA) function in AS. Our aim is to clarify the relationship between LA function measured at severe AS diagnosis (evaluated by means of volumetric assessment) and all-cause mortality during follow-up. Methods: We retrospectively evaluated patients diagnosed with severe AS for the first time at our echocardiography laboratory. We evaluated LA reservoir, conduit and pump function by measuring LA volumes at different timings of cardiac cycle. Treatment strategy was decided according to heart team consensus and patient decision. We divided patients into groups according to terciles of LA reservoir, conduit and pump function. Primary outcome was defined by the occurrence of all-cause mortality during follow-up. Results: A total of 408 patients were included in the analysis, with a median follow-up time of 45 months (interquartile range 54 months). 57.9% of patients underwent AVR and 44.9% of patients registered the primary outcome during follow-up. Left atrial emptying fraction (LAEF) was the best LA functional parameter and the best overall parameter in discriminating primary outcome (AUC 0.845, 95%CI 0.81-0.88, P <0.001). After adjustment for clinical, demographic and echocardiographic variables, cumulative survival of patients with LAEF <37% and LAEF 37 to 53% relative to patients with LAEF ≥54% remained significantly lower (HR 13.91, 95%CI 6.20-31.19, P <0.001 and HR 3.40, 95%CI 1.57-7.37, P =0.002, respectively). After adjustment for AVR, excess risk of LAEF <37% and LAEF 37 to 53% relative to LAEF ≥54% remained significant (HR 11.71, 95%CI 5.20-26.40, P <0.001 and HR 3.59, 95%CI 1.65-7.78, P =0.001, respectively). Conclusions: In patients with a first diagnosis of severe AS, LA function, evaluated by means of volumetric assessment, is an independent predictor of all-cause mortality and a more potent predictor of death compared to classical severity parameters. These data can be useful to identify high-risk patients who might benefit of AVR.

Quality assurance, reporting, and normal ranges

As discussed in this chapter, any echocardiography laboratory should have a quality assurance (QA) system in place. This allows for continuous monitoring and evaluation of the quality of echocardiographic studies and of the unit’s functioning. The chapter also gives an overview of accreditation and how to adopt a standard approach to reporting. It then gives the normal ranges for all aspects of the heart that are examined in echocardiography.

An echocardiographic study of Rheumatic Heart Disease amongst children in a Tertiary Centre

Background: Rheumatic Heart Disease (RHD) is a major form of acquired heart disease amongst children in developing countries, where it continues to be a cause of childhood morbidity and mortality. Objective: To describe the prevalence and spectrum of valvular affectation in children with RHD from the echocardiography laboratory. Methods: The records of echocardiographic scans conducted over a 10-year period in a tertiary health facility were reviewed. Children with RHD, diagnosed using standard criteria were identified. The pattern and severity of valvular involvement and other associated cardiac abnormalities were recorded. Results: Forty-one of the 2742 (1.5%) children who had echocardiography had RHD. Their mean age was 10.9±3.1 years; 28 (68.3%) were aged ≥10 years while 21 (51.2%) were males. Mitral valve was the predominantly affected valve in 40 (97.6%) children and mitral regurgitation was the commonest valvular abnormality in 19 (46.3%) cases. Nineteen (46.3%) had severe valvular damage, 5 (12.2%) had pericardial effusion while 7 (17.1%) died. Only 2 (4.9%) children had valvular repair surgery. Conclusion: Although the prevalence of RHD in this study is low, it is noteworthy that RHD still affects Nigerian children. The poor access to surgery emphasizes the need to strengthen both primary and secondary prevention of RHD while enhancing facilities for surgical intervention.