Shing Ching

A woman in her 50s with known MYBPC3‐associated obstructive hypertrophic cardiomyopathy was evaluated for septal reduction therapy. The electrocardiogram, echocardiogram, and cardiac magnetic resonance found several features inconsistent with the diagnosis. Targeted screening confirmed adult‐onset Fabry disease. Enzyme replacement therapy was started.

A 78‐year‐old man was evaluated for a non‐sustained ventricular tachycardia episode detected in a dual‐chamber pacemaker (Boston Scientific Essentio MRI EL L131) implanted for sick sinus syndrome. Blanking of a premature ventricular beat caused delivery of ventricular pacing in the vulnerable period triggering non‐sustained ventricular tachycardia. Review of literature found more than half of reported cases involved Boston devices. The commonalities of such phenomenon are summarized and reprogramming strategies presented.

Background: While left-bundle-branch-block-related contraction patterns as well as echocardiography-derived strain are variably associated with the volumetric response to cardiac resynchronization therapy (CRT), the role of CMR-derived strain parameters is unexplored. Methods: A total of 50 patients receiving CRT implantation were retrospectively analyzed, all of whom had undergone CMR imaging within one year before, and echocardiography within 6 months before and 6–12 months after CRT implantation. We assessed CMR-derived morphological and functional parameters with regard to the echocardiographic response, defined as a reduction in the left ventricular end-systolic volume of ≥15%. Results: Among the standard CMR parameters, the indexed right ventricular volumes in end-diastole (RVEDVi) (74.5 ± 19.5 vs. 94.8 ± 30.2 mL/m², p = 0.006) and end-systole (RVESVi) (43.2 ± 13.3 vs. 61.6 ± 28.8 mL/m², p = 0.003), as well as the left atrial (LA) area (24.8 ± 3.5 vs. 30.4 ± 9.5 cm², p = 0.020), differed significantly between CRT responders and non-responders. In strain analysis, CRT responders showed a significantly better LA global longitudinal strain (GLS) (25.1 ± 10.4 vs. 15.3 ± 10.5, p = 0.002), LA global circumferential strain (GCS) (27.9 ± 14.7 vs. 17.1 ± 13.1%, p = 0.012), RV GLS (−25.0 ± 6.5 vs. −18.9 ± 7.6%, p = 0.004) and RV free wall strain (−31.1 ± 7.9 vs. −24.9 ± 9.5, p = 0.017). Conclusions: CMR-derived peak septal circumferential strain and RVEDVi correlated with the echocardiographic volumetric response to CRT at 6–12 months.

Background: While left-bundle-branch-block-related contraction patterns as well as echocardiography derived strain are variably associated with volumetric response to cardiac resynchronization therapy (CRT), the role of CMR-derived strain parameters is unexplored. Methods: 50 patients receiving CRT implantation were retrospectively analysed, all of which had undergone CMR imaging within one year before, and echocardiography within 6 months before and 6-12 months after CRT implantation. We assessed CMR-derived morphological and functional parameters with regard to echocardiographic response, defined as reduction of left ventricular end-systolic volume ≥15%. Results: Among standard CMR parameters, indexed right ventricular volumes in end-diastole (RVEDVi) (74.5 ±19.5 vs. 94.8 ±30.2 ml/m², P=.006) and end-systole (RVESVi) (43.2 ±13.3 vs. 61.6 ±28.8 ml/m², P=.003) as well as left atrial (LA) area (24.8 ±3.5 vs. 30.4 ±9.5 cm², P=.020) differed significantly between CRT responders and non-responders. In strain analysis, CRT responders showed significantly better LA global longitudinal strain (GLS) (25.1 ±10.4 vs. 15.3 ±10.5, P=.002), LA global circumferential strain (GCS) (27.9 ±14.7 vs. 17.1 ±13.1%, P=.012), RV GLS (-25.0 ±6.5 vs. -18.9 ±7.6%, P=.004) and RV free wall strain (-31.1 ±7.9 vs. -24.9 ±9.5, P=.017). Conclusions: CMR-derived peak septal circumferential strain and RVEDVi correlated with echocardiographic volumetric response to CRT at 6-12 months.

A 53-year-old woman was evaluated for left ventricular hypertrophy. Transthoracic echocardiography showed a mobile linear density in the ascending aorta and eccentric aortic insufficiency, raising the concern of aortic dissection. Transesophageal echocardiography and cardiac computed tomography (CT) found complex bands of tissue extending from the commissure to the aortic wall, confirming aortic valve tendon. It is an extremely rare malformation of aortic valve associated with aortic regurgitation that mimics aortic dissection. Although preoperative diagnosis has been challenging, three-dimensional echocardiography and CT reformatting aided the visualization of the spatially complex structure.

C ardiac involvement in eosinophilic granulomatosis with polyangiitis (EPGA) is increasingly recognized, but it is unusual to be the initial presentation. We report a case of vasculitic myocardial infarction (MI) and cardiogenic shock in an elderly man caused by EPGA. A 73-year-old man presented with acute chest pain radiating to back for two days. He has a history of asthma and allergic rhinitis for more than ten years well controlled by inhaled corticosteroid. The electrocardiogram showed sinus rhythm, right bundle-branch block and anterolateral ST-segment depression. He had elevated troponin T of 548 ng/L (reference: < 14 ng/L), creatine kinase of 454 IU/L (reference: 39-308 IU/L), white blood cell count of 13.8 × 10 9 /L (reference: 3.7-9.3 × 10 9 /L) and eosinophil count of 12.4 × 10 9 /L (reference: 0.0-0.6 × 10 9 / L). Antineutrophil cytoplasmic, anti-extractable nuclear antigen, and anti-nuclear antibodies were negative. The evolution of laboratory parameters is shown (Figure 1). Transthoracic echocardiogram showed reduced left ventricular ejection fraction of 40%, anterolateral hypokinesia and 1 cm pericardial effusion near left ventricular apex. Aspirin, clopidogrel and low molecular weight he

Sinus of Valsalva pseudoaneurysm (SVpA) is a rare cause of right ventricular inflow obstruction. We report such a case presented with atrial flutter and cardiogenic shock caused by tricuspid valve obstruction by a narrow-necked right SVpA complicating aortic valve infective endocarditis demonstrated on transesophageal echocardiography and cardiac computed tomography. Despite restoration of sinus rhythm, the patient ran a fatal course due to aneurysmal rupture. We show the value of transesophageal echocardiography in evaluating unstable patients with cardiogenic shock, and the need of urgent surgery in selected patients to avoid a dismal outcome.