A 68-year-old man presented with a six-month history of painless dysphagia, malnutrition, anorexia and vocal hoarseness. Signs of severe mitral regurgitation and preserved left atrial dimensions were discovered on transthoracic echocardiography. However, electrocardiography and chest radiography were strongly suggestive of left atrial enlargement. Further investigations confirmed extrinsic compression of the oesophagus, which caused the dysphagia. Computed tomography of the throrax revealed a giant left atrium that was not appreciated on echocardiography. Hoarseness was found to be caused by right recurrent laryngeal nerve palsy. Ortner’s syndrome, which describes the occurrence of vocal hoarseness due to a cardiopulmonary disease that results in the compression of the left recurrent laryngeal nerve, is usually associated with severe mitral stenosis. Herein, we report an unusual case of Ortner’s syndrome caused by a giant left atrium, which resulted from severe mitral regurgitation, causing extrinsic oesophageal compression and right recurrent laryngeal nerve palsy. Physicians should remain cognisant of cardiovascular disorders as uncommon causes of painless dysphagia or vocal hoarseness.
Aged ; Cardiovascular Diseases ; diagnosis ; Deglutition Disorders ; diagnosis ; Dysphonia ; diagnosis ; Electrocardiography ; Hoarseness ; diagnosis ; Humans ; Male ; Mitral Valve Insufficiency ; diagnosis ; Radiography, Thoracic ; Syndrome ; Tomography, X-Ray Computed ; Treatment Outcome ; Vocal Cord Paralysis ; diagnosis

The use of implantable cardioverter defibrillators (ICDs) in young women has been increasing in recent years owing to greater awareness about inherited cardiac conditions that increase the risk of sudden death. Traditional placement of ICDs in the infraclavicular region among young women often leads to visible scars, a constant prominence that causes irritation from purse or bra straps and can result in body image concerns and device-related emotional distress. In this case series, two women with long QT syndrome required placement of ICDs for prevention of sudden cardiac death. Submammary placement of ICDs was performed in collaboration with electrophysiologists. We describe our local experience and technique in submammary placement of ICDs as well as the challenges faced.
Death, Sudden, Cardiac/prevention & control* ; Defibrillators, Implantable ; Female ; Heart Diseases ; Humans ; Prosthesis Implantation/methods* ; Singapore

INTRODUCTION: Risk stratification in dilated cardiomyopathy (DCM) is imprecise, relying largely on echocardiographic left ventricular ejection fraction (LVEF) and severity of heart failure symptoms. Adverse cardiovascular events are increased by the presence of myocardial scarring. Late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) imaging is the gold standard for identifying myocardial scars. We examined the association between LGE on CMR imaging and adverse clinical outcomes during long-term follow-up of Asian patients with DCM. METHODS: Consecutive patients with DCM undergoing CMR imaging at a single Asian academic medical centre between 2005 and 2015 were recruited. Clinical outcomes were tracked using comprehensive electronic medical records and mortality was determined by cross-linkages with national registries. Presence and distribution of LGE on CMR imaging were determined by investigators blinded to patient outcomes. Primary endpoint was a composite of heart failure hospitalisations, appropriate implantable cardioverter-defibrillator shocks and cardiovascular mortality. RESULTS: Of 86 patients, 64.0% had LGE (80.2% male; mean LVEF 30.1% ± 12.7%). Mid-wall fibrosis (71.7%) was the most common pattern of LGE distribution. Over a mean follow-up period of 4.9 ± 3.2 years, 19 (34.5%) patients with LGE reached the composite endpoint compared to 4 (12.9%) patients without LGE (p = 0.01). Presence of LGE, but not echocardiographic LVEF, independently predicted the primary endpoint (hazard ratio 4.15 [95% confidence interval 1.28-13.50]; p = 0.02). CONCLUSION LGE presence independently predicted adverse clinical events in Asian patients with DCM. Routine use of CMR imaging to characterise the myocardial substrate is recommended for enhanced risk stratification and should strongly influence clinical management.

Cytochrome P4502J2 (CYP2J2) metabolizes arachidonic acid (AA) to cardioprotective epoxyeicosatrienoic acids (EETs). Dronedarone, an antiarrhythmic drug prescribed for treatment of atrial fibrillation (AF) induces cardiac adverse effects (AEs) with poorly understood mechanisms. We previously demonstrated that dronedarone inactivates CYP2J2 potently and irreversibly, disrupts AA-EET pathway leading to cardiac mitochondrial toxicity rescuable via EET enrichment. In this study, we investigated if mitigation of CYP2J2 inhibition prevents dronedarone-induced cardiac AEs. We first synthesized a deuterated analogue of dronedarone (termed poyendarone) and demonstrated that it neither inactivates CYP2J2, disrupts AA-EETs metabolism nor causes cardiac mitochondrial toxicity in vitro. Our patch-clamp experiments demonstrated that pharmacoelectrophysiology of dronedarone is unaffected by deuteration. Next, we show that dronedarone treatment or CYP2J2 knockdown in spontaneously beating cardiomyocytes indicative of depleted CYP2J2 activity exacerbates beat-to-beat (BTB) variability reflective of proarrhythmic phenotype. In contrast, poyendarone treatment yields significantly lower BTB variability compared to dronedarone in cardiomyocytes indicative of preserved CYP2J2 activity. Importantly, poyendarone and dronedarone display similar antiarrhythmic properties in the canine model of persistent AF, while poyendarone substantially reduces beat-to-beat variability of repolarization duration suggestive of diminished proarrhythmic risk. Our findings prove that deuteration of dronedarone prevents CYP2J2 inactivation and mitigates dronedarone-induced cardiac AEs.