Extensive cardiac FDG uptake in a patient with AL amyloidosis.

Cardiac AL amyloidosis is a medical emergency causing rapid deterioration of cardiac function; however, it remains to be a diagnostic challenge especially when presenting with unusual symptoms and clinical findings. We present case of a 44-year-old patient with typical angina, persistently elevated troponin and normal epicardial coronary arteries. He was initially treated for myocarditis due to chest pain with troponin rise. However, CMR finding of subendocardial enhancement, increased native T1 values as well as extensive diffuse FDG uptake on PET-CT also suggested inflammatory cardiac conditions. Rapid decline in LV function and clinical deterioration led to further investigations including serum free light chains and bone marrow biopsy which confirmed systemic AL amyloidosis. Although the pathophysiology of unusual FDG PET-CT findings remains unknown, marked myocardial FDG uptake might have been caused by various features that were associated with AL amyloidosis including myocardial cell toxicity/inflammation or microvascular dysfunction. Awareness of these features specific to AL amyloidosis among physicians and description of associated cardiac FDG uptake findings has a potential to aid early diagnosis.

Assessment of left ventricular tissue mitochondrial bioenergetics in patients with stable coronary artery disease.

Recurrent myocardial ischemia can lead to left ventricular (LV) dysfunction in patients with coronary artery disease (CAD). In this observational cohort study, we assessed for chronic metabolomic and transcriptomic adaptations within LV myocardium of patients undergoing coronary artery bypass grafting. During surgery, paired transmural LV biopsies were acquired on the beating heart from regions with and without evidence of inducible ischemia on preoperative stress perfusion cardiovascular magnetic resonance. From 33 patients, 63 biopsies were acquired, compared to analysis of LV samples from 11 donor hearts. The global myocardial adenosine triphosphate (ATP):adenosine diphosphate (ADP) ratio was reduced in patients with CAD as compared to donor LV tissue, with increased expression of oxidative phosphorylation (OXPHOS) genes encoding the electron transport chain complexes across multiple cell types. Paired analyses of biopsies obtained from LV segments with or without inducible ischemia revealed no significant difference in the ATP:ADP ratio, broader metabolic profile or expression of ventricular cardiomyocyte genes implicated in OXPHOS. Differential metabolite analysis suggested dysregulation of several intermediates in patients with reduced LV ejection fraction, including succinate. Overall, our results suggest that viable myocardium in patients with stable CAD has global alterations in bioenergetic and transcriptional profile without large regional differences between areas with or without inducible ischemia.

Reliability of pediatric ventricular function analysis by short-axis "single-cycle-stack-advance" single-shot compressed-sensing cines in minimal breath-hold time.

OBJECTIVES: Cardiovascular magnetic resonance (CMR) cine imaging by compressed sensing (CS) is promising for patients unable to tolerate long breath-holding. However, the need for a steady-state free-precession (SSFP) preparation cardiac cycle for each slice extends the breath-hold duration (e.g. for 10 slices, 20 cardiac cycles) to an impractical length. We investigated a method reducing breath-hold duration by half and assessed its reliability for biventricular volume analysis in a pediatric population. METHODS: Fifty-five consecutive pediatric patients (median age 12 years, range 7-17) referred for assessment of congenital heart disease or cardiomyopathy were included. Conventional multiple breath-hold SSFP short-axis (SAX) stack cines served as the reference. Real-time CS SSFP cines were applied without the steady-state preparation cycle preceding each SAX cine slice, accepting the limitation of omitting late diastole. The total acquisition time was 1 RR interval/slice. Volumetric analysis was performed for conventional and "single-cycle-stack-advance" (SCSA) cine stacks. RESULTS: Bland-Altman analyses [bias (limits of agreement)] showed good agreement in left ventricular (LV) end-diastolic volume (EDV) [3.6 mL (- 5.8, 12.9)], LV end-systolic volume (ESV) [1.3 mL (- 6.0, 8.6)], LV ejection fraction (EF) [0.1% (- 4.9, 5.1)], right ventricular (RV) EDV [3.5 mL (- 3.34, 10.0)], RV ESV [- 0.23 mL (- 7.4, 6.9)], and RV EF [1.70%, (- 3.7, 7.1)] with a trend toward underestimating LV and RV EDVs with the SCSA method. Image quality was comparable for both methods (p = 0.37). CONCLUSIONS: LV and RV volumetric parameters agreed well between the SCSA and the conventional sequences. The SCSA method halves the breath-hold duration of the commercially available CS sequence and is a reliable alternative for volumetric analysis in a pediatric population. KEY POINTS: • Compressed sensing is a promising accelerated cardiovascular magnetic resonance imaging technique. • We omitted the steady-state preparation cardiac cycle preceding each cine slice in compressed sensing and achieved an acquisition speed of 1 RR interval/slice. • This modification called "single-cycle-stack-advance" enabled the acquisition of an entire short-axis cine stack in a single short breath hold. • When tested in a pediatric patient group, the left and right ventricular volumetric parameters agreed well between the "single-cycle-stack-advance" and the conventional sequences.

Unusual Complicated Fungal Endocarditis in a Patient With Vascular Ehlers-Danlos Syndrome.

A 24-year-old man with dilated aortic root and vascular type Ehlers-Danlos syndrome underwent aortic root replacement with a stentless bioprosthesis. A year later he developed aortic valve endocarditis necessitating a redo valve replacement. Excised valve tissue grew fungus Purpureocillium lilacinum. Over a period of 4 years the case was further complicated with mycotic aneurysm of the infrarenal aorta, a retroperitoneal abscess (treated surgically), a right common iliac aneurysm, right lower limb emboli (femoral embolectomy and left-to-right femoral bypass was performed), 2 false aneurysms of the ascending aorta with a large mobile mass attached to the anterior aneurysm, and brain abscesses.

SYNTAX score predicts postoperative atrial fibrillation in patients undergoing on-pump isolated coronary artery bypass grafting surgery.

OBJECTIVE: Atrial fibrillation (AF) is the most common arrhythmia following coronary artery by-pass graft surgery (CABG). The value of SYNTAX score to predict postoperative atrial fibrillation (PoAF) has not been clearly addressed. We aimed to evaluate this relationship in patients undergoing isolated CABG. METHODS: This study was designed as a single-center, non-randomized, observational, prospective study. Ninety-four patients undergoing isolated on-pump CABG, who had sinus rhythm and were older than 18 years, were enrolled. Demographic characteristics of the patients were recorded; SYNTAX score was calculated preoperatively for each patient. The univariate and multivariate logistic regression analysis were used to determine for predictors of PoAF. RESULTS: The median SYNTAX score of the enrolled patients was 21, (56-5). PoAF was observed in 31 (33.3%) patients. Univariate logistic regression showed that age, chronic obstructive pulmonary disease (COPD), red blood cell distribution width (RDW), urea, initial troponin I, peak postoperative troponin I, interventricular septum, left atrial diameter, and SYNTAX score were significantly associated with the frequency of PoAF following CABG. An independent association was identified with age [ß: 0.088, p:0.023, OR: 1.092, 95% CI (1.012-1.179)], COPD [(ß: 2.222, p:0.003, OR: 9.228, 95% CI (2.150-39.602)], and SYNTAX score [(ß: 0.130, p:0.002, OR: 1.139, 95% CI (1.050-1.235)]. CONCLUSION: This study showed that a higher SYNTAX score was related to more frequent PoAF in patients undergoing isolated on-pump CABG.

Asymptomatic Pulmonary Embolism after Ablation.

BACKGROUND: Pulmonary embolism (PE) is a life-threatening event with a broad presentation spectrum ranging from asymptomatic cases to sudden cardiac arrest. It is unclear if right atrial emboli cause PE in patients with atrial fibrillation (AF) or if mild PE itself increases right cardiac pressure provoking AF. OBJECTIVE: To determine the incidence and predictors of asymptomatic PE in patients undergoing AF ablation. METHOD AND RESULTS: Patients (n = 93) were screened and those with previous or current symptomatic PE or venous thromboembolism, pulmonary hypertension, increased right heart pressures detected on echocardiography, a history of stroke, transient ischemic attack, coagulopathy or cancer and inappropriate contrast for the evaluation of pulmonary arterial tree were excluded. The remaining AF patients (n = 71) underwent guided ablation controlled with 3-dimensional, left atrial and pulmonary venous computed tomography. The asymptomatic PE was defined by using the modified Miller score by 2 independent assessors in 6 patients. Univariate logistic regression showed that age (OR: 1.094, 95% CI 1.007-1.188, p = 0.033), diabetes (OR: 12.000, 95% CI 1.902-75.716, p = 0.008), CHA2DS2-VASc score (OR: 2.800, 95% CI 1.304-6.013, p = 0.008), and pulmonary artery diameter (OR: 1.221, 95% CI 1.033-1.444, p = 0.019) were significantly associated with PE. However, multivariate analysis revealed that the CHA2DS2-VASc score (p = 0.047) remained the exclusive significant predictor for asymptomatic PE. CONCLUSION: The incidence of random asymptomatic PE in AF patients is high (>8%). The CHA2DS2-VASc score can predict silent PE. Since patients with a high CHA2DS2-VASc score are already anticoagulated, our results do not change clinical practice but are noteworthy in terms of the cause-effect relationship between AF and PE.

Atrial longitudinal strain parameters predict left atrial reverse remodeling after mitral valve surgery: a speckle tracking echocardiography study.

Volume overload in chronic severe mitral regurgitation (MR) causes left atrial (LA) remodeling. Volume overload generally diminishes after mitral valve surgery and LA size and shape are expected to recover. The recovery of LA functions named as reverse remodeling is said to be related with prognosis and mortality. A few clinical and echocardiographic parameters have been reported to be associated with LA reverse remodeling. In this study, we investigated the relationship between LA peak longitudinal strain (reservoir strain) assessed with 2-dimensional speckle tracking echocardiography (2D STE) and LA reverse remodeling. 53 patients (24 females and 29 males, mean age: 45.7 ± 13.5 years) with severe MR and preserved left ventricular systolic function were included in the study. All patients had normal sinus rhythm. The etiology of MR was mitral valve prolapse (MVP) in 37 patients and rheumatic valvular disease in 16 patients. Mitral valve repair was performed in 30 patients while 23 underwent mitral valve replacement. Echocardiography was performed before the surgery and 6 months later. LA peak atrial longitudinal strain (PALS) was assessed with speckle tracking imaging. LA reverse remodeling was defined as a percent of decrease in LA volume index (LAVI). Left atrial volume index significantly decreased after surgery (58.2 ± 16.6 vs. 43.9 ± 17.2 ml/m2, p ≤ 0.001). Mean LAVI reduction was 22.5 ± 27.2%. There was no significant difference in LAVI reduction between mitral repair and replacement groups (22.1 ± 22.6 vs. 23.1 ± 32.8 %, p = 0.9). Although the decrease in LAVI was higher in MVP group than rheumatic group, it was not statistically significant (24.4 ± 26.8 vs. 18.2 ± 28.9%, p = 0.4). Correlates of LAVI reduction were preoperative LAVI (r 0.28, p = 0.039), PALS (r 0.36, p = 0.001) and age (r -0.36, p = 0.007). Furthermore, in multivariate linear regression analysis (entering models), preoperative LAVI, age and PALS were all significant predictors of LA reverse remodeling (p ≤ 0.001, p = 0.04, p = 0.001 respectively). Left atrial peak longitudinal strain measured by 2D STE, in conjunction with preoperative LAVI and age is a predictor of LA reverse remodeling in patients undergoing surgery for severe MR. We suggest that in this patient population, PALS may also be used as a preoperative prognostic marker.

The dangerous fifth chamber: congenital left atrial appendage aneurysm.

Aneurysms of the left atrial appendage are extremely rare. Enlargement of the left atrial appendage can be congenital or acquired. Dysplasia of the left atrial muscles leads to congenital left atrial appendage aneurysm and usually presents as atrial tachyarrhythmia or embolic events in the second or third decade of life. We report a case of an asymptomatic 12-year-old child with a congenital left atrial appendage aneurysm. Transthoracic and transoesophageal echocardiography demonstrated a large left atrial appendage aneurysm without thrombus or spontaneous echo-contrast. The patient was successfully treated with surgical resection of the aneurysm.

A rare cause of severe mitral regurgitation: mitral valve aneurysm.

Perforation of a mitral valve aneurysm is a rare cause of acute mitral regurgitation, and valvular aneurysm formation and its rupture without infectious involvement are unusual. An 80-year-old man was admitted with acute onset and progressive dyspnea. He had no history of chest pain, palpitation, or fever. Laboratory findings did not suggest any signs of infection. Transthoracic echocardiography revealed an aneurysm of the mitral septal leaflet protruding into the left atrium during systole and color-flow Doppler ultrasonography showed severe mitral regurgitation. There was no aortic regurgitation nor evidence for rheumatic involvement of the valvular structures. Pulmonary artery systolic pressure estimated from the tricuspid regurgitation jet was 50 mmHg. Transesophageal echocardiography showed a saccular, thin-walled, mitral valve aneurysm on the atrial surface, expanding during systole and a small tissue defect on the aneurysmatic segment of the mitral leaflet. There were no signs of connective tissue disease. The patient was submitted to surgery. The aneurysmatic and perforated parts on the septal leaflet were resected and an annuloplasty ring was placed. The histopathological examination of the mitral valve tissue showed nonspecific degenerative changes. The postoperative period was uneventful and the patient was discharged on the fifth postoperative day.