Cardiac Magnetic Resonance Imaging Features in Hypertrophic Cardiomyopathy Diagnosed at <21 Years of Age.

Hypertrophic cardiomyopathy (HC) is the most common inherited cardiomyopathy, with varied timing of phenotypic and clinical presentation. Literature describing cardiac magnetic resonance (CMR) imaging and late gadolinium enhancement (LGE) in young patients with HC is limited. This study included patients diagnosed with HC at young age (<21 years) between January 1990 and January 2015 who underwent transthoracic echocardiography and CMR with assessment of LGE at a single tertiary referral center. LGE was quantified via a method of 6 standard deviations and patients were grouped based upon presence or absence of LGE (≤1% and >1% LGE, respectively). Sudden cardiac death (SCD) risk was assessed in patients >16 years of age using the European SCD risk score. A composite outcome of New York Heart Association class III-IV symptoms, aborted SCD, heart transplantation, and all-cause mortality was assessed via Kaplan-Meier curves with log-rank analysis. Overall, 126 patients were included (78 male; 62%). Median age of diagnosis was 15 (12 to 18) years. LGE was present in 81 (64%) patients, although only 4 (3%) patients had LGE >15%. Median age at CMR imaging was 19 (15 to 23) years. Patients with LGE had greater wall thickness (25 ± 8 mm vs 22 ± 7 mm, p = 0.01). Median European SCD risk score was 4.7 (2.9 to 6.5). Median follow-up was 6.5 (2.5 to 13) years with 26 patients (21%) meeting the composite outcome. There were no significant differences in composite outcome since age of diagnosis when stratified by presence/absence of LGE (p = 1.0). The presence of LGE in young HC patients was not an independent risk factor for cardiovascular morbidity and mortality. Wall thickness was greater in patients with LGE. There remains a need for further evaluation of this unique HC cohort.

Comparison of Laser In Situ Keratomileusis Flap Morphology and Predictability by WaveLight FS200 Femtosecond Laser and Moria Microkeratome: An Anterior Segment Optical Coherence Tomography Study.

PURPOSE: To evaluate laser in situ keratomileusis (LASIK) flap thickness predictability and morphology by femtosecond (FS) laser and microkeratome (MK) using anterior segment optical coherence tomography. METHODS: Fifty-two candidates for the LASIK procedure were stratified into two groups: FS laser-assisted (Allegretto FS-200) and MK flap creation (Moria 2). Flap thickness was determined at five points. The side-cut angle was measured in three directions at the margin interface. LASIK flap assessment was performed one month postoperatively by Spectralis anterior segment optical coherence tomography. RESULTS: Fifty-two patients (93 eyes) were recruited; 49 eyes were stratified to the FS group and 44 eyes to the MK group. The FS group had relatively even flap configurations, and the MK group had meniscus-shaped flaps. Mean differences between planned and actual flap thickness were 12.93 ± 8.89 and 19.91 ± 5.77 µm in the FS and MK groups, respectively. In thin flaps (100 to 110 µm), there was a significant disparity between the two groups (7.80 ± 4.71 and 19.44 ± 4.46 µm in the FS and MK groups, respectively). However, in thicker flaps (130 µm), comparable flap thickness disparity was achieved (18.54 ± 9.52 and 20.83 ± 5.99 µm in the FS and MK groups, respectively). Mean side-cut angle was 74.29 ± 5.79 degrees and 32.34 ± 4.94 degrees in the FS and MK groups, respectively. CONCLUSIONS: Comparable flap thickness predictability was achieved in thicker flaps (130 µm), while the FS laser technique yielded a more predictable result in thinner flaps (100 to 110 µm). Different flap morphology was observed in meniscus flaps in MK-LASIK and flap morphology in FS-LASIK.

77-year-old female with syncope.

CLINICAL INTRODUCTION: A 77-year-old female was referred for evaluation of an episode of syncope while eating breakfast. There was no history of fall, syncope, prodrome, dyspnoea, chest discomfort or palpitations. Medical history was notable for hyperlipidaemia and treated hypertension. Blood pressure was 140/90 mm Hg, pulse 85  beats per minute (BPM). No murmurs were present on cardiac examination. ECG revealed normal sinus rhythm with left ventricular (LV) hypertrophy (see online supplementary figure S1). Holter monitor demonstrated rare premature ventricular complexes (<1% of beats), without heart block or ventricular tachycardia. Transthoracic echocardiogram is shown in figure 1. QUESTION: Which of the following is the explanation for the flow indicated by the yellow arrow? Aortic stenosisCoronary artery flow, indicative of coronary fistulaHypertrophic cardiomyopathy with apical pouchHypertensive heart diseaseMitral stenosis.

Prognostic value of segment involvement score compared to other measures of coronary atherosclerosis by computed tomography: A systematic review and meta-analysis.

BACKGROUND: The segment involvement score (SIS) is a semiquantitative measure of the extent of atherosclerosis burden by coronary computed tomography angiography (CTA). We sought to evaluate by meta-analysis the prognostic value of SIS, and to compare it with other CTA measures of coronary artery disease (CAD). METHODS: Electronic databases from 1946 to January 2016 were searched. Studies reporting SIS, or an equivalent measure by coronary CTA, and clinical outcomes were included. Maximally adjusted hazard ratios (HR), predominantly for clinical variables, were extracted for SIS, obstructive CAD, Agatston coronary artery calcium score, and plaque composition. These were pooled using DerSimonian-Laird random effects models. RESULTS: Eleven nonrandomized studies with good methodological quality enrolling 9777 subjects (mean age 61 ± 11 years, 57% male, mean follow up 3.3 years) who had 472 (4.8%) MACE (cardiac or all cause death, non-fatal myocardial infarction or late revascularization), were included. SIS (per segment increase) had pooled HR of 1.25 (95% CI: 1.16,1.35; I2 = 71.4%, p < 0.001) for MACE. HR for MACE was 1.37 (95% CI: 1.32,1.42; I2 = 95.6%, p < 0.001) for number of segments with stenosis (per segment increase), 3.39 (95% CI: 1.65,6.99; I2 = 87.8%, p = 0.001) for obstructive CAD (binary variable) and 1.00 (95% CI: 1.00,1.01; I2 = 75.0%, p = 0.490) for Agatston score (per unit increase). HRs by plaque composition (calcified, non-calcified and mixed; per segment change) were 1.24 (95% CI: 1.10,1.39; I2 = 81.6%, p = 0.001), 1.20 (95% CI: 0.97,1.48; I2 = 92.9%, p = 0.093) and 1.27 (95% CI: 1.03,1.58; I2 = 89.8%, p = 0.029), respectively. CONCLUSION: Despite heterogeneity in endpoints, extent of CAD as quantified by SIS on coronary CTA is a strong, independent predictor of cardiovascular events.

Acromegaly-induced cardiomyopathy with dobutamine-induced outflow tract obstruction.

A 50-year-old man with a history of acromegaly was referred for preoperative cardiac evaluation preceding trans-sphenoidal resection of a pituitary macroadenoma. Dobutamine stress echocardiography was negative for myocardial ischaemia. Resting left ventricular (LV) LV ejection fraction (LVEF) was 64% and there was hypertrophy of ventricular septum (18 mm) without resting LV outflow tract obstruction. With 40 µg/kg/min of dobutamine, the LVEF became hyperdynamic at 80%, and there was a maximal instantaneous LV outflow tract gradient of 77 mm Hg. There was no delayed myocardial enhancement on cardiac MRI and the pattern of hypertrophy was concentric. Acromegaly-induced cardiomyopathy can mimic hypertrophic cardiomyopathy in the setting of dobutamine provocation. Because cardiomyopathy is an important cause of mortality in acromegaly, diagnosis and appropriate management are critical to improve survival.