Loeys-Dietz syndrome is a genetic disorder that predisposes patients to aortic aneurysms. If left untreated, the natural history of the associated aortopathy often culminates in fatal aortic dissection. We describe the case of a 21-year-old man who was diagnosed with Loeys-Dietz syndrome after 2 family members died of aortic dissection. This case highlights the importance of increased physician awareness of this syndrome, which can play a crucial role in preventing premature sudden cardiac death caused by aortic catastrophe.

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare panencephalitis that can present with severe cardiac dysrhythmias. We present a case of a 19-year-old woman with no significant medical history who presented with progressive changes in mental status and profound ictal asystole that necessitated the placement of an external temporary pacemaker. She was diagnosed with and treated for anti-NMDA receptor encephalitis, and she recovered after a prolonged and complicated hospitalization. We review the pathophysiology and management of anti-NMDA receptor encephalitis, as well as its cardiac manifestations.

We report an unusual case of a patient with hypertrophic obstructive cardiomyopathy, anomalous aortic origin of a coronary artery, obesity hypoventilation syndrome, and acquired long QT syndrome who was able to defy the odds of sudden cardiac death in the rarest of circumstances. (Level of Difficulty: Advanced.).

Echocardiography is considered as an initial imaging modality of choice in patients with congenital heart disease (CHD), and magnetic resonance (MR) imaging is preferred for detailed functional information. Multi-detector computed tomography (CT) plays an important role in clinical practice in assessing post-operative morphological and functional information of patients with complex CHD when echocardiography and MR imaging are not contributory. Radiologists should understand and become familiar with the complex morphology and physiology of CHD, as well as with various palliative and corrective surgical procedures performed in these patients, to obtain CT angiograms with diagnostic quality and promptly recognise imaging features of normal post-operative anatomy and complications of these complex surgeries.

Coronary artery disease is the leading cause of mortality worldwide. Diagnosis is conventionally performed by direct visualization of the arteries by invasive coronary angiography (ICA), which has inherent limitations and risks. Measurement of fractional flow reserve (FFR) has been suggested for a more accurate assessment of ischemia in the coronary artery with high accuracy for determining the severity and decision on the necessity of intervention. Nevertheless, invasive coronary angiography-derived fractional flow reserve (ICA-FFR) is currently used in less than one-third of clinical practices because of the invasive nature of ICA and the need for additional equipment and experience, as well as the cost and extra time needed for the procedure. Recent technical advances have moved towards non-invasive high-quality imaging modalities, such as magnetic resonance, single-photon emission computed tomography, and coronary computed tomography (CT) scan; however, none had a definitive modality to confirm hemodynamically significant coronary artery stenosis. Coronary computed tomography angiography (CCTA) can provide accurate anatomic and hemodynamic data about the coronary lesion, especially calculating fractional flow reserve derived from CCTA (CCTA-FFR). Although growing evidence has been published regarding CCTA-FFR results being comparable to ICA-FFR, CCTA-FFR has not yet replaced the invasive conventional angiography, pending additional studies to validate the advantages and disadvantages of each diagnostic method. Furthermore, it has to be identified whether revascularization of a stenotic lesion is plausible based on CCTA-FFR and if the therapeutic plan can be determined safely and accurately without confirmation from invasive methods. Therefore, in the present review, we will outline the pros and cons of using CCTA-FFR vs. ICA-FFR regarding diagnostic accuracy and treatment decision-making.

Cardiac aspergillomas are rare and associated with high mortality. We present a case of right ventricular cardiac aspergilloma in an asymptomatic renal transplant recipient. Intracavitary aspergilloma is an important consideration in evaluation of an intracardiac mass in an immunocompromised patient. (Level of Difficulty: Advanced.).

Takotsubo cardiomyopathy (TC) is associated with significant short-term morbidity and mortality. Several risk factors for poor outcomes have been identified; however, the prognostic implications of pre-existing comorbidity in TC are poorly delineated. We sought to assess the association of aggregate pre-existing comorbidity with short-term outcomes in TC. We performed a retrospective observational study of adult subjects diagnosed with TC at two academic tertiary care hospitals between 2005 and 2018. Overall burden of medical comorbidity was estimated using the Charlson comorbidity index (CCI). Multivariable logistic regression was used to test for independent association of CCI with 30-day mortality and severe shock at index presentation. Multivariable poisson regression was performed to assess the association of CCI with duration of hospitalization. Five-hundred and thirty-eight subjects were diagnosed with TC during the study period. The median CCI score of all subjects was 2 (IQR 1-4). Among subjects with physical triggers of TC, the median CCI score was 2 (IQR 1-4) compared to a median CCI score of 1 (IQR 0-1) in subjects with non-physical triggers of TC (P < 0.001). Seventy-six (14%) subjects died within 30 days of index diagnosis and 185 (34%) subjects experienced severe shock. The median duration of hospitalization was 7 days (IQR 3-14 days). In multivariable logistic regression, CCI was not associated with 30-day mortality or severe shock. In multivariable Poisson regression, CCI (IRR 1.17, 95% CI 1.16-1.18, P < 0.001) was associated with duration of hospitalization. Increased burden of pre-existing medical comorbidity was not independently associated with 30-day mortality or severe shock at index presentation, but was associated with increased duration of hospitalization after diagnosis of TC.

Background

There is significant interindividual variability in the rate of aortic stenosis (AS) progression that is not accounted for in the current surveillance algorithms. We sought to examine the association between changes in peak aortic jet velocity (Vmax) and mean gradient (MG) among patients with mild or moderate AS and risk of progression to severe disease.

Methods

Adult subjects referred for echocardiography at a single academic referral center with a diagnosis of mild or moderate AS and ≥2 additional surveillance echocardiograms were included in the study. Changes in Vmax and MG between the first two echocardiograms were indexed to time and tested for association with future progression to severe AS.

Results

Among three hundred and sixty-four subjects, the median time between first and second echocardiograms was 1.3 years and initial changes in Vmax and MG indexed to time were +0.16 m/s per year and +1.44 mmHg per year, respectively. Fifty-three (15%) and fifty-six (15%) subjects progressed to severe AS defined by Vmax and MG, respectively. In multivariable logistic regression, initial increase in Vmax (OR = 4.19, 95% CI 1.93-9.10, p < 0.001) and initial increase in MG (OR = 1.12, 95% CI 1.06-1.18, p < 0.001) were associated with progression to severe AS.

Conclusions

Initial changes in Vmax and MG among patients with mild or moderate AS are strongly associated with risk of progression to severe AS and may help guide individualized surveillance strategies.

In the present case report, we describe the management of severe coronary artery disease in a patient with Glanzmann thrombasthenia. To the best of our knowledge, there are no established guidelines for revascularization in this setting, and we pose novel discussion points regarding the nuanced care of this patient. (Level of Difficulty: Intermediate.).