An Extremely Rare Congenital Muscle Bundle Crossing the Right Atrial Cavity

Muscle bundles in the right atrium are an extremely rare congenital anomaly. We report the case of a patient with 2 atrial septal defects and a large muscle bundle crossing the right atrium. Only 3 comparable cases have previously been published. (Level of Difficulty: Intermediate.)

Right ventricular involvement in left ventricular non-compaction cardiomyopathy

Background: Left ventricular non-compaction cardiomyopathy (LVNC) features extensive trabeculations. Involvement of the right ventricle (RV) has been reported; however, distinction from normal RV trabeculation is difficult. This study aimed at assessing RV morphology and function in LVNC by cardiac magnetic resonance (CMR) and transthoracic echocardiography (TTE).Methods: Dimensional and functional parameters were assessed according to guidelines. Novel CMR parameters were RV end-diastolic (ED) trabeculated area, RV ED trabeculated volume, and RV ED non-compacted to compacted (NC/N) ratio in short axis (SAX) as well as in four-chamber view (4CH).Results: Twenty patients with LVNC and 20 controls were included. RV size and function were comparable in LVNC and controls and exhibited a good correlation between TTE and CMR. Although RV trabeculated area, RV trabeculated volume, and RV ED NC/C ratio in SAX as well as in 4CH were larger in LVNC, there was a major overlap with values in controls. RV ED NC/C ratio in SAX correlated with LV ED NC/C ratio (not in 4CH). Quantitative assessment of RV non-compaction was not feasible in TTE.Conclusions: Right ventricle size and function in LVNC can be measured by CMR and TTE, while RV trabeculation can only be quantified by CMR. RV myocardium displays more trabeculations in LVNC; however, overlap with normal individuals is extensive, not allowing separation of patients with LVNC from controls

Pericardial effusion unrelated to surgery is a predictor of mortality in heart transplant patients

Background: Hemodynamically irrelevant pericardial effusion (PeEf) is a predictor of adverse outcome in heart failure patients. The clinical relevance of a PeEf unrelated to surgery in heart transplant patients remains unknown. This study assesses the prognostic value of PeEf occurring later than 1 year after transplantation. Methods: All patients undergoing heart transplantation in Zurich between 1989 and 2012 were screened. Cox proportional hazard models were used to analyze mortality (primary) and hospitalization (secondary endpoint). PeEf time points were compared to baseline for rejection, immunosuppressants, tumors, inflam­mation, heart failure, kidney function, hemodynamic, and echocardiographic parameters. Results: Of 152 patients (mean age 48.3 ± 11.9), 25 developed PeEf. Median follow-up period was 11.9 (IQR 5.8–17) years. The number of deaths was 6 in the PeEf group and 46 in the non-PeEf group. The occurrence of PeEf was associated with a 2.5-fold increased risk of death (HR 2.49, 95% CI 1.02–6.13, p = 0.046) and hospitalization (HR 2.53, 95% CI 1.57–4.1, p = 0.0002). Conclusions: This study reveals that the finding of hemodynamically irrelevant PeEf in heart trans­plant patients is a predictor of adverse outcome, suggesting that a careful clinical assessment is war­ranted in heart transplant patients exhibiting small PeEf

Augmentation Index in Patients with Thoracic Aortic Aneurysm: A Matched Case-Control Study

Thoracic aortic aneurysms (TAA) may be associated with complications such as rupture and dissection, which can lead to a fatal outcome. Increased central arterial stiffness has been proposed to be present in patients with TAA compared to unmatched controls. We aimed to assess whether wall properties in patients with TAA are also altered when compared to a matched control group. Applanation tonometry was performed in 74 adults with TAA and 74 sex, age, weight, height, and left ventricular ejection fraction matched controls. Subsequently analysis of the pulse wave was done using the SphygmoCor System. For comparing the two groups, AIx was adjusted to a heart rate of 75/min (AIx@75). 148 1-to-1 matched participants were included in the final model. There was no significant difference in the Alx@75 between the TAA group and the matched control group [mean (SD) of 24.7 (11.2) % and 22.8 (11.2) %, p = 0.240]. Adjusted for known cardiovascular risk factors, there was no association between TAA and AIx@75. Patients with TAA showed comparable arterial wall properties to cardiovascular risk factor matched controls. Since higher arterial stiffness is associated with TAA progression, it remains to be investigated if increased central arterial stiffness is a relevant factor of TAA emergence. Keywords: thoracic aortic aneurysm; augmentation index; arterial stiffnes

Renal dysfunction and outcome in left ventricular non-compaction

Background: While renal function has been observed to inversely correlate with clinical outcome in other cardiomyopathies, its prognostic significance in patients with left ventricular non-compaction cardiomyopathy (LVNC) has not been investigated. The aim of this study was to determine the prognostic value of renal function in LVNC patients. Methods: Patients with isolated LVNC as diagnosed by echocardiography and/or magnetic resonance imaging in 4 Swiss centers were retrospectively analyzed for this study. Values for creatinine, urea, and estimated glomerular filtration rate (eGFR) as assessed by the CKD-EPI 2009 formula were collected and analyzed by a Cox regression model for the occurrence of a composite endpoint (death or heart transplantation). Results: During the median observation period of 7.4 years 23 patients reached the endpoint. The age- and gender-corrected hazard ratios (HR) for death or heart transplantation were: 1.9 (95% confidence interval [CI] 1.4–2.6) for each increase over baseline creatinine level of 30 µmol/L (p < 0.001), 1.6 (95% CI 1.2–2.2) for each increase over baseline urea level of 5 mmol/L (p = 0.004), and 3.6 (95% CI 1.9–6.9) for each decrease below baseline eGFR level of 30 mL/min (p ≤ 0.001). The HR (log2) for every doubling of creatinine was 7.7 (95% CI 3–19.8; p < 0.001), for every doubling of urea 2.5 (95% CI 1.5–4.3; p < 0.001), and for every bisection of eGFR 5.3 (95% CI 2.4–11.6; p < 0.001). Conclusions: This study provides evidence that in patients with LVNC impairment in renal function is associated with an increased risk of death and heart transplantation suggesting that kidney function assessment should be standard in risk assessment of LVNC patients

White matter microstructure and its relation to clinical features of obsessive–compulsive disorder: findings from the ENIGMA OCD Working Group

Microstructural alterations in cortico-subcortical connections are thought to be present in obsessive–compulsive disorder (OCD). However, prior studies have yielded inconsistent findings, perhaps because small sample sizes provided insufficient power to detect subtle abnormalities. Here we investigated microstructural white matter alterations and their relation to clinical features in the largest dataset of adult and pediatric OCD to date. We analyzed diffusion tensor imaging metrics from 700 adult patients and 645 adult controls, as well as 174 pediatric patients and 144 pediatric controls across 19 sites participating in the ENIGMA OCD Working Group, in a cross-sectional case-control magnetic resonance study. We extracted measures of fractional anisotropy (FA) as main outcome, and mean diffusivity, radial diffusivity, and axial diffusivity as secondary outcomes for 25 white matter regions. We meta-analyzed patient-control group differences (Cohen’s d) across sites, after adjusting for age and sex, and investigated associations with clinical characteristics. Adult OCD patients showed significant FA reduction in the sagittal stratum (d = −0.21, z = −3.21, p = 0.001) and posterior thalamic radiation (d = −0.26, z = −4.57, p < 0.0001). In the sagittal stratum, lower FA was associated with a younger age of onset (z = 2.71, p = 0.006), longer duration of illness (z = −2.086, p = 0.036), and a higher percentage of medicated patients in the cohorts studied (z = −1.98, p = 0.047). No significant association with symptom severity was found. Pediatric OCD patients did not show any detectable microstructural abnormalities compared to controls. Our findings of microstructural alterations in projection and association fibers to posterior brain regions in OCD are consistent with models emphasizing deficits in connectivity as an important feature of this disorder

Red Flags Suggesting Cardiac Transthyretin Amyloidosis (ATTR) in Clinical Practice

Cardiac wtATTR is caused by extracellular deposition of misfolded proteins in the heart. It mostly affects elderly men and is still clearly underdiagnosed. Recognizing red flags suggesting wtATTR is key for a timely diagnosis, enabling the patient to profit from effective therapies. If general practitioners suspect cardiac amyloidosis, it is crucial to rapidly exclude AL-amyloidosis by immunoelectrophoresis, immunofixation as well as light-chain assay, because AL-amyloidosis needs urgent hematologic therapy. After that, the patient should be referred to the cardiologist for further assessment

Complete recovery from severe tachycardia-induced cardiomyopathy in a patient with Ebstein's anomaly

We report the case of a young patient with repaired Ebstein's anomaly who developed severe tachycardia-induced cardiomyopathy and a large apical thrombus as a consequence of sustained atrial flutter with a 2:1 conduction. In spite of a dramatic course in hospital with prolonged mechanical resuscitation and extracorporeal membrane oxygenation, she survived and made a rapid and full recovery. This remarkable case underlines that atrial arrhythmias, the most common complication in adults with congenital heart disease, may have devastating outcomes when timely recognition is missed and treatment delayed-thus, emphasizing the importance of good patient education