Imaging in mitral stenosis.

PURPOSE OF REVIEW: Mitral stenosis remains clinically relevant in developing countries where rheumatic heart disease is the predominant culprit. In the western world, mitral annular and valvular calcification is an increasingly recognized cause, particularly in an aging population. Echocardiography plays a primary role in imaging mitral stenosis with a growing role for cardiac computed tomography and magnetic resonance imaging. In this review, we aim to revisit mitral stenosis assessment and quantification using multimodality imaging. RECENT FINDINGS: There is an increasing role for advanced cardiac imaging especially in the era of transcatheter mitral valve intervention. Also, when echocardiography is suboptimal or discordant with symptoms, computed tomography can provide anatomical data, whereas magnetic resonance imaging can provide anatomical along with hemodynamic data. SUMMARY: Diagnosis of mitral stenosis is crucial as it carries an increased morbidity and mortality risk. Echocardiography is the cornerstone imaging modality with alternative, complementary advanced imaging considered when images are suboptimal.

Assessment of myocardial viability by cardiac MRI.

PURPOSE OF REVIEW: Left ventricular systolic dysfunction because of coronary artery disease is common, and ascertaining which patients will benefit from revascularization can be challenging. Viability testing is an accepted means by which to base this decision, with multiple noninvasive imaging modalities available for this purpose. This review aims to highlight the key role of cardiac magnetic resonance in myocardial viability assessment, with a focus on its unique strengths over other imaging modalities. RECENT FINDINGS: Transmural extent of hyperenhancement with late gadolinium imaging has been shown to be greater acutely in ST elevation myocardial infarction patients undergoing primary percutaneous coronary intervention and regress at follow-up studies. An explanation for this reported phenomenon and an argument against redefining CMR viability criteria in the acute setting will be offered. SUMMARY: Although not universally available, cardiac magnetic resonance is an exceptionally powerful and well tolerated imaging modality that should be considered when viability testing will influence patient management. Although observational outcomes data suggest a promising prognostic role for viability, randomized studies in this area are needed.

Origin-specific epigenetic program correlates with vascular bed-specific differences in Rgs5 expression.

Cells from multiple origins contribute to vascular smooth muscle cell (VSMC) development. Phenotypic heterogeneity of VSMCs is associated with their point of developmental origin; however, the mechanisms driving such differences are unknown. We here examined the mechanisms controlling vascular bed-specific differences in Rgs5 expression during development. Rgs5 levels were similar across different regions of the vasculature in neonatal animals but were >15-fold higher in descending aortas compared with carotid arteries of adult mice. Thus, vessel bed-specific changes in regulation of Rgs5 expression occurred during vessel maturation. Examination of adult Rgs5-LacZ reporter mice revealed lower Rgs5 expression in VSMCs originating from the third (carotid artery) branchial arch compared with those originating in the fourth and sixth (aortic B segment, right subclavian, and ductus arteriosus) branchial arches. Indeed, a mosaic Rgs5 expression pattern, with discreet LacZ boundaries between VSMCs derived from different developmental origins, was observed. Furthermore, Rgs5-LacZ expression was correlated with the site of VSMC origin (splanchic mesoderm ≈ local mesenchyme > somites > proepicardium > mesothelium). Surprisingly, Rgs5 reporter activity in cultured carotid artery- and descending aorta-derived cells did not recapitulate the differences observed in vivo. Consistent with a developmental origin-specific epigenetic mechanism driving the observed expression differences in vivo, the Rgs5 promoter showed increased methylation on CpG dinucleotides in carotid arteries compared with that in descending aortas in adult but not in neonatal mice. In vitro methylation of the Rgs5 promoter confirmed that its activity is sensitive to transcriptional down-regulation by CpG methylation. These data suggest that an origin-dependent epigenetic program regulates vascular bed- and maturation state-dependent regulation of VSMC-specific gene transcription.

Rare Coronary Embolism Secondary to Cardioversion of Atrial Fibrillation.

The diagnosis and management of myocardial infarction with nonobstructive coronary arteries (MINOCA) are difficult due to its variable presentations, different causes, and challenging diagnostic approaches. Cardiac imaging modalities including cardiac magnetic resonance (CMR) are very useful tools for diagnosing and managing MINOCA. Myocardial infarction (MI) can be caused by coronary emboli that can be contributed to atrial fibrillation (AF). Rarely, coronary embolism with resultant MINOCA can occur after direct current cardioversion (DCCV) even in fully anticoagulated patients. We present a rare case of a coronary embolism following DCCV as well as a CMR finding of microvascular obstruction (MVO), which has not previously been reported after DCCV. This case also emphasizes the value of obtaining a CMR for patients with MINOCA.

Between a Rock and a Hard Place: Idiopathic Cardiac Osseous Metaplasia Complicating Severe Mitral Regurgitation.

A 77-year-old woman presented for assessment of symptomatic mitral regurgitation. Multimodality cardiac imaging revealed severe mitral regurgitation secondary to mitral valve prolapse. Significant mitral annular calcification with dramatic intramyocardial calcification was also incidentally discovered. She was then given a diagnosis of idiopathic cardiac osseous metaplasia and was managed conservatively. (Level of Difficulty: Advanced.).

A case of ruptured sinus of Valsalva aneurysm and reversible flow-induced pulmonary hypertension.

Pulmonary hypertension (PH) in adults with congenital heart disease (CHD) and significant systemic-to-pulmonary shunting is a significant cause of morbidity and mortality. Its pathophysiology is incompletely understood, but involves a flow-induced pulmonary arteriopathy characterized by endothelial cell dysfunction and vascular remodeling that alters pulmonary arterial vasoreactivity. There is a paucity of literature linking PH with left-to-right shunting due to ruptured sinus of Valsalva aneurysms (SOVA). We present a unique case of reversible, flow-associated PH due to a ruptured congenital right SOVA fistulizing into the right atrium (RA), with emphasis on non-invasive and invasive assessment of pulmonary hemodynamics before and after surgical intervention.

Experience with bisoprolol in long-QT1 and long-QT2 syndrome.

BACKGROUND: The protective effect of beta-blockers in patients with inherited Long-QT syndrome is well established. Recent reports have suggested that beta-blockers are not equally effective in Long-QT (LQT). Bisoprolol is an attractive candidate for use in LQT because of its cardioselective properties and favorable side-effect profile. METHODS: We performed a retrospective cohort study of 114 consecutive patients with gene-positive Long-QT syndrome type 1 (LQT1) or Long-QT syndrome type 2 (LQT2) treated with bisoprolol, nadolol or atenolol with a total of 580 person-years of follow-up. Electrocardiogram (ECG) parameters and cardiac events during follow-up were compared. In addition, exercise treadmill testing was performed in bisoprolol-treated patients. RESULTS: Fifty-nine patients were treated with bisoprolol, 39 with atenolol and 16 with nadolol. Overall, 59 % were females and 62 % had LQT1. Baseline heart rate and corrected QT (QTc) interval were similar between the groups. QTc shortening was observed in individuals on bisoprolol (ΔQTc -5 ± 31 ms; p = 0.049) and nadolol (ΔQTc -13 ± 16 ms; p = 0.02) but not on atenolol (ΔQTc +9 ± 24 ms; p = 0.16). Median follow-up was similar for bisoprolol and nadolol (3 years), but longer for atenolol (6 years; p = 0.03); one cardiac event occurred in the bisoprolol group (1.7 %) and two events occurred in the atenolol group (5.1 %; p = 0.45), whereas none occurred in nadolol-treated patients. Beta-blocker efficacy was not affected by the underlying genotype. The antiadrenergic effect of bisoprolol correlated with the reduction of peak heart rates at exercise testing. CONCLUSIONS: Bisoprolol treatment results in QTc shortening in gene-positive LQT1 and LQT2 patients and is well tolerated during long-term administration. The equivalence of bisoprolol for protection from ventricular arrhythmia in LQT patients compared to established beta-blockers remains unknown. Further large-scale studies are required.

Arrhythmogenic right ventricular cardiomyopathy with recessive inheritance related to a new homozygous desmocollin-2 mutation.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia is an inherited cardiomyopathy that is transmitted in autosomal dominant and autosomal recessive forms and involves mutations in desmosomal and extradesmosomal genes. We present a case of arrhythmogenic right ventricular cardiomyopathy that cosegregates in a Lebanese family with a previously unreported desmocollin-2 mutation (c.712_714delGAT). We believe this newly described genetic variant displays autosomal recessive inheritance without the cutaneous manifestations expected in recessive genotypes, and represents the latest addition to the compendium of desmosomal mutations with pathogenic potential.