Autosomal dominant polycystic kidney disease and pericardial effusion: coincidence? I think not! Case report and review of the literature.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary disease causing chronic renal failure, with a high incidence of extra-renal manifestations including pericardial effusion. Case summary: We present the case of a 41-year-old female, known for ADPKD, who presented to our emergency department with epigastric pain radiating to the interscapular area. Blood exams showed moderate increase in inflammatory markers. Echocardiography revealed a circumferential pericardial effusion of 10 mm. She was put under treatment with colchicine therapy (1 mg b.i.d.) based on a presumptive diagnosis of acute pericarditis with pericardial effusion. She was hospitalized due to increase in pericardial effusion, underwent pericardial drainage, and started prednisone therapy with rapid recovery. We started a close follow-up on a monthly basis, with progressive decrease in pericardial effusion and progressive amelioration in symptoms, although the patient continued to report mild asthenia. Discussion: Pericardial effusion and ADPKD are conditions that both require an interdisciplinary discussion for optimal patient care that avoids neglecting pivotal symptoms and avoidable invasive examinations.

Mitral annulus morphometry in degenerative mitral regurgitation phenotypes.

OBJECTIVES: Degenerative mitral regurgitation (DMR) is classified into different phenotypes based on the extent of leaflet degeneration. Our aim is to demonstrate that phenotype complexity predicts the extent of structural abnormalities of mitral annulus (MA). METHODS AND RESULTS: Seventy-five patients with DMR and severe valve regurgitation and 23 patients with normal mitral valve were studied using 3D transesophageal echocardiography. Classification of DMR was done by allocating each 3D echocardiography result under five categories: fibroelastic deficiency (FED), FED+, forme fruste, Barlow's disease Mitral annular disjunction (BD MAD)- or BD MAD+. MA was reconstructed in early systole and in end systole. We tested for a trend toward enlargement and flattening of MA in end systole and for a difference in MA dynamics from early systole to end systole with a worsening of DMR phenotype, in the whole spectrum of subjects ranging from controls to BD MAD+. A significant trend was observed toward larger anteroposterior diameter, intercommissural diameter, annulus circumference, and annulus area (P < .001). A reduction was found in annulus height to commissural width ratio (P = .003): This indicates a progressive MA flattening. Prolapse height and prolapse volume tended to be larger (P < .001). CONCLUSION: Based on the extent of leaflet degeneration, DMR is classified into different phenotypes. As the disease progresses, a related increase in MA size is found, with rounder annular shape, loss of saddle shape, and increase in height and volume of leaflet prolapse. The most pronounced alterations are found in BD MAD+.

Diastolic dysfunction grade in acute coronary syndromes: Application of 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging recommendations.

BACKGROUND: Diagnosis and grading of diastolic dysfunction (DD) is challenging, with different studies using heterogeneous criteria and guidelines not routinely applied in clinical practice. Our aim was to apply the 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging classification of DD among a contemporary population of patients with acute coronary syndromes (ACS) by analyzing its correlation with N-terminal pro b-type natriuretic peptide (NT-proBNP) and impact on clinical outcomes. METHODS: Independent investigators blinded to each other and to the clinical history reviewed digitally stored images to apply 2016 and 2009 DD definitions to 380 patients (mean age 66 ± 13 years, 75% men) with ACS admitted to the coronary care unit between January 2016 and March 2018. RESULTS: DD was frequent with both definitions, yet the concordance was weak (kappa =0.21, p < 0.01). Inter-observer reliability was greater by applying the 2016 algorithm (kappa = 0.89, p < 0.001). There was a significant correlation between NT-proBNP and worsening DD (Spearman's rho r = 0.54 for 2016 and r = 0.24 for 2009 algorithms, both p < 0.001). Worse DD was associated with worse clinical presentation and increased risk of events (HR for the cumulative incidence of heart failure and death during follow-up 2.15 [95% CI 1.66-2.78, p < 0.001] and 1.82 [95% CI 1.39-2.40, p < 0.001] for 2016 and 2009 classifications, respectively, all p < 0.001). CONCLUSIONS: The agreement between 2016 and 2009 DD definitions was poor, with newer guidelines having grater interobserver reliability. The positive graded association between 2016 DD classification and NT-proBNP and its association with clinical outcomes provide a validation of the latest guideline algorithm in ACS patients.

"Where is the Heart?" When Cardiac Magnetic Resonance Imaging Helps if Echocardiography is Inconclusive.

Cardiovascular magnetic resonance (CMR) is the gold standard technique to comprehensively assess cardiac structure and function. A 64-year-old male, planned for surgical coronary revascularization, underwent transthoracic and transesophageal echocardiography for a mitral regurgitation, with an eccentric jet of unclear mechanism; these examinations were inconclusive because of the lack of adequate visualization of the cardiac structures. A CMR was then performed to quantify mitral regurgitation and, additionally, it documented a giant hiatus hernia with gastric sliding into the thorax. In this case, CMR helped to better define the severity of a valvular disease and provided ancillary information from the extracardiac findings.

Anatomy of mitral annulus insights from non-invasive imaging techniques.

The mitral annulus (MA) is not a continuous ring of connective tissue from which are suspended mitral leaflets. Instead, it is a much more complex structure made up of a mix of fibrous, muscular, and adipose tissues. MA is a key structure in any type of mitral valve repair and recently it has been targeted for transcutaneous devices. Thus, a deep understanding of MA anatomy has never been more important. Traditionally, cardiac anatomy has been described using anatomic specimens. Currently, sophisticated non-invasive techniques allow imaging of MA with a richness of anatomical details unimaginable only two decades ago. The aim of this review is to provide a better understanding of the peculiar aspects of MA as they are revealed through these imaging techniques and discuss clinical implications related to this complex structure.