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LEARNING OBJECTIVES 1. Understand the methodology of mitral annular segmentation by computed tomography (CT) prior to transcatheter mitral valve replacement. 2. Learn about CT reformats and reconstruction planes in the assessment of mitral valve pathology. 3. Conceptualize mitral annular dynamism and factors affecting annular measurement on cardiac CT angiography with whole cardiac cycle coverage. 4. Utilize CT data for pre-procedural planning: identification of fluoroscopic angles, localization…

LEARNING OBJECTIVES 1. Review how valvular regurgitation can be quantified with cardiac magnetic resonance imaging. 2. Discuss the role of cardiac magnetic resonance imaging for mitral valve disease. 3. Review the advantages/disadvantages of cardiac magnetic resonance imaging. INTRODUCTION Over the last several decades, cardiac magnetic resonance (CMR) has emerged as an invaluable tool in cardiovascular imaging. CMR is a comprehensive noninvasive imaging modality that offers high…

LEARNING OBJECTIVES 1. Understand the general principles and specific imaging techniques involved in screening for mitral transcatheter edge-to-edge repair (TEER). 2. Understand the imaging sequence and specific goals of intraprocedural imaging at each stage in TEER. 3. Recognize procedural complications and imaging challenges during TEER. INTRODUCTION Imaging plays an essential role in structural transcatheter interventions revealing intracardiac landscape and events throughout the diagnostic work-up, procedural planning,…

LEARNING OBJECTIVES Transesophageal echocardiography is the imaging modality of choice to evaluate the mitral valve complex prior to transcatheter interventions A comprehensive assessment of the mitral valve for interventions includes defining the morphology, etiology, and severity of disease Quantifying mitral regurgitation severity at baseline, intraprocedurally, and at follow-up requires a multi-parametric and multi-modality (two- [2D] and three-dimensional [3D]) echocardiographic assessment. INTRODUCTION With the addition of transcatheter…

LEARNING OUTCOMES Comprehensively assess the mitral valve apparatus and the left ventricle to distinguish degenerative versus functional mitral regurgitation as they are different disease processes with different prognoses and different treatments. Evaluate the severity of mitral regurgitation using an integrated approach as no single parameter is accurate. It is essential to evaluate color-flow Doppler (flow convergence, vena contracta, color jet area), pulsed-wave Doppler (mitral inflow, pulmonary…

LEARNING OBJECTIVES Define anatomic components of the mitral valve apparatus Describe the etiology of two main types of mitral valve pathology, i.e., mitral stenosis and mitral regurgitation; their mechanistic effects on mitral valve leaflets and physical examination findings. THE MITRAL VALVE Anatomy Mitral valve apparatus components include: two main leaflets, anterior and posterior, each with three distinct scallops (A1, A2, A3, and P1, P2, P3), mitral…

LEARNING OBJECTIVES To review treatment options and mitral valve recommendations from the 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease and discuss differences compared to the 2021 European Society of Cardiology/European Association for Cardio-Thoracic Surgery (ESC/EACTS) Guidelines for the Management of Valvular Heart Disease. INTRODUCTION Guidelines and recommendations for the treatment of valvular syndromes are periodically published by the American College of…

LEARNING OBJECTIVES Recognize abnormalities of the mitral valve that lead to a disease state. Describe how abnormalities of the mitral valve cause pathology. Predict the outcomes of patients with each mitral valve pathology. Differentiate functional, primary, and mixed pathology. INTRODUCTION Understanding the normal structure and function of the components of the mitral apparatus is key in understanding the resultant pathophysiology when these components, or their interaction…

LEARNING OUTCOMES The mitral valve is a complex and highly dynamic structure comprised of various structures that work as a single functional unit. The mitral valve annulus is a nonplanar saddle-shaped structure with its highest point at the mid-anterior annulus close to the aortic valve and with a second minor peak at the mid-posterior annulus. The fibrous skeleton is concentrated at the base of the ventricular…

Idiopathic pulmonary arterial hypertension (IPAH) is a rare progressive disease that eventually, if left untreated, progresses to right heart failure and death. The first pathologic description of the condition dates to 1891, but until about two decades ago, no adequate treatment existed, and the disorder received relatively little clinical attention. The advent of disease-targeting therapies has greatly improved prognoses, stimulated research into the condition, and provided…

Marfan syndrome is an autosomal dominant disorder of connective tissue in which cardiovascular, skeletal, and ocular abnormalities may be present to a highly variable degree. Prevalence has been estimated at 2 to 3 in 10,000, and 25% to 30% of cases represent new mutations. Prognosis is mainly determined by progressive dilatation of the aorta, potentially leading to aortic dissection and death at a young age (…

Until the second half of the past century, cardiac tumors were diagnosed almost exclusively at autopsy. However, advances in cardiac imaging and the development of cardiopulmonary bypass made cardiac tumors treatable. Primary cardiac tumors are a rare entity with an incidence of 0.056% to 1.23% according to autopsy reports. Although patients with cardiac tumors may present with cardiovascular or constitutional symptoms, the diagnosis is frequently incidental…

Rheumatic fever (RF) is an inflammatory disease that affects various systems such as the skin, brain, cardiovascular system, and many mucosal membranes, that is, the pericardium, the pleura and the peritoneum. The joints are involved to a great extent. However, although the peripheral joints produce the most noticeable symptoms, it is the cardiovascular system that suffers the most long-lasting influences. It remains the most common pathogenic…

Definitions Noncompacted myocardium (NCM) is a cardiac abnormality involving the myocardial wall, characterized by numerous, excessively prominent trabeculations and deep intertrabecular recesses penetrating into the midmyocardium ( Fig. 64.1 ). Noncompaction of the left ventricle can occur as an isolated cardiac feature (left ventricular noncompaction [LVNC]) or in association with other primary cardiomyopathies as idiopathic dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), or even restrictive cardiomyopathy (RCM).…

Acknowledgments This work has been supported by TRANSAC, University of Padua Strategic Project CPDA133979/13, Padua, Italy; Registry for Cardio-cerebro-vascular Pathology, Veneto Region, Venice, Italy; Target Project, Regional Health System, Venice, Italy; PRIN Ministry of Education, University and Research 2010BWY8E9_004, Rome, Italy; University Research Grant CPDA144300, Padua, Italy. Arrhythmogenic cardiomyopathy (AC) is a rare, genetically determined cardiomyopathy featured by progressive myocardial dystrophy with fibrofatty replacement afflicting the…

The clinical presentation of constrictive pericarditis is similar to that of restrictive cardiomyopathy: predominantly signs of systemic venous congestion and less pronounced signs of low cardiac output. The distinction between these two diagnoses is difficult but very important because constrictive pericarditis is treatable, whereas restrictive cardiomyopathy is likely not. This chapter emphasizes the hemodynamic differences and the diagnostic techniques that can be used to differentiate between…

Definition Hypertrophic cardiomyopathy (HCM) is defined as left ventricular (LV) hypertrophy in the absence of abnormal loading conditions (valve disease, hypertension, congenital heart defects) sufficient to explain the degree of hypertrophy. Although asymmetrical septal hypertrophy was first described in the late 19th century, it was only after landmark reports by Sir Russell Brock (later Lord Brock) in 1957 and Donald Teare in 1958 that HCM became…

The cardiomyopathies represent an important group of heart muscle disorders that are all associated with significant morbidity and mortality. The most prevalent of these conditions is dilated cardiomyopathy (DCM), which accounts for more than half of all cases. A proportion of cases of DCM result from the progression of an initial inflammatory insult to the myocardium during an episode of acute myocarditis. Significant efforts have been…

Definition/Morphology Tomisaku Kawasaki first reported Kawasaki disease (KD) in 1967. At first, KD was thought to be an acute and self-limiting febrile disorder; however, the first nationwide survey in Japan revealed that 1.7% of patients had died from acute cardiac failure with necropsy showing coronary arteritis accompanied by aneurysms and thrombotic occlusion. KD is an acute febrile multisystem vasculitic syndrome of unknown etiology that occurs predominantly…

Anomalous coronary arteries are frequently seen in conjunction with certain other congenital cardiac defects and may even be considered inherent in some anomalies, such as in transposition of the great vessels. These abnormalities, which may be called secondary coronary artery anomalies, will be discussed in brief at the end of this chapter. Abnormalities of the origin or the course of coronary arteries in the absence of…