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…
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…
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…
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,…
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…
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,…
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…
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…
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…
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…