Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis-associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension.

Rationale: Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality rate despite the progress achieved with pulmonary arterial vasodilator therapies.

Objectives: We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease phenotype when compared with idiopathic pulmonary arterial hypertension (IPAH) on the basis of progression of echocardiographic right ventricular (RV) dysfunction.

Methods: Retrospective analysis of echocardiographic data in 13 patients with SSc-PAH and 11 patients with IPAH was used to delineate the progression of RV dysfunction during single or combination pulmonary arterial vasodilator therapy.

Atrioventicular block in familial amyloidosis; revisiting an old debate.

Familial amyloidosis with polyneuropathy (FAP) is the rarest of the cardiac amyloidoses, with less than 200 cases diagnosed each year. In this disease, cardiac involvement is characterized by extracellular amyloid infiltration throughout the heart, usually resulting in sinoatrial nodal dysfunction, cardiomyopathy, and congestive heart failure. While cardiac conduction disturbances are a common feature of FAP, the mechanism of these disturbances seems to be a point of contention among researchers.

Traumatic disruption of the fibrous skeleton of the heart, with injury of the tricuspid and mitral valves, aortic annulus, and ventricular septum.

In an automobile accident, a young man sustained blunt trauma to the chest that caused injury to the fibrous skeleton of the heart. The mitral and tricuspid valves and their annuli were lacerated, the aortic annulus was separated from the ventricular septum, and the ventricular septum was disrupted; however, with surgical management, the patient survived.