Severe Mitral Paravalvular Leak Treated with Percutaneous Paravalvular Leak Closure with Underlying Severe Mitral Annular Calcium.

Standard operative mitral valve replacement for mitral stenosis in the setting of severe mitral annular calcium has been associated with increased morbidity and mortality. Inability to ensure a well seated prosthesis may lead to periprosthetic leak. We present a case of severe paravalvular leak, causing significant hemolysis, after mitral valve replacement with underling severe mitral annular calcium. The leak was successfully repaired using a transseptal percutaneous approach, with subsequent resolution of hemolysis.

Isolated congenitally corrected transposition of the great arteries with dextroversion discovered incidentally in a patient with cocaine-induced acute myocardial infarction.

Complex cardiac congenital anomalies can occasionally be found in adult patients who have no knowledge of their condition. Here we present the case of a 27-year-old man with cocaine-induced acute myocardial infarction in whom an isolated congenitally corrected transposition of the great arteries with dextroversion was discovered incidentally.

Dramatically different phenotypic expressions of hypertrophic cardiomyopathy in male cousins undergoing cardiac transplantation with identical disease-causing gene mutation.

Described herein are certain findings in 2 male cousins who underwent cardiac transplantation for severe heart failure (HF), one of the diastolic type (ejection fraction ≈65%), and one of the systolic type (ejection fraction ≈20%), both the consequence of hypertrophic cardiomyopathy (HC), and each had identical disease-causing gene mutations. The implanted heart in one had normal-sized ventricular cavities and no grossly visible ventricular wall lesions (except in one papillary muscle) and the other patient had severely dilated ventricular cavities and multiple extensive ventricular wall scars. The heart mass and the age of onset of symptoms was similar in each patient. A number of other family members had evidence of HC.

Imaging of low-gradient severe aortic stenosis.

Although most patients with severe aortic stenosis (AS) have high peak velocity and mean transvalvular gradient, there is a subset of patients with low-flow, low-gradient severe AS (LGSAS). Assessment and management of such patients can be difficult and dobutamine echocardiography has been recommended to distinguish those with pseudo-AS (low calculated AVA due to insufficient flow to fully open the valve) from those with contractile reserve and true LGSAS, who may have good outcomes with surgery. More recently, a group of patients with LGSAS and preserved LV function have been identified. These patients are often elderly with hypertension, small left ventricular cavities, and concentric left ventricular hypertrophy. Because cardiac imaging plays a vital role in hemodynamic classification of patients with suspected LGSAS and determining appropriate management, this review was undertaken to summarize the current state of knowledge of this important but complex condition.

Renal infarction secondary to cor triatriatum sinister.

A 30 year old, otherwise healthy man presented with flank pain and was ultimately found to have a rightsided renal infarction. Transthoracic echocardiography suggested, and then transesophageal echocardiography (TEE) confirmed, the presence of cor triatriatum sinister. Given the lack of other sources of emboli, this was felt to be the most likely source. We describe the case and both the echocardiographic and CT findings of this rare condition. This case demonstrates the need for TEE in some cases where 2D echocardiography is not sensitive enough to "rule out" cardio-embolic sources. This is only the second case in the literature of a systemic embolization due to cor triatriatum, and the first one in the Western literature.

Unusual origin of type 1 atrioventricular block with comments on Wenckebach's contribution.

We present a case that demonstrates the rare occurrence of type 1 second-degree atrioventricular block (Wenckebach or Mobitz type 1 block) as a result of block in the infranodal conduction system. This extremely rare occurrence often predicts progression to complete heart block and necessitates cardiac pacing. It also demonstrates the value of an electrophysiological study and exercise stress testing to identify the level of block, predict prognosis, and plan a management strategy.