Myocardial fibrosis and its relation to adverse outcome in transposition of the great arteries with a systemic right ventricle.

Background: Myocardial dysfunction has been implicated in gradual heart failure in transposition of the great arteries (TGA) with a systemic right ventricle (RV). Fibrosis can be assessed using the extracellular volume fraction (ECV). Our aim was to measure ECV and determine its associations with clinical findings and outcomes.

Limited Accuracy of Administrative Data for the Identification and Classification of Adult Congenital Heart Disease.

Background: Administrative data sets utilize billing codes for research and quality assessment. Previous data suggest that such codes can accurately identify adults with congenital heart disease (CHD) in the cardiology clinic, but their use has yet to be validated in a larger population.

Methods And Results: All administrative codes from an entire health system were queried for a single year.

Exercise intolerance in patients with congenitally corrected transposition of the great arteries relates to right ventricular filling pressures.

Background: Patients with congenitally corrected transposition of the great arteries (ccTGA) have significantly reduced exercise tolerance. Progressive right ventricular (RV) dysfunction with tricuspid regurgitation (TR) and other haemodynamic lesions are common among them. We hypothesised that interaction of these factors may result in increased systemic RV filling pressure, which in turn impact on exercise capacity.

Clinical characteristics of 304 kindreds evaluated for familial dilated cardiomyopathy.

Background: Familial dilated cardiomyopathy (FDC) is dilated cardiomyopathy of unknown cause occurring in 2 or more closely related family members.

Methods And Results: Members of 304 families suspected to have FDC were evaluated by family history (FH) and medical record review and were categorized as affected with idiopathic dilated cardiomyopathy (IDC), unaffected, unknown, or no data. Pedigrees were categorized with confirmed FDC, probable FDC, possible FDC or IDC based on strength of evidence.

Late gadolinium enhancement cardiovascular magnetic resonance of the systemic right ventricle in adults with previous atrial redirection surgery for transposition of the great arteries.

Background: Patients treated for transposition of the great arteries by atrial redirection surgery have a right ventricle (RV) that sustains systemic pressures long term. Late RV dysfunction occurs in these patients; the reasons for this are unclear, but myocardial fibrosis may be important. Myocardial fibrosis can be visualized by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR).

Validation of the myocardial performance index by echocardiography in mice: a noninvasive measure of left ventricular function.

Background: The myocardial performance index (MPI) is a Doppler-based measure of left ventricular (LV) function. It is noninvasive, independent of LV shape, and does not require dimensional measurements. However, it has never been validated in mice.

Perinatal hypoxia causes ventricular enlargement associated with increased atrial natriuretic peptide (ANP) mRNA levels in newborn mice.

We sought to examine both the short-term and residual effects of perinatal hypoxia on ventricular mass and function of mice. We postulated that the magnitude of the ventricular hypertrophy would be determined by the timing of the exposure, be linked to augmented atrial natriuretic peptide (ANP) expression, and would persist to young adulthood. Furthermore, mice deficient in the ANP receptor type A (ANPRA) would have even greater hypertrophy.