Cardiomyopathy in the peripartum period due to left ventricular non-compaction and association with Ebstein's anomaly: a case report.

BACKGROUND: Left ventricular non-compaction (LVNC) cardiomyopathy is a persistence of abnormal foetal myocardium and is a rare cause of cardiomyopathy in the peripartum period. Unlike other causes of peripartum cardiomyopathy which typically improve, LVNC has significant long-term personal and family implications and needs lifelong follow-up. CASE SUMMARY: We describe a unique case of a 30-year-old woman who developed cardiomyopathy in the peripartum period which was revealed on cardiovascular magnetic resonance imaging to be due to occult LVNC. Our patient also had Ebstein's anomaly, which is a known LVNC association. DISCUSSION: Cardiomyopathy in the peripartum period can be a decompensation of previously asymptomatic subclinical cardiomyopathy. It is important to assess for LVNC in patients presenting with this. Cardiovascular magnetic resonance imaging is the gold-standard imaging modality and allows accurate diagnosis of LVNC, associated structural complications and rare associations such as Ebstein's anomaly. Left ventricular non-compaction is irreversible and has implications for patients and their family members.

Radiation-induced dystrophic calcification and severe valvular stenosis: the central role of multimodality 3D cardiac imaging in disease assessment and planning of combined transcatheter aortic and mitral valve replacement.

Cardiac disease after mediastinal radiotherapy can result in progressive valvular thickening and dystrophic calcification with ensuing leaflet restriction and dysfunction. This can ultimately manifest as valvular stenosis and/or regurgitation. We report a case of a 61-year-old woman with symptomatic severe aortic stenosis and severe mitral stenosis due to severe dystrophic calcification postmediastinal radiotherapy for lymphoma. She was deemed surgically inoperable due to dense, continuous calcification throughout the leaflets and annuli of both valves, aortomitral continuity, proximal coronary arteries and proximal porcelain aorta. She underwent simultaneous transcatheter aortic valve replacement and transcatheter mitral valve replacement with an excellent technical and clinical result at 7-month follow-up. We also describe the central role of multimodality three-dimensional transoesophageal echocardiography and multidetector cardiac CT imaging in assessing the severity of valve disease, characterising the nature of cardiac calcification and guiding decisions on surgical operability and transcatheter intervention.

Heart disease in pregnancy: ischaemic heart disease.

Coronary artery disease and in particular acute coronary syndromes in pregnancy are increasing with high risk of mortality and significant morbidity. Whilst women with atherosclerotic risk factors are at greater risk of developing problems in pregnancy, it is important to remember that women can develop problems even in the absence of atherosclerosis-secondary to thrombosis or coronary dissection. A low threshold to investigate women with chest pain is paramount, and women with raised troponin levels should be investigated seriously. Acute coronary syndromes should be managed using an invasive strategy where possible and women should not have coronary angiography withheld for fear of foetal harm. This article aims to review the limited available data of coronary artery disease in pregnancy and give practical advice on the management of stable and acute coronary disease, with particular emphasis on the latter.

Specific beta(2)AR blocker ICI 118,551 actively decreases contraction through a G(i)-coupled form of the beta(2)AR in myocytes from failing human heart.

BACKGROUND: We have observed direct (noncatecholamine-blocking) negative inotropic effects of the selective beta(2)-adrenoceptor (AR) antagonist ICI 118,551 in myocytes from failing human ventricle. In this study we characterize the effect in parallel in human myocytes and in myocytes from animal models where beta(2)ARs or G(i) proteins are overexpressed. METHODS AND RESULTS: Enzymatically isolated, superfused ventricular myocytes were exposed to betaAR agonists and antagonists/inverse agonists, and contraction amplitude was measured. ICI 118,551 decreased contraction in ventricular myocytes from failing human hearts by 45.3+/-4.1% (n=20 hearts/31 myocytes, P<0.001) but had little effect in nonfailing hearts (4.9+/-4%, n=5 myocytes/3 hearts). Effects were significantly larger in patients classified as end-stage. Transgenic mice with high beta(2)AR number and increased G(i) levels had normal basal contractility but showed a similar negative inotropic response to ICI 118,551. Overexpression of human beta(2)AR in rabbit myocytes using adenovirus potentiated the negative inotropic effect of ICI 118,551. In human, rabbit, and mouse myocytes, the negative inotropic effects were blocked after treatment of cells with pertussis toxin to inactivate G(i), and overexpression of G(i)alpha(2) induced the effect de novo in normal rat myocytes. CONCLUSIONS: We hypothesize that ICI 118,551 binding directs the beta(2)AR to a G(i)-coupled form and away from the G(s)-coupled form (ligand-directed trafficking). ICI 118,551 effectively acts as an agonist at the G(i)-coupled beta(2)AR, producing a direct negative inotropic effect. Conditions where beta(2)ARs are present and G(i) is raised (failing human heart, TGbeta(2) mouse heart) predispose to the appearance of the negative inotropic effect.