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Fig. 1. A: Late-systolic still-frame of a transthoracic short axis view depicts the cleft (⁎) of the mildly thickened posterior mitral leaflet. AML denotes anterior mitral leaflet. B: Colour Doppler showing a moderate mitral regurgitation. A 64-year-old lady was referred for evaluation of a recently detected heart murmur. The patient was completely asymptomatic and an avid tennis player. Auscultation revealed a loud midto late systolic click followed by a grade III midto late systolic regurgitant murmur. Transthoracic echocardiography showed an isolated cleft of the mildly thickened posterior mitral valve leaflet with a moderate late systolic mitral regurgitation (Fig. 1). The anterior mitral valve leaflet and the subvalvular apparatus were normal, especially the insertion of the papillary muscles. No other cardiac anomalies were present. Isolated cleft posterior mitral valve leaflet is a very rare congenital malformation that is usually diagnosed in early childhood. The cleft is more commonly found in the anterior leaflet and in association with other cardiac anomalies such as atrioventricular septal defects and anomalies of the papillary muscles [1,2]. A retrospective analysis of more than 13,000 children revealed only ten isolated cleft mitral valve leaflets with none of them affecting the posterior leaflet [3]. Cleft mitral valve leaflet may result in severe mitral regurgitation and the need for surgical repair [4]. Our patient was diagnosed with the malformation at age sixty-four, had only moderate mitral regurgitation and remained asymptomatic.

Background Patients with a systemic right ventricle (RV) have a compromised late outcome caused by ventricular dysfunction. Standard medical heart failure therapy has not been shown to improve RV function and survival in these patients. Phosphodiesterase (PDE)-5 inhibition increases contractility in experimental models of RV hypertrophy, but not in the normal RV. In clinical practice, the effects of PDE-5 inhibition on systemic RV function and exercise capacity in adults with a systemic RV have not been tested. Methods The SERVE protocol is a double-blind, randomized placebo-controlled multicenter superiority trial to study the effect of PDE-5 inhibition with Tadalafil on RV volumes and function in patients with either D-transposition of the great arteries repaired with an atrial switch procedure or with congenitally corrected transposition of the great arteries. Tadalafil 20mg or placebo will be given over a study period of 3years. The primary endpoint is the change in mean end-systolic RV volumes from baseline to study end at 3years of follow-up (or at the time of permanent discontinuation of the randomized treatment if stopped before 3- years of follow-up), and will be measured by cardiovascular magnetic resonance imaging (CMR) or by cardiac computed tomography in patients with contraindications for CMR. Secondary endpoints are changes in RV ejection fraction, VO2max and NT-proBNP. Conclusion The objective of this study is to assess the effect of PDE-5 inhibition with Tadalafil on RV size and function, exercise capacity and neurohumoral activation in adults with a systemic RV over a 3-year follow-up period.

Background In adults with prior arterial switch operation (ASO) for d-transposition of the great arteries, the need for routine coronary artery assessment and evaluation for silent myocardial ischemia is not well defined. In this observational study we aimed to determine the value of a comprehensive cardiovascular magnetic resonance (CMR) protocol for the detection of coronary problems in adults with prior ASO for d-transposition of the great arteries. Methods Adult ASO patients (≥18 years of age) were recruited consecutively. Patients underwent a comprehensive stress perfusion CMR protocol that included measurement of biventricular systolic function, myocardial scar burden, coronary ostial assessment and myocardial perfusion during vasodilator stress by perfusion CMR. Single photon emission computed tomography (SPECT) was performed on the same day as a confirmatory second imaging modality. Stress studies were visually assessed for perfusion defects (qualitative analysis). Additionally, myocardial blood flow was quantitatively analysed from mid-ventricular perfusion CMR images. In unclear cases, CT coronary angiography or conventional angiography was done. Results Twenty-seven adult ASO patients (mean age 23 years, 85% male, 67% with a usual coronary pattern; none with a prior coronary artery complication) were included in the study. CMR stress perfusion was normal in all 27 patients with no evidence of inducible perfusion defects. In 24 cases the coronary ostia could conclusively be demonstrated to be normal. There was disagreement between CMR and SPECT for visually-assessed perfusion defects in 54% of patients with most disagreement due to false positive SPECT. Conclusions Adult ASO survivors in this study had no CMR evidence of myocardial ischemia, scar or coronary ostial abnormality. Compared to SPECT, CMR provides additional valuable information about the coronary artery anatomy. The data shows that the asymptomatic and clinically stable adult ASO patient has a low pre-test probability for inducible ischemia. In this situation it is likely that routine evaluation with stress CMR is unnecessary.