Left ventricular tumour is a rare condition in children. The causes include vegetations, thrombus, and fibroma. 2-year-old asymptomatic female presented with an innocent heart murmur at 6 months of age. Subsequent follow-ups at 18 months of age showed left ventricular mass. Surgical pathology revealed “nodular fasciitis.” This type of tumour has never been described in the heart before.

A rare case of unique and previously unreported variant of transposition of great arteries with bilateral coni, ventricular septal defect, and triple-chambered left ventricle which led to an unexpected and accelerated post-natal cardiac anatomical and physiologic deviation from prenatal and immediate postnatal diagnosis altering the surgical management and patient outcome.

The flow patterns in the heart, in health and disease, have been of great interest for several years. Modern fluid dynamics analyses elucidate how underlying inefficient energetic or mixing characteristics of these flow patterns correlate with adverse effects. Unfortunately, translation of such modern analyses to the clinical stage remains a challenge. In this experimental work, we propose and demonstrate that braids of random and sparse particle trajectories provide an intuitive, global and practical description of cardiovascular flows. Moreover, we expose the flow pattern in an experimental healthy left ventricle model as a highly effective blood mixer at the topological level. Flow topologies that deviate from this pattern are accompanied by a reduction in energetic efficiency, as shown through comparisons with diseased flow models. These results suggest an ideal clinical approach to patient follow-up and the evaluation of the performance of medical devices.

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present.

Linoleic acid (LA), an essential n-6 fatty acid (FA), is critical for fetal development. We investigated the effects of maternal high LA (HLA) diet on offspring cardiac development and its relationship to circulating FA and cardiovascular function in adolescent offspring, and the ability of the postnatal diet to reverse any adverse effects. Female Wistar Kyoto rats were fed low LA (LLA; 1·44 % energy from LA) or high LA (HLA; 6·21 % energy from LA) diets for 10 weeks before pregnancy and during gestation/lactation. Offspring, weaned at postnatal day 25, were fed LLA or HLA diets and euthanised at postnatal day 40 (n 6–8). Maternal HLA diet decreased circulating total cholesterol and HDL-cholesterol in females and decreased total plasma n-3 FA in males, while maternal and postnatal HLA diets decreased total plasma n-3 FA in females. α-Linolenic acid (ALA) and EPA were decreased by postnatal but not maternal HLA diets in both sexes. Maternal and postnatal HLA diets increased total plasma n-6 and LA, and a maternal HLA diet increased circulating leptin, in both male and female offspring. Maternal HLA decreased slopes of systolic and diastolic pressure–volume relationship (PVR), and increased cardiac Col1a1, Col3a1, Atp2a1 and Notch1 in males. Maternal and postnatal HLA diets left-shifted the diastolic PVR in female offspring. Coronary reactivity was altered in females, with differential effects on flow repayment after occlusion. Thus, maternal HLA diets impact lipids, FA and cardiac function in offspring, with postnatal diet modifying FA and cardiac function in the female offspring.

We describe a rare case of aneurysmal right coronary artery drainage into left ventricle in a 38-year old male with entailed coronary CT images. After median sternotomy surgery, the patient recovered well.

A case of an asymptomatic child presenting with persistent systolic ejection click and found to have an unusual left ventricular false tendon attached to aortic valve that has not been described previously and may be responsible for the click.

Post-infective pseudoaneurysm of the left ventricle in children is very rare, with only five cases reported in English medical literature so far. Patients usually have a short history of infection by Staphylococcus aureus. Timely surgical intervention has generally a good outcome. We present a case of post-infectious pseudoaneurysm in a 2-year-old girl with a review of literature.

Objectives: This publication aims to report the cases of four children with pseudoaneurysm of the mitral-aortic intervalvular fibrosa and carry out a review of the literature. Background: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a very rare anomaly in children. It can be either congenital or acquired, namely, after bacterial endocarditis or cardiac trauma. This pathology does not usually cause specific symptoms but its outcome may be potentially fatal. Methods: We report the cases of four patients presenting with pseudoaneurysm of the mitral-aortic intervalvular fibrosa, referred for treatment in a paediatric cardiology clinic. Patient clinical notes were retrospectively reviewed for aetiology, clinical presentation, diagnostic work-up, surgical treatment, and follow-up. Literature on the subject was extensively reviewed. Results: In three patients, pseudoaneurysm of the mitral-aortic intervalvular fibrosa was acquired, being secondary to bacterial endocarditis in two cases and establishing after mitral surgery in another case. The remaining patient had a “congenital” aetiology – no other cause could be traced. The diagnosis was achieved by transthoracic echocardiography for all patients, and confirmed in all by trans-oesophageal echocardiography, to better define morphological details and to access flow into the aneurysmal formation. All patients were submitted to corrective cardiac surgery. Of the patients, three survived and were cured by surgery, staying asymptomatic, and one died after repeated interventions, for persistent endocarditis. Conclusions: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a rare but potentially fatal anomaly. In our experience, surgical cure was achieved for the majority of the cases, except for a case for which infection could not be locally eradicated, leading to multiple reinterventions.

Behçet's disease is a chronic autoimmune disease with vascular complications that are most frequently manifested as thromboembolism in veins and pseudoaneurysm in arteries. We report the case of a 13-year-old boy admitted for clinical and biological signs of rheumatic fever associated with chest pain. The clinical examination found heart sounds with a discrete systolic murmur of mitral regurgitation. The electrocardiogram showed a microvoltage with diffuse repolarisation disorder. Biologically, he had inflammatory syndrome. Transthoracic echocardiography showed circumferential pericardial effusion with anterosepto-apical hypokinesia of the left ventricle with systolic dysfunction, and a minimal mitral regurgitation. The patient was treated by corticotherapy and antibiotherapy. The outcome was marked by orogenital aphthous ulceration and decreased visual acuity related to intermediate uveitis. The retinal angiography showed a vasculitis. The late appearance of this symptom led to the right diagnosis of Behçet's syndrome. Transthoracic echocardiography showed a hypokinetic dilated cardiomyopathy left ventricular with septo apical and anterior akinesia and severe systolic dysfunction, with a defect of the inferior septal with a collar communicating the left ventricle with a giant pseudo aneurysm. Magnetic resonance imaging showed a giant pseudoaneurysm communicating with the left ventricle. The coronary computed tomography was normal. The patient had undergone surgical treatment for the pseudoaneurysm with good outcomes.

Objectives: The aim of the study was to combine clinical results from the European Cohort of the REVERSE study and costs associated with the addition of cardiac resynchronization therapy (CRT) to optimal medical therapy (OMT) in patients with mild symptomatic (NYHA I-II) or asymptomatic left ventricular dysfunction and markers of cardiac dyssynchrony in Spain.

Methods: A Markov model was developed with CRT + OMT (CRT-ON) versus OMT only (CRT-OFF) based on a retrospective cost-effectiveness analysis. Raw data was derived from literature and expert opinion, reflecting clinical and economic consequences of patient's management in Spain. Time horizon was 10 years. Both costs (euro 2010) and effects were discounted at 3 percent per annum.

Results: CRT-ON showed higher total costs than CRT-OFF; however, CRT reduced the length of hospitalization in ICU by 94 percent (0.006 versus 0.091 days) and general ward in by 34 percent (0.705 versus 1.076 days). Surviving CRT-ON patients (88.2 percent versus 77.5 percent) remained in better functional class longer, and they achieved an improvement of 0.9 life years (LYGs) and 0.77 years quality-adjusted life years (QALYs). CRT-ON proved to be cost-effective after 6 years, except for the 7th year due to battery depletion. At 10 years, the results were €18,431 per LYG and €21,500 per QALY gained. Probabilistic sensitivity analysis showed CRT-ON was cost-effective in 75.4 percent of the cases at 10 years.

Conclusions: The use of CRT added to OMT represents an efficient use of resources in patients suffering from heart failure in NYHA functional classes I and II.

To determine if matrix metalloproteinase (MMP)-28 mediates cardiac aging, wild-type (WT) and MMP-28−/− young (7 ± 1 months, n = 9 each) and old (20 ± 2 months, n = 7 each) female mice were evaluated. MMP-28 expression in the left ventricle (LV) increased 42% in old WT mice compared to young controls (p < 0.05). By Doppler echocardiography, LV function declined at 20 ± 2 months of age for both groups. However, dobutamine stress responses were similar, indicating that cardiac reserve was maintained. Plasma proteomic profiling revealed that macrophage inflammatory protein (MIP)-1 α, MIP-1β and MMP-9 plasma levels did not change in WT old mice but were significantly elevated in MMP-28−/− old mice (all p < 0.05), suggestive of a higher inflammatory status when MMP-28 is deleted. RT2-PCR gene array and immunoblotting analyses demonstrated that MIP-1α and MMP-9 gene and protein levels in the LV were also higher in MMP-28−/− old mice (all p < 0.05). Macrophage numbers in the LV increased similarly in WT and MMP-28−/− old mice, compared to respective young controls (both p < 0.05). Collagen content was not different among the WT and MMP-28−/− young and old mice. In conclusion, LV inflammation increases with age, and MMP-28 deletion further elevates inflammation and extracellular matrix responses, without altering macrophage numbers or collagen content.