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Dobutamine stress echocardiography has become widely accepted in the evaluation of adult patients with coronary heart disease. We wanted to assess the feasibility and the physiologic responses of stress echocardiography at low doses of dobutamine in a population of normal children and adults. Once achieved, we submitted a group of post-anthracycline patients to the test to assess the sensitivity of low-dose dobutamine stress echocardiography in the detection of cardiac dysfunction. Thirty-two healthy children and young adults (19 male and 13 female subjects, median age 15 years [range 6 to 26]) were studied. After the initial study of normal subjects, we submitted a cohort of 39 patients (18 female and 21 male, aged 6 to 25 years), who completed anthracycline chemotherapy, to the same protocol. Dobutamine was infused at rates of 0.5 to 2.5 and 5 microg/kg/min and echocardiographic measurements were obtained at rest and at the end of each stage. The test could be completed in 100% of the subjects without major complications. Statistically significant differences between resting echocardiographic values of systolic and diastolic function and values at 2.5 and 5 microg/kg/min of dobutamine were found. Moreover, dobutamine revealed or enhanced differences between normal subjects and the post-anthracycline patients. Thus, low-dose dobutamine stress echocardiography is feasible and safe in older children. The test is very sensitive for the detection of subclinical cardiac dysfunction in post-anthracycline patients and could possibly assess functional myocardial reserve. [ABSTRACT FROM AUTHOR]

BACKGROUND: Some decades ago, the Mustard or Senning operation was used to treat patients who were born with Transposition of the Great Arteries (TGA). Although life expectancy is good, previous studies have demonstrated limitations in exercise capacity. To what extent these limitations affect day-to-day functioning of the patients is not yet known. AIMS: We therefore investigated self-reported physical activities in patients after the Mustard or Senning operation, compared this with those of matched, healthy counterparts, and explored potential associations with age, gender and complexity of the TGA. METHODS: Seventy patients were matched with healthy controls. Habitual physical activity was measured using the Baecke questionnaire, resulting in a work, sports, and leisure time index, which ranges from 1 to 5. RESULTS: The median index scores of patients were 2.63 for work, 2.50 for sports; and 2.75 for leisure time. Only for sports, a significantly lower index score was found in patients. Furthermore, we found that older patients were less frequently engaged in sport activities (Spearman's rho=-0.38; p=0.02). CONCLUSION: The results showed that the level of physical activities of patients after the Mustard and Senning operation is normal, except for sport activities. This indicates that patients after a Mustard or Senning operation tend to partake in all activities that they can comfortably perform. [ABSTRACT FROM AUTHOR]

In a retrospective study of 32 consecutive patients undergoing a total cavopulmonary connection (TCPC), we tried to determine if the trend for decreasing age at Fontan completion and reducing the intervals between the staged procedures during the past decade was associated with a change in morbidity and outcome. In 8 patients the Fontan circulation was completed in one stage and in 24 patients an intermediate step by hemi-Fontan or bidirectional cavopulmonary anastomosis was performed before Fonlan completion. Mean age at TCPC and mean interval since the previous palliation have decreased significantly during the past decade. Although major complications were significantly reduced over time the occurrence and duration of postoperative pleural effusions were not. Decreasing age as well as intervals in staged Fontan palliation have beneficial influence on major complications and outcome, without significantly affecting the duration of pleural effusions at Fontan completion. [ABSTRACT FROM AUTHOR]

Left ventricular non-compaction (cardiomyopathy) (LVN(C)) is a rare hereditary cardiac condition, resulting from abnormal embryonic myocardial development. While it mostly occurs as an isolated condition, association with other cardiovascular manifestations such as Ebstein anomaly (EA) has been reported. This congenital heart defect is characterized by downward displacement of the tricuspid valve and leads to diminished ventricular size and function. In an autosomal dominant LVN(C) family consisting of five affected individuals, of which two also presented with EA and three with mitral valve insufficiency, we pursued the genetic disease cause using whole exome sequencing (WES). WES revealed a missense variant (p.Leu113Val) in TPM1 segregating with the LVN(C) phenotype. TPM1 encodes α-tropomyosin, which is involved in myocardial contraction, as well as in stabilization of non-muscle cytoskeletal actin filaments. So far, LVN(C)-EA has predominantly been linked to pathogenic variants in MYH7. However, one sporadic LVN(C)-EA case with a de novo TPM1 variant has recently been described. We here report the first LVN(C)-EA family segregating a pathogenic TPM1 variant, further establishing the association between EA predisposition and TPM1-related LVN(C). Consequently, we recommend genetic testing for both MYH7 and TPM1 in patients or families in which LVN(C)/non-compaction and EA coincide., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

This report describes the feasibility and safety of cardiac catheterization in a developing country without access to a regular cardiac catheterization laboratory. The equipment used for imaging consisted of a monoplane conventional C-arm X-ray system and a portable ultrasound machine using the usual guidewires and catheters for cardiovascular access. In this study, 30 patients, including 17 children younger than 2 years and 2 adults, underwent catheterization of the following cardiac anomalies: patent ductus arteriosus (20 patients) and pulmonary valve stenosis (9 patients, including 2 patients with critical stenosis and 3 patients with a secundum atrial septal defect). Except for two cases requiring surgery, the patients were treated successfully without complications. They all were discharged from hospital, usually the day after cardiac catheterization, and showed significant clinical improvement in the follow-up evaluation. Cardiac catheterization can be performed safely and very effectively in a country with limited resources. If patients are well selected, this mode of treatment is possible without the support of a sophisticated catheterization laboratory.

Cyanosis after Fontan surgery or surgery for total cavopulmonary connection (TCPC), due to different types of communications (fenestration, venovenous collaterals or fistula), is not uncommon. We present the case of an 8-year-old girl presenting with increasing cyanosis during exercise 4 years after an intracardiac TCPC with lateral tunnel. Angiography showed a fistulous trajectory originating at the superior vena cava towards the base of the right atrial appendage. Due to the difficult anatomy in our patient, closure with conventional devices was not possible. We finally used a new Amplatzer PDA II AS plug to successfully close the fistula. In conclusion, closure of lateral baffle leak and device choice in case of difficult anatomy can be cumbersome. The new PDA II AS type plug can offer an elegant alternative for successful closure of some fistula.