Arrhythmogenic right ventricular cardiomyopathy presenting as heart failure in a child.

Arrhythmogenic right ventricular cardiomyopathy is an uncommon diagnosis in the paediatric population, most commonly presenting with arrhythmia. We report an 11-year-old male presenting with right heart failure due to biventricular systolic dysfunction found to have arrhythmogenic right ventricular cardiomyopathy with de novo Desmin and MYH7 mutations.

Repair of a mixed form of supracardiac total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection is a rare congenital heart defect. We report an infant with a mixed form of supracardiac TAPVC, in whom all pulmonary veins, except the right upper, entered a pulmonary venous confluence that is connected to a vertical vein and drained into the superior vena caval-right atrial junction. Several segmental right upper pulmonary veins entered the superior vena cava, superior to the entry of the vertical vein. Surgical repair consisted of the Warden procedure combined with direct anastomosis of the vertical vein to the left atrium. Separate pulmonary venous drainage pathways decreased the risk of post-operative pulmonary venous obstruction. Our patient had an uneventful post-operative course and encouraging 2-month follow-up echocardiography. Careful follow-up is warranted to detect post-operative complications, including obstruction of the pulmonary venous and cavoatrial anastomoses.

Pediatric malignancies: Is the prechemotherapy left ventricular function normal?

PURPOSE: We compared the left ventricular (LV) systolic function in children with cancer before initiation of chemotherapy with matched controls using speckle tracking echocardiography. METHODS AND RESULTS: In this retrospective study, we analyzed the echocardiograms of 89 cancer patients before the initiation of chemotherapy and 82 age- (8.4 ± 5.2 vs. 8.9 ± 3.9 years, P = .4) and gender-matched (64% vs. 67%, males, P = .4) healthy controls. Peak systolic LV longitudinal strain (LS) was significantly lower in cancer patients in apical two (-19.8 ± 3.0 vs. -23.5 ± 4.0, P < .001), three (-19.4 ± 3.2 vs. -23.4 ± 4.0, P < .001), and four-chamber views (-19.7 ± 3.4 vs. -22.5 ± 3.0, P < .001) compared to controls, as was global longitudinal strain (GLS) (-19.8 ± 2.7 vs. -23.4 ± 3.2, P < .001). The prechemotherapy group also had a higher E/e' ratio compared to controls at the septal (9.3 ± 3.9 vs. 7.9 ± 1.7, P = .005) and lateral annulus (7.9 ± 3.3 vs. 5.9 ± 1.4, P < .001) of the mitral valve. The LV ejection fraction was lower in cancer patients compared to controls (63.5 ± 4.9 vs. 66.8 ± 4.1, P < .001), although still within normal limits. There were no differences in LV myocardial performance index (0.30 ± 0.05 vs. 0.30 ± 0.09, P < .65) and shortening fraction (35.8 ± 5.2 vs. 36.1 ± 6.1, P < 0.75) between the two groups. Subgroup analysis showed no difference in LV GLS between patients with solid tumors (n = 56) and blood cancers (n = 33) (GLS -19.2 ± 2.9 vs. 19.5 ± 2.4, P > 0.05). CONCLUSION: Our data demonstrating abnormalities in LV GLS in pediatric cancer patients even prior to initiation of chemotherapy are novel and perplexing. Further longitudinal follow-up is required to assess the implications of this abnormal LV function in these patients.

Left ventricle segmental function in childhood cancer survivors using speckle-tracking echocardiography.

AIM: Anthracycline-associated cardiotoxicity in childhood cancer survivors may relate to global or segmental left ventricular abnormalities from associated thromboembolic events and myocardial microinfarcts. We characterized left ventricular segmental changes by two-dimensional speckle-tracking echocardiography in anthracycline-treated asymptomatic childhood cancer survivors. METHODS AND RESULTS: Childhood cancer survivors' echocardiograms with normal left ventricular fractional shortening >1 year after anthracycline chemotherapy were studied. Cancer-free control children had normal echocardiograms. Apical two-, three-, and four-chamber peak systolic left ventricular longitudinal and global longitudinal strain, and peak systolic left ventricular radial and circumferential strain at papillary muscle levels were analyzed. The mean (standard deviation) age was 12.7 (3.8) years in 41 childhood cancer survivors. The median (interquartile range) follow-up after anthracycline chemotherapy was 4.73 (2.15-8) years. The median (range) cumulative anthracycline dose was 160.2 (60-396.9) mg/m2. In childhood cancer survivors, the mean (standard deviation) left ventricular longitudinal strain was lower in two- (-18.6 [3.2] versus -21.3 [2.5], p < 0.001), three- (-16.3 [6.0] versus -21.7 [3.0], p < 0.001), and four- (-17.6 [2.7] versus -20.8 [2.0], p < 0.001) chamber views compared to controls. The left ventricular global longitudinal strain (-17.6 [2.7] versus -21.3 [2.0]) and circumferential strain (-20.8 [4.3] versus -23.5 [2.6], p < 0.001) were lower in childhood cancer survivors. Among childhood cancer survivors, 12 out of 16 left ventricular segments had significantly lower longitudinal strain than controls. CONCLUSIONS: Asymptomatic anthracycline-treated childhood cancer survivors with normal left ventricular fractional shortening had lower global longitudinal and circumferential strain. The left ventricular longitudinal strain was lower in majority of the segments, suggesting that anthracycline cardiotoxicity is more global than regional.

Echocardiography of hypoplastic left heart syndrome.

Echocardiography is critical in the assessment of patients with hypoplastic left heart syndrome. Fundamental techniques and standardised approaches are useful when evaluating patients with hypoplastic left heart syndrome prenatally, after birth, and before the Norwood operation (Stage 1); after the Norwood operation, before and after the superior cavopulmonary anastomosis (Stage 2); before and after the Fontan operation (Stage 3); and for chronic surveillance after the Fontan operation. From foetal assessment to ongoing surveillance after the Fontan procedure, echocardiography remains the primary technique for cardiac monitoring in this growing population of children and adults.

Cardiovascular Evaluation of Children With Malignancies.

PURPOSE OF REVIEW: The anthracycline (AC) group of drugs is widely used for cancer chemotherapy and has improved outcomes in many childhood malignancies. However, cardiovascular complications are major causes of morbidity and mortality in AC recipients, with the greatest risk factor being a higher cumulative dosage. The purpose of this review is to describe the etio-pathogenesis and risk factors of AC induced cardiotoxicity, with emphasis on currently available and emerging modalities of non-invasive imaging in its surveillance, and to review guidelines on its prevention and treatment. RECENT FINDINGS: Presently, ejection fraction and shortening fraction derived from two-dimensional echocardiography are the most widely used parameter for monitoring of cardiac function in childhood cancer survivors. The newer speckle tracking echocardiography has shown potential to detect abnormalities in ventricular function prior to the conventional measures such as ejection fraction and shortening fraction. When available, three-dimensional echocardiography should be used as it allows for more accurate estimation of ejection fraction. Newer magnetic resonance imaging (MRI) techniques, such as delayed enhancement and T1 mapping, are useful adjuncts for cardiac evaluation in cancer survivors, especially in patients with poor echocardiographic windows. Early detection and management of cardiovascular diseases is one of the major goals in the long-term follow-up of childhood cancer survivors. In addition to conventional two-dimensional echocardiography, newer techniques such as speckle tracking echocardiography and three-dimensional echocardiography should be incorporated due to its ability to detect early changes in anthracycline-induced cardiotoxicity. However further research are needed to guide changes in management due to abnormalities in speckle tracking echocardiography.

Sports participation during teenage years.

Ensuring the safety of young athletes is a priority among health care providers. Controversy remains as to the best method of preparticipation screening. The American Academy of Pediatrics and the American Heart Association currently recommend screening with history and physical examination, without routine electrocardiogram or echocardiography. Meticulous conduction of a cardiac focused history and exam during the preparticipation evaluation can help identify those who may be at risk for sudden cardiac arrest. Understanding presenting signs of the most common cardiac diseases is useful in recognizing those in need of a directed cardiac evaluation before sports participation.

Echocardiographic diagnosis of right ventricular inflow compression associated with pectus excavatum during spinal fusion in prone position.

INTRODUCTION: Pectus excavatum is commonly viewed as a benign condition. Associated alterations in hemodynamics are rare. We present an unusual case of right ventricular inflow obstruction and hemodynamic compromise as a consequence of pectus excavatum encountered during surgical intervention. CASE: a 15-year-old male with pectus excavatum and thoracolumbar scoliosis developed severe hypotension after induction of general anesthesia and placement in the prone position for elective spinal fusion. A transesophageal echocardiogram revealed anterior compression of the right heart by the sternum with peak and mean right ventricular inflow gradients of 7 and 4 mm Hg, respectively. The gradient resolved with supine positioning and was reproduced with direct compression of the sternum. CONCLUSIONS: Although pectus excavatum is generally a benign condition, the cardiologist should be aware of the potential for serious hemodynamic compromise related to positioning in these patients.