Echocardiographic two-dimensional speckle tracking identifies acute regional myocardial edema and sub-acute fibrosis in pediatric focal myocarditis with normal ejection fraction: comparison with cardiac magnetic resonance.

The aim here was to describe the role of speckle tracking echocardiography (STE), in identifying impairment in systolic function in children and adolescents with focal myocarditis and without reduction in ejection fraction. We describe data from 33 pediatric patients (age 4-17 years) admitted for focal myocarditis, confirmed by cardiac magnetic resonance (CMR), and without impaired ejection fraction and/or wall motion abnormalities. All children underwent Doppler echocardiography examination with analysis of global (G) and segmental longitudinal strain (LS) and CMR for the quantification of edema and myocardial fibrosis. Reduction in LS was defined according to age-specific partition values. At baseline, impaired GLS was present in 58% of patients (n = 19), albeit normal ejection fraction. LS was also regionally impaired, according to the area of higher edema at CMR (i.e. most impaired at the level of the infero-lateral segments as compared to other segments (p < 0.05). GLS impairment was also moderately correlated with the percentage edema at CMR (r = - 0.712; p = 0.01). At follow-up, GLS improved in all patients (p < 0.001), and normal values were found in 13/19 patients with baseline reduction. Accordingly persistent global and regional impairment was still observed in 6 patients. Patients with persistent LS reduction demonstrated residual focal cardiac fibrosis at follow-up CMR. Both global and regional LS is able to identify abnormalities in systolic longitudinal mechanics in children and adolescents with focal myocarditis and normal ejection fraction. The reduction in LS is consistent with edema amount and localization at CMR. Furthermore, LS identifies regional recovery or persistent cardiac function impairment, possibly related to residual focal fibrosis.

Evidence of impaired longitudinal strain in pre-Fontan palliation in functional single left ventricle.

BACKGROUND: We evaluated two-dimensional speckle-tracking echocardiography longitudinal strain (L2DSE) in functionally single left ventricles (LV). METHODS: We retrospectively analyzed 21 patients with functionally single LV. We divided patients into two groups according to pre-Fontan cardiac catheterization data: group 1, adequate data for Fontan procedure and group 2, inadequate data. RESULTS: LV strain correlated with predicted pressure in the Fontan system (r = 0.64; P = 0.003), pressure in the Glenn system (r = 0.57; P = 0.010), and transpulmonary gradient (r = 0.59; P = 0.008), but not with left atrial pressure (r = 0.292; P = 0.226) or ejection fraction (r = 0.254; P = 0.294). In multiple regression analysis, four-chamber LV strain was correlated with predicted pressure in the Fontan (ß = 0.642: P = 0.003), whereas no association was found with LV ejection fraction (ß = 0.254; P = 0.294), or time from Glenn palliation (ß = 0.082; P = 0.893). When dividing the population into two groups according to catheterization data, significantly lower four-chamber longitudinal 2DSE (-25.8 ±â€Š3.2 vs.-19.5 ±â€Š5.1; P = 0.004) was found to be not suitable for the Fontan procedure, as compared with those who underwent successful Fontan completion. CONCLUSION: Regardless of ejection fraction, four-chamber L2DSE is associated with high predicted pressure in the Fontan system. Patients with hemodynamic data unsuitable for Fontan operation have significantly lower mean longitudinal strain parameters. 2DSE may represent a valuable tool in assessing patients with single LV physiology and might provide useful pre-Fontan information.

Dual-source computed tomography coronary artery imaging in children.

Computed tomography (CT) has a well-established diagnostic role in the assessment of coronary arteries in adults. However, its application in a pediatric setting is still limited and often impaired by several technical issues, such as high heart rates, poor patient cooperation, and radiation dose exposure. Nonetheless, CT is becoming crucial in the noninvasive approach of children affected by coronary abnormalities and congenital heart disease. In some circumstances, CT might be preferred to other noninvasive techniques such as echocardiography and MRI for its lack of acoustic window influence, shorter acquisition time, and high spatial resolution. The introduction of dual-source CT has expanded the role of CT in the evaluation of pediatric cardiovascular anatomy and pathology. Furthermore, technical advances in the optimization of low-dose protocols represent an attractive innovation. Dual-source CT can play a key role in several clinical settings in children, namely in the evaluation of children with suspected congenital coronary artery anomalies, both isolated and in association with congenital heart disease. Moreover, it can be used to assess acquired coronary artery abnormalities, as in children with Kawasaki disease and after surgical manipulation, especially in case of transposition of the great arteries treated with arterial switch operation and in case of coronary re-implantation.

New findings concerning cardiovascular manifestations emerging from long-term follow-up of 150 patients with the Williams-Beuren-Beuren syndrome.

AIMS: We investigated the prevalence, type, and course of congenital cardiac defects and systemic hypertension in our patients with Williams-Beuren-Beuren syndrome. METHODS AND RESULTS: We reviewed the clinical records of all patients with Williams-Beuren syndrome examined between 1981 and 2006. We identified 150 patients, aged from 7 months to 45 years, with a follow-up from 6 months to 25 years, the mean being 6.4 years. A cardiac anomaly was present in 113 of the 150 patients (75%). Defects were typical in over four-fifths of the group. We found supravalvar aortic stenosis in 73 of 113 patients (64.6%), isolated in 43. Pulmonary stenosis, isolated in 18 cases, was found in 51 of 113 (45.1%), while aortic coarctation and mitral valvar prolapse were each found in 7 (6.2%), 3 of the lesions is isolation. Atypical defects were found in 19 patients, tetralogy of Fallot in 2, atrial septal defects in 4, aortic and mitral valvar insufficiencies in 1 each, bicuspid aortic valves in 2, and ventricular septal defects in 9, 4 of the last being isolated. Systemic hypertension, observed in 33 patients (22%), was poorly controlled in 10. Diagnostic and/or interventional cardiac catheterization was undertaken in 24 patients, with 30 surgical procedures performed in 26 patients. Of the group, 3 patients died. CONCLUSION: Cardiac defects were present in three-quarters of our patients. Pulmonary arterial lesions generally improved, while supravalvar aortic stenosis often progressed. Atypical cardiac malformations, particularly ventricular septal defects, occurred frequently. Systemic hypertension was found in one-fifth, even in the absence of structural cardiac defects. The short-term mortality was low.

Echocardiographic Assessment of Ebstein's Anomaly in a 60-Year-Old Man.

We present an echocardiographic evaluation of an elderly man affected with Ebstein's anomaly. In the natural history of this congenital disease only 5% of patients survive beyond the fifth decade. The patient presented severe right heart failure and he was refered to our institution for heart transplantation.

Impact of three-dimensional echocardiography in complex congenital heart defect cases: the surgical view.

Other authors have demonstrated the ability of three-dimensional (3D) echocardiography to produce "en face" views of anomalies such as atrioventricular valve disease and atrial and ventricular septal defects. Few data exist about the usefulness of 3D images for more complex congenital heart defects and the surgical impact of this relatively new technology. This study, covering a period of 8 months and including 43 young patients affected by complex congenital heart defects, demonstrated that the routine use of 3D echocardiography is feasible and valuable for some types of cardiac defects. In fact, 3D images have provided more detailed anatomic definition of interrelations between structures in about one-third (15/43) of our cases, yielding new insight into the anatomy analogous to what can be derived from examining a heart specimen. Our surgeons found the 3D images particularly helpful for providing a realistic and almost specimen-like preview of the surgical anatomy that facilitates planning of the surgical program.

Acute ST elevation myocardial infarction in pregnancy due to coronary vasospasm: a case report and review of literature.

Acute myocardial infarction during pregnancy is rare but may be associated with high risk complications. We present a very rare case about an acute myocardial infarction with anterior ST elevation in a 40-year-old woman, at 38th week of gestational period. The coronary arteriograms showed a diffuse left coronary vasospasm. The coronary arteriography at puerperium showed no organic narrowings. This is the first case with a well documented coronary artery vasospasm in pregnancy. Management should follow the usual principles of care for acute myocardial infarction. However, selection of diagnostic and therapeutic approaches may be greatly influenced by fetal safety.

Echocardiography diagnosis of ruptured congenital right coronary sinus of Valsalva aneurysm into right ventricle.

We describe a case of ruptured aneurysm of the right coronary sinus of Valsalva (ASV) diagnosed by transthoracic two-dimensional echocardiography which allowed to quickly establish a correct diagnosis in a patient with a recent onset of continuous murmur and acute right congestive heart failure.

Outcome of percutaneous hybrid coronary revascularization: bare metal stents jeopardize the benefit of sirolimus-eluting stents in the real world.

BACKGROUND: In an effort to contain procedural costs while limiting the risk of in-stent restenosis, hybrid percutaneous revascularization (ie, stenting with at least one sirolimus-eluting stent [SES] and at least one bare metal stent [BMS] in the same patient) is felt to be a cost-effective alternative to exclusive SES use. OBJECTIVE: To describe the outcome of hybrid percutaneous revascularization for the treatment of patients with multiple coronary artery lesions. METHODS AND RESULTS: Fifty-six patients (42 men; mean age [+/- SEM] 64+/-2) underwent hybrid stenting (average of 1.2 SES/patient and 1.3 BMS/patient). SES were used to treat lesions at higher restenotic potential, including longer lesions, smaller target vessels and bifurcation lesions (mean stent length [+/- SEM] was 21.1+/-1.2 mm for SES and 16.0+/-0.6 mm for BMS; stent diameter mean [+/- SEM] was 2.9+/-0.0 mm for SES and 3.1+/-0.1 mm for BMS; bifurcation lesions were 43% for SES and 7% for BMS; all P<0.01). At nine months of clinical follow-up, no death or myocardial infarction was reported. Twenty-one patients underwent clinically driven repeat coronary angiography at a mean (+/- SEM) of 8+/-1 of months (range two to 12 months) follow-up. Target lesion revascularization procedures were recorded in six patients (11%) for nine lesions (6%). Of these lesions, seven were categorized after blinded analysis as due to in-BMS restenosis and two to in-SES restenosis (P=0.01); three patients (5.4%) underwent reangioplasty for de novo lesions. There was one case of acute in-SES thrombosis. SES showed significantly less neointimal hyperplasia (late lumen loss was 0.4+/-0.1 mm for SES and 1.3+/-0.1 mm for BMS; loss index was 0.15+/-0.05 for SES and 0.48+/-0.05 for BMS; all P<0.001). CONCLUSIONS: The use of SES resulted in less neointimal hyperplasia even when used to treat lesions at higher risk for restenosis based on angiographic characteristics. BMS implantation significantly limits this beneficial effect, compromising the outcome of hybrid percutaneous coronary revascularization.

Skin reflectance changes in preterm infants with patent ductus arteriosus.

INTRODUCTION: Patent ductus arteriosus (PDA) is a common complication in preterm infants. While two-dimensional echocardiography is the method of choice for diagnosing a PDA, clinical signs are known to be of limited value. STUDY DESIGN: Haemodynamically significant PDA (hs-PDA) was defined as a ductal left-to-right shunt with elevated left atrial/aortic root ratio ( > 1.6:1), a ductal diameter > 2 mm, retrograde diastolic flow exceeding 30% of the anterograde flow and anterograde peak diastolic flow velocity in left pulmonary artery > 50 cm/s. A hs-PDA may affect the cerebral circulation and skin color is known to be related to local perfusion. In this study, we tested the value of a caudal-to-cephalic skin reflectance differential in detecting preterm infants with hs-PDA. The study was blinded and prospective. SUBJECTS: Fifteen infants with a hs-PDA (M: 8, F: 7; gestational age: 28.0 +/- 2.0 weeks, birth weight: 880 +/- 130 g) and 15 gender- and gestational age-matched infants without a haemodynamically significant PDA (M: 8, F: 7; gestational age: 28.2 +/- 2.3 weeks, birth weight: 885 +/- 135 g) participated to the study. OUTCOME MEASURE: Skin reflectance measurements were performed using a reflectance spectrophotometer (CM-2600d/2500d, Minolta, Osaka, Japan). Sole ("postductal" site) to forehead ("preductal" site) skin reflectance ratio (caudal-to-cephalic ratio). RESULTS: hs-PDA infants showed significantly lower forehead light reflectance values on for the whole visible spectrum (p < or = 0.043) with the exception of 580-590 nm (p = 0.058), whereas no statistically significant differences were observed for the sole skin reflectance between the two groups in the examined visible spectrum. Consequently, hs-PDA infants showed a significantly higher caudal to cephalic ratio in the 410-430 nm (p < or = 0.042), 460-530 nm (p < or = 0.027) and 590-700 nm (p < or = 0.011) wavelength ranges. CONCLUSIONS: These findings may provide a previously unrecognised clinical marker for haemodynamically significant PDA in preterm infants, thus predicting those preterm infants who may require intervention for PDA.

Three-vessel left-ventricular microfistulization syndrome: a rare case of angina.

A 55-year-old woman with angina was found to have multiple fistulae from all three major coronary arteries to the left ventricle. The aortography mimicked a severe aortic insufficiency. This rare vascular anomaly can cause a coronary steal phenomenon and subsequent myocardial ischemia in patients without angiographic evidence of major atherosclerotic coronary artery disease.