Search

Background: To investigate deep vein thrombosis (DVT) in hospitalized patients with coronavirus disease 2019 (COVID-19), we performed a single institutional study to evaluate its prevalence, risk factors, prognosis, and potential thromboprophylaxis strategies in a large referral and treatment center. Methods: We studied a total of 143 patients with COVID-19 from January 29, 2020 to February 29, 2020. Demographic and clinical data, laboratory data, including ultrasound scans of the lower extremities, and outcome variables were obtained, and comparisons were made between groups with and without DVT. Results: Of the 143 patients hospitalized with COVID-19 (age 63±14 years, 74 [51.7%] men), 66 patients developed lower extremity DVT (46.1%: 23 [34.8%] with proximal DVT and 43 [65.2%] with distal DVT). Compared with patients who did not have DVT, patients with DVT were older and had a lower oxygenation index, a higher rate of cardiac injury, and worse prognosis, including an increased proportion of deaths (23 [34.8%] versus 9 [11.7%]; P =0.001) and a decreased proportion of patients discharged (32 [48.5%] versus 60 [77.9%]; P P =0.031), Padua prediction score ≥4 (odds ratio, 4.016; P =0.04), D-dimer >1.0 μg/mL (odds ratio, 5.818; P 1.0 μg/mL has a sensitivity of 88.52% and a specificity of 61.43% for screening for DVT. In the subgroup of patients with a Padua prediction score ≥4 and whose ultrasound scans were performed >72 hours after admission, DVT was present in 18 (34.0%) patients in the subgroup receiving venous thromboembolism prophylaxis versus 35 (66.0%) patients in the nonprophylaxis group ( P =0.010). Conclusions: The prevalence of DVT is high and is associated with adverse outcomes in hospitalized patients with COVID-19. Prophylaxis for venous thromboembolism may be protective in patients with a Padua protection score ≥4 after admission. Our data seem to suggest that COVID-19 is probably an additional risk factor for DVT in hospitalized patients.

In patients with disabling symptoms caused by hypertrophic obstructive cardiomyopathy (HOCM), echocardiography-guided percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) could be a less invasive treatment option.This study aimed to investigate the safety and efficacy of the PIMSRA for left ventricular outflow tract (LVOT) gradient reduction in HOCM.The study enrolled 15 patients with HOCM. These patients underwent electrocardiography, imaging, and blood biochemistry examination over 6 months of follow-up.At 6 months of follow-up, patients showed significant reductions in peak LVOT gradients (resting gradient: from 88.00 [66.00] mm Hg to 11.00 [6.00] mm Hg; p = 0.001; stress-induced gradient: from 117.00 [81.00] mm Hg to 25.00 [20.00] mm Hg; p = 0.005) and interventricular septum (IVS) thickness (anterior IVS: from 25.00 [21.00] mm to 14.00 [12.00] mm; p = 0.001; posterior IVS: from 24.00 [21.00] mm to 14.00 [11.50] mm; p = 0.001). The reductions in IVS thickness and LVOT gradients were associated with improvement in New York Heart Association functional classification (from 3.00 [2.00] to 1.00 [1.00]; p 0.001), total exercise time (from 6.00 [5.50] min to 9.00 [8.00] min; p = 0.007), and pro B-type natriuretic peptide levels (from 924.00 [370.45] pg/ml to 137.45 [75.73] pg/ml; p = 0.028). No patient had bundle branch block or complete heart block.PIMSRA is a safe and effective treatment approach for severe, symptomatic HOCM and results in sustained improvement in exercise capacity, persistent reduction in LVOT gradient, and sustained improvement in cardiac function.

Objectives We sought to investigate pulmonary hypoplasia (PH) and associated risk factors in fetuses with congenital conotruncal defect (CTD). Methods A total of 75 fetuses with CTD (gestational age (GA): 22-32 weeks) and 150 normal GA-matched fetuses as the control group were studied. We measured diameters of aorta (Ao); main, left, and right pulmonary artery (PA); and their Z-scores by fetal echocardiography (FE). We also measured the lung area, lung area/chest area ratio (LCR), lung-to-head circumference ratio (LHR), right lung area/head circumference2 (quantitative lung index, QLI), and Z-scores. Results The PA, left pulmonary artery (LPA), RPA, and their Z-scores and the lung area measurements (except for QLI) were significantly smaller in the CTD group, compared with the normal control group. Subgroup analysis showed the following: (1) CTD with right ventricular outflow tract obstruction (RVOTO) had smaller main and branch PA dimensions and small lung areas in contrast to CTD without RVOTO. (2) CTD with pulmonary atresia had smaller LPA and RPA dimensions but no difference in lung areas. (3) In the common types of CTD, lung area variables were mostly smaller in double outlet right ventricle (DORV) and tetralogy of Fallot (TOF), but there was no difference in transposition of the great arteries (TGA). Conclusions CTD is associated with hypoplastic main and branch pulmonary arteries as well as PH. Risk factors for PH included defect types (DORV and TOF but not TGA) and RVOTO. It is plausible that pulmonary blood flow, rather than the type of CTD, RVOTO, or pulmonary artery dimensions, plays a key role in PH.

BACKGROUND AND OBJECTIVE It is hypothesized that diminished cerebral vascular resistance or the "brain sparing effect" is associated with fetuses with complex congenital heart defects (CHD) and may affect their neurodevelopmental outcome. An alternative explanation is that it is related to the location, cardiac output, pressure, and resistance in left heart obstructive CHDs. We sought to determine the effects of various left and right heart obstructive defects on the cerebral and placental hemodynamics and to evaluate the utility of these variables for the assessment and prognosis of CHDs. METHODS A total of 290 fetal echocardiograms were reviewed, including 91 fetuses with CHD and 199 normal ones. The CHD fetuses were divided into four groups, that is, left-sided obstructive lesions (LSOL), hypoplastic left heart syndrome (HLHS), right-sided obstructive lesions (RSOL), and hypoplastic right heart syndrome (HRHS). The pulsatility index of middle cerebral artery (MCA-PI) and umbilical artery (UA-PI) were measured by pulse-wave Doppler, and their Z scores were also derived. Cerebroplacental ratio (CPR) was calculated as: CPR=MCA-PI/UA-PI. RESULTS There was no significant difference in MCA-PI between the 4 CHD and normal control groups (P>.05). However, MCA-PI and Z score decreased in aortic stenosis but not in interrupted aortic arch or coarctation subgroups, whereas they increased in pulmonary atresia but not pulmonary stenosis or tetralogy of Fallot subgroups compared with normal group (P

This study was designed to determine the long-term safety and efficacy of using modified double-disk occluders for perimembranous ventricular septal defect (pmVSD) closure in adults. From January 2004 to December 2014, 337 adults with pmVSDs were treated through transcatheter intervention using 2 types of double-disk occluders; 302 patients received a symmetrical concentric pmVSD occluder, and 35 patients received an asymmetrical concentric pmVSD occluder. All patients were followed up through electrocardiography and transthoracic echocardiography until June 2015. The success rate was 100% for both procedures. During the median 71-month follow-up period, no cases of infective endocarditis, cerebrovascular accidents, heart failure, or death occurred. Two major adverse events (0.6%) were recorded: complete atrioventricular block requiring surgical treatment in one patient and severe tricuspid valvular regurgitation requiring surgical repair in another patient. Cardiac conduction block was the most common minor adverse event. The mean left ventricular (LV) end-diastolic volume decreased from 96.6 ± 23.2 ml before intervention to 86.0 ± 22.0 ml (p

In this Special Issue of the Journal, 8 review articles that represent the new developments and applications of fetal echocardiography and fetal cardiology for diagnosis, prognosis, and treatment of fetal cardiovascular disease are included. The goal was to provide an updated review of the evidence for the current and emerging use of fetal echocardiography and cardiac magnetic resonance, improved diagnosis of challenging congenital heart disease, new tools for evaluation of fetal systolic and diastolic function, better prognosis and risk stratification of newborns with congenital heart diseases, and new and promising therapies for fetuses with cardiovascular disease.

BACKGROUND The previous literature on common pulmonary vein atresia (CPVA) mainly consists of neonatal case reports. There is a lack of research on the prenatal diagnosis of CPVA. METHODS We conducted a retrospective study of all fetuses with CPVA confirmed by autopsy between August 2010 and May 2018. Prenatal echocardiographic features, autopsy findings, and genetic test results were analyzed. We compared fetal CPVA with total anomalous pulmonary venous return (TAPVR) and neonatal CPVA. RESULTS During the study period, fetal echocardiography was performed on 31 617 fetuses. Six cases of CPVA were identified by autopsies, including 1 case performed with a cardiovascular cast. All 6 cases (100%) had asplenia syndrome (AS) and bilateral superior vena cava (BSVC). In 1 case (16.7%), the prenatal ultrasound results were in complete agreement with the postmortem findings. Four cases (66.7%) were misdiagnosed as TAPVR by prenatal echocardiography. For the remaining case (16.7%), no pulmonary venous anomalies were detected on prenatal echocardiography. No aneuploidy was identified in any of the cases. There were no statistically significant differences among the proportions of associated complex anomalies and AS between the fetal CPVA and TAPVR groups. The proportion of associated complex anomalies and AS in the fetal CPVA group was higher than that in the neonatal group (P

Background Premature restriction or closure of foramen ovale (FO) in otherwise structurally normal hearts may be associated with right ventricular dilation, tricuspid regurgitation, pericardial effusion, heart failure, even poor perinatal outcomes. Data about these rare conditions are lacking. Methods We retrospectively reviewed the echocardiographic records of 9704 fetuses seen from 2010 to 2014 in Beijing Anzhen Hospital, a regional and national referral center, to ascertain the presence of restriction or closure of FO. We collected the fetal echocardiography and perinatal outcome data for this group of fetuses with restriction or closure of FO. Results In this large, single-institution cohort (n = 9704), 6707 fetuses seen between 23 and 37 weeks of gestation had normal heart structures; of these, 60 (0.89%) had restrictive FO (rFO) and 5 (0.07%) had closure of FO (cFO). Fetal echocardiographic images showed right atrial dilation in 48 (73.84%), right ventricular dilation in 38 (58.46%), tricuspid regurgitation in 19 (29.23%), and pericardial effusion in 10 (15.38%). Also in this group, 50 (83.3%) with rFO and 4 (80.0%) with cFO had follow-up data. No prenatal deaths occurred in either the rFO or the cFO group, but the neonatal mortality included 1 in the rFO group and 2 in the cFO group. Conclusion Premature rFO/cFO are rare in fetuses with otherwise structurally normal hearts. The fetal echocardiographic characteristics include right atrial and ventricular dilated, tricuspid regurgitation, and pericardial effusion. Most fetuses had a good outcome, although there was an association between rFO, especially cFO, with neonatal morality and complications (prematurity, maternal preeclampsia and placental abruption, hydrops fetalis, and necrotizing enterocolitis with perforation).

Normative ranges of fetal echocardiographic measurements are important for quantitative diagnosis of fetal cardiovascular disease. The current normative ranges were derived from small samples and were based on the hypothesis of a normal distribution of these measurements during fetal cardiovascular growth. The aims of this study were to test the hypothesis of a normal distribution of fetal echocardiographic measurements in a large multicenter cohort and to propose a reference system without the normal distribution hypothesis to improve accuracy of fetal echocardiographic measurements.Fifty-two variables from 6,343 normal fetal echocardiographic examinations were acquired from seven Chinese centers. The hypothesis of a normal distribution used in ordinary least squares regression was tested with the Jarque-Bera test. The quantile score (q score) derived from quantile regression without normal distribution hypothesis was compared with the Z score derived from ordinary least squares regression. A total of 288 fetuses with outflow tract and great artery abnormalities and 300 normal fetuses were used to compare the diagnostic accuracy of q and Z scores.All fetal echocardiographic measurements showed non-normal distributions (P .001). The normal range was underestimated by ordinary least squares regression compared with quantile regression by 30 ± 11%. The partial normalized areas under the receiver operating characteristic curve within the 20% false-positive rate were 0.62 and 0.50 for the q and Z scores, respectively.The q score provides a more robust system for determining normative ranges of fetal echocardiographic measurements. The improved sensitivity of matched false-positive rates makes the q score a more accurate reference for prenatal diagnosis, assessment, and prognosis of fetal cardiovascular disease.

Objective Prenatal diagnosis of total anomalous pulmonary venous connection (TAPVC) by fetal echocardiography (FE) remains a challenge. We sought to ascertain the diagnostic accuracy of 2D and 3D spatiotemporal image correlation (STIC) FE and the potential incremental value of 3D STIC FE for prenatal diagnosis and assessment of TAPVC. Methods This study was conducted retrospectively in a single tertiary referral center. The study population consisted of 74 TAPVC from 17 063 fetuses by FE from August 2010 to April 2016. The 3D volume acquisition was also performed by STIC. Results A total of 17 063 fetal echocardiograms in our institution were queried and 74 (0.4%) were identified with TAPVC. In the TAPVC group, 11 had postnatal echocardiographic study, 25 had autopsies, 36 declined autopsy and 2 lost for follow-up. The sensitivity of FE for diagnosis of TAPVC was 97.14%, specificity 99.98%, respectively. In our cohort, 31 fetuses were evaluated by both 2D and 3D STIC and were confirmed by postnatal echocardiography or autopsies. TAPVC was diagnosed in all 31 patients (100%) by both 2D FE and 3D STIC. Furthermore, the classification of subtypes of TAPVC and accurate diagnosis of the drainage pathway was correct in 26 (83.9%) and 24 (77.4%) cases by 2D FE, respectively; but in 31 (100%) and 31 (100%) cases by 3D STIC. Conclusion 2D and 3D STIC FE provide accurate diagnosis of TAPVC with excellent sensitivity and specificity. 3D STIC FE has incremental value in further evaluation of classification of TAPVC types and drainage pathways.

Objectives We sought to develop a hemodynamic model of aortic and ductal arches using computational fluid dynamics (CFD) and 3D/4D spatio-temporal image correlation (STIC) fetal echocardiography and to investigate the hemodynamics of coarctation of aorta (CoA) in human fetuses using this approach. Methods We obtained 3D/4D STIC fetal echocardiographic images of the aortic and ductal arches (DA) in five normal fetuses. Based on these images, we simulated the hemodynamics in the two arches using CFD. Subsequently, we reduced the dimensions of aortic isthmus from 100% to 85%, 70%, 55%, 40%, and 25% of the original dimension digitally. Numerical simulation was repeated in each condition, and flow profile, velocity, pressure, and wall shear stress (WSS) were compared with those of the baseline normal aortic and ductal arches. Results With the progressive narrowing in the aortic isthmus, there were alterations in the flow profile, velocity, pressure, and WSS. The downstream vortexes disappeared, and the double helix profile became single helix. When the aortic isthmus reduced by 55% in dimension, there was an exponential increase in velocity and WSS and decrease in pressure. Conclusions The aortic and ductal arch geometry and flow lead to the alterations in flow profile, velocity, pressure, and WSS in the aortic isthmus in normal and CoA models, which are conductive of ductal issue migration into these areas. A 55% reduction in the dimension of aortic isthmus is associated with exponential change in velocity, pressure, and WSS, a probable threshold for hemodynamically significant CoA.

Diabetic cardiomyopathy (DCM) is the leading cause of morbidity and mortality among the diabetic patients and currently there is no effective means to reverse its pathological progress. Basic fibroblast growth factor (bFGF) has shown promise as a molecular therapy for DCM, but its delivery is inefficient and non-specific. In the present study, a therapy combining nanoparticle (NP) carrier and ultrasound-targeted microbubble destruction (UTMD) was reported the first time for bFGF delivery to the heart of diabetic rats. bFGF-loaded NP (bFGF-NP) were prepared with Poloxamer 188-grafted heparin copolymer using water-in-water technique, and the morphology, encapsulation efficiency, and bioactivity of bFGF-NP were studied. The cellular uptake and cytotoxicity of bFGF-NP were evaluated with primary cultures of the left ventricular (LV) cardiomyocytes in vitro. Therapeutic effects of bFGF-NP/UTMD on the heart of DCM rats were studied by measuring LV systolic and diastolic functions, hemodynamic characteristics and indicators of cardiac remodeling including myocardial collagen volume fraction and capillary density. Results demonstrated that bFGF-NP showed good round morphology, efficient bFGF encapsulation and stable bioactivity of bFGF in vitro. bFGF-NP/UTMD combined treatment significantly enhanced the efficiency of bFGF cellular uptake (P

Objectives The objective of this study was to evaluate the safety and efficacy of the surgical versus transcatheter approach to correct perimembranous ventricular septal defects (pmVSDs) in a prospective, randomized, controlled clinical trial. Background pmVSD is a common congenital heart disease in children. Surgical closure of pmVSD is a well-established therapy but requires open-heart surgery with cardiopulmonary bypass. Although the transcatheter approach is associated with significant incidence of complete atrioventricular block, it may provide a less invasive alternative. Critical comparison of the safety and efficacy of the 2 interventions necessitates a prospective, randomized, controlled trial. Methods Between January 2009 and July 2010, 229 children with pmVSD were randomly assigned to surgical or transcatheter intervention. Clinical, laboratory, procedural, and follow-up data over a 2-year period were compared. Results Neither group had mortality or major complications. However, statistical analysis of the 2 groups demonstrated significant differences (p Conclusions Transcatheter device closure and surgical repair are effective interventions with excellent midterm results for treating pmVSD in children. Transcatheter device closure has a lower incidence of myocardial injury, less blood transfused, faster recovery, shorter hospital stay, and lower medical expenses. (Transcatheter Closure Versus Surgery of Perimembranous Ventricular Septal Defects; NCT00890799 )

Quantitative measurement of left ventricular (LV) volumes, mass, and function is one of the most common and important indications for echocardiography. These measurements are among the most powerful tools for diagnosis and prognosis of congenital and acquired heart diseases and for assessment of medical, percutaneous, and surgical interventions. Awareness is also growing of the importance of right ventricular (RV) volume, mass, and function in many cardiopulmonary diseases. Furthermore, there are challenges and opportunities to measure the volume, mass, and function of complex chambers such as the left atrium, right atrium, and the univentricular heart. As echocardiography continues to be the imaging modality of choice for these measurements, the strengths and limitations of M-mode, two-dimensional (2D), and recently three-dimensional (3D) echocardiographic (3DE) methodologies for accurate and reproducible measurement of these indices have been extensively investigated for congenital and acquired heart diseases. Evidence suggests that 3DE provides improved accuracy and reproducibility over 2D methods for measurement of LV volume and function calculation in adults and in children. Data have accumulated on the utility of 3DE for measuring chamber volumes and function for the RV and for the single ventricle, which may become more widely used in clinical and research arenas in the future. Finally, new advanced modes of analysis such as 3D strain and synchrony analysis by 3DE are promising methodologies that warrant further investigation.

Objective To evaluate the value and improve the diagnostic accuracy of echocardiography in the diagnosis of a sinus of Valsalva aneurysm (SVA) with rare pathological patterns. Methods Echocardiographic features and surgical findings from 270 Chinese patients with SVA treated in the last 18 years (1995–2013) at the Union Hospital were compared retrospectively; 22 of 270 cases had rare patterns. Results The patients with SVA, a rare origin, a rare extending position, or a rare course accounted for 3.4%, 7.4%, and 0.4% of the 270 cases, respectively. The three most common aneurysmal complications of the patients with rare patterns were severe aortic regurgitation (16), obstruction of the ventricular outflow tract or valvular orifice (3), and conduction disturbance (3). The origin, course, extending position and rupture status of the SVAs determined by echocardiography were entirely consistent with surgical findings in 81.8% of the 22 cases. With the exception of one failed diagnosis of an aneurysmal wall dissection and one misdiagnosis of a descending aortic dissection, the echocardiographic results of SVA complications and associated cardiovascular lesions were also confirmed. Conclusion We could accurately diagnose SVAs with rare pathological patterns by echocardiographic identification of distinguishing features. However, for several conditions, we could not accurately identify the origin or course of the aneurysm or define its relationship to adjacent structures using conventional echocardiography alone. Therefore, we recommend combining conventional echocardiography with different imaging techniques, such as transesophageal echocardiography, three-dimensional echocardiography, computed tomography angiography, and aortic angiography.

This study aimed to evaluate the role of echocardiography-based strain analysis in the early diagnosis and guidance for management of dilated cardiomyopathy (DCM). Muscular dystrophy mice (which spontaneously develop DCM) and control (C57 BL/6 J) mice were sequentially evaluated by ultrasound biomicroscopy, conventional left ventricle (LV) measurement, two-dimensional (2-D) strain analysis and myocardial histologic analysis for 12 consecutive months. Significant alternation of LV remodeling and dysfunction could be detected by conventional echocardiography after 9 mo, by strain analysis after 5 mo and by histologic analysis after 4 mo. The global longitudinal systolic peak strain (PK) was the most sensitive strain marker for early detection of myocardial structural abnormality in the subclinical stage. Moreover, losartan administration before the PK decrease was associated with significantly preserved LV function. These results suggest that myocardial strain analysis (particularly longitudinal PK) is sensitive for the early detection of LV dysfunction in mice with dilated cardiomyopathy.

We investigated a new method for combining microbubble-enhanced ultrasound cavitation (MEUC) with hemocoagulase (HC) atrox. Our goal was to induce embolic effects in the vasculature and combine these with an anti-angiogenic treatment strategy. Fourteen days after being implanted with a single slice of the liver VX2 tumor, rabbits were randomly divided into five groups: (i) a control group injected intra-venously with saline using a micropump; (ii) a group given only an injection of HC; (iii) a group treated only with ultrasound cavitation; (iv) a group treated with MEUC; (v) a group treated with MEUC + HC. Contrast-enhanced ultrasound was performed before treatment and 1 h and 7 d post-treatment to measure tumor size, enhancement and necrosis range. QontraXt software was used to determine the time–intensity curve of tumor blood perfusion and microvascular changes. At 1 h and 7 d after treatment with MEUC + HC, the parameters of the time–intensity curve, which included peak value, regional blood volume, regional blood flow and area under the curve value and which were measured using contrast-enhanced ultrasound, were significantly lower than those of the other treatment groups. The MEUC + HC treatment group exhibited significant growth inhibition relative to the ultrasound cavitation only, HC and MEUC treatment groups. No damage was observed in the surrounding normal tissues. These results support the feasibility of reducing the blood perfusion of rabbit VX2 liver tumors using a new method that combines MEUC and HC.

Background and Objective The specific aim of this study was to evaluate the feasibility, reproducibility and maturational changes of LV rotation, twist and torsion variables by real-time 3D speckle-tracking echocardiography (RT3DSTE) in children. Methods A prospective study was conducted in 347 consecutive healthy subjects (181 males/156 females, mean age 7.12 ± 5.3 years, and range from birth to 18-years) using RT 3D echocardiography (3DE). The LV rotation, twist and torsion measurements were made off-line using TomTec software. Manual landmark selection and endocardial border editing were performed in 3 planes (apical “2”-, “4”-, and “3”- chamber views) and semi-automated tracking yielded LV rotation, twist and torsion measurements. LV rotation, twist and torsion analysis by RT 3DSTE were feasible in 307 out of 347 subjects (88.5%). Results There was no correlation between rotation or twist and age, height, weight, BSA or heart rate, respectively. However, there was statistically significant, but very modest correlation between LV torsion and age (R2 = 0.036, P< 0.001). The normal ranges were defined for rotation and twist in this cohort, and for torsion for each age group. The intra-observer and inter-observer variabilities for apical and basal rotation, twist and torsion ranged from 7.3% ± 3.8% to 12.3% ± 8.8% and from 8.8% ± 4.6% to 15.7% ± 10.1%, respectively. Conclusions We conclude that analysis of LV rotation, twist and torsion by this new RT3D STE is feasible and reproducible in pediatric population. There is no maturational change in rotation and twist, but torsion decreases with age in this cohort. Further refinement is warranted to validate the utility of this new methodology in more sensitive and quantitative evaluation of congenital and acquired heart diseases in children.

Real time three-dimensional echocardiography (RT3DE) has been increasingly used in the diagnosis and assessment of congenital heart disease. A growing body of literature suggests that this new technology can be used as an integrated approach to assess the morphology of simple and complex congenital heart defects, flow abnormality, and left, right, and single ventricular function both qualitatively and quantitatively. This review summarizes the available evidence for the use of RT3DE in each of these areas. Future technology refinement in RT3DE and development of practice guidelines will increase the utilization of this new technology as a valuable tool to compliment 2D echocardiography/Doppler in clinical care and research to improve the care and outcome of congenital heart disease.

We read with great interest the article by Osoegawa et al. [2014]whichshowedincreasedriskofsexchromosomeaneuploidyamongmales with congenital conotruncal defects. A boy born with trans-position of the great arteries (TGA) was found to have a 47,XXYchromosomecomplement which isconsistentwithaclinical diag-nosisofKlinefeltersyndrome(KS)[Osoegawa etal.,2014].Adatiaetal.[1987]documentedTGAinaneonatewith47,XXYKSin1987forthe first time. Here we report, to the best of our knowledge, onthe third such case.Klinefelter syndrome is the most common sex-chromosome dis-orderaffectingapproximately1in660men.KSischaracterizedbythepresence of one or more extra X chromosomes, with X disomy (47,XXY)beingthemostprevalentvariant[BojesenandGravholt,2007].KSwith47,XXYisseriouslyunderdiagnosed.Specificfeaturessuchastallstature,infertility,andsmalltestesoftenpresentafterthepuberty.Adiagnosisatbirthseemsimpossible,only25%ofexpectedcasesarediagnosedinlifetime,andlessthan5%ofdiagnosisaremadepriortoage 10 [Abramsky and Chapple, 1997; Bojesen and Gravholt, 2007].Despitebeinghistoricallyassociatedwithincreasedmortality,limitedstudyhasbeenconductedoncardiaclesionsinKS[Adatiaetal.,1987;Paduchetal.,2008].Onlyuntilrecently,cardiovascularabnormalitieshave been proposed to contribute to the reduced life span in KS[Pasquali et al., 2013].Transposition of the great arteries occurs when the pulmonaryarteryandtheaortaareswitchedinposition,andisoneofthemostseverecongenitalheartdiseases(CHD).TGAisseenin2–3/10,000livebirths,representingabout20–30%ofconotruncaldefectswithsitussolitus,andismorecommonamongmales[Relleretal.,2008;Unolt et al., 2013]. TGA is not associated with genetic syndromesexceptheterotaxy[Unoltetal.,2013].Ofnote,KSwasnotsuspectedinitiallyforanyofthethreecaseswithTGAdiscussedinthisletter.Theproband,aCaucasianmale,referredfromanoutsidehospitaldue to a prenatal diagnosis of TGA by fetal echocardiography in thefirsttrimester.Hewasborntoa43-year-old,G1P0motherat315/7weeks gestation with a healthy twin sister via cesarean. His APGARscoreswere4at1minand8at5min,w ithaweight(1.40kg),alength(41cm) and a head circumference (27.5cm) all around 10th–50thcentile.Hewasintubatedandplacedonventilatoratthedelivery,andtransportedtoourhospitalwherehestayedinNICUfor3months.Hehad mild nuchal fold thickening, posteriorly rotated ears, bilateralimmature vascularization in eyes, long fingers, sandal toe gaps, andmild jaundice. He had normal male external genitalia. Physicalexamination was otherwise unremarkable. The pregnancy wasachievedthroughinvitrofertilization(IVF)withanunknownspermdonor. Prenatal genetic counseling was received but prenatal genetictesting was declined. The mother had no major medical conditions,and denied any history of tobacco, alcohol, or illicit drug use duringthe pregnancy. Maternal family history was not significant for con-genital heart diseases, genetic disorders, birth defects, intellectual

The aims of this study were (1) to quantify changes in 3-dimensional (3D) strain in obese children using real-time 3D echocardiography (RT3DE) and 3D speckle tracking echocardiography (3DSTE), and (2) to investigate the utility of left ventricular (LV) strain variables in measuring early cardiovascular changes in children with obesity. A total of 181 obese children (study group) aged 4-18 years old were prospectively enrolled and compared with 229 healthy subjects (control group). We acquired demographic, clinical, biochemical, and 2D echocardiography/Doppler data. Also, RT3DE and 3DSTE were performed to measure LV volume, left ventricular ejection fraction (LVEF), LV mass (LVM), LV peak systolic global longitudinal strain (GLS), radial strain (GRS), circumferential strain (GCS), and global strain (GS). There were significant differences in anthropometric measurements, blood pressures, Cholesterol, C-reactive protein (CRP), Intima-media thickness (IMT), left atrium end-systolic dimension (LASD), interventricular septal end-diastolic dimension (IVSD), LV posterior wall end-diastolic dimension (LVPWD), LV end-diastolic dimension (LVEDD), LV end-systolic dimension (LVESD), LV end-diastolic volumes (LVEDV), and LV end-systolic volumes (LVESV), E and A velocities, E/A,e', e'/a', E/e', LVM, LV mass index (LVMI), GLS, GRS, GCS, and GS between the study and control groups. The receiver operating characteristic curves (ROC) for the statistically significant echocardiographic variables showed that the range of areas of ROC curves varied from 0.76 (GLS), 0.74 (GRS), 0.72 (LASD), to 0.58 (LVESD), respectively. In conclusion, LV 3D strain variables by RT3DE and 3DSTE decrease in obese children. LV 3D strain is more sensitive than other echocardiographic and vascular ultrasound variables in detecting cardiovascular changes in children with obesity.

Cardiac remodeling during heart failure development induces a significant increase in the activity of the L-type Ca2+ channel (Cav1.2). However, the effects of enhanced Cav1.2 activity on myocyte excitation-contraction (E-C) coupling, cardiac contractility, and its regulation by the β-adrenergic system are not clear. To recapitulate the increased Cav1.2 activity, a double transgenic (DTG) mouse model overexpressing the Cavβ2a subunit in a cardiac-specific and inducible manner was established. We studied cardiac (in vivo) and myocyte (in vitro) contractility at baseline and upon β-adrenergic stimulation. E-C coupling efficiency was evaluated in isolated myocytes as well. The following results were found: 1) in DTG myocytes, L-type Ca2+ current ( ICa,L) density, myocyte fractional shortening (FS), peak Ca2+ transients, and sarcoplasmic reticulum (SR) Ca2+ content (caffeine-induced Ca2+ transient peak) were significantly increased (by 100.8%, 48.8%, 49.8%, and 46.8%, respectively); and 2) cardiac contractility evaluated with echocardiography [ejection fraction (EF) and (FS)] and invasive intra-left ventricular pressure (maximum dP/d t and −dP/d t) measurements were significantly greater in DTG mice than in control mice. However, 1) the cardiac contractility (EF, FS, dP/d t, and −dP/d t)-enhancing effect of the β-adrenergic agonist isoproterenol (2 μg/g body wt ip) was significantly reduced in DTG mice, which could be attributed to the loss of β-adrenergic stimulation on contraction, Ca2+ transients, ICa,L, and SR Ca2+ content in DTG myocytes; and 2) E-C couplng efficiency was significantly lower in DTG myocytes. In conclusion, increasing Cav1.2 activity by promoting its high-activity mode enhances cardiac contractility but decreases E-C coupling efficiency and the adrenergic reserve of the heart.

Background: Loss of synchronous contraction between or within the right and left ventricle (RV, LV) leads to adverse ventricular function. We used real time three-dimensional echocardiography (RT3DE) for evaluation of severity of interventricular dyssynchrony and function in a porcine heart model. Methods: Six fresh in vitro porcine hearts were used to create a controlled model of LV and RV dyssynchrony using two sets of pulsatile pumps. Synchronized and dyssynchronized pump settings were used with two different dyssynchronized settings: LV filled first and RV filled first. Results: There was good correlation between actual measurement and RT3DE for interventricular time difference (r = 0.95, P < 0.0001) and stroke volume (SV) for LV and RV (0.89, 0.85; P < 0.0001, respectively). RT3DE data showed a small but significant underestimation for actual volume (P < 0.05). The intra- and interobserver variabilities are 2.9 ± 1.5% and 3.1 ± 5.4% for LV and RV SVs, and 1.7 ± 2.4% and 2.2 ± 3.2% for time differences by RT3DE. There was significant difference in RV SV between synchrony and dyssynchrony when the RV filled first (P < 0.05), but not in other groups. The same pattern was found in RT3DE derived SVs (synchrony versus dyssynchrony with RV filled first, P < 0.05). Conclusions: There is no compromise in LV SV during interventricular dyssynchrony; but RV SV was significantly diminished when the RV filled first. RT3DE is a feasible, robust and reproducible method to identify interventricular dyssynchrony and to evaluate ventricular SVs. (Echocardiography 2010;27:709-715)

In 1974, shortly after the advent of two-dimensional (2D) echocardiography (2DE), Dekker et al published the results of their graduate work at Stanford University, ‘‘A System for Ultrasonically Imaging the Human Heart in Three Dimensions.’’ Their vision was clear: ‘‘With the four dimensional data stored on the computer, programs to determine such properties as ejection fraction, wall thickness, aneurysms, defects, valve motions, structure velocities and heart power can be developed as well as image enhancement routines.’’ In the more than 3 decades that have passed, many milestones have been achieved in three-dimensional (3D) echocardiography (3DE). Several generations of 3D or four-dimensional (3 dimensions and time) echocardiographic systems have been developed. With the newest matrix-array transducer technology and software, a four-dimensional data set can be obtained with the push of a button. A large body of scientific literature has become available: a PubMed search reveals >3000 articles published to date on the utility of 3DE. As more evidence for the utility of 3DE became available, the American Society of Echocardiography published a position paper on its use.

To evaluate the value and improve the diagnostic accuracy of echocardiography in the diagnosis of a sinus of Valsalva aneurysm (SVA) with rare pathological patterns.Echocardiographic features and surgical findings from 270 Chinese patients with SVA treated in the last 18 years (1995-2013) at the Union Hospital were compared retrospectively; 22 of 270 cases had rare patterns.The patients with SVA, a rare origin, a rare extending position, or a rare course accounted for 3.4%, 7.4%, and 0.4% of the 270 cases, respectively. The three most common aneurysmal complications of the patients with rare patterns were severe aortic regurgitation (16), obstruction of the ventricular outflow tract or valvular orifice (3), and conduction disturbance (3). The origin, course, extending position and rupture status of the SVAs determined by echocardiography were entirely consistent with surgical findings in 81.8% of the 22 cases. With the exception of one failed diagnosis of an aneurysmal wall dissection and one misdiagnosis of a descending aortic dissection, the echocardiographic results of SVA complications and associated cardiovascular lesions were also confirmed.We could accurately diagnose SVAs with rare pathological patterns by echocardiographic identification of distinguishing features. However, for several conditions, we could not accurately identify the origin or course of the aneurysm or define its relationship to adjacent structures using conventional echocardiography alone. Therefore, we recommend combining conventional echocardiography with different imaging techniques, such as transesophageal echocardiography, three-dimensional echocardiography, computed tomography angiography, and aortic angiography.

BACKGROUND A discrete subaortic membrane cannot only cause left ventricular outflow tract obstruction, but can grow onto the aortic valve leaflets. The late finding of this encroachment is aortic valve insufficiency or stenosis. Echocardiography is used to follow the progression of outflow tract obstruction, but its ability to show subaortic membrane encroachment onto the aortic valve is unclear. The purpose of this study is to determine the sensitivity and specificity of echocardiography for diagnosing whether a discrete subaortic membrane involves the aortic valve. METHODS A pre-operative determination of aortic valve involvement by a discrete subaortic membrane was obtained by review of the official pre-operative echocardiogram reading and a retrospective blinded review of the pre-operative echocardiogram by an independent echocardiographer. These findings were compared to the intra-operative findings. RESULTS A total of 48 consecutive patients underwent primary resection for isolated discrete subaortic membrane between October, 1995 and May, 2006. The pre-operative and blinded readings both predicted a statistically lower rate of aortic valve involvement - 35% in 11 of 31 patients and 31% in 10 of 31 patients, respectively - than found at surgery - 65% in 31 of 48 patients. The sensitivity and specificity of pre-operative echocardiography to diagnose aortic valve involvement is 35% and 76%. Overall survival was 100%. There were no strokes, re-operations for bleeding or wound infections, or need for a pacemaker. CONCLUSION Echocardiography is not sensitive in assessing whether a discrete subaortic membrane involves the aortic valve. Since the morbidity and mortality for discrete subaortic membrane resection is negligible, resection may be indicated at the time of diagnosis to minimise aortic valve impairment.

Background Airway obstruction is a leading cause of mortality and morbidity in patients with pulmonary artery sling (PAS). Early identification of PAS is imperative for prompt initiation of appropriate treatments. Objective Our aim was to investigate the prevalence and clinical impact of PAS on school-aged children through a large-scale pre-sports participation cardiovascular screening (PPCVS) study. Methods A total of 186,213 school-aged children underwent portable two-dimensional echocardiography (2DE) study in a PPCVS study between 2001 and 2004 in Taichung City, Taiwan. We prospectively collected data on the prevalence of PSA, demographics, clinical features, tracheo-bronchial anomalies (TBA), on computed tomography (CT), pulmonary function test (PFT), follow-up data in 1 year, and clinical outcomes. Results PAS was identified in 11 children (8 males/3 females) with a median age of 13 years (range: 7.2–13.7 years). The prevalence of PAS was 1 in 17,000 school-aged children. The majority of children with PAS had recurrent broncho-pulmonary infections (90.9%) and asthmatic cough (81.8%), which had a low positive predictive value of PAS (

Background Measurements of left ventricular (LV) size, mass, and function are the most common and important tasks for echocardiography in clinical practice and research in children with congenital and acquired heart diseases. There are little data to compare the utility of M-mode (MM), 2-dimensional (2D), and 3-dimensional (3D) echocardiographic techniques for quantification of LV indices. The objective of the study was to assess the accuracy, reproducibility, and efficiency of these echocardiographic methods for measurement of LV indices in children. Methods A prospective study was conducted in 20 consecutive children (mean 10.6 ± 2.8 years, 11 male and 9 female subjects) using conventional MM, 2D, and real-time 3D echocardiography (RT3DE). A Sonos 7500 system (Philips Medical Systems, Andover, MA) was used. M-mode and 2DE measurements were made according to the American Society of echocardiography recommendations. To include the entire LV for volumetric measurement, full-volume 3D data sets were acquired from 4 electrocardiogram gated subvolumes. The 3DE measurements were made off-line manually using 4-plane and 8-plane algorithms by 4D Echo-View (TomTec Imaging Systems, Munich, Germany) and a semiautomated algorithm by QLAB (Philips Medical Systems). Magnetic resonance imaging studies were also performed to determine the LV indices by a disk summation method based on the Simpson principle. Results The correlation and agreement between MM, 2D, and RT3D echocardiography and magnetic resonance imaging measurements are good (r = 0.81-0.97) for the 3 methods. The correlation was superior for RT3DE compared with 2DE and MM. The correlation and agreement were similar for the three 3DE methods. The intra- and interobserver variabilities ranged from MM (4.3%-4.8% and 7.0%-8.7%), 2DE (3.3%-4.5% and 5.5%-7.3%), and 3DE (0.4%-2.3%, and 0.2%-4.8%). The total time (acquisition and analysis) used for MM measurements was the least compared with 2DE and 3DE. The total time for 3DE using the semiautomated algorithms was not significantly different compared with that for 2DE. Conclusions Our study showed that MM provides the most efficient assessment of LV indices but is the least accurate and reproducible technique compared with 2DE and 3DE. Three-dimensional echocardiography using both automated and manual analysis algorithm is superior to MM and 2DE for measurements of LV indices, and the automated 3DE algorithm is as efficient as 2DE. Therefore, 3DE using the automated algorithm is the method of choice for quantification of LV indices.

Cardiac malposition is a rare but important finding when detected on fetal ultrasound. The purpose of this study was to evaluate the incidence of fetal cardiac malposition, associated abnormalities, and clinical outcome in a tertiary-care medical center. Records of fetuses (1993 to 2006) with dextroposition, dextrocardia, mesocardia, ectopia cordis, or heterotaxy were reviewed. The presence of congenital heart disease (CHD), extracardiac anomalies, and outcome were noted. Cardiac malposition was present in 101 fetuses among a total of 3313 (3%) pregnancies. In 78 (78%) patients, the heart was positioned in the right hemithorax. Of those, 26 (33%) had dextrocardia (CHD = 21), and 52 (67%) had dextroposition (CHD = 14). Sixteen (16%) patients had mesocardia (CHD = 8), and 7 (7%) had ectopia cordis (CHD = 6). The majority (58%) of fetal cardiac malposition was caused by intrathoracic masses. Concomitant CHD occurred in 50%. Outcome was available in 97%. The perinatal and neonatal mortality rate was 30%; the elective termination rate was 4%. Patients with CHD had higher mortality rates. The highest mortality rates occurred in ectopia cordis and combined disease of diaphragmatic hernia and CHD.

Understanding of right ventricular (RV) remodeling is needed to elucidate the mechanism of RV dysfunction in the overloaded right ventricle, but is hampered by the chamber's complex shape. We imaged 15 patients with repaired tetralogy of Fallot (TOF) and 8 normal subjects by magnetic resonance imaging in long- and short-axis views. We reconstructed the right ventricles in 3 dimensions using the piecewise smooth subdivision surface method. Shape was analyzed from cross-sectional contours generated by intersecting the right ventricle with 20 planes evenly spaced from apex to tricuspid annulus. Patients with TOF had dilated right ventricles compared with normal (end-diastolic volume index 216 +/- 99 vs 81 +/- 16 ml/m(2), p

Measurement of right ventricular (RV) volumes and ejection fraction (EF) by two-dimensional echocardiography has limited accuracy and reproducibility because of the complex RV geometry.This study sought to validate real-time three-dimensional echocardiography (RT3DE) using a disk summation method for assessment of RV volumes and RVEF in children by comparing it with magnetic resonance imaging (MRI) measurements.A total of 20 children (mean age 10.6 +/- 2.8 years) were studied. Transthoracic RT3DE was performed using a RT3DE system to acquire full-volume RT3DE data sets from apical windows and data were processed offline using a software package. RV end-systolic volume and end-diastolic volume (EDV) were measured using a disk summation method by manually tracing the endocardial borders. RVEF was calculated as: RVEF = (EDV - end-systolic volume)/EDV x 100%. All participants also underwent MRI studies for comparison of RV indexes.Of the 20 children, 3 were excluded because of poor or incomplete RV images (two RT3DE and one MRI study). For the remaining 17 children, good correlation and agreement between RT3DE and MRI were found (RVEDV: r = 0.98, P.001, mean difference = -7.0 +/- 9.0 mL, P.01; RV end-systolic volume: r = 0.96, P.001, mean difference = -3.2 +/- 7.1 mL, P.05; RVEF: r = 0.89, P.001, mean difference = -0.3 +/- 7.1%, P.05). The intraobserver and the interobserver variabilities ranged from -1.1% to 5.8%.Measurement of RV volumes and EF by RT3DE is feasible, accurate, and reproducible in children compared with MRI measurements.

Background Early, sensitive, and reproducible evaluation of left ventricular function is imperative for the diagnosis of cardiac dysfunction in patients with Duchene muscular dystrophy. The aim of this study was to test the hypothesis that combining two-dimensional strain analysis with catecholamine stress could be a sensitive method for detecting early cardiac dysfunction. Methods Mdx (C57BL/10ScSn-Dmd mdx /J, a mouse model of DMD) and control (C57BL/10ScSn) mice were studied with conventional M-mode and high-frequency ultrasound-based two-dimensional speckle-tracking echocardiography using long- and short-axis images of the left ventricle at baseline and after intraperitoneal isoprenaline (ISO) administration (2 μg/g body weight). Results Conventional M-mode analysis showed no differences in left ventricular fractional shortening, wall thickness, or internal diameter at diastole between mdx and control mice before the age of 6 months. ISO increased left ventricular ejection fraction and fractional shortening to the same extent in mdx and control mice at young ages (3, 4, and 5 months). No differences in basal peak systolic strain (PSS) but increased SDs of times to PSS between young mdx and control mice were found. After ISO, PSS and percentile changes of PSS were significantly diminished in mdx mice compared with control mice at young ages. ISO increased the normalized maximum difference of times to PSS in young mdx mice but not in young control mice, suggesting that ISO reduces cardiac contractile synchrony in young mdx mice. Conclusions This study suggests that catecholamine stress coupled with two-dimensional strain analysis is a feasible and sensitive approach for detecting early onset of cardiac dysfunction, which is instrumental for early diagnosis of cardiac dysfunction and early treatment.

We developed and assessed a real-time 3-dimensional (3D) digital Doppler method for measurement of flow volumes through the mitral valve in children. A total of 13 children (aged 10.46 +/- 2.5 years; 8 boys/5 girls) were enrolled. An ultrasound system (Sonos 7500, Philips, Andover, Mass) was used to acquire raw 3D velocity data for flow measurement based on Gaussian control surface theorem [flow (mL/s) = mean velocity x flow area]. Stroke volume (SV) measured by real-time 3D digital Doppler with the control surface at the mitral valve annulus or orifice was compared with the SV by phase velocity cine magnetic resonance imaging (MRI) at the ascending aorta and by left ventricular volumetric MRI measurement. The best correlation and agreement were seen at the mitral valve orifice by real-time 3D digital Doppler compared with SV by phase velocity cine MRI at the ascending aorta (r = 0.92, mean difference = -5.2 +/- 12.0 mL) and SV by left ventricular volumetric MRI measurement (r = 0.94, mean difference = -0.2 +/- 10.3 mL).

The cardiac flow volumes through the mitral valve orifice were measured using a Gaussian control surface-based, Doppler angle-independent method on real-time three-dimensional digital Doppler (3DDD) data from 13 children. Our flow volume measurements correlated and agreed well with the ascending aorta flow volumes measured by PVC MRI and stroke volumes obtained from MRI left ventrical volumetric measurements.

Introduction: Hypoplastic left heart syndrome (HLHS) is the term used to describe a group of congenital malformations characterized by marked underdevelopment of the left side of the heart. HLHS accounts for nearly 25% of cardiac deaths in the first year of life. Although much has been reported regarding diagnosis, gross morphology and surgical treatment, no information on gene expression in HLHS myocytes is available. Methods: We examined heart tissue from patients with HLHS using routine histology, immunohistochemistry, quantitative polymerase chain reaction (PCR), two-dimensional (2-D) gel electrophoresis and protein identification by mass spectrometry. Results: Histologic examination of right and left ventricles from HLHS patients revealed characteristic features of myocyte differentiation, including striations and intercalated disc formation. Immunohistochemical staining using antibody to N-cadherin demonstrated clear development of intercalated discs between myocytes. However, many of the myocytes contained scant cytoplasm and were grouped in small, disorganized bundles separated by abundant connective tissue and dilated, thin-walled vessels. Quantitative PCR analysis demonstrated that both left and right ventricular tissue from HLHS hearts expressed the fetal or “heart failure” gene expression pattern. Two-dimensional gel electrophoresis and protein identification by mass spectrometry also confirmed that myocytes from HLHS ventricles were differentiated but expressed the fetal isoform of some cardiac specific proteins. However, HLHS myocytes in all of the heart samples (n=21) were inappropriately expressing platelet-endothelial cell adhesion molecule-1 (PECAM-1, CD31), a member of the cell adhesion molecule (CAM) family that has a primary role in the regulation of tissue morphogenesis. These findings indicate that myocytes from HLHS syndrome patients, while differentiated, have a unique gene expression pattern.

Ultrasound alone or combined with contrast agent microbubbles offers wide-spread applicability, ranging from well-established diagnostic tools to adjunctive therapeutic methods. Ultrasound could elicit anti-inflammatory and tissue stimulating effects, as already shown in clinical trials and

Hypertrophic cardiomyopathy (HCM) is a disease predisposes to unexplained left ventricular (LV) hypertrophy and dysfunction and non-dilated ventricular chambers associated unusual myocardial mechanics. The most serious complications of HCM is sudden death for young subclinical patients, thus

Conjoined twins (CT) are rare with possible serious malformations in which soft tissue, bone, or some organs are joined in utero. The extent of cardiac fusion and intracardiac anatomy of CT determine the viability, natural history, and outcome of potential surgical intervention. Early prenatal diagnosis and assessment may provide a window of opportunity to counsel the family for their informed decision on the pregnancy and to plan for prenatal and perinatal care. In this report, we describe a case of thoracopagus twins diagnosed by fetal echocardiography at 23-week gestation. The 2 hearts fused at the atrial and ventricular levels. The outcome and review of literature on fetal echocardiographic characteristics of this malformation are discussed.

Aims To determine whether altered beta-adrenergic responses contribute to early cardiac dysfunction in mdx (X-linked muscular dystrophy) mice, an animal model for human Duchenne muscular dystrophy. Methods and results Replacement fibrosis in mdx hearts gradually increased with age, suggesting a gradual loss of cardiomyocytes. Echocardiography and intra-left ventricular haemodynamic measurements detected baseline cardiac dysfunction in mdx mice at ≥8 months. However, a reduction of cardiac beta-adrenergic response to isoproterenol (ISO) was already present in mdx mice at 4 months. Ventricular myocytes (VMs) isolated from 4- and 8-month-old mdx mice had greater baseline contractile function {fractional shortening, [Ca2+]i, and sarcoplasmic reticulum (SR) Ca2+ content} and I Ca-L than age-matched control VMs and than myocytes isolated from 2-month-old mdx mice. ISO increased myocyte function in the VMs of 4- and 8-month-old mdx mice to the same level as in age-matched control VMs. In the VMs of 12-month-old mdx mice, ISO failed to increase myocyte function to the level in VMs of 12-month-old control mice and could not further increase I Ca-L. No differences were observed in the expression of Cav1.2α1c, Cav1.2β1, Cav1.2β2, sarco/endoplasmic reticulum Ca2+ ATPase (SERCA), and the Na+/Ca2+ exchanger. In contrast, total ryanodine receptor 2 (RyR2) and basal phosphorylation of RyR2, phospholamban, and Cav1.2α1c were found to be increased in hearts of 4-month-old mdx mice; baseline protein kinase A activity was also increased. After ISO treatment, phosphorylation levels were the same in mdx and control hearts. VMs of 4-month-old mdx mice had reduced beta1-adrenergic receptor (β1-AR) density and beta-adrenergic sensitivity. Conclusion In young mdx mice, the myocyte increases its contractile function to compensate for myocyte loss. However, these myocytes with enhanced baseline function have reduced potential for stimulation, decreased β1-AR density/sensitivity, leading to blunted cardiac beta-adrenergic response.

In this prospective trial, intraoperative 2-dimensional (2-D) and 3-dimensional (3-D) transesophageal echocardiography (TEE) examinations were performed on 60 consecutive patients undergoing cardiac valve surgery. Both 2-D (including color flow and Doppler data) and 3-D images were reviewed by blinded observers, and major valvular morphologic findings recorded. In vivo morphologic findings were noted by the surgeon and all explanted valves underwent detailed pathologic examination. To test reproducibility, 6 patients also underwent 3-D TEE 1 day before surgery. A total of 132 of 145 attempted acquisitions (91%) were completed with a mean acquisition time of 2.8 +/- 0.2 minutes. Acquisition time was significantly shorter in patients with regular rhythms. Reconstructions were completed in 121 of 132 scans (92%) and there was at least 1 good reconstruction in 56 of 60 patients (93%). Mean reconstruction time was 8.6 +/- 0.7 minutes. Mean effective 3-D time, which was the time taken to complete an acquisition and a clinically interpretable reconstruction, was 12.2 +/- 0.8 minutes. Intraoperative 3-D echocardiography was clinically feasible in 52 patients (87%). Three-D echocardiography detected most of the major valvular morphologic abnormalities, particularly leaflet perforations, fenestrations, and masses, confirmed on pathologic examination. Three-D echocardiography predicted all salient pathologic findings in 47 patients (84%) with good quality images. In addition, in 15 patients (25%), 3-D echocardiography provided new additional information not provided by 2-D echocardiography, and in 1 case, 3-D echocardiographic findings resulted in a surgeon's decision to perform valve repair rather than replacement. In several instances, 3-D echocardiography provided complementary morphologic information that explained the mechanism of abnormalities seen on 2-D and color flow imaging. In the reproducibility subset, preoperative and intraoperative 3-D imaging detected a similar number of findings when compared with pathology. Thus, in routine clinical intraoperative settings, 3-dimensional TEE is feasible, accurately predicts valve morphology, and provides additional and complementary valvular morphologic information compared with conventional 2-D TEE, and is probably reproducible.

The early detection of abnormal left ventricular systolic functions in patients with hypertrophic cardiomyopathy (HCM) remains a challenge. The aim of this study was to identify a novel method for the assessment of left ventricular systolic function in patients with HCM. A total of 65 patients with HCM were included in this study. The patients were divided into obstructive HCM (HOCM; 16 cases) and non-obstructive HCM (NOHCM; 49 cases) groups. The healthy control group comprised 48 participants. Two-dimensional (2D) speckle-tracking technology was used to measure the left ventricular global and segmental longitudinal strains and mitral annular displacement (MADs). Compared with healthy control group, the six segmental strains and the global strain of the left ventricle (LSglobal) increased while six segmental MADs and MADglobal of the mitral annulus decreased in the HOCM and NOHCM groups (P

In this article, we review a number of topics that we believe reflect new and exciting aspects of fetal echocardiography. These new advances include early fetal cardiovascular imaging around 14 weeks, the utility of three/four dimensional imaging technology for the fetus, and finally the utility of fetal echocardiography for antenatal and perinatal care of congenital heart diseases to improve and optimize outcome. Finally, we briefly discussed future directions in fetal cardiac intervention.

The development of echo contrast agents that can provide reliable opacification of the myocardium after intravenous injection is an important advancement for the clinical application of contrast echocardiography. In this study, the hemodynamic effects and echocardiographic characteristics of a new lipid-fluorocarbon echo contrast agent, Aerosomes MRX 115 (ImaRx Pharmaceutical Corp., Tucson, Ariz.) were studied in six anesthetized ventilated pigs. Intravenous injection of this new agent in doses ranging from 0.0005 to 0.01 ml/kg produced significant measurable and visible myocardial opacification without any effect on heart rate, systemic pressure, partial pressure of oxygen, or left ventricular systolic function. The two largest doses (0.005 and 0.01 ml/kg), however, resulted in mild reversible increases in mean pulmonary artery pressure of 12 and 16 mm Hg, respectively. In four animals, epicardial images were obtained before and during coronary artery occlusion. Intravenous contrast injection during coronary occlusion permitted delineation of the hypoperfused myocardial segment. This capability may further expand the utility of contrast echocardiography.

Our study indicates that (1) the biplane TEE probe provides an accurate assessment of the surgical repair of ToF in infants and children; (2) the longitudinal plane especially offers important information showing right ventricular outflow tract anatomy that cannot be fully defined using conventional transverse plane TEE; and (3) implementation of steerable CW Doppier aids quantification of residual right ventricular outflow tract obstruction, especially when implemented from transgastric views.