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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 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.

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.

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.

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.

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.

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).

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.

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.

Three-dimensional (3D) strain analysis may help overcome the limitations of Doppler and two-dimensional strain analysis for the left ventricle and become the method of choice for left ventricular (LV) systolic function. The aims of this study were to evaluate the feasibility and reproducibility of LV global 3D systolic strain by real-time 3D speckle-tracking echocardiography (STE) in children and to establish their maturational growth patterns and normal values.A prospective study was conducted in 256 consecutive healthy subjects using real-time 3D echocardiography. Full-volume 3D data were acquired using a 3D matrix-array transducer. Three-dimensional LV peak systolic global strain (GS), global longitudinal strain (GLS), global radial strain (GRS), and global circumferential strain (GCS) values were determined using real-time 3D STE.A total of 228 patients (89%) met the criteria for analysis; 28 (11%) were excluded. The correlations between age and strain variables by real-time 3D STE were poor (R(2) = 0.01-0.05, P.05). The differences in GLS and GCS among the five age groups were statistically significant but clinically irrelevant. There were no statistical differences in GRS and GS values among the age groups, nor were there statistical differences between the genders for all 3D strain parameters. Intraobserver and interobserver variability ranged from 5.0 ± 4.3% to 10.1 ± 8.5% versus 6.9 ± 6.1% to 17.0 ± 16.2% for coefficients of variation, respectively. Interclass correlation coefficients ranged from 0.78 to 0.87 and from 0.75 to 0.79 for intraobserver and interobserver measurements for GS, GLS, GCS, and GRS, respectively.LV global 3D systolic strain analysis using the new 3D STE is feasible and reproducible in the pediatric population. There are small maturational changes in GLS and GCS, but not in GRS and GS, that are statistically significant but probably clinically irrelevant. Further investigation is warranted for potential clinical application of this new technology in a pediatric population.

Ultrasound (US)-mediated cavitation of microbubbles has evolved into a new tool for organ-specific gene and drug delivery. This paper was to investigate the feasibility of acidic fibroblast growth factor (aFGF) intravenous delivery to the ischemic myocardium of rats by ultrasonic microbubbles modified with heparin. Heparin modified microbubbles (HMB) were prepared by the freeze-dried method. Acute myocardial infarction (AMI) model was established and the cardio protective effect of the aFGF combing with HMB (aFGF-HMB) under US-mediated cavitation technique was investigated. aFGF-HMB combined with US-mediated cavitation technique was examined by ECG. Ejection fraction (EF), fractional shortening (FS) and left ventricular diastolic diameter (LVDd) were measured to monitor the improvement of global myocardial contractile function. Myocardial tissue was stained with hematoxylin and eosine (HE) to evaluate the elaborate general morphology of the ischemic myocardium. From morphologic observation and echocardiography in rat heart, aFGF-HMB had suitable size distribution, physical stability and good acoustic resonance function. From AMI rat experiments, aFGF-HMB under US-mediated cavitation technique exerted aFGF cardio protective effect in ischemic myocardium. From histological evaluation, US-mediated cavitation of aFGF-HMB showed improvement of myocardial ischemia. With the visual imaging and US-triggered drug release advantages, US-mediated cavitation of aFGF-HMB might be developed as a novel technique for targeting delivery of aFGF into ischemic myocardium.

66 Conventional Doppler echocardiography, including pulsed-wave, continuous-wave, and color-flow Doppler techniques, is an integral component of an echocardiographic study for the diagnosis and assessment of cardiovascular disease. It is used routinely to provide hemodynamic information for flow measurement, diastolic function, estimation of right-sided pressures, pressure gradients and valve areas, valve regurgitation, shunt lesions, and prosthetic valves, to name a few, that would otherwise require invasive investigation. Compared with other modalities, such as cardiac magnetic resonance and invasive measurements by cardiac catheterization, Doppler echocardiography has a few limitations. One of the ‘‘soft spots’’ is the lack of accuracy of quantitative flow measurement in assessing stroke volume (SV), cardiac output, shunt lesions, and regurgitant volumes and fractions, which limits not only the routine evaluation of various structural and flow abnormalities but also the assessment of medical, percutaneous, and surgical interventions and longitudinal follow-up.

Background— Dilated cardiomyopathy (DCM) is a primary disease of the heart muscle associated with sudden cardiac death secondary to ventricular tachyarrhythmias and asystole. However, the molecular pathways linking DCM to arrhythmias and sudden cardiac death are unknown. We previously identified a S196L mutation in exon 4 of LBD3 -encoded ZASP in a family with DCM and sudden cardiac death. These findings led us to hypothesize that this mutation may precipitate both cytoskeletal and conduction abnormalities in vivo. Therefore, we investigated the role of the ZASP4 mutation S196L in cardiac cytoarchitecture and ion channel biology. Methods and Results— We generated and analyzed transgenic mice with cardiac-restricted expression of the S196L mutation. We also performed cellular electrophysiological analysis on isolated S196L cardiomyocytes and protein-protein interaction studies. Ten month-old S196L mice developed hemodynamic dysfunction consistent with DCM, whereas 3-month-old S196L mice presented with cardiac conduction defects and atrioventricular block. Electrophysiological analysis on isolated S196L cardiomyocytes demonstrated that the L-type Ca 2+ currents and Na + currents were altered. The pull-down assay demonstrated that ZASP4 complexes with both calcium (Ca v 1.2) and sodium (Na v 1.5) channels. Conclusions— Our findings provide new insight into the mechanisms by which mutations of a structural/cytoskeletal protein, such as ZASP, lead to cardiac functional and electric abnormalities. This work represents a novel framework to understand the development of conduction defects and arrhythmias in subjects with cardiomyopathies, including DCM.

We tested the accuracy and reproducibility of knowledge-based reconstruction (KBR) for measuring right ventricular (RV) volume and function. KBR enables rapid assessment of the right ventricle from sparse user input by referencing a database. KBR generates a 3-dimensional surface to fit points that the user enters at anatomic landmarks. We measured the RV volume using KBR from magnetic resonance images in 20 patients with repaired tetralogy of Fallot at end-diastole and end-systole. We entered points in the long- and short-axis and/or oblique views. The true volume was computed by manually tracing the RV borders for 3-dimensional reconstruction using the piecewise smooth subdivision surface method. The reference database included 54 patients with tetralogy of Fallot patients. The KBR values agreed closely with the true values for the end-diastolic volume (r = 0.993), end-systolic volume (r = 0.992), and ejection fraction (EF; r = 0.930). KBR slightly overestimated the end-diastolic volume (4 +/- 10 ml, p = NS), end-systolic volume (1 +/- 9 ml, p = NS), and EF (4 +/- 3%, p = NS). No bias in the error was found by Bland-Altman analysis (p = NS for end-diastolic and end-systolic volume and EF). The KBR volumes had approached the true volumes (235 +/- 93 vs 243 +/- 93, p = 0.012, r = 0.978 for end-diastolic and end-systolic volumes combined) already after the first run and the entry of 19 +/- 3 points. In conclusion, KBR provided accurate measurement of the RV volume and EF with minimal user input. KBR is a clinically feasible alternative to full manual tracing of the heart borders from imaging data.

Preliminary speckle-tracking echocardiographic studies show that patients with single ventricles (SVs) have significantly decreased twisting and dyssynchrony of twisting. This could be related to abnormal cardiac looping, which leads to hearts that lack helical fiber patterns. The aim of this study was to analyze gradient cine magnetic resonance imaging (MRI) using Velocity Vector Imaging to assess cardiac mechanics. Subjects were 38 patients (aged 8 to 37 years) with SVs of left ventricular (n = 30) and indeterminate (n = 8) type who underwent cardiac MRI. Controls were 14 normal children and adults. Gradient cine MRI sequences close to the apex were subjected to a Velocity Vector Imaging analysis program adapted for MRI. In the control group, mean circumferential strain was -18.02 +/- 7.31%, mean dispersion of peak circumferential strain was 44.23 +/- 37.14 ms, and average rotation was -7.7 +/- 1.38 degrees . The rotation values were negative, or counterclockwise. In patients with SVs, mean circumferential strain was -8.87 +/- 7.30%, mean dispersion of peak circumferential strain was 181.55 +/- 76.07 ms, and average rotation was -2.6 +/- 1.24 degrees (p0.001). Mean dispersion of the peak of rotation in the control group was 39.6 +/- 22.8 ms, compared to 166.5 +/- 72.4 ms in patients with SVs. In conclusion, this study showed a dramatic decrease in apical rotation and circumferential strain in the SV group compared to the control group. Strain and rotation mechanics at the apex in patients with SVs showed marked dyssynchrony.

nderA 2-month-old boy was brought to our outpatient department with respiratory distress, poor feeding tolerance, and inadequate weight gain. Initial 2-dimensional (2D) echocardiography demonstrated an aortopulmonary window (APW) (type II) between the distal ascending aorta and the main pulmonary artery from a subcostal view (Figure 1 and V 1), coarctation of aorta, patent ductus arterio (PDA), and ventricular septal defect. 3 Color Doppler

Background We sought to assess the accuracy and reproducibility of an automated real-time (RT) 3-dimensional (3D) Doppler echocardiography (RT3DDE) technique for measuring volumetric flow (VF) in children. Methods A total of 19 healthy children (age = 11.5 ± 3.5 years) were studied to measure VF through mitral valve (MV), aortic valve (AV), pulmonary valve (PV), and tricuspid valve (TV) by RT3DDE. RT 3D echocardiography was also performed to measure left ventricular (LV) end-systolic volume, LV end-diastolic volume, and stroke volume (stroke volume=LV end-diastolic volume − LV end-systolic volume), which served as a reference standard for comparison with VF by RT3DDE. Results Compared with stroke volume by RT 3D echocardiography, the correlation with VF was excellent for MV ( r = 0.91), good for AV ( r = 0.89) and PV ( r = 0.89), but poor for TV ( r = 0.20) by RT3DDE. There were good agreements for AV (bias = 0.9 ± 5.0 mL), PV (bias = −0.4 ± 5.7 mL), and MV (bias = 4.1 ± 4.7 mL), and marked underestimation for TV (bias = −24.4 ± 14.6 mL). Conclusions Our data demonstrated that VF measurement by RT3DDE is feasible and reasonably accurate for MV, AV, and PV but problematic for TV.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by progressive degeneration of the right ventricular myocardium, ventricular arrhythmias, fibrous-fatty replacement, and increased risk of sudden death. Mutations in 6 genes, including 4 encoding desmosomal proteins (Junctional plakoglobin (JUP), Desmoplakin (DSP), Plakophilin 2, and Desmoglein 2), have been identified in patients with ARVD/C. Mutation analysis of 66 probands identified 4 variants in DSP; V30M, Q90R, W233X, and R2834H. To establish a cause and effect relationship between those DSP missense mutations and ARVD/C, we performed in vitro and in vivo analyses of the mutated proteins. Unlike wild-type (WT) DSP, the N-terminal mutants (V30M and Q90R) failed to localize to the cell membrane in desomosome-forming cell line and failed to bind to and coimmunoprecipitate JUP. Multiple attempts to generate N-terminal DSP (V30M and Q90R) cardiac-specific transgenes have failed: analysis of embryos revealed evidence of profound ventricular dilation, which likely resulted in embryonic lethality. We were able to develop transgenic (Tg) mice with cardiac-restricted overexpression of the C-terminal mutant (R2834H) or WT DSP. Whereas mice overexpressing WT DSP had no detectable histologic, morphological, or functional cardiac changes, the R2834H-Tg mice had increased cardiomyocyte apoptosis, cardiac fibrosis, and lipid accumulation, along with ventricular enlargement and cardiac dysfunction in both ventricles. These mice also displayed interruption of DSP-desmin interaction at intercalated discs (IDs) and marked ultra-structural changes of IDs. These data suggest DSP expression in cardiomyocytes is crucial for maintaining cardiac tissue integrity, and DSP abnormalities result in ARVD/C by cardiomyocyte death, changes in lipid metabolism, and defects in cardiac development.

Real-time 3-dimensional echocardiography is increasingly used in clinical cardiology. Studies have been shown that this technique can be accurately used to assess both cardiac mass and chamber volumes. We review the work showing that real-time 3-dimensional Doppler echocardiography can be used to accurately calculate intracardiac flow volumes that can potentially be used to assess cardiac function, intracardiac shunt, and valve regurgitation.

A 2-month-old infant with a double-outlet right ventricle, pulmonary stenosis, a subaortic ventricular septal defect, a hypoplastic mitral valve, a hypoplastic left ventricle, and a restrictive atrial septal defect was seen at an outside institution. The initial plan was to palliate the child with an atrial septectomy, have the pulmonary artery oversewn, and perform a modified Blalock-Taussig shunt. During surgery, an anomalous origin of the left lower pulmonary artery was noted originating from the right pulmonary artery. The possibility of a left …

A direct and accurate method of assessing aortic valve area (AVA) in patients with aortic stenosis (AS) is desirable because of the well-known theoretical and practical limitations of the currently available methods. We assessed the clinical feasibility and accuracy of a novel index, the 3-dimensional surface area (3-DSA) of the aortic valve orifice by 3-dimensional transesophageal echocardiography (3-DTEE) in patients with AS.Intraoperative 3-DTEE was performed in 23 consecutive patients (mean age 58 +/- 15 years) with valvular AS using a Toshiba SSA-380A system with a multiplane TEE probe and a TomTec EchoScan system. The 3-DTEE acquisition, processing and reconstruction were conducted and the aortic valve orifice presented using a "surgeon's aortotomy view" (aortic valve orifice as if viewed through an open aortic root). The 3-D images were videotaped and calibrated and the 3-DSA measured by planimetry of the inner surface of the aortic valve leaflets at the maximal systolic opening using the dynamic 3-D images. For comparison, the 2-D cross sectional area (2-DCSA) of the aortic valve was also determined by 2-DTEE. The 3-DSA and 2-DCSA were compared with the AVA by the invasive Gorlin formula and the Doppler continuity equation method by transthoracic echocardiography.The 3-DSA and 2-DCSA measurements were feasible in all but one patient. Both 3-DSA and 2-DCSA correlated moderately well with the AVA by the Gorlin formula (n = 17, r = 0.66, standard error of the estimate [SEE] = 0.3 cm2, P.05 for 3-DSA and r = 0.61, SEE = 0. 5 cm2 P.05 for 2-DCSA, respectively). They also correlated well with the AVA by Doppler continuity equation method (n = 22, r = 0.90, SEE = 0.1 cm2, P.05 for 3-DSA and r = 0.83, SEE = 0.3 cm2, P.05 for 2-DCSA, respectively). There was no statistically significant difference between the 3-DSA and AVA by both the Gorlin formula (Delta = 0.1 +/- 0.3 cm2, P =.3) and the Doppler continuity equation (Delta = -0.0 +/- 0.3 cm2, P =.7). In contrast, the 2-DCSA significantly overestimated AVA by the Gorlin formula (Delta = 0.5 +/- 0.5 cm2, P.005) and by the Doppler continuity equation (Delta = 0.5 +/- 0.6 cm2, P.0001).Planimetry of 3-DSA of the aortic valve orifice by 3-DTEE is a clinically feasible and relatively accurate technique for assessment of AVA and is superior to 2-DCSA by 2-DTEE.

This report describes three-dimensional transesophageal echocardiographic findings in three consecutive patients with discrete subaortic stenosis. The discrete subaortic stenosis lesions included a circumferential, a remnant crescent, and a broken fibrotic subaortic membrane. The lesions were best imaged by using a three-dimensional transesophageal echocardiography–generated "aortotomy view" of the left ventricular outflow tract immediately below the plane of the aortic valve. The three-dimensional images correlated well with surgical and pathologic findings. The three-dimensional surface areas of the left ventricular outflow tract at the level of discrete subaortic stenosis during systole (0.8 ± 0.5 cm 2 ) and diastole (1.7 ± 0.7 cm 2 ) were measured by planimetry of the three-dimensional transesophageal echocardiographic images. The novel "aortotomy view" offered by three-dimensional transesophageal echocardiography provided direct visualization and quantification of discrete subaortic stenosis in a dynamic fashion. In summary, three-dimensional transesophageal echocardiography can accurately display and quantify discrete subaortic stenosis and could be a new clinically useful tool for assessing discrete subaortic stenosis and guiding surgical and transcatheter interventions. (J Am Soc Echocardiogr 1997;10;713–6.)

Objectives To observe the protective effects of left ventricular function in diabetic cardiomyopathy rats after delivery of acidic fibroblast growth factor (aFGF) to myocardium by using ultrasound-targeted microbubble destruction (UTMD) and investigate the possible therapeutic mechanism. Methods Twenty-four rats were induced to be DCM model by streptozotocin through intraperitoneal injecting and were randomly divided into the DCM group and the aFGF therapy group, and twelve normal rats were included as normal control group in the study. The aFGF therapy group animals were infused with SonoVue-aFGF mixed fluids through tail vein and simultaneous using UTMD. Four weeks after intervention, all rats underwent the cardiac catheter exam to obtaine the left ventricular contraction end pressure (LVESP), left ventricular end-diastolic pressure (LVEDP) and the maximal rate of pressure increase/decrease (± dp/dt max ). At last, myocardial tissue of all rats were stained with immunohistochemistry to quantify myocardial microvascular density, improved Masson staining of collagen to determination the myocardial collagen volume fraction (CVF), TUNEL method to detect the myocardial tissue apoptosis index (AI). Results Four weeks after intervention, the LVESP and LV ± dp/dt max in the aFGF therapy group were significantly increased than in DCM group (P Conclusions Delivery of aFGF to diabetic myocardium by using UTMD could improve the left ventricular function and may be a new feasible therapeutic method for DCM.