Search

In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology. Prenatal diagnosis of LVHT can be established by fetal echocardiography. A review of 106 published cases showed that 46 cases with prenatally diagnosed LVHT were alive 0.5-120 months after birth. Since the course of cases with prenatally LVHT after publication is unknown, we aimed to collect follow-up-information. Information regarding vital status, cardiac and extracardiac morbidity was gathered by contacting the authors of the 46 cases. Fourteen of the 28 authors answered and gave information about 18 cases (six females, seven males, five gender-unknown, age 18 months to 10 years, mean follow-up 60 months). No differences were found between the 18 cases with follow-up and the 28 cases without follow-up regarding age, gender, cardiac or extracardiac comorbidities, and interventions. Three of the 18 cases had died subsequently from heart failure, osteosarcoma, and enterocolitis, respectively. Mutations or chromosomal abnormalities were found in six of the seven examined patients, extracardiac abnormalities in nine patients. Three patients received a pacemaker because of complete AV block, and two patients underwent heart transplantation. Cardiac surgical or interventional procedures were carried out in four patients. None suffered from malignant arrhythmias or had a cardioverter-defibrillator implanted. Based on the limited information, there are indications that cases with fetal diagnosis of LVHT have a continuing morbidity and mortality, even if they receive appropriate care. Since fetal LVHT is frequently associated with genetic abnormalities, further research about survival and underlying genetic causes is needed.

Objectives: Trans-oesophageal echocardiographic imaging is valuable in the pre- and post-operative evaluation of children and adults with CHD; however, the frequency by which trans-oesophageal echocardiography guides the intra-operative course of patients is unknown., Methods: We retrospectively reviewed 1748 intra-operative trans-oesophageal echocardiograms performed between 1 October, 2005 and 31 December, 2010, and found 99 cases (5.7%) that required return to bypass, based in part upon the intra-operative echocardiographic findings., Results: The diagnoses most commonly requiring further repair and subsequent imaging were mitral valve disease (20.9%), tricuspid valve disease (16.0%), atrioventricular canal defects (12.0%), and pulmonary valve disease (14.1%). The vast majority of those requiring immediate return to bypass benefited by avoiding subsequent operations and longer lengths of hospital stay. A total of 14 patients (0.8%) who received routine imaging required further surgical repair within 1 week, usually due to disease that developed over ensuing days. Patients who had second post-operative trans-oesophageal echocardiograms in the operating room rarely required re-operations, confirming the benefit of routine intra-operative imaging., Conclusions: This study represents a large single institutional review of intra-operative trans-oesophageal echocardiography, and confirms its applicability in the surgical repair of patients with CHD. Routine imaging accurately identifies patients requiring further intervention, does not confer additional risk of mortality or prolonged length of hospital stay, and prevents subsequent operations and associated sequelae in a substantial subset of patients. This study demonstrates the utility of echocardiography in intra-operative monitoring of surgical repair and highlights patients who are most likely to require return to bypass, as well as the co-morbidities of such manipulations.

An infant presented with features suggestive of an anomalous left coronary artery was found to have normal origins of both coronary arteries. Echocardiography during episodes of ischaemia showed marked aortic regurgitation with retrograde coronary flow. The left coronary leaflet was mildly hypoplastic. Surgical re-suspension of this leaflet prevented aortic regurgitation and the patient had no further symptoms and recovered cardiac function.

In the 1970s, diagnosing anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was often uncertain using imaging alone; however, with the advances in high-frequency transducers, advanced image processing, and other ultrasound modalities such as Doppler colour flow imaging, tissue Doppler imaging, and speckle tracking to asses regional wall motion abnormalities, modern echocardiography now permits accurate diagnosis of ALCAPA with greater certainty. Although many consider ultrasound to be the only imaging test necessary if there is a question as to the diagnosis, other imaging modalities such as MRI, CT, and cardiac catheterisation with angiography remain valuable complementary tests, especially in older patients.

Background: Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era., Methods and Results: Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001)., Conclusion: In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival., (© 2015 American Heart Association, Inc.)

I surveyed our echocardiographic database of the years between 1998 and 2012 for congenital abnormalities of the mitral valve in patients over 14 years. A total of 249 patients with mitral valve abnormalities were identified. Abnormalities included clefts in the mitral valve in 58 patients, double orifice of the mitral valve in 19, mitral stenosis with two papillary muscles in 72, and mitral stenosis with one papillary muscle in 51 patients. Supravalvar rings were found in 35 patients with a single papillary muscle, and mitral stenoses with two papillary muscles were found in 22 patients. Mitral prolapse occurred in 44 patients and mitral valvar straddle in five patients. The patients were evaluated by all modalities of ultrasound available over the course of time. Although some lesions were isolated, there were many lesions in which more than one mitral deformity presented in the same patient. The patients are presented showing anatomical correlation with autopsy specimens, some of which came from the patients in this series, and others matched to show correlative anatomy. These lesions remain rare as a group and continue to have high morbidity and mortality.

Objectives: Optimal perinatal management of total anomalous pulmonary venous return (TAPVR) involves timely identification followed by surgical correction. Antenatal diagnosis, however, has long been a challenge. We aimed to identify consistent prenatal sonographic features in this condition in a large cohort in whom the diagnosis was made antenatally and confirmed postnatally., Methods: We conducted a systematic retrospective review of the 2-dimensional and Doppler sonographic features that had helped make the diagnosis of TAPVR at our institution from 2001 to 2012., Results: Twenty-six patients had prenatal diagnosis of TAPVR (mean gestational age, 24.1 weeks). Four of the fetuses with a prenatal diagnosis represented isolated cases of TAPVR; 22 had heterotaxy syndrome, additional cardiac abnormalities, or both. Prenatally diagnosed abnormal pulmonary venous connections were supracardiac (type I) in 18 cases, cardiac (type II) in 1, and infradiaphragmatic (type III) in 7. Lack of a visible connection of the pulmonary veins to the atrium (100%) and the presence of a visible venous confluence on axial 4-chamber views (96%) were the most consistent findings. Cardiac asymmetry and the presence of additional vertical venous channels on 3-vessel or axial abdominal views were also noted but less consistently. Abnormal pulmonary venous spectral Doppler findings were present in 25 of the 26 fetuses., Conclusions: The diagnosis of TAPVR can be suspected on standard axial views included in second-trimester obstetric screening examinations of the fetal heart and confirmed on fetal echocardiography with the use of pulsed wave Doppler imaging. Clues recognizable on obstetric sonographic screening have the potential to contribute to increasing the diagnostic yield for prenatal detection of TAPVR., (© 2014 by the American Institute of Ultrasound in Medicine.)

Background: Atrioventricular septal defects (AVSD) are very commonly diagnosed in utero. Heterotaxy/chromosomal abnormalities frequently coexist with AVSD. However, outcomes of fetal AVSD are not precisely known. We attempted to define mortality risk factors in AVSD., Method: We retrospectively searched our database, electronic records, and echocardiograms with diagnosis of fetal AVSD from 2003 to 2012. We investigated the following risk factors: atrial situs, heart rate/rhythm, ventricular dominance/morphology, atrioventricular valve regurgitation, cardiothoracic ratio, ejection fraction, and extracardiac anomalies., Results: Forty-five fetuses with a median gestational age of 28 weeks (17.5-37.1) were determined to have AVSD during the 10 years, of which 12 were either lost to follow-up (6) or underwent termination (6). There were 16 deaths (48%); two died in utero. Isomerism was identified in 17 of 45 (37%) fetuses (11 left atrial, 6 right atrial isomerism) and chromosomal abnormalities were identified in 12 (27%). Twenty-eight of 33 fetuses, not lost to follow-up or terminated, had extracardiac anomalies which had associated increased mortality (57% vs. 0%, P = .04). Heart block (75% vs. 43%, P = .12), left ventricular noncompaction (80% vs. 43%, P = .17), and isomerism (63% vs. 41%, P = .28) were associated with mortality but without statistical significance. Twenty-five of 45 (56%) had unbalanced AVSD. Positional abnormalities of the great arteries or semilunar valve stenosis were present in 20/45 (44%) while venous anomalies were present in 16/45 (36%). Presence of ventricular dominance, atrioventricular valve regurgitation, elevated cardiothoracic ratio, or diminished ejection fraction were not associated with mortality., Conclusion: Overall mortality rate for fetuses with AVSD was 48%. The presence of extracardiac anomalies is an independent risk factor for prediction of fetal or neonatal demise. Heart block, isomerism, and noncompaction in fetal AVSD appear to be associated with poor outcomes as well but did not reach statistical significance. This information is useful for counseling parents with fetus AVSD., (© 2013 Wiley Periodicals, Inc.)

This presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism in association with tricuspid atresia. Several distinctive sonographic features of isomerism of the right atrial appendages were present in this fetus: complex cardiac abnormality, ventriculoarterial discordance, juxtaposition of the aorta and the inferior vena cava to the right side, pulmonary atresia, and anomalous pulmonary venous return to the morphological right atrium. Tricuspid atresia, which is an extremely rare lesion within heterotaxy spectrum disorders, was present. Postnatal investigations confirmed all prenatally diagnosed abnormalities, with additional findings of pulmonary atresia with discontinuous pulmonary arteries and bilateral arterial ducts, asplenia, and bilateral eparterial bronchi. To our knowledge, tricuspid atresia in the setting of isomerism of the right atrial appendages has not previously been diagnosed or reported prenatally. Because of the complexity of cardiac lesions that may be present in cases of atrial isomerism, these disorders should be considered even if sonographic findings are uncommon or atypical.

The successful diagnosis, surgical planning, and long-term care of children with transposition of the great arteries require high-quality cardiac imaging with echocardiography. Echocardiography must identify the relevant anatomic variants of transposition of the great arteries, such as of ventricular septal defects and aortic arch anomalies. Methodical and detailed imaging of the coronary arteries is particularly important, as translocation of the coronary arteries is a critical component of the arterial switch procedure. Familiarity with the potential coronary artery variants and the ideal imaging planes is essential for an echocardiographer. Knowledge of both the early and late complications following the arterial switch procedure is essential to optimise post-operative echocardiography. These complications can include residual lesions leading to haemodynamic compromise or progressive late phenomena, such as aortic root dilatation and aortic insufficiency. Echocardiography will continue to be the cornerstone to the lifelong management of transposition of the great arteries, and improvements in technology and increased familiarity with modalities such as stress echocardiography will enhance the role of advanced imaging even further.

The patient is a 13-year-old Mexican-American immigrant who had no previous medical care. Upon arrival to the United States she was diagnosed with severe mitral valve stenosis and regurgitation. In addition she had severe aortic stenosis with mild to moderate aortic valve regurgitation, which was thought to be rheumatic heart disease. On the basis of the clinical and echocardiographic findings she was taken to the operating room for both mitral and aortic valve replacement. Her operation was complicated by the discovery that her aorta was completely calcified, as were her coronary arteries, mitral valve and aortic valve. She underwent aortic and mitral valve replacement as well as replacement of her aortic arch. Her coronary arteries were patch augmented and reimplanted into the newly created ascending aorta. Based on the pathologic examination of the surgical tissue a diagnosis of Gaucher disease was made., (© 2010, Wiley Periodicals, Inc.)

In the past, coronary arterial anomalies have been difficult to diagnose by non-invasive methods. Identification of coronary arterial origins is now a routine part of the standard paediatric echocardiogram. Anomalous origin of a coronary artery from the pulmonary trunk is an extremely important diagnosis to make. Many echocardiographic features are not directly related to the visualisation of the coronary arterial origin. Left ventricular dilation and abnormal ventricular performance are common, along with mitral regurgitation and evidence of collateralisation of the flow from the coronary artery that has an aortic origin. In some cases, the anomalous coronary artery can be seen to arise directly from the pulmonary trunk. Congenital atresia of the main stem of the left coronary artery has a similar echocardiographic presentation, except that its aortic origin is not determined. Anomalous aortic origin of the coronary artery has important implications, as the first presenting symptom can be sudden death. With meticulous attention to the origins of the coronary arteries, echocardiographic diagnosis can also be achieved. In contrast to the anomalous origin of a coronary artery from the pulmonary trunk, ventricular performance is usually normal. Whenever there is doubt as to the definition of the origin of the coronary arteries and, indeed, when there is serious clinical concern that a coronary artery has an anomalous origin, other testing, such as cine-computed tomography, magnetic resonance imaging, or cardiac catheterisation may be indicated for confirmation or to provide greater anatomic detail.

There is a collection of rare congenital cardiac defects that can produce significant haemodynamic embarrassment. Owing to their rarity, these lesions may be overlooked or mistaken for other more common congenital cardiac defects. Using careful segmental echocardiographic techniques, such as multiple planes of imaging and inferential Doppler findings, it is possible to identify these lesions and thus plan surgical management. Some of the lesions of importance reviewed include the aortopulmonary window, the aorto-ventricular tunnels, the aorto-atrial tunnels, and aneurysm of the sinuses of Valsalva. Some conditions such as the aortopulmonary window and aneurysm of the sinuses of Valsalva often occur in the setting of other congenital cardiac lesions. At times, the presence of coexisting lesions may mask some of the characteristic features of these defects. With prompt identification, all of these rare conditions are amenable to complete surgical repair. Newer modalities such as three-dimensional echocardiography can further enhance surgical planning.

Objective: To determine whether a continuous infusion of heparin reduces the rate of catheter-related thrombosis in neonates and infants post cardiac surgery. Central venous and intracardiac catheters are used routinely in postoperative pediatric cardiac patients. Catheter-related thrombosis occurs in 8% to 45% of pediatric patients with central venous catheters., Design: Single-center, randomized, placebo-controlled, double-blinded trial., Setting: Cardiovascular intensive care unit, university-affiliated children's hospital., Patients: Children <1 yr of age recovering from cardiac surgery., Interventions: Patients were randomized to receive either continuous heparin at 10 units/kg/hr or placebo. The primary end point was catheter-related thrombosis as assessed by serial ultrasonography., Results: Study enrollment was discontinued early based on results from an interim futility analysis. Ninety subjects were enrolled and received the study drug (heparin, 53; placebo, 37). The catheter-related thrombosis rate in the heparin group, compared with the placebo group, was 15% vs. 16% (p = .89). Subjects in the heparin group had a higher mean partial thromboplastin time (52 secs vs. 42 secs, p = .001), and this difference was greater for those aged <30 days (64 secs vs. 43 secs, p = .008). Catheters in place > or = 7 days had both a greater risk of thrombus formation (odds ratio, 4.3; p = .02) and catheter malfunction (odds ratio, 11.2; p = .008). We observed no significant differences in other outcome measures or in the frequency of adverse events., Conclusions: A continuous infusion of heparin at 10 units/kg/hr was safe but did not reduce catheter-related thrombus formation. Heparin at this dose caused an increase in partial thromboplastin time values, which, unexpectedly, was more pronounced in neonates.

Objectives: We report our recent experience with atrioventricular septal defect (AVSD) diagnosed in utero., Methods: We reviewed fetal echocardiograms diagnosed with AVSD between November 2002 and November 2004, comparing fetuses with and without aneuploidy. We compared results with previous studies., Results: Twenty (1.8%) fetuses had AVSD. Mean maternal age was 33 years (range 19-43). Mean gestational age was 26 weeks (range 18-38). Indications for fetal echocardiography were: abnormal obstetrical ultrasound (75%), chromosomal anomaly (15%), undetermined (10%). AVSD was an isolated cardiac defect in 5 (25%), associated with double-outlet right ventricle (9) or tetralogy of Fallot (3) in 12 (60%). Four had aortic arch anomalies. Atrioventricular valve regurgitation was mild in 7 (35%) and moderate in 4 (20%). Heart block existed in 2 (10%). Five (25%) with trisomy had Rastelli type A AVSD as a single lesion (odds ratio 24, P < .01). Extracardiac anomalies existed in 6, with and without aneuploidy. Pregnancy was terminated in 4 (20%), neonatal death in 4 (20%), and reparative surgery in 6 (30%), not ascertained in 6., Conclusion: Atrioventricular septal defect is usually an isolated cardiac lesion in fetuses with aneuploidy. In the absence of aneuploidy, fetal AVSD is often associated with conotruncal and aortic arch abnormalities, which are important in determining outcomes. Pregnancy termination and neonatal death continue to be prevalent.

We prospectively studied 29 consecutive neonates undergoing an arterial switch operation to determine if segmental wall motion abnormalities (SWMA) represented myocardial ischemia. Intraoperative transesophageal echocardiogram was recorded at baseline and twice after cardiopulmonary bypass. Cardiac troponin I (cTnI) levels were measured before sternal incision and 3, 6, 12, 24, 48, and 72 h after removal of the aortic cross-clamp. Immediate postoperative Holter and 15-lead electrocardiograms (ECG) were evaluated for ischemia. Transthoracic echocardiograms were obtained before hospital discharge. At bypass termination, immediately after protamine administration, segmental wall motion was normal in nine neonates and abnormal in 20. SWMA were transient in five and present at the time of chest closure in 15 neonates. Neonates in whom SWMA were present at chest closure had more segments involved than those in whom SWMA were transient (P > 0.001). Neonates with SWMA at chest closure had higher cTnI levels postoperatively versus neonates with normal wall motion (P = 0.02). Postoperative ECG data were available in 26 neonates. There was ECG evidence of myocardial ischemia in two of eight neonates with normal wall motion, one of five with transient SWMA, and nine of 13 with SWMA at chest closure. CTnI levels at 12, 24, and 48 h and intraoperative SWMA were predictive of postoperative SWMA. We believe these data indicate that SWMA, which persist at the completion of an arterial switch operation, and which are present in multiple myocardial segments, correlate with myocardial ischemia. Further follow-up of these patients is needed to determine if increased intraoperative myocardial ischemia correlates with long-term outcomes.

Systole (S) and diastole (D) are fundamental to cardiac function, yet their durations are not routinely evaluated. We defined the S/D duration ratio in 11 children with restrictive cardiomyopathy (CM) and 31 control subjects, using tricuspid regurgitant flow duration. We correlated S/D ratio, S duration, and D duration with heart rate and compared groups. Heart rates of control subjects and patients with restrictive CM were similar (P = .995). The S/D ratio was higher in patients with restrictive CM than in control subjects (1.6 +/- 0.81 vs 0.8 +/- 0.19, P < .001) as a result of shortened D (0.43 +/- 0.13 vs 0.56 +/- 0.06, P < .01) and prolonged S (0.58 +/- 0.12 vs 0.44 +/- 0.06, P < .01). The S/D ratio was highly correlated to heart rate in restrictive CM (r = 0.95, P < .0001). The high S/D ratio found in restrictive CM, found also in dilated CM, suggests that this index is generic to heart failure rather than to a specific disease. The S/D ratio is easily measured using Doppler flow, enhancing echocardiographic assessment of D function in children.

This report describes two infants with an aortopulmonary window in association with anomalous origin of the right coronary artery from the pulmonary artery. In both cases the diagnosis was made pre-operatively by transthoracic echocardiography, with the initial clue being extensive collateral flow within the myocardium. In each case there was surgical confirmation of the echocardiographic findings. These two cases demonstrate that coronary artery evaluation should be an integral part of every new echocardiographic evaluation, particularly in the setting of conotruncal anomalies.

Objectives: To examine the echocardiographic features, associated anomalies, and outcomes of fetuses diagnosed with double outlet right ventricle (DORV)., Methods: Fetuses with DORV were identified from 987 fetal echocardiograms performed from August 1, 2002 to August 1, 2004. The medical records of 19 fetuses with DORV were reviewed., Results: Nineteen fetuses were diagnosed with DORV. The pregnancy was terminated in 15.8% (3/19) of cases. Postnatal assessment was not obtained in 26.3% (5/19) cases. Accurate prenatal diagnosis of the relationship of the ventricular septal defect (VSD) with the great arteries was achieved in 14 of 14 cases (100%). Chromosomal abnormalities (21.4%), abnormalities of body situs (35.7%), and other extracardiac abnormalities were diagnosed in association. Only 4 of 13 (30.8%) infants with confirmed DORV survived beyond 28 days., Conclusions: DORV is found in fetuses with a large spectrum of associated cardiac and extracardiac lesions. The overall prognosis for fetuses with DORV is poor throughout the spectrum of these lesions, not only related to the primary lesion but also depending on the associated intra- and extracardiac abnormalities. While the complexity of the heart disease in fetuses with DORV makes detailed fetal diagnosis challenging, it can be accomplished with a high degree of accuracy.

Systole and diastole are the fundamental periods of the cardiac cycle, yet little emphasis has been placed on their relative duration when evaluating heart failure. Cardiac intervals are used to assess ventricular function, but the relative duration of systole and diastole for defining function have not been evaluated. We hypothesized that in heart failure, systole is prolonged and diastole shortened. We defined systole and diastole in 16 children with idiopathic dilated cardiomyopathy and in 16 normal controls, matched for age and gender, using the mitral regurgitant (MR) and tricuspid regurgitant (TR) flow duration. The systole and diastole durations (expressed as a fraction of the cardiac cycle) were correlated with heart rate and age and compared between groups. The subjects were compared with gender- and age-matched controls (9.98 +/- 6.1 vs 9.88 +/- 6.08 years, p = NS). The 2 groups had similar heart rates (104 +/- 31 vs 92 +/- 34 beats/min, p = NS). The systole duration was not significantly different when measured by MR versus TR duration (0.60 +/- 0.10 vs 0.57 +/- 0.11 of the cardiac cycle, respectively, p = NS). Systole was prolonged in subjects compared with controls (0.60 +/- 0.1 vs 0.42 +/- 0.08, respectively, using MR duration, p < 0.0001 and 0.57 +/- 0.11 vs 0.41 +/- 0.07, respectively, using TR duration, p = 0.0008). The systolic/diastolic ratio was 0.77 +/- 0.24 in the controls versus 1.57 +/- 0.98 in the patients with idiopathic dilated cardiomyopathy using the TR duration (p < 0.005) and 1.67 +/- 0.68 using the MR duration (p < 0.0001). The systole duration correlated with heart rate in subjects (r = 0.79, p = 0.0003) and controls (r = 0.69, p = 0.003). In conclusion, systole is significantly prolonged and diastole correspondingly shortened in heart failure. Reversal of the normal systolic/diastolic ratio may compromise cardiac filling and function. The systole and diastole duration are easily measured using routine Doppler flow, enhancing assessment of cardiac function in children.

This study investigated the rare association of ventricular noncompaction with isomerism of the left atrial appendage and heart block, demonstrating a dire prognosis for this condition. Prenatal recognition is possible by ultrasound, facilitating appropriate prenatal counseling.

Color M-mode (CMM) Doppler flow propagation velocity (Vp) is a reliable diastolic index, but may be difficult to define. We hypothesized that because the valve is opened by flow, the anterior mitral leaflet excursion velocity (MEVApex ), interrogated from the cardiac apex, may be similar to the CMM-Vp. We investigated the MEVApex by apical M-mode echocardiography in 49 children and adolescents with normal echocardiograms, and compared it with the CMM-Vp and other indices. The mean CMM-Vp and MEVApex were similar (41.5 +/- 6 vs 40 +/- 5.7, respectively, mean +/- SD; P = .5) and correlated positively ( r = 0.71, P < .0001, Bland-Altman analysis). The peak velocity of mitral inflow (E wave) divided by the Vp (E/Vp) and E/MEVApex correlated well (2.54 +/- 0.58 vs 2.52 +/- 0.58, respectively, mean +/- SD; r = 0.857, P < .001, Bland-Altman analysis). The MEVApex is comparable with the CMM-Vp and the E/MEVApex with E/Vp. The Vp may be assessed either by CMM or conventional M-mode from the apex. In our experience, the latter is easier to do. Study in other populations is warranted.

Background: The Tei index is a useful echocardiographic measure of ventricular function in adults and children. Most studies have used pulse or tissue Doppler imaging to calculate this index. M-mode, with its higher frame rate and sharp deflections, may be an accurate method of calculating the Tei index., Methods: M-mode and pulse Doppler (PD) measurement of the Tei index were performed in 68 children. The mitral closure to opening time ("a") and ejection time ("b") intervals for calculating the Tei index were measured by M-mode and PD methods., Results: Both M-mode and PD were found to be reproducible methods of calculating the Tei index. There was a close correlation between measurements of the "a" and "b" intervals by M-mode and PD, with less variability with M-mode than with PD., Conclusion: M-mode is a simple, reproducible echocardiographic alternative method for calculating the Tei index in children and has a lower variance than PD.

Objectives: We hypothesized that increased facility with fetal echocardiographic diagnosis by obstetricians is associated with changes in its indications and yields., Methods: We reviewed 300 fetal echocardiograms (December 2002-August 2003) and compared our findings with previous studies., Results: Mean maternal age was 31 +/- 6 (range 16-44) years. Gestational age was 24 +/- 5 weeks (mean +/- SD; median 22, range 15-38). Indications for fetal echocardiography included family history of congenital heart disease (CHD) (23%), maternal diabetes (18%), obstetrical scan suspicious for CHD (13%), arrhythmia (12%) maternal rheumatologic disease (7%), extracardiac congenital anomalies (6%), chromosomal anomaly (6%) and exposure to a potential fetal teratogen (5%). High yield indications included chromosomal anomaly (47%) and a suspicious obstetrical scan (42%). Low yield indications included family history of CHD (4%) and teratogen exposure (0%). 1/7 of the patients with increased nuchal translucency had pulmonary atresia/intact ventricular septum. No anomalies were associated with the single umbilical artery., Conclusion: Indications and yields of fetal echocardiography have changed over the last decade. The frequency of an obstetrical scan suspicious for CHD has increased 2.5 to 3 times over a decade and continues to have high yield. Thus, increasing prenatal detection of CHD depends, to a large extent, on increasing the skills of obstetricians., (Copyright (c) 2004 John Wiley & Sons, Ltd.)

We evaluated echocardiographic accuracy for defining coronary artery course in d-transposition of great arteries and the impact of a 2-reviewer method on this accuracy. The echocardiogram reports of 108 patients with d-transposition of great arteries were reviewed for coronary anatomy and compared with the operative report. In method 1, from January 1995 to December 1997, a single reader performed the echocardiogram. In method 2, from January 1998 to December 2000, 2 readers scanned individually and a consensus diagnosis was made. Comparing methods 1 and 2, the sensitivity of the echocardiogram to detect variants in coronary anatomy was 68% versus 86%, and negative predictive value was 72% versus 91%. Using a 2-reviewer method improved the accuracy of echocardiographic diagnosis of coronary anatomy with d-transposition of great arteries, aiding in risk assessment and treatment of the patient preoperatively. This study also illustrates that echocardiographic accuracy may be lower in an institution with a surgical volume more representative of the usual pediatric cardiothoracic surgical center.

Objective: Pericardial teratomas are rare congenital tumors which invade the developing mediastinum, compressing the venous return to the heart, leading to hydrops. Tumors, with large cystic components, have been treated previously with in utero pericardiocentesis with some success. We present the first reported case of in utero open resection of a fetal pericardial teratoma., Methods: A 31-year-old G1P0 woman was found to have a fetus with a pericardial teratoma. Hydrops developed at 24 weeks' gestation. After counseling, open fetal resection was performed via a fetal median sternotomy., Results: Although the tumor was successfully removed, the hydrops did not resolve. In addition, over the course of 3 weeks, the mother developed maternal mirror syndrome which prompted an emergent cesarean section. Neonatal death ensued shortly after birth., Conclusions: The fetus with a pericardial teratoma complicated by hydrops is compromised. Treatment options include early delivery, aspiration of the pericardial effusion, and in utero operative resection., (Copyright 2002 S. Karger AG, Basel)

Objectives: This study sought to compare the safety, efficacy and clinical utility of the Amplatzer septal occluder (ASO) for closure of secundum atrial septal defect (ASD) with surgical closure., Background: The clinical utility of a device such as the ASO can only be judged against the results of contemporaneous surgery., Methods: A multicenter, nonrandomized concurrent study was performed in 29 pediatric cardiology centers from March 1998 to March 2000. The patients were assigned to either the device or surgical closure group according to the patients' option. Baseline physical exams and echocardiography were performed preprocedure and at follow-up (6 and 12 months for device group, 12 months for surgical group)., Results: A total of 442 patients were in the group undergoing device closure, whereas 154 patients were in the surgical group. The median age was 9.8 years for the device group and 4.1 years for the surgical group (p < 0.001). In the device group, 395 (89.4%) patients had a single ASD; in the surgical group, 124 (80.5%) (p = 0.008) had a single ASD. The size of the primary ASD was 13.3 +/- 5.4 mm for the device group and 14.2 +/- 6.3 mm for the surgery group (p = 0.099). The procedural attempt success rate was 95.7% for the device group and 100% for the surgical group (p = 0.006)., Conclusions: The early, primary and secondary efficacy success rates were 94.8%, 98.5% and 91.6%, respectively, for the device group, and 96.1%, 100% and 89.0% for the surgical group (all p > 0.05). The complication rate was 7.2% for the device group and 24.0% for the surgical group (p < 0.001). The mean length of hospital stay was 1.0 +/- 0.3 day for the device group and 3.4 +/- 1.2 days for the surgical group (p < 0.001). Mortality was 0% for both groups. The early, primary and secondary efficacy success rates for surgical versus. device closure of ASD were not statistically different; however, the complication rate was lower and the length of hospital stay was shorter for device closure than for surgical repair. Appropriate patient selection is an important factor for successful device closure. Transcatheter closure of secundum ASD using the ASO is a safe and effective alternative to surgical repair.

Background: Maternal anti-Ro and anti-La antibodies are associated with congenital heart block (CHB). Although endocardial fibroelastosis (EFE) has been described in isolated cases of autoantibody-mediated CHB, the natural history and pathogenesis of this disease are poorly understood., Methods and Results: We retrospectively reviewed the clinical history, echocardiography, and pathology of fetuses and children with EFE associated with CHB born to mothers positive for anti-Ro or anti-La antibodies at 5 centers. Thirteen patients were identified, 6 with a prenatal and 7 with a postnatal diagnosis. Six mothers were positive for anti-Ro and anti-La antibodies, and 7 were positive for anti-Ro antibodies only. Only 1 mother had autoimmune disease. Severe ventricular dysfunction was seen in all fetal and postnatal cases. Four fetal and 3 postnatal cases had EFE at initial presentation. However, 2 fetal and 4 postnatal cases developed EFE 6 to 12 weeks and 7 months to 5 years from CHB diagnosis, respectively, even despite ventricular pacing in 6 postnatal cases. Eleven (85%) either died (n=9) or underwent cardiac transplantation (n=2) secondary to the EFE. Pathologic assessment of the explanted heart, available in 10 cases, revealed moderate to severe EFE in 7 and mild EFE in 3 cases, predominantly involving the left ventricle. Immunohistochemistry in 4 cases (including 3 fetuses) demonstrated deposition of IgG in 4 and IgM in 3 and T-cell infiltrates in 3 cases, suggesting an immune response by the affected fetus or child., Conclusions: EFE occurs in the presence of autoantibody-mediated CHB despite adequate ventricular pacing. Autoantibody-associated EFE has a very high mortality rate, whether developing in fetal or postnatal life.