Right Ventricular Remodeling in Hypoplastic Left Heart Syndrome is Minimally Impacted by Cardiopulmonary Bypass: A Comparison of Norwood vs. Hybrid.

Right ventricular (RV) remodeling in hypoplastic left heart syndrome (HLHS) begins prenatally and continues through staged palliations. However, it is unclear if the most marked observed remodeling post-Norwood is secondary to cardiopulmonary bypass (CPB) exposure or if it is an adaptation intrinsic to the systemic RV. This study aims to determine the impact of CPB on RV remodeling in HLHS. Echocardiograms of HLHS survivors undergoing stage 1 Norwood (n = 26) or Hybrid (n = 20) were analyzed at pre- and post-stage 1, pre- and post-bidirectional cavo-pulmonary anastomosis (BCPA), and pre-Fontan. RV fractional area change (FAC), vector velocity imaging for longitudinal & derived circumferential deformation (global radial shortening (GRS) = peak radial displacement/end-diastolic diameter), and deformation ratio (longitudinal/ circumferential) were assessed. Both groups had similar age, clinical status and functional parameters pre-stage 1. No difference in RV size and sphericity at any stage between groups. RVFAC was normal (> 35%) throughout for both groups. Both Norwood and Hybrid patients had increased GRS (p = 0.0001) post-stage 1 and corresponding unchanged longitudinal strain, resulting in decreased deformation ratio (greater relative RV circumferential contraction), p = 0.0001. Deformation ratio remained decreased in both groups in subsequent stages. Irrespective of timing of the first CPB exposure, both Norwood and Hybrid patients underwent similar RV remodeling, with relative increase in circumferential to longitudinal contraction soon after stage 1 palliation. The observed RV remodeling in HLHS survivors were minimally impacted by CPB.

Impaired Left Ventricular Reserve in Childhood Cancer Survivors Treated With Anthracycline Therapy.

BACKGROUND: Childhood cancer survivors show evidence of diffuse myocardial fibrosis that is related to exercise capacity. The mechanism of reduced exercise tolerance in anthracycline cardiotoxicity remains unclear. We explored the determinants of exercise intolerance by evaluating left ventricular (LV) distensibility and functional reserve. METHODS: Patients (n = 22) and healthy controls (n = 10) underwent two-dimensional echocardiography while supine, upright, and during cycle exercise. LV distensibility was measured as the change in end-diastolic cavity area (EDCA) from supine to the upright position. LV functional reserve was assessed during peak exercise, and measured as the exercise-induced change in systolic circumferential strain rate (SR) and early-diastolic SR (EDSR). The peak rate of oxygen consumption was measured by indirect calorimetry. RESULTS: Median age of patients was 16 years (range 8-19) and controls 14 years (range 8-19). Median time since anthracycline therapy was 6 years (range 2-16). Peak oxygen consumption was significantly lower in patients compared to controls (35 ml/kg/min [28-60] vs. 45 ml/kg/min [44-53], P = 0.005). Transitioning from the supine position to the upright position caused a similar reduction in LV EDCA, suggesting similar LV distensibility between patients (-22% [-46 to -4]) and controls (-20% [-46 to -3], P = 0.3). However, during exercise, both systolic SR and EDSR reserve were significantly impaired in patients (∆SR: 93% [14-308], ∆EDSR: -4.5% [-88 to 121]) compared to controls (∆SR: 128% [54-230], P = 0.046; ∆EDSR: 74% [22-234], P = 0.02). CONCLUSIONS: Our findings suggest that impaired LV contractility and functional reserve play a role in the reduced exercise capacity in anthracycline cardiotoxicity rather than LV distensibility.

Three-dimensional echocardiography for the assessment of atrioventricular valves in congenital heart disease: past, present and future.

Echocardiography has developed as an imaging technology over 60 years to become the mainstay for investigating heart disease, providing invaluable structural and functional information. In the last 20 years, 3-dimensional echocardiography (3DE) has emerged as an adjunct to 2-dimensional echocardiography in adult and congenital heart disease. Early work with 3-dimensional imaging of the mitral valve describing normal annular shape and function significantly changed the understanding of mitral valve dynamics. Further work led to our current understanding of the mitral valve working as a unit, with all components vital to its normal function. With improving technology and ease of use, similar 3DE techniques have been used in congenital heart disease to study the unique anatomy and function of atrioventricular (AV) valves, specifically the tricuspid valve in hypoplastic left heart syndrome, and the left AV valve in atrioventricular septal defects. This paper describes the role of 3DE in assessing AV valve function in normal valves, and in congenital heart disease.

A Unique Intracardiac Shunt: Identifying Complex Anatomy in Reoperative Adult Congenital Heart Disease.

Adult patients with repaired congenital heart disease are presenting with previously unseen types of residual lesions and consequences of prior repair. Patients with d-transposition of the great arteries repaired with atrial switch operations are returning with dysrhythmias and atrioventricular valve disease requiring intervention. We present the challenging case of a young adult with a residual shunt identified on preoperative three-dimensional transthoracic echocardiography, the precise anatomy of which was only characterized intraoperatively.

Tricuspid Valve Adaptation during the First Interstage Period in Hypoplastic Left Heart Syndrome.

BACKGROUND: Tricuspid regurgitation (TR) is an important risk factor for morbidity and mortality in hypoplastic left heart syndrome (HLHS), yet the evolution of tricuspid valve (TV) dysfunction in HLHS is poorly understood. This study sought to examine changes in TV function in HLHS between the first two stages of surgical palliation and to determine the mechanism of TR at the time of stage two surgery-bidirectional cavopulmonary anastomosis (BCPA). METHODS: We prospectively investigated 44 infants at two time points-prior to Norwood-Sano (T1 - median age 5.4 days) and prior to BCPA (T2 - median age 4.7 months) using two-dimensional (2DE) and three-dimensional echocardiography (3DE). Right ventricular (RV) size, function and shape was assessed with 2DE. Extracted spatial coordinates from 3DE were used to calculate TV leaflet and annular area, tethering and prolapse volumes, bending angle, and coaptation index. TR was graded qualitatively, and 2D and 3D vena contracta (VC) were measured. RESULTS: The cohort from T1 to T2 had increased indexed leaflet and annular area (P < .0001) and tethering volume (P < .0001), with no change in coaptation. Significant TR was present in 14 infants (32%) at T2 and was associated with greater leaflet (P = .02) and annular areas (P = .002) and greater prolapse volume (P = .008), but not tethering volume or reduced coaptation. At latest follow-up (median 23 months), 13 patients died or required transplantation. Only 3DE VC at T2 was associated with death or transplantation. CONCLUSIONS: The TV in HLHS adapts to interstage stressors (increased preload and afterload) by increasing leaflet size to maintain adequate leaflet coaptation. Significant TR at T2 was associated with greater leaflet size and prolapse. This may represent TV maladaptation from an excessive response in leaflet expansion to stressors.

Determinants of outcome in fetal pulmonary valve stenosis or atresia with intact ventricular septum.

Pulmonary valve stenosis or atresia with intact ventricular septum represents a spectrum of severity. This study aimed to identify ultrasound markers of biventricular versus non-biventricular outcome. The fetal echocardiograms of 41 fetuses diagnosed with pulmonary stenosis or atresia and right ventricular (RV)/left ventricular (LV) length ratios >0.4 from 17 to 31 weeks of gestation were reviewed. Of 27 live-born patients with intention to treat, 8 had non-biventricular outcomes and 19 had biventricular circulation. At the time of diagnosis, poor RV function, flow reversal in the arterial duct, the degree of tricuspid valve (TV) regurgitation, and inferior vena cava Doppler flow pattern did not differ between the 2 outcome groups. However, RV sinusoids, the RV/LV length ratio, the TV/mitral valve ratio, and TV inflow duration were significantly different. Cut-off values derived from receiver-operating characteristic curves yielding the best sensitivity and specificity for a non-biventricular outcome were TV/mitral valve ratio <0.7, RV/LV length ratio <0.6, TV inflow duration <31.5% of cardiac cycle length, and the presence of RV sinusoids. If 3 of these 4 criteria were fulfilled, this predicted a non-biventricular outcome with sensitivity of 100% and specificity of 75%. In conclusion, in fetuses < or =31 weeks of gestation with pulmonary stenosis or atresia and intact ventricular septum, progression to a non-biventricular outcome can be predicted by a 4-criterion scoring system. The criteria may be useful in selecting fetuses for prenatal catheter intervention to prevent progressive RV hypoplasia.

Impaired Single Right Ventricular Function Compared to Single Left Ventricles during the Early Stages of Palliation: A Longitudinal Study.

BACKGROUND: Single right ventricles (SRV) are postulated to be disadvantaged compared with single left ventricles (SLV). We compared the evolution of SRV versus SLV function during infancy using conventional measures and speckle-tracking echocardiography (STE). We hypothesized that the SRV is mechanically disadvantaged during early infancy. METHODS: SRVs (n = 32) were compared with SLVs (n = 16) at the neonatal (presurgery) and pre-bidirectional cavopulmonary anastomosis (pre-BCPA) stages. Functional measures (fractional area change, indexed ventricular annular plane systolic excursion [iVAPSE], isovolumic acceleration [IVA], myocardial performance index, E and A velocities, tissue Doppler imaging annular velocities and STE-measured global longitudinal and circumferential strain, strain rate [SR], and early diastolic SR [EDSR]) were compared between SRV and SLV at each stage and between presurgery and pre-BCPA. RESULTS: Compared with SLV, presurgery SRV had lower circumferential strain (-10.6% vs -16.5%; P = .0002) and EDSR (1.41%/sec vs 2.13%/sec; P = .001). Pre-BCPA SRV had decreased IVA (1.2 vs 2.1 m/sec2; P = .006): longitudinal strain (-15.3% vs -19.1%; P = .001), SR (-0.97%/sec vs -1.53%/sec; P = .0001), EDSR (1.5%/sec vs 2.1%/sec; P = .001); circumferential strain (-10.6% vs -14.9%; P = .002), SR (-0.8%/sec vs -1.21%/sec; P = .0001), and EDSR (1.3%/sec vs 1.8%/sec; P = .009). SRV showed reduction of iVAPSE, IVA, s', e', a' velocities, longitudinal strain, SR, EDSR, and circumferential SR (P < .05) from presurgery to pre-BCPA, while circumferential strain was unchanged. SLV showed no significant change in these parameters during this interval. CONCLUSIONS: The progressive reduction in SRV longitudinal and circumferential function suggests that SRV may have a mechanical disadvantage from birth and progressive impairment with age.

Clinical features, management, and outcome of children with fetal and postnatal diagnoses of isomerism syndromes.

BACKGROUND: Isomerism is associated with a complex spectrum of anomalies. There is paucity of data on prenatally detected cases. METHODS AND RESULTS: Between January 1990 and February 2004, 83 of 166 cases (50%) had a prenatal diagnosis of left isomerism (LAI; 52 of 97) or right isomerism (RAI; 31 of 69) at our institution. The spectrum of anomalies, management, and outcomes was compared for fetal and postnatal diagnoses of LAI and RAI. RAI more often than LAI was associated with AV septal defect (90% versus 56%; P<0.0001), pulmonary outflow obstruction (91% versus 37%; P<0.0001), total anomalous pulmonary venous drainage (73% versus 13%; P<0.0001), and abnormal VA connections (68% versus 33%; P<0.0001), whereas inferior vena cava interruption (3% versus 93%; P<0.0001), complete AV block (0% versus 13%; P=0.004), aortic obstruction (6% versus 33%; P<0.0001), and extracardiac defects (5% versus 25%; P=0.006) were less common. The spectrum of lesions was comparable for fetal and postnatal cases, except for AV block (fetal, 25%; postnatal, 0%; P=0.0002) and AV septal defect (fetal, 67%; postnatal, 42%; P=0.023) in LAI. Fetal demise was due mainly to pregnancy termination (LAI, 42%; RAI, 45%). Survival of actively managed children with LAI was significantly better than for those with RAI (P<0.0001) but did not differ with regard to fetal versus postnatal diagnosis. Most LAI cases required no intervention or underwent successful biventricular cardiac surgery (65%), unlike RAI cases (13%; P<0.0001). CONCLUSIONS: Prenatal diagnosis did not affect overall survival despite facilitated care. The prognosis of RAI was worse compared with LAI because of more complex associated cardiac defects and the inability to perform successful surgical procedures.

Borderline left ventricles in prenatally diagnosed atrioventricular septal defect or double outlet right ventricle: echocardiographic predictors of biventricular repair.

BACKGROUND: Atrioventricular septal defect (AVSD) and double outlet right ventricle (DORV) with normally related great arteries and normal ventricular sizes are associated with a good long-term prognosis after biventricular (BV) repair. The outcome of cases with a borderline small left ventricle (bLV) is unclear. The purpose of the study was to retrospectively determine echocardiographic predictors of successful BV repair in fetuses with AVSD or DORV with a bLV. METHODS AND RESULTS: From 1991 to 2004, 24 fetuses with AVSD plus bLV and 24 with DORV plus bLV were identified. Fetal echocardiographic parameters comparing BV repair versus single ventricle (SV) palliation were obtained, including the presence or absence of an apex-forming bLV was recorded. A bLV was defined as a right ventricular/left ventricular end-diastolic dimension ratio between 2 and 4 SDs for gestational age. The overall survival from fetal diagnosis was 21% (5/24) for AVSD/bLV and 13% (3/24) for DORV/bLV. Of 11 liveborns with AVSD/bLV and 8 liveborns with DORV/bLV, 6 underwent BV repair (5 survivors), 7 SV palliation (3 survivors), and 1 cardiac transplant. Five infants receiving compassionate care only were excluded from the analysis. Parameters such as ratio of valve annuli, ventricular end-diastolic dimensions, degree of valve regurgitation, and the presence of endocardial fibroelastosis were not too predictive of outcome. The presence of an apex-forming bLV was the only predictor of BV repair (6/6 BV repair vs 2/8 SV palliation, P < .05). CONCLUSIONS: Prenatally diagnosed AVSD or DORV with bLV has a very poor prognosis. An apex-forming bLV predicts successful BV repair and is an important prognostic indicator.

Impact of selective laser ablation of placental anastomoses on the cardiovascular pathology of the recipient twin in severe twin-twin transfusion syndrome.

OBJECTIVES: We investigated the impact of selective laser ablation on the cardiovascular pathology of the recipient twin in twin-twin transfusion syndrome. STUDY DESIGN: Fetal echocardiograms and medical records were reviewed from 22 pregnancies with severe twin-twin transfusion syndrome where echocardiography was performed before and after laser. RESULTS: Before laser, cardiomegaly associated with right and/or left ventricular hypertrophy without ventricular dilatation, was observed in most cases. Right ventricular and left ventricular systolic dysfunction (shortening fraction <28%) was present in 59% and 27%, respectively, and diastolic dysfunction (based on inflow and venous Dopplers) in 73%. Shortly after laser, biventricular systolic function improved significantly and diastolic function tended to improve (50%, P = .06). Functional pulmonary atresia, secondary to right ventricular systolic dysfunction, resolved in 2 of 2 cases at post-laser echocardiography. On serial assessment, diastolic function was normal in 7 of 10, hydrops regressed in 4 of 5, and neither progressive myocardial hypertrophy nor anatomical right ventricular outflow obstruction were found. CONCLUSIONS: Selective laser ablation in severe twin-twin transfusion syndrome acutely improves biventricular systolic function and tends to improve diastolic function in the recipient twin.

Adverse neonatal and cardiac outcomes are more common in pregnant women with cardiac disease.

BACKGROUND: Pregnant women with heart disease (HD) are at increased risk for cardiac (CV) complications. However, the frequency of neonatal (NE) complications in pregnant women with HD relative to pregnant women without HD has not been examined. METHODS AND RESULTS: Pregnant women with HD were prospectively monitored during 302 pregnancies. The frequency of NE and CV complications was compared with those in a control group without HD during 572 pregnancies. The frequency of NE complications was higher in the HD group (18% versus 7%; HD versus controls). The NE complication rate was lowest in pregnancies of women age 20 to 35 years who did not smoke during pregnancy, did not receive anticoagulants, and had no obstetric risk factors: 4% in control patients, 5% in HD patients with no cardiac risk factors for NE complications (left heart obstruction, poor functional class, or cyanosis), and 7% in HD patients with > or =1 such risk factor. In contrast, the event rate in pregnancies of controls age <20 or >35 years who had obstetric risk factors or multiple gestation or who smoked was 11%. In the HD group, women age <20 or >35 years who had obstetric risk factors or multiple gestation, who smoked, or who received anticoagulants experienced an even higher NE complication rate (27% with no cardiac risks for NE events and 33% in the presence of >or =1 cardiac risk factors). The frequency of CV complications was higher in the HD group (17% versus 0%; HD versus controls). CONCLUSION: Pregnant women with HD are at increased risk for both NE and CV complications. The risk for NE adverse events in pregnant women with HD is highest in those with both obstetric and cardiac risk factors for NE complications.

Fetal cardiomyopathies: pathogenic mechanisms, hemodynamic findings, and clinical outcome.

BACKGROUND: Although the prenatal diagnosis of most fetal structural heart defects and dysrhythmias has been described, there is a paucity of information about cardiomyopathies (CMs) in prenatal life. METHODS AND RESULTS: To determine the pathogenic mechanisms, hemodynamic findings, and outcome of fetal CM, we reviewed the fetal echocardiograms and perinatal histories of 55 affected fetuses. Dilated CM was diagnosed in 22 cases, including 2 with congenital infections, 5 familial cases, 6 with endocardial fibroelastosis related to maternal anti-Ro/La antibodies, and 9 idiopathic cases. Thirty-three had hypertrophic CM, 7 associated with maternal diabetes, 2 with Noonan's syndrome, 2 with alpha-thalassemia, 18 with twin-twin transfusion syndrome, 1 with familial hypertrophy, and 3 with idiopathic hypertrophy. Systolic dysfunction was present in all cases of dilated CM and 15 cases of hypertrophic CM. Diastolic dysfunction was present in 19 of 30 fetuses with assessment of diastolic function parameters. Significant mitral or tricuspid valve regurgitation was seen in 32 cases. Eight fetuses were hydropic and 23 had signs of early hydrops. Seven pregnancies were terminated. Of 46 continued pregnancies with follow-up, 29 (63%) died perinatally. The presence of systolic dysfunction, diastolic dysfunction, and significant atrioventricular valve regurgitation were identified as risk factors for mortality. By multiple logistic regression, diastolic dysfunction was associated with an 8-fold increased risk relative to the other parameters. CONCLUSIONS: Fetal CM has a broad spectrum of intrinsic and extrinsic causes. A poor outcome is observed in many affected fetuses. Diastolic dysfunction in fetal CM is associated with the highest risk of mortality.

Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis.

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.

The relative roles of transthoracic compared with transesophageal echocardiography in children with suspected infective endocarditis.

OBJECTIVES: The study evaluated the additional benefit of transesophageal echocardiography (TEE) versus transthoracic echocardiography (TTE) in pediatric cases with suspected bacterial endocarditis. BACKGROUND: In adult patients, TTE has a lower sensitivity and specificity than TEE for the detection of vegetations or aortic root abscess formation. Few data are available about the relative benefits of TEE over TTE in the pediatric age group. METHODS: Patients were included if they had positive blood cultures for typical microorganisms and had a TTE and TEE within 14 days of each other. The patients had to meet the Duke criteria for a positive diagnosis of bacterial endocarditis. The TTE and TEE data were analyzed using the McNemar test for the significance of change. RESULTS: Twenty-one patients fulfilled the criteria, at a median age of 9.5 years. Congenital heart disease was present in 13 patients; 4 patients were previously healthy and 4 patients had other medical problems. Nine patients had surgical confirmation of bacterial endocarditis. Fifteen patients had a positive cardiac finding, with 12 vegetations, 2 vegetations plus aortic root abscess, and 1 isolated abscess. There was excellent agreement between TTE and TEE in those cases with positive cardiac findings, with a p = 0.32, kappa 0.89. Using positive TEE cardiac findings as the gold standard, the sensitivity for TTE was 86% for all 15 events and 93% for the detection of a vegetation. CONCLUSIONS: In pediatric cases, TTE has a high degree of sensitivity for the detection of supportive evidence of endocarditis, and TEE should be reserved for patients with a poor transthoracic window.

Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome.

OBJECTIVES: We sought to determine whether direct foramen ovale (FO) assessment or pulmonary venous (PV) flow patterns in fetal hypoplastic left heart syndrome (HLHS) correlate with clinical markers of postnatal left atrial (LA) hypertension severity associated with restrictive FO. BACKGROUND: Restrictive FO places a newborn with HLHS at high risk of mortality and morbidity. METHODS: We reviewed the prenatal and postnatal echocardiograms and outcomes of 45 fetuses with variants of HLHS diagnosed since May 1999 to determine whether direct FO assessment or PV flow patterns correlate with clinical LA hypertension after birth. RESULTS: Direct FO assessment in utero showed a poor correlation with postnatal FO size, Pao(2), base excess, and the need for atrial septoplasty (p > 0.05). In 40 fetuses with available PV spectra, three PV flow patterns were observed: 1). continuous forward flow with a small a-wave reversal (velocity time integral [VTI] for reverse/forward flow [VTIR/VTIF ratio <0.18]); 2). continuous forward flow with increased a-wave reversal (VTIR/VTIF ratio >or=0.18); and 3). brief to-and-fro flow. Among 19 live-borns, the postnatal FO diameter was smaller in patients with type B than in those with type A flow (1.6 +/- 1.6 mm and 4.5 +/- 2.1 mm, respectively; p = 0.0015), and all patients with type C flow had an intact atrial septum. All three patients with type C flow were critically ill at birth, requiring emergent atrial septoplasty, and two died after heart transplantation, whereas patients with type A or B flow were clinically stable, with only one postoperative death. CONCLUSIONS: Prenatal PV flow patterns in HLHS identify the fetus at risk of severe LA hypertension at birth.

Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block.

OBJECTIVES: This study was designed to document the association of endocardial fibroelastosis (EFE) and maternal autoantibodies. BACKGROUND: Neonatal lupus erythematosus is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies, leading to complete atrioventricular block (CAVB). In some cases, CAVB is associated with EFE. Isolated EFE may be independently related to maternal anti-Ro and anti-La antibodies. METHODS: We identified three cases (one fetus and two infants, all female) of isolated EFE in infants born to autoantibody-positive mothers in the absence of CAVB. Demographics, echocardiograms, and pathology were reviewed. Immunohistochemical analyses for immunoglobulin (Ig)G, IgM, IgA, T-cell, B-cell, and terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) (test for cell apoptosis) staining were performed on multiple sections of the heart in each case and compared with negative controls. RESULTS: Two cases died and one received a cardiac transplant. All three cases had histologically confirmed EFE. All cases demonstrated significant diffuse IgG infiltration. To a lesser extent, myocardial tissue was also positive for IgM, CD43, and Granzyme B. None of the specimens were TUNEL positive. CONCLUSIONS: These are the first reported cases of isolated EFE associated with maternal anti-Ro and anti-La antibodies in the absence of CAVB. The diffuse deposition of IgG and the presence of a T-cell infiltrate throughout the myocardium suggest that the transplacental passage of maternal autoantibodies induces an immune reaction within the myocardium, leading to isolated EFE. Autoantibody-mediated EFE may be an etiologic factor in cases of fetal and neonatal "idiopathic" dilated cardiomyopathy.

Three-dimensional echocardiography improves the understanding of left atrioventricular valve morphology and function in atrioventricular septal defects undergoing patch augmentation.

OBJECTIVES: We sought to address the role of 3-dimensional echocardiography in the evaluation of the left atrioventricular valve in children with an atrioventricular septal defect who underwent patch augmentation of their valve for either regurgitation or left ventricular outflow tract obstruction. METHODS: Five children whose ages ranged between 4.5 and 9.2 years and who underwent patch augmentation of their left atrioventricular valve had a preoperative and postoperative transesophageal echocardiogram with 3-dimensional reconstruction to evaluate the left atrioventricular valve. The indication for operation was left atrioventricular valve regurgitation in 3 patients and left ventricular outflow tract obstruction in 2 patients. Three were rerepairs, and 2 were primary repairs. Both 3-dimensional morphology and color Doppler data were obtained. Two- and 3-dimensional findings were correlated with surgical observations through the use of direct inspection and video images obtained with a head-mounted super-VHS camera. RESULTS: In each case there was precise correlation between the 3-dimensional and surgical findings as to the cause of leaflet failure in those with regurgitation. The site that would require leaflet augmentation could be determined by means of 3-dimensional echocardiography. Three-dimensional echocardiography provided more specific detail as to the morphology and function of the left atrioventricular valve than did its 2-dimensional counterpart. CONCLUSIONS: Three-dimensional echocardiography provides detailed information about the status of the left atrioventricular valve in the atrioventricular septal defect and can aid in the planning of either primary or secondary repair.

Doppler tissue echocardiography: can transesophageal echocardiography be used to acquire functional data?

BACKGROUND: To date there is no direct comparison of transesophageal and transthoracic Doppler tissue data in patients with cardiac malposition. We compared data acquired from both methods in an animal model. METHOD: We studied 10 15-kg Yorkshire pigs during incremental atrial pacing to modify myocardial responses. Peak isovolumic velocity (IVV) and isovolumic acceleration (IVA) during isovolumic contraction, ejection (S wave), and diastolic velocities (early passive and active filling, E and A wave, respectively) were measured. RESULTS: Bland-Altman plots showed comparable values for IVA, IVV, and E velocity using both methods. Measurements of A velocities were significantly different. S wave velocity measurements were significantly different in animals with cardiac malposition but not in those with levocardia. Malposition did not significantly effect measurements of IVA or IVV by either technique. CONCLUSION: Assessment of systolic function using the indices IVA and IVV by tranesophageal and transthoracic echocardiography are comparable. The tranesophageal assessment of velocities associated with greater degrees of myocardial shortening or lengthening cannot be directly compared with measurements derived from a transthoracic approach in patients with cardiac malposition.

Double-outlet right ventricle: An echocardiographic approach.

Using echocardiography it is possible to obtain detailed information about the basic morphology, subtypes, and associated abnormalities in double outlet right ventricle. Armed with this information, surgical management can be planned in the majority of cases utilizing echocardiography alone. This article addresses the basic echocardiographic approach, provides an in-depth understanding of the various subtypes, as well as outlining the additional features that often complicate complete repair. Copyright 2000 by W.B. Saunders Company

Abnormal Ultrasonic Tissue Characterization of the Myocardium in Children Receiving Doxorubicin for Cancer Therapy.

Ultrasonic tissue characterization by integrated backscatter is a sensitive tool to detect myocardial changes related to specific diseases. Cardiotoxicity related to doxorubicin use is well known and remains a major concern. To determine if ultrasonic tissue characterization of the myocardium is abnormal in patients receiving doxorubicin, we studied the myocardium of pediatric patients receiving doxorubicin by a real-time integrated backscatter (IB) imaging system. Three values of IB parameters were averaged from the left ventricular posterior wall at the level of the tip of the mitral valve. In addition to standard echo parameters, we obtained the following IB parameters: peak, nadir, cyclic variation (CV), end-diastole, heart-rate corrected delay of nadir (Delay(c)), and the ratio of CV over end-diastole. IB parameters were normalized as Z scores from multiple linear regression equations including echo wall thickness and functional indices from a normal control group of 72 children. We evaluated 27 patients at a median age of 11.6 years (1.6 years to 20.3 years) and median time of 1.7 m (2 days to 7.2 years) after a mean cumulative dose of doxorubicin of 188 +/- 120 mg/m(2) for treatment of neoplasm. Mean (+/-SD) Z scores for IB variables were as follows: zPeak 0.15 +/- 1.07, P = 0.47; zNadir 0.41 +/- 1.16, P = 0.08; zCV -0.49 +/- 0.95, P = 0.01; zEnd-diastole 0.17 +/- 0.94, P = 0.38; zDelay(c) 0.33 +/- 0.80, P = 0.06, and zCV/Peak -0.59 +/- 1.06, P = 0.009. This study shows that ultrasonic IB of the myocardium of children receiving doxorubicin is abnormal and is independent of the cumulative dosage of doxorubicin or the amount of time since the last dose.