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Background: The long-term prognosis of hypoplastic left heart syndrome is limited by progressive right ventricular dysfunction. The aim of this study was to determine the trends in single right ventricular systolic function between staged palliative surgeries using speckle-tracking and conventional echocardiography., Methods: There were 76 patients with functionally single right ventricles at the (1) pre-Norwood (n = 26), (2) pre-bidirectional cavopulmonary anastomosis (BCPA; n = 19), (3) pre-Fontan (n = 16), and (4) post-Fontan (n = 15) stages, compared with 30 controls of similar ages. Speckle-tracking-derived longitudinal and circumferential strain and strain rate, postsystolic strain index, and mechanical dyssynchrony index were compared with conventional measures of ventricular function. Differences between stages were analyzed using analysis of variance (P < .05)., Results: Strain rate was highest at the pre-Norwood stage and decreased at the other stages (longitudinal P < .0001, circumferential P = .0002), as opposed to controls, in whom strain rate was maintained. Longitudinal strain was significantly decreased at the pre-BCPA stage compared with the pre-Norwood stage (P = .004), but circumferential strain was maintained, resulting in a corresponding decrease in the ratio of longitudinal to circumferential strain, which failed to resemble that of controls. Longitudinal (P = .003) and circumferential (P = .002) postsystolic strain indices were greatest at the pre-BCPA stage., Conclusions: A decline in contractility occurred at the pre-BCPA stage. Although there was evidence of adaptation of the single right ventricle, this failed to resemble the normal left ventricle and may be insufficient to handle the chronic volume load or insult from previous surgery. These findings suggest an intrinsic inability of the single right ventricular myocardium to fully adapt to chronic systemic pressures., (Copyright © 2014 American Society of Echocardiography. All rights reserved.)

Background: Single ventricle (SV) exercise performance is impaired and limited by reduced ventricular preload reserve. The atrium modulates ventricular filling, and enhancement of atrial compliance can increase cardiac performance. We aimed to study atrial mechanics in SV hearts across staged surgical palliation compared with healthy children by using novel speckle-tracking echocardiography techniques., Methods: A cross-sectional study of 81 patients with SV (1 day to 6.5 years) at 4 stages of surgical palliation (presurgery, 22; prebidirectional cavopulmonary anastomosis, 23; pre-Fontan, 22; post-Fontan, 14). The dominant atrium was assessed with speckle-tracking echocardiography for active (εact), conduit (εcon), and reservoir (εres) strain; strain rate (SR); and εact/εres ratio before each stage of surgical palliation. Findings were compared with the left atrium of 51 healthy children (1 day to 5.5 years)., Results: Single ventricle atrial size was increased (P < .01), and atrial εres was decreased (P < .01) compared with healthy controls. SV atrial εcon (P < .01) and SRcon (P < .0001) was decreased, increased εact persisted (P < .05), and εact/εres was increased (P < .001) between surgical stages. Although the expected maturational trend of increasing εcon, decreasing εact, and εact/εres occurred in SV, they lagged behind healthy maturational changes (P < .0001)., Conclusion: Single ventricle atrium is dilated, has deceased compliance, decreased early diastolic emptying, and increased reliance on active atrial contraction for ventricular filling. This deviates from normal early childhood maturational changes and appears to parallel those of an atrium facing early ventricular diastolic dysfunction., (Crown Copyright © 2013. Published by Mosby, Inc. All rights reserved.)

Background: Differences in single right ventricle (SRV) and single left ventricles (SLV) function are poorly described, although myocardial dysfunction is an important risk factor for morbidity and mortality. The aims of this study were to compare function between patients with SRVs and those with SLVs using newer echocardiographic techniques and to determine differences across staged palliation., Methods: In this cross-sectional study comparing 30 patients with SRVs and 30 with SLVs of similar ages (2.5 ± 1.7 vs 2.6 ± 1.6 years), patients were matched for surgical stage (20 pre-bidirectional cavopulmonary anastomosis, 20 pre-Fontan, and 20 post-Fontan patients). Circumferential and longitudinal strain, strain rate (SR), early diastolic SR, postsystolic strain index, and myocardial dyssynchrony index were measured. Comparisons between SRV and SLV parameters were made as a whole group and by subanalysis at each surgical stage., Results: Patients with SRVs had reduced systolic SRs (circumferential: -1.0%/sec vs -1.2%/sec, P = .01; longitudinal: -1.1%/sec vs -1.3%/sec, P = .002), reduced early diastolic SRs (circumferential: 1.4%/sec vs 1.9%/sec, P = .03; longitudinal: 1.6%/sec vs 2.2%/sec, P = .001), and increased circumferential postsystolic strain indexes (8% vs 0%, P < .0001). Subanalysis at each surgical stage showed that the greatest disparity in systolic parameters occurred before bidirectional cavopulmonary anastomosis (longitudinal SR, P = .009; postsystolic strain index, P = .005) and that parity was regained after the Fontan procedure, while traditional diastolic parameters (E velocity, P = .004; E/E' ratio, P = .0003) were reduced in patients with SRVs after the Fontan procedure., Conclusions: The SRV has reduced contractility and diastolic function compared with the SLV. Ventricular systolic performance in patients with SRVs was poorest before bidirectional cavopulmonary anastomosis, while differences in diastolic function were more prominent after Fontan completion., (Crown Copyright © 2012. Published by Mosby, Inc. All rights reserved.)

Background: Left ventricular (LV) torsion and untwisting are important components of LV performance, but there is little information on the effect of age, particularly in younger populations., Methods: LV rotation and LV rotation rate, torsion, recoiling, and untwisting were measured in normal subjects (n=111) aged 3 to 40 years (mean age, 19.3 years) using speckle-tracking imaging., Results: LV torsion increased with age because of the augmentation of apical LV rotation, but this disappeared when normalized by LV length. Although peak LV torsion and apical LV rotation increased with age, the normalized peak torsion rate decreased. As well, the peak untwisting rate decreased with age and was enhanced when normalized by LV length. Younger hearts demonstrated greater untwisting and recoiling of the apex during isovolumic relaxation and early diastole. The time difference between apical and basal events decreased with advancing age., Conclusion: The heart maintains a constant LV torsion and LV rotation profile when normalized by length and cardiac cycle. Younger hearts tend to twist, untwist, and deform faster., (Copyright (c) 2010 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

Background: Non-compaction of the left ventricular myocardium is an important cause of cardiomyopathy. There is no clear consensus about its diagnostic criteria or the diagnostic test of choice. MRI is increasingly used in the pediatric cardiac field because of its superior and objective image quality., Objective: To compare the echocardiographic and MRI findings in four patients with recently diagnosed ventricular non-compaction., Materials and Methods: We compared the extent of myocardial involvement shown at MRI and echocardiography in four individuals, two patients with echocardiographic diagnosis of left ventricular non-compaction, and two family members of one of the patients., Results: In all patients, MRI showed wider area of involvement than echocardiography. A definite diagnosis was entertained in only two patients by echocardiography but in all by MRI. Cine imaging was diagnostic of the disease in all patients. Black-blood pool imaging with double-inversion recovery sequence also helped to visualize the abnormal areas by showing slow flow artifacts in the four- and two-chamber images., Conclusion: MRI provided better delineation of the extent of the abnormal trabeculation in patients with non-compaction of the left ventricular myocardium. It was particularly useful when the myocardial involvement was subtle, as in the asymptomatic family members.

Nonobstructive cardiomyopathies (CMs) may be familial in 20â"55% of cases. Little is known about the role of fetal echocardiography in such cases. We evaluated the cardiac function serially pre- and postnatally in cases with a family history of nonobstructive CM. The fetal and postnatal studies were performed in the echocardiogarphy laboratory at a tertiary institution. Twenty-six cases from 16 families with a family history of CM were studied. Three fetal echocardiograms were performed at or near 18, 25, and 32 weeks of gestation for complete cardiac functional assessment. Postnatally clinical evaluation, electrocardiogram, and an echocardiogram were performed within the first 3 months, with serial reevaluation for those identified with CM. The mean follow-up was 46 +/- 9 months. Abnormal cardiac function was observed in 8 cases (30%). Six had a previously affected sibling, 1 had other family members affected, and 1 had both antecedents. Four had dilated CM diagnosed prenatally of which 1 recovered, 2 died in utero, and 1 died soon after birth. The remaining 4 had normal fetal echoes and were diagnosed with CM in the first 3 months of life. Three had dilated CM with recovery, and 1 had restrictive CM requiring cardiac transplantation. This study demonstrates a high familial recurrence rate of CM. Fetal echo is useful for early diagnosis, although a normal study does not preclude ventricular dysfunction at a later stage, justifying serial prenatal and postnatal evaluation. Early identification may expedite listing for transplantation.

Objective: To review the pattern of presentation, management and outcome of fetal complete atrioventricular block (CAVB) associated with major structural congenital heart disease (CHD), when compared to isolated CAVB., Methods: Retrospective analysis of the medical records and echocardiograms of all CAVB cases, diagnosed prenatally at two tertiary care centers between the years 1990 and 2002., Results: Of a total of 59 consecutive fetal cases of CAVB, 24 (41%) had underlying major CHD, mainly left isomerism (n = 18) and congenitally corrected transposition of the great arteries (cc-TGA) (n = 3). When compared to isolated CAVB (n = 35), cases with CHD were detected earlier (21 +/- 6 vs. 26 +/- 6 weeks; P < 0.02) and-despite comparable heart rates-more often had fetal hydrops (38% vs. 9%; P < 0.02), while pregnancy continuation (66% vs. 94%; P < 0.02) or prenatal treatment (19% vs. 64%; P < 0.001) was less likely. Of 16 CHD cases with pregnancy continuation, beta-inotropic treatment of fetal bradycardia was attempted in three cases: all had left isomerism and died early postnatally. Livebirth and 1-year survival rates of CAVB with CHD were 56% and 19%, respectively, when compared to isolated CAVB with 88% and 75%, respectively (P < 0.0001). The four neonatal survivors (one left isomerism, three cc-TGA) had heart rates persistently > 60 bpm throughout gestation and 3/4 underwent a biventricular repair., Conclusions: Fetal CAVB with CHD continues to be associated with a poor outcome, in particular in the presence of left isomerism and fetal heart rates < 60 bpm., (Copyright (c) 2005 ISUOG.)

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.

Background: Cardiac catheterization is commonly performed before repair of ventricular septal defect (VSD) in infancy. No study has addressed the accuracy of echocardiography alone in defining all of the important anatomic features in this population., Methods: Consecutive infants undergoing VSD repair between 1991 and 1995 (n = 156) were reviewed. The number of additional VSDs and the presence of commonly associated lesions were recorded for each technique and compared with a reference standard consisting of a combination of surgical findings plus postoperative echocardiography and clinical findings. The associated lesions were right ventricular muscle bundles, subaortic ridge, and persistent ductus arteriosus., Results: The sensitivity and specificity of echocardiography in the detection of additional VSDs was 60% and 99% compared with 53% and 97% for cardiac catheterization and 73% and 96% for both techniques. These differences were not statistically significant. No differences were seen in the detection of commonly associated lesions., Conclusion: Routine preoperative cardiac catheterization for infants with a primary diagnosis of VSD is probably no longer justified.

In utero isolated ductal closure is uncommon and can lead to fetal hydrops and death if not recognized. Five fetuses were diagnosed prenatally by echocardiography. The reasons for referral were hydrops (2), cardiomegaly (1), polyhydramnios and choroid plexus cyst (1), and polyhydramnios and teratoma of the neck (1). Gestational age was median 32, range 18-41 weeks. Two mothers received indomethacin for polyhydramnios, with the echocardiogram performed 5 and 3 days after the last dose. Three had a negative history of drug ingestion. Fetal echocardiograms showed absent flow in the ductus arteriosus, dilated right ventricle with decreased function, and mild or moderate tricuspid and pulmonary insufficiency in all. The left ventricle was hypercontractile, with significantly increased left ventricular stroke output when compared to the right. Four fetuses were delivered by cesarean section and had an uneventful course. One fetus died shortly after birth due to airway obstruction from a large teratoma. Autopsy showed ductal constriction. Postnatal echocardiograms showed absent ductal flow and dilated right ventricle. On follow-up, survivors remained asymptomatic with cardiac size returning to normal. Premature closure of the ductus arteriosus should be considered in hydrops of unknown etiology, right ventricular dysfunction, and following indomethacin therapy. Urgent delivery results in an excellent prognosis.

This study sought to assess pregnant diabetic women for the presence of fetal cardiac anomalies and to determine whether better diabetic control was associated with a reduced risk to the fetus. Between 1988 and 1995, pregnant type I and II diabetic women routinely underwent fetal echocardiography. Hemoglobin A1c values were used as an indicator of maternal diabetic control and any relation between congenital heart disease in the fetus and maternal hemoglobin A1c levels was sought. Cardiac defects were identified in 7 of 328 pregnancies assessed, for an incidence of congenital heart disease of 2.1% (95% confidence interval: 0.6-3.6%). A review of the postnatal cardiac database did not reveal any undetected major malformations. The mean hemoglobin A1c level was 7.6% +/- 2.0% obtained at a mean gestational age of 12 +/- 7 weeks. Hemoglobin A1c levels of mothers carrying a fetus with congenital heart disease did not significantly differ from those with a normal fetus: 8.1% +/- 3.4% versus 7.6% +/- 1.9% (p = 0.48). Mothers with an affected fetus demonstrated a wide range of HbA1c levels (4.1 to 13.7%). Thus, the incidence of significant fetal cardiac abnormalities is low and not significantly related to maternal diabetic control.

Objectives: The purpose of this study was to determine the role of high resolution two-dimensional echocardiographic imaging and color flow Doppler study in assessing the pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect (VSD)., Background: Although echocardiography is a well established technique for assessing central pulmonary arteries in pulmonary atresia VSD and for determining the presence or absence of a patent arterial duct, few data are available on its role in patients whose source of blood supply is from collateral vessels., Methods: Forty-two patients aged a few hours to 19 months (mean 29 days) were prospectively assessed by high resolution echocardiography, including color flow Doppler study, during a 4-year period ending in 1994, before any intervention other than intravenous administration of prostaglandins. Angiographic confirmation was available in 29 patients, including 18 (95%) of 19 with aortopulmonary collateral channels., Results: A patent arterial duct was correctly identified as the sole source of pulmonary blood supply in 23 patients, whereas aortopulmonary collateral channels were detected in 19, with one of these having a small patent arterial duct and collateral channels. The patent arterial duct originated from the undersurface of the aorta in 16 (67%) of 24 patients and from the base of the brachiocephalic trunk in 7 (33%) of 24. All patients with a patent ductus as the sole source of pulmonary blood supply had confluent pulmonary arteries. Nonconfluent pulmonary arteries were present in six patients, with all but one having aortopulmonary collateral channels as the sole source of pulmonary flow. Aortopulmonary collateral channels were direct in 17 (89%) of 19 patients, whereas in 2 (11%) of 19, both direct and indirect collateral channels were present. Color flow Doppler study was accurate in determining the presence or absence, the side and the origin of the collateral channels in all patients, with the correct number being determined in 12 (67%) of 18. "Wash-in" to the hilar pulmonary arteries (retrograde color flow) was seen in 12 (92%) of 13 patients with collateral channels and confluent pulmonary arteries. Failure to identify a tiny central pulmonary artery occurred in one patient., Conclusions: High resolution imaging and color flow Doppler study provide good appreciation of the source of pulmonary blood supply in neonates and young infants with pulmonary atresia VSD.

Background: Transcatheter device occlusion of atrial septal defects is an attractive approach, but its efficacy and place in patient management remain to be determined., Methods and Results: To evaluate the medium-term results of atrial septal defect device occlusion and factors influencing residual shunting, transesophageal and transthoracic echocardiograms of 49 patients were reviewed. Transesophageal echocardiograms on 48 patients immediately following surgical closure revealed residual shunting in 2% compared with 91% after device occlusion; this proportion decreased to 53% after a mean follow-up of 10 months. The actuarial analysis suggests a progressive resolution of shunting with time. One patient had residual shunting by transesophageal echocardiography immediately after surgical closure compared with 29 after a mean follow-up of 10 months after device occlusion. Residual shunting was not influenced by (1) dimension, location, or position with relation to the device as assessed by transesophageal echocardiography; (2) location of the defect; or (3) device size relative to the stretched dimension of the defect. In 15 patients, a poor correlation existed between transesophageal and transthoracic echocardiographic findings. Variability in serial transthoracic echocardiographic findings was observed in 14. Right ventricular dimension, heart size, and presence of a murmur at follow-up did not correlate with the presence or size of residual shunting after device occlusion., Conclusions: These results suggest that ongoing spontaneous resolution of residual shunting occurs after device insertion. Factors related to the defect or device could not predict eventual resolution of residual shunting. Transthoracic echocardiography in the follow-up of these patients may not be reliable in determining presence of residual shunting.

Objectives: This study was designed to assess the temporal relation between early coronary artery abnormalities and left ventricular function in Kawasaki disease., Background: Although late segmental wall motion abnormalities may be seen in patients with Kawasaki disease who have coronary artery stenosis, the impact of early coronary artery abnormalities is unclear., Methods: Regional left ventricular wall motion was assessed by two-dimensional echocardiography in 18 patients with Kawasaki disease and echocardiographic evidence of coronary artery enlargement at 3 weeks and 3 months and at either 6 or 12 months after the onset of fever. Four patients had a persistent left coronary artery aneurysm, four had regression of their aneurysm, two had persistent left coronary artery ectasia and eight had regression of ectasia. Left ventricular wall motion was assessed by measuring regional area change in parasternal and apical views. After planimetry of an end-systolic and an end-diastolic frame, the ventricle was divided into eight equal segments and the percent area change was calculated. A floating system correcting for translation and rotation was applied. The measurements in the patient group were compared with values previously obtained in 55 normal age-matched infants and children., Results: A transient regional wall motion abnormality 3 and 6 months after the onset of fever was discovered in the inferolateral wall of one patient with a persistent left coronary artery aneurysm. One patient with regression of coronary artery ectasia had a persistent wall motion abnormality in the anterolateral left ventricular wall. There was no correlation between the extent of coronary artery enlargement and the presence or absence of wall motion abnormalities., Conclusions: These early changes are most likely secondary to associated myocarditis rather than coronary artery abnormalities.

Because comprehensive normal data and the effect of observer variability for echocardiographic evaluation of regional left ventricular wall motion are not available in children or newborns, left ventricular wall motion was assessed by measuring regional area change. The study group comprised 55 infants and children with a normal heart: 15 neonates (greater than 1 week to less than 1 month old), 10 infants (greater than 1 month to less than 1 year old) and 30 children, 10 each in the age group greater than 1 year to less than 5 years, greater than 5 to less than or equal to 10 years and greater than 10 years. A combination of parasternal, apical and subcostal two-dimensional echocardiographic views was applied. After planimetry of an end-systolic and end-diastolic frame, the left ventricle was divided into eight equal segments and the percent area change calculated. Both a fixed reference and a floating system correcting for translation and rotation were applied. Intraobserver variability for percent area change measurements was 2.8 +/- 0.9% and 3.8 +/- 1% for observers 1 and 2, respectively. The mean interobserver difference of regional percent area change was 4.7 +/- 1.8%. Normal values for the eight anatomic segments were established in each echocardiographic imaging plane. The overall results were independent of the type of reference system utilized. The subcostal views yielded different results from their parasternal counterparts, probably because of differing imaging planes. These normal values establish a data base in the pediatric age range that can be used to detect abnormal segments in children at risk for developing regional left ventricular wall motion abnormalities.

Regional left ventricular wall motion was assessed by two-dimensional echocardiography in 21 patients with complete transposition of the great arteries at a mean of 2.2 years (range 0.3 to 7) after an arterial switch operation. Fourteen patients had undergone a two-stage and seven patients a primary repair. Twenty patients were found to have echocardiographic images adequate for wall motion analysis. The results of these studies were correlated with thallium-201 myocardial perfusion scans. Left ventricular wall motion was assessed by measuring regional area change in parasternal and apical views. After planimetry of an end-systolic and an end-diastolic frame, the ventricle was divided into eight equal segments and the percent area change was calculated. Both a fixed reference and a floating system correcting for translation and rotation were applied. The measurements in the patient group were compared with normal age-matched values previously obtained in 55 normal infants and children. Wall motion abnormalities, found in seven patients, were located at the apex in three, at the inferior septum and anterolateral wall in one and the inferior, anterolateral and lateral walls in one patient each. All had a myocardial perfusion defect in a corresponding anatomic location. One patient with a small reversible perfusion defect at the basilar septum had normal regional wall motion. The sensitivity of detecting impairment of myocardial perfusion was 0.95. Wall motion abnormalities were found only in patients who had undergone a two-stage repair. Echocardiographic wall motion analysis can be used as a screening method to identify patients with suspected myocardial ischemia after the arterial switch operation.

Azygos continuation of the inferior vena cava has importance for both the invasive diagnosis of congenital heart disease by catheterization and for surgical treatment. Cross-sectional echocardiography was used to examine 1,000 patients (1 day to 16 years, mean 3.3 years) who also had angiographic or surgical confirmation. Twenty-eight patients (3%) had azygos continuation (left 13, right 14, bilateral 1) and, in 26 patients, the hepatic portion of the inferior vena cava was absent. Azygos continuation was prospectively detected in all and was directly visualized in subcostal scans as a venous structure posterior to the aorta coursing behind the heart and not entering the inferior aspect of either atrium in 26/28 (93%). Azygos connection to the ipsilateral superior vena cava or atrium was correctly predicted in all. The inferior vena cava was visualized in all patients without azygos continuation, except one neonate with omphalocele. We conclude that cross-sectional echocardiography can accurately detect azygos continuation of the inferior vena cava and predict its side and connection.

Congenital arteriovenous fistulas presenting in the newborn period pose difficult diagnostic problems and simulate structural heart disease. Angiocardiography, when performed, demonstrates enlarged brachiocephalic vessels and rapid cerebral venous return. The value of echocardiographic imaging and measurement of the aortic arch and brachiocephalic vessels, and evaluation of the Doppler flow profile in these vessels as a means of making a rapid diagnosis of cerebral or thoracic arteriovenous fistula, was therefore assessed in 10 infants with these diagnoses seen over a 4 year period (1983 to 1987). Twenty-nine infants (median age 6 weeks) undergoing two-dimensional echocardiography but with no significant lesions were prospectively selected as controls. Nine of the 10 patients had congestive heart failure at presentation (mean age 2 days). A cranial bruit was heard in three and arteriovenous fistula was suspected in five patients. Aortic arch segments and brachiocephalic vessel dimensions expressed as ratios of the abdominal aorta showed significantly larger values in patients for the ascending aorta (p = 0.01), innominate artery (p less than 0.001), right and left subclavian arteries (p less than 0.001) and left common carotid artery (p less than 0.05). The thoracic descending aorta was, however, significantly smaller in patients (p less than 0.002). Retrograde diastolic Doppler flow in the descending aorta proximal to the ductus arteriosus and anterograde diastolic flow with a mean spectral flow-time integral 27% of systolic were present in patients only, whereas Doppler diastolic flow in brachiocephalic vessels, present in 5 of 29 control infants, was less than 15% of systolic flow and not accompanied by dilation of these vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

At present there is no reliable method of recognising atrial isomerism by two dimensional echocardiography. We therefore used two dimensional echocardiography to examine 158 patients including 25 with atrial isomerism and four with situs inversus. Particular attention was paid to the short and long axis subcostal scans of the abdomen. Using the position of the inferior vena cava and the aorta with respect to the spine it was possible to separate those with situs solitus from the others. Two false positives for abnormal situs had exomphalos. In situs solitus the aorta lay to the left of the spine and the inferior vena cava lay to the right. One patient with situs solitus and azygos continuation of the inferior vena cava also had inferior vena cava to right atrial connection. In the four patients with situs inversus the mirror image of the normal pattern was present. In nine patients with right isomerism the inferior vena cava and aorta ran together on one or other side of the spine. The inferior vena cava, anterior to the aorta at the level of the diaphragm, received at least the right hepatic veins (normal or partial anomalous hepatic venous connection). Of the 16 patients with left isomerism, 14 had azygos continuation of the inferior vena cava which was visualised posterior to the aorta in all but two. All patients with left isomerism had total anomalous hepatic venous connection to one or both atria via one or two separate veins. Two dimensional echocardiography therefore provides the means of detecting abnormal atrial situs and of diagnosing right or left isomerism in the great majority of patients, if not all.

The feasibility of using balloon dilatation to relieve stenosis caused by dysplasia of the pulmonary valve was assessed in seven patients (five female, mean age two years) with angiographically confirmed dysplasia who were identified among 38 patients with pulmonary valve stenosis selected for balloon dilatation over a two year period. The clinical features in three patients were consistent with Noonan's syndrome. In all patients the gradient across the valve was assessed by cross sectional echocardiography and Doppler echocardiography before cardiac catheterisation. Balloon dilatation was performed by conventional techniques. In one patient, who had balloon dilatation in the operating room before surgical valvectomy, the diameter of the valve orifice increased from 3 mm to 10 mm. Inspection showed a tear along the anterior commissure. The mean (SD) pressure gradients between the right ventricle and pulmonary artery before and immediately after dilatation in five patients were not significantly different (58 (28) and 47 (12) mm Hg) respectively. There was no overall significant change in the degree of stenosis when four of these patients were examined by Doppler echocardiography six months after operation (44 (17) mm Hg), although one patient (case 5) did show a significant reduction in gradient. This patient had angiographic and echocardiographic features of dysplasia and commissural fusion. Several echographic features were common to all patients and distinguished them from cases of typical pulmonary valve stenosis. These were: pronounced thickening of leaflets; leaflet immobility in diastole and systole; no dilatation of the sinuses of Valsalva in diastole, and supra-annular narrowing. These poor results of balloon dilatation suggest that commissural fusion is not an important mechanism for causing stenosis in the dysplastic pulmonary valve. When dysplasia of the pulmonary valve is identified clinically and echocardiographically, balloon dilatation is unlikely to improve haemodynamic function; it should be attempted if commissural fusion is present.

To determine the sensitivity and specificity of cross-sectional echocardiography in diagnosing anomalous systemic venous return we used the technique in 800 consecutive children with congenital heart disease and whom the diagnosis was ultimately confirmed by angiography. Cross-sectional echocardiography was performed without prior knowledge of the diagnosis in all but 11 patients, who were recalled because of a known abnormality of atrial situs. The sensitivity of cross-sectional echocardiographic detection of various structures was as follows: right superior vena cava 792/792 (100%); left superior vena cava 46/48 (96%); bilateral superior vena cava 38/40 (95%); bridging innominate vein with bilateral superior vena cava 13/18 (72%); connection of superior caval segment to heart (coronary sinus or either atrium) (100%); absence of suprarenal inferior vena cava 23/23 (100%); azygos continuation of the inferior vena cava 31/33 (91%); downstream connection of azygos continuation, once seen, 21/21 (100%); partial anomalous hepatic venous connection (one hepatic vein not connected to the inferior vena cava) 1/1 (100%); total anomalous hepatic venous connection (invariably associated with left isomerism) 23/23 (100%). The specificity of each above diagnoses was 100% except in one infant with exomphalos in whom absence of the suprarenal inferior vena cava was incorrectly diagnosed. Thus cross-sectional echocardiography is an extremely specific and highly sensitive method of recognizing anomalous systemic venous return. It is therefore of great value of planning both cardiac catheterisation and cannulation for open heart surgery.

The role of combined two-dimensional and pulsed Doppler echocardiography in the postoperative assessment of patients with total anomalous pulmonary venous connection was evaluated. Twenty-two cases with a median age of 9.5 weeks at the initial examination were evaluated. Serial ultrasound examinations were performed throughout the study period. The ultrasound results were compared with chest radiographs obtained during the same period. Of the 22 patients, 16 had normal pulmonary venous flow profiles characterized by low-velocity laminar flow. Of this group 12 had persistent radiographic postoperative pulmonary edema that cleared in all by 4 months. Six patients with pulmonary venous obstruction were identified, the diagnosis being confirmed at catheterization or autopsy. The venous flow pattern in this group was uniformly high velocity and turbulent. It was possible to localize the site of obstruction in each case. Although pulmonary edema was present in each patient, the chest radiograph did not provide reliable information as to the exact site of obstruction. Combined two-dimensional and Doppler echocardiography is a useful adjunct in the postoperative evaluation of patients with total anomalous pulmonary venous connection.

Since 1983, 36 patients with the combination of right ventricular muscle bundles and a perimembranous ventricular septal defect have been studied in our institution to address the incidence of on associated subaortic abnormality. Of that total 32 (88%) had echocardiographic evidence of such an abnormality (29 had a typical subarotic ridge protruding from the crest of the interventricular septum and the remaining 3 had an echodense area in the same location). Surgical confirmation of the presence or absence of a subaortic abnormality was available in 26. There was correlation between the surgical and echocardiographic findings in all patients. A resting Doppler gradient of greater than or equal to 10 mm Hg was present in only 10. During the study period, 6 patients had Doppler evidence of progression of their gradient. The incidence of subaortic abnormalities in right ventricular muscle bundles and ventricular septal defects appears to be far greater than previously suspected. The exact significance of this finding in the absence of a pressure gradient is still unclear.

To establish an integrated non-invasive method for diagnosing coarctation, cross-sectional echocardiographic appearances of 48 neonates and infants with coarctation were combined with clinical information on the peripheral pulses. Measurements of the ascending aorta, aortic arch, and isthmus were made and compared with those from controls matched for weight and age. Confirmation of the coarctation was available in all cases. Angiocardiographic measurements were performed in 15 patients from either the group with coarctation or the controls. After the aortic arch had been analysed segment by segment 40 patients were found to have preductal coarctation, five juxtaductal coarctation, and three postductal coarctation. In one of the patients in the latter group the obstruction was situated in the abdominal aorta. Specific echocardiographic features were present in each subgroup. Echocardiographic measurements were about two thirds of those obtained by angiocardiography. By combining information on the peripheral pulses, isthmic size, and the presence of a discrete shelf in the aorta it was retrospectively possible to predict correctly the presence of coarctation in 45 out of 48 cases. Since the beginning of this study 29 patients have undergone surgery without prior invasive investigation. A combination of clinical assessment and cross-sectional echocardiographic features allows a reliable diagnosis of coarctation to be made in most cases.

Auscultation of a fetal bradyarrhythmia led to the prenatal echocardiographic detection of an intracardiac tumor. After birth the diagnosis of tuberous sclerosis was established. Therapeutic implications for current and future pregnancies, when a fetal cardiac tumor is found, are discussed.

Pulsed Doppler evaluation of pulmonary venous flow was performed in three groups of patients who had undergone either a Mustard or Senning procedure. Group I consisted of 43 patients in sinus rhythm who had undergone the Mustard procedure 9 months or more before the Doppler examination and 16 who were evaluated immediately after surgery. Group II consisted of 12 patients in sinus rhythm who had undergone the Senning procedure 9 months or more before the Doppler evaluation. Group III consisted of eight patients with mid baffle obstruction of the pulmonary venous atrium and seven with isolated stenosis of the pulmonary vein of which all but one had previously had associated mid baffle obstruction. In those without obstruction, the pulmonary venous flow pattern mirrored the left atrial pressure trace, with peak forward flow occurring during the x and y descent. Obstruction produced a specific high-velocity turbulent pattern, whether at the mid baffle or pulmonary venous level. This technique provides reliable noninvasive information about pulmonary venous flow after the Mustard or Senning procedure.

Pulmonary venous flow was evaluated by pulsed Doppler echocardiography in 38 patients with total anomalous pulmonary venous connection. Twenty-nine of these 38 had no associated intracardiac anomaly (Group I), and 9 had complex intracardiac anatomy associated with low pulmonary blood flow (Group II). In Group I the drainage was infracardiac in nine, supracardiac in seven, intracardiac in eight and mixed in five. In both groups, in those with venous obstruction the flow in the individual pulmonary veins and ascending or descending vein was nonphasic, varying only with respiration. Flow in the absence of obstruction was phasic, varying with the cardiac cycle. Distal to a site of obstruction the flow was nonlaminar and of high velocity irrespective of the amount of pulmonary blood flow. The pulsed Doppler technique provides important physiologic information in the patient with total anomalous pulmonary venous connection before surgical intervention.

Twelve patients with univentricular heart and common atrioventricular valve were identified by two-dimensional echocardiography. Seven had an ostium primum atrial septal defect and five a common atrium. The common atrioventricular valve had the appearance of a free floating anterior leaflet flanked by two lateral leaflets. The position of the rudimentary chamber in relation to the atrioventricular valve was posterior in six and anterior in two; no chamber was identified in four. Two-dimensional echocardiography provides a reliable method for the identification of common atrioventricular valve in univentricular heart.

Twenty consecutive patients with ventricular septal defect and aortic valve prolapse were evaluated by cross-sectional echocardiography. Angiographic confirmation was available in all and surgical confirmation was found in 17. In 19, the right coronary cusp was involved and appeared to plug the defect in the precordial long- and short-axis cut. The cusp was deformed and appeared to pivot from the crest of the interventricular septum. In all 19 angiography demonstrated prolapse of the right cusp. Noncoronary cusp prolapse was observed in two by cross-sectional echocardiography and in six by angiocardiography. The ventricular septal defect was perimembranous in 14 and doubly committed subarterial in six by echocardiography. Angiographically, the ventricular septal defect was felt to be perimembranous in 15 and doubly committed in five. Aortic regurgitation was detected by Doppler interrogation in seven, all of whom underwent plication of the right coronary cusp. Angiographic evidence of regurgitation was noted in 11, but four were mild and possibly related to catheter position. Five patients had associated muscular right ventricular outflow tract obstruction and three had a subaortic ridge. Combined cross-sectional and pulsed Doppler echocardiography provide a reliable assessment of right coronary cusp prolapse associated with a ventricular septal defect. Noncoronary cusp prolapse appears more difficult to detect. This technique should help optimize the management of patients by providing a means of early detection prior to the development of aortic regurgitation.

Sixteen patients with a straddling tricuspid and two with a straddling mitral valve were identified by two dimensional echocardiography. In all but one the atrioventricular valves appeared at the same level, indicating absence of the ventriculoatrial septum. A straddling valve was diagnosed by identifying subvalvular apparatus from one atrioventricular valve in both chambers, independent of whether they were ventricles or rudimentary chambers. Further confirmation was obtained during real time study where the valve leaflets appeared to fly through the ventricular septal defect. Overriding of the valve annulus was greater than 50% in 12 and less than 50% in five, with one other patient having none detectable. The relation of the central fibrous body to the tip of the interventricular septum was reliable in assessing overriding of greater than 50%, but where it was less than 50% other views were necessary to detect its presence. The diagnosis of straddling with or without overriding of an atrioventricular valve can be reliably made by two dimensional echocardiography, and carries important implications relating to the type of surgical intervention possible, and in those with a straddling tricuspid valve, the position of the atrioventricular node.

Extraparenchymal pulmonary vein flow profiles were assessed by pulsed Doppler echocardiography in three groups of patients. Group I comprised 41 normal patients. Group II comprised 16 patients, 10 of whom had nonpulsatile pulmonary artery flow (5 with a right atrium to pulmonary artery Fontan procedure and 5 with a Glenn shunt). Six patients with pulsatile pulmonary artery flow had simultaneous Doppler and left atrial pressure measurements during cardiac catheterization. Group III comprised one patient with pulmonary vein obstruction, six with a large left to right shunt at ventricular level and two with pulmonary vascular disease. In Group I, biphasic forward pulmonary vein flow occurring during ventricular systole and diastole was observed in 26 subjects, 15 others had triphasic flow. In those with triphasic flow, the ventricular systolic component was divided into early and late. Reversed flow in the pulmonary veins during atrial systole was seen in 36 of the 41 subjects. The flow pattern in Group II was identical irrespective of the presence of pulsatile or nonpulsatile flow. The two periods of ventricular systolic flow occurred during the a to c and c to x descent, with the ventricular diastolic flow occurring during the y descent. Variations in waveform were mirrored in the left atrial pressure. Neither increased nor decreased pulmonary artery flow substantially altered the pattern. Pulmonary vein obstruction produced a distinctive pattern of high velocity turbulent flow. This technique demonstrates that extraparenchymal pulmonary vein flow is dependent on left atrial pressure events. It has major potential applications in patients who are prone to develop pulmonary vein obstruction.

Ninety-six patients with an atrioventricular septal defect were assessed by two dimensional echocardiography. Forty-eight patients were judged as having two discrete valve orifices. In this group, 37 had an intact ventricular septum and 11 a ventricular septal defect. Two patterns of attachment of the valve leaflets to the interventricular septum were noted. Forty-eight patients had a common valve orifice. In 29 patients there was absence of tethering of the anterior bridging leaflet to the interventricular septum. A further 19 had varying degrees of tethering of the anterior bridging leaflet. A ventricular septal defect was identified under the anterior bridging leaflet in all cases. In 58 cases where the posterior bridging leaflet was identified, a ventricular septal defect was visualised in eight and missed in two. Seventy-eight patients had an ostium primum defect, 12 a common atrium, and a further six an intact interatrial septum. Two dimensional echocardiography provides in depth morphological information about the type of atrioventricular defect and can correctly identify the presence of a ventricular septal defect beneath the anterior or posterior bridging leaflet, unless it exists between short crowded chordae.

Patients with anomalous origin of the left coronary artery from the pulmonary trunk usually have a large right coronary artery. This study examines the diagnostic value of measuring the diameter of the right coronary artery by echocardiography in distinguishing between this lesion and other causes of dilated cardiomyopathy. The diameter of the right coronary artery and the right coronary artery/aorta ratio were measured in the parasternal short axis view in 40 controls, 11 patients with dilated cardiomyopathy, and 10 with anomalous origin of the left coronary artery from the pulmonary trunk. In the controls, the diameter of the right coronary artery increased with age, but the right coronary artery/aorta ratio remained constant. In the control group the 95% upper limits of prediction for right coronary artery diameter were 1.6 mm for one month of age, 1.8 mm for three months, 2.0 mm for one year, 2.2 mm for two years, 2.4 mm for three years, 2.6 mm for four years, 2.7 mm for six years, 3.0 mm for eight years, and 3.2 mm for 10 years; and for right coronary/aorta ratios the limits were 0.17 for one month to one year, 0.18 for one to six years, 0.19 for six to 10 years, and 0.20 for more than 10 years. All patients with dilated cardiomyopathy had normal right coronary artery diameters and right coronary artery/aorta ratios (0.10-0.13). Those patients with anomalous origin of the left coronary artery from the pulmonary trunk had larger than normal right coronary artery diameter and a significant increase in the right coronary artery/aorta ratio (0.21-0.29). The presence of an anomalous left coronary artery was likely if the diameter of the right coronary artery or the right coronary artery/aorta ratio was larger than the normal 95% limits of prediction.

Fifteen preterm infants who weighed 0.7 to 2.0 kg and had clinical evidence of a patent ductus arteriosus (PDA) were studied by combined 2-dimensional and Doppler echocardiography before and after the administration of indomethacin. In 10 patients the PDA was widely patent at the time of the study and in 5 the lumen was narrow. In this latter group, the PDA was narrow at the pulmonary artery end in 2 patients, in the middle in 2 patients and at the aortic end and the middle in 1 patient. After the administration of intravenous indomethacin, the PDA closed completely in 12 patients and constricted in 3. The patterns of closure could be documented in those in whom serial studies were performed. In 3 patients, closure occurred after a single dose of indomethacin, in 3 after 2 doses and in the rest after a full course of 3 doses. Doppler interrogation at the aortic and pulmonary artery end of the PDA demonstrated the shunting patterns and provided a reliable assessment of patency after the ductal lumen was outside the range of lateral resolution following constriction. In no case did the PDA reopen after the course of indomethacin. This combined approach is a reliable method of assessing a PDA before and after a course of indomethacin. It should provide the means to answer many of the questions regarding the effect of various manipulations on the PDA in the preterm infant.