Real-time 3-dimensional echocardiography provides new insight into mechanisms of tricuspid valve regurgitation in patients with hypoplastic left heart syndrome.

BACKGROUND: Tricuspid regurgitation in hypoplastic left heart syndrome has an impact on outcome, but its mechanisms remain unclear. METHODS AND RESULTS: Real-time 3-dimensional echocardiography was performed in 35 patients with hypoplastic left heart syndrome (age, 1 month to 10 years; 10 after first-stage Norwood, 12 after superior cavopulmonary shunt, 13 after Fontan). From the 3-dimensional data set, we marked the annulus in systole and diastole. At mid systole, we marked the location of the papillary muscle tip and point of chordal attachment to the leaflet. We traced the surfaces of the tricuspid valve leaflets and measured the volume of leaflet prolapse, tethering, annular and septal leaflet areas, and papillary muscle position. Seventeen patients had moderate tricuspid regurgitation (prolapse, 7; tethered leaflets, 7) and 18 mild (prolapse, 0; tethered leaflets, 7). Multiple linear regression analysis revealed that moderate tricuspid regurgitation is associated with leaflet tethering and prolapse; that in hypoplastic left heart syndrome with tethered leaflets, the papillary muscle is displaced laterally and the tricuspid annulus is more planar; and that enlargement of the annulus at mid systole, small septal leaflet area, and age affect the degree of prolapse. CONCLUSIONS: In hypoplastic left heart syndrome, moderate tricuspid regurgitation may be associated with increasing age, geometrical changes of the annulus, leaflet prolapse, lateral papillary muscle displacement, and subsequent leaflet tethering, as well as a smaller septal leaflet.

A case of restrictive cardiomyopathy presenting in fetal life with an isolated pericardial effusion.

We describe the evolution during pregnancy of a case of restrictive cardiomyopathy which first presented at 22 weeks' gestation with a large pericardial effusion. Measurements of cardiac function were normal and remained near normal until late in the third trimester, when pulsed and tissue Doppler data suggested impairment in ventricular relaxation. This disease progressed in postnatal life to symptomatic restrictive cardiomyopathy by 2 years of age necessitating cardiac transplant. To our knowledge, this is the first time this unusual association has been reported.

Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: comparison of intrauterine, postnatal and postmortem diagnoses.

OBJECTIVES: The clinical outcome of prenatally diagnosed congenital heart defects (CHD) continues to be affected significantly by associated extracardiac and chromosomal abnormalities. We sought to: determine the frequency and type of major extracardiac abnormalities (with impact on quality of life) and chromosomal abnormalities associated with fetal CHD; and compare the extracardiac abnormalities detected prenatally to the postnatal and autopsy findings in affected fetuses, to find the incidence of extracardiac abnormalities missed on prenatal ultrasound. METHODS: We reviewed the computerized database of the Division of Cardiology of the Hospital for Sick Children in Toronto to identify all cases of major CHD detected prenatally from 1990 to 2002. Medical records, fetal echocardiograms and ultrasound, cytogenetic and autopsy reports were reviewed. The types of CHD detected were grouped into categories and the frequencies of major extracardiac and chromosomal abnormalities in these categories were noted. Prenatal ultrasound findings were compared with those at autopsy or postnatal examination. RESULTS: Of 491 fetuses with major structural CHD, complete data were obtained for 382. Of these, there were 141 (36.9%) with major extracardiac abnormalities at autopsy or postnatal exam, of which 46 had chromosomal abnormalities and 95 did not. In the absence of chromosomal abnormalities, the organ systems most affected were urogenital (12.2%) and gastrointestinal (11.6%). CHDs with the highest incidence of extracardiac abnormalities (>25%) included: heterotaxy, single left ventricle and tricuspid atresia, hypoplastic left heart syndrome and tetralogy of Fallot. Ninety-four of 334 (28.1%) fetuses tested had chromosomal abnormalities. The most common chromosomal abnormalities were trisomies 21 (43.6%), 18 (19.1%) and 13 (9.6%), monosomy X (7.4%) and 22q11.2 deletion (7.4%). Of 289 extracardiac abnormalities from the complete series, 134 (46.4%) were not identified prenatally. Of the missed extracardiac abnormalities, 65 were considered not detectable at prenatal ultrasound, so 23.9% (69/289) of detectable extracardiac abnormalities were missed prenatally. CONCLUSIONS: Major extracardiac and chromosomal abnormalities are common in fetuses with major fetal CHD. Many important associated extracardiac abnormalities may be missed prenatally, which should be taken into consideration in the prenatal counseling for fetal CHD.

Prospective multicenter study of pregnancy outcomes in women with heart disease.

BACKGROUND: The maternal and neonatal risks associated with pregnancy in women with heart disease receiving comprehensive prenatal care have not been well defined. METHODS AND RESULTS: We prospectively enrolled 562 consecutive pregnant women with heart disease and determined the outcomes of 599 pregnancies not ending in miscarriage. Pulmonary edema, arrhythmia, stroke, or cardiac death complicated 13% of pregnancies. Prior cardiac events or arrhythmia, poor functional class or cyanosis, left heart obstruction, and left ventricular systolic dysfunction independently predicted maternal cardiac complications; the cardiac event rate can be predicted using a risk index incorporating these predictors. Neonatal complications (20% of pregnancies) were associated with poor functional class or cyanosis, left heart obstruction, anticoagulation, smoking, and multiple gestations. CONCLUSIONS: Pregnancy in women with heart disease is associated with significant cardiac and neonatal complications, despite state-of-the-art obstetric and cardiac care. Maternal cardiac risk can be predicted with the use of a risk index.

Pulsed Doppler echocardiography in the preoperative evaluation of total anomalous pulmonary venous connection.

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.

Serial analysis of regional left ventricular wall motion by two-dimensional echocardiography in patients with coronary artery enlargement after Kawasaki disease.

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.

Pulsed Doppler echocardiographic assessment of extraparenchymal pulmonary vein flow.

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.

Echocardiographic assessment of pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect.

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.

Echocardiographic analysis of regional left ventricular wall motion in normal children and neonates.

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.

Echocardiographic analysis of regional left ventricular wall motion in children after the arterial switch operation for complete transposition of the great arteries.

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.

Echocardiographic evaluation of partial anomalous pulmonary venous drainage.

OBJECTIVES: This study was undertaken to determine the accuracy of routine echocardiography in the detection of partial anomalous pulmonary venous drainage. BACKGROUND: Although there are occasional case reports of the echocardiographic appearance of partial anomalous pulmonary venous drainage, no large series have addressed the accuracy of this technique in a large cohort of patients. METHODS: Between January 1983 and December 1993, 50 patients with partial anomalous pulmonary venous drainage (with or without an associated atrial septal defect as the only other significant intracardiac defect) were identified from the data base at the Hospital For Sick Children, Toronto. Routine echocardiographic reports were reviewed, and the results were compared with surgical or catheterization findings. Risk factors related to diagnostic errors were sought using a Fisher exact test, chi-square analysis, t test and Kruskal-Wallis analysis of variance. RESULTS: Confirmation of the diagnosis was available in 45 patients whose data were subsequently used for risk factor analysis. The median age at echocardiography was 4.1 years (range 1 month to 18 years). Right-sided drainage was present in 43 patients (86%), with left-sided drainage in 7 (14%). Thirteen patients had an intact atrial septum, 7 a patent foramen ovale and 30 a secundum atrial septal defect. Right ventricular dilation was observed in 46 patients. Two had normal dimensions (two not assessed). The diagnosis was missed by echocardiography in 15 (33%) of the 45 patients with a confirmed diagnosis. Year of study and use of color flow mapping were the only significant variables related to detection rate (7% missed diagnosis with vs. 62% without the use of color flow, p < 0.0005). The median year of missed diagnosis was 1985 versus 1990 (p < 0.002). Transesophageal echocardiography accurately defined the site of drainage in all three patients in whom it was utilized. CONCLUSIONS: Two-dimensional echocardiography in conjunction with color flow mapping is a valuable tool for the diagnosis of partial anomalous pulmonary venous drainage.

Outcomes of left atrial isomerism over a 28-year period at a single institution.

OBJECTIVES: We determined long-term outcomes in a large cohort with left atrial isomerism (LAI). BACKGROUND: Left atrial isomerism is associated with a complex spectrum of cardiac and noncardiac anomalies that may impact on outcomes. METHODS: The records of all patients with LAI, born between 1970 and 1998, and treated at one center were reviewed. Kaplan-Meier survival was estimated, and independent factors associated with time-related death were identified. RESULTS: There were 163 patients (63% women), and extracardiac anomalies were noted in 36%, including biliary atresia in 10%. Cardiac defects included interrupted inferior caval vein in 92%, anomalous pulmonary veins in 56%, atrioventricular septal defect in 49%, pulmonary atresia or stenosis in 28% and aortic coarctation in 16%, with congenital atrioventricular block in 7%. Of 22 patients with a normal heart, 18% died of extracardiac anomalies. Of 71 patients with hearts suitable for biventricular repair, 62 (87%) had surgery, with survival of 80% at one year, 71% at five years, 66% at 10 years and 63% after 15 years. Of 70 patients with unbalanced cardiac defects suitable for single-ventricle palliation, 47 (67%) had surgery, with survival of 73% at one year, 61% at five years, 53% at 10 years and 48% at 15 years (p < 0.001). Independent factors associated with time-related death included congenital atrioventricular block, aortic coarctation, single ventricle, biliary atresia and other gastrointestinal malformations. CONCLUSIONS: Both cardiac and noncardiac anomalies contribute to a high mortality with LAI. Cardiac transplantation may need to be a considered a primary option for selected high-risk patients.

Mortality in potential arterial switch candidates with transposition of the great arteries.

OBJECTIVES: We reviewed the factors contributing to or causing death before surgery in neonates with d-transposition of the great arteries (TGA) despite anatomy suitable for the arterial switch operation (ASO) to develop strategies to minimize preoperative attrition. BACKGROUND: Currently the ASO for neonates with TGA carries a low operative mortality. However, there is a paucity of information regarding the patients who die before the ASO. Strategies to ensure survival to operation are of importance to improve overall outcome. METHODS: We reviewed all neonates with TGA and patent forearm ovale (PFO) < or = 2 mm, a birthweight <2 kg, or who died before surgery, between 1988 and 1996. RESULTS: We identified 12 out of 295 neonates with TGA (4.1%) with anatomy suitable for the ASO who died prior to surgery. All had TGA/intact ventricular septum (IVS) and presented with a severely restrictive PFO. In 11 of 12 cases the cause of death was attributed to the sequelae of profound hypoxemia from inadequate mixing. Contributing factors were prematurity, 41.7%; severe respiratory distress syndrome, 25%; and persistent pulmonary hypertension of the newborn (PPHN), 16.7%. All patients received prostaglandin E1 (PGE1) infusion. Urgent balloon atrial sepstostomy (BAS) was performed in 66.7% with improved oxygenation. No cases were diagnosed prenatally. In contrast, all patients with a PFO < or = 2 mm who survived to ASO had a significantly better response to PGE1 infusion (p=0.03) than nonsurvivors. The ASO was accomplished without mortality in four of nine with a weight <2 kg. CONCLUSIONS: Of those neonates admitted with TGA, 4.1% died before surgery. Eleven of 12 (3.7%) died due to consequences of inadequate interatrial mixing despite PGE1 infusion. Earlier diagnosis and BAS are critically important in determining survival. Early ASO may improve survival in patients weighing <2 kg. Prenatal diagnosis with delivery in a high-risk obstetrical unit with facilities for immediate BAS and supportive therapy for pulmonary hypertension and ventricular failure may be necessary to salvage this group of patients.

Postoperative follow-up of fibromuscular subaortic stenosis.

OBJECTIVES: This study attempted to determine whether early subaortic resection at lower levels of obstruction reduces the rate of recurrence of subaortic stenosis or reduces secondary damage to the aortic valve, or both. BACKGROUND: Fibromuscular subaortic stenosis is a progressive condition, and at present it is unclear whether early operation reduces the recurrence rate along with decreasing the incidence of aortic insufficiency. METHODS: Thirty-seven patients with fibromuscular subaortic stenosis and no other significant cardiac abnormality who underwent open subaortic resection were evaluated. The preoperative, early and late postoperative catheterization or echocardiographic findings as well as the operative reports were reviewed. The median age at operation was 6.4 years (range 1.1 to 17.3). The entire group has been followed up postoperatively for a median of 5.2 years (range 1.1 to 11). Mean systolic gradients across the left ventricular outflow tract were used for the purpose of this study. RESULTS: There was a significant correlation between the preoperative mean systolic gradient and the incidence of preoperative aortic regurgitation and late postoperative aortic valve thickening as well as the incidence and degree of late postoperative aortic regurgitation. Late postoperative gradient and degree of aortic regurgitation correlated significantly with the follow-up interval. Aortic regurgitation was progressive in some patients despite subaortic resection. A preoperative mean gradient > 30 mm Hg provided a reasonable cutoff for the likelihood postoperatively of needing a reoperation, having a postoperative shelf, a thickened aortic valve, moderate aortic regurgitation or a gradient of > 10 mm Hg. CONCLUSIONS: Our results suggest that although early subaortic resection may not reduce the rate of recurrence of fixed subaortic stenosis, it is likely to reduce acquired damage to the aortic valve.

Doppler echocardiographic measurement of pulmonary artery pressure from ductal Doppler velocities in the newborn.

The ductal flow velocities in 37 newborns (group 1: persistent pulmonary hypertension [n = 16], transient tachypnea [n = 3], other [n = 2]; group 2: respiratory distress syndrome [n = 16]) were prospectively evaluated by Doppler ultrasound for the purpose of deriving systolic pulmonary artery pressures. Maximal tricuspid regurgitant Doppler velocity in 21 of these patients was used to validate the pulmonary artery pressures derived from ductal flow velocities. There was a significant linear correlation between tricuspid regurgitant Doppler velocity and pulmonary artery systolic pressure derived from ductal Doppler velocities in patients with unidirectional (pure left to right or pure right to left) ductal shunting (p less than 0.001, r = 0.95, SEE 8) and in those with bidirectional shunting (p less than 0.001, r = 0.95, SEE 4.5). Systolic pulmonary artery pressure in group 1 (67 +/- 13 mm Hg) was significantly higher than that in group 2 (39 +/- 10 mm Hg) (p less than 0.001). In those with bidirectional shunting, duration of right to left shunting less than 60% of systole was found when pulmonary artery pressure was systemic or less, whereas duration greater than or equal to 60% was associated with suprasystemic pulmonary artery pressures. Serial changes in pulmonary artery systolic pressure, reflected by changes in ductal Doppler velocities, correlated with clinical status in persistent pulmonary hypertension of the newborn. Persistently suprasystemic pulmonary artery pressure was associated with death in five group 1 patients. It is concluded that ductal Doppler velocities can be reliably utilized to monitor the course of pulmonary artery systolic pressures in newborns.

Echocardiographic evaluation of the spectrum of cardiac anomalies associated with trisomy 13 and trisomy 18.

To investigate the role that cardiac anomalies play in the early death frequently seen in the trisomy 13 and the trisomy 18 syndromes, two-dimensional and Doppler echocardiograms from 31 newborn infants with cytogenetic confirmation of these syndromes seen at one institution over a 4.5 year period (1983 to 1988) were reviewed. The mean age at echocardiography was 1.5 days, and the median age at death was 14 days. Significant cyanosis was present in 58%. Cardiac anomalies that would be considered lethal within the neonatal period were present in only 19% of patients. The most common lesions were atrial septal defect (81%), ventricular septal defect (61%) and patent ductus arteriosus (85%). Most ventricular septal defects and patent ductus arteriosus were large. Valvular dysplasia of one or more valves, graded as mild in most cases, was found in 68%, but was not associated with Doppler evidence of significant regurgitation or stenosis in any subject. Of the four valves, the pulmonary valve, followed by the tricuspid valve, was the most commonly dysplastic. Doppler evidence suggestive of elevated pulmonary artery pressure (low velocity bidirectional flow across the ventricular septal defect and patent ductus arteriosus), although expected, was accompanied by greater than normal mean right ventricular cavity and free wall dimensions in these patients. Thus, although the cardiac anomalies most frequently encountered in trisomy 13 and trisomy 18 are nonlethal, the combined findings of frequent cyanosis and increased right ventricular dimensions suggest that other factors such as pulmonary hypertension, perhaps related to maldevelopment of the pulmonary vasculature, may contribute to early death in some of these infants.

Echocardiographic and Doppler evaluation of the aortic arch and brachiocephalic vessels in cerebral and systemic arteriovenous fistulas.

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)

Management and outcomes of right atrial isomerism: a 26-year experience.

OBJECTIVES: We sought to determine, in a large series of patients with right atrial isomerism, factors associated with mortality. BACKGROUND: Right atrial isomerism is associated with complex congenital heart disease and high morbidity and mortality. METHOD: All data from patients diagnosed with right atrial isomerism between January 1970 and March 1996 were reviewed. RESULTS: A total of 91 consecutive patients (54 male) were identified. Most patients (89%) presented within the first month of life, 62% at birth. Cardiac abnormalities included common atrioventricular (AV) valve (81%), ventricular hypoplasia or single ventricle (73%), abnormal ventriculoarterial connections (96%), pulmonary outflow tract obstruction (84%), anomalous pulmonary venous drainage (87%) and pulmonary vein obstruction (30%). The overall mortality rate was 69%. No interventions were planned or performed in 24%, 95% of whom died. The mortality rate for patients requiring their first cardiovascular operation in the neonatal period was 75% versus 51% for those with later first operations (p < 0.05). The surgical mortality rate for patients undergoing pulmonary vein repair was 95%. Overall survival estimates were 71% at 1 month, 49% at 1 year and 35% at 5 years. Independent risk factors for decreased time to death included the absence of pulmonary outflow obstruction (relative risk [RR] 2.23, p < 0.03), presence of major AV valve anomaly (RR 5.23, p < 0.03) and obstructed pulmonary veins (RR 5.43, p < 0.0001). CONCLUSIONS: Right atrial isomerism continues to have an associated high mortality despite surgical innovations. Management of pulmonary vein obstruction remains a serious problem and is associated with high mortality.

Echocardiographic assessment of obstructive lesions in atrioventricular septal defects.

OBJECTIVES: We sought to determine the accuracy of transthoracic echocardiography (TTE) in identifying risk factors in patients with an atrioventricular septal defect (AVSD). BACKGROUND: Atrioventricular septal defect is a common lesion, and many decisions about it are based on echocardiography alone. The identification of associated left-sided inflow and outflow obstructive lesions is important, as they are responsible for mortality and morbidity. METHODS: Between 1983 to 1998, 549 patients with AVSD underwent repair. The TTE findings were correlated with surgery, angiocardiography, autopsy or postoperative TTE. Papillary muscle measurements were made in those with either a left ventricular outflow tract (LVOT) or left ventricular inflow abnormality and compared with those measurements from control subjects. Measurements of the LVOT were made in patients with an identified LVOT abnormality. RESULTS: There were 63 missed lesions, decreasing over time. Double-orifice left atrioventricular valve (DOLAVV) and nonobstructive chordae in the LVOT were more often missed. Reoperation was performed to address a missed lesion in 2 of 68 patients. Two of 55 patients died of reasons related to a missed lesion. In 67% of patients, DOLAVV was missed. Abnormal papillary muscle angles were seen with either a LVOT abnormality or DOLAVV. High insertion of the anterolateral papillary muscle was a risk factor for death or residual LVOT obstruction. Abnormal LVOT measurements were found in patients with tunnel obstruction and those with an acquired subaortic ridge. CONCLUSIONS: Transthoracic echocardiography provides accurate preoperative information on AVSD.

Acquired right ventricular outflow tract obstruction in the recipient twin in twin-twin transfusion syndrome.

OBJECTIVES: The goal of this study was to determine the prevalence and evolution of acquired right ventricular outflow tract obstruction (RVOTO) in the recipient twin in twin-twin transfusion syndrome (TTTS). BACKGROUND: Twin-twin transfusion syndrome complicates 4% to 26% of diamniotic monochorionic twin gestations and is associated with high fetal morbidity and mortality. Cardiac dysfunction and biventricular hypertrophy may develop in the recipient twin with the potential for RVOTO. METHODS: This was a retrospective review of a two-center experience of TTTS to describe the prevalence and evolution of acquired RVOTO in the recipient twin. Right ventricular outflow tract obstruction was diagnosed or excluded by fetal or postnatal echocardiography or clinical assessment. RESULTS: Of 73 twin pregnancies with TTTS identified between 1994 to 1998, a total of seven (9.6%) were complicated by RVOTO in the recipient twin: two subvalvar/muscular, four valvar and one combined. Of 44 pregnancies with fetal echo, six had in utero RVOTO with antegrade flow diagnosed at gestational ages ranging from 19 to 27 weeks. In utero progression occurred in four cases over a period of four to eight weeks, with the development of RVOT atresia by delivery. Postnatal progression of RVOTO occurred in two cases, one of which required pulmonary balloon valvuloplasty at age two years. Postnatal regression of subvalvar RVOTO occurred in two cases in early infancy. Death related directly or indirectly to the RVOTO occurred in all four patients who developed complete RVOT obliteration. CONCLUSIONS: Right ventricular outflow tract obstruction may occur in the recipient twin of at least 9% of pregnancies complicated by TTTS. Right ventricular outflow tract obstruction progression is common in utero and may worsen neonatal outcome.