Fetal and neonatal atrial arrhythmias: an association with maternal diabetes and neonatal macrosomia.

OBJECTIVE: To determine if the incidence of maternal diabetes mellitus or neonatal macrosomia is more frequent in fetuses and neonates with atrial arrhythmias than the general population. METHODS: Fetuses and neonates <30 days of age with atrial flutter or ectopic atrial tachycardia and structurally normal hearts were identified retrospectively through the cardiology databases. Electrocardiograms, echocardiograms, and medical records of mothers and infants were reviewed. RESULTS: Thirty-one patients (15 fetuses, 12 diagnosed in-utero) were identified. Infants with atrial flutter or ectopic atrial tachycardia were more likely to be macrosomic or to be born to diabetic mothers than the general population. Two had left atrial dimension z-scores above +2, and two had interventricular thickness z-scores above +2. Eighteen of 19 had abnormal mitral E/A ratios, suggesting left ventricular diastolic dysfunction. CONCLUSIONS: Fetuses and neonates with atrial flutter or ectopic atrial tachycardia were more likely to be macrosomic or be born to diabetic mothers than the general population. Postnatal echocardiography suggests that there may be abnormal diastolic left ventricular filling in some babies with these arrhythmias. Independent of ventricular hypertrophy, we speculate that isolated, non-recurrent fetal or neonatal atrial flutter, or ectopic atrial tachycardia may be caused by cardiac diastolic dysfunction and atrial stretch in utero.

Postoperative complications following the Fontan procedure: the role of aprotinin.

OBJECTIVE: To determine how the anti-inflammatory properties of aprotinin impact on postoperative complications in children undergoing the Fontan procedure. METHODS: We included all patients between 14 months and 18 years (n=56) undergoing a Fontan operation at our institution between January 2005 and June 2009. The study group (n=29) included patients from January 2005 through December 2007 all of whom received aprotinin. The control group (n=27) included all patients from January 2008 through June 2009 who did not receive aprotinin. We reviewed all medical records and collected preoperative, intraoperative and postoperative data. Duration and volume of chest tube drainage were the primary outcome measures. RESULTS: Of the 20% of patients who had postoperative arrhythmias, multivariate logistic regression analysis demonstrated only aprotinin was associated with significantly decreased postoperative arrhythmias (P=0.01). Renal function and fenestration or Fontan thrombosis did not differ significantly; there was no statistically significant difference in volume or duration of chest tube drainage. Median duration of chest tube drainage was 7 days in the aprotinin group and 8 days for patients who did not receive aprotinin (P=0.36). CONCLUSION: The anti-inflammatory properties of aprotinin may be protective against postoperative arrhythmias. Aprotinin does not confer increased risks of prolonged chest tube drainage, renal dysfunction or thrombosis in patients undergoing the Fontan procedure.

Standardized delivery room management for neonates with a prenatal diagnosis of congenital heart disease: A model for improving interdisciplinary delivery room care.

Precise characterization of cardiac anatomy and physiology through fetal echocardiography can predict early postnatal clinical course. Some neonates with prenatally defined critical congenital heart disease have anticipated precipitous compromise during perinatal transition for which specialized, diagnosis-specific delivery room care can be arranged to expeditiously stabilize cardiopulmonary hemodynamics. In this article, we describe our institutional approach to the delivery room care of neonates with prenatally diagnosed congenital heart disease, emphasizing our diagnosis-specific care pathways for newborns with critical disease.

Heart sounds at home: feasibility of an ambulatory fetal heart rhythm surveillance program for anti-SSA-positive pregnancies.

OBJECTIVE: Fetuses exposed to anti-SSA (Sjögren's) antibodies are at risk of developing irreversible complete atrioventricular block (CAVB), resulting in death or permanent cardiac pacing. Anti-inflammatory treatment during the transition period from normal heart rhythm (fetal heart rhythm (FHR)) to CAVB (emergent CAVB) can restore sinus rhythm, but detection of emergent CAVB is challenging, because it can develop in ⩽24 h. We tested the feasibility of a new technique that relies on home FHR monitoring by the mother, to surveil for emergent CAVB. STUDY DESIGN: We recruited anti-SSA-positive mothers at 16 to 18 weeks gestation (baseline) from 8 centers and instructed them to monitor FHR two times a day until 26 weeks, using a Doppler device at home. FHR was also surveilled by weekly or every other week fetal echo. If FHR was irregular, the mother underwent additional fetal echo. We compared maternal stress/anxiety before and after monitoring. Postnatally, infants underwent a 12-lead electrocardiogram. RESULTS: Among 133 recruited, 125 (94%) enrolled. Among those enrolled, 96% completed the study. Reasons for withdrawal (n=5) were as follows: termination of pregnancy, monitoring too time consuming or moved away. During home monitoring, 9 (7.5%) mothers detected irregular FHR diagnosed by fetal echo as normal (false positive, n=2) or benign atrial arrhythmia (n=7). No CAVB was undetected or developed after monitoring. Questionnaire analysis indicated mothers felt comforted by the experience and would monitor again in future pregnancies. CONCLUSION: These data suggest ambulatory FHR surveillance of anti-SSA-positive pregnancies is feasible, has a low false positive rate and is empowering to mothers.

Hemodynamic consequences of a restrictive ductus arteriosus and foramen ovale in fetal transposition of the great arteries.

D-transposition of the great arteries (D-TGA) is the most commonly diagnosed cyanotic congenital heart disease presenting in the neonatal period. The survival after an arterial switch operation, with freedom from adverse cardiovascular events, has been reported to be as high as 93% at 25 years. However, despite excellent surgical outcomes, there continues to be significant preoperative morbidity and potential mortality due to compromise in the delivery room from foramen ovale closure requiring urgent balloon atrial septostomy for stabilization in the first minutes of life. The prenatal diagnosis of D-TGA using fetal echocardiography has aided in the perinatal management and delivery planning of these infants, lowering preoperative morbidity and mortality and preventing delivery room compromise. Fetuses with D-TGA have more highly oxygenated blood supplying the pulmonary arteries and ductus arteriosus which likely results in ductal constriction and increased pulmonary blood flow. This may be the cause of foramen ovale restriction or closure in-utero, which then increases the risk for postnatal compromise at delivery. Theories regarding the cause of the abnormal pulmonary vasculature that may be seen in D-TGA, including aorto-pulmonary collateral formation, have been proposed but to our knowledge, observation of the ultrasound findings throughout mid and late gestation describing the progression of the abnormal fetal physiology have not been previously described. We present a case of D-TGA in which serial assessment using fetal echocardiography enabled observation of the in-utero progression of disease, predicting postnatal compromise and facilitating the planning of life-saving specialized delivery room care and intervention.

Fetal cardiology: changing the definition of critical heart disease in the newborn.

Infants born with congenital heart disease (CHD) may require emergent treatment in the newborn period. These infants are likely to benefit the most from a prenatal diagnosis, which allows for optimal perinatal planning. Several cardiac centers have created guidelines for the management of these high-risk patients with CHD. This paper will review and compare several prenatal CHD classification systems with a particular focus on the most critical forms of CHD in the fetus and newborn. A contemporary definition of critical CHD is one which requires urgent intervention in the first 24 h of life to prevent death. Such cardiac interventions may be not only life saving for the infant but also decrease subsequent morbidity. Critical CHD cases may require delivery at specialized centers that can provide perinatal, obstetric, cardiology and cardiothoracic surgery care. Fetuses diagnosed in mid-gestation require detailed fetal diagnostics and serial monitoring during the prenatal period, in order to assess for ongoing changes and identify progression to a more severe cardiac status. Critical CHD may progress in utero and there is still much to be learned about how to best predict those who will require urgent neonatal interventions. Despite improved therapeutic capabilities, newborns with critical CHD continue to have significant morbidity and mortality due to compromise that begins in the delivery room. Fetal echocardiography is the best way to predict the need for specialized care at birth to improve outcome. Once the diagnosis is made of critical CHD, delivery at the proper time and in appropriate institution with specific care protocols should be initiated. More work needs to be done to better delineate the risk factors for progression of critical CHD and to determine which newborns will require specialized care. The most frequently described forms of critical CHD requiring immediate intervention include hypoplastic left heart syndrome with intact or severely restricted atrial septum, obstructed total anomalous pulmonary venous return and transposition of the great arteries with restrictive atrial septum.

Brain Injury in Neonates with Complex Congenital Heart Disease: What Is the Predictive Value of MRI in the Fetal Period?

BACKGROUND AND PURPOSE: Brain injury in neonates with congenital heart disease is an important predictor of adverse neurodevelopmental outcome. Impaired brain development in congenital heart disease may have a prenatal origin, but the sensitivity and specificity of fetal brain MR imaging for predicting neonatal brain lesions are currently unknown. We sought to determine the value of conventional fetal MR imaging for predicting abnormal findings on neonatal preoperative MR imaging in neonates with complex congenital heart disease. MATERIALS AND METHODS: MR imaging studies were performed in 103 fetuses with confirmed congenital heart disease (mean gestational age, 31.57 ± 3.86 weeks) and were repeated postnatally before cardiac surgery (mean age, 6.8 ± 12.2 days). Each MR imaging study was read by a pediatric neuroradiologist. RESULTS: Brain abnormalities were detected in 17/103 (16%) fetuses by fetal MR imaging and in 33/103 (32%) neonates by neonatal MR imaging. Only 9/33 studies with abnormal neonatal findings were preceded by abnormal findings on fetal MR imaging. The sensitivity and specificity of conventional fetal brain MR imaging for predicting neonatal brain abnormalities were 27% and 89%, respectively. CONCLUSIONS: Brain abnormalities detected by in utero MR imaging in fetuses with congenital heart disease are associated with higher risk of postnatal preoperative brain injury. However, a substantial proportion of anomalies on postnatal MR imaging were not present on fetal MR imaging; this result is likely due to the limitations of conventional fetal MR imaging and the emergence of new lesions that occurred after the fetal studies. Postnatal brain MR imaging studies are needed to confirm the presence of injury before open heart surgery.

Early changes in ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery.

OBJECTIVES: This study investigated the phenomenon of, and the relation between, alterations in ventricular geometry after acute surgical volume unloading of the ventricle and the development of subaortic stenosis in patients with a single ventricle and ventricular septal defect-dependent systemic flow. BACKGROUND: Subaortic outflow obstruction has been observed to occur in patients with a single left ventricle after placement of a pulmonary artery band. The timing and etiology of this phenomenon are not well defined. METHODS: The preoperative and postoperative echocardiograms of 18 patients 14.9 +/- 22.8 months old (mean +/- SD) with a diagnosis of single left ventricle who underwent pulmonary artery banding or cavopulmonary connection were reviewed. Postoperative studies were performed a mean of 7.0 +/- 6.5 days after operation. The ventricular septal defect diameter was measured in two orthogonal views and the area calculated using the formula for an ellipse. Interventricular septal and posterior wall thickness and left ventricular diameter and length were also measured. RESULTS: Mean ventricular septal defect area indexed to body surface area diminished by 36 +/- 23% (3.1 +/- 2.7 to 2.0 +/- 1.8 cm2/m2, p < 0.01). Mean interventricular septal and posterior wall thickness increased significantly, and left ventricular diameter and length decreased significantly. A greater diminution in ventricular septal defect area was noted after cavopulmonary connection (41 +/- 19%, p < 0.01) than after pulmonary artery banding (25 +/- 28%, p = 0.22). CONCLUSIONS: In the single left ventricle, diminution in ventricular septal defect size occurs early and is related to an acute alteration in ventricular geometry that accompanies the decrease in ventricular volume. Ventricular septal defect diminution was greater after volume unloading of the ventricle after cavopulmonary connection than after pulmonary artery banding.

Congenital aortic regurgitation: natural history and management.

OBJECTIVES AND BACKGROUND: Congenital aortic regurgitation is rare as an isolated lesion. We describe seven children with no physical features of the Marfan syndrome in the patients or their families and no other cardiac lesions who had congenital valvular aortic regurgitation. METHODS: From 1954 to the present, seven children with auscultatory and physiologic characteristics of aortic regurgitation were evaluated for a total of 108 patient-years. We report on their natural history, clinical and laboratory findings, management and outcome. RESULTS: In five of the seven children congenital aortic regurgitation was diagnosed in infancy. In four, progressive severity of the regurgitation led to valve replacement at age 3, 10, 15 and 20 years, respectively, and to resection of an aneurysm of the ascending aorta in the 10-year old patient. Two patients had cystic medial necrosis on aortic biopsy. One of these patients died after reoperation for dissecting aneurysm of the thoracic aorta at 22 years of age; the other died after dissection and rupture of the ascending aorta at age 25 years. After obstructing pannus developed, the 3-year old patient underwent replacement of the St. Jude valve at age 10 years. The other three patients were asymptomatic at last follow-up at age 8, 10 and 20 years, respectively. CONCLUSIONS: Supportive management is recommended until it becomes necessary to intervene surgically when regurgitation becomes severe. The need for surgical treatment is indicated by the appearance of a diastolic thrill, left ventricular strain on the electrocardiogram or other evidence of left ventricular dysfunction on the echocardiogram or exercise stress testing by treadmill or radionuclide cineangiocardiography. Close follow-up of these patients is important to detect progression of aortic regurgitation, especially in the presence of cystic medial necrosis.

Effect of persistent left superior vena cava on hemodynamic calculations.

When comparing means, there is no difference between right and left SVC saturations, and the difference does not affect cardiac output calculations. Because of the wide variability between right and left SVC saturations and their calculated cardiac output within patients, along with the higher correlation of left versus right SVC saturation with pulmonary artery saturation, the Fick-derived cardiac index calculation should be interpreted with caution when a left SVC is present. A prospective study with near-simultaneous sampling in all vessels should be undertaken.

Comparison of patterns of pulmonary venous blood flow in the functional single ventricle heart after operative aortopulmonary shunt versus superior cavopulmonary shunt.

In this study we investigated the patterns of pulmonary venous flow in children with functional single ventricles to obtain a better understanding of the determinants of transpulmonary blood flow. Sixty-eight patients with functional single ventricles and aortopulmonary shunt (n = 34, group I), or superior cavopulmonary connection (n = 34, group II) underwent transesophageal Doppler echocardiographic assessment of flow in the left upper pulmonary vein before undergoing the next stage of surgery. Twelve patients from group II also underwent simultaneous evaluation of superior vena caval flow. Biphasic forward pulmonary venous flow was noted in 62 patients in sinus rhythm (S wave in systole, D wave in diastole); in 6 patients with junctional rhythm, significant early systolic reversal of flow was present. Both the S- and D-wave velocity-time integrals (VTI) were greater in group I than in group II (S(VTI) 9.9 +/- 4.2 vs 8.0 +/- 2.6, p = 0.02; D(VTI) 8.0 +/- 3.5 vs 4.2 +/- 2.6, p <0.001). In both groups, pulmonary venous flow was predominantly systolic; however, the proportion of flow during ventricular systole was significantly greater in group II than in group I (S(VTI)/D(VTI) group II: 2.4 +/- 1.5; group I 1.4 +/- 0.5, p = 0.001; percent systolic fraction of pulmonary venous flow group II = 67%, group I = 56%, p <0.001). Analysis of superior vena caval flow in group II revealed a single predominant wave with onset at early systole and peak in late systole at a mean of 150 ms after the pulmonary venous S-wave peak. Our data suggest that ventricular systole (i.e., atrial relaxation, atrioventricular valve descent) asserts great influence on transpulmonary blood flow in the functional single ventricle.

Effect of volume unloading surgery on coronary flow dynamics in patients with aortic atresia.

OBJECTIVES: The objectives of this study were to define physiologic effects on and a clinical correlate to coronary blood flow during volume unloading surgery in patients with aortic atresia. METHODS: Twenty-two patients with aortic atresia (group I, 13 patients with stage I reconstruction undergoing hemi-Fontan operation; group II, 9 patients with hemi-Fontan undergoing Fontan operation) underwent perioperative transesophageal echocardiography. Doppler spectral patterns, peak velocity, velocity time integral, and blood flow in the native ascending aorta were measured. Preoperative hemodynamics and postoperative clinical data were analyzed. Significance was defined as p < 0.05. RESULTS: Higher values of coronary blood flow (982.9 +/- 321.7 vs 548.6 +/- 333.8 ml/min per square meter), velocity time integral (20.7 +/- 5.6 vs 12.6 +/- 4.0 cm), and peak velocity (96.1 +/- 21.4 vs 51.0 +/- 18.2 cm/sec) were found before operation in group I than after operation and in group II at both times. Flow changed from predominately systolic in preoperative group I to both systolic and diastolic after operation and in group II. Before operation in groups I and II, a number of hemodynamic parameters such as superior vena cava oxygen saturation correlated with coronary blood flow dynamics. After operation in group II, urine output (r = 0.86) and central venous pressure (r = -0.85) correlated with coronary blood flow dynamics. CONCLUSION: Coronary blood flow parameters were higher in group I as a result of the increased energy needs required to pump to two circulations. No changes were found in group II. A number of coronary blood flow parameters correlated with preoperative hemodynamics and postoperative clinical data. These parameters appear to be useful in assessing the performance status of the myocardium after the Fontan operation, consistent with the notion that myocardial perfusion relates directly to ventricular function.

Acute changes in preload, afterload, and systolic function after superior cavopulmonary connection.

BACKGROUND: Superior cavopulmonary connection reduces the volume work of the single ventricle. METHODS: To determine the effects of superior cavopulmonary connection on preload, wall stress (or afterload), and systolic ventricular function, we studied 9 patients before and after operation, and at hospital discharge. Using echocardiography, preload was estimated by the ventricular end-diastolic area, and wall stress was calculated at end-systole and peak-systole. Ventricular function was represented by rate-corrected velocity of circumferential fiber shortening and fractional area change divided by rate-corrected ejection time. RESULTS: End-diastolic area and wall stress decreased postoperatively. Ventricular wall thickness increased with a concomitant decrease in cavity area. There was no change in mean blood pressure or heart rate or in rate-corrected velocity of circumferential fiber shortening or fractional area change divided by rate-corrected ejection time. These findings persisted at hospital discharge. CONCLUSIONS: In single ventricles, superior cavopulmonary correction results in an immediate decrease in preload and afterload. The decrease in afterload results primarily from alterations in ventricular geometry. Although no improvement in systolic function was noted, diminished work related to the reduction in loading conditions may have beneficial long-term effects on preserving myocardial performance.

Avoidance of subaortic obstruction in staged management of single ventricle.

BACKGROUND: Subaortic obstruction is a frequent accompaniment of single-ventricle anatomy. Most often, the aorta arises from an outflow chamber that is connected to the single ventricle by a bulboventricular foramen or ventricular septal defect. This connection may be restrictive of flow at birth, or may become obstructive after surgical procedures that reduce the volume work of the ventricle. Subaortic obstruction is recognized as a risk factor for reconstructive surgical procedures for single ventricle. METHODS: To prevent the consequences of subaortic obstruction, we have routinely amalgamated the proximal main pulmonary artery with the ascending aorta and arch early in the management of these patients. From September 1990 through September 1994, 29 neonates and infants with single ventricle and established or potential subaortic obstruction underwent staged reconstructive surgical procedures. The initial operation in the newborn period was a Norwood procedure (18 patients) or a pulmonary artery band (5 patients). All survivors underwent a hemi-Fontan procedure at approximately 6 months. RESULTS: Eighteen patients have undergone a completion Fontan operation with no deaths. Five await completion Fontan. None has subaortic obstruction, and none has pulmonary valve insufficiency that is graded more than mild. CONCLUSIONS: Early association of the proximal main pulmonary artery with the ascending aorta appears to obviate the risks and complications associated with subaortic obstruction in patients with single ventricle.

Tetralogy of Fallot with absent pulmonary valve: echocardiographic morphometric features of the right-sided structures and their relationship to presentation and outcome.

Respiratory symptoms in tetralogy of Fallot with absent pulmonary valve are believed to be due to bronchial compression secondary to dilated pulmonary arteries; however, not all patients are born compromised. Echocardiographic morphometry of the right-sided structures was investigated to determine the possible relationship between anatomy and clinical presentation. Twenty-five patients were identified, and 15 had preoperative echocardiograms. Patients were divided into two groups: those with respiratory distress (group I, n = 9) and those without (group II, n = 6). No difference was noted in branch pulmonary artery diameters between groups; however, the pulmonary valve/ aortic valve ratio, reflecting the dimension of the narrowest pathway from the right ventricle, was larger in group I (0.74 +/- 0.15 versus 0.60 +/- 0.07, p < 0.05). Pulmonary valve diameter correlated with main and right pulmonary artery diameters. We conclude that patients with tetralogy of Fallot with absent pulmonary valve and respiratory compromise have a greater pulmonary valve/aortic valve ratio but do not have greater dilatation of proximal branch pulmonary arteries. This suggests that the pathophysiology is not due solely to compression of the bronchi but is also related to the blood flow dynamics in the pulmonary vessels.

Evaluation of regional wall motion and quantitative measures of ventricular function during dobutamine stress echocardiography in pediatric cardiac transplantation patients.

BACKGROUND: Graft coronary disease is a leading cause of death in patients who have undergone cardiac transplantation. The purpose of this study was to evaluate regional wall motion response and quantitative measures of ventricular function during dobutamine stress echocardiography (DSE) in pediatric transplantation patients. METHODS: Eleven patients were evaluated the first year after transplantation (10/11 no rejection). Ten of the 11 were reevaluated 1.2 +/- 0.3 years later (9/10 no rejection). RESULTS: Dobutamine stress echocardiography revealed the following: (1) baseline regional wall motion abnormalities in 80% that resolved in all, (2) increased heart rate and blood pressure, (3) no change in left ventricular end-diastolic diameter, (4) decreased end-systolic diameter, (5) decreased wall stress and increased velocity of circumferential fiber shortening, (6) increased VCFcZ score (representing systolic left ventricular function), and (7) a decreased mitral passive-to-active filling ratio. Patients with rejection had abnormal VCFcZ scores at rest. CONCLUSIONS: Pediatric transplantation patients without rejection have baseline regional wall motion abnormalities. With DSE, the following are present: (1) resolution of wall motion abnormalities, (2) increased contractility independent of load, and (3) changes in diastolic parameters that reflect increased heart rate. Patients with rejection may have abnormal contractility at rest.

Cardiopulmonary resuscitation: qualifications and performance of housestaff and trained internists.

Eighty-six physicians in the Internal Medicine Department of the Mount Sinai Medical Center were evaluated on attitudes and knowledge of basic and advanced cardiac life support. Information was gathered over a one-week period using a self-administered questionnaire. Eighteen (21%) of the respondents did not know of the availability of basic and advanced cardiac life support courses at this institution. Of the physicians who had never taken either course, 8 (25%) stated it was because it was not offered at a convenient time or place. All housestaff officers were noted to have completed both courses, a percentage much higher than that of the trained internists (p less than .0001). Previous formal training in cardiac life support was found to be associated with a higher level of confidence in the ability to administer cardiac lifesaving techniques (p less than .0001) and a higher overall knowledge score (p = .003). We determined that the housestaff officers in internal medicine were the physicians most qualified to perform resuscitation efforts because of their completion of life support courses, their confidence in administering these techniques, and their greater overall knowledge. Wider publicity on the availability of cardiac life support courses, more convenient scheduling of such courses, and mandatory certification in basic life support for all physicians, with additional certification in advanced cardiac life support for physicians with extensive ward responsibility, are needed.

Prevalence and spectrum of in utero structural brain abnormalities in fetuses with complex congenital heart disease.

BACKGROUND AND PURPOSE: Brain injury is a major complication in neonates with complex congenital heart disease. Preliminary evidence suggests that fetuses with congenital heart disease are at greater risk for brain abnormalities. However, the nature and frequency of these brain abnormalities detected by conventional fetal MR imaging has not been examined prospectively. Our primary objective was to determine the prevalence and spectrum of brain abnormalities detected on conventional clinical MR imaging in fetuses with complex congenital heart disease and, second, to compare the congenital heart disease cohort with a control group of fetuses from healthy pregnancies. MATERIALS AND METHODS: We prospectively recruited pregnant women with a confirmed fetal congenital heart disease diagnosis and healthy volunteers with normal fetal echocardiogram findings who underwent a fetal MR imaging between 18 and 39 weeks gestational age. RESULTS: A total of 338 fetuses (194 controls; 144 with congenital heart disease) were studied at a mean gestational age of 30.61 ± 4.67 weeks. Brain abnormalities were present in 23% of the congenital heart disease group compared with 1.5% in the control group (P < .001). The most common abnormalities in the congenital heart disease group were mild unilateral ventriculomegaly in 12/33 (36.4%) and increased extra-axial spaces in 10/33 (30.3%). Subgroup analyses comparing the type and frequency of brain abnormalities based on cardiac physiology did not reveal significant associations, suggesting that the brain abnormalities were not limited to those with the most severe congenital heart disease. CONCLUSIONS: This is the first large prospective study reporting conventional MR imaging findings in fetuses with congenital heart disease. Our results suggest that brain abnormalities are prevalent but relatively mild antenatally in fetuses with congenital heart disease. The long-term predictive value of these findings awaits further study.

Fetal cardiac tumors: prenatal diagnosis and outcome.

We present our experience in the management of fetuses diagnosed with huge cardiac tumors. These cases illustrate that the size of the tumor likely does not impact on survival as much as the location of the tumor and how it compromises blood flow into and out of the ventricles. We speculate that obstruction of right-sided inflow and/or simultaneous obstruction to outflow from both ventricles may lead to diminished cardiac output, atrial and caval hypertension, and hydrops fetalis. Obstruction can occur at any point in gestation and depends on both the size and the location of the tumor in relation to all cardiac structures. We therefore suggest serial assessment of these fetuses throughout gestation, particularly after the point of postnatal viability, to assess the hemodynamic effects that the tumor has on the heart. If obstruction to blood flow and/or early fetal compromise is noted, then the decision of whether to deliver early can be made. At the time of birth, if obstruction to blood flow persists, surgery can be considered, keeping in mind that the natural history of these tumors is to shrink and become clinically less important over time.