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.

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.

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.

Hoarseness as the initial clinical presentation of anomalous left coronary artery from the pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly that, if left untreated, will most often result in severe myocardial ischemia and significant morbidity and mortality. We report an unusual presentation of this defect in a 2-month-old infant who had an initial complaint of a "hoarse cry." We theorize that impingement of the recurrent laryngeal nerve due to dilatation of the pulmonary artery was the most likely etiology of the patient's symptoms. This case serves as an important reminder that serious congenital heart disease may present with any number of complaints and unusual findings.

Autoregulation of cerebral blood flow in fetuses with congenital heart disease: the brain sparing effect.

Fetuses with congenital heart disease (CHD) have circulatory abnormalities that may compromise cerebral oxygen delivery. We believe that some CHD fetuses with decreased cerebral oxygen supply have autoregulation of blood flow that enhances cerebral perfusion (brain sparing). We hypothesize that cerebral autoregulation occurs in CHD fetuses, and the degree of autoregulation is dependent on the specific CHD and correlates with intrauterine head circumferences. CHD fetuses were compared to normal fetuses. Data included cardiac diagnosis, cerebral and umbilical artery Doppler, head circumference, weight, and gestational age. The cerebral-to-placental resistance ratio (CPR) was assessed as a measure of cerebral autoregulation. CPR = cerebral/umbilical resistance index (RI) and RI = systolic-diastolic/systolic velocity (normal CPR > 1). CPR > 1 was found in 95% of normal vs 44% of CHD fetuses. The incidence of CPR < 1 was greatest in hypoplastic left or right heart fetuses. Compared to normal, cerebral RI was decreased in CHD fetuses. The CPR vs gestational age relationship, and the relationship among weight, head circumference, and CPR differed across normal and CHD fetuses. Fetuses > 2 kg with CHD and a CPR < 1 had smaller head circumferences than normal. Brain sparing occurs in CHD fetuses. Fetuses with single ventricular physiology are most affected. Inadequate cerebral flow in CHD fetuses, despite autoregulation, may alter brain growth.

Autosomal dominant myocardial disease diagnosed by fetal presentation of proband with an aneurysm of the muscular interventricular septum.

We describe an inherited form of a disorder in which four patients spanning three generations were affected with congenital myocardial disease. The youngest member of the family, diagnosed as a fetus with a large aneurysm of the muscular interventricular septum, demonstrates an antenatal pathogenic process. Study of the specific findings in each patient suggests that congenital aneurysms of the muscular interventricular septum may be in some way associated with specific developmental pathways of the heart. We believe that screening family members of patients with muscular interventricular septal aneurysms may be indicated to assess for silent myocardial disease.

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.

Transient neonatal cyanosis: unusual presentation of right-sided cardiac masses.

We report a case of a neonate with multiple cardiac masses, cyanosis, and a heart murmur. Arterial desaturation was the result of right-to-left shunting at the foramen ovale level caused by tricuspid regurgitation. Three right-sided cardiac masses were detected by echocardiography. By 2 weeks of age the patient had complete resolution of his cyanosis and improved tricuspid regurgitation following the normal decrease in pulmonary vascular resistance. At 2 years of age, he has no cardiovascular symptoms and the masses are calcified and have no hemodynamic consequences.

Multiple left-sided cardiac lesions in one of Noonan's original patients.

We describe a complex case of obstruction of the left ventricular outflow tract in one of Dr. Noonan's original patients. Intraoperative findings revealed pathology at the valvar, subvalvar and supravalvar positions. Patients with Noonan syndrome are traditionally described as having right-sided cardiac pathology. Review of the literature revealed left-sided lesions to occur in a substantial number of these patients. We therefore suggest the routine employment of cardiac ultrasonography in all patients with Noonan syndrome with attention directed toward left-sided pathology, as well as the frequent pulmonary valvar pathology.

Regional wall motion and strain of transplanted hearts in pediatric patients using magnetic resonance tagging.

Abnormal ventricular systolic torsion is present during histological rejection in adult cardiac transplant patients. Because biomechanical properties of transplanted hearts in the baseline state have not been studied in children, pediatric patients were evaluated to quantify ventricular wall motion and strain. Eight transplant studies and eight normal controls were evaluated. Magnetic resonance tagging was performed to determine radial shortening, twist, and strain in four ventricular anatomic areas at two short-axis levels. Controls had counterclockwise twist. Six transplant studies had clockwise twist, six had akinetic regions, and all had regions of no twist. One demonstrated paradoxical motion of the septum. A comparison between transplant patients and controls revealed strain to be similar in all regions except one (superior wall at the atrioventricular valve level) and strain distribution to be different only in two of eight regions. Pediatric transplant patients demonstrate regional wall motion abnormalities in the absence of rejection. Compared with normal controls, the transplanted left ventricle maintains normal strain in the presence of abnormal twist. This may be a compensatory mechanism and have clinical implications.

Fetal presentation of congenital long QT syndrome.

Thirty-two-week prenatal ultrasound revealed a fetal heart rate of 100 bpm with decreased variability on the heart rate tracing. Echocardiogram documented normal anatomy and sinus bradycardia. Newborn electrocardiogram revealed sinus rhythm at 100 bpm with a QTc of 0.657. Follow-up electrocardiogram revealed a QTc interval of 0.568, 2:1 atrioventricular block with a ventricular rate of 60 bpm, and ventricular ectopy. The infant received a pacemaker and beta-blocker therapy. Long QT syndrome should be in the differential diagnosis of the fetus with bradycardia and decreased heart rate variability in the absence of distress. Early diagnosis allowed for preventative care in the infant and identification of family members at risk.

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.

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.

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.

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.