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AbstractBackground:The transition from residency to paediatric cardiology fellowship is challenging due to the new knowledge and technical skills required. Online learning can be an effective didactic modality that can be widely accessed by trainees. We sought to evaluate the effectiveness of a paediatric cardiology Fellowship Online Preparatory Course prior to the start of fellowship.Methods:The Online Preparatory Course contained 18 online learning modules covering basic concepts in anatomy, auscultation, echocardiography, catheterisation, cardiovascular intensive care, electrophysiology, pulmonary hypertension, heart failure, and cardiac surgery. Each online learning module included an instructional video with pre-and post-video tests. Participants completed pre- and post-Online Preparatory Course knowledge-based exams and surveys. Pre- and post-Online Preparatory Course survey and knowledge-based examination results were compared via Wilcoxon sign and paired t-tests.Results:151 incoming paediatric cardiology fellows from programmes across the USA participated in the 3 months prior to starting fellowship training between 2017 and 2019. There was significant improvement between pre- and post-video test scores for all 18 online learning modules. There was also significant improvement between pre- and post-Online Preparatory Course exam scores (PRE 43.6 ± 11% versus POST 60.3 ± 10%, p < 0.001). Comparing pre- and post-Online Preparatory Course surveys, there was a statistically significant improvement in the participants’ comfort level in 35 of 36 (97%) assessment areas. Nearly all participants (98%) agreed or strongly agreed that the Online Preparatory Course was a valuable learning experience and helped alleviate some anxieties (77% agreed or strongly agreed) related to starting fellowship.Conclusion:An Online Preparatory Course prior to starting fellowship can provide a foundation of knowledge, decrease anxiety, and serve as an effective educational springboard for paediatric cardiology fellows.

Byline: Francesco Seddio, V.Mohan Reddy, Doff B. McElhinney, Wayne Tworetzky, Norman H. Silverman, Frank L. Hanley Abstract: Background: Congenital heart lesions with multiple ventricular septal defects remain a surgical challenge. Traditional approaches often rely on either ventriculotomy for exposure or palliation with pulmonary artery banding. However, indications for repair versus palliation and for various approaches to surgical exposure are not clearly defined. Methods: From July 1992 to January 1998, 45 patients with multiple ([greater than or equal to]2) ventricular septal defects (37 with associated lesions) underwent surgery. Median age was 86 days; all but 4 patients were infants. The mean number of defects was 3.7, and almost half of the patients had more than 3 defects. Apical muscular defects were present in 62% of patients. Thirty-one patients underwent primary complete repair through a right atriotomy or trans-semilunar valve approach (group 1), 8 had palliation (group 2), and 6 underwent complete repair after prior palliation elsewhere (group 3). No patient had a ventriculotomy. Results: One early death occurred in a group 1 patient. Four patients who had had palliation (50%) underwent early reoperation for pulmonary artery band revision because of failure to thrive or band removal after spontaneous closure of the defects. At follow-up (median 22 months), there was 1 death in a group 2 patient (palliation) and 1 other group 2 patient required cardiac transplantation. The only late reoperation was for removal of the pulmonary artery band and closure of multiple apical defects in a group 2 (palliation) patient. No patients who underwent repair have hemodynamically significant residual defects. Conclusions: In our experience, palliation of multiple ventricular septal defects is associated with greater morbidity than primary repair. Multiple defects can almost always be repaired adequately in early infancy without ventriculotomy, although 'Swiss-cheese' septum may be an indication for palliation. (J Thorac Cardiovasc Surg 1999;117:134-40) Author Affiliation: From the Divisions of Cardiothoracic Surgery.sup.a and Pediatric Cardiology,.sup.b University of California, San Francisco Article History: Received 8 May 1998; Revised 16 July 1998; Revised 30 September 1998; Accepted 30 September 1998 Article Note: (footnote) [star] Address for reprints: V. Mohan Reddy, MD, UCSF Medical Center, 505 Parnassus Ave, M593, San Francisco, CA 94143-0118., [star][star] 12/6/94820

Blood flow in the ductus arteriosus occurs later and accelerates more slowly than in the aortic isthmus during the heart beat in fetal lambs. Researchers studied blood flow in seven fetal lambs, and found characteristic flow patterns in the aortic isthmus, ductus arteriosus, and the pulmonary artery. The ductus arteriosus causes blood to bypass the circulation in the lung during gestation, and closes by birth.

Byline: V.Mohan Reddy, Doff B. McElhinney, Norman H. Silverman, Stefano M. Marianeschi, Frank L. Hanley Abstract: Background: Partial biventricular repair consists of bidirectional cavopulmonary anastomosis in the presence of separated systemic and pulmonary circulations, with antegrade flow of inferior caval return through an intact or reconstructed pulmonary outflow tract. This versatile procedure may be used as a definitive repair for patients with a functional right heart complex incapable of supporting an entire cardiac output or in patients with complicated anatomy. Methods: From July 1992 to April 1997, 23 patients (median age 5.2 years) underwent partial biventricular repair. In 15 of these cases the entire repair, including bidirectional cavopulmonary anastomosis, intracardiac repair, and right ventricular outflow reconstruction, was performed as a planned procedure at our institution. The other eight patients had previously been placed on a Fontan track and had undergone bidirectional cavopulmonary anastomosis; their circulations were converted to a partial biventricular circulation. Results: There were no early deaths. Complete atrioventricular block developed in two patients with straddling tricuspid valve. At a median follow-up of 17 months, there were no late deaths and three patients had undergone reintervention. Partitioning of the pulmonary arteries to create a classic Glenn anastomosis with antegrade flow to the left lung was performed in one case; another patient underwent an atrial septectomy, and the third patient required revision of tricuspid valve repair. All patients are in New York Heart Association functional class I. Conclusions: Partial biventricular repair is a versatile strategy that can be used to manage a variety of forms of complex congenital heart disease. Cases for which the repair is useful include those in which complete biventricular repair is unlikely to be achieved because of limited size or function of the right side of the heart and those in which a patient with a ventricle capable of supporting inferior vena caval return was previously placed on a Fontan track. (J Thorac Cardiovasc Surg 1998;116:21-7) Article History: Received 8 July 1997; Revised 9 October 1997; Revised 11 February 1998; Accepted 19 February 1998 Article Note: (footnote) [star] From the Divisions of Cardiothoracic Surgerya and Pediatric Cardiology,b University of California, San Francisco, Calif., [star][star] Read at the Twenty-third Annual Meeting of The Western Thoracic Surgical Association, Napa, Calif., June 25-28, 1997., a Address for reprints: V. Mohan Reddy, MD, Division of Cardiothoracic Surgery, 505 Parnassus Ave., M593, San Francisco, CA 94143-0118., aa 12/6/89805

Byline: Doff B. McElhinney, V.Mohan Reddy, Norman H. Silverman, Frank L. Hanley Abstract: Background: A modified Damus-Kaye-Stansel procedure is one of several options for palliation of single ventricle with subaortic obstruction, but results in neonates have been disappointing. In the presence of arch obstruction, this procedure is typically performed with circulatory arrest, which may contribute to neurologic insult. Methods: Since 1990, a modified Damus-Kaye-Stansel procedure has been performed in 14 neonates and seven infants with single ventricle and subaortic stenosis, including 15 with arch obstruction. Diagnoses were double-inlet left ventricle (n = 12), tricuspid atresia (n = 2), and other forms of hypoplastic ventricle with subaortic obstruction (n = 7). Three patients underwent concurrent bidirectional Glenn shunt. In the most recent seven patients with arch obstruction, arch repair was achieved with an end-to-side anastomosis of the descending aorta to the ascending aorta with continuous upper body perfusion. Results: One early death occurred among the 14 neonates (7%) and three among the infants, for an early mortality of 19%. At a median follow-up of 33 months, there were no late deaths or neurologic complications. Nine patients underwent subsequent bidirectional Glenn anastomosis, including three who had Fontan completion and one who later underwent conversion to a partial biventricular repair. One patient required a transplant for cardiomyopathy of unknown etiology. The remaining 12 patients are considered good candidates for Fontan completion. No patient has recurrent arch obstruction. Four patients have mild (n = 1) or trivial (n = 3) semilunar valvular regurgitation. Conclusion: The modified Damus-Kaye-Stansel procedure is an effective primary palliation for single ventricle and subaortic stenosis, with or without arch obstruction. Results are especially encouraging in neonates. Arch repair can be achieved without circulatory arrest to the brain (J Thorac Cardiovasc Surg 1997;114:718-26). Article History: Received 7 May 1997; Revised 12 June 1997; Revised 11 July 1997; Accepted 17 July 1997 Article Note: (footnote) [star] From the Divisions of Cardiothoracic Surgerya and Pediatric Cardiology,b University of California, San Francisco, Calif., [star][star] Read at the Seventy-seventh Annual Meeting of The American Association for Thoracic Surgery, Washington, D.C., May 4-7, 1997., a Address for reprints: V. Mohan Reddy, MD, 505 Parnassus Ave., M589, San Francisco, CA 94143-0118., aa 0022-5223/97 $5.00 + 0 12/6/84731

Byline: Jacques A.M. van Son, V.Mohan Reddy, Norman H. Silverman, Frank L. Hanley Abstract: The cases of five patients with previous Senning (n = 4) or Mustard (n = 1) operations and failing systemic ventricles in whom banding of the pulmonary artery was performed as an interim step toward an arterial switch procedure are reported. The rise in the ratio of left to right mean systolic ventricular pressure, from 0.35 before operation to 0.90 during operation and 0.80 on the first postoperative day, caused a shift of the ventricular septum from a leftward to a midline or nearly midline position. This shift was associated with a reduction of tricuspid regurgitation. At a median interval of 5.1 months after pulmonary artery banding, the mean left ventricular posterior wall thickness had increased to 8.2 mm, versus 5 mm before operation, and the mean left ventricular myocardial mass index had increased to 90 gm/m.sup.2, versus 55.6 gm/m.sup.2 before operation. After the arterial switch operation, which was performed in four patients, the tricuspid regurgitation decreased to a trivial amount (n = 1) or disappeared completely (n = 3). (J THORAC CARDIOVASC SURG 1996;111:342-7) Author Affiliation: San Francisco, Calif. Article History: Received 13 February 1995; Accepted 13 April 1995 Article Note: (footnote) [star] From the Divisions of Cardiothoracic Surgery and Pediatric Cardiology, UCSF, San Francisco, Calif., [star][star] Address for reprints: F. L. Hanley, MD, Division of Cardiothoracic Surgery, UCSF, 505 Parnassus Ave., San Francisco, CA 94143-0118., a 0022-5223/96 $5.00 + 0, aa 12/1/65670

Doppler echocardiography of right ventricular output in fetal sheep appears to accurately estimate fetal heart output. Doppler echocardiography in the human fetus has been limited, and its accuracy in measuring heart output and stroke volume remains unknown. Right ventricular output was measured in six sheep fetuses using Doppler echocardiography, a transit-time ultrasonic flowmeter and a microsphere technique. Of 72 flow measurements, 34 were obtained by the transabdominal approach and 38 were obtained by the transuterine approach. The transabdominal approach estimated fetal heart output well, but the correlation between fetal heart output and Doppler echocardiography was significantly better when the transuterine approach was used. The correlation between the transit-time flowmeter and the microsphere measurement was extremely good, indicating the accuracy of Doppler echocardiography in measuring right ventricular output.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2010.05.012 Byline: Derek Y. Obayashi (a), Jay Yeh (b), Norman H. Silverman (a), Frank L. Hanley (c) Author Affiliation: (a) Division of Pediatric Cardiology, Stanford University School of Medicine, Stanford, Calif (b) Department of Pediatrics, Children's Hospital and Research Center Oakland, Oakland, Calif (c) Department of Cardiothoracic Surgery, Stanford University School of Medicine, Stanford, Calif Article History: Received 25 July 2008; Revised 7 May 2010; Accepted 16 May 2010

Assessment of cardiac dimensions of the chambers, great arteries and veins in the human fetus is important to distinguish abnormal dimensions from normal. This study establishes normal values based on cross-sectional echocardiographic measurements over the gestational period where these measurements may be clinically useful. Ventricular and atrial dimensions were measured from the 4-chamber view, the short-axis dimension immediately below the mitral and tricuspid valve leaflets in diastole, and the long axis from the closed apposed atrioventricular valves to their respective apices. The ventricular walls and septum were measured at the level at which cavity dimensions in diastole were measured, defining both the left and right ventricular wall thickness, as well as that of the ventricular septum Furthermore, the long axis of the right and left atria was measured from the center of the apposed atrioventricular valve leaflets to the posterior atrial wall, and the sizes of the atrial chambers were defined using their widths at the prospective broadest points through the area of foramen ovale. From a variety of views, diameters were measured at maximal expansion of the main, left and right pulmonary arteries, the ductus arteriosus, and the superior and inferior venae cavae. The data were evaluated longitudinally from 18 weeks to term, and regression analysis was performed using the best fit of a linear or polynomial equation. The data provide a means for evaluating the normal sizes and dimensions of the fetal heart chambers, as well as the thickness of the ventricular walls and septum (Am J Cardiol 1992;70:1459-1467)

Conjoined twins (joined together; commonly known as Siamese twins) do not often occur. Most conjoined twins are female and many die before birth. Diagnosis of this condition prior to birth has been greatly facilitated by new technologies. The imaging techniques of ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MR) can detect and help evaluate this condition. Early detection of conjoined twins helps in making decisions about terminating pregnancy, type of delivery, and care and treatment of problems associated with this defect. The problems vary greatly, depending upon the location and extent of the fusion of the fetuses. This study reviewed imaging information on 14 cases of conjoined twins to develop useful guidelines for future cases. Imaging examinations should be considered when a twin pregnancy is found to have a single placenta and the amniotic membrane is not visibly separated; these are indications of conjoined twins. US indicators of conjoined twins include inseparable fetal bodies, inseparable skin contours, and no relative changes in fetal position. The sonograms must be examined closely since false-positive diagnosis (identification of fusion in a normal twin pregnancy) may occur. US findings revealed that most conjoined twins were fused such that they faced each other, and many shared the same heart as well as other organs. This information is important in determining whether or not to separate the twins at birth, and whether or not to terminate the pregnancy. Imaging is important in determining type of delivery, because vaginal delivery can adversely affect both the mother and the twins, depending on the size of the twins and how they are connected. The prognosis for conjoined twins is poor, but accurate diagnosis and assessment are necessary in deciding what steps to take to ensure the safety of the mother and, if possible, the survival of one or both of the twins. (Consumer Summary produced by Reliance Medical Information, Inc.)

Early postoperative left ventricular (LV) dysfunction is observed following repair of large ventricular septal defects (VSDs), but the frequency and rate of recovery of LV function are unknown. This study aims to characterize the incidence and rate of recovery of postoperative LV dysfunction following repair of large VSDs and to improve understanding of LV mechanics before and after VSD repair.

AbstractDr Abraham Rudolph, one of the foremost leaders in Pediatric Cardiology, passed away on April 9, 2023, aged 99. His entire career was marked by imagination, creativity, and a devotion to the care of children with heart disease. He will be sorely missed by the many who knew him personally and the countless physicians whose understanding of their specialty was enhanced by his discoveries and teachings.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2005.02.018 Byline: Kimberly L. Gandy (a), Matthew A. Burtelow (b), V. Mohan Reddy (a), Norman H. Silverman (c) Author Affiliation: (a) Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Stanford, Calif. (b) Department of Pathology, Stanford University School of Medicine, Stanford, Calif. (c) Division of Pediatric Cardiology, Stanford University School of Medicine, Stanford, Calif. Article History: Received 19 January 2005; Accepted 8 February 2005

AbstractObjectivesTrans-oesophageal echocardiographic imaging is valuable in the pre- and post-operative evaluation of children and adults with CHD; however, the frequency by which trans-oesophageal echocardiography guides the intra-operative course of patients is unknown.MethodsWe retrospectively reviewed 1748 intra-operative trans-oesophageal echocardiograms performed between 1 October, 2005 and 31 December, 2010, and found 99 cases (5.7%) that required return to bypass, based in part upon the intra-operative echocardiographic findings.ResultsThe diagnoses most commonly requiring further repair and subsequent imaging were mitral valve disease (20.9%), tricuspid valve disease (16.0%), atrioventricular canal defects (12.0%), and pulmonary valve disease (14.1%). The vast majority of those requiring immediate return to bypass benefited by avoiding subsequent operations and longer lengths of hospital stay. A total of 14 patients (0.8%) who received routine imaging required further surgical repair within 1 week, usually due to disease that developed over ensuing days. Patients who had second post-operative trans-oesophageal echocardiograms in the operating room rarely required re-operations, confirming the benefit of routine intra-operative imaging.ConclusionsThis study represents a large single institutional review of intra-operative trans-oesophageal echocardiography, and confirms its applicability in the surgical repair of patients with CHD. Routine imaging accurately identifies patients requiring further intervention, does not confer additional risk of mortality or prolonged length of hospital stay, and prevents subsequent operations and associated sequelae in a substantial subset of patients. This study demonstrates the utility of echocardiography in intra-operative monitoring of surgical repair and highlights patients who are most likely to require return to bypass, as well as the co-morbidities of such manipulations.

AbstractIn the 1970s, diagnosing anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was often uncertain using imaging alone; however, with the advances in high-frequency transducers, advanced image processing, and other ultrasound modalities such as Doppler colour flow imaging, tissue Doppler imaging, and speckle tracking to asses regional wall motion abnormalities, modern echocardiography now permits accurate diagnosis of ALCAPA with greater certainty. Although many consider ultrasound to be the only imaging test necessary if there is a question as to the diagnosis, other imaging modalities such as MRI, CT, and cardiac catheterisation with angiography remain valuable complementary tests, especially in older patients.

AbstractAn infant presented with features suggestive of an anomalous left coronary artery was found to have normal origins of both coronary arteries. Echocardiography during episodes of ischaemia showed marked aortic regurgitation with retrograde coronary flow. The left coronary leaflet was mildly hypoplastic. Surgical re-suspension of this leaflet prevented aortic regurgitation and the patient had no further symptoms and recovered cardiac function.

AbstractI surveyed our echocardiographic database of the years between 1998 and 2012 for congenital abnormalities of the mitral valve in patients over 14 years. A total of 249 patients with mitral valve abnormalities were identified. Abnormalities included clefts in the mitral valve in 58 patients, double orifice of the mitral valve in 19, mitral stenosis with two papillary muscles in 72, and mitral stenosis with one papillary muscle in 51 patients. Supravalvar rings were found in 35 patients with a single papillary muscle, and mitral stenoses with two papillary muscles were found in 22 patients. Mitral prolapse occurred in 44 patients and mitral valvar straddle in five patients. The patients were evaluated by all modalities of ultrasound available over the course of time. Although some lesions were isolated, there were many lesions in which more than one mitral deformity presented in the same patient. The patients are presented showing anatomical correlation with autopsy specimens, some of which came from the patients in this series, and others matched to show correlative anatomy. These lesions remain rare as a group and continue to have high morbidity and mortality.

Background:Preterm infants with a patent ductus arteriosus (PDA) are at risk for death or development of bronchopulmonary dysplasia (BPD). However, PDA treatment remains controversial. We investigated if PDA treatment and other clinical or echocardiographic (ECHO) factors were associated with the development of death or BPD.Methods:We retrospectively studied clinical and ECHO characteristics of preterm infants with birth weight <1,500?g and ECHO diagnosis of a PDA. Logistic regression and classification and regression tree analyses were performed to assess variables associated with the combined outcome of death or BPD.Results:Of 187 preterm infants with a PDA, 75% were treated with indomethacin or surgical ligation and 25% were managed conservatively. Death or BPD occurred in 80 (43%) infants. The results of logistic regression analyses showed that lower gestational age (odds ratio (OR): 0.5), earlier year of birth during the study period (OR: 0.9), and larger ductal diameter (OR: 4.3) were associated with the decision to treat the PDA, whereas gestational age was the only variable associated with death or BPD (OR: 0.6; 95% confidence interval: 0.5–0.8).Conclusion:Only lower gestational age and not PDA treatment or ECHO score was associated with the adverse outcome of death or BPD. Further investigation of PDA management strategies and effects on adverse outcomes of prematurity is needed.

Rare cases of aortic–left atrial tunnel exist in the literature. This case report highlights the echocardiographic characterization of this vascular anomaly and provides the first description of an aortic–left atrial tunnel closed by interventional cardiac catheterization in a pediatric patient.

AbstractThis presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

AbstractThe successful diagnosis, surgical planning, and long-term care of children with transposition of the great arteries require high-quality cardiac imaging with echocardiography. Echocardiography must identify the relevant anatomic variants of transposition of the great arteries, such as of ventricular septal defects and aortic arch anomalies. Methodical and detailed imaging of the coronary arteries is particularly important, as translocation of the coronary arteries is a critical component of the arterial switch procedure. Familiarity with the potential coronary artery variants and the ideal imaging planes is essential for an echocardiographer. Knowledge of both the early and late complications following the arterial switch procedure is essential to optimise post-operative echocardiography. These complications can include residual lesions leading to haemodynamic compromise or progressive late phenomena, such as aortic root dilatation and aortic insufficiency. Echocardiography will continue to be the cornerstone to the lifelong management of transposition of the great arteries, and improvements in technology and increased familiarity with modalities such as stress echocardiography will enhance the role of advanced imaging even further.

Left ventricular noncompaction (LVNC) has rarely been described in the fetus.

In complete transposition of the great vessels, a restrictive patent foramen ovale leads to inadequate circulatory mixing and severe cyanosis. Urgent balloon atrial septostomy (BAS) improves mixing and bridges neonates to surgery. Several studies have determined risk factors in utero for poor postnatal outcomes in complete transposition of the great vessels, particularly a restrictive patent foramen ovale and ductus arteriosus. In addition to these risk factors, we studied two new features, a hypermobile septum and reverse diastolic patent ductus arteriosus shunt, to determine which patients will require an urgent BAS.

AbstractIn the past, coronary arterial anomalies have been difficult to diagnose by non-invasive methods. Identification of coronary arterial origins is now a routine part of the standard paediatric echocardiogram. Anomalous origin of a coronary artery from the pulmonary trunk is an extremely important diagnosis to make. Many echocardiographic features are not directly related to the visualisation of the coronary arterial origin. Left ventricular dilation and abnormal ventricular performance are common, along with mitral regurgitation and evidence of collateralisation of the flow from the coronary artery that has an aortic origin. In some cases, the anomalous coronary artery can be seen to arise directly from the pulmonary trunk. Congenital atresia of the main stem of the left coronary artery has a similar echocardiographic presentation, except that its aortic origin is not determined. Anomalous aortic origin of the coronary artery has important implications, as the first presenting symptom can be sudden death. With meticulous attention to the origins of the coronary arteries, echocardiographic diagnosis can also be achieved. In contrast to the anomalous origin of a coronary artery from the pulmonary trunk, ventricular performance is usually normal. Whenever there is doubt as to the definition of the origin of the coronary arteries and, indeed, when there is serious clinical concern that a coronary artery has an anomalous origin, other testing, such as cine-computed tomography, magnetic resonance imaging, or cardiac catheterisation may be indicated for confirmation or to provide greater anatomic detail.

AbstractThere is a collection of rare congenital cardiac defects that can produce significant haemodynamic embarrassment. Owing to their rarity, these lesions may be overlooked or mistaken for other more common congenital cardiac defects. Using careful segmental echocardiographic techniques, such as multiple planes of imaging and inferential Doppler findings, it is possible to identify these lesions and thus plan surgical management. Some of the lesions of importance reviewed include the aortopulmonary window, the aorto-ventricular tunnels, the aorto-atrial tunnels, and aneurysm of the sinuses of Valsalva. Some conditions such as the aortopulmonary window and aneurysm of the sinuses of Valsalva often occur in the setting of other congenital cardiac lesions. At times, the presence of coexisting lesions may mask some of the characteristic features of these defects. With prompt identification, all of these rare conditions are amenable to complete surgical repair. Newer modalities such as three-dimensional echocardiography can further enhance surgical planning.

To determine whether a continuous infusion of heparin reduces the rate of catheter-related thrombosis in neonates and infants post cardiac surgery. Central venous and intracardiac catheters are used routinely in postoperative pediatric cardiac patients. Catheter-related thrombosis occurs in 8% to 45% of pediatric patients with central venous catheters.

It has long been known that the most complex combinations of cardiac malformations are those found in the setting of the so-called “splenic syndromes”. Many aspects of these syndromes have been controversial over recent years, not least the presence or absence of features of isomerism within the heart. Recent experience with genetic manipulation of mice, nonetheless, has now shown that it is possible to generate unequivocal evidence of cardiac isomerism, particularly in those animals which show features of right isomerism when the genes responsible for morphologically leftness are knocked out. Furthermore, when the crucial philosophical principle known as the “morphological method” is applied to the hearts of patients known to have visceral heterotaxy, it is equally clear that patients falling within these groups, when judged on the extent of the pectinate muscles relative to the atrioventricular junctions, exhibit isomerism of either the morphologically right or left atrial appendages.

AbstractObjective:Pericardial teratomas are rare congenital tumors which invade the developing mediastinum, compressing the venous return to the heart, leading to hydrops. Tumors, with large cystic components, have been treated previously with in utero pericardiocentesis with some success. We present the first reported case of in utero open resection of a fetal pericardial teratoma. Methods:A 31-year-old G1P0 woman was found to have a fetus with a pericardial teratoma. Hydrops developed at 24 weeks’ gestation. After counseling, open fetal resection was performed via a fetal median sternotomy. Results:Although the tumor was successfully removed, the hydrops did not resolve. In addition, over the course of 3 weeks, the mother developed maternal mirror syndrome which prompted an emergent cesarean section. Neonatal death ensued shortly after birth. Conclusions:The fetus with a pericardial teratoma complicated by hydrops is compromised. Treatment options include early delivery, aspiration of the pericardial effusion, and in utero operative resection.Copyright © 2002 S. Karger AG, Basel

The echocardiographic diagnosis of systemic venous anomalies often is rendered difficult by the variety of lesions that exist. An understanding of the embryologic processes that result in these lesions is essential for accurate identification, since these lesions often are not obvious on routine echocardiographic examination. Standard echocardiographic views may demonstrate some lesions, whereas many require modified views to outline the abnormal systemic venous anatomy. This paper reviews the basic embryologic processes of the development of the normal and abnormal systemic venous system, as well as the echocardiographic identification of these major systemic venous malformations.

OBJECTIVES

The purpose of our study in fetal sheep was to assess the measurement agreement between fetal transesophageal echocardiography (FTEE) and conventional maternal transabdominal echocardiography (CMTFE) by the Bland–Altman method. We performed our study in 11 fetal sheep between 95–103 days of gestation (term = 145 days). FTEE was performed by imaging the fetal heart in horizontal planes utilizing a 10-F, 10-MHz intravascular ultrasound catheter. CMTFE was carried out using a 5.0-MHz phased-array transducer replicating the FTEE imaging planes. We found close agreement between FTEE and CMTFE measurements of great vessel and cardiac valvar dimensions. Conversely, the variability between both techniques for measuring ventricular dimensions was inadequate. We conclude that FTEE permits measurement of great vessel and cardiac valve dimensions with high agreement with CMTFE measurements. This finding strengthens the applicability of FTEE as a monitoring tool during experimental open or fetoscopic fetal cardiac interventions.

Abstract: A subtype of supracardiac total anomalous pulmonary venous return (TAPVR) consists of the vertical vein passing between the left pulmonary artery and the left mainstem bronchus resulting in relative obstruction to pulmonary venous return. This has been termed the vascular vise. In this situation, the supracardiac type of TAPVR (Type I) may mimic radiographically the infradiaphragmatic type (Type 3).

OBJECTIVES

Summary We report here a patient with interrupted aortic arch and a right descending aorta, in whom the surgical management was complicated by the development of right bronchus compression and unilateral emphysema, due to the presence of the right ductus arteriosus.

Objectives. We sought to determine the prevalence and natural history of cardiac tumors in patients referred for fetal echocardiography.