Thrombus formation after the Fontan operation.

Background: Thrombus formation is common after a Fontan operation. We investigated the frequency and location of thrombus in our population of children based on the type of Fontan operation performed.

Methods And Results: Between January 1987 and January 1999, 592 patients underwent echocardiography after Fontan operation and 52 (8.

Evaluation and follow-up of patients with left ventricular apical to aortic conduits with 2D and 3D magnetic resonance imaging and Doppler echocardiography: A new look at an old operation.

Background: Although the interposition of left ventricular apical to descending aorta conduits has diminished with the advent of the Ross-Konno operation, it remains a useful option. We reviewed our institutional experience imaging these conduits and tested the hypothesis that the gradient across the native left ventricular outflow tract (LVOT) by echocardiography correlated with the conduit gradient by cardiac catheterization. In a patient with an unobstructed conduit, no gradient should exist across the native LVOT.

Outcome following tricuspid valve detachment for ventricular septal defects closure.

Objectives: Detachment of the septal leaflet of the tricuspid valve from the annulus (TVD) has been used to improve visualization of ventricular septal defects (VSDs), but may be associated with increased operative time, heart block, and the development of tricuspid regurgitation (TR).

Methods: Patients undergoing VSD closure between 1/1/96 and 31/12/99 were retrospectively reviewed. Follow-up was obtained from the patients' cardiologists.

Echocardiographic evaluation of the fetus with congenital cystic adenomatoid malformation.

Objectives: The development of nonimmune hydrops is a known complication of congenital cystic adenomatoid malformation (CCAM) in the fetus. The aim of this study was to investigate the impact of CCAM on hemodynamics in the fetus and to determine whether cardiac dysfunction contributes to the development of hydrops in this patient population.

Methods: The echocardiographic data from 41 consecutive fetuses diagnosed prenatally with CCAM were reviewed.

Evaluation of ventricular septal defect repair using intraoperative transesophageal echocardiography: frequency and significance of residual defects in infants and children.

Intraoperative transesophageal echocardiography (IOTEE) is commonly used to assess for residual defect and the need to return to bypass after repair of ventricular septal defect (VSD). The frequency and significance of residual septal defects as noted on IOTEE has not been well defined. We evaluated the frequency of residual VSD via IOTEE and the relationship between size of a residual VSD and rate of reoperation.

Assessment of pulmonary/systemic blood flow ratio after first-stage palliation for hypoplastic left heart syndrome: development of a new index with the use of doppler echocardiography.

Objective: Circulatory maldistribution is believed to be a major cause of early death after first-stage palliation for hypoplastic left heart syndrome. Flow reversal in the reconstructed aorta may reflect the pulmonary/systemic blood flow ratio. The purpose of our study was to investigate the utility of arterial PO (2), arterial oxygen saturation, and a newly developed Doppler-derived flow index in predicting the pulmonary/systemic flow ratio after first-stage palliation for hypoplastic left heart syndrome.

Outcome following single-stage repair of coarctation with ventricular septal defect.

Objective: A recent multi-institutional study suggested improved survival (97%) after staged repair of coarctation with ventricular septal defect (VSD) compared with single-stage repair. The current study was undertaken to determine outcome and need for reintervention following single-stage repair of coarctation and VSD at our institution.

Methods: Retrospective review of patients undergoing single-stage repair of coarctation with VSD between October 1, 1994 and August 15, 1999.

Pulmonary hypertension in children following extracorporeal membrane oxygenation therapy and repair of congenital diaphragmatic hernia.

Objective: Pulmonary hypertension (PHT) is present in all children at birth, but its degree and rate of resolution in infants diagnosed with congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) need to be established.

Study Design: Twenty-one ECMO/CDH survivors (aged 3.2 +/- 1.

Early results of the Ross procedure in simple and complex left heart disease.

Background: The Ross procedure has been used increasingly to treat aortic valve disease in children and young adults. Benefits include the lack of anticoagulation after surgery and the potential growth and durability of the autograft. The purpose of this study was to review our institutional experience with the Ross procedure and to compare early outcome in simple aortic valve disease and complex left heart disease.

The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome.

Objectives: The purpose of this study was to investigate the outcome in infants with hypoplastic left heart syndrome and intact atrial septum and to evaluate the relationship of atrial morphology, left atrial decompression pathway and lung histopathology to outcome.

Background: In the hypoplastic left heart syndrome, severe restriction at the atrial level results in marked systemic hypoxemia after birth. Infants with intact atrial septum may be at high risk for mortality after Norwood operation.

Outcome after repair of tetralogy of Fallot with absent pulmonary valve.

Background: Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression.

Methods: Since January 1, 1984, 28 patients with TOF/ APV have undergone complete repair (median age 11 days, range 1 day to 16 years).

Results: Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3.

Usefulness of intraoperative transesophageal echocardiography in predicting the degree of mitral regurgitation secondary to atrioventricular defect in children.

The objectives of this study were to determine the validity of the grade of mitral regurgitation (MR) as imaged by intraoperative transesophageal echocardiography (TEE) in predicting the grade of MR at follow-up. Intraoperative TEE and corresponding follow-up transthoracic studies were retrospectively reviewed and the regurgitant jet area to left atrial area ratio was used to quantify the MR. Patient records were reviewed to identify factors contributing to the development of a certain grade of MR.

Caval contribution to flow in the branch pulmonary arteries of Fontan patients with a novel application of magnetic resonance presaturation pulse.

Background: A complete understanding of fluid mechanics in Fontan physiology includes knowledge of the caval contributions to right (RPA) and left (LPA) pulmonary arterial blood flow, total systemic venous return, and relative blood flow to each lung.

Methods And Results: Ten Fontan patients underwent cine MRI. Three cine scans of the pulmonary arteries were performed: (1) no presaturation pulse, (2) a presaturation pulse labeling inferior vena cava (IVC) blood (signal void), and (3) a presaturation pulse labeling superior vena cava (SVC) blood.

Changes in oxygenation with inhaled nitric oxide in severe bronchopulmonary dysplasia.

Background: Severe bronchopulmonary dysplasia (BPD), which is associated with high mortality and morbidity, is thought to be the result of mechanical, inflammatory, and oxidant injury to the immature lung, and includes the development of pulmonary hypertension with vascular remodeling.

Methods: A phase II pilot study was conducted to determine the effect of inhaled nitric oxide (iNO) on oxygenation in severe BPD. This was an open-labeled, noncontrolled trial to evaluate safety and determine appropriate dosing for a future randomized controlled trial.

Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection.

Background And Methods: Between January 1, 1984, and December 1, 1997, 73 infants with functional single ventricle and total anomalous pulmonary venous connection were admitted to our institution. A retrospective review was undertaken to determine factors influencing survival.

Results: Heterotaxy syndrome was present in 52 patients and hypoplastic left heart syndrome in 14.

Growth characteristics of the aortic arch after the Norwood operation.

Objectives: We sought to characterize the growth of the reconstructed aortic arch after the Norwood operation (NO).

Background: The first stage of surgical palliation of hypoplastic left heart syndrome (HLHS), the NO, includes augmentation of the aortic arch with homograft. Growth characteristics of the reconstructed aortic arch, which is comprised of both native aortic tissue and homograft, have not been characterized.

Outcome after operations for pulmonary atresia with intact ventricular septum.

Objective: Pulmonary atresia with intact ventricular septum is an anatomically heterogeneous anomaly with a variety of surgical strategies possible. We sought to compare the outcome of patients with a single ventricle approach to those with a biventricular repair and to compare outcome of patients with coronary abnormalities to those with normal coronary arteries.

Methods: A retrospective review of our surgical database revealed 67 patients with pulmonary atresia with intact ventricular septum operated on between 1981 and 1998.

Mechanics of the single left ventricle: a study in ventricular-ventricular interaction II.

Background: Left ventricular (LV) effects on right ventricular (RV) function are well known. Less is understood about the effect of the RV on systemic LV mechanics. To determine this interaction, we compared systemic LVs with and without an RV mechanically coupled to them.

Can echocardiography predict the presence or absence of endocardial fibroelastosis in infants <1 year of age with left ventricular outflow obstruction?

This study assesses the use of echocardiography in detecting endocardial fibroelastosis in 32 infants with obstructive left-sided lesions. The data demonstrate that neither endocardial echo-brightness nor geometric variables of the left ventricle allow for accurate prediction of the presence of endocardial fibroelastosis.

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.

Right ventricular function in congenital heart disease: pressure and volume overload lesions.

The right ventricle is often subject to both pressure and volume overload in congenital heart disease. Evaluating right ventricular function in both the native lesion and after surgery in light of these loading conditions, presents a unique challenge for investigators studying these misshapen hearts. The purpose of this article is to briefly delineate what is generally known about right ventricular function in congenital heart disease and to touch on some noninvasive imaging modalities which have helped shed some light on this matter.

The nature of flow in the systemic venous pathway measured by magnetic resonance blood tagging in patients having the Fontan operation.

Objectives: Our objectives were twofold: (1) to determine cardiac and respiratory dependency of systemic venous pathway flow of patients having the Fontan operation with a total cavopulmonary connection and (2) to describe the velocity profile. Systemic venous pathway flow is hypothesized to be mostly respiratory dependent, to be laminar, and to have a smooth velocity profile.

Methods: Twenty-two patients having the Fontan operation (aged 8.

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.