Transseptal left heart catheterization for a patient with a prosthetic mitral valve using live three-dimensional transesophageal echocardiography.

The images and videos presented in this article illustrate a diagnostic transseptal left heart catheterization for a patient with a prosthetic mitral valve guided by live three-dimensional (3D) transesophageal echocardiography. This method provided high-quality 3D imaging that was useful in guiding transseptal puncture and demonstrating prosthetic valve function during this evaluation.

Experience with use of multiplane transesophageal echocardiography to guide closure of atrial septal defects using the amplatzer device.

By providing unlimited imaging planes, multiplane transesophageal echocardiography (MTEE) should improve real-time guidance of interventional procedures. The potential advantages of MTEE in this scenario have not been systematically evaluated. We retrospectively reviewed our experience with MTEE-guided Amplatzer device closure of atrial septal defects (ASDs) MTEE angles used to obtain images for guiding all measurements and maneuvers were recorded. These angles were compared to the range of MTEE angles that are postulated to be available from biplane TEE. Images obtained using MTEE angles from 21 degrees to 70 degrees and from 111 degrees to l59 degrees were defined as only obtainable by MTEE. The MTEE probe was successfully introduced in all (89) patients. Thirteen patients (15%) had multiple defects. Ninety-five devices (5-32 mm in diameter) were deployed. In 66% of patients, balloon sizing and device deployment necessitated imaging planes that are only obtainable by MTEE. All devices were well positioned, with no impingement on inflows or outflows. At follow-up, 79 of 89 (88.7%) patients had no residual ASDs. Each of the remaining 10 patients (11.3%) had a small (<3 mm) residual defect. MTEE played an important role in guiding device closure of ASD, particularly during the phases of balloon sizing and device deployment.

Noninvasive diagnosis of partial anomalous left pulmonary artery.

The branching patterns of the pulmonary arteries may vary, with one or more lobes of the left lung being supplied by a branch originating from the right pulmonary artery-a condition termed partial anomalous left pulmonary artery. If this anomalous branch courses anterior and inferior to the trachea, it is unlikely to result in airway obstruction. It is important that this anomaly not be confused with pulmonary artery sling, where the anomalous branch courses posterior to the trachea, encircling it almost completely. Partial anomalous left pulmonary artery has previously been diagnosed with angiography or by direct visualization during surgery. We present the first cases of noninvasive diagnosis of this anomaly with the use of echocardiography and magnetic resonance imaging. The complementary roles of these two modalities in detecting the anomaly and defining the spatial relationship of the anomalous pulmonary artery to the trachea are emphasized.

Association of viral genome with graft loss in children after cardiac transplantation.

BACKGROUND: The survival of recipients of cardiac allografts is limited by rejection, lymphoproliferative disease, and coronary vasculopathy. The purpose of this study in children who had received heart transplants was to evaluate the cardiac allografts for myocardial viral infections and to determine whether the presence of viral genome in the myocardium correlates with rejection, coronary vasculopathy, or graft loss. METHODS: We enrolled heart-transplant recipients 1 day to 18 years old who were undergoing evaluation for possible rejection and coronary vasculopathy. Endomyocardial-biopsy specimens were evaluated for evidence of rejection with the use of standard criteria and were analyzed for the presence of virus by the polymerase chain reaction (PCR). RESULTS: PCR analyses were performed on 553 consecutive biopsy samples from 149 transplant recipients. Viral genome was amplified from 48 samples (8.7 percent) from 34 patients (23 percent); adenovirus was found in 30 samples, enterovirus in 9 samples, parvovirus in 5 samples, cytomegalovirus in 2 samples, herpes simplex virus in 1 sample, and Epstein-Barr virus in 1 sample. In 29 of the 34 patients with positive results on PCR (85 percent), an adverse cardiac event occurred within three months after the positive biopsy, and 9 of the 34 patients had graft loss due to coronary vasculopathy, chronic graft failure, or acute rejection. In 39 of the 115 patients with negative results on PCR (34 percent), an adverse cardiac event occurred within three months of the negative PCR finding; graft loss did not occur in any of the patients in this group. The odds of graft loss were 6.5 times as great among those with positive results on PCR (P=0.006). The detection of adenovirus was associated with considerably reduced graft survival (P=0.002). CONCLUSIONS: Identification of viral genome, particularly adenovirus, in the myocardium of pediatric transplant recipients is predictive of adverse clinical events, including coronary vasculopathy and graft loss.

Functional pulmonary atresia in neonatal Marfan's syndrome: successful treatment with inhaled nitric oxide.

Functional pulmonary atresia is characterized by a structurally normal pulmonary valve not opening during right ventricular ejection. We report this rare condition in a premature newborn of a twin pregnancy, in which fetal echocardiography findings were consistent with critical pulmonary stenosis. After birth, features of neonatal Marfan's syndrome were noted. Echocardiography showed a morphologically normal but immobile pulmonary valve with continuous regurgitation. Right ventricular pressure was subsystemic. In this case, initial treatment with nitric oxide, followed by pharmacological duct closure, was successful. Differentiating between anatomic and functional pulmonary valve atresia may be difficult. The echocardiographic criteria are discussed.

Posttransplant recoarctation of the aorta: a twelve year experience.

OBJECTIVES: This study was undertaken to investigate the incidence of posttransplant recoarctation of the aorta, delineate the mode of presentation, identify risk factors that predict recoarctation and examine the results of intervention for posttransplant recoarctation. BACKGROUND: Patients with aortic arch hypoplasia require extended arch reconstruction at transplant, with an inherent possibility of subsequent recoarctation of the aorta. METHODS: This was a retrospective review of all children (age <18 years) who underwent cardiac transplantation over a 10-year period. Collected data included pretransplant diagnosis, details of the transplant procedure and posttransplant data including development of recoarctation of the aorta, interventions for recoarctation and the most recent follow-up assessment of the aortic arch. RESULTS: Two hundred eighty-eight transplants were performed on 279 children (follow-up = 1,075 patient-years; range 0 to 133 months, median 43.7). Thirty-two of 152 patients (21%) who underwent extended aortic arch reconstruction subsequently developed recoarctation. All but one patient developed recoarctation within 2 years after transplant; 87% were hypertensive at presentation. Of 30 patients who underwent intervention for recoarctation (balloon angioplasty [n = 26] and surgical repair of recoarctation [n = 4]), 26 (87%) have remained recurrence-free (follow-up = 133 patient-years; range 8 to 106 months, median 47). CONCLUSIONS: The high frequency of recoarctation after cardiac transplantation with extended aortic arch reconstruction mandates serial echocardiographic evaluation of the aortic arch. Patients typically present with systemic hypertension within the first two years after transplantation. Balloon angioplasty is a safe, effective and durable method of treatment.

Dobutamine stress echocardiography for assessing coronary artery disease after transplantation in children.

OBJECTIVES: The purpose of this study was to determine the feasibility, safety and diagnostic accuracy of dobutamine stress echocardiography (DSE) for evaluating posttransplant coronary artery disease (TxCAD) in children, and to determine the frequency of selected cardiac events after normal or abnormal DSE. BACKGROUND: Posttransplant coronary artery disease is the most common cause of graft loss (late death or retransplantation) after cardiac transplantation (CTx) in children. Coronary angiography, routinely performed to screen for TxCAD, is an invasive procedure with limited sensitivity. The efficacy of DSE for detecting atherosclerotic coronary artery disease is established, but is unknown in children after CTx. METHODS: Of the 78 children (median age 5.7 years, range 3 to 18) entered into the study, 72 (92%) underwent diagnostic DSE by means of a standard protocol, 4.6 +/- 1.9 years after CTx. The results of coronary angiography performed in 70 patients were compared with DSE findings. After DSE, subjects were monitored for TxCAD-related cardiac events, including death, retransplantation and new angiographic diagnosis of TxCAD. RESULTS: No major complications occurred. Minor complications, most often hypertension, occurred in 11% of the 72 subjects. The sensitivity and specificity of DSE were 72% and 80%, respectively, when compared with coronary angiography. At follow-up (21 +/- 8 months), TxCAD-related cardiac events occurred in 2 of 50 children (4%) with negative DSE, versus 6 of 22 children (27%) with positive DSE (p < 0.01). CONCLUSIONS: DSE is a feasible, safe and accurate screening method for TxCAD in children. Positive DSE identifies patients at increased risk of TxCAD-related cardiac events. Negative DSE predicts short-term freedom from such events.

Echocardiographic spectrum of supracardiac total anomalous pulmonary venous connection.

Although infracardiac total anomalous pulmonary venous connection (TAPVC) is almost always associated with venous obstruction, the incidence and location of obstruction in supracardiac TAPVC has not been completely delineated. This report summarizes our experience with 20 cases of supracardiac TAPVC diagnosed by transthoracic echocardiography from Jan. 1989 to Mar. 1997. Fifty percent were obstructed, and five different sites of narrowing were found. The most common sites of obstruction were at the level of the left pulmonary artery (left vertical vein) and at the insertion into the superior vena cava (right vertical vein). Because nonobstructed Doppler flow patterns are present proximal to the actual site of obstruction in the anomalous pathway, a thorough interrogation of the entire venous channel with two-dimensional and Doppler echocardiography is essential to provide complete preoperative anatomic and hemodynamic details to determine the nature and timing of surgery in this condition.

The use of larger size pulmonary homografts for the Ross operation in children.

Nine pediatric patients received an adult size cryopreserved pulmonary homograft for right ventricular outflow tract reconstruction as part of the Ross procedure. The early postoperative results are excellent. It is suggested that a full, adult size pulmonary homograft should be used in the future in pediatric patients undergoing the Ross procedure.

Ventricular remodeling following infant-pediatric cardiac transplantation. Does age at transplantation or size disparity matter?

Early left ventricular (LV) remodeling following pediatric cardiac transplantation has not been described. To identify patterns and determinants of change in left ventricular mass and volume posttransplant, we studied 125 consecutive children who underwent cardiac transplantation between January 1, 1989 and July 31, 1993. Two-dimensional imaging-directed M-mode echocardiograms were studied weekly until 26 weeks post-transplant. LV mass and volume (indexed to BSA1.5) were measured. LV mass index increased until 3 weeks post-transplant, and then decreased. The mean decrement in LV mass index after 8 weeks post-transplant (relative to baseline) was significantly larger in patients with donor-recipient weight ratio > 1.5 compared with patients with donor-recipient weight ratio < or = 1.5 (-2.2 g/m3 compared with 33.4 g/m3, respectively, P < 0.01). Multiple linear regression was performed employing donor-recipient weight ratio, time since transplantation, ischemic time, and age at transplant as prognostic variables. Donor-recipient weight ratio (P < 0.0001), time since transplant (P < 0.01), and age at transplant (P = 0.02) were identified as independent predictors of change in LV mass index. Donor-recipient weight ratio (P = 0.001) and time since transplantation (P = 0.02) were independent predictors of change in LV volume index. There was an interaction between donor-recipient weight ratio and time since transplantation, suggesting that donor-recipient weight ratio has an independent effect as well as a time-dependent effect on change in LV mass and volume indices. LV mass and volume indices increased early posttransplant and then decreased; this pattern was temporally predictable, and dependent on donor-recipient weight ratio and age at transplant.

Quantitation of echocardiographic predictors of outcome in infants with isolated ventricular septal defect.

Clinical and morphometric features such as ventricular septal defect (VSD) size and location may determine outcome in infants with an isolated VSD. However, no currently available data allow quantitative estimation of the probability of spontaneous closure or surgery in individual patients. To identify independent predictors of outcome and to quantitate the probability of spontaneous closure and surgery in patients with isolated VSD, we studied 156 consecutive infants who had a diagnosis of an isolated VSD between January 1, 1988, and December 31, 1990, and who were subsequently monitored for 28.5 +/- 15 months. Of the 149 patients with membranous (n = 100) and muscular (n = 49) defects who were studied, 46 (31%) patients had spontaneous closure, and an additional 37 (25%) patients underwent surgical repair. Univariate analysis identified defect cross-sectional area indexed to body surface area, location in the muscular septum, presence of Down syndrome, and in membranous defects the presence of aneurysmal tissue as potential predictors of spontaneous closure or surgery. Multiple logistic regression analysis with these candidate variables identified indexed defect cross-sectional area as an independent predictor of spontaneous closure and surgery (p < 0.001). An inverse nonlinear relationship was seen between indexed VSD area and the probability of spontaneous closure (probability = (1 + e[-1.74 + 4.57CSA])-1 and a positive nonlinear relationship between indexed VSD area and the probability of surgery (probability = (1 + e[3.39 - 2.31CSA])-1). Muscular defects were more likely to close spontaneously than membranous defects (odds ratio 2.6, 95% CL = 1.01 - 6.8, p = 0.04).(ABSTRACT TRUNCATED AT 250 WORDS)

Maternal-neonatal serum vitamin A concentrations.

Prevention of neonatal vitamin A deficiency is related to the adequacy of maternal vitamin A stores. In this study we investigated maternal and cord serum vitamin A and retinol-binding protein (RBP) values in an Indian population including, for the first time, clinically vitamin A-deficient mothers. Twenty-eight maternal-neonatal pairs were selected from maternal cohorts of high socioeconomic status without clinical evidence of vitamin A deficiency (group I) and low socioeconomic status with conjunctival xerosis and Bitot's spots (group II). Maternal education, caloric and vitamin A intakes, weight, height, hemoglobin, and birth weight were significantly lower in group II. Serum vitamin A levels were significantly higher in group I mothers and newborns as were RBP levels in group I mothers. However, a significant difference between groups I and II in cord blood RBP was not observed. Upon correlation of maternal vitamin A levels with cord blood vitamin A levels, a logarithmic relationship was revealed, suggesting saturable transplacental transport of vitamin A.