Cor triatriatum sinister in an adult: management guided by real time three-dimensional transesophageal echocardiography and stress echocardiography.

A 39-year-old female had cor triatriatum (CT) detected as an incidental finding on transthoracic echocardiography performed to evaluate chest pain. By conventional two- and real time three-dimensional transesophageal echocardiography, the CT membrane had a communicating orifice connecting the accessory and main left atrial chambers that measured 1.3 × 0.8 cm. The resting mean transmembrane gradient was 2 mm Hg. The postexercise mean transmembrane gradient and pulmonary artery pressure were 6 and 40 mm Hg. Extrapolating from cutoff values for postexercise gradients and pulmonary pressures in patients with mitral stenosis, we advised deferring surgery and close clinical and echocardiographic follow up.

Usefulness of tissue Doppler echocardiography for evaluating ventricular function in children without heart disease.

Tissue Doppler echocardiographic imaging (TDI) is a novel method for accurately evaluating ventricular function. Currently, scant data are available on the distribution of tissue Doppler indexes in healthy children in the age range of 1 to 18 years. The aims of this study were to assess the distribution of tissue Doppler indexes of systolic and diastolic ventricular function in healthy children, to assess the influence of age on these indexes, and to compare them with conventional Doppler indexes. A total of 151 consecutive children aged 1 to 18 years were enrolled in the study. Nine different (7 diastolic and 2 systolic) TDI parameters were assessed. Peak velocities of systolic and diastolic excursions of the mitral and tricuspid annuli were obtained from the apical 4-chamber view. Mean velocities of early diastolic recoil of mitral and tricuspid annuli were measured from the apical 4-chamber view. The mean velocity of early diastolic relaxation of the left ventricular posterior wall was measured in the parasternal long-axis view. Results showed a statistically significant difference in some of the TDI indexes among the different pediatric age groups. Most of the TDI indexes showed a very weak correlation with age. There was no significant correlation (p >0.05) between peak velocities of the early diastolic mitral inflow Doppler pattern (E wave) and the corresponding TDI index. In contrast, there was a significant (p <0.001) correlation between the corresponding Doppler indexes in the tricuspid annulus. We conclude that TDI is a valuable tool for assessing ventricular function, particularly diastolic function. Establishment of normative data for TDI in the pediatric age group should broaden the clinical applicability of this useful modality for assessing ventricular function.

Nonimmune hydrops fetalis in the liveborn: series of 32 autopsies.

Nonimmune hydrops fetalis (NIHF) or generalized soft tissue edema and cavity effusions may be due to cardiovascular diseases, congenital infections, genitourinary malformations, thoracic masses, placental conditions, chromosomal abnormalities, and idiopathic. We report 32 cases of NIHF from among 429 neonates who underwent autopsies (incidence 7.45%). Sixteen cases (50%) had cardiovascular disease; all were due to low output cardiac failure; 7 had structural congenital heart disease. Three of the children with congenital heart disease also had chromosomal abnormalities: 2 had trisomy 18 and 1 had Noonan syndrome. Among myocardial conditions were five subjects with cardiomyopathies (1 of each of the following types): oncocytic, dilated, endocardial fibroelastosis, cardiac glycogenosis, and carnitine deficiency; 3 had myocarditis, and 1 had cardiac rhabdomyomas. Congenital infections were due to cytomegalovirus in 3 cases, bacteria in 2, and parvovirus in 1. The mechanism of NIHF in these cases might be a combination of decreased myocardial contractility due to myocarditis and fetal anemia. Genitourinary diseases were present in 5 newborns: Two had congenital nephrotic syndrome, 1 had VACTER association, 1 had prune-belly syndrome, and 1 had urogenital sinus malformation. Intrathoracic lesions were found in 2 babies (pulmonary sequestration and diaphragmatic hernia). One twin died of volume overload due to twin transfusion syndrome. Only 2 newborns were classified as idiopathic. Our study shows that cardiovascular diseases that lead to heart failure or impaired venous return are more common in the liveborn (50%), whereas congenital infections are more common in the stillborn with NIHF.

Value of autopsy in nonimmune hydrops fetalis: series of 51 stillborn fetuses.

Nonimmune hydrops fetalis (NIHF) is used to describe fetuses and newborns with generalized edema and cavity effusions. It is helpful to alert physicians about the presence of anemia, heart failure, and/or hypoproteinemia, but this diagnosis is frequently overlooked. We reviewed the autopsy files from 1990 to 2000, selected all cases with NIHF including clinical information (with maternal laboratory tests and ultrasound), and classified patients by etiology. Among 840 stillborn autopsies during the 11-year period, we found 51 with NIHF (6.07%). The clinical summary had mentioned hydrops in 14 patients and the etiology in another 7 by fetal ultrasonography, but without addressing the possibility of hydrops. In the remaining 30 cases neither hydrops nor an etiology was mentioned. Other pertinent diagnoses were maternal diabetes mellitus (4), congenital heart disease (3), and cystic hygroma (2). The following diagnoses were made in one instance each: cardiac tumor, twin transfusion syndrome, congenital adenomatoid malformation, syphilis, Turner syndrome, and cerebral arteriovenous malformation. Postmortem and placental examination confirmed the following etiologies: congenital infections (17); placental pathology significant enough to explain NIHF (10); cardiovascular diseases (8) (further classified as congenital heart disease [3], rhabdomyoma [1], and vascular malformations [4]); chromosomal abnormalities (6); uncontrolled maternal diabetes (4); intrathoracic lesions (2); prune-belly syndrome (2); and idiopathic NIHF (2). Only 3.9% of the cases studied had no identifiable etiology. The cause of hydrops was confirmed by autopsy in 47 fetuses (92%), which further supports the importance of performing an autopsy. Thirty-two cases (62.74%) had placental abnormalities helpful to the etiology (parvovirus, syphilis, Turner's syndrome, etc.). In 20 instances, the clinical summary had no mention of either hydrops or any of the diseases leading to it. The autopsy in conjunction with placental examination and fetal ultrasound represent the best combination to determine the etiology of NIHF among stillborn fetuses.