Usefulness of echocardiographic evidence of pericardial effusion and mitral regurgitation during the acute stage in predicting development of coronary arterial aneurysms in the late stage of Kawasaki disease.

In 28 patients with Kawasaki disease, the relation of specific echocardiographic findings identified during the acute study of the illness, including valvular regurgitation, to development of coronary aneurysms was evaluated. Initial studies were performed at the time of clinical presentation, 5 to 10 days after the onset of fever, and follow-up studies were performed 1 to 2 months later. Patients in whom coronary aneurysms developed were more likely to have pericardial effusion (p = 0.0006) or mitral regurgitation (MR) (p = 0.014) at initial echocardiographic study than those without aneurysms. Presence of either mitral regurgitation (MR) or pericardial effusion had a positive predictive value of 0.84 for aneurysm development. Twenty-three percent of patients had MR, and it was associated with mild LV dilatation (35 +/- 3 vs 32 +/- 5 mm, p less than 0.05). Insufficiency of other valves was rare. Thus, MR and pericardial effusion on acute phase echocardiographic examination may predict development of coronary aneurysms in Kawasaki disease. Mild MR occurs frequently in acute Kawasaki disease and is associated with mild LV dilation.

Double-horned or caplike right ventricle: diagnosis and operative treatment.

BACKGROUND: Three patients reported here and 4 from the literature serve as background for the state-of-art diagnostic and operative considerations for an unusual congenital cardiac malformation: double-horned or caplike right ventricle. METHODS: This is a retrospective analysis of cardiac catheterization, cineangiography, and two-dimensional echocardiography findings, as well as palliative and corrective operations in 3 previously unreported patients. Four patients from the literature are reviewed. RESULTS: Characteristic morphologic features recognizable by invasive and noninvasive imaging distinguish double-horned right ventricle from complex malformations such as criss-cross hearts, superior-inferior ventricles, and univentricular hearts with a small outflow chamber. CONCLUSION: Double-horned or caplike right ventricle is a congenital malformation characterized by an unusual ventricular morphology, which may be the result of incomplete development of the right ventricle. The two-horned appearance may be secondary to an absence of the apical trabeculated compartment, with the left ventricle wedged between the two horns. It is invariably associated with double right ventricular outlet. Surgical experience so far suggest that most patients with typical double-horned right ventricle should be considered for anatomic surgical correction.

Echocardiographic evaluation of the aortic root and mitral valve in children and adolescents with isolated pectus excavatum: comparison with Marfan patients.

Pectus excavatum, mitral valve prolapse (MVP), and dilated aortic root occur frequently in patients with Marfan's syndrome (MS). Patients with isolated pectus excavatum (IPE) have a high prevalence of MVP, but it is not known whether aortic root dilatation is a risk in those patients. To test the hypothesis that IPE and MS represent a spectrum of connective tissue dystrophy with MV and aortic root involvement, two-dimensional (2D) echocardiography was used to measure the aortic root diameter and assess for MVP in IPE (n = 31), MS (n = 14), and normal (n = 16) gender- and age-matched patients. Aortic root was measured in parasternal long- and short-axis views, just above the aortic sinuses, at end systole, in six cardiac cycles, and averaged. Parasternal long-axis view was used to assess for MVP. Aortic root diameter in IPE patients was not different from that in normal subjects, 24 +/- 4 mm vs 22 +/- 4 mm (p = NS), respectively, both were significantly smaller than that in MS patients (30 +/- 5 mm; p less than 0.05). MVP was present in 17 of 31 (55%) IPE patients vs 12 of 14 (86%) MS patients (p = NS) and in only 1 of 16 (6%) normal subjects (p less than 0.05) vs both IPE and MS. We conclude that young patients with IPE represent an isolated form of connective tissue abnormality because of the presence of pectus excavatum and MVP, but this is different from the systemic involvement of MS because of the lack of other systemic findings, including aortic root dilatation and changes in body habitus.

Transmission of full-length echocardiographic images over ISDN for diagnosing congenital heart disease.

OBJECTIVES: To study the feasibility of transmitting full-length diagnostic-quality echocardiograms with video conferencing systems linked by ISDN. METHODS: Forty-three previously video-recorded full-length echocardiograms (22 complete two-dimensional Doppler studies, 21 separate M-mode studies) were transmitted from a site 30 miles distant using desktop video conferencing systems linked with one ISDN line (128 kbps). Blinded interpretations of the original recorded and transmitted studies were made by two pediatric cardiologists (two-dimensional Doppler) and a pediatric sonographer (M-mode). RESULTS: Diagnoses of normal (N = 12) and abnormal (N = 10) conditions were made from the original tapes; the same interpretations were made from the transmitted studies. Twenty-one specific abnormalities in the 10 abnormal studies were seen on both original and transmitted videotapes. These abnormalities ranged from simple congenital heart defects, including ventricular septal defect, atrial septal defect, and patent ductus arteriosus, to complex ones, such as coronary artery fistula, double-outlet right ventricle, and complete atrioventricular canal. Qualitatively, there was mild degradation in gray scale, brightness, and contrast of the images. Six M-mode variables from the transmitted images had excellent concordance with the original tracings (P = 0.506 to 0.838; r = 0.86 to 0.97). CONCLUSIONS: Although our sample size was small, this preliminary experience indicates that video conferencing equipment utilizing ISDN technology is a reliable method for transmitting full-length diagnostic-quality echocardiographic studies from remote sites. This technology may have a significant impact on the management of pediatric patients with suspected heart disease. Further evaluation is warranted.

Effect of body size, ponderosity, and blood pressure on left ventricular growth in children and young adults in the Bogalusa Heart Study.

BACKGROUND: The measurement of left ventricular mass (LVM) is important because individuals with increased LVM are at increased risk for cardiovascular diseases, including myocardial infarction and congestive heart failure. There are limited longitudinal data on the acquisition of LVM in children and young adults and the relative importance of sex, growth, excess body weight, and blood pressure (BP) on change in LVM. METHODS AND RESULTS: The study cohort consisted of a cross section of 160 healthy children and young adults 9 to 22 years of age at first exam in the biracial community of Bogalusa, La. All had stable BP levels recorded over a 2- to 3-year period. Repeated examinations were performed 4 to 5 years apart. At each exam, 6 BPs were obtained with a mercury sphygmomanometer by trained examiners. The mean of the observations was used, with the fourth Korotkoff phase serving as the measure of diastolic BP. Anthropometric data, including height (HT), weight (WT), and triceps skin fold thickness (TSF), were also obtained, and M-mode echocardiograms were performed. Ponderal index (PI = WT/HT3) was used as a measure of weight-for-height. Tracking of HT (r = .68 to .76), WT (r = .73 to .82), PI (r = .77 to .89), TSF (r = .70 to .80), BP (r = .47 to .60), and LVM (r = .40 to .70) was strong in both sexes (P < .0001). LVM indexed for linear growth (LVM/HT2.7) tracked in females (r = .56, P < .0001) but not in males. In univariate cross-sectional analyses, LVM/HT2.7 correlated with WT, PI, and TSF in both sexes (r = .21 to .60, P < .05) and with systolic BP (SBP) in females (r = .23, P < .05). WT was the only independent correlate of LVM/HT2.7 in both sexes in multivariate cross-sectional analysis in a model containing age, SBP, WT, and TSF as independent variables (r2 = .08 to .28, P < .02). In longitudinal univariate analyses, initial measurements of WT, PI, and TSF predicted final LVM/HT2.7 in both sexes (r = .28 to .56, P < .01), and SBP was significant for females (r = .27, P < .05). In multivariate analyses, initial WT was associated with final LVM and LVM/HT2.7 in both sexes (r2 = .27 to .54, P < .01). Finally, baseline LVM correlated with final SBP in both sexes (r = .21 to .27, P < .05), and initial LVM/HT2.7 correlated with final SBP in females (r = .26, P < .05) with a trend for males (r = .17). CONCLUSIONS: These data indicate that linear growth is the major determinant of cardiac growth in children and that excess weight may lead to the acquisition of LVM beyond that expected from normal growth. Increased mass may also precede the development of increased BP. The development of obesity may therefore be a significant, and possibly modifiable, risk factor for developing left ventricular hypertrophy and hypertension, risk factors for cardiovascular morbidity and mortality.

Echocardiographic prediction of neonatal ECMO outcome.

Cardiopulmonary physiology was assessed by Doppler echocardiography in neonates undergoing pre-ECMO evaluation for meconium aspiration syndrome, congenital diaphragmatic hernia, persistent fetal circulation, and sepsis, from March 1987 through July 1992 (n = 136). Percent survival by diagnosis was: meconium aspiration syndrome, 86%; persistent fetal circulation, 68%; congenital diaphragmatic hernia, 63%; sepsis, 33%. Survival odds by diagnosis predicted a better outcome for meconium aspiration syndrome than for congenital diaphragmatic hernia and sepsis, and a better outcome for persistent fetal circulation than for sepsis. Percent survival for right-to-left patent ductus arteriosus flow (PDA) was 56%; other patent ductus arteriosus flow was 84%. In multivariate analysis, percent survival in congenital diaphragmatic hernia and persistent fetal circulation patients with right-to-left PDA flow suggested a worse outcome (% survival right-to-left vs other: congenital diaphragmatic hernia, 13% vs 70%; persistent fetal circulation, 25% vs 85%), whereas percent survival did not appear to suggest the same in meconium aspiration syndrome or sepsis patients. Similar analysis in non-ECMO patients suggested a worse outcome with right-to-left PDA flow in patients with meconium aspiration syndrome and congenital diaphragmatic hernia. Right-to-left PDA flow, sepsis, and congenital diaphragmatic hernia were associated with a poorer ECMO outcome. Initial assessment of PDA flow helps predict ECMO outcome.