Correlations Between Echocardiographic Systolic and Diastolic Function with Cardiac Catheterization in Biventricular Congenital Heart Patients.

Newer echocardiographic techniques may allow for more accurate assessment of left ventricular (LV) function. Adult studies have correlated these echocardiographic measurements with invasive data, but minimal data exist in the pediatric congenital heart population. Purpose of this study was to evaluate which echocardiographic measurements correlated best with LV systolic and diastolic catheterization parameters. Patients with two-ventricle physiology who underwent simultaneous echocardiogram and cardiac catheterization were included. Images were obtained in the four-chamber view. LV systolic echocardiographic data included ejection fraction, displacement, tissue Doppler imaging (TDI) s' wave, global longitudinal strain, and strain rate (SR) s' wave. Diastolic echocardiographic data included mitral E and A waves, TDI e' and a' waves, and SRe' and SRa' waves. E/TDI e', TDI e'/TDI a', E/SRe', and SRe'/SRa' ratios were also calculated. Catheterization dP/dt was used as a marker for systolic function, and LV end-diastolic pressure (EDP) was used as a marker for diastolic function. Correlations of the echocardiographic and catheterization values were performed using Pearson correlation. Twenty-nine patients were included (14 females, 15 males). Median age at catheterization was 3.4 years (0.04-17.4 years). dP/dt was 1258 ± 353 mmHg/s, and LVEDP was 10.8 ± 2.4 mmHg. There were no significant correlations between catheterization dP/dt and systolic echocardiographic parameters. LVEDP correlated significantly with SRe' (r = -0.4, p = 0.03), SRa' (r = -0.4, p = 0.03), and E/SRe' (r = 0.5, p = 0.004). In pediatric congenital heart patients, catheterization dP/dt did not correlate with echocardiographic measurements of LV systolic function. Further studies are needed to determine which echocardiographic parameter best describes LV systolic function in this population. Strain rate analysis significantly correlated with LVEDP. Strain rate analysis should be considered as an alternative method to estimate LVEDP in this patient population.

Postnatal atresia of extraparenchymal pulmonary veins, fulminant necrotizing pulmonary arteritis and elevated circulating immune complexes.

An infant with operatively corrected total anomalous pulmonary venous connection developed postnatal atresia of the extraparenchymal left pulmonary veins with secondary arteritis of the ipsilateral intraparenchymal pulmonary arteries. Atresia of the right or left main pulmonary veins or of the common pulmonary vein is a rare occurrence and it is believed that association of such with necrotizing pulmonary arteritis has never been reported. This case illustrates the potential consequences of severe pulmonary venous obstruction in the absence of a left to right shunt.

Atrioventricular valve abnormalities in infancy: two-dimensional echocardiographic and angiocardiographic comparison.

The results of two-dimensional echocardiography and biplane angiocardiography from 47 infants with congenital atrioventricular (AV) valve abnormalities were compared. Eleven patients had atresia of the right AV valve, 10 had atresia of the left AV valve, 4 had hypoplasia of the right AV valve and 5 had hypoplasia of the left AV valve. Twelve patients had endocardial cushion defect, three had single ventricle and two had straddling of the left AV valve. There was agreement between the two techniques as to the number of AV valves present in each patient. The echocardiographic estimate of valve anular diameter was below normal in seven of the eight patients thought to have a hypoplastic anulus by angiocardiography. In 10 of the 12 patients with endocardial cushion defect, there was agreement between the two techniques as to the presence or absence of atrial and ventricular septal defect. The chordal attachments of straddling valves were better visualized by echocardiography; flow patterns and effective orifice size were better demonstrated by angiocardiography. The subcostal four chamber echocardiographic views and cranially angulated oblique angiocardiographic views were comparable and provided the best images for determination of the size and number of AV valves and their relation to the atrial and ventricular septa.

Children with heart murmurs: can ventricular septal defect be diagnosed reliably without an echocardiogram?

OBJECTIVES: This study was undertaken to determine the accuracy of expert examination for ventricular septal defect (VSD) among children with a heart murmur. BACKGROUND: Because the frequency and nature of errors that might be made by reliance solely on expert examination for diagnosis of VSD are speculative, the role of echocardiography in such diagnosis is controversial. METHODS: Two hundred eighty-seven consecutive previously unevaluated pediatric subjects were enrolled in the study. For each child, the pediatric cardiologists prospectively recorded a working diagnosis and their level of confidence in the diagnosis, categorizing any VSD diagnosed as small or moderate to large. After echocardiography, VSDs were subcategorized by location and requirement for treatment as minor, intermediate or major. Receiver-operating characteristic (ROC) curves described the accuracy of the clinical examination. RESULTS: Seventy-three subjects had a VSD (minor in 52, intermediate in 10 and major in 11). ROC areas (1.0 = perfect discrimination, 0.5 = indiscriminate) were minor VSD 0.92 +/- 0.02 and major/intermediate VSD 0.69 +/- 0.07 (p = 0.0016). Four of 52 minor VSDs were not identified at any level of suspicion; the clinical diagnoses were moderate to large VSD in two patients and atrial septal defect and unlimited differential diagnosis in one patient each. Fourteen of 235 patients without a minor VSD were believed with confidence to have a small VSD, but the final diagnosis was intermediate VSD in 4, innocent murmur in 3, major VSD in 2, pulmonary stenosis in 2 and subaortic membrane, atrial septal defect and mitral regurgitation in 1 patient each. CONCLUSIONS: Almost all minor VSDs are recognized without echocardiography; however, errors can occur even when an expert examiner is confident. Clinical recognition of an intermediate or major VSD is less accurate than clinical recognition of a minor VSD. Failure to distinguish VSDs of major or intermediate importance from minor VSDs is a weakness of the expert clinical examination.

Echocardiographic pulsed Doppler features of absent pulmonary valve syndrome in the neonate.

M mode echocardiography was used to evaluate nine neonates with absent pulmonary valve syndrome. Six were also studied with two dimensional echocardiography and two with pulsed Doppler echocardiography. M mode echocardiography demonstrated a large, overriding great artery and right ventricular dilation in all nine patients and abnormal septal motion in eight. Two dimensional echocardiography demonstrated aneurysmal dilation of the main pulmonary artery in all six patients studied. Pulsed Doppler echocardiography in the two infants studied demonstrated anterograde systolic and retrograde diastolic flow in the main pulmonary artery and right ventricular outflow tract. The echocardiographic features of absent pulmonary valve syndrome appear to be unique and allow the diagnosis to be made noninvasively, thus obviating or delaying the need for potentially high risk cardiac catheterization.

Ventricular septal defect in infancy: detection with two dimensional echocardiography.

To determine the sensitivity and specificity of two dimensional echocardiography in detecting ventricular septal defect two dimensional echocardiograms were performed on 53 infants under 1 year of age. The diagnosis of ventricular septal defect was confirmed by cardiac catheterization in 27 patients; an intact ventricular septum was confirmed by catheterization in 18 and clinically in 8. Using a 35 degree mechanical sector scanner with a 3.5 megahertz transducer, we imaged the ventricular septum in the long axis and in a four chamber view (apical or subcostal, or both). Images were recorded on videotape and reviewed independently by two observers unaware of the diagnosis. Interobserver agreement was 94 percent. Among the 27 patients with a ventricular septal defect, the lesion was correctly identified in 20 (74 percent) and was undetected in 7 (3 of whom had a defect less than 4 mm in diameter, as determined by angiography). No defect less than 4 mm in diameter was detected. Among the 26 patients with an intact septum, a defect was correctly excluded in 23 (88 percent); a false positive diagnosis was made in 3. The apical and subcostal views demonstrated the greatest number of defects (20 of 20), but also gave the highest number of false positive diagnoses (3 of 3). The long axis view was helpful when positive, but showed only 9 of 20 of the defects. In this study, two dimensional echocardiography detected approximately three fourths of ventricular septal defects large enough to warrant cardiac catheterization in the 1st year of life. False positive diagnoses were related to dropout of echoes in the membranous septum when imaged in the four chamber views.

Resolution and accuracy in two dimensional echocardiography.

The resolution and accuracy of commercially available two dimensional echocardiographic systems were tested by imaging two types of in vitro test objects. One consisted of a series of fine parallel threads spaced at known intervals and the other was a tissue phantom in which a series of holes of known size had been cut. The echocardiographic systems tested included a mechanical single element sector scanner, a three element rotary sector scanner and a phased array system. Azimuthal resolution, lateral resolution and accuracy of horizontal distance measurements were assessed at depths of 2 and 6 cm. For each system, azimuthal resolution was better than lateral resolution, especially when assessed with use of the parallel threads. When the tissue phantom was imaged, the best resolution (2 mm in azimuthal and lateral directions) was obtained with the highest frequency transducer tested (3.5 MHz). The apparent size of a tissue defect was sensitive to gain settings, especially at a depth of 2 cm with lower frequency transducers; at a depth of 6 cm, echographic measurements of defect size were accurate to within 2.5 mm. Recent applications of two dimensional echocardiography require near the apparent limits of current equipment. It is recommended that the highest frequency transducer and lowest possible gain settings be used in these situations.

Intermediate-term outcome of transcatheter secundum atrial septal defect closure using the Bard Clamshell Septal Umbrella.

Of 31 patients who underwent transcatheter atrial septal defect occlusion with the Bard Clamshell Septal Umbrella, only 1 had a clinically significant residual atrial septal defect at a mean follow-up of 41 months. All patients were asymptomatic despite the occurrence of device arm fractures in 84% of these patients.

Sustained ventricular tachycardia after repair of tetralogy of Fallot: new electrophysiologic findings.

Ventricular arrhythmia originating from the outflow tract of the right ventricle is a presumed cause of late sudden death in patients after repair of tetralogy of Fallot. Exercise testing has been shown to enhance detection, and phenytoin has been shown to control ventricular arrhythmias in these patients. This study reports new findings in 3 patients who underwent electrophysiologic studies at postoperative cardiac catheterization; in each, sustained ventricular tachycardia was induced and found to originate from the inflow-septal area of the right ventricle. Using serial studies, the same sustained ventricular tachycardia was induced during therapeutic serum concentrations of phenytoin but not after propranolol. No patient had ventricular arrhythmia during a 24-hour electrocardiogram or during exercise. Although no patient had normal hemodynamic function, only 1 patient had right ventricular pressure greater than two-thirds systemic pressure. Each patient had undergone initial intracardiac repair at a relatively late age (3, 9, and 9 years).

The management of tetralogy of Fallot with pulmonary atresia and diminutive pulmonary arteries.

Since September 1991, 14 consecutive patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries have undergone staged repair. All patients had multiple aortopulmonary collateral arteries and the ductus arteriosus was absent in 11. Mean sizes of the right and left pulmonary arteries were 2.2 +/- 0.7 mm and 1.9 +/- 0.8 mm, respectively (range 0.5 to 3.0 mm). Eight patients (57%) have subsequently received complete repair. Age at initial procedure (shunt, right ventricle-pulmonary artery conduit, or direct aorta-pulmonary artery anastomosis) in this group was 5.3 +/- 6.8 months. The number of operative procedures to achieve complete repair was 2.9 +/- 0.8 per patient (range 2 to 4). Intraoperative postrepair peak right ventricle-left ventricle pressure ratio was 0.57 +/- 0.17. Six of 8 patients (75%) required additional interventional procedures (mean 1.5 +/- 1.2 per patient) for angioplasty of peripheral pulmonary artery stenoses, coil embolization of aortopulmonary collateral arteries, or intra-operative insertion of intravascular pulmonary artery stents. Mean follow-up from complete repair was 8.7 +/- 8.3 months (range 0.5 to 23.8 months) and is complete. There was one in-hospital death at 45 days, and one late cardiac death at 20.3 months. Six patients had initial palliative operations (unifocalization, right ventricle-pulmonary artery conduit, direct aorta-pulmonary artery anastomosis, or transannular outflow patch) but have not undergone complete repair. Age at initial procedure in this group was 27.9 +/- 56.9 months (range 0.27 to 155 months), and mean follow-up from initial procedure was 10.9 +/- 11.2 months (range 0 to 31.4 months). The operative mortality rate was 33% (2 of 6 patients). There was one late noncardiac death at 5.3 months. Three patients are awaiting further intervention or repair. This experience suggests that complete repair is feasible even in patients with extremely diminutive pulmonary arteries (< or = 3.0 mm). Pulmonary artery growth is facilitated by early (3 to 6 month) establishment of central pulmonary artery flow by right ventricle-pulmonary artery conduit (pulmonary arteries > 1.5 mm) or by direct ascending aorta-pulmonary artery anastomosis (pulmonary arteries < 1.5 mm). Subsequent interventional catheterization and operative procedures as required for pulmonary artery stenoses and coil embolization of collateral arteries allow continued recruitment of central pulmonary arteries and may obviate or minimize the need for unifocalization procedures.

Hemophilus influenzae purulent pericarditis in children: diagnostic and therapeutic considerations.

Purulent pericarditis is an unusual complication of infection in infancy and has been associated with an extremely high mortality rate. Early diagnosis followed by combined antibiotic therapy and surgical drainage of the pericardium has markedly improved survival. Between APril, 1975, and February, 1979, nine patients with purulent pericarditis secondary to Hemophilus influenzae type B were treated at the Oklahoma Children's Memorial Hospital. In every case signs and symptoms of congestive heart failure were present, and a pericardial effusion was demonstrated by echocardiography and confirmed by pericardiocentesis. The organism was identified with countercurrent immunoelectrophoresis and antibiotic sensitivity determined by rapid beta lactamase assay. All patients were treated with a combination of parenteral antibiotics and open surgical drainage of the pericardium. There were no deaths and all patients demonstrated marked improvement following operation. Follow-up echocardiography revealed no evidence of pericardial effusion or signs of constriction in any patient.

Nonoperative management of subcapsular hematomas of the liver.

The liver is the second most common organ injured in blunt abdominal trauma, and with the use of diagnostic techniques such as hepatic scanning, an increasing number of patients with intrahepatic lesions are being identified. Five patients with this type of lesion are presented. A plan of managament, including observation in an intensive care unit, repeated physical examinations, frequent reevaluation of laboratory values, bed rest, gastrointestinal tract decompression and broad spectrum antibiotics, is suggested as the treatment of choice for this type of injury. All patients in the series have done well with no abscess formation, delayed rupture of hemobilia, and all show resolution of the defect on following-up hepatic scanning.

Cardiovascular parameters after acute myocardial infarction and streptokinase administration in patients receiving coronary artery bypass grafts.

In 16 patients requiring coronary artery bypass grafting (10 control and 6 streptokinase patients), we compared the preoperative, operative, and postoperative cardiovascular parameters. Streptokinase patients had an acute myocardial infarction and attempted reperfusion with streptokinase before coronary artery bypass grafting. One patient failed to recannalize with streptokinase and one patient had reocclusion after withdrawal of heparin necessitating coronary artery bypass grafting. Examination of hemodynamic parameters revealed a lower preoperative mean blood pressure and an elevated pulmonary artery wedge pressure in streptokinase patients. The elevated pulmonary artery wedge pressure persisted through the postoperative period of observation. These results indicate that only minor differences exist between control and streptokinase patients. Emergency and elective coronary artery bypass grafting can be safely performed in patients treated with streptokinase for acute myocardial infarction without associated cardiogenic shock.

The effects of anabolic steroids on myocardial structure and cardiovascular fitness.

To determine the effects of anabolic steroids on myocardial structure, VO2max, and body composition, experienced age-matched male weight trainers (M age 26.5 yr) who either used (U) (N = 11) or did not use (NU) (N = 13) anabolic steroids were evaluated. Steroid users were tested while off cycle (U-OFF) for at least 8 wk, again at the peak (U-ON) of their subsequent cycle, and to the nonuser group of weight trainers. Echocardiographic measurements revealed significant differences in left ventricular (LV) mass (182.8 +/- 26.9 g vs 210.6 +/- 42 g; P < 0.05) and interventricular septum thickness (IVS) (10.3 +/- 1.2 mm vs 11.1 +/- 1.2 mm; P < 0.05) between U-OFF and U-ON, respectively. NU measurements were also significantly different than U-ON for LV mass and IVS (186.5 +/- 36.2 g; P < 0.05 and 9.3 +/- 1.2 mm; P < 0.05, respectively). LV diameter in diastole was significantly greater in U-ON (59.1 mm) than in NU (55.7 mm; P < 0.05). In addition, LV posterior wall thickness in diastole was greater in U-ON compared with NU (11.2 mm vs 9.5 mm; P < 0.05). VO2max values for both user groups were significantly lower than those for NU (U-OFF = 41.0 +/- 4.5 ml.kg-1.min-1, U-ON = 41.0 +/- 5.7 ml.kg-1.min-1, and NU = 50.2 +/- 6.4 ml.kg-1.min-1; P < 0.05). Despite these morphological changes within the myocardium, there were no concomitant increases in shortening fraction.

Juvenile polysaccharidosis with cardioskeletal myopathy.

Polysaccharidoses with ultrastructural features reminiscent of glycogenosis type IV, but without enzymatic correlation, have been observed in several adolescent and adult patients. Little is known of the clinical, pathologic, or biochemical nature of these disorders. We describe a patient with ultrastructural characteristics consistent with glycogenosis type IV, but with normal brancher enzyme activity in dermal fibroblasts and cardiac muscle. During life and at autopsy, electron microscopy revealed amylopectin-like polysaccharide deposits present in a wide variety of tissues. The polysaccharidosis of our patient and similar patients may be a variant of glycogenosis type IV with a yet to be defined enzymatic defect.

Toward a practical mobile robotic aid system for people with severe physical disabilities.

A simple, relatively inexpensive robotic system that can aid severely disabled persons by providing pick-and-place manipulative abilities to augment the functions of human or trained animal assistants is under development at Rice University and the Baylor College of Medicine. A stand-alone software application program runs on a Macintosh personal computer and provides the user with a selection of interactive windows for commanding the mobile robot via cursor action. A HERO 2000 robot has been modified such that its workspace extends from the floor to tabletop heights, and the robot is interfaced to a Macintosh SE via a wireless communications link for untethered operation. Integrated into the system are hardware and software which allow the user to control household appliances in addition to the robot. A separate Machine Control Interface device converts breath action and head or other three-dimensional motion inputs into cursor signals. Preliminary in-home and laboratory testing has demonstrated the utility of the system to perform useful navigational and manipulative tasks.

The 'fixed' removable partial denture: report of case.

Careful monitoring of tissue that had been the site of three previous surgical procedures was necessary, but the patient needed a functional, esthetically acceptable prosthesis. The Andrews bridge was selected because it combines the stabilizing qualities of a fixed prosthesis with the accessibility to the tissues of a removable prosthesis. The recurring nature of the verrucous carcinoma along with extensive tissue loss were additional factors in selecting this appliance. The final results satisfied all the criteria. The patient has been seen at regular follow-up visits for 2 years since the last surgical procedure and there has been no clinical change in the previously involved tissue. The patient has tolerated the prosthesis well and is satisfied with her appearance.

Applying robotic technology to aid people with severe disabilities.

Researchers at Rice University and the Baylor College of Medicine in Houston have developed a prototype robotic aid for people with severe disabilities, with emphasis on low cost, versatility, and ease of use. Using custom software written for the Macintosh¿ line of personal computers and a custom input device that emulates a mouse, a user can command a modified HERO 2000¿ robot to perform simple fetch and carry tasks in a structured indoor environment. Further interaction with the environment is possible using a household appliance controller that is also interfaced to the computer.

Meta-analysis: the safety and efficacy of dilation in eosinophilic oesophagitis.

BACKGROUND: Oesophageal dilation is one of the most effective options in the management of symptoms of eosinophilic oesophagitis (EoE). However, earlier reports described an increased rate of complications. AIM: To perform a meta-analysis of population-based studies of the risks associated with dilation and the clinical efficacy and duration of response to dilation in EoE. METHODS: Using MEDLINE and EMBASE, a systematic search was performed for published articles since 1977 describing cohort or randomised controlled trials of dilation in EoE. Summary estimates, including 95% confidence interval (CI), were calculated for the occurrence of complications associated with dilations (perforations, haemorrhage, chest pain, lacerations) and percentage of patients with symptom improvement following dilation. Heterogeneity was calculated using the I² statistic. RESULTS: The search resulted in 232 references, of which 9 studies were included in the final analysis. The studies described 860 EoE patients, of whom 525 patients underwent at least one oesophageal dilation and a total of 992 dilations. There were three cases of perforation (95% CI 0-0.9%, I² 0%) and one haemorrhage (95% CI 0-0.8%, I² 0%). Six studies reported postprocedural chest pain in 2% of cases (95% CI 1-3, I² 53%). Clinical improvement from dilation occurred in 75% of patients (95% CI 58-93%, I² 86%). CONCLUSIONS: Dilation in patients with eosinophilic oesophagitis is a safe procedure with a low rate of serious complications (<1%), and seems to result in at least a short-term improvement of symptoms in the majority of patients.