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Studies the aortic root dilation in Kawasaki disease, a systemic vasculitis which is a leading cause of acquired heart disease in children. Characteristics of patients involved in the study; Comparison of the aortic root dimension in patients with Kawasaki disease with those free from known heart disease; Detection of mild aortic regurgitation by color Doppler flow imaging.

Data from exercise tests, echocardiograms, and lung perfusion scans were analyzed to determine whether the excessive minute ventilation (VE) often encountered among patients with tetralogy of Fallot is due to ventilation-perfusion mismatch secondary to branch pulmonary artery stenoses. Patients with branch PA stenoses had lower peak oxygen consumptions and higher VE during exercise than did patients without stenoses, and a strong correlation existed between the degree of pulmonary blood flow maldistribution on lung perfusion scan and the amount of excessive VE during exercise. [ABSTRACT FROM AUTHOR]

"Shaken baby syndrome" (SBS) results in intracranial and intraocular hemorrhages with no evidence of external trauma. The cause of these injuries is vigorous shaking of an infant being held by the chest, shoulders, or extremities. A significant number of cases of head trauma in infants and young children are a direct result of SBS. In extreme cases, SBS may result in death. Many cases of SBS go unidentified because of the absence of external injuries, no witnesses to the event, and the failure of the abuser to admit his or her actions. Because of the numbers of children affected and the difficulty in detecting SBS, it is essential that critical care nurses who care for children be aware of SBS as a potentially lethal form of abuse. This article educates nurses about the signs and symptoms of SBS in an attempt to foster early recognition and interventions in cases of SBS to reduce mortality and morbidity associated with the syndrome.

In Munchausen Syndrome by Proxy (MSBP), a caregiver fabricates or induces illness in another individual. In most cases, the parent is the perpetrator and the child, the victim. MSBP is a form of child abuse that in extreme cases may result in death. A large percentage of health care providers are not familiar with the syndrome. Early recognition of MSBP is necessary to reduce morbidity and mortality associated with the syndrome.

Critical care nurses may encounter a victim of domestic violence or abuse when caring for an individual with traumatic injuries. Understanding the injuries that are associated with acts of violence is only the first step. A vital part of the nurse's responsibilities is the precise written documentation of observations, physical assessments, and other factors that may later become vital evidence in a court of law.

Kawasaki disease is the leading cause of acquired heart disease in childhood. Despite treatment with intravenous gamma globulin, 2% to 4% of patients have coronary abnormalities. Those with giant aneurysms are at risk for stenosis and myocardial ischemia/infarction, and require aggressive anticoagulation with frequent follow-up, including stress testing and coronary angiography. In rare cases, patients will have coronary artery bypass grafting. Those with less severe coronary involvement need antiplatelet therapy and infrequent noninvasive testing. Patients with normal echos after the acute phase are not treated, but the future impact of the disease is not certain particularly in the setting of adult onset coronary artery disease.

Background: Cisapride is a prokinetic agent that facilitates gastrointestinal motility and is widely used for the treatment of gastroesophageal reflux disease (GERD) in adults and children. However, reports of ventricular proarrhythmia have been noted in patients taking cisapride, particularly in conjunction with other drugs that may inhibit hepatic metabolism of cisapride via the cytochrome P450 3A4 system., Objective: We designed a prospective, blinded study to evaluate the effect of cisapride on ventricular repolarization in children with GERD., Methods: We analyzed the electrocardiograms (ECGs) from 35 children (age 0.4 to 18 years, mean 5.2 years) including measurement of the resting QT interval (QTc), JT interval (JTc), as well as QT and JT interlead dispersion markers. Data from these patients were compared with ECGs from a control group of 1000 normal children., Results: Eleven (31%) of 35 patients receiving cisapride had a prolonged QTc (> or = 450 ms). The JTc was prolonged > or = 360 ms in 16 of 35 patients (46%). The mean QTc in the cisapride group was 428 +/- 35 ms and mean JTc was 336 +/- 35 ms. An increased QT or JT dispersion (> 70 ms) was seen in only 3 of 35 children. Of the 11 children with QTc prolongation, 2 had documented torsades de pointes ventricular tachycardia. Both patients were taking cisapride concomitantly with a macrolide antibiotic. All other patients were treated with either cisapride alone or in conjunction with other GERD agents, such as ranitidine or omeprazole., Conclusions: Cisapride may cause prolongation of ventricular repolarization in children. There does not appear to be increased heterogeneity of repolarization or delayed depolarization in this small sample. The proarrhythmia may be exacerbated by medications that inhibit cytochrome P450 3A4 hepatic metabolism, overdosage, or mechanisms that result in decreased serum clearance. ECG intervals should be monitored in children maintained on cisapride, particularly when used in combination with other known QT-prolonging medications.

Junctional tachycardias comprise several arrhythmia types with differing mechanisms, principally involving the region of the atrioventricular (A-V) junction. Neonatal radiofrequency catheter ablation has typically been reserved for life-threatening, drug-refractory cases due to the unique concerns regarding patient size and development. We performed radiofrequency catheter ablation on two neonates with incessant, rapid junctional tachycardias and hemodynamic compromise after failing conventional medical therapy. This report describes 2 neonates who underwent emergent radiofrequency catheter ablation, and compares these two patients to a larger pediatric catheter ablation patient cohort. Both neonates had an acutely successful outcome and were able to be discharged within a week of the ablation procedure. Fluoroscopy time and total procedure time were shorter in these two patients than in the course of the average pediatric catheter ablation. Though long-term developmental consequences of neonatal catheter ablation are yet unknown, in unique extreme situations, radiofrequency catheter ablation can be performed in neonates, as in older children and adults, without excessive acute morbidity.

Following surgery for tetralogy of Fallot (TOF), children may develop late onset ventricular arrhythmias. Many patients have both depolarization and repolarization abnormalities, including right bundle branch block (RBBB) and QT prolongation. The goal of this study was to improve prospective risk-assessment screening for late onset sudden death. Resting ECG markers including QRS duration, QTc, JTc, and interlead QT and JT dispersion were statistically analyzed to identify those patients at risk for ventricular arrhythmias and sudden cardiac death. To determine predictive markers for future development of arrhythmia, we examined 101 resting ECGs in patients (age 12 +/- 6 years) with postoperative TOF and RBBB, 14 of whom developed late ventricular tachycardia (VT) or sudden death. These ECGs were also compared with an additional control group of 1000 age- and gender-matched normal ECGs. The mean QRS (+/- SD) in the VT group was 0.18 +/- 0.02 seconds versus 0.14 +/- 0.02 seconds in the non-VT group (P < 0.01). QTc and JTc in the VT group was 0.53 +/- 0.05 seconds and 0.33 +/- 0.03 seconds compared with 0.50 +/- 0.03 seconds and 0.32 +/- 0.03 seconds in the non-VT group (P = NS). There was no increase in QT dispersion among TOF patients with VT or sudden death compared with control patients or TOF patients without VT, although JT dispersion was more common in the TOF groups. A prolonged QRS duration in postoperative TOF with RBBB is more predictive than QTc, JTc, or dispersion indexes for identifying vulnerability to ventricular arrhythmias in this population, while retaining high specificity. The combination of both QRS prolongation and increased JT dispersion had very good positive and negative predictive values. These results suggest that arrhythmogenesis in children following TOF surgery might involve depolarization in addition to repolarization abnormalities. Prospective identification of high-risk children may be accomplished using these ECG criteria.

The purpose of this study was to evaluate the fate of mitral regurgitation (MR) following repair of atrioventricular septal defects (AVSDs). Echocardiograms of all survivors of isolated AVSD surgery between 1986 and 1996, who had had > or =2 postoperative color Doppler studies (39 patients), were reviewed. On each study, MR severity was graded on a 1+ to 4+ scale, based upon the size of the MR jet. Median age at surgery was 9 months (range 3 to 169); median age at postoperative follow-up was 45 months (range 3 to 107). Mild deterioration of mitral valve function was fairly common. MR severity increased by > or =1 grade in 16 patients (41%) during the course of the study. However, the deterioration in mitral valve function occurred primarily during the early postoperative time intervals. After the initial 32 postoperative months, MR worsened on only 4 occasions and in each instance worsened by only 1 grade. Deterioration to 4+ MR occurred in only 3 patients, and was not observed after the initial 30 postoperative months. Survival curve analysis predicted a 90% probability of not having severe (4+) MR after 30 months (lower 95% confidence bound: 80%). Postoperative MR remains fairly stable following AVSD repair. Serious deterioration is rare, especially after the initial 30 postoperative months.

Lung perfusion scans reveal that multiple-coil closure of the ductus does not interfere with pulmonary blood flow. However, caution is necessary when placing multiple coils in small infants.

Our objective was to assess the immediate and short-term results of stent implantation to relieve pulmonary artery stenosis (PAS). Thirty-seven patients underwent an attempt at stent implantation at a median age of 7.0 years (range, 0.8-31.4 years) and a median weight of 20.5 kg (range, 7.4-85 kg). Twenty-two patients had previous tetralogy of Fallot repair. A total of 55 stents were implanted successfully in 36 patients. The peak systolic gradient across the stenotic segment decreased from a mean of 43 +/- 20.4 mmHg prestent to 13 +/- 13.9 mmHg (P < 0.001) poststent. The diameter of the narrowest segment increased from a mean of 4.8 +/- 1.6 mm to 10.5 +/- 2.6 mm (P < 0.001). The right ventricular-to-aortic mean systolic pressure ratio decreased from 0.74 +/- 0.2 to 0.52 +/- 0.19 (P < 0.001). Complications included balloon rupture prior to full stent expansion in 4 patients (in 2 patients the stent was positioned in the superior vena cava, and in 2 in the inferior vena cava), distal migration of a stent which was successfully retrieved at surgery 1 mo later in 1 patient, and tethering of the stent to the balloon requiring surgical removal in 1 patient. One patient died several hours after stent placement. Sixteen patients underwent repeat catheterization at a mean follow-up interval of 0.9 +/- 0.5 years (range, 0.2-2.0 years). The mean gradient across the stent for these 16 patients was 26.7 +/- 19.8 mmHg, and there was no change in the mean diameter (9.4 +/- 3.2 mm). Two patients developed stenosis related to neointimal proliferation at the stent site which was redilated successfully. In conclusion, stent implantation is generally safe and effective in relieving PAS.

Kawasaki syndrome (KS) is an acute multisystem vasculitis of infancy and early childhood associated with the development of myocarditis and coronary artery abnormalities. Despite the widely held belief that KS is caused by an infectious agent, there remains considerable controversy over its etiology. Recent immunologic and microbiologic studies suggest a potential role for staphylococcal and streptococcal toxins (superantigens) in the pathogenesis of KS. Confirmation of these findings could result in more effective diagnostic and therapeutic approaches to this common cause of acquired heart disease in children.

Objectives: This study attempted to determine the site, relative size and dynamic geometry of atrial septal defects using dynamic three-dimensional echocardiography., Background: Recent studies have demonstrated the feasibility of dynamic three-dimensional echocardiography. Images are acquired from computerized reconstruction of sequential, tomographic ultrasound "slices" of the heart. Ultrasound images can be obtained by linear progression of a transducer within a transesophageal imaging probe. In small infants and children the large transducer size has not allowed transesophageal placement, and the probe has been placed on the thorax or in the subcostal position. Other scanning devices, housed in plastic containers, acquire images in a rotational format and can also be placed in a transthoracic or subcostal position., Methods: Specially designed transesophageal probes and a dedicated computer unit were used for two-dimensional image retrieval and reconstruction of three-dimensional images. Sixteen patients with atrial septal defects were studied (median age 18 months, range 1 day to 18 years). In one patient, images were obtained by transesophageal probe placement; in the other 15 patients, the probe was placed in the transthoracic or subcostal position., Results: A dynamic three-dimensional echocardiogram of the atrial septal defect could be obtained in 13 of the 16 patients. The distinguishing features of the atrial septal defects and their spatial orientation could be visualized in unique three-dimensional views., Conclusions: Dynamic three-dimensional imaging could be applied to the specific evaluation of atrial septal defects. Unique views of the heart allowed for spatial comprehension of the defects, rendering potentially important clinical information.

Objectives: The aim of this study was to determine whether persistent coronary aneurysms in patients with Kawasaki disease are associated with altered myocardial perfusion and function., Background: Some patients with Kawasaki disease have died suddenly because of severe coronary artery stenosis; others have chronic coronary aneurysms., Methods: Eleven patients with chronic coronary aneurysms were enrolled in the study. The size of the aneurysms and the degree of associated stenosis were determined by angiography in nine patients and by echocardiography in two. All patients underwent simultaneous function and myocardial perfusion assessment during symptom-limited exercise by echocardiography and technetium-99m sestamibi imaging, respectively., Results: Of 33 vascular territories, 18 contained coronary aneurysms measuring 3.5 to 10 mm. Three aneurysms were associated with significant stenosis as detected by angiography. Of the 18 vascular territories, 13 were normal, and 5 manifested stress-induced perfusion defects; of the latter 5 areas, 4 had associated wall motion abnormalities. The three territories supplied by stenotic coronary arteries had both abnormal regional function and perfusion demonstrated during exercise., Conclusions: Patients with chronic coronary aneurysms may have associated stenosis, as detected by angiography, with a subjacent myocardium that is subject to abnormal perfusion and function. However, the majority of coronary aneurysms are associated with normal regional coronary flow reserve, as assessed by myocardial perfusion imaging, and even giant coronary aneurysms may be associated with normal coronary flow reserve and preserved regional myocardial function during stress.

Objective: To evaluate the safety and efficacy of a new low profile balloon septostomy catheter in neonatal animals as well as in one newborn infant., Background: Balloon atrial septostomy remains one of the most commonly performed palliative procedures in pediatric cardiology. The currently available septostomy catheter requires a large introducer sheath (6 or 7F), does not have an end hole for confirmation of position or pressure measurement and is limited in patients with a small left atrium due to its large balloon inflated diameter., Methods: Four neonatal piglets (average weight 3.9 kg) underwent percutaneous balloon atrial septostomy using the new balloon catheter inflated to 1 cc via a 5F sheath in the femoral vein. Two other piglets (average weight 4.9 kg) underwent septostomy with the conventional catheter inflated to 3.5 cc via a 6 or 7F sheath in the femoral vein. All animals underwent transthoracic echocardiography pre and post septostomy. All animals were sacrificed after the procedure and the size of the atrial defect created was measured. One neonate with Taussig-Bing anomaly underwent septostomy with the new balloon catheter., Results: The left atrium was entered in all piglets. It was easier to enter the left atrium with an end hole catheter which was exchanged over a wire with the septostomy catheter. Septostomy was performed with the new or conventional catheters without complications. Echocardiography demonstrated a very small patent foramen ovale prior to the procedure and a large atrial defect after septostomy. The average size of the defect created by the new catheter was 11.3 x 10 mm in diameter and 11 x 10 mm using the conventional catheter. A 10 x 10 mm atrial communication was created in the neonate., Conclusions: This study demonstrates the safety and efficacy of this new catheter. This catheter will be of potential importance in patients with a small left atrium and in small neonates with congenital heart disease requiring septostomy.

Three-dimensional cardiac reconstruction generated from transesophageal interrogation can be performed using an integrated unit that captures, processes, and postprocesses tomographic parallel slices of the heart. This probe was used for infants and young children in the transthoracic position to evaluate the feasibility of producing three-dimensional cardiac images with capability for real-time dynamic display. Twenty-two infants and children (range 1 day-3.5 years) underwent image acquisition using a 16 mm 5 MHz 64 element probe placed over the precordium. Two infants were also imaged from the subcostal position. Data was obtained and stored over a single cardiac cycle after acceptable cardiac and respiratory gating intervals were met. The transducer was advanced in 0.5-1 mm increments over the cardiac structures using identical acquisition criteria. The images were reconstructed from the stored digital cubic format and could be oriented in any desired plane. In 9 of the 22 infants the images obtained were of optimal quality. The images obtained displayed normal cardiac structures emphasizing depth relationships as well as visualization of planes not generally demonstrated by two-dimensional imaging. Several lesions were also depicted in a unique fashion using this technique. Though the method employed was limited by movement artifact and reconstruction time, the quality of the three-dimensional display was excellent and enhanced by real-time demonstration. The transthoracic approach was successful in capturing sufficient data to create three-dimensional images, which may have further application in more accurate diagnosis of complex cardiac abnormalities and generation of planes of view which could duplicate surgical visualization of a lesion. Further assessment of the technique in infants with congenital heart disease is warranted.

Kawasaki syndrome (KS), the main cause of acquired heart disease in children, is associated with the selective expansion of V beta 2+ T cells in peripheral blood. Our study suggests that KS may be caused by a superantigen--a staphylococcal or streptococcal toxin. Bacteria were cultured without knowledge of their origin, from the throat, rectum, axilla, and groin of 16 patients with untreated acute KS and 15 controls. Bacteria producing toxins were isolated from 13 of 16 KS patients but from only 1 of 15 controls (p < 0.0001). Toxic shock syndrome toxin (TSST) secreting Staphylococcus aureus was isolated from 11 of the 13 toxin-positive cultures, and streptococcal pyogenic exotoxin (SPE) B and C were found in the other 2. These toxins are known to stimulate V beta 2+ T cells. All TSST-producing KS isolates were tryptophan auxotrophs indicating they were clonally related. S aureus isolates from acute KS patients were unusual because they produced less lipase, haemolysin, and protease compared to other isolates (p < 0.01). S aureus colonies from KS patients were white, and could be easily mistaken for coagulase-negative staphylococci, whereas colonies of non-KS isolates were gold. These observations suggest that the expansion of V beta 2+ T cells in most patients with KS may be caused by a new clone of TSST-producing S aureus, and, in a minority of patients, SPEB-producing or SPEC-producing streptococci.

Background: Infants with cardiac disease or prematurity are at risk for severe illness caused by respiratory syncytial virus. Immune globulin with a high titer of antibodies against respiratory syncytial virus may offer infants and young children at risk protection from this serious, common respiratory illness., Methods: We studied 249 infants and young children (mean age, eight months) who had bronchopulmonary dysplasia due to prematurity (n = 102), congenital heart disease (n = 87), or prematurity alone (n = 60). Respiratory syncytial virus immune globulin was given monthly to some of these children in either a high dose (750 mg per kilogram of body weight; n = 81) or low dose (150 mg per kilogram; n = 79); 89 controls received no immune globulin. Group assignments were random. Assessments of respiratory illness and management were conducted without knowledge of the children's group assignments., Results: There were 64 episodes of respiratory syncytial virus infection: 19 in the high-dose group, 16 in the low-dose group, and 29 in the control group. In the high-dose group there were fewer lower respiratory tract infections (7, vs. 20 in the control group; P = 0.01), fewer hospitalizations (6, vs. 18 in the control group; P = 0.02), fewer hospital days (43, vs. 128 in the control group; P = 0.02), fewer days in the intensive care unit (P = 0.05), and less use of ribavirin (P = 0.05). In the low-dose group there was a significant reduction only in the number of days in the intensive care unit (P = 0.03). Adverse events during the 580 infusions were generally mild and included fluid overload (in five children), oxygen desaturation (eight), and fever (six). Six children died: three in the high-dose group, three in the low-dose group, and none in the control group (P = 0.15), but no death was attributed to the use of immune globulin or to illness caused by respiratory syncytial virus., Conclusions: Administration of high doses of respiratory syncytial virus immune globulin is a safe and effective means of preventing lower respiratory tract infection in infants and young children at high risk for this disease.

Background: Severe pulmonary regurgitation (PR) and associated right ventricular (RV) dilatation are late complications of surgical repair of tetralogy of Fallot (TOF). For the past several years, we have restored pulmonary valve competence with the exclusive use of cryopreserved allografts., Methods and Results: Sixteen patients with symptoms of diminished exercise tolerance and echocardiographic evidence of progressive PR with severe RV dilatation underwent placement of allografts in the RV outflow tract at a median age of 12 years (10 years after TOF repair). Abnormal exercise tolerance tests were documented in 10 patients. Additional surgical procedures included pulmonary artery augmentation (n = 6), closure of residual left to right shunts (n = 3), and subendocardial resection for monomorphic ventricular tachycardia (n = 1). Six patients had either preoperative or postoperative balloon dilations of pulmonary artery stenoses. All patients had symptomatic improvement after allograft insertion. At a mean follow-up of 26.4 +/- 3.4 months, the severity of PR improved in all but one patient. In 12 patients (group 1), conduit regurgitation was either trace (n = 11) or mild (n = 1). Four patients (group 2) had moderate conduit regurgitation. In a retrospective analysis, pulmonary artery diameters and cross-sectional areas were significantly smaller in the group 2 patients compared with the group 1 patients. With the exception of one patient, RV end-diastolic diameter (RVEDD/BSA) fell after allograft insertion in each patient (P < .01). The reduction in RVEDD/BSA was significantly greater in group 1 than in group 2 (31.8 +/- 3.4% versus 21.4 +/- 11.0%, P < .05)., Conclusions: Thus, restoration of the pulmonary valve with cryopreserved allografts improved exercise tolerance and diminished RV volume overload in patients with severe PR after previous repair of TOF. Optimal results were achieved in patients who did not have significant residual pulmonary artery distortion.

Objective: We performed this study in order to evaluate the usefulness of a new balloon expandable stent for maintaining ductal patency in a neonatal piglet model and to evaluate the ability to re-expand the stent weeks following initial implantation., Background: Maintaining patency of the ductus arteriosus without administration of Prostaglandin E has been reported previously using balloon dilation and stent implantation techniques. However, the experience is limited and the currently available stents are not modified for neonates., Methods: 14 newborn piglets all at age 12 days and median weight 3.6 Kg (range 2.7-4.3 Kg), underwent initial balloon dilation of the ductus arteriosus. Angiography after dilation demonstrated no significant left to right shunt. All piglets underwent successful stent (3.5 mm x 17 mm) placement in the ductus arteriosus., Results: Percutaneous ductal stent implantation via the arterial route was successful in all piglets with angiographic demonstration of a significant left to right shunt. Follow-up studies at weekly intervals with color flow Doppler were used to confirm patency of the stents. In 3 piglets the stent was not patent at initial follow-up and autopsy revealed sub-optimal stent placement. In two animals the stent was later re-expanded to 4 mm at 22 days, in one to 4 mm at 30 days and in one to 6 mm at 15 days, maintaining flow for an additional period of 15 to 34 days.(ABSTRACT TRUNCATED AT 250 WORDS)

One week after treatment with intravenously administered immune globulin and aspirin, a child with Kawasaki disease had persistent fever and an increase in coronary artery diameter to greater than 3 mm. Two additional doses of immune globulin were given intravenously. Rapid hemolysis occurred, followed by disseminated intravascular coagulation and serum sickness. Clinicians should be aware that immune globulin preparations contain antibodies to blood-type antigens that may cause significant hemolysis and disseminated intravascular coagulation.

Background: Treatment of acute Kawasaki syndrome with a four-day course of intravenous gamma globulin, together with aspirin, has been demonstrated to be safe and effective in preventing coronary-artery lesions and reducing systemic inflammation. We hypothesized that therapy with a single, very high dose of gamma globulin would be at least as effective as the standard regimen., Methods: We conducted a multicenter, randomized, controlled trial involving 549 children with acute Kawasaki syndrome. The children were assigned to receive gamma globulin either as a single infusion of 2 g per kilogram of body weight over 10 hours or as daily infusions of 400 mg per kilogram for four consecutive days. Both treatment groups received aspirin (100 mg per kilogram per day through the 14th day of illness, then 3 to 5 mg per kilogram per day)., Results: The relative prevalence of coronary abnormalities, adjusted for age and sex, among patients treated with the four-day regimen, as compared with those treated with the single-infusion regimen, was 1.94 (95 percent confidence limits, 1.01 and 3.71) two weeks after enrollment and 1.84 (95 percent confidence limits, 0.89 and 3.82) seven weeks after enrollment. Children treated with the single-infusion regimen had lower mean temperatures while hospitalized (day 2, P less than 0.001; day 3, P = 0.004), as well as a shorter mean duration of fever (P = 0.028). Furthermore, in the single-infusion group the laboratory indexes of acute inflammation moved more rapidly toward normal, including the adjusted serum albumin level (P = 0.004), alpha 1-antitrypsin level (P = 0.007), and C-reactive protein level (P = 0.017). Lower IgG levels on day 4 were associated with a higher prevalence of coronary lesions (P = 0.005) and with a greater degree of systemic inflammation. The two groups had a similar incidence of adverse effects (including new or worsening congestive heart failure in nine children), which occurred in 2.7 percent of the children overall. All the adverse effects were transient., Conclusions: In children with acute Kawasaki disease, a single large dose of intravenous gamma globulin is more effective than the conventional regimen of four smaller daily doses and is equally safe.

The clinical and laboratory findings in and the treatment of congestive heart failure, supraventricular tachycardia, pericardial disease, and hypoxemic spells are discussed.

Forty-four patients, aged 6 months to 11 years, meeting the clinical criteria for mucocutaneous lymph node syndrome (Kawasaki disease) were studied from March 1980 to March 1981. A protocol was designed for evaluation of cardiac involvement including electrocardiogram, chest X-ray film, cardiac enzyme determinations and M mode and two dimensional echocardiograms. Aspirin was given during the acute febrile period as an anti-inflammatory agent in a dosage of 100 mg/kg per day; followed by 30 mg/kg per day for 10 weeks or longer if platelet counts were elevated. Angiocardiography was performed in 38 patients. Twenty-one patients (48 percent) showed abnormal M mode echocardiographic findings (flat or greatly decreased ventricular septal motion, pericardial effusion or decreased left ventricular function) during the acute febrile stage. Coronary artery disease developed in seven patients, all of whom had abnormal M mode echocardiographic abnormalities in the acute stage. Five patients had coronary aneurysms and two patients had dilated coronary arteries. Two dimensional echocardiograms identified coronary arterial lesions accurately if present proximally but failed to detect aneurysms beyond 1.5 cm from the aortic root. The study shows that serial M mode and two dimensional echocardiograms provide sufficient information to rule out cardiac involvement in Kawasaki disease. Echocardiograms should be obtained every 3 to 4 days during the acute febrile stage because all patients with coronary artery disease detected with angiography had abnormal echocardiographic findings during that period.

The outcome in 13 children with congenital valvar aortic stenosis who underwent a repeat valvotomy for significant residual obstruction was reviewed. There was no hospital mortality (confidence limits 0% to 32%), while one late death (8%) occurred (confidence limits 1% to 57%) following the repeat procedure. Cardiac catheterization was performed in 10 patients (77%) following the second valvotomy (2 years, 7 months mean follow-up): (1) three children, all infants at initial valvotomy, had a peak systolic ejection gradient less than or equal to 40 mm Hg and only mild, at most, aortic regurgitation after the repeat operation; (2) the other seven had a peak systolic ejection gradient greater than 40 mm Hg or at least moderate aortic regurgitation, of whom two were infants at first valvotomy while the others ranged in age from 1 to 12 years at initial surgery. These results suggest that repeat aortic valvotomy may not significantly improve the hemodynamic status of those who did not require initial valvotomy early in life.

Coronary artery inflammation in Kawasaki disease is accompanied by thrombocytosis and platelet activation. It was hypothesized that abnormal metabolism of bioactive eicosanoids could result from or contribute to these events. Circulating plasma thromboxane B2, 6-keto-prostaglandin F1 alpha and prostaglandin E were measured by double antibody radioimmunoassay in patients with Kawasaki disease before and after aspirin alone or aspirin and intravenous gamma globulin therapy. Plasma prostaglandin E concentrations were normal in all patient groups. Pretreatment thromboxane B2 was elevated compared with age-matched controls, fell moderately with high-dose aspirin (60 to 100 mg/kg/day) and marginally increased with low-dose aspirin (3 to 5 mg/kg/day) 6 to 8 weeks after treatment. Plasma 6-keto-prostaglandin F1 alpha was not detected in 12 of 16 patients before therapy and remained low in all but 1 patients by 6 to 8 weeks. Thromboxane B2 correlated weakly with serum salicylate concentration but had no relation to platelet mass. The results in these patients with Kawasaki disease indicate only partial thromboxane suppression and depressed prostacyclin generation regardless of therapy. This balance favors coronary vasoconstriction and platelet aggregation capable of potentiating myocardial ischemia or infarction. The results justify consideration of higher or more frequent aspirin doses for longer duration and thromboxane receptor blockade in this disease.

Eight patients, aged 1 to 8 years, with discrete subaortic stenosis (DSS) and ventricular septal defect (VSD) were studied by 2-dimensional (2-D) and M-mode echocardiography. Initial cardiac catheterization and angiocardiography showed VSD and other associated cardiac lesions, including coarctation of the aorta and patent ductus arteriosus. None had evidence of DSS. Six patients underwent surgical repair of the associated lesions, but none required closure of the VSD. Ultimately, 6 patients had spontaneous closure of VSD, and 2 had a residual small VSD. Subsequent serial echocardiography showed evidence of subaortic membrane, prompting repeat cardiac catheterization, which confirmed moderate to severe peak systolic pressure gradients between the left ventricle and ascending aorta. Surgical resection of the membrane was performed in 5 patients. Thus, in patients with small or spontaneously closed VSDs, DSS may develop. Evaluation of the left ventricular outflow tract area is recommended in patients with small or closed VSD in whom a significant heart murmur or electrocardiographic abnormality remains.

Of 75 patients meeting clinical criteria for Kawasaki disease, 67 patients were followed with serial ECG and echocardiograms (echo) for at least 1 year. Sixteen patients had coronary artery aneurysms and three had dilated coronary arteries as assessed by initial two-dimensional (2D) echo and/or angiocardiographic studies. ECG findings of first and second-degree heart block and ST-T wave changes (36%) as well as M-mode echo abnormalities (45%) including pericardial effusion, decreased ventricular septal motion, and decreased left ventricular function were all normalized by 6 months, except in two infants who continued to have decreased function and flat ventricular septal motion. In four patients with single proximal coronary aneurysm and two with dilated coronary artery, resolution of lesions occurred by 1 year. Among 11 patients with multiple coronary aneurysms discovered by initial studies, the 2D echo became normal in seven and showed aneurysms in four as corroborated by angiogram. One patient with an isolated distal coronary aneurysm, not seen by initial or serial echo, had a normal follow-up angiogram. Our study suggests that in Kawasaki disease careful, serial, 2D echo is sufficient to monitor the course of coronary artery disease that tends to regress and follow a benign course. However, the long-term effects of this disease on cardiac and coronary arteries remain to be elucidated.

To assess the natural history of ventricular tachycardia (VT) in children without heart disease, the clinical course of 26 children, aged 1 day to 15 years at initial detection, was reviewed. Symptoms related to the rhythm were present in 8 children at some time during their course of follow-up. Evaluation included echocardiogram (19 patients), Holter monitoring (22 patients), graded treadmill exercise testing (16 patients) and invasive electrophysiology (4 patients). Exercise induced or exacerbated VT in 9 patients, but suppressed rhythm in 7. Ten patients were never treated. Of the remaining 16 treated patients, therapy was discontinued in 10 on the basis of the electrocardiographic or Holter monitor recordings. Six continued to receive therapy without complications. There were no known deaths over a period of 1 month to 34 years (mean 59 months, median 42 months). Although 2 patients have been lost to follow-up, based on our findings and a review of the reported cases of VT in children to date, after undergoing complete noninvasive cardiac evaluation symptomatic patients should be treated and studied with invasive electrophysiology if antiarrhythmic control is inadequate. The rate of VT or age at onset are not predictive of outcome in asymptomatic patients. As a group, these patients do not appear to benefit from therapy, but warrant follow-up since deaths have been reported in untreated asymptomatic patients.

The purpose of these studies was to develop a high performance liquid chromatographic (HPLC) assay for plasma levels of indomethacin. A reversed phase (C18 Sep-Pak) cartridge was used to process plasma for absorption of indomethacin, the internal standard, and impurities. The recovery of plasma indomethacin and the added internal standard was quantitative from 0.5 ml of plasma. The assay was linear from 50 micrograms/l to 10 mg/l without concentration of the effluent from the Sep-Pak cartridge. The intra-assay coefficients of variation, for ten injections each to calibrate points at 0.15, 0.30, 0.50 and 1.00 mg/l, were 8.15%, 6.29%, 5.47% and 5.39%, respectively and for duplicates of duplicate calibration points were 7.51%, 6.32%, 4.41%, and 2.05%, respectively. The inter-assay coefficients of variation were 8.49%, 6.48%, 5.10%, and 2.22%, respectively. The sensitivity of the assay can be increased by a 3-5 fold concentration of the effluent from the Sep-Pak and preliminary experiments have indicated that as little as 100 microliter of starting plasma can be utilized in the assay. The assay can be used to determine the concentration of indomethacin in small volumes of plasma. Since C18 Sep-Pak cartridges were employed to remove contaminating substances, sensitivity and reproducibility were both high while column longevity and efficiency were excellent.

An infant girl is described who had cor triatriatum and partial anomalous pulmonary venous connection of the left pulmonary veins to the coronary sinus, the first report of this combination of lesions. The infant also had a Dandy-Walker malformation and multiple facial and intrathoracic hemangiomas. The cardiac diagnosis was made by two-dimensional echocardiography. Cardiac catheterization and angiography confirmed the findings and also demonstrated a persistent left superior vena cava draining to the coronary sinus. The infant underwent successful surgical repair. Partial anomalous pulmonary venous connection and left superior vena cava not infrequently are associated with cor triatriatum. Although two-dimensional echocardiography is sensitive for the detection of cor triatriatum, preoperative cardiac catheterization is necessary to identify unequivocally systemic and pulmonary venous connections.

Extravascular lung water (EVLW) was measured in 17 patients with congenital heart disease by the cold-green-dye, double indicator-dilution technique. In 5 control patients, EVLW was 4.7 +/- 0.5 ml/kg (111 +/- 13 ml/m2) (mean +/- standard deviation). Twelve patients were studied immediately after correction of their heart defects. In 6 patients with normal or decreased pulmonary blood flow preoperatively (Group I), EVLW was 6.2 +/- 1.9 ml/kg (122 +/- 46 ml/m2). This value is not significantly different from that of the control patients. In 6 patients with increased pulmonary blood flow and congestive heart failure preoperatively (Group II), EVLW was 15.7 +/- 3.8 ml/kg (270 +/- 60 ml/m2), which is significantly different from both control and Group I patients (p less than 0.01). There was no correlation of EVLW with pre- or postoperative left atrial pressure, length of cardiopulmonary bypass or deep hypothermic circulatory arrest, postoperative serum protein, albumin, hematocrit or cardiac index. Thus, EVLW in the immediate postoperative period is determined by preoperative pathophysiologic characteristics rather than by intraoperative management, and patients with congestive heart failure resulting from left-to-right shunts have increased EVLW despite normal left atrial pressures.