Severe Takayasu's arteritis of the pulmonary arteries: report of a case with successful surgical treatment.

Although involvement of the aorta and its large branches is more common, Takayasu's arteritis involving the pulmonary arteries is well recognized. This report describes an adolescent girl with an uncommonly severe form of Takayasu's arteritis involving the pulmonary arteries. A successful surgical treatment is presented.

Intracardiac undifferentiated sarcoma in infancy.

A rare case of an intracardiac undifferentiated sarcoma in a 3 month old infant is described together with the clinical, angiographic, echocardiographic, surgical and histopathologic findings. The tumor was successfully removed surgically, and monthly echocardiographic follow-up is being performed.

Aortico-left ventricular tunnel with ventricular septal defect: two-dimensional/Doppler echocardiographic diagnosis.

Aortico-left ventricular tunnel is a rare congenital anomaly that presents as aortic regurgitation and congestive heart failure in childhood. Its association with a ventricular septal defect is even more rare. Because of the distortion of the normal anatomy around the aortic valve and the rarity of this combination of defects, the diagnosis of aortico-left ventricular tunnel with ventricular septal defect may be difficult. The two-dimensional and Doppler echocardiographic findings of aortico-left ventricular tunnel are described.

Hemodynamic effects of dobutamine in children.

Dobutamine is useful for augmenting cardiovasuclar function in adults. However, no information is available on the action of dobutamine in children. To determine its hemodynamic effects in children, we infused dobutamine into 12 children with congenital heart disease during diagnostic cardiac catheterization. We administered dobutamine in two doses: first 2 and then 7.75 microgram/kg per min for 10 minutes each. We meaured heart rate, cardiac output, systemic and pulmonary arterial, right atrial and pulmonary capillary blood pressures before and during the infusion of dobutamine. Systemic and pulmonary vascular resistances, cardiac index and stroke index were calculated. Cardiac output, cardiac index, stroke volume, stroke index and systemic arterial phasic and mean blood pressures increased sugnificantly (P less than 0.05) and pulmonary capillary mean blood pressure decreased significantly (P less than 0.05) during the infusion of each dose of dobutamine compared with control values. Heart rate, pulmonary and right atrial mean blood pressure and systemic and pulmonary vascular resistance were unchanged with either dose of dobutamine. We noted no adverse effect from the drug.

Successful triple coronary artery bypass in a child with multiple coronary aneurysms due to Kawasaki's disease.

A 9-year-old girl developed ischemic cardiac symptoms 3 years after she first presented with characteristic manifestations of Kawasaki's disease, namely, high fever, conjunctivitis, lymphadenopathy, macular truncal skin rash, and erythema of both hands followed by desequamation of the skin of the fingertips. This acute illness resolved spontaneously within 2 weeks. Because of progressive and severe anginal symptoms and electrocardiographic signs of myocardial ischemia, she underwent cardiac catheterization and coronary angiography, which demonstrated multiple aneurysms of both right and left coronary artery systems. The two larger aneurysms of the right main and left main coronary arteries were clotted, causing complete occlusion of these vessels. Only collateral branches from the proximal right coronary artery which were supporting the entire coronary circulation, prevented her from having a myocardial infarction. A triple saphenous vein bypass was performed with excellent immediate results. One year later the patient was completely free of symptoms; she was living a normal life and a stress electrocardiogram was entirely normal.

Successful surgical correction of infradiaphragmatic total anomalous pulmonary venous return into the inferior vena cava. A fourteen-year follow-up.

This case report describes the long-term follow-up of a patient who, at 7 weeks of age, underwent surgical correction of infradiaphragmatic total anomalous pulmonary venous return (TAPVR) to the inferior vena cava. Fourteen years after operation, the patient has normal anatomy and nearly normal hemodynamics. The difference between this type of infradiaphragmatic TAPVR with posthepatic drainage is compared with the more common type which drains prehepatically.

False aneurysm of the ascending aorta from an expanded polytetrafluoroethylene (Gore-Tex) aortopulmonary shunt.

A false aneurysm of the ascending aorta developed in a 25-month-old male infant with tetralogy of Fallot because of an expanded polytetrafluoroethylene (PTFE) shunt that had been inserted between the ascending aorta and right pulmonary artery when the patient was 3 months of age. Surgical repair of tetralogy of Fallot with ligation of the PTFE graft was performed at 19 months of age. The false aneurysm at the site of the systemic anastomosis to the shunt was discovered 6 months later and was successfully repaired. Because of the potential for graft dehiscence secondary to growth, we recommend that PTFE shunts be removed completely or, at least, ligated and divided at the time of corrective surgical intervention.

Transient dilated cardiomyopathy in a newborn exposed to idarubicin and all-trans-retinoic acid (ATRA) early in the second trimester of pregnancy.

Acute promyelocytic leukemia was diagnosed in a 28-year-old pregnant woman at 13 gestational weeks. She was immediately started on idarubicin and all-trans-retinoic acid (ATRA) and achieved remission after her fourth cycle of treatment. Serial fetal ultrasonograms throughout pregnancy did not reveal any intrauterine growth retardation or other obvious malformations. The mother delivered a term (36.7 gestational weeks), 2720-gram female neonate. The infant was admitted to the intermediate care nursery for observation due to transient mild respiratory distress during the peripartum period. Because of right ventricular hypertrophy on an electrocardiogram, an echocardiogram was performed on the first day of life which showed moderate dilation of the right atrium and right ventricle with mildly depressed function, two small secundum atrial septal defects, and a small patent ductus arteriosus. The neonate remained hemodynamically stable and no arrhythmias were detected. The remainder of the hospital course was uneventful. When reassessed 1-1/2 months later, she was doing well and did not show any signs of congestive heart failure. A repeat echocardiogram at that time demonstrated complete resolution of the right heart enlargement and closure of the ductus arteriosus with persistence of the small and hemodynamically insignificant secundum atrial septal defects.

Infradiaphragmatic total anomalous pulmonary venous return. Diagnosis and surgical repair in a 10-year-old child.

A unique case of infradiaphragmatic total anomalous pulmonary venous return in a 10-year-old girl is described. A persistent ductus arteriosus, ventricular septal defect, and large atrial septal defect were also present. Corrective surgery was performed at 10 years of age, with an excellent clinical result. The factors which permitted this unprecedented period of survival included non-obstructed drainage of the pulmonary venous blood into the inferior vena cava and a large atrial septal defect.

Intracardiac electrography in children and young adults.

The interpretation of IE recorded in children has been hampered by a lack of agreement regarding normal values. We recorded IE in 158 children and young adults (ages, three days to 33 years) to define the various conduction intervals in normal and disease states. The HBP was recorded in 156 subjects. In 85 subjects with normal conduction indicated by surface ECG, including 19 subjects with normal hearts, there were no statistically significant age-related differences in internodal, A-V nodal, or His-Purkinje conduction intervals. Therapeutic levels of digitalis did not alter the conduction intervals. In 11 subjects with first degree A-V block and in five subjects with congenital complete A-V block, the site of block as determined by IE could not be predicted from the surface ECG. No abnormalities in conduction intervals were found in 18 subjects with right bundle branch block (surgically induced in 17 cases). Intracardiac electrography with recording of the HBP was found to be a safe, informative technique for electrophysiologic investigations in children and young adults.

Recurrent Kawasaki disease.

This case report describes a boy who had Kawasaki disease (KD) at age 12 months and had a recurrence one year later. The coronary arteries were normal following the initial episode; however, during the second episode he developed coronary aneurysms. Gallium-67 radionuclide imaging, echocardiography, and angiography were used to diagnose the coronary abnormalities.

Late development of pulmonary venous obstruction following Mustard's operation using a dacron baffle.

Three patients developed late pulmonary vein obstruction (PVO) following Mustard's operation for transposition of the great arteries. In all three the absence of PVO had been documented by an earlier postoperative cardiac catheterization. At reoperation shrinkage and kinking of the dacron baffle was evident. After Mustard's operation, patients should be observed closely for symptoms and signs of PVO since it can occur insidiously despite previously proven absence of this complication.

Blade atrial septostomy: experience with the first 50 procedures.

We have performed 50 blade and balloon atrial septostomies in 46 patients with diagnoses of transposition of the great arteries--32 patients; mitral atresia or stenosis--10 patients; total anomalous pulmonary venous drainage--2 patients; tricuspid atresia--1 patient; and pulmonary valve atresia with hypoplastic right ventricle--1 patient. The patients' age ranged from 1 day to 72 months (median = 8 months) and weights ranged from 2.7 to 14.5 kg. In patients with transposition the systemic saturation increased from an average of 62% to 74.6% (p less than 0.001) and the inter-atrial mean pressure gradient was reduced from 7.74 +/- 5.3 to 1.4 +/- 2.04 mm Hg. Patients with mitral atresia had no significant increase in systemic arterial saturation but a significant decrease in the mean inter-atrial gradient from 19.6 +/- 12.4 to 3.8 +/- 5.3 mm Hg. In three patients the blade septostomy was unsuccessful for technical reasons and the condition of the patient. Complications included one death due to atrial laceration, blood loss requiring transfusion in 5 patients, transient CVA in one patient, and failure of the blade to close in one patient. We have found the palliative use of the blade catheter in conjunction with balloon atrial septostomy to be an effective and safe procedure.

The use of exercise testing in children to evaluate abnormalities of pacemaker function not apparent at rest.

The purpose of this study was to evaluate the use of exercise testing in identifying abnormalities of pacemaker function and in confirming set parameters not apparent at rest in children with implanted atrial synchronous physiologic pacemakers. Maximal exercise tests were performed on 24 children (15 boys, 9 girls) from 4.5 to 18 years of age (median = 15) with physiologic pacemakers. The lower rate limit was observed before or following testing in 19 of 24 cases. In each case this correlated with the set lower rate limit. The upper rate limit was reached in 10 of 24 cases and was found to be lower than that programmed in one case in which a long atrial refractory period had limited the upper rate limit. Six children reached the maximum upper rate limit to which their pacemaker could be programmed. No abnormalities of atrial capture or ventricular capture occurred during exercise testing. Ventricular sensing was normal in each case. Atrial sensing was observed to be normal in 15 of the 24 cases. Two patients had decreased atrial sensing with exercise. Reversion to the "noise rate" due to myopotential inhibition was found in seven other cases. Subsequent tests on two of these children showed normal sensing.(ABSTRACT TRUNCATED AT 250 WORDS)