Utility of SPECT DMSA renal scanning in the evaluation of children with primary vesicoureteral reflux.

OBJECTIVES: DMSA renal scanning is more sensitive than ultrasound in detecting renal parenchymal scars. We proposed to determine the utility of single-photon emission computed tomography (SPECT) dimercaptosuccinic acid (DMSA) renal scanning in children with primary vesicoureteral reflux (VUR). METHODS: During a 24-month period, we evaluated the charts of 368 patients who had undergone SPECT DMSA renal scanning for primary VUR. Patients were divided into three age groups: (a) less than 1 year, (b) between 1 and 5 years, and (c) older than 6 years. Renal scars were deemed severe or focal. The data were analyzed to evaluate the utility of SPECT DMSA scanning in children with primary VUR and to determine the indications for performing SPECT DMSA. We also evaluated the sensitivity of recent renal ultrasound technology in detecting focal and diffuse scars. RESULTS: One hundred twenty-eight patients were younger than 1 year at presentation. These included 24 cases that were detected prenatally. One hundred eighty-five were between the ages of 1 and 5 years, and 55 were 6 years or older. Reflux nephropathy at presentation was found in 99 (26.9%) of 368 patients. DMSA scanning changed the treatment in only 13 patients (3.5%). When scarring was diffuse, ultrasound examination correlated 100% with DMSA scanning; when focal scarring was present, the correlation was poor. CONCLUSIONS: Our results suggest that DMSA scans should be tailored to children who have ultrasound abnormalities, high-grade reflux, or recurrent breakthrough urinary tract infections. These guidelines will result in a substantial cost savings and a significant decrease in radiation exposure.

Assessment of myocardial perfusion and function in childhood systemic lupus erythematosus.

OBJECTIVES: To determine the prevalence of abnormalities in myocardial perfusion or function in children with systemic lupus erythematosus (SLE), and describe potential factors that may predict their development. STUDY DESIGN: Patients (n = 40; 30 female) were enrolled through the Lupus Clinic at The Hospital for Sick Children between 1990 and 1992. Resting and exercise thallium myocardial perfusion scans, radionuclide angiography with multiple gated acquisition (MUGA), and resting M-mode and two-dimensional echocardiography were performed. RESULTS: All patients were free of symptoms, and none had a history of ischemic heart disease. Their median age was 15.9 years (range 10.5 to 19.8 years) at enrollment. Abnormalities of coronary perfusion were found in 5 (16%) of 31 patients (95% confidence interval: 3%, 29%) and included a large fixed perfusion defect in 1; 5 of 27 MUGA scans showed marginally low left ventricular ejection fractions at rest, whereas all had normal exercise responses. In the group with abnormal thallium scans, three of five patients had antiphospholipid antibodies detected, and two of four had an abnormal plasma lipid profile. This group tended to have a shorter disease duration and had received a lower cumulative dose of corticosteroids; these differences were not statistically significant compared with the group with normal scans. CONCLUSION: Asymptomatic abnormalities of myocardial perfusion occur in children with SLE and are more common than previously suspected. Patients with these abnormalities of myocardial perfusion may be predisposed to the previously recognized early-onset ischemic heart disease seen in adults with SLE.

99mTechnetium HMPAO imaging in children with the Sturge-Weber syndrome: a study of nine cases with CT and MRI correlation.

Demonstration of the full extent of abnormality in patients with the Struge-Weber syndrome (SWS) is important for prognosis and in planning surgery to remove the seizure focus. We compared single-photon emission computed tomography (SPECT), MRI and CT in nine children under the age of 4 years with seizures as part of SWS, in an attempt to determine the optimal method of imaging in different clinical settings. Seven unilateral and two bilateral cases were studied by interictal 99mtechnetium hexamethylpropyleneamineoxime (HMPAO) SPECT, and contrast-enhanced CT and MRI, giving information on 11 abnormal hemispheres. All imaging modalities showed abnormalities in every child. Perfusion imaging showed focal regions of decreased uptake in 9 of 11 (82%) abnormal hemispheres and demonstrated a widespread decrease but no focal defect in 2; it also revealed crossed cerebellar diaschisis in 2 cases. CT demonstrated typical gyriform calcification in 9 of 11 (82%) affected hemispheres. Contrast-enhanced MRI showed more extensive involvement than contrast-enhanced CT in 5 of 11 (45%) cases. The area of hypoperfusion shown by SPECT was smaller than the area of contrast enhancement on MRI in 6 of 11 cases (55%), comparable in 3 (27%) and larger in 2 cases (18%). CT is sufficient to confirm the clinical diagnosis of SWS, but MRI frequently shows more extensive abnormal areas. 99mTc HMPAO imaging is a useful addition when it is important to know the full extent of the disease, for example prior to surgery. It is likely to detect areas of hypoperfusion, representing ischaemic regions, which may act as an epileptogenic focus and may not be shown by CT or MRI.

Infantile spasms: cerebral blood flow abnormalities correlate with EEG, neuroimaging, and pathologic findings.

This ongoing study examines abnormalities of cerebral perfusion in a consecutive series of children with infantile spasms and correlates cerebral blood flow (CBF) abnormalities with electroencephalographic (EEG), neuroimaging, and pathologic findings. A consecutive series of children with infantile spasms, diagnosed by standard clinical and EEG criteria, had cerebral perfusion studies using 99Tc-HmPAO single photon emission computed tomography (SPECT), together with neuroimaging studies using computed tomography (CT) and/or magnetic resonance imaging (MRI), interpreted independently and correlated with surgical pathologic findings. Twenty children aged 2-13 months (mean 9.3 months) were studied over a 4-year period; 60% had symptomatic infantile spasms due to cerebral dysgenesis (33%), other congenital lesions (25%), tuberous sclerosis (17%), or other causes (25%), and the remaining patients were cryptogenic (40%). CBF abnormalities were present in 85%: multifocal decrease (40%), focal increase (25%), diffuse decrease (15%), and focal increase (10%), while the remaining 15% had normal cerebral blood flow. Focal cortical lesions may lead to infantile spasms, even in cryptogenic patients diagnosed by functional neuroimaging such as 99Tc-HmPAO SPECT. In selected patients, surgical excision of the cortical lesions leads to improved seizure control and possibly outcome. The localization and surgical excision of focal cortical lesions in infantile spasms required further investigation with functional and structural neuroimaging, EEG, and intraoperative electrocorticography.

Neurologic manifestations of pediatric systemic lupus erythematosus.

Central nervous system involvement is a common but rarely reviewed feature of pediatric systemic lupus erythematosus (SLE). We retrospectively reviewed the charts of 91 patients with pediatric SLE and using a standardized data abstraction form documented 40 patients with central nervous system (CNS-SLE) involvement. The mean age of onset of SLE was 13.3 years. In 19 patients the CNS manifestation was a presenting symptom, in 12 patients CNS involvement was present within the first year of diagnosis, and in 9 patients it took up to 7 years for CNS disease to become evident. Nineteen children (48%) manifested neuropsychiatric SLE, which included depression, concentration or memory problems, and frank psychosis. Seizures were present in 8 patients (20%), 6 had cerebral ischemic events (15%), 1 had chorea (3%), 2 had papilledema (5%), and 2 patients had a peripheral neuropathy (5%). Nine patients (22%) had severe headache consistent with lupus headache. Seven children had more than one CNS manifestation. In the investigation of CNS-SLE, computed tomography and/or magnetic resonance imaging scans were helpful in patients with focal ischemic lesions and venous sinus thrombosis. Electroencephalography was abnormal only in 33% of patients with seizure disorders and rarely helpful in patients with diffuse neuropsychiatric symptoms. Single-photon emission computed tomography scans were abnormal in most patients with neuropsychiatric SLE, especially in those with frank psychosis. The lupus anticoagulant was present in the patient with chorea and was frequently present in patients with cerebral vascular events. Long-term outcome was good: only 1 child died of cerebral hemorrhagic infarction and 3 others had significant persistent CNS deficits. The majority of patients (90%) had excellent recovery from CNS-SLE.

Location of epileptic foci on interictal and immediate postictal single photon emission tomography in children with localization-related epilepsy.

Several neuroimaging techniques that supplement electrophysiologic methods of evaluating pediatric patients with localization-related epilepsies before surgery assess both structural and functional abnormalities. For example, single photon emission computed tomography (SPECT) with technetium-99m-hexamethylpropyleneamine oxime (99mTc-HMPAO) has been used to demonstrate abnormal cerebral perfusion. States of cerebral perfusion during the interictal and immediate postictal periods have been reported to correlate with epileptiform foci identified by electroencephalogram (EEG). Between January 1987 and March 1993, we studied 55 pediatric patients with intractable seizures with prolonged video EEG telemetry in the epilepsy monitoring unit, followed by computed tomography, magnetic resonance imaging, and SPECT, before surgery to determine whether SPECT studies with 99mTc-HMPAO improved the accuracy of locating the epileptic focus. Interictal SPECT was performed on all patients, and immediate postictal SPECT (within 10 minutes after seizure ended) on 17 patients monitored in the epilepsy monitoring unit. In 15 (88%) of the 17, the combination of interictal and postictal SPECT studies yielded results corresponding to the EEG abnormality, a result significantly better than that obtained from interictal studies alone: 21 (55%) of 38 (chi 2 = 5.647, P = .0175). SPECT scans showed localized abnormal perfusion in the ipsilateral temporal lobe in all six patients with mesial temporal sclerosis, but precise results were not obtained in cases of dual pathology and neuronal migration disorders. Depiction of cerebral perfusion by interictal and immediate postictal SPECT studies can lead to greater accuracy in the localization of epileptic foci.

Contradictory supranormal nuclear renographic differential renal function: fact or artifact?

We previously reported contradictory supranormal nuclear renographic differential renal function in cases of unilateral hydroureteronephrosis, in which the kidneys with hydroureteronephrosis paradoxically had a greater differential function than the contralateral normal mate, based on diethylenetriaminepentaacetic acid (DTPA) nuclear renography. To evaluate whether DTPA supranormal differential function represented true hyperfunction, patients with DTPA supranormal differential function were evaluated with dimercaptosuccinic acid (DMSA) nuclear renography and the results were compared. A total of 16 patients with unilateral hydronephrosis was identified to have DTPA differential function of 53% or more. They were younger than age 1 year and had never undergone any urological surgery. In all 16 patients the DMSA differential function (mean 51.1%, range 42 to 57%) was lower than their own corresponding DTPA differential function (mean 58.3%, range 53 to 66%, p < 0.0001). In addition, the DMSA differential function was not significantly different from the intuitively anticipated mean of 50% (p = 0.48). The DTPA supranormal differential function identified in our patients was not corroborated by the DMSA differential function. With recent evidence that DMSA differential function may be a better predictor of outcome following relief of unilateral ureteral obstruction consideration should be given to using DMSA as a potentially more relevant method for differential function measurement in the setting of unilateral hydronephrosis. Conversely, until the potential deficiencies of DTPA are fully understood caution should be exercised in the interpretation of DTPA differential function in the setting of hydronephrosis.

Neuroimaging studies in children with temporal lobectomy.

Twenty-eight children with intractable seizures who subsequently underwent a temporal lobectomy were studied by electroencephalogram (EEG), prolonged video EEG telemetry, computed tomography (CT), magnetic resonance imaging (MRI), and single photon emission computed tomography (SPECT) for the localization of epileptogenic foci. MRI showed abnormalities indicating epileptogenic foci in 21/25 patients and a increased signal intensity in 7/11 patients with mesial temporal sclerosis (MTS). SPECT showed corresponding abnormalities in 17/22 patients, including an interictal decrease in regional cerebral blood flow corresponding to the epileptogenic zone in 15. CT showed localized abnormalities in 16/28. All 12 patients with benign, slow-growing neoplasms showed an abnormality on CT scan. In children, MRI is essential in localizing epileptogenic abnormalities, especially MTS and cortical dysplasia. SPECT contributes to the localization of epileptogenic foci, which are often coincident with EEG abnormalities, particularly in single pathology. CT depicts benign neoplasms with calcification in the temporal lobe, which are likely to provoke complex partial seizures.

Contradictory supranormal differential renal function during nuclear renographic investigation of hydroureteronephrosis.

We selected a group of children with unilateral hydroureteronephrosis to assess the significance of the differential renal function as determined by nuclear renography as a factor in defining obstructive dilatation. All children who presented to our institution during a 21-month period with unilateral hydroureteronephrosis and a normal contralateral kidney were reviewed. Patients with reflux or bladder pathology were excluded, resulting in 13 evaluable patients younger than age 2 years. Diuretic renography was performed using 99mtechnetium-diethylenetriaminepentaacetic acid in accordance with the well tempered renogram. Ancillary studies included voiding cystourethrography, ultrasonography and/or excretory urography. Of the 13 patients 5 had a differential function greater than 55% in the hydronephrotic kidney. Ten of the 13 patients, including the 5 with supranormal function, underwent surgical correction in the form of ureteral reimplantation for recurrent infections, increasing hydronephrosis, decreasing or decreased function and loss of parenchyma. Stenotic distal adynamic ureteral segments with proximal dilatation were confirmed in all cases. The implication by nuclear renography that a dilated kidney has better function than its morphologically normal counterpart is at variance with any available clinical or experimental data. The etiology of this apparently elevated function is unclear and its presence on a renographic study should not be interpreted as suggestive that the kidney is free of risk of nephron loss. Management decisions should not be based solely on the differential function as determined by nuclear renography.

Comparison of bone and 67Ga scintigraphy in the initial diagnosis of bone involvement in children with malignant lymphoma.

The accurate staging of Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL) in children has important therapeutic and prognostic implications. The presence of secondary bone involvement or primary malignant lymphoma of bone can be determined by both radiographic and radionuclide methods. We wished to compare the relative efficacy of bone and 67Ga scintigraphy for detecting the presence of bone involvement by malignant lymphoma. We evaluated 83 children (mean age 12 years) with histologically proven HD and NHL utilizing bone and 67Ga scintigraphy which were performed prior to treatment. Three of these patients had biopsy proven primary malignant lymphoma of bone. Bone scintigraphy detected 18 bone lesions in 13 patients and 67Ga scintigraphy detected the same 18 bone lesions and in addition 101 extraosseous lesions. Radiography confirmed the presence of focal bone lesions. Both osteolytic and osteoblastic lesions were identified. We conclude that bone scintigraphy is not necessary in the initial staging of children with malignant lymphoma unless there are specific osseous symptoms. 67Ga scintigraphy is the radionuclide method of choice in the initial staging of children with malignant lymphoma.

Influence of ventricular morphology on diastolic filling performance in double-inlet ventricle after the Fontan procedure.

OBJECTIVES: The purpose of this study was to define the influence of dominant chamber morphology on ventricular performance after the Fontan procedure in patients with double-inlet ventricle. BACKGROUND: Previous studies have reported the impact of ventricular morphology on preoperative ventricular performance and surgical outcome. However, the influence on postoperative ventricular performance has not been addressed. METHODS: Twenty-six clinically asymptomatic patients > 1 year after repair (mean age at procedure 6.1 +/- 3.7 years) were evaluated with ventricular cineangiography and radionuclide blood pool studies (18 with a dominant left ventricular morphology [LV group], 8 with a dominant right ventricular morphology [RV group]) and compared with normal control subjects. RESULTS: Ventricular volume, mass and systolic variables were similar between patient groups. In the LV group, however, the mass/volume ratio was significantly elevated compared with values in control subjects (1.11 +/- 0.28, 0.97 +/- 0.19, p < 0.05), whereas this ratio in the RV group (0.90 +/- 0.11) was within the normal range and significantly lower than that in the LV group (p < 0.05). Mean right atrial and pulmonary artery pressures in the RV group were significantly higher than those in the LV group (p < 0.05). Peak filling rates (2.87 +/- 0.70, 2.41 +/- 1.15 and 3.84 +/- 0.51 end-diastolic volume/s [LV and RV groups and control subjects, respectively]) were significantly lower in both groups than in control subjects (p < 0.001), without intergroup difference. CONCLUSIONS: Ventricular filling abnormalities after atrial to pulmonary anastomosis are common regardless of the type of dominant ventricular morphology, and these abnormalities in patients with dominant right ventricular morphology do not coexist with ventricular hypertrophy. Such diastolic abnormalities may be related to either intrinsic myocardial or acquired factors, not to excessive hypertrophy alone. Those differences may become clinically more apparent with longer follow-up and may raise concerns over the long-term course.

Comparison of planar and SPECT 99Tcm-DMSA scintigraphy for the detection of renal cortical defects in children.

Renal cortical scintigraphy with 99Tcm-dimercaptosuccinic acid is an excellent imaging modality for the detection of renal cortical defects in children presenting with vesicoureteric reflux and urinary tract infections. Single photon emission computed tomography (SPECT) has proven increased sensitivity over planar scintigraphy for lesion detection in the heart, liver and brain. However, its role in the evaluation of renal cortical defects compared to planar scintigraphy has not been fully assessed. We wished to determine whether SPECT increases the sensitivity for detecting renal cortical defects in children compared to high-resolution planar scintigraphy. Forty-one children (90 renal units) with underlying vesicoureteric reflux and/or recently treated urinary tract infection (UTI) were evaluated. There was no statistically significant difference in the number of defects detected between a single-head SPECT system and high-resolution planar scintigraphy, 24 and 20, respectively (P = 0.54). There were four defects detected by SPECT not reported by planar scintigraphy; two of these were only seen retrospectively on planar scintigraphy. There were two children with renal cortical defects detected by SPECT and not by planar scintigraphy. All defects defected by planar scintigraphy were detected by SPECT. It is concluded that although the risk of missing renal cortical defects is low when using high-resolution, three-view planar scintigraphy, SPECT should be employed whenever feasible. Single photon emission computed tomography detected renal cortical defects in children not diagnosed by planar scintigraphy and hence patient management may be altered.

Detection of silent coronary artery disease in adolescents and young adults with familial hypercholesterolemia by single-photon emission computed tomography thallium-201 scanning.

Familial hypercholesterolemia (FH), a genetic disease characterized by increased levels of total and low-density lipoprotein cholesterol in the blood, results in a markedly increased incidence of atherosclerosis and coronary artery disease in homozygotes and to a lesser extent in heterozygotes. The purpose of this study was to detect the presence of myocardial ischemia, particularly in heterozygotes, with stress single-photon emission computed tomography thallium-201 scanning and to determine if there were any differentiating variables between heterozygotes with normal and abnormal thallium-201 scans. Fifty-four patients (mean age 16 years; range 8 to 24) with FH were analyzed (4 homozygotes and 50 heterozygotes). Eleven heterozygotes and 3 homozygotes had abnormal thallium-201 scans. Family history, lipid profile, age and sex of heterozygotes with FH did not predict the presence of myocardial ischemia. The mean total cholesterol level in heterozygotes with normal thallium-201 scans was 7.68 +/- 2.29 mmol/liter (297 mg/dl), which was not significantly different from that in heterozygotes with abnormal scans (7.63 +/- 1.07 mmol/liter [295 mg/dl]; p = 0.91). The coronary angiography of 1 homozygote who had an abnormal thallium-201 scan demonstrated a 50% stenosis of the left anterior descending artery. Aggressive, repetitive plasma exchange was then instituted. The 11 heterozygotes with abnormal thallium-201 scans underwent more rigorous dietary and drug therapy. It is concluded that myocardial ischemia with stress in heterozygotes with FH can occur at a young age and that thallium-201 scanning should be performed early as a screening test and to guide patient management.

Ventricular performance before and after Fontan repair for univentricular atrioventricular connection: angiographic and radionuclide assessment.

OBJECTIVES: This study was designed to evaluate changes in ventricular volume, mass and cardiac function before and after creation of an atrial to pulmonary connection in patients with a univentricular atrioventricular connection. BACKGROUND: Intact systolic and diastolic performance is critical for successful establishment of an atrial dependent circulation, and few studies are available comparing cardiac performance before and after creation. METHODS: With the use of radionuclide blood pool imaging and ventricular cineangiography, 54 patients (mean age 6.4 +/- 3.4 years) were studied. Twenty-eight patients were investigated preoperatively and 36 greater than 1 year after repair and compared with a control population. RESULTS: Before operation, end-diastolic volume and wall mass were significantly increased compared with those of control subjects; however, the mass/volume ratio was normal (1.08 +/- 0.31 g/ml for the preoperative group; 0.97 +/- 0.19 for control subjects). Although end-diastolic volume returned to normal after the procedure, wall mass remained elevated and contributed to an elevated mass/volume ratio (1.20 +/- 0.38 g/ml). After the procedure, systemic vascular resistance index was significantly elevated compared with that before surgery or with that of control subjects (1,199 +/- 373, 2,120 +/- 645, 1,556 +/- 275 dynes.s.cm-5.m2: pre- and postrepair and control subjects, respectively). Radionuclide studies demonstrated that preoperative ejection fraction (52 +/- 9, 50 +/- 9, 60 +/- 8%), peak ejection (2.58 +/- 0.66, 2.95 +/- 0.81, 3.73 +/- 0.70 EDV/s) and peak filling rates (2.84 +/- 0.75, 2.75 +/- 0.79, 3.84 +/- 0.51 end-diastolic volumes [EDV/s]) were significantly reduced compared with those of control subjects and remained so after surgery. CONCLUSIONS: These data suggest that systolic and diastolic function is depressed preoperatively in these patients, remains unchanged after the creation of an atrial-dependent circulation and is associated with an increased systemic vascular resistance. Long-term issues addressing preservation of cardiac function need to be prospectively studied.

Evaluation of ventricular diastolic function in normal children using equilibrium radionuclide angiography.

Twenty children, aged 10.3 +/- 4.5 (3.3 to 17.5) years, were studied by equilibrium radionuclide angiography to establish a normal range for diastolic parameters. Ejection fraction (EF), peak ejection rate (PER) and time to peak ejection rate (TPER), peak filling rate (PFR) and time to peak filling rate (TPFR) were obtained from ventricular time-activity curves and their first derivative curves, and PFR and TPFR were considered as the diastolic parameters. Normal ranges obtained were as follows: EF, 60 +/- 8%; PER, 3.73 +/- 0.70 EDV-1; TPER, 109 +/- 25 ms; PFR, 3.84 +/- 0.51 EDVs-1; TPFR, 136 +/- 21 ms. There were significant correlations between PER and EF (P less than 0.001), PFR and EF (P less than 0.05), while age and heart rate had no influence upon these variables. Such normal ranges will be useful for evaluation of systolic and diastolic function in children with heart disease.

Decrease in gastric emptying time and episodes of regurgitation in children with spastic quadriplegia fed a whey-based formula.

The gastric emptying times associated with three whey-based formulas were significantly shorter than that associated with a casein-based formula in nine gastrostomy-fed patients with spastic quadriplegia (p less than 0.001). Patients fed whey-based formulas had significantly fewer episodes of emesis than when they were fed casein-based formula (p less than 0.001). We conclude that whey-based formulas reduce the frequency of emesis by improving the rate of gastric emptying.

Comparison of SPECT, EEG, CT, MRI, and pathology in partial epilepsy.

Twenty children with partial epilepsy who had surgery between the ages of 4 1/2 months and 18 years were studied preoperatively with electroencephalography (EEG), computed tomography (CT), and technetium-99m hexamethylpropyleneamineoxime 99mTc-HmPAO single photon emission computed tomography (SPECT; 20 interictal, 4 postictal). Fourteen had magnetic resonance imaging (MRI). All had an epileptiform focus (12 unilateral, 8 predominantly unilateral) on EEG. The combination of interictal and postictal regional cerebral blood flow (rCBF) abnormalities alone correlated with EEG foci in 16 of 20 patients. Interictal rCBF abnormalities correlated with EEG foci in 14 of 20. CT findings correlated with EEG foci in 14 of 20. MRI findings correlated with EEG foci in 13 of 14. Pathology demonstrated tumor in 6, cortical dysplasia in 4, mesial temporal sclerosis in 3, Sturge-Weber in 2, cavernous hemangioma in 1, Rasmussen encephalitis in 1, porencephalic cyst and gliosis in 1, and cysts found at surgery (but normal histology) in 2. Interictal and postictal SPECT, EEG foci, and CT findings each correlated with the pathology site in 17, 19, and 15 patients, respectively. MRI correlated with pathology site in 13 of 14 patients. Postictal and interictal abnormalities of rCBF correlated with EEG and pathology as frequently as CT. In 5 patients with normal CT scans and in 1 with a normal MRI, postictal and interictal rCBF correlated with EEG and pathology results; however, these 6 patients all had abnormalities on CT or MRI. SPECT, therefore, may be considered a valuable additional diagnostic procedure in the evaluation of epilepsy surgery candidates in that it adds to the evidence of abnormality at the involved site.(ABSTRACT TRUNCATED AT 250 WORDS)

Ventricular function during supine bicycle exercise in univentricular connection with absent right atrioventricular connection.

Fourteen patients with univentricular connection, absent right connection (tricuspid valve atresia) and normally related great arteries underwent rest and supine bicycle exercise equilibrium radionuclide blood pool studies. Ejection fraction, heart rate, systemic blood pressure and oxygen saturation (ear oximetry) were measured. There were 6 male and 8 female patients. Mean age +/- standard error of the mean was 14.5 +/- 1.1 years (range 6.3 to 21.1). Eight patients (group 1) had systemic to pulmonary shunts placed as palliation 8.2 +/- 2.2 years before study and 6 patients (group II) had caval to pulmonary shunts placed 11.8 +/- 1.6 years previously. Ejection fraction at rest was 54 +/- 2% and an abnormal response to exercise (failure of ejection fraction to increase greater than or equal to 5% from rest to peak exercise) was found in 9 of 14 patients. When analyzed separately, ejection fraction at rest in group I was higher than in group II; however, this difference disappeared at peak exercise. There was a significant negative correlation between ejection fraction at peak exercise and the interval since palliative surgery, although it was not apparent at rest. These data suggest that ventricular function is compromised during exercise and that abnormal performance is influenced by long-standing volume overload. Importantly, this abnormal state is concealed at rest and the choice of palliative shunting procedure appears to have little effect on normalizing pump performance.