Context Patients with chronic fatigue syndrome (CFS) are more likely than healthy persons to develop neurally mediated hypotension (NMH) in response to prolonged orthostatic stress.Objective To examine the efficacy of fludrocortisone acetate as monotherapy for adults with both CFS and NMH.Design Randomized, double-blind, placebo-controlled trial conducted between March 1996 and February 1999.Setting Two tertiary referral centers in the United States.Patients One hundred individuals aged 18 to 50 years who satisfied Centers for Disease Control and Prevention criteria for CFS and had NMH provoked during a 2-stage tilt-table test. Eighty-three subjects had adequate outcome data to assess efficacy.Intervention Subjects were randomly assigned to receive fludrocortisone acetate, titrated to 0.1 mg/d (n=50) or matching placebo (n=50) for 9 weeks, followed by 2 weeks of observation after discontinuation of therapy.Main Outcome Measure Proportion of subjects in each group with at least a 15-point improvement on a 100-point global wellness scale.Results Baseline demographic and illness characteristics between the groups were similar; CFS had been present for at least 3 years in 71%, Using an intention-to-treat analysis, 7 subjects (14%) treated with fludrocortisone experienced at least a 15-point improvement in their wellness scores compared with 5 (10%) among placebo recipients (P=.76). No differences were observed in several other symptom scores or in the proportion with normal follow-up tilt test results at the end of the treatment period.Conclusions In our study of adults with CFS, fludrocortisone as monotherapy for NMH was no more efficacious than placebo for amelioration of symptoms, Failure to identify symptomatic improvement with fludrocortisone does not disprove the hypothesis that NMH could be contributing to some of the symptoms of CFS. Further studies are needed to determine whether other medications or combination therapy are more effective in treating orthostatic intolerance in patients with CFS.

We describe a patient with protein-losing enteropathy who presented 6 months after undergoing a modified Fontan operation. After failing to respond to medical therapy, the Fontan tunnel was fenestrated by catheter intervention with immediate improvement and resolution of hypoproteinemia and enteric protein loss.

OBJECTIVE:Fibromyalgia is a common but poorly understood problem characterized by widespread pain and chronic fatigue. Because chronic fatigue has been associated with neurally mediated hypotension, we examined the prevalence of abnormal responses to upright tilt table testing in 20 patients with fibromyalgia and 20 healthy controls.METHODS:Each subject completed a symptom questionnaire and underwent a three stage upright tilt table test (stage 1:45 minutes at 70 degrees tilt; stage 2, 15 minutes at 70 degrees tilt with isoproterenol 1-2 micrograms/min; stage 3, 10 minutes at 70 degrees tilt with isoproterenol 3-4 micrograms/min). An abnormal response to upright tilt was defined by syncope or presyncope in association with a drop in systolic blood pressure of at least 25 mm Hg and no associated increase in heart rate.RESULTS:During stage 1 of upright tilt, 12 of 20 fibromyalgia patients (60%), but no controls had an abnormal drop in blood pressure (P < 0.001). Among those with fibromyalgia, all 18 who tolerated upright tilt for more than 10 minutes reported worsening or provocation of their typical widespread fibromyalgia pain during stage 1. In contrast, controls were asymptomatic (P < 0.001).CONCLUSION:These results identify a strong association between fibromyalgia and neurally mediated hypotension. Further studies will be needed to determine whether the autonomic response to upright stress plays a primary role in the pathophysiology of pain and other symptoms in fibromyalgia.

Two pediatric cases of cervical aortic arch with aneurysm formation are reported. Only female patients with cervical aortic arch have developed aneurysms, which may influence risk counseling of such patients.

OBJECTIVETo determine the diagnostic accuracy of clinical assessment of heart murmurs in children and specific clinical features that are predictive of cardiac disease.DESIGNConcurrent case series with pretest-posttest assessment.SETTINGPediatric cardiology outpatient clinic.PARTICIPANTSFive full-time academic pediatric cardiologists.MEASURESFor each of 222 consecutive patients who were seen for first-time evaluation of a heart murmur, the clinical findings and diagnostic impressions were recorded after clinical assessment. The results of electrocardiograms and echocardiograms were then reviewed, and changes in diagnostic impressions were recorded and compared with the original impressions.RESULTSThe prevalence of cardiac disease was 33%. Clinical assessment differentiated those patients with pathologic murmurs with a sensitivity of 92%, specificity of 94%, positive predictive value of 88%, and negative predictive value of 96%. If diagnostic uncertainty was considered an indication for echocardiography, then sensitivity and specificity increased to 97% and 98%, respectively. Missed disease included only trivial or minor lesions. Clinical features that were independently predictive of the presence of disease included murmurs that were pansystolic (odds ratio [OR], 54.0), grade 3 or more in intensity (OR, 4.84), heard best at the left upper sternal border (OR, 4.24) and harsh in quality (OR, 2.37), and the presence of an abnormal second heart sound (OR, 4.09) and an early or midsystolic click (OR, 8.35).CONCLUSIONSClinical assessment by a pediatric cardiologist is sufficient to distinguish pathologic from innocent heart murmurs. A genetic approach by using specific clinical features that are independently associated with disease may have some practical utility to noncardiologists.

Between January 1962 and December 1991, 179 children less than 1 year of age underwent repair of coarctation of the aorta. Group I (1962 to 1971) consisted of 19 patients, group II (1972 to 1981) of 57 patients, group III (1982 to 1991) of 103 patients. Neonates (< 30 days old) made up 60% of group I, 57% of group II, and 70% of group III. The proportion of infants with associated complex cardiac abnormalities was 7% in group I, 25% in group II, and 39% in group III. Techniques of repair included resection with end-to-end anastomosis (n = 65), subclavian flap repair (n = 85), patch aortoplasty (n = 18), and other procedures (n = 11). The early mortality (< 30 days) was lowest in group III (group I, 21%; group II, 21%; and group III, 7%; p < 0.05), but the late mortality was similar in all groups (group I, 11%; group II, 13%; and group III, 15%). The overall actuarial survival was 57.7% +/- 0.15% at 27.1 years in group I, 65.7% +/- 0.07% at 19.7 years in group II, and 77.5% +/- 0.04% at 9.3 years in group III (p = not significant). Twenty-five restenoses requiring intervention occurred in 23 patients, for an overall restenosis rate of 16.4%. The incidence of restenosis was 23% for the patients who underwent end-to-end anastomosis, 11% for those who underwent subclavian flap repair (p < 0.1), and 27% for those who underwent patch aortoplasty (p < 0.01). Balloon angioplasty was successful in relieving 11 of the 12 restenoses in groups II and III.(ABSTRACT TRUNCATED AT 250 WORDS)

This study sought to determine the extent of anxiety and misperceptions about heart murmurs for consenting parents of 182 children referred for first-time pediatric cardiology assessment (including echocardiography) of a heart murmur. From questionnaires completed before assessment, 22% of parents indicated that they were extremely concerned, and only 16% could define a heart murmur as a sound made by the heart. From 1-month follow-up questionnaires obtained from parents of children without heart disease at assessment, 10% continued to believe that their child had a heart problem. Cardiology assessment may not provide complete reassurance to all families and additional interventions may be necessary.

Autoantibody, HLA studies and C4 phenotypes were performed on twins discordant for isolated congenital heart block. Serum from the mother and cord blood from the infants revealed Ro(SSA) and La(SSB) antibodies in all three sera. No significant difference in Ro(SSA) antibody titre was noted in the cord blood of either twin when compared with maternal titres, as detected by a sensitive ELISA assay. The infants' mother was HLA-DR3 positive. Both infants had identical HLA and C4 phenotypes. Immunoblot analysis revealed that sera from both mother and infants reacted with the 52-kDa Ro(SSA) macromolecule. Quantitative cord blood IgM levels were not elevated in either twin. This study indicates that placental transfer of anti-Ro(SSA) or anti-La(SSB) alone to the fetus is not sufficient for the expression of congenital complete heart block. Vie conclude from this experiment of Nature that there must be a second event determining which infant develops complete heart block, but this is unknown at present.

After its successful introduction in 1982 for the treatment of congenital heart lesions, percutaneous balloon dilatation has been applied to many stenotic lesions found in preoperative and postoperative congenital heart disease.1,2 There have been rare reports of this technique's application to surgical aortopulmonary connection with varying results. Fischer et al3 described successful dilatation of a Blalock-Taussig anastomosis, and Gibbs et al4 described balloon dilatation of a Waterston anastomosis after which pulmonary edema developed and the patient died 12 hours after the procedure. A recent case report of long-term follow-up after initially successful balloon dilatation of a variant of a Blalock-Taussig shunt documented late remodeling at the angioplasty site and distal pulmonary hypertension.5 We describe the successful long-term outcome after balloon dilatation of a stenotic Waterston anastomosis.

BACKGROUND:The objective of this study was to determine the long-term outcome of patients after percutaneous balloon pulmonary valvuloplasty (BPV) treatment of congenital pulmonary valve stenosis.METHODS AND RESULTS:This study represents a case series with duration (mean +/- SD) of follow-up of 4.6 +/- 1.9 years. Forty-six patients with a median age of 4.6 years (range, 3 months to 56 years) had BPV at one academic institution between June 1981 and December 1986. Mean peak systolic pressure gradients from the right ventricle to the pulmonary artery were as follows: before BPV, 70 +/- 36 mm Hg; immediately after BPV, 23 +/- 14 mm Hg; at intermediate follow-up by cardiac catheterization or Doppler echocardiography at less than 2 years after BPV, 23 +/- 16 mm Hg (n = 33); and at long-term follow-up by Doppler at more than 2 years after BPV, 20 +/- 13 mm Hg (n = 42). BPV acutely reduced the gradient to less than 36 mm Hg for 41 of 46 (89%) patients. Available gradients at long-term follow-up were less than 36 mm Hg for 36 of 42 (86%) patients without additional procedures. A patient age of less than 2 years at the initial BPV was a significant risk factor for gradients over 36 mm Hg at follow-up.CONCLUSIONS:BPV provides long-term relief of pulmonary valvular obstruction in the majority of patients. Close follow-up of patients who require BPV at less than 2 years of age is warranted.

Multiple indications for fetal echocardiography have been proposed that includes polyhydramnios. In this report, the prenatal diagnosis of tetralogy of Fallot with absent pulmonary valve and absent ductus arteriosus was made in a patient presenting with polyhydramnios. Tracheobronchial and esophageal compression secondary to gross dilation of the pulmonary artery is considered the etiology of the polyhydramnios, and in the case of this structural defect is an indicator of poor prognosis.

Three hundred three fetuses were studied with fetal echocardiography and 21 were diagnosed with congenital heart disease (CHD). Indications for study, prenatal findings, and postnatal outcomes were examined in all fetuses studied. The most common indications for referral were previous family history and fetal dysrhythmia. Of 189 fetuses studied for these indications, six had CHD. Indications with the highest proportion of abnormal studies included an abnormal four-chamber view of the heart (50% abnormal), fetal hydrops (30.8% abnormal), and polyhydramnios (25% abnormal). Five of 21 with CHD had chromosomal defects and eight had extracardiac anomalies. Two of the 21 fetuses with CHD survived. Present referral indications led to the diagnosis of the most severe lesions, yet reassurance is provided to many couples with normal fetuses. Fetal echocardiography is important in the care of the fetus at risk for CHD.

Complications after successful angioplastic dilatation of pulmonary artery narrowings have been well documented, and are known to include rupture and death.1 Transient unilateral pulmonary edema, resolving within a few days after dilatation has occurred.2 Successful dilatation of stenosed Blalock-Taussig (B-T) shunts, both classic and modified, has been performed for palliation in selected patients.2–4 This report notes a late complication of shunt angioplasty—the development of excessive pulmonary blood flow.

Data on 111 lesions, in addition to pulmonary valve, aortic valve, branch pulmonary stenosis, native coarctation and recoarctation, were submitted to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Because there was a very heterogenous group of lesions both in types of lesions and techniques of dilation, only minimal data can be offered concerning the efficacy of the dilation of any or all of these lesions. The registry data do, however, demonstrate the safety of these dilations compared to alternative surgical therapy.

The preoperative evaluation of pulmonary sequestration requires delineation of the arterial supply and venous drainage of both the sequestered segment and the neighboring lung. In this case magnetic resonance imaging was the sole modality which demonstrated all components of the arterial and venous circulations of a sequestration and the remainder of the ipsilateral lung.

Alterations in ventricular loading conditions lead to changes in action potential duration and arrhythmias via contraction-excitation feedback; a decrease in load leads to prolongation of repolarization. To determine whether changes in right ventricular load alter ventricular repolarization in man, the corrected QT interval, a measure of overall ventricular repolarization, was measured in 32 patients before and after valvuloplasty for pulmonary stenosis. Right ventricular systolic pressure decreased (82.5 +/- 30.7 to 40.5 +/- 9.5 mm Hg, p less than .001) and the QTc increased concurrently (409.1 +/- 24.3 to 440.7 +/- 28.0 msec, p less than .001) after successful valvuloplasty. The increase in QTc was most marked for those patients with a greater than 30 mm Hg decrease in right ventricular pressure (40.0 +/- 23.3 vs 16.3 +/- 21.3 msec, p = .006). In a subset of seven patients in whom monophasic action potentials were recorded, monophasic action potential duration, a measure of local repolarization, was prolonged (230.0 +/- 24.3 vs 216.9 +/- 21.9, p less than .001) after successful valvuloplasty, confirming that the QTc prolongation reflected changes in local ventricular repolarization. In addition, during nine acute right ventricular outflow tract occlusions in a subset of six patients, monophasic action potential duration shortened (206.6 +/- 17.6 vs 221.7 +/- 20.9 msec, p less than .01) and early afterdepolarizations developed consistent with contraction-excitation feedback. These data suggest that, in humans, changes in mechanical load are associated with changes in ventricular repolarization consistent with contraction-excitation feedback.