Jean S. Kan's research while affiliated with Johns Hopkins University and other places

We examined parental preferences for locus of service delivery for their teenager's congenital heart disease (CHD) and the influence of disease severity, sociodemographic factors, and insurance on these preferences. A consecutive sample of parents of teenagers followed in a pediatric cardiology clinic completed a mailed questionnaire. Disease severity was classified as low (</=1 cardiovascular procedure), moderate (>1 cardiovascular procedure), and high (cyanosis or single ventricle physiology). Eighty-six of 148 parents responded (58%): 40, low severity; 36, moderate severity; and 10, high severity of illness. Parents preferred using primary care providers (PCPs) as a point of first contact for all 11 of 11 general health concerns and 5 of 7 potential cardiovascular-related concerns: chest pain (52%), syncope (73%), seeming seriously ill (79%), sports physical examination (79%), and endocarditis prophylactic antibiotics (94%). Increasing disease severity was significantly associated with preferring cardiologists for 6 of 7 cardiovascular-related concerns. Overall, 58% of parents viewed their care as a PCP-cardiologist comanagement model versus a cardiologist-dominated model. Lower family income (odds ratio [OR]: 1.5; confidence interval [CI]: 1.0-2.2) and severity of illness (OR: 2.1; CI: 1.0-4.4) were associated with a comanagement model of health care versus a cardiologist-dominated model. This study suggests that the majority of parents of teenagers with CHD prefer to use their teenager's PCP for all routine health care needs and many cardiovascular health needs. Severity of illness and family income are positively associated with greater preference for cardiologist care.

We investigated the prevalence of neurological abnormalities and learning problems in a population cohort of children with dextro-transposition of the great arteries (d-TGA) born between January 1, 1981 and July 1, 1990. Fifty-seven of the 60 survivors and 35 siblings in the control group underwent neurodevelopmental assessments. As compared with population norms, children with d-TGA were more likely to have abnormal neurological examination findings, learning disabilities, and behavioral disorders. There was no significant difference in IQ or frequency of abnormal neurological examination results between children undergoing atrial as compared with arterial switch procedures. Compared with their siblings, the children with d-TGA had more neurological findings and learning disabilities. The siblings of children with d-TGA had more learning problems than expected. The findings suggest that ongoing surveillance is indicated for children surviving d-TGA. Furthermore, a familial tendency for learning differences should to be taken into consideration when neurodevelopmental outcomes of various perioperative parameters are examined.

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.

Fourteen children (ages 2 to 14 years) and 1 adult (32 years) have undergone a modification of the Fontan procedure in which an extracardiac lateral tunnel or conduit is used in combination with staged or simultaneous bidirectional Glenn shunt(s). Extracardiac lateral tunnels (n = 9) were constructed using a polytetrafluoroethylene patch (n = 7), pericardial patch (n = 1), or in situ pericardial flap (n = 1). Extracardiac lateral conduits (n = 6) were constructed using nonvalved homografts (n = 2) or polytetrafluoroethylene tube grafts (n = 4). Fenestrations were created in 4 patients (2 each in extracardiac lateral tunnel and extracardiac lateral conduit patients). Aortic cross-clamping was completely avoided in 12/15 patients (aortic cross-clamping in 2 patients for atrial septal defect enlargement and 1 for Damus-Kaye-Stansel procedure). There have been no operative deaths. Prolonged postoperative chest tube drainage (> 2 weeks) has been rare (n = 1). At follow-up (range, 6 to 54 months; mean, 27.5 months), all patients are in New York Heart Association class I or II and remain in normal sinus rhythm. Late protein-losing enteropathy was seen in 1 patient and was successfully treated by percutaneous creation of a stented fenestration from the extracardiac tunnel to the systemic atrium. Late catheterizations reveal unobstructed extracardiac lateral tunnel function and low pulmonary pressures (range, 11 to 13 mm Hg). Advantages of the extracardiac Fontan include (1) avoidance of aortic cross-clamping in most patients, (2) the hemodynamic benefits of total cavopulmonary connection, (3) avoidance of atriotomy and intraatrial suture lines, (4) preservation of sinus rhythm and no arrhythmias at 2 year follow-up, (5) drainage of the coronary sinus to low pressure atrium, (6) allowance for early/late fenestrations, (7) prevention of baffle leaks and intraatrial obstruction, and (8) allowance for growth (tunnel procedures only). We recommend this extracardiac procedure for all suitable patients undergoing surgical conversion to the Fontan circulation.

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. 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. 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). 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.

In the setting of functional single ventricle with pulmonary overcirculation, pulmonary artery banding is frequently used to alleviate symptoms and to prepare for staged repair. At subsequent cavopulmonary anastomosis or Fontan procedure, the pulmonary artery may be ligated at the site of the pulmonary band. This article describes the association of embolic stroke and thrombus in a ligated or divided pulmonary artery stump in three patients with functional single ventricle. These events occurred from 1990 through 1992 among the 1700 inpatient pediatric cardiology admissions at two institutions. The patients, ranging in age from 15 months to 9 years, had cerebral infarctions documented by computed axial tomography scan or magnetic resonance imaging associated with the echocardiographic finding of thrombus in the proximal pulmonary artery stump after the embolic strokes. The strokes occurred 5 days to 5 years after surgery. Two patients had a second infarction within 2 to 5 weeks of the initial stroke. It is concluded that the presence of the ligated pulmonary artery stump may place patients at risk for embolic stroke. Surgical approaches to reduce the risk of thrombus formation should be considered prospectively in this patient group.

To determine the diagnostic accuracy of clinical assessment of heart murmurs in children and specific clinical features that are predictive of cardiac disease. Concurrent case series with pretest-posttest assessment. Pediatric cardiology outpatient clinic. Five full-time academic pediatric cardiologists. For 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. The 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). Clinical 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.