Operation for partial atrioventricular septal defect: a forty-year review.

BACKGROUND: We describe the long-term outcome of repair of partial atrioventricular septal defect by determining the rates of survival, reoperation, and occurrence of left atrioventricular valve regurgitation, left atrioventricular valve stenosis, left ventricular outflow tract obstruction, and arrhythmia. METHODS: We studied 334 patients who underwent repair of partial atrioventricular septal defect before 1995. RESULTS: The 30-day and 5-, 10-, 20-, and 40-year survival were 98%, 94%, 93%, 87%, and 76%, respectively. Closure of the left atrioventricular valve cleft (P =. 03) and age less than 20 years at operation (P <.001) were associated with better survival. Reoperation was performed for 38 patients (11%). Repair of residual/recurrent left atrioventricular valve regurgitation or stenosis was the most common reason for reoperation. Left ventricular outflow tract obstruction occurred in 36 patients, and 7 patients underwent reoperation to relieve this obstruction. Supraventricular arrhythmias were observed in 58 patients (16%) after the operation. Supraventricular arrhythmias increased with increasing age at primary operation (P =.001). Complete atrioventricular block occurred in 9 patients (3%). Permanent pacemakers were implanted in 11 patients. CONCLUSIONS: Long-term survival after repair of partial atrioventricular septal defect is good. It is important to close the cleft in the left atrioventricular valve. Reoperation for persistent or recurrent left atrioventricular valve malfunction and relief of left ventricular outflow tract obstruction is necessary in approximately 11% of patients.

Percutaneous echocardiographically guided pericardiocentesis in pediatric patients: evaluation of safety and efficacy.

The purpose of this study was to evaluate the safety and efficacy of echocardiographically (echo) guided pericardiocentesis in pediatric patients. Echo-guided pericardiocenteses performed in pediatric patients (age >/=16 years) at the Mayo Clinic between 1980 and 1997 were identified. Presentation, cause and characteristics of the effusion, details of the pericardiocentesis procedure, and outcome were determined by comprehensive chart review supplemented by telephone interviews when necessary. Seventy-three pediatric patients, median age 6.7 years (range 1 day to 16 years), underwent 94 consecutive echo-guided pericardiocenteses for effusions of various causes. Twenty-one (22%) procedures were performed in children younger than 2 years. All but 1 procedure were successful (99%). A mean fluid volume of 237 mL (range 4 to 970 mL) was withdrawn. Only a single attempt was needed for entry into the pericardial space in 87 (93%) procedures. No deaths were associated with the pericardiocentesis procedure. Only 1 major complication occurred (1%), a pneumothorax requiring chest tube reexpansion. Three (3%) minor complications-2 instances of right ventricular puncture and a small pneumothorax-did not require treatment. Extended catheter drainage for a mean of 5.2 +/- 4.5 days (range 1 to 19 days) was used with 30 (32%) of the 94 procedures. For the 52 patients who underwent pericardiocentesis without catheter drainage as the initial management strategy, 18 required 21 repeat pericardiocenteses for recurrence of effusion. In contrast, for the 21 patients who had pericardial catheterization as the initial management strategy, none had recurrences necessitating a repeat procedure (P <.001). Increased utilization of a pericardial catheter was associated with a concomitant decrease in the number of surgical pericardial procedures over the study period. Echo-guided pericardiocentesis was the only therapeutic modality for the management of effusion in 73% of all patients. Echo-guided pericardiocentesis is safe and effective in pediatric patients, including children younger than 2 years. The increasing use of pericardial catheterization in conjunction with this technique was associated with significant reduction of recurrence and decreased frequency of surgical interventions for treatment of pericardial effusion. Echo-guided pericardiocentesis with extended catheter drainage should be considered as primary management strategy for clinically significant pericardial effusions in pediatric patients.

Antiperinuclear factor in juvenile rheumatoid arthritis.

The serological diagnosis of juvenile rheumatoid arthritis (JRA) is difficult, with only 7-10% of patients 19S IgM rheumatoid factor positive. About 60-70% of patients are positive for hidden 19S IgM rheumatoid factor, but this test requires serum separation and is not available in most laboratories. Antiperinuclear factor has been described in both seropositive and seronegative adult patients with rheumatoid arthritis, but has not been thoroughly evaluated in children with JRA. This study determined the diagnostic sensitivity and specificity of antiperinuclear factor in patients with JRA. Serum samples from 64 children with JRA, 24 with systemic lupus erythematosus (SLE), and 24 control subjects were tested for the presence of antiperinuclear factor. A total of 10 (83%) of seropositive, polyarticular onset and six (37%) of seronegative, polyarticular onset patients with JRA were positive for antiperinuclear factor. The occurrence of antiperinuclear factor in five (19%) with pauciarticular onset and one (10%) with systemic onset (JRA) as well as in four (17%) with SLE was not increased compared with the control subjects (1/24 (4%)). These data show an overall diagnostic sensitivity and specificity of 34 and 90% respectively in this group of patients. Although less sensitive than the hidden rheumatoid factor assay, the antiperinuclear factor assay is easier to perform and may contribute to the serological diagnosis of JRA.

Correlation of antiperinuclear factor with antibodies to streptococcal cell-wall peptidoglycan-polysaccharide polymers and rheumatoid factor.

Antiperinuclear factor (APF) has been noted in most seropositive rheumatoid arthritis (RA) and juvenile rheumatoid arthritis (JRA) patients. The nature of the antigen is unknown; however, there are some suggestions that it might be a glycoprotein or proteoglycan. We studied the correlation of APF with antiproteoglycan antibodies and the reactivity of IgM-rheumatoid factor (RF) with the perinuclear antigen. Ten serum samples were separated to IgG, IgM, and IgM-RF enriched fractions. In seven samples, APF was found in the IgG fraction. Only 4 had APF in their IgM rheumatoid factor (RF)-containing fraction. In two of these, APF activity was present solely in the IgM RF fraction and was inhibited by pre-incubation with IgG. Fifty-five JRA patients' sera were also tested for the presence of antibodies to Streptococcal cell wall peptidoglycan-polysaccharide polymers (PG-PSP). 76% of the APF-positive sera were anti-PG-PSP positive and 59% of the APF-negative sera were also anti-PG-PSP negative. Furthermore, 75% of the APF-positive sera lost their APF activity following adsorption to Streptococcal cell wall PG-PSP. Our results show that in JRA sera APF are polyclonal antibodies of both the IgG and IgM classes. Although the presence of APF correlates with RF positivity, and they sometimes may cross-react, many IgM RF-containing fractions do not show APF activity. However, the presence of APF does correlate with anti-PG-PSP positivity and the data suggest cross-reactivity between these two antibodies. This implies antigenic similarity between Streptococcal cell wall PG-PSP and the perinuclear antigen.

Antibody to streptococcal cell wall peptidoglycan-polysaccharide polymers in sera of patients with juvenile rheumatoid arthritis but absent in isolated immune complexes.

Sera of 88 children with juvenile rheumatoid arthritis (JRA) (10 seropositive, polyarticular onset, 29 seronegative, polyarticular onset, 32 pauciarticular onset, and 17 systemic onset) were evaluated for the presence of serum antibodies to streptococcal cell wall peptidoglycan-polysaccharide polymers (PG-PSP). Immune complexes (IC) isolated by the antihuman IgM (HIgM) affinity column method were also evaluated for the presence of antibodies to PG-PSP. Forty-one of 88 patients with JRA (7 of 10 seropositive, polyarticular onset, 11 of 29 seronegative, polyarticular onset, 16 of 32 pauciarticular onset, and 7 of 17 systemic onset) showed elevated levels of antibodies to PG-PSP in their sera. IgM rheumatoid factors (RF) were demonstrated in 70/88 isolated IC fractions of patients with JRA and IgG RF in 7; however, none of the patients demonstrated the presence of antibodies to PG-PSP in their isolated IC fractions from the anti-HIgM affinity column. These data indicate that antibodies are produced to PG-PSP in all JRA onset types, but they are not constituents of isolated IC by the anti-HIgM affinity column method.

IgM rheumatoid factor plaque-forming cells in juvenile rheumatoid arthritis.

Using a direct, plaque-forming cell (PFC) assay with sensitized sheep erythrocytes, lymphocytes that secrete IgM rheumatoid factors (RF) have been detected in the peripheral blood of patients with juvenile rheumatoid arthritis. Of 15 juvenile rheumatoid arthritis patients tested, 8 were seropositive and 7 were seronegative, but 6 of the seronegative patients had hidden 19S IgM-RF. Ten patients (5 seropositive and 5 with hidden 19S IgM-RF) demonstrated RF-PFC in their peripheral blood (range 15 to greater than 200 RF-PFC/10(6) mononuclear cells). Of 11 patients who had active disease, 10 had RF-PFC, and the 4 patients who had inactive disease had no PFC in their peripheral blood. HLA typing of all 15 patients revealed no correlation between the presence of RF-PFC and HLA type.

Search for correlates of resistance to virulent challenge in mice immunized with Coxiella burnetii.

Mice immunized with live phase I or phase II Coxiella burnetii, with killed phase I or phase II organisms or with trichloroacetic acid (TCAE) or phenol (PE) extracts were resistant to intraperitoneal infection with phase I C. burnetii irrespective of whether or not they displayed phase I antibody response at the time of virulent challenge. Increased phagocytosis of purified phase I organisms by blood leukocytes or peritoneal exudate cells (PEC) was noticed only in mice with phase I agglutinating antibodies in their sera or peritoneal washings. Passive transfer of resistance was made possible only by sera containing phase I agglutinating antibodies. Adoptive transfer of immunity by spleen cells, but not by PEC, was achieved providing that these cells were taken from mice immunized with live phase I C. burnetii.