Functional health status of adults with tetralogy of Fallot: matched comparison with healthy siblings.

BACKGROUND: Survival prospects for adults with repaired tetralogy of Fallot (TOF) are now excellent. Attention should therefore shift to assessing and improving functional health status and quality of life. We aimed to assess late functional health status of adults surviving TOF repair by matched comparison to their healthy siblings. METHODS: All 1,693 TOF repairs performed at our institution between 1946 and 1990 were reviewed. A matched comparison was undertaken whereby presumed survivors and their healthy sibling were contacted and asked to complete the Ontario Health Survey 1990 and the 36-Item Short Form Health Survey (SF-36) questionnaire. RESULTS: Both questionnaires were completed by 224 adult survivors and their sibling closest in age. Adults with repaired TOF had lower scores for self-perceived general health status (p < 0.001), were less likely to rate their health as good or excellent (p < 0.001), and had lower SF-36 scores for physical functioning and general health (p = 0.001) than their siblings. However, patients reported similar satisfaction with their lives, similar levels of social participation and support, and were as likely to be in long-term partnerships. Worse physical and mental health scores were associated with older age at surgery and at time of questionnaire completion and recent requirement for noncardiac medication. CONCLUSIONS: Although reporting lower functional health status then their siblings, quality of life and life satisfaction for adults who underwent surgery for TOF during childhood is comparable to that of their siblings without heart defects. Follow-up of younger adults is required to understand current health outcomes attributable to improvements in the management of TOF.

Functional health status in adult survivors of operative repair of tetralogy of fallot.

We aimed to determine late functional health status of the growing adult population with repaired tetralogy of Fallot (TOF). We studied all 840 patients with TOF born from 1927 through 1984 who survived to adulthood (> 18 years of age). Clinical follow-up was by chart review, telephone interview (n = 706), and echocardiographic reports (n = 339). Functional health status was assessed using Short Form-36 (SF-36) surveys (n = 396) indexed to normative data. Risk of reoperation was low (≈ 1%/year) but increased beyond age 40 years. At latest follow-up moderate or severe pulmonary regurgitation was common (54%) and right ventricular outflow tract stenosis presented in 1/3. Consequently, evidence of right ventricular dilatation and dysfunction and tricuspid regurgitation was typical. Left-sided abnormalities were also common: hypertrophy (p < 0.0001) and outflow tract dilation (p < 0.0001) with at least mild aortic regurgitation in > 50%. Cardiorespiratory symptoms were reported in 45% (palpitations 27%, dyspnea 21%, chest pain 17%). SF-36 scores were significantly below normal for 4 physical domains (p < 0.001). Decrements in physical functioning were associated particularly with older age at follow-up (p < 0.0001), associated syndromes/lesions, reoperations, ventricular dysfunction, tricuspid regurgitation, residual septal defects, and cardiorespiratory symptomatology. Echocardiographic abnormalities were more common in older patients (p < 0.0001). All 3 SF-36 domains specific to psychosocial well-being were normal. In conclusion, despite excellent survival prospects, physical compromise is common in adults with repaired TOF. Greater decrements in older patients may reflect late deterioration with advancing age or cohort effects related to historical management. Efforts to limit ventricular and outflow tract dysfunction may translate into improved late functional status.

Comparison of renal function between cyanotic and acyanotic congenital heart disease in children and adolescent.

BACKGROUND: Glomerular and tubular dysfunction can be found in congenital heart disease (CHD) especially in older children and adults. OBJECTIVE: To evaluate the prevalence renal dysfunction and to compare glomerular and tubular function between cyanotic and acyanotic CHD in children and adolescent. Correlations among clinicalfactors, urinary glomerular and tubular markers for kidney injury were also determined. MATERIAL AND METHOD: Renal function was determined by estimated glomerular filtration rate, urine protein/creatinine, urine microalbumin/creatinine, FE Na+, FE Mg2, and urine NAG/creatinine in children and adolescent with CHD. RESULTS: Forty-six patients, 15 cyanotic (group 1), and 31 acyanotic CHD (group 2), were studied. Only the differences of urine NAG/creatinine (median, 3.59 vs. 1.64 unit/gram creatinine; p = 0.008), FE Mg2+ (mean, 5.03 +/- 3.61% vs. 2.48 +/- 1.8%; p = 0.019), and urine protein/creatinine between the two groups were statistically significant (0.16 vs. 0.08; p = 0.001). No significant differences of clinical features, BUN, creatinine, eGFR, diastolic blood pressure, FE Na+, and urine microalbumin/creatinine were found between the two groups. Significantly higher prevalence ofabnormal biochemical markers in group 1 compared to those of group 2:86.6% vs. 43.38% (p = 0.02) for FE Mg2+; 46.6% vs. 9.67% (p = 0.008) for urine NAG/creatinine; 46.6% vs. 6.45% for significant proteinuria (p = 0.003); and 40% and 9.67% (p = 0.042) for microalbuminuria, respectively. The authors found moderate correlation between hemoglobin and functional class of the patients (r = 0.58) and highly negative correlation between oxygen saturation and functional class (r = -0.716). The relationships among other clinical or biochemical makers showed only low correlations. CONCLUSION: Cyanotic CHD patients had more prevalence and higher abnormal biochemical markers for renal dysfunction than those of acyanotic CHD. Their urine protein/creatinine, FE Mg2 and urine NAG/creatinine were higher than those of acyanotic CHD. Only low correlation among biochemical markers was found

Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades.

BACKGROUND: Adult survivors with tetralogy of Fallot constitute a growing population with congenital heart disease. We investigated an inception cohort who underwent surgical repair. We aimed to characterize late hazard or risk for death, and determine the time-related risk of late re-operation and pulmonary valve replacement (PVR). METHODS: All children (n=1181) with tetralogy of Fallot born before 1984 who underwent surgical repair at our institution were included. Follow-up (median 20 years after repair) was obtained from 2003 to 2006 via chart review, clinic consultation and telephone interview. Outcomes were analyzed using parametric and competing risks techniques with bagging. RESULTS: Corrective repair performed from 1960 to 1998 included transannular patch (n=370), right ventricular outflow tract patch (n=326), no patch (n=333) and right ventricular-pulmonary artery conduit (n=54). Overall, 85+/-1% survived to adulthood (age >18 years). Thirty years after repair, survival was 80+/-1%, instantaneous hazard or risk of death was 0.5+/-0.07% per year and half of survivors had undergone surgical re-operation. Surgical era of repair did not influence late risk of death. Therefore, with early surgical mortality <2% since 1985, 20-year survival has improved to 94+/-1%. If trends in late risks match those of the earliest repairs, 40-year survival will be approximately 90% for children repaired in the modern era. Pulmonary atresia variant (n=88) was associated with three-fold higher late risk of death than classic tetralogy of Fallot (n=1069). Presence of associated branch pulmonary artery stenosis or atrioventricular septal defects conferred a less optimal late prognosis. Risk of PVR was low, constant (0.8% per year) and independent of surgical era. Both pulmonary atresia and absent pulmonary valve (n=15) variants were associated with higher risk of late re-operation or PVR. Survival after re-operation or PVR (88+/-3% and 94+/-3% at 20 years, respectively) was excellent. CONCLUSIONS: Surgical progress has not influenced late risks for death, re-operation or PVR in adults with repaired tetralogy of Fallot. Instead, reduction of early surgical mortality to <2% is responsible for excellent late survival >90% overall. The constant risk of PVR is low and independent of repair type. Baseline morphologic features are important determinants of late outcome.

The impact of pulmonary valve replacement after tetralogy of Fallot repair: a matched comparison.

OBJECTIVE: While there are no controlled studies, pulmonary valve replacement (PVR) after late tetralogy of Fallot repair is performed for patients with symptoms, arrhythmia or ventricular dysfunction. We sought to determine the impact of PVR on clinical outcomes. METHODS: In a matched cohort study, 82 patients with PVR after tetralogy of Fallot repair without a history of ventricular arrhythmia were matched and compared with similarly followed non-PVR control subjects. A propensity-score adjusted analysis using repeated measures regression techniques was performed. RESULTS: For the PVR subjects, the mean age was 28 years with a mean duration follow-up of 9 years. Before PVR, these subjects were significantly more likely than matched non-PVR subjects to have had non-ventricular arrhythmias, symptoms, lower functional class, longer QRS duration, lower right ventricular ejection fraction and higher right ventricular pressure, and reduced exercise duration but not aerobic capacity. During follow-up, sudden death occurred in four non-PVR subjects compared with no PVR subjects, with three episodes of ventricular tachycardia in the PVR subjects versus none in the non-PVR subjects (p=0.49). Symptoms and functional class improved in the PVR subjects with no change in the non-PVR subjects (p<0.001). The change in QRS duration was not significantly different between PVR and non-PVR subjects (p=0.48). Oxygen consumption at peak exercise did not significantly change in either group. For PVR subjects, there was a significant qualitative reduction in pulmonary (p<0.001) and tricuspid valve regurgitation (p=0.009) and right ventricular size (p<0.001) and dysfunction (p<0.001) noted on echocardiography. CONCLUSIONS: Symptoms and functional status are improved after late PVR, with a reduction in pulmonary and tricuspid valve regurgitation and right ventricular size and dysfunction. While a significant impact on arrhythmia was not detected, there were no sudden deaths in the PVR subjects.

Congenital right atrial diverticulum, atrial septal defect within the oval fossa, and complex pulmonary valvar obstruction in an infant with chromosome 8(p23.1) deletion.

We describe an unusual right atrial aneurysm, occurring with progressive obstruction of the right ventricular outflow tract and increasing cyanosis, in a nine-month-old boy with chromosome 8(p23.1) deletion. Surgical resection of the diverticulum, and relief of the right ventricular obstruction, was successful, although impairment of right ventricular compliance persists after 19 months follow-up.

Documenting junctional ectopic tachycardia following pediatric open heart surgery.

OBJECTIVE: To determine appropriated documentations for diagnosis junctional ectopic tachycardia (JET) before treatment in post-operative open heart surgery and identify risk factors for post-operative cardiac arrhythmias in children. MATERIAL AND METHOD: The authors performed a retrospective chart review in 277 patients who underwent surgical corrections at British Columbia's Children Hospital from January 1st, 2000 to December 31st, 2001. History, clinical symptoms, complication of surgery and post-operative cardiac arrhythmias were reviewed from medical records. The authors investigated whether JET was being diagnosed accurately and whether it was being adequately documented prior to the initiation of therapy. The authors also identified risk factors that were associated with JET. All documentations before treatment were reviewed by Pediatric cardiologists to confirm diagnosis. RESULTS: Although the diagnostic accuracy (84%), sensitivity (87%), and specificity (84%) are high, a significant number of patients with post-operative arrhythmias were treated without adequate documentation of the arrhythmia. The documentation of arrhythmias in the Intensive Care Unit was largely limited to rhythm strips, with very few 12-lead ECGs and wire studies performed to assist with the diagnosis. CONCLUSION: The presented data indicates that, in this critically-ill population, there was an unacceptable number of patients with post-operative arrhythmias who may have been treated inappropriately. It is very important to emphasize the interpretation of wire studies, an investigation normally done in a critical care setting and whose interpretation is very important to the accurate diagnosis of pediatric arrhythmias.

Sensitivity of clinical assessment in term neonates by general pediatric residents.

OBJECTIVES: To determine the accuracy of physical examination in detecting congenital heart diseases by pediatric residents and identify risk factors of congenital heart diseases. MATERIAL AND METHOD: Five hundred term infants (GA > or = 37wks) who were born at Phramongkutklao Hospital from July 1st, 1999 to June 3th, 2000 were examined by pediatric residents and pediatric cardiologists within the first week of life. The echocardiography was performed in all 500 infants for definite diagnosis. The accuracy of physical examination was determined by comparing with echocardiographic finding and presented as sensitivity and specificity. RESULTS: Congenital heart diseases were found in 18 of 500 term infants (3.6%). The physical examination by pediatric residents and pediatric cardiologists showeds a sensitivity of 39% and 94% and the specificity of 98% and 97%, respectively. After excluding spontaneously closed PDA within 2 weeks, the first and second most common congenital heart diseases in infants were Ventricular septum defect (VSD) (44%) and Patent ductus arteriosus (PDA) (33%). The positive other cardiovascular signs, such as cyanosis or abnormal pulses with heart murmur were significantly associated to diagnosis of congenital heart diseases. CONCLUSION: Although echocardiography is the major tool to determine the definite diagnosis, most congenital heart diseases can be well detected during physical examination by skilled physicians. As a result, the value of physical examination of the cardiovascular system should be emphasized in training general pediatricians concerning lower costs and early detection of congenital heart diseases.