An audit of surgical repair of Tetralogy of Fallot in an African tertiary care centre

Background: For patients undergoing Tetralogy of Fallot (TOF) repair at institutions in sub-Saharan Africa, data on type of surgical repair, operative mortality and important determinants of outcome such as age at operation and development of pulmonary regurgitation (PR) post-repair is scanty. Objective: To describe the outcomes of children diagnosed with TOF who underwent surgical repair at our center with emphasis on post-operative PR.Methods: This was a retrospective cohort study undertaken from 1 January 1994 to 31 December 2003 at Charlotte Maxeke Johannesburg Academic Hospital (CMJAH). The descriptive analysis of the clinical audit was done in 2010. Results: Fifty four (75%) patients were operated upon: 50 (92.5%) had complete repair and 4 (7.4%) had a palliative procedure in the form of a Blalock-Taussig (B-T) shunt. The median age for corrective surgery was 39.5 months. Twenty out of 50 (40%) patients had simple repair, 10 (20%)had repair which included pulmonary valvotomy, 15 (30%) had a transannular patch (TAP) repair with or without monocusp and 5 (10%) had conduit insertion. An evaluation of severe PR among all the repair groups in the immediate post-operative period (<1 year) revealed that 5 out of 15 (33.3%) patients who had TAP repair developed severe PR, whilst no severe PR was noted in the simple, pulmonary valvotomy and conduit repair groups. During the intermediate post-operative period (1 - 5 years), severe PR was documented in 4 (27%) patients with TAP repair, 2 (10%) with simple repair and 2 (20%) in the pulmonary valvotomy group only. In the long term period (>5 years), severe PR was documented in 3 (30%) patients with pulmonary valvotomy, 8 (53%) who had TAP repair, 2 (10%) patients with simple repair including 1 (20%) patient with a xenograft conduit. One out of 50 (2%) surgically corrected patients who had a complex anatomy died in the immediate post-operative period. Twenty eight out of 50 (56%) patients who had complete correction were lost to follow-up. Conclusion: Surgical correction occurs much later in infancy and childhood compared to developed countries. Severe PR is a serious complication strongly associated with all types of surgical repair of TOF and these patients require lifelong follow-up. Despite the small sample size, the operative mortality compares favourably to fi rst world centers

Industrial Developmental Toxicants and Congenital Heart Disease in Urban and Rural Alberta, Canada

The etiology of congenital heart defects (CHD) is not known for many affected patients. In the present study, we examined the association between industrial emissions and CHD in urban and rural Alberta. We acquired the emissions data reported in the Canadian National Pollutant Release Inventory (n = 18) and identified CHD patients born in Alberta from 2003–2010 (n = 2413). We identified three groups of emissions after principal component analysis: Groups 1, 2, and 3. The distribution of exposure to the postal codes with births was determined using an inverse distance weighted approach. Poisson or negative binomial regression models helped estimate associations (relative risk (RR), 95% Confidence Intervals (CI)) adjusted for socioeconomic status and two criteria pollutants: nitrogen dioxide and particulate matter with a mean aerodynamic diameter of ≤2.5 micrometers. The adjusted RR in urban settings was 1.8 (95% CI: 1.5, 2.3) for Group 1 and 1.4 (95% CI: 1.3, 1.6) for both Groups 2 and 3. In rural postal codes, Groups 1 and 3 emissions had a RR of 2.6 (95% CI: 1.03, 7). Associations were only observed in postal codes with the highest levels of emissions and maps demonstrated that regions with very high exposures were sparse

Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow-up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1-30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality

Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow-up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1-30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality