Right-sided Weakness in a Rwandan Patient with Unrepaired Tetralogy of Fallot

Background

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease encountered in pediatrics. Long-term survival after surgical repair has improved; however, reported mortality rates in unrepaired TOF are significant. Associated complications include neurological sequelae, most frequently brain abscess and stroke. In countries without early intervention for congenital heart disease including TOF, delayed presentations and complications require recognition by healthcare workers.

Case presentation

A 22 year old male with a history of unrepaired TOF presented to Rwanda’s tertiary university hospital, University Teaching Hospital of Kigali (CHUK) with acute right-sided hemiparesis. Diagnostic imaging identified a left-sided brain lesion consistent with brain abscess and cardiac mass concerning for an endocardial vegetation. He was managed with intravenous antibiotics, but subsequently died due to complications of septicemia.

Conclusion

In countries where surgical repair of TOF is not available, early recognition and medical management are key in temporizing the development of devastating sequelae. Describing the prevalence of CHD in Rwanda is urgent, requiring further research by which effective prevention and treatment strategies can be developed.

Tetralogy of Fallot Repair in Developing Countries: International Quality Improvement Collaborative.

Isolated reports from low- and middle-income countries (LMIC) for surgical results in Tetralogy of Fallot (TOF) are available. The International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) seeks to improve surgical results promoting reductions in infection and mortality in low and middle income countries.All cases of TOF in the IQIC database performed between 2010 and 2014 at 32 centers in 20 LMIC were included. Excluded from the analysis were TOF with any associated lesions. A logistic regression analysis was performed to identify risk factors for in-hospital mortality after surgery for TOF.A total of 2164 patients were identified. There were 1,839 initial primary repair, 200 with initial systemic to pulmonary artery shunt and 125 underwent secondary repair following initial palliation. Overall mortality was 3.6% (78/2164), initial primary repair was 3.3% (60/1839), initial systemic to pulmonary artery shunt 8.0% (16/200) and secondary repair 1.6% (2/125); (p = 0.003). Major infections occurred in 5.9% (128/2164) of the entire cohort. Risk factors for mortality following initial primary repair were oxygen saturation < 90 and weight/body mass index for age below the 5th percentile (p < 0.001). Over half, 54% (991/1839) underwent initial primary repair after 1 year of age. Older age at initial primary repair was not a risk factor for mortality (p = 0.21).In LMIC, TOF patients are often operated after age 1 year, unlike developed countries older age is not a risk factor for mortality. Nutritional and hypoxemic status were associated with higher mortality and infections. This information fills a critical knowledge gap for surgery in LMIC .