Simple open-heart surgery protocol for sickle-cell disease patients: a retrospective cohort study comparing patients undergoing mitral valve surgery.

OBJECTIVES: Sickle-cell disease (SCD) patients are considered to be at high risk from open-heart surgery. This study assessed the role of a simple sickling-prevention protocol. METHODS: Perioperative non-specific and SCD-specific morbidity and 30-day mortality are investigated in a retrospective cohort study on patients undergoing isolated mitral valve surgery. Patients with and without SCD were compared. In the SCD cohort, a bundle of interventions was applied to limit the risk of sickling: 'on-demand' transfusions to keep haemoglobin levels of around 7-8 g/dl, cardiopulmonary bypass (CPB) with higher blood flow and perfusion temperature, close monitoring of acid-base balance and oxygenation. RESULTS: Twenty patients with and 40 patients without SCD were included. At baseline, only preoperative haemoglobin levels differed between cohorts (8.1 vs 11.8 g/dl, P < 0.001). Solely SCD patients received preoperative transfusions (45.0%). Intraoperative transfusions were significantly larger in SCD patients during CPB (priming: 300 vs 200 ml; entire length: 600 vs 300 ml and 20 vs 10 ml/kg). SCD patients had higher perfusion temperatures during CPB (34.7 vs 33.0°C, P = 0.01) with consequently higher pharyngeal temperature, both during cooling (34.1 vs 32.3°C, P = 0.02) and rewarming (36.5 vs 36.2°C, P = 0.02). No mortality occurred, and non-SCD-specific complications were comparable between groups, but one SCD patient suffered from perioperative cerebrovascular accident with seizures, and another had evident haemolysis. CONCLUSIONS: SCD patients may undergo open-heart surgery for mitral valve procedures with an acceptable risk profile. Simple but thoughtful perioperative management, embracing 'on-demand' transfusions and less-aggressive CPB cooling is feasible and probably efficacious.

Treating Children With Advanced Rheumatic Heart Disease in Sub-Saharan Africa: The NGO EMERGENCY's Project at the Salam Centre for Cardiac Surgery in Sudan.

Rheumatic heart disease is endemic in Sub-Saharan Africa and while efforts are under way to boost prophylaxis and early diagnosis, access to cardiac surgery is rarely affordable. In this article, we report on a humanitarian project by the NGO EMERGENCY, to build and run the Salam Centre for Cardiac Surgery in Sudan. This hospital is a center of excellence offering free-of-charge, high-quality treatment to patients needing open-heart surgery for advanced rheumatic and congenital heart disease. Since it opened in 2007, more than 8,000 patients have undergone surgery there; most of them Sudanese, but ~20% were admitted from other countries, an example of inter-African cooperation. The program is not limited to surgical procedures. It guarantees long-term follow-up and anticoagulant treatment, where necessary. By way of example, we report clinical features and outcome data for the pediatric cohort: 1,318 children under the age of 15, operated on for advanced rheumatic heart disease between 2007 and 2019. The overall 5-year survival rate was 85.0% (95% CI 82.7-87.3). The outcomes for patients with mitral valves repaired and with mitral valves replaced are not statistically different. Nevertheless, observing the trend of patients undergoing valve repair, a better outcome for this category might be assumed. RHD in children is an indicator of poor socio-economic conditions and an inadequate health system, which clearly will not be cured by cardiac surgery alone. Nevertheless, the results achieved by EMERGENCY, with the crucial involvement and participation of the Sudanese government over the years, show that building a hospital, introducing free cardiac surgery, and offering long-term post-operative care may help spread belief in positive change in the future.