Hypothermic cardiopulmonary bypass without exchange transfusion in sickle-cell patients: a matched-pair analysis.

OBJECTIVES: Sickle-cell patients undergo cardiopulmonary bypass (CPB) surgery in our institution without perioperative exchange transfusion. We sought to determine whether this protocol increased mortality or important sickle-cell-related complications. METHODS: We adopted a 1:1 matched-pair case-control methodology to evaluate the safety of our protocol. Sickle-cell patients who underwent CPB between January 1995 and January 2014 were matched with haemoglobin AA (HbAA) controls according to sex, age, weight and type of cardiac procedure. RESULTS: Thirty-three sickle-cell patients (21 HbAS, 7 HbSS and 5 HbSC) underwent CPB surgery using our institutional protocol. Sickle-cell patients and controls were similar according to the matching criteria. Preoperatively, haemoglobin SS (HbSS) and haemoglobin SC (HbSC) patients were anaemic (8.5 ± 1.4 vs 13.5 ± 1.9 g/dl; P <0.01 and 11.0 ± 0.6 vs 12.7 ± 0.9 g/dl; P = 0.01, respectively). Operative procedures included valve repair and replacement (12) as well as repair of congenital cardiac malformations (21). The duration of CPB and lowest CPB temperatures was similar for sickle-cell patients and controls. Systemic hypothermia (23.8-33.5°C), aortic cross-clamping, cold crystalloid antegrade cardioplegia and topical hypothermia were used in sickle-cell patients without complications. There was no acidosis, hypoxia or low cardiac output state. No mortality or important sickle-cell-related complications occurred. Although blood loss was similar between sickle-cell patients and controls, HbSS (unlike HbAS and HbSC) patients required more blood transfusion than controls (30.0 ± 13.3 vs 10.8 ± 14.2 ml/kg; P = 0.02) to counter haemodilution and replace blood loss. In-patient stay was similar for sickle-cell patients and controls. CONCLUSIONS: Perioperative exchange transfusion is not essential for a good outcome in sickle-cell patients undergoing CPB. A simple transfusion regimen to replace blood loss is safe in HbSS patients; blood transfusion requirements for HbSC and HbAS patients undergoing CPB are similar to those of matched HbAA controls. The use of systemic hypothermia during CPB does not increase sickle-cell-related complications. Cold crystalloid cardioplegia and topical hypothermia provide safe myocardial protection without the need for more sophisticated measures.

Strictly-posterior thoracotomy: a minimal-access approach for construction of the modified Blalock-Taussig shunt in West African children.

INTRODUCTION: In resource-poor settings, the modified Blalock-Taussig shunt (MBTS) is often performed for symptomatic relief of Fallot's tetralogy. From September 2011, we adopted the strictly posterior thoracotomy (SPOT), a minimal-access technique for the MBTS and report the cosmetic advantages in this communication. METHODS: We retrospectively analyzed the records of consecutive patients in whom the SPOT approach was used to construct the MBTS. Study end-points were early mortality, improvement in peripheral oxygenation, morbidity, and the cosmetic appeal. RESULTS: Between September 2011 and January 2013, 15 males and 8 females, median age 4 years (1.3 - 17 years) and weight 13 kg (11 - 54 kg) underwent the MBTS through the SPOT approach. The polytetrafluoroethylene grafts used ranged from sizes 4 - 6mm (median 5mm). The median preoperative SpO2 was 74% (55% - 78%), increasing to a postoperative median value of 84% (80% - 92%). Shunts were right-sided in 22 patients and left-sided in one. There were no shunt failures. Hospital stay ranged from 7 - 10 days. There was one early death (4.3%), and two postoperative complications (re-exploration for bleeding and readmission for drainage of pleural effusion). The surgical scars had excellent cosmetic appeal: they ranged from 5-10 cm in length; all were entirely posterior and imperceptible to the patient. CONCLUSION: The SPOT approach represents a safe and cosmetically superior alternative to the standard posterolateral thoracotomy, the scar being imperceptible to the patient. The excellent cosmetic appeal and preservation of body image makes this approach particularly attractive in children and young adults.

Massive intrathoracic lipoma: a report of two cases, one being congenital.

Massive intrathoracic lipomas are uncommon. Few cases have been reported worldwide. We report two cases, one of which was congenital. They were managed by thoracotomy and complete excision, with excellent outcomes.

Hepatic venous occlusion during cardiopulmonary bypass in patients with heterotaxy syndrome: a safe but underutilized option.

Direct intracardiac drainage of separate right and left hepatic veins remote from each other (independent hepatic veins) in heterotaxy patients complicates procedures requiring cardiopulmonary bypass (CPB). Temporary occlusion of such independent hepatic veins during CPB is an alternative to cannulation but is rarely used because of concerns about acute congestive hepatopathy. Consequently, temporary single hepatic venous occlusion has not been well described as a safe and simple alternative to hepatic venous cannulation during CPB. We report 2 patients with the polysplenia variant of heterotaxy in whom independent hepatic veins were safely occluded for 55 and 86 minutes, respectively, in the course of intracardiac repair. Temporary hepatic venous occlusion simplified the CPB technique and minimized clutter of the operative field. The intrahepatic hemodynamics during CPB using temporary hepatic venous occlusion is illustrated.

Divided left atrium (cor triatriatum) in the setting of common atrium.

Cor triatriatum is commonly used to describe the congenital malformation in which an anomalous fibromuscular shelf divides the left atrium. We report the successful surgical repair of an extremely rare combination of divided left atrium, virtual absence of the interatrial septum, and anomalous right pulmonary venous connection in a 7 year-old girl. Preoperative transthoracic echocardiography could not detect the dividing left atrial shelf. In this report, we point out the limitations of transthoracic echocardiography in the diagnosis of atypical cases of so-called cor triatriatum and highlight errors in the scientific literature resulting from the variance in terminology ascribed to congenital division of the left atrium.