Outcomes of Norwood procedure with hypoplastic left heart syndrome: Our 12-year single-center experience.

Background: In this study, we aimed to analyze the predictors and risk factors of mortality in patients who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome. Methods: Between January 2009 and December 2020, a total of 139 patients (95 males, 44 females) who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome in our center were retrospectively analyzed. Results: The median birth weight was 3,200 (range, 3,000 to 3,350) g and the median age at the time of operation was seven (range, 5 to 10) days. Pulmonary flow was achieved with a Sano shunt in the majority (72%) of patients. Survival rate was 41% after the first stage. Reoperation for bleeding (p=0.017), reoperation for residual lesion (p=0.011), and postoperative peak lactate level (p=0.029), were associated with in-hospital mortality. Nineteen (33%) of 57 patients died before the second stage. Thirty-three (58%) patients underwent second stage, and survival after the second stage was 94%. Thirteen patients underwent third stage, and survival after the third stage was 85%. Estimated probability of survival at six months, and one, two, three, and four years were 33%, 33%, 25%, 25%, and 22% respectively. Conclusion: Hospital and inter-stage mortality rates are still high and this seems to be the most challenging period in term of survival efforts of the patients with hypoplastic left heart syndrome. Early recognition and reintervention of anatomical residual defects, close follow-up in the inter-stage period, and the accumulation of multidisciplinary experience may help to improve the results to acceptable limits.

A dramatic learning curve of extracardiac Fontan operation in the modern era.

BACKGROUND: This study aimed to describe the learning curve of the extracardiac Fontan procedure in a single center and to analyze the changes in clinical applications and outcomes. METHODS: A retrospective chart review of all extracardiac Fontan patients in a single tertiary care center was undertaken. Patients with a diagnosis of hypoplastic left heart syndrome and those who had undergone a lateral tunnel modification, intra/extracardiac Fontan, Kawashima procedure, or inferior vena cava-to-azygous vein connection were excluded from the analysis. RESULTS: Between May 2004 and February 2018, data of 159 extracardiac Fontan patients were analyzed. The median age was 5.5 years (range 4.5-8.2 years). Based on a cumulative sum analysis, a hinge point was determined to divide the cohort into 2 phases. Phase 1 ( n = 70) represented the first learning phase and phase 2 ( n = 89) represented the later phase. Mortality decreased in phase 2 (2/89; 2%) compared to phase 1 (10/70; 14%; p = 0.004). Two (3%) patients had extracorporeal membrane oxygenation in phase 1, and 5 (6%) in phase 2 ( p = 0.47). More patients in phase 2 underwent a prior bidirectional Glenn procedure (83/89 vs. 57/70; p = 0.02), fenestration (80/89 vs. 9/70; p < 0.001), and pulmonary artery reconstruction (37/89 vs. 2/70; p < 001). CONCLUSIONS: This study shows that increased use of extracorporeal membrane oxygenation, strict implementation of the three-stage management plan, routine fenestration, and a low threshold for pulmonary artery reconstruction may be associated with decreased mortality in the extracardiac Fontan procedure.

Randomized comparison between mild and moderate hypothermic cardiopulmonary bypass for neonatal arterial switch operation.

OBJECTIVES: To compare neonates receiving arterial switch operation (ASO) either with mild or moderate hypothermic cardiopulmonary bypass. METHODS: Forty neonates undergoing ASO were randomized to receive either mild (Mi > 32 °C, n = 20) or moderate (Mo > 26 °C, n = 20) hypothermic cardiopulmonary bypass (CPB) between April 2007 and June 2010. All patients were diagnosed with simple transposition of the great arteries. Mean age (Mi: 8.32 ± 4.5 days, Mo: 7.54 ± 5.0 days, P = 0.21) and body weight were similar in both groups (Mi: 3.64 ± 0.91 kg, Mo: 3.73 ± 0.84 kg, P = 0.14). Follow-up was 3.1 ± 2.5 years for all patients. RESULTS: Lowest perioperative rectal temperature was 33.5 ± 1.4 °C (Mi) versus 28.2 ± 2.1 °C (Mo) (P < 0.001). All patients safely weaned from CPB required lower doses of dopamine (Mi: 5.1 ± 2.4 µg/kg min, Mo: 6.5 ± 2.1 µ/kg min, P = 0.04), dobutamine (Mi: 7.2 ± 2.5 µg/kg min, Mo: 8.6 ± 2.4 µ/kg min, P = 0.04) and adrenalin (Mi: 0.02 ± 0.02 µg/kg min, Mo: 0.05 ± 0.03 µ/kg min, P = 0.03) in mild hypothermia group. Intraoperative blood transfusion (Mi: 190 ± 58 ml, Mo: 230 ± 24 ml, P = 0.03) and postoperative lactate levels (Mi: 2.7 ± 0.9 mmol/l, Mo: 3.1 ± 2.2 mmol/l, P = 0.02) were lower under mild hypothermia. Secondary chest closure was performed in 30% (Mi) versus 35% (Mo) (P = 0.65). Duration of inotropic support (Mi: 7 (4-11) days, Mo: 11 (7-15) days, P = 0.03), time to extubation (Mi: 108 (88-128) h, Mo: 128 (102-210) h, P = 0.04), lengths of intensive care unit (ICU) stay (Mi: 9 (5-14) days, Mo: 12 (10-18) days, P = 0.04) and hospital stay (Mi: 19 (10-29) days, Mo: 23 (15-37) days, P = 0.04) were significantly shorter under mild hypothermia. Two-year freedom from reoperation was 100% for both the groups. CONCLUSIONS: The ASO under mild hypothermia seemed to be beneficial for pulmonary recovery, need for inotropic support and length of ICU and hospital stay. No worse early- or intermediate-term effects of mild hypothermia were found.

Multiple transcatheter interventions in the same session in congenital cardiopathies.

BACKGROUND: To investigate the methods of percutaneous transcatheter interventions for combined congenital heart disease and to evaluate its efficacy in children. MATERIALS AND METHODS: Thirty cases (ages 3 days-13.5 years, body weight 3-35 kg) that underwent two multiple transcatheter interventions for combined or solitary congenital heart disease were retrospectively analyzed and presented. RESULTS: A total of 61 transcatheter interventions were performed in 30 patients as combined procedures. In 29 patients, two different procedures were combined in the same session, in remaining one patient, combination of three interventions were performed in the same catheter session. Interventions performed in combined procedures were as follows: Coarctation balloon angioplasty in 12 cases, pulmonary balloon valvuloplasty in 10, PDA coil embolization in 10, aortic balloon valvuloplasty in 8, VSD closure in 5, ASD closure in 4, ductal stent implantation in 4, palliative pulmonary balloon valvuloplasty in 3, recanalization and angioplasty of the systemic-pulmonary shunt in 2, balloon atrial septostomy in one, aortic coarctation stent implantation in one, coil embolization of a pulmonary lobar sequestration in one and pulmonary valve perforation plus pulmonary balloon valvuloplasty in one were performed as first or second procedure. There was no mortality or major morbidity in relation to combined procedures. CONCLUSION: Multiple transcatheter interventions in the same session are feasible, safe and effective with satisfactory good results. Second intervention may be performed as complementary procedure or independently to the first intervention.

Traumatic aneurysm in persistent sciatic artery.

A 59-year-old male patient admitted to our hospital with a pulsatile right gluteal mass with history of formation after blunt trauma. Persistent sciatic artery and its traumatic aneurysm was diagnosed after medical examination followed by peripheric digital substraction angiography. Surgery was performed under general anesthesia, and the patient was discharged from the hospital on postoperative day 7 without any problems. The sciatic artery, a continuation of the internal iliac artery into the popliteal-tibial vessels, represents the major blood supply to the lower limb in early embryologic development. Its persistence is very rare, and the aneurysmatic dilatation is the most common pathology diagnosed. Here, we present a rare case of persistent sciatic artery presenting with a traumatic aneurysm treated by iliopopliteal bypass surgery and ligation of the internal iliac artery proximal to the aneurysm.