Minimally invasive mitral valve repair and ablation of concomitant atrial fibrillation in a patient with severe hemophilia A.

Minimally invasive mitral valve repair and ablation of atrial fibrillation, combined with FVIII level-controlled replacement therapy, can be safely performed in patients with severe hemophilia.

Residual Lesion Diagnostics in Pediatric Postcardiotomy Extracorporeal Membrane Oxygenation and Its Outcomes.

BACKGROUND: To assess the impact of diagnostic procedures in identifying residual lesions during extracorporeal membrane oxygenation (ECMO) on survival after pediatric cardiac surgery. METHODS: Between January 2012 and December 2017, 74 patients required postcardiotomy ECMO. Patients were retrospectively divided into 2 groups: Group I underwent only echocardiography ([echo only] 46 patients, 62.2%) and group II (echo+) underwent additional diagnostic tests (ie, computed tomography [CT] or cardiac catheterization; 28 patients, 37.8%). Propensity score matching was used to balance the 2 groups by baseline characteristics. RESULTS: Two equal groups (28 patients in each group) were formed by propensity score matching. Fourteen (50%) patients in the echo-only group and 20 (71%) patients in the echo+ group were successfully weaned from ECMO (P = .17). Four (14.3%) patients survived in the echo-only group and 15 (53.5%) patients survived in the echo+ group (P = .004). Patients in the echo+ group had a lower chance of dying compared to the echo-only group (odds ratio, 0.14.6; 95% CI, 0.039-0.52; P = .003). The residual lesions, which may have served as a mortality factor, were found by autopsy in 8 (40%) patients in the echo-only group, while none were found in the echo+ group (P = .014). CONCLUSIONS: The autopsies of patients who died despite postcardiotomy ECMO support showed that in 40% of cases that had been investigated by echo only, residual lesions that had not been detected by echocardiography were present. The cardiac catheterization and CT during ECMO are effective and safe for identifying residual lesions. Early detection and repair of residual lesions may increase the survival rate of pediatric cardiac patients on ECMO.

Effect of intraoperative dexamethasone on major complications and mortality among infants undergoing cardiac surgery The DECISION Randomized Clinical Trial

IMPORTANCE Corticosteroids are widely used in pediatric cardiac surgery to blunt systemic inflammatory response and to reduce complications; nevertheless, their clinical efficacy is uncertain. OBJECTIVE To determine whether intraoperative administration of dexamethasone is more effective than placebo for reducing major complications and mortality during pediatric cardiac surgery. DESIGN, SETTING, AND PARTICIPANTS The Intraoperative Dexamethasone in Pediatric Cardiac Surgery was an investigator-initiated, double-blind, multicenter randomized trial that involved 4 centers in China, Brazil, and Russia. A total of 394 infants younger than 12 months, undergoing cardiac surgery with cardiopulmonary bypass were enrolled from December 2015 to October 2018, with follow-up completed in November 2018. INTERVENTIONS The dexamethasone group (n = 194) received 1mg/kg of dexamethasone; the control group (n = 200) received an equivolume of 0.9%sodium chloride intravenously after anesthesia induction. MAIN OUTCOMES AND MEASURES The primary end pointwas a composite of death, nonfatalmyocardial infarction, need for extracorporeal membrane oxygenation, need for cardiopulmonary resuscitation, acute kidney injury, prolonged mechanical ventilation, or neurological complications within 30 days after surgery. There were 17 secondary end points, including the individual components of the primary end point, and duration of mechanical ventilation, inotropic index, intensive care unit stay, readmission to intensive care unit, and length of hospitalization. RESULTS All of the 394 patients randomized (median age, 6 months; 47.2%boys) completed the trial. The primary end point occurred in 74 patients (38.1%) in the dexamethasone group vs 91 patients (45.5%) in the control group (absolute risk reduction, 7.4%; 95%CI, −0.8%to 15.3%; hazard ratio, 0.82; 95%CI, 0.60 to 1.10; P = .20). Of the 17 prespecified secondary end points, none showed a statistically significant difference between groups. Infections occurred in 4 patients (2.0%) in the dexamethasone group vs 3 patients (1.5%) in the control group. CONCLUSIONS AND RELEVANCE Among infants younger than 12 months undergoing cardiac surgery with cardiopulmonary bypass, intraoperative administration of dexamethasone, compared with placebo, did not significantly reduce major complications and mortality at 30 days. However, the study may have been underpowered to detect a clinically important difference.

Effect of Intraoperative Dexamethasone on Major Complications and Mortality Among Infants Undergoing Cardiac Surgery: The DECISION Randomized Clinical Trial.

Importance: Corticosteroids are widely used in pediatric cardiac surgery to blunt systemic inflammatory response and to reduce complications; nevertheless, their clinical efficacy is uncertain. Objective: To determine whether intraoperative administration of dexamethasone is more effective than placebo for reducing major complications and mortality during pediatric cardiac surgery. Design, Setting, and Participants: The Intraoperative Dexamethasone in Pediatric Cardiac Surgery was an investigator-initiated, double-blind, multicenter randomized trial that involved 4 centers in China, Brazil, and Russia. A total of 394 infants younger than 12 months, undergoing cardiac surgery with cardiopulmonary bypass were enrolled from December 2015 to October 2018, with follow-up completed in November 2018. Interventions: The dexamethasone group (n = 194) received 1 mg/kg of dexamethasone; the control group (n = 200) received an equivolume of 0.9% sodium chloride intravenously after anesthesia induction. Main Outcomes and Measures: The primary end point was a composite of death, nonfatal myocardial infarction, need for extracorporeal membrane oxygenation, need for cardiopulmonary resuscitation, acute kidney injury, prolonged mechanical ventilation, or neurological complications within 30 days after surgery. There were 17 secondary end points, including the individual components of the primary end point, and duration of mechanical ventilation, inotropic index, intensive care unit stay, readmission to intensive care unit, and length of hospitalization. Results: All of the 394 patients randomized (median age, 6 months; 47.2% boys) completed the trial. The primary end point occurred in 74 patients (38.1%) in the dexamethasone group vs 91 patients (45.5%) in the control group (absolute risk reduction, 7.4%; 95% CI, -0.8% to 15.3%; hazard ratio, 0.82; 95% CI, 0.60 to 1.10; P = .20). Of the 17 prespecified secondary end points, none showed a statistically significant difference between groups. Infections occurred in 4 patients (2.0%) in the dexamethasone group vs 3 patients (1.5%) in the control group. Conclusions and Relevance: Among infants younger than 12 months undergoing cardiac surgery with cardiopulmonary bypass, intraoperative administration of dexamethasone, compared with placebo, did not significantly reduce major complications and mortality at 30 days. However, the study may have been underpowered to detect a clinically important difference. Trial Registration: ClinicalTrials.gov Identifier: NCT02615262.

Quantitative assessment of peripheral limb perfusion using a modified distal arterial cannula in venoarterial ECMO settings.

In cases of severe cardiopulmonary deterioration, quick establishment of venoarterial extracorporeal membrane oxygenation (ECMO) represents a support modality. After successful arterial peripheral cannulation, a certain grade of peripheral limb malperfusion is a fairly common phenomenon. Detection of peripheral malperfusion is vital, since it can result in compartment syndrome or even loss of the affected limb. To prevent or resolve emerging lower limb ischaemia, a newly designed perfusion catheter is placed into the superficial femoral artery, distal to the arterial cannula via ECMO. The aim of our study was to evaluate flow and haemodynamic characteristics of this novel distal limb perfusion cannula for ECMO therapy and present these important findings for the first time. The distal perfusion cannula blood flow increases in linear correlation with ECMO blood flow The variability of distal perfusion cannula blood flow with a 15 Fr cannula ranges between 160 ± 0.40 mL min-1 at 1.5 L min-1 ECMO flow rate and 480 ± 80 mL min-1 at 5.0 L min-1 ECMO blood flow, respectively. Comparatively, the 17-Fr-sized cannula performs on a scale of 140 ± 20 to 390 ± 60 mL distal perfusion cannula blood flow at 1.5-5.0 L min-1 ECMO blood flow, respectively. The quantitative assessment of the distal perfusion cannula blood flow has revealed that distal perfusion cannula blood flow can measure up to 10% of the ECMO blood flow. Furthermore, it has been also well demonstrated that the novel distal perfusion cannula is sufficient to compensate peripheral limb ischaemia.

Extracorporeal cardiopulmonary resuscitation after pulmonary artery rupture.

Pulmonary artery rupture during pulmonary balloon valvuloplasty is a rare and life-threatening complication. Here, we present a pulmonary artery rupture in a 10-month-old infant. The patient had a tamponade, ineffective cardiac massage for 40 minutes and extreme hemodilution due to blood loss. Extracorporeal life support was used for three days. The patient was discharged without any neurological sequelae.

Chronic Lung Disease and Mortality after Cardiac Surgery: A Prospective Cohort Study.

OBJECTIVE: To investigate the 1-year survival in cardiac surgical patients with lung disease, including previously undiagnosed cases. DESIGN: Prospective cohort study. SETTING: Tertiary hospital. PARTICIPANTS: Patients scheduled for elective coronary artery bypass graft (CABG) surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Pulmonary function tests (PFTs) were performed in 454 patients before surgery. Abnormal respiratory patterns were defined as follows: obstructive (forced expiratory volume in 1 second/forced vital capacity <0.70), restrictive (forced expiratory volume in 1 second/forced vital capacity ≥0.70 and forced vital capacity <80% of predicted), and mixed. Overall 1-year mortality was 3.3%. Among 31 patients with documented chronic obstructive pulmonary disease (COPD), mortality was 9.6%, hazard ratio (HR) 1.28, 95% confidence interval (CI) 1.02-12.80, p = 0.04. Of 423 patients without history of COPD, 57 obstructive, 46 restrictive, and 4 mixed abnormal patterns were identified. Of a total of 72 with obstructive lung disease confirmed by PFT (ie, 15 of COPD patients and 57 newly identified cases), 6.9% died, HR 2.75, 95% CI 0.98-8.07, p = 0.06. When combined with cases of COPD where a respiratory abnormality was confirmed (26 patients), newly diagnosed obstructive lung disease (57 patients) was significantly associated with 1-year mortality, HR 4.13, 95% CI 1.50-11.42, p = 0.006. The adjustment for EuroSCORE II did not change the results. CONCLUSIONS: Combination of confirmed preexisting lung disease and newly diagnosed cases provides a clear link to mid-term mortality.

Postcardiotomy refractory ventricular fibrillation: rescue using veno-arterial extracorporeal membrane oxygenation.

We present a case of 7-hour ventricular fibrillation with successful use of veno-arterial extracorporeal membrane oxygenation as a bridge to recovery in a 30-year-old patient with grown-up congenital heart disease who underwent pulmonary valve replacement.

Modified reverse aortoplasty versus extended anastomosis in patients with coarctation of the aorta and distal arch hypoplasia.

OBJECTIVES: The aim of our prospective randomized study was to compare modified reverse aortoplasty (MRA) and extended end-to-end anastomosis (EEA). METHODS: We have assessed the operative correction results in 54 infants with coarctation of the aorta and distal aortic arch hypoplasia who underwent primary repair in our institute between July 2013 and February 2014. All of the patients were <12 months old when they had the operation. Patients were randomly assigned to 2 arms: modified reverse subclavian flap angioplasty (MRA group; n = 27) or extended end-to-end anastomosis (EEA group; n = 27). RESULTS: We found a significant difference in residual arterial hypertension between the groups: 2 (7.7%) patients in the MRA group and 8 (30.8%) patients in the EEA group, respectively (P = 0.03). Risk factors for arterial hypertension were precoarctation area rigidity and endocardial fibroelastosis. During the last follow-up visit, recoarctation was observed in 1 (3.8%) patient in the MRA group and in 2 (7.7%) patients in the EEA group, respectively (P = 0.50). Cox regression analysis showed that the only risk factor for recoarctation was the patient's low weight before surgery [odds ratio (95% confidence interval) 0.016 (0.001-0.51), P = 0.047]. An aortic aneurysm developed in 2 (7.7%) patients in the MRA group; however, no significant difference was found between the groups (P = 0.15). CONCLUSIONS: The results of surgical repair of coarctation of the aorta using modified reverse subclavian flap angioplasty and extended anastomosis were comparable in mortality rates and early postoperative complications. Nevertheless, MRA may be beneficial in reducing residual arterial hypertension at the mid-term follow-up visit.

Extracorporeal membrane oxygenation for Kawasaki disease: two case reports and the Extracorporeal Life Support Organization experience 1999-2015.

Kawasaki disease is usually a limited illness of early childhood. However, life-threatening cardiac manifestations can occur, either at acute presentation or as a consequence of coronary arterial involvement. We report the successful use of veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) for cardiac support in two children with Kawasaki disease: one with acute Kawasaki disease shock syndrome, the other with complications of coronary arteritis and subsequent surgery. We also reviewed the reported experience in the ELSO database and available literature.

Risk Factors For Unfavorable Outcomes After Bidirectional Cavopulmonary Anastomosis.

BACKGROUND: Bidirectional cavopulmonary anastomosis (BCPA) is an important preliminary step toward the Fontan procedure; thus, understanding of risk factors for morbidity and mortality after BCPA may ultimately promote improved rates of success with Fontan completion and general survival. This study evaluated survival and predictors of unfavorable outcomes in patients after BCPA. METHODS: Clinical data of 157 patients who underwent BCPA from 2003 to 2015 at a single center were retrospectively analyzed. RESULTS: Three-year and nine-year survival after BCPA were 87.1% ± 2.8% and 85.8% ± 2.9%, respectively. Freedom from unfavorable outcomes (mortality, BCPA takedown, nonsuitability for Fontan procedure) was 83.8% ± 3.1% at three years and 73.5% ± 4.8% at nine years. Multivariate proportional hazards regression analysis revealed that total anomalous pulmonary venous connection (TAPVC; hazard ratio [HR]: 3.74, 95% confidence interval [CI]: 1.35-10.36; P = .01) and increased mean pressure in BCPA circuit (HR: 1.17, 95% CI: 1.02-1.34; P = .03) were independent risk factors for unfavorable outcomes. Postoperative mean pressure in BCPA circuit in patients with poor outcomes was median 16 mm Hg (interquartile range [IQR]: 14-18 mm Hg) versus median 14 mm Hg (IQR: 12-15.5 mm Hg) in patients with favorable outcomes ( P < .01). Preoperative (HR: 1.87, 95% CI: 1.20-2.91; P < .01) and postoperative atrioventricular valve regurgitation (AVVR; HR: 2.22, 95% CI: 1.24-3.94; P < .01) were also associated with unfavorable outcome in univariate Cox regression. CONCLUSIONS: Elevated mean pressure in the BCPA circuit is the main predictor of unfavorable outcome; therefore, thorough preoperative examination and careful patient selection are critical points for successful intermediate-stage and later Fontan completion. Total anomalous pulmonary venous connection and insufficient correction of AVVR worsen the prognosis in this patient group.

Prevalence and Implications of Abnormal Respiratory Patterns in Cardiac Surgery: A Prospective Cohort Study.

OBJECTIVE: To investigate the prevalence and impact of abnormal respiratory patterns in cardiac surgery patients. DESIGN: Prospective cohort study. SETTING: Tertiary hospital. PARTICIPANTS: Patients scheduled for elective coronary artery bypass graft surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Pulmonary function tests were performed in 454 patients before surgery. Abnormal respiratory patterns were defined as follows: obstructive (forced expiratory volume in 1 s [FEV1]/forced vital capacity [FVC]<0.70), restrictive (FEV1/FVC≥0.70 and FVC<80% of predicted), and mixed (FEV1/FVC<0.70 and both FEV1 and FVC<80% of predicted). Of the 31 patients with a history of chronic obstructive pulmonary disease, no abnormal respiratory pattern was confirmed in 5. Of the 423 patients without a history of lung disease, the authors newly identified 57 obstructive, 46 restrictive, and 4 mixed patterns. Therefore, lung disease was reclassified in 24.7% of cases. Independent predictors of obstructive pattern were age, male sex, history of smoking, and chronic obstructive pulmonary disease. Obstructive lung disease was associated with 16 hours or longer ventilation. A reduced FEV1 was associated with a likelihood of atrial fibrillation (1-L decrement, odds ratio: 1.38, 95% confidence interval: 1.01-to-1.90, p = 0.04) and hospitalization time (regression coefficient: 1.23, 95% confidence interval: 0.54-to-1.91, p<0.001). CONCLUSIONS: Abnormal respiratory patterns are common and often underdiagnosed in the cardiac surgery setting. Pulmonary function tests help reveal patients at risk of complications and may provide an opportunity for intervention.

Bidirectional cavopulmonary anastomosis with additional pulmonary blood flow: good or bad pre-Fontan strategy.

Objectives: This study aimed to evaluate the influence of preserved additional pulmonary blood flow (APBF) on survival after bidirectional cavopulmonary shunt (BCPS) and completion of Fontan circulation. Methods: From March 2003 and April 2015, 156 patients with a single ventricle underwent BCPS. After performing propensity score analysis (1:1) for the entire sample, 50 patients with APBF (APBF group) were matched with 50 patients without APBF (no-APBF group). Results: Age ( P = 0.90), sex ( P = 0.57), weight ( P = 0.75), single ventricle morphology ( P = 0.87), type of neonatal palliative procedure ( P = 0.52), saturation ( P = 0.35), ejection fraction ( P = 0.90), Nakata index ( P = 0.70) and mean pulmonary artery pressure ( P = 0.72) were not significantly different between the groups. No significant survival difference was demonstrated ( P = 0.54). One and 4-year survival rates were both 89.1% ± 4.6% in the APBF group and 87.2% ± 4.9% and 83.4% ± 5.9%, respectively, in the no-APBF group. There was no significant difference in rates of Fontan completion ( P = 0.24), which was achieved in 22 patients from the APBF group (55.0%) and 26 patients from the no-APBF group (65.0%). However, Fontan completion occurred significantly earlier in the no-APBF group ( P < 0.01). In this group, Fontan procedure was performed before 36 months of inter-stage period in 45.9% ± 8.5% of cases (95% CI 31.0-63.7%) compared to only 13.3 ± 5.6% (95% CI 5.8-29.1%) in the APBF group. Conclusions: Our study demonstrates that APBF does not affect survival after BCPS or Fontan completion rate. APBF allows postponing the Fontan procedure without a negative effect on clinical status.

Pseudoaneurysm and aorto-bronchial fistula following balloon dilation of recoarctation.

This case report documents the successful surgical repair of an aorto-bronchial fistula and a giant aortic pseudoaneurysm at the proximal anastomosis of a dacron interposition tube graft that was balloon dilated for recurrent coarctation. Balloon dilation for recoarctation of a dacron interposition tube graft may lead to serious complications.

Efficiency of Various Cerebral Protection Techniques Used during the Surgical Treatment of Chronic Pulmonary Thromboembolism.

Circulatory arrest during pulmonary thromboendarterectomy (PTE) for chronic pulmonary embolism leads to an increased risk of cerebral ischemia and neurological complications. This study aimed to assess the efficacy of various cerebral protection techniques used during the surgical treatment of chronic pulmonary thromboembolism. We prospectively studied 61 patients with chronic pulmonary thromboembolism who underwent PTE. We compared the dynamics of cerebral oxygen saturation (rSO2, %) during the surgical treatment and analyzed neurological complications during the early postoperative period in two groups of patients: 30 patients who underwent surgery under conditions of moderate hypothermia (23°C-24°C) combined with antegrade unilateral cerebral perfusion (ACP group) and 31 patients who underwent thromboendarterectomy under deep hypothermic circulatory arrest (18°C, DHCA group) combined with craniocerebral hypothermia. In the ACP group, regional rSO2 decreased by less than 20% from baseline during the course of PTE. In the DHCA group, a more profound reduction of cerebral oxygen supply (by >30% from baseline) was recorded compared with the ACP group (p < .05). During the early postoperative period, 29% of patients in the DHCA group exhibited neurological complications, compared with only 7% of patients from the ACP group. The results of logistic regression analysis indicated that the risk of progressive neurological deficit depended on the duration of the intraoperative period when the absolute values of regional rSO2 were <40%. The method with the best adjustment to human physiology in patients with chronic pulmonary thromboembolism was antegrade cerebral perfusion. This method provides a smaller decrease in cerebral oxygen supply during thromboendarterectomy and significantly reduces the risk of ischemia and neurological complications in the early postoperative period.

Outcomes after aortic arch reconstruction for infants: deep hypothermic circulatory arrest versus moderate hypothermia with selective antegrade cerebral perfusion.

OBJECTIVES: Optimal cerebral and visceral protection is crucial in aortic arch surgery. The main method for this protection has traditionally been deep hypothermic circulatory arrest (DHCA). Recently, antegrade cerebral perfusion with moderate hypothermia has become the preferred strategy for adult patients and some children undergoing aortic arch surgery. Continuous cerebral perfusion should reduce the incidence of neurological complications, but the degree of damage to organs and systems resulting from the lack of blood flow distal to the aortic arch remains unclear. Here, we aimed to evaluate the efficacy and safety of methods of protecting the brain and internal organs during aortic arch surgery in infants. METHODS: We performed a retrospective review of 62 patients who underwent aortic arch reconstruction to assess their neurological status and internal injuries after different methods of cerebral protection. RESULTS: Surgical correction of aortic arch congenital abnormalities was performed under DHCA in 27 patients (Group I), and unilateral selective antegrade cerebral perfusion (SACP) was performed in 35 patients (Group II). In Group I, 30.8% of patients had neurological complications, whereas in Group II 5.9% had neurological complications. The odds ratio for a neurological event was significantly lower in Group II compared with Group I-0.14 [(95% CI 0.02-0.63), P = 0.02]. However, incidence of renal dysfunction was significantly higher in the second group than the first: 21 (61.2%) vs 5 (19.2%) cases, respectively [odds ratio 6.49 (95% CI 1.41-38.26), P = 0.02]. CONCLUSIONS: Aortic arch reconstruction accompanied by SACP has a lower risk of neurological complications compared with DHCA. However, the high incidence of renal complications with SACP requires further study.