Risk factors for prolonged ventilation after the modified Fontan procedure.

OBJECTIVE: To investigate the risk factors associated with prolonged ventilation after Fontan surgery. DESIGN: Retrospective case series. SETTING: Tertiary childrens hospital. PATIENTS: We included 123 children who underwent Fontan surgery without delayed sternal closure or extracorporeal membrane oxygenation between 2011 and 2017. INTERVENTION: Fontan surgery. MEASUREMENTS AND MAIN RESULTS: Prolonged ventilation was defined as intubation for more than 24 hours after surgery. Preoperative, intraoperative, and perioperative data were collected retrospectively from medical records. Multivariate logistic regression analysis was used to identify risk factors for prolonged ventilation. The median age and weight of patients were 2.2 years and 10.0 kg, respectively. Seventeen per cent of the patients (n = 21) received prolonged mechanical ventilation, and the median intubation period was 2.9 days. There were no 90-day or in-hospital deaths. The independent predictors of prolonged ventilation identified were fenestration (p < 0.01), low pulmonary artery index (p = 0.02), and advanced atrioventricular regurgitation (p < 0.01). The duration of ICU stay was significantly longer in the prolonged ventilation group than in the early extubation group (10 days versus 6 days, p < 0.01). CONCLUSION: Fenestration, low pulmonary artery index, and significant atrioventricular regurgitation are risk factors for prolonged ventilation after Fontan surgery. Careful preoperative and perioperative management that considers the risk factors for prolonged ventilation in each individual is important.

Pseudoaneurysm rupture presenting as bleeding from the cannulation site in a paediatric patient with dilated cardiomyopathy and congenital skin lesions requiring EXCOR® Paediatric ventricular assist device: a case report.

BACKGROUND: EXCOR® Paediatric is used worldwide as a bridge-to-transplant treatment. It provides improved patient stability during the waiting period compared with previous ventricular assist device (VAD). However, investigations into complications which may occur among the paediatric population during long waiting periods are still sparse. CASE SUMMARY: We describe the case of a 7-year-old girl who presented with severe heart failure due to dilated cardiomyopathy. She also had a skin lesion which appeared soon after birth. She had received an EXCOR® implant and was waiting for heart transplant. Her skin lesion worsened after implantation and she suffered recurrent infections. Multiple bleeding episodes from the cannulation site occurred; therefore, surgical exploration of the bleeding was performed. She passed away during the procedure due to massive bleeding caused by rupture of a pseudoaneurysm caused by blood-stream infection. DISCUSSION: Patients with skin disease may be at increased risk of infection when on a VAD. Infections that occur during VAD therapy may cause serious complications such as pseudoaneurysm. The possibility of pseudoaneurysm should be considered when bleeding occurs in a patient on VAD.

Extubation Failure in Neonates After Cardiac Surgery: Prevalence, Etiology, and Risk Factors.

BACKGROUND: The purpose of this study was to explore the prevalence, etiology, and risk factors of extubation failure (EF) in post-cardiac surgery neonates. METHODS: Neonates (30 days old or younger) who underwent cardiac surgery and were admitted to the cardiac intensive care unit between September 2010 and February 2016 were included. The prevalence and etiology of EF, defined as reintubation within 48 hours, were reviewed. Demographic, operative, and perioperative data were retrospectively collected. Multiple logistic regression models were constructed to identify the risk factors for EF. RESULTS: The median age at surgery was 10 days. Extubation failure occurred in 25 of 156 cases (16.0%; 95% confidence interval: 10.6% to 22.7%), because of respiratory dysfunction (n = 16), hemodynamic instability (n = 4), upper airway obstruction (n = 4), or gastrointestinal bleeding (n = 1). Subsequent extubations were successful in 17 cases (68%) because of medical optimization of the causes of reintubation. The remaining 8 cases needed surgical reintervention, including tracheostomy and cardiac surgery. The inhospital mortality rate was 2.6%. In a bivariate analysis, younger age, airway diseases, ventilation before surgery, prolonged mechanical ventilation, and delayed sternal closure were associated with EF. The multivariable analysis identified airway diseases (adjusted odds ratio 18.2, 95% confidence interval: 3.8 to 88.6, p = 0.0003) and mechanical ventilation longer than 7 days (adjusted odds ratio 8.2, 95% confidence interval: 1.9 to 34.9, p = 0.0046) as risk factors for EF. CONCLUSIONS: The prevalence of EF is relatively high in neonatal cardiac surgery. The etiologies can be diverse. Extubation of neonates at high risk after cardiac surgery, based on these possible risk factors, requires more diligent approaches.

Fontan completion in patients with atrial isomerism and separate hepatic venous drainage.

OBJECTIVE: Fontan completion in patients with atrial isomerism, in which the inferior vena cava (IVC) and the hepatic vein (HV) drain separately, is technically challenging. Herein, we review our surgical approach to these patients. METHODS: The medical records of 50 consecutive patients with atrial isomerism who underwent Fontan completion between 1998 and 2008 were reviewed retrospectively. RESULTS: Separate HV drainage was present in 17 patients. Patients with interrupted IVC were excluded. Patient characteristics were as follows: median age, 26 months (range 15-149); median weight, 9.6 kg (range 8.1-47.2); right atrial isomerism, 16 patients; and left atrial isomerism, one. The IVC and the separate HV at the level of diaphragm were contralateral in 16 patients, and ipsilateral in one. The surgical procedures for directing blood flow from the IVC and the separate HV to the pulmonary arteries were as follows: en bloc resection of the IVC and the HV and anastomosing these veins to an extracardiac conduit in 10 patients; connecting the IVC to the HV in a side-to-side fashion before anastomosing them to an extracardiac conduit in one; and lateral tunnel in another. When the IVC and the HV were widely separated by the vertebrae, we chose an intra-extracardiac conduit (intra-atrial septation) in four patients and an extracardiac conduit for the IVC and the right HV and lateral tunnel for the separate left HV in one. There was no mortality. Five re-operations were performed (pacemaker in two patients; one each of fenestration, release of outflow obstruction and ligation of collateral arteries). Sixteen patients underwent follow-up catheterisation, which revealed central venous pressure of 12.0 + or - 2.0 mmHg and arterial oxygen saturation of 92% + or - 6%. CONCLUSIONS: The mid-term results of the Fontan completion in patients with atrial isomerism and separate HV drainage were excellent. The distance between the IVC and the separate HV and the position of the vertebrae should be considered when choosing a surgical technique.

Functional single ventricle with extracardiac total anomalous pulmonary venous connection.

OBJECTIVE: The purpose of this study is to evaluate the surgical repair of functional single ventricle and extracardiac total anomalous pulmonary venous connection (TAPVC). METHODS: Between January 1998 and December 2007, 26 consecutive patients underwent surgical repair of extracardiac TAPVC. Their characteristics were as follows: median age, 34 (range 0-744) days; median weight 3.2 (range 2.0-9.6) kg; supracardiac TAPVC, 11 patients; infracardiac, 5; mixed, 10; right atrial isomerism, 24; pulmonary atresia, 16; and obstructed TAPVC, 17. Concomitant procedures included systemic-to-pulmonary shunt in 9 patients, pulmonary artery banding in 5, ventricle-to-pulmonary artery shunt in 1, Norwood procedure in 1, bidirectional Glenn in 9, and Fontan procedure in 1. RESULTS: The overall survival after the repair of TAPVC was 58% (95% confidence interval [CI], 39-77%) and 54% (95% CI, 34-73%) at 1 and 5 years, respectively. Of the 14 survivors (supracardiac, 9; infracardiac, 4; and mixed, 1), 12 underwent Fontan completion; 1, bidirectional Glenn; and 1 is awaiting bidirectional Glenn. Anastomotic stenosis did not occur, but recurrent pulmonary venous ostial stenosis (PVS) was observed in nine patients. Freedom from recurrent PVS was 56% (95% CI, 34-78%) at both 1 and 5 years. Reoperation for recurrent PVS was performed in six patients; of these patients, two underwent Fontan completion, but three with bilateral and multiple PVS declined. By Cox multivariate regression analysis, mixed TAPVC (p=0.001, hazard ratio, 13.4; 95% CI, 2.8-64.4) was a risk factor for mortality, and atrioventricular valve regurgitation, which required surgical intervention at the palliative stage (p=0.024, hazard ratio, 23.4; 95% CI, 1.5-363.4) was a risk factor for recurrent PVS. CONCLUSIONS: The mid-term results of the surgical repair of functional single ventricle with supracardiac or infracardiac TAPVC are acceptable. The surgical treatment of patients with mixed TAPVC and with severe atrioventricular valve regurgitation is not promising, but can be improved.

Surgical repair of multiple muscular ventricular septal defects: the role of re-endocardialization strategy.

OBJECTIVE: Surgical repair of multiple muscular ventricular septal defects (Swiss cheese septum) is associated with important morbidity and mortality. We sought to examine factors associated with permanent heart block, early mortality, and time-related survival. Additionally, we evaluated a new approach, transatrial re-endocardialization of interventricular septum, to mitigate risk. METHODS: One hundred sixteen patients underwent surgery for multiple muscular ventricular septal defects (1982-2005), of whom 64 (55%) had associated cardiac anomalies. Twenty-seven consecutive patients (median age 0.54 years, range 15 days-7.2 years) underwent transatrial re-endocardialization (2002-2005). Forty-four percent had Swiss cheese septum (>4 defects). Multivariable regression analysis determined risk factors for pacemaker and survival. RESULTS: Operative mortality for the entire cohort was 9%. Risk factors for death were double-outlet right ventricle (odds ratio 44.7, P = .003), ventriculotomy (odds ratio 6.4, P = .03), and fewer multiple muscular ventricular septal defects repaired (odds ratio 4.7/defect, P = .04). Era mortalities differed: 16% for 1982 through 1990, 13% for 1990 through 1998, and 0% for 1999 through 2005, P = .006). Fourteen patients (12%) required a pacemaker. Time-related survivals at 1 and 10 years were 90% +/- 3% and 82% +/- 5%. Risk factors for death were double-outlet right ventricle (hazard ratio 8.3, P = .02) and longer bypass (hazard ratio 1.02/min, P = .02). In 27 re-endocardialization patients, a combined closure strategy to close 184 defects were applied: transatrial re-endocardialization (median 5, range 2-21), patch (median 1, range 0-4), and device (range 0-1). Post-repair ventricular function was good in 25 of 27 patients. The median number of residual defects was 1.5 (range 0-3), and median residual jet width on color Doppler was 2.3 mm (range 0-4.2 mm). One child required a pacemaker. There were no early or late deaths. CONCLUSIONS: Outcome of surgical repair of multiple muscular ventricular septal defects (Swiss cheese septum) has improved. Transatrial re-endocardialization strategy enables early complete or nearly complete obliteration of multiple muscular ventricular septal defects with minimal residual lesions (shunt, ventricular dysfunction). Long cardiopulmonary bypass duration is well tolerated. The incidence of permanent heart block has improved. Early echocardiographic and clinical outcomes are promising.