Impact of a quality improvement initiative with a dedicated anesthesia team on outcomes after surgery for adult congenital heart disease.

Objectives: A quality improvement initiative was introduced to the adult congenital cardiac surgery program at Toronto General Hospital in January 2016. A dedicated Adult Congenital Anesthesia and intensive care unit team was introduced within the cardiac group. The use of factor concentrates was introduced. The study compares perioperative mortality, adverse events, and transfusion burden before and after this process change. Methods: We performed a retrospective analysis of all adult congenital cardiac surgeries from January 2004 to July 2019. Two groups were analyzed: patients undergoing operation before and after 2016. The primary outcome was in-hospital mortality. One-year mortality and prevalence of key morbidities were analyzed as secondary outcomes. A separate analysis looked at patients who had and had not attended an anesthesia-led preassessment clinic. Results: In-hospital mortality was significantly reduced in patients undergoing operation after 2016 (1.1% vs 4.3%, P = .003) despite a higher risk profile. One-year mortality (1.3% vs 5.8%, P = .003) and ventilation times (5.5 hours [3.4-13.0] vs 6.3 hours [4.2-16.2], P = .001) were also reduced. The incidence of stroke and renal failure was similar between groups. Blood product exposure was comparable, but the incidence of chest reopening decreased (1.8% vs 4.8%, P = .022), despite more patients with multiple previous chest wall incisions, on anticoagulation, and with more complex cardiac anatomy. There were no significant outcome differences between those who did or did not attend the preassessment clinic. Conclusions: Both in-hospital and 1-year mortality were significantly reduced after the introduction of a quality improvement program, despite a higher risk profile. Blood product exposure remained unchanged, but there were less chest reopenings.

Special Considerations in Critical Care of the Congenital Heart Disease Patient.

Outcomes of congenital heart disease have improved markedly over the past 20 years, with survival to adulthood now close to 90%. The mean age of admission to an intensive care unit (ICU) is 40 years. The incidence of hospital and critical care admissions have increased significantly as a consequence of this improved survival. Intensivists are now confronted with the management not only of complex adult congenital heart disease (ACHD) lesions from a cardiac perspective, but also of extracardiac organ consequences of years of abnormal circulation after surgical or palliative correction. Kidney and liver dysfunction and respiratory and hematologic abnormalities are very common in this population. ACHD patients can present to the ICU for a vast number of reasons, classified in this review as medical noncardiac, medical cardiac, and surgical. Community/hospital-acquired infections, cerebrovascular accidents, and respiratory failure, alongside arrhythmias and heart failure, are responsible for medical admissions. Surgical admissions include postoperative management after correction or palliation, but also medical optimisation and work-up for advanced therapies. ICU management of this large heterogeneous group requires a thorough understanding of the pathophysiology in order to apply conventional adult critical care modalities; left ventricular or right ventricular dysfunction, pulmonary hypertension, intracardiac, extracardiac, and palliative surgical shunts can be present and require additional consideration. This review focuses on the pathophysiology, long-term sequelae, and different treatment modalities to supply a framework for the ICU physician caring for these patients. Successful outcome, especially in complex lesions, depends on early involvement of specialised ACHD centres.

Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation.

The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients' original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. This review focuses on the anesthetic and intensive care unit (ICU) management of these patients during their perioperative journey, with an emphasis on the continuity of preintervention planning, referral pathways, and postintervention ICU management. Requests for recipes and doses of medications are frequent; however, as in normal anesthesia and ICU practice, the method of anesthesia and dosing are dependent on the presenting medical/surgical conditions and the underlying anatomy and physiologic reserve. A patient with Fontan palliation in their early 20s attending school full-time with a cavopulmonary connection is likely to have more reserve than a patient in their late 40s with an atriopulmonary Fontan at home waiting for a heart transplant. Each case will require an anesthetic and critical care plan tailored to the situation. The critical care environment is a natural extension of the anesthetic management of a patient, with complex considerations for a patient with Fontan palliation.

Fate of the liver in the survivors of adult heart transplant for a failing Fontan circulation.

The Fontan circulation has inherent long-term vulnerabilities such that adult Fontan patients now comprise the largest, most rapidly growing subgroup of adult congenital heart disease referred for transplant assessment. Almost all have Fontan Associated Liver Disease (FALD). There is an absence of mid to late hepatic outcome data after heart transplant alone. Therefore, we analyzed outcomes of survivors of heart only transplant in patients with failing Fontan circulation. Including all 10 of our adult Fontan patients surviving >1 year after isolated heart transplant, we report evolution of their clinical features, bloodwork, hemodynamic data, and liver ultrasound findings over a median of 4.7 years. Nonprogression of FALD, resolution of ascites and freedom from hepatocellular carcinoma in the mid-term highlight the outcomes in this selected group once normal cardiac output and venous pressures are established by heart transplant.

Adult congenital heart disease and anesthesia: An educational review.

Prognosis has dramatically improved among children with congenital heart disease (CHD), and the median survival for severe CHD is currently 25 years (ie, into adulthood). However, additional cardiac surgeries are often necessary in adults with CHD, whose unique cardiovascular anatomy and physiology necessitate specialized management by experts in adult CHD (ACHD) during the perioperative period. ACHD is characterized by a combination of congenital cardiac lesions, intervention-related anomalies that have developed over time, comorbidities caused by long-standing CHD, and comorbidities related to various syndromes and lifestyle factors. The present educational review discusses the transition from pediatric to adult cardiac care, comorbidities that develop as a result of ACHD, the assessments necessary for patients with ACHD prior to both cardiac and noncardiac surgeries, and the key ACHD lesions relevant to perioperative management.

Comparison of Heart Transplantation Outcomes: Adult Congenital Heart Disease vs Matched Cardiac Patients in a Quaternary Reference Centre.

BACKGROUND: The number of transplantations performed for adult congenital heart disease (ACHD) patients is increasing. We sought to compare survival and post-transplantation complications, including graft failure, rejection, dialysis, and use of a right ventricular assist device, between ACHD and a cohort of dilated (DCM) and ischemic (ICM) cardiomyopathy patients matched by age and year of transplantation. METHODS: We retrospectively reviewed our single-institution heart transplantation database and selected all patients who had surgery from 1988 to 2017. In our primary analysis, we looked at survival and post-transplantation complications across cardiomyopathy groups. Our secondary analysis was matched to mitigate era effects as well as differences in age at transplant. RESULTS: We analyzed a cohort consisting of 303 heart transplant patients with cardiomyopathy due to either 1) ACHD (n = 38), 2) ICM (n = 110), or 3) DCM (n = 155). Kaplan-Meier analysis and a multivariable Cox proportional hazard regression model were used for all-cause mortality, and cause-specific hazard regression for cause-specific mortality and morbidity. There was no statistically significant survival difference across groups. The 1-year survival was 68.5% for ACHD, 85.4% for ICM, and 85.5% for DCM. In multivariable analysis, ICM and DCM patients showed a 66% lower risk of death relative to the ACHD group. The matched analysis showed no significant difference in survival across groups. CONCLUSIONS: ACHD patients represent a growing high-risk patient cohort referred for transplantation. To improve survival outcomes we need to address modifiable risk factors.

Noncardiac determinants of death and intensive care morbidity in adult congenital heart disease surgery.

OBJECTIVES: Predicting perioperative morbidity and mortality in cardiac surgery for adult congenital heart disease is challenging because it encompasses a wide spectrum of disease. There is a paucity of published outcome data, and there are no perioperative risk score calculators for this population group. We set out to identify robust determinants of morbidity and mortality in this patient population under going cardiac surgery. METHODS: We collected data on 20 socioeconomic and pathophysiologic variables in 784 consecutive adults with congenital heart disease who underwent cardiac surgery between 2004 and 2015 at a single center. Using logistic regression, we sought to identify which of these factors were associated with the primary composite adverse outcome of in-hospital mortality, prolonged ventilation exceeding 7 days, and severe acute renal failure requiring dialysis. Secondary outcome analysis identified variables that were significant predictors for 1-year mortality. RESULTS: Composite adverse outcome occurred in 54 of 784 patients (6.9%). Significant predictors of the composite adverse outcome by multivariate regression include Mayo End-Stage Liver Disease modified score, cognitive impairment, number of chest wall incisions from previous cardiac surgery, body mass index, and cardiac anatomic category. Two survivors of the composite adverse outcome died within a few weeks postdischarge. Only 657 of 784 patients had 1-year follow-up data; 40 of 657 patients died at 1 year. One-year mortality was predicted by anticoagulation, Mayo End-Stage Liver Disease modified score, and anatomic category. CONCLUSIONS: Recognition and quantification of noncardiac comorbidities preoperatively predict the risk of adverse events and mortality in addition to cardiac anatomic factors.

Total Hip Replacement in a Patient With a Fontan Circulation: A Case Report.

Anesthetic management of the adult patient with a Fontan circulation is complex and requires understanding of the specific physiology of the individual patient. Long-term survival in this cohort has increased to the point where patients are presenting for noncardiac surgery related to degenerative diseases of aging. We describe the perioperative management of a patient with a Fontan circulation undergoing total hip arthroplasty using combined spinal-epidural anesthesia and discuss the issues requiring special consideration for this surgical procedure in this group of patients.

Anesthesia for Percutaneous Pulmonary Valve Implantation: A Case Series.

BACKGROUND: Twenty percent of patients born with congenital heart disease present with right ventricular outflow tract abnormalities. These patients require multiple surgical procedures in their lifetime. Transcatheter pulmonary valve replacement (TPVR) has become a viable alternative to conventional pulmonary valve and right ventricular outflow tract surgery in pediatric and adult populations. In this retrospective review, we analyze the perioperative management of adult patients who underwent TPVR in our center. METHODS: The study consisted of a chart review of patients who underwent TPVR at Toronto General Hospital between 2006 and 2015. Information about preoperative assessment, intraoperative anesthetic management, and intra- and postprocedural complications was collected. Two types of percutaneous valves have been used for a conduit or valve size between 16 and 28 mm. These procedures are done via the femoral, jugular, or subclavian vein under general anesthesia. RESULTS: Seventy-nine adults (17-68 years of age) who underwent elective TPVR procedures were included. General anesthesia was used in all cases. Defibrillation was necessary in 1 case, and bradycardia was spontaneously resolved in another 1. Eighty-five percent were successfully extubated at the end of the procedure. Five patients required intraoperative inotropic support. Three patients presented self-resolved hemoptysis. Mechanical ventilation for >24 hours was necessary in 3 cases, 2 of which also required concomitant inotropic support. Four failed deployments and 1 case of persistent conduit stenosis were reported. Three patients required reintubation. All patients were discharged home. CONCLUSIONS: Patients undergoing TPVR represent a complex and heterogeneous population. General anesthesia with endotracheal intubation is preferred. Setup for urgent lung isolation and cardiac defibrillation should be considered. Postoperative monitoring and intensive care setting are required. Anesthesiologists with cardiac anesthesia training are probably better suited to manage these patients.

Phenotype, management and predictors of outcome in a large cohort of adult congenital heart disease patients with heart failure.

OBJECTIVE: Although heart failure (HF) is the leading cause of premature death in adult congenital heart disease (ACHD), little population-specific data exist. This study reports early experience from a dedicated, sub-specialty adult congenital heart disease-heart failure (ACHD-HF) clinic, aiming to identify risk factors for adverse outcome. METHODS: Between 2012 and 2015, 126 patients (57% male) attended the ACHD-HF clinic. Baseline and follow-up data were analysed and compared across 4 anatomical/physiological subgroups: cyanotic ACHD, Fontan circulation (1V), biventricular circulation with a subaortic right ventricle (2V-RV) and biventricular circulation with a subaortic left ventricle (2V-LV). Predictors of the composite primary outcome: death, transplant or ventricular assist device (VAD) were identified using multivariable Cox proportional hazard models. RESULTS: Mean age at first visit was 38±13years. Patients were grouped as follows: cyanotic ACHD 10%, 1V 24%, 2V-RV 29% and 2V-LV 37%. During a median follow-up of 1.7 (IQR 0.8-2.9) years, 38 patients (30%) reached the primary outcome. Event-free survival was 89%, 78% and 63% at 1, 2 and 3years. Forty (31.7%) patients experienced 69 HF hospitalisations. Between-group differences were noted for systolic function, valvular regurgitation, pacing prevalence and invasive hemodynamics. Multivariable analysis revealed 2V-RV subgroup (p=0.001), NYHA class (p=0.002) B-type natriuretic peptide >164pg/ml (p=0.003) and sodium <136mmol/L (p=0.036) as independently associated with death, transplant or VAD. CONCLUSIONS: Our young ACHD-HF patients experienced high adverse event rates during a short period of follow-up. The prognostic markers identified will aid clinicians to stratify short-term risk and thereby guide advanced HF management decisions in ACHD.

The anesthesiologist's role in adults with congenital heart disease.

Anesthesia for adults with congenital heart disease has many challenging physiologic considerations. Collaborative relationships of a multidisciplinary team including cardiology, cardiac surgery, anesthesiology, and intensive care are essential to ensure positive outcomes in this population for noncardiac and cardiac surgery.

Case report: management of life-threatening oropharyngeal bleeding with recombinant factor VIIa.

PURPOSE: To report the use of recombinant factor VIIa (rFVIIa) for management of profuse oropharyngeal bleeding in a cirrhotic patient that nearly resulted in a 'cannot intubate, cannot ventilate' scenario. CLINICAL FEATURES: A 42-yr-old woman with end-stage liver disease presented for orthotopic liver transplantation. She was dialysis dependent and had marked coagulopathy [international normalized ratio (INR) = 3.1], without evidence of active bleeding. Following uneventful induction of anesthesia, routine airway manipulation for tracheal intubation caused profuse upper airway bleeding making visualization of her airway by direct laryngoscopy impossible. Moreover, several further attempts at tracheal intubation along with the bleeding made manual ventilation progressively more difficult, nearly resulting in a 'cannot intubate, cannot ventilate' scenario. In an attempt to control the bleeding, rFVIIa 4.8 mg iv was administered. Within five minutes, her INR had decreased to 1.1, bleeding was markedly reduced, the vocal cords were successfully visualized using an anterior commissure laryngoscope, and intubation of the trachea was achieved with the use of a gum-elastic bougie. Postintubation examination of the airway showed several abrasions along the right oropharyngeal wall with minimal bleeding. The remainder of surgery and postoperative airway management were uneventful. CONCLUSIONS: This report demonstrates that in the relatively uncommon setting of upper airway hemorrhage in a patient with pre-existing coagulopathy, rFVIIa can be effective in gaining rapid control of bleeding to facilitate visualization of the vocal cords and securing of the airway.

Malignant hyperthermia: considerations for the general surgeon.

Malignant hyperthermia is a potentially fatal genetic myopathy that presents when the patient is under anesthesia. It manifests as a hypermetabolic state involving tachycardia, hypercarbia, base deficit, rigidity and fever. Many of the hallmark traits of an acute malignant hyperthermic crisis overlap with signs and symptoms of an emergent abdominal condition. Historically, there has been a reluctance in local community hospitals to manage patients known to be susceptible to malignant hyperthemia, and this is a source of frustration for many families in which there is a history of this condition. This article outlines the diagnosis and management of an acute malignant hyperthermic crisis from the viewpoint of a community general surgeon and provides a review of the management of patients known to be susceptible to this condition in the surgeon's elective and emergent practice.