Anesth Analg 2006;103:1432-1435
© 2006 International Anesthesia Research Society
doi: 10.1213/01.ane.0000243331.61117.f2
PEDIATRIC ANESTHESIA
Anesthetic Management of a Young Adult with Complex Congenital Heart Disease and Bronchopleural Fistula for Rigid Bronchoscopy
Katherine M. Bailey, MD*,
Erin A. Gottlieb, MD
,
Joseph L. Edmonds, Jr, MD
, and
Wanda C. Miller-Hance, MD, FACC
From the *Department of Anesthesia, British Columbia Children’s Hospital, Vancouver, BC, Canada; Departments of Pediatrics and Anesthesiology, Baylor College of Medicine; and Texas Pediatric Otolaryngology Center, Texas Children’s Hospital, Houston, Texas.
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Abstract
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The number of adults with congenital heart disease and those who require anesthetic care are increasing. We describe the anesthetic management of a young adult with palliated complex congenital heart disease and a chronic postsurgical bronchopleural fistula for rigid bronchoscopy. Perioperative considerations in the care of patients with single ventricle physiology for noncardiac procedures are reviewed. Specific requirements for rigid bronchoscopy are discussed in addition to the anesthetic implications of a bronchopleural fistula and particular concerns in the patient with single ventricle physiology.
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Introduction
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More infants and children with congenital cardiovascular defects are surviving into adulthood and presenting for noncardiac surgery or procedural sedation. Many of these patients continue to have abnormal cardiac anatomy, function, and/or circulation that may increase the risks associated with anesthesia and the surgical intervention or procedure. In this report, we describe the perioperative care of a patient with complex single ventricle physiology and a bronchopleural fistula resulting from prior lung resection surgery requiring rigid bronchoscopy for removal of a foreign body. The considerations and challenges in the management of this patient are discussed.
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CASE REPORT
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A 27-year-old, 38.7 kg male with a history of double-inlet left ventricle, malposed great arteries, pulmonary atresia, and a palliative aortopulmonary shunt performed in infancy presented for rigid bronchoscopy and removal of an airway foreign body. Cardiac catheterization at the age of 18 yr demonstrated acquired atresia of the right pulmonary artery and pulmonary blood flow originating from a restrictive direct anastomosis between the descending aorta and left pulmonary artery (Pott’s shunt) (Fig. 1). This was associated with mild increases of left pulmonary artery pressures (36/25 mm Hg, mean pressure of 28 mm Hg). When the patient was 20 yr of age, a right upper lobectomy was performed because of a cavitating pulmonary infection. This was complicated by prolonged air leakage and a chronic postoperative pleural space problem without associated pleural effusion. Because of the potential morbidity associated with surgical intervention to address a bronchopleural fistula in the patient with single ventricle physiology, conservative management had been recommended.
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Figure 1. Diagramatic representation of cardiovascular pathology in this patient (double inlet left ventricle, pulmonary trunk atresia, isolated left pulmonary artery, postoperative Pott’s shunt).
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Six months before the scheduled procedure, the patient developed a persistent, incapacitating nonproductive cough. Four months later, an episode of decompensated heart failure and hemoptysis led to his hospitalization. Cardiac magnetic resonance imaging and computerized chest tomography demonstrated severe left ventricular (single ventricle) dysfunction (ejection fraction of 25%) and numerous aortopulmonary collaterals to the right lung. A large cystic cavity was noted in the right upper thorax in addition to findings consistent with a chronic bronchopleural fistula. Pulmonary function testing documented a severe restrictive defect in addition to a mild obstructive component without significant bronchodilator response.
The week prior to the planned procedure, the patient underwent cardiac catheterization/angiography and flexible bronchoscopy to assess the etiology of the persistent cough and hemoptysis. Bronchoscopy confirmed the presence of a bronchopleural fistula. In addition, suture material from the bronchial stump was observed to be protruding into the carina. Rigid bronchoscopy was scheduled to remove the suture material, as it was considered the likely etiology of chronic airway irritation and intractable cough.
During the immediate preoperative assessment, the patient’s clinical status was that of compensated heart failure with a functional capacity consistent with NY Heart Association Class III. Vital signs: heart rate 97 beats per minute, respiratory rate 18 breaths per minute, and arterial blood pressure 114/62 mm Hg. Pulse oximetry demonstrated an oxygen saturation of 80% on room air. Medications included aspirin, furosemide, carvedilol, and losartan. The patient’s hemoglobin concentration was 20.8 g/dL. Transthoracic echocardiography was suboptimal for diagnostic assessment secondary to poor windows.
A peripheral IV catheter was placed and isotonic fluid was administered to replace the intravascular volume deficit related to the period of fasting. After premedication with IV midazolam, standard ASA monitors were applied. Standard and advanced airway equipment was immediately available. Equipment for emergent institution of advanced circulatory support in the form of cardiopulmonary bypass or extracorporeal membrane oxygenation was available, although not set up in the operating room, as the need for these was considered unlikely. Anesthesia was induced using a combined IV and inhaled technique. Small doses of etomidate were titrated and increasing concentrations of sevoflurane were administered, maintaining spontaneous ventilation. The vocal cords and trachea were anesthetized with aerosolized lidocaine given by the surgeon. Supplemental oxygen was administered throughout the procedure and anesthesia was maintained with continuous infusions of low-dose propofol (20–50 mcg · kg–1 · min–1) and remifentanil (0.1 mcg · kg–1 · min–1). Anesthetic depth was titrated to the level of airway stimulation by supplementation with low concentrations of sevoflurane (<0.8% end-tidal). A ventilating bronchoscope was inserted into the trachea and the presence of suture material that originated from the right bronchial stump and that oscillated in and out of the carina throughout the respiratory cycle was confirmed (Fig. 2, left panel). The redundant suture was cut and removed without difficulty (Fig. 2, right panel). The patient tolerated the 1-h procedure well. Mild reductions in systemic arterial blood pressure upon emergence were managed by the administration of IV fluid and boluses of phenylephrine (50–100 mcg total) to maintain a mean arterial blood pressure within 20% of baseline. His oxygen saturation and end-tidal carbon dioxide concentration remained stable throughout the procedure (75–85% and 40–50 mm Hg, respectively). His arterial carbon dioxide concentration was likely much higher, although not directly measured. During the course of the anesthetic, the patient also received ondansetron, decadron, and ampicillin IV. He recovered uneventfully and was discharged home after 6 h of observation in the postanesthesia care unit with arrangements for follow-up. Over the ensuing months, his cough improved dramatically and his condition has remained stable.
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Figure 2. Left Panel, bronchoscopic view during exhalation demonstrating suture material originating from the right main stem bronchus and extending into the carina and distal trachea (Rt = right; Lf = left). Right Panel, the figure depicts the suture material removed during rigid bronchoscopy.
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DISCUSSION
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Clinical outcome in congenital heart disease is highly dependent on the anatomic diagnosis and the success of palliation or "correction." A palliated patient continues to have an abnormal circulation, although the surgical intervention should improve the stability of the circulation and decrease the likelihood of the severe repercussions of the disease. In the adult patient with palliated congenital heart disease, a number of acquired comorbidities may result from long-standing cyanosis, including the detrimental effects of chronic polycythemia and hyperviscosity. Other sequelae, several of which were present in this case, include rhythm disturbances, pulmonary hypertension, ventricular dysfunction, and heart failure (1,2).
The anesthetic management of adult patients with congenital heart disease undergoing noncardiac surgery is in many cases far from routine, and is significantly influenced by the natural history of the defect, continuing hemodynamic perturbations, and nature of the planned intervention. In the patient with single ventricle physiology, myocardial performance is likely to be negatively impacted by long-standing alterations in cardiac chamber pressures and/or volumes, as well as chronic hypoxemia. This may place these individuals at increased risk for hemodynamic compromise or cardiovascular collapse during anesthesia if further reductions in myocardial contractility, systemic vascular resistance, or myocardial blood flow occur.
Our patient presented a number of concerns (Table 1) and an appraisal of these anesthetic considerations in addition to the nature of the anticipated surgical procedure allowed us to formulate management goals and plan accordingly.
A primary objective was to maintain hemodynamic variables and arterial oxygen saturation near baseline values and to avoid significant alterations in myocardial function. Volatile anesthetics have been shown to significantly decrease cardiac output by causing reductions in both myocardial contractility and systemic vascular resistance (3–5). Propofol may cause significant reductions in peripheral vascular resistance, and mild myocardial depression has also been reported (6,7). Opioids, although lacking amnestic properties, may provide greater cardiac stability with minimal changes in heart rate and arterial blood pressure (8).
Our goal was to choose an anesthetic technique that would minimize hemodynamic changes elicited by intense airway stimulation, avoid awareness, and allow for optimal operating conditions. The continuous infusion of two IV drugs, propofol and remifentanil, allowed for the administration of relatively low doses of each, minimizing the negative side effects associated with the use of either drug alone at a higher dose. At the same time, we were able to maintain a constant level of adequate anesthetic depth, particularly during periods when the airway was instrumented and end-tidal concentrations of sevoflurane were variable. The supplemental administration of sevoflurane provided for rapid adjustments of anesthetic depth during anticipated periods of intense airway stimulation without the need for additional boluses of IV drugs or frequent adjustments in the infusion rates that could have resulted in acute cardiorespiratory changes, or may have distracted from patient care.
It is critical to preserve the balance between pulmonary and systemic blood flow and to maintain arterial oxygenation when caring for patients with shunt-dependent pulmonary circulation. Factors that may influence arterial oxygenation in these patients include systemic blood pressure, oxygen-carrying capacity, and pulmonary vascular tone. In our patient, the restrictive nature of the aortopulmonary connection (Pott’s shunt) and aortopulmonary collaterals provided limited and relatively fixed pulmonary blood flow. By ensuring adequate intravascular volume status and oxygenation, and by minimizing anesthetic-related decreases in systemic vascular resistance, we were able to avoid significant reductions in pulmonary blood flow and arterial oxygen saturation during the procedure.
Additional anesthetic considerations were required in this patient because of the presence of a bronchopleural fistula. The communication between the bronchial tree and pleural cavity resulted as a complication of a prior pulmonary resection, as is the case in most patients with this condition. An awake, spontaneously breathing patient with a chronic bronchopleural fistula may be asymptomatic. However, when anesthetized and placed under positive pressure ventilation, hypoxemia and/or hypercarbia can develop secondary to loss of tidal volume into the pleural space. If a thoracostomy tube is not present, a tension pneumothorax may develop and that, if unrecognized, can lead to cardiovascular collapse. In addition, if the bronchopleural fistula is associated with an empyema, positive pressure ventilation without lung isolation can result in contamination of the controlateral lung. For these reasons, spontaneous ventilation is often preferred in patients with this condition. If a patient with a bronchopleural fistula must be mechanically ventilated, certain strategies can be used to reduce loss of tidal volume through a large low resistance communication and promote fistula closure in acute cases (9). Such maneuvers include ventilating with low tidal volumes, limiting positive end-expiratory pressure, reducing the respiratory rate and shortening the inspiratory time. More advanced interventions include single lung ventilation, chest tube valve systems, high frequency ventilation, and differential lung ventilation. Anesthesiologists should be alert to the possibility of tension pneumothorax.
Consequences of a bronchopleural fistula in the patient with single ventricle physiology can be extremely serious. The patient with ductal-dependent pulmonary circulation, an aortopulmonary shunt, or a bidirectional Glenn connection may suffer a significant decrease in pulmonary blood flow related to an acute increase in pulmonary vascular resistance. This may result from hypoxemia and hypercarbia because of the inability to effectively oxygenate and ventilate secondary to the loss of tidal volume through the bronchopleural fistula. In addition, the decrease in preload as a result of a tension pneumothorax may be poorly tolerated in these patients and, in particular, those with Fontan physiology. An increased intrathoracic pressure associated with a tension pneumothorax and resultant severe impairment in oxygenation, ventilation, ventricular diastolic filling, and systemic cardiac output would have likely resulted in a catastrophic, devastating event in our already compromised patient.
In our case, the benefits of a balanced anesthetic technique during bronchoscopy using a muscle relaxant to facilitate positive-pressure ventilation would include: 1) the ability to limit the use of other drugs and their concomitant negative effects on cardiac output, 2) maintenance of oxygenation by avoiding atelectasis and overcoming airway resistance related to the instrumenting hardware, and 3) enhanced ability to ensure adequate minute ventilation and normocarbia (10). As the current prior anesthetic in this patient documented adequate ventilation with conventional methods, we considered the need for sophisticated ventilatory techniques that may be required in patients with a bronchopleural fistula unlikely. We chose to maintain spontaneous ventilation with a low threshold to initiate controlled ventilation at the minimum necessary peak inspiratory pressure, if an alteration in technique was required to maintain gas exchange. Rigid bronchoscopy performed under spontaneous ventilation requires the maintenance of an adequate anesthetic depth to avoid laryngospasm, breathholding, and inadequate ventilation that may contribute to hypoxemia, hypercarbia, and potential alterations in pulmonary vascular tone. The baseline oxygen saturation of 80% placed our patient on the sharp slope of the oxyhemoglobin dissociation curve. Although with shunt-dependent pulmonary blood flow, systemic arterial saturation is not a direct reflection of alveolar oxygen tension, we were, nonetheless, concerned that an airway-related event, triggered by an inadequate level of anesthesia without an endotracheal tube could potentially contribute to rapid arterial desaturation. The selection of remifentanil was based on our clinical observations and those of others regarding the decrease in airway reactivity provided during instrumentation with careful titration of this drug (11,12).
As in all patients with structural heart disease, we followed the recommendations outlined by the American Heart Association for endocarditis prophylaxis (13).
In summary, we report the anesthetic management of a patient with palliated congenital heart disease, single ventricle physiology, and a chronic bronchopleural fistula undergoing rigid bronchoscopy, highlighting many of the considerations involved in the care of patients with complex cardiac anatomy and physiology. As the population of adults with congenital heart disease continues to increase, the need for noncardiac interventions in these patients will continue to grow. Although the favorable outcome in this case cannot be attributed to a unique strategy, specific regimen, or novel anesthetic technique, our report emphasizes the critical importance of careful assessment of considerations related to the individual, the anesthetic and the planned procedure when caring for these patients. A thorough appraisal of these considerations may limit perioperative morbidity and mortality in adult patients with complex congenital heart disease.
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Footnotes
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Accepted for publication August 14, 2006.
Addresss correspondence and reprint requests to Wanda C. Miller-Hance, MD, Division of Pediatric Cardiovascular Anesthesiology, Texas Children’s Hospital, 6621 Fannin St., WT 19345H, Houston, TX 77578. Address e-mail to wcmiller{at}texaschildrenshospital.org.
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- Holzman RS, van der Velde ME, Kaus SJ, et al. Sevoflurane depresses myocardial contractility less than halothane during induction of anesthesia in children. Anesthesiology 1996;85: 1260–7.[Web of Science][Medline]
- Rivenes SM, Lewin MB, Stayer SA, et al. Cardiovascular effects of sevoflurane, isoflurane, halothane, and fentanyl-midazolam in children with congenital heart disease: an echocardiographic study of myocardial contractility and hemodynamics. Anesthesiology 2001;94:223–9.[Web of Science][Medline]
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Abstract
Introduction
