Ann Thorac Surg 2006;81:1008-1012
© 2006 The Society of Thoracic Surgeons
Original article: Cardiovascular
Important Excess Morbidity Due to Upper Airway Anomalies in the Perioperative Course in Infant Cardiac Surgery
Jean-Pierre Pfammatter, MD
a
,
*
,
Carmen Casaulta, MD
b
,
Mladen Pavlovic, MD
a
,
Pascal A. Berdat, MD
c
,
Urs Frey, MD, PhD
b
,
Thierry Carrel, MD
c
a Department of Pediatric Cardiology, University Children's Hospital, Berne, Switzerland
b Department of Pediatric Pulmonology, University Children's Hospital, Berne, Switzerland
c Department of Cardiothoracic Surgery, University Children's Hospital, Berne, Switzerland
Accepted for publication September 8, 2005.
* Address correspondence to Dr Pfammatter, Pediatric Cardiology, University Children's Hospital, CH 3010 Berne, Switzerland (Email: jean-pierre.pfammatter{at}insel.ch).
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Abstract
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BACKGROUND: The study aimed at defining the excess morbidity or mortality caused by an additional airway malformation in children with congenital heart disease requiring surgery.
METHODS: All patients requiring surgery for heart disease during an 8-year period ending in 2003 who had an associated upper airway malformation were retrospectively studied. All patients were seen in 2004 for a prospective follow-up examination.
RESULTS: Eleven patients with upper airway anomalies were identified (tracheobronchial malacia in 6 patients, long-segment tracheal stenosis in 3, and bilateral vocal cord paralysis and tracheal hemangioma in 1 patient each). They accounted for 1.5% of the entire cardiac surgical load of 764 patients. In 5 infants, the airway anomaly was diagnosed before cardiac repair, in 6 patients thereafter. Diagnosis was made by bronchoscopy in all patients, by additional bronchography in 2. Failure of rapid postoperative extubation was the most common finding. Airway management was surgical in 2 and conservative in 8 patients, 1 newborn having been denied therapy because of the severity of airway hypoplasia. Compared with patients with isolated cardiac disease, those with additional airway anomalies had significantly longer duration of postoperative mechanical ventilation (median, 24 days versus 3), perioperative hospitalization (median, 72 days versus 11) and total number of days of hospitalization during the first year of life (median, 104 days versus 14). After a maximum follow-up of 8 years (median, 37 months) only 3 of 10 surviving patients remained symptomatic owing to the airway malformation.
CONCLUSIONS: Upper airway anomalies accompanying heart disease in infancy resulted in a significant prolongation of perioperative intensive care and hospital stay, as well as duration of mechanical ventilation. Failure of early postoperative extubation was the leading symptom.
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Introduction
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Among pediatric patients with congenital heart defects, those with additional malformations of other organs present with special problems in the acute perioperative as well as in the longterm management. Because invasive access to the upper airways with intubation and mechanical ventilation is part of the management, children with malformations or functional anomalies of the upper airways show specific and challenging problems in the immediate postoperative period after repair of congenital heart disease.
There are very few data with regard to the excess morbidity or eventually mortality caused by upper airway anomalies associated with congenital heart disease. It was the aim of this study to evaluate the perioperative and postoperative course in infants requring surgery for heart defects in the presence of an additional congenital anomaly of the upper airways.
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Patients and Methods
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By means of a retrospective search in the hospital database, all patients were identified who underwent surgical repair of heart disease in our institution between 1996 and the end of 2003 and in whom associated upper airway anomaly was detected, either preoperatively or postoperatively.
Excluded were patients (n = 7) in whom respiratory symptoms were attributed to the perioperative intubation and not to an independent congenital airway malformation. Patients with external airway compression due to a vessel anomaly (n = 13) were excluded, as these patients were not considered to have primary congenital airway disease and were typically much older when becoming symptomatic.
For the diagnosis of an upper airway anomaly to be affirmatively made, it was requested that at least a bronchoscopy was performed by an experienced pneumologist (flexible bronchoscopy using an Olympus baby bronchoscope 2.2 mm). There are no generally accepted criteria for the diagnosis of tracheobronchial malacia: in our institution the diagnosis was made in children with the typical clinical picture in the presence of a tracheal luminal narrrowing of greater than 50%, and a further expiratory luminal collapse [1].
Follow-up data on all patients were prospectively obtained in the cardiac outpatient clinic during 2004, where patients routinely had a clinical examination, chest roentgenogram, and echocardiogram. Pneumologic evaluation was done only in the presence of persistent respiratory symptoms.
Informed consent was obtained from the families during the prospective follow-up evaluation.
Statistical analysis consisted of median and range for the descriptive data; comparison between groups was made with analysis of variance using a commercially available software (Statview). A p value less than 0.05 was considered significant.
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Results
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Patient Characteristics
During the 8-year study period, a total of 764 pediatric cardiac surgical cases were treated in our institution. Of these, 426 had surgery in infancy. Associated upper airway anomalies were found in 11 patients, accounting for 1.5% of the whole surgical volume, or 2.5% of all infants operated on during that period. Syndromic patients were clearly overrepresented among infants with airway malformations. Six of 11 patients had underlying genetical disease (2 patients each with Down syndrome and microdeletion 22 syndrome, 1 patient each with VACTERL association and Valproate fetopathy). For comparison, syndromic patients accounted for only 13% of the remaining surgical population (102 of 753 patients).
Spectrum and Treatment of Cardiac Disease
Cardiac malformations seen in the patients are shown in Table 1, together with the proportion of patients among all cases operated upon for each individual cardiac diagnosis. Median age at cardiac surgery was 2 weeks (range, 1 to 18). Surgery was corrective in 8 and palliative in 2 infants (Blalock shunt neonatally in a Fallot patient, ductus ligation with tracheal resection while leaving a ventricular septal defect untouched in another newborn). One newborn with total anomalous pulmonary venous return died untreated at the age of 4 days, surgery having been denied because of the severity of tracheobronchial hypoplasia.
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Table 1. Cardiac Diagnosis and Proportion of Patients With Airway Anomaly Related to Total Number of Patients With Identical Cardiac Diagnosis
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Airway Anomalies and Treatment
Airway malformations consisted of tracheobronchial malacia in 6 patients, long segment tracheal stenosis in 3 (involving the whole trachea and proximal bronchii in 1 newborn, and complete tracheal rings involving 4 segments and a stenotic narrowing extending over 4 segments in 1 patient each), and 1 patient each had bilateral congenital vocal cord paralysis and a large tracheal hemangioma.
Diagnosis of airway anomalies was made by flexible bronchoscopy in all patients with additional bronchography in 2 newborns. The main symptoms leading to the diagnosis are shown in detail in Table 2, the main feature in all patients diagnosed postoperatively having been failure of timely postoperative extubation. Failure of early extubation was seen in addition in 2 more patients in whom the diagnosis of airway malformation has already been made preoperatively. Treatment of airway anomalies was surgical in 2 patients: the newborn with Fallot's tetralogy and with vocal cord paralysis required trachestomy; and in another newborn with ventricular septal defect complete tracheal rings involving 4 segments, a tracheal resection with end-to-end anastomosis was done as a first step together with ligation of a persistent ductus. In 8 patients, airway management was conservative, including prolonged home oxygen (for a period of 2 to 5 months) in 5 patients during infancy. In 1 newborn with total anomalous pulmonary venous return presenting with severe respiratory distress syndrome neonatally, a severe form of global tracheobronchial hypoplasia was discovered at a failed attempt of intubation before cardiac catheterization. Treatment was denied to this newborn owing to the severity of both malformations.
Outcome of Patients
The follow-up of the patients ranged from 1 to 8 years (median, 37 months). Except for 2 reinterventions (balloon dilatation of recoarctation and conduit replacement in truncus arteriosus), the cardiac follow-up was uneventful, and all surviving patients are asymptomatic and off all drugs. Respiratory symptoms were absent in all patients after the first year of life, except for 2 patients with tracheomalacia having residual minor symptoms (stridor) during airway infection and for the patient with the tracheal hemangioma who is now at 4 years of life being evaluated for definitive therapy. Five infants had prolonged oxygen therapy at home for a maximum of 5 months. Two patients required rehospitalization owing to an infection with respiratory syncytial virus. Except for the patient who was denied therapy, there was no excess mortality in the patients studied. When compared with the patients with identical cardiac diagnosis, patients with additional airway disease had significantly longer duration of perioperative intubation (p < 0.05), perioperative hospitalization (p < 0.05), and overall days in hospital in the first year (p < 0.05) of life as outlined in Table 3. A comprehensive overview of the individual patient data is shown in Table 4.
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Comment
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The current study showed that malformations of the upper airways were a rare finding complicating the perioperative course in 2.5% of an unselected population of infants undergoing cardiac surgery. The morbidity associated with airway anomalies was relevant with a significantly prolonged duration especially of postoperative mechanical ventilation. It was further shown that failure of early postoperative extubation was a predominant clinical sign that should prompt search of an additional airway problem in cardiac patients, especially in children with underlying syndromic disease who were significantly overrepresented among the study patients.
Several studies have focused on the association between congenital heart disease and additional airway malformations, usually describing mixed populations of pediatric cardiac patients with primary congenital and secondary airway disease. In such heterogenous populations of children with airway malformations and also with extrinsic mechanical airway compression, it has already been described that prolonged mechanical ventilation was a leading clinical feature in these patients [2]. In a similarly heterogenous study population, it was shown that complex cardiac disease was a risk factor for increased morbidity and (in contrast to our study) also mortality of airway disease, which amounted to 29% in an intensive care unit population of 48 children [3]. In the same study, prolonged mechanical ventilation has also been identified as one predominant feature in the majority of patients. With a median of 38 days, the duration of mechanical ventilation has been in a similar range as in our study patients. Independent of the quite uniform symptomatology of these patients, it has been shown that external compression of the upper airways and predominantly of the left main bronchus were much more frequently the cause of perioperative and postoperative complications than intrinsic congenital airway malformations [4, 5].
The spectrum of underlying heart disease is wide and unspecific [2], although a significant incidence of airway malformations (11%) has been described in individual heart defects such as tetralogy of Fallot [6]. The numbers of affected patients in our study was too small to draw conclusions of the increased proportion of patients with airway anomalies among children with total anomalous pulmonary venous drainage and truncus arteriosus. The increased prevalence of airway malformations in patients with underlying genetical and syndromic disease was not a new finding, but has already been recognized by other authors as well [7, 8].
Bronchoscopy is the method of choice for a rapid diagnosis of airway disease, especially in the setting of an intensive care unit [4]. Bronchography [3] or computed tomography [9] may serve as alternative or supplementary diagnostic methods. These tools should be used liberally when postoperative extubation after cardiac repair is delayed. In our institution, an airway problem is actively sought in case a postoperative child cannot be weaned from mechanical ventilation after several days (depending on the type of surgery performed), when a residual surgical problem has been ruled out, when there are no signs of a low cardiac output, and when a significant pulmonary problem has been excluded. A high level of suspicion is warranted in children who already preoperatively manifest with clinical signs of possible additional airway obstruction. In these cases, we now advocate bronchoscopy by a pneumologist at the time of the immediate preoperative intubation.
Having in mind the difficulties encountered in the invasive management of these upper airway anomalies [10–12], it was important to find that the majority of our patients had a good long-term outcome with a conservative approach alone, whereas only in a minority of our patients (2 of 10 infants) was surgical treatment required.
Conclusion and Clinical Implications
Among infants undergoing cardiac surgery, there is a relatively small proportion affected with additional airway malformations leading to increased perioperative and postoperative morbidity. Prolonged need for postoperative mechanical respiratory support was a predominant feature in these patients, and should prompt a diagnostic work-up for an underlying airway anomaly. The long-term prognosis of these patients either for the cardiac disease or the respiratory symptoms was good.
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References
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Abstract
Introduction
