Case Report
Lung Decortication and Lobectomy in a Child With Unrepaired Tetralogy of Fallot
World Journal for Pediatric and Congenital Heart Surgery 4(4) 430-432 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113493017 pch.sagepub.com
Brijindera Singh Sethi, MD1, Kushant Gupta, MD1, Sandeep Chauhan, MD1, and Shiv Kumar Choudhary, Mch2
Abstract Patients with uncorrected tetralogy of Fallot (TOF) have been reported as undergoing emergency noncardiac surgeries such as cesarean section, brain abscess drainage, and major abdominal surgery. The uncorrected TOF group presents a great challenge with issues related to long-term effects of chronic hypoxemia and decreased pulmonary blood flow modifying patient physiology. We report a rare case of a child with uncorrected TOF with necrotizing streptococcal pneumonia complicated by empyema and bronchopleural fistula. The child successfully underwent lung decortication and right middle lobectomy in the first stage followed by an intracardiac repair (ICR) 15 days later. This staged approach was directed at controlling the infective focus, improving the pulmonary status, and following it up with a definitive ICR electively. Keywords tetralogy of Fallot, decortication, lobectomy Submitted March 14, 2013; Accepted May 16, 2013.
Introduction Noncardiac surgery in children with uncorrected congenital heart disease (CHD) is associated with increased risk of complications.1 There are reports of patients with uncorrected tetralogy of Fallot (TOF) undergoing various emergency surgeries like cesarean section,2 brain abscess drainage,3 and major abdominal surgeries.4 A patient with uncorrected TOF undergoing lung decortication and lobectomy has been rarely reported.5 We report one such case and discuss our concerns regarding potential life-threatening hypoxemia and the decision to perform lobectomy and intracardiac repair (ICR) as a staged procedure vis-a`-vis single setting.
Case Report A 10-year-old child from a remote rural area with little access to health care services presented to our hospital with fever, breathlessness, and cough for the past two months. He also gave history of cyanosis and recurrent spells since two months of age. Hemoglobin was 23 g/dL with a rise in total leukocyte count. Echocardiography revealed a large subaortic malaligned ventricular septal defect, severe valvular, and infundibular pulmonary stenosis (peak systolic gradient of 86 mm Hg) with aortic override and right ventricular hypertrophy. The pulmonary arteries (PAs) were confluent (both right and left PA 9 mm), and descending aorta was 12 mm in size. Computed tomographic (CT) angiography confirmed the findings of echocardiography
and showed few small aortopulmonary collaterals and normal coronary anatomy. Multiple thin-walled cavitatory lesions were seen in both the lung fields with areas of consolidation in the surrounding lung tissue. The child was being planned for an ICR when he developed a spontaneous right-sided pneumothorax for which intercostal drainage tube was inserted. Within a week, the child developed empyema and bronchopleural fistula (BPF) and was considered temporarily unfit for ICR. Blood culture grew Streptococcus pneumoniae sensitive to ciprofloxacin and vancomycin. The child was given ciprofloxacin, vancomycin, and later amphotericin B was added empirically. Serum immunoglobulin levels of immunoglobulin (Ig) G (IgG), IgM, and IgA were within normal limits, and screening for human immunodeficiency virus was negative. As a bailout procedure to increase systemic oxygen saturation (SaO2 on room air 71%), pulmonary valve balloon dilation (PVBD) was attempted unsuccessfully. Despite adequate antibiotic therapy and optimal chest tube placement, the response
1 Department of Cardiac Anaesthesiology, All India Institute of Medical Sciences, New Delhi, India 2 Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, India
Corresponding Author: Brijindera Singh Sethi, Department of Cardiac Anaesthesia, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India. Email: [email protected]
Sethi et al
431
Acronyms and Abbreviations BPF CHD CPB CT DLT ICR ICU Ig OLV PA PVBD SaO2 TOF
bronchopleural fistula congenital heart disease cardiopulmonary bypass computed tomographic double lumen tube intracardiac repair intensive care unit immunoglobulin one-lung ventilation pulmonary artery pulmonary valve balloon dilation oxygen saturation tetralogy of Fallot
to treatment was poor, and the child continued to be febrile with tachypnea and increasing cyanosis. Repeat CT chest revealed a right pyopneumothorax with persistent collapse, consolidation of the right middle and lower lobe, and communication of subsegmental bronchi with the right pleural space (Figure 1). The child was posted for right thoracotomy, lung decortication, and right middle lobectomy. Inside the operation theater, standard noninvasive monitoring was applied, and induction was performed with ketamine and fentanyl. Intubation was facilitated with rocuronium. A left-sided double lumen tube (DLT) of 26F was inserted, and its position was confirmed by fiberoptic bronchoscopy. Anesthesia was maintained with oxygen air, isoflurane, fentanyl, and vecuronium. Invasive arterial pressure, central venous pressure, and nasopharyngeal temperature were monitored throughout the procedure. Onelung ventilation (OLV) was initiated with 100% oxygen to maintain SaO2 of 75%, PaO2 of 46 to 49 mm Hg, and PaCO2 between 30 and 40 mm Hg. On opening the right hemithorax, there was a thick fibrotic feel over the entire right lung with pus pockets in the middle and lower part of the pleural cavity. Purulent material was removed, and a large BPF involving the right middle lobe was identified. Complete decortication and right middle lobectomy were performed. After anesthetic induction, SaO2 was 75% and remained between 71% and 75% throughout the procedure, but after completion of lobectomy and resumption of ventilation to both lungs, the SaO2 on FiO2 0.5 improved to 79% (PaO2 52 mm Hg). Intravascular volume was maintained with Ringer lactate and hydroxyl-ethyl starch. The patient was shifted to the intensive care unit (ICU) after replacing the DLT with a 6.0mm-internal diameter cuffed endotracheal tube. The child desaturated on initial weaning and could finally be extubated on the third postoperative day. No organism was grown in the pus cultures obtained intraoperatively from the pleural cavity and excised lung tissue. The ICU stay was seven days, and meropenem, teicoplanin, and amphotericin B were continued in the postoperative period for 14 days. The patient became afebrile with satisfactory parenchymal reexpansion and resolution of the air leaks on the 11th postoperative day. After 15 days, the patient was taken up for ICR of TOF on cardiopulmonary bypass (CPB) with a transannular patch
Figure 1. Computed tomographic virtual bronchoscopy image showing a large pneumothorax with lung collapse and an intercostal chest tube in situ.
augmentation of the right ventricular outflow tract using autologous pericardium. The patient was discharged from the hospital 11 days after the definitive procedure in stable condition.
Discussion Patients with CHD have a 3.5-fold increased risk of perioperative complications with noncardiac surgery, depending on the extent of surgery, severity of underlying abnormality, cyanosis, and heart failure.6 Lung resection surgery in a child with uncorrected TOF can pose a severe challenge. Both the conditions can cause profound life-threatening hypoxemia independently as well as cumulatively. The TOF with its chronic hypoxemic state has profound hematologic effects. Polycythemia improves oxygen transport at the expense of an increased viscosity that puts the patient at a risk of thrombosis and stroke.7 Platelet function abnormalities and multiple coagulation factor deficiencies, common in these patients, increase the risk of bleeding from raw-exposed surfaces of lung after decortication and lobectomy. Chronic hypoxia induces myocardial dysfunction with reduction in ventricular diastolic compliance and myocardial reserve.8 The poor myocardial reserve along with decreased pulmonary reserve due to patient’s lung condition places the patient at an increased risk of decompensation. Our concern was whether this child will tolerate OLV with its attendant further increase in R–L shunt. Our strategy was to preserve hypoxic pulmonary vasoconstriction and if required to clamp the right PA and keep CPB standby. The dilemma we faced was whether to perform surgery including lobectomy and ICR of TOF in a single setting or as a staged repair. We considered the following four options. The
432 first option we had was to attempt PVBD to improve SaO2; however, it was unsuccessful due to infundibular hypertrophy. The second option was to perform ICR and lobectomy in a single setting. The presence of empyema as a focus of infection in a patient with febrile with increased leukocyte count was a deterrent. The inflammatory response accompanying the CPB would have placed the patient at an increased risk of multiorgan dysfunction syndrome. The advantage of this single setting surgery would have been an improved PaO2 in the postoperative period along with increased ability to fight infection and better wound healing. The third option of lobectomy along with surgical palliation of TOF like a left-modified Blalock-Taussig shunt was considered risky, as ventilating the diseased lung (right) with major air leak and retraction of the healthy lung (left) with a clamp on the left PA would have lead to severe hypoxia. Rightmodified Blalock-Taussig shunt was not considered because of proximity to the infective focus. The option that we chose was for lobectomy in the first stage with CPB standby (for emergency ICR) followed by an elective ICR at a later date. This in our opinion was essential to control infection and improve the patient’s pulmonary status prior to taking up for surgical correction of TOF. We found that SaO2 on FiO2 0.5 increased from 71% preinduction to 79% after the lobectomy, and decortication was completed, probably due to removal of the diseased segment, better expansion of the adjacent fibrotic lung, and diversion of blood away from this zone of wasted perfusion. Karthekeyan et al reported carrying out lobectomy for congenital bronchiectasis and intracardiac TOF repair in the same setting as there was no active infection, and anatomy was favorable, that is, confluent good-sized PAs, normal coronaries, and no associated cardiac defects.5 Pikwer et al reported a patient, where lobectomy was performed for pulmonary sequestration in a child with uncorrected TOF followed by ICR eight months later.9 In contrast to the above-mentioned reports, our case was complicated by the presence of active infection (not responding to medical management) in addition to a major air leak from the BPF. To the best of our knowledge, the association of necrotizing streptococcal pneumonia in a immunocompetent child with TOF requiring surgical intervention has not been reported previously. Through this report, we tried to highlight the risks involved and the various treatment options
World Journal for Pediatric and Congenital Heart Surgery 4(4) available to a clinician in the care of a cyanotic child with a fulminant pulmonary pathology. In conclusion, the staged approach to surgical repair of TOF along with lobectomy for a severe pulmonary infection may be an acceptable option for a favorable outcome. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Hennein HA, Mendeloff EN, Cilley RE, Bove EL, Coran AG. Predictors of postoperative outcome after general surgical procedures in patients with congenital heart disease. J Pediatr Surg. 1994; 29(7): 866-870. 2. Katsetos CP, Kontoyannis MB, Koumousidis A, Petropoulou O, Delos C, Katsoulis M. Uncorrected tetralogy of Fallot and pregnancy: a case report. Clin Exp Obstet Gynecol. 2012;39(3): 382-383. 3. Raha A, Ganjoo P, Singh A, Tandon MS, Singh D. Surgery for brain abscess in children with cyanotic heart disease: an anesthetic challenge. J Pediatr Neurosci. 2012;7(1): 23-26. 4. Haack M, Machotta A, Boemke W, Ho¨hne C. Anesthesia in an infant with uncorrected tetralogy of Fallot for transanal pullthrough for Hirschprung’s disease. Paediatr Anaesth. 2006;16(1): 95-96. 5. Karthekeyan BR, Ravullapalli H, Vakamudi M, Thangavelu P. A rare case of tetralogy of Fallot with congenital tubercular bronchiectasis. Indian J Thorac Cardiovasc Surg. 2010;26(1): 34-37. 6. Baum VC, Barton DM, Gutgesell HP. Influence of congenital heart disease on mortality after noncardiac surgery in hospitalized children. Pediatrics. 2000;105(2): 332-335. 7. Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol. 1996; 28(3): 768-772. 8. Lovell AT. Anaesthetic implications of grown up congenital heart disease. Br J Anaesth. 2004;93(1): 129-139. 9. Pikwer A, Gyllstedt E, lillo-Gil R, Jo¨nsson P, Gudbjartsson T. Pulmonary sequestration—a review of 8 cases treated with lobectomy. Scand J Surg. 2006;95(3): 190-194.
Lung Decortication and Lobectomy in a Child With Unrepaired Tetralogy of Fallot
World Journal for Pediatric and Congenital Heart Surgery 4(4) 430-432 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113493017 pch.sagepub.com
Brijindera Singh Sethi, MD1, Kushant Gupta, MD1, Sandeep Chauhan, MD1, and Shiv Kumar Choudhary, Mch2
Abstract Patients with uncorrected tetralogy of Fallot (TOF) have been reported as undergoing emergency noncardiac surgeries such as cesarean section, brain abscess drainage, and major abdominal surgery. The uncorrected TOF group presents a great challenge with issues related to long-term effects of chronic hypoxemia and decreased pulmonary blood flow modifying patient physiology. We report a rare case of a child with uncorrected TOF with necrotizing streptococcal pneumonia complicated by empyema and bronchopleural fistula. The child successfully underwent lung decortication and right middle lobectomy in the first stage followed by an intracardiac repair (ICR) 15 days later. This staged approach was directed at controlling the infective focus, improving the pulmonary status, and following it up with a definitive ICR electively. Keywords tetralogy of Fallot, decortication, lobectomy Submitted March 14, 2013; Accepted May 16, 2013.
Introduction Noncardiac surgery in children with uncorrected congenital heart disease (CHD) is associated with increased risk of complications.1 There are reports of patients with uncorrected tetralogy of Fallot (TOF) undergoing various emergency surgeries like cesarean section,2 brain abscess drainage,3 and major abdominal surgeries.4 A patient with uncorrected TOF undergoing lung decortication and lobectomy has been rarely reported.5 We report one such case and discuss our concerns regarding potential life-threatening hypoxemia and the decision to perform lobectomy and intracardiac repair (ICR) as a staged procedure vis-a`-vis single setting.
Case Report A 10-year-old child from a remote rural area with little access to health care services presented to our hospital with fever, breathlessness, and cough for the past two months. He also gave history of cyanosis and recurrent spells since two months of age. Hemoglobin was 23 g/dL with a rise in total leukocyte count. Echocardiography revealed a large subaortic malaligned ventricular septal defect, severe valvular, and infundibular pulmonary stenosis (peak systolic gradient of 86 mm Hg) with aortic override and right ventricular hypertrophy. The pulmonary arteries (PAs) were confluent (both right and left PA 9 mm), and descending aorta was 12 mm in size. Computed tomographic (CT) angiography confirmed the findings of echocardiography
and showed few small aortopulmonary collaterals and normal coronary anatomy. Multiple thin-walled cavitatory lesions were seen in both the lung fields with areas of consolidation in the surrounding lung tissue. The child was being planned for an ICR when he developed a spontaneous right-sided pneumothorax for which intercostal drainage tube was inserted. Within a week, the child developed empyema and bronchopleural fistula (BPF) and was considered temporarily unfit for ICR. Blood culture grew Streptococcus pneumoniae sensitive to ciprofloxacin and vancomycin. The child was given ciprofloxacin, vancomycin, and later amphotericin B was added empirically. Serum immunoglobulin levels of immunoglobulin (Ig) G (IgG), IgM, and IgA were within normal limits, and screening for human immunodeficiency virus was negative. As a bailout procedure to increase systemic oxygen saturation (SaO2 on room air 71%), pulmonary valve balloon dilation (PVBD) was attempted unsuccessfully. Despite adequate antibiotic therapy and optimal chest tube placement, the response
1 Department of Cardiac Anaesthesiology, All India Institute of Medical Sciences, New Delhi, India 2 Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, India
Corresponding Author: Brijindera Singh Sethi, Department of Cardiac Anaesthesia, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India. Email: [email protected]
Sethi et al
431
Acronyms and Abbreviations BPF CHD CPB CT DLT ICR ICU Ig OLV PA PVBD SaO2 TOF
bronchopleural fistula congenital heart disease cardiopulmonary bypass computed tomographic double lumen tube intracardiac repair intensive care unit immunoglobulin one-lung ventilation pulmonary artery pulmonary valve balloon dilation oxygen saturation tetralogy of Fallot
to treatment was poor, and the child continued to be febrile with tachypnea and increasing cyanosis. Repeat CT chest revealed a right pyopneumothorax with persistent collapse, consolidation of the right middle and lower lobe, and communication of subsegmental bronchi with the right pleural space (Figure 1). The child was posted for right thoracotomy, lung decortication, and right middle lobectomy. Inside the operation theater, standard noninvasive monitoring was applied, and induction was performed with ketamine and fentanyl. Intubation was facilitated with rocuronium. A left-sided double lumen tube (DLT) of 26F was inserted, and its position was confirmed by fiberoptic bronchoscopy. Anesthesia was maintained with oxygen air, isoflurane, fentanyl, and vecuronium. Invasive arterial pressure, central venous pressure, and nasopharyngeal temperature were monitored throughout the procedure. Onelung ventilation (OLV) was initiated with 100% oxygen to maintain SaO2 of 75%, PaO2 of 46 to 49 mm Hg, and PaCO2 between 30 and 40 mm Hg. On opening the right hemithorax, there was a thick fibrotic feel over the entire right lung with pus pockets in the middle and lower part of the pleural cavity. Purulent material was removed, and a large BPF involving the right middle lobe was identified. Complete decortication and right middle lobectomy were performed. After anesthetic induction, SaO2 was 75% and remained between 71% and 75% throughout the procedure, but after completion of lobectomy and resumption of ventilation to both lungs, the SaO2 on FiO2 0.5 improved to 79% (PaO2 52 mm Hg). Intravascular volume was maintained with Ringer lactate and hydroxyl-ethyl starch. The patient was shifted to the intensive care unit (ICU) after replacing the DLT with a 6.0mm-internal diameter cuffed endotracheal tube. The child desaturated on initial weaning and could finally be extubated on the third postoperative day. No organism was grown in the pus cultures obtained intraoperatively from the pleural cavity and excised lung tissue. The ICU stay was seven days, and meropenem, teicoplanin, and amphotericin B were continued in the postoperative period for 14 days. The patient became afebrile with satisfactory parenchymal reexpansion and resolution of the air leaks on the 11th postoperative day. After 15 days, the patient was taken up for ICR of TOF on cardiopulmonary bypass (CPB) with a transannular patch
Figure 1. Computed tomographic virtual bronchoscopy image showing a large pneumothorax with lung collapse and an intercostal chest tube in situ.
augmentation of the right ventricular outflow tract using autologous pericardium. The patient was discharged from the hospital 11 days after the definitive procedure in stable condition.
Discussion Patients with CHD have a 3.5-fold increased risk of perioperative complications with noncardiac surgery, depending on the extent of surgery, severity of underlying abnormality, cyanosis, and heart failure.6 Lung resection surgery in a child with uncorrected TOF can pose a severe challenge. Both the conditions can cause profound life-threatening hypoxemia independently as well as cumulatively. The TOF with its chronic hypoxemic state has profound hematologic effects. Polycythemia improves oxygen transport at the expense of an increased viscosity that puts the patient at a risk of thrombosis and stroke.7 Platelet function abnormalities and multiple coagulation factor deficiencies, common in these patients, increase the risk of bleeding from raw-exposed surfaces of lung after decortication and lobectomy. Chronic hypoxia induces myocardial dysfunction with reduction in ventricular diastolic compliance and myocardial reserve.8 The poor myocardial reserve along with decreased pulmonary reserve due to patient’s lung condition places the patient at an increased risk of decompensation. Our concern was whether this child will tolerate OLV with its attendant further increase in R–L shunt. Our strategy was to preserve hypoxic pulmonary vasoconstriction and if required to clamp the right PA and keep CPB standby. The dilemma we faced was whether to perform surgery including lobectomy and ICR of TOF in a single setting or as a staged repair. We considered the following four options. The
432 first option we had was to attempt PVBD to improve SaO2; however, it was unsuccessful due to infundibular hypertrophy. The second option was to perform ICR and lobectomy in a single setting. The presence of empyema as a focus of infection in a patient with febrile with increased leukocyte count was a deterrent. The inflammatory response accompanying the CPB would have placed the patient at an increased risk of multiorgan dysfunction syndrome. The advantage of this single setting surgery would have been an improved PaO2 in the postoperative period along with increased ability to fight infection and better wound healing. The third option of lobectomy along with surgical palliation of TOF like a left-modified Blalock-Taussig shunt was considered risky, as ventilating the diseased lung (right) with major air leak and retraction of the healthy lung (left) with a clamp on the left PA would have lead to severe hypoxia. Rightmodified Blalock-Taussig shunt was not considered because of proximity to the infective focus. The option that we chose was for lobectomy in the first stage with CPB standby (for emergency ICR) followed by an elective ICR at a later date. This in our opinion was essential to control infection and improve the patient’s pulmonary status prior to taking up for surgical correction of TOF. We found that SaO2 on FiO2 0.5 increased from 71% preinduction to 79% after the lobectomy, and decortication was completed, probably due to removal of the diseased segment, better expansion of the adjacent fibrotic lung, and diversion of blood away from this zone of wasted perfusion. Karthekeyan et al reported carrying out lobectomy for congenital bronchiectasis and intracardiac TOF repair in the same setting as there was no active infection, and anatomy was favorable, that is, confluent good-sized PAs, normal coronaries, and no associated cardiac defects.5 Pikwer et al reported a patient, where lobectomy was performed for pulmonary sequestration in a child with uncorrected TOF followed by ICR eight months later.9 In contrast to the above-mentioned reports, our case was complicated by the presence of active infection (not responding to medical management) in addition to a major air leak from the BPF. To the best of our knowledge, the association of necrotizing streptococcal pneumonia in a immunocompetent child with TOF requiring surgical intervention has not been reported previously. Through this report, we tried to highlight the risks involved and the various treatment options
World Journal for Pediatric and Congenital Heart Surgery 4(4) available to a clinician in the care of a cyanotic child with a fulminant pulmonary pathology. In conclusion, the staged approach to surgical repair of TOF along with lobectomy for a severe pulmonary infection may be an acceptable option for a favorable outcome. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Hennein HA, Mendeloff EN, Cilley RE, Bove EL, Coran AG. Predictors of postoperative outcome after general surgical procedures in patients with congenital heart disease. J Pediatr Surg. 1994; 29(7): 866-870. 2. Katsetos CP, Kontoyannis MB, Koumousidis A, Petropoulou O, Delos C, Katsoulis M. Uncorrected tetralogy of Fallot and pregnancy: a case report. Clin Exp Obstet Gynecol. 2012;39(3): 382-383. 3. Raha A, Ganjoo P, Singh A, Tandon MS, Singh D. Surgery for brain abscess in children with cyanotic heart disease: an anesthetic challenge. J Pediatr Neurosci. 2012;7(1): 23-26. 4. Haack M, Machotta A, Boemke W, Ho¨hne C. Anesthesia in an infant with uncorrected tetralogy of Fallot for transanal pullthrough for Hirschprung’s disease. Paediatr Anaesth. 2006;16(1): 95-96. 5. Karthekeyan BR, Ravullapalli H, Vakamudi M, Thangavelu P. A rare case of tetralogy of Fallot with congenital tubercular bronchiectasis. Indian J Thorac Cardiovasc Surg. 2010;26(1): 34-37. 6. Baum VC, Barton DM, Gutgesell HP. Influence of congenital heart disease on mortality after noncardiac surgery in hospitalized children. Pediatrics. 2000;105(2): 332-335. 7. Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol. 1996; 28(3): 768-772. 8. Lovell AT. Anaesthetic implications of grown up congenital heart disease. Br J Anaesth. 2004;93(1): 129-139. 9. Pikwer A, Gyllstedt E, lillo-Gil R, Jo¨nsson P, Gudbjartsson T. Pulmonary sequestration—a review of 8 cases treated with lobectomy. Scand J Surg. 2006;95(3): 190-194.
