Successful extracardiac total cavo pulmonary connection (TCPC) after external tracheobronchial stenting for tracheobronchomalacia
http://www.100md.com
《血管的通路杂志》
a Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan
b Division of Cardiovascular Surgery, Matsudo Municipal Hospital, Chiba, Japan
c Division of Neonatology, Matsudo Municipal Hospital, Chiba, Japan
Abstract
Objective: A girl who was diagnosed with cyanotic congenital heart disease in a newborn, showed anoxic spell attacks which we thought were correlated with the congenital heart disease. Although she underwent the palliative operation at 8 months old and her SpO2 was increased, she experienced severe and life-threatening respiratory dysfunction many times after that. After careful examinations, the respiratory complaint was proved to be not only due to cyanotic congenital heart disease but also tracheobronchomalacia. Method: She had undergone the external stenting to the trachea and right bronchus at 1 year old. After that, she was examined by cardiac catheterization and the Fontan-type operation was successful using a tissue-engineered graft at 2 years old. Results: Her post-operative course was uneventful and she was discharged. Conclusion: It is very important to remember the possible existence of tracheobronchomalacia and prevent a life-threatening attack when congenital cardiac patients experience a prolonged respiratory failure or abnormal respiration.
Key Words: Tracheobronchomalacia; External stenting; TCPC
1. Introduction
Tracheobronchomalacia is a symptomatic disease that is associated with recurrent pneumonia or severe respiratory failure. In addition, it is often associated with simple cardiac defects with pulmonary hypertension or tetralogy of Fallot with an absent pulmonary valve [1]. However, it is difficult to distinguish a combination of bronchomalacia from a cardiac defect alone. Under these circumstances, several treatments such as aortopexy [2,3,8] have been attempted, although satisfactory results have not been obtained. Recently, external stenting has been reported as being useful for tracheomalacia and bronchomalacia [6]. We report a successful experience of Fontan completion after external stenting for a rare case with double-outlet right ventricle (DORV), pulmonary stenosis (PS) and primary tracheobronchomalacia.
2. Case report
A female (1376 g) was born at 34 weeks and 3 days by cesarian, and was noted as cyanotic immediately after birth. An echocardiogram revealed DORV, I,D,D, PS, right aortic arch, and a small left ventricle (LV). Because she had repeated anoxic spells which were not intented to the ventilation support, we performed some cardiac examinations. A cardiac catheterization at 8 months old showed an LVEDV of 35% of normal, LVEF of 70%, RVEDV of 146% of normal, RVEF of 55%, a pulmonary artery (PA) index of 491 (right PA: 8.0 mm, left PA: 11.4 mm), a right PA pressure (PAP) of 10 mmHg, a left PAP of 18 mmHg, Qp/Qs of 0.6, and SaO2 of 74%, pressure gradient (RV-main PA) of 60 mmHg, PA valve diameter of 6.7x6.0 mm, aortic valve diameter of 13.0x10.7 mm. She underwent a right modified Blalock-Taussig shunt operation to increase pulmonary flow and prevent anoxic spells using a 5-mm ePTFE tube graft through a right thoracotomy at 8 months old. Her right PA was measured as 7 mm in diameter during the operation. She increased the SpO2 from 74% to 90%, and the pulmonary artery pressure (PAP) 10 mmHg to 21 mmHg. However, she experienced severe respiratory dysfunction 1 month later and was resuscitated at the outpatient division and needed the mechanical ventilation at first. Although she could always be weaned to CPAP support, she could not pass the extubation. She needed continuous 10 cm airway pressure. After repeated inadequate ventilation, we suspected not only cardiac but also airway system disturbance. Careful examinations and bronchoscopy elucidated severe tracheobronchomalacia while her trachea and right bronchus had an abnormal softness and had collapsed by the absence of tracheobronchial cartilages.
External stenting to the trachea and right main bronchus was performed using a ring-reinforced ePTFE graft (16 mm in diameter), through a median sternotomy (Fig. 1). At that time, we noted that she had an abnormal softness of the trachea and right bronchus. Also, an enlarged main pulmonary artery required an additional procedure of pulmonary artery banding (15 mm in PA circumstance). This procedure produced a decrease in PAP from 21 to 16 mmHg. Intra-operative bronchoscopic monitoring confirmed a well-opened trachea and bronchus. A cardiac catheterization at 9 months after the procedure showed an LVEDV of 62% of normal, LVEF of 36%, RVEDV of 247% of normal, RVEF of 46%, a PA index of 492 (right PA: 11 mm, left PA: 12 mm), a PAP of 16 mmHg, Qp/Qs of 2.6, and SaO2 of 90%, which indicated the Fontan-type operation. The extracardiac TCPC was successfully performed at 2 years old using a tissue-engineered graft (18 mm in diameter) seeded with collected autologous bone marrow cells [7]. The extubation was performed the next day. She has been maintained in a stable condition for 3 years at the outpatient division (Fig. 2).
3. Discussion
Although tracheobronchomalacia is considered a relatively rare disease, it is characterized by an abnormal softness or absence of tracheobronchial cartilages. It is very important to distinguish a combination of tracheobronchomalacia from a cardiac defect alone. Many of these malformations are secondary to compression by an enlarged pulmonary artery in patients such as those with tetralogy of Fallot [1]. Historically, the maintenance of a high PEEP therapy which prevents airway collapse, was attempted in these patients [5]. However, prolonged mechanical ventilation under deep sedation presents problems in terms of psychomotor development. In addition, tracheostomy in children receiving long-term ventilation should be avoided due to the occurrence of tracheal fibrosis and erosion. Although several articles have described the efficacy of aortopexy [2,3,8], this procedure may not be effective in patients with severe transformation of the trachea and bronchus, or those with primary tracheobronchomalacia. Similarly, internal airway stenting is reported, however, this technique has potential for infection, re-stenosis due to intraluminal granulation [1,4], difficulty in removal and a limitation to sizes that are not ideal for very small airways. On the other hand, external stenting allows reduced ventilator dependency, effectiveness of primary tracheobronchomalacia and less risk of re-stenosis [6]. Therefore, we think this external stabilization technique could be the most effective for tracheobronchomalacia.
In our patient, the appearance of cyanosis in early infancy may have been associated with an inadequate ventilation due to tracheobronchomalacia. Severe PS was suspected at first and a systemic-pulmonary shunt was performed since the existence of tracheobronchomalacia was not known, and we could not see her trachea and peripheral bronchus at the shunt operation. However, the episode of repeated sudden respiratory dysfunction after the shunt operation, and the necessity for more inflation pressure to her lungs at the resuscitation, and a high PAP of 21 mmHg at the stenting operation, when pulmonary artery banding was needed, indicated the existence of tracheobronchomalacia. Consequently, the previous shunt operation may have been unnecessary for this patient. Although it was very difficult to determine if she needed the shunt operation regarding our case, it will be necessary to do more examinations of respiratory systems when we find abnormal respiratory symptoms. At the present I am concerned about this case, because she had anoxic spells with wheezing, it may have been only a sign for us to probe with more examinations of the respiratory systems before the shunt operation. By the way, although she had tracheobronchomalacia, a life-threatening respiratory attack was not found in a newborn. We think that some types of tracheobronchomalacia will present many symptoms after a few months old because of respiratory muscle growth. Our findings show that it is important to keep in mind this complication when repeated respiratory dysfunction at not only newborn but also anytime including cyanosis is recognized, particularly in Fontan candidates.
References
Dodge-Khatami A, Baker CL, Holinger LD, Baden H, Mavroudis C. Complete repair of tetralogy of Fallot with absent pulmonary valve including the role of airway stenting. J Card Surg 1999; 14:82–91.
Malone PS, Kiely EM. Role of aortopexy in the management of primary tracheomalacia and tracheobronchomalacia. Arch Dis Child 1990;65438–440.
McElhinney DB, Reddy VM, Pian MS, Moore P, Hanley FL. Compression of the central airways by a dilated aorta in infants and children with congenital heart disease. Ann Thorac Surg 1999; 67:1130–1136.
Jacobs JP, Quintessenza JA, Botero LM, van Gelder HM, Giroud JM, Elliott MJ, Herberhold C. The role of airway stents in the management of pediatric tracheal, carinal, and bronchial disease. Eur J Cardiothorac Surg 2000; 18:505–512.
Panitch HB, Allen JL, Alpert BE, Schidlow DV. Effects of CPAP on lung mechanics in infants with acquired tracheobronchomalacia. Am J Respir Crit Care Med 1994; 150:1341–1346.
Hagl S, Jakob H, Sebening C, van Bodegom P, Schmidt K, Zilow E, Fleischer F, Ulmer H. External stabilization of long-segment tracheobronchomalacia guided by intraoperative bronchoscopy. Ann Thorac Surg 1997; 64:1412–1420.
Isomatsu Y, Shin'oka T, Matsumura G, Hibino N, Konuma T, Nagatsu M, Kurosawa H. Extracardiac total cavopulmonary connection using a tissue-engineered graft. J Thorac Cardiovasc Surg 2003; 126:1958–1962.
Abdel-Rahman U, Ahrens P, Fieguth HG, Kitz R, Heller K, Moritz A. Surgical treatment of tracheomalacia by bronchoscopic monitored aortopexy in infants and children. Ann Thorac Surg 2002; 74:315–319.
Sakamoto T, Nagase Y, Hasegawa H, Shin'oka T, Tomimatsu H, Kurosawa H. One-stage intracardiac repair in combination with external stenting of the trachea and right bronchus for tetralogy of Fallot with an absent pulmonary valve and tracheobronchomalacia. J Thorac Cardiovasc Surg 2005; 130:1717–1718.
Valerie EP, Durrant AC, Forte V, Wales P, Chait P, Kim PCW. A decade of using intraluminal tracheal/bronchial stents in the management of tracheomalacia and/or bronchomalacia: is it better than aortopexy J Pediatr Surg 2005; 40:904–907.
Morabito A, MacKinnon E, Alizai N, Asero L, Bianchi A. The anterior mediastinal approach for management of tracheomalacia. J Pediatr Surg 2000; 35:1456–1458.
Kamata S, Usui N, Sawai T, Nose K, Kitayama Y, Okuyama H, Okada A. Pexis of the great vessels for patients with tracheobronchomalacia in infancy. J Pediatr Surg 2000; 35:454–457.
McLaren CA, Elliott MJ, Roebuck DJ. Tracheobronchial intervention in children. Eur J Radiology 2005; 53:22–34.
Pillai JB, Smith J, Hasan A, Spencer D. Review of pediatric airway malacia and its management, with emphasis on stenting. Eur J Cardio-Thorac Surg 2005; 27:35–44.
Wyatt ME, Hartley BE, Roebuck D, McLaren C, Elliott M, Pigot N, Wallis C, Mok Q. Metallic tracheobronchial stenting in the pediatric airway. Otolaryng Head Neck Surg 2004; 131:P220.(Shizuko Iwasa, Toshiharu )
b Division of Cardiovascular Surgery, Matsudo Municipal Hospital, Chiba, Japan
c Division of Neonatology, Matsudo Municipal Hospital, Chiba, Japan
Abstract
Objective: A girl who was diagnosed with cyanotic congenital heart disease in a newborn, showed anoxic spell attacks which we thought were correlated with the congenital heart disease. Although she underwent the palliative operation at 8 months old and her SpO2 was increased, she experienced severe and life-threatening respiratory dysfunction many times after that. After careful examinations, the respiratory complaint was proved to be not only due to cyanotic congenital heart disease but also tracheobronchomalacia. Method: She had undergone the external stenting to the trachea and right bronchus at 1 year old. After that, she was examined by cardiac catheterization and the Fontan-type operation was successful using a tissue-engineered graft at 2 years old. Results: Her post-operative course was uneventful and she was discharged. Conclusion: It is very important to remember the possible existence of tracheobronchomalacia and prevent a life-threatening attack when congenital cardiac patients experience a prolonged respiratory failure or abnormal respiration.
Key Words: Tracheobronchomalacia; External stenting; TCPC
1. Introduction
Tracheobronchomalacia is a symptomatic disease that is associated with recurrent pneumonia or severe respiratory failure. In addition, it is often associated with simple cardiac defects with pulmonary hypertension or tetralogy of Fallot with an absent pulmonary valve [1]. However, it is difficult to distinguish a combination of bronchomalacia from a cardiac defect alone. Under these circumstances, several treatments such as aortopexy [2,3,8] have been attempted, although satisfactory results have not been obtained. Recently, external stenting has been reported as being useful for tracheomalacia and bronchomalacia [6]. We report a successful experience of Fontan completion after external stenting for a rare case with double-outlet right ventricle (DORV), pulmonary stenosis (PS) and primary tracheobronchomalacia.
2. Case report
A female (1376 g) was born at 34 weeks and 3 days by cesarian, and was noted as cyanotic immediately after birth. An echocardiogram revealed DORV, I,D,D, PS, right aortic arch, and a small left ventricle (LV). Because she had repeated anoxic spells which were not intented to the ventilation support, we performed some cardiac examinations. A cardiac catheterization at 8 months old showed an LVEDV of 35% of normal, LVEF of 70%, RVEDV of 146% of normal, RVEF of 55%, a pulmonary artery (PA) index of 491 (right PA: 8.0 mm, left PA: 11.4 mm), a right PA pressure (PAP) of 10 mmHg, a left PAP of 18 mmHg, Qp/Qs of 0.6, and SaO2 of 74%, pressure gradient (RV-main PA) of 60 mmHg, PA valve diameter of 6.7x6.0 mm, aortic valve diameter of 13.0x10.7 mm. She underwent a right modified Blalock-Taussig shunt operation to increase pulmonary flow and prevent anoxic spells using a 5-mm ePTFE tube graft through a right thoracotomy at 8 months old. Her right PA was measured as 7 mm in diameter during the operation. She increased the SpO2 from 74% to 90%, and the pulmonary artery pressure (PAP) 10 mmHg to 21 mmHg. However, she experienced severe respiratory dysfunction 1 month later and was resuscitated at the outpatient division and needed the mechanical ventilation at first. Although she could always be weaned to CPAP support, she could not pass the extubation. She needed continuous 10 cm airway pressure. After repeated inadequate ventilation, we suspected not only cardiac but also airway system disturbance. Careful examinations and bronchoscopy elucidated severe tracheobronchomalacia while her trachea and right bronchus had an abnormal softness and had collapsed by the absence of tracheobronchial cartilages.
External stenting to the trachea and right main bronchus was performed using a ring-reinforced ePTFE graft (16 mm in diameter), through a median sternotomy (Fig. 1). At that time, we noted that she had an abnormal softness of the trachea and right bronchus. Also, an enlarged main pulmonary artery required an additional procedure of pulmonary artery banding (15 mm in PA circumstance). This procedure produced a decrease in PAP from 21 to 16 mmHg. Intra-operative bronchoscopic monitoring confirmed a well-opened trachea and bronchus. A cardiac catheterization at 9 months after the procedure showed an LVEDV of 62% of normal, LVEF of 36%, RVEDV of 247% of normal, RVEF of 46%, a PA index of 492 (right PA: 11 mm, left PA: 12 mm), a PAP of 16 mmHg, Qp/Qs of 2.6, and SaO2 of 90%, which indicated the Fontan-type operation. The extracardiac TCPC was successfully performed at 2 years old using a tissue-engineered graft (18 mm in diameter) seeded with collected autologous bone marrow cells [7]. The extubation was performed the next day. She has been maintained in a stable condition for 3 years at the outpatient division (Fig. 2).
3. Discussion
Although tracheobronchomalacia is considered a relatively rare disease, it is characterized by an abnormal softness or absence of tracheobronchial cartilages. It is very important to distinguish a combination of tracheobronchomalacia from a cardiac defect alone. Many of these malformations are secondary to compression by an enlarged pulmonary artery in patients such as those with tetralogy of Fallot [1]. Historically, the maintenance of a high PEEP therapy which prevents airway collapse, was attempted in these patients [5]. However, prolonged mechanical ventilation under deep sedation presents problems in terms of psychomotor development. In addition, tracheostomy in children receiving long-term ventilation should be avoided due to the occurrence of tracheal fibrosis and erosion. Although several articles have described the efficacy of aortopexy [2,3,8], this procedure may not be effective in patients with severe transformation of the trachea and bronchus, or those with primary tracheobronchomalacia. Similarly, internal airway stenting is reported, however, this technique has potential for infection, re-stenosis due to intraluminal granulation [1,4], difficulty in removal and a limitation to sizes that are not ideal for very small airways. On the other hand, external stenting allows reduced ventilator dependency, effectiveness of primary tracheobronchomalacia and less risk of re-stenosis [6]. Therefore, we think this external stabilization technique could be the most effective for tracheobronchomalacia.
In our patient, the appearance of cyanosis in early infancy may have been associated with an inadequate ventilation due to tracheobronchomalacia. Severe PS was suspected at first and a systemic-pulmonary shunt was performed since the existence of tracheobronchomalacia was not known, and we could not see her trachea and peripheral bronchus at the shunt operation. However, the episode of repeated sudden respiratory dysfunction after the shunt operation, and the necessity for more inflation pressure to her lungs at the resuscitation, and a high PAP of 21 mmHg at the stenting operation, when pulmonary artery banding was needed, indicated the existence of tracheobronchomalacia. Consequently, the previous shunt operation may have been unnecessary for this patient. Although it was very difficult to determine if she needed the shunt operation regarding our case, it will be necessary to do more examinations of respiratory systems when we find abnormal respiratory symptoms. At the present I am concerned about this case, because she had anoxic spells with wheezing, it may have been only a sign for us to probe with more examinations of the respiratory systems before the shunt operation. By the way, although she had tracheobronchomalacia, a life-threatening respiratory attack was not found in a newborn. We think that some types of tracheobronchomalacia will present many symptoms after a few months old because of respiratory muscle growth. Our findings show that it is important to keep in mind this complication when repeated respiratory dysfunction at not only newborn but also anytime including cyanosis is recognized, particularly in Fontan candidates.
References
Dodge-Khatami A, Baker CL, Holinger LD, Baden H, Mavroudis C. Complete repair of tetralogy of Fallot with absent pulmonary valve including the role of airway stenting. J Card Surg 1999; 14:82–91.
Malone PS, Kiely EM. Role of aortopexy in the management of primary tracheomalacia and tracheobronchomalacia. Arch Dis Child 1990;65438–440.
McElhinney DB, Reddy VM, Pian MS, Moore P, Hanley FL. Compression of the central airways by a dilated aorta in infants and children with congenital heart disease. Ann Thorac Surg 1999; 67:1130–1136.
Jacobs JP, Quintessenza JA, Botero LM, van Gelder HM, Giroud JM, Elliott MJ, Herberhold C. The role of airway stents in the management of pediatric tracheal, carinal, and bronchial disease. Eur J Cardiothorac Surg 2000; 18:505–512.
Panitch HB, Allen JL, Alpert BE, Schidlow DV. Effects of CPAP on lung mechanics in infants with acquired tracheobronchomalacia. Am J Respir Crit Care Med 1994; 150:1341–1346.
Hagl S, Jakob H, Sebening C, van Bodegom P, Schmidt K, Zilow E, Fleischer F, Ulmer H. External stabilization of long-segment tracheobronchomalacia guided by intraoperative bronchoscopy. Ann Thorac Surg 1997; 64:1412–1420.
Isomatsu Y, Shin'oka T, Matsumura G, Hibino N, Konuma T, Nagatsu M, Kurosawa H. Extracardiac total cavopulmonary connection using a tissue-engineered graft. J Thorac Cardiovasc Surg 2003; 126:1958–1962.
Abdel-Rahman U, Ahrens P, Fieguth HG, Kitz R, Heller K, Moritz A. Surgical treatment of tracheomalacia by bronchoscopic monitored aortopexy in infants and children. Ann Thorac Surg 2002; 74:315–319.
Sakamoto T, Nagase Y, Hasegawa H, Shin'oka T, Tomimatsu H, Kurosawa H. One-stage intracardiac repair in combination with external stenting of the trachea and right bronchus for tetralogy of Fallot with an absent pulmonary valve and tracheobronchomalacia. J Thorac Cardiovasc Surg 2005; 130:1717–1718.
Valerie EP, Durrant AC, Forte V, Wales P, Chait P, Kim PCW. A decade of using intraluminal tracheal/bronchial stents in the management of tracheomalacia and/or bronchomalacia: is it better than aortopexy J Pediatr Surg 2005; 40:904–907.
Morabito A, MacKinnon E, Alizai N, Asero L, Bianchi A. The anterior mediastinal approach for management of tracheomalacia. J Pediatr Surg 2000; 35:1456–1458.
Kamata S, Usui N, Sawai T, Nose K, Kitayama Y, Okuyama H, Okada A. Pexis of the great vessels for patients with tracheobronchomalacia in infancy. J Pediatr Surg 2000; 35:454–457.
McLaren CA, Elliott MJ, Roebuck DJ. Tracheobronchial intervention in children. Eur J Radiology 2005; 53:22–34.
Pillai JB, Smith J, Hasan A, Spencer D. Review of pediatric airway malacia and its management, with emphasis on stenting. Eur J Cardio-Thorac Surg 2005; 27:35–44.
Wyatt ME, Hartley BE, Roebuck D, McLaren C, Elliott M, Pigot N, Wallis C, Mok Q. Metallic tracheobronchial stenting in the pediatric airway. Otolaryng Head Neck Surg 2004; 131:P220.(Shizuko Iwasa, Toshiharu )