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Case Study
Neonatal repair of total anomalous pulmonary venous connection and lung agenesis
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314524754 aan.sagepub.com
Yuji Kaku, Mitsugi Nagashima, Goki Matsumura and Kenji Yamazaki
Abstract Here we report a neonatal case of total anomalous pulmonary venous connection with left lung agenesis. Diagnostic imaging demonstrated that the left pulmonary veins were totally absent and the right pulmonary veins connected with the common pulmonary chamber. Drainage from the common pulmonary venous chamber entered the persistent left suerior vena cava. In addition, it revealed complete absence of the left main bronchus and left lung vessels. The neonate successfully underwent surgical repair 18 days after birth.
Keywords Abnormalities, multiple, heart defects, congenital, infant, newborn, lung agenesis, pulmonary circulation
Introduction Congenital lung agenesis is a rare condition, defined as total absence of the bronchial tree, vascular structures, and lung parenchyma. The prevalence of lung agenesis is reported to be 34 per 1,000,000 live births.1 Total anomalous pulmonary venous connection (TAPVC) is also a rare disease and the combination of lung agenesis with TAPVC is an extremely rare condition.
Case report A 32-year-old pregnant woman (gravida 0, para 0) was referred to our hospital because of a fetal cardiac anomaly and left lung hypoplasia. TAPVC and left lung hypoplasia were suspected on fetal echocardiography. At 38 weeks and 1 day of gestation, uterine contractions started and a vaginal delivery was performed. The birth weight of the neonate was 2437 g, and his Apgar scores were 7 at 1 min and 7 at 5 min. No external anomaly was observed. Chest radiography showed opacity of the left lung field, hyperinflation of the right lung with congestion, and left displacement of the mediastinum (Figure 1). The baby was hemodynamically stable and had no respiratory failure, although cyanosis was present. Transthoracic echocardiography revealed
a perimembranous ventricular septal defect and patent ductus arteriosus, and the right pulmonary veins returned abnormally to the persistent left superior vena cava. There was no apparent indication of pulmonary venous obstruction. Fourteen days after the birth, echocardiography showed slight acceleration of the pulmonary venous flow, suggesting progression of the pulmonary venous obstruction. The patient underwent urgent surgical repair of TAPVC 18 days after birth. A median sternotomy was performed. After opening the pericardium, the patent ductus arteriosus was ligated with a hemoclip. Although the left pulmonary artery was explored by peripheral tracking of the main pulmonary artery, it was totally absent. The ventricular septal defect was closed with an expanded polytetrafluoroethylene patch through a right atriotomy. Drainage from the 2 right-sided pulmonary veins entered the common pulmonary venous chamber, and the left-sided pulmonary veins were totally absent.
Department of Cardiovascular Surgery, Tokyo Women’s Medical University Hospital, Tokyo, Japan Corresponding author: Yuji Kaku, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan. Email: [email protected]
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Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. Chest radiograph showing opacity of the left lung field, hyperinflation of the right lung with congestion, and left displacement of the mediastinum.
Drainage from the common pulmonary venous chamber entered the persistent left superior vena cava (TAPVC type Ib). Anastomosis of the common pulmonary venous chamber with the left atrium was performed under hypothermic very low-flow bypass through a right-sided left atriotomy. The atrial septal defect was closed using an autologous pericardial patch with a 2-mm hole that was expected to function as a safety valve during a postoperative pulmonary hypertensive crisis. The systemic pressure was 73/47 mm Hg, and the pulmonary artery pressure was 40/20 mm Hg (mean 22 mm Hg). The patient was transferred to the intensive care unit with his sternum open. On postoperative day 4, the sternum was closed. He was extubated 2 weeks after the operation, but the next day, reintubation was required because of tachypnea. On postoperative day 22, a right-sided pleural effusion was found on a chest radiograph, and a thoracic drainage tube was placed. Chylous effusion was drained; the amount of pleural effusion was a maximum of 500 mL per day. The pleural effusion was not controlled with conservative treatment, including tubal feeding of medium-chain triglyceride milk and administration of somatostatin analogue. A computed tomography scan was carried out to evaluate the amount of intrathoracic effusion. Three-dimensional computed tomography images of cardiovascular and lung structures showed complete absence of the left main bronchus and lung vessels (Figure 2), which finally led to the diagnosis of left lung agenesis rather than hypoplasia. Urgent thoracic duct ligation was required on postoperative day 28. After the surgery, the daily amount of effusion gradually decreased, and the thoracic drainage tube was removed. The baby was carefully weaned from mechanical ventilation and extubated on postoperative day 71. Postoperative transthoracic echocardiography
Figure 2. Three-dimensional computed tomography images (posterior view) of cardiovascular and lung structures, showing complete absence of the left main bronchus and lung vessels.
Figure 3. Postoperative chest radiograph.
showed that the TAPVC route was not stenotic, and the estimated right ventricular pressure from tricuspid regurgitation was 37 mm Hg; no pulmonary hypertension was observed. The patient was discharged on postoperative day 107 with home oxygen therapy. He has been followed up for 1 year at an outpatient clinic, and no complications have been reported thus far (Figure 3).
Discussion The pathogenesis of lung agenesis is unknown and vascular disruption and teratogenic factors have been reported as a potential causes. The condition is usually associated with other congenital malformations affecting the skeletal, cardiovascular, gastrointestinal, and
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genitourinary systems. Prognosis is related to the associated anomalies, thus the short-term prognosis of lung agenesis mainly depends on associated cardiac anomalies. There is very little information about long-term follow-up in patients with lung agenesis. It was reported that some adult patients had no associated complications.2 However, Chou and colleagues3 reported that 9 of 14 patients died within 2 years because of airway complications including bronchial compression by surrounding vessels, airway stenosis, or infection. To the best of our knowledge, only 5 cases of TAPVC with unilateral lung agenesis have been reported in the literature.4–7 Surgical repair was performed in 4, and only one patient was reported to have survived beyond 1 year after surgery. Our patient has been in a good clinical status, but long-term followup will be required, especially for respiratory complications. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
References 1. Mardini MK and Nyhan WL. Agenesis of the lung. Report of four patients with unusual anomalies. Chest 1985; 87: 522–527. 2. Ueda T, Nozoe M, Nakamoto Y, Irie Y and Mizushige K. Right pulmonary agenesis in an elderly woman complicated by transient ischemic attack. Intern Med 2011; 50: 463–466. 3. Chou AK, Huang SC, Chen SJ, et al. Unilateral lung agenesis—detrimental roles of surrounding vessels. Pediatr Pulmonol 2007; 42: 242–248. 4. Boxer RA, Hayes CJ, Hordof AJ and Mellins RB. Agenesis of the left lung and total anomalous pulmonary venous connection. Hemodynamic studies before and after complete surgical correction. Chest 1978; 74: 106–109. 5. Finci V, Beghetti M, Kalangos A and Brundler MA. Unilateral total and contralateral partial pulmonary agenesis associated with total anomalous pulmonary venous drainage. J Pediatr 1999; 134: 510–512. 6. Ootaki Y, Yamaguchi M, Yoshimura N and Oka S. Pulmonary agenesis with congenital heart disease. Pediatr Cardiol 2004; 25: 145–148. 7. Fischer M, Gorenflo M and Eichhorn J. First description of unilateral lung agenesis and anomalous of pulmonary venous connection in newborn with Goldenhar syndrome. Cardiol Young 2012; 22: 344–345.
Conflicts of interest statement None declared.
Downloaded from aan.sagepub.com at NORTHERN ARIZONA UNIVERSITY on June 13, 2015
(AAN)
[1–3] [PREPRINTER stage]
Case Study
Neonatal repair of total anomalous pulmonary venous connection and lung agenesis
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314524754 aan.sagepub.com
Yuji Kaku, Mitsugi Nagashima, Goki Matsumura and Kenji Yamazaki
Abstract Here we report a neonatal case of total anomalous pulmonary venous connection with left lung agenesis. Diagnostic imaging demonstrated that the left pulmonary veins were totally absent and the right pulmonary veins connected with the common pulmonary chamber. Drainage from the common pulmonary venous chamber entered the persistent left suerior vena cava. In addition, it revealed complete absence of the left main bronchus and left lung vessels. The neonate successfully underwent surgical repair 18 days after birth.
Keywords Abnormalities, multiple, heart defects, congenital, infant, newborn, lung agenesis, pulmonary circulation
Introduction Congenital lung agenesis is a rare condition, defined as total absence of the bronchial tree, vascular structures, and lung parenchyma. The prevalence of lung agenesis is reported to be 34 per 1,000,000 live births.1 Total anomalous pulmonary venous connection (TAPVC) is also a rare disease and the combination of lung agenesis with TAPVC is an extremely rare condition.
Case report A 32-year-old pregnant woman (gravida 0, para 0) was referred to our hospital because of a fetal cardiac anomaly and left lung hypoplasia. TAPVC and left lung hypoplasia were suspected on fetal echocardiography. At 38 weeks and 1 day of gestation, uterine contractions started and a vaginal delivery was performed. The birth weight of the neonate was 2437 g, and his Apgar scores were 7 at 1 min and 7 at 5 min. No external anomaly was observed. Chest radiography showed opacity of the left lung field, hyperinflation of the right lung with congestion, and left displacement of the mediastinum (Figure 1). The baby was hemodynamically stable and had no respiratory failure, although cyanosis was present. Transthoracic echocardiography revealed
a perimembranous ventricular septal defect and patent ductus arteriosus, and the right pulmonary veins returned abnormally to the persistent left superior vena cava. There was no apparent indication of pulmonary venous obstruction. Fourteen days after the birth, echocardiography showed slight acceleration of the pulmonary venous flow, suggesting progression of the pulmonary venous obstruction. The patient underwent urgent surgical repair of TAPVC 18 days after birth. A median sternotomy was performed. After opening the pericardium, the patent ductus arteriosus was ligated with a hemoclip. Although the left pulmonary artery was explored by peripheral tracking of the main pulmonary artery, it was totally absent. The ventricular septal defect was closed with an expanded polytetrafluoroethylene patch through a right atriotomy. Drainage from the 2 right-sided pulmonary veins entered the common pulmonary venous chamber, and the left-sided pulmonary veins were totally absent.
Department of Cardiovascular Surgery, Tokyo Women’s Medical University Hospital, Tokyo, Japan Corresponding author: Yuji Kaku, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan. Email: [email protected]
Downloaded from aan.sagepub.com at NORTHERN ARIZONA UNIVERSITY on June 13, 2015
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Asian Cardiovascular & Thoracic Annals 0(0)
Figure 1. Chest radiograph showing opacity of the left lung field, hyperinflation of the right lung with congestion, and left displacement of the mediastinum.
Drainage from the common pulmonary venous chamber entered the persistent left superior vena cava (TAPVC type Ib). Anastomosis of the common pulmonary venous chamber with the left atrium was performed under hypothermic very low-flow bypass through a right-sided left atriotomy. The atrial septal defect was closed using an autologous pericardial patch with a 2-mm hole that was expected to function as a safety valve during a postoperative pulmonary hypertensive crisis. The systemic pressure was 73/47 mm Hg, and the pulmonary artery pressure was 40/20 mm Hg (mean 22 mm Hg). The patient was transferred to the intensive care unit with his sternum open. On postoperative day 4, the sternum was closed. He was extubated 2 weeks after the operation, but the next day, reintubation was required because of tachypnea. On postoperative day 22, a right-sided pleural effusion was found on a chest radiograph, and a thoracic drainage tube was placed. Chylous effusion was drained; the amount of pleural effusion was a maximum of 500 mL per day. The pleural effusion was not controlled with conservative treatment, including tubal feeding of medium-chain triglyceride milk and administration of somatostatin analogue. A computed tomography scan was carried out to evaluate the amount of intrathoracic effusion. Three-dimensional computed tomography images of cardiovascular and lung structures showed complete absence of the left main bronchus and lung vessels (Figure 2), which finally led to the diagnosis of left lung agenesis rather than hypoplasia. Urgent thoracic duct ligation was required on postoperative day 28. After the surgery, the daily amount of effusion gradually decreased, and the thoracic drainage tube was removed. The baby was carefully weaned from mechanical ventilation and extubated on postoperative day 71. Postoperative transthoracic echocardiography
Figure 2. Three-dimensional computed tomography images (posterior view) of cardiovascular and lung structures, showing complete absence of the left main bronchus and lung vessels.
Figure 3. Postoperative chest radiograph.
showed that the TAPVC route was not stenotic, and the estimated right ventricular pressure from tricuspid regurgitation was 37 mm Hg; no pulmonary hypertension was observed. The patient was discharged on postoperative day 107 with home oxygen therapy. He has been followed up for 1 year at an outpatient clinic, and no complications have been reported thus far (Figure 3).
Discussion The pathogenesis of lung agenesis is unknown and vascular disruption and teratogenic factors have been reported as a potential causes. The condition is usually associated with other congenital malformations affecting the skeletal, cardiovascular, gastrointestinal, and
Downloaded from aan.sagepub.com at NORTHERN ARIZONA UNIVERSITY on June 13, 2015
XML Template (2014) [10.2.2014–3:26pm] //blrnas3/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/140036/APPFile/SG-AANJ140036.3d
(AAN)
[1–3] [PREPRINTER stage]
Kaku et al.
3
genitourinary systems. Prognosis is related to the associated anomalies, thus the short-term prognosis of lung agenesis mainly depends on associated cardiac anomalies. There is very little information about long-term follow-up in patients with lung agenesis. It was reported that some adult patients had no associated complications.2 However, Chou and colleagues3 reported that 9 of 14 patients died within 2 years because of airway complications including bronchial compression by surrounding vessels, airway stenosis, or infection. To the best of our knowledge, only 5 cases of TAPVC with unilateral lung agenesis have been reported in the literature.4–7 Surgical repair was performed in 4, and only one patient was reported to have survived beyond 1 year after surgery. Our patient has been in a good clinical status, but long-term followup will be required, especially for respiratory complications. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
References 1. Mardini MK and Nyhan WL. Agenesis of the lung. Report of four patients with unusual anomalies. Chest 1985; 87: 522–527. 2. Ueda T, Nozoe M, Nakamoto Y, Irie Y and Mizushige K. Right pulmonary agenesis in an elderly woman complicated by transient ischemic attack. Intern Med 2011; 50: 463–466. 3. Chou AK, Huang SC, Chen SJ, et al. Unilateral lung agenesis—detrimental roles of surrounding vessels. Pediatr Pulmonol 2007; 42: 242–248. 4. Boxer RA, Hayes CJ, Hordof AJ and Mellins RB. Agenesis of the left lung and total anomalous pulmonary venous connection. Hemodynamic studies before and after complete surgical correction. Chest 1978; 74: 106–109. 5. Finci V, Beghetti M, Kalangos A and Brundler MA. Unilateral total and contralateral partial pulmonary agenesis associated with total anomalous pulmonary venous drainage. J Pediatr 1999; 134: 510–512. 6. Ootaki Y, Yamaguchi M, Yoshimura N and Oka S. Pulmonary agenesis with congenital heart disease. Pediatr Cardiol 2004; 25: 145–148. 7. Fischer M, Gorenflo M and Eichhorn J. First description of unilateral lung agenesis and anomalous of pulmonary venous connection in newborn with Goldenhar syndrome. Cardiol Young 2012; 22: 344–345.
Conflicts of interest statement None declared.
Downloaded from aan.sagepub.com at NORTHERN ARIZONA UNIVERSITY on June 13, 2015
