Left atrial approach to close the atrial septal defect type II in case of right lung agenesis with extreme dextroversion
http://www.100md.com
《血管的通路杂志》
a Cardiac Surgery Department, Polish Mother's Research Institute, ód, Poland
b Imaging Department, Polish Mother's Research Institute, ód, Poland
Presented at the 55th International Congress of the European Society for Cardiovascular Surgery, St Petersburg, Russian Federation, May 11–14, 2006.
Abstract
This paper presents a case of a rare combination of right lung agenesis and atrial septal defect type II (ASD II). Successful surgical repair of ASD II was performed at the age of six years with total circulatory arrest (TCA) and deep hypothermia, because of difficult approach. Computed tomography and intraoperative findings are described. From patho-embryological consideration, defects in the primitive respiratory apparatus in embryo stages were presumably responsible for agenesis of the lung. The patient was found to have a very short stump of the right pulmonary artery, but this malformation seems to be secondary.
Key Words: Right lung agenesis; Atrial septal defect; Cardiovascular imaging
1. Introduction
Agenesis of the lung is defined (by Schneider) as the complete absence of the following structures: lung tissue, carina, main bronchus and pulmonary vasculature. In aplasia a carina and main bronchus without lung tissue are observed. Bronchial underdevelopment associated with reduced amount of lung tissue is noted in hypoplasia [1].
Pulmonary agenesis is found once in every 10,000–15,000 autopsies and distributed equally between the right and left side [2,3]. While agenesis of the lung or lungs (lethal) is a rare condition, lobar agenesis is even more uncommon [4]. Almost half of the patients with pulmonary agenesis have other congenital anomalies, the incidence being greater in those with right-sided agenesis [5]. The most common are esophageal atresia and tracheal stenosis [6].
2. Case report
A boy weighing 3280 g was born at a gestational age of 42 weeks with Apgar score of nine to a healthy woman (gravida 3, para 3). In infancy, the right pulmonary agenesis was diagnosed by roentgenographic findings, due to pneumonia. At bronchoscopy there was no sign of a carina or main right bronchus stem. The terminal trachea appeared to be slightly narrowed. Because of inappropriate parents policy the transthoracic echocardiographic evaluation was done at the age of six years. It revealed in subcostal view an atrial septal defect type II, sized 12 mm in diameter with hemodynamically significant left to right shunt. Other echocardiographic windows due to malposition of the heart were not diagnostic. The patient with mild circulatory symptoms was referred to surgery.
Chest X-rays taken before the operation showed the absence of the right lung, displacement of the cardiac shadow, and enhanced vascular markings in the left lung. Also, left-sided thoracic scoliosis with hemivertebra T7 was noted. Cardiac catheterization revealed the origin of the right pulmonary artery with a very short stump. A few collateral vessels to the left atrium from the pulmonary artery were observed. Blood pressure in pulmonary artery amounted to 45/10 mmHg.
The intracardiac surgery was performed through a midline sternotomy under a Fenthanyl anesthesia. A cardiopulmonary bypass with TCA and deep hypothermia (16.5 °C) was used. Exploration of the chest confirmed complete absence of the right lung and right pleural cavity, as well as large malposition of the heart. The right atrium was identified at the costovertebral region and the left atrium was positioned anteriorly. The main pulmonary artery and the single left pulmonary artery were also viewed. The arterial cannula was inserted into the ascending aorta and one venous cannula (because of difficult approach) into the right atrium, positioned deep posteriorly. The left atrium was incised in the appendage and a large atrial septal defect, located behind the ostium of mitral valve was directly sutured. The pulmonary artery pressure, after completing the correction, diminished to 24/16 mmHg. The postoperative course was uneventful.
Eight years after surgery the boy was re-examined. The echocardiographic data were within normal range. His physical growth was satisfactory. He had no complaints. Malposition of the heart (extreme dextroversion with counter clockwise rotation) was noted on computed tomography (CT) (Figs. 1 and 2).
On spirometry there was a moderate mixed deterioration of the ventilation. The forced expiratory volume in 1 s (FEV1) was diminished to 1.12 l (50% predicted) as well as forced vital capacity expiratory (FVC EX) to 1.36 l (49%). Their ratio as a Tiffenou index was within normal limits (98%). Expiratory flows were decreased and demonstrated a restrictive pattern.
3. Discussion
A combination of congenital anomalies in our patient includes among others: absence of the right lung and right pleural cavity with displacement of the heart (extreme dextroversion) and mediastinum, nonbifurcated trachea and a remnant of the right pulmonary artery. Similar conditions were reported in literature [6–8]. Three patients required aortopexy due to posteriorly divergent aortic arch causing tracheal compression [9].
patho-embryological considerations it is known that the trachea develops in the embryo about the fourth week, in its 5-mm stage. Differentiation of the sixth arch into the pulmonary artery does not take place until the 7-mm embryo stage [6]. Thus, in our case, the defects in the primitive respiratory apparatus, but not malformation of the pulmonary artery, were presumably responsible for complete absence of the lung.
References
Schneider P. Die Missbildungen der Atmungsorgane. In: Schwalbe E. editor Die Morphologie der Tiere1912;Jena: Fischer 817–822. In:.
Fergusan CF, Neuhauser EB. Congenital absence of the lung agenesis and other anomalies of the tracheobronchial tree. Am J Roentgenol 1944; 52:459–471.
Oyomada A, Gasul BM, Holinger PH. Agenesis of the lung. Report of a case with the review of all previously reported cases. Am J Dis Child 1953; 85:182–201.
Rosenberg DM. Pulmonary agenesis. Dis Chest 1962; 42:68–73.
Wexels P. Agenesis of the lung. Thorax 1951; 6:171–192.
Nelson CS, McMillan KR, Bharucha PK. Tracheal stenosis, pulmonary agenesis and patent ductus arteriosus. Thorax 1967; 22:7–12.
Yaghmai I. Agenesis of the lung. Am J Roentgenol 1970;564–568.
Argent AC, Cremin BJ. Computed tomography in agenesis of the lung in infants. Br J Radiol 1992; 65:221–224.
Dohlemann C, Mantel K, Schneider K, Gunter M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: airway obstruction and its release by aortopexy. J Pediatr Surg 1990; 25:290–293.(Witold D. Pietrzykowskia,, Maciej Molla,)
b Imaging Department, Polish Mother's Research Institute, ód, Poland
Presented at the 55th International Congress of the European Society for Cardiovascular Surgery, St Petersburg, Russian Federation, May 11–14, 2006.
Abstract
This paper presents a case of a rare combination of right lung agenesis and atrial septal defect type II (ASD II). Successful surgical repair of ASD II was performed at the age of six years with total circulatory arrest (TCA) and deep hypothermia, because of difficult approach. Computed tomography and intraoperative findings are described. From patho-embryological consideration, defects in the primitive respiratory apparatus in embryo stages were presumably responsible for agenesis of the lung. The patient was found to have a very short stump of the right pulmonary artery, but this malformation seems to be secondary.
Key Words: Right lung agenesis; Atrial septal defect; Cardiovascular imaging
1. Introduction
Agenesis of the lung is defined (by Schneider) as the complete absence of the following structures: lung tissue, carina, main bronchus and pulmonary vasculature. In aplasia a carina and main bronchus without lung tissue are observed. Bronchial underdevelopment associated with reduced amount of lung tissue is noted in hypoplasia [1].
Pulmonary agenesis is found once in every 10,000–15,000 autopsies and distributed equally between the right and left side [2,3]. While agenesis of the lung or lungs (lethal) is a rare condition, lobar agenesis is even more uncommon [4]. Almost half of the patients with pulmonary agenesis have other congenital anomalies, the incidence being greater in those with right-sided agenesis [5]. The most common are esophageal atresia and tracheal stenosis [6].
2. Case report
A boy weighing 3280 g was born at a gestational age of 42 weeks with Apgar score of nine to a healthy woman (gravida 3, para 3). In infancy, the right pulmonary agenesis was diagnosed by roentgenographic findings, due to pneumonia. At bronchoscopy there was no sign of a carina or main right bronchus stem. The terminal trachea appeared to be slightly narrowed. Because of inappropriate parents policy the transthoracic echocardiographic evaluation was done at the age of six years. It revealed in subcostal view an atrial septal defect type II, sized 12 mm in diameter with hemodynamically significant left to right shunt. Other echocardiographic windows due to malposition of the heart were not diagnostic. The patient with mild circulatory symptoms was referred to surgery.
Chest X-rays taken before the operation showed the absence of the right lung, displacement of the cardiac shadow, and enhanced vascular markings in the left lung. Also, left-sided thoracic scoliosis with hemivertebra T7 was noted. Cardiac catheterization revealed the origin of the right pulmonary artery with a very short stump. A few collateral vessels to the left atrium from the pulmonary artery were observed. Blood pressure in pulmonary artery amounted to 45/10 mmHg.
The intracardiac surgery was performed through a midline sternotomy under a Fenthanyl anesthesia. A cardiopulmonary bypass with TCA and deep hypothermia (16.5 °C) was used. Exploration of the chest confirmed complete absence of the right lung and right pleural cavity, as well as large malposition of the heart. The right atrium was identified at the costovertebral region and the left atrium was positioned anteriorly. The main pulmonary artery and the single left pulmonary artery were also viewed. The arterial cannula was inserted into the ascending aorta and one venous cannula (because of difficult approach) into the right atrium, positioned deep posteriorly. The left atrium was incised in the appendage and a large atrial septal defect, located behind the ostium of mitral valve was directly sutured. The pulmonary artery pressure, after completing the correction, diminished to 24/16 mmHg. The postoperative course was uneventful.
Eight years after surgery the boy was re-examined. The echocardiographic data were within normal range. His physical growth was satisfactory. He had no complaints. Malposition of the heart (extreme dextroversion with counter clockwise rotation) was noted on computed tomography (CT) (Figs. 1 and 2).
On spirometry there was a moderate mixed deterioration of the ventilation. The forced expiratory volume in 1 s (FEV1) was diminished to 1.12 l (50% predicted) as well as forced vital capacity expiratory (FVC EX) to 1.36 l (49%). Their ratio as a Tiffenou index was within normal limits (98%). Expiratory flows were decreased and demonstrated a restrictive pattern.
3. Discussion
A combination of congenital anomalies in our patient includes among others: absence of the right lung and right pleural cavity with displacement of the heart (extreme dextroversion) and mediastinum, nonbifurcated trachea and a remnant of the right pulmonary artery. Similar conditions were reported in literature [6–8]. Three patients required aortopexy due to posteriorly divergent aortic arch causing tracheal compression [9].
patho-embryological considerations it is known that the trachea develops in the embryo about the fourth week, in its 5-mm stage. Differentiation of the sixth arch into the pulmonary artery does not take place until the 7-mm embryo stage [6]. Thus, in our case, the defects in the primitive respiratory apparatus, but not malformation of the pulmonary artery, were presumably responsible for complete absence of the lung.
References
Schneider P. Die Missbildungen der Atmungsorgane. In: Schwalbe E. editor Die Morphologie der Tiere1912;Jena: Fischer 817–822. In:.
Fergusan CF, Neuhauser EB. Congenital absence of the lung agenesis and other anomalies of the tracheobronchial tree. Am J Roentgenol 1944; 52:459–471.
Oyomada A, Gasul BM, Holinger PH. Agenesis of the lung. Report of a case with the review of all previously reported cases. Am J Dis Child 1953; 85:182–201.
Rosenberg DM. Pulmonary agenesis. Dis Chest 1962; 42:68–73.
Wexels P. Agenesis of the lung. Thorax 1951; 6:171–192.
Nelson CS, McMillan KR, Bharucha PK. Tracheal stenosis, pulmonary agenesis and patent ductus arteriosus. Thorax 1967; 22:7–12.
Yaghmai I. Agenesis of the lung. Am J Roentgenol 1970;564–568.
Argent AC, Cremin BJ. Computed tomography in agenesis of the lung in infants. Br J Radiol 1992; 65:221–224.
Dohlemann C, Mantel K, Schneider K, Gunter M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: airway obstruction and its release by aortopexy. J Pediatr Surg 1990; 25:290–293.(Witold D. Pietrzykowskia,, Maciej Molla,)