Ann Thorac Surg 2001;72:1742-1744
© 2001 The Society of Thoracic Surgeons
Case report
Pulmonary agenesis with interrupted aortic arch
Yoshio Ootaki, MD*a,
Masahiro Yamaguchi, MDa,
Yoshihiro Oshima, MDa,
Naoki Yoshimura, MDa,
Shigeteru Oka, MDa
a Department of Cardiothoracic Surgery, Kobe Children’s Hospital, Kobe, Hyogo, Japan
Accepted for publication January 19, 2001.
* Address reprint requests to Dr Ootaki, 1-1-1 Takakuradai, Suma-ku, Kobe, Hyogo, 654-0081, Japan
e-mail: y.ootaki{at}nifty.ne.jp
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Abstract
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This report describes a unique case of an infant with right pulmonary agenesis associated with interrupted aortic arch, ventricular septal defect, and aortic valvular stenosis. Blalock-Park arterial anastomosis and pulmonary arterial banding were performed without incidence. These techniques were useful for this complicated heart defect with pulmonary agenesis. The postoperative course of the patient was uneventful.
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Introduction
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Unilateral pulmonary agenesis is a rare congenital anomaly whose cause is not completely known. Approximately one in 15,000 children are born with a congenital absence of one lung. Associated malformations, mainly affecting the cardiovascular, gastrointestinal, and musculoskeletal systems, influence the prognosis of these patients, as well as the location of the missing lung. This report describes a unique case of an infant with right pulmonary agenesis associated with interrupted aortic arch.
A 39-week-gestation male infant was born to a gravida 2, para 1 mother without prenatal risk factors. He was presented to us at 2 months of age with a history of wheezy breathing since birth. He weighed 2.92 kg and had respiratory distress with a respiratory rate of 50 breaths per minute. He was mildly cyanotic and had severe recession. Oxygen saturation was 94% in room air. Blood pressure of upper extremity was 100 mm Hg and lower extremity was 60 mm Hg. A heart murmur was unclear. Chest radiographs showed an overexpanded left lung with mediastinal shift to the right. There was no aeration of the lung in the right side. Two-dimensional color Doppler echocardiography confirmed the displacement of the heart to the right hemithorax with severe counterclockwise rotation and revealed a large perimembranous ventricular septal defect with pulmonary hypertension. The right pulmonary artery and veins were not visible. Mild aortic valvular stenosis, suspecting bicuspid aortic valve, was present. Subaortic stenosis was not found. Ascending aorta was mildly hypoplastic and was 5.2 mm in diameter. Aortic arch was not detected. Contraction of the left ventricle was poor. Cardiac catheterization showed a single pulmonary artery, an interrupted aortic arch just distal to the left subclavian artery, and pulmonary hypertension with a systolic pulmonary-to-systemic pressure ratio of 1.1 (Fig 1A, 1B). Ejection fraction of left ventricle was 37%. Bronchoscopy at the same time of the cardiac catheterization showed the absence of the right main bronchus and no vascular compression of the trachea or left main bronchus, but the left main bronchus was unusually narrow.
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Fig 1. (A) Pulmonary angiogram shows total absence of the right pulmonary artery. (B) Digital subtraction aortogram shows an interrupted aortic arch just distal to the left subclavian artery.
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An emergency operation was performed through the left thoracotomy. The left subclavian artery and the upper part of the thoracic descending aorta were exposed using utmost effort not to impair the ventilation of the left lung. The aorta was mobilized and all intercostal arteries were preserved. The ligated subclavian artery was divided as distally as possible and a lateral arteriotomy was performed. The descending aortotomy was carried distally for sufficient length to be well outside the area of ductal tissue to help prevent coarctation of the anastomotic site. The anastomosis between the left subclavian artery and the descending thoracic aorta was performed with a single running suture of 7-0 polypropylene and 7-0 polydioxanone in a stable condition, as Blalock and Park [1] reported in 1944. Pulmonary artery banding was also performed with the circumference of 24.0 mm.
The paient’s hemodynamic and respiratory condition improved remarkably after this operation. He recovered uneventfully after 3 days of respiratory support. Echocardiography showed a sufficient anastomosis between the subclavian artery and the descending thoracic aorta (Fig 2). The child was discharged from the hospital 29 days after the operation without complication. Follow-up at age 11 months revealed that the child was thriving without any cardiac or respiratory symptoms, and cardiac catheterization showed mild aortic valvular stenosis (pressure gradient was 14 mm Hg), mild coarctation (pressure gradient was 12 mm Hg) and normal left ventricular contraction (ejection fraction was 71%). The child is waiting for total correction of the heart defect.
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Fig 2. Echocardiography showed a sufficient anastomosis between the subclavian artery and the descending thoracic aorta.
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Comment
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Pulmonary agenesis has been categorized according to the classification of Boyden in 1955. In group 1, the entire lung and its pulmonary artery are absent. In group 2, the lung and pulmonary artery are absent, but there is a rudimentary bronchus coming off the trachea. In group 3, there is hypoplastic lung and a fully formed bronchus. Hypoplastic lung, when present, is subject to atelectasis and infection. The patient in our case was classified to group 1 by the bronchoscopy. Although the bronchoscopy might carry substantial morbidity, it might be better to indicate in a patient with repeated infections of the lung or required for correction of the congenital heart disease, because the contralateral bronchial abnormalities might occur in the unilateral pulmonary agenesis. Especially in the case of right-sided defects the greater mediastinal shift produced by right-sided agenesis could lead to more significant distortion of the bronchus. Magnetic resonance imaging also accurately defines the entire spectrum of airway and vascular anatomic abnormalities, however magnetic resonance imaging has a disadvantage of the inability to appreciate the dynamic component of tracheobronchomalasia that could be seen on bronchoscopy.
Pulmonary agenesis occurs on the right and left sides with equal frequency; however, the prognosis for right-sided agenesis is much worse than a left-sided lesion. This difference has been ascribed to a greater shift of the heart and mediastinum with a consequent distortion of the great vessels and airway. The left bronchus was compressed between the enlarged left pulmonary artery anteriorly and a malpositioned descending aorta posteriority. The malposition of the arch and descending aorta appeared to be caused by the extreme dextroposition and rotation of the heart resulting from the absence of the right lung. A similar appearance would be expected in the right pneumonectomy syndrome [2].
Morbidity and mortality in unilateral pulmonary agenesis are related to the severity of associated anomalies as well as to respiratory disease. Almost half of the patients with pulmonary agenesis have other congenital abnormalities, with the incidence being greater in those with right-sided agenesis. Twice as many children whose right lung is absent die by the age of 10 years compared to children whose left lung is absent [3]. Concomitant anomalies in other organ systems frequently coexist with unilateral pulmonary agenesis. They include cardiac anomalies, malformation of the ribs and vertebrae, imperforate anus, tracheoesophageal fistula, and renal agenesis. Ventricular septal defect or total anomalous pulmonary venous drainage were reported as concomitant abnormalities with pulmonary agenesis [4, 5]. However, we could not locate any literature that contains an example of pulmonary agenesis with interrupted aortic arch.
Hemodynamic stability during aortic arch repair is mandatory for extensive circumferential dissection and cross-clamping of the aorta, and also for reduction in the development of residual or recurrent coarctation. However, in the patient with pulmonary agenesis, minimizing the compression to the lung is also essential to repair the aortic arch. Furthermore, the end-to-end anastomosis of the aorta risks left bronchial compression by the reconstructed aorta [6]. The Blalock-Park anastomosis has an advantage in these points.
Because of the shortcomings of the two-stage approach, there has been increasing enthusiasm during the past several years for the one-stage approach, in which the aortic arch is reconstructed and the ventricular septal defect is closed during a single operation. A number of institutions have demonstrated the feasibility of this approach, which largely now supplant the two-stage method. However, in the case of the more complicated heart defects, the operative mortality rates are higher [7]. Although primary single-stage repair is the procedure of choice, the two-stage repair reported here is applicable in the very rare circumstance when primary definitive repair cannot be performed.
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References
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Blaock A., Park E.A. The surgical treatment of experimental coarctation (atresia) of the aorta. Ann Surg 1944;119:445-456.[Medline]
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Stolar C., Berdon W., Reyes C., et al. Right pneumonectomy syndrome: a lethal complication of lung resection in a newborn with cystic adenomatoid malformation. J Pediatr Surg 1988;23:1180-1183.[Medline]
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Schaffer A.J. Pulmonary agenesis and hypoplasia. In: Schaffer A.J., Avery M.E., eds. Disease of the newborn. Philadelphia: WB Saunders, 1960:124-209.
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Ishikawa K., Hoshino S., Iwaya F., et al. A case of ventricular septal defect and atrial septal defect in an infant with right lung aplasia. Jpn J Cardiovasc Surg 1999;28:163-166.
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Finci V., Begbetti M., Kalangos A., Brundler M.A. Unilateral total and contralateral partial pulmonary agenesis associated with total anomalous pulmonary venous drainage. J Pediatr 1999;134:510-512.[Medline]
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Karl T.R., Sano S., Brawn W., Mee R.B.B. Repair of hypoplastic or interrupted aortic arch via sternotomy. J Thorac Cardiovasc Surg 1992;104:688-695.[Abstract]
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Jonas R.A., Quaegebeur J.M., Kirklin J.W., Blackstone E.H., Daicoff G. Outcomes in patients with interrupted aortic arch and ventricular septal defect. J Thorac Cardiovasc Surg 1994;107:1099-1113.[Abstract/Free Full Text]
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Abstract
Introduction
