Ann Thorac Surg 1996;61:220-222
© 1996 The Society of Thoracic Surgeons
Case Report
Truncus Arteriosus With Total Anomalous Pulmonary Venous Return and Interrupted Arch
Farhouch Berdjis, MD,
Winfield J. Wells, MD,
Vaughn A. Starnes, MD
Divisions of Pediatric Cardiology and Cardiothoracic Surgery, Children's Hospital of Los Angeles, University of Southern California School of Medicine, Los Angeles, California
Accepted for publication July 15, 1995.
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Abstract
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An infant with truncus arteriosus, total anomalous pulmonary venous return of the supracardiac type and interrupted aortic arch distal to the left subclavian artery is presented. One-stage repair was performed in the newborn period using a 12-mm aortic homograft. Recovery was complicated by congenital stenosis of the left main bronchus. The cardiovascular status remains stable 2 years after repair.
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Introduction
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Truncus arteriosus can be associated with many other congenital cardiac defects [1–33]. These include partial anomalous pulmonary venous return [3], but total anomalous pulmonary venous return associated with truncus arteriosus is exceedingly rare [4]. We present such a case.
A full-term female infant weighing 3.2 kg presented with meconium aspiration and cyanosis. The cardiac examination revealed a hyperactive precordium, a single and loud second sound, and a 2/6 ejection murmur at the left middle sternal border; diastole was clear. The pulses were full and symmetric, and the liver was 2 cm below the costal margin. An electrocardiogram was compatible with right ventricular hypertrophy. A chest roentgenogram demonstrated moderate cardiomegaly, increased pulmonary markings, and some hemivertebrae. At an inspired oxygen fraction of 0.90 arterial blood gas analysis revealed a pH of 7.28, oxygen tension of 78 mm Hg, carbon dioxide tension of 45 mm Hg, and an arterial saturation of 91%. An echocardiogram demonstrated situs solitus, truncus arteriosus with a large malalignement ventricular septal defect and a quadricusp semilunar valve, interrupted aortic arch, and the possibility of total anomalous pulmonary venous return of the supracardiac type. The infant was placed on prostaglandin E1 due to the interrupted aortic arch. Cardiac catheterization was performed, confirming the diagnoses. The truncus communis separated into a very short main pulmonary segment and an ascending aorta bifurcating into two brachiocephalic trunks. The aortic arch was interrupted distal to the left subclavian artery, and the ductus arteriosus supplied flow to the descending aorta (Fig 1
). A vertical vein was identified, draining all four pulmonary veins via a common confluence (Fig 2) with a 10 mm Hg gradient.
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Fig 1. . Lateral aortogram with balloon occlusion of the descending aorta illustrating the interruption of the aortic arch and origin of the main pulmonary artery.
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The patient underwent operation on day 11 of life. The branch pulmonary arteries measured 4 to 5 mm in diameter. The infant was placed on cardiopulmonary bypass and cooled to 18°C. After circulatory arrest had been completed an atrial septal defect was closed. The anomalous pulmonary venous confluence was opened along its horizontal portion. A side-to-side anastomosis with the left atrium was created. The aorta and neck vessels were completely mobilized. The area of the isthmus was excised and an end-to-end anastomosis was performed. The pulmonary arteries were detached, and the opening in the posterior aspect of the aorta was closed primarily. To achieve complete visualization of the morphology we maintained circulatory arrest for the remainder of the repair. The right ventricle was opened beneath the truncal valve in the infundibular area. The ventricular septal defect was closed with a patch. The 12-mm aortic homograft was anastomosed distally end-to-end to the main pulmonary artery confluence. It was sutured into the right ventricle with the help of a beveled Dacron hood. After completion of the repair the infant was recannulated and rewarmed. Normal sinus rhythm returned. Weaning from cardiopulmonary bypass was possible with 7 µg•kg-1•min-1 of dopamine, 10 µg• kg-1•min-1 of dobutamine, and 0.05 µg•kg-1•min-1 of prophylactic prostaglandin E1. Total perfusion time was 71 minutes with a total circulatory arrest time of 76 minutes. The total amount of cardioplegia was 60 mL. Due to myocardial edema the sternum was left open and covered with silicone sheeting to avoid compromise of cardiac output. It was closed 6 days later without complications. The infant returned to the intensive care unit in moderately stable condition. Postoperatively she remained hemodynamically stable but complete left lung collapse became evident. A bronchoscopy revealed a hitherto undetected stenosis of the left main bronchus. With the help of sigh ventilation, extubation was possible on postoperative day 19. The arterial oxygen saturation remained greater than 95% on room air, and after the patient established a satisfactory feeding pattern and weight gain she was discharged in stable condition receiving oral digoxin and diuretics. A residual ventricular septal defect closed spontaneously within a few weeks. Mild aortic regurgitation and moderate peripheral pulmonary stenosis persisted.
Cardiac catheterization was performed 6 months postoperatively. The right ventricular/left ventricular pressure ratio was 0.55 with a 10 mm Hg gradient across the homograft and a further 15 mm Hg gradient toward both branch arteries without a localized obstruction or compression of the right pulmonary artery by the aortic root [5]. The branch pulmonary arteries remained small. Moderate regurgitation of the neoaortic valve was present. Mild narrowing of the distal aortic arch was seen without a gradient. Pulmonary venous return was unobstructed. At the time of this writing the patient is 2 years old. A magnetic resonance imaging study demonstrated persistent stenosis of the main left bronchus. The patient continues to receive nebulizer treatments due to continuing bronchospasm. From a cardiovascular standpoint the child remains very stable on a regimen of diuretics and digoxin. Developmentally she demonstrates mild delay. Her growth is slightly less than the fifth percentile for length and weight.
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Comment
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Our report constitutes a description of a patient with truncus arteriosus, interrupted aortic arch, and total anomalous pulmonary venous return in situs solitus. The search for additional congenital malformations is important as evidenced by the presence of severe anomalies within the respiratory tract in our patient. We presume the bronchial stenosis to be congenital as the postoperative angiography did not demonstrate any vascular structure compressing the respiratory tree. Our report demonstrates that with exact preoperative diagnosis and careful intraoperative technique and postoperative management even very complex congenital heart disease can be repaired successfully in one stage during infancy. Further, in this particular case, any palliative procedure would have involved repairing the obstructed pulmonary veins and interrupted arch, both involving circulatory arrest. Therefore the best approach in this infant was total repair. Careful preoperative assessment, as demonstrated in this case, is vital to the planning of the surgical options for these critically ill infants with complex congenital cardiac lesions.
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Footnotes
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Address reprint requests to Dr Berdjis, Division of Cardiology, Children's Hospital of Orange County, 455 S Main St, Orange, CA 92668.
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References
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- Mair DD, Edward WD, Fuster V, Seward JB, Danielson GK. Truncus arteriosus and aortopulmonary window. In: Anderson RH, ed. Paediatric cardiology. Edinburgh: Churchill Livingstone, 1987:913-5.
- Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications: a study of 57 necropsy cases. Am J Cardiol 1965;16:406–25.[Medline]
- Mair DD, Ritter DG, Davis GD, Wallace RB, Danielson GK, McGoon DC. Selection of patients with truncus arteriosus for surgical correction: anatomic and hemodynamic considerations. Circulation 1974;49:144–51.[Abstract/Free Full Text]
- Bharati S, McAllister HA Jr, Rosenquist GC, Miller RA, Tatooles CJ, Lev M. The surgical anatomy of truncus arteriosus communis. J Thorac Cardiovasc Surg 1974;67:501–10.[Medline]
- Rao IM, Swanson JS, Horaguimian H, McIrvin DM, King DH, Starr A. Anterior pulmonary translocation for repair of truncus arteriosus with interrupted arch. Ann Thorac Surg 1995;59:216–8.[Abstract/Free Full Text]
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Abstract
Introduction
