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Ann Thorac Surg 1998;65:1790-1791
© 1998 The Society of Thoracic Surgeons

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Case Reports

The Large Window Ductus: A Surgical Trap

^a Department of Pathology, Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
^b Division of Cardiology, Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
^c Division of Cardiothoracic Surgery, Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

Accepted for publication January 13, 1998.

Address reprint requests to Dr Richard Van Praagh, Cardiac Registry, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115

	Abstract

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A rare window type of patent ductus arteriosus is reported that was large (15 mm in maximal transverse dimension) but had virtually no length and hence was externally invisible. The smaller aortic isthmus (4 mm in diameter), which was intrapericardial, was mistaken for the ductus and was inadvertently clip-occluded, leading to death. After a specific diagnosis is made, the large window ductus should be patched on cardiopulmonary bypass with a transpulmonary approach.

	Introduction

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When a large patent ductus arteriosus (PDA) is wide in diameter and very short in length, upon external inspection it may not be visible. This is a serious surgical problem, as the following case illustrates.

An 18-month-old boy with multiple congenital anomalies underwent echocardiography and cardiac catheterization that were interpreted as showing cor triatriatum, persistent left superior vena cava, unroofed coronary sinus, secundum atrial septal defect, a hemodynamically large but structurally ill-defined PDA, and pulmonary hypertension.

Cardiac surgical repair at 19 months of age consisted of excision of an obstructive diaphragm within the left atrium, rerouting of the persistent left superior vena cava to the right atrium via a Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) tunnel, patch closure of the secundum atrial septal defect and of the enlarged coronary sinus ostium using bovine pericardium, and occlusion by two clips of the intrapericardial structure interpreted to be the PDA. The postoperative course was characterized by hypoxemia, acute renal failure, hepatic and intestinal ischemia, poor peripheral perfusion, and absent pulses in the left leg, leading to death 1 day postoperatively.

Autopsy revealed right atrial hypertrophy (2 to 3 mm in thickness/1 mm in normal controls), marked right ventricular hypertrophy and enlargement (6 to 10 mm/2 to 3 mm), and moderate dilatation of the main pulmonary artery and branches (Fig 1A).

View larger version (105K): [in this window] [in a new window]   Fig 1. (A) External frontal view of the specimen of heart and lungs. Note the two surgical clips (Clips) occluding the aortic arch just proximal to the large patent ductus arteriosus (PDA). (B) Frontal view of opened right ventricle (RV), pulmonary valve (PV), main pulmonary artery (MPA), PDA, and descending thoracic aorta (Desc Ao) to show very large and extremely short window ductus. Note the absence of wrinkled ductal intimal pads. The MPA is confluent via the PDA with the Desc Ao. (Asc Ao = ascending aorta; Innom = innominate artery; LA = left atrium; LCC = left common carotid artery; LS = left subclavian artery; LV = left ventricle; RA = right atrium.)

Autopsy also revealed a persistent left superior vena cava connecting with the coronary sinus and draining into the right atrium, a small left innominate vein, partial unroofing of the coronary sinus consisting of a 10-mm-long coronary sinus septal defect adjacent to the left atrial appendage, totally anomalous pulmonary venous connection to the coronary sinus, no evidence of cor triatriatum, bilateral severe cerebral dysplasia with asymmetric megalencephaly and polygyria (greater of the left cerebrum than of the right), almost total absence of the left cerebellar hemisphere, and histologic evidence of widespread ischemic changes below the diaphragm (involving the liver, gastrointestinal tract, pancreas, kidneys, lumbar spinal cord, and skeletal muscles), but none above the diaphragm.

	Comment

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The large window ductus is a surgical trap because the presence of a huge, very short PDA is not apparent on external inspection. Instead, the main pulmonary artery appears to be directly continuous with the descending thoracic aorta, resembling one continuous artery with no visible interposed ductus. If the relatively small aortic isthmus is also intrapericardial, as in the present rare case, then the aortic isthmus strongly resembles a PDA. This combination of (1) a large but externally invisible window ductus and (2) a relatively small and intrapericardial aortic isthmus together constitutes a potentially lethal surgical trap. Mistaken for the PDA, the aortic isthmus can be erroneously occluded, as in the present case.

The surgeon needs to know that it is anatomically and developmentally impossible for the main pulmonary artery to be directly continuous with the descending thoracic aorta, despite external appearances. The main pulmonary artery always communicates with the descending thoracic aorta via the PDA, not directly. When the PDA is very large and unconstricted, as in the normal embryo and fetus, then the PDA is as wide as the main pulmonary artery and the descending thoracic aorta. Hence, upon external inspection, the PDA "disappears," particularly if it is extremely short, as in the present case.

The surgeon should also understand that the PDA is located directly above the ostium of the left pulmonary artery (see Fig 1B). Another potential source of confusion is that when viewed from the front as via a median sternotomy, a very large PDA obscures the origin of the left pulmonary artery (see Fig 1A). It is also helpful to understand that the left recurrent laryngeal branch of the left vagus nerve curls under the aortic arch from left to right, distal to the PDA—not proximal to the PDA and hence not beneath the preductal portion of the aortic arch. Consequently, the recurrent laryngeal nerve may be regarded as a surgical guide to the PDA: no recurrent laryngeal nerve, no ductus arteriosus.

Postoperatively, the presence of aortic isthmic interruption can be masked by right-to-left blood flow through the large unoccluded window ductus into the descending thoracic aorta. Consequently, two-dimensional echocardiography with color-flow Doppler may not detect total iatrogenic interruption of the aortic isthmus. However, the lower body is perfused postoperatively by unsaturated venous blood, as was confirmed histologically in the present case.

Simulating a very distal aortopulmonary window, the large window ductus appears to be exceedingly rare. Among the 3,400 postmortem cases of congenital heart disease currently retained in the Cardiac Registry of the Children’s Hospital, Boston, there is not a single case of large window ductus. In the literature, we have been able to find only 1 similar case that was reported in 1953 by Lev (his Figure 98b, page 130) [1].

Because a large externally invisible window ductus cannot be ligated and divided, or clip-occluded in the usual way, we propose that appropriate surgical management should consist of patch occlusion on cardiopulmonary bypass with a transpulmonary approach.

	References

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1. Lev M. Autopsy diagnosis of congenitally malformed hearts. Springfield: Charles C Thomas, 1953:130.

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Jürg Grünenfelder Richard Van Praagh Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Grünenfelder, J. Articles by Schwartz, D. C. Search for Related Content PubMed PubMed Citation Articles by Grünenfelder, J. Articles by Schwartz, D. C.