Aortic Origin of the Right Pulmonary Artery with Patent Ductus Arteriosus

Transcription

1 Aortic Origin of the Right Pulmonary Artery with Patent Ductus Arteriosus Paul W. Sanger, M.D., Frederick H. Taylor, M.D., Francis Robicsek, M.D., and Akram Najib, M.D. 0 rigin of the right pulmonary artery from the ascending aorta is a peculiar anomaly which stands halfway between a true aplasia and a ductus arteriosus. Fraentzel [6], who in 1867 first described this malformation, regarded it only as an anatomical curiosity; however, recent reports [2-5, 7, 8, 101 indicate that this condition is a distinct clinical entity with an extremely dim prognosis. In the following we present a case of a 2-year-old child who, besides suffering from this disease, also had a large patent ductus arteriosus. Our patient was a 2-year-old boy who was known to have had a heart murmur since birth. He had normal mental but retarded physical development. There was no history of cyanosis or congestive heart failure. Physical Examination. Physical examination revealed a somewhat underdeveloped child not in apparent distress. Pulse rate was 122 per minut,e; blood pressure, 115/55 mm. Hg on the left arm and 125/50 on the right arm. Peripheral pulses were all palpable. The heart appeared to be enlarged to the left, with the forceful apical beat located in the left fifth interspace in the anterior axillary line. Pulmonary second sound was accentuated and split. There was a Grade 1 systolic murmur audible over the entire precordium with a maximum point of intensity in the left third interspace parasternally. X-ray and ECG. X-ray examination of the chest showed both ventricles to be enlarged. The pulmonary conus was prominent, and the vascular markings were equally heavy on both sides. Electrocardiogram indicated biventricular hypertrophy antl strain. Laboratory findings were noncontributory. Catheterization. A right heart catheterization was done through the dissected right saphenous vein. The catheter entered the right atrium, right ventricle, and main and left pulmonary arteries. The right pulmonary artery could not be entered. From the left pulmonary artery the catheter passed repeatedly through a patent ductus arteriosus and appeared in the descending aorta. The pressure was 85/2mm.Hg in the right ventricle, 80/45 in the pulmonary artery, antl 98/62 in the aorta. The oxygen saturation was 33.8% in the right ventricle, 48% in the pulmonary artery, antl 84% in the aorta. Cineangiography. Cineangiography was clone in three different catheter positions: With the catheter tip at the base of the right ventricle (Fig. la), 10ml. of Angio-Conray were injected manually. The dye filled the right ventricle, which From Cardio-Pulmonary Research Laboratory, Charlotte Memorial Hospital, Charlotte, K.C. This work was supported by grants from the John A. Hartford Foundation. Received for publication Aug. 12, VOL. 1, NO. 2, MAK.,

2 SANGER, TAYLOR, ROBICSEK, AND NAJIB FIG. 1. Cineangiocardiography with catheter tip located at A, right ventricle; B, main pulmonary artery; C, descending aorta. appeared to be normal. From the right ventricle the dye rapidly passed into the main and left pulmonary arteries and also outlined the ductus arteriosus. A small amount of the contrast material flowed through the ductus arteriosus into the aorta. The dye injection was repeated with the catheter tip in the main pulmonary artery (Fig. 1B). The contrast material outlined again the main and left pulmonary arteries, as well as the ductus arteriosus, aortic arch, and descending aorta, but not the right pulmonary artery. The catheter was then passed through the ductus into the thoracic aorta and another cineangiogram was made (Fig. 1C). A good filling of the thoracic aorta and its main branches was achieved, and they appeared to be normal. This time also the right pulmonary artery was visualized and it appeared to be originating from the ascending aorta. Surgical Procedure. Because of the bidirectional (primarily left-to-right) shunt through the ductus arteriosus, exploration of the ductus was decided upon. The chest was opened through a left posterolateral thoracotomy incision and the ductus was clamped, while the pressures were monitored in the aorta, left pulmonary artery, and inferior vena cava. Following the occlusion of the ductus a rise in the aortic and a fall in the pulmonary arterial pressure were observed. There was no change in the central venous pressure, and the heart beat remained regular and forceful. The ductus was double-ligated permanently and the chest closed. Outcomc~. The child tolerated the surgery well and had an uneventful postoperative recovery. He was discharged 10 days following the procedure, at which time no murmurs could be heard. When seen as an outpatient one week later, 180 THE ANNALS OF THORACIC SURGERY

3 CASE REPORT: Aortic Origin of Pulmonary Artery the child apparently was doing well. Nine weeks after his discharge from the hospital he suddenly turned pale antl collapsed. He was rushed to the hospital where he was pronounced dead on arrival. An autopsy revealed hypertrophy of both ventricular chambers with acute dilatation of the right ventricle. The ductus arteriosus was ligated. The main pulmonary artery continued into the left branch. There was no communication between the pulmonary trunk antl the right pulmonary artery. The latter originated from the posterior aspect of the aorta right above the left sinus Valsalva (Fig. 2). The right pulmonary artery closely resembled a large systemic artery. There were advanced sclerotic changes and medial hypertrophy in the pulmonary arterioles. FIG. 2. Postmortem photography of the heart shows the ligated ductus and the right pulmonary nrtery arising from the posterior surface of the ascending aorta. DISCUSSION According to Schneiderinan [9], aortic origin of the pulmonary artery is caused by the retarded migration of the right sixth aortic arch to the left side of the truncus arteriosus. Others [8, 121 postulate that this malformation is due to the persistence of the distal portion of the sixth primitive arch. In 1957 Car0 et al. [4] collected from the literature five cases of aortic origin of the pulmonary artery with patent ductus arteriosus diagnosed at autopsy, all but one in infants. Since then eight additional cases have been reported. Griffiths [7] and co-workers have pointed out that there are no clinical signs diagnostic of this disease and the diagnosis depends entirely on contrast studies. Up to this date only two such studies have been presented, one in which the abnormality was VOL. 1, NO. 2, MAR.,

4 SANGER, TAYLOK, ROBICSEK, AND NAJIB visualized by peripheral venous angiocardiography and one in which the diagnosis was made by cineangiocardiography [Z]. The question naturally arises of what to do with such a condition after it has been diagnosed. Four cases have been reported in which surgery was undertaken for aortic origin of the right pulmonary artery and patent ductus arteriosus. In 1957 Car0 et al. [4] operated on a 23-year-old man. The patent ductus was ligated, the right pulmonary artery was detached from the aorta, and the distal stump was anastomosed with the aid of an Ivalon graft to the main pulmonary artery. The patient died shortly after the procedure, apparently of hemorrhage. In 1960 DuShane et al. [51 limited the procedure to the obliteration of the patent ductus in a 2-month-old infant. The child did well for a few hours following division and suture of the ductus arteriosus, but died the day after the operation. Vlad and Lambert [l 11 extended the procedure in a 4-monthold infant to the ligation of both the patent ductus arteriosus and the anomalous pulmonary artery. There was a dramatic relief in the congestive heart failure, but the infant died four weeks later of pneumonia. Armer et al. [Z] in 1962 were able to restore normal circulation in a 1 0-month-old boy by ligating the patent ductus arteriosus, and dividing the anomalous pulmonary artery and anastomosing it with an interpolated Dacron graft to the main pulmonary trunk. The child was examined 15 months after surgery and was reported as normal. Their surgical accomplishment undoubtedly points to the fact that this should have been the procedure chosen also for our patient. SUMMARY The clinical, cineangiographic, and autopsy findings of a Z-yearold boy with aortic origin of the right pulmonary artery and patent ductus arteriosus have been presented. The child died after ligation of the patent ductus. A brief review of the literature has been given and an appeal made for the attempt of complete repair. REFERENCES 1. Ambrus, G. Congenital absence of the right pulmonary artery with bleeding into the right lung. J. Tech. Meth. 15: 103, Armer, R. M., Shumacker, H. B., Jr., and Klatte, E. C. Origin of the right pulmonary artery from the ascending aorta: Report of a surgically corrected case. Circulation 24:662, Bopp, F. Abnormal arterial blood supply of the right lung (right pulmonary artery originating from the aorta with normal supply of the left lung from the pulmonary artery). Zbl. Chir. 85:155, Caro, C., Lermanda, V. C., and Lyons, H. Aortic origin of pulmonary artery. Brit. Heart J. 19:345, THE ANNALS OF THORACIC SURGERY

5 CASE REPORT: Aortic Origin of Pulmonary Artery 5. DuShane, J. W., Weidman, W. H., Ongley, P. A., Swan, H. J. C., Kirklin, J. W., Edwards, J. E., and Schmutzler, H. Clinico-Pathological Conference. Amer. Heart J. 59:782, Fraentzel, 0. Angeborener Defect der rechten Lungenarterie. Virchow Arch. Path. Anat. 43:420, Griffiths, S. P., Levine, R. O., and Anderson, D. H. Aortic origin of the right pulmonary artery. Circulation 25:73, Porter, D. D., Canent, R. V., Spach, M. S., and Baylin, G. J. Origin of the right pulmonary artery from the ascending aorta: Unusual cineangiocardiographic and pathologic findings. Circulation 27:589, Schneiderman, L. J. Isolated congenit,al absence of the right pulmonary artery: A caution as to its diagnosis and a proposal for its embryogenesisreport of a case with review. Amer. Heart. J. 55:772, Sikl, H. Unusual malformation of arterial trunk: Origin of a main branch of pulmonary artery from the aorta. Cas. Lek. Cesk. 91:1366, Vlad, P., and Lambert, E. C. Personal communication to Armer et al., Wagenvoort, C. A., Neufeld, H. N., Birge, R. F., Caffrey, J. A., and Edwards, 1. E. Origin of the right pulmonary artery from ascending aorta. Circulation 23:84, VOL. 1, NO. 2, MAR.,