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ANOMALOUS ORIGIN OF LEFr PULMONARY ARTERY and others,2s3but not as often as Coxsackie viruses. Since carditis due to ECHO viruses has been associated with serious consequences, including life-threatening arrhythmias and even death, the diagnosis of asymptomatic or truly benign pericarditis or myocarditis occurring with ECHO viruses is obviously i m p ~ r t a n t . ~ - ~ Therefore, the so-called "benign" pericarditis associated with enterovirus disease may be a somewhat misleading classification. The electrocardiographic changes in our patient were thought to be consistent with viral pericarditis and myocarditis. There were no historical or clinical grounds to associate them to coronary artery disease, hypokalemia, alkalosis or severe central nervous system disease. The elevations of the serum glutamic pyruvic transaminase pointed to liver involvement. There was a sustained rise in the alkaline phosphatase. Confirmation of the hepatic origin of these admittedly small enzyme changes were furnished by the simultaneous elevation of the patient's serum guanase and his ornithine carbamyl transferase. These two latter enzymes, particularly guanase, are specific for parenchymatous liver disease. The patient's appetite remained depressed for a month, and he was found to be hypoglycemic on several occasions despite reduction of insulin dosage. This anorexia might possibly have been due to hepatic involvement. Hepatitis associated with ECHO 9 virus disease has been reported in a previous communication by A recent editorialR in the Journal of the American Medical Association appears pertinent in regard to this case. The editorial writer states that isolation and identification of ECHO virus from the spinal fluid is preferable for definitive diagnosis in patients with this type of meningitis as compared to isolation from the throat and feces. The former was accomplished in our ~ a t i e n t . ~ The pathophysiology of cardiac involvement in enterovirus disease is a subject for a recent editorial by L e r n e r . V e points out the occurrence of an early acute infectious phase and prolonged auto-immune and recovery phase of enteroviral myocarditis. Accordingly, our patient was not permitted to return to work until his electrocardiogram had become completely normal and his fatigue had subsided. REFERENCES 1 Melnick JL, Aagren K: Poliomyelitis and Coxsackie viruses isolated from normal infants in Egypt. Proc Soc Exper Biol Med 81 :621-624, 1952 2 Kibrick S: Current status of Coxsackie and ECHO viruses in human disease. Prog Med Virol6:27-70, 1964 3 Bell EJ, Grist NR: ECHO viruses, carditis and acute pleurodynia. Am Heart J 82:133-135, 1971 4 Mendex-Cashion D, Sanchez-Longo LP, Valcarcel MI, et al: ECHO virus type 1 and aseptic meningitis. J Pediat 63:432-436, 1963 5 Taytsch FZ: Wirusy Echo izolowane z przypadkow zachorowan z objawarni zapalenia opon mozgowych. P ~ e g l a dEpidemiologicmy ROK XV: 179-187, 1981 6 Johnson RT, Portnoy B, Rogers NG, et al: Acute benign pericarditis. Arch Intern Med 108:823-832, 1961 7 Schleissner LA, Portnoy B: Hepatitis and pneumonia associated with ECHO virus, type 9, infection in two adult siblings. Ann Intern Med 68:1315-1319, 1968 8 Editorial: Echovirus as a cause of meningism. JAMA 212:1206, 1970 9 Lemer AM: Coxsackie virus myocardiopathy. (Editorial) J Infect Dis 120:496-499, 1969 Anomalous Origin of Left Pulmonary Artery from Ascending Aorta, Right Aortic Arch and Right Patent Ductus ~rteriosus* Walter H. Herbert, M.D., F.C.C.P.;" Michael Rohman, M.D., F.C.C.P.;? Peter Farworth, M.D.;$ and Saraswathi Swamy, M.D.5 A case of anomdous left pulmonary artery (ALPA) arising from the ascendiog aorta, a right aortic arch and a right patent ductus arterims, a combination not previously reported, is described. Current concepts regarding the genesis of an ALPA also predict main pulmonary artery hypoplasia and defects of the ventricular septum. These associated defects were not present io our patient. Their absence suggests that the developmental faults associated with these abnormalities are even more complex than previously suspected. The need for early diagnosis and the efficacy of surgical correctioo are emphasized. I n 1969 Caudill and co-workers' described a case of anomalous origin of the left pulmonary artery arising from the aorta and added the 35th such report to the world literature. Anomalous origin of the right or left pulmonary artery (ALPA, ARPA) from the aorta or its primary divisions (ie innominate) is a rare phenomenon although it was first recognized over 100 years ago.2 It was not until 1949, however, that Bopp3 demonstrated an ARPA at postmortem examination. The importance of angiographic studies to demonstrate the origin of both pulmonary arteries has been emphasized by several authors1.4-'2 as an ALPA or ARPA is a correctible lesion and successful surgical It is, of intervention has been reported.1~e~7*10-1Z course, most important to meticulously seek out associated lesions which are so frequently p r e ~ e n t . ~ ~ ~ - ' ~ * ' ~ This presentation describes the association of an ALPA arising from the ascending aorta, a right aortic arch and a right patent ductus arteriosus, a combination which has not, to our knowledge, been previously reported. This association of defects is particularly noteworthy as it does not include pulmonary outflow anomalies or defects of the ventricular septum. These latter deficiencies would be anticipated by the current and 'From the Cardiopulmonary Laboratory and Departments of Surgery and Pediatrics, Grasslands Hosnital, Valhalla; and De artments of Medicine and Surgery, New York Medical coRege, New York. ''Assistant Professor of Medicine. tProfessor of Surgery. :Director of Pediatrics, Grasslands Hospital. $Fellow, Westchester Heart Association. Reprint requests: Dr. Herbert, Grasslands Hospital, Vahalh, New York 10595 CHEST, VOL. 63, NO. 3, MARCH, 1973 Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/20935/ on 05/02/2017 HERBERT ET AL FIGURE1. The catheter passes up from the right saphenous vein to enter the right atrium, right ventricle (RV), main pulmonary artery ( MPA), right pulmonary artery ( RPA) and the descending aorta (DAo) in the right chest via a right duchls artenosus. FIGURE2. Contrast media is delivered to the descending aorta ( DAo) via the right ductus arteriosus ( D ) . The proximal aorta also fills demonstrating the large left pulmonary artery ( LPA ) arising from the ascending aorta ( AAo ) . widely held concepts of developmental faults which cause main trunk pulmonary arteries to arise from the aorta.ll.lS ly unsaturated. Cineangiocardiographic studies demonstrated the left pulmonary artery to arise from a left lateral position on the ascending aorta (Fig 2,3). It rose steeply to approximately the same height as the aortic arch and then ramified normally to supply the left lung. The ventricular septum was intact (Fig 3). A large right pulmonary artery rose from a large main pulmonary trunk ( Fig 4 ) . A six-week-old white boy was referred with a history of cyanotic spells beginning after a few days of life. These episodes were related to crying and feeding. The infant was delivered normally at term with a birth weight of 5%Ib. A diagnosis of preeclampsia was made in the third trimester. The pertinent physical findings were: weight, 7%lb and slight facial cyanosis. The respiratory rate was 36 per minute. The lungs were clear. The heart was enlarged to percussion and a grade 111 rough systolic murmur was heard best at the left sternal border. The liver edge was palpable below the right costal margin. There were bilateral talipes equinovarus deformities of the legs. A venous catheter was advanced to the heart via the right saphenous vein. The catheter passed easily into the right atrium, right ventricle, right pulmonary artery and then into the descending aorta in the right chest via the right ductus arteriosus (Fig 1 ). It was also possible to manipulate the catheter from the right to the left atrium and then into the left ventricle. There were no demonstrable systolic gradients across either the pulmonary or the aortic valves. Systolic pressures in the mid portions of both ventricles, the pulmonary artery and the aorta were similar. The left ventricular end-diastolic pressure was abnormally elevated (Table 1 ). Left atrial, left ventricular and aortic blood was moderate- On February 2, 1972, surgical exploration confirmed the catheterization findings. The left main pulmonary artery had a diameter of 1 cm and originated from the left side of the ascending aorta, approximately 2 cm distal to the origin of the left coronary artery. The ascending aorta turned posteriorly toward the right and descended behind the right main pulmonary artery. There was a patent ductus arteriosus 1 cm in length and 0.4 cm in diameter between the proximal portion of the descending aorta and the right main pulmonary artery. After ascertaining that temporary interruption of the ALPA could be tolerated, this structure was transected and anas- Table 1 4 a t h e t e r i z a t i o n Data - - Site Right atrium Left atrium Right ventricle Left ventricle Pulmonary artery Aorta Saturation 56 86 63 88 59 87 70 Pressure mm Hg 4 4 85/3 87/15 82/34 79/33 FIGURE3. A left ventriculogram (LV) was exposed in a left anterior oblique projection. The large left pulmonary artery arising from the ascending aorta is clearly seen ( Catheter, C ) . CHEST, VOL. 63, NO. 3, MARCH, 1973 Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/20935/ on 05/02/2017 ANOMALOUS ORIGIN OF LEFT PULMONARY ARTERY and a ventricular septal defect, overridden by a large aorta, ensues." The ontogenetic theory of Cucci and co-workerslS reasonably explains the usual anatomic alignment of patients with anomalous origin of a main pulmonary artery. However, the findings in our case, demonstrated in the catheterization laboratory and at surgical exploration, of a large pulmonary artery with no evidence of pulmonary trunk hypoplasia or ventricular septal defects and yet ALPA and right aortic arch suggest that the developmental fault may be still more complex. The successful surgical correction in a six-week old infant underscores the need for early angiographic diagnosis. Since cyanosis, severe pulmonary hypertension and progressive pulmonary vascular bed changes are an integral part of this syndrome, early diagnosis and surgical correction are warranted. FIGURE 4. Contrast material was delivered to the main pulmonary artery (MPA). Only a right distribution is apparent. tomosed to the side of the rather large main pulmonary artery. The ductus arteriosus was then isolated and obliterated. The procedure was well tolerated and the infant made a rapid uncomplicated recovery. Cucci and colleagues13 discussed the various theories which have been suggested to account for the absence of a primary division of the pulmonary trunk. They concluded that the theories invoking developmental errors of the sixth arch did not account for the combinations of abnormalities observed and that defective truncoconal septation did. Specifically, they noted that prior theories accounted for neither the association of ALPA and tetralogy of Fallot1."11J3 nor the location of the aortic arch on the side opposite that of the absent pulmonary artery. They postulated that dorsorotation of the left or right truncoconal ridge caused the ipsilateral sixth arch to be incorporated into the ascending aorta. Either rotational fault would cause the resultant aorta to be large at the expense of the pulmonary trunk. In addition, either would (due to spiral rotation) cause blood to flow preferentially into the contralateral fourth arch. This would then cause this arch to persist as the aortic arch while its paired branch would resorb. In the event of dorsorotation of the right truncoconal ridge, the right pulmonary artery will then arise from the ascending aorta and the left fourth arch will persist normally. The degree of rotation needed to produce this combination is slight and is therefore usually an isolated finding. Left dorsorotation sufficient to cause the left pulmonary artery to arise from the ascending aorta and a persistence of the right fourth arch is substantially greater. It is to this striking degree of rotation that Cucci and associates13 attribute the association of Fallot type abnormalities. These authors state, "The incorporation of the left sixth arch into the ascending aorta requires a degree of dorsorotation so pronounced as to cause hypoplasia of the pulmonary trunk and of the right ventricular outflow tract. The truncoconal and the muscular ventricular septa lying on different planes cannot fuse ACKNOWLEDGMENT: We would like to express our appreciation for the efforts of Deborah Telesco, RN, Philomena Adinaro, PN, Margaret Teague, RN, Pattie Baskette, Catherine Doyle, anet Waldie and Alberta Varble in the preparation of this wor . L 1 Caudill DR, Helmsworth JA, Daoud C, et al: Anomalous origin of left pulmonary artery from ascending aorta. J Thorac Cardiovasc Surg 57:493-506, 1969 2 Fraentzel 0:Ein fall von abnormer communication der aorta mit der arteria pulmonalis. Arch Anat Physiol 43 :420-426, 1868 3 Bopp F: Anonnale arterielle Gef'iissversorgung der rechen lunge (Abgang der rechen arteria pulmonalis aus der aorta bei norrnaler versorgung der linken lunge durch die pulmonalarterie) . Zentralbl Path Path Anat 85: 155-160. 1949 4 Cam C, Lermanda VC, Lyons HF: Aortic origin of the right pulmonary artery. Br Heart J 19:345-352, 1957 5 Dushane JW, Weidman WH, Ongley PA, et al: Clinicalpathologic Conference. Am Heart J 59:782-788, 1960 6 Armer RM, Schumacker HB, Klatte EC: Origin of the right pulmonary artery from the ascending aorta. Report of a surgically corrected case. Circulation 24:662-668. 1961 7 Criffiths SP, Levine OR, Andersen DH: Aortic origin of the right pulmonary artery. Circulation 25:73-84,1962 8 Pool PE, Vogel JHK, Blount SG Jr: Congenital absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am J Cardiol 10:706-732, 1962 9 Czarnecki SW, Hopeman AR, Child PL: Tetralogy of Fallot with aortic origin of the left pulmonary artery. Radiographic and arteriographic considerations. Dis Chest 46:97-101, 1964 10 Weintraub RA, Fabian CE, Adarns DF: Ectopic origin of one pulmonary artery from the ascending aorta. Radiology 86:66-676,1966 11 Winship WS, Beck W, Schrire V: Congenital "absence" and anomalous origin of the main pulmonary arteries. Br Heart J 29:34-42, 1967 12 Flege JB Jr, Durnin RE, Rossi NP: Aortic origin of the right pulmonary artery and ventricular septal defect. J Thorac Cardiovasc Surg 59:468-473, 1970 13 Cucci CE, Doyle EF, Lewis EW Jr: Absence of a primary division of the pulmonary trunk. An ontogenetic theory. Circulation 29: 124-131. 1964 CHEST, VOL. 63, NO. 3, MARCH, 1973 Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/20935/ on 05/02/2017