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Anomalous Pulmonary and Systemic Venous Drainage by A Thorax: first published as 10.1136/thx.11.2.119 on 1 June 1956. Downloaded from Thorax (1956), 11, 119. ANOMALOUS PULMONARY AND SYSTEMIC VENOUS DRAINAGE BY A. A. FITZGERALD PEEL, K. BLUM, J. C. C. KELLY, AND T. SEMPLE From the Victoria Infirmary, Glasgow (RECEIVED FOR PUBLICATION NOVEMBER 26, 1955) In Abbott's (1936) necropsy series of 1,000 cases coronary sinus, either with or without a connexion of congenital heart disease, anomalies of the great to the right superior vena cava through a left veins were found in 13. Of these nine had innominate vein; and (b) those in which the left abnormal major systemic veins and four had superior vena cava receives one or more pulmon- anomalous pulmonary veins. This classical series ary veins. In group 2 no right superior vena cava is based on findings before such modern methods is present. It will be noted that in group 1 (b) as angiocardiography and cardiac catheterization arterial blood enters the superior vena cava, but were widely used. Taussig (1947) differentiates groups 1 (a) and 2 involve systemic veins alone cases in which some pulmonary veins drain into and do not lead to an arterio-venous shunt. A the right atrium from those in which all pulmonary veno-arterial shunt occurs only in those cases, veins do so. She regards anomalies of the apparently few in number, in which the left superior and inferior venae cavae as rare and superior vena cava enters the left atrium. copyright. difficult to diagnose. She records one case in Examples have been described by McCotter (1916), which both superior and inferior venae cavae Rodriguez Diaz, Castellanos, and Garcia Faes entered the left atrium, one in which there was (1949), Mankin and Burchell (1953), and Feindt no inferior vena cava, and two in which a left- and Hauch (1953). Rarely the inferior vena cava sided vena cava entered the left side of a common enters the left atrium, producing a veno-arterial http://thorax.bmj.com/ atrium. She concludes that anomalies of the shunt (Taussig, 1947; Gardner and Cole, 1955). superior and inferior venae cavae rarely constitute A persistent left superior vena cava is most the sole malformation, and that only the coexist- often found as an additional abnormality in ing cardiac malformation permits diagnosis. patients with some other form of cardiac mal- With improved diagnostic methods many more formation. Brown (1952) reports a case of aortic examples of abnormal venous drainage are being coarctation with a single left superior vena cava. recognized and reported. A survey of the litera- Winter (1954) has reviewed the literature and ture suggests that, for practical purposes, these added 30 new cases. In 28 of the 30 cases the may be divided into three groups: left superior vena cava persisted in its entirety, on September 26, 2021 by guest. Protected (1) Anomalies involving the systemic veins while in two it persisted only in part. Anomalous alone. drainage of pulmonary veins was found in three (2) Anomalies involving the pulmonary veins of these cases: on the other hand a septal defect alone. was present in 25. Other associated anomalies (3) Anomalies involving both systemic and pul- were aortic septal defect, Fallot's tetralogy, truncus veins and associated with arteriosus, coarctation of the aorta, patent ductus monary pulmonary arteriosus, and pulmonary stenosis. Campbell and systemic communications. Deuchar (1954) found 46 examples in their series of cases of congenital heart disease. In 18 of these ANOMALIES INVOLVING SYSTEMIC VEINS ALONE there was partial or complete transposition of the Of these the most common is a persistent left viscera, and 13 of them had either proved or superior vena cava. McManus (1941) differen- suspected pulmonary stenosis; in four instances tiated two main groups of cases. In group 1 both the inferior vena cava was absent. In the remain- right and left superior venae cavae persist; this ing 28 cases the heart and abdominal viscera were group is further subdivided into (a) those in which normally placed, but various additional abnormali- the left superior vena cava is connected to the ties were present. In most of their cases the left Thorax: first published as 10.1136/thx.11.2.119 on 1 June 1956. Downloaded from 120 A. A. FITZGERALD PEEL, K. BLUM, J. C. C. KELLY, and T. SEMPLE superior vena cava joined the coronary sinus to of recorded cases had mounted to 45, to which reach the right atrium. They have not seen an Keith, Rowe, Viad, and O'Hanley (1954) added example in which it entered a normal left atrium, another 14. Yet another case has been added by but have seen it open into a common atrium or Arthurton, Gibson, and Woodwark (1954). into the left half of an atrium with a large septal Jorgens, LaBree, Adams, and Rigler (1952) first defect. Anomalous pulmonary venous drainage described the radiological features of venous was noted in one of their cases only. They do not drainage anomalies, demonstrating a persistent consider the presence of a left superior vena cava left superior vena cava and also drainage of pul- to be of much practical importance, but suggest monary veins into the superior vena cava. Snellen that, if it is suspected, cardiac catheterization is and Albers (1952) describe partial pulmonary best carried out from the right side, and, if dis- drainage into the superior vena cava, right atrium, covered at operation, that ligation should not be and left innominate vein, and total pulmonary performed. On the other hand, Rodriguez Diaz drainage into the superior vena cava, coronary and his co-workers have ligated a left superior sinus, right atrium, left innominate vein and the vena cava which drained into the left atrium with portal vein. They draw attention to a " figure of an excellent result. Difficulties may be encountered eight" appearance in the postero-anterior radio- with catheterization if the atrium is entered graph, and stress the frequency of right bundle through the coronary sinus from a left superior branch block due to coexisting atrial septal de- vena cava (McMichael and Mounsey, 1951). fect. Tori (1952) reports a case in which a right pulmonary vein opened into the superior vena ANOMALIES OF PULMONARY VEINS ALONE cava, while Odman (1953) describes a patient with Anomalies may consist of an abnormal number high coarctation of the aorta in whom a persistent of pulmonary veins or abnormal termination of left superior vena cava received pulmonary veins some or all pulmonary veins (Kugel and Poschl, and joined the left atrium. 1954). Provided there is no pulmonary-systemic A symposium from the Mayo Clinic on pulmon-copyright. venous communication, the condition appears to ary-systemic venous connexions includes interest- be of academic importance only. We know of ing papers by Edwards (1953), Swan, Burchell, one patient who was found at pneumonectomy for and Wood (1953) and Mankin and Burchell (1953). right-sided bronchiectasis to have a shrunken lung Edwards emphasizes the important distinction be- with normal stem bronchus, a hypoplastic pulmon- tween total and partial pulmonary-systemic venous http://thorax.bmj.com/ ary artery, and no right pulmonary veins (Bar- connexion. In total pulmonary-systemic venous clay, 1955). connexion both veno-arterial and arterio-venous shunts are present; the prognosis is poor, most of ANOMALIES INVOLVING PULMONARY-SYSTEMIC the patients dying in infancy. In partial pulmon- VENOUS COMMUNICATION ary-systemic venous connexion there is an arterio- These necessarily involve an arterio-venous shunt venous shunt but not necessarily a veno-arterial which may or may not be associated with a veno- shunt; many patients reach adult life. Necropsies were performed at the Mayo Clinic in 135 cases; arterial shunt elsewhere. In striking contrast to on September 26, 2021 by guest. Protected anomalies which are confined to the systemic or associated malformations included mitral atresia, to the pulmonary veins alone, they cause an altera- Fallot's tetralogy, cor biloculare with subpulmonic tion in the mechanics of the circulation; they may stenosis, and atresic common pulmonary veins. profoundly change the clinical picture and the A method of demonstrating the pulmonary prognosis of coexisting cardiac malformations; drainage pathway by arterial dilution curves is and they often produce a characteristic radio- described by Swan and others (1953). The dilu- logical picture. Anomalous pulmonary venous tion pattern of an indicator (T 1824) is recorded drainage may be total or partial. In the former during its initial circulation after injection into case, all the pulmonary veins drain into the right the vena cava, pulmonary vein, right pulmonary atrium, either directly or via a systemic vein. artery, or left pulmonary artery. Differences in In partial anomalous pulmonary venous drainage, the recorded patterns will demonstrate the some pulmonary veins enter the left atrium in the presence or absence of anomalous drainage from normal manner while others enter a systemic vein abnormal venous connexions. In patients who or the right atrium. also have an atrial septal defect with a large pul- Brody (1942) found six cases of total anomalous monary blood flow, it can sometimes be shown venous drainage recorded in the literature and that the blood draining from the right lung is added a seventh of his own. By 1954 the number shunted preferentially across the defect. By this Thorax: first published as 10.1136/thx.11.2.119 on 1 June 1956. Downloaded from ANOMALOUS PULMONARY AND SYSTEMIC VENOUS DRAINAGE 121 method, Swan demonstrated partial drainage of (1953) described additional cases but furnished no the right lung into the inferior vena cava in one new information. case in which there was no other circulatory de- Gardner and Oram (1953) have published four fect. In another case cardiac catheterization with cases with a persistent left superior vena cava arterial dilution curves showed (1) union of the which was joined by pulmonary veins; they right pulmonary vein with a persistent left superior correspond to McManus's group 1 (b).
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