Left-to-right shunt at atrial level due to anomalous venous connection of left lung

J THoRAc CARDIOVASC

SURG

84:626-630, 1982

Left-to-right shunt at atrial level due to anomalous venous connection of left lung Report of seven cases Our experience with seven patients with partial anomalous pulmonary venous connection (PAPVC) of the left lung, treated between 1955 and 1980. is reviewed. The developmental aspects and the hemodynamic effects of the anomaly are discussed. and the patterns of the abnormal venous connection in our patients are described. All the patients except one had an additional atrial septal defect (ASD). Treatment consisted in closure of the ASD and creation of a large anastomosis between the detached or ligated anomalous connecting vein and the left atrium. preferably the left auricular appendage. No operative or late deaths occurred; and except for atrial flutter. which lasted a few days in one case. there were no serious complications. The indication for repair. the problems of the anastomosis. and the points of postoperative attention are discussed.

Andreas Bauer, M.D., Rainer Korfer, M.D., and Wolfgang Bircks, M.D.,* Dusseldorf, Federal Republic of Germany

Anomalous connnections between the pulmonary veins and the right atrium or the systemic veins are assumed to be due to a persistence of connections between the primitive pulmonary vascular plexus or the splanchnic plexus and the tributaries of the cardinal venous system in embryonic life. The result is a failure in the connection of the primary lung veins to endothelial outgrowths from the dorsal wall of the sinus venosus, the outgrowths being the precursor of the common pulmonary vein, which is later absorbed into the wall of the left atrium. Another possibility is that this precursor is never developed at all. The developmental anatomy has been described in previous publications, 1-5 in detail by Edwards" and by Neill. 7 The partial forms of this congenital cardiovascular anomaly are frequently accompanied by other cardiac defects, such as atrial septal defect or patent ductus arteriosus, but survival for the total form requires that a From the Department Surgery B (Thoracic and Cardiovascular Surgery). University of Dusseldorf, Federal Republic of Germany Received for publication Oct. 6. 1981.

communication exist between the right and the left sides of the heart. Anomalous venous drainage of the left lung may involve the entire lung (Fig. 1) or, more commonly, only part of it, mainly the upper lobe. Hardly ever is the abnormal drainage directly into the right atrium but, more often, through a persistent left superior vena cava (S VC) into the left innominate vein; another possibility of abnormal connection is directed to the left subclavian vein or to the left innominate vein. A junction with the coronary sinus or the inferior vena cava is a rarity. In all types of anomalous pulmonary venous connection (APVC), especially in the total form, a strain on the right side of the heart occurs because of volume overload, which may, in time, cause a raised pulmonary artery pressure. The diagnosis of an associated atrial septal defect (ASD) is difficult by hemodynamic study alone. Therefore, the injection of contrast medium into the pulmonary artery is essential for demonstrating all aspects of the anomaly; contrast echocardiography is another practical method. 8

Accepted for publication Jan. 13. 1982. Address for reprints: Andreas Bauer, M.D., Medizinische Einrichtungen der Universitat Diisseldorf, Moorenstra,Be 5. D-4000 Diisseldorf, Federal Republic of Germany. *Head and Director, Department of Surgery B.

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0022-5223/82/100626+05$00.50/0 © 1982 The C. V. Mosby Co.

Among 2,050 patients with shunt at the atrial level seen at the Department Surgery B, University of Dusseldorf, between 1955 and 1980, there were 353 with

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Table I. Distribution of isolated left-to-right shunts at the atrial level (1955-1980) , Department Surgery B, University of Dusseldorf No ofpatienis c

Atrial septal defects Anomalous pulmonary venous connection APVC of the right lung APVC of the left lung Total APVC

2,050 353 310 7 36

APVC (Table I) . As often reported, partial anomalous pulmonary venous connection (PAPVC) involving the veins of the left lung is quite rare. In the period under discussion, seven patients with this anomaly have been treated at our hospital; five of them have already been mentioned in an earlier report. * The first publication from the University of Dusseldorf relating to this subject dates from 1957 . 9 The incidence in comparison to all cases of atrial shunt is about 0.34%, and to all cases ofPAPVC, about 2.2% . Because of this rarity, reports of PAPVC of the left lung usually are composed of a few sporadic cases,2-5, 10-23 apart from the first collection of cases from the literature by Brody .24 Four of our patients were female and three male ; the youngest was 6 years old and the oldest, 52. Symptoms and signs One patient had no complaints, one had repeated respiratory infections , one experienced vertigo after exertion and had collapsed repeatedly, three had noted tiredness and dyspnea after effort for the past several years, and one woman had had progression of dyspnea after activities for the past 15 years. The last-mentioned woman finally was admitted to the hospital with congestive right heart failure, edema, cyanosis, and pulmonary congestion; the liver was palpable 4 em below the right costal border. Two patients had cyanosis (according to the increase of pulmonary artery pressure to 46/14 and 50/10 mm Hg). Except for the woman with congestive heart failure, none of the patients had a palpable liver, edema, a visible jugular venous pulse, or audible abnormalities on auscultation of the lungs. In all patients a systolic ejection murmur was audible along the left sternal border with a maximum impulse from the second to the fourth intercostal space. An accentuated and more or less widely split second heart *Bircks w, Korfer R, Krian A, Schulte HD: Anomalous pulmonary venous connections. Presented at the annual meeting of the Scandinavian Association for Thoracic and Cardiovascular Surgery, Aarhus, Denmark, Oct. 19-21, 1977.

Fig. 1. Entire left lung draining into a persistent left superior vena cava. (From Krian A, Rumpf P, Bircks W: Anomalien auf Vorhofebene, Allgemeine und spezielle Operationslehre, Band VIITeil2, M Kirschner, R Zenker, G Heberer, G Hegemann, eds. , Berlin-Heidelberg-New York, 1978, SpringerVerlag, p 158 .) sound also could be heard. There was no thrill. Bulging of the heart was noted in one patient.

Examination The electrocardiogram revealed sinus rhythm, right axis deviation, and right ventricular hypertrophy in all patients. An incomplete right bundle branch block was found in six patients and a complete block in one patient. Roentgenograms of the che st showed a slight enlargement of the heart and prominent pulmonic vessels. A vascular shadow along the left upper mediastinum was seen in two patients . Angiocardiography with injection of contrast medium into the pulmonary artery disclosed the anomaly (Fig . 2). Previously, cardiac catheterization had revealed a significant increase in the oxygen saturation at the site of entry of the connecting veins. Four patients had a moderate elevation of the right ventricular systolic pressure, and two of them had elevation of the pulmonary artery pressure, as well .

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Fig. 2. Angiocardiogram, with injection of contrast medium into the pulmonary artery, showsthe upperlobe veindraining into a left superior vena cava.

Anatomic findings and surgical treatment Exposure in all patients except one (bilateral thoracotomy) was through a longitudinal sternotomy and pericardiotomy. All operations were performed with the help of cardiopulmonary bypass and moderate hypothermia. There were no defects of the tricuspid or pulmonary valves, but in one case there was a mild infundibular stenosis of the right ventricle which did not necessitate surgical correction. The right atrium, right ventricle, and pulmonary artery were enlarged. All patients except one had an additional secundum ASD, which was closed with a running suture in five of them. In three patients, the entire left lung drained by a common venous trunk through a persistent left SVC into the left innominate vein. In two patients the upper lobe vein drained through a persistent left SVC into the left innominate vein and, in one instance, directly into the left innominate vein. One patient had a connection between the upper lobe vein and the right atrium, in this case the additional secundum ASD was closed by a prosthetic patch with deviation of the anomalous venous return to the left atrium. Surgical correction in the other patients was done as follows: In one case a side-to-side anastomosis was constructed between an incision in the left atrium from the auricular appendage to the mitral valve and an incision in the common venous trunk of the entire left lung. In two cases the anastomosis was between the disconnected or distal ligated common venous trunk and the

Fig. 3. Surgical procedure (detached or ligated persistent left superior vena cava), showing end-to-side anastomosis or side-to-side anastomosis (insert). (From Krian A, Rumpf P, Bircks W: Anomalien auf Vorhofebene, Allgemeine und spezielle Operationslehre, Band VI/Teil 2, M Kirschner, R Zenker, G Heberer, G Hegemann, eds., Berlin-HeidelbergNew York, 1978, Springer- Verlag, p 158.) left auricular appendage. In two other cases, an endto-end anastomosis was made between the detached upper lobe vein, the detached left SVC, and the left auricular appendage. Finally, in one case, an end-toside anastomosis was constructed between the detached left SVC and the left atrium next to the entry of the lower lobe vein. The intraoperative pressure data after repair were normalized. The surgical procedure is shown in Fig. 3. All of the patients had uneventful postoperative courses except one, who had atrial flutter for a few days. At present, 1, 6, 7, 11, 12, 13, and 15 years after repair, all patients are well and can work in their professions. Recatheterization was not necessary. The patient with congestive right heart failure, however, requires medication.

Comment Symptoms and signs in patients with PAPVC of the left lung were similar to those of patients with an ASD and left-to-right shunt. The electrocardiographic find-

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Table II. Review of reported cases of surgical treatment since 1953 Ref. No.

Cases

26

I I I I I I I I I I

10

16 2

3 5 11 12 23 22 14 17 19 18

4 I I I I

2 I

Anomalous connection

Repair

Entire left lung Detached venous trunk to left auricular appendage Entire left lung Ligated vessel to left auricular appendage Entire left lung No statement Entire left lung Vein to left auricular appendage Entire left lung plus mitral stenosis Vein to auricular appendage plus mitral commissurotomy Entire left lung Detached vein to left atrium Entire left lung to coronary sinus Deviation by closing an additional ASD Entire hypoplastic left lung into IVe Left lung removed Entire left lung Vein to left atrium Upper lobe and lingual vein Vertical vein to left atrium Entire left lung No statement Left upper lobe Vein to auricular appendage Left upper lobe plus patent ductus Vein to auricular appendage Left upper lobe Vein to posterior left atrium Entire left lung Vein to auricular appendage Entire left lung Detached trunk to left auricular appendage Entire left lung Detached trunk to left auricular appendage

Follow-up No No Died of pulmonary edema No Died No One year, good No No No One died postop. Eight months, good Two months, good No One year, good No Two years, good

Legend: IVe, Inferior vena cava. ASD, Atrial septal defect.

ings signaled right ventricular overload. In the frontal roentgenogram of the chest, a vascular shadow in the left upper part of the mediastinum, forming the wellknown "figure of eight," could be seen in only two cases. Heart catheterization and angiocardiography disclosed the anomaly. Six patients had an additional ASD. Surgical treatment of this malformation has become safe. In 1947, Brantigan'" suggested the removal of the portion of the lung that was anomalously connected in order to abolish the additional volume load on the right heart and the pulmonary circulation. In 1951, Muller" described two cases in which a side-to-side anastomosis between the left atrium and the anomalous veins was performed. The concomitant ASD was left open, and the veins were not ligated. In 1953, Kirklin'": 27 mobilized the entire venous trunk, which emptied into a persistent SVC, separated the trunk from the innominate vein, and implanted it into the left auricular appendage: This was the first case of a complete correction. With the development of extracorporeal circulation and improvement in anesthesia, the actual risk of correction is now minimal. The danger from the natural history of the anomaly exceeds the risk of correction. The indication for transplanting the anomalous vessels depends on two facts: (1) an increased pulmonary flow, which may damage the pulmonary vessels and the right heart, and (2) diseases of the lungs, which will become dangerous. Pulmonary hypertension is not a contraindication, since repair will decrease the right ventricular overload. Our experience with surgical correction is good, although a review of the literature since the first correction reveals that two of 21 patients died postoperatively (Table II).

A large anastomosis is important for surgical correction, because small vein-to-auricular appendage or vein-to-atrium shunts quickly become fibrosed and shut off in the low-pressure system. In four of our patients, a large side-to-side or end-to-side anastomosis was carried out. Postoperative attention has to be paid to arrhythmias such as atrial flutter or fibrillation and to pulmonary complications such as atelectasis.

2

3

4 5 6

7 8

9

REFERENCES Darling RC, Rothney WB. Craig JM: Total pulmonary venous drainage into the right side of the heart. Lab Invest 6:44-64, 1957 Gilman RA, Skowron CAR, Musser BG, Bailey CP: Partial anomalous venous drainage. Am J Surg 94:688-694, 1957 Risch F, Halm C: The technique of surgical correction of anomalies of the pulmonary veins in a series of 25 cases. Thorax 13:251-260, 1958 Sepulveda G, Lukas DS, Steinberg J: Anomalous drainage of pulmonary veins. Am J Med 18:883-899, 1955 Swan H, Baer SB: Anomalous pulmonary venous drainage. Arch Surg 77:900-902, 1958 Edwards EJE: Pathologic and developmental considerations in anomalous pulmonary venous connection. Mayo Clin Proc 28:441-452, 1953 Neill CA: Development of the pulmonary veins. Pediatrics 18:880-887, 1956 Danilowicz D, Kronzon J: Use of contrast echocardiography in the diagnosis of partial anomalous pulmonary venous connection. Am J Cardiol 43:248-252, 1979 Derra E, Irrner W, Kremer K, Lohr B: Weitere Entwicklungen in der Technik der Chirurgie des Herzens und seiner gro,Ben Gefa,Be. Chirurgische Operationslehre, Band III, Beitrag IE, B Breitner, ed., Munchen-Berlin-

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Wien, 1957, Urban und Schwarzenberg, pp 191-212 10 Cooley DA, Mahaffey DE: Anomalous pulmonary venous drainage of the entire left lung. Report of case with surgical correction. Ann Surg 142:986-991, 1955 11 D'Cruz JA, Arcilla RA: Anomalous venous drainage of the left lung into the inferior vena cava. Am Heart J 67: 539-544, 1964 12 Frye RL, Krebs M, Rahimtoola SH, Ongley PA, Hallermann FL, Wallace RB: Partial anomalous pulmonary venous connection without atrial septal defect. Am J Cardiol 22:242-250, 1968 13 Geraci JE, Kirklin JW: Transplantation of left anomalous pulmonary vein to left atrium. Mayo Clin Proc 28:472475, 1953 14 Ghassemi AR, Thomson NB: Repair of atrial septal defect with partial anomalous pulmonary venous return from the left upper lobe. Ann Thorac Surg 23:73-76, 1977 15 Helmer F, Wolner E, Domanig E: The clinical features and therapy of anomalous pulmonary venous return. Wein Klin Wochenschr 84:424-428, 1972 16 Hickie JB, Gimlette TMD, Bacon APe: Anomalous pulmonary venous drainage. Br Heart J 18:365-377, 1956 17 Lichey C: Isolierte Transposition der gesamten linken Lungenvenen in eine endverschlossene linkspersistierende obere Hohlvene. Zentralbl Chir 102:52-55, 1977 18 Lauridsen P, Wennevold A, Efsen F: Anomalous pulmonary venous drainage from the left lung to the right atrium. Scand J Thorac Cardiovasc Surg 13:·17-20, 1979 19 Miller SW, Dinsmore RE, Liberthson RR, Goldblatt A, Daggett WM, Buckley MJ: Anomalous pulmonary venous connection of entire left lung with intact atrial septum. Radiology 122:591-595, 1977 20 Muller WH: The surgical treatment of transposition of the pulmonary veins. Ann Surg 134:683-693, 1951

Thoracic and Cardiovascular Surgery

21 Nieveen J, DeVries H, ten Hoor F: Complete anomalous drainage of left pulmonary veins. Acta Med Scand 172: 137-141, 1962 22 Oropeza G, Hernandez FA, Callard GM, Jude JR: Anomalous pulmonary venous drainage of the left upper lobe. Ann Thorac Surg 9: 180-185, 1970 23 Snellen HA, vim Ingen HC, Hoefsmit CM: Patterns of anomalous pulmonary venous drainage. Circulation 38: 45-63, 1968 24 Brody H: Drainage of the pulmonary veins into the right side of the heart. Arch Pathol Lab Med 33:221-240, 1942 25 Brantigan OC: Anomalies of the pulmonary veins. Surg Gynecol Obstet 84:653-658, 1947 26 Kirklin JW: Surgical treatment of anomalous pulmonary venous connections. Mayo Clin Proc 28:476-479, 1953 27 Kirklin JW, Ellis F, Wood EH: Treatment of anomalous pulmonary venous connections in association with interatrial communications. Surgery 39:389-398, 1956 28 Crafoord C, Bjork VO: Surgical treatment of atrial septal defects, Encyclopedia of Thoracic Surgery, E Derra, ed., Berlin-Gottingen-Heidelberg, 1959, Springer-Verlag, p 306 29 Derra E, Loogen F, Rotthoff F: Der Vorhofseptumdefekt mit Lungenvenentransposition und seine operative Beseitigung. Wien Med Wochenschr 109: 1-5, 1959 30 Samaan HA: Surgical correction of anomalous pulmonary venous drainage. J Cardiovasc Surg (Torino) 14:65-75, 1973 31 Seling A: Die Vorhofseptumdefekte. Handbuch der Thorexchirurgie, Erganzungswerk, Herzchirurgie I, E Derra, W Bircks, eds., Berlin-Heidelberg-New York, 1976, Springer Verlag, p 525