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Subdiaphragmatic total anomalous pulmonary venous drainage: Report of a successful surgical correction
Subdiaphragmatie
total
venous surgical
Report
drainage: correction
anomalous
pulmonary
09 a successful
H. S. Jofle, M.B., Ch.B., M.iWed.(Paed.) T. 6. O’Donovans F.R.C.S.(Edin.), F.R.A.C.S. B. P. Glaun, M.B., Ch.B., M.R.C.P., D.C.N. E. Chesler, M.D.(Rand.), M.R.C.P.(Edin.) Vs Schrire, M.Sc., Ph.D., M.D., F.R.C.P.E., F.R.C.P., Cape Town, South Africa
he results of surgery for total anomalous pulmonary venous drainage (TAPVD) have been uniformly poor” particularly in the subdiaphragmatic type.2 The latter patients invariably have obstruction to pulmonary venous return and therefore present early, deteriorate rapidly, and inevitably die unless alleviated surgically.3-7 Since the first reported case of successful correction in 1962, only 6 examples have been documented.8-13 This paper describes the case of an infant with subdiaphragmatic TAPVD in whom the diagnosis was established by angiography and successful surgical correction was achieved. Case
report
This male infant is the first child of healthy parents and the product of a normal full-term pregnancy and vertex delivery. The birth weight was 3.149 Kg. (6 lb., 1.5 oz.) and the length was 53.7 cm. Within 3 hr. after birth, he became cyanosed and developed respiratory distress. The treatment in-
F.A.C.C.
cluded oxygen (which raised the arterial ~OZ from 30 to 65 mm. Hg) and an intragastric drip of dextrose and bicarbonate. On the fourth day, he developed congestive cardiac failure for which digoxin and furosemide (Lasix) were given. Failure to improve led to his transfer to Groote Schuur Hospital on the ninth day. On admission, he was deeply cyanosed and in heart failure, with tachycardia, a respiratory rate of more than 100 per minute, peripheral edema, and a liver edge palpable 5 cm. below the costal margin. Femoral artery pulsations were markedly diminished. There was chest wall retraction, diminished breath sounds, and scattered bilateral crepitations. The heart was not clinically enlarged and there were no bruits, but a gallop rhythm was heard. A chest x-ray (Fig. 1, A) showed complete opacification of both lung fields compatible with pulmonary edema, and the cardiac outline could not be defined. For technical reasons, an electrocardiogram could not be obtained. Shortly after admission, he had episodes of apnea and intermittent positive pressure ventilation (IPPV) was initiated using a Bird Mark VIII respirator with infant circle. The arterial pH rose from 7.24 to 7.56, pCOz fell from 62 to 24.5 mm. Mg and the ~02 (on IPPV with 100 per cent 02) was
the Cardiac Clinic, Groote Schuur Hospital. Departments of Medicine, Surgery, and Child He&h, University of Cape Town and the Cardiovascular-Pulmonary Research Group. Supported in the Department of Medicine by the South African Medical Research Council and the Cape Town City Council, Cape Town, South Africa. Received for publication Jan. 19, 1970. Reprint requests to: H. S. Jo&, University of Cape Town, Cardiac Clinic, Groote Schuur Hospital, Observatory, Cape Town. South Africa. From
2.50
American Heart Journal
F&uary,
1971
Yoi.
81, No.
2, pp. 250-254
Volame
Number
81
Subdiafihragmatic
2
Fig. 1. A, Preoperative line. B, Eleven weeks and normal heart size.
straight chest after operation
x-ray showing gross pulmonary showing clearing of lung fields
Fig. 2. Pulmonary venous phase of right ventricular seen draining from pulmonary venous branches into the portal vein. Gross pulmonary edema obliterates pulmonary vein; per = portal vein.
46 mm. Hg. After 24 hours, his clinical condition was much improved and peripheral pulses were more readily palpable but of small volume. The arterial pH was 7.59, the pC0~ was 24.5 mm. Hg, and the PO2 was 60 mm. Hg. A second chest x-ray showed slight clearing of the pulmonary edema and a normalsized cardiac outline could be discerned. A diagnosis of TAPVD with obstruction was entertained, but the hypoplastic left heart syndrome could not be excluded. Investigation was performed with the infant still on IPPV. Cineangiography following injection of contrast medium into the left atrium revealed no abnormality of the left atrium, left ventricle, or aorta. Injection into the right ventricle revealed no
TAP VD
edema and blurring of the cardiac with residual congestion of right
251
outlung
cineangiogram in anteroposterior projection. Contrast is a common channel which descends to communicate with the cardiac outline and the diaphragm. @v. = Common
abnormality of this chamber or the pulmonary arteries, but the left atrium did not fill in the venous phase. After 3 sec., dye was seen to drain from the pulmonary veins into a common venous channel which descended to communicate with the portal vein, confirming the diagnosis of subdiaphragmatic TAPVD (Fig. 2). Three hours later, surgery was performed on cardiopulmonary bypass. Hemodilution (50 per cent blood, SO per cent electrolyte solution), hypothermia, and circulatory arrest were used. Following median sternotomy, gross tissue edema and lung congestion were noted and the right ventricle was hypertrophied. The anomalous pulmonary venous connection was confirmed. Bypass was instituted and the
common p~~lmonary vein was dissected while the infant was cooled to 20” 13. and the heart-lung machine was stopped. The descending vein was snared and the common pulmonary vein opened with a transverse incision one cm. in length. The posterior aspect of the left atrium was opened transversely from the atrial septum to the root of the atria1 appendage, an incision of one cm. in length. A sideto-side anastomosis was fashioned between the common pulmonary vein and the left atrium, using a continuous suture of 5-O silk. The heart-lung machine was restarted and the descending vein ligated inferiorly. Rewarming was uneventful, the heart taking over strongly. Bypass was discontinued and the chest closed with two mediastinal drainage tubes. Postoperatively, the infant was maintained on IPPV administered by endotracheal tube. He survived 2 episodes of cardiac arrest within 2 hours of operation. The lungs cleared progressively over the next 7 days. He then developed gross pulmonary effusions bilaterally orecioitated bv left uooer lobe pneumonia, Pseudonzonas'aeruginosa beingLcLultured. One week later, a second episode of pneumonia in the right upper lobe produced a massive effusion in the right chest. Pseudomonas and Klebsiella aerogenes were cultured from the tracheal aspirate. The treatment included parenteral colistin and gentamicin. He could not be weaned from the respirator until tracheostomy was performed 3 wk. postoperatively. He then improved steadily but evidence of congestion of the right lung persisted. At the age of 11 wk. and at the weight of 4.321 Kg. (9 lb., IO oz.), he was successfully extubated and discharged from the hospital in good health. A follow-up x-ray 11 wk. after the operation showed improvement (Fig. 1, B).
Discussion Total anomalous pulmonary venous drainage (TAPVD) was first described in 1798 in association w-ith other cardiac anomalies,14 and in 1868 as an uncomplicated lesion.15s16 The unusual variety in which a common pulmonary vein drains into a subdiaphragmatic site was first recorded in 1913 in association with multiple cardiac defectsI and in 1916 as an isolated entity. I8 Since then, many examples from autopsy material have been reported; Burroughs and Edwards identified 188 cases of TAPVD in the literature until 1956, of which 28 drained below the diaphragm.3 Hastreiter and co-workers4 described another 37 cases of TAPVD with pulmonary venous obstruction. Clinical recognition of TAPVD has been reported since 19.5Wg and surgical correction was first attempted in 1951.20 The embryological basis for the development of subdiaphragmatic TAPVD has
been presented by I%lwards21 and Yeill? and requires no elaboration. The uniformly fatal outcome has been ascribed to obstruction of pulmonary venuus return with consequent severe pulmonary venous congestion and right heart failure.5 Several mechanisms for the obstruction have been postulated, including the increased resistance offered by the common pulmonary vein as a result of its length or small diameither at the eter ,4 or from constriction level of the diaphragm2J3 or at the site of entry into the portal vein, ductus venosus, hepatic veins, or inferior vena cava. Drainage into the portal system may also be restricted by arborization of veins within the liver18J4~25or by persistent higher relative pressures in the portal system as found in fetal life.26*27 It seems iikely that the anatomical arrangement and degree of restriction govern the clinical presentation, death occurring at any time between 2 days and 4 months.2-6a27 Characteristically, the patient presents with dyspnea, cyanosis, and congestive cardiac failure, and the radiological features of severe pulmonary congestion with a normal-sized heart.5*28r2g Llewellyn and associatesr2 advise operation on the basis of these findings without further investigation. We consider angiocardiography to be essential before surgery, to exclude the hypoplastic left heart syndrome and to identify the site of obstruction. The case presented in this paper manifested gross pulmonary edema on the plain film, so that the cardiac outline was obscured and accurate evaluation of cardiac size was rendered impossible. In addition, depleted venous return to the left heart possibly aggravated by restricted atria1 communication, or reduced cardiac output from severe cardiac failure, resulted in poor peripheral pulses. The hypoplastic left heart syndrome therefore could not be excluded on clinical grounds. Furthermore, it is now recognized that a patient with TAPVD into a supracardiac vein may also have pulmonary venous obstruction, resulting in an identical clinical and radiological presentation.28-30 The exact anatomy should therefore be identified before surgery. The present case illustrates that limited investigation can be performed even on a
Volume
81
Nztmber
2
Subdiaphragmatic
Table I. Clinical and surgical facton corrected by surgery ~~ Investigatoivs of cases Sloan et al.* Cooley and Balasgt Woodwark et al.lo Jegier et al.ll Llewellyn et a1.12 Mody et al.‘3 Present case
Age at onset of severe symptoms
Not
7 days recorded 3 wk. 5 daysf 6 days 4 wk.5 3 hr.
in cases of subdiaphragmatic
Age at investigation 11 days 7 wk. 6 wk. 5 days Not performed 5 wk. 11 days
Age at surgery 12 7 6 6 12 5 11
days wk. wk. days days wk. days
TAPVD
TAP VD
successfully
Closure of ASD,/ PFO
Ligation DCVP
Postoperative course
Yes* Yes No No No Yes No
Yes Yes No. No Yes Yes Yes
Satisfactory Satisfactory Satisfactory Satisfactory Satisfactory Minor complication Complicated
of
ASD = Atrial septal defect; PFO = patent foramen wale; and DCPV = descending common pulmonary vein. “Also enlarged left atrium with Teflon graft. tCooley and B&s describe another successful operation in an infant of one month, who died two months later anastomosis. iTransient euisodlz of cyanosis at 6 hours, responding rapidly to oxygen administration. $Rapid rap&ions noted soon after birth.
critically ill child. The associated acid-base disturbances and ventilatory insufficiency resulting from profuse pulmonary edema must be controlled by adequate blood gas monitoring and IPPV if necessary. This service can best be provided by a neonatal intensive care unit. The surgical technique used is the same as that advised by Cooley and co-workerslBg wherebmy the common pulmonary vein is anastomoseld to the posterior wall of the left atrium and ligated distally in its caudal course. The patent foramen ovale was not closed, theoretically to provide an escape route if the left atria1 capacity were too small to cope with the increased flow. There is conflicting opinion as to the advisability of ligation of the descending vein and closure of the PFO (atria1 septal defect)‘z31 and there is as yet insufficient data for evaluation. The age at presentation and variations in technique in the 7 successfully corrected cases are outlined in Table I. The stormy postoperative course in this patient was probably due in part to the marked degree of pulmonary venous obstruction as judged by the early onset and the severity of the clinical presentation. Pulmonary edema is the most frequent complication following repair of TAPVDr and may be due to absolutes1 or relative inadequacy of left atria1 capacity,r or to an
253
from
stenosis
of the
inadequate anastomosis. The recurrence of right-sided pulmonary congestion in our case suggests residual partial obstruction of the right pulmonary vein. Summary
The seventh reported case of successful surgical correction of total anomalous pulmonary venous drainage with subdiaphragmatic obstruction is presented. Angiographic confirmation of the diagnosis and identification of the site of obstruction was obtained prior to operation. Adequate investigation in a moribund infant was facilitated by the use of intermittent positive pressure ventilation. Surgical correction was achieved by anastomosis of the common pulmonary vein to the left atrium followed by ligation of the anomalous connection to the portal system. Early investigation of these critically ill infants is advocated, since without surgical intervention, the prognosis is hopeless. We wish to thank Dr. T. Voss of the Department of Anaesthetics, Red Cross Children’s Hospital, for his assistance in postoperative care, and the Medical Superintendents of Groote Schuur and Red Cross Children’s Hospitals, respectively, for permission to publish. REFERENCES 1.
Cooley, R. D.: drainage.
D. A., Hallman, G. L., and Leachman, Total anomalous pulmonary venous Correction with the use of cardio-
2.
3.
4.
5
6.
7.
8.
9.
10.
11.
12.
13.
14.
1.5.
pulmonary bypass in 62 cases, J. Thorac. Cardiovasc. Surg. 51~88, 1966. Mustard, W. T., Trusler, 6. A., 2nd McLennan, T. A.: Tot21 anomalous pulmonary venous drainage in the first six months of life, & Cassels, Donald E., editor: Heart and circulation in the newborn and infant, New York, 1966, Grune & Stratton, Inc., p. 339. Burroughs, Jo T., and Edwards, J. E.: Total anomalous pulmonary venous connection, AZIER. HEART J. 59:913, 1960. Hastreiter, A. R., Paul, M. H., Molthan, M. E., and Miller, R. A.: Total anomalous pulmonary venous connection with severe pulmonary venous obstruction, Circulation 25:916, 196i. Johnson, A. L., Wiglesworth, F. W., Dunbar, J. S., Siddoo, S., and Gr2jo, M.: Infradiaphragmatic total anomalous pulmonary venous connection, Circulation 17/31-O, 19.58. Lucas. R. V.. Tr.. Adams. P.. Tr.. Anderson. R. C.,‘Varco, ‘R: Ll, Edwards, J. E.,‘and Lester; R. G. : Total anomalous pulmonary venous connection to the port21 venous system: a cause of pulmonary venous obstruction, Amer. J, Roentgen. 86:561, 1961. Snellen, H. A., and Bruins, C.: Anomalies of venous return, in Watson, Hamish, editor: Paediatric cardiology, London, 1968, LloydLuke (Medical Books) Ltd., pp. 417-436. Sloan, H., MacKenzie, J., Morris, J. D., Stern, A., and Sigmann, J.: Open heart surgery in infancy, J. Thorac. Cardiovasc. Surg. 44:4.59, 1962. Cooley, D. A., and Balas, P. E.: Total anomaious pulmonary venous drainage into inferior vena cava: report of successful surgical correction, Surgery 51:798, 1962. Woodwark. 6. M.. Vince. D. 1.. and Ashmore. P. G.: Total 2nbm2lous pulmonary venous return to port21 vein, J. Thorac. Cardiovasc. Surg. 45:662, 1963. Jegier, W., Charrette, E., and Dobell, A. R. C.: Infradiaphragmatic anomalous pulmonary venous drainage: normal haemodynamics following operation in infancy, Circulation 35:396, 1967. Llewellyn, M. A., Cullum, P. A., Thomas, J. B., and Anderson, I. M.: Infracardiac total anomalous pulmonary venous drainage, Brit. Med. J. 3:35, 1968. Mody, M. R., Gallen, W. J., and Lepley, I).: Total anomalous pulmonary venous drainage below the diaphragm: successful surgical correction in an infant, Amer. J. Cardiol. 24:57.5, 1969. Wilson, J.: A description of 2 very unusual formation of the human heart, Phil.- Tr. Roy. Sot. London 88:346. 1798. Friedlowsky, A.: Vrtljschr. Prak. Heilk. 25:45, 1868. Cited in reference 16.
16.
17.
18.
19.
20.
21.
22. 23.
24.
25.
26.
27.
28.
29.
30.
31.
Brody, H.: Drainage of the pulmonic veins into the right side of the heart, Arch. Path. (Chicago) x%:221, 1942. Rivet, L., and Girard, L.: IJn cas de malformation cardiaque avec anomalies multiples de l’appareill circulatoire, Arch. Mal. Coeur 6~720, 1913. Ghon, A.: Ein beitrag zu den anomalien der pulmonalvenen, Beitr. Path. Anat. 2:175, 1916. Friedlich, A., Bing, R. J., 2nd Blount, S. G., Jr.: Physiological studies in congenital heart disease, IX. Circulatory dynamics in the anomalies of venous return to the heart, including pulmonary arterio-venous fistula, Bull. Hopkins Hosp. 86:20, 1950. Muller, W. II.: The surgical treatment of transposition of the puimonary veins, Ann. Surg. 134~683, 1951. Edwards, J. E.: Pathologic and developmental considerations in anomalous pulmonary venous connection, Proc. Mayo Ciin. 28:441, 1953. Neill, C. A.: Development of the pulmonary veins, Pediatrics 18:880, 1956. Russell, V. L., Jr., Adams, P., Jr., Anderson, R. C., Varco, R. L., and Lester, R. G.: Total anomalous pulmonary venous connection to the portal venous system, Amer. J. Roentgen. 86:436, 1960. Gott, V. L., Lester, R. G., Lillehei, C. W., and Varco, R. L.: Total anomalous pulmonary venous return: An analysis of 30 cases, Circulation 13:543, 1956. Gunteroth, W. G., Nadas, A. S., 2nd Gross, R. E.: Transposition of the pulmonary veins, Circulation 18~117, 19.58. Sherman, F. E., and Bauersfield, S. R.: Total uncomplicated anomalous pulmonary venous connection. Pediatrics 25:656. 1960. Levin, B.,’ 2nd White, H.: ‘Total anomalous pulmonary venous drainage into the portal system, Radiology 16:894, 1961. Harris, G. B. C., Neuhauser, E. B. D., and Giedion, A. : Total anomalous pulmonary venous return below the diaphragm, Amer. J. Roentgen. 84:436, 1960. Eisen, S., and Elliott, L. P.: A plain film sign of tot21 anomalous pulmonary venous connection below the diaphragm, Amer. J. Roentgen. 102:372, 1968. Kauffman, S. L., Ores, C. IN., and Andersen, D. II.: Two cases of total anomalous pulmonary venous return of supracardiac type _ with stenosis simulating infradiaphragmatic drainage, Circulation 25:376, 1962. Mustard, W. T., Keith, J. D., 2nd Trusler, G.: Two stage correction for total anomalous pulmonary venous drainage in childhood, J. Thorat. Cardiovasc. Surg. 44:477, 1962.
total
venous surgical
Report
drainage: correction
anomalous
pulmonary
09 a successful
H. S. Jofle, M.B., Ch.B., M.iWed.(Paed.) T. 6. O’Donovans F.R.C.S.(Edin.), F.R.A.C.S. B. P. Glaun, M.B., Ch.B., M.R.C.P., D.C.N. E. Chesler, M.D.(Rand.), M.R.C.P.(Edin.) Vs Schrire, M.Sc., Ph.D., M.D., F.R.C.P.E., F.R.C.P., Cape Town, South Africa
he results of surgery for total anomalous pulmonary venous drainage (TAPVD) have been uniformly poor” particularly in the subdiaphragmatic type.2 The latter patients invariably have obstruction to pulmonary venous return and therefore present early, deteriorate rapidly, and inevitably die unless alleviated surgically.3-7 Since the first reported case of successful correction in 1962, only 6 examples have been documented.8-13 This paper describes the case of an infant with subdiaphragmatic TAPVD in whom the diagnosis was established by angiography and successful surgical correction was achieved. Case
report
This male infant is the first child of healthy parents and the product of a normal full-term pregnancy and vertex delivery. The birth weight was 3.149 Kg. (6 lb., 1.5 oz.) and the length was 53.7 cm. Within 3 hr. after birth, he became cyanosed and developed respiratory distress. The treatment in-
F.A.C.C.
cluded oxygen (which raised the arterial ~OZ from 30 to 65 mm. Hg) and an intragastric drip of dextrose and bicarbonate. On the fourth day, he developed congestive cardiac failure for which digoxin and furosemide (Lasix) were given. Failure to improve led to his transfer to Groote Schuur Hospital on the ninth day. On admission, he was deeply cyanosed and in heart failure, with tachycardia, a respiratory rate of more than 100 per minute, peripheral edema, and a liver edge palpable 5 cm. below the costal margin. Femoral artery pulsations were markedly diminished. There was chest wall retraction, diminished breath sounds, and scattered bilateral crepitations. The heart was not clinically enlarged and there were no bruits, but a gallop rhythm was heard. A chest x-ray (Fig. 1, A) showed complete opacification of both lung fields compatible with pulmonary edema, and the cardiac outline could not be defined. For technical reasons, an electrocardiogram could not be obtained. Shortly after admission, he had episodes of apnea and intermittent positive pressure ventilation (IPPV) was initiated using a Bird Mark VIII respirator with infant circle. The arterial pH rose from 7.24 to 7.56, pCOz fell from 62 to 24.5 mm. Mg and the ~02 (on IPPV with 100 per cent 02) was
the Cardiac Clinic, Groote Schuur Hospital. Departments of Medicine, Surgery, and Child He&h, University of Cape Town and the Cardiovascular-Pulmonary Research Group. Supported in the Department of Medicine by the South African Medical Research Council and the Cape Town City Council, Cape Town, South Africa. Received for publication Jan. 19, 1970. Reprint requests to: H. S. Jo&, University of Cape Town, Cardiac Clinic, Groote Schuur Hospital, Observatory, Cape Town. South Africa. From
2.50
American Heart Journal
F&uary,
1971
Yoi.
81, No.
2, pp. 250-254
Volame
Number
81
Subdiafihragmatic
2
Fig. 1. A, Preoperative line. B, Eleven weeks and normal heart size.
straight chest after operation
x-ray showing gross pulmonary showing clearing of lung fields
Fig. 2. Pulmonary venous phase of right ventricular seen draining from pulmonary venous branches into the portal vein. Gross pulmonary edema obliterates pulmonary vein; per = portal vein.
46 mm. Hg. After 24 hours, his clinical condition was much improved and peripheral pulses were more readily palpable but of small volume. The arterial pH was 7.59, the pC0~ was 24.5 mm. Hg, and the PO2 was 60 mm. Hg. A second chest x-ray showed slight clearing of the pulmonary edema and a normalsized cardiac outline could be discerned. A diagnosis of TAPVD with obstruction was entertained, but the hypoplastic left heart syndrome could not be excluded. Investigation was performed with the infant still on IPPV. Cineangiography following injection of contrast medium into the left atrium revealed no abnormality of the left atrium, left ventricle, or aorta. Injection into the right ventricle revealed no
TAP VD
edema and blurring of the cardiac with residual congestion of right
251
outlung
cineangiogram in anteroposterior projection. Contrast is a common channel which descends to communicate with the cardiac outline and the diaphragm. @v. = Common
abnormality of this chamber or the pulmonary arteries, but the left atrium did not fill in the venous phase. After 3 sec., dye was seen to drain from the pulmonary veins into a common venous channel which descended to communicate with the portal vein, confirming the diagnosis of subdiaphragmatic TAPVD (Fig. 2). Three hours later, surgery was performed on cardiopulmonary bypass. Hemodilution (50 per cent blood, SO per cent electrolyte solution), hypothermia, and circulatory arrest were used. Following median sternotomy, gross tissue edema and lung congestion were noted and the right ventricle was hypertrophied. The anomalous pulmonary venous connection was confirmed. Bypass was instituted and the
common p~~lmonary vein was dissected while the infant was cooled to 20” 13. and the heart-lung machine was stopped. The descending vein was snared and the common pulmonary vein opened with a transverse incision one cm. in length. The posterior aspect of the left atrium was opened transversely from the atrial septum to the root of the atria1 appendage, an incision of one cm. in length. A sideto-side anastomosis was fashioned between the common pulmonary vein and the left atrium, using a continuous suture of 5-O silk. The heart-lung machine was restarted and the descending vein ligated inferiorly. Rewarming was uneventful, the heart taking over strongly. Bypass was discontinued and the chest closed with two mediastinal drainage tubes. Postoperatively, the infant was maintained on IPPV administered by endotracheal tube. He survived 2 episodes of cardiac arrest within 2 hours of operation. The lungs cleared progressively over the next 7 days. He then developed gross pulmonary effusions bilaterally orecioitated bv left uooer lobe pneumonia, Pseudonzonas'aeruginosa beingLcLultured. One week later, a second episode of pneumonia in the right upper lobe produced a massive effusion in the right chest. Pseudomonas and Klebsiella aerogenes were cultured from the tracheal aspirate. The treatment included parenteral colistin and gentamicin. He could not be weaned from the respirator until tracheostomy was performed 3 wk. postoperatively. He then improved steadily but evidence of congestion of the right lung persisted. At the age of 11 wk. and at the weight of 4.321 Kg. (9 lb., IO oz.), he was successfully extubated and discharged from the hospital in good health. A follow-up x-ray 11 wk. after the operation showed improvement (Fig. 1, B).
Discussion Total anomalous pulmonary venous drainage (TAPVD) was first described in 1798 in association w-ith other cardiac anomalies,14 and in 1868 as an uncomplicated lesion.15s16 The unusual variety in which a common pulmonary vein drains into a subdiaphragmatic site was first recorded in 1913 in association with multiple cardiac defectsI and in 1916 as an isolated entity. I8 Since then, many examples from autopsy material have been reported; Burroughs and Edwards identified 188 cases of TAPVD in the literature until 1956, of which 28 drained below the diaphragm.3 Hastreiter and co-workers4 described another 37 cases of TAPVD with pulmonary venous obstruction. Clinical recognition of TAPVD has been reported since 19.5Wg and surgical correction was first attempted in 1951.20 The embryological basis for the development of subdiaphragmatic TAPVD has
been presented by I%lwards21 and Yeill? and requires no elaboration. The uniformly fatal outcome has been ascribed to obstruction of pulmonary venuus return with consequent severe pulmonary venous congestion and right heart failure.5 Several mechanisms for the obstruction have been postulated, including the increased resistance offered by the common pulmonary vein as a result of its length or small diameither at the eter ,4 or from constriction level of the diaphragm2J3 or at the site of entry into the portal vein, ductus venosus, hepatic veins, or inferior vena cava. Drainage into the portal system may also be restricted by arborization of veins within the liver18J4~25or by persistent higher relative pressures in the portal system as found in fetal life.26*27 It seems iikely that the anatomical arrangement and degree of restriction govern the clinical presentation, death occurring at any time between 2 days and 4 months.2-6a27 Characteristically, the patient presents with dyspnea, cyanosis, and congestive cardiac failure, and the radiological features of severe pulmonary congestion with a normal-sized heart.5*28r2g Llewellyn and associatesr2 advise operation on the basis of these findings without further investigation. We consider angiocardiography to be essential before surgery, to exclude the hypoplastic left heart syndrome and to identify the site of obstruction. The case presented in this paper manifested gross pulmonary edema on the plain film, so that the cardiac outline was obscured and accurate evaluation of cardiac size was rendered impossible. In addition, depleted venous return to the left heart possibly aggravated by restricted atria1 communication, or reduced cardiac output from severe cardiac failure, resulted in poor peripheral pulses. The hypoplastic left heart syndrome therefore could not be excluded on clinical grounds. Furthermore, it is now recognized that a patient with TAPVD into a supracardiac vein may also have pulmonary venous obstruction, resulting in an identical clinical and radiological presentation.28-30 The exact anatomy should therefore be identified before surgery. The present case illustrates that limited investigation can be performed even on a
Volume
81
Nztmber
2
Subdiaphragmatic
Table I. Clinical and surgical facton corrected by surgery ~~ Investigatoivs of cases Sloan et al.* Cooley and Balasgt Woodwark et al.lo Jegier et al.ll Llewellyn et a1.12 Mody et al.‘3 Present case
Age at onset of severe symptoms
Not
7 days recorded 3 wk. 5 daysf 6 days 4 wk.5 3 hr.
in cases of subdiaphragmatic
Age at investigation 11 days 7 wk. 6 wk. 5 days Not performed 5 wk. 11 days
Age at surgery 12 7 6 6 12 5 11
days wk. wk. days days wk. days
TAPVD
TAP VD
successfully
Closure of ASD,/ PFO
Ligation DCVP
Postoperative course
Yes* Yes No No No Yes No
Yes Yes No. No Yes Yes Yes
Satisfactory Satisfactory Satisfactory Satisfactory Satisfactory Minor complication Complicated
of
ASD = Atrial septal defect; PFO = patent foramen wale; and DCPV = descending common pulmonary vein. “Also enlarged left atrium with Teflon graft. tCooley and B&s describe another successful operation in an infant of one month, who died two months later anastomosis. iTransient euisodlz of cyanosis at 6 hours, responding rapidly to oxygen administration. $Rapid rap&ions noted soon after birth.
critically ill child. The associated acid-base disturbances and ventilatory insufficiency resulting from profuse pulmonary edema must be controlled by adequate blood gas monitoring and IPPV if necessary. This service can best be provided by a neonatal intensive care unit. The surgical technique used is the same as that advised by Cooley and co-workerslBg wherebmy the common pulmonary vein is anastomoseld to the posterior wall of the left atrium and ligated distally in its caudal course. The patent foramen ovale was not closed, theoretically to provide an escape route if the left atria1 capacity were too small to cope with the increased flow. There is conflicting opinion as to the advisability of ligation of the descending vein and closure of the PFO (atria1 septal defect)‘z31 and there is as yet insufficient data for evaluation. The age at presentation and variations in technique in the 7 successfully corrected cases are outlined in Table I. The stormy postoperative course in this patient was probably due in part to the marked degree of pulmonary venous obstruction as judged by the early onset and the severity of the clinical presentation. Pulmonary edema is the most frequent complication following repair of TAPVDr and may be due to absolutes1 or relative inadequacy of left atria1 capacity,r or to an
253
from
stenosis
of the
inadequate anastomosis. The recurrence of right-sided pulmonary congestion in our case suggests residual partial obstruction of the right pulmonary vein. Summary
The seventh reported case of successful surgical correction of total anomalous pulmonary venous drainage with subdiaphragmatic obstruction is presented. Angiographic confirmation of the diagnosis and identification of the site of obstruction was obtained prior to operation. Adequate investigation in a moribund infant was facilitated by the use of intermittent positive pressure ventilation. Surgical correction was achieved by anastomosis of the common pulmonary vein to the left atrium followed by ligation of the anomalous connection to the portal system. Early investigation of these critically ill infants is advocated, since without surgical intervention, the prognosis is hopeless. We wish to thank Dr. T. Voss of the Department of Anaesthetics, Red Cross Children’s Hospital, for his assistance in postoperative care, and the Medical Superintendents of Groote Schuur and Red Cross Children’s Hospitals, respectively, for permission to publish. REFERENCES 1.
Cooley, R. D.: drainage.
D. A., Hallman, G. L., and Leachman, Total anomalous pulmonary venous Correction with the use of cardio-
2.
3.
4.
5
6.
7.
8.
9.
10.
11.
12.
13.
14.
1.5.
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