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Surgical Management of Infants with Complex Cardiac Anomalies Associated with Reduced Pulmonary Blood Flow and Total Anomalous Pulmonary Venous Drainage
Surgical Management of Infants with Complex Cardiac Anomalies Associated with Reduced Pulmonarv Blood Flow and Total Anomalous Pulmonary Venous Draihage Serafin Y. DeLeon, M.D., Samuel S. Gidding, M.D., Michel N. Ilbawi, M.D., Farouk S. Idriss, M.D., Alexander J. Muster, M.D., Roger B. Cole, M.D., and Milton H. Paul, M.D. ABSTRACT Eight infants with complex cardiac anomalies and pulmonary stenosis or atresia were noted to have obstructed total anomalous pulmonary venous drainage (TAPVD) either at the initial cardiac catheterization (Group 1; n = 2) or after creation of systemic-pulmonary artery shunts (Group 2; n = 6 ) . The 2 patients in Group 1 underwent early repair of TAPVD (1 at 7 days, the other at 1% months of age) before any subsequent operation and are now doing well at IS months of age. The 6 patients in Group 2 underwent repeat cardiac catheterization because of persistent severe cyanosis with faint or absent continuous murmur and were found to have patent shunts and obstructed TAPVD (1 mild, 5 severe). One patient who underwent repair of TAPVD at 2% months of age survived and is well at 2 years of age, whereas 4 patients who underwent repair at an average age of 6 months (age range, 3-16 months) subsequently died. The sixth patient, who did not undergo repair, remained severely cyanotic with hypoplastic pulmonary arteries in spite of repeated shunts. We feel that increased awareness of the possible association of TAPVD and reduced pulmonary blood flow in infants with complex cardiac defects, in combination with echocardiography, oxygen saturation studies, and angiography with prostaglandin El challenge, should lead to early diagnosis, avoidance of unnecessary systemic-pulmonary artery shunts, and increased survival rates in these infants. The surgical management of isolated total anomalous pulmonary venous drainage (TAPVD) is well established [l-31. Success has been limited, however, in the presence of other hemodynamically marked cardiac lesions. Management becomes particularly difficult and the results less satisfactory when the associated defects cause decreased pulmonary blood flow [4, 51. Angiographic visualization of the pulmonary veins, especially when obstructed, becomes difficult, leading to missed or delayed diagnosis. The association of TAPVD and reFrom the Divisions of Cardiovascular-Thoracic Surgery and Cardiology, the Children’s Memorial Hospital, and the Departments of Surgery and Pediatrics, Northwestern University Medical School, Chicago, IL. Accepted for publication Apr 21, 1986 Address reprint requests to Dr. DeLeon, Division of CardiovascularThoracic Surgery, The Children’s Memorial Hospital, 2300 Children’s Plaza, Chicago, IL 60614.
207 Ann Thorac Surg 43:207-211, Feb 1987
duced pulmonary blood flow has been reported in several patients with tetralogy of Fallot and more commonly in patients with asplenia [6-111. Creation of a systemicpulmonary artery shunt in these patients without prior recognition and repair of obstructed pulmonary veins may result in persistent severe hypoxemia, pulmonary hypertension, and low cardiac output. At our institution, we have encountered several infants with complex cardiac defects who were seen with pulmonary stenosis or atresia and were found to have obstructed TAPVD, either at initial cardiac catheterization or after a systemic-pulmonary artery shunt had been performed. To our knowledge, no organized approach to this difficult combination of defects has been reported in infants, who, if successfully palliated, can undergo either anatomical or orthoterminal repair at a later age [12, 131. To determine whether such a goal is possible in this group, we analyzed our experience.
Material and Methods Data were collected on 8 surgically treated infants (3 boys, 5 girls) with pulmonary stenosis or atresia and TAPVD in a review of the medical records in our institution over the past 10 years. Five patients were seen in the last 3 years. Clinical summary is outlined in Table 1. Six infants were seen with severe cyanosis and metabolic acidosis at birth, and the remaining 2 were seen at 6 weeks and 3 months of age, respectively. Asplenia was present in 7 patients, and polysplenia was suspected in the eighth. Initial chest roentgenography showed decreased pulmonary vascular markings in 7 patients and questionable pulmonary venous congestion in the eighth patient. Two-dimensional echocardiography was performed in 5 patients, and total anomalous pulmonary venous drainage was diagnosed in 2. Initial cardiac catheterization was performed at the time of clinical presentation on all patients. Five patients were receiving prostaglandin El infusion at the time of cardiac catheterization. In 1 patient, prostaglandin El was started at the end of cardiac Catheterization. The 2 remaining patients, 1 seen early in the series and the other at 3 months of age, did not receive prostaglandin El infusion. The presence of TAPVD was established before any surgical intervention in 4 patients (2 supracardiac, 2 infracardiac); 3 were diagnosed at initial study. The fourth patient with double-outlet right ventricle with pulmonic stenosis who had the initial cardiac
208 The Annals of Thoracic Surgery Vol 43 No 2 February 1987
Table 1. Clinical Summa y of 8 Infants with Complex Cardiac Anomalies and Pulmona y Stenosis or Atresia Major Patient No. Anomalies
Type of TAPVD
Initial Operation (age)
Group 1 1
Infracardiac
Repair of TAPVD (7 days) S-P shunt (4 mo)
Well (18 mo)
Infracardiac
Repair of TAPVD (1%mo) Relief of right pulmonary vein stenosis (7 mo) Repair of DORV (12 mo)
Well (18 mo)
2
Group 2 3 4
ASD, DORV, PS, BIL SVC DORV. PS
Subsequent Operation (age) Outcome (age)
Repair of TAPVD (16 mo) Repair of TAPVD (6 mo); AP shunt 1) S-P shunt (3 mo) 2) S-P shunt (48 mo)
CAVC, DORV, PA CAVC, DORV, PA
Supracardiac Supracardiac
S-P shunt (17 days) S-P shunt (3 days)
5
CAVC, DORV, PA
Supracardiac
AP shunt (10 days)
6
CAVC, CORRECTED TGA, PA CAVC, DORV, PS, BIL SVC
Supra-paracardiac" AP shunt (4 days)
Repair of TAPVD (2% mo)
Infracardiac
S-P shunt (3 mo)
CAVC, DORV, PS, BIL SVC
Infracardiac
S-P shunt (4 days)
1) Repair of TAPVD (3 mo) 2) Revision of CPV-LA anastomosis (18 mo) Repair of TAPVD (4 mo) Repair of left pulmonary vein stenosis (8 mo)
7
8
Died Died Alive (132 mo) Severe cyanosis, small pulmonary arteries Well (24 mo)
Died
Died
AP = aortopulmonary; ASD = atrial septa1 defect; CAVC = common atrioventricular canal; CPV = common pulmonary vein; DORV = double-outlet right ventricle; LA = left atrium; PNPS = pulmonary atresiaktenosis; S-P = subclavian-pulmonary artery; SVC = superior vena cava; TAPVD = total anomalous pulmonary venous drainage; TGA = transposition of the great arteries; BIL = bilateral. "Common pulmonary vein draining to coronary sinus and small vertical vein connected to innominate vein.
catheterization at 6 weeks of age in another institution was referred to us for a systemic-pulmonary artery shunt. Because the pulmonary veins were not visualized on angiogram and the chest x-ray film suggested pulmonary venous congestion, we performed a repeat cardiac catheterization with contrast injected directly into the pulmonary artery that revealed an infracardiac TAPVD (Figure). Limited oxygen saturation data were obtained at initial cardiac catheterization in 5 patients (Table 2). At initial operation, 2 patients (Group 1) underwent repair of an infracardiac TAPVD at 7 days and 1% months of age, respectively, by anastomosis of the common pulmonary vein to the left atrium under cardiopulmonary bypass and profound hypothermia. The anastomosis was performed with interrupted sutures in both patients, using a fine, nonabsorbable monofilament suture. Six patients (Group 2) underwent creation of a systemic-pulmonary artery shunt. Three patients had modified Blalock-Taussig shunt using 5- or 6-mm polytetrafluoroethylene graft, 1 had the classic BlalockTaussig shunt, and 2 had a direct ascending aorta-tomain pulmonary artery shunt.
Results Both patients in Group 1 who had early repair of TAPVD survived and did well. One patient who underwent repair of TAPVD at 7 days of age and a left subclavian-
pulmonary artery shunt using a 5-mm polytetrafluoroethylene graft at 4 months of age is now 1%years old. The other patient, who underwent the repair at 1% months of age and subsequently underwent total repair of double-outlet right ventricle and relief of peripheral obstruction of the right pulmonary veins, is now 1% years old. All patients in Group 2 continued to have moderately severe cyanosis postoperatively with faintly audible or absent shunt murmur. Pulmonary venous congestion on chest roentgenography was present in 3 patients and was suspected or absent in the remaining 3. Repeat cardiac catheterization performed 13 days to 14 months following creation of systemic-pulmonary artery shunt showed patent shunts with systolic pulmonary artery pressure (obtained in 4 patients) ranging from 15 to 70 mm Hg and obstructed TAPVD. In Patients 3 and 4 (see Table 1)with supracardiac drainage, the left vertical vein was compressed between the left pulmonary artery and bronchus. Patient 5 had mild stenosis at the junction of the vertical and common pulmonary veins. Patient 6 had severe stenosis of the vertical vein draining into the coronary sinus, and Patients 7 and 8 had infracardiac type drainage. All 5 patients with severely obstructed pulmonary veins subsequently underwent a common pulmonary vein-to-atrium anastomosis, using continuous sutures of fine, nonabsorbable monofilament suture
209
DeLeon, Gidding, Ilbawi, et al: Complex Cardiac Anomalies in Infants
0
( A ) Right ventriculogram of the patient with double-outlet right ven-
tricle with infundibular stenosis. The pulmonary veins were not visualized. (B)Pulnionary arteriograrn shou~ingfaint oisualization of the pulmonary veins (arrows)draining toward the diaphragm.
in 2 patients and interrupted sutures in 3 patients, under cardiopulmonary bypass and profound hypothermia. One patient died intraoperatively of low cardiac output, and another died 2 weeks later, probably of sepsis. A repeat cardiac catheterization on the latter patient showed a patent systemic-pulmonary artery shunt and common pulmonary vein-to-atrium anastomosis. Be-
cause of recurrence of marked cyanosis, 2 patients who had initial improvement following relief of their obstructed pulmonary veins had repeat cardiac catheterization at 4 months and 15 months, respectively, which showed stenosis of the common pulmonary vein-toatrium anastomosis in 1 and stenosis of individual pulmonary veins in the other. Both died intraoperatively in an attempt to provide further relief of the pulmonary venous obstruction. The fifth patient with mild obstruction underwent creation of two more systemic-pulmonary artery shunts without marked improvement in cyanosis. The patient is now 11 years old, but no further
210 The Annals of Thoracic Surgery Vol 43 No 2 February 1987
Table 2. lnitial Cardiac Catheterization Saturation Data RSVC Patient No.
Type of TAPVD
1 2 3 6 7
Infracardiac lnfracardiac Supracardiac Supra-paracardiac Infracardiac
RA
LSVC
IVC
(%)
(%)
(%I
...
60
...
71 57
92
46 42 79 28
... ... ...
...
...
...
43 64
52
...
Aorta (%)
Diagnosed
80 64 45 79 44
Yes No No Yes No
TAPVD = total anomalous pulmonary venous drainage; IVC = inferior vena cava; LSVC = left superior vena cava; RA = right atrium; RSVC = right superior vena cava.
surgical repair could be done because of hypoplastic pulmonary arteries. Patient 6, who had a direct ascending aorta-to-main pulmonary artery shunt at 4 days of age and repair of total anomalous pulmonary venous drainage 2% months later, survived and is doing well at 2 years of age.
Comment The clinical presentation of infants with complex cardiac anomalies associated with decreased pulmonary blood flow and TAPVD is similar to the more common cyanotic lesions. The pulmonary veins often are not visualized on angiography, leading to missed diagnosis. Thus, creation of a systemic-pulmonary artery shunt is often performed when the appropriate approach should be repair of the TAPVD, with or without the shunting procedure. Freedom and colleagues [ 141 suggested using prostaglandin El challenge to visualize the pulmonary veins in patients with asplenia. This technique probably helped establish the diagnosis in 3 of 5 patients in our series who were receiving prostaglandin El at the time of initial cardiac catheterization. However, none of our patients experienced pulmonary edema following the prostaglandin El infusion. Careful attention to oxygen saturation data may also be helpful. Although only 2 of 5 patients with available oxygen saturation data at initial cardiac catheterization had angiographic visualization of the pulmonary veins, retrospective analysis showed the aortic saturation equal to or lower than the oxygen saturation in the superior or inferior vena cava, suggesting the presence of anomalous pulmonary venous drainage in all 5 patients. Because 50% of our patients who had persistent severe cyanosis with faint or absent continuous murmur following creation of systemic-pulmonary artery shunts did not show roentgenographic evidence of pulmonary edema, ill-advised shunt revisions might have been performed in some of these patients had repeat cardiac catheterization not been done. Additionally, Patient 2 might erroneously have had a systemic-pulmonary artery shunt created at initial operation had a repeat cardiac catheterization with pulmonary angiography not been performed. Although the 4 patients who eventually died following relief of obstructed pulmonary veins had a very difficult combination of lesions, the delay in diagnosis
may have been the major factor contributing to their death (see Table 1). These 4 patients had the operation performed at an average age of 6 months (range, 3-16 months), whereas the 3 patients who survived and are doing well had the operation at an average age of 1 month (range, 7 days to 2% months). There is some suggestive evidence that the incidence of recurrence of pulmonary venous obstruction, presumably from progressive intimal hypertrophy and fibrosis of the pulmonary veins, is higher and the prognosis poorer in patients in whom repair was done late [15, 161. However, although the outlook is grim once recurrent pulmonary venous obstruction occurs, aggressive treatment can still be rewarding. The outlook for the fourth survivor, who now has small pulmonary arteries in spite of several systemic-pulmonary artery shunts, may have been different had the TAPVD been repaired early. In summary, we suggest that infants with complex cardiac anomalies, especially in the presence of asplenia and polysplenia, who are seen with pulmonary stenosis or atresia should be thoroughly investigated with echocardiography, oxygen saturation studies, and angiography with prostaglandin challenge. If TAPVD is detected, it should be repaired early, before or in conjunction with a systemic-pulmonary artery shunt. If the diagnosis has not been established before shunt creation, repeat cardiac catheterization should be performed urgently if the patient has an unsatisfactory response to the shunt. Aggressive and early repair of TAPVD appears to improve prognosis. Supported in part by the A.C. Buehler Foundation, Park Ridge, IL.
References 1. Hawkins JA, Clark EB, Doty DB: Total anomalous pulmonary venous connection. Ann Thorac Surg 36548, 1983 2. Bove EL, DeLeval MR, Taylor JFN, et al: Infradiaphragmatic total anomalous pulmonary venous drainage: surgical treatment and long-term results. Ann Thorac Surg 31:544, 1981 3. Katz NM, Kirklin JW, Pacific0 AD: Concepts and practices in surgery for total anomalous pulmonary venous connection (collective review). Ann Thorac Surg 25:479, 1978 4. Ruttenberg HD: Corrected transposition (I-transposition)of
211 DeLeon, Gidding, Ilbawi, et al: Complex Cardiac Anomalies in Infants
5. 6. 7.
8.
9. 10.
the great arteries and splenic syndromes. In Adams FH, Emmanouilides GC (eds): Moss’ Heart Disease in Infants, Children, and Adolescents. Baltimore, Williams & Wilkins, 1981, pp 333-350 Gersony WM: Obstruction to pulmonary venous return obscured by decreased pulmonary blood flow. Chest 64:283, 1973 Van Mierop LHS, Gessener IH, Schiebler GL: Asplenia and polysplenia syndrome. Birth Defects 8:74, 1972 Rose V, Izukawa T, Moes CAF: Syndromes of asplenia and polysplenia: a review of cardiac and non-cardiac malformation in 60 cases with special reference to diagnosis and prognosis. Br Heart J 37:840, 1975 Delisle G, Ando M, Calder AL: Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J 91:99, 1976 Muster AJ, Paul MH, Nikaidoh H: Tetralogy of Fallot associated with total anomalous pulmonary venous drainage. Chest 64:323, 1973 Gerlis LM, Fiddler GI, Pearse RG: Total anomalous pulmo-
11. 12. 13. 14.
15. 16.
nary venous drainage associated with tetralogy of Fallot: report of a case. Pediatr Cardiol4:297, 1983 Gutierrez J, DeLeon JP, DeMarco E, et al: Tetralogy of Fallot with total anomalous pulmonary venous drainage. Pediatr Cardiol 4:293, 1983 Ando F, Shirotani H, Kawai J, et al: Successful total repair of complicated cardiac anomalies with asplenia syndrome. J Thorac Cardiovasc Surg 72:33, 1976 Marcelletti C, Donato RD, Nijveld A, et al: Right and left isomerism: the cardiac surgeon’s view. Ann Thorac Surg 35:400, 1983 Freedom RM, Olley PM, Coceani F, Rowe RD: The prostaglandin challenge: test to unmask obstructed total anomalous pulmonary venous connection in asplenia syndrome. Br Heart J 40:90, 1978 Newfeld EA, Wilson A, Paul MH, Reisch JS: Pulmonary vascular disease in total anomalous pulmonary venous drainage. Circulation 61:103, 1980 Higashino SM, Shaw GG, May IA, Ecker RR: Total anomalous pulmonary venous drainage below the diaphragm. J Thorac Cardiovasc Surg 68:711, 1974
Notice from the American Board of Thoracic Surgery The American Board of Thoracic Surgery began its recertification process in 1984. Diplomates interested in participating in this examination should maintain a documented list of the cardiothoracic operations they performed during the year prior to application for recertification. They should also keep a record of their attendance at thoracic surgical meetings, and other continuing medical education activities pertaining to thoracic surgery and thoracic disease, for the two years prior to application. A minimum of 100 hours of approved CME activity is required. In place of a cognitive examination, candidates for recertification will be required to complete both the general thoracic and cardiac portions of the SESATS 111 syllabus (Self-EducatiodSelf-Assessment in Thoracic Surgery). It is not necessary for candidates to purchase
SESATS 111booklets prior to applying for recertification. SESATS 111 booklets will be forwarded to candidates after their applications have been accepted. Diplomates whose 10-year certificates will expire in 1989 may begin the recertification process in 1987. This new certificate will be dated 10 years from the time of expiration of the original certificate. Recertification is also open to any Diplomate with an unlimited certificate and will in no way affect the validity of the original certificate. The deadline for submission of applications is July 1, 1987. A recertification brochure outlining the rules and requirements for recertification in thoracic surgery is available upon request from the American Board of Thoracic Surgery, One American Plaza, Suite 803, Evanston, IL 60201.
207 Ann Thorac Surg 43:207-211, Feb 1987
duced pulmonary blood flow has been reported in several patients with tetralogy of Fallot and more commonly in patients with asplenia [6-111. Creation of a systemicpulmonary artery shunt in these patients without prior recognition and repair of obstructed pulmonary veins may result in persistent severe hypoxemia, pulmonary hypertension, and low cardiac output. At our institution, we have encountered several infants with complex cardiac defects who were seen with pulmonary stenosis or atresia and were found to have obstructed TAPVD, either at initial cardiac catheterization or after a systemic-pulmonary artery shunt had been performed. To our knowledge, no organized approach to this difficult combination of defects has been reported in infants, who, if successfully palliated, can undergo either anatomical or orthoterminal repair at a later age [12, 131. To determine whether such a goal is possible in this group, we analyzed our experience.
Material and Methods Data were collected on 8 surgically treated infants (3 boys, 5 girls) with pulmonary stenosis or atresia and TAPVD in a review of the medical records in our institution over the past 10 years. Five patients were seen in the last 3 years. Clinical summary is outlined in Table 1. Six infants were seen with severe cyanosis and metabolic acidosis at birth, and the remaining 2 were seen at 6 weeks and 3 months of age, respectively. Asplenia was present in 7 patients, and polysplenia was suspected in the eighth. Initial chest roentgenography showed decreased pulmonary vascular markings in 7 patients and questionable pulmonary venous congestion in the eighth patient. Two-dimensional echocardiography was performed in 5 patients, and total anomalous pulmonary venous drainage was diagnosed in 2. Initial cardiac catheterization was performed at the time of clinical presentation on all patients. Five patients were receiving prostaglandin El infusion at the time of cardiac catheterization. In 1 patient, prostaglandin El was started at the end of cardiac Catheterization. The 2 remaining patients, 1 seen early in the series and the other at 3 months of age, did not receive prostaglandin El infusion. The presence of TAPVD was established before any surgical intervention in 4 patients (2 supracardiac, 2 infracardiac); 3 were diagnosed at initial study. The fourth patient with double-outlet right ventricle with pulmonic stenosis who had the initial cardiac
208 The Annals of Thoracic Surgery Vol 43 No 2 February 1987
Table 1. Clinical Summa y of 8 Infants with Complex Cardiac Anomalies and Pulmona y Stenosis or Atresia Major Patient No. Anomalies
Type of TAPVD
Initial Operation (age)
Group 1 1
Infracardiac
Repair of TAPVD (7 days) S-P shunt (4 mo)
Well (18 mo)
Infracardiac
Repair of TAPVD (1%mo) Relief of right pulmonary vein stenosis (7 mo) Repair of DORV (12 mo)
Well (18 mo)
2
Group 2 3 4
ASD, DORV, PS, BIL SVC DORV. PS
Subsequent Operation (age) Outcome (age)
Repair of TAPVD (16 mo) Repair of TAPVD (6 mo); AP shunt 1) S-P shunt (3 mo) 2) S-P shunt (48 mo)
CAVC, DORV, PA CAVC, DORV, PA
Supracardiac Supracardiac
S-P shunt (17 days) S-P shunt (3 days)
5
CAVC, DORV, PA
Supracardiac
AP shunt (10 days)
6
CAVC, CORRECTED TGA, PA CAVC, DORV, PS, BIL SVC
Supra-paracardiac" AP shunt (4 days)
Repair of TAPVD (2% mo)
Infracardiac
S-P shunt (3 mo)
CAVC, DORV, PS, BIL SVC
Infracardiac
S-P shunt (4 days)
1) Repair of TAPVD (3 mo) 2) Revision of CPV-LA anastomosis (18 mo) Repair of TAPVD (4 mo) Repair of left pulmonary vein stenosis (8 mo)
7
8
Died Died Alive (132 mo) Severe cyanosis, small pulmonary arteries Well (24 mo)
Died
Died
AP = aortopulmonary; ASD = atrial septa1 defect; CAVC = common atrioventricular canal; CPV = common pulmonary vein; DORV = double-outlet right ventricle; LA = left atrium; PNPS = pulmonary atresiaktenosis; S-P = subclavian-pulmonary artery; SVC = superior vena cava; TAPVD = total anomalous pulmonary venous drainage; TGA = transposition of the great arteries; BIL = bilateral. "Common pulmonary vein draining to coronary sinus and small vertical vein connected to innominate vein.
catheterization at 6 weeks of age in another institution was referred to us for a systemic-pulmonary artery shunt. Because the pulmonary veins were not visualized on angiogram and the chest x-ray film suggested pulmonary venous congestion, we performed a repeat cardiac catheterization with contrast injected directly into the pulmonary artery that revealed an infracardiac TAPVD (Figure). Limited oxygen saturation data were obtained at initial cardiac catheterization in 5 patients (Table 2). At initial operation, 2 patients (Group 1) underwent repair of an infracardiac TAPVD at 7 days and 1% months of age, respectively, by anastomosis of the common pulmonary vein to the left atrium under cardiopulmonary bypass and profound hypothermia. The anastomosis was performed with interrupted sutures in both patients, using a fine, nonabsorbable monofilament suture. Six patients (Group 2) underwent creation of a systemic-pulmonary artery shunt. Three patients had modified Blalock-Taussig shunt using 5- or 6-mm polytetrafluoroethylene graft, 1 had the classic BlalockTaussig shunt, and 2 had a direct ascending aorta-tomain pulmonary artery shunt.
Results Both patients in Group 1 who had early repair of TAPVD survived and did well. One patient who underwent repair of TAPVD at 7 days of age and a left subclavian-
pulmonary artery shunt using a 5-mm polytetrafluoroethylene graft at 4 months of age is now 1%years old. The other patient, who underwent the repair at 1% months of age and subsequently underwent total repair of double-outlet right ventricle and relief of peripheral obstruction of the right pulmonary veins, is now 1% years old. All patients in Group 2 continued to have moderately severe cyanosis postoperatively with faintly audible or absent shunt murmur. Pulmonary venous congestion on chest roentgenography was present in 3 patients and was suspected or absent in the remaining 3. Repeat cardiac catheterization performed 13 days to 14 months following creation of systemic-pulmonary artery shunt showed patent shunts with systolic pulmonary artery pressure (obtained in 4 patients) ranging from 15 to 70 mm Hg and obstructed TAPVD. In Patients 3 and 4 (see Table 1)with supracardiac drainage, the left vertical vein was compressed between the left pulmonary artery and bronchus. Patient 5 had mild stenosis at the junction of the vertical and common pulmonary veins. Patient 6 had severe stenosis of the vertical vein draining into the coronary sinus, and Patients 7 and 8 had infracardiac type drainage. All 5 patients with severely obstructed pulmonary veins subsequently underwent a common pulmonary vein-to-atrium anastomosis, using continuous sutures of fine, nonabsorbable monofilament suture
209
DeLeon, Gidding, Ilbawi, et al: Complex Cardiac Anomalies in Infants
0
( A ) Right ventriculogram of the patient with double-outlet right ven-
tricle with infundibular stenosis. The pulmonary veins were not visualized. (B)Pulnionary arteriograrn shou~ingfaint oisualization of the pulmonary veins (arrows)draining toward the diaphragm.
in 2 patients and interrupted sutures in 3 patients, under cardiopulmonary bypass and profound hypothermia. One patient died intraoperatively of low cardiac output, and another died 2 weeks later, probably of sepsis. A repeat cardiac catheterization on the latter patient showed a patent systemic-pulmonary artery shunt and common pulmonary vein-to-atrium anastomosis. Be-
cause of recurrence of marked cyanosis, 2 patients who had initial improvement following relief of their obstructed pulmonary veins had repeat cardiac catheterization at 4 months and 15 months, respectively, which showed stenosis of the common pulmonary vein-toatrium anastomosis in 1 and stenosis of individual pulmonary veins in the other. Both died intraoperatively in an attempt to provide further relief of the pulmonary venous obstruction. The fifth patient with mild obstruction underwent creation of two more systemic-pulmonary artery shunts without marked improvement in cyanosis. The patient is now 11 years old, but no further
210 The Annals of Thoracic Surgery Vol 43 No 2 February 1987
Table 2. lnitial Cardiac Catheterization Saturation Data RSVC Patient No.
Type of TAPVD
1 2 3 6 7
Infracardiac lnfracardiac Supracardiac Supra-paracardiac Infracardiac
RA
LSVC
IVC
(%)
(%)
(%I
...
60
...
71 57
92
46 42 79 28
... ... ...
...
...
...
43 64
52
...
Aorta (%)
Diagnosed
80 64 45 79 44
Yes No No Yes No
TAPVD = total anomalous pulmonary venous drainage; IVC = inferior vena cava; LSVC = left superior vena cava; RA = right atrium; RSVC = right superior vena cava.
surgical repair could be done because of hypoplastic pulmonary arteries. Patient 6, who had a direct ascending aorta-to-main pulmonary artery shunt at 4 days of age and repair of total anomalous pulmonary venous drainage 2% months later, survived and is doing well at 2 years of age.
Comment The clinical presentation of infants with complex cardiac anomalies associated with decreased pulmonary blood flow and TAPVD is similar to the more common cyanotic lesions. The pulmonary veins often are not visualized on angiography, leading to missed diagnosis. Thus, creation of a systemic-pulmonary artery shunt is often performed when the appropriate approach should be repair of the TAPVD, with or without the shunting procedure. Freedom and colleagues [ 141 suggested using prostaglandin El challenge to visualize the pulmonary veins in patients with asplenia. This technique probably helped establish the diagnosis in 3 of 5 patients in our series who were receiving prostaglandin El at the time of initial cardiac catheterization. However, none of our patients experienced pulmonary edema following the prostaglandin El infusion. Careful attention to oxygen saturation data may also be helpful. Although only 2 of 5 patients with available oxygen saturation data at initial cardiac catheterization had angiographic visualization of the pulmonary veins, retrospective analysis showed the aortic saturation equal to or lower than the oxygen saturation in the superior or inferior vena cava, suggesting the presence of anomalous pulmonary venous drainage in all 5 patients. Because 50% of our patients who had persistent severe cyanosis with faint or absent continuous murmur following creation of systemic-pulmonary artery shunts did not show roentgenographic evidence of pulmonary edema, ill-advised shunt revisions might have been performed in some of these patients had repeat cardiac catheterization not been done. Additionally, Patient 2 might erroneously have had a systemic-pulmonary artery shunt created at initial operation had a repeat cardiac catheterization with pulmonary angiography not been performed. Although the 4 patients who eventually died following relief of obstructed pulmonary veins had a very difficult combination of lesions, the delay in diagnosis
may have been the major factor contributing to their death (see Table 1). These 4 patients had the operation performed at an average age of 6 months (range, 3-16 months), whereas the 3 patients who survived and are doing well had the operation at an average age of 1 month (range, 7 days to 2% months). There is some suggestive evidence that the incidence of recurrence of pulmonary venous obstruction, presumably from progressive intimal hypertrophy and fibrosis of the pulmonary veins, is higher and the prognosis poorer in patients in whom repair was done late [15, 161. However, although the outlook is grim once recurrent pulmonary venous obstruction occurs, aggressive treatment can still be rewarding. The outlook for the fourth survivor, who now has small pulmonary arteries in spite of several systemic-pulmonary artery shunts, may have been different had the TAPVD been repaired early. In summary, we suggest that infants with complex cardiac anomalies, especially in the presence of asplenia and polysplenia, who are seen with pulmonary stenosis or atresia should be thoroughly investigated with echocardiography, oxygen saturation studies, and angiography with prostaglandin challenge. If TAPVD is detected, it should be repaired early, before or in conjunction with a systemic-pulmonary artery shunt. If the diagnosis has not been established before shunt creation, repeat cardiac catheterization should be performed urgently if the patient has an unsatisfactory response to the shunt. Aggressive and early repair of TAPVD appears to improve prognosis. Supported in part by the A.C. Buehler Foundation, Park Ridge, IL.
References 1. Hawkins JA, Clark EB, Doty DB: Total anomalous pulmonary venous connection. Ann Thorac Surg 36548, 1983 2. Bove EL, DeLeval MR, Taylor JFN, et al: Infradiaphragmatic total anomalous pulmonary venous drainage: surgical treatment and long-term results. Ann Thorac Surg 31:544, 1981 3. Katz NM, Kirklin JW, Pacific0 AD: Concepts and practices in surgery for total anomalous pulmonary venous connection (collective review). Ann Thorac Surg 25:479, 1978 4. Ruttenberg HD: Corrected transposition (I-transposition)of
211 DeLeon, Gidding, Ilbawi, et al: Complex Cardiac Anomalies in Infants
5. 6. 7.
8.
9. 10.
the great arteries and splenic syndromes. In Adams FH, Emmanouilides GC (eds): Moss’ Heart Disease in Infants, Children, and Adolescents. Baltimore, Williams & Wilkins, 1981, pp 333-350 Gersony WM: Obstruction to pulmonary venous return obscured by decreased pulmonary blood flow. Chest 64:283, 1973 Van Mierop LHS, Gessener IH, Schiebler GL: Asplenia and polysplenia syndrome. Birth Defects 8:74, 1972 Rose V, Izukawa T, Moes CAF: Syndromes of asplenia and polysplenia: a review of cardiac and non-cardiac malformation in 60 cases with special reference to diagnosis and prognosis. Br Heart J 37:840, 1975 Delisle G, Ando M, Calder AL: Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J 91:99, 1976 Muster AJ, Paul MH, Nikaidoh H: Tetralogy of Fallot associated with total anomalous pulmonary venous drainage. Chest 64:323, 1973 Gerlis LM, Fiddler GI, Pearse RG: Total anomalous pulmo-
11. 12. 13. 14.
15. 16.
nary venous drainage associated with tetralogy of Fallot: report of a case. Pediatr Cardiol4:297, 1983 Gutierrez J, DeLeon JP, DeMarco E, et al: Tetralogy of Fallot with total anomalous pulmonary venous drainage. Pediatr Cardiol 4:293, 1983 Ando F, Shirotani H, Kawai J, et al: Successful total repair of complicated cardiac anomalies with asplenia syndrome. J Thorac Cardiovasc Surg 72:33, 1976 Marcelletti C, Donato RD, Nijveld A, et al: Right and left isomerism: the cardiac surgeon’s view. Ann Thorac Surg 35:400, 1983 Freedom RM, Olley PM, Coceani F, Rowe RD: The prostaglandin challenge: test to unmask obstructed total anomalous pulmonary venous connection in asplenia syndrome. Br Heart J 40:90, 1978 Newfeld EA, Wilson A, Paul MH, Reisch JS: Pulmonary vascular disease in total anomalous pulmonary venous drainage. Circulation 61:103, 1980 Higashino SM, Shaw GG, May IA, Ecker RR: Total anomalous pulmonary venous drainage below the diaphragm. J Thorac Cardiovasc Surg 68:711, 1974
Notice from the American Board of Thoracic Surgery The American Board of Thoracic Surgery began its recertification process in 1984. Diplomates interested in participating in this examination should maintain a documented list of the cardiothoracic operations they performed during the year prior to application for recertification. They should also keep a record of their attendance at thoracic surgical meetings, and other continuing medical education activities pertaining to thoracic surgery and thoracic disease, for the two years prior to application. A minimum of 100 hours of approved CME activity is required. In place of a cognitive examination, candidates for recertification will be required to complete both the general thoracic and cardiac portions of the SESATS 111 syllabus (Self-EducatiodSelf-Assessment in Thoracic Surgery). It is not necessary for candidates to purchase
SESATS 111booklets prior to applying for recertification. SESATS 111 booklets will be forwarded to candidates after their applications have been accepted. Diplomates whose 10-year certificates will expire in 1989 may begin the recertification process in 1987. This new certificate will be dated 10 years from the time of expiration of the original certificate. Recertification is also open to any Diplomate with an unlimited certificate and will in no way affect the validity of the original certificate. The deadline for submission of applications is July 1, 1987. A recertification brochure outlining the rules and requirements for recertification in thoracic surgery is available upon request from the American Board of Thoracic Surgery, One American Plaza, Suite 803, Evanston, IL 60201.