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Total anomalous pulmonary venous drainage with ventricular septal defect
Total with
anomalous ventricular
pulmonary venous septal defect
drainage
Carl N. Steeg, M.D. Kent E&is, M.D. Welton M. Gersony, M.D. New York, N. Y.
T
he association of a large ventricular septal defect with total anomalous pulmonary venous drainage has been reported only rarely. In2 The clinical implications of this combination of lesions have not been previously emphasized. Three patients with total anomalous pulmonary venous drainage and ventricular septal defect have been documented at this institution within a one-year period. One infant had additional pulmonic stenosis. All presented with clinical findings compatible with large left-to-right shunts. The diagnosis was made at cardiac catheterization and angiocardiography in two patients but, in the third infant, the ventricular septal defect was not discovered until repeat catheterization following repair of the total anomalous pulmonary venous drainage. Incomplete diagnosis may result in serious errors in management. The intent of this report is to outline an approach to diagnosis and treatment of these defects. Case reports Case 1. L. A., a one-month-old Negro boy, was admitted to Babies Hospital for cardiac evaluation. A murmur had been noted on the first day of life From
the Division
Physicians New York,
of Pediatric Cardiology. and Surgeons, Columbia N. Y.
This work was supported Received
Reprint
for publication
by Training Nov.
Vol. 86, No. 3, pp. 341-346
York.
Department of Pediatrics, and the Department of Radiology, College of University and Babies Hospital. Columbia-Presbyterian Medical Center,
Grant
HE Q5389-12
of the National
Institutes
of Health.
13, 1972.
requests to: Carl N. Steeg. M.D..
168 St., New
and congestive heart failure and pneumonia occurred at nine days of age. The infant was digital&d and treated with penicillin and kanamycin at another hospital. Transfer to Babies Hospital was arranged when the baby did not improve. Physical examination disclosed a vigorous acyanotic infant in moderate respiratory distress. The weight was 2.6 kilograms; heart rate, 120; respiratory rate, 100. The lungs were clear to auscultation. Examination of the heart revealed a grade III/VI blowing pansystolic murmur at the lower left sternal border. St was single. The liver was palpable 3 cm. below the right costal margin. The chest x-ray showed an enlarged heart with pulmonary vascular overcirculation. An electrocardiogram (ECG) showed a QRS axis of +120”, an RsR’ in VI, and right ventricular predominance. Oxygen saturation in room air was 90 per cent. The infant showed no improvement on a maximal cardiotonic regimen. Three days following admission cardiac catheterization and selective right ventricular angiocardiography demonstrated total anomalous pulmonary venous drainage via the coronary sinus. Left ventricular angiocardiography was not performed. The data are summarized in Table I. Three days following the cardiovascular study, the total anomalous venous drainage was totally repaired by means of cardiopulmonary bypass, The postoperative course was characterized by recurrent pneumonia and atelectasis with Pcol’s ranging from 40.9 to 61 mm. Hg. Persistent pulmonary failure led to long-term administration of high oxygen concentrations. A lower left sterna1 border holosystolic murmur persisted. Two months postoper-
Cardiovascular
Laboratory.
Columbia-Presbyterian
Medical
Center. 622 W.
N. Y. 10032.
September, 1973
American Heart Journal
341
342
Table
Steeg, Ellis,
I. Cardiac
Am. Heart I. Sc~tember, 1973
and Gersony
catheterization
data
SW -__I
Patienl
sal.*J L. A. No. 1 No. 2
81 72
N. G.
93
R. R.
845 69
P’
M=
*Sat. = Oxygen saturation; tM = mean. $ED = End diastolic.
&.t.;p
2
85 73
97
saf./ RVp
&,t.iPAp
89 82
65/12EDt 33/3ED
88 82
65/24, 35/16,
ii ii
95
M=
511 93
102/7ED
87
85/45,
ii
85
M=
7
86/8qED
91
32/10,8
ii
tM = 6 M= 2
89
sat.;p
M = 3
sat./
Lvp
96
74/2ED
(RU/M.Y lvR
3.6 2.2
M= M=7
711 83 98
1 lO/lOED
4.4
86/-
0.9
P = pressure.
WC. /INot simultaneous. DDirect withdrawal.
atively a second catheterization was carried out, and a left-to-right shunt at the ventricular level was suspected on the basis of oxygen saturation data. The pulmonary artery pressure was only minimally increased (Table I), and pulmonary vascular resistance (PVR) was calculated at 2.2 resistance units (RU). The infant continued to do poorly over the succeeding months. Feedings were not taken well and respiratory distress continued. Despite the use of a feeding gastrostomy, the infant failed to thrive. His physical status gradually deteriorated and he died 7% months postoperatively. Postmortem examination. The heart and lungs weighed 250 grams (normal = 119 * 30). The total anomalous pulmonary venous drainage was well repaired and there was no visible obstruction to pulmonary venous return. A ventricular septal defect 0.8 cm. in diameter was found above the crista supraventricularis. The right ventricle was dilated, as was the main pulmonary artery, which was larger in diameter than the ascending aorta. The left ventricle was slightly larger than the right. Microscopic examination of the lungs revealed hypertrophy of arteries and veins with mild muscular arterial hyperplasia (Stage II Pulmonary Vascular Disease)3 as well as septal and focal interlobular fibrosis. These fibrotic changes may represent the effects of prolonged oxygen administration. Case 2. N. G., a 4-month-old Caucasian girl, was admitted to Babies Hospital for cardiac surgery. The history was unremarkable until 2% months of age, when tachypnea and increasing fatiguability were noted. A murmur compatible with the clinical diagnosis of ventricular septal defect was heard one month later. The infant was admitted to another hospital and digitalized with minimal improvement. Cardiac catheterization and angiocardiography showed total anomalous pulmonary venous drainage via a left superior vena cava. A left ventricular angiocardiogram was not done. She was then transferred to Babies Hospital.
On admission the infant was noted to be acyanotic and in mild respiratory distress. The weight was 4.1 kilograms; heart rate, 150; respiratory rate, 70. The lungs were clear to auscultation. Examination of the heart revealed a visible right ventricular impulse, a grade III/VI holosystolic murmur at the left sternal border, and a single second sound. There was no hepatosplenomegaly or peripheral edema. The ECG showed an axis of +135 with right ventricular enlargement. The T waves were upright in VI. A chest x-ray showed cardiomegaly with a wide superior mediastinum and pulmonary vascular engorgement. Because of the murmur suggestive of ventricular septal defect, the infant was recatheterized, The catheterization data confirmed total anomalous pulmonary venous drainage via the superior vena cava with pulmonary hypertension (Table I). An angiocardiogram performed with the catheter in the left ventricle demonstrated the presence of a large, high ventricular septal defect with left-to-right shunt. Two days later, an open-heart operation was performed in an attempt at total correction. In addition to the total anomalous pulmonary venous drainage to the left innominate vein, a large membranous ventricular septal defect was found. Correction of these anomalies required 80 minutes of cardiopulmonary bypass. Four hours postoperatively, the patient became severely acidotic. Multiple cardiac arrests ensued and she died eight hours postoperatively. An autopsy was not obtained. Case 3. R. R., a 2-month-old Caucasian boy, was admitted to Babies Hospital because of congestive heart failure. He was the product of an uncomplicated term pregnancy and appeared normal at birth. A heart murmur was noted at one week of age, but the infant thrived at home with good weight gain. One week prior to admission, tachypnea, fatigue with feedings, and excessive sweating were noted. Physical examination revealed a well-developed, well-nourished, acyanotic infant in moderate respiratory distress. The weight was 4.6 kilograms; heart rate, 120; respiratory rate, 90. Bilateral moist rLles
Volume Number
86 3
Total anomalous pulmonary
were heard over both posterior lung fields. Cardiac examination revealed a prominent systolic thrill at the left lower sternal border. The second sound was single. A grade IV/VI .rough holosystolic murmur was heard along the left lower sternal border. The liver was palpable 7 cm. below the right costal margin. There was no cyanosis. The peripheral pulses were strong and equal. Chest x-ray showed an enlarged heart with marked increase in prominence of the pulmonary vasculature (Fig. 1). The ECG revealed the QRS to be +llO” with right ventricular preponderance (Fig. 2). P waves in Lead II were 2.3 mm. and peaked. The infant improved following treatment with digoxin, furosemide, and oxygen. One week following admission, cardiac catheterization and right ventricular angiocardiography documented total anomalous pulmonary venous drainage to the right atrium (apparently via the coronary sinus) (Fig. 3). The catheterization data showed right ventricular hypertension and some right ventricular outflow tract obstruction within a 52 mm. peak systolic gradient between the pulmonary artery and the bodv of the right ventricle (Table I). A selective-left ventricular angiocardiogram was also performed which showed a large left-to-right shunt at the ventricular level (Fig. 4). The infant’s condition remained stable and he was discharged on digoxin three days following the procedure. He is presently doing well at home at 18 months of age.
Discussion
The reported incidence of total anomalous pulmonary venous drainage with a ventricular septal defect as the sole complicating lesion varies. Brody,4 reviewing 102 cases of anomalous pulmonary venous drainage of which 37 were total, found no case of associated ventricular septal defect. Burroughs and Edwards5 did not find a ventricular septal defect as a single associated malformation in their review of 188 casesof total anomalous pulmonary venous drainage, although 66 had other multiple major cardiac anomalies including, in many cases, the asplenia syndrome. In a review of 30 patients with total anomalous pulmonary venous drainage Gott and associates6 found six casesto have associated anomalies but none was an isolated ventricular septal defect. The extensive review of Nakib and colleagues7 and Blake and colleagues* also failed to show ventricular septal defect alone as a frequent complicating lesion. On the other hand, Bonham-Carter and associates,’ in an analysis of 75 children with total anomalous pulmonary venous drainage, found that, of 17 with major associated anomalies, four had ventricular
venous drainage with
VSD
Fig. 1. Admission chest roentgenogram showing cardiomegaly with increased
343
(Case3) vascularity.
septal defect. Our experience since 1966 consists of 19 patients with total anomalous pulmonary venous drainage, three of whom had ventricular septal defects. When total anomalous pulmonary venous drainage with pulmonary hypertension exists as an isolated lesion, we have reported in a previous publication that early surgical correction of the defect by means of cardiopulmonary bypass has been a successful method of treatment at this institution.g However, when a complicating ventricular septal defect is present, this may not be the best approach to management. Indeed, failure to diagnose the additional anomaly may prove fatal if correction of the total anomalous venous drainage alone is undertaken. In all three of these cases the diagnosis of ventricular septal defect with left-toright shunt was considered on the basis of the clinical presentation, including especially the auscultatory findings. In two of these cases, however, after the documentation of total anomalous pulmonary venous drainage during the initial catheterization, the possibility of ventricular septal defect was not further investigated-partly in view of the supposed rarity of this association. The murmurs were then attributed to tricuspid insufficiency. In retrospect, however, the holosystolic left sternal border murmurs were not caused by tricuspid insufficiency, but were secondary to the ventricular septal defects as initially suspected. In one of these cases, repeat catheterization did reveal the presence of the
344
Am. Heart I. Sefitember, 1973
Steeg, Ellis, and Gersony
Fig. 2. Admission ECG (Case 3).
Axis:
+llO”.
Right
Fig. 3. Frontal angiocardiogram (Case 3) showing late phase of pulmonary vein opacification 3 sec. following injection of radiopaque contrast material into right ventricle. The expected opacification of the left atrium is not seen. instead the pulmonary veins opacify the right atrium (RA) apparently via the coronary sinus (a~rorus).
complicating ventricular septal defect prior to the attempt at corrective surgery. In the third case, the initial diagnosis of ventricular septal defect was confirmed by a left ventricular angiocardiogram in the early phases of the catheterization, and total anomalous pulmonary venous drain-
ventricular
preponderance.
P waves
are peaked
in Lead
II.
age was not really suspected until subsequent completion of the right-sided study. Since left and right ventricular oxygen saturations are similar in total anomalous pulmonary venous drainage, oxygen saturation determinations cannot be expected to be of great value in the detection of a complicating ventricular septal defect. Oxygen saturation data, however, might have been of value in one of our patients (N. G., Case 2), in whom a consistent “step-down” in saturation was noted on entering the pulmonary artery from the right ventricle (Table I). In this instance, left heart saturations were somewhat lower than those in the right ventricle-presumably due to streaming of less saturated inferior vena caval blood into the left heart via the foramen ovale. Thus a left-to-right shunt at the ventricular level may have been paradoxically responsible for the modest fall or “step-down” of oxygen saturation in the pulmonary artery. The hemodynamic significance of a large ventricular septal defect in total anomalous pulmonary venous drainage is complex. In our cases, left-to-right shunts occurred via the ventricular septal defect. This diversion of some of the left ventricu-
V&me Nrmber
86 3
Total anomalous pulmonary
venous drainage with
VSD
345
Fig. 4. Frontal (left) and lateral (right) angiocardiograms (Case3) during injection of radiopaquecontrast materialinto the relatively small left ventricle. A substantial left-to-right shunt via a ventricular septal defect ooacifies the righht ventricle and pulmonary arteries. The right ventricular b; catheter wiihdrawal. _
lar stroke output tends to increase blood flow in the pulmonary circuit and, at the same time, reduce the systemic output. Such hemodynamics require relatively large flows from right-to-left across the interatria1 septum. If resistance in the pulmonary circuit is high, left-to-right shunting via the ventricular septal defect would be
limited, and in rare instances it might be speculated that right-to-left shunting through such a ventricular septal defect would occur, reducing pulmonary blood flow while augmenting systemic output. In the absence of a ventricular septal defect, surgical elimination of the large pulmonary
vein-right
atria1
shunt
reduces
right ventricular return and right ventricular output. The pulmonary artery pressure is correspondingly reduced even assuming no change in pulmonary vascular resistance. When a large ventricular septal defect is present, however, the reduced right ventricular output would tend to be matched by a corresponding increase in the left-toright
shunt
via
the
ventricular
septal
defect. Thus, by repair of the anomalous venous connections alone, the work of pumping a large left-to-right shunt is shifted from the right to the left ventricle. This radical change in the output requirements for the left ventricle may not be tolerated. In Case 1, the limited size of the ventricular septal defect probably prevented immediate catastrophe following repair of the total anomalous pulmonary venous drainage and death seemed unrelated to the interventricular communication.
Since
outflow tract gradient was identified
the ventricular
septum
is usually
intact in total anomalous pulmonary venous drainage, palliative pulmonary artery banding has not been considered. However, when a large ventricular septal defect complicates total anomalous pulmonary venous drainage in a critically ill infant with pulmonary hypertension and congestive heart failure, a palliative operation-banding of the pulmonary artery-might be considered. This procedure would be expected to reduce both pulmonary blood flow and pulmonary hypertension while simultaneously augmenting systemic output. Such beneficial effects are suggested by the favorable course of R. R. (Case 3), who had a moderate obstruction and pressure gradient across the pulmonary outflow tract. Summary Three patients with total anomalous pulmonary venous drainage and ventricular septal defect with left-to-right shunt are presented. The ventricular septal defect was diagnosed with left ventricular angiocardiography in two patients and confirmed on postmortem examination in the third. The complex hemodynamic implications of this combination of lesions are discussed. Pulmonary artery banding is suggested as a possible method of management in early infancy. When total anomalous pulmonary venous drainage is demonstrated in infants initially suspected of having large ventricular septal defects on the basis of auscultation, left ventricular angiocardiography is mandatory. Preoperative diagnosis of a compli-
346
Am. Heart I. September, 1973
Steeg, Ellis, and Gersony
eating ventricular septal defect is essential to optimal management. REFERENCES Bonham-Carter, R. E., Capriles, M., and Noe, Y.: Total anomalous pulmonary venous drainage: a clinical and anatomical study of 75 children, Br. Heart J. 31:4.5, 1969. Darling, R. C., Rothney, W. B., and Craig, J. M.: Total pulmonary venous drainage into the right side of the heart, Lab. Invest. 6:44, 1957. Heath, D., and Edwards, J. E.: The pathology of hypertensive pulmonary vascular disease. A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects, Circulation 18533, 1958. Brody, H.: Drainage of the pulmonary veins into
the right side of the heart, Arch. Pathol. 33:221, 1942. 5. Burroughs, J. T., and Edwards, J. E.: Total anomalous pulmonary venous connection. AM. HEART
J. 59:913,
1960.
6. Gott, V. L., Lester, R. G., Lillehei, C. W., and Varco, R. L.: Total anomalous pulmonary return: An analysis of 30 cases, Circulation 13:543, 1956. Nakib, A., Moller, J. H., Kanjuh, V. I., and Edwards, J. E.: Anomalies of the pulmonary veins, Am. J. Cardiol. 26:77, 1967. Blake, H. A., Hall, R. J., and Manion, W. C.: Anomalous pulmonary venous return, Circulation 32:406, 1965. Gersony, W. M., Bowman, F. O., Jr., Steeg, C. N., Hayes, C. J., Jesse, M. J., and Maim, J. R.: Management of total anomalous pulmonary venous drainage in early infancy. Circulation 43 (Suppl. 1):19, 1971.
anomalous ventricular
pulmonary venous septal defect
drainage
Carl N. Steeg, M.D. Kent E&is, M.D. Welton M. Gersony, M.D. New York, N. Y.
T
he association of a large ventricular septal defect with total anomalous pulmonary venous drainage has been reported only rarely. In2 The clinical implications of this combination of lesions have not been previously emphasized. Three patients with total anomalous pulmonary venous drainage and ventricular septal defect have been documented at this institution within a one-year period. One infant had additional pulmonic stenosis. All presented with clinical findings compatible with large left-to-right shunts. The diagnosis was made at cardiac catheterization and angiocardiography in two patients but, in the third infant, the ventricular septal defect was not discovered until repeat catheterization following repair of the total anomalous pulmonary venous drainage. Incomplete diagnosis may result in serious errors in management. The intent of this report is to outline an approach to diagnosis and treatment of these defects. Case reports Case 1. L. A., a one-month-old Negro boy, was admitted to Babies Hospital for cardiac evaluation. A murmur had been noted on the first day of life From
the Division
Physicians New York,
of Pediatric Cardiology. and Surgeons, Columbia N. Y.
This work was supported Received
Reprint
for publication
by Training Nov.
Vol. 86, No. 3, pp. 341-346
York.
Department of Pediatrics, and the Department of Radiology, College of University and Babies Hospital. Columbia-Presbyterian Medical Center,
Grant
HE Q5389-12
of the National
Institutes
of Health.
13, 1972.
requests to: Carl N. Steeg. M.D..
168 St., New
and congestive heart failure and pneumonia occurred at nine days of age. The infant was digital&d and treated with penicillin and kanamycin at another hospital. Transfer to Babies Hospital was arranged when the baby did not improve. Physical examination disclosed a vigorous acyanotic infant in moderate respiratory distress. The weight was 2.6 kilograms; heart rate, 120; respiratory rate, 100. The lungs were clear to auscultation. Examination of the heart revealed a grade III/VI blowing pansystolic murmur at the lower left sternal border. St was single. The liver was palpable 3 cm. below the right costal margin. The chest x-ray showed an enlarged heart with pulmonary vascular overcirculation. An electrocardiogram (ECG) showed a QRS axis of +120”, an RsR’ in VI, and right ventricular predominance. Oxygen saturation in room air was 90 per cent. The infant showed no improvement on a maximal cardiotonic regimen. Three days following admission cardiac catheterization and selective right ventricular angiocardiography demonstrated total anomalous pulmonary venous drainage via the coronary sinus. Left ventricular angiocardiography was not performed. The data are summarized in Table I. Three days following the cardiovascular study, the total anomalous venous drainage was totally repaired by means of cardiopulmonary bypass, The postoperative course was characterized by recurrent pneumonia and atelectasis with Pcol’s ranging from 40.9 to 61 mm. Hg. Persistent pulmonary failure led to long-term administration of high oxygen concentrations. A lower left sterna1 border holosystolic murmur persisted. Two months postoper-
Cardiovascular
Laboratory.
Columbia-Presbyterian
Medical
Center. 622 W.
N. Y. 10032.
September, 1973
American Heart Journal
341
342
Table
Steeg, Ellis,
I. Cardiac
Am. Heart I. Sc~tember, 1973
and Gersony
catheterization
data
SW -__I
Patienl
sal.*J L. A. No. 1 No. 2
81 72
N. G.
93
R. R.
845 69
P’
M=
*Sat. = Oxygen saturation; tM = mean. $ED = End diastolic.
&.t.;p
2
85 73
97
saf./ RVp
&,t.iPAp
89 82
65/12EDt 33/3ED
88 82
65/24, 35/16,
ii ii
95
M=
511 93
102/7ED
87
85/45,
ii
85
M=
7
86/8qED
91
32/10,8
ii
tM = 6 M= 2
89
sat.;p
M = 3
sat./
Lvp
96
74/2ED
(RU/M.Y lvR
3.6 2.2
M= M=7
711 83 98
1 lO/lOED
4.4
86/-
0.9
P = pressure.
WC. /INot simultaneous. DDirect withdrawal.
atively a second catheterization was carried out, and a left-to-right shunt at the ventricular level was suspected on the basis of oxygen saturation data. The pulmonary artery pressure was only minimally increased (Table I), and pulmonary vascular resistance (PVR) was calculated at 2.2 resistance units (RU). The infant continued to do poorly over the succeeding months. Feedings were not taken well and respiratory distress continued. Despite the use of a feeding gastrostomy, the infant failed to thrive. His physical status gradually deteriorated and he died 7% months postoperatively. Postmortem examination. The heart and lungs weighed 250 grams (normal = 119 * 30). The total anomalous pulmonary venous drainage was well repaired and there was no visible obstruction to pulmonary venous return. A ventricular septal defect 0.8 cm. in diameter was found above the crista supraventricularis. The right ventricle was dilated, as was the main pulmonary artery, which was larger in diameter than the ascending aorta. The left ventricle was slightly larger than the right. Microscopic examination of the lungs revealed hypertrophy of arteries and veins with mild muscular arterial hyperplasia (Stage II Pulmonary Vascular Disease)3 as well as septal and focal interlobular fibrosis. These fibrotic changes may represent the effects of prolonged oxygen administration. Case 2. N. G., a 4-month-old Caucasian girl, was admitted to Babies Hospital for cardiac surgery. The history was unremarkable until 2% months of age, when tachypnea and increasing fatiguability were noted. A murmur compatible with the clinical diagnosis of ventricular septal defect was heard one month later. The infant was admitted to another hospital and digitalized with minimal improvement. Cardiac catheterization and angiocardiography showed total anomalous pulmonary venous drainage via a left superior vena cava. A left ventricular angiocardiogram was not done. She was then transferred to Babies Hospital.
On admission the infant was noted to be acyanotic and in mild respiratory distress. The weight was 4.1 kilograms; heart rate, 150; respiratory rate, 70. The lungs were clear to auscultation. Examination of the heart revealed a visible right ventricular impulse, a grade III/VI holosystolic murmur at the left sternal border, and a single second sound. There was no hepatosplenomegaly or peripheral edema. The ECG showed an axis of +135 with right ventricular enlargement. The T waves were upright in VI. A chest x-ray showed cardiomegaly with a wide superior mediastinum and pulmonary vascular engorgement. Because of the murmur suggestive of ventricular septal defect, the infant was recatheterized, The catheterization data confirmed total anomalous pulmonary venous drainage via the superior vena cava with pulmonary hypertension (Table I). An angiocardiogram performed with the catheter in the left ventricle demonstrated the presence of a large, high ventricular septal defect with left-to-right shunt. Two days later, an open-heart operation was performed in an attempt at total correction. In addition to the total anomalous pulmonary venous drainage to the left innominate vein, a large membranous ventricular septal defect was found. Correction of these anomalies required 80 minutes of cardiopulmonary bypass. Four hours postoperatively, the patient became severely acidotic. Multiple cardiac arrests ensued and she died eight hours postoperatively. An autopsy was not obtained. Case 3. R. R., a 2-month-old Caucasian boy, was admitted to Babies Hospital because of congestive heart failure. He was the product of an uncomplicated term pregnancy and appeared normal at birth. A heart murmur was noted at one week of age, but the infant thrived at home with good weight gain. One week prior to admission, tachypnea, fatigue with feedings, and excessive sweating were noted. Physical examination revealed a well-developed, well-nourished, acyanotic infant in moderate respiratory distress. The weight was 4.6 kilograms; heart rate, 120; respiratory rate, 90. Bilateral moist rLles
Volume Number
86 3
Total anomalous pulmonary
were heard over both posterior lung fields. Cardiac examination revealed a prominent systolic thrill at the left lower sternal border. The second sound was single. A grade IV/VI .rough holosystolic murmur was heard along the left lower sternal border. The liver was palpable 7 cm. below the right costal margin. There was no cyanosis. The peripheral pulses were strong and equal. Chest x-ray showed an enlarged heart with marked increase in prominence of the pulmonary vasculature (Fig. 1). The ECG revealed the QRS to be +llO” with right ventricular preponderance (Fig. 2). P waves in Lead II were 2.3 mm. and peaked. The infant improved following treatment with digoxin, furosemide, and oxygen. One week following admission, cardiac catheterization and right ventricular angiocardiography documented total anomalous pulmonary venous drainage to the right atrium (apparently via the coronary sinus) (Fig. 3). The catheterization data showed right ventricular hypertension and some right ventricular outflow tract obstruction within a 52 mm. peak systolic gradient between the pulmonary artery and the bodv of the right ventricle (Table I). A selective-left ventricular angiocardiogram was also performed which showed a large left-to-right shunt at the ventricular level (Fig. 4). The infant’s condition remained stable and he was discharged on digoxin three days following the procedure. He is presently doing well at home at 18 months of age.
Discussion
The reported incidence of total anomalous pulmonary venous drainage with a ventricular septal defect as the sole complicating lesion varies. Brody,4 reviewing 102 cases of anomalous pulmonary venous drainage of which 37 were total, found no case of associated ventricular septal defect. Burroughs and Edwards5 did not find a ventricular septal defect as a single associated malformation in their review of 188 casesof total anomalous pulmonary venous drainage, although 66 had other multiple major cardiac anomalies including, in many cases, the asplenia syndrome. In a review of 30 patients with total anomalous pulmonary venous drainage Gott and associates6 found six casesto have associated anomalies but none was an isolated ventricular septal defect. The extensive review of Nakib and colleagues7 and Blake and colleagues* also failed to show ventricular septal defect alone as a frequent complicating lesion. On the other hand, Bonham-Carter and associates,’ in an analysis of 75 children with total anomalous pulmonary venous drainage, found that, of 17 with major associated anomalies, four had ventricular
venous drainage with
VSD
Fig. 1. Admission chest roentgenogram showing cardiomegaly with increased
343
(Case3) vascularity.
septal defect. Our experience since 1966 consists of 19 patients with total anomalous pulmonary venous drainage, three of whom had ventricular septal defects. When total anomalous pulmonary venous drainage with pulmonary hypertension exists as an isolated lesion, we have reported in a previous publication that early surgical correction of the defect by means of cardiopulmonary bypass has been a successful method of treatment at this institution.g However, when a complicating ventricular septal defect is present, this may not be the best approach to management. Indeed, failure to diagnose the additional anomaly may prove fatal if correction of the total anomalous venous drainage alone is undertaken. In all three of these cases the diagnosis of ventricular septal defect with left-toright shunt was considered on the basis of the clinical presentation, including especially the auscultatory findings. In two of these cases, however, after the documentation of total anomalous pulmonary venous drainage during the initial catheterization, the possibility of ventricular septal defect was not further investigated-partly in view of the supposed rarity of this association. The murmurs were then attributed to tricuspid insufficiency. In retrospect, however, the holosystolic left sternal border murmurs were not caused by tricuspid insufficiency, but were secondary to the ventricular septal defects as initially suspected. In one of these cases, repeat catheterization did reveal the presence of the
344
Am. Heart I. Sefitember, 1973
Steeg, Ellis, and Gersony
Fig. 2. Admission ECG (Case 3).
Axis:
+llO”.
Right
Fig. 3. Frontal angiocardiogram (Case 3) showing late phase of pulmonary vein opacification 3 sec. following injection of radiopaque contrast material into right ventricle. The expected opacification of the left atrium is not seen. instead the pulmonary veins opacify the right atrium (RA) apparently via the coronary sinus (a~rorus).
complicating ventricular septal defect prior to the attempt at corrective surgery. In the third case, the initial diagnosis of ventricular septal defect was confirmed by a left ventricular angiocardiogram in the early phases of the catheterization, and total anomalous pulmonary venous drain-
ventricular
preponderance.
P waves
are peaked
in Lead
II.
age was not really suspected until subsequent completion of the right-sided study. Since left and right ventricular oxygen saturations are similar in total anomalous pulmonary venous drainage, oxygen saturation determinations cannot be expected to be of great value in the detection of a complicating ventricular septal defect. Oxygen saturation data, however, might have been of value in one of our patients (N. G., Case 2), in whom a consistent “step-down” in saturation was noted on entering the pulmonary artery from the right ventricle (Table I). In this instance, left heart saturations were somewhat lower than those in the right ventricle-presumably due to streaming of less saturated inferior vena caval blood into the left heart via the foramen ovale. Thus a left-to-right shunt at the ventricular level may have been paradoxically responsible for the modest fall or “step-down” of oxygen saturation in the pulmonary artery. The hemodynamic significance of a large ventricular septal defect in total anomalous pulmonary venous drainage is complex. In our cases, left-to-right shunts occurred via the ventricular septal defect. This diversion of some of the left ventricu-
V&me Nrmber
86 3
Total anomalous pulmonary
venous drainage with
VSD
345
Fig. 4. Frontal (left) and lateral (right) angiocardiograms (Case3) during injection of radiopaquecontrast materialinto the relatively small left ventricle. A substantial left-to-right shunt via a ventricular septal defect ooacifies the righht ventricle and pulmonary arteries. The right ventricular b; catheter wiihdrawal. _
lar stroke output tends to increase blood flow in the pulmonary circuit and, at the same time, reduce the systemic output. Such hemodynamics require relatively large flows from right-to-left across the interatria1 septum. If resistance in the pulmonary circuit is high, left-to-right shunting via the ventricular septal defect would be
limited, and in rare instances it might be speculated that right-to-left shunting through such a ventricular septal defect would occur, reducing pulmonary blood flow while augmenting systemic output. In the absence of a ventricular septal defect, surgical elimination of the large pulmonary
vein-right
atria1
shunt
reduces
right ventricular return and right ventricular output. The pulmonary artery pressure is correspondingly reduced even assuming no change in pulmonary vascular resistance. When a large ventricular septal defect is present, however, the reduced right ventricular output would tend to be matched by a corresponding increase in the left-toright
shunt
via
the
ventricular
septal
defect. Thus, by repair of the anomalous venous connections alone, the work of pumping a large left-to-right shunt is shifted from the right to the left ventricle. This radical change in the output requirements for the left ventricle may not be tolerated. In Case 1, the limited size of the ventricular septal defect probably prevented immediate catastrophe following repair of the total anomalous pulmonary venous drainage and death seemed unrelated to the interventricular communication.
Since
outflow tract gradient was identified
the ventricular
septum
is usually
intact in total anomalous pulmonary venous drainage, palliative pulmonary artery banding has not been considered. However, when a large ventricular septal defect complicates total anomalous pulmonary venous drainage in a critically ill infant with pulmonary hypertension and congestive heart failure, a palliative operation-banding of the pulmonary artery-might be considered. This procedure would be expected to reduce both pulmonary blood flow and pulmonary hypertension while simultaneously augmenting systemic output. Such beneficial effects are suggested by the favorable course of R. R. (Case 3), who had a moderate obstruction and pressure gradient across the pulmonary outflow tract. Summary Three patients with total anomalous pulmonary venous drainage and ventricular septal defect with left-to-right shunt are presented. The ventricular septal defect was diagnosed with left ventricular angiocardiography in two patients and confirmed on postmortem examination in the third. The complex hemodynamic implications of this combination of lesions are discussed. Pulmonary artery banding is suggested as a possible method of management in early infancy. When total anomalous pulmonary venous drainage is demonstrated in infants initially suspected of having large ventricular septal defects on the basis of auscultation, left ventricular angiocardiography is mandatory. Preoperative diagnosis of a compli-
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Steeg, Ellis, and Gersony
eating ventricular septal defect is essential to optimal management. REFERENCES Bonham-Carter, R. E., Capriles, M., and Noe, Y.: Total anomalous pulmonary venous drainage: a clinical and anatomical study of 75 children, Br. Heart J. 31:4.5, 1969. Darling, R. C., Rothney, W. B., and Craig, J. M.: Total pulmonary venous drainage into the right side of the heart, Lab. Invest. 6:44, 1957. Heath, D., and Edwards, J. E.: The pathology of hypertensive pulmonary vascular disease. A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects, Circulation 18533, 1958. Brody, H.: Drainage of the pulmonary veins into
the right side of the heart, Arch. Pathol. 33:221, 1942. 5. Burroughs, J. T., and Edwards, J. E.: Total anomalous pulmonary venous connection. AM. HEART
J. 59:913,
1960.
6. Gott, V. L., Lester, R. G., Lillehei, C. W., and Varco, R. L.: Total anomalous pulmonary return: An analysis of 30 cases, Circulation 13:543, 1956. Nakib, A., Moller, J. H., Kanjuh, V. I., and Edwards, J. E.: Anomalies of the pulmonary veins, Am. J. Cardiol. 26:77, 1967. Blake, H. A., Hall, R. J., and Manion, W. C.: Anomalous pulmonary venous return, Circulation 32:406, 1965. Gersony, W. M., Bowman, F. O., Jr., Steeg, C. N., Hayes, C. J., Jesse, M. J., and Maim, J. R.: Management of total anomalous pulmonary venous drainage in early infancy. Circulation 43 (Suppl. 1):19, 1971.