PEDIATRIC CARDIOLOGY .
.
.
.
.
.
.
.
.
.
.
.
.
.
Use of Contrast Echocardiography in the Diagnosis of Partial Anomalous Pulmonary Venous Connection
DELORES DANILOWICZ, MD, FACC* ITZHAK KRONZON, MD t
New York, New York
From the Departments of Pediatrics* and Medicine, ~rNew York University Medical Center, 550 First Avenue, New York, New York 10016. Manuscript received July 25, 1978; revised manuscript received August 22, 1978, accepted September 1, 1978. Address for reprints: Delores Danilowicz, MD, University Hospital, H-617, 560 First Avenue, New York, New York 10016.
248
Contrast echocardiography is useful in diagnosing the presence of partial anomalous pulmonary venous connection, particularly when it occurs in association with an atrial septal defect. Injections of saline solution, Cardiogreen or the patient's own blood were made in the inferior vena cava, left atrium and pulmonary veins of 27 patients. In all patients, the inferior vena caval injections showed only right heart clouding with no "evidence of a right to left shunt. In 21 patients, the left atrial and pulmonary venous injections showed clouding of both the right and left heart structures, indicating a left to right atrial shunt and normal pulmonary venous connection. In six patients, the left atrial injections showed right and left heart clouding, but the right pulmonary venous injections showed only right heart structures, indicating a left to right atrial shunt with partial anomalous pulmonary venous connection. Contrast echocardiography proved to be a sensitive method of diagnosing the anomalous venous connection without the use of dye curves. The method is not useful when a right to left atrial shunt is present and may show false positive results for partial anomalous pulmonary venous connection if left atrial visualization is not adequate during injection into the pulmonary vein.
Partial anomalous pulmonary venous connection is seen in a large proportion of patients with a sinus venosus atrial septal defect; it occurs less commonly in association with other congenital anomalies and is rarely an isolated defect. 1-5 The differential diagnosis of partial anomalous pulmonary venous return (connection versus drainage) is often difficult w h e n an atrial septal defect is present. 6 In most patients with the latter defect, the pulmonary veins return normallyto the left atrium but can selectively stream into the right atrium (that is, partial anomalous pulmonary venous drainage). This is particularly true of the right pulmonary veins, which drain close to the septum and may empty almost directly into the right atrium when the posterior atrial septum is minimal or absent. In some patients with an atrial defect, most frequently those with a sinus venosus type, there are pulmonary veins that return to the right atrium or superior vena cava (that is, partial anomalous pulmonary venous connection). Again, the right upper and right middle pulmonary veins are usually those that connect anomalously. When an atrial septal defect is present, the passage of the catheter into a pulmonary vein (specifically into a right pulmonary vein) is often accomplished by crossing the atrial defect when the approach is from the femoral vein. The catheter often appears to have entered the vein directly from the right atrium, and the question of partial anomalous pulmonary venous return is raised. If the pressures differ in the two atria, then the pressure in the pulmonary vein will indicate the site of connection. However, many patients with an atrial defect will have similar, if not identical, pressures in the right and left atria. Because of the left to right
February1979 The American Journal of CARDIOLOGY Volume43
PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION--DANILOWICZ AND KRONZON
atrial s h u n t and the proximity of the right p u l m o n a r y •veins to the atrial septum, an injection of contrast material into the p u l m o n a r y vein m a y show major opacification of the right a t r i u m without obvious contrast agen t in the left atrium. Dye-dilution curves f r o m t h e pulmonary vein, left atrium and right atrium may define the lesion, 7's b u t the technique is tedious, requires an arterial line and m a y be difficult or impossible to perform in a smallchild. Because cardiac catheterization of a p a t i e n t with an atrial septal defect is often completed without a retrograde arterial catheterization, the use of dye curves and an arterial l i n e w o u l d add to the morbidity of the procedure. Also, with dye-dilution curves, the sampling site is well downstream from the site of the s h u n t and venous return. Partial anomalous p u l m o n a r y venous drainage and partial anomalous pulmonary venous connection w o u l d t h e r e f o r e look similar. Because the echocardiographic technique can visualize the left atrium directly, it should be a more sensitive m e t h o d for separating these entities. T h e use of contrast echocardiography requires minimal time and effort with no increase in m o r b i d i t y to the patient. Method
Contrast echocardiography: M mode echocardiography was performed during routine cardiac catheterization. An Ekoline 20A echoCardiograph with an 1856 Honeywell strip chart recorder was used with a paper speed of 25 to 50 mm/sec. A 2.25 megahertz nonfocused transducer was positioned at the third or fourth intercostal space along the left sternal border. The right ventricle, ventricular septum and left ventricle with the mitral valve were visualized and recorded. When necessary, the view showing the right ventricular outflow tract, aortic root and left atrium was also used. With a catheter positioned in the pulmonary vein, left atrium or inferior vena cava, 5 to 10 cc of Cardiogreen®, normal saline solution or the patient's own blood was injected rapidly by hand. The microcavitation caused by this maneuver is seen as a heavy density cloud in the echocardiogram, and the clouding follows the direction of blood flow. 9-11 In the patients studied, if a right to left shunt was evident from the inferior vena cavaI injection (clouding of the left atrium, l e f t ventricle and aorta), no distinction between partial anomalous pulmonary venous drainage and partial anomalous pulmonary connection" could be made. Bidirectional shunting at the atrial level would cause clouding of both the right and left heart chambers during the left atrial and pulmonary venous injections as well as the inferior vena caval injection. The site of pulmonary venous connection could therefore not be defined. In patients who had no evidence of a right to left shunt at the atrial level from an inferior vena caval injection (clouding in the right ventricle or right ventricular outflow tract only), the pulmonary venous and left atrial injections were also reCorded. If the injections filled both the left and rightheart chambers, it was concluded that the pulmonary veins connected normally and that "a left to right atrial shunt was present. If the combined right and left ventricular view is recorded With echocardiography during the pulmonary venous injection and shows left ventricular filling through the mitral valve, a normal pulmonary venous connection is indicated because a right to left atrial shunt has been ruled out. However, if the right and left ventricular view shows only right ventricular filling from a pulmonary venous injection, the view
showing the right ventricular outflow tract, aorta and left atrium must also be obtained. Partial anomalous pulmonary venous drainage can decompress quickly into the right atrium so that no detectable clouding enters the left ventricle, but left atrial clouding should still be seen. A misdiagnosis of partial anomalous pulmonary venous connection was made in one adult patient when only the right and left ventricular view was recorded, during the injections. At operation, a large secundum atrial defect was found with a remnant of the posterior septum present and a normal pulmonaryvenous connection. Although we may find in the future that an occasional partial anomalous pulmonary venous drainage will still be labeled incorrectly, we think that this echocardiographic technique will be far more specific and sensitive than the dye-dilution curves, and it is certainly easier to use. 7,8 Clinical material (Table I): Twenty-seven patients (22 children and 5 adults) were found to have a left to right atrial shunt with no right to left component on oximetry. In these patients, M mode echocardiography had shown the usual pattern of right ventricular diastolic overload with paradoxic or fiat septal motion and high normal or increased right ventricular dimension.12A 3 M mode echocardiography with injections as described earlier were then performed at the time of routine cardiaccatheterization. Three additional patients, all adults, had a right to left component to the atrial shunt and were excluded from further analysis. Results
Atrial septal defect with normal p u l m o n a r y venous connection: In 21 patients (3 adults and 18 children), the left atrial and p u l m o n a r y venous injections were similar, showing b o t h right and left heart structures with the left ventricular c l o u d i n g occurring t h r o u g h the orifice of the mitral valve (Fig. 1). This confirmed the left to right atrial shunt and showed t h a t the p u l m o n a r y veins connected n o r m a l l y t o t h e left atrium. N i n e t e e n of t h e 2 1 patients u n d e r w e n t operative repair, and normal p u l m o n a r y venous connection
TABLE I Patterns of Clouding in Contrast Echocardiography Diagnosis
cases (no.)
.., inferior Vena Cava
Injection Sites Left Atrium
Pulmonary Vein
A. Atrial SeptalDefect With Left to Right Shunt ASD ASD, PAPVC
21
Right heart only
6
Right heart only
Right and _, left heart . Right and left heart .
Right and left heart APVmright heart only NPV--right and leftheart
B. Other Causes of Left to Right Shunt. PAPVC, no. ASD PFO
1 5
TAPVC
2
Right heart . o.nly / Right heart only Right and left-heart
Not entered Left heart only Left heart only
APV--right heart only Left heart only Right and left heart
APV = anomalous pulmonarY vein; ASD = atrial septal defect; NPV = normal •pulmonary vein; PAPVC = partial anomalous pulmonary venous connection; PFO -- patent foramen ovale; TAPVC -- total anomalous pulmonary venous connection. "
February 1979
The American Journal of CARDIOLOGY
Volume 43
249
PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION--DANILOWICZ AND KRONZON
ili;iiii~i ii~:': ~~:~::~ ~ ~~ d~i'~i:::::i::::~* i ':'::'~:~:~:~:~ !~~i~! !~:: .~' --~. !::::~-::~":' :'::::::::::' iii~!ili~i~ .... ~"i:!~i:~i' ~i :i''iii~ ~!iii! ~i~!ii!i~i~!i~ i'i~i!:ii!i!~! ~!i~i~iiiiiiii~ ~,!i,i!~!~!i ~i~i~i~iii~i~
:t :~
~j~i~:~i~!!i!i~, ~~~ii!i!ii~ii~i~i i~!~ii~i:!ii~iii~i~ i~ii !iii!R~::: ii~!~i~iliiiiii!i!i!iii!ii•i~!! !iiii!i•!ii!i!iiil;!il i ~:: ~ ~ :
~:~: ? ~ii
....... .... ,::~:%:~::~:
~:'~:,~~~:::~
~::'...... ~i~:~:~:
~ i i ~~ i i l
~:~: ,~, ' ~::~ :
~::~: '
~!~
,
e ~,; .....
'
.... ,- ~7:-~:~: ~ IV:-:::: ° ~i~ ~::~::~:~::~' ~:~.:;-:: ~::~::J:~:~::::~::> ~:::::~::;~:~::~:~:::~::~:::i::~!i~::~::~::~:~:::;-::~::~:~ ~:~:~::~-~:~:~:-~:~:~:
~:~:i~!i~ ~!::.:~ ...... i ~iiiiili~ ~:~::;i~i~
-,~?iiii:~
::::~: ~..... ~i~i::! ::~::: :i~~:-~-::~-~: -. .:~i:!:!~i.~..
~~ i:::~ ~:::~:::~:~::~:~:~:: ~~ii i i ~i:i i i::~:~i~:;~:i.i!i~,i:::~:: i:!i!:!::~';~::~::::~::~:!~!,~!' .~.~!;-'~i~: !~:~!;~5:!~ ~i:i!~i~i~i~:~i:-~~-~i:~~i~.~.~:i~:~J~;~::-:' ili~~!: i'~: ~~:!i.i:,.~-:::~::,~:~ : :~i'i~: :.:~:~:ii:i~;:i~
i ~::~:!:-:::~i::":.~::i ~ ::i~:!~::i::~;¥!~:.i~::ii~i i~::~i:ii:i:!i:i~:.ii~!i~:i:::!i,.::i~:~":'~i!i!i~":!~i ~i!i~:~¥.:" i!!~ .~."~ !" :-~~:~::~. :~: .~:~!i~i~i~i~i~i~!~:~!~i!~;~:~ ~ ::~~::::!~:~i~.::.!~.:~i.~: : :~i~: ~: :, ~~i-ii~:ii:~::~...~:::~:~.:.~. .~;~!i~: ~~::!-~~:~:.~~: ~:!~..:::.:.... ".-~i.:.::..i~.:.~ . •~ ~:~: :~::~:~~:~:-:~:~:v
'
.
.
~-~::~ ~
:.~:: .. .... ,..... ~ ~;i~.::..~ ~..............~.......:,... ~i:.::~....
~ ~ ::~ ~:
FIGURE 1. Echocardiograms showing the clouding pattern seen in patients with a left tO right atrial shunt and normal pulmonary venous connection. Left, injection into the inferior vena cava, proving that no right to left shunt is present (there is clouding only inthe right ventricle [RV]). Right, injection into the right upper pulmonary vein, showing opacification of the left ventricle (LV) through the mitral valve (that is, normal drainage of the pulmonary veins) as well as opacification of the right ventricle (left to right shunt).
was confirmed at operation. The remaining two patients had an atrial shunt with a pulmonary to systemic flow (Qp/Qs) ratio of less than 2 and normal pulmonary arterial pressures; operation was therefore not advised. Atrial septal defect with partial anomalous pulmonary venous connection: In six patients (two adults and four children), injections into the right uppe r or right middle pulmonary veins (or both) showed clouding of the right ventricle only. However, with left atrial injections both the left and right heart structures were visualized (Fig. 2), thus confirming the diagnosis of a left to right atrial shunt with partial anomalous pulmonary venous connection. Five of these six patients (one adult and four children) underwent operative repair. In two of these five patients (one adult and one child) the entire right lung drained into the right atrium or right atrialsuperior vena caval junction with a sinus venosus atrial defect. In two children the right upper and right middle pulmonary veins drained into the right atrium; one of these children had a sinus venosus defect and a perforated foramen ovale, the other had a high secundum atrial defect. In the fifth patient, a child, the right upper and right middle pulmonary veins drained into the right atrial superior vena caval junction with a sinus venosus defect. In four of these five patients a persistent left superior vena cava drained into the coronary sinus. The sixth patient, an adult, is asymptomatic and has refused operation. Anomalous pulmonary venous drainage in other congenital lesions: In addition to these patients with an atrial shunt, one child with a ventricular septal defect had a pulmonary Vein entered and injection showed only right ventricular clouding (that is, anomalous connection). Unlike an atrial septal defect, this defect could be diagnosed easily because no atrial communication could
250
February 1979
The
American
Journal
of
CARDIOLOGY
be crossed or demonstrated. The diagnosis was confirmed at operation. An additional seven patients had injections performed at the time of cardiac catheterization. In five children, an atrial communication was crossed at the time of cardiac catheterization although measurement of hydrogen arrival time in the right atrium ruled out a left to right shunt. Left atrial and pulmonary venous injections showed only left heart clouding, and the inferior vena caval injections showed only right ventricular clouding, as would be expected with a patent foramen ovale. Apparently, in these five children at least, the catheter position across the foramen did not distort the opening enough to allow a shunt that could be detected with the echocardiographic injection technique. Finally, two children with total anomalous pulmon~y venous connection (one into the right atrium and one into a persistent vertical vein) were evaluatedusing this technique. Injections into the inferior vena cava and pulmonary vein showed both right and left heart clouding, indicating an apparent "bidirectional" shunt that was actually only a right to left shunt because of the anatomy; left atrial injections showed only left heart clouding. Although this pattern might be seen in some patients with an atrial septal defect and pulmonary arterial hypertension (with or without partial anomalous pulmonflry venousconnection), the timing and progression of the clouding might be helpful in differential diagnosis.
Discussion To date, we have been successful in diagnosing partial anomalous pulmonary venous connection preopera-
Volume 43
PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION--DANILOWlCZ AND KRONZON
FIGURE 2. Echocardiograms obtained intwo patients (A and B) with an atrial septal defect and anomalous pulmonary venous connection of the right upper andmiddle pulmonary veins. Left panels, injections made into the anomalous pulmonary veins, revealing only filling of the right ventricle (RV) (top left,' arr0w) and right ventricular outflow tract (bottom left), thus proving the anomalous connection. Right panels, injections made into the left atrium (LA) showing entry of Clouding through the mitral valve orifice (arrow) with opacificati0n of the left (LV) and right ventricles (left to right atrial shunt).
tively withthe use of contrast echocardiography. The technique using the M mode is not useful if a right to left shunt ispresent at the atrial level. When injection into a pulmonary vein does not ~cause left ventricular clouding, then the left a t r i u m m u s t be viewed to minimize false diagnoses Of partial anomalous pulmonary venous connection. In the 22 children and 5 adults in our study, only a left to right shunt was found with both oximetry and echocardiography. This is certainly the usual physiologic finding in a child with an atrial septal defect.14 The development of pulmonary arterial hypertension Or the presence of anatomic variations that directright to left atrial shunting are 1.ess frequently seen. 1
Clinical importance of preoperative diagnosis of partial anomalous pulmonary venous drainage: Although missing t h e diagnosis of partial anomalous
pulmonary venous connection is not usually a major error because the surgeon should and usually does confirm the pulmonary venous connection at the time o f repair, the preoperative preparation of the patient or his family may be influenced by knowledge of this diagnosis. The technique of repair uses a deviating patch to reroute the anomalous pulmonary veins back to the left atrium by way of the atrial defect. 15 More atrial trauma results during this procedure than during the routine suture closure of a secundum atrial defect. Supraventricular arrhythmias and coronary sinus and junctional rhythms occur more frequently after repair of partial anomalous pulmonary venous connection than after repair of a secundum atrial septal defect. A large proportion of our patients with partial anomalouspulmonary Venous connection (4 of 5) had a persistent left superior vena cava draining to the
February 1979
The American J0urnal,of CARDIOLOGY
Volume 43
251
PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION--DANILOWICZ AND KRONZON
coronary sinus. Because this vessel may need to be cannulated at the time of operation, it is useful to know it is present. Again, advance knowledge is not imperative because the structure often makes itself known through the increased, return of blood through the coronary sinus even when the surgeon does not identify it before heart-lung bypass is instituted. An innominate vein injection or direct entry into the left superior vena cava from the coronary sinus will confirm this diagnosis and is probably worthwhile in any patient suspected of having partial anomalous pulmonary venous connection. The more complete and correct the diagnosis is preoperatively, the fewer last minute modifications of procedure will be needed in the operating room, a benefit that should appeal to both the cardiologist and the surgeon. Although there is no guarantee that this method will always correctly differentiate partial anomalous pulmonary venous drainage from partial anomalous pul,
monary venous connection, the technique does seem to be more sensitive and is certainly far less tedious than the use of dye-dilution curves. Addendum
After this paper was accepted, the adult with partial anomalous pulmonary venous connection agreed to operation, and the diagnosis was confirmed: sinus venosus atrial septal defect with right upper and right middle pulmonary veins draining into the right atrium. An additional three children had these injections, with one showing partial anomalous pulmonary venous connection. All three were operated on and the diagnoses confirmed: secundum atrial septal defect and normal pulmonary venous connection (two patients) and sinus venosus atrial septal defect with right upper and middle pUlmonary veins draining into the right atrium (one patient).
References 1. Bedford DE: The anatomical types of atrial septal defect: their incidence and clinical diagnosis. Am J Cardiol 6:568-574, 1960 2. Gotsman MS, Astley P, Parsons CG: Partial anomalous pulmonary venous drainage in association with atrial septal defect. Br Heart J 27:566-571, 1965 3. Brock R, Ross DN: The sinus venosus type of atrial septal defect. Guys Hosp Rep 108:291-304, 1959 4. Hancock EW: Coronary sinus rhythm in sinus venosus defect and persistent left superior vena cava. Am J Cardiol 14:608-615, 1964 5. Morrow AG, Awe WC, Aygen MM: Total unilateral anomalous pulmonary venous connection with intact atrial septum. Am J Cardiol 9:933-937, 1962 6. Perloff JK: Clinical Recognition of Congenital Heart Disease. Philadelphia, WB Saunders, 1970, p 220 7. Swan HJC, Kirklin JW, Becu LM, Wood EH: Anomalous connection of right pulmonary veins to superior vena cava with interatrial communications: hemodynamic data in eight cases. Circulation 16:54-66, 1957 8. Swan HJC, Burchell HB, Wood EH: Differential diagnosis at cardiac catheterization of anomalous pulmonary venous drainage related
252
February 1979
The American Journal of CARDIOLOGY
to atrial septal defects or abnormal venous connections. Proc Staff Meet. Mayo Clin 28:452-462, 1953 9. Hagler DJ: The utilization of echocardiography in the differential diagnosis of cyanosis in the neonate. Mayo Clin Proc 51:143-158, 1976 10. Seward JB, Tajik AJ, Hagler Da, Rifler DG: Peripheral venous • contrast echocardiography. Am J Cardiol 39:202-212, 1977 11. •Seward JB, Tajik AJ, Spangler JG, Ritter DG: Echocardiographic contrast•studies. Mayo Clin Proc 50: t63-192, 1975 12. Tajik.AJ: Echocardiographic pattern,of right.ventricular diastolic overload in children•. Circulation 46:36-43, 1972 13. Diamond MA, Dillon JC, Haine CL, Chang S, Feigenbaum H: Echocardiographic features of atrial septal defect. Circulation 43:129-135, 1971 14. Swan HJC, Burchell HB, Wood EH: The presence of venoarterial shur~ts in patients with interatrial, communications. Circulation 10:705-713, 1954 15. Reed GE, Clauss RH, Tice DA: Correction of anomalous pulmonary venous drainage to the superior vena cava. Surg Gyn Obs 115: 770-771, 1962
Volume 43
.
.
.
.
.
.
.
.
.
.
.
.
.
Use of Contrast Echocardiography in the Diagnosis of Partial Anomalous Pulmonary Venous Connection
DELORES DANILOWICZ, MD, FACC* ITZHAK KRONZON, MD t
New York, New York
From the Departments of Pediatrics* and Medicine, ~rNew York University Medical Center, 550 First Avenue, New York, New York 10016. Manuscript received July 25, 1978; revised manuscript received August 22, 1978, accepted September 1, 1978. Address for reprints: Delores Danilowicz, MD, University Hospital, H-617, 560 First Avenue, New York, New York 10016.
248
Contrast echocardiography is useful in diagnosing the presence of partial anomalous pulmonary venous connection, particularly when it occurs in association with an atrial septal defect. Injections of saline solution, Cardiogreen or the patient's own blood were made in the inferior vena cava, left atrium and pulmonary veins of 27 patients. In all patients, the inferior vena caval injections showed only right heart clouding with no "evidence of a right to left shunt. In 21 patients, the left atrial and pulmonary venous injections showed clouding of both the right and left heart structures, indicating a left to right atrial shunt and normal pulmonary venous connection. In six patients, the left atrial injections showed right and left heart clouding, but the right pulmonary venous injections showed only right heart structures, indicating a left to right atrial shunt with partial anomalous pulmonary venous connection. Contrast echocardiography proved to be a sensitive method of diagnosing the anomalous venous connection without the use of dye curves. The method is not useful when a right to left atrial shunt is present and may show false positive results for partial anomalous pulmonary venous connection if left atrial visualization is not adequate during injection into the pulmonary vein.
Partial anomalous pulmonary venous connection is seen in a large proportion of patients with a sinus venosus atrial septal defect; it occurs less commonly in association with other congenital anomalies and is rarely an isolated defect. 1-5 The differential diagnosis of partial anomalous pulmonary venous return (connection versus drainage) is often difficult w h e n an atrial septal defect is present. 6 In most patients with the latter defect, the pulmonary veins return normallyto the left atrium but can selectively stream into the right atrium (that is, partial anomalous pulmonary venous drainage). This is particularly true of the right pulmonary veins, which drain close to the septum and may empty almost directly into the right atrium when the posterior atrial septum is minimal or absent. In some patients with an atrial defect, most frequently those with a sinus venosus type, there are pulmonary veins that return to the right atrium or superior vena cava (that is, partial anomalous pulmonary venous connection). Again, the right upper and right middle pulmonary veins are usually those that connect anomalously. When an atrial septal defect is present, the passage of the catheter into a pulmonary vein (specifically into a right pulmonary vein) is often accomplished by crossing the atrial defect when the approach is from the femoral vein. The catheter often appears to have entered the vein directly from the right atrium, and the question of partial anomalous pulmonary venous return is raised. If the pressures differ in the two atria, then the pressure in the pulmonary vein will indicate the site of connection. However, many patients with an atrial defect will have similar, if not identical, pressures in the right and left atria. Because of the left to right
February1979 The American Journal of CARDIOLOGY Volume43
PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION--DANILOWICZ AND KRONZON
atrial s h u n t and the proximity of the right p u l m o n a r y •veins to the atrial septum, an injection of contrast material into the p u l m o n a r y vein m a y show major opacification of the right a t r i u m without obvious contrast agen t in the left atrium. Dye-dilution curves f r o m t h e pulmonary vein, left atrium and right atrium may define the lesion, 7's b u t the technique is tedious, requires an arterial line and m a y be difficult or impossible to perform in a smallchild. Because cardiac catheterization of a p a t i e n t with an atrial septal defect is often completed without a retrograde arterial catheterization, the use of dye curves and an arterial l i n e w o u l d add to the morbidity of the procedure. Also, with dye-dilution curves, the sampling site is well downstream from the site of the s h u n t and venous return. Partial anomalous p u l m o n a r y venous drainage and partial anomalous pulmonary venous connection w o u l d t h e r e f o r e look similar. Because the echocardiographic technique can visualize the left atrium directly, it should be a more sensitive m e t h o d for separating these entities. T h e use of contrast echocardiography requires minimal time and effort with no increase in m o r b i d i t y to the patient. Method
Contrast echocardiography: M mode echocardiography was performed during routine cardiac catheterization. An Ekoline 20A echoCardiograph with an 1856 Honeywell strip chart recorder was used with a paper speed of 25 to 50 mm/sec. A 2.25 megahertz nonfocused transducer was positioned at the third or fourth intercostal space along the left sternal border. The right ventricle, ventricular septum and left ventricle with the mitral valve were visualized and recorded. When necessary, the view showing the right ventricular outflow tract, aortic root and left atrium was also used. With a catheter positioned in the pulmonary vein, left atrium or inferior vena cava, 5 to 10 cc of Cardiogreen®, normal saline solution or the patient's own blood was injected rapidly by hand. The microcavitation caused by this maneuver is seen as a heavy density cloud in the echocardiogram, and the clouding follows the direction of blood flow. 9-11 In the patients studied, if a right to left shunt was evident from the inferior vena cavaI injection (clouding of the left atrium, l e f t ventricle and aorta), no distinction between partial anomalous pulmonary venous drainage and partial anomalous pulmonary connection" could be made. Bidirectional shunting at the atrial level would cause clouding of both the right and left heart chambers during the left atrial and pulmonary venous injections as well as the inferior vena caval injection. The site of pulmonary venous connection could therefore not be defined. In patients who had no evidence of a right to left shunt at the atrial level from an inferior vena caval injection (clouding in the right ventricle or right ventricular outflow tract only), the pulmonary venous and left atrial injections were also reCorded. If the injections filled both the left and rightheart chambers, it was concluded that the pulmonary veins connected normally and that "a left to right atrial shunt was present. If the combined right and left ventricular view is recorded With echocardiography during the pulmonary venous injection and shows left ventricular filling through the mitral valve, a normal pulmonary venous connection is indicated because a right to left atrial shunt has been ruled out. However, if the right and left ventricular view shows only right ventricular filling from a pulmonary venous injection, the view
showing the right ventricular outflow tract, aorta and left atrium must also be obtained. Partial anomalous pulmonary venous drainage can decompress quickly into the right atrium so that no detectable clouding enters the left ventricle, but left atrial clouding should still be seen. A misdiagnosis of partial anomalous pulmonary venous connection was made in one adult patient when only the right and left ventricular view was recorded, during the injections. At operation, a large secundum atrial defect was found with a remnant of the posterior septum present and a normal pulmonaryvenous connection. Although we may find in the future that an occasional partial anomalous pulmonary venous drainage will still be labeled incorrectly, we think that this echocardiographic technique will be far more specific and sensitive than the dye-dilution curves, and it is certainly easier to use. 7,8 Clinical material (Table I): Twenty-seven patients (22 children and 5 adults) were found to have a left to right atrial shunt with no right to left component on oximetry. In these patients, M mode echocardiography had shown the usual pattern of right ventricular diastolic overload with paradoxic or fiat septal motion and high normal or increased right ventricular dimension.12A 3 M mode echocardiography with injections as described earlier were then performed at the time of routine cardiaccatheterization. Three additional patients, all adults, had a right to left component to the atrial shunt and were excluded from further analysis. Results
Atrial septal defect with normal p u l m o n a r y venous connection: In 21 patients (3 adults and 18 children), the left atrial and p u l m o n a r y venous injections were similar, showing b o t h right and left heart structures with the left ventricular c l o u d i n g occurring t h r o u g h the orifice of the mitral valve (Fig. 1). This confirmed the left to right atrial shunt and showed t h a t the p u l m o n a r y veins connected n o r m a l l y t o t h e left atrium. N i n e t e e n of t h e 2 1 patients u n d e r w e n t operative repair, and normal p u l m o n a r y venous connection
TABLE I Patterns of Clouding in Contrast Echocardiography Diagnosis
cases (no.)
.., inferior Vena Cava
Injection Sites Left Atrium
Pulmonary Vein
A. Atrial SeptalDefect With Left to Right Shunt ASD ASD, PAPVC
21
Right heart only
6
Right heart only
Right and _, left heart . Right and left heart .
Right and left heart APVmright heart only NPV--right and leftheart
B. Other Causes of Left to Right Shunt. PAPVC, no. ASD PFO
1 5
TAPVC
2
Right heart . o.nly / Right heart only Right and left-heart
Not entered Left heart only Left heart only
APV--right heart only Left heart only Right and left heart
APV = anomalous pulmonarY vein; ASD = atrial septal defect; NPV = normal •pulmonary vein; PAPVC = partial anomalous pulmonary venous connection; PFO -- patent foramen ovale; TAPVC -- total anomalous pulmonary venous connection. "
February 1979
The American Journal of CARDIOLOGY
Volume 43
249
PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION--DANILOWICZ AND KRONZON
ili;iiii~i ii~:': ~~:~::~ ~ ~~ d~i'~i:::::i::::~* i ':'::'~:~:~:~:~ !~~i~! !~:: .~' --~. !::::~-::~":' :'::::::::::' iii~!ili~i~ .... ~"i:!~i:~i' ~i :i''iii~ ~!iii! ~i~!ii!i~i~!i~ i'i~i!:ii!i!~! ~!i~i~iiiiiiii~ ~,!i,i!~!~!i ~i~i~i~iii~i~
:t :~
~j~i~:~i~!!i!i~, ~~~ii!i!ii~ii~i~i i~!~ii~i:!ii~iii~i~ i~ii !iii!R~::: ii~!~i~iliiiiii!i!i!iii!ii•i~!! !iiii!i•!ii!i!iiil;!il i ~:: ~ ~ :
~:~: ? ~ii
....... .... ,::~:%:~::~:
~:'~:,~~~:::~
~::'...... ~i~:~:~:
~ i i ~~ i i l
~:~: ,~, ' ~::~ :
~::~: '
~!~
,
e ~,; .....
'
.... ,- ~7:-~:~: ~ IV:-:::: ° ~i~ ~::~::~:~::~' ~:~.:;-:: ~::~::J:~:~::::~::> ~:::::~::;~:~::~:~:::~::~:::i::~!i~::~::~::~:~:::;-::~::~:~ ~:~:~::~-~:~:~:-~:~:~:
~:~:i~!i~ ~!::.:~ ...... i ~iiiiili~ ~:~::;i~i~
-,~?iiii:~
::::~: ~..... ~i~i::! ::~::: :i~~:-~-::~-~: -. .:~i:!:!~i.~..
~~ i:::~ ~:::~:::~:~::~:~:~:: ~~ii i i ~i:i i i::~:~i~:;~:i.i!i~,i:::~:: i:!i!:!::~';~::~::::~::~:!~!,~!' .~.~!;-'~i~: !~:~!;~5:!~ ~i:i!~i~i~i~:~i:-~~-~i:~~i~.~.~:i~:~J~;~::-:' ili~~!: i'~: ~~:!i.i:,.~-:::~::,~:~ : :~i'i~: :.:~:~:ii:i~;:i~
i ~::~:!:-:::~i::":.~::i ~ ::i~:!~::i::~;¥!~:.i~::ii~i i~::~i:ii:i:!i:i~:.ii~!i~:i:::!i,.::i~:~":'~i!i!i~":!~i ~i!i~:~¥.:" i!!~ .~."~ !" :-~~:~::~. :~: .~:~!i~i~i~i~i~i~!~:~!~i!~;~:~ ~ ::~~::::!~:~i~.::.!~.:~i.~: : :~i~: ~: :, ~~i-ii~:ii:~::~...~:::~:~.:.~. .~;~!i~: ~~::!-~~:~:.~~: ~:!~..:::.:.... ".-~i.:.::..i~.:.~ . •~ ~:~: :~::~:~~:~:-:~:~:v
'
.
.
~-~::~ ~
:.~:: .. .... ,..... ~ ~;i~.::..~ ~..............~.......:,... ~i:.::~....
~ ~ ::~ ~:
FIGURE 1. Echocardiograms showing the clouding pattern seen in patients with a left tO right atrial shunt and normal pulmonary venous connection. Left, injection into the inferior vena cava, proving that no right to left shunt is present (there is clouding only inthe right ventricle [RV]). Right, injection into the right upper pulmonary vein, showing opacification of the left ventricle (LV) through the mitral valve (that is, normal drainage of the pulmonary veins) as well as opacification of the right ventricle (left to right shunt).
was confirmed at operation. The remaining two patients had an atrial shunt with a pulmonary to systemic flow (Qp/Qs) ratio of less than 2 and normal pulmonary arterial pressures; operation was therefore not advised. Atrial septal defect with partial anomalous pulmonary venous connection: In six patients (two adults and four children), injections into the right uppe r or right middle pulmonary veins (or both) showed clouding of the right ventricle only. However, with left atrial injections both the left and right heart structures were visualized (Fig. 2), thus confirming the diagnosis of a left to right atrial shunt with partial anomalous pulmonary venous connection. Five of these six patients (one adult and four children) underwent operative repair. In two of these five patients (one adult and one child) the entire right lung drained into the right atrium or right atrialsuperior vena caval junction with a sinus venosus atrial defect. In two children the right upper and right middle pulmonary veins drained into the right atrium; one of these children had a sinus venosus defect and a perforated foramen ovale, the other had a high secundum atrial defect. In the fifth patient, a child, the right upper and right middle pulmonary veins drained into the right atrial superior vena caval junction with a sinus venosus defect. In four of these five patients a persistent left superior vena cava drained into the coronary sinus. The sixth patient, an adult, is asymptomatic and has refused operation. Anomalous pulmonary venous drainage in other congenital lesions: In addition to these patients with an atrial shunt, one child with a ventricular septal defect had a pulmonary Vein entered and injection showed only right ventricular clouding (that is, anomalous connection). Unlike an atrial septal defect, this defect could be diagnosed easily because no atrial communication could
250
February 1979
The
American
Journal
of
CARDIOLOGY
be crossed or demonstrated. The diagnosis was confirmed at operation. An additional seven patients had injections performed at the time of cardiac catheterization. In five children, an atrial communication was crossed at the time of cardiac catheterization although measurement of hydrogen arrival time in the right atrium ruled out a left to right shunt. Left atrial and pulmonary venous injections showed only left heart clouding, and the inferior vena caval injections showed only right ventricular clouding, as would be expected with a patent foramen ovale. Apparently, in these five children at least, the catheter position across the foramen did not distort the opening enough to allow a shunt that could be detected with the echocardiographic injection technique. Finally, two children with total anomalous pulmon~y venous connection (one into the right atrium and one into a persistent vertical vein) were evaluatedusing this technique. Injections into the inferior vena cava and pulmonary vein showed both right and left heart clouding, indicating an apparent "bidirectional" shunt that was actually only a right to left shunt because of the anatomy; left atrial injections showed only left heart clouding. Although this pattern might be seen in some patients with an atrial septal defect and pulmonary arterial hypertension (with or without partial anomalous pulmonflry venousconnection), the timing and progression of the clouding might be helpful in differential diagnosis.
Discussion To date, we have been successful in diagnosing partial anomalous pulmonary venous connection preopera-
Volume 43
PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION--DANILOWlCZ AND KRONZON
FIGURE 2. Echocardiograms obtained intwo patients (A and B) with an atrial septal defect and anomalous pulmonary venous connection of the right upper andmiddle pulmonary veins. Left panels, injections made into the anomalous pulmonary veins, revealing only filling of the right ventricle (RV) (top left,' arr0w) and right ventricular outflow tract (bottom left), thus proving the anomalous connection. Right panels, injections made into the left atrium (LA) showing entry of Clouding through the mitral valve orifice (arrow) with opacificati0n of the left (LV) and right ventricles (left to right atrial shunt).
tively withthe use of contrast echocardiography. The technique using the M mode is not useful if a right to left shunt ispresent at the atrial level. When injection into a pulmonary vein does not ~cause left ventricular clouding, then the left a t r i u m m u s t be viewed to minimize false diagnoses Of partial anomalous pulmonary venous connection. In the 22 children and 5 adults in our study, only a left to right shunt was found with both oximetry and echocardiography. This is certainly the usual physiologic finding in a child with an atrial septal defect.14 The development of pulmonary arterial hypertension Or the presence of anatomic variations that directright to left atrial shunting are 1.ess frequently seen. 1
Clinical importance of preoperative diagnosis of partial anomalous pulmonary venous drainage: Although missing t h e diagnosis of partial anomalous
pulmonary venous connection is not usually a major error because the surgeon should and usually does confirm the pulmonary venous connection at the time o f repair, the preoperative preparation of the patient or his family may be influenced by knowledge of this diagnosis. The technique of repair uses a deviating patch to reroute the anomalous pulmonary veins back to the left atrium by way of the atrial defect. 15 More atrial trauma results during this procedure than during the routine suture closure of a secundum atrial defect. Supraventricular arrhythmias and coronary sinus and junctional rhythms occur more frequently after repair of partial anomalous pulmonary venous connection than after repair of a secundum atrial septal defect. A large proportion of our patients with partial anomalouspulmonary Venous connection (4 of 5) had a persistent left superior vena cava draining to the
February 1979
The American J0urnal,of CARDIOLOGY
Volume 43
251
PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION--DANILOWICZ AND KRONZON
coronary sinus. Because this vessel may need to be cannulated at the time of operation, it is useful to know it is present. Again, advance knowledge is not imperative because the structure often makes itself known through the increased, return of blood through the coronary sinus even when the surgeon does not identify it before heart-lung bypass is instituted. An innominate vein injection or direct entry into the left superior vena cava from the coronary sinus will confirm this diagnosis and is probably worthwhile in any patient suspected of having partial anomalous pulmonary venous connection. The more complete and correct the diagnosis is preoperatively, the fewer last minute modifications of procedure will be needed in the operating room, a benefit that should appeal to both the cardiologist and the surgeon. Although there is no guarantee that this method will always correctly differentiate partial anomalous pulmonary venous drainage from partial anomalous pul,
monary venous connection, the technique does seem to be more sensitive and is certainly far less tedious than the use of dye-dilution curves. Addendum
After this paper was accepted, the adult with partial anomalous pulmonary venous connection agreed to operation, and the diagnosis was confirmed: sinus venosus atrial septal defect with right upper and right middle pulmonary veins draining into the right atrium. An additional three children had these injections, with one showing partial anomalous pulmonary venous connection. All three were operated on and the diagnoses confirmed: secundum atrial septal defect and normal pulmonary venous connection (two patients) and sinus venosus atrial septal defect with right upper and middle pUlmonary veins draining into the right atrium (one patient).
References 1. Bedford DE: The anatomical types of atrial septal defect: their incidence and clinical diagnosis. Am J Cardiol 6:568-574, 1960 2. Gotsman MS, Astley P, Parsons CG: Partial anomalous pulmonary venous drainage in association with atrial septal defect. Br Heart J 27:566-571, 1965 3. Brock R, Ross DN: The sinus venosus type of atrial septal defect. Guys Hosp Rep 108:291-304, 1959 4. Hancock EW: Coronary sinus rhythm in sinus venosus defect and persistent left superior vena cava. Am J Cardiol 14:608-615, 1964 5. Morrow AG, Awe WC, Aygen MM: Total unilateral anomalous pulmonary venous connection with intact atrial septum. Am J Cardiol 9:933-937, 1962 6. Perloff JK: Clinical Recognition of Congenital Heart Disease. Philadelphia, WB Saunders, 1970, p 220 7. Swan HJC, Kirklin JW, Becu LM, Wood EH: Anomalous connection of right pulmonary veins to superior vena cava with interatrial communications: hemodynamic data in eight cases. Circulation 16:54-66, 1957 8. Swan HJC, Burchell HB, Wood EH: Differential diagnosis at cardiac catheterization of anomalous pulmonary venous drainage related
252
February 1979
The American Journal of CARDIOLOGY
to atrial septal defects or abnormal venous connections. Proc Staff Meet. Mayo Clin 28:452-462, 1953 9. Hagler DJ: The utilization of echocardiography in the differential diagnosis of cyanosis in the neonate. Mayo Clin Proc 51:143-158, 1976 10. Seward JB, Tajik AJ, Hagler Da, Rifler DG: Peripheral venous • contrast echocardiography. Am J Cardiol 39:202-212, 1977 11. •Seward JB, Tajik AJ, Spangler JG, Ritter DG: Echocardiographic contrast•studies. Mayo Clin Proc 50: t63-192, 1975 12. Tajik.AJ: Echocardiographic pattern,of right.ventricular diastolic overload in children•. Circulation 46:36-43, 1972 13. Diamond MA, Dillon JC, Haine CL, Chang S, Feigenbaum H: Echocardiographic features of atrial septal defect. Circulation 43:129-135, 1971 14. Swan HJC, Burchell HB, Wood EH: The presence of venoarterial shur~ts in patients with interatrial, communications. Circulation 10:705-713, 1954 15. Reed GE, Clauss RH, Tice DA: Correction of anomalous pulmonary venous drainage to the superior vena cava. Surg Gyn Obs 115: 770-771, 1962
Volume 43
