Echocardiographic features of total anomalous pulmonary venous drainage into the coronary sinus

Echocardiographic Features of Total Anomalous Pulmonary Venous Drainage Into the Coronary Sinus

KALIM U. AZIZ, MD MILTON H. PAUL, MD, FACC SAROJA BHARATI, MD, FACC MAURICE LEV, MD, FACC KATHLEEN SHANNON Chicago, Illinois

From the Willis J. Potts Children’s Heart Center, The Children’s Memorial Hospital and ths Department of Pediatrics, Northwestern University Medical School, Chicago, itlinois and The Congenital Heart Disease Research and Training Center, Chicego, Illinois. This research was sup ported in part by The Feldstein Family Fund, The Marlene Apfelbaum Msmortal Fund and the Irving Price Foundation for Heart Research, Chicago, Illinois; and Grant HL 0760616 from the National Heart,Lung,andBhXKthlstMe.NationalInstlMes of Health, Bethesda, Maryland. Manuscript received December 27, 1977; revised manuscript received March 16, 1978, accepted March 22, 1978. Address for reprints: Kallm U. Azir, MD, The Willis J. Potts Chtldren’s Hsart Center, Dfvislon of Cardiology, The Children’s Memorial Hospital, 2300 Children’s Plaza, Chicago, Illinois 00614.

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Echocardiograms were obtained from flve infants with total anomalous pulmonary venous drainage to the coronary sinus or the portal system or the superior vena cava and from one child wlth a secundum atria1 septal defect and a large coronary slnus due to persistence of the left superior vena cava. The results demonstrate that an enlarged coronary sinus Is posltloned consistently posterior to the left atrium In approximately the same horizontal plane as the aortlc valve. Echocardlographlcally the coronary sinus can be located as an echo complex behlnd the left atrium by using the aortlc root as a reference point. The echo complex can be dlfferentlated from the other spurious echoes In the left atrium by Its characteristic phaslc motions. The addltlonal demonstratlon of the enlarged common pulmonary venous chamber behlnd the rlght atrium conflrms the dlagnosls of anomalous pulmonary venous drainage to the coronary slnus. For other types of anomalous pulmonary venous return, anatomic dlagnosls wlth single crystal M mode echocardlography Is not always possible because of the posltlonal varlablllty of the common pulmonary venous chamber In relatlon to the left atrium.

Echocardiography may be helpful in establishing the diagnosis of total anomalous pulmonary venous drainage. Indirect signs such as right ventricular volume overload, due to an enlarged right ventricle and paradoxic intraventricular septal motion,‘*2 and a small left atrium3 can be suggestive of this lesion. Recently, visualization of the common pulmonary venous chamber as an echo-free space posterior to the left atrium and bounded anteriorly by an anomalous linear echo complex has been reported* as a diagnostic anatomic feature of total anomalous pulmonary venous drainage to various segments of the systemic venous system. However, the diagnostic echocardiographic features distinguishing different types of total anomalous pulmonary venous drainage and the diagnostic difficulties of using single crystal M mode echocardiography have not been fully considered. This study presents observations on five consecutive infants with total anomalous pulmonary venous drainage to various parts of the systemic venous circuit, describes the characteristic features of total anomalous pulmonary venous return to the coronary sinus and discusses the reasons for the diagnostic difficulties we and others have encountered when using single crystal echocardiography in evaluating the supra- and infracardiac types of anomalous pulmonary venous drainage. Material

and Methods

Patients: Five infants (Table I), aged 15 hours to 39 months, with total anomalous pulmonary venous drainage to (1) the coronary sinus (three infants), (2) the portal system (one infant), or (3) the superior vena cava (one infant) were studied. In addition, an infant with a secundum atria1 septal defect and a large coronary sinus due to persistence of the left superior vena cava was studied. Echocardiograms were performed on the same day as cardiac catheterization.

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TABLE I Preoperative Hemodynamic and Echocardlographlc Data ot Five Patlents With Total Anomalous Pulmonary Venous Drainage

case1 Ae B%A(m*) site of PVD

cm cm/m2 LV (cm) RVlLV (%) LA/A0 (%) IVS motion CPVC echo localization Pulmonary valve PEP (msec) z;;RtEyc) RVDWT km1

4mo

case 2

Case 3

0.25 cs

2mo 0.18 cs

36 hr 0.21 cs

65120 (45)

55720 (30)

55/Q (24)

2.0 8.0 0.85 143 0.68 Parad. AVJ and dorsal to RA

::4 0.32 0.4

2; 0:75

::7’ 0.90

Parad. AVJ and dorsal to RA

100 0.76 Parad. AVJ and dorsal to RA

68 155 0.44 0.6

40 195 0.21 0.5

179

0.58

Case 4

Case 5

15hr 0.24 Infradiaphragmatic 80140 (60)

39 mo 0.49 LSVC

1.4

5’.: 2:oo

39/12 (23)

ZO li6 0.86 Parad. ND

108 1.00 Parad. Dorsal to RA

75 185 0.41 0.5

20 0.13 1.1

Fi ures in parentheses indicate mean pressures. A8 = aorta; APD = anteroposterior dimension: AVJ = atrioventricular junction; WA = bcdy surface area: CPVC = common pulmonary vencus chamber; CS = coronary sinus; IVS = interventricular septum; LA = left atrium; LSVC = left superior vena cava; LV = left ventricle; ND = not Fkr;i;~$ PAP = pulmonary arterial pressure; Parad. = paradoxic; PEP = preejection period; PVD = pulmonary venous drainage; RA = right ; = right ventricle; RVDWT = right ventricular diastolic wall thickness; RVET = right ventricular ejection time.

The hemodynamic, angiographic and operative findings established the anatomic diagnosis in all infants. Echocardiographic procedures: Postoperative echocardiograms were available in all. Echocardiograms were obtained using a focused 5 or 3.5 megahertz transducer, positioned at the second to fourth intercostal space, with a Smith-Kline echocardiograph interfaced with a Honeywell 1356 strip chart recorder. The left atrium was explored distally and proximally to the level of the aortic root by recording from both high (second left interspace) and low (fourth interspace) pamsternal positions. The atrioventricular (A-V) junction was viewed using multiple scans from the aorta to the left ventricle and varying the transducer angulation along the leftwardsuperior and leftward-inferior direction as well as along the long axis of the left ventricle. The right atrium was scanned by angulating the transducer to the right and superiorly from a lower left sternal position so that the right atrium would be visualized superior to the septal leaflet of the tricuspid valve. Technically satisfactory echocardiograms were obtained in all infants. The coronary sinus was visualized echocardiographically during cardiac catheterization in two infants, one with an enlarged coronary sinus due to persistent left superior vena cava and the other with total anomalous pulmonary venous return to the coronary sinus, by injecting 2 to 3 ml of 5 percent dextrose in water into the left superior vena cava and the left atrium, respectively. Measurements: The right ventricular end-diastolic anterior wall thickness and anteroposterior dimensions were measured at the time of the R wave peak of the electrocardiogram. The right ventricular dimensions were indexed to body surface areas and were also expressed as a ratio between the right and left ventricles. The left ventricular end-diastolic dimensions were measured at the level of maximal mitral valve amplitude at the time of R wave peak.6 The preejection period and the ejection time of the right ventricular systole were measured from the pulmonary valve echo, and the results expressed as a ratio.’ The anteroposterior dimensions of the left atrium and aorta were measured at end-systole and also expressed as a ratio.8

Pathologic material: The positional variability of the common pulmonary venous chamber in relation to the left atrium and the aortic root was studied in 20 postmortem heart specimens from patients with total anomalous pulmonary venous drainage who had not been surgically treated. In 13 hearts the common puhnonary vein drained to the innominate vein, in 4 to the portal system, and in 3 to the region of the coronary sinus.

Results The hemodynamic and echocardiographic data of the five patients with total anomalous pulmonary venous return are shown in Table I. Echocardiographic contrast studies in two patients with an enlarged coronary sinus: These studies, performed during cardiac catheterization, showed a space posterior to an anomalous undulating echo complex at the A-V junction behind the aortic root. This space represented the enlarged coronary sinus. The space immediately anterior to this anomalous echo represented the left atrium, which was encroached on posteriorly by the enlarged coronary sinus (Fig. 1 and 2). The anomalous echo complex itself represented the echoes from the posterior left atria1 and anterior coronary sinus walls. Echocardiographic studies in total anomalous pulmonary venous drainage: Morphologicallysimilar abnormal linear echoes were seen dorsal to the left atrium in the three patients with pulmonary venous drainage to the coronary sinus and in one patient with persistent left superior vena cava draining to the coronary sinus. These echoes were best visualizedbehind the aortic root at the A-V junction (Fig. 3). They were also well seen at a location posterior and superior to the tricuspid valve behind the right atrium in the patients with total anomalous pulmonary venous drainage to the superior vena cava or coronary sinus and they repreJuly 1978

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sented the right horn of the common pulmonary venous chamber (Fig. 4). The common pulmonary venous chamber was not detected in the one infant with anomalous pulmonary venous drainage to the portal system. Phasic movements of echo complexes: These linear echo complexes behind the aortic root and the left atrium showed characteristic anterior motions during late ventricular diastole and mid systole (Fig. 3). The diastolic anterior motion was consistently observed between the P wave and the QRS wave of the electrocardiogram at the time of atrial systole. The systolic anterior motion followed the QRS complex of the electrocardiogram and had variable amplitude. These phasic movements reflect volume changes in the coronary sinus and the left atrium. During atrial systole the left atrium contracts with anterior movement of its posterior wall; there is presystolic emptying of the coronary sinus and diminution of its volume. However, during ventricular systole, when the right atrium is being filled from the systemic veins, coronary sinus flow

ET AL.

is impeded, resulting in coronary sinus expansion and increased encroachment on the left atrium manifested in the echocardiogram by the systolic anterior motion of the anterior coronary sinus wall. Echocardiographic measurements: Right ventricular enlargement and paradoxic interventricular septal motion were noted in all patients (Table I). The left atrium and left ventricle were small in four of the five patients with anomalous pulmonary venous drainage. The systolic time intervals of the right ventricle showed an abnormally increased ratio between preejection time and right ventricular ejection time in three of the four patients with elevated pulmonary arterial pressure. Postoperative echocardiographic examinations: These studies failed to demonstrate the characteristic abnormal echo in the patients who had correction of total anomalous pulmonary venous drainage, but the echo was repeatedly observed in the patient with persistent left superior vena cava draining to the coronary sinus after repair of a secundum atrial septal defect.

FIWRE 1. Case 8. Enlarged coronary sinus due to persistent left superior vena cava. Injection of 3 ml of 5 percent dextrose in water into the left superior vena cava shows initial opacification (arrow) of the coronary sinus (CS) posterior to ths anomalous undulatingedlo(‘)locatedbshindtheacrtic root (AO) and left atrium (LA). The left atrium lies anterior to this anomalous echoandshowsnoe&ocardiogaphic contrast material. The right ventricular outflow tract (RVO) subsequently becomes heavily opacifled because of forwaKlflowfromthetightatrilMll.Note the distortion of the aortlc root due to oblique angulation.

RV FIGURE 2. Case 3. Total anomalous pulmonary venous drainage with anomalous pulmonary venous return to the coronary sinus (CS). InjectIon of 3 ml of 5 percent dextrose in water into ths left atrlun (LA) shows opackatiion of this chamber (arrow): the space posterior to the anomalous echo (‘) remains clear and indicates the pogitlon of the enlarged uxonaty sinus (‘23). A0 = aorta; RVO = right ventricular outflow tract.

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Pathologic studies: These studies showed that in the three heart specimens in which the pulmonary veins returned to the coronary sinus, the enlarged coronary sinus was in close and consistent proximity to the posterior wall of the aorta. The aortic valve and the coronary sinus were in approximately the same horizontal plane, and the coronary sinus was related to the inferior part of the posterior wall of the left atrium as in the normal heart. In the 13 heart specimens in which the pulmonary

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venous drainage entered the left superior vena cava by way of the innominate vein, the common pulmonary venous chamber lay posterior and superior to the level of the aortic valve. This chamber was directly posterior to the left atrium in four patients but was related only to the superior aspect of the left atrium in nine patients. In the four heart specimens with infradiaphragmatic pulmonary venous return, the common pulmonary venous chamber formed the upper two limbs of a Y-

1

RVO

RV

IS

A0

LV

LA cs

RVO

ET AL.

I

RV

[

S

A”[

IV

csc

cs

LAC

FIGURE 3. Scans from the aorta (AO) to ths left ventricle (LV) in Cases 1 (top), 2 (center) and 3 (bettern) with total ancmakus pulmonary venous drainage into the coronary sinus (CS) showing morphokgkally Identical ~uxM#ngechoes(‘)atthe A-V junctkn dorsal to the aortic root (AO)andbftatrhnn(LA).anteriormotknoftheanomakWecho is well seen during atrial (a) and ventricular (v) aystole. This anomalous echo m&s the enterkr wall of the

I

RVO

RV

A0 Theleftatrlumlsrepresentedbythe clear space between the comnary sinus and aortk raot. MV = mitral valWRV=rl@tventrk&RV0=ril)lt ventrkular outflow traact,S = septum; TV = tricuspid valve.

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LA

I

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RV

[ T VARA

RV TVA:

I

RAi CPVC [

FIGURE 4. Top, tricuspid vehre (TV) view in a childwithcoarctatlonof the aorta,obtainedby placing the &mxardiographic transducerat the low left parastemalregionand arigulating it 80 that the ultresonic beam is directed rightwerdand super&@. Five mf of 5 percent dextrose In water was injected into the right atrium(RA), which opaclfiedfirst with subsequentopaclflcationof the rightventricle@IV). ~~~~~~~~~~Ul~ (TVA). Con&r and bottom, similar trlcupsid valveviewincases1(culter)arKl6@dtan), with total anomalous pulmonary venous dr8inage,!&owing8n anorrelousecho complex ~~~~ ri# atrium@A) ~~~ and the commonpulmonaryvenouschamber (CPVC) posteriorly.

RV TVA-

!

RAI CPVC[

lT2

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shaped structure. This chamber was caudal to the aortic valve and lay behind the posterior wall of the left atrium in two patients and was related only to the lowermost part of the left atrium in two (Fig. 5). Discussion Echocardiographic features of enlarged coronary sinus due to anomalous pulmonary venous drainage: Our study has shown that an abnormally enlarged coronary sinus, resulting from either anomalous pulmonary venous drainage or persistence of a left superior vena cava, can be detected posterior to the left atrium at the A-V groove using the aortic valve as a reference point. This structure is further characterized by a linear echo complex with distinctive systolic and diastolic phasic motion. The pathologic material indicates that when the coronary sinus is grossly enlarged, it encroaches on the dorsal aspect of the left atrium. The characteristic coronary sinus echo complex was detected at this site in all three patients with anomalous pulmonary venous return to the coronary sinus and, additionally, the right horn of the common pulmonary venous chamber was detected at a site posterior to the right atrium. However, no such chamber was seen behind the right atrium in the patient with persistent left superior vena cava draining to the coronary sinus, and this echocardiographic finding may serve as a distinguishing feature between these two causes of an enlarged coronary sinus. The normal-sized coronary sinus does not encroach on the posterior wall of the atria and is thus not detectable with the echocardiogram in normal hearts or hearts with other congenital malformations. Spurious linear echoes of a different type, without a distinct phasic pattern, may be seen behind the aortic root in the left atrium of normal children and children with congenital heart disease.9 In our experience these echoes are indistinct and interrupted and have no consistent oscillatory pattern during the cardiac cycle. Furthermore, they are not consistently visualized at any particular site, and with proper angulation can usually be made to disappear. Total anomalous pulmonary venous drainage to sites other than the coronary sinus: This condition has not been consistently diagnosed echocardiographically by us or others. In this form of the anomaly, the pathologic material indicates considerable positional variation of the common pulmonary venous chamber with reference to the left atrium. In patients with supracardiac pulmonary venous return, the common pulmonary venous chamber was directly posterior to the

FIGURE 5. Lateral view cineangiographic tracing of the left atrium (L.A.) and the common pulmonary venous chamber (C.P.V.C.) from an infant wlth total anomakus pulmonary venous drainage to the portal system. The common pulp venous chamber lies inferior to the lower aspect of the left atrium and farther caudal to the aortk root (AO.) and would require a very oblique angulation of the ultrasonic beam (cross-bar scan position 3) to be detected. The right pulmonary veins (R.P.V.) lie to the right of the left atrium. L.P.V. = left pulmonary veins; L.V. = left ventricle: M.V. = mitral valve; T = transducer: V.V. = vertical vein.

left atrium in a few specimens but was related to its superior aspect in the majority. Thus, in most cases the common pulmonary venous chamber is near the superior aspect of the left atrium and lies far cephalad to the aortic valve: hence, a very oblique transducer angulation is required for its detection. In patients with subdiaphragmatic pulmonary venous drainage, the common pulmonary venous chamber was caudal to the aortic root and caudal to the inferior aspect of the left atrium in half of the specimens examined (Fig. 5). These variations in the relation of the common pulmonary venous chamber to the left atrium and the constraints placed on successful transducer alignment contribute to the difficulty in achieving a consistent diagnosis of this lesion with the single crystal M mode technique.

References 1. TaJlk AJ, Gau Gl, Schattenberg lT Echocardiogram in total anomalous pulmonary venous bainage. kayo Clin Proc 47:247-250, 1972 2. Goodman MJ, lham, P, Kidd BBLr Echocardiography in evaluation of the cyanotic newborn infant. Br Heart J 36:154-166, 1974 3. Meyer RA, Kaplan S: Noninvasive technique in pediatric cardiovascular disease. Prog Cardiovasc Dis 15341-367.1973 4. Paquet M, Gufgesell H: Echocardlographic features of total anomalous pulmonary venous connection. Circulation 51599-605, 1975 5. TajHc AJ, Gau GT, Rftter Do, et al: Echocardiographic pattern of right

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ventricular diastolic volume overload in children. Circulation 46: 36-43, 1972 Gekberg BJ, Allen HD, Sahn RI: Pediatric and Adolescent Echocardiography. Chicago, Year Book Medical Publishers, 1975, p 64 Hfrschfekf S, Meyer R, B&warts DC, al d: The edxxardiographlc assessment of pulmonary artery pressure and pulmonary vascular resistance. Circulation 52642-650, 1975 In Ref 6, p 70 ic Diagnosis of Congenital WlfBamRG,TuckerCRrEchocerdiograph Heart Disease, Boston, Little, Brown, 1977, p 64

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