Total anomalous pulmonary venous drainage below the diaphragm

Total

Anomalous

Drainage

Pulmonary

Below

Successful Surgical R.

MADHU

MODY,

M.D., WILLIAM

the Diaphragm

Correction

J. GALLEN, Milwaukee,

Venous

M.D.

in an Infant*

and DERWARD

LEPLEY,

M.D.,

F.A.C.C.

Wisconsin

A 5 week old baby with total anomalous pulmonary venous drainage into the portal vein with pulmonary venous obstruction is described. Since medical treatment alone leads invariably to death, complete surgical correction under cardiopulmonary bypass was performed. The surgical procedure comprised an anastomosis between the anomalous pulmonary venous trunk and the left atrium, closure of the interatrial communication and ligation of the anomalous venous trunk beneath the anastomosis. After the procedure, the right ventricular pressure dropped considerably. At six months follow-up examination, the child has grown and is completely asymptomatic, and the electrocardiogram has shown regression of right ventricular hypertrophy. To our knowledge, our patient represents the first successful complete surgical repair of total anomalous pulmonary venous drainage into the portal vein.

I

T HAS BEEN WELL anomalous

DOCUMENTED~‘~ that

pulmonary

venous

The respiratory rate was 50 and the heart rate was 150/min. All the peripheral pulses were palpable and normal. The blood pressure in the right arm was 70 mm. Hg by the flush method. There was a mild degree of cyanosis of the nailbeds and slight circumoral cyanosis, which became more pronounced on crying. Palpation of the precordium revealed an overactive right ventricular impulse. On auscultation, the first heart sound was normal, the second heart sound was clearly split with the pulmonary component accentuated. The splitting behaved in a normal fashion in relation to respiration. There was a grade 2/6 ejection systolic murmur along the left sternal border. The liver was palpable 3 cm. below the right costal margin, but no bruit was heard over it.

total

drainage

a very poor prognosis in infancy. The prognosis is even worse when the drainage is below the diaphragm and most infants die before the age of six months.’ Although surgical mortality of this lesion is very high, this is the only chance for survival. On reviewing the literature, we were able to find very few successful surgical repairs of anomalous pulmonary venous drainage below the diaphragm.3-6 It is our purpose to review the case of an infant with total anomalous pulmonary venous drainage into the portal vein who underwent a successful complete surgical repair. carries

The electrocardiogram (Fig. 1) was abnormal and showed a QRS axis of +180° and right ventricular hypertrophy.

CASE HISTORY

The &.st roentgenogram showed slight cardiomegaly, but the pulmonary vascular markings were unimpressive (Fig. 2).

This was the first admission of this 5 week old male infant to the Milwaukee Children’s Hospital. He was the product of a full-term, normal delivery and the birth weight was 8 lb., 6 oz. Rapid respirations were noted shortly after birth, and a cardiac lesion was suspected one week before admission, when it was noted that the child was cyanotic and tachypneic and had failed to gain weight. On ptysical examination the infant’s weight was 7 lb.. which was below the third percentile for his age.

Cardiac catheterization was performed through the right saphenous vein (Table I). The oxygen saturations at various sites indicated a large left to right shunt at the atria1 level, where complete mixing of blood occurred. This resulted in identical saturations (82 per cent) in the right ventricle, pulmonary artery, left atrium, left ventricle and femoral artery. The

* From the Fairchild Cardiac Study Center, Milwaukee Children’s Hospital and the Marquette School of Medicine’ Milwaukee, Wise. Manuscript received September 25, 1968, accepted January 8, 1969. Address for reprints: Madhu R. Mody, M.D., Division of Pediatric Cardiology, Henry Ford Hospital, 2799 West Grand Blvd., Detroit, Mich. 48202. VOLUME

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OCTOBER

1969

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Mody et al.

576

Table I.

Site

VI Figure

V5 1.

Electrocardiogram showing

right

&i ventricular

hypertrophy. left atrium and left ventricle were entered through an atria1 septal defect. The pressure tracings indicated severe pulmonary arterial and right ventricular hypertension to systemic levels (Fig. 3). The mean pulmonary wedge pressure of 23 mm. Hg (Fig. 4) was considerably higher than the mean left atria1 pressure of only 4 mm. Hg, thereby suggesting that there was severe obstruction proximal to the left atrium. In view of the total mixing at the atria1 level, flows and resistances were not calculated. Cineangiocardiograms performed from the right, left and main pulmonary arteries and right ventricle revealed that on recirculation the dye from all pulmonary veins drained into a common vertical channel. This apparently drained into the portal vein since “flushing” of the liver and subsequently the inferior vena cava and right-sided heart circulation could be seen (Fig. 5). No dye was seen entering the left atrium. This confirmed the diagnosis of total anomalous pulmonary venous drainage into the portal system. A left ventricular injection demonstrated an intact ventricular septum and normally situated aorta. SURGICAL

Cardiac

Catheterization

01 Sat. (%)

svc IVC RA RV P.4 LA LV FA RPC

Data

Pressures (mm. Hg)

52 69

80 76 77 78 78 82

a = 4; u = 3; m = 3 81/2 82/42 m = 58 D = 5; u = 6; m = 4 80/5 80/46 m = 56 m = 23

FA = femoral artery; IVC = inferior vena cava; LA = left atrium; LV = left ventricle; m = mean; 02 Sat. = oxygen saturation; PA = pulmonary artery; RA = right atrium; RPC = right pulmonary capillary wedge; RV = right ventricle; SVC = superior vena cava. choice. The patient was given general anesthesia, and the chest was opened with a vertical sternal-splitting incision. Complete cardiopulmonary bypass was established. Examination of the heart revealed enlargement of the right atrium, right ventricle and pulmonary artery. All the pulmonary veins were isolated and looped with tapes for partial occlusion during anastomosis. The common trunk drained through the diaphragm, and this was looped with tape and later tied off completely. An anastomosis, 1 cm. in length, was made between the posterior wall of the left atrium and the anterior wall of the confluence of veins. A right atriotomy was then performed and an atria1 septal defect, 1 cm. in diameter, was closed. Pressures measured after the pump was turned off were 95/55 mm. Hg in the aorta and 45/7 in the right ventricle (Fig. 6), a considerable drop in the latter

PROCEDURE

After the baby was digitalized, it was considered that immediate total correction was the procedure of

i / i : !

:

?8

Figure

2.

Roentgenogram of chest with barium

swallow.

s

:

Figure 3. Simultaneous right ventricular and femoralarterial pressure tracings before operation. Note that the right ventricular systolic pressure was at systemic levels. THE

AMERICAN

JOURNAL

OF

CARDIOLOGY

Total Anomalous

Pulmonary

577

L’enous Drainage

.

I

. . . //,

‘ii

‘I

Figure 4. Withdrawal tracing from @nonary capillary zedge position to pulmonary artery showing that both of these were elevated. M = mean pressure in mm. Hg: PA = pulmonary artery; PC: = pulmonary capillary wedge. after operation. The chest was closed, and the infant was returned to the intensive care unit. Thepostoperative course was uncomplicated with the exception of transient atelectasis of the right upper pulmonary lobe. Since the operation, the child has developed well and is completely asymptomatic. He is now 7 months old and weighs 19 lb. The cardiac findings are normal and the electrocardiogram shows regression of right ventricular hypertrophy.

DISCUSSION Infradiaphragmatic type of total anomalous pulmonary venous drainage, a relatively rare anomaly, was first described by Ghan’ in 1916. In 1961, Lucas et al.s found 29 reported cases and described 4 of their own. Few other reports on this subject have appeared since then.4Bg The prognosis of total anomalous pulmonary venous drainage in infancy is generally poor and is

worse for the infradiaphragmatic type. Seventyfour per cent of 81 patients with total anomalous pulmonary venous drainage of all types described by Burroughs and Edwards’ died by age 1 year, most in 6 months. Eighty-five per cent of 80 patients described in the series of Darling et al.* were dead in less than 2 years. None of those with the infradiaphragmatic type survived for more than 6 months.‘,? Surgical

correction

of the infradiaphragmatic

type

has been attempted, but to our knowledge only 4 successful repairs have been reported.3-6v10 In 2 of these 4 the anomalous venous connection was with the inferior vena cava,3,5 and these cases were fully corrected. In the other 2 cases draining into the portal vein”*‘” anastomosis was made between the anomalous pulmonary \‘enous trunk and the left atrium, but

Figure 6. Figure 5. Cineangiocardiogram from pulmonary artery showing the pulmonary venous return in the common vertical channel draining into the portal vein.

VOLUME24, OCTOBER1969

ventricle

Simultaneous recordings of prtwure in the right (RV) and aorta (AO) after surgical repair showing

the right ventricu1a.r one-half the systemic.

systolic

pressure

to be less than

578

Mody et al.

the interatrial communication and the anomalous venous trunk below the anastomosis were left open. The patient that we have described thus represents the first complete case of surgical correction of total anomalous pulmonary venous drainage into the portal vein in which anastomosis between the anomalous pulmonary venous trunk and the left atrium was performed as well as closure of an interatrial communication and ligation of the anomalous pulmonary venous trunk below the anastomosis. Cooley et a1.4 reported on a similar patient who did well initially but was readmitted and died two months after operation because of stenosis of the anastomosis between the descending vein and left atrium. The reasons for poor prognosis in this group are numerous. To begin with, these infants are acutely ill with heart failure and are underdeveloped. Hemodynamically, they have severe obstruction at the pulmonary venous level or at the level of the vertical vein into which all the pulmonary venous blood is channeled to the liver.” Obstruction to the pulmonary venous drainage has been implicated9 as the basis for characteristic radiologic features with a relatively small heart associated with haziness and reticulation of both lung fields. Parsons et a1.12 pointed out that the intensification of cyanosis and pulmonary congestion with straining can be attributed to compression of the long and narrow pulmonary venous trunk by increased intraabdominal and intrathoracic pressures. The causes of death at operation in anomalous venous drainage of all types include pulmonary edema, which is the most common, sudden cardiac arrest, respiratory insufficiency, complete heart block, renal failure and cerebral edema.4 Two-stage correction for total anomalous pulmonary venous drainage of various types has been recommended by Mustard et a1.13 Pulmonary Venous Obstruction and Prognosis: Some of the causes of pulmonary venous obstruction enumerated by Elliot and Edwards” include high resistance to the flow offered by the long, relatively narrow channel, crowding of the anomalous pulmonary venous trunk as it passes into the abdomen through the esophageal hiatus of the diaphragm, and occurrence of localized stenotic lesions in the anomalous channel. Our patient had features of pulmonary venous obstruction manifested by a pulmonary capillary wedge pressure that was significantly elevated compared to the left atria1 pressure. The pulmonary arterial and right ventricular pressures, which were equal to the systemic pressure before

operation (Fig. 3), were noted to be less than one-half the systemic levels postoperatively (Fig. 6), suggesting that the obstructive element of the right-sided hypertension was relieved significantly. We presume that the obstruction in our patient occurred at the level of the common vertical vein. The successful result in our patient, we believe, is entirely because of relief of pulmonary venous obstruction which is probably the only major factor deciding the prognosis of these infants. We agree with Cooley and Hallman14 that surgery is especially urgent in infants with obstruction to the pulmonary venous return since the life span of these patients is usually measured in weeks. Medical treatment is usually effective for only a temporary period and, in most instances, surgical intervention provides the only chance for survival. The operation is best performed with the aid of cardiopulmonary bypass and in a one stage procedure comprising a good-sized anastomosis between the confluence of pulmonary veins and the left atrium, ligation of the common venous trunk draining through the diaphragm, and closure of the atria1 septal defect. ACKNOWLEDGMENT We thank Dr. William Westly, Jr., for his kind permission to present this case. REFERENCES 1. BURROUGHS,J. T. and EDWARDS, J. E. alous pulmonary

venous connection.

Total anom-

Am. Heart J.,

59:913, 1960. 2. DARLING, R. C., ROTHNEY, W. B. and CRAIG, J. M. Total

pulmonary

venous drainage

into the right

side of the heart: Report of 17 autopsied cases not associated with other major cardiovascula: anomalies. Lab. Invest., 6~44, 1957. 3. COOLEY, D. A. and BALAS, P. E.

Total anomalous pulmonary venous drainage into inferior vena cava: Report of successful surgical correction. Surgery, 51:798, 1962. 4. COOLEY, D. A., HALLMAN, G. L. and LEACHMAN, R. L. Total anomalous pulmonary venous drainage-correction with use of bypass in 62 cases.

JT Thoracic B Cardiovas. Surg., 51:88, 1966. 5. SLOAN. H.. MACKENZIE, J., MORRIS, J. D., STERN, A. Ad- SIGMANN, J.. bpen he&t su&ry in infancy. J. Thor&c N Cardiovas. Surg., 44~459, 1962. 6. JECIER, W., CHARRETTE, E. and COBELL, A. R. C. Infradiaphragmatic anomalous pulmonary venous drainage, normal hemodynamics following operation in infancy. Circulation, 35 ~396, 1967. 7. GHON, A. Ein Beitrag zu den Anomalien der Pulmalvenen. Beitr.path. Anat., 62~175, 1916. 8. LUCAS, R. F., ADAMS, P., ANDERSON, R. D., VARCO, R. L., EDWARDS, J. E. and LESTER, R. G. Total THE AMERICAN JOURNAL OF CARDIOLOGY

Total

Anomalous

Pulmonary

anomalous pulmonary venous obstruction. Am. J. 1961. 9. HASTREITER, A~\. R., PAUL, M. H., MOLTHAN, M. E. and MILLER, R. A. Total anomalous pulmonary venous connection with severe pulmonary venous obstruction. Circulation, 25~916, 1962. 10. WOODWARD, G. M., VINCE, D. J. and ASHMORE, P. G. Total anomalous pulmonary venous return to the portal vein: Report of a case of successful surgical treatment. J. Thoracic & Cardiovas. Surg.,

Hoentgenol., 86:561,

Venous

Drainage

579

pulmonary venom connection to the left innominate vein (Edit.). Circulation, 25:913, 1962. 12. PARSONS, H. G., PURDY, A. and JESSUP, B. r2nomalies of the pulmonary veins and their surgical significance. Pediatrics, 9:152, 1952. 13. MUSTARD, W. T., KEITH, J. D. and TRUSLER, G. A. Two-stage correction for total anomalous pulmonary venous drainage in childhood. J. Thor&c &

Cardiouas. Surg., 44 ~477, 1962. 14. COOLEY, D. A. and HALLMAN, G. L.

Criteria for recommending surgery in total anomalous pulmonary venous drainage. rim. J. Cardiol., 12:98,

45 1662, 1963. 11. ELLIOT, L. P. and EDWARDS, J. E. The problem of pulmonary venous obstruction in total anomalous

1963.

DRUGS IN USE 5%Ol-oO Renal-ActingDiuretic

440 DiureticsOther Design

Expwimwtol

lcohol-withdrawalstate, acute. Hospitalized

adulta

Dosage: 80 mg. q.d. Route:

oral

Duration:

2652

Results wan total bodv water deNone observed crease from 6& to 532 ml./ kg. Gvated liver function Plasma Na, K, Cl, Mg & eryth tests signif. reduced rocyte electrolyte cont.: after treat. avg. values within normal Jo drug-related hematoReason for Drw Use: iuretic, in overhydration of ran.@?before & after theraR logic abnormality. acute alcohol withdrawal. Post-treatmentvalues: -Red cell Na sianif. decreased. " Red cell K signif. increased. Extracellular fluid vol. decreased. Urinary output increased markedly. Urinary ele~ia_:gw sxcretio increased; max increase durin fgzt $t hrs. Q fil"fzrl. Comnent II ...diuresis of the...ncutely withdrawn may be a useful therapeutic adjunct and may expedite the detoxification procedure."

Nalea No. Patients

Age:

6/69 LASIX

u 2321/148 Furosemide

3 days

Concomitant Therapy: Hydroxyzine pamoat. Diphenylhydantoin Statistical Study

Knott, D.H. & Heard, J.D. (Tennessee Peychiat. 110s~.& U. Tennessee Coil. Basic Med. Sci., Memphis, Term) A diuretic apPxwach to acute withdrawal from alcohol. Southern hed. J. 62:485-489 (Ap.) 1969

Prepared VOLUME

24,

by Paul

OCTOBER

tie Haen,

1969

Inc. for the drug index

card system,

“de Haen

Drugs in Use.”