Pulmonary Ventilation and Perfusion Abnormalities and Ventilation Perfusion Imbalance in Children with Pulmonary Atresia or Extreme Tetralogy

of Fallot
Susan C. Dowdle, Derek G. Human,*
Department ofPaediatrics

and Michael D. Mann

ofChild Health, University ofCape Town and Red Cross War

and Child Health, Institute

Memorial Children s Hospital, Rondebosch, South Africa

Xenon-i33 lungventilation andperfusionscansweredone

preoperatively after cardiac catheterization and cineangio

cardiography in 19 children; 6 had pulmonary atresia with an intactventricular septumandhypoplastic rightventricle, 4 pulmonary atresia with associated complex univentricu lar heart, and 9 extreme Tetralogy of FalIot. The four

patientswith discrepancies inthe sizesof the left andright

pulmonary arteries on angiography had marked asymme

try of pulmonary perfusionandventilation-perfusion imbal anceon scintigraphy.Similardegreesof asymmetryand imbalance werepresentin 6 of the 15 childrenwith equal size pulmonaryvessels.Asymmetryof pulmonaryperfu
sion and ventilation-perfusion imbalance were associated with a poor prognosis.

occurrence ofresidual abnormalities ofpulmonary per fusion and ventilation-perfusion imbalance in patients who had had aortic-pulmonary shunts prior to total repair (4). There is, however, little information on the functional effects and clinical significance of asymmet neal perfusion and ventilation-perfusion imbalance at the time of surgery. It is difficult to wean some of these patients off the ventilator after surgery despite a clinically satisfactory shunt. The ventilation and perfusion lung scans we performed postoperatively on two such children showed gross asymmetry ofperfusion and ventilation-perfusion imbalance. The magnitude of the abnormalities pro
vided an adequate explanation for the ongoing need for assisted ventilation but the results were oflittle value in

J NucIMed 1990;31:12761279

nfants with pulmonary atresia and reduced pulmo nary blood flow usually require a systemic to pulmo nary artery shunt within the first year of life for imme
diate palliation and long-term development of the pul monary vascular bed. The pulmonary blood supply of

these patients is via a ductus arteriosus or segmental

broncho-pulmonary collaterals and cardiac catheteri

zation with cineangiocardiography is required to plan a shunt procedure (1). There are a number of descriptions of abnormal
anatomical development
components of the lungs

planning further management. They could have been due to a pre-existing abnormality, a complication of surgery or a combination ofboth. We decided to include ventilation and perfusion lung scans in the preoperative work-up ofinfants with pulmonary atresia as the results would be useful if postoperative investigations were needed. This paper describes the relationship between the results of the preoperative lung ventilation and perfusion scans and the outcome in 19 infants with congenital heart disease and reduced pulmonary blood flow. METHODS
Nineteen patients were studied while they were being as

ofthe respiratory and vascular

in children with congenital

sessedfor surgery.Their agesrangedfrom 7 daysto 6 yr, with

all but three being less than 9-mo-old. Cardiac catheterization and angiocardiography were performed under local anaes
thesia and sedation via the right femoral vein utilizing the

heart disease (2,3). In a study of the pulmonary perfu sion and ventilation of children who had undergone
total repair of Tetralogy of Fallot (TET) 1 to 55 mo

previously, Alderson et al. documented the frequent

percutaneous modified Seldinger technique (5).

7700Rondebosch, SouthAfrica.
* Current address: Department

Sixofthe 19patients had pulmonaryatresiawith an intact ventricularseptum and hypoplasticright ventricle(IVS),four had pulmonaryatresiawithassociatedcomplexuniventricular Received Oct.18,1989;revision accepted Feb.13,1990. For reprintscontact:ProfessorM.D.Mann,Instituteof ChildHealth, heart (UVH), and nine had extreme TET. Six of those with
of Cardiology, IWK Children's Hospital,

TET were entirely dependent upon ductal or collateral flow

Halifax, Nova Scotia, caneda.

for pulmonary perfusion.

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TheJournal

of Nuclear Medicine Vol. 3i No. 8 August 1990

Wheneverpossible,the lungventilationand perfusionscans were done immediatelyafter cardiac catheterization.In this

event, the femoral vein sheath was left in situ and the patient transferred to the adjacent nuclear medicine imaging suite. Ventilation and perfusion scans were done using a modifi
cation of the method described by Ronchetti

The angiographicand radionuclide data were compared

and the symmetry of pulmonary perfusion and degree of
ventilation-perfusion imbalance assessed. They were corre lated with the eventual outcome.

et al. (6) and RESULTS Godfreyand McKenzie(7). The modificationswere the use ofxenon-133(33Xe) in placeofnitrogen-l3 and the collection Twelve ofthe children were alive and well six months of data at 2 sec per frameinsteadof 6 sec per frame.The after discharge from hospital. Three died prior to sur 33Xe gas usedin the singlebreathventilationscansand the gery and four died in the immediate postoperative 33Xe solution in the perfusion scans was supplied by the period. None of the patients required shunt revision or Radiochemical Centre, Amersham, England. Patients were reoperation. studied lying supine above the gamma camera, breathing The age, diagnosis, angiographic appearance of the spontaneously. For the inhalation studies, a bolus of200 MBq of 33Xe gas was delivered into the nasopharynx via a nasal pulmonary arteries, degree of asymmetry of perfusion catheter at end-expiration. For the perfusion studies, a bolus (P), index of ventilation-perfusion imbalance (>.@)@ and outcome ofeach patient are given in Table of 37 MBq of 33Xedissolved in normal saline was injected score, into either the femoral vein through the sleeve ofthe catheter used for cardiac catheterization or an arm vein via an indwell ing needle. Bolus injection was achieved by flushing the cath

eters through with air or saline as appropriate. Data was recorded in a 64 x 64 matrix at two seconds a frame for 4
mm after injection. At the end of the study, each frame was

Severe hypoplasia of the left pulmonary artery was found in the four patients with pulmonary arteries which differed in size on angiography. All had marked
asymmetry of perfusion and ventilation-perfusion im

inspected and selected frames grouped to establish the margins ofthe lungs. Each lung field was divided into upper and lower zones at the level of the hilum. Using the selected individual and grouped frames, regions of interest were drawn around the upper and lower zones of each lung and background area
in the abdomen below the liver. Time-activity curves were

generated and the background curve was subtracted from each of the pulmonary curves. Ventilation and perfusion of each zone were expressed as percentages of total pulmonary ventilation and perfusion,
using the following equation: Distnbution
. . . . Peak counts Zone i

% of Zone i =

SPeak counts all Zones

x 100.

The same frame was used to measure peak counts in all

zones. The frame selected was the frame giving the peak count for the lungs as a whole. Particular care was taken to ensure that activity in the subclavian vein and the superior or inferior vena cava was excluded from measurement of peak pulmo nary activity. Indices of the symmetry of pulmonary perfusion (P), yen
tilation-perfusion imbalance (sf) and a composite

balance. Finding arteries of the same size on angiogra phy did not exclude abnormalities of this type and magnitude. They were present in 6 of the 15 children with equal-size vessels. There was a relationship between the angiographic appearance of the pulmonary arteries and the outcome in two of the three diagnostic categories. Two of the four children with pulmonary atresia and UVH had pulmonary arteries which were not the same size and both patients died. The two infants with equal-sized pulmonary arteries underwent successful shunt proce dures. Seven patients with TET had normal pulmo nary arteries on angiography. All underwent successful palliative or corrective surgery but the two children with unilateral pulmonary artery hypoplasia died, one
before surgery and the other shortly after a shunt pro

cedure was performed. This relationship between the

angiographic appearance ofthe pulmonary arteries and

outcome was not found in the children with pulmonary atresia and IVS. All six had equal-sized pulmonary
Symmetry of pulmonary perfusion and outcome

which incorporated P and sf1, were then calculated. The value of P was the absolute difference between perfusion of the left and right lungs:
l(P@@t@ + PLL PRU pRL)l,

score,arteries but three of them died shortly after surgery.

where Piu was the % perfusion of the left upper zone, PLLthe % perfusion ofthe left lower zone, PRUthe % perfusion of the
right upper zone, and PRL the % perfusion of the right lower

tended to be associated in each of the three groups. All the children with low values of P survived and most of those with high values died. Intermediate values of P, however, did not differentiate those who survived from
those who died. There was a similar relationship be

tween the index ofventilation-perfusion imbalance, @, zone. The indexof imbalance, @, wasthe sum of the factors and outcome. The patients with low values survived f@. The value of f, was determined by the absolute value of the difference between the ventilation and perfusion of zone i and those with high values died, but intermediate results were of no prognostic value. when both were expressed as percentages: The effect of combining measures of asymmetry of I(% ventilation % perfusion) I perfusion and ventilation-perfusion imbalance is shown <5 0
59.9 >10
0.5

in Table

1. With one exception,

the score clearly

The score wasthe product of P and

@.

separates the children who survived from those who died.

Lung Scintigraphy in Pulmonary Atresia Dowdle et al

1277

TABLE 1 Age in Months,Diagnosis, Angiographic Appearance of the Pulmonary Arteries,Asymmetryof Perfusion(P),

Ventilation-Perfusion Imbalance (@f), Score, and Outcome of the Patients

Age

Diagnosis

Procedur&

Angiographyt

@;fi

Score

Outcome

0.25IVSIE18118Alive4lVSMBE20240Alive0.75IVSMBE301.545Alive0.5IVSIE262.565Died0.5IVSBE26378Died2IVSNE49298Died5UVHM

. Procedure

abbreviations:

I =

Stage

repair;

full

correction;

Blalock

shunt;

MB

Modified

Blaloch

(1

1);

thoracotomy

but vessels too small for shunt procedure; and = died before surgery.
t Angiography definition: E = equal, pulmonary arteries of equal size on angiography and U = unequal, pulmonary arteries unequal

in size on angiography.

DISCUSSION
Surgical intervention is always required in patients

with pulmonary atresia and reduced pulmonary blood flow. Although immediate primary correction is ideal,
palliative procedures are usually done because the corn

poorly perfused is an additional load on a heart which has reduced cardiac reserve. Unlike angiography, 33Xe provides quantitative information on the magnitude of any asymmetry of perfusion and ventilation-perfusion imbalance. The patients with IVS and UVH fared worst in this
study, and it may be significant that all but one had
asymmetric perfusion (higher P values). IVS is typically

plexity of underlying defect or the degree of hypoplasia ofthe pulmonary vasculature, or both preclude primary
repair. Cardiac catheterization is the only definitive preoperative method of determining pulmonary artery anatomy but it is of limited value in establishing the

prognosis. If angiography shows grossly abnormal per fusion the prognosis is poor, but neither a normal angiogram nor one showing relatively minor abnor malities are necessarily associated with a successful
outcome. In general, when the results of shunt surgery

are assessed, the emphasis is on survival and pulmonary

perfusion as assessed by angiography and blood-gas analysis. Postoperative deaths are often attributed to inadequate shunt construction or an anatomically in adequate pulmonary vascular bed, and blood-gas ab normalities ascribed to the known extra-pulmonary shunt. The possibility that there may be significant ventilation-perfusion imbalance is ignored. It is not surprising that abnormal pulmonary perfusion with ventilation-perfusion imbalance is an important addi tional risk factor in a child who has congenital heart disease. The work done to perfuse lung that is not adequately ventilated and to ventilate areas that are

associated with intrauterine cardiomegaly and compres sion of the lung. Pulmonary hypoplasia has been de scribed (8). The results ofpalliative surgery in this group have generally been worse than those in Tetralogy, and frequently a second shunt procedure is required within the early postoperative period (9). Surgical intervention was not attempted in the two patients who had UVH, because of the associated complex intracardiac defects and the absence of the left pulmonary artery. Both patients who underwent shunt procedures survived. Six of the nine patients in the TET group had asso ciated pulmonary atresia and the complex nature of the pulmonary blood supply in this condition has been well
described (1). The arterial supply may come from one

or both central pulmonary arteries and there are likely to be two to five additional aorto-pulmonary collateral arteries which may or may not interconnect with the central pulmonary artery. In this setting, it is not sur
prising that significant areas of ventilation-perfusion

imbalance may exist. Postoperative studies following repair of TET have documented imbalance (4) and in

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TheJournalof NuclearMedicine Vol. 31 No. 8 August1990

patients undergoing staged repair of pulmonary atresia with TET, significant perfusion defects have been dem onstrated with areas oflung not perfused via pulmonary or systemic collaterals (10). The number of patients in each diagnostic category is small but this study shows that a score which
incorporates estimates of asymmetry of perfusion and

2. Haworth SG, Reid L. Quantitative structural study of pul monary circulation in the newborn with pulmonary atresia.
Thorax1977; 32:129133. 3. Rabinovitch M, Herrera-De Leon V, Costenda AR, Reid L. Growth and development of the pulmonary vascular bed in

patients with TetralogyofFallot with and without pulmonary

atresia. Circulation 1981; 64:12341249. 4. Alderson PA, Boonvisut S. McKnight RC, Hartman AF.

of ventilation-perfusion imbalance predicts the out come. It appears that the values of the score which carry a poor prognosis are not the same in all groups. Adjustments in the method ofcalculating this score will be needed as more patients are investigated and expe rience is gained. However, the results do show that assessment of pulmonary ventilation and perfusion are important and that awareness and definition of appar ent pulmonary abnormalities may be useful in selecting
appropriate palliative or corrective surgery. The venti lation-perfusion lung scan is a noninvasive, quick method of investigation with a low radiation dose and

Pulmonary perfusionabnormalities and ventilation-perfusion imbalance in children after total repair ofTetralogy of Fallot.
Circulation 1976; 53:332337. 5. Conti CR, Grossman W. Percutaneous approach and trans septal catheterization. In: Grossman W, ed. Cardiac cat heter ization and angiography, 2nd Edition. Philadelphia: Lea and Febinger, 1980:5356.

6. Ronchetti R, StocksJ, Freedman N, Glass H, Godfrey S. The

clinical application ofregional lung function studies in infants

and small children using 3N. Arch Dis Child 1975; 50:595 603.
7. Godfrey MD, McKenzie S. (1977) Studies of pulmonary circulation in children with heart or lung disease using 3N. Chest1977; 7l(suppl):28028l. 8. Allen L, Crawford DC, Tynan MJ. Pulmonary atresia in prenatallife. JAm CoilCardiol 1986; 8:11311136.

the results serve as a useful guide to the attending

clinician.

9. Kirklan JW, Barrat-BoyesBG. Cardiac surgery. New York:

John Wiley& Sons;1986:847848. REFERENCES

1. Macartney F, Haworth SO. Investigation ofpulmonary atresia with ventricular septal defect. In: Anderson RH, Macartney FJ, Shinebourne EA, Tynan M, eds. Paediatric cardiology. New York: Churchill Livingstone: 1983:5, 111125.

10. del Torso S, KellyMi, KalffV, StellinG, Mee RBB,Venables

AW. Non-invasive assessment of pulmonary blood supply

after staged repair of pulmonary atresia. Br Heart J 1985;

54:209214. 11. de Laval MR. McKay R, Jones M, Stark J, Macartney FJ.

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