Download Tetralogy of Fallot Associated with Total Anomalous Pulmonary

Tetralogy of Fallot Associated with Total
Anomalous Pulmonary Venous Drainage*
Alexander I . Muster, M.D., Milton H. Paul, hl.D., and
Hisashi Nikaidoh, h1.D.
A previously unreported association of tetralogy of Fallot and total anomalous
pulmonary venous drainage is described in two infants. The initial clinical manifestation was tetralogy, and both patients had systemic-pulmonary shunt surgery
to increase the pulmonary blood flow. Postoperatively, one infant died of massive
pulmonary edema, and at postmortem examination total anomalous pulmonary
venous drainage below the diaphragm was found. The other patient had the
association of tetralogy with anomalous pulmonary venous drainage into the
coronary sinus diagnosed by cardiac catheterization. Following shunt surgery,
prolonged continuous positive airway pressure was necessary to adequately ventilate the lungs, presumably the result of pulmonary venous congestion and
low pulmonary compliance. The infant eventually died from tracbeostomy complications. The diagnosis, clinical course, surgical implications and pathology
of this association are discussed.
etralogy of Fallot is not infrequently associated
Twith
other cardiac malformations.'-6 Association
with partial anomalous pulmonary venous drainage
has been reported preiiously4*5and, indeed, was
found in six of our patients with tetralogy (Table 1).
In contrast, total anomalous pulmonary venous
drainage occurring simultaneously with tetralogy
tion, 233 patients; ( 2 ) operation and autopsy, 58
patients; ( 3 ) autopsy, 22 patients; and ( 4 ) operation alone, 143 patients. It is likely that in the 143
surgically treated patients who had neither cardiac
catheterization nor autopsy, some minor associated
defects remain undiagnosed (Table 1 ) .
For editorial comment, see page 283
has not been reported in the literature. In a survey of
592 medical records of patients with tetralogy of
Fallot at The Children's Memorial Hospital, Chicago, we found two patients with this rare combination. The case reports of these two infants with
discussion of surgical
management
and of the difficulties encountered postoperatively are the subject
of this presentation.
In our survey, the presence of associated abnormalities was established by: ( 1) cardiac catheteriza'From the Divisions of Cardiology and Cardiovascular-Thoracic Surgery (Willis J. Potts Children's Heart Center), The
Children's Memorial Hospital and the Departments of Pediatrics and Surgery, Northwestern University Medical
School, Chicago
Supported in part by grant HE-05770 from the National Institutes of Health, Bethesda, Md.
hlanuscript received January 3; revision accepted January
31, 1973.
Reprit~trequests: Dr. Mrrster, 2300 Children's Plaza, Chicago
6061 4
A one-month-old white girl was admitted to The Children's Memorial Hospital Nov. 27, 1970, with symptoms of
excessive irritability, difficulty in feeding and cyanosis. A
grade 3/6 ejection systolic murmur was audible at the midleft sternal border. The second heart sound was loud and
single. The chest x-ray film showed a normal-sized heart,
wide mediastinum, normal pulmonary vascularity and a right
aortic arch. The electrocardiogram ( E C G ) showed right axis
deviation and right ventricular hypertrophy. The clinical
impression was tetralogy of Fallot. At two months of age, the
infant was readmitted (Dec. 30, 1970) because of hypoxic
spells with essentially unchanged clinical, x-ray, and ECG
findings.
At cardiac catheterization, severe pulmonary stenosis was
demonstrated. and a ventricular and atrial seutal defect was
traversed with the catheter. The mean atrial pressure was
higher in the right atrium, and bidirectional shunting at the
atrial level was demonstrated by O:! saturation data. The
aortic oxygen saturation was 68 percent and the pulmonary
blood flow was markedly diminished. A cineangiocardiogran~
of the right ventricle (Fig 1 ) disclosed classic tetralogy of
CHEST, VOL. 64, NO. 3, SEPTEMBER, 1973
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MUSTER, PAUL, NlKAlDOH
Table 1--Survey of Cardiac Malfornuatiolu in A~mciationwith Tetralogy of Fallot in 592 Patientr
Diagnosis*
No.
%
Diagnosis*
No.
Aberrant right subclavian artery
Partial anomalous pulmonary venous drainage
Persistent A-V canal
Left pulmonary artery arising from aorta
Absent left pulmonary artery
Vascular ring
Mitral stenosis
Left superior vena cava into left atrium
Interrupted inferior vena cava
Total anomalous pulmonary venous drainage
TOTALNUMBER
SURVEYED
No associated abnormalities
Right aortic arch
Pulmonary atresia
Patent ductus arteriosus ( > 1 m) **
Atrial septal defect (secundum)
Patent foramen ovale ( > 1 m) **
Left superior vena cava into
coronary sinus
Rudimentary pulmonic valve
Tricuspid stenosis
- -
*Others: obstructed ventricular septal defect 2; hypoplastic right ventricle 2; anomalous brachiocephalic vessels 2; aneurysm of
sinus of Valsalva 2; absent right pulmonary artery 1; absent left subclavian artery 1; cor triatriatum 1; abnormal
tricuspid valve 1; aortic regurgitation 1; aortic stenosis 1.
**Patients over one month of age.
t
Fallot. The venous phase of a pulmonary arteriogram (Fig 2 )
showed all the right-sided and most of the left-sided pulmonary veins draining into the coronary sinus. The left upper
pulmonary vein was connected to a persistent left vertical
vein, which drained into the innominate vein.
An ascending aorta to right pulmonary artery anastomosis
( 4 mm incision), performed when the patient was ten weeks
of age, resulted in a loud continuous murmur over the right
chest. Postoperatively, continuous positive airway pressure
(CPAP) and supplementary oxygen were required to maintain adeauate ventilation and, on the third postoperative day,
The C P a i~d Oz were
a trachistomy was
discontinued on the sixth postoperative day, but the tracheal
tube could not be removed because of copious tracheal
secretions and right vocal cord paralysis. The infant was
discharged on the 43rd postoperative day with the tracheostomy tube in place. The chest x-ray film and the electrocardiogram on the day of discharge were essentially unchanged
compared to the preoperative findings. The infant died at
home on the day following discharge from apparent upper
airway obstruction.
At autopsy, the surgical anastomosis was patent, with a
diameter of 2.5 mm. The left vertical pulmonary vein drained
the upper left lobe into the innominate vein. All other
pulmonary veins returned to the coronary sinus (Fig 3 ) . The
outilow tract of the right ventricle was narrowed by a
&CURE 1. Case 1. Cineangiocardiogram ( AP view) with injection into right ventricle ( RV ). Note typical infundibular
stenosis (PSI), early filling of aorta ( Ao), right aortic arch,
and moderately well developed main pulmonary artery (PA).
deviated parietal band as in classic tetralogy (Fig 4). The
ventricular septal defect measured 0.5 cm, and a fossa ovali.
defect measured 0.3 cm in greatest dimension. Microscopic
examination of the lung revealed bilateral generalized intraalveolar capillary congestion.
A white infant boy in whom slight cyanosis was noted
shortly after birth (Jan. 30, 1951), was admitted to The
Children's Memorial Hospital Feb. 24, 1951, at one month
of age, because of marked cyanosis. The auscultatory, x-ray,
and ECG findings were consistent with the diagnosis of
severe tetralogy of Fallot with pulmonary atresia and right
aortic arch. No murmurs were audible. At two months of age,
the right pulmonary artery and the descending aorta were
anastomosed (Potts anastomosis) through a 4 mm incision.
The cyanotic condition improved immediately. No continuous
murmur was heard postoperatively, but there was a systolic
murmur in the right subclavian area, not audible before the
operation. The murmur disappeared 12 days after surgery
and, at this time, increasing dyspnea, pulmonary rales and
hepatomegaly ( 5 cm) were noted. The clinical course was
FIGURE2. Case 1. Angiocardiogram after injection into main
pulmonary artery. Pulmonary venous phase illustrates drainage of right pulmonary veins (RPV) and of most left pulmonary veins ( LPV ) into coronary sinus ( CS ) . Left upper
pulmonary vein drains separately into persistent left vertical
vein ( a r m ) and subsequently into innominate vein ( Inn V).
CHEST, VOL. 64, NO. 3, SEPTEMBER, 1973
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TETRALOGY OF FALLOT
duced by the increase in pulmonary blood flow after
the surgical systemic-pulmonary anastomosis. In this
case, the association of tetralogy and of the anomalous pulmonary veins was known preoperatively,
and a systemic-pulmonary anastomosis was considered a first step in palliation. The autopsy findings
revealed an atrial septal defect only 3 rnm in
diameter. Although the infant responded to medical
management and CPAP in the postoperative period,
atrial balloon septostomy or surgical excision of the
atrial septum would have provided better communication between the two atria and could have reduced the pulmonary venous pressure.
Recent reports7s8indicate the feasibility of corrective operation for most types of total anomalous
pulmonary venous drainage as well as for tetralogy
of Fallot even in neonates. The successful application of profound hypothermia and limited cardie
pulmonary bypass to multiple and complex intracardiac anomalies in neonatese suggests that initial corrective surgery can be considered for the
rare association of anomalies that occurred in our
two infants. However, if a shunt operation is chosen,
as in Case 1, and pulmonary congestion and edema
FIGURE
3. Case 1. Posterior view. Note markedly enlarged coronary sinus (CS ) with right ( RPV) and left ( L W ) pulmonary vein connection. RA=right atrium; LAA=left atrial appendage; LV=left ventricle; RV=right ventricle.
that of increasing cyanosis and progressive pulmonary congestion, with death on the 24th postoperative day.
Autopsy revealed a classic tetralogy of Fallot with pulmonary atresia and total anomalous pulmonary venous drainage
to the portal vein. The lungs were congested, atelectatic, and
hemorrhagic. The surgical aortopulmonary anastomosis measured 1.5m m in diameter.
DISCUSSION
The symptomatology in isolated tetralogy of Fallot is related to decreased pulmonary blood flow and
reduced systemic arterial saturation. Heart failure in
classic tetralogy is very rare.l In contrast, infants
with isolated total anomalous pulmonary venous
drainage frequently present with congestive heart
failure and develop pulmonary edema if the pulmonary venous return is obstructed. When tetralogy
and total anomalous pulmonary venous drainage occur simultaneously, as in our two cases, the presenting manifestations appear to be those of tetralogy.
Patient 1, who had anomalous pulmonary venous
return to the coronary sinus and to the left superior
vena cava, required continuous positive airway pressure for five days after palliative shunt surgery. This
reduction in pulmonary compliance was interpreted
as the result of pulmonary venous congestion in-
FIGURE4. Case 1. Right ventricle (RV). Note classic tetralogy morphology with anterior deviation of crista supraventricularis ( CS ), stenotic pulmonary valve ( PVa), and large
ventricular septal defect (VSD); TV=tricuspid valve; PA=
pulmonary artery.
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MUSTER, PAUL, NlKAlDOH
are encountered postoperatively, prompt recatheterization is in order to assess the size of the surgical
shunt a n d the degree of pulmonary venous hypertension. Atrial septostomy b y balloon catheter or
appropriate surgical measures m a y be indicated in
the early postoperative period.
I n Case 2 t h e subdiaphragmatic pulmonary
venous return was not recognized during life. T h e
preoperative clinical picture was tetralogy with
pulmonary atresia, a n d there was no pulmonary
venous congestion evident clinically o r in the chest
roentgenograms, presumably d u e to a low pulmonary arterial a n d venous blood flow. After the Potts
anastomosis, the pulmonary circulation increased
and, because of the obstructed pulmonary venous
drainage into the portal system, the patient developed progressive pulmonary edema resulting in
death.
In an infant with tetralogy of Fallot a n d subdiaphragmatic o r other obstructed anomalous pulmonary venous drainage, lasting improvement cannot
be expected from a systemic-pulmonary anastomosis
alone. A palliative shunt procedure must b e combined with simultaneous anastomosis of the pulmonary veins to the left atrium, o r corrective surgery
Whales, the largest mammals in the world, have challenged man's skill and ingenuity, and aroused his cupidity, since earliest times. They were originally sought
primarily for their oil, which was used for heat and light,
as an essential ingredient in paints, and in soap. During
World War I there was an enormous demand for whale
oil as a source of glycerol used in the manufacture of
explosives. Some measure of the food requirement of
these creatures is provided by their rate of growth. The
blue whale grows a t a rate of 200 pounds a day. The
whale is a mammal and like all other mammals it nurses
its young. The newborn whale is about twenty-five feet
long and weighs about two tons. It suckles for about
seven months during which time it doubles its length
be directed towards both malformations.
1 Keith JD, Rowe RD, Vlad P: Heart Disease in Infancy and
Childhood. New York, Macmillan Co, 1967
2 Gasul BM, Arcilla RA, Lev M: Heart Disease in Children.
Philadelphia, JB Lippincott Co, 1966
3 Miller RA, Lev M, Paul MH: Congenital absence of the
pulmonary valve: the clinical syndrome of tetralogy of
Fallot with pulmonary regurgitation. Circulation, 26:266,
1962
4 Blalock A: Surgical procedures employed and anatomical
variations encountered in the treatment of congenital pulmonic stenosis. Surg Gynecol Obstet 87:385, 1948
5 Kjellberg SR, Mannheimer E, Rudhe V, et al: Diagnosis of
Congenital Heart Disease. Chicago, Yearbook Medical
Publishers, 1959, p 249
6 Rao BNS, Anderson RC, Edwards JE: Anatomic variations
in the tetralogy of Fallot. Am Heart J 81:361-371, 1971
7 Gersony WM, Bowman FO Jr, Steeg CN, et al: Management of total anomalous pulmonary venous drainage in
early infancy. Circulation 43: ( Suppl 1 ) 19-24, 1971
8 Barratt-Boyes BG, Simpson M, Neutze J: Intracardiac
surgery in neonates and infants using deep hypothermia
with surface cooling and limited cardiopulmonary bypass.
Circulation 43: ( Suppl 1 ) 25-30, 1971
9 Barratt-Boves BG. Nicholls TT. Brandt PWT. et al: Aortic
arch interruption associated with patent ductus arteriosus,
ventricular septa1 defect, and total anomalous pulmonary
venous connection. J Thorac Cardiovasc Surg 63:367, 1972
and may add more than twenty tons to its weight. The
nursing mother produces over a ton of milk per daymilk that may contain ten times more butter fat than
cow's milk. The young whale is adapted to swallow and
breathe simultaneously. Basically this is the same structural refinement that permits horses to drink without
drowning-a separation between respiratory and digestive portions of the pharynx that converts this common
chamber into two functional parts.
Lane, C E, in Idyll, C P: Exploring
the Ocean World-A History of
Oceanography, New York, T Y Crowell,
1969
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