Br J: first published as 10.1136/hrt.32.1.61 on 1 January 1970. Downloaded from British HeartJournal, I970, 32, X6i. Balloon in complete transposition of great arteries in infancy

A. W. Venables From Royal Children's Hospital, Melbourne, Australia

The results of 26 completed balloon atrial septostomies in complete arterial transposition in infancy are described, and complications discussed. Of 7 deaths following this procedure, 3 were clearly or probably related to it or to its failure to produce an adequate septal defect, while 4 were unrelated. Atrial perforations occurred on 4 occasions. Necropsy information regarding the defects produced by the procedure is given. Anatomical features of the fossa ovalis appear to determine the size of the defect created. The effect of the procedure is illustrated by photographs of representative necropsy specimens. Apparently adequate initial defects do not guarantee satisfactory long-term palliation, and 4 of iI infants followed for more than 6 months after effective initial palliation have required surgical procedures to provide more adequate atrial mixing of . Despite this, the procedure appears to offer considerable advantage over initial surgical procedures to create atrial defects.

The value of palliative procedures in com- Subjects and methods plete arterial transposition is indisputable. From to mid-May I23 cases of trans- ig60 i969, http://heart.bmj.com/ Most commonly, atrial septal defects arz cre- position of the great arteries in infancy were diag- ated in order to increase effective pulmonary nosed in the Cardiac Unit of the Royal Children's flow. Since I966 the balloon tech- Hospital, Melbourne. These cases include 53 nique of atrial septostomy introduced by infants previously discussed (Venables, I966). Rashkind (Rashkind and Miller, I966) has Palliative procedures to create atrial septal defects come widely into use, displacing the surgical were performed in 88 infants, with 59 survivors to create who obtained effective immediate palliation. De- procedures previously employed fects were made surgically in 62, while balloon atrial septal defects (Plauth et al., I968; Ven- septostomy was completed in 26 infants. Balloon ables, I966). Though there is no doubt of the septostomy has been performed since late I966 on October 1, 2021 by guest. Protected copyright. value of this procedure in obtaining palliation in preference to initial surgical procedures, pro- in many cases (Watson and Rashkind, I967), vided balloon were available. there are few detailed reports of series indicat- was performed after the ing limitations of technique and follow-up diagnosis of complete transposition of the great progress, and permitting asse3sment of its arteries had been made by selective angiocardio- true role in treatment of complete transposi- graphy from right and left or left tion in infancy (Rashkind and Miller, I968; ventricle. Pressure and oxygen saturation mea- surements were not always possible when the Venables, I968). procedures were performed as emergency mea- This paper presents information regarding sures. Information about the response of these 26 infants in whom balloon atrial septostomy parameters to septostomy is therefore not available was completed as initial palliative treatment in all cases. Initially U.S. Catheter Corporation for complete transposition. The results are catheters were used, but from early in I968 the related to previous and current surgical ex- Edwards Company balloon catheter was em- perience in the same Unit (Venables, I966), ployed routinely (Venables, I968). and problems are discussed. Information is Age at the time of balloon septostomy ranged given about the necropsy findings in those from 6 hours to 7 weeks. Weight ranged from 2-I6 kg. to 4-8o kg., with a mean weight of 3-38 kg. infants who died early, either as a result of Eight infants weighed less than 3 kg. The degree technical failure or unrelated problems. of and of consequent metabolic acidosis varied considerably. Six infants presented with Received 13 June I969. pH values in capillary (heel-prick) blood of 7TI5 Br Heart J: first published as 10.1136/hrt.32.1.61 on 1 January 1970. Downloaded from 62 A. W. Venables

or less, the lowest pH recorded being 6-95. Acid- TABLE I Initial palliative procedures osis was treated with intravenous sodium bicar- bonate in while appropriate dosage preparations No. Unrelated were made for . of Early being Fur- cases deaths* late deaths ther bicarbonate was not ordinarily necessary after septostomy, the pH and base excess return- Surgical atrial septal defects, I960-68 62 22* I0 ing to normal levels and stabilizing there (Fig. i). Surgical defects, I967-68 I3t 2 I Ventricular septal defects of varying size were Balloon atrial septostomy (completed), 26 7 2 detected in I0 of the infants in whom balloon I966-69 septostomy was completed. Persistence ofthe duc- tus arteriosus was shown angiocardiographically * Includes some early failures of surgical procedures in I6 instances. One infant with ventricular septal to create adequate defects. defect required banding be- t Includes 2 after abandonment of balloon cause of the development of cardiac failure after procedure because of atrial perforation. septostomy, and one infant died of heart failure due to persistence of a large ductus. In one in- fant included in the series, the attempt at septos- TABLE 2 Balloon septostomy tomy showed the presence of an contemplated through which a completely filled balloon passed but abandoned readily without evidence of preliminary splitting of the fossa ovalis. No. of The outcome of the procedures is shown in cases Table i. Balloon septostomy was contemplated but abandoned in 5 of the infants operated on in Failure to introduce balloon into 2 i967-I968. The reasons for this are shown in Atrial perforation recognized, procedure 2 Table 2. On two occasions accidental perforation abandoned of the atrial wall occurred before septostomy was Manipulative difficulty I attempted. The procedure was abandoned and the baby treated by immediate thoracotomy, repair of the perforation, and a Blalock-Hanlon procedure. These infants have been transferred to the surgical TABLE 3 Balloon atrial septostomy group (Table I). One survived, but the other died of complications of the surgical procedure. No. of The factors responsible for early death in the cases

first few days after completed balloon septostomy http://heart.bmj.com/ are set out in Table 3. Two deaths clearly repre- Procedures completed 26 sent technical failures. They were due to tricuspid Early deaths 7 Tricuspid valve laceration I Failure to split fossa ovalis I Circulatory failure I FIG. I pH and base excess levels before and Diffuse pulmonary haemorrhage, atrial i after balloon septostomy in an infant with perforation complete arterial transposition. Neonatal septicaemia Cardiac failure from persistent ductus I Anoxic organ damage I on October 1, 2021 by guest. Protected copyright. 7.5. A.M. W + 3-4 kg. 74. 73.3 TABLE 4 Initial balloon atrial septostomy (completed) 7.1 Survivor follow-up

70t No. of cases

0 Procedure performed before November 1968

-.._ Total survivors II -5. u Further procedure necessary 4 E Late deaths -10 Infection x.4 Sagittal sinus thrombosis 2 -15. 2OmEq sod-bicarb. iv. Current survivors 9 -20' Balloon septostomy Procedure performed since November 1963 I~~~~~~~~~~~~~~~~~~~~~~~~ Total survivors 8 2 3 4 5 Late deaths 0 Days of life Br Heart J: first published as 10.1136/hrt.32.1.61 on 1 January 1970. Downloaded from Balloon septostomy in complete transposition 63 valve laceration and to an inadequate defect. The of the detection of inadequate atrial defects. death from circulatory failure appeared to be re- In this series, 4 of II infants observed for lated at least partly to the presence of a greater more than 6 months since septostomy have degree of acidosis than appreciated. The remain- required further procedures to provide a ing 4 deaths were due to unrelated problems, though the baby who was moribund from diffuse more adequate atrial septal defect. The others, pulmonary haemorrhage when catheterization was including 2 infants observed for 32 and 2I begun was found at necropsy to have previously months, respectively, remain satisfactory. unrecognized atrial perforation and haemoperi- Rashkind (Rashkind and Miller, I968) re- cardium. Necropsies were performed after 6 of ported 3 repeat septostomies between i4 and the 7 early deaths. 5 months after the initial procedure, further Follow-up data are given in Table 4. Of II study being prompted by 'the development survivors with satisfactory initial palliation fol- of cardiomegaly plus some increase in cyano- lowed for more than 6 months, 4 have required sis'. further surgical palliation at ages of from 5 to 7 months. All had Blalock-Hanlon procedures, The degree of cyanosis after successful though in one a further balloon procedure was palliation has varied in the present series as first performed without satisfactory response. after successful Blalock-Hanlon procedures The 2 late deaths occurred after secondary sur- (Venables, I966). The patients who were con- gical procedures, but were unrelated to . sidered to require enlargement of their atrial These deaths were due to presumed sagittal sinus defects were considerably cyanosed. Their thrombosis and to severe infection. Necropsy was condition had become generally unsatisfactory not performed in either case. The infants who with irritability, poor feeding, and acidosis. have survived balloon septostomy since November I968 are currently all in satisfactory condition. No In one case inadequate atrial shunts and a information is available about the ultimate size of high left atrial pressure were shown at a fur- the defects in these infants with transposition. ther catheterization procedure. This study However, 2 infants from a small group with total was aimed mainly at measuring pulmonary anomalous pulmonary venous drainage also sub- artery pressure and flow in reference to pos- jected to balloon septostomy in the same unit have sible pulmonary artery banding because of an now had operation at approximately IS months of associated large ventricular septal defect. Un- age. The defects produced by previous septostomy fortunately, no attempt was made to assess the were then both approximately I 5 cm. in diameter. actual defect size by use of a balloon catheter. When, however, another child was recatheter- Discussion ized recently at 4 months of age because of http://heart.bmj.com/ The material presented confirms that balloon some increase in cyanosis associated with con- atrial septostomy provides an acceptable tech- sistent mild acidosis, with pH readings of nique for immediate palliation of infants with about 7 3o, a balloon catheter passed freely complete arterial transposition needing im- through the atrial defect when filled with 3-o provement in effective pulmonary flow. There ml. of dye mixture. This defect is regarded as were 3 deaths after septostomy to which the adequate. More satisfactory criteria are neces- procedure appeared definitely or probably sary, therefore, to determine when to perform contributory. An additional death followed a second procedure. These will be formulated on October 1, 2021 by guest. Protected copyright. atrial perforation complicating introduction only on a basis of careful study of haemo- of a balloon catheter into the heart with- dynamics and assessment of defect size in out actually performing septostomy. This was patients who appear to be unsatisfactory. of purely surgical nature but, even when this Necropsy studies of 6 of 7 infants dying is taken into consideration, the mortality re- early after balloon septostomy revealed ade- lated to the procedure compares favourably quate defects in all but one. Fig. 2a, b, and c not only with over-all but with recent surgical show typical examples of these defects, in experience in the same unit. Further, pallia- which the thin floor of the fossa ovalis has tion has quite evidently been available to small been torn across transversely from its free infants and to very acidotic infants in whom edge at the , creating defects an operation would have been unlikely to that correspond essentially to the fossa ovalis. succeed (Rashkind and Miller, I968; Ven- Fig. 2d shows the inadequate defect, with no ables, I968). splitting of the fossa ovalis, and associated Early deaths from unrelated causes, and with a much more ovoid, tough-edged fossa. late deaths from such causes as infection and In this patient the maximum balloon volume cerebrovascular accidents, occurred as in that could be pulled through the septum was other studies of infants with complete trans- i -8 ml., and it was doubtful at the time if position. adequate splitting had been achieved. The It is important to consider the long-range pre-existing acidosis was controlled, but the effectiveness of palliation and the problem baby developed heart failure and died several Br Heart J: first published as 10.1136/hrt.32.1.61 on 1 January 1970. Downloaded from 64 A. W. Venables

f 0 cm. 2 a http://heart.bmj.com/

FIG. 2 (a, b, and c) Atrial septal defects produced by balloon septostomy in infants with on October 1, 2021 by guest. Protected copyright. complete arterial transposition, showing tearing offloor offossa ovalis. (d) An inadequate defect with no tearing of a much tougher fossa. Specimens viewed from right atrial aspect. days later. In the 25 other completed proce- comparable to those in Fig. 2a-c is shown by dures, the balloon volume ranged from 2-0 to Fig. 3, which shows the defect produced in 3-5 ml., with a mean volume of 3-0 ml. In I7 an infant with total anomalous pulmonary infants a balloon containing 3-0 ml. or more venous drainage by passage of a single lumen was withdrawn after the fossa ovalis had been U.S. Catheter Corporation catheter contain- split. The balloon volume was limited to 2-0 ing only 2-0 ml. It should be noted that filling ml. in 2 cases. In one, the limiting factor in of the balloon of the Edwards double lumen passage of a larger volume was impaction of catheter used in most of the present series the balloon in the inferior vena cava before it beyond 2-5 ml. causes elongation of the bal- had passed wholly through the atrial septum. loon along the catheter rather than increase in At a later catheterization at 4 months a 3-o ml. its diameter. Balloon volume is a useful and balloon passed through this defect easily. In reliable reference point. The Edwards double the other infant the septum was felt to tear, lumen catheter with a volume of 3 o ml. of but only 2-0 ml. could be withdrawn. That dye mixture produces a balloon diameter of such a volume can in fact produce a defect approximately I5 mm. Balloon septostomy in complete transposition 65 Br Heart J: first published as 10.1136/hrt.32.1.61 on 1 January 1970. Downloaded from

tages of avoiding thoracotomy and a surgical procedure to create an atrial septal defect. It is evident that great care is required to avoid atrial perforation. In addition to the three atrial perforations described above, a further perforation occurred during a pro- cedure when defect size was being tested after In this infant the .;; performance of septostomy...... ° catheter tip failed consistently to pass into a >,|- left upper lobe pulmonary vein as usual, and .,s.,.-e2:sg.,f6. ! - .... ; ...... j? - ,_E - finally slipped through the end of the left * ... ;.;.&lSis.: zX= atrial appendage during manipulation there. wBf§ s There was no evidence of haemopericardium, but cardiac arrest occurred and external car- diac massage was necessary. In the experience e. 88 @! s si of the unit cardiac perforation has been con- \.R.'Bs.... : ¢ fined to balloon septostomy. Perforations were not noted by Rashkind (Rashkind and Miller, I968), but one instance was recorded by Cartmill, Celermajer, and Bowdler (I967).

The author is grateful to those who have referred patients. Palliative surgical procedures were per- formed by Mr. G. W. Westlake and Mr. P. G. Jones. Observations on the defects seen at the time of reparative surgery in infants with total anomalous pulmonary venous drainage were made FIG. 3 Atrial septal defect produced by by Mr. H. D'Arcy Sutherland at the Royal balloon septostomy in an infant with total Adelaide Hospital. anomalous pulmonary venous drainage, viewed from inferior vena cava. References http://heart.bmj.com/ Cartmill, T. B., Celermajer, J. M., and Bowdler, J. D. (I967). Cyanotic congenital heart disease: new Inspection of Fig. 2a-c suggests that the trends in management of transposition of the great size of defect made by balloon septostomy is arteries. MedicalJournal of Australia, 2, 1019. limited by the size of the fossa ovalis and its Plauth, W. H., Jr., Nadas, A. S., Bernhard, W. F., and Gross, R. E. (I968). Transposition of the great thin floor. These defects are smaller than the arteries; clinical and physiological observations on defects usually seen after the Blalock-Hanlon 74 patients treated by palliative surgery. Circula- If there is no real sensation of tion, 37, 316.

procedure. on October 1, 2021 by guest. Protected copyright. splitting and the volume that can be with- Rashkind, W. J., and Miller, W. W. (I966). Creation of an atrial septal defect without thoracotomy; a drawn is limited, by resistance at the septum palliative approach to complete transposition of the rather than other factors such as impaction great arteries. Journal of the American Medical in the inferior vena cava, one must expect the Association, I96, 99I. defect to prove inadequate. Balloon filling be- -, and - (I968). Transposition of the great arteries; results of palliation by balloon atrio- yond about 2z5 ml. probably merely confirms septostomy in thirty-one infants. Circulation, 38, maximum splitting rather than contributes 453. significantly to the size of the defect. Venables, A. W. (I966). Complete transposition of the Apparent maximum splitting does not great vessels in infancy with reference to palliative surgery. British HeartJournal, 28, 335. guarantee long-term adequacy of the defect. - (I968). Creation of atrial septal defects by the Balloon filling ranged between 2-5 and 3.5 ml. balloon catheter technique in infants with complete in those infants who have required later secon- transposition of the great vessels. Australian Paedi- dary procedures to produce a larger defect. atric Journal, 4, 236. of cases with early ade- Watson, H., and Rashkind, W. J. (1967). Creation of Though a proportion atrial septal defects by balloon catheter in babies quate palliation will require secondary pro- with transposition of the great arteries. Lancet, I, cedures, many will enjoy the obvious advan- 403.

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