348 Discussion
The incidence of pheochromocytoma is 0.001% in the general population, and 0.5% in patients with hypertension [1,2]. Malignant change occurs in 3 to 14%, and it represents a particular diagnostic problem because of the potential for a multicentric origin and the absence of specific histologic criteria for malignant change [3]. The diagnosis is dependent on distant metastasis which occurs to lymph nodes, liver, bones, muscle, or lungs. The clinical presentation of pheochromocytoma is variable [2]. To the best of our knowledge a presentation with sudden onset paraplegia caused by collapse of the first lumbar vertebra due to osseous metastasis from the pheochromocytoma has not been reported previously. The treatment of the malignant form of the tumor comprises controlling excess circulating catecholamines with adrenergic blocking agents alone or in combination with labetolol and/or metyrosine. Therapeutic embolization to reduce tumor load and catecholamine
production can be attempted [2]. Chemotherapy with cyclophosphamide, vincristine and dacarbazine have not shown persistent benefits [4]. Malignant change in pheochromocytoma heralds poor prognosis with a 5 year survival of 44% as against 96% for benign tumors PI. References Beard CM, Sheps SG. Kurland LT, Carney JA, Lie JT. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc 1983;58:802-804. Sheps SG, Jiang NS. Klee GG, Van Heerdan JAV. Recent developments in the diagnosis and treatment of pheochromocytoma. Mayo Clin Proc 1990;65:88-95. Poutasse EF, Gifford GH. Pheochromocytoma: diagnosis and treatment. Prog Cardiovasc Dis 1965;8:235-252. Auerbuch SD, Steakly CS, Young RC, Gelman EP, Goldstein DS, Stull R, Keisser HR. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine and dacarbazine. Ann Intern Med 1980:109:267-273. Van Heerdan JAV, Sheps SG, Hamberger 8, Sheedy PF II. Poston JG, ReMine WH. Pheochromocytoma: current status and changing trends. Surgery 1982:91:367-373.
International Journal of Cardiology, 34 (1992) 348-350 0 1992 Elsevier Science Publishers B.V. All rights reserved 0167.5273/92/$05.00
CARD10 14104
Successful treatment of a nondeflatable septostomy catheter
balloon atria1
Siiheyla 6zkutlu and Nazan 6zbarlas Hacettepe UniLlersityChild Health Institute, Department of Pediatric Cardiology, Ankara, Turkey
(Received 29 July 1991; revision accepted 27 September 1991)
We present a rare complication of balloon atrial septostomy in arteries and review the management of this problem. The balloon the atria1 septum. We punctured the balloon with a stylet which double-lumen septostomy catheter. We suggest that this method employed.
Key words: Balloon atria1 septostomy; Transposition
Correspondence
to: Dr. Siiheyla Gzkutlu, Department
an infant with complete transposition of the great could not be deflated after the eighth pass across was passed through the circulation lumen of the should once be tried before other methods are
of the great arteries
of Pediatric Cardiology, Hacettepe University, Ankara, Turkey.
339
Introduction The technique of creating an atria1 septal defect to improve interatrial mixing, using a balloon-tipped catheter in infants with transposition of the great arteries was introduced in 1966 by Rashkind and Miller [l]. This has become accepted as the palliative procedure and is performed by fluoroscopy or echocardiography [1.2]. However, following the widespread use of this procedure, complications have been reported. One of these complications is the inability to deflate the inflated balloon in the right atrium. There are some methods described in the literature to achieve this [3-51. In this study, we propose a simple method, based on our experience on one patient. Case Report A 14-day-old newborn (weighing 2800 g) with probable transposition of the great arteries was admitted with heart failure and cyanosis. The diagnosis was confirmed by echocardiography and angiography, and balloon septostomy was performed. A No. 5.5 double lumen open-end balloon catheter (USC1 Rashkind DL) was inserted through the femoral vein into the left atrium and inflated with 1 ml of a contrast media mixture. The balloon was withdrawn sharply into the right atrium with considerable effort. Eight subsequent passes were made with gradually enlarging balloon size. After the last pull-through with 4 ml of the fluid, it was observed that the balloon could not be deflated. No blood or fluid could be aspirated or injected into either of the two lumens. Fluoroscopy revealed that the balloon was still inflated and changed in shape asymmetrically. In an attempt to deflate the balloon, a 0.012 inch blue pacemaker electrode stylet was passed through the pressure lumen of the septostomy catheter and advanced until the point protruded beyond the end hole and the balloon was successfully punctured. Subsequently another septostomy catheter was utilized to enlarge the atrial septostomy successfully. The result of the septostomy was satisfactory and the baby has made considerable progress. Discussion Balloon atria1 septostomy, from its inception, produced a marked improvement in the survival of infants with complete transposition. There are few reports of complications of this procedure. The most common complication is the inability to deflate the catheter balloon. A nondeflatable balloon can be due to two reasons. The first is a blocked catheter lumen causing failure of deflation of the balloon [5]. The second,
which is more widely described, is slipping of the proximal tie of the balloon distally so that the hole in the catheter is then not in communication with the balloon [3.4]. Various methods have been described for the management of the nondeflatable balloon. Scott reported two cases of nondeflatable balloon, during septostomy [3]. The problem was that the dye was trapped in the balloon due to movement of the proximal tethering. Deflation of the balloon was achieved in each case by puncturing it with a needle introduced into the right atrium through the chest wall. Wilson et al. suggest that a transhcpatic approach to deflate the balloon can be effective, quick and safe. especially when the patient’s condition requires urgent action [5]. However, deflation of the balloon with an external needle applied through the chest wall or transhepatic approach may be life threatening. Ellison ct al. pulled the catheter sharply against the right atrium-inferior caval vein junction and this caused the proximal attachment of the balloon to break, deflate and to evert [4]. However. this technique has potential dangers such as tcaring of the right atrium-inferior caval vein junction or wedging of the balloon in the inferior caval vein with obstruction of venous return. Ellison’s second solution was to puncture the balloon with a metal guide passed through a Lehman catheter inserted via the other femoral vein [4]. The USC1 Company recommends an identical procedure. However. although this procedure seems reasonable, there may be additional technical problems and it may increase the duration of the procedure. In our case, we used the circulation lumen of the septostomy catheter for deflation of the balloon. Nondeflation was caused by a blocked catheter lumen. The balloon slipped and became asymmetric in shape, so the end hole of the pressure lumen and a part of the balloon became closer to each other while the balloon was passing through the interatrial septum against rcsistance. The stylet which protruded beyond the end hole punctured the balloon. The success of this method depends on using a double lumen catheter and the altered shape of the balloon so that the end hole and the balloon are in close proximity. This particular configuration only occurs by chance, but we suggest that this method should be tried before a second venous line is instituted and more aggressive methods are employed. It may work and is safer. References
1 Rashkind
WJ. Miller WW. Creation of an atria1 septat defect without thoracotomy: a palliative approach to complete transposition of the great arteries. JAMA 1966:196: 991-992.
3.50 2 Allan
LD, Leanage R, Wainwright R, Joseph MC, Tynan Balloon atria1 septostomy under two-dimensional echocardiographic control. Br Heart J 1982;47:41-43. 3 Scott 0. A new complication of Rashkind balloon septostomy. Arch Dis Child 1970;45:716-717. 4 Ellison RC, Plauth WH, Gazzaniga AB. Fyler DC. Inability
M.
International Journal of Cardiology, 34 (1992) 350-352 0 1992 Elsevier Science Publishers B.V. All rights reserved
to deflate catheter balloon: a complication of balloon atrial septostomy. J Pediatr 1970:76:604-606. 5 Wilson NJ, Culham JAG, Sandor GGS. Successful treatment of a non-deflatable balloon atrial septostomy catheter. Pediatr Cardiol 1990:11:150-152.
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CARD10 14105
Membranous subvalvar pulmonary stenosis in complete transposition; advantage of biplane transoesophageal echocardiography for the diagnosis in a young adult Pierre Decoodt
‘, Henri Verhaaren
3 and Frank Deuvaert
’
’ Departments of Cardiology and ’ Cardiac Surgev, Unir~ersityHospital Brugmann. Brussels, Belgium, 3 Division of Paediatric Cardiology, Unirersi& Hospital Ghent, Ghent, Belgiam
A young adult who had previously undergone a Mustard repair for complete transposition developed symptomatic left ventricular outflow tract obstruction. Biplane transoesophageal echocardiography allowed an optimal preoperative assessment. In the longitudinal plane, a discrete calcified membrane was imaged. Consequently, a pulmonary arteriotomy was chosen for the relief of the malformation. Key words: Pulmonary
stenosis;
Complete
transposition;
Introduction Obstruction of the left ventricular outflow tract is a well known associated lesion in complete transposition of the great arteries with intact interventricular septum. For a good surgical approach, differentiation should be made between valvar and subvalvar obstruction. In the latter, distinction has to be made between a discrete, a tubular or a hypertrophic (dynamic) stenosis [1,2]. Although transthoracic echocardiography allows the correct diagnosis in infants and children [3], Correspondence fo: P. Decoodt, M.D., Dept. of Cardiology, University Hospital Brugmann, Place Van Gehuchten 4. 1020 Brussels, Belgium.
Transesophageal
echocardiography
this method presents limitations in adults, due to poorer ultrasound penetration and window problems. Transoesophageal echocardiography, especially using the longitudinal plane, offers a better view of the outflow tract [4,5]. In our patient, it allowed a correct preoperative characterization of the morphology of the stenoSk.
Case Report Our patient underwent a Mustard repair for transposition of the great arteries at the age of four. After surgery a murmur of pulmonary stenosis was heard. During follow-up, a steady infundibular gradient of 50 mmHg was found by Doppler and repeated catheteri-