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Fig. 2. Surgical s p e c i m e n of e m b o l u s s h o w n in Fig. 1.
Table
I. Cases of i m p e n d i n g p a r a d o x i c e m b o l i s m Age Sex
Nellessen et al. 9 (1985) Loscalzo4 (1986) Speechly-Dick et al. s (1991) Nelson et al. 7 (1991) Barnard et al. 3 (1991) Nagelhout et al. 2 (1991) Black et al. 6 (1993)
56 68 50 20 69 56 51
M F M M F M F
Underlying disease
Intracardiac Imaging defect modality
Phlebothrombosis Adenocarcinoma Cerebrovascular accident Cavernous hemangioma Phlebothrombosis Coronary artery disease Meningioma
ASD PFO PFO PFO PFO PFO PFO
TEE TTE TTE TTE TEE TEE TEE
Arterial embolism Treatment No Yes Yes Yes Yes Yes No
Surgery Medical Surgery Surgery Surgery Surgery Surgery
Outcome Alive Alive Alive Alive Alive Alive Alive
ASD, Atrial septal defect.
o g r a p h y as a reliable a n d r e a d i l y available n o n i n v a s i v e b e d s i d e d i a g n o s t i c tool in clinically s u s p e c t e d p a r a d o x i c embolism.
Interventional cardiac catheterization under transesophageal echocardiographic guidance
REFERENCES
1. Johnson BJ. Paradoxical embolism. J Clin Pathol 1951;4:316-32. 2. Nagelhout DA, Pearson AC, Labovitz AJ. Diagnosis of paradoxic embolism by transesophageal echocardiography. AM HEART J 1991;121: 1552-4. 3. Barnard SP, Kulatilake ENP, Azzu AA, Ikram S. Straddle embolus-imminent paradoxical embolus diagnosed by echocardiography and treated surgically. Eur J Cardiothorac Surg 1991;5:105-7. 4. Loscalzo J. Paradoxical embolism: clinical presentation, diagnostic strategies and therapeutic options. AM HEARTJ 1986;112:141-5. 5. Hagen PT, Scholz DG, Edwards WD. Incidence and size of patent foramen ovale during the first 10 decades of life: an autopsy study of 965 normal hearts. Mayo Clin Proc 1984;59:17-20. 6. Black MD, Masters RG, Sochowski RA, Higginson LAJ, Keon WJ. Paradoxical embolism-in-transit: diagnosis and surgical treatment. Can J Cardiol 1993;9:437-40. 7. Nelson CW, Snow FR, Barnett M, McRoy L, Wechsler, Nixon JV. Impending paradoxical embolism: echocardiographic diagnosis of an intracardiac thrombus crossing a patent foramen ovale. AM HEART J 1991;122:859-62. 8. Speechly-Dick ME, Middleton SJ, Foale RA. Impending paradoxical embolism: a rare but important diagnosis. Br Heart J 1991;65:163-5. 9. Nellessen U, Daniel WG, Matheis G, Oelert H, Depping K, Lichtlen PR. Impending paradoxical embolism from atrial thrombus: correct diagnosis by transesophageal echocardiography and prevention by surgery. J Am Coll Cardiol 1985;5:1002-4.
A l a n D. T o n g , M D , a A b r a h a m R o t h m a n , M D , a T a k a h i r o S h i o t a , M D , b M a r y Rice, M D , b D a n i e l G. B l a n c h a r d , M D , a W i l l i a m H e l l e n b r a n d , M D , c a n d D a v i d J. S a h n , M D b San Diego, Calif.,
Portland, Ore., and New Haven, Conn.
T h e r e c e n t p r o l i f e r a t i o n o f t e c h n i q u e s a n d devices for use in i n f a n t s a n d c h i l d r e n d u r i n g i n t e r v e n t i o n a l c a r d i a c c a t h e t e r i z a t i o n h a v e u n d e r l i n e d t h e n e e d for i m p r o v e d diagFrom the aDivisionof Pediatric Cardiology,Department of Pediatrics, University of California, San Diego; the bClinical Care Center for Congenital Heart Disease, Department of Pediatrics, Oregon Health Sciences University; and the CSection of Pediatric Cardiology, Yale University School of Medicine. Reprint requests: Abraham Rothman, MD, Division of Pediatric Cardiology, No. 8445, UCSD Medical Center, 200 West Arbor Dr., San Diego, CA 92103. AM HEARTJ 1995;129:827-31. Copyright © 1995 by Mosby-Year Book, Inc. 0002-8703/95/$3.00 + 0 4/4/61209
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Fig. 1. Before (top) and after dilation (middle and bottom) of pulmonary venous atrial (PVA) obstruction (patient 5, Table II) in transverse (left) and longitudinal (right) plane TEE views. Narrowed area has been increased from 3 to 8 mm in diameter.
nostic and monitoring modalities to aid in the performance and assess the effectiveness of these interventions. Transesophageal echocardiography (TEE) has emerged as an important adjunct to interventional cardiac catheterization in the creation of atrial septal defects (ASD), closure
April 1995 American Heart Journal
of ASD and ventricular septal defects (VSD), and relief of posterior intracardiac obstructions 1, 2; however, TEE's efficacy, limitations, and risks are still being defined. 3 We describe 13 patients, 11 in the pediatric age group, who underwent cardiac catheterization in which TEE was helpful for evaluation of defects or outcome of interventional procedures. Methods. Between July 1990 and March 1993, 13 patients underwent TEE monitoring during interventional cardiac catheterizations. The patients ranged in age from 26 months to 56 years (mean 12.5 + 15.0 years, median 6.8 years) and in weight from 11 to 78 kg (mean 31.7 _+ 21.4 kg, median 20.9 kg). Excluding the adult patients, the mean age was 6.0 __ 4.4 years and the mean weight 23.9 + 21.7 kg. Allpediatric patients underwent cardiac catheterization under general anesthesia with endotracheal intubation and mechanical ventilation. The adult patients received conscious sedation, one with intravenous midazolam, and the other with intravenous ketamine and midazolam. ~11 patients received additional sedation with intravenous fentanyl and midazolam as necessary. Patients underwent TEE using Aloka (Wallington, Conn.), Hewlett-Packard (Andover, Mass.), or Toshiba (Tustin, Calif.) echocardiographic systems interfaced with 5 MHz biplane TEE probes attached to the end of a flexible, steerable endoscope. Topical lidocaine spray was applied to the posterior pharynx and viscous lidocaine jelly to the TEE probe before insertion of the probe into the esophagus, Twodimensional images, pulsed and continuous wave Doppler tracings, and color Doppler flow images were recorded on each patient as appropriate during the catheterization or before and after the intervention if a procedure was performed. TEE was performed on five patients durifig transcatheter closure of secundum ASD by using clamshell closure devices (USCI Angiographic Systems, Tewksbury, Mass.). The mean age of these patients was 3.7 _+ 1.2 years and mean weight 15.0 _+ 2.3 kg. ASDs were evaluated by surface echocardiography before catheterization, then by TEE and angiography. The method of placement of the clamshell closure device was as described previously. 4 TEE was used during transseptal atrial puncture and balloon ' dilation of the atrial septum for palliation of primary pulmonary hypertension in 3 patients: 2 (a 14-year-old girl and a 56-year-old woman) had had symptoms for approximately 2 years and I (a 35-year-old woman) for approximately I year. All three patients had worsening fatigue, respiratory distress, and syncopal episodes and had been placed on a waiting list for lung transplantion. The method of puncture and balloon dilation of the atrial septum in primary pulmonary hypertension was as previously described. 5 TEE was used during catheterization to evaluate three patients with D-transposition of the great arteries after atrial switch operations; 1 had undergone a Mustard procedure at 22 months of age and the other 2 had undergone Senning procedures at 6 and 7 months of age. The first patient underwent catheterization to perform balloon dilation of a superior vena cava to neo-right atrium obstruction. The second patient underwent catheterization
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Fig. 2. Before (upper) and after (lower) balloon dilation and stenting for superior vena caval (SVC) systemic venous atrial (SVA) obstruction after Mustard procedure (patient 6, Table II). Improvement is visible on both angiography (left) and biplane TEE (right).
for evaluation of decreased exercise tolerance and recurrent pneumonia, and two dilations were performed under TEE guidance, 1 in the superior vena cava baffle, and I in the pulmonary venous baffle at two catheterizations performed 1 month apart (Fig. 1). The third patient underwent balloon dilation of an SVC obstruction and stent placement under TEE guidance (Fig. 2). Multilevel left ventricular outflow tract (LVOT) obstructions were evaluated by TEE in the last 2 patients. The first was a 13-yearold, 78 kg girl with an estimated pressure gradient of 64 mm Hg by transthoracic Doppler interrogation in the LVOT; two-dimensional images, however, were unable to define the level(s) of stenosis because of inadequate windows. The second patient was a 7-year-old girl who had undergone repair of complete atrioventricular canal at 7 months of age and resection of accessory atrioventricular valve tissue that had caused progressive subaortic stenosis at age 5 years. Transthoracic echocardiography revealed an estimated pressure gradient of 50 mm Hg in the LVOT; however, two-dimensional studies did not provide optimal images to
determine the nature and site(s) of the obstruction. In both patients, direct pullback pressure measurements and angiography at cardiac catheterization confirmed the presence of moderate LVOT obstruction but could not precisely identify the levels of the stenoses. Results and comments. In our study group, TEE was performed without complication. Ten of 11 patients in whom transcatheter interventions were performed had a successful procedure; in another 2 patients, the information obtained with TEE changed the decision to perform an intervention. In the 5 patients with ASD, 4 had isolated secundum defects and 1 had a secundum defect associated with pulmonary atresia and an intact ventricular septum; all 5 devices were successfully deployed (Table I). TEE helped determine defect size, aided in guiding the distal half of the clamshell device on the atrial septum, confirmed device position after delivery of its proximal half, and detected trivial shunting in all 5 patients immediately after device placement. At follow-up (mean 14.0 + 4.5 months) only 2 patients showed residual ASD flow by transthoracic
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Table ]. Transcatheter ASD closures ASD size (ram) Patient
Diagnosis
Age (yr)
Weight (kg)
Surface echocardiogram
Angiogram
Stretched
Qp:Qs
Device size (ram)
Result
1 2 3 4 5
ASD PA/IVS, ASD ASD ASD ASD
5.1 2.8 2.2 3.8 4.5
16.8 15.0 11.0 16.1 16.0
11 12 12 12 12
14 12 11 10 10
19 18 12 12 13
2.2 0.8 1.5 1.5 2.4
33 33 23 23 28
Closed Trivial leak Closed Closed Trivial leak
PA/IVS, Pulmonary atresia with intact ventricular septum; Qp:Qs, pulmonary-to-systemicflowratio.
Table II. Other Interventional Catheterizations Patient
Diagnosis
Age (yr)
Weight (kg)
1
PPH
14
59
2
PPH
35
56
3
PPH
56
53
4
D-TGA, s/p Mustard D-TGA, s/p Senning
5
Catheterization findings OS 88% to 81% OS 88% to 82% OS 80% to 7O% MDS 8 mm
TEE findings 7-8 mm ASD flow 5 mm ASD flow
ASD creation
4 mm ASD flow
ASD creation
MDS 8 mm
Balloon dilation Balloon dilation
7.5
25
6.3
16
SVC-neoRA MDS 1.5 mm
6.3
17
PVA MDS 3 mm SVC-neoRA MDS 8 mm
SVC-neoRA MDS 1.5->4.5 mm PVA MDS 3->8 mm SVC-neoRA MDS <8 mm
LVOT gradient 45-50 mm Hg LVOT gradient 40 mm Hg
Muscular sub AS Multilevel sub AS
6
D-TGA, s/p Mustard
11
39
7
AS, sub AS
13.1
78
8
AVC, sub AS
7.3
26
Interventions
ASD creation
Balloon dilation Balloon dilation; 18 mm stent placement --
AS, Aorticstenosis;AV,atrioventricular;A VC, atrioventricularcanal;D- TGA, D-transposition ofgreat arteries;MDS, minimumdiameter of stenosis;neoRA, neo-right atrium; PPH, primary pulmonary hypertension; PVA, pulmonary venousatrium; OS, oxygensaturation; s/p, status post; sub AS, subaortic stenosis; SVC, superior vena cava.
echocardiography. In the 3 patients with primary pulmonary hypertension undergoing ASD creation, T E E was used to guide the transseptal needle onto the atrial septum and estimate the defect size by T E E from color Doppler flow patterns across the atrial septum after balloon dilation. Two patients had defects, one measuring 4 m m and the other measuring and 7 to 8 mm, and had no more episodes of syncope; they underwent successful single lung transplantation 3 and 9 months after catheterization, respectively. T h e third patient, a 56-year-old woman, had a 4 m m ASD created but died of severe symptoms of primary pulmonary hypertension 3 days after an uncomplicated procedure; transthoracic echocardiography performed after catheterization showed persistent right-toleft flow across the ASD. In the 3 patients with D-transposition of the great arteries after atrial switch
procedures, T E E was useful in assessing systemic and pulmonary venous baffle obstructions. A 7-year-old boy with D-transposition of the great arteries after a Mustard procedure was found to have an 8 m m stenosis of the superior vena cava with a gradient of 7 to 8 m m Hg to the n eo - ri ght atrium which was dilated with side-by-side 12 and 15 m m balloons, without improvement. A 6-year-old boy had 2 stenoses after a Senning procedure, 1 in the superior vena cava n eo - r i g h t atrium junction with a gradient of 8 m m Hg and m i n i m u m diameter of 1.5 mm, and 1 in the pulmonary venous atrium with a gradient of 19 m m Hg and m i n i m u m diameter of 3 mm. These were dilated with 12 m m and side-by-side 8 m m balloons, respectively; T E E documented both an increase in diameter and decrease in velocities in both after dilation (Fig. 1, Table II). An l l - y e a r - o l d girl had balloon dilation after Mustard procedure and an 18 m m
Volume 129, Number 4 American Heart Journal
stent placement in an superior vena cava obstruction with a decreased gradient documented by TEE (Fig. 2). In 2 patients with multilevel LVOT obstruction, TEE confirmed transthoracic echocardiographic and angiographic findings but was superior to both in defining the nature of the stenoses. A 13-year-old girl had a gradient of 50 mm Hg in the LVOT at catheterization; TEE alone detected the presence of a muscular tunnel. In a 7-year-old girl with recurrent LVOT obstruction with a gradient of 40 mm Hg at catheterization, TEE demonstrated a fibromuscular ridge, a discrete membrane, and recurrent growth of atrioventricular valve tissue after complete atrioventricular canal repair. On the basis of these findings, no interventions were performed at the time of catheterization in these two patients. Conclusions. In this study, TEE performed in conjunction with cardiac catheterization provided crucial information regarding the atrial septum, postsurgical intraatrial obstructions of systemic and pulmonary venous connections, and the LVOT. This information may not be obtainable by transthoracic echocardiography or angiography. Our findings support the use of TEE to monitor the performance and success of these selected interventional catheterization procedures. REFERENCES
1. Van der Velde ME, Perry SB, Sanders SP. Transesophageal echocardiography with color Doppler during interventional catheterization. Echocardiography 1991;8:721-30. 2. Stumper O, Witsenburg M, Sutherland GR, Cromme-Dijkhuis A, Godman MJ, Hess J. Transesophageal echocardiographic monitoring of interventional cardiac catheterization in children. J Am Coll Cardiol 1991;18:1506-14. 3. Weintraub R, Shiota T, Elkadi T, Golebiovski P, Zhang Z, Rothman A, Ritter S, Sahn DJ. Transesophageal echocardiography in infants and children with congenital heart disease. Circulation 1992;86:711-22. 4. Rome JJ, Keane JF, Perry SB, Spevak PJ, Lock JE. Double-umbrella closure of atrial defects; initial clinical applications. Circulation 1990;82:751-8. 5. Rothman A, Beltran D, Kriett JM, Smith C, Wolf P, Jamieson SW. Graded balloon dilation atrial septostomy as a bridge to lung transplantation in pulmonary hypertension. AM HEART J 1993;125:1763-6.
Chaotic atrial tachycardia in children Mubadda A. Salim, MD, a Christopher L. Case, MD, b and Paul C. Gillette, MD b Charleston, S. C. Chaotic atrial tachycardia (CAT) is characterized by the presence of three or more P-wave morphologic features on
From the Divisions of Pediatric Cardiology, the aUniversity of Tennessee and the 5Medical University of South Carolina. Reprint requests: Christopher L. Case, MD, Division of Pediatric Cardiology, Medical University of South Carolina, 171 Ashley Ave., Charleston, SC 29425-0680. AM HEART J 1995;129:831-3. Copyright ® 1995 by Mosby-Year Book, Inc. 0902-8703/95/$3.00 + 0 4/4/61191
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the surface electrocardiogram, absence of a dominant atrial pacemaker, and variable P-P, R-R, and P-R intervals with an atrial rate of >100 beats/min. 1 CAT is predominantly a disease of adults with respiratory illness. Fewer than 100 cases have been reported in children. 2-6 The correct diagnosis and treatment is important, because this arrhythmia can be difficult to control and can be fatal. We describe our experience with this arrhythmia, concentrating on the clinical presentation, acute and chronic treatment regimens, and outcome. During the period between October 1986 and December 1993, five infants arrived at the Medical University of South Carolina with CAT. Over the same time period, 1500 new onset arrhythmias were diagnosed. This represents an incidence of 0.2 %. We retrospectively reviewed the charts of these patients and evaluated their response to therapy. There were three boys and 2 girls aged 3.0 _+ 3.7 months (Table I). Three of the five patients had symptoms at initial examination. Two patients had respiratory distress after several days of nonspecific symptoms, and one patient had tachycardia in utero along with polyhydramnion. At initial examination 3 infants showed signs of congestive heart failure and 2 had no abnormalities other than rapid irregular heart rate. Cardiomegaly was present on the chest radiographs of 3 patients. Diagnosis of CAT was made on the basis of 12-lead electrocardiogram; according to the diagnostic criteria (Fig. 1), the mean number of different P-wave morphologic features was 4 __ 2 (range 3 to 7). The atrial rate could only be estimated from the surface electrocardiogram. The PR interval ranged between 50 and 180 msec. The mean ventricular rate was 178 + 25 beats/rain. The QRS complex morphologic characteristics were norreal in 4 patients; the fifth patient had an incomplete right bundle branch pattern. The mean QRS axis was 100 _+ 37 degrees. Echocardiograms demonstrated 2 patients to have normal structure and function. Of the remaining 3 patients, 1 had a hypertrophic left ventricle and 2 had secundum atrial septal defects, with left superior vena cava to coronary sinus in 1. The left ventricular shortening fraction was 33 % to 47 %. Acute treatment of this arrhythmia was attempted in three patients at referring institutions. Adenosine in 1 patient and direct current cardioversion in two patients failed to terminate the arrhythmia. Acute therapy with procainamide in 3 patients, quinidine in 2 patients, and verapamil in I patient, were unsuccessful in terminating CAT. All patients were hospitalized for chronic treatment. Digoxin was initially tried in all patients and was successful as the sole drug in 1 of 5 patients. This patient responded with conversion to sinus rhythm after 2 days of initiating therapy. Amiodarone, a second-line drug used in the 4 remaining patients, was successful in combination with digoxin in I patient. The third line of therapy was a combination of class 1C antiarrhythmic drugs with amiodarone and digoxin. This combination was used in 3 patients and was successful in 2. In patient 1, for whom the previous regimen failed, atenolol was added to the combi~ nation of digoxin, amiodarone, and encainide to control the