Pulmonic balloon valvuloplasty in adults

186

January 1990 American Heart Journal

Brief Communications

1S Fig. 2. T•rsadesdep•intes.Aself-terminatingepis•de•fp•lym•rphictachycardia•initiatedbya``late•• ventricular p r e m a t u r e beat, occurred on the downslope of the T wave. P a p e r speed = 25 mm/sec. mmol/L) b u t became normal after 2 days without treatment. Alcohol excess was denied by the p a t i e n t at the time of admission, b u t it later emerged t h a t he had been drinking heavily during the p r e c e d i n g week. Echocardiography and exercise testing were normal. Blood pressure remained normal and antihypertensive t h e r a p y was not recommenced. The association between Q T prolongation and torsades de pointes is well established, 1, 2 b u t the quantitative relationship is less clear. 5 No critical QT duration has been identified and not all clinical syndromes with long Q T exhibit the arrhythmia, indicating t h a t it is not simply the length of the Q T interval t h a t is arrhythmogenic. Dispersion and not just prolongation of repolarization is necessary for the development of the arrhythmia. 5 Successful shortterm t r e a t m e n t of torsades de pointes is directed not only at shortening the QT interval b u t at unifying repolarization, as can be achieved by pacing. 2 Short-term drug treatm e n t of torsades de pointes is less predictable. Lidocaine will suppress the a r r h y t h m i a in less than half of cases. 7, 8 Class l a drugs, which m a y cause further QT prolongation, are rarely effective and m a y exacerbate the arrhythmia. 2; s Similarly, the class III drug bretylium has been reported as relatively ineffective 7 or proarrhythmic. 8 Amiodarone, another class III drug, m a y cause prolongation of the Q T interval and torsades de pointes, s, 9 Proarrhythmia, however, is uncommon with amiodarone and QT prolongation is more often associated with a n t i a r r h y t h m i c therapeutic efficacy) Combinations of drugs t h a t prolong the QT interval have been advised against because of concern about increased arrhythmogenic potential. 2, 7 This might have been expected in the present case, as the use of amiodarone to treat sotalol-induced torsades de pointes was associated with marked prolongation of the Q T interval. T h e opposite occurred, however, with suppression of the ventricular arrhythmias. The efficacy of amiodarone may be due simply to suppression of ventricular ectopy, which often precedes torsades de pointes, 7 or may d e p e n d on its correcting dispersion of repolarization, 5 by prolonging action potential duration throughout the h e a r t ) ~ A previous study s of drug-induced polymorphic ventricular tachycardia (which included b u t was not limited to torsades de pointes) reported one case of successful short-term t r e a t m e n t with intravenous amiodarone, b u t without details of its effects on the QT interval. These same investigators also found amiodarone to be useful as alternative long-term antiarrhythmic therapy, often in spite of QT interval prolonga-

tion. However, there is insufficient evidence to recommend amiodarone as the short-term t r e a t m e n t of choice for torsades de pointes, which remains atrial or ventricular pacing,2, 7 b u t we p r e s e n t this case to illustrate the arrhythmic and antiarrhythmic associations of QT prolongation.

REFERENCES 1. Schwartz PJ. Idiopathic long QT syndromes: progress and questions. AM HEART J 1985;109:399. 2. Stratmann HG, Kennedy HL. Torsades de pointes associated with drugs and toxins: recognition and management. AM HEART J 1987;113:1470. 3. Roden DM, Woosley RL. QT prolongation and arrhythmia suppression. AM HEART J 1985;109:411. 4. Torres V, Tepper D, Flowers D, Wynn J, Lam S, Keefe D, Miura D, Somberg JC. QT prolongation and the antiarrhythmic efficacy of amiodarone. J Am Coll Cardiol 1986;7:]42. 5. Surawicz B, Knoebel SB. Long QT: good, bad or indifferent? J Am Coll Cardiol 1984;4:398. 6. McKibbon JK, Pocock WA, Barlow JB, Scott Millar RN, Obel IWP. Sotalol, hypokalaemia, syncope, and torsades de pointes. Br Heart J 1984;51:157. 7. Kay GN, Plumb VJ, Arciniegas JG, Henthorn RW, Waldo AL. Torsades de pointes: the long-short initiating sequence and other clinical features: observations in 32 patients. J Am Coll Cardiol 1983;2:806. 8. Nguyen PT, Scheinmann MM, Seger J. Polymorphous ventricular tachycardia: clinical characterization, therapy, and the QT interval. Circulation 1986;74:340. 9. Sclarovsky S, Lewin RF, Kracoff 0, Strasberg B, Arditti A, Agmon J. Amiodarone-induced polymorphous ventricular tachycardia. AM HEART J 1982;105:6. 10. Yabek SM, Kato R, Singh BN. Effects of amiodarone and its metabolite, desethylamiodarone, on the electrophysiologic properties of isolated cardiac muscle. J Cardiovasc Pharmacol 1986;8:197.

P u l m o n i c b a l l o o n v a l v u l o p l a s t y in a d u l t s Warren Sherman, MD, a Ronnie Hershman, MD, a Dimitrios Alexopoulos, MD, a Marc Cohen, MD, a J a y Midwall, MD, c Joshua Kieval, MD, c and Richard Golinko, MD. b

New York, N.Y., and Atlantis and Miami, Fla. From the Divisionsof aCardiologyand bpediatric Cardiology,Mount Sinai Hospital, New York; and eJohn F. Kennedy Medical Center, Atlantis; and the University of Miami, School of Medicine. Reprint requests:Warren Sherman, MD, Divisionof Cardiology,Box 1030, Mount Sinai Hospital, New York, NY 10029. 4/4/16723

Volume 119 Number

1

Balloon dilatation of stenotic heart valves has become a useful treatment for certain subsets of adult patients. The indications for and techniques of this procedure are evolving for left-sided valve lesions. ~ For right-sided valve lesions, the procedure is less well studied in the adult population. Pulmonic valvular stenosis is not uncommon in adults with congenital heart disease. 2 When causing symptoms, it usually does so in young or middle-aged adults, in whom the valvular stenosis has progressed. The use of balloon dilatation in the treatment of pulmonic valvular stenosis was first reported in 19793 in children; the utility of this procedure in older adults has not been reported. We have recently treated four adult patients with pulmonic valvular stenosis with balloon valvuloplasty, including two with the remarkable finding of dense tricuspid annular calcification. Their cases are herein discussed. Patients were considered to be candidates for this procedure if the following existed: (1) Pulmonic valvular stenosis as determined by physical examination and echocardiographic criteria. (2) Peak valvular gradient equal to or greater than 65 mm Hg as determined by cardiac catheterization or Doppler echocardiography. (3) The absence of other significant valvular, myocardial, coronary artery, or other congenital disease. Four such patients meeting these criteria were referred for the dilatation procedure. Following informed consent, a right-sided heart catheterization with either a 7F thermodilution catheter (three patients) or a 7F end-hole nonthermodilution catheter (one patient), and a systemic arterial catheterization with a 5F catheter were performed transfemorally. Two to five thousand units of heparin were given intravenously and hemodynamics with thermodilution cardiac outputs (three patients) or Fick cardiac output 4 were measured. A second right-sided heart flotation catheter was introduced into the pulmonary artery. This,catheter was then exchanged over a guide wire for the balloon dilating catheter (Mansfield Scientific, Mansfield, Mass.). In one patient (case No. 2), a second dilating catheter was inserted through an additional femoral venous puncture site. The following balloon sizes (outer diameter) were used, having been chosen to exceed the pulmonic anulus size by 10%: 20 mm (case No. 1); two 12 mm (double balloon technique, case No. 2); 20 mm (case No. 3); and 18 mm (case No. 4). Two or three inflations, lasting 10 to 30 seconds each, were generally applied. Hemodynamics were repeated. After the procedure, all catheters were removed; discharge occurred the following day. Clinical follow-up was obtained by telephone review. Case No. 1. A 61-year-old woman with a murmur since birth presented with progressive exertional fatigue (New York Heart Association5 class 2) over the previous 6-month period. Previously, she had been in good health. Prior to a work-up for gastric ulcer disease, an echocardiogram demonstrated severe pulmonic stenosis and she was referred for cardiac catheterization and possible balloon valvuloplasty. She was taking no medications. On examination, the vital signs were normal. There were prominent atrial waves in her jugular pulse. A right ventricular lift was prominent. The first heart sound was normal and the second sound was single. A grade 4/6 systolic murmur, which augmented

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Fig. 1. Case No. 1 Upper panel, Left lateral view of a balloon catheter traversing the superior aspect of the tricuspid valve. Lower panel, The early phase of a right coronary artery (RCA) injection in the 60-degree left anterior oblique projection. Arrows indicate calcification in the tricuspid anulus.

markedly with inspiration, was observed in the third left interspace. There was no peripheral edema. An electrocardiogram showed normal sinus rhythm, a QRS axis of +100 degrees, right atrial conduction abnormality, and right ventricular hypertrophy. On chest radiography there was right ventricular prominence and an enlarged right ventricular outflow tract. An echocardiographic and Doppler flow study displayed a thickened pulmonic valve with reduced excursion and doming, marked right ventricular hypertrophy, a peak instantaneous gradient across the pulmonic valve of 80 mm Hg, and mild tricuspid insufficiency.

1 88

January 1990

-----Brie[ Communications

American Heart Journal

BEFORE

l"

,see--

AFTER

~-I sec-~

"l

ECG~

ECG ~

200

20O

I00

J

100

"

RV PA

'

Fig. 2. Hemodynamic recordings before and after balloon dilatation in case No. 1. RV, Right ventricle; PA, pulmonary artery.

]'able i. Hemodynamic data

RA mean

Case 1 Pre Post Case 2 Pre Post Case 3 Pre Post Case 4 Pre Post

RV

PA

PA Mean

PAW

Ao

Mean pulmonic gradient

CO

9 10

170/8 65/5

25/12 20/8

16 13

12 --

130/95 135/95

115 32

4.8 5.0

2 --

95/5 50/2

18/6 14/4

9 8

---

135/70 138/80

64 35

4.4 4.5

10 --

169/7 70/10

22/3 21/8

13 13

---

164/70 --

129 38

2.7 4.2

25/5 30/15

15 22

5 --

110/70 --

58 20*

5.0 4.8

2 --

90/5 50/10"

Pressures in millimeters of mercury (mm Hg). RA, Right atrial; RV, right ventricular; PA, pulmonary arterial; PAW, pulmonary artery wedge; Ao, aortic; CO, cardiac output (L/min), pre, predilatation; post, postdilata~ion. *Represents subpulmonic valvular pressure; there was a 20 mm Hg gradient across the infundibulum.

The pulmonic anulus diameter was 18 mm. Right ventriculography demonstrated an elongated right ventricuiar outflow tract, a mildly calcified and domed pulmonic valve, and moderately dilated main pulmonary artery. There was a markedly calcified tricuspid anulus (Fig. 1). Hemodynamics are listed in Table I. A peak gradient of 130 m m Hg across the pulmonic valve decreased immediately after dilatation to 70 m m Hg, and 10 minutes later to 40 m m Hg (Table I and Fig. 2). Eight months following dilatation, the patient has returned to NYHA class i functional status; a repeat Doppler study demonstrated a 30 m m Hg valvular gradient. Case No. 2. A 48-year-old woman was discovered at 8 years of age to have severe pulmonic stenosis, and at age 17 required right ventricular infundibular resection and pulmonic valvotomy. She remained symptom-free thereafter. At age 48, due to the recurrence of physical and echocardiographic findings consistent with severe restenosis, the patient was referred for cardiac catheterization. On physical examination, she was found to be normotensive with a

heart rate of 75 beats/min. There was no cyanosis or edema. There was a right ventricular lift, the first heart sound was normal, and the second heart sound was widely split with a diminished pulmonic closure sound. A loud ejection click as well as a grade 3 to 4/6 systolic m u r m u r were heard in the pulmonic area. The electrocardiogram showed normal sinus rhythm, an axis of +80 degrees, mildly delayed right ventricular forces, and a right atrial conduction abnormality. At echocardiography there was a pliable b u t stenotic pulmonic valve, a peak instantaneous gradient of 90 m m Hg (mean gradient of 65 m m Hg), and tricuspid regurgitation. The pulmonic valve anulus measured 19 m m in diameter. Cardiac catheterization (Table I) demonstrated a 64 mm Hg peak-to-peak gradient across the pulmonic valve. Right ventriculography confirmed the presence of a domed and thickened pulmonic valve, with marked dilatation of the main pulmonary artery. Balloon dilatation resulted in a reduction of the peak-to-peak gradient to 34 m m Hg, without a change in cardiac output. Case No. 3. A 67-year-old woman with a history of type

Volume 119 Number 1

2 diabetes mellitus, carcinoma of the breast, and pulmonic stenosis developed progressive exertional dizziness and dyspnea over a 4- to 6-month period. There was also a history of a chest pain syndrome for which verapamil, 240 mg per day, and oral nitrates had been given. She was taking hydrochlorothiazide and triamterene for dyspnea. On examination the vital signs were normal. Atrial waves were prominent in the jugular venous pulse. The first and Second heart sounds were normal and there was a grade 4/6 ejection type murmur at the second left interspace without clicks. The remainder of her examination was normal. The electrocardiogram revealed normal sinus rhythm, an axis of +110 degrees, and right ventricular hypertrophy. There was cardiomegaly and marked prominence of the proximal and main pulmonary arteries. An echocardiogram-Doppler study demonstrated an 85 mm Hg peak instantaneous gradient. At cardiac catheterization there was moderate calcification of the tricuspid anulus. The pulmonic valve was domed and moderately calcified. The coronary arteries were normal. With dilatation the mean gradient decreased from 129 to 38 mm Hg, with an increase in cardiac output from 2.7 to 4.2 L/min, Symptoms returned to baseline and have remained so 1 year later. A follow-up echocardiogram demonstrated a residual 30 to 40 mm Hg gradient. Case No. 4. A 52-year-old woman presented with 6 months of progressive exertional fatigue classifying her status as NYHA class 3. Pulmonic stenosis had recently been documented by echocardiography. On physical examination the vital signs were normal. Jugular venous pulsations were of normal amplitude but with prominent atrial waves. There was a parasternal lift, a normal first heart sound, a decreased pulmonic closure sound, and a grade 3/6 mid-peaking systolic murmur at the left sternal border that increased with inspiration. There was no pedal edema. An electrocardiogram revealed normal sinus rhythm, a QRS axis of +120 degrees, and right ventricular hypertrophy with secondary repolarization "abnormalities. A twodimensional echocardiogram displayed a thickened and domed pulmonic valve, right ventricular hypertrophy, and a pulmonic anulus dimension of 18 mm. Catheterization data are listed in the Table I. Following dilatation there was a residual 20 mm Hg subvalvular gradient. On follow-up the patient has reverted to New York Heart Association class 1. A repeat echocardiogram 6 months later demonstrated a 15 mm Hg gradient across the pulmonic valve. Pulmonic valvular stenosis is a rare finding in the general adult population. Symptoms of easy fatigabi!ity and exertional dyspnea usually occur when the valve gradient exceeds 70 mm Hg, at which time progressive right ventricular dysfunction may ensue. The management of patients with pulmonic valve stenosis has been based on medical and surgical treatment. However, since 1979 when the first balloon dilatation was employed in an infant with pulmonic stenosis, expansion of this technique has occurred to include young adults. 6, 7 The cases illustrated above represent the first reported series demonstrating the ease and utility of performing balloon dilatations in older adults (age>45 years) with pulmonic valvular stenosis, and document the very unusual finding of calcification of the tricuspid anulus in two such patients.

Brief Communications

189

The technique used for pulmonic balloon valvulop|asty differs significantly from that of aortic valve dilatation. 1 First, a transvenous antegrade approach using a flotation catheter simplifies later passage of dilatation catheters. Second, and most important, the choice of balloon dilating catheter size varies with each situation. In dilatation of the aortic valve, the use of a balloon with a diameter comparable to (or larger than) the size of the valve anulus is ill advised due to the dense valvular scarring and calcification that are frequently present and to the increased incidence of valvular insufficiency. However, in pulmonic valve dilatations, the balloon size is chosen to exceed the anulus diameter, as such scarring and calcification are rarely present. in addition, the pulmonic insufficiency that may result is generally well tolerated. The safety of "oversizing" has been demonstrated in children, in whom balloon diameters of 10 % to 20 % in excess of pulmonic anulus diameter are used. The role of double-balloon, or "eccentric" dilatations in adults with pulmonic stenosis is not clear, although in Children it is of proven efficacy. It should be of similar value in adults, since the congenital deformity of the valve is similar in both age groups. This is in contradistinction to aortic valve dilatations in the adult, in whom most valves are trileaflet and therefore likely to resPond to a more concentric dilatation. The excellent hemodynamic results obtained in our patients correlate well with clinical efficacy. In all patients the valve gradient fell to less than 40 mm Hg, a reduction of between 45 % and 72 %. In one patient a residual gradient was caused largely by a subpulmonic obstruction. Such findings have been reported s and are known to be temporary; infundibular stenosis recedes along with the generalized right ventricular hypertrophy following balloon pulmonic valvuloplasty. Post-dilatation echocardiograms confirmed the gradient reductions observed at catheterization, and also documented mild pulmonic insufficiency in all patients. Our patient who underwent double balloon dilatation had a 50% reduction in valve gradient, reducing right ventricular systolic pressure to a level similar to that achieved with single balloon dilatation in the other three patients. In follow-up all patients are asymptomatic at 2 to 8 months. At 6 to 8 months follow-up, noninvasive evaluation in three of four patients has shown persistent gradient reductions. Two of our four patients demonstrated dense tricuspid annular calcification with normal-appearing tricuspid valves at echocardiography. To our knowledge, there have been no previous descriptions of this entity. The mechanisms responsible for tricuspid annular calcification are probably multifold. In severe right ventricular hypertrophy; the systolic stress placed on the tricuspid valve and its anulus is high, and over a period of years may predispose the annular tissue to fibrosis and calcification. As in patients with mitral annular calcification, 9where increased age, female gender, and ventricular systolic blood pressure are independently associated with this entity, so too may tricuspid annular calcification be affected by these variables. In summary, pulmonic valvular stenosis in older adults responds favorably over the short term to balloon dilatation; long-term follow-up is required in this small population of patients in order to compare it with results

January 1990

190

Brief Communications

American Heart Journal

seen with surgical correction. The tricuspid annular calcification observed in two of our patients raises the question of the role of systolic ventricular dynamics in the pathophysiology of this entity. REFERENCES

1. McKay RG. Balloon valvuloplasty for treating pulmonic, mitral and aortic valve stenosis. Am J Cardiol 1988;61:102G108G. 2. Hoffman JIE, Christianson t~. t~ongemtal heart disease in a cohort of 19,502 births with long-term follow-up. Am J Cardiol 1978;42:641-7. 3. Kan JS, White RI Jr, Mitchell SE, Gardner TJ. Percutaneous balloon valvuloplasty: a new method for treating congenital pulmonary valve stenosis. N Engl J Med 1985;56:527-32. 4. Grossman W. Blood flowmeasurement and the cardiac output. In: Grossman W, ed. Cardiac catheterization and angiography. Philadelphia: Lea & Febiger, 1980:93-8. 5. The Criteria Committee of the New York Heart Association. Nomenclature and criteria for diagnosis of diseases of the heart and great vessels. Boston: Little, Brown & Co, 1973. 6. Pepine CJ, Gessner IH, Feldman RL. Percutaneous balloon valvuloplasty for pulmonic valve stenosis in the adult. Am J Cardiol 1982;50:1442-5. 7. Cooke JP, Seward JB, Holmes DR. Transluminal balloon valvotomy for pulmonic stenosis in an adult. Mayo Clin Proc 1987;62:306-11. 8. Ben-Sachar G, Cohen MH, Sivakoff MD, et al. Development of obstruction after percutaneous pulmonary balloon valvuloplasty, J Am Coll Cardiol 1985;5:754-6. 9. Savage DD, Garrison RJ, Castellt WP, e t al. Prevalence of submitraI (annular) calcium and its correlates in a general population-based sample (The Framingham Study). Am J Cardioi 1983;51:1375-8.

Aortic rupture following percutaneous catheter balloon coarctoplasty in an adult Daniel L. Kulick, MD, Adam Kotlewski, MD, Richard J. Hurvitz, MD. Michael Jamison, MD. and Shahbudin H. Rahimtoola, MB. FRCP.

Los Angeles, Calif.

Percutaneous catheter balloon coarctoplasty (CBC) has been demonstrated to be an effective short-term alternative to surgery in the t r e a t m e n t of congenital coarctation of the aorta. 1-6 While local aneurysm formation has been observed following the procedure, 6s overt aortic rupture necessitating emergency surgical intervention has not to our knowledge been previously described, except in a 1-week-old infant following recrossing of a freshly dilated coarction. 9 We report a case of a young adult with unoperFrom the Section of Cardiology, Department of Medicine. and the Section of Thoracic and Cardiovascular Surgery, Division of Surgery, University of Southern California School of Medicine and the Los Angeles County University of Southern California Medical Center. Reprint requests: Daniel L. Kulick. MD. Cardiac Catheterization Laboratory, Section of Cardiology, USC School of Medicine. 2025 Zonal Ave.. Los Angeles. CA 90033. 4/4/16722

Fig. 1. Contrast aortography, revealing a discrete coarctation of the aorta below the left subclavian artery. The Coarctation measures 7 mm at its narrowest diameter, with the adjacent thoracic aorta proximal to the coarctation measuring 20 m m in diameter.

ated congenital aortic coarctation in whom this complication occurred following CBC. A 30-year-old Hispanic woman with a 10-year history of hypertension treated with (~-adrenergic blocking agents presented to Los Angeles County-University of Southern California Medical Center with complaints of headache and bilateral lower extremity claudication. Blood pressure in both upper extremities was 210/110 mm Hg. F u n d n seopic examination revealed grade II hypertensive changes. There was no evidence of congestive heart failure on physical examination. Cardiac examination revealed normal jugular venous pulsations, a forceful left ventricular impulse, an $4 gallop, a prominent aortic component of the second heart sound, and a systolic ejection m u r m u r along the left sterna] border. Femoral pulses were delayed and diminished bilaterally, and no pulses were palpable distal to ~his level. Laboratory examination was unremarkable. Chest x-ray films revealed a normal cardiac silhouette with rib notching. Electrocardiography revealed sinus rhythm, left atrial abnormality, and nonspecific T wave changes. Two-dimensional echocardiography revealed concentric left ventricular hypertrophy and a trileaflet aortic valve. Doppler examination revealed no significant aortic valve gradient or regurgitation. Aortic angiography was performed through a pigtail catheter in the transverse aorta, which had been inserted percutaneously from the right brachial artery, and demonstrated a discrete (2 m m in length) coarctation in the descending thoracic aorta distal to the left subclavian artery (Fig. 1). The diameter of the