608 Brief communications

of left ventricular hypertrophy. The chest radiograph showed an enlarged cardiac silhouette and evidence of chronic obstructive lung disease. At surgery, mitral valve repair was considered, but the valve leaflets were believed to be so abnormal that valve replacement was performed. The floppy mitral valve was excised and was replaced with a 29 mm Carpentier-Edwards bioprosthesis. The excised mitral valve was typical of mitral valve prolapse. The pa- tient was weaned from cardiopulmonary bypass without difficulty; however, upon arrival in the recovery room, she was severely hypotensive and vasopressors were given. Nevertheless, the hypotension worsened, and 3 hours postoperatively, fatal cardiac arrest occurred. Initially, closed-chest cardiac massage was performed. When it was unsuccessful, the chest was opened, and the posterior wall of the left ventricle was found to have ruptured. The tear was repaired. The left ventricular cavity was distended and was difficult to empty by manual compression. (The ven- tricle likely ruptured as a result of external chest compres- sions on the distended, non-emptying left ventricle.) Fur- ther resuscitative efforts were unsuccessful. The heart weighed 425 gm. A 2.5 cm transverse tear in the posterior wall of the left ventricle had been closed with sutures. The ventricular cavities were of normal size. The bioprosthesis nearly occluded left ventricular outflow (Fig. 1). The three other cardiac valves were normal, no foci of myocardial ne- crosis or fibrosis were present, and the epicardial coronary arteries were normal.

In the above-described patient, the substitute cardiac valve inserted was too large (or the heart was too small), and the consequence was extreme obstruction to left ven- tricular outflow. Why was such a large-sized prosthesis in- serted in this small-sized person? The hemodynamic mitral lesion before operation was pure regurgitation, i.e., no element of mitral stenosis, a lesion which when chronic usually results in dilatation of the left ventricular cavity. In the described patient, however, the left ventricular cavity was not dilated preoperatively (confirmed by left ventric- ular angiography). Although the heart was larger (425 gm) than normal in mass, it contained an excessive amount of fat, which probably accounted for the increased weight.

What precautions can a surgeon take to prevent this me- chanical complication of mitral valve replacement? (1) He/she should utilize relatively small-sized prostheses in relatively small-sized persons irrespective of the type of hemodynamic lesion produced by the native valve to be re- placed. (2) If the left ventricular cavity is of normal or near-normal size, the surgeon should err on the side of smaller-sized rather than larger-sized prostheses or bio- prostheses. (3) The surgeon should be aware that any type of prosthesis or bioprosthesis is capable of producing left ventricular outflow obstruction, or specifically that a cen- tral flow substitute valve, as was inserted in the aforede- scribed patient, does not prevent this complication. (4) If the prosthesis or bioprosthesis fills the entire “floor” of the left atria1 cavity, it may be too large for the left ventricle, where the major portion of some substitute valves will re- side. (5) Mechanical obstruction to left ventricular outflow (or inflow) may be suspected when a patient has a difficult

February 1991

Amerlcsn Heart Journal

time being weaned from cardiopulmonary bypass or devel- ops severe hypotension shortly after weaning from bypass. (6) Intraoperatively, echocardiography is useful in diag- nosing this complication.13

REFERENCES

1.

2.

3.

4.

5.

6.

7.

8.

9.

10.

Il.

12.

13.

Roberts WC, Morrow AG. Mechanisms of acute left atria1 thrombosis after mitral valve replacement. Pathologic find- ings indicating obstruction to left atria1 emptying. Am J Car- diol 1966;18:497-503. Roberts WC, Morrow AG. Causes of early postoperative death following cardiac valve replacement. Clinico-pathologic cor- relation in 64 patients studied at necropsy. J Thorac Cardio- vast Surg 1967;54:422-37. Roberts WC, Morrow AG. Anatomic studies of hearts con- taining caged-ball prosthetic valves. Johns Hopkins Med J 1967;121:271-95. Shepard RL, Glancy DL, Stinson EB, Roberts WC. Hemo- dynamic confirmation of obstruction to left ventricular inflow by a caged-ball prosthetic mitral valve. Case report. J Thorac Cardiovasc Surg 1973;65:252-4. Roberts WC, Bulkley BH, Morrow AG. Pathologic anatomy of cardiac valve replacement: a study of 224 necropsy patients. Prog Cardiovasc Dis 1973;15:539-87. Seningen RP, Bulkley BH, Roberts WC. Prosthetic aortic stenosis. A method to prevent its occurrence by measurement of aortic size from preoperative aortogram. Circulation 1974;49:921-4. Roberts WC, Hammer WJ. Cardiac pathology after valve re- placement with a tilting-dies prosthesis (Bjiirk-Shiley type). A study of 46 necropsy patients and 49 Bjiirk-Shiley prostheses. Am J Cardiol 1976;37:1024-32. Roberts WC. Complications of cardiac valve replacement: characteristic abnormalities of prostheses pertaining to any or specific site. AM HEART J 1982;103:113-22. Rahimtoola SH. The problem of valve prosthesis-patient mismatch. Circulation 1978;58:20-4. Pelikan PCD, Chew PH, Fortuin NJ, Yin FCP. Left ventric- ular outflow obstruction caused by a Starr-Edwards mitral prosthesis. Am J Cardiol 1983;52:552-3. Currie PJ, Seward JB, Lam JB, Gersh BJ, Pluth JR. Left ven- tricular outflow tract obstruction related to a valve prosthesis: case caused by a low-profile mitral prosthesis. Mayo Clin Proc 1985;60:184-7. Mihaileanu S, Marino JP, Chauvaud S, Perier P, Forman J, Vissoat J, Julien J, Dreyfus G, Abastado P, Carpentier A. Left ventricular outflow obstruction after mitral valve repair (Car- pentier’s technique). Circulation 1988;78:78-84. Byrnes TJ, Cuillory WR, Hanley HG. Left ventricular outflow obstruction by a mitral valve prosthesis: Doppler ultrasound and cardiac catheterization findings. Cathet Cardiovasc Diagn 1989;17:34-8

Cessation of gastrointestinal bleeding from angiodysplasia after surgery for idiopathic hypertrophic subaortic stenosis

Mohsin Alam, MD, and Joseph W. Lewis, Jr., MD. Detroit, Mich.

From the Heart and Vascular Institute, Henry Ford Hospital.

Reprint requests: Mohsin Alam, MD, Cardiology Dept. K-14, Henry Ford Hospital, 2799 W. Grand Blvd., Detroit, MI 48202.

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Number 2, Part 1 Brief communications 609

Recurrent bleeding from submucosal gastrointestinal an- giodysplastic vessels and severe aortic stenosis has been rep0rted.l Cessation of gastrointestinal bleeding after aor- tic valve replacement has also been well documented.2 In the world literature, there are very few reported cases of bleeding from gastrointestinal angiodysplasias in associa- tion with subvalvular aortic obstruction.3-6 We report a patient with idiopathic hypertrophic subaortic stenosis with severe left ventricular outflow obstruction, severe mi- tral valve insufficiency, and congestive heart failure, who had 11 separate episodes of gastrointestinal bleeding. Af- ter mitral valve replacement, septal myotomy, and myo- mectomy surgery, this patient has had no more bleeding after 18 months of follow-up. To the best of our knowledge, this is the first reported patient with idiopathic hyper- trophic subaortic stenosis who has had no recurrence of gastrointestinal bleeding after surgical relief of subaortic obstruction.

AW, a 74-year-old white woman with a known history of idiopathic hypertrophic subaortic stenosis, presented to us with dark red and tarry stools. She had an l&month his- tory of similar bleeding episodes requiring multiple blood transfusions at an outside hospital. Pertinent laboratory studies in the past included negative upper and lower bowel barium x-ray scans and endoscopies. Superior mesenteric artery arteriography during active bleeding had confirmed bleeding from angiodysplastic vessels of the right colon. The patient had also complained of increasing shortness of breath in the prior 2 months. She was short of breath at rest and had three different episodes of documented pulmonary edema. She was initially treated with a P-blocker and ver- apamil; later diuretics and digoxin were added when par- oxysmal atria1 fibrillation and pulmonary edema occurred. She was referred to the Cardiology Department specifically to evaluate her cardiac status in preparation for colon re- section surgery for bleeding angiodysplasias. Physical ex- amination revealed the patient to be a cachectic, thin in- dividual. She had a brisk carotid pulse with an elevated jugular venous pulse. Her blood pressure was 94/60 mm Hg and her pulse had an irregular rate of t)O/min. There was a systolic thrill present at the apex and the point of maximal cardiac impulse was diffuse and laterally displaced. On auscultation, the heart sounds were normal with an Sq gal- lop. A grade IV/VI holosystolic murmur was best heard at the apex radiating to the axilla. There was also a grade III/ VI systolic ejection murmur at the lower left sternal border. This murmur did not radiate to the carotids nor did it in- crease during the straining phase of a Valsalva maneuver. An electrocardiogram (ECG) revealed normal sinus rhythm, premature atria1 activity, left ventricle hypertro- phy, intraventricular conduction delay, and ST-T changes. A previous ECG had revealed the patient to be in atria1 fi- brillation. An echocardiogram showed asymmetric septal hypertrophy with a septal posterior wall ratio of 1.7 to 1. There was systolic anterior motion of the mitral valve compatible with hypertrophic subaortic stenosis and mod- erate left atria1 dilatation. The left ventricular systolic function was normal. Color flow Doppler study revealed severe mitral valve insufficiency. A chest x-ray film re-

vealed cardiomegaly without pulmonary congestion. Other pertinent laboratory studies included a hemoglobin of 10.8 gm/dl and normal prothrombin and partial thromboplas- tin time. Cardiac catheterization and angiography revealed severe mitral valve insufficiency, hyperdynamic left ven- tricular function, and severe left ventricular outflow ob- struction with a resting gradient of 75 mm Hg. Since the patient was not responding to the usual medical therapy, mitral valve replacement with a bioprosthetic valve, septal myotomy, and myomectomy surgery was performed. The postoperative course was complicated by pneumonitis and heart block, requiring a permanent atrioventricular pace- maker. Since her surgery 18 months ago (as of this writing), the patient’s exercise tolerance has improved and she has no further episodes of gastrointestinal bleeding. Her post- operative echocardiogram and cardiac Doppler studies have revealed no significant left ventricular outflow ob- struction and a normal functioning bioprosthetic mitral valve.

Our report suggests that surgical correction of left ven- tricular outflow obstruction in patients with idiopathic hy- pertrophic subaortic stenosis may not only relieve cardiac symptoms but also control gastrointestinal bleeding from colonic angiodysplasias, when present. Cardiac surgery may be considered over colon resection in selected individ- uals with both severe left ventricular outflow obstruction and bleeding angiodysplasias. In our patient, P-blocker and verapamil therapy were not effective in reducing left ven- tricular outflow obstruction or in preventing gastrointesti- nal bleleding. Cessation of bleeding, however, was dramatic after cardiac surgery and was not likely to be just coinci- dental. The exact explanation for this phenomenon is not known. We believe that the same hypothesis advocated for patients with severe aortic stenosis and bleeding from in- testinal angiodysplasia may also apply to subvalvular aor- tic stenosis. Boley et a1.l suggest that gastrointestinal an- giodysplasias are acquired with aging due to intermittent venous obstruction in the bowel. These lesions may bleed more frequently with aortic stenosis due to mucosal ischemia.l In conclusion, angiodysplasia should be sus- pected as a possible cause of gastrointestinal hemorrhage in the appropriate clinical setting-in the elderly patient, in those with recurrent painless hemorrhage, in those hav- ing persistent iron deficiency anemia, etc. The presence of idiopathic hypertrophic subaortic stenosis should not make any difference in the differential diagnosis. Additionally, this case suggests that surgical reduction of left ventricu- lar outflow tract obstruction may have a beneficial effect on angiodysplastic bleeding.

REFERENCES

1. Boley SJ, Sammartano R, Adams A, DiBase A, Kleinhaus S, Spmvregen S. On the nature and etiology of vascular ectasias of the colon. Gastroenterology 1977;72:650-60.

2. Cane11 MS. Lebwohl 0. Cessation of recurrent bleeding from gastrointestinal angiodysplasias after aortic valve replace- ment. Ann Intern Med 1986;105:54-7.

3. Kimmel DJ, Paster SB, Burstein J, Stovring J, Cochran PT. Cardiac disease and recurrent gastrointestinal bleeding. An-

6 10 Brief communications

giodysplasia with accompanying hypertrophic subaortic steno- sis. Rocky Mt Med J 1977;74:318-19. Bonneau A, Firouz-Abadi M, Cognault JP, Couradeau B, Du- mas M. Angiodysplasie de L’ileon et cardiomyopathie. Ann Cardiol Angeiol (Paris) 1984;33:389-94. Waxman BP, Buzzard AJ, Cox J, Stephens MJ. Gastric and intestinal bleeding in multiple neurofibromatosis with cardi- omyopathy. Aust NZ J Surg 1986;56:171-3. Peris P, Campa MT, Coca A. Angiodisplasia intestinal aso- ciada a estenosis subaortica hipertrofica. Med Clin (Barcelo- na) 1989;92:599.

Reversible catecholamine-induced cardiomyopathy

R. Wood, BSc, BM, BCh, P. J. Commerford, MBChB, A. G. Rose, MD,” and A. Tooke, MBChB. Cape Town, South Africa

A young adult female presented with a a-week history of cardiac failure. Echocardiography and radionuclide angio- cardiography initially demonstrated a nondilated left ven- tricle with severely impaired systolic function. The acute clinical presentation following a feverish prodrome to- gether with the histologic features on endomyocardial bi- opsy were felt to be in keeping with a diagnosis of post- viral myocarditis. The subsequent clinical course, however, was more suggestive of a pheochromocytoma, which was confirmed by biochemical and imaging techniques. A right adrenal mass was surgically removed and the subsequent improvement of cardiac function to normal is documented over a 36-month follow-up period.

Case presentation. A 34-year-old woman of mixed race presented in cardiac failure with a 2-week history of fever, diaphoresis, dyspnea, and a nonproductive cough. She had previously been completely well except for an ectopic pregnancy at the age of 20 years. Examination revealed profuse diaphoresis with a normal temperature, a sinus tachycardia of 130/min, and a blood pressure of 120/75 mm Hg. Venous pressure was not elevated; there was no clini- cal cardiomegaly; on auscultation there was a prominent summation gallop; the lung fields were clear. The electro- cardiogram showed a sinus tachycardia, an axis of +60 de- grees, a prolonged QT interval (0.29 second), and wide- spread T wave flattening and inversion. A chest x-ray film showed the heart size to be at the upper limit of normal, with clear lung fields. M-mode and two-dimensional echocardiography demonstrated a normal-sized left ven- tricle with a reduced fractional shortening of 15”. and an enlarged left atrium; mitral and tricuspid regurgitation were noted on the Doppler study. Red cell ketolase, thyroid

From the Cardiac Clinic, Department of Medicine and aDepartment of An- atomical Pathology, University of Cape Town and Groote Schuur Hospital.

Reprint requests: Dr. R. Wood. Cardiac Clinic, Groote Schuur Hospital, Observatory. 7925, Cape Town, South Africa.

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American Heart Journal

function tests, antistreptococcal antibody titer (ASOT), and viral studies were normal. There was symptomatic re- sponse to bed rest and diuretic therapy.

Two weeks later the patient represented with an exacer- bation of her symptoms; she was now bed-bound with a dry cough and dyspnea on minimal effort. Examination again revealed profuse diaphoresis with cold peripheries, a thready pulse of 140/min with alternans, a venous pressure elevated at 4 cm with a prominant V wave in the neck, and a blood pressure of 105/80 mm Hg. The apex was now dis- placed and diffuse, there was a prominent summation gal- lop, and the murmurs of tricuspid and mitral regurgitation were noted. A mobile chest x-ray film showed a bulky- looking heart with congested lung fields. A right heart catheter study showed the following pressures (in millime- ters of mercury): right atria1 mean, 9; right ventricle, 55/12-16; pulmonary artery mean, 45; pulmonary wedge, a46, ~36, with a mean of 37. An endomyocardial biopsy showed a mild focal interstitial infiltrate of lymphocytes with scanty myocytolysis (Fig. 1) but no myocyte hyper- trophy nor fibrosis. A presumptive diagnosis of post-viral myocarditis was made and treatment commenced with prednisone and intravenous dobutamine. Initially the pa- tient remained dependent on the inotrope; when the dose was lowered, the wedge pressure would rise and the mean arterial pressure would fall. After the introduction of digoxin and captopril, it was possible to withdraw the do- butamine slowly over a 4-day period. At the end of this pe- riod, she was undistressed at rest; heart rate was 80/min; mean arterial pressure was 110 mm Hg; pulmonary wedge pressure was 10 mm Hg; cardiac output was 5.73 L/min; and the systemic vascular resistance was 18.5 units. Equi- librium radionuclide angiocardiography (ERNA) demon- strated focal wall movement abnormalities and a reduced left ventricular ejection fraction of 35 ST;, . Four days later she became acutely hypotensive and the blood pressure was unrecordable but responded to intravenous fluids; how- ever, the following day hypertension was noted for the first time (blood pressure of 190/140 mm Hg). She now became very unstable with wild fluctuations in blood pressure from 80/50 to 180/116 mm Hg, pulmonary capillary wedge pres- sure from 2 to 44 mm Hg, and pulmonary artery pressures from 18/8 to 60/40 mm Hg. A diagnosis of a pheochromo- cytoma was confirmed by an elevated urinary normeta- nephrine (NMA) level of 43.5 Fmo1/24 hours. Computer axial tomography demonstrated a right adrenal mass, which was confirmed by a positive 1131 meta-iodoben- ziguanidine scan. A 9 x 5 cm pheochromocytoma was removed at surgery following stabilization of the blood pressure with the a-blocker phenoxybenzamine. The pre- operative left ventricular ejection fraction measured by ERNA was 26 % . The postoperative course was uneventful, with continued clinical improvement. Initially she was hy- potensive with blood pressures recorded at 80/60 mm Hg, but from 6 months onward she has remained normotensive. The patient now leads a completely normal life without any therapy. The electrocardiogram was normal at 12 months, urinary NMAs have remained within normal limits, and repeat echocardiography (Fig. 2) and ERNAs (Fig. 3) have