Download Stenting To Reverse Left Ventricular Ischemia Due To Left Main

9 mm Hg and an EF of 0.62. Left ventriculography showed mild
hypokinesis of the basal anterior wall. Coronary arteriography
revealed CO-LMCA with extensive right-to-left collateral channels filling the LAD and LCX. The dominant RCA had a proximal
75% stenosis. The patient underwent bypass grafting of the LAD
and the LCX. The grafts were patent 3 months later, and the
patient has been free of symptoms during exertion. A stress 201Tl
myocardial SPECT scan showed no abnormality.
transaxial emission computed tomography: quantitative versus qualitative analysis for evaluation of coronary artery
disease. J Am Coll Cardiol 1984; 4:1213–1221
9 Topaz O, Disciascio G, Cowley MJ, et al. Complete left main
coronary artery occlusion: angiographic evaluation of collateral vessel patterns and assessment of hemodynamic correlates. Am Heart J 1991; 121:450 – 456
Discussion
The patients described in this report had characteristic
scintigraphic findings on stress 201Tl myocardial SPECT
scans. A distinct decrease in the 201Tl washout rate was
most prominent in the basal anterior and/or anterolateral
wall, where a decreased 201Tl washout rate is not usually
observed in patients with ischemic heart disease. In the
setting of proximal stenosis of the LAD, the most prominent redistribution and decreased washout rate are commonly found in the mid to apical region of the anterior LV
wall distal to the lesion on stress 201Tl myocardial SPECT
scanning7,8 (Fig 1, bottom, D and Fig 2, bottom, D). In
contrast, in the setting of CO-LMCA with extensive
right-to-left collateral channels, the LAD and the LCX
territories are perfused in a retrograde manner by collateral flow. Therefore, the basal anterior and anterolateral
wall of the LV, which is closest to the LMCA and furthest
from the RCA, should be the most ischemic area during
exercise.9 This could explain the unique scintigraphic
pattern seen in the patients with CO-LMCA. The differences in the scintigraphic patterns between patient 1 and
patient 3 might be caused by the extent of the collateral
channels to LCX, which were less extensive in patient 1.
In summary, we described three patients with COLMCA who had decreased uptake and washout rates in
the basal anterior and anterolateral wall of the LV on stress
201
Tl myocardial SPECT scans. We suggested that this
unusual characteristic scintigraphic pattern may be a
specific finding for this rare condition and may be useful
for the noninvasive diagnosis of CO-LMCA.
References
1 Frye RL, Gura GM, Chesebro JH, et al. Complete occlusion of
the left main coronary artery and the importance of coronary
collateral circulation. Mayo Clin Proc 1977; 52:742–745
2 Goldberg S, Grossman W, Markis JE, et al. Total occlusion of
the left main coronary artery. Am J Med 1978; 64:3– 8
3 Greenspan M, Iskandrian AS, Segal BL, et al. Complete
occlusion of the left main coronary artery. Am Heart J 1979;
98:83– 86
4 Zimmern SH, Rogers WJ, Bream PR, et al. Total occlusion of
the left main coronary artery: the coronary artery surgery
study (CASS) experience. Am J Cardiol 1982; 49:2003–2010
5 DePace NL, Kimbiris D, Iskandrian AS, et al. Total occlusion
of left main coronary artery without angina pectoris. Arch
Intern Med 1983; 143:1064 –1065
6 Topatz O. Total left main coronary artery occlusion: the acute,
the chronic, and the iatrogenic. Chest 1992; 101:843– 846
7 Rigo P, Bailey IK, Griffith LSC, et al. Value and limitations of
segmental analysis of stress thallium myocardial imaging for
localization of coronary artery disease. Circulation 1980;
61:973–981
8 Tamaki N, Yonekura Y, Mukai T, et al. Stress thallium-201
Stenting To Reverse Left
Ventricular Ischemia Due To
Left Main Coronary Artery
Compression in Primary
Pulmonary Hypertension*
Stuart Rich, MD, FCCP; Vallerie V. McLaughlin, MD; and
William O’Neill, MD
Angina is a common symptom of severe pulmonary
hypertension. Although many theories for the source
of this pain have been proposed, right ventricular
ischemia is the one most commonly accepted as the
cause. We report on two patients with primary pulmonary hypertension who had angina with normal
activity or on provocation. One patient had severe
left ventricular dysfunction. Both were found to have
severe ostial stenosis of the left main coronary artery
as a result of compression from a dilated pulmonary
artery. Both patients underwent stenting of the left
main coronary artery with excellent angiographic
results, and complete resolution of the signs and
symptoms of angina and left ventricular ischemia.
Left ventricular ischemia due to compression of the
left main coronary artery may be a much more
common mechanism of angina and left ventricular
dysfunction in patients with pulmonary hypertension
than previously acknowledged. Stenting of the coronary artery can be done safely with the resolution of
these symptoms.
(CHEST 2001; 120:1412–1415)
Key words: coronary artery stenting; left main coronary stenosis;
primary pulmonary hypertension
Abbreviation: PPH ⫽ primary pulmonary hypertension
ngina, like chest pain, commonly has been associated
A with
the development of primary pulmonary hyper-
tension (PPH).1 The etiology of the chest pain has been
debated, with theories ranging from painful dilatation of
*From the Section of Cardiology (Drs. Rich and McLaughlin),
Rush Medical College, Chicago, IL; and the Division of Cardiology (Dr. O’Neill), William Beaumont Hospital, Royal Oak, MI.
Manuscript received November 28, 2000; revision accepted
March 27, 2001.
Correspondence to: Stuart Rich, MD, FCCP, the Rush Heart
Institute, Center For Pulmonary Heart Disease, Rush-Presbyterian-St. Luke’s Medical Center, 1725 West Harrison St, Suite
020, Chicago, IL 60612-3824; e-mail: [email protected]
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Selected Reports
the pulmonary artery to overt right ventricular ischemia.2
Because most patients with PPH are young, it has not
been suggested that their angina is a result of coronary
artery disease. Similarly, left ventricular dysfunction has
been described in patients with PPH, the mechanism of
which is also uncertain.3,4 Many investigators4 have
pointed to the shared interventricular septum, with left
ventricular dysfunction a consequence of ventricular interdependence. Ischemia of the left ventricle has not been
suggested as a mechanism.
We describe two patients who presented with PPH and
angina that was found to be attributable to extrinsic
compression of the left main coronary artery by the
pulmonary artery. Both patients underwent successful
stenting of the left main coronary artery, resulting in the
resolution of their symptoms of angina and manifestations
of left ventricular ischemia. Coronary angiography was
essential in making the diagnosis.
Case Reports
permanent AV sequential pacemaker had been placed in the
patient’s chest at an outside hospital to treat the syncopal
episodes. Her current evaluation included an electron beam CT
scan, which was negative for coronary artery calcium, and an
echocardiogram, which revealed right ventricular dilatation and
pulmonary hypertension, as well as a normal sized left ventricle
with a markedly decreased ejection fraction (25%) and regional
wall motion abnormalities. A right heart catheterization revealed
the following: pulmonary artery pressure, 110/50 mm Hg; right
atrial pressure, 10 mm Hg; pulmonary capillary wedge pressure,
5 mm Hg; cardiac output, 3.1 L/min; and pulmonary vascular
resistance, 19.3 U. Coronary angiography revealed a right dominant system with a 90% ostial left main lesion. The patient was
initially stabilized with IV prostacyclin therapy for 30 days, at
which time her dyspnea had improved but her angina persisted.
She then underwent stenting of the left main coronary artery. At
the end of the procedure, an intravascular ultrasound revealed a
10% residual stenosis and no evidence of atherosclerotic coronary
artery disease. One month following the procedure, the patient
was considerably improved and completely free of angina with all
activity. On echocardiography, her left ventricular ejection fraction returned to normal without any evidence of the previous
underlying wall motion abnormality (Fig 1).
Case 1
A 71-year-old woman was referred for management. She had a
history of PPH, which had been diagnosed 12 years prior and had
been treated conservatively. Although her major symptom had
been dyspnea with effort, which had slowly progressed, she
recently had presented with a syncopal episode and frequent
chest discomfort that radiated down her right arm. The description of the chest discomfort was characteristic of angina, coming
on more frequently and responsive to sublingual nitroglycerin. A
Case 2
A 53-year-old woman was referred after experiencing dyspnea
for 2 years. The patient was evaluated at an outside hospital with
an exercise test that documented her symptoms and a cardiac
catheterization that revealed pulmonary hypertension and stenosis of the left main coronary artery. A subsequent workup
confirmed PPH. At the time of referral, she denied effort angina,
but her exercise tolerance was markedly limited (New York Heart
Figure 1. The effects of coronary artery stenting on left ventricular function. Left ventricular function
and wall motion are illustrated echocardiographically in one patient before (Pre) and after (Post)
stenting of the left main coronary artery. Prior to the stenting, the left ventricular ejection fraction was
reduced, and the left ventricular apex was dyskinetic. Postprocedure left ventricular function and wall
motion completely normalized. ED ⫽ end-diastole; ES ⫽ end-systole.
CHEST / 120 / 4 / OCTOBER, 2001
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Association functional class IV). An echocardiogram revealed
marked right ventricular enlargement and severe pulmonary
hypertension with normal left ventricular size and function. A
cardiac catheterization revealed the following: pulmonary artery
pressure, 78/38 mm Hg; right atrial pressure, 21 mm Hg;
pulmonary capillary wedge pressure, 10 mm Hg; and cardiac
output, 4.8 L/min. Pulmonary vascular resistance calculated to
7.7 U. An attempt was made to evaluate the pulmonary vasodilator reserve with IV adenosine, but the patient developed severe
resting angina with the initiation of the infusion (50 ng/kg/min),
and it had to be terminated immediately. Coronary angiography
then was performed and revealed a severe stenosis of the ostial
left main coronary artery (85%) with no other coronary lesions
noted. The patient was started on IV prostacyclin therapy and was
stabilized for 30 days. She remained sedentary but denied any
angina. She then underwent stenting of the left main coronary
artery. After the stent was placed, intravascular ultrasound failed
to reveal any atherosclerotic lesions or residual left main stenosis
(Fig 2). In the follow-up 1 month later, the patient reported a
dramatic improvement in symptoms of dyspnea with a marked
increased in her day-to-day activities without any limitations from
angina.
Discussion
Angina is a well-documented common symptom associated with severe pulmonary hypertension. Although the
mechanism has been debated, there have been studies
demonstrating right ventricular ischemia in the absence of
coronary artery disease. The basis appears to be, in part, a
reduction in the coronary driving pressure of the right
ventricle.5 There are no studies implicating left ventricular
ischemia in these patients, and because they are usually
young, atherosclerotic coronary artery disease is typically
not considered to be a likely cause of effort angina. For
this reason, coronary angiography is not routinely performed in the evaluation of these patients.
Compression of the left main coronary artery in patients
with PPH and pulmonary hypertension from congenital
heart disease has infrequently been described previously.6 – 8 The left main coronary artery has a characteristic
angiographic appearance, with severe narrowing at the ostium and then gradual enlargement to the distal left main
artery with normal appearance of the remainder of the
coronary circulation. Some have suggested that noninvasive
studies, such as cardiac MRI, may also be helpful.8
Both patients we described had angina. We chose not to
perform stress testing since cardiac catheterization was
necessary in each case. One patient had resting left
ventricular dysfunction with regional wall motion abnormalities. Left ventricular dysfunction has been recently
described to occur in patients up to 20% of patients with
severe pulmonary hypertension, the mechanism of which
has been debated.4 Although many investigators point to
an abnormal interventricular septum and ventricular interdependence, we demonstrated reversible left ventricular dysfunction from left ventricular ischemia in one of our
patients. In addition to affecting left ventricular performance, severe left ventricular ischemia can provoke arrhythmias and sudden cardiac death, both of which commonly occur in patients with severe pulmonary
hypertension.9
This is the first report of successful stenting of the left
main coronary artery due to compression from the pulmonary artery in patients with pulmonary hypertension. We
chose to initially stabilize both patients with IV prostacyclin, not knowing whether or not their conditions would
become hemodynamically unstable during the stenting
procedure. Fortunately, both patients did extremely well
and did not require any additional medical support.
However, stenting of an unprotected left main coronary
artery has been associated with significant morbidity10,11
and, in these high-risk patients, would be best performed
in a highly experienced center.12 It is unknown whether IV
prostacyclin therapy could have contributed to the success
Figure 2. Left main coronary artery compression in a patient with PPH. The appearance of the left
main coronary artery prior to stenting (Pre) is shown. There is a tight ostial stenosis with gradual
enlargement to the distal left main artery with a normal appearance of the remaining coronary artery
tree. Following stenting (Post), the left coronary artery looks entirely normal.
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Selected Reports
of the procedure or to the relief of left ventricular
ischemia in the long term. However, we have seen prostacyclin aggravate chest pain in patients with PPH and be
associated with worsening left ventricular function, the
basis of which has never been explored.
The management of patients with pulmonary hypertension is difficult and their prognosis is guarded.9 The
reported common occurrences of angina (41%),1 left
ventricular dysfunction (20%),4 and sudden death (26%)9
raise the possibility that compression of the left main
coronary artery may occur frequently and is overlooked.
With interventional techniques to allow one to correct this
problem without cardiac surgery, clinicians need to look
carefully for its presence. Coronary artery compression is
rarely considered in these patients, but its detection and
treatment may be lifesaving.
Conclusion
Left main coronary artery compression is a treatable
cause of angina and LV ischemia in patients with PPH. We
recommend that coronary angiography be performed on
patients with severe pulmonary hypertension who present
with effort angina or left ventricular dysfunction. As
pulmonary hypertension progresses and is associated with
a reduction in systemic BP, the left ventricular ischemia
would predictably worsen over time and likely would be
refractory to all medical treatments. In addition, patients
who would be listed for lung transplantation might be
denied if their left ventricular dysfunction develops or
progresses. Given the difficulty in treating patients with
severe pulmonary hypertension successfully, one needs to
be aggressive in detecting and reversing any medical
problem that contributes to worsening cardiac function.
Stenting a compressed left main coronary artery can be
done safely in experienced hands.
References
1 Rich S, Dantzker DR, Ayres S, et al. Primary pulmonary
hypertension: a national prospective study. Ann Intern Med
1987; 107:216 –233
2 Rich S. Primary pulmonary hypertension. Prog Cardiovasc
Dis 1988; 31:205–238
3 Phoon CK, Silverman NH. Conditions with right ventricular
pressure and volume overload, and a small left ventricle:
“hypoplastic” left ventricle or simply a squashed ventricle?
J Am Coll Cardiol 1997; 30:1547–1553
4 Vizza CD, Lynch JP, Ochoa LL, et al. Right and left
ventricular dysfunction in patients with severe pulmonary
disease. Chest 1998; 113:576 –583
5 Vlhakes G, Turley K, Hoffman J. The pathophysiology of
failure in acute right ventricular hypertension: hemodynamic
and biochemical correlation. Circulation 1981; 63:87–95
6 Fujiwara K, Naito Y, Higashine S, et al. Left main coronary
trunk compression by dilated main pulmonary artery in atrial
septal defect. Thorac Cardiovasc Surg 1992; 104:449 – 452
7 Patrat J-F, Jondeau G, Dubourg O, et al. Left main coronaryartery compression during primary pulmonary hypertension.
Chest 1997; 112:842– 843
8 Kawut SM, Silvestry FE, Ferrari VA, et al. Extrinsic compression of the left main coronary artery by the pulmonary
artery in patients with long-standing pulmonary hypertension.
J Am Coll Cardiol 1999; 83:984 –986
9 D’Alonzo G, Barst R, Ayres S, et al. Survival in patients with
primary pulmonary hypertension: results from a national
prospective registry. Ann Intern Med 1991; 115:343–349
10 Park SJ, Park SW, Hong MK, et al. Stenting of unprotected
left main coronary artery stenoses: immediate and late outcomes. J Am Coll Cardiol 1998; 31:37– 42
11 Kosuga K, Tamai H, Ueda K, et al. Initial and long-term
results of angioplasty in unprotected left main coronary
artery. Am J Cardiol 1999; 83:32–37
12 Silvestri M, Barragan P, Sainsous J, et al. Unprotected left
main coronary artery stenting: immediate and medium-term
outcomes of 140 elective procedures. J Am Coll Cardiol 2000;
35:1543–1550
Multiple Coronary Artery-Left
Ventricular Fistulas Associated
With Hereditary Hemorrhagic
Telangiectasia*
Mina A. Jacob, MD; Sanjeev B. Goyal, MD;
Luigi Pacifico, MD; and David H. Spodick, MD, DSc, FCCP
Coronary artery-left ventricular (LV) fistulas are
extremely rare and can cause myocardial ischemia
from coronary steal. We describe an elderly woman
who presented with unstable angina from multiple
and extensive coronary artery-LV fistulas. She also
had clinical features suggestive of hereditary hemorrhagic telangiectasia (HHT). Association of coronary
artery-LV fistulas with HHT has not been reported
and can pose a management dilemma in view of the
risks of extensive cardiopulmonary surgery and potential complications of myocardial ischemia, stroke,
and brain abscess.
(CHEST 2001; 120:1415–1417)
Key words: adult; coronary artery fistula; coronary steal; hereditary hemorrhagic telangiectasia; Osler-Rendu-Weber syndrome;
pulmonary arteriovenous fistula
Abbreviations: CAD ⫽ coronary artery disease; CAF ⫽ coronary artery fistula; HHT ⫽ hereditary hemorrhagic telangiectasia; LV ⫽ left ventricular
oronary artery fistula (CAFs) are rare and are found in
C approximately
0.1% of patients undergoing cardiac
catheterization.1 CAF involving all three major cardiac
vessels and emptying into the left ventricle (arteriosystemic fistulas) are extremely uncommon. They are usually
asymptomatic but can cause myocardial ischemia due to
coronary steal mechanism, congestive heart failure, infec*From the Department of Cardiology, University of Massachusetts-Saint Vincent Hospital, Worcester, MA.
Manuscript received November 14, 2000; revision accepted
March 7, 2001.
Correspondence to: Sanjeev B. Goyal, MD, Department of Cardiology, University of Massachusetts-Saint Vincent Hospital, 20
Worcester Center Blvd, Worcester, MA 01608; e-mail:
[email protected]
CHEST / 120 / 4 / OCTOBER, 2001
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