Download Multiple Coronary Artery-Left Ventricular Fistulas Associated With

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]
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tive endocarditis, and rupture or thrombosis of the fistula.2–3 We present an elderly woman admitted to the
hospital with unstable angina and subsequently found to
have extensive coronary artery-left ventricular (LV) fistulas, pulmonary arteriovenous shunting, and mucocutaneous telangiectasia suggesting hereditary hemorrhagic telangiectasia (HHT; Osler-Rendu-Weber syndrome). To
our knowledge, CAFs associated with HHT have not been
reported.
Case Report
A 72-year-old woman with chronic atrial fibrillation, hypertensive heart disease, and hypothyroidism presented with recurrent
episodes of classical angina associated with palpitations. Family
history was significant for premature coronary artery disease
(CAD) and negative for pulmonary disease, cirrhosis, or bleeding
diathesis. Her medications included atenolol, digoxin, warfarin,
furosemide, conjugated estrogen, and L-thyroxine. A physical
examination revealed perioral and palatal telangiectasia. Cardiovascular examination findings were normal. ECG showed atrial
fibrillation and LV hypertrophy. A chest radiograph revealed
cardiomegaly. Cardiac enzyme levels and routine laboratory
index findings were normal. In view of her high pretest likelihood
of CAD, she underwent cardiac catheterization, which revealed a
dominant right coronary artery, dilated tortuous coronary arteries, no significant CAD, and extensive shunting of blood between
all major epicardial coronary arteries and the left ventricle. The
contrast medium streamed into the left ventricle via a maze of
fine vessels from the diagonal branches of the left anterior
descending artery (Fig 1), midportion of the circumflex artery,
and acute marginal branches of the right coronary artery. Left
ventriculography showed moderate mitral regurgitation and preserved global and regional LV function. Right-heart catheterization showed moderate pulmonary hypertension (pulmonary artery pressure, 50/23 mm Hg), elevated right atrial pressure (19
mm Hg), and elevated pulmonary artery wedge pressure (26
mm Hg). Systemic arterial oxygen saturation was noted to be 89%
on room air. In view of the hypoxemia, a shunt study was
performed that demonstrated an anatomic right-to-left shunt of
8% and a venous admixture of 12%, which worsened in the
standing position. Two-dimensional echocardiography showed
concentric LV hypertrophy, moderate tricuspid regurgitation,
and moderate pulmonary hypertension. Injection of agitated
saline solution into a peripheral vein showed a delayed appearance of air bubbles in the left atrium with the Valsalva maneuver.
The patient’s symptoms improved with titration of atenolol;
considering her age and the extensiveness of the fistulas, she was
discharged receiving medical therapy.
Discussion
This case involves an elderly woman with several risk
factors for CAD, who was hospitalized for unstable angina.
Cardiac catheterization revealed normal coronary arteries
and multiple CAFs involving all three major coronary
arteries communicating with the left ventricle. The patient’s angina was most probably the result of coronary
steal due to diversion of oxygen-rich blood into the LV
cavity via the low-resistance fistulous channels bypassing
the myocardium.2 The delayed appearance of bubbles on
the left side of the heart on contrast echocardiography was
suggestive of an intrapulmonary arteriovenous shunt.4 The
worsening of the hypoxemia on standing was consistent
with the presence of a shunt at the base of the lungs, which
is usually the location of arteriovenous malformations in
patients with HHT.5 Standing causes preferential blood
flow at the bases due to gravity and an arteriovenous
communication in this location would worsen arterial
hypoxemia. The additional presence of mucocutaneous
telangiectasia was diagnostic of HHT in spite of absence of
other features.5 The patient also had moderate passive
pulmonary hypertension and an elevated pulmonary capillary wedge pressure. This was probably due to LV
diastolic dysfunction from concentric LV hypertrophy and
decreased LV compliance. Poor LV diastolic compliance
was due to engorgement of the LV wall from increased
diastolic blood flow via the multiple CAFs. This was also
the probable mechanism for the diastolic equalization of
right-heart pressures. The marked dilatation and tortuosity
of the coronary arteries seen on angiography (Fig 1) in our
case is similar to the changes seen in the arterioles of the
dermis in patients with HHT.6 Since the basic vascular
pathology in HHT is an arteriovenous malformation,6 we
believe that the CAFs were in essence coronary arteriolethebesian vein-LV cavity communications that have been
described in the literature.7 Symptomatic CAFs can be
closed with coil embolization if their size and number
permit transcutaneous catheterization.8 Due to the extensive nature and small size of the CAF, our patient was
managed medically.
Conclusion
Figure 1. Left anterior oblique view of a coronary angiogram
demonstrating multiple and extensive coronary artery-LV fistulas
(arrowheads) from the terminal branches of a dilated and tortuous left anterior descending artery.
This case illustrates a hitherto unknown combination of
pulmonary arteriovenous shunt and coronary artery-LV
fistulas in an elderly woman with mucocutaneous telangiectasia suggesting HHT. Cardiac vascular malformations
associated with HHT have not been described before and
pose a management challenge in view of the potential
complications of myocardial ischemia, neurologic compli-
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Selected Reports
cations of brain abscess and stroke due to intrapulmonary
shunting, and the risks of extensive cardiothoracic surgery
in an elderly woman.
References
1 Yamanaka O, Hobbs RE. Coronary artery anomalies in
126,595 patients undergoing coronary arteriography. Cathet
Cardiovasc Diagn 1990; 21:28 – 40
2 Stierle U, Giannitsis E, Sheikhzadeh A, et al. Myocardial
ischemia in generalized coronary artery-left ventricular microfistulae. Int J Cardiol 1998; 63:47–52
3 Perloff JK. Congenital coronary arterial fistula. In: Perloff JK,
ed. The clinical recognition of congenital heart disease.
Philadelphia, PA: W.B. Saunders Company, 1978; 576 –589
4 Oh JK, Seward JB, Tajik AJ. Contrast echocardiography. In:
The echo manual. Philadelphia, PA: Lippincott Williams and
Wilkins, 1999; 245–249
5 Guttmacher AE, Marchuk DA, White RI Jr. Current concepts: hereditary hemorrhagic telangiectasia. N Engl J Med
1995; 333:918 –924
6 Braverman IM, Keh A, Jacobson BS. Ultrastructure and
three-dimensional organization of the telangiectases of hereditary hemorrhagic telangiectasia. J Invest Dermatol 1990;
95:422– 427
7 Coussement P, De Geest H. Multiple coronary artery-left
ventricular communications: an unusual prominent Thebesian system; a report of four cases and review of the literature.
Acta Cardiol 1994; 49:165–173
8 Dorros G, Thota V, Ramireddy K, et al. Catheter-based
techniques for closure of coronary fistulae. Cathet Cardiovasc
Interv 1999; 46:143–150
Rescue Percutaneous
Coronary Intervention
Immediately Following
Coronary Artery Bypass
Grafting*
Robert N. Piana, MD; Mark R. Adams, MBBS, PhD;
James L. Orford, MBChB; Jeffrey J. Popma, MD;
David H. Adams, MD; and Samuel Z. Goldhaber, MD, FCCP
Perioperative graft failure after coronary artery bypass graft (CABG) can result in acute myocardial
infarction with dire clinical consequences. We report
a case of rescue percutaneous coronary intervention
immediately after unsuccessful CABG. This approach salvaged the patient from cardiogenic shock
and should be recognized as a viable alternative to
immediate reoperation for certain patients.
(CHEST 2001; 120:1417–1420)
Key words: angioplasty; catheterization; coronary artery bypass
graft; stent
Abbreviations: CABG ⫽ coronary artery bypass graft;
LIMA ⫽ left internal mammary artery; PCI ⫽ percutaneous coronary intervention
mmediate cardiac surgical “backup” for coronary angioI plasty
has historically been considered mandatory because procedural complications have necessitated emergency “rescue” coronary artery bypass graft (CABG)
surgery in as many as 3% of cases. Here, we describe the
reverse circumstance of a patient who suffered an acute
anterior myocardial infarction immediately after CABG.
In this case, rescue percutaneous coronary intervention
(PCI) was required for a complication of CABG.
Case Report
A 78-year-old woman had several episodes of chest pain at rest
and after eating. Results of a modified Bruce protocol exercise
treadmill test proved markedly positive, and coronary angiography demonstrated a 70% ostial left main coronary artery stenosis
and three-vessel coronary artery disease. She was therefore
referred for urgent CABG. Preoperative echocardiography
showed normal left ventricular function. She received a left
internal mammary artery (LIMA) graft to the left anterior
descending and reverse saphenous vein grafts to the first marginal and to the posterior descending arteries. Intraoperatively,
the grafts had good flow and runoff. Concomitant left carotid
endarterectomy was performed because of an incidentally discovered 85% left internal carotid artery stenosis. Cardiopulmonary bypass time was 54 min, and aortic cross-clamp time was 43
min. Postoperatively, 250 mg of protamine sulfate was administered and the accelerated clotting time was 121 s on arrival in
the ICU.
At the time of transfer to the ICU, her systemic arterial
pressure fell from 156/63 to 91/48 mm Hg. Initial ECG showed
extensive ST-segment elevation consistent with acute anterior
and lateral infarction. Thirty minutes later, a follow-up ECG
showed loss of R waves and new Q waves in the anterior
precordial leads (Fig 1), indicating evolution of myocardial
infarction.
Emergent coronary angiography showed a 70% stenosis in the
LIMA graft at its anastomosis to the left anterior descending
artery. Flow in the LIMA graft was delayed and pulsatile, with no
effective filling of the left anterior descending artery. There was
also a 90% stenosis in the native left anterior descending coronary
artery directly underlying the LIMA graft anastomosis. The two
vein grafts were widely patent. Heparin, 3,000 IU, was administered, and an accelerated clotting time of 292 s was documented
prior to percutaneous coronary intervention. The patient also
received aspirin, 325 mg, and clopidogrel, 150 mg, by nasogastric
tube. Rescue balloon angioplasty of the LIMA graft lesion
resulted in a 30% residual stenosis with a small linear dissection
and improved antegrade flow, but without resolution of the
ST-segment elevation or hypotension. The stenosis in the native
left anterior descending artery was therefore dilated with a
3.0 ⫻ 18-mm Duet stent (Guidant Corporation; Temecula, CA),
delivered through the left main and positioned spanning the
LIMA graft anastomosis. The dissection site in the left internal
*From the Cardiovascular Division (Dr. Piana), Vanderbilt University Medical Center, Nashville, TN; and the Cardiovascular
and Cardiac Surgical Divisions (Drs. M. Adams, Orford, Popma,
D. Adams, and Goldhaber), Brigham and Women’s Hospital,
Harvard Medical School, Boston, MA.
Manuscript received March 7, 2000; revision accepted March 8,
2001.
Correspondence to: Robert N. Piana, MD, Director, Cardiac
Catheterization Laboratories, Vanderbilt University Medical
Center, 2311 Pierce Ave, Nashville, TN 37232-8802; e-mail:
[email protected]
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