Ann Thorac Surg 2004;77:1823-1825
© 2004 The Society of Thoracic Surgeons
Case report
Acute respiratory insufficiency and giant coronary artery aneurysm with fistula
Carmelo Mignosa, MD, FETCSa*,
Salvatore Agati, MDa,
Innocenzo Bianca, MDa,
Dario Salvo, MDa,
Giuseppe Pittellaa,
Giuseppe Ciccarello, FEBCPb
a Department of Cardiologia Medica e Chirurgica dell'Età Evolutiva, Azienda USL 5–Ospedale San Vincenzo, Taormina (Messina), Italy
b Perfusion Service, Edwards Lifesciences, Milan, Italy
Accepted for publication June 6, 2003.
* Address reprint requests to Dr Mignosa, Unità Operativa di Cardiochirurgia, Presidio Ospedaliero San Vincenzo, Contrada Sirina, 98039 Taormina (Messina), Italy
e-mail: carmignosa{at}tiscali.it
 |
Abstract
|
|---|
Several causes of acute respiratory insufficiency have been reported in the literature. We describe a case in which it was caused by a rare combination of a giant right coronary artery aneurysm with fistula that occurred in a 56-year-old woman with concomitant congenital hypothyroidism. Diagnostic tools, differential diagnosis, surgical techniques, and follow-up at 1 year are discussed.
|
Introduction
|
|---|
Coronary artery aneurysms are rare, occurring at a reported rate of between 0.3% and 4.9%. Even more rare is the combination of a coronary artery aneurysm with a fistula formation (0.2%) [1]. We describe the case of a 56-year-old woman in whom such a combination had a clinical presentation characterized by acute respiratory insufficiency that required emergent hospital admission.
A 56-year-old woman with congenital hypothyroidism required emergent admission to her local hospital because of acute respiratory insufficiency after several weeks of irrepressible cough and progressive cyanosis. A chest roentgenogram showed cardiomegaly and pulmonary plethora. A computed tomographic scan revealed a posterior intrapericardial mass compressing the tracheal bifurcation. For further evaluation, the patient was transferred to our Department of Cardiac Surgery at the San Vincenzo Hospital in Taormina. Echocardiography showed a situs solitus and normal atrioventricular and ventriculoarterial connections. The right coronary ostium was abnormally dilated. Both atrial chambers were enlarged with a dilated right ventricle.
The patient underwent cardiac catheterization to define the diagnosis. The right coronary artery was abnormally dilated with a worm-like appearance. It had a fistulous communication into the coronary sinus. A left-to-right shunt at the atrial level was present (QP/QS = 3.9), and pulmonary artery pressure was 62/25 (mean 35 mm Hg) with low pulmonary resistance (2.5 Wood units). Bronchoscopy confirmed the extrinsic compression of the trachea at its bifurcation.
During the surgical procedure, the pericardium was opened and it was possible to visualize a worm-like appearance of the aneurysmal right coronary artery (Fig 1).
The diaphragmatic area of the right ventricle was inspected and an enormous, tennis ball-sized dilatation of the coronary sinus was detected (Fig 2).
After cardiopulmonary bypass was instituted with double venous cannulation, the aorta was cross-clamped. It was not possible from a right atriotomy to find the fistulous communication into the coronary sinus. The ostium was not dilated. The right coronary artery was then longitudinally opened from its origin and followed for its entire course. Distally, just before its bifurcation, a fistula into the coronary sinus was found. It was oversewn with running 4-0 Prolene (Ethicon, Somerville, NJ) suture. The dilated coronary sinus was partially plicate. Finally, a segment of reversed autologous saphenous vein was anastomosed end-to-end to the distal right coronary artery at the site of its bifurcation. The proximal anastomosis on the ascending aorta was performed in a classic end-to-side fashion. The right coronary ostium was closed with a glutaraldehyde-treated autologous pericardial patch. The procedure was then completed in the usual fashion.
View larger version (120K):
[in this window]
[in a new window]
|
Fig 1. Giant right coronary artery with a worm-like appearance. (RCAAn = right coronary artery aneurysm; Ao = aorta.)
|
|
View larger version (95K):
[in this window]
[in a new window]
|
Fig 2. Diaphragmatic view of the heart showing the aneurysmal coronary sinus. (CS = coronary sinus; RA = right atrium; Diaph = diaphragm.)
|
|
The patient made an uneventful recovery and was discharged home on postoperative day 6. At 1-year follow-up she remains symptom free. Echocardiography shows no intracardiac shunts and almost normal right ventricle dimensions with normal electrocardiographic findings.
|
Comment
|
|---|
Coronary artery aneurysm in combination with fistula is an extremely rare entity, often asymptomatic, and discovered as an incidental finding during coronary angiography. Acquired coronary artery aneurysms occur with polyarteritis nodosa, Kawasaki disease, arteriosclerosis, postpercutaneous transluminal coronary angioplasty, and endocarditis [2]. Cardiac anomalies represent the most frequent malformations associated with congenital hypothyroidism, but it is extremely rare in combination with the cardiac anomaly we describe.
The frequency of additional congenital malformations was analyzed in a recent population-based study from the Italian Registry for Congenital Hypothyroidism [3]. Cardiac anomalies, with a prevalence of 5.5% (76 of 1372), were found to be the most frequently associated congenital malformation observed in congenital hypothyroidism. Mutation in developmental control genes during early stages of thyroid formation is associated with some cardiac congenital malformation [3, 4]. Ostium secundum atrial septal defect, tetralogy of Fallot, and pulmonary stenosis were the most frequent diseases [3].
The peculiarity of the case we report is indeed its clinical presentation. As described previously, coronary aneurysms are usually incidental findings during coronary angiography, and symptoms related to a coronary fistula are secondary to the entity of the shunt. We, however, encountered a space-occupying mass—the enormously dilated coronary sinus—that was responsible for tracheal compression and the patient's symptoms of acute respiratory insufficiency. This clinical onset and the presence of the giant coronary artery aneurysm required urgent surgical treatment because of the high risk of complications such as endocarditis and aneurysm rupture or tracheal fistulization. A resection with end-to-end anastomosis was not possible because of the fusiform aneurysm of the coronary artery [5], and marsupialization was not possible because of the fistula association [4]. The case we report adds a clinical entity that must be considered in the differential diagnosis of patients with respiratory insufficiency due to tracheal compression.
|
References
|
|---|
- In: Hurst J.W., Schlant R.C., Rackley C.E., Sonnenblick E.H., Wenger N.K., eds. The heart: arteries and veins, 7th ed. New York, NY: McGraw-Hill, Inc, 1990:1233-1234.
- Mawatari T., Koshino T., Morishita K., et al. Successful surgical treatment of giant coronary artery aneurysm with fistula. Ann Thorac Surg 2000;70:1394-1397.[Abstract/Free Full Text]
- Olivieri A., Stazi M.A., Mastroiacovo P., et al. The Study Group of Congenital Hypothyroidism. A population-based study on the frequency of additional congenital malformations in infants with congenital hypothyroidism: data from the Italian Registry for Congenital Hypothyroidism (1991–1998). J Clin Endocrinol Metab 2002;87:557-562.[Abstract/Free Full Text]
- Firstenberg M.S., Azoury F., Lytle B.W., et al. Interposition vein graft for giant coronary aneurysm repair. Ann Thorac Surg 2000;70:1397-1398.[Abstract/Free Full Text]
- Channon K.M., Wadsworth S., Bashir Y.D.M. Giant coronary artery aneurysm presenting as a mediastinal mass. Circulation 1998;82:1307-1308.
Top
Abstract
Introduction
