Ann Thorac Surg 2003;76:619-621
© 2003 The Society of Thoracic Surgeons
Case report
Uncommon presentation and surgical correction of unroofed coronary sinus syndrome
Federico Brunelli, MD*a,
Andrea Amaducci, MDa,
Zen Mhagna, MDa,
Giovanni Troise, MDa,
Eugenio Quaini, MDa
a Cardiac Surgery Unit, Private No-Profit Hospital Poliambulanza, Brescia, Italy
Accepted for publication December 23, 2002.
* Address reprint requests to Dr Brunelli, Cardiac Surgery Unit, Private No-Profit Hospital Poliambulanza, via Bissolati 57, Brescia 25125, Italy
e-mail: cch-segreteria.poli{at}poliambulanza.it
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Abstract
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A 59-year-old man with signs and symptoms of congestive heart failure, occurring a few months after an infective episode, underwent cardiac investigations revealing severe biventricular dysfunction, persistent left superior vena cava with almost completely unroofed coronary sinus, and critical stenosis of the proximal right coronary artery. Surgical correction of the congenital malformation associated with revascularization of the right coronary allowed a prompt recovery of clinical conditions and ventricular function.
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Introduction
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In 1965, Raghib and colleagues [1] described the developmental complex characterized from termination of the left superior vena cava (LSVC) in the left atrium, atrial septal defect, and absence of coronary sinus. This malformation was recognized in 8 patients of a surgical series reported in 1979 in which the partition between the coronary sinus and the left atrium was totally or almost totally absent [2]. Pathophysiology of the defect consists of bi-directional atrial shunting, increased pulmonary blood flow and decreased systemic oxygen saturation [3]. Cyanosis and the risk of cerebral complication make surgical correction advisable. We present an uncommon case of an adult patient who was hospitalized because of an episode of congestive heart failure, in whom the demonstration of the congenital malformation was totally unexpected.
A 59-year-old man with a history of hypertension, hypercholesterolemia, past smoking, and chronic obstructive pulmonary disease who was treated with bronchodilators, after a 1-month period of mild, intermittent pyrexia accompanied by progressively reduced exercise tolerance, was referred to our cardiology department because of clearly evident congestive heart failure (New York Heart Association functional class IV). Referral diagnosis was postmyocarditis dilated cardiomyopathy. Peripheral edema and orthopnea were cured with aggressive medical treatment (diuretics and vasodilators), and the echocardiogram revealed dilated right (moderate tricuspid valve regurgitation) and left ventricles (transverse diastolic diameter, 69 mm), systolic left ventricular (LV) dysfunction (ejection fraction, 35%), akinesia of the inferoposterior LV wall, persistent LSVC without a precise demonstration of ASD (atrial septal defect). Cardiac catheterization demonstrated systemic oxygen saturation of 91%, pulmonary artery systolic pressure of 51 mm Hg, and a cardiac index of 1.96 L/min/m2. Injection into the LSVC revealed a wide defect in the roof of the coronary sinus with bi-directional shunt (Fig 1).
Coronarography demonstrated a severe stenosis of the right coronary ostium. Ventriculography showed akinesia of the LV inferior wall, LV diastolic volume of 233 mL, systolic volume of 157 mL, and global hypokinesia with an LVEF of 33%. Blood gas analysis revealed an arterial PO2 of 55 mm Hg with room air, with a PCO2 of 35, a hemoglobin level of 17.2 g/L, and a hematocrit value of 53%.
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Fig 1. Right heart catheterization. Selective injection in the left superior vena cava showing contrast filling of the left atrium through a wide defect in the roof of the coronary sinus (arrow) and of the right atrium through a large coronary sinus orifice. (LA = left atrium; LSVC = left superior vena cava; RA = right atrium.)
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During the operation the pericardium was found to be adherent to the epicardium as is usually seen after a recent pericarditis. After the skeletonized right internal mammary artery was harvested, cardiopulmonary bypass was established by cannulation of the ascending aorta and the three caval veins selectively. During aortic cross-clamp time with cardioplegic arrest, the intact atrial septum was opened through the right atriotomy, and the anatomy of the defect was demonstrated; a small remnant of the coronary sinus wall was present just upstream the large orifice in the right atrium, whereas at least three quarters of the theoretical course of the coronary sinus beneath the left atrium was unroofed, and the LSVC was drained at the left upper corner of the left atrium, close to the ostium of the left appendage (Fig 2).
Rerouting of the LSVC into the right atrium was achieved by reroofing the coronary sinus with an autologous glutaraldehyde-treated pericardial patch. The atrial septum was reconstructed, the right atriotomy was closed, and a termino-lateral anastomosis between the right internal mammary artery and the proximal right coronary was completed. The patient was easily weaned from cardiopulmonary bypass, and modified ultrafiltration was performed. The postoperative course was uneventful. The patient was discharged from the hospital on the postoperative day 4 with normal oxygen saturation (arterial PO2, 117 mm Hg), normal sinus rhythm, and improved LV function (ejection fraction, 45%). Six months postoperatively, the patient is in New York Heart Association functional class I, and echocardiographic examination shows normalized right ventricular dimension and contractility with trivial tricuspid regurgitation, reduced LV dilatation, and persistently improved contractility (ejection fraction, 50%).
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Fig 2. Intraoperative photograph. The atrial septum is incised through a right atriotomy. An aspirator is advanced through the orifice of the coronary sinus and appears in the left atrium, just beyond a small, fibrous remnant of the distal roof of the coronary sinus. (AS = atrial septum; MV = mitral valve; TV = tricuspid valve).
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Comment
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Unroofed coronary sinus is a rare congenital anomaly, which is generally well tolerated when it is not associated with other, more complex, congenital defects [4]. The association of a persistent LSVC determines a right-to-left shunt with variable degrees of cyanosis that usually addresses further cardiac investigations in the pediatric age. As in other types of right-to-left shunt, the clinical picture, the natural history, and the life expectancy are dominated by cerebral complications and other problems associated with increasing cyanosis and polycythemia [2].
The singularity of this case lies in the confounding clinical presentation in an adult, referred in poor clinical conditions with two possible causes of cardiomyopathy: (1) infective congestive heart failure after long-lasting intermittent pyrexia or (2) ischemia demonstrated by echocardiography showing diffuse LV hypocontractility with a segmental akinetic area. A previous clinical diagnosis of chronic obstructive pulmonary disease may explain the moderate systemic oxygen desaturation. A transesophageal echocardiography demonstrated the presence of the LSVC with an atrial septal defect and angiographic injection, which revealed the correct anatomy of the defect associated with a single-vessel coronary disease.
Our interpretation of the clinical evolution and presentation picture is as follows: (1) long-standing, prevalent left-to-right shunt and volume overload of the right ventricle; (2) progressively decreased right ventricular function caused by right coronary disease; (3) rising up of right atrial pressure and increasing right-to-left shunt from the LSVC; (4) myocardial hypoxia; and (5) inflammatory response to an infectious episode precipitating congestive heart failure. The indication for correction of the congenital malformation along with myocardial revascularization is aimed to improve the oxygen supply to the myocardium and reduce volume overload. The ease of the postoperative course and the immediate clinical improvement, as well as the increase of the LVEF, confirmed the positive indication.
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References
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- Raghib G., Ruttemberg H.D., Anderson R.C., Amplatz K., Adams P., Jr, Edwards J.E. Termination of left superior vena cava in left atrium, atrial septal defect and absence of coronary sinus. Circulation 1965;31:906-918.[Abstract/Free Full Text]
- Quaegebeur J., Kirklin J.W., Pacifico A.D., Bargeron L.M., Jr Surgical experience with unroofed coronary sinus. Ann Thorac Surg 1979;27:418-425.[Abstract/Free Full Text]
- Lee M.E., Sade R.M. Coronary sinus septal defect. Surgical consideration. J Thorac Cardiovasc Surg 1979;78:563-569.[Abstract]
- Freedom R.M., Culham J.A.G., Rowe R.D. Left atrial to coronary sinus fenestration (partially unroofed coronary sinus). Morphological and angiocardiographic observation. Br Heart J 1981;46:63-68.[Free Full Text]
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Abstract
Introduction
