Case study Herz 2014 DOI 10.1007/s00059-014-4077-2 Received: 3 October 2013 Revised: 19 November 2013 Accepted: 4 February 2014 © Urban & Vogel 2014
Additional material online This article includes two additional videos. You will find this supplemental at dx.doi.org/10.1007/s00059-014-4077-2.
Coronary artery fistulas are rare abnormalities of the coronary arteries with an incidence of 0.08–0.3% [1]. After orthotopic cardiac transplantation the incidence increases to approximately 8%, which is very likely related to repeated endomyocardial biopsies usually taken from the right ventricular portion of the interventricular septum [2]. Fistulas can occur in any of the three major coronary arteries. Most of these fistulas are related to the right coronary artery (RCA) or the left anterior descending artery (LADA); however, the circumflex coronary artery (CX) is also affected infrequently (RCA in approximately 55% of cases, LCA in 35%, and both in 5%) [3]. More than 90% of coronary artery fistulas drain into the low-pressure venous system resulting in a left-to-right shunt. Fistula drainage occurs into the left ventricle [4] in only 3% of cases. Most fistulas present single communications; nevertheless, multiple fistulization has been described. Fistulas are usually of congenital origin but there are also acquired forms. The clinical presentation can be asymptomatic or vary from fatigue to dyspnea, orthopnea, angina pectoris, endocarditis, arrhythmias, stroke, and myocardial ischemia or infarction.
M. Wallner1 · R. Zweiker1 · R. Maier1 · D. Strunk2 · D. von Lewinski1 1 Division of Cardiology, Medical University of Graz, Graz 2 Stem Cell Research, Medical University of Graz, Graz
Massive fistulization into the left ventricle of a transplanted heart Case report In September 2012, a 56-year-old male patient was admitted for emergency hospitalization displaying New York Heart Association (NYHA) class III dyspnea. The patient was already well known because of a long and very challenging medical history. In January 2004 heart transplantation was performed due to endstage failing dilated cardiomyopathy with recurrent ventricular tachycardia. A left ventricular assist device (TCI HeartMate, Thoratec, Pleasanton, Calif.) was implanted as a bridge to transplantation. The orthotopic heart transplantation was complex and entailed several complications. Weaning from the extracorporeal circulation succeeded only with administration of high-dose catecholamine. Due to postoperative bleeding, an operative revision had to be performed twice. During the course the patient developed acute renal failure treated with short-term dialysis, hypoxic liver damage, and necrosis of the feet, but recovered without limitations and was discharged in May 2004 on immunosuppressive therapy consisting of mycophenolate sodium (720 mg twice daily), everolimus (0.75 mg twice daily), and prednisolone (5 mg once daily). Possible rejection was monitored by frequent right ventricular endomyocardial biopsies, which were all within normal limits. In October 2006 the patient underwent coronary angiography, after which graft sclerosis and coronary artery disease were excluded. In May 2009 a retrosternal abscess was opened and sup-
ported with a vacuum-assisted closure (VAC) system. At the time of admission in 2012 the patient was in a reduced general condition and displayed NYHA class III dyspnea. Echocardiography revealed a normal left ventricular systolic function, a moderate to severe tricuspid regurgitation, and pacemaker leads in the right atrium and right ventricle. Subsequently cardiac catheterization was performed with an artery angiogram and left ventriculogram. Multiple coronary artery fistulas were observed arising from all three major coronary arteries, especially from the LADA (Fig. 1a, video 1) and RCA (Fig. 1b, video 2), draining into the left ventricle so that the selective coronary angiogram gave the impression of a left ventriculogram. Furthermore, there was a very high flow velocity within the coronary arteries. The ensuing echocardiography revealed 36% higher blood flow in the aorta compared with the pulmonary artery (Qp:Qs =0.7:1; Fig. 1c). An increased concentration of vascular endothelial growth factor A (VEGF-A; 142 pg/ml, reference: 0–42.6 pg/ml) and a normal level of VEGF receptor (VEGFR;
Additional material online This article includes two additional videos. You will find this supplemental at dx.doi.org/10.1007/s00059-014-4077-2.
Coronary artery fistulas are rare abnormalities of the coronary arteries with an incidence of 0.08–0.3% [1]. After orthotopic cardiac transplantation the incidence increases to approximately 8%, which is very likely related to repeated endomyocardial biopsies usually taken from the right ventricular portion of the interventricular septum [2]. Fistulas can occur in any of the three major coronary arteries. Most of these fistulas are related to the right coronary artery (RCA) or the left anterior descending artery (LADA); however, the circumflex coronary artery (CX) is also affected infrequently (RCA in approximately 55% of cases, LCA in 35%, and both in 5%) [3]. More than 90% of coronary artery fistulas drain into the low-pressure venous system resulting in a left-to-right shunt. Fistula drainage occurs into the left ventricle [4] in only 3% of cases. Most fistulas present single communications; nevertheless, multiple fistulization has been described. Fistulas are usually of congenital origin but there are also acquired forms. The clinical presentation can be asymptomatic or vary from fatigue to dyspnea, orthopnea, angina pectoris, endocarditis, arrhythmias, stroke, and myocardial ischemia or infarction.
M. Wallner1 · R. Zweiker1 · R. Maier1 · D. Strunk2 · D. von Lewinski1 1 Division of Cardiology, Medical University of Graz, Graz 2 Stem Cell Research, Medical University of Graz, Graz
Massive fistulization into the left ventricle of a transplanted heart Case report In September 2012, a 56-year-old male patient was admitted for emergency hospitalization displaying New York Heart Association (NYHA) class III dyspnea. The patient was already well known because of a long and very challenging medical history. In January 2004 heart transplantation was performed due to endstage failing dilated cardiomyopathy with recurrent ventricular tachycardia. A left ventricular assist device (TCI HeartMate, Thoratec, Pleasanton, Calif.) was implanted as a bridge to transplantation. The orthotopic heart transplantation was complex and entailed several complications. Weaning from the extracorporeal circulation succeeded only with administration of high-dose catecholamine. Due to postoperative bleeding, an operative revision had to be performed twice. During the course the patient developed acute renal failure treated with short-term dialysis, hypoxic liver damage, and necrosis of the feet, but recovered without limitations and was discharged in May 2004 on immunosuppressive therapy consisting of mycophenolate sodium (720 mg twice daily), everolimus (0.75 mg twice daily), and prednisolone (5 mg once daily). Possible rejection was monitored by frequent right ventricular endomyocardial biopsies, which were all within normal limits. In October 2006 the patient underwent coronary angiography, after which graft sclerosis and coronary artery disease were excluded. In May 2009 a retrosternal abscess was opened and sup-
ported with a vacuum-assisted closure (VAC) system. At the time of admission in 2012 the patient was in a reduced general condition and displayed NYHA class III dyspnea. Echocardiography revealed a normal left ventricular systolic function, a moderate to severe tricuspid regurgitation, and pacemaker leads in the right atrium and right ventricle. Subsequently cardiac catheterization was performed with an artery angiogram and left ventriculogram. Multiple coronary artery fistulas were observed arising from all three major coronary arteries, especially from the LADA (Fig. 1a, video 1) and RCA (Fig. 1b, video 2), draining into the left ventricle so that the selective coronary angiogram gave the impression of a left ventriculogram. Furthermore, there was a very high flow velocity within the coronary arteries. The ensuing echocardiography revealed 36% higher blood flow in the aorta compared with the pulmonary artery (Qp:Qs =0.7:1; Fig. 1c). An increased concentration of vascular endothelial growth factor A (VEGF-A; 142 pg/ml, reference: 0–42.6 pg/ml) and a normal level of VEGF receptor (VEGFR;
