Incidental congenital coronary artery vascular fistulas in adults: Evaluation with adenosine-13N-ammonia PET-CT.

AIM: To assess the functionality of congenital coronary artery fistulas (CAFs) using adenosine stress 13N-ammonia positron emission tomography computed tomography (PET-CT). METHODS: Congenital CAFs were incidentally detected during coronary angiography (CAG) procedures in 11 adult patients (six males and five females) with a mean age of 64.3 years (range 41-81). Patients were collected from three institutes in the Netherlands. The characteristics of the fistulas (origin, pathway and termination), multiplicity of the origins and pathways of the fistulous vessels were assessed by CAG. Five patients underwent adenosine pharmacologic stress 13N-ammonia PET-CT to assess myocardial perfusion and the functional behavior of the fistula. RESULTS: Eleven patients with 12 CAFs, 10 unilateral and one bilateral, originating from the left anterior descending coronary artery (n = 8), right coronary artery (n = 2) and circumflex (n = 2). All fistulas were of the vascular type, terminating into either the pulmonary artery (n = 11) or coronary sinus (n = 1). The CAG delineated the characteristics of the fistula (origin, pathway and termination). Multiplicity of the origins and pathways of the fistulous vessels were common in most fistulas (8/12, 67% and 9/12, 75%, respectively). Multiplicity was common among the different fistula components (23/36, 64%). Adenosine pharmacologic stress 13N-ammonia PET-CT revealed normal myocardial perfusion and ejection fraction in all but one patient, who showed a reduced ejection fraction. CONCLUSION: PET-CT may be helpful for assessing the functional status of congenital CAFs in selected patients regarding clinical decision-making. Studies with a larger patient series are warranted.

Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases.

AIM: To compare the behavior of pulmonary hypertension (PHT) associated with coronary artery fistulas (CAFs) between the Asian and Caucasian subjects. METHODS: CAFs may be complicated with PHT secondary to left-to-right shunt. Literature review limited to the English language. A total of 211 reviewed patients were collected. Of those, 111 were of Asian and 100 were of Caucasian ethnic origin. The mean age of the Asian and the Caucasian groups of patients were 48.9 (range 19-83) and 49.9 years (range 16-85), respectively. In both groups, right heart catheterization was the most commonly (95%) used method for determining pulmonary artery pressure. RESULTS: From all of the reviewed subjects, PHT was found in 49 patients (23%), of which 15 were Asian and 34 were Caucasian. In 75% of PHT subjects, mild to moderate PHT was reported and 76% of the fistulas had a vascular mode of termination. Treatment was surgical in 61%, followed by percutaneous therapeutic embolization (27%) and finally conservative medical management in 12% of PHT subjects. PHT was associated with a slight female gender predominance. The majority demonstrated mild to moderate PHT. PHT was reported more frequent in the Caucasian compared with the Asian ethnicity group. The majority of fistulas in patients with PHT had a vascular mode of termination. The results of this review are intended to be indicative and require cautious interpretation. CONCLUSION: The likelihood for a CAF patient to develop PHT is presented when possessing the following features, with a Caucasian female having a fistula with a vascular mode of termination.

Characteristics of Congenital Coronary Artery Fistulas Complicated with Infective Endocarditis: Analysis of 25 Reported Cases.

Congenital coronary artery fistulas (CAFs) are infrequent congenital coronary artery anomalies. Complications such as left-to-right shunt, congestive heart failure, myocardial infarction, pericardial effusion, aneurysm formation, rupture, hemopericardium, pulmonary hypertension, infective endocarditis (IE), syncope, stroke, and sudden death may occur with a variable low frequency. To describe the clinical characteristics of patients with CAFs complicated by IE. A search was conducted through PubMed using the terms "CAFs" and "IE." Papers with a full description of the fistula characteristics and detailed data regarding bacterial endocarditis were included for evaluation. In the overall group of reviewed subjects (n = 25, 9 females), the mean patient age was 42.5 years (range: 16 and 87). The right coronary artery (RCA) and left coronary artery (LCA) contributed equally to fistula formation. Terminations into the right heart side occurred in 19 (76%) fistulas. The majority of the fistulas (92%) were unilateral. The cultured microorganism was Streptococcus in 14 (56%) and Staphylococcus in 4 (16%) of the reviewed subjects. Echocardiographic single or multiple valvular regurgitation was found in 8 (32%) of the reviewed subjects. Small and large intracardiac vegetations were detected in 18 patients (72%). Antibiotic therapy was initiated in 20 (80%) subjects and 16 fistulas were treated surgically. During surgery, spontaneous closure of the fistula was observed in one patient. Percutaneous therapeutic embolization (PTE) was successfully performed in two subjects. CAFs complicated by IE may affect all age groups with a slight male preponderance. Unilateral fistulas, either arising from the right or left coronary artery, are predominant, draining mainly into the right heart side. It is emphasized that antibiotic prophylaxis is strongly advised for pediatric and adult patients with congenital CAFs.

Cardiac and non-cardiac causes of T-wave inversion in the precordial leads in adult subjects: A Dutch case series and review of the literature.

AIM: To describe the electrocardiographic (ECG) phenomena characterized by T-wave inversion in the precordial leads in adults and to highlight its differential diagnosis. METHODS: A retrospective chart review of 8 adult patients who were admitted with ECG T-wave inversion in the anterior chest leads with or without prolongation of corrected QT (QTc) interval. They had different clinical conditions. Each patient underwent appropriate clinical assessment including investigation for myocardial involvement. Single and multimodality non-invasive, semi-invasive and invasive diagnostic approach were used to ascertain the diagnosis. The diagnostic assessment included biochemical investigation, cardiac and abdominal ultrasound, cerebral and chest computed tomography, nuclear medicine and coronary angiography. RESULTS: Eight adult subjects (5 females) with a mean age of 66 years (range 51 to 82) are analyzed. The etiology of T-wave inversion in the precordial leads were diverse. On admission, all patients had normal blood pressure and the ECG showed sinus rhythm. Five patients showed marked prolongation of the QTc interval. The longest QTc interval (639 ms) was found in the patient with pheochromocytoma. Giant T-wave inversion (≥ 10 mm) was found in pheochromocytoma followed by electroconvulsive therapy and finally ischemic heart disease. The deepest T-wave was measured in lead V3 (5 ×). In 3 patients presented with mild T-wave inversion (patients 1, 5 and 4 mm), the QTc interval was not prolonged (432, 409 and 424 msec), respectively. CONCLUSION: T-wave inversion associated with or without QTc prolongation requires meticulous history taking, physical examination and tailored diagnostic modalities to reach rapid and correct diagnosis to establish appropriate therapeutic intervention.

Coronary artery disease in congenital single coronary artery in adults: A Dutch case series.

AIM: To assess the current diagnostic and therapeutic management and the clinical implications of congenital single coronary artery (SCA) in adults. METHODS: We identified 15 patients with a SCA detected from four Dutch angiography centers in the period between 2010 and 2013. Symptomatic patients who underwent routine diagnostic coronary angiography (CAG) for suspected coronary artery disease and who incidentally were found to have isolated SCA were analyzed. RESULTS: Fifteen (7 females) with a mean age of 58.5 ± 13.78 years (range 43-86) had a SCA. Conventional CAG demonstrated congenital isolated SCA originating as a single ostium from the right sinus of Valsalva in 6 patients and originating from the left in 9 patients. Minimal to moderate coronary atherosclerotic changes were found in 4, and severe stenotic lesions in another 4 patients. Seven patients were free of coronary atherosclerosis. Runs of non-sustained ventricular tachycardia were documented in 2 patients, one of whom demonstrated transmural ischemic changes on presentation. Myocardial perfusion scintigraphic evidence of transmural myocardial ischemia was found in 1 patient due to kinking and squeezing of the SCA with an interarterial course between the aorta and pulmonary artery. Multi-slice computed tomography (MSCT) was helpful to delineate the course of the anomalous artery relative to the aorta and pulmonary artery. Percutaneous coronary intervention was successfully performed in 3 patients. Eight patients were managed medically. Arterial bypass graft was performed in 4 patients with the squeezed SCA. CONCLUSION: SCA may be associated with transient transmural myocardial ischemia and aborted sudden death in the absence of coronary atherosclerosis. The availability and sophistication of MSCT facilitates the delineation of the course of a SCA. We present a Dutch case series and review of the literature.

Coronary-cameral fistulas in adults (first of two parts).

This is a case series and review of the literature adding 11 new cases. Coronary-cameral fistulas (CCFs) are infrequent anomalies which are in general co-incidentally found during diagnostic coronary angiography (CAG). To delineate the characteristics of congenital and acquired CCFs in adults, we performed a PubMed search for papers dealing with congenital or acquired CCFs in adults. Publications on coronary-vascular fistulas or paediatric subjects were not included. From the world literature, a total of 243 adult patients were identified who had congenital (65%) or acquired (35%) CCFs. In this review, which is part one of a two-part series on CCFs, we describe and discuss the congenital fistulas, give an overview on the published literature and report details of our own series of 11 patients with MMFs and solitary macro CCFs. Of the congenital group, 85% were small or large solitary macro CCFs (cut-off 1.5 mm) and 15% were coronary artery-ventricular multiple micro-fistulas (MMFs). Apical hypertrophic cardiomyopathy was reported in some of the reviewed subjects with MMFs (3/24 = 13%) but not was seen in our own series. Conservative medical management was generally the treatment of choice in congenital MMFs; prophylactic implantable cardioverter defibrillators (ICD) were implanted in 2/24 (8%) of subjects, especially when extensive micro-fistulisations were involved. None of the patients of our own series required an ICD, as the MMFs were of limited size. Congenital or acquired CCFs in adults are infrequent anomalies having a wide spectrum of clinical presentation may varies from asymptomatic to severely devastating states requiring different treatment modalities.

Exercise-induced left bundle branch block: an infrequent phenomenon: Report of two cases.

Exercise-induced left bundle branch block (EI-LBBB) is infrequent phenomenon. We present two patients with angina pectoris who developed EI-LBBB during exercise tolerance test. The first patient with typical angina pectoris had significant obstructive coronary artery disease (CAD) requiring percutaneous coronary intervention of multiple lesions including placement of drug eluting stents. The second patient had atypical chest pain without signs of CAD at all. EI-LBBB occurred at a heart rate of 80 bpm and 141 bpm in the first and second patient, respectively. EI-LBBB remained visible through the test till the recovery period in the first patient at a heart rate of 83 bpm and disappeared at 96 bpm in the second patient. Both patients with this infrequent phenomenon are discussed and the literature is reviewed.

Coronary-cameral fistulas in adults: Acquired types (second of two parts).

Acquired coronary artery fistulas (CCFs) are infrequently detected during conventional coronary angiography. To delineate the characteristics of congenital (first part) and acquired (second part) CCFs in adults, a PubMed search was conducted for papers dealing with congenital or acquired CCFs. None of the publications describing patients with coronary-vascular fistulas were included. Papers dealing with pediatric subjects were excluded. From the world literature, a total of 243 adult patients were selected who had congenital (n = 159/243, 65%) and acquired (n = 84/243, 35%) CCFs. Among the acquired types (n = 72, 85.7%) were traumatic (iatrogenic (n = 65/72, 90%), accidental (n = 7/72, 10%) and (n = 12, 14.3%) spontaneously developing in relation to severe coronary atherosclerosis or myocardial infarction. A high incidence of spontaneous resolution of iatrogenic CCFs resulting from endomyocardial biopsy or following post-septal myectomy was reported. Spontaneous CCFs associated with myocardial ischemia or infarction resolved completely in 8% of the subjects. Early surgical intervention was the treatment of choice in acquired traumatic accidental CCFs. The congenital types are addressed in a previous issue of this journal (first part). In this review (second of two parts, part II), we describe the acquired coronary-cameral fistulas.

Current characteristics of congenital coronary artery fistulas in adults: A decade of global experience.

AIM: To describe the characteristics of coronary artery fistulas (CAFs) in adults, including donor vessels and whether termination was cameral or vascular. METHODS: A PubMed search was performed for articles between 2000 and 2010 to describe the current characteristics of congenital CAFs in adults. A group of 304 adults was collected. Clinical data, presentations, diagnostic modalities, angiographic fistula findings and treatment strategies were gathered and analyzed. With regard to CAF origin, the subjects were tabulated into unilateral, bilateral or multilateral fistulas and compared. The group was stratified into two major subsets according to the mode of termination; coronary-cameral fistulas (CCFs) and coronary-vascular fistulas (CVFs). A comparison was made between the two subsets. Fistula-related major complications [aneurysm formation, infective endocarditis (IE), myocardial infarction (MI), rupture, pericardial effusion (PE) and tamponade] were described. Coronary artery-ventricular multiple micro-fistulas and acquired CAFs were excluded as well as anomalous origin of the coronary arteries from the pulmonary artery (PA). RESULTS: A total of 304 adult subjects (47% male) with congenital CAFs were included. The mean age was 51.4 years (range, 18-86 years), with 20% older than 65 years of age. Dyspnea (31%), chest pain (23%) and angina pectoris (21%) were the prevalent clinical presentations. Continuous cardiac murmur was heard in 82% of the subjects. Of the applied diagnostic modalities, chest X-ray showed an abnormal shadow in 4% of the subjects. The cornerstone in establishing the diagnosis was echocardiography (68%), and conventional contrast coronary angiography (97%). However, multi-slice detector computed tomography was performed in 16%. The unilateral fistula originated from the left in 69% and from the right coronary artery in 31% of the subjects. Most patients (80%) had unilateral fistulas, 18% presented with bilateral fistulas and 2% with multilateral fistulas. Termination into the PA was reported in unilateral (44%), bilateral (73%) and multilateral (75%) fistulas. Fistulas with multiple origins (bilateral and multilateral) terminated more frequently into the PA (29%) than into other sites (10.6%) (P = 0.000). Aneurysmal formation was found in 14% of all subjects. Spontaneous rupture, PE and tamponade were reported in 2% of all subjects. In CCFs, the mean age was 46.2 years whereas in CVFs mean age was 55.6 years (P = 0.003). IE (4%) was exclusively associated with CCFs, while MI (2%) was only found in subjects with CVFs. Surgical ligation was frequently chosen for unilateral (57%), bilateral (51%) and multilateral fistulas (66%), but percutaneous therapeutic embolization (PTE) was increasingly reported (23%, 17% and 17%, respectively). CONCLUSION: Congenital CAFs are currently detected in elderly patients. Bilateral fistulas are more frequently reported and PTE is more frequently applied as a therapeutic strategy in adults.

Congenital solitary coronary artery fistulas characterized by their drainage sites.

Last centuries have witnessed tremendous sophistication and progress in the detection, diagnosis and treatment of coronary artery fistulas (CAFs). In many countries, CAFs were reported to be visualized and treated using several imaging techniques and different management strategies. Reports from nearly all continents of the globe have contributed to the description of CAFs, not only in Asia and Europe but also throughout North and Latin America. However, these reports have to be cautiously analyzed as many of them were published as a case report and careful interpretation is warranted due to possible publication bias. A literature search was performed using PubMed search interface to select papers dealing with congenital CAFs in adult population between 2000-2009. A total of 233 subjects were collected, and analysed according to their drainage site and treatment modality. They were divided into two subgroups: percutaneous transluminal embolization group (PTE group, n = 122) and surgical ligation group (SL group, n = 111). In the SL group, atherosclerotic coronary artery disease (19%) and associated congenital lesions (23%) were more prevalent compared with the PTE group (9% and 8%), respectively. Infective endocarditis was more frequently seen in the SL group besides syncope, congestive heart failure and hemopericardium. In both groups multimodality diagnostic workup composed of several non-invasive and invasive imaging techniques for fistula visualization were performed and drainage sites into the different cardiac chambers and intrathoracic great vessels were similarly distributed in the two groups.