Long-Term Follow-Up of Pediatric Patients After Congenital Coronary Artery Fistula Closure.

The surgical closure of congenital coronary artery fistulas (CAF) is associated with excellent immediate outcomes. Few studies have investigated the long-term prognosis in patients who have undergone surgery for the closure of CAF or differentiated among types of CAF or types of surgical procedures. In this study, we performed clinical examinations and computed tomography angiography (CTA) to characterize outcomes after CAF closure in pediatric patients. The medical records of 79 pediatric patients who underwent surgical closure of CAF were retrospectively reviewed. The median age of the patients included in the study at the time of surgery was 3.4 years (range 0.2 to 15.3 years). The patients had been followed up for 11 years (range 1 to 17 years) with electrocardiography, echocardiography, and coronary CTA. There were 67 medium-to-large CAF and 12 small CAF. Twenty-six (32.9%) CAF arose from the branch coronary artery (proximal type); the others arose from the parent coronary artery (distal type). The surgical procedure included endocardial closure in 16 cases, epicardial distal ligation in 51 cases, epicardial proximal and distal ligation in 12 cases. There was no instance of perioperative death among the cases included in the study. Twenty-eight patients were treated with antiplatelet medication postoperatively. No patient required re-operation during the follow-up period. Coronary thrombi were detected in 27 patients (34.2%). There was no instance of myocardial ischemia related to thrombosis. Among the patients with thrombosis, 26 had medium-to-large CAF (96.3%), and 23 had distal-type CAF (85.2%). Average age at surgery was higher among the patients with thrombosis than among the patients without thrombosis (7.4 years vs. 3.3 years, t = 5.509, P = 0.000). Among the patients with distal-type CAF, thrombosis was more common among the patients treated with ligation than treated with endocardial closure (41.5% vs. 16.7%, χ2 = 3.742, P = 0.043). There was no difference in risk for thrombosis between the patients who did vs. did not receive antiplatelet therapy (P = 0.436). The most common complication after CAF closure was thrombosis. Increased risk for thrombosis was associated with large fistulae, distal-type CAF, and older age at presentation. Antiplatelet treatment did not appear to decrease the risk of thrombosis. Among patients with distal-type CAF, risk for thrombosis was lower among patients treated with endocardial closure, compared with patients treated with epicardial ligation.

Sex-linked differences in the mortality in Ren-2 transgenic hypertensive rats with aorto-caval fistula: effects of treatment with angiotensin converting enzyme alone and combined with inhibitor of soluble epoxide hydrolase.

We found recently that in Ren-2 transgenic hypertensive rats (TGR) addition of soluble epoxide hydrolase inhibitor (sEHi) to treatment with angiotensin-converting enzyme inhibitor (ACEi), surprisingly, increased the mortality due to heart failure (HF) induced by creation of the aorto-caval fistula (ACF). Since TGR exhibit sex-related differences in mortality, we examined here if such differentiation exists also in the response to the treatment with ACEi (trandolapril), alone or combined with sEHi [cis-4-[4-(3-adamantan-1-yl-ureido)cyclohexyloxy]benzoic acid, (c-AUCB)]. ACEi improved survival in males to 74 % (vs. 0 %) and in females to 65 % (vs. 32 %). ACEi and sEHi combined also improved the survival in male ACF TGR, however, it was significantly less (38 %) than after ACEi alone. In contrast, in females the combined treatment significantly improved the final survival rate (84 %). There were no significant sex-linked differences in survival rate in untreated or treated normotensive Hannover Sprague-Dawley rats. In conclusion, in HF patients with co-existing hypertension and RAS hyperactivity, the sex may co-determine the rate of HF progression, and can influence the effectiveness of the therapeutic measures applied. Therefore, in the relevant pre-clinical studies the sex-linked differences should be seriously considered. Our data indicate that TGR might be an optimal model for such studies.

Hypertrophic obstructive cardiomyopathy with multiple coronary arteries to right ventricular microfistulas: a case report and review of the literature.

BACKGROUND: Coronary artery microfistulas are a rare anomaly; their association with hypertrophic cardiomyopathy is even rarer and can lead to serious cardiac complications owing to coronary steal phenomena such as angina pectoris, myocardial infarction, congestive heart failure, ventricular and supraventricular arrhythmias, syncope, and sudden death. CASE PRESENTATION: A 32-year-old Indian woman presented to our institute with severe angina on exertion and multiple episodes of pre-syncope. Echocardiography revealed hypertrophic obstructive cardiomyopathy. Coronary angiography showed no significant atherosclerotic lesions; however, it revealed multiple microfistulas originated from all three major coronary arteries and draining into her right ventricle. This finding was confirmed by the rapid filling of the pulmonary artery after dye was injected into her left coronary artery during a cardiac catheterization study and by a significant oxygen step up of 15 % seen from her right atria to right ventricle during oximetry analysis. We treated our patient's condition with medical therapy including metoprolol and nicorandil. She improved and angina grade had decreased from class III to class II on a follow-up visit 1 month after discharge. CONCLUSIONS: In this case report and literature review, we highlight an unusual but important association that can lead to symptomatic worsening of angina in young patients with hypertrophic cardiomyopathy owing to coronary steal phenomena.

Propranolol as a treatment for multiple coronary artery micro-fistulas.

We present a case of congenital multiple coronary artery-left ventricle micro-fistulas, which were treated with propranolol disappearing within 6 months. She had a malformative syndrome associated with a chromosomal abnormality. The treatment for coronary artery fistula includes surgical ligation and transcatheter closure, but they are not indicated in congenital micro-fistulas. We propose propranolol as a treatment in this type of diffuse fistula.

Case report and review of literature: coronary artery fistulae.

Coronary artery fistula (CAF) is a rare disease. In more than 90 percent of cases, a single fistula drains into the right heart chambers or into the pulmonary artery. Fistulae draining into the left ventricle are uncommon; further, multiple CAF involving the three major coronary artery vessels are very rare. This report will describe two rare cases of CAF, one of multiple CAF, and the other of single CAF, all of which drained into the left ventricular chamber.

[Coronary ventricular fistula as a late complication of permanent pacing]. / Fistola coronaro-ventricolare come complicanza tardiva di elettrostimolazione ventricolare definitiva.

A coronary angiography performed for the occurrence of atypical chest pain allowed us to discover a coronary-right ventricular fistula as a rare complication of myocardial penetration by a tined ventricular catheter implanted some years earlier.

Multiple coronary cameral fistulae: a rare anomaly and cause of ischaemia.

An 82-year-old hypertensive, diabetic woman was admitted to our department for pre-operative cardiac evaluation. A myocardial perfusion scan revealed apicoseptal and inferior segment hypoperfusion. Coronary angiography exhibited extensive multiple coronary cameral fistulae draining into the left ventricle in a homogeneous and circular way.

Multiple coronary fistulas originating from all major coronary arteries.

Coronary fistulas to one of the cardiac chambers are infrequent anomalies and usually are found unexpectedly. Fistulas communicating between coronary artery and left heart chambers are extremely rare, especially those emptying into the left ventricle. We report a case in which all three major coronary arteries emptied into the left ventricle through multiple microfistulas.

Snake heart: a case of atavism in a human being.

Atavism is the rare reappearance, in a modern organism, of a trait from a distant evolutionary ancestor. We describe an apparent case of atavism involving a 59-year-old man with chest pain whose coronary circulation and myocardial architecture resembled those of the reptilian heart. The chest pain was attributed to a coronary steal phenomenon. The patient was discharged from the hospital on a heightened regimen of ß-blockers, and his symptoms improved significantly. To our knowledge, this is only the 2nd reported clinical case of a human coronary circulation similar to that of reptiles.

Myocardial infarction caused by a fistula between the left anterior descending coronary artery and the left ventricle.

Herein, we describe the case of a 50-year-old man who had a fistula, located between the left anterior descending coronary artery and the left ventricle, that caused myocardial infarction. Electrocardiography revealed a loss of R-wave progression in leads V(1) through V(4), and transthoracic echocardiography showed an apical aneurysm. Selective coronary angiography was performed. Dimensions of the left anterior descending coronary artery, and digital caliper measurements of stenosis within, were normal. After the injection of angiographic contrast material from the distal part of the left anterior descending coronary artery, a smoky, opaque accumulation colored the left ventricle. The digital caliper measurement of the left anterior descending coronary artery was again found to be normal. In the apex of the left ventricle, ventriculography in the left anterior oblique position revealed a small aneurysm, and a myocardial perfusion scan showed a fixed perfusion defect.Cases of coronary fistulae that result in myocardial infarction are rare. Because the patient had no ongoing symptoms after his myocardial infarction, medical therapy was prescribed.

A case of coronary cameral fistula.

An 85-year-old woman underwent transthoracic echocardiography for the investigation of breathlessness and atypical chest discomfort. Clinical examination was unremarkable. A standard 12 lead ECG demonstrated anterior T wave inversion, but was otherwise normal. Transthoracic echocardiography demonstrated a normally functioning left ventricle with hypertrophy and trabeculation of the apical and lateral segments. Imaging with colour flow Doppler demonstrated blood flow from the epicardial surface into the left ventricular cavity through the hypertrophied segment of myocardium during diastole. A diagnosis of multiple, diffuse coronary-left ventricular fistulae predominantly of a large diagonal branch of the left anterior descending artery was made at coronary angiography. The patient responded well to oral beta-blockade, reporting an improvement in symptoms 2 months later in the outpatient clinic. The echocardiographic appearances of coronary fistulae may cause diagnostic confusion, particularly in the presence of myocardial hypertrophy and trabeculation.

Successful one-stage operation of aortoesophageal fistula from thoracic aneurysm using a rifampicin-soaked synthetic graft.

Aortoesophageal fistula secondary to thoracic aneurysm is rare, but is usually lethal, and few survivors have been reported. We report successful surgery for aortoesophageal fistula in a one-stage operation. Repair involved in situ replacement of the thoracic aneurysm using a rifampicin-soaked graft, primary repair of the esophagus, omental wrap and tube jejunostomy. This is the original report of the surgical repair of aortoesophageal fistula using a rifampicin-soaked graft.

Aorto-cardiac fistulas in seven horses.

This report describes the history, clinical, electrocardiographic and echocardiographic findings, treatment, outcome and post-mortem findings in seven horses with aorto-cardiac fistula. Affected horses included 5 stallions, one gelding and one mare; 2 each of the Thoroughbred, Arabian and Standardbred breeds and one Thoroughbred-cross with a mean +/- s.d. age of 12 +/- 4 years, range 6-18 years. The presenting signs were acute distress (four horses), exercise intolerance (two horses) and the lesion was detected during a routine examination in one horse. Five horses had monomorphic ventricular tachycardia on admission and one other had a history of this arrhythmia. Five horses had a characteristic continuous murmur loudest in the right fourth intercostal space. Echocardiography (six horses) and/or post-mortem examination (four horses) revealed the horses had aorto-cardiac fistulas arising from the right aortic sinus in all five horses in which the site was recorded. Two horses had ruptured aneurysmal dilatations of the aortic wall at this site. Fistulas extended into the right ventricle in four horses; the right atrium in two horses, the left ventricle in one horse, and five horses had dissecting tracts in the septal myocardium. Horses survived for periods ranging from 24 h to 4 years. Aorto-cardiac fistula should be considered in the differential diagnosis for horses presenting with acute distress, bounding arterial pulse, a right-sided continuous murmur and/or monomorphic ventricular tachycardia, particularly in middle-aged or older stallions. Echocardiography is the technique of choice for confirming the diagnosis and demonstrating accompanying cardiac changes.