Rupture of aneurysmal circumflex coronary artery into the left atrium after ligation of its arteriovenous fistula.

Aneurysmal circumflex coronary artery (Cx) with fistulous connection to the coronary sinus is a rare clinical entity that usually remains asymptomatic until later in life, so the ideal therapeutic strategy is poorly defined. The timing of surgical treatment for asymptomatic patients is a big issue, and whether to leave or exclude the diffuse aneurysm in addition to ligation of the fistula is controversial, considering the native myocardial circulation. Complete surgical repair, including exclusion of a diffusely aneurysmal Cx and coronary revascularization to a graftable branch in the circumflex area combined with ligation of its fistula, is quite challenging and sometimes fatal because of a broad posterolateral myocardial infarction without revascularization caused by a lack of graftable branches. A case of diffuse aneurysmal Cx, which ruptured into the left atrium after surgical ligation of its fistulous connection to the coronary sinus, is presented. Simple ligation of the fistula, leaving a gigantic aneurysmal circumflex artery, is hazardous for later rupture and should be avoided. Therapeutic strategies for this complex disorder are discussed, including the optimal timing of surgical treatment.     

Congenital coronary arteriovenous fistula

Three verified cases of congenital coronary arteriovenous fistula have been described, bringing the total reported cases to forty-six. The combination of a machinery murmur in an unusual location, catheterization evidence of a left to right shunt, and opacification of a dilated coronary artery by angiography confirms the clinical diagnosis.

Surgical correction has proved to be safe and effective. The number of surgical cures (including two cases reported herein) now totals fifteen cases with one death due to surgery.     

Congenital arteriovenous fistula between the internal mammary artery and the pulmonary artery

This is the fourth reported case of congenital arteriovenousfistula between the internal mammary artery and pulmonary artery.Precise and complete diagnostic evaluation is required to localize,delineate and appreciate the haemodynamic significance of thistype of arteriovenous shunt. A brief review of the literatureis given with suggestions for diagnosis.     

Congenital coronary arteriovenous fistula associated with dilated cardiomyopathy

A 59-year-old housewife with heart failure was found to have dilated cardiomyopathy associated with bilateral coronary artery fistula to the pulmonary artery. Coexistence of coronary arteriovenous fistula and dilated cardiomyopathy has not been reported and seems to be a casual association.     

Congenital absence of the left circumflex coronary artery

We report a rare case of congenital coronary artery anomaly with recurrent chest pain. A 44-year-old Taiwanese female patient presented with exertional chest pain that had lasted for 2 years. An electrocardiogram showed right axis deviation and an rS pattern in leads I and aVL, and an exercise stress test was inconclusive. A thallium-201 myocardial perfusion study revealed perfusion defects in the septal and inferior walls which normalized in the delayed imaging. Coronary angiography revealed the absence of a left circumflex coronary artery and a superdominant right coronary artery with terminal branches supplying the left ventricular inferior and posterolateral walls. An aortogram revealed no evidence of the existence of a left circumflex coronary artery. Administration of nitrates and calcium antagonists could not ameliorate her symptoms, but she remained stable during ordinary activity. In young premenopausal female patients who present with symptoms suggestive of angina pectoris and a positive stress test, congenital anomaly of the coronary artery should be considered and an angiographic study should be performed.     

A case of bilateral coronary artery to pulmonary artery fistulas associated with hepatic arteriovenous fistula

A 55-year-old man, previously treated for primary hypothyroidism, was admitted for evaluation of atypical chest pain. On physical examination, the pulse rate was 60 and blood pressure was 132/84 mmHg. Heart sounds were normal and no murmur was heard. Abdominal palpation showed no abnormal finding, and bruit was not heard. The electrocardiogram was normal and stress-induced ischemia was not found. The chest X-ray showed no sign of pulmonary hyperaemia or mass lesion, and central shadow was normal (CTR = 44%). During right heart catheterization, the pressures were normal in the cardiac chambers, pulmonary artery, and wedge position. A significant increase in oxygen saturation was disclosed at pulmonary artery level (10%) and the upper site of the inferior vena cava (22%). Coronary arteriography found coronary artery fistulas from the left main trunk, the left anterior descending artery, the left circumflex, and the right coronary artery to the pulmonary trunk. The left and right ventriculograms were normal. The digital subtraction angiography of celiac artery showed hepatic arteriovenous fistula. Ultrasonography and computed tomography found no mass lesion of the liver. Since combination of the bilateral coronary artery with pulmonary artery fistula, and hepatic arteriovenous fistula is very rare, the present case is worth noting for the investigation of the pathogenesis of congenital arteriovenous fistulas.     

Congenital absence of the circumflex coronary artery associated with dilated cardiomyopathy

We report the first autopsied case of congenital absence of the left circumflex coronary artery. The patient was a 12-year-old girl in whom the clinical diagnosis was idiopathic dilated cardiomyopathy. This type of heart disease is uncommon among children. The coexistence of the two conditions therefore suggests a possible aetiologic relationship between them. The pathological findings, however, do not support such an association. Rather, they suggest that they co-exist by chance.     

Therapeutic embolization of the pulmonary artery in pulmonary arteriovenous fistula

A 35 year old white man was admitted in January 1976 for further treatment of pulmonary A-V fistula. In 1975, he had dyspnea, cyanosis and clubbing; pulmonary angiogram showed a fistula in the lower lobe of the left lung and one in the upper lobe of the right lung; the arterial oxygen tension (PaO₂) was 54 mm Hg and the calculated intrapulmonary shunt was 25 per cent of the cardiac output; a lobectomy of the left lung was performed. On this admission, dyspnea, cyanosis and clubbing were still present: the PaO₂ was 67 mm Hg and the intrapulmonary shunt 14 per cent. Since it was possible to show angiographically that, by balloon inflation during Swan-Ganz catheterization of the upper lobe of the right lung, the single pulmonary A-V fistula was completely obliterated with correction of the intrapulmonary shunt, mechanical occlusion was attempted as an alternative to further surgery. Through a No. 7 French catheter inserted via a femoral vein into the pulmonary artery of the upper lobe of the right lung and with the help of repeated selective angiography, six mechanical occluding devices (each consisting of a 5 cm segment of steel guidewire with 3 cm wool tails) were placed into the vessels feeding the fistula. Twenty minutes after placement of the last two coils, the fistula was almost totally occluded on angiogram; the PaO₂ was 79 mm Hg and the intrapulmonary shunt was 7 per cent; over the following three weeks, without further manipulation, these returned to normal (91 mm Hg and 4 per cent, respectively). No complications have been noted. The patient has been symptom free during 12 months of close follow-up; results of oxygen studies have remained normal; on chest film the six coils have not changed position. The procedure described here, to our knowledge performed for the first time for correction of pulmonary A-V fistula, should be considered in preference to surgical resection in selected patients with this condition.     

Multicystic aneurysmal dilatation of bilateral coronary artery fistula

Bilateral coronary artery fistula constitutes an uncommon subgroup of coronary artery fistulas that may have a distinct embryologic origin. Coronary artery fistulas usually show a tortuous arrangement upon coronary angiography, but aneurysmal dilatation is rare. We report here an extremely rare case of coronary artery fistula originating from both coronary arteries, which showed multicystic aneurysmal dilatation.     

Congenital coronary arteriovenous fistula: a diagnostic challenge

This report describes a case of coronary arteriovenous (A-V) fistula involving both the right and the left coronary arteries. The venous component of the two circulations combines into a single channel that empties into the left ventricle just below the mitral valve.