Abstract
Coronary artery fistulae (CAF) are uncommon heart defects defined as a communication between a coronary artery and a cardiac chamber or vascular structure. They are frequently asymptomatic; nevertheless, they can produce angina, dyspnea or cardiac failure. CAF are believed to be congenital; however, isolated cases of CAF have been described as rare complications of cardiac surgery. We report the percutaneous closure of a giant CAF in an adult patient with angina and previous pericardiectomy.
Highlights
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Coronary artery fistulae in adults are a rare finding.
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Coronary artery fistulae can be consequence of previous cardiac surgery.
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Transcatheter closure is a feasible and effective alternative therapy.
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The “wire-maintaining technique” for percutaneous closure of fistulae is suggested.
1
Introduction
Coronary artery fistulae (CAF) are uncommon heart defects defined as a communication between a coronary artery and a cardiac chamber or vascular structure . They are frequently asymptomatic; nevertheless, they can produce angina, dyspnea or cardiac failure.
CAF are believed to be congenital; however, isolated cases of CAF have been described as rare complications of cardiac surgery . We report the percutaneous closure of a giant CAF in an adult patient with angina and previous pericardiectomy.
2
Case report
A 69-year-old woman with a three-month history of angina and minimal effort dyspnea was admitted for percutaneous closure of a left coronary circumflex (LCX) CAF. The patient had suffered a constrictive pericarditis 14 years before, with a normal coronary angiogram followed by a surgical pericardiectomy; and was under oral anticoagulant therapy with apixaban due to chronic atrial fibrillation. A right catheterization ruled out significant constriction showing data concordant with high cardiac output (11l/min). The coronary angiogram performed in another center showed a big circumflex coronary artery, with a tortuous pathway and an independent drainage into the right atrium, through the coronary sinus ( Fig. 1 A ).
Given the high risk of a new surgical intervention (Euroscore II 4.7%), the patient’s case was discussed in the Heart Team and a percutaneous closure of the coronary artery fistula was planned.
Right femoral artery was canalized, advancing a multipurpose 6 French catheter to the left aortic coronary sinus, pushing a 0.35″hydrophilic Terumo wire through the tortuous pathway of the fistula, reaching the right atrium ( Fig. 1 B, Video 1 ). Given the need of an accurate support to advance the closure device, an arteriovenous loop was performed, canalizing the right femoral vein, to capture the wire in the right atrium with a 30 mm gooseneck, externalizing it through the venous access to establish the circuit ( Fig. 1 C). This maneuver allowed for the selective canalization of the fistula from the arterial side, permitting a direct angiography inside the vessel to measure the distal length of the drainage mouth at the right atrium, with a maximal diameter of 10 mm ( Fig. 1 D). A 10 French Amplatzer sheath was then progressed from the venous side of the loop, advancing a 14× 10mm Vascular Plug II through the sheath, near the arteriovenous wire, that was maintained during the entire procedure ( Fig. 2 A , Video 2 ). After verifying the complete closure of the fistula, the device was released with embolization to the left pulmonary artery in the following beats ( Video 3 ). The left femoral vein was then canalized to capture the device with a 20 × 10 Gooseneck, exteriorizing it through the femoral vein ( Fig. 2 B, Video 4 ). A 12 × 10 mm-ductal occluder device was then implanted at the fistula drainage, verifying stability of the device before the release, and confirming a complete closure of the fistula with contralateral injections from the arterious side ( Fig. 2 C and D, Videos 5 and 6 ).
The procedure was finalized without complications and the patient was discharged 24 h after the intervention under treatment with oral anticoagulant therapy. Six months after the procedure, she remains asymptomatic, with a control CT scan showing complete closure of the fistula with accurate position of the implanted device ( Fig. 3 ).
2
Case report
A 69-year-old woman with a three-month history of angina and minimal effort dyspnea was admitted for percutaneous closure of a left coronary circumflex (LCX) CAF. The patient had suffered a constrictive pericarditis 14 years before, with a normal coronary angiogram followed by a surgical pericardiectomy; and was under oral anticoagulant therapy with apixaban due to chronic atrial fibrillation. A right catheterization ruled out significant constriction showing data concordant with high cardiac output (11l/min). The coronary angiogram performed in another center showed a big circumflex coronary artery, with a tortuous pathway and an independent drainage into the right atrium, through the coronary sinus ( Fig. 1 A ).
Given the high risk of a new surgical intervention (Euroscore II 4.7%), the patient’s case was discussed in the Heart Team and a percutaneous closure of the coronary artery fistula was planned.
Right femoral artery was canalized, advancing a multipurpose 6 French catheter to the left aortic coronary sinus, pushing a 0.35″hydrophilic Terumo wire through the tortuous pathway of the fistula, reaching the right atrium ( Fig. 1 B, Video 1 ). Given the need of an accurate support to advance the closure device, an arteriovenous loop was performed, canalizing the right femoral vein, to capture the wire in the right atrium with a 30 mm gooseneck, externalizing it through the venous access to establish the circuit ( Fig. 1 C). This maneuver allowed for the selective canalization of the fistula from the arterial side, permitting a direct angiography inside the vessel to measure the distal length of the drainage mouth at the right atrium, with a maximal diameter of 10 mm ( Fig. 1 D). A 10 French Amplatzer sheath was then progressed from the venous side of the loop, advancing a 14× 10mm Vascular Plug II through the sheath, near the arteriovenous wire, that was maintained during the entire procedure ( Fig. 2 A , Video 2 ). After verifying the complete closure of the fistula, the device was released with embolization to the left pulmonary artery in the following beats ( Video 3 ). The left femoral vein was then canalized to capture the device with a 20 × 10 Gooseneck, exteriorizing it through the femoral vein ( Fig. 2 B, Video 4 ). A 12 × 10 mm-ductal occluder device was then implanted at the fistula drainage, verifying stability of the device before the release, and confirming a complete closure of the fistula with contralateral injections from the arterious side ( Fig. 2 C and D, Videos 5 and 6 ).
The procedure was finalized without complications and the patient was discharged 24 h after the intervention under treatment with oral anticoagulant therapy. Six months after the procedure, she remains asymptomatic, with a control CT scan showing complete closure of the fistula with accurate position of the implanted device ( Fig. 3 ).
3
Discussion
3.1
CAF origin
CAF are rare, with an observed prevalence of less than 1% of patients undergoing coronary angiography . CAF may be either congenital or acquired; sometimes related to disease processes that damage the vessels, such as infection, inflammation and malignancy. In addition, trauma to the vessels, whether iatrogenic (as in cardiothoracic surgery and interventional procedures) or non-iatrogenic, may lead to fistula formation .
In this patient, the association between previous pericardiectomy and CAF seems extremely rare, so that a casual association between both entities may be postulated. Cannulation of the right atrium for cardiopulmonary bypass during extracorporeal circulation has been reported as a cause of CAF and may have been related to the development of this entity in this patient. Surgical manipulation of the visceral pericardium at the coronary sulcus may have also played a role starting with an inflammatory process of the coronary sinus drainage.
3.2
CAF, to treat or not to treat: events during follow-up
There is controversy about the best management of CAF, given the fact that the natural history of this entity has not yet been well studied. Cases of spontaneous closure, although rare, have been reported, usually occurring in pediatric patients . However, a considerable number of large fistulas have been documented to expand aneurysmally and even dissect or rupture, with fatal consequences . In adult patients, the main consensus of opinions is in favor of operation once the condition is diagnosed. In contrast, little CAF found in children population could benefit from a provisional conservative management, with an intensive surveillance.
Several reports have warned about the potential adverse events after CAF closure, with described cases of thrombosis or myocardial infarction during the follow-up . The risk factors for these events have not been yet adequately identified. Recent works have suggested that large size, distal type of CAF , and CAF draining to the coronary sinus may be at higher risk for coronary thrombosis; however, limited data are available, and the published articles collect only small series of cases, with a limited value.
Reviewing the literature, there are only 11 published series with subsequent follow-up ( Table 1 ), with a high variability of patients (children and adults) and closure techniques (surgical or transcatheter). These series show significant differences between the rates of CAF thrombosis; probably related to the selected method employed for the surveillance of the patients, with very few cases with an available coronary angiogram during the follow-up. Although in these series the majority of CAF thrombosis occurred after surgical closure ; these cases corresponded to the more complex anatomy CAF (large fistulae with multiple drainages); so that definite conclusion cannot be inferred from these findings. Table 2 summarizes the reported cases of percutaneous closure of coronary artery fistulae in adult patients.
