High-output Cardiac Failure Caused by Large Coronary Artery Fistulas Treated with Coil Embolization

Etiology, Characteristics and Clinical Outcomes in High Output Heart Failure: A 15 Year Experience

Transcatheter closure of medium and large congenital coronary artery fistula using wire-maintaining technique

Coronary artery fistulas (CAF) are uncommon entities often associated with myocardial ischemia and high output failure. Surgical options include ligation of the fistula, with/without simultaneous coronary artery bypass grafting (CABG). We report a case of left main coronary artery (LMCA) fistula to the coronary sinus (CS), which was associated with high-output bi-ventricular failure, and moderate mitral (MR) and tricuspid regurgitation (TR), related to the volume overload and annular dilatation. This was tackled elegantly by off-pump CABG to protect the territories supplied by the LMCA, followed by ligation of the fistula. This resulted in resolution of the MR and TR. Intraoperative transesophageal echocardiogram (TEE) greatly facilitated the surgical treatment, by identifying the origin and the draining points for the fistula, and aided in the quantification of MR and TR, which had regressed sufficiently at the end of the procedure and did not require surgical correction. This article outlines the importance of multi-disciplinary treatment approach for this complex condition.

Coronary artery fistula (CAF) is uncommon but remains the most frequent hemodynamically significant congenital coronary artery anomaly.1 The majority of fistula is single and drains into the right heart, only 3.5% into the left ventricle.2 A large fistula requires closure to prevent complications such as myocardial ischemia resulting from coronary steal, congestive heart failure, endocarditis and potential aneurysmal dilatation and rupture.3-5 Here we presented a rare case of CAF with multiple origins involving left anterior descending artery (LAD), left circumflex branch (LCX) and right coronary artery (RCA), and draining into the left ventricle, which was successfully closed by coil embolization. CASE REPORT A 61-year-old man who complained of chest pain and fatigue on exertion for two years, was admitted to our hospital in March 2007. Physical examination revealed nothing particular and no continuous murmur audible. A chest X-ray showed a cardiothoracic ratio of 45.5%, and there was normal sinus rhythm with no significant ST-T changes on regular electrocardiogram (ECG). On two-dimensional echocardiography, the RCA was dilated. Doppler echocardiography showed a continuous flow into the left ventricle (LV) beneath the posterior leaflet of the mitral valve (Figure 1). A RCA-to-LV fistula was suspected. Selective coronary angiograms demonstrated a common large tortuous fistula arising from the distal LAD, LCX and RCA draining into the LV. The proximal LAD, LCX and RCA were dilated and tortuous (Figure 2). After careful discussion, a staged transcatheter coil embolism regimen was taken.Figure 1.: Doppler echocardiography shows a continuous flow into the left ventricle beneath the posterior leaflet of the mitral valve.Figure 2.: Selective coronary angiograms show a common fistula arising from the distal LCA and RCA running in the LV.The RCA was engaged with a 7F JR 4.0 (Cordis, USA) guiding catheter. Due to the microcatheter (Cook, Europe) not long enough to reach CAF, we had to shorten the length of JR 4.0 guiding catheter by cutting the proximal of 7F JR 4.0 guiding catheter and connecting to 6F JR 4.0 guiding catheter tail-to-tail. A runthrough 0.3556 mm (0.014') guide wire (Terumo, Japan) and a microcatheter were passed through this guiding catheter to the RCA together, after guide wire removed, 2 larger microcoils (Cook) (6 mm × 5 mm) were placed as anchors and then 2 smaller microcoils (3 mm × 3 mm) were released to embolize the CAF. And then the LCX-CAF was successfully closed with similar method. Briefly, 7F JL 4.0was used to engage the left coronary artery (LCA), a microcatheter was positioned in the distal LCX over the guide wire, and 4 microcoils (two 6 mm × 5 mm, two 3 mm × 3 mm) were used to close LCX-CAF successfully. Repeated angiography 15 minutes later showed satisfactory result of the RCA-CAF and LCX-CAF without residual shunting. A month later, the patient was readmitted for another transcatheter closing for LAD-CAF. Satisfied result in LCX and RCA was verified by repeated coronary angiogram. 7F JL 4.0 was used to engage the LCA, 2 microcoils (3 mm × 3 mm) were used to close LAD-CAF through microcatheter successfully. Complete closure of the coronary artery fistula was confirmed by angiography (Figure 3). The patient is on close follow up. At 6 months, patients did not present with any symptoms of chest discomfort and repeated Doppler echocardiography confirmed persistent occlusion of the fistula with no residual shunt.Figure 3.: Post-intervention selective coronary angiograms show complete occlusion of the fistula.DISCUSSION CAF, an uncommon entity of congenital heart disease, first described by Krause in 1865, was characterized by the involved coronary artery having a normal origin from the aorta with a fistulous communication with the atria or ventricles, or with the pulmonary artery (PA).6 During the embryonic development, sinusoid gap of trabeculae of cardiac muscle is connected to coronary artery. Along with the development of myocardium the sinusoid gap was compressed and degraded to capillary. But if sinusoid gap persisted instead of degraded it would turn to be CAF. The majority of fistula drains into the right heart, with 15% into the pulmonary artery and 7% into the coronary sinus. Congenital arteriosystemic fistula rarely drains into the left heart, only 5% draining into the left atrium and 3.5% into the left ventricle.2 And most of the CAF is single. Here we presented a case of CAF with multiple origins involving LAD, LCX and RCA, and draining into the left ventricle. CAF with large shunt can cause heart failure, myocardial ischemia, arrhythmia, infective endocarditis or aneurysm progressive enlargement and rupture.3-5 In this case with significant shunt, blood may preferentially flow to the coronary fistula at the expense of the other coronary arteries, a phenomenon also known as “coronary steal”. Coronary steal is particularly evident during times of stress and can result in myocardial ischemia to territories not supplied by the arterial fistula. So the patient presented with chest pain. Recently, transcatheter intervention was provided as a new therapeutic approach for characteristics of high efficacy and micro-invasive. Coils, detachable balloons, polyvinyl alcohol foam and occlusive devices have been successfully used in the closure of CAF.7-11 In this case with multiple CAF, which might result in longer operation time, greater contrast volume, it needs to be performed by stage for the sake of safety. Moreover, the location of CAF, which is located in the distal coronary artery and extremely tortuous, inspired us to use coil other than Amplatzer duct occluder or else.10 During implementing the RCA coil embolization, due to the obvious tortuous and the microcatheter can not reach the satisfactory position, we connect a 7F JR 4.0 guiding catheter with a 6F JR 4.0 one, which can effectively shorten the length of guiding catheter and easily send the microcatheter to proper position to release coil. This technology is mainly used in the percutaneous transcoronary angioplasty. The application to CAF embolization is rarely to be reported. In conclusion, we successfully treated a complicated, high-flow coronary artery fistula by transcatheter coil embolization. Catheter embolization is an effective and safe treatment for coronary artery fistula in the strictly selected cases.

A 45-year-old female patient was referred due to the abnormal myocardial perfusion scintigraphy showing ischemia in the inferior and lateral wall. Coronary arteries were normal, and a large fistula was detected from the proximal portion of the circumflex coronary artery (Cx) draining into the pulmonary artery. Percutaneous closure of the coronary artery fistula (CAF) was considered, and a 3 mm × 50 mm-Balt coil was planned to place the proximal portion of the fistulized artery. Unfortunately, during placement of the coil, it was opened early and migrated to the proximal segment of the Cx, the left anterior descending artery, and the distal part of the left main coronary artery. A snare was moved into the extra backup guiding catheter immediately. The migrated coil was retrieved with the snare successfully. Subsequently, 4 mm × 12 mm and 2 mm × 25 mm-Balt coils were placed in the mid portion of the fistulized artery until total occlusion was obtained. A CAF is described as a direct connection between one or more of the coronary arteries and a cardiac chamber or great vessel. The fistula may cause serious hemodynamic disturbances such as myocardial ischemia, high-flow heart failure, right ventricle volume overload, endocarditis, rupture, thrombosis, embolism, and arrhythmias. Percutaneous closure is the prior technique, in the absence of complex conditions such as multiple fistulas and large fistula branches and in cases where the fistula can be simply reached. There have been very rare data which contain complications about the percutaneous closure of CAFs.

IP155. Intraoperative Ultrasound Guidance for Banding of an Arteriovenous Fistula Causing High Cardiac Output Heart Failure

Renal arteriovenous fistula associated high-output heart failure treated with embolization

Background: The literature on the etiologies and complications of high-output heart failure (HOHF) is limited. Objective: To study the causes and complications related to HOHF in the United States (US). Methods: Data from the national readmissions database (NRD) sample that constitutes 49.1% of the stratified sample of all hospitals in the United States, representing more than 95% of the national population were analyzed for hospitalizations with primary diagnosis of HOHF for the years 2017-2018. Etiology associated with HOHF were extracted using ICD-10 codes. Results: A total of 2,107 index hospitalizations (mean age 62.2 ± 19.1) with primary diagnosis of HOHF were recorded in the NRD for the years 2017-2018. The most common causes of HOHF include sepsis 204 (9.7%), leukemia 53 (2.5%), arteriovenous fistula 13 (0.6%), liver cirrhosis 155 (7.4%), Hyperthyroidism 133 (6.3%), thalassemia 23 (1.14%), sickle cell disease 71 (3.35%), morbid obesity 188 (8.95%), COPD 406 (19.3%), myeloproliferative disorders 166 (7.87%). Among the HOHF group, major complications include acute ischemic stroke (42 or 2%), acute kidney injury (593 or 28.1%), hypertensive emergency (74 or 3.5%), atrial fibrillation (409 or 19.4%), ventricular tachycardia/fibrillation (77 or 3.7%), and conduction block (81 or 3.8%) and ST-Elevation myocardial infarction (11 or 0.5%). A total of 83 (3.9%) patients had died during the inpatient hospitalization. Out of the remaining 2,024 patients, a significant portion (62 or 3.1%) required readmission within 30 days. Conclusion: HOHF is an under-reported cardiovascular complication associated with non-cardiovascular disorders. HOHF is associated with significant 30-day readmissions and mortality rates. Proper management of the underlying etiology can prevent the development of HOHF and associated complications. Keywords: cirrhosis; hemodynamics; obesity, leukemia, myeloproliferative disorders, ST-Elevation myocardial infarction (STEMI).

BackgroundThis case reviews a challenging but successful transcatheter coil embolization of a large congenital coronary artery fistula (CAF) causing a significant left-to-right shunt.Case summaryA 51-year-old female with no significant prior history presented with symptoms of dyspnoea and chest discomfort. Extensive evaluation revealed a large CAF between a tortuous right coronary artery (RCA) and the coronary sinus (CS) composed of three aneurysmal pseudochambers. Closure of the RCA-CS fistula was attempted through coil deployment into the fistula neck. However, due to the brisk flow through the fistula, both coils embolized into the fistula sac. An alternative location was subsequently identified on three-dimensional rendering of a computed tomography angiography scan, which revealed a sharp bend in the RCA prior to the fistula neck and distal to the posterior descending artery (PDA) takeoff. Repeat attempt at embolization was accomplished using a telescoping system to reach and occlude the targeted bend. The coil mass remained stable and angiography demonstrated reduced flow through the fistula and preserved patency of the PDA. The decreased residual flow through the fistula secondary to the initial embolization attempt likely aided the successful deployment of coils in the second and final attempt. At 1 year, the patient was doing well with resolution of her symptoms and no clinical symptoms of coronary ischaemia.DiscussionWe suggest that an initial unsuccessful attempt at transcatheter embolization of a CAF should not preclude subsequent attempts for closure when there exists an appropriate indication.

Patients with high-flow arteriovenous (AV) access are at risk of developing high-output cardiac failure (HOCF) and subsequent hospitalization. However, diagnosing HOCF is challenging and often requires invasive procedures. The role of systemic vascular resistance (SVR) in diagnosing HOCF is underestimated, and its predictive value is limited. Our study aims to identify non-invasive risk factors for HOCF to facilitate early diagnosis and timely surgical interventions. We included 109 patients with high-flow AV access who underwent serial echocardiography. The retrospective cohort was divided into two groups based on their hospitalization due to HOCF. The two groups were matched for age and gender. After a mean follow-up of 25.1months, 19 patients (17.4%) were hospitalized due to HOCF. The two groups had similar baseline characteristics. However, the HOCF group had a higher value of vascular access blood flow (Qa) (2168±856 vs. 1828±617mL/min; P=0.045). Echocardiographic analysis revealed that the HOCF group had more pronounced left ventricular diastolic dysfunction (E/e': 21.1±7.3 vs. 16.2±5.9; P=0.002), more severe pulmonary hypertension (right ventricular systolic pressure: 41.4±16.7 vs. 32.2±12.8; P=0.009), a higher Doppler-derived cardiac index (CI) (4.3±0.8 vs. 3.7±1.1; P=0.031), and a lower Doppler-derived estimated SVR (eSVR) value (5.5±0.3 vs. 6.9±0.2; P=0.002) than the non-HOCF group. Using multivariable Cox regression analysis, a low eSVR value (<6) emerged as an independent predictor of HOCF hospitalization with a hazard ratio of 9.084 (95% confidence interval, 2.33-35.39; P=0.001). Receiver operating characteristic curve analysis indicated that CI/eSVR values more accurately predicted HOCF hospitalization [sensitivity: 94.7%, specificity: 51.0%, area under the curve (AUC): 0.75, P<0.001] than the Qa/cardiac output ratio (AUC: 0.50, P=0.955), Qa values≥2000mL/min (AUC: 0.60, P=0.181), and Qa values indexed for height in metres (AUC: 0.65, P=0.040). In patients with high-flow AV access, low eSVR values obtained through non-invasive Doppler echocardiography were associated with a high rate of HOCF hospitalizations. Therefore, routine eSVR screening in these patients might expedite the diagnosis of HOCF.

Introduction: High output heart failure (HOHF) can be attributed to a wide array of diseases such as severe anemia, hyperthyroidism, arteriovenous fistulas, cirrhosis, chronic lung disease, and morbid obesity. Amongst the causes, obesity is the most common, accounting for 31% of those diagnosed with HOHF; however, there is limited data regarding this topic despite the increasing prevalence of obesity worldwide. We recount a unique case of HOHF in a young woman. Case Description: A 22-year-old woman with a history of class 3 obesity (body mass index 60.1 kg/m2) presented with shortness of breath for 3 weeks and new bilateral lower extremity edema. She was found to have newly reduced left ventricular function of 10-15% with severe dilation of the left ventricle and global hypokinesis. Her cardiac catheterization showed insignificant coronary artery disease. However, hemodynamics showed elevated filling pressures, a cardiac index of 4.6 L/min/m2 and output of 11.9 L/min, both measured by thermodilution; establishing a diagnosis of high output heart failure. The patient’s right ventricular biopsy returned and showed chronic cardiomyopathy, demonstrated by hypertrophied myocytes. Other etiologies of HOHF were ruled out with further workup, including arteriovenous shunting, thyroid disease, liver disease, and pulmonary pathologies. The etiology of her HOHF was ultimately attributed to severe obesity. After diuresis, the patient was discharged on guideline directed medical therapy, which included sacubitril-valsartan, metoprolol succinate, spironolactone, and empagliflozin. She was set to follow up with advanced heart failure and bariatric surgery. Conclusion: HOHF is due to unmet demands by the body, by increased oxygen consumption or by decrease systemic vascular resistance. The pathophysiology for HOHF due to obesity is not entirely understood, however there are several working hypotheses. The pathophysiology stems from obesity causing increased leptin, neprilysin, and decreased adiponectin; increased vasoactive adipokines increasing increased vasodilation; insulin resistance causing cardiac remodeling seen as impaired diastology with low ejection fractions. Treatment of HOHF due to morbid obesity is with bariatric surgery and lifestyle changes. However, given the role of active adipokines in this disease, future studies can aim to investigate intervening at this level to prevent severe consequences, as seen in our patient.

Introduction: Coronary artery fistulas are rare congenital anomalies that are usually incidentally discovered on cardiac imaging or coronary angiography. We present an extremely rare case of a large left main coronary artery (LMCA)/left circumflex artery (LCX) fistula to the superior vena cava (SVC) successfully managed by transcatheter closure through coil embolization. Case Presentation: A 63-year-old male competitive bodybuilder, with no prior cardiac history, presented with lightheadedness and wide complex tachyarrhythmia at a heart rate over 240 beats per minute in the field, requiring two defibrillator shocks. His subsequent EKG demonstrated diffuse ST depressions. Coronary angiography revealed a large tortuous coronary fistula from the LMCA/LCX emptying into the SVC. Transthoracic echocardiogram showed a left ventricular (LV) ejection fraction of 56% and lateral LV wall hypokinesis. Cardiac MRI demonstrated an enlarged right ventricle without delayed myocardial enhancement. Given right sided enlargement and concerns for coronary steal, decision was made to intervene. The patient declined sternotomy to preserve his active lifestyle. Thus, a nonsurgical multi-disciplinary approach was planned for fistula closure. Vascular plug placement was attempted through retrograde venous and antegrade coronary wiring but was unsuccessful due to a narrow and fenestrated single fistula ostium opening and long and tortuous fistula length. From the coronary side, stasis was achieved in the fistula through embolization using 12 microcoils. An electrophysiology study was performed, and the patient’s arrhythmia was consistent with ventricular tachycardia. A subcutaneous implantable defibrillator was inserted for secondary prevention. Discussion: This patient’s uniquely late presentation and resulting unstable arrhythmia, cardiac chamber enlargement and ischemia emphasizes the importance of identifying indications for fistula closure. Pre-procedure planning with various imaging modalities and collaboration of multiple specialties allows for productive discussion of both surgical and interventional options. Conclusion: A multidisciplinary heart team is essential in successfully treating complex arteriovenous malformations.

The case of a patient in whom hemodynamic and electrocardiographic studies using the occlusion test for coronary artery fistulas (CAF) were safely performed prior to catheter embolization is reported. A 1-year-old girl had a separate right coronary artery arising from a left single coronary artery that formed a significant coronary artery fistula to the right ventricle. Coronary steal by the large coronary artery fistula narrowed the left coronary artery. The right coronary artery branches could not be clearly identified due to an overlap with the fistula. Due to the long porous CAF, embolic procedures could cause serious complications. We confirmed the safety by performing an occlusion test of the CAF's proximal blood vessels. Following total occlusion of the CAF for 10 minutes, pulmonary arterial pressure and aortic blood pressure were not significantly changed. No bradycardia, atrioventricular block, or ST changes were observed. Coil embolization treatment was performed safely. For patients with long distal CAF complicated with a single coronary artery, myocardial ischemia and conduction system disorders can be identified by performing the occlusion test before embolization.

Aortic Stenosis Severity: Rhythm Makes a Difference

IT'S NOT THE HEART