Percutaneous closure of a complex fistula that originates from all coronary arteries and drains to the pulmonary artery.

A coronary-to-pulmonary-artery fistula (CPAF) is defined as a connection between the coronary arteries and the pulmonary arteries (PAs). Invasive treatment options for CPAFs include surgical ligation and transcatheter coil or plug embolization. A 60-year-old female patient was referred to our hospital with symptoms of exercise-induced angina (Canadian Cardiovascular Society Class III [CCS-3]). Her relevant history included elective stenting of the left anterior descending (LAD) artery in 2013 and surgery for an LAD to PA fistula in 2015. Upon recurrence of the same fistula in 2017, she underwent a failed antegrade (from LAD to PA) attempt for percutaneous closure of the fistula. A retrograde approach was decided because of the extensive tortuosity of the fistula's proximal part that led to the previous failed attempt and the likelihood of ceasing whole blood flow at the fistula's distal neck. Through right femoral venous access, we advanced a 5 Fr. 45 cm TorqVue low-profile delivery system (St. Jude Medical, Little Canada, MN, USA) over a J-tip 0.035-inch guidewire to the PA. The antegrade imaging guidance enabled us to advance the guidewire to the distal neck of the fistula retrogradely. As the distal part of the fistula was similar to a tunnel-shaped patent ductus arteriosus (PDA) and was measured 4 mm at the narrowest diameter, we opted for an Amplatzer duct occluder II 6 × 6 (Abbott Vascular, Chicago, IL, USA) to close it.

Coronary artery fistula in adults: Incidence and appearance on cardiac computed tomography and comparison of detectability and hemodynamic effects with those on transthoracic echocardiography.

BACKGROUND: The widespread use of cardiac computed tomography (CT) has increased the incidental discovery of fistulas of the coronary artery (CAF). This condition is rare and can affect hemodynamic parameters, but few reports focus on its hemodynamic effects. We investigated the frequency and types of CAF on CT and compared them with those of transthoracic echocardiography (TTE) to evaluate the detectability of CAF and its hemodynamic effects. MATERIALS AND METHODS: We retrospectively evaluated cardiac CT images of 6789 adult patients who underwent imaging from January 1, 2013 through September 30, 2019 at our institution. We assessed the CT images for the presence of CAF and compared our findings with those obtained by TTE in control cases without CAF. RESULTS: The prevalence of CAF determined with cardiac CT was 0.91%, with the left anterior descending artery (67.7%) as the most common site of origin and the main pulmonary artery (82.3%) as the most common origin of drainage. The incidence of aneurysm accompanying CAF was 48.4%. Color Doppler in TTE demonstrated abnormal flow that would suggest the presence of CAF of only 23.1%. Echocardiographic findings of hemodynamics did not differ significantly between patients with and without CAF nor between elderly and non-elderly patients with CAF. CONCLUSIONS: Our study revealed differing prevalence and types of CAF from those reported using coronary angiography and little impact of CAF on hemodynamics. Color Doppler in TTE did not readily depict abnormal flow that might suggest the presence of a fistula, but cardiac CT allowed noninvasive and comprehensive assessment of CAF. Thus, we believe the acquisition of cardiac CT is necessary to establish the cause of continuous murmur that is not identified with TTE.

The role of the fractional flow reserve in the coronary steal phenomenon evaluation caused by the coronary-pulmonary fistulas: case report and review of the literature.

BACKGROUND: Congenital coronary-pulmonary fistulas (CPFs) are commonly unilateral; however, bilateral and multilateral fistulas are relatively rare. The steal phenomenon aroused from bilateral or multilateral CPFs, and was uncertain and seldom reported. We possess a new tool to assess the hemodynamic significance of coronary artery fistulas. This study aimed to describe the clinical presentation, diagnostic modalities, and management of the coincidentally detected congenital bilateral CPFs. CASE PRESENTATION: A case of a 52 year-old female with 10 years history of typical palpitations and chest tightness was presented. The selective coronary arteriography showed a right dominant coronary circulation without significant stenosis; however, with anomalous vessels originating from the proximal right and left anterior descending coronary arteries, draining into the pulmonary artery through a plexus of small vessels. We introduced the fractional flow reserve (FFR) to evaluate the hemodynamic significance of CPFs. The patient was successfully treated with coil embolization. CONCLUSIONS: We presented the case of a female with typical palpitations and chest tightness due to the steal phenomenon that aroused from bilateral CPFs. The fistulas were safely and successfully closed by coil embolization. We showed a new tool for the sophisticated evaluation of the hemodynamic significance of CPFs using FFR measurement and temporary occlusion of the fistula with a standard balloon. FFR could be a promising means for the treatment of decision making of the CPFs.

Altered Renal Vascular Responsiveness to Vasoactive Agents in Rats with Angiotensin II-Dependent Hypertension and Congestive Heart Failure.

OBJECTIVE: We evaluated the hypothesis that the development of renal dysfunction and congestive heart failure (CHF) caused by volume overload in rats with angiotensin II (ANG II)-dependent hypertension is associated with altered renal vascular responsiveness to ANG II and to epoxyeicosatrienoic acids (EETs). METHODS: Ren-2 transgenic rats (TGRs) were used as a model of ANG II-dependent hypertension. CHF was induced by volume overload achieved by the creation of the aorto-caval fistula (ACF). Renal blood flow (RBF) responses were determined to renal arterial administration of ANG II, native 11,12-EET, an analog of 14,15-EETs (EET-A), norepinephrine (NE), acetylcholine (Ach) and bradykinin (Bk) in healthy (i.e., sham-operated) TGR and ACF TGR (5 weeks after ACF creation). RESULTS: Selective intrarenal administration of neither vasoactive drug altered mean arterial pressure in any group. Administration of ANG II caused greater decreases in RBF in ACF TGR than in sham-operated TGR, whereas after administration of NE the respective decreases were comparable in the 2 groups. Administration of Ach and Bk elicited significantly higher RBF increases in ACF TGR as compared with sham-operated TGR. In contrast, administration of 11,12-EET and EET-A caused significantly smaller RBF increases in ACF TGR than in sham-operated TGR. CONCLUSION: The findings show that 5 weeks after creation of ACF, the TGR exhibit exaggerated renal vasoconstrictor responses to ANG II and reduced renal vasodilatory responses to EETs, suggesting that both these alterations might play an important role in the development of renal dysfunction in this model of CHF.

Exercise-induced ventricular arrhythmia due to bilateral coronary to pulmonary artery fistulas.

Ischemic polymorphic ventricular ectopy was documented during exercise testing in a 65-year-old Caucasian male patient. Coronary angiogram revealed four coronary to pulmonary artery fistulas (CPAFs) originating from the right and left coronary artery, leading to myocardial ischemia due to steal phenomenon. The three dominant fistulas were coiled percutaneously, while one small fistula was left untreated. During follow-up, no significant residual ventricular arrhythmia was detected.

The evaluation of coronary artery-to-pulmonary artery fistula in adulthood on 256-slice CT coronary angiography: Comparison with coronary catheter angiography and transthoracic echocardiography.

OBJECTIVE: To review the imaging features of coronary artery-to-pulmonary artery fistula (CPAF) on CT coronary angiography (CTCA) and evaluate its diagnostic performance compared with coronary catheter angiography (CCA) and transthoracic echocardiography (TTE). MATERIALS AND METHODS: We retrospectively reviewed with a diagnosis of CPAF from among 19855 consecutive CCTA performed with 256-slice MDCT scanner for suspected coronary artery disease. CT images were evaluated for - origin, number, size and course (tubular/worm-like dilation/significant aneurysm formation/wall attachment sign) of fistula vessels, drainage site, drainage site imaging features (pierced sign, isodensity sign, smoke sign, jet sign), and main pulmonary artery (MPA) enlargement. 25 patients of CPAF also underwent CCA and 47 patients underwent TTE. RESULTS: There were 72 patients with CPAF (0.36%) in our study, of which 44 were men and 28 were women, with mean age of 55.8 ±â€¯13.2 years (range 22-85 years). CPAF originated from conus artery, left anterior descending artery (LAD), combined conus artery and LAD in 55, 67, 50 cases, respectively. Tubular dilation, worm-like dilation and aneurysm was seen in 14, 58 and 35 cases, respectively. Wall attachment sign was noted in 69 cases. All the cases demonstrated only a single drainage site, with left lateral wall, left anterolateral, anterior, right lateral and right anterolateral walls of MPA in 44, 21, 5, 1 and 1 cases, respectively. The mean diameter of the fistula drainage site was 2.6 ±â€¯1.3 mm. Pierced sign, jet sign, smoke sign, isodensity sign was seen in 72, 46, 41 and 24 cases, respectively. MPA enlargement was seen in 20 patients. CCA showed CPAF in only 20 cases among 25 patients; while TTE showed CPAF in only 9 patients among 47 patients. CONCLUSION: CTCA is competent in detecting and characterizing CPAF with an excellent diagnostic performance as the first imaging modality of choice, which is valuable for giving a distinct and intuitive explanation to patients and physicians and making an objective and exact assessment for further management.

Endovascular Embolization of Coronary Artery-Pulmonary Artery Fistulas with Double Coronary Aneurysms.

A 75-year-old woman with chest discomfort and a continuous murmur was admitted to our hospital. During noninvasive examination, computed tomography angiography showed a coronary artery-pulmonary artery fistula with double giant coronary aneurysms (one was 42 mm× 32 mm× 32 mm, and the other was 25 mm× 20 mm× 17 mm) arising from the proximal part of the left anterior descending (LAD) artery. Stress myocardial scintigraphy showed ischemia at the LAD area. Given her frailty, the heart team, including cardiac surgeons, judged that surgical treatment would be difficult. Thus, endovascular embolization for the abnormal vessels was selected. After coronary angiography, two coronary aneurysms were embolized by 53 coils, and the feeding artery was embolized by two coils and one Amplatzer Vascular Plug 4™. A small pulmonary artery fistula remained after the procedures; thus, additional embolization was performed 3 months after the index procedure. Thereafter, angiography showed no flow into the aneurysms, and her symptoms improved.Endovascular embolization might be an effective treatment to achieve aneurysm occlusion in patients at high risk for surgical treatment. Although the present case had double coronary aneurysms with a large feeder vessel, the combination procedure of coils and vascular plug was able to embolize this abnormal vessel.

Coronary Artery Fistula.

Coronary artery fistulae are rare abnormalities of the coronary arteries that are usually discovered incidentally on coronary angiogram. Most patients remain asymptomatic, and significant hemodynamic consequences are rarely observed. The following report describes the case of a patient presenting with symptoms of congestive cardiac failure due to multiple coronary artery fistulae.

Functional Improvement and Regression of Medial Hypertrophy in the Remodeled Pulmonary Artery after Correction of Systemic Left-to-Right Shunt.

The presence of systemic left-to-right shunt and increased pulmonary blood flow can result in right heart failure and pulmonary arteriopathy. Correction of left-to-right shunt has been shown to improve cardiac function and physical performance. However, the cardiopulmonary remodeling processes following cessation of left-to-right shunt have yet to be reported. In this experimental study, excessive pulmonary flow was restored through ligation of the aortocaval fistula in rats with flow-induced pulmonary hypertension. The cardiopulmonary morphometric functions were assessed, and phenotypic switching of pulmonary vascular smooth muscle cells (VSMC) was determined. Ligation of aortocaval fistula significantly attenuated pulmonary blood flow and right ventricular mass, and potentiated the isometric contraction of pulmonary artery. Inflammatory cytokines IL-1ß and IL-6 were reduced in the lung after ligation. Reduction of pulmonary blood flow restored the expressions of smooth muscle myosin heavy chain and α-smooth muscle actin in pulmonary artery, indicating the switching of VSMCs to the contractile phenotype. Our study demonstrated that normalization of pulmonary blood flow in flow-induced pulmonary hypertension reverses the remodeling in the right ventricle and pulmonary artery. The remodeling process of flow-induced pulmonary hypertension is functionally and morphometrically reversible by inducing transdifferentiation of pulmonary VSMC to contractile phenotypes and modulation of tissue inflammatory cytokines.

Friesian horses as a possible model for human acquired aortopulmonary fistulation.

BACKGROUND: Acquired aortopulmonary fistulation is a rare condition in humans. It usually results as a late complication of a true or pseudoaneurysm of the thoracic aorta. It is most commonly associated with trauma or surgery, less commonly with atherosclerosis, inflammation, hypertension or Marfan's syndrome. Aortopulmonary fistulation is also seen as a rare complication of acute aortic dissection. On rare occasions, acquired aortopulmonary fistulation is reported in aged patients without any of the above mentioned triggering factors. Thus, these cases should be considered as idiopathic aortopulmonary fistulation. Clearly, the pathogenesis of this condition is not yet completely understood. Friesian horses are highly inbred and are affected by several genetic conditions. Rupture of the thoracic aorta has a relatively high prevalence in Friesian horses and is often characterized by the formation of a pseudoaneurysm with subsequent fistulation into the pulmonary artery. Affected animals may survive for several weeks to months. FINDINGS: Here we performed vascular casting in three affected Friesian horses. In all three cases, an aortic rupture at the caudoventral side of the aorta was connected with a rupture of the main pulmonary artery just proximal to its bifurcation. CONCLUSIONS: Affected Friesians show a consistent location and configuration of the aortic rupture site, very similar to the human condition and therefore could act as a spontaneous model to study this disease.

Novel Technique to Evaluate Pulmonary Vascular Resistance in a Patient With Aortopulmonary Window.

We report a case of a 15-month-old female presenting with a large aortopulmonary window during a medical mission. The optimal surgical correction for this patient would be based on an estimation of her pulmonary vascular reactivity. This report details the use of antegrade/retrograde flow ratios using Doppler measurements in the proximal descending aorta at baseline and while receiving 100% oxygen. A large increase in the proportion of retrograde flow under the 100% oxygen condition suggested a reactive pulmonary vascular bed. She underwent a complete repair with patch closure of the aortopulmonary window. A postoperative echocardiographic assessment showed near normal right ventricular pressures.

Fractional flow reserve-guided percutaneous coronary intervention for an intermediate stenosis complicated by a coronary-to-pulmonary artery fistula.

A 65-year-old man was referred to our hospital following repetitive chest pain. Invasive coronary angiography showed an intermediate stenosis of the proximal left anterior descending artery (LAD), and a coronary fistula originating distal to the stenosis draining into the main pulmonary artery. To evaluate the functional abnormality arising from the stenosis and coronary steal due to the fistula, fractional flow reserve (FFR) was measured using a pressure wire with pullback recording. The FFR value was 0.74 at the distal LAD, 0.78 distal to the fistula, 0.81 proximal to the fistula (distal to the stenosis), and abruptly increased to 1.0 proximal to the stenosis. Based on these FFR results, percutaneous coronary intervention was performed to the stenosis. After stent placement, the FFR value improved to 0.87 at the distal LAD, and no abrupt pressure gradient was observed beyond the fistula and the stent. FFR-guided intervention with pullback pressure recording could be a useful and practical method to apply in cases with coronary stenosis complicated by coronary fistula in the same vessel.

Coronary artery fistula between single right coronary artery and right pulmonary artery: a case report and literature review.

BACKGROUND: Coronary artery fistula and single coronary artery are two different rare congenital anomalies. The cases with co-existed the two anomalies are more rare. To the best of our knowledge with literature review, the coronary artery fistula between single right coronary artery and right pulmonary artery has not been previously reported. CASE PRESENTATION: In the present article, we report a Chinese patient (a 8-month-old male) who presented cyanosis when cried and heart murmur. The cardiac angiography confirmed coronary artery fistula between single coronary artery arising from the right aortic sinus and right pulmonary artery. Furthermore, the right pulmonary artery was interrupted with main pulmonary artery and the pulmonary blood supplied by single right coronary artery. Following the surgical procedure, the anomalous fistula vessel was cut and sutured. The right pulmonary artery was reconstructed to connect with main pulmonary artery. The patient had an uneventful postoperative course and discharged. Then we reviewed the related literature with Medline and Pubmed databases for further details. CONCLUSION: We believe our patient is the very particular case about coronary artery fistula combined with single coronary artery, and it is first reported with our literature review. As other coronary anomalies, coronary or aortic root angiography is the gold standard method for the diagnosis. Furthermore, early surgery is an optimal treatment in our case.