Transcatheter retrieval of atrial septal defect and patent ductus arteriosus occluder: a guidance for device retrieval based on comprehensive bench tests.

OBJECTIVES: The aim of this study is to establish a guidance for device retrieval based on comprehensive bench tests. BACKGROUND: Device embolisation remains a major complication in transcatheter closure of atrial septal defect and patent ductus arteriosus. Although percutaneous retrieval is feasible in the majority of cases, surgical retrieval may be required in complicated circumstances. However, the methods of transcatheter device retrieval have not been completely established. METHODS: Bench tests of device retrieval were performed to verify the appropriate retrieval method according to device type/size. The devices used for testing were Amplatzer Septal Occluder (Abbott, Chicago, IL, United States of America), Figulla Flex II (Occlutech GmbH, Jena, Germany), Amplatzer Duct Occluder-I (Abbott), Amplatzer Duct Occluder-II (Abbott), and Amplatzer Vascular Plug-II (Abbott). The retrieval equipment constituted diagnostic catheters (multipurpose catheter and right Judkins catheter, 4-Fr or 5-Fr, Gadelius Medical, Tokyo, Japan), delivery sheath and cables for each device, Amplatz goose neck snares (Medtronic, Minneapolis, MN, United States of America), OSYPKA CATCHER (Osypka ag, Rheinfelden-Herten, Germany), and OSYPKA LASSOS (Osypka). We investigated the retrieval equipment and sheath sizes required for a successful retrieval procedure for variously sized devices. RESULTS: For patent ductus arteriosus devices, the type of snare and the snaring position are considered important. For atrial septal defect devices, simple snare capture or a double-snare technique with a sufficiently large sheath is effective. Special care should be taken when using the OSYPKA CATCHER for device retrieval. CONCLUSIONS: The results of this study may assist in the selection of both capture devices and a retrieval sheath or a catheter for complete retrieval.

Static balloon atrial septostomy in Japan in shortage of standard balloon septostomy catheter.

BACKGROUND: The supply of Rashkind balloon atrial septostomy (BAS) catheters (Medtronic, Minneapolis, MN, USA) has suddenly been discontinued in the world due to its recall. Consequently, Japan fell into a critical shortage of standard BAS catheters. Although the use of static BAS is off-label in Japan, its importance is increasing in such a situation. A nationwide survey of static BAS is needed in such a critical period. METHODS: A questionnaire survey was conducted among centers in Japan regarding BAS performed between October 1, 2020, and December 15, 2020, while the supply of Rashkind BAS catheter was discontinued. RESULTS: We received answers from 70 of the 90 centers, for a response rate of 78%. In this survey, 25 patients who underwent static BAS were enrolled, and a total of 47 BAS procedures were performed. Median age and weight at static BAS were 10 days of life and 3001 g, respectively. The most common diagnosis was transposition of the great arteries without pulmonary stenosis and hypoplastic left heart syndrome and its variants, with 8 cases each. The most frequently used balloon diameter was 10 mm (13 balloons), followed by 12 mm (10 balloons), and 3 cases required double-balloon techniques. The 3-point scale of the efficacy of static BAS rated by physicians were 10 excellent, 15 good, and 0 poor, respectively. Complications included cardiac tamponade during the procedure in 1 patient and the need for Rashkind BAS later in 2 patients. Comparing the share of static BAS in all transcatheter atrial septostomy, its share in the current survey (28/53) is significantly higher compared to the annual registry data in 2018 (86/304) (p < 0.01). CONCLUSIONS: This survey shows that static BAS is widely performed in Japan and is effective and safe. Static BAS cases have increased significantly due to a shortage of standard BAS catheters.

Diagnosing Atrial Septal Defect from Electrocardiogram with Deep Learning.

The heart murmur associated with atrial septal defects is often faint and can thus only be detected by chance. Although electrocardiogram examination can prompt diagnoses, identification of specific findings remains a major challenge. We demonstrate improved diagnostic accuracy realized by incorporating a proposed deep learning model, comprising a convolutional neural network (CNN) and long short-term memory (LSTM), with electrocardiograms. This retrospective observational study included 1192 electrocardiograms of 728 participants from January 1, 2000, to December 31, 2017, at Tokyo Women's Medical University Hospital. Using echocardiography, we confirmed the status of healthy subjects-no structural heart disease-and the diagnosis of atrial septal defects in patients. We used a deep learning model comprising a CNN and LTSMs. All pediatric cardiologists (n = 12) were blinded to patient groupings when analyzing them by electrocardiogram. Using electrocardiograms, the model's diagnostic ability was compared with that of pediatric cardiologists. We assessed 1192 electrocardiograms (828 normally structured hearts and 364 atrial septal defects) pertaining to 792 participants. The deep learning model results revealed that the accuracy, sensitivity, specificity, positive predictive value, and F1 score were 0.89, 0.76, 0.96, 0.88, and 0.81, respectively. The pediatric cardiologists (n = 12) achieved means of accuracy, sensitivity, specificity, positive predictive value, and F1 score of 0.58 ± 0.06, 0.53 ± 0.04, 0.67 ± 0.10, 0.69 ± 0.18, and 0.58 ± 0.06, respectively. The proposed method is a superior alternative to accurately diagnose atrial septal defects.

Detachable coil embolisation for iatrogenic femoral arteriovenous fistula in patients with CHDs.

We present two cases of patients with iatrogenic femoral arteriovenous fistula who underwent successful embolisation using three-dimensional shape detachable coils. A 49-year-old male with Tetralogy of Fallot had arteriovenous fistula which developed from the common femoral artery to the femoral vein with an aneurysm and a 17-year-old female with single ventricle after total-cavo-pulmonary-connection had two arteriovenous fistulas which developed from the internal iliac artery to the femoral vein. A total of six and seven pieces of detachable coils were necessary for complete occlusion, respectively. No complications were recorded. The advantage of the detachable coil is a wide variation and repositioning until the coil achieves good stabilisation and an ideal configuration.

A novel procedure of puncture to access the pulmonary venous atrium in complex congenital heart disease with surgical correction.

BACKGROUND: In complex congenital heart disease, supraventricular arrhythmia develops long term after surgical repair. The arrhythmia could have pharmacological tolerance and sometimes be fatal. We report our experiences with puncturing tough or calcified surgical repair material to access the pulmonary venous atrium for the purpose of the management of arrhythmia in complex congenital heart disease with surgical correction. METHODS: From June 2016, subsequent 9 patients underwent the procedure. Their age at the procedure ranged from 11 to 43 years old (median 26.4 years old). Surgical procedures were Mustard procedure (XenomedicaⓇ) in 6, lateral tunnel total cavo-pulmonary connection with autologous pericardium in 2, and extra-cardiac total cavo-pulmonary connection with expanded polytetrafluoroethylene (ePTFE) tube in 1. RESULTS: Multi-planar reconstruction imaging by cardiac computed tomography was done for making plans before the procedures. Under intracardiac echocardiography guidance, an 8 French steerable sheath was perpendicularly adjusted to target objects. The sharpened edge of 0.014 inch guide wire penetrated with gentle forward force. Subsequently, the puncture site was dilated from a small balloon to a large one step by step. In all patients, penetration was successfully completed. Catheter ablation was achieved in 8 patients and a pacemaker was implanted through the re-canalized superior caval vein in 1 patient. No complications were recorded. CONCLUSION: The trans-catheter puncture of the synthetic or calcified material is safe and feasible, although careful planning is required with imaging. A steerable sheath could easily be controlled to the make appropriate angle to the target object for preventing slippage and conveying effective force.

Mid- to long-term surgical outcomes of partial anomalous pulmonary venous connection.

OBJECTIVE: To investigate mid- to long-term morbidity and mortality after the repair of partial anomalous pulmonary venous connections (PAPVCs). METHODS: We retrospectively analyzed the cases of 29 consecutive patients who underwent surgery in 1991-2010 at Tokyo Women's Medical University Hospital. Median follow-up period: 9.9 years. Median age at operation: 7.4 years. Median body weight: 22.4 kg. Right PAPVC (n = 23), left-PAPVC (n = 5), and mixed PAPVC (n = 1) were identified. RESULTS: In the 14 patients with a right upper pulmonary vein (PV) connected to the superior vena cava (SVC) and/or cavo-atrial junction, the following were performed: single-patch diversion (n = 6), double-patch diversion (n = 4), Warden procedure (n = 1), and atrial septation with the atrial wall (n = 3). In the nine patients with a right anomalous PV connected to the right atrium (RA) and/or coronary sinus, intra-atrial rerouting was performed (n = 7) and translocation of the atrial septum (n = 2). Intra-atrial rerouting of the left upper and lower PVs to the coronary sinus (n = 1) and direct anastomosis of the left upper PV to the left atrial appendage (n = 4) were performed. Double-patch diversion and direct anastomosis of the left upper PV to the left atrial appendage was performed in a patient with mixed PAPVC. There were no post-operative deaths or reoperations, although transient sinus node dysfunction occurred (n = 2 with atrial septation and intra-atrial rerouting), while mild stenosis of the SVC occurred (n = 1 with double-patch diversion). CONCLUSIONS: The various methods which we used for PAPVC repair all appear to have reasonable outcomes, although rhythm disturbance and stenotic complications were confirmed in a few patients.

Clinical trial of the CP stent for pulmonary artery stenosis: the first investigator-initiated clinical trial for pediatric interventional cardiology in Japan.

Stenting is an important treatment option for pulmonary artery stenosis (PS) associated with congenital heart disease (CHD). However, no stent has been approved for this indication in Japan, despite negotiation between academia and the regulatory bodies for longer than 20 years. To evaluate efficacy and safety of the CP stent, we performed the first investigator-initiated clinical trial for pediatric interventional cardiology in Japan. This trial was designed as a single-arm, prospective, clinical trial. Patients who had postoperative PS associated with CHD were included. Stenting was attempted in 24 cases and succeeded in 22 cases. The median age of the patients was 11 years (3-36 years) and weight was 38 kg (12-69 kg), while follow-up for 12 months was completed. In all 22 cases, stenting was successful, with a 50% increase in the minimum lumen diameter (MLD) in 86.4% of patients (90% confidence interval, 68.4-96.2%). The mean percent change in MLD was 119.3 ± 52.5%. In two-ventricle repair, the mean percent change in systolic right ventricular/aortic pressure was - 8.5 ± 16.1%, while that of pressure gradient was - 55.9 ± 41.7%. In single-ventricle repair, the percent change in the mean pressure gradient was - 100.0 ± 0%, while that of SaO2 was 1.4 ± 1.7%. No serious adverse events or significant restenosis was reported. The CP stent is highly effective and safe for PS associated with CHD. This study has significant importance in not only scientific but also social considerations.

Efficacy of catheter ablation for patients with atrial fibrillation and atrial septal defect.

INTRODUCTION: Given that few studies investigated the efficacy of catheter ablation (CA) in patients with paroxysmal atrial fibrillation (AF) and atrial septal defect (ASD), this study evaluated its effectiveness in patients with paroxysmal AF and ASD. METHODS AND RESULTS: Of the 216 patients who underwent ASD device closure at two hospitals, 36 patients had paroxysmal AF. After April 2012, CA for AF was performed before ASD device closure (ASD-CA group; n = 20). The ASD-CA group had a significantly higher AF-free survival rate after ASD device closure compared to patients without CA for AF before ASD device closure (ASD-non-CA group; n = 16) (ASD-CA group: 2 patients vs. ASD-non-CA group: 9 patients; follow-up period: 4.2 ± 2.5 years; log-rank p = .01). In addition, the AF-free survival rates were similar between the ASD-CA group and 80 paroxysmal AF patients who underwent CA without any detectable structural heart disease (non-SHD-CA group). The two groups were matched by propensity scores for age, sex, and left atrium dimension (ASD-CA group: 2 patients vs. non-SHD-CA group: 5 patients; follow-up period: 3.3 ± 1.8 years; log-rank p = .28). CONCLUSION: CA for AF before ASD device closure might be an effective treatment option for patients with paroxysmal AF and ASD.

Skeletal muscle index determined by bioelectrical impedance analysis is a determinant of exercise capacity and a prognostic predictor in patients with congenital heart disease.

BACKGROUND: Patients with congenital heart disease (CHD) reportedly have reduced exercise capacity. Underlying cardiac anatomy and a sedentary lifestyle are thought to be associated with exercise impairment. However, little has been reported regarding the relationship between quantitative body composition and exercise capacity. Bioelectrical impedance analysis (BIA) is a rapid and safe assessment method that has been widely used in clinical research. We hypothesized that bioelectrical impedance parameters are determinants of exercise capacity, and the skeletal muscle index (SMI) determined by BIA is a prognostic predictor in patients with CHD. METHODS: We conducted a retrospective single-center study of 305 consecutive patients with CHD [median age, 26 years (range 12-60]; 48% males] admitted between 2014 and 2017. The BIA parameters were reviewed, including the edema index (EI, extracellular water to total body water ratio), SMI (skeletal muscle mass/height2), mineral index (MI, mineral mass/height2), percent body fat (%BF), and exercise capacity [peak oxygen uptake (peakVO2)] via a cardiopulmonary exercise test. RESULTS: The multivariate analysis revealed a significant correlation between peakVO2 and EI (r=0.55) and peakVO2 and SMI (r=0.49). The receiver operating characteristic curve analysis showed that the EI cut-off for peakVO2<20ml/kg/min was 0.386 [area under the curve (AUC), 0.77; sensitivity, 0.67; specificity 0.76], and the SMI cut-off was 7.6kg/m2 (AUC, 0.78; sensitivity, 0.76; specificity 0.75). Compared with patients who had biventricular morphology, patients with single ventricular morphology had a higher EI (mean, 0.381 vs. 0.387, respectively) and lower SMI (8.5 vs. 7.7, respectively), resulting in a lower peakVO2 (27.1 vs. 20.8, respectively). The Kaplan-Meier analysis showed that a low SMI was associated with an increased risk of future heart failure-related admissions. CONCLUSIONS: SMI determined by BIA is a determinant of exercise capacity and can be used as a prognostic predictor in patients with CHD.

Silibinin Upregulates CXCR4 Expression in Cultured Bone Marrow Cells (BMCs) Especially in Pulmonary Arterial Hypertension Rat Model.

Previously we reported that silibinin ameliorated pulmonary arterial hypertension (PAH) in rat PAH models, possibly through the suppression of the CXCR4/SDF-1, until the point where PAH became a severe and irreversible condition. To further investigate how silibinin ameliorates PAH, we first attempted to clarify its effect on bone marrow cells (BMCs), since the CXCR4/SDF-1 axis is known to regulate stem cell migration and attachment in BM niches. Rat PAH models were established through a combination of a single subcutaneous injection of monocrotaline (MCT) and chronic hypoxic conditions (10% O2). BMCs were harvested and cultured, and reverse transcription-quantitative polymerase chain reaction (RT-qPCR) and flow cytometry (FCM) were performed to investigate whether silibinin affected CXCR4 expression. Silibinin upregulated the gene expression of stem cell related markers CXCR4, SDF-1, SCF, and c-Kit, inflammatory markers IL-6 and TNFα, mesenchymal stem cell (MSC)-related markers CD44 and CD29, and the granulocyte/monocyte-macrophage marker CD14 in cultured BM in PAH rats, but not in normal rats, except CXCR4. FCM showed that silibinin increased the CXCR4-positive cell population in a granulocyte fraction of cultured BMCs. However, immunohistochemical (IHC) staining showed no significant change in CXCR4 expression in the BM of the tibias. These results suggest that silibinin increases the expression of CXCR4 in BM, and the increased CXCR4-positive cells could be granulocytes/monocyte-macrophages.

Partnership Between Japan and the United States for Early Development of Pediatric Medical Devices　- Harmonization By Doing for Children.

BACKGROUND: The Harmonization By Doing (HBD) program was established in 2003 as a partnership among stakeholders of academia, industry and regulatory agencies in Japan and the United States, with a primary focus on streamlining processes of global medical device development for cardiovascular medical devices. While HBD has traditionally focused on development of devices intended to treat conditions prevalent in adults, in 2016, HBD established the "HBD-for-Children" program, which focuses on the development of pediatric devices as the development of medical devices for pediatric use lags behind that of medical devices for adults in both countries.Methods and Results:Activities of the program have included: (1) conducting a survey with industry to better understand the challenges that constrain the development of pediatric medical devices; (2) categorizing pediatric medical devices into five categories based on global availability and exploring concrete solutions for the early application and regulatory approval in both geographies; and (3) facilitating global clinical trials of pediatric medical devices in both countries. CONCLUSIONS: The establishment of the HBD-for-Children program is significant because it represents a global initiative for the introduction of pediatric medical devices for patients in a timely manner. Through the program, academia, industry and regulatory agencies can work together to facilitate innovative pediatric device development from a multi-stakeholder perspective. This activity could also encourage industry partners to pursue the development of pediatric medical devices.

Characteristics of hepatocellular carcinoma arising from Fontan-associated liver disease.

AIM: Hepatocellular carcinoma (HCC) can arise from Fontan-associated liver disease (FALD); this is known as FALD-HCC. The clinical features of FALD-HCC are unclear. Thus, we examined the incidence and clinical characteristics of FALD-HCC. METHODS: From 1972 to 2019, 122 patients developed liver disease after undergoing Fontan procedures. HCC was diagnosed in 12 (9.8%) FALD patients. We compared FALD-HCC and non-HCC patients. RESULTS: The incidence of HCC was 0.8% and 2.9% in FALD 10 and 20 years after the Fontan procedure, respectively. The median age of patients at diagnosis of HCC was 32.5 years (range 20.6-46.1 years), and seven of the 12 patients were men. Patients with FALD-HCC had a higher incidence of liver cirrhosis and polysplenia than non-HCC patients. Liver tumors were detected as single nodules in eight patients, and the median diameter was 47 mm (range 11-105 mm). HCC was treated by surgical resection in two patients, transcatheter arterial chemoembolization or chemotherapy in three patients, and proton beam therapy in four patients. Three patients could not be treated because of their poor condition. Four patients died of liver/cardiac failure and HCC, and HCC was controlled in three patients. The survival rate after 25 years was significantly lower in patients with FALD-HCC than non-HCC patients (68.6% vs. 97.9%, respectively; P < 0.01). CONCLUSIONS: Of the 122 patients with FALD, 12 developed HCC 20 years after surgery. Because complications of HCC are associated with poor prognosis, constant surveillance for HCC should begin 10 years after surgery.

Risk Factors and Predictors of Cardiac Erosion Discovered from 12 Japanese Patients Who Developed Erosion After Atrial Septal Defect Closure Using Amplatzer Septal Occluder.

Cardiac erosion is a rare serious complication following Amplatzer septal occluder (ASO) placement for atrial septal defect. Although multiple risk factors have been found, a useful predictor to prevent it has not been established yet. In 12 patients who developed erosion between 2005 and 2016 in Japan, we retrospectively observed patients' characteristics and transesophageal echocardiography findings immediately before and after ASO placement and at erosion onset. We compared risk factors of erosion, including absent aortic rim, device size/body weight ratio, device shape, or Valsalva sinus wall deformation pressed by either disk-edge, the maximum depth of which from the standard curve of the Valsalva wall was defined as Dent, between the 12 patients and 95 patients with Valsalva sinus wall deformation immediately after placement who did not develop erosion for 5 ± 3 years. Of the 12 patients, nine developed pericardial effusion with eight cardiac tamponade and three aorta-atrium fistula; all were surgically rescued. Surgical findings revealed that erosion in all patients occurred at the right and/or left atrial roof beside the Valsalva in the non-coronary cusp on which the disk-edge seemed to be pressing. The mean Dent immediately after the placement in patients with erosion was significantly deeper than without (2.48 ± 0.32 vs. 1.28 ± 0.38; p < 0.001). There were no differences in the other risk factors between the two groups. Dent is believed to be a useful indicator of erosion development after ASO placement. If Dent is > 2.0 mm, it is desirable to change the size or to replace the device.

Clinical Significance of Central Venous Pressure During Exercise After Fontan Procedure.

Various late complications are associated with the Fontan procedure. In patients who undergo the Fontan procedure, the central venous pressure (CVP) tends to be higher than normal. However, the relationship between CVP (at rest and during exercise) and late complications associated with the Fontan procedure is unknown. Thirty-four patients who underwent the Fontan procedure were enrolled in this study. The median age was 19.3 years, and the median time after the Fontan procedure was 12.7 years. With exercise, the CVP increased from a median of 11 to 19 mmHg, and the cardiac index increased from a median of 2.1 to 4.4 l/min/m2. In 38% of the patients, CVP measured at the resting condition and during exercise differed. Laboratory results indicated no significant difference between the patients in the high-CVP group and low-CVP group at rest. In contrast, during exercise, brain natriuretic peptide (67 ± 38 vs. 147 ± 122 pg/ml, p < 0.05), gamma-glutamyl transpeptidase (63 ± 33 vs. 114 ± 30 IU/l, p < 0.05), type IV collagen 7S (6.7 ± 1.3 vs. 8.1 ± 1.3 ng/ml, p < 0.05), and creatinine (0.72 ± 3.14 vs. 0.83 ± 3.16 mg/dl, p < 0.05) levels were significantly higher in the high-CVP group than in the low-CVP group. Elevated CVP during exercise may be associated with long-term complications after the Fontan procedure; hence, CVP should be measured during exercise in patients who underwent the Fontan procedure to accurately predict the risk of developing such complications.

Successful staged tricuspid valve replacement following cardiac resynchronization therapy in a congenitally corrected transposition of the great arteries.

Simple tricuspid valve surgery for complex heart disease with systemic right ventricular dysfunction is a high-risk procedure; however, staged tricuspid valve surgery following cardiac resynchronization therapy seems to be a beneficial method to expect reverse systemic ventricular remodeling.

Risk factors of non-sustained ventricular tachycardia by technetium-perfusion imaging in patients with coronary artery lesions caused by Kawasaki disease.

BACKGROUND: Sudden death can occur in some patients with non-sustained ventricular tachycardia (NSVT) after myocardial infarction (MI) in those with coronary artery lesions (CAL) caused by Kawasaki disease (KD). The aim of this study was to determine the risk factors for NSVT in the late period after KD by technetium-99m-tetrofosmin myocardial perfusion imaging (MPI). METHODS: We retrospectively analyzed the relation between the appearance of NSVT and the findings in MPI single-photon-emission computed tomography (SPECT) in 75 patients (55 males and 20 females) who had had CAL caused by KD. All the patients had undergone MPI and 24-h Holter electrocardiogram at the same time between 2003 and 2012. The age at MPI ranged from 2 to 44 years (median 19 years), and the time from the onset of KD to MPI ranged from 1 to 44 years (median 18 years). We evaluated extent score (ES), summed rest score (SRS), summed stress score (SSS), summed difference score (SDS), and left ventricular ejection fraction (LVEF) by quantitative gated SPECT software. We analyzed which factors related to NSVT using multivariate logistic regression. Further, we calculated the cut-off point for NSVT using receiver operating characteristic curve. RESULTS: The affecting factors were ES (OR, 0.63, 95%CI, 0.35-0.92, p=0.013) and the interval from the onset of KD to MPI (OR, 0.82, 95%CI, 0.69-0.96, p=0.004). The cut-off points for ES and the interval from the onset of KD were 11% (AUC, 0.931, p<0.001) and 18 years (AUC, 0.732, p=0.007), respectively. CONCLUSIONS: ES is the strongest parameter for predicting NSVT in the late period. In patients with post-KD, adolescence and young adults with ES ≥11% are at risk of fatal ventricular arrhythmia.

Bioelectrical impedance analysis in the management of heart failure in adult patients with congenital heart disease.

OBJECTIVE: The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD. DESIGN: This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015. We evaluated BIA parameters (intra- and extracellular water, protein, and mineral levels, edema index [EI, extracellular water-to-total body water ratio]), laboratory values, and HF-related admission prevalence. RESULTS: Patients with New York Heart Association (NYHA) functional classes III-IV had a higher EI than those with NYHA classes I-II (mean ± SD, 0.398 ± 0.011 vs 0.384 ± 0.017, P < .001). EI was significantly correlated with brain natriuretic peptide level (r = 0.51, P < .001). During the mean follow-up period of 7.1 months, Kaplan-Meier analysis showed that a discharge EI > 0.386, the median value in the present study, was significantly associated with a future increased risk of HF-related admission (HR = 4.15, 95% CI = 1.70－11.58, P < .001). A body weight reduction during hospitalization was also related to EI reduction. CONCLUSIONS: EI determined using BIA could be a useful marker for HF severity that could predict future HF-related admissions in adult patients with CHD.

Dual origin multiple plexus-like coronary to pulmonary artery fistulas - Consideration of their etiology and therapeutic strategy.

BACKGROUND: Multiple plexus-like coronary to pulmonary fistulas (CAPFs) originating from bilateral coronary arteries are a rare type of coronary artery fistula (CAF). Their etiology and therapeutic strategy are discussed. METHODS AND RESULTS: Three patients were diagnosed with dual origin plexus-like CAFs drained to the pulmonary artery trunk. Their ages ranged from 40 to 78 years. Enhanced computed tomography could clearly demonstrate three-dimensional anatomy of CAPFs. Four catheter interventions were performed in all 3 patients. Antegrade approach was applied in 2 procedures and retrograde approach in 2 procedures. A detachable coil was used in 3 procedures and a combination of a vascular plug and detachable coils for 1 procedure. The effective occlusion was achieved in all patients without complications. Follow-up myocardial scintigraphy showed no perfusion defect in all patients. CONCLUSIONS: CAPFs in our cases, which developed in their adulthood, anatomically correspond with the vasa vasorum of the proximal of the great arteries. The dual origin multiple plexus-like CAPFs might develop from the vasa vasorum of the proximal portion of the great arteries with age. The antegrade approach would be effective because even dual origin multiple plexus-like CAFs converged and drained to a single major exit of the pulmonary trunk. Catheter occlusion could be feasible and safe for dual origin multiple plexus-like CAPFs.