Familial Intraductal Papillary Mucinous Neoplasm Associated With the Germline MSH6 Missense Variant and Progression of Pancreatic cancer.

OBJECTIVES: Intraductal papillary mucinous neoplasm (IPMN) in individuals with at least one first-degree relative with IPMN is defined as familial IPMN. However, few studies have reported on familial IPMN, its clinical characteristics, or the associated genetic factors. MATERIALS AND METHODS: We report the case of a 58-year-old woman with multifocal IPMN and a mural nodule in the pancreatic body. The patient underwent a distal pancreatectomy and developed pancreatic head cancer 1 year and 6 months postoperatively. The patient had a family history of multifocal IPMN in her father. Therefore, a genetic predisposition to IPMN and pancreatic cancer was suspected. The patient was analyzed for germline variants, and the resected IPMN was subjected to immunohistochemical and somatic variant analyses. RESULTS: Next-generation sequencing revealed a heterozygous germline missense variant in exon 5 of MSH6 (c.3197A>G; Tyr1066Cys). The pathogenicity of this variant of uncertain significance was suspected based on multiple in silico analyses, and the same MSH6 variant was identified in the patient's father's colonic adenoma. The mural nodule in the pancreatic body was pathologically diagnosed as a high-grade IPMN with ossification and somatic KRAS and PIK3CA variants. CONCLUSIONS: This case revealed a possible genetic factor for familial IPMN development and presented interesting clinicopathological findings.

Evaluation of the holding-up uterus technique for placenta accreta spectrum cesarean hysterectomy in shocked patients with a high shock index: a case series study.

BACKGROUND: Placenta accreta spectrum (PAS) cesarean hysterectomy is performed under conditions of shock and can result in serious complications. This study aimed to evaluate the usefulness of the "Holding-up uterus" surgical technique with a shock index (S.I.) > 1.5. METHODS: Twelve patients who underwent PAS cesarean hysterectomy were included in the study. RESULTS: Group I had S.I. > 1.5, and group II had S.I. ≤ 1.5. Group I had more complications, but none were above Grade 3 or fatal. Preoperative scheduled uterine artery embolization did not result in serious complications, but three patients who had emergency common iliac artery balloon occlusion (CIABO) and a primary total hysterectomy with S.I. > 1.5 had postoperative Grade 2 thrombosis. Two patients underwent manual ablation of the placenta under CIABO to preserve the uterus, both with S.I. > 1.5. CONCLUSIONS: The study found that the "Holding-up uterus" technique was safe, even in critical situations with S.I. > 1.5. CIABO had no intervention effect. The study also identified assisted reproductive technology pregnancies with a uterine cavity length of less than 5 cm before conception as a critical factor.

Rectus Sheath Hematoma Triggered by Post-cesarean Anticoagulant Therapy for Intraoperative Acute Pulmonary Thromboembolism: A Case Report.

Anticoagulant therapy is essential for the prevention or treatment of peripartum venous thromboembolism (VTE). Administration of a therapeutic dose of anticoagulant immediately after cesarean section may result in the formation of a rectus sheath hematoma. A 32-year-old Japanese woman delivered twin neonates by cesarean section at 37+5 weeks of gestation. After the removal of the placenta, the patient suddenly complained of left anterior chest pain and dyspnea with hypotension and desaturation, requiring the administration of oxygen and vasopressors. Postoperative contrast-enhanced computed tomography (CT) revealed pulmonary embolism and massive right ovarian vein thrombosis (OVT). An inferior vena cava filter was placed and continuous intravenous heparin was started. A rectus sheath hematoma was noted on postoperative day 2 (POD 2). On POD 5, heparin administration was temporarily discontinued because of an enlarged rectus sheath hematoma. Approximately 24 hours later, the hemoglobin level recovered, and heparin administration was resumed. No further expansion of the hematoma was observed. When a rectus sheath hematoma is formed due to treatment with a therapeutic dose of anticoagulant immediately after cesarean section for peripartum VTE, temporary suspension of anticoagulant administration is reasonable to prevent further expansion of the hematoma without fatal complication.

Impact of visiting restrictions on Edinburgh postnatal depression scale screening scores at one month postpartum during the spread of COVID-19: a single-center case-control study in Japan.

BACKGROUND: This study aimed to evaluate whether "visiting restrictions" implemented due to the coronavirus disease 2019 (COVID-19) pandemic are a risk factor for postpartum depression using the Edinburgh Postnatal Depression Scale (EPDS). METHODS: This case-control study participants who gave birth during the spread of COVID-19 (COVID-19 study group) and before the spread of COVID-19 (control group). Participants completed the EPDS at 2 weeks and 1 month after childbirth. RESULTS: A total of 400 cases (200 in each group) were included in this study. The EPDS positivity rate was significantly lower with visiting restrictions than without (8.5% vs.18.5%, p = 0.002). Multivariate analysis of positive EPDS screening at the 1st month checkup as the objective variable revealed that visiting restrictions (odds ratio (OR): 0.35, 95% confidence interval (CI): 0.18-0.68), neonatal hospitalization (OR: 2.17, 95% CI: 1.08-4.35), and prolonged delivery (OR: 2.87, 95% CI: 1.20-6.85) were factors associated with an increased risk of positive EPDS screening. CONCLUSION: Visiting restrictions on family during the hospitalization period for delivery during the spread of COVID-19 pandemic did not worsen EPDS screening scores 1 month postpartum, but stabilized the mental state of some mothers.

A 41-Year-Old Woman with Spontaneous Hemoperitoneum in Pregnancy at 28 Weeks.

BACKGROUND Spontaneous hemoperitoneum in pregnancy (SHiP), defined as nontraumatic, acute intra-abdominal bleeding during pregnancy or the postpartum period, is a serious life-threatening complication to mother and child. Endometriosis is a major risk factor for SHiP. This study presents the case of a 41-year-old woman with adenomyosis who developed hemoperitoneum due to endometriosis at 28 weeks of pregnancy. CASE REPORT The patient was a 41-year-old woman (gravida 1, para 0) who conceived via artificial insemination. She had diffuse adenomyosis in the posterior uterine wall and was admitted to our hospital at 12 weeks of gestation with persistent lower abdominal pain. She had started treatment with hydroxyprogesterone caproate to reduce the focal inflammation of adenomyosis. At 28 weeks of gestation, she developed severe lower abdominal pain, and ultrasonography revealed prolonged fetal heart rate deceleration. An emergency cesarean delivery was performed, and a 907 g female infant with an Apgar score of 2/3 was delivered. Umbilical artery blood pH was 7.15. Bleeding from the veins surrounding an endometriotic lesion on the posterior wall of the uterus was observed, and SHiP was diagnosed. CONCLUSIONS Pregnancies complicated by endometriosis or adenomyosis require perinatal management, considering the possibility of SHiP complication. If acute abdominal pain and fetal heart rate deceleration occur during pregnancy, a search for intra-abdominal bleeding should be performed and emergent open hemostasis or cesarean delivery should be considered.

Low-frequency maternal novel MYH7 mosaicism mutation in recurrent fetal-onset severe left ventricular noncompaction: a case report.

Background: Left ventricular noncompaction (LVNC) is a rare inherited cardiomyopathy with a broad phenotypic spectrum. The genotype-phenotype correlations in fetal-onset LVNC have not yet been fully elucidated. In this report, we present the first case of severe fetal-onset LVNC caused by maternal low-frequency somatic mosaicism of the novel myosin heavy chain 7 (MYH7) mutation. Case presentation: A 35-year-old pregnant Japanese woman, gravida 4, para 2, with no significant medical or family history of genetic disorders, presented to our hospital. In her previous pregnancy at 33 years of age, she delivered a male neonate at 30 weeks of gestation with cardiogenic hydrops fetalis. Fetal echocardiography confirmed LVNC prenatally. The neonate died shortly after birth. In the current pregnancy, she again delivered a male neonate with cardiogenic hydrops fetalis caused by LVNC at 32 weeks of gestation. The neonate died shortly after birth. Genetic screening of cardiac disorder-related genes by next-generation sequencing (NGS) was performed which revealed a novel heterozygous missense MYH7 variant, NM_000257.3: c.2729A > T, p.Lys910Ile. After targeted and deep sequencing by NGS, the same MYH7 variant (NM_000257.3: c.2729A > T, p.Lys910Ile) was detected in 6% of the variant allele fraction in the maternal sequence but not in the paternal sequence. The MYH7 variant was not detected by conventional direct sequencing (Sanger sequencing) in either parent. Conclusions: This case demonstrates that maternal low-frequency somatic mosaicism of an MYH7 mutation can cause fetal-onset severe LVNC in the offspring. To differentiate hereditary MYH7 mutations from de novo MYH7 mutations, parental targeted and deep sequencing by NGS should be considered in addition to Sanger sequencing.

Small solitary atrial fractionated electrogram zone as a novel ablation target for persistent atrial fibrillation.

INTRODUCTION: Various adjunctive approaches to pulmonary vein isolation (PVI) have been attempted for persistent atrial fibrillation (perAF) and longstanding persistent AF (ls-perAF). We aimed to identify the novel zones responsible for perpetuation of AF. METHODS: To identify novel zones acting as a source of perAF and ls-perAF after PVI/re-PVI, we performed fractionation mapping in 258 consecutive patients with perAF (n = 207) and ls-perAF (n = 51) in whom PVI/re-PVI failed to restore sinus rhythm. RESULTS: In 15 patients with perAF (5.8%: 15/258), fractionation mapping identified a small solitary zone (<1 cm2 ) with high-frequency and irregular waves, showing fractionated electrograms (EGM). We defined this zone as the small solitary atrial fractionated EGM (SAFE) zone. The small SAFE zone was surrounded characteristically by a homogeneous area showing relatively organized activation with nonrapid and nonfractionated waves. Only one small SAFE zone was detected in each patient. This characteristic electrical phenomenon was observed stably during the procedure until ablation. AF duration, (defined as the duration between initial detection of AF and the current ablation) was longer in patients with the small SAFE zone than in those without (median, [25 and 75 percentiles]; 5.0 [3.5, 7.0] vs. 1.1 [1.0, 4.0] years, p = .0008). Longer AF cycle length was observed in patients with the small SAFE zone than in those without. The ablation of the small SAFE zone terminated AF in all 15 patients without any need for other ablations. AF/atrial tachycardia-free rate at follow-up was 93% (14/15) at 6 months, 87% (13/15) at 1 year, and 60% (9/15) at 2 years. CONCLUSIONS: Using fractionation mapping, this study identified a small SAFE zone surrounded characteristically by a homogeneous, relatively organized, low-excitability EGM lesion. The ablation of the small SAFE zone terminated AF in all patients, demonstrating it as a substrate for perpetuated AF. Our findings provide novel ablation targets in perAF patients with prolonged AF duration. Further studies to confirm the present results are warranted.

Impact of maternal late hospital arrival on adverse outcome of offspring affected by placental abruption: A regional multicenter nested case-control study in Japan.

AIMS: To elucidate the influence of the time-intervals between the onset and arrival (TIME 1), onset and delivery (TIME 2), and the decision to deliver and delivery (TIME 3) on severe adverse outcomes of offspring born to mothers complicated by placental abruption outside the hospital. METHODS: This is a multicenter nested case-control study about placental abruption at Fukui Prefecture, a regional area in Japan, through 2013 to 2017. Multiple pregnancy, fetal or neonatal congenital abnormality, and unknown detailed information at onset of placental abruption were excluded. A composite of perinatal death and cerebral palsy or death at 18-36 months of corrected age was defined as the adverse outcome. The relationship between time-intervals and the adverse outcome was analyzed. RESULTS: The 45 subjects for analysis were divided into two groups, including a group with and without adverse outcome (poor, n = 8; and good, n = 37). TIME 1 was longer in the poor group (150 vs. 45 min, p < 0.001). A subgroup analysis targeted to 29 cases with preterm birth at the third trimester indicates that TIME 1 and TIME 2 were longer in the poor group (185 vs. 55 min, p = 0.02; and 211 vs. 125 min, p = 0.03), while TIME 3 was shorter in the poor group (21 vs. 53 min, p = 0.01). CONCLUSIONS: Long time-intervals between onset and arrival or onset and delivery may be correlated with perinatal death or cerebral palsy in surviving infants affected by placental abruption.

Optimal prevention method of phrenic nerve injury in superior vena cava isolation: efficacy of high-power, short-duration radiofrequency energy application on the risk points.

BACKGROUND OR PURPOSE: Superior vena cava isolation (SVCI) is widely performed adjunctively to atrial fibrillation (AF) ablation. Right phrenic nerve injury (PNI) is a complication of this procedure. The purpose of the study is to determine the optimal PNI prevention method in SVCI. METHODS: A total of 1656 patients who underwent SVCI between 2009 and 2022 were retrospectively examined. PNI was diagnosed based on the diaphragm position and movement in the upright position on chest radiographs before and after SVCI. RESULTS: With the introduction of various PN monitoring systems over the years, the incidence of SVCI-associated PNI has decreased. However, complete PNI avoidance has not been achieved. PNI incidence according to fluoroscopy-guided PN monitoring, high-output pace-guided, compound motor action potential-guided, and 3-dimensional electro-anatomical mapping (EAM) systems was 8.1% (38/467), 2.7% (13/476), 2.4% (4/130), and 2.8% (11/389), respectively. However, a high-power, short-duration (50 W/7 s) radiofrequency (RF) energy application only on PNI risk points tagged by a 3-dimensional EAM system completely avoids PNI (0%; 0 /160 since April 2021). PNI showed no symptoms and recovered within an average of 188 days post-SVCI, except for a few patients who required > 1 year. CONCLUSIONS: Although PNI incidence decreased annually with the introduction of various monitoring systems, these monitoring systems did not prevent PNI completely. Most notably, the delivery of a high-power, short-duration RF energy only on risk points tagged by EAM prevented PNI completely. PNI recovered in all patients. The application of higher-power, shorter-duration RF energy on risk points tagged by EAM appears to be an optimal PNI prevention maneuver.

Timing of Delivery for Twins With Growth Discordance and Growth Restriction: An Individual Participant Data Meta-analysis.

OBJECTIVE: First, to evaluate the risks of stillbirth and neonatal death by gestational age in twin pregnancies with different levels of growth discordance and in relation to small for gestational age (SGA), and on this basis to establish optimal gestational ages for delivery. Second, to compare these optimal gestational ages with previously established optimal delivery timing for twin pregnancies not complicated by fetal growth restriction, which, in a previous individual patient meta-analysis, was calculated at 37 0/7 weeks of gestation for dichorionic pregnancies and 36 0/7 weeks for monochorionic pregnancies. DATA SOURCES: A search of MEDLINE, EMBASE, ClinicalTrials.gov, and Ovid between 2015 and 2018 was performed of cohort studies reporting risks of stillbirth and neonatal death in twin pregnancies from 32 to 41 weeks of gestation. Studies from a previous meta-analysis using a similar search strategy (from inception to 2015) were combined. Women with monoamniotic twin pregnancies were excluded. METHODS OF STUDY SELECTION: Overall, of 57 eligible studies, 20 cohort studies that contributed original data reporting on 7,474 dichorionic and 2,281 monochorionic twin pairs. TABULATION, INTEGRATION, AND RESULTS: We performed an individual participant data meta-analysis to calculate the risk of perinatal death (risk difference between prospective stillbirth and neonatal death) per gestational week. Analyses were stratified by chorionicity, levels of growth discordance, and presence of SGA in one or both twins. For both dichorionic and monochorionic twins, the absolute risks of stillbirth and neonatal death were higher when one or both twins were SGA and increased with greater levels of growth discordance. Regardless of level of growth discordance and birth weight, perinatal risk balanced between 36 0/7-6/7 and 37 0/7-6/7 weeks of gestation in both dichorionic and monochorionic twin pregnancies, with likely higher risk of stillbirth than neonatal death from 37 0/7-6/7 weeks onward. CONCLUSION: Growth discordance or SGA is associated with higher absolute risks of stillbirth and neonatal death. However, balancing these two risks, we did not find evidence that the optimal timing of delivery is changed by the presence of growth disorders alone. SYSTEMATIC REVIEW REGISTRATION: PROSPERO, CRD42018090866.

Tadalafil treatment for fetuses with early-onset growth restriction: a protocol for a multicentre, randomised, placebo-controlled, double-blind phase II trial (TADAFER IIb).

INTRODUCTION: TheTADAlafil treatment for Fetuses with early-onset growth Restriction: multicentrer, randomizsed, phase II trial (TADAFER II) study showed the possibility of prolonging the pregnancy period in cases of early-onset fetal growth restriction; however, it was an open-label study. To establish further evidence for the efficacy of tadalafil in this setting, we planned a multicentre, randomised, placebo-controlled, double-blind trial. METHODS AND ANALYSIS: This trial will be conducted in 180 fetuses with fetal growth restriction enrolled from medical centres in Japan; their mothers will be randomised into three groups: arm A, receiving two times per day placebo; arm B, receiving one time per day 20 mg tadalafil and one time per day placebo and arm C, receiving 20 mg two times per day tadalafil. The primary endpoint is the prolongation of gestational age at birth, defined as days from the first day of the protocol-defined treatment to birth. To minimise bias in terms of fetal baseline conditions and timing of delivery, a fetal indication for delivery as in TADAFER II will be established in this trial. The investigator will evaluate fetal baseline conditions at enrolment and decide the timing of delivery based on this indication. ETHICS AND DISSEMINATION: This study has been approved by Mie University Hospital Clinical Research Review Board on 22 July 2019 (S2018-007). Written informed consent will be obtained from all mothers before recruitment. Our findings will be widely disseminated through peer-reviewed publications. TRIAL REGISTRATION: jRCTs041190065.

Laparoscopic surgery-associated massive subcutaneous emphysema requiring mechanical ventilation in a patient with endometriosis: a case report.

Although subcutaneous emphysema is a common benign complication of laparoscopic surgery, airway obstruction can occur due to pharyngeal emphysema when it extends to the neck. Here, we report a case of subcutaneous emphysema extending to the neck that required mechanical ventilation in a 51-year-old patient with endometriosis and severe adhesions during total laparoscopic hysterectomy and bilateral salpingo-oophorectomy. Although surgical or disease-specific risk stratification has not yet been established, the severe adhesions due to endometriosis and massive peritoneal defect due to the procedure might lead to the fragility of the subcutaneous tissue, resulting in a massive subcutaneous emphysema. This study highlights the importance of preoperative risk assessment in addition to intraoperative and postoperative monitoring for ventilation disorders and subcutaneous emphysema.

Rates of atrial flutter occurrence and cavotricuspid isthmus reconduction after prophylactic isthmus ablation performed during atrial fibrillation ablation: a clinical study, review, and comparison with previous findings.

PURPOSE: Based on the high rate of coexisting atrial fibrillation (AF) and atrial flutter (AFL), prophylactic cavotricuspid isthmus ablation (CTIA) adjunctive to AF ablation has recently been attempted in patients with AF and without AFL. The present study aimed to determine the rates of AFL occurrence and CTI reconduction after performing CTI ablation adjunctive to AF ablation. METHODS: We analyzed the data of 3833 consecutive patients with AF, who underwent prophylactic CTIA with AF ablation between 2009 and 2020. RESULTS: In all patients, CTIA and AF ablations were successful. Clinical AFL occurred in seven patients (0.18%, 7/3,833), and the observed rate was lower than those reported for cases of AF ablation without CTIA and for those of CTIA for pure AFL. A second ablation was needed in 745 patients at a median of 253 days (25 and 75 percentiles, 116 and 775 days) after the first ablation. In 12.1% (90/745) of the patients, CTI reconduction was observed. The reconduction rate was lower than that previously reported for CTIA for pure AFL. CONCLUSIONS: The present retrospective study found acceptably low rates of clinical AFL occurrence and CTI reconduction following prophylactic CTIA performed with AF ablation, which was supported by the findings obtained after performing a comparison of the rates with those of other ablations (AF ablation only and CTIA for pure AFL). Considering the high correlation between AF and AFL, the present study provided information regarding the efficacy of adjunctive CTIA.

Acute type B aortic dissection in a pregnant woman with undiagnosed Marfan syndrome: A case report and review of the literature.

Aortic dissection during pregnancy is rare but can be life-threatening to both the mother and the foetus. Marfan syndrome is a major risk factor for acute aortic dissection during pregnancy. Here, we present the case of a woman who had not been diagnosed with Marfan syndrome prior to pregnancy and who developed acute type B dissection at 32 weeks of gestation. The maternal hemodynamic status was stable, and foetal well-being was ensured. However, under conservative treatment, the dissection extended to the descending aorta, reaching the bilateral iliac artery 2 days later. Due to foetal distress, preterm delivery was performed via caesarean section. The primary treatment of type B aortic dissection is conservative medical treatment, with the goals of hemodynamic stabilisation, minimising the extent of the dissection and decreasing the risk of rupture. However, type B aortic dissection, even the uncomplicated type, in pregnant women may require early and aggressive obstetric interventions to improve maternal and foetal prognoses.

Mentality of pregnant women and obstetric healthcare workers about prenatal SARS-CoV-2 testing: A regional survey over the first wave of the COVID-19 pandemic in Japan.

AIM: To clarify the mentality of pregnant women and obstetric healthcare workers about prenatal severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) screening testing. METHODS: A multicenter questionnaire survey about prenatal SARS-CoV-2 screening testing was conducted among pregnant women, midwives and nurses (M&Ns), and obstetricians at all delivery facilities in Fukui Prefecture between June 30, 2020 and July 22, 2020. RESULTS: Of 297 pregnant women, 150 (50.5%) underwent prenatal polymerase chain reaction (PCR) testing, and 107 of them (71.3%) answered that because of prenatal PCR tests, they could give birth with relief. One hundred forty-five (48.8%) were concerned about the disadvantages of receiving positive prenatal PCR results. Of 287 M&Ns, 151 (52.6%) answered that prenatal PCR screening testing could reduce anxiety about infection to themselves; this belief was more common among M&Ns working at the nonreception facility than among those at COVID-19 reception facilities (60.7% vs. 47.1%, P = 0.02). Of 57 obstetricians, 31 (54.4%) agreed to prenatal SARS-CoV-2 PCR screening testing, the rate of which was significantly higher among obstetricians at nonreception facilities than those at reception facilities (70.3% vs. 25.0%, P < 0.01). Fourteen obstetricians (24.6%) were concerned about excessive medical treatment for asymptomatic pregnant women with false-positive PCR results. CONCLUSIONS: Pregnant women experience anxieties during the COVID-19 pandemic, and prenatal SARS-CoV-2 screening may reduce their anxiety to some extent. However, obstetrics staff at COVID-19 reception facilities are aware of the limits of prenatal screening and are concerned about excessive medical intervention due to false-positive results.

Influence of preoperative light vaginal bleeding for perinatal adverse outcomes on physical examination-indicated cerclage.

PURPOSE: To clarify the relationship between light vaginal bleeding (LVB) before physical examination-indicated cerclage (PEIC) and perinatal adverse outcomes. METHODS: This was a retrospective cohort study involving 94 singleton pregnancies undergoing PEIC <26 weeks of gestation at a single perinatal medical center between 2008 and 2015. The primary outcome was set as spontaneous preterm birth (sPTB) <34 weeks of gestation. The secondary outcomes were set as the second-trimester loss prior to 22 weeks of gestation, sPTB before 28 weeks of gestation, sPTB before 37 weeks of gestation, and stillbirth or neonatal death. Relationships between LVB and adverse outcomes were evaluated using logistic regression analysis. RESULTS: Preoperative LVB was detected in 16 cases (17.0%). Multivariate logistic regression analyses revealed that preoperative LVB was an independent risk factor for sPTB <34 weeks of gestation (adjusted odds ratio [aOR]: 8.42; 95% confidence interval [CI]: 1.72-41.1; p < .01), sPTB <28 weeks of gestation (aOR: 5.98; 95% CI: 1.67-21.4; p < .01) and perinatal death (aOR: 8.47; 95% CI: 1.11-64.5; p = .04). CONCLUSIONS: Vaginal bleeding prior to PEIC, even nonsignificant or self-limiting, is associated with sPTB before 28 or 34 weeks of gestation and perinatal death.

Efficacy of an Adjunctive Electrophysiological Test-Guided Left Atrial Posterior Wall Isolation in Persistent Atrial Fibrillation Without a Left Atrial Low-Voltage Area.

BACKGROUND: Electrical remodeling precedes structural remodeling. In adjunctive left atrial (LA) low-voltage area (LVA) ablation to pulmonary vein isolation of atrial fibrillation (AF), LA areas without LVA have not been targeted for ablation. We studied the effect of adjunctive LA posterior wall isolation (PWI) on persistent AF without LA-LVA according to electrophysiological testing (EP test). METHODS: We examined consecutive patients with persistent AF with (n=33) and without (n=111) LA-LVA. Patients without LA-LVA were randomly assigned to EP test-guided (n=57) and control (n=54) groups. In the EP test-guided group, an adjunctive PWI was performed in those with positive results (PWI subgroup; n=24), but not in those with negative results (n=33). The criteria for positive EP tests were an effective refractory period ≤180 ms, effective refractory period>20 ms shorter than the other sites, and/or induction of AF/atrial tachycardia (AT) during measurements. LVA ablation was performed in the patients with LA-LVA. RESULTS: During the follow-up period (62±33 weeks), the EP test-guided group had significantly lower recurrence rates (19%,11/57 versus 41%, 22/54, P=0.012) and higher Kaplan-Meier AF/AT-free survival curve rates than the control group (P=0.01). No significant differences in the recurrence and AF/AT-free survival curve rates between the PWI (positive EP test) and non-PWI (negative EP test) subgroups were observed. Therefore, PWI for positive EP tests reduced the AF/AT recurrence in the EP test-guided group. A stepwise Cox proportional hazard analyses identified EP test-guided ablation as a factor reducing the recurrence rate. The recurrence rates in the LA-LVA ablation group and EP test-guided group were similar. CONCLUSIONS: This pilot study proposed that an EP test-guided adjunctive PWI of persistent AF without LA-LVA potentially reduced AF/AT recurrences. The results suggest that there is an AF substrate in the LA with altered electrophysiological function even when there is no LA-LVA. Graphic Abstract: A graphic abstract is available for this article.