Comprehensive Risk Assessment in Patients With Pulmonary Arterial Hypertension Referred for Lung Transplantation.

BACKGROUND: Whether comprehensive risk assessment predicts post-referral outcome in patients with pulmonary arterial hypertension (PAH) referred for lung transplantation (LT) in Japan is unknown.Methods and Results: We retrospectively analyzed 52 PAH patients referred for LT. Risk status at referral was assessed using 3- and 4-strata models from the 2022 European Society of Cardiology and European Respiratory Society guidelines. The 3-strata model intermediate-risk group was further divided into 2 groups based on the median proportion of low-risk variables (modified risk assessment [MRA]). The primary outcome was post-referral mortality. During follow-up, 9 patients died and 13 patients underwent LT. There was no survival difference among 3-strata model groups. The 4-strata model classified 33, 16, and 3 patients as low intermediate, high intermediate, and high risk, respectively. The 4-strata model identified high-risk patients with a 1-year survival rate of 33%, but did not discriminate survival between the intermediate-risk groups. The MRA classified 15, 28, 8, and 1 patients as low, low intermediate, high intermediate, and high risk, respectively. High intermediate- or high-risk patients had worse survival (P<0.001), with 1- and 3-year survival rates of 64% and 34%, respectively. MRA high intermediate- or high-risk classification was associated with mortality (hazard ratio 12.780; 95% confidence interval 2.583-63.221; P=0.002). CONCLUSIONS: Patients classified as high intermediate or high risk by the MRA after treatment should be referred for LT.

Multimodality Risk Assessment of Patients with Ischemic Heart Disease Using Deep Learning Models Applied to Electrocardiograms and Chest X-rays.

Comprehensive management approaches for patients with ischemic heart disease (IHD) are important aids for prognostication and treatment planning. While single-modality deep neural networks (DNNs) have shown promising performance for detecting cardiac abnormalities, the potential benefits of using DNNs for multimodality risk assessment in patients with IHD have not been reported. The purpose of this study was to investigate the effectiveness of multimodality risk assessment in patients with IHD using a DNN that utilizes 12-lead electrocardiograms (ECGs) and chest X-rays (CXRs), with the prediction of major adverse cardiovascular events (MACEs) being of particular concern.DNN models were applied to detection of left ventricular systolic dysfunction (LVSD) on ECGs and identification of cardiomegaly findings on CXRs. A total of 2107 patients who underwent elective percutaneous coronary intervention were categorized into 4 groups according to the models' outputs: Dual-modality high-risk (n = 105), ECG high-risk (n = 181), CXR high-risk (n = 392), and No-risk (n = 1,429).A total of 342 MACEs were observed. The incidence of a MACE was the highest in the Dual-modality high-risk group (P < 0.001). Multivariate Cox hazards analysis for predicting MACE revealed that the Dual-modality high-risk group had a significantly higher risk of MACE than the No-risk group (hazard ratio (HR): 2.370, P < 0.001), the ECG high-risk group (HR: 1.906, P = 0.010), and the CXR high-risk group (HR: 1.624, P = 0.018), after controlling for confounding factors.The results suggest the usefulness of multimodality risk assessment using DNN models applied to 12-lead ECG and CXR data from patients with IHD.

[Successful cord blood transplantation for refractory gamma-delta hepatosplenic T-cell lymphoma developed during treatment of Crohn's disease].

The patient was a 21-year-old man who had been diagnosed with Crohn's disease and received infliximab and azathioprine six years earlier. He was admitted with fever and fatigue. Peripheral blood examination showed LDH 2,473 U/l and thrombocytopenia, and contrast-enhanced computed tomography (CT) showed hepatosplenomegaly. Bone marrow biopsy and liver biopsy showed CD4+CD56+TCRγδ＋CD8- atypical cells, leading to a diagnosis of hepatosplenic T-cell lymphoma (HSTCL). The patient was refractory to CHOP and DA-EPOCH, and therefore received cord blood transplantation with myeloablative conditioning. CT showed reduced in hepatosplenomegaly and peripheral blood examination showed LDH 165 U/l and plt 180,000/µl, so the patient was discharged on day117. HSTCL is a tumor of immature γδT cells with a Vδ1 mutation in the spleen, and immunodeficiency has been implicated in its pathogenesis. Patients with inflammatory bowel disease treated with azathioprine are known to have an increased risk of lymphoproliferative disease. In this case, use of immunosuppressive drugs for Crohn's disease may have caused malignant transformation of γδ cells in the intestinal epithelium. Although the patient was refractory to chemotherapy, he was able to achieve remission with early cord blood transplantation and long-term survival is expected.

Impact of psychiatric disorders on the hemodynamic and quality of life outcome of balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension: a retrospective study.

BACKGROUND: Balloon pulmonary angioplasty (BPA) has beneficial effects on pulmonary hemodynamics, exercise capacity, and quality of life (QOL) in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Recently, emerging evidence suggests a relationship between CTEPH and psychiatric disorders (PD). However, data on the clinical efficacy of BPA in CTEPH patients with PD are lacking. METHODS: We retrospectively analyzed 75 patients with inoperable/residual CTEPH who underwent BPA and right-sided heart catheterization before the initial BPA and within 1 year after the last procedure. QOL was evaluated using the European Quality of Life Five Dimension (EQ-5D) scale in 27 patients before and after BPA sessions. Baseline and post-procedural hemodynamic, functional, and QOL parameters were compared between the patients with and without PD. RESULTS: Among the 75 participants, 22 (29.3%) patients were categorized in the PD group. Although PD group had a similar mean pulmonary artery pressure level compared with non-PD group (40 ± 7 vs. 41 ± 9 mmHg, p = 0.477), they tended to have unfavorable QOL status (0.63 ± 0.22 vs. 0.77 ± 0.19, p = 0.102). BPA significantly improved pulmonary hemodynamics, laboratory parameters and exercise tolerance in both groups. BPA also significantly improved EQ-5D scores in the non-PD group (from 0.77 ± 0.19 to 0.88 ± 0.13, p < 0.001), but the scores remained unchanged in the PD group (from 0.63 ± 0.22 to 0.67 ± 0.22, p = 0.770). During the long-term period [1,848 (1,055-2,565) days], both groups experienced similar mortality rates (PD 4.6% vs. non-PD 5.7%, p = 1.000). CONCLUSIONS: BPA improved hemodynamic and functional parameters irrespective of PD, but its effect on QOL was limited in patients with PD.

Prophylactic negative pressure wound therapy is not effective for preventing driveline infection following left ventricular assist device implantation.

BACKGROUND: Driveline infection (DLI) following left ventricular assist device (LVAD) implantation remains an unresolved problem. Negative pressure wound therapy (NPWT) promotes wound healing by applying negative pressure on the surface of the wound. Recently, the prophylactic application of NPWT to closed surgical incisions has decreased surgical site infections in various postsurgical settings. Therefore, we evaluated the efficacy and safety of prophylactic NPWT for preventing DLI in patients with LVAD implantation. METHODS: Prophylactic NPWT was provided to 50 patients who received continuous-flow LVADs as bridge-to-transplant therapy at our institution between May 2018 and October 2020 (NPWT group). The negative pressure dressing was applied immediately after surgery and retained on the driveline exit site for 7 days with a continuous application of -125 mm Hg negative pressure. The primary outcome was DLI within 1 year of LVAD implantation. We compared the rate of DLI incidence in the NPWT group with that in the historical control cohort (50 patients) treated with the standard dressing (SD) who received LVAD implantation between July 2015 and April 2018 (SD group). RESULTS: No severe complications were associated with the NPWT. During the follow-up period, DLI was diagnosed in 16 participants (32%) in the NPWT group and 21 participants (42%) in the SD group. The rates of DLI incidence and freedom from DLI did not differ between groups (p = 0.30 and p = 0.63). CONCLUSIONS: Prophylactic NPWT at the driveline exit site was safe following LVAD implantation. However, it did not significantly reduce the risk of DLI.

Fractional exhaled nitric oxide in adult congenital heart disease.

BACKGROUND: Fractional exhaled nitric oxide levels are related to various clinical diseases. This study investigated the associations between the clinical characteristics and the level of fractional exhaled nitric oxide in patients with adult congenital heart disease. METHODS AND RESULTS: Fractional exhaled nitric oxide values were measured in 30 adult patients with stable congenital heart disease who had undergone right heart catheterization and 17 healthy individuals (controls). There was no significant difference in fractional exhaled nitric oxide values between patients with congenital heart disease and healthy controls. Depending on whether their fractional exhaled nitric oxide values were above or below the median value, patients with congenital heart disease were divided into two groups (low vs. high fractional exhaled nitric oxide groups). The relationship between fractional exhaled nitric oxide values and clinical characteristics was investigated. There was a higher percentage of patients with cyanosis in the low fractional exhaled nitric oxide group (50%) than in the high fractional exhaled nitric oxide group (7.1%). There was no significant difference in right heart catheterization data between the low and high fractional exhaled nitric oxide groups. The fractional exhaled nitric oxide value was correlated to the number of neutrophils in patients with cyanosis (r = 0.84 (N = 8), p = 0.005). CONCLUSIONS: In this cohort of patients with adult congenital heart disease, lower levels of fractional exhaled nitric oxide corresponded to the presence of cyanosis.

Significant impact of left ventricular assist device models on the value of flow-mediated dilation: effects of LVAD on endothelial function.

The precise physiological changes associated with the use of left ventricular assist device (LVAD) are not well characterized. We examined the impact of changes in hemodynamic state using LVAD on endothelial function. We measured flow-mediated vasodilation (FMD) to evaluate endothelial vasodilator function of the brachial artery in 53 patients (dilated cardiomyopathy: 39, ischemic cardiomyopathy: 4, and others: 10) with an implanted LVAD (DuraHeart, EVAHEART, or HeartMate II). We found that FMD value in the HeartMateII LVAD group (9.3% ± 2.9%) was significantly higher than those in the other two groups (EVAHEART: 6.7% ± 2.8% and DuraHeart: 6.2% ± 4.0%). Other factors that affected the FMD value were age (r = - 0.31, p = 0.026), Brinkman index (r = - 0.30, p = 0.029); however, aortic opening, aortic regurgitation, and other hemodynamic parameters such as cardiac index or pulmonary capillary wedge pressure did not correlate with FMD. Multivariate analyses revealed that the difference among the LVAD models most significantly affected the FMD values after adjusting for age and smoking status (t = 2.6, p = 0.014). Event free survival rate of death and cerebral infarction was not significantly different according to the value of FMD. The difference among the LVAD groups most significantly affected the state of endothelial function and it had more impact than other clinical factors.

Novel Balloon Pulmonary Angioplasty Technique for Chronic Thromboembolic Pulmonary Hypertension.

This study aimed to clarify the usefulness of the Ikari-curve left (IL) guiding catheter for balloon pulmonary angioplasty (BPA).The current BPA strategy for chronic thromboembolic pulmonary hypertension is dilation of as many branches as possible to normalize hemodynamics and oxygenation. The shape of the guiding catheter is a major factor in achieving this. However, conventional guiding catheters are difficult to introduce into particular branches. The IL guiding catheter may be suitable; however, its utility remains unclear.We retrospectively analyzed 202 consecutive BPA sessions of 40 patients from November 2016 to October 2019 and divided these sessions into two groups: the IL group where the IL guiding catheter was used and the non-IL group where other catheters were utilized. The occurrence of lung injury was determined by the presence of bloody sputum. We compared the rates of successful introduction into target vessels and assessed for the occurrence of lung injury.The average age of enrolled patients was 60.3 ± 14.4 years, with females comprising 65%. There were 99 sessions in the IL group. The median treated branches per session differed between the 2 groups (IL group: 15 versus non-IL group: 10, P < 0.05). The occurrence of lung injury was lower in the IL group (4.0% versus 11.7%, P = 0.07). The IL group had more successful vessel insertions than the non-IL group (78.8% versus 42.7%, P < 0.01).The IL guiding catheter may be introduced into branches that cannot be accessed by conventional guiding catheters.

The Clinical Efficacy of Endothelin Receptor Antagonists in Patients with Pulmonary Arterial Hypertension.

Therapeutic strategies for pulmonary arterial hypertension (PAH) have made remarkable progress over the last two decades. Currently, 3 types of drugs can be used to treat PAH; prostacyclins, phosphodiesterase 5 inhibitors, and endothelin receptor antagonists (ERA). In Japan, the first generation ERA bosentan was reimbursed in 2005, following which the 2nd generation ERAs ambrisentan and macitentan were reimbursed in 2009 and 2015, respectively. The efficacy of each ERA on hemodynamics in PAH patients remains to be elucidated. The aims of this study were to evaluate the hemodynamic effects of ERAs and compare these effects among each generation of ERAs.We retrospectively examined the clinical parameters of 42 PAH patients who were prescribed an ERA (15 bosentan, 12 ambrisentan, and 15 macitentan) and who underwent a hemodynamic examination before and after ERA introduction at our institution from January 2007 to July 2019.In a total of 42 patients, mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) were significantly decreased and cardiac index was significantly increased after ERA introduction (P < 0.001) and the World Health Organization-Functional class (WHO-Fc) was significantly improved after ERA introduction (P = 0.005). Next, in a comparison between 1st and 2nd generation ERAs, 2nd generation ERAs were found to have brought about greater improvements in hemodynamic parameters (mPAP and PVR. P < 0.01), heart rate, brain natriuretic peptide, arterial oxygen saturation, and mixed venous oxygen saturation than the 1st generation ERA bosentan.We conclude that all ERAs could successfully improve the hemodynamics of PAH patients and that the newer generation ERAs, ambrisentan and macitentan, seemed to be preferable to bosentan.

Correction to: High-intensity aerobic interval training can lead to improvement in skeletal muscle power among in-hospital patients with advanced heart failure.

In the original publication of the article, the unit of hand in Fig. 2 was wrongly given as "N/kg" instead of "kg".

High-intensity aerobic interval training can lead to improvement in skeletal muscle power among in-hospital patients with advanced heart failure.

This study investigated the effectiveness and safety of interval training during in-hospital treatment of patients with advanced heart failure. Twenty-four consecutive patients with advanced symptomatic heart failure who were referred for cardiac transplant evaluation were recruited. After performing aerobic exercise for approximate intensity, high-intensity interval training (HIIT) was performed. The protocol consisted of 3 or 4 sessions of 1-min high-intensity exercise aimed at 80% of peak VO2 or 80% heart rate reserve, followed by 4-min recovery periods of low intensity. In addition to the necessary laboratory data, hand grip strength and knee extensor strength were evaluated at the start of exercise training and both at the start and the end of HIIT. Knee extensor strength was standardized by body weight. The BNP level at the start of exercise training was 432 (812) pg/mL and it significantly decreased to 254 (400) pg/mL (p < 0.001) at the end of HIIT. Hand grip strength did not change during course. By contrast, knee extensor strength significantly increased during HIIT [4.42 ± 1.43 â†’ 5.28 ± 1.45 N/kg, p < 0.001], whereas the improvement of knee extensor strength was not significant from the start of exercise training to the start of HIIT. In addition, the change in knee extensor strength during HIIT was significantly associated with the hemoglobin A1c level at the start of exercise (R = - 0.55; p = 0.015). HIIT has a positive impact on skeletal muscle strength among in-hospital patients with advanced heart failure.

Regular Treatment Strategy with a Large Amount of Carvedilol for Heart Failure Improves Biventricular Systolic Failure in a Patient with Repaired Tetralogy of Fallot.

It is widely known that ß-blockers exert beneficial effects on non-ischemic and ischemic systolic heart failure (sHF) in nonstructural hearts. However, whether ß-blockers exert similar effects on sHF associated with congenital heart disease (CHD), particularly in an anatomical right ventricle, remains under debate.Here we report the case of an adult man with repaired tetralogy of Fallot suffering from biventricular heart failure. Treatment with carvedilol directly improved the systolic function of the right and left ventricles. This case report strongly suggests there is potential for carvedilol to exert a beneficial effect on heart failure in CHD. The appropriate titration of carvedilol and patient follow-up for long-term effects are important when treating adult patients with CHD with ß-blockers.

Clinically Worsening Chronic Thromboembolic Pulmonary Hypertension by Riociguat After Balloon Pulmonary Angioplasty.

Riociguat, a soluble guanylate cyclase stimulator, induces pulmonary artery dilatation through blood flow and is effective in treating chronic thromboembolic pulmonary hypertension (CTEPH). There are two types of vasculopathies in CTEPH based upon its location, in other words, proximal or distal to the thrombus-medicated obstruction. Distal vasculopathy is characterized by intrapulmonary shunts due to diminished blood flow. While other therapeutic interventions for CTEPH including pulmonary endarterectomy and balloon pulmonary angioplasty achieve reperfusion to the distal vasculopathy vessels, the effects of riociguat on distal vasculopathy vessels remain undetermined. Herein, we describe a case of a 66-year-old woman who exhibited deterioration of mean pulmonary artery pressure and exercise tolerance after a 4-month treatment with riociguat. She received balloon pulmonary angioplasty prior to riociguat administration. Her lung perfusion scintigraphy and pulmonary angiography findings did not change over the course of treatment. Notably, after the discontinuation of riociguat, her clinical values returned to their levels prior to riociguat administration. Her intrapulmonary shunt ratio followed a similar course as her hemodynamic status. We demonstrate that riociguat can deteriorate hemodynamic status, which may mediate the dilatation of intrapulmonary shunts. We should perform close monitoring of symptoms and hemodynamic status after riociguat administration, especially in patients in whom the reperfused DVs occurred due to invasive treatment.

Effectiveness of Nitroglycerin in Managing Subacute Lung Bleeding Induced by Balloon Pulmonary Angioplasty.

Lung bleeding (LB) and hemoptysis is a common but life-threating complication of balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension. LBs related to BPA mostly occur acutely during BPA session. Therefore, it can usually be managed with occlusion balloon or other catheter-based approaches. While LB also develops subacutely after BPA session, the pharmacological option to subacute LB is currently limited. Here, we present a case of subacute LB which can be managed with intravenous administration of nitroglycerin. Nitrate mediated venous dilation can be an effective therapeutic option in managing LB and hemoptysis after BPA session.

Combined Surgical and Medical Therapy for Candida Prosthetic Endocarditis in a Patient with Repaired Tetralogy of Fallot.

Candida prosthetic endocarditis (CPE) is an uncommon and fatal complication in adults with congenital heart disease. The current guidelines for the management of fungal endocarditis recommend a combination of surgical and medical therapy. However, it still remains uncertain when surgical management in CPE patients should be performed. Therefore, the prognosis of CPE patients is very poor. Here we report a case of CPE in a 31-year-old woman who had undergone surgical repair for tetralogy of Fallot during childhood and pulmonary valve replacement at the age of 21 years. She underwent re-pulmonary valve replacement after being sufficiently sterilized with a 5-week course of antifungal medical therapy, leading to clinical improvement. In CPE patients, it is necessary to perform surgical therapy while suppressing the activity of fungi as much as possible.

Risk factors affecting third molar autotransplantation during 5 and 10 years.

The aim of this study was to investigate risk factors affecting 5- and 10-year survival in autotransplantation of third molars with complete root formation at dental clinics. Participating dentists were requested to provide information on transplantations performed between 1 January 1990 and 31 December 2009. After data screening and elimination, 183 teeth in 171 men aged 20-72 years (mean, 44.8 years) and 205 teeth in 189 women aged 20-74 years (mean, 42.0 years) were included in the study. A single-factor analysis using the log-rank test revealed that the following factors had a significant influence (p<0.05) on 5-year survival in transplanted teeth in men: recipient site in the maxilla and fewer than 25 present teeth; those for 10-year survival, on the other hand, were recipient site tooth extraction due to periodontal disease, recipient site in the maxilla, fewer than 25 present teeth, and Eichner index Group B1 to C. Cox regression analysis revealed that the odds ratio for 5-year survival for recipient site in the maxilla was 2.873 (95% CI, 1.073-7.695), while that for 10-year survival was 3.713 (95% CI, 1.601-8.609) for recipient site extraction due to periodontal disease, 2.190 (95% CI, 1.021-4.700) for recipient site in the maxilla, and 3.110 (95% CI, 1.470-6.581) for fewer than 25 present teeth. In women, the log-rank test indicated experience of less than 10-year in performing treatment as a significant factor (p <0.05) in 5-year survival. These results suggest that medium-term survival in transplanted teeth is influenced by operational risk factors in women, while long-term survival in transplanted teeth is influenced by individual oral status in men.

Predictors of long-term success after successful explantation of continuous flow left ventricular assist device support.

OBJECTIVES: Predictors and evaluations of continuous flow left ventricular assist device (cf-LVAD) explantation in recovered patients remain under discussion due to lack of evidence on long-term safety and efficacy. This study summarized our experiences regarding cf-LVAD explantation in non-ischaemic dilated cardiomyopathy patients and estimated a predictor for sufficient myocardial recovery allowing left ventricular assist device explant. METHODS: We retrospectively identified 135 adult patients with cf-LVAD therapy as bridge to heart transplant due to non-ischaemic dilated cardiomyopathy. Of those, 13 patients underwent device explantation (recovery group) after myocardial recovery. Twelve (92%) of the explanted patients were evaluated using our weaning protocol and underwent surgical explantation. Meanwhile, the remaining 122 continued with cf-LVAD therapy (non-recovery group). RESULTS: Multivariate logistic regression analysis revealed time interval between the first heart failure event and cf-LVAD implantation as an independent predictor for successful explantation. The optimal time interval cutoff value to predict cf-LVAD explantation was 7 months, with a sensitivity of 91.0% and specificity of 84.6%. Echocardiography in patients with successful cf-LVAD explantation showed significant improvement of left ventricular function and dimensions at 6 months postoperatively. The 13 explanted patients are currently alive at a median of 30 (interquartile range; 18-58) months after explantation. The survival rate free from rehospitalization due to heart failure following explantation was 100%. Left ventricular function and remodelling after explantation were also preserved. CONCLUSIONS: In non-ischaemic dilated cardiomyopathy patients with a short interval between the first heart failure event and cf-LVAD therapy, left ventricular myocardium may recover in an early phase after device implantation.

The potential of the transformer-based survival analysis model, SurvTrace, for predicting recurrent cardiovascular events and stratifying high-risk patients with ischemic heart disease.

INTRODUCTION: Ischemic heart disease is a leading cause of death worldwide, and its importance is increasing with the aging population. The aim of this study was to evaluate the accuracy of SurvTrace, a survival analysis model using the Transformer-a state-of-the-art deep learning method-for predicting recurrent cardiovascular events and stratifying high-risk patients. The model's performance was compared to that of a conventional scoring system utilizing real-world data from cardiovascular patients. METHODS: This study consecutively enrolled patients who underwent percutaneous coronary intervention (PCI) at the Department of Cardiovascular Medicine, University of Tokyo Hospital, between 2005 and 2019. Each patient's initial PCI at our hospital was designated as the index procedure, and a composite of major adverse cardiovascular events (MACE) was monitored for up to two years post-index event. Data regarding patient background, clinical presentation, medical history, medications, and perioperative complications were collected to predict MACE. The performance of two models-a conventional scoring system proposed by Wilson et al. and the Transformer-based model SurvTrace-was evaluated using Harrell's c-index, Kaplan-Meier curves, and log-rank tests. RESULTS: A total of 3938 cases were included in the study, with 394 used as the test dataset and the remaining 3544 used for model training. SurvTrace exhibited a mean c-index of 0.72 (95% confidence intervals (CI): 0.69-0.76), which indicated higher prognostic accuracy compared with the conventional scoring system's 0.64 (95% CI: 0.64-0.64). Moreover, SurvTrace demonstrated superior risk stratification ability, effectively distinguishing between the high-risk group and other risk categories in terms of event occurrence. In contrast, the conventional system only showed a significant difference between the low-risk and high-risk groups. CONCLUSION: This study based on real-world cardiovascular patient data underscores the potential of the Transformer-based survival analysis model, SurvTrace, for predicting recurrent cardiovascular events and stratifying high-risk patients.

A leadless pacemaker implantation for a patient with systemic right ventricle under ventricular assist device support.

Patients with congenitally corrected transposition of the great arteries (ccTGA) often develop complete atrioventricular block and heart failure due to the abnormal disposition of atrioventricular node and disadvantage of systemic right ventricle. These issues are managed with a pacing system and a ventricular assist device (VAD), respectively. While technological advances offer new treatment strategies, the simultaneous deployment of a leadless pacemaker and a VAD in cases of ccTGA remains unexplored. Here, we present a case of leadless pacemaker implantation for a VAD-supported ccTGA patient. The safety of a leadless pacemaker for a subpulmonary left ventricle and electromagnetic interference between devices are major concerns when implanting a leadless pacemaker; however, the current case overcomes these obstacles. There were no perioperative complications, and both devices were functioning without problems during a one-year follow up. We expect that, even in patients with cardiac complexity such as systemic right ventricle under VAD support, a leadless pacemaker could become the treatment of choice if the indication is appropriate, although careful and close follow up is needed. Learning objective: Technological advances expand treatment strategies and provide significant benefits to patients with adult congenital heart disease (ACHD). However, discussion of the combination of a leadless pacemaker and a ventricular assist device (VAD) is rare. We demonstrated the efficacy of a leadless pacemaker for a subpulmonary left ventricle in a patient with systemic right ventricle on VAD. This approach could be an option even for ACHD patients.