Stiff left atrial syndrome with pulmonary veins occlusion after percutaneous radiofrequency ablation: a life-long complication that can lead to heart transplantation.

BACKGROUND: Stiff left atrial syndrome (SLAS) and pulmonary vein (PV) occlusion are rare yet potentially major life-long complications after radiofrequency ablation for atrial fibrillation. While mostly controlled by medical management, SLAS can progress to refractory congestive heart failure. Treatment of PV stenosis and occlusion remains a challenging problem with ongoing risk for recurrence regardless of techniques employed. Herein we present the case of a now 51-year-old male with acquired PV occlusion and SLAS who, over the course of eleven years, despite multiple interventions, ultimately required heart transplantation. CASE PRESENTATION: After undergoing three radiofrequency catheter procedures for paroxysmal atrial fibrillation (AF), a hybrid ablation was planned due to reappearance of symptomatic AF. Preoperative echocardiography and chest computed tomography (CT) revealed an occlusion of both left PVs. Furthermore, left atrial dysfunction, high pulmonary artery and pulmonary wedge pressures were diagnosed as well as an important reduction of the left atrial volume. The diagnosis of stiff left atrial syndrome was made. Primary surgical repair of the left-sided PVs was performed using a pericardial patch as a tubular neo-vein, combined with cryoablation in the left and right atrium to treat the patient's arrhythmia. Initial results were favorable, however, after two years the patient experienced progressive restenosis with hemoptysis. Therefore, stenting of the common left PV was performed. Over the years, progressive right heart failure with severe tricuspid regurgitation developed, despite maximal medical therapy, which led to the need for heart transplantation. CONCLUSION: The impact of PV occlusion and SLAS after percutaneous radiofrequency ablation can be lifelong and devastating for the clinical course of the patient. Since the presence of a small left atrium could be an important predictor for SLAS in case of redo ablation, preprocedural imaging should guide the operator to an algorithm of a decision-making containing lesion set, energy source, and safety of re-ablation.

Supracardiac total anomalous pulmonary venous connection type Ib: Morphology and outcomes.

BACKGROUND: Supracardiac total anomalous pulmonary venous connection is the most common subtype of total anomalous pulmonary venous connection. We aimed to describe the morphological spectrum of supracardiac total anomalous pulmonary venous connection and to identify risk factors for death and postoperative pulmonary venous obstruction. METHODS: From February 2009 to June 2019, 241 patients diagnosed with supracardiac-Ia (left-sided vertical vein, n = 185) or supracardiac-Ib (right-sided connection directly to superior vena cava, n = 56) total anomalous pulmonary venous connection underwent initial surgical repair at our institute. Cases with functionally univentricular circulations or atrial isomerism were excluded. Patients' postoperative survival was described by Kaplan-Meier curves. Cox proportional hazards models and competing risk regression models were used to identify clinical risk factors for death and postoperative pulmonary venous obstruction. RESULTS: There were 8 early deaths and 4 late deaths. The overall survivals at 30 days, 1 year, and 10 years were 97.1%, 94.8%, and 94.8%, respectively, in the supracardiac-Ia group (2.7%, 5/185) (hazard ratio, 4.8; P = .003). Five patients required reoperation for pulmonary venous obstruction, including 2 patients who required reintervention for superior vena cava syndromes (all in the supracardiac-Ib group). One patient required superior vena cava balloon dilation for superior vena cava syndromes. Multivariable analysis showed that the supracardiac-Ib group (12.5%, 7/56) had a significantly higher mortality rate than the supracardiac-Ia group (adjusted hazard ratio, 8.5, P = .008). Surgical weight less than 2.5 kg (adjusted hazard ratio, 10.8, P = .023), longer duration of cardiopulmonary bypass (adjusted hazard ratio, 1.15 per 10 minutes, P = .012), and supracardiac-Ib subtype (adjusted hazard ratio, 4.7, P = .037) were independent risk factors associated with death. The supracardiac-Ib subtype (adjusted hazard ratio, 4.8, P = .003) was an incremental risk factor associated with postoperative pulmonary venous obstruction. CONCLUSIONS: Morphological features of supracardiac total anomalous pulmonary venous connection, especially the supracardiac-Ib subtype, were risk factors associated with postoperative pulmonary venous obstruction and survival. Patients with unique anatomic subtypes might require more individualized surgical planning.

Endovascular transatrial stenting of pulmonary vein stenosis after lung transplantation.

Pulmonary vein stenosis (PVS) and pulmonary vein occlusion (PVO) represent rare complications after lung transplantation (LTx), with limited therapeutic options and a high risk of graft loss. We present 2 cases of successful endovascular transatrial stenting following double LTx. A 60-year-old woman with chronic obstructive pulmonary disease who underwent double lobar LTx was diagnosed at postoperative day 72 with a high-grade PVS on the left side. A 22-year-old woman with idiopathic pulmonary arterial hypertension who underwent double LTx was diagnosed 9 days later with PVO of the left upper lobe vein. To avoid surgical reintervention, endovascular transatrial dilatation and stenting were performed successfully in both cases. Transatrial endovascular stenting of PVS or PVO after LTx seems an effective and safe treatment option that should be considered for these life-threatening complications and executed with care.

Anatomical attention-based prediction of postoperative pulmonary venous obstruction via CTA images.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease, with which some patients suffer from postoperative pulmonary venous obstruction (PPVO), requiring particular follow-up strategies and treatments. PPVO prediction has important clinical significance, while building a PPVO prediction model is challenging due to limited data and class imbalance distribution. Inspired by the anatomical evidence of PPVO, which is related to the structure of the left atrium (LA) and pulmonary vein (PV), we design an effective multi-task network for PPVO classification. The proposed method incorporates clinical priors and merits of the segmentation-based network into the classification task. The features learned from segmenting LA and PV are concatenated into the PPVO classification branch to constrain the learning of discriminative features. Anatomical-guided attention is applied in the aggregation of these features to restrict them focusing on TAPVC-related regions. To deal with the imbalance classification problem of PPVO, a novel classification loss derived by masked class activation map (MCAM) is designed to improve the classification performance. Computed tomography angiography (CTA) images of 146 patients diagnosed with supracardiac TAPVC in Shanghai Children's Medical Center and Guangdong Provincial People's Hospital were enrolled in this work. The comprehensive experiments demonstrate the effectiveness and generalization of our proposed method. The automatic PPVO prediction model shows the potential application in helping clinicians develop follow-up strategies, thereby improving the survival rate of TAPVC patients.

[Emergent Operation for Total Anomalous Pulmonary Venous Return].

Total anomalous pulmonary venous return with pulmonary venous obstruction is one of the congenital heart diseases requiring an emergent operation just after birth. The patients with this condition have severe lung congestion and pulmonary hypertension, and cannot survive without relief of pulmonary venous obstruction. During the emergent operation, an appropriate confluence between the common pulmonary venous chamber and left atrium needs to be created under cardiopulmonary bypass. The typical approaches for the anastomosis and the anastomosis techniques are summarized in this article. At the weaning from the cardiopulmonary bypass, the necessary treatments such as nitric oxide or open chest need to be applied. The early surgical outcome for total anomalous pulmonary venous return is referenced from the Japanese National Clinical Database.

Invited commentary: Total anomalous pulmonary venous connection remains a challenging pediatric disease.

Cervantes-Salazar and colleagues report the long-term surgical outcomes of 414 patients with total anomalous pulmonary venous connection (TAPVC) from January 2003 to June 2019. With an overall survival rate of 87.2% from 2003 to 2019, the authors found that an increased mortality risk was associated with infracardiac TAPVC, pulmonary venous obstruction, and postoperative mechanical ventilation. Their comprehensive study with a large sample size of varying age groups, and patients with late referrals for surgery, provide valuable insight into TAPVC surgical outcomes. Improved survival for these patients continues to be a major goal of clinical teams striving to transform treatment paradigms. The promising result of the study reported by Cervantes-Salazar and colleagues gives our field hope for a better future for these patients.

Feasibility of Assessing Cryoballoon Pulmonary Vein Occlusion With Saline Injection and a Novel Mapping System.

Thirty-eight patients had assessment of pulmonary vein occlusion with the dielectric mapping system and injection of saline as an alternative to contrast. Contrast injection was required to ascertain pulmonary vein occlusion in 31.6% (12 of 38) of subjects and 17.4% (27 of 155) of veins. No contrast was required in the last 13 subjects. In this single center study, a novel mapping-guided cryoablation approach appeared to minimize the use of contrast in pulmonary vein isolation for the treatment of atrial fibrillation.

Risk factors for postoperative pulmonary venous obstruction after surgical repair of total anomalous pulmonary venous connection: a systemic review and meta-analysis.

OBJECTIVES: A meta-analysis was performed to investigate the risk factors for postoperative pulmonary venous obstruction (PVO) after surgical repair of total anomalous pulmonary venous connection (TAPVC). METHODS: Data bases including PubMed, Embase, Web of Science and Cochrane Library were searched systematically. The goal was to discuss the risk factors for postoperative PVO after TAPVC. Publications were screened by 2 authors independently for criteria inclusion, methodological quality assessment and data extraction. The Newcastle-Ottawa Scale and the Agency for Healthcare Research and Quality checklist were obtained to assess the quality of the studies. Data were pooled by the random effect model or the fixed effect model according to the heterogeneity test. RESULTS: A total of 16 studies (2,385 participants) were included in the meta-analysis. All included studies were retrospective studies. Six potential risk factors were pooled, 5 of which were significantly associated with postoperative PVO. Patients with preoperative PVO were more likely to suffer from postoperative PVO [odds ratio (OR)=5.27, 95% confidence interval (CI) = (2.75, 10.11), P < 0.01]. Compared with a sutureless procedure, the conventional operative procedure was associated with postoperative PVO [OR = 1.80, 95% CI=(1.20, 2.71), P < 0.01]. A mixed type TAPVC plays a critical role in postoperative PVO [OR = 3.78, 95% CI=(1.08, 13.18), P = 0.04]. Inverse variance analysis showed that longer cardiopulmonary bypass time [hazard ratio (HR)=1.01, 95% CI=(1.01, 1.02), P < 0.00001] and aortic cross-clamp time [HR = 1.01, 95% CI=(1.01, 1.02), P < 0.01] were significantly associated with postoperative PVO. Heterotaxy [OR = 1.18, 95% CI = 0.13, 10.45, P = 0.88] was not statistically significant as a risk factor for postoperative PVO. CONCLUSIONS: This meta-analysis may provide a perspective on the risk factors for postoperative PVO after TAPVC, thus leading to more studies predicting postoperative PVO after TAPVC with our findings.

Total anomalous pulmonary venous connection: 16 years of surgical results in a single center.

PURPOSE: The aim of the study was to analyze the surgical outcome of patients with total anomalous pulmonary venous connection (TAPVC) who underwent cardiac surgery. METHODS: A retrospective study was carried out. Patients with diagnosis of TAPVC undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2003 and June 30, 2019 were included. Descriptive statistics were calculated, as well as a bivariate analysis of the variables associated with mortality. A logistic regression model was included to determine risk factors associated with the main outcome and survival was analyzed using the Kaplan-Meier method. RESULTS: A total of 5314 patients diagnosed with congenital heart disease (CHD) underwent surgery, 414 (7.8%) were patients with TAPVC, with an average age of 17.1 ± 34.6 months, 58.2% were male. It was frequent in infants (61.6%) and preschool (19.6%). Predominant type was supracardiac TAPVC (47.4%). Pulmonary venous obstruction (PVO) occurred in 32.1%. Risk factors associated with mortality were infracardiac TAPVC (odds ratio [OR]: 3.26; 95% confidence interval [CI]: 1.17-9.03; p = .02), PVO (OR: 2.56; 95% CI: 1.05-6.22; p = .03) and postoperative mechanical ventilation (OR: 1.005; 95% CI: 1.002-1.008; p = .01). Overall survival was 87.2%, with better outcomes in adolescents (100%), children (94.1%), mixed TAPVC (96%), and cardiac TAPVC (91.9%; p < .001). CONCLUSIONS: The survival of our institution after surgical correction of TAPVC is similar to that of other referral centers, where patients with infracardiac TAPVC and newborns worse outcomes. All patients must undergo a rigorous evaluation to determine an adequate repair strategy.

Prognosis and related risk factors analysis of total anomalous pulmonary venous connection correction.

OBJECTIVE: This study was performed to analyze the surgical outcomes of our center biventricular correction with total anomalous pulmonary venous connection (TAPVC) and to explore the risk factors associated with postoperative mortality and postoperative pulmonary venous obstruction (PVO). METHODS: In total, 104 patients diagnosed with TAPVC and underwent biventricular correction from January 1, 2009 to December 31, 2021, in Beijing Children's Hospital Affiliated with Capital Medical University were included. The primary endpoints were early and late postoperative mortality and postoperative pulmonary vein obstruction. RESULTS: Multivariable analysis indicated that prolonged cardiopulmonary bypass (CPB) time was the only independent risk factor for early postoperative mortality. Emergency surgery, preoperative moderate, and severe pulmonary hypertension (PH), and prolonged CPB time were independent risk factors for postoperative PVO. According to ROC curve analysis, the cut-off value of CPB time for predicting early mortality was 148 min (AUC = 0.916, 95% CI 0.811-1.000). CONCLUSION: In the past 12 years, with surgical technique and perioperative management advancement, the prognosis of children treated with TAPVC biventricular correction in our center has generally improved. However, surgical repair remains challenging, and early mortality remains high in children with prolonged CPB time during surgery. Postoperative PVO often occurs in children who underwent emergency surgery, combined with moderate and severe PH and prolonged CPB time.

Sutureless Closure Versus Conventional Technique in the Primary Surgery of Total Anomalous Pulmonary Venous Connection: A Systematic Review and Meta-analysis.

Sutureless closure has been used for primary repair of total anomalous pulmonary venous connection (TAPVC) for over 20 years but its superiority over conventional technique is still uncertain. This systematic review was conducted to compare the effectiveness of sutureless closure and conventional surgery as the primary repair for TAPVC. Systematic search was performed in June 2021 on 12 databases. All studies comparing sutureless and conventional surgery for TAPVC were included. The primary endpoints were early mortality, overall mortality, postoperative pulmonary venous stenosis (PVS), and reoperation. Meta-analysis of two-arm studies was performed with several sensitivity and subgroup analyses. Six retrospective studies with 767 patients were included in meta-analyses. Sutureless closure significantly reduced the risk of early mortality, overall mortality, postoperative PVS, and reoperation by 53%, 45%, 77%, and 67% compared to conventional technique, respectively. No heterogeneity was found and presence of publication bias was non-significant. The results were consistent in all sensitivity analyses. Subgroup analyses revealed that sutureless closure was superior to conventional technique in patients with and without preoperative pulmonary venous obstruction, and neonates and non-neonates. Sutureless closure is better than conventional closure as the primary surgery for TAPVC patients. We advocate using sutureless closure for patients with TAPVC. Future large-scale observational studies or clinical trials are required to confirm our findings.

Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.

BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) is a major risk factor in infants with single ventricle (SV). Exact definition of TAPVC anatomy is crucial for surgical planning. AIM: To evaluate the role of cardiac computed tomography (CT) in this setting. METHODS: Retrospective review of 13 infants who underwent TAPVC repair associated with SV from May 2016 to October 2021. Anatomy, incidence, and mechanisms of pulmonary venous obstruction (PVO) were described. Cardiac CT diagnostic yield was compared to echocardiography (echo). RESULTS: Of 13 infants, median age and weight were 24 days (range 2-303 days) and 3.2 (range 2.6-9.1) kg, 8 (62%) were male, 4 (31%) premature, and 11 (85%) had heterotaxy syndrome. All infants had pre- and postoperative echo; 13 had preoperative and 8 (62%) had postoperative cardiac CT. Type of TAPVC: six (46%) supracardiac, two (15%) intracardiac, one (8%) infracardiac, and four (31%) mixed, with pulmonary veins draining in >1 confluence in nine (69%). PVO was present in 6/13 (46%) preoperatively and 5/13 (31%) postoperatively. Mechanisms of PVO: 9/11 (82%) stenosis, 1/9 (9%) membrane formation, and 1/9 (9%) external compression. The sensitivity to diagnose PVO was 45.5% for echo and 100% for cardiac CT, the specificity was 100% for both. No discrepancy was found between cardiac CT and intraoperative findings, but echo had a complete preoperative diagnosis in 1/13 (8%) (p < .00001, Fisher exact test). CONCLUSIONS: Cardiac CT is essential to evaluate pre- and postoperative TAPVC in SV for surgical decision making and long term follow up.

Expanded Polytetrafluoroethylene Patching for Recurrent Pulmonary Venous Obstructions.

Recurrent postoperative pulmonary venous obstruction after total anomalous pulmonary venous connection repair is a challenging complication. Here, we present such a case that required 4 reoperations, focusing on a sutureless method with an expanded polytetrafluoroethylene patch for the fourth reoperation. The use of artificial patch materials to reconstruct the atrial septum and neoatrial roof could be a promising alternative approach of this method to expand its applicability.

Building a Risk Prediction Model for Postoperative Pulmonary Vein Obstruction via Quantitative Analysis of CTA Images.

Total anomalous pulmonary venous connection (TAPVC) is a rare but mortal congenital heart disease in children and can be repaired by surgical operations. However, some patients may suffer from pulmonary venous obstruction (PVO) after surgery with insufficient blood supply, necessitating special follow-up strategy and treatment. Therefore, it is a clinically important yet challenging problem to predict such patients before surgery. In this paper, we address this issue and propose a computational framework to determine the risk factors for postoperative PVO (PPVO) from computed tomography angiography (CTA) images and build the PPVO risk prediction model. From clinical experiences, such risk factors are likely from the left atrium (LA) and pulmonary vein (PV) of the patient. Thus, 3D models of LA and PV are first reconstructed from low-dose CTA images. Then, a feature pool is built by computing different morphological features from 3D models of LA and PV, and the coupling spatial features of LA and PV. Finally, four risk factors are identified from the feature pool using the machine learning techniques, followed by a risk prediction model. As a result, not only PPVO patients can be effectively predicted but also qualitative risk factors reported in the literature can now be quantified. Finally, the risk prediction model is evaluated on two independent clinical datasets from two hospitals. The model can achieve the AUC values of 0.88 and 0.87 respectively, demonstrating its effectiveness in risk prediction.

Review of surgical experience in 61 patients with mixed total anomalous pulmonary venous connection.

OBJECTIVES: Prior studies have reported a high mortality and incidence of post-repair pulmonary venous obstruction (PVO) in mixed total anomalous pulmonary venous connection (TAPVC). This study sought to review the surgical outcomes in this entity. METHODS: A review of 61 patients undergoing surgical repair of mixed TAPVC was conducted. Patients with a single ventricle were excluded. Patients were subdivided into 3 groups according to Chowdhury's classification. Predictors for death and postoperative PVO were explored by Cox regression model. RESULTS: This study trended towards an older cohort with a median age of 88 days (interquartile range, 56.5-177). Twelve patients belonged to '2 + 2' type, 40 belonged to '3 + 1' type and the remaining 9 belonged to bizarre type. There were no early death and 7 late deaths. Follow-up was available in 96.7% of the patients after discharge with a median duration of 53 months (range, 1-177). Nineteen patients developed post-repair PVO among whom 2 required reintervention. Patients with preoperative PVO had a 4-fold higher risk (95% confidence interval, 1.36-12.38) of postoperative PVO than those without and were more likely to die (P = 0.009). No statistical difference was observed among the 3 subgroups in terms of mortality (P = 0.058) and postoperative PVO (P = 0.186). CONCLUSIONS: Preoperative PVO was significantly associated with postoperative PVO. There was no statistical difference in terms of death and postoperative PVO among the 3 subtypes of mixed TAPVC. Mid-term results favoured a complete rechanneling of pulmonary veins in '3 + 1' type.

Total anomalous pulmonary venous connection: Influence of heterotaxy and venous obstruction on outcomes.

BACKGROUND: Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients. METHODS: A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates. RESULTS: A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P < .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P < .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029). CONCLUSIONS: Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort.

Sutureless Repair With Extended Atriotomy for Post-Repair Pulmonary Venous Obstruction.

Post-repair pulmonary venous obstruction is a common cause of reoperation after total anomalous pulmonary venous return repair. Herein, we report 3 cases of specific type of post-repair pulmonary venous obstruction with eccentric stenosis of pulmonary vein ostium due to retained composite neoseptum and the technique used for subsequent repair.

A Modified Approach with Caval Transection for Supracardiac Total Anomalous Pulmonary Venous Connection: Comparison Between Conventional and Sutureless Surgery in 173 Patients.

The efficacy of primary sutureless repair for supracardiac total anomalous pulmonary venous connection (TAPVC) needs to be confirmed. This study aimed to compare the long-term outcomes between the conventional surgery and the sutureless technique with a modified approach in superior TAPVC. Between January 2008 and December 2018, 173 patients with supracardiac TAPVC underwent surgery either with the conventional procedure (n = 130) or the sutureless repair (n = 43). Multivariate analysis and competing-risk analysis were used to identify risk factors for early death and postoperative pulmonary venous obstruction (PVO), respectively. Among 173 patients who underwent repair of supracardiac TAPVC, 46 (28%) had preoperative PVO, and 22 (12.7%) had postoperative PVO. The sutureless group had a lower postoperative PVO rate compared with the conventional group (p = 0.027). The risk factors for death were age ≤ 28 days [odds ratio (OR), 11.56; 95% confidence interval (CI) 1.33-100.47, p = 0.015], weight ≤ 3 kg (OR 9.57; 95% CI 1.58-58.09, p = 0.009), emergency operation (OR 19.24; 95% CI 3.18-116.35, p = 0.002), cardiopulmonary bypass time (OR 2.16; 95% CI 1.36-3.43, p = 0.003), cross-clamp time (OR 1.73; 95% CI 1.20-2.50, p = 0.022), and duration of ventilation (OR 1.11; 95% CI 1.02-1.21, p = 0.027). Age ≤ 28 days [Hazard Ratio (HR) 1.92; 95% CI 1.92-11.02, p < 0.001] and preoperative PVO (HR 41.70; 95% CI 8.15-213.5, p < 0.001) were associated with postoperative PVO. The sutureless repair is a reliable technique for supracardiac TAPVC. Age ≤ 28 days is associated with 30-day mortality and postoperative PVO.

Dyspnoea and diffuse pulmonary nodules in a patient with pulmonary veno-occlusive disease: a case report and literature review.

Pulmonary veno-occlusive disease (PVOD) is a rare type of pulmonary hypertension characterized by capillary damage or arterial pulmonary hypertension. Early lung transplantation is the only effective treatment for PVOD because of the lack of specificity in its clinical manifestations and its rapid progression and poor prognosis. A 28-year-old woman presented with exertional dyspnoea. A chest computed tomography scan revealed diffuse centrilobular ground glass opacities in both lungs, a ratio of the transverse diameter of the main pulmonary trunk to the ascending aorta of >1, and enlargement of the right ventricle and right atrium. A right atrial floating catheter test showed right ventricular pressure of 82/0/4 mmHg, mean pulmonary artery pressure of 83/34/53 mmHg, and pulmonary artery wedge pressure of 15/8/12 mmHg. A mutation was found in the eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) gene. Thus, the patient was diagnosed with PVOD and subsequently given standard bosentan treatment (62.5 mg twice a day). However, after 6 months of follow-up, there was no significant improvement in the pulmonary artery pressure or activity tolerance (6-minute walking test). Therefore, cardiopulmonary transplantation was performed. Early diagnosis and timely treatment of PVOD may improve the patient's prognosis.