Pulmonary veno-occlusive disease: illustrative cases and literature review.

Pulmonary veno-occlusive disease (PVOD), also known as "pulmonary arterial hypertension (PAH) with overt features of venous/capillary involvement", is a rare cause of PAH characterised by substantial small pulmonary vein and capillary involvement, leading to increased pulmonary vascular resistance and right ventricular failure. Environmental risk factors have been associated with the development of PVOD, such as occupational exposure to organic solvents and chemotherapy, notably mitomycin. PVOD may also be associated with a mutation in the EIF2AK4 gene in heritable forms of disease. Distinguishing PVOD from PAH is critical for guiding appropriate management. Chest computed tomography typically displays interlobular septal thickening, ground-glass opacities and mediastinal lymphadenopathy. Life-threatening pulmonary oedema is a complication of pulmonary vasodilator therapy that can occur with any class of PAH drugs in PVOD. Early referral to a lung transplant centre is essential due to the poor response to therapy when compared with other forms of PAH. Histopathological analysis of lung explants reveals microvascular remodelling with typical fibrous veno-occlusive lesions. This review covers the main features distinguishing PVOD from PAH and two clinical cases that illustrate the challenges of PVOD management.

Recanalization of a Chronic Total Pulmonary Vein Occlusion Occurring after Transcatheter Atrial Fibrillation Ablation.

Pulmonary vein occlusion (PVO) is a known complication of radiofrequency ablation for atrial fibrillation. We present a case with delayed presentation leading to chronic total PVO. Computed Tomography (CT) imaging did not predict the presence of residual flow. Despite this, the occlusion was successfully stented using wire escalation techniques adapted from chronic total occlusion coronary angioplasty, with resolution of symptoms. This emphasises the importance of combining CT with invasive angiography for patient selection and interventional strategy. Innovative angioplasty techniques used to overcome PVO need to be balanced against additional risk of perforation when dealing with extra-cardiac structures.

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.

Atrial Fibrillation Ablation-induced Pulmonary Venous Occlusion Requiring Pneumonectomy.

A 71-year-old woman with a history of atrial fibrillation underwent a catheter-based ablation procedure. Months later, she presented with dyspnea and a left-sided pleural effusion. Diagnostic evaluation revealed left-sided pulmonary venous occlusion, with essentially absent left lung perfusion. The patient underwent left pneumonectomy, with left atrial appendage occlusion. Although lobectomy for pulmonary venous occlusion of a single vein after pulmonary vein isolation has been described, this appears to be a novel report of occluded pulmonary venous drainage of an entire lung necessitating pneumonectomy.

Pulmonary Veno-occlusive Disease that Developed Following Hematopoietic Stem Cell Transplantation for Acute Myeloid Leukemia.

We herein report a case of pulmonary veno-occlusive disease (PVOD) induced by allo-hematopoietic stem cell transplantation (HSCT) in a 48-year-old man who was diagnosed with acute myeloid leukemia. Five months after transplantation, he developed dyspnea and was diagnosed with pulmonary hypertension based on right heart catheterization. Although he received treatment with pulmonary vasodilators, diuretics, and corticosteroids, his pulmonary artery pressure did not decrease, and his pulmonary edema worsened. Based on the clinical course, hypoxemia, diffusion impairment, and computed tomography findings, the patient was diagnosed with HSCT-related PVOD. Critical attention should be paid to dyspnea after HSCT for the early diagnosis of PVOD.

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.

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.

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.

Case report: pulmonary veno-occlusive disease: a rare but fatal cause of pulmonary hypertension in a patient following allogeneic stem cell transplantation.

Pulmonary veno-occlusive disease (PVOD) represents a rare and challenging form of pulmonary hypertension, characterized by preferential remodelling of the pulmonary venules. PVOD may be idiopathic as well as related to other conditions with environmental and genetic factors contributing to its development. Recently, bi-allelic mutations in the EIF2AK4-gene have been identified as a cause of heritable PVOD. PVOD shares an overlapping disease phenotype with pulmonary arterial hypertension (PAH) and is regularly misdiagnosed as such, although differentiation between these two conditions is important because of the different prognosis and therapeutic approach. The diagnosis of PVOD is frequently delayed because of the complex diagnostic process. The gold standard remains histology with widespread obliteration of small pulmonary veins by fibrous intimal thickening and patchy capillary proliferation as the pathological hallmark. At present, neither treatment guidelines nor curative medical therapies are available for PVOD. Because of the progressive nature of the disease, a quick referral for lung transplantation remains the only definitive therapy in subjects below the age of 65.

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.

Pulmonary vasodilators can lead to various complications in pulmonary "arterial" hypertension associated with congenital heart disease.

Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-ranging pathological mechanism in PAH could elucidate the clinical state of severe CHD-PAH. Using electronic medical records, we retrospectively analyzed six infants with severe CHD-PAH who had treatment-resistant PH. All patients were born with congenital malformation syndrome. After starting on a pulmonary vasodilator, five of the six patients developed complications including pulmonary edema and interstitial lung disease (ILD), and four patients had alveolar hemorrhage. After steroid therapy, the clinical condition improved in four patients, but two patients died. The autopsy findings in one of the deceased patients indicated the presence of recurrent alveolar hemorrhage, pulmonary venous hypertension, ILD, and PAH. Based on the clinical course of these CHD-PAH in patients and the literature, CHD-PAH can occur with pulmonary vascular obstructive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH), ILD, and/or alveolar hemorrhage. The severity of CHD-PAH may depend on a genetic disorder, respiratory infection, and upper airway stenosis. Additionally, pulmonary vasodilators may be involved in the development of PVOD/PCH and ILD. When patients with CHD-PAH show unexpected deterioration, clinicians should consider complications associated with PVOD/PCH and/or pulmonary disease. In addition, the choice of upfront combination therapy for pediatric patients with CHD-PAH should be selected carefully.

Venoocclusive Disease With Both Hepatic and Pulmonary Involvement.

Pulmonary venoocclusive disease (PVOD) is a rare form of pulmonary vascular disease with pulmonary hypertension characterized by preferential involvement of the pulmonary venous system. Hepatic venoocclusive disease (HVOD), also known as sinusoidal obstruction syndrome, is a condition that occurs in 13% to 15% of patients after hematopoietic stem cell transplantation (HSCT). Although hepatic and pulmonary venoocclusive diseases may share some pathologic features as well as some etiologies such as HSCT, these two disorders have never been described together in a single adult patient. We report the case of a patient who received HSCT and developed HVOD and PVOD within 9 months. Despite their differences, PVOD and HVOD share common risk factors and associated conditions, suggesting that in the context of HSCT, the two diseases share common pathophysiological mechanisms. Optimal treatment for HSCT-related PVOD remains to be determined.