[Migration of Internal Pancreaticojejunostomy Stents into the Bile Ducts in Patients Undergoing Pancreaticoduodenectomy].

The patient was a 68-year-old male. Pancreaticoduodenectomy(PD)for papillary carcinoma was performed. Computed tomography(CT)3 months after surgery revealed the migration of a lost Pit-stent into the bile duct. However, there were no symptoms. On CT 2 years after surgery, the lost stent had spontaneously fallen off. However, hepaticolithiasis was observed, and lithotripsy was performed using endoscopic retrograde cholangiopancreatography(ERCP). A pancreatic fistula after PD is an important complication. To prevent pancreatic fistulae, pancreatic stenting at the site of PD is performed in many cases. The postoperative in vivo kinetics of a lost stent remains to be clarified. Several case reports on complications have been published. In this study, we report a patient in whom the migration of a lost Pit-stent into the intrahepatic bile duct after PD led to hepaticolithiasis, and review the literature.

[A Patient with Small Intestinal Leiomyosarcoma and Intussusception Who Underwent Single Port Surgery].

The patient was a 33-year-old female. She was referred to our hospital from a previous clinic with abdominal pain and vomiting. Contrast-enhanced abdominal CT revealed intussusception, leading to a diagnosis of intussusception with a small intestinal tumor as an advanced part. Intestinal obstruction symptoms were observed, and emergency surgery was performed on the same day. As a technique, single port surgery was selected, and laparoscopic reduction was attempted. However, it was ineffective. The site of intussusception was induced outside the body through a small laparotomy wound, and reduction was performed using Hutchinson's procedure. As an adjacent lymph node was markedly swollen, the mesentery involving this lymph node was dissected in a fan shape, and the tumor was extirpated. The tumor measured 40 mm in long diameter, being a hemicircular, protruding lesion. Histologically, disarray of short spindle tumor cells was observed. Immunostaining showed Kit- and DOG1-negtive reactions and partially α-SMA and desmin-positive reactions, suggesting leiomyosarcoma. With the establishment of an immunostaining-test-based classification, leiomyosarcoma is currently rare. In this study, we report a patient in whom single port surgery for intussusception related to small intestinal leiomyosarcoma was successful.

[A Case of Preoperatively Diagnosed Early Cystic-Duct Carcinoma].

A 88-year-old man presented with abdominal discomfort. Computed tomography(CT)images showed gallbladder tumor, and the patient was referred to our hospital. In addition to the above, CT images showed a tense gallbladder and EUS showed papillary raised lesions mainly from the cystic duct to the gallbladder neck. Based on the above, we diagnosed cystic duct cancer and performed full-thickness cholecystectomy, extrahepatic bile duct resection, regional lymph node dissection at our department. Macroscopic findings of the resected specimen showed a Villous ridge in the cystic duct. Histopathological findings revealed well-differentiated adenocarcinoma with an irregular papillary structure centered on the cystic duct. The depth of invasion remained within the epithelium, and a diagnosis of primary early cystic duct cancer was made. Primary cystic duct cancer is a relatively rare disease and often does not lead to preoperative diagnosis. This time, we experienced a case in which cystic duct cancer was diagnosed preoperatively due to complaints of abdominal discomfort and could be surgically resected.

[A Case of Small Intestinal GIST Diagnosed by Intussusception].

The patient was a 34-year-old female. She consulted our hospital with epigastric pain. Abdominal computed tomography (CT)revealed a small intestinal tumor, measuring 30 mm in diameter, with contrast effects. For detailed examination, enteroscopy was scheduled, but abdominal pain suddenly occurred. CT showed marked dilatation of the small intestine and intussusception, and emergency surgery was performed on the same day. Intussusception was observed on the anal side 70 cm from Treitz' ligament. The intestinal wall was black, and fissures of the serosa were partially noted. It was difficult to release the intussusception, and a 55 cm area of the jejunum involving the site of intussusception was resected. The patient was discharged on the 8th postoperative day. At the tip of the intussusception, a submucosal tumor measuring 25 mm in maximum diameter was present. Pathologically, the proliferation of spindle-shaped cells originating from the muscularis propria was observed, comprising an intricate structure. On immunostaining, KIT-positive and CD34, S-100, α-SMA-negative reactions were detected, leading to the diagnosis of a gastrointestinal stromal tumor(GIST). Nuclear divisions were noted in <5/50 visual fields. According to the risk classification, the risk was evaluated as low. The patient is being followed-up in accordance with guidelines.

[Patient with Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma with Small Intestinal Perforation Development during Chemotherapy].

Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is classified under type Ⅱ enteropathy-associated T-cell lymphoma(EATL). It is a rare disease with a low incidence rate. This study reports a case of a patient with MEITL who developed small intestinal perforation during chemotherapy. The patient was a 55-year-old woman who presented to a previous clinic with epigastric pain. Enteroscopy results showed a map-like ulcer in the jejunum. Examination of the tissue specimen collected from this site suggested T-cell lymphoma. The patient was referred to our hospital for chemotherapy. Seven days following the initiation of chemotherapy, an abdominal computed tomography(CT)revealed free air, leading to a diagnosis of gastrointestinal perforation. Emergency surgery was performed. Intraoperatively, bowel perforation and a degenerative ulcer were observed at 95 cm and 80 to 115 cm from the Treitz' ligament, respectively. In addition, all-layer intestinal necrosis was noted 150 and 90 cm from the terminal ileum. Total resection and anastomosis were performed. Postoperatively, the patient developed sepsis due to chemotherapy-related pancytopenia but recovered. She was discharged on postoperative day 24. Subsequently, positron emission tomography(PET)-CT revealed residual intestinal tumor cells and peritoneal dissemination. Chemotherapy was initiated, but there was no response. The patient died after 6.5 months. A radical treatment for MEITL has not yet been established. More case reports are needed to improve the prognosis of this disease.

[An Early Gastric Cancer with Sarcoid Reactions of the Regional Lymph Nodes].

The patient was a 73-year-old male who was referred to our hospital for detailed examination because computed tomography(CT)revealed lymph node swelling. Upper gastrointestinal endoscopy revealed a 0-Ⅱc lesion in the greater curvature of the middle gastric body. The periphery of the lesion site was not reached using endoscopy. CT revealed lymph node swelling, but positron emission tomography(PET)-CT did not show abnormal accumulation in any area other than the lesion site involving the lymph nodes. Under a diagnosis of cT2N0M0, Stage Ⅰ tumor, total gastrectomy via laparotomy and lymph node dissection(D2+No.10)was performed. The histopathological diagnosis suggested early gastric cancer pT1b (SM)N0M0, Stage ⅠA. Although lymph node metastasis was not observed, the outgrowth of non-caseating epithelioid cell granulomas was observed in all lymph nodes. There was no granulomatous lesion at any other site, including the lung, leading to a diagnosis of sarcoid reactions. The"sarcoid reaction"refers to non-caseating epithelioid cell granuloma formation in a local area or the regional lymph nodes of a malignant tumor through reactions to extraneous foreign bodies in the absence of the general condition or signs as sarcoidosis. Sarcoid reactions to early gastric cancer are rare. In this study, we report a patient with early gastric cancer who showed sarcoid reactions of the regional lymph nodes and review the literature.

[A Case of Elective Laparoscopic Resection for Sigmoid Colon Cancer with Irreducible Intussusception].

An 85-year-old man was hospitalized for a right greater trochanteric fracture. Rectal intussusception was found by diagnostic imaging but left untreated because of minor gastrointestinal symptoms. As a result of work-up for persistent mucous stool, he was diagnosed with sigmoid colon cancer with intussusception. The intussusception could not be reduced during barium enema examination but could undergo elective laparoscopic surgery with a good postoperative course. Adult intussusception may be asymptomatic and require no emergency treatment. In such a case, elective surgery can be performed. Many facilities employ laparotomy as a standard of care for intussusception. With the recent technological advances in endoscopic surgeries, laparoscopic surgery can be considered as a treatment option.

Thickness dependent homogeneous crystallization of ultrathin amorphous solid water films.

The crystallization mechanism and kinetics of amorphous materials are of paramount importance not only in basic science but also in the application field because they are closely related to their thermal stability. In the case of amorphous nanomaterials, thermal stability distinctively different from that of bulk materials often emerges. Despite intensive studies in the past, a thorough understanding of the stability at the molecular level has not been reached particularly on how crystallization processes depend on size and are influenced by their surface and interface. In this article, we report the film-size-dependent crystallization of thermally relaxed nonporous ASW ultrathin films on a Pt(111) surface as a benchmark system of amorphous molecular films. The crystallization processes at the surface and interior of the ASW ultrathin films are monitored simultaneously with thermal desorption and infrared reflection absorption, respectively, as a function of the film thickness. Here, we demonstrate that the crystallization is initiated solely by "homogeneous nucleation" irrespective of the film thickness while the crystallization rate remarkably depends on the thickness; the rate of 5-layer (∼1.5 nm) ASW films is one order of magnitude higher than that of 20-layer (∼6 nm) films. Moreover, we found a clear correlation between the film-thickness-dependent crystallization kinetics and microscopic structural disorder associated with the broad distribution of hydrogen-bond lengths between water molecules.

[A Case of Mesenteric Undifferentiated Pleomorphic Sarcoma].

Undifferentiated pleomorphic sarcoma develops in adult soft tissues and has a poor prognosis. It often recurs in the limbs and trunk, but is rare in the mesentery. Complete resection of the tumor is the first-line treatment, and there are previously reported cases of the usefulness of chemotherapy and radiation therapy; however, several factors remain to be clarified. We report a case of undifferentiated pleomorphic sarcoma originating in the ascending mesocolon.

[A Case of Goblet Cell Carcinoid].

The patient was a 26-year-old female who had undergone conservative treatment for acute appendicitis at another clinic and was referred to our hospital for interval appendectomy. We performed a single-incision laparoscopic appendectomy, and the patient was diagnosed with goblet cell carcinoid(GCC)based on the postoperative pathological examination. Since GCC is considered a high-grade tumor, we performed a laparoscopic ileocolic resection with D3 lymphadenectomy. There was no residual disease or lymph node metastasis detected in the resected specimen. Patients with advanced GCC typically have poor prognosis, because GCC is characterized by peritoneal dissemination and lymph node metastasis. However, our findings suggested that an additional laparoscopic surgery could be one of the curative and safe treatment options for selected pa- tients with GCC.

[Resection of Synchronous Liver Metastasis from Ascending Colon Cancer with Aplastic Anemia-A Case Report].

The aplastic anemia(AA)syndrome is characterized by pancytopenia and bone marrow hypoplasia. Although anemia, bleeding tendency, and susceptibility to infection are issues of concern during surgery, few reports have been published on the perioperative management, and management methods have not been established. A 77-year-old woman visited our hospital with chief complaints of melena and fatigability. Marked pancytopenia was observed at the first visit. After a detailed examination, she was diagnosed with ascending colon cancer accompanied by AA and solitary liver metastasis. As AA responded poorly to treatment, without improvement in pancytopenia, we decided to perform colectomy. The perioperative management, including blood transfusion and administration of a G-CSF preparation, was performed in collaboration with a hematologist, followed by right hemicolectomy and hepatic lateral segmentectomy. She was transferred to the department of hematology on hospital day 8 without complications. In conclusion, a highly invasive surgery, as in the present case, can be performed safely with an appropriate perioperative management even in cases complicated by AA.

[Spontaneous Disappearance of Undifferentiated Pleomorphic Primary Mesenteric Sarcoma-A Case Report].

The patient was a 49-year-old man with persistent fever since the introduction of hemodialysis(HD). Vomiting and abdominal swelling appeared 4 months after initiating hemodialysis. Computed tomography(CT)scan revealed a tumor measuring 9 cm, and disorders of passage from the jejunum. Surgery was performed, and resection was impossible because of peritoneal dissemination. Histopathological examination of the disseminated nodes suggested an undifferentiated pleomorphic sarcoma. Postoperatively, drainage from the gastric fistula was approximately 2,000mL/day. Chemotherapy was considered impossible because of HD, and palliative therapy was selected. However, the volume of drainage from the gastric fistula gradually decreased, and the disorders of passage reduced. CT scan confirmed marked reduction in the size of the intraperitoneal tumor and its subsequent disappearance. At the 2-year-and-5-month postoperative follow-up, no relapses were observed, and the course had been uneventful. Undifferentiated pleomorphic sarcomas develop in the soft tissue of adults and have a poor prognosis. However, mesenteric development is rare. Total tumorectomy is the first choice of treatment. A consensus on the usefulness of chemotherapy or radiotherapy has not been reached. Furthermore, no studies have reported spontaneous tumor disappearance in the absence of treatment. Here, we report a case of minor undifferentiated primary mesenteric sarcoma and its spontaneous disappearance and review the literature.

Long-Term Outcome of Chronic Thromboembolic Pulmonary Hypertension at a Single Japanese Pulmonary Endarterectomy Center.

BACKGROUND: Several new treatments for chronic thromboembolic pulmonary hypertension (CTEPH) have appeared in recent years, which have led to changes in the treatment algorithm. Changes in survival rates and prognostic factors, however, have not been estimated so far.Methods and Results:Two hundred and eighty patients were diagnosed with CTEPH at Chiba University Hospital between June 1986 and June 2016. Survival rate was investigated by date of treatment initiation (group 1, 1986-1998; group 2, 1999-2008; group 3, 2009-2016). Survival rates were also evaluated by treatment strategy: balloon pulmonary angioplasty (BPA), pulmonary endarterectomy (PEA), and medical treatment. Group 3 had significantly better disease-specific survival than groups 1 and 2 (5-year survival: 91.9% vs. 67.1%, 77.0%, respectively). For the non-PEA (BPA+medication) strategy, group 3 had better disease-specific survival than groups 1 and 2 (5-year survival: 94.9% vs. 54.6%, 74.2%, respectively). The PEA strategy had significantly better survival than the medication strategy in groups 1 and 2, whereas no difference was observed between the BPA, PEA, and medication strategies in group 3. CONCLUSIONS: Survival in CTEPH in the recent era has significantly improved, especially in non-PEA patients. BPA and selective pulmonary vasodilators could improve survival in the non-PEA group. In the present study, no difference in survival was found between PEA and non-PEA.

Endothelial cell-related autophagic pathways in Sugen/hypoxia-exposed pulmonary arterial hypertensive rats.

Pulmonary arterial hypertension (PAH) is characterized by progressive obstructive remodeling of pulmonary arteries. However, no reports have described the causative role of the autophagic pathway in pulmonary vascular endothelial cell (EC) alterations associated with PAH. This study investigated the time-dependent role of the autophagic pathway in pulmonary vascular ECs and pulmonary vascular EC kinesis in a severe PAH rat model (Sugen/hypoxia rat) and evaluated whether timely induction of the autophagic pathway by rapamycin improves PAH. Hemodynamic and histological examinations as well as flow cytometry of pulmonary vascular EC-related autophagic pathways and pulmonary vascular EC kinetics in lung cell suspensions were performed. The time-dependent and therapeutic effects of rapamycin on the autophagic pathway were also assessed. Sugen/hypoxia rats treated with the vascular endothelial growth factor receptor blocker SU5416 showed increased right ventricular systolic pressure (RVSP) and numbers of obstructive vessels due to increased pulmonary vascular remodeling. The expression of the autophagic marker LC3 in ECs also changed in a time-dependent manner, in parallel with proliferation and apoptotic markers as assessed by flow cytometry. These results suggest the presence of cross talk between pulmonary vascular remodeling and the autophagic pathway, especially in small vascular lesions. Moreover, treatment of Sugen/hypoxia rats with rapamycin after SU5416 injection activated the autophagic pathway and improved the balance between cell proliferation and apoptosis in pulmonary vascular ECs to reduce RVSP and pulmonary vascular remodeling. These results suggested that the autophagic pathway can suppress PAH progression and that rapamycin-dependent activation of the autophagic pathway could ameliorate PAH.

Prognostic and pathophysiological marker for patients with chronic thromboembolic pulmonary hypertension: Usefulness of diffusing capacity for carbon monoxide at diagnosis.

BACKGROUND AND OBJECTIVE: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease in some patients, despite improved treatments. Microvasculopathy has been implicated in the poor outcomes of patients with CTEPH. A reduction in the diffusing capacity for carbon monoxide (DLCO ) was previously suggested to indicate microvasculopathy in CTEPH patients; therefore, we assessed DLCO /alveolar ventilation (DLCO /VA ) as a prognostic and pathophysiological marker in CTEPH. METHODS: We performed a retrospective cohort study of 214 CTEPH patients consecutively diagnosed between 1986 and 2011. After exclusion of 24 patients because of missing DLCO data or severe obstructive impairment, the mortality rates of medically treated patients classified with normal or decreased DLCO values were compared, and prognostic factors were determined. The relationship between long-term surgical outcomes and DLCO /VA was also investigated. RESULTS: Ninety-one inoperable patients were treated medically, two of whom underwent balloon pulmonary angioplasty. Ninety-nine underwent pulmonary endarterectomy. The 5-year survival rate of medically treated patients was significantly lower in patients with decreased DLCO /VA than in those with normal values (61.4% vs 90.4%, P = 0.017). Decreased preoperative DLCO /VA was associated with a smaller percent decrease in post-operative pulmonary vascular resistance, but not with the extent of proximal thrombi; these results may support our hypothesis that DLCO reflects microvascular involvement. CONCLUSION: Decreased DLCO /VA was associated with poor outcomes of medically treated CTEPH patients; and may be useful for identifying high-risk patients, potentially leading to earlier and more appropriate interventions.

[A Case of Successful Collective Treatment of Colon Cancer with Multiple Metachronous Pulmonary Metastases].

A case is a 46-year-old woman visited us with a chief complaint of bloody stools. A diagnosis of rectal cancer(Rs)was made, and laparoscopic resectomy plus D3 was performed. After progressing to pT4a(SE)N2, M0, pStage III b, postoperative adjuvant chemotherapy(6 courses of XELOX)was administered. Two months after initiating chemotherapy, since the CEA value increased, chest abdominal CT was performed. Five nodules were found in the bilateral lungs and diagnosed as lung metastases(PUL2). Systemic chemotherapy(IRIS plus BV)is administered to PUL2(Grade C)of rectal cancer metachronous metastases. After 3 courses, the effect judgment was SD. Based on the recurrence period from postoperative adjuvant chemotherapy and the findings during this time, it was judged that weight loss surgery was appropriate for the rectal cancer lung metastatic lesions in which chemotherapy was ineffective, and partial resection of both lungs under thoracoscopic assistance was performed. Systemic chemotherapy(TAS-102 plus BV)was initiated to prevent postoperative recurrence. The patient is currently alive without relapse after 12 months. We reported a case of metachronous metastasis of colon cancer in which multidisciplinary treatment was successful.

Endothelial-to-mesenchymal transition in lipopolysaccharide-induced acute lung injury drives a progenitor cell-like phenotype.

Pulmonary vascular endothelial function may be impaired by oxidative stress in endotoxemia-derived acute lung injury. Growing evidence suggests that endothelial-to-mesenchymal transition (EndMT) could play a pivotal role in various respiratory diseases; however, it remains unclear whether EndMT participates in the injury/repair process of septic acute lung injury. Here, we analyzed lipopolysaccharide (LPS)-treated mice whose total number of pulmonary vascular endothelial cells (PVECs) transiently decreased after production of reactive oxygen species (ROS), while the population of EndMT-PVECs significantly increased. NAD(P)H oxidase inhibition suppressed EndMT of PVECs. Most EndMT-PVECs derived from tissue-resident cells, not from bone marrow, as assessed by mice with chimeric bone marrow. Bromodeoxyuridine-incorporation assays revealed higher proliferation of capillary EndMT-PVECs. In addition, EndMT-PVECs strongly expressed c-kit and CD133. LPS loading to human lung microvascular endothelial cells (HMVEC-Ls) induced reversible EndMT, as evidenced by phenotypic recovery observed after removal of LPS. LPS-induced EndMT-HMVEC-Ls had increased vasculogenic ability, aldehyde dehydrogenase activity, and expression of drug resistance genes, which are also fundamental properties of progenitor cells. Taken together, our results demonstrate that LPS induces EndMT of tissue-resident PVECs during the early phase of acute lung injury, partly mediated by ROS, contributing to increased proliferation of PVECs.

Coagulation-Fibrinolysis System and Postoperative Outcomes of Patients With Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: The postoperative changes in the coagulation-fibrinolysis system and the association between the system and postoperative course of patients with chronic thromboembolic pulmonary hypertension (CTEPH) who have undergone pulmonary endarterectomy (PEA) remain unclear. METHODS AND RESULTS: Between 1986 and 2013, 117 patients (55.1±11.2 years, preoperative mean pulmonary arterial pressure 46.5±10.5 mmHg) underwent PEA, and 15 patients died during the perioperative period. We studied the association between the preoperative coagulation-fibrinolysis markers and surgical outcomes of all patients, and the long-term outcomes of the 102 survivors from the date of PEA. We also investigated the postoperative changes in coagulation-fibrinolysis markers and their association with residual pulmonary hypertension (PH) in 20 consecutive patients. Only an elevated factor VIII level was associated with perioperative death. Thrombomodulin and plasminogen values were significantly increased after PEA. Univariate logistic regression analysis revealed that D-dimer positivity at follow-up was a risk factor for residual PH. Patients with both an elevated fibrinogen level (≥291 mg/dl [median]) and decreased plasminogen activity (<100% [median]) had significantly worse disease-specific survival than the other patients (5-year disease-specific survival: 84.0% vs. 100%, respectively; P=0.0041 [log-rank test]). CONCLUSIONS: Preoperatively high fibrinogen and low plasminogen values in patients with CTEPH are associated with poor long-term postoperative outcome. PEA benefited not only the pulmonary hemodynamics but also the coagulation-fibrinolysis system of patients.

Synergistic Effects of ACE Insertion/Deletion and GNB3 C825T Polymorphisms on the Efficacy of PDE-5 Inhibitor in Patients with Pulmonary Hypertension.

BACKGROUND: The insertion/deletion (I/D) polymorphism in the angiotensin-converting enzyme gene (ACE) and the C825T polymorphism in the G-protein ß3 subunit gene (GNB3) are associated with the efficacy of phosphodiesterase-5 inhibitor (PDE-5I) in erectile dysfunction. In addition, GNB3 genotypes could be associated with clinical worsening in pulmonary hypertension (PH) treated with PDE-5I. However, no studies have described the synergistic effects of gene polymorphisms on drug efficacy in patients with PH. OBJECTIVES: We aimed to examine the effects of combined ACE/GNB3 polymorphisms on the efficacy of PDE-5I in patients with PH. METHODS: This was a retrospective uncontrolled study. Ninety patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic PH (CTEPH) were treated with PDE-5I. Freedom from clinical worsening and pre- and post-treatment parameters, including the 6-min walk distance (6MWD) and serum brain natriuretic peptide (BNP) levels, were compared between patients with ACE/GNB3 II/TT and non-II/TT genotypes. RESULTS: Time to clinical worsening was significantly longer in patients with the II/TT genotype than in those with the non-II/TT genotype (5-year freedom from clinical worsening: 100 vs. 48.8%, respectively; p = 0.018), even in patients with CTEPH alone. Post-treatment 6MWD and BNP levels in patients with the II/TT genotype tended to be better than those in patients with the non-II/TT genotype. The ACE/GNB3 genotype was a significant predictor of clinical worsening, even after adjusting for pulmonary vascular resistance and 6MWD. CONCLUSIONS: ACE and GNB3 polymorphisms may synergistically influence the efficacy of PDE-5I in patients with PH.

Effects of Surgical and Medical Treatment on Quality of Life for Patients With Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: This study aimed to investigate the predictors of quality of life (QOL) in patients with chronic thromboembolic pulmonary hypertension (CTEPH), changes in QOL after surgical and medical treatments, and the relationship between baseline QOL and survival. METHODS AND RESULTS: QOL was measured in 128 patients with CTEPH (male/female: 42/86, age: 56±12 years, surgical/medical: 65/63) using the Short-Form 36 (SF-36) questionnaire. Multiple regression analysis showed pulmonary vascular resistance (PVR) and 6-min walking distance (6MWD) were associated with physical functioning (PF) (P<0.01) and physical component summary (PCS) (P<0.01). In the surgical group, 7 subscales and 2 summary scores improved significantly, and in the medical group 6 subscales and the mental component summary, although the change in QOL was greater in the surgical group. The patients in the conventional therapy group with higher PF had significantly better survival than those with lower PF (5-years survival: 89.5% vs. 50.8%, P=0.002). This difference in survival was not observed in the group receiving pulmonary arterial hypertension (PAH)-specific therapy (100% vs. 100%, P=0.746). CONCLUSIONS: PVR and 6MWD were associated with PF or PCS in CTEPH patients. QOL improved after surgical or medical therapy, with a greater change in the surgical group. PAH-specific therapy improved survival in patients with lower PF at diagnosis.