Multimodal Approach of Pulmonary Artery Intimal Sarcoma: A Single-Institution Experience.

INTRODUCTION: Pulmonary artery sarcoma (PAS) is a rare tumor, whose therapeutic approach is mainly based on surgery, either pneumonectomy or pulmonary endarterectomy (PEA). The prognosis reported in published series is very poor, with survival of 1.5 months without any kind of treatment. PATIENTS AND METHODS: From January 2010 to January 2016, 1027 patients were referred to our hospital for symptoms of acute or chronic pulmonary thromboembolic disease. Twelve patients having a confirmed diagnosis of PAS underwent PEA. Median age was 64.5 years. Most patients had a long history of symptoms, having a median time of 7.5 months from onset of symptoms to surgery. RESULTS: Following PEA and cardiopulmonary rehabilitation, 10 patients received conventional chemotherapy with doxorubicin and ifosfamide, starting at a median of 42 days from surgery. Four patients also received radiotherapy. Four patients have died due to disease progression, while 7 are still alive, with 5 being disease-free at 4-55+ months from diagnosis. CONCLUSIONS: In patients with PAS, a multimodal approach including PEA, CT, and RT is feasible but it should be evaluated individually, according to the tumor extension and the patient's clinical condition. Apart from improving quality of life mainly by reducing or delaying symptoms due to PH, it may improve life expectancy.

Recovery of deambulation after cardiothoracic surgery: a single center experience.

BACKGROUND: Surgical procedure and postoperative bed rest lead to musculoskeletal system alterations with a possibility of new walking dependence of patients who undergo cardiothoracic surgery, which is sometimes associated with prolonged hospitalization and increased health expenditure. AIM: The aim of this study was to assess the postoperative motor disability in inpatients admitted to the cardiothoracic surgical ward, and the results of customized rehabilitation in terms of recovery of postural changes and walking capacity with respect to the preoperative condition and destination after discharge. DESIGN: A prospective observational study was conducted. SETTING AND POPULATION: Four hundred seventeen inpatients, who had undergone cardiothoracic surgery, were enrolled between March 2011 and January 2012 in a Hospital Unit of Cardiothoracic Surgery. METHODS: A computerized system was used to collect data about ambulation at home, type and number of rehabilitation sessions proposed, ambulation at discharge, destination after discharge from ward of origin. All patients, who give their consent, undergone rehabilitative treatment on the ward of origin with an expert physiotherapist. RESULTS: Three hundred seventy-five inpatients were examined in Cardiac Surgery. One patient (0.26%) refused rehabilitative treatment. Two patients (0.53%) died. At the time of discharge 236 (74.45%) patients had recovered the ability to walk independently. After discharge 87.64% of patients was transferred to a specialist ward for intensive rehabilitation. Forty-two inpatients were enrolled in thoracic surgery. Two patients died whilst in hospital. At the time of discharge, 36 patients (94.73%) were able to walk independently. After discharge 80% of patients returned home. CONCLUSION: In our study, the application of an early and simple rehabilitation program on the ward of origin after surgery has made possible the recovery of ambulation of most inpatients who referred independence at home in a few days, limiting hospitalization and health expenditure. CLINICAL REHABILITATION IMPACT: Data about recovery of ambulation with respect to the preoperative condition and destination after discharge resume the importance of identifying motor impairment after surgery, in order to apply an adequate, early and feasible rehabilitation protocol to inpatients, limiting hospitalization and health expenditure.

Hospital survival and long term quality of life after emergency institution of venoarterial ECMO for refractory circulatory collapse.

BACKGROUND: Thanks to significant technical improvements, VA-ECMO is increasingly used to reverse circulatory collapse refractory to standard treatments. METHODS: We studied patients who underwent VA-ECMO due to primary cardiogenic shock or cardiac arrest between January 2008 and June 2011 at our institution. Variables related to hospital survival were analyzed. Long-term survival and health-related quality of life were checked. RESULTS: VA-ECMO was instituted in 23 patients: 17 outpatients and 6 inpatients. Seven of the outpatients were admitted to hospital under ongoing CPR. In these pts, time to CPR was 7 min (6-8) and time to ECMO 93 min (74-107); after 20 hours (16-22), all these pts died. Among remaining 16 pts, 6 were bridged to heart transplant and 4 to heart recovery, 8 survived to hospital discharge and 7 were alive with high health-related quality of life after 46 months (36-54). Ongoing CPR, inotropic score and lactates at cannulation did not differ between survivors and non-survivors; duration of shock, SOFA score and serum creatinine at ECMO institution, and lactates and fluid balance after 36 hours were higher in non-survivors. Patients could be kept on spontaneous breathing for >30% of time while on VA-ECMO. CONCLUSION: Emergency VA-ECMO institution can reverse refractory acute cardiovascular collapse, provided it is carried out before significant organ dysfunction occurs. Light sedation and spontaneous breathing while on VA-ECMO can be well tolerated by patients, but related clinical benefits should be proved. Patients successfully bridged to heart recovery or transplant are candidates for long-term good quality of life.

Use of the Oto lung donor score to analyze the 2010 donor pool of the Nord Italia Transplant program.

INTRODUCTION: The feasibility and utility of a lung donor score that has been recently proposed was tested among a pool of lung donors referred to the Nord Italia Transplant program (NITp) organ procurement organization. MATERIAL AND METHODS: Each lung donor was assigned an Oto score including, age, smoking history, chest X-ray, secretions and ratio of arterial oxygen tension to inspired oxygen fraction (PaO(2)/FiO(2)). Based on clinical compromise, each variable received a score between 0 and 3, except for PaO(2)/FiO(2), which was scored between 0 and 6 given its overall relevance. RESULTS: Throughout 2010, 201 multiorgan donors were initially considered to be potential lung donors. Among these, 59 (29.4%) eventually yielded 67 lung transplantations (named "Used group"). Among the 142 (70.6%) refused lungs, 28 were not used due to logistic or medical problems ("general exclusion" group, GE) and 114, because of poor lung function ("lung exclusion" group, LE). Median lung donor scores were 1 (range, 0 to 3), 4 (range, 2.5 to 6.5), and 7 (range, 5 to 9) in the Used, GE, and LE groups, respectively (one-way analysis of variance, P < .001). Some donors with Oto scores ≤7 worsened over time so that the score had significantly increased by the time of organ retrieval. Overall, subjects who died after lung transplantation were characterized by higher lung donor scores, (2 [1-4] versus 0.5 [0-3], P = .003). CONCLUSION: Our analysis suggested that the use of a donor score as a dynamic tool over the donation process was of great utility to describe and analyze a pool of lung donors.

Incidence of neoplastic disease following lung transplantation: a 17-year single-center experience.

OBJECTIVE: Chronic immunosuppressive therapy following solid organ transplantation has been correlated with an increased risk of posttransplantation neoplastic disease (PTND). In this study we evaluated PTND incidence and outcome at our institution over a 17-year period among patients receiving lung transplantation. MATERIALS AND METHODS: Between February 1992 and December 2008, we performed 290 lung transplantations in 280 patients, including 139 single (48% with 5 retransplantations), and 151 double lung transplantations (52% with 5 retransplantations). Among the 280 patients, 2 had undergone previous double lung transplantation in other hospitals. Follow-up of transplant recipients was performed up to December 2009. RESULTS: Forty-two patients died in the hospital, producing a cumulative early (30-day) mortality rate of 15%. Among the 238 patients discharged from the hospital who entered our follow-up program, 36 (15%) experienced PTND. The mean time between transplantation and diagnosis was 47 ± 42 months, and patients' mean age at time of diagnosis was 55 ± 14 years. Overall freedom from PTND was 97%, 84%, and 73% at 1, 5, and 10 years, respectively. PTND was considered to be the direct cause of death in 11 patients (30%). Overall survival of patients with PTND at five years (45%) did not differ from the remainder of the transplanted population (46%). However, PTND became a relevant cause of death in the long-term (>5 years) follow-up. CONCLUSION: Our experience confirms that PTND was frequently diagnosed following lung transplantation. Even if PTND did not seem to significantly affect the survival of patients undergoing lung transplantation, it may become a significant cause of death among those surviving beyond 5 years.

Preemptive therapy for systemic and pulmonary human cytomegalovirus infection in lung transplant recipients.

The incidence and treatment of both systemic and pulmonary human cytomegalovirus (HCMV) infection as well as HCMV-specific T-cell immune responses were investigated in 57 consecutive lung transplant recipients (LTR) by using as cutoffs for preemptive therapy: 300 000 DNA copies/mL whole blood for systemic infections and 100 000 DNA copies/mL bronchoalveolar lavage fluid for lung infections. Results showed that out of 29/57 LTR (50.9%) needing preemptive antiviral therapy, 15 (51.7%) reached the blood cutoff, 8 (27.6%) the pulmonary cutoff and 6 (20.7%) both the blood and the lung cutoff (3 simultaneously and 3 subsequently). Recovery of HCMV-specific T-cell immune responses was achieved much earlier for CD8+ than CD4+ T cells. However, protection from HCMV reactivation was conferred by the presence of both arms of the T-cell response. In two LTR reaching the pulmonary cutoff and not preemptively treated, a full HCMV-specific CD4+ and CD8+ T-cell response was associated with resolution of lung infection. Antirejection steroid therapy suppressed T-cell immune responses, thus facilitating HCMV reactivation. In conclusion, in LTR, monitoring HCMV infection in both blood and lungs, may improve preemptive therapy efficacy. In addition, monitoring the HCMV-specific T-cell immune response appears useful for predicting control of HCMV infection in the posttransplant period.

Cardiac transplant vasculopathy treated by percutaneous coronary intervention.

AIM: Cardiac transplant vasculopathy is a limit to long-term survival in heart transplantation (H-Tx) recipients. PTCA results in our H-Tx population were retrospectively analyzed. METHODS: From November 1985 to May 2004, 767 patients underwent heart transplantation. All patients received immunosuppressive therapy with cyclosporine or tacrolimus, azathioprine, steroids and mycophenolate mofetil. Lymphocyte was administrated by 3-7 days course of either rabbit antithymocyte globulins or anti-lymphocyte globulins or by a 14 days course of OKT3. Coronary angiograms were performed every year and more frequently if graft vasculopathy was already diagnosed or suspected. RESULTS: Fifty-two coronary artery lesions were treated during 42 percutaneous transluminal cardioangioplasty (PTCA)/stent procedures in 36 patients. Mean time since heart transplantation to PTCA was 80 +/- 27 months. Indication to PTCA was asymptomatic angiographic graft vasculopathy in 34 patients (94%) and acute myocardial infarction in 2 patients (6%). PTCA was performed on left anterior descending artery in 34 cases (65.4%), on circumflex artery in 10 cases (19.2%), on right coronary artery in 8 cases (15.4%). There were no procedure related deaths. None of the patients required emergency bypass surgery. Two patients had transient acute renal failure. Patient follow-up showed 10 deaths after 1 +/- 54 months from PTCA. Six died for progression of graft vasculopathy, three for cancer and one for gastrointestinal bleeding. Two patients underwent heart retransplantation after 20 and 107 months from the first procedure. Mean follow-up of the remaining patients is 78.3 +/- 50.3 months. CONCLUSION: PTCA may represent a reasonable treatment for graft vasculopathy in selected heart transplant recipients.

Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension: short-term functional assessment in a longitudinal study.

AIM: Presently, the surgical treatment choice in chronic thromboembolic pulmonary hypertension (CTEPH) consists in a pulmonary endarterectomy (PEA). The aim of the present study is the functional assessment of patients submitted to PEA both preoperatively and shortly after the intervention. A longitudinal study was developed to study the quality and quantity of functional performance possible in these subjects. METHODS: Twenty-two subjects were assessed immediately prior to PEA and 3 months later in order to obtain quantitative measurements of short-term functional recovery. The functional assessment included the 6-min walk test (6mWT), the measurement of the oxygen percent saturation (HbS%O(2)) and the degree of dyspnea subjectively perceived by each patient. RESULTS: Three months after the surgical intervention, there was a definite increase in the number of meters walked during the 6mWT with respect to preintervention; the difference between the distances walked in the 6mWT (6mWD) in the pre and post-PEA was statistically significant (Paired t-test P<0.001). CONCLUSION: In this study the 6mWT resulted to be a useful tool in the functional evaluation of patients affected by CTEPH and submitted to PEA. The average 6mWD significantly improved already at 3 months after the intervention, thus reaching the minimum limit of the range predicted for the healthy control, but remains lower than the average theoretical value predicted (about 75% of the same).

Post-traumatic pseudoaneurysm of internal mammary artery: a case report.

Pseudoaneurysm of the internal mammary artery can be a rare complication of surgery, particularly post-sternotomy, or determined by a direct trauma, usually a stab wound. This report presents a pseudoaneurysm by a stab, diagnosed by chest computed tomography scan performed for hemothorax recurrence. The patient underwent left thoracotomy in third intercostal space; mammary vessels were identified above and below the pseudoaneurysm sac and tied. The postoperative course was uneventful.

Heterotopic heart transplantation: a single-centre experience.

INTRODUCTION: Orthotopic heart transplantation (OHTx) represents the therapy of choice for end-stage heart disease not treatable with medical or conservative surgical approach. Heterotopic heart transplantation (HHTx) is a surgical procedure in which the graft is connected to the native heart in a parallel fashion and it was especially employed in precyclosporine era. The aim of this paper is to present our experience with HHTx. METHODS: From November 1985 till May 2003, 713 heart transplanted patients included 12 (1.7%) received HHTx. Eleven were male, mean age was 50.7 +/- 5.8 years. Five patients suffered from dilated cardiomyopathy and seven from ischemic cardiomyopathy. Indication for HHTx was: a body size mismatch in 11 cases and availability of a marginal organ in one case. RESULTS: Mean ischemic time was 149 +/- 48 minutes and mean cross-clamp time was 82.3 +/- 19.1 minutes. In four cases left ventricle aneurysm resection was associated with HHTx. Hospital mortality was 8.3% (one patient due to multiorgan failure). The actuarial survival rates were 92% and 64% at 1 and 5 years, respectively. The causes of death were: liver cancer, liver cirrosis, aortic dissection, cerebrovascular accident, and chronic rejection. CONCLUSIONS: In our experience, HHTx survival rate is comparable to OHTx. Because of the scarcity of donors, use of an undersized or marginal graft is a valid option to increase the number of transplanted patients. The major disadvantages of HHTx are the need for anticoagulant therapy, the more difficult hemodynamic and immunologic follow-up, and the presence of the diseased native heart.

Heart transplantation in patients with amyloidosis: single-center experience.

INTRODUCTION: Amyloidosis is a systemic disease. Heart transplantation in this subset of patients is contraindicated by the majority of authors. In our center, patients with heart failure due to amyloidosis have been evaluated for cardiac transplantation since 1991. The aim of this study was to analyze the outcome of these patients waiting for transplant and the effectiveness of this therapy. MATERIALS AND METHODS: Since 1991, eight patients affected by amyloidosis have been evaluated and enrolled on the waiting list for transplant: five affected by AL lambda type; two by APO A1; and one by TTR. Four were transplanted, three died waiting for a donor (two from cardiac failure, one from sudden death), and one has been recently transplanted after 17 months on waiting list. RESULTS: Since 1985, 713 patients underwent heart transplantation in our center, five of whom were affected by amyloidosis (0.7%). Two are still alive (60 and 41 months) without evidence of cardiac amyloidotic infiltration. One patient recently underwent a combined heart-liver transplantation. Two patients died after the intervention: one sudden death after 23 months with amyloidotic infiltration of transplanted heart, and one multiple organ failure (MOF) due to progression of the systemic disease. CONCLUSIONS: Despite the small size of the group preventing us from drawing definitive conclusion, heart transplantation may prevent therapy to arrest organ damage in patients with isolated cardiac involvement. Cardiac events are the main cause of death. Patients must be followed-up for evolution of systemic disease. The midterm survival is encouraging.

"Twinning procedure" in lung transplantation: influence of graft ischemia on survival and incidence of complications.

The limited number of suitable lung donors is the major obstacle to clinical application of lung transplantation. The "twinning procedure" may represent one strategy to optimize the use of the small pool of available grafts. From November 1991 to May 2003, 99 single lung transplants (SLTx) were performed including 46 (46%) cases of the "twinning procedure." We divided the study population into two groups: group A (recipients of the "first" lung) and group B (recipients of the "second" lung). The ischemia time was significantly different (A: 216 +/- 48 minutes, B: 310 +/- 89 minutes, P <.001). Differences were not observed in the incidence of graft failure (A: 2, B: 0, P = NS), in the length of mechanical ventilation (A: 12.8 +/- 29.4 days, B: 7.8 +/- 15.2 days, P = NS), or ICU stay (A: 18.8 +/- 50.6 days, B: 15.2 +/- 17.1 days, P = NS), or of hospitalization (A: 37.8 +/- 56.8 days, B: 31.4 +/- 31.7 days, P = NS). Three bronchial anastomotic complications occurred in each group. The incidence of infections (A: 0.015 events/patient/month, B: 0.011 events/patient/month, P = NS) and of treated acute rejections (A: 0.011 events/patient/month, B: 0.011 events/patient/month, P = NS) was similar in the two groups. One-year survival rates were 86% +/- 7% and 72% +/- 10% in group A and B patients, respectively (P = NS). In our experience the different ischemia times related to the twinning procedure did not increase the mortality or morbidity in the early and midterm period.

Solitary cardiac metastasis from a clear cell carcinoma of the kidney. A case report and review of the literature.

A case of solitary septal metastasis from a clear cell carcinoma of the kidney is reported in a 55-year-old man who 5 years before had undergone right nephrectomy and adrenalectomy. Since then, he had been successfully treated by means of chemotherapy, radiation therapy, or operated on, at almost yearly intervals, for secondary pancreatic, pulmonary and cerebral single metastases. Diagnosis was obtained by routine computed tomography. The septal mass was surgically removed and the patient was discharged on the 4th postoperative day.

Predictors of mortality in patients with Eisenmenger syndrome and admission to the lung transplantation waiting list.

BACKGROUND: Patients with Eisenmenger Syndrome (ES) have very severe irreversible pulmonary hypertension but the criteria for admitting such patients to a lung transplantation waiting list (LTWL) is not clear. Indeed it has been demonstrated that the natural survival of patients with ES is better than the survival achieved through lung transplantation: it follows that no guidelines are available for these patients' admission to an LTWL. The aim of our study was to identify possible predictors of mortality in ES patients in order to reserve admission to the LTWL solely for those patients who would otherwise have the lowest probability of survival. METHODS: Since 1991, 57 patients with ES from our rehabilitative centre were admitted to the LTWL of the Division of Cardiac Surgery at San Matteo Hospital, University of Pavia. At the time of the retrospective analysis, patients were divided into a group of non-transplanted survivors (27 patients--47% of the total) and a group who had died prior to transplantation (16 patients--28% of the total). The 14 transplanted patients (25% of the total) were not considered in the statistical analysis, considering transplantation as an "external event". Unpaired t tests were used to compare the following factors in the survivors and in those who died: sex, "complexity" of the congenital heart disease underlying the ES, previous cardiac surgery, arterial blood gases, pulmonary function and hemodynamic parameters. Moreover, a stepwise discriminant analysis was performed in order to define a possible set of prognostic factors. RESULTS: PaCO2 was higher in those who subsequently died (36.15 +/- 7.42 mmHg) compared with those who survived (32.5 +/- 5.33 mmHg), although this difference did not reach a statistical significance (p = 0.08). Discriminant analysis defined a model in which (a) complexity of the congenital heart disease, (b) sex (male) and (c) cardiac output were predictive of a higher risk of mortality. CONCLUSIONS: This new knowledge can be used in the decision of admission to LTWL in ES patients.

Heart transplantation in chemotherapeutic dilated cardiomyopathy.

Anthracycline cardiotoxicity can induce dilated cardiomyopathy (DCM). Nine patients (four men) experienced postchemotherapy DCM: age at time of tumour diagnosis ranged from 1-45 years (mean 13.5 +/- 19 years); interval time between tumour and HT was 3-23 years (mean 10.8 +/- 6.6) and age at HT ranged from 10-65 years (30.8 +/- 20.1). Interval between end of chemotherapy and beginning of cardiac symptoms was 5.71 +/- 4.6 years. Mean age at DCM diagnosis was 19.2 +/- 19.7 (range 1-50 years). Interval between start of chemotherapy and DCM ranged from 1 month to 10 years (mean 3.15 +/- 3.6 years). Tumours were Ewing sarcoma (7-year-old boy), paratesticular rabdomyosarcoma (1-year-old boy), Wilms tumor with pulmonary metastasis (3-year-old girl), bilateral breast carcinoma (45-year-old woman), uterine leiomyosarcoma (44-year-old woman), acute myelocytic leukemia (1.5-year-old boy and 17-year-old girl), and chronic myelocytic leukemia (5-year-old boy). All patients had high pulmonary resistance values. One patient with chronic myelocytic leukemia (14 year-old at HT) died due to graft failure on the first postoperative day. At follow-up (mean, 80.4 +/- 69.3 months) two patients died: a 32-year-old woman (acute myelocytic leukemia) 1 year after HT for sepsis and a 68-year-old woman who had breast adenocarcinoma recurrence 81 months after HT. The remaining patients are alive, in good condition with no difference in survival from other transplanted patients (P =.757). Patients with end-stage postchemotherapy DCM without evidence of tumour recurrence can safely undergo HT.

Lung transplantation in a Jehovah's witness. Case report in a twinning procedure.

Jehovah's Witnesses, patients who refuse blood transfusions, are generally not considered as candidates for lung transplantation owing to the frequent requirement for transfusions. A successful procedure in a Jehovah's Witness is presented and to our knowledge this is the 2(nd) reported case. The patient, a 38-year-old female, type I diabetes, affected by idiopatic pulmonary fibrosis underwent left lung transplantation. From the same pulmonary bloc a twinning procedure was obtained by means of right lung transplantation in a 58-year-old man affected by the same pathology. Surgical strategies employed in achieving a successful outcome, ethical and moral aspects are discussed.

Pulmonary thromboendarterectomy almost 50 years after the first surgical attempts.

With increased experience in preoperative evaluation, surgical approach and postoperative care of chronic thromboembolic pulmonary hypertension, pulmonary thromboendarterectomy can be performed with an acceptably low risk of death. Most patients, even those in a very compromised state, have excellent, long lasting results.

Influence of underlying lung disease on early postoperative course after single lung transplantation.

BACKGROUND: Single lung transplantation can be a suitable therapeutic option for a wide range of end-stage lung diseases: pulmonary fibrosis, emphysema, primary pulmonary hypertension and Eisenmenger's syndrome. Yet, patients suffering from different diseases have significantly different cardiovascular and respiratory functional profiles that can exert a profound influence on their response to the perioperative procedures. Our purpose is to analyze whether the patient's underlying disease can influence the early postoperative outcome after single lung transplantation. METHODS: We carried out a retrospective analysis on perioperative charts of patients undergoing single lung transplantation during an 8-year period. We focused our attention on the following data: underlying lung disease, age, sex, baseline cardiorespiratory data (pulmonary artery pressure, cardiac index, forced expired volume, vital capacity, arterial blood gases, body mass index), intraoperative data (duration of graft ischemia, use of cardiopulmonary bypass) and indexes of adverse postoperative outcome (in-hospital death, mechanical ventilatory support >7 days). Patients were gathered in 3 groups (restrictive, obstructive and vascular) according to the kind of disease and functional data and the association between disease and outcome was assessed by means of logistic regression analysis. Moreover, we evaluated whether any of the patient's functional parameters could be considered predictive of adverse postoperative outcome. RESULTS: We observed a weak association between restrictive disease and adverse postoperative outcome while, on the other hand, obstructive and vascular forms showed a close association with an adverse outcome, with a borderline statistical significance. Among all the considered variables, only intraoperative use of CPB turned out to be predictive of adverse outcome, while other variables simply indicated a trend towards a better outcome. CONCLUSIONS: Patients with vascular and obstructive diseases have the worst postoperative course, with a higher in-hospital mortality rate and longer duration of ventilation; in particular, the perioperative course of vascular patients is heavily influenced by the intraoperative use of cardiopulmonary bypass.

Plaque composition in plexogenic and thromboembolic pulmonary hypertension: the critical role of thrombotic material in pultaceous core formation.

BACKGROUND: Patients with pulmonary hypertension develop intimal plaques in large pulmonary arteries. OBJECTIVE: To test the hypothesis that the composition of such plaques differs depending on whether the aetiology of the disease is thromboembolic or hypertensive. DESIGN: Chronic thromboembolic and plexogenic pulmonary hypertension (primary and secondary (Eisenmenger syndrome)) were investigated. These are spontaneous human models and were used to examine the independent role of thrombus and hypertension in plaque composition. SETTING: A national tertiary referral centre for lung transplantation and pulmonary thromboendoarterectomy. PATIENTS: Thirty nine patients with chronic thromboembolic pulmonary hypertension who had undergone thromboendoarterectomy (n = 32) or lung transplantation (n = 7), 28 with plexogenic diseases (nine primary and 19 Eisenmenger), and three with Eisenmenger syndrome complicated by thromboembolic events. INTERVENTIONS: The lung and thromboendoarterectomy samples were sectioned, stained with Movat pentachrome, and immunostained with antibodies for fibrin, platelets, inflammatory cells, smooth muscle cells, and erythrocyte membrane glycophorin A. MAIN OUTCOME MEASURE: Composition of the plaques affecting large pulmonary arteries. RESULTS: Two types of intimal lesion were distinguished in chronic thromboembolic pulmonary hypertension: fibrous plaques with angioneogenesis; and core-rich atherosclerotic plaques with pultaceous cores largely consisting of glycophorin immunoreactive material, with cholesterol clefts (61.5%), CD68 positive macrophages (84.6%), T lymphocytes (87%), and calcification (46.1%). The samples from the patients with Eisenmenger syndrome and thromboembolic complications had similar characteristics, whereas those from patients with uncomplicated primary pulmonary hypertension had core-free fibrous plaques, spotted with macrophages and T lymphocytes. CONCLUSIONS: Chronic thromboembolic pulmonary hypertension is associated with atherosclerotic plaques with glycophorin-rich pultaceous cores, and plexogenic pulmonary hypertension with fibrous plaques. Thromboembolic material thus plays a critical role in the formation of pultaceous cores, of which erythrocyte membrane derived glycophorin is a major component.