Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension with bronchial obstruction by a carcinoid tumor.

Pulmonary endarterectomy (PEA) is a standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH combined with bronchial obstruction by a tumor is rare but should be assessed carefully because PEA for obstructed segments can be less therapeutic and make the subsequent surgical resection challenging. This report describes a case of CTEPH with bronchial obstruction by a typical carcinoid tumor in a 75-year-old man. On-site evaluation and removal of the obstructive tumor were performed bronchoscopically, increasing the effectiveness of subsequent PEA for all affected pulmonary segments. This report illustrates a PEA strategy to treat CTEPH with bronchial tumor obstruction.

Time Trajectory of Pulmonary Artery Involvement in Takayasu Arteritis Confirmed by Histopathology.

The early diagnosis of Takayasu arteritis (TAK) remains challenging when TAK-related pulmonary artery involvement occurs as an isolated or initial clinical manifestation. We describe the long-term course of a patient with isolated pulmonary TAK who was initially diagnosed with pulmonary arterial hypertension with in situ thrombus but was re-diagnosed following thromboendarterectomy surgery. (Level of Difficulty: Beginner.).

Effectiveness of balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension despite having lesion types suitable for surgical treatment.

BACKGROUND: Balloon pulmonary angioplasty (BPA) has been performed in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, some patients have inoperable CTEPH despite having lesions suitable for surgical treatment. The effectiveness of BPA in such cases is unclear. The aim of this study was to clarify the effectiveness of BPA in these cases. METHODS: We retrospectively investigated patients with inoperable CTEPH and divided them into two groups: BPA-suitable and BPA-unsuitable groups based on the findings of pulmonary angiography, computed tomography, and perfusion scintigraphy. The BPA-unsuitable group included patients whose lesions are suitable for surgical treatment but who did not undergo the procedure for any specified reason. We analyzed the hemodynamic, respiratory, and functional status of the patients before and after BPA. RESULTS: Forty-three consecutive patients with inoperable CTEPH (age, 62.6 ±â€¯13.5 years; 31 women) were included; all of them underwent BPA. There were 10 patients in the BPA-unsuitable group. In all patients, the mean pulmonary artery pressure, pulmonary vascular resistance, arterial oxygen saturation level, and 6-min walking distance significantly improved after BPA (mean pulmonary artery pressure, from 43.3 ±â€¯7.8 mmHg to 23.9 ±â€¯4.7 mmHg; pulmonary vascular resistance, from 924.1 ±â€¯462.2 dynes/s/cm-5 to 319.7 ±â€¯163.8 dynes/s/cm-5; arterial oxygen saturation level, from 89.3 ±â€¯4.3% to 93.4 ±â€¯3.3%; 6-min walking distance, from 370.0 ±â€¯107.4 m to 443.8 ±â€¯101.4 m). Notably, none of the parameters significantly differed between the groups after BPA. Importantly, the amount of lung bleeding did not differ between them. However, several sessions were required in the BPA-unsuitable group (BPA-unsuitable group: six sessions vs. BPA-suitable group: four sessions). CONCLUSIONS: BPA safely improved the hemodynamic and functional statuses of the patients with CTEPH who are judged as inoperable for any reason despite lesion being suitable for surgical treatment. However, numerous BPA sessions were required in these patients.

Predictors of Outcomes After Surgery for Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: Pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) is technically demanding. We tried to identify the predictors for short- and long-term outcomes after PEA for CTEPH with aggressive use of pulmonary vasodilators, including epoprostenol sodium. METHODS: From 2005 to 2013, 122 CTEPH patients, whose preoperative mean pulmonary artery pressure (mPAP) was 47 ± 10 mm Hg and pulmonary vascular resistance was 847 ± 373 dynes/s/cm5, underwent PEA with hypothermic circulatory arrest. Before PEA, all patients underwent pulmonary vasodilator therapy, including epoprostenol sodium of 2 to 6 ng/kg/min. We collected the perioperative and follow-up data retrospectively to identify the predictors for early and late outcomes after PEA. RESULTS: In-hospital mortality was 7.4% (n = 9). Predictors for in-hospital death were age older than 65 years and New York Heart Association Functional Classification IV. Among the 113 PEA survivors, the mPAP and pulmonary vascular resistance significantly decreased. After the median follow-up of 6.8 years, the overall survival rates were 91.8%, 89.2%, 89.2%, 89.2%, and 86.1%, and the cardiac events-free rates were 100%, 98.1%, 95.8%, 85.5%, and 49.0%, at 1, 3, 5, 7, and 10 years, respectively, in the Kaplan-Meier model. A multivariate Cox proportional hazard model identified postoperative mPAP exceeding 30 mm Hg as the only predictor for late cardiac events. CONCLUSIONS: Early and late outcomes of PEA for CTEPH with perioperative aggressive pulmonary vasodilator treatment seem satisfactory. However, residual pulmonary hypertension remains challenging to achieve further improvement of late outcomes.

Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: Results of a Multicenter Registry.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for pulmonary endarterectomy-the standard therapy. Currently, most reported results of BPA are from relatively small cohorts treated at single centers. The present study evaluated the safety and efficacy of BPA for chronic thromboembolic pulmonary hypertension based on a multicenter registry. METHODS AND RESULTS: A total of 308 patients (62 men and 246 women; mean age, 61 years) underwent 1408 procedures at 7 institutions in Japan. Data were retrospectively reviewed to evaluate clinical outcome and complications. Hemodynamics were significantly improved in 249 patients in whom BPA was terminated, most often because of improvement in mean pulmonary arterial pressure or symptomatic improvement after 1154 procedures. In 196 patients who underwent follow-up right heart catheterization, improvement of hemodynamic parameters was maintained. Mean pulmonary arterial pressure decreased from 43.2±11.0 to 24.3±6.4 mm Hg after final BPA and 22.5±5.4 mm Hg at follow-up, with significant reduction of concomitant use of pulmonary hypertension-targeted therapy and oxygen supplementation. Complications occurred in 511 (36.3%), including pulmonary injury (17.8%), hemoptysis (14.0%), and pulmonary artery perforation (2.9%). Twelve patients (3.9%) died during follow-up, including 8 patients who died within 30 days after BPA. The leading causes of death were right heart failure, multiorgan failure, and sepsis. Overall survival was 96.8% (95% confidence interval, 93.7%-98.4%) at 1 and 2 years and 94.5% (95% confidence interval, 89.3%-97.3%) at 3 years, respectively, after the initial BPA procedure for all 308 patients. CONCLUSIONS: This multicenter registry suggested improved hemodynamic results after BPA. Complication rates were high, but overall survival was comparable with pulmonary endarterectomy. BPA may be an important therapeutic option in patients with chronic thromboembolic pulmonary hypertension.

Additional percutaneous transluminal pulmonary angioplasty for residual or recurrent pulmonary hypertension after pulmonary endarterectomy.

BACKGROUND: Pulmonary endarterectomy (PEA) has been the most effective therapy for chronic thromboembolic pulmonary hypertension (CTEPH). However, residual or recurrent pulmonary hypertension often persists after PEA. Recently, catheter-based angioplasty, called percutaneous transluminal pulmonary angioplasty (PTPA) or balloon pulmonary angioplasty, has been developed as a promising strategy for CTEPH. Therefore, the usefulness of PTPA for residual or recurrent pulmonary hypertension after PEA was investigated. METHODS: Thirty-nine patients underwent PEA from January 2000, and a total of 423 consecutive PTPA sessions in 110 patients were performed from January 2009 to May 2014. Of them, 9 patients (23.0% of 39 patients undergoing PEA and 8.2% of 110 patients undergoing PTPA) had undergone previous PEA and additional PTPA. RESULTS: In these 9 patients, pulmonary vascular resistance (PVR) was 15.6 (7.8-18.9) wood units at baseline, and significantly improved after PEA [5.6 (3.5-6.5) wood units] (p<0.05). However, PVR gradually deteriorated before PTPA [8.1 (6.1-12.3) wood units] compared to after PEA, suggesting that these 9 patients had residual or recurrent pulmonary hypertension after PEA. PTPA was performed at 4.1 (2.7-7.9) years after PEA. Follow-up catheterization at 1.9 (1.3-3.3) years after PTPA revealed significant improvement of PVR [4.2 (2.8-4.8) wood units] (p<0.05). CONCLUSIONS: A hybrid approach combining PEA and additional PTPA may be reasonable for patients with both proximal and very distal lesions not easily approachable by PEA. PTPA could be a promising alternative therapeutic strategy for residual or recurrent pulmonary hypertension after PEA.

A new era of therapeutic strategies for chronic thromboembolic pulmonary hypertension by two different interventional therapies; pulmonary endarterectomy and percutaneous transluminal pulmonary angioplasty.

BACKGROUND: Pulmonary endarterectomy (PEA) is established for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). Recently, percutaneous transluminal pulmonary angioplasty (PTPA) has been added for peripheral-type CTEPH, whose lesions exist in segmental, subsegmental, and more distal pulmonary arteries. A shift in clinical practice of interventional therapies occurred in 2009 (first mainly PEA, later PTPA). We examined the latest clinical outcomes of patients with CTEPH. METHODS AND RESULTS: This study retrospectively included 136 patients with CTEPH. Twenty-nine were treated only with drug (Drug-group), and the other 107 underwent interventional therapies (Interventions-group) (39 underwent PEA [PEA-group] and 68 underwent PTPA [PTPA-group]). Total 213 PTPA sessions (failures, 0%; mortality rate, 1.47%) was performed in the PTPA-group (complications: reperfusion pulmonary edema, 7.0%; hemosputum or hemoptysis, 5.6%; vessel dissection, 2.3%; wiring perforation, 0.9%). Although baseline hemodynamic parameters were significantly more severe in the Interventions-group, the outcome after the diagnosis was much better in the Interventions-group than in the Drug-group (98% vs. 64% 5-year survival, p<0.0001). Hemodynamic improvement in the PEA-group was a 46% decrease in mean pulmonary arterial pressure (PAP) and a 49% decrease in total pulmonary resistance (TPR) (follow-up period; 74.7 ± 32.3 months), while those in the PTPA-group were a 40% decrease in mean PAP and a 49% decrease in TPR (follow-up period; 17.4 ± 9.3 months). The 2-year survival rate in the Drug-group was 82.0%, and the 2-year survival rate, occurrence of right heart failure, and re-vascularization rate in the PEA-group were 97.4%, 2.6%, and 2.8%, and those in the PTPA-group were 98.5%, 2.9%, and 2.9%, respectively. CONCLUSION: The patients who underwent interventional therapies had better results than those treated only with drugs. The availability of both of these operative and catheter-based interventional therapies leads us to expect the dawn of a new era of therapeutic strategies for CTEPH.

A case report of pulmonary thromboendarterectomy for chronic thromboembolism in a patient with protein C deficiency.

The patient was a 41-year-old female with chronic thromboembolism. She was admitted to an affiliated hospital with exertional dyspnea, leg swelling, and hemoptysis, and she was treated medically with tissue plasminogen activator and warfarin therapy. When transferred to our hospital, she was oxygen-dependent with severe dyspnea. A pulmonary arteriogram showed occlusion and stenosis of the pulmonary arteries. Cardiac catheterization revealed marked pulmonary hypertension. The lung perfusion scintigram showedmultiple defects in the right and left lungs. Preoperative laboratory data showed a markedly decreased protein C antigen level. Magnetic resonance angiography showed that a myoma uteri compressed the pelvic vein and that she had deep vein occlusion of the left leg. After the administration of an epoprostenol infusion and the insertion of an inferior vena cava filter, she underwent an operation. Under deep hypothermia, the bilateral pulmonary artery was opened and an endarterectomy was performed during intermittent circulatory arrest. After surgery, her pulmonary vascular resistance was in the normal range. Her New York Heart Association functional classification changed from class IV to class I. She has been in good condition for 7 years since the surgery.

Histopathological examination by lung biopsy for the evaluation of operability and postoperative prognosis in patients with chronic thromboembolic pulmonary hypertension.

BACKGROUND: To evaluate the prognosis after pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension (CTEPH), a lung biopsy was performed in 34 patients with central CTEPH and in 7 patients with peripheral CTEPH during PTE. METHODS AND RESULTS: Postoperative prognosis was classified from A to E based on the postoperative hemodynamic parameters and clinical condition, and was compared with the index of occlusion (IOCTEPH), which indicates the degree of occlusion in the small pulmonary arteries. Criteria of (A-E) were established only for central CTEPH. Category (A) corresponded to an IOCTEPH from 1.0 to 1.4, (B) from 1.5 to 1.7, (C) from 1.8 to 2.0, and (D) from 2.1 to 2.4. One patient with an index of 3.0 was rated as (E). This patient had collateral vessels around the obstructed small pulmonary arteries and died postoperatively. In all 12 patients who underwent PTE after the criteria were established, postoperative hemodynamic parameters and clinical conditions were consistent with the IOCTEPH. One patient with a high degree of medial atrophy in their small pulmonary arteries died after PTE. CONCLUSIONS: These results indicate that a lung biopsy during PTE is useful for prognostication in patients with CTEPH.

A combination of anatomical and functional evaluations improves the prediction of cardiac event in patients with coronary artery bypass.

OBJECTIVE: To study the usefulness of combined risk stratification of coronary CT angiography (CTA) and myocardial perfusion imaging (MPI) in patients with previous coronary-artery-bypass grafting (CABG). DESIGN: A retrospective, observational, single centre study. SETTING AND PATIENTS: 204 patients (84.3% men, mean age 68.7±7.6) undergoing CTA and MPI. MAIN OUTCOME MEASURES: CTA defined unprotected coronary territories (UCT; 0, 1, 2 or 3) by evaluating the number of significant stenoses which were defined as the left main trunk ≥50% diameter stenosis, other native vessel stenosis ≥70% or graft stenosis ≥70%. Using a cut-off value with receiver-operating characteristics analysis, all patients were divided into four groups: group A (UCT=0, summed stress score (SSS)<4), group B (UCT≥1, SSS<4), group C (UCT=0, SSS≥4) and group D (UCT≥1, SSS≥4). RESULTS: Cardiac events, as a composite end point including cardiac death, non-fatal myocardial infarction, unstable angina requiring revascularisation and heart-failure hospitalisation, were observed in 27 patients for a median follow-up of 27.5 months. The annual event rates were 1.1%, 2%, 5.7% and 12.9% of patients in groups A, B, C and D, respectively (log rank p value <0.0001). Adding UCT or SSS to a model with significant clinical factors including left ventricular ejection fraction, time since CABG and Euro SCORE II improved the prediction of events, while adding UCT and SSS to the model improved it greatly with increasing C-index, net reclassification improvement and integrated discrimination improvement. CONCLUSIONS: The combination of anatomical and functional evaluations non-invasively enhances the predictive accuracy of cardiac events in patients with CABG.

Respiratory and hemodynamic changes in patients with chronic thromboembolic pulmonary hypertension 1 year after pulmonary endarterectomy.

We reviewed the results of thromboembolectomy, which was performed for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH), 1 year after the operation. We obtained hemodynamic and respiratory data of 60 patients from the 112 patients who were operated at our institute. The hemodynamic parameters such as mean pulmonary arterial pressure (PAP), pulmonary vascular resistance (PVR), and cardiac index (CI) were significantly improved after the operation, and this improvement of pulmonary hemodynamics persisted even a year after the operation. A significant improvement in gas exchange was observed immediately after the operation and a further elevation in the partial pressure of oxygen in arterial blood (PaO2) was observed 1 year after the operation. (English Translation of J Jpn Coll Angiol 2012; 52: 53-58).

Arch replacement using antegrade selective cerebral perfusion for shaggy aorta.

BACKGROUND: Embolic stroke during arch replacement is a serious concern in patients with shaggy aorta. OBJECTIVE: To evaluate shaggy aorta in patients who received total aortic arch replacement with antegrade selective cerebral perfusion utilizing axillary perfusion. METHOD: Between January 2005 and December 2010, 63 patients underwent preoperative contrast-enhanced computed tomography scanning of the aorta to evaluate atheromatous plaque. We analyzed operative data to investigate which factors were associated with outcomes and survival. RESULTS: Shaggy aorta was found in 34 (54%) patients. There were 3 (5%) cases in the ascending aorta, 26 (41%) in the aortic arch, and 19 (30%) in the descending aorta. Operative mortality occurred in 1 (2%) patient. Although stroke occurred in 2 (3%) shaggy aorta patients, shaggy aorta was not associated with an increased likelihood of stroke (p = 0.4951). Survival was significantly lower in patients with shaggy descending aorta (p = 0.0411) and in patients >75-years old (p = 0.0200); these traits were identified as independent risk factors for late death (p = 0.0368 and p = 0.0100, respectively). CONCLUSION: We concluded that our perfusion technique protects patients with shaggy aorta against embolism, and that the survival is lower in patients with shaggy descending aorta.

Excellent durability of Starr-Edwards ball valves implanted in the aortic and mitral positions for 27 years: report of a rare surgical case.

A 59-year-old male who had undergone aortic and mitral valve replacement with Starr-Edwards ball valves 27 years ago was admitted to our hospital for hemolytic anemia and heart failure. Echocardiography revealed prosthetic valve failure with a high-pressure gradient and small effective orifice area. The Starr-Edwards ball valves were successfully replaced with bileaflet mechanical valves. The explanted valves revealed no structural abnormalities.

Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension.

BACKGROUND: Chronic thromboembolic pulmonary hypertension leads to pulmonary hypertension and right-sided heart failure. The purpose of this study was to investigate the efficacy of percutaneous transluminal pulmonary angioplasty (PTPA) for the treatment of chronic thromboembolic pulmonary hypertension. METHODS AND RESULTS: Twenty-nine patients with chronic thromboembolic pulmonary hypertension underwent PTPA. One patient had a wiring perforation as a complication of PTPA and died 2 days after the procedure. In the remaining 28 patients, PTPA did not produce immediate hemodynamic improvement at the time of the procedure. However, after follow-up (6.0 ± 6.9 months), New York Heart Association functional classifications and levels of plasma B-type natriuretic peptide significantly improved (both P<0.01). Hemodynamic parameters also significantly improved (mean pulmonary arterial pressure, 45.3 ± 9.8 versus 31.8 ± 10.0 mm Hg; cardiac output, 3.6 ± 1.2 versus 4.6 ± 1.7 L/min, baseline versus follow-up, respectively; both P<0.01). Twenty-seven of 51 procedures in total (53%), and 19 of 28 first procedures (68%), had reperfusion pulmonary edema as the chief complication. Patients with severe clinical signs and/or severe hemodynamics at baseline had a high risk of reperfusion pulmonary edema. CONCLUSIONS: PTPA improved subjective symptoms and objective variables, including pulmonary hemodynamics. PTPA may be a promising therapeutic strategy for the treatment of chronic thromboembolic pulmonary hypertension. CLINICAL TRIAL REGISTRATION: URL: http://www.umin.ac.jp. Unique identifier: UMIN000001572.

Non-invasive evaluation of internal thoracic artery anastomosed to the left anterior descending artery with 320-detector row computed tomography and adenosine thallium-201 myocardial perfusion scintigraphy.

PURPOSE: We evaluated the relationship between internal thoracic artery (ITA) stenosis anastomosed to the left anterior descending artery (LAD) and the degree of LAD stenosis using 320-detector row computed tomography (320-ADCT) and adenosine thallium-201 myocardial perfusion scintigraphy (Tl-201-MPS). METHODS: We included 101 patients who underwent coronary artery bypass grafting (CABG) using ITA grafts; 320-ADCT and adenosine Tl-201-MPS were performed 2-3 months after CABG. Clinical parameters, degree of LAD stenosis, and regional myocardial ischemia of the LAD territory were compared between patients without ITA stenosis (Group A) and with ITA stenosis (Group B). RESULTS: Thirty patients (30%) had ≤75% LAD stenosis, and 9 patients (30%) showed significant ITA stenosis. Regional ischemia was noted in 23 patients (23%). There were no differences in clinical parameters between the 2 groups. Twenty-two patients (24%) in Group A and 8 patients (89%) in Group B had ≤75% LAD stenosis (P <0.002). No Group B patients had regional myocardial ischemia of the LAD territory. CONCLUSION: We concluded that ≤75% LAD stenosis significantly influences ITA stenosis, without associated regional myocardial ischemia of the LAD territory. Non-invasive 320-ADCT and adenosine Tl-201-MPS for ITA evaluation may be useful for long-term follow-up of patients after CABG.

The importance of preoperative magnetic resonance imaging in valve surgery for active infective endocarditis.

PURPOSE: Valve surgery for active infective endocarditis (IE) can cause fatal brain hemorrhage. Our current study aimed to evaluate the incidence of septic cerebral lesions in active IE patients by performing preoperative magnetic resonance imaging (MRI) including T(2)*- weighted sequences and magnetic resonance angiography (MRA) before urgent valve surgery, and to investigate whether such preoperative evaluation affects postoperative outcomes. METHODS: Eighteen patients were referred to our department for native valve IE during 2006-2010. Urgent surgery was indicated in cases of hemodynamic failure resulting from valve destruction, refractory sepsis, and mobile vegetations measuring >10 mm. For these patients, we performed preoperative MRI and MRA. RESULTS: Males comprised 67% of the subjects, with average age 53 ± 15 years. No clinical evidence of acute stroke was noted. Of the 18 patients, urgent surgery was indicated in 15; of these, 10 (67%) showed a brain lesion related to IE: 6 patients had acute or subacute brain infarctions, 2 patients had brain infarction with brain abscess, and 2 patients had hemorrhagic brain infarction and so did not undergo urgent surgery. Thus, 13 patients underwent urgent valve surgery. Among the 5 patients who did not undergo urgent surgery, 4 patients later underwent valve surgery for healed IE. No hospital deaths or neurological complications occurred. CONCLUSION: MRI of patients with active IE revealed a high incidence of cerebral lesions caused by IE. The use of MRI to detect septic embolism and intracerebral hemorrhage may provide important information for better surgical outcomes.

Pseudoaneurysm of ascending aorta 16 years after coronary artery bypass grafting.

Pseudoaneurysm of the ascending aorta after cardiac surgery is a rare but life threatening complication, which can result in rupture. Pseudoaneurysms are usually related to the aortic cannulation, the proximal site of graft anastomosis, or the suture line of aortotomy, and often occur after mediastinal infection. We report a case of pseudoaneurysm of the ascending aorta associated with aortic cannulation and the proximal anastomosis of a saphenous vein graft without an obvious history of mediastinal infection.