Chronic thromboembolic pulmonary hypertension.

Pulmonary arterial hypertension is a severe disease that has been ignored for a long time. However, over the past 20 yrs chest physicians, cardiologists and thoracic surgeons have shown increasing interest in this disease because of the development of new therapies, that have improved both the outcome and quality of life of patients, including pulmonary transplantation and prostacyclin therapy. Chronic thromboembolic pulmonary arterial hypertension (CTEPH) can be cured surgically through a complex surgical procedure: the pulmonary thromboendarterectomy. Pulmonary thromboendarterectomy is performed under hypothermia and total circulatory arrest. Due to clinically evident acute-pulmonary embolism episodes being absent in > 50% of patients, the diagnosis of CTEPH can be difficult. Lung scintiscan showing segmental mismatched perfusion defects is the best diagnostic tool to detect CTEPH. Pulmonary angiography confirms the diagnosis and determines the feasibility of endarterectomy according to the location of the disease, proximal versus distal. The technique of angiography must be perfect with the whole arterial tree captured on the same picture for each lung. The lesions must start at the level of the pulmonary artery trunk, or at the level of the lobar arteries, in order to find a plan for the endarterectomy. When the haemodynamic gravity corresponds to the degree of obliteration, pulmonary thromboendarterectomy can be performed with minimal perioperative mortality, providing definitive, excellent functional results in almost all cases.     

肺动脉血栓内膜剥脱术治疗慢性栓塞性肺动脉高压效果分析

目的分析慢性栓塞性肺动脉高压(CTEPH)患者应用肺动脉血栓内膜剥脱术(PTE)治疗的围手术期及中、晚期效果。方法选择CTEPH患者81例,按临床病理分型分为中央型组60例和外周型组21例,在深低温停循环下行PTE,随访5年,观察生存率。回顾性按年龄将患者分为老年组(≥60岁,19例)和非老年组(<60岁,62例),比较2组早期及晚期生存率。结果中央型组围手术期死亡1例。外周型组患者体外循环时间、主动脉阻断时间、深低温停循环时间均显著长于中央型组;围手术期肺动脉高压危象发生率显著高于中央型组,肺再灌注损伤发生率显著低于中央型组;肺动脉收缩压、肺循环阻力显著高于中央型组(P<0.05,P<0.01)。随访期间1例死亡,5年生存率为98.77%。老年组与非老年组术后早期及晚期生存率差异无统计学意义(P>0.05)。结论 PTE治疗CTEPH患者有较好的围手术期及中、晚期生存率,老年患者术后早期及晚期生存率与非老年患者无显著差异。术前准确评估,可提高PTE早期及中、晚期生存率。     

Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension

OBJECTIVES: The continuous IV administration of prostacyclin improves pulmonary hemodynamics and prognosis in patients with primary pulmonary hypertension. We investigated whether the administration of prostacyclin therapy to patients before they undergo pulmonary thromboendarterectomy ameliorates pulmonary hypertension in patients with the most severe form of chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: Of the 33 patients with CTEPH who were candidates for pulmonary thromboendarterectomy, 12 patients with severe pulmonary hypertension (pulmonary vascular resistance, > 1,200 dyne. s. cm(-5)) received IV prostacyclin prior to undergoing pulmonary thromboendarterectomy. Right heart catheterization and plasma brain natriuretic peptide (BNP) measurements were repeated at baseline, immediately before surgery, and 1 month after surgery. RESULTS: During a mean (+/- SEM) follow-up period of 46 +/- 12 days, the IV administration of prostacyclin resulted in a 28% decrease in pulmonary vascular resistance (1,510 +/- 53 to 1,088 +/- 58 dyne. s. cm(-5); p < 0.001) before surgery. Prostacyclin therapy markedly decreased plasma BNP level (547 +/- 112 to 188 +/- 30 pg/mL; p < 0.01), suggesting improvement in right heart failure. Pulmonary thromboendarterectomy caused a further reduction of pulmonary vascular resistance (302 +/- 47 dyne. s. cm(-5)) and plasma BNP levels (60 +/- 11 pg/mL) compared to each preoperative value (p < 0.05). Operative mortality rates were relatively low (8.3%) in patients with the most severe form of CTEPH. CONCLUSION: The IV administration of prostacyclin caused beneficial hemodynamic effects in patients with severe CTEPH and may serve as pretreatment for patients undergoing pulmonary thromboendarterectomy.     

Chronic Thromboembolic Pulmonary Hypertension: Evolving Therapeutic Approaches for Operable and Inoperable Disease

Chronic thromboembolic pulmonary hypertension (CTEPH), a rare consequence of an acute pulmonary embolism, is a disease that is underdiagnosed, and surgical pulmonary thromboendarterectomy (PTE) remains the preferred therapy. However, determination of operability is multifactorial and can be challenging. There is growing excitement for the percutaneous treatment of inoperable CTEPH with data from multiple centers around the world showing the clinical feasibility of balloon pulmonary angioplasty. Riociguat remains the only approved medical therapy for CTEPH patients deemed inoperable or with persistent pulmonary hypertension after PTE. We recommend that expert multidisciplinary CTEPH teams be developed at individual institutions. Additionally, optimal and standardized techniques for balloon pulmonary angioplasty need to be developed along with dedicated interventional equipment and appropriate training standards. In the meantime, the percutaneous revascularization option is appropriate for patients deemed inoperable in combination with targeted medical therapy, or those who have failed to benefit from surgery.     

Cytokine response to pulmonary thromboendarterectomy

BACKGROUND: Pulmonary thromboendarterectomy (PTE) is an effective but challenging treatment for chronic thromboembolic pulmonary hypertension (CTEPH). PTE is associated with marked hemodynamic instability in the perioperative course, suggesting the involvement of circulating mediators. The aim of this study was to characterize the expression of proinflammatory and anti-inflammatory cytokines in patients undergoing PTE. METHODS: Fourteen patients with CTEPH (mean [+/- SD] pulmonary vascular resistance, 1,056 +/- 399 dyne.s.cm(-5)) underwent PTE using cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). Peripheral arterial blood samples were drawn prior to patients undergoing sternotomy, during CPB, before and after DHCA, and 0, 8, 16, 24, and 48 h after surgery. An enzyme-linked-immunosorbent assay was used to analyze the plasma levels of tumor necrosis factor (TNF)-alpha, interleukin (IL)-6, and IL-10. Seven patients undergoing aortic arch replacement (ARCH) in DHCA served as a control group. RESULTS: Prior to and during PTE, the CTEPH patients exhibited elevated TNF-alpha levels, which decreased within the first 24 postoperative hours (p = 0.02). There was no TNF-alpha release among patients in the ARCH group. IL-6 levels were similar in both groups throughout the perioperative course. A profound anti-inflammatory response was observed in the PTE group, which was reflected by elevated IL-10 levels prior to surgery and a marked peak level immediately after surgery. A positive correlation was found between maximum vasopressor support and peak levels of IL-6 (r = 0.82) in the PTE patients. CONCLUSION: Heart failure due to CTEPH appears to generate a pronounced inflammatory response with the release of proinflammatory and anti-inflammatory cytokines. PTE results in the rapid normalization of preoperatively elevated TNF-alpha levels. IL-6-mediated systemic inflammatory cascades may be involved in the regulation of peripheral vascular tone after PTE.     

Dilatation of bronchial arteries correlates with extent of central disease in patients with chronic thromboembolic pulmonary hypertension.

BACKGROUND: Dilatation of the bronchial arteries is a well-recognized feature in patients with chronic thromboembolic pulmonary hypertension (CTEPH). The purpose of the current study was to use computed tomography (CT) to assess the relationship between dilated bronchial arteries and the extent of thrombi, and to evaluate the predictive value of the former for surgical outcome. METHODS AND RESULTS: Fifty-nine patients with CTEPH and 16 with pulmonary arterial hypertension (PAH) were retrospectively evaluated. The total cross-sectional area of bronchial arteries was measured by CT and its relationship with the central extent of thrombi or surgical outcome was assessed. The total area of the bronchial arteries in CTEPH patients was significantly larger than that in PAH patients (median [range], 6.9 [1.7-29.5] mm(2) vs 3.2 [0.8-9.4] mm(2)), with the total area of bronchial arteries correlating with the central extent of thrombi. In patients who had undergone pulmonary thromboendarterectomy (PTE) (n=22), the change in PaO(2) after surgery had a tendency to correlate with the total area of the bronchial arteries. CONCLUSION: The total cross-sectional area of the bronchial arteries correlated with the extent of central disease in patients with CTEPH, and it might predict gas exchange improvement after PTE.     

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??Abstract??The pulmonary thromboembolism is divided into acute pulmonary embolism and chronic thromboembolic pulmonary hypertension (CTEPH).Antithrombotic treatment of acute pulmonary embolism is divided into the initial treatment and long-term treatment.Initial treatment includes parenteral anticoagulation??systemic thrombolysis??catheter thrombectomy??surgical thrombectomy and vena cava filter implantation.Long-term treatment needs comprehensive consideration in patients with predisposing factors for VTE and bleeding risk factors.It is divided into (1)treatment of a shorter period??(2)treatment with anticoagulation for 3 months??(3)treatment of a longer time-limited period (eg??6 or 12 months)??(4)extended therapy.In selected patients with CTEPH??such as those with central disease under the care of an experienced thromboendarterectomy team??we suggest pulmonary thromboendarterectomy (PEA).In those patients not suitable for the PEA surgery??it is recommended that the use of the pulmonary targeting antihypertensive drugs and long-term anticoagulation.     

Implementation of a new programme for the surgical treatment of CTEPH in the Czech Republic--Pulmonary endarterectomy

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) in indicated cases can be successfully cured by endartectomy of pulmonary arteries (PEA). Symptomatic nontreated CTEPH has a very poor prognosis; the five-year survival rate in patients with a medium pressure of over 50 mmHg in the main pulmonary artery is as low as 10 %. This kind of operation was previously not available in the Czech Republic. In 2004, a PEA programme was launched at the Cardiocentre of the General Teaching Hospital in Prague in co-operation with the institution of a well known specialist in this field (Prof. Mayer, Johannes Gutenberg University in Mainz, Germany). PATIENTS: Between September 2004 and January 2006, 21 patients (14 males and 7 females; average age 48 years) with CTEPH were operated on, after a complex investigation. The mean pressure in the main pulmonary artery in these patients was 54.8 mmHg; 7 patients suffered from coagulopathy. METHOD: The new surgical technique, modifications of which are used at most facilities, was developed by Jamieson and Daily at the University of California in San Diego: an arrest of circulation in deep hypothermia to protect the brain is vital for the visualisation of distal branches of the pulmonary artery. RESULTS: 21 patients were operated on with a mortality of 4.76 % (1 patient died). Other surgeries performed were suture of a defect of the atrial septum (three times), aortocoronary bypass (three times), and cryoablation of the right atrium for flutter (once). The average circulatory arrest time was 42 minutes, the average total pumping time was 331 minutes, and the average total duration of an operation was 450 minutes; the average duration of mechanical ventilation was 58 hours. Within one month there was a considerable improvement or normalisation of haemodynamic parameters and an increase in the average walking distance on the six-minute walking test by 132 metres. CONCLUSIONS: PEA is a curative method for patients with CTEPH with a surgically accessible obstruction of the pulmonary artery. Centralisation of the care of these patients is a rational necessity, as this enables the centre to gain a maximum of experience with this complicated diagnosis and treatment. Multidisciplinary co-operation is a sine qua non for success in these programmes.     

The influence of fractional pulse pressure on the outcome of pulmonary thromboendarterectomy.

Although pulmonary thromboendarterectomy is an effective modality for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH), the mortality in patients with severe haemodynamic disease is still high. Recently it was reported that fractional pulse pressure (pulmonary arterial pulse pressure/mean pulmonary arterial pressure) was higher in CTEPH than in primary pulmonary hypertension (PPH). It was hypothesized that fractional pulse pressure might be low in CTEPH with inaccessible distal thrombi and/or secondary pulmonary hypertensive change, resulting to the high operative mortality. To determine the influence of fractional pulse pressure to the outcome of surgery, 32 patients with CTEPH who had thromboendarterectomy between 1985 and 1998 were studied. Pulmonary haemodynamics and fractional pulse pressure were compared between survivors (n=26) and nonsurvivors (n=6) postoperatively. Those parameters in PPH (n=18) and large vessel pulmonary arteritis (n=6) were also analysed. Fractional pulse pressure in CTEPH (1.23+/-0.21) was significantly higher than in PPH (0.93+/-0.22; p=0.0017) and lower than in pulmonary arteritis (1.69+/-0.32; p=0.03). Fractional pulse pressure in survivors (1.26+/-0.21) was significantly higher than in nonsurvivors (1.06+/-0.16; p=0.03). Fractional pulse pressure is a significant predictor for mortality in patients with high pulmonary vascular resistance >1100 dynes.sec.cm(-5). To conclude fractional pulse pressure in addition to pulmonary vascular resistance might be useful in predicting for the outcome of surgery, especially in patients with severe haemodynamic impairment.     

Increase in thrombomodulin concentrations after pulmonary thromboendarterectomy in chronic thromboembolic pulmonary hypertension

STUDY OBJECTIVES: The objectives of the study were as follows: (1) to identify differences in endothelial dysfunction and altered hemostasis in patients with chronic thromboembolic pulmonary hypertension (CTEPH) compared with patients with acute pulmonary thromboembolism (APTE) uncomplicated by pulmonary arterial hypertension, by measuring the concentrations of thrombomodulin (TM), a receptor for thrombin and a major anticoagulant proteoglycan on the endothelial membrane, and other plasma factors of coagulation and fibrinolysis; and (2) to examine the effects of thromboendarterectomy on TM levels as a parameter of endothelial cell injury leading to abnormal hemostasis as well as to examine the clinical significance of TM as a marker of endothelial injury. DESIGN: Prospective comparison of concentrations of TM and other plasma parameters among patients with CTEPH or APTE and control subjects. PARTICIPANTS: We studied 22 healthy subjects (ie, control subjects), 22 patients who had been clinically stabilized after APTE, and 44 patients with CTEPH. In 21 of the patients with CTEPH, measurements were repeated after they had undergone pulmonary thromboendarterectomy. MEASUREMENTS AND RESULTS: Plasma concentrations of soluble TM in patients with CTEPH were measured and compared with those in patients with APTE. The mean (+/- SD) TM concentration in the CTEPH group (2.5 +/- 0.7 ng/mL) was significantly lower than that in the control group (4.0 +/- 0.6 ng/mL; p < 0.05). In contrast, the mean plasma TM concentration in the APTE group (4.6 +/- 1.9 ng/mL) was similar to that in the control group. After patients underwent pulmonary thromboendarterectomy, the mean TM concentration increased from 2.0 +/- 0.4 to 2.9 +/- 0.7 ng/mL (p < 0.05). In the CTEPH group, the plasma TM concentration was negatively correlated with mean pulmonary arterial pressure and total pulmonary resistance (p < 0.05). CONCLUSIONS: A decreased plasma TM concentration may reflect pulmonary vascular endothelial dysfunction leading to altered anticoagulant and fibrinolytic function in CTEPH, which rarely develops after APTE. Plasma TM measurements may be useful in distinguishing CTEPH with severe pulmonary hypertension from recurrent APTE.     

Thoracic research scholarship 1988: pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension at the University of California, San Diego

At the University of California, San Diego pulmonary thromboendarterectomy (PTE) has emerged as an effective measure in the treatment of chronic thromboembolic pulmonary hypertension. Unresolved emboli become organized by incorporation into the vascular wall and may form strictures, webs, bands and/or membranous occlusions and cause pulmonary hypertension refractory to medical treatment. When pulmonary vascular resistance exceeds 300 dyn.sec.cm-5 and the vascular wall changes are located to begin at or proximal to the lobar artery level, surgery is indicated. The operation is performed using cardiopulmonary bypass, deep hypothermia and periods of circulatory arrest. The dissection of each segmental artery is carried out in the media layer from separate incisions in the right and left pulmonary artery at the level of the pericardial flexion. Pulmonary reperfusion edema may complicates the postoperative course, and pulmonary hemorrhage, respiratory insufficiency necessitating prolonged ventilatory support and secondary multi organ failure are main causes of hospital mortality. Between October 1984 and September 1988 103 patients with a mean age of 50 +/- 16 years underwent PTE. Consequently, pulmonary vascular resistance could be reduced from 788 +/- 370 to 299 +/- 150 dyn.sec.cm-5 and cardiac index increased from 2.0 +/- 0.6 to 3.2 +/- 0.8 l/min-m2. Hospital mortality was 11.7% (12/103 patients). Thus, pulmonary thromboendarterectomy effectively reduces pulmonary hypertension at an acceptable low risk. The results indicate that patients should be diagnose and referred for surgery as early as possible.     

Survival and quality of life for patients with peripheral type chronic thromboembolic pulmonary hypertension.

BACKGROUND: The validity of pulmonary thromboendarterectomy for treatment of relatively peripheral type of chronic thromboembolic pulmonary hypertension (CTEPH) remains uncertain. The survival and quality of life (QOL) of patients with relatively peripheral type of CTEPH was investigated at follow up. METHODS AND RESULTS: Between April 1999 and March 2006, 83 consecutive patients with CTEPH were evaluated for surgical indication and underwent computed tomography angiography. The extent of central disease was scored (ie, CD score), and a CD score of 相似文献   

An Update on the Management of Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening form of pulmonary artery hypertension that is defined as a mean arterial pulmonary pressure greater than 25 mm Hg that persists for more than 6 months following anticoagulation therapy in the setting of pulmonary emboli. CTEPH is categorized by the World Health Organization as group IV pulmonary hypertension and is thought to be due to unresolved thromboemboli in the pulmonary artery circulation. Among the 5 classes of pulmonary hypertension, CTEPH is unique in that it is potentially curable with the use of pulmonary thromboendarterectomy surgery. Despite an increasing array of medical and surgical treatment options for patients with CTEPH over the past 2 decades, patients commonly present with advanced disease and carry a poor prognosis, thus, the need for early diagnosis and appropriate referral to an expert center. This review article first highlights the epidemiology, pathophysiology, and clinical presentation of CTEPH. The article then provides diagnostic and therapeutic algorithms for the management of the patient with suspected CTEPH.     

Chronic Thromboembolic Pulmonary Hypertension: A Comprehensive Review and Multidisciplinary Approach to Surgical Treatment

Chronic thromboembolic pulmonary hypertension (CTEPH) is an underdiagnosed and undertreated sequelae of acute pulmonary embolism. In this comprehensive review, we provide an introductory overview of CTEPH, highlight recent advances in its diagnostic imaging, and describe the surgical technique for pulmonary thromboendarterectomy (PTE), the only established curative treatment for CTEPH. We also discuss the emerging role of balloon pulmonary angioplasty, both independently and combined with PTE, for patients with inoperable, residual, or refractory pulmonary hypertension post PTE. Finally, we stress the importance of a specialized multidisciplinary team approach to CTEPH patient care and share our approach to optimizing care for these patients.     

Continuous intravenous epoprostenol for chronic thromboembolic pulmonary hypertension.

Pathophysiological findings in chronic thromboembolic pulmonary hypertension (CTEPH) have suggested that a secondary small vessel arteriopathy may contribute to the haemodynamic impairment observed in these patients. It was hypothesised that this element of the elevated vascular resistance may be responsive to continuous intravenous epoprostenol therapy. Retrospectively, the clinical and haemodynamic responses to continuous intravenous epoprostenol were evaluated in nine CTEPH patients who subsequently underwent pulmonary thromboendarterectomy (PTE). Cardiopulmonary haemodynamics were determined prior to the initiation of epoprostenol, while on epoprostenol, prior to PTE, and after PTE. Six patients, treated for 2-26 months prior to PTE, experienced either clinical stability or improvement that was associated with a mean reduction in pulmonary vascular resistance (PVR) of 28% (median 33%, range 0-46%). Three patients, treated for 3-9 months, experienced clinical deterioration during epoprostenol administration, with a significant increase in PVR in two patients. Subsequent PTE resulted in a highly significant improvement of cardiac index, mean pulmonary artery pressure and total pulmonary resistance. To conclude, selected patients with chronic thromboembolic pulmonary hypertension may benefit clinically and haemodynamically from continuous intravenous epoprostenol treatment prior to pulmonary thromboendarterectomy. Factors predictive of a beneficial response, and whether this intervention influences either morbidity or mortality associated with pulmonary thromboendarterectomy, remain to be established.     

From acute pulmonary embolism to chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH), a disease associated with considerable morbidity and mortality, is the consequence of unresolved thromboembolic occlusion in pulmonary vasculature. CTEPH was considered a rare disease occurring in 0.1-0.5% of patients with pulmonary emboli who survive. Recently, a much higher incidence was reported and some risk factors such as a previous pulmonary embolism (PE), an idiopathic form of PE and the severity of perfusion defect at the time of diagnosis have been identified. Exertional dyspnea is the main symptom at the beginning of the disease while later on patients may suffer from syncope related to low cardiac output or hemoptysis as a consequence of high pulmonary artery pressure. In suspected patients, a confirmation of pulmonary arterial hypertension should be ascertained at transthoracic echocardiography. Then the obstructive nature of the disease may be revealed by ventilation-perfusion lung scan but is better described at pulmonary angiography. Computed tomography scan may be useful to rule out confounding disorders. To prevent recurrences, long-term oral anticoagulants to maintain an INR between 2.5 and 3.5 (target 3.0) are indicated. Treatment of severe CTEPH is essentially surgical (thromboendarterectomy). This procedure may be difficult when distal branches of pulmonary vascular tree are involved. In selected cases, alternative therapies may be the arterial pulmonary vessel angioplasty and lung transplantation.     

Chronic thromboembolic pulmonary hypertension: A review of current practice

Pulmonary artery hypertension is a complex and multi-faceted disease process with numerous etiologies. Chronic thromboembolic pulmonary hypertension (CTEPH) is an underdiagnosed and highly treatable form of pulmonary hypertension. In this disease, certain patients with a history of pulmonary thromboembolic disease go on to develop elevated pulmonary artery pressures, shortness of breath, and progressive right heart failure. This article will review the epidemiology, pathophysiology, diagnosis and treatment of CTEPH with a review of pulmonary thromboendarterectomy surgery.     

Chronic thromboembolic pulmonary arterial hypertension: correlation of postoperative results of thromboendarterectomy with preoperative helical contrast-enhanced computed tomography

INTRODUCTION: Pulmonary thromboendarterectomy is the treatment of choice for patients with chronic thromboembolic pulmonary arterial hypertension (CTEPH). Some patients do poorly after this procedure and may be better candidates for heart-lung transplant. The purpose of this study was to correlate preoperative findings on helical contrast-enhanced computed tomography (CT) with surgical outcome. METHODS: Thirty-seven patients (mean age 52.9, range 22-71) who underwent pulmonary thromboendarterectomy and had preoperative helical contrast-enhanced CT followed by High Resolution CT (HRCT) scans were included in the study. The CTs were evaluated for the presence of central and segmental disease and for the presence of mosaic perfusion pattern. RESULTS: The presence of central disease, as well as the presence of segmental disease, correlated negatively with the postoperative mean pulmonary arterial pressure [r(c) = -0.401, P = 0.015, r(s) = -0.38, P = 0.024)] and the pulmonary vascular resistance [(r(c) = -0.37, P = 0.027, r(s) = -0.39, P = 0.019]. No correlation was found between the clinical variables and the presence of mosaic perfusion pattern. CONCLUSION: Patients with CTEPH and evidence of chronic PE in the central or segmental pulmonary arteries have a better clinical outcome after pulmonary thromboendarterectomy than patients without these findings. The presence of mosaic perfusion pattern is not helpful in predicting postoperative outcome.     

Results of one-year anticoagulation in patients with newly detected chronic thromboembolic pulmonary hypertension not treated with pulmonary endarterectomy

INTRODUCTION: Currently pulmonary endarterectomy is considered the method of choice in patients with chronic thromboembolic pulmonary hypertension (CTEPH). It is not known if this option should be recommended in all suitable patients as it is highly variable with respect to prognosis. There is also doubt about selection of adequate time to refer patients with CTEPH for surgery. AIM: To establish whether some patients with CTEPH may clinically benefit from isolated anticoagulation with drugs and if the use of anticoagulation may have any impact on the time of patient referral for pulmonary endarterectomy. METHODS: The prospective analysis involved 29 patients (9 male, 20 female) aged 37 to 82 years, with pulmonary arterial systolic pressure ranging from 39 to 133 mmHg and newly diagnosed CTEPH who had not been treated with pulmonary endarterectomy and were not receiving anticoagulation. Survival, functional status according to NYHA classification, duration of thromboembolism, exercise tolerance and echocardiographic parameters of right ventricular overload before and at one year after initiation of therapy with anticoagulants were evaluated. RESULTS: During follow-up, 3 patients with PASP ranging from 120 to 133 mmHg died. In 26 patients with PASP 39-115 mmHg, who survived, improvement in echocardiographic parameters of right ventricular overload, better exercise tolerance as well as functional status according to NYHA classification was observed. In 12 survivors, pulmonary pressure returned to normal. CONCLUSIONS: The results of this study suggest that favourable effects of isolated anticoagulation are likely in patients with newly detected CTEPH, mild and moderate baseline pulmonary hypertension and acceptable exercise tolerance. They also indicate the necessity of anticoagulation in these patients prior to possible referral for pulmonary endarterectomy.     

Necrotizing enterocolitis in pulmonary atresia with intact ventricular septum

Necrotizing enterocolitis (NEC) occurred in 2 neonates with pulmonary atresia and intact ventricular septum. Both infants underwent open heart surgery for the insertion of a right ventricular outflow tract patch under profound hypothermia, surface cooling and a fibrillating heart, without circulatory arrest. In both patients the development of NEC preceded both the cardiac catheterization study and open heart surgery. Urgent repair of the cardiac lesion was undertaken in an attempt at improving the critical bowel wall ischemia. Postoperatively, exacerbation of the NEC reached an advanced stage rapidly leading to the death of the patients. Recommendations regarding the management of future cases exhibiting this potentially lethal combination of disease are presented.