291 * LONG-TERM FOLLOW-UP ASSESSMENT OF SURGICAL TREATMENT OF CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION

The aim – to present to the medical community the functioning algorithm of the heart team concept in the diagnosis and surgical treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in Ukraine. The concept of the CTEPH team implies a multi-disciplinary approach to the diagnosis and, consequently, surgical treatment of CTEPH with the participation of a radiologist, a pulmonologist, a cardiologist, a hematologist, a neurologist, and a cardiac surgeon. Considering the low incidence of the disease, the need for high-tech diagnostic tools, including CT angiography and angiopulmonography, as well as challenges of evaluating the operability of patients and the technical complexity of operations, patients with CTEPH are get together in the so-called reference centers. Within the framework of the CTEPH team concept, specific protocols and «road maps» have been developed for both diagnosis and treatment of the disease. In order to eliminate subjectivity in determining the operability of a patient with CTEPH, a special algorithm has been developed, which is applied to a clinical case for illustration. Monitoring patients with pulmonary embolism who are qualifed for a newly coined definition – postembolic pulmonary syndrome, has become a new task of the reference centers. The new syndrome is suggested to include CTEPH, as well as a similar, but not identical pathological condition, named chronic thromboembolic lung disease. The treatment of choice for CTEPH is pulmonary thrombendarterectomy. Mandatory elements of the surgical protocol include the creation of a «dry» operating field by means of a temporary circulatory arrest under deep hypothermia of 18 °C. Operations for distal lesions of the pulmonary arteries have become a recent achievement. The success of the operation depends on the anatomical type of the lesions, the degree of distal arteriopathy, the extent of the intervention, and the comorbid factors. Immediate and long-term results of the operation are evaluated as good and very good. The long-term survival of patients after surgery is significantly higher than of those who were administered medical treatment.Conclusions. Patients with suspected CTEPH should be referred to a reference expert center for diagnosis verification and operability determining. The standard diagnosis of CTEPH is angiopulmonography with simultaneous measurement of pressure in the right heart; the standard treatment is pulmonary thrombendarterectomy.

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Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report

Monaldi Archives for Chest Disease ◽

10.4081/monaldi.2017.880 ◽

2017 ◽

Vol 87 (3) ◽

Author(s):

Paolo Ruggeri ◽

Federica Lo Bello ◽

Francesco Nucera ◽

Michele Gaeta ◽

Francesco Monaco ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Nephrotic Syndrome ◽

Main Pulmonary Artery ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Exercise Intolerance ◽

Artery Thrombosis ◽

Stable Conditions ◽

Thromboembolic Pulmonary Hypertension

We present here the case of a 30-year-old man with a long term history of nephrotic syndrome (NS) who developed an episode of acute left main pulmonary artery thrombosis complicated by a lung abscess. During the hospital admission was also identified a concomitant hyperhomocysteinemia. After an atypical resection of the left upper pulmonary lobe and the starting of long term anticoagulation the patient was discharged but did not attend the planned follow up visits until one year later when he was seen again for severe dyspnea and exercise intolerance. At this time chronic thromboembolic pulmonary hypertension (CTEPH) was diagnosed by lung perfusion scintigraphy and right heart catheterization. He initially refused the surgical treatment but, after six months, for the presence of worsening dyspnea was referred for bilateral pulmonary endarterectomy followed by a cardio-thoracic rehabilitation program. After a follow-up of seven years the patient is alive and in stable conditions. NS and hyperhomocysteinemia are both known risk factors for pulmonary embolism (PE), but their association with CTEPH is extremely rare. We discuss here the possible mechanisms linking these conditions. CTEPH must be suspected in any patient with NS, with or without hyperhomocysteinemia, and unexplained dyspnea.

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Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension

Asian Cardiovascular and Thoracic Annals ◽

10.1177/021849239700500108 ◽

1997 ◽

Vol 5 (1) ◽

pp. 35-38

Author(s):

Gao Chang Qing ◽

Rodolfo Barragan Garcia

Keyword(s):

Pulmonary Hypertension ◽

Median Sternotomy ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Pulmonary Thromboendarterectomy ◽

Pulmonary Hemodynamic ◽

Major Vessel ◽

Thromboembolic Pulmonary Hypertension ◽

Hemodynamic Improvement

We describe our experience of treating chronic major vessel thromboembolic pulmonary hypertension with pulmonary thromboendarterectomy. Thromboendarterectomy was performed in 15 patients through a median sternotomy incision using extracorporeal circulation and deep hypothermia. Postoperatively mean pulmonary artery pressure, mean pulmonary vascular resistance, and cardiac index were significantly improved (p < 0.001). Four patients died during surgery, mainly from perfusion edema (operative mortality 27%). The 11 surviving patients received long-term anticoagulant therapy and no incidence of thromboembolism has occurred during the follow-up period. All have shown sustained pulmonary hemodynamic improvement; however, there were 2 late deaths from cardiac failure. We conclude that thromboendarterectomy is an effective treatment for chronic major vessel thromboembolic pulmonary hypertension.

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