CHAPTER X - SECONDARY PULMONARY HYPERTENSION CHRONIC PULMONARY THROMBOEMBOLISM (HTP). PULMONARY THROMBENDARTERECTOMY

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1 CHAPTER X - SECONDARY PULMONARY HYPERTENSION CHRONIC PULMONARY THROMBOEMBOLISM (HTP). PULMONARY THROMBENDARTERECTOMY Walter KLEPETKO, PhD, VIENNA - AUSTRIA Marian GASPAR, PhD, TIMISOARA Definition. Incidence. Thromboembolic chronic hypertension is the result of intraluminal thrombosis, fibrosis which produces stenosis and / or blockage of pulmonary artery branches, with increasing pulmonary artery pressure above 50 mmhg, pressure in ventricle, right atrium and right heart failure setting. Pulmonary thrombendarterectomy is the surgical technique of extracting old thrombotic material from pulmonary artery system. It is the only efficient method of treatment in secondary thromboembolic pulmonary hypertension. The cause of secondary pulmonary hypertension may be repeated pulmonary embolism in areas of venous peripheral thrombosis. In other cases, there is no obvious cause. Acute pulmonary embolism is one of the most common cardiovascular diseases. The real impact of this disease is underestimated because of uncertain diagnosis. Death by pulmonary embolism ranks third after heart disease and cancer. Most sources of embolism are deep peripheral venous thrombosis, but 75% may be asymptomatic. Incidence of pulmonary hypertension caused by repeated pulmonary embolism is even more unclear than the acute pulmonary embolism. It is estimated that patients in the U.S. survive acute pulmonary embolism and of them progress to thromboembolic chronic pulmonary hypertension every year. Real incidence of both acute and chronic form is much underrated. Pathology and pathogenicity. In many cases acute episode of deep vein thrombosis as a source of pulmonary embolism can not be identified from the patient history. The embolic material that reaches the pulmonary artery and its branches, depending on quantity, size, consistency, undergoes certain changes that can lead to stenosis, reducing the lumen in different segments or occlusion of branches. These changes occur gradually until they become severe to be symptomatic and the patient to be diagnosed Clinical symptoms and signs. Thromboembolic pulmonary hypertension has no specific symptoms or signs. Effort dyspnea is among the first allegations of the patient. It is increasing until it occurs in smaller and smaller efforts and then it is set as rest dyspnea. Gaps are also common in this situation, given by the patient's hypoxia. Nonspecific chest pains occur in advanced stages. Haemoptysis may occur as a result of expansion and rupture of vessels by increasing intravascular pressure. Peripheral edema,

2 hepatomegaly, jugular stasis are the signs of right heart failure with secondary tricuspidian regurgitation Clinical and laboratory diagnosis. It goes through the usual stages: history, objective clinical examination, routine investigations and specific investigations before surgery. Cardiothoracic radiography it may be normal or it may show signs suggestive of segmentary or lobar pulmonary artery occlusion (Fig.). Central pulmonary arteries are enlarged, increasing the right cardiac cavities, right atrium and right ventricle, peripheral olighemia. Fig. Chest X- ray shows enlargement of heart shadow on the right heart cavities dilatation, dilatation of the right lung with reduced peripheral blood. (Pat N., 60 years, IBCV, Timisoara) Electrocardiogram - may show signs of right ventricular hypertrophy, with right electrical axis deviation, right branch block or hemiblock (Fig.). CT-scan it is normal or shows diffuse nonuniform perfusion. CT-scan of ventilation-perfusion is the investigation which excludes both acute and chronic thromboembolism if it is normal.

3 Fig. CT-scan shows thrombi in the right pulmonary artery. Pulmonary angiography and cardiac catheterization - is the clearest method of diagnosis and assessment of the degree of severity (Figure). It is biplane, the digital subtraction angiography performed by a specialist may show vascular bed irregularities, intravascular filling defects, stenosis and /or occlusion of central, lobar, peripheral and segmentary vessels, it calculates pulmonary artery pressure and pulmonary vascular resistances most accurately, a criterion in assessing the surgical risk. Pulmonary angiofiberscopy - is rarely necessary when angiography is not clear on patient diagnosis and operability. It is difficult to differentiate primitive pulmonary hypertension from the thromboembolic disease of the small distal vessels. In these situations pulmonary angioscopy is used. Echocardiography highlights the right heart cavities, AD, VD, enlarged in volume and geometry-modified, the pulmonary artery pressure is above 50 mmhg, tricuspidian regurgitation grade II-III, but with left ventricular function within normal limits. It can detect an oval patent foramen with right left shunt or other congenital anomalies (anomalous venous drainage). Coronarography - is made in patients over 45 years old to exclude an associated coronary artery disease. It rates left ventricular function and excludes alternative congenital defects.

4 Fig. Pulmonary artery angiography shows the presence of a massive thrombus in lower right lobar artery, 90 mmhg pressure in AP. Angiography of digital subtraction. Multislice CT-dimensional reconstruction - is a modern investigation, it shows high performance and accurate picture of stenosis and obstructions on all pulmonary branches (Fig.) Fig. CT Angiography - tridimensional multislice scan, which emphasizes the irregularities of pulmonary vessels (Neuromed - Timisoara) Natural evolution and prognosis. Natural evolution is very severe. In the absence of surgical treatment, the evolution of these patients is to death, inversely proportional to pulmonary hypertension value.

5 Management of pulmonary hypertension secondary to thromboembolism. Medication treatment - treatment with chronic anticoagulation Sintrom brings INR in the therapeutic area of 3 to 3.5. It is required both preoperatively and postoperatively. Right ventricular failure is treated with diuretics, pulmonary vasodilators (Cialis). In case of inferior vena cava thrombosis, a cav filter is placed to prevent recurrent pulmonary embolism PULMONARY THROMBENDARTERECTOMY It represents the only effective, curative treatment of chronic thromboembolism with severe secondary pulmonary hypertension. It consists of complete removal of thrombotic fibrotic material from the pulmonary artery tree, as distal as possible. The operation is performed by cardio-thoracic surgeons using extracorporeal circulation (heart-lung machine), in deep hypothermia at 18 * C, with periods of total intermittent circulatory stop, to have dry operating field and to remove all thrombotic material (Fig.). Fig. Pulmonary thrombendarterectomy surgery, intraoperative aspect (W. Klepetko, M. Gaspar), specimen removed from right pulmonary artery (February Institute of Cardiovascular Diseases, Timisoara) Surgical indication Once diagnosed with HPT indication for surgery is depending on the severity of the condition of the patient, associated diseases. There is not any other more effective treatment. Atrial septostomy - is a palliative method, first used in 1983 to create an interatrial septal defect, with symptomatic relief through right heart decompression and blood mixture. It can be done in the cardiac catheterization laboratory, using a specific probe- flask, Raskind. It is performed when the surgery risk is too high, in very advanced stages of disease for symptomatic relief and prolongation of evolution.

6 Surgical technique. The surgery is done by ECC median sternotomy. The ascending aorta, superior and inferior vena cava and right sides of the cannula occlusion are cannulated. The heart is protected like in routine heart surgery, by administering cardioplegia in aortic root in order to stop, and then intermittently every 20 minutes, 200 ml St. Thomas II cardioplegia. Once ECC started, the patient begins to cool at 18 * C. At this time right pulmonary artery is dissected between ascending aorta and superior vena cava and is exposed using a Beckman separator with plastic- protected arms to avoid vessels tear (Fig.). Fig. Exposure of right pulmonary artery between the superior vena cava and aorta. Right pulmonary arteriotomy The vent placed in the pulmonary artery trunk ensures a relatively clean field. A longitudinal arteriotomy in the right pulmonary artery extending in Y in the superior lobar and right inferior artery is performed for better access. To perform pulmonary TEA long special decollators and more thin metal aspirators are used from the pump and Cell-Saver. Sometimes thrombotic material is clear and finding clivage plan between intima and media is easy. With great care in order to avoid vascular wall tear, but at the same time firmly intima is decollated with thrombotic-fibrotic material on all pulmonary vascular axis. Sometimes the opening intravascular pulmonary artery may be deceptively normal in appearance and only experienced surgeons can find the right decollation plan. Complete detailed removal, as distal as possible, is the art of this technique and the immediate and late postoperative result depends on it (Fig.).

7 Fig. Thrombotic material extracted from right and left pulmonary artery (Pat. N., 60 years, Pat. D. I, 56 years - IBCV, Timisoara 2006) When the patient's temperature reached 18 * C the pump stops for short periods of minutes to get pulmonary vascular field clearly visible. Based on intraoperative observation of intravascular obstructive material, Jamienson divides it into four morphological types: type I - thrombotic material is present in the central area (Fig.) - Type II - with intima thickening, fibrous bands,-type III occlusions of segmentary and subsegmentary rivers, which requires a distal approach and - type IV - secondary thrombosis in situ with primary pulmonary hypertension, which requires pulmonary transplant. After TEA is performed in the right side pulmonary artery is sutured with Prolene thread 6 / 0. Then the left pulmonary artery is exposed and it will be treated in the same way (Fig.). Fig. Exposure of left pulmonary artery After extraction of thrombotic from the left side, it is sutured and the patient reheated from 18 to 37 * C, which is done in about 90 minutes. When the temperature reaches above 30 * C, the aorta may be declamped and heart starts spontaneously or electrically defibrillated. Hemostasis is rigorously controlled and when the patient has been reheated at 37 * C, the ECC can be stopped. Lung Transplant, Heart - Lung Transplant- is made only when the indication for TEA is outdated, in patients with severe primitive pulmonary hypertension and distal intravascular thrombosis. These cases are difficult to diagnose, pulmonary TEA may be attempted first and in case of failure the patient is pulmonary transplanted. Postoperative care. Complications It must be extremely meticulous. Patient monitoring is completed from the operating room by the catheter Swan - Ganz, to measure pressure in the pulmonary arteries which decreases significantly if conducted accurately and completely. Specific postoperative complications of this surgery are: crises of pulmonary hypertension treated with pulmonary vasodilators (nitric oxide, sildenafil, Viagra). They can be caused by incomplete removal of embolic material or incorrect indication in long-lasting secondary pulmonary hypertension forms. reperfusion edema. Occurs on the 3rd postoperative day and expresses the radiological opacity occurring in pulmonary fields and pulmonary arterial blood desaturation, decreased PaO2. These

8 patients usually stay intubated several days, up to ventilation and cardiovascular stability. Management must be prompt with diuretics, sometimes ultrafiltration, FiO2, as low as possible, but at least a 90% PaOr. Administration of nitric oxide may be useful in some situations. In some cases ECMO is set. This population is very susceptible to bleeding (deep hypothermia, chronic liver stasis, previous anticoagulant therapy) and infection. Arrhythmias - are those common in heart surgery, ventricular atrial extrasystolic, atrial fibrillation which may be present preoperatively. Neuropsychiatric disorders - may be the result of over 30-minute circulation stop. Fluid pericarditis - accumulation of pericardic and pleural liquid requires placement of drain tubes and keeps them longer than in heart surgery. Results. This type of surgery is not performed in many centers. High mortality in the beginning gradually decreased along with increased experience in surgical technique, selection of cases, and improvement of intensive care. Currently at peak centers, mortality is of 5-7%. Hemodynamic outcome is sometimes spectacular, patients in NYHA class III - IV become active and some resume their professional activity. Postoperative echocardiographic examination shows reduced right ventricular geometry and overload, decreased pulmonary artery pressure, disappearance or reduction of tricuspidian regurgitation.

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