Pulmonary bubble microstitis combined pulmonary arterial hypertension left lung transplantation anaesthetic management 1 case

1Case informationpatient male, 52 years old, height 170 cm, weight 50 kg, due to "chest tightness for four years, aggravated ten days of admission", nearly half a year repeated gas chest does not heal, by fiber bronchoscopy (TBLB) biopsy, prompted for pulmonary blister microstitisChest CT tip: two lungs see more bubble-like light and large areas of grinding glass high-density shadow, left gas chest, left lung compression 30%Preoperative diagnosis: (1) alveve osmosis, (2) left-hand gas chestIt is proposed to assist in the in vitro membrane lung (ECMO) downstream left lung transplantpatient sit in the operating room at 12:30 on April 9, 2018, giving the mask oxygen absorption at a slope of 15 degrees, opening the peripheral veins, and monitoring the oxygen saturation of the artery, electrocardiogram and pulseWhole hemp induction drug is: Medaaazole 2mg, relying on mitenrol 20mg, shun benzene sulfonate aquuke ammonium 20mg and Shufentani 20ug, choose 37 left double cavity bronchoscoscoconal catheter intubation, after the intubation to check the catheter position, to ensure that the catheter position is correct, double lung isolation perfectanaesthetic maintenance using pump-in-apophenol, riffentanis and saqualku ammonium, in the operation according to the anaesthetic depth monitoring of the sub-injection of sufffentani deep anaestheticAfter the completion of anesthesia induction, the right cervical veins were placed with the Swan-Ganz catheter and the double cavity central venous catheter, respectively, to monitor pulmonary artery pressure (PA), through the left femoral artery intubation, the pulse profile temperature dilution of continuous heart displacement monitoring (PICCO monitoring), and to monitor the extravascular pulmonary water (EVLW) and peripheral vascular resistance index (SVRI)Considering the presence of pulmonary arterial hypertension, instant pulmonary artery pressure of 86/53mmHg, and intraoperative single pulmonary ventilation can not satisfy oxygenation, through the right femoral artery and femoral intubation line in vitro membrane pulmonary assisted oxygenation, flow is 2.5L/min, ECMO transcirculation pulmonary artery pressure reduced to 51/35mmHg14:35 patients in the right lycdown down the left breast open, 15:16 left pulmonary artery blocking, blocking the pulmonary artery pressure gradually increased to 96/65mmHg, intravenous pump prosther to reduce pulmonary artery pressureThe procedure is to match the left bronchial tube, pulmonary artery and pulmonary vein sin in turnPulmonary artery opening before 15min to the methyl nylon 500mg, 17:30 left pulmonary artery began to open, open 10min found that the pulmonary artery pressure did not drop, but rose to 112/78mmHg, 17:44 electrocardiogram chamber tachycardia, and then quickly turned into a fibrillation chamber, immediately intrathal heart massage, while again blocking the pulmonary artery, increase ECMO flow to 3.5 L/min, intravenous epinephrine 100ug, Lidoca in 80mg, to be thin-fibrillation to rough tremor after intracardial defibrillation, energy for 20 joules, after the re-jumping pump epinephrine 0.08ug kg-1 min-1 to improve myocardial contraction, pump injection of nitroglycerin 0.5ug kg-1-min-1 to reduce the pre-loadSlowly open the pulmonary artery, after 30min pulmonary artery pressure gradually reduced to 49/34mmHg, after surgery to replace the single cavity trachea catheter, 18:45 with ECMO into the ICUThe blood loss was 1600mL, the urine volume was 1200mL, the blood transfusion was 1600mL, and the crystal colloid was 1500mL The patient removed ECMO 41 hours after surgery, removed the trachea catheter from the ventilator on the 3rd day after surgery, left the ICU on the 7th day after surgery, and recovered and discharged from the hospital after 45 days 1 left lung specimen; Figure 2 specimen cut-out to see large amounts of calcium phosphate microcrystalline deposition 2.Discuss alveolir microlitiosis (PAM) is a rare diffuse lung disease, with alveoliic intra-polyphosphoric phosphoric acid Calcium salt microcrystalline is widely deposited as the main feature, is an autosomal recessive genetic disease, it is believed that the sLC34A2 gene mutation of the alveolar epithelial ii.b sodium phosphate transporter protein causes calcium phosphate-rich microstones to form and accumulate in the alveoli, is the main cause of the disease The course of alveolar micropathy is slow, most patients can have no clinical symptoms or mild symptoms in the early stage, late stage can occur interpulmonary fibrosis, concurrent emphysema, emphysema, emphysema, and then develop into pulmonary arterial hypertension and pulmonary-induced heart disease there is no definitive medical treatment for alveolar micropathy, adrenal corticosteroid therapy is mostly ineffective, most cases are treated with bronchopulmonary irrigation, for patients with end-stage alveoli microscosis, lung transplantation is an effective treatment Lung transplant patients due to long-term lung disease lead to low lung function, has entered the stage of respiratory failure, and different degrees of combined pulmonary arterial hypertension or right heart enlargement, heart function is fragile, anesthesia induction period is very prone to low blood pressure, and even cardiac arrest, should be under the monitoring of invasive arterial pressure slow administration, sub-administered, and prepare vascular contraction drugs, can be combined with the throat and tracheal surface anesthesia, reduce trachea stress, avoid severe low blood pressure During anaesthetic, according to hemodynamic monitoring index and anaesthetic depth index, adjust the depth of anesthesia, in liquid management should be measured to avoid excessive infusion, liquid selection mainly colloid, can be moderately input plasma and albumin, maintain moderate colloidal osmotic pressure, reduce the incidence of pulmonary edema The patient has a long course of disease, has developed into pulmonary hypertension, the main point of anesthesia management is the treatment of pulmonary arterial hypertension, especially during the surgery side pulmonary artery blocking, pulmonary artery pressure will be further elevated, the surgery is very prone to right heart failure In this case, patients found that the pulmonary artery pressure is 86/53mmHg after placing a floating catheter, and the choice of celiac intravenous ECMO transflow can effectively reduce the right heart load and pulmonary artery pressure, reduce the right heart load after the surgical side pulmonary artery blocking, and pump the prosander during pulmonary artery blocking to reduce pulmonary vascular resistance patients in this case after the opening of the pulmonary artery pulmonary artery pressure does not drop back, and the occurrence of ventricular fibrillation, estimated with the patient long-term pulmonary artery hypertension cross pulmonary blood flow reduction, left heart long-term pre-load deficiency, resulting in the left heart shrink, myocardial contraction force severely reduced, when the pulmonary artery opened after a large number of pulmonary veinblood into the left heart, short-term left heart overload, acute left heart failure, we again block the pulmonary flow To reduce the left heart load, and after the heart defibrillation to enhance myocardial contraction, the application of nitric acid glycerin to reduce peripheral vascular resistance, after the circulation of stability slowly open lung artery, effectively reduce the burden of the left heart, and continue to ECMO auxiliary transflow after surgery, and assist in the application of strong heart drugs, so that the left heart can gradually adapt to the re-distribution of blood flow after lung transplantation, the use of gradually reduce ECMO flow, to the left heart function of the basic normal removal of the left heart function