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Surgical Treatment Saves the Life of End-stage Pulmonary Hypertension Patients
Source: Posted Date:05-30-2018

Recently, the CTEPH Treatment Center of China-Japan Friendship Hospital (CJFH) has successfully performed an extremely difficult salvage pulmonary thromboendarterectomy surgery for a rare and extremely-critical patient with chronic thromboembolic pulmonary hypertension.

I.The Patient’s Medical History

The patient was a 57-year-old man. He has been beset with dyspnea after exercise for more than 7 years. He was diagnosed as “pulmonary embolism” in the local hospital and felt much better after anticoagulant therapy. However, symptoms like shortness of breath afterexercise, gradual decrease of post-exercise tolerance andanhelation after walking for 50 meters still existed and they were accompanied byedema of lower extremity as well aspleural and peritoneal effusion. The patient was finally confirmed as “chronic thromboembolic pulmonary hypertension and right heart failure” (Figure 1) by the local hospital, receiving no efficient treatment after trying in many hospitals. The patient suffered from severe heart failure and was in great danger. Medical treatment has failed to improve the symptoms. He turned to many well-known cardiovascular hospitals in China for help but it is believed by all the hospitals thatthe patient’s condition was too serious and there was no chance for surgery. Therefore, the patient and his family came to our hospital with the last glimmer of hope.

II. Treatment Process

Right after being admitted to the hospital, the patient was brought to the Respiratory and Critical Care Department II, where a comprehensive examination was performed to determine the extent of the lesion, cardiopulmonary function assessment was conducted and medical treatment was given to maintain the best preoperativecardiopulmonary function condition. According to the preoperative assessments ofRespiratory and Critical Care Department II, Cardiovascular Surgery Department and Anesthesiology Department,there existed great difficulty in the surgery and postoperative management. The patient has a long medical history, and no satisfactory treatment effect can be obtained without removing extravascular lungsmall-vessel lesions andthe remained postoperative pulmonary hypertension; due to the extensive extent of the lesion, pulmonary hemorrhage is easy to occur after thrombectomy;because of severe right heart failure, disused left heart failure and impaired liver and kidney function, postoperative heart failure, reperfusion pulmonary edema and multiple organ failure arepossible to occur; and due to long-term malnutrition and cachexia, it is highly possible for the patient to suffer from postoperative infection. The CEPTH team is willing to give it a go because of the full trust from the patient and his families who enjoy a comprehensive understanding of the disease and treatment risks.

According to the results of the preoperative examination,the surgical and anesthesia teams mapped the range of pulmonary vascular lesions and the resection scheme. At the same time, the extracorporeal circulation was established during the thoracotomy. In response to the prolonged extracorporeal circulation and repeated circulatory arrests, a balanced temperature reduction and recoverymeasure was adopted. Constant surface hypothermic protection for major organs such as the heart and brain was provided and there existedpulmonary arterydistal lesions due to the extensive scope of lesions. The surgical team led by CJFH Vice President Liu Peng, performed the pulmonary thromboendarterectomy surgery completely for the patient, realized adequate hemostasis and removed the pulmonary artery obstructionthoroughly (Figure 2) in 17 hours and 20 minutes according to the accurate judgement of preoperative imaging. Preoperative assessment has been fully performed andpreparations for using ECMO have been made. But due to the inability to maintain the oxygenation and complex conditions such as combined bi-side cardiac dysfunction, a simple VA or VV ECMO cannot meet the needs. After the final decision of the surgical team, the V-AV ECMO of femoral vein-femoral artery-jugular vein was decided to be placed. Although the preoperative preparation was sufficient, it was stillbreath-taking. A complete pulmonary thromboendarterectomy surgery was successfully performed and the patient was transferred to SICU with the aid of V-AV ECMO.

The postoperative management of V-AV ECMO is undoubtedly a huge challenge for SICU. SICU immediately launched an emergency plan. Special personnelwere designated to look after the patient in the daytime andfive male doctors, including the department heads, took care of the patients in turn for night shifts. In addition, three senior advanced doctors also followed up the management and a joint ward rounds consisting of the Cardiac Surgery Department, Department of Respiratory and Critical Care Medicine and SICU twice a day was formed simultaneously (Figure 3).

In terms of nursing care, ECMO’s pipeline maintenance and infection prevention have become the top priority. The Head Nurse of SICU led the nursing team leaders personally to ensure that the patient couldreceive the best intensive care and hospitalization prevention and control measures, providing patients with adequate time and guarantees for cardiopulmonary function recovery.The patient suffered from hypoxia for five times after entering SICU. Hypoxia and hypotension during the postoperative period and on the first postoperative day were considered to be resulted from insufficient circulation volume and ECMO flow and the condition was improved after adopting ultrasound-guided fluid replacement therapy. To avoid reperfusion pulmonary edema and postoperative cardiac insufficiency, CRRT mass dehydration treatment should be used immediately after the stabilization of the circulation. On the second postoperative day, hypoxia was considered to be related to atelectasis. On the 4th day after operation, the ECMO condition was reduced; hypoxia occurred during the deconditioning process;bedside ultrasound showed pneumothorax; and the oxygenation was improved after changing to a negative pressure drainage vessel. On the 5th dayafter operation, ECMO was withdrawn, and on the 6th day, hypoxia was associated with airway bloodysputum bolt. After the sputum bolt was removed through tracheoscope, the oxygenation was stabilized. The tracheal intubation was removed on the 7th day after operation and the patient was transferred to the Cardiac Surgery Department on the 8th day. Right heart failure appeared in the patient’s rehabilitation phase and it was considered to be resulted from partialexudative pericardial constriction of the right heart.The patient discharged from the hospital after receiving hormone therapy and getting better(Figure 4).

III. Treatment Experience

Chronic pulmonary embolism, also known as chronic thromboembolic pulmonary hypertension, is rare in clinical practice. It is characterized by dyspnea after taking exercise and death of respiratory failureeventually. Surgery is the only curing method.

Pulmonary thromboendarterectomy surgery is applicable t

1. Patients with significant symptoms of chronic progressive respiratory failure, hypoxemia and hypercapnia;those receiving no curative effect after 6 months of anticoagulant therapy; and those with NYHA class III and IV.

2. The average pulmonary artery pressure is more than 30mmHg; and the pulmonary circulation resistance is ≥300dyne/(s.cm5).

3. Pulmonary angiography shows that the obstruction range is >50%, which can be achieved through artery surgeryabove the segment of lung and is particularly suitable for those in the main branch of the pulmonary artery or the proximal part of the lobar pulmonary artery. Distal obstruction of pulmonary artery and extensive arteriolar embolizationcannot be removed; and severe right heart failure and critical diseases in other organs are clearly listed as contraindications.

The patient had distal thrombus, severe right heart dysfunction and relevant problems in other organs. This surgery was considered as extremely difficult or impossible to complete by all thehospitals at home and abroad and the prognosis was extremely poor. The reason why CJFH teams dared to accept this challenge was that they had completed many cases of difficult PEA surgeries before and enjoyed mature surgical skills as the basis; second, they were encouraged by the unparalleled understanding and trust of patients and their families, as well as thesound doctor-patient relationship thus they were willing to give it a last try for the sake of the patient’s health; and finally, a strong team is the key to a successful treatment: any weakness or absence of links includingdisease identification by the outpatientclinic, preoperative maintenance by the internal medicine, adequate preoperative evaluation of the anesthesia and surgery departments, formulation of surgical planning, intraoperative situation estimation and emergency response as well ascareful postoperative management and rehabilitation by SICU, can lead to failure of successful treatment for patients.

IV.For further information, please contact the CTEPH diagnosis and treatment team:

Xie Wanmu, Associate Senior Doctor of Department of Respiratory and Critical Care Medicine: Tel.: 15611734410

Zhen Yanan, Attending Doctor of Cardiothoracic Surgery Department: Tel.: 13264217275