2018年 · 第46卷第12期

Two days ago, I read an article on the education system, which counted the total enrollment of more than 500,000 graduate students across the country in the 10 years from 1990 to 1999. Since 2010, the total number of graduate students enrolled in China has exceeded 500,000 every year. The substantial expansion of postgraduate enrollment reflects the demand brought by national development, but at the same time, it is also necessary to slow down the quality of our tutors and the success rate of postgraduate training.

In the 1960s, due to the pandemic of pulmonary hypertension caused by the diet drug amirrex in Europe, the prognosis of patients was extremely poor. In 1973, the World Health Organization invited more than a dozen authoritative experts from Europe and America to hold the 1st World Conference on Pulmonary Hypertension in Geneva, which divided pulmonary hypertension into two categories: primary pulmonary hypertension and secondary pulmonary hypertension. Essential pulmonary hypertension lacks basic epidemiological data and is tricky to treat. Therefore, the Institute of Heart, Lung and Blood of the National Institutes of Health launched a multi-center essential pulmonary hypertension registry study in the United States. In 1987 and 1991, the baseline data and patient prognosis of the registry study were published, respectively-the incidence rate was 1~2/million person-years, and the median survival time was 2.8 years. These two data identify essential pulmonary hypertension as a rare disease with a bad prognosis like a tumor.

The global plateau area is vast, including the Qinghai-Tibet Plateau, the Pamir Plateau, the Bolivian Plateau, the Brazilian Plateau, etc. The permanent population of the plateau reaches 140 million. The Qinghai-Tibet Plateau in China stretches from Qinghai to Tibet, covering an area of more than 2 million square kilometers. It is the highest area in the world, with an average altitude of more than 4,000 m. It is known as the "roof of the world". Because of the extreme environment of hypoxia and alpine cold, it is listed as the "third pole" of the earth together with the Arctic and Antarctic. Because the atmospheric oxygen partial pressure on the Qinghai-Tibet Plateau is only half that of the sea level, except for Tibetans who have the ability to adapt physiologically, most others regard it as a "forbidden zone for life". However, the vast plateau area contains huge resources, such as water resources, solar energy, mineral resources, geothermal resources and tourism resources, which need to be developed urgently; The plateau area is often located at the junction of continental plates, and natural disasters such as earthquakes occur from time to time, which makes it difficult to rescue them; The plateau area is located in the border, with ethnic minorities living in concentrations, and there are many political instability factors, so the task of maintaining stability is heavy; The plateau area is bordered by many countries, in which the Tibetan Liberation War and war with neighboring countries have occurred, etc. Its military strategic position is very important, and it is the key area of China's national defense construction. However, acute or chronic high altitude exposure can lead to hypoxic pulmonary vasoconstriction and/or pulmonary vascular remodeling, resulting in increased pulmonary vascular resistance and elevated pulmonary artery pressure, even right ventricular insufficiency, inducing high altitude pulmonary edema, and significantly affecting the quality of life and working ability. Therefore, it is of great significance to explore the physiological characteristics, mechanism and clinical significance of pulmonary vascular reaction at high altitude for the prevention and treatment of pulmonary vascular diseases and the conquest of extreme environment in high altitude.

With the opening of the national two-child policy, pulmonary hypertension in pregnancy has become a clinical problem that needs urgent attention. pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are common clinical pulmonary vascular pathological conditions. The former is an increase in pulmonary circulatory pressure caused by various etiologies in a broad sense, which is manifested as the mean pulmonary artery pressure (mPAP) ≥25 mmHg (1 mmHg =0.133 kPa); The latter is an increase in pulmonary arterial pressure and pulmonary vascular resistance caused by precapillary pulmonary arteriolopathy due to genetic and/or underlying disease, which is characterized by mean pulmonary arterial pressure (mPAP) ≥25 mmHg, pulmonary arteriolar wedge pressure (PAWP) ≤15 mmHg, and pulmonary vascular resistance (PVR)>3 Wood units measured by right cardiac catheter. Pulmonary hypertension can occur in men and women of all ages, and common types of pulmonary hypertension such as idiopathic pulmonary hypertension and connective tissue disease-related pulmonary hypertension are more common in young women. When a female patient suffering from pulmonary hypertension is pregnant or has new pulmonary hypertension during pregnancy, it is pregnancy complicated with pulmonary hypertension, referred to as pregnancy pulmonary hypertension.

For a long time, the research on left ventricular function has attracted much attention, while the right ventricle is usually regarded as a "bystander" of cardiovascular system and a "victim" of cardiovascular system diseases, while the research on right ventricular function is obviously backward, resulting in insufficient understanding and understanding of right ventricle. Furthermore, the right ventricular muscle tissue is slender, pumps blood only to a single organ, and its own anatomical structure and location, which limits its measurement and evaluation.

In July 2007, the Cardiovascular Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Cardiovascular Diseases jointly released the "Expert Consensus on Screening, Diagnosis and Treatment of Pulmonary Hypertension" in China^[1]Referring to the definition, clinical classification, diagnosis process and treatment strategy of pulmonary hypertension at the 3rd World Congress on pulmonary hypertension held in Venice, Italy in 2003, the consensus put forward practical suggestions for the screening, diagnosis and treatment of pulmonary arterial hypertension (PAH) in China, which played an important role in standardizing clinicians' diagnosis and treatment behavior and improving the clinical diagnosis and treatment level of pulmonary hypertension in China.

The patient was a 70-year-old male. The main cause of "paroxysmal chest pain for 5 years, aggravated for half a month" was admitted to hospital. The patient began to have paroxysmal chest pain 5 years ago. He had been treated as coronary heart disease, oral aspirin and statins, and had always had symptoms. Half a month ago, my chest pain worsened, and I felt chest pain and tightness when I walked, which could be relieved by myself after rest. I went to an outside hospital, checked my electrocardiogram and blood troponin, and was diagnosed as "coronary heart disease, unstable angina pectoris". I went to our hospital for further treatment. Previous history of hypertension for 10 years, blood pressure up to 186/100 mmHg (1 mmHg =0.133 kPa), oral nifedipine controlled-release tablets can control blood pressure below 140/90 mmHg, lacunar infarction for 8 years, no limb mobility disorder. History of diabetes and drug allergies denied. There was no family genetic history of coronary heart disease, no history of smoking, no history of drinking alcohol. Physical examination: Body temperature 36.5 ℃, pulse 63 beats/min, breathing 18 beats/min, blood pressure 146/70 mmHg. The development was normal, the consciousness was clear, the respiratory movement of both lungs was normal, no dry and wet rales and pleural friction were heard, the heart rate was 63 beats/min, the rhythm was uniform, and no murmur was heard in the auscultation area of each valve. The abdomen is flat and the whole abdomen is soft. The liver and spleen were not palpable, and there was no edema in both lower limbs. Admission diagnosis: coronary heart disease, unstable angina pectoris; Hypertension Grade 3; Old cerebral infarction. ECG: Sinus rhythm, roughly normal ECG.

A 52-year-old male was admitted to the hospital on August 2, 2017 due to "intermittent chest tightness and chest pain for more than 1 year, aggravated for 3 days". Past history, personal history, marriage and childbirth history are not special. Physical examination at admission: blood pressure 130/84 mmHg (1 mmHg =0.133 kPa), heart rate 89 beats/min, rhythm is uniform, and systolic murmur of grade 4/6 can be heard between 3 and 4 costs at the left margin of the sternum, conducted to the neck. ECG: sinus rhythm, V_3~6The lead T wave was inverted, and there was no dynamic evolution in the electrocardiogram after multiple reexaminations. Coronary angiography: About 70% local stenosis of the distal circumflex branch. Transthoracic echocardiography after admission showed that the patient had left atrial enlargement [left atrial volume index (LAVI) was 41.2 ml/m²], normal left ventricular systolic function [left ventricular ejection fraction (LVEF) of 67%], asymmetric ventricular septal hypertrophy (ratio of ventricular septum to left ventricular posterior wall thickness of approximately 1.5,FIG. 1A), systolic forward movement of the mitral valve (SAM sign,FIG. 1B), left ventricular outflow tract obstruction at rest (peak and mean left ventricular outflow tract pressure differential of 75 and 31 mmHg, respectively), and the short axis section of the parasternal aorta showed the aortic valve as a bifoliate valve (Figure 1C), mildly restricted valve opening (peak and mean aortic valve pressure differential of 32 and 22 mmHg, respectively), combined with mild to moderate aortic regurgitation, and the fishmouth-like morphology of conventional mitral valve opening was not observed in the short axis section of the parasternal mitral valve, but clover-like morphology (Figure 1D), the curved rectilinear pattern when the normal mitral valve is closed also becomes a Mercedes-Benz logo-like pattern (Figure 1E), the three leaflets are similar in size (length: septal leaflet 28 mm, lateral leaflet 27 mm, posterior leaflet 27 mm), and there are three leaflet junctions (anterolateral, inferolateral, and posteromedial), the three junctions are equal in spatial distribution, the junction of the three leaflets is located in the center, and the mitral valve open area is in the normal range (the valve orifice area measured in two dimensions is about 5.33 cm²), combined with moderate mitral regurgitation, no obvious abnormalities in the mitral papillary muscle, and normal fibrous connections between the aortic and mitral valves (FIG. 1A), the apical four-chamber heart section suggests normal ventricular position (Figure 1F）。 After admission, the patient completed relevant examinations and was clinically diagnosed as obstructive hypertrophic cardiomyopathy, trilobar mitral valve with moderate regurgitation, bilobar aortic valve with mild stenosis with mild to moderate regurgitation, and coronary heart disease. Patients were instructed to avoid strenuous exercise, and beta-blockers were given to improve ventricular relaxation, reduce left ventricular outflow tract obstruction, antiplatelet and lipid-lowering therapy. The patient's symptoms improved and he was discharged from the hospital. After discharge, he took medication regularly, and his symptoms were acceptable. There was no obvious chest tightness, chest pain and asthma in general daily activities.

hypertrophic cardiomyopathy (HCM) is a genetically heterogeneous myocardial disease characterized by left ventricular hypertrophy that is not entirely caused by abnormal cardiac load^[1]。 The prevalence rate is about 1/500, and more than 1,400 HCM-related mutations are currently found in at least 11 genes encoding sarcomyelin-related genes^[2]。 Due to the presence of gene expression modifications, the clinical manifestations of HCM are diverse, ranging from asymptomatic to sudden cardiac death. The hypertrophic site mainly involves the left ventricle, especially the interventricular septum, presenting a typical asymmetric left ventricular hypertrophy^[3]。 Sometimes the lesion can involve the apex, right ventricular wall and other parts. In patients with HCM, right ventricular wall thickening is relatively common, but simple right ventricular disease or right ventricular outflow tract obstruction HCM is very rare^[4,5]。 Therefore, the 2011 American College of Cardiology Foundation (ACCF) /American Heart Association (AHA) and the 2014 European Society of Cardiology (ESC) guidelines for the diagnosis and management of HCM do not describe the involvement of right ventricular lesions in detail. The 2017 Chinese Adult Hypertrophic Cardiomyopathy Diagnosis and Treatment Guidelines only mentioned that HCM contains a special type of right ventricular hypertrophy^[6]。 In order to deepen the comprehensive understanding and management of HCM, this article reviews the right ventricular hypertrophic cardiomyopathy with HCM involving right ventricle.

Pulmonary hypertension is characterized by progressive increase of pulmonary vascular resistance, which can lead to right heart failure or even death. The main pathophysiological changes are pulmonary vascular and right ventricular remodeling, pulmonary arteriole spasm and in situ thrombosis, etc. Remodeling plays a key role in the pathogenesis of various types of pulmonary hypertension. The mechanisms underlying pulmonary hypertension and its remodeling are complex, including inflammatory states and alterations in cell proliferation, metabolism, autophagy, and apoptosis. Many signaling pathways play different regulatory roles in multiple links of remodeling. Recent studies have found that protein kinase B/mammalian rapamycin target (Akt/mTOR) signaling pathway is involved in almost every link of remodeling. It is of great significance to study the key factors and important proteins in the Akt/mTOR signaling pathway for more accurate targeted therapy of pulmonary hypertension. This paper reviews the research progress of Akt/mTOR signaling pathway and its inhibitors in pulmonary hypertension.