On October 24, 2020, the grand event in the field of pulmonary hypertension, and the first China Pulmonary Hypertension Summit Forum, will be broadcast live simultaneously in Beijing and Shanghai. This forum conducted extensive and in-depth discussions and exchanges on a series of latest hot topics such as cutting-edge data, evidence-based guidelines, and clinical practice in the field of pulmonary hypertension. The
conference covered many top scholars in the field of pulmonary hypertension in China. Mr. Li Bin, Vice President of Xi’an Janssen and Head of Medical Affairs, and Professor Ge Junbo, Academician of the Chinese Academy of Sciences, jointly delivered an opening speech. Academic leaders from the Pulmonary Hypertension Center of Excellence from all over the country gathered in Beijing , Shanghai, two major conference venues, jointly discussed the development of the subject and the frontier of the field, exchanged experience in clinical treatment, continued to write the legend of the future, and realized the vision of life!
This article organizes the wonderful content of the "Dream Builder Sub-forum" special session for readers!
Dream Builder: Guarding the Motherland’s Flowers, Describing the Expected Chinese Dream
Professor Zhang Hao and Professor Hua Yimin co-chaired and launched the meeting to discuss and share children’s pulmonary hypertension with many experts in children’s cardiology or heart surgery (PAH) diagnosis and treatment progress.
Professor Gu Hong pointed out that the IPAH/HPAH and PAH-CHD types of Chinese children with PAH are the most common. Children with PAH symptoms lack specificity and delayed diagnosis is serious. The five-year survival rate is only 37.8%, which is much lower than that of developed countries in Europe and America. IPAH is a diagnosis of exclusion, so it is necessary to perform comprehensive examination and evaluation as much as possible to avoid missed diagnosis or misdiagnosis; children with PAH-CHD should undergo perioperative PAH management as soon as possible; pay attention to the growth and development of children with PAH, WHO FC, NT-proBNP, TAPSE Such indicators can help guide treatment. Bosentan four-leaf clover can improve or stabilize the WHO FC of children with PAH, effectively improve the growth and development of children, and the 4-year survival rate is as high as 84%. The endpoints of children's PAH-specific research are changing. Children's PAH research with event-driven primary endpoints is ongoing, and we look forward to more options in the future.
Professor Yan Jun pointed out that children with congenital heart disease combined with increased pulmonary artery pressure are more common, and the prognosis of children with CHD combined with PAH is worse than that of children with simple congenital heart disease; children with PAH-CHD are most common in the left-to-right shunt type with larger shunt Children with congenital heart disease and left-to-right shunt PAH-CHD will gradually have irreversible structural remodeling of the pulmonary artery; emphasize that the nature of PAH in children with PAH-CHD should be correctly assessed, and different treatment strategies should be adopted: the dynamic type should be operated as soon as possible Treatment; the resistance type should be treated with targeted drugs for a long time; the gray zone patients should be individualized assessment, and the treatment strategy of targeted drugs combined with surgery should be adopted. For PVRi6-8 gray zone PAH-CHD patients, perioperative PAH management is recommended. Targeted drugs should be taken before surgery or combined therapy before evaluating surgical indications; if pulmonary hypertension still exists after congenital heart surgery, single drugs should be used Bosentan/PDE-5i or combined Bosentan+PDE-5i treatment, and long-term follow-up evaluation. Studies have shown that in children with PAH-CHD (left to right shunt), treatment with bosentan can significantly reduce pulmonary artery pressure, improve WHO FC and motor function, and has good safety.
Professor Xu Zhuoming shared new progress in the diagnosis and treatment of children with pulmonary hypertension, pointing out that the mPAP standard for children with pulmonary hypertension is lowered, and the timing of future treatment is expected to be earlier and improve patient prognosis; the application and follow-up procedures of important examinations (such as AVT, etc.) need to be standardized and popularized, and research In children, it is a marker for judging the severity and prognosis of the disease; the treatment of children also needs to maintain a low-risk state as the goal. The latest expert consensus proposes early combined therapy.
Professor Xie Yumei first pointed out the following dilemmas in the treatment of children with PAH: 1) the lack of pharmacokinetic data in children; 2) the lack of evidence-based evidence for children, mainly based on adult clinical research and expert experience; 3) over-instructions The application is more common. It is emphasized that individualized treatment can improve the management of children's PAH; and accurate dosage, safe use and avoiding over-treatment are the basis of individualized treatment for children. Therefore, it is particularly important to carry out PK/PD research in children. The BREATHE-3 study explored the dosage of bosentan for children with PAH and confirmed that bosentan can improve hemodynamic parameters such as average pulmonary artery pressure and pulmonary vascular resistance index in children; from FUTURE-1 to FUTURE-3 studies have shown that bosentan pediatric formulations can improve WHO FC in children with iPAH/HPAH, with a 4-year survival rate of 84%, and it is safe and effective for pediatric patients.
Professor Zhu Limin and Professor Chen Rui shared their experience and experience in the diagnosis and treatment of children with pulmonary hypertension in clinical practice.
Professor Cui Yanqin pointed out that patients with different types of congenital heart disease are at risk of developing PAH. After repaired congenital heart disease, there is still a risk of PAH and should be followed up for a long time; early surgery/interventional treatment for congenital heart disease will have a better prognosis; With PAH, surgical opportunities may be lost as the disease progresses; therefore, the treatment of PAH-CHD should be two-pronged: cardiac repair + continuous management of pulmonary vascular disease. For patients with congenital heart disease with PAH, attention should be paid to distinguish the classification of PAH-CHD, and the surgical indications and contraindications should be clarified: active PAH should be actively operated and long-term management of PAH; patients in gray zone should be treated with targeted PAH drugs Re-evaluate later and adopt the "Treat-to-Treat" strategy; patients with PVRi>8 should be treated with targeted drugs first, and then the chance of surgery should be evaluated; patients with Eisenmenger syndrome and patients with PAH accompanied by small defects should be contraindicated in surgery , Recommend long-term treatment with targeted drugs.
Carry on the past, never forget the original intention, forge ahead, the future can be expected!
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