Edited by Andie (Xi Yue)

Recently, the Cardiology Department of Sir Run Run Shaw Hospital (SRRSH), affiliated with Zhejiang University School of Medicine successfully treated a patient with transthyretin amyloid cardiomyopathy (ATTR-CM) again, which not only demonstrated the strength of SRRSH’s medical technology, but also the warmth of compassionate care.

Mr. Jiang (not his real name), aged over 60, was admitted to SRRSH urgently because of chest tightness, shortness of breath, edema of lower limbs, and other symptoms. He was preliminarily diagnosed with pericardial effusion, but later cardiac imaging, endocardial myocardiabiopsy, and gene detection revealed transthyretin amyloid cardiomyopathy (ATTR-CM). This underdiagnosed and potentially fatal disease of the heart muscle, rarely reported in China, is hard to determine with poor prognosis.

In the face of such a disease that comes out of nowhere, Mr. Jiang and his family not only have to bear great psychological pressure, but also face a heavy economic burden for the medications to treat ATTR-CM is prohibitive. In view of this, the Cardiology Department at SRRSH swiftly made a meticulous treatment plan and helped the patient apply for one of the essential medications to treat the condition, tafamidis soft capsules. Thanks to the team’s concerted efforts, Mr. Jiang obtained the drug, which eased his financial burden.

During the treatment, with an enduring sense of responsibility and sympathy, the team of the Cardiology Department of SRRSH closely monitored Mr. Jiang’s condition, adjusted the treatment plan in time, and showed strong support to him and his family. Physicians answered their questions patiently and explained the disease to shore up their confidence. Nurses took care of the patient to ensure his rest and nutrition. It was the dedication of the whole team that finally stabilized Mr. Jiang’s condition.

At present, the treatment for ATTR-CM mainly includes drug therapy and organ transplantation. In terms of drug treatment, small-molecule drugs such as tafamidis and diflunisal, widely used in clinic, can significantly slow down disease progression. Additionally, drugs targeted at gene expression such as patisiran and inotersen are under in-depth research. For patients with more serious conditions, heart transplantation remains an important choice. Though crucial to the treatment, it is not the first choice for all patients for the scarcity of donors and the high risks of transplantation surgery.

New treatment approaches are emerging. For example, SGLT-2 inhibitors, a class of medicine used to reduce blood glucose levels, are found to be able to resist diuretic resistance in patients with ATTR-CM. Moreover, cutting-edge technology such as gene therapy is still in exploration, bringing hope to people suffering from ATTR-CM for more targeted and effective treatment.

Going forward, multidisciplinary cooperation will be the key to improving the diagnosis and treatment level of ATTR-CM. Concerted efforts of specialties such as cardiology, genetics, pathology, and imaging will provide a more comprehensive and personalized diagnosis and treatment plan for patients with ATTR-CM.