Report seeks answers about mysterious, dangerous heart disease in kids

在一次平静的怀孕之后, 阿曼达·布兰奇菲尔德以为她生下了一个健康的孩子, 一个她和丈夫叫卡什的男孩.

轻微的心脏杂音提示医生有问题. An echocardiogram uncovered cardiomyopathy, a disease of the heart muscle.

In Cash's case, 阻塞使血液难以流经他的心脏, 使它更努力地工作，变得更大. A heart that should have been the size of a strawberry instead nearly took up his entire chest.

“这是他的医生见过的最严重的病例, 因为他的心太厚了,布兰奇菲尔德回忆道.

在他出生后的两个月内, Cash was placed on a transplant list where he waited 51 days for a new heart.

如今，他是一个健康的3岁孩子. But so much about the condition that nearly killed him remains a mystery. It's why a new report from the 美国心脏协会 aims to raise awareness about the disease in children and urge more research to find better treatments.

而儿童心肌病是罕见的, 它可能“导致一些最糟糕的儿科心脏病结果”,“根据 星期二发表的科学声明 in the journal Circulation.

Cardiomyopathy occurs when cells in the heart muscle form abnormally or are affected adversely by other conditions, 使心脏难以有效地泵血. Nearly 40% of children born with cardiomyopathy that results in heart failure or other symptoms either undergo a heart transplant or die within the first two years after diagnosis. The condition is the primary reason children older than 1 year old get heart transplants.

The statement is intended to help declare "what do we know and where are the gaps," said Dr. 声明撰写委员会主席Steven Lipshultz说.

“每个人都想要临床实践指南, 但是这个领域还没有做足够的临床试验来证明, “确实有强有力的证据表明, if you see this, 你应该用这种药, 或者你应该这样对待,’”利普舒尔茨说, chairman of the pediatrics department at the Jacobs School of Medicine and Biomedical Sciences at New York's University of Buffalo.

小儿心肌病的发生率约为百分之一,000名儿童——一些儿童癌症的发病率大致相同, 如淋巴瘤和神经母细胞瘤. But there is far less research on the disease and little improvement in treatment: The percentage of children who undergo a heart transplant because of cardiomyopathy has not declined over the past decade.

这种疾病背后的原因往往是未知的, 尽管在大多数情况下，遗传原因“可能存在”.

心肌病可分为四类. Lisa Yue's husband was diagnosed as a teenager with a type known as hypertrophic cardiomyopathy, 但他的病情被认为是温和的. Still, it was enough of a concern for Yue to mention the disease to the pediatrician for her son Bryan.

"The thought at the time was cardiomyopathy wouldn't show up in young kids," Yue said.

看完儿科医生一个月后, Bryan's undiagnosed hypertrophic cardiomyopathy led to a fatal cardiac arrest. 1999年，他在11个月大时去世.

A year later, the couple welcomed a second son, Kevin, who was diagnosed with cardiomyopathy.

“我记得儿科心脏病学的负责人告诉我们, “我们对这种疾病的了解还不足以有效地治疗它,'" Yue said.

凯文于2001年在等待心脏移植时去世. 他9个月大.

The limited information available at the time led Yue and her husband to create the nonprofit Children's Cardiomyopathy Foundation to help advance research and increase education. Yue said she hopes the AHA scientific statement will "help physicians better understand this potentially life-threatening heart condition so that more lives can be saved."

Lipshultz said he hopes additional research will eventually lead to pinpointing causes behind cardiomyopathy so children can be treated in more targeted ways.

"(This condition) can cause incredible pain and suffering for children and their families, 这对社会来说是非常昂贵的," he said. “随着线上电子游戏飞禽走兽的推移，研究正在带来可衡量的、更好的结果. But we still have a long way to go if we really want to protect and preserve the next generation."