Entries in Hypertrophic Cardiomyopathy (2)


Hypertrophic Cardiomyopathy Kills Seven in One NJ Family

Courtesy Lisa Salberg(NEW YORK) -- Lisa Salberg has so far lost seven family members to an insidious heart disease -- a medical mystery that took four generations of tragedy to unravel.

Her great-great uncle, an Irish immigrant, mysteriously dropped dead at the age of 19 in a New Jersey iron mine a century ago.  At 50, her great-grandmother died of "dropsy" -- an old-fashioned term for the accumulation of fluid associated with heart failure.

Salberg's grandfather had a heart murmur and died at 43.  Her father missed a date with her mother because he had to administer CPR to his dying father.  

Salberg's aunt died at 36 of "the flu."  Another aunt died of a stroke at 52, and an uncle died of heart failure at 48.

Fast forward to the mid-1970s, when Salberg's sister Laurie was properly diagnosed with what looked like the cause of death of so many in her family: hypertrophic cardiomyopathy (HCM), or an enlarged heart.

Her death in 1995 hit Salberg the hardest, as she struggled to raise Laurie's children along with her own newborn daughter.

"I don't know how many there are, but we are dropping like flies," said Salberg.  "The pieces came in dribs and drabs, and it took years to get the information imparted.  There was no connecting the dots."

Their father was diagnosed with HCM in 1989 and died in 2008.

"It affected generations of people and it's everywhere in my family," said Salberg, 44, who got her diagnosis in 1979.

Salberg's now 17-year-old daughter, as well as Laurie's children, her niece, 28, and nephew, 30, also have HCM.  Several cousins also have the disease.

Cardiomyopathy is a familial disease that primarily affects the muscle of the heart. With HCM, the normal alignment of muscle cells is disrupted, a phenomenon known as myocardial disarray. It also causes disruptions of the electrical functions of the heart and, depending on whether it obstructs the outflow of the heart from the left ventricle, can be obstructive or nonobstructive.

HCM is an autosomal dominant genetic condition, which means the mutation only needs to be passed down from one parent.  Because cardiomyopathy is a spectrum of diseases, each person is affected differently.

The disease is "actually pretty common," affecting about 1 in 500 Americans, said Dr. Sripal Bangalore, assistant professor in cardiology at NYU Langone Medical Center in New York City.  An estimated 600,000 Americans are living with the disease.

"A lot of people walking around lead a normal life into their 70s and 80s with no problems," he said.  "At the other spectrum, young athletes die while playing sports."

Often there are no symptoms, so the disease is diagnosed by evaluating family history.  Children at risk should have an echocardiogram to see if the heart muscle is enlarged.  That must be repeated every five years until adulthood and is not always conclusive.  Many doctors do not recommend genetic testing because of its complexity -- there are more than 1,000 genes associated with cardiomyopathy.

Treatments may include medications like statins, beta blockers and calcium channel blockers; surgery to burn away the thick part of the heart muscle; and implanted defibrillator devices.

Copyright 2013 ABC News Radio


Texas Teen Posted Powerful Videos Before Christmas Death

A Texas teenager, Ben Breedlove, who cheated death three times despite a dangerous heart condition died on Christmas night from a heart attack, but not before posting a two-part video on YouTube telling his story and describing a series of powerful visions. (Youtube)(AUSTIN, Texas) -- A Texas teenager who said he cheated death three times despite a dangerous heart condition died Christmas night from a heart attack, but not before posting a two-part video on YouTube telling his story and describing a series of powerful visions.

In the videos that have since gone viral, 18-year-old Ben Breedlove of Austin can be seen silently sitting in a room and using handwritten note cards to tell his story. The teen suffered from hypertrophic cardiomyopathy, a condition in which one part of the heart is thicker than the other parts, making it difficult for the heart to pump blood.

He described cheating death three times.

The most recent scare was Dec. 6, when he passed out at school and awoke surrounded by EMS medics preparing to use shock pads to revive him.

He posted a two-part video Dec. 18 titled "This is my story." One week later, on Christmas night, he suffered a heart attack and died. As of Wednesday afternoon, the first video had been viewed online more than 476,000 times.

"It was obvious to all of us that knew him that he knew what he was doing when he made that video," close family-friend Pam Kohler said. "There are times that [the family is] overwhelmed by the pain and the loss of Ben, but then it's replaced with knowing that he was at peace with what was going to happen."

He had two popular YouTube channels, "BreedloveTV" and "OurAdvice4You," on which he would talk about his own life as well as dishing out relationship advice for his peers. Facebook and YouTube have been inundated with tributes to Breedlove.

"When you think of Ben, you can't help but smile," Kohler said. "He was curious, creative. You never knew what he was up to. He was always full of surprises. We look on all of it as a gift from God through Ben."

Kohler and her husband, Mark Kohler, were driving to the Breedlove home Christmas when they first realized something might be wrong.

"We were going over to share Christmas dinner with them that night and on our way over there, a police car passed us with sirens on," Kohler said. "My husband said, 'Start praying because it could be Ben.'"

When they arrived at the house, they saw ambulances and fire trucks. Breedlove had had a heart attack and medics were trying to revive him. He made it to the hospital, but died there.

At this point, Kohler said the family did not know about Breedlove's last videos. He had shared his visions with his sister, but no one had seen the videos.

He said in the video, through note cards, that the first time he cheated death was when he was 4 and described an experience he had in the hospital as he was being wheeled on a stretcher.

"There was this big bright light above me ... I couldn't make out what is was because it was so bright. I told my mom, 'Look at the bright light' and pointed up. She said she didn't see anything," Breedlove wrote. "There were no lights on in this hall. I couldn't take my eyes off it. And I couldn't help but smile. I had no worries at all, like nothing else in the world mattered."

As Breedlove held up each card telling the story, he alternated serious looks with broad smiles. "I cannot even begin to describe the peace, how peaceful it was," he wrote. "I will NEVER forget that feeling or that day."

"Because of the experiences he'd had, he was ready and he was prepared. He really wanted to know that peace again. He was facing more hospital stays and he was tired of it," Kohler said. "He wanted [his family] to know that he wasn't scared and was looking forward to returning to that place."

In the videos, Breedlove went through the details of his journey.

He underwent surgery May 3, 2009, to insert a pacemaker and the second time Breedlove cheated death, he said, was in the summer of 2011. He went to the hospital for tonsil surgery and ended up going into cardiac arrest.

"It was a miracle that they brought me back," Breedlove wrote. "I was scared to die, but am SO glad I didn't."

His third brush with death was the one earlier this month. "I really thought to myself, this is it. I'm dying," Breedlove wrote.

He recalled a dream or vision where he was in a silent, white room with no walls and he felt "that same peaceful feeling I had when I was 4."

"I was wearing a really nice suit and, so was my fav rapper, Kid Cudi," Breedlove wrote.

He said he thought to himself, "Damn, we look good."

"I then looked at myself in the mirror, I was proud of MYSELF, of my entire life, everything I have done," Breedlove wrote. "It was the BEST feeling."

He said he thought of lyrics from a Kid Cudi song that said, "When will the fantasy end, when will the heaven begin?" Kid Cudi sat him down at a glass desk and told him, "Go now."

"I didn't want to leave that place," Breedlove wrote. "I wish I NEVER woke up."

Kid Cudi responded to the videos on his website, writing that he "broke down" when he saw the video.

"This has really touched my heart in a way I can't describe, this is why I do what I do. Why I write my life, and why I love you all so much," Kid Cudi wrote. "We love you Ben. Forever. Thank you for loving me."

Cudi added, "To Ben's family, you raised a real hero, he's definitely mine. You have my love."

Breedlove's funeral is scheduled to take place Thursday afternoon in Austin.

"The one thing he wanted was for everyone to feel joy for him because he knew where he would be and where he wanted to be," Kohler said. "The tears come and they're followed by a smile."

Kohler said Breedlove would be thrilled that his video and story have already touched so many. She said he would have "wanted his generation to know the love of God and the peace he was feeling."

"Do you believe in angels or God?" Breedlove asked on a card at the end of the video. The last card read simply, "I do."

Copyright 2011 ABC News Radio

ABC News Radio