Peter Brunner makes a visually arresting black-and-white debut with this experimental study of a man suffering from Marfan syndrome.
Experimental narrative “My Blind Heart” follows a man suffering from the effects of Marfan syndrome — a genetic connective tissue disorder that can affect numerous body parts and functions — as he lives a semi-feral life in Vienna after the death of his mother. Visually arresting in high-contrast black-and-white, Peter Brunner’s debut feature is hardly a hot commercial prospect, but will appeal to fest and cinematheque programmers looking for challenging, accomplished new work.
Read More: Variety
A common drug that is used to treat high blood pressure in the general population has been found to significantly reduce a dangerous and frequently fatal cardiac problem in patients with Marfan syndrome.
Results of the COMPARE (COzaar in Marfan PAtients Reduces aortic Enlargement) study reveal that patients treated with losartan (Cozaar) had a significantly reduced rate of aortic enlargement after 3 years compared to patients who did not receive the treatment.
“Our study is the first large, prospective randomized study to assess the effects of losartan on aortic enlargement in adults with Marfan syndrome, and confirms previous findings in a mouse model,” said lead investigator Maarten Groenink MD, PhD from the Departments of Cardiology and Radiology at Academic Medical Centre in Amsterdam, The Netherlands.
“We’re very excited to see that such a commonly used drug that is not expensive and has a familiar side-effect profile could have a significant effect on this very serious and frightening risk factor for these patients. These findings may change standard clinical management.”
Marfan syndrome, a heritable connective tissue disorder, affects 2-3 in 10,000 people. It causes progressive enlargement of the aorta, making it prone to rupture, which can be fatal in more than 50% of cases. Currently, the only effective treatment is prophylactic surgical aortic root replacement.
Read More: Yottafire
With his trailblazing plan, a Lankan doctor from Oxford seeks to fill a life-threatening void in heart surgery in the North.
Spotting whom many call “the tallest person in Sri Lanka”, he walks up to him and tells him to hold out his hands, palm downwards, followed by a look into his mouth. After a cursory glance in which he sees “long thinned fingers and a high-arched palate”, he murmurs that 7’3″ tall Kunasingam Kasinthiran has features of Marfan Syndrome and should get it confirmed and followed up at the Jaffna Teaching Hospital.
As he questions Kasinthiran about some of the symptoms that he has been experiencing, it is obvious that he knows what he is talking about. For, Marfan Syndrome is a genetic disorder of the body’s connective tissue which can cause the aorta (the main vessel taking blood from the heart to the body) to become stretched or weak.
It is not only this very knowledge and skill but also deep concern that has brought eminent Heart Surgeon Dr. Ravi Perumalpillai all the way from Oxford in England to Jaffna in Sri Lanka. The prime mover behind the Oxonian Heart Foundation (OHF), he hopes to help hundreds of men, women and children in the Northern Province as well as the North Central Province and the Eastern Province.
Read More: The SundayTimes
Objectives Mitral valve (MV) regurgitation is a common manifestation in patients with Marfan syndrome (MFS) and is age dependent. Valve pathology shares some features with myxomatous MV disease. Surgical treatment is still being debated and not well characterized in patients with MFS.Patients and Methods We retrospectively evaluated the results of mitral valve repair (MVR) of symptomatic patients with MFS who underwent surgery between January 2004 and April 2011. MFS was diagnosed following the Ghent criteria. MVR was performed in 12 patients. Three patients underwent minimally invasive MVR despite severe thorax deformities. Mean follow-up was 60.1 months (95% CI: 48-72) and was complete.Results Thirty-day mortality was 0%.
Full Article: http://www.ncbi.nlm.nih.gov/pubmed/23839872?dopt=Abstract
Personalized external aortic root support has completed initial evaluation and has technology appraisal in the UK for patients with Marfan syndrome for use as an alternative to root replacement. Its long-term success in preventing aortic dissection remains uncertain. Here, we report a study in sheep to establish whether the externally supporting mesh, as used clinically, is biologically incorporated. The strength of the resulting mesh/artery composite has been tested.
The carotid artery of growing sheep (n = 6) was enclosed in a mesh sleeve made of a polymer, polyethylene terephthalate. After a predefined interval of 4–6 months, a length of the artery was excised, including the sleeved and unsleeved portions, and was stress tested and examined histologically.
Full Article: http://icvts.oxfordjournals.org/content/17/2/334.short?rss=1
- Tyson Wallis was just 30 years old when his heart ruptured
- He had a rare genetic syndrome, and now his baby daughters are getting treatment for the same condition
Tyson Wallis had it all. Seemingly healthy at 30 years old, Tyson and his wife Kristin were deeply in love. After years of trying, they finally had the family they always wanted — twin baby girls, Olivia and Eleanor.
“He kept saying, ‘I finally have everything I’ve ever wanted,'” Kristin Wallis said. “It was just so perfect. It was really perfect.” But tragically, “perfect” couldn’t save Tyson from a silent killer — a killer he had been living with all of his life.
Ten months after their daughters were born, Tyson said goodbye to his wife as she rushed off to work. They said they loved each other, as always. Before Kristin even got to work, a frantic call sent her into a tailspin: Tyson had collapsed. He wasn’t breathing. His heart had ruptured, and within hours, he was dead. Within hours, the life he had worked so hard to achieve was gone.
“Out of the blue, my entire world fell apart,” Kristin says. “He didn’t have heartburn. He didn’t have heart or chest pains. He didn’t have anything. He was perfect. He was fine.”
Tyson Wallis had Loeys-Dietz Syndrome, a condition so rare that only a few hundred people have been diagnosed. For Tyson, his diagnosis came too late, but it may help save the lives of his daughters. The condition enlarges the aorta and tangles smaller blood vessels, weakening them and putting the sufferer at risk for a heart attack or stroke. It is also genetic, and after a few tests, it was determined that Olivia and Eleanor also have Loeys-Dietz Syndrome. Without the tragic discovery of their father’s condition, they may have never known.
Read on: http://www.hlntv.com/article/2012/06/29/dads-death-may-save-daughters-life-loeys-dietz-syndrome
May 21st started out like any other for Andrew Mattison. Before the day was over the 26-year-old timberjack would find himself in a battle for his life and an eight and one-half hour surgery with less than a five percent chance of survival.
That morning Mattison went to work at The Great Smoky Mountain Lumberjack Feud where he works as a performer. On this particular day he was meeting with three co-workers that were helping organize the Stihl U.S. Timbersports Competition. Mattison was seated at a desk in front of computer on a conference call with event organizers. In the office with him were co-workers and long-time friends Chuck Peters, Mike Downs and Matt Samler…