Emergency surgery followed many missed chances to diagnose illness

Emergency surgery followed many missed chances to diagnose illness

By Sandra G. Boodman


Songer, then 54, was alone in his Jacksonville, Fla., home after a long day at work when he suddenly felt a searing pain — “like something hit me on the back of the head with a sledgehammer” — that radiated through his jaw, down his back and into his legs. He lay down hoping he would feel better and thinking of his wife and children, who were in the process of moving to the family’s new house 60 miles away. After a few minutes he felt worse, so he got up and drove himself to a nearby hospital, announcing as he dragged himself into the emergency room, “I think I’m having a heart attack.”

A few hours later, a thoracic surgeon told Songer he had a 50-50 chance of surviving the emergency open heart surgery he was about to undergo. The following day, Songer learned from his wife the name of the disorder that had caused his cardiac problem — along with several other maladies — which had gone undiagnosed for more than 50 years.

“No one had ever said anything, ever,” Songer said. “It was a huge relief finding out what was wrong,” although he remains convinced that a timely diagnosis might have averted the catastrophic event and spared him years of pain and anxiety from related disorders that had been wrongly blamed on a protein-deficient diet.

Tall and gangly

As a child, Songer was unusually tall and skinny, with severe buck teeth and weak wrists and ankles that were susceptible to repeated sprains. Growing up in Florida in the 1960s and ’70s, none of the doctors his parents consulted found anything to account for his weak muscles. At age 5 he underwent surgery for a double inguinal hernia, a common childhood malady that occurs when soft tissue protrudes through weak abdominal muscles. For many people, hernia repair is a one-time fix; in Songer’s case it would recur repeatedly as he got older.

By ninth grade, Songer was so tall and thin that his classmates nicknamed him “T-bone”: he stood 6-2 and weighed about 110 pounds. As he got older, doctors never offered an explanation for his frequently sprained wrists and ankles or painful lower back.

In 1995, when he was 38, Songer underwent the second of six hernia surgeries after his abdominal tissue inexplicably tore. Twelve years later, during his third hernia operation, the surgeon noted his lack of abdominal muscle. He chided Songer about his diet and advised him to eat more protein, which builds muscle, and to lay off carbohydrates.

Songer protested that his diet, while far from stellar, was not deficient in protein. “I said, ‘I eat plenty of meat,’ ” Songer recalled, although it was clear the surgeon did not believe him.

He was puzzled: Although he had gained weight as he got older, his diet didn’t seem different from that of his friends, none of whom had recurrent hernias. After his sixth hernia surgery in 2009, his doctor told him that losing weight would prevent future hernias. At 6-4 and 228 pounds, he was overweight but not obese.

That same year, Songer’s blood pressure hit 140/90, the threshold for hypertension. His doctor put him on medicine to lower it.

At 51, Songer said, he felt decades older. “Every day I would wake up and think, ‘I wonder what is wrong with me.’ I was feeling lousy and I was thinking to myself, ‘Maybe I really am a hypochondriac.’ I felt very depressed about my health because I could see people around me doing a lot of things.” His doctors, he said, seemed perpetually rushed and didn’t suggest probing the reasons he felt awful. “I don’t think they had a clue,” he said.

Sometimes when he was working strenuously — he and his wife owned a firm that designed and installed green roofs, which required Songer to climb up and down ladders and move material — he experienced a tearing sensation in his neck that would last a few seconds, followed by a brief, intense headache that also disappeared quickly.

He did not mention these episodes to his doctors; he didn’t want to sound crazy.

Almost fatal

The Monday after Thanksgiving, Nov. 28, 2011, was unusually busy. After arriving home around 7 p.m. Songer, who had spent the day working on a client’s roof, took a shower and was packing boxes when he felt an explosive pain in the back of his head, which proceeded down his body.

Songer drove to the hospital, only to discover that the parking spaces near the ER entrance were taken and he had to walk across the garage. He remembers wondering if he could make it inside; he recalls little else other than the ER staff sprinting toward him after he said he was having a heart attack and, later, a radiology tech exclaiming, “Oh my God, look at that aorta!” and realizing that the man was talking about him.

A CT scan showed that Songer had suffered an extensive acute aortic dissection. This occurs when the body’s largest blood vessel, which carries blood from the heart to the brain and other parts of the body, stretches or weakens, causing a tear in the wall of the aorta that allows blood to leak. Dissections can be caused by uncontrolled hypertension, a genetic predisposition or an inherited disorder such as Marfan syndrome.

Actor John Ritter, who became ill on a Hollywood set in 2003, died hours later of an aortic dissection at age 54. So did diplomat Richard Holbrooke, 69, who collapsed in his State Department office in 2010 and died at George Washington University Hospital. Treatment usually involves emergency surgery to repair or replace the aorta; the mortality rate within the first 48 hours is 50 percent.

“It’s very dangerous,” said Nathan R. Bates, the cardiothoracic surgeon who operated on Songer at Jacksonville’s Memorial Hospital. Dissections are sometimes mistaken for a heart attack, as happened in the case of Ritter, whose family received millions of dollars in malpractice payments for the misdiagnosis and subsequent treatment that may have contributed to his death.

Bates said that after meeting Songer and reviewing his CT scan, he was fairly certain that the dissection was the result of Marfan syndrome: Songer’s aortic root had the characteristic pear-shaped appearance seen in such patients, and he had the requisite body type: tall with spiderlike fingers and a narrow face.

Bates said he knew he would have to replace the aortic valve with a mechanical valve and repair the tear with a dacron graft. “You don’t know until you get in there how bad it is,” he said. The chief risk is that the aorta can rupture at any time, which is instantly fatal.

The extent of Songer’s dissection made surgery “a lot more complicated,” Bates said. A portion of the tear that extended into his groin cannot be repaired and is being closely monitored and managed with medications, said his cardiologist Juzar Lokhandwala.

When he learned that his dissection was the result of a genetic disorder present at birth that doctors had missed for more than 50 years, Songer said he was both furious and relieved. His weak wrists and ankles, recurrent hernias and body type were all signs of Marfan syndrome, although he suspects his previous doctors had never heard of it. And those episodes of neck pain followed by a headache were small tears that presaged the big dissection.

A connective tissue disorder that varies in severity and is usually caused by a defect in a gene inherited from one parent, Marfan affects about one in 5,000 people worldwide; many have not been diagnosed. An enlarged aorta is often evident in childhood or adolescence; the disorder, which causes overgrowth of long bones in the body, can also result in eye problems and spinal curvature known as scoliosis. When people with Marfan stretch out their arms, the measurement from fingertip to fingertip exceeds their height.

Medical historians have long debated whether Abraham Lincoln had Marfan; swimmer Michael Phelps, who has some characteristics, was tested but has said he is free of the disorder. Like Songer, most patients receive a diagnosis based not on genetic screening but on physical characteristics and cardiac tests.

“In his case it was so obvious we don’t need to do a genetic screen,” said Lokhandwala.

Songer said he believes one of his sisters who had severe scoliosis had Marfan, as do his two children, who are undergoing genetic testing and are being closely followed by a pediatric cardiologist.

“It really affects them,” Songer said, because they are forbidden to play contact sports, which are deemed too risky for their hearts.

Had he known what was wrong before his near-fatal heart problem, Songer said he would done whatever it took to prevent the dissection, such as strictly controlling his blood pressure and avoiding exertion. Marfan patients are given medicines to drive down their blood pressure; Songer monitors his several times a day.

“I feel like I’ve finally been given a GPS for my life,” said Songer, who can no longer work. “I’m thankful to be alive and I’m grateful that Dr. Bates knew what was happening. At least now, I know what I’m dealing with.”

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