Actor and former California Gov. Arnold Schwarzenegger announced that he had undergone a procedure at the Cleveland Clinic to replace his aortic valve. He tweeted:
“Thanks to the team at the Cleveland Clinic, I have a new aortic valve to go along with my new pulmonary valve from my last surgery. I feel fantastic and have already been walking the streets of Cleveland enjoying your amazing statues. Thank you to every doc and nurse on my team!”
As the “Terminator” star stated, this was not his first valve replacement surgery. His mother had a history of heart problems (two abnormal heart valves), and when he took her to a cardiologist in the late 1970s, found out that his maternal grandmother also had a heart problem and died at 67 years old. The doctor told Schwarzenegger that he should have his heart checked, and he was found to have a congenital heart condition, a bicuspid aortic valve.
Afterward, he had his heart checked regularly until 1997, when the cardiologist said that his aortic valve was failing and that he should have his valve replaced. Because he felt that a mechanical valve (which would require long-term blood thinners) might limit his physical activity (not to mention his acting career) and capacity to exercise, Schwarzenegger elected to undergo a surgery called a Ross Procedure. In this procedure, a patient’s own pulmonary valve is removed and placed in the aortic valve position. The removed pulmonary valve is then replaced with a human tissue valve (typically a cadaver valve). The Ross Procedure is not widely used, for although it eliminates the need for anticoagulant treatment, tissue valves are not as durable as mechanical valves, and may require replacement again in the future.
In a 2016 interview with journalist Graham Bensinger, Schwarzenegger revealed that the procedure did not go as smoothly as he had hoped. Initially, things seem to be going well enough that he admitted to getting on a Lifecycle bicycle while still hooked up to all his monitors shortly after surgery (How did he get away with that?!). But later that day, because “my body was too big and too strong,” the pressure on the new aortic valve (previously his pulmonary valve) was too high. He started to cough, and he became short of breath as his lungs began to fill with fluid. His physician, Vaughn Starnes, MD, told him that the surgery had failed and that he needed to be taken back to the operating room to repair the problem. The repeat procedure was successful, and he continued his acting career as well as serving as governor of California from 2003-2011.
As mentioned above, over time, tissue heart valves wear out and need to be replaced. In March 2018, Schwarzenegger had his pulmonary valve replaced. Although he hoped to have the valve replaced using a minimally invasive catheter-based intervention, he ultimately wound up having an open heart procedure instead. Afterward, his publicist reported: “Update: @Schwarzenegger is awake and his first words were actually ‘I’m back’, so he is in good spirits.”
Last month, Schwarzenegger tweeted that he had his aortic valve replaced at the Cleveland Clinic. Although he didn’t say whether this was an open heart or minimally invasive procedure, the same tweet included pictures of “the Governator” walking around Cleveland admiring their statues.
Common Valve Defect
A bicuspid aortic valve (BAV) is the most common congenital heart defect, occurring in approximately 1%-2% of the general population. It is two to three times more common in males than in females.
In a bicuspid valve, two of the three leaflets of the valve fused together, leaving two leaflets of unequal size. The larger leaflet is referred to as the conjoined leaflet. Functional abnormalities of the valve may depend on the absence or presence of redundant tissue in the leaflets. Aortic stenosis tends to occur with little to no redundancy, while aortic insufficiency is more common with redundant tissue.
BAV can be sporadic or familial. The mode of inheritance is variable but may be autosomal dominant. Approximately 20%-30% of family members will also have a bicuspid aortic valve. It is recommended that anyone with a first-degree relative with BAV have a cardiac evaluation.
The fusion of right and left coronary cusps are most common and this anomaly is associated with coarctation of the aorta (50%-75%). Fusion of the non-coronary cusp and right coronary cusp can be associated with valvular abnormalities like aortic stenosis and aortic regurgitation. Other associated cardiac abnormalities include Turner syndrome (30%), ascending aortic dilatation, aortic aneurysm, and abnormalities of the coronary arteries.
Although it is agreed that BAV occurs at the time of embryological formation of the valves, the exact mechanism by which this happens is currently unknown.
The natural history of BAV is highly variable. Initially, a patient may be asymptomatic — although on the physical exam they may have a low-pitched ejection murmur and ejection click. Although a small percentage of patients will remain asymptomatic, the majority (about 75%) will experience progressive fibrocalcific stenosis of the valve and will eventually need replacement. Some patients will experience increasing aortic regurgitation, also requiring repair. Symptoms of BAV are primarily related to the degree of aortic stenosis and/or aortic insufficiency.
- Shortness of breath with exercise
- Chest pain
- Lightheadedness or fainting
- Unable to exercise or loss of stamina
- Tiredness (fatigue)
The risk of infective endocarditis is also higher in patients with a bicuspid valve. Patients with BAV should consult their physician as to whether prophylactic antibiotics are suggested prior to dental or other surgical procedures.
Management of adults with BAV
Patients with BAV should be monitored by echocardiogram every 1-2 years, depending on the degree of pressure gradient across the aortic valve. Patients with mild stenosis may be seen every 3-5 years. As patients get older, the frequency of revaluation may increase.
Medical therapy is limited, and consists mainly of treatment of hypertension, if present. It is generally felt that blood pressure should be aggressively controlled to try and slow the progression of aortopathy (i.e., dilation of the aortic at its root). The joint ACC/AHA guidelines suggested the use of beta-blockers as first-line therapy in these patients.
Aortic valve surgery is needed when symptoms develop, especially angina, dyspnea, or syncope. Left ventricular dysfunction and/or severe aortic stenosis or insufficiency are other indications. Surgical options include:
Aortic valve replacement: the damaged valve is replaced with a biologic tissue valve (from cow, pig, or human) or with a mechanical valve. As previously mentioned, tissue valves have a functional lifetime of about 15-20 years, then need to be replaced. Mechanical valves last longer but require blood-thinning medication for the rest of the patient’s life.
Balloon valvuloplasty: a catheter with a balloon at its tip is positioned across the valve. The balloon is inflated, expanding the opening of the valve. In adults, the valve tends to narrow again, and there may be some aortic regurgitation after the procedure.
Transcatheter Valve Therapy: a newer, less invasive procedure for valve replacement. It is referred to as TAVR when it involves the aortic valve. In the procedure, a catheter is threaded into the heart, typically through the femoral artery. The catheter, which is fitted with a collapsed replacement valve, is positioned in the center of the diseased valve. A balloon is then inflated, expanding the replacement valve into place within the natural valve. The catheter is then removed. Sometimes a balloon valvuloplasty is performed prior to the valve insertion if a wider opening is necessary. A video of this procedure can be found in our story of Rolling Stones frontman Mick Jagger, who underwent this procedure in 2019.
Overall, surgical outcomes are excellent, and most patients return to a normal quality of life.
Sources: StatPearls: Bicuspid Aortic Valve; Bicuspid Aortic Valve Disease: A Comprehensive Review; NHLBI
Michele R. Berman, MD, and Mark S. Boguski, MD, PhD, are a wife and husband team of physicians who have trained and taught at some of the top medical schools in the country, including Harvard, Johns Hopkins, and Washington University in St. Louis. Their mission is both a journalistic and educational one: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.
Last Updated November 04, 2020
Source: MedicalNewsToday.com
