Bicuspid Aortic Valve Aortopathy in an Athlete

Abstract

Background

Bicuspid aortic valve (BAV) is a common congenital condition associated with progressive aortopathy and remains a frequent justification for sporting restriction despite limited data on exercise-related aortic events.

Case Summary

An asymptomatic 18-year-old athlete undergoing evaluation for military service was discovered to possess a type-1 BAV, accompanied by a dilated aortic root (4.2-4.3 cm), with trivial to mild aortic regurgitation. Serial imaging demonstrated rapid progression of aortic dilatation to 4.7 cm. Such findings posed significant implications for military eligibility and athletic participation. Consequently, given long-term aspirations for athletic and military participation, a valve-sparing aortic root replacement was performed.

Review Summary

BAV-associated aortopathy in athletes requires a personalized risk assessment, careful surveillance, and shared decision-making. Despite conservative historical guidelines, the risk of exercise-related aortic dissection is low, and individualized management, including prompt surgical intervention, may allow safe return to athletic participation.

Take-Home Messages

Bicuspid aortic valve aortopathy is a distinct pathological process that may be complicated by physiologic athletic remodeling. Decisions on participation should adhere to established dimensional thresholds and guidelines, while being personalized within a shared decision-making framework and risk stratification. Following surgical intervention, a gradual, supervised return-to-play strategy, guided by postoperative imaging stability and multidisciplinary input, is necessary to ensure the safe resumption of athletic participation.

Visual Summary

Acute aortic syndromes encompass a range of aortic conditions, which collectively account for approximately 1.6% of sudden cardiac deaths (SCDs) among young athletes.¹ Importantly, clinical attention on aortopathies is highlighted by the risk of dissection, which is reported to have an overall annual incidence of 4.7 cases per 100,000 individuals.² Risk factors for the development of aortic dilatation include the presence of a bicuspid aortic valve (BAV), age, male gender, indexed size relative to body surface area, hypertension, family history of dilatation or dissection, and relevant genetic syndromes.³ Athletic training may be a potential risk factor for aortic dilation, as recent data suggest a higher-than-expected prevalence of clinically significant aortic dilatation (defined as >40 mm in diameter).4, 5, 6, 7, 8 The importance of such findings is ambiguous, as the observed enlargement may be a sequela of athletic training or secondary to underlying cardiovascular risk factors, such as subclinical hypertension, or maladaptive remodeling that includes arterial stiffness, diastolic dysfunction, or left ventricular hypertrophy. Nonetheless, the risk of progressing to an acute aortic syndrome or need for intervention is unknown for this population. In contrast, BAV is a well-recognized entity that is associated with progressive aortic dilatation.⁹ Therefore, participating in sports among athletes with BAV and clinically relevant aortic dilatation is challenging, nuanced, and typically focuses on shared decision-making (SDM).

Take-Home Messages

• •Bicuspid aortic valve aortopathy is a distinct pathological process that may be complicated by physiologic athletic remodeling.
• •Decisions on participation should adhere to established dimensional thresholds and guidelines, while being personalized within a shared decision-making framework and risk stratification.

Case Presentation

An 18-year-old male with no significant past medical or family history presented to the sports cardiology clinic for risk stratification regarding military enlistment and collegiate American football participation. His primary goal was to serve in the military with a career aspiration of becoming a fighter pilot. During a preparticipation examination for the Air Force Academy, a systolic murmur was auscultated, prompting further evaluation. He denied any cardiovascular symptoms, including chest pain, dyspnea, or palpitations. Importantly, he was an avid weightlifter, bench-pressing up to 400 pounds, with several years of competitive experience, and training 3 hours per session, 4 to 5 times weekly. He noted no family history of aortic dilatation, dissection, or connective tissue disease.

His height and weight were 188 cm and 96.2 kg, respectively (body mass index, 27.2 kg/m²). Examination was normal. Electrocardiography revealed sinus rhythm with tall QRS amplitude, which was deemed a normal variant (Figure 1). Echocardiography identified a BAV (type 1) with mild aortic regurgitation. There was no aortic stenosis. The ascending aorta was enlarged with effacement of the sinotubular junction and aortic sinuses measuring 4.3 cm (Z-score 3.4) and the proximal ascending aorta measuring 3.6 cm (Z-score of 2.5). The left ventricle was normal in size and thickness with a preserved left ventricular ejection fraction at 66% (Figure 2). Computed tomography (CT) chest angiography confirmed an aortic root dimension of 4.3 cm (1.95 cm/m², indexed for body surface area) (Figure 3).

Figure 1.

Electrocardiography With Normal Sinus Rhythm and Prominent QRS Amplitude, Normal Variant

Figure 2.

Transthoracic Echocardiography Revealing Bicuspid Aortic Valve, Dilated Aortic Root With Mild Aortic Regurgitation

Figure 3.

Computed Tomography Revealing Bicuspid Aortic Valve, With a Dilated Aortic Root With Trivial to Mild Aortic Regurgitation

The presence of an enlarged aorta in conjunction with a BAV was considered clearly pathological in nature. This led to medical disqualification from serving in the Air Force as a fighter pilot, due to the risk of dissection at extreme gravitational forces. However, after extensive discussions with the patient's family and the military medical staff, he was granted a waiver to enroll in the United States Military Academy, utilizing a SDM framework to participate in tactical training and football.

After enrollment, he continued to follow closely with serial imaging and remained clinically asymptomatic. Four years after initial presentation, aortic root dilatation had progressed to 4.7 cm (3 mm increase in 1 year) on magnetic resonance angiography (MRA), a finding confirmed on CT angiography (Figure 4). Based on the rate of growth, he was placed on leave from the Army out of concern for immediate and long-term risks associated with physical activity and his inability to perform his duties. Considering the rate of aortic growth, the high likelihood he would require an intervention during his lifetime, and his career aspirations, he consulted with cardiac surgery. After several multidisciplinary discussions, he elected to undergo a valve-sparing root procedure given his desire to continue participating in Army activities. He ultimately underwent an uncomplicated valve-sparing root-replacement procedure without complications. Once the sternal wound adequately heals, he will undergo postoperative imaging, namely echocardiography, CT angiography, and MRA, to demonstrate graft stability and assess potential complications that might arise from the surgical procedure. During subsequent visits to both the sports cardiology and aortopathy clinics, the primary focus shall be on optimizing blood pressure and minimizing activities that cause isovolumetric strain (eg, weightlifting and Valsalva maneuvers). Initially, he will engage in gradual resumption of light endurance activity, and after 3 months, while collaborating with military personnel, he will begin a phased return to sports and tactical training (Figure 5).

Figure 4.

Computed Tomography Revealing Progression in Aortic Root Dimension on Serial Imaging

Figure 5.

Graduated Return to Play Following Surgical Repair

Maximum predicted heart rate calculated using cardiopulmonary exercise testing. BPM = beats per minute; ADL = activities of daily living; MPHR = maximum predicted heart rate; RPE = rate of perceived exertion; TTE = transthoracic echocardiography.

The Mini-Review

Aortopathy in athletes occurs at the intersection of physiological vascular remodeling and pathological structural disease. Importantly, the cardiovascular and aortic responses to exercise are influenced by training intensity, volume, and the specific type of sport, which determine the pattern of hemodynamic load and subsequent vascular remodeling.¹⁰ Although athletes exhibit slightly larger aortic dimensions in comparison to sedentary controls, these measurements rarely surpass established clinical cutoffs.11, 12, 13, 14 For instance, a study involving ∼2,300 athletes found that 1.3% of male Olympians had an aortic root measurement >40 mm.¹⁵ Moreover, the prevalence may be considerably higher among masters and older athletes, with estimates as high as 30%, particularly among retired professional American football players.^5,6 It is crucial to recognize that even in cases of extreme body dimensions, there exists a limit for physiological normality. Therefore, it is essential to consider an aortic diameter exceeding 40 to 41 mm in males, and >36 mm in females, as typically pathological. This is further supported by findings from a National Basketball Association study, which reported that no athlete had an aortic root diameter exceeding 42 mm, with 95% exhibiting a root diameter below 40 mm.¹⁶

The key clinical challenge is the coexistence of a structural abnormality, such as a BAV, which is well recognized as a clinically important cause of aortopathy. Morphological changes such as sinotubular effacement, mitral valve prolapse, rapid growth (≥3 mm/y), and the presence of aortic regurgitation provide additional clues to pathological variants. Moreover, distinct anatomic phenotypes within BAV aortopathy are associated with varying clinical risks; the “root phenotype,” characterized by dilation of the aortic root, may be associated with a higher incidence of aortic events, emphasizing the significance of underlying congenital aortic wall abnormalities.^17,18 A family history of aortopathy, dissection, or unexplained SCD should further strengthen concern for underlying genetic susceptibility. Ultimately, such theoretical concerns of aortic dissection and SCD have influenced guideline recommendations. However, empirical data indicate that aortic dissection during athletic participation is rare.¹⁹ A recent analysis of sports-related SCDs attributed to aortic dissection during sporting participation revealed an exceptionally low incidence (3%), with only 22% of those athletes having a BAV as the underlying pathology, suggesting current restrictions are likely more conservative than what may be justified.¹⁹

Both the European Society of Cardiology and the American Heart Association (AHA) guidelines, along with the more recent statement, provide nuanced recommendations for athletes with BAV and aortopathies, emphasizing an individualized, multidisciplinary evaluation.20, 21, 22 Importantly, there is a growing emphasis on physiologic assessment and personalized measurements, such as the aortic size index, aortic height index, and cross-sectional area-to-height ratio.²³ Emerging imaging-based phenotypes, such as aortic length and vertebral artery tortuosity, can also likely identify patients at higher risk of dissection.²⁴ Relying exclusively on dimensional cutoffs may miss those who remain at risk of acute aortic syndromes despite having diameters below the thresholds.²⁵ Consequently, risk assessment should incorporate multiple factors, including aortic size, underlying heritable pathology, and sport-specific hemodynamic load.

Different exercise modalities impose distinct hemodynamic stresses on the aortic wall. Endurance training enhances cardiac output and pulsatile flow, exposing the aortic wall to repetitive shear stress and cyclic tension.¹⁰ In contrast, strength or resistance training (such as American football or weightlifting) induces brief, intermittent surges in systolic pressure and afterload, resulting in localized wall stress that promotes concentric remodeling and increased arterial stiffness, in addition to aortic dilation.⁸ Despite these adaptations, there is uncertainty that athletic training accelerates pathological aortic growth or precipitates aortic events.

Counseling should be performed collaboratively with the athlete to discuss the physiological effects of exercise exposure, ultimately aiming for SDM that promotes safe physical activity while preventing future risk of aortic complications. Concerns related to collision sports and indicators of increased risk, such as family history of dissection and genetic factors, should be carefully considered during the SDM discussion.^26,27 An important facet of risk mitigation is close surveillance of the aorta to identify increases in size and, in turn, risk of an adverse event. More importantly, tactical athletes and military personnel represent a unique population in whom occupational demands may exceed traditional sporting participation.

The approach for surgical intervention in aortopathy is primarily based on population-level risk and is not athlete-specific. The European Society of Cardiology guidelines classify risk using threshold-based tiers, with an aortic diameter <40 mm considered low risk, permitting unrestricted sports participation with follow-up at least every 2 to 3 years. The intermediate-risk group comprises those with aortic measurements up to 45 mm, including those who have had successful surgery. They should avoid high-intensity, power, and contact sports, instead opting for endurance sports.²⁰ Follow-up should occur every 6 months to 2 years, depending on the severity of the dilatation. Athletes with aortic diameters >45 mm or >50 mm are advised to either participate in skilled sports or endurance activity at low intensity or abstain from all sports participation, respectively.²⁰ The more recent AHA statement incorporates a more detailed and layered approach, aligning sport participation thresholds with aortic size, etiology, and patient preference.²² Here, unrestricted competitive participation is considered appropriate when the aortic diameter is <40 mm.^22,23 Those with mild to moderate dilation (40-44 mm) may continue to compete following SDM and close imaging surveillance. A diameter of ≥45 mm confers a greater risk of aortic complications with continued participation on a case-by-case basis.²² Low-risk athletes may participate in both competitive and recreational sports, whereas higher-risk patients should engage in individualized programs, with regular surveillance to ensure long-term safety.^20,22 Post surgical intervention, athletes may return to sporting participation once there is complete sternal healing. Residual risk still persists, necessitating ongoing longitudinal surveillance with supervised cardiac rehabilitation.

Future Directions

Despite recent modifications to the AHA statement and the low incidence of aortic dissection resulting in SCD from ongoing exercise, a conservative approach toward athletes with BAV-associated aortopathy continues to be prevalent.^28,29 Furthermore, identifying pathologic aortic enlargement requires a more sophisticated assessment than absolute size-based thresholds. Advanced imaging techniques, such as computed tomography angiography and MRA, can provide insights into wall stiffness, shear stress, and 4-dimensional flow, facilitating precision around timing and necessity of intervention.³⁰ In parallel, genetic testing has become a valuable tool, guiding cascade screening and enabling individualized surveillance and risk stratification.³¹

There is a paucity of data on postoperative risk and outcomes in athletes after surgical repair of ascending aortic aneurysms. As in the case presented here, it is plausible that the risks of dissection are mitigated via repair with graft material. However, this requires further formal examination, including other potential complications and impact on athletic performance. Furthermore, strategies to facilitate earlier intervention with reduced procedural risk may help to address the unique needs of athletically oriented patients.

Conclusions

Ultimately, remodeling of the athlete's aorta exists on a continuum between physiological adaptation and pathological change, wherein genetic predisposition, training load, and cumulative hemodynamic exposure determine whether vascular alterations are physiologic or evolve toward disease expression. Our case exemplifies this issue, as we observed rapid dilation of the aortic root in an athlete with BAV and a root-phenotype aortic dilation, which was suspected to be a pathologic change. Continued surveillance revealed ongoing enlargement, ultimately necessitating surgical intervention. This emphasizes the critical importance of continued monitoring, the integration of multidisciplinary care, and a SDM approach, all of which are paramount for promoting safe athletic participation.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Visual Summary.

Case Timeline

Timeline	Events
Initial evaluation	•Evaluation at sports cardiology clinic after murmur detected •Echocardiogram: type 1 BAV, mild AR •Aortic root 4.3 cm (Z-score 3.4); ascending aorta 3.6 cm •CT angiography: 4.3 cm (1.95 cm/m2) •ECG normal variant with tall QRS voltage
	•Determined not eligible for fighter pilot role due to aortic dilation •Shared decision-making involving family and military medical review •Granted waiver for enrollment at West Point •Plan for ongoing surveillance imaging
∼4 years	•Aortic root increases to 4.7 cm (growth ∼3 mm/year) on MRA/CTA •Concern for progression and long-term risk of aortic dissection •Temporarily removed from Army
∼ Shortly afterward	•Cardiothoracic surgical consultation •Discussions on career goals, lifetime risk, and preventive intervention •Decision made for valve-sparing aortic root replacement
∼ Postoperative period	•Surgery without complications •Recovery uneventful •Resumes follow-up and pending gradual return to sporting participation and return to the Army

AR = aortic regurgitation; BAV = bicuspid aortic valve; CT = computed tomography; CTA = computed tomography angiography; ECG = electrocardiography; MRA = magnetic resonance angiography.