Palpitations and Dyspnea in a 50-Year-Old Female

INTRODUCTION The preparticipation examination represents the foundation of an athlete's medical clearance for activity. While each year thousands of these examinations are performed in the United States, this undertaking has been frequently called into question, as the yield is considerably low. In addition, Maron et al. along with others have noted a lack of a national standard and identified key components at the examination that fulfill the cardiovascular screen (1). This case of a 50-yr-old former runner highlights the potential importance of a well-done preparticipation examination with attention to detail during the physical examination. CASE PRESENTATION A 50-yr-old woman presented to a family medicine clinic with the complaint of a "racing heart." The patient was in her usual state of health 5 d before presentation, when she developed palpitations. They were only transient, however, and once they disappeared, she felt to be her usual self again. The following day, the patient developed these palpitations again, but this time they were accompanied by dyspnea with climbing stairs, which she never had experienced before. Then, 3 d before presentation, she had worsening dyspnea on exertion and fatigue, diaphoresis, and sharp chest pain accompanying the palpitations. On the day of presentation, she decided to seek medical care because the palpitations, which had been coming and going, were now persisting, and her associated symptoms of fatigue and diaphoresis were worsening. Review of systems was negative for orthopnea, paroxysmal nocturnal dyspnea, cough or recent upper respiratory symptoms, fevers or chills, headaches, dizziness, syncope, and recent change in weight. Past medical history was notable for chronic hypertension, which was first diagnosed in her early 20s when she lived in Bolivia. Although she was taking a blood pressure medication for several years in Bolivia, she had not been taking any blood pressure medications for the past 12 years since her move to the United States, mostly due to financial difficulty. Other than green tea extract for her chronic constipation, she denies any other medications, supplements, or herbals. Past surgical history was significant for a cesarean section 22 years ago. Obstetric history included no miscarriages and one pregnancy, although she reports trying to get pregnant for years unsuccessfully. Family history was positive for diabetes but otherwise unremarkable. The patient denied ever using tobacco, did not currently use alcohol, and denied illicit drug use. She was born and raised in Bolivia where, as a teenager, she used to run competitively. She denies any recent travel or sick contacts. She lives with her sister and daughter and cleans houses for a living. Examination on presentation demonstrated a blood pressure of 132/80, a pulse initially of about 180, and other vital signs within normal limits. Significant findings on physical exam included an irregularly, irregular heart rate with a 3/6 holosystolic and early diastolic murmur heard throughout her chest and including her back. Jugular venous distention was present. Extremities were well perfused without clubbing, cyanosis, or edema. Her peripheral pulses were all 2+. Her laboratory data included a complete blood count, which was normal, and a basic metabolic profile, which was normal to include a magnesium of 2.0, thyroid stimulating hormone level of 1.99, and a negative set of cardiac enzymes. Her serum ethanol level also was negative. Electrocardiogram revealed an irregular rate, no P waves, tachycardia at about 150, left ventricular hypertrophy with an axis around -60, and ST segment elevations in leads III, AVF, V2, V3, and V4 with ST segment depression in leads V5 and V6. Chest x-ray was read as showing marked enlargement of the cardiomediastinal silhouette and mild pulmonary vascular congestion. The heart border extended laterally to the left costophrenic junction (Fig. 1).Figure 1: Patient's chest x-ray showing classic signs of coarctation of the aorta (COA): rib notching (arrow) and an enlarged heart.After being transferred to a nearby hospital, her pulse was lowered using diltiazem and a repeat electrocardiogram revealed an irregular pulse in the 80s with ST segment elevations and depressions resolved. She was admitted to the hospital, and serial cardiac enzyme tests were performed to rule out a myocardial infarction as the cause of her atrial fibrillation. The patient never had an increase in her troponins, and she continued to be rate-controlled on diltiazem. On hospital day 3, she had an echocardiogram done, which showed severe left ventricular dysfunction and an ejection fraction of 15%. She also had marked left ventricular dilation, decreased contractility without any definite wall motion abnormality, an enlarged right ventricle, a trileaflet but sclerotic aortic valve with one leaflet appearing fixed, mild mitral regurgitation, moderate tricuspid regurgitation, and significant pulmonary hypertension. Because of her enlarged heart and severely reduced ejection fraction, the decision was made to perform a pharmacologic stress thallium for assessment of possible cardiac ischemia. The test confirmed an ejection fraction of 15%; however, it also showed an ischemia involving the anteroseptal region and apex of the heart consistent with disease in the left anterior descending artery. Although a myocardial infarction was ruled out at the beginning of her hospitalization with serial cardiac enzymes, severe blockage had not been ruled out, and the decision to perform a cardiac catheterization was made based upon results of the pharmacologic stress thallium test. Cardiac catheterization was attempted using a 6 French catheter in the right femoral artery. The procedure was halted, however, after discovery of an interrupted descending aortic arch. Computed tomography (CT) angiogram of her chest was then done, which showed a discontinuation of the descending thoracic aorta at the level of the ductus just beyond the left subclavian artery. There was extensive collateralization (Fig. 2), which reconstituted the descending thoracic aorta just millimeters after the interruption (Fig. 3). The great vessels were enlarged, there was rib notching noted, significant left ventricular hypertrophy and dilation, and the lungs demonstrated a patchy ground glass appearance. The cause of this patient's symptoms and clinical findings had been discovered: the patient had an undiscovered coarctation of the aorta just beyond the takeoff of the left subclavian artery.Figure 2: Patient's proximal aortic arch, interrupted just after the takeoff of the left subclavian artery, with tortuous, collateral vessels.Figure 3: Tortuous, collateral vessels reconstituting the distal aorta after the coarctation.DISCUSSION Coarctation of the aorta (COA) represents about 6%-8% of all congenital heart defects and is more commonly found in males than in females (2). Although sometimes infants born with COA will have cyanosis just after birth when the PDA closes, it occurs more often that patients will remain asymptomatic with only subtle signs in childhood or adolescence, such as pain or weakness in the legs after exercise (3). Depending on the age, patients with COA will have characteristic presentations that, if recognized, will lead to earlier detection of this abnormality. In infancy, two common signs are blood pressure discrepancy between the upper and lower extremities and weak or delayed femoral pulses. Blood pressure, when taken in upper and lower extremities, is normally 10-20 mm Hg higher in the legs when compared to the arms. In COA, blood pressure in the legs is lower than in the arms and often may be difficult to obtain (3). Weak or delayed femoral pulses are found when compared with the upper extremities, and the femoral, popliteal, posterior tibial, and dorsalis pedis pulses are found to be weak or absent in up to 40% of patients (3). A murmur also may be present on physical exam if there is an associated cardiac defect such as a patent ductus arteriosus, aortic stenosis, ventriculoseptal defect, or a bicuspid aortic valve. After about 5 yr of age, children will still have hypertension in upper extremities compared with lower extremities, however, murmurs from collateral vessels, such as the internal thoracic, intercostals, subclavian, and subscapular arteries, may be present. These murmurs typically present as a continuous murmur that may be heard throughout the thorax including the back, and they are often discovered incidentally as part of an emergency room visit, by a school nurse, or as part of an insurance physical (4). It is usually after detection of a murmur coupled with high blood pressure that these patients are then referred to a pediatric cardiologist where they are then diagnosed with COA (4,5). Electrocardiographic tests in patients with COA may be normal or may show evidence of left ventricular hypertrophy. Imaging using chest radiograph will show an enlarged heart due to significant hypertrophy. A pathognomonic finding seen with COA is notching or sclerosis of the undersurface of the ribs or scapulae. This is caused by marked enlargement of the intercostal collaterals with erosion by these dilated vessels and is rarely seen before age 6 (6-8). Occasionally a "Figure 3" sign, which is caused by aortic dilation proximal to the coarctation, indentation at the coarctation, and then poststenotic segment dilation, may be present (7). For those with untreated or undiscovered COA, only about 25% of patients will survive until mid-adulthood (2,3). These patients will have hypertension, but blood pressure discrepancy is not observed because of regional autoregulation of blood flow. They will have all of the classic radiologic findings, such as notching of the ribs on chest x-ray and an enlarged heart from hypertrophy. On physical exam, pulses are likely to be equal throughout, but the femoral pulses may be delayed when compared with the right radial pulse. These patients are likely to present to the hospital after the third decade with a common but serious complication from their chronic hypertension, such as premature coronary artery disease, heart failure, hypertensive encephalopathy, or intracranial hemorrhage (2,3,9,10). Currently, screening recommendations for COA are lacking in preparticipation physicals. Further, recommendations usually include palpation of pulses, which have not always been shown to be a sensitive screening tool. According to one study, COA would have been missed in 82% of cases if absent lower extremity pulses were required as a diagnostic feature (5). The third edition of Preparticipation Physical Evaluation suggests simultaneous palpation of the radial and femoral pulses as a screen for coarctation of the aorta (11); however the guide fails to mention that the right radial pulse should be used, since some coarctations appear before the takeoff of the right subclavian artery. The American Heart Association's guide to the cardiovascular evaluation for a preparticipation examination mentions palpation of "femoral pulses to exclude aortic coarctation"; however, there is no mention of comparison with a radial artery pulse (1). As previously mentioned, palpation of femoral pulses alone is inadequate and would miss the majority of cases of coarctation. As published in Pediatrics (5), a systolic pressure gradient between the arms and legs of greater than 10 mm Hg and a murmur were present in 100% of patients with COA. The recommendation in that publication was to screen "all children for coarctation of the aorta by routinely measuring upper and lower extremity blood pressures during at least one physical examination after the newborn period." It is actually the discrepancy in blood pressure between upper and lower extremities rather than the absolute systolic blood pressure value that is more striking in COA (2). Even with this evidence, there is still no guideline for upper extremity versus lower extremity blood pressure screening in either well-child exams or preparticipation physicals, despite the fact that this is the most pathognomonic feature of COA. Further, there is usually a delay in diagnosis of COA due to the subtle signs and lack of an effective screening during childhood physical exams. Undetected early in life, those who are diagnosed and treated for COA after 5 yr of age are still found to have secondary effects from the coarctation, such as demonstrated in this case report. CONCLUSION Coarctation of the aorta, while an uncommon disorder, is routinely screened for during well-baby checks, physical exams, and preparticipation examinations. There are many instances, both in this hospitalization and earlier in this patient's life, when the coarctation could have been discovered and properly treated. Current guidelines in the sports medicine community, in the authors' opinion, are inadequate to screen and detect the athlete with possible coarctation of the aorta. It is the authors' recommendation that upper and lower extremity blood pressure measurements be performed not only in the evaluation of hypertension, but at least one time during a competitive athlete's career.