An octogenarian presented to our hospital in shock after being “found down” at home. She was dehydrated, hypernatremic, and suffering from a urinary tract infection. Findings of a murmur and those on electrocardiography led to the performance of echocardiography, with all findings consistent with a diagnosis of hypertrophic obstructive cardiomyopathy. The patient was volume resuscitated and administered antibiotics; after stabilization, she was started on a low-dose beta-blocker. Hypertrophic cardiomyopathy is a common genetic disorder that is usually diagnosed in the second to fifth decades of life, rarely being diagnosed at an advanced age. It is also an uncommon cause or contributor to shock. We briefly review the diagnosis and management of hypertrophic cardiomyopathy in older adults, particularly in the setting of shock.
The differential diagnosis of patients presenting with shock is broad and includes sepsis, hypovolemia, anaphylaxis, neurogenic compromise, impaired cardiac output from a low ejection fraction, and tamponade, among other causes. Although hypertrophic cardiomyopathy (HCM) is common in the general population, it is an uncommon cause of or contributor to shock. It is also usually diagnosed in younger individuals. The condition is increasingly diagnosed in older patients because of an increased index of suspicion and use of imaging. However, diagnosing an octogenarian with HCM is uncommon and may be a complex endeavor. We report a case of an initial diagnosis of HCM in a patient who presented with shock and review pertinent diagnostic, prognostic, and management considerations.
An 84-year-old woman was brought to the emergency department (ED) after being found at home (where she lived independently) by her son with whom she had not had any contact for 4 days. In the ED, she was found to have a heart rate of 103 bpm, blood pressure (BP) of 83/53 mmHg, and oxygen saturation of 96% on ambient air. She had very dry mucous membranes, and a 4/6 systolic murmur was best heard at the base and in her bilateral carotid arteries. Laboratory examinations showed a sodium level of 165 mEq/L, a creatinine level of 5.4 mg/dL, an international normalized ratio (INR) of 3.2, an elevated white blood cell count, and urinalysis findings consistent with an infection (culture grew pan-sensitive
The patient received 2 L of lactated Ringer’s solution, and antibiotic therapy was initiated. Her BP increased to 107/64 mmHg; her mental status also improved (able to answer yes/no questions), and she was able to follow basic instructions. Because of the murmur observed and the findings on electrocardiography, she underwent echocardiography (
HCM is a common genetic condition affecting at least one in 500 persons.
When an older adult presents with symptoms that may be attributable to HCM, the differential diagnosis is extensive. Dyspnea on exertion, chest pain, and syncope can have many etiologies or be multifactorial owing to age-related problems, including volume depletion, deconditioning, or comorbidities such as lung conditions (e.g., chronic obstructive pulmonary disease), coronary artery disease, valve disease, arrhythmias, or heart failure. Physical examination may be challenging, as the murmur due to HCM may be confused with or masked by that due to aortic sclerosis or stenosis. These other etiologies may be pursued before cardiac imaging (usually echocardiography). Once imaging raises the possibility of a differential diagnosis of HCM, the diagnosis may remain uncertain as other conditions can have similar morphologic appearances.
Other entities that may be considered include age-related structural changes (discussed below), hypertensive cardiomyopathy, and amyloid or other infiltrative cardiomyopathies (e.g., hemochromatosis). A detailed approach to the diagnosis of these is beyond the scope of this report; however, more global hypertrophy and a history of un/under-treated hypertension and other end-organ damage suggest hypertensive cardiomyopathy and hypertrophy on echocardiography but a low voltage on ECG, whereas other specific findings on imaging or comorbid conditions may point toward amyloid or other infiltrative cardiomyopathy.
A sigmoid-shaped septum (also called discrete upper septal thickening or upper septal knuckle) is found in older adults (particularly those with hypertension) and may result from normal aging. The prevalence is up to 10%, and an increased angle of the aorta to the long axis of the left ventricle (LV) is also common. In the Framingham Heart Study, 1.5% of 3,562 participants had a sigmoid-shaped septum (17.8% of those >85 years). The positive correlates were increasing age, hypertension, decreased LV fraction, and mitral annular calcification, whereas the inverse correlates were the LV diastolic dimension. After adjusting for cardiovascular risk factors, over 15 years, the condition was not found to be associated with cardiovascular events or mortality.
Differentiating normal aging from genetic HCM is challenging. Patients with late-onset HCM tend to have less impressive septal hypertrophy and an ovoid or ellipsoid (not crescentic) LV.
Of the 3% of patients in the Framingham Heart Study (n=1,862; mean age, 60 years) with unexplained LV hypertrophy, only 18% had sarcomeric contractile protein mutations.
The clinical course of patients diagnosed with HCM at an advanced age is generally better than that of those diagnosed at a younger age.
Standard treatment may be considered for older adults diagnosed with HCM, including medical therapy (e.g., beta-blockers, calcium channel blockers, and disopyramide). Medically refractory patients, even those of advanced age, may be evaluated for alcohol septal ablation or surgical septal myectomy.
In conclusion, after correction of the free water deficit and the administration of antibiotics, the patient’s laboratory abnormalities resolved and her mental status returned to baseline. Her shock was likely multifactorial, owing to a combination of dehydration and urinary tract infection, resulting in volume depletion and increased myocardial demand, both of which worsened the LVOT obstruction, further impairing her cardiac output. After stabilization, the patient was started on a low-dose beta-blocker in addition to antibiotics. She and her son declined further evaluation of her HCM (including cardiac MRI, genetic evaluation, and outpatient rhythm monitoring for ventricular arrhythmias/risk stratification for sudden death), and she was discharged to a rehabilitation facility with plans for outpatient follow-up.
The researcher claims no conflicts of interest.
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(A) Electrocardiogram obtained at admission showing normal sinus rhythm with premature atrial complexes, as well as voltages and repolarization abnormalities consistent with left ventricular hypertrophy. The remaining panels show findings consistent with hypertrophic obstructive cardiomyopathy, including (B) predominant septal hypertrophy, (C) systolic anterior motion of the mitral valve, (D) a very high left ventricular outflow tract gradient, and (E) moderate mitral regurgitation. Other findings included a normal-sized left ventricle with hyperdynamic function (ejection fraction >70%).