A 38-year-old man is brought to the emergency department due to sudden onset of shortness of breath and diaphoresis. He has no fever, chills, cough, or abdominal pain. The patient has no other medical problems and takes no medications. He had surgery for bilateral inguinal hernias at age 16. The patient does not use tobacco, alcohol, or illicit drugs. Blood pressure is 95/60 mm Hg, pulse is 116/min and regular, and respirations are 28/min. He is in marked respiratory distress. Pallor and diaphoresis are noted. His skin is velvety and has many atrophic scars. The apical impulse is hyperdynamic. Cardiac auscultation shows a soft, early-systolic decrescendo murmur at the cardiac apex. The first sound is barely audible; the second heart sound is normal. Lung examination shows bibasilar crackles. Jugular venous distension is present. The abdomen is soft, nontender, and nondistended. Neurologic examination shows no abnormalities. The electrocardiogram (ECG) shows sinus tachycardia with occasional premature ventricular complexes. Chest x-ray reveals no cardiomegaly, but bilateral alveolar infiltrates and hilar prominence are present.
Item 1 of 2
Which of the following is the most likely cause of this patient’s condition?
- A. Acute myocardial infarction [0%]
- B. Infective endocarditis [11 %]
- C. Papillary muscle rupture [34%]
- D. Pulmonary embolism [6%] .,
- E. Rupture of chordae tendineae [49%]
0 voters
Explanation:
This patient most likely has Ehlers-Danlos syndrome (EDS), a connective tissue disorder that is characterized by joint hypermobility, recurrent joint dislocations, hyperextensible skin with easy bruisability, and delayed healing with atrophic scars. Patients may have associated skeletal abnormalities, including thoracolumbar scoliosis and pes planus. Other features include hernias, cervical insufficiency, and uterine prolapse. Mitral valve prolapse with myxomatous degeneration of the valvular apparatus is a complication of EDS and can lead to rupture of chordae tendineae and acute mitral regurgitation (MR).
(Choice A) This patient’s symptoms, clinical features, and electrocardiogram (ECG) findings do not support a diagnosis of coronary artery disease.
(Choice C) Mitral valve prolapse is frequently seen in patients with Marfan syndrome, and patients may have acute or worsening MR due to rupture of the chordae tendineae. However, the characteristics of Marfan syndrome, including overgrowth of long bones, arachnodactyly, pectus excavatum or carinatum, hindfoot valgus, and lens dislocation, are not present in this patient.
(Choice D) Acute rheumatic fever (RF) is an inflammatory condition most often seen following group A streptococcal infection. Major manifestations are migratory arthritis, carditis or valvulitis, central nervous system involvement with Sydenham chorea, erythema marginatum, and subcutaneous nodules. Minor manifestations include fever, arthralgia, elevated inflammatory markers, and prolonged PR interval. Acute RF may occasionally cause rupture or elongation of the mitral valve chordae tendineae, leading to flail leaflets and severe MR. It is most common in children and adolescents; it is uncommon in adults.
(Choice E) Thyrotoxicosis can lead to high-output congestive heart failure, but it does not cause acute MR. Patients with Graves’ disease (hyperthyroidism) can also develop hyperpigmented, violaceous, and