The MKSAP Challenge
A 34-year-old woman is evaluated in the emergency department because of worsening shortness of breath over the past two weeks progressing to dyspnea at rest and coughing of pink sputum. She is seven weeks pregnant. She had one uncomplicated pregnancy 14 years ago. She has no history of hypertension, hyperlipidemia or chronic illness. She smokes one pack of cigarettes over four days and rarely consumes alcoholic beverages.
On physical examination, her heart rate is 110/min and regular, and blood pressure is 120/70 mm Hg. Arterial oxygen saturation by pulse oximetry is 85% on room air, and nasal cannular oxygen is started. Jugular venous pressure is elevated to the angle of the mandible and there are diffuse crackles bilaterally. A prominent summation gallop is noted, and a grade 3/4 holosystolic murmur at the apex radiates to the axilla. An accentuation of the pulmonary component of the S2 is heard at the lower left sternal border. Her liver is slightly enlarged, and a trace of ankle edema is noted. A chest radiograph shows pulmonary edema and cardiomegaly.
Which one of the following is the most likely diagnosis?
A. Rheumatic mitral valve regurgitation
B. Primary pulmonary hypertension
C. Pulmonary thromboembolism
D. Congenital aortic stenosis
E. Peripartum cardiomyopathy
Recognize that pulmonary pressure in a pregnant woman is related to valvular heart disease.
The physical examination of this patient is compatible with pulmonary hypertension that may be of long-standing duration. The etiology of the pulmonary hypertension could be valvular because a murmur of mitral regurgitation is also noted. There are multiple etiologies possible for mitral valve disease. Mitral regurgitation can be the result of a congenital abnormality such as Marfan syndrome leading to myxomatous valve degeneration. In a young, otherwise healthy individual, a spontaneous chordal rupture should be ruled out by echocardiography.
Mitral valve prolapse as a cause of significant mitral regurgitation is unusual in a female patient of this age group, but could occur in an older male. Although mostly eradicated in the United States, rheumatic fever with subsequent mitral valve disease continues to be a health problem in developing countries. Rheumatic heart disease should be ruled out by echocardiography. Any valvular lesion may have progressed over years despite a normal first pregnancy.
An angiotensin-converting enzyme (ACE) inhibitor is indicated when the ejection fraction is below 35%. However, this patient is pregnant, and ACE inhibitors, which are associated with teratogenicity, should be avoided. Mitral regurgitation in the presence of pulmonary hypertension is a contraindication to completing the pregnancy without further medication or diagnostic procedures. The timing of the patient's symptoms is not compatible with peripartum cardiomyopathy.
1. Geva T, Mauer MB, Striker L, Kirshon B, Pivarnik JM. Effects of physiologic load of pregnancy on left ventricular contractility and remodeling. American Heart Journal. 1997;133:53-59.
2. Pearson GD, Veille J, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, et al. Peripartum cardiomyopathy: National Heart, Lung and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review. Journal of the American Medical Association. 2000;283:1183-8.
3. Elkayam U, Tummala PP, Rao K, Akhter MW, Karaalp IS, Wani OR, et al. Maternal and fetal outcomes of subsequent pregnancies in women with peripartum cardiomyopathy. New England Journal of Medicine. 2001;344:1567-71.
The above feature contains questions and answers excerpted from MKSAP 12 Update, an enhancement to the College's popular self-assessment program. For more information about MKSAP 12 Update, contact ACP Customer Service at 800-523-1546, ext. 2600, or 215-351-2600.
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