Abstract 10887: Love-Making Reveals a Broken Heart: A 46-Year-Old Man with Recurrent Hemoptysis During Sexual Intercourse
A 46-year-old man with type 2 diabetes presented with recurrent hemoptysis during sexual intercourse, chest pain, and dyspnea. Physical exam and CXR were normal. ECG showed normal voltage and nonspecific T wave abnormalities. Stress echo revealed moderate concentric LVH and diastolic dysfunction at rest, chest pain at 6 min. (total exercise time=8 min.), and inferolateral and inferoseptal ischemia. However, cardiac catheterization demonstrated normal coronary arteries and normal filling pressures. Detailed pulmonary evaluation (including PFTs, chest CT, and bronchoscopy with transbronchial biopsy) was negative for a pulmonary cause of hemoptysis. Adenosine perfusion cardiac MRI (CMR) demonstrated no focal fibrosis or infiltration (i.e., no areas of late gadolinium enhancement) but did show a circumferential subendocardial defect consistent with microvascular ischemia. However, the patient’s exertional hemoptysis remained unexplained. The lack of a unifying diagnosis required re-analysis of each cardiac test. The baseline echo was re-analyzed, and longitudinal systolic function (s’ velocity and global longitudinal strain) was found to be severely reduced, despite a normal LVEF, consistent with a cardiomyopathic process. Re-analysis of the CMR with a novel T1 mapping protocol demonstrated extremely high extracellular volume (Ve) fraction (43%; normal<25%), consistent with a severe, diffuse infiltrative cardiomyopathy. Finally, repeat RHC was performed with supine bike exercise. After 1 min. of exercise, mean PA pressure and PCWP both rose from high-normal (24 mmHg and 15 mmHg, respectively, at rest) to severely elevated (54 mmHg and 37 mmHg, respectively, at peak exercise), consistent with severe exercise-induced pulmonary venous hypertension due to marked LV diastolic dysfunction--the likely cause of intercourse-induced hemoptysis. Cardiac biopsy demonstrated amyloidosis and further fibril typing, serum IFE, and bone marrow biopsy confirmed the diagnosis primary (AL) amyloidosis. This case illustrates the power of re-examining primary cardiac data using advanced techniques (tissue Doppler, speckle tracking, CMR T1 mapping, and exercise hemodynamics) in order to make the diagnosis of a rare presentation of a rare disease.
- © 2012 by American Heart Association, Inc.