Author + information
- Charles A. Cevallos, DO, MPH∗ (, )
- Hajir Zohourian, DO,
- Samuel Sacks, DO and
- Arnoux Blanchard, MD
- ↵∗Address for correspondence:
Dr. Charles A. Cevallos, Broward Health Medical Center, 1600 South Andrews Avenue, Fort Lauderdale, Florida 33316.
A 15-year-old female who was 1-month post-partum presented with shortness of breath and hemoptysis. Echocardiography revealed findings suspicious for spontaneous papillary muscle rupture. We illustrate the evaluation, differential diagnosis, and management of spontaneous papillary muscle rupture. (Level of Difficulty: Beginner.)
A 15-year-old female who was 4 weeks post-spontaneous vaginal delivery presented with complaints of shortness of breath, hemoptysis, and decreased exercise tolerance for 3 days. On presentation, she was hypoxic at 86% on room air, tachycardic with a heart rate of 153 beats/min, tachypneic with a respiratory rate of 41 breaths/min, and a temperature of 100.9°F. Physical examination revealed a systolic murmur 4/6 best heard at apex, bibasilar crackles, and jugular venous distention.
Chest x-ray revealed right pleural effusion with bilateral infiltrates consistent with congestive heart failure. Two-dimensional transthoracic echocardiographic (TTE) images showed findings suspicious for endocarditis, prolapse, and papillary muscle rupture (Figure 1A). Color Doppler imaging revealed severe mitral regurgitation with a coanda effect (Figure 1B).
She was taken for surgical mitral valve replacement. The intraoperative 3D echocardiogram showed prolapsing A1-A2 and P1-P2 scallops with possible papillary muscle rupture (Figure 1C). Inspection of papillary muscle during surgery revealed that one-half of the anteromedial papillary muscle was infarcted with a 50% tear in the papillary muscle itself (Figures 1D and 1E). Pathology confirmed infarcted myocardial tissue (Figure 1F).
Papillary muscle rupture is a rare life-threatening event and usually presents as a complication in 0.25% of patients who experience acute myocardial infarction (1). However, the pathophysiology of nonischemic spontaneous papillary muscle rupture is unique in that it lacks coronary artery disease predisposition. Therefore, understanding the mechanism of rupture requires investigation into other underlying disorders in which the papillary muscles and its surrounding apparatuses are predisposed to failure. Such disorders include connective tissue diseases, myocarditis, infectious endocarditis, takotsubo cardiomyopathy, and mitral annular calcification (2). After assessment of gross pathology and detailed evaluation of the patient’s history and physical examination, no evidence of infectious, rheumatological, or congenital causes of papillary muscle failure were identified.
A review of the literature identified 11 cases of spontaneous mitral papillary rupture in adult patients (2). To the best of our knowledge, this may be the first reported case of spontaneous mitral papillary muscle rupture in a pediatric patient that occurred in a post-partum period.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Case Reports author instructions page.
- Received October 24, 2019.
- Revision received December 13, 2019.
- Accepted February 10, 2020.
- 2020 The Authors