Author + information
- Received July 27, 2013
- Revision received August 30, 2013
- Accepted September 10, 2013
- Published online January 21, 2014.
- Travis J. Vowels, BBA∗,
- Gonzalo V. Gonzalez-Stawinski, MD†,
- Jong M. Ko, BA∗,
- Gregory D. Trachiotis, MD‡,
- Brad J. Roberts, BS, RCS∗,
- Charles S. Roberts, MD§ and
- William C. Roberts, MD∗,‖,¶∗ ()
- ∗Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas
- †Department of Cardiothoracic Surgery, Baylor University Medical Center, Dallas, Texas
- ‡Division of Cardiothoracic Surgery, The George Washington University Medical Center and Veterans Affairs Medical Center, Washington, DC
- §Palmetto Cardiovascular and Thoracic Associates, Trident Medical Center, Charleston, South Carolina
- ‖Department of Internal Medicine (Division of Cardiology), Baylor University Medical Center, Dallas, Texas
- ¶Department of Pathology, Baylor University Medical Center, Dallas, Texas
- ↵∗Reprint requests and correspondence:
Dr. William C. Roberts, Baylor Heart and Vascular Institute, Baylor University Medical Center at Dallas, 3500 Gaston Avenue, Dallas, Texas 75246.
Objectives This report calls attention to an unappreciated cause of both acute and chronic aortic regurgitation (AR).
Background Although stenosis develops in most patients with a congenitally bicuspid aortic valve (BAV), in others with this anomaly, pure AR (no element of stenosis) develops, some in the absence of infection or other clear etiology.
Methods We describe 5 men who underwent aortic valve replacement for pure AR associated with a BAV containing an anomalous cord attaching the raphe of the conjoined cusp near its free margin to the wall of the ascending aorta cephalad to the sinotubular junction.
Results Three of these 5 patients had a history of progressive dyspnea, and the anomalous cord, which was intact at operation, appeared to cause chronic AR by preventing proper coaptation of the 2 aortic valve cusps. The other 2 patients heard a “pop” during physical exertion and immediately became dyspneic, and at operation, the anomalous cord was found to have ruptured. Prolapse of the conjoined aortic valve cusp toward the left ventricular cavity resulted in severe acute AR.
Conclusions This variant of the purely regurgitant BAV may cause either chronic AR (when the anomalous cord does not rupture) or acute severe AR (when the cord ruptures).
The congenitally bicuspid aortic valve (BAV) occurs in an estimated 1% of the population, such that in the United States, an estimated 3 million individuals have this malformation (1). Although in some individuals the BAV functions normally for an entire lifetime, stenosis develops in most, superimposed infection (infective endocarditis) develops in some, and pure aortic regurgitation (AR) unassociated with infective endocarditis or its consequences develops in some (2). A subgroup of those with pure AR unassociated with infection have an anomalous cord extending from the raphe of 1 of the 2 cusps to the wall of the aorta, and the cord serves to keep the raphe cusp from prolapsing toward the left ventricle (Table 1). During a 50-year period, we examined 5 operatively excised purely regurgitant BAVs with an anomalous cord from the raphe to the wall of the aorta. A brief description of these 5 cases is the purpose of this report.
During a nearly 50-year period, we examined 5 operatively excised aortic valves that were congenitally bicuspid and had a cord attached from the margin of the raphe cusp to the wall of the aorta. The clinical records in all 5 patients were subsequently examined, and all 5 valves were photographed.
Pertinent findings in the 5 patients are summarized in Table 2; all patients were men. Three (Patients #1, #2, and #5) had chronic AR and 2 had acute AR. The latter 2 were asymptomatic until 1 or 2 days before aortic valve replacement: both heard a popping noise in his chest, 1 patient while working in his yard and 1 while working on his car beneath the hood. Both became suddenly dyspneic, and the dyspnea progressed rather rapidly, prompting pulmonary edema and hospitalization. A median sternotomy was emergently performed in each for severe AR. The cord extending from the raphe to the wall of the aorta had ruptured, causing the raphe cusp to prolapse toward the left ventricular cavity (Fig. 1). Each of the other 3 patients had chronic AR, and in none of them had the anomalous cord ruptured. Photographs of the operatively excised BAVs are shown in Figures 2 to 5⇓⇓⇓⇓.
This report describes 5 men with a BAV and pure AR with an anomalous cord extending from the raphe of the conjoined cusp to the wall of the ascending aorta cephalad to the sinotubular junction. In the 3 patients who presented with chronic AR, the cord appeared to prevent complete coaptation of the 2 aortic valve cusps by pinning the raphe of the conjoined cusp to the aortic wall. In the 2 patients who presented with acute AR, the cord had ruptured, resulting in the conjoined cusp prolapsing toward the left ventricular cavity and the acute onset of symptoms.
This variant of the BAV has been previously described (Table 1), and its association with AR has been well documented (1,3–13). Of the 33 previously published cases, 25 (76%) had evidence of chronic AR as a result of having an intact anomalous cord, and 8 (24%) patients presented with acute AR due to rupture of the anomalous cord. Waller et al. (14) in 1973 described 2 other patients, not included in Table 2, with similar BAVs: 1 was an 8-year-old boy with coarctation of the aorta, and 1 was an 81-year-old man with no other associated congenital anomalies. There was no mention of function of the BAV in these 2 patients. In 1994, Walley et al. (11) described 8 patients with this anomalous cord attached to a stenotic BAV in addition to the 9 patients with a purely regurgitant BAV included in Table 1. The presence of an anomalous cord extending to the wall of the aorta from the raphe of a BAV thus may be associated with stenosis but far more commonly with pure AR.
In none of the 5 men included in the present study was the diagnosis of this variant of the purely regurgitant BAV made pre-operatively. Most symptomatic patients with this congenital anomaly present with chronic AR and slowly progressing symptoms. Rupture of an anomalous cord should be considered as a possible etiology of pure acute AR when infection, aortic dissection, and trauma have been ruled out.
The study was funded by the Baylor Health Care System Foundation, Dallas, Texas. Mr. Vowels is a second-year medical student at the University of Texas Medical School at Houston. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Abbreviations and Acronyms
- aortic regurgitation
- bicuspid aortic valve
- Received July 27, 2013.
- Revision received August 30, 2013.
- Accepted September 10, 2013.
- American College of Cardiology Foundation
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