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‘Loud Continuous Murmur’ After Aortic Surgery: A Clue to an Aortic Root to Right Ventricular Outflow Tract Fistula
CLINICAL COMMUNICATION TO THE EDITOR
‘Loud Continuous Murmur’ After Aortic Surgery: A Clue to an Aortic Root to Right Ventricular Outflow Tract Fistula To the Editor: A 23-year-old man with a history significant for systemic lupus erythematosus and end-stage renal disease was admitted with chest pain and worsening shortness of breath. He had a past history of aortic valve endocarditis needing tissue aortic valve replacement. A few weeks later he was readmitted with prosthetic aortic valve endocarditis from coagulase negative staphylococcal bacteremia, further complicated by an aortic root abscess. He underwent redo aortic valve replacement with a mechanical aortic valve and repair of the aortic root abscess. He was febrile, with a blood pressure of 165/83 mm Hg and a heart rate of 75 beats per minute. Pertinent physical examination findings included a collapsing pulse and a continuous machinery murmur. Laboratory evaluation was notable for anemia with hemoglobin of 8.5 g/dL, an elevated white blood cell count, and creatinine at 4.9 mg/dL. International normalized ratio was 1.7. Chest X-ray was suggestive of a moderate-size right pleural effusion. In the presence of a continuous murmur and previous history of prosthetic valve endocarditis, a transthoracic echocardiogram was performed. The findings were concerning for prosthetic valve endocarditis with an aortic root abscess, and a fistulous connection between the aortic root and the right ventricular outflow tract. A real-time 3-dimensional transesophageal echocardiogram (RT-3DTEE) confirmed an aortic root to right ventricular outflow tract fistula (Figure A). There was also a dehiscence of the prosthetic aortic valve well visualized on RT-3DTEE (Figure B) with significant aortic regurgitation (Figure C). The patient underwent redo median sternotomy, removal of the mechanical aortic valve, replacement with an aortic valve/homograft (23 mm; CryoLife, Kennesaw, Ga), and repair of aortic
Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and a role in writing the manuscript. Requests for reprints should be addressed to Rajesh Janardhanan, MD, Banner - University Medical Center, Sarver Heart Center, 1501 N. Campbell Avenue, Tucson, AZ 85724. E-mail address: [email protected] 0002-9343/$ -see front matter Ó 2016 Elsevier Inc. All rights reserved.
root to right ventricular outflow tract fistula. His postoperative course was unremarkable. Continuous murmurs are rare and usually due to a complex cardiovascular pathology. They are heard owing to continuous blood flow through a high-pressure circuit to an area of low resistance during systole and diastole. These murmurs may be physiologic in the absence of structural heart disease; or pathologic owing to aortic-pulmonary or arterio-venous communications. The location and characteristics of the murmur often provides a clue to the underlying pathology.1 Trans-esophageal echocardiography has a higher sensitivity and specificity than transthoracic echocardiogram in the diagnosis of these fistulae associated with prosthetic valve endocarditis.2 Real-time 3DTEE is able to delineate the course of shunts and help plan for optimal surgical therapy.3 Aortic root to right ventricular outflow tract fistula are rare and may be congenital or acquired. They can occur as a complication of infective endocarditis, rupture of a sinus of valsalva aneurysm, aortic dissection, penetrating cardiac trauma, and rarely as a complication of aortic valve replacement.4 The incidence of fistula development in patients with prosthetic aortic valve endocarditis is approximately 0.05%.5 Aortic fistula development in endocarditis is associated with high morbidity and mortality. Approximately 60% of patients develop heart failure. The severity of heart failure is proportional to the severity of the shunt created by the fistula.3 Surgical intervention offers definitive management. A high index of clinical suspicion should be maintained in the context of recent history of endocarditis, valve replacement, and the development of a new, continuous murmur, suggesting the presence of high output shunt due to fistula formation. Safal Shetty, MDa Naktal Hamoud, MDb Keri O’Farrell, RDCS, BAb Scott Lick, MDc Rajesh Janardhanan, MDa a
Department of Medicine University of Arizona Tucson b Department of Cardiology Sarver Heart Center University of Arizona Tucson c Department of Cardiothoracic Surgery University of Arizona Tucson
http://dx.doi.org/10.1016/j.amjmed.2016.06.027
2
The American Journal of Medicine, Vol -, No -,
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2016
Figure (A) Real-time 3-dimensional transesophageal echocardiography demonstrating the aortic root to right ventricular outflow tract (RVOT) fistula. (B) Red arrow points to a dehiscence of the prosthetic aortic valve well visualized on real-time 3-dimensional transesophageal echocardiography. (C) Significant aortic regurgitation (red arrow).
References 1. Ginghina C, Nastase OA, Ghiorghiu I, Egher L. Continuous murmur— the auscultatory expression of a variety of pathological conditions. J Med Life. 2012;5(1):39-46. 2. Subramaniam K, Wei L. Left ventricular outflow tract to right atrial fistula after aortic valve replacement. J Cardiothorac Vasc Anesth. 2009;23(3):360-363. 3. Yared K, Solis J, Passeri J, et al. Three-dimensional echocardiographic assessment of acquired left ventricular to right atrial
shunt (Gerbode defect). J Am Soc Echocardiogr. 2009;22(4):435. e1-435.e3. 4. Tiwari KK, Salvati AC, di Summa M, et al. Aorta-to-right ventricular outflow tract fistula with coronary cusp prolapse. Asian Cardiovasc Thorac Ann. 2013;21(2):193-195. 5. Anguera I, Miro JM, Vilacosta I, et al. Aorto-cavitary fistulous tract formation in infective endocarditis: clinical and echocardiographic features of 76 cases and risk factors for mortality. Eur Heart J. 2005;26(3): 288-297.
‘Loud Continuous Murmur’ After Aortic Surgery: A Clue to an Aortic Root to Right Ventricular Outflow Tract Fistula To the Editor: A 23-year-old man with a history significant for systemic lupus erythematosus and end-stage renal disease was admitted with chest pain and worsening shortness of breath. He had a past history of aortic valve endocarditis needing tissue aortic valve replacement. A few weeks later he was readmitted with prosthetic aortic valve endocarditis from coagulase negative staphylococcal bacteremia, further complicated by an aortic root abscess. He underwent redo aortic valve replacement with a mechanical aortic valve and repair of the aortic root abscess. He was febrile, with a blood pressure of 165/83 mm Hg and a heart rate of 75 beats per minute. Pertinent physical examination findings included a collapsing pulse and a continuous machinery murmur. Laboratory evaluation was notable for anemia with hemoglobin of 8.5 g/dL, an elevated white blood cell count, and creatinine at 4.9 mg/dL. International normalized ratio was 1.7. Chest X-ray was suggestive of a moderate-size right pleural effusion. In the presence of a continuous murmur and previous history of prosthetic valve endocarditis, a transthoracic echocardiogram was performed. The findings were concerning for prosthetic valve endocarditis with an aortic root abscess, and a fistulous connection between the aortic root and the right ventricular outflow tract. A real-time 3-dimensional transesophageal echocardiogram (RT-3DTEE) confirmed an aortic root to right ventricular outflow tract fistula (Figure A). There was also a dehiscence of the prosthetic aortic valve well visualized on RT-3DTEE (Figure B) with significant aortic regurgitation (Figure C). The patient underwent redo median sternotomy, removal of the mechanical aortic valve, replacement with an aortic valve/homograft (23 mm; CryoLife, Kennesaw, Ga), and repair of aortic
Funding: None. Conflict of Interest: None. Authorship: All authors had access to the data and a role in writing the manuscript. Requests for reprints should be addressed to Rajesh Janardhanan, MD, Banner - University Medical Center, Sarver Heart Center, 1501 N. Campbell Avenue, Tucson, AZ 85724. E-mail address: [email protected] 0002-9343/$ -see front matter Ó 2016 Elsevier Inc. All rights reserved.
root to right ventricular outflow tract fistula. His postoperative course was unremarkable. Continuous murmurs are rare and usually due to a complex cardiovascular pathology. They are heard owing to continuous blood flow through a high-pressure circuit to an area of low resistance during systole and diastole. These murmurs may be physiologic in the absence of structural heart disease; or pathologic owing to aortic-pulmonary or arterio-venous communications. The location and characteristics of the murmur often provides a clue to the underlying pathology.1 Trans-esophageal echocardiography has a higher sensitivity and specificity than transthoracic echocardiogram in the diagnosis of these fistulae associated with prosthetic valve endocarditis.2 Real-time 3DTEE is able to delineate the course of shunts and help plan for optimal surgical therapy.3 Aortic root to right ventricular outflow tract fistula are rare and may be congenital or acquired. They can occur as a complication of infective endocarditis, rupture of a sinus of valsalva aneurysm, aortic dissection, penetrating cardiac trauma, and rarely as a complication of aortic valve replacement.4 The incidence of fistula development in patients with prosthetic aortic valve endocarditis is approximately 0.05%.5 Aortic fistula development in endocarditis is associated with high morbidity and mortality. Approximately 60% of patients develop heart failure. The severity of heart failure is proportional to the severity of the shunt created by the fistula.3 Surgical intervention offers definitive management. A high index of clinical suspicion should be maintained in the context of recent history of endocarditis, valve replacement, and the development of a new, continuous murmur, suggesting the presence of high output shunt due to fistula formation. Safal Shetty, MDa Naktal Hamoud, MDb Keri O’Farrell, RDCS, BAb Scott Lick, MDc Rajesh Janardhanan, MDa a
Department of Medicine University of Arizona Tucson b Department of Cardiology Sarver Heart Center University of Arizona Tucson c Department of Cardiothoracic Surgery University of Arizona Tucson
http://dx.doi.org/10.1016/j.amjmed.2016.06.027
2
The American Journal of Medicine, Vol -, No -,
-
2016
Figure (A) Real-time 3-dimensional transesophageal echocardiography demonstrating the aortic root to right ventricular outflow tract (RVOT) fistula. (B) Red arrow points to a dehiscence of the prosthetic aortic valve well visualized on real-time 3-dimensional transesophageal echocardiography. (C) Significant aortic regurgitation (red arrow).
References 1. Ginghina C, Nastase OA, Ghiorghiu I, Egher L. Continuous murmur— the auscultatory expression of a variety of pathological conditions. J Med Life. 2012;5(1):39-46. 2. Subramaniam K, Wei L. Left ventricular outflow tract to right atrial fistula after aortic valve replacement. J Cardiothorac Vasc Anesth. 2009;23(3):360-363. 3. Yared K, Solis J, Passeri J, et al. Three-dimensional echocardiographic assessment of acquired left ventricular to right atrial
shunt (Gerbode defect). J Am Soc Echocardiogr. 2009;22(4):435. e1-435.e3. 4. Tiwari KK, Salvati AC, di Summa M, et al. Aorta-to-right ventricular outflow tract fistula with coronary cusp prolapse. Asian Cardiovasc Thorac Ann. 2013;21(2):193-195. 5. Anguera I, Miro JM, Vilacosta I, et al. Aorto-cavitary fistulous tract formation in infective endocarditis: clinical and echocardiographic features of 76 cases and risk factors for mortality. Eur Heart J. 2005;26(3): 288-297.