Pediatric Sinus of Valsalva Aneurysm Treatment & Management: Medical Care, Surgical Care, Activity

Medical Care

Direct the medical care of the patient with a ruptured sinus of Valsalva aneurysm toward hemodynamic stabilization, prevention or treatment of endocarditis, and management of arrhythmias, cardiac ischemia, or both using the following indications and medications:

• Heart failure: Administer diuretics, digitalis, and ACE inhibitors, and perform stabilization of cardiac rhythm (as indicated).
• Cardiac ischemia: Administer nitrates and beta-blockers.
• Endocarditis: Standard prophylaxis is no longer routinely recommended. Standard prophylaxis should be used after an episode of bacterial endocarditis has occurred to prevent reoccurrence. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

Surgical Care

Prompt surgical therapy is recommended when a ruptured sinus of Valsalva aneurysm is diagnosed. A combined approach from the affected chamber and from inside the aorta is most helpful to allow inspection of the aortic valve and to avoid injury to the coronary vessels. The procedure is described as follows:

The fistula tract from the ruptured aneurysm is closed, and an associated ventricular septal defect can be repaired.

The aorta is reunited with the valve annulus either by direct anastomosis or by the interposition of a graft, if required. Competency of the aortic valve is tested using transesophageal Doppler ultrasound, and valve repair can be undertaken, if necessary. Preservation of the aortic valve, particularly in children, is of paramount importance; therefore, early surgical intervention may be warranted.

No consensus as to when to perform surgery on a fortuitously discovered unruptured sinus of Valsalva aneurysm has been reached. Regular follow-up of these patients using echocardiography or MRI to document the size of the aneurysm is indicated. Undertake elective repair of a known sinus of Valsalva aneurysm at the same time as surgical repair of any other intracardiac shunt or defect.

Although surgical closure is generally indicated and reserved for patients with ruptured sinus of Valsalva, particularly complicated cases (eg, severe aortic regurgitation, complex lesions [endocarditis, bicuspid aortic valve, tunnel-type fistulous connections, large defects, multiple rupture sites]), percutaneous closure has been found to be safe and effective in patients who are not candidates for bypass, have mild/absent aortic regurgitation, simple associated defects (eg, muscular ventricular septal defects, secundum atrial septal defect, small patent ductus arteriosus).

Percutaneous, transcatheter closure of a ruptured sinus of Valsalva aneurysm was first described in 1994.Numerous occluder devices have been used, especially the Amplatzer device.Guidance for transcatheter closure is provided by 2-dimensional or 3-dimensional transesophageal echocardiographic guidance.

Although the traditional approach for transcatheter closure is antegrade, successful closure with a retrograde approach has also been reported.

Activity

Patients with a sinus of Valsalva aneurysm should avoid participation in contact sports or activities involving vigorous exertion or sustained heavy lifting. Chest trauma may precipitate rupture of a sinus aneurysm.

Patients with ruptured aneurysms who are awaiting surgical repair can be allowed activity to tolerance levels. Activity may be limited because of symptoms of congestive heart failure.

Medication



Edward J Bayne, MD Sibley Heart Center Cardiology, Children's Healthcare of Atlanta; Assistant Professor, Division of Pediatric Cardiology, Emory University School of Medicine;

Edward J Bayne, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society of Echocardiography, American College of Cardiology, American Heart Association

Coauthor(s)

Lynn Cronin, MD Clinical Cardiology Fellow, Department of Pediatrics, Division of Cardiology, William Beaumont Hospital

Lynn Cronin, MD is a member of the following medical societies: American College of Physicians, American Society of Echocardiography

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Chief Editor

Howard S Weber, MD FSCAI, Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions



Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

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Sinus of Valsalva aneurysm. Color-flow Doppler ultrasonography is performed in the right ventricle through a supracristal ventricular septal defect with fingerlike prolapse of the right coronary sinus wall (arrow).