Coronary Steal from a Native Aorta to Right Ventricle Tunnel after Norwood Palliation
Presenter
Kaitlin E. Swanson, MD, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL
Kaitlin E. Swanson, MD, Dr. Paul Tannous, M.D., Ph.D., FSCAI, Jeremy Fox, M.D., Conor O'Halloran, M.D. and Alan W. Nugent, M.B.B.S., FSCAI, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL
Keywords: Congenital Heart Disease (CHD) and Occlusion Devices
Title:
Coronary Steal from Native Aorta to Right Ventricle Tunnel after Norwood Palliation
Introduction:
Aorta-left ventricular tunnels and coronary artery fistulae are described lesions that can present with heart failure in the first year of life. Our patient presented for hemodynamic catheterization in the setting of Norwood single ventricle physiology, with concerns for clinical overcirculation and low systemic output. A connection from the native aorta to the right ventricle (RV) was discovered, thought to be contributing to both systemic hypoperfusion and coronary steal, and was closed successfully with a 5mm Micro Plug Set (KA Medical, LLC).
Clinical Case:
A 5-week-old male with history of double outlet RV, normally related great arteries, mitral atresia, and functional aortic atresia underwent Norwood operation with 6mm Sano shunt (valved femoral vein homograft) at 3 days of life. His post-operative course was complicated by moderate tricuspid regurgitation and presence of a coronary cameral fistula. He tolerated extubation but clinically remained overcirculated with congestive heart failure symptoms likely related to valve regurgitation and increased pulmonary blood flow, requiring milrinone therapy. He presented to the catheterization lab to better assess his physiology. An echocardiogram prior was notable for an unrestrictive atrial septal defect, moderate tricuspid regurgitation, normal RV systolic function, trivial neo-aortic valve regurgitation, and a small jet of high-velocity continuous flow directed from the subaortic region into the right atrium. Access was obtained in the right femoral vein and artery. Hemodynamics were notable for a cardiac index of 2.2 L/min/m2, Qp:Qs of 2.8 to 1. Mean PA pressures were 14mmHg, and pulmonary vascular resistance was normal at 1.3 Woods units.m2. Angiography demonstrated an abnormal connection from the native aortic root to the RV. It appeared analogous to a sinus of Valsalva aneurysm versus an aorta to RV tunnel. It had an elongated entry into the ventricle that tapered just before its entry. After multidisciplinary discussion with the medical and surgical teams, we decided to attempt occlusion. The tunnel was closed with a 5mm Micro Plug Set. Given concerns for possible thrombus propagation superiorly to the device in the remainder of the tunnel leading to coronary occlusion, the patient started heparin and aspirin, and eventually transitioned to aspirin and Xarelto. He transitioned to an oral afterload-reducing regimen and was discharged from the hospital at 80 days of life. He had a pre-Glenn catheterization at 4 months of age that demonstrated no residual flow through the tunnel and unobstructed coronary flow. Tricuspid regurgitation remained moderate, and both neo- and native-aortic valve regurgitation were described as trivial.
Discussion:
Management of overcirculation in single ventricle physiology can be a challenge, especially in the setting of moderate tricuspid regurgitation. Our patient had an additional source of inefficiency with the native aorta to ventricle tunnel. The patient was able to wean support after the procedure and ultimately be discharged home. This case teaches us that a full survey is necessary to identify potential etiologies, multidisciplinary approaches can be beneficial when dealing with unexpected results, and sometimes it’s not just a diagnostic catheterization.