Images of Pediatric and Congenital Heart Disease
Three-Dimensional Rotational Angiography During Percutaneous Device Closure of Fontan Fenestration
World Journal for Pediatric and Congenital Heart Surgery 4(3) 324-325 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113484162 pch.sagepub.com
James Hill, MD1, Christopher Bellotti, MD1, and Alex Golden, MD1
Abstract The Fontan circulation was originally described as palliation for tricuspid atresia but was subsequently used for a wide variety of single-ventricle physiologies, with the common thread being inability to separate the ventricles for independent use in the systemic and pulmonary circulations. Fontan baffle fenestrations have been created since the late 1980s to assist in maintaining the cardiac output in the presence of increased resistance to flow through the pulmonary circuit. Many institutions continue to use an approach to Fontan circulation that utilizes routine fenestration followed by percutaneous device closure at a later time, whereas other institutions use fenestrations sparingly. We present the case of a five-year-old female with hypoplastic left heart syndrome with a fenestrated Fontan, who underwent three-dimensional rotational angiography at the time of catheterization for percutaneous device closure of the fenestration. Keywords Fontan, angiography, congenital heart disease, imaging (all modalities), pediatric Submitted January 30, 2013; Accepted March 5, 2013.
The Fontan circulation was originally described as palliation for tricuspid atresia but was subsequently used for a wide variety of single-ventricle physiologies, with the common thread being inability to separate the ventricles for independent use in the systemic and pulmonary circulations.1 Fontan baffle fenestrations have been created since the late 1980s to assist in maintaining cardiac output in the presence of increased resistance to flow through the pulmonary circuit. Of course maintaining cardiac output in those situations comes at a price: decreased saturations from right-to-left shunting through the fenestration. Many institutions continue to use an approach to Fontan circulation2 that utilizes routine fenestration followed by percutaneous device closure at a later time, whereas other institutions use fenestrations sparingly.3 We present the case of a 5-year-old female with hypoplastic left heart syndrome who had her original palliation with a Norwood and right ventricle to pulmonary artery conduit in the newborn period followed by a bidirectional Glenn at 6 months of age, both performed at an outside hospital. She presented to our institution for her third stage of palliative surgery and underwent an extracardiac Fontan at 4 years of age. The decision was made to fenestrate based on preoperative hemodynamics, with a mean superior vena cava pressure of 16 mm Hg and angiography that showed a need for significant pulmonary arterioplasty at the time of the Fontan. She had initial saturations in the low 80% range after her Fontan, and her
Fontan pressures were approximately 20 mm Hg in the early postoperative period. She recovered from her surgery and remained hemodynamically stable, but several months after her surgery the saturations remained in the range of 78% to 82%. She was brought to the catheterization laboratory approximately one year later for hemodynamic assessment and potential fenestration closure. Fontan mean pressure was 13 mm Hg with right ventricular end-diastolic pressure of 7 mm Hg. With balloon test occlusion of the fenestration, her saturation increased from 70% to 99%, Fontan pressure increased mildly to 15 mm Hg, and cardiac output remained stable. Complete anatomical surveillance was obtained with three-dimensional (3-D) rotational angiography (Siemens DynaCT, Munich, Germany)4,5 as shown in Figure 1 and in the accompanying supplemental video. As shown in Figure 2, the fenestration was then closed successfully with a 15-mm Helex septal occluder (Gore Medical,
1 Department of Pediatric Cardiology and Pediatric Cardiothoracic Surgery, Cleveland Clinic Children’s Hospital, Cleveland, OH, USA
Corresponding Author: James Hill, Department of Pediatric Cardiology and Pediatric Cardiothoracic Surgery, M-41, Cleveland Clinic Children’s Hospital, 9500 Euclid Avenue, Cleveland, OH 44195, USA. Email: [email protected]
Downloaded from pch.sagepub.com at Karolinska Institutets Universitetsbibliotek on May 30, 2015
Hill et al
325
Figure 1. Single frame in right anterior oblique/cranial angulation from three-dimensional rotational angiogram performed with a 5.5second power injection of 20 mL of one-third strength contrast (Ultravist; Bayer Healthcare, Wayne, New Jersey) through a 4F Pigtail catheter in the Fontan baffle demonstrated the complete Fontan circulation with unobstructed flow into the pulmonary arteries. A stream of contrast is seeing traversing the fenestration and opacifying the right atrium. The three-dimensional movie of this angiogram is shown in the supplemental material.
Flagstaff, Arizona), under fluoroscopic and transesophageal echocardiographic guidance. As an adjuct guidance tool, the 3-D volume set was superimposed on the fluoroscopic image during device deployment. It is our opinion that this newly available 3-D modality can be very helpful in understanding and navigating the complex anatomy that can characterize pre- and postoperative congenital heart lesions. Specifically, we believe that 3-D angiography can provide improved diagnostic capabilities, rapid selection of optimal angulation for 2-D imaging during the procedure, and real-time intraprocedural guidance by superimposition of the 3-D volume set on the live fluoroscopic image. We suspect that this technology will facilitate reductions in radiation doses, contrast doses, anesthesia time, and ultimately will improve clinical outcomes. Further research will be necessary to determine whether these expected benefits are observed. Supplemental Material Supplemental video located online at http://wjpchs.sagpub.com/ supplemental.
Figure 2. Single frame from a standard two-dimensional angiogram in right anterior oblique angulation after device closure of the Fontan fenestration demonstrated successful occlusion of the fenestration. A tiny residual right-to-left shunt was seen at the superior margin of the 15-mm Helex device, but there was no residual shunting seen the next day on transthoracic echocardiography.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26(3): 240-248. 2. Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation. 1990;82(5): 1681-1689. 3. Bando K, Turrentine MW, Park HJ, Sharp TG, Scavo V, Brown JW. Evolution of the Fontan procedure in a single center. Ann Thorac Surg. 2000;69(6): 1873-1879. 4. Fahrig R, Fox AJ, Lownie S, Holdsworth DW. Use of a C-arm system to generate true three-dimensional computed rotational angiograms: preliminary in vitro and in vivo results. AJNR Am J Neuroradiol. 1997;18(8): 1507-1514. 5. Glatz AC, Zhu X, Gillespie MJ, Hanna BD, Rome JJ. Use of angiographic CT imaging in the cardiac catheterization laboratory for congenital heart disease. JACC Cardiovasc Imaging. 2010;3(11): 1149-1157.
Downloaded from pch.sagepub.com at Karolinska Institutets Universitetsbibliotek on May 30, 2015
Three-Dimensional Rotational Angiography During Percutaneous Device Closure of Fontan Fenestration
World Journal for Pediatric and Congenital Heart Surgery 4(3) 324-325 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113484162 pch.sagepub.com
James Hill, MD1, Christopher Bellotti, MD1, and Alex Golden, MD1
Abstract The Fontan circulation was originally described as palliation for tricuspid atresia but was subsequently used for a wide variety of single-ventricle physiologies, with the common thread being inability to separate the ventricles for independent use in the systemic and pulmonary circulations. Fontan baffle fenestrations have been created since the late 1980s to assist in maintaining the cardiac output in the presence of increased resistance to flow through the pulmonary circuit. Many institutions continue to use an approach to Fontan circulation that utilizes routine fenestration followed by percutaneous device closure at a later time, whereas other institutions use fenestrations sparingly. We present the case of a five-year-old female with hypoplastic left heart syndrome with a fenestrated Fontan, who underwent three-dimensional rotational angiography at the time of catheterization for percutaneous device closure of the fenestration. Keywords Fontan, angiography, congenital heart disease, imaging (all modalities), pediatric Submitted January 30, 2013; Accepted March 5, 2013.
The Fontan circulation was originally described as palliation for tricuspid atresia but was subsequently used for a wide variety of single-ventricle physiologies, with the common thread being inability to separate the ventricles for independent use in the systemic and pulmonary circulations.1 Fontan baffle fenestrations have been created since the late 1980s to assist in maintaining cardiac output in the presence of increased resistance to flow through the pulmonary circuit. Of course maintaining cardiac output in those situations comes at a price: decreased saturations from right-to-left shunting through the fenestration. Many institutions continue to use an approach to Fontan circulation2 that utilizes routine fenestration followed by percutaneous device closure at a later time, whereas other institutions use fenestrations sparingly.3 We present the case of a 5-year-old female with hypoplastic left heart syndrome who had her original palliation with a Norwood and right ventricle to pulmonary artery conduit in the newborn period followed by a bidirectional Glenn at 6 months of age, both performed at an outside hospital. She presented to our institution for her third stage of palliative surgery and underwent an extracardiac Fontan at 4 years of age. The decision was made to fenestrate based on preoperative hemodynamics, with a mean superior vena cava pressure of 16 mm Hg and angiography that showed a need for significant pulmonary arterioplasty at the time of the Fontan. She had initial saturations in the low 80% range after her Fontan, and her
Fontan pressures were approximately 20 mm Hg in the early postoperative period. She recovered from her surgery and remained hemodynamically stable, but several months after her surgery the saturations remained in the range of 78% to 82%. She was brought to the catheterization laboratory approximately one year later for hemodynamic assessment and potential fenestration closure. Fontan mean pressure was 13 mm Hg with right ventricular end-diastolic pressure of 7 mm Hg. With balloon test occlusion of the fenestration, her saturation increased from 70% to 99%, Fontan pressure increased mildly to 15 mm Hg, and cardiac output remained stable. Complete anatomical surveillance was obtained with three-dimensional (3-D) rotational angiography (Siemens DynaCT, Munich, Germany)4,5 as shown in Figure 1 and in the accompanying supplemental video. As shown in Figure 2, the fenestration was then closed successfully with a 15-mm Helex septal occluder (Gore Medical,
1 Department of Pediatric Cardiology and Pediatric Cardiothoracic Surgery, Cleveland Clinic Children’s Hospital, Cleveland, OH, USA
Corresponding Author: James Hill, Department of Pediatric Cardiology and Pediatric Cardiothoracic Surgery, M-41, Cleveland Clinic Children’s Hospital, 9500 Euclid Avenue, Cleveland, OH 44195, USA. Email: [email protected]
Downloaded from pch.sagepub.com at Karolinska Institutets Universitetsbibliotek on May 30, 2015
Hill et al
325
Figure 1. Single frame in right anterior oblique/cranial angulation from three-dimensional rotational angiogram performed with a 5.5second power injection of 20 mL of one-third strength contrast (Ultravist; Bayer Healthcare, Wayne, New Jersey) through a 4F Pigtail catheter in the Fontan baffle demonstrated the complete Fontan circulation with unobstructed flow into the pulmonary arteries. A stream of contrast is seeing traversing the fenestration and opacifying the right atrium. The three-dimensional movie of this angiogram is shown in the supplemental material.
Flagstaff, Arizona), under fluoroscopic and transesophageal echocardiographic guidance. As an adjuct guidance tool, the 3-D volume set was superimposed on the fluoroscopic image during device deployment. It is our opinion that this newly available 3-D modality can be very helpful in understanding and navigating the complex anatomy that can characterize pre- and postoperative congenital heart lesions. Specifically, we believe that 3-D angiography can provide improved diagnostic capabilities, rapid selection of optimal angulation for 2-D imaging during the procedure, and real-time intraprocedural guidance by superimposition of the 3-D volume set on the live fluoroscopic image. We suspect that this technology will facilitate reductions in radiation doses, contrast doses, anesthesia time, and ultimately will improve clinical outcomes. Further research will be necessary to determine whether these expected benefits are observed. Supplemental Material Supplemental video located online at http://wjpchs.sagpub.com/ supplemental.
Figure 2. Single frame from a standard two-dimensional angiogram in right anterior oblique angulation after device closure of the Fontan fenestration demonstrated successful occlusion of the fenestration. A tiny residual right-to-left shunt was seen at the superior margin of the 15-mm Helex device, but there was no residual shunting seen the next day on transthoracic echocardiography.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26(3): 240-248. 2. Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation. 1990;82(5): 1681-1689. 3. Bando K, Turrentine MW, Park HJ, Sharp TG, Scavo V, Brown JW. Evolution of the Fontan procedure in a single center. Ann Thorac Surg. 2000;69(6): 1873-1879. 4. Fahrig R, Fox AJ, Lownie S, Holdsworth DW. Use of a C-arm system to generate true three-dimensional computed rotational angiograms: preliminary in vitro and in vivo results. AJNR Am J Neuroradiol. 1997;18(8): 1507-1514. 5. Glatz AC, Zhu X, Gillespie MJ, Hanna BD, Rome JJ. Use of angiographic CT imaging in the cardiac catheterization laboratory for congenital heart disease. JACC Cardiovasc Imaging. 2010;3(11): 1149-1157.
Downloaded from pch.sagepub.com at Karolinska Institutets Universitetsbibliotek on May 30, 2015
