ARTICLE IN PRESS doi:10.1510/icvts.2010.251298 Editorial
www.icvts.org
ESCVS article - Congenital
Received 10 August 2010; received in revised form 3 November 2010; accepted 5 November 2010
Historical Pages Brief Case Report Communication
Our institutional review board approved the study, and all the parents of the study patients gave their written informed consent. The patients were included prospectively at our surgical center from January 2004 to June 2009. All patients underwent echocardiography. The first 11 patients then underwent both cardiac catheterization with angiography and MDCT. Subsequently, cardiac catheterization was not performed.
Nomenclature
䊚 2011 Published by European Association for Cardio-Thoracic Surgery
2.1. Study design
Best Evidence Topic
*Corresponding author. Departement des Cardiopathies Conge ´nitales, Centre Chirurgical Marie-Lannelongue, 133 avenue de la Re ´sistance, 92350 Le Plessis Robinson, France. Tel.: q33-1-40948525; fax: q33-1-40948507. E-mail address:
[email protected] (E. Le Bret).
2. Materials and methods
State-of-the-art
Sinus venosus-atrial septal defect (SV-ASD) is a defect involving both the atrial septum and the inflow of the superior vena cava (SVC). One or both right-sided pulmonary veins may drain into the right atrium directly or via a vena cava, a feature known as partial anomalous pulmonary venous return (PAPVR) w1, 2x. SV-ASDs account for 10–15% of all atrial septal defects w1x. Surgical repair is the only treatment for SV-ASD, and the best surgical strategy varies according to the configuration of the veins. Therefore, obtaining accurate preoperative information on the venous abnormalities is highly desirable w2–4x. Although transthoracic echocardiography (TTE) is the usual first-line imaging technique for both the diagnosis and the preoperative workup of SV-ASD, the restricted imaging windows beyond the heart limit the assessment of extracardiac abnormalities, such as PAPVR. Cardiac catheterization usually provides the definitive diagnosis but is both invasive and costly. In contrast, contrast-enhanced multidetector computed
tomography (MDCT) is a minimally-invasive investigation that has been reported to be highly accurate for detecting anomalous venous connections w5–7x. To our knowledge, the diagnostic performance of MDCT for detecting PAPVR associated with SV-ASD has not been evaluated in a large series of pediatric patients. The aim of this study was to evaluate the diagnostic accuracy of MDCT for detecting PAPVR in pediatric patients with high suspicion of SV-ASD (abnormal pulmonary venous return, no visualization of pulmonary venous ostia, or unexpected dilatation of right cavities on TTE). Surgery was the reference standard.
Follow-up Paper
1. Introduction
Negative Results
Keywords: Computed tomography; Partial abnormal pulmonary venous return; Sinus venosus; Children, sensitivity, specificity
Proposal for Bailout Procedure
Objective: To prospectively assess the value of multidetector computed tomography (MDCT) for detecting partial anomalous pulmonary venous return (PAPVR) in children with suspected sinus venosus-atrial septal defect (SV-ASD). Methods: Forty-four children (mean age, 7.3 years; range, nine months–16 years) from whom transthoracic echocardiography (TTE) was inconclusive for the diagnosis underwent MDCT after contrast medium injection. Diagnosis was suspected on TTE by abnormal pulmonary venous return, no visualization of pulmonary venous ostia, or unexpected dilatation of right cavities. The first 11 children also underwent cardiac catheterization. Surgical findings constituted the diagnostic reference standard. Results: Thirty-two (73%) children had SV-ASD with PAPVR. Of the first 11 patients, one had PAPVR by MDCT and 10 by conventional angiography; these 11 patients had PAPVR by surgery. Of the remaining 33 patients, 21 had SV-ASD and 12 had ostium secundum ASD, by both MDCT and surgery. MDCT had 100% sensitivity, 100% specificity, 100% positive predictive value, and 100% negative predictive value for diagnosing PAPVR in patients with suspected SV-ASD. Conclusion: Contrast-enhanced MDCT is a highly accurate, minimally-invasive technique for detecting PAPVR associated with SV-ASD. Contrast-enhanced MDCT may be used safely to replace conventional angiography for the definitive diagnosis and preoperative evaluation of children with suspected SV-ASD. 䊚 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.
ESCVS Article
Abstract
Institutional Report
Department of Pediatric Cardiology, Centre Chirurgical Marie-Lannelongue, Le Plessis Robinson, France b Department of Radiology, Centre Chirurgical Marie-Lannelongue, Le Plessis Robinson, France
a
Protocol
Flore Amata, Emmanuel Le Breta,*, Anne Sigal-Cinqualbreb , Mathieu Coblencea, Virginie Lamberta, Adela Rohneanb, Jean Franc ¸ois Paulb
Work in Progress Report
Diagnostic accuracy of multidetector spiral computed tomography for preoperative assessment of sinus venosus atrial septal defects in children
New Ideas
Interactive CardioVascular and Thoracic Surgery 12 (2011) 179–182
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The following data were recorded based on findings from MDCT, cardiac catheterization, and surgery: whether PAPVR was present, number of pulmonary veins with abnormal connections, presence of anomalous systemic venous connections, and whether other cardiovascular abnormalities were found. When PAPVR is present, the location of the anomalous venous connection relative to the azygos vein– SVC junction is a major consideration for planning surgery. We therefore, described this feature on the MDCT images, as follows: type A junction close to the right atrium (SVC transposition not required) or type B junction close to the azygos vein–SVC junction (SVC transposition possibly required). MDCT findings were compared to surgical findings, which served as the reference standard. MDCT findings were also compared to cardiac catheterization findings (first 11 patients). 2.2. Cardiac catheterization technique Cardiac catheterization with conventional angiography was performed only in the first 11 patients. Of these 11 patients, five required premedication only, three required general anesthesia, and three required local anesthesia. 2.3. Multidetector computed tomography technique Due to technological improvements over time, 16-slice, 64-slice, and dual-source MDCT were used in succession. All machines were from the same manufacturer (Siemens Medical Solutions, Forchheim, Germany). We performed spiral acquisition without cardiac gating using 0.6- or 0.7mm collimation. To minimize radiation exposure of the patients, we used an 80-kV tube with high pitch (1.2–1.5). Mean radiation dose was estimated in millisieverts (mSv) from the dose-length product after correction for body size. Non-ionic low-osmolar contrast medium (iopromide 300 mg Iyml) in a dosage of 2 mlykg was injected into a peripheral vein at a low rate (0.5–1 mlys) to avoid streak artifacts in the SVC. None of the patients received anesthesia. Sedation was given if needed. Time from injection to scanning initiation was set at 40 s to ensure homogenous contrast at the venous phase. Experienced staff and readers were required to manage children and reading images. Analysis was guided by the results of TTE.
diography findings were considered conclusive. The remaining 44 patients were included. Their mean age was seven years four months (range, nine months–16 years). Surgery showed that 32 of the 44 patients had SV-ASD with PAPVR and that 12 patients had ostium secundum ASD with normal pulmonary vein connections. In the 11 patients investigated by both MDCT and cardiac catheterization, surgery consistently showed SV-ASD with PAPVR. MDCT showed SV-ASD with PAPVR in all 11 patients and cardiac catheterization in 10 patients. MDCT detected PAPVR in all 32 patients with SV-ASD and PAPVR by surgery (no false-negatives) and in none of the 12 patients without PAPVR (no false-positives). Thus, compared to surgery for the detection of PAPVR in children with suspected SV-ASD, MDCT had 100% sensitivity w95% CI (0.8911; 1)x, 100% specificity w95% CI (0.7354; 1)x, 100% PPV, and 100% NPV. Youden’s index was 1, indicating perfect diagnostic accuracy of contrast-enhanced helical MDCT for detecting PAPVR in children with suspected SV-ASD w8x. Of the 32 patients with PAPVR, 22 had MDCT findings of type A junction (Figs. 1 and 2) and 10 of type B junction (Fig. 3). Surgery consistently confirmed the MDCT findings regarding junction type. Multiple abnormalities of pulmonary vein connections were found in 23 patients (Videos 1 and 2). Of these 23 patients, seven had separate connections of the upper and lower right-sided pulmonary veins and one had an accessory vein. Furthermore, five patients had abnormal connections of the left-sided pulmonary veins or abnormal systemic vein connections. All these abnormalities were visualized by MDCT. No adverse events of MDCT were recorded. Acquisition time ranged from 1 to 3 s and the radiation dose ranged from 0.5 to 1.5 mSv.
2.4. Statistical analysis In the first 11 patients, we compared the MDCT and angiography findings to the surgical findings on a case-bycase basis. Then, in the overall population of 44 patients, we computed the sensitivity (95% CI), specificity (95% CI), positive predictive value (PPV), and negative predictive value (NPV) of MDCT vs. surgery for detecting PAPVR. 3. Results From January 2004 to June 2009, 46 patients younger than 18 years of age were referred to our surgical center for TTE suspicion of SV-ASD: detection abnormal pulmonary venous return by the TTE, no visualization of pulmonary venous ostia, or an unexpected dilatation of right cavities. Among them, two were not included, because the echocar-
Fig. 1. Sinus venosus with type A junction (low) in maximum intensity projection. SVC, superior vena cava; AO, aorta; RA, right atrium; arrowheads, right inferior pulmonary vein; arrow, right superior pulmonary vein.
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181 Editorial New Ideas Work in Progress Report Proposal for Bailout Procedure Negative Results Follow-up Paper
Treatment was with creation of a baffle to redirect the venous flow in 22 patients whose right-sided pulmonary veins connected to the SVC close to the right atrium. The remaining 10 patients were managed using Warden’s procedure. There were no postoperative deaths and no patient experienced postoperative complications.
ESCVS Article
Fig. 2. Sinus venosus with type A junction (low) in volume rendering technique. SVC, superior vena cava; AO, aorta; RA, right atrium; arrowheads, right inferior pulmonary vein; arrow, right superior pulmonary vein.
State-of-the-art Best Evidence Topic Nomenclature
PAPVR is common in patients with SV-ASD w4x and requires a specific surgical procedure selected based on the location of the abnormal venous connections w3x. Thus, connection of the right-sided pulmonary veins to the SVC close to the right atrium can be managed by creating a baffle to redirect the venous flow. In contrast, when the connection is located high on the SVC, near the azygos vein–SVC junction, the preferred treatment is Warden’s procedure (SVC division and anastomosis to the right atrial appendage with closure of the septal defect) w9–11x. Accurate preoperative assessment of the septal defect and venous connections allows the surgeon to plan the procedure and therefore, to decrease the risk of complications, such as SVC obstruction or sinus node injury w2, 4x. TTE provides accurate information on the hemodynamic consequences of ASDs. However, this method may fail to provide a complete assessment of the anatomical abnormalities. Furthermore, the diagnostic performance of echocardiography is heavily dependent on the operator’s experience and the patient’s acoustic window w12, 13x. Cardiac catheterization may be performed to assess suspected SVASD but involves a high-level of radiation exposure w14, 15x, as well as risks associated with the invasiveness of the procedure. Cardiac catheterization may require general anesthesia, particularly in young children, and may fail to detect sinus venosus defects and PAPVR w16x. In our study, contrast-enhanced MDCT identified all cases of SV-ASD and PAPVR and also provided detailed information on the venous connections. MDCT was equal or superior to cardiac catheterization in the first 11 patients for diagnosing SV-ASD
Institutional Report
4. Discussion
Protocol
Video 1. Sinus venosus with type B junction and multiple superior pulmonary veins. Right pulmonary artery has been electronically eliminated. Arrows showing several superior pulmonary veins.
Historical Pages
Video 2. Sinus venosus with type B junction and multiple superior pulmonary veins. All vessels are in place.
Brief Case Report Communication
Fig. 3. Sinus venosus with type B junction (high) in volume rendering technique. SVC, superior vena cava; AO, aorta; RA, right atrium; blue arrow, right inferior pulmonary vein; white arrow, right superior pulmonary vein draining near the azygos vein.
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with PAPVR. MDCT is faster, less expensive, and less invasive than cardiac catheterization. Mild brief sedation is sufficient, and general anesthesia is unnecessary. High-quality images are obtained with radiation exposures of -1.5 mSv w17x, so that the benefits of the procedure far outweigh the theoretical risks associated with irradiation w18x, even in children. Accurate 3-D images of the abnormal vein connections are obtained, making MDCT highly reliable as a tool for planning the surgical strategy. The main limitation of our study is the single-center recruitment of patients with suspected SV-ASD based on echocardiography findings. As the predictive value of a test varies with the prevalence of the abnormality being sought, and as echocardiography is heavily operator dependent, our findings may not be applicable to other centers. 5. Conclusion MDCT is a minimally-invasive imaging tool that is highly accurate for detecting PAPVR in patients with suspected SV-ASD. The detailed 3-D anatomical information provided by MDCT is helpful for selecting the surgical strategy. Based on the results reported here, we have stopped performing cardiac catheterization in children with suspected SV-ASD. Instead, we routinely obtain MDCT after echocardiography and before surgery. References w1x Van Praagh S, Carrera ME, Sanders SP, Mayers JE, Van Praagh R. Sinus venosus defects: unroofing of the right pulmonary veins – anatomic and echocardiographic findings and surgical treatment. Am Heart J 1994;128:365–379. w2x McCarthy KP, Ho SE, Anderson RHA. Defining the morphologic phenotypes of atrial septal defects and interatrial communications. Images Paediatr Cardiol 2003;15:1–24. w3x Duncan BW. Sinus venosus atrial septal defect: repair with an intrasuperior vena cava baffle. Operat Tech Thorac Cardiovasc Surg 2006; 11:33–44.
w4x Gustafson RA. Cavo-atrial anastomosis technique for partial anomalous pulmonary venous connection to the superior vena cava – the Warden procedure. Operat Tech Thorac Cardiovasc Surg 2006;11:22–32. w5x Dillman JR, Yarram SG, Hernandez RJ. Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol 2009;192:1272–1285. w6x Kim TH, Kim YM, Suh Ch, Cho DJ, Park IS, Kim WH, Lee YT. Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR Am J Roentgenol 2000;175:1381–1386. w7x Kawano T, Ishii M, Takagi J, Maeno Y, Eto G, Sugahara Y, Toshima T, Yasunaga H, Kawara T, Todo K, Kato H. Three-dimensional helical computed tomographic angiography in neonates and infants with complex congenital heart disease. Am Heart J 2000;139:654–660. w8x Youden WJ. Index for rating diagnostic tests. Cancer 1950;3:32–35. w9x Warden HE, Gustafson RA, Tarnay TJ, Neal WA. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1984;38:601–605. w10x Gustafson RA, Warden HE, Murray GF, Hill RC, Rozar GE. Partial anomalous pulmonary venous connection to the right side of the heart. J Thorac Cardiovasc Surg 1989;98:861–868. w11x Gustafson RA, Warden HE, Murray GE. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;60:S614–S617. w12x Kronzon I, Tunick PA, Freedberg RS, Trehan N, Rosenzweig BP, Schwinger ME. Transesophageal echocardiography is superior to transthoracic echocardiography in the diagnosis of sinus venosus atrial septal defect. J Am Coll Cardiol 1991;17:537–542. w13x Muhler EG, Engelhardt W, Von Bernuth G. Detection of sinus venosus atrial septal defect by two-dimensional echocardiography. Eur Heart J 1992;13:453–456. w14x Rassow J, Schmaltz AA, Hentrich F, Streffer C. Effective doses to patients from paediatric cardiac catheterization. Br J Radiol 2000; 73:172–183. w15x Dragusin O, Gewillig M, Desmet W, Smans K, Struelens L, Bosmans H. Radiation dose survey in a paediatric cardiac catheterization laboratory equipped with flat-panel detectors. Radiat Prot Dosimetry 2008;129:91– 95. w16x Freed MD, Nadas AS, Norwood WI, Castaneda AR. Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects. J Am Coll Cardiol 1984;4:333–336. w17x Lembcke A, Razek V, Kivelitz D, Rogalla N, Rogalla P. Sinus venous atrial septal defect with partial anomalous pulmonary venous return: diagnosis with 64-slice spiral computed tomography at low radiation dose. J Pediatr Surg 2008;43:410–411. w18x Rice HE, Frush DP, Farmer D, Waldhausen JH, APSA Education Committee. Review of radiation risks from computed tomography: essentials for the pediatric surgeon. J Pediatr Surg 2007;42:603–607.
