Radiographic characteristics of Cook detachable and Gianturco coils as well as clinical results of transcatheter closure of the patent ductus arteriosus

PURPOSE: To describe the radiographic appearance of the Gianturco and the Cook detachable coils and present the clinical results in patients who underwent transcatheter closure of patent ductus arteriosus. MATERIALS AND METHODS: Between January 1994 and June 1997, eighty-two patients underwent closure of patent ductus arteriosus (PDA) using either Gianturco or Cook detachable coils. The chest x-ray and echocardiography of all patients were reviewed and the following parameters were evaluated: 1) the size of the heart (cardiothoracic ratio), 2) the position and the type of the coils in the postero-anterior and the lateral projection, 3) the number of coils used, 4) the existence of residual ductal flow, 5) Doppler velocity in the left pulmonary artery. RESULTS: Complete occlusion was achieved in 94%, and cardio-thoracic ratio regressed from 0.57 to 0.53 (p < 0.01), after a mean follow-up of 1.2 years. The identification of the different coils on the chest radiograph was successful in only 47% of cases, difficulties arising especially, when multiple coils were used. In 55 patients (67%) the coil position was judged to be optimal, in 27 patients (33%) suboptimal. The latter correlates with the presence of residual shunt. Multiple coils correlated more with a left pulmonary artery flow velocity exceeding 1.5 m/s. CONCLUSION: Coil-occlusion of patent ductus arteriosus is effective and leads to reduced cardio-thoracic ratio. Radiographic coil identification is possible but may be difficult if multiple coils are deployed. Suboptimal coil position led more often to residual PDA shunt. Multiple coils are more commonly associated with increased LPA velocities, but hemodynamic significant obstruction to flow is rare.     

EVALUATION OF PULMONARY FLOW BY DOPPLER ECHOCARDIOGRAPHY AND SCINTIGRAPHY AFTER PERCUTANEOUS CLOSURE OF PATENT DUCTUS ARTERIOSUS

Background: Most patients who submitted to percutaneous closure of a patent ductus arteriosus (PCPDA), show some degree of protrusion of the occlusive device into the left pulmonary artery (LPA). The purpose of this study is to assess the prevalence of coil protrusion, and whether it causes significant obstruction. Methods: A study group (SG) of 70 patients was submitted to PCPDA with Gianturco coils (mean age: 8.6 years). Doppler Echocardiographic studies were performed, with a mean time follow‐up of 55 months. Coil and LPA were assessed by suprasternal, short‐axis, and high left parasternal views. LPA diameter was indexed to body surface area. A Doppler Index was calculated by the differences of the velocities from each pulmonary artery in relation to the pulmonary trunk, expressed in percentage (VD index). Pulmonary scintigraphy was performed using intravenous injection of 99 Tcm labeled macroaggregated albumin to evaluate lung perfusion. The diameters and disturbances in flow velocities by Doppler echocardiography of the pulmonary arteries were compared with lung perfusion by pulmonary scintigraphy. The same measurements were performed in a control group (CG) of 22 patients (normal or with minimal heart defects). VD Index values of the SG were compared with pulmonary scintigraphy values of the CG to establish normal and abnormal values. Mean time interval between the two studies was 81 days. Results: In 66 patients (95%), the coil protruded into the LPA. Mean LPA diameter was 13mm (SG and CG). Mean VD Index was 22% in the SG and 1.7% in the CG. A cut‐off value of 50% for the VD Index was determined (ROC curve), with 100% sensibility and 98% of specificity. In 8 patients (11%) the VD Index was > 50%, with abnormal left pulmonary perfusion (LPP) in 7 patients. A significant correlation between the VD Index and LPP was demonstrated (R²= 65.19). To estimate the LPP by Doppler a regression equation was obtained: LPP = 47.8 ? 0.09 × VD Index. The LPA diameter was compared between patients with VD Index > 50%, < 50%, and CG, with no statistically significant difference (p = 0.6). Conclusion: Protrusion of the coil to LPA was frequently found. LPA diameters did not correlate with flow disturbances and reduced LPP. Patients showing blood flow acceleration >50% at the left pulmonary artery by Doppler have significantly lower values of lung perfusion.     

Transcatheter management of residual shunts after initial transcatheter closure of a patent arterial duct

OBJECTIVES: To assess the efficacy and safety of transcatheter reocclusion of persistent leaks following previously attempted transcatheter occlusion of persistent arterial duct. DESIGN: Retrospective study. SETTING: Tertiary pediatric cardiology centre. PATIENTS: From February 1987 through October 1996, trans-catheter occlusion of a residual ductal shunt was attempted in 42 consecutive patients at a median age of 5.0 years (range 1.6 years to 16.2 years). INTERVENTIONS: Fourty patients had successful placement of a double umbrella occluder (n=27) or coils (n=13) across residual shunts. Complications included device embolization in two patients and hemolysis in one patient. OUTCOME MEASURES AND RESULTS: Mean z-score for left ventricular end-diastolic dimension (LVEDD) at initial echocardiography was +2.55 +/- 1.89 (P<0.0001 versus normal); z-score for left pulmonary artery (LPA) diameter was +2.00 +/- 1.52 (P<0.0001). Mean LPA to right pulmonary artery (RPA) diameter ratio was 1.05 +/- 0.18. At follow-up echocardiogram, a median of two years (range six months to 7.7 years) after the second procedure, a shunt was persistent in 3% of the patients. Mean LVEDD and LPA diameter z-value, and mean LPA to RPA diameter had dropped significantly to +0.42 +/- 1.31, +0.07 +/- 1.15 and 0.86 +/- 0.14 (P<0.001), respectively. LPA flow acceleration was present in 25% of patients. Three of nine patients, in whom lung perfusion scan was performed, had left lung perfusion below 40%. Small weight and age at catheterization were significant risk factors for LPA flow disturbance. CONCLUSIONS: Repeat transcatheter occlusion is safe and successful in eliminating residual shunt across the arterial duct. Attention should, however, be addressed to the potential for LPA stenosis and growth, and flow should be regularly assessed.     

Pulmonary perfusion scans following transcatheter patent ductus arteriosus closure using the Amplatzer devices

Introduction: Obstruction to flow in the left pulmonary artery (LPA) is a well‐known complication after transcatheter device closure of patent ductus arteriosus (PDA). This complication has been studied for different devices using lung perfusion radionuclide scintigraphy (LPRS), but not for Amplatzer devices. This study was performed to evaluate the effect of such devices on lung perfusion using LPRS. Methods: This is a retrospective study that looked at all patients who had PDA closure using different Amplatzer devices at our center between July 1999 and January 2007. All patients underwent LPRS within 24 hr of the procedure. We compared LPRS with other hemodynamic data obtained by cardiac catheterization and echocardiography. Results are presented as mean ± SD or median and ranges. Results: A total of 70 patients had PDA closure using an Amplatzer device; median age was 1.8 years (4 months to 75 years) and median weight was 12 kg (5–112 Kg). Nine patients had associated cardiac anomalies. Sixty eight patients had available LPRS. The mean percent of left lung perfusion (LLP) was 42.7% (±6.7%). Excluding patients with pre‐existing LPA stenosis, 17% had abnormally decreased LLP. On hemodynamic measurements, 62 patients had available direct pressure measurements following PDA closure. None had significant increase. No correlation was found with echocardiographic data. Conclusion: PDA closure with Amplatzer family of devices is associated with a relatively significant risk of decreased perfusion to the left lung, mostly mild abnormalities. Comparison with catheterization and echocardiographic measurements showed lack of correlation with LPRS findings.© 2010 Wiley‐Liss, Inc.     

Does ductal occlusion with the gianturco coil cause left pulmonary artery and/or descending aorta obstruction?

Thirty-two patients (median age 4.5 years) underwent transcatheter Gianturco coil occlusion of a patent ductus arteriosus. Transthoracic echocardiography was performed the day after coil placement and at intermediate follow-up (median 8.6 months). Echocardiographic results were compared with angiographic and hemodynamic data obtained during catheterization. Two-dimensional (2D) echocardiography performed the day after ductal occlusion displayed evidence of coil protrusion into the left pulmonary artery in 28 of 31 patients (90%) and into the descending aorta in 17 of 29 (59%). However, pulsed Doppler analysis demonstrated normal left pulmonary arterial flow velocities in 28 of 29 patients (97%) and normal descending aortic flow velocities in 26 of 27 (96%). Pulse Doppler results were corroborated by angiographic and hemodynamic catheterization data, which showed no evidence of adjacent vessel obstruction. Peak Doppler velocities among patients with and without 2D echocardiographic left pulmonary artery or descending aorta coil impingement did not differ significantly. The discrepancy between 2D and pulse Doppler findings did not change significantly at intermediate follow-up. Thus, transcatheter occlusion of the patent ductus arteriosus with properly implanted Gianturco coils does not cause significant obstruction to flow in the left pulmonary artery or descending aorta despite frequently misleading 2D echocardiographic images of coil impingement on these vessels.     

Lung perfusion studies after transcatheter closure of persistent ductus arteriosus with the Amplatzer duct occluder

Background : Reduced left lung perfusion has been described after transcatheter closure of the patent ductus arteriosus (PDA) with several prostheses. Although the Amplatzer ductal occluder (ADO) device is currently the most widely used occluder for closure of large‐sized PDAs, the potential consequences of flow distribution to the lungs of this device have not been completely clarified. We evaluated lung perfusion following occlusion of PDA with the ADO device. Methods : Forty‐seven patients underwent successful transcatheter PDA occlusion using the ADO device were included in this study. Lung perfusion scans were performed 6 months after the procedure. Results : Decreased perfusion to the left lung (defined as < 40% of total lung flow) was observed in 17 patients (36%), 5 of whom were low‐weight symptomatic infants. Ductal ampulla length was significantly shorter and minimal ductal diameter to ampulla diameter ratio was significantly higher in patients with decreased left lung perfusion and correlated well with left lung perfusion values (r = 0.516 and r = ?0.501, respectively). A cut‐off value of ≤5.8 mm for the ductal ampulla length and ≥1.9 for ampulla diameter to ampulla length ratio showed high sensitivity and specificity for reduced lung perfusion. Conclusions : The incidence of abnormal left lung perfusion is high 6 months after transcatheter closure of PDA with the ADO, more likely in the low weight symptomatic infants and in patients with a short duct or a relatively shallow duct having abrupt narrowing of a large ampulla. © 2010 Wiley‐Liss, Inc.     

Lung perfusion studies after detachable coil occlusion of persistent arterial duct

OBJECTIVE: To evaluate relative lung perfusion following complete occlusion of persistent arterial duct with detachable Cook coils. METHODS: Ductal occlusion using detachable coils was performed in 35 patients (median age 3.9 years, range 0.5 to 16; 32 native ducts, three patients with previous devices). If the duct could be crossed with a 0.035 inch guidewire and a 4 F catheter after coil implantation, a further coil was implanted. Between one and seven coils were used (median two). RESULTS: Complete ductal occlusion was confirmed by echocardiography 24 hours after the procedure in all patients. Lung perfusion scans were performed three months after the procedure in 33 of 35 patients (two older patients with a single coil each did not attend). Decreased perfusion to the left lung (defined as < 40% of total lung flow) was observed in only one patient, who had previously had a 17 mm Rashkind umbrella implanted. There was no correlation between left lung perfusion and peak left pulmonary artery Doppler velocities (r = 0.27 and p = 0.125 for the entire group; r = 0.29 and p = 0.124 after excluding patients with previous devices). CONCLUSIONS: Coil occlusion is effective in achieving complete closure of the duct. An aggressive approach using multiple coils did not compromise perfusion to the left lung.     

Advantages and disadvantages of coils for transcatheter closure of patent ductus arteriosus

Different coils have been used to close the patient ductus arteriosus (PDA). In small- and moderate-sized PDA, coils are an adequate alternative to surgery and/or to other devices. The aim of the study is to review and discuss the advantages and disadvantages of using coils (excluding PFM coils PFM Medical, Germany) to close PDA. Cambier was the first to successfully close a PDA using a Gianturco coil. To date, thousands of patients worldwide have undergone transcatheter closure of PDA using this or other types of coils. The use of coils is analyzed with regard to costs in comparison with other therapeutic modalities; techniques--anterograde, retrograde approach, selection of coil size--in relation to the size of the PDA and the available sizes of coils; efficacy of the rate of complete occlusion and the need for reocclusion; and safety in relation to embolization rate, other complications including hemolysis, left pulmonary artery LPA stenosis and coarctation. It is concluded that coils are a cheap alternative for the occlusion of PDA in the small-to-moderate PDA. The technique can be learned quite quickly, it has a high rate of complete occlusion, and has an acceptable rate of safety. The disadvantages include a moderate rate of coil embolization and of hemolysis in patients with residual shunt after coil occlusion in large PDAs. When more than one coil is used, the potential for developing LPA stenosis is high.     

Transcatheter closure of large patent ductus arteriosus (> or = 4 mm) with multiple Gianturco coils: immediate and mid-term results.

OBJECTIVE: To assess the immediate and mid-term results of transcatheter closure of patent ductus arteriosus (PDA) > or = 4 mm with multiple Gianturco coils. (Transcatheter closure of large PDAs using the Rashkind occluder or the buttoned device is associated with a 7-38% incidence of residual shunt.) METHODS: 19 patients (7 male, 12 female) underwent an attempt at anterograde transcatheter closure with multiple Gianturco coils of a large PDA at a median age of 3.8 yr (range 2 weeks-34 yr) and median weight of 14 kg (range 2.3-80 kg). RESULTS: The median PDA diameter at the narrowest segment was 4.3 mm (range 4-7 mm) and the mean (SD) Qp/Qs was 1.9 (0.8). Each patient had left atrial and left ventricular volume overload. A 4F catheter was used to deliver the coils in all patients. There was immediate and complete closure in 16/18; one patient had residual shunt that was closed at a second procedure and the other had spontaneous disappearance of the residual shunt at the six week visit. A short ductus (angiographic type B) in one patient could not be closed. The median number of coils placed at the first attempt to close the ductus was 4 (range 2-6 coils) and the median fluoroscopy time was 40 minutes (range 13-152 minutes). Mild left pulmonary artery stenosis occurred in the two smallest patients. Coil migration to the lung occurred in 3 patients with retrieval of coils in two patients. All procedures but one were done on an outpatient basis. At a median follow up of 1.6 yr (range 2 weeks-2.2 yr) all patients had complete closure with no new complications. CONCLUSIONS: Anterograde transcatheter closure with multiple Gianturco coils is an effective treatment for most patients with large PDA of diameters up to 7 mm. This technique can be performed in small infants on an outpatient basis without the need for general endotracheal anaesthesia.     

Safety of transcatheter patent ductus arteriosus closure in small weight infants

Background: Patent ductus arteriosus (PDA) is a frequent congenital heart disease. Its transcatheter closure has become the treatment of choice in children and adults. However, the device closure of PDA in children with low weight is still challenging with high rate of complications. The aim of this study was to report further experience with trancatheter closure of PDA using the Amplatzer Duct Occluder(ADO) for children weighing less or equal to 8 kg. Methods: Twenty‐two patients (5 male, 17 female) underwent transcatheter closure of a PDA using ADO at a median age of 10 months (range 4 to 26) and a median weight of 7 kg (range 4.3 to 8). Follow‐up evaluations were performed with Doppler echocardiography at 24h, and at 6 and 12 months. Results: The device was implanted successfully in all patients. The median fluoroscopy time was 17.25 min (range 10 to 29). Within 24h, color Doppler revealed complete closure in 15 patients (68%), the other patients had a small residual shunt. No deaths were associated with the procedure. Two patients had a slight aortic protrusion of the device without coarctation and in one patient the device encroached partially on the left pulmonary artery without significant acceleration on Doppler echocardiography. All patients were discharged home the next day. All patients completed the 6‐month follow‐up with complete closure in 18 patients (81%). At the last evaluation in all patients at any time, there has been documentation of complete PDA closure in 20 (91%) of 22 patients. Conclusion: In patients weighing less or equal to 8 kg, percutaneous closure of PDA using an ADO is a safe and effective procedure. (J Interven Cardiol 2012;25:391–394)     

Long-term outcome of transcatheter coil closure of small to large patent ductus arteriosus

Coil closure of patent ductus arteriosus (PDA) has become an accepted alternative to surgical closure in most pediatric cardiac centers. However, little is known about the mid- to long-term outcome of this procedure. Therefore, we evaluated the immediate, short-, and long-term outcome of transcatheter coil closure (TCC) of PDA using single or multiple Gianturco coils or the Gianturco-Grifka Vascular Occlusive Device (GGVOD). One hundred forty-nine patients underwent an attempt at TCC of their PDAs at a median age of 2.4 years (2 weeks to 55 years) and median weight of 13.5 kg (2.3–87 kg). There were 33 patients < 1 year of age. The median PDA minimal diameter was 2 mm (0.4–7 mm) with 26 patients whose PDA minimal diameter was > 4 mm. A 4 Fr catheter was used for coil deployment in 136 patients, a 3 Fr in 4, and an 8 Fr in 4 patients who received the GGVOD. A single coil was used in 77 patients and multiple coils (2–6) were used in 66 patients. One hundred forty-six patients had successful closure (142 had immediate complete closure and 4 had residual shunt), 3 patients failed the initial attempt (2 underwent surgical ligation and 1 had a successful second attempt a year later). Of the four patients with residual shunt, three underwent a second procedure with implantation of 1–3 coils resulting in complete closure in all and one patient had spontaneous resolution of the residual shunt. Complications were encountered in nine patients: six had coil migration with successful retrieval in four; two had left pulmonary artery stenosis (2.4 kg and 6.3 kg infants), and one patient had loss of femoral arterial pulse. The median fluoroscopy time was 16 min (2–152 min). One hundred forty-two patients had the procedure as an outpatient, five patients stayed greater than 24 hr, and two of these patients were in hospital for 1 month for noncardiac reasons. At a median follow-up interval of 3.0 years (1 month to 5.1 years), there were no episodes of delayed coil migration, delayed recanalization, thromboembolic episodes, or bacterial endocarditis. Lung perfusion scans performed at a median follow-up interval of 1.6 years in 31 patients who received multiple coils revealed 45% ± 5% blood flow to the left lung. Long-term follow-up of coil closure of PDA indicates that the technique is safe and effective for most pa-tients with PDA up to a diameter of 7 mm. Cathet. Cardiovasc. Intervent. 47:457–461, 1999. © 1999 Wiley-Liss, Inc.     

Transcatheter closure of patent ductus arteriosus and aorto‐pulmonary vessels using non‐ferromagnetic Inconel MReye embolization coils

Objectives . We report the use of non‐ferromagnetic embolization coils for transcatheter PDA closure. Background . Transcatheter patent ductus arteriosus (PDA) closure has been performed for 40 years. A number of devices have been used with varying degrees of success. Gianturco embolization coils have been used frequently since 1992 with excellent results. These coils are a stainless steel alloy, and create an artifact when subsequent MRI imaging is performed. Methods . Eight patients underwent right and left heart catheterization and transcatheter PDA closure. Angiography displayed a PDA with left to right shunting. The minimum PDA diameter was measured. An Inconel MReye coil was implanted using standard retrograde technique. A postimplant angiogram was performed. Evaluations were performed the following morning and after 2 months. Results . The median age was 5.5 years, median weight was 24 kg. The PDA minimum diameter was 1.7 mm (range 1.4–2.4 mm), with a median Qp:Qs=1.33:1. In all patients, the PDA was completely immediately closed using one Inconel coil. Two patients also had a small aorto‐pulmonary collateral vessel that was occluded using a separate Inconel coil. All patients had follow‐up evaluation the following day; the PDA remained completely occluded and there was no obstruction of the pulmonary artery branches or descending aorta. Seven patients had subsequent follow‐up and echocardiograms; the PDA remained completely occluded. There were no complications. Conclusion . The Inconel MReye coil is safe and effective for coil occlusion of small PDA and aorto‐pulmonary vessels. Additional studies are needed to define the maximum vessel diameter for Inconel coil occlusion. © 2008 Wiley‐Liss, Inc.     

Influence of pulmonary regurgitation inequality on differential perfusion of the lungs in tetralogy of Fallot after repair: a phase-contrast magnetic resonance imaging and perfusion scintigraphy study.

OBJECTIVES: The purpose of this study was to evaluate the influence of pulmonary regurgitation inequality on differential perfusion of the lungs in tetralogy of Fallot (TOF) after repair. BACKGROUND: Asymmetry of lung perfusion is one of the best predictors of outcome in TOF after repair. A recent phase-contrast magnetic resonance imaging (PC-MRI) study found prominent regurgitation inequality between the bilateral pulmonary arteries in TOF after repair. METHODS: Forty-three TOF post-repair patients (median age = 51 months, 31 men) received PC-MRI and 99mTc-labeled macroaggregates of albumin perfusion scintigraphy (PS) in the same day. We took PC-MRI measurements of forward flow volume (FFV), backward flow volume (BFV), and net flow volume (NFV) (NFV = FFV - BFV) and regurgitation fraction (RF) (RF = BFV/FFV) at the left and right pulmonary arteries (LPA and RPA). The differential perfusion of the left lung (L%) (L% = left lung/left + right lung) as calculated by NFV ratio, by FFV ratio of PC-MRI, and by PS were compared. RESULTS: The discrepancy between L% by NFV versus L% by PS was affected by the severity of RF of LPA (r = -0.51, p = 0.001); agreement between L% by NFV versus L% by PS was good (intraclass correlation coefficient [Ri] = 0.87) if RF of LPA <0.4 (n = 23) but downgraded (Ri = 0.51) and underestimated the L% (median of error = -14%, range = -25.3% to 5.5%) if RF of LPA > or =0.4 (n = 20). In contrast, agreement between L% by FFV versus L% by PS was high and unaffected by RF of LPA (Ri = 0.94, 0.92, respectively). CONCLUSIONS: While integrating PC-MRI of pulmonary artery as a comprehensive MRI evaluation of TOF after repair, conventional NFV ratio method tended to underestimate the left lung perfusion and may lead to unnecessary intervention. The FFV ratio method should be used for precise assessment of differential lung perfusion.     

动脉导管未闭合并重度肺动脉高压Amplatzer法封堵治疗的临床应用--12例初步报告

目的评价Amplatzer封堵器治疗动脉导管未闭(PDA)合并重度肺动脉高压(SPH)的初步疗效.方法对12例(女9例,男3例)PDA合并SPH患者实施封堵治疗.其中10例采用Amplatzer动脉导管未闭封堵器,2例采用Amplatzer房间隔缺损封堵器.结果全组12例PDA封堵器均放置成功.11例PDA封堵后30分至1小时肺动脉收缩压、肺动脉平均压均明显降低.1例封堵术后即刻肺动脉压无变化.术后36小时彩色多普勒估测肺动脉压明显下降,封堵术后30分降主动脉造影,无残余分流9例,微量残余分流2例,少量残余分流1例.全组术后24～48小时彩色多普勒检查,动脉水平左向右分流均完全消失.无重要并发症发生.随访1～24个月(平均8个月),患者症状改善,11例心脏缩小,无1例发生再通.结论采用Amplatzer法封堵治疗PDA合并SPH,近期疗效满意.     

经导管介入治疗冠状动脉瘘

目的：探讨经导管介入治疗冠状动脉瘘的方法及临床疗效。方法：经导管堵塞冠状动脉瘘14例，平均年龄7．1岁。结果：13例应用弹簧圈堵塞，平均瘘口大小为3．65mm，除3例失败外均获成功；1例（瘘口6．6mm）应用Amplatzer动脉导管未闭堵塞器堵塞成功。所有病例随访1个月－4年，均无残余分流及任何并发症。结论：经导管介入治疗冠状动脉瘘具有良好的临床疗效及安全性。可控弹簧圈一般用于堵塞瘘口较小的冠状动脉瘘，而瘘口较大的冠状动脉瘘可选用Amplatzer动脉导管未闭堵塞器。     

Pulmonary Perfusion Asymmetry in Patients after Repair of Tetralogy of Fallot: A 4D Flow MRI-Based Study

Background: Repaired Tetralogy of Fallot (rTOF) patients may have residual lesions such as main (MPA) and branch pulmonary artery stenosis (BPAS). While MPA stenosis is well studied, few data are available on BPAS in rTOF. We aimed to describe pulmonary perfusion in a large paediatric cohort of rTOF and its impact on right ventricular and outflow-tract hemodynamics using 4D flow CMR. Methods: 130 consecutive patients (mean age at CMR 14.3 ± 4.6 years) were retrospectively reviewed. 96 patients had transannular patch without valve preservation while 34 patients had conserved annulus or valved conduit. A pulmonary blood flow ratio (right pulmonary artery (RPA)/left pulmonary artery (LPA)) between 0.75 and 1.56 was considered normal. Results: Asymmetric pulmonary perfusion was present in 59/130 patients (45%), with 54/59 (91%) having left lung hypoperfusion (blood flow ratio >1.56). RPA/LPA perfusion ratio in the whole cohort was independently associated with the LPA Z-score (−0.053, p = 0.007), the RPA regurgitant fraction (RF) (0.013, p = 0.011) and previous LPA stenting (0.648, p = 0.004). Decreasing LPA % perfusion (and conversely increasing RPA % perfusion) was significantly associated with higher MPA diameter Z-score (−0.06, p = 0.007). On multivariate analysis, MPA Z-score was independently associated with pulmonary RF (0.48, p < 0.001) and with right ventricular indexed volumes (coefficient 3.6, p = 0.023). In patients with transannular patch repair, asymmetric pulmonary flow was an independent predictor of right ventricular ejection fraction (RVEF) (−3.66, p = 0.04). Conclusions: Pulmonary perfusion asymmetry is frequent in rTOF and is associated with abnormal right ventricular and outflow-tract hemodynamics, including MPA dilatation and decreased RVEF in patients after transannular patch.     

Tetralogy of Fallot with absent pulmonary valve—When the ductus is present: A case of isolated branch pulmonary artery and review of literature

Tetralogy of Fallot/Absent Pulmonary Valve (TOF/APV) has been classically associated with the absence of a patent ductus arteriosus (PDA). We present a rare case of APV in TOF with a discontinuous left pulmonary artery (LPA) that was suspected during fetal echocardiogram. Postnatal echocardiogram confirmed the origin of a hypoplastic LPA from the PDA. Despite an aneurysmal (right pulmonary artery) (RPA), axial imaging demonstrated widely patent tracheobronchial system with no evidence of bronchial compression. Clinically, the child required only minimal respiratory support. Genetic testing was positive for 22 q11deletion, commonly associated with this lesion. Surgery consisted of unifocalization of the discontinuous LPA with placement of a valved pulmonary homograft during complete repair of this lesion. Our case highlights the importance of prenatal detection, to aid in the prompt initiation of prostaglandins so as to ensure early rehabilitation of the left lung. Inability to visualize one of the branch pulmonary arteries (PA's) and a PDA on fetal echocardiogram in TOF/APV must raise suspicion for an eccentric branch PA with ductal origin.     

Validation of Doppler-derived pulmonary arterial pressure in patients with ductus arteriosus under different hemodynamic states

Twenty-nine patients with a patent ductus arteriosus (PDA) in isolation (n = 17) or in combination with other lesions (n = 12) underwent simultaneous hemodynamic assessment and evaluation of PDA flow velocity by the Doppler method. The accuracy with which Doppler velocity across the PDA predicted pulmonary arterial pressure and the influence of PDA size and shape on the Doppler velocity-pressure relationship were examined. Seventy percent had a cone-shaped PDA (narrowest at the pulmonary artery end), and the remainder were tubular. Narrowest PDA diameter ranged from 1.5 to 9 mm (mean 3.5 mm). Peak systolic and mean pulmonary arterial pressure ranged from 10 to 116 and 8 to 72 mm Hg, respectively. Twenty-one patients (group 1) had left-to-right shunting only. The following variables showed significant correlation in this group: peak instantaneous systolic aortic-to-main pulmonary arterial (MPA) pressure gradient and maximum Doppler velocity across the PDA (slope = 1.03, SEE = 13 mm Hg, r = .94, p less than .001), mean aortic-to-MPA pressure gradient and mean Doppler velocity (slope = 1.06, SEE = 10 mm Hg, r = .95, p less than .001), and end diastolic aortic-to-MPA pressure gradient and minimum Doppler velocity (slope = 1.12, SEE = 8 mm Hg, r = .96, p less than .001). Eight patients (group 2) had bidirectional shunting. In this group peak instantaneous aortic-to-MPA pressure gradient significantly correlated with maximum Doppler velocity measured from the left-to-right shunt (slope = .70, SEE = 2 mm Hg, r = .92, p less than .002) and mean pressure gradient correlated with mean Doppler velocity (slope = .83, SEE = 3 mm Hg, r = .78, p less than .003). Right-to-left Doppler velocities showed no correlation with pressures. In six patients with pulmonary hypertension Doppler velocity changes accurately predicted the effect of pulmonary vasodilation on pulmonary arterial pressure. Doppler velocity of PDA flow reliably predicts pulmonary arterial pressure over a wide range of pressures and PDA shapes and sizes.     

Stent angioplasty to relieve left pulmonary artery obstruction caused by patent ductus arteriosus device occlusion

A 7‐month‐old patient in congestive heart failure due to a moderate sized patent ductus arteriosus (PDA) underwent uncomplicated implantation of an Amplatzer Ductal Occluder (ADO1). Two months after percutaneous device PDA closure, left pulmonary artery (LPA) stenosis was discovered. Rather than spontaneous improvement as reported in previous cases, our patient's LPA stenosis progressed in severity 7 months after ADO1 placement. A catheterization demonstrated a 32 mm Hg peak gradient from her main pulmonary artery to her LPA. She underwent successful stent angioplasty of her LPA with an excellent result and preserved PDA closure. This case demonstrates that stent angioplasty is a feasible an effective method of relieving LPA obstruction caused by a PDA occluder device. Additionally, despite slight deflection by the stent, the ADO1 device continued to provide complete ductal closure. Stent angioplasty should be considered in patients who have LPA stenosis caused by ADO1 occluder device that does not improve over time. © 2013 Wiley Periodicals, Inc.     

Caval contribution to flow in the branch pulmonary arteries of Fontan patients with a novel application of magnetic resonance presaturation pulse

BACKGROUND: A complete understanding of fluid mechanics in Fontan physiology includes knowledge of the caval contributions to right (RPA) and left (LPA) pulmonary arterial blood flow, total systemic venous return, and relative blood flow to each lung. METHODS AND RESULTS: Ten Fontan patients underwent cine MRI. Three cine scans of the pulmonary arteries were performed: (1) no presaturation pulse, (2) a presaturation pulse labeling inferior vena cava (IVC) blood (signal void), and (3) a presaturation pulse labeling superior vena cava (SVC) blood. The relative signal decrease is proportional to the amount of blood originating from the labeled vena cava. This method was validated in a phantom. Whereas 60+/-6% of SVC blood flowed into the RPA, 67+/-12% of IVC blood flowed toward the LPA. Of the blood in the LPA and RPA, 48+/-14% and 31+/-17%, respectively, came from the IVC. IVC blood contributed 40+/-16% to total systemic venous return. The distributions of blood to each lung were nearly equal (RPA/LPA blood=0.94+/-11). CONCLUSIONS: In Fontan patients with total cavopulmonary connection, SVC blood is directed toward the RPA and IVC blood is directed toward the LPA. Although the right lung volume is larger than the left, an equal amount of blood flow went to both lungs. LPA blood is composed of equal amounts of IVC and SVC blood because IVC contribution to total systemic venous return is smaller than that of the SVC. This technique and these findings can help to evaluate design changes of the systemic venous pathway to improve Fontan hemodynamics.