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Transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium
International Journal of Cardiology 170 (2013) 221–226
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International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium Debora Luciano a, Daniela Laux b, Younes Boudjemline b, Sébastien Hascoët c, Jean-René Lusson d, Clio Sorensen a, Caroline Ovaert e, Bernard Kreitmann a, Richard Van Praagh f, Alain Fraisse a,⁎ a
Cardiologie pédiatrique et congénitale, Hôpital de la Timone-Enfants, Marseille, France M3C, Centre de Référence Malformations Cardiaques Congenitales Complexes, Hôpital Necker-Enfants-Malades, 149 rue de Sèvres, 75015 Paris, France Service de Cardiologie, Hôpital Rangueil, CHU de Toulouse, Toulouse, France d CHU NGM St Jacques, rue Montalembert, Clermont Ferrand, France e Cardiologie Pédiatrique, Université Catholique de Louvain, Bruxelles, Belgium f Division of Cardiology, Children's Hospital, Harvard Medical School, Boston, MA, USA b c
a r t i c l e
i n f o
Article history: Received 5 May 2013 Received in revised form 26 September 2013 Accepted 19 October 2013 Available online 30 October 2013 Keywords: Congenital heart disease Intervention Pulmonary vein
a b s t r a c t Background: A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium. Methods: We retrospectively studied such patients in 5 institutions. Results: Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n = 2) and secundum atrial septal defects (n = 3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n = 2), secundum atrial septal closure (n = 2), left upper pulmonary vein stenosis stenting (n = 1), and coarctation stenting (n = 1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40 years of age tend to be symptomatic at presentation (p = 0.056). Conclusion: In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients. © 2013 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Partially anomalous pulmonary venous return (PAPVR) occurs in 0.4 to 0.7% of postmortem examinations with abnormal return of the pulmonary veins (PVs) to the central venous circulation. PAPVR occurs more frequently on the right side. The anomalous PV connection may be into the superior vena cava, right atrium, inferior vena cava, left innominate vein or coronary sinus. PAPVR is usually found in association with other cardiac malformations, most commonly in atrial septal defect (ASD). Surgical correction is the standard treatment when PAPVR results in right heart volume overload and/or clinical symptoms [1].
Occasionally, the existence of a dual drainage connecting the PAPVR to the left atrium (LA) has been reported [2–15]. In such patients, transcatheter therapy may represent an attractive alternative to surgery. Only a few cases have been published and data on mid-term results is lacking [2–9]. We retrospectively reviewed our experience in 5 centers to describe the anomalous connection and patients' characteristics and to better clarify the indications and results of transcatheter therapy. We also present a review of the feasibility and the safety of transcatheter closure in such cases. 2. Patients and method 2.1. Study population
⁎ Corresponding author at: Cardiologie pédiatrique et congénitale, Hôpital de la TimoneEnfants, 264 rue Saint Pierre, 13385 Marseille, France. Tel.: +33 491386750; fax: +33 491385638. E-mail address: [email protected] (A. Fraisse). 0167-5273/$ – see front matter © 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.10.061
Since January 2000, we retrospectively collected all data from patients with PAPVR and an additional drainage to the LA in 5 institutions (La Timone Hospital, Marseille, France; Necker-Enfants-Malades, Paris, France; CHU Rangueil, Toulouse, France; CHU NGM St Jacques, Clermont-Ferrand, France; Université Catholique de Louvain, Bruxelles,
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Belgium). Inclusion criterion was PAPVR with an additional connection to the LA, through a normal PV or any other connecting vein. We excluded patients with previous corrective surgery of anomalous pulmonary venous return because transcatheter closure may be indicated for a vertical vein (VV) left open after surgical redirection of PV flow to the LA [16]. All the charts were reviewed to collect medical history, age at presentation and diagnosis, clinical signs at diagnosis, echocardiographic evidence for PAPVR and additional connection to the LA as well as any other abnormality, result of computed tomography (CT) scan, age at catheterization(s), technical approach, hemodynamic data as well as difficulties and complications, type and size of device used in cases with transcatheter interventions, operative reports in cases with surgical therapy, and patient's follow-up and outcome. We searched MEDLINE through PubMed and Google search engines using a combination of the terms “partial abnormal pulmonary venous return” and “interventional catheterization”. We also used in combination the terms “vertical vein”, “levoatriocardinal vein” or “left superior vena cava” with “interventional catheterization”. Literature review included any unoperated PAPVR with an additional venous connection to the LA treated by interventional catheterization.
Table 2 Characteristics of patients with partial abnormal pulmonary venous return and dual supply undergoing transcatheter therapy. References
Year
Number of cases/type
Devices used
Recto [7] Forbess [2] Lee [4] Mas [8]
2007 1998 2007 2000
1/VV 2/SV (1), VV (1) 1/SV 1/SV
AVP I/6 Coils ADO I ADO I
Kasarala [5] Gomez [6] Dähnert [3] Cullen [9] Present report
2011 2012 2007 2012
1/VV 1/VV 2/VV 1/VV 8/SV (2), VV (6)a
AVP II AVP II Coils and ADO I AVP II ADO I, AMVSD, ASO, AVP I
2.2. Statistical analysis Descriptive statistics such as mean (± SD), median (range) and frequency were calculated for each demographic and clinical characteristic whenever appropriate. Fisher's exact test was used on categorical comparisons of the 2 groups of PAPVR whereas the Wilcoxon rank sum test was performed to compare the age at presentation and at transcatheter closure. A p value b0.05 was indicative of statistical significance. Statistical analysis was performed using R version 2.15.2 for Windows Software (©The R Foundation for Statistical Computing, www.r-project.org).
Associated procedures ASD closure Occlusion SAS (coils)
Left PV stenting, occlusion SAS (2), ASD closure (2), Coa stenting
ADO, Amplatzer Duct Occluder; AMVSD, Amplatzer muscular ventricular septal defect; ASD, atrial septal defect; ASO, Amplatzer Septal Occluder; AoV, aortic valve; AVP, Amplatzer Vascular Plug; Coa, aortic coarctation; PV, pulmonary vein; SAS, systemic arterial supply; SV, scimitar vein type; VV, vertical vein type. a One failure due to restriction of the left PV during occlusion testing.
patients: the right pulmonary artery and lung were moderately hypoplastic and a systemic arterial supply to the sequestration was found.
3. Results (Tables 1 and 2, Figs. 1–4) 3.1. Baseline characteristics The study population consisted of 10 patients (6 girls) presenting at a median age of 8 (0.1 to 54) years. Diagnosis was confirmed in all the cases by thoracic CT scan and/or cardiac catheterization. Two anatomic types of PAPVR were encountered (Figs. 1–3): - Vertical vein (VV) type in 8 cases, with dual venous drainage from the upper lobe of the left lung to the innominate trunk via a large VV and to the LA via another more or less developed left PV (Figs. 1 and 2). - Scimitar vein (SV) type in 2 cases with an anomalous venous connection from the middle and lower pulmonary lobes of the right lung to the inferior vena cava below the level of the diaphragm, associated with an anomalous venous connection of the SV to the LA (Figs. 1 and 3). There was a sequestration syndrome in both
The 2 patients with SV type presented with recurrent respiratory infections and dyspnea on exertion. The diagnosis was suspected on the chest X-ray with mildly hypoplastic right lung in one case. In the other patient, the classical radiological picture with a tubular shadow along the right heart border resembling a curved Turkish sword was seen. Transthoracic echocardiography showed an abnormal vessel connecting with the inferior vena cava–right atrium junction in both cases. The precise diagnosis of a SV was confirmed by a CTscan. Among the 8 VV type cases, only one 43-year-old woman presented with clinical signs of right ventricular failure due to the PAPVR, and a VV was seen by transthoracic echocardiography. Another 54-year-old woman presented dyspnea due to left sided congestive heart failure. She was previously operated upon an aortic coarctation at 12 years of age. Her symptoms at presentation were due to a severe bicuspid
Table 1 Characteristics of the patients and intervention. Pt no.
Age at cath (years)
Symptoms
1 2
8 21
3
16
4
1
5
13
VV
6 7
23 8
VV VV
8
43
+
VV
9
55
+ (related to AoS)
VV
10
16
+
PAPVR type
mPAP (mm Hg)
Qp/Qs
Devices
12 16
N/A 1.3/1
8/6 mm ADO I/6 8 mm AMVSD
15
N/A
6/4 mm ADO I
20
1/1
10 and 6 mm AVP
ASD
20
2/1
10 mm ASO
ASD ASD
15 15
1/1 N/A
16 mm AVP
30
N/A
14 mm ASO
18
N/A
13
N/A
VV SV VV
+
Associated CHD
Coa
SV
VV
Coa (repaired), AoS
Other treatments
Occlusion SAS (coils) Coa stenting (cvrdCP stent) Occlusion SAS (coils) ASD closure (24 mm ASO) ASD closure (ASO 16 mm) Left PV stent (10 mm, Genesis PG2910P) Surgery: AoV replacement, VV ligation Surgery: VV ligation
ADO, Amplatzer Duct Occluder; AMVSD, Amplatzer muscular ventricular septal defect; ASD, atrial septal defect; ASO, Amplatzer Septal Occluder; AoS, aortic stenosis; AoV, aortic valve; AVP, Amplatzer Vascular Plug I; cath, catheterization; CHD, congenital heart disease; cvrdCP, Covered Continuous Platinum stent (NuMED Inc., Hopkinton, New York, USA); Coa, aortic coarctation; mPAP, mean pulmonary artery pressure; Pt, patient; PAPVR, partial abnormal pulmonary venous return; PV, pulmonary vein; Qp/Qs, pulmonary to systemic flow ratio; SAS, systemic arterial supply; SV, scimitar vein type; VV, vertical vein type.
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Fig. 1. Two anatomic types of partial abnormal pulmonary venous return. In A, the vertical vein (VV) type with an abnormal left upper pulmonary vein (LUPV) connecting to the innominate trunk (IT) via a VV and to the left atrium (LA) via another more or less developed pulmonary vein (PV). The connection to the IT may also include the lower PV (dotted lines). In B, the scimitar vein (SV) type with an anomalous venous connection from the middle, lower (and potentially the upper: dotted lines) right PV to the inferior vena cava (IVC), associated with a connecting vein (CV) of the SV to the LA. RA, right atrium.
aortic valve stenosis with left ventricular hypertrophy and diastolic dysfunction. In her case, the VV was also seen by transthoracic echocardiography. In the 6 other asymptomatic patients, the PAPVR was diagnosed in 4 cases by transthoracic echocardiography. Three had signs of right ventricular volume overload with an associated ostium secundum ASD in 2 cases, while one patient had a small secundum ASD without right ventricular volume overload. In these 6 patients with an echocardiographic diagnosis of the VV, a CT-scan was performed to confirm the diagnosis and precise description of the PAPVR. In the last 2 cases, the VV type PAPVR was diagnosed on a systematic thoracic CT-scan performed for another reason: to assess the morphology of an aortic coarctation in a 16 year-old female, and
because of persistent sepsis with inflammatory syndrome after a surgical appendectomy for acute appendicitis in an 8 year-old girl. 3.2. Hemodynamics and transcatheter therapy (Table 1, Figs. 2–4) The 10 patients underwent cardiac catheterization at a median age and weight of 16 (1 to 55) years and 44.5 (9 to 85) kg, under general anesthesia in 8 patients and under local anesthesia in 2 adult patients. Right heart catheterization revealed normal pulmonary artery pressure in all cases (mean: 17 ± 5.5 mm Hg) except in the 43-year-old woman with VV type and signs of right ventricular failure. She had mildly elevated pulmonary artery pressure (mean: 30 mm Hg) and a severe
Fig. 2. A: Computed tomography scan showing a vertical vein (VV) type partial abnormal pulmonary venous return with an abnormal left upper pulmonary vein (LUPV) connecting to the innominate vein via a VV. In addition, there is a small pulmonary vein (PV, arrow) that connects to the left atrium (LA). In B, a frontal angiogram of the patient in Fig. 1 showing flow through a small LUPV. The VV is occluded with an 8/6 mm Amplatzer ADO I (C). In D, a controlled computed tomography scan shows 6 months after occlusion of the VV that the small left PV has increased in size near its connection to the LA.
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Fig. 3. Angiogram showing the venous drainage from the middle and lower lobes of the right lung through a scimitar vein (SV) with a stenotic connection to the inferior vena cava. This angiogram also reveals a tortuous connecting vein joining the SV to the left atrium (A, arrow). In B, occlusion of the SV is performed with an 8 mm Amplatzer muscular ventricular septal defect.
stenosis of the PV at the junction with the LA. The pulmonary to systemic blood flow ratio was calculated in 6 cases (mean: 1.15 ± 0.41). Only one patient with an associated large ASD had a significant leftto-right shunt with a pulmonary-to-systemic flow ratio of 2/1. In 2 patients, transcatheter therapy of the PAPVR was not attempted. In one case, the parents declined transcatheter closure and surgical ligation of the VV was accomplished. In the 55-year-old patient with a severely stenotic bicuspid aortic valve, surgical aortic valve replacement was performed with concomitant ligation of the VV. These 2 patients were excluded from subsequent analysis. Transcatheter closure was attempted in 8 patients at a median age of 16 (1 to 43) years. An occlusion test was performed with a balloon in 6 cases, including the 2 SV type cases and 4 patients with a VV type. The left PV pressure did not change in 4 cases, whereas it significantly increased in 2 VV type cases (Fig. 4). In one asymptomatic patient without significant left-to-right shunt closure of the VV was not performed and a secundum ASD measuring 5 mm by transesophageal echocardiography was also left open. The other patient was the 43-year-old woman with signs of right ventricular failure and pulmonary hypertension. A left PV angiogram revealed a stenosis of the venous connection at the junction with the LA. A stent was successfully implanted and the PV pressure did not change after a new test occlusion of the VV. In 7 patients, transcatheter therapy of the PAPVR was successfully performed. The 2 patients with SV types underwent occlusion of their SV with Amplatzer
devices (AGA Medical Corporation, MN, USA) just proximal to their insertion into the inferior vena cava (Fig. 3). An anomalous systemic arterial supply was occluded with PDA coils (Cook Cardiology, Inc., Bloomington, Indiana) in both patients. In the 5 remaining VV type patients, occlusion of the VV was also performed with Amplatzer devices. In one case, occlusion of the VV with a 16/14 mm Amplatzer Duct Occluder failed, with device embolization in the left pulmonary artery. The device was successfully retrieved and the VV was closed one month later with an Amplatzer Septal Occluder device. Other associated cardiac malformations were concomitantly treated in 3 patients: an aortic coarctation was successfully stented in one teenager and a secundum ASD was percutaneously closed in 2 other cases. 3.3. Follow-up (Fig. 2D) Postcatheterization and postoperative courses were uneventful in the 9 treated patients. They were discharged home 24 h after catheterization whereas the 2 operated patients went home on postoperative days 7 and 10, respectively. Four patients were put on anti-platelet therapy after coarctation stenting (n = 1), LUPV stenting (n = 1) and ASD closure (N = 2) for 6 months. After a median follow-up of 3.1 (0.2 to 11.2) years, the 10 study patients are asymptomatic. By TTE, there is normal right ventricular volume and pulmonary artery pressure in all
Fig. 4. Pressure recorded in the left pulmonary vein before (A) and during the occlusion of the vertical vein (B) showing significant rise of the V wave (related to the pulmonary blood flow) from 10 to 15 mm Hg.
D. Luciano et al. / International Journal of Cardiology 170 (2013) 221–226 Table 3 Comparison between the 2 types of partial abnormal pulmonary venous connection with dual venous supply by Fisher's exact test or the Wilcoxon rank sum test (*). Characteristics
Age at presentation (year-old) Age at transcatheter repair (year-old) Female Symptoms at presentation Pulmonary hypertension (mean N 25 mm Hg) Significant left-to-right shunt (Qp/Qs N 1.5/1) Complication Associated procedure
VV type
SV type
Mean (±SD) or numbers (%)
Mean (±SD) or numbers (%)
22.5 (±21.9) 23.9 (±21.3)
15.9 (±22.1) 16.4 (±22)
p value
0.34* 0.3*
9 (69%) 6 (46%) 2 (15%)
2 (40%) 5 (100%) 1 (20%)
0.32 0.1 1
5 (38%)
3 (60%)
0.61
1 (8%) 5 (38%)
0 (0%) 3 (60%)
1 0.61
SD, standard deviation; SV, scimitar vein type; VV, vertical vein type.
the cases, including the untreated patient with a restrictive left PV. One patient had a CT-scan 6 months after transcatheter closure of a VV because the drainage was initially poorly developed between the VV and the LA. It appears more developed (Fig. 2D) with unrestrictive flow by pulsed and color Doppler. 3.4. World experience on transcatheter therapy for PAPVR (Tables 2 and 3) Results of the literature review of transcatheter closure in patients with PAPVR and dual supply [2–9] are presented in Table 2, including the 8 cases from the present study. In total, 18 transcatheter repairs of PAPVR were attempted in 11 females (61%), at a median age of 13.5 (1 to 65) years. The VV type PAPVR was the most frequently encountered in 13 cases (72%). In a few cases, the connection between the innominate vein and left PV was described as a persistent left superior vena cava [7] or as a levoatriocardinal vein [9]. However, there was no visible connection with the coronary sinus and the angiographic pictures were similar to the other VV type cases. In addition to our findings in the VV type, there were 2 patients in whom all the PVs from the left lung connected with the innominate vein via a VV that returned to the LA through a single left PV [5,6]. Morphologic variations in the SV cases included no systemic arterial supply in 2 cases [2,4] and all the PVs of the right lung draining into the SV in 2 patients [4,8]. Eleven patients (61%) presented with symptoms, all of whom were over 40 years of age at presentation. With the exception of the patients from the present study with a failed occlusion test (Fig. 4), all the procedures were successful with various coils and Amplatzer devices (Table 2). No complications occurred, except for the device embolization in one of our study patients. Additional transcatheter procedures were performed in 8 cases (44%), most frequently secundum ASD closure (3 cases). Comparison between the VV and SV types of PAPVR showed no difference (Table 3). Patients presenting after 40 year-old tend to have more symptoms (p = 0.056). 4. Discussion We report a series of 10 PAPVR cases with dual venous drainage to the systemic veins and to the LA. Two anatomic types are described (Figs. 1–3), the VV type being the most frequent. Percutaneous correction of the PAPVR was feasible in the majority of the cases. Similar to previous reports, transcatheter repair was easily accomplished, without complications [2–9]. Transcatheter closure of the abnormal venous connection is certainly the treatment of choice for PAPVR with dual venous supply, although surgical ligation of the SV [14] or of the VV [3] was successfully reported and also performed in two of our study patients. In addition to providing a less invasive therapeutic approach than surgery, interventional catheterization offers the possibility of performing an occlusion test. This is
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important when the connecting vein to the LA is potentially too small to receive all the redirected pulmonary venous flow after VV or SV closure. In two of our study patients, the occlusion test resulted in a significant increase of the left PV pressure (Fig. 4). One of them was untreated whereas PV–LA junction stenosis was successfully stented in the other patient before closure of the VV. Various types of occlusion devices and coils were implanted in our study patients and in previously published cases. In two of our cases and in the majority of the reports, the Amplatzer vascular plugs I and II that are specifically designed to embolize vascular structure of high flow, were successfully used [2,3,5–7,9]. Conversely in five of our study patients and in few other reported cases [2,4,8], other Amplatzer devices were used in positions they have not been designed for, like the Amplatzer Duct Occluder, Muscular Ventricular Septal Defect Occluder and Septal Occluder, respectively designed for patent ductus arteriosus, muscular ventricular septal defect and secundum ASD closure. Our choice of devices was based on size and morphology of the vein to occlude. Except for one initial failure with an Amplatzer Duct Occluder device too small to occlude a large VV, all the other devices were deployed uneventfully and all the collateral veins were closed without further complications. The incidence of PAPVR cases with dual drainage is difficult to estimate, especially in VV types since patients are often asymptomatic and may escape detection. Echocardiographic signs of right ventricular volume overload should raise suspicion, as well as an enlarged superior vena cava. The question remains whether or not asymptomatic cases without any hemodynamically significant left-to-right shunt should be corrected, even by a less invasive transcatheter approach. However, these defects share the same risk that any long standing left-to-right shunt does, since they combine drainage of PV to the right side (as do other types of PAPVR) and free drainage of the LA to the right atrium. Moreover, any connection between a systemic vein and the LA may provide pathways for right-to-left shunting as possible sources for paradoxical embolism [7,9,17]. Interestingly, two of the previously published patients with a VV type presented with stroke-like symptoms. Although there was no significant left-to-right shunt, transcatheter correction was recommended and successfully performed [7,9]. Other cases of patients experiencing neurological symptoms without patent foramen ovale on transesophageal echocardiography have been diagnosed as having venous communications between the innominate trunk and the LA by contrast echocardiography and/or CT scan [17]. In the present study, four patients of the VV type and no clinical symptoms or significant left-to-right shunt underwent transcatheter (n = 3) or surgical (n = 1) closure of the VV. PAPVRs from the left lung are usually connected to the left cardinal system, including the innominate vein and the coronary sinus. In the present study we call the connector to the innominate vein a VV. Embryologically, the VV is the left precardinal (or anterior cardinal or superior cardinal) vein [1]. Some authors name it a persistent left superior vena cava [7,11,15]. However it is now widely agreed that if such a VV does not communicate with the coronary sinus, it should not be called a persistent left superior vena cava, even though the left superior vena cava is the left superior cardinal vein that opens into the left common cardinal vein (the coronary sinus) [1]. The VV has also been termed the levoatrio or levoatrial cardinal vein [9]. As its name indicates, the levoatrial cardinal vein is a vein that communicates both with the left atrium and with the left superior cardinal vein. In other words, this is a left PV that has an abnormal persisting anastomosis with the left superior cardinal vein. It is often associated with severe pulmonary venous and left atrial obstruction caused by mitral atresia or severe stenosis. In this hemodynamic situation, the levoatrial cardinal vein helps to decompress the hypertensive pulmonary veins and left atrium. Interestingly, levoatrial cardinal vein has also been occasionally reported in association with coarctation of the aorta [18], as in two of our patients. This raises the
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question concerning whether coarctation of the aorta predisposes toward persistence of anastomoses between pulmonary and adjacent systemic veins, such as the left superior cardinal vein, resulting in a levoatrial cardinal vein. Finally, the distinction between a levoatrial cardinal vein and a coronary sinus septal defect (unroofed coronary sinus) was not made in this study. Unroofing of the coronary sinus because of a coronary sinus septal defect between the coronary sinus posteriorly and the LA anteriorly may be difficult or impossible to distinguish diagnostically (by imaging studies) from a levoatrial cardinal vein. In both anomalies the left superior cardinal vein communicates with the LA. Anatomically, the difference is that a pulmonary vein opens into the LA in a more superior or cephalad location than does a coronary sinus septal defect which is located more inferiorly or caudal, this being the relationship between the common pulmonary vein (above) and the coronary sinus (below). The size of the communication with the LA can be a diagnostically distinguishing feature. PVs openings into the LA are relatively small whereas unroofing of the coronary sinus can be total, resulting in a large opening into the LA. But coronary sinus septal defects can also be small [1,19]. Semantically in the present study, we prefer to call the connector to the innominate vein a VV, as in the majority of published cases [2,3,5,6]. Interestingly, almost one century ago, an anatomist from Edinburgh University named TB Johnston accurately described: “A rare vascular anomaly—Opening of the left upper pulmonary vein into a persistent left superior vena cava”. This is probably the first published description of this anomaly [15]. 4.1. Study limitations Several factors may have influenced the results of the present study. Besides its retrospective design, these include bias in case reports, unreported procedures, and undiagnosed additional connection to the LA. Because different devices were used for occlusion of the PAPVR, we cannot recommend any specific device for transcatheter therapy. Finally, in general, it is difficult to compare the results of the present study to those published in the literature because patient mix is heterogenous in age, indication of treatment, and PAPVR anatomy. 5. Conclusion We describe two anatomic types of PAPVR with additional connection to the LA. Patients tend to be more frequently symptomatic after 40 years of age. Transcatheter therapy can be offered as an alternative to surgery in the majority of patients. It seems indicated even in the asymptomatic VV type because of the risk of left-to-right shunt and of paradoxical embolization. Potential conflict of interest Alain Fraisse is a consultant and proctor for St Jude.
Acknowledgment We acknowledge Pascal Amedro, MD (Service de Pédiatrie 1, Hôpital Arnaud de Villeneuve, 34095 Montpellier cedex 10, France) and François Wernert, MD (cabinet de cardiologie, 20 rue Guirlande 13002, Marseille, France). References [1] Ward KE, Mullins CE. Anomalous pulmonary venous connection, pulmonary vein stenosis, and atresia of the common pulmonary vein. In: Garson A, editor. The science and practice of pediatric cardiology. 2nd ed. Williams and Wilkins; 1998. p. 1431–62. [2] Forbess LW, O'Laughlin MP, Harrison JK. Partially anomalous pulmonary venous connection: demonstration of dual drainage allowing nonsurgical correction. Catheter Cardiovasc Diagn 1998;44:330–5. [3] Dähnert I, Riede FT, Kostelka M. Partial anomalous pulmonary venous drainage of the left upper pulmonary vein. Catheter interventional treatment is sometimes possible. Clin Res Cardiol 2007;96:511–3. [4] Lee ML, Yang SC, Yang AD. Transcatheter occlusion of the isolated scimitar vein anomaly camouflaged under dual pulmonary venous drainage of the right lung by the Amplatzer ductal occluder. Int J Cardiol 2007;115: e90–3. [5] Kasarala G, Fiore A, Schowengerdt K, Jureidini S. Partial anomalous pulmonary venous return: transcatheter repair. Pediatr Cardiol 2011;32:1238–40. [6] Gomez J, Soledispa C. Redirection of anomalous venous pulmonary flow to left atrium using a vascular plug II. J Invasive Cardiol 2012;24:E96–8. [7] Recto MR, Sadlo H, Sobczyk WL. Rare case of persistent left superior vena cava to left upper pulmonary vein: pathway to paradoxical embolization and development of transient ischemic attack and subsequent occlusion with an Amplatzer vascular plug. J Invasive Cardiol 2007;19:E313–6. [8] Mas C, Goh TH, Wilkinson JL. New interventional therapeutic approach for dual drainage of the scimitar vein. Catheter Cardiovasc Interv 2000;51: 192–5. [9] Cullen EL, Breen JF, Rihal CS, Simari RD, Ammash NM. Levoatriocardinal vein with partial anomalous venous return and a bidirectional shunt. Circulation 2012;126: e174–7. [10] Odman P. A persistent left superior vena cava communicating with the left atrium and pulmonary vein. Acta Radiol 1953;40:554–60. [11] Gazzaniga AB, Matloff JM, Harken DE. Anomalous right pulmonary venous drainage into the inferior vena cava and left atrium. J Thorac Cardiovasc Surg 1969;57:251–4. [12] Pearl W. Scimitar variants. Pediatr Cardiol 1987;8:139–41. [13] Geggel RL. Scimitar syndrome associated with partial anomalous pulmonary venous connection at the supracardiac, cardiac and infracardiac levels. Pediatr Cardiol 1993;14:234–7. [14] Takeda S, Imachi T, Arimitsu K, Minami M, Hayakawa M. Two cases of scimitar variant. Chest 1994;105:292–314. [15] Johnston TB. A rare vascular anomaly—opening of the upper left pulmonary vein into a persistent left superior vena cava. J Anat Physiol 1915; 49:182–6. [16] Hares DL, Tometzki AJ, Martin R. Use of the Amplatzer vascular occluder to occlude large venous vessels in adults and children with congenital heart disease: a case series. Catheter Cardiovasc Interv 2007;69:33–9. [17] Saremi F, Vojdani E, Vorobiof G, et al. Right to left shunting through communications between the left superior intercostal vein tributaries and the left atrium: a potential cause of paradoxical embolism. Int J Cardiol Sep 2013;167(6):2867-74. [18] Bernstein HS, Moore P, Stanger P, Silverman NH. The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary-systemic connection. J Am Coll Cardiol 1995;26:995–1001. [19] Douglas YL, Jongbloed MRM, DeRuiter MC, Gittenberger-de Groot AC. Normal and abnormal development of pulmonary veins: state of the art and correlation with clinical entities. Int J Cardiol 2011;147:13–24.
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International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium Debora Luciano a, Daniela Laux b, Younes Boudjemline b, Sébastien Hascoët c, Jean-René Lusson d, Clio Sorensen a, Caroline Ovaert e, Bernard Kreitmann a, Richard Van Praagh f, Alain Fraisse a,⁎ a
Cardiologie pédiatrique et congénitale, Hôpital de la Timone-Enfants, Marseille, France M3C, Centre de Référence Malformations Cardiaques Congenitales Complexes, Hôpital Necker-Enfants-Malades, 149 rue de Sèvres, 75015 Paris, France Service de Cardiologie, Hôpital Rangueil, CHU de Toulouse, Toulouse, France d CHU NGM St Jacques, rue Montalembert, Clermont Ferrand, France e Cardiologie Pédiatrique, Université Catholique de Louvain, Bruxelles, Belgium f Division of Cardiology, Children's Hospital, Harvard Medical School, Boston, MA, USA b c
a r t i c l e
i n f o
Article history: Received 5 May 2013 Received in revised form 26 September 2013 Accepted 19 October 2013 Available online 30 October 2013 Keywords: Congenital heart disease Intervention Pulmonary vein
a b s t r a c t Background: A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium. Methods: We retrospectively studied such patients in 5 institutions. Results: Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n = 2) and secundum atrial septal defects (n = 3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n = 2), secundum atrial septal closure (n = 2), left upper pulmonary vein stenosis stenting (n = 1), and coarctation stenting (n = 1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40 years of age tend to be symptomatic at presentation (p = 0.056). Conclusion: In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients. © 2013 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Partially anomalous pulmonary venous return (PAPVR) occurs in 0.4 to 0.7% of postmortem examinations with abnormal return of the pulmonary veins (PVs) to the central venous circulation. PAPVR occurs more frequently on the right side. The anomalous PV connection may be into the superior vena cava, right atrium, inferior vena cava, left innominate vein or coronary sinus. PAPVR is usually found in association with other cardiac malformations, most commonly in atrial septal defect (ASD). Surgical correction is the standard treatment when PAPVR results in right heart volume overload and/or clinical symptoms [1].
Occasionally, the existence of a dual drainage connecting the PAPVR to the left atrium (LA) has been reported [2–15]. In such patients, transcatheter therapy may represent an attractive alternative to surgery. Only a few cases have been published and data on mid-term results is lacking [2–9]. We retrospectively reviewed our experience in 5 centers to describe the anomalous connection and patients' characteristics and to better clarify the indications and results of transcatheter therapy. We also present a review of the feasibility and the safety of transcatheter closure in such cases. 2. Patients and method 2.1. Study population
⁎ Corresponding author at: Cardiologie pédiatrique et congénitale, Hôpital de la TimoneEnfants, 264 rue Saint Pierre, 13385 Marseille, France. Tel.: +33 491386750; fax: +33 491385638. E-mail address: [email protected] (A. Fraisse). 0167-5273/$ – see front matter © 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.10.061
Since January 2000, we retrospectively collected all data from patients with PAPVR and an additional drainage to the LA in 5 institutions (La Timone Hospital, Marseille, France; Necker-Enfants-Malades, Paris, France; CHU Rangueil, Toulouse, France; CHU NGM St Jacques, Clermont-Ferrand, France; Université Catholique de Louvain, Bruxelles,
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Belgium). Inclusion criterion was PAPVR with an additional connection to the LA, through a normal PV or any other connecting vein. We excluded patients with previous corrective surgery of anomalous pulmonary venous return because transcatheter closure may be indicated for a vertical vein (VV) left open after surgical redirection of PV flow to the LA [16]. All the charts were reviewed to collect medical history, age at presentation and diagnosis, clinical signs at diagnosis, echocardiographic evidence for PAPVR and additional connection to the LA as well as any other abnormality, result of computed tomography (CT) scan, age at catheterization(s), technical approach, hemodynamic data as well as difficulties and complications, type and size of device used in cases with transcatheter interventions, operative reports in cases with surgical therapy, and patient's follow-up and outcome. We searched MEDLINE through PubMed and Google search engines using a combination of the terms “partial abnormal pulmonary venous return” and “interventional catheterization”. We also used in combination the terms “vertical vein”, “levoatriocardinal vein” or “left superior vena cava” with “interventional catheterization”. Literature review included any unoperated PAPVR with an additional venous connection to the LA treated by interventional catheterization.
Table 2 Characteristics of patients with partial abnormal pulmonary venous return and dual supply undergoing transcatheter therapy. References
Year
Number of cases/type
Devices used
Recto [7] Forbess [2] Lee [4] Mas [8]
2007 1998 2007 2000
1/VV 2/SV (1), VV (1) 1/SV 1/SV
AVP I/6 Coils ADO I ADO I
Kasarala [5] Gomez [6] Dähnert [3] Cullen [9] Present report
2011 2012 2007 2012
1/VV 1/VV 2/VV 1/VV 8/SV (2), VV (6)a
AVP II AVP II Coils and ADO I AVP II ADO I, AMVSD, ASO, AVP I
2.2. Statistical analysis Descriptive statistics such as mean (± SD), median (range) and frequency were calculated for each demographic and clinical characteristic whenever appropriate. Fisher's exact test was used on categorical comparisons of the 2 groups of PAPVR whereas the Wilcoxon rank sum test was performed to compare the age at presentation and at transcatheter closure. A p value b0.05 was indicative of statistical significance. Statistical analysis was performed using R version 2.15.2 for Windows Software (©The R Foundation for Statistical Computing, www.r-project.org).
Associated procedures ASD closure Occlusion SAS (coils)
Left PV stenting, occlusion SAS (2), ASD closure (2), Coa stenting
ADO, Amplatzer Duct Occluder; AMVSD, Amplatzer muscular ventricular septal defect; ASD, atrial septal defect; ASO, Amplatzer Septal Occluder; AoV, aortic valve; AVP, Amplatzer Vascular Plug; Coa, aortic coarctation; PV, pulmonary vein; SAS, systemic arterial supply; SV, scimitar vein type; VV, vertical vein type. a One failure due to restriction of the left PV during occlusion testing.
patients: the right pulmonary artery and lung were moderately hypoplastic and a systemic arterial supply to the sequestration was found.
3. Results (Tables 1 and 2, Figs. 1–4) 3.1. Baseline characteristics The study population consisted of 10 patients (6 girls) presenting at a median age of 8 (0.1 to 54) years. Diagnosis was confirmed in all the cases by thoracic CT scan and/or cardiac catheterization. Two anatomic types of PAPVR were encountered (Figs. 1–3): - Vertical vein (VV) type in 8 cases, with dual venous drainage from the upper lobe of the left lung to the innominate trunk via a large VV and to the LA via another more or less developed left PV (Figs. 1 and 2). - Scimitar vein (SV) type in 2 cases with an anomalous venous connection from the middle and lower pulmonary lobes of the right lung to the inferior vena cava below the level of the diaphragm, associated with an anomalous venous connection of the SV to the LA (Figs. 1 and 3). There was a sequestration syndrome in both
The 2 patients with SV type presented with recurrent respiratory infections and dyspnea on exertion. The diagnosis was suspected on the chest X-ray with mildly hypoplastic right lung in one case. In the other patient, the classical radiological picture with a tubular shadow along the right heart border resembling a curved Turkish sword was seen. Transthoracic echocardiography showed an abnormal vessel connecting with the inferior vena cava–right atrium junction in both cases. The precise diagnosis of a SV was confirmed by a CTscan. Among the 8 VV type cases, only one 43-year-old woman presented with clinical signs of right ventricular failure due to the PAPVR, and a VV was seen by transthoracic echocardiography. Another 54-year-old woman presented dyspnea due to left sided congestive heart failure. She was previously operated upon an aortic coarctation at 12 years of age. Her symptoms at presentation were due to a severe bicuspid
Table 1 Characteristics of the patients and intervention. Pt no.
Age at cath (years)
Symptoms
1 2
8 21
3
16
4
1
5
13
VV
6 7
23 8
VV VV
8
43
+
VV
9
55
+ (related to AoS)
VV
10
16
+
PAPVR type
mPAP (mm Hg)
Qp/Qs
Devices
12 16
N/A 1.3/1
8/6 mm ADO I/6 8 mm AMVSD
15
N/A
6/4 mm ADO I
20
1/1
10 and 6 mm AVP
ASD
20
2/1
10 mm ASO
ASD ASD
15 15
1/1 N/A
16 mm AVP
30
N/A
14 mm ASO
18
N/A
13
N/A
VV SV VV
+
Associated CHD
Coa
SV
VV
Coa (repaired), AoS
Other treatments
Occlusion SAS (coils) Coa stenting (cvrdCP stent) Occlusion SAS (coils) ASD closure (24 mm ASO) ASD closure (ASO 16 mm) Left PV stent (10 mm, Genesis PG2910P) Surgery: AoV replacement, VV ligation Surgery: VV ligation
ADO, Amplatzer Duct Occluder; AMVSD, Amplatzer muscular ventricular septal defect; ASD, atrial septal defect; ASO, Amplatzer Septal Occluder; AoS, aortic stenosis; AoV, aortic valve; AVP, Amplatzer Vascular Plug I; cath, catheterization; CHD, congenital heart disease; cvrdCP, Covered Continuous Platinum stent (NuMED Inc., Hopkinton, New York, USA); Coa, aortic coarctation; mPAP, mean pulmonary artery pressure; Pt, patient; PAPVR, partial abnormal pulmonary venous return; PV, pulmonary vein; Qp/Qs, pulmonary to systemic flow ratio; SAS, systemic arterial supply; SV, scimitar vein type; VV, vertical vein type.
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Fig. 1. Two anatomic types of partial abnormal pulmonary venous return. In A, the vertical vein (VV) type with an abnormal left upper pulmonary vein (LUPV) connecting to the innominate trunk (IT) via a VV and to the left atrium (LA) via another more or less developed pulmonary vein (PV). The connection to the IT may also include the lower PV (dotted lines). In B, the scimitar vein (SV) type with an anomalous venous connection from the middle, lower (and potentially the upper: dotted lines) right PV to the inferior vena cava (IVC), associated with a connecting vein (CV) of the SV to the LA. RA, right atrium.
aortic valve stenosis with left ventricular hypertrophy and diastolic dysfunction. In her case, the VV was also seen by transthoracic echocardiography. In the 6 other asymptomatic patients, the PAPVR was diagnosed in 4 cases by transthoracic echocardiography. Three had signs of right ventricular volume overload with an associated ostium secundum ASD in 2 cases, while one patient had a small secundum ASD without right ventricular volume overload. In these 6 patients with an echocardiographic diagnosis of the VV, a CT-scan was performed to confirm the diagnosis and precise description of the PAPVR. In the last 2 cases, the VV type PAPVR was diagnosed on a systematic thoracic CT-scan performed for another reason: to assess the morphology of an aortic coarctation in a 16 year-old female, and
because of persistent sepsis with inflammatory syndrome after a surgical appendectomy for acute appendicitis in an 8 year-old girl. 3.2. Hemodynamics and transcatheter therapy (Table 1, Figs. 2–4) The 10 patients underwent cardiac catheterization at a median age and weight of 16 (1 to 55) years and 44.5 (9 to 85) kg, under general anesthesia in 8 patients and under local anesthesia in 2 adult patients. Right heart catheterization revealed normal pulmonary artery pressure in all cases (mean: 17 ± 5.5 mm Hg) except in the 43-year-old woman with VV type and signs of right ventricular failure. She had mildly elevated pulmonary artery pressure (mean: 30 mm Hg) and a severe
Fig. 2. A: Computed tomography scan showing a vertical vein (VV) type partial abnormal pulmonary venous return with an abnormal left upper pulmonary vein (LUPV) connecting to the innominate vein via a VV. In addition, there is a small pulmonary vein (PV, arrow) that connects to the left atrium (LA). In B, a frontal angiogram of the patient in Fig. 1 showing flow through a small LUPV. The VV is occluded with an 8/6 mm Amplatzer ADO I (C). In D, a controlled computed tomography scan shows 6 months after occlusion of the VV that the small left PV has increased in size near its connection to the LA.
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Fig. 3. Angiogram showing the venous drainage from the middle and lower lobes of the right lung through a scimitar vein (SV) with a stenotic connection to the inferior vena cava. This angiogram also reveals a tortuous connecting vein joining the SV to the left atrium (A, arrow). In B, occlusion of the SV is performed with an 8 mm Amplatzer muscular ventricular septal defect.
stenosis of the PV at the junction with the LA. The pulmonary to systemic blood flow ratio was calculated in 6 cases (mean: 1.15 ± 0.41). Only one patient with an associated large ASD had a significant leftto-right shunt with a pulmonary-to-systemic flow ratio of 2/1. In 2 patients, transcatheter therapy of the PAPVR was not attempted. In one case, the parents declined transcatheter closure and surgical ligation of the VV was accomplished. In the 55-year-old patient with a severely stenotic bicuspid aortic valve, surgical aortic valve replacement was performed with concomitant ligation of the VV. These 2 patients were excluded from subsequent analysis. Transcatheter closure was attempted in 8 patients at a median age of 16 (1 to 43) years. An occlusion test was performed with a balloon in 6 cases, including the 2 SV type cases and 4 patients with a VV type. The left PV pressure did not change in 4 cases, whereas it significantly increased in 2 VV type cases (Fig. 4). In one asymptomatic patient without significant left-to-right shunt closure of the VV was not performed and a secundum ASD measuring 5 mm by transesophageal echocardiography was also left open. The other patient was the 43-year-old woman with signs of right ventricular failure and pulmonary hypertension. A left PV angiogram revealed a stenosis of the venous connection at the junction with the LA. A stent was successfully implanted and the PV pressure did not change after a new test occlusion of the VV. In 7 patients, transcatheter therapy of the PAPVR was successfully performed. The 2 patients with SV types underwent occlusion of their SV with Amplatzer
devices (AGA Medical Corporation, MN, USA) just proximal to their insertion into the inferior vena cava (Fig. 3). An anomalous systemic arterial supply was occluded with PDA coils (Cook Cardiology, Inc., Bloomington, Indiana) in both patients. In the 5 remaining VV type patients, occlusion of the VV was also performed with Amplatzer devices. In one case, occlusion of the VV with a 16/14 mm Amplatzer Duct Occluder failed, with device embolization in the left pulmonary artery. The device was successfully retrieved and the VV was closed one month later with an Amplatzer Septal Occluder device. Other associated cardiac malformations were concomitantly treated in 3 patients: an aortic coarctation was successfully stented in one teenager and a secundum ASD was percutaneously closed in 2 other cases. 3.3. Follow-up (Fig. 2D) Postcatheterization and postoperative courses were uneventful in the 9 treated patients. They were discharged home 24 h after catheterization whereas the 2 operated patients went home on postoperative days 7 and 10, respectively. Four patients were put on anti-platelet therapy after coarctation stenting (n = 1), LUPV stenting (n = 1) and ASD closure (N = 2) for 6 months. After a median follow-up of 3.1 (0.2 to 11.2) years, the 10 study patients are asymptomatic. By TTE, there is normal right ventricular volume and pulmonary artery pressure in all
Fig. 4. Pressure recorded in the left pulmonary vein before (A) and during the occlusion of the vertical vein (B) showing significant rise of the V wave (related to the pulmonary blood flow) from 10 to 15 mm Hg.
D. Luciano et al. / International Journal of Cardiology 170 (2013) 221–226 Table 3 Comparison between the 2 types of partial abnormal pulmonary venous connection with dual venous supply by Fisher's exact test or the Wilcoxon rank sum test (*). Characteristics
Age at presentation (year-old) Age at transcatheter repair (year-old) Female Symptoms at presentation Pulmonary hypertension (mean N 25 mm Hg) Significant left-to-right shunt (Qp/Qs N 1.5/1) Complication Associated procedure
VV type
SV type
Mean (±SD) or numbers (%)
Mean (±SD) or numbers (%)
22.5 (±21.9) 23.9 (±21.3)
15.9 (±22.1) 16.4 (±22)
p value
0.34* 0.3*
9 (69%) 6 (46%) 2 (15%)
2 (40%) 5 (100%) 1 (20%)
0.32 0.1 1
5 (38%)
3 (60%)
0.61
1 (8%) 5 (38%)
0 (0%) 3 (60%)
1 0.61
SD, standard deviation; SV, scimitar vein type; VV, vertical vein type.
the cases, including the untreated patient with a restrictive left PV. One patient had a CT-scan 6 months after transcatheter closure of a VV because the drainage was initially poorly developed between the VV and the LA. It appears more developed (Fig. 2D) with unrestrictive flow by pulsed and color Doppler. 3.4. World experience on transcatheter therapy for PAPVR (Tables 2 and 3) Results of the literature review of transcatheter closure in patients with PAPVR and dual supply [2–9] are presented in Table 2, including the 8 cases from the present study. In total, 18 transcatheter repairs of PAPVR were attempted in 11 females (61%), at a median age of 13.5 (1 to 65) years. The VV type PAPVR was the most frequently encountered in 13 cases (72%). In a few cases, the connection between the innominate vein and left PV was described as a persistent left superior vena cava [7] or as a levoatriocardinal vein [9]. However, there was no visible connection with the coronary sinus and the angiographic pictures were similar to the other VV type cases. In addition to our findings in the VV type, there were 2 patients in whom all the PVs from the left lung connected with the innominate vein via a VV that returned to the LA through a single left PV [5,6]. Morphologic variations in the SV cases included no systemic arterial supply in 2 cases [2,4] and all the PVs of the right lung draining into the SV in 2 patients [4,8]. Eleven patients (61%) presented with symptoms, all of whom were over 40 years of age at presentation. With the exception of the patients from the present study with a failed occlusion test (Fig. 4), all the procedures were successful with various coils and Amplatzer devices (Table 2). No complications occurred, except for the device embolization in one of our study patients. Additional transcatheter procedures were performed in 8 cases (44%), most frequently secundum ASD closure (3 cases). Comparison between the VV and SV types of PAPVR showed no difference (Table 3). Patients presenting after 40 year-old tend to have more symptoms (p = 0.056). 4. Discussion We report a series of 10 PAPVR cases with dual venous drainage to the systemic veins and to the LA. Two anatomic types are described (Figs. 1–3), the VV type being the most frequent. Percutaneous correction of the PAPVR was feasible in the majority of the cases. Similar to previous reports, transcatheter repair was easily accomplished, without complications [2–9]. Transcatheter closure of the abnormal venous connection is certainly the treatment of choice for PAPVR with dual venous supply, although surgical ligation of the SV [14] or of the VV [3] was successfully reported and also performed in two of our study patients. In addition to providing a less invasive therapeutic approach than surgery, interventional catheterization offers the possibility of performing an occlusion test. This is
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important when the connecting vein to the LA is potentially too small to receive all the redirected pulmonary venous flow after VV or SV closure. In two of our study patients, the occlusion test resulted in a significant increase of the left PV pressure (Fig. 4). One of them was untreated whereas PV–LA junction stenosis was successfully stented in the other patient before closure of the VV. Various types of occlusion devices and coils were implanted in our study patients and in previously published cases. In two of our cases and in the majority of the reports, the Amplatzer vascular plugs I and II that are specifically designed to embolize vascular structure of high flow, were successfully used [2,3,5–7,9]. Conversely in five of our study patients and in few other reported cases [2,4,8], other Amplatzer devices were used in positions they have not been designed for, like the Amplatzer Duct Occluder, Muscular Ventricular Septal Defect Occluder and Septal Occluder, respectively designed for patent ductus arteriosus, muscular ventricular septal defect and secundum ASD closure. Our choice of devices was based on size and morphology of the vein to occlude. Except for one initial failure with an Amplatzer Duct Occluder device too small to occlude a large VV, all the other devices were deployed uneventfully and all the collateral veins were closed without further complications. The incidence of PAPVR cases with dual drainage is difficult to estimate, especially in VV types since patients are often asymptomatic and may escape detection. Echocardiographic signs of right ventricular volume overload should raise suspicion, as well as an enlarged superior vena cava. The question remains whether or not asymptomatic cases without any hemodynamically significant left-to-right shunt should be corrected, even by a less invasive transcatheter approach. However, these defects share the same risk that any long standing left-to-right shunt does, since they combine drainage of PV to the right side (as do other types of PAPVR) and free drainage of the LA to the right atrium. Moreover, any connection between a systemic vein and the LA may provide pathways for right-to-left shunting as possible sources for paradoxical embolism [7,9,17]. Interestingly, two of the previously published patients with a VV type presented with stroke-like symptoms. Although there was no significant left-to-right shunt, transcatheter correction was recommended and successfully performed [7,9]. Other cases of patients experiencing neurological symptoms without patent foramen ovale on transesophageal echocardiography have been diagnosed as having venous communications between the innominate trunk and the LA by contrast echocardiography and/or CT scan [17]. In the present study, four patients of the VV type and no clinical symptoms or significant left-to-right shunt underwent transcatheter (n = 3) or surgical (n = 1) closure of the VV. PAPVRs from the left lung are usually connected to the left cardinal system, including the innominate vein and the coronary sinus. In the present study we call the connector to the innominate vein a VV. Embryologically, the VV is the left precardinal (or anterior cardinal or superior cardinal) vein [1]. Some authors name it a persistent left superior vena cava [7,11,15]. However it is now widely agreed that if such a VV does not communicate with the coronary sinus, it should not be called a persistent left superior vena cava, even though the left superior vena cava is the left superior cardinal vein that opens into the left common cardinal vein (the coronary sinus) [1]. The VV has also been termed the levoatrio or levoatrial cardinal vein [9]. As its name indicates, the levoatrial cardinal vein is a vein that communicates both with the left atrium and with the left superior cardinal vein. In other words, this is a left PV that has an abnormal persisting anastomosis with the left superior cardinal vein. It is often associated with severe pulmonary venous and left atrial obstruction caused by mitral atresia or severe stenosis. In this hemodynamic situation, the levoatrial cardinal vein helps to decompress the hypertensive pulmonary veins and left atrium. Interestingly, levoatrial cardinal vein has also been occasionally reported in association with coarctation of the aorta [18], as in two of our patients. This raises the
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question concerning whether coarctation of the aorta predisposes toward persistence of anastomoses between pulmonary and adjacent systemic veins, such as the left superior cardinal vein, resulting in a levoatrial cardinal vein. Finally, the distinction between a levoatrial cardinal vein and a coronary sinus septal defect (unroofed coronary sinus) was not made in this study. Unroofing of the coronary sinus because of a coronary sinus septal defect between the coronary sinus posteriorly and the LA anteriorly may be difficult or impossible to distinguish diagnostically (by imaging studies) from a levoatrial cardinal vein. In both anomalies the left superior cardinal vein communicates with the LA. Anatomically, the difference is that a pulmonary vein opens into the LA in a more superior or cephalad location than does a coronary sinus septal defect which is located more inferiorly or caudal, this being the relationship between the common pulmonary vein (above) and the coronary sinus (below). The size of the communication with the LA can be a diagnostically distinguishing feature. PVs openings into the LA are relatively small whereas unroofing of the coronary sinus can be total, resulting in a large opening into the LA. But coronary sinus septal defects can also be small [1,19]. Semantically in the present study, we prefer to call the connector to the innominate vein a VV, as in the majority of published cases [2,3,5,6]. Interestingly, almost one century ago, an anatomist from Edinburgh University named TB Johnston accurately described: “A rare vascular anomaly—Opening of the left upper pulmonary vein into a persistent left superior vena cava”. This is probably the first published description of this anomaly [15]. 4.1. Study limitations Several factors may have influenced the results of the present study. Besides its retrospective design, these include bias in case reports, unreported procedures, and undiagnosed additional connection to the LA. Because different devices were used for occlusion of the PAPVR, we cannot recommend any specific device for transcatheter therapy. Finally, in general, it is difficult to compare the results of the present study to those published in the literature because patient mix is heterogenous in age, indication of treatment, and PAPVR anatomy. 5. Conclusion We describe two anatomic types of PAPVR with additional connection to the LA. Patients tend to be more frequently symptomatic after 40 years of age. Transcatheter therapy can be offered as an alternative to surgery in the majority of patients. It seems indicated even in the asymptomatic VV type because of the risk of left-to-right shunt and of paradoxical embolization. Potential conflict of interest Alain Fraisse is a consultant and proctor for St Jude.
Acknowledgment We acknowledge Pascal Amedro, MD (Service de Pédiatrie 1, Hôpital Arnaud de Villeneuve, 34095 Montpellier cedex 10, France) and François Wernert, MD (cabinet de cardiologie, 20 rue Guirlande 13002, Marseille, France). References [1] Ward KE, Mullins CE. Anomalous pulmonary venous connection, pulmonary vein stenosis, and atresia of the common pulmonary vein. In: Garson A, editor. The science and practice of pediatric cardiology. 2nd ed. Williams and Wilkins; 1998. p. 1431–62. [2] Forbess LW, O'Laughlin MP, Harrison JK. Partially anomalous pulmonary venous connection: demonstration of dual drainage allowing nonsurgical correction. Catheter Cardiovasc Diagn 1998;44:330–5. [3] Dähnert I, Riede FT, Kostelka M. Partial anomalous pulmonary venous drainage of the left upper pulmonary vein. Catheter interventional treatment is sometimes possible. Clin Res Cardiol 2007;96:511–3. [4] Lee ML, Yang SC, Yang AD. Transcatheter occlusion of the isolated scimitar vein anomaly camouflaged under dual pulmonary venous drainage of the right lung by the Amplatzer ductal occluder. Int J Cardiol 2007;115: e90–3. [5] Kasarala G, Fiore A, Schowengerdt K, Jureidini S. Partial anomalous pulmonary venous return: transcatheter repair. Pediatr Cardiol 2011;32:1238–40. [6] Gomez J, Soledispa C. Redirection of anomalous venous pulmonary flow to left atrium using a vascular plug II. J Invasive Cardiol 2012;24:E96–8. [7] Recto MR, Sadlo H, Sobczyk WL. Rare case of persistent left superior vena cava to left upper pulmonary vein: pathway to paradoxical embolization and development of transient ischemic attack and subsequent occlusion with an Amplatzer vascular plug. J Invasive Cardiol 2007;19:E313–6. [8] Mas C, Goh TH, Wilkinson JL. New interventional therapeutic approach for dual drainage of the scimitar vein. Catheter Cardiovasc Interv 2000;51: 192–5. [9] Cullen EL, Breen JF, Rihal CS, Simari RD, Ammash NM. Levoatriocardinal vein with partial anomalous venous return and a bidirectional shunt. Circulation 2012;126: e174–7. [10] Odman P. A persistent left superior vena cava communicating with the left atrium and pulmonary vein. Acta Radiol 1953;40:554–60. [11] Gazzaniga AB, Matloff JM, Harken DE. Anomalous right pulmonary venous drainage into the inferior vena cava and left atrium. J Thorac Cardiovasc Surg 1969;57:251–4. [12] Pearl W. Scimitar variants. Pediatr Cardiol 1987;8:139–41. [13] Geggel RL. Scimitar syndrome associated with partial anomalous pulmonary venous connection at the supracardiac, cardiac and infracardiac levels. Pediatr Cardiol 1993;14:234–7. [14] Takeda S, Imachi T, Arimitsu K, Minami M, Hayakawa M. Two cases of scimitar variant. Chest 1994;105:292–314. [15] Johnston TB. A rare vascular anomaly—opening of the upper left pulmonary vein into a persistent left superior vena cava. J Anat Physiol 1915; 49:182–6. [16] Hares DL, Tometzki AJ, Martin R. Use of the Amplatzer vascular occluder to occlude large venous vessels in adults and children with congenital heart disease: a case series. Catheter Cardiovasc Interv 2007;69:33–9. [17] Saremi F, Vojdani E, Vorobiof G, et al. Right to left shunting through communications between the left superior intercostal vein tributaries and the left atrium: a potential cause of paradoxical embolism. Int J Cardiol Sep 2013;167(6):2867-74. [18] Bernstein HS, Moore P, Stanger P, Silverman NH. The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary-systemic connection. J Am Coll Cardiol 1995;26:995–1001. [19] Douglas YL, Jongbloed MRM, DeRuiter MC, Gittenberger-de Groot AC. Normal and abnormal development of pulmonary veins: state of the art and correlation with clinical entities. Int J Cardiol 2011;147:13–24.