Ann Thorac Surg 2008;85:1007-1014. doi:10.1016/j.athoracsur.2007.11.038
© 2008 The Society of Thoracic Surgeons
Original Articles: Cardiovascular
Left-Sided Partial Anomalous Pulmonary Venous Connections
Andrew W. ElBardissi, BSa,
Joseph A. Dearani, MDb,*,
Rakesh M. Suri, MD, DPhilb,
Gordon K. Danielson, MDb
a Harvard School of Public Health, Boston, Massachusetts
b Division of Cardiovascular Surgery, Mayo Clinic and Foundation, Rochester, Minnesota
Accepted for publication November 12, 2007.
* Address correspondence to Dr Dearani, Division of Cardiovascular Surgery, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905 (Email: dearani.joseph{at}mayo.edu).
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Abstract
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Background: Left-sided partial anomalous pulmonary venous connection (PAPVC) is a congenital defect where pulmonary veins from the left lung drain into the right atrium. If left untreated, PAPVC may result in severe right ventricular failure and pulmonary vascular disease. We sought to determine the effectiveness and long-term outcome after surgical correction of this anomaly.
Methods: From 1954 to 2006, 376 patients were treated at our institution for PAPVC. Of these, 27 (7.1%) patients underwent surgical repair for left-sided PAPVC. Thirteen patients were treated with a minimally invasive approach and 14 were treated with median sternotomy and cardiopulmonary bypass (CPB).
Results: Mean age at time of operation was 33 ± 18 years. Eleven patients had complete left-sided PAPVCs; patients with one anomalous vein had a significantly higher right ventricular systolic pressure (RVSP) than those with two (31 vs 42 mm Hg, p = 0.003). There was a trend toward early surgical treatment in patients with two anomalous veins (27.5 vs 34.6 years, p = 0.14). Patients who underwent off-pump correction were younger (p = 0.04), presented later in our surgical experience (p = 0.004), and had more tricuspid regurgitation (TR) (p = 0.04). Repair of left-sided PAPVC was performed by left thoracotomy without CPB in 13 patients. There were no early or late deaths. Three patients were rehospitalized for cardiovascular reasons during the follow-up period. All patients had right-sided chamber reduction at follow-up, decreased RVSP (p = 0.02), and decreased TR (p = 0.04). Patients who presented with higher grades of TR showed evidence of pulmonary vascular disease at follow-up (p = 0.017).
Conclusions: Repair of PAPVC can be completed successfully with low morbidity. Patients with left-sided PAPVC, right-sided chamber enlargement, evidence of TR, or clinical symptoms should undergo surgical repair.
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Introduction
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Partial anomalous pulmonary venous connection (PAPVC) is a congenital anomaly present in 0.4% to 0.7% of postmortem examinations [1, 2]. This rare congenital condition where one (or more), but not all, of the pulmonary veins fail(s) to connect to the left atrium usually occurs in the right lung, with only 10% of anomalous pulmonary veins originating from the left lung [3–5]. The PAPVC may go undiagnosed or undetected until late adulthood as it often presents with few clinical symptoms. When anomalous pulmonary veins are present in conjunction with an atrial septal defect (ASD), however, patients may have significant symptoms and present in early childhood. The increased severity of symptoms is likely due to the increase in left-to-right shunting that occurs when both defects coexist [6]. Because of the rarity of this condition, subtle symptoms, and difficulty of diagnosis there is a paucity of literature reporting operative technique, patient outcome, and follow-up after surgical correction.
Our surgical management of patients with left-sided PAPVC has evolved, over the past 51 years, from an invasive on-pump approach to a minimally invasive off-pump approach. The goal of this study was to comprehensively evaluate our surgical experience with this anomaly, underscoring current methods of diagnosis and operative technique. Additionally, we sought to determine survival after correction and aimed to identify prognostic indicators that may lead to poor outcomes in the follow-up period. Here, we describe our technique of surgical correction and short-term and long-term outcome of patients who had repair of isolated anomalous pulmonary venous connections originating from the left lung.
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Patients and Methods
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From March 28, 1954 to October 3, 2006, 376 patients underwent surgical correction of PAPVC at Mayo Clinic (Rochester, MN). After exclusion of patients with complex congenital heart disease, we present 27 (7.1%) consecutive patients who had isolated left-sided PAPVC and subsequently underwent surgical correction. Medical records were reviewed for patient demographics, past cardiac medical and surgical history, operative procedure, preoperative and postoperative cardiac data, early and late morbidity, and survival. The Mayo Foundation Institutional Review Board approved this study and all patients or their families gave written informed consent.
The primary endpoints were predefined and included operative mortality, postoperative complications, rehospitalization due to cardiovascular causes, and all-cause mortality. Our less invasive off-pump surgical approach is described below.
Surgical Technique
An anterolateral thoracotomy through the left fourth intercostal space is carried out with the left lung deflated and a ratcheted retractor is placed (Fig 1A). The left vertical vein is exposed from the hilum to the innominate vein and any systemic tributaries are ligated and divided. The pericardium is opened posterior to the phrenic nerve and retracted anteriorly with 2-0 silk stay sutures (Fig 1B; C). After 5,000 units of heparin are given, the left pulmonary artery is exposed and is snared or clamped. A Satinsky clamp is placed just below the insertion of the vertical vein into the innominate vein and a small bulldog clamp is placed at least 4 cm proximally on the vertical vein (Fig 2A). The vertical vein is transected and the vertical vein-innominate confluence is repaired using a 4-0 or 5-0 prolene suture (Ethicon, Somerville, NJ) (Fig 2B). A side-biting clamp is placed at the base of the left atrial appendage, the tip of which is opened and the trabeculae trimmed. The sutured connection between the vertical vein and the opened appendage is planned using either an end to end (left upper lobe to vertical vein) or an end to side (left upper plus lower lobe to vertical vein) configuration. Stay sutures are placed at the superior and inferior margins, and the back wall of the anastomosis is created using a running 5-0 prolene suture (Fig 2C). Stay sutures are removed and the anterior wall anastomosis is performed using interrupted stitches (Fig 2D). The clamp on the proximal vertical vein is removed and air is aspirated with fine needle, after which the left atrial appendage clamp and the left pulmonary artery clamps are removed in sequence (Fig 2E; F). The left lung is expanded. An invasive pressure monitoring line is used to measure the transanastomotic gradient. A gradient greater than 3 mm Hg warrants revision of the anastomosis. The pericardium is loosely approximated inferiorly but left open superiorly to prevent obstruction of the anastomosis. Two chest tubes are placed and the patient can be extubated as appropriate and sent to a cardiac surgical floor when stable.

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Fig 1. Exposure of left-sided partial anomalous pulmonary venous connections. An anterolateral thoracotomy through the left fourth intercostal space is carried out with the left lung deflated and a ratcheted retractor is placed (A). The pericardium is opened posterior to the phrenic nerve and retracted anteriorly with 2-0 silk stay sutures (B) and (C).
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Fig 2. Left-sided partial anomalous pulmonary venous connection operative technique. A Satinsky clamp is placed just below the insertion of the vertical vein into the innominate vein and a small bulldog clamp is placed at least 4 cm proximally on the vertical vein (A). The vertical vein is transected and the vertical vein-innominate confluence is repaired using 4-0 or 5-0 prolene (B). Stay sutures are placed at the superior and inferior margins, and the back wall of the anastomosis is created using a running 5-0 prolene suture (C). Stay sutures are removed and the anterior wall anastomosis is performed using interrupted stitches (D). The clamp on the proximal vertical vein is removed and air is aspirated with fine needle, after which the left atrial appendage clamp and the left pulmonary artery clamps are removed in sequence (E) and (F).
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Statistical Analysis
Demographic and other patient-related data were obtained from the Mayo Clinic (Rochester, MN) medical records. Follow-up information was obtained from subsequent clinic visits, written correspondence from local physicians, and mailed questionnaires to patients or families. Continuous variables are expressed as a mean ± standard deviation. The Student t tests were performed to compare continuous variables, whereas the Pearson
2 and Fischer exact tests were performed to analyze categorical variables. Multivariate linear regression and logistic regression analyses were used to determine associations between continuous and binary variables, respectively. Statistical significance was considered at a p value less than 0.05. Early operative mortality was defined as death occurring within 30 days of operation or at any time during the index hospitalization.
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Results
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Patient demographic characteristics and symptoms at presentation are shown in Table 1. Although the mean age at surgery was 32.5 years, a noteworthy amount of variation existed in this patient population (Fig 3). A linear regression model (Fig 4) revealed that patient age was positively associated with the year when diagnosis was made (p = 0.0017, R2 = 0.33), indicating that patients in the current surgical era predominantly consist of an older demographic. Based on univariate testing, the number of anomalous pulmonary veins (p = 0.36), symptomatology (New York Heart Association [NYHA] class, p = 0.66), presence of ASD/patent foramen ovales (PFOs) (p = 0.70), degree of tricuspid regurgitation (TR) (p = 0.68), and right ventricular systolic pressure (RVSP) (p = 0.99) were not predicted by age at presentation. Patient diagnoses were most commonly made incidentally (37%) while undergoing evaluation for other medical conditions. The majority of patients were minimally symptomatic (85% NYHA I/II) at the time of presentation. Initial diagnostic tests (Table 1) indicated that the majority of patients had evidence of an electrocardiographic abnormality (58%) and cardiomegaly (56%) on chest X-ray. Only one patient was in atrial fibrillation and all other patients were in normal sinus rhythm.
Because our surgical experience spans over 50 years, our approach to characterization of the severity of this anomaly has also evolved. Patients in our early series primarily underwent cardiac catheterization (mean year of diagnosis, 1963, n = 8), while those operated on more recently underwent both cardiac catheterization and echocardiography (mean year of diagnosis, 1996, n = 8). Our current preference includes only the use of echocardiography as a means for diagnosis (mean year of diagnosis, 2001, n = 9). Table 2
outlines the preoperative findings using these modalities. All patients diagnosed with echocardiography had evidence of right-sided enlargement, with 82% of patients having moderate or severe right-sided enlargement. Furthermore, nine (33%) patients had pulmonary hypertension (defined as a mean pulmonary artery pressure of greater than 25 mm Hg or a systolic pulmonary artery pressure of greater than 40 mm Hg) while 53% and 33% of patients who were diagnosed with echocardiography had evidence of TR and pulmonary regurgitation, respectively.
Anatomic characteristics of the anomalous pulmonary veins (APVs) are shown in Figure 5. Nineteen (70%) patients had a single APV, all of which originated from the left upper lobe, and eight (30%) patients had complete left-sided APVs. Overall, 44% (n = 12) of patients had a coexisting ASD or PFO; these included five patients with a PFO, four patients with an ostium secundum ASD, two patients with a sinus venosus ASD, and one patient with an ostium primum ASD. Other coexisting congenital anomalies included one patient with a ventricular septal defect, one patient with supravalvular pulmonary stenosis, and one patient with mitral valve cleft. The site of drainage was predominantly to the innominate vein, either directly or through a vertical vein (n = 22, 81%). Three patients with an APV of the left upper lobe had direct drainage into the right atrium and two patients with complete left-sided APVs drained into the subclavian vein.
Complete repair of left-sided APVs was performed in 13 (48%) patients without cardiopulmonary bypass (CPB) by the approach described above. Of those who required the use of CPB, mean bypass time was 57 ± 24 minutes and cross-clamp time was 30 ± 17 minutes. Patients who underwent on-pump surgical repair were older (38.8 years vs 26.7 years, p = 0.04), operated on in an earlier surgical era (1983 vs 1999, p < 0.001), and had less TR (
2 = 8.3, p = 0.04) than those who had off-pump repair. There was a trend toward earlier surgical treatment in patients with complete left-sided APVs (27.5 vs 34.6 years, p = 0.14); however, patients with complete left-sided APVs had a lower right ventricular systolic pressure than those with one (31 mm Hg vs 43 mm Hg, p < 0.05).
Concomitant operations included the following: two patients with pericardial patch augmentation of the pulmonary artery for supravalvar pulmonary stenosis; mitral valve commissurotomy for mitral valve stenosis in one; aortic valve replacement and coronary artery bypass grafting in one; ligation of persistent vena cava in one; pericardiectomy and PFO closure in one; pulmonary valvotomy in one; ASD pericardial patch closure in one; and Belsey Mark IV herniorrhaphy in one. The site of anastomosis was the left atrial appendage in 23 (85%) patients, although three patients with direct connections to the right atrium underwent redirection of the pulmonary veins by means of a baffle through an ASD, and one patient who required posterolateral thoracotomy for Belsey Mark IV herniorrhaphy had an anomalous left upper pulmonary vein anastomosed to the left inferior pulmonary vein. Mean anastomotic gradient using a monitoring line at the time of operation was 2 mm Hg (range, 0 to 5 mm Hg). There were no operative mortalities. Early nonfatal morbidity included atrial arrhythmias in two patients; one was successfully treated with digoxin and one required cardioversion. One patient had postpericardiotomy syndrome and one patient had an episode of sinus tachycardia postoperatively that remitted spontaneously. One patient had a postoperative hemothorax requiring chest tube placement.
Late follow-up was available in 24 patients (89%) at a mean of 9.1 ± 9 years and 23 (96%) patients were followed with echocardiography (Table 3). All patients were alive at follow-up. The vast majority of patients continued to be minimally symptomatic at follow-up (NYHA I/II, n = 22 92%). There was a significant reduction in the size of the right chambers, with only 8% of patients having moderate to severe enlargement versus 82% preoperatively (p < 0.05). There was also a reduction in grade of TR (p = 0.04) and right ventricular systolic pressure (31 mm Hg vs 40 mm Hg, p = 0.02). Linear regression indicated that grade of preoperative TR was associated with increased right ventricular pressure at the time of follow-up (R2 = 0.71, p = 0.008); however, age at surgical intervention (p = 0.533), number of anomalous veins (p = 0.25), or presence of an ASD/PFO (p = 0.17) was not associated with RVSP at follow-up.
Morbidity
Three patients were rehospitalized in the follow-up period due to cardiovascular reasons (Fig 6). One patient had marked volume overload of right atrial and right ventricle (RV) with mild TR and normal pulmonary artery pressures at the time of presentation. Postoperative echocardiography demonstrated a 4 mm Hg anastomotic gradient with significant residual right heart enlargement. An echocardiogram in the follow-up period revealed severe right-sided enlargement with mildly decreased RV function and moderate TR. This patient was hospitalized for left sided pneumonias and symptoms of congestive heart failure (CHF) and eventually underwent tricuspid valve replacement.
Two patients were readmitted shortly after discharge for management of CHF; one patient required mediastinal exploration for recurrent bilateral pleural effusions and the second patient was aggressively diuresed with no additional complications. One patient had episodic atrial flutter in the follow-up period.
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Comment
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The prevalence of PAPVC is between 0.4% to 0.7% [1, 2], with left-sided PAPVCs occurring in 10% to 18.2% of all reported PAPVC cases [4]. While most patients with PAPVC are asymptomatic, the natural history dictates that if a significant left-to-right shunt exists, patients may develop irreversible pulmonary hypertension, pulmonary vascular obstructive disease, or right ventricular failure [7–10]. Previously, the indication for surgical treatment of PAPVC mimicked that of ASDs, with treatment recommended for patients with a pulmonary flow to systemic flow ratio (Qp/Qs) of greater than 1.5 [11–14]. Patients in early reported series were more symptomatic compared with the patients presented here, and likely experienced irreversible cardiac deterioration at the time of surgical correction. In this series, 52% of the patients were asymptomatic (NYHA I) and PAPVC was discovered incidentally in 37% during evaluation for other medical conditions. The early detection of most cases of PAPVC in this series can be attributed to diagnosis by echocardiography, which has contributed significantly to early intervention of patients with this congenital anomaly and has since replaced cardiac catheterization as the gold standard [4]. Patients in the current surgical era who are undergoing correction of this defect consist of patients who were previously undiagnosed and are now being discovered after evaluation with echocardiography, thus explaining the age variation appreciated in this analysis. Prior to this study, the largest single reported series of left-sided PAPVCs by Van Meter and colleagues [11] described 13 patients; thus making this study the largest cohort of patients with left-sided PAPVC while demonstrating that correction can be performed with excellent surgical results in the short and long term.
The strong association between ASDs and PAPVCs (>75%) has been well-described in the literature [15]. Based on this association and the ability to diagnose PAPVC with echocardiography efficiently [4], all patients who are found to have an ASD (especially with out-of-proportion dilation of the right heart chambers) should be investigated for PAs. There have been numerous reports of patients who have undergone device closure in the cardiac catheterization laboratory and were subsequently found to have PAPVC after progression of symptoms [16, 17]; two patients in this study presented in a similar manner. Aggressive investigation of PAPVCs with echocardiography cannot be overemphasized in an era where ASD closures are routinely performed in the cardiac catheterization laboratory.
The surgical approach of anterolateral thoracotomy without the use of CPB is advantageous for a number of reasons, including cosmesis [18, 19] and avoidance of CPB risks [20, 21]. Although our results were excellent in patients who underwent either procedure, we strongly favor the off-pump technique when possible. An obvious limiting factor is the presence of concurrent cardiac anomalies (most commonly ASD) that require repair. However, we do not believe the presence of an ASD is a contraindication to this approach because many ASDs and PFOs have minimal hemodynamic significance [22]. This finding is confirmed by our results, which found no association between ASDs/PFOs and right ventricular systolic pressure. When left-sided APVs are found to coexist with an ASD, a multidisciplinary approach is appropriate to evaluate the best possible treatment strategy. Off-pump repair of PAPVC in patients with hemodynamically insignificant ASD/PFOs is certainly an option. Additionally, ASD closure in the catheterization laboratory followed by operative repair is also a suitable strategy and should be strongly considered in patients who have ASDs that are amenable to device closure.
Many have found that older patients with PAPVCs have increased right ventricular strain and symptoms of CHF [23]. Although the degree of left-to-right shunting is theoretically associated with symptomatology, studies have found that pulmonary vascular disease may still occur in patients with one PAPVC [7]; thus the number of PAPVCs or concurrent ASD/PFOs should not be the sole indication for operative therapy. Importantly, we found that patients who were found to have a high grade of TR at time of presentation demonstrated evidence of pulmonary vascular disease in the follow-up period even after surgical correction. We did not, however, appreciate an association between age, presence of ASDs/PFOs, number of APVs, and pulmonary vascular disease at follow-up. Although our evaluation was not powered appropriately to detect these differences, we do believe that TR may be a surrogate of the early stages of right ventricular overload and should be considered a marker that pulmonary vascular disease has occurred.
Although prior indications for operative intervention were based on Qp:Qs (an acceptable surrogate for the severity of left-to-right shunt) cardiac catheterization has since been phased out as the primary modality of diagnosis. Our results indicate that timing of surgical intervention should be considered early in the clinical course in order to prevent irreversible right ventricular failure and pulmonary vascular disease. In light of the excellent surgical outcome and unpredictability of this congenital anomaly, we recommend that all patients who are found to have PAPVCs with evidence of right ventricular dilation, mild to moderate TR, or early stages of pulmonary vascular disease, be offered surgical treatment in order to prevent the progression of right ventricular failure and irreversible pulmonary vascular disease.
Previous studies [5, 11, 24] have indicated that surgical correction is associated with a number of complications including atrial fibrillation, complete heart block, cardiac arrest, and pulmonary venous obstruction. The improvement in surgical technique and the ability to perform left-sided corrections off-pump have facilitated our ability to perform these procedures with minimal morbidity and mortality as demonstrated in this study. The results of this study, however, emphasize the importance of a gradient free anastomosis, which was the likely cause of recurrent left-sided pneumonias and reoperation in one patient in this series. Our group routinely performs intraoperative echocardiograms as well as measurement of anastomotic gradients with a monitoring line, and will take down and reconstruct the anastomosis in gradients greater than 4 mm Hg. Patients who underwent this procedure early in this series were not discharged on medications unless the postoperative course was complicated by arrhythmias. As our experience with this anomaly has evolved, we have instituted anticoagulation for three months postoperatively to achieve a goal international normalized ratio of 2.0; this empiric therapy has been started in light of echocardiographic images of patients who have not received anticoagulation demonstrating opacities at the anastomotic site, which raised the concern of thrombosis. Although no patients experienced an embolic event or pulmonary venous obstruction, we believe this finding is due to the low-flow state of the pulmonary venous circulation in conjunction with suture lines that are a substrate for thrombosis, thereby warranting intermediary anticoagulation. All patients are followed up at six months with an electrocardiogram and echocardiogram to evaluate RA/RV size, patency of the anastomosis, and valvular function.
Left-sided PAPVC can be repaired with minimal morbidity and mortality when the operation is performed correctly. Because the natural history of this disorder includes a wide variety of complications, including arrhythmias and pulmonary obstructive disease [11], we recommend that surgical correction be performed when patients are symptomatic or show evidence of right-sided overload. The results of this study indicate that both on-pump and off-pump surgical procedures result in excellent long-term outcome when performed correctly.
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