Pulmonary Venous Stenosis Research Articles

Surgical management of an aneurysm of the left atrial appendage to prevent potential sequelae

An aneurysm of the left atrial appendage is an extremely rare anomaly that is commonly associated with supraventricular arrhythmia, compression of the coronary arteries, intracardiac thrombus and pulmonary venous stenosis. This condition may be caused by congenital dysplasia of the musculi pectinati and is usually diagnosed in the second to fourth decades of life. We report the surgical management of an asymptomatic 9-year old girl with this anomaly. She was referred to us because of abnormal chest X-ray findings, and investigation revealed an aneurysm of the left atrial appendage. As this condition may have potentially fatal complications, the aneurysm was completely resected under cardiac arrest with cardiopulmonary bypass to prevent recurrence and thrombus formation. We suggest that resection of an aneurysm of the left atrial appendage under cardiac arrest with cardiopulmonary bypass is a reasonable treatment option to prevent potential complications, particularly in children.

Primary sutureless repair for simple total anomalous pulmonary venous connection with a risk of postoperative pulmonary venous stenosis

Late mortality following surgical repair of total anomalous pulmonary venous connection (TAPVC) is often associated with pulmonary venous stenosis. We describe here two successful cases of primary sutureless repair for simple TAPVC in patients who had a potential risk of postoperative pulmonary venous stenosis. A 10-day-old neonate with mixed-type TAPVC and a 30-day-old infant with supracardiac TAPVC underwent primary sutureless repair with our modification. In the early follow-up, both patients are now doing well and have no signs of pulmonary venous stenosis. The sutureless repair can be applied as a primary surgical option to prevent postoperative pulmonary venous stenosis in selective patients with simple TAPVC.

Long-term results of treatments for functional single ventricle associated with extracardiac type total anomalous pulmonary venous connection

Surgical outcomes of patients with functional single ventricle have improved, though those for patients whose condition is complicated by extracardiac type total anomalous pulmonary venous connection (TAPVC) remain poor. We retrospectively reviewed our 21 years of surgical experiences with this challenging group. From 1990 to 2010, 48 consecutive patients with functional single ventricle complicated by extracardiac TAPVC (26 males, 46 with right atrial isomerism) underwent initial surgical palliation at our centre. The median age and body weight at surgery were 69 days and 3.5 kg, respectively. The type of TAPVC was supracardiac in 31 patients, infracardiac in 14 and mixed type in 3. TAPVC was repaired in 25 patients before bidirectional Glenn (BDG) and 18 at BDG, while it remained in 3 patients. Since 2007, stent implantation for obstructive drainage veins for patients with preoperative pulmonary venous obstruction and sutureless marsupialization for relief of postoperative pulmonary venous stenosis (PVS) have been initiated. The mean follow-up period was 4.2 ± 5.1 years. The overall survival rates at 1, 3 and 5 years after the initial surgical intervention were 58.3, 41.1 and 31.3%, respectively. Sixteen patients achieved the Fontan operation (33.3%). The freedom from postoperative PVS rates at 1 and 3 years after repair was 68.7 and 63.4%, respectively. Univariate analysis detected that infracardiac TAPVC (P = 0.036), coexisting major aortopulmonary collaterals (P = 0.017), and TAPVC repair before BDG (P = 0.036) all reduced survival, and multivariable analysis indicated the repair of TAPVC before BDG as the only risk factor (P = 0.032). Whereas the occurrence of postoperative PVS did not reduce survival, which had a significant negative impact on achieving the Fontan operation (P = 0.008). The cumulative survival rate did not improve by surgical era. Surgical outcomes of patients with functional single ventricle undergoing the repair of extracardiac TAPVC in the neonatal period due to obstruction of the venous drainage pathway remain poor. Stent implantation for obstructive drainage veins to delay the timing of surgical correction and sutureless marsupialization as relief of postoperative PVS are expected to improve the late outcomes; however, the effect is still limited.

Sutureless technique for recurrent pulmonary vein stenosis after pericardial patchplasty in an adult

Postoperatively, posterior leaflet tethering tended to be ameliorated, just as was the anterior leaflet tethering. AML, Anterior mitral leaflet; PML, posterior mitral leaflet; LA, left atrium; LV, left ventricular; LVDd, left ventricular diastolic diameter; LVEF, left ventricular ejection fraction;MR,mitral regurgitation; NS, not significant; PA, pulmonary artery; PG, pressure gradient. Surgical Techniques

Primary correction of total anomalous pulmonary venous return with a modified sutureless technique

The objective was to evaluate primary sutureless repair of total anomalous pulmonary venous return (TAPVR) in neonates using a modified technique that minimizes hypothermia and circulatory arrest times. From 2009 to 2011, seven consecutive patients underwent primary sutureless repair for the treatment of TAPVR, by which the prepared posterior pericardium was sutured to an opening in the left atrium. Three patients had the obstructed infracardiac type, and four patients had the unobstructed supracardiac type of TAPVR. Moderate hypothermia was used in all patients with a median temperature of 28°C (26-32). Circulatory arrest was not used except for the opening of the collector, which lasted between 3 and 5 min. The connecting vein was ligated in all seven patients (five during repair and two early postoperatively). The follow-up was 100% complete, with a median duration of 652 (range 370-1023) days. There was no operative mortality and no late death. No patient required reoperation. Postoperative echocardiography showed unobstructed pulmonary venous flow in all patients. Recurrent pulmonary venous stenosis was not seen during the follow-up in any patient. The sutureless technique is an effective technique with potential advantages even for the primary correction of TAPVR. With the described technique, the need for circulatory arrest is substantially reduced. Not handling the pulmonary venous collector by avoiding a direct anastomosis may contribute to better compliance, better growth and the absence of subsequent stenosis.

Pulmonary Venous Stenosis in a Premature Infant with Bronchopulmonary Dysplasia: Clinical and Autopsy Findings of these Newly Associated Entities

Pulmonary venous stenosis is rare and is most commonly found in association with cardiac malformations. Recent studies have associated pulmonary venous stenosis with prematurity, especially with bronchopulmonary dysplasia, although no such case has been documented at autopsy. We report the case of a 26-week-gestation infant who required ventilation at birth and who, among other complications, developed chronic lung disease of prematurity by the age of 3 months. Imaging showed suprasystemic right-sided pressures and pulmonary venous stenosis. Despite aggressive management of respiratory status and surgical marsupialization of stenoses, the infant expired after 3 weeks. At autopsy, 3 of 4 pulmonary veins showed a fibrous ridge obstructing atrial ostia with otherwise normal anatomy. The lungs showed bronchopulmonary dysplasia, pulmonary hypertensive vascular changes, and features of venous obstruction. Pulmonary venous stenosis and bronchopulmonary dysplasia in premature infants may be pathogenetically related. Coincidence of these diseases is likely underrecognized, and careful cardiac examination in these patients is warranted.

Single-patch, 2-patch, and caval division techniques for repair of partial anomalous pulmonary venous connections: Does it matter?

We reviewed our experience with the single-patch, 2-patch, and caval division techniques for repair of a partial anomalous pulmonary venous connection. From 1990 to 2009, 124 patients (65 male patients; 52%) were identified. The single-patch, two-patch, and caval division technique was used in 60 (49%), 24 (19%), and 40 (32%) patients, respectively. The median age was 34.5 years (range, 1-76). Of the 124 patients, 99 (80%) had an atrial septal defect. There was no early mortality. Survival at 5, 10, and 15 years was 99%, 95%, and 82%, respectively. One patient (3%) in the caval division group required early reoperation for superior vena caval obstruction. All patients, exept for 3, had regained sinus rhythm at discharge. Late superior vena caval obstruction developed in 3 patients (7.5%) in the caval division group, 3 (5%) in the single-patch group, and 1 (4%) in the 2-patch group (Р = .5). Two patients underwent reoperation for late pulmonary vein obstruction: 1 (2%) in the single-patch group and 1 (4%) in the 2-patch group (P = .08). A permanent pacemaker was required in 3 patients (3%). Surgical treatment of partial anomalous pulmonary venous connections is associated with excellent outcomes. The overall incidence of late superior vena caval or pulmonary vein stenosis is low. Although not significant, the 2-patch technique might be associated with a greater incidence of sinus node dysfunction and late pulmonary venous stenosis. The late development of superior vena caval obstruction is a concern with all techniques, necessitating close follow-up.

Histopathological effects and evolution of transvenous -radiation applications in right and left atria: an animal study

β-radiation is a novel potential energy source for the creation of myocardial lesions. While the feasibility of delivering β-radiation via a deflectable transvenous catheter has been described, dose effects and the time course of histopathological changes have not been previously assessed. The purpose of this study was to characterize pathological aspects of cardiac lesions induced by β-radiation in an animal model at various stages of evolution and in response to different dose exposures. Nine dogs and one pig were studied. The cavotricuspid isthmus, antrum of pulmonary veins (PVs), and mitral isthmus were irradiated (25, 50, 75, or 100 Gy) with strontium-yttrium-90, delivered via a deflectable catheter (cavotricuspid isthmus and mitral isthmus) or a double-loop catheter (antrum of PVs). Eighteen lesions were created. Animals were sacrificed at 2 weeks, 1 month, 3 months, or 6 months. Lesions were processed for morphometric histopathological analyses. Over the first month, lesions were characterized by inflammation, haemorrhage, and myocyte necrosis. Thereafter, fibrotic replacement was predominant. Transmurality of lesions was observed in 50% of cases, with no dose-response effect (P = 0.976). Surface fibrin thrombus was present in 50% of cases and was essentially limited to lesions assessed within the first month. No neighbouring injury or pulmonary venous stenosis was observed. Atrial lesions created by β-radiation are characterized by an inflammatory phase with surface fibrin thrombosis during the first month and replacement fibrosis thereafter. No appreciable dose-response effect was noted within the 25-100 Gy range tested.

Successful stenting of anastomotic stenosis of the left pulmonary artery after single lung transplantation

To the Editors: Lung transplantation is an established therapy for a variety of end-stage lung diseases. Successful transplantation improves prognosis and quality of life in most recipients. In the current setting where lung donors are scarce, single-lung transplantation allows for more extensive utilisation of the limited donor organ pool [1]. Although forced expiratory volume in 1 s (FEV1) recovery is lower and the risk of bronchiolitis obliterans syndrome is higher, single lung transplant recipients still have comparable exercise tolerance and quality-of-life scores when compared to bilateral lung transplant recipients [2]. Fortunately, vascular anastomotic stenoses are an uncommon event following lung transplantation. There are two types of vascular complications: either pulmonary arterial stenosis or pulmonary venous stenosis. The structures affected determine the clinical manifestations: arterial obstruction leads to pulmonary ischaemia and infarction, and venous obstruction leads to pulmonary oedema. The diagnosis should be considered in the presence of unexplained exertional hypoxaemia and persistent pulmonary hypertension. The diagnosis can be confirmed with computed tomography pulmonary angiogram (CTPA) and pulmonary angiography. As the complication is rare there are no clear treatment algorithms. In the very early phase (1 week), surgical treatment of the stenosis is the preferred option but may still carry a poor prognosis. However, later the options are broader …

A 50-Year-Old Woman With a History of Atrial Fibrillation Presents With Acute Dyspnea and Pleuritic Chest Pain

A 50-Year-Old Woman With a History of Atrial Fibrillation Presents With Acute Dyspnea and Pleuritic Chest Pain

Total anomalous pulmonary venous connection: Results of surgical repair of 100 patients at a single institution

Surgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome. Our cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990-2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0-4 years), and the median weight was 3.5 kg (range, 1.3-15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy. There were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P < .01) and 9 late deaths (6 in the biventricular group [7%] and 3 in the single-ventricle group [18%], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1%), cardiac (1/15; 6.7%), infracardiac (3/23; 13.0%), and mixed (5/10; 50%). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27%) and in 7 patients in the single-ventricle group (41%; P = .25). Mortality was 9 of 29 (31%) in those with pulmonary venous obstruction and 12 of 71 (17%) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5%; 11 in the single-ventricle group, 64.7%). Mean deep hypothermic circulatory arrest time was 31.4 +/- 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4-753 days). Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.

The Recent Surgical Result of Total Anomalous Pulmonary Venous Return

Background and ObjectivesRecent surgical results from total anomalous pulmonary venous return (TAPVR) are reported to have improved; however, mortality and morbidity are still high in the univentricles. This review was performed in order to demonstrate surgical results from TAPVR for recent years in a single institute.Subjects and MethodsA total of 53 patients with TAPVR underwent surgery between January 2000 and December 2008. Preoperative anatomy and hemodynamics were evaluated by 2-dimensional echocardiogram. We reviewed medical records on preoperative management, age, and body weight at time of surgery, postoperative mortality, and pulmonary venous obstruction.ResultsThe study included 36 biventricle patients and 17 univentricle patients. Median age and weight at time of surgery were 26 days and 3.5 kg, respectively. During the mean follow up period of 48.2 months, a total of 11 deaths and 8 early deaths occurred. Postoperative pulmonary venous stenosis occurred in 8 patients. Among 36 biventricle patients, mortality occurred in 1 patient and postoperative pulmonary venous stenosis occurred in 6 patients. No statistical significances were observed between postoperative pulmonary venous stenosis and other factors. Among 17 univentricule patients, mortality was 10, including 7 early deaths, and postoperative pulmonary venous stenosis occurred in 2 patients. Combined palliations were modified Blalock-Taussig (BT) shunts in 7, pulmonary artery banding (PAB) in 3, bidirectional cavopulmonary shunt in 5, and the Fontan procedure in 1. The highest mortality was recorded in patients with a modified BT shunt (85.7%) and next in patients with PAB (66.6%). Mortality was not related to anatomy of TAPVR, nor preoperative obstruction, but with significant age and weight at time of surgery.ConclusionDespite improvement in operative results from TAPVR, surgery in univentricles is highly risky, especially with low weight and early age, with concomitant palliation.

Ｉａ＋ＩＩａ型総肺静脈還流異常症術後に発生した肺静脈狭窄の１治験例

Ｉａ＋ＩＩａ型総肺静脈還流異常症術後に発生した肺静脈狭窄の１治験例

Functional single ventricle with extracardiac total anomalous pulmonary venous connection☆

The purpose of this study is to evaluate the surgical repair of functional single ventricle and extracardiac total anomalous pulmonary venous connection (TAPVC). Between January 1998 and December 2007, 26 consecutive patients underwent surgical repair of extracardiac TAPVC. Their characteristics were as follows: median age, 34 (range 0-744) days; median weight 3.2 (range 2.0-9.6) kg; supracardiac TAPVC, 11 patients; infracardiac, 5; mixed, 10; right atrial isomerism, 24; pulmonary atresia, 16; and obstructed TAPVC, 17. Concomitant procedures included systemic-to-pulmonary shunt in 9 patients, pulmonary artery banding in 5, ventricle-to-pulmonary artery shunt in 1, Norwood procedure in 1, bidirectional Glenn in 9, and Fontan procedure in 1. The overall survival after the repair of TAPVC was 58% (95% confidence interval [CI], 39-77%) and 54% (95% CI, 34-73%) at 1 and 5 years, respectively. Of the 14 survivors (supracardiac, 9; infracardiac, 4; and mixed, 1), 12 underwent Fontan completion; 1, bidirectional Glenn; and 1 is awaiting bidirectional Glenn. Anastomotic stenosis did not occur, but recurrent pulmonary venous ostial stenosis (PVS) was observed in nine patients. Freedom from recurrent PVS was 56% (95% CI, 34-78%) at both 1 and 5 years. Reoperation for recurrent PVS was performed in six patients; of these patients, two underwent Fontan completion, but three with bilateral and multiple PVS declined. By Cox multivariate regression analysis, mixed TAPVC (p=0.001, hazard ratio, 13.4; 95% CI, 2.8-64.4) was a risk factor for mortality, and atrioventricular valve regurgitation, which required surgical intervention at the palliative stage (p=0.024, hazard ratio, 23.4; 95% CI, 1.5-363.4) was a risk factor for recurrent PVS. The mid-term results of the surgical repair of functional single ventricle with supracardiac or infracardiac TAPVC are acceptable. The surgical treatment of patients with mixed TAPVC and with severe atrioventricular valve regurgitation is not promising, but can be improved.

Congenital Pulmonary Venous Stenosis Combined with Valvular Aortic Stenosis: A Very Rare Case

Congenital pulmonary venous stenosis is a rare condition leads to progressive pulmonary hypertension and heart failure. It may be isolated or associated with other cardiac malformations. The condition ranges from hypoplasia of individual pulmonary veins to constriction of the intima of the venoatrial junction. We present a case of congenital PVS involving 4 pulmonary veins combined with severe valvular and subvalvular aortic stenoses.

Massive systemic-to-pulmonary collateral arteries in the setting of a cavopulmonary shunt and pulmonary venous stenosis

Massive systemic-to-pulmonary collateral arteries in the setting of a cavopulmonary shunt and pulmonary venous stenosis

Unilateral Pulmonary Edema After Pulmonary Embolism in a Bilateral Lung Transplant Patient

We report a case of unilateral pulmonary edema due to the decompensation of an asymptomatic ipsilateral pulmonary venous stenosis by a contralateral pulmonary embolism. Emergency surgery included pulmonary embolectomy and refashioning of the stenotic pulmonary venous anastomosis.

Massive systemic-to-pulmonary collateral arteries in the setting of a cavopulmonary shunt and pulmonary venous stenosis

We report a patient in whom a cavopulmonary anastomosis had been constructed, along with repair of anomalous pulmonary venous drainage. Left-sided pulmonary venous obstruction led to redistribution of the flow to the right lung. The reversal of flow in the left pulmonary artery was accentuated by flow through collateral arteries feeding the left lung. Within 14 months, the collateral flow increased six-fold, resulting in a doubling of the cardiac output.

P32.05: Prenatal echocardiography of total anomalous pulmonary venous connection (TAPVC) : isolated or accompanied by pulmonary venous stenosis

P32.05: Prenatal echocardiography of total anomalous pulmonary venous connection (TAPVC) : isolated or accompanied by pulmonary venous stenosis

Intracardiac echocardiography in electrophysiology

Intracardiac echocardiography (ICE) broadens the spectrum of echocardiographic techniques. Modern 10F sector echocardiographic catheters introduced into the right atrium allow high quality imaging of all cardiac structures, including pulse and continuous wave Doppler and/or color Doppler. The main indication for ICE appears to be monitoring of catheter ablation of complex arrhythmic substrates such atrial fibrillation, postincisional tachycardias and ventricular tachycardias. The other important role of ICE is the early diagnosis and prevention of complications during ablation procedures. These include those occurring during transseptal catheterization, damage to cardiac structures, left atrial thrombus formation, pulmonary venous stenosis, esophageal injury and pericardial effusion.