A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution

2016 ◽  
Vol 27 (5) ◽  
pp. 870-876 ◽  
Author(s):  
Brian Kogon ◽  
Jan Fernandez ◽  
Subhadra Shashidharan ◽  
Kirk Kanter ◽  
Bahaaldin Alsoufi

AbstractBackgroundPatients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.MethodsWe reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.ResultsA total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).ConclusionThe surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

2007 ◽  
Vol 15 (5) ◽  
pp. 438-440 ◽  
Author(s):  
Shin Takabayashi ◽  
Hideto Shimpo ◽  
Kazuto Yokoyama ◽  
Masaki Kajimoto ◽  
Koji Onoda

An 11-month-old boy with congenital pulmonary vein stenosis, partial anomalous pulmonary venous connection, and ventricular septal defect is described. Angiocardiography demonstrated stenosis between the right upper pulmonary vein and high superior vena cava and obstruction of the right lower pulmonary vein. For pulmonary vein stenosis, we performed transverse sutured plasty for the right upper pulmonary vein, followed by right lower lobectomy. In some patients, combined management for pulmonary vein stenosis is effective.


2010 ◽  
Vol 26 (4) ◽  
pp. 440-448 ◽  
Author(s):  
Shingo Maeda ◽  
Yoshito Iesaka ◽  
Kiyoshi Otomo ◽  
Kikuya Uno ◽  
Yasutoshi Nagata ◽  
...  

2020 ◽  
Vol 48 (12) ◽  
pp. 2881-2886
Author(s):  
Heath P. Melugin ◽  
Rena F. Hale ◽  
Jun Zhou ◽  
Matthew LaPrade ◽  
Christopher Bernard ◽  
...  

Background: Femoroacetabular impingement (FAI) is a common cause of hip pain and a known risk factor for hip osteoarthritis (OA) and total hip arthroplasty (THA) at a young age. Unfortunately, little is known about the specific factors associated with an increased risk of OA. Purpose: To (1) report the overall rate of symptomatic hip OA and/or THA in patients with FAI without surgical intervention and (2) identify radiographic features and patient characteristics associated with hip OA. Study Design: Case-control study; Level of evidence, 3. Methods: A geographic database was used to identify all patients with hip pain and radiographs between 2000 and 2016. Chart review was performed to identify patients with FAI. Patient medical records were reviewed to obtain demographic information, clinical history, physical examination findings, imaging details, and treatment details. Kaplan-Meier analysis was used to determine the rate of hip OA. Univariate and multivariate proportional hazard regression models were performed to determine risk factors for OA. Results: The study included 952 patients (649 female; 303 male; 1104 total hips) with FAI. The majority of hips had mixed type (n = 785; 71.1%), 211 (19.1%) had pincer type, and 108 (9.8%) had cam type. Mean age at time of presentation was 27.6 ± 8.7 years. Mean follow-up time was 24.7 ± 12.5 years. The rate of OA was 13.5%. THA was performed in 4% of patients. Male sex, body mass index (BMI) greater than 29, and increased age were risk factors for OA (male sex: hazard ratio [HR], 2.28; P < .01; BMI >29: HR, 2.11; P < .01; per year of increased age: HR, 1.11; P < .01.). Smoking and diabetes mellitus were not significant risk factors. No radiographic morphological features were found to be significant risk factors for OA. Conclusion: At mean follow-up of 24.7 years, 14% of hips had symptomatic OA and 4% underwent THA. BMI greater than 29, male sex, and increased age at the time of presentation with hip pain were risk factors for hip OA. The cohort consisted of a large percentage of mixed-type FAI morphologies, and no specific radiographic risk factors for OA were identified.


2017 ◽  
Vol 104 (1) ◽  
pp. 182-189 ◽  
Author(s):  
David Kalfa ◽  
Emre Belli ◽  
Emile Bacha ◽  
Virginie Lambert ◽  
Duccio di Carlo ◽  
...  

2020 ◽  
Vol 23 (6) ◽  
pp. E833-E836
Author(s):  
Jae Yoon Na ◽  
Jinyoung Song ◽  
June Huh ◽  
I-Seok Kang ◽  
Ji-Hyuk Yang ◽  
...  

Background: Infracardiac obstructive total anomalous pulmonary venous return (TAPVR) has a poor outcome following surgical correction. We compared the surgical outcomes of obstructive TAPVR between non-infracardiac and infracardiac types. Methods: Among 51 patients who underwent surgical repair for obstructive TAPVR, 23 with infracardiac type and 28 with non-infracardiac type were included in this investigation. The study compared the immediate postoperative courses in the intensive care unit and long-term mortality and pulmonary vein stenosis. The risk factors for long-term survival in obstructive TAPVR also were investigated. Results: The postoperative follow-up period was 79.8 ± 81.5 months. Immediate major operative complications were observed in 22 patients (43.1%); 10 patients (19.6%) died, and eight patients (15.7%) experienced pulmonary vein stenosis during the follow-up period. The Kaplan-Meier curve showed better cumulative survival in patients with infracardiac TAPVR (P = 0.308). The significant factors for survival after surgical repair of obstructive TAPVR did not include anatomical type but instead were postoperative course of ventilator care and lengths of intensive care unit and hospital stays. Conclusion: Patients with non-infracardiac TAPVR with obstruction had a longer postoperative course and experienced more complications. Their survival rate was poorer, and postoperative pulmonary vein stenosis was more frequent in those patients compared with infracardiac TAPVR patients. However, a large-scale study is mandatory to gather more data and confirm our findings.


Children ◽  
2021 ◽  
Vol 8 (9) ◽  
pp. 726
Author(s):  
Edward Y. Lee ◽  
Sara O. Vargas ◽  
Kathy J. Jenkins ◽  
Ryan Callahan ◽  
Halley J. Park ◽  
...  

Purpose: To evaluate extravascular findings on thoracic MDCT angiography in secondary pulmonary vein stenosis (PVS) due to total anomalous pulmonary venous connection (TAPVC) repair in children. Materials and Methods: All patients aged ≤18 years with a known diagnosis of secondary PVS after TAPVC repair, confirmed by echocardiography, conventional angiography, and/or surgery, who underwent thoracic MDCT angiography studies between July 2008 and April 2021 were included. Two pediatric radiologists independently examined MDCT angiography studies for the presence of extravascular thoracic abnormalities in the lung, pleura, and mediastinum. The location and distribution of each abnormality (in relation to the location of PVS) were also evaluated. Interobserver agreement between the two independent pediatric radiology reviewers was studied using kappa statistics. Results: The study group consisted of 20 consecutive pediatric patients (17 males, 3 females) with secondary PVS due to TAPVC repair. Age ranged from 2 months to 8 years (mean, 16.1 months). In children with secondary PVS due to TAPVC repair, the characteristic extravascular thoracic MDCT angiography findings were ground-glass opacity (19/20; 95%), septal thickening (7/20; 35%), pleural thickening (17/20; 85%), and a poorly defined, mildly heterogeneously enhancing, non-calcified soft tissue mass (17/20; 85%) which followed the contours of affected pulmonary veins outside the lung. There was excellent interobserver kappa agreement between two independent reviewers for detecting extravascular abnormalities on thoracic MDCT angiography studies (k = 0.99). Conclusion: Our study characterizes the extravascular thoracic MDCT angiography findings in secondary pediatric PVS due to TAPVC repair. In the lungs and pleura, ground-glass opacity, interlobular septal thickening, and pleural thickening are common findings. Importantly, the presence of a mildly heterogeneously enhancing, non-calcified mediastinal soft tissue mass in the distribution of the PVS is a novel characteristic thoracic MDCT angiography finding seen in pediatric secondary PVS due to TAPVC repair.


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