Clinical characteristics of pulmonary vein stenosis in children / 中华实用儿科临床杂志

Objective:To analyze the clinical characteristics of pulmonary vein stenosis (PVS) in children, and to explore its treatment and prognostic factors.Methods:The clinical data of 19 children with PVS treated in Beijing Children′s Hospital, Capital Medical University from October 2016 to March 2022 were analyzed retrospectively.There were 16 males and 3 females.The median age at diagnosis was (2.81±1.95) years.A descriptive analysis of clinical characteristics of children was made.Results:Of the 19 children, 14 cases (73.7%) had primary PVS and 5 cases (26.3%) had secondary PVS after surgery of anomalous pulmonary venous connection (APVC). Thirteen children (68.4%) had hemoptysis.In the hemoptysis children, 5 cases had life-threatening massive hemoptysis, and 11 cases (57.9%) had a history of recurrent respiratory tract infection or pneumonia.Other manifestations of hemoptysis included failure to thrive (6 cases), cyanosis (5 cases), and dyspnea (3 cases). Complications were pulmonary hypertension (6 cases) and right heart failure (3 cases). There were 16 cases (84.2%) of unilateral PVS and 3 cases of bilateral PVS.Interlobular septal thickening, grid shadow and ground glass opacities were found on CT of all PVS cases.Ten cases underwent surgery, and 2 cases of them received angioplasty, but restenosis occurred in both of them.Eight children underwent pulmonary lobectomy, and their clinical symptoms were all relieved after operation.Nine patients were treated conservatively, and 3 cases of them died of bilateral PVS secondary to APVC.The remaining 6 alive cases still had intermittent clinical symptoms during follow-up.Conclusions:Hemoptysis and recurrent respiratory tract infection are the main clinical manifestations of PVS in children, and life-threatening massive hemoptysis can occur.Lobectomy is an effective treatment for unilateral PVS.The prognosis of secondary PVS after APVC is poorer and its mortality is higher, compared with primary PVS.

Angioplastia con balón de venas pulmonares por estenosis tras ablación percutánea de fibrilación auricular / Balloon angioplasty of pulmonary vein stenosis after percutaneous ablation of atrial fibrillation

Resumen Se presenta el caso de un hombre de 31 años, con historia de fibrilación auricular paroxística, a quien se realizó exitosamente una ablación por radiofrecuencia de venas pulmonares guiada por un sistema de mapeo tridimensional EnSite™. Tres meses después del procedimiento presentó hemoptisis y dolor torácico de características pleuríticas, motivo por el cual se le realizó una angiotomografía computarizada coronaria que evidenció una estenosis grave de la vena pulmonar superior izquierda y una estenosis moderada de la vena inferior izquierda. El paciente fue sometido a angioplastia con balón, con la cual se normalizó la perfusión pulmonar.

Abstract We report the case of a 31-year-old male patient with a history of paroxysmal atrial fibrillation, who underwent a successful radiofrequency pulmonary vein ablation using EnSite™ three-dimensional mapping system. Three months after the procedure, patient presented with hemoptysis and pleuritic chest pain. A coronary computed tomography angiography was performed, which showed a severe left superior pulmonary vein stenosis and a moderate left inferior pulmonary vein stenosis. A balloon angioplasty was performed with subsequent restoration of pulmonary perfusion.

Estenosis congénita de venas pulmonares y malformación vascular broncopulmonar / Congenital pulmonary vein stenosis and bronchopulmonary vascular malformation

Resumen El objetivo es mostrar el diagnóstico y la evolución de una paciente con estenosis de venas pulmonares y secuestro pulmonar. Se trata de una niña de 1 año de edad, con bronconeumonías de repetición, acrocianosis, 2R intenso, cardiomegalia, hipertensión venocapilar pulmonar, con diagnóstico clínico de comunicación interauricular. El ecocardiograma mostró estenosis de venas pulmonares izquierdas. El cateterismo cardiaco detectó fístulas arteriovenosas en la región apical del pulmón derecho. La imagen de resonancia magnética y la angiografía mostraron un vaso arterial aberrante paralelo a la aorta abdominal y con flujo dirigido al lóbulo pulmonar derecho. La angiotomografía reportó confluencia de las venas pulmonares del lado derecho. Se realizó lobectomía derecha. La paciente falleció en el posoperatorio debido a una hemorragia masiva pulmonar. Esta paciente es la primera descrita en la literatura con estenosis de venas pulmonares congénita asociada a secuestro pulmonar. La ecocardiografía es el estudio diagnóstico ideal inicial en los pacientes con estenosis congénita de venas pulmonares.

Abstract The objective is demonstrate the diagnostic process and evolution of a patient with a diagnosis of congenital pulmonary vein stenosis and broncho-pulmonary vascular malformation. One year old female patient with repeated bronchopneumonia, acrocyanosis, split S2, cardiomegaly, pulmonary hypertension, with a clinical diagnosis of atrial septal defect. The echocardiogram demonstrated left sided vein pulmonary stenosis. The cardiac catheterization demonstrated arterial-venous fistulas apical on the right lung. Magnetic Resonance image and angiography showed an aberrant arterial vessel parallel to the abdominal aorta which flow the right pulmonary lobe. The cardiac tomography angiography reported confluence of right-sided pulmonary veins. A lobectomy is performed. Patient died in post-operative due to massive pulmonary hemorrhaging. This is the first patient mentioned in written literature with pulmonary vein stenosis associated with pulmonary sequestration, with normal venous connection. Echocardiography represents the specific standard study ideal for initial diagnostic for patients with pulmonary vein stenosis.

Analysis of risk factors for surgical treatment of congenital pulmonary venous stenosis combined with congenital heart disease / 中国胸心血管外科临床杂志

@#Objective    To explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease. Methods    From May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed. Results    Preoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥ 1.2 m/s at discharge was an independent risk factor for mortality and restenosis. Conclusion    The prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.

Surgery for pulmonary venous stenosis after total anomalous pulmonary venous connection repair: Midterm results of 9 patients / 中国胸心血管外科临床杂志

@#Objective    To review our experience of reoperations for pulmonary venous stenosis (PVS) after total anomalous pulmonary venous connection (TAPVC) repair for the past decade in Fuwai Hospital. Methods    Nine patients underwent reoperation for PVS between 2009 and 2019 in Fuwai Hospital, including 4 males and 5 females with an average age of 5.10±5.00 years. The patients were divided into a sutureless group (n=3) and a non-sutureless group (n=6). Clinical data were reviewed and analyzed. Results    For primary TAPVC type, 4 patients were supracardiac, 2 patients were cardiac, 1 patient was infracardiac, and 2 patients were mixed-type anomaly. The median cardiopulmonary bypass time was 95 (63, 208) min, aortic clamping time was 58 (30, 110) min, ICU stay was 24 (24, 2 136) h. Early hospital death occured in 1 (11.1%) patient. One (11.1%) patient with single ventricle physiology had hospital comorbidity, who underwent hemofitration therapy. The follow-up time was 11.9 (2.2, 18.0) months, during which 1 patient died of restenosis of pulmonary vein and another patient died of stroke. No statistically significant difference was found between the sutureless group and non-sutureless group in postoperative or follow-up results (P>0.05). Conclusion    Surgery is effective for treatment of PVS after repair of TAPVC, yet with a realatively high morbidity and mortality. The advantage of sutureless repair over conventional repair for this particular group of patients is yet to be verified.

Comparative analysis of the treatment of partial anomalous pulmonary vein connection by double-patch method or Warden Technique / 中华胸心血管外科杂志

Objective:To compare the surgical and long-term follow-up results of partial anomalous pulmonary vein connection treated by double-patch method or Warden Technique.Methods:There were 33 cases of right pulmonary vein connected with the superior vena cava from May 2010 to May 2019 in our center treated by double-patch method or Warden technique. 21 cases were treated by double-patch method and 12 cases by Warden technique. Echocardiography and electrocardiogram were followed up regularly to observe the occurrence of arrhythmia, superior vena cava stenosis and pulmonary vein stenosis postoperatively.Results:All patients were discharged uneventfully, and were followed up for 1~8 years. In double-patch group, 2 cases with arrhythmia, 1 of whom was junctional arrhythmia which was automatically converted to sinus rhythm 1 day after surgery. The other had an early second degree atrioventricular block after surgery, and sinus rhythm was restored 3 days later with temporary pacemaker. 1 case had superior vena cava stenosis by echocardiography(PD 8 mmHg). No arrhythmia was found in long-term follow-up in Warden group. 2 cases had superior vena cava stenosis by echocardiography(PD 6 mmHg). Right pulmonary vein stenosis(PD 8 mmHg) was found in 1 case by echocardiography, no obvious aggravation was found in long-term follow-up.Conclusion:The double-patch method and Warden Technique are both safe and effective in the treatment of partial anomalous pulmonary venous connection.

Impact factors of in-stent restenosis following successful stent implantation for acquired severe pulmonary vein stenosis post atrial fibrillation ablation / 中华心血管病杂志

Objective: To investigate the influencing factors of in-stent restenosis (ISR) following successful stent implantation in patients with ablation-associated severe pulmonary vein stenosis (PVS) who undergo atrial fibrillation ablation. Methods: Data of patients who underwent pulmonary vein angiography to confirm PVS after radiofrequency ablation for atrial fibrillation (AF) and received pulmonary vein stenting at Shanghai Chest Hospital from March 2010 to December 2017 were retrospectively analysed. All patients were followed up for a long period of time (pulmonary vein contract-enhanced CT within 6 to 12 months after operation was performed, and pulmonary angiography was performed if CT indicated stenosis>50%). The incidence of ISR was recorded. According to angiography, the patients were divided into ISR group and non-ISR group. The clinical and intraoperative imaging characteristics and interventional data were compared between the two groups. Logistic regression was used to analyse the influencing factors of ISR. Results: A total of 47 patients ((47.1±12.2) years old) were enrolled in this study, including 28 males(59.6%). There were 19 cases in ISR group and 28 cases in non-ISR group. Compared with the non-ISR group, the ISR group received more pulmonary vein isolation ((2.8±0.9) vs. (1.8±1.3), P=0.02), and the interval between last ablation and stenting was longer ((19.4±9.6) vs. (13.0±12.4), P=0.03). The incidence of ISR in patients with stent diameter≤8 mm was significantly higher than those with stent diameter>8 mm (33.3%(20/60) vs. 8.1%(3/37), P<0.01). Logistic regression analysis found that the number of radiofrequency ablation>1 (OR=2.1, 95%CI 1.3-3.9, P=0.02), and the time from the last ablation to stent placement>12 months (OR=1.5, 95%CI 1.1-2.5, P=0.03), reference diameter of stenosed distal vessel (OR=0.7, 95%CI 0.5-0.9, P=0.04), post procedural minimal luminal diameter (OR=0.4, 95%CI 0.2-0.8, P=0.02) and stent diameter (OR=0.6, 95%CI 0.3-0.9, P=0.03) were independent factors of ISR. Conclusions: The greater number of radiofrequency ablations and the longer time from the last ablation to stent placement increase the risk of ISR. The larger reference diameter of the stenosed distal vessel, stent diameter and post procedural minimal luminal diameter are the protective factors of ISR.

Implantación de stent en drenaje venoso anómalo supracardiaco obstructivo / Stent implantation in obstructive supracardiac anomalous drainage

Resumen El drenaje venoso anómalo pulmonar total representa un grupo de defectos cardíacos congénitos con baja incidencia pero alto potencial de complicaciones. Se expone el caso de una lactante menor de bajo peso al nacer, con drenaje venoso anómalo pulmonar total obstructivo, programada para implante de stent en vena vertical y posterior cirugía correctiva, evidenciando obstrucción residual mínima en la llegada de la vena pulmonar derecha y reducido tamaño auricular izquierdo, con prolongada estancia en cuidado intensivo secundaria a dificultad para el destete de la ventilación mecánica debido a edema venocapilar. Este caso demostró que la intervención endovascular es una opción en neonatos de alto riesgo con drenaje venoso anómalo pulmonar total obstructivo como parte de la estrategia de estabilización cardiovascular preoperatoria; la cirugía correctiva debe considerarse una vez las condiciones clínicas lo permitan, con el objetivo de minimizar la morbilidad asociada al compromiso de la distensibilidad de las cavidades izquierdas.

Abstract Total anomalous pulmonary venous return is a group of congenital cardiac defects. They have a low incidence but a high potential for complication. The case is presented of a young infant of low birthweight, with total obstructive anomalous pulmonary venous return, scheduled for a stent implant in a vertical vein, and subsequent corrective surgey. This showed evidence of a minimal residual obstruction on reaching the right pulmonary vein, and a reduced left atrial size. She had a long stay in intensive care secondary to difficulty in weaning off mechanical ventilation due to the presence of venous-capillary edema. This case demonstrates that endovascualr intervention is an option in high risk infants with a total obstructive anomalous pulmonary venous return as part of the strategy of pre-operative cardiovascular stabilisation. Corrective surgery should be considered when the clinical conditions permit it, with the aim of minimising the morbidity associated with the compromise of the distensibility of the left cavities.

A piglet model of pulmonary vein stenosis / 中国胸心血管外科临床杂志

@#Objective    Pulmonary vein banding was used to establish a piglet model of pulmonary vein stenosis. We investigated the pathomorphological alterations of pulmonary veins in the model and compared it with the vascular tissue of recurrent stenosis after total anomalous pulmonary venous connection (TAPVC). Methods    Ten pigs of 6 weeks old were selected and randomly divided into 2 groups: 5 in a sham operation group and 5 in a pulmonary vein banding group. The operation had two stages, in which thoracotomies through intercostal space were done respectively on both sides. Biocompatible materials were applied around the pulmonary veins in the experimental group. The same method was used in the sham group. But the pulmonary veins were not banded. Six weeks after the operation, the pulmonary veins of the animals were harvested for hematoxylin-eosin staining and immunofluorescence staining to observe the pathological alterations of pulmonary veins. The proliferative tissues of patients with recurrent stenosis after TAPVC repair were collected and observed by hematoxylin-eosin staining and immunofluorescence staining. Results    Both the sham operation group and the pulmonary vein banding group survived. But the pulmonary vein banding group had obvious clinical manifestations of pulmonary venous stenosis. Compared with the sham group, the pulmonary vein banding group showed intimal hyperplasia, decreased expression of endothelial marker and increased expression of mesenchymal markers, and co-expression of endothelial and mesenchymal markers in intimal cells. Human pathology also showed intimal hyperplasia and co-expression of endothelial and mesenchymal markers in intimal cells. Conclusion    The surgical pulmonary vein stenosis in piglets shows intimal hyperplasia and myofibroblasts, which was consistent with clinical pathology.

Feasibility and efficacy of percutaneous pulmonary vein stenting for the treatment of patients with severe pulmonary vein stenosis due to fibrosing mediastinitis / 中华心血管病杂志

Objective@#To evaluate the feasibility and safety percutaneous pulmonary vein intervention in patients with severe pulmonary vein stenosis (PVS) caused by fibrosing mediastinitis(FM).@*Methods@#This retrospective analysis included 5 FM patients (2 male, 3 female, 54-77 years old) confirmed by clinical presentation and chest computed tomography (CT) scan from January to June 2018 who were from Gansu Provincial Hospital and Shanghai Chest Hospital. CT pulmonary angiography (CTPA) further revealed severe PVS caused by fibrotic tissue compression in mediastinum. After selective pulmonary vein angiography, gradually balloon angioplasty was used to expand the pulmonary vein and then stents were implanted in the pre-dilated stenotic pulmonary veins. Evaluation of therapeutic effect was made at 6 months after the procedure.@*Results@#All of 11 serious compression PVS were treated with stent implantation (diameter: 7-10 mm, length: 17-27 mm). After stenting, degree of pulmonary vein stenosis decreased from (83±16)% to (12±4)% (P<0.01). The minimal diameter of the stenotic pulmonary vein was significantly increased from (0.8±0.5)mm to (7.5±0.8)mm (P<0.01). Trans-stenotic gradient decreased from (27.0±15.1)mmHg (1 mmHg=0.133 kPa) to (2.50±0.58)mmHg (P<0.05). Mean pulmonary pressure measured by cardiac catheter decreased from (45.0±9.0)mmHg to (38.7±8.4)mmHg (P<0.05). One patient experienced cardiac arrest due to vagal nerve reflex during big sizing balloon stent dilation and recovered after cardiopulmonary resuscitation. There were no other serious procedure related complications. During the follow-up, severe stenosis at end of proximal stent was evidenced in 1 patient due to fibrotic compression, and another patient developed in-stent thrombosis due to discontinuation of prescribed anticoagulant.@*Conclusion@#Percutaneous intervention for severe pulmonary vein stenosis caused by FM is feasible and safe, and can improve hemodynamic caused by the compression of mediastinal vascular structures in these carefully selected patients.

Lung Infarction due to Pulmonary Vein Stenosis after Ablation Therapy for Atrial Fibrillation Misdiagnosed as Organizing Pneumonia: Sequential Changes on CT in Two Cases

Pulmonary vein (PV) stenosis is a complication of ablation therapy for arrhythmias. We report two cases with chronic lung parenchymal abnormalities showing no improvement and waxing and waning features, which were initially diagnosed as nonspecific pneumonias, and finally confirmed as PV stenosis. When a patient presents for nonspecific respiratory symptoms without evidence of infection after ablation therapy and image findings show chronic and repetitive parenchymal abnormalities confined in localized portion, the possibility of PV stenosis should be considered.

An Unusual Cause of an Uncommon Condition: Pulmonary Vein Stenosis.

Pulmonary vein stenosis in adults is historically a rare condition, but is becoming a recognised complication, albeit an uncommon one, of radiofrequency ablation around the pulmonary veins for treatment of atrial fibrillation. It may also be due to infiltrating mediastinal processes such as neoplasm or sarcoidosis. In this case report, a 55-year old man underwent resection of a mediastinal phaeochromocytoma involving the left atrial wall and the right inferior pulmonary vein. One year later he subsequently presented with increasing dyspnoea and atypical chest pain. Transthoracic echocardiogram showed severe pulmonary hypertension, right ventricular dilatation and dysfunction. Transesophageal echocardiogram demonstrated severe bilateral pulmonary vein stenosis with peak/mean gradients across the left pulmonary veins of about 25/20mmHg. The diagnosis was also confirmed on CT pulmonary angiography with 3D reconstruction. Open pulmonary vein stenting was planned but unfortunately the patient died suddenly before the procedure. Pulmonary vein stenosis is an uncommon but serious condition and may present with signs and symptoms indistinguishable from other conditions and may easily be missed. Clinicians should have a high index of suspicion when patients present with unexplained respiratory symptoms, especially in the context of catheter ablation or mediastinal processes such as neoplasm. Transesophageal echocardiography played an indispensible part in the correct diagnosis in our patient.

Tratamiento de estenosis sintomática de venas pulmonares secundaria a ablación por radiofrecuencia

El aislamiento de las venas pulmonares mediante la aplicación de radiofrecuencia es un tratamiento efectivo de la fibrilación auricular. Una de las complicaciones potenciales y de mayor compromiso clínico de esta técnica invasiva es la estenosis de una o varias venas pulmonares. Esta complicación puede ser tratada mediante angioplastia con o sin colocación de stent, logrando una adecuada mejoría clínica, aunque con un alto índice de recurrencia por re-estenosis.

Isolation of the pulmonary veins by applying radiofrequency is an effective treatment for atrial fibrillation. One of the potential complications with higher clinical compromise utilizing this invasive technique is the occurrence of stenosis of one or more pulmonary veins. This complication can be treated by angioplasty with or without stent implantation, with an adequate clinical improvement, but with a high rate of restenosis.

Evaluation of Post-operative Complication in Patients With Total Anomalous Pulmonary Venous Connection by Echocardiography / 中国循环杂志

Objective: To evaluate the post-operative complication of pulmonary vein stenosis in patients with total anomalous pulmonary venous connection (TAPVC) by echocardiography. Methods: A total of 66 TAPVC pediatric patients with surgical repair in our hospital were retrospectively studied. The peri-operative and mid-term post-operative echocardiography was conducted to evaluate the complication of pulmonary vein stenosis. Results: The peri-operative and mid-term post-operative echocardiography presented the increased left ventricular diastolic end diameter (LVEDd) and decreased right ventricular diameter in all 66 patients, P0.05. Conclusion: Echocardiography may monitor the operative effect of TAPVC, the occurrence of post-operative pulmonary vein stenosis could be found by examining right ventricular diameter and pulmonary artery pressure in pediatric patients.

Estenosis de venas pulmonares post ablación por radiofrecuencia / Pulmonary vein stenosis after radio frequency ablation

Es importante estar atento ante la aparición de síntomas respiratorios luego de la realización de un procedimiento de ablación por radiofrecuencia en el tratamiento de la fibrilación auricular, pues la estenosis de venas pulmonares (EVP) tiene una incidencia de entre 1 y 3% y puede aparecer hasta en los dos años posteriores al procedimiento1. Presentamos el caso de un paciente de 41 años de sexo masculino, que ingresó por un cuadro de hemoptisis y toracodinia de tres semanas de evolución, con antecedente de ablación por radiofrecuencia 6 meses antes de la admisión. La angiotomografía no evidenció tromboembolismo pulmonar (TEP) y la angiorresonancia detectó hipoperfusión deI lóbulo superior del pulmón izquierdo (LSI). Debido a los antecedentes de ablación se solicitó angiotomografía de venas pulmonares, que evidenció estenosis de la vena del LSI. Se realizó estudio hemodinámico con dilatación y colocación de stent.

Physicians should be alert to the occurrence of respiratory symptoms after radio frequency ablation for the treatment of atrial fibrillation. Pulmonary veins stenosis could appear with an incidence of between 1and 3% during the two years following the procedure. We present the case of a 41year-old-male patient admitted with a three weeks old hemoptysis and thoracodinia and a prior history of a radiofrecuency ablation procedure performed six months earlier. The angiotomography was not compatible with the diagnosis of pulmonary embolism and the angio-MRI detected hypoperfusion of the left upper pulmonary lobe. Consequently pulmonary veins angiotomography was requested, showing upper pulmonary lobe vein stenosis. An hemodynamic study with vein expansion and stent placement was successfully performed.

Recurrent Pulmonary Venous Stenosis after Repair of Mixed-Type Total Anomalous Pulmonary Venous Connection / 日本心臓血管外科学会雑誌

A 10-day-old male neonate underwent repair of mixed-type total anomalous pulmonary venous connection. The left upper pulmonary vein connected to the left innominate vein by way of a vertical vein. The other veins converged to form a common pulmonary vein and drained to the coronary sinus. As the common pulmonary vein was not stenotic, normal coronary sinus unroofing was undertaken and the postoperative course was uneventful. Five months later pulmonary vein stenosis (PVS) occurred at the junction of the common pulmonary vein and coronary sinus. At reoperation the common pulmonary vein was deeply incised to the point near the pulmonary venous orifice, and the stenotic tissue was resected. Although he was discharged from the hospital on the 10th postoperative day, PVS recurred at age 9 months and a second reoperation was undertaken. This time, the common pulmonary vein was excised and the anterior wall of each pulmonary vein was incised to drain independently and directly to the left atrium without causing turbulence. The left upper pulmonary vein was anastomosed to the left atrial appendage. Pulmonary angiography 18 months after the second reoperation revealed the pulmonary venous pathway to be nonstenotic.

Bilateral Congenital Pulmonary Vein Stenosis with a Normal Connection

Congenital pulmonary vein stenosis (CPVS) with an anatomically normal connection is a rare cardiac malformation. This cardiac anomaly usually is accompanied by other cardiac abnormalities. Bilateral CPVS is a more severe form of a CPVS and it usually leads to progressive pulmonary hypertension and death if it is not treated. Here, we report a patient with a history of cough, tachypnea and hemoptysis and suspected CPVS due to an abnormal thoracic roentgenogram with dilated right pulmonary arteries and pulmonary cornus. The two-dimensional and color Doppler echocardiography demonstrated three stenosed pulmonary veins connected to the left atrium. However, the fourth vessel could not be visualized. There were no other cardiac malformations associated with the CPVS. The Technetium-99m macro-aggregate lung perfusion scan showed absent or diminished perfusion to the affected lobes of the lungs. In addition, the chest computed tomography with angiogram and cardiac catheterization confirmed the findings of the echocardiogram.

A Case of Congenital Pulmonary Vein Stenosis Diagnosed in an Infant with Recurrent Hemoptysis / 소아알레르기및호흡기학회지

Congenital pulmonary vein stenosis is a rare cardiac malformation with a poor prognosis and is not easy to diagnose, because symptoms emerge in infants accompanying progressively worsening pulmonary hypertension. A nine-month-old infant visited our hospital due to recurrent respiratory infections and hemoptysis. He was diagnosed with congenital pulmonary vein stenosis by cardiac catheterization. Cardiac catheterization findings revealed that two pulmonary veins were stenotic and one pulmonary vein was occluded. Pulmonary artery catheterization showed the evidence of severe pulmonary hypertension with a mean pulmonary artery pressure of 55 mmHg and a pulmonary artery wedge pressure of 30 mmHg. Due to the aggravation of pulmonary hypertension symptoms, we performed sutureless pericardial marsupialization to reduce the obstruction and the restenosis, but he died a week. In cases of infants with recurrent hemoptysis, we should rule out congenital pulmonary vein stenosis.

Postoperative Pulmonary Vein Stenosis (PVS) in Patients with TAPVR / 대한흉부외과학회지

BACKGROUND: Despite recent advances in surgical technique and perioperative care of total anomalous pulmonary venous return (TAPVR), post-repair pulmonary vein stenosis (PVS) remains as a serious complication. We thought that the most important factors of TAPVR repair to prevent PVS were good exposure, proper alignment, and sufficient stoma size. We analyzed our experience retrospectively. MATERIAL AND METHOD: Between Jan. 1995 and Feb. 2005, we studied 74 patients diagnosed with TAPVR suitable for biventricular repair. Supra-cardiac type (n=41, 55.4%) was the most common. Mean CPB time, ACC time, and TCA (40.5%, 30/74) time were 92.1+/-25.9 min, 39.1+/-10.6 min, and 30.2+/-10.7 min, respectively. Mean follow-up duration was 41.4+/-29.1 months and follow-up was possible in all patients. RESULT: The median age and body weight at operation were 28.5 days (0~478 days) and 3.4 kg (1.4~9 kg). Early mortality was 4.1% (3/74). Causes of death were pulmonary hypertensive crisis, sepsis, and sudden death. There was PR-PVS in 2 patients (early: 1, late: 1). Both patients were cardiac type TAPVR drained to coronary sinus. Re-operations were done but only one patient survived. Cumulative survival rate in 5 year and percent freedom from PVS were 94.5+/-2.7% and 97.2+/-2.0%, respectively. CONCLUSION: There was no PVS in patients who underwent extra-cardiac anatomosis between LA and CPVC. Therefore it could be said that our principle might be effective in preventing PR-PVS in patients suitable two-ventricle.

Congenital Pulmonary Vein Stenosis Manifested by Severe Cyanosis in Infancy / 소아과

Congenital pulmonary vein stenosis(CPVS) with anatomically normal connection, a rare anomaly, usually leads to progressive pulmonary hypertension, cardiac failure in infancy, and death if untreated. Most are combined with other anomalies, particularly left to right shunt lesions. Very often, the detection of CPVS is overlooked on the initial cardiac echocardiogram, because it may be mild in its severity initially, but progresses over time. CPVS shows the turbulence at color Doppler echocardiogram and a pulsed-wave Doppler signal of >1.6 m/sec with loss of phase. We experienced a case showing a small sized secundum atrial septal defect and mild turbulence at the right pulmonary vein on the initial color Doppler echocardiogram, and at follow-up, severe cyanosis, pulmonary hypertension, right heart failure, and reopened ductus with bidirectional shunt. Complete examination of echocariogram must be warranted at the initial stage and follow-up, in order not to miss CPVS.