Recurrent Pulmonary Vein Stenosis Research Articles

Application of primary sutureless repair for obstructed total anomalous of pulmonary venous connection at the Vietnam National Children's Hospital

Objectives: This study evaluates the results of applying primary sutureless technique in surgical repair of total anomalous pulmonary venous connection at Children Heart Center, Vietnam National Children's Hospital. Methods: A retrospective study was conducted to evaluate the short-term outcomes of primary sutureless repair for all patients diagnosed with obstructed total anomalous venous connection (TAPVC) from July 2015 to December 2016. Results: There were 25 patients undergoing primary sutureless repair during the study period. The mean age and the mean weight are 77.00 ± 23.26 days and 3.93 ± 1.16 kg, respectively. There were 19/25 (76%) cases with preoperative respiratory failure with 14 cases (56%) requiring mechanical ventilation before surgery, of which 9 patients (36%) had life-threatening acute pulmonary edema from before emergency surgical repair. There were 17 cases admitted to the hospital with cardiogenic shock. Evaluation of transthoracic echocardiography showed 15 cases of supracardiac TAPVC, 5 cases of infracardiac TAPVC, 2 cases of intracardiac, 2 cases of mixed, and 1 case of partial TAPVC. The mean aortic cross-clamp time was 65.40 ± 23.34 minutes, the average cardiopulmonary bypass time was 119.72 ± 34.85 minutes, the mean surgery time was 200.04 ± 39.42 minutes. 9 cases underwent circulatory arrest and 7 cases underwent low-flow bypass during surgery. 3 patients died in the postoperative period, of which 2 from septic shock and multi-organ failure, 1 patient from respiratory failure after extubation due to laryngomalacia. The reoperation rate was 2/25 as a consequence of recurrent pulmonary vein stenosis. Conclusions: The primary sutureless repair for obstructed anomalous pulmonary venous connection is safe and favorable. Long-term outcomes follow-up are essentially required.

Artificial Roofing for Recurrent Pulmonary Vein Stenosis

Pulmonary vein stenosis occurs in up to one-fifth of patients after repair of total anomalous pulmonary venous connection and carries a poor prognosis. Current options for repair include patch venoplasty, endovenectomy, and the “sutureless” (pericardial well) technique. Approaches to repair vary and may include combinations of these techniques, but data suggest that the sutureless technique is associated with less restenosis.1 Yet, the sutureless technique is not a panacea: 30% of patients with pulmonary vein stenosis will undergo reoperation or die within 2 years.

Pulmonary Vein Stenosis-Evolving Surgical Management of a Challenging Disease.

Pulmonary vein stenosis (PVS) is an extremely challenging clinical problem in congenital heart disease. It has traditionally required multimodal therapy given its complex underlying pathophysiology. As with other modalities, surgical therapy has undergone tremendous evolution since the 1950s. These evolving strategies have been based upon an improved understanding of the substrates that cause PVS and recurrent vein obstruction. More recent anatomic-based surgical strategies have focused on the pulmonary vein course, and how adjacent mediastinal structures can create a fulcrum effect on the pulmonary veins as they pass from the lung parenchyma to the left atrium. The consequent angulation of pulmonary veins creates altered wall shear stress and likely serves as a nidus for recurrent PVS. Encouraging early results suggest that eliminating pulmonary vein angulation and shortening/straightening the pulmonary vein course may prove effective in surgically managing PVS.

Surveillance of a Patient Born With Tetralogy of Fallot and Total Anomalous Pulmonary Venous Return With Recurrent Pulmonary Vein Stenosis: The Role of Echocardiography to Aid in Treatment Options

Surveillance of patients with congenital heart disease is difficult in some clinical settings. This case study follows the progress of a patient, born with prenatally missed diagnoses of tetralogy of Fallot (TOF) and total anomalous pulmonary venous return (TAPVR), for over two years. The patient acquired pulmonary vein stenosis following initial TOF/TAPVR repair, which made the case an interesting longitudinal study. Multiple cardiac catheterizations for stents and balloon angioplasties of the pulmonary veins were necessary. This case study illustrates this patient’s success with treatment options such as chemotherapy medications, as well as the failure of each treatment. It was also important to note that the interval of time between each interventional treatment weighed heavily upon results of frequent echocardiograms.

Complex treatment of recurrent pulmonary vein stenosis

Pulmonary vein stenosis (PVS) in children is a rare heart disease characterized by high recurrence and mortality rates.
 Objective. We describe a case of recurrent PVS, which was treated surgically and subsequently with repeated balloon angioplasties.
 Materials and methods. Echocardiogram showed total anomalous pulmonary venous connection, mixed form. A CT scan of the chest confirmed the diagnosis. Month later after initial surgery angiography detected the presence of stenosis in all pulmonary veins.
 Results. Currently patient has moderate residual stenosis in each PV. His prognosis is considered bleak due to continuous requirement in aggressive interventions in order at least temporarily resolve pulmonary vein stenosis.
 Conclusions. Pulmonary vein stenosis still bears high recurrence, reintervention and mortality rates. Rigorous surveillance and combination of surgical and interventional treatment is warranted in this subset of patients to maintain quality of life.

Hybrid Pulmonary Vein Stenting in Infants with Refractory to Surgical Pulmonary Vein Stenosis Repair.

Pulmonary vein stenosis (PVS) is still a frustrating disease with extremely high mortality, especially in children with multiple severe PVS. Hybrid pulmonary vein stenting (HPVS) is a rescue treatment for recurrent and malignant PVS. The aim of this study is to share our successful experience with intraoperative HPVS for recurrent PVS after total anomalous pulmonary venous connection (TAPVC) repair in infant. Six patients were identified between 2013 and January 2018, who were diagnosed with recurrent PVS and underwent HPVS in the operating room. The mean age at the time of the HPVS was 10.3 ± 2.7months (range 7-14months) and the mean body weight was 7.9 ± 2.6kg (range 4.1-10.5kg). Prior pulmonary vein surgery had been performed on average 2.7 times (range 2-3) in all patients. We used a bare-metal stent (BMS) of 6-8mm diameter in 15 veins of five patients and a drug-eluting coronary stent (DES) in two veins of one patient. All patients had undergone several elective further pulmonary vein in-stent balloon dilatations or another stent insertion after HPVS. Over a mean follow-up of 17.3 ± 13.7 months (range 6-44months), all patients maintained patency of stents although two patients died due to respiratory failure not associated with PVS. HPVS is a useful treatment modality for recurrent PVS patient that could save the life and achieve longer freedom from restenosis than repetitive surgical pulmonary vein widening only. Even though the prognosis of severe multiple PVS is very poor, planned HPVS could be a good palliation in this patients group.

Recurrent Pulmonary Vein Stenosis After Pulmonary Vein Isolation

Recurrent Pulmonary Vein Stenosis After Pulmonary Vein Isolation

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

Patients 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. We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015. A 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). The 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.

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

Serum Levels After Everolimus-Stent Implantation and Paclitaxel-Balloon Angioplasty in an Infant with Recurrent Pulmonary Vein Obstruction After Repaired Total Anomalous Pulmonary Venous Connection

Everolimus-eluting stents and paclitaxel-coated balloons are used in the interventional treatment of coronary artery disease in adults to reduce the restenosis rate and in small-vessel disease. Both substances are released into the circulation. We report systemic drug exposure after implantation of one everolimus-eluting stent and dilation with one paclitaxel-coated balloon in an 8-month-old infant, which was used as an innovative therapy for recurrent pulmonary vein stenosis.

Correction of Total Anomalous Pulmonary Venous Connection of the Cardiac Type

Surgical treatment of the cardiac type of total anomalous pulmonary venous connection requires special techniques. The treatment and outcome in 17 consecutive patients who had undergone primary repairs of the cardiac type between 1965 and 1996 were reviewed. The median age was 3 months and median weight 4.2 kg. The connection was the coronary sinus in ten patients, and the right atrium in six. Interatrial communication was routinely augmented. The right atrial cavity was partitioned using a patch to direct the anomalous pulmonary veins into the left atrium through the atrial septal defect in the first 13 patients. In the last four patients, the free wall flap of the right atrium was developed as a neoseptum. There were three early postoperative deaths during the early period of conventional repair. Two patients developed residual or recurrent diffuse obstruction in the individual lobar veins; reoperation to relieve the obstruction was attempted but unsuccessful. One sudden death occurred in a patient with occasional heart block. Ten survivors have been asymptomatic during follow-up, but two incomplete heart blocks and one atrial flutter were noted among patients who underwent conventional repair. The right atrial wall flap technique was not associated with any mortality or morbidity, such as arrhythmia and recurrent pulmonary vein stenosis during 12 to 63 months of postoperative obstruction. In conclusions, a flap technique using the right atrial wall appears to be a promising method to decrease the incidence of supraventricular arrhythmias and pulmonary vein drainage obstruction following repair of the cardiac type.

Correction of total anomalous pulmonary venous connection of the cardiac type

Surgical treatment of the cardiac type of total anomalous pulmonary venous connection requires special techniques. The treatment and outcome in 17 consecutive patients who had undergone primary repairs of the cardiac type between 1965 and 1996 were reviewed. The median age was 3 months and median weight 4.2 kg. The connection was the coronary sinus in ten patients, and the right atrium in six. Interatrial communication was routinely augmented. The right atrial cavity was partitioned using a patch to direct the anomalous pulmonary veins into the left atrium through the atrial septal defect in the first 13 patients. In the last four patients, the free wall flap of the right atrium was developed as a neoseptum. There were three early postoperative deaths during the early period of conventional repair. Two patients developed residual or recurrent diffuse obstruction in the individual lobar veins; reoperation to relieve the obstruction was attempted but unsuccessful. One sudden death occurred in a patient with occasional heart block. Ten survivors have been asymptomatic during follow-up, but two incomplete heart blocks and one atrial flutter were noted among patients who underwent conventional repair. The right atrial wall flap technique was not associated with any mortality or morbidity, such as arrhythmia and recurrent pulmonary vein stenosis during 12 to 63 months of postoperative obstruction. In conclusions, a flap technique using the right atrial wall appears to be a promising method to decrease the incidence of supraventricular arrhythmias and pulmonary vein drainage obstruction following repair of the cardiac type.