Pulmonary Vein Obstruction Research Articles

Abstract 17696: Pulmonary Vein Obstruction: a European Congenital Heart Surgeons Association (ECHSA) Multicentric Study

Pulmonary vein obstruction (PVO) still has a poor prognosis and its optimal management and risk factors for pejorative outcomes remain controversial in the absence of large multicentric studies. We assessed the hypothesis that diffuse PVO and postoperative pulmonary hypertension are associated with worse outcomes while the sutureless surgical technique is associated with better outcomes. Methods: One hundred and seven patients treated for native or acquired PVO in 16 European or North-American centers (2000-2012) were included retrospectively. A specific PVO severity score (range:0-16) was developed. Endpoints were 1) PVO-related death, 2) persistence or recurrence of PVO, and 3) PV reintervention or PVO-related death. A univariate and multivariate risk analysis (logistic regression and Cox model) was performed. Mean follow-up was 34±41 months. Results: Patient characteristics, outcomes and risk analyses are summarized in Tables 1, 2 and 3 respectively. In conclusion, both native and acquired PVO still have a poor prognosis. Sutureless repair is significantly associated with lower risks of PVO recurrence and PV reoperation/PVO-related mortality in the univariate analysis. A postoperative pulmonary hypertension and a high degree of severity of the disease evaluated by a new severity score are independent risk factors for pejorative outcomes.

Abstract 17965: Echocardiographic Assessment of Surgically Placed Melody Valves in the Mitral Position in Young Children

Introduction: Mitral valve replacement (MVR) in young children is limited by lack of small prostheses, resulting in high re-intervention rates and mortality. Therefore, in 2010, we began performing MVR with modified, surgically placed, stented jugular vein grafts (Melody valve) to allow for tailoring to small annular dimensions as well as subsequent expansion in the catheterization lab. We aimed to analyze key pre- and post-operative (op) echo variables to refine surgical planning for this novel procedure. Methods: We included 17 patients who underwent surgically placed Melody MVR. The pre- and post-op echoes before discharge were retrospectively reviewed for anatomic and physiologic variables. On the pre-op echo, we performed “potential” mitral annular measurements in orthogonal planes to estimate the maximum intra-op balloon sizing. Given concern for left ventricular outflow tract obstruction (LVOTO), a ratio of the narrowest subaortic region in systole to the actual mitral annular dimension (SubA:MV) was assessed in the parasternal long axis view. Values are presented as median (range), where applicable. Results: Melody MVR was performed at 8 months of age (weight 5.2 kg) for stenosis (5), regurgitation (1), and mixed disease (11). On pre-op echo, the potential mitral measurement in the lateral dimension correlated well with the intra-op balloon size selected by the surgeon (ρ=0.58, p=0.01). The Melody valve was expanded to 1.0 times the potential lateral dimension (median 14 mm). The SubA:MV measured >0.5 in all patients. By post-op day 10 (5-35), the mean mitral gradient improved by 9 mm Hg (1-16), and right ventricular pressure was <½ systemic in 76% (vs. 12% pre-op, p<0.001). Only 2 patients had mild regurgitation and 1 had a mild perivalvar leak. The internal diameter of the Melody valve measured 3.4 mm (1-6) less than the intra-op balloon size. No patient had LVOTO >25 mm Hg or pulmonary vein obstruction. Conclusion: Intra-op balloon sizing of Melody MVR may be guided by pre-op echo indices to achieve adequate mitral valve function without significant LVOTO. The post-op Melody valve diameter was found to measure less than the intra-op balloon size, which should be considered as experience with the Melody in native, non-conduit positions evolves.

Surgical outcomes of total anomalous pulmonary venous connection repair: a 22-year experience.

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD), whose surgical repair is associated with high mortality and reoperation rates. We sought to identify predictors of early and late outcomes. Data from medical records of patients who underwent surgical repair for TAPVC from 1989 to 2012 were included. The patients were divided in two groups, according to absence or presence of associated major CHDs. Forty-six patients were included (M/F: 26/20, median age 26 days, interquartile range 15 to 59, median weight 3.350 kg, interquartile range 1800 to 4470). Anatomic types of TAPVC were: supracardiac in 48%, intracardiac in 20%, infracardiac in 20%, and mixed in 12%; TAPVC was obstructive in 33%; TAPVC was isolated in 63%, complex in 37%. Single ventricle physiology was present in 11 patients, heterotaxy in eight patients. Overall operative mortality was 19.6% (9/46): 6.9% in isolated TAPVC, 41.2% in complex type (p-value: 0.002). It was associated with low weight at intervention (<3 kg, p = 0.027), single ventricle physiology (p = 0.047), and aortic cross-clamp time >60 minutes (p = 0.097). At a median follow-up of 2.97 years (range 43 days to 22 years, 91% complete), there were nine late deaths (24.3%); 15 patients (40.5%) had major events (including late death). Multivariate analysis of event-free survival showed worse outcome in the complex group (p = 0.001). Surgical mortality and morbidity remain consistent in patients with TAPVC and associated major CHD, while the isolated type shows excellent outcomes. Pulmonary vein obstruction is a rare but highly lethal complication. doi: 10.1111/jocs.12399 (J Card Surg 2014;29:678-685).

Haemoptysis due to pulmonary venous stenosis.

Haemoptysis is a potentially life-threatening condition with the need for prompt diagnosis. In about 10-20% of all cases the bleeding source remains unexplained with the standard diagnostic approach. The aim of this article is to show the necessity of widening the diagnostic approach to haemoptysis with consideration of pulmonary venous stenosis as a possible cause of even severe haemoptysis and haemoptoe. A review of the literature was performed using the Medline/PubMed database with the terms: "pulmonary venous stenosis", "pulmonary venous infarction" and "haemoptysis". Further references from the case reports were considered. 58 case reports and case collections about patients with haemoptysis due to pulmonary venous stenosis were detected. This review gives an overview about the case reports and discusses the underlying pathophysiology and the pros and cons of different imaging techniques for the detection of pulmonary venous stenosis. Several conditions predispose to the obstruction of the mediastinal pulmonary veins. Clinical findings are unspecific and may be misleading. Pulmonary venous stenosis can be detected using several imaging techniques, yet three-dimensional magnetic resonance-angiography and three-dimensional contrast-enhanced computed tomography are the most appropriate. Pulmonary venous stenosis should be considered in patients with haemoptysis.

Modified Primary Sutureless Repair Technique of Total Anomalous Pulmonary Venous Connection

Surgical correction of total anomalous pulmonary venous connection is associated with an important risk of postrepair pulmonary vein obstruction. Primary sutureless anastomosis at the time of initial surgical repair might mitigate that risk. A simplified primary sutureless technique is described in this report.

A Surgical Lung Biopsy Case of Pulmonary Veno-occlusive Disease Associated with Limited Cutaneous Systemic Sclerosis

Pulmonary veno-occlusive disease (PVOD), a rare form of pulmonary arterial hypertension (PAH) characterized by a progressive obstruction of small pulmonary veins, is both difficult to diagnose and treat effectively. We reported a case of limited cutaneous systemic sclerosis complicated with PAH in which early definitive diagnosis of PVOD was made by surgical lung biopsy. A 53-year-old Japanese woman, with no history of smoking, was admitted to our hospital because of dry cough and progressive dyspnea. She had Raynaud’s phenomenon, gastroesophageal reflux and skin sclerosis and she was positive for anticentromere antibody. Chest high-resolution computed tomography (HRCT) revealed diffuse centrilobular ground-glass opacities and septal lines in addition to mediastinal lymph node enlargement; radiographic triad of HRCT signs of PVOD. PAH was found by right heart catheterization. Although limited cutaneous systemic sclerosis-associated PAH was diagnosed, histological findings obtained by surgical lung biopsy showed not only wall thickening of muscular arteries and arterioles but diffuse occlusion of pulmonary venules, that is, PVOD. In addition, pleuritis and limited, mild cellular interstitial pneumonia was also observed. First-line corticosteroid therapy and careful combination therapy with sildenafil and ambrisentan initially stabilized her symptoms and pulmonary hemodynamics, but she died due to cardiac arrest approximately 2.5 years after initiating PAH therapy. The need to establish a correct and early diagnosis of PVOD is important because of the impaired prognosis of these patients and their risk of developing severe pulmonary edema with specific PAH therapy. HRCT is a useful noninvasive diagnostic modality in the evaluation of suspected PVOD.

The research on operation of obstructed total anomalous pulmonary venous connection in neonates.

Objectives. Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease. This study aimed to evaluate the outcomes of TAPVC repair in neonates, controlling for anatomic subtypes and surgical techniques. Methods. Between 1997 and 2013, 88 patients (median age: 16 days) underwent repair for supracardiac (31), cardiac (18), infracardiac (36), or mixed (3) TAPVC. All the patients underwent emergency operation due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included a side-to-side anastomosis between the pulmonary venous confluence and left atrium. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Results. The early mortality rate was 2.3% (2/88 patients). The echocardiogram showed no obstruction in the pulmonary vein anastomosis, and flow rate was 1.1–1.42 m/s in the 3-year follow-up period. Conclusions. The accurate preoperative diagnosis, improved protection of heart function, use of pulmonary vein tissue to anastomose and avoid damage of the pulmonary vein, and delayed sternum closure can reduce the risk of mortality. The preoperative severity of pulmonary vein obstruction, the timing of the emergency operation, and infracardiac or mixed-type TAPVC can affect prognosis. Using our surgical technique, the TAPVC mortality among our patients was gradually reduced with remarkable results. However, careful monitoring of the patient with pulmonary vein restenosis and the timing and method of reoperation should also be given importance.

An infant with primary pulmonary vein stenosis, associated with fatal occlusion of intraparenchymal small pulmonary veins

Primary pulmonary vein stenosis (PVS) is rare within the pediatric population and its pathophysiology remains unclear, especially as to how the histopathology relates to its refractoriness to treatment. We report the case of a 4-month-old girl with primary PVS. The lesion in this patient was characterized by fatal obstruction of intraparenchymal small pulmonary veins, associated with localized stenosis at the four pulmonary veno-atrial junctions. All four localized stenoses underwent transcatheter stent implantation. Although the procedure was technically successful, her clinical status failed to improve, and she died 2 months after stenting. Histopathological examination of lung specimens showed severe luminal obstruction by marked intimal proliferation with fibrosis in the intraparenchymal small pulmonary veins, and these findings were present in every lobe. To the best of our knowledge, the histopathological findings and clinical course in this case, including the response to treatments, are extremely rare. We suggest that the histological findings of the small pulmonary veins are important in deciding the indication and appropriate timing of intervention. <Learning objective: The outcome of primary pulmonary vein stenosis has remained poor despite aggressive treatment, while the indication and appropriate timing of intervention has not been clarified. The histological findings of small pulmonary veins are important to predict the response to treatment and outcome, and early intervention might prevent the secondary progression of this disease.>.

The Natural and Surgically Modified History of Anomalous Pulmonary Veins From the Left Lung

Reconstruction of anomalous left pulmonary veins (ALPV) requires an anastomosis at a nonanatomic position, posing the potential risk of pulmonary vein obstruction (PVO). The 514 patients who were diagnosed with a pulmonary vein abnormality from 1990 to 2010 were reviewed. Thirty-eight patients (7.4%; median age, 1.4 years; interquartile range, 0.1 to 5.7 years) were identified. ALPV was diagnosed as an isolated anomaly in 23 (61%) or as part of mixed total anomalous pulmonary venous drainage in 15 (39%). Patients were divided into 3 groups (group 1: partial ALPV, treated; group 2: total ALPV, treated; or group 3: partial ALPV, untreated). Freedom from poor clinical (death/reoperation) and functional (any PVO, mean pressure gradient > 3 mm Hg) outcomes were analyzed. Repair in 30 ALPV patients (79%) was performed with direct anastomosis to the left atrium (n = 26 [68%]) or by sutureless repair (n = 4 [11%]). Two deaths occurred in group 2 (heart failure and PVO). The 4 reoperations in group 2 were prompted by PVO and occurred within 6 months of the initial repair. There was a nonsignificant trend of lower freedom from poor outcomes in group 2 (74.6%) vs group 1 (100%) at 10 years (p = 0.105). There was no difference in the incidence of any left PVO among the groups (p = 0.381). Severe left PVO did not develop in group 3 (n = 8 [21%]). Total ALPV carries a high risk of early PVO. Thus, the optimal surgical approach remains elusive. Untreated partial ALPV remained unobstructed during midterm follow-up. Therefore, surgical treatment may not be necessary in patients with partial ALPV.

An unusual complication of infective endocarditis: left ventricular outflow tract perforation

A 24-year-old female was admitted to our hospital complaining of fever, chest pain, cough and dyspnoea. Physical examination revealed tachycardia with heart rate 115 bpm, dull and decreased respiratory sounds in the lower left lung field, systolodiastolic murmur at the aortic and mesocardiac area. Chest X-ray showed an increased cardiothoracic ratio and left-sided pleural effusion. The erythrocyte sedimentation rate was found to be 64 mm/h. Echocardiography revealed moderate aortic insufficiency. Pathological flow was detected at left ventricular outflow tract (LVOT) by Doppler imaging. LV free wall was seen to be compressed partially by the haematoma surrounding the heart. Chest computed tomography (CT) showed the perforation at LVOT, partial compression of LV lateral wall, main pulmonary artery, superior vena cava, and nearly complete obstruction of left pulmonary veins (Figs. 1–3). The patient underwent emergent cardiac surgery. A pseudoaneurysm sac was detected starting from the lateral side of the aorta extending through transverse sinus and including the left anterior descending coronary artery region. Aortotomy was performed. The aortic valve was detected to be bicuspid. A perforation with a diameter of 10 × 15 mm was seen just below the aortic valve (Fig. 4). The perforation was repaired with a dacron graft, and aortic valve replacement was performed. The pathological examination revealed inflammation and vegetation at the sample taken from the perforation region, which demonstrated that the aetiology of perforation was mural infective endocarditis. Mural endocarditis is an inflammation and disruption of the nonvalvular endocardial surface of the cardiac chambers. Mural endocarditis may develop on LVOT secondary to chronic aortic regurgitation due to chronic endocardial trauma. Traumatised endocardium provides a nidus for the development of bacterial vegetation. It is well known that inflammation and vegetation may cause destructive lesions and perforation usually affecting valves, and result in acute valvular regurgitation, or may result in fistula formation. But myocardial perforation and pseudoaneurysm formation has rarely been reported. Excision of the pseudoaneurysm with LV reconstruction should be the treatment of choice in patients presenting with myocardial perforation and pseudoaneurysm formation.

Learning from the Pulmonary Veins

The purpose of this article is to review the basic embryology and anatomy of the pulmonary veins and the various imaging techniques used to evaluate the pulmonary veins, as well as the radiologic findings in diseases affecting these structures. Specific cases highlight the clinical importance of the imaging features, particularly the findings obtained with multidetector computed tomography (CT). Pulmonary vein disease can be broadly classified into congenital or acquired conditions. Congenital disease, which often goes unnoticed until patients are adults, mainly includes (a) anomalies in the number or diameter of the vessels and (b) abnormal drainage or connection with the pulmonary arterial tree. Acquired disease can be grouped into (a) stenosis and obstruction, (b) hypertension, (c) thrombosis, (d) calcifications, and (e) collateral circulation. Pulmonary vein stenosis or obstruction, which often has important clinical repercussions, is frequently a result of radiofrequency ablation complications, neoplastic infiltration, or fibrosing mediastinitis. The most common cause of pulmonary venous hypertension is chronic left ventricular failure. This condition is difficult to differentiate from veno-occlusive pulmonary disease, which requires a completely different treatment. Pulmonary vein thrombosis is a rare, potentially severe condition that can have a local or distant cause. Calcifications have been described in rheumatic mitral valve disease and chronic renal failure. Finally, the pulmonary veins can act as conduits for collateral circulation in cases of obstruction of the superior vena cava. Multidetector CT is an excellent modality for imaging evaluation of the pulmonary veins, even when the examination is not specifically tailored for their assessment.

Outcome predictors and implications for management of scimitar syndrome

Scimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome. The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic pulmonary pressure >0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). Pulmonary artery systolic pressure <0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow <1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention. Postoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. These observations may guide management decisions in patients with scimitar syndrome.

Use of Breast Implants to Fill Postinfarct Pneumonectomy Cavity in Cases of Bilateral Lung Transplantation

Infarction of the lung graft following lung transplantation (LTx) needing graft pneumonectomy and retransplantation is rare and carries a poor prognosis. It is important to maintain the volume of hemithorax after pneumonectomy until retransplantation.A young woman who received bilateral LTx for idiopathic pulmonary hypertension underwent left graft pneumonectomy owing to infarction related to obstruction of pulmonary veins. After pneumonectomy, left hemithorax was filled with silicon breast implants (1 × 500 mL and 2 × 250 mL). A Taurolidine drip was started into the left chest cavity and was maintained 24 h/d for 5 d to avoid infection of breast implants (Fig 1). After 9 weeks when suitable single lung donor became available, the left chest was opened, the breast implants were removed, and a new organ was transplanted. The patient made a rapid recovery after retransplantation and was discharged home after 4 weeks. In another woman in her mid 40s with bilateral LTx for pulmonary fibrosis, two 500-mL implants were placed following left graft pneumonectomy for pulmonary artery obstruction (Fig 2). Gas exchange from a single lung was inadequate and required extracorporeal life support, from which she could not be weaned and died after 1 month from refractory liver failure.Fig 2View Large Image Figure ViewerDownload (PPT)In these cases, graft pneumonectomy was performed within the first week of transplantation. The plan was to offer retransplantation as the patients' condition stabilized and suitable donor organs became available. It was important to preserve the space in hemithorax, because mediastinal shift, diaphragmatic elevation, and rib crowding seen after pneumonectomy would have obliterated it. The use of breast implants for filling the hemithorax preserved the space so that the retransplantation with an appropriately sized organ would be possible. Breast implants also had a tamponade effect and prevented bleeding from the hilum and chest wall after pneumonectomy. The risk of herniation of the heart through the pericardial rent made for hilar dissection of the native lung was also prevented by breast implants. Infarction of the lung graft following lung transplantation (LTx) needing graft pneumonectomy and retransplantation is rare and carries a poor prognosis. It is important to maintain the volume of hemithorax after pneumonectomy until retransplantation. A young woman who received bilateral LTx for idiopathic pulmonary hypertension underwent left graft pneumonectomy owing to infarction related to obstruction of pulmonary veins. After pneumonectomy, left hemithorax was filled with silicon breast implants (1 × 500 mL and 2 × 250 mL). A Taurolidine drip was started into the left chest cavity and was maintained 24 h/d for 5 d to avoid infection of breast implants (Fig 1). After 9 weeks when suitable single lung donor became available, the left chest was opened, the breast implants were removed, and a new organ was transplanted. The patient made a rapid recovery after retransplantation and was discharged home after 4 weeks. In another woman in her mid 40s with bilateral LTx for pulmonary fibrosis, two 500-mL implants were placed following left graft pneumonectomy for pulmonary artery obstruction (Fig 2). Gas exchange from a single lung was inadequate and required extracorporeal life support, from which she could not be weaned and died after 1 month from refractory liver failure. In these cases, graft pneumonectomy was performed within the first week of transplantation. The plan was to offer retransplantation as the patients' condition stabilized and suitable donor organs became available. It was important to preserve the space in hemithorax, because mediastinal shift, diaphragmatic elevation, and rib crowding seen after pneumonectomy would have obliterated it. The use of breast implants for filling the hemithorax preserved the space so that the retransplantation with an appropriately sized organ would be possible. Breast implants also had a tamponade effect and prevented bleeding from the hilum and chest wall after pneumonectomy. The risk of herniation of the heart through the pericardial rent made for hilar dissection of the native lung was also prevented by breast implants.

Life-threatening haemoptysis due to tracheal varices secondary to pulmonary vein obstruction

A 30-year-old man with a 3 day history of increasing breathlessness and frank haemoptysis was suspected of having pulmonary embolism and anticoagulated with heparin. Haemoptysis worsened so anticoagulation was reversed...

Complex TAPVC-Experience with Six Patients

Total anomalous pulmonary venous connection becomes a totally different subset when associated with complex congenital anomalies. The combination of two separate life-threatening congenital heart defects complicates the management of these patients. Six patients with total anomalous pulmonary venous connection associated with complex congenital heart disease were studied. There were 2 girls and 4 boys. Three of them were less than 5 kg in weight, and the other 3 were more than 5 kg in weight. Four patients had severe pulmonary arterial hypertension and 2 patients had pulmonary stenosis. Three patients had supracardiac type with a right vertical vein, one had drainage to the right atrium superior vena cava junction, one patient had supra cardiac type but split flow to both the superior vena cava and one patient had cardiac type. Three patients had double outlet right ventricle. Three patients had atrioventricular canal defect and 2 patients had preoperative pulmonary vein obstruction. All patients underwent rerouting of pulmonary veins. Concomitant procedures included intraventricular tunnel repair of ventricular septal defect and infundibular resection in double outlet right ventricle. Atrioventricular canal repair was done for Rastelli type A atrioventricular canal. Superior vena caval plasty, atrioventricular canal repair and pulmonary artery banding were done in unbalanced atrioventricular septal defect and large double outlet right ventricle. Intracardiac repair through transatrial approach was done for tetralogy of Fallot. Right ventricle-pulmonary artery conduit was done for truncus arteriosus. Single ventricle repair was done for corrected transposition of great arteries. There were 2 hospital deaths.

FROM MURAL THROMBUS TO SUPERIOR VENA CAVA SYNDROME: IS SEEING BELIEVING?

ObjectivesA 28-year-old woman presenting with a 3-month history of dyspnoea after the parturition was referred to our department for the diagnosis of mural thrombus in the left atrium. In the...

Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension

Pulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) characterized by obstruction of small pulmonary veins. Pulmonary venous involvement has been reported in pathologic assessment of patients with systemic sclerosis (SSc) presenting with precapillary PH. High-resolution computed tomography (HRCT) of the chest is a noninvasive diagnostic tool used to screen for PVOD. No HRCT data are available on SSc patients with precapillary PH. We undertook this study to evaluate the frequency and effect on prognosis of HRCT signs of PVOD in SSc patients with precapillary PH. We reviewed chest HRCT data from 26 SSc patients with precapillary PH and 28 SSc patients without pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD). The radiographic triad of HRCT signs of PVOD (lymph node enlargement [57.7% versus 3.6%], centrilobular ground-glass opacities [46.2% versus 10.7%], and septal lines [88.5% versus 7.1%]) was significantly more frequent in SSc patients with precapillary PH than in SSc patients without PAH or ILD (all P < 0.005). Indeed, 61.5% of SSc patients with precapillary PH had ≥ 2 of these signs. Cardiomegaly (P < 0.0001), pulmonary artery enlargement (P < 0.0001), and pericardial effusion (P < 0.0005) were also significantly more frequent in SSc patients with precapillary PH. Pulmonary venous involvement was histologically confirmed in 2 patients with radiographic signs of PVOD. The presence of ≥ 2 radiographic signs of PVOD was associated with the occurrence of pulmonary edema after initiation of PAH-specific therapy (in 8 of 16 patients) and with more rapid progression from diagnosis of PH to death. HRCT signs of PVOD are frequently observed in SSc patients with precapillary PH, correlated with histologic assessment, and were associated with a high risk of pulmonary edema.

Optimal Initial Palliation for Patients With Functionally Univentricular Hearts

This review will outline the optimal, initial palliation for children who are born with a functionally univentricular heart. Optimizing the initial palliation is of critical importance in this patient population to prevent potential problems such as systemic outflow and pulmonary vein obstruction that may complicate further surgical intervention. The palliative techniques that are discussed include pulmonary artery banding, modified Blalock-Taussig shunt, Damus-Kaye-Stansel procedure, modified Norwood, hybrid, and early bidirectional Glenn. Our recommendations for optimal palliation for children with a univentricular heart are based on our experience with nearly 200 patients who had either a lateral tunnel or extracardiac Fontan procedure and 130 patients who had Fontan conversion with arrhythmia surgery.

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.

Idiopathic mediastinal fibrosis with involvement of the pulmonary vessels and left main coronary artery

Idiopathic mediastinal fibrosis (IMF) is an uncommon clinical condition and the manifestations of this disease vary from a relatively benign disorder with minor symptoms to severe pulmonary vascular occlusion leading to mortality, commonly presenting a diagnostic and therapeutic challenge. Here, we present the case of a Taiwanese woman with obstruction of the left pulmonary veins and artery and stenosis of the left main coronary artery (LMCA) resulting from IMF. To our knowledge, this is the first reported case of IMF successfully treated with percutaneous balloon angioplasty with stent placement for the LMCA stenosis.