Decompression of the thoracic duct: A novel transcatheter approach

In patients with total cavopulmonary connections, elevated central venous pressures (CVP) have detrimental effects on the lymphatic system causing an imbalance in fluid production and drainage of the interstitium. This combination may result in life‐threatening lymphatic complications including plastic bronchitis (PB), protein losing enteropathy (PLE), chylothorax, and ascites. While embolization of the abnormal lymphatics has greatly improved outcomes from these complications, alternative treatment strategies have been proposed that would result in improved lymphatic drainage while leaving the lymphatic system intact. We report two novel transcatheter approaches for thoracic duct (TD) decompression in two patients who developed PLE after completion of the Fontan procedure as part of staged palliation for congenital heart disease. In addition, one patient had severe concurrent PB. In both patients, a connection was created between a left superior vena cava (LSVC) to the left atrium allowing for a nonsurgical method to decompress the TD. This procedure resulted in significant clinical and laboratory improvement of both patients' PLE and other symptoms of lymphatic dysfunction.     

Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient: Evolving Understanding and Emerging Therapies

Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are rare but potentially devastating complications of the Fontan circulation. PB occurs in ～4% of Fontan patients, typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing, fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia, lymphopenia, hypogammaglobulinemia, and elevated fecal alpha-1 antitrypsin 1. These disorders have similar pathophysiology involving disruption of the lymphatic system resulting from elevated central venous pressure combined with elevated lymphatic production and inflammation, resulting in lymphatic drainage into low-pressure circuits such as the airways (PB) and duodenum (PLE). Our understanding of these disorders has greatly improved over the past decade as a result of advances in imaging of the lymphatic system through magnetic resonance lymphangiography and early success with lymphatic interventions including lymphatic embolisation, thoracic duct embolisation, and percutaneous thoracic duct decompression. Both PB and PLE require a multidisciplinary approach that addresses and optimises residual hemodynamic lesions through catheter-based intervention, lowers central venous pressure through medical therapy, minimises symptoms, and targets abnormal lymphatic perfusion when symptoms persist. This review summarises the pathophysiology of these disorders and the current evidence base regarding management, proposes treatment algorithms, and identifies future research opportunities. Key considerations regarding the development of a lymphatic intervention program are also highlighted.     

Effect of increased systemic venous pressure on thoracic duct and peripheral lymph flow in dogs

In congestive heart failure, lymph flow from the cannulated thoracic duct is greatly increased. However, there has been scant data on lymph flow in the intact lymphatic system with systemic circulatory congestion. In the present study, thoracic duct and peripheral lymph flow were qualitatively determined using heated cross-thermocouples in seven mongrel dogs. Central venous pressure was raised artificially by infusing large volumes of crystalloid solution equivalent to a maximum of 30% of body weight. Although both thoracic duct and peripheral lymph flow increased with an intact (closed) lymphatic system, the increase was notably less than with a transected (opened) cervical thoracic duct. With systemic circulatory congestion, cannulated thoracic duct lymph flow circumvents a major lymph impediment to lymph flow (i.e. high central venous pressure) and therefore considerably overestimates in vivo central lymph flow in this condition.     

Sarcoidosis: A cause of innominate vein obstruction and massive pleural effusion

Obstruction of the major venous channels from the upper body is rarely due to benign disorders. We report a 53-year-old man with a ten year history of sarcoidosis who developed acute symptomatic left innominate vein obstruction and massive left-sided pleural effusions. Left neck exploration revealed obstruction of the innominate vein and thoracic duct by the matted sarcoid lymph nodes. Symptoms of left innominate vein obstruction and the massive effusion subsided shortly after lymphadenectomy. In view of the frequency of sarcoid cervical lymphadenopathy, this disease should be considered in the differential diagnosis of benign causes of symptomatic obstruction of the venous drainage of the upper part of the body.     

Sarcoidosis: A cause of innominate vein obstruction and massive pleural effusion

Obstruction of the major venous channels from the upper body is rarely due to benign disorders. We report a 53-year-old man with a ten year history of sarcoidosis who developed acute symptomatic left innominate vein obstruction and massive left-sided pleural effusions. Left neck exploration revealed obstruction of the innominate vein and thoracic duct by the matted sarcoid lymph nodes. Symptoms of left innominate vein obstruction and the massive effusion subsided shortly after lymphadenectomy. In view of the frequency of sarcoid cervical lymphadenopathy, this disease should be considered in the differential diagnosis of benign causes of symptomatic obstruction of the venous drainage of the upper part of the body.     

Lymphoscintigraphy in plastic bronchitis,A pediatric case report

Plastic bronchitis (PB) is an uncommon, potentially fatal disease, marked by endobronchial cast formation causing variable degrees of respiratory distress. Primary and secondary pulmonary lymphatic abnormalities have been identified among the underlying mechanisms of cast formation. We present a case of PB where lymphoscintigraphy demonstrated the underlying lymphatic defect. A 6‐year‐old Hispanic male with congenital heart disease (CHD; post‐Fontan) presented with recurrent pneumonia, respiratory distress. Bronchoscopy showed inflamed hypervascular mucosa and thick mucus plugs; no casts were seen. Later, PB was diagnosed after the patient expectorated a bronchial cast. Cast analysis showed lymphocytic aggregates with mucin and fibrin. Lymphoscintigraphy revealed abnormal lymphatic collaterals and retrograde trace reflux into the superior mediastinum, a picture consistent with thoracic duct lymph leakage into the tracheobronchial tree. The pathogenesis of PB is not fully understood, especially in patients with CHD. Chyle in bronchial casts suggests abnormal lymphatic flow. Reports of lymph flow abnormalities, especially endobronchial lymph leakage in CHD are limited. Lymphoscintigraphy in our case demonstrated clear evidence of retrograde lymph reflux and leakage into the bronchial tree. The case presented suggests that in some patients following Fontan surgery, high intrathoracic lymphatic pressure and retrograde lymph flow may contribute to recurrent cast formation. Finding the underlying lymphatic abnormality helps in specific case management. Lymphoscintigraphy is a safer and easier method than lymphangiography. Surgical lymphatic–venous shunting may be possible in select cases. Pediatr Pulmonol. 2013; 48:515–518. © 2012 Wiley Periodicals, Inc.     

Dilated cardiomyopathy associated with chylous ascites

Chylous ascites is a well-documented sequelae of traumatic rupture of the thoracic duct and mechanical obstruction of the lymphatic system due to neoplastic, inflammatory, or congenital anomalies. Less commonly, chylous ascites results from altered hemodynamics and lymphatic flow, as seen in cirrhosis and constrictive pericarditis. Rarely, severe right-sided heart failure from a variety of causes has also resulted in chylous ascites or a protein-losing enteropathy. We report a case of chylous ascites due to dilated cardiomyopathy with autopsy findings. The pathophysiology of chylous ascites formation in right heart failure will be discussed, with a review of the literature.     

Ultrasonographic Imaging of the Cervical Thoracic Duct in Children with Congenital or Acquired Heart Disease

Conditions that increase central venous pressure lead to secondary dilation of the thoracic duct and impaired lymphatic circulation. We report the use of ultrasound to directly image the cervical part of the thoracic duct in children without the need for invasive techniques or contrast agents. Systematic evaluation of the thoracic duct may be useful in cardiovascular conditions with congestion of the lymphatic system such as single ventricle following Glenn or Fontan procedures.     

Giant coronary sinus and absent right superior vena cava

Although persistent left superior vena cava (PLSVC) is rare, it is the most common thoracic venous anomaly. The prevalence of PLSVC is 0.3 % in a general population and 10 % in patients with congenital heart disease. Diagnosis of PLSVC is usually incidental during cardiovascular imaging or surgery. Although PLSVC is usually not associated with any negative hemodynamic effect, it is important to be aware of its existence, since it may cause problems in central venous catheterization, pacemaker implantation and cardiopulmonary bypass. The overall proportion of absent right superior vena cava in patients with PLSVC is approximately 20 %. If PLSVC is associated with absence of the right superior vena cava, the coronary sinus may become gigantic due to excessive inflow. Herein, we report a case of PLSVC complicated by the absence of a right superior vena cava resulting in a giant coronary sinus.     

Systemic venous anatomy in congenital heart disease: implications for electrophysiologic testing and catheter ablation

Introduction  

Cardiac arrhythmias are a significant problem in patients with congenital heart disease. Many patients with congenital heart disease have abnormal systemic venous anatomy which can complicate electrophysiologic testing, catheter ablation and pacemaker and defibrillator implantation. We reviewed the systemic venous anatomy in a cohort of patients undergoing electrophysiologic testing and catheter ablation.     

Effect of phosphodiesterase III inhibitor (Olprinone) on thoracic duct lymph flow in anesthetized sheep with experimentally induced heart failure by endothelin-1

We investigated the short-term effects of a phosphodiesterase III inhibitor (Olprinone) on hemodynamics and thoracic duct lymph flow in anesthetized open-chest sheep with heart failure induced by endothelin-1 (cardiogenic shock). Ultrasound transit-time flow probes were attached to the thoracic duct, the ascending aorta and the renal artery. Arterial, pulmonary and central venous pressures were monitored. Endothelin-1 was infused intravenously at a dosage that reduced cardiac output to 50% or more of baseline (n=11). The effects of Olprinone were examined (n=5) by intravenous infusion after endothelin-1 administration. Other sheep (n=6) were used as controls. Olprinone significantly increased cardiac output that had been decreased by endothelin-1 and further increased thoracic duct flow that had been increased by endothelin-1. Increased arterial and pulmonary pressures induced by endothelin-1 administration were rapidly decreased by Olprinone. Renal arterial flow and central venous pressure were, however, unchanged by Olprinone. Overall, Olprinone acutely improved experimental cardiogenic shock (heart failure) induced by endothelin-1, and maintained thoracic duct lymph flow at a high level after endothelin-1 administration.     

Anomalous subaortic course of the left brachiocephalic (innominate) vein: echocardiographic diagnosis and report of an unusual association

Subaortic left brachiocephalic, or innominate, vein is an uncommon finding in congenital heart disease, usually associated with obstruction of the right ventricular outflow tract. We describe our experience with 14 patients in whom the lesion was identified echocardiographically, 12 of them with right ventricular obstruction, one with totally anomalous pulmonary venous connection in the absence of obstruction to the right ventricular outflow tract, and the final one with a normal heart. A precise diagnosis of this venous anomaly is of great importance, since it needs to be differentiated from a central pulmonary artery, a pulmonary venous confluence, or an ascending vertical vein in totally anomalous pulmonary venous connection. In patients referred for surgery without catheterization, an incorrect echocardiographic diagnosis could lead to disastrous surgical results.     

Pearls for Ablation in Congenital Heart Disease

Ablation in Congenital Heart Disease. Tachyarrhythmias occur in patients having congenital heart disease most commonly related to surgically created or naturally occurring conduction obstacles, and to postoperative hemodynamic effects on cardiac muscle. Less frequently, the underlying disease is associated with tachycardia substrates. Thorough knowledge of the patient's congenital anatomy and surgical procedures is required before considering catheter ablation. In many instances, procedural considerations should include meticulous hemodynamic surveillance, analogous to patients having structurally normal heart but cardiomyopathy. This includes careful selection of sedating and anesthetic agents. Congenital heart defects that have a higher than expected incidence of naturally occurring tachyarrhythmia substrates include Ebstein anomaly of the tricuspid valve, congenitally corrected transposition, and some of the heterotaxies. Intraatrial reentry tachycardia and atrial flutter are especially prevalent following the Mustard or Senning operations for d‐transposition of the great arteries and the earlier Fontan type operations. Although these tachyarrhythmias are not as frequent following atrial septal defect repair, the high incidence of this defect also makes these patients germaine to this discussion. Focal atrial tachyacrdia and atrioventricular nodal reentry tachycardia also occur in these patient groups. Macroreentry ventricular tachycardia occurs most frequently following right ventricular outflow tract surgery, especially following repair of tetralogy of Fallot. This article focuses on the technical aspects of catheter ablation of these arrhythmias, due to the challenges presented by the underlying anatomy compared with patients having normal hearts. (J Cardiovasc Electrophysiol, Vol. 21, pp. 223‐230, February 2010)     

Hemostatic properties of the lymph: relationships with occlusion and thrombosis

Lymphatic thrombosis is a rare occurrence, and although its frequency is likely underestimated, its burden remains substantially lower than that of venous or arterial thrombosis. Current evidence suggests that despite measurable levels of fibrinogen, von Willebrand factor and other coagulation factors in the lymph, fibrin generation is substantially inhibited under physiological conditions, essentially making the lymph a hypocoagulable biological fluid. Although factor VIIa-tissue factor-catalyzed activation of factor X is possible in the lymph, fibrin generation is largely counteracted by the unavailability of cell surface anionic phospholipids such as those physiologically present on blood platelets, combined with only low levels of coagulation factors, and the strong inhibitory activity of heparin, antithrombin, and tissue factor pathway inhibitor. Enhanced fibrinolytic activity further contributes to reduce the development and growth of lymph clots. Nevertheless, lymphatic thrombosis is occasionally detected, especially in the thoracic duct, axillary, or inguinal lymphatics. Pathogenetic mechanisms are supported by the release of thromboplastin substances from the injured lymphatic endothelium accompanied by chronic obstruction of lymph flow in the presence of a hypercoagulable milieu, thereby mirroring the Virchow triad that otherwise characterizes venous thrombosis. In theory, any source of lymphatic vessel occlusion, such as internal obliteration, external compression, or increased lymphatic pressure, might predispose to localized lymphatic thrombosis. The leading pathologies that can trigger thrombosis in the lymphatic vessels include cancer (due to external compression, neoplastic obliteration of the lymphatic lumen by metastatic cells, or lymphatic dysfunction after lymph node dissection), infections (especially lymphatic filariasis or sustained by Chlamydia trachomatis, Mycobacterium tuberculosis, Treponema pallidum, or Streptococcus pyogenes), congestive heart failure, chronic edema and inflammation of the distal lower limb, complications of central venous catheterization, coronary artery bypass grafting, thoracic outlet syndrome, and amyloidosis.     

Catheterization of the pulmonary artery using a 3 French catheter in patients with congenital heart disease

A 3 French (3F) coaxial polyurethane catheter was utilized for catheterization of the pulmonary artery in patients with complex congenital heart disease with concomitant stenosis or atresia of the pulmonary valve, with or without Blalock-Taussig shunts. Mean aortic and atrial pressures measured with the 3F catheter were compared with those measured with 5–6F conventional catheters. The values measured with the 3F catheter were identical to those measured with 5–6F catheters. Mean pulmonary arterial pressure measured using the 3F catheter was significantly higher than that measured using the 5–6F catheters, but it was not significantly different from mean pulmonary venous wedge pressure. Of 43 patients, 5–6F conventional catheters could be inserted into the branch pulmonary artery in 22 patients, but the 3F catheter could be inserted into the pulmonary artery in all patients. These data suggest that the 3F catheter is useful for catheterization in patients with complex congenital heart disease associated with pulmonary atresia or severe pulmonary stenosis. Cathet. Cardiovasc. Diagn. 45:45–50, 1998. © 1998 Wiley-Liss, Inc.     

Percutaneous translumbar cardiac catheterization and central venous line insertion: An alternative approach in children with congenital heart disease

Children with congenital heart disease present major problems with venous access, eliminating conventional routes for cardiac catheterization. Although the transhepatic approach has recently gained popularity, we describe here an alternative approach using percutaneous translumbar approach for cardiac catheterization and/or in-dwelling central line insertion in three children with congenital heart disease. Diagnostic hemodynamic studies, transcatheter delivery of an intravascular stent for left pulmonary artery (LPA) stenosis, and chronic central venous line insertion were performed using this technique. Disadvantages include interventionalist's unfamiliarity with technique, awkward patient positioning, technically more difficult than transhepatic, and potential injury to kidney and bowel. Advantages include avoidance of vascular-rich hepatic parenchyma, thus reducing risk of hemorrhage; providing an alternative where transhepatic entry may be contraindicated; avoidance of bile duct, portal vein, and hepatic artery injury; and providing another alternative for not only transvenous, but also transarterial access that may be required for intravascular aortic stent delivery. The interventional radiologist should be utilized as a valuable resource to the cardiologist to help teach and supervise this technique in selected infants and children with limited vascular access. Cathet. Cardiovasc. Intervent. 46:187–192, 1999. © 1999 Wiley-Liss, Inc.     

Persistent Left Superior Vena Cava Identified During Central Line Placement: A Case Report

INTRODUCTION: A persistent left superior vena cava is found in 0.3-0.5% of the general population and in up to 10% of patients with a congenital cardiac anomaly. It is the most common thoracic venous anomaly and is usually asymptomatic. Being familiar with such anomaly could help clinicians avoid complications during placement of central lines, Swan-Ganz catheters, PICC lines, dialysis catheters, defibrillators, and pacemakers. CASE PRESENTATION: We describe a case of persistent left superior vena cava that was noted after placement of a central line. Mr JJ is a 41 year old African American man who was hospitalized for evaluation and management of alcoholic necrotizing pancreatitis. He required multiple central lines placements. He was noted to have a persistent left superior vena cava that was not recognized initially and thus lead to an unnecessary extra central line placement. DISCUSSION: This anatomic variant may pose iatrogenic risks if it is not recognized by the clinician. A central catheter that tracks down the left mediastinal border may also be in the descending aorta, internal thoracic vein, superior intercostal vein, pericardiophrenic vein, pleura, pericardium, or mediastinum. CONCLUSION: Our case is significant because the patient had two extra central venous catheter placements. This case strongly demonstrates the importance of knowing the thoracic venous anomalies.     

Isolated chylopericardium after intrapericardial procedures: possible role of inadvertent right efferent lymphatic trunk injury

Chylopericardium after an intrapericardial procedure is rare, and satisfactory explanations of its possible causes are lacking.Herein, we present 4 cases of chylopericardium that developed after intrapericardial surgery, and we review the literature.Our literature review revealed 29 cases of chylopericardium that complicated intrapericardial operations, to which we added our 4 cases for analysis. The 33 surgical procedures involved repair for congenital heart disease (n=21), valve surgery (n=5), coronary artery bypass grafting (n=6), and other (n=1). Causes were verified in 7 patients: small lymphatic injury in 3 and high venous pressure or venous thrombosis in 4. Of the 26 patients with chylopericardium of unknown origin, 15 had congenital heart disease. Ten of these 15 had chromosomal abnormalities, especially trisomy 21 (Down syndrome); these patients typically had increased lymphatic permeability, which raised the likelihood of chylopericardium. Five revascularizations for coronary artery disease required harvesting of the left internal thoracic artery for reconstruction, incurring a risk of damage to the drainage site of the right efferent lymphatic trunk. In addition, all 26 patients with chylopericardium of unknown origin underwent dissection of the ascending aorta and the main pulmonary artery, near the right efferent lymphatic trunk. Inadvertent injury to the trunk during the dissection would have increased the risk of chylopericardium. Accordingly, even though the overall incidence of chylopericardium during intrapericardial procedures is low, we recommend a meticulous dissection of the ascending aorta from the main pulmonary artery.     

Hypertrophic osteoarthropathy in cyanotic congenital heart disease. its prevalence and relationship to bypass of the lung

The frequency of hypertrophic osteoarthropathy in cyanotic congenital heart disease has previously been considered to be very low. Only a few isolated reports have described such an association. We studied 32 consecutive patients older than 6 years with various types of cyanotic congenital heart disease and examined each case for hypertrophic osteoarthropathy. We also assessed the role of the altered cardiopulmonary hemodynamics in the development of hypertrophic osteoarthropathy by means of cardiac catheterization. Our results showed that 31% of the patients had hypertrophic osteoarthropathy. When the hemodynamic parameters of the group of patients with hypertrophic osteoarthropathy were compared with those of the remaining patients, we found significant differences in the systemic blood flow (P ⩽ 0.05), right-to-left shunt (P ⩽ 0.05), and arterial oxygen unsaturation (P ⩽ 0.005). Thus, we found a much higher prevalence of hypertrophic osteoarthropathy in patients with cyanotic congenital heart disease than has previously been recognized. The development of hypertrophic osteoarthropathy is related to the degree of bypass of the lung. This is consistent with the concept that hypertrophic osteoarthropathy results from mediators in the systemic venous circulation that escape inactivation in the pulmonary capillary bed.     

Albumin uptake by renal lymphatics with and without obstruction of the renal vein

Experiments involving injection of radio-iodinated albumin into the left renal arteries or left ureters of dogs indicate that the renal lymphatics are capable of a major contribution in returning to the circulation albumin (or other large molecules) escaping from renal capillaries. I131-albumin was injected into the jugular vein of controls or into the left renal artery or in retrograde manner into the left ureter of female dogs. Experimental groups included those with no obstruction, occlusion of left renal veins or left renal lymphatics, or both. Collections were made from the right femoral artery, both renal veins, thoracic duct and both ureters at frequent intervals for 2 to 4 hours. Data analysis included I131 concentration, specific activity, rate of recovery and selected ratios. After renal arterial injection, the percentage of I131 recovered in thoracic duct lymph of dogs without renal venous obstruction was 5 to 10 times that recovered in those that received injections into the jugular vein. In dogs with renal venous obstruction, recovery from the thoracic duct was 10 to 1,000 times that in control dogs. Most of the differences occurred during the first hour, after which time as recirculation and redistribution occurred the rates of appearance in thoracic duct lymph in each group were similar. The differences in the ratios of concentration in thoracic duct lymph to concentration in femoral arterial blood were also much greater when the renal vein was obstructed than in dogs with no obstruction. The greater return of albumin through the thoracic duct in those with impeded renal venous outflow was probably related to both sequestered concentration and increased intrarenal pressure.(ABSTRACT TRUNCATED AT 250 WORDS)