BACKGROUND—Plastic bronchitis is a potentially fatal disorder occurring in children with single-ventricle physiology, and other diseases, as well, such as asthma. In this study, we report findings of ...abnormal pulmonary lymphatic flow, demonstrated by MRI lymphatic imaging, in patients with plastic bronchitis and percutaneous lymphatic intervention as a treatment for these patients.
METHODS AND RESULTS—This is a retrospective case series of 18 patients with surgically corrected congenital heart disease and plastic bronchitis who presented for lymphatic imaging and intervention. Lymphatic imaging included heavy T2-weighted MRI and dynamic contrast-enhanced magnetic resonance lymphangiogram. All patients underwent bilateral intranodal lymphangiogram, and most patients underwent percutaneous lymphatic intervention. In 16 of 18 patients, MRI or lymphangiogram or both demonstrated retrograde lymphatic flow from the thoracic duct toward lung parenchyma. Intranodal lymphangiogram and thoracic duct catheterization was successful in all patients. Seventeen of 18 patients underwent either lymphatic embolization procedures or thoracic duct stenting with covered stents to exclude retrograde flow into the lungs. One of the 2 patients who did not have retrograde lymphatic flow did not undergo a lymphatic interventional procedure. A total of 15 of 17(88%) patients who underwent an intervention had significant symptomatic improvement at a median follow-up of 315 days (range, 45–770 days). The most common complication observed was nonspecific transient abdominal pain and transient hypotension.
CONCLUSIONS—In this study, we demonstrated abnormal pulmonary lymphatic perfusion in most patients with plastic bronchitis. Interruption of the lymphatic flow resulted in significant improvement of symptoms in these patients and, in some cases, at least temporary resolution of cast formation.
Congestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking.
Liver biopsies and cardiac catherizations were ...performed as part of an evaluation offered to all patients ≥10 years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD). Biopsies from included subjects were compared to stained specimens from controls without known fibrotic liver disease. Patient characteristics, echocardiographic findings, and hemodynamic measures were evaluated as potential risk factors. The cohort consisted of 67 patients (31 female) at mean age of 17.3±4.5 years and mean time from Fontan of 14.9±4.5 years. Right ventricular morphology was present in 37 subjects. Median %CD by Sirius red staining was 21.6% (range 8.7% to 49.4%) compared to 2.6% (range 2.2% to 3.0%) in controls. There was a significant correlation between time from Fontan and degree of Sirius red staining (
=0.33,
<0.01). Serum liver enzymes and platelet count did not correlate with %CD. The median inferior vena cava pressure was 13 mm Hg (range 6-24 mm Hg) and did not correlate with %CD. There was no difference in %CD based on ventricular morphology or severity of atrioventricular valve insufficiency.
In this cohort of predominantly asymptomatic children and adolescents electively evaluated after a Fontan operation, all exhibited evidence for hepatic fibrosis as measured by collagen deposition in the liver. Time from Fontan was the only factor significantly associated with collagen deposition. These findings demonstrate that liver fibrosis is an inherent feature of Fontan physiology and that the degree of fibrosis increases over time.
Congenital heart disease can lead to notable lymphatic complications such as chylothorax, plastic bronchitis, protein-losing enteropathy, and ascites. Recent improvements in lymphatic imaging and the ...development of new lymphatic procedures can help alleviate symptoms and improve outcomes.
Abstract Objectives This study sought to confirm that the short-term hemodynamic effectiveness of the Melody transcatheter pulmonary valve (TPV) (Medtronic, Inc., Minneapolis, Minnesota) achieved by ...real-world providers is equivalent to the historical results established in the initial 5-center Investigational Device Exemption trial. Background TPV replacement has been used to treat right ventricular outflow tract (RVOT) conduit dysfunction for >10 years. The Melody TPV received U.S. Food and Drug Administration approval in 2010 as a Humanitarian Use Device. Methods Patients with dysfunctional RVOT conduits were entered in this prospective, nonrandomized study at 10 centers. The primary endpoint was acceptable hemodynamic function at 6 months post-implantation, defined as a composite of RVOT echocardiographic mean gradient ≤30 mm Hg, pulmonary regurgitation less than moderate as measured by echocardiography, and freedom from conduit reintervention and reoperation. Results Cardiac catheterization was performed in 120 patients for potential implantation of the Melody TPV; of these, 100 patients were implanted, with a 98.0% procedural success rate. There were no procedure-related deaths. Acceptable hemodynamic function at 6 months was achieved in 96.7% of patients with evaluable data (87.9% of the entire implanted cohort), with results maintained through 1 year. No patient had moderate or severe pulmonary regurgitation after implantation. No patient required catheter reintervention in the first year after implantation, and 2 patients required reoperation for conduit replacement. The rate of freedom from TPV dysfunction was 96.9% at 1 year. Conclusions This first prospective, real-world experience with the Melody TPV in the United States demonstrates continued high procedural success, excellent short-term TPV function, and low reintervention and reoperation rates at 1 year. (Melody Transcatheter Pulmonary Valve Post-Approval Study; NCT01186692 )
As the cohort of survivors with the single-ventricle type of congenital heart disease grows, it becomes increasingly evident that the state of chronically elevated venous pressure and decreased ...cardiac output inherent in the Fontan circulation provides the substrate for a progressive decline in functional status. One organ at great risk is the liver. Wedged between two capillary beds, with the pulmonary venous bed downstream, which typically has no pulsatile energy added in the absence of a functional right ventricle, and the splanchnic bed upstream, which may have compromised inflow due to inherent cardiac output restriction characteristic of the Fontan circulation, the liver exists in a precarious state. This review summarizes a consensus view achieved at a multidisciplinary symposium held at The Children’s Hospital of Philadelphia in June 2011. The discussion includes current knowledge concerning the hemodynamic foundations of liver problems, the diagnostic tools available, the unique histopathology of the liver after the Fontan operation, and proposed mechanisms for hepatic fibrosis at the cellular level. At the completion of the symposium, a consensus recommendation was made by the authors’ group to pursue a new prospective protocol for clinical evaluation of the liver for all patients in our practice 10 years after the Fontan operation.
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.
Protein-losing enteropathy (PLE) and plastic bronchitis are serious complications that occur after single-ventricle surgery. A lymphatic cause for these conditions has been proposed, but imaging ...correlation has not been reported. The objective of this study was to evaluate lymphatic abnormalities in patients after functional single-ventricle palliation compared with patients with non-single-ventricle congenital heart conditions using T2-weighted MR lymphangiography.
We retrospectively reviewed imaging data from 48 patients who underwent T2-weighted MR lymphangiography in our institution between May 1, 2012, and October 24, 2012. The patients were divided into four groups: patients who underwent superior cavopulmonary connection, patients who underwent total cavopulmonary connection, patients with total cavopulmonary connection and lymphatic complications, and patients with non-single-ventricle cardiac anomalies.
There were 38 patients with single ventricles in this study. The lymphatic abnormalities observed in these patients included thoracic duct dilation greater than 3 mm (31%), lymphangiectasia and lymphatic collateralization (78%), and tissue edema (86%). There were five patients with PLE, one patient with plastic bronchitis, and one patient with chronic chylous effusions and superior cavopulmonary connection. The patients with PLE and plastic bronchitis had statistically significant larger thoracic duct maximal diameters (median, 3.9 mm; range, 3-7.2 mm) than did the other patients with total cavopulmonary connection (p < 0.01). In the two-ventricle patient group, there were no abnormal lymphatic findings.
Lymphatic abnormalities are found in many patients after functional single-ventricle palliation. T2-weighted unenhanced MRI is capable of anatomic assessment of the lymphatic system in this patient population and has promise for guiding treatment in the future.
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