Systemic-pulmonary Collateral Arteries Research Articles

A RARE CASE OF HISTOPLASMA CAUSING OBLITERATION OF THE SUPERIOR VENA CAVA AND SUBSEQUENT DEVELOPMENT OF COLLATERAL CIRCULATION

A RARE CASE OF HISTOPLASMA CAUSING OBLITERATION OF THE SUPERIOR VENA CAVA AND SUBSEQUENT DEVELOPMENT OF COLLATERAL CIRCULATION

Staged correction of pulmonary atresia, ventricular septal defect, and collateral arteries.

ObjectivesPulmonary atresia (PA) with ventricular septal defect (VSD) and systemic‐pulmonary collateral arteries (SPCAs) presents with variable anatomy with regard to the pulmonary vasculature, requiring personalized surgical treatment. A protocol consisting of staged unifocalization and correction was employed.MethodsSince 1989, 39 consecutive patients were included (median age at first operation 13 months). In selected cases, a central aorto‐pulmonary shunt was performed as the first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure, and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Echocardiographic data were obtained postoperatively and at interval follow‐up.ResultsIn 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, of which four have since died. One patient is awaiting further correction. A correction was performed successfully in 28 patients. Operative mortality was 3% and late mortality was 11%. Median follow‐up after the correction was 19 years. Eleven patients required homograft replacement. Freedom from conduit replacement was 88%, 73%, and 60% at 5, 10, and 15 years respectively. Right ventricular function was reasonable or good in 75% of patients. All but one patient were in NYHA Class I or II.ConclusionsAfter complete unifocalization 30/37 patients (81%) were considered correctable. The staged approach of PA, VSD, and SPCAs results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.

Homograft durability after correction of pulmonary atresia and ventricular septal defect with or without systemic pulmonary collateral arteries

BackgroundPulmonary atresia and ventricular septal defect (PA-VSD), with or without systemic pulmonary collateral arteries (SPCAs), represents a complex anatomic and surgical spectrum of congenital heart disease. Currently, there is limited evidence on homograft durability after complete correction, which potentially could be affected by anatomic differences in pulmonary vasculature. MethodsThis retrospective single-center study included all 69 consecutive PA-VSD patients (46 with SPCAs, 23 without SPCAs) operated on between 1978 and 2018. The primary interest was in homograft durability after complete repair. Longitudinal echocardiographic homograft function and right ventricular systolic pressure were analyzed with linear mixed-effects models. ResultsThe median duration of follow-up was 20 years. Of the 46 patients with SPCAs, 37 (80.4%) underwent biventricular correction at a median age of 2.7 years (interquartile range [IQR], 1.8-6.3 years). Two patients are currently awaiting unifocalization and correction. All 23 patients without SPCAs underwent successful complete correction at a median age of 1.6 years (IQR, 1.1-3.6 years). Freedom from any reintervention after 20 years was 15%. When a homograft was used during correction, freedom from homograft replacement after 20 years was comparable in the 2 groups (P = .925), at 32 ± 11% in the SPCA group and 32 ± 13% in the non-SPCA group. Indications for homograft replacement were isolated stenosis (n = 7; 46.7%), isolated regurgitation (n = 3; 20.0%), and mixed stenosis and regurgitation (n = 5; 33.3%) in the SPCA group and isolated stenosis (n = 8; 88.9%) and stenosis and regurgitation (n = 1; 11.1%) in the non-SPCA group. Peak homograft gradient was significantly (P = .0003) higher in patients without SPCA, with a comparable rate of progression in the 2 groups. However, the prevalence of severe pulmonary regurgitation (PR) was higher in patients with SPCAs, estimated at 35% at 10 years, compared with 15% in patients without SPCAs. ConclusionsHomografts used for right ventricular outflow tract reconstruction in patients with PA-VSD, either with or without SPCAs, have similar limited durability. Repeated reintervention is common, and careful follow-up with attention to severe PR is warranted.

Use of the pressure wire method for measuring pulmonary arterial pressures in patients with pulmonary atresia

The objective of the study was to analyse the use of the pressure wire for the acquisition of intravascular pulmonary pressures in the presence of pulmonary atresia and systemic-dependent pulmonary blood flow. In this study, we included patients with pulmonary atresia and systemic-dependent pulmonary circulation referred for diagnostic catheterisation for evaluation of pulmonary pressures during the period from April, 2012 to April, 2013. The systemic-pulmonary collateral arteries were selectively catheterised, and in the absence of a critical stenosis angiographically determined; the pressure wire was introduced in these arteries to reach the main pulmonary artery, and/or lobar, and segmental branches. Aortic and pulmonary pressures were simultaneously obtained. We evaluated the feasibility and safety of the method. We studied 10 patients (age 21 days to 11 years). In all of them, the pressures of pulmonary circulation - main artery, and/or lobar, and segmental branches - were successfully measured with the pressure wire. Of eight patients with indication for Rastelli surgery, the pulmonary pressures were considered normal in five, and slightly increased in three. In two patients requiring univentricular correction - total cavopulmonary anastomosis - the diastolic pressure was increased (20 mmHg). All procedures were performed without haemodynamic instability, cardiac arrhythmia, systemic saturation reduction, death, or any other complication. Measurement of pulmonary vascular pressures using the pressure wire in small patients with pulmonary atresia is safe and effective. It allows the acquisition of reliable pressure curves, even in the presence of small vessels, bending and tortuosity, without the risk usually associated with the use of conventional diagnostic catheters.

Assessment of systemic-pulmonary collateral arteries in children with cyanotic congenital heart disease using multidetector-row computed tomography: Comparison with conventional angiography

Background The present study aimed to assess the feasibility of multidetector-row computed tomography (MDCT) for the evaluation of systemic-pulmonary collateral (SPC) arteries in children with congenital heart disease associated with reduced pulmonary blood flow. Methods Forty-eight consecutive patients (mean age 9 ± 5 months; range, 0–30 months) underwent MDCT angiography of the thorax with a 16-detector row scanner prior to cardiac catheterization and operation. Conventional angiographic findings were used as a gold standard for the detection of SPC vessels. Findings on CT angiograms, including CT scans, maximum intensity projections, and three-dimensional volume-rendered images, were used to evaluate depiction of SPC arteries. Quantification of measurements at the SPC artery diameter was evaluated independently on MDCT and conventional invasive angiography. Results Among the 48 patients, 115 SPC arteries were identified with conventional angiography, and 94 SPC arteries were identified with MDCT. In 89 (77%) vessels, concordant findings were observed with both modalities, with adequate depiction in 53 vessels and suboptimal depiction in 36 vessels. In 26 (23%) vessels, MDCT was unable to identify SPC arteries. Further, CT angiography resulted in the false-positive identification of vessels in 5 cases. There was an excellent correlation between MDCT- and conventional angiography-based measurement of SPC vessel diameter ( R 2 = 0.83), although a systematic overestimation was observed with MDCT (bias 0.19 ± 0.74 mm). Conclusions This study demonstrates that MDCT is a potentially useful tool, which may have implications for planning percutaneous interventions and surgical repair in the future.

Clinical Investigation of Systemic-Pulmonary Collateral Arteries

Systemic-pulmonary collateral arteries are known to develop in children with congenital heart disease, chronic pulmonary infection, and prematurity. At present, these abnormal connections between the systemic and the pulmonary systems are thought to develop from the vascular plexus, which normally gives rise to the pulmonary and bronchial vasculature. The objective of this study was to review our patients with systemic-pulmonary collateral arteries and evaluate possible risk factors. The records of patients with systemic-pulmonary collateral arteries seen at our hospital over a 4-year period were retrospectively reviewed. They were grouped into one of the following five categories: premature, heart disease, pulmonary disease, healthy, and others. Age, gender, weight, and the results of echocardiography were recorded, as was the condition on follow-up. We reviewed the records of 284 patients: 130 premature, 13 heart disease, 30 pulmonary disease, 92 healthy, and 19 others. Over the same period, 3314 healthy 1-month-old infants had undergone echocardiography for health screening. Among the 92 healthy children with systemic-pulmonary collateral arteries, 52 were diagnosed at the health-screening exam. Therefore, we estimate the incidence of unsuspected systemic-pulmonary collateral arteries in healthy 1-month-old infants to be 1.57% (52/3314). We conclude that systemic-pulmonary collateral arteries may be present normally after birth and then gradually disappear. However, if there are certain predisposing factors, they may persist in order to augment pulmonary flow.

Usefulness of Magnetic Resonance Angiography for Diagnosis of Scimitar Syndrome in Early Infancy

This study evaluated the feasibility and accuracy of the magnetic resonance angiography (MRA) evaluation of scimitar syndrome in early infancy in 4 subjects. MRA imaging was successful, with excellent imaging quality to evaluate the main and lobar pulmonary artery branches, lobar pulmonary veins, scimitar vein, and systemic-pulmonary collateral arteries in all subjects. There was good agreement between findings with MRA, x-ray angiography (n = 3), surgical inspection (n = 3), and autopsy (n = 1).

Pulmonary atresia with intact ventricular septum and systemic-pulmonary collateral arteries

Pulmonary atresia with intact ventricular septum and major systemic-pulmonary collateral arteries is a very rare congenital heart lesion with dismal natural history. Herein we report on a case of pulmonary atresia with intact ventricular septum with hypoplastic right ventricle, very small confluent pulmonary arteries, absent arterial duct, and pulmonary blood flow exclusively provided by bronchial-type systemic-pulmonary collateral arteries that was successfully treated at our institution.

Systemic-pulmonary collateral arteries and coil embolization in patients with staged Fontan operation

Systemic-pulmonary collateral arteries and coil embolization in patients with staged Fontan operation

A new surgical approach for preparation of candidates for the Fontan operation with acquired systemic-pulmonary collateral arteries

SummaryA new surgical technique is described to reduce the flow through acquired systemic-pulmonary collateral arteries, a complication which has been recognized as a risk factor in patients undergoing the Fontan operation. When systemic-pulmonary collateral arteries have developed subsequent to previous construction of an ipsilateral Blalock-Taussig shunt or a Glenn anastomosis, and are identified by angiography in advance of the Fontan operation, the technique is indicated as a preparative procedure. It consists of rethoracotomy aimed at isolating the lung from the thoracic wall (peeling), and wrapping of the upper half of the lung with expanded polytetrafluoroethylene sheets to avoid redevelopment of collateral vessels into the lung (wrapping). This decreases the systemic-collateral flow through small vessels which cannot be handled by conventional embolization using catheterization techniques, and permits the Fontan operation to be performed more safely.

The restricted surgical relevance of morphologic criteria to classify systemic-pulmonary collateral arteries in pulmonary atresia with ventricular septal defect

Now that systemic-pulmonary collateral arteries are used for unifocalization in patients with pulmonary atresia and ventricular septal defect, the question arises whether morphologic criteria of these collateral arteries could help to provide better results. In an attempt to classify the morphologic features of systemic-pulmonary collateral arteries, we studied 31 heart-lung autopsy specimens with pulmonary atresia and ventricular septal defect. The course of the systemic-pulmonary collateral arteries (origin, branching pattern, and conections with systemic and central pulmonary arteries) was related to their histologic characteristics. The results show that systemic-pulmonary collateral arteries cannot be classified according to their course related to the trachea and the main branches of the bronchi. The histologic features of these collateral arteries vary along their course to the lungs. Nearly all systemic-pulmonary collateral arteries contain a muscular or a musculoelastic segment. One type of collateral artery (complex loop anastomoses) is completelymuscular and resembles a bronchial artery. Nutritive branches (bronchial arteries) arise from all histologic types of systemic-pulmonary collateral artery segments. The size and number of intimal proliferations in muscular, elastic, and musculoelastic segments did not differ significantly. In 29 of 31 cases a ductus arteriosus did not coexist with large collateral arteries (two cases unknown). It is concluded that a classification of large systemic-pulmonary collateral arteries based on morphologic features results in a highly variable system, which does not facilitate decisions for the suitability of these arteries for unifocalization procedures. The variability of the systemic-pulmonary collateral arteries corresponds with the recent embryologic finding that during development, collateral artery formation is possible during extended periods. (J Thorac Cardiovasc Surg 1994;108:692-9)

Development of the pharyngeal arch system related to the pulmonary and bronchial vessels in the avian embryo. With a concept on systemic-pulmonary collateral artery formation.

The literature is ambiguous as to the question of the developmental background of systemic-pulmonary collateral arteries. These are found in combination with various congenital heart malformations such as pulmonary atresia. From a clinical point of view, it is of interest to know whether we are dealing with the persistence of transient embryological vessels such as ventral segmental arteries or parts of pharyngeal arch arteries or with the prenatal or postnatal recruitment of the bronchial vasculature that normally supplies the lung. This study of the embryology of the extrapulmonary and intrapulmonary vasculature aims at a better understanding of the variations in origin, course, branching pattern, and histology of collateral arteries. Serial sections of quail embryos ranging between stage HH11 and stage HH28 were incubated with a monoclonal antibody (alpha MB1) against endothelial cells and their precursors. Additional series of chick embryos were injected with india ink to study the lumenized vascular patterns. A splanchnic plexus consisting of endothelial cells and precursors is present around the foregut before the lung buds develop. This plexus expands and gives rise to the pharyngeal arch arteries, the ventral pharyngeal veins, the pulmonary vessels, and the bronchial vessels, including the intrapulmonary vessel network. During two subsequent periods, the splanchnic plexus is transiently connected to the systemic arteries and veins. The bronchial arteries and veins develop in the second period from these transient vessels. The expansion and extension of the splanchnic plexus to many organs during the formation of the bronchial vessels explains the varying course and branching pattern of the bronchial vasculature. These results show that we are not dealing with two or more individual vascular systems that contribute to the developing vessels of the lungs but with one vascular plexus that normally gives rise to the pulmonary and bronchial vasculature but has the potential to give rise to other systemic-pulmonary connections.

Solitary arterial trunk with pulmonary atresia and arteries with supply to the left lung from both an arterial duct and systemic-pulmonary collateral arteries

We report a case of pulmonary atresia with ventricular septal defect and absent pulmonary trunk. The sources of pulmonary blood supply were the arterial duct and systemic pulmonary collateral arteries. The left lung received blood from both sources which is very rare. One of the systemic pulmonary collateral arteries arose from the right coronary artery in an unusual fashion.

Patterns of pulmonary arterial anatomy and blood supply in complex congenital heart disease with pulmonary atresia

To identify the pattern of pulmonary anatomy and pulmonary blood supply, we analyzed the angiocardiograms of 40 patients with pulmonary atresia and complex congenital heart disease. All patients but two (95%) had confluent pulmonary arteries supplied by a patent ductus arteriosus. These two patients (5%) had nonconfluent pulmonary arteries, one with major systemic-pulmonary collateral arteries and absent patent ductus arteriosus and the other with a patent ductus arteriosus supplying the right lung and small collateral arteries supplying the left. The low incidence of pulmonary arterial abnormalities and the uniform pattern of pulmonary blood supply make the patients with pulmonary atresia and complex congenital heart disease potential candidates for palliative operation without cardiac catheterization in the neonatal period.

Staged surgical repair of pulmonary atresia, ventricular septal defect, and hypoplastic, confluent pulmonary arteries

In patients with pulmonary atresia and ventricular septal defect, hypoplasia of the central pulmonary arteries prevents single-stage complete repair. Over an interval of 8 1/2 years, 105 patients underwent establishment of continuity between the right ventricle and a hypoplastic central pulmonary arterial confluence (first stage). There were 12 hospital deaths (11%) and 11 late deaths before second-stage (complete) repair. Twenty-five patients await late evaluation. The remaining 57 individuals have had follow-up cardiac catheterization a mean of 33 months postoperatively. In 31 of these, final repair was deferred because of insufficient pulmonary arterial enlargement (14), restricted peripheral arborization (nine), or both (eight). The final 26 patients were accepted for second-stage repair, which has been performed in 24. Complete repair included ventricular septal defect closure (24), right ventricular outflow tract reconstruction (18), relief of central pulmonary arterial stenosis (14), and ligation of systemic-pulmonary collateral arteries (10). The mean postrepair peak systolic right ventricular-left ventricular pressure ratio was 0.67 (range 0.32 to 1.0). One of these patients (4%) died in the hospital and there was one late death (4%) from sepsis after tricuspid valve replacement. Three patients were lost to follow-up; the remaining 19 patients are in functional Class I or II. A two-stage surgical approach is highly successful in those patients whose pulmonary arteries are too hypoplastic to allow a single-stage repair.

Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect.

Angiograms of 30 patients with congenital pulmonary valve atresia, ventricular septal defect, and large systemic-pulmonary collateral arteries (SPCAs) were evaluated. All had aortography, 28 had SPCA arteriography, and 26 had right ventriculography. Seventeen (65%) of 26 patients had a right ventricular infundibulum, 23 (77%) had a pulmonary artery confluence, and five of the nine patients without a right ventricular infundibulum had a confluence. Sixty-six SPCAs of aortic origin were seen; 28 (42%) had narrowing and 21 patients (70%) had one or more narrowed SPCAs. Five patients had collaterals from internal mammary, subclavian or innominate arteries. Fourteen (47%) had hilar pulmonary artery stenosis. Of these 14 patients mild peripheral stenosis was demonstrated in five. Right aortic arch was present in 15 patients (50%). Complete angiographic delineation of pulmonary vasculature is an essential procedure for preoperative detection of pulmonary and SPCA stenoses in these patients.