Discontinuous Pulmonary Arteries Research Articles

Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy: How Best to Approach

Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy: How Best to Approach

Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy

BackgroundWe describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. MethodsWe reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair. ResultsMedian age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11). ConclusionsSuccessful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.

A Novel Model for Xenograft Right Ventricle to Pulmonary Artery Conduit.

The last 40 years have shown dramatic improvement in outcomes for neonatal cardiac surgery for a spectrum of congenital heart disease diagnoses. With more patients surviving into adulthood, the long-term impact of initial management strategies of these patients has come into focus. This is particularly true for patients with pediatric heart valve disease. Many patients born with right ventricular to pulmonary artery (RVPA) discontinuity require placement of a valved conduit in the neonatal period. Valved conduit options are limited in this patient population due to patient size and inability to respond to somatic growth. Genetically engineered porcine (GEP) donors may offer a xenograft conduit alternative that can grow with the patient. We have developed a model utilizing GEP donor RVPA conduits placed in infantile nonhuman primate (NHP) recipients. Our recipient is maintained on single-drug immunosuppression and demonstrates no evidence of pulmonary valve insufficiency or stenosis during short-term follow-up. Further studies and long-term outcomes are necessary to determine the utility of this technology in human application.

ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study.

We sought to report characteristics and outcomes of children with complex anomalous left coronary artery from the pulmonary artery (ALCAPA) and tocompare the outcomes to children with isolated ALCAPA. We performed a retrospective data analysis of children with ALCAPA who underwent cardiac surgery between 1/2009 and 3/2018 at 21 centers. Characteristics and outcomes of patients with complex ALCAPA are provided using descriptive statistics. Outcomes were compared between complex ALCAPA and isolated ALCAPA using Fisher's exact test. We reviewed 258 patients who underwent surgical repair of ALCAPA at 21 centers. We identified 10 patients (3.9%) with complex ALCAPA. Median age at initial cardiac surgery was 49days (range: 4days, 12.8years). Cardiac lesions associated with ALCAPA were HLHS(n = 3); scimitar syndrome (n = 2); VSD with aortic coarctation (n = 2); VSDwith right pulmonary artery discontinuity (1); DORV with mitral atresia (n = 1); and ToF(n = 1). ALCAPA was diagnosed prior to surgical intervention in 1 patient; during the initial cardiac surgery in 4 patients; in the early postoperative period via cardiac catheterization in 3 patients; and later in childhood after initial surgical repair in 2 patients. Following ALCAPA repair, patients with complex ALCAPA, as compared to patients with isolated ALCAPA, were more likely to be placed on ECMO (50% vs 12%, p = 0.002), receive CPR (30% vs 6%, p = 0.017), or suffer operative mortality (50% vs 3%, p < 0.001). Complex ALCAPA is uncommon. All but one with complex ALCAPA was not diagnosed preoperatively and postoperative morbidity and mortality were significantly greater in these complex patients compared to patients with isolated ALCAPA.

Transcatheter Resolution of a Failed Neoconfluence in Pulmonary Atresia With Discontinuous Pulmonary Arteries

We present a case of a full-term newborn with complex congenital heart defects, including single-ventricle physiology and discontinuous pulmonary arteries. Prompt surgical intervention was performed, which involved pulmonary neoconfluence with autologous pericardium graft and systemic-to-pulmonary shunt placement. However, postoperative complications required stenting to address pulmonary artery stenosis.

Rare Sinusal Variant of Truncus Arteriosus with Discontinuous Pulmonary Arteries.

Rare Sinusal Variant of Truncus Arteriosus with Discontinuous Pulmonary Arteries.

Bilateral ductal stent in an andean newborn with discontinuous pulmonary arteries

Bilateral ductal stent in an andean newborn with discontinuous pulmonary arteries

Very preterm and very low birthweight infant with pulmonary atresia intact ventricular septum, right ventricle-dependent coronary circulation, and discontinuous pulmonary arteries.

Prematurity and low birthweight are associated with increased mortality in infants undergoing cardiac surgery. Pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation carries one of the highest risks of mortality. We present a patient who was born at 28 weeks of gestation at 1.2 kg, with pulmonary atresia intact ventricular septum, right ventricle-dependent coronary circulation, coronary artery atresia, and discontinuous pulmonary arteries, who successfully underwent palliation with a modified Blalock-Taussig shunt, pulmonary arterioplasty, and subsequently a bidirectional Glenn.

Short and intermediate term outcome post right ventricle to pulmonary artery conduit surgery

Abstract Background Surgery for congenital heart disease has progressed by leaps and bounds in the last few decades, but the right ventricular outflow tract continues to pose a challenge to the congenital heart surgeon. Objective To describe short and intermediate term outcome in congenital heart disease patients undergoing surgical repair using right ventricle to pulmonary artery conduits. Patients and Methods Our study included 33 patients that were operated upon by putting a conduit between the right ventricle and the pulmonary artery in a single center (Al Agouza police hospital) between 2015 and 2019. Results This study was done in order to follow up patients who underwent surgery for conduit placement between the right ventricle (RV) and the pulmonary artery (PA), to observe the reintervention rates and to determine the most important determinants of re-operation. 33 patients were included in this study, with age range 1.5-17 years and mean age of 8.29 +/- 4.7 years. The mean age at placement of the first conduit was 3.57 +/- 3.18 years. The youngest patient at time of conduit placement for the first time was 0.2 years old and the oldest had 12.5 years. Conclusion The use of conduits to treat the RV to PA discontinuity is a cornerstone in the treatment of some congenital heart diseases requiring construction of the right ventricle outflow tract (RVOT). Nevertheless, conduit failure and replacement is inevitable, and depends on many factors: as age at first operation, type of conduit, mean and peak pressure gradient across the conduit. The higher the age at first conduit, the bigger the event / re-intervention free survival period.

Surgical repair of discontinuous right pulmonary artery utilising an autologous main pulmonary artery flap: a case report and literature review

This is a case of an infant with unilateral discontinuous right pulmonary artery. Cardiac catheterisation with pulmonary wedge injection diagnosed the anomaly and aided in surgical planning. The patient underwent semi-autologous surgical repair utilising an autologous main pulmonary artery flap. One month following discharge, he underwent successful balloon dilation of the residual stenosis and was discharged the same day.

Pulmonary atresia with a ventricular septal defect and left pulmonary artery discontinuity: a case report\xa0

BackgroundUnilateral pulmonary artery discontinuity is a rare malformation that is associated with other intracardiac abnormalities. Cases accompanied by other cardiac abnormalities are often missed on prenatal echocardiography. The prenatal diagnosis of isolated unilateral pulmonary artery discontinuity can also be delayed. However, undiagnosed this malformation would have an effect on further prognosis. We report our case of a prenatal diagnosis of pulmonary atresia with ventricular septal defect and left pulmonary artery discontinuity.Case presentationA 33-year-old Asian woman visited our institution at 24 weeks of gestation because of suspected fetal congenital heart disease. Fetal echocardiography revealed a small atretic main pulmonary artery giving rise to the right pulmonary artery without bifurcation and the left pulmonary artery arising from the ductus arteriosus originating from the left subclavian artery. The neonate was delivered by cesarean section at 376/7 weeks of gestation. Postnatal echocardiography and multidetector computed tomography showed a right aortic arch, with the small right pulmonary artery originating from the atretic main pulmonary artery and the left pulmonary artery originating from the left subclavian artery. Patency of the ductus arteriosus from the left subclavian artery was maintained with prostaglandin E1. Right ventricular outflow tract reconstruction and pulmonary angioplasty with Gore-Tex graft patch was performed 25th day after birth. Unfortunately, the neonate died because of right heart failure 8 days postoperation.ConclusionThere is a possibility that both pulmonary arteries do not arise from the same great artery (main pulmonary artery or common arterial trunk). Therefore, clinicians should check the origin of both pulmonary arteries.

A large aortopulmonary window found in the setting of a right aortic arch with discontinuous pulmonary arteries.

Aortopulmonary window (APW) is a rare but serious congenital cardiac malformation, most patients with APW will die from congestive heart failure within one year after birth. In fact, patients with large APW is rarely seen in childhood or adult life. However, we report an older child with a large anomalous "window" on the ascending aorta, with discontinuous pulmonary arteries, and the left pulmonary artery (LPA) arising via a left-sided arterial duct in the presence of a right aortic arch. Preoperative diagnosis made by echocardiography and chest computerized tomography revealed anatomical futures clearly. Cardiac catheterization indicated that the pulmonary resistances indices were 2.92 Wood unit/m2 in LPA and 3.35 Wood unit/m2 in RPA, was 3.26. This patient underwent surgical correction at the age of 10 and successfully survived.

Abstract 16377: Cardiovascular Computed Tomography Can Predict Branch Pulmonary Artery Stenosis in Neonates With Ductal Dependent Pulmonary Blood Flow

Background: A subpopulation of neonates with ductal dependent pulmonary blood flow (PBF) are at risk for branch pulmonary artery (PA) stenosis in the PA that receives the patent ductus arteriosus (PDA). Identification of this population is important in planning initial and future interventions. The objective of this study was to identify novel cardiovascular computed tomography (CCT) measures in the preoperative period that are associated with the need for future branch PA surgical or catheter-based intervention. Methods: In this single center retrospective study, we identified neonates (&lt; 28 days) with ductal dependent PBF who underwent CCT prior to initial intervention from 11/2003-12/2019. A single reviewer made conventional and novel measurements of the branch PAs as well as PDA size and configuration. The primary outcome was reached if there was any initial or subsequent surgical or catheter-based intervention on the branch PA that received the PDA. Results: A total of 47 neonates (16 female, 31 male) were included. 30 (64%) reached the primary outcome. Upon multivariable regression analysis, PDA origin at the aortic isthmus [OR 7.13 (95% CI 1.01-50.4), p = 0.049] and distance to the largest diameter (DLD) (Figure 1) [OR 1.24 (95% CI 1.01-1.51), p = 0.040] were independently associated with the primary outcome. DLD is the distance of the largest diameter of the PA with the PDA from the bifurcation in confluent PAs or from the insertion of the PDA in discontinuous PAs. On receiver operating characteristic analysis, area under the curve for DLD = 0.75, p = 0.01. A DLD &gt; 6 mm displayed 82% sensitivity and 67% specificity in identifying patients who met the primary outcome. Conclusions: PDA origin at the aortic isthmus and DLD can risk stratify patients at high risk for requiring surgical or catheterization-based branch PA interventions. Further analysis is needed to determine if intervention strategies based on these measurements improve patient outcomes.

Percutaneous salvage interventions in the Glenn circuit: a case series

BackgroundIn majority of children bidirectional Glenn shunt is a safe and efficacious procedure with minimal post-operative issues. Rarely, there may be dysfunction in the Glenn pathway due loss of anatomical integrity or derangements in normal physiological or hemodynamic milieu. We report 4 cases in the last 3 years (2016-2019) where complications in the Glenn circuit led to serious consequences requiring transcatheter interventions.Case presentationTwo of our patients presented with frank features of superior vena cava syndrome. One of them had right Glenn anastomotic site narrowing leading to severe obstruction along with significant left pulmonary artery origin stenosis. The other child had excessive antegrade flow impeding normal Glenn flow leading to superior vena cava syndrome. The next child in our series was initially lost to follow-up after bidirectional Glenn surgery. Later on, this child was noted to have discontinuous left pulmonary artery with perfusion only to the right lung from the Glenn. The remaining child described in this series had developed a large tortuous venous collateral post Glenn shunt leading to severe cyanosis. All the above children needed prompt percutaneous interventions to revert back to their basal state. On follow-up, the benefit was sustained in all.ConclusionsPercutaneous intervention procedures often provide a successful bailout option in various complicated situations post Glenn surgery with reasonable efficacy and safety.

Preprocedural three-dimensional planning aids in transcatheter ductal stent placement: A single-center experience.

Describe the use of three-dimensional (3D) patent ductus arteriosus (PDA) modeling to better define ductal anatomy to improve preprocedural planning for ductal stent placement. Ductal stenting is an alternative to surgical shunting in patients with ductal dependent pulmonary blood flow. Ductal anatomy is often complex with extreme tortuosity and risk of pulmonary artery isolation, thus increasing procedural risks. CT angiograms were segmented to produce 3D PDA models. Ductal morphology was characterized with attention to access approach, degree of pulmonary artery offset/risk of isolation and ductal tortuosity. 3D models were retrospectively compared with biplane angiography. 3D modeling was performed in 12 patients with adequate image quality for complete analysis in 11; median (interquartile range) age/weight 17 days (8-20 days) and 3.1 kg (2.4-3.9 kg). The PDA was reverse oriented in nine with average length of 17.2 ± 2.5 mm and high tortuosity (mean tortuosity index 52, range 3-108). From 3D modeling, two patients were excluded from ductal stenting-extreme ductal tortuosity and threatened pulmonary artery discontinuity, respectively. Ductal stenting was successful in the remaining nine with no major procedural complications. 3D modeling predicted a successful access approach based on the aortic orientation of the ductus in all patients (five carotid, two axillary, two femoral). When comparing 2D angiography with 3D models, angiography consistently underestimated ductal length (-3.2 mm ± 1.6 mm) and tortuosity (-14.8 ± 7.2). 3D modeling prior to ductal stent placement for ductal dependent pulmonary blood flow is useful in procedural planning, specifically for eligibility, access approach, and accurate ductal measurements. Further studies are needed to determine if 3D planning improves procedural outcomes.

Staged Hybrid Approach for Ductal Origin of the Pulmonary Artery in a Low Birth Weight Neonate.

Early recruitment of a discontinuous branch pulmonary artery will give the best long-term outcome for congenitally disconnected pulmonary arteries. This may be achieved using a staged approach. Even in low birth weight neonates, a hybrid approach with the first stage consisting of transcervical (via the common carotid artery) patent ductus arteriosus stenting can be performed to recruit the pulmonary artery where conventional treatment strategies may not be feasible or may be associated with higher risk.

Validity of Pulmonary Valve Z-Scores in Predicting Valve-Sparing Tetralogy Repairs-Systematic Review †.

There is a lack of consensus regarding the preoperative pulmonary valve (PV) Z-score “cut-off” in tetralogy of Fallot (ToF) patients to attempt a successful valve sparing surgery (VSS). Therefore, the aim of this study was to review the available evidence regarding the association between preoperative PV Z-score and rate of re-intervention for residual right ventricular outflow tract (RVOT) obstruction, i.e. successful valve sparing surgery. A systematic search of studies reporting outcomes of VSS for ToF was performed utilizing PubMed, EMBASE, and Scopus databases. Patients with ToF variants such as pulmonary atresia, major aortopulmonary collaterals, absent pulmonary valve, associated atrioventricular septal defect, and discontinuous pulmonary arteries were excluded. Out of 712 screened publications, 15 studies met inclusion criteria. A total of 1091 patients had surgery at a median age and weight of 6.9 months and 7.2 kg, respectively. VSS was performed on the basis of intraoperative PV assessment in 14 out of 15 studies. The median preoperative PV Z-score was −1.7 (0 to −4.9) with a median re-intervention rate of 4.7% (0–36.8%) during a median follow-up of 2.83 years (1.4–15.8 years). Quantitatively, there was no correlation between decreasing preoperative PV Z-scores and increasing RVOT re-intervention rates with a correlation coefficient of −0.03 and an associated p-value of 0.91. In observational studies, VSS for ToF repair was based on intraoperative evaluation and sizing of the PV following complete relief of all levels of obstruction of the RVOT, rather than pre-operative echocardiography derived PV Z-scores.

Catheterization and surgical collaboration in the treatment of complex congenital heart disease with discontinuous pulmonary arteries.

A term, male neonate presents with pulmonary atresia, discontinuous branch pulmonary arteries (PA) arising from independent ducti arteriosus, and dextrocardia presents a challenging treatment plan. The right pulmonary artery (RPA) arose from the ascending aorta while the left pulmonary artery (LPA) arose from the left subclavian artery, with the proximal segments of each spaced about 1 cm apart, making primary unifocalization suboptimal. The cardiac anatomy included: dextrocardia with situs inversus, double outlet right ventricle (DORV) with malposed great arteries and pulmonary atresia, left sided inferior and superior venae cava, and a right aortic arch with mirror image branching. The neonate underwent cardiac catheterization on day of life 12 wherein the bilateral patent ducti arteriosus (PDA) were stented as an alternative to high-risk surgery. In addition, the pulsatile flow via the stents provided a stimulus for PA growth, facilitating transcatheter rehabilitation of distal PA hypoplasia, and optimizing later PA reconstruction. The patient underwent surgery at 13 months of age, with autologous tube creation to unifocalize the Pas and enable the Glenn procedure. Postoperative autograft stenosis was later managed via cardiac catheterization. This report describes the clinical management and utility of staged percutaneous intervention for complex anatomy.

Fate of Duct-Dependent, Discontinuous Pulmonary Arteries After Arterial Duct Stenting.

Significant and balanced PA growth following arterial duct (AD) stenting has already been consistently reported in literature. However, to date, no data are available about the role of this approach as palliation of congenital heart disease with a duct-dependent discontinuous pulmonary artery (dPA). The aim of this study was to evaluate the fate of a dPA of ductal origin following trans-catheter AD stabilization. Angiographic PA evaluation was performed in seven patients submitted to neonatal AD stenting as palliative recruitment of dPA. Five patients showed discontinuity of one PA, while two patients had both PAs served by bilateral ducts. PA growth was evaluated as per the Nakata index, McGoon ratio as well as dPA (n=9) versus heart-dependent PA (hPA; n=5) size and z-score changes. AD stabilization was performed using coronary stents dilated to 3.2±0.3mm (median 3.4), with significant increase of O2 saturation (from 83±11 to 95±5%, p<0.02). Control angiography was performed 5.1±2.8months (median 6 months) after duct stenting, showing significant growth of the dPA (from 3.7±1.0 to 7.6±2.7mm, p<0.001; z-score from -0.7±1.4 to 1.7±2.2, p<0.01). A trend toward better growth of the dPA as compared with the hPA was found (117±87 vs. 54±34%, p=NS). The final vessel size was still significantly different between the groups (dPA 7.6±2.7 vs. hPA 11.9±3.4mm, p=0.02), although the final z-score value did not significantly differ (dPA 1.7±2.2 vs. hPA 3.8±0.9mm, p=NS). In conclusion, percutaneous AD stenting is effective in promoting a significant catch-up growth of duct-dependent dPA, being, therefore, advisable as a reliable alternative to surgical palliation.

53. Bilateral ductal stenting for nonconfluent pulmonary arteries in a newborn

Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Limited data available for bilateral PDA stenting. Bilateral PDA stenting in nonconfluent pulmonary arteries is challenging procedure but can be considered as an option in the management of complex conditions like this. 12 days old Preterm (36 weeks gestation) male baby with birth weight of 2.6 kg developed respiratory distress with severe cyanosis and desaturation upto 50%. Baby was intubated and started on Prostaglandin 0.05 mic/kg/mt. His saturation improved to 80%. Echocardiogram showed complex cyanotic heart disease, Situs ambiguous, dextrocardia, complete unbalanced AV septal defect, pulmonary atresia , nonconfluent small branch pulmonary arteries supplied by the bilateral patent ductus arteriosus (PDA) from right aortic arch and all four pulmonary veins form a confluence and drain into superior vena cava(SVC )through vertical vein with no obstruction. Baby was taken up for PDA stenting. descending aortogram showed right aortic arch with vertical tortuous duct to right pulmonary artery (RPA) and another short duct with acute angle from left subclavian artery to left pulmonary artery (LPA ). Both ducti stented with coronary stents. Vertical vein angiogram showed both lungs drain to a confluence and then to SVC via ascending vertical vein with no obstruction. After stenting lung perfusion improved and the baby was stable and maintained 80% saturation on room air. Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Our case is unique with Heterotaxy, TAPVC, Dextrocardia and double ducti. Eventhough bilateral ductal stenting is technically challenging it is successful through femoral artery approach.