Major Aortopulmonary Collaterals Research Articles

The Indispensable Role of Cardiac Catheterization Before Surgical Intervention of Pediatrics Undergoing Tetralogy of Fallot Total Correction; A Single Center Experience

Background: Inadequate pulmonary blood flow in the tetralogy of Fallot (ToF) can lead to the development of major aortopulmonary collaterals, interfering with surgical repair. Objectives: We aimed to depict the crucial role of cardiac catheterization before the surgical intervention of pediatrics undergoing ToF total correction. Methods: This retrospective cohort study was conducted on consecutive pediatric patients under two years of age who underwent ToF total correction surgery. All of the patients were diagnosed with ToF with doubled times trans-thoracic echocardiography, and the patients with high suspicion of the presence of major aortopulmonary collateral arteries (MAPCAs) underwent cardiac catheterization before ToF total correction surgery. Conversely, if the catheterization approach failed, they underwent ToF total correction surgery simultaneously with MAPCA closure. On the other hand, the patients with no evidence of MAPCA in the echocardiographic findings (as considered undiagnosed patients) underwent ToF total correction surgery. Results: Two hundred fifty eligible pediatrics who underwent ToF total correction surgery participated. The ICU stay (P < 0.001), hospital stay (P < 0.001), and weaning time from mechanical ventilation (P < 0.001) in patients with correct diagnosis before the ToF surgery were statistically lower than misdiagnosed open MAPCA patients who underwent the surgical approach of the ToF group. Some intra-operative and postoperative parameters showed no statistical differences between patients in the two groups. We had three mortality in the patients whose trans-thoracic echocardiography (TTE) findings were undiagnosed as there was no evidence to rely on the presence of open MAPCA. Conclusions: Based on this study's findings, we strongly recommend that all ToF patients undergo cardiopulmonary catheterization before the surgical approach.

Double Outlet Right Ventricle with Pulmonary Atresia and Antero-Posterior Malposition of Great Arteries: Transthoracic Echocardiographic Evaluation of a rare cyanotic congenital heart disorder

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle, This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We are reporting a case of seven year old deeply cyanotic child with DORV, subaortic VSD with rare anatomic features of antero-posterior malposition of great arteries (GA) and pulmonary vavular atresia, with conspicuous absence of patent ductusarteriosus (PDA) or major aorto-pulmonary collaterals (MAPCAs), despite the presence of atretic pulmonary valve. Keywords: DORV, Pulmonary atresia, TGA, VSD, subaortic VSD, overriding of Aorta, cyanotic congenital heart defects.

Major Aortopulmonary Collaterals From Coronary Arteries and Dual Left Anterior Descending Artery in an Adult With Tetralogy of Fallot.

Major aortopulmonary collaterals (APCs) are more common with tetralogy of Fallot (TOF) with pulmonary atresia. Collateral arteries, when present, arise most commonly from the descending thoracic aorta, less commonly from the subclavian arteries, and rarely from the abdominal aorta or its branches or from the coronary arteries. Collaterals arising from the coronary arteries can cause myocardial ischemia due to coronary steal phenomenon. They can be addressed either by endovascular interventions like coiling or surgical ligation during the intracardiac repair. Coronary anomalies are seen in 5%-7% of TOF patients. In approximately 4% of TOF patients, the left anterior descending artery (LAD) or an accessory LAD takes origin from the right coronary artery or right coronary sinus and crosses the right ventricular outflow tract in its course toward the left ventricle. Presence of such anomalous coronary anatomy poses certain challenges during intracardiac repair of TOF.

Transcatheter Closure of a Coronary Artery Fistula in a Neonate with Tetralogy of Fallot.

Coronary artery-to-pulmonary artery fistulae are a not uncommon finding in patients with Tetralogy of Fallot (TOF) and collateral-dependent pulmonary blood flow. Management for these fistulae is often primary surgical ligation or unifocalization at the time of complete repair, dependent on the presence of dual blood flow to the involved areas. We present the case of a 32-week premature boy weighing 1.79kg with TOF, confluent branch pulmonary arteries, major aortopulmonary collaterals, and right coronary artery to main pulmonary artery fistula. The patient demonstrated evidence of coronary steal into the pulmonary vasculature with an elevation in the troponin level without hemodynamic instability, and subsequently underwent successful transcatheter occlusion of the fistula via right common carotid access using a Medtronic 3Q microvascular plug. This case demonstrates the realistic potential for early coronary steal in this physiology and possibility of transcatheter therapy even in a small neonate.

Abstract 10090: Computed Tomography is Non-Inferior to Cardiac Angiography in Differentiating Dual- versus Single- Supply Major Aortopulmonary Collaterals Among Patients With Pulmonary Atresia or Critical Stenosis

Introduction: Anatomic characterization of pulmonary blood flow is of paramount importance in patients with major aortopulmonary collaterals (MAPCAs). Although computed tomography angiography (CTA) is an appealing alternative to cardiac angiography (CA), its ability to differentiate between single- versus dual-supply MAPCAs is not well-established. We investigated whether CTA is non-inferior to CA in defining MAPCAs supply. Methods: Retrospective analysis of patients who underwent both CTA and CA for MAPCA delineation between 2016-2021. Diagrams were created for patients labeling MAPCAs numerically for consistency in data collection. Images were independently reviewed by blinded CTA and CA readers. The diagnostic performance of CTA was compared to CA as the gold standard. Nonparametric paired tests were used for statistical analysis, and 95% confidence intervals were calculated using exact methods. Results: Seventeen patients, with 55 MAPCAs, met the study criteria. The median age at CTA and CA was 7 days (1-745) and 74 days (7-751), respectively. CA was performed under general anesthesia (GA) in all patients compared to 17% of CTAs (p=0.0001). Contrast load (median 2 vs 7.1 ml/kg) and adverse events (0 vs 47%) were lower in CTA than diagnostic CA (p <0.0001 and p.=0.008, respectively). Fifty-three MAPCAs (96%) were concordantly classified as single or dual supply (95% CI, 87-100%). Compared to CA, CTA detected dual-supply MAPCAs with sensitivity of 97% (95% CI, 84-100%) and specificity of 96% (77-100%); similarly, PPV was 97% (84-100%) and NPV was 96% (77-100%). The proportion of MAPCAs labeled discordantly was non-inferior at both the 10% margin (p=0.0001) and 5% margin (p=0.031). Conclusions: CTA is accurate in differentiating single- versus dual-supply of MAPCAs. Additionally, CTA required a lower contrast load, less frequent use of GA, and resulted in fewer adverse events. Our results suggest that CTA may be used as the initial diagnostic modality for complete MAPCAs delineation, with CA reserved when an intervention or hemodynamic assessment is necessary.

Adult outcomes of complex pulmonary atresia, ventricular septal defect and major aortopulmonary collaterals following repair during childhood, a single centre experience

Abstract Background Complex pulmonary atresia with a ventricular septal defect and major aortopulmonary collateral arteries (PA VSD MAPCA) is a rare and complex form of congenital heart disease. Staged surgical palliation is carried out in childhood, and in expert centres, is associated with good medium term survival. Adult outcomes following transition to adult care are not known. Purpose To establish adult outcomes of PA VSD MAPCA following repair during childhood in our institution Methods Review of clinical records for outcome data including life status, NYHA class, employment status, re-intervention in adult life, arrhythmia, pregnancy, exercise capacity and mode of death, where known. We predefined our population into two groups based on their status at transition to adult care, A) complete repair with RV-PA conduit and VSD closed and B) repaired but VSD left open. Results 188 patients from our series have transitioned to adult services. As many were tertiary referrals (nationally and internationally) the local follow up population was 53 adults (54.7% male), mean age of those alive under follow up is 27.2±6.5 years, with a mean follow up of 25.3±6.1 years. There were 43 in group A and 10 in group B. Mortality in the total population was 18.9% (10/53), group A (16.3%), group B (30.0%), mean age at death 37.5±10.3 years and 29.9±8.4 years, 6/10 deaths were sudden. 22.6% of the whole group had DiGeorge/22q11 microdeletion, with a learning deficit in 32.7% of the cohort, where this could be assessed. Reinterventions included RV-PA conduit change, coil occlusion, pacemaker implantation, ICD implantation and ablation procedures, overall 39.6% underwent reintervention in adult life. Pregnancy occurred in 33.3% of 24 female patients. 50% of individuals were found to have undertaken some form of paid work and of these 11.5% had DiGeorge. Conclusions Complex PA VSD MAPCA is associated with good late survival with late attrition including sudden death and heart failure. 22q11 is associated with learning deficit and consequent lack of employment. Non 22q11 have good educational outcomes and there is less observed deficit regarding employment. Successful pregnancy is possible within this population. Funding Acknowledgement Type of funding sources: None.

The angiographic study of right ventricular outflow tract and pulmonary artery anatomy in tetralogy of Fallot

Objective: The objective of the study is to determine various anatomic variations in the pulmonary vasculature and associated cardiac defects in patients with tetralogy of Fallot (TOF). Methods: This was a cross-sectional descriptive study conducted at Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore from January 2009 to December 2017. A total of 100 patients irrespective of age and gender, who were subjected to cardiac catheterization were enrolled in the study which included all age groups. Measurement of pulmonary valve annulus, main pulmonary artery, right pulmonary artery (RPA), left pulmonary artery (LPA), and descending aorta was taken and corresponding z scores were calculated. McGoon ratio and Nakata index were also calculated. Results: The age group in our study varied from 9 months to 49 years of age. Male-to-female gender ratio was 3:2. All patients had subvalvar (infundibular) stenosis and 82% of patients had stenosis at the valvar level. Seven patients had discrete stenosis, another seven patients had disconnection of RPA or LPA and six patients had supravalvar stenosis in the form of diffuse hypoplasia of one of the pulmonary arteries (PA). Significant communicating major aortopulmonary collaterals arteries (MAPCAs) were seen in 5% of patients and 16% of the patients had a persistent patent ductus arteriosus (PDA). Persistent left superior vena cava was present in 9 patients and 13% of the patients had coronary anomalies. Conclusion: Subvalvular stenosis, confluence of PA, discrete, disconnected, and diffuse stenosis of LPA and RPA were the most common PA abnormalities found in patients with TOF. Significant-associated cardiac lesions including communicating MAPCA, PDA, and coronary anomaly were more commonly observed in these patients.

Major aortopulmonary collateral arteries in association with atrioventricular septal defect with balanced ventricles and trisomy 21.

A major aortopulmonary collateral artery is a rare and easily missed diagnosis that is usually associated with Tetralogy of Fallot or pulmonary atresia. We present two cases of major aortopulmonary collaterals associated with trisomy 21 and atrioventricular septal defect with balanced ventricles in which the diagnosis went undetected until after initial cardiac repair.

The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a complex congenital heart defect that includes a heterogeneous subgroup of patients. Variation in the sources of pulmonary blood flow contributes to the complexity of the lesion and the diversity of approaches to its management. Unifocalization and rehabilitation focus on mobilization of collateral arteries and growth of native pulmonary arteries, respectively, with the ultimate surgical goal of achieving separated systemic and pulmonary circulations with the lowest possible right ventricular pressure. Regardless of the strategy, outcomes have altered the natural history of the disease, with a complete repair rate of approximately 80% and low early and late mortality rates. Given this heterogeneity of pulmonary vasculature, a tailored approach should be adopted for each patient, using all diagnostic methods currently offered by technical developments.

Major Aortopulmonary Collateral Arteries Requiring Percutaneous Intervention Following the Arterial Switch Operation: A Case Series and Systematic Review.

Background: Dextro transposition of the great arteries (d-TGA) is the most common critical congenital cardiac defect surgically treated in the neonatal period by arterial switch operation (ASO). Major aortopulmonary collaterals (MAPCAs) can be present in this population and may complicate the early postoperative period. Our aim was to review our institutional data and systematically review the available literature to provide further insight on the clinical significance of MAPCAs during the early postoperative course after ASO. Methods: This is a retrospective study of patients with simple d-TGA who underwent ASO between March 1998 and September 2020 at Boston Children's Hospital. The MEDLINE, Embase, and Cochrane databases were searched from inception to June 2020. Results: Of the 671 d-TGA patients who underwent ASO at our center, 13 (1.9%) were diagnosed with MAPCAs. Five were diagnosed before ASO, while eight were diagnosed after ASO. Of these, two patients required catheterization for MAPCAs coiling during the same hospitalization on the 2nd and 11th postoperative days. The systematic review retrieved a total of 34 articles after duplicates were removed. Finally, nine studies reporting on 23 patients were deemed eligible for our analysis. The average time to MAPCAs coiling was 12 days, while the mean hospital stay was 36 days. Conclusions: MAPCAs should be included in the differential diagnosis of ASO complicated by cardiac or respiratory failure, or pulmonary hemorrhage acutely postoperatively. Once managed, recovery of these patients is predictable, and mortality is low. Further studies investigating the diagnostic value of echocardiography and the long-term outcomes of these MAPCAs are necessary.

Repercussions of a giant major aortopulmonary collateral aneurysm adjacent to vertebral artery origin.

ABSTRACTAn aneurysm of major aortopulmonary collateral in an adult with congenital cyanotic heart disease was detected incidentally following a hemoptysis episode. The location and size of the aneurysm needed special concern during treatment to avoid aneurysm-related complications and thromboembolism secondary to intervention.

OUTCOMES OF PALLIATIVE RIGHT VENTRICLE TO PULMONARY ARTERY CONNECTION FOR TETRALOGY OF FALLOT WITH PULMONARY ATRESIA AND MAPCAS

<h3>BACKGROUND</h3> Tetralogy of Fallot with pulmonary atresia with major aorto pulmonary artery collaterals (TOF-PA MAPCAS) is a rare and complex cyanotic congenital heart malformation with a high incidence of early mortality and morbidity. Different surgical interventions have been described including construction of a systemic to pulmonary artery shunt (Modified Blalock-Taussig shunt or central shunt) and unifocalization, however the perioperative mortality remains significant due to the high incidence of thrombosis, obstruction, and pulmonary artery (PA) distortion. Another alternative is a palliative right ventricle to pulmonary artery connection (RVPAC) to bring antegrade flow to diminutive PAs. The objective of our study was to evaluate the impact of RVPAC on mortality, survival, morbidity, PA growth and eligibility for biventricular repair. We believe that the RVPAC approach allows for better PA growth and obviates the need for a conduit at the time of complete repair. <h3>METHODS AND RESULTS</h3> We performed a retrospective single-institution study from January 2016 to March 2021 that included 15 consecutive patients diagnosed with TOF-PA MAPCAS who underwent an RVPAC at CHU Sainte-Justine as their initial palliation. Median age at palliation and complete repair was 18 (4 -100) and 309 (96 – 1044) days respectively. Mortality after a RVPAC was 0 % with a median hospital length of stay of 14,5 days (6 - 55), while the late interstage mortality rate was 0 %. Overall survival was 100 %. 9 patients (60%) had a biventricular repair, and 6 patients (40%) are awaiting repair. RVPAC allowed significant growth of the native pulmonary arteries with a mean Nakata index at CT scan of 70 ± 48,04 mm2/m2 before the RVPAC and 251 ± 121,05 mm2/m2 after (P = 0.001). 4 patients (44%) required reinterventions after completed repair by catheterization for stenotic pulmonary artery branches, while no patients required a late reoperation. 8 patients (53,33%) had embolization of collaterals and none required unifocalization. <h3>CONCLUSION</h3> This reproducible and safe technique allows for excellent growth of the PA tree. Biventricular repair was possible in most cases without the use of a conduit or unifocalization of collaterals. This technique appears to be suitable for any type of TOF-PA MAPCAS with confluent native branch pulmonary arteries.

Neonatal or infantile palliation for Tetralogy of Fallot with or without pulmonary atresia: Modified Blalock–Taussig shunt versus restrictive right ventricle to pulmonary artery connection. A 20-year experience

To compare mortality, morbidity and efficiency of modified Blalock–Taussig shunt (mBTS) versus restrictive right ventricle to pulmonary artery connection (RVPAc) in patients with Tetralogy of Fallot (TOF) and TOF with pulmonary atresia (TOF-PA). We performed a monocentric retrospective review of 198 patients (93 TOF and 105 TOF-PA) who had palliation between 1996 to 2016 in their first year of life. Patients with Major aorto-pulmonary collaterals were excluded. Median follow-up was 11.0 [95% CI: 5.8–16.2] years after palliation. Five patients were lost at the follow-up. Ninety-five patients underwent mBTS, and 103 had an RVPAc. Nakata index before palliation was 129 (80–170) mm 2 /m 2 in mBTS group and 100 (71–146) mm 2 /m 2 in RVPAc group ( P = 0.001). Weight at palliation was similar between mBTS and RVPAc patients ( P = 0.1). Age at the palliation was 12 days (7–24.7) in RVPAc group and 21 days (8–87.5) in mBTS group ( P = 0.0002). One hundred and seventy-five patients underwent repair and 5 are waiting for it. Among them 39 necessitated a repair with a pulmonary valve conduit (23 mBTS and 16 RVPAc P = 0.004). Two patients were unsuitable for repair. Eleven patients died before repair (5.7%). There was no difference in mortality rate regarding the type of palliation ( P = 0.92) or the anatomical group ( P = 0.76). Interstage reintervention before repair was n = 37 (20%). Thirty-three were surgical and four were percutaneous. No difference in reintervention rate between RVPAc or mBTS regarding the age, the weight at palliation or the anatomical group ( P = 0.56). The delay between the palliation and the complete repair was shorter in RVPAc [12.3 (6.3–37.9) months versus 6.5 (4.9–10.3) months for mBTS ( P < 0.01)]. RVPAc provide a better and a faster pulmonary artery growth in our population. It led us to limit the use of RVPAc conduit at the repair. We found no significant difference between mBTS and RVPAc for mortality and interstage reintervention.

Computed tomography in tetralogy of Fallot: pre- and postoperative imaging evaluation.

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease (CHD) and the most frequent complex CHD encountered in adulthood. Although children with TOF share four characteristic features (subaortic ventricular septal defect, overriding aorta, right ventricular hypertrophy, pulmonary stenosis), the clinical spectrum and course are in fact greatly heterogeneous. Echocardiography remains the mainstay for diagnosis, presurgical planning and postoperative follow-up. However, with continued technological advances, CT now plays an increasing role in TOF evaluation and management, helping to minimize routine invasive catheter angiography. Preoperatively, CT is uniquely suited to assess associated pulmonary arterial, aortic and coronary anomalies as well as extra-cardiovascular structures and is particularly helpful for delineating complex anatomy in the TOF subtypes of absent pulmonary valve and pulmonary atresia with major aortopulmonary collaterals. Postoperatively, CT is useful for identifying surgical complications and for long-term monitoring including volumetry quantification, especially in children for whom MRI is contraindicated or limited by implanted devices such as pacemakers and stents. In this article, we review key clinical features and considerations in the pre- and postoperative TOF patient and the burgeoning role of CT for facilitating accurate diagnosis and personalized intervention.

Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia.

Tetralogy of Fallot with pulmonary atresia (ToF-PA) is a rare diagnosis that includes an extraordinarily heterogeneous group of complex anatomical findings with significant implications for physiology and prognosis. In addition to the classic findings of ToF, this particular diagnosis is characterized by complete failure of forward flow from the right ventricle to the pulmonary arterial system. As such, pulmonary blood flow is entirely dependent on shunting from the systemic circulation, most frequently via a patent ductus arteriosus, major aortopulmonary collaterals, or a combination of the two. The pathophysiology of ToF-PA is largely attributable to the abnormalities of the pulmonary vasculature. Ultimately, these patients require operative intervention to create a reliable, controlled source of pulmonary blood flow and ideally complete intracardiac repair. Even after operative correction, these patients remain at risk for pulmonary arterial stenoses and pulmonary hypertension. Although there have been significant advances in surgical and interventional management of ToF-PA leading to dramatic improvements in survival and long-term functional status, there is ongoing debate about the optimal management strategy given the risk of development of irreversible abnormalities of the pulmonary vasculature and the morbidity and mortality associated with sometimes multiple, complex operative interventions often occurring early in infancy. This review will discuss the findings in patients with ToF-PA with a focus on the perioperative and anesthetic management and will highlight challenges faced by the anesthesiologist in caring for these patients.

Balloon Angioplasty for Pulmonary Artery Stenosis After Complete Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals.

The objective of this study was to assess procedural outcomes of balloon pulmonary artery (PA) angioplasty procedures after complete repair of tetralogy of Fallot with major aortopulmonary collateral arteries (TOF/MAPCAs). Our approach to patients with TOF/MAPCAs emphasizes early complete unifocalization and repair. Major PA reinterventions are relatively uncommon. Balloon PA angioplasty is often used, but the effectiveness of balloon PA angioplasty in this population is unknown. The study cohort comprised patients who underwent complete unifocalization and repair of TOF/ MAPCAs at our center between 2002-2018 and underwent balloon PA angioplasty after repair. To assess immediate procedural outcomes, pre- and postintervention PA measurements were compared. We reviewed 134 vessels that were dilated a median of 1.1 years after repair in 60 patients (median 2 PA branches per patient). Treated vessels included 15 central, 64 lobar, and 55 segmental branches. The median PA diameter at the level of stenosis increased from 1.9 mm to 3.3 mm (P<.001), and the median diameter increase was 50%. All but 6 treated vessels were enlarged. The stenosis-distal diameter ratio increased from a median of 64% to 89% (P<.001). The median central PA to aortic systolic pressure ratio was 47% before and 39% after intervention (P<.001). Balloon PA angioplasty was acutely effective at treating most stenoses of reconstructed PA branches after repair of TOF/MAPCAs. Simple angioplasty can be a useful tool in treating isolated or modest stenoses after unifocalization/PA reconstruction surgery using our approach.

Masquerader of Lip Angioedema After Lip Injection

A 27-year-old woman with specific antibody deficiency who was receiving immunoglobulin replacement therapy, with complex congenital heart disease (tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals), with group I pulmonary hypertension and resolved Cushing syndrome resulting from prior chronic steroid treatment, presented urgently with acute lip angioedema.

Impact of Treatment Strategy on Outcomes in Isolated Pulmonary Artery of Ductal Origin.

Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.

Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years.

Background: Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking. Methods: PAH-ACHD patients ≥ 40 years of age under active follow-up between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history, were retrieved from hospital records. The primary end-point was all-cause mortality. Results: In total, 65 patients (67.7% female, mean age 45.19 ± 6.75 years) were included. Out of these, 46 (70.8%) had a shunt lesion, 12 (18.5%) had PAH associated with complex congenital heart defects, and 7 (10.8%) had segmental pulmonary hypertension due to major aorto-pulmonary collaterals. Down syndrome was present in 13 patients (20.0%). During a median follow-up of 4.2 years (IQR 1.2–7.5), 16 patients (24.6%) died. On univariate analysis, NT-proBNP (log), creatinine, and a previous history of ventricular arrhythmias were predictors of all-cause mortality. Upon multivariate analysis, NT-proBNP (log) (HR: 4.1, 95% CI: 1.2–14.4, p = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2–118.7, p = 0.006) remained as independent predictors of all-cause mortality. Conclusions: PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors.

Outcomes of management of major aortopulmonary collaterals for pulmonary atresia and ventricular septal defect.

This study aimed to investigate the association between long-term survival and different management of major aortopulmonary collateral arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. From November, 2009 to October, 2018, a total of 98 consecutive patients with pulmonary atresia, ventricular septal defect, major aortopulmonary collateral arteries, and hypoplastic pulmonary arteries treated with modified Blalock-Taussig shunt or right ventricle-pulmonary artery connection were included. Fifty-five patients who received occlusion or ligation of major aortopulmonary collateral arteries during or after palliative procedure were occlusion group, and the other 43 patients were no occlusion group. The early and late outcomes were compared. The mean duration of follow-up was 30.9 months in no occlusion group and 49.8 months in the occlusion group (p < 0.001). Multivariate analysis showed that only no occlusion of major aortopulmonary collateral arteries was predictive of total mortality (Hazard Ratio: 4.42, 95% CI: 1.27 to 15.42, p = 0.02). The Kaplan-Meier survival curves confirmed that patients without occlusion of major aortopulmonary collateral arteries demonstrated worse survival as compared with the occlusion group (p = 0.013). The Kaplan-Meier survival curves of patients who underwent different palliative procedures showed no differences. For patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries when a primary repair is not feasible, those without occlusion of major aortopulmonary collateral arteries have a higher risk of death following an initial palliative procedure compared with patients who underwent occlusion of major aortopulmonary collateral arteries. The occlusion of major aortopulmonary collateral arteries is not associated with a higher rate of complete repair or better improvement of pulmonary artery growth.