2025 AATS专家共识文件：肺动脉闭锁伴室间隔完整患者的管理

共识综合与多学科· 未分类与其他 · 2025

2025 American Association for Thoracic Surgery Congenital Cardiac Surgery Working Group-Expert consensus document on the management of patients with pulmonary atresia with intact ventricular septum

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James Jaggers, MD, a David Winlaw, MD, FRACS, b Stephanie Fuller, MD,Neeta Sethi, MD,Lazaros Kochilas, MD, e Iki Adachi, MD, Matthew Stone, MD, a Lorna Browne, MD, a Nee Khoo, MD eduare da Cruz, MD, chirother Petit, MD, damian Lapar, MD, MSc, Karen Stout, MD,k Mary Donofrio, MD, FAAP, FACC, FASE, and James St. Louis, MD, FACCS

ABSTRACT

Background: Pulmonary atresia with intact ventricular septum(PAIVS) presents significant morphologic and pathophysiologic variation. Outcomes depend on indi-vidual patient characteristics and the determination of appropriate triage for neonatal catheter-based and surgical therapy. Therapeutic interventions and prac-tice patterns vary significantly. There is currently no consensus on many diagnostic and therapeutic options in clinical care, and sizeable gaps in evidence exist.

Methods: The American Association for Thoracic Surgery Congenital Clinical Practice Standards Committee identified PAIVS as a complex lesion for which practitioners could benefit from an expert consensus document to provide management guidelines.This document focuses on the diagnosis, triage, identification and timing of optimal surgical or interventional strategies, and postinterventional care of patients with PAIVS.

Results/Conclusions: In this document, we identify gaps in knowledge in the care for PAIVS and provide evidence-based and expert-derived consensus statements with avail-able strength of class of recommendation and level of evidence with supporting data and discussion. Section 1 addresses the current state of fetal diagnosis and manage-ment and the potential indications for fetal interventions. Section 2 discusses postnatal diagnosis and anatomic characterization, including right ventricle, tricuspid valve,and coronary circulation, as well as the appropriate triage of patients for initial management strategies. Section 3 concentrates on the relative efficacy of procedures to establish pulmonary blood flow and decompress the right ventricle when indicated. We also examine the appropriate use of mechanical circulatory support and indications for heart transplantation in patients with the most severe form of PAIVS. Finally, Section 4 focuses on long-term outcomes, especially with single-ventricle palliation and ongoing risk related to possible ischemia in patients with right ventricle-dependent coronary cir-culation. Much remains to be learned concerning the care of children and adults with PAIVS. Future research and careful review of outcomes will continue to guide clin-ical decision-making.(J Thorac Cardiovasc Surg 2025;170:336-52)

![](2/aj0YEe4oUhYkBdfpda0fYP/figurebbc3e8ee7adf46ac9a8306a5b5d6cd78_0_9.png) PAIVS: Definitively not so simple.

CENTRAL MESSAGE

PAIVS congenital cardiac defect represents a wide spectrum of pathologic morphology, varied diagnostic modalities, and treat-ment strategies. Outcomes vary with the severity and associated RVDCC.

PERSPECTIVE

Pulmonary atresia with intact ventricular septum(PAIVS) occurs with significant morphologic and pathophysiologic variation. Outcomes depend on the risk profile and appropriate management deci-sions. Practice variation between centers and indi-vidual providers exists, with significant gaps in evidence supporting specific treatment pathways.

From the Department of Cardiothoracic Surgery, Children's Hospital Colorado,Aurora, Colo, b'Division of Cardiovascular-Thoracic Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Ill,‘Department of Pediatrics, Duke University Hospital, Durham, NC; 4Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa; Department of Pediatrics, Children's Healthcare of Atlanta, Atlanta, Ga; Division of Congenital Heart Sur-gery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex; Department of Pediatrics, Stollery Children's Hospital, Edmonton, Alberta, Canada; \{Division of Pediatric Cardio-vascular Critical Care, Boston Children's Hospital, Boston, Mass; ${}^{\prime}$ Department of Pediatrics, Columbia University Vagelos College of Physicians \& Surgeons New York, NY; Department of Cardiovascular Surgery, University of Texas M-6Govern Medical School, Houston, Tex; ${}^{\text{division of Cardiology, University of} 2025}$ and ${}^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ . ${}^{\text{2025; 2025;}}$ and $^{\text{36.0}}$ in $W_{\text{G}}$ .

National Hospital, Washington, DC; and Department of Surgery and Pediatrics,Children's Hospital of Georgia,Augusta University,Augusta,Ga.

Drs Jaggers and Winlaw contributed equally to this article and share first authorship.Read at the 105th Annual Meeting of The American Association for Thoracic Sur-gery, Seattle, Washington, May 2-5, 2025.

Received for publication Feb 2, 2025; revisions received March 28, 2025; accepted for publication March 31,2025; available ahead of print May 2,2025.

Address for reprints: James Jaggers, MD, Congenital Cardiac Surgery, Children's Hospital Colorado, 13123 E 16th Ave, B200, Aurora, CO 80045(E-mail:james.jaggers@childrenscolorado.org).

S0022-5223/$36.00

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# Abbreviations and Acronyms

AATS= American Association for Thoracic Surgery

CoR= class of recommendation

CPB= cardiopulmonary bypass

CT = computed tomography

CTA= computed tomography angiography

LD=limited data

LoE= level of evidence

NR= not randomized

PA = pulmonary artery

PAIVS= pulmonary atresia with intact ventricular septum

PBF= pulmonary blood flow

PDA=patent arterial duct

PV= pulmonary valve

RV = right ventricular

RVD = right ventricular decompression

RVDCCC=right ventricle-dependent coronary circulation

RVOT= right ventricular outflow tract

TV = tricuspid valve

VAD= ventricular assist device

To view the AATS Annual Meeting Webcast, see the URL next to the webcast thumbnail.

Pulmonary atresia with intact ventricular septum(PAIVS) is a rare congenital heart disease comprising fewer than 1% of all heart defects. The anomaly was first described by Hunter in 1783 as reported in a publication by Peacock in 1869.19 This condition is characterized by membran atresia of the pulmonary valve(PV) or muscular atresia of the right ventricular outflow tract(RVOT), both accompanied by varying degrees of hypopla-sia of the tricuspid valve(TV) and right ventricular(RV)cavity. Morphologic variability of the right ventricle and PV size and right ventricle-dependent coronary circulation(RVDCC) influence postoperative outcomes.

Severity of RV hypoplasia is associated with abnormal coronary artery formation, especially in the presence of a competent TV. The same cardiac morphology and clinical pathophysiology can also result in patients with rudimentary patency of the RVOT. Embryologically, PAIVS occurs after the completion of ventricular septal formation. Compared with muscular atresia, membranous pulmonary atresia is

generally associated with more normal development of the right ventricle and has a better long-term prognosis. Membra-nous PA is less likely to be associated with abnormalities of the coronary artery development. There is no interventricular communication in PAIVS, and there must be obligatory right-to-left atrial communication for the systemic venous return to reach the systemic ventricle to generate adequate cardiac output. Development of the coronary arteries involves the formation of sinusoidal connections of the RV cavity to the epicardial surface. The fistulous communications then regress, leaving epicardial coronary channels that later coa-lesce into vessels and connect to the aorta.

In some cases, stenosis or complete occlusion of the cor-onary arteries occurs proximal to the fistulous connection.In these cases, the coronary artery perfusion is considered dependent on the high intracavitary RV pressure for perfu-sion of the myocardium in the distribution of the coronary artery(RVDCCC). It is thought that the hypertensive, hypo-plastic ventricles result in the persistence of the fistulous connection with a greater incidence of abnormal connec-tions between the right ventricle and coronary arteries.However, there is also the alternate view that abnormal cor-onary development may precede the development of RV and typoplasia. Either way, the presence of RVDCC is asso-ciated with cardiac ischemia, and the prognosis of PAIVS is related to the extent or the number of coronary arteries that are dependent on the RV as a surrogate for vulnerable myocardium.2-5 Interestingly, Ebstein malformation of the TV with tricuspid regurgitation occurs in approximately 8% of patients with PAIVS. These patients rarely have any evidence of RVDCC.

With improving access to prenatal ultrasound imaging and subsequent fetal echocardiography, diagnosis is often made during the prenatal period. In some cases, fetal intervention may have potential benefits, but this approach remains controversial. After delivery, pulmonary blood flow(PBF)depends on the ductus arteriosus, and prostaglandin E1 infu-sion is necessary to maintain ductal patency. Characterization of the ductus arteriosus, size, and insertion site on the PA is important for initial management decisions. Echocardi-ography confirms the diagnosis and characterizes important details, including the size of the interatrial communication,degree of hypoplasia and function of the TV, degree of RV hy-poplasia, and type of pulmonary atresia, either simple plate-like valvar atresia or atresia in combination with infundibular muscular atresia of the RVOT. Finally, the coronary circula-tion must be characterized to assess the risk for cardiac ischemia and to triage to a management strategy.

Although echocardiography may detect coronary abnor-malities, they are best characterized by coronary angiography.Computed tomography(CT) imaging of the coronary arteries can be accomplished in even small infants and may eventually provide sufficient information that might avoid the need for invasive angiography. Because PBF is dependent upon ductal

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patency after delivery, newborns may become quite ill postna-tally with ductus closure, with severe hypoxemia and hemodynamic instability reported. Intervention is necessary to provide a source of PBF either from the RV or by ductal stenting or aortopulmonary shunt.

In patients who have either inadequate antegrade PBF after RV decompression or who are not candidates for RV decom-pression, an alternative source of PBF is necessary. This can be accomplished by a surgical aortopulmonary shunt or a catheter-based ductus stent.° In recent years, the trend has been to use less-invasive catheter-based procedures when-ever possible. However, determining which patients will benefit from these competing interventions is still not known.After RV decompression, it may be difficult to predict which patients will ultimately have sufficient antegrade PBF and which patients will need an additional source of PBF. This determination will usually depend on anatomic limitations of the right heart structure and/or the compliance of the RV.

In some patients with severe hypoplasia of the TV and RV and/or RVDCC, it is inappropriate to decompress the right ventricle. Instead, it is most appropriate in this population to establish PBF with plans for single-ventricle palliation or trans-plantation. The long-term outcome for PAIVS ultimately depends upon the RV's capacity and function, the TV's size and function, and the presence of coronary artery anomalies.In patients who require single-ventricle palliation, ongoing risk of complications related to the Fontan circulation and po-tential risks related to coronary ischemia persist indefinitely.

Several studies have shown a gradual improvement in the survival of patients with this disorder. Multicenter series published in the early 1990s through the mid-2000s reported an approximately 80% overall discharge survival and approximately 60% 10- to 15-year survival af-ter surgical repair.10-12 Independent risk factors for poor outcomes include low birth weight, unipartite right ventricle, and RVDCC, especially atresia of the coronary ostia.11,13 More contemporary multicenter studies have shown significantly improved outcomes. In a Pediatric Car-diac Intensive Care Society study, the cumulative risk esti-mate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1 month, 6 months, 1 year, and 5 years. The TV z score was inversely associated with death or transplantation, and coronary atresia was positively associated with death or transplantation.2 In another single-center study, the overall survival at 1, 3, and 10 years was 82.4%, 80.6%, and 79.8%. In this series, lower birth weight, single-ventricle palliation, and RVDCC were predictors of poor outcomes.14

METHODS

The American Association for Thoracic Surgery(AATS) Congenital Clinical Practice Standards Committee identified PAIVS as a topic on which practitioners could benefit from an expert consensus document to provide(1) a collection of the currently available data;(2) expert interpre-tation of the data and supplementation with expert opinion where data are

lacking; and(3) identification of areas that would most benefit from future research.

This document is designed to provide information and guidance for the most clinically relevant and controversial questions in the management of pa-tients with PAIVS. This resource focuses on the diagnosis, identification of optimal surgical or interventional strategies, and triage and timing of these in-terventions. We also discuss the pertinent decision points in determining corrective 2-ventricle, 1.5-ventricle, and single-ventricle palliative strategies.Because the various strategies and triage require assessment of the coronary artery circulation, we review the pertinent diagnosis and contribution of RVDCC to the management and ultimate outcome in PAIVS.

AATS selected surgeon chairs(J.J., D.W.), who then solicited and convened a multidisciplinary panel of experts consisting of practicing pediatric cardiac surgeons, pediatric and adult congenital cardiologists, advanced cardiac imagers, and pediatric interventional cardiologists. In addition, the panel was guided and supported by AATS personnel skilled in the Delphi methodology and library sciences.An outline was created and the participants divided into subgroups to focus on specific topics. Once the final form was reached, it was reviewed by 14 additional experts in PAIVS consisting of pediatric cardiac surgeons and pediatric Cardiologists, including international experts.

Attempts were made to identify all currently available relevant data about PAIVS and its treatment. With the assistance of a medical librarian,a robust search was performed using PubMed, EMBASE, SCOPUS, and Web of Science. The search was limited to the English language and the year 1990 or later. However, it became clear that there were still very relevant studies and literature preceding this date, so when relevant,earlier literature was reviewed. Clearly, the literature search process was iterative, and additional literature was identified by reviewing the initial bibliography.

Manuscripts pertaining to pulmonary atresia with ventricular septal defect, major apotmonary collateral arteries, and pulmonary atresia associated with a hypoplastic left ventricle were excluded from this consensus document. Manuscripts identified as review articles,comments, or meta-analysis were not considered for unique state-ment creation. However,to the extent these articles contribute to the cur-rent management, they may have been considered in the overall composition of the consensus document. Meta-analyses were consid-ered when evaluating the level of evidence(LoE). All manuscripts were compiled into Excel spreadsheets(Microsoft) for group review.Greater consideration was given to more recent studies, those with larger patient populations, and those using comparison groups, espe-cially with randomization or propensity score matching. Two members read each abstract. Authors within writing sections then read every pertinent manuscript.

After reviewing these manuscripts,clinical questions were designed us-ing the patient intervention comparison outcome(ie, PICO) format. Expert consensus statements with the class of recommendation(CoR) and LoE were developed using a modified Delphi Method. Throughout this process,the entire group critically examined and revised each statement and subse-quently voted upon for validation. To consider the statement having reached a consensus, 80% of the members must have voted with 75% in agreement(voted as“agree” or“strongly agree”).15 Voting was blind but allowed for comments to clarify disagreements and assist with statement modifications. Any statement in which a consensus was not reached after 3 voting sessions was removed.

CoR is provided primarily on the basis of the size of the treatment ef-fect, the estimated size of the treatment effect, or the magnitude of benefit compared with the risk of harm.13 LoE provides an estimation of the certainty of the treatment effect on the basis of the currently avail-able data. Although the magnitude of the treatment effect was not considered for the LoE, consideration was given such that a greater LoE allowed for a greater CoR, even with only marginal benefits. As

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with the formation of the statements, consensus was required before finalizing the CoR or LoE for each statement with the same voting re-quirements. The language for each statement was chosen to be consis-tent with that recommended in the harmonizing guidelines consensus document by the AATS, European Association for Cardio-Thoracic Sur-gery, European Society of Thoracic Surgeons, and Society of Thoracic Surgeons(Fig 1). ${}^{124}$ At times, the correlating language helped to deter-mine the appropriate CoR.

Once the recommendations and their CoR and LoE were determined,the narrative was constructed to help fully inform the reader of the intent and meaning of each statement, main points and counterpoints. Further-more, the narration supplies the reader with the data used to make each recommendation. Areas lacking data and other areas for future research were noted.

LIMITATIONS AND BIAS

Like many congenital heart defects, PAIVS is a rare lesion with a highly variable morphologic presentation and different degrees of severity, treatment strategies, and outcomes. Most publications have been single-center series with heterogeneous patient populations, often covering

extended periods. The most commonly available literature is single-center case series, which are limited in defining best practices for several reasons. Although case series can provide useful information about treatment strategies and share experiences with new therapies, they lack the comparison groups necessary to effectively guide manage-ment strategies.

Also, many single-institution series introduce new therapies, often resulting in evolution in practices over time, hence limiting the utility and validity of long-term follow-up data. New surgical and interventional procedures are emerging rapidly. Short-term outcomes for most congenital heart defects have improved. By the time a study is published, new techniques have been developed. Given that the full impact of some of these variations in treatment may not be apparent for decades, this complicates the deci-sion on whether to adopt these modifications. As with other congenital heart diseases, more statistically rigorous random-ized trials in PAIVS are very difficult to design and execute.

![Class of Recommendation\nSuggested phrases for writing recommendations\nStrong recommendation\n● Is recommended\n● Is indicated\nBenefit >>> risk\n● Should be considered\n● Is reasonable\n● Can be useful/effective/beneficial\nIIa\nWeak recommendation\n● May/might be reasonable\nIIb\nBenefit≥ risk\n● May/might be considered\nModerate recommendation\nNo benefit: risk = benefit\n● Is contraindicated\nStrong recommendation\n● Should not be performed/administrated\nHarm:\nrisk > benefit\n](2/aj0YEe4oUhYkBdfpda0fYP/figure1e543c6aa52e4e75b87f94a5c90e29fb_0_9.png) ![High-quality evidence from more than one RCT with no concern for high risk of bias, or\nmeta-analyses of high-quality RCTs with no concerns for significant bias.\nRandomized\nModerate-quality evidence from 1 or more RCT or meta-analysis of\n(B-R):\nRCTS.\nNon-Randomized Moderate-quality evidence from 1 or more well-designed,\n(B-NR):\nwell-executed nonrandomized studies, observational studies,\nor registry studies or meta-analyses of such studies.\nLimited data\nStudies with limitations of design or execution, meta-analyses of\n(C-LD):\nsuch studies, case series, or physiological or mechanistic studies\nin human subjects.\nExpert opinion\nConsensus of expert opinion based on clinical experience.\n(C-EO):\n](2/aj0YEe4oUhYkBdfpda0fYP/figureeb2a80d0457b4f4e815f316b3d0a0c51_0_10.png) AATS,American Association for Thoracic Surgery;EACTS,European Association for Cardio-Thoracic Surgery;ESTS,European Society of Thoracic Surgeons; STS, Society of Thoracic Surgeons: RCT,randomized controlled trial.

FIGURE 1. Adopted definitions of classes of recommendation and levels of evidence. From Milojevic and colleagues. ${}^{124}$

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Alternative registry or database studies have nonuniform practice patterns and biases, differing center-specific out-comes, and varying degrees of experience. As a result, they lack the granularity and equipoise necessary to guide treat-ment in such a heterogeneous patient population.

DEFINITION OF RVDCC

For this consensus statement, we will use the following definition to characterize patients with important coronary artery abnormalities.

Any patient with PAIVS with any of these 3 findings related to the coronary anatomy will be considered to have RVDCC:

1. The presence of RV-coronary artery fistulae, with at least 2 of 3 major coronary arteries having severe stenosis or at least 1 coronary with complete aortocoronary atresia.

2. The presence of RV-coronary fistula and coronary artery stenosis, with a significant portion of the myocardium perfused by the RV cavity.

3. Evidence of myocardial ischemia at any point during the postnatal course.

PAIVS is commonly associated with coronary anomalies.In approximately 50% to 70% of patients, coronary sinu-soids that have persisted since fetal life directly communicate through fistulae from a hypoplastic RV to the coronary arte-rial bed. RVDCC is generally considered to be present when a significant portion of the ventricular myocardium de-pends on the right ventricle for the blood supply. In more recent studies, the true prevalence of RVDCC is unknown but reported to be between 9% and 25%. 6,16,17 In one study reviewing angiograms of patients with known PAIVS, fistulas were found in 87 patients(75%), interruptions of major cor-onary arteries in 40 patients(34%), lack of connections be-tween the coronary arteries and the aorta in 18 patients(16%), and single origin of a coronary artery, with the right coronary artery arising from the left, in 6 patients(5%). ${}^{18}$

Clinical decision-making in PAIVS depends in part upon accurate and timely assessment of the coronary arterial anatomy. It is well established that anomalies of the coro-naries may prohibit decomposition of the RV and result in significantly decreased survival. Proper diagnosis usually requires cardiac catheterization with aortic root or selective coronary artery injection. In some cases, direct RV injection will be necessary to define important stenosis or atresia of the coronary arteries. The most common description of RVDCC in the literature is“the presence of ventriculocoro-nary artery fistulae with at least 2 major coronary arteries having severe stenosis or at least one coronary with complete aortocoronatory atresia,"4,5,13 However, other de-scriptions may also be found in the literature.

Importantly, stenosis or occlusion of the coronary can be proximal or distal to the RV fistula. The position of the obstruction and not the number of coronary

arteries involved predicted poor outcomes and decreased transplant-free survival in one study.5 The authors found that in multivariable analysis, obstruction of at least 1 cor-onary artery has a hazard ratio of 3.6. In one large multicenter cohort, RVDCC of the right coronary artery only was not associated with decreased transplant-free sur-vival(Fig 2).2,124 Most practitioners would agree that the risk of cardiac ischemia associated with decompression of the RV is associated with the amount of myocardium solely perfused by the RV.13,19

Section 1.1: Fetal echocardiography should be performed whenever feasible, to assess disease progression through gestation, right heart adequacy, and may help to predict postnatal outcome of a single-ventricle or 2-ventricle circulation in fetuses with PAIVS.(CoR I LoE B-NR)

PAIVS is a morphologically and physiologically variable cardiac lesion that may evolve during gestation. There are multiple single-center studies identifying fetal echocardio-graphic markers that are predictive of postnatal outcomes.However, given possible disease progression through gesta-tion, as well as recognizing features that signify that a 2-ventricle repair is less likely, prenatal counseling remains challenging. Prenatal fetal echocardiography provides crucial diagnostic information with the characterization of the TV, RV anatomy, morphology, and functional parame-ters. Given possible disease progression through gestation, serial fetal echocardiograms are recommended to assess changes in the fetal cardiac anatomy and physiology, prog-nostate the postnatal outcome, and appropriately plan for perinatal and delivery care.

The TV annular diameter is the most predictive of an eventual successful 2-ventricle strategy, with a TV z score of-3 found to differentiate between single- and 2-ventricle repairs in several studies.20-23 In fetuses that later undergo single-ventricle palliation, the rate of TV growth through gestation has been reported to be reduced when directly compared with the growth of left heart struc-tures.20,21,24,25 TV inflow, reflecting RV diastolic filling as a surrogate for TV function and RV compliance, is another parameter to consider with a shorter TV inflow duration relative to the total cardiac cycle length seen in fetuses with subsequent single-ventricle circulation.24,25 Finally,the presence of mild-to-moderate TV insufficiency in utero is likely to stimulate RV growth and function and is predic-tive of an eventual 2-ventricle circulation.20,26,27

The RV size, morphology(bipartite vs tripartite and degree of intracavity hypertrophy), and function are important co-variables to consider in assessment of a neonate with PAIVS28 Measurements of right-sided heart structures compared with left-sided structures(tricuspid to mitral valve ratio, pulmonary to aortic valve annulus ratio,or right ventricle to left ventricle length ratio) are useful parameters to determine right heart adequacy. ${}^{20-24,26}$

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![a RVDDC\na n a nary clais\nRVDCC\n+ RVDCC\n+ right\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n+ RVDCC\n](2/aj0YEe4oUhYkBdfpda0fYP/figureda25ee5844ad416bbbf15ba61d10f6ab_0_3.png) FIGURE 2. PMID 35751685. Transplant-free survivals. Kaplan-Meier estimates of survival probabilities for patients without RVDCC(red), RVDCC and no coronary atresia(blue), RVDCC and right coronary atresia(green), and RVDCC left coronary atresia(purple). RVDCC, Right ventricle-dependent coronary circulation.124

Coronary fistulae may be identified using color Doppler while imaging the RV myocardium, although detection of these in utero does not predict the presence of significant coronary artery anomalies such as RVDCC, which must be confirmed postnatally.29

Section 1.2: In a patient diagnosed with PA/IVS, genetic counseling and genetic testing should be considered at the time of diagnosis(CoR IIa LoE C-LD)

Although most patients with PAIVS have no known genetic or chromosomal associations, there is a reported recurrence risk of right heart obstructive disease in subse-quent pregnancies.30-33 PAIVS may be secondary to associated pathogenic single-gene variants, but this is not yet fully understood.34-40 Therefore, genetic counseling should be offered at the time of diagnosis, along with possible genetic testing, including whole-exome sequencing if desired.

Section 1.3: Given that PAIVS is a ductal-dependent cardiac lesion for PBF, delivery of a fetus with PAIVS should be planned at a center that can appropriately initiate prostaglandin therapy to maintain ductal patency in the newborn with expedited transfer to a specialized pediatric cardiac center for further care.(CoR I, LoE C-LD)

Newborns with PAIVS rely on patency of the ductus arte-rirosus for PBE. Given that the ductus arteriosus remains patent for approximately 24 to 72 hours after delivery,41 neonates with PAIVS generally have minimal risk for hypoxemia or hemodynamic instability in the delivery room or during peri-natal transition. However, timely initiation of prostaglandin infusion is essential for ensuring continued ductal patency and neonatal stability. Therefore, after prenatal diagnosis of PAIVS, delivery should be planned at a center that can obtain secure intravenous access(umbilical venous line or peripheral intravenous line) to initiate prostaglandin infusion. 42 If the pa-tient is in a facility without pediatric cardiac care capabilities,the patient should be expeditiously transferred to obtain appropriate testing and more definitive care. 43,44

Patients with PAIVS and evidence of in utero cardiac dysfunction and high suspicion of or confirmed RVDCC will require a greater level of perinatal care and delivery at a facility with availability of appropriate personnel and critical care support.45 This recommendation also includes patients with PAIVS with Ebstein anomaly or TV dysplasia resulting in severe than pericid insufficiency and massive right heart dilation and those with hydrops fetals secondary to associated foramen ovale closure or restriction.46,47 There are also rare reports of neonates with significant coronary artery anomalies, such as bilateral coronary artery ostial

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stenosis or atresia, who have coronary insufficiency or myocardial infarction in utero or in the early postnatal period, resulting in in utero demise and/or rapid onset of cardiac dysfunction and postnatal instability. 48,49

Fetal cardiac intervention with ultrasound-guided in utero pulmonary valuloplasty may be considered in a small sub-set of fetuses with membranous PAIVS, with the objective of creating antegrade PBF. This may promote RV growth and functional development with the goal of increasing the like-lihood of a of 2-ventricle circulation.50-52 Fetal candidates may include patients with favorable plate-like PV atresia and limited fibromuscular infundibular obstruction. These patients have a lower risk of RVDCC with less procedural risk and greater procedural success rates.53-56

Overall, patient selection for fetal cardiac intervention in PAIVS remains challenging, given the limitations of accu-rately predicting postnatal outcomes on the basis of a midg-estation fetal echocardiogram, lack of definitive patient selection criteria, and limited single-center experience with low case volumes and unclear long-term data. ${}^{50,51,54}$ There are additional significant technical challenges in fetal cardiac intervention, given the anterior orientation of the RVOT that uniquely carries a high risk of fetal periproce-dural hemodynamic instability and demise compared with other fetal cardiac intervention procedures. 57,58 This proce-dural risk must be carefully balanced against any predicted potential benefits, which remain unknown.

Section 2.1: For patients with PAIVS, a multidisci-plinary team approach should be engaged to accurately assess the anatomic characteristics and physiologic needs of each individual patient because optimal management remains challenging as a result of wide variations of RV malformations and coronary patterns.(CoR I, LoE B-NR)

Diagnostic techniques and palliative strategies have evolved such that multiple options are available to achieve successful anatomic definition and physiologic stabilization of neonates with PAIVS For example, PV perforation with a wire, laser, or radiofrequency energy has become an alter-native to surgery in the initial palliation of neonates to establish antegrade PBF in PAIVS. Ductal stent deployment is a reasonable alternative to a surgically placed shunt to augment PBF with favorable anatomy. Strategies discussed herein include recommendations for timing and modality of fetal imaging to inform diagnosis and prognosis, perinatal anatomic and physiologic assessment by various imaging modalities, suitability for either catheter-based, surgical palliative interventions or both, and direct referral to cardiac transplantation. In many circumstances, more than one technique may be considered.

Given the significant variability in treatment pathways, a multidisciplinary team approach to accurately assess each patient's anatomic characteristics and physiologic needs is

required to achieve optimal treatment success. ${}^{59}$ In fact, data support that collaborative decision-making between interven-tional cardiology and cardiac surgery, guided by the individual patient's anatomy, contribute to excellent midterm results.21,60 In addition, multidisciplinary support may be beneficial when planning high-risk interventions and procedures.61

Section 2.2: Neonates with PAIVS should undergo echocardiographic assessment for RV size and morphology, TV size, morphology, and function; ade-quacy of the atrial communication, ductal arteriosus insertion, and branch pulmonary artery(PA); and abnormal connections of coronary arteries to determine optimal management.(CoR I, LoE B-NR)

Establishing the presence of RVDCC is the most impor-tant factor in determining initial interventional strategy.Echocardiography may be useful for detecting coronary ar-tery ostial atresia and the presence of RV fistulous connec-tions to the coronary circulation. Echocardiography alone is inadequate for determining the presence of RVDCC; hence,definitive coronary artery assessment with invasive coro-nary artery angiography should be performed. More recently, computed tomography angiography(CTA) has been proposed as an alternate noninvasive imaging modal-ity to determine RVDCC.

Although promising, this modality requires further vali-dation. ${}^{64}$ A significant correlation exists between RVDCC and RV hypoplasia and morphology and TV size, so echo-cardiographic assessment of these structures remains important. In a cohort of 72 patients with PAIVS, muscular pulmonary atresia, defined as the obliteration of its infun-dibulum by muscle without an identifiable PV tissue, is highly related to RVDCC, with a sensitivity of 82% and specificity of 98%. The presence of coronary ostial atresia is also strongly associated with muscular pulmonary atresia and is likely only present in patients with this RV morphology. Similarly, hypoplastic TV(z score<-2.5 to-3) is also highly associated with RVDCC with reported sensitivity of 100% and specificity of 83% to 86%.65,66 In a cohort of 171 patients with PAIVS, TV annular size was the only variable(independent of RVDCC) that was a risk factor for not achieving 2 ventricular repairs at 5 years follow-up. A TV z score of>-1.5 suggests a 2-ventricle repair destination, whereas a z score of<-4 suggests single-ventricle palliation. ${}^{12}$

A compilation study of reported TV z score cutoffs for achieving 2 ventricle repairs, when normalized to published normal TV data in children by Awori and colleagues, ${}^{67}$ sug-gested a cut-off z score of>-2.8. Having a smaller TV size is also a predictor of the requirement of a systemic-pulmonary shunt to achieve adequate PBF, whereas the presence of significant TV incompetence is associated with increased mortality. Although major aortopulmonary collateral arteries with discontinuous or absent central pulmonary arteries is

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usually associated with PA with ventricular septal defect, rare cases have been reported in PAIVS.68 Ductal insertions into the ipsilateral branch PA origin rather than between the bifur-cation occurs in approximately 25% of neonates with PAIVS and is associated with an increased risk of subsequent prox-imal branch PA stenosis should the ductal tissue not be fully resected during surgical shunt insertion or if the catheter intervention with patent arterial duct(PDA) stent does not extend across the proximal branch PA. ${}^{6}$

Section 2.3: Because diagnosing RVDCC in PAIVS typically requires anatomic and physiologic assessment,cardiac catheterization should be considered the stan-dard modality for evaluating the risk of cardiac ischemia. However, in centers with expertise, cardiac to-mography is emerging as a valuable screening tool for identifying key anatomic features.(CoR IIb, LoE B-NR)

Abnormal coronary fistulous connections to the right ventricle are described in up to 90% of patients with PAIVS, with additional severe coronary artery obstructive lesions in 9% to 30% of patients. These coronary anom-alies lead to the potential for a RV-dependent coronary circulation whereby the left ventricular myocardium is dependent on the high RV filling pressures for adequate perfusion and may result in catastrophic cardiac ischemia after RV decompression. Cardiac catheterization with right ventriculography, balloon occlusion aortograms, and selec-tive coronary injections is the gold standard for the evalua-tion of coronary circulation in patients with PAIVS. ${}^{71}$ However, the procedure is invasive, requires relatively high ionizing radiation exposures, is challenging to perform and interpret, and is associated with risk of vessel injury and life-threatening arrhythmias. ${}^{70}$

Comparatively, cardiac CT is relatively noninvasive,easier to perform, and associated with a lower risk. It is currently widely used in patients with PAIVS to determine pulmonary arterial and ductal anatomy before interven-tion.72 In neonates, coronary arteries can be challenging to see on cardiac CT and may require additional expertise and ultrafast acquisitions. Despite these challenges, in a few specialized centers, cardiac CT is being used to evaluate

the coronary circulation as well as the with the pulmonary arterial anatomy in neonates with PAIVS. The existing data on the use of cardiac CT for coronary evaluation in PAIVS is limited by cohort size, single-center experiences,and variable PAIVS morphologies. Accurate CT character-ization of the abnormal fistulous connections and associated obstructive lesions is possible, illustrating the potential car-diac CT holds both coronary and pulmonary evaluations in PAIVS(Table 1).64,70,73-75

Section 3.1: In patients with favorable morphologic features(plate-like membranous pulmonary atresia,tripartite RV, and TV annular z score of≥-2) and no ev-idence of RVDC, RV decompression in the neonatal period is recommended(CoR I, LoE B-NR); surgical or catheter-based RV decompression may be considered on the basis of specific morphologic factors and individ-ual center experience.(CoR IIb, LoE C-LD)

Neonates with favorable RV anatomy have anatomic features that tend to coexist, including a tripartite RV consisting of an inlet, sinus, and outflow portion, a TV annulus z score of≥-2.0, and absence of RVDCC.22,76,77 In such patients, neonatal RV decompression is recommen-ded to promote biventricular circulation with the attendant benefits of a subulmonary ventricle. Decompression of the RV early in life allows for lower RV systolic pressures as the RVOT obstruction is relieved.7,87 Effective decompression potentiates the resolution of RV hypertro-phy, which ultimately allows for improvement in RV diastolic function. This transition, when successful, allows for biventricular circulation.

Studies have demonstrated that RV decompression in this population with favorable RV anatomy is associated with RV and right-sided structural growth, including the TV annulus and RV volume. The most common manner of RV decompression in the current era is transcatheter PV perforation and balloon angioplasty. ${}^{8,81}$ However, surgi-cal decompression with valvotomy is also acceptable ther-apy. Surgical decompression may include a transannular patch with or without subvalvar RV muscle resection.82 The goal of either approach to RV decompression is to

TABLE 1. Important morphologic factors in PAIVS and recommended diagnostic modalities for characterization and decision-making

|Morphologic factors|Diagnostic modality| |---|---| |RV morphology(unipartite, bipartite, or tripartite)|Fetal echo, postnatal transthoracic echo| |Pulmonary valve morphology: membranous vs muscular pulmonary atresia|Fetal echo, postnatal CT scan, cardiac catheterization| |Presence of and extent of coronary sinusoids or fistula, presence of RVDCP|Fetal echo, postnatal CT scan, cardiac catheterization| |Tricuspid valve size, morphology, and function|Fetal echo, postnatal echo and 3-D echo| |Ductus arteriosus size, position, and PA connection|Fetal echo, postnatal echo, CT scan, cardiac catheterization| |Interatrial communication adequacy to support systemic output|Fetal echo, postnatal echo, cardiac catheterization|

PAIVS, Pulmonary atresia with intact ventricular septum; RV, right ventricular; RVDCC, right ventricle-dependent coronary circulation; CT, computed tomography; 3-D,3-dimensional; PA, pulmonary artery.

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effectively remove all RVOT obstruction to achieve the aforementioned benefits.

Section 3.2: For neonates with PA/IVS without evidence of RVDCC, it is reasonable to perform RV decompres-sion, systemic to PA shunt/PDA stent, or both after consideration of type of pulmonary atresia(muscular vs membranous), TV z score, RV morphology, and cavity size.(CoRIIa, LoE B-NR)

The goal of RV decompression for patients with neonatal PAIVS without evidence of RVDCC is to provide a source of antegrade PBF as well as to encourage RV development and growth. For many patients, RV decompression alone will not provide an adequate source of PBF. As a result, an alternative source of PBF is necessary. For those undergoing surgical RV decompression with or without additional resection of obstructive RV muscle, an alternative source of PBF in the form of a systemic-PA shunt may be beneficial. Antegrade flow through the TV may be encouraged by limiting the size of the atrial septal defect. However, determining the appropriate size of the atrial septal defect is clearly inexact and is left to the clinical experience of the surgeon and treat-ment team. For those patients undergoing transcatheter-based RV decompression, concomitant PDA stenting may be possible depending upon ductal and PA anatomy.3,83,84 In either scenario, the competence of the TV is important in the absence of a PV to avoid the potential for circular shunting after establishing an RV-PA connection in the setting of systemic-PA shunt. Similarly, the potential for the systemic-PA shunt/PDA stent to compete with antegrade PBF through the RV-PA connection should be considered. ${}^{85}$

Section 3.3: In patients with less-favorable morphologic features(muscular pulmonary atresia, bipartite RV,TV z score<-3), RV decompression may be considered to promote RV growth if there is not RVDCC.(CoR IIb,LoE

Surgical-or catheter-based RV decompression may be considered on the basis of specific morphologic factors and individual center experience.(CoRIIIb, LoE C-LD)

A 2-ventricle(biventricular) circulation should be pur-sued for this subset of patients with PAIVS without RVDCC. Therapeutic efforts directed toward early RV decompression may promote antegrade RV and PBF with the hope for an eventual 2-ventricle circulation. Surgical RV decompression involves creation of a connection be-tween the RV cavity and the pulmonary arterial bed with an RVOT incision and transannular patch reconstruction.This approach may be combined with division and/or resec-tion of RV muscle bundles(typically in the RV apex and beneath the TV) to enlarge the size of the RV and to improve TV inflow.11

This approach is sometimes referred to as RV“overhaul,”rehabilitation, or recruitment and, in combination with sur-gical RV decompression, can be performed safely with the use of cardiopulmonary bypass(CPB) and cardioplegic arrest. This procedure is contraindicated in patients with RVDCC. In some patients with muscular outflow tract atresia, it may be possible to establish RV-PA continuity with catheter-based outflow tract and valve-perforation and placement of an RVOT stent. This procedure carries a greater risk for complications than simple perforation of the plate-like PV atresia. Anatomic and morphologic factors as well as individual center experience should guide patient-specific candidate for transcatheter-based RV decompression in patients with RVOT muscular atresia.Surgical and CPB standby may be indicated depending on individual center operations and experience.

Section 3.4: A surgical shunt or ductal stenting are both reasonable therapies to provide or augment PBF in patients with PAIVS.(CoR IIa, LoE C-LD)

Catheter-based systemic-PA shunt options are reasonable as either(1) primary palliative therapy in the presence of RVDCC or(2) adjunctive palliative therapy for those under-going RV decompression in the absence of RVDCC. In those who undergo RV decompression with either a catheter-based pulmonary valvotomy or a surgical RVOT reconstruction, it can be difficult to predict which patients will need an additional source of PBF. Several anatomic and physiologic factors can help guide management. Small baseline TV and RV size are predictive of the need for an additional PFB source. However, in those patients who un-dergo an RV decompressing procedure, residual RV-PA obstruction, RV systolic pressure, and net directional flow across the atrial septum can all be helpful predictors of the need for additional PBF. These factors can be assimi-lated into a comprehensive score that may help predict the need for additional PBF.86

Although surgical sources of systemic-PA shunting can be used for all patients with or without(eg, RVDCC)CPB, the choice for PDA stenting is often dictated by the combination of ductal and/or PA anatomy. Accordingly,for neonates requiring surgical or catheter-based palliative systemic-PA shunt procedures, adjunctive CTA imaging often helps in the evaluation of PDA stenting candidacy as well as preoperative planning for surgical systemic-PA shunt placement. ${}^{64}$ For patients with unfavorable anatomy of ductal insertion onto one of the branch PAs, a coarctation of the PA may result from the ductal tissue at the insertion of the PDA. Although it is possible to place a stent across the ductus into one of the branch PAs, the subsequent surgical interventions may be compromised. In these patients, surgi-cal PDA division/resection with concomitant PA recon-struction with the Blalock Taussig Thomas shunt may be preferred.

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Section 3.5: In patients with PAIVS and no RVDCC in whom the morphology a physiology are not amenable to a definitive 2-ventricle repair, a 1.5-ventricle pallia-tive strategy may be considered.(CoR IIb, LoE C-LD)

A subset of patients with PAIVS and RV size and/or phys-iology that is insufficient for a definitive 2-ventricle repair and without RVDCC may be candidates for RV decompres-sion. In these patients with diminutive right heart structures,most will not be able to proceed with a 2-ventricle repair. A 1.5-ventricle palliative strategy may provide an alternative to single-ventricle palliation. First described in children with PAIVS in 1989, a 1.5-ventricle strategy consists of the addition of a bidirectional Glenn procedure, which maintains inferior vena cava flow through the previously de-compressed right ventricle and PA.87 By diverting upper body venous return from the small right ventricle, right atrial hypertension and right to left atrial shunting can often be minimized. 87,88 This approach may allow closure of the atrial septal defect while maintaining appropriate cardiac output and saturations. 89,90

In practice, this strategy is uncommonly employed(5% of all patients with PAIVS in 2 large series).10,91 Data on optimal timing, indications, and long-term outcomes are sparse.Although most children undergo 1.5-ventricle completion with Glenn in the first year of life, successful transition to 1.5-ventricle physiology at older ages has been reported with reasonable short-term outcomes.10,91-94 Indications for a 1.5-ventricle approach also vary substantially between cen-ters.10 Smaller RV and TV have been predictive of 1.5-ventricle versus 2-ventricle strategies.2,10,60 Successful 1.5-ventricle has been reported at a TV z score of less than 1.5-ventricle has been reported at a TV z score of less than-5.89,95 Several centers use cyanosis in infancy or early child-hood after neonatal RV decompression as an indication to proceed with the transition to 1.5-ventricle strategy.60,88,96

Overall survival for those with 1.5-ventricle palliation is not significantly different than those reaching single ventricle palliation or 2-ventricle repair, and evaluation of physiology and functional status of survivors has shown mixed results. ${}^{91,97}$ In the evaluation of exercise testing in 70 adolescents with PAIVS, one study found that those who had undergone 1.5-ventricle palliation had significantly greater oxygen consumption than single-ventricle palliation or 2-ventricle repair patients. In this study, there was a trend toward improved chronotropic response compared with 1 ventricle, reproduced in a smaller series. 77,98 In a cardiac catheterization study of 1.5-ventricle survivors, central venous pressure or cardiac output was no different than Fon-tan controls, highlighting potential challenges with this particular approach. ${}^{99}$ Further research on the long-term physiologic outcomes of the 1.5-ventricle palliation is needed to identify the best candidates for this approach.

Section 3.6: In patients with PAIVS without RVDCC,resection of RV musculature to increase RV and diastolic volume and increase mobility of TV apparatus may be a useful strategy to facilitate 1.5- or 2-ventricle repair.(CoR IIb, LoE B-NR)

To avoid single-ventricle palliation for PAIVS, strategies to increase RV volume and enhance TV mobility have been suggested, particularly for patients with less severe RV and TV hyplasia without evidence of RVDCC.100,101 In that regard, several approaches have been suggested aiming to increase the RV volume and tricuspid mobility in patients with tripartite RV and a TV z score between-3 and-5.The patient selection criteria and the specific strategy vary widely between institutions advocating for this approach,with the concept of resecting RV musculature evolving from the original bifudular resection to RV sinus myec-tomy with some later modifications extending this approach to a subgroup of patients with RVDCC.102

Such modifications include off-bypass ligation of the right ventricle to coronary artery fistulas with intraoperative monitoring for evidence of coronary compromise. ${}^{103}$ Another approach involves staged RV decompression via a limited pulmonary valvotomy aiming to maintain sys-temic or supra-systemic RV pressure. This technique has a limited pulmonary valvotomy aiming to maintain sys-temic or supra-systemic RV pressure. This technique has shown an approximately 50% regression of RV-to-coronary fistulas.104 Success rates for achieving 1.5- or 2-ventricle repair with these approaches reach 60% to 70%, with reported improvements in RV volume and reasonably low morbidity and mortality. However, long-term survival and functional outcomes with these approaches are still under investigation.

Section 3.7: Right ventricle decompression should not be performed in neonates with PAIVS and RVDCC.(CoR III, LoE B NR)

Right ventricle-to-coronary artery fistulas are common in patients with PAIVS, particularly in those with smaller TVs and right ventricles with fibromuscular pulmonary atresia. ${}^{17}$ When accompanied by coronary stenoses or interruptions,and these fistulas from the hypertensive RV may supply a signif-icant portion of myocardium. Early experience with death shortly after right ventricular decompression(RVD) as the result of myocardial ischemia, suspected to be related to an abrupt decrease in RV pressure and thus coronary perfu-sion, led to the description of RVDCC to denote those at risk for such events. 29,105,106

Much of our understanding of the danger of RVD in those with RVDCC and what anatomy typically constitutes RVDCC comes from the 1992 single-center study by Giglia and colleagues.29 In this study, the authors had a protocol of performing RVD in all patients with PAIVS The authors re-ported on outcomes among those with different variations of RVDCC. They found that those with coronary fistulae

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but no stenoses were able to tolerate RVD(0 deaths/7 patients), whereas those with stenoses in both right and left coronary systems did not(3/3 died). Those with stenosis to a single coronary artery had more mixed results, with 2 of 6 patients dying, although only one had evidence of left ventricular dysfunction.

The authors' summary is consistent with current under-standing of the risks:“Death after RVD seems to depend on the amount of left ventricular myocardium at risk for ischemia, namely that which is distal to coronary artery steno-ses in the presence of stenoses in other vessels that prevent effective collateralization," highlighting why very careful definition of the coronary circulation is critical for appropriate triage of the patient with PAIVS. Given the overwhelming consensus that RVD is contraindicated in those with RVDCC,it is not surprising that no recent studies have evaluated the consequences of decompression. Reasonable short-term sur-vival in more recent studies of RVD in those without RVDCC suggests that current approaches have minimized mortality secondary to coronary ischemia after RVD. 78,95

Section 3.8: In patients with PAIVS and RVDCC, it is reasonable to proceed with single-ventricle palliation;cardiac transplantation may also be reasonable depend-ing upon coronary anatomy and institutional experi-ence.(CoR IIa, LoE B-NR)

Primary heart transplantation is an acceptable alternative management strategy to surgery in infants with PAIVS and RVDCC, with multicenter institutional data demonstrating primary transplantation used in 4% of infants with PAIVS3,82,107 Primary listing for heart transplantation may be considered after the diagnosis of high-risk anatomic features. Multicenter review has demonstrated that 1 in 5 patients with PAIVS experience major cardiac events after an initial intervention, with the presence of 2 major coro-nary stenoses and lower weight at the time of intervention being independent risk factors for complications.3 Thus,dultisciplinary evaluation and perceived risk stratifica-tion for initial procedural or surgical intervention is war-ranted to determine whether primary transplantation provides the most reliable treatment strategy in the presence of high-risk anatomic features.

Data from the Pediatric Heart Transplant Society have demonstrated greater 1-, 5-, and 10-year survival rates for patients with PAIVS(74%, 65%, 61%) compared with other patients with single ventricle(62%, 54%, 50%),with PAIVS being an independent predictor of improved waitlist and posttransplantation survival. ${}^{108}$ Important to the interpretation of these data is that patients with aortopul-monary shunts had improved waitlist outcomes within the PAIVS cohort, potentially reflecting a listing selection bias. ${}^{108}$ Despite this potential confounding factor, outcomes for transplantation in PAIVS remain acceptable and support

consideration of this primary treatment strategy in select,high-risk infants with RVDCC.

Section 3.9: In a patient with PAIVS with evidence of myocardial ischemia and/or cardiac arrest, use of mechanical circulatory support might be considered as bridge to recovery, cardiac transplantation, and/or decision.(CoR IIb, LoE C-EO)

The role of mechanical circulatory support in patients with PAIVS showing evidence of ongoing or impending myocardial ischemia is 2-fold. The first scenario is acute resuscitation from rapid deterioration and/or cardiac arrest,typically using the extracorporeal membrane oxygenation circuit.109-111 The second situation is with an intention of more chronic support, likely as a bridge to cardiac transplant, but possibly to recovery of the heart and end-organs, often with the ventricular assist device(VAD)system.112,113

The expected duration of support is usually only temporary in the former, whereas the support tends to be in-termediate to long-term in the latter. A controversy exists regarding the type of pump incorporated in the VAD system: extracorporeal continuous-flow centrifugal versus paracorporeal pulsate pumps.114 The strategy to maintain PBF is also a matter of debate.115,116 Although a surgical shunt can be created concomitant with VAD placement,the feasibility of serial dilation may be an appealing advan-tage of the ductal stent approach when substantial somatic growth occurs during support.

CPB can provoke cardiac ischemia in hearts with RVDCC by depleting preload to the RV resulting from external volume shift to the reservoir(open circuit).117 Un-like CPB, extracorporeal membrane oxygenation or VAD preserves cardiac preload because the circuit is closed(no reservoir) and is less likely to cause cardiac ischemia in pa-tients with RVDCC. Instead, myocardial oxygen supply/de-mand mismatch likely improves if managed appropriately by reducing oxygen consumption with myocardial decom-pression and improving systemic cardiac output including coronary perfusion.112,113 Exceptions may include bilateral coronary atresia, in which neither coronary system is con-nected to the systemic circulation, thereby limiting the benefit of improved systemic cardiac output.16,118

Section 4: Patients with PAIVS should be followed by cardiologists experienced in congenital heart disease because of the ongoing risks of complications.(CoR IIa LoE C-LD)

Patients with PAIVS should be followed by providers with experience in managing congenital heart disease regardless of the circulation achieved. Pediatric care should be actively transitioned to adult congenital heart disease care at an appropriate time. ${}^{119}$

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For the Fontan pathway, the risks are well described and time-related, with a progressive increase in incidence from the second decade onwards. ${}^{120}$ Some of these complications are related to the Fontan circulation itself and some specif-ically to PAIVS. Complications of the Fontan pathway include atrial dysrhythmia, the sequelae of elevated inferior vena cava pressure(hepatic dysfunction and lymphatic dis-orders such as protein-losing enteropathy and plastic bron-chitis), and progressive systolic and diastolic dysfunction of the systemic left ventricle. For patients with RVDCC, there is an ongoing risk of myocardial ischemia, even late after Fontan completion. The long-term outcomes of Fontan palliation in PAIVS with RVDCC may be inferior to pa-tients with PAIVS without RVDCC who undergo Fontan. ${}^{121}$

For the 1.5-ventricle and 2-ventricle pathways, a similar profile of issues may arise depending on the inferior vena

cava pressure and systolic and diastolic properties of the right ventricle. Over time, the size of the atrial communica-tion may require treatment, particularly if there is important cyanosis. The need for PV replacement and TV repair or replacement may also be considered to optimize efficiency of the circulation and preserve RV function in the long term.Unusual volumetric guidelines for PV replacement in the context of PV insufficiency(such as after repair of tetralogy of Fallot) may require customization in patients with PAIVS where the RV has a history of severe diastolic noncompliance.

Exercise testing is an important modality in identifying dysfunction or ischemia and documenting the functional performance of the circulation.122 Cardiac magnetic reso-nance imaging is an essential tool in assessing RV volume and function and determining the risk of major cardiac

![ PAIVS Diagnostic Decision Tree\n Postnatal Echocardiogram\n Tricuspid valve annular dimension morphology and function\n Characterize atrial septal anatomy\n Ventricular Function\n Findings inconsistent with coronary artery anomalies\n Findings inconsistent with coronary artery anomalies\n Findings inconsistent with coronary artery anomalies\n Coronary Artery Evaluation\n CT angiogram (emerging role)\n Coronary Angiography\n](2/aj0YEe4oUhYkBdfpda0fYP/figure63c0e492def44e2ba4a500ad0ecaa8bc_0_7.png) FIGURE 3. Proposed diagnostic and therapeutic decision tree on the basis of the consensus statement in pulmonary atresia with intact ventricular septum.PAIVS, Pulmonary atresia with intact ventricular septum; RV, right ventricular; CT, computed tomography; TV, tricuspid valve; PV, pulmonary valve;RVDCC, right ventricle-dependent coronary circulation; PBF, pulmonary blood flow.

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![ Neonatal PAIVS Therapeutic Decision Tree\nCharacteristics of PAIVS\n Unfavorable Anatomy; mild or less RV\n QuasiPAVS Therapeutic Decision Tree\n Favorable anatomy; mild or less RV\n TV Z-score - 2 to - 3\n moduli, TV Z-score\n VLOCC\n Unfavorable Anatomy;\n Severe RV hypoplasia,\n TV Z-score <- 3\n TV Z-score <- 2\n PLOCC\n With out RVDCC\n W nVLDCC\n Establish Stable Pulmonary Blood flow\n via systemic Surgical\n Shunt or Ductal stent\n R IVDCCC\n Right Coronary Ostial Stenosis or Atresia;\n Less risk for ventricular ischemia\n R 死er risk for ventricular ischemia\n Plan Single Ventricle\n Palliation, Establish stable PBT or Aresia;\n P 900 0\n Evidence of cardiac ischemia\n No Cardiac ischemia\n Plan Single Ventricle\n evidence of cardiac ischemia\n No Cardiac Ischemia\n TENT"\n S 3Tens\n Consider RV\n recruitment procedure\n if continued inadequate\n RV size or function\n Two Ventricle Circulation\n If inadequate RV or TV growth\n FIGURE 3.(continued).\n2333\n1.5 Vertical Circulation if inadequate RV or TV\n](2/aj0YEe4oUhYkBdfpda0fYP/figure3ba33b4dace7470798901d14218206c3_0_2.png) FIGURE 3.(continued).

events. ${}^{123}$ Mechanical circulatory support as a bridge to transplantation may be considered for patients at any stage when conventional end-stage heart failure strategies are ineffective.2

FUTURE DIRECTIONS

As with many congenital heart defects, the genetic basis of the disease is rapidly becoming clearer. Even though there is not a single identified genetic cause for PAIVS, a significant proportion have identified genetic variants and/or familial inheritance. The identification of fetuses with high-risk genotypes may allow for earlier diagnosis and facilitate the identification of patients who might most benefit from early diagnosis and fetal intervention.

There has been promising work on more sophisticated post-natal imaging. The characterization of the coronary arteries is crucial to proper risk stratification and triage of therapy. Accu-rate identification of coronary artery anomalies in the neonatal period is essential. As technology improves, further studies may show CTA as a safe and efficacious diagnostic tool without the risks associated with cardiac catheterization.

In those patients with favorable anatomy, surgical decompression of the right ventricle and creation of a surgi-cal aortopulmonary shunt, if necessary, have been the main-stay of neonatal intervention for PAIVS. The emergence of catheter-based techniques and technology has shifted the pendulum toward less-invasive radiofrequency perforation and dilation of the PV and duct stenting to establish and

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maintain PBF. Defining the lowest risk and most efficacious strategy for RV decompression and predicting the need for additional PBF remains challenging. It would be optimal to make this decision at the time of RV decompression to avoid additional procedures. Clinical trials are ongoing to help define the safest, most efficacious strategies for those in which a 2-ventricle outcome is anticipated.

Investigators have proposed strategies and survival techniques in hope of increasing the chances of a 2-ventricle outcome.The terms RV remodeling, rehabilitation, or recruitment have all been coined to describe this concept. Although these augmentation procedures have been successful in some cases, identification of the anatomic and physiologic factors associated with successful outcome are unclear. Further studies may help clarify candidates for specific pathways.Also, there is ongoing work regarding stem cell or stem cell products that may aid in the remodeling of the RV mus-cle to promote a more normal form and function. The crea-tion of a viable autologous PV construct for replacement of the PV after initial palliative procedure may also improve the long-term outcome for patients with PAIVS.

Some patients with PAIVS and RVDCC may experience cardiac ischemia. Defining which patients should continue down a single-ventricle palliation pathway and which will require a cardiac transplant remains difficult. For patients for whom cardiac transplantation is clearly the optimal ther-apy, management until an organ is available is challenging,and providing mechanical circuitry support can be an effective bridge to transplantation. Appropriate selection of which patients will most benefit and what form of support is optimal remains to be defined.

Finally, the long-term outcome for patients with PAIVS depends on effective and durable right ventricle function.Even in patients with 2-ventricle physiology, diastolic dysfunction of the small, noncompliant right ventricle can have adverse long-term consequences. The ongoing risk of cardiac ischemia, with or without RVDC, and compli-cations associated with single-ventricular palliation persists well beyond the pediatric period and will remtained the subject of future study and research(Fig 3).

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Conflict of Interest Statement

The authors reported no conflicts of interest.

The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or re-viewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

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by the American association for Thoracic Surgery(AATS), European Associa-tion for Cardio-Thoracic Surgery(EACTS), European Society of Thoracic Surgeons(ESTS), and Society of Thoracic Surgeons(STS). J Thorac Cardio-vasc Surg.2025;169(1):170-185.https://doi.org/10.1016/j.jtcvs2024.07.048

Key Words: Pulary atresia, intact ventricular septum,IVS, sinusoids, fistula, RVDCC, ductal stent, BTT shunt,RV decompression, palliation, Fontan procedure, single ventricle, heart transplantation, mechanical circulatory sup-port, coronary artery abnormalities

表 1
Morphologic factors	Diagnostic modality
RV morphology(unipartite, bipartite, or tripartite)	Fetal echo, postnatal transthoracic echo
Pulmonary valve morphology: membranous vs muscular pulmonary atresia	Fetal echo, postnatal CT scan, cardiac catheterization
Presence of and extent of coronary sinusoids or fistula, presence of RVDCP	Fetal echo, postnatal CT scan, cardiac catheterization
Tricuspid valve size, morphology, and function	Fetal echo, postnatal echo and 3-D echo
Ductus arteriosus size, position, and PA connection	Fetal echo, postnatal echo, CT scan, cardiac catheterization
Interatrial communication adequacy to support systemic output	Fetal echo, postnatal echo, cardiac catheterization

图示：原图识别中

图示：Class of Recommendation Suggested phrases for writing recommendations Strong recommendation ● Is recommended ● Is indicated Benefit >>> risk ● Should be considered ● Is reasonable ● Can be useful/effective/beneficial IIa Weak recommendation ● May/might be reasonable IIb Benefit≥ risk ● May/might be considered Moderate recommendation No benefit: risk = benefit ● Is contraindicated Strong recommendation ● Should not be performed/administrated Harm: risk > benefit

图示：High-quality evidence from more than one RCT with no concern for high risk of bias, or meta-analyses of high-quality RCTs with no concerns for significant bias. Randomized Moderate-quality evidence from 1 or more RCT or meta-analysis of (B-R): RCTS. Non-Randomized Moderate-quality evidence from 1 or more well-designed, (B-NR): well-executed nonrandomized studies, observational studies, or registry studies or meta-analyses of such studies. Limited data Studies with limitations of design or execution, meta-analyses of (C-LD): such studies, case series, or physiological or mechanistic studies in human subjects. Expert opinion Consensus of expert opinion based on clinical experience. (C-EO):

图示：a RVDDC a n a nary clais RVDCC + RVDCC + right + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC + RVDCC

图示：PAIVS Diagnostic Decision Tree Postnatal Echocardiogram Tricuspid valve annular dimension morphology and function Characterize atrial septal anatomy Ventricular Function Findings inconsistent with coronary artery anomalies Findings inconsistent with coronary artery anomalies Findings inconsistent with coronary artery anomalies Coronary Artery Evaluation CT angiogram (emerging role) Coronary Angiography

图示：Neonatal PAIVS Therapeutic Decision Tree Characteristics of PAIVS Unfavorable Anatomy; mild or less RV QuasiPAVS Therapeutic Decision Tree Favorable anatomy; mild or less RV TV Z-score - 2 to - 3 moduli, TV Z-score VLOCC Unfavorable Anatomy; Severe RV hypoplasia, TV Z-score <- 3 TV Z-score <- 2 PLOCC With out RVDCC W nVLDCC Establish Stable Pulmonary Blood flow via systemic Surgical Shunt or Ductal stent R IVDCCC Right Coronary Ostial Stenosis or Atresia; Less risk for ventricular ischemia R 死er risk for ventricular ischemia Plan Single Ventricle Palliation, Establish stable PBT or Aresia; P 900 0 Evidence of cardiac ischemia No Cardiac ischemia Plan Single Ventricle evidence of cardiac ischemia No Cardiac Ischemia TENT" S 3Tens Consider RV recruitment procedure if continued inadequate RV size or function Two Ventricle Circulation If inadequate RV or TV growth FIGURE 3.(continued). 2333 1.5 Vertical Circulation if inadequate RV or TV

引用 / 出处

2025 AATS专家共识文件：肺动脉闭锁伴室间隔完整患者的管理. 2025.