Materiały źródłowe

• #1 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  Pulmonary atresia with intact ventricular septum is a rare congenital heart disease comprising less than 1% of all heart defects, characterized by membranous or muscular atresia of the right ventricular outflow tract without any ventricular communication, often involving underdevelopment of the right ventricle and tricuspid valve. […] This activity for healthcare professionals is designed to enhance the learner’s competence in identifying pulmonary atresia with intact ventricular septum, performing the recommended evaluation, and implementing an appropriate interprofessional approach when managing this condition. […] Managing PA-IVS begins with prostaglandin infusion to maintain ductal patency, which is crucial for survival preoperatively. Intervention, either catheter-based or surgical, is necessary for neonates. Treatment is highly individualized, encompassing biventricular repair, systemic-to-pulmonary shunts, single-ventricular repair, or heart transplantation, depending on the characteristics of the right ventricle and tricuspid valve, as well as the anatomy of the coronary arteries.

• #2 Pulmonary atresia – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_atresia

  The treatment of pulmonary atresia consists of administration of an IV medication called prostaglandin E1, which is used for treatment of pulmonary atresia by stopping the ductus arteriosus from closing. This allows mixing of blood from the pulmonary and systemic circulations. Prostaglandin E1 can also be dangerous, however, as it can cause apnea. Another example of preliminary treatment is heart catheterization to evaluate the defect(s) of the heart; this procedure is much more invasive. Ultimately, however, the individual will need to have a series of surgeries to improve the blood flow permanently. The first surgery will likely be performed shortly after birth. A shunt can be created between the aorta and the pulmonary artery to help increase blood flow to the lungs. As the child grows, so does the heart and the shunt may need to be revised in order to meet the body’s requirements.

• #3 18: Pulmonary Atresia with Intact Ventricular Septum | Veterian Key

  https://veteriankey.com/18-pulmonary-atresia-with-intact-ventricular-septum/

  Pulmonary atresia with intact ventricular septum (PA/IVS) is a relatively uncommon congenital heart defect characterized by luminal discontinuity between the right ventricular outflow tract and the main pulmonary artery in the absence of a ventricular septal defect. […] Over the ensuing decades, the diagnostic evaluation has become more refined and the treatment plans more tailored to the anatomic variants, resulting in dramatically improved prognosis for infants with PA/IVS. […] Without treatment, 50% of infants die within 2 weeks, and 85% die by 6 months of age. […] The goal of treatment is to achieve a biventricular circulation in as many infants as possible without compromising myocardial perfusion. […] The first step is creation of antegrade pulmonary blood flow. This can be achieved by placement of a right ventricular outflow tract patch, surgical valvotomy, or by transcatheter perforation and balloon dilation of the pulmonary valve.

• #4 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. This action is vital for the preoperative survival of these patients, as the ductus arteriosus is the sole source of pulmonary blood flow. […] Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure. Due to the complexity and heterogeneity of this disorder, no single method is effective for all patients. […] The preferred surgical or transcatheter intervention is influenced by several variables, which include the size and function of the tricuspid valve, the anatomy of the right ventricle and coronary arteries, and the type of pulmonary atresia. […] Single-ventricular repair (bidirectional Glenn procedure followed by Fontan) should be considered in patients with severe right ventricular hypoplasia and RVDCC.

• #5 About Pulmonary Atresia | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/pulmonary-atresia.html

  Most babies will need medication to keep their ductus arteriosus open after birth. Keeping this blood vessel open will help with blood flow to the lungs until the pulmonary valve can be repaired. […] Treatment for pulmonary atresia depends on its severity. In some cases, blood flow can be improved by using cardiac catheterization. During this procedure, doctors can expand the valve using a balloon to keep the ductus arteriosus open. In some cases, they may need to place a stent (a small tube) to keep it open. […] In most cases of pulmonary atresia, a baby may need surgery soon after birth. During surgery, doctors widen or replace the pulmonary valve and enlarge the passage to the pulmonary artery. If a baby has a VSD, the doctor will use a patch. The patch closes the hole between the lower chambers of the heart. These actions will improve blood flow to the lungs and the rest of the body. If a baby has an underdeveloped right ventricle, staged surgical procedures might be needed. The staged surgical procedures are similar to surgical repairs for hypoplastic left heart syndrome (HLHS).

• #6 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Pulmonary atresia treatment depends on how the condition affects your child and on their: […] Usually, children with pulmonary valve atresia will need surgery to improve blood flow to their lungs. […] Temporary treatments include medication and balloon atrial septostomy. Most children will probably need more surgery to improve blood flow to their lungs. […] Newborns with pulmonary atresia usually receive an IV drug (injected into a vein) called alprostadil. This drug prevents the ductus arteriosus (which connects the aorta to the pulmonary artery) from starting to close as it normally would within a few hours to days after birth. […] Your childs healthcare provider can use cardiac catheterization to enlarge the opening in their septum (the wall between the right atrium and left atrium). This improves blood flow to your babys lungs.

• #7 Pulmonary atresia with intact ventricular septum – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-intact-ventricular-septum/cdc-20396714

  Pulmonary atresia with intact ventricular septum, also called PA/IVS, is a rare heart condition that’s present at birth. […] Treatment for pulmonary atresia with intact ventricular septum may include medicines and one or more procedures or surgeries to fix the heart. […] Babies with pulmonary atresia need treatment right away. It’s best to get treatment at a medical center with surgeons and other healthcare professionals who have experience with complex heart conditions present at birth. […] Treatment for pulmonary atresia with intact ventricular septum, also called PA/IVS, may include medicines and one or more procedures or surgeries. […] Medicine may be given through an IV to keep the ductus arteriosus open. This is not a permanent treatment for pulmonary atresia. But it keeps the baby stable until a surgery or procedure can be done to permanently fix blood flow.

• #8 Pulmonary atresia with intact ventricular septum | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia-with-intact-ventricular-septum?content_id=CON-20380876

  Treatment for pulmonary atresia with intact ventricular septum may include medicines and one or more procedures or surgeries to fix the heart. […] Babies with pulmonary atresia need treatment right away. It’s best to get treatment at a medical center with surgeons and other healthcare professionals who have experience with complex heart conditions present at birth. […] Treatment for pulmonary atresia with intact ventricular septum, also called PA/IVS, may include medicines and one or more procedures or surgeries. […] Medicine may be given through an IV to keep the ductus arteriosus open. This is not a permanent treatment for pulmonary atresia. But it keeps the baby stable until a surgery or procedure can be done to permanently fix blood flow. […] A baby with pulmonary atresia with intact ventricular septum needs one or more surgeries or procedures to improve blood flow and fix the heart.

• #9 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Pulmonary atresia treatment depends on how the condition affects your child and on their: […] Usually, children with pulmonary valve atresia will need surgery to improve blood flow to their lungs. […] Temporary treatments include medication and balloon atrial septostomy. Most children will probably need more surgery to improve blood flow to their lungs. […] Newborns with pulmonary atresia usually receive an IV drug (injected into a vein) called alprostadil. This drug prevents the ductus arteriosus (which connects the aorta to the pulmonary artery) from starting to close as it normally would within a few hours to days after birth. […] Your childs healthcare provider can use cardiac catheterization to enlarge the opening in their septum (the wall between the right atrium and left atrium). This improves blood flow to your babys lungs.

• #10 Management algorithm in pulmonary atresia with intact ventricular septum – PubMed

  https://pubmed.ncbi.nlm.nih.gov/16572430/

  Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 – ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.

• #11 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. This action is vital for the preoperative survival of these patients, as the ductus arteriosus is the sole source of pulmonary blood flow. […] Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure. Due to the complexity and heterogeneity of this disorder, no single method is effective for all patients. […] The preferred surgical or transcatheter intervention is influenced by several variables, which include the size and function of the tricuspid valve, the anatomy of the right ventricle and coronary arteries, and the type of pulmonary atresia. […] Single-ventricular repair (bidirectional Glenn procedure followed by Fontan) should be considered in patients with severe right ventricular hypoplasia and RVDCC.

• #12 Pulmonary Atresia With Intact Ventricular Septum | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-intact-ventricular-septum-5/

  This chapter describes the disease spectrum of pulmonary atresia with intact ventricular septum (PA/IVS). […] PA/IVS is a highly variable continuum of severe congenital heart disease. […] Many programs have adopted a primary transplant strategy for these fragile patients. […] For those patients whose anatomy allows for potential one-and-one-half- or two-ventricle pathways we have adapted a hybrid treatment strategy for early palliation followed by completion of biventricular repair or one-and-one-half-ventricle repair, usually within the first year of life with excellent early and midterm results. […] Outcomes for the treatment of PA/IVS have improved significantly over the past several decades. […] Because of this wide spectrum and variability in disease severity, mainly related to tricuspid valve size, RV chamber anatomy, and coronary artery anomalies, complex algorithms for treatment are required to ensure optimum outcomes for these often fragile patients.

• #13 Pulmonary Atresia (PA) | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/pa

  Specific treatment for pulmonary atresia will be determined by your child’s doctor based on: […] After birth, it is important to make sure the ductus arteriosus stays open to allow blood to get to the lungs to pick up oxygen. An IV medicine called prostaglandin (PGE-1) is used to keep the ductus arteriosus open. […] Many babies will then undergo a procedure to provide more stable blood flow to the lungs and allow them to grow before they undergo further surgeries. This procedure can be done either with surgery or with a catheter-based procedure. […] Some patients, particularly those who had a pulmonary valve that did not form normally but do not have a lot of muscle under the valve, may be eligible for another procedure. […] After this initial procedure, the next steps are determined by the size of the right side of the heart, including the tricuspid valve between the atrium (collecting chamber) and the ventricle, the right ventricle itself, and any other associated abnormalities.

• #14 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including: […] Usually, the right ventricle is well developed and can pump blood to your babys lungs. Surgery involves: […] In this case, your babys right ventricle is usually poorly developed. They need a series of three operations during the first few years of their lives. […] After heart surgery, your child will need to spend a week or two in the hospital, with some of that time in the intensive care unit. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your babys birth, they can treat your newborn to improve their oxygen circulation. […] Medical procedures and surgeries can improve your childs condition, but they arent cures.

• #15

  https://link.springer.com/article/10.1007/s00246-025-03769-w

  Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. […] The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). […] We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS. […] The primary outcome assessed was survival. […] Our data also show low mortality in patients with PA/IVS. One patient underwent a neonatal transplant, and only 24.1% required single ventricle circulation, with 65.5% completing either a 2V or 1.5V circulation. Survival was 93.1%, with only two mortalities. […] This method promotes regular reassessments aimed at achieving the best possible survival rates with minimal morbidity and a high likelihood of complete or near complete septation.

• #16 Pulmonary Atresia With Intact Ventricular Septum | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-intact-ventricular-septum-5/

  These algorithms have resulted in improved survival in patients diagnosed with PA/IVS, particularly in the more severe forms of the spectrum. […] Patients are maintained on prostaglandin infusion after birth, and standard workup for newborns with complex congenital heart disease is undertaken. […] PA/IVS is a rare form of complex congenital heart disease with significant morphologic heterogeneity. […] Because of the significant morphologic heterogeneity seen in PA/IVS, repair or palliation strategies have a wide variation, from single-ventricle palliation to complete biventricular repair.

• #17 Pulmonary atresia: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001091.htm

  A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs. This medicine keeps a blood vessel open between the pulmonary artery and aorta. The vessel is called a PDA. […] Multiple treatments are possible, but which one is selected depends on the extent of the heart abnormalities that accompany the pulmonary valve defect. Potential invasive treatments include: […] Biventricular repair — This surgery separates the blood flow to the lungs from the circulation to the rest of the body by creating two pumping ventricles. […] Univentricular palliation — This surgery separates the blood flow to the lungs from the circulation to the rest of the body by constructing one pumping ventricle. […] Heart transplant.

• #18 Management algorithm in pulmonary atresia with intact ventricular septum – PubMed

  https://pubmed.ncbi.nlm.nih.gov/16572430/

  Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 – ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.

• #19 Management algorithm in pulmonary atresia with intact ventricular septum – PubMed

  https://pubmed.ncbi.nlm.nih.gov/16572430/

  Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 – ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.

• #20

  https://link.springer.com/article/10.1007/s00246-024-03566-x

  This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS) treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention. […] The aim of this retrospective study was to compare the long-term morbidity of patients treated with catheter-based therapy with those who underwent heart surgery as the initial intervention for right ventricular decompression. […] This retrospective population-based multicenter study demonstrates that both catheter-based intervention and heart surgery are safe procedures. […] The systematic literature review displays a great heterogeneity in study design, with no definitive golden standard treatment. […] In our study we found that decompression of the right ventricle could be performed safe, with no associated mortality, using either catheter-based intervention or heart surgery.

• #21 Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum | Heart

  https://heart.bmj.com/content/95/18/1520.short

  Objective: To review the outcome of patients with pulmonary atresia with intact ventricular septum after interventional perforation of the pulmonary valve, to assess the capability of this procedure to avoid neonatal or late intervention and to obtain a long-term biventricular repair. […] The results confirm that percutaneous interventional perforation is an effective first-stage procedure in patients with pulmonary atresia with intact ventricular septum. The right heart appeared to be adequate to maintain a long-term biventricular circulation in the large majority of cases.

• #22 Pulmonary Atresia with Intact Ventricular Septum (PA IVS) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/pulmonary-atresia-intact-ventricular-septum-pa-ivs

  Treatment for pulmonary atresia with intact ventricular septum depends on each child’s heart anatomy. Sometimes, PA/IVS can be treated with a cardiac catheterization technique called balloon valvuloplasty. In this procedure, an interventional cardiologist will insert a thin tube (catheter) through a vein in the leg to reach the heart. With the catheter, the doctor can create a hole in the closed-off pulmonary valve. Then, another catheter, which has a balloon on the end of it, is inserted. The interventional cardiologist will briefly inflate the balloon to open up the valve, then deflate the balloon and remove the catheter. […] In other cases, open heart surgery is needed to allow enough blood to flow to the lungs. Surgeons may open the pulmonary valve using a special patch. In other cases, they may place a narrow tube (called a Blalock-Taussig-Thomas shunt) from the aorta to the pulmonary artery (the large blood vessel that carries blood from the heart to the lungs) to make sure enough blood can reach the lungs. Other times, an interventional cardiologist can place a stent in the ductus arteriosus during a cardiac catheterization procedure, allowing blood to pass from the aorta to the lungs. […] If your child’s right ventricle is poorly developed and cannot function properly, a series of surgeries, called staged reconstruction, may be needed. These staged procedures create a pathway for blood to flow back to the heart and directly to the lungs, bypassing the right ventricle.

• #23 Guided transcatheter valvulotomy in pulmonary atresia with intact ventricular septum – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-guided-transcatheter-valvulotomy-in-pulmonary-articulo-13050465

  Transcatheter valvulotomy in pulmonary atresia with an intact ventricular septum can be used as a first step to create biventricular circulation and to stimulate further development of the hypoplastic right ventricle. […] To limit the possibility of cardiac perforation, which has been reported with all of these techniques, we performed transcatheter perforation in a newborn with this severe heart defect, using a special coronary guidewire guided by an open gooseneck snare positioned just above the atretic valve, followed by valvuloplasty. […] Transcatheter valvotomy in PAIVS requires favorable anatomical conditions: a tripartite right ventricle with a moderate degree of hypoplasia allowing subsequent development, a patent infundibulum, and a membranous-type valvular atresia with anatomical continuity.

• #24 Pulmonary Atresia With Intact Ventricular Septum. Perforation and Pulmonary Valvuloplasty Using a Modified Mechanical Technique. Medium-Term Follow-Up – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-pulmonary-atresia-with-intact-ventricular-articulo-13109935

  The technique involved snare-assisted anterograde or retrograde perforation with the soft tip of a special guidewire used for chronic total coronary artery occlusions, use of an arteriovenous loop, and progressive balloon dilatation from a diameter of 2 mm to a maximum diameter of 9.6 mm. […] Pulmonary valvuloplasty using a mechanical technique proved effective in patients with PAIVS. […] The modification of the classic technique through the use of special guidewires for the total chronic coronary obstruction by its directed soft part is less aggressive and improves results. […] In this series with favorable anatomy, the results are similar to those obtained through radiofrequency.

• #25 Pulmonary Atresia with Intact Ventricular Septum (PA IVS) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/pulmonary-atresia-intact-ventricular-septum-pa-ivs

  Treatment for pulmonary atresia with intact ventricular septum depends on each child’s heart anatomy. Sometimes, PA/IVS can be treated with a cardiac catheterization technique called balloon valvuloplasty. In this procedure, an interventional cardiologist will insert a thin tube (catheter) through a vein in the leg to reach the heart. With the catheter, the doctor can create a hole in the closed-off pulmonary valve. Then, another catheter, which has a balloon on the end of it, is inserted. The interventional cardiologist will briefly inflate the balloon to open up the valve, then deflate the balloon and remove the catheter. […] In other cases, open heart surgery is needed to allow enough blood to flow to the lungs. Surgeons may open the pulmonary valve using a special patch. In other cases, they may place a narrow tube (called a Blalock-Taussig-Thomas shunt) from the aorta to the pulmonary artery (the large blood vessel that carries blood from the heart to the lungs) to make sure enough blood can reach the lungs. Other times, an interventional cardiologist can place a stent in the ductus arteriosus during a cardiac catheterization procedure, allowing blood to pass from the aorta to the lungs. […] If your child’s right ventricle is poorly developed and cannot function properly, a series of surgeries, called staged reconstruction, may be needed. These staged procedures create a pathway for blood to flow back to the heart and directly to the lungs, bypassing the right ventricle.

• #26 Pulmonary Atresia With Intact Ventricular Septum. Perforation and Pulmonary Valvuloplasty Using a Modified Mechanical Technique. Medium-Term Follow-Up – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-pulmonary-atresia-with-intact-ventricular-articulo-13109935

  Pulmonary atresia with an intact septum (PAIS) is a serious cardiopathy that has an incidence of 0.7% to 3.1% of the congenital cardiopathies. It is characterized by great anatomical variability, where development of the right ventricle (RV) goes from a severely hypoplasic form and whose definitive treatment is a univentricular type correction, to the presence of a normal RV or with slight hypodevelopment with good possibility for a biventricular type correction. […] For these favorable cases with RV with acceptable development, and if the coronary distribution allows it, the treatment in the first days of life is valvular opening to favor the pulmonary flow and development of the right cavities. […] Our experience in valvular opening in PAIS began in 2001. […] We described a modified less aggressive form directed with a catheter loop in its anterograde form.

• #27 Pulmonary Atresia With Intact Ventricular Septum. Perforation and Pulmonary Valvuloplasty Using a Modified Mechanical Technique. Medium-Term Follow-Up – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-pulmonary-atresia-with-intact-ventricular-articulo-13109935

  The technique involved snare-assisted anterograde or retrograde perforation with the soft tip of a special guidewire used for chronic total coronary artery occlusions, use of an arteriovenous loop, and progressive balloon dilatation from a diameter of 2 mm to a maximum diameter of 9.6 mm. […] Pulmonary valvuloplasty using a mechanical technique proved effective in patients with PAIVS. […] The modification of the classic technique through the use of special guidewires for the total chronic coronary obstruction by its directed soft part is less aggressive and improves results. […] In this series with favorable anatomy, the results are similar to those obtained through radiofrequency.

• #28 About Pulmonary Atresia | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/pulmonary-atresia.html

  Most babies will need medication to keep their ductus arteriosus open after birth. Keeping this blood vessel open will help with blood flow to the lungs until the pulmonary valve can be repaired. […] Treatment for pulmonary atresia depends on its severity. In some cases, blood flow can be improved by using cardiac catheterization. During this procedure, doctors can expand the valve using a balloon to keep the ductus arteriosus open. In some cases, they may need to place a stent (a small tube) to keep it open. […] In most cases of pulmonary atresia, a baby may need surgery soon after birth. During surgery, doctors widen or replace the pulmonary valve and enlarge the passage to the pulmonary artery. If a baby has a VSD, the doctor will use a patch. The patch closes the hole between the lower chambers of the heart. These actions will improve blood flow to the lungs and the rest of the body. If a baby has an underdeveloped right ventricle, staged surgical procedures might be needed. The staged surgical procedures are similar to surgical repairs for hypoplastic left heart syndrome (HLHS).

• #29 Pulmonary Atresia with Intact Ventricular Septum (PA IVS) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/pulmonary-atresia-intact-ventricular-septum-pa-ivs

  Treatment for pulmonary atresia with intact ventricular septum depends on each child’s heart anatomy. Sometimes, PA/IVS can be treated with a cardiac catheterization technique called balloon valvuloplasty. In this procedure, an interventional cardiologist will insert a thin tube (catheter) through a vein in the leg to reach the heart. With the catheter, the doctor can create a hole in the closed-off pulmonary valve. Then, another catheter, which has a balloon on the end of it, is inserted. The interventional cardiologist will briefly inflate the balloon to open up the valve, then deflate the balloon and remove the catheter. […] In other cases, open heart surgery is needed to allow enough blood to flow to the lungs. Surgeons may open the pulmonary valve using a special patch. In other cases, they may place a narrow tube (called a Blalock-Taussig-Thomas shunt) from the aorta to the pulmonary artery (the large blood vessel that carries blood from the heart to the lungs) to make sure enough blood can reach the lungs. Other times, an interventional cardiologist can place a stent in the ductus arteriosus during a cardiac catheterization procedure, allowing blood to pass from the aorta to the lungs. […] If your child’s right ventricle is poorly developed and cannot function properly, a series of surgeries, called staged reconstruction, may be needed. These staged procedures create a pathway for blood to flow back to the heart and directly to the lungs, bypassing the right ventricle.

• #30

  https://link.springer.com/article/10.1007/s00246-024-03566-x

  The approach regarding supplementary procedures during the initial intervention differed in the two cohorts. […] The hybrid approach avoids CPB while still offering the opportunity to place a mBTS if needed and it decreases the risk of perforating the right ventricle or the pulmonary artery. […] The findings of this retrospective population-based multicenter study demonstrate that both catheter-based intervention and heart surgery are safe procedures with a low risk of complications.

• #31 Pulmonary atresia with intact ventricular septum – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-intact-ventricular-septum/cdc-20396714

  A baby with pulmonary atresia with intact ventricular septum needs one or more surgeries or procedures to improve blood flow and fix the heart. […] Some of these treatments are done in the first days to weeks of a baby’s life. Others are done later. The type of surgery or procedure depends on many things. These include the size of the right lower heart chamber and heart valves and whether the baby has other heart conditions. […] After treatment, babies with pulmonary atresia with intact ventricular septum, also called PA/IVS, should have regular health checkups with a heart doctor trained to treat children.

• #32 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including: […] Usually, the right ventricle is well developed and can pump blood to your babys lungs. Surgery involves: […] In this case, your babys right ventricle is usually poorly developed. They need a series of three operations during the first few years of their lives. […] After heart surgery, your child will need to spend a week or two in the hospital, with some of that time in the intensive care unit. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your babys birth, they can treat your newborn to improve their oxygen circulation. […] Medical procedures and surgeries can improve your childs condition, but they arent cures.

• #33 Surgical treatment of pulmonary atresia with intact ventricular septum. | Heart

  https://heart.bmj.com/content/38/9/957

  Fifty-nine children with pulmonary atresia and intact ventricular septum underwent various forms of surgical treatment at the Hospital for Sick Children, Toronto, during 1950 to mid 1975. […] Twenty-three patients had pulmonary valvotomy, 15 direct, 2 indirect, and 6 both direct valvotomy and infundibulectomy. […] Recently we have been treating infants with small right ventricles with balloon atrial septostomy at cardiac catherterization followed by a Potts anastomosis and pulmonary valvotomy. […] We believe that this operative combination of Potts anastomosis and pulmonary valvotomy offers the infant with pulmonary atresia and a small right ventricle a relatively low initial mortality and the possibility of right ventricular enlargement and subsequent repair.

• #34 Pulmonary Atresia (PA) | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/pa

  If the tricuspid valve and right ventricle are normal sized, the child should be able to have surgery to bring blood from the right ventricle to the lungs. […] If the tricuspid valve and right ventricle size is in between (not very small, but not normal sized), then the child may require a combination of the above surgeries so the right ventricle does not have to handle all the blood that would normally go through it to the lungs. […] Finally, in some patients with significant coronary abnormalities who do not have good blood supply to their heart muscle, a heart transplant may be required.

• #35 Type I and II pulmonary atresia with intact ventricular septum in infants: a 10-year experience in initial surgery at one center | BMC Cardiovascular Disorders | Full Text

  https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/s12872-022-02549-1

  To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum (PA/IVS). […] The effect of BT shunt combined with PDA ligation and pulmonary valve incision is superior to BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, and the effect of BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation is superior to BT shunt combined with PDA ligation, RVOT incision and transannular patch. […] The reasonable choice of the initial surgery method is the key to improving the survival rate, which is also an important factor affecting the prognosis. […] Early active surgical treatment of PA/IVS is the key to improving the survival rate, and staged decompression is relatively stable, even if this may increase the rate of postoperative reintervention. The reasonable choice of the initial surgical strategy is an important factor affecting the prognosis.

• #36 Pulmonary Atresia with intact ventricular septum

  https://www.rch.org.au/cardiology/heart_defects/Pulmonary_Atresia_with_intact_ventricular_septum/

  As in Pulmonary Atresia with VSD, this defect is also associated with complete obstruction of the Pulmonary Artery. However, as there is no associated VSD, blood is diverted from the right atrium to the left atrium via the Foramen Ovale. The right ventricle (RV) is usually small (Hypoplastic), though its wall may be thickened (Hypertrophied). Survival depends on the ductus remaining open in the early days of life (in order for blood to reach the lungs), Most babies will need a 'Shunt’ operation during infancy, involving insertion of a tiny piece of artificial tube (made from Goretex) between the Aorta, or a branch (usually one of the arm arteries), and one of the branch Pulmonary Arteries. […] Complete repair may be possible, but often necessitates several operations.

• #37 Pulmonary Atresia With Intact Ventricular Septum | SCAI – Seconds Count

  https://www.secondscount.org/condition/pulmonary-atresia-intact-ventricular-septum

  In certain circumstances, the valve plate may be able to be perforated in the catheterization lab. A balloon dilation catheter may be used to open the valve plate (balloon valvuloplasty). This will allow blood from the right ventricle to pass directly into the lung arteries. For some babies, this may allow enough blood to pass through to the lungs. However, some babies will need additional blood flow to the lungs. If this occurs, surgery will be performed to place a tube from one of the arteries directly to the lungs, which is called a modified Blalock-Taussig shunt. Another option is for a small metal tube called a stent to be placed in the ductus arteriosus to keep it from closing. […] As the child grows, the valve area may renarrow, and it may be necessary to undergo another valvuloplasty to open the valve further. Some children will need to undergo another operation to make sure that enough blood gets to the lungs. […] If the right ventricle and its components are too small, the child may have to go down a single ventricle pathway for treatment. This is a staged surgical route ending up with a Fontan repair.

• #38 Pulmonary atresia with intact ventricular septum | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia-intact-ventricular-septum

  Shunting. This surgery uses a tube to create a new path for blood to flow to the pulmonary arteries. The tube is called a shunt. An example of this treatment is the Blalock-Taussig shunt procedure or RV-PA shunt. Once this is done, medicine is not needed to keep the ductus arteriosus open. Babies eventually outgrow this shunt. […] Glenn operation. This surgery most often is done between 4 and 6 months of age. The surgeon connects a large vein, called the superior vena cava, to the pulmonary artery. Another large vein keeps blood flowing to the right side of the heart. If this surgery is done without using a heart-lung machine, it’s called an off-pump Glenn procedure. […] 1.5 ventricle repair. This surgery may be done in babies with PA/IVS who have a relatively well-formed lower right heart chamber. A surgeon creates a pathway from the lower right heart chamber to the pulmonary artery. The Glenn procedure also is done. The term „1.5 ventricle” means that the lower right heart chamber is doing some but not all of the heart’s work.

• #39 Pulmonary Atresia with intact ventricular septum | Little Hearts Matter

  https://www.lhm.org.uk/pulmonary-atresia/

  Pulmonary Atresia with intact ventricular septum is a congenital heart condition (a problem that a baby is born with). Below is a description of the heart condition and possible treatments offered. […] Possible treatments for single ventricle conditions that affect the flow of blood to the lungs include surgical treatment, supported comfort care, and transplantation. […] The aim of surgical treatment is to re-route the blood through the right side of the heart, bypassing the blockages on the left side. This surgery is offered in three stages over three or more years and involves extremely high-risk, open-heart surgery. […] Heart transplantation is one of the possible treatments for single ventricle heart disease, but it is not offered as a first treatment within the United Kingdom for the following reasons; very few donor hearts small enough for a baby are available in the United Kingdom and babies with a single ventricle heart would not survive long enough for a donor to become available.

• #40 Pulmonary Atresia with intact ventricular septum | Little Hearts Matter

  https://www.lhm.org.uk/pulmonary-atresia/

  The first stage of treatment may involve shunts, ductal stenting, banding, or septostomy/sepectomy to manage blood flow. […] The second stage of treatment, known as the Cavo-Pulmonary Connection, is normally performed at between three and twelve months of age. […] The final stage of surgery will be performed as the child starts to show that they need more blood flow to the lungs. […] The Fontan procedure or Total Cavo-Pulmonary Connection aims to more permanently separate the blue (deoxygenated) blood supply and the red (oxygenated) blood supply.

• #41 Pulmonary atresia with intact ventricular septum | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia-intact-ventricular-septum

  Fontan procedure. If the lower right lower heart chamber stays too small to do its work, surgeons may make a new path for blood to flow. The pathway lets most, if not all, of the blood coming to the heart to go into the pulmonary artery. The Fontan procedure is most often done when a child is 3 or 4 years old. […] Hybrid procedures. These are surgical and catheter treatments that are done at the same time. They’re sometimes done without a heart-lung machine. […] After treatment, babies with pulmonary atresia with intact ventricular septum, also called PA/IVS, should have regular health checkups with a heart doctor trained to treat children. This type of healthcare professional is called a pediatric cardiologist. Due to advances in treatment and technology, many people with PA/IVS live into adulthood. Adults with the condition should be followed by a doctor with training in adult congenital heart diseases.

• #42 Pulmonary atresia – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_atresia

  The type of surgery recommended depends on the size of the right ventricle and the pulmonary artery. If the right ventricle is small and unable to act as a pump, the surgery performed would be the Fontan procedure. In this three-stage procedure, the right atrium is disconnected from the pulmonary circulation. The systemic venous return goes directly to the lungs, bypassing the heart. Very young children with elevated pulmonary vascular resistance may not able to undergo the Fontan procedure. Cardiac catheterization may be done to determine the resistance before going ahead with the surgery.

• #43 Pulmonary atresia with intact ventricular septum | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia-intact-ventricular-septum

  Shunting. This surgery uses a tube to create a new path for blood to flow to the pulmonary arteries. The tube is called a shunt. An example of this treatment is the Blalock-Taussig shunt procedure or RV-PA shunt. Once this is done, medicine is not needed to keep the ductus arteriosus open. Babies eventually outgrow this shunt. […] Glenn operation. This surgery most often is done between 4 and 6 months of age. The surgeon connects a large vein, called the superior vena cava, to the pulmonary artery. Another large vein keeps blood flowing to the right side of the heart. If this surgery is done without using a heart-lung machine, it’s called an off-pump Glenn procedure. […] 1.5 ventricle repair. This surgery may be done in babies with PA/IVS who have a relatively well-formed lower right heart chamber. A surgeon creates a pathway from the lower right heart chamber to the pulmonary artery. The Glenn procedure also is done. The term „1.5 ventricle” means that the lower right heart chamber is doing some but not all of the heart’s work.

• #44 Pulmonary Atresia (PA) | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/pa

  If the tricuspid valve and right ventricle are normal sized, the child should be able to have surgery to bring blood from the right ventricle to the lungs. […] If the tricuspid valve and right ventricle size is in between (not very small, but not normal sized), then the child may require a combination of the above surgeries so the right ventricle does not have to handle all the blood that would normally go through it to the lungs. […] Finally, in some patients with significant coronary abnormalities who do not have good blood supply to their heart muscle, a heart transplant may be required.

• #45 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  Patients with adequate and functionally tripartite right ventricle size, a tricuspid valve z-score 2.5, and normal coronary artery anatomy benefit from biventricular repair, radiofrequency perforation of the pulmonary valve for pulmonary atresia, and surgical right ventricular outflow tract reconstruction for long-segment atresia. […] Primary heart transplantation might be a reasonable approach in patients with myocardial ischemia related to coronary artery abnormalities.

• #46 Pulmonary atresia with intact ventricular septum | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia-intact-ventricular-septum

  Shunting. This surgery uses a tube to create a new path for blood to flow to the pulmonary arteries. The tube is called a shunt. An example of this treatment is the Blalock-Taussig shunt procedure or RV-PA shunt. Once this is done, medicine is not needed to keep the ductus arteriosus open. Babies eventually outgrow this shunt. […] Glenn operation. This surgery most often is done between 4 and 6 months of age. The surgeon connects a large vein, called the superior vena cava, to the pulmonary artery. Another large vein keeps blood flowing to the right side of the heart. If this surgery is done without using a heart-lung machine, it’s called an off-pump Glenn procedure. […] 1.5 ventricle repair. This surgery may be done in babies with PA/IVS who have a relatively well-formed lower right heart chamber. A surgeon creates a pathway from the lower right heart chamber to the pulmonary artery. The Glenn procedure also is done. The term „1.5 ventricle” means that the lower right heart chamber is doing some but not all of the heart’s work.

• #47 Pulmonary atresia with intact ventricular septum | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia-intact-ventricular-septum

  Fontan procedure. If the lower right lower heart chamber stays too small to do its work, surgeons may make a new path for blood to flow. The pathway lets most, if not all, of the blood coming to the heart to go into the pulmonary artery. The Fontan procedure is most often done when a child is 3 or 4 years old. […] Hybrid procedures. These are surgical and catheter treatments that are done at the same time. They’re sometimes done without a heart-lung machine. […] After treatment, babies with pulmonary atresia with intact ventricular septum, also called PA/IVS, should have regular health checkups with a heart doctor trained to treat children. This type of healthcare professional is called a pediatric cardiologist. Due to advances in treatment and technology, many people with PA/IVS live into adulthood. Adults with the condition should be followed by a doctor with training in adult congenital heart diseases.

• #48

  https://link.springer.com/article/10.1007/s00246-024-03566-x

  The approach regarding supplementary procedures during the initial intervention differed in the two cohorts. […] The hybrid approach avoids CPB while still offering the opportunity to place a mBTS if needed and it decreases the risk of perforating the right ventricle or the pulmonary artery. […] The findings of this retrospective population-based multicenter study demonstrate that both catheter-based intervention and heart surgery are safe procedures with a low risk of complications.

• #49 Pulmonary atresia with intact ventricular septum: contemporary surgical and treatment approaches – FCV

  https://www.fcv.org/co/editorial-fcv/articulos-editorial/pulmonary-atresia-with-intact-ventricular-septum-contemporary-surgical-and-treatment-approaches

  Although catheter-based therapies have advanced, surgical intervention continues to be crucial for numerous PA-IVS patients, especially those with severe right ventricular hypoplasia or coronary abnormalities. […] Surgical options include right ventricular outflow tract reconstruction, systemic-to-pulmonary artery shunt creation, or one-and-a-half ventricle repair involving a Glenn shunt. […] A hybrid approach, combining surgical and catheter-based interventions, is also used in specific patients. […] The management of PA-IVS requires a personalized approach, with treatment options ranging from catheter-based interventions and fetal procedures to traditional surgical repairs and hybrid approaches. […] Advances in catheter-based technologies have made it possible for some patients to avoid surgery altogether, while fetal interventions offer hope for improving right ventricular development before birth.

• #50 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  Patients with adequate and functionally tripartite right ventricle size, a tricuspid valve z-score 2.5, and normal coronary artery anatomy benefit from biventricular repair, radiofrequency perforation of the pulmonary valve for pulmonary atresia, and surgical right ventricular outflow tract reconstruction for long-segment atresia. […] Primary heart transplantation might be a reasonable approach in patients with myocardial ischemia related to coronary artery abnormalities.

• #51 18: Pulmonary Atresia with Intact Ventricular Septum | Veterian Key

  https://veteriankey.com/18-pulmonary-atresia-with-intact-ventricular-septum/

  If antegrade flow is established and significant cyanosis persists, an alternate source of pulmonary blood flow may be needed. […] In many case series, there is a subset of patients who thrive after a right ventricular outflow tract procedure and a shunt, yet fail attempts to remove accessory sources of pulmonary blood flow from the circulation. […] These strategies are not available to patients with right ventricular-dependent coronary artery circulation due to their risk for myocardial ischemia and death if the RV is decompressed and the ventricular pressure decreases. […] Management of patients with PA/IVS and massive RV dilation is challenging. […] Some of these patients will require single ventricle strategy.

• #52 Pulmonary Atresia With Intact Ventricular Septum and Right Ventricle Dependent Coronary Circulation: Location of Coronary Obstruction Impacts Survival

  https://watch.giblib.com/video/6666

  This is the treatment pathways for the 18 patients with proximal coronary obstruction. One patient underwent primary transplant. One patient died prior to intervention. 13 patients had to be shunted to stabilize their pulmonary blood flow. And of these patients, five have successfully achieved a Fontan circulation or are awaiting completion. […] The overall outcomes for these patients, patients with distal obstruction were more likely to have achieved the Fontan circulation than patients with proximal obstruction. Inversely, patients with proximal obstruction were therefore more likely to have died or required a transplant. […] By univariate analysis, looking at risk factors associated with transplant or death, a higher birth weight was associated with decreased risk for transplant or death, and proximal obstruction was associated with increased risk relative to patients in the distal obstruction. […] In conclusion, proximal coronary obstruction is associated with decreased transplant free survival relative to distal coronary obstruction and that this may be a potential branch point in the management of these patients.

• #53 Pulmonary Atresia with intact ventricular septum | Little Hearts Matter

  https://www.lhm.org.uk/pulmonary-atresia/

  Pulmonary Atresia with intact ventricular septum is a congenital heart condition (a problem that a baby is born with). Below is a description of the heart condition and possible treatments offered. […] Possible treatments for single ventricle conditions that affect the flow of blood to the lungs include surgical treatment, supported comfort care, and transplantation. […] The aim of surgical treatment is to re-route the blood through the right side of the heart, bypassing the blockages on the left side. This surgery is offered in three stages over three or more years and involves extremely high-risk, open-heart surgery. […] Heart transplantation is one of the possible treatments for single ventricle heart disease, but it is not offered as a first treatment within the United Kingdom for the following reasons; very few donor hearts small enough for a baby are available in the United Kingdom and babies with a single ventricle heart would not survive long enough for a donor to become available.

• #54 Pulmonary atresia with intact ventricular septum: contemporary surgical and treatment approaches – FCV

  https://www.fcv.org/co/editorial-fcv/articulos-editorial/pulmonary-atresia-with-intact-ventricular-septum-contemporary-surgical-and-treatment-approaches

  This approach has proven to be both safe and effective, with numerous patients exhibiting substantial right ventricular growth, enabling subsequent interventions directed at establishing a biventricular circulation. […] Transcatheter pulmonary valve replacement has been established as an effective therapeutic option for patients necessitating delayed valve replacement, enabling some individuals with PA-IVS to forgo open-heart surgery altogether. […] Fetal cardiac intervention is an emerging therapeutic approach for critical congenital heart defects, such as PA-IVS. […] Specifically, fetal pulmonary valvuloplasty is performed to alleviate right ventricular outflow tract obstruction, foster right ventricular growth, and potentially improve the likelihood of achieving a biventricular circulation after birth.

• #55 Pulmonary atresia with intact ventricular septum

  https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/heart/pulmonary-atresia-with-intact-ventricular-septum.html

  Pulmonary atresia with intact ventricular septum encompasses a group of cardiac anomalies that have in common a complete obstruction of the valve that connects the right ventricle (RV) and the pulmonary artery that carries blood to the lungs, in the presence of an intact interventricular septum. […] Treatment of PA-IVS may include a combination of medications, procedures, or surgery to correct the congenital heart defect. […] Intrauterine cardiac intervention in selected cases of PA-IVS may improve the prognosis of these fetuses. […] All patients with a prenatal diagnosis of PA-IVS should be born in a centre with high-risk pregnancy specialists, access to pediatric heart specialists, and an intensive care nursery.

• #56 Pulmonary Atresia With Intact Ventricular Septum | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28009

  Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure. Due to the complexity and heterogeneity of this disorder, no single method is effective for all patients. Even though biventricular repair is the ideal and preferred surgical approach, managing this disorder must be highly individualized. […] The preferred surgical or transcatheter intervention is influenced by several variables, which include the size and function of the tricuspid valve, the anatomy of the right ventricle and coronary arteries, and the type of pulmonary atresia. […] Clinicians should also recall that patients in categories 2, 3, and 4 require a systemic-to-pulmonary artery shunt or stenting of the PDA in the immediate newborn period.

• #57 Pulmonary Atresia With Intact Ventricular Septum | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-intact-ventricular-septum-5/

  This chapter describes the disease spectrum of pulmonary atresia with intact ventricular septum (PA/IVS). […] PA/IVS is a highly variable continuum of severe congenital heart disease. […] Many programs have adopted a primary transplant strategy for these fragile patients. […] For those patients whose anatomy allows for potential one-and-one-half- or two-ventricle pathways we have adapted a hybrid treatment strategy for early palliation followed by completion of biventricular repair or one-and-one-half-ventricle repair, usually within the first year of life with excellent early and midterm results. […] Outcomes for the treatment of PA/IVS have improved significantly over the past several decades. […] Because of this wide spectrum and variability in disease severity, mainly related to tricuspid valve size, RV chamber anatomy, and coronary artery anomalies, complex algorithms for treatment are required to ensure optimum outcomes for these often fragile patients.

• #58

  https://link.springer.com/article/10.1007/s00246-025-03769-w

  Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. […] The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). […] We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS. […] The primary outcome assessed was survival. […] Our data also show low mortality in patients with PA/IVS. One patient underwent a neonatal transplant, and only 24.1% required single ventricle circulation, with 65.5% completing either a 2V or 1.5V circulation. Survival was 93.1%, with only two mortalities. […] This method promotes regular reassessments aimed at achieving the best possible survival rates with minimal morbidity and a high likelihood of complete or near complete septation.

• #59 Management algorithm in pulmonary atresia with intact ventricular septum – PubMed

  https://pubmed.ncbi.nlm.nih.gov/16572430/

  Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 – ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.

• #60 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. This action is vital for the preoperative survival of these patients, as the ductus arteriosus is the sole source of pulmonary blood flow. […] Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure. Due to the complexity and heterogeneity of this disorder, no single method is effective for all patients. […] The preferred surgical or transcatheter intervention is influenced by several variables, which include the size and function of the tricuspid valve, the anatomy of the right ventricle and coronary arteries, and the type of pulmonary atresia. […] Single-ventricular repair (bidirectional Glenn procedure followed by Fontan) should be considered in patients with severe right ventricular hypoplasia and RVDCC.

• #61

  https://link.springer.com/article/10.1007/s00246-025-03769-w

  Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. […] The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). […] We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS. […] The primary outcome assessed was survival. […] Our data also show low mortality in patients with PA/IVS. One patient underwent a neonatal transplant, and only 24.1% required single ventricle circulation, with 65.5% completing either a 2V or 1.5V circulation. Survival was 93.1%, with only two mortalities. […] This method promotes regular reassessments aimed at achieving the best possible survival rates with minimal morbidity and a high likelihood of complete or near complete septation.

• #62 Pulmonary atresia with intact ventricular septum (PA/IVS) – UpToDate

  https://www.uptodate.com/contents/pulmonary-atresia-with-intact-ventricular-septum-pa-ivs

  Pulmonary atresia with intact ventricular septum (PA/IVS) is characterized by complete obstruction to right ventricular (RV) outflow with varying degrees of RV and tricuspid valve (TV) hypoplasia. Blood is thus unable to flow from the RV to the pulmonary artery and lungs, and an alternative source of pulmonary blood flow is required for survival. If untreated, PA/IVS is almost always fatal. Outcomes of surgical interventions are improving, with a five-year survival rate of approximately 80 percent. […] The definition, anatomy, physiology, clinical presentation, management (including follow-up care), and outcome of PA/IVS will be reviewed here. […] PA/IVS is a rare congenital cardiac defect that consists of atresia of the pulmonary valve resulting in an absent connection between the right ventricular outflow tract (RVOT) and pulmonary arteries as well as an IVS that allows no connection between the right and left ventricles.

• #63

  https://link.springer.com/article/10.1007/s00246-024-03566-x

  The approach regarding supplementary procedures during the initial intervention differed in the two cohorts. […] The hybrid approach avoids CPB while still offering the opportunity to place a mBTS if needed and it decreases the risk of perforating the right ventricle or the pulmonary artery. […] The findings of this retrospective population-based multicenter study demonstrate that both catheter-based intervention and heart surgery are safe procedures with a low risk of complications.

• #64 Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum | Heart

  https://heart.bmj.com/content/95/18/1520.short

  Objective: To review the outcome of patients with pulmonary atresia with intact ventricular septum after interventional perforation of the pulmonary valve, to assess the capability of this procedure to avoid neonatal or late intervention and to obtain a long-term biventricular repair. […] The results confirm that percutaneous interventional perforation is an effective first-stage procedure in patients with pulmonary atresia with intact ventricular septum. The right heart appeared to be adequate to maintain a long-term biventricular circulation in the large majority of cases.

• #65

  https://link.springer.com/article/10.1007/s00246-025-03769-w

  Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. […] The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). […] We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS. […] The primary outcome assessed was survival. […] Our data also show low mortality in patients with PA/IVS. One patient underwent a neonatal transplant, and only 24.1% required single ventricle circulation, with 65.5% completing either a 2V or 1.5V circulation. Survival was 93.1%, with only two mortalities. […] This method promotes regular reassessments aimed at achieving the best possible survival rates with minimal morbidity and a high likelihood of complete or near complete septation.

• #66

  https://link.springer.com/article/10.1007/s00246-025-03769-w

  Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. […] The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). […] We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS. […] The primary outcome assessed was survival. […] Our data also show low mortality in patients with PA/IVS. One patient underwent a neonatal transplant, and only 24.1% required single ventricle circulation, with 65.5% completing either a 2V or 1.5V circulation. Survival was 93.1%, with only two mortalities. […] This method promotes regular reassessments aimed at achieving the best possible survival rates with minimal morbidity and a high likelihood of complete or near complete septation.

• #67 Pulmonary atresia with intact ventricular septum | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia-intact-ventricular-septum

  Fontan procedure. If the lower right lower heart chamber stays too small to do its work, surgeons may make a new path for blood to flow. The pathway lets most, if not all, of the blood coming to the heart to go into the pulmonary artery. The Fontan procedure is most often done when a child is 3 or 4 years old. […] Hybrid procedures. These are surgical and catheter treatments that are done at the same time. They’re sometimes done without a heart-lung machine. […] After treatment, babies with pulmonary atresia with intact ventricular septum, also called PA/IVS, should have regular health checkups with a heart doctor trained to treat children. This type of healthcare professional is called a pediatric cardiologist. Due to advances in treatment and technology, many people with PA/IVS live into adulthood. Adults with the condition should be followed by a doctor with training in adult congenital heart diseases.

• #68

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-intact-ventricular-septum

  After treatment, babies with pulmonary atresia should have regular health checkups, ideally with a pediatric heart doctor. This type of healthcare professional is called a pediatric cardiologist. Due to advances in treatment and technology, many people with pulmonary atresia with intact ventricular septum (PA/IVS) live into adulthood. Adults with PA/IVS should be followed by a doctor with specialized training in adult congenital heart disease.

• #69 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including: […] Usually, the right ventricle is well developed and can pump blood to your babys lungs. Surgery involves: […] In this case, your babys right ventricle is usually poorly developed. They need a series of three operations during the first few years of their lives. […] After heart surgery, your child will need to spend a week or two in the hospital, with some of that time in the intensive care unit. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your babys birth, they can treat your newborn to improve their oxygen circulation. […] Medical procedures and surgeries can improve your childs condition, but they arent cures.

• #70 SSA – POMS: DI 23022.585 – Pulmonary Atresia – 08/28/2020

  https://secure.ssa.gov/poms.nsf/%20lnx/0423022585

  Pulmonary atresia is a rare congenital heart disease in which the pulmonary valve does not form properly. […] Surgery is necessary shortly after birth to form a shunt from the systemic circulation to the pulmonary circulation in order to oxygenate the blood. […] Infants are given medication (i.e. Prostaglandin) to help keep the blood vessel open between the pulmonary artery and the aorta (patent ductus arteriosis or PDA) until the initial shunt surgery can occur. Later, multi-staged surgeries to repair the vessel, open-heart surgery to repair or replace a pulmonary valve, reconstruction of the heart chambers, and heart transplant have been used to treat patients with this defect. […] Infants surviving into adulthood should be monitored by a cardiologist specializing in the care of adults with congenital heart disease to assess medication needs, surgery, and infection throughout their lifetime.

• #71 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  Pulmonary atresia with intact ventricular septum is a rare congenital heart disease comprising less than 1% of all heart defects, characterized by membranous or muscular atresia of the right ventricular outflow tract without any ventricular communication, often involving underdevelopment of the right ventricle and tricuspid valve. […] This activity for healthcare professionals is designed to enhance the learner’s competence in identifying pulmonary atresia with intact ventricular septum, performing the recommended evaluation, and implementing an appropriate interprofessional approach when managing this condition. […] Managing PA-IVS begins with prostaglandin infusion to maintain ductal patency, which is crucial for survival preoperatively. Intervention, either catheter-based or surgical, is necessary for neonates. Treatment is highly individualized, encompassing biventricular repair, systemic-to-pulmonary shunts, single-ventricular repair, or heart transplantation, depending on the characteristics of the right ventricle and tricuspid valve, as well as the anatomy of the coronary arteries.

• #72 Pulmonary Atresia With Intact Ventricular Septum | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-intact-ventricular-septum-5/

  These algorithms have resulted in improved survival in patients diagnosed with PA/IVS, particularly in the more severe forms of the spectrum. […] Patients are maintained on prostaglandin infusion after birth, and standard workup for newborns with complex congenital heart disease is undertaken. […] PA/IVS is a rare form of complex congenital heart disease with significant morphologic heterogeneity. […] Because of the significant morphologic heterogeneity seen in PA/IVS, repair or palliation strategies have a wide variation, from single-ventricle palliation to complete biventricular repair.

• #73 Pulmonary atresia with intact ventricular septum – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-intact-ventricular-septum/cdc-20396714

  Pulmonary atresia with intact ventricular septum, also called PA/IVS, is a rare heart condition that’s present at birth. […] Treatment for pulmonary atresia with intact ventricular septum may include medicines and one or more procedures or surgeries to fix the heart. […] Babies with pulmonary atresia need treatment right away. It’s best to get treatment at a medical center with surgeons and other healthcare professionals who have experience with complex heart conditions present at birth. […] Treatment for pulmonary atresia with intact ventricular septum, also called PA/IVS, may include medicines and one or more procedures or surgeries. […] Medicine may be given through an IV to keep the ductus arteriosus open. This is not a permanent treatment for pulmonary atresia. But it keeps the baby stable until a surgery or procedure can be done to permanently fix blood flow.

• #74

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-intact-ventricular-septum

  After treatment, babies with pulmonary atresia should have regular health checkups, ideally with a pediatric heart doctor. This type of healthcare professional is called a pediatric cardiologist. Due to advances in treatment and technology, many people with pulmonary atresia with intact ventricular septum (PA/IVS) live into adulthood. Adults with PA/IVS should be followed by a doctor with specialized training in adult congenital heart disease.

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