Pulmonary Atresia With Intact Ventricular SeptumNCT07095829
Prognostic Model of Postnatal Circulation in Pulmonary Atresia-critical Stenosis With Intact Ventricular Septum
An observational study of Pulmonary Atresia With Intact Ventricular Septum and Congenital Heart Disease, run by Hospital Universitario 12 de Octubre at 2 sites in 2 countries.
Trial summary
Pulmonary atresia (PA)/critical stenosis (CS) with intact ventricular septum (PA/CS-IVS) is a rare congenital heart disease (CHD), that presents heterogeneously. Prognosis is conditioned by the possibility of achieving a primary repair with biventricular circulation (BV) or a one-and-a-half ventricle solution vs. Read more about this study
- Who is it for?
- Ages 16 to 28 weeks
- Conditions the study is for
- Pulmonary Atresia With Intact Ventricular Septum, Congenital Heart Disease, Fetal Cardiac Disorder
- How many people
- 150 participants
- Where
- 2 sitesin 2 countries
- Study dates
- 1 Feb 2024to 31 Dec 2029
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 16 to 28 weeks
- All sexes
- No healthy volunteers
- Child
Study population: Screening data: All pregnant women with a fetus with suspected PA-CS/IVS will be screened for eligibility. If they meet all the inclusion criteria and none of the exclusion ones, they will be offered to participate. A complete count of screened, eligible, and included participants will be detailed in the final report of the study. Eligibility: As described in the next section, cases must meet all the inclusion criteria, and none of the exclusion ones.
Who may be able to join
- Absence of flow at the pulmonary valve (PA) or presence of thickened and domed. pulmonary valve cusps with a pinhole jet of flow.
- Doppler evidence of ductal-dependent pulmonary circulation.
- Intact ventricular septum.
Who cannot join
- Poor imaging windows and incomplete/poor quality scan
- Termination of pregnancy
- Cases initially included that undergo prenatal pulmonary valvuloplasty later on in pregnancy.
- Unconfirmed PA-CS/IVS at birth.
- Functional PA-CS/IVS (Ebstein malformation, monochorionic twins)
- Any associated cardiac defect except persistent left superior vena cava and aberrant right subclavian artery.
- Any significant (i.e that might influence outcome) extracardiac anomaly and/or known genetic syndromes. Also, if such a condition is present at inclusion but diagnosed only after birth, the case will be retrospectively excluded.
Trial locations and contacts
2 sites in 2 countries. Contact the study team or a site near you to ask about taking part.
2 sites in 2 countries
Spain1 site, 1 recruiting
- Recruiting
Hospital Universitario 12 de Octubre
Madrid, 28041
Cecilia Villalain, MD PhD, cecilia.villalain@salud.madrid.org
About this study
Official title: Development and Validation of a Prognostic Model of Postnatal Circulation in Fetuses With a Diagnosis of Pulmonary Atresia-critical Stenosis With Intact Ventricular Septum A Prospective Observational Cohort Study
Pulmonary atresia (PA)/critical stenosis (CS) with intact ventricular septum (PA/CS-IVS) is a rare congenital heart disease (CHD), that presents heterogeneously. Prognosis is conditioned by the possibility of achieving a primary repair with biventricular circulation (BV) or a one-and-a-half ventricle solution vs. a palliative approach bound to a univentricular (UV) circulation in which both survival and quality of life are significantly impaired. Predicting UV circulation prenatally is still a challenge.
The aim of this study is: 1/ to evaluate the natural history of the disease and develop a prognostic model for the prediction of transplantation-free survival with a biventricular or a one-and-a-half repair at 2 years postnatal age 2/ To develop a model to predict the risk of right ventricle dependent coronary circulation 3/ To evaluate prenatal and postnatal outcomes in non-intervened fetuses with a confirmed postnatal diagnosis of PA-CS/IVS including Intrauterine death, neonatal/Infant death, number of required postnatal procedures, need for oxygen support, need for cardiac transplantation
Read the detailed description
This is an international retrospective and prospective observational cohort study including non-intervened fetuses with a diagnosis of PA-CS/IVS between 16+0 and 28+6 weeks of gestation.
A baseline fetal ultrasound examination will be recorded for all included cases. The primary aim of the study is to develop a prognostic and validate model for the type of postnatal circulation (transplantation-free survival with a biventricular or a one-and-a-half repair at 2 years postnatal age vs univentricular palliation or transplanted).
In order to maximize the number of included cases in this rare entity, the model will be developed on retrospective data from 2013 until 2023 and validated in a prospective cohort. Cases will be recruited in referral centers regardless of their offering of fetal pulmonary valvuloplasty. However, only non-prenatally-intervened cases will be used for analysis. Fetal and postnatal echocardiographic examinations will be reviewed by a core laboratory to confirm eligibility for inclusion and identify potential measurement errors.
Data from at least one fetal echo (the first diagnostic evaluation at the referral center) and one postnatal (the first one performed after birth) will be collected, each containing a comprehensive set of two-dimensional and Doppler measurements. Additionally, one additional follow-up scan performed at least 8 weeks apart from the baseline diagnostic scan (ideally between 28 and 32 weeks) will be included in the study as well. Analysis of the prenatal change of dimensions of the right heart structures and selected hemodynamic parameters will enable comparisons between centers regardless of off-protocol center-specific postnatal treatment policies.
- Conditions
- Pulmonary Atresia With Intact Ventricular Septum, Congenital Heart Disease, Fetal Cardiac Disorder
- Being tested
- No intervention
- Keywords
- Prognosis, Prenatal diagnosis, Ultrasound
- Medical subject headings
- Pulmonary Atresia with Intact Ventricular Septum, Heart Defects, Congenital
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
No interventionOther
Evaluation of natural history of the disease
| Study group | Role | What they receive |
|---|---|---|
| Retrospective cohort | Retrospective cases that meet all the inclusion criteria and none of the exclusion criteria from 2013 until 2023 will be included for model development. They should be non-intervened cases in order to assess the natural history of the disease. Receives: Other: No intervention | |
| Prospective cohort | Prospectively recruited cases will be included when meeting all the inclusion criteria and none of the exclusion ones. This cohort will serve as a validation cohort for the previously developed model. The nature of the study is observational. Clinicians will not be given the result of the model during the study and clinical practice will be routine. Receives: Other: No intervention |
What is being measured
The primary outcome is the main question the study is designed to answer.
Prediction of postnatal circulation
Transplantation-free survival with a non-univentricular circulation (biventricular or a one-and-a-half repair) at 2 years postnatal age: yes/no
Measured: 3 years (1 recruitment, 2 follow-up)
Secondary outcomes (7)
Prediction of coronary circulation
Prediction of right ventricle-dependent coronary circulation confirmed by cath or autopsy (yes/no)
Measured: 3 years (1 recruitment, 2 follow-up)
Intrauterine death
Intrauterine death (yes/no)
Measured: 3 years (1 recruitment, 2 follow-up)
Neonatal/Infant death in the first 2 years
Neonatal/Infant death in the first 2 years (yes/no)
Measured: 3 years (1 recruitment, 2 follow-up)
Number of required postnatal surgical and catheter interventional procedure
Number of required postnatal surgical and catheter interventional procedures
Measured: 3 years (1 recruitment, 2 follow-up)
Type of required postnatal surgical and catheter interventional procedures
Type of required postnatal surgical and catheter interventional procedures
Measured: 3 years (1 recruitment, 2 follow-up)
O2 saturation at 30 days (%)
O2 saturation at 30 days (%)
Measured: 3 years (1 recruitment, 2 follow-up)
Transplantation in the first two years
Transplantation in the first two years (yes/no)
Measured: 3 years (1 recruitment, 2 follow-up)
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted31 Jul 2025
- Study start1 Feb 2024
- Primary completion1 Feb 2028 (estimated)
- Study completion31 Dec 2029 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for Pulmonary Atresia With Intact Ventricular SeptumStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 150 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Hospital Universitario 12 de Octubre
- Sponsor type
- University, hospital or other
- Collaborators
- Instituto de Salud Carlos III
- Responsible party
- Sponsor
- Investigators
- Cecilia Villalaín González, MD PhD, Principal investigator, Instituto de investigación imas12Alberto Galindo Izquierdo, MD PhD, Principal investigator, Instituto de investigación imas12
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07095829
- Sponsor's protocol ID
- PROGPAIVS
- Status last verified
- Sep 2025
- First submitted
- 18 Mar 2024
- Last updated
- 15 Sep 2025
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
Villalain C, Moon-Grady AJ, Mellander M, Okhman A, Lopez IL, Gomez-Montes E, Conti B, Albert L, Galindo A. Development and validation of a prognostic model of postnatal circulation in fetuses with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum: protocol for an observational cohort study with prospective validation. BMC Pregnancy Childbirth. 2026 Mar 17. doi: 10.1186/s12884-026-08947-z. Online ahead of print.
PubMed 41845255
Study at a glance
From the registry record, last updated by the sponsor on 15 Sep 2025. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Development and Validation of a Prognostic Model of Postnatal Circulation in Fetuses With a Diagnosis of Pulmonary Atresia-critical Stenosis With Intact Ventricular Septum A Prospective Observational Cohort Study
- ClinicalTrials.gov ID
- NCT07095829
- Acronym / sponsor ID
- PROGPAIVS
- Conditions
- Pulmonary Atresia With Intact Ventricular Septum, Congenital Heart Disease, Fetal Cardiac Disorder
- Keywords
- Prognosis, Prenatal diagnosis, Ultrasound
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 150 participants (estimated)
- Lead sponsor
- Hospital Universitario 12 de OctubreUniversity, hospital or other
- Collaborators
- Instituto de Salud Carlos III
- Study start
- 1 Feb 2024 (actual)
- Primary completion
- 1 Feb 2028 (estimated)
- Study completion
- 31 Dec 2029 (estimated)
- First posted
- 31 Jul 2025 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Pulmonary atresia (PA)/critical stenosis (CS) with intact ventricular septum (PA/CS-IVS) is a rare congenital heart disease (CHD), that presents heterogeneously. Prognosis is conditioned by the possibility of achieving a primary repair with biventricular circulation (BV) or a one-and-a-half ventricle solution vs. a palliative approach bound to a univentricular (UV) circulation in which both survival and quality of life are significantly impaired. Predicting UV circulation prenatally is still a challenge.
The aim of this study is: 1/ to evaluate the natural history of the disease and develop a prognostic model for the prediction of transplantation-free survival with a biventricular or a one-and-a-half repair at 2 years postnatal age 2/ To develop a model to predict the risk of right ventricle dependent coronary circulation 3/ To evaluate prenatal and postnatal outcomes in non-intervened fetuses with a confirmed postnatal diagnosis of PA-CS/IVS including Intrauterine death, neonatal/Infant death, number of required postnatal procedures, need for oxygen support, need for cardiac transplantation
Detailed description
This is an international retrospective and prospective observational cohort study including non-intervened fetuses with a diagnosis of PA-CS/IVS between 16+0 and 28+6 weeks of gestation.
A baseline fetal ultrasound examination will be recorded for all included cases. The primary aim of the study is to develop a prognostic and validate model for the type of postnatal circulation (transplantation-free survival with a biventricular or a one-and-a-half repair at 2 years postnatal age vs univentricular palliation or transplanted).
In order to maximize the number of included cases in this rare entity, the model will be developed on retrospective data from 2013 until 2023 and validated in a prospective cohort. Cases will be recruited in referral centers regardless of their offering of fetal pulmonary valvuloplasty. However, only non-prenatally-intervened cases will be used for analysis. Fetal and postnatal echocardiographic examinations will be reviewed by a core laboratory to confirm eligibility for inclusion and identify potential measurement errors.
Data from at least one fetal echo (the first diagnostic evaluation at the referral center) and one postnatal (the first one performed after birth) will be collected, each containing a comprehensive set of two-dimensional and Doppler measurements. Additionally, one additional follow-up scan performed at least 8 weeks apart from the baseline diagnostic scan (ideally between 28 and 32 weeks) will be included in the study as well. Analysis of the prenatal change of dimensions of the right heart structures and selected hemodynamic parameters will enable comparisons between centers regardless of off-protocol center-specific postnatal treatment policies.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
- Number of arms
- 2
| Arm | Type | Receives |
|---|---|---|
| Retrospective cohort | – | Other: No interventionRetrospective cases that meet all the inclusion criteria and none of the exclusion criteria from 2013 until 2023 will be included for model development. They should be non-intervened cases in order to assess the natural history of the disease. |
| Prospective cohort | – | Other: No interventionProspectively recruited cases will be included when meeting all the inclusion criteria and none of the exclusion ones. This cohort will serve as a validation cohort for the previously developed model. The nature of the study is observational. Clinicians will not be given the result of the model during the study and clinical practice will be routine. |
Who may qualify
- Ages
- 16 to 28 weeks
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child
- Study population
- Screening data: All pregnant women with a fetus with suspected PA-CS/IVS will be screened for eligibility. If they meet all the inclusion criteria and none of the exclusion ones, they will be offered to participate. A complete count of screened, eligible, and included participants will be detailed in the final report of the study. Eligibility: As described in the next section, cases must meet all the inclusion criteria, and none of the exclusion ones.
Key inclusion criteria (3 listed)
- Absence of flow at the pulmonary valve (PA) or presence of thickened and domed. pulmonary valve cusps with a pinhole jet of flow.
- Doppler evidence of ductal-dependent pulmonary circulation.
- Intact ventricular septum.
Key exclusion criteria (7 listed)
- Poor imaging windows and incomplete/poor quality scan
- Termination of pregnancy
- Cases initially included that undergo prenatal pulmonary valvuloplasty later on in pregnancy.
- Unconfirmed PA-CS/IVS at birth.
- Functional PA-CS/IVS (Ebstein malformation, monochorionic twins)
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| No interventionEvaluation of natural history of the disease | Other | Prospective cohort, Retrospective cohort |
Primary outcome
Prediction of postnatal circulation
Time frame: 3 years (1 recruitment, 2 follow-up)
Transplantation-free survival with a non-univentricular circulation (biventricular or a one-and-a-half repair) at 2 years postnatal age: yes/no
7 secondary outcomes
Prediction of coronary circulation
Time frame: 3 years (1 recruitment, 2 follow-up)
Intrauterine death
Time frame: 3 years (1 recruitment, 2 follow-up)
Neonatal/Infant death in the first 2 years
Time frame: 3 years (1 recruitment, 2 follow-up)
Number of required postnatal surgical and catheter interventional procedure
Time frame: 3 years (1 recruitment, 2 follow-up)
Type of required postnatal surgical and catheter interventional procedures
Time frame: 3 years (1 recruitment, 2 follow-up)
O2 saturation at 30 days (%)
Time frame: 3 years (1 recruitment, 2 follow-up)
Transplantation in the first two years
Time frame: 3 years (1 recruitment, 2 follow-up)
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 15 Sep 2025.
- Derived from this study: PubMed 41845255Villalain C, Moon-Grady AJ, Mellander M, Okhman A, Lopez IL, Gomez-Montes E, Conti B, Albert L, Galindo A. Development and validation of a prognostic model of postnatal circulation in fetuses with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum: protocol for an observational cohort study with prospective validation. BMC Pregnancy Childbirth. 2026 Mar 17. doi: 10.1186/s12884-026-08947-z. Online ahead of print.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Cecilia Villalaín González, MD PhD principal investigator
Instituto de investigación imas12
Alberto Galindo Izquierdo, MD PhD principal investigator
Instituto de investigación imas12
2 sites in 2 countries: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Pulmonary Atresia With Intact Ventricular Septum and Congenital Heart Disease, run by Hospital Universitario 12 de Octubre at 2 sites in 2 countries.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 3 inclusion and 7 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 15 Sep 2025. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 2 sites in Spain, Sweden. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Cecilia Villalain, MD PhD (+34913908310, cecilia.villalain@salud.madrid.org), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Prediction of postnatal circulation (time frame: 3 years (1 recruitment, 2 follow-up)). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT07095829, which also lists 1 linked publication.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 15 Sep 2025.