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Pulmonary atresia with intact ventricular septum

MedGen UID:
87491
•Concept ID:
C0344975
•
Congenital Abnormality
Synonym: Pulmonary atresia-intact ventricular septum syndrome
SNOMED CT: PAIV - Pulmonary atresia with intact ventricular septum (253590009); Pulmonary atresia with intact ventricular septum (253590009)
 
Monarch Initiative: MONDO:0009931
OMIM®: 265150
Orphanet: ORPHA1208

Definition

A rare form of cyanotic congenital heart malformation characterized by severe cyanosis and tachypnea. It presents significant morphologic diversity: at the end of the spectrum are patients with a mildly hypoplastic and tripartite right ventricle (RV) and mild tricuspid valve (TV) hypoplasia, and at the other end are patients with severe RV and TV hypoplasia, often with RV-dependent coronary circulation. [from ORDO]

Clinical features

From HPO
Hypoplastic right heart
MedGen UID:
451017
•Concept ID:
C0265856
•
Disease or Syndrome
Underdevelopment of the right-sided structures of the heart.
See: Feature record | Search on this feature
Pulmonary artery atresia
MedGen UID:
82723
•Concept ID:
C0265908
•
Congenital Abnormality
A congenital anomaly with a narrowing or complete absence of the opening between the right ventricle and the pulmonary artery.
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary atresia with intact ventricular septum
Follow this link to review classifications for Pulmonary atresia with intact ventricular septum in Orphanet.

Professional guidelines

PubMed

Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease.
Zhao L, Wang L, Xia H, Wu Y, Jiao X, Zhu H, Chen S, Sun K
Chin Med J (Engl) 2024 Jun 20;137(12):1431-1436. Epub 2023 Jul 24 doi: 10.1097/CM9.0000000000002796. PMID: 37488672Free PMC Article
Management algorithm in pulmonary atresia with intact ventricular septum.
Alwi M
Catheter Cardiovasc Interv 2006 May;67(5):679-86. doi: 10.1002/ccd.20672. PMID: 16572430
Comprehensive management of pulmonary atresia with intact ventricular septum.
Rao PS
Ann Thorac Surg 1985 Oct;40(4):409-13. doi: 10.1016/s0003-4975(10)60082-3. PMID: 3901947

Recent clinical studies

Etiology

Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.
Shirozu H, Kodama Y, Kuraoka A, Ishikawa Y, Nakano T, Kado H, Sagawa K
Pediatr Cardiol 2022 Oct;43(7):1653-1658. Epub 2022 May 13 doi: 10.1007/s00246-022-02898-w. PMID: 35562506
Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum.
Liu L, Wang H, Cui C, Li Y, Liu Y, Wang Y, Fan T, Peng B
Medicine (Baltimore) 2019 Oct;98(42):e17492. doi: 10.1097/MD.0000000000017492. PMID: 31626103Free PMC Article
Discontinuous Pulmonary Artery.
Eltayeb O, Mongé MC, Popescu AR, Sarwark AE, Harris T, Backer CL
World J Pediatr Congenit Heart Surg 2017 Jan;8(1):106-110. Epub 2016 Jun 22 doi: 10.1177/2150135115618871. PMID: 26993757
Hybrid approach for pulmonary atresia with intact ventricular septum: early single center results and comparison to the standard surgical approach.
Zampi JD, Hirsch-Romano JC, Goldstein BH, Shaya JA, Armstrong AK
Catheter Cardiovasc Interv 2014 Apr 1;83(5):753-61. Epub 2014 Jan 8 doi: 10.1002/ccd.25181. PMID: 23996932
Evolution of the management approach for pulmonary atresia with intact ventricular septum.
Mi YP, Chau AK, Chiu CS, Yung TC, Lun KS, Cheung YF
Heart 2005 May;91(5):657-63. doi: 10.1136/hrt.2004.033720. PMID: 15831657Free PMC Article

Diagnosis

Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum.
Liu L, Wang H, Cui C, Li Y, Liu Y, Wang Y, Fan T, Peng B
Medicine (Baltimore) 2019 Oct;98(42):e17492. doi: 10.1097/MD.0000000000017492. PMID: 31626103Free PMC Article
Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.
Chikkabyrappa SM, Loomba RS, Tretter JT
Semin Cardiothorac Vasc Anesth 2018 Sep;22(3):245-255. Epub 2018 Feb 7 doi: 10.1177/1089253218756757. PMID: 29411679
Hybrid approach for pulmonary atresia with intact ventricular septum: early single center results and comparison to the standard surgical approach.
Zampi JD, Hirsch-Romano JC, Goldstein BH, Shaya JA, Armstrong AK
Catheter Cardiovasc Interv 2014 Apr 1;83(5):753-61. Epub 2014 Jan 8 doi: 10.1002/ccd.25181. PMID: 23996932
Evolution of the management approach for pulmonary atresia with intact ventricular septum.
Mi YP, Chau AK, Chiu CS, Yung TC, Lun KS, Cheung YF
Heart 2005 May;91(5):657-63. doi: 10.1136/hrt.2004.033720. PMID: 15831657Free PMC Article
Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.
Giglia TM, Mandell VS, Connor AR, Mayer JE Jr, Lock JE
Circulation 1992 Nov;86(5):1516-28. doi: 10.1161/01.cir.86.5.1516. PMID: 1423965

Therapy

Fetal cardiac interventions: Where do we stand?
Friedman KG, Tworetzky W
Arch Cardiovasc Dis 2020 Feb;113(2):121-128. Epub 2020 Feb 26 doi: 10.1016/j.acvd.2019.06.007. PMID: 32113817
Pacemaker treatment after Fontan surgery-A Swedish national study.
Alenius Dahlqvist J, Sunnegårdh J, Hanséus K, Strömvall Larsson E, Nygren A, Dalén M, Berggren H, Johansson Ramgren J, Wiklund U, Rydberg A
Congenit Heart Dis 2019 Jul;14(4):582-589. Epub 2019 Mar 18 doi: 10.1111/chd.12766. PMID: 30883035
Hybrid intraoperative pulmonary artery stenting in redo congenital cardiac surgeries.
Sridhar A, Subramanyan R, Premsekar R, Chidambaram S, Agarwal R, Manohar SR, Cherian KM
Indian Heart J 2014 Jan-Feb;66(1):45-51. Epub 2013 Dec 26 doi: 10.1016/j.ihj.2013.12.018. PMID: 24581095Free PMC Article
Hybrid approach for pulmonary atresia with intact ventricular septum: early single center results and comparison to the standard surgical approach.
Zampi JD, Hirsch-Romano JC, Goldstein BH, Shaya JA, Armstrong AK
Catheter Cardiovasc Interv 2014 Apr 1;83(5):753-61. Epub 2014 Jan 8 doi: 10.1002/ccd.25181. PMID: 23996932
Outcomes and predictors of reintervention in patients with pulmonary atresia and intact ventricular septum treated with radiofrequency perforation and balloon pulmonary valvuloplasty.
Schwartz MC, Glatz AC, Dori Y, Rome JJ, Gillespie MJ
Pediatr Cardiol 2014 Jan;35(1):22-9. Epub 2013 Jun 19 doi: 10.1007/s00246-013-0733-1. PMID: 23780553

Prognosis

Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.
Shirozu H, Kodama Y, Kuraoka A, Ishikawa Y, Nakano T, Kado H, Sagawa K
Pediatr Cardiol 2022 Oct;43(7):1653-1658. Epub 2022 May 13 doi: 10.1007/s00246-022-02898-w. PMID: 35562506
Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum.
Liu L, Wang H, Cui C, Li Y, Liu Y, Wang Y, Fan T, Peng B
Medicine (Baltimore) 2019 Oct;98(42):e17492. doi: 10.1097/MD.0000000000017492. PMID: 31626103Free PMC Article
Pacemaker treatment after Fontan surgery-A Swedish national study.
Alenius Dahlqvist J, Sunnegårdh J, Hanséus K, Strömvall Larsson E, Nygren A, Dalén M, Berggren H, Johansson Ramgren J, Wiklund U, Rydberg A
Congenit Heart Dis 2019 Jul;14(4):582-589. Epub 2019 Mar 18 doi: 10.1111/chd.12766. PMID: 30883035
Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.
Chikkabyrappa SM, Loomba RS, Tretter JT
Semin Cardiothorac Vasc Anesth 2018 Sep;22(3):245-255. Epub 2018 Feb 7 doi: 10.1177/1089253218756757. PMID: 29411679
Evolution of the management approach for pulmonary atresia with intact ventricular septum.
Mi YP, Chau AK, Chiu CS, Yung TC, Lun KS, Cheung YF
Heart 2005 May;91(5):657-63. doi: 10.1136/hrt.2004.033720. PMID: 15831657Free PMC Article

Clinical prediction guides

Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.
Shirozu H, Kodama Y, Kuraoka A, Ishikawa Y, Nakano T, Kado H, Sagawa K
Pediatr Cardiol 2022 Oct;43(7):1653-1658. Epub 2022 May 13 doi: 10.1007/s00246-022-02898-w. PMID: 35562506
Pulmonary atresia with intact ventricular septum: Intended strategies.
Sukhavasi A, McHugh-Grant S, Glatz AC, Mondal A, Griffis H, Burnham N, Chen JM, Mascio CE, Gaynor JW, Spray TL, Fuller SM
J Thorac Cardiovasc Surg 2022 Nov;164(5):1277-1288. Epub 2022 Mar 16 doi: 10.1016/j.jtcvs.2021.11.104. PMID: 35414413
Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum.
Liu L, Wang H, Cui C, Li Y, Liu Y, Wang Y, Fan T, Peng B
Medicine (Baltimore) 2019 Oct;98(42):e17492. doi: 10.1097/MD.0000000000017492. PMID: 31626103Free PMC Article
Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.
Chikkabyrappa SM, Loomba RS, Tretter JT
Semin Cardiothorac Vasc Anesth 2018 Sep;22(3):245-255. Epub 2018 Feb 7 doi: 10.1177/1089253218756757. PMID: 29411679
Outcomes and predictors of reintervention in patients with pulmonary atresia and intact ventricular septum treated with radiofrequency perforation and balloon pulmonary valvuloplasty.
Schwartz MC, Glatz AC, Dori Y, Rome JJ, Gillespie MJ
Pediatr Cardiol 2014 Jan;35(1):22-9. Epub 2013 Jun 19 doi: 10.1007/s00246-013-0733-1. PMID: 23780553

Recent systematic reviews

Prediction of postnatal circulation in pulmonary atresia/critical stenosis with intact ventricular septum: systematic review and external validation of models.
Villalaín C, Moon-Grady AJ, Herberg U, Strainic J, Cohen JL, Shah A, Levi DS, Gómez-Montes E, Herraiz I, Galindo A
Ultrasound Obstet Gynecol 2023 Jul;62(1):14-22. Epub 2023 Jun 5 doi: 10.1002/uog.26176. PMID: 36776132
Fetal Echocardiographic Predictors of Postnatal Surgical Strategies in Critical Pulmonary Stenosis or Atresia with Intact Ventricular Septum: A Meta-Analysis.
Shivaram P, Van den Eynde J, Barnes BT, Danford DA, Cedars A, Kutty S
Fetal Diagn Ther 2022;49(5-6):225-234. Epub 2022 Jul 6 doi: 10.1159/000525718. PMID: 35793649
Patent Ductus Arteriosus Stent Versus Surgical Aortopulmonary Shunt for Initial Palliation of Cyanotic Congenital Heart Disease with Ductal-Dependent Pulmonary Blood Flow: A Systematic Review and Meta-Analysis.
Tseng SY, Truong VT, Peck D, Kandi S, Brayer S, Jason DP 3rd, Mazur W, Hill GD, Ashfaq A, Goldstein BH, Alsaied T
J Am Heart Assoc 2022 Jul 5;11(13):e024721. Epub 2022 Jun 29 doi: 10.1161/JAHA.121.024721. PMID: 35766251Free PMC Article
Biventricular repair versus uni-ventricular repair for pulmonary atresia with intact ventrical septum: A systematic review.
Li FF, Du XL, Chen S
J Huazhong Univ Sci Technolog Med Sci 2015 Oct;35(5):656-661. Epub 2015 Oct 22 doi: 10.1007/s11596-015-1485-3. PMID: 26489617
Hybrid approach for pulmonary atresia with intact ventricular septum: early single center results and comparison to the standard surgical approach.
Zampi JD, Hirsch-Romano JC, Goldstein BH, Shaya JA, Armstrong AK
Catheter Cardiovasc Interv 2014 Apr 1;83(5):753-61. Epub 2014 Jan 8 doi: 10.1002/ccd.25181. PMID: 23996932

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