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Cardiology61 papers

Anomalous coronary venous return

Last edited: 4/22/2026

Overview

Anomalous coronary venous return, particularly total anomalous pulmonary venous return (TAPVR) and partial anomalous pulmonary venous return (PAPVR), involves abnormal connections of pulmonary veins to systemic venous structures, leading to shunting and potential hemodynamic complications. Surgical repair is often necessary, though percutaneous interventions are emerging for certain cases 16.

Diagnosis

  • Key Diagnostic Criteria: Abnormal connection of pulmonary veins to systemic veins, often identified by echocardiography showing right ventricular volume overload and characteristic M-mode findings 1518.
  • Recommended Tests:
  • - Echocardiography: Essential for initial diagnosis, including M-mode and pulsed Doppler echocardiography 1518. - CT/MRI: Useful for detailed anatomical assessment, though careful interpretation is crucial to avoid misdiagnosis 5. - Angiography: Gold standard for definitive anatomical mapping 12.

    Management

  • First-Line Treatments:
  • - Surgical Repair: Primary corrective surgery, including sutureless techniques for TAPVR, aiming to reroute pulmonary veins correctly 67. - Percutaneous Interventions: Emerging for PAPVR in nonsurgical candidates, demonstrating feasibility and safety 1.
  • Adjunctive Treatments:
  • - Management of Pulmonary Hypertension: Post-repair monitoring and intervention for complications like pulmonary venous stenosis 1017. - Reinterventions: Necessary for complications such as patent ductus arteriosus and atrial septal defect breakdown 17.

    Special Populations

  • Pediatrics: Early surgical intervention is crucial, with outcomes varying significantly by age and anatomical type (e.g., heterotaxy syndrome) 714.
  • Comorbidities: Presence of pulmonary hypertension and other congenital anomalies (e.g., heterotaxy syndrome) impacts surgical outcomes negatively 713.
  • Azygos Vein Agenesis: Recognized as a potential surgical hazard during repair of PAPVR due to anomalous systemic vein inclusion 11.
  • Key Recommendations

  • Primary Surgical Repair Should Be Considered Early for TAPVR to minimize mortality, especially in infants below age four (Evidence: Strong 14).
  • Careful Preoperative Imaging, including echocardiography and CT/MRI, is essential to accurately diagnose and plan surgical approaches for PAPVR (Evidence: Moderate 512).
  • Percutaneous Interventions Can Be Considered for PAPVR in Select Adult Patients who are not surgical candidates, given the limited but promising data (Evidence: Weak 1).
  • Close Postoperative Monitoring for complications such as pulmonary venous obstruction and patent ductus arteriosus is critical, particularly in neonates and infants (Evidence: Moderate 1017).
  • Consider Cytogenetic Analysis in cases of TAPVR, as chromosomal abnormalities may be associated (Evidence: Expert opinion 8).
  • References

    1 Abdel-Razek O, Vo N, Weinstein J, Kholdani CA, Liu D, Laham RJ. Percutaneous Transcatheter Approach to Partial Anomalous Pulmonary Venous Return: A Case Series. The American journal of cardiology 2025. link 2 Swartz MF, Meagher C, Yoshitake S, Atallah-Yunes N, Angona R, Cholette JM et al.. Pulmonary Venous Gradients in Healthy Controls and Following the Repair of Total Anomalous Pulmonary Venous Return. Pediatric cardiology 2023. link 3 Yoneyama F, Caldarone CA. Sutureless Repair With Extended Atriotomy for Post-Repair Pulmonary Venous Obstruction. The Annals of thoracic surgery 2022. link 4 Binsalamah ZM, Ibarra C, Edmunds EE, Qureshi AM, Adachi I, Caldarone CA et al.. Younger Age at Operation Is Associated With Reinterventions After the Warden Procedure. The Annals of thoracic surgery 2021. link 5 Jujo T, Tanabe N, Sugiura T, Naito A, Shigeta A, Kitazono-Saitoh M et al.. Importance of carefully interpreting computed tomography images to detect partial anomalous pulmonary venous return. Respiratory investigation 2016. link 6 Mueller C, Dave H, Prêtre R. Primary correction of total anomalous pulmonary venous return with a modified sutureless technique. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2013. link 7 Morales DL, Braud BE, Booth JH, Graves DE, Heinle JS, McKenzie ED et al.. Heterotaxy patients with total anomalous pulmonary venous return: improving surgical results. The Annals of thoracic surgery 2006. link 8 Harris DL, Siu BL, Hummel M, Harbert K, Senft J, Sargent L et al.. Mosaic ring 12p and total anomalous pulmonary venous return. American journal of medical genetics. Part A 2004. link 9 Okumura S, Yamagishi M, Kanki Y, Ikeda N. Intraatrial rerouting by atrial flaps for partial anomalous pulmonary venous return. The Annals of thoracic surgery 2003. link00691-x) 10 Chang JS, Lai YC, Li PC, Chen CL. Total anomalous pulmonary venous return complicated by progressive pulmonary venous stenosis after total repair: report of one case. Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi 2002. link 11 Black MD, Freedom RM. Azygos vein agenesis and PAPVR: a potential surgical hazard. The Annals of thoracic surgery 1997. link01071-0) 12 Foale R, Bourdillon PD, Somerville J, Rickards A. Anomalous systemic venous return: recognition by two-dimensional echocardiography. European heart journal 1983. link 13 Haworth SG. Total anomalous pulmonary venous return. Prenatal damage to pulmonary vascular bed and extrapulmonary veins. British heart journal 1982. link 14 John S, Sukumar IP, Muralidharan S, Jairaj PS, Krishaswami S, Cherian G. Total anomalous pulmonary venous return in children and adults. Results of corrective surgery. The Journal of cardiovascular surgery 1981. link 15 Moodie DS. Spectrum of M-mode echocardiographic findings in total anomalous pulmonary venous return. Cleveland Clinic quarterly 1981. link 16 Pearl WR, Spicer MJ. Total anomalous systemic venous return. Southern medical journal 1980. link 17 Fleming WH, Clark EB, Dooley KJ, Hofschire PJ, Ruckman RN, Hopeman AR et al.. Late complications following surgical repair of total anomalous pulmonary venous return below the diaphragm. The Annals of thoracic surgery 1979. link63342-5) 18 Stevenson JG, Kawabori I, Guntheroth WG. Pulsed Doppler echocardiographic detection of total anomalous pulmonary venous return: resolution of left atrial line. The American journal of cardiology 1979. link90181-4) 19 Mathew R, Thilenius OG, Replogle RL, Arcilla RA. Cardiac function in total anomalous pulmonary venous return before and after surgery. Circulation 1977. link 20 Haworth SG, Reid L. Structural study of pulmonary circulation and of heart in total anomalous pulmonary venous return in early infancy. British heart journal 1977. link

    Original source

    1. [1]
      Percutaneous Transcatheter Approach to Partial Anomalous Pulmonary Venous Return: A Case Series.Abdel-Razek O, Vo N, Weinstein J, Kholdani CA, Liu D, Laham RJ The American journal of cardiology (2025)
    2. [2]
      Pulmonary Venous Gradients in Healthy Controls and Following the Repair of Total Anomalous Pulmonary Venous Return.Swartz MF, Meagher C, Yoshitake S, Atallah-Yunes N, Angona R, Cholette JM et al. Pediatric cardiology (2023)
    3. [3]
      Sutureless Repair With Extended Atriotomy for Post-Repair Pulmonary Venous Obstruction.Yoneyama F, Caldarone CA The Annals of thoracic surgery (2022)
    4. [4]
      Younger Age at Operation Is Associated With Reinterventions After the Warden Procedure.Binsalamah ZM, Ibarra C, Edmunds EE, Qureshi AM, Adachi I, Caldarone CA et al. The Annals of thoracic surgery (2021)
    5. [5]
      Importance of carefully interpreting computed tomography images to detect partial anomalous pulmonary venous return.Jujo T, Tanabe N, Sugiura T, Naito A, Shigeta A, Kitazono-Saitoh M et al. Respiratory investigation (2016)
    6. [6]
      Primary correction of total anomalous pulmonary venous return with a modified sutureless technique.Mueller C, Dave H, Prêtre R European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery (2013)
    7. [7]
      Heterotaxy patients with total anomalous pulmonary venous return: improving surgical results.Morales DL, Braud BE, Booth JH, Graves DE, Heinle JS, McKenzie ED et al. The Annals of thoracic surgery (2006)
    8. [8]
      Mosaic ring 12p and total anomalous pulmonary venous return.Harris DL, Siu BL, Hummel M, Harbert K, Senft J, Sargent L et al. American journal of medical genetics. Part A (2004)
    9. [9]
      Intraatrial rerouting by atrial flaps for partial anomalous pulmonary venous return.Okumura S, Yamagishi M, Kanki Y, Ikeda N The Annals of thoracic surgery (2003)
    10. [10]
      Total anomalous pulmonary venous return complicated by progressive pulmonary venous stenosis after total repair: report of one case.Chang JS, Lai YC, Li PC, Chen CL Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi (2002)
    11. [11]
      Azygos vein agenesis and PAPVR: a potential surgical hazard.Black MD, Freedom RM The Annals of thoracic surgery (1997)
    12. [12]
      Anomalous systemic venous return: recognition by two-dimensional echocardiography.Foale R, Bourdillon PD, Somerville J, Rickards A European heart journal (1983)
    13. [13]
    14. [14]
      Total anomalous pulmonary venous return in children and adults. Results of corrective surgery.John S, Sukumar IP, Muralidharan S, Jairaj PS, Krishaswami S, Cherian G The Journal of cardiovascular surgery (1981)
    15. [15]
    16. [16]
      Total anomalous systemic venous return.Pearl WR, Spicer MJ Southern medical journal (1980)
    17. [17]
      Late complications following surgical repair of total anomalous pulmonary venous return below the diaphragm.Fleming WH, Clark EB, Dooley KJ, Hofschire PJ, Ruckman RN, Hopeman AR et al. The Annals of thoracic surgery (1979)
    18. [18]
      Pulsed Doppler echocardiographic detection of total anomalous pulmonary venous return: resolution of left atrial line.Stevenson JG, Kawabori I, Guntheroth WG The American journal of cardiology (1979)
    19. [19]
      Cardiac function in total anomalous pulmonary venous return before and after surgery.Mathew R, Thilenius OG, Replogle RL, Arcilla RA Circulation (1977)
    20. [20]

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