eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiothoracic Surgery

Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment: Follow-up

Author: Jayme Scott Bennetts, MD, Fellow, Department of Cardiac and Thoracic Surgery, Flinders Medical Centre
Coauthor(s): Christopher A Caldarone, MD, Associate Professor, Department of Surgery, The Hospital for Sick Children, University of Toronto
Contributor Information and Disclosures

Updated: May 1, 2009

Outcome and Prognosis

Early total anomalous pulmonary venous drainage (TAPVD)-associated hospital mortality ranges from 2-20%. Risk factors include poor preoperative status (eg, acidosis), obstruction, high pulmonary vascular resistance, young age, small left ventricle, and single-ventricle anatomy.

Long-term prognosis after successful repair of TAPVD is favorable. Approximately 10-15% of patients have evidence of late pulmonary-vein obstruction. For this reason, long-term surveillance is important. Postoperative stenosis tends to be recurrent and progressive.

Long-term prognosis for patients after repair of partial anomalous venous drainage (PAPVD) is excellent in the absence of irreversible pulmonary hypertension. In children, closure of an associated atrial septal defect (ASD) almost eliminates the risk of the late development of atrial arrhythmias. However, in adults, closure of an ASD is associated with persistent risk of late atrial arrhythmias.

Future and Controversies

Improved neonatal care and surgical techniques have vastly improved surgical survival rates in neonates requiring repair of total anomalous pulmonary venous drainage (TAPVD). However, in subsets of patients, the risk of surgical death is extremely high. In particular, patients with single ventricles and TAPVD requiring repair in the neonatal period have a high rate of surgical mortality largely because postoperative management of hemodynamics is difficult.

A second frontier in the treatment of TAPVD is in patients with postsurgical obstruction of a pulmonary vein. Improved understanding of the vascular biology responsible for diffuse pulmonary vein stenosis in this group will improve therapy. At present, surgical therapy with sutureless techniques and/or transplantation may offer the most potential.

Current changes in surgical techniques, with increasing acceptance and expanded indications for the sutureless type anastomosis, may further improve the outcomes of patients, especially those with postoperative and recurrent stenosis.

 


More on Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment

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Workup: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
Treatment: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
Follow-up: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
Multimedia: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
References
Further Reading
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References

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Keywords

total anomalous pulmonary venous connection, TAPVC, partial anomalous pulmonary venous connection, PAPVC, total anomalous pulmonary venous drainage, TAPVD, partial anomalous pulmonary venous drainage, PAPVD, total anomalous pulmonary venous return, TAPVR, partial anomalous pulmonary venous return, PAPVR, scimitar syndrome, anomalous pulmonary venous drainage, sinus venosus atrial septal defect, cardiac defect, heart defect, mixed pulmonary venous drainage, pulmonary venous obstruction, cardiac surgery, atrial septal defect, ASD, right lung hypoplasia, scimitar syndrome, pulmonary hypertension, Eisenmenger syndrome, cyanosis, treatment, diagnosis

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Contributor Information and Disclosures

Author

Jayme Scott Bennetts, MD, Fellow, Department of Cardiac and Thoracic Surgery, Flinders Medical Centre
Jayme Scott Bennetts, MD is a member of the following medical societies: Royal Australasian College of Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Christopher A Caldarone, MD, Associate Professor, Department of Surgery, The Hospital for Sick Children, University of Toronto
Christopher A Caldarone, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Jonah Odim, MD, PhD, MBA, Senior Medical Officer, Transplantation Immunology Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases, National Institutes of Health
Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physician Executives, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, Canadian Cardiovascular Society, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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