Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment Workup

  • Author: Nicola Viola, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Jul 21, 2010
 

Laboratory Studies

ABG values, including pO2, pCO2, pH, base excess, lactate concentration, and mixed venous oxygen saturations permit quantitative assessment of the patient's oxygenation and systemic perfusion. Acute ABG evaluation assists in the resuscitation of a neonate with obstructed total anomalous pulmonary venous connection (TAPVC). Severe metabolic acidosis and hypoxemia are often seen.

Hematocrit levels are checked to confirm adequate oxygen-carrying capacity.

BUN and/or creatinine levels are useful in critically ill neonates presenting with obstructed pulmonary venous return.

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Imaging Studies

Chest radiography

In partial anomalous pulmonary venous connection (PAPVC), lung fields often demonstrate increased pulmonary vascular markings. In addition, an enlarged right-heart border from the volume loaded right heart is seen. In patients with scimitar syndrome, a diagnostic vertically-directed crescent shadow is observed to the right of the mediastinal silhouette.

In TAPVC, obstruction to pulmonary venous drainage determines the appearance of the lung fields on chest radiography. In patients without obstruction, the pulmonary vascular bed is plethoric and pulmonary artery is prominent. In patients with obstruction, severe pulmonary edema is the commonest finding. A prominence of the pulmonary artery shadow and the right atrial (RA) silhouette are often observed. In supracardiac drainage, the prominence of the upper mediastinum can create the classic snowman or figure-8 appearance.

Echocardiography

In PAPVC, echocardiography is typically used to help delineate the anatomy of the pulmonary venous drainage and the atrial septum. Confirmation of the normal drainage of the remaining pulmonary veins is an important part of the echocardiographic examination.[14, 15]

In TAPVC, with 2-dimensional echocardiography and color-flow Doppler mapping, the anomalous venous anatomy is usually well defined. Diagnostic findings include distension of the right ventricle, the presence of a vascular confluence coupled with absent venous drainage to the left atrium on the Doppler interrogation, the presence of an accessory common vein, a dilated coronary sinus and turbulent flow in the right atrium with a right-to-left shunt. Demonstration of turbulence or flow acceleration in the pulmonary veins is also used to diagnose obstruction in the pulmonary venous circuit. In addition, right-heart pressures and other cardiac anomalies can be determined. Echocardiography has shown excellent sensitivity and specificity in fetal diagnosis[15] and as a prognostic tool and has supplanted angiography in the vast majority of cases.[16]

CT and MRI

In nonurgent cases of PAPVC or TAPVC, CT or MRI may be used to further delineate the cardiac anatomy. The ability to form 3-dimensional reconstructions with these imaging modalities is evolving rapidly.

In particular, contrast-enhanced magnetic resonance angiography (MRA) provides complete anatomical and functional assessment of the pulmonary circulation. When compared to echocardiography, MRIA has been shown to offer a better visualization of the whole length of the pulmonary veins, the communicating veins, and the accessory pathways. Additional benefits include the possibility of qualitatively and quantitatively evaluating the amount of anomalous pulmonary blood flow, the Qp:Qs (using phase-contrast MRI). The secondary effects of the shunt on pulmonary arteries as well as cardiac chambers are also detectable in the same study.[17, 18]

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Diagnostic Procedures

Cardiac catheterization is used infrequently for diagnosis in routine TAPVC or PAPVC because of the refinements in echocardiography. Cardiac catheterization is helpful in patients in whom echocardiographic findings are ambiguous or in patients with other complex defects. As a result of the mixing of oxygenated pulmonary venous effluent and deoxygenated systemic venous blood (oxygen saturations) are almost identical in all chambers of the heart in patients with TAPVC.

The site of the anomalous connection is located precisely with angiography by locating the step-up in saturations along the systemic venous pathway. Catheterization is also helpful in defining the anatomy of pulmonary-vein stenosis, which may develop after TAPVC is repaired.

In older patients with PAPVC, cardiac catheterization may be required to exclude coronary artery disease, to assess right-heart pressures, to ascertain the reversibility of any pulmonary arterial hypertension, and to calculate the shunt fraction.

Balloon atrial septostomy (BAS) may assist in evaluating the hemodynamic status of patient by unmasking previously undetected severe venous obstruction. The persistence of pulmonary hypertension following BAS should raise the suspicion of an obstruction to the venous drainage in an extracardiac location.

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Histologic Findings

TAPVC is associated with hypertrophy of the media of the pulmonary veins and arteries. This finding is most prominent in patients with evidence of pulmonary venous obstruction, and it is most important in the extrapulmonary and intrapulmonary veins. Intimal proliferation and fibrous thickening of the pulmonary veins, with lymphangiectasia, is a common microscopic finding in patients with TAPVC.

In patients with recurrent stenosis a diffuse fibrous proliferation of the intima is often seen, usually at the site of the surgical anastomosis, although these changes can be seen along the whole length of the vein. Occasionally, those nonspecific changes can involve the intraparenchymal portions of the venous bed, mimicking the pathological changes of the veno-occlusive disease.

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Contributor Information and Disclosures
Author

Nicola Viola, MD  Congenital Surgeon, Department of Cardiothoracic Surgery, Southampton University Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher A Caldarone, MD  Chair, Division of Cardiac Surgery, Professor of Surgery, University of Toronto; Staff Surgeon, Cardiovascular Surgery, Hospital for Sick Children, 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.

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.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD  Professor and Chief, Cardiothoracic Surgery, Department of Surgery, Louisiana State University Health Sciences Center-Shreveport

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.

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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Types of total anomalous pulmonary venous connection.
Partial anomalous pulmonary venous connection (PAPVC) of right-sided pulmonary veins to the right superior venoatrial junction. Note the enlarged confluence and superior vena cava. SVC = Superior vena cava; IVC = Inferior vena cava; RUPV = Right upper pulmonary vein; RMPV = Right middle pulmonary vein.
(A): Patient with obstructed scimitar syndrome (magnetic resonance angiography). The right upper pulmonary vein (RUPV) is diffusely narrow and presents a discrete stenosis at the confluence with the right lower pulmonary vein (red arrow). (B): The confluence at the atrial level appears unobstructed (white arrow) in the 3D reconstruction.
(A): Total anomalous pulmonary venous connection (TAPVC) of supracardiac type. All pulmonary veins drain into a long confluence and a short communicating vein is attached to the right superior vena cava (SVC). The left lower pulmonary vein appears stenosed (red arrow). (B): These findings are confirmed by the 3D reconstruction (white arrow).
Follow-up CT scan performed after repair of total anomalous pulmonary venous connection (TAPVC). The right upper pulmonary vein (RUPV) appears severely stenosed (red arrow).
 
 
 
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