Pulmonary Atresia With Ventricular Septal Defect Treatment & Management

  • Author: Edwin Rodriguez-Cruz, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Apr 17, 2012
 

Medical Therapy

In patients with pulmonary atresia with ventricular septal defect (PA-VSD) a ductal-dependent circulation, prostaglandin E2 is often required to keep the ductus arteriosus open in the early neonatal period until surgery can be performed. A neonate who is ill may require fluid and acidosis management, but mechanical ventilation is rarely needed.

Medical treatment with digitalis, diuretics, and other agents may be indicated in patients with congestive heart failure (CHF) resulting from increased pulmonary blood flow. Phlebotomy to relieve the adverse effects of extreme polycythemia in very hypoxic patients is rarely performed. In patients with CHF and increased work of breathing, a high-energy diet is required. Rarely, a patient may require placement of a nasogastric tube to achieve the goals of energy intake.

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Surgical Therapy

Various options are available, depending on the anatomy of the individual patient.

Palliative surgery

If the atresia is limited to the pulmonary valve (eg, imperforate pulmonary valve, membranous pulmonary atresia), the valve can be perforated percutaneously using special devices designed for this specific purpose, such as a needle or a radiofrequency ablation catheter. Then, after the perforation is done, the valve is dilated with a balloon catheter. Stents can be placed in stenosed aortopulmonary collateral arteries in patients with hypoplastic pulmonary arteries.

Palliative extracardiac systemic-to-pulmonary shunts can be placed to promote growth of pulmonary arteries. Direct aortopulmonary shunts (eg, Waterston shunt, Pott shunt) were used in the past but, subsequently, were found to create severe distortion, scarring, interruption of the pulmonary arteries, and, on occasion, pulmonary hypertension. Thus, the use of these shunts has fallen into disfavor. Currently, the modified Blalock-Taussig shunt is used most commonly and is connected from the subclavian or innominate artery to the pulmonary artery (when anatomy permits). In recent years, a direct right ventricle to pulmonary arteries shunt has been placed with good results.

Valveless conduits or homografts may be used to connect the right ventricle (RV) to the pulmonary artery. This may promote the growth of pulmonary arteries.

In infants with CHF caused by excessive aortopulmonary collateral arteries, flow can be reduced by performing surgical interruption or by judicious banding or percutaneous coil occlusion of selected systemic arterial collaterals.

Complete surgical repair

The objective of complete repair is to create an unrestricted continuity between the RV outflow tract and the pulmonary arterial tree using nonvalved or valved conduits. Subsequently, all extracardiac sources of pulmonary blood flow need to be ligated. The atrial septal defects (ASDs) and ventricular septal defects need to be closed. An important goal is to achieve a satisfactory ratio between the peak systolic pressures in the RV and the left ventricle (LV).

Various approaches have been devised to achieve a complete surgical repair, including the following:

  • If a patient meets all the criteria for complete repair, single-stage unifocalization of pulmonary blood supply and complete intracardiac repair is the procedure of choice.
  • Single-stage unifocalization and postponement of ventricular septal defect closure to a second operation is an option.
  • Sequential unilateral unifocalization followed by intracardiac repair is preferred in some patients.

Heart catheterization

More recently, new techniques and devices are being used to treat stenoses and insufficiency of the right ventricle–to–pulmonary artery conduits and/or homografts in these patients. After complete surgical repair of the condition, patients require close follow-up and, on occasion, angioplasties and stent placement to overcome stenotic segments. In current practice, valved stents can be inserted during a heart catheterization, without the need to open the chest, in the presence of severe pulmonary valve insufficiency or stenosis. These percutaneous valves can be implanted using fluoroscopic guidance.

Heart-lung transplantation

In patients with completely atretic main, left, and right pulmonary arteries, heart-lung transplantation is a viable option.

Complications of surgery include the following:

  • In the unifocalization procedure, severe airflow limitation occurs after the unifocalization surgery because of tracheobronchial epithelial ischemia resulting from interruption of the tracheobronchial blood supply. This complication significantly contributes to early postoperative morbidity and mortality rates.
  • Aortic regurgitation or aortic root dilation may occur.
  • Restenoses of the shunts and neopulmonary arteries may occur, and subsequent interventions may be required.
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Preoperative Details

The use of blood is a consideration in any patient who is to undergo a major surgical procedure. Thus, evaluation for the concomitant presence of other medical problems such as Di George syndrome must be performed.[3] Di George syndrome may be present in patients with conotruncal abnormalities such as pulmonary atresia with ventricular septal defect. When blood is used in these patients, it should be previously irradiated to avoid problems during transfusion.

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Follow-up

Careful monitoring for drug dosing and adverse effects is necessary. Monitor patients for adequacy of repair and postoperative complications. Perform echocardiography on a regular basis, paying special attention to surgically created shunts, residual shunts, and the flow through RV outflow tract conduits.

For patient education resources, see the Heart Center, as well as Tetralogy of Fallot and Ventricular Septal Defect.

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Complications

Possible complications of pulmonary atresia with ventricular septal defect (PA-VSD) include the following:

  • Congestive heart failure (CHF)
  • Erythrocytosis
  • Infective endocarditis
  • Brain abscess
  • Delayed growth and puberty
  • Arrhythmias and sudden death
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Outcome and Prognosis

Patients may require repeated surgeries for a complete repair of pulmonary atresia with ventricular septal defect (PA-VSD). Educate family members regarding congenital heart disease and how to perform cardiopulmonary resuscitation (CPR). Genetic counseling for future pregnancies is necessary.

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Contributor Information and Disclosures
Author

Edwin Rodriguez-Cruz, MD  Assistant Professor, Department of Pediatrics, San Juan Bautista Medical School and Medical Center; Consulting Interventional/Clinical Pediatric Cardiologist, Department of Pediatrics, Hospital El Maestro and San Juan Bautista Medical Center; Consulting Interventional/Clinical Pediatric Cardiologist, Department of Cardiology, Cardiovascular Center of Puerto Rico and the Caribbean and Veterans Affairs Hospital and Medical Center of Puerto Rico

Edwin Rodriguez-Cruz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Heart Association, American Medical Association, American Society of Echocardiography, Puerto Rico Medical Association, Society of Cardiac Angiography and Interventions, and Society of Pediatric Echocardiography

Disclosure: NOVARTIS Grant/research funds INVESTIGATOR

Coauthor(s)

Sanjeev Aggarwal, MD, MBBS  Staff Physician, Department of Pediatrics, Children's Hospital of Michigan

Sanjeev Aggarwal, MD, MBBS is a member of the following medical societies: American Academy of Pediatrics and American Medical Association

Disclosure: Nothing to disclose.

Ralph E Delius, MD  Associate Professor, Department of Surgery, Wayne State University

Ralph E Delius, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Thoracic Surgery, American College of Chest Physicians, American College of Surgeons, American Heart Association, American Medical Association, and Society of Thoracic 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; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD  Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in 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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Left ventricular angiography (right anterior oblique caudal view) in a patient with pulmonary atresia with ventricular septal defect (PA-VSD). The catheter has been advanced from the inferior vena cava to the right atrium, across the atrial septal defect to the left atrium, and then to the left ventricle. The left ventricle fills with contrast and has good systolic function. Left-to-right shunting of contrast is present across a ventricular septal defect; however, no blood is flowing out the right ventricle. A blind pouch is observed in the area of the right ventricular outflow tract. All of the contrast medium (flow) exits the heart via the aorta. The pulmonary circulation is supplied by collateral vessels arising from the descending aorta. See Media files 2-3 for still frames.
Left anterior oblique ventriculogram in a patient (same patient as Media files 1-3) with pulmonary atresia with ventricular septal defect (PA-VSD). The angiogram shows the left and right ventricles with a large malalignment ventricular septal defect between them. The only outflow from the heart is the aorta. No evidence of pulmonary blood flow is observed arising from the ventricles directly to the lungs. LV=left ventricle; RV=right ventricle; Asc Ao=ascending aorta; Desc Ao=descending aorta.
Anteroposterior view of an aortogram in a patient (same patient as Media files 1-2) with pulmonary atresia with ventricular septal defect (PA-VSD). The pulmonary circulation is supplied by collateral vessels (Collaterals) that arise from the descending aorta. Desc Ao=descending aorta.
Short-axis parasternal view in a patient with pulmonary atresia with ventricular septal defect (PA-VSD). Note the trileaflet aortic valve in the center of the picture. The tricuspid valve is on the left (9- to 10-o'clock position). The normal pulmonary valve position is on the right (2- to 3-o'clock position). This echocardiogram demonstrates that the pulmonary valve is atretic. See Media file 5 for a still frame.
Short-axis parasternal view (1) and diagram (3) in a patient with pulmonary atresia and ventricular septal defect (PA-VSD). Short-axis parasternal view (2) and diagram (4) in a patient with normal anatomy. RA=right atrium; LA=left atrium; RV=right ventricle; PA=pulmonary artery; TR=tricuspid valve; PV=pulmonary valve.
 
 
 
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