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Pulmonary Atresia With Ventricular Septal Defect Treatment & Management

  • Author: Edwin Rodriguez-Cruz, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Apr 21, 2015
 

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

There is extreme variation of the anatomy, which will require individualized approach for each patient.

Staged repair

Management algorithm for patients with pulmonary a Management algorithm for patients with pulmonary atresia with ventricular septal defect (PA-VSD) and major aortopulmonary collateral arteries (MAPCAs), based on the nature of pulmonary vascular supply. PAs = pulmomary arteries. Courtesy of Elsevier (Gupta A, Odim J, Levi D, Chang RK, Laks H. Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Experience with 104 patients. J Thorac Cardiovasc Surg. 2003;126(6):1746–52).

The aims of the staged approach (see the image above) in a patient with pulmonary atresia with ventricular septal defect (VSD) are to increase the flow of the native pulmonary arteries by establishing a direct continuity between the aorta or the right ventricle (RV) and the small pulmonary artery (PA), thereby stimulating its growth. This is followed by unifocalization of the collaterals in both lungs and, finally, closing the VSD and establishing RV-PA continuity.

The main advantage of staged palliation is that it breaks the entire repair into smaller and better tolerated procedures.

Increasing pulmonary blood flow can be accomplished either by performing a shunt between the aorta or one of its branches and one of the PAs, or by using a nonvalved conduit between the RV and main PA if it was of adequate size.

Direct aortopulmonary shunts (eg, Waterston shunt, Pott shunt) were used in the past, but these were subsequently found to create severe distortion, scarring, interruption of the PAs, and, on occasion, pulmonary hypertension. Thus, the use of these shunts has fallen into disfavor.

Currently, the modified Blalock-Taussig shunt is most commonly used, and it is connected from the subclavian or innominate artery to the PA (when anatomy permits). A central ascending aorta to the main PA shunt (Melbourne shunt) can also be considered in the presence of confluent hypoplastic main PAs.

The second stage involves ligation and transplantation of MAPCAs. Ligation is performed when there is a dual blood supply to the same segment of the lung from a native pulmonary artery as well as from the major aortopulmonary collateral arteries (MAPCAs). MAPCAs need to be transplanted when they are the only source of blood supply to a bronchopulmonary segment, there is no stenosis, and they are not hypertensive.[7]

Diagnostic angiography must be performed to establish the size and distribution of the MAPCAs. Using this road map, the surgeon will then establish anastomotic communications between these vessels and, often, the native PAs.

Initial unifocalization attempts are usually deferred until the patient is age 3-6 months. Early unifocalization around age 1-3 months can be performed when protective stenosis in the MAPCAs is absent and congestive heart failure symptoms continue to worsen despite maximal medical therapy. Flow can also be reduced by percutaneous coil occlusion in the cardiac catheterization laboratory.

The last stage is usually performed between ages 1 to 3 years and involves closure of the VSD with or without fenestration, and establishment of continuity between the RV outflow tract and the PAs. The RV systolic pressure (RVSP) after correction should be less than 75% of that in the left ventricle.[8]

Complete repair

The objective of complete repair is to create an unrestricted continuity between the RV outflow tract and the PA 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. After correction, it is desirable to have a right ventricular pressure that is low, as close to normal as possible.

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 VSD 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 RV-PA) 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 PAs, heart-lung transplantation is a viable option.

Complications of surgery include the following:

  • Bronchospasm occurs after the unifocalization surgery because of tracheobronchial epithelial ischemia. This complication significantly contributes to early postoperative morbidity and mortality rates.
  • Pulmonary parenchymal reperfusion injury, pulmonary hemorrhage, and phrenic nerve injury.
  • 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.[9] 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

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. Patients will require antibiotic prophylaxis for subacute bacterial endocarditis (SBE) for an indefinite period.

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 Director, Section of Cardiology, Department of Pediatrics, San Jorge Children’s Hospital, Puerto Rico; Private Practice in Interventional Pediatric Cardiology and Internal Medicine, Centro Pedíatrico y Cardiovascular

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

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: St Jude's Medical Co.<br/>Received grant/research funds from NOVARTIS for investigator; Received consulting fee from St. Jude Medical Corp. for speaking and teaching.

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, 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, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Salaam Sallaam, MD Pediatric Cardiology Fellow, Children's Hospital of Michigan, Detroit Medical Center

Salaam Sallaam, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Society of Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

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, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, 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, 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, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

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Management algorithm for patients with pulmonary atresia with ventricular septal defect (PA-VSD) and major aortopulmonary collateral arteries (MAPCAs), based on the nature of pulmonary vascular supply. PAs = pulmomary arteries. Courtesy of Elsevier (Gupta A, Odim J, Levi D, Chang RK, Laks H. Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Experience with 104 patients. J Thorac Cardiovasc Surg. 2003;126(6):1746–52).
Anteroposterior still image obtained from angiography in the aortic arch of a 3-week-old infant born with pulmonary atresia with ventricular septal defect (PA-VSD) who is receiving prostaglandin E infusion. A patent ductus arteriosus (PDA) is seen supplying confluent branch pulmonary arteries. Courtesy of Dr Thomas Forbes.
Anteroposterior angiographic view in the aortic arch of a 3-week-old infant born with pulmonary atresia with ventricular septal defect (PA-VSD) who is receiving prostaglandin E infusion. A patent ductus arteriosus (PDA) is seen supplying confluent branch pulmonary arteries. Courtesy of Dr Thomas Forbes.
Anteroposterior still image obtained from angiography in a 3.5-mm right modified Blalock–Taussig (BT) shunt in the previous patient at age 4 months. There is a patent BT shunt with mild proximal right upper lobe and right lower lobe branch stenoses. Courtesy of Dr Thomas Forbes.
Anteroposterior angiographic view in a 3.5-mm right modified Blalock–Taussig (BT) shunt in the previous patient at age 4 months. There is a patent BT shunt with mild proximal right upper lobe and right lower lobe branch stenoses. Courtesy of Dr Thomas Forbes.
Lateral still image obtained from angiography in the previous patient at age 21 months. The infant underwent Rastelli operation with placement of a 15-mm pulmonary homograft. In this image, there is free homograft insufficiency without stenosis. Courtesy of Dr Thomas Forbes.
Lateral angiographic view in the previous patient at age 21 months. The infant underwent Rastelli operation with placement of a 15-mm pulmonary homograft. The presence of free homograft insufficiency with no stenosis is observed. Courtesy of Dr Thomas Forbes.
Lateral still image obtained from angiography in a 7-year-old boy born with pulmonary atresia with ventricular septal defect (PA-VSD) who underwent Rastelli operation with a 17-mm right ventricle to pulmonary artery (RV-PA) homograft. There is mild proximal conduit stenosis and free conduit insufficiency. The right ventricle appears moderately dilated. Courtesy of Dr Thomas Forbes.
Lateral angiographic view in a 7-year-old boy born with pulmonary atresia with ventricular septal defect (PA-VSD) who underwent Rastelli operation with a 17-mm right ventricle to pulmonary artery (RV-PA) homograft. There is mild proximal conduit stenosis and free conduit insufficiency. The right ventricle appears moderately dilated. Courtesy of Dr Thomas Forbes.
Lateral still image from angiography in the previous patient at age 8 years following Melody valve placement in the prestented 17-mm right ventricle to pulmonary artery (RV-PA) homograft. The Melody valve appears in good position. There is no Melody valve insufficiency. Courtesy of Dr Thomas Forbes.
Lateral angiographic view in the previous patient at age 8 years following Melody valve placement in the prestented 17-mm right ventricle to pulmonary artery (RV-PA) homograft. The Melody valve appears in good position. There is no Melody valve insufficiency. Courtesy of Dr Thomas Forbes.
Left anterior oblique ventriculogram in a patient (same patient as in the next image) with pulmonary atresia with ventricular septal defect (PA-VSD). The angiogram shows the left and right ventricles with a large malalignment VSD 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. Asc Ao = ascending aorta; Desc Ao = descending aorta; LV = left ventricle; and RV = right ventricle.
Anteroposterior view of an aortogram in a patient (same patient as in the previous image) 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).
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. LA = left atrium; PA = pulmonary artery; PV = pulmonary valve; RA = right atrium; RV = right ventricle; and TR = tricuspid valve.
 
 
 
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