Guidelines Summary

The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020.^[2]Their recommendations regarding surgical intervention in and follow-up of patients with pulmonary atresia with ventricular septal defect (PA-VSD) are outlined below.

Surgical intervention

Note the following:

Patients with unrepaired PA-VSD, or those who underwent previous palliative procedures, who survive to adulthood: Modern surgical or interventional procedures may be beneficial.
Consider the following patients for surgery: Those with good-sized confluent PAs and those with large major aortopulmonary collateral arteries (MAPCAs) that are anatomically suitable unifocalization, who have not developed severe pulmonary vascular disease owing to protecting stenosis
Catheter interventions may include balloon dilation/stenting of collateral vessels for pulmonary blood flow enhancement; however, patients with severe hemoptysis may need to undergo coiling of the ruptured collateral vessels.
Patients with good-sized PAs absent a pulmonary trunk: Repair with a right ventricle (RV) to pulmonary artery (RV–PA) conduit.
Patients with confluent but hypoplastic PAs: An arterial shunt or reconstruction of the RV outflow tract (OT) (without VSD closure) is usually needed. This may enhance PA growth and then be reviewed at a later stage for repair using a valved conduit.
Patients with nonconfluent PAs and adequate, but not excessive, pulmonary blood flow in infancy: These patients can survive into adulthood without surgery.

Follow-up

Periodic follow-up of patients with PA-VSD is recommended at least once every year at a specialized ACHD institution. Monitor closely and evaluate and review sooner in the setting of symptoms such as dyspnea, increasing cyanosis, change in shunt murmur, heart failure, or arrhythmias.

Consider patients with segmental pulmonary arterial hypertension (PAH) for targeted PAH therapy.

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Media Gallery

Pulmonary Atresia With Ventricular Septal Defect. 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).
Pulmonary Atresia With Ventricular Septal Defect. 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 a prostaglandin E infusion. A patent ductus arteriosus (PDA) is seen supplying confluent branch pulmonary arteries. Courtesy of Dr Thomas Forbes.
Pulmonary Atresia With Ventricular Septal Defect. 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 a prostaglandin E infusion. A patent ductus arteriosus (PDA) is seen supplying confluent branch pulmonary arteries. Courtesy of Dr Thomas Forbes.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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.
Pulmonary Atresia With Ventricular Septal Defect. 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).
Pulmonary Atresia With Ventricular Septal Defect. 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.