Management of pulmonary atresia with ventricular septal defect

Opinion statement It has been nearly 40 years since Kirklin, at the Mayo Clinic in 1964, performed the first surgical repair of pulmonary atresia with ventricular septal defect using a nonvalved extracardiac conduit, which he created out of the patient’s pericardium, and this patient continues to do well. In the subsequent four decades, great advances have been made in the diagnosis, with regard to the often very complex anatomy of pulmonary blood supply that this extremely heterogeneous group of patients manifest, and their subsequent surgical management. Unifocalization procedures have permitted eventual complete correction in patients with nonconfluent pulmonary artery segments, and advances in infant surgery have enabled most patients to achieve correction in the early childhood years. Although the ideal extracardiac conduit has yet to be developed, necessitating periodic conduit replacement in corrected patients, the late results in this group of patients are generally excellent, with many now achieving survival well into the adulthood years with good quality of life. Continued advances in surgery and interventional catheterization techniques hold great promise for ever-improving outcomes in children being born with this complex congenital cardiovascular malformation.     

Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect

The heart and lung specimens in 31 cases of pulmonary value atresia and ventricular septal defect were studied at autopsy. Three types of natural arterial blood supply to the lungs were identified: 1) ductus arteriosus (patient or ligamentous) (12 cases); 2) major collateral arteries (20 cases); and 3) diffuse small pleural arterial plexus coexisting with either ductus arteriosus or major collateral arteries (17 cases). The ductus arteriosus and major collateral arteries did not coexist in the same lung in these cases. Confluent central pulmonary arteries were present in 22 (71%) of the 31 cases, involving 7 (58%) of the 12 cases of ductus arteriosus, 14 (70%) of the 20 cases with major collateral arteries and 1 case with an aorticopulmonary window. The pulmonary trunk (atretic or patent) was identifiable in 24 (77%) of the 31 cases. A lung or lungs that connected to a ductus (or ligamentum) had a complete and unifocal intrapulmonary arterial distribution (without arborization abnormalities). Major collateral blood supply was frequently multifocal and associated with arborization abnormalities. The size of the central pulmonary arteries was not related to the type of arterial blood source but seemed to be related to the amount of blood flow actually reaching the vessels, This study demonstrated a complex systemic arterial system supplying the lungs in these cases. The size, sources and relation among the ductus, the pulmonary artery confluence, the large and small collateral vessels and the intrapulmonary system are far more varied than has ever been reported previously. Careful and thorough premortem studies are crucial if surgical intervention is contemplated.     

Pulmonary atresia with ventricular septal defect in adult patients

Hemodynamic data and angiograms of 15 adult patients with pulmonary atresia and ventricular septal defect were reviewed to assess the pulmonary circulation and other associated features. The most common variety of pulmonary atresia was that of pulmonary valve, main pulmonary artery, and the confluence of pulmonary arteries (6 cases, 40%). The collateral vessels to the lungs were well developed in all cases; selective injections into the collateral vessels were of great value in their delineation. The left ventricle was well developed in 11 cases (73.3%). Congestive heart failure was seen in 8 (53.3%), tricuspid regurgitation in 10 (66.7%), and aortic regurgitation in 7 (46.7%) cases. The long survival in these patients was related to the favorable anatomy of central pulmonary arteries (12 cases, 80%) and adequate pulmonary collateral circulation.     

Coronary artery-pulmonary artery fistula in pulmonary atresia with ventricular septal defect

Summary This paper reports two patients presenting a combination of coronary artery-pulmonary artery fistula and pulmonary atresia with ventricular septal defect. A confluent central pulmonary artery arose from the proximal portion of the left coronary artery in both patients. The fistulous segment between the coronary artery and the central pulmonary artery was large, and no coronary arterial stenosis was found in either patient. Besides this fistulous origin of the pulmonary blood supply, large collateral vessels originated from the descending aorta in both patients. Neither patient has shown findings of myocardial ischemia, at least in consecutive rest and exercise electrocardiograms. Both patients were successfully operated, at the ages of 3 and 8 years, respectively. The clinical and embryological implications of this rare malformation are briefly reviewed.     

Coronary artery to pulmonary artery communications in pulmonary atresia with ventricular septal defect

Coronary artery to pulmonary artery communications (CAPAC) are an important source of pulmonary blood flow in approximately 10% of patients with pulmonary atresia and ventricular septal defect (PA-VSD). A diligent look for these abnormal communications is important to prevent perioperative complications and achieve a complete repair. We present two cases of PA-VSD with CAPAC, one in a 7-year-old child and the other in a 33-year-old adult. The method of occlusion of these communications could be either surgical or catheter based.     

Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect.

A histologic study was performed on 22 specimens of pulmonary atresia with ventricular septal defect to 1) ascertain the existence of the main pulmonary artery; 2) distinguish the ductus arteriosus from the systemic collateral arteries (SCA); 3) establish the nutritive or functional nature of collateral circulation; and 4) evaluate the morphology of the distal pulmonary bed. Three cases had absent main pulmonary artery, one with and two without signs of infundibular septation. We suggest that absent main pulmonary artery may exist with both infundibular pulmonary atresia and persistent truncus arteriosus. SCAs have been found to have similar histological features as systemic muscular arteries of the same size--their medial muscular layer merges gradually into an elastic one at different depth inside the lungs. Injection of contrast material allowed us to demonstrate that these vessels are functional, since they inosculate into efficient pulmonary arteries ending in the respiratory units. When the distal pulmonary vascular bed is perfused by large SCAs, proliferative lesions like those found in large left-to-right shunts may occur. Early in infancy, banding of large, nonstenotic SCAs could protect the distal pulmonary vasculature. Moreover, total surgical repair should be associated with ligation of the SCA to avoid residual left-to-right shunt, if the pulmonary arteries can carry the full pulmonary blood flow.     

Cannulation of patent arterial duct in patients with pulmonary atresia and ventricular septal defect.

During cardiac catheterization in patients with pulmonary atresia and ventricular septal defect, cannulation of the arterial duct was attempted in 23 children via antegrade approach. It was successful in all but two children. Injection of contrast through antegradely placed catheter resulted in improved opacification of pulmonary arteries. This technique facilitated transcatheter dilation of spontaneously closed arterial duct in two children.     

Pregnancy after biventricular repair for pulmonary atresia with ventricular septal defect

Information on pregnancy and delivery in women with biventricular repair for isolated noncomplex pulmonary atresia with a ventricular septal defect (PAVSD) is limited. Using a nationwide congenital heart disease registry (CONgenital CORvitia [CONCOR]), 9 women with biventricular repair for PAVSD (aged 21 to 38 years) were identified. Ten pregnancies were observed in 5 different women with PAVSD, including 3 spontaneous miscarriages. Clinically significant (non)cardiac complications were documented in 3 of 7 completed pregnancies. These complications were: (1) atrioventricular reentry tachycardia with symptomatic right-sided heart failure; (2) eclampsia with hemolysis, elevated liver enzymes, and low platelets syndrome further complicated by abruptio placentae leading to premature delivery of a small-for-gestational-age child; and (3) premature delivery due to cervical insufficiency with antepartum demise of an immature child. Furthermore, none of the women reported infertility. Moreover, none of the women reported irregularities of their natural menstrual cycle (age at menarche 13 years; cycle duration 28 days), with the exception of delayed menarche (>16 years) in 2 patients. In conclusion, successful pregnancy in patients with biventricular repair of PAVSD is possible, although often complicated by serious clinically significant events. Infertility and menstrual cycle disorders do not appear to be more prevalent, except for a high incidence of primary amenorrhea.     

Duplicate sources of pulmonary blood supply in pulmonary atresia with ventricular septal defect.

In pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, there is a tendency for each collateral artery to be the sole supply to a particular region of lung. On injection into a collateral artery, however, "wash-out" of contrast medium by non-opacified blood from a second source is sometimes seen. Alternatively, contrast medium may faintly "wash-in" to an adjacent lobar artery supplied from a second source. "Wash-in" and "wash-out" therefore show that a duplicate blood supply exists. To assess the importance of these phenomena, we reviewed the angiograms of 50 patients with this condition. Duplicate pulmonary blood supply not resulting from surgical shunts was found in 25 patients (50%), and in 24 of 37 patients (65%) who had selective collateral injections. In one patient two collaterals anastomosed with each other. Thirty-nine instances of duplicate supply occurred in the remaining 24. The duplicate connection was to a region of lung connected to a central pulmonary artery in 29 of 39 instances (74%). Determination of how much of the peripheral pulmonary vasculature is connected to central pulmonary arteries greatly affects decisions about palliative and corrective surgery. These results show that unless "wash-in" and "wash-out" are specifically looked for, the information obtained from angiography may be erroneous.     

The value of Doppler color flow mapping in determining pulmonary blood supply in infants with pulmonary atresia with ventricular septal defect

Thirty-two neonates and infants with pulmonary atresia with ventricular septal defect were initially investigated with cross-sectional and spectral Doppler echocardiography and Doppler color flow mapping. All 32 had subsequent correlative angiography. This demonstrated that 24 infants had adequate-sized right and left pulmonary arteries (19 confluent, 5 nonconfluent). Of the five infants with nonconfluent pulmonary arteries, four had bilateral ductus arteriosus and one had a single left-sided ductus with anomalous origin of the right pulmonary artery from the ascending aorta. Nineteen infants had confluent pulmonary arteries, all of which were supplied by a single ductus. Eight infants had complete absence of or inadequate pulmonary arteries; all had multiple aortopulmonary collateral vessels arising from the descending aorta. The presence of adequate-sized right and left pulmonary arteries was correctly predicted in 21 of 24 infants by cross-sectional echocardiography alone and in all 24 by Doppler color flow mapping. Confluence of the right and left pulmonary arteries was predicted by cross-sectional imaging in 14 of the 19 infants in whom it occurred, and by Doppler color flow mapping in all 19 infants. The precise definition of the pulmonary blood supply was correctly predicted by Doppler color flow mapping in 16 of the 19 infants with confluent pulmonary arteries and a single ductus. However, in three infants in this group, Doppler color flow mapping made a false diagnosis of multiple aortopulmonary collateral vessels.(ABSTRACT TRUNCATED AT 250 WORDS)     

Stenting stenosed aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect

We present two cases of pulmonary atresia with ventricular septal defect who were not suitable for corrective surgery due to absent or hypoplastic native pulmonary arteries and were quite symptomatic following shunt surgery. We dilated and stented stenosed aortopulmonary collaterals as palliative procedure with improvement in oxygen saturation, and significant symptomatic relief.     

Annuloplasty of cryopreserved pulmonary homograft with Delrin stent in pulmonary atresia with ventricular septal defect

A six-year-old child was suffering from pulmonary atresia with ventricular septal defect after a total correction at 1 year of age using a bovine pericardial monocusp valve in the reconstruction of the right ventricle outflow tract. The infant evolved with significant pulmonary valve regurgitation (PVR) and right ventricle dysfunction. On reoperation, a cryopreserved pulmonary homograft (CPH) was implanted with annuloplasty utilizing a Delrin ring with the aim of avoiding geometric distortion of the vessel. After two years, an echocardiogram proved a similar state to the immediate postoperative period with slight pulmonary valve regurgitation and normal right ventricular function suggesting that this maneuver may be used as coadjuvant treatment to optimize the result of CPH implantation.     

Coronary artery fistulas in pulmonary atresia and ventricular septal defect

Pulmonary atresia with ventricular septal defect is an anomaly with highly variable anatomy. Rarely, a coronary artery-to-pulmonary artery fistula may contribute to pulmonary blood flow. Since 1996, we have treated 4 patients with coronary-pulmonary fistula associated with pulmonary atresia and ventricular septal defect. Two fistulas originated from the left coronary, one from the right coronary, and one from a right-sided solitary coronary system. All terminated in the main pulmonary artery, which was adequate in all cases. The fistulas were managed by direct internal closure. Total intracardiac repair was then accomplished in all patients at the same sitting. There was one death. In children with favorable anatomy, direct closure of the fistula from the pulmonary artery is adequate and allows single-stage intracardiac repair.