Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

INTRODUCTION: In order to establish the best strategy of treatment and predictors of outcome in infants with pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries, we reviewed our institutional experience concerning 47 infants. METHODS: Inclusion criteria included an angiographic diagnosis of pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries with or without central pulmonary arteries and a repair by the same surgeon. Thirty-one patients had confluent (type III) and 16 absent (type IV) central pulmonary arteries. Pulmonary arteries were considered to be adequate when they measured > or = 4 mm. RESULTS: There were 8 deaths (17%). Complete repair was performed in 24 patients (18 in group III and 6 in group IV) with 79.3% had a right/left ventricular pressure <0.5, 16; 5%<1 and 4.2%>1. Eleven patients are waiting for complete repair and 4 will be operated on pulmonary arteries. One stage complete repair was performed in 3 patients, two and third stage repair (after unifocalisation or right ventricle to pulmonary arteries conduit) was attained in 21 patients. Among patients having had a complete repair and showing a right/left ventricular pressure <0.5, 88.9% had a pulmonary atresia with ventricular septal defect type III and 50% a pulmonary atresia with ventricular septal defect type IV. Only 56% of type III patients with a right/left ventricular pressure <0.5 had adequate central pulmonary arteries. CONCLUSION: In our study, the small size and the absence of central pulmonary arteries do not prevent a positive outcome.     

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Effect of systemic pulmonary anastomosis.

In nine patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, the effect of a systemic-pulmonary anastomosis on the lung was studied by comparison of pre- and postoperative cine angiograms. Selective injections into the collateral arteries were performed in all patients and the source of blood supply to each bronchopulmonary segment was sought. Central pulmonary arteries were present in six children and absent in three, as confirmed at thoracotomy. After insertion of a shunt, central pulmonary arteries increased in size, but the intrapulmonary vessels with which they connected remained abnormally small and were frequently stenosed. In addition, in each case the central pulmonary arteries increased flow to only five to 11 segments of lung. In the absence of central pulmonary arteries, shunting to a hilar or a lobar pulmonary artery increased perfusion to five to nine segments of lung per case. In all these three cases the pulmonary arteries at lobar level showed aneurysmal dilatation proximal to a severe stenosis. Sixty-eight per cent of collateral arteries were stenosed. The findings suggest that in this anomaly, when the central pulmonary arteries are hypoplastic, the intrapulmonary branches are also hypoplastic, emphasising the need for early surgical intervention to increase blood flow while the lung still has growth potential. Further, one collateral artery may connect with at least as many bronchopulmonary segments as does a central pulmonary artery, and the peripheral intrapulmonary arteries with which it connects may appear at least as normal angiographically as do vessels connected to central pulmonary arteries. These observations suggest that segments of lung connected to collaterals alone should, and sometimes can, be connected to central pulmonary arteries, the aim being to produce a unifocal blood supply as a prelude to total correction.     

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.     

Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery.

Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.     

Monosomy 22q11 in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries

Objective—To describe the morphology of the pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries with and without monosomy 22q11.
Design—A retrospective analysis of all patients with this congenital heart defect who are being followed at the University Children's Hospital Erlangen.
Setting—A tertiary referral centre for paediatric cardiology and paediatric cardiac surgery.
Patients—21 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Monosomy 22q11 was diagnosed by fluorescent in situ hybridisation using the D22S75 probe (Oncor). The morphology of the pulmonary arteries was assessed on the basis of selective angiograms.
Results—10 patients (48%) were shown to have a microdeletion in 22q11 (group I). There was no difference with respect to the presence of confluent central pulmonary arteries between these patients (80%) and the remaining 11 patients (group II) without monosomy 22q11 (91%). Patients of group I, however, more often had arborisation anomalies of the pulmonary vascular bed (90% in group I v 27% in group II). Because of the more severe abnormalities of the pulmonary arteries, a biventricular repair had not been possible in any of the children with monosomy 22q11, though repair had been carried out in 64% of the children in group II.
Conclusions—The developmental disturbance caused by monosomy 22q11 seems to impair the connection of the peripheral pulmonary artery segments to the central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, resulting in a lower probability of biventricular repair.

Keywords: CATCH 22;  pulmonary atresia and ventricular septal defect;  major aortopulmonary collateral arteries;  conotruncal anomaly face syndrome     

Pulmonary atresia and intact ventricular septum and corrected transposition of the great arteries.

We report an 82-day-old infant with a rare combination of anomalies: pulmonary atresia associated with corrected transposition of the great arteries and intact ventricular septum; this is the second such recorded case. By using echocardiography, chest radiography, and electrocardiography the correct anatomical diagnosis was obtained before invasive investigation and necropsy.     

Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Twenty-four-hour electrocardiograms were recorded in the first 10 days of life on 134 healthy full-term infants with birthweights greater than 2.5 kg. The highest heart rate a minute, measured over nine beats, was 175 +/- 19 (SD). The lowest rates, measured over three, five, and nine beats were 82 +/- 12, 87 +/- 12, and 93 +/- 12, respectively. At their lowest rates 109 infants had sinus bradycardia and 25 had junctional escape rhythms. Thirty-three infants showed changes in P wave configuration with or without pronounced variation in PR interval. Atrial premature beats were present in 19 infants but only one had more than 12 per hour. In a randomly selected subgroup of 71 infants sinus pauses were found in 51 (72%). Five (7%) had electrocardiographic patterns and rhythm disturbances that could not be differentiated from those previously described as complete sinuatrial exit block or sinus arrest, eight (11%) had patterns indistinguishable from 2:1 sinuatrial block, and 23 (32%) had pattern indistinguishable from sinuatrial Wenckebach block. This study shows that normal infants have variations in heart rate and rhythm hitherto considered to be abnormal.     

Pulmonary atresia with intact ventricular septum

Opinion statement The prognosis for patients with pulmonary atresia with intact ventricular septum is poor with and without conventional surgical treatment. Because of this reason, a comprehensive program of medical, transcatheter, and surgical treatment is necessary to improve the long-term outlook of these infants. Algorithms of management plans should be developed based on the presence of right ventricular-dependent coronary circulation as well as size and morphology of the right ventricle. In a tripartite or bipartite right ventricle, transcatheter radiofrequency perforation is preferable. Alternatively, surgical valvotomy may be performed. Augmentation of pulmonary blood flow by prolonged infusion of prostaglandin E₁, stenting the ductus, or a surgical modified Blalock-Taussig shunt may be necessary in some of these patients. In patients with a unipartite or very small right ventricle or a right ventricular-dependent coronary circulation (Tricuspid valve Z score < -2.5), augmentation of pulmonary flow along with atrial septostomy should be undertaken. Follow-up studies to determine the feasibility of biventricular repair should be undertaken and, if feasible, surgical or transcatheter methods may be used to achieve the goals. If not suitable for biventricular repair, one-ventricle (Fontan) or one and one-half ventricular repair should be considered. Comprehensive and well-planned treatment algorithms may help improve survival rate.     

Pulmonary atresia with intact ventricular septum and antenatal left ventricular failure

Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.     

Pulmonary valvular and infundibular atresia with intact ventricular septum

This case is one of combined infundibular and valvular pulmonary atresia with intact ventricular septum in which virtual absence of the tricuspid valve and a normal sized right ventricle were associated. Of particular interest was the presence of an endothelial-lined blind infundibular chamber. This finding, never previously reported, complicated surgical repair.     

Tetralogy of Fallot with major aortopulmonary collateral arteries.

The clinical features of eight patients with tetralogy of Fallot and major aortopulmonary collateral arteries were determined and found to be usually unhelpful in differentiating such patients from those with pulmonary atresia with ventricular septal defect. The point of connection between the pulmonary and collateral arteries could usually be demonstrated at cineangiography by observing washin and washout between the two systems. The central pulmonary arteries connected with the entire pulmonary vascular bed in the five patients in whom the anatomy of the pulmonary circulation was clearly demonstrated and were thought to do so in the remaining three patients also. In six out of eight patients only one collateral artery connected with each hilar pulmonary artery. These findings suggest that the anatomy of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries is usually less complex than in pulmonary atresia with ventricular septal defect, making the outlook for complete repair more hopeful. The major determinant of successful complete repair appears to be pulmonary arterial size. Early palliative surgery to increase pulmonary blood flow is recommended to encourage normal growth of the central and intrapulmonary arteries.