Atresia of right pulmonary veins with intact atrial septum and major aorto-pulmonary collateral treated with percutaneous stent implantation and embolization

Unilateral pulmonary vein (PV) atresia is a rare congenital cardiac malformation with evolution toward irreversible pulmonary hypertension. Pneumonectomy or lung transplant is currently the treatment of choice for such a disease. We describe an unusual case of right PV atresia and major aorto-pulmonary collaterals treated with percutaneous angioplasty, stent implantation, and aorto-pulmonary collateral embolization.     

Accuracy of MRI evaluation of pulmonary blood supply in patients with complex pulmonary stenosis or atresia

Detailed imaging of pulmonary artery (PA) anatomy and significant aorto-pulmonary collaterals (APCs) is crucial for surgical planning and follow-up in patients with complex congenital heart disease (CHD) and pulmonary stenosis or atresia. Because examination by echocardiography is often technically limited and catheterization is invasive, this study evaluated the diagnostic accuracy of magnetic resonance imaging (MRI) as an alternate non-invasive tool. Thirteen patients (median age 28 years, range: 1–44 years) underwent both cardiac catheterization and MRI within a median of two months (range 0.1–8 months). Diagnoses included tetralogy of Fallot (TOF) with pulmonary atresia (n = 8), TOF with pulmonary stenosis (n = 2), single left ventricle with pulmonary stenosis (n = 2), and complex heterotaxy with pulmonary stenosis (n = 1). The MRI sequences used in this study were ECG-gated spin echo and gradient echo sequences acquired in multiple planes. Compared to catheterization, MRI had 100% sensitivity and specificity for the diagnosis of main PA (n = 6) and branch PA (n = 13) hypoplasia or stenosis, as well as discontinuous (n = 4) or absent (n = 10) branch PAs. There was complete agreement between catheterization and MRI identification of significant APCs (n = 18). Main PA atresia was noted by MRI in four patients but was not definitively seen by catheterization in any. MRI but not catheterization precisely defined the distance between discontinuous PAs and their relationship to other mediastinal structures. In conclusion, cardiac MRI is a reliable non-invasive imaging modality to define PA and APC anatomy in patients with complex pulmonary stenosis or atresia.     

Early restenosis following biodegradable stent implantation in an aortopulmonary collateral of a patient with pulmonary atresia and hypoplastic pulmonary arteries.

Traditionally, the implantation of stents in young children with small blood vessels has been avoided as the presence of a metallic or fixed stent structure limits the ability to further dilate the stent with vessel growth. Pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries represents one such cohort where the placement of fixed nondegradable stents has been difficult. We report a 2-month-old girl with pulmonary atresia, VSD, and multiple aorto-pulmonary collaterals with severely hypoplastic pulmonary arteries who underwent placement of a biodegradable magnesium stent within a stenotic aorto-pulmonary collateral. Although there was an initial significant increase in vessel diameter, significant restenosis occurred 4 months after stent placement. This to our knowledge represents the first case of the use of biodegradable stents in a child with severely hypoplastic pulmonary arteries. Although further refinements in stent technology are required, this development will hopefully usher in a new era of potential intervention in children previously deemed unsuitable for stent placement.     

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.     

In response to the article by Andrew J. Powell, Taylor Chung, Michael J. Landzberg & Tal Geva Accuracy of MRI evaluation of pulmonary blood supply in patients with complex pulmonary stenosis or atresia

Detailed imaging of pulmonary artery (PA) anatomy and significant aorto-pulmonary collaterals (APCs) is crucial for surgical planning and follow-up in patients with complex congenital heart disease (CHD) and pulmonary stenosis or atresia. Because examination by echocardiography is often technically limited and catheterization is invasive, this study evaluated the diagnostic accuracy of magnetic resonance imaging (MRI) as an alternate non-invasive tool. Thirteen patients (median age 28 years, range: 1–44 years) underwent both cardiac catheterization and MRI within a median of two months (range 0.1–8 months). Diagnoses included tetralogy of Fallot (TOF) with pulmonary atresia (n = 8), TOF with pulmonary stenosis (n = 2), single left ventricle with pulmonary stenosis (n = 2), and complex heterotaxy with pulmonary stenosis (n = 1). The MRI sequences used in this study were ECG-gated spin echo and gradient echo sequences acquired in multiple planes. Compared to catheterization, MRI had 100% sensitivity and specificity for the diagnosis of main PA (n = 6) and branch PA (n = 13) hypoplasia or stenosis, as well as discontinuous (n = 4) or absent (n = 10) branch PAs. There was complete agreement between catheterization and MRI identification of significant APCs (n = 18). Main PA atresia was noted by MRI in four patients but was not definitively seen by catheterization in any. MRI but not catheterization precisely defined the distance between discontinuous PAs and their relationship to other mediastinal structures. In conclusion, cardiac MRI is a reliable non-invasive imaging modality to define PA and APC anatomy in patients with complex pulmonary stenosis or atresia.     

Aorto-pulmonary collaterals in an infant with 22q11 monosomy

The authors present the case of a young girl in whom the diagnosis of pulmonary atresia with ventricular septal defect and multiple aorto-pulmonary collateral vessels was established in the context of microdeletion of chromosome 22. Surgical treatment was excluded following the discovery by angiography of the absence of central pulmonary arteries, a multifocal pulmonary collateral circulation and the appearance of multiple peripheral pulmonary artery stenoses on various aorto-pulmonary collateral vessels during the first months of life. This case illustrates the hypothesis recently developed in the literature according to which the chromosomal abnormality is responsible not only for an anomaly of conotrunkal septation, but also early disorders of branching of the pulmonary vascular bed and disturbances of the connection of peripheral pulmonary segments to central arteries, which are responsible for severe pulmonary vascular abnormalities making biventricular repair of this heart disease in children with 22q11 microdeletion almost always impossible.     

Usefulness of interlocking detachable coil for embolization of the major aorto-pulmonary collateral artery: A case report

A 5-yr-old male had undergone a right-modified Blalock-Taussig shunt operation at the age of 1 mo for stenotic patent ductus arteriosus and pulmonary atresia with ventricular septal defect. Before a corrective operation, the angiogram revealed one large major aorto-pulmonary collateral artery from the descending aorta to the right upper lung. Embolization of the major aortopulmonary collateral artery was successfully performed using interlocking detachable coils. This is the first reported case of embolization of the major aorto-pulmonary collateral artery using this device.     

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.     

Three-dimensional multislice CT scanner: value in patients with pulmonary atresia with septal defect

The aim of this study was to evaluate the interest of multislice CT scanning using three-dimensional reconstructions for pre- or postoperative management of patients with pulmonary atresia with ventricular septal defect. Twenty-one consecutive patients (median age: 8 months, median weight: 5.6 kg) with pulmonary atresia with ventricular septal defect were explored by multislice CT. Standard protocol consisted in one helical thoracic acquisition, associated with an injection of 2 to 3 mL/kg of iodinated contrast medium at a flow rate from 0.5 to 1 mL/s. Three-dimensionnal reconstructions were systematically performed. Before operation, status and size of central pulmonary arteries, major aorto-pulmonary collateral arteries, and associated anomalies were analyzed. In 13 operated patients, the permeability of palliative anastomosis and postoperative anatomical changes were studied. The status of central pulmonary arteries was assessed in all cases and the sizes found were in agreement to surgical findings. Main aorto-pulmonary collateral arteries were depicted and their relative position to central airways was assessed. Various associated anomalies were found: right aortic arch, anomalous coronary artery, abnormal pulmonary venous return, left superior vena cava. The anastomosis and the surgical connections (unifocalization, complete repair) were clearly analyzed. The multislice CT with three-dimensional reconstructions is a complementary imaging technique, reliable, useful to pre-operative management and postoperative follow-up of patients with pulmonary atresia with ventricular septal defect.     

Clinical and angiocardiographic observations on the so-called absence of a branch of the pulmonary artery

We present 5 cases with angiographically absent pulmonary artery: 4 with absence of right pulmonary artery and 1 with absence of the left. We excluded cases of atresia of pulmonary artery, ventricular septal defect and major aorto-pulmonary collaterals. We excluded also cases of anomalous origin of a pulmonary artery from the aorta. Surgical inspection in 3 patients demonstrated total absence of pulmonary main branch (1 case), with intraoperative death, and presence of the hilar portion of the pulmonary artery (2 cases), with successful palliative procedure. It is supportive of efforts to identify as early in life as is feasible the absent vessel, for surgical purposes. In fact the early surgical procedure will ensure development of the vessel and normal lung growth, and will decrease the risk of the following intracardiac repair, when needful.     

Pregnancy outcome in patients with complex pulmonary atresia: case report and review of the literature

Pulmonary atresia, a rare and complex congenital heart disease, is characterized by the absence of the central pulmonary artery and by the presence of a ventricular septal defect and aortopulmonary collaterals. Pregnancy reports concerning maternal and offspring outcome after palliative operation or repaired pulmonary atresia are sparse. We report here on the outcome of pregnancy in a woman, aged 36, with complex pulmonary atresia in whom palliative operation had been performed at the age of 23. We review the medical literature on pregnancy course as well as maternal and foetal outcome in cases involving this maternal congenital heart disease.     

Coil embolization of iatrogenic arterio-venous fistula from pulmonary collateral artery to subclavian vein

A young woman with surgically treated tetralogy of Fallot with pulmonary atresia, with multiple aorto-pulmonary collateral arteries, developed an arteriovenous fistula subsequent to transvenous insertion of an automatic implantable defibrillator via the left subclavian vein. The fistula extended between this vein and a systemic-to-pulmonary collateral artery arising from the subclavian artery. Unilateral peau d'orange, and painful congestion of the left arm and breast, ensued. These were cured by coil embolization of a fistula-related aneurysm.     

Pulmonary atresia, "intact ventricular septum", and aortopulmonary collateral arteries.

In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricuspid tissue. In both cases the pulmonary blood supply came entirely from aortopulmonary collaterals. In case 1 a collateral artery connected the left subclavian artery and hypoplastic pulmonary arteries, and several aortopulmonary collaterals arose from the descending aorta, without overlap between these two circulations. In case 2 the pulmonary trunk and arterial duct were absent and the pulmonary blood supply came entirely from collateral arteries. The right ventricle was of normal size and tripartite with a closed perimembranous ventricular septal defect, discovered only at postmortem examination. These observations suggest right ventricular outflow tract obstruction early in fetal development, with involution of the pulmonary trunk and sixth arch derivatives, and persistence of primitive aortopulmonary connections. The morphology in case 1 is at odds with the theoretical division of pulmonary atresia with intact septum and pulmonary atresia with ventricular septal defect into two separate pathological entities that occur at different stages in fetal development.     

Rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect. Medium term results

The treatment of pulmonary atresia with ventricular septal defect, hypoplasia of the native pulmonary arteries and major aorto-pulmonary collaterals is controversial with a wide range of options from symptomatic treatment to surgical correction, but usually unifocal surgery. However, the collateral vessels used in the reconstruction are often tortuous and stenosed, resulting in a problematic haemodynamic result. The authors report the results of medico-surgical rehabilitation of the native pulmonary arteries in three stages. Since 1990, patients with an average Nakata index of 27.5 +/- 16.7 mm2/m2 underwent elective surgical connection of the native pulmonary arteries to the right ventricle (1st stage) followed by their rehabilitation with catheter occlusion of the collaterals (2nd stage) with the aim of preparing surgical correction (3rd stage). The first surgical stage at an median age of 5 months (0.1 to 25.2) was complicated by one death. After an average of 2.1 +/- 1 catheterisations, 17 patients underwent surgical correction at an median age of 2.18 (0.6 to 10.3) years with an average Nakata index of 207 +/- 91 mm2/m2. All survived surgery with three late deaths in patients with poor haemodynamic results (right ventricular/left ventricular pressure ratio>80%). After an median follow-up of 9.2 (3.5 to 17) years, the 14 survivors are in NYHA Class I or II with a good haemodynamic result in 10 patients. The authors conclude that despite some late deaths and raised right ventricular and pulmonary artery pressures in a minority of patients, the rehabilitation of the pulmonary arteries is an effective management of this cardiopathy.     

Collateral pulmonary circulation in Fallot's tetralogy

An angiographic study of 231 patients with tetralogy of Fallot (TOF) in both neonatal and adult age groups (0 to 49 years) was in this condition. Cases of pulmonary atresia with ventricular septal defect were excluded. Two types of collateral pulmonary circulation were observed: the most common form is the result of distal intrapulmonary anastomosis between systemic parietal, mammary and bronchial vessels with the pulmonary arteries. This type of collateral circulation represents an evolutive stage of TOF and is directly related to age and to the degree of hypoxia; the other, oa rare and probably congenital form (1.7 p. 100 of cases) observed even in young children, consisting of proximal anastomoses between systemic vessels arising from the aorta (1 to 3 vessels) and the pulmonary arterial tree. The rare forms of TOF with single pulmonary arteries acquired after palliative or congenital anastomoses (1.4 p. 100) have both types of collateral circulation. Preoperative diagnosis of the collateral pulmonary circulation requires opacification of the aorta especially of the descending thoracic segment and its branches, completed in cases of an absent pulmonary artery by wedged pulmonary venous opacification. The haemodynamic effects of this collateral circulation on the pulmonary bed to a variable degree of histological change. This may partially explain some of the poor surgical results of repair of TOF and, in view of recent progress in cardiopulmonary bypass techniques in children, incite to earlier surgical correction of this condition.     

Stent implantation in right-sided patent ductus arteriosus to relieve severe cyanosis in adult patient with pulmonary atresia and ventricular septal defect.

Patients with unrepaired pulmonary atresia and ventricular septal defect may develop stenosis of collaterals or shunts to the pulmonary arteries leading to hypoperfusion of lungs and systemic hypoxemia. A 25-year-old female with pulmonary atresia and ventricular septal defect presented with progressively increasing cyanosis and exercise intolerance. A restrictive right-sided patent ductus arteriosus was identified as the main source of pulmonary blood flow. We report transcatheter implantation of a balloon-expandable stent across the stenosed duct to augment the pulmonary blood flow as a palliative management option. Patient had immediate improvement in arterial oxygen saturation from 66% to 85% with excellent clinical improvement and stable oxygen saturation on 8 months of follow-up.     

Rehabilitation of hypoplastic pulmonary arteries and anatomic correction of pulmonary atresia with interventricular communication]

Conventional treatment of pulmonary atresia with ventricular septal defect (VSD), hypoplastic pulmonary arteries (PA) and major aorto-pulmonary collaterals (MAPCAs) is controversial: from symptomatic and palliative treatment for some authors to surgery with unifocalisation of collaterals for others. These treatments never use native pulmonary arteries as only source of pulmonary flow, but create "neo-pulmonary arteries". Nine cases of pulmonary atresia with VSD, hypoplastic PA and MAPCAs were treated by rehabilitation of native PA through a staged approach: 1) surgical neonatal connection between right ventricule (RV) and hypoplastic PA, 2) evaluation and interventionnal catheterism with angioplasty of PA stenosis and closure of collaterals, 3) complete surgical correction with reconstruction of right outflow track and PA and closure of VSD. After first surgical stage of RV-PA connection at the mean age of 4.8 months (+/- 5.6 months), 8 patients were alive and underwent 22 cardiac catheterisms (mean of 2.7 per patient), with angioplasty of PA, and occlusion of MAPCAs in 6 and 2 patients respectively. Seven patients underwent complete anatomical correction at the mean age of 28.8 months (+/- 17.7 months) with one late death. The 6 remaining patients had encouraging hemodynamic status (RV pressure/LV pressure ratio at 0.6 +/- 0.26; mean left and right distal pulmonary pressure at 15.2 mmHg (+/- 9.1 mmHg)), and good functionnal status (3 in NYHA functionnal class 1, and 3 in class 2), for a mean follow-up of 79.5 months (+/- 41.4 months). One patient had reoperation on right outflow track stenosis, 6 years after correction. This small series enhances the feasibility of a staged approach with rehabilitation of small PA, allowing complete surgical correction with the native PA with good hemodynamic and functional results in pulmonary atresia, with VSD, hypoplastic PA and MAPCAs.     

Stenting of stenosed aortopulmonary collaterals and shunts for palliation of pulmonary atresia/ventricular septal defect.

Patients with unrepaired pulmonary artery atresia and ventricular septal defect (PA/VSD) depend on aortoplumonary collaterals and surgically created shunts for pulmonary blood flow. These vessels frequently develop stenoses with time, leading to hypoperfusion of lung segments and systemic hypoxemia. The purpose of this article is to describe catheter palliation of hypoxemic patients with PA/VSD who were not candidates for surgical repair. We present our experience with stent implantation for stenosis of aortopulmonary collaterals and shunts in these patients. Three patients with hypoplastic pulmonary arteries underwent stent placement in aortopulmonary collateral arteries (APCAs) or their shunts. Technical aspects of the interventional catheterization procedure are discussed in detail. Case 1 underwent placement of five stents in collateral vessels and one stent in the Blalock-Taussig shunt (BT) with dramatic increase in vessel size and improvement in saturations from 70% to 89%. Case 2 underwent placement of two overlapping stents in a collateral vessel with an increase in diameter of the collateral vessel from 2.3 to 6 mm and an improvement in saturation from 68% to 88%. Case 3 underwent placement of three overlapping stents in a BT shunt with an increase in diameter of the shunt from 2.2 to 6.6 mm and an improvement in saturation from 71% to 89%. All three patients had excellent clinical improvement and stable saturation at follow-up. Stent placement for maintaining patency of APCAs and aortopulmonary shunts is feasible and safe.     

Haemoptysis in a patient with tetralogy of Fallot: a combined surgical and interventional approach

Haemoptysis may occur in patients with tetralogy of Fallot and major aorto-pulmonary collateral arteries. We describe such a patient in whom bleeding from a major aorto-pulmonary collateral artery produced severe pulmonary haemorrhage. Interventional closure of the artery could not be performed because it perfused the native pulmonary arteries. Instead, we inserted a conduit between the right ventricle and the native pulmonary arteries, followed by percutaneous closure of the collateral artery. Our patient demonstrates the increasing necessity for combined surgical and interventional procedures.     

A large coronary-pulmonary artery fistula in a cyanotic patient leading to severe biventricular dysfunction and heart failure

In cases of pulmonary atresia (PA) with ventricular septal defect (VSD), the commonest source of pulmonary blood flow is via major aortoplumonary collaterals. Collaterals may also arise from the coronary arteries and the reported prevalence of such coronary-pulmonary artery fistulas (CPAF) in PA with VSD is 10%. However gross congestive heart failure (CHF) and ventricular dysfunction is extremely rare in such cases. We report a 17-year-old male with PA with VSD and a large CPAF from the anterior right aortic sinus connecting to the left pulmonary artery, who presented with severe CHF. The left anterior descending and the right coronary artery both arose from the proximal part of the CPAF, possibly leading to extensive coronary steal and biventricular dysfunction.