Pulmonary Branches Research Articles

Communication between the right pulmonary artery and left atrium with aneurysm formation

Communication between the pulmonary artery and the left atrium is a rare congenital anomaly. A 7-year-old boy was referred to our hospital with a diagnosis of pulmonary arteriovenous fistula. Referral to a pediatric cardiologist had been delayed because he had only slight cyanosis and no heart murmur. Catheterization and computed tomography scans confirmed that the right pulmonary branches and pulmonary venous returns were normal and that an additional aneurysmal communication was located between the right pulmonary artery and the left atrium. Surgery proceeded uneventfully together with a cardiopulmonary bypass. The connection from the right pulmonary artery to the aneurysm was ligated, and the connection from the left atrium to the aneurysm was closed with a polytetrafluoroethylene patch. We describe this rare anomaly with cyanosis and no heart murmur.

Evidence Mounts That Nitrite Contributes to Hypoxic Vasodilation in the Human Circulation

Hypoxic vasodilation is a conserved physiological response to hypoxia that matches blood flow and oxygen delivery to tissue metabolic demand. This fundamental physiological process has been characterized for >100 years since the initial description by Roy and Brown1 in 1880. Hypoxic vasodilation requires a sensor mechanism that can detect a divergence in the normal relationship between delivered blood oxygen and tissue oxygen consumption.2 This hypoxic sensor mechanism must be coupled to the feedback generation of vasodilatory effectors that increase blood flow to maintain adequate tissue oxygenation. In short, hypoxic vasodilation requires hypoxia and/or pH sensing coupled to the release of a vasodilating signal. In mammalian species, the set point for hypoxic vasodilation occurs as the hemoglobin desaturates from 60% to 40%, around a partial pressure of oxygen ranging from 40 to 20 mm Hg.3 Despite the fact that the hypoxic vasodilation response was discovered almost 150 years ago, the identities of the oxygen sensor mechanism and the specific feedback vasodilator effectors remain uncertain. Although a number of mediators have been considered, including adenosine, nitric oxide (NO), ATP-sensitive potassium (KATP) channels, endothelium-derived hyperpolarizing factor (candidates include CO, H2O2, or ONOO−), and prostacyclin,2,4 the specific blockade of many of these pathways fails to completely inhibit hypoxic vasodilation.2 Article p 670 The present study published by Maher and colleagues5 in this issue of Circulation provides compelling evidence in normal human volunteers that the circulating anion salt nitrite (NO2−) may be an effector of hypoxic vasodilation. They report that nitrite potently vasodilates the venous forearm circulation under resting conditions and potently vasodilates the arteriolar circulation during experimental hypoxia, achieved by systemic breathing of 11% oxygen. Their studies indicate that the vasodilatory effect of nitrite is maximal in the deoxygenated venous …

Balloon pulmonary valvotomy as interim palliation for symptomatic young infants with tetralogy of Fallot

Objectives:To describe the case selection, technique and immediate and short-term results of balloon pulmonary valvotomy (BPV) in young infants with tetralogy of Fallot (TOF).Background:Symptomatic young infants with TOF can either undergo corrective surgery or Blalock-Taussig (BT) shunt. Corrective surgery in early infancy is associated with significant morbidity and is not a realistic option in many centers. BT shunt carries the risk of branch pulmonary artery distortion and shunt occlusion.Methods:Infants less than three months with a significant valvar pulmonary stenosis (with or without associated infundibular and annular component) and oxygen saturation ≤80% were offered BPV. The right ventricular outflow tract (RVOT) was crossed with 4F Judkin's right coronary catheter and the valve was crossed with 0.014” coronary guide wire. Serial balloon dilatations were done with over the wire coronary balloons (3-4 mm) and Mini Tyshak balloons up to a balloon annulus ratio of 2:1, depending upon the improvement in saturation and formation of annular waist.Results:Seventeen infants less than three months of age with tetralogy of Fallot (median age: 33 days, range: 10-90 days, weight: 3.47 ± 0.87 kg, pulmonary annulus Z score: -5.59 ± 1.04) including eight neonates underwent palliative BPV between May 2004 and March 2007. The mean balloon annulus ratio was 1.4 ± 0.28 and fluoroscopy time was 26.18 ± 20.2 minutes. The mean oxygen saturation increased significantly from 73 ± 7% to 90 ± 3.68% following BPV (p = 0.0001). The only major complication was RVOT perforation and pericardial tamponade in one infant. The mean follow-up period was 23 ± 12 months. Two babies developed significant desaturation requiring surgery in the six months following BPV. There was a significant increase in pulmonary annulus. The z score for the pulmonary annulus improved from -5.59 ±1.04 before BPV to - 4.31 ± 1.9 at the time of last follow-up (p = 0.018). The mean Z score of hilar right pulmonary artery (RPA) increased significantly from -1.19 ± 1.78 before BPV to 0.7 ± 0.91 after BPV (p = 0.001). The mean Z score of hilar left pulmonary artery (LPA) increased significantly from -1.28 ± 1.41 to 0.03 ± 1.29 after BPV (p = 0.005). Eight patients underwent corrective surgery.Conclusions:Balloon pulmonary valvotomy is safe and effective. It significantly improves the growth of pulmonary annulus and branch pulmonary arteries. Thus it can be considered as an interim palliative procedure for symptomatic young infants with TOF and predominant valvar pulmonary stenosis.

Clinical Characteristics of Patients with Acute Pulmonary Embolism: Data from PIOPED II

Background Selection of patients for diagnostic tests for acute pulmonary embolism requires recognition of the possibility of pulmonary embolism on the basis of the clinical characteristics. Patients in the Prospective Investigation of Pulmonary Embolism Diagnosis II had a broad spectrum of severity, which permits an evaluation of the subtle characteristics of mild pulmonary embolism and the characteristics of severe pulmonary embolism. Methods Data are from the national collaborative study, Prospective Investigation of Pulmonary Embolism Diagnosis II. Results There may be dyspnea only on exertion. The onset of dyspnea is usually, but not always, rapid. Orthopnea may occur. In patients with pulmonary embolism in the main or lobar pulmonary arteries, dyspnea or tachypnea occurred in 92%, but the largest pulmonary embolism was in the segmental pulmonary arteries in only 65%. In general, signs and symptoms were similar in elderly and younger patients, but dyspnea or tachypnea was less frequent in elderly patients with no previous cardiopulmonary disease. Dyspnea may be absent even in patients with circulatory collapse. Patients with a low-probability objective clinical assessment sometimes had pulmonary embolism, even in proximal vessels. Conclusion Symptoms may be mild, and generally recognized symptoms may be absent, particularly in patients with pulmonary embolism only in the segmental pulmonary branches, but they may be absent even with severe pulmonary embolism. A high or intermediate-probability objective clinical assessment suggests the need for diagnostic studies, but a low-probability objective clinical assessment does not exclude the diagnosis. Maintenance of a high level of suspicion is critical.

P40.15: Pulmonary underdevelopment: the clues are in mediastinal shift and pulmonary vessels

Pulmonary underdevelopment is usually first suspected in the postnatal period at chest radiography and has been classified according to the degree of bronchial development. However the pulmonary artery ipsilateral branch is usually also affected in a similar fashion. In pulmonary agenesis and aplasia the artery is absent while in hypoplasia there are variable degrees of underdevelopment. Pulmonary agenesis in which bronchus and lung are absent occurs during the embryogenic period and is usually unilateral. More than 50% of children with pulmonary agenesis have other associated congenital anomalies Pulmonary hypoplasia is characterized by the presence of both bronchi and alveoli in an underdeveloped lobe, occurs after the embryogenic period, and can be caused by factors compromising the thoracic space available for lung growth or decreased pulmonary vascular perfusion. Variable degrees of compensatory contralateral lung hypertrophy, mediastinal shift and ipsilateral diaphragm elevation are also usual findings in agenesis-hypoplasia. We have in our institution followed two cases of unilateral pulmonary underdevelopment through gestation. Both women were sent for routine scan at 25 weeks' gestation. The diagnosis was suspected by the mediastinal shift and pulmonary asymmetry. The absence in one case and hypoplasia of pulmonary artery in the other was helpful to confirm the diagnosis. There was no abnornal venous pulmonary return. As pulmonary artery is earlier and more easily seen than bronchial branches in prenatal ultrasound, especially with the aid of color Doppler, the identification of an abnormality of these vessels could be valuable information to reassure the diagnosis.

Optimal breathing protocol for dynamic contrast-enhanced MRI of solitary pulmonary nodules at 3 T

The purpose of this study was to evaluate optimal breathing maneuvers that minimize lung parenchymal movement for dynamic contrast-enhanced MRI (DCE-MRI), which requires longer scan times, beyond the limit of a single breath hold. A healthy volunteer was scanned on a 3 T MR scanner using two different breathing maneuvers. In the first, the healthy volunteer was instructed to hold his breath as much as possible and breathe in between breath holds while an image was obtained. In the second, the volunteer was instructed to breathe shallowly and freely throughout the scan. On the obtained images, the excursion of the highest point of the right diaphragm and the pulmonary vessel branches located in the four different anatomic regions of the lung were measured in two orthogonal planes. A patient with a solitary pulmonary nodule (SPN) underwent DCE-MRI utilizing a 2D spoiled gradient-echo (SPGR) sequence while the patient breathed shallowly and freely during the scan. The standard deviations of the excursion of the highest point and selected pulmonary vessels were much smaller during shallow, free breathing maneuver scans than those during breath hold maneuver scans. A dynamic perfusion-fitting curve of the SPN was obtained during the DCE-MRI using shallow free breathing. Shallow, free breathing allows smaller diaphragmatic cranial caudal and lung parenchymal displacements. Therefore, it can be useful during exams where targeting of the lesion is necessary, in studies with long scan times, such as dynamic MRI. This breathing maneuver makes it possible to analyze SPN with DCE-MRI while making use of the advantages of a higher magnetic field in conjunction.

Right Ventricular Outflow Tract Reconstruction With the Bovine Jugular Vein Graft: 5 Years’ Experience With 133 Patients

We analyzed the results in two centers of using bovine jugular vein graft for right ventricular outflow tract reconstruction. From April 1999 to July 2005, 133 children with a median age of 30.9 months (range, 4 days to 19 years) underwent graft implantation. Echocardiography was performed during follow-up and retrospectively reviewed. Nongraft-related early mortality occurred in 8 patients. Late mortality occurred in 11 patients, 2 late deaths were graft related (endocarditis). Median follow-up was 31.6 months (range, 1 to 73). Twelve patients received a new graft, because of endocarditis (3), distal pulmonary artery branch stenosis (4), graft obstruction caused by fibrosis (4), or thrombosis (1). Echocardiography Doppler studies showed good conduit function, with 92% of the patients having absent, trivial, or only mild valve regurgitation at last follow-up. A moderate degree of conduit stenosis due to external compression was observed in 2 patients. Twenty-five patients with otherwise intact conduits had hemodynamically significant distal stenosis. In most cases, the pulmonary branch stenosis was related to preoperative small pulmonary arteries and young age at operation. At 31.6 months, significant graft dilatation was observed in 4 grafts and was related to pulmonary artery branch obstruction or pulmonary hypertension. Calcification did not occur in 5 years time. Survival was 85.7%, freedom from conduit explantation was 91%, and freedom from intervention for pulmonary artery branch stenosis was 80% after 5 years. The bovine jugular vein graft is a valuable right ventricular outflow tract conduit, but younger age and small pulmonary arteries increase the risk of distal conduit stenosis.

Chronische Lungenembolie – Radiologische Bildmorphologie und Differenzialdiagnose

In chronic pulmonary embolism branches of the pulmonary arterial tree remain partially or totally occluded. This may lead to pulmonary hypertension with the development of right ventricular hypertrophy as well as structural changes of pulmonary arteries. Imaging of chronic pulmonary embolism should prove vessel occlusions (pulmonary angiography, MSCT, MRI) and reduction of regional lung perfusion (lung scanning, MSCT, MRI). According to current guidelines ventilation-perfusion lung scanning and pulmonary angiography are still recommended as the methods of choice. MSCT and MRI provide technical alternatives which are helpful in differential diagnosis versus other types of pulmonary hypertension. In spite of medical and surgical measures (in rare cases pulmonary thromboendarterectomy) the prognosis of chronic pulmonary embolism remains unfavourable.

Evaluation of Pulmonary Atresia with 64‐Slice Multidetector Computed Tomography (MDCT)

Pulmonary atresia with ventricular septal defect is a complex congenital heart disease. We report a case of a 46-year-old woman with pulmonary atresia and ventricular septal defect, surgically treated when she was 15 years old with an implant of a conduit between ascending aorta and left pulmonary branch. A 64-slice computed tomography was performed to assess the conduit patency. The contrast enhanced MDCT study showed the conduit was pervious and correctly connected with an enlarged left pulmonary artery implant of a conduit between ascending aorta and left pulmonary branch.

Radiography comparison of the pulmonary embolism

目的 评价X线胸片、超声心动图、核素肺灌注扫描、电子束CT(EBCT)、MR肺动脉造影(MRPA)、肺动脉造影(PAG)在肺动脉栓塞(PTE)诊断中的应用价值.方法 自2003年7月至2004年3月间临床确诊的PTE患者25例.常规行胸部正侧位摄片、超声心动图、EBCT肺动脉造影、核素肺灌注扫描、MRPA及PAG.PAG由2名有经验医师共同分析每一支肺动脉及其叶段分支,诊断意见作为标准并与其他方法进行对照.结果 PAG技术成功率100%,775个受检肺动脉分支中共检出556个分支受累(71.7%).25例患者中X线胸片提示诊断12例.超声心动图诊断9例,右房室增大21例,肺动脉高压18例.核素肺灌注显像结果显示500个肺段中有247个肺段受累(52.0%),与PAG对照对段的敏感度为64.66%.EBCT肺动脉造影(EBCTPA)的分析显示775个肺肺动脉分支中共有523个主干及叶段分支受累,段以上分支敏感度为94.06%;10例行MRPA的患者中8例检查成功,248各肺动脉分类中共155个分支受累(62.5%),段以上敏感度为81.29%.结论 EBCT对PTE的诊断具有较高的敏感度,X线胸片和超声心动图为必不可少的检查手段,EBCT可作为一线影像学诊断方法,核素肺灌注扫描及MRPA可作为二线诊断方法。

Effect of chemical stabilizers of hypoxia-inducible factors on early lung development

Low oxygen stimulates pulmonary vascular development and airway branching and involves hypoxia-inducible factor (HIF). HIF is stable and initiates expression of angiogenic factors under hypoxia, whereas normoxia triggers hydroxylation of the HIF-1alpha subunit by prolyl hydroxylases (PHDs) and subsequent degradation. Herein, we investigated whether chemical stabilization of HIF-1alpha under normoxic (20% O(2)) conditions would stimulate vascular growth and branching morphogenesis in early lung explants. Tie2-LacZ (endothelial LacZ marker) mice were used for visualization of the vasculature. Embryonic day 11.5 (E11.5) lung buds were dissected and cultured in 20% O(2) in the absence or presence of cobalt chloride (CoCl(2), a hypoxia mimetic), dimethyloxalylglycine (DMOG; a nonspecific inhibitor of PHDs), or desferrioxamine (DFO; an iron chelator). Vascularization was assessed by X-gal staining, and terminal buds were counted. The fine vascular network surrounding the developing lung buds seen in control explants disappeared in CoCl(2)- and DFO-treated explants. Also, epithelial branching was reduced in the explants treated with CoCl(2) and DFO. In contrast, DMOG inhibited branching but stimulated vascularization. Both DFO and DMOG increased nuclear HIF-1alpha protein levels, whereas CoCl(2) had no effect. Since HIF-1alpha induces VEGF expression, the effect of SU-5416, a potent VEGF receptor (VEGFR) blocker, on early lung development was also investigated. Inhibition of VEGFR2 signaling in explants maintained under hypoxic (2% O(2)) conditions completely abolished vascularization and slightly decreased epithelial branching. Taken together, the data suggest that DMOG stabilization of HIF-1alpha during early development leads to a hypervascular lung and that airway branching proceeds without the vasculature, albeit at a slower rate.

Surgical treatment of a pulmonary artery aneurysm

We would like to report a rare case of post-stenotic aneurysm of the pulmonary trunk and its left branch in a 51-year-old man. His cardiac disease, which was first diagnosed at the age of 4, was left untreated because of absence of symptoms and normal physical development. A CT scan, recently performed because of decrease in exercise tolerance and worsening dyspnea, showed a pulmonary artery aneurysm (52 mm x 79 mm). The echocardiography revealed a severe pulmonary commissural stenosis. Through a surgical approach the pulmonary trunk and its left branch were excised and reconstructed using a 30 mm Dacron graft; the right pulmonary branch was then reimplanted on the right side of the tube. The patient's postoperative course was uneventful. He was discharged on the seventh postoperative day and there were no adverse events or complications at 1- and 3-month follow-up.

Main pulmonary artery translocation for left pulmonary stenosis

Surgical arterioplasty for left pulmonary branch stenosis has often produced unsatisfactory results. We report a new operative technique involving the main pulmonary artery (MPA) translocation technique. This technique provided a wide patency without overstretching. Technique The operation is performed through a median sternotomy. In cases that require an additional aortopulmonary shunt, this procedure is performed through a standard left posterolateral thoracotomy. The left pulmonary artery and MPA are completely dissected to allow anastomosis without overstretching, and the stenotic portion is transected. Next, the MPA is transected and the distal stump is translocated to the distal left pulmonary artery. The anastomosis is completed with a running suture of 7-0 polypropylene. After this anastomosis, a modified Blalock‐Taussig (BT) shunt or a bidirectional Glenn (BDG) shunt is created. Results

Silver-coated endotracheal tubes: Is the bactericidal effect time limited?

Section on Pulmonary and Cardiac Assist Devices, Pulmonary and Critical Care Medicine Branch, NHLBI, NIH, Bethesda, MD (Li Bassi, Kolobow) Massachusetts General Hospital, Anesthesia and Critical Care Department, Boston, MA (Berra)

Effects of age on pulmonary perfusion heterogeneity measured by magnetic resonance imaging

Normal aging is associated with a decline in pulmonary function and efficiency of gas exchange, although the effects on the spatial distribution of pulmonary perfusion are poorly understood. We hypothesized that spatial pulmonary perfusion heterogeneity would increase with increasing age. Fifty-six healthy, nonsmoking subjects (ages 21-76 yr) underwent magnetic resonance imaging with arterial spin labeling (ASL) using a Vision 1.5-T whole body scanner (Siemens Medical Systems, Erlangen, Germany). ASL uses a magnetically tagged bolus to generate perfusion maps where signal intensity is proportional to regional pulmonary perfusion. The spatial heterogeneity of pulmonary blood flow was quantified by the relative dispersion (RD = SD/mean, a global index of heterogeneity) of signal intensity for voxels within the right lung and by the fractal dimension (D(s)). There were no significant sex differences for RD (P = 0.81) or D(s) (P = 0.43) when age was considered as a covariate. RD increased significantly with increasing age by approximately 0.1/decade until age 50-59 yr, and there was a significant positive relationship between RD and age (R = 0.48, P < 0.0005) and height (R = 0.39, P < 0.01), but not body mass index (R = 0.07, P = 0.67). Age and height combined in a multiple regression were significantly related to RD (R = 0.66, P < 0.0001). There was no significant relationship between RD and spirometry or arterial oxygen saturation. D(s) was not related to age, height, spirometry, or arterial oxygen saturation. The lack of relationship between age and D(s) argues against an intrinsic alteration in the pulmonary vascular branching with age as being responsible for the observed increase in global spatial perfusion heterogeneity measured by the RD.

A cryptogenic pulmonary embolism: Left atrial myxoma occluding an atrial septal defect

Acute pulmonary embolism represents a severe condition associated with significant morbidity and mortality. The main source of pulmonary embolism is represented by deep venous thrombosis. Less frequent causes are right-sided heart chamber clots, neoplastic emboli into the systemic veins, or the association of left atrial (LA) thrombi and atrial septal defects (ASDs). We describe a unique case of acute pulmonary embolism related to the contemporary presence of LA myxoma and ASD, with the mass behaving as an ASD occluder.

IL-6 Is Constitutively Expressed During Lung Morphogenesis and Enhances Fetal Lung Explant Branching

Previous studies have shown that chorioamnionitis, with increased IL-6, promotes fetal lung maturation and decreases the incidence of respiratory distress syndrome in premature neonates. However, the expression pattern and the effects of IL-6 on fetal lung growth mechanisms remain unknown. IL-6 expression was assessed by in situ hybridization and by real-time PCR between 14.5 and 21.5 d postconception. Normal and nitrofen-induced hypoplastic lung explants were cultured with increasing IL-6 doses or IL-6 neutralizing antibodies. Branching, cellular proliferation (Ki-67) and MAPK phosphorylation in fetal lung explants were analyzed. Pulmonary primitive epithelium expressed IL-6 constitutively throughout all gestational ages, displaying highest levels during earliest stages. In normal and hypoplastic lung explants, IL-6 neutralizing antibodies significantly reduced, whereas IL-6 supplementation induced a biphasic effect (lower doses increased, while the highest dose did not accomplish additional effect) on branching and cellular proliferation. IL-6 enhanced p38-MAPK phosphorylation without changing MEK1/2 and JNK pathways. The present study suggests a physiological role for IL-6 on pulmonary branching mechanisms most likely involving p38-MAPK intracellular signalling pathway.

High-Resolution MDCT of Pulmonary Septic Embolism: Evaluation of the Feeding Vessel Sign

The objective of this study was to use high-resolution MDCT to assess the relation of the pulmonary vasculature to septic emboli with particular attention to the feeding vessel sign. The MDCT scans of nine patients with septic emboli were retrospectively, blindly evaluated by two observers. A control group of 10 patients with documented pulmonary metastasis and pathologically proven carcinoma also were included. Transverse images, multiplanar reconstructions, and maximum intensity projections were used to analyze nodules and the pulmonary vasculature. The CT scans were obtained with 1- to 1.25-mm collimation on a 4-, 8-, or 16-MDCT scanner. The feeding vessel sign was defined as a vessel coursing directly into a nodule. The patients with septic embolism had a total of 141 nodules and 52 wedge-shaped opacities. Transverse images showed that 52 (37%) of the nodules and 11 (22%) of the wedge-shaped opacities had a vessel that appeared to enter the nodule, but multiplanar reconstructions (without IV contrast enhancement) and maximum intensity projections (with IV contrast enhancement) showed the vessels passed around the nodules. Twenty-one (15%) of the spherical nodules and seven (13%) of the wedge-shaped opacities exhibited a central vessel entering the lesion in all imaging planes. All of these vessels were traced to the left atrium on transverse images, a finding consistent with pulmonary vein branches. Similar findings were seen in pulmonary metastatic lesions. Although pulmonary septic emboli often appear to have a feeding vessel on conventional cross-sectional images, multiplanar reconstructions show that most of these vessels course around the nodule and that the others are pulmonary veins.

Encouraging results for the Contegra conduit in the problematic right ventricle–to–pulmonary artery connection

The Contegra conduit was developed for right ventricular outflow tract reconstruction. This report evaluates the Contegra conduit, with focus on certain subpopulations in which conduits are known to perform poorly (ie, patients with previous homograft conduits and infants). A retrospective review of 76 patients who had 77 Contegra conduits placed for right ventricular outflow tract reconstruction (January 2001 through August 2005) was completed. Characteristics include the following: median age of 1.6 years (range, 17 days-15.1 years), weight of 9.8 kg (range, 2.5-64.0 kg), and conduit diameter of 16 mm (range, 12-22 mm). Operations performed include right ventricular outflow tract reconstruction for pulmonary atresia-stenosis (n = 33), conduit exchange (n = 28), truncus repair (n = 7), primary conduit placement (n = 6), and the Ross procedure (n = 3). Seventy-nine percent were reoperations. There was no hospital mortality. Mean follow-up was 20 +/- 14 months. One-, 2-, and 3- year freedom from severe conduit regurgitation was 97%, 86%, and 81%, respectively, and freedom from severe conduit stenosis was 100%. Freedom from reoperation for conduit failure at 1 and 3 years is 98.3% and 93.1%, respectively. All conduit failures (n = 3) were for asymptomatic conduit pseudoaneurysms in the setting of multiple-level pulmonary branch stenoses. Survival at 3 years is 96%. Infants (n = 26) had a freedom from Contegra conduit failure at 3 years of 100%. Patients with previous homograft conduits (n = 26) had a freedom from Contegra conduit failure at 3 years of 100%. At midterm follow-up, the Contegra conduit remains a reliable, accessible, and easily implantable conduit for right ventricular outflow tract reconstruction. It appears to be the most promising conduit option for patients with previous homograft conduits and for infants.

Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve

To analyze fetal echocardiographic findings of absent pulmonary valve syndrome (APVS), its association with chromosomal and extracardiac anomalies including nuchal translucency (NT) and the outcome after diagnosis. Data of 14 fetuses with confirmed APVS retrospectively collected in two tertiary referral centers between 1998 and 2004 were analyzed. The variables examined were: reason for referral, gestational age at diagnosis and associated abnormalities, including first trimester NT thickness. Cardiac evaluation included measurement of cardiothoracic ratio, diameter of pulmonary arteries and Doppler flow in the pulmonary trunk. Information was retrieved from clinical files, recorded videotapes and stored images. Karyotyping including examination for the 22q11 deletion was performed in all cases. Mean gestational age at diagnosis was 28 weeks, with 5/14 (36%) diagnosed before 22 weeks. In 13/14 (93%) there was an associated ventricular septal defect (subaortic in 12 fetuses and inlet-type in one) and all 13 had tetralogy of Fallot. Enlargement of the central pulmonary arteries and cardiomegaly were present in all cases diagnosed after 22 weeks. Of the five fetuses in which APVS was detected before 22 weeks, four (80%) had a normal pulmonary trunk diameter, two (40%) had normal pulmonary branches and three (60%) had normal cardiac size. The arterial duct was absent in 11/14 (79%). A correlation between presence of the arterial duct and the size of the central pulmonary arteries or cardiomegaly could not be established. Increased NT was observed in 4/10 cases (40%) for which this information was available. 22q11 microdeletion was diagnosed in three fetuses (21%). There were five terminations of pregnancy, one intrauterine death, five neonatal deaths and one infant death. Of the six neonates with respiratory distress, only one (17%) survived and of the eight babies in whom there was an intention to treat, two survived (25%). APVS can be accurately diagnosed by fetal echocardiography but screening ultrasound in the mid-second trimester is likely to have a low detection rate, probably due to the incomplete expression of the disease at this point. Many fetuses with APVS have an increased NT in the first trimester and this may help an earlier recognition of the defect. The most common associated karyotype anomaly is 22q11 microdeletion. Enlargement of the central pulmonary arteries is mainly related to the gestational age at diagnosis. Our results confirm that the outlook for these patients is extremely poor.