Major Aortopulmonary Collateral Arteries Research Articles

An Overview of Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare and complex congenital cardiac lesion that has historically carried a poor prognosis. With advancements in surgical management, we have seen an improvement in the outcomes for children affected by this disease. However, this population continues to present challenges due to the complex anatomy and physiology associated with PA/VSD/MAPCA. This summary of material presented during one of the nursing sessions of the 2014 Meeting of the Pediatric Cardiac Intensive Care Society provides an overview for those in cardiac intensive care units who do not have a large experience with this lesion. We will review the anatomy, physiology, surgical approach, postoperative management strategies, and cardiac catheter intervention options for PA/VSD/MAPCAs. We will also discuss recent innovations that may lead to continued improvement in outcomes for this challenging patient population.

Pulmonary Arterial Hypertension Associated With Tetralogy of Fallot

Pulmonary arterial hypertension (PAH) is a common postoperative complication in patients with congenital heart disease (CHD). Although the recent clinical classification of pulmonary hypertension divided PAH associated with CHD (PAH-CHD) into several subclasses, the anatomical and hemodynamic features of postoperative PAH-CHD vary enormously. Therefore, it is still difficult to obtain clinical evidence supporting the indication of pulmonary vasodilators for PAH-CHD. We often encounter patients with PAH occurring after surgical treatment of tetralogy of Fallot (TOF), especially patients with major aortopulmonary collateral arteries (MAPCAs). PAH might be caused by pulmonary agenesis, hypoplasia and/or thrombosis, inadequate closure of the ventricular septal defect, relief of the pulmonic stenosis, or an excessively large prior systemic-to-pulmonary shunt. Moreover, patients with TOF and MAPCAs who are diagnosed as inoperable because of the presence of PAH show similar hemodynamic and clinical features to patients with Eisenmenger syndrome. The MAPCAs in these patients usually show hypoplastic and abnormal arborization. Based on our experience, we believe that PAH-targeted therapies are effective in some patients with PAH occurring after surgical treatment of TOF and MAPCAs, especially as an adjunct to percutaneous pulmonary angioplasty. To help classify patients with PAH associated with TOF, especially with MAPCAs, we propose several new subclassifications: "PAH due to hypoplastic pulmonary arterial beds", "PAH due to abnormal pulmonary arborization", or "segmental PAH associated with CHD." A multicenter registry of patients using a unified protocol is essential to explore the indications and efficacy of pulmonary vasodilators for postoperative PAH-CHD.

乳児期後期Rastelli型手術における16 mm自作3弁付きePTFE導管の中期遠隔成績

Background: In small children, conduits for right ventricular out ow tract reconstruction are limited because of patient size and conduit resources, particularly in Japan. Methods: Since 2007, we have performed the Rastelli-type procedure using 16-mm hand-made trilea et expanded polytetra uoroethylene (ePTFE) conduits in nine approximately 1-year-old children. e median age and body weight at surgery was 17.3 months (range: 10~22 months) and 8.9 kg (range: 6.8~11 kg), respectively. e patient diagnoses were double-outlet right ventricle (DORV) with pulmonary stenosis (PS) in four, pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCA) in four, and PA/VSD in one patient. All patients had undergone previous surgical procedures, including modi ed Blalock shunt in ve and unifocalization of MAPCA and modi ed Blalock shunt in four patients. Conduit function, re-intervention, and right ventricular function were retrospectively investigated. Results: ere was no early or late mortality. During the median follow-up of 45 months (range: 18~79 months), balloon dilation for conduit stenosis was performed in two patients at 47 and 51 months post surgery, respectively. One of these patients required conduit replacement 27 months a er balloon dilation. Excluding this patient, the most recent echocardiographic pressure gradients of the conduits were 0~20 mmHg in four, 21~40 mmHg in four, and no patient had conduit stenosis with the pressure gradients over 41 mmHg. Conduit regurgitation was trivial in four, mild in two, and moderate in two patients. Conclusion: e Rastelli-type procedure using 16-mm hand-made trilea et ePTFE conduits in approximately 1-year-old infants showed acceptable mid-term results. Conduit stenosis gradually developed over 5~6 years. Balloon dilation for conduit stenosis has the potential to delay conduit replacement without increasing conduit regurgitation.

Pulmonary Atresia and Ventricular Septal Defect in a Thirty-Six Year Old Woman

Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare form of congenital heart disease. The blood supply to the pulmonary arteries is provided by a patent arterial duct or by major aorto-pulmonary collateral arteries (MAPCAs). Here we present a thirty six years old Syrian war victim who was referred to our clinic with complaints of dyspnea on exertion and syncope. When questioned she admitted that she was having these complaints since her childhood and had a disease that she was offered cardiac catheterization and operation which she refused. Echocardiographic and computed tomography angiography findings revelaed pulmonary atresia with ventricular septal defect and aortopulmonary collateral arteries provided the blood flow to pulmonary arteries. Most patients without surgery do not live more than three decades. The presented case is one of those surviving more than three decades without surgery.

A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect

We evaluated our clinical experiences on rehabilitation of native pulmonary arteries (PAs) with a central shunt using an expanded polytetrafluoroethylene (ePTFE) tube graft in management of pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs) with diminutive PAs. From March 1993 to June 2013, a total of 28 consecutive patients underwent surgery for pulmonary atresia with VSD and MAPCAs. We identified 15 patients who underwent a central shunt procedure using an ePTFE tube graft. Median age and weight at the initial operation were 1.91 months and 5.22 kg, respectively. Ligation of MAPCAs was performed in 3 patients concomitantly. The techniques used were a U-shaped central shunt in 13 patients, and a side-to-side aorto-ePTFE tube graft anastomosis in 2 patients. There was no surgical mortality, and no shunt occlusion. The mean follow-up duration was 70.7 ± 67.1 months. Complete repair was achieved in 13 patients (13 of 14 of the follow-up patients; 92.9%), and the remaining patient is waiting for complete repair. The median age at complete repair was 19.4 months. The mean PA index before the initial central shunt procedure was 22.7 ± 13.2 mm(2)/m(2), which increased to 149.4 ± 86.6 mm(2)/m(2) at the final recordings before shunt division, and 185.9 ± 84.0 mm(2)/m(2) before complete repair. There were 2 late mortality cases. The overall survival rates were 92.9%, 82.5%, and 82.5%, at 1, 5, and 10 years, respectively. A central shunt, using an ePTFE tube graft, is an effective initial palliative procedure for rehabilitation of the diminutive PAs in patients with pulmonary atresia with VSD and MAPCAs.

OP05.01: Prenatal diagnosis of major aortopulmonary collateral arteries in a series of 17 fetuses with pulmonary atresia with ventricular septal defect

<scp>OP</scp>05.01: Prenatal diagnosis of major aortopulmonary collateral arteries in a series of 17 fetuses with pulmonary atresia with ventricular septal defect

Variations of pulmonary arteries and other associated defects in Tetralogy of Fallot.

BackgroundThe objective of study was to determine pulmonary artery variations and other associated cardiac defects in patients with Tetralogy of Fallot. This cross-sectional, descriptive study was carried out at The Children's Hospital and the Institute of Child Health, Lahore, from January 2006 to December 2012. All patients with Tetralogy of Fallot, who underwent cardiac catheterization during this period, were included. Standard cine-angiograms were done to record the pulmonary artery sizes and associated cardiac defects.DescriptionA total of 576 patients with Tetralogy of Fallot were catheterized. Pulmonary Artery abnormalities were present in 109 (18.92%) patients. The commonest abnormality was isolated Left Pulmonary Artery stenosis (n = 60, 10.4%) followed by supra-valvular stenosis (n = 9, 1.6%). Left Pulmonary Artery was absent in seven patients(1.2%), while 1 patient (0.2%) had both absent right and left Pulmonary Arteries with segmental branch pulmonary arteries originating directly from Main Pulmonary Artery. Associated cardiac lesions included right aortic arch in 72 (12.5%), additional muscular Ventricular Septal Defect in 31 (5.4%), Patent Ductus Arteriosus in 31 (5.4%), bilateral Superior Vena Cava 36(6.2%), Atrial Septal Defect 4(0.7%) and Major Aortopulmonary Collateral Arteries in 75(13%) patients. Significant coronary artery abnormalities were present in 28(4.9%) children.ConclusionPulmonary artery abnormalities were present in 18.92% of patients with Tetralogy of Fallot. Isolated Left Pulmonary Artery origin stenosis was the most common abnormality. Significant associated cardiac lesions including Patent Ductus Arteriosus , additional muscular Ventricular Septal Defect, coronary artery abnormalities, bilateral Superior Vena Cava, Atrial Septal Defect and Major Aortopulmonary Collateral Arteries were present in one-third of the patients.

Assessment of Vascular Growth after Surgical and Transcatheter Interventions (TCI) in Patients with Pulmonary Atresia (PA), Ventricular Septal Defect (VSD), and Major Aortopulmonary Collateral Arteries (MAPCAs)

Assessment of Vascular Growth after Surgical and Transcatheter Interventions (TCI) in Patients with Pulmonary Atresia (PA), Ventricular Septal Defect (VSD), and Major Aortopulmonary Collateral Arteries (MAPCAs)

Development of major aorto-pulmonary collateral arteries in vegf120/120 isoform mouse embryos with tetralogy of fallot.

The degree of right ventricular outflow tract obstruction, pulmonary stenosis (PS) and the development of major aorto-pulmonary collateral arteries (MAPCAs) in patients with tetralogy of Fallot (TOF) is related to clinical outcome. Vegf120/120 mutant mouse embryos develop TOF with various degrees of PS, comparable to humans. We aimed to study the ontogeny of the development of MAPCAs in this mouse model. The development of the right ventricular outflow tract, pulmonary arteries, and ductus arteriosus (DA) and formation of MAPCAs were studied in both wild type as well as Vegf120/120 mice from embryonic day 10.5 until day 19.5. Of the 49 Vegf120/120 embryos, 35 embryos (71%) had ventral displacement of the outflow tract and a subaortic ventricular septal defect. A time-related development in severity of PS to pulmonary atresia (PA) was observed. From embryonic day 12.5, hypoplasia of the DA was seen in 13 (37%) and absent DA in 12 (37%) of these embryos. The 3 (6%) embryos with PA and absent DA developed MAPCAs, after day 15.5. In all, the MAPCAs arose from both subclavian arteries, running posterior in the thoracic cavity, along the vagal nerve. The MAPCAs connected the pulmonary arteries at the site of the hilus. A time-related development of PS to PA can lead, in combination with absent DA, to the development of MAPCAs later in embryonic life as an alternative route for pulmonary perfusion in this mouse model. This finding contributes to a better understanding of the consecutive morphological changes in the development toward MAPCAs in humans.

Risk Factors Associated with Prolonged Mechanical Ventilation after Corrective Surgery for Tetralogy of Fallot.

This study examined early postoperative results to identify perioperative factors that are associated with prolonged mechanical ventilation (PMV) in tetralogy of Fallot (TOF) patients undergoing corrective surgery. We retrospectively examined the role of perioperative variables in determining the period of mechanical ventilatory support in TOF patients undergoing corrective surgery. A total of 821 patients were included in the study. The cohort was divided into a PMV group that included patients with >90th percentile for duration of mechanical ventilation and a non-PMV group which included all other patients. Non-PMV group consisted of 751 patients (454 males, 297 females; median age 12 months, interquartile range 8-19 months; mean weight 9.60 ± 2.98 kg). PMV group consisted of 70 patients (51 males, 19 females; median age 8 months, interquartile range 6.75-13 months; mean weight 8.64 ± 1.95 kg). No patients died in the non-PMV group compared with two deaths due to acute respiratory distress syndrome in the PMV group. Univariate risk factors for PMV included age, weight, left ventricular end-diastolic volume index (LVEDVI), McGoon ratio, Nakata index, previous palliative operations, cardiopulmonary bypass (CPB) time, aortic cross-clamp (ACC) time, preoperative major aortopulmonary collateral arteries (MAPCAs) occlusion by coils in hybrid procedure, postoperative right ventricular/left ventricular systolic pressure ratio, central venous pressure (CVP), left atrial pressure (LAP), endotracheal reintubation, vasoactive-inotropic score (VIS), renal replacement therapy, and early-onset ventilator-associated pneumonia (VAP). In a multivariable model, age, LVEDVI, McGoon ratio, Nakata index, previous palliative operations, CPB time, blood returning to left atrium during surgery as a surrogate marker for significant aortopulmonary collateral presence, and early-onset VAP were the independent risk factors for PMV. The risk factors for PMV were age, LVEDVI, McGoon ratio, Nakata index, previous palliative operations, CPB time, VIS, LAP, blood returning to left atrium during surgery, and early-onset VAP.

Tetralogy of Fallot: aorto-pulmonary collaterals and pulmonary arteries have distinctly different transcriptomes.

Tetralogy of Fallot patients with pulmonary atresia (TOF/PA) present a pulmonary blood supply directly from aortic collateral arteries. Major aorto-pulmonary collateral arteries (MAPCAs) present substantial clinical and surgical management challenges. Surgical operations to reestablish and promote further development of a pulmonary arterial connection preferentially utilize MAPCAs for reconstruction of central pulmonary arteries. However, the propensity of some MAPCAs to develop stenosis rather than growth may impair the response to reconstructions. Probe sets prepared from MAPCAs, PA, and aorta mRNA were used to interrogate human genome microarrays. We compared expression differences between pairs of the three vessels to determine whether MAPCAs display distinct expression patterns. Functional clustering analysis identified differences in gene expression, which were further analyzed by gene ontology classification. A subset of highly regulated genes was validated using quantitative PCR. Expression differences among vessel types were observed for multiple gene classes. Of note, we observed that MAPCAs differentially express several genes at much higher levels than either PA or aorta. MAPCAs differ from PA or aorta by significantly altered levels in gene expression, suggesting a transcriptional basis for their physiology that will guide a further understanding of the pathobiology of MAPCAs and TOF.

Giant major aortopulmonary collateral artery: A rare cause of heart murmur in newborns

s p c t a s t e P Major aortopulmonary collateral arteries (MAPCAs) are anomalous vessels that develop from the aorta or its main branches and supply the pulmonary parenchyma with oxygenated blood. They are usually associated with congenital heart disease and are rare in isolation. We report the case of a newborn girl, full term, with no relevant history, in whom a III/VI murmur was detected on the left sternal border on the first day of life. At seven days a remote cardiology consultation raised the suspicion of right inferior pulmonary vein stenosis despite normal pulmonary artery systolic pressure. Echocardiography in the Hospital Pediatrico Carmona da Mota showed ‘‘an anomalous vessel draining into the right inferior pulmonary vein’’. At one month of age computed tomography angiography revealed a single MAPCA emerging from the celiac trunk to

Outcomes of patients who underwent staged repair for pulmonary atresia with ventricular septal defects and major aorto-pulmonary collateral arteries at Southampton University Hospital, United Kingdom

Objective: To assess the long-term outcomes of multistage surgical approach in 18 surviving patients in areas of mortality, requirement for postoperative interventional cardiac catheterization, saturations on air, right ventricular (RV) pressures in relation to the systemic pressures, RV and left ventricular (LV) functions and the adequacy of pulmonary arborisation. Method: Data of all surviving patients who underwent complete repair at Southampton University Hospital were reviewed. Data were obtained using case notes and computer based patient records. No data were gathered by direct patient contacts. LV and RV ejection fractions were measured by cardiac magnetic resonance imaging. Pulmonary arborisation patterns were assessed using cardiac catheter data. Results: A total of 26 patients had undergone complete repair. There were 8 (30.8%) deaths and the 18 survivors constituted the study group. Eleven (61.1%) had undergone three or more surgeries to attain complete repair. Fifteen (83.3%) maintained resting saturations above 80% after surgery. Nine (50%) had undergone three or more catheter interventions after complete repair. Eleven (61.1%) had undergone one or more pulmonary ballooning. Nine (50%) had undergone pulmonary artery (PA) stenting. Four (22.2%) had a RV ejection fraction (EF) 50% of LV pressures. Certain zones of the lungs were significantly under-filled on complete repair. Conclusions : Children with pulmonary atresia, ventricular septal defect and major aorto-pulmonary collaterals can be treated using a staged repair. There is a high mortality (31%) with average functional results. Sri Lanka Journal of Child Health , 2014; 43 (2): 84-87 (Key words: Pulmonary atresia; ventricular septal defect; major aorto-pulmonary collaterals) DOI: http://dx.doi.org/10.4038/sljch.v43i2.7008

The Fuwai hospital experience with patients presenting late with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary arteries

The reported experience from the team of Fuwai Hospital with rehabilitation procedures of the pulmonary arteries performed beyond 6 months of age in patients born with pulmonary atresia, ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs) shed new light on the potential of these techniques to achieve repair when these patients are diagnosed later in life [1]. Their experience should be followed closely because their access to a very large population is likely to make them acquire expertise in this field more rapidly than anywhere else: in a period of only 3 years they treated close to 40 of these older patients, more than any other centre in the world could hope to see. As they adequately emphasized, patients presenting after 6 months of age are a selected group of patients promised to a better survival than their younger counterparts, a fact that we also noticed in our previous experience. The authors demonstrated that, as patients with this condition grow older, an increasing proportion of collateral arteries become stenotic, likely causing the early death of some of the patients at the worse end of the spectrum of the disease. Some of the patients studied presented signs of severity of the disease: close to a third of them had no central pulmonary arteries. Others seemed to have a more favourable condition with half presenting with a persistent ductus, a characteristic usually absent in patients with lung circulation exclusively dependent on collateral flow. The quality of their results is undoubtedly remarkable. In a relatively short period, up to 80% of the patients could hope to undergo final repair with no mortality directly related to the procedures or the condition. This undoubtedly constitutes an argument demonstrating that rehabilitation procedures of the native pulmonary arteries are effective in this age group. The authors adopted an aggressive approach towards the occlusion of the collaterals arteries before or at the same time as the surgeries. All MAPCAs not bearing stenosis were occluded if they brought dual blood supply and if their occlusion did not cause significant desaturation. This aggressive policy has some benefits. It avoids the competition of flow between the collateral arteries and the forward flow in the native vessels. We have noticed in our past experience that this flow competition can impede the growth of the native vessels. By decreasing the pulmonary artery pressures, it also undoubtedly helped to reduce right ventricular pressures after completion of the repair. We have so far been reluctant to adopt such a systematic policy in Melbourne in order to avoid any compromise to their pulmonary blood supply. Surgeons are divided between partisans of unifocalization and those who support rehabilitation of the native pulmonary vessels [2, 3]. The present work demonstrates clearly the feasibility of re

Role of 64-slice multidetector computed tomography in the diagnosis of abnormal vascular connections in congenital heart disease

The aim of this study was to evaluate the role of 64-slice multidetector computed tomography in the diagnosis of abnormal systemic and pulmonary vascular connections and the associated congenital anomalies in comparison to echocardiography. The study enrolled 100 consecutive patients with the provisional diagnosis of congenital heart disease referred for further evaluation by MDCT. Low dose protocol was used for imaging. ECG-gating was used only when coronary anatomy needed to be defined. Ninety-one abnormal vascular connections were found in 73 cases. Abnormal venous connections were further classified into 19 anomalous pulmonary venous connections and 13 anomalous systemic venous connections. Abnormal arterial connections include systemic-systemic connections: 8 coronary artery fistulas, 7 cases of systemic collaterals in aortic coarctation and 2 vascular rings, as well as systemic-pulmonary connections: 38 patent ductus arteriosus (PDA), 4 major aortopulmonary collateral arteries (MAPCAs) and one coronary artery to pulmonary artery fistula. There was significant agreement between echocardiography and MDCT in the diagnosis of partial anomalous pulmonary venous connections (PAPVC) and total anomalous pulmonary venous connections (TAPVC) but not in determining the site drainage, in which MDCT was more accurate. In comparison to MDCT, echocardiography had a sensitivity and specificity of 90.9–95.5% respectively in the diagnosis of PAPVC and 100% and 98.9% in the diagnosis of TAPVC, while only a sensitivity of 45.5% in determining the site of drainage in PAPVC and 75% in TAPVC. However, echocardiography showed all intracardiac defects including that missed by MDCT. The majority of PAPVC were right-sided anomalous veins. Most TAPVC were supracardiac and half were isolated TAPVC. All anomalous systemic venous connections were incidentally discovered by MDCT and were not detected by echocardiography. The left superior vena cava was a component of a duplicated SVC in all cases. The majority of persistent azygos venous system cases were associated with complex CHD. MDCT clearly showed the origin, course and termination of coronary artery fistulas. Echocardiography showed the intracardiac defects in all cases, including that missed by MDCT. Three fistulas originated from the right coronary artery, five from the left coronary artery and one fistula originated from both the right coronary artery and the left circumflex. All coronary-cameral fistulas drained into right cardiac chambers. Less than half were associated with other CHD. Systemic collaterals in coarctation were clearly demonstrated by MDCT, none of which could be visualized by echocardiography. MDCT was not indicated for the diagnosis of PDA but for the associated CHD. Furthermore, MDCT clearly showed MAPCAs with echocardiography being able to detect one case. MDCT provides complete and accurate visualization of extracardiac vasculature but is less reliable for intracardiac defects. It can be used safely in neonates with complex congenital heart disease. It has advantages of non-invasiveness, fast acquisition, high spatial and temporal resolution and three-dimensional reconstruction.

Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance

Children with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) are at risk for reperfusion pulmonary edema (RPE) after unifocalization procedures to reconstruct the central pulmonary arteries. The purpose of this study was to determine the incidence of RPE, describe the clinical course of patients with RPE, and explore the mechanism of RPE in this population by measuring plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation. Levels of plasma receptor for advanced glycation end products (RAGE), intercellular adhesion molecule 1 (ICAM-1), and interleukin 6 (IL-6) were measured at baseline and postoperative day (POD) 0, 1, and 2 after unifocalization. A pediatric radiologist reviewed chest radiographs from the same time points and scored each lung segment for the degree of pulmonary edema. A pediatric interventional cardiologist reviewed the preoperative angiograms for each patient and determined the degree of stenosis for each aortopulmonary collateral vessel. RPE was defined as localized pulmonary edema with a pulmonary edema score of at least 2 occurring in the lung segment demonstrating the greatest degree of angiographic stenosis within the first 48 hours after surgery and with resolution by discharge. Thirty-five patients who underwent 37 unifocalization procedures were enrolled, and 32 patients were included in the analysis. Of these, 16 of 32 (50%) demonstrated evidence of RPE based on our defined criteria. There was no significant difference in RAGE (P=.60), ICAM-1 (P=.34), or IL-6 (P=.31) levels between those with and without RPE at any time point. The mean duration of mechanical ventilation in patients with RPE versus those without was not significantly different (5.1±4.2 vs 5.6±4.5 days, respectively; P=.57). Fifty percent of children with TOF/PA/MAPCAs undergoing unifocalization surgery developed RPE. Levels of plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation were not increased in patients with RPE compared with those without RPE. The presence of RPE did not affect the duration of respiratory failure and mechanical ventilation. The process of RPE is clinically self-limited and seems unlikely to be associated with vascular changes.

Growth of Diminutive Central Pulmonary Arteries After Right Ventricle to Pulmonary Artery Homograft Implantation

The management of tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries is controversial because of the wide variability of pulmonary artery (PA) and major aortopulmonary collateral arteries morphology. Several different staged strategies have been used to promote growth of diminutive PA branches. We have preferred a right ventricular (RV)-PA homograft for symmetrical growth of the central PA branches. In this study we evaluated the success of this strategy. Between 2006 and 2012, 23 patients with pulmonary atresia and diminutive PAs underwent RV-PA homograft implantation. Median age was 2 months (range, 4 days to 18 months), and median body weight was 5.1 kg (range, 1.7 to 8.5 kg). The type of homograft was aortic in 8, pulmonary in 6, and femoral vein in 9. The mean diameter of the homograft was 10.5 mm (range, 6 to 16 mm). All procedures were performed on cardiopulmonary bypass. The PA diameter was measured at the time of the operation and subsequent catheterization. The median size of the branch PA was 2.1 mm. In the 18 patients who had serial assessment of PA size, the right PA increased by 307% ± 184%, the left PA increased by 283% ± 139%, and the Nakata index increased from 28.8 ± 20.1 mm(2)/m(2) to 253 ± 96 mm(2)/m(2) during a median period of 347 days (range, 44 to 1,520 days). The PA growth ratio (PA growth in mm/mo) was similar between the right PA (0.42 ± 0.46 mm/mo) and the left PA (0.43 ± 0.47 mm/mo). There was no acute conduit failure. Seventeen patients required 28 percutaneous interventionsfor embolization of an aortopulmonary collateral or stenosis of the conduit or PA. There were no hospital deaths. Three patients died late after other procedures during a mean follow-up of 44.7 months. Twenty patients (87%) have undergone complete repair to date. RV-PA homograft implantation can be performed in neonates and infants with minimal risk of acute occlusion. The RV-PA homograft promotes rapid and balanced growth of central pulmonary arteries leading to complete repair in most patients.

Airway Complications after Single-Stage Unifocalization for Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries

Objective: We analyze the incidence of postoperative severe airflow limitation after single-stage unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/ MAPCAs) and comment on the treatment performed. Methods: From 1994 until 2012, 106 patients with diagnosis of PA, VSD, MAPCAs underwent surgical treatment. Four of them (3.8%) developed in the postoperative course severe airflow complication. Chromosome 22q11 deletion was present in three of them. Median age at the time of unifocalization was 6.2 months (range 21 days – 11 months). Results: The first developed malacia and compression of the left bronchus from the distal RV-PA conduit treated with external bronchial stenting with two incomplete costal cartilage rings. The second patient developed recurrent esophagus-left bronchus fistula treated with multiple surgical esophageal and bronchus reconstruction. The third child presented bilateral bronchial malacia treated with bilateral stenting and after with surgical elongation of the neo-left pulmonary artery to avoid external compression. The last developed bilateral bronchomalacia treated with bilateral bronchial stenting and after with RV-PA conduit replacement and endobronchial stenting calibration Conclusion: Particular categories of patients (22q11 chromosome deletion, neonates/infants, patients with dominant/exclusive collaterals,) may be more predisposed to develop airway limitation. The treatment of the lesion should be individualized according to the supposed pathogenic mechanism. We suggest endoluminal treatment in absence of compression by vascular structures while surgery was used in proven case of extrinsic compression.

The essential echocardiographic features of tetralogy of Fallot

This presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

Diagnostic accuracy of CT angiography in infants with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries

BackgroundCT angiography (CTA) is increasingly used for the evaluation of congenital heart disease. ObjectiveThe aim was to determine the diagnostic accuracy of CTA in infants with tetralogy of Fallot with pulmonary atresia (ToF-PA) and major aortopulmonary collateral arteries (MAPCAs). MethodsWe retrospectively evaluated 18 consecutive patients (7 girls; median age, 6 days; range, 1–334 days) with ToF-PA and MAPCAs. Findings on CTA were compared with diagnostic catheterization (n = 16) or intraoperative findings (n = 2) for the number of MAPCAs, their diameter, origin, and supplied lung lobes and for the presence and diameter of central pulmonary arteries. Spearman correlation coefficient was calculated to assess the correlation between diameter measurements on CTA and catheterization. CTA dose-length product and catheterization dose-area product were recorded, and effective radiation doses were calculated with the use of age-specific conversion factors. ResultsAgreement was found between CTA and catheterization or intraoperative findings for the number of MAPCAs, their origin, and supplied lung lobes in all cases. In 11 of 13 patients, CTA accurately demonstrated central pulmonary arteries. A good correlation was found between diameter measurements on CTA and catheterization for MAPCAs (r = 0.83) and central pulmonary arteries (r = 0.82). Median effective doses were 0.9 mSv for CTA and 14.4 mSv for catheterization (P < .001). ConclusionCTA is accurate in the preoperative evaluation of infants with ToF-PA and MAPCAs and is associated with a substantially lower radiation dose than catheterization. Preoperative diagnostic catheterization, therefore, may only be necessary in select patients with small MAPCAs in whom the precise assessment of central pulmonary arteries is required for surgical planning.