A. Seale

Total Anomalous Pulmonary Venous Connection: Morphology and Outcome From an International Population-Based Study

Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival. # Clinical Perspective {#article-title-32}

Z-scores of the fetal aortic isthmus and duct: An aid to assessing arch hypoplasia

Prenatal diagnosis of isolated coarctation of the aorta suffers from high false positive and false negative rates. The aim of our study was to develop Z‐scores for the aortic isthmus in normal fetuses as a reference for fetuses with suspected coarctation.

Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study

Objective: To describe clinical features, morphology, management and outcome of pulmonary vein stenosis (PVS) in childhood. Design and setting: Retrospective international collaborative study involving 19 paediatric cardiology centres in the UK, Ireland and Sweden. Patients: Cases of PVS presenting between 1 January 1995 and 31 December 2004 were identified. Cases where pulmonary veins connected to a morphological left atrium were included. Functionally univentricular hearts and total anomalous pulmonary venous connection were excluded. All available data and imaging were reviewed. Results: 58 cases were identified. In 22 cases (38%) there was premature delivery. 46 (79%) had associated cardiac lesions; 16 (28%) had undergone previous cardiac surgery before PVS diagnosis. 16 children (28%) had a syndrome or significant extracardiac abnormality. 36 presented with unilateral disease of which 86% was on the left. Where there was adequate sequential imaging, disease progression was shown with discrete stenosis leading to diffusely small pulmonary veins. Collateral vessels often developed. 13 patients had no intervention. Initial intervention was by catheter in 17 and surgery in 28. Overall 3-year survival was 49% (95% CI 35% to 63%) with patients undergoing initial surgical intervention having greater freedom from death or re-intervention (hazard ratio 0.44, 95% CI 0.2 to 0.99, p = 0.023). Conclusions: PVS is a complex disease of uncertain cause and frequently associated with prematurity. Early intervention may be indicated to deter irreversible secondary changes.

Total anomalous pulmonary venous connection: impact of prenatal diagnosis

To investigate whether prenatal screening is effective in the detection of total anomalous pulmonary venous connection (TAPVC) and to identify common prenatal features.

Sonographic predictors of surgery in fetal coarctation of the aorta

Isolated fetal coarctation of the aorta (CoA) has high false‐positive diagnostic rates by cardiologists in tertiary centers. Isthmal diameter Z‐scores (I), ratio of isthmus to duct diameters (I:D), and visualization of CoA shelf (Shelf) and isthmal flow disturbance (Flow) distinguish hypoplastic from normal aortic arches in retrospective studies, but their ability to predict a need for perinatal surgery is unknown. The aim of this study was to determine whether these four sonographic features could differentiate prenatally cases which would require neonatal surgery in a prospective cohort diagnosed with CoA by a cardiologist.

Relationship between orifices of pulmonary and coronary arteries in common arterial trunk

OBJECTIVE Variability in pulmonary arterial and coronary arterial origins in common arterial trunk has been investigated previously but only as separate entities. We hypothesise that combinations of relationships between the two arterial structures have important clinical implications. METHODS We identified pulmonary arterial and coronary arterial origins in 56 heart specimens. The orifices were plotted according to the location on the circumference of the common trunk and distance from the level of the sinutubular junction. RESULTS Pulmonary orifice was sinusal when the lowest margin of the orifice was below the sinutubular junction (n=12, 21%). It was defined as low when located </=2mm above the sinutubular junction (n=11, 20%). Pulmonary origin >2mm above the sinutubular junction was designated as normal (n=33, 59%). Circumferentially, there was a distinct predilection for sinusal origin to be located within the left-anterior segment of the common trunk, as opposed to low and normal origins that almost always resided within the left-posterior segment. Furthermore, hearts with sinusal origin (75%; 9 hearts out of 12) had significantly higher prevalence of proximity (defined as a distance of </=2mm) between pulmonary and coronary orifices than those with low origin (27%; 3 hearts out of 11) and normal origin (3%; 1 heart out of 33) (p=0.039 and p<0.001, respectively). CONCLUSIONS Owing to its unique location, frequently close to a coronary orifice, hearts with sinusal origin warrant special attention in both diagnostic and surgical management. At the same time, however, its peculiar pulmonary arrangement may facilitate direct right ventricular-pulmonary connection and dispense with the need for augmentation with an external conduit that inevitably will be outgrown by the patient.

Prenatal identification of the pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia.

OBJECTIVE To define the patterns of flow of blood to the lungs in fetuses with tetralogy of Fallot and pulmonary atresia. BACKGROUND In this condition, supply of blood to the lungs is provided via an arterial duct or systemic-to-pulmonary collateral arteries, or very rarely through other conduits such as coronary arterial fistulas or an aortopulmonary window. The intrapericardial pulmonary arteries vary in size, and may be absent. These variables influence the prognosis and management. METHODS We carried out a retrospective review of cases from a tertiary service for fetal cardiology, identifying all cases of tetralogy of Fallot with pulmonary atresia diagnosed antenatally between January, 1997, and April, 2006. We established pre- and postnatal outcomes, and compared the prenatal diagnosis with postnatal or autopsy findings. RESULTS Of 6587 fetuses scanned during this period, 11 were diagnosed as having tetralogy of Fallot with pulmonary atresia and no other cardiac defect. In 5, arterial flow to the lungs was via an arterial duct, and in the other 6, the main identified source of flow was systemic-to-pulmonary collateral arteries. Of the latter 6 pregnancies, 4 were terminated, along with 3 of the 5 with ductal supply. The presence of systemic-to-pulmonary collateral arteries was confirmed at postmortem examination in 3 instances, and in the two delivered neonates, in neither of whom was an infusion of prostaglandin commenced. CONCLUSION The patterns of pulmonary flow can be identified prenatally in the setting of tetralogy with pulmonary atresia. Supply through systemic-to-pulmonary collateral arteries impacts on counselling, introducing uncertainty regarding postnatal surgical management.

Common Arterial Trunk With Atrioventricular Septal Defect: New Observations Pertinent to Repair

BACKGROUND The coexistence of abnormalities in both atrioventricular and ventriculoarterial junctions occasionally represents a formidable challenge to the surgeon. The association of common arterial trunk with atrioventricular septal defect is such an example. To date, only two reports have described successful operative outcome. This paucity of success might reflect the anatomical complexity that could prevent favorable results. METHODS We reviewed six specimens with common arterial trunk and atrioventricular septal defect, focusing on how to establish a nonobstructed connection between the left ventricle and the truncal valve. RESULTS In all cases, the common trunk arose exclusively from the right ventricle, and the only exit from the left ventricle was the ventricular component of the septal deficiency. In particular, the preferential route was limited to a space below the superior bridging leaflet that did not have any tendinous cords inserting onto the ventricular crest, in contrast to the inferior bridging leaflets that were always tethered to the crest with many short cords. Accordingly, the size of potential left ventricular outflow depended on the shape of the anterosuperior margin of the ventricular crest below the superior bridging leaflet. The potential outflow was narrower than the truncal valvar area in all hearts but one having extensive anterosuperior excavation of the ventricular crest, suggesting the necessity of septal enlargement had anatomical repair been attempted during life. CONCLUSIONS Owing to the unique ventriculoarterial connection, the surgeon, considering anatomical repair, needs to pay attention to the anterosuperior margin of the ventricular scoop, which determines the adequacy of left ventricular outflow size.

Total Anomalous Pulmonary Venous Connection to the Supradiaphragmatic Inferior Vena Cava

Total anomalous pulmonary venous connection to the inferior vena cava is a rare form of total anomalous pulmonary venous connection infrequently described in the literature. We report two cases where the pulmonary venous connection was to the supradiaphragmatic portion of the inferior vena cava. In both patients, preoperative echocardiography findings were misleading, which suggested a cardiac type of total anomalous pulmonary venous connection.

Total Anomalous Pulmonary Venous Connection

Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.Background— Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results— We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Conclusions— Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival. # Clinical Perspective {#article-title-32}