Objective

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

PVS is a complex disease of uncertain cause and frequently associated with prematurity. Early intervention may be indicated to deter irreversible secondary changes.

on behalf of the British Congenital Cardiac AssociationBackground-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. Key Words: follow-up studies Ⅲ pulmonary veins Ⅲ pulmonary vein stenosis Ⅲ heart defects, congenital T otal anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease in which all pulmonary veins connect to the systemic veins, right atrium, or coronary sinus. TAPVC can occur in conjunction with a wide variety of cardiac malformations, especially atrial isomerism. Before the advent of cardiac surgery, almost all of these children died in the first few months of life. With treatment, there has been continued improvement in the mortality and morbidity of isolated TAPVC. [1][2][3][4] This can be attributed to progress in surgical expertise and to developments in intensive care such as use of nitric oxide and extracorporeal membrane oxygenation, which have led to salvage of the sickest neonates. Conclusions-Preoperative Clinical Perspective on p 2726Nevertheless, there is an ongoing late mortality in patients with TAPVC. It is frequently associated with postoperative pulmonary venous obstruction (PVO), with an incidence of 5% to 18%. [1][2][3][5][6][7][8][9] Postoperative PVO may be a consequence of an inadequate ana...

Objectives 22.4 (range. 16.6-7.0) weeks and the median number of scans per fetus was three (range, one to five). I < −2 at final scan was the most powerful predictor (odds ratio, 3.6 (95% CI,). Shelf was identified in 66% and Flow in 50% of fetuses with CoA.

No abstract

Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur.

Objective: Approximately 2.8% of pregnancies are Ro/La antibody positive. 3-15% of fetuses develop complete heart block (CHB). First-degree atrioventricular heart block (1˚AVB) is reported in a third of Ro/La fetuses but as most have a normal postnatal ECG this may reflect inadequacies of Doppler measurement techniques. Methods: Comparison was made between mechanical (mPR) and electrical (ePR) intervals obtained prospectively using Doppler and non-invasive fetal ECG (fECG) in 52 consecutive Ro/La pregnancies in 46 women carrying 54 fetuses in an observational study at a fetal medicine unit. 121 mPR and 37 ePR intervals were recorded in 49 Ro/La fetuses. Five were referred with CHB and excluded. ePR was measured successfully in 35/37 (94%) and mPR was measured in all cases. 1˚AVB was defined as PR .95% CI. Logistic regression predicted abnormal final fetal rhythm from first mPR or ePR.Results: The ePR model gave 66.7% sensitivity (6 of 8 final abnormal fetal rhythm cases were predicted correctly in fetuses .20 weeks) and 96.2% specificity. mPR gave 44.4% sensitivity (4 of 9 cases) and 88.5% specificity. Z scores for ePR (zPR) were calculated from 199 normal fetuses. The area under the receiver operator characteristic (ROC) curve was 0.88 (95% CI, 0.754 to 1.007). A cut-off of 1.65 gave a sensitivity of 87.5% and specificity of 95% for those with prolonged and normal ePR intervals, respectively. Conclusion: zPR is better than mPR at differentiating between normal and prolonged PR intervals, suggesting that fECG is the diagnostic tool of choice to investigate the natural history and therapy of conduction abnormalities in Ro/La pregnancies. A nti-Ro or La antibody positive pregnancies (Ro/La) have been found in about 2.8% of the pregnant population. The most serious consequence of transplacental transfer of these antibodies to the fetus is complete heart block (CHB), which affects 3% of Ro/La pregnancies, with the risk rising to 15% in a subsequent pregnancy. [1][2][3][4] In addition to the morbidity and mortality associated with pacing procedures in young infants, 5 6 progressive myocardial fibrosis has been reported which affects long-term cardiac function and may necessitate transplantation.7-9 The alloimmune process is thought to exert its effect in more than the 3% of fetuses affected by CHB with PR interval prolongation (first-degree atrioventricular heart block or 1˚AVB) described in up to a third of fetuses in studies using Doppler methods of measurement.10 As most babies have a normal outcome, with progression to CHB described in only a small proportion, there is some debate over whether these findings represent a transient biological response to the presence of antibodies or whether they may be due to inadequacies of the existing measurement techniques. 11A simple and robust method of monitoring the PR interval in affected pregnancies is required to enable studies to assess the extent and determinants of progression to CHB in Ro/La pregnancies. Such a tool would also permit the assessment of different trea...