This review article will guide the reader through the background of prenatal screening for congenital heart disease. The reader will be given insight into the normal screening views, common abnormalities, risk stratification of lesions and also recent advances in prenatal cardiology.
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.
Introduction: Despite improvement in early mortality for total anomalous pulmonary venous connection (TAPVC), late mortality is frequently associated with pulmonary venous obstruction (PVO). We aim...
Ventricular and arterial disproportion with right sided dominance in the fetus are associated with coarctation of aorta. We present the postnatal outcomes of fetuses diagnosed with arterial and/or ventricular disproportion with emphasis on management strategy.
Methods
Retrospective review of fetal echocardiography reports and postnatal outcome of fetuses with arterial and/or ventricular disproportion evaluated between January 2012 and December 2014 at Birmingham Women’s Hospital. Parameters assessed were arterial disproportion, ventricular disproportion, posterior shelf and abnormal flow across the isthmus. The fetuses were categorised into the following three groups - Group A (High risk) – Delivery at unit with a tertiary neonatal centre and postnatal prostaglandin prior to echocardiogram; Group B (moderate risk) – No prostaglandin but echocardiogram prior to discharge, and Group C (low risk) – postnatal clinical assessment.
Results
Of the 68 fetuses with disproportion, there were 3 terminations of pregnancy, 1 intrauterine death and 2 lost to follow up. Of the remaining 62 fetuses, 38 were in Group A, 11 in Group B and 13 in group C. In Group A, 21/38(55%) had coarctation of aorta confirmed postnatally. In Group B, 1 neonate had coarctation which was identified and operated on prior to discharge. No cases of coarctation were identified in group C. Comparing the fetuses with postnatal coarctation in Group A to those that did not, fetuses with coarctation had more antenatal scans (median 3 vs. 2; p < 0.05) and the decision to start prostaglandin was made earlier in pregnancy (median 29 weeks gestation versus 33 weeks; p < 0.05).
Conclusions
Risk stratification was a safe and effective way of managing fetuses with antenatal disproportion. However, 45% (n = 17) of neonates started on prostaglandin did not have coarctation. Further study is required to improve the predictive accuracy of antenatal diagnosis in this difficult subset of patients.
To appreciate congenital heart disease fully, a detailed understanding of the anatomical presentation, as well as the physiology, is required. This is often introduced at an advanced stage of training. Professor Anderson has been influential in the Clinical Anatomy Intercalated BSc programme at the University of Birmingham, in particular in his teaching on Sequential Segmental Analysis. This article describes the experiences of the latest cohort of students on this programme, who undertook varying research projects using the Birmingham Cardiac Archive, with the guidance of Professor Anderson. The projects outlined include various aspects of isomerism, encompassing both the cardiac and abdominal manifestations, as well as details of congenitally corrected transposition of the great arteries and prenatally diagnosed right aortic arch and double arch. These studies all aimed to increase the knowledge base of their respective cardiac malformations and provide a basis for further research.
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