Objective-Fetal twin-twin transfusion syndrome (TTTS) presenting in the second trimester has been associated with almost no perinatal survival until recently, when serial drainage of amniotic fluid has improved the prognosis to 70%-80%. Most Clinical manifestations in utero range from mild to critical pulmonary stenosis or lethal cardiomyopathy. Although perinatal prognosis seems to be related to the severity of dysfunction when first diagnosed in utero, follow up in infancy is recommended in view of the possibility of progressive pulmonary stenosis. (Br Heart J 1994;72:74-79) Twin-twin transfusion syndrome (TTTS) is a severe complication of monozygotic twinning. It arises in 4%-26% of diamniotic monochorionic gestations, presumably as the result of vascular anastomoses between the circulation of one twin (the donor) and that of its co-twin (the recipient) leading to circulatory disequilibrium.1 As a result of the transfusion, the donor twin becomes growth retarded and oliguric and develops oligohydramnios, whereas the recipient twin becomes polyuric with severe hydramnios and may develop hydrops. The traditional explanation that transfusion of blood from one twin to the other along placental vascular anastomoses produces hypovolaemia and anaemia in the donor and circulatory overload with polycythaemia and hyperviscosity in the recipient2 may be oversimplified. Recent evidence suggests that haematological discordance is unlikely in second trimester l-l-S as investigated by fetal blood sampling.' Also, umbilical venous pressure is not significantly raised in all hydropic recipient twins.4 These are features inconsistent with the simple explanation of circulatory overload.Severe 'I-'-S presenting in the second trimester has until recently been associated with nearly 100% perinatal death.2 The main cause of perinatal loss is premature delivery due to severe polyhydramnios. Serial amnioreduction now allows survival in 70%-80%56 mainly by allowing prolongation of pregnancy, but also possibly by improving fetal condition6 with most cases of severe second trimester 'TITS now progressing into the third trimester. We have found that most recipient twins develop cardiac dysfunction in utero, predominantly affecting the right ventricle and pulmonary artery, which can result in neonatal morbidity and mortality.This report describes the clinical and echocardiographic features of cardiac dysfunction in recipient twins in second trimester T'1TS. Patients and methodsWe studied five pregnancies complicated with T'-TS. Gestational age at referral was 17-25 weeks (median 19). The inclusion criteria were: (a) monochorial twins of the same sex;

Objective-To determine the effects of residual pulmonary regurgitation on exercise tolerance after complete repair of tetralogy of Fallot.Design-Prospective study of symptom free patients more than five years after complete repair. Results-There was a significant negative correlation between the degree of residual regurgitation and both total duration of exercise and maximal heart rate achieved. Maximal heart rate and total duration of exercise were significantly lower in the patients than in normal controls. Patients with an abnormal maximal oxygen uptake (less than 85% of the predicted normal value) had significantly greater residual pulmonary regurgitation than those in whom oxygen uptake was normal.Conclusions-Impaired exercise capacity after complete repair of tetralogy of Fallot is directly related to the degree of residual pulmonary regurgitation. These data should be taken into account when deciding the optimal timing and nature of corrective surgery. relation between the degree of impairment of exercise and the heart size on chest radiography, which led them to speculate that residual pulmonary incompetence may be related to exercise ability after operation.5We recently described a new method of measuring the amount of pulmonary regurgitation by analysing right ventricular pressure-volume loops.8 In our study there was a linear relation between the pulmonary regurgitant volume and right ventricular end diastolic and end systolic volumes. No such relation could be shown, however, between regurgitant volume and the right ventricular ejection fraction.In the present study we used this technique to examine the possible influence of pulmonary regurgitation on patients' exercise ability after complete repair. Patients and methods PATIENTSThe initial study group comprised 18 patients. In two patients data from cardiac catheterisation were unsatisfactory (residual ventricular septal defect (VSD) in one, unsuitable data for analysis in one), and a further four patients failed to achieve a respiratory quotient > 1 0 during graded exercise testing (see below). These six patients were not included in the analysis.Satisfactory data from cardiac catheterisation and exercise were therefore obtained for 12

The best predictive scores for post-natal outcome in fetal PAIVS/CPS are a combination of morphologic and physiological variables, which predict a BV circulation with a sensitivity of 92% and specificity of 100% before 26 weeks.

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...

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To present further experience and intermediate term outcome in 30 patients with single muscular ventricular septal defects (MVSDs) who underwent transcatheter closure with the Amplatzer ventricular septal defect occluder (AVSDO). Patients and design: Thirty patients, aged 4 months to 16 years, with MVSDs underwent transcatheter closure with the AVSDO. The device consists of two low profile disks made of Nitinol wire mesh with a 7 mm connecting waist. The prosthesis size (waist diameter) was selected to be equal to the balloon ''stretched'' diameter of the defect. A 7-9 French sheath was used to deliver the AVSDO. Fluoroscopy and transoesophageal echocardiography guided the procedure. Results: The stretched diameter of the defects ranged from 6-14 mm. The communication was completely occluded in 28 of 30 patients (93% closure rate). One patient (a 4 month old infant) with sustained complete left bundle branch block after the procedure went on to develop complete heart block one year later. No other complications were observed during a mean follow up of 2.2 years (range 0.25-4.5 years). Conclusions: The AVSDO is an efficient prosthesis that can be safely used in the majority of patients with a single MVSD. Further studies are required to establish long term results in a larger patient population.

Objectives-The starting hypothesis was that some perimembranous ventricular septal defects can be closed safely and effectively with a Bard Rashkind double umbrella introduced through a long transvenous sheath. Design-A descriptive study of all patients who underwent attempted transcatheter umbrella closure of a perimembranous ventricular septal defect. Those patients selected for the study had symptoms of a ventricular septal defect and a perimembranous ventricular septal defect shown by transthoracic echocardiography. The morphological criteria used were a posterior perimembranous defect with a diameter of < 8 mm not associated with overriding of the aortic or pulmonary valve or with aortic valve prolapse. The haemodynamic criteria for inclusion in the study were a right to left ventricular systolic pressure ratio of > 0.45, a Doppler derived right ventricular systolic pressure of > 50 mm Hg, and a pulmonary to systemic flow ratio > 3:1.