Katarina Hanseus

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}

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation The TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) Study

Background —The Fontan procedure has improved survival in children with functionally univentricular hearts. With time however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial but pulmonary vascular resistance (PVR) probably plays a crucial role. Elevated PVR has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardio-pulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. Methods and Results —Seventy-five adolescents and adults were randomized 1:1 to 14 weeks treatment with bosentan or placebo. Cardio-pulmonary exercise-test (CPET), functional class, blood samples and quality-of-life questionnaire were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 (from 28.7 to 30.7) ml/kg/min in the bosentan group compared to 0.6 (from 28.4 to 29.0) ml/kg/min in the placebo group, p=0.02. CPET time increased 0.48 (from 6.79 to 7.27) vs. 0.08 (from 6.94 to 7.02) minutes, p=0.04. Nine bosentan treated patients improved one functional class, whereas none improved in the placebo group, p=0.0085. Side effects were mild and occurred equally in both groups. No serious adverse effects were seen and no patients had liver enzyme levels above three-fold upper limit. Conclusions —Bosentan improves exercise capacity, exercise time and functional class in Fontan patients without serious adverse events or hepatotoxicity. Clinical Trial Registration Information —www.clinicaltrials.gov. Identifier [NCT01292551][1]. [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT01292551&atom=%2Fcirculationaha%2Fearly%2F2014%2F10%2F20%2FCIRCULATIONAHA.113.008441.atomBackground— The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. Methods and Results— Seventy-five adolescents and adults were randomized 1:1 to 14 weeks of treatment with bosentan or placebo. Cardiopulmonary exercise test, functional class, blood samples, and quality-of-life questionnaires were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 mL·kg−1·min−1 (from 28.7 to 30.7 mL·kg−1·min−1) in the bosentan group compared with 0.6 mL·kg−1·min−1 (from 28.4 to 29.0 mL·kg−1·min−1) in the placebo group (P=0.02). Cardiopulmonary exercise test time increased by 0.48 minute (from 6.79 to 7.27 minutes) versus 0.08 minute (from 6.94 to 7.02 minutes; P=0.04). Nine bosentan-treated patients improved 1 functional class, whereas none improved in the placebo group (P=0.0085). Side effects were mild and occurred equally in both groups. No serious adverse effects were seen, and no patients had liver enzyme levels above the 3-fold upper limit. Conclusions— Bosentan improves exercise capacity, exercise time, and functional class in Fontan patients without serious adverse events or hepatotoxicity. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT01292551.

Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation: The TEMPO Study

Background —The Fontan procedure has improved survival in children with functionally univentricular hearts. With time however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial but pulmonary vascular resistance (PVR) probably plays a crucial role. Elevated PVR has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardio-pulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. Methods and Results —Seventy-five adolescents and adults were randomized 1:1 to 14 weeks treatment with bosentan or placebo. Cardio-pulmonary exercise-test (CPET), functional class, blood samples and quality-of-life questionnaire were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 (from 28.7 to 30.7) ml/kg/min in the bosentan group compared to 0.6 (from 28.4 to 29.0) ml/kg/min in the placebo group, p=0.02. CPET time increased 0.48 (from 6.79 to 7.27) vs. 0.08 (from 6.94 to 7.02) minutes, p=0.04. Nine bosentan treated patients improved one functional class, whereas none improved in the placebo group, p=0.0085. Side effects were mild and occurred equally in both groups. No serious adverse effects were seen and no patients had liver enzyme levels above three-fold upper limit. Conclusions —Bosentan improves exercise capacity, exercise time and functional class in Fontan patients without serious adverse events or hepatotoxicity. Clinical Trial Registration Information —www.clinicaltrials.gov. Identifier [NCT01292551][1]. [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT01292551&atom=%2Fcirculationaha%2Fearly%2F2014%2F10%2F20%2FCIRCULATIONAHA.113.008441.atomBackground— The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. Methods and Results— Seventy-five adolescents and adults were randomized 1:1 to 14 weeks of treatment with bosentan or placebo. Cardiopulmonary exercise test, functional class, blood samples, and quality-of-life questionnaires were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 mL·kg−1·min−1 (from 28.7 to 30.7 mL·kg−1·min−1) in the bosentan group compared with 0.6 mL·kg−1·min−1 (from 28.4 to 29.0 mL·kg−1·min−1) in the placebo group (P=0.02). Cardiopulmonary exercise test time increased by 0.48 minute (from 6.79 to 7.27 minutes) versus 0.08 minute (from 6.94 to 7.02 minutes; P=0.04). Nine bosentan-treated patients improved 1 functional class, whereas none improved in the placebo group (P=0.0085). Side effects were mild and occurred equally in both groups. No serious adverse effects were seen, and no patients had liver enzyme levels above the 3-fold upper limit. Conclusions— Bosentan improves exercise capacity, exercise time, and functional class in Fontan patients without serious adverse events or hepatotoxicity. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT01292551.

Dimensions of cardiac chambers and great vessels by cross-sectional echocardiography in infants and children.

SummaryA total of 120 healthy infants, children, and teenagers were examined by cross-sectional echocardiography. Right and left atrial and ventricular dimensions and areas were measured in the parasternal, apical, and subcostal views. Dimensions of the inferior caval vein, the pulmonary artery and the aorta were obtained in the parasternal, suprasternal, and subcostal views. Reproducibility was studied in separate material consisting of 19 children with various forms of congenital heart disease examined consecutively by two different observers. Interobserver reproducibility was expressed as the 95% tolerance limit for the difference between two measurements.Good correlation with body surface area was demonstrated for all measurements, and the regression equations for the normal values of the parameters studied are given. Interobserver reproducibility was fairly good for measurements in the parasternal views, but moderate or low for measurements in the apical and the subcostal four-chamber views.Measurements in cross-sectional echocardiography are clinically useful, especially in the study of the right-sided cardiac structures that are difficult to evaluate with M-mode echocardiography, but the problems of reproducibility have to be taken into account.

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.

Collateral flow in coarctation of the aorta with magnetic resonance velocity mapping: Correlation to morphological imaging of collateral vessels

To correlate quantification of collateral flow in aortic coarctation with the morphological visualization of the collateral vessels and to compare different approaches to measurement of collateral flow.

Analysis of Atrioventricular Plane Movements by Doppler Tissue Imaging and M-Mode in Children with Atrial Septal Defects Before and After Surgical and Device Closure

Our objective was to compare the effects of surgical and device closure of atrial septal defects (ASDs) on atrioventricular plane function. In healthy individuals, both short- and long-axis motion contribute to ventricular pump function. Short-axis function (i.e., the amplitude and velocity of atrioventricular plane movements) may be evaluated by M-mode and Doppler tissue imaging. The study group consisted of 19 children with ASD of the secundum type before and after surgical (n = 12) or device (n = 7) closure and 10 healthy controls. Surgical and device closures were uncomplicated and all defects were completely closed. Registrations of atrioventricular plane systolic and diastolic amplitude and velocity were made from the tricuspid and the mitral annulus and from the septum in the apical four-chamber view. Comparisons were made between examinations before and after closure, between the two subgroups of patients treated by surgical and device closure, respectively, and between the patient group and the control groups. Before ASD closure, all measurements were normal or near normal. After surgery, systolic amplitudes and velocities of the tricuspid annulus and in the septum decreased significantly, whereas no changes were seen in the device group. Less marked changes were seen in diastolic measurements. However, in the surgical group significant decrease to subnormal values were found in the tricuspid annulus and in the septum, which may indicate a decreased diastolic function postoperatively. Mitral valve annulus amplitude and velocity were not affected by the treatment. Atrioventricular function is normal in children with right ventricular volume overload. The decrease to subnormal values after open-heart surgery is not seen after device closure of the ASD, indicating that surgery affects right ventricular function.

Cross-sectional echocardiographic measurements of right ventricular size and growth in patients with pulmonary atresia and intact ventricular septum

SummaryFifteen patients with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum were studied. All were operated on in the neonatal period, with valvotomy or a systemic to pulmonary arterial shunt, or both. In 12 patients, right ventricular to pulmonary arterial communication was established in the neonatal period. In three patients, only systemic to pulmonary arterial shunts were constructed. Six patients died. The median follow-up period for the surviving patients was 65 months (range, 12–87 months).Right and left atrial and ventricular dimensions and areas, the tricuspid annular diameter, and the cross-sectional area of the aortic root were measured in cross-sectional echocardiograms from the neonatal period, at the age of 1 year, and at the latest clinical follow-up. A classification of right ventricular morphology was made, based on identification of the inlet, the trabecular, and the outlet parts.Most of the patients had hypoplastic right ventricles at birth but at the latest follow-up, seven of nine surviving patients had right ventricles in the normal range. Right ventricular growth was better in patients who were given a right ventricular to pulmonary arterial communication in the neonatal period and those with complete right ventricular anatomy. The patients who died had severely hypoplastic right ventricles and small tricuspid valves.

Long-Distance Transports of Newborn Infants with Congenital Heart Disease

Abstract. Little has been published about specific problems that may occur during long-distance transports of newborn cardiac patients. During a 4-year period after centralization of pediatric heart surgery in Sweden, 286 transports were prospectively investigated. A majority (77.3%) of the transports were carried out by nonspecialized teams. Ten severe adverse events, including the death of 1 infant, occurred during the 286 transports (3.5%). Another infant died later of cerebral complications from hypoxia, rendering a transport-related mortality of 0.7%. Twenty-two infants (7.7%) were severely hypoxic (oxygen saturation ≤65%) at arrival, and 12 of these infants suffered from transposition of the great arteries. During the second 2-year period increased use of intravenous prostaglandin E1 and transportation from tertiary-level units was associated with better transport outcome. During the same time period, overall 30-day postoperative mortality for pediatric cardiac surgery decreased from 4.0% to 1.2% in our hospital. When highly specialized treatment is centralized for quality reasons it is also important that risks associated with transport are considered and that the quality of transport is high. For some cardiac malformations antenatal diagnosis and referral of the mother for delivery to a center with pediatric cardiac surgery would probably further increase the chance of healthy survival in some infants.

Parental Reactions to Fetal Echocardiography

The impact of fetal echocardiography (FE) was studied in 65 families where a previous child had been born with heart disease and where FE showed normal cardiac anatomy in the fetus. Forty-six of the families had lost their child with heart disease (Group 1), while the child was alive in 19 of the families (Group 2). A questionnaire concerning the attitude of the parents toward FE was sent to all families. The possibility of having FE had influenced the decision to have another child in 64% in Group 1 and in 26% in Group 2 (P < 0.01). If a serious cardiac malformation had been found at FE, 69% in Group 1 and 29% in Group 2 would have chosen abortion (P < 0.01). The anxiety decreased significantly in both groups after FE. Of the participating mothers, 96% wishes FE at the next pregnancy. The study indicates that FE probably is very important to families who had previously had a child with a serious malformation but also that many families intend to continue the pregnancy even if a malformation is diagnosed.