P. Bonhoeffer

Long-term fate of the coronary arteries after the arterial switch operation in newborns with transposition of the great arteries.

OBJECTIVE: Concern continues to be expressed about the long-term impact of coronary artery translocation after the arterial switch operation for transposition of the great arteries. This study was conducted to determine the prevalence of obstructions of the translocated coronary arteries by the use of selective coronary artery angiography. METHODS AND RESULTS: 64 children (mean age 7.6 (SD) 1.5 years) who had survived an arterial switch operation underwent evaluation. They had been operated on by one surgeon and they were followed up by a single hospital. Selective coronary artery angiography was possible in 58 patients. Five patients showed occlusion or stenosis of a coronary artery: one occlusion and two stenoses of the left coronary trunk, two occlusions of the circumflex artery. The prevalence of late coronary artery complications was 7.8 (SD) 6.6% (95% CI 1.2 to -14.4%). The three patients with occlusion of one coronary artery had perioperative ischaemic complications, with associated electrocardiogram evidence of ischaemia and left ventricular dysfunction with mitral valve insufficiency. Both patients with stenosis of the left main coronary artery trunk did not have any evidence of an anomaly before catheterisation. CONCLUSIONS: The prevalence of the late coronary artery complications after an arterial switch operation was low in this series. This accords with the view that the arterial switch operation remains the preferred treatment for such patients. Screening for late coronary artery patency should be done by using selective coronary artery angiography, because even patients who remain symptom free can have coronary artery anomalies.

Quality of life and perceived health status in surviving adults with univentricular heart

OBJECTIVE To evaluate the quality of life in patients with univentricular heart and to determine the impact of sociodemographic and clinical characteristics. DESIGN AND SETTING Retrospective, cross sectional study conducted in a regional paediatric cardiology centre. PATIENTS The health records of 89 survivors with univentricular heart (median age 21 years; range 17–49 years) were reviewed. Sixty seven answered the Duke questionnaire. Sociodemographic and clinical variables were similar in the responders and non-responders. The impact of sociodemographic and clinical variables on individual Dukes measures was assessed. RESULTS The Duke scores of adults with univentricular heart were similar to the normal population. Cyanosis predicted a worse score for physical (pu2009=u20090.05) and perceived health measures (pu2009=u20090.02). A higher educational level predicted a better score for physical (pu2009=u20090.004), mental (pu2009=u20090.01), and general health measures (pu2009=u20090.02). Orthopaedic problems worsened the social score (pu2009=u20090.05). Psychosocial problems worsened the pain score (pu2009=u20090.04). In comparison with the other anatomical types, mitral atresia worsened the perceived health score (pu2009=u20090.02). Patients younger than 23 years scored better for almost all health and dysfunction measures. CONCLUSIONS Despite repeated interventions and other disease related everyday stresses, a selected group of adults with univentricular heart had a satisfying quality of life.

Relation of genotype 22q11 deletion to phenotype of pulmonary vessels in tetralogy of Fallot and pulmonary atresia–ventricular septal defect

Objective To compare the morphology of the pulmonary vessels in tetralogy of Fallot or pulmonary atresia–ventricular septal defect (PA–VSD) with (del22q) and without 22q11 deletion (non-del22q). Patients 94 consecutive infants (54 with tetralogy of Fallot, 40 with PA–VSD) were studied using ultrasound and catheterisation. Molecular investigations Identification of the 22q deletion was performed either by fluorescent in situ hybridisation or polymerisation chain reaction genotyping. Results 25 patients were del22q (16/40 (40%) PA–VSD v 9/54 (17%) tetralogy of Fallot; pu2009<u20090.02). Major aortopulmonary collateral arteries was more common in patients with PA–VSD-del22q (pu2009<u20090.03). Such collaterals were identified in 13 patients: 10 del22q and three non-del22q (pu2009<u20090.001). The size of the right and left pulmonary arteries expressed as a standard deviation (SD) difference of the normal range was −4.2 (quartiles −5.3 and −2.9) for PA–VSD del22q, and −2.6 (−3.1 and −1.8) for PA–VSD non-del22q (pu2009=u20090.02). The mean (SD) difference between the measured and theoretical Nakata index was −373 (94) for PA–VSD del22q v−245 (93) in PA–VSD non-del22q (pu2009=u20090.0002). In tetralogy of Fallot patients with and without del22q, the size of the pulmonary arteries was similar (pu2009=u20090.6). Conclusions A “specific” phenotype could be defined in patients with deletion: PA–VSD, major aortopulmonary collateral arteries with complex loop morphology, and small central pulmonary arteries. Differences in the morphology of the pulmonary vessels may indicate a different timing of the faulty developmental pathway in patients with and without 22q11 deletion.

Maintaining tricuspid valve competence in double discordance: a challenge for the paediatric cardiologist

Objectives To establish the prevalence of tricuspid valve abnormalities in children with a double discordant heart (or congenitally corrected transposition of the great arteries); to study the influence of the loading conditions induced by various surgical interventions on the right and left ventricle in patients with double discordance and an abnormal tricuspid valve; and to propose a rational surgical approach. Methods Case notes were reviewed of 141 consecutive patients admitted in the first year of life with various types of double discordance (intact ventricular septum (group 1), ventricular septal defect (group 2), ventricular septal defect and pulmonary obstruction (group 3)). A study group of 62 patients with an abnormal tricuspid valve was selected by cross sectional echocardiography. These were followed up through palliative and open heart procedures with grading of tricuspid regurgitation. Results Tricuspid valve abnormalities were more common in groups 1 and 2 (60% and 56%) than in group 3 (31%). Preoperative tricuspid regurgitation was more common in group 2 (90%) than in groups 1 and 3 (38% and 36%). Ten patients in groups 1 and 2 died in the neonatal period with severe tricuspid regurgitation, associated with coarctation of the aorta in 60%. Eight patients in group 1 had no surgery and are doing well, with a competent tricuspid valve. Palliative procedures were undertaken in 28 patients: 14 had pulmonary artery banding, which resulted in a decrease in tricuspid regurgitation, 12 in group 2 by reducing the pulmonary blood flow and two in group 1 by changing the septal geometry; 14 in group 3 had an aortopulmonary shunt, which induced tricuspid regurgitation in two. Twenty patients are still alive after palliation, with stable tricuspid valve function. Repair of the tricuspid valve was unsuccessful in the three patients who underwent conventional surgery, leaving the right ventricle facing the systemic circulation. In two patients with a competent but abnormal tricuspid valve, conventional surgery induced severe tricuspid regurgitation. Of the 15 patients who underwent conventional surgery, only 10 survived (mortality 33%): eight with a tricuspid valve prosthesis and two with severe residual tricuspid regurgitation. However, tricuspid regurgitation decreased after anatomical correction (nine patients), restoring a systemic left ventricle and a subpulmonary right ventricle, even when the tricuspid valve was not repaired (five patients). Eight patients are doing well after anatomical correction (mortality 11%). Conclusions Tricuspid valve function in double discordance with an abnormal tricuspid valve depends on the loading conditions of both ventricles and on the septal geometry. Interventions that increase right ventricular volume or decrease left ventricular pressure are likely to induce tricuspid regurgitation, while those that decrease right ventricular volume or increase left ventricular pressure are likely to improve tricuspid valve function. Repair of the tricuspid valve always failed when the right ventricle was left in a systemic position and always succeeded when the right ventricle was placed in a subpulmonary position. These results should be taken in to account when dealing with patients with double discordance and an abnormal tricuspid valve.

Assessment of the geometric profile of the Amplatzer and Cardioseal septal occluders by three dimensional echocardiography

OBJECTIVE To apply three dimensional echocardiography to describe the geometric profile of the Amplatzer and Cardioseal occluders after deployment for closure of atrial septal defect. METHODS 20 patients (mean (SD) age, 14 (5) years) were enrolled for transcatheter closure of a secundum atrial septal defect with the Amplatzer occluder (10) or with the Cardioseal occluder (10). The two populations were matched for the stretched diameter of the defect (mean 18 (6) mm). The profile of the two occluders was examined. RESULTS Transoesophageal echocardiography did not show any residual shunts after Amplatzer occluder deployment, whereas three patients had a small residual leak after Cardioseal deployment. One patient had transient atrioventricular block with the Amplatzer device. The mean surface area of the Amplatzer occluder was 6.9 (2)u2009cm2, and that of the Cardioseal device 5.4 (3)u2009cm2 (pu2009=u20090.03). The mean volume of the Amplatzer occluder was 9.2 (1)u2009cm3, while that of the Cardioseal occluder was 3.5 (1)u2009cm3 (pu2009<u20090.0001). From the three dimensional views, the Cardioseal occluder looked like a flat square after deployment whereas the Amplatzer occluder took up a ball shape in the atrial cavity. CONCLUSIONS Three dimensional views by multiplane transoesophageal echocardiography allow a realistic in vivo description of atrial septal occluders. The Amplatzer occluder, with its high geometric profile, allows complete closure of large atrial septal defects but with some risk of mechanical complications. Use of the Cardioseal device, with its small surface coverage and high residual shunt rate, should be limited to transcatheter closure of a patent foramen ovale or small atrial septal defects.

Pulmonary balloon valvuloplasty in the palliation of complex cyanotic congenital heart disease.

OBJECTIVE: To assess the value of pulmonary balloon valvuloplasty in the interim palliation of complex congenital heart disease and pulmonary stenosis in children, who often require numerous palliative operations before definitive surgical repair. METHODS: Evaluation of pulmonary balloon valvuloplasty procedures performed over a five year period in 18 patients (age 8 days--29 years; mean 5.5 years) with complex cyanotic congenital heart disease. RESULTS: After pulmonary balloon valvuloplasty oxygen saturation increased from a mean (SD) of 69 (7.5)% to 83 (7.0)% (P < 0.001). Mean pulmonary artery pressure increased from a mean (SD) of 11.3 (3.8) mm Hg to 15.7 (3.9) mm Hg (P < 0.001). Transient complete atrioventricular block occurred in one patient. No other complications were encountered. In 5 patients (28%) there was an inadequate improvement in cyanosis compared with pre-procedure values (72 (4.7)% v 66 (8.1)%). Reasons for failure were increasing infundibular stenosis in three and inadequate mixing in one child. In 13 patients (72%) pulmonary balloon valvuloplasty gave adequate interim palliation over a mean follow up of 1.1 (1.3) years. Oxygen saturation was 81 (5.6)% at last follow up compared with 70 (7.3)% before pulmonary balloon valvuloplasty (P < 0.001). CONCLUSION: Pulmonary balloon valvuloplasty is a safe and effective technique in the palliation of patients with complex cyanotic congenital heart disease associated with pulmonary valve stenosis.

Thrombus in coarctation of the aorta masquerading as an interrupted aortic arch.

Accepted for publication 27 September 1996 Coarctation of the aorta can be congenital or develop shortly after birth at the time of duct closure. The interrupted aortic arch is a different congenital heart disease both in its development during embryogenesis and in the anatomy of the aortic arch. Complete disconnection between the ascending and descending aorta is characteristic of interruption of the aortic arch. Atresia of the aortic arch is an extremely rare form of aortic coarctation in which the continuity of the aortic arch persists only through an imperforate fibrous cord. Here we report an unusual case of intra-aortic thrombosis masquerading as an interrupted aortic arch in a newborn infant who was 12 hours old. Aortic coarctation was clinically suspected because of multiorgan failure in the absence of femoral pulses. The echocardiogram showed a horizontal and initial descending aorta of normal size but a mass in the aortic arch caused complete obstruction to flow at this level. The left ventricle was hypokinetic and the duct was closed at the time of examination. The interventricular septum was intact. A left ventricular angiogram was obtained by the transvenous approach with the catheter passed through the patent foramen ovale. The angiogram confirmed occlusion of the aortic arch after the origin of the brachiocephalic trunk and a large thrombus was identified in the aortic arch (figure). The lower body was supplied with blood only through thoracic collateral vessels, and the contralateral upper part of the body was perfused by retrograde flow from the left common carotid artery through the circle of Willis. At the age of three days (3000 g) the child underwent surgery. Above a severe typical coarctation of the aorta, the surgeon found a large old thrombus extending from the isthmus back to the brachiocephalic trunk which obstructed the left subclavian and common carotid arteries. The thrombus was removed and the coarctation was successfully repaired. Despite heparinisation and thrombolysis, multiple venous thrombi developed, particularly in the inferior vena cava, leading to death 13 days postoperatively. Together with the aortic thrombus, these diffuse clotting complications were suggestive of an underlying constitutive haemostatic disease that could not be identified.

The multi-track angiography catheter: a new tool for complex catheterisation in congenital heart disease.

OBJECTIVE: To develop a simple and versatile catheter system for complex cardiac catheterisation because angiography and pressure measurements during diagnostic and interventional cardiac catheterisation are often unsatisfactory. METHODS: The Multi-Track Angio catheter system is a single lumen side-hole catheter with a short distal extension containing a lumen for a standard guidewire. The catheter is introduced over a previously placed guidewire running through this distal extension. It can then be manipulated within the heart by sliding along the guidewire. The tip of the catheter is always stabilised by the guidewire. This stability enhances angiography and pressure recordings. RESULTS: The Multi-Track Angio catheter system was used in 84 patients (age 1 day-20 years). Thirty one procedures were diagnostic and 53 interventional. The decision to use the Multi-Track Angio catheter was based on three criteria: firstly, unsatisfactory angiography obtained with conventional equipment; secondly, difficult catheter course requiring use of a guidewire; and thirdly, requirement for angiography and pressure recordings during interventional procedures. No complications were encountered. High quality angiography could be performed in all cases without catheter recoil. CONCLUSIONS: The Multi-Track Angio catheter system allows for high quality angiography and pressure recordings during diagnostic and interventional cardiac catheterisation. The advantage of the system is that both angiography and pressure recordings can be performed repeatedly from stable catheter positions using a previously placed guidewire. This reduces the need for guidewire manipulations or catheter exchanges and decreases procedure time and the risk of complications.

Melody Valve Implantation in Pulmonary Position

Surgical pulmonary valve replacement lacks longevity as conduit dysfunction usually occurs within 10–15 years and exposes patients to multiple risky operations over their lifetime. The recent availability of a percutaneous approach to treat RVOT dysfunction, therefore, offers an attractive solution, as it permits earlier intervention without the problems associated with surgery and cardiopulmonary bypass. Initial midterm results are promising, and the technique has proved safe and has provided efficacious relief of pressure and/or volume overload. These results are associated with improvement of symptoms and objective exercise capacity. However, PPVI is not free from possible complications. Due to anatomical (size and morphology) and dynamic reasons, with the current device, only 15 % of patients with RVOT dysfunction are eligible for such a treatment.