The aorto-left ventricular tunnel from a fetal perspective: Original case series and literature review.

INTRODUCTION: Aorto-left ventricular tunnel (ALVT) accounts for <0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited. With this retrospective international case series, we provide information on the outcome of fetuses with ALVT. METHODS: All members of the Association for European Pediatric and Congenital Cardiology's (AEPC) fetal working group and fetal medicine units worldwide were invited for participation. We observed antenatal parameters, neonatal outcome and postnatal follow-up. Additionally, a systematic search of the literature was performed. RESULTS: Twenty fetuses with ALVT were identified in 10 participating centers (2001-2019). Fetal echocardiographic characteristics of ALVT included an increased cardiac-thorax ratio (95%), left ventricular end-diastolic diameter (90%) and a dysplastic aortic valve (90%). Extracardiac malformations were rare (5%). Eight fetuses died at a median gestational age (GA) of 21 + 6 weeks (range, 19-24): all showed signs of hydrops prior to 24 weeks or at autopsy. All others (60%, 12/2) were live-born (median GA 38 + 4, range 37-40), underwent surgery and were alive at last follow up (median 3.2 years, range 0.1-17). The literature reported 22 ALVT fetuses with similar outcome. CONCLUSIONS: In the absence of fetal hydrops, ALVT carries a good prognosis. Fetuses who survive to 24 weeks without hydrops are likely to have a good outcome.

The Leiden Convention coronary coding system: translation from the surgical to the universal view.

AIMS: The Leiden Convention coronary coding system structures the large variety of coronary anatomical patterns; isolated and in congenital heart disease. It is widely used by surgeons but not by cardiologists as the system uses a surgeons' cranial view. Since thoracic surgeons and cardiologists work closely together, a coronary coding system practical for both disciplines is mandatory. To this purpose, the 'surgical' coronary coding system was adapted to an 'imaging' system, extending its applicability to different cardiac imaging techniques. METHODS AND RESULTS: The physician takes place in the non-facing sinus of the aortic valve, oriented with the back towards the pulmonary valve, looking outward from the sinus. From this position, the right-hand sinus is sinus 1, and the left-hand sinus is sinus 2. Next, a clockwise rotation is adopted starting at sinus 1 and the encountered coronary branches described. Annotation of the normal anatomical pattern is 1R-2LCx, corresponding to the 'surgical' coding system. The 'imaging' coding system was made applicable for Computed Tomography (CT), Magnetic Resonance Imaging (MRI), echocardiography, and coronary angiography, thus facilitating interdisciplinary use. To assess applicability in daily clinical practice, images from different imaging modalities were annotated by cardiologists and cardiology residents and results scored. The average score upon evaluation was 87.5%, with the highest scores for CT and MRI images (average 90%). CONCLUSION: The imaging Leiden Convention is a coronary coding system that unifies the annotation of coronary anatomy for thoracic surgeons, cardiologists, and radiologists. Validation of the coding system shows it can be easily and reliably applied in clinical practice.

Implantable cardioverter defibrillator therapy for prevention of sudden cardiac death in children in the Netherlands.

INTRODUCTION: Implantable cardioverter defibrillator (ICD) therapy is increasingly used in children. The purpose of this multicenter study is to evaluate mid-term clinical outcome and to identify predictors for device discharge in pediatric ICD recipients. METHODS AND RESULTS: From 1995 to 2006, 45 patients in The Netherlands under the age of 18 years received an ICD. Mean age at implantation was 10.8 +/- 5.2 years. Primary prevention (N = 22) and secondary prevention (N = 23) were equally distributed. Underlying cardiac disorders were primary electrical disease (55%), cardiomyopathy (20%), and congenital heart disease (17%). The follow-up was 44 +/- 32.9 months. Three patients (7%) died and one patient (2%) underwent heart transplantation. ICD-related complications occurred in eight patients (17%), seven of whom had lead-related complications. Fourteen patients (31%) received appropriate ICD shocks; 12 patients (27%) received inappropriate ICD shocks. Fifty-five percent of 22 ICD recipients under the age of 12 years received appropriate shocks, which was higher as compared with 9% of 23 older ICD recipients (P = 0.003). Although the incidence of appropriate shocks in the present study was larger in secondary prevention (9/23; 39%) as compared with primary prevention (5/22; 23%), this difference did not reach significance. CONCLUSIONS: In our population of patients, children <12 years of age had more appropriate shocks than patients 13-18 years. The complication rate is low, and is mainly lead related.

Electrocardiographic criteria for left ventricular hypertrophy in children.

Previous studies to determine the sensitivity of the electrocardiogram (ECG) for left ventricular hypertrophy (LVH) in children had their imperfections: they were not done on an unselected hospital population, several criteria used in adults were not applied to children, and obsolete limits of normal for the ECG parameters were used. Furthermore, left ventricular mass (LVM) was taken as the reference standard for LVH, with no regard for other clinical evidence. The study population consisted of 832 children from whom a 12-lead ECG and an M-mode echocardiogram were taken on the same day. The validity of the ECG criteria was judged on the basis of an abnormal LVM index, either alone or in combination with other clinical evidence. The ECG criteria were based on recently established age-dependent normal limits. At 95% specificity, the ECG criteria have low sensitivities (<25%) when an elevated LVM index is taken as the reference for LVH. When clinical evidence is also taken into account, the sensitivity improved considerably (<43%). Sensitivities could be further improved when ECG parameters were combined. The sensitivity of the pediatric ECG in detecting LVH is low but depends strongly on the definition of the reference used for validation.

Follow-up outcomes 10 years after arterial switch operation for transposition of the great arteries: comparison of cardiological health status and health-related quality of life to those of the a normal reference population.

The purpose of this study was to investigate the cardiological health status and health-related quality of life after the arterial switch operation (ASO) for transposition of the great arteries (TGA) in comparison with a normative reference group. Chart review and cross-sectional systematic follow-up, including echocardiography, exercise testing, and electrocardiography, were performed on all survivors of ASO for TGA between 1990 and 1995. Health-related quality of life (HRQOL) was assessed using a standardized questionnaire. A normative reference group was included. Forty-nine survivors [median age at operation 13 days, mean age at follow-up 11 +/- 2 years (37/49 with intact ventricular septum] were identified. Thirty-three of 49 patients (67%) [22/33 TGA with intact ventricular septum (IVS)] participated in cross-sectional follow-up. Cumulative 10-year event-free survival was 88% and the re-intervention rate 6%. Aortic root dilatation occurred in 70% of patients; none had severe aortic regurgitation. Left ventricular function was normal. Exercise performance (85% of reference capacity, p = 0.02), maximal oxygen uptake (85%, p < 0.01) and peak heart rate (95%, p < 0.01) were decreased. Exercise electrocardiogram was normal as was rhythm status. Unfavourable outcomes on HRQOL were found for motor functioning and positive emotional functioning. Overall there were no significant differences between TGA/IVS and TGA/VSD. We conclude that at mid- to long-term follow-up after ASO, major events and re-interventions (6%) occur infrequently. Exercise capacity and maximal oxygen uptake are lower than those in a reference population, which could not be related to diminished ventricular function. Aortic root dilatation is frequent, irrespective of the anatomical subgroup. Severe aortic regurgitation or left ventricular dilatation was not found. The unfavourable health-related quality of life deserves further attention.

Carbon monoxide poisoning mimicking long-QT induced syncope.

Carbon monoxide (CO)poisoning is a rare cause of QT prolongation, and is therefore easily missed. The case of a patient with unexplained syncope and QT prologation on the electrocardiogram that turned out to be related to CO poisoning is reported here. In patients with QT prolongation, uncommon causes also should be looked for.

Virtual reality 3D echocardiography in the assessment of tricuspid valve function after surgical closure of ventricular septal defect.

BACKGROUND: This study was done to investigate the potential additional role of virtual reality, using three-dimensional (3D) echocardiographic holograms, in the postoperative assessment of tricuspid valve function after surgical closure of ventricular septal defect (VSD). METHODS: 12 data sets from intraoperative epicardial echocardiographic studies in 5 operations (patient age at operation 3 weeks to 4 years and bodyweight at operation 3.8 to 17.2 kg) after surgical closure of VSD were included in the study. The data sets were analysed as two-dimensional (2D) images on the screen of the ultrasound system as well as holograms in an I-space virtual reality (VR) system. The 2D images were assessed for tricuspid valve function. In the I-Space, a 6 degrees-of-freedom controller was used to create the necessary projectory positions and cutting planes in the hologram. The holograms were used for additional assessment of tricuspid valve leaflet mobility. RESULTS: All data sets could be used for 2D as well as holographic analysis. In all data sets the area of interest could be identified. The 2D analysis showed no tricuspid valve stenosis or regurgitation. Leaflet mobility was considered normal. In the virtual reality of the I-Space, all data sets allowed to assess the tricuspid leaflet level in a single holographic representation. In 3 holograms the septal leaflet showed restricted mobility that was not appreciated in the 2D echocardiogram. In 4 data sets the posterior leaflet and the tricuspid papillary apparatus were not completely included. CONCLUSION: This report shows that dynamic holographic imaging of intraoperative postoperative echocardiographic data regarding tricuspid valve function after VSD closure is feasible. Holographic analysis allows for additional tricuspid valve leaflet mobility analysis. The large size of the probe, in relation to small size of the patient, may preclude a complete data set. At the moment the requirement of an I-Space VR system limits the applicability in virtual reality 3D echocardiography in clinical practice.

Implantable cardioverter defibrillator implantation in children in The Netherlands.

UNLABELLED: To evaluate the indications, underlying cardiac disorders, efficacy and complications involved with implantable cardioverter-defibrillators (ICDs) in paediatric patients in The Netherlands, the records of all patients aged 18 years or younger who underwent ICD placement were reviewed retrospectively. Between January 1995 and September 2002, 23 patients (11 males, 12 females; median age 12 years, range 6 months to 16 years) underwent ICD implantation. The ICD was implanted for aborted sudden cardiac death ( n = 14), syncope ( n = 5) or for primary prevention of sudden cardiac death ( n = 4). Underlying cardiac disorders were electrical diseases ( n = 16), hypertrophic or dilated cardiomyopathy ( n = 4) and congenital cardiac malformations ( n = 3). Five patients had an epicardially placed ICD, while 18 underwent a transvenous approach. The generator was placed in an abdominal position in eight patients, whereas it was placed in the subpectoral region in 15. There was no early mortality. Median hospital stay was 5 days (range 2-30 days). Median follow-up time was 29 months (range 1 month to 7 years). Seven patients experienced an inappropriate shock after a median period of 7 months; five patients an appropriate shock after a median period of 3 months. The reasons for inappropriate shock were supraventricular tachycardia ( n = 1), sinus tachycardia ( n = 4) or T-wave sensing (n = 2). One patient with malignant long QT syndrome died of intractable arrhythmias and irreversible cardiomyopathy. Generator replacement was necessary in four patients after 28, 44, 51 and 54 months respectively. CONCLUSION: Implantable cardioverter-defibrillator implantation in paediatric patients is a safe procedure with a good medium-term outcome. The most serious problem is the occurrence of a significant number of inappropriate shocks.

Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect.

BACKGROUND: Left-sided atrioventricular valve regurgitation is the main indication for reoperation in patients after repair of both partial and complete atrioventricular septal defect. Until now, the timing for reoperation is difficult. We sought to determine the outcome of severe residual left-sided atrioventricular valve regurgitation, either medically treated or reoperation. In this regard risk factors were determined for severe residual left-sided atrioventricular valve regurgitation and reoperation, and the most appropriate strategy for patients with postoperative severe left-sided atrioventricular valve regurgitation was identified. METHODS: Retrospective review of clinical, operative, and echocardiographic data was performed. From 1990 until 2001 164 patients underwent correction of their atrioventricular septal defect. RESULTS: Five patients died in the immediate postoperative period, and 2 patients were lost to follow-up. During follow-up (median, 66 months; range, 9 months to 12 years), 30 patients (19%) had severe left-sided atrioventricular valve regurgitation. Sixteen patients had severe left-sided atrioventricular valve regurgitation in the immediate postoperative period; 4 of them showed spontaneous regression to near-normal valve function during follow-up. Fourteen patients exhibited left-sided atrioventricular valve regurgitation during follow-up; 8 of them remained stable with medication only. Fifteen of the 30 patients with severe left-sided atrioventricular valve regurgitation underwent reoperation. A significant risk factor for the development of severe left-sided atrioventricular valve regurgitation and reoperation was the presence of preoperative severe left-sided atrioventricular valve regurgitation. CONCLUSIONS: Severe left-sided atrioventricular valve regurgitation develops in a significant number of patients after correction of atrioventricular septal defect, and preoperative severe left-sided atrioventricular valve regurgitation is an important risk factor. Although reoperation usually results in good valve function, spontaneous regression after the immediate postoperative period is possible and should be given a fair chance.

Isolated unilateral absence of a pulmonary artery: a case report and review of the literature.

OBJECTIVE: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). BACKGROUND: Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear. METHODS: A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward. RESULTS: Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%. CONCLUSION: Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.