Tachyarrhythmia in patients with congenital heart disease: inevitable destiny?

The prevalence of patients with congenital heart disease (CHD) has increased over the last century. As a result, the number of CHD patients presenting with late, postoperative tachyarrhythmias has increased as well. The aim of this review is to discuss the present knowledge on the mechanisms underlying both atrial and ventricular tachyarrhythmia in patients with CHD and the advantages and disadvantages of the currently available invasive treatment modalities.

Release of growth-differentiation factor 15 and associations with cardiac function in adult patients with congenital heart disease.

BACKGROUND: Growth-differentiation factor-15 (GDF-15), a cytokine with broad cardiac and non-cardiac activity, has diagnostic and prognostic value in various diseases, including heart failure. We aimed to investigate the release of GDF-15 in adults with congenital heart disease (ConHD), and assess the association with cardiac function and functional capacity. METHODS: A total of 587 consecutive adults with ConHD (median age 33 [IQR 25-41] years, 59% men, and 90% NYHA I) underwent electrocardiography, echocardiography, venepuncture and were seen by a cardiologist. A subset of 143 patients underwent bicycle ergometry on the same day. RESULTS: Median plasma GDF-15 was 618 [IQR 487-867] ng/L. In 87 patients (15%), GDF-15 was above the reference value of normal (1109 ng/L). GDF-15 levels were higher in older patients (r=0.367, p<0.001). GDF-15 was higher in patients with elevated pulmonary pressure (median 1114 [IQR 796-2320 ng/L) than in patients with normal pulmonary pressure (median 606 [IQR 481-826] ng/L, p<0.001). GDF-15 correlated positively with NT-proBNP (r=0.445, p<0.001). In multivariate analysis adjusting for age, sex, and NT-proBNP, hs-TnT and hs-CRP, GDF-15 above the reference value was associated with NYHA class (odds ratio for NYHA≥II: 3.5 [95% CI 1.8-6.8], p<0.001), and decreased exercise capacity (odds ratio for workload >85%:0.2 [95% CI 0.06-0.8], p=0.018), but not with systolic ventricular function or ECG rhythm. CONCLUSIONS: GDF-15 is elevated in a substantial number of patients and higher in those with elevated pulmonary pressures, regardless of underlying congenital diagnosis. GDF-15 is associated with NYHA class, NT-proBNP and exercise capacity, suggesting the marker has diagnostic and potential prognostic value in adults with ConHD.

Transcatheter closure of a traumatic ventricular septum defect resulting from a stab wound.

A 25-year-old man with a ventricular septal defect resulting from a stab wound to his chest was admitted to our hospital. Because of extensive comorbidity and favourable location, transcatheter closure with an Amplatzer device was preferred over surgical repair. Ventricular septal defects are an uncommon complication of cardiac trauma, but when they do occur from this cause, they often have more dramatic consequences. Transcatheter closure is an attractive, less-invasive alternative in patients with increased surgical risk, multiple previous surgical interventions, or poorly accessible defects.

Transcatheter device closure of atrial septal defects in patients aged 40 years and older.

OBJECTIVE: To evaluate the safety and effect of transcatheter device closure in ostium secundum atrial septal defects (ASD II) in patients aged 40 years and older. METHODS: Retrospective single-centre study concerning 47 consecutive ASD transcatheter occlusion procedures performed between January 1999 and December 2008. Electrocardiography, echocardiography and clinical assessments of the patients were conducted pre- and post-intervention and at follow-up. RESULTS: Of the 130 patients who were referred for interventional ASD closure, 47 were 40 years and older and all of them actually had the device inserted. There were no major complications during the intervention. Mean follow-up time was 15±15 months. During follow-up, three patients needed surgical reintervention because of device embolisation (n=2) or dislocation (n=1). Of the patients with severe right ventricular (RV) dilatation, more than half (58%) had no or mild dilatation at last follow-up. Reduction of RV dilatation was not related to age. Pulmonary hypertension was present in 63% before the procedure and was reduced to 38% at follow-up. NYHA class improved in all age groups, also in patients over 60 years of age. In two of the three patients who died during follow-up, no cause of death could be established, but both had responded well to treatment regarding the echocardiographic and clinical findings. CONCLUSION: Transcatheter device closure of ASD is a successful and effective treatment, also for patients aged 40 years and older. Patients showed regression of right ventricular enlargement and an improvement in functional class. (Neth Heart J 2010;18:537-42.).

Endovascular stenting for aortic (re)coarctation in adults.

Objectives. Stenting for native and recurrent coarctation (COA) in adults has become an important therapeutic strategy. In this prospective observational study we evaluated the intermediate-term outcome of stent implantation for either native or recurrent (re)COA in adults.Methods. All adults who underwent stent implantation in our institution between January 2003 and December 2008 were included. Diagnosis of (re)COA was based upon a combination of clinical signs, noninvasive imaging or invasive gradient measurements. NuMED stents were implanted under general anaesthesia.Results. Twenty-four patients (50% male) underwent stent implantation for native (n=6) or reCOA (n=18) at a median age of 36 (18 to 60) years. There was significant improvement in pre- versus post-stent invasive systolic gradient (19 vs. 0 mmHg, p<0.001) and COA diameter (10 vs. 16 mm, p<0.001). Acute complications (12.5%) included death due to aortic rupture despite immediate successful coverage with a covered stent (n=1) and groin haematoma (n=2). During a median follow-up period of 33 (8 to 77) months (n=22), late complications occurred in three patients (13.6%): stent migration to the ascending aorta (n=1), pseudoaneurysm at the site of the initial stent (n=1), and occluded external iliac artery (n=1). Stent implantation did not reduce the need for antihypertensive medication or blood pressure at last follow-up. Conclusion. COA stenting results in a significant gradient decrease and increase in vessel diameter. However, serious complications do occur and hypertension remains in the majority of patients. (Neth Heart J 2010;18:430-6.).

Congenital valvular aortic stenosis: limited progression during childhood.

OBJECTIVE: Congenital valvular aortic stenosis is a common congenital heart malformation. The rate of progression in childhood, however, remains to be established. We assessed the progression of peak aortic velocity before intervention as well as the frequency of intervention in paediatric patients with isolated congenital valvular aortic stenosis. METHODS: A retrospective cohort study was performed in 245 consecutive patients with aortic stenosis. Both clinical and echocardiographic data were obtained. RESULTS: Over a period of 9.0 (SD 5.2) years (range 0.1-19.4), the mean annual increase in peak systolic velocity was 0.04 m/s/year (95% CI 0.028 to 0.056 m/s/year; p<0.001) as shown by ANOVA. 40 patients underwent a cardiac intervention shortly after their first echocardiogram. Another 33 patients underwent intervention during follow-up. Interventions were performed significantly more often in patients diagnosed at a younger age and/or with a higher peak velocity at diagnosis (p<0.001). Mortality was considerable in those diagnosed in infancy (5-year survival rate of 73% (SD 9%), whereas it was nearly absent in patients diagnosed after infancy. Most patients who died during infancy had progressive left ventricular dysfunction despite adequate relief of left ventricular outflow obstruction. CONCLUSIONS: Valvular aortic stenosis in the paediatric age group usually has a good prognosis beyond the neonatal period. Progression over time is usually limited, although a considerable proportion of patients need intervention shortly after initial diagnosis. Mortality, except for the neonatal age group, is nearly absent.

Baffle puncture guided by transoesophageal echocardiography in a patient with dextrocardia and Mustard correction.

A baffle puncture is a challenging procedure but can be safely done using direct visualization of the region of interest. To our knowledge, however, it has never been performed in a patient with dextrocardia. We present a 62-year-old male with dextrocardia, right isomerism, congenitally corrected transposition of the great arteries, persistent left-sided superior and inferior caval veins, atrial septum defect, and pulmonary valve stenosis. The atrial septum defect was surgically closed with a Teflon patch, a variant Mustard operation was performed, and also a prosthetic tricuspid valve was implanted. The patient developed multiple episodes of atrial tachycardia leading to acute heart failure on many occasions. An electrophysiological study was undertaken in order to create a bi-atrial electro-anatomical map. Owing to the presence of a prosthetic tricuspid valve, the femoral venous access was used and a baffle puncture was performed using continuous monitoring with fluoroscopy and transoesophageal echocardiography (TEE). The baffle puncture was successful and the tachycardia was ablated in the systemic venous atrium. To our knowledge, we present the very first case report demonstrating a successful baffle puncture in a patient with dextrocardia and Mustard correction. Direct imaging using TEE seems to be a very useful tool for guiding the puncture.

Patent ductus arteriosus in adults: Case report and review illustrating the spectrum of the disease.

Patent ductus arteriosus (PDA) is a congenital heart defect in which the ductus arteriosus, a vascular structure between the pulmonary artery and the aorta that normally closes shortly after birth, remains open. We present two cases of adults with PDA. A 28-year-old lady had a small PDA without any symptoms or left heart overload. The PDA was closed for endocarditis prevention using a coil. In a 37-year-old lady with palpitations, collapse, dyspnoea and left heart overload caused by a large PDA, percutaneous closure of the PDA was performed with an Amplatzer device. Transcatheter closure has been established to be the method of choice for treating a PDA in adults. (Neth Heart J 2008;16:255-9.).

Intracardiac echocardiographic guidance for hemodynamic assessment in a patient with congenital abnormalities and a prosthetic aortic valve.

In this case report, we present the use of intracardiac echocardiography (ICE) for guiding the cardiac catheterization and subsequent hemodynamic investigation in an unusual patient case with multiple congenital abnormalities (bicuspid aortic valve, left cervical aortic arch, two aortic coarctations) and two aortic valve replacement operations in the past. The ICE catheter (AcuNav) permitted us to accurately and safely puncture the interatrial septum and place the Swan-Ganz catheter in the left ventricle; additionally, visualization of the aortic coarctation in the ascending aorta was also achieved.

[Closure of a ventricular septum defect in a 4-year-old boy during percutaneous heart catheterisation]. / Sluiting van een ventrikelseptumdefect bij een 4-jarige jongen tijdens percutane hartkatheterisatie.

A 4-year-old boy in whom a muscular ventricular septal defect (VSD) had been diagnosed at birth presented with limited exercise tolerance and mild cardiomegaly. The moderately large defect was closed with an umbrella device via percutaneous heart catheterisation. Thereafter, his symptoms disappeared and the dimensions of the left heart returned to normal. A VSD occurs in 1.5-3.5 per 1,000 live births. In symptomatic patients with a medium or large-sized VSD, surgical closure is indicated to prevent the development of a fixed pulmonary resistance hypertension, ventricular dysfunction and the risk of endocarditis. Depending on the size and localisation of the defect, closure with an umbrella device may be chosen. The initial results have been promising in children. The safety and efficacy in the long term are still unknown.

Slide tracheoplasty for congenital tracheal stenosis.

BACKGROUND/PURPOSE: The aim of this study was to add to the experience of slide tracheoplasty in infants with congenital tracheal stenosis (CTS), to review the recent literature on this subject, and to evaluate the diagnostic workup in infants with CTS. METHODS: A retrospective review of 3 infants with CTS treated with slide tracheoplasty was conducted at our institution. They all underwent bronchoscopy, tracheobronchography, and echocardiography. Therapy consisted of slide tracheoplasty and simultaneous correction of associated vascular malformations. RESULTS: A pulmonary artery sling was missed initially on esophagography in 2 patients. Stridor became worse in 2 patients after tracheobronchography. After surgical therapy, 2 patients survived and were discharged 12 days after surgery. Both are without symptoms at follow-up of 12 and 20 months. One patient had inoperable restenosis and died 27 days after the operation. CONCLUSIONS: Esophagography is not a reliable tool to depict associated vascular anomalies for patients with CTS. According to the literature, computer tomography has become more reliable to depict the tracheobronchial tree and is useful to elucidate associated vascular anomalies as well. Compared with other surgical techniques for infants with CTS reported in the literature, slide tracheoplasty has fewer postoperative complications and comparable survival rates.

Follow-up after surgical closure of congenital ventricular septal defect.

OBJECTIVES: The purpose of this retrospective study was to assess long-term outcome of children after surgical closure of a ventricular septal defect (VSD). MATERIAL AND METHODS: Between January 1992 and December 2001 a consecutive series of 188 patients (100 females) were operated for closure of a VSD. Temporary tricuspid valve detachment (TVD) was applied in 46 patients (24%) to enhance exposure of the defect using transatrial approach. Pre-operative baseline characteristics showed that the detached group was younger (0.79+/-1.8 vs 2.1+/-3.5 years, p=0.002) and had a lower weight (6.5+/-6.4 vs 10.0+/-11.0 kg, p=0.009). RESULTS: There was no difference in cross-clamp time (temporary TVD 36.2+/-11.3 vs non-temporary TVD 33.6+/-13.1 min, p=0.228). Postoperative echocardiography showed that 67 patients (36%) had trivial/minimal regurgitation, 10 patients (22%) from the temporary TVD group vs 57 patients (40%) from the non-detached group (p=0.02). There was no tricuspid stenosis. Hospital mortality comprised two patients (1%). One patient died due to a pulmonary hypertensive crisis and one in relation to an acute patch dehiscence for which an emergency reoperation was necessary. At first postoperative echocardiography no shunting was detected in 113 patients, trivial shunting in 73 and significant shunting in none. Multivariate logistic regression analysis revealed that weight at operation was a predictive factor for the occurrence of residual shunting (OR 0.95, C.I. 0.91-0.99). One patient with conduction disturbances needed a permanent DDD-pacemaker. Three patients were lost to follow-up. Mean follow-up time was 2.6 years (range 0.1-9.4). During follow-up no reoperations were necessary for closing a residual VSD. One patient died 7 months postoperative due to a bronchopneumonia. During follow-up in 37 (51%) of the 73 patients the trivial shunting disappeared spontaneously at a median time of 3.9 years. According to actuarial analysis all trivial shunting had disappeared at 8.4 years. CONCLUSION: Trivial residual shunting disappeared spontaneously at a median follow-up time of 3.9 years. During follow-up no patient needed to be reoperated for residual VSD. TVD proved to be a safe method to enhance the exposure of a VSD.

Transcatheter pulmonary valve perforation and balloon dilatation in neonates with pulmonary atresia and intact ventricular septum.

BACKGROUND: Pulmonary atresia and intact ventricular septum is characterised by a great morphological variety. Treatment is not uniform. OBJECTIVE: To evaluate our experience with transcatheter valvotomy and balloon dilatation in neonates with pulmonary atresia and intact ventricular septum. DESIGN: Retrospective. METHODS: Between January 1997 and September 2000 five neonates with pulmonary atresia and intact ventricular septum underwent transcatheter valvotomy and balloon dilatation. RESULTS: The catheter intervention was performed at a mean age of 27 days (range 3-95 days). The atretic pulmonary valve was successfully perforated in all neonates. Subsequent balloon dilatation was successful in four neonates. Balloon dilatation was unsuccessful in one patient, who underwent an elective surgical valvotomy of the pulmonary valve after five days. Three patients needed a modified Blalock-Taussig shunt after a mean of 23 days. Four patients required repeated balloon dilatation after a mean of 227 days. Mean follow-up was 2.7 years (range 1-5 years). CONCLUSIONS: Transcatheter perforation of the pulmonary valve membrane and balloon dilatation is a good, safe initial therapy in selected neonates with pulmonary atresia and intact ventricular septum. This procedure can prevent open-heart surgery in these patients in the first months of life.

Pacemaker dislocation--truly ectopic activation necessitating surgical treatment.

Intra-abdominal migration of a generator from an epicardial pacemaker system is a rare but potentially life-threatening complication. We report on a case of a 2-year-old child in whom the generator silently migrated from the sheath of the rectus abdominis muscle in the upper abdominal wall down into the peritoneal cavity. We advise that if there is any evidence of intra-abdominal migration, the generator should be properly repositioned without delay.

Minimum bandwidth requirements for recording of pediatric electrocardiograms.

BACKGROUND: Previous studies that determined the frequency content of the pediatric ECG had their limitations: the study population was small or the sampling frequency used by the recording system was low. Therefore, current bandwidth recommendations for recording pediatric ECGs are not well founded. We wanted to establish minimum bandwidth requirements using a large set of pediatric ECGs recorded at a high sampling rate. METHODS AND RESULTS: For 2169 children aged 1 day to 16 years, a 12-lead ECG was recorded at a sampling rate of 1200 Hz. The averaged beats of each ECG were passed through digital filters with different cut off points (50 to 300 Hz in 25-Hz steps). We measured the absolute errors in maximum QRS amplitude for each simulated bandwidth and determined the percentage of records with an error >25 microV. We found that in any lead, a bandwidth of 250 Hz yields amplitude errors <25 microV in >95% of the children <1 year. For older children, a gradual decrease in ECG frequency content was demonstrated. CONCLUSIONS: We recommend a minimum bandwidth of 250 Hz to record pediatric ECGs. This bandwidth is considerably higher than the previous recommendation of 150 Hz from the American Heart Association.

Is there a place for pediatric valvotomy in the autograft era?

OBJECTIVE: Valvotomy and the autograft procedure are the most common surgical treatment options for children with valvular aortic stenosis. We evaluated the results of these surgical procedures in our institution. METHODS: Retrospective analysis was done of all patients presenting with aortic stenosis and operated upon before the age of 18. In 11 patients a valvotomy was performed and in 36 an autograft procedure. RESULTS: There was no hospital mortality. Mean follow-up in the valvotomy group was 4.8 years (SD 3.3), in the autograft group 4.5 years (SD 3.3). During follow-up one patient died suddenly 2 months after valvotomy. Two patients in the autograft group died (not valve-related). After valvotomy three patients underwent a balloon valvotomy, in one followed by an autograft procedure and one patient had a repeat valvotomy. In the autograft group one patient was reoperated for severe aortic regurgitation and moderate pulmonary stenosis. At last echocardiography after valvotomy (eight remaining patients) in only two patients (25%) no aortic stenosis or regurgitation was present. In the remaining six patients aortic stenosis is mild in two and moderate in three, including one with moderate aortic regurgitation. In one patient without stenosis, moderate aortic regurgitation was seen. No pulmonary stenosis or regurgitation is present. Echocardiography after autografting (33 remaining patients) showed no aortic stenosis. Aortic regurgitation was mild in seven patients, moderate in two, severe in one. Pulmonary stenosis was present in two patients (16%). Pulmonary regurgitation was mild in three patients and moderate in one. CONCLUSIONS: In selected patients with valvular aortic stenosis who are beyond infancy, valvotomy may be adequate and may postpone further surgery for a significant length of time. After valvotomy the main problem is residual aortic stenosis while after autografting a shift occurs to aortic regurgitation and problems related to the pulmonary valve. Careful clinical and echocardiographic follow-up is therefore warranted in young patients after the autograft procedure.

New normal limits for the paediatric electrocardiogram.

AIMS: Previous studies that determined the normal limits for the paediatric ECG had their imperfections: ECGs were recorded at a relatively low sampling rate, ECG measurements were conducted manually, or normal limits were presented for only a limited set of parameters. The aim of this study was to establish an up-to-date and complete set of clinically relevant normal limits for the paediatric ECG. METHODS AND RESULTS: ECGs from 1912 healthy Dutch children (age 11 days to 16 years) were recorded at a sampling rate of 1200 Hz. The digitally stored ECGs were analysed using a well-validated ECG computer program. The normal limits of all clinically relevant ECG measurements were determined for nine age groups. Clinically significant differences were shown to exist, compared with previously established normal limits. Sex differences could be demonstrated for QRS duration and several amplitude measurements. CONCLUSIONS: These new normal limits differ substantially from those commonly used and suggest that diagnostic criteria for the paediatric ECG should be adjusted.