Catheter interventions in congenital heart disease without regular catheterization laboratory equipment: the chain of hope experience in Rwanda.

This report describes the feasibility and safety of cardiac catheterization in a developing country without access to a regular cardiac catheterization laboratory. The equipment used for imaging consisted of a monoplane conventional C-arm X-ray system and a portable ultrasound machine using the usual guidewires and catheters for cardiovascular access. In this study, 30 patients, including 17 children younger than 2 years and 2 adults, underwent catheterization of the following cardiac anomalies: patent ductus arteriosus (20 patients) and pulmonary valve stenosis (9 patients, including 2 patients with critical stenosis and 3 patients with a secundum atrial septal defect). Except for two cases requiring surgery, the patients were treated successfully without complications. They all were discharged from hospital, usually the day after cardiac catheterization, and showed significant clinical improvement in the follow-up evaluation. Cardiac catheterization can be performed safely and very effectively in a country with limited resources. If patients are well selected, this mode of treatment is possible without the support of a sophisticated catheterization laboratory.

Closure of a lateral baffle leak in a Fontan patient with an amplatzer PDA II AS plug.

Cyanosis after Fontan surgery or surgery for total cavopulmonary connection (TCPC), due to different types of communications (fenestration, venovenous collaterals or fistula), is not uncommon. We present the case of an 8-year-old girl presenting with increasing cyanosis during exercise 4 years after an intracardiac TCPC with lateral tunnel. Angiography showed a fistulous trajectory originating at the superior vena cava towards the base of the right atrial appendage. Due to the difficult anatomy in our patient, closure with conventional devices was not possible. We finally used a new Amplatzer PDA II AS plug to successfully close the fistula. In conclusion, closure of lateral baffle leak and device choice in case of difficult anatomy can be cumbersome. The new PDA II AS type plug can offer an elegant alternative for successful closure of some fistula.

Pulmonary function in children after surgical and percutaneous closure of atrial septal defect.

This study aimed to study differences in lung function after surgical and percutaneous atrial septal defect (ASD) closure. Several studies have demonstrated abnormalities of pulmonary function in adults and children with ASD. These abnormalities persist even a few years after correction. This study compared pulmonary function between patients who underwent ASD closure by surgery and those who had closure by device. This is the ideal pediatric population for studying changes in lung function caused by cardiopulmonary bypass or sternotomy. The 46 patients in this study were treated by percutaneous closure (group 1) or surgical closure (group 2) of ASD and then scheduled for pulmonary function testing an average of 5.8 years after ASD closure. The mean values of functional residual capacity, total lung capacity, and residual volume did not differ between the two groups. The surgical group showed a significant decrease in expiratory reserve volume (p < 0.04) and forced vital capacity (p < 0.03). Expiratory flow at 25, 50, and 75% of forced vital capacity did not differ between the two groups but was on the lower limit of normal in both groups. Percutaneous closure of ASD can minimize the side effects of surgical closure on lung function. Longitudinal lung function follow-up assessment after cardiac surgery is warranted to detect and measure restrictive abnormalities in this type of congenital heart disease and others.

Plastic bronchitis in a 5-year-old boy causing asystoly and fatal outcome.

Here we present the case of a 5-year-old boy who experienced a prehospital cardiac arrest after a period of wheezing, upper respiratory tract infection, and diarrhea. After successful resuscitation, ventilation was initially extremely difficult for no obvious reason. Various bronchodilatatory therapies were started with only limited result. Diagnosis of plastic bronchitis was made after bronchoscopy and at autopsy when therapy was withdrawn in view of the absence of cerebral circulation. A thickened basal membrane, a specific hallmark of asthma, was also present. Cardiac arrest in plastic bronchitis due to inflammatory and allergic disease is very rare. Pathological examination of the cast showed a mucinous cast with neutrophilic granulocytes, which is also seldom seen. This case illustrates that the diagnosis of plastic bronchitis can be extremely difficult if only minor respiratory symptoms occur. This resulted in a delayed diagnosis and fatal outcome.

Congenital heart disease in 111 225 births in Belgium: birth prevalence, treatment and survival in the 21st century.

AIM: To investigate the birth prevalence, treatment modalities and short-term survival of children with congenital heart disease who were born in 2002. METHODS: We undertook a retrospective review of medical records of all patients who were born in 2002, and were diagnosed, treated and/or followed-up in one of the seven-paediatric cardiology programmes in Belgium. RESULTS: In 111 225 births, 921 children with congenital heart disease were detected, yielding a birth prevalence of 8.3 per 1000. The most frequently occurring conditions were ventricular septal defects (VSDs) (33%), ostium secundum atrial septal defects (18%) and pulmonary valve abnormalities (10%). Thirty-nine percent of the children either had a cardiosurgical operation or catheter intervention. In this study, 4% of the children died. The actuarial survival at 6 months and 1 year of age was 97% and 96%, respectively and remained stable after then. Compared to other heart defects, mortality was higher in univentricular physiology, pulmonary atresia with VSD, left ventricle outflow obstruction and tetralogy of Fallot. CONCLUSION: Survival of congenital heart disease is excellent and continued to improve in the early 21st century. New therapeutic options are increasingly used. This study provides baseline data for the longitudinal follow-up of this cohort.

Transthoracic echocardiography guidance of transcatheter atrial septal defect closure in children.

Transesophageal echocardiography (TEE) guidance is part of interventional closure of secundum atrial septal defect (ASD) in children and adults. However, sometimes TEE is impossible for technical or anatomical reasons. If available, intracardiac echocardiography can be used, but especially in children, transthoracic echocardiography (TTE) can be an easy, safe, and cheap alternative. We report two cases in which TEE was replaced by TTE during percutaneous ASD closure. In the first case VACTERL association with a surgically repaired tracheoesophageal fistula was a relative contraindication to TEE. In the second case, technical failure of the transesophageal probe occurred during the procedure. In both cases the ASD was successfully closed with an atrial septal occluder device under TTE guidance. Using TTE can be sufficient and safe in children with good imaging windows, especially from subcostal views.

Unsuccessful resuscitation of a preterm infant due to a pneumothorax and a masked tension pneumopericardium.

Pneumopericardium is the least common form of air leak in infants. A tension pneumopericardium is even more infrequent but associated with a very high mortality rate. We describe the case of an unsuccessful resuscitation in a preterm infant due to a pneumothorax and tension pneumopericardium. Despite relatively mild pressure ventilation the patient developed massive pulmonary interstitial emphysema. The extra-alveolar air spread from the interstitium towards the mediastinal space (Macklin effect) and caused a pneumothorax and pneumopericardium, which evolved towards a tension pneumopericardium after a traumatic mechanical procedure. The infant deteriorated acutely. Despite prompt pleural drainage there were no signs of recovery at any time. Postmortal examination revealed a tension pneumopericardium and massive interstitial pulmonary emphysema, which was not obvious on radiographical investigation. In cases of acute deterioration of a ventilated neonate, one should always rule out pneumothorax. If the patient does not recover after pleural drainage and cardiac resuscitation a (tension) pneumopericardium should be considered.

Left ventricular non-compaction with Ebstein anomaly attributed to a TPM1 mutation.

Left ventricular non-compaction (cardiomyopathy) (LVN(C)) is a rare hereditary cardiac condition, resulting from abnormal embryonic myocardial development. While it mostly occurs as an isolated condition, association with other cardiovascular manifestations such as Ebstein anomaly (EA) has been reported. This congenital heart defect is characterized by downward displacement of the tricuspid valve and leads to diminished ventricular size and function. In an autosomal dominant LVN(C) family consisting of five affected individuals, of which two also presented with EA and three with mitral valve insufficiency, we pursued the genetic disease cause using whole exome sequencing (WES). WES revealed a missense variant (p.Leu113Val) in TPM1 segregating with the LVN(C) phenotype. TPM1 encodes α-tropomyosin, which is involved in myocardial contraction, as well as in stabilization of non-muscle cytoskeletal actin filaments. So far, LVN(C)-EA has predominantly been linked to pathogenic variants in MYH7. However, one sporadic LVN(C)-EA case with a de novo TPM1 variant has recently been described. We here report the first LVN(C)-EA family segregating a pathogenic TPM1 variant, further establishing the association between EA predisposition and TPM1-related LVN(C). Consequently, we recommend genetic testing for both MYH7 and TPM1 in patients or families in which LVN(C)/non-compaction and EA coincide.

Female children and adolescents with type 1 diabetes have more pronounced early echocardiographic signs of diabetic cardiomyopathy.

OBJECTIVE: This study was designed to assess whether children and adolescents with type 1 diabetes have early echocardiographic signs of subclinical cardiac dysfunction and whether sex, state of metabolic control, and diabetes duration are of influence. RESEARCH DESIGN AND METHODS: Systolic and diastolic blood pressure in supine and upright positions and echocardiographic parameters, including tissue Doppler measurements of the septal mitral annulus, were evaluated in 80 children and adolescents with stable type 1 diabetes and 52 age- and sex-matched control subjects. A possible correlation was examined for age, sex, HbA(1c), and diabetes duration with univariate and multivariate regression analysis. RESULTS: Female diabetic patients showed significantly larger left ventricular wall dimensions (left ventricular posterior wall in diastole 0.54 +/- 0.08 vs. 0.48 +/- 0.11 cm) and signs of significant diastolic filling abnormalities on conventional and tissue Doppler echocardiography (mitral valve-atrial contraction velocity 0.47 +/- 0.12 vs. 0.40 +/- 0.09 m/s; tricuspid valve-atrial contraction velocity 0.35 +/- 0.09 vs. 0.30 +/- 0.07 m/s; early filling velocity/myocardial velocity during early filling 7.15 +/- 1.47 vs. 6.17 +/- 1.07; isovolumetric relaxation time [IVRT] 66 +/- 8 vs. 58 +/- 8 ms) compared with female control subjects, suggesting delayed myocardial relaxation. Male diabetic patients only differed significantly from their control subjects for IVRT (66 +/- 9 vs. 59 +/- 8 ms). The measured parameters showed an expected correlation with age and BMI standard deviation scores in the control group. This correlation was significantly weaker in the diabetic population; only a weak influence was found for diabetes duration and glycosylated hemoglobin levels. CONCLUSIONS: Young diabetic patients already have significant changes in left ventricular dimensions and myocardial relaxation, with the girls clearly being more affected. Tissue Doppler proved to have additional value in the evaluation of ventricular filling in this population. Almost no correlation was found for diabetes duration and HbA(1c) with the cardiovascular changes.

Late post-repair ventricular function in patients with origin of the left main coronary artery from the pulmonary trunk.

This study investigated ventricular function in postoperative patients with abnormal origin of the the left main coronary artery from the pulmonary trunk using ultrasound-based deformation imaging. Although radial left ventricular deformation parameters were completely normalized, longitudinal deformation remained abnormal.

Health status, functional abilities, and quality of life after the Mustard or Senning operation.

BACKGROUND: Life expectancy of patients who underwent atrial switch operation for the transposition of the great arteries is relatively good. However, many patients are faced with residua and sequelae, which may hamper their functioning and quality of life. This multicenter study assessed the perceived health status, functional abilities, and quality of life in long-term survivors of the Mustard or Senning operation. METHODS: A group of 89 patients (58% male) were selected from four tertiary care centers, consisting of 37 Mustard and 52 Senning operation patients. Perceived health status was measured using a linear analog scale. The educational level, employment status, New York Health Association classification, ability index, and Baecke questionnaire were used to evaluate functional abilities. Quality of life was assessed with a linear analog scale, the Satisfaction with Life Scale, and the Congenital Heart Disease-TNO/AZL Adult Quality of Life. RESULTS: Patients reported good to very good perceived health, functional capacities, and quality of life. The responses of patients with complex transposition were equivalent to those of patients with simple transposition. The most dominant concerns reported by survivors of the Mustard and Senning operations were experiences about physical limitations and worries about a current or future job or income. CONCLUSIONS: Long-term survivors after atrial inflow correction demonstrated favorable perceived health, functional status, and quality of life; these conditions were, to a large extent, comparable with the status of the general population. These outcome variables were not negatively affected by the complexity of the transposition.

Persistent ductus arteriosus in the adult: clinical features and experience with percutaneous closure.

Persistent ductus arteriosus in the adult carries a risk for becoming symptomatic with dyspnoea or palpitations, developing infective endarteritis, and finally for developing pulmonary hypertension with ensuing heart failure morbidity and mortality. Surgical closure is considered more and more to be outdated as first choice treatment since percutaneous closure is a safe and effective alternative. Only the Eisenmenger syndrome remains a clear contra-indication for closure, whether surgical or interventional. We report on our own experience in nine patients with persistent ductus arteriosus including eight cases who underwent percutaneous closure and one patient with Eisenmenger syndrome. In addition, our experience with clinical features and therapeutic modalities is related to the literature.

Late cardiotoxicity after low dose of anthracycline therapy for acute lymphoblastic leukemia in childhood.

INTRODUCTION: Late cardiotoxicity is a known complication of anthracycline therapy but the long-term effects of low cumulative doses are not well documented. We studied late cardiotoxicity in survivors of childhood acute lymphoblastic leukemia (ALL) treated with low anthracycline doses 10 to 20 years earlier. METHODS: Seventy-seven ALL survivors who received a cumulative anthracycline dose <250 mg/m² and were at least 10 years after treatment were evaluated for signs of clinical heart failure. Cardiac function was assessed by echocardiography including tissue Doppler measurements of the septal mitral annulus in 37 ALL survivors 10.6-18.3 years (median 13.3 years) after anthracycline treatment with cumulative doses of 180 (n = 19) or 240 mg/m² (n = 18). The control group consisted of 30 healthy volunteers matched for age, sex, BSA, and BMI. RESULTS: No clinical relevant cardiotoxicity was found. Left ventricular shortening fraction (SF) was significantly reduced in male ALL survivors. Three of the 19 male ALL survivors had an SF below 30%. Male ALL survivors showed a significantly lower early filling velocity to atrial contraction velocity ratio but myocardial velocity during early filling was comparable between patients and controls. ALL survivors had a significantly longer isovolumetric relaxation time (IVRT). Thirty percent of the ALL survivors have an abnormal IVRT compared to the normal range of the controls. CONCLUSION AND IMPLICATIONS FOR CANCER SURVIVORS: At a median of 13.3 years after exposure to cumulative doses of anthracyclines of 180 or 240 mg/m², no clinical relevant cardiotoxicity was found but subclinical cardiac abnormalities were present in 30% of the patients.

Effective cardioverter defibrillator implantation in children without thoracotomy: a valid alternative.

The implantation method for a cardioverter defibrillator in children is poorly standardized because of obvious features related to size and predisposing cardiac disease in children presenting with malignant ventricular arrhythmia. We propose an alternative method of implanting a cardioverter defibrillator without the need for associated thoracotomy, based on the subxiphoidal insertion of an epicardial bipolar ventricular pacing and sensing lead, an active can placed in the abdomen, and a subcutaneous array tunneled along the left thoracic wall as a shock electrode. This technique offers the advantage of an effective and minimally invasive implantable cardioverter defibrillator with wide applicability for children, independent of their size and cardiac status.

Pleural effusions, water balance mediators and the influence of lisinopril after completion Fontan procedures.

OBJECTIVE: To investigate whether the duration of pleural drainage after Fontan completion operations can be influenced by postoperative lisinopril administration or can be related to water balance hormone levels. METHODS: In a prospective trial 21 patients scheduled for total cavopulmonary connection were randomized into two groups, with group I receiving lisinopril postoperatively, and group II receiving no angiotensin converting enzyme inhibitor. Plasma levels of antidiuretic hormone, renin and aldosteron were analyzed preoperatively and at four-time points postoperatively. Groups were comparable for age, preoperative saturation and pulmonary pressure, cardiopulmonary bypass time, cross-clamp time and preoperative hormone levels. RESULTS: Mean duration of pleural drainage was comparable for both groups (group I: 9.6+/-8 days vs group II: 10+/-7 days; p=0.78). The hormone profiles in each group changed significantly at 1 and 24h postoperatively compared to preoperative values (p<0.05), with no significant differences between the treatment groups. The duration of pleural drainage correlated significantly with hormone levels at 24h postoperatively, and with longer bypass times. At multivariate analysis only aldosteron and antidiuretic hormone level at 24h came out as factors reaching significance for prolonged pleural drainage. CONCLUSIONS: The Fontan completion induces significant changes in the levels of antidiuretic hormone, aldosteron and renin. Prolonged drainage correlates significantly with elevated levels of aldosteron, renin and antidiuretic hormone postoperatively, and with longer bypass time, but is not influenced by lisinopril. The eventual adjunct therapy with aldosteron antagonists warrants further study.