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Journal: Pediatric cardiology


The association of tetralogy of Fallot with a partial anomalous pulmonary vein is rare. Although this combination is generally treated with surgery, in this paper we present the case of an 8-year-old boy whose anomalous venous drainage was successfully closed with an Amplatzer vascular plug after a total correction of a tetralogy of Fallot. The patient was asymptomatic at his last follow-up.

Concepts: Blood, Lung, Heart, Blood vessel, The Association, Vein, Tetralogy of Fallot, Total anomalous pulmonary venous connection


Controversial data exist about the long-term results of aortic coarctation (AC) repair. This study explored the prevalence and predictors of left ventricular (LV) hypertrophy, late hypertension, and hypertensive response to exercise in 48 subjects (age, 15.1 ± 9.7 years) currently followed in the authors' tertiary care hospital after successful AC repair. Data on medical history, clinical examination, rest and exercise echocardiography, and ambulatory blood pressure monitoring were collected. The time from AC repair to follow-up evaluation was 12.9 ± 9.2 years. The prevalence of LV hypertrophy ranged from 23 to 38 %, based on the criteria used to identify LV hypertrophy, and that of concentric geometry was 17 %. One sixth of the patients without residual hypertension experienced late-onset hypertension. One fourth of those who remained normotensive without medication showed a hypertensive response to exercise. Age at AC repair was the strongest independent predictor of LV hypertrophy, defined using indexation either for body surface area (odds ratio [OR], 1.03; p = 0.0090) or for height (OR 1.02; p = 0.029), and it was the only predictor of late hypertension (OR 1.06; p = 0.0023) and hypertensive response to exercise (OR 1.09; p = 0.029). The risk of LV hypertrophy was 25 % for repair at the age of 3.4 years but rose to 50 and 75 % for repair at the ages of 5.9 and 8.4 years, respectively. Similar increases were found for the risk of late-onset hypertension and hypertensive response to exercise. A considerable risk of LV hypertrophy, late hypertension, and hypertensive response to exercise exists after successful AC repair. Older age at intervention is the most important predictor of these complications.

Concepts: Myocardial infarction, Atherosclerosis, Hypertension, Cardiology, Stroke, Blood pressure, Left ventricular hypertrophy, Cortisol


Rheumatic heart disease (RHD) is a chronic condition characterized by fibrosis and scarring of the cardiac valves and damage to the heart muscle, leading to congestive heart failure and death. This prospective cohort study was conducted to investigate the possible relation between the levels of serum adhesion molecules and acute rheumatic fever (ARF) carditis, valvular insult severity, and residual valvular lesion after improvement of rheumatic activity. Serum levels of intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), and E-selectin were assayed by enzyme-linked immunoassay (ELISA) for 50 children with ARF carditis during activity and after improvement and for 50 healthy children as control subjects. After the acute attack, patients were followed up regularly to detect residual valvular lesion. The serum levels of these adhesion molecules were significantly higher in the patients than in the control group (p < 0.001). In addition, the levels of serum adhesion molecules were significantly higher in the patients with severe carditis than in the patients with mild to moderate carditis (p < 0.001). Among the severe carditis group, the level of serum adhesion molecules was significantly higher among the patients with heart failure than among the patients without heart failure (p < 0.001). Furthermore, the pretreatment serum levels of ICAM-1 and VCAM-1 were significantly higher among the patients with residual valve lesion (p = 0.002) than among those without the lesion (p < 0.001). The cutoff values were obtained for the prediction of residual valvular lesion (ICAM-1, >1,032.3 μg/ml; VCAM-1, >3,662.3 μg/ml; E-selectin, >104.8 μg/ml). Finally, by combining the three adhesion molecules in a single prediction model, the highest area under the curve (AUC) ± standard error (SE) was obtained (0.869 ± 0.052), and the positive likelihood ratio for having a residual valvular lesion was increased (17.33). Levels of serum adhesion molecules could predict residual valvular lesions in RHD patients. The authors recommend that the serum level of adhesion molecules be measured in all cases of ARF carditis.

Concepts: Cardiology, Heart, Rheumatic fever, Cell adhesion molecule, Rheumatism, Intercellular adhesion molecule, VCAM-1, Heart valve


The change in clinical status of patients status post-Fontan surgery who relocated from low (<1,500 feet) to moderate (>4,000 feet) altitude was assessed. Cardiology databases were queried for patients meeting inclusion criteria. The clinical records of these patients for the 6 months before and 6 months after relocation were then reviewed. Between 1990 and 2010, 16 patients relocated to moderate altitude. All patients developed a new cardiac-related adverse event within 6 months of relocation. A decrease in New York Heart Association functional classification occurred in 15 (94 %) patients, and 11 (69 %) of these required hospitalization. Clinical deterioration at higher altitude is common in patients who have undergone Fontan surgery. Physicians at lower altitudes should caution these patients about the potential risks of relocation to moderate altitude.

Concepts: Medicine, Patient, Hospital, Surgery, Physician, Pharmaceutical industry, Clinical research, Fontan procedure


Barth syndrome (BTHS) is associated with myocardial disease, frequently left ventricular noncompaction cardiomyopathy, which may necessitate cardiac transplantation or lead to death in some patients. We report a child with BTHS who had an “undulating cardiac phenotype” and ultimately developed decompensated heart failure requiring mechanical circulatory support with a ventricular assist device as a bridge to transplantation. His course was complicated by acute lung injury requiring placement of an in-line oxygenator to maintain end-organ function. Not only was his course complicated by cardiac and respiratory failure but his BTHS associated comorbidities complicated the management of his therapy using mechanical assist device support. He was successfully supported and subsequently was transplanted. Here we discuss the management of a child with BTHS using mechanical circulatory support and describe the use of an in-line oxygenator, Quadrox, with the Berlin Excor device.

Concepts: Blood, Cardiology, Cardiomyopathy, Heart failure, Heart, Circulatory system, Noncompaction cardiomyopathy, Barth syndrome


This report describes a case involving the rare combination of persistent truncus arteriosus type A2, double aortic arch, and mitral stenosis. At the age of 26 days, the patient underwent division of the right-sided aortic arch with tracheal compression and bilateral pulmonary banding. Fontan completion was successfully achieved after separation of the pulmonary artery from the arterial trunk, atrial septostomy, and modified Blalock-Taussig shunt at the age of 7 months and bilateral bidirectional Glenn anastomosis at the age of 1 year and 3 months. At this writing, the patient is doing well 2 years and 6 months after Fontan completion.

Concepts: Blood, Cardiology, Heart, Artery, Pulmonary artery, Cardiovascular system, Pulmonary hypertension, Persistent truncus arteriosus


Sildenafil, a phosphodiesterase-5 inhibitor, is a controversial treatment option for pulmonary arterial hypertension (PAH), a significant complication of bronchopulmonary dysplasia (BPD). The objective of this study was to evaluate the use of sildenafil in infants with PAH secondary to BPD. This was a retrospective review of medical records of all premature infants with PAH associated with BPD treated with sildenafil between January 2009 and May 2013 in a level 3 neonatal intensive care unit. The primary outcomes were clinical response (20 % decreases in respiratory support score or oxygen requirements) and echocardiographic response (20 % decrease in tricuspid regurgitation gradient or change of at least 1° of septal flattening). Twenty-three infants were included in the study. Significant echocardiographic and clinical responses were, respectively, observed in 71 and 35 % of cases. Most clinical responses were observed in the first 48 h of treatment, and the median time to an echocardiographic response was of 19 days. The median dose of sildenafil used was 4.4 mg/kg/day, with a median time to reach the maximum dose of 9 days. Transient hypotension was the primary reported side effect, and it was observed in 44 % of our study population. Sildenafil treatment in patients with PAH secondary to BPD was associated with an echocardiographic improvement in the majority of patients, whereas clinical improvement was observed in a minority of patients. Many infants presented with transient hypotension during the course of the treatment. Further prospective studies are required to better assess safety and efficacy of this treatment in this population.

Concepts: Myocardial infarction, Cardiology, Blood pressure, Effectiveness, Intensive care medicine, Tricuspid valve, Pulmonary hypertension, Hypotension


Postural tachycardia syndrome (POTS) is characterized by symptoms of orthostatic intolerance. Antibodies of acetylcholine receptor (AChR-ab) affect acetylcholine transmission between the ganglia and result in imbalance of the autonomic nervous system in POTS. This study was designed to analyze the clinical characteristics of POTS patients with AChR-ab positive and explore the value of AChR-ab in children with POTS. In 82 children with POTS, twenty patients (24.39 %) were found as AChR-ab positive. Their clinical characteristics and hemodynamic responses to orthostatic changes were compared with the remaining 60 patients with negative AChR-ab. Symptoms of POTS children with AChR-ab positive were significantly severe than those of AChR-ab negative patients (p = 0.001). Preceding infection was predominant in patients with AChR-ab positive compared with that of patients with AChR-ab negative (p < 0.001). Syncope and fatigue were more common in the AChR-ab positive patients (p < 0.05). The change of upright heart rate was increased significantly in AChR-ab positive patients compared with AChR-ab negative cases (p = 0.013). Multiple logistic regression analysis revealed that preceding infection (OR 22.356, 95 % CI 2.151-34.920), syncope (OR 11.570, 95 % CI 2.098-63.810), and fatigue (OR 11.145, 95 % CI 1.658-74.911) were independent risk factors for POTS with AChR-ab positive. In conclusion, POTS with positive AChR-ab was a heterogeneous disorder. Preceding infection, syncope and fatigue were their main clinical characteristics.

Concepts: Nervous system, Regression analysis, Logistic regression, Cardiology, Acetylcholine, Orthostatic hypotension, Postural orthostatic tachycardia syndrome, Orthostatic intolerance


Complex congenital heart disease (CHD) affects cardiac blood flow, generating a pressure overload in the compromised ventricles and provoking hypertrophy that over time will induce myocardial dysfunction and cause a potential risk of imminent death. Therefore, the early diagnosis of complex CHD is paramount during the first year of life, with surgical treatment of patients favoring survival. In the present study, we analyzed cardiac tissue and plasma of children with cardiac hypertrophy (CH) secondary to CHD for the expression of 11 miRNAs specific to CH in adults. The results were compared with the miRNA expression patterns in tissue and blood of healthy children. In this way, we determined that miRNAs 1, 18b, 21, 23b, 133a, 195, and 208b constitute the expression profile of the cardiac tissue of children with CHD. Meanwhile, miRNAs 21, 23a, 23b, and 24 can be considered specific biomarkers for the diagnosis of CH in infants with CHD. These results suggest that CH secondary to CHD in children differs in its mechanism from that described for adult hypertrophy, offering a new perspective to study the development of this pathology and to determine the potential of hypertrophic miRNAs to be biomarkers for early CH.

Concepts: Gene, Blood, Myocardial infarction, Heart, MicroRNA, Cardiac muscle, Heart disease, Circulatory system


Cardiac MR traditionally requires breath-holding for cine imaging. Younger or less stable patients benefit from free-breathing during cardiac MR but current free-breathing cine images can be spatially blurred. Motion corrected re-binning (MOC) is a novel approach that acquires and then reformats real-time images over multiple cardiac cycles with high spatial resolution. The technique was previously limited by reconstruction time but distributed computing has reduced these times. Using this technique, left ventricular volumetry has compared favorably to breath-held balanced steady-state free precession cine imaging (BH), the current gold-standard, however, right ventricular volumetry validation remains incomplete, limiting the applicability of MOC in clinical practice. Fifty subjects underwent cardiac MR for evaluation of right ventricular size and function by end-diastolic (EDV) and end-systolic (ESV) volumetry. Measurements using MOC were compared to those using BH. Pearson correlation coefficients and Bland-Altman plots tested agreement across techniques. Total scan plus reconstruction times were tested for significant differences using paired t-test. Volumes obtained by MOC compared favorably to BH (R = 0.9911 for EDV, 0.9690 for ESV). Combined acquisition and reconstruction time (previously reported) were reduced 37% for MOC, requiring a mean of 5.2 min compared to 8.2 min for BH (p < 0.0001). Right ventricular volumetry compares favorably to BH using MOC image reconstruction, but is obtained in a fraction of the time. Combined with previous validation of its use for the left ventricle, this novel method now offers an alternative imaging approach in appropriate clinical settings.