Chronic chest pain after percutaneous atrial septal defect closure.

Percutaneous closure of atrial septal defect is recognised as a safe and effective procedure, however, in some patients complications may occur. Although chest pain has been sporadically reported, its exact aetiology has been poorly studied. Herein, a 14-year-old female with an atypical and long-lasting chest pain after percutaneous atrial septal defect closure is described.

Acute Myocarditis with Infarct-like Presentation in a Pediatric Population: Role of Cardiovascular Magnetic Resonance.

Chest pain is a typical symptom of acute myocarditis in adolescents. It may be indistinguishable from myocardial ischemia so it is called "infarct-like pattern." Cardiovascular magnetic resonance has an important role as a non-invasive diagnostic tool. The aim of our study is to provide a description of an acute myocarditis series with infarct-like pattern and to evaluate the cardiovascular magnetic resonance role in a pediatric population. We included all pediatric patients (0-16 years) admitted to our hospital (May 2007-May 2016) with clinical diagnosis of acute myocarditis and infarct-like presentation (chest pain, EKG alterations, and released cardiac biomarkers). Diagnosis was confirmed with cardiovascular magnetic resonance using Lake Louise criteria. Seven patients (five males, two females) with a median age of 14 years (12.5-15.2) were included. All patients showed ST-segment changes and released cardiac biomarkers. Three patients had left ventricular hypertrophy and two presented mild systolic left ventricular dysfunction. All patients had at least two positive Lake Louise criteria. Late gadolinium enhancement was positive in all of them. With a median follow-up of 23 months (8-47), all of them are alive, with no cardiac symptoms and normal ventricular function. Infarct-like pattern is a typical presentation of acute myocarditis in adolescents. CMR should be performed in this population and may be considered as a first-line diagnostic tool. Its high sensitivity in infarct-like acute myocarditis may allow us to avoid endomyocardial biopsy. Unlike what was described in adults, late gadolinium enhancement does not imply worse outcome in our series.

Characteristics and outcomes of the Spanish registry for pediatric patients with bicuspid aortic valve (REVAB).

INTRODUCTION AND OBJECTIVES: Bicuspid aortic valve (BAV) disorder is the most common congenital heart disease. The aim of this study was to describe the characteristics of 0- to 18-year olds with BAV in a population-based registry. METHODS: Data from all pediatric patients were obtained from the Spanish registry for pediatric patients with bicuspid aortic valve (REVAB) (< 18 years). For data analysis, patients with BAV were divided into 2 groups by their features: isolated BAV and BAV with associated congenital heart disease. RESULTS: We included 1681 patients from 33 hospitals. Males accounted for 69.6% (n = 1158). Valve morphology was horizontal in 63.4% (n = 1012) and pure (Sievers type 0) in 28.4% (n=469). Isolated BAV was present in 63.7% (n=1060), and concomitant left-sided obstructive lesions in 23.4% (n=390). Interventions were required in 8.6% (n=145). CONCLUSION: These data represent the first large, population-based description of the clinical presentations and outcomes of patients enrolled in the Spanish registry for pediatric patients with bicuspid aortic valve.

Adolescents with bicuspid aortic valve: Which criteria should we use for aortic dilatation?

BACKGROUND: Criteria to define aortic dilatation in bicuspid aortic valve (BAV) patients are different for children and adults. The objective of this study was to find the best reference tool to define dilation of the aortic root (AR) and the ascending aorta (AA) in BAV adolescents with an adult body surface area (BSA). METHODS: Patients recruited were ≥10-years-old with a BSA ≥1.5 m2. Three measurements of the AR and AA were compared: z-score, the BSA-indexed value (BSA-IV) and the absolute value (AV), with thresholds in +2/+3, 21 mm/m2 and 40 mm, respectively. RESULTS: 231 subjects were collected from the Pediatric REVAB database, with a median age and BSA of 14-year-old and 1.67 m2. Significant differences were reported in the AA: 109 (47%) patients had a z-score ≥2 and 67 (29%) a Z ≥ 3, but only 9 (3%) a BSA-IV ≥21 mm/m2 (p < 0.01 and p < 0.01) and 2 (0.9%) an AV ≥40 mm (p = 0.22 and p = 0.08). CONCLUSION: Our results indicate that in the AA there are a significant number of patients in which it would be recommendable changing to BSA-IV when children are older than 10-year-old and BSA ≥1.5 m2. Regarding the AR, criteria for dilatation seems not to be influenced by the reference chosen.

Stenting Coarctation of the "Fifth Aortic Arch": A Safe and Attractive Therapeutic Alternative to Surgery.

"Persistence of the fifth aortic arch" is a rare congenital cardiovascular anomaly that consists of an abnormal vessel arising from the distal ascending aorta connecting with the systemic or pulmonary circulation. We report a case of a type A interruption of the aortic arch and a coarctation of the fifth aortic arch, which connected the ascending with the descending aorta. No cardiac surgery was required because a covered stent was successfully implanted in the fifth aortic arch when the patient was 4 years old. A chromosome 9 q arm duplication of uncertain significance was also found, an anomaly never described before in this clinical context.

The Chiari Network-Only an Embryonic Remnant or a Confusing Finding?

Chiari network is an embryonic remnant of valves of the sinus venosus, which can be observed in several locations in the right atrium. Although it is usually considered a normal anatomic variant, when associated with certain clinical conditions, the Chiari network may become a confusing finding, and a careful differential diagnosis is required. It should be differentiated from a tricuspid valve disruption, vegetation, thrombus, or tumoral mass. In this case report, we describe a singular case of endocarditis over a Chiari network in a seven-year-old boy that was successfully managed in a conservative fashion.

Characterization of electrocardiographic findings in young students.

INTRODUCTION AND OBJECTIVES: The resting 12-lead electrocardiogram (ECG) has been used in the evaluation of young asymptomatic individuals to detect pre-existing heart disease, but systematic ECG use is controversial and there are no data on this population in our environment. We aimed to determine the prevalence and spectrum of electrocardiographic findings in a population of secondary school students. METHODS: We conducted an observational, cross-sectional study of resting ECG findings in all 13 to 14-year-old secondary school students in a region of the province of Gerona between 2009 and 2017. ECG findings were classified into 3 groups according to the modified criteria of Corrado et al.: normal ECG findings, ECG findings suggestive of adaptive changes, and pathologic findings. Students with pathologic ECG findings were referred to a tertiary hospital, and complementary tests were performed according to a pre-established protocol. RESULTS: A total of 1911 ECGs were obtained, with a participation rate of 79% of all high school students. In all, 1321 students (69%) had a normal ECG, 554 (29%) showed ECG findings suggestive of adaptive changes, and 36 (2%) had pathologic ECG findings. Among the group with pathologic findings, 5 (14%) had cardiovascular disease. The prevalence of heart disease in this group of asymptomatic secondary school students was 0.3%. CONCLUSIONS: One third of the students had ECG findings that were mostly suggestive of physiological adaptation. One seventh of the students with pathologic ECG findings had pre-existing heart disease, although the overall prevalence of pre-existing heart disease was low.

Supraventricular tachycardia in children from the perspective of a specialised between-hospital transport team.

AIMS: The aim of this study is to establish the incidence of supraventricular tachycardia (SVT) as a main reason for between-hospital transfer in children, as well as to describe the clinical presentation, prognosis and treatment, risk factors presenting with haemodynamic compromise, and to propose a specific management protocol for the transport. METHODS: A retrospective observational study was conducted on all patients with supraventricular tachycardia transferred by the Hospital Vall d'Hebron Sistema de Emergencias Médicas Pediátricas (SEM-P) between January 2005 and June 2017. RESULTS: During the study period, 67 (0.9%) patients (out of a total number of 7348 transfers) suffered from SVT. The median age was 57 days (2 h-18 years old). There was clinical evidence of cardiogenic shock on admission in 14 (20.9%) patients. Age ≤1 year was the only independent risk factor associated with presenting with cardiogenic shock on admission, with an OR of 10.2 (95% CI: 1.2-89.9; p = 0.004). The majority of patients could be treated appropriately by the local hospital team, except for oral intubation and cardioversion that were performed mainly by the transport team on arrival at the local hospital. Median stabilisation time was 35 min (9-169), and median total transport time was 30 min (9-165). CONCLUSIONS: Only 0.9% of transport cases are due to SVT, but this can be highly demanding as patients can be critically ill. Age ≤1 year was the only independent risk factor associated with presenting with cardiogenic shock on admission. Coordination between the local and the transport teams is crucial for a good clinical outcome.

[Supraventricular tachycardia in children from the perspective of a specialised between-hospital transport team]. / Taquicardia paroxística supraventricular desde la perspectiva del transporte interhospitalario.

AIMS: The aim of this study is to establish the incidence of supraventricular tachycardia (SVT) as a main reason for between-hospital transfer in children, as well as to describe the clinical presentation, prognosis and treatment, risk factors presenting with haemodynamic compromise, and to propose a specific management protocol for the transport. METHODS: A retrospective observational study was conducted on all patients with supraventricular tachycardia transferred by the Hospital Vall d'Hebron Sistema de Emergencias Médicas Pediátricas (SEM-P) between January 2005 and June 2017. RESULTS: During the study period, 67 (0.9%) patients (out of a total number of 7348 transfers) suffered from SVT. The median age was 57 days (2 hours-18 years old). There was clinical evidence of cardiogenic shock on admission in 14 (20.9%) patients. Age ≤ 1 year was the only independent risk factor associated with presenting with cardiogenic shock on admission, with an OR of 10.2 (95% CI: 1.2-89.9; P=.004). The majority of patients could be treated appropriately by the local hospital team, except for oral intubation and cardioversion that were performed mainly by the transport team on arrival at the local hospital. Median stabilisation time was 35minutes (9-169), and median total transport time was 30minutes (9-165). CONCLUSIONS: Only 0.9% of transport cases are due to SVT, but this can be highly demanding as patients can be critically ill. Age ≤ 1 year was the only independent risk factor associated with presenting with cardiogenic shock on admission. Coordination between the local and the transport teams is crucial for a good clinical outcome.

Echocardiography in the Assessment of Left Atrial Pressure After Pediatric Heart Surgery: A Comparison Study With Measurements Obtained From Left Atrial Catheter.

BACKGROUND: Correlation between ventricular end-diastolic pressure and pulsed Doppler and tissue Doppler-derived E/e' ratio has been widely reported in adults but scarcely studied in children with congenital heart diseases. This ratio is defined as the relationship between diastolic transmitral flow velocity (cm/s; E) and myocardial diastolic relaxation velocity (cm/s; e') in the lateral aspect of the mitral annulus. Our main objective was to ascertain whether a correlation existed between direct measurement of left atrial pressure and echocardiographic E/e' ratio in children after heart surgery. METHODS: Prospective study including 27 consecutive children after pediatric heart surgery. Data were analyzed according to whether they were obtained within the first 72 hours following surgery or later on. Sensitivity, specificity, positive and negative predictive values, and areas under the receiver-operating characteristics curve of E/e' ratio in detection of left atrial pressure values ≥13 mm Hg were evaluated. RESULTS: Forty-eight studies were conducted in 27 patients. Thirty-two studies were performed during the first 72 hours after heart surgery and 16 beyond the third day. Median patient age was 0.82 years (5 days-16 years). Median left atrial pressure values and E/e' measurements of the whole cohort (N = 48) were 12.0 and 10.2, respectively. Intraclass correlation index between left atrial pressure values and echocardiographic E/e' ratio was 0.35, 0.25 for studies performed within 72 hours, but 0.78 (P < .01) for those performed later. There was also a high positive predictive value, since in 13 (87%) of 15 studies with an E/e' ratio ≥13, the left atrial pressure was ≥13 mm Hg. CONCLUSION: While echocardiographic E/e' ratio did not show a good correlation with left atrial pressure in the immediate postoperative period, the positive predictive value may suffice to aid clinicians in predicting elevated pressures.