The use of N-terminal (1–76) pro-brain natriuretic peptide in the aetiology of severe respiratory distress in the paediatric emergency department

Introduction: Acute respiratory distress is one of the most common reasons for paediatric emergency visits. Paediatric patients require rapid diagnosis and treatment. Our aim in this study was to use N-terminal (1–76) pro-brain natriuretic peptide to differentiate respiratory distress of cardiac and pulmonary origin in children. Our aim was to investigate the role of N-terminal (1–76) pro-brain natriuretic peptide in the detection of patients with new-onset heart failure in the absence of an underlying congenital heart anomaly. Methods: All children aged 0–18 years who presented to the paediatric emergency department due to severe respiratory distress were included in the study prospectively. The patients’ demographic characteristics, presenting complaints, clinical findings, and N-terminal (1–76) pro-brain natriuretic peptide concentrations, were investigated. In patients with severe Pediatric Respiratory Severity Score, congestive heart failure score was calculated using the modified Ross Score. Results: This study included 47 children between the ages of 1 month and 14 years. The median N-terminal (1–76) pro-brain natriuretic peptide concentration was 5717 (IQR:16158) pg/mL in the 25 patients with severe respiratory distress due to heart failure and in the 22 patients with severe respiratory distress due to lung pathology was 437 (IQR:874) pg/mL (p < 0.001). In the 25 patients with severe respiratory distress due to heart failure, 8281 (IQR:8372) pg/mL in the 16 patients with underlying congenital heart anomalies, and 1983 (IQR:2150) pg/mL in the 9 patients without a congenital heart anomaly (p < 0.001). The 45 patients in the control group had a median N-terminal (1–76) pro-brain natriuretic peptide concentration of 47.2 (IQR:56.2) pg/mL. Conclusion: Using scoring systems in combination with N-terminal (1–76) pro-brain natriuretic peptide cut-off values can help direct and manage treatment.

An Incidental Finding of Unroofed Coronary Sinus During Angiography Confirmed By 3D Echocardiography; A Case Report

Unroofed Coronary Sinus (UCS) is an uncommon congenital heart anomaly and the most unusual type of Atrial Septal Defect.This report presents a-71-year-old female with palpitation and dyspnea following an emotional stress, who was referred for ablation of typical atrial flutter.Finally diagnosed with a partially UCS causing a bidirectional shunt.

Fontan operation for tricuspid atresia with absent pulmonary valve: a case series

Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.

Surgical management in tetralogy of Fallot with rare unilateral pulmonary anomalies: A literature review

Tetralogy of Fallot with unilateral pulmonary anomalies such as the unilateral absence of pulmonary artery or unilateral pulmonary agenesis is an extremely rare complex congenital heart anomaly. There is no established surgical algorithm for tetralogy of Fallot with concomitant unilateral pulmonary anomalies. This condition is still challenging, especially in the surgical field. In this review we also present our experiences in our center, Dr Cipto Mangunkusumo General Hospital, Jakarta, Indonesia. This literature review aimed to discuss systematic treatment options and hoped to help the decision-making process when surgeons face these rare anomalies.

Early Neonatal Cyanosis as a Presentation of a Rare Cardiac Anomaly: Truncus Arteriosus Type IV

Truncus arteriosus (TA) is a rare congenital heart anomaly presenting with mild cyanosis and congestive heart failure. It occurs when the blood vessels coming out of the heart in the developing baby fail to separate completely during development, leaving a common trunk responsible for the pulmonary and systemic perfusion. There are several variants of TA, depending on the specific anatomy and arterial connection. We report a case of a full-term newborn who developed cyanosis and desaturation during the first day of life. Investigations and echocardiography were consistent with TA type IV. Prostaglandin infusion was immediately started, and then, a successful palliative right modified Blalock Taussig shunt was performed.

Truncus Arteriosus Type 1: A Prenatal Diagnosis, Case Report, and Literature Review

Common arterial trunk (persistent truncus arteriosus) is a rare, congenital heart anomaly and characterized by Ventricular Septal Defect (VSD), single truncal valve, and a common ventricular outflow tract. We reported a case of truncus arteriosus type 1 in the fetus of a 28 years-old G2-P1-L1 pregnant female at 24 weeks of gestational age with large sub truncul VSD, truncal overriding, and main pulmonary artery bifurcation to the right and left pulmonary arteries.

Evaluation and diagnostic approach for heart murmurs in children

Heart murmurs are common in children. Most of them are innocent murmurs with normal heart anatomy and function, and only a few are pathologic murmurs with congenital heart anomaly or abnormal heart function. However, a heart murmur may be the sole symptom of serious heart disease. Therefore, careful evaluation of heart murmurs for distinguishing pathologic murmurs from innocent murmurs is important. Heart murmurs are described by their intensity, timing in the cardiac cycle, location, transmission, and quality. Murmurs, such as a holosystolic or diastolic murmur, of grade 3 or higher intensity, harsh quality, an abnormal S2, a systolic click, or increased intensity when the patient stands are more likely to be pathologic murmurs. Innocent murmurs are more likely to be systolic murmurs, with soft sounds, short duration, low pitch, and varying intensity with phases of respiration and posture (disappears with standing). Not only auscultation but also physical examination findings are important to evaluate heart murmurs. The gold standard test for the evaluation of any potentially pathologic murmur is echocardiography. For the appropriate use of echocardiography, close physical examination, including auscultation, is essential and requires considerable practice.

Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension

Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension.

Multiple Congenital Heart Abnormalities

Congenital heart abnormalities are rare and most often occur separately. These structural and functional disorders can cause a range of diseases that vary in severity, from mild or even asymptomatic to severe and life-threatening. Occasionally these conditions could cause no symptoms in which case they are found accidentally when the patient is examined for other reasons or during autopsy. In this article we present the case of a 47-year-old male patient with triple congenital heart anomaly detected in two consecutive tests performed in pre-hospital medical care.