Pulmonary Atresia with Intact Ventricular Septum associated with Ventriculo-Coronary Arterial Communication in a Fetus at 21 Weeks of Gestation

Pulmonary atresia with the intact ventricular septum (PA-IVS) is a rare anomaly that has an absent communication between the right ventricle and pulmonary arterial circulation. PA-IVS has a hypoplastic and hypokinetic and thickened right ventricle with the intact ventricular septum. It can be diagnosed with PA-IVS in routine obstetric ultrasound examination because the four-chamber view of PA-IVS is generally abnormal. The size of the right ventricular cavity is proportional to the Z value of the diameter of the tricuspid valve. The essential associated cardiac finding of PA-IVS is ventricular-coronary arterial communications (VCAC). The hypertensive RV forces blood through the intramyocardial sinusoids with continuous fistulous contact with the epicardial branches of RCA. It is called the VCAC. A color Doppler can detect VCAC due to its location in the pericardium along the coronary artery. If patients have VCAC and tricuspid Z score below -4, all of these conditions are infallible signs of high mortality rates in their fetal or postnatal lives. Our aim in presenting this case is to remind perinatologists if they detect an abnormal four-chamber view of the fetus’s heart, they should also carefully examine whether VCAC exists. Herein, we report a case of PA-IVS and VCAC at 21 weeks’ gestation with fetal echocardiographic images

A Rare Cause of Pulmonary Hypertension in a 4-Year-Old Toddler: Association of Cor Triatriatum Sinister and Pulmonary Arteriovenous Malformation

Cor triatriatum sinister is a rare congenital cardiac anomaly. The anomaly is caused by a fibromuscular membrane that divides the left atrium into two cavities. This membrane can lead to the obstruction of left atrial flow and also create pulmonary venous hypertension. Pulmonary arteriovenous malformation (PAVM) is notorious for its aberrant blood flow between the pulmonary arteries and veins. Herein, we report a case of a 4-year-old toddler who had a unique form of pulmonary hypertension presenting with cor triatriatum sinister and diffuse PAVM. After the surgical treatment of cor triatriatum sinister, both pulmonary arteriovenous malformation and pulmonary hypertension disappeared

The Possible Role of IVIG in the Treatment of Atrial Fibrillation Accompanied by Fulminant Myocarditis in a 12-Year-Old Pediatric Patient

Background. Fulminant myocarditis (FM) is a potentially lethal condition in children due to rapid progressive hemodynamic instability and cardiogenic shock. Patients with FM might show different clinical manifestations on emergency department admission. Case. Herein, we describe the case of a 12-year-old girl who was admitted to our institution’s emergency department due to complaints of abdominal pain and incessant vomiting. However, we detected an early onset of atrial fibrillation (AF) accompanied by FM. The patient’s condition of AF and severe hemodynamic disorder was successfully treated in our institution’s pediatric intensive care unit. Conclusion. To the best of our knowledge, this is the first report of the co-occurrence of FM and AF successfully treated in childhood. This case report will serve as a guide for the treatment of cases with FM accompanied by AF

Assessment of Right Heart Functions in Children with Mild Cystic Fibrosis

Background Cystic fibrosis (CF) is a multisystemic disease that prevalently involves the lungs. Hypoxemia occurs due to the existing of progressive damage to the pulmonary parenchyma and pulmonary vessels. The condition may cause systolic and diastolic dysfunction to the right ventricle due to the effects of high pulmonary artery systolic pressure (PASP). The study aimed to determine echocardiographic alterations in PASP, right ventricle (RV) anatomy, and functions in mild CF children