Transcatheter closure of a fistula between the right pulmonary artery and left atrium using the Amplatzer septal occluder

A congenital fistula between the right pulmonary artery (RPA) and left atrium (LA) is a rare condition that results in central cyanosis. An 11-year-old boy was admitted with exertional dyspnea and easy fatigability. He had severe cyanosis of the lips and limbs with clubbing of the fingers. Systemic oxygen saturation was 70%. There was no abnormal finding on electrocardiography, chest radiography, and echocardiography. Agitated saline injection showed early appearance of contrast bubbles in the LA. A pulmonary arteriovenous fistula was suspected and diagnostic cardiac catheterization was performed. Angiography demonstrated a large fistula between the proximal RPA and LA. The narrowest part of the fistula was 13.8 mm in balloon sizing. A 14-mm Amplatzer septal occluder was deployed at the narrowest site; however, the device migrated to the LA and then to the aortic arch. The device was removed and was successfully reimplanted to the fistula. After the procedure, arterial oxygen saturation increased from 70% to 96% and control angiography demonstrated complete occlusion of the fistula. The patient was symptom-free on follow-up evaluations at 6, 12, and 18 months, with a mean oxygen saturation of 96%. This case represents the first pediatric patient in whom a septal occluder was used

Kearns-Sayre syndrome presenting as somatomedin C deficiency and complete heart block

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease in which neuromuscular structures, endocrine glands, and cardiac conduction systems are most commonly involved. An 11-year-old boy was admitted with blurred consciousness, respiratory instability, and bradycardia of two-hour onset. He was immediately intubated. His medical history included growth retardation and myopic refractive defect for six years, therapy for somatomedin C deficiency for 15 months, and bilateral ptosis for three months. On physical examination, he was unconscious, had a peak heart rate of 40/min, blood pressure of 60/20 mmHg, and a weak pulse. Laboratory findings showed elevated blood lactate and blood pyruvate levels and an increased lactate/pyruvate ratio. The electrocardiogram showed complete atrioventricular block and echocardiography showed mitral valve prolapse. Following implantation of a temporary transvenous cardiac pacemaker, his heart rate and clinical condition improved. Further analysis with cranial magnetic resonance (MR) imaging demonstrated hyperintense signal changes in the subcortical white matter of the two cerebral hemispheres, bilateral thalamus, putamen, cerebral peduncles, dorsal medulla, and midbrain. The typical clinical and MR findings confirmed the initial diagnosis of KSS. A permanent cardiac pacemaker was implanted into the right ventricle

Balloon pulmonary valvuloplasty in a premature neonate

Interventional cardiac catheterization procedures in low-birth-weight neonates are associated with specific problems including hypothermia during the procedure, difficulty in vascular access, and problems related to the small size of cardiac structures. Physical examination of a premature neonate, delivered by cesarean section at the 34th week of gestation with birth weight of 1790 g revealed a systolic murmur of 3/6 intensity. The murmur continued on the following day and cardiomegaly was observed on a chest radiogram. Echocardiographic examination revealed a patent foramen ovale with right-to-left shunt, patent ductus arteriosus, right ventricular hypertrophy, and valvular pulmonary stenosis. Balloon pulmonary valvuloplasty was performed upon detection of increased right ventricular pressure and pulmonary artery pressure gradient and development of marked tachypnea and dyspnea. Following the procedure, right ventricular pressure and pulmonary gradient decreased and the infant was discharged eight days after the procedure. Pulmonary artery pressure gradient was 56 mmHg after a month