A nonrestrictive interatrial communication is mandatory in some patients with complex cyanotic heart disease. Up to the present time, Park blade atrial septostomy with a Park blade catheter is performed only under fluoroscopic guidance. We successfully monitored only under transthoracic 2-dimensional (2D) echocardiography (TTE), in a 7-week-old infant with d-Transposition of Great Arteries (d-TGA), a restrictive patent foramen ovale (PFO), and small ventricular septal defect (VSD), who presented with severe hypoxemia. TTE confirmed the position of the blade before and during pullback to the right atrium, followed by balloon septoplasty. There was an adequate enlargement of the interatrial communication with subsequent improvement in the oxygenation of the infant. This is the first reported case of the performance of blade atrial septostomy as a bedside TTE guided procedure here in the Philippines.

Human ; Male ; Infant ; Heart Septal Defects, Ventricular ; Foramen Ovale, Patent ; Philippines ; Echocardiography ; Heart Atria ; Cardiac Surgical Procedures ; Transposition Of Great Vessels ; Hypoxia

CLINICAL PRESENTATION: This report describes a case of a 24-year-old, acyanotic, female patient presenting with mild effort dyspnea, a right ventricular heave, a displaced cardiac apex, a loud single S2, a holosystolic murmur on the left parasternal area, and a continuous murmur at the left posterior chest. Transthoracic and transesophageal echocardiography demonstrated dilated left atrium and ventricle, hypertrophied right ventricle and a large ventricular septal defect with overriding of the aorta. However, both studies failed to visualize the pulmonic valve. The patient underwent cardiac catheterization which revealed the presence of pulmonary atresia. A descending aortogram showed the presence of a single large major aortopulmonary collateral artery (MAPCA) arising from the descending thoracic aor ta which supplies confluent left and right pulmonary arteries. The pulmonary arterial vasculature is enlarged and pruned in appearance. The main pulmonary artery is non-atretic. Mean proximal pulmonary artery pressure was 68 mmHg, indicating severe pulmonary hypertension. Patient wasstarted on digoxin and sildenafil.
SIGNIFICANCE: This case report emphasizes the importance of a well-developed central pulmonary circulation and an adequate but not excessive pulmonary blood supply from an aortopulmonary vascular connection in the survival of patients with pulmonary atresia into adulthood.

Human ; Female ; Adult ; Young Adult ; Aorta ; Cardiac Catheterization ; Dyspnea ; Heart Atria ; Heart Septal Defects, Ventricular ; Hypertension, Pulmonary ; Pulmonary Atresia ; Pulmonary Circulation ; Pulmonary Valve ; Tetralogy Of Fallot