A term 3-kg-baby girl born by Cesarean section had severe respiratory distress, cyanosis and shock immediately after birth. APGAR was six at 1 minute and eight at 5 minutes. The initial evaluation showed cyanosis, poor perfusion and heart rate of 160/min. Examination of the cardiovascular system did not reveal any significant findings. The chest roentgenogram showed pneumothorax on the left side and uniform reticular pattern on the right side . The ABG showed pH of 7.27, PO2 of 22 mmHg, PCO2 of 29 mmHg, saturation of 60% and base excess of – 12 mEq/L. The left pneumothorax was drained with an 18-G needle; however, baby did not improve and continued to have significant desaturation. Hence, an emergency echocardiogram was obtained which showed normal situs, normal atrioventricular-ventriculoarterial concordance. There was increased flow in the right superior vena cava. All pulmonary veins formed a small confluence from which an ascending vertical vein was seen draining into left innominate vein. The color flow mapping of vertical vein showed long segment of critical obstruction with a threadlike lumen. There was a 5-mm interatrial communication and a 4-mm arterial duct with right-to-left shunt. The baby was ventilated in view of clinical deterioration and refractory hypoxemia. A few hours later, baby became unstable and developed cardiac arrest twice which was successfully managed.
Chest reontgenogram showing pulmonary venous hypertension with pneumothorax on the left side
Cardiac catheterization with intention of stenting of vertical vein was planned at 12 hours of life in view of unstable hemodynamics. The cine angiogram showed supracardiac TAPVR with critical obstruction of left vertical vein and small confluence . The obstructed left vertical vein was crossed with a 0.014” Galeo extra support wire (Biotronics, Germany), using a 5F Judkins right coronary guiding catheter (Cordis, USA). A 4 × 28 mm ProLink stent (Vascular Concepts Limited, India) was positioned and deployed under fluoroscopy guidance. An additional 4 × 10 mm ProLink stent was deployed to cover the small residual stenosis. Post-procedure angiogram showed establishment of good communication between the pulmonary venous confluence and the left innominate vein . The postprocedure ABG showed PO2 of 44 mmHg and saturation of 76%. The pulmonary artery pressure could not be obtained due to unstable hemodynamics.
Figure 2 Pre and post cine-angiogram in anterio - posterior projection of endovascular stenting of the vertical vein. (a) Cine angiogram showing the critically obstructed vertical vein with thread like lumen (arrow). (b) Poststent deployment showing good flow (more ...)
A transumblical balloon atrial septostomy was performed on day three in view of restrictive interatrial communication. The repeat chest roentgenogram showed some degree of reduction of pulmonary venous congestion and increased pulmonary vascularity . Serial echocardiograms showed good stent flow. The shunt across the arterial duct became left-to-right.
The chest roentgenogram showing plethora and the stent position corresponding to the vertical vein (arrow)
The baby developed features suggestive of sepsis and blood culture showed growth of Gram-negative bacteria; hence surgery was rescheduled till the completion of antibiotics. Extubation was attempted twice, on day 7 and day 10, but was failed. The baby had severe sepsis and succumbed at the end of the third week.