Hemodynamic vise in obstructed total anomalous pulmonary venous connection

Excerpt

A 10-day-old neonate presented with respiratory distress and poor feeding from the 7th day of life. The baby was in congestive heart failure. Precordial examination revealed cardiomegaly, notably split second heart sound with a loud pulmonary component and a short mid-systolic murmur in the pulmonary area. The oxygen saturation on room air was 90% in all limbs. Cardiomegaly and pulmonary venous congestion were conspicuous on chest roentgenogram. Electrocardiography showed right ventricular hypertrophy and normal QRS axis. Two-dimensional transthoracic echocardiography showed supracardiac total anomalous pulmonary venous connection (TAPVC). The interatrial communication was not restrictive, measuring 5 mm and showed continuous laminar flow from right to left. The interventricular septum was intact, and the arterial duct was not patent. Doppler interrogation showed right ventricular systolic pressure of 50 mmHg. The suprasternal view (Fig. 1a, Supplementary video 1) revealed the entire anomalous course of pulmonary venous drainage. The vertical vein which connected to the left brachiocephalic vein had focal obstruction [pulsed-wave Doppler mean gradient 9 mmHg with aliasing (Fig. 1b), continuous wave Doppler mean gradient 15 mmHg (Fig. 1c)] at the region of the crossing left pulmonary artery anteriorly and left main-stem bronchus posteriorly, a point often referred to as the “hemodynamic vise” in this setting. Based on satisfactory echocardiographic demonstration of anatomy, the patient was taken up for surgery without the need for additional imaging by computed tomography or magnetic resonance imaging, after a Joint Cardiac Meet. The child underwent primary sutureless TAPVC repair and tanned pericardial patch closure of the ASD, and is doing well on follow-up.

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