Sutureless left pulmonary vein augmentation for primary endoluminal pulmonary vein ostial stenosis: role of pulmonary venous Doppler.

An 11-month-old girl presented with complaints of recurrent episodes of upper respiratory tract infection since birth. Transthoracic echocardiography revealed a large ostium secundum atrial septal defect (ASD), small subaortic ventricular septal defect and left superior and inferior pulmonary vein (PV) ostial stenosis. Computed tomography angiography was done to confirm the findings, and the child were subsequently planned for closure of septal defects and sutureless left-sided pulmonary venous augmentation.

Intraoperative transoesophageal echocardiography (TEE) showed a narrowed opening of the left superior PV ostium into the left atrium in the mid esophageal four chamber view [Figure 1]. Application of color flow Doppler showed turbulence in the course of the left sided PVs [Figure 1 and Video 1]. Continuous-wave Doppler showed a gradient of 18 mm Hg across this membrane and a peak flow velocity of 213 cm/s [Figure 2] indicating a functionally significant obstruction.[1]

Figure 1

Color flow Doppler in mid esophageal four chamber view showing stenosis at the opening of left pulmonary veins and turbulence along the pulmonary venous flow

Figure 2

Continuous wave Doppler in mid esophageal four chamber view showing a continous pulmonary venous flow pattern with a peak gradient of 18 mm Hg and a flow velocity of 213 cm/sec across the stenotic pulmonary vein ostium

Longitudinal incisions were given over the PV ostia and obstruction was released as both the left sided PVs were laid open freely draining into the pericardium left atrial walls were subsequently sutured over the pericardium containing the left PV drainage. Intracardiac repair of the ventricular and ASDs was then performed, and the child was weaned off cardiopulmonary bypass. Postoperative TEE showed no turbulence in the course of the left superior PV [Figure 3 and Video 2] and the gradient across it decreased to 1 mm Hg and normal PV flow pattern was resumed [Figure 4].

Figure 3

Color flow Doppler in mid esophageal four chamber view after the surgery showing no stenosis at the opening of the pulmonary veins along with a nonturbulent pulmonary venous flow

Figure 4

Postleft pulmonary vein augmentation pulsed wave Doppler analysis of the pulmonary venous drainage in mid esophageal four chamber view showing a normal pulmonary venous flow pattern and no significant gradient or velocity

Primary PV stenosis results from an abnormal incorporation of the common PV into the left atrium. On echocardiography, it may appear as a discrete shelf, long narrow segment or as a diffuse hypoplasia of the PVs resulting in pulmonary venous hypertension secondary to an impeded pulmonary venous drainage.[2] Seen almost exclusively in young children; it is highly associated with other cardiac defects, primarily septal defects, making it imperative that echocardiographic evaluation of patients with pulmonary hypertension specifically include pulmonary venous profiling. In the sutureless marsupialization technique, any direct stitches over the cut edges of the PVs are avoided as the pericardium around the PVs is directly attached to the left atrium. According to a limited literature, an unimpeded pulmonary venous drainage is best achieved using this technique in comparison to the previous techniques that have used direct anastomosis after resection of stenotic segments or patching of the stenotic veins.[34]